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DOI: 10.1002/pbc.

29952 Pediatric
Blood &
Cancer The American Society of
Pediatric Hematology/Oncology
ABSTRACTS

ORAL ABSTRACTS
GLOBAL HEALTH felt that the material will impact their practice. Group discussions were
highlighted by 73% as being a most effective aspect. The top 3 voted
GLOBAL HEALTH: BEST OF SIOP GLOBAL HEALTH NETWORK themes for future workshops were statistical analysis (64%), writing &
FREE PAPERS 28-09-2022 8:15 AM - 9:45 AM publishing (45%), and quality improvement (39%).
Conclusions: This workshop demonstrates the potential for HIC-LMIC
O001 / #129 ADDRESSING THE GAP IN RESEARCH network partnerships in targeting research training gaps. Lessons
METHODOLOGIES EDUCATION IN PEDIATRIC ONCOLOGY IN learned will be applied to the planning of future workshops to
THE EASTERN MEDITERRANEAN REGION strengthen research in LMICs.

Raya Saab1 , Victor Santana2 , Meenakshi Devidas2 , Anas Obeid1 , Asim


Belgaumi3 , Nickhill Bhakta2 , Paula Naidu2 , Vaskar Saha4 , Iyad Sultan5 , O002 / #638 SOCIOECONOMIC STATUS HAS NO LONGER
Ramandeep Arora6 , Lilly Mukoka2 , Carlos Rodriguez-Galindo2 , Sima IMPACT ON OS AND EFS OF CHILDHOOD ALL IN
Jeha2 YOGYAKARTA, INDONESIA AFTER INTRODUCTION OF
1 American University of Beirut, Pediatrics, BEIRUT, Lebanon; 2 St Jude UNIVERSAL HEALTH CARE ACT
Children’s Research Hospital, Global Pediatric Medicine, Memphis, United
States of America; 3 Aga Khan University, Pediatrics, Karachi, Pakistan; Claudia Adelin1 , Rahmadani Lestari1 , Braghmandita Indraswari1 ,
4 Tata Memorial Center, Pediatrics, Mumbai, India; 5 King Husien Casncer Sutaryo Sutaryo1 , Anjo Veerman1,2
Center, Pediatric, AMMAN, Jordan; 6 Max Super-Specialty Hospital, Medical 1 Universitas Gadjah Mada, Department Of Pediatrics, Yogyakarta, Indone-
Oncology, New Delhi, India sia; 2 Emma Children’s Hospital, Amsterdam UMC, Vrije Universiteit Amster-
dam, Pediatric Oncology, Amsterdam, Netherlands
Background and Aims: Formal training in research methodologies is
limited in LMICs. The Pediatric Oncology East & Mediterranean Group Background and Aims: About 60% of our patients come from low
(POEM) and St. Jude Global developed a workshop focused on capacity socioeconomic status (SES). Our previous study showed that low SES
building in research skills. We describe its structure, implementation, negatively affected the outcome of children with ALL. This study aimed
and early results. to re-examine whether SES influences the outcome of treatment in
Methods: Leveraging virtual capabilities, lectures and small group children with ALL after the implementation of the Universal Health
‘breakout’ exercise sessions were conducted for 3 hours per day on 2 Care Act (UHC).
consecutive days over 2 weeks, at 15:00-18:00 GMT to accommodate Methods: Since February 2013 until November 2018, 362 children
time zones. Topics included research design, data registries, healthcare with ALL were registered in Sardjito General Hospital Yogyakarta,
statistics, research ethics, and scientific writing. Each applicant submit- 174 using ALL2013 protocol and 188 using ALL2016 protocol. They
ted a research study abstract. Breakout groups selected one abstract were divided into two groups: low SES and higher SES. Families with a
each for further development, which they subsequently presented in a monthly income of less than 2.5 million IDR (approximately US 5 a day)
groupwide session. Feedback was captured through an online survey. or treated in third class ward were labeled low SES. The 4-year pOS and
Results: Attendance included 29 registrants from 12 countries and 6 pEFS were analyzed using Kaplan Meier method.
disciplines (17 physicians, 6 research staff, 2 nurses, 4 other). Final Results: Among 362 children, 241 (66.6%) come from low SES fami-
output presentations included a retrospective, a prospective observa- lies. The 4-year-pOS of the low SES and the higher SES in ALL2013
tional, a prospective interventional, and a registry study proposal. After were 48.4% and 54.2% respectively (P= 0.346) and in ALL2016 59.1%
the workshop, participants were invited to further develop their origi- and 61.6% (P= 0.504). The 4-year-pEFS of the low SES and the higher
nal abstracts and 3 received additional mentoring. The post-workshop SES in ALL2013 were 34.9% and 41.7% (P= 0.305), and in ALL2016
survey was filled by 100% of attendees, with a score of “excellent” or 48.7% and 50.7% (p=0.668). The number of abandonments decreased
“very good” given by 84% on overall quality, 87% on platform suitabil- significantly compared to our previous study (35% in 2006, 11.5% in
ity, and 72% on course length. Course objectives were deemed met by ALL2013; P<0.001, and 5.9% in ALL2016; P<0.001). After the imple-
79-94% of participants, by specific session. At least 25% of the material mentation of UHC, more people from lower SES could access health
was new for 93%, and more than 50% was new for 51%. All attendees facilities and were less hindered by financial problems.

Pediatr Blood Cancer. 2022;69(Suppl. 5):e29952. wileyonlinelibrary.com/journal/pbc © 2022 Wiley Periodicals LLC. S1 of S635
https://doi.org/10.1002/pbc.29952
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S2 of S635 ABSTRACTS

Conclusions: After free access to healthcare facilities, economic fac- O004 / #500 PEDCAN: ACCEPTABILITY AND FEASIBILITY OF
tors seemed to no longer significantly affect the outcome of children A MOBILE APPLICATION BY PRIMARY HEALTH-CARE
with ALL. WORKERS TO RECOGNIZE CHILDREN WITH CANCER IN
UGANDA

O003 / #346 MULTI-CENTER, PHASE 3, RANDOMIZED Barnabas Atwiine


CONTROLLED TRIAL, COMPARING DAUNORUBICIN AND ARAC Mbarara University of Science and Technology, Paediatrics And Child
(DA) VERSUS ARAC, DAUNORUBICIN, AND ETOPOSIDE (ADE) Health, Mbarara, Uganda
INDUCTION CHEMOTHERAPY IN PEDIATRIC ACUTE MYELOID
LEUKEMIA (INPOG-AML-01-14) Background and Aims: Early diagnosis of cancer in children is depen-
dent on a high index of suspicion and referral by primary health
Venkatraman Radhakrishnan1 , Sameer Bakhshi2 , Smita Kayal3 , care workers (PHCW) to cancer specialists at tertiary centres. The
Cherian Thampy1 , Ankit Batra1 , Praveen Kumar Shenoy1 , Hemanth aim of this study was to assess the acceptability and feasibility of
Kumar1 , Shilpi Chaudhary2 , Reema Bisht2 , Swaminathan Rajaraman4 , PEDCan mobile application by primary health care workers to rec-
Biswajit Dubashi3 , Trivadi Ganesan5 ognize children with cancer-related clinical features, initiate remote
1 Cancer Institute (W.I.A), Medical Oncology, CHENNAI, India; 2 All India consultations with specialists and refer them timely for diagnosis.
Institue of Medical Sciences, Medical Oncology, New Delhi, India; 3 JIPMER, Methods: We developed a mobile application, called PEDCan, to aid
Medical Oncology, Puducherry, India; 4 Cancer Institute (W.I.A), Biostatis- PHCWs recognize children with cancer, hold remote consultations
tics, Chennai, India; 5 Sri Ramachandra Medical College, Medical Oncology, with specialists and refer them to cancer centres. In May 2021, we con-
Chennai, India ducted a cross-sectional descriptive study among consented PHCWs
in southwest Uganda to collect data about knowledge regarding child-
Background and Aims: The benefit of three-drug induction hood cancer, smart phone ownership and internet usage. After training
chemotherapy over a two-drug induction has not been evaluated them on the use of the application using mock clinical scenarios, their
in pediatric acute myeloid leukemia (AML). We, therefore, conducted ease and readiness to use it was assessed. Descriptive statistics were
a multi-center, open-label, investigator-initiated phase III randomized used to calculate proportions of participants able and willing to use it.
controlled trial to ascertain the benefit of a three-drug induction Ethical approval was provided by Research and Ethics Committee of
regimen in pediatric AML. Mbarara University of Science and Technology
Methods: Patients aged between 1-18 years with newly diagnosed Results: Forty-four PHCWs participated in the study. Twenty-five
AML were recruited in the study between 22nd May 2014 and (56.8%) were nurses/midwives, 8 (18.2%) clinical officers, 3 (6.8%) gen-
14th November 2019 across three sites in India. They were randomized eral doctors and 8 (18.2%) other cadres. All had heard about childhood
to two cycles of induction chemotherapy with daunorubicin and ara-C cancer and 28 (63.6%) had ever seen a child suspected to have can-
(DA) or two cycles of ara-C, daunorubicin, and etoposide (ADE). After cer. Thirty-eight (86.4%) participants owned a smart phone and used
completing the induction, patients in both arms received consolidation internet and 12 (31.6%) had access to internet all the time. Thirty-eight
with two cycles of high-dose ara-C. The study’s primary objective was (86.3%) participants found the use of PEDCan very easy or easy and all
to compare the event-free survival (EFS) between the two arms. The were willing to use it.
secondary objectives were to compare the complete-remission (CR) Conclusions: PHCWs were able and willing to use PEDCan application
rates, overall survival (OS), and toxicities. to recognize children with cancer and initiate remote expert consul-
Results: The study randomized 149 patients, 77 patients in the DA tations. A pilot study with real patients would further demonstrate
and 72 in the ADE arm. The median age was 8.7 years, and 92 (62%) effectiveness in real life clinical settings.
patients were males. The median follow-up was 50.9 months. After the
first induction, the CR rate in the DA and ADE arm were 65% and 68%
(P=0.68), respectively, while it was 82% and 79% (P=0.68) after the IPSO
second induction. There were 13 (17%) induction deaths in the DA arm,
of which nine were treatment-related, and 12 (17%) in the ADE arm, of IPSO FPS 1: RENAL AND LIVER TUMOURS, AND
which seven were treatment-related (P=0.97). Forty patients relapsed NEUROBLASTOMA 28-09-2022 8:15 AM - 9:45 AM
in DA arm and 37 in ADE (P=0.94). The 5-year EFS in the DA and
ADE arm was 34.5% and 34.5%, respectively (P=0.66). The 5-year OS O005 / #1526 OPTIMAL TREATMENT STRATEGIES FOR
in the DA and ADE arm was 41.4% and 42.1%, respectively (P=0.77). HEPATOBLASTOMA WITH ADVANCED PRETEXT STAGE
There were no significant differences in toxicities between the
regimens. Kyung-Nam Koh1 , Jin Kyung Suh2 , Jung-Man Namgoong3 , Hee Mang
Conclusions: ADE induction regimen did not improve EFS, OS, and CR Yoon4 , Young Kwon Koh1 , Su Hyun Yoon1 , Aae Jin Kang1 , Sung Han
in pediatric AML compared to DA regimen. Kang1 , Hyery Kim1 , Dae Yeon Kim3 , Ho Joon Im1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S3 of S635

1 Asan Medical Center, University of Ulsan College of Medicine, Pediatrics, III-IV) either primary or recurrent. Rescue transplants for recurrence
Seoul, Korea, Republic of; 2 Korea Cancer Center Hospital, Pediatrics, Seoul, have been associated with worse outcomes. We aim to present a single
Korea, Republic of; 3 Asan Medical Center, Pediatric Surgery, Seoul, Korea, institutional experience with resection for PRETEXT III-IV tumors and
Republic of; 4 Asan Medical Center, Radiology, Seoul, Korea, Republic of compare outcomes to those after liver transplantation for HPB.
Methods: A review of patients operated for PRETEXT III-IV HPB
Background and Aims: The advances in surgical techniques and (including hepatocellular neoplasm, not otherwise specified, HCN-
risk-stratified chemotherapy have improved the survival rate of hepa- NOS) at a single national pediatric hepatobiliary referral center was
toblastoma (HB) to over 80%. However, in patients with advanced HB, performed (1997-2021). We compared outcomes among 3 groups:
the survival outcomes remain approximately 50-60%. Therefore fur- resection alone (G1), resection followed by rescue transplant (G2), and
ther optimization of treatment strategies in advanced HB is imperative primary transplant (G3). p<0.05 was considered significant.
to improve outcomes. Results: Sixty-three patients presented with PRETEXT III-IV HPB
Methods: We retrospectively reviewed the medical records of 44 (including 9 patients with HCN-NOS tumor): 45 underwent primary
patients with HB ≥ PRETEXT stage III. We evaluated overall survival resection of which 4 recurred. Seven patients received a rescue
(OS), event-free survival (EFS), and transplant-free survival (TFS) in transplant including 2 who were referred with recurrent tumor after
association with prognostic factors including pre-treatment extent of being initially treated and resected at another institution. Sixteen
tumor (PRETEXT) stage, post-treatment extent of tumor (POSTTEXT) patients received a primary transplant. Comparing transplant groups
stage with their annotation factors, and newly defined Children’s Hep- (G3 and G2), the 5-year event free survival (EFS) and overall patient
atic tumors International Collaboration-Hepatoblastoma Stratification survival (OS) were superior in G3 vs G2 (5-yr EFS: 93.8% vs. 42.9%,
(CHIC-HS) system. p<0.001; OS G3 100.0% vs. G2 57.1%, p=0.006). Post-transplant
Results: OF the 44 patients, 27 (61%) were PRETEXT 3. Fifteen (34%) tumor recurrence was more frequent in rescue transplants (G3 57.1%
had metastasis at diagnosis: all were lung metastasis. Except for 3 vs. G2 6.3%, p=0.006). Of patients with HCN-NOS, 3/3 patients who
patients, one who received upfront surgery and 2 who died before received a primary transplant had EFS in contrast to only 3/6 after a
surgery, 41 received surgery after a median 6 cycles of chemotherapy primary resection (p=0.13). Overall, mortality from recurrent disease
(range, 3-15): 22 received hepatectomy, and 19 received LT. The 5-year was similar between G3 group and all patients who underwent a
OS, EFS, and TFS were 87%, 77%, and 43%. The known prognostic fac- resection as primary treatment (G3 0.0% vs. G1+G2 12.8%, p=0.13).
tors including PRETEXT, POSTTEXT, and annotations did not affect the Conclusions: Rescue transplant for hepatoblastoma has improved
OS and EFS, while patients who received LT had better outcomes than from previous reports but remains challenging. Primary trans-
those who received hepatectomy (5-year OS and EFS of 100% and 90% plant and resection alone for PRETEXT III and IV hepatoblastoma
vs. 82% and 73%, P<0.05). Otherwise, in the analysis of TFS, the PRE- both offer similar excellent survival. Patients with HCN-NOS have
TEXT and POSTTEXT were significant. Among the annotation factors, worst outcomes and would benefit from early referral to centers
P and F affected the TFS, while M did not affect it. of excellence and aggressive initial management including primary
Conclusions: Our study represented LT as a valuable modality to over- transplantation.
come poor prognostic factors in the treatment of advanced HB. Further
refinement of surgical strategies, including LT indications based on a
careful review of PRETEXT and POSTTEXT with annotations, is needed O007 / #1696 PROGNOSTIC FACTORS AND TREATMENT
to improve the outcomes. OUTCOMES IN PRIMITIVE NEUROECTODERMAL TUMOURS OF
THE SPINE: A SINGLE CENTRE ANALYSIS OF 83 PATIENTS

O006 / #1793 SURGICAL MANAGEMENT OF PRETEXT III-IV Archana Sasi1 , Sameer Bakhshi1 , Shuvadeep Ganguly1 , Bivas Biswas2 ,
HEPATOBLASTOMA: COMPARISON OF OUTCOMES AFTER Deepam Pushpam1 , Sandeep Agarwala3 , Shah Khan4 , Venkatesan
PRIMARY TRANSPLANT, RESCUE TRANSPLANT AND LIVER Kumar4 , Ahitagni Biswas5 , Shashank Kale6
RESECTION 1 All India Institue of Medical Sciences, Medical Oncology, New Delhi, India;

2 Tata Medical Centre, Medical Oncology, Kolkata, India; 3 All India Insti-
Caroline Lemoine1 , Katherine Brandt1 , Chelsea Self2 , Riccardo tute of Medical Sciences, Pediatric Surgery, New Delhi, India; 4 All India
Superina1 Institue of Medical Sciences, Orthopaedics, New Delhi, India; 5 All India
1 Ann & Robert H. Lurie Children’s Hospital of Chicago, Division Of Trans- Institute of Medical Sciences New Delhi, Radiotherapy And Oncology, New
plant And Advanced Hepatobiliary Surgery, Chicago, United States of Delhi, India; 6 ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI,
America; 2 Ann & Robert H. Lurie Children’s Hospital of Chicago, Division Neurosurgery, new delhi, India
Of Hematology, Oncology, And Stem Cell Transplantation, Chicago, United
States of America Background and Aims: Primitive neuroectodermal tumours (PNETs)
of the spine are rare childhood cancers with sparse literature on their
Background and Aims: Liver transplantation for hepatoblastoma survival outcomes. We aim to describe the treatment outcomes and
(HPB) is indicated for patients with unresectable tumor (PRETEXT prognostic factors in a cohort of patients with spinal PNET treated in
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S4 of S635 ABSTRACTS

a single institution using a uniform treatment protocol over an 18-year information of surgery. Three patients underwent surgery rather than
period. biopsy before neoadjuvant chemotherapy. Finally, 21 patients were
Methods: We conducted a single institutional retrospective analysis selected and analyzed IDRFs in association with surgical complications.
of patients with PNET of the spine registered at a tertiary care oncol- Results: Three patients had localized tumor only and 18 patients had
ogy centre between 2003 to 2018. Data pertaining to clinical and local and metastatic lesions. All patients were treated by neoadju-
demographic characteristics of the patients and treatment outcomes vant chemotherapy. Surgery rather than biopsy was performed after
was collected from hospital records. Univariable and multivariable Cox 2 to 9 courses of neoadjuvant chemotherapy. IDRFs were present in
regression analysis was used to identify the association of baseline 18 patients at diagnosis and 11 patients preoperatively. Major surgi-
clinical parameters with event free survival (EFS) and overall survival cal complications such as class III or IV intraoperative bleeding, major
(OS). vascular injury, bowel obstruction, renal atrophy was observed in 7
Results: A cohort of 83 patients was analysed including 37 (44.5%) patients. Of these, IDRFs were present in 7patients at diagnosis and
patients with metastatic disease. The median age was 16 years (IQR: 4 patients preoperatively. Regarding surgical complications, sensitivity
13-19.5 years) with 73% of the patients being male. Nearly 20% of and specificity of IDRFs was 100% and 21.4% at diagnosis, 57.1% and
all patients (n=15) had been misdiagnosed to have spinal tuberculo- 57.1% preoperatively respectively.
sis at presentation and received antitubercular therapy. Local therapy Conclusions: Compared to the low-risk protocol JN-L-10, surgical com-
was administered in 60 (72.3%) patients with surgery alone in 8 (9.6%), plications were observed more frequently in the intermediate-risk
radiotherapy alone in 17 (20.5%) and both surgery and radiother- patients (14.7% in low-risk, 33.3% in intermediate-risk). Surgery should
apy in 35 (42.2%) patients. The median EFS and OS were 15 months be performed carefully in the patients with IDRFs at diagnosis, if IDRFs
(IQR: 7.7-31 months) and 18 months (IQR 9.4-41.5 months) respec- would decrease or disappeared by neoadjuvant chemotherapy.
tively. On univariable analysis, age above 15 years, haemoglobin (<10
g/dL), serum albumin (<3.5 g/dL) and female gender were found to pre-
dict for shorter OS and EFS. Serum albumin (<3.5) remained the only O009 / #925 GANGLIONEUROMAS IN CHILDHOOD: A
factor predictive for EFS (HR 2.42; p-value 0.005) and OS (HR 2.11; SINGLE CENTER EXPERIENCE WITH 70 CASES
p-value=0.032) on multivariable analysis. Survival outcomes were
similar in localised and metastatic disease. Elif Aydin Goker1 , Bilgehan Yalçın2 , Ibrahim Karnak3 , Diclehan
Conclusions: Serum albumin level was the only prognostic factor for Orhan4 , Mithat Haliloglu5 , Saniye Ekinci3 , Berna Oguz5 , Burca Aydin2 ,
event free and overall survival in patients with PNET of the spine. Nilgun Kurucu2 , Ali Varan2 , Tezer Kutluk2
1 Hacettepe University Faculty of Medicine, Department Of Pediatrics,
Ankara, Turkey; 2 Hacettepe University Faculty of Medicine, Department
O008 / #634 SURGICAL EVALUATION IN THE Of Pediatric Oncology, Ankara, Turkey; 3 Hacettepe University, Department
INTERMEDIATE-RISK NEUROBLASTOMA PROTOCOL JN-I-10 Of Pediatric Surgery, Ankara, Turkey; 4 Hacettepe University Faculty of
FROM THE JAPAN CHILDREN’S CANCER GROUP Medicine, Department Of Pathology, Ankara, Turkey; 5 Hacettepe University
NEUROBLASTOMA COMMITTEE Faculty of Medicine, Department Of Radiology, Ankara, Turkey

Akihiro Yoneda1,2 , Tomoko Iehara1 , Atsushi Kikuta1 , Toshihiro Background and Aims: Ganglioneuromas (GNs) are rare benign
Muraji1 , Kazuaki Tokiwa1 , Hideto Takahashi1 , Satoshi Teramukai1 , peripheral neuroblastic tumors (PNTs). The primary treatment modal-
Tetsuya Takimoto1 , Shigeki Yagyu1 , Hajime Hosoi1 , Tatsuro Tajiri1 ity is surgical resection. We aimed to review our institutional experi-
1 Japan Children’s Cancer Group, Neuroblastoma Committee, Kyoto, Japan; ence with childhood GNs.
2 National Center for Child Health and Development, Surgical Oncology, Methods: Hospital files of the children with PNTs between 1995 and
Tokyo, Japan 2021 were reviewed, and cases with the histopathological diagnosis
of GN were identified. Data concerning demographic characteristics,
Background and Aims: The Japan Children’s Cancer Group (JCCG) clinical, laboratory, and radiological findings, image-defined risk factors
Neuroblastoma Committee (JNBSG) conducted the JN-I-10 for (IDRF), INRG stages, diagnostic and surgical procedures, histopatho-
intermediate-risk patients using Image-defined risk factors (IDRFs) logical findings, and overall outcome were recorded.
as the main factor for determining treatment. This sJN-I-10 is the Results: Of 668 cases with PNTs, 70 (10.4%) had GNs. The median age
first clinical trial on the efficacy of IDRF-based surgical decision and was 7.4 years (range, 2.6-15.7 years; 16/70 <5 years) (females/males,
stepwise treatment intensification for patients with intermediate-risk 41/29). Common presenting complaints were abdominal pain and
neuroblastomas. In order to clarify the predictive capability of IDRFs cough; 33/70 cases were diagnosed incidentally. Primary tumor sites
for surgical complications in intermediate-risk neuroblastoma, we were the abdomen in 41/70, the thorax in 25/70, the neck in 3 cases,
made surgical evaluation in JN-I-10. and the pelvis in one. Urinary HVA and VMA were elevated in 8 cases.
Methods: Of the 61 patients enrolled in JN-I-10, 54 patients were eli- The median tumor size was 6.5 cm (range, 1.4-17). Fifty cases (71.4%)
gible. Of these, 28 patients underwent surgery rather than biopsy. Four with no IDRF were staged as INRG-L1; 20 cases with IDRF(s) (15 sin-
patients were excluded from the analysis due to lacking the precise gle, five >1) were staged as INRG-L2. Complete and partial tumor
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S5 of S635

resections were performed in 58/70 and 6/70 cases, respectively (6 gical resection and conservative ‘ watch and wait ‘ surveillance. This
had no resection). The overall complication rate was 17.1% (11/64) study details management, complications and long term follow up of
(Horner syndrome 3, renal atrophy 2, leg hypoesthesia 2). At a median GN/GNBi comparing the outcomes of surgery versus surveillance.
follow-up of 9 years (range, 0-27), 5 cases were lost to follow-up; 65 Methods: Retrospective UK nationwide study including patients (aged
were alive. One patient with a gross tumor residue underwent total 0-24 yrs) diagnosed with localized GN or GNBi from 1990-2020.
resection due to tumor progression 13 years after the initial surgery. Results: Eleven of 17 UK CCLG centres participated in the study and
Eleven other cases with gross residual tumors experienced no tumor 242 patients were included. Median age at presentation was 6.41
progression in the follow-up. yrs (IQR: 3.88-11.09); median follow up 5.08 yrs. The majority were
Conclusions: GNs are benign PNTs, and most are free of IDRFs. Even female (n=147, 61%). 51 (21%) patients were initially managed with
with incomplete or partial resection, long-term outcomes are excellent. active surveillance versus 191 (79%) with surgery. Patients who under-
A multidisciplinary approach is necessary, and the decision of tumor went surveillance were found to have more IDRFs than the surgery
resection should be made meticulously for each case. group (54% versus 38%; p=0.09). There was no significant difference(s)
in tumour volume observed (109.8cm3 vs 108.1cm3 ; p=0.60). Twenty
patients (39%) underwent intervention after an initial period of obser-
O010 / #198 MANAGEMENT OF GANGLIONEUROMA AND vation (19 had surgery, 1 had cryoablation) due to clinical changes (i.e.
GANGLIONEUROBLASTOMA INTERMIXED - A RETROSPECTIVE increased size of the tumour, worsening/severe symptoms), parental
UK STUDY anxiety or to confirm histopathology. The extent of tumour resection
was comparable between the delayed and initial ‘up front’ surgery
Katherine Burnand1 , Annita Budzanowski2 , Bruce Okoye1 , Kate groups (77% vs 71% had >95% gross macroscopic resection). Of 208
Cross2 , Kate Wheeler3 , Rhiannon Collins3 , Amy Drawbridge3 , patients who underwent surgery, two patients died (1%) and three
Jonathan Neville4 , Juliet Gray4 , Nigel Hall4 , Ramya Ramanujachar4 , cases (1.4%) had significant morbidity (Clavien Dindo IIIa, IIIb or IV).
Max Pachl5 , Snigdha Reddy5 , Susanne Gatz5 , Carla Kielrulff6 , Lisa 49 patients (24%) have ongoing symptoms (N=42 predate surgery).
Howell7 , Chun Sui Kwok7 , Rajeev Shukla7 , Barry Pizer7 , Timothy Overall, five patients died - 2 from surgery complications and 3 non
Rogers8 , Robin Garrett-Cox8 , Nadeem Alkhafaji8 , Vicky Anne tumour-related causes (one in surveillance group).
Carruthers9 , Deborah Tweddle9 , Giuseppe Barone10 , John Conclusions: In this study most patients with GN/GNBi underwent
Anderson2 , Sucheta Vaidya11 , Sally George11 , Nichola Seymour12 , ‘upfront’ surgical resection. Watchful surveillance may represent a
Sarah Braungart13 , Guy Makin12 , Michael Jacovides14 , Paul Losty15 , safe approach in select patients with GN/GNBi and IDRFs. However,
Hany Gabra16 , Paola Angelini11 a significant proportion who were initially observed (39%) ultimately
1 St George’s Hospital, Paediatric Surgery, QT, United Kingdom; 2 Great required surgery. Overall mortality (2%) in both groups remains
Ormond Street, Paediatric Surgery, London, United Kingdom; 3 Oxford low.
University Hospital NHS Foundation Trust, Paediatric Haematology And
Oncology, Oxford, United Kingdom; 4 University of Southampton, Cancer
Sciences Unit, Southampton, United Kingdom; 5 Birmingham children’s hos- O011 / #1332 LAPAROSCOPIC NEPHRON SPARING
pital, Paediatric Surgery, Birmingham, United Kingdom; 6 Royal Aberdeen SURGERY FOR PEDIATRIC KIDNEY TUMOURS: PRELIMINARY
Children’s Hospital, Paediatric Oncology, Aberdeen, United Kingdom; 7 Alder EXPERIENCE
Hey Childrens Hospital NHS Foundation Trust, Paediatric Oncology, Liv-
erpool, United Kingdom; 8 University Hospitals Bristol and Weston NHS Sabine Irtan1 , Christophe Laplace2 , Daniel Orbach3 ,
foundation trust, Department Of Pediatric Surgery, Bristol, United King- Marie-Dominique Tabone4 , Hélène Boutroux4 , Frederic Hameury5
dom; 9 Newcastle University, Newcastle University Centre For Cancer, 1 Trousseau Hospital, Paediatric Surgery, Paris, France; 2 CHU Pointe-à-
Newcastle upon Tyne, United Kingdom; 10 Great Ormond Street Hospital Pitre, Guadeloupe, Pointe-à-Pitre, France; 3 Institut Curie, Siredo Oncology
for Children NHS Foundation Trust, Paediatric Oncology, London, United Department, Paris, France; 4 Armand Trousseau Hospital -APHP, Pediatric
Kingdom; 11 Royal Marsden Hospital, Children And Young Peoples Unit, Oncology, Paris, France; 5 Hospices Civils de Lyon, Pediatric Surgery, Bron,
Surrey, United Kingdom; 12 University of Manchester, Division Of Can- France
cer Sciences, Manchester, United Kingdom; 13 Royal Manchester Children’s
Hospital, Paediatric Surgery, Manchester, United Kingdom; 14 Glasgow Chil- Background and Aims: Nephron sparing surgery (NSS) is a key
dren’s Hospital, Paediatric Surgery, Glasgow, United Kingdom; 15 University surgical technique heading to preserve as much as possible kidney
of Liverpool, Institute Of Life Course And Medical Sciences, Liverpool, United function while treating with strict oncological rules the different
Kingdom; 16 Great hany.Gabra North Children’s Hospital, Paediatric Surgery, histological types of pediatric kidney tumours. NSS is a demand-
Newscastle, United Kingdom ing technique usually performed by open surgery. Laparoscopy is
of increasing use for total nephrectomy but has not yet been
Background and Aims: Ganglioneuroma (GN) and ganglioneurob- developed for NSS. The aim of our presentation is to describe
lastoma intermixed (GNBi) classically follow a more benign clinical a preliminary experience of laparoscopic NSS in pediatric kidney
course than neuroblastoma (NB). Management can vary between sur- tumours.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S6 of S635 ABSTRACTS

Methods: Laparoscopy was intended for tumours located at one part of nephrectomy. The most frequent contraindication to LN was infil-
the kidney whatever their size or contact with calices but with respect tration of extrarenal structures or extension beyond the ipsilateral
to oncological indication of partial nephrectomy. border of spinal column 11/21 (52%); 4/21 (19%) had >1 contraindi-
Results: Four patients (3 girls and one boy) aged from 3 to 11 years cation. NSS would have been suitable for 1/24 (4%); 18/23 (78%)
were operated on upfront for three of them (2 renal cell carcinoma and had >1 contraindication for NSS, with the most frequently encoun-
one epithelioid angiomyolipoma) and after neoadjuvant chemother- tered limiting factor being tumour volume at diagnosis >300 ml, 17/23
apy for one patient with Wilms tumour. Tumours were located at the (74%).
right upper part of the kidney in two cases, in the lower part of the Conclusions: Retrospective comparison of a series of WT patients
left kidney in 2 cases and measured a mean of 4 cm [2.7-6.3]. The sur- against current guidelines demonstrates that adoption of MIS and NSS
gical technique relied on a 10 mm 3D optic port and 2 or 3 working in WT is limited in most children by advanced local stage at diagnosis.
ports of 5 mm. The coagulation of the cut parenchyma was obtained Identification of factors permitting earlier diagnosis and/or extension
with bipolar, coagulation device and parenchyma compression but no of the conservative, yet safe SIOP/CCLG appear necessary if less inva-
vascular clamping was needed. The mean operative time was 210 mn sive surgical approaches in WT are to be more widely achieved in the
[120-265]. The calices were opened in two patients to assure good sur- UK.
gical margins and closed by sutures with resorbable stitches. The per
and postoperative courses were uneventful. All patients are alive with
good oncological outcome and kidney function at a mean follow-up of O013 / #1090 ATTITUDE OF UK ONCOLOGY SURGEONS
12 months [4-18]. TOWARDS NEPHRON SPARING SURGERY (NSS) FOR WILMS
Conclusions: Laparoscopic NSS for kidney renal tumours seemed feasi- TUMOUR
ble and safe with no warm ischemia time. Further experience is needed
to confirm this preliminary experience. Liliana-Elena Banias, Katherine Burnand, Bruce Okoye
St George’s Hospital, Paediatric Surgery, London, United Kingdom

O012 / #1507 BARRIERS TO IMPLEMENTING Background and Aims: NSS for bilateral Wilms tumour (BWT) is the
MINIMALLY-INVASIVE AND NEPHRON SPARING SURGERY FOR accepted standard. It is also desirable in unilateral syndromic cases
WILMS TUMOUR NEPHRECTOMIES – A UK SINGLE (USD). NSS in cases of unilateral non syndromic disease (UNSD) is still
INSTITUTIONAL STUDY not universally accepted due to concerns about upstaging. The aim
of our study was to evaluate the attitude of UK oncology surgeons
Florin Djendov, Anastasia Mentessidou, Anna-May Long, Claire towards NSS in general.
Jackson Methods: A 15-question survey was distributed to all UK paediatric
Cambridge University Hospitals, Department Of Paediatric Surgery, Cam- oncological surgeons.
bridge, United Kingdom Results: 24 surgeons from 16 centres participated. 10/16 (62.5%) treat
between 5-10 WT per year. 14/16 (87.5%) centres would perform NSS
Background and Aims: The role of minimally invasive surgery (MIS) in for bilateral disease and 13/16 (81.2%) for USD. However only 10/16,
nephrectomy for Wilms tumour (WT) is constantly evolving. Similarly, 62.5% said that they would perform NSS for UNSD. One centre would
use of nephron sparing surgery (NSS) for unilateral tumours in non- refer all cases of NSS to another centre. 81.2% had performed NSS for
syndromic children is increasingly advocated. The aim of this study was BWT, 56.2% for USD and 43.7% for UNSD in the last 5 years. Only 9/21
to identify factors limiting the use of laparoscopy in WT nephrectomy (42.8%) of surgeons felt that all UNSD patients who met the “Umbrella”
(LN) and NSS within our population. criteria for NSS should be offered NSS. 16/23 (69.5%) felt that par-
Methods: A retrospective review of consecutive children who under- ents should always be given the option of NSS where children met the
went nephrectomy for WT from January 2015 - January 2022 was “Umbrella” criteria. Most surgeons (20/24, 83.3%) considered the local
undertaken. Those with bilateral WT or requiring cardiac input were MDT as the ideal forum for NSS decisions to be made. 11/24 (45.8%)
excluded. Preoperative data were evaluated against the latest Chil- felt it should be the surgeon’s choice while only 6/24 (25%) felt the deci-
dren’s Cancer and Leukaemia Group Clinical Management Guidelines sion should involve the National Renal Advisory Panel (NRAP). 37.5%
(CCLG/SIOP Umbrella Protocol – January 2020) for renal tumours, of respondents felt that referral to the NRAP should be made for NSS
to assess feasibility of LN or NSS. Data are presented as median in BWT and USD, and 50% for UNSD. Only 6/24 (25%) thought that
(IQR). NSS should be centralised. All surgeons not doing NSS for UNSD would
Results: Twenty-four children treated for WT met the inclusion crite- be willing to change their practice.
ria, ten were excluded by the above criteria. Median age at diagnosis Conclusions: NSS for unilateral non syndromic Wilms tumour has not
was 33 months (23 - 67) and the median tumour volume prior been fully embraced in the UK. There is minimal support for the cen-
to neoadjuvant chemotherapy was 471 ml (258 - 743). Three chil- tralisation of NSS. It is important to gain consensus on this issue in the
dren (13%) would have been amenable to LN and underwent open UK.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S7 of S635

O014 / #554 VIRTUAL RESECTION: A NEW TOOL FOR apy (ABVAPCT) could have prognostic value on event-free (EFS) and
PREPARING FOR NEPHRON-SPARING SURGERY IN PATIENTS overall survival (OS).
WITH WILMS TUMOR Objective: Investigate relationship between ABVAPCT with EFS and
OS in patients with intermediate risk wilms tumor.
Jasper Van Der Zee1,2 , Matthijs Fitski2 , Cornelis Van De Ven2 , Aart Methods: Our study collected patients from 2010 to 2021 in Onco-
Klijn3 , Marc Wijnen2 , Alida Van Der Steeg2 Pediatric Depatment. The ABVAPCT was calculated using the formula
1 University of Twente, Techmed Centre, Enschede, Netherlands; 2 Princess of Umbrella protocol: length × depth × thickness × 0.523 × (1- %
Máxima Center for Pediatric Oncology, Pediatric Surgery, Utrecht, Nether- post chemotherapy necrotic fraction) × % fraction of blastema. Student
lands; 3 University Medical Center Utrecht / Wilhelmina Children’s Hospital, test was used for qualitative variables and Pearson test for quantita-
Pediatric Urology, Utrecht, Netherlands tive parameters. Survival curves were obtained by Kaplan-Meier and
compared by log-rank test.
Background and Aims: Nephron-Sparing surgery (NSS) in patients Results: Our series consisted of 61 patients; 34 boys and 27 girls with
with Wilms Tumor (WT) is a surgically challenging procedure used in a median age of 36 months (10-192 months). The histological type was
highly selective cases only. Virtual resections can be used for preoper- stromal in 15 cases (24,6%), blastemal in one case (1,6%), epithelial in
ative planning of NSS to estimate the remnant renal volume (RRV) and 2 cases (3,3%), regressive in 18 cases (29,9%) and mixte in 25 cases
to virtually mimic radical tumor resection. In this single-center valida- (41%). There was not a significant relationship between the ABVAPCT
tion study, virtual resection for NSS planning and the user experience and the histological types (p=0,077), laterality (p=0,094) and gender
were evaluated. (p=0,052). Nevertheless, there was a significant association between
Methods: Virtual resection was performed in nine patients with WT EFS and the ABVAPCT in the absence of a threshold (p=0,001) or
cases by two pediatric surgeons and one pediatric urologist. Pre- and when the cutoff of 100ml was taken into consideration (p= 0,016).
postoperative MRI scans were used for 3D visualization. The virtual This was not the case of the ABVAPCT with the threshold of 20ml
RRV was acquired after performing virtual resection and a question- (p=0,633). There was not a significant difference in term of survival
naire was used to assess the ease of use. The actual RRV was derived when a cut off 20ml or 100ml were considered. However, this was not
from the postoperative 3D visualization and compared to the derived the case between the total absence and the presence of the ABVAPCT
virtual RRV. (p=0,001).
Results: Virtual resection resulted in virtual RRVs that matched nearly Conclusions: The ABVAPCT seems to be of prognostic relevance,
perfectly with the actual RRVs. The questionnaire showed that virtual showing that the total absence of the absolute blastemal volume have
resection appeared to be straightforward and was not considered to be a better outcome and a better survival.
difficult.
Conclusions: This study demonstrated the potential of virtual resec-
tion as a new planning tool to estimate the RRV after NSS in patients IPSO
with WT. Future research should further evaluate the clinical relevance
of virtual resection by relating it to surgical outcome in NSS. IPSO FPS 2: SARCOMA, ENDOCRINE, OVARIAN TUMOURS,
AND THORACIC SURGERY 28-09-2022 1:45 PM - 3:15 PM

O015 / #438 THE PROGNOSTIC VALUE OF RESIDUAL O016 / #58 WHAT DO WE KNOW ABOUT SURVIVAL IN
BLASTEMAL VOLUME AFTER PRE-OPERATIVE CHEMOTHERAPY SKELETALLY PREMATURE CHILDREN AGED 0 TO 10 YEARS
IN PATIENTS WITH INTERMEDIATE RISK WILMS TUMOR WITH EWING SARCOMA? MULTICENTER 10-YEAR FOLLOW-UP
STUDY IN 60 PATIENTS
Yoldez Houcine1 , Gada Sahraoui2 , Faten Fedhila3 , Med Amine
Mansouri3 , Ilhem Jebabli4 , Lamia Charfi2 , Karima Mrad2 , Raoudha Lizz Van Der Heijden1 , Sarah Bosma1 , L Sierrasesumaga2 , Hans
Doghri2 Merks3 , Lianne Haveman3 , Michiel Van De Sande1 , Mikel San Julián4
1 Salah Azaeiz Institution, Immuno-histo-cytology Department„ Ariana, 1 Leiden University Medical Center, Orthopaedic Surgery, Leiden, Nether-
Tunisia; 2 Salah Azaeiz Institution, Pathology Department, Tunis, Tunisia; lands; 2 Clínica Universidad de Navarra, Pediatrics, Pamplona, Spain;
3 Bechir Hamza Children Hospital, (3) department Of Pediatric A, Tunis, 3 Prinses Máxima Centrum voor Kinderoncologie, Pediatric Oncology,
Tunisia; 4 Bechir Hamza Children’s Hospital, (3) department Of Pediatric A, Utrecht, Netherlands; 4 Clínica Universidad de Navarra, Service Of Ortho-
Tunis, Tunisia pedic Surgery And Traumatology, Pamplona, Spain

Background and Aims: INTRODUCTION Background and Aims: Younger age has been associated with bet-
Nearly 40% of relapses in Wilms tumor (WT) occur in children whose ter overall survival (OS) in Ewing sarcoma (ES), especially under the
tumors were classified as intermediate risk. It was hypothesized age of 10. Favorable survival in younger patients underlines the need
that the absolute blastema volume after preoperative chemother- for minimizing treatment burden and late sequalae. Our study aimed
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S8 of S635 ABSTRACTS

at describing clinical characteristics, treatment and outcome of ES ment of aorta (n=4), superior mesenteric artery and celiac axis (n=3),
patients aged 0-10. iliac arteries (n=8), involvement of sacral/obturator nerves (n=5),
Methods: In this retrospective multicenter study, all consecutive ES hydronephrosis (n=7), ureteric encasement (n=7) with renal atrophy
patients aged 0-10, treated in four sarcoma centers in the Netherlands (n=2), and sarcomatosis peritonei (n=2). Seven tumors with peri-
(n=33) and one in Spain (n= 27) (1982-2008), with minimum follow- iliac extension were associated with ureteric encasement. Respective
up of 10 years were included. OS, local recurrence free survival (LRFS) application of perivascular tumor excision techniques, intraoperative
and distant metastasis free survival (DMFS) were calculated. Potential neuromonitoring, ureteric stenting and ureterolysis, and cytoreductive
factors of influence on OS (risk and protective factors) were analyzed. surgical technique optimized outcome. All patients received radiother-
Results: Sixty patients with median follow-up 13.03 years were apy for local control. In mean follow-up of 4.8 (1-11.8) years, 3 died of
included. All patients were treated with chemotherapy and local treat- disease, 1 died of second malignancy, 1 alive with bone relapse.
ment, being surgery in 30 (50%) patients, radiotherapy (RT) in 12 (20%) Conclusions: RMS in abdomen and pelvis typically present with large
patients or surgery plus RT in 18 (30%) patients (12 pre- and 6 post- tumors with risk of rupture. Preoperative chemotherapy improves
operative). Limb salvage was achieved in 93% of patients. The 10-OS, the feasibility of R1 resection. The identified surgical risk factors
-LRFS and -DMFS is 81% (95%CI 71-91%), 89% (95%CI 85-93%) and highlighted the need for new precautionary techniques for better
81% (95%CI 71-91%), respectively. Six patients developed LR, of which outcome.
2 with subsequent DM; all had axial ES (pelvis, spine or chest wall)
and these patients all died. Ten patients developed DM; 8 died due
to progressive disease and two are currently in remission, both with O018 / #1854 EVALUATING THE ROLE OF SURGICAL
pulmonary metastasis only. Negative or wide resection margin was sig- RESECTION AND RECONSTRUCTION IN THE MANAGEMENT
nificantly associated with better OS. Age <6, tumor volume <200ml, OF EWING SARCOMA OF THE CHEST WALL IN THE
absence of metastatic disease and treatment after 2000 showed trends PAEDIATRIC POPULATION; A SYSTEMATIC REVIEW
towards better OS. 2 patients developed secondary malignancy, both
had chemotherapy combined with definitive RT for local treatment. Darragh Rice1 , Seán Barrett2 , Jonathan Mcguinness3
Conclusions: Today, overall survival of these youngest patients with ES 1 Mater Misericordiae University Hospital, Cardiothoracic Surgery, Dublin,
was very good. Limb salvage surgery was achieved in >90% of patients. Ireland; 2 Mater Misericordiae University Hospital, Cardiothoracic Surgery,
Wide resection margin was the only factor significantly associated with Dublin, Ireland; 3 Children’s Health Ireland (CHI) at Crumlin, Cardiothoracic
better survival. Surgery, Dublin, Ireland

Background and Aims: The management of Ewing sarcoma in chil-


O017 / #932 SURGICAL RISK FACTORS IN dren has evolved over the last 30 years with surgical role and approach
RHABDOMYOSARCOMA OF ABDOMEN AND PELVIS following the collaborative oncology group guidelines. This review of
the literature aimed to assess how these surgical guidelines have been
Chan Hon Chui applied in the modern era.
Mount Elizabeth Medical Centre, Surgery Centre For Children, Singapore, Methods: A systematic review was conducted in accordance with
Singapore PRISMA guidelines across four major literature databases. Data
regarding overall survival, rate of recurrence, role of surgery, adjuvant
Background and Aims: Introduction: Surgical resection of rhab- therapy role was extracted.
domyosarcoma (RMS) of abdomen and pelvis is associated with many Results: 17 single centre observational studies and 8 retrospective
challenges and RO resection is unachievable. We aim to identify the reviews of multicentre trials met criteria for final analysis. There were
associated surgical risk factors. 1028 patients identified, with a male predominance in their adoles-
Methods: A retrospective review of patients with RMS of the abdomen cent years. 5-year overall survival ranged from 35% to 89%. A review
and pelvis from 2010-2022 was conducted. Tumors originated from in 2003 established the role for neo-adjuvant chemotherapy before
bladder/prostate, genitalia and biliary tract were excluded. Clinical surgery with improved negative margins(77% vs 50%) and reduced
charts, imaging, surgery and pathology reports were analyzed. post-op radiotherapy requirement(48% vs 71%). There was high vari-
Results: Among 16 patients identified, mean age 5.2 (1.4-8.9) years ation in the degree of resection of surrounding tissue to obtain free
with 7 boys, the tumors were located in the abdomen (n=4), pelvis margins so the collaborative oncology group guidelines for resecting
(n=4) and abdomino-pelvis (n=8). Two in the latter group originated a normal rib above and below and 2-3cm margins along the rib were
from urachus. Except for a patient with alveolar RMS, the rest had not really followed. Some databases found similar 5 yr survival whether
embryonal RMS. Distant metastases were present in 5 patients. All partial or total rib resection was performed. If negative margins were
patients had large tumors at presentation and 5 ruptured before achieved then further radiotherapy was not shown to improve survival
chemotherapy commenced. All received pre-operative chemotherapy further. However if microscopic positive margins were present then
but 7 showed limited or no response. At surgery, we identified surgical additional radiotherapy could improve survival in some studies similar
risk factors associated with challenges to include vascular encase- to microscopic free margin resections.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S9 of S635

Conclusions: The review suggests that surgery should be included ern bespoke cancer therapy protocols is crucial to address the
as part of multimodality treatment for most patients, with the question.
current collaborative oncology group guidelines for surgical mar-
gins probably being too aggressive which may limit surgery being
applied for some patients. Macroscopic free margins are an abso- O020 / #1570 SURGICAL MANAGEMENT OF PEDIATRIC
lute, but microscopic positive margins can be compensated for THYROID NEOPLASMS. EXPERIENCE OF A NATIONAL
by radiotherapy, and neo-adjuvant chemotherapy is an absolute PEDIATRIC ONCO-SURGICAL REFERENCE CENTER
requirement.
Cristian Urla1 , Joerg Fuchs1 , Andreas Schmidt1 , Gerhard Binder2 ,
Helmut Dittmann3 , Martin Ebinger4 , Juergen Schaefer5 , Steven
O019 / #1632 DO CHILDREN WITH OSTEOSARCOMA Warmann1
REALLY BENEFIT FROM PULMONARY METASTECTOMY? - A 1 University Children’s Hospital Tuebingen, Pediatric Surgery And Pedi-
SYSTEMATIC REVIEW OF PUBLISHED STUDIES atric Urology, Tuebingen, Germany; 2 University Children’s Hospital Tue-
bingen, Pediatric Endocrinology, Tuebingen, Germany; 3 University Hospital
Tristan Boam1 , Bethan Rogoyski2 , Wajid Jawaid3 , Paul Losty4 Tuebingen, Nuclear Medicine And Clinical Molecular Imaging, Tübingen,
1 Nottingham Childrens Hospital, Paediatric Surgery, Nottingham, United Germany; 4 University Children’s Hospital Tuebingen, Hematology / Oncol-
Kingdom; 2 De Montfort University, Leicester School Of Allied Health ogy, Tübingen, Germany; 5 University Hospital Tuebingen, Diagnostic And
Sciences, Leicester, United Kingdom; 3 Addenbrookes Hospital, Paediatric Interventional Radiology, Tuebingen, Germany
Surgery, Cambridge, United Kingdom; 4 University of Liverpool, Institute Of
Life Course And Medical Sciences, Liverpool, United Kingdom Background and Aims: Thyroidectomy is rarely performed in pediatric
patients. The aim of the present study was to analyze our experi-
Background and Aims: Pulmonary metastectomy (PM) for resectable ence of thyroidectomy in children and identify factors associated with
oligometastatic Osteosarcoma (OS) is recommended as the standard of postoperative complications.
care in pediatric and adult cancer therapy protocols. Recent data from Methods: Between January 2009 and April 2022, 27 patients (21
the PulMiCC trial demonstrated no survival benefit from PM in Col- female, 6 male) with thyroid neoplasms were operated on at our
orectal Cancer. Paradigm shifts focusing on the quality of life costs of institution. The diagnostic workup consisted of ultrasound, thyroid
childhood cancer treatment mandates re-evaluation of the effects of scintigraphy, and/or magnetic resonance imaging (MRI). All patients
repeated ‘re-do’ thoracotomies. The aim of this study was therefore to were treated according to the guidelines of the Society of Pediatric
critically re-examine the evidence for a survival benefit of PM for OS in Hematology and Oncology (GPOH). The indication for surgery was
the pediatric population. established by a multidisciplinary tumor board. Surgery was performed
Methods: A comprehensive systematic review was undertaken accord- using a reduced Kocher incision. A retrospective review of patient’s
ing to PRISMA methodology guidelines. Medline and Embase were records was carried out.
searched for ALL studies detailing pediatric OS patients (<18 years) Results: The median age at operation was 14 years (6-16). The his-
undergoing PM with a comparison group that did not receive PM. Stud- tologies were: 13 papillary carcinoma, 9 follicular adenoma, 3 struma
ies where data was not distinguishable from included adult patients, nodosa, 2 cystic lesion. A hemithyroidectomy was performed in 11
those without a ‘no PM comparison group’, and those with < 4 patients cases, 16 patients underwent complete thyroidectomy (including neck-
were excluded. dissection in 4 cases). In 6/16 children completion thyroidectomy was
Results: The initial search yielded 944 abstracts from which 47 full performed as second-step after previous hemithyroidectomy until his-
papers were selected for screening. Twelve studies met the full inclu- tological confirmation of papillary carcinoma. Intraoperative recurrent
sion criteria dating from 1984 – 2017, detailing a total of 530 patients. laryngeal nerve monitoring was used in all cases. The resection status
All studies were retrospective, the majority being medium to large was R0 in 24 cases, R1 in 2 cases, and R2 in 1 case (infiltration of recur-
case series and no report directly compared PM vs no PM in pediatric rent laryngeal nerve). Median length of hospital stay was 4 days (3-16).
patients as its main study objectives. Overall a positive survival benefit Postoperative complications occurred in 3 patients (1 transient vocal
was declared in favour of PM. Patients not undergoing PM were usu- cord palsy; 1 hematoma which necessitated hemostasis; 1 hypocal-
ally those with unresectable disease and/or considered to have a poor cemia). The overall survival was 100% after a median follow-up of 48
prognosis. months (4-147).
Conclusions: These study findings cannot clearly demonstrate strong Conclusions: Surgical management of pediatric thyroid neoplasms can
evidence ‘for or against’ a survival benefit of PM for OS in pedi- be very complex. Treatment within interdisciplinary trial protocols is
atric patients. All studies detail outdated treatments protocols and essential. The use of intraoperative recurrent laryngeal nerve monitor-
are not designed to address the questions directly. The ostensi- ing is essential in reducing the incidence of postoperative vocal cord
ble survival benefit(s) of PM is likely due to selection bias of palsy. With sufficient surgical expertise, an excellent functional and
‘favourable cases’. A randomised controlled trial incorporating mod- oncological outcome can be obtained.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S10 of S635 ABSTRACTS

O021 / #398 IS DYNAMIC RISK STRATIFICATION OF Background and Aims: To report the epidemiological aspects, surgical
DIFFERENTIATED THYROID CARCINOMA (DTC) APPLICABLE IN challenges, and outcomes of children with benign retroperitoneal germ
PAEDIATRIC POPULATION? cell tumours (GCT) presenting to our centre.
Methods: A retrospective study was conducted, and all patients with
Ritesh Suthar1 , Sneha Shah2 , Sajid Qureshi2 , Girish Chinnaswamy2 , retroperitoneal GCTs, managed from January 1998 to January 2022
Maya Prasad2 , Badira Cheriyalinkal Parambil2 , Nilendu Purandare1 , were enrolled. Patients with increased alpha-fetoprotein (AFP) or with
Ameya Puranik1 , Archi Agrawal1 , Venkatesh Rangarajan1 malignant histology were excluded. The prospectively collected data of
1 Tata Memorial Hospital, Nuclear Medicine And Molecular Imaging, Mum- the included patients were analysed for patient demographics, presen-
bai, India; 2 Tata Memorial Centre, Paediatric Oncology, Mumbai, India tation, radiologic and histologic findings, surgical procedures, and the
challenges faced in tumour excision, postoperative complications, and
Background and Aims: Dynamic risk stratification(DRS) is re risk recurrence.
stratification of DTC based on initial treatment response into excellent Results: Of the total 130 cases of extracranial GCT that presented
response, biochemical residual disease, structural disease or indeter- to us, 26 (20%) had retroperitoneal tumours and formed the study
minate disease. This has proven to be useful in management of adult group. The median age was 10.5 months (10 days – 14.8 years) at pre-
patients however it lacks evidence in pediatric population. An audit was sentation. Of the total cohort, 21/26 (80%) patients presented with
done to evaluate application of DRS in pediatric patients treated at our a palpable abdominal mass; unusual presentations, such as polymen-
centre orrhagia (n=1) and hypertensive emergency (n=1), were also noted.
Methods: Retrospective single centre audit of pediatric DTC treated Bilateral hydronephrosis was present in 3/21 (14%) patients. All the
with total thyroidectomy followed by radioactive iodine (RAI) during patients underwent operative intervention, with 25/26 (96%) under-
2008-2018 were included. Patients were risk stratified immediately going complete tumour excision. Intraoperatively, 7/26 (27%) patients
post surgery as per ATA 2015 criteria and were restratified as per the had the tumour in close relationship with the aorta or inferior vena cava
DRS at completion of therapy. A follow up was done for outcomes. (4/26), duodenum (2/26) and diaphragmatic infiltration (1/26). These
Results: 63 patients were identified and risk stratified into 13 low risk, patients required fine intraoperative dissection, and, in a few cases,
14 intermediate and 36 high risk as per the ATA 2015 criteria. On DRS additional procedures such as bowel resection (1/26), diaphragmatic
all low and intermediate risk patients (including those who received < repair (1/26) and redo resection (1/26) were also required. In 4/26
100mCi) achieved excellent response and continued to be disease free (15%) of the patients, immature teratoma was present on histopathol-
at mean follow up of 7 years 26/36 high risk showed excellent response ogy. The median duration of follow-up was 24 months (1 month –
of which only 1 had a local relapse. 3/36 showed biochemical resid- 168 months), and one patient had a significant complication (adhesive
ual disease of which 1 had local relapsed and 6/36 showed structural obstruction). There were no recurrences reported in the cohort.
residual disease, of which 3 patient progressed. Rest of the patients on Conclusions: Retroperitoneal germ cell tumours have an excellent out-
follow up continued to be disease free. The mean follow up was 5.6 come if completely excised. The current study highlights the need for
years for high risk group. Mean doses of RAI for achieving excellent adequate preoperative evaluation and intraoperative visualization of
response in high risk patients with only nodal disease and with lung the vital structures to achieve complete excision in these patients.
metastases were 145mCi and 750mCi respectively.
Conclusions: Excellent response noted on DRS even in high risk pae-
diatric DTC and continued disease free (DF) on follow up, favouring its O023 / #570 LONG-TERM FUNCTIONAL OUTCOMES OF
use in management. This re stratification will prompt lesser diagnostic SACROCOCCYGEAL TERATOMA: A SYSTEMATIC REVIEW OF
and therapeutic interventions in children. Patients with nodal disease PUBLISHED STUDIES EXPLORING "REAL WORLD" OUTCOMES
can be treated with lower doses of radioactive iodine with excellent
response. Adeline Salim1 , Arimatias Raitio2 , Paul Losty3
1 Alder Hey Children’s NHS Foundation Trust, Paediatric Surgery, Liverpool,
United Kingdom; 2 University of Turku and Turku University Hospital, Pae-
O022 / #1007 BENIGN RETROPERITONEAL GERM CELL diatric Surgery, Turku, Finland; 3 University of Liverpool, Institute Of Life
TUMORS – SURGICAL CHALLENGES AND OUTCOMES Course And Medical Sciences, Liverpool, United Kingdom

Apoorv Singh1 , Vishesh Jain1 , Sandeep Agarwala1 , Sameer Bakhshi2 , Background and Aims: Sacrococcygeal teratoma (SCT) is a rare
Manisha Jana3 , Devasenathipathy Kandaswamy3 , Anjan Dhua1 , neoplasm affecting 1:35,000 newborns. Long-term impaired blad-
Devendra Yadav1 der/bowel function has been reported by mainly small observational
1 All India Institute of Medical Sciences, Department Of Paediatric Surgery, series. This study therefore comprehensively analyses ALL published
New Delhi, India; 2 All India Institue of Medical Sciences, Department Of studies to define the true long-term functional sequelae.
Medical Oncology, New Delhi, India; 3 All India Institute of Medical Sciences, Methods: Medline/Embase databases were searched according to
Department Of Radiodiagnosis, New Delhi, India PRISMA guidelines. Data were extracted following paper selection by
study authors.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S11 of S635

Results: Final analysis yielded 36 studies involving 1,116 patients native tissue ingrowth and complete implant resorption. Complica-
(854 female; 77%). Individual data were available in 14 studies (222 tions, functional and aesthetic results were obtained with review of
patients). SCT diagnosis was established antenatally (20%), during photography and axial imaging.
the neonatal period (39%), infancy (20%), and after the first year Results: Seven patients were included: 5 males and 2 females, median
of life (20%). Mean tumour size was 8.5cm (SD 4.6). According to age 4 years (range 0-10), median follow-up 8 years (range 0- 9).
the Altman classification, there were 298/845 (35%) type I, 252/845 Pathology included 4 Ewing sarcomas, 1 osteochondroma, 1 chon-
(30%) type II, 133/845 (16%) type III, and 128/845 (15%) type IV dromesenchymal hamartoma and 1 high grade spindle-cell sarcoma.
tumours. Most neoplasms were benign (640/858; 75%), 77/858 (9%) 6 patients remain disease free whilst 1 patient is deceased suffering
immature, and 141/858 (16%) malignant. The commonest associ- metastatic Ewing relapse (with local control persisting). Complications
ated congenital anomalies were anorectal malformations 42/90 (47%), included a seroma at 2 months in one patient, a sterile collection
spinal dysraphism 19/90 (21%), and renal tract/urogenital disorders at 8 months in another (both drained and resolved) and early post-
10/90 (11%). Abnormal bladder function was reported in 7/39 (18%) operative wound infection in one patient treated with antibiotics.
Altman type I, 23/61 (37.7%) type II, 11/34 (32.4%) type III, and 15/25 The reconstruction provided good initial thoracic integrity enabling
(60%) type IV cases (p=0.007). Such findings were also more com- early extubation. Long term cosmetic and functional results are sat-
monly recorded in immature/malignant vs benign tumours and in those isfactory with good chest wall shape and function. There were no
patients requiring re-operation(s); p=0.002 and p=0.01, respectively. post-operative restrictions on daily activities, including sport. Pitfalls
Soiling was documented in 19% and constipation in 26% patients include the low-level PET avidity of the prosthesis (when considering
with no apparent significant associations with individual tumour char- relapse surveillance) and the presence of mild scoliosis.
acteristics. Abnormal gait was more common in those with Altman Conclusions: Poly-L-lactide struts and Permacol® offer bioabsorbabil-
type I-II 2/14 lesions (14.3%) vs type III-IV tumours (5/6, 83.3%), ity and a physiologically advantageous method for reconstruction of
p=0.003. Additionally, 55 patients reported unsatisfactory cosmesis, the chest wall allowing adaptation and growth with good functional and
and surgical scar site revision was performed in 13/55 (24%). aesthetic long-term outcomes in the paediatric population.
Conclusions: Higher Altman stage, unfavourable histology, and re-
operation are associated with poorer functional outcome(s) most
notably bladder dysfunction. Multidisciplinary management from O025 / #546 FEASIBILITY OF BILATERAL SIMULTANEOUS
1st diagnosis of SCT is crucial to optimise outcomes across surgical MUSCLE-SPARING POSTEROLATERAL THORACOTOMIES FOR
specialities. CHILDREN WITH BILATERAL SYNCHRONOUS PULMONARY
METASTASIS

O024 / #1672 LONG-TERM OUTCOMES OF PAEDIATRIC Tomomasa Hiramatsu1 , Margaret Mutch1 , Jonathan Karpelowsky1,2,3
CHEST WALL RECONSTRUCTION USING BIOABSORBABLE 1 The Children’s Hospital at Westmead, Department Of Paediatric Surgery,
PROSTHETIC MATERIAL Westmead, Sydney, Australia; 2 Kids Research Institute, The Children’s Hos-
pital at Westmead, Children’s Cancer Research Unit, Westmead, Sydney,
Margaret Mutch1 , Tomomasa Hiramatsu1 , Michael Collin1 , Jonathan Australia; 3 The University of Sydney, Division Of Child And Adolescent
Karpelowsky1,2,3 Health, Sydney, Australia
1 The Children’s Hospital at Westmead, Department Of Paediatric Surgery,
Westmead, Sydney, Australia; 2 Kids Research Institute, The Children’s Hos- Background and Aims: There are several surgical strategies for
pital at Westmead, Children’s Cancer Research Unit, Westmead, Sydney, patients with synchronous pulmonary metastases undergoing resec-
Australia; 3 The University of Sydney, Division Of Child And Adolescent tion. This includes simultaneous bilateral or staged thoracotomies,
Health, Sydney, Australia median or transverse sternotomy. There are currently only a few pae-
diatric reports of simultaneous bilateral posterolateral thoracotomies.
Background and Aims: Paediatric chest wall tumours are rare. Chest The aim of the study was to evaluate the safety and feasibility of simul-
wall resection and reconstruction is challenging but essential to the taneous bilateral muscle-sparing posterolateral thoracotomies (BT) in
management. Challenges include unique characteristics related to paediatric patients compared with unilateral thoracotomy (UT).
growth in children and the variability of the post-surgical chest wall Methods: We conducted a retrospective review of all patients who
defects in size and anatomic location. Described techniques (using non- underwent thoracotomy for pulmonary malignancy at Children’s Hos-
absorbable prosthetics) have limitations. We aim to review long-term pital at Westmead between January 2010 and March 2021. Medical
outcomes when using bioabsorbable prosthetic materials. records were reviewed and patients’ demographics, types of pain man-
Methods: This retrospective series includes pre-pubertal children that agement, underlying diseases, operative time, days with intercostal
have undergone chest wall reconstruction at our institution. Restitu- catheter (ICC), length of stay after surgery (LOS), and perioperative
tion of musculoskeletal integrity and soft tissue coverage was achieved complications were analyzed.
using a combination of Poly-L-lactide struts, a porcine dermal collagen Results: 34 patients (24 males, 10 females, average age 11
patch and a combination of muscle/skin flaps where needed, allowing year) underwent 44 thoracotomies during the study period. All
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S12 of S635 ABSTRACTS

thoracotomies were muscle-sparing posterolateral thoracotomy. Four patients had been diagnosed with pGvHD before the onset of TALS,
patients underwent bilateral simultaneous thoracotomies. The most with a mean time lapse of 276 days (range 42 – 513). These patients
common underlying malignancy was Osteosarcoma (n= 15, 38%), fol- experienced on average 4.5 air leak episodes (range 3 – 6). All the
lowed by NRSTS (n= 6, 15%) and Ewing sarcoma (n=5, 13%). Epidural patients experienced at least two episodes before surgery. One patient
analgesia was used for pain management in 31 patients (78%) including underwent emergency tube thoracostomy only, three patients under-
all four patients who underwent bilateral thoracotomies. When we went thoracoscopic pleurodesis and two patients underwent thora-
compared outcomes for patients undergoing UT versus BT, median cotomy. After surgery, patients were free from air leak symptoms for
length of hospital stay was 5 days in UT and 8 days in BT (p<0.01), a mean time of 176 days (range 25 – 477). Pulmonary function pro-
and days with intrathoracic drain was 2 days in UT versus 3.5 days in gressively deteriorated, and all the patients eventually died because of
BT (p=0.06). Postoperative complications occurred in four patients respiratory failure after a mean time of 483 days (range 127 – 1045)
(pneumonia, prolonged air leakage, urinary retention, and Clostridium after the first episode of air leak.
difficile colitis) in UT versus one patient (prolonged air leakage) in BT Conclusions: Surgery provides temporary relief to symptoms related
(p=0.4). to TALS. When TALS develops, pulmonary function progressively
Conclusions: Simultaneous bilateral muscle-sparing thoracotomies worsens toward respiratory failure and death.
seem safe and feasible with acceptable postoperative outcomes.

CCI
O026 / #742 RECURRENT THORACIC AIR LEAK SYNDROME
IN PATIENTS AFFECTED BY PULMONARY GRAFT-VERSUS-HOST CCI: NOTHING ABOUT US WITHOUT US! ENGAGING PATIENTS
DISEASE: SURGICAL STRATEGIES AND OUTCOME AND PARENTS IN PAEDIATRIC CANCER RESEARCH & CARE
28-09-2022 5:30 PM - 6:30 PM
Giorgio Persano1 , Alessandro Crocoli2 , Cristina Martucci2 , Valerio
Pardi2 , Pier Luigi Di Paolo3 , Francesca Petreschi4 , Giulia Cafiero5 , O027 / #531 PARENTAL ROLE IN PEDIATRIC CANCER
Alessandro Inserra2 TREATMENT DECISION MAKING AT TIKUR ANBESSA
1 Bambino Gesù Children’s Hospital IRCCS, Surgical Oncology Unit – General SPECIALIZED HOSPITAL, ETHIOPIA: MIXED METHOD STUDY
And Thoracic Surgery Unit, Department Of Surgery, Rome, Italy; 2 Bambino
Gesù Children’s Hospital IRCCS, Rome, Italy, Surgical Oncology Unit – Daniel Wolde1 , Leul Kitawu2 , Nataliya Lindström3
General And Thoracic Surgery Unit, Department Of Surgery, Rome, Italy; 1 TikurAnbessa Specialized Hospital, College of Health Sciences, Addis
3 Bambino Gesù Children’s Hospital IRCCS, Rome, Italy, Radiology Unit, Ababa University, Pediatric Oncology, Addis Ababa, Ethiopia; 2 Addis Ababa
Department Of Diagnostic Imaging, Rome, Italy; 4 Bambino Gesù Children’s University, Nursing, Addis Ababa, Ethiopia; 3 University of Gothenburg,
Hospital, IRCCS, Italy, Bronchopneumology Unit, Academic Department Applied Information Technology Division Of Informatics, Göteborg, Sweden
Of Pediatrics, Rome, Italy; 5 Bambino Gesù Children’s Hospital IRCCS,
Rome, Italy, Sport And Hypertension Medicine Unit, Department Of Cardiac Background and Aims: TikurAnbessa Specialized Hospital (public),
Surgery, Cardiology, Heart And Lung Transplant, Rome, Italy Addis Ababa, Ethiopia (>120 million people) has 42 pediatric oncology
inpatient beds, and outpatient sees 150-200 children/week. Annu-
Background and Aims: Thoracic air leak syndrome (TALS) is a ally, >500 children with cancer are diagnosed late (advanced disease)
complication related to chronic pulmonary graft-versus-host disease and in pain. Aim: Explore parental role in pediatric cancer treatment
(pGvHD) that affects approximately 0.83% to 3.08% patients after allo- decision-making and identify influencing factors.
genic hematopoietic stem cell transplant. Such complication is defined Methods: A convergent mixed-method study from April-May 2020
as the occurrence of any form of air leak in the thorax, including spon- used Control Preference Scale for Pediatrics (CPS-P), Krantz Health
taneous pneumomediastinum or pneumopericardium, subcutaneous Opinion Survey (KHOS), and Trust in Physicians Scale (TPS) (interper-
emphysema, interstitial emphysema and pneumothorax and has a neg- sonal trust between the parent and health-care provider). In-depth
ative impact on post-transplant survival. The aim of the present study interviews provided qualitative data. Tools translated to Amharic
is to describe a single-center experience in the surgical management of and back-translated to English. Final tool reviewed by five pediatric
recurrent TALS in adolescents and young adults and its outcome. oncology nurses/doctors; necessary modifications made. Two data col-
Methods: The clinical notes of patients with previous allogenic lectors (nurses) trained. Eligibility: parent of a child with any cancer
hematopoietic stem cell transplant who underwent surgical proce- (<13 years) attending inpatient or outpatient unit and < 30 days
dures for recurrent TALS from January 2016 until March 2021 were post-diagnosis. IRB approved.
retrospectively reviewed. Clinical data, number of episodes of thoracic Results: A total of 167 parents (70 mothers and 97 fathers) completed
air leak, surgical procedures and outcome were analyzed. all tools. Parental role in treatment decision-making was passive 129
Results: In the examined period, four patients, aged 16 to 25 years, (77.2%), collaborative 37 (22.2%), and active 1 (0.6%). Most (82.6%)
underwent surgical procedures for TALS, including thoracostomy parents had a role they preferred. Interpersonal trust and parental
tube placement, thoracoscopic pleurodesis and thoracotomy. All the information preference were statistically significant predictors of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S13 of S635

passive role matched interview data. Parents seeking much informa- ing obstacles of implementing PPIE in research (social barriers, lack of
tion had an increased role in treatment decision-making. Parents with resources).
high trust in the healthcare team became less involved and relin- Conclusions: The results highlight the necessity of raising awareness
quished more decisions to health providers than parents who were on the “What and How” to implement PPIE in the research process:
suspicious about all aspects of their child’s care and maintained a high impart knowledge about the concept via specialist trainings, improve
decision-making role. communication and to develop measures that close the gap and meet
Conclusions: Preferred parental role in treatment decision-making the needs of both, HCP and patients, equally.
was passive and affected by parent trust in the health provider and
informational preference. Therefore, it is essential to improve
these relationships and interpersonal trust. Factors influenc- PROS
ing parent preferences included understanding of the health
provider role in the health system, communication, previous clin- PROS: FREE PAPER SESSION 1 29-09-2022 9:40 AM - 11:10 AM
ical experiences, educational level, level of satisfaction with care,
and preferred level of involvement, as documented in in-depth O029 / #843 IMPACT OF COVID-19 PANDEMIC ON
interviews. DELIVERY OF PEDIATRIC RADIOTHERAPY: A SYSTEMATIC
REVIEW

O028 / #1832 PATIENT AND PUBLIC INVOLVEMENT AND Naba Ali1 , Mithra Ghalibafian2 , Jeanette Parkes3 , Bilal Qureshi4 ,
ENGAGEMENT IN PEDIATRIC ONCOLOGY – THAT’S IS HOW IT Natia Esiashvili5
WORKS! 1 Emory University, Radiation Oncology, Atlanta, United States of America;
2 Mahak Pediatric Cancer Treatment and Research Center, Radiotherapy,
Liesa J. Weiler-Wichtl1 , Anita Kienesberger2 , Johannes Gojo1 , Rita Tehran, Iran; 3 University of Cape Town, Radiotherapy, Cape Town, South
Hansl1 , Maximilian Hopfgartner1 , Ulrike Leiss1 , Carina Schneider3 Africa; 4 The Aga Khan University, Radiotherapy, Karachi, Pakistan; 5 Emory
1 Medical University of Vienna, Comprehensive Center For Pedi- University, Department Of Radiation Oncology, Atlanta, United States of
atrics/department For Pediatrics And Adolescent Medicine, Vienna, America
Austria; 2 CCI-Europe, Chair, Vienna, Austria; 3 CCI-Europe, Managing
Director, Vienna, Austria Background and Aims: The COVID-19 pandemic has resulted in sig-
nificant disruptions in healthcare globally. Notably, it has prevented
Background and Aims: Patient and Public Involvement and Engage- the timely diagnosis and treatment of many diseases, including cancer.
ment (PPIE) in research, advocates for research conducted ‘with’ not Children with cancer are amongst the most vulnerable populations in
‘for’ the patients and their families (Polanco, 2021). Considering PPIE the world. Thus, the impact of COVID-19 on their treatment and out-
in the research process is associated with a higher patient-oriented comes warrants investigation. Radiotherapy is an integral component
outcome and meets patients’ needs best. Unfortunately, disparities in of cancer treatment in the pediatric population. As a resource heavy
PPIE activities and ambiguity of terminology across Europe is more therapy that stretches over weeks, we hypothesize that it has experi-
than evident. enced significant disruptions resulting from the COVID-19 pandemic.
Methods: In a multilevel and interdisciplinary approach (including We sought to review the published data on the impact of the COVID-19
patient representatives) all levels of PPIE (participation, engagement pandemic on the delivery of pediatric radiotherapy.
and involvement) were integrated (1) to investigate in a Europe-wide Methods: Literature published from January 2020 through Decem-
online survey the current knowledge about and attitude towards PPIE ber 2022 containing information regarding pediatric tumors and
among the HCP (n=134) and patients (n=168). (2) To develop effective COVID-19 were reviewed. The reports that comprised of information
ways to practice PPIE, a workshop was held with N=47 participants, regarding radiotherapy disruptions were selected and analyzed.
including dual moderation teams (HCP and patient expert). (3) The Results: Disruptions in radiotherapy were reported amongst inter-
outcome resulted in an awareness film. ruptions of other therapies (surgery, chemotherapy). There was not
Results: Generally, PPIE was classified as relevant: HCPs assume to enough data to link disruptions with infection surges however sev-
involve in many research areas (participation in Studies, communica- eral reports commented on disruptions occurring in the setting of
tion on data). However, this is not perceived to the same extent by quarantines. Disruptions were more common in low-income (78%)
patients (X2 = 42.70, p < .001). Although, there is willingness on both and low middle-income (68%) countries compared with upper mid-
sides to integrate PPIE, both HCP and patients indicate a low level of dle income (46%) and high income countries (10%). Several papers
knowledge (patients: t(334) = -2.817, p = .004; HCPs: t(270) = -2.883, were published with recommendations for mitigation strategies includ-
p = .004). Within HCP, incongruent perspective issues are mentioned ing implementation of COVID-19 testing and isolation protocols,
significantly more often: disease-related constraints in patients (X2 = utilization of staggering shifts, and telemedicine. Altered treatment
11.86, p < .001) and a lack of objectivity in patients (X2 = 25.52, p regimens were also common, including use of active surveillance
< .001). However, HCP and patients are fairly unanimous consider- for low grade tumors, systemic therapy to delay local therapies and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S14 of S635 ABSTRACTS

accelerated/hypofractionated dose delivery. The impact of mitigation O031 / #1059 OMITTING RADIATION IN YOUNG ADULT
measures on patient outcome was not reported. FEMALES WITH HODGKIN’S DISEASE, IS THERE AN IMPACT
Conclusions: COVID-19 has impacted radiotherapy delivery in the ON OUTCOME?
pediatric population globally. Countries with limited resources may
be affected more. Various mitigation strategies have been developed. Samah Semary1 , Emad Moussa2 , Mohamed Saad Zaghloul3 , Eman
Efficacy of mitigations measures warrants farther investigation. Khorshed4 , Mohamed Hamza5 , Salma Abdel Aziz6 , Madeha Elwakeel7 ,
Asmaa Hamouda2
1 clinical oncology, Beni-suef University, Children’s Cancer hospital Egypt
O030 / #667 TREATMENT OUTCOMES OF PAEDIATRIC 57357, Pediatric Oncology, cairo, Egypt; 2 Children’s Cancer hospital Egypt
CANCER PATIENTS REFERRED FOR RADIOTHERAPY IN 57357, Pediatric Oncology, Cairo, Egypt; 3 Children’s Cancer hospital Egypt
LIMITED-RESOURCE SETTINGS – EXPERIENCE AT THE UGANDA 57357/ National Cancer Institute - Cairo University, Radiotherapy, Cairo,
CANCER INSTITUTE Egypt; 4 Children’s Cancer hospital Egypt 57357/ National Cancer Institute
- Cairo University, Surgical Pathology, Cairo, Egypt; 5 Children Cancer Hospi-
Cissy Bangidde, Awusi Kavuma, Daniel Kanyike tal Egypt, Clinical Research, cairo, Egypt; 6 Children Cancer Hospital Egypt,
Uganda Cancer Institute, Radiotherapy, Kampala, Uganda Nuclear Medicine, cairo, Egypt; 7 Children’s Cancer hospital Egypt 57357/
National Cancer Institute - Cairo University, Radiology, Cairo, Egypt
Background and Aims: Cancer is one of the major causes of death
for paediatric patients worldwide and the incidences are increasing Background and Aims: Ionizing radiation is an established breast
with time. Most children die because of numerous social-economic fac- cancer risk factor [1]. This retrospective comparative study aimed
tors including poverty, few diagnostic health facilities, limited access to to compare between the outcome of young adult female diagnosed
education/information, scattered rural populations, scarcity of oncol- and treated as classic Hodgkin disease while omitting radiotherapy
ogy experts, limited treatment facilities, etc. The pattern of paediatric for fear of high risk of occurrence of breast cancer and those who
tumours in our resource-challenged centre is not well documented. received radiotherapy, to explore the impact of radiotherapy on the
The aim was to evaluate the distribution and treatment outcomes of outcome of this group of patients, and to assess the late side effect
paediatric patients referred for radiotherapy. of radiotherapy and the incidence of breast cancer in this group of
Methods: We retrospectively reviewed all the paediatric patient’s patients.
records/files with confirmed histological diagnoses, referred for radio- Methods: About 152 young adult female > or = 12 years old diagnosed
therapy, from January-2015 to December-2018. The study age was and treated as classic Hodgkin disease in Children Cancer Hospital
according to the American Academy of paediatrics which recommends Egypt during the period between July 2007 and end of December
people under paediatric care to be up to the age of 21 years; cate- 2018, the first group (58 patients) received chemotherapy while omit-
gorised into infancy (birth-2), childhood (2–12) and adolescence (12– ting radiotherapy, the second group (94 patients) received chemother-
21) years. Information retrieved from patients records/files included apy and radiotherapy. In both group we classify the patients according
age, sex, histological diagnosis, stage, pre-treatment received, ECOG to the initial stage, the presence of B symptoms, and response to
status, treatment intention, radiation dosages (fractionation/total treatment using interim PET CT scan post second cycle chemotherapy.
dose) and follow-up. Results: Overall survival affected by presentation with advanced stage,
Results: A total of 304(»8% of all patients) pediatrics, M: F ratio=1.2:1, and presence of B symptoms with significant P- value, 0.0059, 0.004,
were referred for radiotherapy. The distributions were infancy-(9.2%), respectively. Event free survival (EFS) affected by presentation with
childhood-(53.6%) and adolescence–(37.2%). The three most com- advanced stage, positive B symptoms, poor response detected by
mon tumours of infancy were Wilms(53.6%), retinoblastoma(17.9%) interim PET CT, and omitting radiotherapy, with significant P- value,
and sarcomas(14.3%); childhood were Wilms(34.4%), sarcomas(21.5%) 0.0059, 0.0001, 0.0022, 0.047, respectively.
and lymphomas(18.4%), while adolescents were sarcomas(25.7%), Conclusions: In conclusion, omitting radiotherapy in this group of
NPC(23.9%) and lymphomas(18.4%). Overall 65.8% of these patients patient affecting the 3 years EVS especially if having positive
presented in ECOG status 0–2 and only 12% presented with disease- B symptoms, advanced stage initially and had poor response to
stage I-II. Overall 61.3% were treated with radical intention and only treatment.
67.7% completed the prescribed doses. At 6 and 12 months of follow-
ups, 47.7% and 56.8% respectively have been confirmed dead or lost to
follow-up. O032 / #1594 RADIATION THERAPY IN PAEDIATRIC
Conclusions: Pediatric tumors are comprised a wide range of cancers, PATIENTS WITH HIGH-RISK NEUROBLASTOMA: A
accounting for ≈8% of all referrals. The results show that ≈90% of the RETROSPECTIVE STUDY OF 51 PATIENTS TREATED TO
patients present with locally advanced diseases and ≈40% are treated PRIMARY AND DISTANT SITES
palliatively, which impedes treatment outcomes.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S15 of S635

Enar Recalde1 , Monica Ramos Albiac2 , Soraya Mico Milla3 , Jordi Sciences, New Delhi, Otolaryngology, New Delhi, India; 5 All India Institue of
Giralt4 Medical Sciences, Medical Oncology, New Delhi, India
1 Hospital Vall d’Hebron, Radiation Oncology, Barcelona, Spain; 2 Hospital
Vall D’Hebron, Radiotherapy, Barcelona, Spain; 3 Hospital Vall D’Hebron, Background and Aims: Nasopharyngeal carcinoma(NPC) is a rare
Radiotherapy, barcelona, Spain; 4 Hospital Vall D’Hebron, Radiation Oncol- malignant tumour in childhood and adolescence(<1% of paediatric
ogy, Barcelona, Spain malignancies).
Methods: Data pertaining to paediatric and adolescent patients with
Background and Aims: Half of the patients diagnosed with high-risk NPC attending our institute from 2014-21 was abstracted by retro-
neuroblastoma (HRNB) die from disease progression despite intensive spective chart review.
multimodal treatment. Radiation Therapy (RT) has excellent local- Results: We identified 66 patients with NPC. The median
control (LR) rates of the primary tumour, but there is still controversy age at presentation was 18 years(range 8-27years). The
about consolidation of residual metastatic sites. We analyze character- male to female ratio was 43:23. On clinicoradiological exam-
istics and outcome of patients with stage IV HRNB at diagnosis who ination 1(1.5%),14(21.2%),11(16.7%),33(50%) and 7(10.6%)
received RT for local and metastatic locations if needed. patients had AJCC 2010 stage II,III,IVA,IVB and IVC tumours
Methods: We analyzed a retrospective cohort of 51 patients diagnosed respectively. Majority(63.6%) of the patients had undif-
with HRNB treated with RT in our hospital between 2011 and 2019 ferentiated NPC. On immunohistochemistry, 53/59(89.8%)
after receiving induction chemotherapy and surgical resection. The tumours stained positive for EBV-LMP1. 65 patients
primary sites were treated in all patients with 21Gy and 15 patients underwent neoadjuvant chemotherapy(median-3 cycles)-
who had 1-3 MIBG-avid metastatic locations after induction therapy PF(Cisplatin,5fluorouracil) in 19(29.2%), TP(Paclitaxel,Carboplatin)
received RT to those locations. in 31(47.7%), GC(Gemcitabine,Cisplatin) in 12(18.5%) and
Results: With a 4 year median follow-up, progression or relapse TPF(Docetaxel,Cisplatin,5fluorouracil) in 3(4.6%). Radiotherapy(RT)
occurred in 29 (59%) patients. Fifteen patients presented out of field to locoregional disease was delivered in 65(98.5%) patients, the
relapse. In field relapses (14 patients, 10 at the primary tumour site, techniques being 2D-conventional in 10, fixed-field IMRT in 5 and
4 at metastatic irradiated sites) occurred at a median time of 18.6 VMAT in 50 patients. The median RT dose was 65,60,54Gy/30 frac-
months (ED 8.7 months, CI95% 13.6-23.7 months) from diagnosis. tions/6 weeks(simultaneous integrated boost). Weekly concurrent
Patients who did not relapse at irradiated sites presented progres- Cisplatin(median-5 cycles) was added in 64(97%) patients. After
sion at 10.9 months (ED 15.6 months, CI95% 5.5-16.2 months) (p a median follow-up of 21 months(mean-27.3 months), 27(40.9%)
= 0.02). Relapse/progression rate was 8.58 per 100 patients (CI95% patients had disease progression(PD) and 14(21.2%) patients
5.24-13.26). Death occurred in 13 patients (26.5%), 7 of these (50%) died(12 due to PD). The common patterns of failure were in the
had in field relapse versus 6 (17%) who did not (p=0.03). Death rate was bones(22),lungs(10), distant lymph nodes(10), liver(4), locore-
5.1 per 100 patients (CI95% 2.71-8.72). No statistical differences were gional(3) and local(1). At last follow-up, 31(47%) and 8(12.1%)
found in local control attending to initial characteristics of patients, patients had complete and partial response, 2(3.03%) and 25(37.9%)
systemic induction, consolidation and surgical or maintenance treat- patients had stable and progressive disease respectively. The
ment. median overall survival(OS) and progression free survival(PFS)
Conclusions: RT seems to provide an adequate local control in both pri- had not been reached. The actuarial rates of OS and PFS were
mary and metastatic sites in patients with HRNB. Most progressions 82.6% and 56.4% at 2 years and 75% and 50.4% at 3 years
occur at distant locations which were not treated with RT. Relapses respectively.
at previously irradiated sites occur later than relapses at other Conclusions: Majority of paediatric and adolescent patients with NPC
sites. present in advanced stage in developing countries. Multimodality man-
agement (NACT followed by CRT) led to modest clinical outcome in our
setting. The most common pattern of failure was distant metastases
O033 / #1678 MULTIMODALITY MANAGEMENT OF in the bones. Attention needs to focussed on early detection of NPC,
NASOPHARYNGEAL CARCINOMA IN PAEDIATRIC AND prompt treatment(timely conformal RT) and overcoming barriers like
ADOLESCENT PATIENTS: UPDATED EXPERIENCE FROM A treatment abandonment.
REGIONAL CANCER CENTRE IN NORTH INDIA

Ahitagni Biswas1 , Swarnaditya Roy1 , Vivek Ghosh1 , Suman Bhasker1 , O034 / #1966 OUTCOMES IN PEDIATRIC NRSTS TREATED
Raja Pramanik2 , Ranjit Sahoo2 , Kapil Sikka3 , Atul Sharma2 , Alok WITH PROTON RADIOTHERAPY
Thakar4 , Sameer Bakhshi5
1 All India Institute of Medical Sciences New Delhi, Radiotherapy And Oncol- Matthew Johonson1 , Julie Bradley1 , Scott Bradfield2 , Christopher
ogy, New Delhi, India; 2 All India Institute of Medical Sciences New Delhi, Morris3 , Danny Indelicato1
Medical Oncology, New Delhi, India; 3 All India Institute of Medical Sciences 1 University of Florida, Radiation Oncology, Jacksonville, United States of
New Delhi, Otolaryngology, New Delhi, India; 4 All India Institute of Medical America; 2 Nemours Childrens, Oncology, Jacksonville, United States of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S16 of S635 ABSTRACTS

America; 3 University of Florida, College Of Medicine, Jacksonville, United 5 Tata Memorial Centre, Homi Bhabha National Institute, Bio Imaging,
States of America Mumbai, India; 6 Tata Memorial Centre, Homi Bhabha National Institute,
Radiodiagnosis, Mumbai, India
Background and Aims: Non-rhabdomyosarcoma soft tissue sarcomas
(NRSTS) are a heterogeneous group of tumors that are rare in chil- Background and Aims: To evaluate disease related outcomes, prog-
dren. Radiation therapy is often indicated in high-grade NRSTS and for nostic factors and toxicity in a prospective cohort of patients with
unresectable or recurrent tumors. The purpose of this study is to char- Askin-Rosai Tumors of the rib treated with multi- modality therapy in
acterize disease control in children with NRSTS tumors treated with a tertiary cancer care centre.
proton therapy (PT). Methods: Patients with primary lesions involving the rib or soft tis-
Methods: We conducted a retrospective review of a prospective reg- sues of the chest wall treated with Induction Chemotherapy (IC)
istry for children from 2007-2021 with non-metastatic NRSTS treated followed by Surgery and Post-Operative Radiotherapy (PORT) or
at a single institution. Collection of clinical and treatment records we Radiotherapy (RT) alone using Helical Tomotherapy (HT) based Inten-
obtained through chart review. Disease outcomes were determined sity Modulated Radiotherapy (IMRT) and maintenance chemotherapy
using Kaplan Meier curves. were included. Data was analyzed for disease related outcomes and
Results: 32 patients were identified. Median age was 12.4 years (range, toxicities.
0.2-21.9). The most common histologies included synovial sarcoma Results: Eighty patients between January 2008 and February 2018
(n=6), malignant peripheral nerve sheath tumor (n=4) and undif- were included. Sixty-eight (85%) were non-metastatic. Among non-
ferentiated sarcoma (n=5). Primary tumors were most commonly metastatic patients, 46 (67%) received Post-Operative RT (PORT-
located in the pelvis and thorax. Histology was high grade in 28 45-50.4Gy in 25-28 fractions) and 22 (33%) received definitive RT
patients. 2 patients had nodal involvement. Most patients received (55.8Gy/31 fractions). At a median follow-up of 42 months, the 5
post-operative PT (n=18), while 4 received pre-operative PT and 10 year estimates of OS, DFS and LC were 50%, 53% and 63% respec-
definitive PT. Median PT dose was 68.4 GyRBE at 1.8 GyRBE/fraction tively for the entire cohort. For patients with non-metastatic disease
(range, 41.4–75.6 GyRBE). Median follow-up was 5.1 years (range, (Median follow-up: 44 months), the 5 year OS, DFS and LC were
0.2-10.1). Five-year local control was 86% (95%CI: 70%-96%), over- 55%, 63% and 69.1% respectively. In patients with metastatic disease
all survival was 76% (95%CI: 58%-88%). and progression-free survival (Median follow-up: 44 months) the 5 year OS and LC were 47.6%
was 60% (95%CI: 41%-76%). Late toxicities included one each of: uri- and 49% respectively. On multivariate analysis, Maximal Standard-
nary incontinence following pelvic surgery and PT, chronic radiation ized Uptake Value (SUVmax ) on the pre-treatment FDG PET ≥ 8.2
cystitis, bone hypoplasia with chest wall asymmetry, limb-length dis- was associated with poorer OS (HR-2.767, 95%CI-1.131-6.768) among
crepancy, wound infection following preopPT and surgery, primary all non-metastatic patients and poorer DFS (HR-7.894, 95%CI-1.551-
hypothyroidism, slipped capital femoral epiphyses, chronic sinusitis, 40.178) in non-metastatic patients receiving PORT. There was one case
chronic otitis media, and keratitis. 2 patients had growth hormone defi- each of acute RTOGGrade 3 dermatitis and esophagitis.
ciency from cranial RT. Two of 17 patients developed bowel obstruction Conclusions: RT as an adjunct or in a definitive role in multimodality
requiring surgery. No patients developed a second malignancy. therapy of Askin-Rosai tumor of rib is associated with good LC, DFS and
Conclusions: PT for pediatric patients with NRSTS allows for con- OS with no significant increase in acute or late toxicity.
formal radiation delivery without marginal failure. Distant metas-
tases were the predominant pattern of disease progression. Improved
understanding of biologic features may assist in individualizing treat- O036 / #93 CLINICAL OUTCOME OF PEDIATRIC,
ment for these heterogeneous tumors.. ADOLESCENTS AND YOUNG ADULTS HEAD AND NECK
SARCOMA PATIENTS TREATED WITH PENCIL BEAM PROTON
THERAPY
O035 / #1028 ASKIN-ROSAI TUMOURS OF CHEST WALL
TREATED WITH MULTIMODALITY THERAPY: DOES Miriam Vázquez, Amaia Ilundain, Dominic Leiser, Damien Weber
RADIOTHERAPY IMPACT OUTCOMES? Paul Scherrer Institute, ETH Domain, Center For Proton Therapy, d,
Switzerland
Siddhartha Laskar1 , Shwetabh Sinha1 , Abhishek Chatterjee1 , Nehal‘
Khanna1 , Jifmi Manjali1 , Girish Chinnaswamy2 , Maya Prasad2 , Badira Background and Aims: To assess the clinical outcome of pediatric,
Parambil2 , Sajid Qureshi3 , George Karimundackal3 , Mukta adolescents and young adults (AYAs) patients with head and neck (HN)
Ramadwar4 , Sneha Shah5 , Akshay Baheti6 , Venkatesh Rangarajan5 sarcomas treated with pencil beam scanning proton therapy (pbsPT).
1 Tata Memorial Centre, Homi Bhabha National Institute, Radiation Oncol- Methods: Seventy-one patients treated between January 2001 and
ogy, Mumbai, India; 2 Tata Memorial Centre, Homi Bhabha National Insti- July 2021 were included. Median age was 9 years (0.3-37.6). Twenty-
tute, Pediatric Oncology, Mumbai, India; 3 Tata Memorial Centre, Homi one (29.6%) were AYA patients (15-39 years). Fifty-eight (81.7%)
Bhabha National Institute, Surgical Oncology, Mumbai, India; 4 Tata Memo- patients were treated according to a protocol. Median radiation dose
rial Centre, Homi Bhabha National Institute, Pathology, Mumbai, India; was 54 Gy (RBE) (36-73.8). Rhabdomyosarcoma (71.8%), and Ewing
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S17 of S635

sarcoma (15.5%) were the most prevalent diagnosis. Kaplan–Meier, ated with significant morbidity. After a median follow-up of 54 months,
log-rank test and Cox-regression were used for the analysis. the 5-year local control (LC), Disease free survival (DFS) and overall
Results: With a median follow-up of 37.2 months (range, 3.5 – 216.6), survival (OS) were 79% 74% and 72% respectively. At the time of last
12 (16.9%) patients died, one death was not related to the disease. follow up, 33(69%) patients were alive & disease free. Six patients had
Eight patients (11.2%) had local failure, 7 (9.8%) had distant failure local relapse only, 3 had distant metastases only, while 2 had both local
and 5 (7%) had both. Actuarial 3-year OS and LC were 85% and 80%, relapse & distant metastases. One patient died of chemotherapy toxic-
respectively. Use of concomitant chemotherapy showed an increased ity. On univariate analysis patients undergoing Sx+ RTh had similar LC,
likelihood of OS of 80% (HR 0.2, 95%CI: 0.05; 0.76) and 75% of DFS & OS compared to RTh alone. None of the prognostic factors were
LC (HR 0.25, 95%CI: 0.07; 0.92). Progressive disease after induction statistically significant.
chemotherapy was associated with worse OS (HR 39,61, 95%CI: 4.27; Conclusions: Multimodality treatment using a combination of CTh, Sx
367,09). Thirteen (18.3%) patients presented acute toxicity grade 3. & RTh results in optimal disease control with acceptable toxicities.
Four (5.6%) patients presented grade 3 late toxicity: cataract, sinusitis, Radiotherapy alone gives outcome similar to combined surgery and
otitis media and hearing impairment. No acute or late grade 4-5 toxicity adjuvant radiotherapy.
was observed.
Conclusions: Excellent clinical outcomes were observed for chil-
dren and AYAs with HN sarcoma treated with pbsPT. Concomitant IPSO
chemotherapy and progressive disease after induction chemotherapy
were major prognostic factors for outcome in these patients. IPSO FPS 3 THE ROBERT ARCECI BEST OF IPSO 29-09-2022
9:40 AM - 11:10 AM

O037 / #179 EWINGS SARCOMA OF THE HEAD AND NECK O038 / #130 MULTIPLEX ORGANOID-BASED 3D LIVE
REGION: LONG TERM OUTCOMES OF PATIENTS TREATED IMAGING PLATFORM TO SCREEN PROBES FOR
WITH A HOMOGENOUS TREATMENT PROTOCOL FLUORESCENCE GUIDED SURGERY

Nehal‘ Khanna1 , Anuradha Krishnan1 , Jifmi Manjali1 , Badira Bernadette Jeremiasse1,2 , Veerle Bok2 , Michiel Kleijnnijenhuis2 ,
Cheriyalinkal Parambil2 , Girish Chinnaswamy2 , Maya Prasad2 , Kumar Ravian Van Ineveld2 , Hannah Johnson2 , Amber Zeeman2 , Marc
Prabhash2 , Sajid Qureshi2 , Gouri Pantavaidya2 , Devendra Chaukar2 , Wijnen1 , Anne Rios2
Prathamesh Pai2 , Mukta Ramadwar2 , Poonam Panjwani3 , Sneha 1 Princess Maxima Center, Surgery, Utrecht, Netherlands; 2 Princess Maxima

Shah2 , Siddhartha Laskar2 Center, Imaging Center, Utrecht, Netherlands


1 Tata Memorial Centre, Radiation Oncology, Mumbai, India; 2 Tata Memo-
rial Centre, Paediatric Oncology, Mumbai, India; 3 Tata Memorial Centre, Background and Aims: Achieving complete tumor resections without
Pathology, Mumbai, India major complications remains challenging. Fluorescence guided surgery
(FGS) with tumor-specific probes can visually assist the surgeon in
Background and Aims: To evaluate disease profile, treatment discriminating tumorous from healthy tissue. However, tumor-specific
response, long term outcomes, patterns of failure and prognostic probes are currently screened one-by-one and per tumor type. Pre-
factors for patients of non-metastatic Ewings sarcoma (ES) of the head dictive platforms to develop FGS probes in a tumor-specific and even
and neck region treated with curative intent with a homogeneous patient-specific way are lacking. Here, we combine patient-derived
treatment protocol. organoids with 3D imaging technology to present a screening platform
Methods: From January 2010 to December 2019, patients with histo- for FGS probes.
logically proven non metastatic ES were retrospectively evaluated for Methods: Both an adult and pediatric tumor organoid biobank were
outcomes. Prognostic factors like age at diagnosis, sex, skeletal/ extra- cultured, respectively breast cancer and neuroblastoma. Surface mark-
skeletal origin, primary site (Paranasal Sinus/Mandibular/others), ers that are upregulated in neuroblastoma and/or breast cancer were
tumor size, hematological & biochemical parameters, response identified based on RNA profiling, the human protein atlas and litera-
to chemotherapy (CTh) and type of local treatment were ture review. Probes targeting these surface markers were conjugated
evaluated. to six different fluorophores ranging between 488nm and 647nm.
Results: Out of total 48 (age 1year to 41years;mean 17years), 27(56%) Together with the general marker efluor, they were used for seven-
were males, 32 (67%) patients had skeletal origin disease, 6(12.5%) color 3D multispectral live imaging using confocal microscopy on
had primary in paranasal sinus and 11 (23%) in mandible; with a mean living organoids. Using segmentation analysis by parallelization of 3D
tumor size of 6.5 cms. All patients received multimodal treatment in datasets (STAPL-3D) organoids were segmented and their fluorescent
the form of EFT 2001 systemic CTh and local treatment comprising of signals of the respective probes quantified. This high-throughput in
Surgery (Sx) alone 5(10%), Sx + adjuvant radiotherapy (RTh) 19(40%) vitro screening was validated by testing the three most promising FGS
or 24(50%) RTh alone. RTh as definitive local treatment was offered probes for neuroblastoma in vivo using a mouse xenograft model with
in cases where Sx was either not feasible or was deemed to be associ- tumors originating from neuroblastoma organoids.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S18 of S635 ABSTRACTS

Results: Our platform was able to simultaneously screen six different these strategies should be studied. The purpose of this study was to
probes on two full organoid biobanks. Segmentation and quantifica- determine the impact on disease outcomes with ultrasound surveil-
tion using STAPL-3D showed heterogeneous expression of markers on lance (US) of involved nodal basins versus completion lymph node
the organoid lines. The three most promising probes for neuroblas- dissection (CLND) in children and adolescents with sentinel lymph
toma (GD2, L1cam and NCAM-1) were tested in vivo and all resulted node (SLN) positive melanoma.
in tumor-to-background ratios above 2.0, indicating good intraopera- Methods: Patients from 12 participating Pediatric Surgical Oncol-
tive visibility. For heterogenous tumors that are not covered with one ogy Research Collaborative hospitals were included. Requirements
probe, this platform offers the chance to screen most potential probe included age <18 years and cutaneous melanoma diagnosed between
combinations. 2010-2020. Data extracted included demographics, histopathology,
Conclusions: We present a novel multiplex organoid-based 3D live surgical strategies including SLN and CLND, surveillance intervals, and
imaging platform to screen FGS probes in a patient-specific man- survival information.
ner with the potential to develop personalized FGS probes or probe Results: 249 patients were included, 52.2% (n=130) female, 90%
combinations. (n=223) non-Hispanic White. 90.8% (n=226) underwent SLN biopsy,
50% (n=113) had at least 1 positive node. Median number of pos-
itive nodes was 1 (IQR: 1-2) and did not differ between US and
O039 / #898 COMPARISON OF OUTCOMES BETWEEN CLND groups (p=0.20). Median Breslow depth was 2.5mm (IQR:
SURVEILLANCE ULTRASOUND AND COMPLETION LYMPH 1.4-4.1) and did not differ between US and CLND groups (p=0.26).
NODE DISSECTION IN CHILDREN AND ADOLESCENTS WITH 59.3% (n=67) with positive SLN underwent CLND while 40.7% (n=46)
SENTINEL LYMPH NODE POSITIVE CUTANEOUS MELANOMA underwent US surveillance of the nodal basin. Younger patients were
more likely to undergo US surveillance (median age 96 months) than
Steven Scoville1 , Joseph Stanek2 , Hafeez Abdelhafeez3 , Pattamon CLND (median age 142 months) (p=0.002). Of those who under-
Sutthatam3 , Lindsay Talbot4 , Dimitra Lotakis5 , Natalie Lopyan5 , Peter went CLND, 21% (n=14) had additional positive nodes removed.
Ehrlich5 , Stephanie Chen6 , Eugene Kim6 , Harold Leraas7 , M. Elisabeth Overall, 11.3% (n=25) experienced disease recurrence: 7 primary,
Tracy7 , Hannah Rinehardt8 , Marcus Malek8 , Juan Favela9 , Hau Le9 , 7 nodal, 10 distant, 1 unknown. There was no difference in dis-
Claire Wilson10 , Jacob Davidson10 , Natashia Seemann10 , Yasmin ease recurrence (11.4% vs 25%, p=0.08) or death from disease
Osman11 , Nelson Piche11 , Yu Lee12 , Akshitha Balagani12 , Stephanie (2.3% vs 9.7%, p=0.29) for those who underwent US vs CLND,
Polites13 , Robin Petroze14 , Victoria Hoang14 , Roshni Dasgupta12 , respectively.
Jennifer Aldrink1 Conclusions: Children and adolescents with cutaneous melanoma fre-
1 Nationwide Children’s Hospital, Pediatric Surgery, Columbus, United quently have nodal metastases identified by SLN. Equivalent disease
States of America; 2 Nationwide Children’s Hospital, Division Of Hema- outcomes were observed with US surveillance and CLND following
tology, Oncology And Bone Marrow Transplantation, Columbus, United identification of a positive SLN.
States of America; 3 St Jude Children’s Research Hospital, Global Pediatric
Medicine, Memphis, United States of America; 4 St. Jude Children’s Research
Hospital, Pediatric Surgery, Memphis, United States of America; 5 University O040 / #1278 EARLY COMPUTED TOMOGRAPHY OF
of Michigan, C.S. Mott Children’s Hospital, Pediatric Surgery, Ann Arbor, TUMOUR BED AFTER GROSS TOTAL RESECTION OF
United States of America; 6 Children’s Hospital of Los Angeles, Pediatric NEUROBLASTOMA WITH IMAGE DEFINED RISK FACTORS TO
Surgery, Los Angeles, United States of America; 7 Duke University Medical OBJECTIVELY ASSESS QUALITY OF RESECTION
Center, Pediatric Surgery, Durham, United States of America; 8 UPMC Chil-
dren’s Hospital of Pittsburgh, Division Of Pediatric General And Thoracic Anna Wojtylko1,2 , Jan Godzinski2 , Dawid Kulda1 , Malgorzata Rapala1
Surgery, Pittsburgh, United States of America; 9 American Family Chil- 1 Marciniak Hospital in Wrocław, Department Of Pediatric Surgery,
dren’s Hospital, University of Wisconsin, Pediatric Surgery, Madison, United Wrocław, Poland; 2 Medical University of Wroclaw, Department Of Paedi-
States of America; 10 Children’s Hospital at London Health Sciences Cen- atric Traumatology And Emergency Medicine, Wroclaw, Poland
tre, Pediatric Surgery, London, Canada; 11 Centre Hospitalier Universitaire
Sainte-Justine, Pediatric Surgery, Montreal, Canada; 12 Cincinnati Children’s Background and Aims: The treatment of neuroblastoma (NBL) pre-
Hospital Medical Center, University of Cincinnati, Division Of Pediatric Gen- senting image defined risk factors (IDRF) pose significant surgical
eral And Thoracic Surgery, Cincinnati, United States of America; 13 Mayo difficulties. Having known that a complete microscopic resection is
Clinic, Pediatric Surgery, Rochester, United States of America; 14 University unlikely in case of IDRF+, gross total resection (GTR) offers a chance
of Florida, Pediatric Surgery, Gainseville, United States of America for better prognosis. A reliable assessment of the residue after GTR
is of utmost importance. Theoretically, ECT before the healing cas-
Background and Aims: Treatment strategies involving nodal basins for cade developed prevents misinterpreting of a regenerative and healing
children and adolescents with melanoma are extrapolated from adult process as a tumour remnant and vice versa. The aim of the study was
trials. There is increasing evidence that important clinical and biologi- to evaluate early computed tomography of tumour bed (ECT) as a tool
cal differences exist between pediatric and adult melanoma, therefore to objectify the quality of GTR.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S19 of S635

Methods: ECT was performed by the postoperative day 7 on stabilised Methods: This study included patients aged up to 25 years with a
patients, before any major regenerative reactions and possibility of an pathological diagnosis of UESL prospectively enrolled from 1995 to
early relapse. Only children in whom GTR was subjectively assessed by 2016 in the SIOP-MMT95, AIEOPX and NRSTS05 trials. The recom-
the surgeon to be > 90% complete, were included. The study involved mended treatment included conservative surgery at diagnosis or a
61 children (31 local, 30 referred from other centres) who underwent biopsy followed by chemotherapy and delayed surgery. The decision to
GTR surgery for IDRF+ neuroblastoma (2018 – 2022). Images of ECT use RT was left to the treating center.
were segmented non-automatically, the boundaries of each tumour Results: Sixty-five patients met the study criteria with a median age
were marked manually, taking a layer thickness of 2.5 mm as the opti- at diagnosis of 8.7 years (0.6-20.8). Fifteen patients had T2 tumors,
mum. The total volumes of the primary masses and the post-operative and one had lymph node spread. Stage distribution included IRS I (14),
residues were calculated and compared in rates and ml of remnants. II (9), III (38), and IV (4). Upfront surgery (28) resulted in 5 oper-
Results: The post-surgical, objectively measured remnants of tumours ative spillages and 11 infiltrated surgical margins, whereas delayed
(ECT) ranged from 0 ml to 3,6 ml, completeness of GTR from 76,3% to surgery (37) resulted in no spillages (P= 0.0119) and 3 infiltrated
100% with median 91,8. margins(P=0.0114). All patients received post-operative chemother-
Conclusions: The subjective assessment of completeness of GTR by apy, including anthracyclines in 47. Radiotherapy was administered
surgeons seems to offer an over optimistic view which may not find in 15 patients. With a median follow-up of 78.6 months, 5 year OS
confirmation at objective assessment by ECT. Calculating the rate of and EFS were 90.1% (95%CI 79.2-95.5) and 89.1% (95%CI 78.4-
resection and measuring volume of the post surgical residue describe 94.6), respectively. Despite 3 out of 4 local relapses (3 others were
the postoperative situation in different ways, thus should be used and metastatic) being associated with infiltrated surgical margins, we did
analysed together. Authors emphasize on early assessment as that not find evidence of an association between infiltrated margins and
which shows direct post operative situation. EFS (P=0.1607) . Similarly, T2 stage (P=0.3870), use of RT (P= 0.8731),
and anthracycline-based chemotherapy (P= 0.1181), did not have an
association with EFS.
O041 / #1319 OUTCOME OF PATIENTS WITH Conclusions: Neoadjuvant chemotherapy with an alkylating agent reg-
UNDIFFERENTIATED EMBRYONAL SARCOMA OF THE LIVER imen for pediatric patients with UESL increases the probability of
TREATED ACCORDING TO EUROPEAN SOFT TISSUE SARCOMA complete surgical resection and decreases tumor spillage. The role of
PROTOCOLS anthracyclines and radiotherapy for localized disease remains unclear
and could be reserved for patients with infiltrated margins,tumor
Florent Guerin1 , Hélène Martelli1 , Timothy Rogers2 , Ilaria Zanetti3 , rupture, locoregional disease or distant extension.
Cecilia Terwisscha-Van Scheltinga4 , Federica De Corti5 , Gabriela
Guillén6 , Veronique Minard-Colin7 , Daniel Orbach8 , Max M. Van
Noesel9 , Hans Merks9 , Andrea Ferrari10 , Gianni Bisogno11 O042 / #1407 EXTRACELLULAR VESICLES – ISOLATION
1 AP-HP Paris-Saclay, Paediatric Surgery, Le Kremlin Bicêtre, France; TECHNIQUES AND THE USE OF BIOMARKERS IN
2 University Hospitals Bristol and Weston NHS foundation trust, Depart- RHABDOMYOSARCOMA AND NEUROBLASTOMA PATIENTS
ment Of Pediatric Surgery, Bristol, United Kingdom; 3 Padova University
Hospital, Haematology Oncology Division, Department Of Women’s And Evi Schmid, Lena Schultheis, Paula Batt, Johannes Stückle, Hanna
Children’s Health, Padova, Italy; 4 Princess Maxima Center, Surgery, Utrecht, Rajab, Benjamin Mayer, Joerg Fuchs, Steven Warmann
Netherlands; 5 Women and Children’s Health Department, University Hos- University Children’s Hospital, Department Of Pediatric Surgery And Pedi-
pital of Padua, Paediatric Surgery, PADUA, Italy; 6 Hospital Infantil Uni- atric Urology, Tuebingen, Germany
versitari Vall d’Hebron, Pediatric Surgery Department, Barcelona, Spain;
7 Gustave Roussy Institute, Département De Cancérologie De L’enfant Et Background and Aims: Extracellular vesicles (EVs) are spherical, lipid
L’adolescent, Villejuif, France; 8 Institut Curie, Siredo Oncology Department, bilayer membrane vesicles, which are released by various cell types.
Paris, France; 9 Princess Máaxima Center for Pediatric Oncology, Oncology, They are found in distinct biofluids such as blood and urine. Although
Utrecht, Netherlands; 10 Fondazione IRCCS Istituto Nazionale dei Tumori, there are many known isolation techniques, there is currently no con-
Pediatric Oncology Unit, Milano, Italy; 11 Pediatric Hematology, Oncology sensus for the optimal EV isolation method with respect to yield,
and Stem Cell Transplant Division., Department Of Women’s And Children’s quality, and purity. Furthermore, no data from pediatric solid tumor
Health University Hospital Of Padova, Padua, Italy patients are available. In this study, we evaluated different EV isola-
tion techniques and studied the application of EV as diagnostic tool in
Background and Aims: To assess the outcomes of pediatric patients children suffering from rhabdomyosarcoma (RMS) and neuroblastoma
with Undifferentiated Embryonal Sarcoma of the Liver (UESL) (NB).
treated according to successive European malignant mesenchymal Methods: Four different isolation techniques (precipitation, size-
tumors trials focusing on the effects of surgical margins, preoper- exclusion, membrane affinity, and ultracentrifugation), were compared
ative chemotherapy, use of radiotherapy (RT) and chemotherapy for EV isolation from plasma of healthy individuals (n=10). The most
regimen. efficient technique was then used to analyze plasma samples from
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S20 of S635 ABSTRACTS

RMS (n=40) and NB (n=41) tumor patients. EV characterization tal„ Department Of Paediatric Research And Department Of Paediatric And
included morphological analysis via electron-microscopy, determina- Adolescent Medicine, Oslo, Norway; 15 Padova University Hospital, Pedi-
tion of particle-size and concentration via nanoparticle tracking anal- atric Surgery Unit, Department Of Women’s And Children’s Health, Padova,
ysis. EV preparation quality, purity, and biomarker expression (GD2, Italy; 16 University of Padua, Hematology/oncology Division, Department Of
CD146, CD171) was assessed using flow cytometry and Western Women’s And Children’s Health, Padua, Italy
Blotting.
Results: All EV isolation techniques were successfully tested. They Background and Aims: Rhabdomyosarcoma of the perianal/perineal
showed approximately the same particle-size (115-147 nm) with a sim- region (PRMS) is rare, with poor survival and limited understanding of
ilar time requirement. The precipitation and membrane affinity meth- the functional consequences of treatment.
ods showed the highest protein purity. The size-exclusion method had Methods: SIOP MMT 95, Italian RMS 96, and EpSSG RMS 2005 stud-
the highest values for the exosome markers CD9 (99%), CD63 (82%), ies were interrogated to identify factors that impact survival; in RMS
and CD81 (97%) and showed higher purity on electron-microscopy. 2005, functional outcomes were analyzed.
The tested biomarkers (CD146 and CD171) allowed to distinguish Results: Fifty patients (non-metastatic) were identified, median age
between NB patients and healthy individuals, which was not the case 6.4 years (range 0.1-19.6): 29 male, 21 female. Tumors were >5cm
in RMS patients. in 33 patients. Histopathological subtype was alveolar in 35. Lymph
Conclusions: EVs seem to be a promising tool for non-invasive diag- nodes were involved in 23 patients. In RMS 2005, 16/21(76%) tested
nosis and treatment monitoring in children suffering from NB. Further alveolar tumors had positive FOXO1-fusion status. Diagnostic biopsy
studies on thhis and other tumor etiologies including determination of was performed in 37. Primary resection(13) was complete (R0) in 1.
expression patterns are needed in order to clarify the possible role of Delayed primary excision(16) was complete in 3. Radiotherapy (RT)
applying EVs in the clinical setting. in 34/50 patients included external beam(28), brachytherapy(3), and
both(3). Nodal RT was given in 16/23 N1 patients(70%). Median follow
up of alive patients (29) was 84.1 months (range 3.6-221.1). Relapse
O043 / #1573 PERIANAL/PERINEAL or progression occurred in 24 patients (48%), 87% were fatal and
RHABDOMYOSARCOMA: RESULTS OF THE SIOP MMT 95, most events (63%) were locoregional. 5-yr EFS was 47.8 (95%CI 32.8-
ITALIAN RMS 96, AND EPSSG RMS 2005 STUDIES 61.3), and 5-yr OS was 52.6 (95%CI 36.7-66.2), with age ≥ 10 years
and tumor size > 5cm impacting 5-year EFS and OS (p<0.05). Func-
Timothy Rogers1 , Ilaria Zanetti2 , Beatrice Coppadora3 , Hélène tional outcome data showed bowel, genito-urinary and psychological
Martelli4 , Meriel Jenney5 , Veronique Minard-Colin6 , Sheila issues; fecal incontinence in 4/21 survivors, and urinary symptoms in
Terwisscha Van Scheltinga7 , Clare Skerritt8 , Raquel Dávila Fajardo9 , 2/21.
Florent Guerin4 , Anna Kelsey10 , Hans Merks11 , Henry Mandeville12 , Conclusions: About 60% of patients with non-metastatic PRMS sur-
Gabriela Burrieza13 , Heidi Glosli14 , Federica De Corti15 , Gianni vive; older patients and those with large tumors have the worst out-
Bisogno16 comes. Biopsy should be the initial procedure and definitive local ther-
1 University Hospitals Bristol and Weston NHS foundation trust, Depart- apy individualized. Quality-of-life and functional studies are needed to
ment Of Pediatric Surgery, Bristol, United Kingdom; 2 Padova University better understand the consequences of treatment.
Hospital, Haematology Oncology Division, Department Of Women’s And
Children’s Health, Padova, Italy; 3 Hematology/Oncology Division, Depart-
ment of Women’s and Children’s Health, Department Of Women’s And O044 / #1628 REDO NEPHRON-SPARING SURGERY IN
Children’s Health, University Of Padua, Padova, Italy; 4 University Paris- STAGE V PEDIATRIC RENAL TUMORS
Saclay, Bicêtre Hospital, Department Of Pediatric Surgery, Paris, France;
5 University hospital of Wales, Department Of Pediatric Oncology, Cardiff, Joerg Fuchs1 , Matthias Schunn1 , Juergen Schaefer2 , Martin Ebinger3 ,
United Kingdom; 6 Gustave Roussy, Department Of Pediatric And Adoles- Steven Warmann1
cent Oncology, Paris, France; 7 Princess Máxima Center for Pediatric Oncol- 1 University Children’s Hospital, Department Of Pediatric Surgery And
ogy, Princess Máxima Center For Pediatric Oncology, Utrecht, Netherlands; Pediatric Urology, Tuebingen, Germany; 2 University Children’s Hospital,
8 University Hospitals Bristol and Weston NHS Foundation Trust, Depart- Department Of Interventional And Diagnostic Radiology, Tuebingen, Ger-
ment Of Pediatric Surgery, Bristol, United Kingdom; 9 University Medical many; 3 University Children’s Hospital, Department Of Pediatric Oncology,
Center Utrecht, Department Of Radiation Oncology, Utrecht, Netherlands; Tuebingen, Germany
10 Central Manchester University Hospitals, Department Of Pathology,
Manchester, United Kingdom; 11 Prinses Máxima Centrum voor Kinderon- Background and Aims: Nephron-sparing Surgery (NSS) is the surgi-
cologie, Pediatric Oncology, Utrecht, Netherlands; 12 The Royal Marsden cal treatment of choice in children with stage V renal tumors. With
Hospital and the Institute of Cancer Research, The Royal Marsden Hospital increasing numbers of this approach, there is also an increase of
And The Institute Of Cancer Research, Sutton, United Kingdom; 13 Hospital tumor relapses in affected children. Aim of this study was to evaluate
Infantil Universitari Vall d’Hebron, Surgical Oncology And Neonatal Surgery, Redo-NSS in children with stage V disease, especially for centrally
Pediatric Surgery Department„ Barcelona, Spain; 14 Oslo University Hospi- located tumor relapses.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S21 of S635

Methods: We retrospectively analysed patients undergoing Redo-NSS c) intramedullary component <50% d) small extramedullary extension
for relapsed kidney tumors between 2009 and 2021 at our institution, e) patient compliance.
which represents a national reference center within the SIOP-2001 Results: Between July 1981 and December 2020 of 398 patients
and UMBRELLA study. The indication for Redo-NSS was established by enrolled, 368 patients underwent LSS (95%). Primary site was femur
a national and local multi-disciplinary tumorboard (MDT). in 171 (46%). Median age was 10.8 years (range, 3-25). Range extent
Results: During the observation period, 45 patients with bilateral dis- of resection was 7-35 cm. Five (1.3%) patients had a pathological frac-
ease underwent primary NSS (69 renal units). In the same period, ture. Intraoperative complications included: a) hemorrhage defined
9 stage V patients receiver Redo-NSS: 5 girls, 4 boys, mean age at as >10% blood volumen loss (25%), vascular injuries requiring repair
surgery: 58 months (12-137). Mean time between primary NSS and (2%), peroneal or radial postoperative neuropathy (6.7%). All recovered
Redo-NSS was 25 months (4-104). All patients had only local recur- within 4 months. Postoperative complications included: a) superficial
rences. Mean operative time for Redo-NSS was 195 minutes (137-260). wound infections (12%) b) implant removal due to refractory infection
R0 resection status was achieved in all children (intraoperative frozen (2.4%) c) postoperative bleeding (2%) d) stem fracture (3.5%) e) implant
sections). Histology after Redo-NSS showed diffuse anaplasia in 2 loosening (4.3%) f) non-union allograft (0.8%) g) local recurrence
patients, which initially had low or intermediate risk tumors. These two (1.3%).
patients died in the further course from combined second relapses. Conclusions: LSS is feasible in >95% of patients. Complications are
Two other patients had second relapses, one of them resected via mild and minimal. Patient satisfaction paramount importance. Early
NSS, the other child underwent tumor nephrectomy plus atypical liver ambulation/rehabilitation are important facets. Major disadvantage
resection. Thus 7/9 patients are alive without evidence of disease, an cited are repeated surgeries for growth or complications (fracture,
impaired renal function was observed in one child. Mean follow-up was loosening, etc).
16 months (0-32).
Conclusions: With sufficient expertise of the interdisciplinary treat-
ing team, Redo-NSS for stage V renal tumors can be performed FREE PAPER SESSION (FPS)
with satisfactory oncological and functional results. The occurrence
of unfavourable tumor histology possibly represents a negative prog- FPS 01: LYMPHOMA 29-09-2022 9:40 AM - 11:10 AM
nostic factor. Among other factors, this needs further evaluation in
multicenter analyses. O046 / #538 KEYNOTE-667: PHASE 2 STUDY OF
PEMBROLIZUMAB IN CHILDREN AND YOUNG ADULTS WITH
NEWLY DIAGNOSED CLASSICAL HODGKIN LYMPHOMA WITH
O045 / #1689 PRIMARY EXTREMITY BONE SLOW EARLY RESPONSE TO FRONTLINE CHEMOTHERAPY
OSTEOSARCOMA. EVOLUTION FROM PRIMARY AMPUTATION
TO LIMB SALVAGE SURGERY Luciana Vinti1 , Stephen Daw2 , Constantino Sabado Alvarez3 , Franca
Fagioli4 , Auke Beishuizen5 , Gerard Michel6 , Maria Luisa Moleti7 ,
Israel Fernandez-Pineda1 , Bhaskar Rao2 , Michael Neel2 , Michael Michaela Cepelova8 , Anne Thorwarth9 , Charlotte Rigaud10 , Diego
Bishop3 , Lindsay Talbot2 , Andrew Murphy2 , Hafeez Abdelhafeez2 , Plaza Lopez De Sabando11 , Judith Landman Parker12 , Ying Zhu13 ,
Sandra Murphy2 , Lisa Emanus2 , Daphne Sanders2 Pallavi Pillai13 , Akash Nahar13 , Christine Mauz-Koerholz14
1 Virgen del Rocio Children’s Hospital, Pediatric Surgery, Sevilla, Spain; 2 St. 1 IRCCS Ospedale Pediatrico Bambino Gesu, Pediatric Hematology And
Jude Children’s Research Hospital, Surgery, Memphis, United States of Oncology, Rome, Italy; 2 University College London Hospitals NHS Founda-
America; 3 St. Jude Children’s Research Hospital, Oncology, Memphis, United tion Trust, Pediatrics And Hematology/oncology, London, United Kingdom;
States of America 3 Hospital Universitari Vall d Hebron, Pediatric Oncology, Barcelona, Spain;
4 Ospedale Infantile Regina Margherita, Pathology And Child Care, Turin,
Background and Aims: The most common primary extremity bone Italy; 5 Princess Máxima Centrum, Hematologic Malignancies, Utrecht,
sarcomas include Ewing sarcoma (ES) and osteosarcoma (OS). Whereas Netherlands; 6 CHU de Marseille Hopital de la Timone Enfants, Pedi-
ES being radio sensitive additional therapeutic measures were avail- atrics And Pediatric Hematology, Marseille, France; 7 Universita degli
able for local control, historically surgery in the form of amputation was Studi di Roma La Sapienza, Pediatric Hematology And Oncology, Rome,
the mainstay for OS. Herein, we describe progressive protocols used to Italy; 8 Fakultni nemocnice v Motole, Pediatric Hematology And Oncol-
obtain local control over the years for OS. ogy, Prague, Czech Republic; 9 Charite-Universitaetsmedizin Berlin Campus
Methods: From 1962-1980 of the 139 cases enrolled only one under- Virchow-Klinikum, Pediatric Oncology And Hematology , Berlin, Germany;
went limb salvage surgery (LSS) with a local control rate of 95%. 10 Gustave Roussy Cancer Campus, Pediatric Hematology And Oncology,
Based on the initial success by Rosen and Marcove using neoadjuvant Villejuif, France; 11 Hospital Universitario La Paz, Pediatric Hematology And
chemotherapy followed by LSS and local control of over 95%, we insti- Oncology, Madrid, Spain; 12 Hopital d’Enfants Armand Trousseau, Pediatrics,
tuted in 1980 a LSS program albeit using strict criteria. These included: Paris, France; 13 Merck & Co., Inc., Medical Oncology, Kenilworth, United
a) age>13years or 75% of anticipated growth b) no distant metastasis States of America; 14 Justus-Liebig University of Giessen, Giessen, Germany
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S22 of S635 ABSTRACTS

and Medical Faculty of the Martin-Luther-University of Halle-Wittenberg, nisms, in pediatric HL. We hypothesize that the transcriptomes of HRSc
Pediatric Hematology And Oncology, Halle, Germany and tumor-infiltrating lymphocytes will inform pathogenic mechanisms
and identify potential therapeutic targets in pediatric HL.
Background and Aims: The phase 2, open-label KEYNOTE-667 Methods: Multi-parameter flow cytometry was used to sort HRSc,
(NCT03407144) study is evaluating efficacy and safety of pem- CD4+/CD8+ T-cells, and CD20+/30+ B-cells from pediatric sub-
brolizumab plus chemotherapy in patients with classical Hodgkin jects’ HL lesions and control tonsils. Gene expression profiles (GEPs)
lymphoma (cHL) and slow early response (SER) to frontline chemother- were assessed using Affymetrix GeneChip HTA 2.0. GEPs of HL cells
apy. An interim analysis in patients with high-risk cHL (Group 2) with were compared to respective controls using a univariate t-test. Sig-
SER is presented. nificance was determined using a multivariate permutation test to
Methods: In Group 2, eligible patients aged 3-17 (children) or estimate false discovery. Differentially expressed genes (DEGs) were
18-25 years (young adults) with newly-diagnosed, high-risk cHL analyzed through ingenuity pathway analysis (IPA). Immunohistochem-
were enrolled to receive induction with vincristine, etoposide, pred- istry (IHC) was performed on pediatric HL lesions to validate results of
nisone/prednisolone, doxorubicin (OEPA) for 2 cycles. After induction IPA. In vitro assays were performed to test the pre-clinical impact of
treatment, patients with rapid early response received nonstudy apoptotic regulation.
therapy and SER patients received 4 cycles of cyclophosphamide, Results: GEPs were compared for HL samples: HRSc vs. control
vincristine, prednisone/prednisolone, dacarbazine (COPDAC-28) plus CD20+/CD30+ (1934/3846 DEGs), HL CD4+ vs. control CD4+ (635
pembrolizumab 2 mg/kg up to 200 mg IV Q3W. All patients with SER DEGs), HL CD8+ vs. control CD8+ (2 DEGs). Transcriptomic analy-
received maintenance pembrolizumab Q3W, for a total of 17 doses. sis (HRSc vs. control CD30+) revealed significant differences in gene
Primary endpoint: objective response rate by blinded independent cen- expression within pathways related to senescence, with upregulation
tral review (BICR) per Cheson 2007 IWG criteria in patients with SER. of anti-apoptotic/senescence-related genes (BCL-2, 5.00-fold; MCL-
Secondary endpoints: rate of PET-negativity after consolidation, and 1, 2.27-fold; GADD45A, 2.94-fold; PLAUR, 7.69-fold; CDKN1A/p21,
safety. 2.27-fold) and downregulation of pro-apoptotic/mitosis-related genes
Results: Median (range) follow-up was 9.6 months (2.5-21.2). Thirty (HRK, 1.23-fold; BIK, 4.17-fold; CCNB1, 11.11-fold; CCNB2, 16.67-
patients with high-risk cHL with SER were included. Median (range) fold; MKI67, 7.14-fold). IHC confirmed expression of senescence-
age was 15y (6-19), 13 (43%) had bulky disease, and 19 (63%) had Ann related proteins p21, BCL2, MCL1, UPAR, and GADD45A within HRSc.
Arbor stage IV disease. Six (20%) patients had completed treatment, Treating primary HRSc with BCL-2 inhibitor, venetoclax, resulted in
and 24 (80%) were ongoing. Of 30 patients, 25 (83%) had late response induction of apoptosis.
assessment, of whom 17 (68%) were PET-negative by BICR; 18 (72%) Conclusions: Flow-sorting can feasibly characterize specific cell pop-
were PET-negative by investigator. All-cause AEs occurred in 23 (77%) ulations within HL tumors. HRSc in this cohort expressed gene and
patients; 14 (47%) had a treatment-related AE. Grade ≥3 treatment- protein signatures consistent with oncogene-induced senescence –
related AEs occurred in 2 (7%) patients. One (3%) patient had a grade 2 a novel finding in pediatric HL and potentially druggable target.
immune-mediated AE of hypothyroidism. These findings support further pre-clinical and clinical testing of novel
Conclusions: In pediatric patients with high-risk cHL and SER to stan- therapeutic strategies that target pathologic senescence in pediatric
dard OEPA induction, pembrolizumab in combination with COPDAC- HL.
28 consolidation therapy was well tolerated and resulted in 68% of
patients having a PET-negative response at end of chemotherapy, and
being spared radiotherapy. O048 / #266 TREATMENT OF POST-TRANSPLANT
LYMPHOPROLIFERATIVE DISORDER (PTLD): A MULTI-CENTER
PEDIATRIC SERIES
O047 / #1465 PEDIATRIC HODGKIN REED-STERNBERG
CELLS HAVE GENE EXPRESSION CONSISTENT WITH Lianna Marks1 , Michael Green2 , Gregory Storch3 , Dita Gratzinger4 ,
ONCOGENIC-INDUCED SENESCENCE Hongjie Gu5 , Charles Goss5 , Vikas Dharnidharka3
1 Stanford University School of Medicine, Pediatrics, Palo Alto, United States

Jennifer Agrusa, Howard Lin, Harshal Abhyankar, Jessica Velazquez, of America; 2 University of Pittsburgh School of Medicine, Pediatrics, Pitts-
Elmoataz Abdel Fattah, Brooks Scull, Nmazuo Ozuah, Olive Eckstein, burgh, United States of America; 3 Washington University School of Medicine
Nader El-Mallawany, Nitya Gulati, Joseph Lubega, Terzah Horton, Kala in St. Louis, Pediatrics, St. Louis, United States of America; 4 Stanford Uni-
Kamdar, Kenneth Mcclain, Chris Man, Carl Allen versity School of Medicine, Pathology, Palo Alto, United States of America;
Baylor College of Medicine, Pediatric Hematology And Oncology, Houston, 5 Washington University School of Medicine in St. Louis, Biostatistics, St.
United States of America Louis, United States of America

Background and Aims: The rarity of Hodgkin Reed-Sternberg cells Background and Aims: Optimal treatment of patients with PTLD is
(HRSc) within Hodgkin lymphoma (HL) tumors (<1%) has limited the complex. Immunosuppression reduction is often recommended as first
characterization of HRSc gene expression, thus pathogenic mecha- line therapy but could induce rejection of the transplanted organ. Many
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S23 of S635

patients require antineoplastic therapy but are unable to tolerate side treatment approach, we conducted a retrospective cohort study to
effects. Understanding outcomes of different therapies helps guide assess outcomes of children treated with high-dose methotrexate
treatment decisions. (>1000 mg/m2/cycle) regimens in Lilongwe, Malawi.
Methods: As part of a multi-institutional study of viral genomes in Methods: Patients were classified as high-risk if they were Murphy
PTLD, we retrospectively collected clinical data (demographics, trans- Stage III with high lactate dehydrogenase or had Stage IV disease.
plant and PTLD characteristics, treatment, and patient survival) from Given its high correlation with true survival in low-resource settings,
three large pediatric transplant centers in the United States from 1991 we estimated the 12-month event-free survival (EFS), defined as sur-
onwards. vival from diagnosis to the earliest of treatment abandonment, relapse
Results: Among 133 children with PTLD, median recipient age at first or death; and overall survival (OS).
transplant was 4.7 years (IQR 1.2 to 12.6); 23% were below 1 year. Results: Between January 2017 and December 2020, we identified
PTLD patients were 52% male, 75% white, recipients of heart allo- 108 patients with high-risk Pediatric BL. They were mostly male
grafts (34%), lung (23%), liver (14%), kidney (13%), multi-organ (7%), (n=61, 56.5%) with a median age of 9.0 years (interquartile range,
hematopoietic (5%), or intestine (4%). Common PTLD locations were 6.0-11.0). Patients received chemotherapy combinations of cyclophos-
lymph nodes (41%) and GI tract (31%). Most were monomorphic dif- phamide, vincristine, doxorubicin and prednisone plus methotrexate
fuse large B-cell lymphoma (DLBCL) (59%) or polymorphic (26%), all dosed at 1000 mg/m2/cycle (n=98, 90.7%) or 3000 mg/m2/cycle.
other types 5% or less. PTLD lesions were diagnosed in the first year Treatment practices were based on practice patterns during different
post-transplant in 26% and were EBV-positive in 71%. Of 99 patients times and not per-patient decisions. A small population (n=23, 21.3%)
with available treatment information, 64% received rituximab and 55% received two additional Cytarabine-methotrexate (CYM) cycles with
received chemotherapy. Chemotherapy received included cyclophos- methotrexate similarly dosed. Treatment abandonment was 15.7%.
phamide (90%), prednisone (80%), vincristine (63%), and doxorubicin The 12-month EFS and OS were 39% (95%CI, 30%-50%) and 53%
(52%). Therapeutic surgery was performed in 11% of patients, 3% (95% CI, 43-65%) respectively. Patients who received methotrexate
received radiation, and 1% received EBV cytotoxic T-cells. Of the 38 3000 mg/m2/cycle had significantly improved EFS: 70% (95%CI, 47%-
DLBCL cases, 29% received rituximab alone, 5% received chemother- 100%) versus 36% (95%CI, 27%-47%), p=0.02. Treatment-related
apy alone, 40% received both, and 21% were treated without either. mortality occurred in 1/10 patients treated with methotrexate 3000
Ten-year overall survival was 100%, 85.7%, and 63.7% for patients with mg/m2/cycle.
non-destructive, polymorphic, and monomorphic PTLD respectively. Conclusions: High-dose methotrexate approximating that given in
Conclusions: In our series, most patients with monomorphic DLBCL high-resource settings yields superior survival outcomes and can be
PTLD received treatment with both rituximab and chemother- feasibly delivered in low-resource settings. Further studies should
apy; outcomes varied by histology. As we accumulate more identify patients for whom methotrexate dose may be optimized while
information, we will perform multivariable analyses of treatment maintaining acceptable treatment-related mortality.
outcomes.

O050 / #1511 OUTCOME OF CHILDHOOD MATURE B-NHLS


O049 / #882 REAL-WORLD EXPERIENCE USING HIGH-DOSE IN INDIA - RESULTS OF A RETROSPECTIVE MULTI-CENTRE
METHOTREXATE IN THE TREATMENT OF HIGH-RISK POOLED ANALYSIS
PEDIATRIC BURKITT LYMPHOMA IN A RESOURCE-LIMITED
CENTER IN LILONGWE, MALAWI Nirmalya Roy Moulik1 , Sameer Bakhshi2 , Venkatraman
Radhakrishnan3 , Amita Trehan4 , Niharendu Ghara5 , Anshul Gupta6 ,
Loviisa Mulanje1 , Casey Mcatee2 , Rizine Mzikamanda1 , Nader Rachna Seth7 , Sripad Banavali1 , Ramandeep Arora8
El-Mallawany3 , Katherine Westmoreland4 , Carl Allen3 , Grace 1 Tata Memorial Centre, Paediatric Oncology, Mumbai, India; 2 All India
Chirwa1 , Nmazuo Ozuah2 Institue of Medical Sciences, Medical Oncology, New Delhi, India; 3 Cancer
1 Baylor College of Medicine Children’s Foundation, Malawi, Texas Children’s Institute (W.I.A), Medical Oncology, CHENNAI, India; 4 Advanced Pediatrics
Global Hope, Lilongwe, Malawi; 2 Texas Children’s Global HOPE Lilongwe, Center, Postgraduate Institute of Medical Education and Research, Pedi-
Baylor College Of Medicine, Lilongwe, Malawi; 3 Texas Children’s Global atric Hematology Oncology Unit, Chandigarh, India; 5 Tata Medical Center,
HOPE, Paediatrics Baylor College Of Medicine, Houston, United States of Department Of Pediatric Hematology And Oncology, Kolkata, India; 6 Sanjay
America; 4 UNC Project Malawi, Cancer Program, Lilongwe, Malawi Gandhi Post Graduate Institute of Medical Sciences, Hematology, Luc-
know, India; 7 All India Institute of Medical Sciences- New Delhi, Division
Background and Aims: Excellent survival outcomes for pediatric Of Pediatric Oncology, Department Of Pediatrics, New Delhi, India; 8 Max
Burkitt lymphoma (BL) have been achieved with intensive regimens Super-Specialty Hospital, Medical Oncology, New Delhi, India
containing methotrexate at doses of 3000 mg/m2/cycle or higher. The
adaptation of these regimens in low-resource settings such as sub- Background and Aims: Published data on outcome of pediatric mature
Saharan Africa has been limited, and often necessitate modifications to B-NHLs in India are scant and difficult to interpret due to non-
methotrexate dosing. To continuously improve our resource-adapted uniformity in the protocols as well as limited numbers reported by each
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S24 of S635 ABSTRACTS

center. We performed a pooled analysis of published Indian data to Methods: We reflect the patient’s narrative from the perspective of his
address this issue. transition during the treatment of his disease with a semi-structured
Methods: Studies published between 2000-2022 were included. Infor- interview Young man currently 21 years old, diagnosed with ALL T at
mation on disease-stage, treatment protocol and outcome (available age 14, in second complete remission after haploidentical transplant at
for all patients) and areas of involvement, FAB/BFM risk groups, tox- age 18 in the adult unit Comparison of experiences lived in the pediatric
icities (available for most patients) was collected from raw-data sent unit and of adults in the transplant unit Review of differences between
by the primary authors. Analysis for baseline characteristics, toxici- Units
ties, treatment outcome and survival was done using SPSS software Results: Review of: -importance of shared care -the time of leaving the
(v. 20). pediatric unit: it creates a perception of abandonment or lack of protec-
Results: Five hundred five children (Male: Female-5:1) with mature B- tion if it is not done in a progressive and shared manner -adaptation of
NHL (Burkitt- 395/DLBL-52/Others-58) from 7 major cancer-centers the information according to age and maturity of the patients: pediatri-
across India were included. Most (401/505) patients presented cians show a greater adaptation -integration in shared decision-making
with advanced disease (Stage 3/4). Bone-marrow and CNS Involve- with the family: greater involvement by pediatrics -tolerance to nego-
ment were seen in 70(13.9%) and 35(6.9%). Grade 3/4 toxicities, tiation demands (sedation for painful procedures): less by the adult
toxic-deaths and relapse/progression were seen in 344/434(79.2%), team “This is Adults and we are going to treat you like an adult” -He
69/484(14.2%) and 59/484(12.1%) patients with available data. Cumu- has not been "infantilized" in the pediatric unit: he considers that he
lative and stage-wise (Stages-1/2/3/4 in order) overall survival (OS) of has enjoyed sharing his activities with other boys his age and also with
the whole cohort at median follow-up of 17 months was 70.4±2.2% younger ones
and 84.7±7.1%, 90.4±4.4%, 70.2±2.8%, 57.2±5.2% (p<0.001) Conclusions: The patient has collaborated in informative recommen-
respectively. Major treatment protocols included LMB(n=208) dations of procedures for other patients through social networks and
and BFM(n=191) followed by MCP(n=61). More patients in educational videos for residents and nurses. The experience of ado-
LMB group had advanced stage/higher LDH (p<0.001). Though lescents as a subject involved in the design of the transition of care
higher incidence of G3/4 toxicities were seen with LMB(92.1%) vs. from pediatric to adult unit is useful for developing transition protocols
BFM(70.8%)/MCP(70.1%) protocols (p<0.001), toxic-death rates between this units. It has not been until recently that pediatric patients
were similar. Incidence of relapse/progression and stage-wise OS have been included up to the age of 18 in our unit, however this step
were similar between protocols (p=0.28 and 0.51).Overall-survival of has been facilitated by good communication and previous relationship
CNS positive patients were 70.9±11.3%, 31.8±28.6 and 37.5±28.6% with the adult hematology unit
(p<0.001) when treated as per LMB, BFM and MCP protocols respec-
tively. Higher stage and inadequate-response on interim-PET were
associated with poor survival on multivariate-analysis. O052 / #451 ADOLESCENTS AND YOUNG ADULTS WITH
Conclusions: Outcome of mature B-NHLs in India, especially that BONE & SOFT TISSUE SARCOMA- EFFICACY OF
of advanced disease remains poor. Promulgation of a new setting- PSYCHOSOCIAL INTERVENTIONS FOR PATIENTS AND THEIR
adapted protocol taking into account the excess toxicity of some pro- SIBLINGS
tocols and inferior efficacy of others especially for advanced disease is
the way forward. Sachi Pandya1 , Jayita Deodhar2 , Mrinalini Purandare3
1 Narayana Health SRCC Children’s Hospital, Department Of Psychological
Medicine And Department Of Family First, mumbai, India; 2 Tata Memorial
CCI Hospital, Department Of Palliative Medicine & Psychiatry, mumbai, India;
3 SNDT Women’s University, Department Of Psychology And Counselling,
CCI: RESPONDING TO THE NEEDS OF ADOLESCENTS AND mumbai, India
YOUNG ADULTS WITH CANCER 29-09-2022 9:40 AM - 11:10
AM Background and Aims: The experience of cancer can be daunting and
isolating for the family leading to life alterations. Recognising unmet
O051 / #1873 TRANSITION FROM THE PEDIATRIC UNIT TO needs of healthy siblings and providing psychosocial support for the
THE ADULT UNIT. I AM A GARDENER AND I AM GLAD TO healthy siblings in the family is crucial to patients’ holistic care. This
HAVE MET YOU study aims to investigate the role of psychosocial interventions in
relieving distress among adolescents and young adults (AYAs) with
Mercedes Guibelalde Del Castillo, David Diaz, Alejandro Terrón, sarcoma and their AYA siblings.
Samuel Navarro Methods: 40 AYA patients, age- 14yrs. to 29yrs., on active treatment,
Son Espases, Pediatric Oncology, Palma, Spain their healthy AYA siblings enrolled for the study; 20 patient-sibling
dyads in each, Experimental group (EG) and Control group (CG). Con-
Background and Aims: Describe our patient’s view as expert in trol group was added later.as comparator. Pre-test post-test quasi
transition from pediatric age to adolescent unit experimental design used to study change in Depression, Anxiety
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S25 of S635

among patients and siblings after psychosocial interventions. Body Hospital, Neuro- Oncology, Vancouver, Canada; 11 The Hospital for Sick Chil-
image was studied in patients alone. Six home-based intervention ses- dren, Division Of Pathology, Toronto, Canada; 12 12Children’s Hospital of
sions for patients-siblings dyads in EG included arts based therapy, Philadelphia, Ophthalmology, Washington, United States of America; 13 The
Cognitive Behavioral Therapy, psycho-education, family and support- Hospital for Sick Children, Hematology Oncology, Toronto, Canada
ive therapy over 3 months. Interview and feedback were obtained from
patients-siblings dyads in EG and qualitatively analysed. CG had no Background and Aims: Background and aims: Several reports have
interventions. suggested promising activity of bevacizumab in paediatric low-grade
Results: Post test scores of both AYA patients and AYA sibling in gliomas (pLGG). However, most studies are limited, and no randomised
each arm of EG and CG were analysed using independent t-test. trial has ever been conducted.
Results revealed statistically significant difference between the two Methods: Trial design: This randomised trial (NCT02840409) enrolled
groups of AYA patients (EG and CG) on Depression (t=4.68; p<0.001*), chemotherapy-naive patients aged 6 months-18 years with low-grade
State Anxiety (t=4.13; p<0.01*), Trait Anxiety (t=3.29; p<0.01*) and glioma. Patients were stratified according to neurofibromatosis and
Body Image (t=3.65; p<0.01*). Healthy AYA siblings in EG and CG BRAF status (fused/mutated/neither) and received (ARM-A) vinblas-
also showed statistical significance on Depression (t=7.64; p<0.001*), tine alone (5 mg/m2 /week) or (ARM B) a combination of vinblastine
State Anxiety (t=6.89; p<0.001*) and Trait Anxiety (t=4.20; p<0.001*). (5 mg/m2 /week) and bevacizumab (biweekly 10 mg/kg for 24 weeks).
Healthy siblings also experience significant distress and benefit by All patients then received maintenance weekly vinblastine to a total of
psychosocial interventions. . 68 weeks. Primary endpoints were radiological response at 6 months
Conclusions: Significant distress is experienced by patients and healthy using revised RANO criteria and for patients with optic pathway
siblings during treatment. Psychosocial interventions help alleviate tumours (OPT) visual changes at 6 months; The goal for enrollment
anxiety and depressive feelings Psychological support is paramount; was 150 patients. An interim analysis was conducted after 50% of the
it enhances mental and social well-being allowing familial cohesive- patients were enrolled.
ness and their coping ability. Inclusion of mental health care along with Results: At the data cut-off (04/2021), 85 patients were enrolled with
treatment improves patient compliance and well-being 78 (44 females; median age 6-year-old) with complete information on
response evaluation. Response rate at 6 months was 24% in ArmA (Sta-
ble disease 73%; Progressive disease 3%) versus 40% in ArmB (Stable
FREE PAPER SESSION (FPS) disease 57%; Progressive disease 3%) (p=0.2). For the 33 patients with
OPT, 26 had visual assessment (HOTV) at baseline and at 6 months:
FPS 02: BRAIN TUMORS - CLINICAL STUDIES 29-09-2022 9:40 HOTV of left eye showed significant difference between ArmA (15%
AM - 11:10 AM improvement, 85% stable) and ArmB (15% improvement, 46% sta-
ble, 38% worsening) (p=0.03), whereas no difference was observed in
O053 / #1605 VINBLASTINE MONOTHERAPY OR IN visual assessment for right eyes ArmA (14% improvement, 71% sta-
COMBINATION WITH BEVACIZUMAB IN CHEMOTHERAPY ble, 14% worsening) and ArmB (25% improvement, 50% stable, 25%
NAÏVE PAEDIATRIC PATIENTS WITH LOW-GRADE GLIOMA: worsening) (p=0.63).
RESULTS OF AN INTERIM ANALYSIS Conclusions: In this interim analysis, although the radiological
response rate at 6 months suggests a slight advantage for Arm B,
Eric Bouffet1 , Adam Fleming2 , Jordan Hansford3 , Beverly Wilson4 , addition of bevacizumab appears not to offer visual benefit. This study
Dolly Aguilera5 , Sebastien Perreault6 , Eugene I Hwang7 , Nick underscores the critical importance of including functional outcomes
Gottardo8 , Magimairajan Issai Vanan9 , Juliette Hukin10 , Tara in therapeutic clinical trials for pLGG.
Mckeown1 , Cynthia Hawkins11 , Robert Avery12 , Uri Tabori13
1 The Hospital for Sick Children, Division Of Hematology And Oncology,
Toronto, Canada; 2 McMaster, Mcmaster Children’s Hospital, Division Of O054 / #539 RADIATION INDUCED HIGH GRADE GLIOMAS:
Pediatric Hematology/oncology„ Hamilton, Canada; 3 Michael Rice Cancer A SINGLE CENTER EXPERIENCE
Centre, Women’s and Children’s Hospital; South Australian Health and
Medical Research Institute; South Australian ImmunoGENomics Cancer Mrinal Joshirao1,2 , Sameer Farouk Sait1 , Nancy Bouvier1 , Katherine
Institute, University of Adelaide, Oncology And Pediatrics, Adelaide, Aus- Hill1 , Yasmin Khakoo3 , Kim Kramer1 , Stephen Gilheeney1 , Suzanne
tralia; 4 Stollery Children’s Hospital, Northern Alberta Children’s Cancer Wolden4 , Craig Nolan3 , Lauren Schaff3 , Marc Rosenblum5 , Tejus
Program, Edmonton, Canada; 5 Children’s Health Care of Atlanta, Pediatric Bale5 , Jonathan Yang4 , Andrew Lin3 , Ira Dunkel1 , Matthias
Oncology, Atlanta, United States of America; 6 CHU Sainte Justine, Hema- Karajannis1
tology Oncology, Montreal, Canada; 7 Children’s National Hospital, Division 1 Memorial Sloan Kettering Cancer Center, Pediatric Neuro-oncology, New
Of Oncology, Washington, United States of America; 8 Perth Children’s Hos- York, United States of America; 2 SUNY Downstate Health Sciences Uni-
pital, Department Of Paediatric And Adolescent Oncology/haematology, versity, Pediatrics, Brooklyn, United States of America; 3 Memorial Sloan
Perth, Australia; 9 University of Manitoba / Cancer Care Manitoba, Pedi- Kettering Cancer Center, Neurology, New York, United States of Amer-
atrics And Child Health, Winnipeg, Canada; 10 British Columbia Children’s ica; 4 Memorial Sloan Kettering Cancer Center, Radiation Oncology, New
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S26 of S635 ABSTRACTS

York, United States of America; 5 Memorial Sloan Kettering Cancer Center, of America; 5 Seattle Children’s Hospital, Pediatrics, Seattle, United States
Pathology, New York, United States of America of America; 6 University of Colorado, Children’s Hospital Colorado, Cen-
ter For Cancer And Blood Disorders, Aurora, United States of America;
Background and Aims: Patients receiving cranial radiotherapy (RT) 7 University of California, Hematology Oncology, San Francisco, United
are at risk for subsequent radiation-induced glioma (RIG). RIGs are States of America; 8 Nationwide Children’s Hospital, Hematology, Oncol-
rare, develop with a latency of two years to several decades, dis- ogy & Bone Marrow Transplant, Columbus, United States of America;
play high-grade histology and an aggressive clinical course with poor 9 University of Alabama Birmingham, Pediatrics, Birmingham, United States

prognosis. of America; 10 Cincinnati Children’s Hospital Medical Center, Pediatrics,


Methods: We retrospectively reviewed patients with a diagnosis of Cincinnati, United States of America; 11 Alberta Children’s Hospital, Pedi-
RIG seen at our institution from 2001–2021, analyzing clinical, histo- atric Hematology Oncology And Bone Marrow Transplantation, Calgary,
logical, molecular, and genetic characteristics. Canada; 12 Washington University School of Medicine, Hematology Oncol-
Results: Twenty-one patients (n=15 male) with a history of ALL (n=6), ogy, St Louis, United States of America; 13 Arnold Palmer Hospital for
medulloblastoma (n=5), germ cell tumors (n=4), or other (n=6) with Children, Pediatrics, Orlando, United States of America; 14 CHU Sainte
a median age at primary cancer diagnosis of 8.3 years (range 1.6 Justine, Hematology Oncology, Montreal, Canada; 15 Memorial Sloan Ket-
to 36.4) were identified. Median age at RIG diagnosis was 18 years tering Cancer Center, Pediatric Neuro-oncology, New York, United States of
(range 7.8 to 66.9). Prior RT was focal+craniospinal (n=7), whole brain America
(n=5), total body (n=3), focal (n=1), or unknown (n=5). Median radi-
ation dose received was 2,340 cGy (range 1,200 to 5,400). Median Background and Aims: Background: Multi-modal therapy with surgi-
time from RT to RIG diagnosis was 7.7 years (range 1.6 to 23.8). All cal resection, high dose chemotherapy (HDC) and risk-adapted radia-
RIGs were histologically high grade (WHO grade 3 or 4). Immunohis- tion therapy (RT) is a reasonable treatment strategy for children with
tochemistry did not reveal IDH1(R132H) (n=9) or H3K27M (n=8) in atypical teratoid rhabdoid tumor (AT/RT), however, the effect of certain
any tumor tested, however, loss of H3K27me3 was observed in 3/6. clinical and treatment variables on outcome remains unknown.
Targeted panel sequencing (n=10) revealed recurrent somatic alter- Methods: We retrospectively collected data on children with AT/RT
ations including CDKN2A/B, PDGFRa/KIT/KDR, TEK, MTAP, ATM and treated at 13 North American institutions with multi-modal ther-
NF1. Germline alterations were detected in 4/12 patients, including apy on study or as per protocol ACNS0333 with or without RT.
pathogenetic variants in ATM, CHEK2, HOXB13 and NF1. With median Data collected included patient characteristics, treatment details,
follow-up of 4.5 years, two-year PFS and OS for the cohort (n=20) toxicity, and survival outcomes. The event-free survival (EFS) was
were 10% and 44%, respectively. Two patients who were treated with defined by time to relapse, progression, or death, whichever occurred
surgery, RT and chemotherapy are alive without progression 5.4 and first.
13.6 years after diagnosis. Results: The cohort included 80 patients with a median age at diagno-
Conclusions: Our findings suggest that heterozygous germline alter- sis of 18 months (range: 3.7-324 months). The 4-year EFS and overall
ations in DNA damage response or tumor suppressor genes may survival (OS) for the entire cohort was 48.7% (95% CI: 36.9-59.5%)
predispose to RIG. Although associated with a poor prognosis, RIGs and 53.4% (95% CI: 41.2–64.1%), respectively. Fifty-five patients (69%)
are not always fatal and aggressive multi-modal therapy should be received RT (41 focal, 14 craniospinal) while 25 received chemotherapy
considered for these patients. alone. Of those 25, 17 completed prescribed therapy while 8 suffered
early progression. Both EFS and OS were better for patients who
received RT compared to those that did not. Patients who received
O055 / #1694 RADIATION THERAPY IS AN IMPORTANT RT had a 4-year EFS and OS of 62.8% (95% CI 47.75–74.62) and
COMPONENT OF MULTI-MODAL THERAPY FOR CHILDREN 66.5% (95% CI 51.43–77.87), respectively, compared to 28.2% (95% CI
WITH ATYPICAL TERATOID RHABDOID TUMOR 9.62–50.5) and 36.76% (95% CI 14.5–59.5) for those who completed
prescribed therapy with chemotherapy alone. Age at diagnosis, tumor
Ashley Margol1,2 , Lindsey Hoffman3 , Jane Tran2 , Jemily Malvar2 , location, metastatic stage, and extent of resection was not associated
Yuen-Yun Chi2 , Sarah Leary4 , Jeffrey Stevens5 , Kelly Faulk6 , Clara with survival in the univariate analysis.
Wu7 , Ralph Salloum8 , Rebecca Ronsley8 , Sara Claire Hutchins9 , Conclusions: Children with AT/RT treated with HDC and RT have
Natasha Pillay-Smiley10 , Lucie Lafay-Cousin11 , Mohammed improved survival compared to those treated without RT and higher
Abdelbaki12 , Andrew Cluster12 , Ana Aguilar-Bonilla13 , Sebastien than previously reported cohorts.
Perreault14 , Stephen Gilheeney15 , Luca Szalontay15 , Alyssa Reddy7 ,
Girish Dhall9
1 Children’s Hospital Los Angeles, Cancer And Blood Disease Institute, Los O056 / #491 SURVIVAL AND PATTERNS OF FAILURE
Angeles, United States of America; 2 Keck School of Medicine of University FOLLOWING RADIATION (RT) IN PATIENTS WITH ATYPICAL
of Southern California, Pediatrics, Los Angeles, United States of America; TERATOID RHABDOID TUMORS (ATRT) ON ACNS0333: A
3 Phoenix Children’s Hospital, Pediatric Oncology, Phoenix, United States of REPORT FROM THE CHILDREN’S ONCOLOGY GROUP
America; 4 Seattle Children’s, Hematology Oncology, Seattle, United States
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S27 of S635

Jared Deck1 , Paul Aridgides1 , Mark Krailo2 , Allen Buxton2 , Anita O057 / #1706 A METRONOMIC ANTIANGIOGENIC REGIMEN
Mahajan3 , Thomas Merchant4 , Douglas Strother5 , Annie Huang6 , (MEMMAT) INDUCES SUSTAINED SURVIVAL BENEFIT IN
Jaclyn Biegel7 , Alexander Judkins8 , Ben Ho9 , Claire Mazewski10 , RECURRENT MEDULLOBLASTOMA
Victor Lewis11 , Ian Pollack12 , Maryam Fouladi13 , Alyssa Reddy14
1 SUNY Upstate, Radiation Oncology, Syracuse, United States of America; Andreas Peyrl1 , Monika Chocholous1 , Magnus Sabel2 , Alvaro
2 Children’s Oncology Group, Statistics, Monrovia, United States of Amer- Lassaletta3 , Jaroslav Sterba4 , Pierre Leblond5 , Karsten Nysom6 , Ingrid
ica; 3 Mayo Clinic, Radiation Oncology, Rochester, United States of America; Torsvik7 , Susan Chi8 , Martin Benesch9 , Neil Jones10 , Stefan Holm11 ,
4 St. Jude Children’s Research Hospital, Radiation Oncology, Memphis, Per Nyberg12 , Helena Mörse13 , Anders Öberg14 , Ulrike Leiss1 ,
United States of America; 5 Alberta Children’s Hospital, Hematology Oncol- Christine Haberler15 , Johannes Gojo1 , Amedeo Azizi1 , Nicolas
ogy, Calgary, Canada; 6 Hospital for Sick Children, Hematology/oncology, Andre16 , Mark Kieran8 , Irene Slavc1
Toronto, Canada; 7 Children’s Hospital Los Angeles, Pathology And Labora- 1 Medical University of Vienna, Department Of Paediatrics, Vienna, Austria;

tory Medicine, Los Angeles, United States of America; 8 Children’s Hospital 2 Sahlgrenska University Hospital, Hematology Oncology, Gothenburg, Swe-

Los Angeles, Pathology, Los Angeles, United States of America; 9 Hospital for den; 3 Hospital Infantil Universitario Niño Jesús, Pediatric Neuro-oncology,
Sick Children, Brain Tumor Research Centre, Toronto, Canada; 10 Children Madrid, Spain; 4 University Hospital Brno, Pediatric Oncology Department,
Healthcare of Atlanta, Hematology Oncology, Atlanta, United States of Brno, Czech Republic; 5 Centre Léon Bérard, Institut D’hématologie Et
America; 11 Alberta Children’s Hospital, Hematology/oncology/transplant D’oncologie Pediatrique, Lyon, France; 6 Rigshospitalet, Paediatric Oncol-
Program, Calgary, Canada; 12 Children’s Hospital of Pittsburgh of UPMC, ogy, Copenhagen, Denmark; 7 Haukeland universitetssjukehus, Onkologisk-
Neurosurgery, Pittsburgh, United States of America; 13 Nationwide Chil- hematologisk Seksjon, Bergen, Norway; 8 Dana- Farber Cancer Institute,
dren’s Hospital, Neuro-oncology Program, Columbus, United States of Pediatric Neuro-oncology, Boston, United States of America; 9 Medical Uni-
America; 14 University of California, Hematology Oncology, San Francisco, versity of Graz, Department Of Paediatrics, Graz, Austria; 10 Salzburger

United States of America Universitätsklinikum, Kinderonkologie, Salzburg, Austria; 11 Karolinska Uni-


versity Hospital, Dept Of Pediatric Hematology And Oncology, Stockholm,
Background and Aims: ACNS0333 incorporated multi-modal therapy, Sweden; 12 Linköping University Hospital, Paediatrics, Linköping, Sweden;
including radiation therapy (RT) to treat AT/RT. Here, we investigate 13 Skånes universitetssjukhus, Pediatrics, Lund, Sweden; 14 Uppsala Univer-
survival outcome and patterns of failure according to radiation dose sity, Pediatrics, Uppsala, Sweden; 15 Medical University of Vienna, Neurol-
and field. ogy, Vienna, Austria; 16 AP-HM, Marseille France, Centre D’essais Précoces
Methods: ACNS0333 therapy included surgery, two cycles of multi- En Cancérologie De Marseille (cepcm), Clipp2, Marseille, France
agent induction chemotherapy, three cycles of high-dose chemother-
apy with stem cell rescue, and RT. Timing of RT was based on patient Background and Aims: Patients with recurrent medulloblastoma have
age, tumor location, and M-status. Forty of 65 evaluable patients a poor prognosis, irrespective of therapy used, including surgery, con-
received RT on study (29 M0, 11 M+); 25 (12 M0, 13 M+) went off ventional chemotherapy, re-irradiation, and high-dose chemotherapy.
protocol prior to RT. Focal RT (M0) followed age and tumor site dose An alternative approach is an antiangiogenic metronomic combination
guidelines (50.4 or 54Gy). Craniospinal (CSI) RT was optional if M+. A therapy that inhibits multiple pro-angiogenic pathways to overcome
p-value of <0.05 was considered statistically significant. treatment resistance.
Results: Median age at RT was 1.8 years (0.7 – 13.9). 4-year Event Methods: We present an international phase II study for recurrent
Free Survival (EFS) and Overall Survival (OS) and were 50% and 59%, or progressive medulloblastoma who were treated with a metro-
respectively from RT start. 4-year EFS for M0 and M+ were 41% and nomic antiangiogenic regimen (MEMMAT). Treatment consisted of
73% respectively (p=0.09). However, more M+ patients (54%) went bevacizumab infusion every two weeks, continuous oral celecoxib,
off protocol therapy prior to RT compared to M0 (29%). Following thalidomide, and fenofibrate, with alternating 21-day cycles of low-
focal RT (M0) 4-year local-only failure was 21%, distant-only 14%, and dose cyclophosphamide and etoposide, as well as intraventricular
combined local/distant 14%. For M+ (focal RT n=5, 23.4Gy CSI n=2, therapy with etoposide and cytarabine.
36Gy CSI n=4), 4-year local-only failure was 9%, distant-only 9%, and Results: Between April 2014 and March 2021, 40 patients were
combined local/distant 9%. 4-year EFS of M+ receiving focal RT and enrolled and received the MEMMAT combination treatment. The 60-
CSI were 80% and 67%, respectively (p=0.72). Local-failure (4-year) month PFS rate was 24.6%. Median PFS and OS were 8.5 and 25.5
was similar between primary dose of 54Gy (n=8) and £50.4Gy (n=32); months, respectively. The clinical benefit rate was 57.5%, and the
28% and 31%, respectively (p=0.66). Among all RT patients, 4-year response rate was 45.0%. No significant differences in PFS or OS was
OS for pre-RT chemotherapy complete response (CR), partial response evident regarding the molecular group or the number of recurrences. In
(PR), and stable disease (SD) were 80%, 51%, and 0% respectively patients who showed any response, the 60-month PFS rate was 49.7%,
(p=0.0069). in patients that had no progression after 12 months of treatment,
Conclusions: Focal radiation for localized and disseminated ATRT the 60-month PFS rate was 66.7%. Most grade 3-4 treatment-related
yielded encouraging outcomes on ACNS0333 when combined with adverse events were hematologic, with neutropenia in 40% and ane-
intensive multimodality therapy. Patients in CR prior to RT exhibited mia 15% of the patients, grade 3-4 infections with febrile neutropenia
highest OS. in 5% and sepsis in 5%, and grade 3-4 neurologic complications with
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S28 of S635 ABSTRACTS

seizures in 7.5% and headache in 7.5% of the patients. One patient died tology Oncology, Pragues, Czech Republic; 24 Paediatric Hospital Dr Juan
from acute myeloid leukemia. P Garrahan, Hematology Oncology, Buenos Aires, Argentina; 25 Memorial

Conclusions: The MEMMAT regimen has promising clinical activity in Sloan Kettering Cancer Center, Hematology Oncology, New York, United
recurrent medulloblastoma and lead to long-term responses in 25% of States of America; 26 Children’s Healthcare of Atlanta, Hematology Oncol-
the patients. ogy, Atlanta, United States of America; 27 Children Healthcare of Atlanta,
Hematology Oncology, Atlanta, United States of America; 28 University of
Texas Health Science Center at San Antonio, Hematology Oncology, San
O058 / #137 RELAPSE SHH MEDULLOBLASTOMA (MB) IN Antonio, United States of America; 29 St Jude Research Hospital, Oncol-
YOUNG CHILDREN. ARE THERE ALTERNATIVES TO FULL-DOSE ogy And Pediatrics, Memphis, United States of America; 30 UCSF Medical
CRANIOSPINAL IRRADIATION? Center-Mission Bay, Hematology Oncology, San Francisco, United States
of America; 31 Saint Jude Children’s Research Hospital, Developmental
Craig Erker1 , Brandon Craig2 , Simon Bailey3 , Maura Massimino4 , Neurobiology, Memphis, United States of America; 32 Newcastle Univer-
Valerie Larouche5 , Jonathan Finlay6 , Cassie Kline7 , George Michaiel8 , sity Centre for Cancer, Hematology Oncology, Newcastle, United Kingdom;
Ashley Margol9 , Kenneth Cohen10 , Chantel Cacciotti11 , Virginia 33 Hospital for Sick Children, Hematology Oncology, Toronto, Canada;
Harrods12 , Kathleen Dorris13 , Mohammed Abdelbaki14 , Nisreen 34 Alberta Children’s Hospital, Pediatric Hematology Oncology And Bone
Amayiri15 , Zhihong Wang16 , Jordan Hansford17 , Juliette Hukin18 , Marrow Transplantation, Calgary, Canada
Ralph Salloum19 , Lindsay Hoffmann20 , Jeffrey Murray21 , Kevin
Ginn22 , Michal Zapotocky23 , Lorena Baroni24 , Stephen Gilheens25 , Background and Aims: Following initial radiation sparing therapy,
Dolly Aguiera26 , Claire Mazewski27 , Shafqat Shah28 , Douglas many young children with relapsed MB can be salvaged with cran-
Strother2 , Amar Gajjar29 , Sabine Mueller30 , Paul Northcott31 , Steve iospinal irradiation (CSI). However, the interval to relapse is short and
Clifford32 , Giles Robinson29 , Eric Bouffet33 , Lucie Lafay-Cousin34 neurocognitive sequelae remain a major concern. The contribution of
1 IWK Health Centre, Pediatrics, Halifax, Canada; 2 Alberta Children’s Hos- molecular subgrouping may help refine indications and modalities of
pital, Hematology Oncology, Calgary, Canada; 3 Great North Children’s salvage strategies in this population
Hospital, Hematology Oncology, Newcastle, United Kingdom; 4 Fondazione Methods: From a cohort of 151 young children with molecularly
IRCCS Istituto Nazionale dei Tumori, Pediatric Oncology Unit, Milan, Italy; characterized relapsed MB, sub-analysis of the SHH MB was con-
5 CHU de Quebec-Centre Hospitalier de l’Universite Laval (CHUL), Hematol- ducted to describe the practice of salvage radiotherapy and associated
ogy/oncology, Quebec City, Canada; 6 OSU- Ohio State University, Emeritus post-relapse survival (PRS).
Professor Pediatrics And Radiation Oncology, Columbus, United States Results: Sixty-seven SHH MB patients (46 M0; 54 GTR; 11 non-
of America; 7 Children’s Hospital of Philadelphia, Hematology Oncology, ND/MBEN) received salvage therapy with curative intent. Before
Philadelphia, United States of America; 8 British Columbia Children’s Hos- relapse, 54 (80.6%) received conventional chemotherapy (CC), 13
pital, Pediatric Hematology/oncology/bm, Vancouver, Canada; 9 Children’s (19.4%) high-dose chemotherapy (HDC), while 7 also received focal
Hospital Los Angeles, Hematology Oncology, Los Angeles, United States radiation (fRT). Median time to relapse was 11.1 months (range 3.8-
of America; 10 Johns Hopkins University/Sidney Kimmel Cancer Center, 41.0), and 43.3% of the relapse were localized. Thirty patients (16
Oncology And Pediatrics, Baltimore, United States of America; 11 Children’s localized relapse) underwent surgery. Forty-seven (71.2%) received
Hospital of London, Pediatric Hematology/oncology, London, Canada; 12 Dell salvage radiation (15 RT alone, 20 with CC; 10 with HDC; 2 unknown).
Children’s Medical Center, Hematology Oncology, Austin, United States of CSI and fRT accounted for 82% and 18% respectively. CSI median
America; 13 Children’s Hospital Colorado, Hematology Oncology, Denver, dose was 36Gy(range 18-39Gy). Ten patients (8 with localized relapse)
United States of America; 14 Washington University School of Medicine, received CSI dose ≤23.4Gy. Nineteen patients (28.8%) did not receive
Hematology Oncology, St Louis, United States of America; 15 King Hussein any radiation (9 HDC; 10 CC only). Radiotherapy was associated with
Cancer Center, Pediatric Oncology, Amman, Jordan; 16 Virginia Common- better 3-year PRS (73.0% vs 36.1%; p=0.001). All patients treated with
wealth University/Massey Cancer Center, Pediatrics, Richmond, United CSI ≤ 23.4Gy were alive at median follow-up of 69 months (24-142).
States of America; 17 Royal Children’s Hospital, Oncology And Pediatrics, Six of 10 patients treated with HDC without radiation were alive at last
Melbourne, Australia; 18 British Columbia Children’s Hospital, Neuro- Oncol- follow-up. Sixty-three percent of patients received either reduced dose
ogy, Vancouver, Canada; 19 Nationwide Children’s Hospital, Hematology CSI (≤23.4Gy), fRT, or no radiation.Their PRS did not significantly differ
Oncology, Columbus, United States of America; 20 Phoenix Children’s Hos- from those who received CSI ≥30.6Gy (p = 0.54).
pital, Hematology Oncology, Phoenix, United States of America; 21 Cook Conclusions: While salvage CSI provided PRS benefit in this SHH MB
Children’s Medical Center, Hematology Oncology, Fort Worth, United States cohort, we describe practice of reduced salvage radiotherapy and radi-
of America; 22 Children’s Mercy Hospitals and Clinics, Hematology Oncology, ation avoidance in 63% of the patients, with 60% of them alive at last
Kansas City, United States of America; 23 University Hospital Motol, Hema- follow-up.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S29 of S635

PROS >54Gy, 4-year OS and EFS was 91% v 89% (OS, p=0.73) and 70% v 77%
(p=0.72). In patients with NTR/STR by < 54Gy and >54Gy, 4-year OS
PROS: FREE PAPER SESSION 2 29-09-2022 1:30 PM - 3:30 PM and EFS was 100% v 56% (p<0.001) and 82% v 46% (p=0.01).
Conclusions: No dose response between 50.4 Gy and 59.4 Gy for dis-
O059 / #1991 DOSE-RESPONSE IN PEDIATRIC ease control was found in patients with GTR. In patients with NTR/STR,
EPENDYMOMA: A PEDIATRIC PROTON/PHOTON CONSORTIUM higher dose correlated with worse outcomes, but this is likely due to
REGISTRY (PPCR) STUDY clinicians choosing to dose escalate in patients with greater amounts
of residual disease.
Torunn Yock1 , Arnold Paulino2 , William Hartsell3 , Victor Mangona4 ,
Nadia Laack5 , Stephanie Perkins6 , Christine Hill-Kayser7 , Young
Kwok8 , Nicholas Denunzio9 , Ralph Ermoian10 , Iain Macewan11 , John O060 / #806 HEARING IMPAIRMENT IN PAEDIATRIC AND
Perentesis12 , Bree Eaton13 , John Chang14 , Joshua Palmer15 , Benjamin TEENAGE YOUNG ADULT CANCER PATIENTS TREATED WITH
Bajaj1 , Miranda Lawell1 , Sara Gallotto1,2 , Danny Indelicato16 PROTON BEAM THERAPY TO THE BRAIN, BASE OF SKULL OR
1 Massachusetts General Hospital, Harvard Medical School, Department HEAD AND NECK
Of Radiation Oncology, Boston, United States of America; 2 MD Anderson
Cancer Center, Radiation Oncology, Houston, United States of Amer- Eunji Hwang1,2,3 , Simona Gaito3,4 , Marianne Aznar4,5 , Anna France3 ,
ica; 3 Northwestern Medicine Chicago Proton Center, Radiation Oncology, Peter Sitch6 , Adrian Crellin4,7 , David Thwaites2,8 , Verity Ahern1,9 ,
Chicago, United States of America; 4 Texas Center for Proton Therapy, Radi- Danny Indelicato10 , Gillian Whitfield4,6 , Ed Smith3,4,6
ation Oncology, Irving, United States of America; 5 Mayo Clinic, Radiation 1 Crown Princess Mary Cancer Centre, Radiation Oncology, WESTMEAD,
Oncology, Rochester, United States of America; 6 Washington University, Australia; 2 University of Sydney, Medical Physics, Sydney, Australia; 3 The
Radiation Oncology, St. Louis, United States of America; 7 University of Christie Proton Beam Therapy Centre, Proton Clinical Outcomes Unit,
Pennsylvania, Radiation Oncology, philadelphia, United States of America; Manchester, United Kingdom; 4 University of Manchester, Division Of Clin-
8 University of Maryland, Radiation Oncology, Baltimore, United States of ical Cancer Science, School Of Medical Sciences, Manchester, United King-
America; 9 Procure Proton Therapy Center, Radiation Oncology, Princeton, dom; 5 The Christie NHS Foundation Trust, Radiotherapy Related Research,
United States of America; 10 University of Washington, Radiation Oncol- Manchester, United Kingdom; 6 The Christie NHS Foundation Trust, Pro-
ogy, Seattle, United States of America; 11 California Proton Center, Radiation ton Beam Therapy, Manchester, United Kingdom; 7 NHS England, National
Oncology, San Diego, United States of America; 12 Cinncinati Children’s Clinical Lead Proton Beam Therapy, Redditch, United Kingdom; 8 Leeds Uni-
Hospital Medical Center, Oncology, Cincinnati, United States of America; versity, Medical Physics, Leeds, United Kingdom; 9 University of Sydney,
13 Emory University, Radiation Oncology, Atlanta, United States of Amer- Westmead Clinical School, Westmead, Australia; 10 University of Florida,
ica; 14 Oklahoma Proton Therapy Center, Radiation Oncology, Oklahoma Radiation Oncology, Jacksonville, United States of America
City, United States of America; 15 Ohio State University, Radiation Oncol-
ogy, Columbus, United States of America; 16 University of Florida, Radiation Background and Aims: Hearing impairment in the paediatric and
Oncology, Jacksonville, United States of America teenage young adult (TYA) population can have significant detrimen-
tal long-term impacts on learning, communication and social skills. This
Background and Aims: There exists controversy about whether dose report aims to detail the incidence and risk factors for moderate to
escalation improves disease control in pediatric ependymoma. severe hearing impairment (HI) in paediatric and TYA patients treated
Methods: We queried the Pediatric Proton/photon Consortium Reg- with proton beam therapy (PBT) for malignancies of the brain, base of
istry (PPCR) for ependymoma patients. 425 patients were iden- skull (BoS) or head and neck (H&N) via the United Kingdom’s Proton
tified. Patients were excluded for lack of follow-up, initial treat- Overseas Programme (POP).
ment for recurrent disease, M+ disease, or data inconsistencies. OS Methods: All paediatric (<16 years) and TYA (<25 years) patients
and EFS were calculated using the Kaplan-Meier method compared treated via the POP between 2008 and September 2020 were
with log-rank test. Dose groups of <=54 Gy and 54+ Gy were included. Clinical and treatment-related data were extracted from the
used. UK national PBT database. Moderate to severe toxicities (≥G3 as per
Results: 220 patients comprise the study cohort with a median follow CTCAE v4) were recorded, and counted only when of new onset or
up of 3.7 years. 61%, 34%, and 5% had anaplastic, classic, and myx- increased severity following PBT compared to baseline.
opapillary histology respectively. Dose range used was 50.4 Gy to 59.4 Results: 605 paediatric and 116 TYA patients were treated for tumours
Gy. 75%, 16%, and 9.5% had a GTR, NTR or STR respectively. 99% of of the H&N (49%), supratentorial brain/BoS (30%) and infratentorial
patients received proton RT. Median time to recurrence was 1.4 years brain (22%). The most common diagnoses were ependymal tumours
for the cohort and 1.9, 1.4 and 0.8 patients with GTR, NTR, or STR. 4- (21%) and rhabdomyosarcoma (19%). The incidence of G3 and G4
year OS and EFS rates for patients with a GTR v NTR/STR were 90% HI was 2.4% and 0.4% respectively. 19 of the 20 patients with HI
and 77% (OS, p=0.01) and 72% and 63% (EFS, p=.08). In the whole were paediatric. Patients <5 years had the highest relative rate of HI
cohort by < 54Gy and >54Gy, 4-year OS was 93% v 79% (p=0.03) and (6.2%, N=11/179) compared to those >5 years (1.9%, N=8/426). The
4-yr EFS was 72% v 68% (p=0.20). In patients with GTR by < 54Gy and rate of HI was highest for infratentorial brain tumours (4.5%), which
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S30 of S635 ABSTRACTS

had a higher prevalence of patients <5 years (N=74/157, 47.1%), the (range, 0.4-8.2 years) and 2.0 years (range, 0.06-7.5 years), respectively.
majority (N=71/74, 95.9%) of whom had a diagnosis of embryonal or Twenty-five patients developed disease progression (systemic in 14,
ependymal tumours. Prescribed dose did not correlate with HI. local in nine, local and systemic in two patients). Twenty-two patients
Conclusions: The incidence of HI in the paediatric and TYA patients deceased, 19 due to tumor progression. The estimated 2-years PFS and
treated for malignancies of the brain, BoS and H&N via the POP is low. OS were 53.6% and 71.0%, respectively. Late radiation-associated tox-
Paediatric, particularly those <5 years of age may be at highest risk of icity ≥ Grade 3 concerned the central nervous system (n=4), general
developing radiotherapy related HI. Cochlear radiotherapy doses and status (n=3), and eye disorders (n=1).
cumulative platinum doses are required to develop models predicting Conclusions: Survival in patients with AT/RT after PBT was promis-
for HI thresholds. ing when compared to the literature. Treatment was well tolerated.
Our data deserves confirmation in larger cohorts with longer follow-
up.
O061 / #443 PROTON BEAM THERAPY IN CHILDREN WITH
ATYPICAL TERATOID RHABDOID TUMORS – PROSPECTIVE
SINGLE-INSTITUTION ANALYSES OF FEASIBILITY AND O062 / #701 TOLERANCE OF BRAIN AND BRAINSTEM
OUTCOME DOSE IN CHILDREN WITH PROTON BEAM RE-IRRADATION IN
RELAPSED TUMOURS OF THE CENTRAL NERVOUS SYSTEM
Sarah Peters1 , Sabine Frisch1 , Danny Jazmati1 , Christian Bäumer2 ,
Christoph Blase3 , Stephan Tippelt4 , Michael C. Frühwald5 , Beate Katarzyna Latocha1 , Sabine Frisch1 , Dirk Geismar1,2 , Catia Barreira3 ,
Timmermann6 Stephan Tippelt4 , Gudrun Fleischhack4 , Beate Timmermann1,2
1 University Hospital Essen, Department Of Particle Therapy, West German 1 University Hospital Essen, Department Of Particle Therapy, West German
Proton Therapy Centre Essen (wpe), West German Caner Center (wtz), Essen, Proton Therapy Centre Essen (wpe), West German Caner Center (wtz), Essen,
Germany; 2 University Hospital Essen, West German Proton Therapy Cen- Germany; 2 German Cancer Consortium, Partner Site Essen, Essen, Germany;
tre Essen, Essen, Germany; 3 AnästhesieNetz Rhein-Ruhr, Westenfelderstr. 3 University Hospital Essen, West German Proton Therapy Centre Essen
62/64, Bochum, Germany; 4 University Hospital Essen, Paediatrics Iii, Pae- (wpe), Essen, Germany; 4 University Hospital Essen, Paediatrics Iii, Paediatric
diatric Haematology And Oncology, Essen, Germany; 5 University Medical Haematology And Oncology, Essen, Germany
Center Augsburg, Paediatrics And Adolescent Medicine, Swabian Children’s
Cancer Center, Ausgburg, Germany; 6 University Hospital Essen, Depart- Background and Aims: Re-irradiation (RT2) in recurrent tumours of
ment Of Particle Therapy, West German Proton Therapy Centre Essen (wpe), the central nervous system (CNS) raises the question of the tolerance
West German Caner Center (wtz), German Cancer Consortium (dktk), Essen, of the brain and brainstem to cumulative RT doses. We analyzed the
Germany outcome after RT2 with protons.
Methods: Data of patients re-irradiated between 08/2015 and
Background and Aims: Despite intensive trimodality treatment prog- 07/2019 was collected within a prospective registry study. Adverse
nosis of atypical teratoid/rhabdoid tumor (AT/RT) remains dismal. Pro- events were documented according to CTCAE V4.0. Performance sta-
ton beam therapy (PBT) is a radiotherapy modality achieving superior tus was assessed with Lansky Play-Performance Scale. Composite
dose distributions, particularly promising in children. We evaluated plans were created using the treatment planning system RayStation®.
outcome and late toxicity after PBT in patients with central nervous Association of adverse events and cumulative doses to brain and
system AT/RT. brainstem was analyzed.
Methods: Fifty-one patients (25 male, 26 female) with AT/RT were Results: Forty-one children (median age 9 years) were analyzed.
treated between 11/2013 and 01/2021. Patients were enrolled to a Histopathologies were ependymal tumours (n=27), embryonal
prospective in-house register. We classified adverse events accord- tumours (n=8), and other entities (n=6). The median prescription dose
ing to CTCAE V4.0. Overall survival (OS) was calculated from first was 55 Gy in RT1 and 54 Gy in RT2. The median time interval between
diagnosis, progression-free survival (PFS) from the end of PBT. irradiations was 29 months. Median follow-up (FU) time after RT2 was
Results: Median age at diagnosis and RT was 1.7 years (range, 0.2- 20 months. Twenty-nine patients experienced disease progression
7.5 years) and 2.2 years (0.9-7.9 years), respectively. Eight patients with a median time to progression of five months after RT2 (systemic
presented with metastatic disease. Patients with local disease were in 14, local in 9, combined failure in 6 patients). Eighteen patients
treated locally (n=43), while patients with metastatic disease received died, 15 due to tumour progression. In 31 patients with DICOM
craniospinal irradiation (CSI) followed by a boost to the tumour bed plan files of RT1, a composite plan was calculated. The cumulative
(n=8) and an additional boost to distant metastases (n=3). A median median Dmean was 41 Gy to the brain and 62 Gy to the brainstem.
total dose of 54.6 Gy(RBE) (range, 45.0-60.0 Gy(RBE)) was delivered The cumulative Dmean to brainstem significantly correlated with
in a median of 31 fractions. Median CSI dose was 24.0 Gy(RBE) (range, grade of late neurological toxicities (Spearman correlation coefficient
23.4-35.2 Gy(RBE)) applied in median 15 fractions (range, 13-22(RBE)). 0.489 (p=0.024)). Majority of the surviving patients had normal
Cumulative dose to metastases were 45.6-49.6 Gy(RBE). Median activity scores (80-100 on Lansky Scale) at last FU. Newly occurred,
follow-up time since first diagnosis and end of PBT was 2.9 years late, high grade adverse events (Grade >=3) concerning nervous
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S31 of S635

system, psychiatric and general disorders were observed in nine be minimized for the lower spine. To ensure the adequacy
patients. of 3 mm target margin or plan robustness, motion detection
Conclusions: Re-irradiation with protons for recurrent CNS tumours and/or effective immobilization is critical particularly for awake
was well feasible. Studies in larger patient cohorts and longer FU are patients.
needed to fully explore re-irradiation in relapsed CNS tumors and the
reasons for long-term toxicities after multimodal relapse therapy.
O064 / #1692 INCIDENCE AND CLINICAL CHARACTERISTICS
OF CEREBROVASCULAR EVENTS AMONG LONG-TERM
O063 / #776 PROSPECTIVE EVALUATION OF SETUP CHILDHOOD CANCER SURVIVORS TREATED WITH UPPER
UNCERTAINTY AND INTRAFRACTIONAL MOTION IN BODY RADIATION THERAPY IN THE DCCSS LATER COHORT
PEDIATRIAC PATIENTS TREATED WITH CRANIOSPINAL
IRRADIATION: GUIDING TARGET MARGIN/ROBUSTNESS Lisanne Verbruggen1 , Judith Kok1 , Leontien Kremer1,2,3 , Geert
DESIGN AND IMAGING/TREATMENT SEQUENCING Janssens4,5 , Paul Nederkoorn6 , A. Birgitta Versluijs7 , Andrica De
Vries1,8 , Ardine Reedijk1 , Dorine Bresters1 , Eelco Hoving1 , Eline Van
Chia-Ho Hua1 , Andrew Saunders1 , Tyler Russell1 , Jared Becksfort1 , Dulmen-Den Broeder9 , Jacqueline Loonen10 , Judith De Bont1 , Joyce
Matthew Marker2 , Jinsoo Uh1 , Kavitha Raghavan3 , Matthew Krasin1 , Wilbers11 , Marloes Louwerens12 , Margriet Van Der Heiden-Van Der
Thomas Merchant1 Loo13 , Marry Van Den Heuvel-Eibrink4,14 , Sebastian Neggers15 , Wim
1 St. Jude Children’s Research Hospital, Department Of Radiation Oncol- Tissing16,17 , Cecile Ronckers1,18 , Jop Teepen1 , Helena Van Der Pal1
ogy, Memphis, United States of America; 2 St. Jude Children’s Research 1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology,
Hospital, Clinical Trials Administration, Memphis, United States of Amer- Utrecht, Netherlands; 2 University Medical Center Utrecht, Wilhelmina
ica; 3 St. Jude Children’s Research Hospital, Department Of Anesthesiology, Children’s Hospital, -, Utrecht, Netherlands; 3 Emma Children’s Hospital,
Memphis, United States of America Amsterdam UMC, University of Amsterdam, Pediatrics, Amsterdam, Nether-
lands; 4 Princess Máxima Center for Pediatric Oncology, Division Of Pedi-
Background and Aims: To quantify patient position uncertainty in chil- atric Oncology, Utrecht, Netherlands; 5 University Medical Center Utrecht,
dren treated with craniospinal irradiation (CSI) using volumetric image Radiation Oncology, Utrecht, Netherlands; 6 Amsterdam UMC location
guidance and improve patient immobilization and target margin/plan University of Amsterdam, Neurology, Amsterdam, Netherlands; 7 Princess
robustness design. Máxima Center for Pediatric Oncology, Research, Utrecht, Netherlands;
Methods: During 2020-2021, 52 patients (aged 2-22 years, median 8 Sophia Children’s Hospital/Erasmus Medical Center, Department Of Pae-
11 years; 40 with intravenous propofol anesthesia) received proton diatric Oncology, Rotterdam, Netherlands; 9 Amsterdam UMC location Vrije
CSI and protocol-specified imaging for multiple treatment isocen- Universiteit Amsterdam, Pediatrics, Amsterdam, Netherlands; 10 Radboud

ters including daily pre-treatment cone-beam CT (CBCT), verification University Medical Center, Department Of Haematology, Nijmegen, Nether-
CBCT after implementing setup corrections with a 6-degrees-of- lands; 11 Radboud university medical center, Center Of Expertise For Cancer
freedom robotic couch, and post-treatment CBCT. Patients were Survivorship, Nijmegen, Netherlands; 12 Leiden University Medical Center,
positioned supine on an indexed range shifter board above the couch Department Of Internal Medicine, Leiden, Netherlands; 13 Dutch Childhood
with legs in a vacuum bag over the knee sponge and immobilized with Oncology Group, Childhood Oncology, Utrecht, Netherlands; 14 Sophia Chil-
custom head and neck support and thermoplastic masks. Therapists drens Hospital, Erasmus Medical Center, Pediatric Oncology, Rotterdam,
positioned patients based on 3-point markings on mask, tattoos at the Netherlands; 15 Erasmus Medical Center, Department Of Internal Medicine,
xiphoid and pelvis, and sagittal alignment marks on the vacuum bag. Section Endocrinology, Rotterdam, Netherlands; 16 Princess Maxima Center
Results: The 95th percentile of residual setup errors in 3D vectors, for Pediatric Oncology, Supportive Care, Utrecht, Netherlands; 17 University
calculated from post-correction verification CBCT, were 1.8 mm and of Groningen, Department Of Pediatric Oncology, Groningen, Netherlands;
2.1 mm for CSI cranium and spine (including upper, lower, and entire 18 Carl v Ossietzky University of Oldenburg, Department Of Health Services

spine isocenters), respectively, for the entire cohort and 1.4 mm and Research, Oldenburg, Germany
1.9 mm for the anesthetized subgroup. Post-treatment position errors
increased to 2.6 and 4.2 mm (all) and 2.4 and 3.6 mm (anesthetized). Background and Aims: Cerebrovascular events (CVEs) are serious
Intrafractional movement was largest in lower spine and smallest in late adverse events among childhood cancer survivors (CCS) and radio-
cranium for both anesthetized and awake patients. Anesthetized young therapy is a prominent risk factor. The objectives of this study are
children treated with a single-isocenter whole spine maintained a high to estimate the incidence and risk factors of symptomatic CVEs and
position accuracy throughout the treatment (95th percentile<1.5 mm to describe the clinical characteristics and outcome among CCS after
in cranium and spine). A high accuracy was also observed for the cra- upper body radiotherapy.
nium boost (post-treatment 95th percentile=1.9 mm for all and 1.3 mm Methods: The Dutch Childhood Cancer Survivor Study LATER cohort-
for anesthetized). study includes CCS diagnosed ≤age 18 years between 1963-2002 and
Conclusions: Moderate motion exists even under anesthesia. For survived ≥5 year after diagnosis. Data about CVEs were retrieved
lengthy CSI, time between imaging and beam delivery should from medical records of eligible survivors treated with upper body
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S32 of S635 ABSTRACTS

radiotherapy (n=1,633). Multivariable Cox regression models were for protons. Resubmission for vertebral delineation or dose issues was
used to identify potential risk factors for developing a symptomatic required for 20% of cases. Approximatively 70% of VBs delineations
CVE. include spinal processes and spinal canal. When re-delineated, only
Results: In 107 CCS a CVE emerged after a median latency time of 14% of the VBs_Adj met the dosimetry constraints.
26.1 years and at a median age of 35.5 years. Within 40 years after Conclusions: Despite the availability of international consensus guide-
diagnosis with childhood cancer, 1 in 11 survivors previously treated lines, a high variability in vertebrae delineation is observed in QUAR-
with upper body radiotherapy developed a CVE. A cranial radiother- TET cases, with impact on dosimetry. Better vertebral sparing was
apy prescription dose >50 Gy was associated with a 6-fold increased observed in photon cases. Guidelines will be amended to reflect these
risk of CVE, compared to those with radiotherapy directed to the results and further improve compliance.
neck/thorax/spine only (HR=6.3, 95%CI: 3.28-12.14). Among the 107
CVE cases, 17 (16%) had a life-threatening situation and two (2%) other
died during hospital admission. In 28%, a second CVE developed during O066 / #190 RECALL REACTION IN CHILDREN
follow up. At the end of follow-up, 29% of CVE cases were deceased
and among those still alive, 40% were unable to carry out normal Cristina Larrosa1 , Soraya Mico Milla2 , Monica Sanchez Celma3 , Alicia
activities or do active work. Castañeda Heredia1 , Monica Ramos Albiac2 , Moira Garraus1
Conclusions: CCS treated with higher doses of cranial radiotherapy 1 Pediatric Cancer Center, Sant Joan de Déu Hospital, Pediatric Oncology,
are at highest risk for developing CVEs. CVEs among CCS occur at Esplugues Llobregat, Spain; 2 Hospital Vall D’Hebron, Oncology Radiother-
young age and cause a high morbidity. There is a clinical need to inves- apy Department„ Barcelona, Spain; 3 Sant Joan de Déu Hospital, Pediatric
tigate how to reduce the risk of CVEs among CCS treated with cranial Oncology Pharmacy, Barcelona, Spain
radiotherapy, thus high-quality studies are warranted.
Background and Aims: Radiation recall reaction is a rare phenomenon
in which an inflammatory reaction occurs in a previously irradiated
O065 / #714 THE VERTEBRAE AS OARS IN PAEDIATRIC field after a triggering agent. It is poorly understood, and deficiently
RADIOTHERAPY CLINICAL TRIALS studied in the paediatric population. Gemcitabine-docetaxel (G/D) is
used in paediatrics as a maintenance or as salvage therapy in high risk
Andrada Turcas1,2 , Sarah Kelly1,2 , Tom Boterberg3 , Henry Mandeville4 sarcomas. This study aims to collect data from pediatric patients who
1 SIOP Europe, Quartet, Brussels, Belgium; 2 EORTC, Rtqa, Brussels, Belgium; presented with recall phenomenon after receiving G/D and to analyze
3 Faculty of Medicine and Health Sciences, Ghent University, Ghent, Bel- possible triggering factors and clinical outcome.
gium, Department Of Human Structure And Repair, Ghent, Belgium; 4 Royal Methods: Retrospective unicentric cohort study of 25 episodes from
Marsden Hospital, Radiotherapy Department, Sutton, United Kingdom 21 patients who received G/D after radiation therapy from 2010-2021.
Results: The majority of patients from our sample (76.2%) presented
Background and Aims: Irradiation of the vertebrae in prepubertal with recall reaction, involving mainly deep tissues beyond dermis
patients can result in growth defects and deformities, related to inho- (58%) and occurring predominantly after 3rd cycle of chemotherapy.
mogeneities in dose distribution. In 2019 the SIOPEROWG published a Comparing the recall and non-recall group the mean time between
consensus on vertebral delineation and constraints in an effort to stan- radiotherapy and chemotherapy was 30,5 days (25;1359) vs 79 days
dardise practice across Europe. QUARTET is a SIOPe project for RTQA (59;364) (p=0,15) and radiation volume 378ml versus 250ml (p=0,27),
in Paediatric Clinical Trials across Europe. All trials incorporate specific respectively. Recall treatment was mostly based on systemic steroids
guidelines which include recommendations on vertebral delineation but mainly partial responses were reported (58%). Re-exposure was
and dosimetry as per the 2019 ROWG consensus. We performed an associated with recurrence (64%), forcing to drug discontinuation.
audit of the first cases enrolled in the trials using QUARTET for RTQA, Conclusions: This study represents a unique series of recall reaction
aiming to identify the main protocol deviations in vertebral delineation in children, restrained to the use of gemcitabine-docetaxel combina-
and their impact on dosimetry. tion. Incidence was higher than previously reported; no risk factors
Methods: All cases undergoing QUARTET review until 22/02/2022 were identified except a greater irradiated field and a shorter inter-
were evaluated. Prepubertal patients with vertebrae in the proximity val between chemotherapy and radiotherapy. Symptomatic treatment
of the target volume were included. Vertebrae adjacent to the target does not resolve the episode and re-exposure is frequently associated
volume (VBs_Adj) and directly cranial and caudal (VB_NAdj_S/I) were with recurrence.
assessed for compliance to delineation and dosimetry guidelines by
a second, independent reviewer. Missing or non-compliant structures
were recontoured and the new DVH metrics were compared with the O067 / #1221 ANALYSIS OF DOSE-TOXICITY PROBABILITY
initial ones. FOR ANTERIOR PITUITARY INSUFFICIENCY IN LONG TERM
Results: VB_NAdj_S/I were not delineated in 49% of the cases. Dose SURVIVORS OF PEDIATRIC HEAD AND NECK
constraints for VBs _Adj were met in 31% of initial submissions. RHABDOMYOSARCOMA
Dosimetry compliance for VB_NAdj_S/I was 53% for photons and 16%
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S33 of S635

Michèle Morfouace1 , Marinka Hol2,3 , Reineke Schoot1 , Jan Wiersma4 , and quantify the probability of expected clinical toxicity for different
Olga Slater5 , Henry Mandeville6 , Raquel Dávila Fajardo7 , Mark Gaze8 , treatment techniques.
Hans Merks1 , Ludi Smeele3,9 , Brian Balgobind4 , Bradley Pieters4 ,
Arjan Bel4 , Hanneke Van Santen1,10 , Danny Indelicato11
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, O068 / #614 SELF-REPORTED HEALTH SURVEY RESPONSE
Utrecht, Netherlands; 2 University Medical Center Utrecht, UMC Utrecht OUTCOMES IN A LARGE COHORT OF PEDIATRIC ONCOLOGY
Brain Center, Department Of Otorhinolaryngology-head And Neck Surgery, PATIENTS TREATED WITH RADIOTHERAPY
Utrecht, Netherlands; 3 Princess Máxima Center for Pediatric Oncology,
Head And Neck Surgery, Utrecht, Netherlands; 4 Amsterdam University Melanie Rose1 , Miranda Lawell2 , Jessica Marinelli2 , Megan Upton2 ,
Medical Centers, Cancer Center Amsterdam, University of Amsterdam, Tristin Flood2 , Benjamin Bajaj2 , Torunn Yock2
Department Of Radiation Oncology, Amsterdam, Netherlands; 5 Great 1 Dartmouth-Hitchcock Medical Center, Radiation Oncology Residency Pro-

Ormond Street Hospital for Children NHS Foundation Trust, Pediatric gram, Lebanon, United States of America; 2 Massachusetts General Hospital,
Oncology, London, United Kingdom; 6 Royal Marsden Hospital, Radio- Harvard Medical School, Department Of Radiation Oncology, Boston,
therapy Department, Sutton, United Kingdom; 7 University Medical Cen- United States of America
ter Utrecht, Department Of Radiation Oncology, Utrecht, Netherlands;
8 University College London Hospitals NHS Foundation Trust, Oncology, Lon- Background and Aims: Over 80% of pediatric patients that receive
don, United Kingdom; 9 Amsterdam University Medical Centers, location radiotherapy (RT) at Massachusetts General Hospital (MGH) are
University of Amsterdam, Department Of Maxillofacial Surgery, Amster- referred from an outside institution and return to their home insti-
dam, Netherlands; 10 Wilhelmina Children’s Hospital, University Medical tution for care. As quaternary care centers, proton centers are par-
Center Utrecht, Department Of Pediatric Endocrinology, Utrecht, Nether- ticularly challenged in obtaining follow-up. Longitudinal patient data
lands; 11 UF Health Proton Therapy Institute, University of Florida, Radiation is vital to evaluate RT outcomes. To improve follow-up collection, we
Oncology, Jacksonville, United States of America implemented an annual direct-to-patient survey.
Methods: Institutional approval was obtained to send surveys to MGH
Background and Aims: One third of pediatric head and neck rhab- participants prospectively enrolled on a clinical trial or the Pediatric
domyosarcoma (HNRMS) survivors develop anterior pituitary insuffi- Proton/Photon Consortium Registry. The survey can be completed
ciency (API). API is associated with multiple health impairments and in <5-minutes and includes verifying/updating contact information,
reduced quality of life. Knowledge on radiation dose tolerance of the health status (recent follow-up and with which specialist(s), imaging,
associated organs at risk (OAR) – the pituitary gland (PG) and hypotha- status of treated disease/secondary tumors, additional treatments,
lamus (HT) - can help prevent API for future patients. Current proposed symptoms), and social updates. Surveys were sent annually from the
dose thresholds to the PG and/or HT vary widely and are based on end of RT either as a mailed letter with a QR code or by email via
research conducted in survivors of brain tumors, who receive differ- REDCap. For emailed surveys, an automatic reminder was sent after
ent radiation doses and different chemotherapy. We aim to describe two-weeks if no response was received. The date of the patient’s last
the specific dose-toxicity relationship for API in long-term HNRMS available clinical status was recorded at time of survey distribution.
survivors. Results: Data was collected between 2/2019-12/2021. Of 479 par-
Methods: Presence of API was assessed by Common Terminology Cri- ticipants sent at least one survey, 236 (49.3%) responded. Patients
teria for Adverse Events grading, biochemical testing, anthropometrics distributed surveys via email were 1.6 times as likely to respond
and chart review in a multicenter cross-sectional cohort study where than patients mailed surveys (p<0.001). Median time (days) to survey
survivors with ≥2 years follow up without recurrent disease were completion for mailed and emailed surveys were 20 and 3, respec-
examined in multidisciplinary clinics. The PG and HT were delineated tively. Survey completion extended the last available clinical status
on 3D radiotherapy planning CTs. Dosimetric data were collected from on record for patients by a median of 9.2 months. Of participants
the original radiotherapy treatment plans (both external beam radio- that responded, 91.7% reported being seen by a clinician in the past
therapy with photons or protons, and brachytherapy). Dose-toxicity year.
probability curves were constructed using binary logistic regression. Conclusions: Survey implementation improved follow-up data collec-
Results: Thirty-eight HNRMS survivors with a median follow-up of 9 tion, with email being more effective than mail as a distribution method.
years (range 2 – 27) were included. Survivors with API received a sta- Approval was recently obtained to add the option of text messaging
tistically significantly higher mean dose to the PG, but not to the HT, and expand the cohort to include all pediatric patients that received RT,
compared to survivors without API. Mean dose >15.4 GyEQD2 to the regardless of past study enrollment.
PG was identified as threshold dose for an increased probability of
API development (sensitivity 83%, specificity 84%). No multivariable
analyses were conducted due to small sample size. O069 / #939 NEUROCOGNITIVE OUTCOMES AFTER
Conclusions: We determined mean dose >15.4 GyEQD2 to the PG as PROTON BEAM THERAPY TO THE BRAIN IN PAEDIATRIC,
threshold dose for an increased risk of API in HNRMS survivors. Our TEENAGERS AND YOUNG ADULTS TUMOURS
results can help to avoid API by ways of avoiding radiation damage
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S34 of S635 ABSTRACTS

Simona Gaito1,2 , Eunji Hwang1,3,4 , Marianne Aznar2 , Anna France1 , Ghufran Aljawi1 , Fuchsia Howard2 , Isabel Serrano Martinez1 , Sara
Peter Sitch5 , Adrian Crellin2,6 , Danny Indelicato7 , Shermaine Pan5 , Mahdavi1 , Luminata Nica1 , Sandy Chang1 , Angie Xiong3 , Andrea Lo1 ,
Gillian Whitfield2,5 , Ed Smith1,2,5 Karen Goddard1
1 The Christie Proton Beam Therapy Centre, Proton Clinical Outcomes 1 BC Cancer Agency, Radiation Oncology, Vancouver, Canada; 2 University of

Unit, Manchester, United Kingdom; 2 University of Manchester, Division Of British Columbia, Applied Science/nursing, Vancouver, Canada; 3 University
Clinical Cancer Science, School Of Medical Sciences, Manchester, United of British Columbia, Faculty Of Family Practice, Vancouver, Canada
Kingdom; 3 Crown Princess Mary Cancer Centre, Radiation Oncology, WEST-
MEAD, Australia; 4 University of Sydney, Medical Physics, Sydney, Australia; Background and Aims: Adolescent and young adults (AYA) central
5 The Christie NHS Foundation Trust, Proton Beam Therapy, Manchester, nervous system (CNS) tumor survivors are at risk for chronic health
United Kingdom; 6 NHS England, National Clinical Lead Proton Beam Ther- problems and late effects (LEs) and this study was conducted to
apy, Redditch, United Kingdom; 7 University of Florida, Radiation Oncology, determine that burden of LEs.
Jacksonville, United States of America Methods: We invited CNS tumor survivors aged between 15 and 39
at diagnosis treated with radiotherapy between 1986 and 2008 in
Background and Aims: Evidence suggests that Proton Beam Ther- British Colombia and surviving at least 5 years post treatment for
apy (PBT) may lessen the risk of neurocognitive decline (NCD) as reassessment. Detailed previous therapy information was collected.
compared to conventional photon radiotherapy (XRT). We report the We assessed for the presence of LEs using history, examination and
incidence of moderate-severe (Grade ≥3) NCD in paediatric, Teenage investigations.
and Young Adult (TYA) patients treated for brain tumours within the Results: Thirteen AYA survivors identified; 69.2% were females and
Proton Overseas Programme (POP). 30.8% males. Age ranged from 16 to 30 (median 25). Median follow
Methods: Baseline (pre-PBT) and follow-up clinical outcomes up was 28 years (range 13 to 39). Survivors received radiotherapy for
data were prospectively collected as part of a national PBT- different CNS pathologies; glioma 23.07.% (3/13), pituitary adenoma
outcomes registry, started in 2008. This study focuses on the 23.07% (3/13), arterial-venous malformations 23.07% (3/13), ependy-
incidence of Grade (G) ≥3 cognitive disturbance (CD) as per moma 15.38% (2/13), intracranial neurocytoma 7.69% (1/13) and
CTCAE (Common Terminology Criteria for Adverse Events) germinoma 7.69% (1/13). Many participants (46.2%, 6/13) reported
toxicity grading scale v4.0 in the paediatric/TYA (< 25 years) chronic headaches post treatment, 61.5% (8/13) had visual problems
cohort. and 38.5% (5/13) had hearing loss. Out of the 9 female survivors, 44.4%
Results: Between 2008-2018, 560 patients < 25 years old were (4/9) had ovarian failure. Learning disabilities were found in 76.9%
treated for brain tumours within the POP. Median age at treatment (10/13); memory (90.0%, 9/10) and executive function (70.0%, 7/10)
was 9 years (1-24). Median prescribed dose was 54GyRBE (34.8- were the commonly affected domains. There were 69.2% (9/13) with
79.2). The commonest diagnoses were Ependymoma (29.6%), followed psychiatric illnesses (depression 100% (9/9) and 44.4% (4/9) anxiety).
by Craniopharyngioma (22%) and Astrocytoma/Oligodendroglioma After recall, 50.0% (6/13) found to have hormone deficiencies and
(18.4%). After a median follow up of 31 months (0-132), 8 patients 50% (3/6) of those developed hypothyroidism. Metabolic syndrome
were reported to experience Grade 3 CD on clinical assessment. was common 46.2% (6/13) had hyperlipidemia, 30.8% (4/13) had
Five of these 8 had some form of neurological deficit at base- glucose intolerance and 54.5% (6/13) required referral to endocrinol-
line, such as visual impairment, speech disturbance or developmental ogy. Screening for survivors who had brain radiotherapy detected
delay. 50% (6/12) thyroid nodules on ultrasound and 25.0% (3/12) radiation
Conclusions: Our results indicate that paediatric/TYA patients under- induced meningioma on magnetic resonance imaging.
going radiotherapy to the brain may experience detrimental effects on Conclusions: AYA survivors face multiple, significant LEs and long-term
cognition. However, the majority had neurological deficit at baseline follow-up is crucial. It is critical to improve links between oncolo-
resulting from tumour, events leading to diagnosis and/or earlier sur- gists and primary care practitioners to facilitate optimal community
gical intervention. Neurocognitive testing has been part of the routine survivorship care.
baseline and follow-up assessment in this patient population since the
establishment of the NHS PBT facilities in UK. Larger cohorts are war-
ranted to establish the role of other risk factors, and aid identification IPSO
of which critical structures most impact specific domains of cognitive
function. IPSO FPS 4 INVESTIGATION, SURGICAL TECHNIQUE AND
LOCALIZATION OF PAEDIATRIC TUMOURS 29-09-2022 1:30 PM
- 3:30 PM
O070 / #371 LATE EFFECTS ASSESSMENT IN ADOLESCENT
AND YOUNG ADULT CENTRAL NERVOUS SYSTEM TUMOR O071 / #21 ULTRASOUND ELASTOGRAPHY IN THE
SURVIVORS TREATED WITH RADIOTHERAPY. A DIAGNOSIS OF MALIGNANT CERVICAL LYMPHADENOPATHY
CROSS-SECTIONAL SURVEY IN CHILDREN: CAN IT REPLACE SURGICAL BIOPSY?
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S35 of S635

Ahmed Elgendy1 , Eslam Elhawary1 , Mohamed Shareef2 , Marwa Background and Aims: Paediatric Hodgkin Lymphoma (HL) is a major
Romeih3 , Ahmed Ebeed4 cause of morbidity and mortality in children. In low to middle-income
1 Tanta University, Surgical Oncology Unit, Tanta, Egypt; 2 Tanta Univer- countries like South Africa, there is a need to understand the clini-
sity, Pathology Department, Tanta, Egypt; 3 Helwan University, Radiology cal practices surrounding diagnosis and surveillance of HL to reduce
Department, Tanta, Egypt; 4 Kafr El-Sheikh University, Radiology Depart- the burden on health systems. Understanding the clinical utility of
ment, Tanta, Egypt PET/CT scans may decrease repeated tissue biopsies during disease
surveillance. This study aimed to compare the utility of the two
Background and Aims: Diagnostic imaging is essential in children investigations.
with unclear cause of cervical lymphadenopathy. Recently, ultrasound Methods: The study is a retrospective cohort study of patients aged
elastography was presented as a promising technology to distinguish less than 18 years, treated for Hodgkin Lymphoma at Chris Hani
between malignant and benign lymph nodes. We aimed to assess Baragwanath Academic Hospital from January 1st 2009 to December
the accuracy of elastography in detecting pediatric malignant cervi- 31st 2018. Ethics HREC No. M210732.
cal lymph nodes, and if this modality can obviate the need for surgical Results: Among the 54 patients included in the study, there was a
biopsies. male to female ratio of 5:1 and a mean age at diagnosis of 9 years
Methods: A prospective study from September 2017 to September old (σ: ±3.7). Nodular Sclerosing HL was the most frequent subtype at
2020 included 64 children with persistent cervical lymphadenopathy. diagnosis and relapse with frequencies of 42.59% (n=23) and 44.44%
Patients were evaluated by meticulous history and physical assess- (p=0.372) respectively. Seventy percent of patients (n=38) received a
ment. B-mode ultrasound, color doppler, and sonoelastography were PET/CT and tissue biopsy during their initial diagnostic workup, and
conducted thereafter. Elastography scans were classified into five pat- 37% (n=17) of patients received a PET/CT scan during surveillance.
terns, and patterns from 3-5 were considered as malignancies. All Tissue biopsy and PET/CT, showed moderate agreement of 48.2% (κ =
children underwent open biopsies followed by pathological exam- 0.14) in diagnosing relapsed disease during surveillance. The false neg-
ination. Results of tissue diagnosis were compared to patterns of ative rate for tissue biopsy during surveillance was 42.9%. No patients
elastography to determine its accuracy. had a negative PET/CT during surveillance with a positive tissue biopsy
Results: Twenty-eight patients (43.8%) had malignant nodes and the result (n=18).
remaining 36 (56.2%) were due to benign causes. Elastography pat- Conclusions: This study is the first cohort to explore the clinical util-
terns of 1-2 were documented in 30 patients, and all of them were ity of PET/CT scans and tissue biopsies in a low-resourced setting.
diagnosed as benign lesions. Patterns of 3-5 were demonstrated in 34 Our findings showed moderate agreement between the modalities
patients. Out of them, 28 were confirmed as malignancies, whilst 6 chil- in diagnosing relapsed disease during surveillance. A portion of this
dren were of benign nature (false-positive). All false-positive results discordance can be attributed to false negative tissue biopsy results.
occurred in pattern 3 of elastography. There were no complications While the sample is limited, our findings are consistent with the
encountered regarding surgical biopsies, and all patients were dis- high negative predictive value of PET/CT scans reported in the
charged on the same day of the procedure. Ultrasound elastography literature.
achieved 100% and 85.7% sensitivity and specificity, respectively, and
an overall accuracy of 90.6% in the differentiation between malignant
and benign entities. The overall accuracy of B-mode and color doppler O073 / #964 SAVING LINES, SAVING LIVES? CENTRAL
were 75% and 82.2%, respectively. VENOUS ACCESS DEVICES AND THEIR RISK FACTORS FOR
Conclusions: Elastography is a useful tool that should be added to FAILURE
ultrasound modalities and diagnostic algorithm for pediatric cervical
lymphadenopathy. Surgical biopsy in eligible patients is imperative to Laura Phillips1 , Chris Perry2 , Elizabeth O’Connor1 , Hany Gabra3
commence proper therapy or to discharge the child. Despite favorable 1 Great North Children’s Hospital, Paediatric Surgery, NEWCASTLE UPON
results of elastography, it cannot replace surgical biopsy or change its TYNE, United Kingdom; 2 Great North Children’s Hospital, Anaesthetics,
indications. Newcastle Upon Tyne, United Kingdom; 3 Great North Children’s Hospital,
Paediatric Surgery, Newcastle upon Tyne, United Kingdom

O072 / #1447 THE CLINICAL UTILITY OF PET/CT SCAN AND Background and Aims: Central venous access device (CVAD) insertion
TISSUE BIOPSY IN THE MANAGEMENT AND FOLLOW UP OF is one of the most common paediatric surgical procedures. Our aim was
PAEDIATRIC HODGKIN LYMPHOMAS IN SOUTH AFRICA to identify the failure rate and the associated risk factors in our centre.
Methods: A prospective audit was undertaken between June 2020-
Derek Harrison, Kelly Heyman, Gabriella Hyman, Kimon Nicolaides, December 2021 for CVAD insertions in our centre. Failure was defined
Jason Mcmaster, Raadiyyah Kolia, Vaishnavi Govender as both mechanical (block, break or dislodge) and infection, necessitat-
University of Witwatersrand, Paediatric Surgery, Johannesburg, South ing unplanned removal and calculated per 1000 line days. Statistical
Africa analysis was performed using Kaplan Meier survival and p value <0.05
was deemed significant.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S36 of S635 ABSTRACTS

Results: A total of 462 CVADs (301 patients) were inserted (234 Hick- utes in low-grade complicated children. Mean intraoperative blood loss
mans, 101 Portacaths, 98 Peripherally Inserted Central Catheters, 29 was more than 300 ml in grade 4 complicated children as compared to
Haemocaths). Chemotherapy was the most common indication for just 50 to 100 ml in low-grade complicated children. More than 50%
insertion (39%). The majority were USS guided percutaenous inser- of children with grade 4 complications had recurrence as compared to
tions (94.5%) with a mean age of 68(SD 63.5) months and weight children with lower grades of complications. Redo surgery was done in
of 22.2(SD 19.4) kg. The IJV was the most frequent vessel accessed 8% of children.
(68%) and most line tips were located at the SVC/RA junction (63.5%). Conclusions: Clavien – Dindo classification can be used in pediatric
The total number of line days was 88248, with an overall failure rate surgical oncology to assess postoperative complications in an objec-
of 1.6 per 1000 line days, of which 58.5% were due to mechanical tive way. Children with a higher grade of complications have increased
reasons (0.9 per 1000 line days). Portacaths were least likely to fail morbidity in form of longer hospital stays, blood transfusions, and a
(p<0.001). Increased rates of failure were associated with more lumens higher rate of redo operations. This also leads to subsequent delays in
(p<0.001), open insertion (p=0.036), emergency setting (p=0.042) and adjuvant treatment.
age less than 60 months (p=0.021). Weight was not a significant factor
(p=0.466).
Conclusions: We identified multiple factors associated with line fail- O075 / #834 EXPLORING THE FRONTIER IN ROBOTIC
ure. Mechanical failure represents a significant proportion of line PEDIATRIC CANCER SURGERY: WHEN TO MOVE FORWARD
failures in children, which historically may have been overlooked AND WHEN TO STOP
compared to infection. As expected, failure is higher in external
lines when compared to portacaths. Future consideration for stud- Federico Palo1 , Stefano Avanzini1 , Gerald Beaubrun1,2 , Ida
ies investigating the value of fixation devices and postoperative Barretta1,2 , Martina Monti1,2 , Michele Torre1 , Massimo Conte3 ,
care bundles to reduce potentially preventable causes for failure Girolamo Mattioli1,2
are important, alongside robust ways to capture and monitor this 1 IRCCS Istituto Giannina Gaslini, Pediatric Surgery, Genoa, Italy;
data. 2 University of Genoa, Dinogmi, Genoa, Italy; 3 IRCCS Istituto Giannina
Gaslini, Pediatric Oncology, Genoa, Italy

O074 / #1323 SURGICAL COMPLICATIONS IN PAEDIATRIC Background and Aims: Since its introduction in children in 2001,
SOLID TUMOURS, LESSONS LEARNT OVER 5 YEARS robotic surgery (RS) has been proposed by experienced pediatric
laparoscopic surgeons in a continuously expanding range of pro-
Arihant Jain1 , Nitin Peters2 , Ram Samujh2 , Muneer Malik2 cedures. The technical capabilities of the robot may be ideal for
1 Post graduate Institute of medical education and research, Paediatric complex pediatric surgical cases that require intricate dissection,
Surgery, Chandigarh, India; 2 PGIMER Chandigarh, Paediatric Surgery, such as in pediatric cancer surgery. Although the comfortable age
CHANDIGARH, India and weight for RS candidates are progressively lowering and new
potential indications are currently being explored, some limits must
Background and Aims: Postoperative complications in children with be acknowledged and possibly recognized during surgery, to avoid
solid tumors are a major cause of morbidity and mortality. The complications or emergency conversion. This study aims to investi-
Clavien – Dindo classification is validated across various surgical gate these issues through a retrospective analysis of a single-center
specialties and thus may be also used to objectify complications experience.
in pediatric solid tumors. We aimed to assess the surgical com- Methods: Data were collected from patients affected by cancer
plications in pediatric solid tumors objectively and validate this treated with a robotic approach in two periods (2015-2016 and 2019-
classification. 2021). Surgical indication and approach were discussed within the mul-
Methods: This is a retrospective study over 5 years conducted in tidisciplinary tumor board. Descriptive statistics, data on conversions,
a tertiary care centre from January 2017 to December 2021. All and complications are reported.
patients with pediatric solid tumors, except brain and bone tumors, Results: Thirty-eight patients were operated on at a median age of 5
were included. Postoperative complications were assessed and graded years (4 months - 24 years), with a median weight of 22 Kg (6 - 96
on the basis of Clavien- Dindo classification system. Outcome mea- Kg). Median console time was 76 minutes. In the majority of patients, 4
sures like operative time, blood loss, length of hospital stay, and redo robotic trocars were used. In the 4 younger patients, 3 robotic trocars
surgery were evaluated. were used. The median distance between trocars was 4.5 cm (4 - 10cm).
Results: A total of 188 patients were included. there was a statistically Eleven patients (28.9%) required conversion to open surgery due to
significant relation between grades of postoperative complications and difficult vascular management (6), bleeding (2), undefined margins (2),
duration of hospital stay with a mean postoperative stay of 29 days in and adhesions (1). Two (5%) intraoperative complications (bleeding,
children with grade 4b complications. There was a significant correla- gallbladder injury) and 3 (7.9%) postoperative complications (incisional
tion with operative time with a mean operative time of 150 minutes in hernia, bowel obstruction, pelvic fluid collection) were reported. The
children with grade 4 complications as compared to just 75 to 100 min- average hospital stay was 5 days.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S37 of S635

Conclusions: RS allows a more comfortable and precise minimally Nicolas Vinit1 , Amane Lachkar1 , Lauriane Lemelle2 , Chrystelle
invasive treatment in pediatric cancer surgery. An adequate learn- Madre3 , Julie Sommet3 , Alaa El Ghoneimi1 , Daniel Orbach2 , Matthieu
ing curve is mandatory to better address surgical indications, reduce Peycelon4 , Pascale Philippe Chomette1
complications, and optimize trocars positioning. The potential risk 1 ROBERT DEBRE HOSPITAL, Pediatric Cancer Surgery, PARIS, France;
for vascular injury is the main reason for conversion to open 2 Institut Curie, Siredo Oncology Department, Paris, France; 3 PEDIATRIC
surgery. SURGERY, Intensive Care Robert Debre Hospital, PARIS, France; 4 ROBERT
DEBRE HOSPITAL, Pediatric Surgery, PARIS, France

O076 / #1597 ROBOTIC SURGERY IN PEDIATRIC TUMORS / Background and Aims: The Enhanced Recovery After Surgery (ERAS)
NEW EXPERIENCE FOR 24 PROCEDURES IN A SINGLE is a pathway designed to achieve ealy recovery for patients under-
INSTITUTION WITH HEAVY LAPAROSCOPIC EXPERIENCE IN going major surgery using a multidisciplinary team approach. In the
PEDIATRIC ONCOLOGY pas two decades, ERAS was improved for adults and proved efficiency
for decrasing length of stay (LOS) without increasing post operative
Pascale Philippe Chomette1 , Matthieu Peycelon2 , Filippo Incerti2 , complications. The effect of RAS for children are not well known. We
Tanase Anca3 , Lauriane Lemelle4 , Daniel Orbach5 , Christine Grapin wanted to asess the feasability of enhanced recovery protocols for
Dagorno1 , Alaa El Ghoneimi1 children undergoing in oncological surgery.
1 ROBERT DEBRE HOSPITAL, Pediatric Cancer Surgery, PARIS, France; Methods: We collected the medical records of consecutive patients
2 ROBERT DEBRE HOSPITAL, Pediatric Surgery, PARIS, France; 3 ROBERT who underwent oncological surgery since 2015 to 2021.The primay
DEBRE HOSPITAL, Radiology, PARIS, France; 4 SIREDO Oncology Center aim was the feasibility of ERP and children were divided into 2 groups
(Care, Innovation and Research for Children and AYA with Cancer) Institut : Group A : ERAS was feasible and Group B ERAS was impossible. cri-
Curie, Oncology, Paris, France; 5 Institut Curie, Siredo Oncology Department, teriae were : drain > 5 days, urinary catheter > 5 days length of stay
Paris, France > 5 days Data were analysed according to the Clavien Dindo classifi-
cation and 30 day readmission rate. Statistical analysis was univariate
Background and Aims: Robotic surgery in pediatric oncology is rare. and multivariate logistic regression to wether RRP were predictive of
Disponibility of robotics in our pediatric surgical department since 4 LOS
years offer the new possibility of oncological resection by robotics. Results: 50 patients were included followed up for 224 days (31-660)
Pediatric oncological surgery by robotics started in june 2019 with and Group A : 37 patients were elligible Group B : 13 patients coudn’t bene-
we report the experience of a single team during a period of 2 years and fit of ERAS . Median Age of surgery was 6.4 years group A : 5 and group
a half. B : 12 p>0.2 Median weight was 19 kgs Group A : 18 and Group B : 21
Methods: A prospective study was started in june 2019 and collected p>0.2 74 % of the patients reached the target of hospital discharge of
data were reviewed in februar 2022. After an experience of classical the fith post operative day . Few complications ocurred : 16% without
robotic procedures in children, we started with pediatric oncological any association with the feasibility of ERAS . One month readmission
surgery beginning with selected cases approved by a certified tumor rate was 3/50 (6%) only in the ERAS group (p>0.2)
board and increasing the indications as we were at the end of the study. Conclusions: These preliminary results suggest that ERAS implemen-
Results: 24 tumors were resected in this period and 22 were malignant. tation for selected oncological surgery in chidren is suitable, associated
The median age at surgery was 5.5 years (range 3 mnths to 15 years with benefits and reduction of overall costs without increasing compli-
old). Renal tumors were the largest group (n=14) including 11 Wilms cations and readmissions.
tumors, 1 mesoblastic tumour and 2 carcinoma. Neuroblastic tumours
was the second largest group with 6 neuroblastomas. The other tumors
were 2 germ cell tumors, 1 pseudopapillary pancreatic tumour and 1 O078 / #131 SENTINEL LYMPH NODE PROCEDURE IN
adrenal adenoma. There was no conversion to an open approach. No PEDIATRIC PATIENTS USING NEAR-INFRARED FLUORESCENCE
intraoperative tumour rupture occured.The median hospital stay was 3 IMAGING WITH INDOCYANINE GREEN
days. One boy suffering of metastatic medullary carcinoma died of his
metastatic disease. Bernadette Jeremiasse, Cecilia Terwisscha-Van Scheltinga, Ludi
Conclusions: Robotic surgery for pediatric tumours can be safely Smeele, Marc Wijnen, Alida Van Der Steeg
started in a team with a heavy laparoscopic experience and could Princess Maxima Center, Surgery, Utrecht, Netherlands
rapidly be "the procedure of choice" for selected cases and after a
certified tumour board meeting approbation. Background and Aims: Standard sentinel lymph node proce-
dure (SNP) in pediatric cancer consists of a preoperative injection
with99m Technetium-nanocolloid in combination with an optional
O077 / #1844 ENHANCED RECOVERY AFTER intraoperative injection with blue dye for visual guidance. However,
ONCOLOGICAL SURGERY IN CHILDREN / WHAT IS SUITABLE? blue dye has disadvantages and the visual detection rate is low with
only 60% of the sentinel lymph nodes (SLNs) staining blue. In adult
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S38 of S635 ABSTRACTS

oncology, near-infrared (NIR) fluorescence imaging using indocyanine patients a single peri-hilar (PH) dose of 5mg of ICG was injected. Suc-
green (ICG) has been shown to be a safe and accurate method for cessful lymph node mapping was defined as presence of fluorescence
intra-operative visual detection of SLNs, with a higher sensitivity (up signal in draining lymph nodes.
to 97%) compared to blue dye. Therefore, our aim is to determine the Results: Eight patients(4M,4F) of median 2(range 2-5) years were
feasibility of the addition of ICG to 99m Technetium-nanocolloid for included. Six had localised and two metastatic disease at presenta-
visual detection of the SLN in pediatric patients. tion. Seven patients had Wilms tumour (WT) and one had clear cell
Methods: Twelve pediatric melanoma, squamous cell carcinoma and sarcoma. All patients underwent intra-operative real-time FGNM with
sarcoma patients were prospectively included. Intra-operative, the no complications from the ICG injection. Fluorescence was observed
SLN was first detected with fluorescence and the gamma probe. Post- in draining nodes at 5-15 minutes following injection. Seven patients
operatively, fluorescence was quantified by tumor-to-background ratio had successful FGNM with 8 (3-15) nodes sampled. Left-sided tumours
(TBR) and surgeons evaluated the use of ICG using a standardized drained to hilar and periaortic lymphatic and right-sided tumours
questionnaire. to hilar, retro-caval, inter-aortocaval, and precaval lymphatic zones.
Results: In all patients at least 1 SLN was detected with a median of 2. Three patients had positive lymph nodes of which two were fluo-
In 9/12 (75%) patients, the SLNs were visible transcutaneous. Twenty- rescent. There was one conversion to open surgery and one child
five SLNs were identified on pre-operative imaging, of which 22 (88%) developed a caecal volvulus.
were detected with fluorescence, while 24 (96%) could be detected Conclusions: ICG injection is feasible without upstaging patients
based on radioactivity. Of all intraoperative detected SLNs, 27/29 on COG or SIOP protocols based on this small set of patients.
(93%) were fluorescent and 29/29 (100%) were radioactive. Further- Early results reveal adequate nodal harvest in WT without proto-
more, ICG led to the identification of five additional SLNs as compared col violation or complications although a larger randomised study is
to preoperative imaging. These additional SLNs were also radioactive. required.
The average TBR in vivo of the SLNs was 7.2 and no adverse events
occurred. The surgical evaluation showed that ICG assisted in SLN
detection and was easy to use. O080 / #535 ADVANCES IN MINIMALLY INVASIVE
Conclusions: ICG combined with 99m Technetium-nanocolloid for the FLUORESCENCE GUIDED PAEDIATRIC ONCOLOGY SURGERY
SNP is a feasible procedure in pediatric patients. It showed an accu-
rate detection rate, was helpful for visual guidance, and did not lead to Max Pachl
adverse events. Birmingham children’s hospital, Paediatric Surgery, Birmingham, United
Kingdom

O079 / #90 FLUORESCENT GUIDED SURGERY FOR LYMPH Background and Aims: Near InfraRed Fluorescence(NIRF) using Indo-
NODE SAMPLING DURING PAEDIATRIC TUMOUR Cyanine Green(ICG) has been available for use for several years and
NEPHRECTOMY: A TRANSATLANTIC COLLABORATION has recently transferred to the paediatric surgical sphere. There is
increasing interest in its use in paediatric oncology surgery due to the
Max Pachl1 , G. Suren Arul1 , Andrew Davidoff1 , Andrew Murphy2 , Enhanced Permeability and Retention effect. We wanted to ascertain
Abdulhafeez Abdulhafeez2 if Minimally Invasive Fluorescent Guided Surgery (MIFGS) could give
1 Birmingham children’s hospital, Paediatric Surgery, Birmingham, United better identification of oncological lesions; allow more precise tumour
Kingdom; 2 St. Jude Children’s Research Hospital, Department Of Surgery, dissection and improve nodal sampling.
Memphis, United States of America Methods: This is a single site, open label, prospective feasibility study
of patients receiving ICG as part of a research trial in MIFGS. Fund-
Background and Aims: Lymph node sampling is critical to Wilms ing was obtained from Children’s Cancer and Leukaemia Group, UK.
tumour staging with failure associated with an increased risk of relapse. All patients undergoing MIS paediatric oncology surgery were eligible
Fluorescent-guided nodal mapping (FGNM) using ICG is used in some for enrolment. Patient demographics, dose and timing of ICG injection,
adult cancer patients to identify lymph nodes during resection surgery. intra-operative appearances, histology and other data were reviewed.
No technique is currently used for paediatric tumour nephrectomy, and The pathologist was blinded to ICG use.
we describe the early results of paediatric FGNM. Results: Thirteen patients were enrolled but two were excluded prior
Methods: Two tertiary level referral centers independently began to surgery. The remaining 11 patients underwent 12 procedures. Diag-
FGNM. Ethical approval was obtained as per institutional practice. noses were Wilms tumour in 4, Neuroblastoma in 3, osteosarcoma,
Consecutive patients fulfilling eligibility criteria were enrolled. ICG Hodgkins lymphoma, Inflammatory myofibroblastic tumour and sus-
was injected during minimally invasive (MIN) and open (ON) rad- pected thoracic sarcoma. All patients underwent treatment according
ical nephroureterectomy in patients with unilateral renal tumours to SIOP protocols Those patients having nodal mapping received ICG
managed on International Society of Paediatric Oncology (SIOP), or intra-operatively. All others received ICG intravenously at induction of
Children’s Oncology Group (COG) protocols. In MIN patients, an intra- anaesthesia. Three patients underwent MIFGS metastectomy with his-
parenchymal (IP) weight dependent ICG dose was injected. In ON tologically clear margins. Two patients underwent MIFGS resection of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S39 of S635

which both had clear margins. Four patients underwent nodal mapping O082 / #656 PULMONARY OSTEOSARCOMA
during MIS tumour nephrectomy a median of 8(3-9) nodes sampled METASTASECTOMY IN PEDIATRIC PATIENTS USING
One tumour was unsuitable for resection. Two patients had tumours NEAR-INFRARED FLUORESCENCE IMAGING WITH
which did not take up ICG. INDOCYANINE GREEN
Conclusions: ICG can be given during induction of anaesthesia rather
than 24 hours beforehand. In this small series MIFGS shows promis- Bernadette Jeremiasse, Caroline Hulsker, Cornelis Van De Ven, Guus
ing results in paediatric oncology surgery. A multicentre, multinational, Bökkerink, Marc Wijnen, Alida Van Der Steeg
open label, single blind, randomised controlled trial(GLOSurgery) is in Princess Maxima Center, Surgery, Utrecht, Netherlands
the setup phase which should give us an idea about the efficacy of this
approach, Background and Aims: Surgical control of pulmonary metastases, the
most common site of metastasis for patients with osteosarcoma, is
considered necessary for long term survival. Resection has been the
O081 / #1482 FLUORESCENCE GUIDED SURGERY IN mainstay of surgical management and can be performed as an open
PEDIATRIC ONCOLOGICAL SURGERY or thoracoscopic procedure. Radiologically detected lesions are some-
times difficult to identify peroperatively, especially when no palpation
Rodrigo Ribeiro1,2 , Alessandra Sousa2 , Bianca Rosa3 , Gustavo Orsi2 , is possible during thoracoscopy. In the adult population, near-infrared
Karina Silva2 , Wilson Oliveira Junior1 fluorescence imaging using indocyanine green (ICG) has been shown
1 Barretos School of Health Science Dr. Paulo Prata - FACISB, Surgery, to be a safe method for intra-operative visual identification of pul-
barretos, Brazil; 2 BARRETOS CHILDRENS CANCER HOSPITAL, Pediatric monary metastases. Our aim is to determine the feasibility of using
Surgery, Barretos, Brazil; 3 BARRETOS CANCER HOSPITAL (AMAZON), ICG for visual identification of pulmonary metastases in pediatric
Pediatric Surgery, PORTO VELHO, Brazil osteosarcoma patients.
Methods: Seven patients received an intravenous dose of ICG 24
Background and Aims: Fluorescence-guided surgery (FGC) is a hours preoperatively. To determine the most optimal dosage, a dose
method that makes it possible to identify structures during surgery. escalation scheme was followed consisting of three patients in every
The basic principle involves the injection of indocyanine green and sub- dosage group. We started with 0.5mg/kg, followed by 1.0mg/kg. Intra-
sequent visualization with an infrared camera The aim of this study is to operatively and post-operatively, the lesions were visualized with a
describe the initial experience with FGC. near-infrared camera system that could be used in open and minimally
Methods: Retrospective study of patients undergoing FGC from Jan- invasive surgical setting. Fluorescence was quantified by tumor-to-
uary 2020 to March 2022. Classification of the type of FGC, the dose background ratio (TBR) and surgeons evaluated the use of ICG for
of indocyanine, the time when the indocyanine was infused, and what metastasis identification.
was the usefulness of the FGC, classifying according to a modified Lik- Results: Two (29%) patients underwent a thoracoscopy and five (71%)
ert scale from 1 to 5. 1: irrelevant; 2: little relevant; 3: did not change underwent a thoracotomy. Three (43%) patients had a fluorescent
behavior but could be helpful in a similar case; 4: partially relevant and metastasis. In four (57%) patients there were no fluorescent metas-
5: totally relevant tases visible during surgery. In two cases the metastasis turned out to
Results: Sixteen FGC were performed. The age ranged from 20 days be necrotic, and in the other two visibility may have been hindered by
to 20 years. The oncological diagnosis was: liver tumor in 3 patients, the depth (>5mm) of the metastases. Four patients received an ICG
sacrococcygeal teratoma in 2, ovarian tumor in 3, and others. FGC dosage of 0.5mg/kg and three patients received 1.0mg/kg. 1.0mg/kg
was used as arteriography in 6 patients, visualization of liver tumor did not improve the TBR significantly. No adverse events occurred.
in 3, cholangiography in 3, cystography in 2, and lymphography in 2. Conclusions: ICG for pulmonary osteosarcoma metastasectomy in
Indocyanine green was injected intravenously in the arteriography, the pediatric population is a feasible procedure, given 24 hours pre-
inside the cyst on cystography, one to two hours before the procedure operatively using a dose of 0.5mg/kg. It does not seem to be useful for
for cholangiography, two to three days before the procedure for liver deep lying and necrotic metastatic disease.
tumors, and in the testis for Palomo surgery. The surgeries performed
were: three regulated hepatectomies, one non-regulated hepatec-
tomy, two sacrococcygeal teratoma resections, two peri-aortic lym- O083 / #1625 INDOCYANINE GREEN FLUORESCENCE
phadenectomies, two cholecystectomies, one salpingo-oophorectomy, IMAGING-GUIDED SURGERY IN PEDIATRIC ONCOLOGY
a laparoscopic Palomo surgery, a thoracoscopic thoracic duct ligation,
and a hepatic hilum lymphadenectomy. The procedures were classi- Maria Molina Mata, Rosa Cabello Laureano, Israel Fernandez Pineda
fied using a Likert scale: one in category 2, three in category 3, eight Virgen del Rocio Children’s Hospital, Pediatric Surgery, Seville, Spain
in category 4, and three in category 5.
Conclusions: The FGC is a tool to assist in various proce- Background and Aims: Indocyanine green (ICG) administration is
dures. Comparative studies may demonstrate the impact of this gaining popularity in pediatric surgery for a broad spectrum of proce-
technology. dures. ICG has also been proved to be useful in metastases detection,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S40 of S635 ABSTRACTS

lymph node evaluation and tumor resection. We aim to describe the occult FDG-avid lesions underwent excisional biopsy utilizing IV FDG
results of the use of ICG administration as a potential tool to guide the and PET probe survey. Prospective data collection of gamma counts
surgical approach in our pediatric cancer patients. from lesions and adjacent background tissue were recorded.
Methods: A prospective non-randomized study was conducted includ- Results: Lesions in the phantom showed an SUV of 4 when the model
ing all pediatric patients with a solid tumor who required a surgical contained background FDG. The PET probe was able to localize these
evaluation at our institution between March 2021 and March 2022. lesions when the background solution was saline but not in dilute FDG.
Inclusion criteria included the presence of a solid tumor requiring Similarly, in clinical use (nine operations in eight patients), the PET
surgery for diagnosis or definitive treatment and absence of iodine probe could not be used to guide dissection due to significant back-
contrast allergies. Depending on diagnosis and intended surgical pro- ground detection. After lesion excision by standard methods, the PET
cedure, ICG was administered: intravenously (1,5mg/kg) 24 hours prior probe did help confirm increased gamma counts ex vivo. In total, 17
surgery (group A), intraoperatively (group B) or subcutaneously (5mg) lesions were excised. Adequate tissue for diagnosis was present in 16
intraoperatively (group C). Parental consent was obtained before ICG (94%) of the specimens and positive for malignancy in 12 (71%). The
administration. Near infrared camera (Storz IMAGE1 S™ RUBINA) was average SUV on pre-op imaging was 8.4 and average tumor size was 2.0
used to assess ICG uptake. Data regarding demographics, diagnosis, cm.
ICG uptake and histological reports were recorded. Conclusions: Use of the PET probe was safe and feasible. The probe’s
Results: ICG was administered to 33 patients (15 males - 18 females) specificity for FDG, however, was poor in both the phantom and in clin-
with a median age of 9 years old [range, 10 days – 16 years]. In group ical use. Tumor-targeted, radiolabeled agents are likely necessary to
A (n=24), 9 patients showed ICG uptake: 2 neurogenic tumors primar- allow the PET probe to identify occult pediatric tumors in vivo with
ily excised, 4 patients with lung metastases, 1 lymphoma undergoing sufficient specificity.
biopsy, 1 undifferentiated sarcoma and 1 Wilms tumor. Group B (n= 7)
included hepatic tumors (n=3), thyroid surgery for parathyroid eval-
uation (n=3) and ovarian sparing surgery (n=1). Patients included in CCI
group C (n=2, rhabdomyosarcoma) were assessed for sentinel lymph
node biopsies, showing ICG uptake in both cases. CCI: MOBILIZING SUPPORT AND MULTIDISCIPLINARY
Conclusions: Our study showed the benefits of ICG administration in COLLABORATION 29-09-2022 1:30 PM - 3:30 PM
pediatric surgical oncology, specifically in lymph node biopsies, lung
metastases and hepatic tumors. However, further investigations are O085 / #1937 SERIES ON QUALITY OF LIFE FOR CHILDREN
needed to unify patient selection criteria and indications. WITH CANCER: DEVELOPING FAMILY-FACING RESOURCES
ABOUT PALLIATIVE CARE THROUGH INTERNATIONAL AND
MULTIDISCIPLINARY COLLABORATION
O084 / #913 HANDHELD PET PROBE FOR PEDIATRIC
CANCER SURGERY Daniel Bastardo Blanco1 , Ximena Garcia2 , Soad Fuentes-Alabi De
Aparicio3 , Mauricio Maza3 , Sara Benitez3 , Michael Mcneil2,4 ,
Hannah Rinehardt1 , Sadie Longo2 , Ryan Gilbert2 , W. Barry Edwards3 , Samantha Ransone1 , Diane Roberts1 , Justin Baker4 , Liliana Vasquez3
Gary Kohanbash4 , Marcus Malek5 1 St. Jude Children’s Research Hospital, Department Of Strategic Commu-
1 UPMC, General Surgery, Pittsburgh, United States of America; 2 University nication, Education And Outreach, Memphis, United States of America;
of Pittsburgh, School Of Medicine, Pittsburgh, United States of America; 2 St. Jude Children’s Research Hospital, Department Of Global Pediatric
3 University of Missouri, Department Of Biochemistry, Columbia, United Medicine, Memphis, TN, United States of America; 3 Pan American Health
States of America; 4 UPMC, Neurologic Surgery, Pittsburgh, United States Organization, Non-communicable Diseases, Washington D.C., United States
of America; 5 UPMC Children’s Hospital of Pittsburgh, Division Of Pediatric of America; 4 St. Jude Children’s Research Hospital, Division Of Quality Of
General And Thoracic Surgery, Pittsburgh, United States of America Life And Palliative Care, Memphis, United States of America

Background and Aims: 18F-fluorodeoxyglucose (FDG) positron emis- Background and Aims: Palliative care (PC) is a holistic approach to
sion tomography (PET) scans are essential in the diagnosis and mon- care that improves the quality of life of patients and their families fac-
itoring of pediatric cancer. Gamma probe (PET probe) localization of ing the problems associated with life-threatening illnesses through the
small FDG-avid lesions has been described in a few adult studies with prevention and relief of suffering. Despite PC being standard of care
only modest results. We sought to assess the utility and limitations of in pediatric oncology, misconceptions and negative perceptions remain
the PET probe and present the first use of the PET probe in pediatric as obstacles to access and acceptance, especially in regions with low
patients. health literacy, such as Latin America (LATAM).
Methods: The PET probe was first assessed in a PET scan calibration Methods: As part of the Global Initiative for Childhood Cancer,
model (phantom) using FDG-dense spheres (lesions) situated in either the Pan American Health Organization, St. Jude Children’s Research
saline or a dilute FDG-solution. Prior to PET probe survey, a PET scan Hospital, and regional PC professionals collaborated in developing
was performed. In a clinical model, pediatric patients with one or more an educational series about PC for parents and caregivers. First, a
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S41 of S635

working group of PC professionals from Peru drafted the content. ings followed four steps. (1) interview with the organizations to identify
Then, an international committee of PC and communications experts their priority topic, needs and expectations, (2) assembling the program
reviewed, edited, and designed the booklets. The writing and design and contents, (3) virtual and in-site training execution, (4) implementa-
incorporated best practices in patient education and considered the tion plan and follow-up. The training included a 20-hour virtual course
families’ socio-cultural context. The series was published in Nov- and 5 days of on-site visit to FNH premises. A short-term implemen-
2021 and distributed to LATAM Ministries of Health and regional tation plan was presented by each organization when finishing the
networks of PC professionals via official PAHO communications and training and is expected to be monitored with follow-up meetings.
the public via the Together by St. Jude™ online resource and social Results: Twelve institutions from eight countries participated in this
media. phase of ENLACE. Training areas were palliative care (5), housing (5),
Results: An international and multidisciplinary collaborative effort and rehabilitation (2). Observed outcomes were the sharing of sim-
produced an educational series that introduces parents and caregivers ilar challenges, improved knowledge and confidence, and increased
to concepts associated with pediatric PC and quality of life, including motivation and willingness to continue a peers’ network.
symptom management, care at home, communication, spirituality, and Conclusions: ENLACE promotes collaborative initiatives between peer
end-of-life care. The eight-module series was written in plain language, organizations to export successful experiences for replication. This
with the content organized into small sections to ensure understand- inspiring and successful strategy can improve the technical and organi-
ability. Additionally, the design utilizes white space, illustrations, and zational capacities of civil society organizations in their path towards
sub-headers to improve readability. Since its launch, the modules have influencing treatment outcomes and health policies for the benefit
been downloaded over 1500 times. of children with cancer. We consider this a valuable program to be
Conclusions: Breaking the stigma associated with PC through replicated among organizations aligned with the Global Initiative for
culturally-sensitive patient and family education is vital to improving Childhood Cancer from the World Health Organization.
acceptance among families of children with cancer. International
collaborations and multidisciplinary efforts have the power to catalyze
the development of high-quality and cost-effective patient education O087 / #852 JOURNEY OF CHILDHOOD CANCER PARENT
resources. GROUP, MOKUYO-KAI: 42 YEARS OF HISTORY AND THE WAY
FORWARD

O086 / #1470 COLLABORATIVE INITIATIVES AND SHARING Kazuko Takahashi


SUCCESSFUL PROGRAMS TO STRENGTHEN CHILDHOOD Mokuyo-kai, Main Office, Ukiha-city, Japan
CANCER ORGANIZATIONS IN LATIN AMERICA
Background and Aims: In 1981, the parent group called “Mokuyo-
Nuria Rossell1 , Jazmine Fernández2 , Vera Celedón3 , Valentina kai” was founded by a coalition of parents whose children were
Espinoza1 , Dunja Roje4 , Josefina Berliner5 , Alejandra Méndez6 , diagnosed with cancer at Kurume University Hospital, Japan. It was
Marcela Zubieta7 essential to join together, share feelings and provide mutual support
1 Fundación Nuestros Hijos, Extensión Y Desarrollo, Santiago, Chile; in collaboration with medical professionals to face challenges of can-
2 Fundación Nuestros Hijos, Servicios Médicos, Santiago, Chile; 3 Fundación cer journey. The group continues to provide psycho-social support,
Nuestros Hijos, Centro De Rehabilitación Oncológica, Santiago, Chile; strength and comfort to the families impacted by childhood cancer
4 Fundación Nuestros Hijos, Gerencia Técnica, Santiago, Chile; 5 Fundación including bereaved families.
Nuestros Hijos, Gerencia General, Santiago, Chile; 6 Fundación Nuestros Methods: Since the group’s establishment, variety of activities are
Hijos, Director, Santiago, Chile; 7 Fundación Nuestros Hijos, President, carried out which include regular meetings, hospital visits to share
Santiago, Chile experiences and feelings, to provide peer-counseling, education and
knowledge about diseases and optimal care. The grief care is also
Background and Aims: With the experience of 30 years working a key activity. Since 1994, the memorial service called “Star Festi-
collaboratively with private and public sectors to achieve best cure val” is held annually in July; in the COVID-19 pandemic, the group
chances for children with cancer in Chile, Fundación Nuestros Hijos continues to hold virtually. The activities extend to supporting sur-
(FNH) implemented ENLACE, a project for the strengthening of child- vivors and social awareness. Since 2008, the Japan-Korea international
hood cancer civil society organizations in Latin America. After com- exchange program is held learning social and cultural differences,
pleting its first phase of evaluation of organizational capacities, the sharing experiences and finding optimal solutions for obstacles and
organizations started a second phase of technical training and exper- challenges.
tise exchange in which four FNH’s signature programs are shared as Results: The activities are designed to meet the needs at each stage
training opportunities. Our aim is to report on this experience and of cancer journey, from diagnosis through treatment and beyond. Col-
share learned lessons to encourage similar initiatives in other regions. laboration, communication and mutual trust lead the group to provide
Methods: The training areas offered were palliative care, hospital the place of sharing feelings and thoughts surrounded by comfort and
schools, housing, and rehabilitation. The implementation of the train- hope. Such actions also lead to motivate parents to help others actively,
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S42 of S635 ABSTRACTS

to empower the group to sustain and to act as a catalyst in achieving ment to build better capacity and achieve their organizational
measures to support children with cancer. goals. This peer exercise helped the organizations to assess
Conclusions: For 42 years, the group’s activities meet the needs their standards, improve minimum organizational criteria and
not only in Japan but also in the neighborhood country with the generate trust for joint work. This methodology for capacity
spirit of not being alone. The accumulation of solid, sustained building and organizational development strengthens the net-
efforts and actions form the basis for the group’s continued path work of civil society organizations working to support children with
which connects the activities to the next generation and enhances cancer.
resilience through childhood cancer journey together as one big
family.
O089 / #987 IMPROVING CHILDHOOD CANCER OUTCOMES
THROUGH QUALITY MEDIA ENGAGEMENT (A CASE STUDY OF
O088 / #1878 ORGANIZATIONAL CAPACITY OF CIVIL CHILDHOOD CANCER AWARENESS MONTH)
SOCIETY ORGANIZATIONS WORKING FOR CHILDHOOD
CANCER IN LATIN AMERICA Korede Akindele, Azeezat Ajose
The Dorcas Cancer Foundation, Programs, Lagos, Nigeria
Nuria Rossell1 , Elizabeth Campos2 , Valentina Espinoza1 , Dunja Roje3 ,
Josefina Berliner4 , Alejandra Méndez5 , Marcela Zubieta6 Background and Aims: It is no doubt that today’s media plays a cru-
1 Fundación Nuestros Hijos, Extensión Y Desarrollo, Santiago, Chile; cial role in shaping the growing health concerns in society. An example
2 Ecconsultant, Organizational Psychology, Bogotá, Colombia; 3 Fundación was the outbreak of Ebola in Africa, and the recent covid 19 pandemic
Nuestros Hijos, Gerencia Técnica, Santiago, Chile; 4 Fundación Nuestros shows how media engagement is a formidable force to drive positive
Hijos, Gerencia General, Santiago, Chile; 5 Fundación Nuestros Hijos, Direc- outcomes for childhood cancer. This study aimed at mapping out evi-
tor, Santiago, Chile; 6 Fundación Nuestros Hijos, President, Santiago, Chile dence of media as an effective tool for bridging the care gap, addressing
inequities and low education, eradicating delay in the presentation of
Background and Aims: Treatment access inequalities are a main cases, and better portraying survivors of childhood cancer
obstacle to curing children with cancer in many regions. Civil soci- Methods: A series of media advocacy was held in 20 media stations
ety organizations have a key role in overcoming such inequalities and 4 social media platforms. A study was conducted during child-
and facilitating access to better quality of care. ENLACE is a col- hood cancer awareness month to identify how people respond to the
laboration program supporting Childhood Cancer International (CCI) information about childhood cancer. The public engagement data was
member organizations in Latin America to improve their organiza- extracted using the key performance indicators and metrics related to
tional development, professionalization, and technical capacity. We traditional and social media communication.
present here the diagnosis of the organizations participating in the Results: Of the twenty (20) media stations and four (4) social media
program. platforms. A total of 2million people were reached, 44.8% of the audi-
Methods: Organizations underwent a self and peer-evaluation process ence could not believe that children do have cancer, 50.2% believe a
for 7 criteria: legal situation, strategic planning, resource management, child could survive cancer with adequate health care facilities (43.4%)
administration, programs, institutional image, and information man- conclude that cancer can be genetic.
agement. Based on peer assessment of documentation presented, a Conclusions: Unfortunately, the media coverage of childhood cancer
report and an improvement plan were generated and discussed. An is quite low. This type of reporting has promoted fear and stigma and
Organizational Development scale was created with a total of 100 spreads false hopes about childhood cancer and as well build a wall
points distributed in three levels. Higher score indicates better orga- against effective participation of government and stakeholders It is
nizational capacity: Level 1 (score 1-33); Level 2 (score 34-67); Level 3 therefore important that as the world strives to advance in the cure
(score 67-100). for childhood cancer, we must also drag the media along to drastically
Results: From September 2020 to November 2021, 14 childhood can- reduce the burden of low presentation of cases and ultimately have
cer organizations from 10 countries participated in ENLACE. Accord- better survival outcomes.
ing to the scale, 11 organizations reached level 3 (scores between
63-95/100). One organization scored at level 2 (score 61/100); 2
organizations in level 1 (scores below 25). For the 14 participat- O090 / #979 IMPLEMENTATION OF AN INNOVATION
ing organizations the criteria most frequently requiring some levels PROJECT IN HUMANIZATION IN A PEDIATRIC
of improvement were strategic planning (10/14), resource manage- ONCOHEMATOLOGY DEPARTMENT
ment (9/14), and information management (9/14). All the organizations
received and discussed an improvement plan according to the specific Pablo Velasco1 , Eunice Blanco2 , Lucas Moreno1 , Maria Angeles
criteria detected as deficient. Aceituno3 , Esther Diaz1
Conclusions: The diagnostic process helped the organizations to 1 Vall d’Hebron University Hospital, Pediatric Oncology And Hematol-
see which areas of organizational development needed improve- ogy, Barcelona, Spain; 2 VALL D’HEBRON HOSPITAL, Social Woeker
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ABSTRACTS S43 of S635

Department, BARCELONA, Spain; 3 Vall d’Hebron University Hospital, Pedi- And Outreach, Memphis, TN, United States of America; 3 Pan American
atrics, Barcelona, Spain Health Organization, Non-communicable Diseases, Pan American Health
Organization, Washington, United States of America; 4 Pan American Health
Background and Aims: Intravenous chemotherapy and blood transfu- Organization, Non-communicable Diseases, Washington D.C, United States
sions can cause adverse events that leads to anxiety and anticipatory of America; 5 St. Jude Children’s Research Hospital, Division Of Quality Of
anguish in patients in the face of new treatments, known as aver- Life And Palliative Care, Memphis, United States of America
sive conditioning. Guided imagery has been shown to reduce these
effects. With the aim of reducing this effect, numerous initiatives Background and Aims: Integration of palliative care (PC) into the care
arose to cover the treatments. Unfortunately, these devices could not of children with cancer is considered the standard of care. However,
be implemented due to lack of security derived from fully covering access and acceptance remain limited throughout the world, especially
the treatments. For this reason, in 2015 the SUPERBOX project was in low- and middle-income countries. A barrier to this integration is par-
developed, a safe device that would motivate adherence to treatment, ents and caregivers’ misconception about the fundamental goal of PC:
following the development processes of a medical device. To attend to suffering and improve the quality of life of children with
Methods: With an interdisciplinary Think Tank (engineers, doctors, life-threatening conditions.
social workers, nurses, assistants, volunteers, psycho-oncologists, Methods: As part of the Global Initiative for Childhood Cancer, the Pan
families and patients) we periodically design and evaluate improve- American Health Organization, St. Jude Children’s Research Hospital,
ments for the device. Quantitative and qualitative pre- and post- and regional partners collaborated in a 3-phase educational project.
implementation evaluation with focus groups through the Design First, an educational needs assessment survey was distributed to 365
Thinking methodology parents and caregivers of children with cancer in Peru. Among the
Results: After several modifications, all the objectives were achieved: needs identified, 67% of parents surveyed selected palliative care as
efficacy in the coverage of the serum, safety in the placement and an educational priority. Then, based on this finding, an interdisciplinary
control by health workers, universal adaptability to serums and serum group of pediatric PC experts developed educational content for par-
sticks, resistance to sterilization, personalized, recyclable, affordable, ents and caregivers. Finally, an international committee of regional
versatile for others centers. The final design can be consulted at PC and communication experts reviewed, designed, and validated the
https://www.superbox.es/ The different prototypes were tested in the resources.
oncohematology department followed by other pediatric and adult Results: This collaboration resulted in the development of an 8-module
departments, as well as in other centers. The evaluations show that the series that introduces parents and caregivers to key concepts of pedi-
device promotes patient empowerment, including them in decision- atric PC. The series intends to decrease acceptance barriers to PC by
making, choosing the illustrations related to their mood, thereby directly addressing caregivers’ stigma associated with PC through cul-
opening a new window for communication with health workers and turally sensitive patient education, taking into consideration the low
providing a pleasant, safe and friendly environment. health literacy levels of parents and caregivers in the region. The mod-
Conclusions: Innovation in hospital humanization projects benefit ules cover management of pain and other distressing symptoms, care
from interdisciplinary work processes and patient-centered evalu- at home, communication, quality of life, and end-of-life care. Since its
ation, achieving the efficiency and safety that any medical device launch in November 2021 and until March 2022, the modules had been
requires. The personalized coverage of treatments allows to improve downloaded 1,532 times and distributed throughout Latin America.
the experience and patient-centered care. Conclusions: Educational material development and anticipatory guid-
ance of PC are considered a priority for parents of children with
cancer throughout Latin America. Materials developed through this
FREE PAPER SESSION (FPS) project are freely available and downloadable through the PAHO
website.
FPS 03: PALLIATIVE AND SUPPORTIVE CARE 29-09-2022 1:30
PM - 3:00 PM
O092 / #1820 PALLIATIVE AND END-OF-LIFE CARE FOR
O091 / #1152 SERIES ON QUALITY OF LIFE FOR CHILDREN CHILDREN WITH CENTRAL NERVOUS SYSTEM TUMORS:
WITH CANCER: IMPROVING PALLIATIVE CARE HEALTH RESULTS OF A SPANISH MULTICENTER STUDY
LITERACY AMONG PARENTS AND CAREGIVERS
Maria Pérez-Torres Lobato1 , Iñigo De Noriega2 , Lucía Navarro
Ximena Garcia1 , Daniel Bastardo Blanco2 , Soad Fuentes-Alabi De Marchena3 , Palma Solano-Páez4 , Miriam Morei Olive5 , Eloisa Rubio
Aparicio3 , Mauricio Maza3 , Sara Benitez4 , Michael Mcneil5 , Justin Pérez6 , Alvaro Lassaletta7 , Carmen Garrido Colino8 , Miriam García
Baker5 Abos9 , María Tallón García10 , Beatriz Huidobro Labarga11 , R Portugal
1 St. Jude Children’s Research Hospital, Department Of Global Pediatric Rodríguez12 , Blanca López Ibor13 , Andrés Morgenstern14 , Lorena
Medicine, Memphis, TN, United States of America; 2 St. Jude Children’s Valero Arrese15 , Anna Llort Sales1 , Lucas Moreno16 , Catalina
Research Hospital, Department Of Strategic Communication, Education Márquez Vega17 , Eduardo Quiroga-Cantero18
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S44 of S635 ABSTRACTS

1 Vall d’Hebrón Hospital, Onco-hematology, Barcelona, Spain; 2 CS Mejo- O093 / #593 THE EFFECT OF FAMILY-CENTERED PEDIATRIC
rada del Campo, Pediatric Unit, Madrid, Spain; 3 Sant Joan de Deu Hospital, ADVANCE CARE PLANNING FOR TEENS WITH CANCER
Pediatric Palliative Care, Barcelona, Spain; 4 HOSPITAL INFANTIL VIRGEN (FACE®-TC) ON ADOLESCENTS’ DECISIONAL SUPPORT,
DEL ROCIO, Pediatric Oncology, SEVILLE, Spain; 5 Vall d’Hebron Hospital, PREPAREDNESS, AND SYMPTOMS AT 3 AND 12-MONTHS
Pediatrics, Barcelona, Spain; 6 Department of Statistic and Research, Vir- POST-INTERVENTION
gen Del Rocio Hospital, Seville, Spain; 7 Hospital Infantil Universitario Niño
Jesús, Pediatric Neuro-oncology, Madrid, Spain; 8 Gregorio Marañon Hos- Justin Baker1 , Sarah Friebert2 , Jennifer Needle3 , Jessica Thompkins4 ,
pital, Pediatric Oncology, Madrid, Spain; 9 7Universitary Hospital Donos- Daniel Grossoehme2 , Jiji Jiang4 , Jichuan Wang4 , Maureen Lyon4
tia, Pediatric Oncology, Guipuzcoa, Spain; 10 Álvaro Cunqueiro Hospital, 1 St. Jude Children’s Research Hospital, Department Of Global Pediatric
Pediatric Oncology, Vigo, Spain; 11 Virgen de la Salud Hospital, Pediatric Medicine, Memphis, United States of America; 2 Haslinger Family Pediatric
Palliative Care, Toledo, Spain; 12 Universitary Hospital Burgos, Pediatric Palliative Care Center, Haslinger Family Pediatric Palliative Care Center,
Oncology, Castilla y Leon, Spain; 13 HM Monteprincipe Hospital, Pediatric Akron, United States of America; 3 Department of Pediatrics and Center for
Oncology, Madrid, Spain; 14 Vall d’Hebron Hospital, Pediatric Palliative Care, Bioethics, Department Of Pediatrics And Center For Bioethics, Minneapolis,
Barcelona, Spain; 15 Vall Hebron Research Institute, Childhood Cancer And United States of America; 4 Children’s National Health System & The George
Blood Disorders, Barcelona, Spain; 16 Vall d’Hebron University Hospital, Pae- Washington University School of Medicine and Health Sciences, Center For
diatric Oncology And Haematology, Barcelona, Spain; 17 Virgen del Rocio Translational Research, Washington DC, United States of America
Hospital, Pediatric Oncology, Seville, Spain; 18 Hospital Infantil Virgen del
Rocio, Pediatric Oncology, SEVILLE, Spain Background and Aims: Background and Aims Pediatric advance care
planning (pACP) is a process of preparation and skill development to
Background and Aims: Central nervous system (CNS) tumors repre- facilitate discussions about future medical care choices. We evaluated
sent the most common cause of cancer-related death in children. Few the efficacy of FACE® -TC on adolescents’ decisional support, prepared-
studies concerning the palliative phase in children with CNS cancer are ness, and quality of life (QoL) and other patient-reported outcome
available.The aim of this study is: i)Describe the major issues during the (PRO) measures.
palliative phase in children with incurable CNS tumors; ii)Determine Methods: Single blinded, intent-to-treat randomized clinical trial. Ado-
whether the use of palliative sedation (PS) depends on the place of lescent/family dyads were randomized at a 2:1 ratio to either FACE®-
death or the attention by a Palliative Care team (PCT). TC or Treatment as Usual (TAU) at four pediatric hospitals. FACE®-TC
Methods: A retrospective study including twelve spanish pediatric dyads received 3 weekly 60-minute sessions: pACP Survey; Next
hospitals was conducted between January 2010 and August 2021. Steps: Respecting Choices interview; and Five Wishes. All received
Patients <18 years old dead from a brain tumor were recruited. pACP information. Outcome measures were: Decisional Support and
Results: 228 patients (59.2% male) were included. Median age at Preparedness; FACIT-Spirituality; and PROMIS measures.
diagnosis was 5 years (IQR2-9), median age at death was 7 years Results: 126 dyads underwent randomization (83 FACE-TC and 43
(IQR4-11). 151 patients were attended by PCT. The most frequent TAU). Compared to TAU, FACE-TC adolescents were significantly more
tumor was medulloblastoma (25.4%) followed by DIPG (24.1%). The likely to agree or strongly agree to “I feel prepared for the future”
most common symptoms were motor deficit (93.4%), astenia (87.5%), (76%vs.94%, p=0.025) and to “I feel we are now on the same page”
headache (83%) and cranial nerve deficit (82%). The most frequently (76%vs.94%, p=0.044) at 3 months post-intervention (N=107). There
prescribed supportive drugs were opioids (81.6%), antiemetics (83.6%) was no intervention effect at 12-months (N=104). Compared to TAU,
and dexamethasone (78.5%). Median antineoplastic line therapies FACE-TC had no significant effect on meaning/purpose or peace at
were 2 (Range 0-5 lines) and 63,2% patients received palliative anti- 3-months, and significantly decreased meaning/peace at 12-months
neoplastic therapy (34,2% radiotherapy, 23,7% chemotherapy, 25% [Mean, SD=28(4) vs.26(5), p=0.029]. Compared to TAU, FACE-TC had
metronomic chemotherapy, 15.4% reirradiation). Palliative sedation no effect on the PROMIS [BJ1] measures at 3 months, and significantly
(PS) was administered to 92 patients (40.4%), 91.3% of them had doc- increased Pain Interference [Mean, SD=40(80 vs.4 (10), p=0.030];
umented refractory symptoms, dysnea being the commonest (42%). PS Anxiety [42(12) vs.52(10), p=0.001]; and Depressive symptoms [43(8)
was more frequent when patients died at the hospital (p=0.02) or when vs.49(11), p=0.007] at 12-months.
they were not attended by a PCT (60%). Conclusions: FACE-TC increased adolescents’ feelings of being pre-
Conclusions: Children dying from CNS tumors have unique end of life pared and supported. By 12-months FACE-TC adolescents had more
problems, that deserve specific management. Early identificationn of pain interference and anxiety and depressive symptoms. The outbreak
symptoms in this population will lead to a better management, a better of COVID during the 12-month follow-up visits may have impacted
quality of life and a lower incidence of hospital admissions. The opti- these outcomes given these effects were not observed at 3-months
mal and time-appropriate indication of PS largely depends on the team post-intervention. Justin N. Baker, Sarah Friebert, Jennifer S. Needle,
experience and should ideally be previously discussed and planned Jessica D. Thompkins, Daniel Grossoehme, Jiji Jiang, Jichuan Wang,
with patients and caregivers. Maureen E. Lyon
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ABSTRACTS S45 of S635

O094 / #476 DISCOVERING NEEDS OF PALLIATIVE CARE IN O095 / #303 THE VALUE OF DAILY PATIENT-REPORTED
CHILDREN WITH CANCER IN INDONESIA OUTCOME MEASUREMENTS IN PEDIATRIC CANCER CARE

Alexandra Pangarso1 , Sri Mulatsih1 , Mei Sitaresmi2 , Sanne Verhulst3 , Andreas Meryk1 , Gabriele Kropshofer1 , Benjamin Hetzer1 , David
Gertjan Kaspers4,5 , Saskia Mostert6 Riedl2 , Jens Lehmann2 , Gerhard Rumpold2 , Alexandra Haid1 , Verena
1 Dr. Sardjito General Hospital/Faculty of Medicine, Public Health and Schneeberger-Carta1 , Bernhard Holzner2 , Roman Crazzolara1
Nursing, Universitas Gadjah Mada, Child Health Department, Pediatric 1 Medical University of Innsbruck, Department Of Pediatrics, Innsbruck,
Hematology Oncology Division, Yogyakarta, Indonesia; 2 Dr. Sardjito Gen- Austria; 2 Medical University of Innsbruck, Department Of Psychiatry,
eral Hospital/Faculty of Medicine, Public Health and Nursing, Universitas Psychotherapy And Psychosomatics, d, Austria
Gadjah Mada, Child Health Department, Social Pediatric And Growth
Development Division, Yogyakarta, Indonesia; 3 Emma Children’s Hospi- Background and Aims: Despite the proven demand of patient-
tal, Amsterdam UMC, Vrije Universiteit, Pediatric Oncology, Amsterdam, reported outcome measurements (PROMs) in cancer care, its routine
Netherlands; 4 Princess Máxima Center for Pediatric Oncology, Pediatric use in pediatrics is still in infancy. This study aims to identify pediatric
Oncology, Utrecht, Netherlands; 5 Emma Children’s Hospital, Amsterdam cancer patients at risk of severe treatment side-effects and provide
UMC, Vrije Universiteit, Pediatric Oncology, Amsterdam, Netherlands; individualized supportive care using PROMs.
6 Princess Maxima Center for Pediatric Oncology, Pediatric Oncology, Methods: In this prospective longitudinal, single-armed utility study,
Utrecht, Netherlands we used ePROtect, a Web-based questionnaire on symptom and
exposure screening during chemotherapy. ePROtect assesses daily six
Background and Aims: World Health Organization has ranked can- questions allocated to four domains: pain, nausea, physical function
cer as leading cause of death in children, only 20% surviving in low and sleep disturbance. In the case of deviations in the PROM score,
and middle-income countries. Cancer and its treatment cause phys- the patients or their caregivers were immediately contacted by the
ical and psychological symptoms also social and spiritual concerns. physicians to confirm the reported symptoms, discuss the situation and
However, since focus has been on curative treatment, palliative care initiate an intervention, if needed.
received little attention. This study explores parents’ perspectives Results: 4410 daily PROMs from forty children (median age: 9.1 years)
on experiences and needs of children dying from cancer during final were analyzed over a median follow-up of 145.5 days. Overall comple-
illness. tion rate was 62.3%, slightly lower during home care versus inpatient
Methods: Home visits were conducted from October 2019 to stay (58.4% vs. 68.0%, respectively, p=.014) - decreasing over time
April 2020 to interview parents of children, diagnosed and treated (66.4% versus 52.3%, first 90 days versus beyond 90 days, p<.001).
for cancer at Indonesian academic hospital, who died between Severe symptoms were reported at 14.9% of time points; the major-
April 2019 and January 2020. Semi-structured questionnaires were ity were associated with physical activity, followed by pain, sleep and
used. nausea. In total, 321 adverse events and cases of health deteriora-
Results: Parents of 49 children (response rate 74%) were home vis- tion were documented and PROMs were completed for 251 (78.2%) of
ited. All children died inside hospital, and 37% of parents stated that these events. Across all events, self-reported pain was the most useful
this was not the location where their child wanted to die. Most com- marker, particularly when analyzed on the day before onset of adverse
mon symptoms during final illness were: breathing difficulties (82%), events, and was associated with an odds ratio [OR] of 3.7 ([95% CI, 1.5
pain (80%), loss of appetite (80%). Psychological symptoms received to 8.6], p=.002) for the presence of mucositis.
least help from medical team: depression (46%), anxiety (45%), sad- Conclusions: This study found that PROMs accurately reflect daily
ness (33%). Boys suffered more often from anxiety (68%) than girls symptoms in children being treated for cancer. Collecting this informa-
(37%; P=0.030). Parents (57%) were not always kept informed about tion is very useful for clinical management.
their child’s condition, and doctors gave confusing information about
treatment (43%). Child’s choice of treatment while dying was: relieving
pain or discomfort (39%), extending life (33%), and 29% parents did not O096 / #269 FACTORS INFLUENCING THE QUALITY OF
know. Yet, many parents (51%) did not talk with doctors about these RELATIONSHIP IN PEDIATRIC ONCOLOGY BETWEEN
treatment wishes. Children (45%) felt lonely in their last month of life CHILDREN, CAREGIVERS AND HEALTHCARE PROVIDERS; A
and would have liked to interact more with school (71%), friends (63%), QUALITATIVE STUDY FROM THREE DIFFERENT CENTERS OF
and family (57%). PAKISTAN
Conclusions: To relief suffering of children with cancer regular phys-
ical, psychological, social and spiritual needs assessment is required. Shahzadi Resham1 , Sadaf Altaf2 , Farwa Ayub2 , Areesh Bhatti2 , Areeba
Families should actively participate in deciding whether to extend life Syed2 , Rida Iqbaal2 , Afia Tul Quanita2 , Clariana De Oliveira3 , Zehra
of their children, or relieve pain and discomfort. This can importantly Fadoo2 , Shamvil Ashraf4 , Uzma Imam5 , Muhammad Rafie Raza4 , Aisha
improve quality of life of children and their families. Yousafzai3
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S46 of S635 ABSTRACTS

1 Aga Khan University Hospital, Pediatric And Child Health & NURSING
Department Of Oncology, Karachi, Pakistan; 2 Aga Khan Univer-
sity Hospital, Department Of Oncology, Karachi, Pakistan; 3 Harvard NURSING ABSTRACT PRESENTATIONS: LISTENING TO
T H Chan School of Public Health, Boston, MA, USA, Department CHILDREN’S VOICES 29-09-2022 2:35 PM - 3:30 PM
Of Global Health And Population, Boston, United States of Amer-
ica; 4 The Indus Hospital, Department Of Oncology, Kaarchi, Pakistan; O097 / #1111 CHILDREN’S HEALTH NARRATIVES
5 National Institute of Child Health, Department Of Oncology, Karachi, REFLECTED THROUGH A CHILD-CENTRIC MHEALTH APP: A
Pakistan SECONDARY ANALYSIS

Background and Aims: There is a scarcity of data on commu- Lauri Linder1 , Amy Newman2 , Katherine Bernier-Carney3 , Sarah
nication, impacting the quality of relationships between families Wawrzynski1 , Se-Hee Jung1 , Jennifer Farnsworth1 , Adam Otto4 ,
with children facing life-threatening illness and their health- Hakop Kardzhyan1 , Hailey Haffey5
care teams, particularly in low- and middle-income countries. 1 University of Utah, College Of Nursing, Salt Lake City, United States of
We aimed to describe the characteristics of current communi- America; 2 Marquette University, College Of Nursing, Milwaukee, United
cation practices between health care providers, children, and States of America; 3 University of Connecticut, School Of Nursing, Storrs,
their families in three major pediatric oncology units in Karachi, United States of America; 4 University of Utah, School Of Business, Salt
Pakistan. Lake City, United States of America; 5 University of Utah, General Internal
Methods: A qualitative phenomenological design was employed. Par- Medicine, Salt Lake City, United States of America
ticipants included children (aged 8–18 years) with life-threatening
illnesses receiving treatment at pediatric oncology units, their care- Background and Aims: Although mobile health apps allow patients to
givers, and their healthcare providers. The team conducted semi- report discrete health outcomes, less attention has been given to the
structured in-depth interviews about their lived experience stratified health narratives, or underlying individual stories, reflected in these
across three major pediatric oncology units. Data collection stopped data. This secondary analysis identified health narratives of children
when a point of saturation was reached. Inductive thematic analysis with cancer reflected in their self-reported data during the feasi-
was conducted, and themes were organized using Dedoose qualitative bility/acceptability trial of a newly developed child-centric symptom
software. reporting app, Color Me Healthy.
Results: Sixty-two semi-structured in-depth interviews were con- Methods: Nineteen children (6-12 years old; 12 boys) receiving cancer
ducted. Fourteen themes were identified. Three major themes will be treatment were asked to use the Color Me Healthy app at least 5 days
highlighted in this abstract. First, healthcare providers held a favorable between clinical visits. In addition to reporting symptoms, children
perception of effective communication. Their practices were found to could customize an avatar and express themselves through a sketch
be significantly influenced by the sociocultural environment, a lack page, diary, and completing individual goals. Data were extracted from
of formal training in communication for the majority, and a variable app-generated files and compiled into investigator-developed tem-
approach to communication. Second, families and healthcare providers plates for analysis. Each child’s data were reviewed by three study
shared similar concerns about treatment-related fears in children team members using a narrative medicine framework. Close reading
and caregivers, which resulted in a reluctance to share information techniques emphasizing time, voice, setting, mood, perspective, and
about children’s illnesses regardless of age. Finally, both healthcare symbolism were used to elicit a holistic view of the child’s sense of self
providers and caregivers believed that empowering children, involv- and understanding of their experience of illness.
ing families and children in treatment plans, and dealing with children Results: App use ranged from 1 to 12 days (median 4). Children’s
in an age-appropriate manner would contribute to the formation self-reported data provided important insight into their individual
of a strong therapeutic alliance among providers, caregivers, and narratives – even for children with limited app use. Across children,
children. narratives reflected the co-occurring experience of usual childhood
Conclusions: Our findings were exploratory in establishing the reali- activities and experiences while living with the consequences of their
ties surrounding the need for effective communication and identifying illness and treatment. Individually, children’s narratives reflected a
communication barriers relevant to the socio-cultural context of Pak- developing sense of identity and agency, important relationships in
istan. This thematic approach will assist clinicians and researchers in their lives, interruptions from cancer symptoms, and adapting to a
identifying intervention strategies to improve communication expe- diagnosis of cancer.
riences in pediatric oncology in similar low- and middle-income Conclusions: The Color Me Healthy app allowed children to communi-
settings. cate their individual health narratives. Future work includes supporting
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ABSTRACTS S47 of S635

clinicians, as recipients of children’s narratives, to use children’s data for efforts to elicit cooperation from children undergoing painful
in support of person-centered care. Work is also needed to depict procedures.
children’s app-generated data in electronic health record systems in a
manner that maintains the underlying narrative and supports clinical
workflow. O099 / #1443 USING QUALITATIVE SYSTEMATIC REVIEW
TO DEVELOP PRACTICE RECOMMENDATIONS FOR ENGAGING
CHILDREN AND ADOLESCENTS WITH CANCER LESS THAN 18
O098 / #708 NURSES’ PERCEPTIONS OF SITUATIONS IN YEARS IN THEIR TREATMENT DECISION MAKING
PEDIATRIC ONCOLOGY CARE THAT INVOLVE CHILDREN’S
VOICES - A CROSS-SECTIONAL SURVEY OF ETHICAL CLIMATE Katherine Kelly1 , Kimberly Pyke-Grimm2 , Ginny Schulz3
AND MORAL DISTRESS 1 Children’s National Hospital, Nursing Research Professional Practice And
Quality, Washington, United States of America; 2 Lucile Packard Children’s
Päivi Ventovaara1 , Margareta Af Sandeberg2 , Gitte Petersen3 , Klas Hospital Stanford, Department Of Nursing Research And Evidence-based
Blomgren4 , Pernilla Pergert5 Practice, Palo Alto, United States of America; 3 Washington University in
1 Karolinska Institutet, Department Of Women’s And Children’s Health, St. Louis, Department Of Pediatrics, Division Of Hematology/oncology, St.
Stockholm, Sweden; 2 Karolinska Institutet, Department Of Women’s And Louis, United States of America
Children’s Health, Stockholm, Sweden; 3 University hospital of Copenhagen
Rigshospitalet, Department Of Child And Adolescent Cancer, Copenhagen, Background and Aims: Children and adolescents < 18 years (C&A)
Denmark; 4 Karolinska Institutet, Department Of Women’s And Children’s report varying treatment decision-making (TDM) preferences and
Health, Stockholm, Sweden; 5 Karolinska Institutet, Women’s And Children’s experiences that might be distinct from what has been reported by
Health, Stockholm, Sweden their parents and clinicians. Identifying these distinctions will provide
insight into care practices to meet child and adolescent’s communica-
Background and Aims: Nurses caring for children with cancer tion needs.
often experience ethically challenging situations. Some of these Methods: Using Joanna Briggs Institute meta-aggregation methods,
situations involve overriding children’s wishes. When unable to we conducted a qualitative systematic review of the literature to
take the action one considers as ethically right, moral distress describe child and adolescent (C&A) self-reported preferences for
may occur. The aim of this study was to explore experiences being involved in making their cancer treatment decisions (CTD).
of moral distress and perceptions of ethical climate in pediatric Parent or clinician responses were purposefully excluded from this
oncology care, with a focus on situations that involve children’s review. PubMed, PsychINFO, CINAHL and SCOPUS database searches
voices. were completed in November 2019 and updated March 2022. Cov-
Methods: Cross-sectional, multicenter survey among nurses (n = 137) idence screening software identified 46 studies (n=461 C&A across
working in pediatric oncology centers in Denmark. Data was collected 15 countries) for synthesis and meta-aggregation (PROSPERO ID:
by using a questionnaire with the translated versions of the Swedish CRD42021219107). We used Atlas.ti qualitative analysis software to
Hospital Ethical Climate Survey - Shortened (HECS-S) and the Swedish extract and code C&A self-reported study findings.
Moral Distress Scale - Revised (MDS-R). Data analysis was performed Results: Children and adolescents’ self-reported experiences yielded
with IBM SPSS Statistics and included descriptive statistics and a non- nuanced TDM preferences. Decision types ranged from daily care deci-
parametric test. sions (e.g. pill vs. liquid medication) to disease-directed decisions (e.g.
Results: A total of 66 nurses returned the questionnaire (response clinical trial) across the illness continuum. Many C&A did not perceive
rate 48 %). Perceptions of ethical climate were positive and health- that there was a treatment choice to make other than following doc-
care professionals were perceived to be attentive to children’s wishes. tors’ recommendations. Most C&A reported preferences for trusted
Situations described in the MDS-R were perceived as highly disturb- adults (parents/clinicians) to make decisions; however, some youth also
ing. Morally distressing situations that most nurses often experienced reported preferences to make their CTD. Younger children focused on
were related to diminished quality of care due to time constraints, lack daily care decisions related to their treatment, not CTDs. The youngest
of continuity, and unsafe staffing levels. Every fifth nurse often per- ages (<5 years) identified that complying with daily care is a decision.
formed procedures against school-aged children’s will and almost 10 Children and adolescents also articulated their rationale for choices
% of the nurses often gave in to family’s demand not to discuss death made.
with a dying child who asks about it. Experiences of morally distressing Conclusions: Our meta-aggregation provides a world wide descrip-
situations and positive perceptions of ethical climate were negatively tion of child and adolescent cancer treatment decision-making. We
correlated. also identified different contexts throughout the illness continuum
Conclusions: Although conclusions on causality cannot be drawn due that influence communication and decision-making at various stages
to methodological limitations, the results suggest that resilient base- of development. Synthesized findings from child and adolescent voices
line staffing levels and increased continuity of care are essential in will identify evidence-based practice recommendations with a focus on
preventing morally distressing situations. Time should be allowed meeting child and adolescent communication needs.
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S48 of S635 ABSTRACTS

O100 / #1284 A QUALITATIVE STUDY OF O101 / #1354 FEASIBILITY AND ACCEPTABILITY OF AN


CANCER-READINESS IN DANISH SCHOOL CLASSES: MHEALTH APP (BMT4ME) FOR ADHERENCE AND SYMPTOM
ALASSMATES’ PERSPECTIVE ON CHILDHOOD CANCER TRACKING USING A MULTIDISCIPLINARY AND
FAMILY-CENTERED APPROACH IN PEDIATRIC HEMATOPOIETIC
Hanne Larsen1,2 , Mette Weibel1 , Natasha Nybro Petersen3 , Martin STEM CELL TRANSPLANT
Fridh4
1 University of Copenhagen, Rigshospitalet, Paediatric Oncology Research Micah Skeens, Jessica Ralph, Emre Sezgin, Parishma Guttoo, Kathryn
Laboratory, Department Of Paediatrics And Adolescent Medicine, Copen- Vannatta, Rajinder Bajwa, Cynthia Gerhardt
hagen, Denmark; 2 University of Copenhagen, Rigshospitalet, Faculty Of The Abigail Wexner Research Institute at Nationwide Children’s Hospital,
Health Sciences, Copenhagen, Denmark; 3 University hospital of Copen- Center For Biobehavioral Health, Columbus, United States of America
hagen, Rigshospitalet, Paediatric Oncology Research Laboratory, Depart-
ment Of Paediatrics And Adolescent Medicine, København, Denmark; Background and Aims: Background/Aims Medication non-adherence
4 Copenhagen University Hospital, Rigshospitalet, Department Of Pediatrics rates in children undergoing pediatric hematopoietic stem cell
And Adolescent Medicine, The Juliane Marie Center, Copenhagen, Denmark transplant (HCT) are suboptimal (52-78%) and worsen over time.
Complexity of treatment regimens and forgetfulness are common
Background and Aims: On school return after treatment completion causes of non-adherence. A paucity of research has examined
50% of children with cancer struggle socially and academically. This the benefit of implementing digital health applications with HCT
study investigates how social relations between children with cancer families to improve adherence. We aim to assess the feasibility
and classmates are affected by the cancer trajectory. and acceptability of a mobile health app (BMT4me) to improve
Methods: By an ethnographic approach, focusing on the classmate’s adherence to immunosuppressants in outpatient HCT by investi-
interaction with the child with cancer, we conducted three months of gating potential use among caregivers and healthcare providers
participant observation before and after school re-entry in six Dan- (HCP).
ish school classes (2end – 7th grade), and 15 semi-structured focus Methods: This study used an iterative mixed methods design to
group interviews with children with cancer (n=2), classmates (n=16) the mobile app prototype development. First, 16 parent/child
and teachers (n=2). dyads (8-17y) completed 1:1 qualitative interviews after viewing
Results: Through daily interactions, in school settings, classmates app wireframes. Then, HCP, including physicians (n=7), nurses
develop a chronic closeness constituted through their physical and (n=13), and advanced practice nurses (n=3), participated in
social interactions. The abruptness of the child with canver’s disap- focus groups by testing the prototype, and evaluating the usabil-
pearance at diagnosis, leaves classmates struggling to navigate the ity of the BMT4me app via the standardized System Usability
presence of absence as an ambiguous interrelation between what is Scale (SUS). Quantitative data were analyzed using descrip-
physically present and not. This experience is intensified by lack of tive statistics. Thematic analysis was used to analyze qualitative
hospital access, longing for news and ways to interact with their ill data.
classmate, and modified where classmates reestablish contact. Dur- Results: Interviews indicated acceptability of the BMT4me app by both
ing treatment, classmates experience how the chronic closeness is caregivers and HCP. Caregivers identified information sharing, medica-
disrupted, and they struggle to reestablish the closeness as they can tion and symptom tracking, and reminders as important features. The
no longer sustain their previous perceptions of their ill classmate. mean SUS score among HCP was μ=84.2, which exceeded the stan-
At treatment completion reentering becomes a process characterized dardized usability score of 68. Providers found BMT4me as a promising
by the ongoing negotiation of the returning classmate’s health, and tool for outpatient management. Thematic analysis resulted in major
new ways to include their classmate in the evolving classroom cul- themes of ensuring patient safety and strategies for caregiver engage-
ture. Accordingly, children with cancer and their classmates engaged ment. Both caregivers and HCP emphasized the importance of sharing
in activities that aims at defining their collective identity and rules of the collected information in BMT4me via the electronic medical
proper behavior. record.
Conclusions: Classmates struggle to navigate the intensified absence Conclusions: Multi-stakeholder engagement is essential to the devel-
and disrupted chronic closeness. We propose the concept of cancer- opment of an acceptable mHealth intervention. Findings identified
readiness referring to a process whereby classmates are increasingly highly usable features and refinement of BMT4me functions to
accommodating of their ill classmate in a dynamic classroom culture improve its utility for families, patients, and providers. Future research
and cancer-related challenges. A prerequisite for facilitating cancer- should continue examining the use of digital interventions and their
readiness is understanding social rehabilitation as a relational and impact on clinical outcomes in children with complex treatment regi-
intersubjective experience, which must be addressed collectively. mens.
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ABSTRACTS S49 of S635

AWARD SESSION Conclusions: This study shows a strong association between BM infil-
tration levels and EFS and OS at different timepoints. End of induction
SIOP AWARD SESSION 29-09-2022 4:40 PM - 6:10 PM BM positivity by qPCR is significantly associated with poor survival and
identifies patients at risk for relapse.
O102 / #978 DETECTION OF MINIMAL RESIDUAL DISEASE
(MRD) IN HIGH RISK NEUROBLASTOMA IS ASSOCIATED WITH
SURVIVAL OUTCOMES: FINAL RESULTS OF INTERNATIONAL O103 / #1217 RHABDOID TUMOR PREDISPOSITION
GPOH-DCOG MRNA QPCR PROSPECTIVE VALIDATION STUDY SYNDROME (RTPS) – FINDING EVIDENCE BY SYSTEMATIC
ANALYSES
Lieke Van Zogchel1,2 , Boris Decarolis3 , Esther Van Wezel1 , Lily
Zappeij-Kannegieter1 , Nina Gelineau1,2 , Roswitha Karolina Nemes1 , Susanne Bens2 , Pascal Johann1,3 , Mona Steinbügl1 ,
Schumacher-Kuckelkorn3 , Thorsten Simon3 , Frank Berthold3 , Hubert Michaela Kuhlen1 , Rhoikos Furtwängler4 , Uwe Kordes5 , Christian
Caron4 , Marta Fiocco5,6,7 , C. Ellen Van Der Schoot1 , Barbara Hero3 , Vokuhl6 , Martin Hasselblatt7 , Thomas Kröncke8 , Brigitte Bison8 ,
Janine Stutterheim2 , Lieve Tytgat1 Patrick Melchior9 , Beate Timmermann10 , Joachim Gerss11 , Reiner
1 Sanquin Research and Landsteiner Laboratory of the AMC- University Siebert2 , Michael C. Frühwald1
of Amsterdam, Department Of Experimental Immunohematology, Ams- 1 University Medical Center Augsburg, Paediatric And Adolescent Medicine,

terdam, Netherlands; 2 Princess Maxima Center for Pediatric Oncology, Swabian Children’s Cancer Center, Augsburg, Germany; 2 Ulm Univer-
Research, Utrecht, Netherlands; 3 Children’s Hospital - University of Cologne, sity & Ulm University Medical Center, Institute Of Human Genetics,
Department Of Pediatric Hematology And Oncology, Cologne, Germany; Ulm, Germany; 3 German Cancer Consortium (DKTK), German Cancer
4 Hoffmann-La Roche Ltd, Research, Basel, Switzerland; 5 Princess Maxima Research Center (DKFZ), Division Of Pediatric Neurooncology, Heidelberg,
Center for Pediatric Oncology, Trial And Data Center, Utrecht, Netherlands; Germany; 4 Saarland University Medical Center, Department For Pedi-
6 Leiden University Medical Center, Medical Statistics Section, Depart- atric Oncology And Hematology, Homburg, Germany; 5 University Hospital
ment Of Biomedical Data Science, Leiden, Netherlands; 7 Leiden University, Hamburg-Eppendorf, Department Of Pediatric Hematology And Oncol-
Mathematical Institute, Leiden, Netherlands ogy, Hamburg, Germany; 6 University Hospital Bonn, Section Of Pediatric
Pathology, Department Of Pathology, Bonn, Germany; 7 University Hospi-
Background and Aims: Bone marrow (BM) invasion, detected by neu- tal Münster, Institute Of Neuropathology, Münster, Germany; 8 University
roblastoma (NB) specific mRNA real-time quantitative PCR (qPCR) Medical Center Augsburg, Department Of Diagnostic And Interventional
has been shown to be associated with survival outcomes. We there- Radiology And Neuroradiology, Augsburg, Germany; 9 Saarland Univer-
fore performed a prospective NB mRNA qPCR validation study in sity Hospital, Department Of Radiation Oncology, Homburg, Germany;
345 high-risk NB patients, treated in NBL2004 (GPOH) and NBL2009 10 University Hospital Essen, Department Of Particle Therapy, West Ger-
(DCOG). man Proton Therapy Centre Essen (wpe), West German Caner Center
Methods: From 345 high-risk NB patients, serial BM samples were (wtz), German Cancer Consortium (dktk), Essen, Germany; 11 University

prospectively collected between 2009 and 2017: at diagnosis, after of Münster, Institut Für Biometrie Und Klinische Forschung, Münster,
2 courses and end of induction. qPCR was performed using five NB- Germany
markers: PHOX2B, TH, DDC, CHRNA3 and GAP43. qPCR results
were compared to BM immunocytology. Association between BM- Background and Aims: Individuals with rhabdoid tumor predispo-
infiltration levels and event-free survival (EFS) and overall survival sition syndrome (RTPS1 – SMARCB1, RTPS2 – SMARCA4) have a
(OS) was studied using Kaplan-Meier’s methodology and multivari- propensity to develop malignant rhabdoid tumors (MRT). Affected
ate Cox regression model with age and MYCN amplification as risk patients typically present < age 12 months with synchronous tumors
factors. (SYN) exhibiting an unusually aggressive clinical behavior. Due to the
Results: BM infiltration >10% by qPCR at diagnosis was prognostic rarity of RTPS, standards for management are evolving.
for survival: the adjusted hazard ratio was 1.82 [95% CI 1.25-2.63] Methods: Clinical, genetic, and treatment data of 90 patients with
and 2.04 [1.33-3.14] for EFS and OS, respectively. After 2 courses, RTPS from 16 countries were analyzed (2004 – 2020). Therapy fol-
the adjusted hazard ratio for BM-infiltration >1% was equal to 1.79 lowed the EU-RHAB recommendations. Tumors and matching blood
[1.07-3.00] and 2.39 [1.37-4.15] for EFS and OS respectively. Poor out- samples were investigated for SMARCB1 and/or SMARCA4 muta-
come was seen for post-induction BM-positivity. The 5-years EFS and tions using FISH, MLPA and Sanger sequencing. DNA-methylation
OS (SE) were 27% (5.2) and 44% (5.9) for qPCRpositive patients, versus subgroups of ATRT were determined using DNA methylation
60% (6.7) and 66% (6.6) or qPCRnegative patients (p<0.0001). In mul- arrays.
tivariate Cox regression model, qPCR positivity was associated with Results: The median age at diagnosis of 52 girls and 38 boys was
EFS and OS, with a hazard ratio of 2.10 [1.27-3.49] and 1.76 [1.00- 5.5 months (0 – 203). 55.5% (50/90) of patients presented with
3.08] respectively. In contrast, immunocytology positivity at the end of an atypical teratoid/rhabdoid tumor (ATRT), 23.5% (21/90) demon-
induction was not associated with EFS or OS (HR 1.14 [0.65-2.00] and strated SYN, and 21% (19/90) extracranial MRT. RTPS1 was present
1.13 [0.61-2.12]). in 84 patients, RTPS2 in six patients. In 77% (65/84) complete data
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S50 of S635 ABSTRACTS

on SMARCB1 mutational status were generated. Methylation sub- dation through nonsense mediated decay. Moreover, gene expression
group status was available in 59% (40/68) of ATRT or SYN. The profile and chromatin landscape of IKAROS KD cells shift towards a
5-year overall- (OS) and event free survival (EFS) rates of patients more myeloid profile, suggesting IKAROS is required to conserve B-cell
with RTPS1 were 19.8 ± 4.8% and 15 ± 4.2%, respectively. Age identity of B-ALL cells. Furthermore, when CART19 cells were co-
< 1 year (n=56) at diagnosis (10.1±4.3% vs. 46.7±11.1%), pres- cultured with IKAROS KD cells, they secreted lower levels of IL2, IFNg
ence of SYN (5.3±5.1% vs. 24.8±6%), histological diagnosis (ATRT and were not able to completely kill IKAROS KD cells. These results
vs. eMRT/RTK/SYN) (26.8±7.1% vs. 11.9±5.6%), localized disease suggest IKAROS KD cells, due to their lower CD19 surface expression,
(34.5±8 vs. 8.3±4.6%), and presence of PGV at C-terminal (33±8.6% do not fully trigger CART19 activation.
vs. 9.4±5.3%) were significant prognostic factors for 5-year OS in Conclusions: In summary, we have shown that IKAROS modulates
univariate analysis. CD19 surface expression affecting B-ALL cells capacity to activate
Conclusions: In the largest cohort of patients with RTPS, predictors sig- CART19. This is a new role for IKAROS in antigen escape and CART19
nificant for positive outcome could be detected: age > 1 year, absence failure.
of SYN, histological diagnosis ATRT, localized disease and PGV located
at C-terminal. In our research project, we aim to characterize the com-
plete pheno- and genotype of patients with RTPS to develop a risk O105 / #578 IDENTIFICATION OF IMMUNOSUPPRESSIVE
score including surveillance recommendation. FACTORS IN RETINOBLASTOMA CELL SECRETOMES AND
AQUEOUS HUMOR FROM PATIENTS

O104 / #756 IKAROS MEDIATES ANTIGEN ESCAPE Maria Cuadrado Vilanova1 , Jing Liu2 , Jaume Catala Mora3 , François
FOLLOWING CD19 CAR T CELL THERAPY IN R/R B-ALL Radvanyi2 , Angel Carcaboso1
1 Hospital Sant Joan de Deu, Pediatric Oncology, Barcelona, Spain; 2 Institut

Pablo Domizi1 , Jolanda Sarno1 , Astraea Jager1 , Maria Caterina Curie, Siredo Oncology Center, Paris, France; 3 Hospital Sant Joan de Deu,
Rotiroti1 , Reema Baskar2 , Warren Reynolds2 , Bita Sahaf2 , Sean Ophthalmology, Barcelona, Spain
Bendall2 , Charles Mullighan3 , Allison Leahy4 , Regina Myers4 , Stephan
Grupp4 , Robbie Majzner1 , Elena Sotillo1 , David Barrett5 , Kara Davis1 Background and Aims: The microenvironment of retinoblastoma
1 Stanford University, Pediatrics, Stanford, United States of America; is immunosuppressive. We hypothesized that retinoblastoma cells
2 Stanford University, Pathology, Stanford, United States of America; 3 St secrete soluble factors that induce such protumoral environment.
Jude Children’s Research Institute, Pathology, Memphis, United States of Methods: We analyzed immunohistochemistry markers in 23 patient
America; 4 Children’s Hospital of Philadelphia, Paediatrics, Philadelphia, samples and characterized 105 secreted cytokines of 11 retinoblas-
United States of America; 5 Tmunity, Immunotherapy, Philadelphia, United toma cell models in culture. We quantified the top-secreted cytokines
States of America in aqueous humor from 20 patients.
Results: We detected profuse infiltration of CD163+ protumoral M2
Background and Aims: CD19-directed chimeric antigen receptor T polarized tumor associated microglia/macrophages (TAMs) in eyes
cells (CART19) is the main therapy for relapsed/ refractory B-cell acute enucleated due to cancer progression. Previous treatment of patients
lymphoblastic leukemia (r/r B-ALL). However, some initial responders increased the number of TAMs but did not affect polarization. M2
will undergo CD19Neg relapse. Previously, we reported lower levels microglia/macrophages were almost absent in five eyes obtained from
of IKAROS in a specific pro-B subpopulation prior CART19 adminis- children enucleated due to non-tumoral causes. CD8+ tumor infiltrat-
tration correlate with CD19Neg relapse. Genetic alterations affecting ing lymphocytes (TILs) were moderately abundant in tumor eyes and
IKAROS have been associated with poor response to front-line treat- very scarce in nontumoral ones. The expression of the immune check-
ment. However, the role for IKAROS in CART19 failure has not been point molecule PD-L1 was absent in 95% of the tumor samples, which
described. Hence, we interrogated the role of IKAROS in CD19 loss and is concordant with the finding of FOXP3+ Tregs infiltrating tumors.
CART19 failure. We confirmed the pathology results using single-cell transcriptome
Methods: To that end, we combined multi-omics technologies (ATAC- analysis of one tumor. We identified the cytokines extracellular matrix
seq, RNAseq and CyTOF) with gene silencing or targeted protein metalloproteinase inducer (EMMPRIN) and macrophage migration
degradation approaches. inhibitory factor (MIF), both with reported immunosuppressive activ-
Results: Through RNA silencing and targeted protein degradation, we ity, secreted at high levels by retinoblastoma cells. Gene expression
confirmed that lower levels of IKAROS translate into lower CD19 analysis of a large retinoblastoma cohort and single cell transcrip-
surface expression. To elucidate how IKAROS modulates CD19 expres- tome analysis confirmed that MIF and EMMPRIN were significantly
sion, we performed ATACseq and RNAseq analysis from isogenic upregulated in retinoblastomas. Upon quantification of both proteins
IKAROS wild type (WT) or knock down (KD) B-ALL cell lines. We dis- by immunoassays in liquid biopsies (aqueous humor obtained from
covered a new role for IKAROS in RNA splicing. Indeed, IKAROS KD more than 20 retinoblastoma patients), we found a significant increase
cells retain intron 10 in CD19 RNA, introducing premature stop codons in the concentration of MIF and EMMPRIN in cancer patients, com-
that may result in CD19 truncated protein or induce CD19 RNA degra- pared to 12 non-cancer ones. Finally, we showed that macrophages
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ABSTRACTS S51 of S635

derived from peripheral blood mononuclear cells increased the expres- to limited diagnostic facilities. These results produced in ChildGICR
sion of M2 polarization markers upon exposure to retinoblastoma- collaboration (https://gicr.iarc.fr/childgicr) validate the importance of
conditioned medium or recombinant MIF. registration of nmCNS in children.
Conclusions: The interaction of retinoblastoma cells and
TAMs through soluble factors secreted by tumor cells
explains, at least partially, the cold tumor environment of O107 / #1677 MATERNAL AND PERINATAL RISK FACTORS
retinoblastoma. FOR ACUTE LYMPHOBLASTIC LEUKEMIA IN SÃO PAULO STATE,
BRAZIL

O106 / #1314 IMPACT OF REGISTRATION PRACTICE ON Karina Braga Ribeiro1 , Carolina Luizaga2 , Rosa Freitas3 , Lilian
INCIDENCE OF CHILDHOOD CNS TUMOURS: ASSESSMENT OF Morais3 , Valmir Aranha3 , Monica Teixeira3 , Celia Gianotti Antoneli4 ,
189 POPULATION-BASED CANCER REGISTRIES Bernadette Waldvogel3 , Sidnei Epelman1 , Jose Eluf-Neto5
1 Hospital Santa Marcelina, Pediatric Oncology, Sao Paulo, Brazil;
Manushak Avagyan1 , Murielle Colombet1 , Anastasia Dolya1 , Charles 2 Fundacao Oncocentro de Sao Paulo, Hospital Cancer Registry, Sao
Stiller2 , Eva Steliarova-Foucher1 Paulo, Brazil; 3 Fundacao SEADE, -, Sao Paulo, Brazil; 4 Universidade Nove
1 International Agency for Research on Cancer, Cancer Surveillance Branch, the Julhio, Pediatric Oncology, São Pablo, Brazil; 5 University of Sao Paulo,
Lyon, France; 2 NHS Digital, National Cancer Registration And Analysis School of Medicine, Department Of Preventive Medicine, Sao Paulo, Brazil
Service, Oxford, United Kingdom
Background and Aims: Perinatal and maternal factors have been
Background and Aims: Central nervous system tumours (CNS) rep- consistently associated with acute lymphoblastic leukemia (ALL). The
resent 20% of childhood cancers. Among them, up to 40% are aim of our study was to investigate the association between these
non-malignant (nmCNS). Untreated nmCNS are life threatening. We characteristics and ALL in the state of São Paulo, Brazil.
analysed registration practices of childhood CNS in population- Methods: This is a registry-based case-control study (deterministic
based cancer registries to highlight the importance of registration of linkage based on name, mother’s name, and date of birth) including
nmCNS. 1032 children <6 years with ALL, diagnosed between 2001 and 2019,
Methods: Tumours classified as intracranial and intraspinal in the retrieved from the São Paulo Hospital-based Cancer Registry (FOSP)
International Classification of Childhood Cancer were extracted and 5160 controls (5:1, frequency-matched by year of birth, gender,
from the database of the International Incidence of Childhood Can- and city of birth), identified through birth certificates (SEADE Founda-
cer volume 3 study. Overall, 189 registries operating in 82 coun- tion). Crude (cOR) and adjusted Odds Ratios (aOR) (with corresponding
tries and territories, covering populations aged 0-14 over variable 95% CI) were estimated using unconditional logistic regression.
time periods during 1982-2015, were included. Age-standardised Results: Crude analysis revealed an increased risk of ALL asso-
incidence rates per million (ASR) and their confidence intervals ciated with high birth weight (>4000g compared to 2500-3999g,
(95% CI) were calculated for pools of registries classified by reg- crude OR=1.47, 95% CI 1.07-2.02), as well as a reduced risk linked
istration and coding practice and the Human Development Index to low birthweight (<1500 g, cOR=0.13, 95% CI 0.03-0.52, 1500-
(HDI). 2499g, cOR=0.76, 95% CI 0.57-1.01). A positive association with
Results: Based on a total of 113,539 CNS, the overall ASR=29.94 congenital anomalies was found (cOR=2.43, 95% CI 1.25-4.71). Mater-
(27.0-32.9). For 60 registries registering only malignant CNS tumours, nal age was associated with an increased risk of ALL (>31 years,
the pooled ASR=18.5 (13.7-23.3). For 129 registries with system- cOR=1.34, 95% CI 1.10-1.64 compared to < 21 years). No signif-
atic registration of nmCNS ASR=33.3 (29.8-36.8). Among 101 reg- icant associations were found for the following variables: maternal
istries in countries with very high HDI, 82 (68.3%) registered nmCNS, education, race/ethnicity, prematurity, mode of delivery, plurality,
while among 24 registries in countries with low or medium HDI APGAR score – 1 minute, APGAR score – 5 minutes, and fetal
levels only 9 (37.5%) did. These data included 18,527 cases of pilo- loss. After adjustment for maternal education, mode of delivery
cytic astrocytoma (PA) with pooled ASR=5.3 (3.9-6.6). The total and prematurity, birthweight (<1500 g, aOR=0.11, 95% CI 0.03-
CNS incidence varied according to registration of PA. In the pool 0.47, 1500-2499g, aOR=0.68, 95% CI 0.49-0.94, >4000g, aOR=1.47,
of 39 registries excluding PA ASR=13.0 (8.4-17.6), among 103 reg- 95% CI 1.07-2.02), congenital anomalies (aOR=2.65, 95% CI 1.33-
istries with PA considered non-malignant ASR=31.7 (26.3-37.1), in the 5.26), and maternal age (>31 years, aOR=1.29, 95% CI 1.05-1.59
pool of 26 registries with malignant PA ASR=36.0 (30.4-41.5) and compared to < 21 years) remained as independent risk factors for
among 21 registries coding PA with either behaviour ASR=32.3 (25.1- ALL.
39.5). Conclusions: Our results provide further evidence of associations
Conclusions: Excluding nmCNS from registration implies underestima- between birth weight, maternal age, congenital anomalies, and ALL
tion of CNS incidence and overall cancer burden in children. The spo- in Brazilian children. We could not confirm the association between
radic registration of nmCNS in countries with low HDI may be linked C-sections and ALL.
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S52 of S635 ABSTRACTS

CCI Maternal And Child Department, Cali, Colombia; 5 Fundacion Valle de Lili,
Psychiatry, Cali, Colombia; 6 St. Jude Children’s Research Hospital, Global
O108 / #123 CCI: PROVIDING SUPPORT TO GRIEVING Pediatric Medicine, Memphis, United States of America
FAMILIES 29-09-2022 4:40 PM - 6:10 PM
Background and Aims: Grief in parents has been described as a very
A HEART-WARMING EXPERIENCE: BENEFITS OF intense long-lasting experience, characterized by deep sadness, and
PSYCHOEDUCATIONAL WORKSHOPS FOR PARENTS COPING social isolation, therefore, the recommendation of scientific societies
WITH MOURNING DUE TO PEDIATRIC CANCER in pediatrics is to provide bereavement care to parents and relatives
of deceased children who have previously been cared for by health
Lucila Boncompagni professionals. In this study, a bereavement workshop for parents is
fundacion mateo esquivo, Santa Fe, Santa fe, Argentina proposed as an intervention strategy, providing accompaniment to the
families of deceased patients who received pediatric palliative care
Background and Aims: *To evidence the benefits of psychoeduca- (PPC).
tional workshops for parents who are in the mourning process after Methods: We conducted a quantitative and qualitative study in a
losing a child due to childhood cancer. ⋅* To look into the most frequent focus group of parents of deceased children who participated in a
emotions addressed by parents, during the workshop. ⋅* To value the bereavement workshop. The intervention consisted of a bereavement
role of the professional of psycho-oncology as an emotional support workshop, guided by the multidisciplinary PPC team, in which 4 activ-
during the mourning process. This is a main part of the entire mourning ities were developed: group psychotherapy, music therapy, gratitude
process since the child is ill. activity, symbolic ritual of delivery of butterfly. The gratitude activity
Methods: Narrative review through updated bibliography and analysis consisted of motivating the families to express their gratitude by writ-
of semi-fixed interviews to parents who are in the mourning process, ing on cards with phrases that encourage reflection: during this process
due to childhood cancer. I am grateful for.., I thank my child or family member for. . . , I thank the
Results: Three different concepts were analyzed an the results were: pediatric palliative care program for. . . ; the cards were transcribed into
* Workshops as a learning tool: the topics were about how to return a database constructed for this study and analyzed by 4 evaluators,
to life by communicating with other parents and talking about de thematic categories were assigned to each reflective sentence.
mourning, in a genuine context, without any judging and while trans- Results: 60 families received the personalized letter of condolences
mitting a love message. *The most frequent emotions: they coincide on with invitation to the workshop, 23 families attended the workshop.
the varied and intensive emotions such as confusion, anxiety, sadness. From the gratitude activity, families made 49 thank you cards, the
Moreover, there exist some feelings of love to their children and spir- following categories were highlighted: Communication, Hope, com-
ituality, that give them a sense to those feelings. * Importance of the passion, acceptance, humanization, learning, gratitude, active listening,
perceived emotional support: coincides on the importance of receiving coping strategies, regards, faith and humanization
attention during the treatment and mourning as a continuum. Parents Conclusions: Strategies that favor adequate emotional management
consider the professional a person to trust in, who knows about their of the grieving process should be promoted. This study reflects the
history. They also value the professional’s personalized support, who perceptions of parents attending a bereavement workshop. It high-
focuses on their needs to express feeling. lights the gratitude, feelings and reflections of the family members
Conclusions: Workshops are a key tool to foster learnings of per- during the health process and the death of the child while receiving
sonal, psychological and social resources. Those tools help to improve accompaniment by a pediatric palliative care team.
the quality of life of parents and tight familiar bonds. The psycho-
oncologist could consider the importance of using this therapeutic
resource so as to improve the quality of life of parents in mourning O110 / #248 BEREAVEMENT EXPERIENCES OF MOTHERS
process. AFTER THE DEATH OF A CHILD WITH CANCER: IS A NEW
BEGINNING POSSIBLE?

O109 / #174 CULTIVATING GRATITUDE IN BEREAVED Ayfer Aydın1 , Hülya Bingol2 , Rejin Kebudi3 , Eysan Hanzade Umac4 ,
FAMILIES: DESCRIPTION OF THE IMPACT OF THE Sayime Başak Koç3 , Sema Bay Buyukkapu3 , Ülkü Miray Yıldırım2 ,
BEREAVEMENT WORKSHOP ON FAMILIES OF DECEASED Bulent Zulfikar2
PATIENTS IN THE PEDIATRIC PALLIATIVE CARE PROGRAM 1 Istanbul University, School Of Nursing, ISTANBUL, Turkey; 2 Istanbul Uni-
versity, Oncology Institute, Istanbul, Turkey; 3 Istanbul University, Oncology
Maria Cuervo1,2 , Angela Devia1 , Luisa Pereira1 , Tatiana Alvarez3 , Institute, Pediatric Hematology-oncology, Istanbul, Turkey; 4 Koc University,
Karen Molina1,4 , Jhon Bolaños3 , Isabel Correa5 , Ximena Garcia6 Nursing, Istanbul/Turkey, Turkey
1 Universidad Icesi, Clinical Medical Sciences, Cali, Colombia; 2 Fundacion
Valle de Lili, Family Medicine Department, Cali, Colombia; 3 Fundacion Valle Background and Aims: The death of child is one of the most painful
de Lili, Scientific Research Center, d, Colombia; 4 Fundacion Valle de Lili, events for parents. Bereaved parents attempt to make sense of their
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ABSTRACTS S53 of S635

child’s death in different ways, and they need support to cope with Results: To feel at home in the hospital was fundamental and
this difficult process. This study aimed to understand the experiences, included having good relations with healthcare professionals. Three
expectations, and needs of bereaved parents who lost their children to themes of important values emerged in this context: To be seen
cancer. and treated with respect as a family, To feel trust and be able
Methods: The phenomenological approach with an in-depth interview to hand over care responsibilities and To receive adapted hon-
method was used. The data were collected using semi-structured face- est information. Furthermore, parents experienced “loss of control”
to-face and over the phone with18 mothers who lost their child to related to the child’s illness and treatment. In this situation par-
cancer. Audio recordings of the in-depth interviews were taken and ents described several ethically difficult situations when they did
were then transcribed verbatim. Once the raw data were gathered, the not know what they should do or what was right to do. The sit-
MAXQDA software was used to analyze the data. uations that were perceived as ethically difficult were facing: the
Results: The study data were categorized into 2 themes, and sub- child’s suffering, deficient communication and overwhelming parental
themes. The first theme, “the feelings”, includes feeling not understood, responsibilities.
regret, and loss of desire for life. The second theme, “coping efforts”, Conclusions: When having a child treated for cancer, parents need psy-
comprises making a new beginning, getting emotional support, trying chosocial support as well as adopted honest information. To be able to
to forget, pretending that everything is alright. handle ethically difficult situations, parents might need forums in which
Conclusions: The bereavement process of mothers never ends but, they can reflect upon the ethically difficult situations that arise in care.
they experience different feelings and use different coping methods in Future research is needed on how parents can be involved in ethics
this process. Inadequate usage of home care services, social networks, support.
having another child, and spiritualism affect the bereavement process
of mothers. All nurses working in end-of-life care need to be aware of
the continuing care needs and expectations for communication with O112 / #1413 SUPPORTING PARENT CAPACITY TO
the healthcare team of mothers after the death of their child. Making MANAGE PAIN IN YOUNG CHILDREN WITH CANCER AT
a new beginning is possible by supporting mothers to develop correct HOME: CO-DESIGN AND USABILITY TESTING OF THE
coping methods. PAINCARE APP

Lindsay Jibb1,2 , William Liu3 , Jennifer Stinson2,4 , Paul Nathan5,6 , Julie


NURSING Chartrand7,8 , Nicole Alberts9 , Elham Hashemi1 , Tatenda Masama2 ,
Hannah Pease10 , Lessley Torres10,11,12 , Haydee Cortes10,11,12 ,
NURSING ORAL ABSTRACT PRESENTATIONS: SUPPORTING Michelle Fortier10,11,12
PARENTS AND FAMILIES 29-09-2022 4:40 PM - 6:10 PM 1 Hospital for Sick Children, Child Health Evaluative Sciences, Toronto,
Canada; 2 University of Toronto, Lawrence S. Bloomberg Faculty Of Nurs-
O111 / #1061 PARENTS’ PERSPECTIVES ON IMPORTANT ing, Toronto, Canada; 3 McMaster University, Faculty Of Health Sciences,
VALUES AND ETHICALLY DIFFICULT SITUATIONS WHEN THEIR Hamilton, Canada; 4 Hospital for Sick Children, Child Health Evaluative Sci-
CHILD IS TREATED FOR CANCER: A QUALITATIVE INTERVIEW ences And Chronic Pain Program, Toronto, Canada; 5 University of Toronto,
STUDY Institute Of Health Policy, Management And Evaluation, Toronto, Canada;
6 Hospital for Sick Childrenn, Haematology/oncology, Toronto, Canada;
Charlotte Weiner, Pernilla Pergert, Cecilia Bartholdson 7 University of Ottawa, Faculty Of Health Sciences, Ottawa, Canada;
Karolinska Institutet, Women’s And Children’s Health, Stockholm, Sweden 8 Children’s Hospital of Eastern Ontario, Children’s Hospital Of Eastern
Ontario Research Institute, Ottawa, Canada; 9 Concordia University, Psy-
Background and Aims: When a child suffers from cancer, the whole chology, Montreal, Canada; 10 University of California Irvine, Sue & Bill Gross
family is affected. In connection with the child’s cancer diagnosis and School Of Nursing, Irvine, United States of America; 11 Children’s Hospital
treatment, various ethically difficult situations can arise. So far, the of Orange County, Pediatric Psychology, Orange, United States of America;
majority of research has been conducted focusing on ethically diffi- 12 University of California Irvine, Uci Center On Stress And Health, Irvine,
cult situations from healthcare professionals’ perspectives, and little is United States of America
known about parents’ perspectives. The purpose of this study was to
explore parents’ experiences of important values and ethically difficult Background and Aims: Young children receiving outpatient cancer
situations in Swedish paediatric oncology. care are vulnerable to undermanaged pain due to limited capacities
Methods: This qualitative study is based on data from four focus group for pain self-report and self-management. Digital health solutions that
interviews with parents of children who were/had been treated for provide pain treatment advice to parents in real-time and in any envi-
cancer. Two focus groups with mothers (n = 9) and two with fathers ronment are poised to provide enhanced access to pain care. We used
(n = 7) were performed by a moderator and an observer, using a a parent co-design approach involving iterative rounds of user testing
digital video platform. Data were analysed according to qualitative and app refinement to develop a usable PainCaRe real-time cancer pain
methodology. management app.
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S54 of S635 ABSTRACTS

Methods: We recruited parents of children (2-11 years) with cancer come was survivors’ physical activity levels at 12-month follow-up.
who spent >25% of their cancer care time outside of the hospital Secondary outcomes were survivors’ cancer-related fatigue levels, left-
and who had experienced pain in the previous week. Parents were and right-handgrip strengths, peak expiratory flow rates, and quality of
recruited from two pediatric tertiary care centers and completed 3 life at 6- and 12-month follow-up.
standardized tasks using a PainCaRe prototype. App usability and Results: Between March 2019 and January 2021, 161 parent-child
acceptability was evaluated using the validated System Usability Scale dyads were enrolled and randomly assigned to the intervention group
and a thematic analysis of app testing sessions and interviews. Test- (n=81) or control group (n=80). The intention-to-treat principle was
ing sessions were conducted until data saturation. Interview themes applied. Generalized estimating equation analyses showed significant
were synthesized into actionable revisions to PainCaRe and additional improvement in outcomes in the intervention group compared with
testing rounds were conducted as necessary. the control group: physical activity levels (group-by-time interaction,
Results: Twenty-two parents participated in 3 testing rounds. Overall, 12-month: β, 3.91 [95% CI 3.45 to 4.36; P<0.001]); cancer-related
parents described PainCaRe as an acceptable and potentially clinically fatigue (12-month: β, −9.16 [−11.31 to −7.00; P<0.001]); left-hand
useful pain management tool. The mean system usability score was grip strength (12-month: β, 5.52 [3.70 to 7.33; P<0.001]); right-hand
in the acceptable scale range and, on average, parents required less grip strength (12-month: β, 5.45 [3.62 to 7.27; P<0.001]); peak expi-
than 3 minutes to complete each app task. Usability issues identified ratory flow rate (12-month: β, 28.51 [16.10 to 40.924; P<0.001]), and
and resolved included those related to software malfunction, compli- quality of life (12-month: β, 14.19 [10.84 to 17.54; P<0.001]).
cated app navigation logic, lack of clarity on pain assessment questions, Conclusions: The mHealth-delivered brief MI for parents was effective
and the need for pain management advice specifically targeted at child in promoting the adoption and maintenance of regular physical activity,
developmental stage. mitigating cancer- and treatment-related adverse effects, and improv-
Conclusions: This study provides insight into how co-design can be ing functional capacity, thereby enhancing the quality of life among
used to create a cancer pain management app that is acceptable and survivors of pediatric cancer.
useful to parents. Next steps will include a PainCaRe pilot randomized
controlled trial. It is expected that an acceptable and usable app will
improve pain outcomes for younger children with cancer-related pain. O114 / #584 AN INTERDISCIPLINARY APPROACH TO
IMPROVING EDUCATIONAL ACCESS FOR CHILDREN
DIAGNOSED WITH CANCER
O113 / #555 EFFICACY OF A MHEALTH-DELIVERED BRIEF
MOTIVATIONAL INTERVIEWING FOR PARENTS TO PROMOTE Clifton Thornton1 , Kathy Ruble2 , Juliana Paré-Blagoev3 , Kimberly
REGULAR PHYSICAL ACTIVITY IN SURVIVORS OF PEDIATRIC Milla4 , Lisa Carey4 , Lisa Jacobson4
CANCER: A RANDOMIZED CONTROLLED TRIAL 1 Johns Hopkins University, Nursing, Baltimore, United States of Amer-
ica; 2 Johns Hopkins University, School of Medicine, Pediatric Oncology,
Ankie Tan Cheung1 , William Ho Cheung Li1 , Laurie Long Kwan Ho1 , Baltimore, United States of America; 3 Johns Hopkins University, School
Godfrey Chi-Fung Chan2,3 , Joyce Oi Kwan Chung4 of Education, Education, Baltimore, United States of America; 4 Kennedy
1 The Chinese University of Hong Kong, Nethersole School Of Nursing, Hong Krieger Institute, Neuropsychology, Baltimore, United States of America
Kong, Hong Kong PRC; 2 Hong Kong Children’s Hospital, Department Of
Paediatrics And Adolescent Medicine, Hong Kong, Hong Kong PRC; 3 The Background and Aims: Neurocognitive effects of pediatric cancer
University of Hong Kong, Lks Faculty Of Medicine, Hong Kong, Hong Kong substantially impact survivors’ education – a pivotal social determi-
PRC; 4 The Hong Kong Polytechnic University, School Of Nursing, Hong Kong, nant of health. An interdisciplinary (nursing, neuropsychology, special
Hong Kong PRC education) quality improvement project identified the degree to which
these issues go unaddressed and gaps in practice and then developed
Background and Aims: Physical activity exerts beneficial effects resources to fill this gap that can be translated to pediatric oncology
that attenuate many cancer- and treatment-related late effects for practices in a multitude of settings.
survivors of pediatric cancer. However, most survivors tend to be phys- Methods: Family interviews informed a survey for parents and clin-
ically inactive to confer the health benefits associated with physical icians across the United States to describe school integration expe-
activity. This study aimed to examine the efficacy of a mHealth- riences and identify barriers (n=203 parents, n=282 clinicians). In a
delivered brief motivational interviewing for parents on promoting stakeholder-informed process, continuing education courses, commu-
regular physical activity among survivors of pediatric cancer. nication support resources, and return-to-school materials for families
Methods: An assessor-blinded, multicentered randomized controlled were developed, integrated into practice, and evaluated.
trial was conducted at two pediatric oncology outpatient clinics in Results: Many (48%) parents denied receiving information about neu-
Hong Kong. Survivors of pediatric cancer aged 9–16 years and their rocognitive impacts and 51% felt inadequately prepared for school.
parents (either mother or father) were randomized 1:1 to receive Of those who received information, higher parental preparedness
either a 6-month brief motivational interviewing delivered via mobile was associated with lower stress, socialization, fitting-in, and anxi-
applications using a menu of strategies or usual care. The primary out- ety when returning to school (OR 1.58-2.28, p<0.02). Most (54%)
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ABSTRACTS S55 of S635

oncologists had no neurocognitive-based training, 42% lacked insti- tive correlation between resilience and quality of life scores (r =0.31, P
tutional guidelines for managing effects, and most avoided discussing < .01). Results indicate that greater parental resilience was associated
this topic with families. International clinicians (n=314) enrolled in with better quality of life, fewer depressive symptoms and lower levels
the online continuing education course with high satisfaction scores. of anxiety.
After integrating developed resources, neuropsychology referral rates Conclusions: The findings of this study add further support that
increased by 42%. Over 80% of clinicians used documentation sup- resilience is a crucial factor to maintain parents’ psychological well-
ports and discussed neurocognitive effects of treatment in 183 patient being in the face of stress and adversity. Given that parents play a
interactions pivotal role in promoting the physical and psychological well-being of
Conclusions: Education is imperative to address in children with can- their child at every step of the cancer journey, it is vital to develop
cer. Parents and clinicians report inattention and lack of knowledge interventions that can enhance resilience for parents and foster the
in this domain, but continuing education programs are well-received. development of their coping mechanisms and positive psychological
Communication resources improve patient-provider discussions and well-being.
referrals to neuropsychology services. In areas where these services
are not available, educating families can support improved school
integration. This 4-year project identifies a gap in holistic care of all O116 / #1264 EXPERIENCE OF SETTING UP A DAYCARE
children with cancer, provides a cost-effective solution translatable ONCOLOGY CENTRE AT A TERTIARY HOSPITAL IN NAIROBI,
to many pediatric oncology institutions, and underscores importance KENYA
of interdisciplinary-informed care delivery to establish standards that
address all aspects of care. Winnie Njuguna
Gertrude’s Children’s Hospital, Nursing-oncology, Nairobi, Kenya

O115 / #808 A DESCRIPTIVE STUDY OF THE RESILIENCE, Background and Aims: Treatment of pediatric oncological conditions
PSYCHOLOGICAL WELL-BEING AND QUALITY OF LIFE IN is a long and resource-intensive process. The total cost, for example, of
PARENTS OF CHILDREN WITH CANCER treatment for acute lymphoblastic leukemia (ALL), can be up to 10,000
USD per child in Kenya. Treatment at Gertrude’s Children’s Hospital
Joyce Oi Kwan Chung1 , William Ho Cheung Li2 (GCH) had been fully in-patient until December 2018 when a daycare
1 The Hong Kong Polytechnic University, School Of Nursing, Hong Kong, oncology service was started, in an effort to reduce costs and improve
Hong Kong PRC; 2 The Chinese University of Hong Kong, Nethersole School efficiency. Doctors, nurses and a pediatric oncologist, run the service.
Of Nursing, Hong Kong, Hong Kong PRC Methods: All pediatric oncology patients who received outpatient
chemotherapy treatment in GCH from December 2018 to August 2021
Background and Aims: The diagnosis of cancer not only undermines were included in the study. The range of services offered, medications
children themselves in physical and psychological, but also it brings administered, through-put time and adverse events encountered were
overwhelming psychological distress for these children’s parents. Par- outlined. Microsoft Excel 2019 was used to analyze treatment costs
ents as the carers have to bear a great burden, both physically and for ALL during the interim maintenance phase, comparing outpatient
psychologically, in taking care of their child throughout the cancer treatment costs to the standard inpatient treatment cost.
trajectory. This study aimed to describe the resilience, psychologi- Results: The unit served 35 oncology patients, recording a total of
cal well-being and quality of life of Hong Kong Chinese parents of 905 visits. Medications given included doxorubicin, vincristine, vin-
children with cancer, which is an essential prerequisite for designing blastine, actinomycin D, cytarabine, L-asperaginase, bleomycin and
appropriate psychological interventions to build parents’ resilience, methotrexate. Cost of treatment for ALL during interim mainte-
consequently enhance their quality of life. nance phase was about 40% cheaper in the outpatient versus the
Methods: A cross-sectional study was conducted to 109 parents of inpatient setting [USD 2230 versus USD 3900]. The average through-
children (aged 0-16 years) with cancer admitted for treatment at put time for delivering intrathecal chemotherapy with sedation was
Hong Kong Children’s Hospital. Parents’ levels of resilience and anxi- 310 (237-390) minutes, while that for intravenous infusions was
ety, depressive symptoms, coping strategies, perceived social support 225 (180-270) minutes in daycare versus an average of 48hrs in
and quality of life were assessed after obtaining the written informed inpatient. One case of a severe adverse event was reported, with
consent. a child developing emesis and dehydration post discharge from
Results: indicated that almost half (48.6%) of the parents displayed daycare.
some depressive symptoms and were potentially at risk for depression. Conclusions: Outpatient care, during certain phases of treatment, can
There were high negative correlations between resilience and depres- be a cost saving, efficient and safe strategy in treatment of childhood
sive symptoms scores (r = -0.52, P < .01) and between resilience and cancer. It should, therefore, be highly considered in low and middle
anxiety scores (r = -0.60, P < .01). Moreover, there was medium posi- income settings.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S56 of S635 ABSTRACTS

IPSO diameter of the primary tumor (p = 0,0043), high levels of lactate dehy-
drogenase (LDH) (p < 0.0001) and low levels of urinary vanilmandelic
IPSO BEST POSTER SESSION 30-09-2022 8:30 AM - 9:30 AM acid (VMA) (p < 0.0001). Two patients needed emergency admission
to ICU; factors associated with the risk of ICU admission were stage
O117 / #1349 CLINICAL FEATURES AT DIAGNOSIS ARE 2 hypertension (p = 0,0229), high levels of LDH (p < 0.0001) and low
ASSOCIATED WITH THE NEED OF EMERGENCY INVASIVE levels of urinary VMA (p < 0.0001).
PROCEDURES AND INTENSIVE CARE UNIT ADMISSION IN Conclusions: Specific clinical, radiological and laboratory features at
PATIENTS WITH HIGH-RISK NEUROBLASTOMA diagnosis are associated with the need of emergency invasive proce-
dures and ICU admission in patients affected by high-risk neuroblas-
Giorgio Persano1 , Valerio Voglino1 , Alessandro Crocoli2 , Aurora toma.
Castellano3 , Annalisa Serra3 , Laura Del Prete4 , Ugo Giordano5 , Gian
Luigi Natali6 , Pier Luigi Di Paolo7 , Cristina Martucci2 , Daniela
Pacella8 , Alessandra Stracuzzi9 , Alessandro Inserra2 O118 / #436 NON-CONTRAST MRA AS NON-INVASIVE
1 Bambino Gesù Children’s Hospital IRCCS, Surgical Oncology Unit – General VASCULATURE IMAGING FOR PEDIATRIC RENAL TUMOR
And Thoracic Surgery Unit, Department Of Surgery, Rome, Italy; 2 Bambino SURGERY, A PEDIATRIC SURGICAL PERSPECTIVE
Gesù Children’s Hospital IRCCS, Rome, Italy, Surgical Oncology Unit –
General And Thoracic Surgery Unit, Department Of Surgery, Rome, Italy; Matthijs Fitski1 , Guus Bökkerink1 , Marc Wijnen1 , Cornelis Van De
3 Ospedale Bambino Gesù IRCCS, Dipartimento Di Onco-ematologia E Ter- Ven1 , Annemieke Littooij2 , Aart Klijn3 , Alida Van Der Steeg1
apia Cellulare E Genica, Rome, Italy; 4 Bambino Gesù Children’s Hospital 1 Princess Máxima Center for Pediatric Oncology, Pediatric Surgery, Utrecht,

IRCCS, Rome, Italy, Pediatric Urology Unit, Department Of Surgery, Rome, Netherlands; 2 University Medical Center Utrecht / Wilhelmina Chil-
Italy; 5 Bambino Gesù Children’s Hospital IRCCS, Rome, Italy, Sport And dren’s Hospital, Radiology And Nuclear Medicine, Utrecht, Netherlands;
Hypertension Medicine Unit, Department Of Cardiac Surgery, Cardiology, 3 University Medical Center Utrecht / Wilhelmina Children’s Hospital, Pedi-
Heart And Lung Transplant, Rome, Italy; 6 Bambino Gesù Children’s Hos- atric Urology, Utrecht, Netherlands
pital IRCCS, Rome, Radiology Unit, Department Of Diagnostic Imaging,
Rome, Italy; 7 Bambino Gesù Children’s Hospital IRCCS, Rome, Italy, Radi- Background and Aims: Surgical resection of renal tumors in children
ology Unit, Department Of Diagnostic Imaging, Rome, Italy; 8 University of is an essential part of treatment. To reduce complications and improve
Naples “Federico II”, Naples, Italy, Department Of Public Health, Naples, surgical understanding of renal arterial vasculature, a high-resolution
Italy; 9 Children’s Hospital Bambino Gesù, IRCSS, Pathology, ROME, Italy Non-Contrast Magnetic Resonance Angiography (NC-MRA) sequence
was implemented. We retrospectively and prospectively assessed the
Background and Aims: Neuroblastoma is the most common solid use of NC-MRA for the preoperative planning of Wilms Tumor surgery.
extracranial tumor in children. Approximately one third of these Methods: The preoperative MRI scans of all patients with a kidney
patients are stratified into high risk group. High-risk patients receive tumor between October 2019 and July 2021 were retrospectively
multimodal treatments, including chemotherapy, surgery and radiation assessed by two pediatric surgeons and one pediatric urologist. The
therapy. Some of these patients also undergo additional emergency surgeons assessed their understanding of the aorta, renal artery, extra-
procedures due to acute complications during induction chemother- parenchymal artery and intraparenchymal artery in the T2-Weighted
apy. The aim of the present study is to find clinical, radiological and sequence (T2W), Contrast-Enhanced MRA (CE-MRA) and NC-MRA.
laboratory parameters associated with the risk of complications. Moreover, they assessed whether the sequence helped them during
Methods: The clinical notes of patients diagnosed with high-risk the preoperative planning. All assessments were based on a 5-point
neuroblastoma from January 2013 until February 2022 were retro- scale. Additionally, the scans of 10 patients undergoing a surgical resec-
spectively reviewed. Primary outcomes were the need of emergency tion were assessed prospectively by one of the performing surgeons.
invasive procedures, including surgery, endoscopy and interventional Results: For the retrospective assessment we included 37 patients.
radiology, and Intensive Care Unit (ICU) admission. Clinical, radiologi- The median additional scan time for NC-MRA was 209 seconds. The
cal and laboratory data at diagnosis were recorded and the correlation NC-MRA visualized the intraparenchymal arteries more accurately
with the primary outcomes was analyzed. than the CE-MRA (3 vs 1 out of 5). Moreover, there was no signif-
Results: In the examined period, 50 patients were diagnosed with icant difference between the scoring of the NC-MRA and T2W at
high-risk neuroblastoma. Six patients required emergency invasive this anatomical region. The NC-MRA sequence was considered useful
procedures: two patients underwent thoracostomy tube placement, during surgical planning (4 out of 5) but there was no significant dif-
one patient underwent thoracostomy tube placement and angio- ference from the T2W sequence. During the prospective assessment,
graphic embolization for hemorrhage and three patients underwent the NC-MRA improved the differentiation of vessels at the level of the
endoscopic ureteral stent placement. Patients who needed emergency intraparenchymal arteries and was therefore considered most useful
procedures presented with severe (stage 2) hypertension (p = 0.0001), for patients undergoing nephron-sparing surgery.
high number of total and vascular Image Defined Risk Factors (IDRF) Conclusions: NC-MRA is considered a helpful additional imaging
of the primary mass (p = 0,028 and p = 0,0068, respectively), larger sequence for the preoperative planning of Wilms Tumor surgery,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S57 of S635

especially for nephron-sparing surgery. For this type of surgery, it may Vishesh Jain1 , Nellai Krishnan1 , Sandeep Agarwala1 , Anjan Dhua1 ,
also possibly replace a preoperative CTA of the kidney. Sameer Bakhshi2 , Manisha Jana3 , Devasenathipathy Kandasamy3 ,
Akshay Bisoi4 , Minu Bajpai1 , Devendra Yadav1 , Prabudh Goel1
1 All India Institute of Medical Sciences, Department Of Pediatric Surgery,
O119 / #136 HOW TO ACCURATELY MEASURE VOLUMES IN New Delhi, India; 2 All India Institue of Medical Sciences, Medical Oncol-
WILMS TUMORS ON MRI ogy, New Delhi, India; 3 All India Institute of Medical Sciences, Department
Of Radiodiagnosis, New Delhi, India; 4 Cardio-thoracic Sciences Centre, All
Myrthe Buser1 , Alida Van Der Steeg2 , Marc Wijnen2 , Matthijs Fitski1 , India Institute of Medical Sciences, Department Of Cardiothoracic And
Heleen Van Den Nieuwenhof1 , Marry Van Den Heuvel-Eibrink3 , Vascular Surgery, New Delhi, India
Annemieke Littooij4 , Bas Van Der Velden5
1 Princess Máxima Center for Pediatric Oncology, Pediatric Surgery, Utrecht, Background and Aims: Intracaval thrombus is present in 4%-10% of
Netherlands; 2 Princess Maxima Center, Surgery, Utrecht, Netherlands; Wilms tumor (WT) patients. The aim is to present our experience in the
3 Princess Máxima Center for Pediatric Oncology, Division Of Pediatric management and outcome of WT with intracaval thrombus.
Oncology, Utrecht, Netherlands; 4 Princess Máxima Center for Pediatric Methods: All children with WT with intracaval thrombus who pre-
Oncology, Pediatric Oncology, Utrecht, Netherlands; 5 UMC Utrecht, Image sented to us from July 2000 to December 2017 were reviewed retro-
Sciences Institute, Utrecht, Netherlands spectively. We evaluated the tumor stage, management and outcomes
in these patients.
Background and Aims: In pediatric patients with Wilms tumor, radiol- Results: Thirty-four patients were included in the study. The median
ogists measure tumor diameter on MRI in three directions to calculate age of presentation was 48 months (11- 84 months). Fifteen (44%) of
clinical tumor volume as a sphere. Manual delineation of the tumor these patients had Stage IV disease. Preoperative chemotherapy was
(i.e. segmentation) does not assume any shape, and might therefore given in 32 (94%), with a median duration of 8 weeks. Intracaval throm-
be more accurate in assessing tumor volume. The aim of our study bus completely resolved in 9 (26%) after neoadjuvant chemotherapy.
was to assess whether manual segmentation yields more accurate Surgical intervention for residual IVC thrombus was performed in 32
volume measurements of paediatric renal tumors than clinically used patients; of which cavotomy in 17 (50%), excision of part of the wall
radiological volume measurements. of the IVC and patch repair in 1 (3%) and cavectomy in 5 (15%). The
Methods: Radiological volume measurements calculated with the median follow-up was 30 months (5 −150 months). At the last follow-
largest tumor diameter measurements in three directions by a dedi- up, 25 patients (73%) were alive and disease-free. The 5-year overall
cated pediatric radiologist. Segmentation-based tumor volumes were survival and event-free survival were 67% (95 CI, 50%-84%) and 54%
derived from manual tumor segmentations made by three observers (95 CI, 35%-75%). The overall survival in children with non-metastatic
under supervision of a pediatric radiologist. Radiological tumor volume disease (94%) was significantly higher than in those with metastases
measurements and the manual segmentation-based volume measure- (29%; p< 0.01). The overall survival in children with complete resolu-
ment were tested for significance using Wilcoxon’s signed rank test. A tion of IVC thrombus (100%) was significantly higher than in those with
P-value < 0.05 was considered statistically significant. persistent thrombus (48%; p=0.025).
Results: Forty-three patients were included with a mean age of 38 Conclusions: The management of WT can be complicated by the
months (range: 7 – 109 months) at diagnosis. In total, 47 tumors in presence of caval thrombus. Patients with metastasis have a signifi-
43 patients were analyzed. Twelve tumors had a smaller volume when cantly poor outcome. Patients in whom, there is complete resolution of
calculated by manual segmentation in comparison to the currently intracaval thrombus on neoadjuvant chemotherapy have significantly
used radiological measurements. One tumor had an identical mea- higher overall survival.
surement with both methods. In all other tumors (N=34), radiological
measurements were an underestimation of tumor volume. On aver-
age, radiological tumor volume measurements underestimated tumor O121 / #1908 MUSCLE MASS CHANGES DRAMATICALLY
volume by 12% (P=0.0001). BEFORE SURGERY IN CHILDREN WITH WILMS TUMOR
Conclusions: Manual segmentation-based volume measurements dif-
fer significantly from radiological volume measurements. Since the Wilson De Oliveira Jr1 , Mariana Murra2 , Leticia Tufi3 , Carlos Eduardo
manual-segmentation volume measurements do not assume a spheri- Cavalcante4 , Roberta Da Silva2 , Marco De Oliveira5 , Bianca Rosa6 ,
cal tumor shape but follow the tumor countours, this method is more Alessandra Sousa1 , Rodrigo Ribeiro7 , Elena Ladas8 , Ronald Barr9
accurate. 1 Hospital de Amor: Unidade Infanto Juvenil, Pediatric Surgery, Barretos,
Brazil; 2 Hospital de Amor, Pediatrics, Barretos, Brazil; 3 FACISB - Faculdade
de Ciencias da Saúde de Barretos "Dr Paulo Prata", Barretos School Of
O120 / #1209 MANAGEMENT AND OUTCOME OF WILMS Medicine, Barretos, Brazil; 4 Hospital de Amor: Unidade Infantojuvenil, Pedi-
TUMOR WITH INTRACAVAL THROMBUS: SEVENTEEN-YEAR atric Radiology, Barretos, Brazil; 5 Hospital de Amor, Center Of Epidemiology
EXPERIENCE FROM A TERTIARY CARE CENTER And Biostatistics, Barretos, Brazil; 6 BARRETOS CANCER HOSPITAL (AMA-
ZON), Pediatric Surgery, PORTO VELHO, Brazil; 7 BARRETOS CHILDRENS
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S58 of S635 ABSTRACTS

CANCER HOSPITAL, Pediatric Surgery, Barretos, Brazil; 8 Columbia Univer- indwelling catheters and tumour type. Continuous variables were anal-
sity Irving Medical Center, Division Of Pediatric Hematology/oncology/stem ysed with the t-test and Mann-Whitney U test, categorical variables
Cell Transplant, New York, United States of America; 9 McMaster Unversity, with a Fishers exact test. A p value <0.05 was considered statistically
Pediatrics, Hamilton, Canada significant.
Results: We identified 28 patients (12 male). Median age was 3 years
Background and Aims: The therapeutic approach to Wilms tumor (4 months- 15 years). Twenty seven patients (96.4%) received a single
(WT) is multidisciplinary and leads to significant patient impairment, dose of antibiotics at induction of anaesthesia. Eight patients (28.6%)
increasing the risk of nutritional compromise and malnutrition. Chil- had post-operative pyrexia in the immediate post-operative period;
dren with cancer are vulnerable to sarcopenia, which has been rec- median time from procedure to pyrexia was 1 day (0-2). Of these
ognized as a negative impact of anticancer therapy. Recent studies patients, 5 (62.5%) received antibiotic treatment, with a median dura-
have highlighted reduction in the total psoas muscle area (TPMA) to be tion of 3.5 days (0-7). Two patients (25%) treated with antibiotics had
associated with a poor prognosis in many pediatric diseases, including a proven source of infection (urinary tract, lower respiratory tract).
cancer. This study aims to evaluate changes in the TPMA compartment There was no correlation between post-operative pyrexia and type of
during the treatment of children with WT. tumour, presence of indwelling catheters or duration of anaesthesia.
Methods: Observational, longitudinal, and retrospective study evalu- Conclusions: In our experience, a quarter of patients undergoing
ating children (1 – 14 years, n=38) with WT between 2014 and 2020. intra-abdominal tumour resection had early post-operative pyrexia,
TPMA was assessed by analyzing previously collected, electronically three-quarters of whom have no proven septic focus. A single dose of
stored, CT images of the abdomen obtained at three time points: diag- antibiotic prophylaxis at induction of anaesthesia provides adequate
nosis, preoperatively, and 1-year after surgery. Height, weight, and antimicrobial cover although clinicians need to be alert to the potential
BMI were collected at the same designated study time points. For all requirement for treatment of sepsis.
patients, Z-scores of weight/age and BMI/age were calculated as per
the current guidelines and TPMA/age with an online calculator.
Results: Our data show a high incidence of sarcopenia (55.3%) at diag- O123 / #1137 RISK OF MALIGNANCY FOR EACH BETHESDA
nosis which increased progressively after 4 to 6 weeks of neoadjuvant CLASS IN PAEDIATRIC THYROID NODULES; AN UPDATE
chemotherapy (73.7%) and remained high (78.9%) one year after the
surgical procedure. Tomomasa Hiramatsu, Margaret Mutch, Gideon Sandler
Conclusions: Using TPMA/age Z-scores curves we have found signifi- The Children’s Hospital at Westmead, Department Of Paediatric Surgery,
cant and rapid muscle loss in children with WT, with little or no recovery Westmead, Sydney, Australia
in the study period, indicating the need for further studies to evaluate
the provision of individualized nutritional support for these patients. Background and Aims: We previously reported our outcomes of FNA
for thyroid nodules in Paediatric population by The Bethesda System
for Reporting Thyroid Cytopathology (BSRTC) which demonstrated
O122 / #1322 POST-OPERATIVE PYREXIA IN PAEDIATRIC difference between adults and children in implied risk of malignancy
ONCOLOGY PATIENTS FOLLOWING MAJOR ABDOMINAL by each class. The aim of this study was to evaluate consecutive thy-
RESECTION roid nodules that underwent FNA following the previous study at our
institute.
Sesi Hotonu, Philip Hammond Methods: We conducted a retrospective review of patients who under-
Royal Hospital for Children and Young People, Paediatric Surgery, Edin- went FNA on thyroid nodules at our institute between August 2013
burgh, United Kingdom and March 2022. We reviewed patients’ demography, nodule size on
ultrasound, BSRTC class (B-I to VI), management after biopsy, and
Background and Aims: Pyrexia in the immediate post-operative histopathological outcome if applicable. Additionally, we assessed data
period is a well-recognized physiological response to major surgery. with our previous data combined.
Due to potential sepsis in oncology patients, empirical treatment with Results: 55 nodules in 51 patients underwent FNA. Mean age was 12.8
broad spectrum antibiotics is often given in response to pyrexia. Antibi- years. Numbers of nodules reported as Bethesda I to VI (B-I to VI) were
otic stewardship remains critically important in the prevention of multi 3,30,12,6,0,4, respectively. Among B-III nodules, nine cases underwent
drug resistant pathogens. This study aims to determine the nature of resection or re-biopsy, with two (22%) revealed malignancy. Among
immediate post-operative pyrexia and infection in paediatric oncology B-IV nodules, all cases underwent resection (hemithyroidectomy 2,
patients. total thyroidectomy 4), with four (67%) revealed malignancy. All B-VI
Methods: We conducted a 5 year retrospective review of all patients nodules observed malignancy. Combined with our previous data, malig-
who had a laparotomy for tumour resection (January 2016- December nancy rate in B-III, IV, and VI were 4/18 (22%), 8/10 (80%), and 7/7
2021). The outcome measures were post-operative pyrexia, and cul- (100%), respectively.
ture proven infection in the immediate post-operative period (0-5 days Conclusions: We observed consistent cancer risk in each Bethesda
post procedure). Predictor variables included duration of anaesthesia, class on updated data. Regarding B-III nodules, our data demonstrated
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S59 of S635

lower malignancy risk than previously reported. Repeat biopsy rather Centro Hospitalar Universitário de São João, Paediatric Surgery Depart-
than immediate resection can potentially be an option for B-III nodules ment, Porto, Portugal
in selected cases given the relatively low malignancy rate.
Background and Aims: Thoracoscopic surgery (TS) is yet to be con-
sidered the standard surgical approach for mediastinal tumours. We
O124 / #850 CLINICAL CHARACTERISTICS AND SURGICAL present our results in mediastinal tumours resection with TS.
OUTCOMES OF PEDIATRIC PANCREATIC TUMOR -A SINGLE Methods: Patients submitted to TS from January 2012 to October
CENTER EXPERIENCE- 2021 were retrospectively reviewed. All patients with <18 years
and an established/suspected diagnosis of mediastinal tumour were
Naonori Kawakubo, Junkichi Takemoto, Ryota Souzaki, Satoshi Obata, included. Demographic’s, clinical data, tumour’s size and location,
Koichiro Yoshimaru, Toshiharu Matsuura, Tatsuro Tajiri intra and post-operative complications and pathological data were
Kyushu University, Pediatric Surgery, Fukuoka, Japan analysed. Categorical variables were expressed as absolute and rel-
ative frequencies, continuous variables as median and interquartile
Background and Aims: Pediatric pancreatic tumors are relatively range.
rare. There are few reports concerning the characteristics of pediatric Results: Seventeen patients underwent TS. Median age was 10.00
patients undergoing pancreatectomy for pancreatic tumors. This study (3.00-14.50) years, median hospital stay was 4.00 (2.00-7.00) days and
aimed to clarify the characteristics and outcomes of pediatric patients median follow-up-time of 19.00 (5.00–56.00) months. Median preop-
who underwent pancreatectomy. erative tumour size was 45.00 (31.50-70.00) mm; six (35%) were in
Methods: Pediatric patients who underwent pancreatectomy in our the anterior mediastinum. The majority (82%) presented symptoms
institution from 1995 to 2021 were included. The diagnosis, operative at diagnosis. There were 14 (82%) tumour resections and 3 (18%)
procedure, complications, mortality, and prognosis were retrospec- thymectomies; 9 (53%) were neurogenic tumours (4 ganglioneuromas,
tively analyzed. 2 neuroblastoma, 2 neurofibromas and 1 schwanoma), 4 (24%) germ
Results: During the above period, 14 patients underwent pancreatec- cell tumours (3 teratomas and 1 seminoma), one (5%) type B2 thy-
tomy for pancreatic tumor (median age, 10 years; range, 6-15 years). moma (2021 WHO Classification) and 3 (18%) benign specimens (1
The diagnosis was solid-pseudopapillary neoplasm (SPN) (12 cases, bronchogenic cyst and 2 miscellaneous). There were three (18%) con-
85.7%), and pancreatoblastoma (PB) (2 cases, 14.3%). The surgical versions to thoracotomy and there was no mortality related to the
procedures included pancreatoduodenectomy (4 cases, 28.5%), distal surgery. Two patients (12%) presented postoperative complications
pancreatectomy (3 cases, 21.5%), and partial pancreatectomy (7 cases, (one with ipsilateral diaphragmatic elevation and another with pleu-
50%). The most common postoperative complication was postopera- ral and pericardial effusion). Given the tumour’s location, two (12%)
tive pancreatic fistula (POPF) (4 cases, 28.5%). Remarkably, patients patients developed Horner’s syndrome and one (5%) ipsilateral pal-
who underwent partial pancreatectomy tended to have a higher post- mar and axillary anhidrosis. There were no recurrences during follow
operative complication rate in comparison to patients who underwent up.
pancreatoduodenectomy or distal pancreatectomy (71.4% vs. 14.2% Conclusions: The rarity of paediatric mediastinal tumours, the variabil-
p=0.02). Regarding long-term complications, there was 1 patient ity in tumour’s size and location, and proximity to important organs
with borderline diabetes who underwent pancreatoduodenectomy. All during resection, hampers implementation of TS as the standard treat-
SPN patients remained alive without recurrence during the follow-up ment. Our results show that, in centres with good experience in TS,
period. In contrast, one of two patients with pancreatoblastoma died this technique is safe and effective for the resection of paediatric
of recurrent disease. mediastinal tumours.
Conclusions: Pancreatectomy in pediatric patients was indicated for
low-grade malignancy, and the prognosis is good in the case of SPN,
regardless of the surgical procedure. If a surgeon selects partial pan- O126 / #1659 OVARIAN FUNCTION AND FERTILITY IN
createctomy for the treatment of pediatric pancreatic tumor, they PREPUBERTAL RATS SUBMITTED TO POST CHEMOTHERAPY
should consider the higher complication rate. Pancreatoduodenec- CRIOPRESERVED OVARIAN TISSUE REIMPLANTATION
tomy or distal pancreatectomy are feasible and effective treatments
that can achieve an acceptable quality of life in pediatric patients with Talita Amianti1 , Simone Abib1 , Mauricio Trotta2 , Jacqueline Camillo3 ,
pancreatic tumors. Alexandre Duarte1 , Fernanda Souza4 , Renato Fraietta3 , Edson Lo
Turco3 , Maria Teresa Alves5
1 Pediatric Onncology Institute - GRAACC - Federal University of São
O125 / #746 THORACOSCOPIC SURGERY – AN OPTION Paulo, Pediatric Surgical Oncology, São Paulo, Brazil; 2 Pediatric Onncology
FOR PAEDIATRIC MEDIASTINAL TUMOURS? Institute - GRAACC - Federal University of São Paulo, Centro De Desen-
volvimento De Modelos Experimentais Para Medicina E Biologia (cedeme),
Joana Mafalda Monteiro, Carolina Soares-Aquino, Mariana São Paulo, Brazil; 3 Federal University of São Paulo, Human Reproduc-
Borges-Dias, Norberto Estevinho, Sofia Vasconcelos-Castro tion, São Paulo, Brazil; 4 Pediatric Oncology Institute - GRAACC - Federal
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S60 of S635 ABSTRACTS

University of São Paulo, Pediatric Surgical Oncology, São Paulo, Brazil; Cancer Consortium, Partnersite Essen, Essen, Germany; 5 University of Syd-
5 IOP/GRAACC/UNIFESP, Pathology Department, Sao Paulo, Brazil ney, Chris O’ Brien Lifehouse, Camperdown, Australia Faculty Of Medicine
And Health, Camperdown, Australia; 6 Université Catholique de Louvain,
Background and Aims: Infertility comes along with the overall survival Cliniques Universitaires Saint Luc, Department Of Pediatric Haematol-
rate close to 80% in pediatric cancer. Ovarian tissue cryopreservation ogy And Oncology, Brussels, Belgium; 7 Charles University, Motol Children’s
in prepubescent children has been offered in some oncology services Hospital, Prague, Czech Republic; 8 University Hospital Muenster, West
as an experimental method. There are few studies about prepubescent German Cancer Center Network, Radiotherapy And Radiooncology, Mün-
ovaries and none that include chemotherapy in the experimental model ster, Germany; 9 Queen Silvia Children’s Hospital, Childhood Cancer Center,
to evaluate ovarian function and fertility in prepubertal rats submitted Gothenburg, Sweden; 10 Leiden University Medical Center, Dept Of Medical
to cryopreservation of ovarian tissue and chemotherapy. Oncology, Leiden, Netherlands; 11 University Hospital Essen, West German
Methods: Eighty Wistar rats aged 21 days. Groups 1, 3, 5 and 7 (control) Cancer Centre, Clinic Of Orthopedics, Essen, Germany; 12 Prinses Máxima
underwent oophorectomy first. On day 23, they received chemother- Centrum voor Kinderoncologie, Pediatric Oncology, Utrecht, Netherlands;
apy (cyclophosphamide 100mg/kg in group 1, ifosfamide 200mg/kg in 13 University Hospital Muenster, West German Cancer Center Network,
group 3, cyclophosphamide 50mg/kg + ifosfamide 100mg/kg in group Gerhard Domagk Institute For Pathology, Essen, Germany; 14 Borsod-Abaúj-
5 and saline solution 0.9% in group 7). Groups 2, 4, 6 and 8 (Sham) Zemplén County University Teaching Hospital, Velkey László Child’s, Health
received chemotherapy at day 21 (cyclophosphamide 50mg/kg in Center, Miskolc, Hungary; 15 University Children’s Hospital Münster, West
group 2, ifosfamide 100mg/kg in group 4, cyclophosphamide 50mg/kg German Cancer Center Network, Department Of Pediatric Hematology And
+ ifosfamide 100mg /kg in group 6 and saline 0, 9% in group 8). On the Oncology, Münster, Germany; 16 HUS Helsinki University Hospital, New Chil-
23rd day of life, oophorectomy was performed in groups 2, 4 and 6 and dren’s Hospital, Div. Hematology And Stem Cell Transplantation, Helsinki,
laparotomy without oophorectomy in group 8. Frozen ovary reimplan- Finland; 17 University Children’s Hospital Basel, Department Of Oncology/
tation at anatomical site was made on the 45th day of life. Analysis of Haematology, Basel, Switzerland; 18 Department of Oncology and Surgi-
estrous cycle was initiated onthe 60th day of life, when rats were placed cal Oncology for Children and Youth, Mother And Child Institute, Warsaw,
for copulation. Observation of estral cycles and pregnancy from 60- Poland; 19 Vilnius University, Faculty Of Medicine, Clinic Of Obstetrics And
120 days. Outcomes: presence or absence of estrous cycle, presence Gynecology, Vilnius, Lithuania; 20 University Hospital Essen, Department Of
or absence of pregnancy and presence of normal ovary tissue in the Particle Therapy, West German Proton Therapy Centre Essen (wpe), West
reimplanted ovary with or without chemotherapy. German Caner Center (wtz), German Cancer Consortium (dktk), Essen, Ger-
Results: Estral variations and viable ovary tissue were observed in all many; 21 St. Josefs Hospital Bochum, University Hospital, Bochum, Patient
rats in all groups.Pregnancy was observed in rats from groups 2 (n=1), Representative, Bochum, Germany
4 (n=2) and 8 (all animals).
Conclusions: Ovarian function was preserved despite chemother- Background and Aims: Local treatment is an essential element in the
apy timing before or after oophorectomy. Pregnancy was multimodal treatment of Ewing sarcoma (EWS). The choice of a local
observed in groups that received chemotherapy in the pubertal control modality for EWS is still controversial. Aim of our study was to
period. analyze the impact of local treatment modalities and clinical variables
on overall survival in patients treated according to the Ewing 2008
protocol.
FREE PAPER SESSION (FPS) Methods: The prospectively collected data of 833 patients with local-
ized EWS treated in the Ewing 2008 trial between 2009 and 2018
FPS 04: BONE TUMORS 30-09-2022 8:30 AM - 9:30 AM were analyzed. All patients received induction chemotherapy prior
to local treatment. We tested proportional hazards assumption using
O128 / #525 VALUE OF LOCAL TREATMENT MODALITIES IN Schoenfeld-residuals and Goodness-of-fit test, hazard ratios (HRs)
PATIENTS WITH LOCALIZED EWING SARCOMA. REPORT FROM with 95% Confidence Intervals (CI) were calculated using Cox regres-
THE EWING 2008 TRIAL sion. Log-rank test was performed.
Results: The 5-year survival probabilities were 0.84 (0.79, 0.89) for
Philip Heesen1,2 , Andreas Ranft3,4 , Vivek Bhadri5 , Benedicte surgery, 0.77 (0.69, 0.86) for radiotherapy and 0.84 (0.79, 0.88) for
Brichard6 , Stephane Collaud4 , Sona Cyprova7 , Hans Eich8 , Torben surgery & radiotherapy, p=0.12. The HR of radiotherapy vs surgery
Ek9 , Hans Gelderblom10 , Jendrik Hardes4,11 , Lianne Haveman12 , alone or combination of surgery and radiotherapy was 1.23 (0.66,
Wolfgang Hartmann13 , Peter Hauser14 , Heribert Jurgens15 , Jukka 2.30) for patients that had a small tumor volume (<200 mL) and 1.76
Kanerva16 , Thomas Kühne17 , Anna Raciborska18 , Jelena Rascon19 , (0.90, 3.45) for patients that had a large tumor volume (≥ 200 mL),
Arne Streitbürger4,11 , Beate Timmermann4,20 , Yasmine Uhlenbruch21 , after adjusting for known prognostic factors as age and tumor site.
Uta Dirksen3,4 Detailed analysis of local treatment modalities in different sites will be
1 University Duisburg-Essen, Pediatrics Iii, Essen, Germany; 2 University of presented.
Zurich, Faculty Of Medicine, Zürich, Switzerland; 3 West German Cancer Conclusions: OS was better in patients treated with surgery or surgery
Center, University Hospital Essen, Pediatrics Iii, Essen, Germany; 4 German & radiotherapy compared to radiotherapy alone, but this difference
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ABSTRACTS S61 of S635

was not statistically significant. OS might be increased in patients OS/EFS was 0.70 (SE=.09)/0.55 (SE=.10) for localized patients and
with large tumor volume when treated with surgery or a combination 0.33 (SE=.12)/0.27 (SE =.11) for metastatic patients (OS: P=.01). Sur-
of surgery & radiotherapy compared to radiotherapy only. Treatment vival in secondary EwS did not differ between hematologic or solid
plans should consider our findings, in line with patient- characteristics primary malignancies.
and -preferences. Conclusions: SMN after EWS remains a rare but severe event and
requires a structured follow-up system. EwS as SMN accounts for
approximately 1% of all reported EwS, and its risk-adjusted treatment
O129 / #1841 TWO RARE SIDES OF ONE RARE DISEASE: should be curative, especially in localized patients.
EWING SARCOMA WITH AND AS SECONDARY MALIGNANCIES
– EPIDEMIOLOGICAL AND CLINICAL ANALYSIS OF AN
INTERNATIONAL TRIAL REGISTRY O130 / #173 IMPACT OF ADDING MIFAMURTIDE TO A
NON-METHOTREXATE THREE-DRUG CHEMOTHERAPY
Stefan K. Zöllner1 , Katja Kauertz1 , Isabelle Kaiser1 , Heribert Jurgens2 , REGIMEN
Christiane Schaefer1 , Ruth Ladenstein3 , Michael Paulussen4 , Thomas
Kühne5 , Lianne Haveman6 , Wolfgang Hartmann7 , Thorsten Langer8 , Rejin Kebudi1 , Sema Bay Buyukkapu1 , Ülkü Miray Yıldırım1 , Ahmet
Andreas Ranft1 , Uta Dirksen1 Salduz2
1 University Hospital Essen, Pediatrics Iii, Essen, Germany; 2 University Chil- 1 Istanbul University, Oncology Institute, Pediatric Hematology-oncology,
dren’s Hospital Münster, West German Cancer Center Network, Department Istanbul, Turkey; 2 Istanbul University Medical Faculty, Orthopedics, Bey-
Of Pediatric Hematology And Oncology, Münster, Germany; 3 Children’s oğlu, Cihangir, Turkey
Cancer Research Institute, Paediatric Oncology, Vienna, Austria; 4 Vestische
Children’s and Youth Hospital Datteln, Pediatric Hematology And Oncol- Background and Aims: Chemotherapy and surgery are the main-
ogy, Datteln, Germany; 5 University Children’s Hospital Basel, Department stay of osteosarcoma treatment. We have used a nonmethotrexate
Of Oncology/ Haematology, Basel, Switzerland; 6 Prinses Máxima Centrum three-drug regimen since 1990. The addition of mifamurtide (MTP)
voor Kinderoncologie, Pediatric Oncology, Utrecht, Netherlands; 7 University was reported to improve the 6-year survival (78vs70%) in non-
Hospital Muenster, West German Cancer Center Network, Gerhard Domagk metastatic osteosarcoma (Meyers,2008). Since 2011, we added MTP
Institute For Pathology, Essen, Germany; 8 University Hospital for Children postoperatively to our regimen. This study aims to evaluate the out-
and Adolescents, Pediatric Oncology And Hematology, Lübeck, Germany come of nonmetastatic OS patients who recieved a nonmethotrexate
three-drug regimen with MTP vs without MTP (historical group) and
Background and Aims: Intensive, multimodality treatment of Ewing surgery.
sarcoma (EwS) improves survival at the expense of late effects such as Methods: Between 1990-2021, 99 children/adolescents with non-
secondary malignant neoplasms (SMN). Patients with secondary EWS metastatic osteosarcoma, treated at the Istanbul University, Oncology
are excluded from risk stratification in several studies and therefore do Institute, were retrospectively evaluated. Patients recieved six courses
not benefit from new therapies. More knowledge about EWS patients (3-pre/3-postoperatively) of a three-drug regimen comprising of ifos-
with SMN or as SMN is needed to identify at-risk patients and adapt famide 1.8 g/m2/d x 3 days, epirubicin 90 mg/m2/d and sisplatinum
follow-up strategies. 100mg/m2/d administered every 21 days. Since 2011 mifamurtide was
Methods: Epidemiology and clinical characteristics of EWS patients added postoperatively (48 doses in 36 weeks). The demographic char-
with SMN or as SMN were analyzed in 4518 and 3874 patients treated acteristics, treatment outcome of patients who recieved MTP were
in the last five and three consecutive international EWS trials, CESS81, compared with those who recieved the same chemotherapy without
CESS86, EICESS92, Euro-E.W.I.N.G.99, and EWING2008, respectively. MTP historically.
Results: Ninety-six patients developed SMN after primary EWS, with Results: 99 children (F/M=52/47) with a median age of 12(4-18) years
solid tumors detected more frequently than hematologic neoplasms were evaluated, fourty received MTP . Local relapse was observed in
(55.2% and 44.8%, respectively). The median latency between EWS and 22/59 (37.2 ) patients at a median of 14 (1-71) months in the his-
first SMN was 4.9 years (range, 0.1-28.1), with a significant difference toric group; in 8/40(20%) at a median of 16 (11-37) months in the
of 6.1 years between earlier development of hematologic malignancies MTP group. Distant metastasis was observed in 24/59(40.6 %) in the
compared with solid tumors (P<.001). The clinical characteristics of the historic group at a median of 15(7-44) months; in 13/40(32.5%) at a
primary EWS did not differ between patients with and without SMN. median of 23 (7-50) months in the MTP group. The 5 year overall sur-
All patients received multichemotherapy, with 80.2% receiving adju- vival was 62.2% for all patients; 56.8% for the historic vs 69 % for the
vant radiotherapy. Forty-four cases of secondary EWS were reported, MTP group (p=0.14). The 5 year event-free survival (EFS) was 54.5%
preceded by a heterogeneous group of malignancies, mainly acute lym- for all patients; 48.7 % for the historic vs 62.6 % for the MTP group
phoblastic leukemias (n=7) and lymphomas (n=7). Two cases (7.6%) (p=0.075).
occurred in the radiation field of the primary tumor. The median age Conclusions: Mifamurtide was well tolerated with the three-drug (cis-
at diagnosis of secondary EwS was 21.4 years (range, 5.9-72) com- platin/epirubicin/ifosfamide) regimen. There is a trend of increased EFS
pared with 10.9 years (range, 0.9-53.5) for primary EwS. The 3-year in the MTP group, which has to be confirmed in larger series.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S62 of S635 ABSTRACTS

CCI Jesse Lemmen1,2 , Nancy Midiwo3 , Festus Njuguna3 , Gertjan


Kaspers1,2 , Saskia Mostert1,2
CCI: SURVIVORS: NEEDS AND CHALLENGES 30-09-2022 8:30 1 Emma Children’s Hospital, Amsterdam UMC, Vrije Universiteit, Pediatric
AM - 10:10 AM Oncology, Amsterdam, Netherlands; 2 Princess Máxima Center for Pedi-
atric Oncology, Pediatric Oncology, Utrecht, Netherlands; 3 Moi University,
O131 / #437 HEROS BEYOND CHEMO - CHAMPIONS’ Department Of Child Health And Pediatrics, Eldoret, Kenya
CIRCLE
Background and Aims: Introduction: The number of children surviv-
Bilal Koush1 , Imad El Hajje2 ing cancer is increasing in low and middle-income countries. At Moi
1 Children’s Cancer Center of Lebanon (CCCL), Marketing And Communi- Teaching and Referral Hospital (MTRH), a tertiary care referral hospital
cations Executive, Beirut, Lebanon; 2 Childrens Cancer Center CCCL, Prea, in Western Kenya, childhood cancer survival rates have continuously
Beirut, Lebanon improved over a period of 10 years. This comes with a new healthcare
challenge: late effects and quality-of-life of survivors. Stigma, myths
Background and Aims: As the number of our childhood cancer and misconceptions about cancer may play a major role in hindering
survivors is fortunately increasing,while the progress of diagno- social reintegration of these children.
sis and treatment is progressing rapidly, The CCCL’s Champions’ Methods: A single-case study was conducted to highlight challenges
Circle saw the light during a special event held on April, 2018 facing Kenyan childhood cancer survivors. Late effects, stigmatiza-
grouping inspirational cancer-fighters from all over Lebanon and tion and social reintegration were explored. Investigators identified a
the region.The aims are to build a strong relationship amongst sur- Hodgkin lymphoma survivor and studied his medical records. The sur-
vivors, enhance and help those survivors’ lives through continu- vivor was interviewed at MTRH in December 2021 and March 2022.
ous fellowship care, socialization, education and activism support, Two independent interviewers used a semi-structured questionnaire.
grow the club to reach all childhood cancer survivors in the region The survivor rendered informed consent.
and to safeguard their wellbeing.We are currently over 250 cancer Results: This case report depicts a young Kenyan man who, after sur-
survivors. viving Hodgkin lymphoma, still faces the long-term consequences of
Methods: The Champions’ Circle program is to equip our heroes with his previous condition. Not only chemotherapy-induced late effects
educational and learning skills sessions, moral support to cope with (chronic heart disease), but also serious stigmatization by his commu-
post-treatment effects,to engage them with outreach community, and nity continue to affect his life on a daily basis. Cancer is regarded as an
provide them with group support sessions and fun activities.Most incurable, contagious curse and his family avoided. Despite the com-
recently, to ensure a healthy future lifestyle with less anxiety and fear, munity’s stigmatization, the survivor has a strong wish to help other
we provided our survivors and their parents with the COVID-19 vac- cancer patients going through similar challenges. He wants to teach
cine to protect them from the global pandemic spread.Also, in line with them that the disease is not a death sentence and encourage them
WHO strategy, we offered survivors HPV vaccine targeting the age to speak out. He would like to help raise awareness about childhood
between 14 to 24 years old.CCCL organized two awareness campaigns cancer.
targeting eligible caregivers and survivors based on multiple published Conclusions: This study highlights that advocacy for a holistic approach
research and physician inputs.Sessions included oncologist interven- in managing the well-being of childhood cancer survivors is required: a)
tions to educate families and survivors about HPV effect on health, to map late effects, adapted follow-up guidelines must be integrated in
risk of second cancers, importance of receiving the HPV vaccination standard care; b) to prevent stigmatization and facilitate social reinte-
following cancer treatment gration, myths and misconceptions about cancer need to be eradicated.
Results: Acknowledging the importance of protecting our survivors This combined approach could empower survivors and improve their
from covid and HPV, we launched both vaccination programs.As a quality-of-life.
result, 3 comprehensive virtual presentations and Q/A sessions by
expert doctors were done, digital registration platforms were created
to obtain registered data, all eligibles had their appointment schedule, O133 / #1200 OVERCOMING THE IDENTITY CRISIS IN
with a result of 130 vaccinated survivors. CHILD CANCER SURVIVORS
Conclusions: Because CCCL cares about the health of our heroes, we
commit to using all available resources, including vaccinations, to help Nicki Virgo1 , Rama Wijaya2
keep young cancer survivors safe. 1 B. Braun Medical Indonesia, Corporate Communication, Jakarta, Indone-
sia; 2 Yayasan Onkologi Anak Indonesia, Non Goverment Organization,
Jakarta, Indonesia
O132 / #1063 SURVIVING CHILDHOOD CANCER IN KENYA:
LATE EFFECTS, STIGMATIZATION AND SOCIAL Background and Aims: Survivors of childhood cancer and coinciding
REINTEGRATION with entering adolescence is a challenge that is not easy. They often
experience identity crises. The lives of teenagers are supposed to have
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ABSTRACTS S63 of S635

identity and friends are hindered by some stigma in society, parents identity. During an important part of their development, they had to
also over-protective, which make activities are limited at home. This deal with the language of disease and the unknown. The uncertainty
happens to Nicki and Rama who are cancer leukemia survivors who and exposure to illness may negatively influence the shaping of their
have been cured for 10 years. Both of us have the same undergoing identity.
long treatment at the hospital and home, but when going back to school Conclusions: Therefore, reframing might be a powerful way to draw
Nicki and Rama have different experiences and worries. Rama with attention to certain parts of the world that were neglected or encoded
physical changes after treatment have no confidence because he has by the context of the disease. Reframing is also useful to increase the
moonface, and bald head. His friend used to make fun of him. Com- vocabulary and to highlight certain aspects of the survivor’s conscious-
pared to Rama, Nicki as a son of a teacher is have a privilege treatment, ness that might have been shaded by the stressors of the illness, its
which made his friend envy. Both of this condition Nicki and Rama feel associated therapies, fragile peer networks, or social supports.
isolated, and unable to live a normal life.
Methods: Joining the community of child cancer survivors at YOAI
(Cancer Buster Community), help see other perspectives and learn NURSING
from the experiences of fellow survivors. Doing hobbies that are liked
such as reading, learning a foreign language, exercising, participating NURSING ORAL ABSTRACT PRESENTATIONS: CREATIVE WAYS
actively in organizations, participating in events or events outside of TO PROMOTE EXCELLENCE IN NURSING 30-09-2022 8:30 AM -
school. 9:30 AM
Results: There are positive changes in our lives, we have our self-
confidence and also a sense of responsibility for the task given. We O135 / #1067 SUCCESS STORIES AND RECOMMENDED
manage to communicate with others easily and also be aware of your IMPROVEMENTS: A QUALITATIVE EVALUATION OF THE
own potential. PAEDIATRIC ONCOLOGY NURSING SPECIALIST EDUCATION
Conclusions: Identity crisis in child cancer survivors varies from one PROGRAM IN GHANA
another depending on factor affected. Therefore, as survivors, we must
look for a positive and consistent hobby. Be yourself in the positive Mavis Obuoh-Evans1 , Pernilla Pergert2 , Abukari Salifu3 , Rachel
ways. Because so many ways to Barcelona. Hollis4 , Wendy Eyiah-Mensah5
1 Ghana College of Nurses and Midwives, Paediatric Oncology Nursing,
Accra, Ghana; 2 Karolinska Institutet, Women’s And Children’s Health,
O134 / #1712 WHAT WORDS DO TO US OR FOR US Stockholm, Sweden; 3 University of Development Studies, Public Health,
Tamale, Ghana; 4 Leeds Teaching Hospitals NHS Trust, Nursing, Leeds,
Ana Costa Campos United Kingdom; 5 Korle Bu Teaching Hospital, Child Health, Accra, Ghana
Acreditar, Volunteer, Alcabideche, Portugal
Background and Aims: Ghana is one of the focus countries of the
Background and Aims: By changing the words we use to define our- WHO Global Initiative for Childhood Cancer. At a National Stakeholder
selves, our identity takes on a new level of meaning. Words have power, Workshop in 2019, nurse education was identified as a priority. The
and reframing may be a way to improve the communication process, Ghana College of Nurses and Midwives (GCNM) pledged to develop
gaining new perspectives, self-esteem and confidence. Language might a programme of specialist education in paediatric oncology nursing.
act as a lens or troublemaker and promote the salience or selection A curriculum was developed in collaboration with local stakeholders
of certain experiences over others. Therefore language is an efficient, and international partners. The one-year programme commenced at
powerful and flexible resource that can influence cognitive processing the GCNM in October 2020. In 2021, an evaluation was conducted by
during mental computations (Ünal & Papafragou, 2016). What is the external evaluators to ascertain the impact of the programme, how it
relation between language and self-esteem? Language guides speakers’ was perceived by various stakeholders, and to make recommendations
perspectives about the world and contributes to supporting their judg- for the future.
ing attitude. The more you focus on some words and thoughts, the more Methods: A descriptive qualitative design was used. An interview guide
you can damage key structures that regulate your memory, feelings, was developed and purposive sampling used to identify participants
and emotions. By deliberately replacing it with words or statements with the requisite experience of the programme; faculty, expatriate and
that encourage confidence and self-esteem, the inner speech changes, graduate nurses, physicians and parents. This was followed by snow-
and so does the behaviour. ball sampling as initial participants recommended others. Qualitative
Methods: During the sessions, the adolescents described themselves content analysis was conducted of transcribed interviews (n 25). Par-
using up to 6 words and simulating a job interview, they had to reframe ticipant and non-participant observation was carried out in the clinical
their negative and positive traits into adequate traits. setting.
Results: My experience with adolescents and young adults that have Results: A positive impact was described in key areas: Nurses’ knowl-
survived childhood cancer, showed me that reframing the way they edge and skills— increased confidence and improved patient care. Mul-
see themselves may be a powerful tool to raise their self-esteem and tidisciplinary collaboration—inputs of graduates were more actively
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S64 of S635 ABSTRACTS

sought and valued. Furthermore, the roles of some graduates changed two) and will involve pediatric oncology nurses from across the
as their institution recognised the specialist education; others saw no globe.
benefit. Local ownership of the programme was described but also a
reliance on international nurses in the development and delivery of the
programme. Recommended improvements included strengthening the O137 / #106 POSTGRADUATE INTERPROFESSIONAL
clinical teaching in practicum and the local faculty. CASE-BASED LEARNING IN CHILDHOOD CANCER
Conclusions: As this evaluation has demonstrated, the perceived
impact of the education was positive but some immediate changes Martha Topperzer1 , Marianne Hoffmann1 , Hanne Larsen2 , Susanne
could enhance learning. The local faculty need to be strengthened to Rosthøj3 , Martin Fridh2 , Louise Roug2 , Liv Andrés-Jensen4 , Peter
improve the sustainability and promote further local ownership. Fur- Pontoppidan1 , Kjeld Schmiegelow1 , Jette Sørensen5
ther systematic efforts are also required to embed this programme into 1 University of Copenhagen, Rigshospitalet, Department Of Child And
local education and career frameworks. Adolescent Cancer, Copenhagen, Denmark; 2 University of Copenhagen,
Rigshospitalet, Paediatric Oncology Research Laboratory, Department Of
Paediatrics And Adolescent Medicine, Copenhagen, Denmark; 3 University
O136 / #1725 DEVELOPMENT OF TIERED of Copenhagen, Section Of Biostatistics, Faculty Of Health Sciences,
PEDIATRIC-ONCOLOGY NURSING COMPETENCIES: PHASE I Copenhagen, Denmark; 4 University of Copenhagen, Rigshospitalet, Depart-
ment Of Paediatrics And Adolescent Medicine„ Copenhagen, Denmark;
Monnie Abraham1 , Sara Day2 5 University of Copenhagen, Rigshospitalet, Juliane Marie Centre, Copen-
1 St. Jude Children’s Research Hospital, Department Of Global Pediatric hagen, Denmark
Medicine, Memphis, TN, United States of America; 2 University of Ten-
nessee Health Science Center, College Of Nursing, Memphis, United States Background and Aims: Optimal collaboration is vital to continuing to
of America improve children and adolescents with cancer’s treatment and care
and ensure greater normality in daily life for survivors. To meet this
Background and Aims: Defining the basic competency requirements end, evidence-based interprofessional education is needed. The aim of
with the necessary knowledge, attitudes, and skills are critical steps this feasibility study was to assess acceptability, demand, implemen-
in preparing a competent pediatric hematology-oncology nurse. The tation, and practicality of postgraduate interprofessional case-based
overall aim of this study is to design a tiered pediatric hematology- learning in childhood cancer at Copenhagen University Hospital –
oncology competency framework based on the Benner framework of Rigshospitalet.
novice to expert and adaptable to the global environment. This frame- Methods: We identified 13 groups of healthcare professionals work-
work will guide nurses practicing at different proficiency levels in ing with children and adolescents with cancer: nurses, doctors, social
various settings and will guide the development of nursing orientation workers, physiotherapists, occupational therapists, pharmacists, phar-
and continuing educational programs. Phase I included a systematic lit- macologists, dieticians, nursing assistants, and professionals with a
erature review to determine the status of pediatric oncology nursing supportive function: teachers, secretaries, priests, and daycare work-
tiered competencies. ers. All participated in one postgraduate interprofessional case-based
Methods: A three-member core group of senior nurses was formed to learning session. Feasibility was assessed using Bowen’s focus areas
complete the search. The literature search utilized three databases: of acceptability, demand, implementation, and practicality. Before and
PubMed, CINAHL, and SCOPUS. Search terms included “pediatric after the intervention session, three measurement tools were used 2–3
oncology competencies” or “pediatric oncology tiered competencies” weeks before participation and 3–4 weeks after participation to col-
and there were no restrictions on publication dates or language. A med- lect data: Assessment of Interprofessional Team Collaboration Scale,
ical librarian independently approved the search strategies. Selection Readiness for Interprofessional Learning Scale, and Safety Attitudes
criteria included: published in a peer reviewed journal, competencies Questionnaire.
specific to pediatric oncology and a tiered approach described. Results: Representing 13 occupational groups, 49 participants com-
Results: Using the three databases and search terms identified, a pleted the case-based learning sessions, indicating acceptability and
total of 76 (PubMed 15; CINAHL 1; Scopus 60) unique articles were practicality. The pre-and post-questionnaires were completed by 79%
identified. Upon careful review none of the articles met the crite- of the participants, 88% of whom rated the professional content as
ria for pediatric oncology competencies and a tiered approach or good or very good. A change over time was detected on all three scales
competencies developed with a global perspective. measuring mean difference postintervention scores. The outcome
Conclusions: The literature search confirmed the need to develop measures can be used to assess the effect of the intervention.
and validate pediatric oncology nursing tiered competencies. Pedi- Conclusions: This study provides knowledge on the development
atric oncology tiered competencies will strengthen the nursing work- of interprofessional content, group size for interprofessional learn-
force and provide competencies that truly reflect the practice of ing, logistics for implementation, and the hierarchical perils to be
pediatric oncology nursing from beginner to expert level. A Del- aware of in interprofessional education. Interprofessional case-based
phi approach will be used for competency development (phase learning is feasible, acceptable, and can be implemented in a clinical
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S65 of S635

context. Management support is imperative for the success of edu- Nassanga2 , Hellen Mbutuka1 , Memory Sabantini1 , Emma Segula1 ,
cational interventions. Optimally, an organisations’ policies, strategies Jennifer Higgins4 , Marilyn Hockenberry4
and practices should integrate interprofessional education. 1 Global HOPE Malawi, Nursing, Lilongwe, Malawi; 2 Global HOPE Uganda,
Nursing, Kampala, Uganda; 3 Global HOPE Botswana, Nursing, BW,
Botswana; 4 Baylor College of Medicine, Global Hope, Houston, United
O138 / #1312 IMPROVING PSYCHOSOCIAL SUPPORT States of America
AMONG NURSES IN PEDIATRIC ONCOLOGY CARE IN
CAMEROON Background and Aims: Nursing career ladders developed in the
1980s enhanced recruitment and retention of experienced staff. These
Vera Samba1 , Megan Cruise2 early programs served as an effective recognition system for clini-
1 Cameroon Baptist Convention Health Services, Mboppi Baptist Hospital cal performance and professional development. While industrialized
Douala, Douala, Cameroon; 2 World Child Cancer, Programmes, London, nations formally recognize clinical nursing professional growth, few
United Kingdom low-middle-income countries have career ladder programs. The spe-
cific aim of this project was to develop a career ladder to recognize
Background and Aims: BACKGROUND The paediatric oncology cen- pediatric hematology/oncology nurses for their professional growth
tres in Cameroon united to form the Cameroon Paediatric Oncology and development in Sub-Saharan Africa.
Group and identified psychosocial support as imperative in their work. Methods: A review of the literature on career ladder programs was
Research conducted by World Child Cancer (WCC) with paediatric completed and a working group of nurse leaders, educators and
oncology nurses in Africa showed that many have increased exposure clinical staff was created. Using the evidence, a five-level career
to highly emotive experiences which can lead to burnout, compassion ladder was developed that includes: Pediatric Oncology Nurse 1,
fatigue and secondary traumatic stress.The WCC psychosocial support Pediatric Oncology Nurse Clinician 2, Pediatric Hematology/Oncology
advisor developed a training package for nurses and health care pro- Advanced Nurse Clinician 3, Pediatric Hematology/Oncology Nursing
fessionals to address these issues. The aim of this project was to train Specialist 4, and Pediatric Hematology/Oncology Nursing Leader 5.
and equip nurses with skills to help them achieve a better emotional The working group established criteria for each level assessing clini-
balance in their day-to-day work. cal excellence, formal education completion, leadership activities, and
Methods: In December 2019 thirteen representatives were trained as quality improvement and/or research project involvement. Career lad-
trainers by the advisor using four modules: the emotional impact of der criteria were reviewed by two African pediatric oncology nursing
childhood cancer; supporting families when a child has cancer; sup- experts external to the sites involved.
porting ourselves and each other; building resilience. Funding from the Results: Criteria for advancement were established for each level;
Sanofi Espoir MCM Award helped the trainers to roll out this training examples include years of pediatric hematology/oncology experience,
in their own institutions. At the end of each training session, a psy- academic degree, annual performance evaluation, specialized formal
chosocial support action plan was developed. Designated nurses are course completion, participation and leadership in online seminars,
responsible for the implementation of different actions. case study presentations, quality improvement projects, presentations
Results: A pool of nurses and other professionals capable of train- at conferences and authorship on abstracts and professional papers.
ing others has been established. They have trained160 health care Standard operating procedures specifically outline the process for clin-
staff across 5 units; 117 were nurses. An action plan for psychoso- ical staff to move up the ladder. A formal recognition ceremony is held
cial support for patients and staff has been developed in each unit to each year to recognize career ladder advancement.
be modified and adapted annually. Actions include establishment of a Conclusions: Positive outcomes from clinical ladder programs have
patient and parents support group and introduction of a psychosocial been observed for years in high-income countries. This career lad-
assessment tool. der program designed specifically for nursing caring for children with
Conclusions: This project has strengthened the whole multidisciplinary hematology and oncology disorders in Sub-Saharan Africa provides
team in the delivery of psychosocial support to patients, families and to opportunities for nurses to be recognized for the excellent work.
each other. The team has responded to requests for training outside
paediatric oncology, and with support staff. WCC has further devel-
oped the training package which could be embraced by all paediatric FREE PAPER SESSION (FPS)
oncology centres in sub-Saharan Africa.
FPS 05: ABDOMINAL TUMORS 30-09-2022 8:30 AM - 9:30 AM

O139 / #88 DESIGNING A CLINICAL LADDER FOR O140 / #327 ADHERENCE TO PROTOCOL
PEDIATRIC ONCOLOGY NURSES IN SUB-SAHARAN AFRICA RECOMMENDATIONS FOR CHILDREN WITH WILMS TUMOUR
IN TWO CONSECUTIVE STUDIES IN THE UK AND IRELAND –
Rhahim Bank1 , Tadala Mulemba1 , Grace Chirwa1 , Virginia Chopi1 , DOES VARIATION MATTER?
Joshi Evelyne2 , Andries Gontshwanetse3 , Queen Jaffu1 , Immaculate
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S66 of S635 ABSTRACTS

Suzanne Tugnait1 , Reem Al-Saadi2 , Richard Williams3 , Minou Conclusions: Variation in adherence to protocol recommendations is
Oostveen4 , Kristina Dzhuma5 , Jesper Brok6 , Sabine Irtan7 , Angela more common in Stage IV disease. Whilst variation does not affect 5-
Lopez Cortes1 , Charles Stiller8 , Mark Weeks9 , Jessica Bate10 , Mark year EFS or OS, it comprises both over- and under-treatment, which
Powis11 , Daniel Saunders12 , Oystein Olsen13 , Sucheta Vaidya14 , Lisa may affect risks of late effects.
Howell15 , Catriona Duncan4 , Gordan Vujanic16 , Tanzina Chowdhury4 ,
Kathy Pritchard-Jones1
1 UCL GOS Institute of Child Health, Developmental Biology And Cancer O141 / #1595 POST-CHEMOTHERAPY
Research And Teaching Department, London, United Kingdom; 2 Great BLASTEMAL-PREDOMINANT WILMS TUMOR IN THE
Ormond Street Hospital, Histopathology, London, United Kingdom; CHILDREN’S ONCOLOGY GROUP (COG) CONTEXT: POOLED
3 Imperial College, Genetics And Genomics, London, United Kingdom; OUTCOMES DATA FROM STUDIES AREN0532, AREN0533 AND
4 Great Ormond Street Hospital, Paediatric Oncology, London, United King- AREN03B2
dom; 5 Great Ormond Street Hospital, Paediatric Surgery, London, United
Kingdom; 6 Rigshospitalet, Copenhagen University Hospital, Copenhagen, Nicholas Evageliou1 , Lindsay Renfro2 , Kelly Vallance3 , Carly Varela4 ,
Denmark; 7 Trousseau Hospital, Paediatric Surgery, Paris, France; 8 NHS Daniel Benedetti5 , Elizabeth Perlman6 , Nicholas Cost7 , Peter Ehrlich8 ,
Digital, National Cancer Registration And Analysis Service, Oxford, United John Kalapurakal9 , Geetika Khanna10 , Jennifer Aldrink11 , Richard
Kingdom; 9 Imperial College, Lung And Heart Institute, London, United King- Glick12 , Michael Ortiz13 , Lauren Parsons14 , Arnold Paulino15 , Maddy
dom; 10 University Hospital Southampton NHS Foundation Trust, Paediatric Artunduaga16 , Ethan Smith17 , David Dix18 , James Geller19 , Conrad
Oncology, Southampton, United Kingdom; 11 Leeds Teaching Hospital Fernandez20 , Jeffrey Dome21 , Elizabeth Mullen22
NHS Foundation Trust, Paediatric Surgery, Leeds, United Kingdom; 12 The 1 Children’s Hospital of Philadelphia, Oncology, Voorhees, United States of
Christie NHS Foundation Trust, Radiation Oncology, Manchester, United America; 2 Children’s Oncology Group, Statistics And Data Center, Monrovia,
Kingdom; 13 Great Ormond Street Hospital, Radiology, London, United United States of America; 3 Cook Childrens Medical Center, Hematol-
Kingdom; 14 Royal Marsden Hospital, Children And Young Peoples Unit, ogy/oncology, Fort Worth, United States of America; 4 Pediatric Specialists
Surrey, United Kingdom; 15 Alder Hey Childrens Hospital NHS Foundation of Virginia, Oncology, Fairfax, United States of America; 5 Vanderbilt Uni-
Trust, Paediatric Oncology, Liverpool, United Kingdom; 16 Sidra Medicine, versity/Ingram Cancer Center, Pediatric Oncology, Nashville, United States
Pathology, Doha, Qatar of America; 6 Lurie Children’s Hospital, Pathology, Chicago, United States
of America; 7 University of Colorado School of Medicine, Children’s Hos-
Background and Aims: Wilms tumour (WT) has excellent event-free pital Colorado, Department Of Surgery, Division Of Urology, And The
and overall survival rates. However, those with advanced disease have Surgical Oncology Program, Aurora, United States of America; 8 University
more variation in adherence to the treatment protocol. We examined of Michigan, C.S. Mott Children’s Hospital, Pediatric Surgery, Ann Arbor,
the extent of variation and its possible effects on survival United States of America; 9 Northwestern University, Department Of Radi-
Methods: Retrospective analysis of all children with unilateral WT ation Oncology, Evanston, United States of America; 10 Children’s Health-
treated with pre-operative chemotherapy in the SIOPWT2001 (2002- care of Atlanta, Pediatric Radiology, Atlanta, United States of America;
11) and IMPORT (2012-18) studies in the UK and Ireland. Pre- and 11 Nationwide Children’s Hospital, Pediatric Surgery, Columbus, United
post-operative treatments (including radiotherapy) were classified as: States of America; 12 The Steven and Alexandra Cohen Children’s Medi-
per protocol (PP); deviation (PDEV) - variation for specified clini- cal Center of New York, Pediatric Surgery, New Hyde Park, United States
cal reasons; violation (PVIOL) - not treated within protocol-defined of America; 13 Memorial Sloan Kettering Cancer Center, Pediatrics, New
parameters. Survival analysis was conducted by Kaplan-Meier, to York, United States of America; 14 Children’s Hospital of Wisconsin, Pathol-
calculate 2 and 5 year OS and EFS. ogy, Milwaukee, United States of America; 15 MD Anderson Cancer Center,
Results: 1130 children with WT were registered by 20 centres. 1044 Radiation Oncology, Houston, United States of America; 16 UT South-
(92%) had unilateral WT treated with pre-operative chemotherapy. All western/Simmons CAncer Center-Dallas, Radiology, Dallas, United States
had centrally reviewed pathology. Case Report Forms allowing cat- of America; 17 Cincinnati Children’s Hospital Medical Center, Radiology,
egorisation of the whole treatment pathway were available for 752 Cincinnati, United States of America; 18 British Columbia Children’s Hos-
patients. Survival rates for both studies were identical, with 5-year pital, Hematology/oncology, Vancouver, Canada; 19 Cincinnati Children’s
OS 92% and 5-year EFS 86%. For patients with localised disease Hospital Medical Center, Division Of Pediatric Oncology, Cincinnati, United
(Stage I-III) 5-year EFS was 88% for PP (n=402) and PDEV (n=123) States of America; 20 IWK Health Centre and Dalhousie University, Depart-
and 84% for PVIOL (n=72), corresponding 5-year OS was 96%, 95% ment Of Pediatric Hematology/oncology, Halifax, Canada; 21 Children’s

and 93%, respectively. For patients with metastatic disease (Stage National Hospital, Division Of Oncology, Washington, DC, United States
IV), 5-year EFS was 80% for PP (n=45) and PDEV (n=50) and 83% of America; 22 Children’s Hospital Boston/Dana-Farber Cancer Institute,
for PVIOL (n=54), corresponding 5-year OS was 89%, 78% and 90%, Department Of Pediatric Oncology, Boston, United States of America
respectively. None of these differences were statistically significant.
In SIOPWT2001 33% of PVIOL patients could be categorised as over- Background and Aims: Blastemal-type histology is a known adverse
treated and 56% as under-treated. In IMPORT, 47% were over-treated prognostic finding for Favorable Histology Wilms tumor (FHWT) in
and 45% were under-treated. the SIOP post-chemotherapy histology classification system. While the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S67 of S635

majority of patients with unilateral tumors enrolled on COG renal deep phenotyping in an unselected national cohort of patients with
tumor protocols undergo upfront nephrectomy, some have a delayed WT.
nephrectomy after preoperative chemotherapy. No alteration of ther- Methods: Prospective whole-genome sequencing (WGS) of germline
apy based on post-chemotherapy histology was recommended on the DNA in all children newly diagnosed with WT in Denmark . Deep
AREN series of trials. We examined the pooled outcomes of patients phenotyping through parent counseling and electronic medical record
stratified by COG by post-chemotherapy histology from these trials. mining.
Methods: Histologic classification was assessed for patients with uni- Results: Gene panel-based analyses found three (13%) of 24 patients
lateral FHWT enrolled on COG studies AREN0532, AREN0533 and with WT harbored pathogenic variants in genes associated with high
AREN03B2 who underwent nephrectomy after chemotherapy. The risk of WT (FBXW7, WT1 and REST). Epigenetic testing revealed
classification was determined by COG post-chemotherapy histology one (4%) patient with uniparental disomy of chromosome 11 and
guidelines, through central pathology review when available (84%) Beckwith-Wiedemann Syndrome (BWS). Another three patients (13%)
or by institutional pathology reviews (16%). Patients with anaplastic with bilateral disease and/or features of BWS had higher birth weights
histology were excluded. (4,780g vs. 3,575g; p=0.002) despite all being female, and the number
Results: Of 376 patients who underwent delayed nephrec- of females with macrosomia (>4,250g, n=5) was higher than expected
tomy, histologic classification was blastemal-predominant in (OR 9.98 [CI95% 2.56-34.66]; p<0.001). Finally, constrained gene anal-
22 (6%), intermediate-risk in 308 (82%), and low-risk in 46 ysis revealed an additional four patients (17%) with loss-of-function
(12%). Among the blastemal-predominant cohort, 7 (32%) had variants in genes evolutionarily intolerant to damage. Both known and
stage III disease and 15 (68%) had stage IV disease; 14 (64%) novel findings were more common in females (29% vs. 0% and 50%
were treated with DD4A (vincristine/dactinomycin/doxorubicin) vs. 10%, respectively; p=0.01). Only one patient had a family history
and 8 (36%) were treated with Regimen M (vin- indicative of an underlying genetic condition.
cristine/dactinomycin/doxorubicin/cyclosphamide/etoposide). Conclusions: In this prospective, nation-wide study, 17% of patients
Four-year event-free survival (EFS) estimates for the low-risk, with WT had a causative genetic or epigenetic condition and an
intermediate-risk, and blastemal-predominant groups were 89% additional 33% had either phenotypic traits or loss-function vari-
(95% CI: 81-99%), 84% (95% CI: 80-89%), and 62% (95% CI: 45- ants possibly linked to WT risk. Interestingly, these percentages were
87%; p=0.001). Four-year overall survival estimates for the low-risk, significantly higher in females.
intermediate-risk, and blastemal-predominant groups were 93% (95%
CI: 87-100%), 93% (95% CI: 90-96%), and 63% (95% CI: 45-90%;
p<0.0001). The 4 year EFS of the blastemal-predominant patients O143 / #731 IDENTIFICATION OF NOVEL THERAPEUTICS
treated with DD4A was 70% (95% CI: 49-100%), compared with 50% FOR HEPATOBLASTOMA USING A PATIENT-DERIVED
(95% CI: 25-100%) for those treated with Regimen M. XENOGRAFT IN VITRO DRUG TESTING PLATFORM
Conclusions: We confirm the adverse prognostic signifance of post-
chemotherapy blastemal-predominant histology in the COG context. Salih Demir1 , Mario Failli2 , Qian Li1 , Thomas Kessler3 , Carolina
The poor outcomes with both Regimens DD4A and M support study Armengol4 , Diego Di Bernardo2 , Stefano Cairo5 , Roland Kappler1
of further therapy intensification for these patients in future protocols. 1 LMU Munich, Pediatric Surgery, Munich, Germany; 2 University of Naples
Federico II, Chemical, Materials And Industrial Engineering, Naples, Italy;
3 Alacris Theranostics, Research, Berlin, Germany; 4 Germans Trias I Pujol
O142 / #1892 COMPREHENSIVE GERMLINE GENOMICS OF Research Institute, Childhood Liver Oncology Group, Badalona, Spain;
PATIENTS WITH WILMS TUMOR REVEALS A HIGH LEVEL OF 5 Xentech, R&d, Evry, France

PREDISPOSITION IN FEMALES
Background and Aims: Treatment of hepatoblastoma, the most com-
Ulrik Stoltze1 , Mathis Hildonen1 , Jesper Brok2 , Thomas Hansen1 , mon pediatric liver tumor, significantly improved in the last decades
Malene Lundsgaard3 , Karen Grønskov1 , Zeynep Tümer1 , Kjeld thanks to refinements of surgical procedures and clinical risk stratifi-
Schmiegelow1 , Karin Wadt1 cation. However, resistance towards conventional chemotherapy and
1 Rigshospitalet, Copenhagen University Hospital, KBH Ø, Denmark; the severe long-term side effects induced by its cytotoxicity remain
2 Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark; a prevalent challenge. The application of computational prediction
3 AALBORG UNIVERSITETSHOSPITAL, Klinisk Genetisk Afdeling, Aalborg, tools on transcriptomic data presents a promising strategy to identify
Denmark novel drugs, but their validation in reliable tumor models is inevitably
needed.
Background and Aims: Studies suggest that 5-10% of Wilms tumors Methods: We have established an in vitro drug-testing platform
(WT) are caused by genetic conditions and that an additional 2- comprised of conventional cell lines and cultures from dissociated
29% of WT are caused by epigenetic conditions, yet investiga- patient-derived xenografts of pediatric liver cancers. We have used an
tions incorporating all existing evidence are lacking. Here we com- 11-point dose-response curve to screen standard-of-care drugs used
bine comprehensive genomics with selective epigenetic testing and in the current PHITT trial as well as compounds targeting a wide range
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S68 of S635 ABSTRACTS

of different cellular pathways, which were predicted by digital tools on a library with over 200 compounds. Results are presented to treating
transcriptomic data of primary tumors. Compounds that induced >50% physicians by a Molecular Tumor Board.
killing after treatment for 48 hours were defined in viability assays. Results: To date, over 400 samples were included: extracranial solid
Results: We identified the ALK inhibitor ceritinib, the CDK9 inhibitors tumors (70.9%); central nervous system tumors (15.2%) and hema-
dinaciclib and alvocidib, and the antihelmintic mebendazole to reduce tological malignancies (13.9%). In 81%, any actionable event was
tumor cell growth in a dose-dependent manner. Subsequent in vitro detected. In 137 patients who completed a minimum of 12 months clin-
assays showed that the four drugs induced apoptosis, reduced colony ical follow up, 21 molecularly matched treatments were applied in 19
formation capability, retarded proliferation, decreased migration, and patients (13.9%). Complete and durable remission was achieved in one
paused 3-dimensional spheroid growth. Furthermore, the combina- patient only. In addition, PDTO were established, and HTS performed
tion of these drugs with cisplatin revealed a strong synergistic effect, for 37 solid tumor samples, including rhabdomyosarcoma (n=11), neu-
comparable to the one of cisplatin and doxorubicin, which is given to roblastoma (n=8), Wilms tumor (n=6), Ewing sarcoma (n=4), rhabdoid
high-risk hepatoblastoma patients. Most importantly, these drugs sig- tumors (n=3) and other (n=5). HTS confirmed existing molecular
nificantly reduced growth of a patient-derived xenograft model when targets such as ALK and c-kit and in addition revealed increased com-
transplanted into nude mice. pound sensitivity to drugs not previously found through sequencing
Conclusions: We were able to establish a platform for rapid, repro- (e.g., PARP inhibitors). Clinical follow-up in a subset of patients confirm
ducible and reliable validation of potential drug candidates. Our results clinical correlation with in vitro drug sensitivity.
strongly suggest that ceritinib, dinaciclib, alvocidib, and mebendazole Conclusions: Pediatric precision medicine programs reveal molecular
can be applied as an alternative therapeutic approach for hepatoblas- targets in a majority of patients. However, clinical benefit is limited to
toma. a small subset. Our data indicate HTS of PDTO could support clinical
decision making and improve clinical outcomes for high-risk pediatric
cancer patients.
FREE PAPER SESSION (FPS)

FPS 06: PRECISION MEDICINE 30-09-2022 8:30 AM - 9:30 AM O145 / #103 VENETOCLAX IN PEDIATRIC AND YOUNG
ADULT PATIENTS WITH RELAPSED/REFRACTORY SOLID
O144 / #1741 TO SCREEN OR NOT TO SCREEN: THAT’S THE TUMORS: UPDATED FINDINGS FROM A PHASE 1 STUDY
QUESTION! – EXPLORING DRUG SENSITIVITY PROFILING IN
THE PRECISION MEDICINE PROGRAM ITHER Daniel Morgenstern1 , Seong Khaw2 , Nicolas Gerber3 , Andrew Place4 ,
Nadege Corradini5 , Mignon Loh6 , Arnauld Verschuur7 , David
Karin Langenberg1 , Eleonora Looze1 , Michael Meister1 , Camilla Ziegler8,9,10 , Chris Fraser11 , Todd Cooper6 , Christopher Forlenza12 ,
Calandrini2 , Femke Ringnalda1 , Natasha Van Eijkelenburg1 , Miranda Maureen O’Brien13 , Sara Federico14 , Natasha Van Eijkelenburg15 ,
Dierselhuis1 , Ronald De Krijger1 , Kimberley Ober1 , Linda Schild1 , Maika Onishi16 , Kristina Unnebrink17 , Xin Chen18 , Mohamed
Jarno Drost3 , Marc Van De Wetering3 , Sander Van Hooff1 , Selma Badawi19 , Majd Ghanim20 , Tammy Palenski21 , Kelly Goldsmith22
Eising1 , Jan Koster4 , Max M. Van Noesel3 , Jan Molenaar1 1 Hospital for Sick Children & University of Toronto, Department Of Paedi-
1 Princess Máxima Center for pediatric oncology, Solid Tumors, Utrecht, atrics, Toronto, Canada; 2 The Royal Children’s Hospital, Paediatric Oncol-
Netherlands; 2 Princess Máxima Center for pediatric oncology, Solid Tumors, ogy, Victoria, Australia; 3 University Children’s Hospital, Department Of
Nijmegen, Netherlands; 3 Princess Máxima Center, Pediatric Oncology, Oncology, Zurich, Switzerland; 4 Dana-Farber/Boston Children’s Cancer &
Utrecht, Netherlands; 4 Amsterdam University Medical Center, Oncoge- Blood Disorders Center, Pediatric Hematology/oncology, Boston, United
nomics, Amsterdam, Netherlands States of America; 5 Institute for Paediatric Haematology and Oncology-
IHOPe, Leon Bérard Cancer Centre, University of Lyon, Department Of
Background and Aims: Precision oncology is a relevant strategy to Paediatric Oncology, Lyon, France; 6 Seattle Children’s Hospital, University
improve outcome for pediatric patients with high-risk, relapsed or of Washington, Department Of Pediatrics, Seattle, United States of America;
refractory cancers. The Individualized Therapies “iTHER” Program 7 Hôpital d’Enfants de la Timone, AP-HM, Pediatric Hematology And Oncol-

aims to provide individual treatment recommendations through iden- ogy, Marseille, France; 8 University of New South Wales, Sydney, Children’s
tification of targetable alterations. With molecular profiling alone, Cancer Institute, Lowy Cancer Research Centre, Sydney, Australia; 9 Sydney
cancer driving gene alterations are discovered in the majority of Children’s Hospital, Kids Cancer Centre, Randwick, Australia; 10 University
patients. However, clinical benefit is limited, stressing the need for of New South Wales, Sydney, School Of Women’s And Children’s Health,
additional models to predict responses. Here, we present results Sydney, Australia; 11 Lady Cilento Children’s Hospital, Paediatric Oncology,
from high-throughput drug screens (HTS) on patient-derived tumor Brisbane, Australia; 12 Memorial Sloan Kettering Cancer Center, Depart-
organoids (PDTO) from patients with solid tumors. ment Of Pediatrics, New York, United States of America; 13 University

Methods: Molecular profiling consists of whole-exome sequencing, of Cincinnati College of Medicine, Department Of Pediatrics, Cincinnati,
RNAseq and methylation array. Results are visualized using the R2 United States of America; 14 St. Jude Children’s Research Hospital, Depart-
bioinformatic platform. In addition, HTS is performed on PDTO using ment Of Oncology, Memphis, United States of America; 15 Princess Máxima
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S69 of S635

Center for Pediatric Oncology, Department Of Solid Tumors, Utrecht, Anirban Das1 , Daniel Morgenstern2 , Adrian Levine1 , Ayse Ercan1 ,
Netherlands; 16 Genentech, Product Development Hematology, South San Vanessa Bianchi1 , Sumedha Sudhaman1 , Santiago Ramirez1 , Liana
Francisco, United States of America; 17 AbbVie Deutschland GmbH & Co. Nobre1 , Lucie Stengs1 , Melissa Edwards1 , Valerie Larouche3 , David
KG, Data And Statistical Sciences, Ludwigshafen, Germany; 18 AbbVie, Inc, Samuel4 , An Van Damme5 , David Gass6 , David Ziegler7 , Stefan
Statistics, North Chicago, United States of America; 19 AbbVie, Inc, Clini- Bielack8 , Shayna Zelcer9 , Michal Yalon10 , Tomasz Sarosiek11 , Kim
cal Pharmacology And Pharmacometrics, North Chicago, United States of Nichols12 , Rebecca Loret De Mola13 , Kevin Bielamowicz14 , Magnus
America; 20 AbbVie, Inc, Oncology, North Chicago, United States of Amer- Sabel15 , Charlotta Frojd15 , Matthew Wood16 , Joana Cristiano Sous
ica; 21 AbbVie, Inc, Oncology Development, North Chicago, United States of Migueis17 , Chenue Abongwa18 , Lee Yi Yen19 , Enrico Opocher20 ,
America; 22 Emory University School of Medicine, Children’s Healthcare Of Duncan Stearns21 , Kanika Bhatia22 , Santanu Sen23 , Eduardo
Atlanta, Atlanta, United States of America Quiroga-Cantero24 , Palma Solano-Páez24 , Bruce Crooks25 ,
Magimairajan Issai Vanan26 , Alyssa Reddy27 , Jenny Adamski28 , Gary
Background and Aims: BCL-2 has been implicated in tumorigenesis Mason29 , Scott Lindhorst30 , Sebastien Perreault31 , Melyssa
and therapeutic resistance in pediatric solid tumors. Venetoclax is an Aronson32 , Birgit Ertl-Wagner33 , Cynthia Hawkins34 , Eric Bouffet35 ,
oral, selective BCL-2 inhibitor. We report updated findings from a study Uri Tabori1
of venetoclax (monotherapy or with cyclophosphamide+topotecan 1 Hopsital for Sick Children, Hematology Oncology, Toronto, Canada;
[Cy-Topo]) in pediatric patients with relapsed/refractory solid tumors. 2 Hospital for Sick Children & University of Toronto, Department Of Paedi-
Methods: This Phase 1, open-label, 2-part study (NCT03236857) atrics, Toronto, Canada; 3 CHU de Quebec-Centre Hospitalier de l’Universite
enrolled patients <25 years with relapsed/refractory malignancies, Laval (CHUL), Hematology/oncology, Quebec City, Canada; 4 Valley Chil-
including solid tumors. In Part 1, the initial cohort received an drens Hospital, Hematology Oncology, Madera, United States of America;
age/weight-adjusted target dose of 800 mg/day venetoclax continu- 5 Cliniques universitaires Saint-Luc, Hematology Oncology, Brussels, Bel-
ously (V800c) with optional addition of Cy-Topo (Cy 250 mg/m2 /day + gium; 6 Levine Children’s Cancer & Blood Disorders, Hematology Oncology,
Topo 0.75 mg/m2 /day; IV max 5 days). Following dose-limiting toxicity Charlotte, United States of America; 7 Sydney Children’s Hospital, Kids
(DLT) assessment, venetoclax was de-escalated to 400 mg/day contin- Cancer Centre, Sydney, Australia; 8 Klinikum Stuttgart, Hematology Oncol-
uously (V400c), or intermittently (400 mg/day [V400i] or 800 mg/day ogy, Stuttgart, Germany; 9 London Health Sciences Center, Hematology
[V800i]; days 1-10 of 21-day cycle). Primary objectives included pre- Oncology, London, Canada; 10 Sheba Medical Center, Hematology Oncol-
liminary safety and efficacy. ogy, Tel Hashomer, Israel; 11 Lux Med Onkologica, Hematology Oncology,
Results: Part 1 comprised 25 patients (data cutoff, February 9, 2022). Warsaw, Poland; 12 St Jude Childrens Research Hospital, Cancer Genet-
Median age was 12 years (range, 2-24). Median time on study was ics, Memphis, United States of America; 13 Helen Devos Childrens Hospital,
4.4 (range, 1.2-45.1) months. Common (≥3 patients) tumor types Hematology Oncology, Michigan, United States of America; 14 University

included neuroblastoma (n=10), rhabdomyosarcoma (n=4), Ewing sar- of Arkansas for Medical Sciences, Hematology Oncology, Arkansas, United
coma (n=4), and Wilms tumor (n=3). Twenty-three (92%) patients had States of America; 15 Sahlgrenska University Hospital, Hematology Oncol-
≥1 Grade 3/4 adverse event, most commonly (≥50%) anemia (68%), ogy, Gothenburg, Sweden; 16 Oregon Health & Science University, Hema-
febrile neutropenia (60%), and white blood cell count decreased (52%). tology Oncology, Oregon, United States of America; 17 Instituto Portugues
Hematologic DLTs occurred in 2/4 patients treated with V800c+Cy- de Oncologia, Hematology Oncology, Lisbon, Portugal; 18 CHOC Hospital,
Topo. Hematologic DLTs and non-hematologic DLTs (hypotension and Hematology Oncology, Orange, United States of America; 19 Taipei Vet-
oral mucositis) occurred in 4/6 patients treated with V400c+Cy- erans General Hospital, Hematology Oncology, Taipei, Taiwan; 20 Padua

Topo. Thrombocytopenia DLT occurred in 1/6 patients treated with University Hospital, Hematology Oncology, Padua, Italy; 21 Rainbow Babies
V400i+Cy-Topo. No DLTs occurred in the V800i+Cy-Topo cohort, and Children’s Hospital, Hematology Oncology, Cleveland, United States
which was determined as the recommended expansion phase dose. The of America; 22 Royal Childrens Hospital, Hematology Oncology, Melbourne,
objective response rate (complete or partial response) was 17% (1/6) Australia; 23 Kokilaben Hospital, Hematology Oncology, Mumbai, India;
with V800i and 20% (5/25) across all dose levels. 24 HOSPITAL INFANTIL VIRGEN DEL ROCIO, Pediatric Oncology, SEVILLE,
Conclusions: These findings demonstrate tolerability and efficacy of Spain; 25 IWK Health, Pediatrics, Halifax, Canada; 26 University of Man-
intermittent venetoclax dosing with Cy-Topo in pediatric patients with itoba / Cancer Care Manitoba, Pediatrics And Child Health, Winnipeg,
relapsed/refractory solid tumors. Part 2 is ongoing to further assess the Canada; 27 University of California, Hematology Oncology, San Francisco,
combination in patients with neuroblastoma or BCL-2–positive solid United States of America; 28 Birmingham Women’s and Children’s Hospital,
tumors. Hematology Oncology, Birmingham, United Kingdom; 29 University of Pitts-
burgh School of Medicine, Hematology Oncology, Pittsburgh, United States
of America; 30 Medical University of South Carolina, Hematology Oncology,
O146 / #579 DUAL CTLA4/ PD-1 BLOCKADE IMPROVES Charleston, United States of America; 31 CHU Sainte Justine, Hematology
SURVIVAL FOR REPLICATION-REPAIR DEFICIENT Oncology, Montreal, Canada; 32 Mount Sinai Hospital, Cancer Genetics,
GLIOBLASTOMA FAILING ANTI PD-1 MONOTHERAPY: AN Toronto, Canada; 33 Hospital for Sick Children, Radiology, Toronto, Canada;
IRRDC STUDY 34 Hopsital for Sick Children, Pathology, Toronto, Canada; 35 The Hospital for

Sick Children, Division Of Hematology And Oncology, Toronto, Canada


15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S70 of S635 ABSTRACTS

1 University Medicine Greifswald, Pediatric Hematology And Oncology,


Greifswald, Germany; 2 Anyxis Immuno Oncology, Immunotherapy, Vienna,
Background and Aims: High-grade gliomas (HGG) with DNA-
Austria; 3 Children’s Cancer Research Institute, Paediatric Oncology, Vienna,
replication-repair-deficiency (RRD) harbour high mutation burden
Austria
(TMB) and are rapidly fatal following chemo-radiation. Although hyper-
mutation drives prolonged survival following checkpoint-inhibition in
Background and Aims: Anti-GD2 antibodies were used in combi-
>30% of patients, salvage following failure of anti-PD1-monotherapy
nation with isotretinoin and cytokines in previous neuroblastoma
remains a challenge.
immunotherapy trials. We evaluated dinutuximab beta (DB) long-
Methods: We performed a real-world study of Ipilimumab (anti-
term infusion (LTI) as a single agent in patients (pts) with high-risk
CTLA4) plus Nivolumab/Pembrolizumab for patients failing anti-PD1-
relapsed/refractory neuroblastoma.
monotherapy.
Methods: 40 pts with relapsed/refractory neuroblastoma were
Results: Among 68 patients with relapsed HGG treated with anti-
enrolled (EudraCT 2014-000588-42; Study 304). Pts received up to 5
PD1, 43 (63%) had progression. Ipilimumab was added to 20/43
cycles of 100 mg/m2 /cycle DB-LTI over 10 days (d). Primary endpoint
(46.5%), 14 (32.5%) received best supportive care (BSC), 9 (21%)
was a treatment response according to the International Neuroblas-
got miscellaneous therapies. For patients receiving CTLA4/PD1-
toma Response Criteria (INRC). Secondary endpoints were safety,
inhibition, median age was 12.3-years (IQR:9;15.6). All had germline
pharmacodynamics and pharmacokinetics, duration of response and 3
predisposition (CMMRD: 70%, Lynch: 25%, polymerase-proofreading-
year (y) overall- and progression-free survival.
deficiency: 5%). All demonstrated hypermutation (median TMB: 182-
Results: Of 40 enrolled pts, 38 remained in the full analysis set. The
mutations/Mb; IQR:15.6;369.4). Time from anti-PD1 to progression
best overall response rate was 36.8% with 4 complete- (CR) and 10
was 8-months (IQR:3.8;18.5). Paired multi-omics analyses demon-
parital-responses (PR). There were 5 pts with a minor-response (MR)
strated clinically-relevant tumor and immune-microenvironment evo-
accounting for an INRC best response rate of 53.0%. Of 14 pts with
lution following anti-PD1. Central radiology review (iRANO) revealed
bone marrow involvement 13 pts responded (93%) (12 CR, 1 PR).
objective responses in 3/20 (15%; TMB: 320, 496, 834 mutations/Mb;
The median duration of response for pts with CR or PR was 238d
included initial pseudoprogression and multi-focal responses), sta-
[108-290d] and the 3y progression-free and overall survival were
ble disease in 5 (25%), progressive disease in 12 (60%). Estimated
31.5% ± 7.8% and 65.5% ± 8.1%, respectively. Survival rates were
progression-free and overall-survival (OS) at 18-months were 11% and
significantly higher in pts with refractory compared to relapsed neu-
25% respectively. Survival was superior to patients on BSC (median
roblastoma. Grade 3&4 adverse events with a frequency >10% were
OS <1-month, versus 12-months on CTLA4/PD1-inhibition; p <0.001).
inflammation (20%), gastrointestinal disorders (12.5%) and pyrexia
All patients on BSC died within 3.5-months, while 8/20 patients (40%)
(10%). Pain, allergic-/hypersensitivity reaction, capillary leak were less
on CTLA4/PD1 combination are alive at a median follow-up of 10.9-
frequent. No paraplegia occurred. Morphine was given in cycle (C)
months (range: 1-48). The combinational immunotherapy resulted in
1 (C1 100%) and decreased from C2 to 5 (C2 95%; C3 12%; C4
significant autoimmune toxicity (n=11/20; 55%), warranting immuno-
0%; C5 0%). The median time of hospitalization decreased from 7d
suppressive therapy in all, and treatment abandonment in 6, contribut-
(C1) to 3d (C5) and all patients received parts of the 10d LTI as
ing to subsequent progression and death. Addition of re-irradiation to
outpatient.
systemic immunotherapy impacted OS (p<0.01).
Conclusions: Single agent usage of DB as long-term infusion is highly
Conclusions: Combined CTLA4/PD1-blockade after failure of anti-
tolerable and effective in relapsed/refractory high-risk neuroblastoma
PD1 monotherapy demonstrates responses and prolonged survival
with a clinically meaningful response-rate and -duration.
in an otherwise rapidly-fatal disease. Biopsy at progression can help
unravel immune-escape and identify novel vulnerabilities. Responses
needs to be assessed in the context of significant autoimmunity, sup-
FREE PAPER SESSION (FPS)
porting the need for the current prospective trial (NCT04500548)
and development of innovative strategies to limit treatment-related
FPS 07: RETINOBLASTOMA AND GERM CELL TUMORS
toxicity.
30-09-2022 10:40 AM - 12:10 PM

O148 / #1380 TOPOTECAN ENHANCES ONCOLYTIC


O147 / #1343 SINGLE AGENT ACTIVITY OF THE ANTI-GD2
ADENOVIRUS INFECTION, REPLICATION AND ANTITUMOR
ANTIBODY DINUTUXIMAB BETA LONG-TERM INFUSION IN
ACTIVITY IN RETINOBLASTOMA
HIGH-RISK NEUROBLASTOMA PATIENTS WITH RELAPSED AND
REFRACTORY DISEASE. A MULTICENTER PHASE II TRIAL
Victor Burgueño1 , Ana Mato-Berciano2 , Guillermo Chantada1 , Angel
Carcaboso1
Holger Lode1 , Karoline Ehlert1 , Stephanie Huber1 , Nikolai Siebert1 ,
1 Hospital Sant Joan de Deu, Pediatric Oncology, Barcelona, Spain; 2 VCN
Sascha Troschke-Meurer1 , Maxi Zumpe1 , Hans Loibner2 , Ruth
Biosciences, Research And Development, Sant Cugat del Valles, Spain
Ladenstein3
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S71 of S635

Background and Aims: The oncolytic adenovirus VCN-01 shows pre- chemotherapy has been shown to prevent metastasis in children with
clinical and clinical activity in chemo-refractory retinoblastoma. The risk factors for extraocular retinoblastoma.
goal of this work was to study the combination of VCN-01 and Methods: Data were collected in a prospective multicenter observa-
chemotherapy. tional study RB-registry for all children with retinoblastoma diagnosed
Methods: We performed in vitro assays in primary retinoblastomas in Germany and Austria between 2013 and 2020. Risk-stratified adju-
and the cell line Y79 to address the antiproliferative activity of VCN- vant therapy was applied according to national guidelines based on
01 combined with topotecan, carboplatin or melphalan. We studied histopathological findings. Children with intermediate risk factors
the viral replication and cell cycle in cells exposed to such treatment (pT2b, pT3) received 3 or 6 cycles of adjuvant chemotherapy depending
combinations. In athymic mice with engrafted human retinoblas- on the risk subgroup. In case of tumor cells reaching the resection mar-
tomas, we inoculated VCN-01 in the tumors and quantified the viral gin of the optic nerve, sub-arachnoid invasion or microscopic extension
genomes upon combination with chemotherapy. In mice with intraoc- beyond the sclera (pT4, IRSS II) additional external beam radiation of
ular retinoblastoma, we evaluated the activity of intravitreous VCN-01 the orbit was performed.
alone or combined with topotecan. Results: Histopathological risk factors that qualified for adjuvant ther-
Results: In vitro, combined treatment of VCN-01 with topotecan, but apy were detected in 26.2 % of patients (n= 56) with retinoblastoma
not with carboplatin or melphalan, increased significantly VCN-01 and primarily enucleated eyes (n=214). The median follow-up was 2.6
infection and replication in retinoblastoma cells (P = 0.0007). S-phase years (range 0.0-7.8 years). None of the children in the low or interme-
cell cycle arrest induced by topotecan was the likely cause for the diate risk group (pT1-pT3, IRSS I) developed extraocular relapse after
enhancement of viral replication. In mice bearing human retinoblas- enucleation alone or with risk adapted adjuvant chemotherapy. Two of
tomas, topotecan administration after intratumoral VCN-01 increased five children with pT4, IRSS II in the high risk group developed extraoc-
viral genomes production and total infected cells compared with the ular relapses resulting in a 2 year EFS of 60% and a 2 year overall
administration of VCN-01 alone (P = 0.0002). Sequential adminis- survival of 80%.
tration of VCN-01 and topotecan increased median ocular survival Conclusions: Primary enucleation alone and with additional adjuvant
significantly, compared to VCN-01 alone (P = 0.0364). chemotherapy treatment provides high cure rates in patients with pT1-
Conclusions: Our study demonstrates that topotecan treatment in 3 retinoblastoma (IRSS I). The overall survival was statistically notable
oncolytic adenovirus-infected retinoblastoma cells enhances viral lower in children with pT4 retinoblastoma despite adjuvant chemo-
infection and replication, leading to the improvement of VCN-01 and radiotherapy. International prospective clinical trials are required
oncolytic activity. Because both agents are approved for research to evaluate reduction of intensity of adjuvant chemotherapy in some
and treatment use in patients with retinoblastoma, we anticipate the risk groups (pT2,pT3) and intensification of the adjuvant therapy in pT4
clinical impact of our experimental results. retinoblastoma. Funded by Deutsche Kinderkrebsstiftung

O149 / #375 SURVIVAL AFTER PRIMARY ENUCLEATION O150 / #1653 CLINICOPATHOLOGICAL SIGNIFICANCE OF
AND ADJUVANT THERAPY FOR ADVANCED LOCALIZED ANGIOGENESIS MARKERS IN PRIMARY AND CHEMOREDUCED
RETINOBLASTOMA IN TWO HIGH INCOME COUNTRIES RETINOBLASTOMA WITH THE PATIENT OUTCOME

Yelena Diarra1 , Karen Fischhuber2 , Saskia Ting3 , Madlen Reschke4 , Nikhil Kumar1 , Seema Kashyap1 , Lata Singh2 , Mithalesh Singh3 ,
Petra Ritter-Sovinz5 , Nikolaos Bechrakis6 , Beate Timmermann7 , Eva Rachna Meel4 , Seema Sen1 , Neiwete Lomi4
Biewald6 , Angelika Eggert4 , Petra Ketteler1 1 All India Institute of Medical Sciences, Ocular Pathology, New Delhi,
1 University Hospital Essen, Department Of Pediatrics Iii, Essen, Ger- India; 2 All India Institute of Medical Sciences, Pediatrics, New Delhi, India;
many; 2 University of Münster, Institute Of Biostatistics And Clinical 3 University of California, Ophthalmology, California, United States of Amer-

Research, Münster, Germany; 3 University Hospital Essen, Institute Of ica; 4 All India Institute of Medical Sciences, Ophthalmology, New Delhi,
Pathology, Essen, Germany; 4 Charité Universitätsmedizin, Department Of India
Pediatric Hematology And Oncology, Berlin, Germany; 5 Medical University
Graz, Department Of Pediatric Hematology And Oncology, Graz, Austria; Background and Aims: Angiogenesis plays a significant role in the
6 University Hospital Essen, Department Of Ophthalmology, Essen, Ger- pathogenesis of retinoblastoma. Our aim is to compare the pathophys-
many; 7 University Hospital Essen, Department Of Particle Therapy, West iological conditions of primary and chemoreduced retinoblastoma (RB)
German Proton Therapy Centre Essen (wpe), West German Caner Center with the help of angiogenesis markers (CD31 and VEGF) and their
(wtz), German Cancer Consortium (dktk), Essen, Germany correlation with the patient outcome.
Methods: Thirty-five patients each of primary and chemoreduced
Background and Aims: Advanced intraocular retinoblastoma can be RB were included in this study. Clinicopathological parameters of
cured by enucleation, but extraocular spread of retinoblastoma cells all patients were recorded and immunohistochemistry and quan-
beyond the natural limits of the eye is related to a high mortality. For titative real-time PCR (qRT-PCR) was performed on all 70 cases.
children with histopathological risk factors for metastasis, adjuvant Overall Survival (OS) and Progression-Free Survival (PFS) was
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S72 of S635 ABSTRACTS

calculated using Kaplan-Meier analysis for both the groups of examine health outcomes, including vision, in patients with RB treated
RB. from 2007 to the present.
Results: In primary RB, both VEGF (p=0.013) and CD31 (p<0.001) Methods: Medical record abstraction was performed for disease pre-
expression were statistically significant with tumor staging. CD31 sentation, treatment, and visual acuity. A subset of patients have
was also significant with choroidal invasion and optic nerve inva- also completed functional vision questionnaires (Child Vision Function
sion (p<0.001). In chemoreduced RB, both CD31 & VEGF expression Questionnaire, ages 0 – 7 and Cardiff Visual Ability Questionnaire for
were significant with choroidal invasion and macrophage infiltration. Children ages >8).
In chemoreduced RB, decreased PFS was observed with increased Results: Among 74 participants treated with IAC, 56 self-reported
expression of both CD31 and VEGF but was statistically significant functional vision, with mean scores of 0.56 for <3 years old, 0.49 for
only with CD31 (p=0.0047). Similarly, in primary RB, the decreased 3-7 years old and -0.98 for those >8 years old, all considered good
PFS was observed with increased expression of CD31 & VEGF but was function. Visual acuity was reported in 56 eyes treated with IAC in
not statistically significant. 52 patients. Eleven eyes underwent secondary enucleation. Of the 45
Conclusions: Our data showed that increased expression of angio- eyes salvaged, 11 had normal vision (20/20 - 20/40), eye group (Inter-
genesis markers in primary and chemoreduced retinoblastoma are national Intraocular Retinoblastoma Classification) 2 B, 5 C, and 4 D.
essential for local and systemic invasive growth. Combination of anti- Moderate vision loss (>20/40 - 20/70) was noted in 10 eyes, 2 B, 1 C,
angiogenic and chemotherapeutic drugs would be more effective in the 6 D, and 1 E. Five eyes had low vision (>20/70 < 20/200) 1 B and 4 D.
management of retinoblastoma. Nineteen eyes were legally blind (>/=20/200). 4 B, 1 C, 12 D, and 2 E.
There was no apparent difference in visual function or acuity in those
also treated with (21) or without (53) systemic chemotherapy. Number
O151 / #1171 VISUAL OUTCOMES FOLLOWING of IAC treatments was not associated with degree of visual acuity.
INTRA-ARTERIAL CHEMOTHERAPY FOR RETINOBLASTOMA: A Conclusions: Among patients treated with IAC, self-reported func-
REPORT FROM THE RIVERBOAT CONSORTIUM tional vision is good, but most had unilateral disease. In contrast, the
majority (24) of IAC treated eyes demonstrate low vision or legal
Debra Friedman1 , Emma Schremp1 , Tatsuki Koyama2 , Lili Sun2 , Lori blindness, most commonly D or E eyes. With accrual ongoing, we will
Ann Kehler3 , Anthony Daniels3 , Amish Shah4 , Ann Leahey4 , Helen gain an improved understanding of visual outcomes following IAC for
Dimaras5 , Rajaram Nagarajan6 , Robert Hayashi7 , Mary Lou Schmidt8 , RB.
Murali Chintagumpala9 , Cynthia Herzog10 , Sandra Luna-Fineman11 ,
Claire Fraley11 , Joanna Weinstein12 , Bruce Crooks13 , Thomas
Olson14 , Cindy Schwartz15 , Joseph Neglia16 O152 / #577 PAEDIATRIC AND ADOLESCENTS GERM CELL
1 Vanderbilt University Medical Center, Pediatrics, NASHVILLE, United TUMORS: RESULTS FROM THE FRENCH TGM13 PROTOCOL
States of America; 2 Vanderbilt University Medical Center, Biostatistics,
NASHVILLE, United States of America; 3 Vanderbilt University Medical Cen- Cécile Faure Conter1 , Daniel Orbach2 , Helene Sudour-Bonnange3 ,
ter, Ophthalmology, NASHVILLE, United States of America; 4 Children’s Brice Fresneau4 , Cécilé Vérité5 , Ludovic Mansuy6 , Angélique Rome7 ,
Hospital of Philadelphia, Pediatrics, Philadelphia, United States of Amer- Cecile Dumesnil8 , Estelle Thebaud9 , Marleen Renard10 , Frederic
ica; 5 Hospital for Sick Kids, Opthalmology, Toronto, Canada; 6 Cincinnati Hameury11 , Aude Fléchon12 , Frederique Dijoud13 , Sylvie Chabaud14
Childrens Medical Center, Pediatrics, Cincinnati, United States of Amer- 1 IHOPE, Pediatry, bron, France; 2 Institut Curie, Siredo Oncology Depart-
ica; 7 Washington University School of Medicine, Pediatrics, st. louis, United ment, Paris, France; 3 Centre Oscar Lambret, Pediatry, Lille, France; 4 Gustave
States of America; 8 Children’s Hospital of the University of Illinois, Pedi- Roussy, Department Of Pediatric Oncology, Université Paris-saclay, Ville-
atrics, chicago, United States of America; 9 Texas Children’s Hospital, Pedi- juif, France; 5 CHU de Bordeaux, Oncologie Et Hematologie Pédiatrique,
atrics, Houston, United States of America; 10 The University Of Texas MD Bordeaux, France; 6 CHU Nancy, Pediatry, Vandoeuvre les nancy, France;
Anderson Cancer Center, Pediatrics, Houston, United States of America; 7 APHM, Department Of Pediatric Immunology, Hematology And Oncol-
11 University of Colorado School of Medicine, Pediatric Oncology, Aurora, ogy, Children’s Hospital Of La Timone Marseille, Provence-Alpes-Côte d’Azur,
United States of America; 12 Ann & Robert H. Lurie Children’s Hospital France; 8 CHU Rouen, Pediatry, Rouen, France; 9 CHU de Nantes, Oncologie
of Chicago, Pediatrics, chicago, United States of America; 13 IWK Health, Et Hématologie Pédiatrique, Nantes, France; 10 université de Leuven, Pedi-
Pediatrics, Halifax, Canada; 14 Children’s Hospital of Atlanta, Pediatrics, atry, Leuven, Belgium; 11 Hospices civils de Lyon, Pediatric Surgery, Bron,
Atlanta, United States of America; 15 Children’s Wisconsin, Pediatrics, Mil- France; 12 Centre Léon Bérard, Medical Oncology, Lyon, France; 13 Hospices
waukee, United States of America; 16 University of Minnesota, Pediatrics, civils de Lyon, Pathology, Bron, France; 14 centre Léon Bérard, Statistics, lyon,
Minneapolis, United States of America France

Background and Aims: Use of intra-arterial chemotherapy (IAC) in Background and Aims: Chemotherapy in non seminomatous germ
retinoblastoma (RB) provides the potential to improve globe salvage cell tumors (NSGCT) exposes to dose-dependent toxicities. TGM13-NS
with vision preservation. The Research Into Visual Endpoints and protocol (EUDRACT 2013-004039-60) aimed to decrease chemother-
RB Health Outcomes After Treatment consortium was established to apy while maintaining five year event free survival (5yEFS) ≥ 80%.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S73 of S635

Methods: Patients ≤ 19 years with disseminated NSGCT were enrolled Methods: A total of 42 GCTs pediatric patients were evaluated, includ-
(05/2014-05/2019) in France and Belgium. Patients were stratified in 4 ing 11 testicles and 31 ovaries with distinct histological patterns. As a
groups: 2 Intermediate Risk [IR: localized tumor with low tumor marker control group, we consider five samples of adjacent normal ovarian tis-
(TM)] groups treated with VBP (vinblastine bleomycin cisplatin) and sue and five testicular. The expression profile of miRNAs was analyzed
2 High Risk (HR: metastatic or high TM) groups treated with etopo- by NanoString platform using the panel Human v3 miRNA Assay CSO.
side and cisplatin and either ifosfamide (VIP) or bléomycine (BEP). IR1 The data was analyzed with R/Bioconductor.
(ovarian tumor any age/testis tumors <10 years) and HR1 (ovarian Results: The hierarchical clustering analysis showed that normal sam-
tumor any age/testis tumors < 10 years and low TM/ testis tumors > 10 ples and teratomas clustered separately from the malignant tumors
years and very low TM) groups received 3 courses, IR2 (extragonadal (yolk sac tumor, embryonic carcinoma, dysgerminoma). Twenty-six
tumor <10 years) and HR2 (remainders) received 4 courses. microRNAs were differentially expressed in common among the
Results: 115 patients were included: median age: 12.8 years (0.4-18.9); three malignant histological types, including miR-302b-3p, miR-302a-
tumors sites: ovary n=44: testis n=37, extragonadal n=34. 5yEFS and 3p, miR-372-3p, miR-373-3p, miR-367-3p, and miR-371a-5p. These
OS were 87% (95%IC, 80-92) and 95% (89-98) respectively (median six microRNAs were related to the clinical-pathological character-
follow up of 3.5 years), comparable to the previous TGM95 protocol istics (histological types, risk, metastasis, AFP marker, survival) and
with lower mean cumulative dose of cisplatin (382 mg/m2 versus 512 miR-302b-3p was the only one that showed significant correlation
mg/m2 respectively). 5yEFS were 93% (95%CI 80-98), 88% (71-95) and with all events. Yolk sac tumor showed significant overexpression of
79% (62-90) for ovarian, testicular and extra gonadal tumors respec- miR-302b-3p compared with embryonal carcinoma, teratomas, and
tively. 5yEFS was significantly different (p=0.022) according to risk dysgerminoma.
groups: 64% (30-85), 90% (66-97), 95% (72-99) and 87% (74-94) for Conclusions: Our results suggest that miRNA-302b-3p are promising
IR2, IR1, HR1 and HR2, respectively. Analysis of TM decline after 1 candidate biomarkers in malignant GCTs and miRNAs may improve the
course showed a prognostic impact of time to normalization (weeks, quality of the care of patients, contributing to personalized medicine.
Fizazi-JCO-2004) and AFP decrease (Log10) on EFS (HR=1.03 (1.003-
1.007) and 3.39 (0.92-12.56), respectively after adjustment on tumor
site and baseline AFP level. FREE PAPER SESSION (FPS)
Conclusions: Risk adapted strategy with limited and fixed number of
chemotherapy courses leads to excellent outcomes, exclusive of young FPS 08: MYELOID LEUKEMIAS 30-09-2022 10:40 AM - 12:10
extragonadal IR tumors that deserve more intensive chemotherapy PM

O154 / #251 IDENTIFYING MECHANISMS OF RESISTANCE


O153 / #724 MIRNA-302B AS POTENTIAL TARGET FOR IN PEDIATRIC PATIENTS WITH ACUTE MYELOID LEUKEMIA
THERAPEUTIC INTERVENTION OF PEDIATRIC MALIGNANT USING COMPREHENSIVE LONGITUDINAL SINGLE CELL
GERM CELL TUMORS PROFILING

Ana Glenda Santarosa Vieira1 , Luciane Da Silva2 , Thaíssa Faria1 , Ana Sander Lambo1 , Diane Trinh1 , Dan Jin1 , Lisa Wei1 , Rhonda Ries2 , Scott
Laus2 , Gisele Martins1 , Rui Reis2 , Mariana Pinto2 , Luiz Fernando Furlan3 , Soheil Meshinchi4 , Marco Marra1
Lopes3 1 BC Cancer, Canada’s Michael Smith Genome Sciences Centre, Vancou-
1 Barretos Children’s Cancer Hospital, Pediatric Oncology, BARRETOS, ver, Canada; 2 Fred Hutchinson Cancer Research Center, Clinical Research
Brazil; 2 Barretos Cancer Hospital, Molecular Oncology Research Center, Division, Vancouver, United States of America; 3 Fred Hutchinson Cancer
BARRETOS, Brazil; 3 Barretos Children’s Cancer Hospital, Pediatric Hospital, Research Center, Clinical Research Division, d, United States of Amer-
Bairro Paulo Prata, Barretos, San Paulo, Brazil ica; 4 Fred Hutchinson Cancer Research Center, Clinical Research Division,
Seattle, United States of America
Background and Aims: Germ cell tumors (GCTs) are derived from
primordial germ cells, can occur in gonadal and extragonadal sites, Background and Aims: Pediatric acute myeloid leukemia (pAML) is
affect individuals of any age group and present distinct histological pat- a heterogeneous disease in terms of driver alterations, treatment
terns. Although alpha-fetoprotein (AFP) and beta fraction of chorionic response and patient outcomes. Although 90% of pAML patients
gonadotropin (BHCG) are biological markers of GCTs, there is no spe- respond to initial treatment, tumors relapse frequently. Despite large-
cific molecular marker for the diagnosis/prognosis of pediatric GCTs. scale genomic sequencing efforts, it remains elusive why tumors
Recent studies have suggested an involvement of microRNAs (miR- relapse. However, abundance of mutations affecting transcriptional
NAs), a group of small non-coding RNAs, as tumor biomarkers and regulation suggests that processes such as epigenetic (de)regulation
possible therapeutic targets. Nevertheless, the involvement of miRNAs may contribute to treatment resistance in pAML. Therefore, we stud-
in pediatric GCT is unclear. Thus, the aim of this study was to evaluate ied how epigenetic patterns and transcriptional regulation change over
the expression of miRNAs in pediatric ovarian and testicular GCTs and the course of treatment using single-cell ATAC sequencing (scATAC-
relate them to clinicopathological data. seq) and single-cell RNA sequencing (scRNA-seq).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S74 of S635 ABSTRACTS

Methods: We profiled 330,047 cells using scRNA-seq and 353,984 Results: We studied 110 pediatric (median age 6.1 years, 38% female)
cells using scATAC-seq derived from 28 patients with pAML, driven by patients (70 ALL, 39 AML, one undifferentiated leukemia). In the
either MLL, CBFB, RUNX1 or FLT3 aberrations, that were all treated unsupervised hierarchical cluster analysis (HCA), patients were sep-
with the proteasome inhibitor bortezomib. Biopsies were taken from arated mainly by lineage, with no association with age, sex, central
the bone marrow or peripheral blood, obtained serially at diagnosis, at nervous system infiltration, or leukocyte count. Kaplan-Meier analy-
remission and after relapse. sis revealed clusters with significantly different (p=0.014) event-free
Results: We used scATAC-seq and scRNA-seq to infer distinct cell pop- survival (EFS): poor (clusters P1 and P4), good (P2), and intermediate
ulations. These populations were used to detect the genomic locations (P3). Notably, clusters P1, P2, and P4 branched off again according to
of active enhancers, super-enhancers and active promoters, signify- lineage (P1 and P4 included most patients with AML and ALL, respec-
ing the regulatory network in each population of cells. We were able tively). Within patients with ALL, we saw a group with a non-significant
to detect cell populations that contract or expand during the course worse outcome (P4ALL) compared with the remaining patients (P2ALL
of treatment and found that relapsed tumors driven by MLL rear- and P3ALL). In contrast, in patients with AML, the HCA distinguished a
rangements acquired an earlier state of differentiation compared to group of 14 patients (P1AML) with significantly worse outcomes (OS at
tumors at diagnosis. Throughout the cohort, resistance did not occur five years 64.3%±12.8) vs. the remaining patients, all showing excellent
through reactivation of the NF-κB signalling pathway, that is canon- survival (P2AML, 90.5%±6.4), p=0.028. EFS at 5 years was also statis-
ically targeted by bortezomib. Instead, we have identified that in tically different: 43%±13.2 (P1AML) vs. 86.5%±7.2 (P2AML), p=0.003.
absence of NF-κB signalling, rewiring resulting from upregulation of P1AML included cases from all molecular categories. We identified
other receptor-mediated signalling can drive the expression of known 24 miRNAs differentially expressed in P1AML patients. The 24 miR-
pAML drivers in relapsed tumors. NAs signature included 23 underexpressed miRNAs (among them,
Conclusions: Our study shows that treatment resistance can occur some miRNAs regulating stemness, cell proliferation, or apoptosis) and
through rewiring of transcriptional networks in patients with pAML. one overexpressed miRNA previously described as an oncomiR. Thus,
These rewired networks may present possible therapeutic strategies within each molecular category, a miRNA signature could distinguish
to increase patient outcome when combined with bortezomib. patients with activated stemness and proliferation pathways and more
aggressive leukemia.
Conclusions: We obtained a distinctive signature of 24 miRNAs
O155 / #1276 A MIRNA SIGNATURE RELATED TO distinguishing pediatric patients with AML with excellent or poor
STEMNESS IDENTIFIES HIGH-RISK PATIENTS IN PEDIATRIC outcomes.
ACUTE MYELOID LEUKEMIA

Elena Esperanza Cebollada1,2 , Soledad Gómez-González3,4 , Nerea O156 / #1520 A GENOME-WIDE ANALYSIS OF CHILDHOOD
Vega García1,2 , Clara Vicente Garcés1,2 , Sara Perez2 , Montserrat ACUTE MYELOID LEUKAEMIA
Torrebadell1,2,5 , Susana Rives2,5,6 , José Luis Dapena2,6 , Albert
Català2,5,6 , Mireia Camós1,2,5 Audrey Bonaventure1 , Cassandre Chupeau1 , Laure Faure1,2 , Anne
1 Pediatric Cancer Center Barcelona (PCCB), Hospital Sant Joan de Déu Boland3 , Jean-François Deleuze3 , Jacqueline Clavel1,2
Barcelona, Hematology Laboratory, Esplugues de Llobregat, Barcelona, 1 CRESS, Université Paris Cité, INSERM UMR 1153, Epidemiology Of Child-

Spain; 2 Pediatric Cancer Center Barcelona (PCCB), Institut de Recerca Hos- hood And Adolescent Cancer (epicea), Paris, France; 2 Registre National des
pital Sant Joan de Déu, Leukaemia And Other Pediatric Hemopathies, Cancers de l’Enfant, Groupe Hospitalier Universitaire Paris-sud, Assistance
Developmental Tumors Biology Group, Esplugues de Llobregat, Spain; Publique Hôpitaux De Paris (ap-hp), Villejuif, France; 3 Université Paris-
3 Hospital Sant Joan de Déu, Pediatric Cancer Center Barcelona (pccb), Saclay, CEA, Centre National De Recherche En Génomique Humaine (cnrgh),
Barcelona, Spain; 4 Fundació Sant Joan de Déu, Developmental Tumor Evry, France
Biology Laboratory, Barcelona, Spain; 5 Instituto de Salud Carlos III, Cen-
tro De Investigación Biomédica En Red De Enfermedades Raras (ciberer), Background and Aims: Genome-wide association studies (GWAS)
Madrid, Spain; 6 Pediatric Cancer Center Barcelona (PCCB), Hospital Sant have successfully identified several genes involved in the develop-
Joan de Déu Barcelona, University of Barcelona, Pediatric Hematology And ment of childhood acute lymphoblastic leukaemia, but few studies have
Oncology Department, Barcelona, Spain focused on pediatric acute myeloid leukaemia (AML). Therefore, we
aimed to conduct a GWAS to assess which common polymorphisms are
Background and Aims: Background: MiRNAs regulating hematopoi- associated with childhood AML.
etic stem cells (HSC) expression could be prognostic biomarkers in Methods: Children enrolled to a French nationwide population-based
leukemia. Aim: Analyze the role of miRNAs regulating HSC pathways study (ESTELLE) in 2009-2010 were genotyped using an Illumina GSA
(NOTCH, WNT/beta-catenin, HOX, and FLT3) in pediatric patients with platform. After strict quality controls and genome-wide imputation of
acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). the autosomal polymorphisms (reference HRC panel), genome-wide
Methods: Analysis of the expression of 89 selected miRNAs by quanti- analyses adjusted for age (0-1, 2-9, 10-14 years) and 5 significant prin-
tative PCR. cipal components were performed using the SNPTEST software. The
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ABSTRACTS S75 of S635

replication analyses will be computed using data from an independent Results: A set of nine upregulated LSC DE-lncRNAs (p<0.05) was
population-based case-control study (ESCALE, 2003-2004). identified with a low absolute expression in HSC. Three of them
Results: 50 out of 99 cases (50.5%) and 196 out of 1421 controls (lnc-CHST2-2, lnc-EPS15L1-3, and lnc-KLHL25-4) were unique to the
(13.8%) were included in the main analyses. Genome-wide association LSC-HSC differential expression analysis. Seven miRNAs (miR-196b-
analyses highlighted a strong signal in region 10q11 (27 SNPs with 5p, miR-381-3p, miR-411-5p, miR-485-5p, miR-654-3p, miR-6724-5p,
10-7 <p<10-6 ), as well as other slightly less significant (10-6 <p<10-5 ) and miR-30a-5p) were anti-correlated (Pearson’s r<0.7) to all upreg-
signals in 4q21 (29 SNPs), 12q21 (13 SNPs). We will present these find- ulated LSC DE-lncRNAs (unique and shared with L-blast fraction). By
ings in the context of childhood AML subtypes and of the findings using GSEA, involvement of (anti-)correlated coding genes in various cancer
data from the ESCALE study. pathways was observed for the three unique upregulated lncRNAs in
Conclusions: These findings highlight genomic regions possibly associ- the LSC.
ated with childhood AML. Further analyses are required for replication Conclusions: We provide a unique set of LSC-and L-blast-specific lncR-
and to assess whether and how these loci could be involved in the NAs in pedAML and their interaction with miRNAs, ultimately impact-
development of childhood AML. ing gene expression. Furthermore, we also present a rich source of
promising targets, most of which not previously described in pedAML,
and networks that could serve as anchor points for further functional
O157 / #444 DECIPHERING THE NON-CODING LANDSCAPE experimental research.
OF PEDIATRIC ACUTE MYELOID LEUKEMIA

Laurens Van Camp1 , Jolien Vanhooren1 , Barbara Depreter2 , Martijn O158 / #1215 THE CLONAL EVOLUTION OF
De Jong1 , Anne Uyttebroeck3 , An Van Damme4 , Laurence Dedeken5 , MLL-REARRANGED THERAPY-RELATED PEDIATIC ACUTE
Marie-Françoise Dresse6 , Jutte Van Der Werff Ten Bosch7 , Mattias MYELOID LEUKEMIA
Hofmans8 , Jan Philippé8 , Barbara De Moerloose1 , Tim Lammens1
1 Ghent University Hospital, Department Of Pediatric Hematology-oncology Eline Bertrums1,2,3 , Axel Rosendahl Huber1,2 , Jurrian De Kanter1,2 ,
(pho), Ghent, Belgium; 2 University Hospital Brussels, Department Of Lab- Marry Van Den Heuvel-Eibrink1,4 , C. Michel Zwaan1,3 , Bianca
oratory Hematology, Brussels, Belgium; 3 UZ Leuven, Pediatric Hemato- Goemans1 , Ruben Van Boxtel1,2
oncology, Leuven, Belgium; 4 Cliniques universitaires Saint-Luc, Hematology 1 Princess Máxima Center, Pediatric Oncology, Utrecht, Netherlands;
Oncology, Brussels, Belgium; 5 Queen Fabiola Children’s University Hos- 2 Oncode Insitute, Oncology, Utrecht, Netherlands; 3 Erasmus Medical Cen-
pital, Department Of Pediatric Hematology-oncology, Brussels, Belgium; ter, Pediatric Oncology, Rotterdam, Netherlands; 4 Utrecht University, Pedi-
6 University Hospital Liège, Department Of Pediatric Hematology-oncology, atric Oncology, Utrecht, Netherlands
Liège, Belgium; 7 University Hospital Brussels, Department Of Pediatric
Onco-hematology, Brussels, Belgium; 8 Ghent University Hospital, Depart- Background and Aims: One of the late consequences of chemother-
ment Of Diagnostic Sciences, Ghent, Belgium apy exposure after cancer treatment is therapy-related acute myeloid
leukemia (t-AML). However, the exact origin and clonal evolution of
Background and Aims: Pediatric acute myeloid leukemia (pedAML) t-AML, especially in children, is thus far not elucidated.
is a heterogeneous blood cancer of childhood age. Although survival Methods: We studied mutation accumulation and performed phylo-
rates have significantly improved over the past decades, still 20-30% genetic lineage tracing in 17 pediatric t-AML patients with a vari-
of children will succumb due to treatment-related toxicity and relapse. ety of first cancers, by whole genome sequencing (WGS) of normal
Extensive characterization of the leukemic stem cell, shown to be hematopoietic stem and progenitor cells (HSPCs) and leukemic blasts,
responsible for relapse, is needed to improve treatment options and before and after chemotherapy exposure.
survival. Recently, it became clear that also non-coding RNAs, including Results: In four t-AML patients, we identified cells that phenotypi-
long non-coding RNAs (lncRNAs) and microRNAs (miRNAs), play a role cally resembled HSPCs, but shared the MLL rearrangement with the
in the development of human diseases, among which pediatric cancer. leukemic blasts (MLLr-HSPCs). We used these examples to study the
Nevertheless, non-coding RNA expression data in pedAML are scarce. effect of chemotherapy exposure on different cells. Five MLLr-HSPCs
Methods: Here, we explored lncRNA (n=30168) and miRNA (n=627) of patient UPN024, treated for T-cell lymphoblastic lymphoma (T-LBL),
expression in pedAML subpopulations (leukemic stem cells (LSC) and showed thiopurine-induced mutations (reflected by mutational signa-
leukemic blasts (L-blast)) and their normal counterparts (hematopoi- ture SBS87). The remaining three MLL-normal HSPCs did not show
etic stem cells (HSC) and control myeloblasts (C-blast), respectively). SBS87-contribution. Interestingly, SBS87 was accompanied by SBS1, a
Potential regulatory activity of differentially expressed (DE-)lncRNAs division-associated signature. Patient UPN014 showed a similar phe-
in the LSC (unique and shared with L-blast fraction) on miRNAs nomenon, treated for ALL, although this t-AML had an additional KRAS
was assessed. Moreover, pre-ranked gene set enrichment analyses driving mutation, not shared by the MLLr-HSPCs. Interestingly, both
(GSEA) of (anti-)correlated protein-coding genes were performed patients developed t-AML already during therapy of their first cancer.
to predict involvement in cancer pathways (using Hallmark gene Contrasting, cisplatin-associated mutations (SBS31) were found in all
sets). HSPCs and t-AML blasts of patient UPN008, treated for osteosarcoma.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S76 of S635 ABSTRACTS

In this patient, the t-AML had no additional mutations compared to the 128.2 ± 15.9 and 158.9 ± 7.3 cm. The mean height z-scores at diagno-
six MLLr-HSPCs, indicating these cells were part of the leukemic blast sis and study enrolment were -0.89 ± 1.4 and -1.05 ± 1.03 (p=0.55).
population. Two patients were stunted at diagnosis as well as at study enrolment.
Conclusions: In multiple t-AML patients MLLr-HSPCs likely gave rise In addition, one patient was stunted at study enrolment as per refer-
to a pool of (pre-)leukemic cells that started dividing during, or shortly ence growth charts; however, he had attained the target mid-parental
after, treatment. In two cases, the t-AML blasts showed that an addi- height.
tional driver was required for full-blown leukemia, which could be Conclusions: Imatinib therapy results in growth deceleration; how-
explained by evolution of pre-leukemic MLLr-HSPCs into leukemia. ever, there is catch-up growth during adolescence and the final height
However, in one case, MLLr-HSPCs were genetically indistinguishable attained is within the reference range.
from the t-AML blasts, similar to previous reports of leukemic stem
cells.
CCI

O159 / #757 THE FINAL HEIGHT IN CHILDREN AND CCI: TACKLING THE CHALLENGES: SURVIVORSHIP PROJECTS
ADOLESCENTS WITH CHRONIC MYELOID LEUKEMIA AROUND THE GLOBE 30-09-2022 10:40 AM - 12:10 PM
RECEIVING IMATINIB IS NOT REDUCED
O160 / #1303 DREAMING WITH SURVIVORS –
Rohit Sadanand1 , Rama Walia2 , Naresh Sachdeva2 , Harvinder Kaur3 , PORTUGUESE PAEDIATRIC CANCER SURVIVORS
Shano Naseem4 , Richa Jain1 , Amita Trehan1 , Deepak Bansal1
1 Advanced Pediatrics Center, Postgraduate Institute of Medical Educa- Tiago Costa, Joana Silvestre, Margarida Cruz
tion and Research, Pediatric Hematology Oncology Unit, Chandigarh, India; ACREDITAR - Associação de pais e amigos de crianças com cancro, South,
2 Postgraduate Institute of Medical Education and Research, Endocrinol- Lisbon, Portugal
ogy, Chandigarh, India; 3 Advanced Pediatrics Center, Postgraduate Institute
of Medical Education and Research, Child Growth And Anthropology Background and Aims: In Portugal, about 400 new cases of children
Unit, Chandigarh, India; 4 Postgraduate Institute of Medical Education and and young people with cancer are diagnosed every year. It is a disease
Research, Hematology, Chandigarh, India that affects the whole family functioning and that, besides the suffering
caused by the treatment, continues to impose several difficulties in the
Background and Aims: Imatinib is widely reported to result in growth period after the illness. Acreditar – Associação de Pais e Amigos de Cri-
deceleration in children with chronic myeloid leukemia (CML). How- anças com Cancro, has accompanied these patients and survivors for
ever, it is unclear if the final height is affected. The aim was to deter- 27 years.
mine if imatinib has a lasting effect on linear growth with prolonged Methods: The project was born from the unequal opportunities felt by
use. survivors of childhood cancer, who together with health professionals
Methods: The cross-sectional study was conducted in a single discussed and debated the main problem areas in living and surviving
center during 2020-21. Patients with chronic-phase CML receiv- the disease. From this discussion, four areas requiring improvement
ing imatinib were screened. The inclusion criteria included age at were prioritized: 1. Long-term follow-up. 2. Socio-economic issues. 3.
diagnosis below 13 years and attainment of skeletal maturity at Caring for those who care. 4. Raising awareness of childhood cancer.
study enrolment. The exclusion criteria included treatment abandon- Results: Eighty-nine young people were involved in the working groups
ment, poor compliance to imatinib, transformation to accelerated and had the following results: a) 20 empowerment actions: 150 young
or blast phase, or treatment with growth hormone or hematopoi- people took part; b) Preparation of a white paper on survivorship
etic stem-cell transplant. Anthropometry (height, weight, triceps follow-up; c) Advocacy campaign with the objective of implementing
skinfold thickness) and sexual maturity rating were obtained. The the right to be forgotten in Portugal: Law N◦ 75/2021 of 18 November;
evaluation included thyroid function, FSH, LH, testosterone, estro- d) 71 carers took part in well-being and clarification activities; e) 19
gen, insulin-like growth factor-1, vitamin-D, parathormone, cortisol, awareness actions; f) Production of a game about paediatric oncology
tTG-IgA, blood gas, and bone age. Additionally, the height at pre- for young people.
vious visits was recorded from the clinic files. Population-specific Conclusions: The Dreaming with Survivors project proved to be rel-
growth charts and mid-parental target height were utilized as a evant in view of the needs identified in the literature on childhood
reference. cancer, being in line with international guidelines in this particularly
Results: Forty-six patients with CML were screened; 16 satisfied the about the importance of giving active voice to children and young
study criteria. The mean age at diagnosis and study enrolment was people and encouraging their participation in the reflection and plan-
9.5 ± 2.6 and 22.2 ± 3.9 years. The median duration of imatinib ther- ning process of health policies and services which affect them. This
apy was 14.3 years (IQR: 11.5, 15.7). The duration of follow-up was project represented a qualitative leap in the intervention carried out
207.5 patient-years. The mean height at diagnosis and enrolment was by Acreditar.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S77 of S635

O161 / #937 PASSPORT2LIFE (P2L) PROJECT – PROVIDING (QI) oral care intervention project to decrease severity of oral mucositis
CHILDHOOD CANCER SURVIVORS ACCESS TO DEDICATED during childhood cancer treatment at three childhood cancer centers.
LATE EFFECT CLINICS IN SURVIVORSHIP IN INDIA Methods: Using a Plan, Do, study, Act (PDSA) approach, evidence was
first explored to determine the best oral care intervention; sodium
Sitara ., Birendra Karki, Ajay Kumar, Nirbhay Singh bicarbonate rinses were chosen. Several oral assessment tools were
Cankids Kidscan, Patient And Survivor Group, New Delhi, India explored, and the Oral Assessment Guide (OAG) was selected. The
OAG scores range from 8-24; a score of 9-15 indicates mucositis and
Background and Aims: In 2016 the sudden and unexpected death of a >16 reveals severe mucositis. Stakeholders at the three sites were
childhood cancer survivor (who was not under any regular follow-up engaged, and nurses were trained how to administer oral care rinses
for late effects) employed at Cankids was the trigger for the Pass- and perform oral assessments using the OAG. Patients without mucosi-
port2life (P2L) PROJECT. This aims to provide access to dedicated late tis received an oral assessment and sodium bicarbonate rinse twice
effects clinics and sensitize and motivate our 1200 survivor community a day and those with mucositis received the intervention four times
to attend them. a day. The severity of mucositis was reassessed in 59 hospitalized
Methods: Then P2L clinics were set up at Cankids as well as our partner children with cancer two months after implementing the oral care
hospitals at each clinic, we issue P2L booklet to keep track of treatment intervention.
exposures and necessary investigations and treatment. Results: Nurses at the three sites found the OAG easy to use and the
Results: Till today, 130 clinics have been conducted at 5 hospitals oral care intervention easy to administer. Two months after implemen-
in 3 cities (Kolkata, Lucknow & New Delhi) in North and East India tation there was no mucositis in 30 patients (51%); and 29 patients
and 6 clinics have been held at Cankids in New Delhi. 564 sur- (49%) had moderate mucositis. No patient had severe mucositis.
vivors have been assessed and advised in these clinics. A research Conclusions: While it is evident that mucositis cannot be totally
project is currently ongoing at New Delhi with a sample size of 500 prevented during childhood cancer chemotherapy, guidelines that pro-
survivors. mote oral care hygiene can decrease severity. The oral assessment and
Conclusions: The P2L project has provided access to dedicated late intervention was implemented without difficulty in the hospital set-
effects clinics and sensitized and motivated our childhood cancer ting. Further work is needed to determine factors that most influence
survivor community to attend them. mucositis severity during childhood cancer treatment.

NURSING O163 / #401 PRE-IMPLEMENTATION PLANNING AND


STAKEHOLDER ENGAGEMENT TO DEVELOP THE PEWS
NURSING ORAL ABSTRACT PRESENTATIONS: STRATEGIES TO ADAPTATION TO SUPPORT HOSPITALS IN AFRICA/ASIA
IMPROVE QUALITY CARE 30-09-2022 10:40 AM - 12:10 PM (PASHA) PROJECT

O162 / #592 AN ORAL CARE INTERVENTION FOR Ayomide Omotola1 , Adolfo Cardenas2 , Liz Sniderman3 , Maria
PEDIATRIC ONCOLOGY HOSPITALIZED PATIENTS RECEIVING Puerto-Torres4 , Angela Carrillo-Acolén2 , Monnie Abraham5 , Sherry
CHEMOTHERAPY Johnson1 , Hilmarie Muniz-Talavera4 , Alejandra Méndez-Aceituno2,6 ,
Dora Soberanis2 , Carlos Acuña7 , Miguel Bonilla8 , Glenn Mbah
Joan Nakabiri1 , Rhahim Bank2 , Anita Ashaba1 , Joyful Beyamu3 , Bonno Afungchwi9 , Cinthia Hernández González10 , Karla
Bodietswane4 , Mary Chasela2 , Virginia Chopi2 , Andries Guerrero-Gomez11 , Elianeth Kiteni12 , Biance Rowe13 , Asya Agulnik8 ,
Gontshwanetse5 , Beatrice Gundo3 , Queen Jaffu2 , Sellina Lemon2 , Nickhill Bhakta14
Mary Mwakaghana3 , Juliet Nabatanzi1 , Peace Namanya1 , Immaculate 1 St. Jude Children’s Research hospital, Global Pediatric Medicine, Mem-
Nassanga1 , Neelo Itheetseng4 , Emma Segula2 , Pearl Semetsa4 , phis, United States of America; 2 St Jude Children’s Research Hospital,
Marilyn Hockenberry6 Global Pediatric Medicine, Memphis, United States of America; 3 Stollery
1 Global HOPE Uganda, Nursing, Kampala, Uganda; 2 Global HOPE Malawi, Children’s Hospital, Northern Alberta Children’s Cancer Care Program,
Nursing, Lilongwe, Malawi; 3 Kamuzu Central Hospital, Nursing, Lilongwe, Edmonton, Canada; 4 St. Jude Children’s Research Hospital, Department Of
Malawi; 4 Princess Marina Hospital, Nursing, Gaborone, Botswana; 5 Global Global Pediatric Medicine, Memphis, United States of America; 5 St. Jude
HOPE Botswana, Nursing, Gaborone, Botswana; 6 Baylor College of Children’s Research Hospital, Department Of Global Pediatric Medicine,
Medicine, Global Hope, Houston, United States of America Memphis, TN, United States of America; 6 National Unit of Pediatric Oncol-
ogy, UNOP, Pediatric Critical Care, Guatemala, Guatemala; 7 Hospital Dr.
Background and Aims: Mucositis severity ranges from mild, pain- Luis Calvo Mackenna, Pediatric Critical Care Unit, Santiago, Chile; 8 St.
less tissue changes to severe ulcerations that prevent oral intake and Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis,
require pain medication. Oral assessment of 80 hospitalized children United States of America; 9 Cameroon Baptist Convention Health Services,
with cancer in Uganda, Malawi, and Botswana revealed 64% had mod- Pediatria Oncology, Bamenda, Cameroon; 10 Hospital Infantil Teletón de
erate to severe mucositis. These findings led to a quality improvement Oncología, Department Of Pediatric Oncology, Querétaro, Mexico; 11 Casa
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S78 of S635 ABSTRACTS

de la Amistad para Niños con Cancer, Project Coordinator, Ciudad de Mex- Background and Aims: Failure to correctly identify hospitalized
ico, Mexico; 12 Muhimbiri National Hospital, Research And Consultancy patients results in medication errors, wrong transfusions, and mistakes
Unit, DaresSalaam, Tanzania; 13 Chris Hani Baragwanath Academic Hos- in procedures. While patient identification is required in industrialized
pital, Department Of Paediatrics And Child Health, Johannesburg, South nations, many lowmiddle income countries (LMIC) have no system for
Africa; 14 St Jude Children’s Research Hospital, Global Pediatric Medicine, hospitalized patient identification. The aim of this quality improvement
Memphis, United States of America project was to standardize the consistent use of patient identification
(ID) bands for all hospitalized children at two childhood cancer centers
Background and Aims: Hospitalized children with cancer are at high in Sub Saharan-Africa
risk for clinical deterioration. Pediatric Early Warning Systems (PEWS) Methods: A working group engaged stakeholders and established
are tools for the early identification of patients with deterioration. Pre- the procedure for patient identification upon hospital admission. The
vious work in Latin America demonstrated PEWS can be effectively standardized approach includes when to place the ID band, what infor-
implemented in diverse settings using intensive quality improvement mation to include on the band, and actions to take when patients have
coaching methods. PASHA (PEWS adaptation to support hospitals in the same name. The new procedure requires patient identification
Africa/Asia) uses implementation science methods to scale-up PEWS prior to medication administration given by any route, blood product
globally. transfusions, and before procedures (e.g., blood draw, bone marrow
Methods: A multidisciplinary team of 21 regional content experts aspiration, biopsy, lumbar puncture, surgery). ID band supplies were
was formed. This team reviewed the successful PEWS implementation obtained, and clinical staff educated on the patient ID band process.
strategy used in Latin America to identify the main components and Documentation guidelines outline how to record patient ID band con-
necessary adaptions. Distance learning platforms were incorporated, firmation on the medical record. A REDCap database was created, and
and PEWS impact research was decoupled from implementation out- a group was trained to collect and enter data
comes and strategies using co-design methods. The multidisciplinary Results: Training on patient ID band use was completed with 43 health
team formed four working groups to develop the onboarding process, care providers and data collection was successful using REDCap at
training methods and logistics, outcome measures, and syllabus. both sites. A month after training 81% of 74 hospitalized patients had
Results: Over six months, the multidisciplinary team met for 20 work- an ID band in place. Documentation of ID band use before medications
ing sessions and identified opportunities for creating a hybrid virtual was found in 65% of the patients
training structure that included both online content and live sessions. Conclusions: Within a month of implementation, most hospitalized
An onboarding process for participants and a curated curriculum which patients had ID bands in place, supporting a standardized approach for
included 22 presentations, 17 live meetings, and 15 assignments was patient identification in LMICs. Findings confirm the feasibility of ID
developed. Balancing, process, and outcomes measures that will pro- band use and the need for continued education on the importance of
vide relevant data for measuring the effect of PEWS implementation patient identification. Careful assessment will ensure proper patient
were also identified. A Pilot cohort of five pediatric cancer centers identification prior to any intervention and provide safer care with
is currently underway to be trained sequentially over 18 months. fewer errors.
Regional stakeholders from Asia and Africa were involved in adapta-
tion decisions during the co-design phase to maintain a project focus
centered on the centers we aim to support in subsequent phases. O165 / #1909 SYMPTOM SCREENING AND ASSESSMENT IN
Conclusions: Dissemination of successful implementation strategies CHILDREN AND YOUNG PEOPLE’S CANCER CARE: A SURVEY
from a regional to a global scale requires adaptation to address dif- OF PRACTICE IN THE UNITED KINGDOM
ferent cultural contexts, variable resources available, and time zones.
A multidisciplinary approach involving stakeholders can facilitate this Faith Gibson1,2 , Gemma Bryan1 , Jess Morgan3 , Bob Phillips4 , Susie
process. Aldiss1
1 University of Surrey, School Of Health Sciences, Guildford, United King-
dom; 2 Great Ormond Street Hospital for Children, Centre For Outcomes
O164 / #829 PATIENT IDENTIFICATION IN HOSPITALIZED And Experience Research In Children’s Health, Illness And Disability (orchid),
CHILDREN WITH CANCER IN SUB-SAHARAN AFRICA (SSA) London, United Kingdom; 3 Leeds Teaching Hospitals NHS Trust, Depart-
ment Of Paediatric Haematology & Oncology, Leeds, United Kingdom;
Grace Chirwa1 , Aisha Nedege2 , Tadala Mulemba1 , Chikondi 4 University of York, Paediatric Oncology, Yorkshire, United Kingdom

Chodzaza1 , Joshi Evelyne2 , Clara Kapindula1 , Martha Kataika1 ,


Mayimoona Kiyemba2 , Hellen Mbutuka1 , Stuart Mumba1 , Immaculate Background and Aims: Children and young people (CYP) with cancer
Nassanga2 , Memory Sabantini2 , Marilyn Hockenberry3 experience complex physical, psychosocial and behavioural symptoms.
1 Baylor College of Medicine Children’s Foundation, Global Hope, Lilongwe, They may accept experiencing these, only seeking help when symp-
Malawi; 2 Global HOPE Uganda, Nursing, Kampala, Uganda; 3 Baylor College toms become particularly severe. The use of a standardised symptom
of Medicine, Global Hope, Houston, United States of America reporting process can normalise and encourage symptom report-
ing. Self-reported measures are the gold standard and a variety of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S79 of S635

instruments are available for use. As part of a wider study, we under- Methods: A cross-sectional study design was employed using conve-
took an examination of the context of care prior to implementing a nience sampling technique to nurses working at pediatric oncology
patient-reported outcome measure in two sites in the United Kingdom unit, UCI. A self-administered questionnaire composed of open and
(UK). closed ended question was used to collect data. 5 point Likert scale (1 =
Methods: A survey of UK Children’s Cancer and Leukaemia Group strongly disagree and 5 = strongly agree) was used and data analyzed
members was conducted to investigate current practice relating to using SPSS.
assessment of symptoms and use of symptom screening/assessment Results: Fourteen nurses (14.3% male, 85.7% females) participated
instruments. The multi-disciplinary research team developed and then in this study where 79% strongly agreed that fertility preservation
piloted the survey. Members received a link to the Qualtrics survey via is an essential component in the care of children and adolescents on
email. cancer treatment. 57% strongly disagreed that fertility preservation
Results: Forty-three professionals responded (44% doctors, 34% facilities are easy to locate in Uganda. Furthermore, 50% nurses dis-
nurses, 22% allied health professionals/other). Formal symptom agreed that fertility preservation is part of nursing scope of practice
assessments were conducted by 84%. The most frequently used ‘tool’ and 57% nurses strongly disagreed that patient’s education on fertility
was the Wong-Baker FACES Scale (n=23) followed by the Common preservation being readily available.
Terminology Criteria for Adverse Events (n=18), and Pediatric Quality Conclusions: Nurses have positive perception and attitude toward fer-
of Life Inventory including subscales (n=14). The Children’s Interna- tility preservation to children and adolescents on cancer treatment,
tional Mucositis Evaluation scale was the most frequently reported however it needs to be incorporated in nursing scope of practice and
modified instrument, only one respondent used the original version. make readily available patient’s education resources on the fertility
Thirty-six (84%) respondents felt that symptoms were missed; mostly preservation in facilities
psychological symptoms, followed by pain and nausea.
Conclusions: Respondents did not seem to distinguish between ’tools’
used as part of a clinical trial and those used in everyday practice. The O167 / #164 CREATING AND ADAPTING AN INFECTION
most frequently reported instruments were simple measures, without MANAGEMENT CARE PATHWAY IN PEDIATRIC ONCOLOGY
need for calculation. Some institutions used local modifications of pre-
viously validated instruments; it is unclear why this is, and whether Deborah Tomlinson1 , Paula Robinson2 , Paul Gibson3 , Melissa
these instruments have been re-validated. The use of patient-reported Beauchemin4 , Allison Grimes5 , Grace Dadzie1 , Mark Mairs1 , Erin
outcomes in symptom screening/assessment does not appear to be Plenert1 , Emily Vettese1 , Stephanie Cox3 , Lee Dupuis1
common practice in children’s cancer care in the UK. 1 Hospital for Sick Children, Child Health Evaluative Sciences, Toronto,
Canada; 2 Pediatric Oncology Group of Ontario, Pogo, Toronto, Canada;
3 McMaster Children’s Hospital, Division Of Haematology/oncology, Hamil-
O166 / #1427 NURSES’ PERCEPTION AND ATTITUDE ton, Canada; 4 Columbia University/Herbert Irving Cancer Center, Pediatric
TOWARDS FERTILITY PRESERVATION IN CHILDREN AND Oncology, New York, United States of America; 5 University of Texas Health
ADOLESCENTS UNDERGOING CANCER TREATMENT AT Science Center at San Antonio, Division Of Pediatric Hematology Oncology,
UGANDA CANCER INSTITUTE (UCI) San Antonio, United States of America

Rose Nankinga1 , Isaac Mulyowa1 , Anthony Kayiira2 , Elianeth Kiteni3 Background and Aims: Recent studies have described an important
1 Uganda Cancer Institute, Pediatric Oncology Clinic, Kampala, Uganda; gap between the care patients should receive and the care they actually
2 Uganda Cancer Institute, Gynaecology, Kampala, Uganda; 3 Muhimbiri receive. Utilization of clinical practice guidelines (CPGs) is a key com-
National Hospital, Nursing, DaresSalaam, Tanzania ponent of high-quality care, and CPG consistent care is associated with
better patient outcomes, greater health care professional satisfaction
Background and Aims: According to GLOBOCAN 2018, it’s estimated and lower costs. Implementation of CPGs is challenging and the devel-
that, 3000 children under 18 years develop cancer annually, with over- opment of care pathways is one approach to facilitate CPG-consistent
all survival rate of 55% in Uganda. These young survivors are at risk care. However, little is known about optimal approaches to develop-
of developing long term cancer treatment side effects particularly in ment and adaptation in pediatric oncology. Our objectives were to
reproductive system such as amenorrhea, premature menopause, sub- develop care pathway templates for pediatric cancer supportive care
fertility in females and testicular failure in males. There’s inadequate that are based upon CPGs and to adapt an infection management care
awareness and policies in Uganda on cancer-related morbidity and lit- pathway for use at a single institution.
tle is known or documented about nurses’ perceptions and attitudes Methods: Study phases were: (1) Creation of care pathway templates
towards fertility preservation in children and adolescents undergoing across multiple supportive care topics; (2) Refinement of the infection
cancer treatment at UCI. Main aim of the study was to assess nurses’ management care pathway template by interviewing pediatric oncol-
perception and attitudes towards fertility preservation in children and ogy clinicians at a single institution; and (3) Adaptation of the infection
adolescents undergoing cancer treatment at UCI. management care pathway template for use at a different institution.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S80 of S635 ABSTRACTS

Results: Informed by seven CPGs, an initial iteration of the infec- tiation of antibiotics and imaging. Reducing HARI may lead to less
tion management care pathway template was created. This template antibiotic use and avoidance of additional imaging. Ongoing monitor-
was then refined based upon 20 interviews with pediatric oncol- ing of HARI continues to elucidate specific isolation measures that are
ogy clinicians. Adaptation of the infection management care pathway successful in reduction of HARI.
template for use at a different institution required many changes to
improve its clinical useability. Specificity and additional information not
considered by the source CPGs were incorporated. FREE PAPER SESSION (FPS)
Conclusions: We developed a process to create care pathway tem-
plates across multiple supportive care topics in pediatric oncology, and FPS 09: BRAIN TUMORS - FROM BASIC SCIENCES TO HEALTH
to refine and adapt the infection management care pathway. While we SERVICES UTILIZATION 30-09-2022 10:40 AM - 12:10 PM
found that the process was feasible, we also identified the need to sub-
stantially modify the care pathway during the adaptation process to O169 / #1761 ONCOGENIC DEPENDENCY OF PEDIATRIC
consider scenarios not addressed by the source CPGs. Future work EPENDYMOMAS ON EXTRACELLULAR VESICLE PATHWAYS
should measure implementation success.
Kendra K. Maass1,2 , Mieke Roosen3 , Torsten Mueller4 , Daniel
Senfter1,5 , Julia Benzel1,2 , Tatjana Wedig1,2 , Jeroen Krijgsveld4 , Stefan
O168 / #1901 DECREASE IN RESPIRATORY INFECTIONS ON Pfister6,7,8 , Kristian Pajtler1,2
PEDIATRIC ONCOLOGY WARD DURING COVID PANDEMIC 1 Hopp Children’s Cancer Center Heidelberg, Pediatric Neurooncology, Hei-
MAY HAVE REDUCED USE OF ANTIBIOTICS delberg, Germany; 2 Heidelberg University Hospital, Pediatric Hematology
And Oncology, Heidelberg, Germany; 3 Princess Máxima Center for Pediatric
Shelby Smith1 , Regan Trappler2 , Xiaoyan Song2 , Birte Wistinghausen1 Oncology, Kool Group, Utrecht, Netherlands; 4 DKFZ Heidelberg, Division
1 Children’s National Hospital, Center For Cancer And Blood Disorders, Of Proteomics Stem Cells And Cancer, Heidelberg, Germany; 5 Medical Uni-
Washington, United States of America; 2 Children"s National Hospital, versity of Vienna, Department Of Pediatrics, Vienna, Austria; 6 Heidelberg
Infection Control, Washington, United States of America University Hospital, Heidelberg, Germany, Pediatric Oncology, Hematology,
And Immunology, Center For Child And Adolescent Medicine, Heidelberg,
Background and Aims: Pediatric oncology and hematopoietic stem Germany; 7 Hopp Children’s Cancer Center, (kitz), Heidelberg, Germany, Hei-
cell (HSCT) patients are at increased risk for bacterial infections delberg, Germany; 8 German Cancer Research Center (DKFZ) and German
requiring prompt initiation of antibiotics with fever. Respiratory viral Cancer Consortium (DKTK), Heidelberg, Germany, Pediatric Neurooncology,
infections may lead to fever, invasive systemic infections and/or delay Heidelberg, Germany
in neutrophil recovery leading to prolonged hospitalizations. Our
hospital has reverse isolation policies according to risk of serious infec- Background and Aims: The majority of pediatric ependymoma (EPN)
tions. During the COVID pandemic, these protocols were expanded to comprise either supratentorial EPN characterized by ZFTA fusions (ST-
include universal masking and reduction in and enhanced screening of EPN-ZFTA) or posterior fossa group A EPN (PF-EPN-A), for both of
visitors, in addition to the expanded society wide restrictions. which only limited therapeutic options are available. Because pediatric
Methods: Hospital acquired respiratory infections (HARI) on the EPNs have a relatively low mutational burden, identification and char-
pediatric oncology/HSCT ward were monitored from 2018-2021. Res- acterization of tumor-associated pathways and molecular processes
piratory infections present on admission or manifesting within the are of critical importance to reveal potential therapeutic targets. Data
incubation period from date of admission were excluded. Collection from previous transcriptional studies and a cross-species in vivo screen
of additional data on patients with HARI included: blood cultures implied aberrant vesicular pathways in ST-EPN-ZFTA, prompting fur-
obtained, antibiotics initiated, imaging obtained. ther investigation of their putative role in EPN pathogenesis.
Results: There was a reduction in HARI during the COVID epidemic Methods: We investigated EPN group-specific differences in extracel-
from an average of 3.25 infections/quarter in 2018-2019 to an average lular vesicle (EV) biogenesis pathways in human EPN transcriptome
of 1 infection/quarter. This difference remained considering a reduc- and proteome datasets. In addition, we characterized isolated EPN EVs
tion of average census from 30.2 patients/day pre COVID to 26.4 by mass spectrometry. EPN-specific EV cargo was further investigated
patients/day post COVID, with 0.12 HARI/patient/day pre COVID to by immunofluorescence staining and western blotting. This enhanced
0.03 HARI/patient/day post COVID. There was no difference in per- understanding of EPN vesicular signaling allowed for a pre-selection
centage of patients with HARI who had blood cultures or chest x-rays of inhibitors targeting specific EV biogenesis pathways. In vitro prolif-
obtained or antibiotics initiated pre- and post COVID. Over the entire eration and invasion assays as well as in vivo treatment studies were
period, the majority of patients had blood cultures drawn (66.7%) and performed on EPN model systems.
antibiotics started (62.9%). Only 1 blood culture returned a positive Results: Integration of multi-omic data from both EPN tissues and
result. 38.9% had chest x-rays obtained. the EPN EV-associated proteome led to identification of an ST-EPN-
Conclusions: A reduction of HARI was seen on a pediatric oncology ZFTA-specific EV population. We could spatially map specific EV mark-
ward during COVID. HARI increase medical interventions such as ini- ers to the perivascular niche that primarily harbors undifferentiated
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S81 of S635

ST-EPN-ZFTA cell populations. Targeting lipid metabolism pathways of Moscow, Russian Federation; 2 Dmitry Rogachev National Medical Research
EVs reduced the abundance of released EVs from ST-EPN-ZFTA result- Center of Pediatric Hematology, Oncology and Immunology, Laboratory
ing in altered growth behavior and decreased invasion of tumor cells in Of Molecular Oncology, Moscow, Russian Federation; 3 Dmitry Rogachev
vitro. In vivo validation of EV release inhibitors in orthotopic ST-EPN- National Medical Research Center Of Pediatric Hematology, Oncology and
ZFTA PDX models significantly reduced tumor growth and increased Immunology, Deputy Director General For Scientific And Clinical Work,
survival. Head Of The Department Of Hematology, Medical Director, Moscow,
Conclusions: In summary, we have leveraged ST-EPN-ZFTA-specific Russian Federation; 4 Children’s National Hospital, Division Of Oncology,
lipid metabolism pathways of EVs as a potential therapeutic vulnerabil- Washington, United States of America
ity. Further mechanistic investigations on EPN EV biogenesis, release,
or uptake are expected to improve our understanding of the cross-talk Background and Aims: Patients with infant midline gliomas have an
between tumor cells and cells of the microenvironment and may lead aggressive course, and conventional chemotherapy is not effective in
to potential new therapeutic avenues. many cases. We analyzed an infant (< 1y/o), sporadic low-grade glioma
cohort consisting of 21 patients diagnosed at Dmitry Rogachev Center
(Moscow, Russia) between 2014 and 2021.
O170 / #1498 IMMUNOREGULATORY CYTOKINES IN THE Methods: Next-generation sequencing was performed to detect drug-
CEREBROSPINAL FLUID OF PATIENTS WITH DIFFUSE gable genetic abberations.
INTRINSIC PONTINE GLIOMA (DIPG) Results: Median age of diagnosis was 8.2 months (range: 2 to 12
months). Eighteen were pilocytic astrocytomas (PAs) and 3 desmoplas-
Merce Baulenas-Farres, Sonia Paco, Angel Carcaboso tic infantile gangliogliomas (DIGs), located in the optico-hypothalamic
Hospital Sant Joan de Deu, Pediatric Oncology, Barcelona, Spain (n=17), hemispheric (n=3), or cerebellar (n=1) regions. Median follow
up time was 2.7 years with 5 -year OS was 91%, 5-year PFS was 34%.
Background and Aims: The microenvironment of DIPG is immunosup- Seventeen of 18 (94%) PAs arose in the optico-hypothalamic region;
pressive. In this work, we aimed to identify tumor-secreted cytokines eight (47%) of these patients had initial metastases, and nine (53%) had
in DIPG and to study their presence in cerebrospinal fluid (CSF) of severe diencephalic syndrome. The most common genetic alteration
patients. detected was KIAA1549-BRAF exon 15-exon 9 fusion (n=9), while
Methods: We used an array kit to perform immunodetection of 105 other KIAA1549-BRAF exonic breakpoints were less common (n=4).
human cytokines in DIPG autopsy or biopsy tissue from 11 patients, BRAF V600E mutation was present in 3 patients and a MEF2D-NTRK1
brainstem tissue from four autopsies of patients without tumors, and fusion in one. Ten patients with PA received targeted therapy for pro-
supernatant from seven DIPG cell models in culture. We used Enzyme- gressive disease after chemotherapy: 8 patients with KIAA1549-BRAF
Linked Immunosorbent Assays (ELISA) to determine the concentration fusion with MEK-inhibitor (trametinib), 2 patients with BRAF V600E
of the top-secreted cytokines in CSF obtained from patients with DIPG received BRAF-inhibitors (one child received vemurafenib, another
and control patients without brain tumors. dabrafenib+trametinib). Median treatment time of trametinib was 8
Results: We identified osteopontin and chitinase-3-like 1 as the top- months. Six patients continue on trametinib therapy (PR – 3, SD-3),
secreted proteins in DIPG tissues and cell models. Median osteopontin 2 patients stopped trametinib therapy (1 due to toxicity, 1 due to PD
concentration in CSF of DIPG patients was 152 ng/mL (range 59-299; after 18 months of treatment). One patient with BRAF V600E died of
n = 13), significantly higher than the one of controls, 23 ng/mL (range disease 3 months after the end of vemurafenib; another patient with
0.8-55; n = 12) (P < 0.0001). Median chitinase-3-like 1 concentration BRAF V600E has SD on combine BRAF- and MEK inhibitors therapy. All
in CSF was 100 ng/mL (range 48-270; n=12), also higher than controls, children with DIGs are presented in complete remission after surgery.
19 ng/mL (range 5-130; n=12) (P < 0.0001). Conclusions: Targeted therapy may be an effective treatment option,
Conclusions: We found high levels of the immunoregulatory cytokines but it is at times restricted by toxicity and development of resistance.
osteopontin and chitinase-3-like 1 in the CSF of patients with DIPG.
These findings could have implications in the fields of treatment and
biomarkers. O172 / #1517 CLINICAL UTILITY OF A TIERED APPROACH
IN THE MOLECULAR CHARACTERIZATION OF CHILDREN AND
YOUNG ADULTS WITH GLIOMAS
O171 / #1302 INFANT LOW-GRADE GLIOMAS: MOLECULAR
CHARACTERISTICS AND RESULTS OF TARGETED THERAPY Rawan Hammad1,2 , Liana Nobre3 , Scott Ryall4,5 , Anthony Arnoldo6 ,
Robert Siddaway4,5 , Uri Tabori3,5,7 , Cynthia Hawkins4,5,8
Ludmila Papusha1 , Andge Valiakhmetova1 , Margarita Zaytseva2 , 1 The Hospital for Sick Children, Pediatric Heamatology Oncology, Toronto,
Alexander Druy2 , Ekaterina Salnikova1 , Irina Vilesova1 , Galina Canada; 2 King Abdulaziz University, Heamtology Department, Jeddah,
Novichkova3 , Eugene I Hwang4 , Roger J Packer4 Saudi Arabia; 3 The Hospital for Sick Children, Hematology Oncology,
1 Dmitry Rogachev National Medical Research Center Of Pediatric Hema- Toronto, Canada; 4 The Hospital for Sick Children, Division Of Pathology,
tology, Oncology and Immunology, Department Of Pediatric Neurooncology, Toronto, Canada; 5 The Hospital for Sick Children, Arthur And Sonia Labatt
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S82 of S635 ABSTRACTS

Brain Tumour Research Centre, Toronto, Canada; 6 The Hospital for Sick Chil- countries. Sixty two percent of patients with CNS tumours needed
dren, Division Of Pathology, Department Of Pediatric Laboratory Medicine, to migrate for treatment between 2012-2019. Although overall sur-
Toronto, Canada; 7 University of Toronto, Department Of Medical Bio- vival of brain tumors has improved over the years, it is still lower than
physics, Toronto, Canada; 8 University of Toronto, Department Of Laboratory the developed world. New strategies are urgently needed to improve
Medicine And Pathobiology, Toronto, Canada outcomes. Objective: To implement a network and remote communi-
cation strategy for early referral, accurate diagnosis and staging, and
Background and Aims: Molecular characterization is key to the diag- adequate treatment of children with brain tumors between Garrahan
nosis and management of pediatric brain tumors. As genomic analysis Hospital and other centers in Argentina.
is complex and expensive, we tested the utility of a tiered testing Methods: This is a prospective qualitative-quantitative study, with
approach to comprehensively characterize pediatric gliomas in a cost- interventions and registration of times of compliance. Biweekly (twice
and time-sensitive manner. per month) interactive multidisciplinary meetings were established
Methods: We used a tiered testing pipeline of immunohistochemistry, between private/public medical centers across the country and Hospi-
customized gene fusion panels, SNP arrays and targeted RNA sequenc- tal Garrahan during 2021.
ing in gliomas over one year. We then analyzed a larger cohort of Results: The active participation of private and public centers of
childhood and adolescent and young adult (AYA) gliomas to determine approximately 70% of the country was achieved; being connected up
the added benefit of targeted RNA sequencing in uncovering clinically to 40 working groups simultaneously. Sixty-seven new CNS patients
actionable alterations. from 15 provinces (90.8% of the country’s population) were discussed
Results: The tiered approach successfully characterized 94.2% (49/52) in a multidisciplinary manner. Only 14 patients required a referral to
of gliomas diagnosed over a one-year period. For 44 low-grade gliomas, a center of high complexity; 50% due to radiotherapy needs and 50%
IHC, fusion panel, or targeted RNAseq solved 32%, 30%, and 33% of for surgical issues. Sixty two CNS MRI were reviewed in Hospital Gar-
cases, respectively. 39% low grade gliomas required targeted RNAseq rahan, improving staging and oncological management. Twenty seven
analysis after negative IHC and fusion panel. Of 8 high-grade glioma tumor samples required a pathology analysis in Hospital Garrahan;
samples, 5 were solved by IHC and the remaining 3 by targeted 7.4% for molecular workup, 22.2% for initial diagnosis and 70.4% for
RNAseq. 4 of 8 had a targetable molecular event detected. Of 233 second review (changing the initial diagnosis by 15% ).
glioma samples from pediatric and AYA patients tested with targeted Conclusions: The exponential benefit of this strategy was established,
RNAseq 79.8% had diagnostic mutations detected of which 69.5% showing to be an useful and fundamental tool to be implemented
were targetable alterations. as a National health policy. The success of the implementation was
Conclusions: Our tiered approach molecularly characterized 94.2% of marked by acceptance, appropriation, feasibility, adherence, coverage
samples in a cost-and time-efficient manner. Such an approach may and sustainability.
be feasible in most neuro-oncology centers worldwide. Moreover, the
addition of targeted RNA seq in specific scenarios is a valuable higher
tier test to detect clinically actionable alterations. O174 / #752 DEVELOPMENT OF A PROTOCOL FOR
PEDIATRIC SURGICAL NEURO-ONCOLOGY IN AWAKE
PATIENTS
O173 / #1168 CLOSING THE GAP: NATIONAL ARGETINIAN
DISCUSSION FORUM ON PEDIATRIC BRAIN TUMOURS Carlos Almeida Junior1 , Alessandra Antoniazzi2 , Bruna Mançano3 ,
Lucas Lourenço3 , Maristela Facholi3 , Rui Reis4
Lorena Baroni1 , Nicolás Fernández Ponce2 , Candela Freytes2 , 1 Barretos Cancer Hospital, Pediatric Neurosurgery, Barretos, Brazil;
Francisco R. Maldonado3 , Natalia Pinto4 , Javier Gonzalez Ramos5 , 2 Barretos’s Children and Young Adults Cancer Hospital, Anesthesiology,
Fabiana Lubieniecki6 , Daniel Alderete1 Barretos, Brazil; 3 Barretos Cancer Hospital, Pediatric Oncology, Barretos,
1 Paediatric Hospital Dr Juan P Garrahan, Hematology Oncology, Buenos Brazil; 4 Barretos Cancer Hospital, Molecular Oncology Research Center,
Aires, Argentina; 2 Paediatric Hospital Dr Juan P Garrahan, Hematology Barretos, Brazil
Oncology, Ciudad Autónoma de Buenos Aires, Argentina; 3 Paediatric Hospi-
tal Dr Juan P Garrahan, Diagnostic Imaging, Ciudad Autónoma de Buenos Background and Aims: To reduce risks, we aimed to develop a protocol
Aires, Argentina; 4 Paediatric Hospital Dr Juan P Garrahan, Radiotherapy, with the necessary recommendations to assist the entire multidis-
Ciudad Autónoma de Buenos Aires, Argentina; 5 Paediatric Hospital Dr Juan ciplinary team involved in awake craniotomy in a pediatric patient,
P Garrahan, Neurosurgery, Buenos Aires, Argentina; 6 Hospital de Pediatria including preoperative preparation, eligibility criteria, and intraoper-
JP Garrahan, Pathology, Buenos Aires, Argentina ative and postoperative management, to ensure the safety of the
procedure and disseminate the technique so that more children can
Background and Aims: Introduction: The incidence of pediatric CNS benefit.
tumours in Argentina is 258 new cases (26 cases per million children) Methods: The ambispective observational study included patients
per year. About 50% of them receives treatment at Hospital Garra- between 7 to 25 years of age with central nervous system tumors
han; most coming from other provinces, as well as other latinoamerican located in eloquent areas of the brain undergoing treatment and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S83 of S635

follow-up at the Barretos Children’s Cancer Hospital. The patient sam- into vision impairment, hearing, cardiac, pulmonary, musculoskeletal,
ple was convenient because this is a rare pediatric surgical procedure. neurological, endocrine, renal or digestive conditions. We used ordered
The awake craniotomy protocol was elaborated by defining eligibil- logistic regression models to identify determinants of a more negative
ity criteria in 4 distinct multi-professional performance stages, which body image.
were preoperative, intra-operative, postoperative, and follow-up. Results: Our study included 504 survivors (48% female) with a mean
Results: A total of 6 patients were included in the study and under- age at study of 17.7 years and 136 siblings. Survivors and siblings
went satisfactory pre-operative preparation and awake craniotomy reported comparable levels of body image (all p>0.05). Among sur-
from May/2018 to June/2021. The patients’ median age (minimum and vivors, a more negative body image was associated with female gender,
maximum) was 14.5 (9.0 - 24.0) years of age. Four patients were male, bone marrow transplantation, and cancer-related chronic health con-
and two were female. Histopathologic results included pilocytic astro- ditions. Survivors with ≥2 chronic health conditions were less satisfied
cytoma, high-grade glioma, gangliogliomas, and two dysembrioplastic with their body image (OR=1.9; 95%-CI: 1.3-2.8) and more often
neuroepithelial tumors. There was no need for conversion to general would like to change things regarding their body (OR=1.6; 95%-CI:
anesthesia, and no patient experienced anesthetic complications. Com- 1.0-2.4) compared to survivors without such conditions. Survivors with
plete brain mapping was successfully performed on all six patients in the poorest body image where those suffering from musculoskeletal,
the study. endocrine, renal, or digestive conditions.
Conclusions: The pediatric awake craniotomy protocol includes eligi- Conclusions: The body image of adolescent survivors of childhood can-
bility criteria and perioperative management of the pediatric popula- cer was overall comparable to healthy siblings, but survivors suffering
tion that seems safe and effective. Furthermore, awake craniotomy has from chronic health conditions may require additional psycho-social
demonstrable high satisfaction, increased survival rate, and improved support to reduce body image concerns.
quality of life for the patient.

O176 / #738 SYMPTOM PHENOTYPES, PHYSICAL


FREE PAPER SESSION (FPS) INACTIVITY AND SMOKING AMONG LONG-TERM SURVIVORS
OF THE CHILDHOOD CANCER SURVIVOR STUDY (CCSS)
FPS 10: PSYCHO-ONCOLOGY AND SURVIVORSHIP 30-09-2022
10:40 AM - 12:10 PM Rachel Tillery Webster1 , Wei Liu2 , Meghan Mcgrady3 , Nicole
Alberts4 , Tara Brinkman5 , Kirsten Ness5 , Bernard Fuemmeler6 , Alicia
O175 / #175 BODY IMAGE IN ADOLESCENT SURVIVORS OF Kunin-Batson7 , Deokumar Srivastava8 , I-Chan Huang9 , Gregory
CHILDHOOD CANCER: THE ROLE OF CHRONIC HEALTH Armstrong9 , Rebecca Howell10 , Daniel Green11 , Yutaka Yasui5 , Kevin
CONDITIONS Krull12
1 St. Jude Children’s Research Hospital, Psychology, Memphis, United States

Luzius Mader1 , Tomáš Sláma1 , Tamara Diesch-Furlanetto2 , Mutlu of America; 2 St. Jude Children’s Research Hospital, Biostatistics, Memphis,
Kartal-Kaess3 , Fabiën Belle1 , Claudia Kuehni1 United States of America; 3 Cincinnati Children’s Hospital Medical Cen-
1 University of Bern, Institute Of Social And Preventive Medicine, Bern, ter, Division Of Behavioral Medicine And Clinical Psychology, Cincinnati,
Switzerland; 2 University Children’s Hospital Basel, University of Basel, Divi- United States of America; 4 Concordia University, Psychology, Montreal,
sion Of Paediatric Oncology/ Haematology, Basel, Switzerland; 3 Inselspital, Canada; 5 ST. JUDE CHILDREN’S RESEARCH, Epidemiology And Cancer
University Hospital, University of Bern, Division Of Paediatric Haematology Control, Memphis, United States of America; 6 Virginia Common Wealth
& Oncology, Department Of Paediatrics, Bern, Switzerland University, Department Of Health Behavior And Policy, Richmond, United
States of America; 7 University of Minnesota, Pediatrics, Minneapolis, United
Background and Aims: Cancer diagnosis and its treatment may impair States of America; 8 ST. JUDE CHILDREN’S RESEARCH, Biostatistics, Mem-
the long-term body image of childhood cancer survivors. This may phis, United States of America; 9 St. Jude Children’s Research Hospital,
be particularly relevant in adolescence, a critical period of psycho- Epidemiology And Cancer Control, Memphis, United States of America;
social development. We compared the body image between adolescent 10 The University of Texas at MD Anderson Cancer Center, Radiation Physics,

cancer survivors and their siblings, determined socio-demographic Houston, United States of America; 11 St. Jude Children’s Research Hos-
and clinical factors associated with body image, and investigated the pital, Oncology, Memphis, United States of America; 12 St. Jude Children’s
association with cancer-related chronic health conditions. Research Hospital, Epidemiology And Cancer Control, Psychology, Memphis,
Methods: As part of the nationwide Swiss Childhood Cancer Survivor United States of America
Study, we sent a questionnaire to all adolescent survivors (aged 16-19
years) registered in the Swiss Childhood Cancer Registry, who sur- Background and Aims: The aims of this study were to examine phys-
vived >5 years, and were diagnosed with cancer between 1976-2010. ical and psychological symptom burden patterns among childhood
Siblings received the same questionnaire. We assessed the level of cancer survivors and their associations with risky health behaviors.
agreement with three statements related to body image on a 4-point Methods: CCSS participants (N=17,231; Mean age=27.4 [5.98]; 79%
Likert scale. Self-reported chronic health conditions were classified non-Hispanic white; 48% female) self-reported sensory, motor, cardiac,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S84 of S635 ABSTRACTS

respiratory, pain, gastrointestinal, fatigue, memory, depression, and Medical Center, Department Of Internal Medicine, Section Endocrinol-
anxiety symptoms at baseline; latent class analysis identified symptom- ogy, Rotterdam, Netherlands; 9 Willem Alexander Children’s Hospital/Leiden
pattern classes. Self-reported smoking status (current, former, never) University Medical Center, Department Of Pediatrics, Leiden, Netherlands;
and physical activity (Inactive: metabolic equivalents [METs]<450; 10 Leiden University Medical Center, Department Of Pediatrics, Leiden,
Active: METs>=450) were assessed a median of 6.3 years after Netherlands
baseline. Logistic regression examined associations between symptom-
pattern class membership and health behaviors at follow-up, adjusting Background and Aims: We aimed to study a wide range of psy-
for sex, age, health insurance, and global chronic health conditions chosocial outcomes in adult long-term survivors of childhood
(CHCs) at baseline. cancer (CCS) and to identify socio-demographic and medical risk
Results: Five classes of symptom patterns were identified: 1) elevated factors.
across all symptoms (Global; 7.7%); 2) emotional distress and pain Methods: CCS from the Dutch Childhood Cancer Survivor Study
(Distress-Pain; 13.3%); 3) neurologic and pain (Neuro-Pain; 10.6%); (DCCSS) LATER cohort (1963-2001) part 2 (attained age ≥18, diag-
4) cardiopulmonary and pain (Cardio-Pain; 5.3%); 5) non-elevated nosed <18, ≥5 years since diagnosis) completed the Rosenberg Self-
symptoms (Norm; 63.1%). Compared to the Norm class, almost all Esteem Scale, Distress Thermometer, Hospital Anxiety and Depression
other classes were associated with greater risk for physical inactiv- Scale, Self-Rating Scale for Post-Traumatic Stress Disorder and the SF-
ity (Cardio-Pain, OR=1.18 [1.07,1.31]; Neuro-Pain, OR=1.16 [1.07, 36 (HRQOL). CCS’ scores were compared to general population-based
1.25]; Global, OR= 1.11 [1.01, 1.23]), and being a current smoker references using analysis of variance and logistic regression analysis,
(Distress-Pain, OR=1.81 [1.54, 2.13]; Cardio-Pain, OR=1.91 [1.49, controlling for age and sex (p<.05). Risk factors for worse psychosocial
2.46]; Neuro-Pain, OR =1.35 [1.11, 1.66]; Global, OR =3.25 [2.67, 3.96]. outcomes were assessed with regression analyses (p<.05).
These classes were also associated with higher likelihood of being a Results: CCS (N=1797, response rate 72.3%, mean age 35.4 years, 49%
former smoker. female, ≥15 years since diagnosis) completed the questionnaires. The
Conclusions: Adjusting for insurance, CHCs, age, and sex, self- percentage of CCS who reported PTSD as a result of childhood cancer
reporting of physical and psychological symptoms at baseline were was 2.9%, and 36.6% of CCS experienced clinical distress. No differ-
associated with future inactivity, previous smoking behaviors, and ences were found between CCS and references on self-esteem and
future smoking behaviors among CCSS participants. Ongoing symptom anxiety. CCS were less depressed than references (d=-.25), but scored
burden may be preventing survivors from engaging in health optimizing worse on the HRQOL scales, except for bodily pain (.01≤d≥-.36).
behaviors or alternatively risky health behaviors may be used to cope Female sex, lower educational attainment, not being in a relation-
with symptom burden (e.g., smoking to reduce distress). Of note, pain ship and being unemployed were associated to almost all psychosocial
was prevalent in all elevated symptom-pattern classes, which suggests outcomes. Of medical characteristics related to childhood cancer,
an important intervention target for improving health behaviors. few (CNS cancers, bone tumours, unspecified/other malignancies, and
radiotherapy in certain areas) were associated to some psychosocial
outcomes.
O177 / #1270 PSYCHOSOCIAL OUTCOMES IN LONG-TERM Conclusions: Overall long-term survivors who have been diagnosed
DUTCH ADULT SURVIVORS OF CHILDHOOD CANCER: THE ≥15 years ago show to be resilient, and report a psychosocial func-
DCCSS-LATER 2 PSYCHO-ONCOLOGY STUDY tioning comparable to the general population with similar clinical
distress, self-esteem and anxiety, and slightly less depression and lower
Anne Maas1 , Heleen Maurice-Stam1 , Leontien Kremer1,2 , Eline Van HRQOL. Other than socio-demographic characteristics and CNS can-
Dulmen-Den Broeder3 , Wim Tissing1,4 , Jacqueline Loonen5 , Helena cers, no clear pattern of impact from medical factors was observed.
Van Der Pal1 , Andrica De Vries1,6 , Marry Van Den Heuvel-Eibrink1,6 , Future studies should focus on other factors in explaining psychosocial
Cecile Ronckers1,7 , Sebastian Neggers1,8 , Dorine Bresters1,9 , Marloes functioning in CCS, such as coping and CCS’ current medical condition.
Louwerens10 , Margriet Van Der Heiden-Van Der Loo1 , Marloes Van
Gorp1 , Martha A. Grootenhuis1
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, O178 / #1403 PSYCHIATRIC DISORDERS IN CHILDHOOD
Utrecht, Netherlands; 2 Emma Children’s Hospital, Amsterdam University CANCER SURVIVORS IN DENMARK, FINLAND AND SWEDEN: A
Medical Center, Vrije Universiteit Amsterdam, Pediatric Oncology, Ams- REGISTER-BASED COHORT STUDY FROM THE SALICCS
terdam, Netherlands; 3 Amsterdam UMC location Vrije Universiteit Ams- RESEARCH PROGRAMME
terdam, Pediatrics, Amsterdam, Netherlands; 4 University of Groningen,
Department Of Pediatric Oncology, Groningen, Netherlands; 5 Radboud Line Frederiksen1 , Friederike Erdmann1,2 , Luzius Mader3 , Hanna
University Medical Center, Center Of Expertise For Cancer Survivorship, Mogensen4 , Camilla Pedersen1 , Line Kenborg5 , Andrea Bautz1 , Mats
Hematology, Nijmegen, Netherlands; 6 Sophia Children’s Hospital/Erasmus Talbäck4 , Elli Hirvonen6 , Thomas Tjørnelund Nielsen1 , Anne Elisabeth
Medical Center, Department Of Paediatric Oncology, Rotterdam, Nether- Andersen7 , Anna Holmqvist8 , Ole Sylvester9 , Jens Richardt Jepsen10 ,
lands; 7 Carl von Ossietzky University of Oldenburg, Faculty of Medicin and Nea Malila6 , Henrik Hasle11 , Laura Madanat-Harjuoja6,12 , Maria
Health Sciences, Health Services Research, Oldenburg, Germany; 8 Erasmus Feychting4 , Jeanette Winther5,13
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S85 of S635

1 Danish Cancer Society Research Center, Childhood Cancer Research O179 / #981 INEQUITIES IN NEUROCOGNITIVE
Group, Copenhagen, Denmark; 2 University Medical Center Mainz, Insti- MORBIDITIES AMONG PEDIATRIC BRAIN TUMOR SURVIVORS
tute Of Medical Biostatistics, Epidemiology And Informatics (imbei),
Mainz, Germany; 3 University of Bern, Institute Of Social And Preventive Christina Sharkey, Hannah Weisman, Karin Walsh, Kristina Hardy
Medicine, Bern, Switzerland; 4 Karolinska Institutet, Institute Of Environ- Children’s National Hospital, Neurology, Washington, United States of
mental Medicine, Stockholm, Sweden; 5 Danish Cancer Society Research America
Center, Childhood Cancer Research Group„ Copenhagen, Denmark; 6 Cancer
Registry of Finland, Finnish Cancer Registry, Helsinki, Finland; 7 Danish Can- Background and Aims: Survivors of pediatric brain tumors are at risk
cer Society Research Center, Statistics And Data Analysis, Copenhagen, for neuropsychological impairments, yet knowledge is lacking about
Denmark; 8 Lund University, Department Of Clinical Sciences, Lund, Swe- modifiable factors associated with increased risk. More information is
den; 9 Child and Adolescent Psychiatric Clinic, Private Clinic, Copenhagen, needed to design interventions that prevent or mitigate these prob-
Denmark; 10 Mental Health Services, Capital Region of Denmark, Univer- lems, which are a major source of long-term morbidity. This study
sity of Copenhagen, Center For Clinical Intervention And Neuropsychiatric aimed to examine the effect of child opportunity, a novel and actionable
Schizophrenia Research And Center For Neuropsychiatric Schizophrenia factor, on neurocognitive and psychosocial outcomes among survivors
Research, Copenhagen, Denmark; 11 Aarhus University Hospital, Depart- of pediatric brain tumors.
ment Of Pediatrics And Adolescent Medicine, Aarhus, Denmark; 12 Dana Methods: Neuropsychological data were abstracted from clinically-
Farber Cancer Institute, Boston Children’s Cancer And Blood Disorders Cen- referred pediatric brain tumor survivors (N=199, Mage=11.63, SD=
ter, Boston, United States of America; 13 Aarhus University, Department Of 4.63, 56.8% male, 71.8% White). Data consisted of parent-report
Clinical Medicine, Faculty Of Health, Aarhus, Denmark questionnaires including the Behavior Rating Inventory of Execu-
tive Function and the Child Behavior Checklist, and performance-
Background and Aims: A childhood cancer diagnosis and treatment- based measures, including an age-appropriate Wechsler Scale. Census
induced somatic late effects may affect the long-term mental health tract geocoding was used to map nationally-normed Child Oppor-
of survivors. We explored whether childhood cancer survivors are tunity Index (COI) scores for each participant. The COI measures
at higher risk of psychiatric disorders later in life than the general neighborhood-level quality of environmental and social conditions that
population and their siblings. contribute to positive health.
Methods: In a population-based cohort study based on Nordic reg- Results: COI scores varied, with 25.6% of survivors classified as living
isters, we included 18,621 five-year survivors of childhood cancer in Very Low to Moderate COI neighborhoods (M=73.21, SD=22.13).
diagnosed before age 20 years in Denmark, Finland and Sweden Higher overall COI scores were significantly related to lower parent-
between 1974 and 2011. Survivors were compared with 88,630 ran- reported General Executive Composite (F(3,181)=4.30, p < 0.01), and
domly selected population comparisons individually matched to the Working Memory (F(3,182)=3.07, p <0.05) problems. Higher opportu-
survivors by year of birth, sex and geographical region, and 24,775 sib- nity was significantly related to higher performance on the Working
ling comparisons. We followed our study population from five years Memory Index (F(1,146)=6.62, p<0.05), and was marginally related
after the childhood cancer diagnosis or corresponding calendar date to the Processing Speed Index (F(1,139)=3.79, p=0.05). Higher COI
for comparisons until end of 2017 and assessed information on hospital scores were significantly related to lower parent-reported Internal-
contacts for any and specific psychiatric disorders. izing Problems (F(1,183)=5.59, p < 0.05), but was not related to
Results: The cumulative incidence proportion of having had a psy- parent-reported Externalizing Problems (F(1,183)=3.38, p = 0.7).
chiatric hospital contact by age 30 years between 1979-2017 Conclusions: The present study identified social and environmen-
was 15.9% (95% CI, 15.3-16.5%) for childhood cancer survivors, tal inequities that contribute to sustained risk for neurocognitive
12.7% (95% CI, 12.4-12.9%) for population comparisons and 14.0% and psychological problems following treatment for pediatric brain
(95% CI, 13.5-14.5%) for siblings. Despite a small absolute dif- tumors. Further exploration of specific indicators that are most rele-
ference, survivors were at higher relative risk of any psychiatric vant for neuropsychological outcomes may provide salient targets for
hospital contact than the general population (HR, 1.34; 95% CI, a problem-prevention approach to mitigating the untoward effects of
1.28-1.39) and their siblings (HR, 1.39; 95% CI, 1.31-1.48). The oncological treatments.
higher risk persisted also at age 50. Furthermore, survivors had
a higher burden of recurrent psychiatric hospital contacts and
had more hospital contacts for different psychiatric disorders than O180 / #1612 SCREENING OF COGNITIVE SYMPTOMS
comparisons. DURING TREATMENT FOR CHILDHOOD ACUTE
Conclusions: Childhood cancer survivors are at higher long-term risk LYMPHOBLASTIC LEUKEMIA (ALL) PREDICTS COGNITIVE
of psychiatric disorders than the general population or their siblings. FUNCTIONING POST-TREATMENT
To improve mental health and the overall quality of life after childhood
cancer, survivorship care should include a focus on early signs of mental Kimberly Raghubar1 , Alyssa Krentzel1 , Johanna Escalante1 , Marilyn
health problems, especially among high-risk groups of survivors. Hockenberry2 , Michael Scheurer3 , Austin Brown4
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S86 of S635 ABSTRACTS

1 Texas Children’s Hospital/Baylor College of Medicine, Pediatrics - Psychol- FREE PAPER SESSION (FPS)
ogy, Houston, United States of America; 2 Baylor College of Medicine, Global
Hope, Houston, United States of America; 3 Baylor College of Medicine, FPS 11: ALL - RELAPSE AND CAR-T CELL THERAPY 30-09-2022
Pediatrics, Houston, United States of America; 4 Texas Children’s Hos- 2:00 PM - 3:30 PM
pital, Baylor College of Medicine, Pediatrics, Houston, United States of
America O181 / #793 COMPREHENSIVE PROTEOMIC PROFILING TO
IDENTIFY BIOMARKERS AND THERAPEUTIC TARGETS FOR
Background and Aims: Children treated for acute lymphoblastic IMMUNE EFFECTOR CELL ASSOCIATED NEUROTOXICITY
leukemia (ALL) are at risk for long-term cognitive weaknesses in (ICANS) AFTER TREATMENT WITH CHIMERIC ANTIGEN
attention, executive functions, and processing speed, which negatively RECEPTOR T CELLS
affect academic and vocational attainment. Therefore, it is important
to identify patients at greatest risk for cognitive symptoms during Caroline Diorio1 , Rawan Shraim1 , Regina Myers1 , Richard Aplenc1 ,
and following treatment in an effort to intervene early and miti- Brenda Banwell1 , Hamid Bassiri1 , Chakkapong Burudpakdee1 ,
gate lasting adverse effects. The primary objective of this study was Edward Behrens1 , Scott Canna1 , Fang Chen2 , Amanda Dinofia1 , Saar
to describe the trajectory of parent-reported child cognitive func- Gill2 , Vanessa Gonzalez2 , Michele Lambert1 , Allison Leahy1 , Bruce
tion during treatment and correlate these parent-reports with post- Levine2 , Robert Lindell1 , Shannon Maude1 , Jennifer Mcguire1 , Haley
treatment perceived and performance-based measures of cognitive Newman1 , Jessica Perazzelli1 , Alix Seif1 , Simon Lacey1 , Carl June2 ,
function. David Barrett3 , Stephan Grupp1 , David Teachey1
Methods: The PROMIS parent-proxy child Cognitive Function 1 Children’s Hospital of Philadelphia, Paediatrics, Philadelphia, United
questionnaire was collected at 4 time-points during child- States of America; 2 University of Pennsylvania, Cellular Immunothera-
hood ALL treatment for 165 participants, including 40 with pies, Philadelphia, United States of America; 3 Tmunity, Immunotherapy,
post-treatment neurocognitive evaluations of estimated IQ, Philadelphia, United States of America
attention, working memory, executive functioning, academic
achievement, and PROMIS parent-proxy reports. The trajec- Background and Aims: Cytokine release syndrome (CRS) and Immune
tory of parent-reported on-treatment cognitive function in their Effector Cell Associated Neurotoxicity Syndrome (ICANS) are poten-
children was evaluated using mixed effects linear regression. tially severe adverse effects associated with the administration of
Parent-reported child cognitive function at each time point was chimeric antigen receptor T-cells (CART). While the standard first-line
compared with post-treatment measures using multivariable linear CRS therapy is blockade of the IL-6 pathway, there is no standard
regression. ICANS treatment. Our aim was to profile the soluble proteome of
Results: Patients (55% male) were diagnosed with high-risk B- serial samples from patients with B-cell acute lymphoblastic leukemia
ALL (54.6%) at a mean age of 9.60 years. Parent-reported child treated with the CD19 directed CART CTL019 to identify targetable
cognitive function significantly declined during the first year of pathways for CRS and ICANS.
treatment (mean = -0.21 standard deviations (95% CI: -0.32, Methods: Using the Olink Explore 1536/384 panel, we measured 1463
-0.09, p<0.001). Parent-reported child cognitive function at proteins at multiple timepoints in 26 patients who did (N=13) and did
the end of the first year of treatment significantly correlated not (N=13) develop severe CRS (grade 3-5). 12/13 with severe CRS and
with those collected post-treatment, an average 3.8 years later 2/13 with minimal CRS developed ICANS. To understand biomarkers
(p=0.027), after accounting for demographic and clinical factors. associated with ICANS only and not CRS we leveraged the serial nature
Post-treatment parent-reported child cognitive function was sig- of our data and identified timepoints where patients were experienc-
nificantly associated with estimated IQ (p<.001), working memory ing ICANS exclusively. We compared to timepoints where both ICANS
(p<.05), attention (p<.05), word reading (p<.05), and calculation and CRS were being experienced using differential expression analysis
(p<.01). (DEA).
Conclusions: This study demonstrates that parent perceptions of Results: Given that the IL-1 receptor antagonist anakinra is being
cognitive decline are detectable shortly after ALL diagnosis and investigated as an ICANS therapy, we first evaluated the IL-1 fam-
persist into survivorship. Moreover, this study presents preliminary ily. While neither IL-1A nor IL-1B levels correlated with ICANS
evidence for use of the parent-proxy PROMIS Cognitive Function symptom onset, another IL1 family cytokine, IL-18, was closely cor-
questionnaire as a screener for cognitive difficulties that may require related with the ICANS onset (p<0.01). DEA demonstrated CELA3A
evaluation. was the protein most associated with ICANS alone. Notably, CHIT1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S87 of S635

and TREM2 were also identified as proteins associated with ICANS O183 / #1146 OUTCOMES FOLLOWING CD19 CART
alone. These proteins are associated with neuroinflammation in other REINFUSION IN CHILDREN AND YOUNG ADULTS WITH B-ALL
settings.
Conclusions: Serial samples from patients experiencing ICANS were Jamie Truscott1 , Adam Lamble2 , Emily Hsieh3 , Bonnie Yates4 , Samuel
profiled with a high throughput proteomic platform, revealing novel John1 , Seth Steinberg5 , Jennifer Sheppard1 , Agne Taraseviciute3 , Lee
potentially targetable proteins that may be contributing to the patho- Chen3 , Regina Myers6 , Susan Rheingold7 , Colleen Annesley2 , Lia
physiology of ICANS. IL-18 is of particular interest given the role of the Gore8 , Theodore Laetsch1,9 , Patrick Brown10 , Michael Pulsipher3,11 ,
IL-1 family in inflammation and should be investigated as a potential Deepa Bhojwani12 , Rebecca Gardner2 , Nirali Shah4
target for blockade in ICANS. 1 University of Texas Southwestern Medical Center, Pediatric Hematol-
ogy/oncology, Dallas, United States of America; 2 University of Washington,
Seattle Children’s Hospital, Pediatric Hematology/oncology, Seattle, United
O182 / #699 EARLY MUTATIONS IN THE BINDING SITE OF States of America; 3 Children’s Hospital of Los Angeles Cancer and Blood
THE RNA BINDING PROTEIN PTBP1 AS NEW MECHANISM OF Disease Institute, Norris Comprehensive Cancer Center, Keck School of
RESISTANCE TO CART-19 THERAPY IN B-ALL Medicine, University of Southern California, Section Of Transplantation And
Cellular Therapy, Los Angeles, United States of America; 4 National Can-
Claudia Paret, Nicole Ziegler, Nadine Lehmann, Francesca Alt, Khalifa cer Institute/Center for Cancer Research, National Institutes of Health„
El Malki, Jörg Faber Pediatric Oncology Branch, Bethesda, United States of America; 5 National
Center for Pediatric and Adolescent Medicine, University Medical Cen- Cancer Institute, National Institutes of Health, Biostatistics And Data
ter of the Johannes Gutenberg-University Mainz, Department Of Pediatric Management Section, Bethesda, United States of America; 6 Children’s Hos-
Hematology/oncology, Mainz, Germany pital of Philadelphia, Paediatrics, Philadelphia, United States of America;
7 Children’s Hospital of Philadelphia, Oncology, Philadelphia, United States
Background and Aims: Despite massive improvements in the treat- of America; 8 Children’s Hospital Colorado and The University of Colorado
ment of B-ALL through CART-19 immunotherapy, a large number of School of Medicine, Pediatrics, Denver, United States of America; 9 The Chil-
patients suffer a relapse due to the loss of the CD19 target anti- dren’s Hospital of Philadelphia, University of Pennsylvania, Developmental
gen. Frame shift mutations and isoforms lacking the CD19 epitope Therapeutics Program, Philadelphia, United States of America; 10 The Johns
have been described in this context. Here we analyzed by deep Hopkins Hospital, Oncology, Baltimore, United States of America; 11 Primary
sequencing which mutations affecting the structure of CD19 exist at Children’s Hospital, Huntsman Cancer Institute at the University of Utah,
diagnosis. Division Of Hematology And Oncology, Salt Lake City, United States of
Methods: Our cohort included 3 controls and 20 pediatric B-ALL America; 12 Children’s Hospital Los Angeles Cancer and Blood Disease
patients at initial diagnosis and, out of them, 15 samples in remission. Institute, Norris Comprehensive Cancer Center, Keck School of Medicine,
A region including exon 1 to 4 and intron 1 to 3 of the CD19 locus University of Southern California, Division Of Hematology/oncology, Los
was analysed by NGS with a minimum depth of coverage of 1000X. The Angeles, United States of America
effect of the mutations on RNA binding proteins (RBPs) binding sites
was evaluated. Background and Aims: CD19-targeted chimeric antigen receptor T-
Results: We identified a small deletion with a low allele frequency in cells (CD19 CART) are highly effective for relapsed/refractory B-cell
intron 2 in 35% of samples at initial diagnosis, but not the control group. acute lymphoblastic leukemia (B-ALL). Relapse following CART infu-
Moreover, the same patients in remission did not harbor this genetic sion (CART1), however, remains a concern. Outcomes following reinfu-
variant, indicating that it is specific to leukemic blasts. The deletion sion (CART2) for those with antigen positive relapse have historically
affected the binding site of PTBP1. In one sample from initial diag- been suboptimal. We explore indications for and outcomes follow-
nosis another mutation in intron 2 was detected with a high allele ing reinfusion of the original CART product (CART2) as pre-emptive
frequency. No leukemic blast specific mutations in the coding region therapy or treatment for relapse.
were identified. Methods: We conducted a multicenter, retrospective review of chil-
Conclusions: Leukemic blasts carry intron mutations in the binding dren and young adults with B-ALL who received either commercial
site of the RBP PTBP1. Loss of PTBP1 induces intron 2 reten- tisagenlecleucel or an investigational CD19-CAR construct for CART1
tion and premature termination of CD19. Such mutations occur at 6 US centers between 2012-2019. Analysis was restricted to
early as we detected them at diagnosis. Under the pressure of patients receiving reinfusion of the same product for CART2 without
CART-19 therapy, clones carrying the intron mutation and there- interval allogeneic stem cell transplant (SCT).
fore less CD19 may be selected. In contrast, frameshift muta- Results: Of 290 patients who recieved CART1, 40 (13.8%) also received
tions in the coding region may occur later, and a possible cor- CART2. The median interval between CART1 and CART2 was 5.5
relation with the therapeutic regimens before CART-19 should months (range: 1.2-33.7 months). Indications for CART2 included:
be considered. Mutations in introns are putative biomarkers for 1) relapse prevention (loss of B-cell aplasia, BCA) (n=23, 57%), 2)
an early detection of patients at risk of relapse under CART-19 relapsed disease (n=16, 40%), and 3) persistent CNS disease (n=1,
therapy. 3%). Cytokine release syndrome (CRS) was absent in 31 patients: grade
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S88 of S635 ABSTRACTS

1-2 in 8 patients and grade 3 in 1 patient. Amongst 23 receiving CART2 O185 / #1456 CYTOGENETIC DETERMINANTS OF
for relapse prevention, 22 maintained remission, but 8 (34.5%) ulti- OUTCOME POST FIRST RELAPSE OF B-CELL ACUTE
mately relapsed. BCA was re-established in 7/23 (30.4%) patients and LYMPHOBLASTIC LEUKEMIA (B-ALL): A CHILDREN’S
7 (30.4%) had CART2 engraftment. Of 17 patients receiving CART2 for ONCOLOGY GROUP (COG) STUDY
relapsed/persistent disease, 6 (35.3%) achieved CR, 8 (47.1%) achieved
BCA and 10 (58.8%) had CART2 engraftment. Of those achieving CR, Susan Rheingold1 , Deepa Bhojwani2 , Lingyun Ji3 , Xinxin Xu3 ,
all ultimately relapsed. Meenakshi Devidas4 , Mary Shago5 , Nyla Heerema6 , Andrew Carroll7 ,
Conclusions: Relapse prevention was the leading indication for CART2, Heather Breidenbach3 , Patrick Brown8 , Lia Gore9 , Naomi Winick10 ,
but CART2 functionality remains unclear particularly with limited William Carroll11 , Stephen Hunger1 , Elizabeth Raetz11 , Mignon Loh12
development of BCA or CART engraftment. Conversely, both CART2 1 Children’s Hospital of Philadelphia, Pediatrics, Philadelphia, United States

CR rates for relapsed disease and durable remissions were low. of America; 2 Children’s Hospital Los Angeles Cancer and Blood Disease
Optimizing CART2 strategies will be imperative to improve outcomes. Institute, Norris Comprehensive Cancer Center, Keck School of Medicine,
University of Southern California, Division Of Hematology/oncology, Los
Angeles, United States of America; 3 Children’s Oncology Group, Statistics,
O184 / #1622 FACTORS IMPACTING REAL-WORLD Monrovia, United States of America; 4 St Jude Children’s Research Hospital,
DECISIONS FOR USE OF HSCT IN TRANSPLANT-NAIVE Global Pediatric Medicine, Memphis, United States of America; 5 Hospital for
PATIENTS FOLLOWING CAR-T THERAPY IN A SINGLE Sick Children, Pathology, Toronto, Canada; 6 Nationwide Children’s Hospi-
INSTITUTION tal, Pathology, Columbus, United States of America; 7 University of Alabama
at Birmingham, Genetics, Birmingham, United States of America; 8 The
Jeremy Rubinstein1 , Christa Krupski2 , Christopher Dandoy2 , Ruby Johns Hopkins Hospital, Pediatrics, Baltimore, United States of America;
Khoury2 , Stella Davies2 , Christine Phillips1 9 Children’s Hospital Colorado and The University of Colorado School of
1 Cincinnati Children’s Hospital Medical Center, Oncology, Cincinnati, Medicine, Pediatrics, Denver, United States of America; 10 UT Southwest-
United States of America; 2 Cincinnati Children’s Hospital Medical Center, ern/Simmons Cancer Center, Pediatrics, Dallas, United States of America;
Bone Marrow Transplantation And Immune Deficiency, Cincinnati, United 11 NYU Langone Health, Pediatrics, New York, United States of America;
States of America 12 Seattle Children’s Hospital, Pediatrics, Seattle, United States of America

Background and Aims: Tisagenlecleucel (tisa-cel) is increasingly being Background and Aims: Pediatric B-ALL survival approaches 90%, but
used in hematopoietic stem cell transplant (HSCT) naïve patients. limited data exist on survival post first relapse based upon diagnostic
Outcomes for HSCT following CAR-T cell therapies demonstrate low cytogenetics.
relapse rates, however a significant portion of patients who receive Methods: We analyzed 5-year overall survival (OS) rate post-relapse,
tisa-cel can maintain remission without HSCT. Multiple factors are con- defined as duration between date of first relapse and death, among
sidered when choosing to proceed to HSCT. We aim to determine rate patients enrolled on 10 COG frontline B-ALL/infant ALL trials from
and timing of post-HSCT, factors that led to HSCT and overall survival. 1996-2014. Karyotype and FISH data were centrally reviewed. OS
Methods: We reviewed 30 patients who received tisa-cel at our rates were estimated with the Kaplan-Meier method and compared
institution who were transplant-naïve at the time of infusion. with logrank tests and Cox regression models at significance level 0.05.
Results: 3 of 30 failed to achieve an MRD negative remission by flow Results: Cytogenetic data for seven common ALL subtypes were avail-
cytometry. 12 of 27 responders remain alive with no evidence of dis- able for 10,361 patients; 6,698 were positive for one, 3,663 patients
ease (NED) without HSCT or any subsequent therapy. Of these who were negative for all (B-ALL-NOS). Patients with double trisomy (DT)
have not received HSCT, 5 of 12 had isolated extramedullary ALL and ETV6-RUNX1 had the longest median first complete remission
(CNS=4, testes=1) and no transplant planned. In the remaining 7 of (CR1) duration (43 months) and OS post-relapse (70% and 74%).
12 with bone marrow disease, close monitoring without HSCT elected Patients with hypodiploidy, KMT2Ar and TCF3-PBX1 had the short-
due to NGS negativity d28 and B cell aplasia (BCA) (n=6) or Down syn- est median CR1 (12.5 months, 18 months, 15.8 months) and worst
drome (n=1). 12 of 27 responders ultimately proceeded to HSCT. 3 of OS post-relapse (14.2%, 31.9%, 36.8% respectively). Older KMT2Ar
27 had planned bridge directly to HSCT due to CD22 negativity and/or patients had better survival than their infant counterparts, 31.9%
provider preference. 3 proceeded to HSCT following early loss of BCA versus 17.2%; with 97% of infants relapsing <36 months. Patients
and all 3 alive with NED. 6 underwent HSCT following relapse, 4 of with BCR-ABL1, iAMP21 and B-ALL-NOS had intermediate OS post-
6 alive with NED. 3 of 27 died following relapse post CART, without relapse (48%, 48.2%, 48.3%). Relapses in patients with hypodiploidy,
HSCT. BCR-ABL1, iAMP21 ALL were more likely to involve the bone mar-
Conclusions: Incorporation of close monitoring of NGS and B cell apla- row (94.4%, 84.6%, 82%) while relapse in patients with TCF3-PBX1
sia as well as site of disease can spare HSCT with maintenance of frequently involved the CNS (43.9%). Although rare, isolated testicu-
leukemia free remission in a subset of patients, while still allowing for lar relapses were more frequent in ETV6-RUNX1 and KMT2Ar ALL.
later uptake of HSCT in others. In our cohort, only a small subset of On extensive multivariable analysis adjusting for patient and disease
patients were unable to proceed to HSCT following relapse post CART. characteristics including time to relapse, and relapse site, KMT2Ar
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S89 of S635

and hypodiploid ALL remained significantly associated with poor OS doses/cycle) was combined with vincristine 1.5 mg/m2 (four pulses
post-relapse and DT and ETV6-RUNX1 with significantly improved OS once weekly), two 5-days blocks of dexamethasone 20 mg/m2 and
post-relapse. intra-thecal therapy. After cycle 1, patients could receive either com-
Conclusions: Cytogenetics at original diagnosis correlated significantly bination therapy or InO single agent.
with time to relapse, relapse site and outcome post-release and Results: Thirty-five patients were screened, 28 treated, and 27 were
remained a significant independent variable for improved (DT/ETV6- evaluable (median age 8.5 years; six were previously transplanted;
RUNX1) or worse (KMT2Ar/hypodiploid) survival on multivariable seven were refractory). Initially, four patients received 1.1 mg/m2 of
analysis. InO. Two DLTs occurred; grade 3 transaminases elevation > 7 days,
and Sinusoidal Obstruction Syndrome (SOS). InO was de-escalated
to 0.8 mg/m2 (n=6) without DLTs. The protocol was then amended
O186 / #321 INOTUZUMAB OZOGAMICIN (INO) COMBINED reducing dexamethasone to 10 mg/m2 /day to re-escalate InO; first
WITH CHEMOTHERAPY IN PEDIATRIC PATIENTS WITH at 1.1 mg/m2 (n=6, one DLT: grade 3 transaminases elevation > 7
RELAPSED/REFRACTORY CD22+ B-CELL PRECURSOR ACUTE days); subsequently at 1.4 mg/m2 (n=3, no DLTs) and then at 1.8
LYMPHOBLASTIC LEUKEMIA (BCP-ALL) – RESULTS FROM THE mg/m2 (n=7, one DLT: Absolute Neutrophil Count below 0.5 x 109 /L
ITCC-059 1B TRIAL > day 42). The most common non-hematological adverse event was
transaminases elevation (n=13, 53.8%), in eight (29.6%) cases at grade
Edoardo Pennesi1 , Erica Brivio1 , Anneke Ammerlaan1 , Yilin Jiang1 , 3/4. None had grade 3/4 bilirubin increase. Four patients developed
Vincent Van Der Velden2 , Berna Beverloo3 , Barbara Sleight4 , Susana SOS, of which one while on treatment. Four (14.8%) patients expe-
Rives5 , Bella Bielorai6 , Claudia Rössig7 , Arnaud Petit8 , Carmelo rienced sepsis, seven (25.6%) febrile neutropenia, and one (3.8%)
Rizzari9 , Ingrid Øra10 , Anna Nilsson10 , Gernot Engstler11 , Cristina another grade 3 infection. Thirteen patients experienced thrombocy-
Diaz12 , Francisco Bautista Sirvent1 , Alba Rubio-San-Simón13 , topenia of grade 3/4. No toxic deaths related to InO were observed.
Benedicte Bruno14 , Benoit Brethon15 , Fanny Rialland16 , Franco Six out of the seven patients treated at 1.8 mg/m2 were respon-
Locatelli17 , C. Michel Zwaan1 ders (bone marrow M1 with or without hematological recovery) after
1 Prinses Maxima Centrum for Pediatric Oncology, Trial And Data Center, cycle 1.
Utrecht, Netherlands; 2 Erasmus MC, University Medical Center, Depart- Conclusions: The RP2D of InO, in combination with vincristine and
ment Of Immunology, Rotterdam, Netherlands; 3 Erasmus MC, University dexamethasone (10 mg/m2 /day), was declared at 1.8 mg/m2 /cycle.
Medical Center, Department Of Clinical Genetics, Rotterdam, Netherlands;
4 Pfizer Inc, Oncology, Groton, Connecticut, United States of America;
5 Hospital Sant Joan de Déu de Barcelona, Pediatric Oncology And Hema- FREE PAPER SESSION (FPS)
tology Department, Barcelona, Spain; 6 Sheba Medical Center, Division Of
Pediatric Hematology And Oncology, Ramat Gan, Israel; 7 University Chil- FPS 12: NEUROBLASTOMA BASIC SCIENCES AND
dren’s Hospital Muenster, Pediatric Hematology And Oncology, Munster, SURVIVORSHIP 30-09-2022 2:00 PM - 3:30 PM
Germany; 8 Hopital Armand Trousseau, APHP, Sorbonne Université, Depart-
ment Of Pediatric Hematology And Oncology, Paris, France; 9 University of O187 / #484 THE IMMUNE CELL ATLAS OF HUMAN
Milano-Bicocca, MBBM Foundation, Pediatric Hematology-oncology Unit, NEUROBLASTOMA
Department Of Pediatrics, Monza, Italy; 10 Karolinska University Hospital,
Pediatric Oncology, Solna, Sweden; 11 St Anna Children’s Hospital, Medi- Bronte Manouk Verhoeven1 , Shenglin Mei2 , Thale Olsen3 , Karin
cal University of Vienna, Pediatric Oncology And Hematology Department, Gustafsson4 , Anders Valind5 , David Gisselsson Nord5 , Shahrzad
Vienna, Austria; 12 Vall D’Hebron Hospital, Pediatric Oncology And Hema- Fard1 , Catharina Hagerling5 , Peter Kharchenko2 , Per Kogner1 , John
tology Department, Barcelona, Spain; 13 Hospital Niño Jesús, Department Inge Johnsen1 , Ninib Baryawno1
Of Pediatric Oncology And Hematology, Madrid, Spain; 14 Hôpital Jeanne de 1 Karolinska Intitutet, Women’s And Children’s Health, Solna, Sweden;
Flandre, CHRU de Lille, Pediatric Hematology, Lille, France; 15 Hôpital Robert 2 Harvard Medical School, Department Of Biomedical Informatics, Boston,
Debré, Pediatric Hematology-oncology Unit, Paris, France; 16 Hôpital Mère- United States of America; 3 Karolinska Institutet, Department Of Women’s
Enfant, Service Onco-hématologie Pédiatrique, Nantes, France; 17 IRCCS And Children’s Health, Stockholm, Sweden; 4 Harvard University, Depart-
Ospedale Pediatrico Bambino Gesú, Department Of Hematology, Oncology ment Of Stem Cell And Regenerative Biology, Cambridge, United States
And Of Cell And Gene Therapy, Roma, Italy of America; 5 Lund University, Department Of Laboratory Medicine, Lund,
Sweden
Background and Aims: The objective of this Phase I trial was to deter-
mine the recommended phase 2 dose (RP2D) of InO in combination Background and Aims: Currently, there is a lack of comprehensive
with UKALL-R3 modified chemotherapy in pediatric BCP-ALL patients. immune cell overview at the single- cell level in neuroblastoma. Pre-
Methods: Multiple relapsed or refractory CD22-positive BCP-ALL vious studies have mainly focused on a single cell type and conflicting
were enrolled. A rolling-6 design was used, assessing Dose-Limiting results remain despite the different immune cells being extensively
Toxicities (DLT) during cycle 1 (28 days). InO (fractionated in 3 studied in vitro and in vivo. Therefore, we aimed to understand the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S90 of S635 ABSTRACTS

complete immune cell composition of neuroblastoma which will be ual disease (MRD). In this in vitro study, we tested the hypothesis
crucial for the development of novel immunotherapeutics. that mesenchymal neuroblastoma cells are unable to specifically
Methods: We performed single-cell RNA-sequencing on nineteen concentrate meta-iodobenzylguanidine (MIBG) due to lack of nore-
human neuroblastoma samples coupled with multiplex immunohisto- pinephrine transporter (NET) and vesicular monoamine transporter
chemistry (IHC) and survival analysis. Furthermore, we used single-cell (VMAT) expression, and, as a consequence, are undetectable on MIBG
RNA-sequencing data from normal fetal adrenal gland and additional imaging and evade targeted radionuclide MIBG therapy.
neuroblastoma datasets to increase the power of our study and Methods: Thirteen human neuroblastoma cell lines (n=3 MES, n=10
characterize cell-state changes from normal to cancerous. ADRN) were tested for NET, VMAT1, and VMAT2 mRNA expres-
Results: Our analysis revealed 27 immune cell subtypes including sion using real-time quantitative polymerase chain reaction (RT-qPCR).
distinct subpopulations of myeloid, NK, B and T cells not identi- In a time series, NET, VMAT1, and VMAT2 mRNA expression was
fied in neuroblastoma before. Infiltration of the main immune cell measured using RT qPCR in two transgenic adrenergic cell lines
types was validated by IHC. A survival benefit was demonstrated containing an inducible regulator of mesenchymal transformation:
for several immune cell subpopulations such as inflammatory mono- SK-N-BE(2)C-PRRX1 and SH-SY5Y-NOTCH3. [125 I]MIBG uptake and
cytes, macrophages, various T cell populations, and Active NK cells. retention assays were performed with seven neuroblastoma cell lines
In contrast to adult cancers and previous neuroblastoma studies, (n=3 MES, n=4 ADRN). Desipramine and reserpine served as NET and
we demonstrated an increase in inflammatory monocyte cell-state VMAT specific uptake inhibitors, respectively.
in tumor tissue, whereas no differences in cytotoxicity and exhaus- Results: NET, VMAT1, and VMAT2 mRNA expression in MES neurob-
tion score for cytotoxic T cells, nor in Treg activity were detected. lastoma cell lines (691-T, SHEP2, GIMEN) is very low or undetectable in
Lastly, receptor-ligand interaction analysis revealed a highly complex comparison with the ADRN cell lines. Reprogramming of the ADRN cell
interactive tumor microenvironment, where we highlighted several lines SK-N-BE(2)C and SH-SY5Y to a MES state using inducible PRRX1
interactions for further investigation. or NOTCH3-IC, resulted in a motile and mesenchymal phenotype,
Conclusions: Our study significantly broadens the understanding accompanied by a dramatic decrease in NET, VMAT1 and
of the immune landscape in neuroblastoma. We provide important VMAT2 mRNA expression over time. MES cell lines (691-T, SHEP2,
insights into the clinical relevance and create a resource for fur- GIMEN) are unable to specifically concentrate or retain MIBG.
ther investigation of interactions between different cell types for the Conclusions: Our study demonstrates that MES neuroblastoma cells
development of novel immunotherapeutics. show no uptake MIBG in vitro due to absence of critical transporters.
MES neuroblastoma cells may escape detection by MIBG imaging and
possibly evade targeted radionuclide MIBG therapy.
O188 / #651 MESENCHYMAL (MES) NEUROBLASTOMA
CELLS ARE UNABLE TO CONCENTRATE OR RETAIN
META-IODOBENZYLGUANIDINE (MIBG) DUE TO LACK OF O189 / #1182 COPPER CHELATION OVERCOMES THE
NOREPINEPHRINE TRANSPORTER (NET) AND VESICULAR IMMUNOSUPPRESSIVE TUMOR MICROENVIRONMENT IN
MONOAMINE TRANSPORTER (VMAT) EXPRESSION NEUROBLASTOMA

Thomas Blom1 , Johan Van Nes2 , Ellen Van Der Schoot3 , André Van Jourdin Rouaen1,2 , Daniele Mercatelli3 , Federica Saletta2 , Jayne
Kuilenburg4,5 , Lieve Tytgat6,7 Murray2 , Nicodemus Tedla4 , Federico Giorgi3 , Orazio Vittorio2
1 Princess Máxima Center for pediatric oncology, Research, Utrecht, Nether- 1 School of Women’s and Children’s Health, Unsw, Sydney, Australia;
lands; 2 Amsterdam UMC, location University of Amsterdam, Depart- 2 Children’s Cancer Institute, Metal Targeted Therapies & Immunology,
ment Of Human Genetics, Amsterdam, Netherlands; 3 Sanquin Research, Sydney, Australia; 3 University of Bologna, Department Of Pharmacy And
Department Of Experimental Immunohematology, Amsterdam, Nether- Biotechnology, Bologna, Italy; 4 UNSW Sydney, School Of Medical Sciences,
lands; 4 Amsterdam UMC, Department Of Clinical Chemistry, Laboratory Sydney, Australia
Genetic Metabolic Diseases, Amsterdam, Netherlands; 5 Cancer Center Ams-
terdam, Research, Amsterdam, Netherlands; 6 Princess Maxima Center for Background and Aims: Neuroblastoma is the most common extra-
Pediatric Oncology, Research, Utrecht, Netherlands; 7 Sanquin Research and cranial pediatric cancer with high-risk disease having a dismal five-year
Landsteiner Laboratory of the AMC- University of Amsterdam, Department overall survival rate of 50%. Antibody-based therapies targeting the
Of Experimental Immunohematology, Amsterdam, Netherlands surface glycolipid disialoganglioside (GD2) have shown promising anti-
tumor activity in patients however efficacy is strongly hampered by
Background and Aims: Intra-tumor heterogeneity of neuroblas- the immunosuppressive microenvironment. Given the emerging link
toma is composed of phenotypically divergent mesenchymal (MES) between copper and cancer immune evasion, we sought to examine
and adrenergic (ADRN) cell types. MES neuroblastoma cells are the impact of copper chelation therapy on the neuroblastoma tumor
more motile and chemotherapy-resistant in comparison with lineage- microenvironment and enhance the anti-tumor immune response.
committed ADRN neuroblastoma cells. Moreover, MES cells lack Methods: Using the Th-MYCN preclincal model, animals were
expression of genes commonly used for detection of minimal resid- treated with saline or the copper chelator tetraethylenepentamine
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S91 of S635

(TEPA; 400mg/kg) for seven days (n=8-10/group). To build a compre- by ethnic group (south Asian/non-south Asian) and Townsend depri-
hensive snapshot of the neuroblastoma tumor microenvironment, vation fifths (I-V) were compared over time for all cancers, leukaemia,
tumors were analysed using single-cell RNA sequencing, Luminex lymphoma, central nervous system (CNS) and other solid tumours. Haz-
xMAP cytokine profiling and mass spectrometry-based immunopep- ard ratios (HR: 95%CI) from adjusted Cox regression models quantified
tidomics. A tissue microarray was used to conduct Opal multiplex the estimated effect of deprivation (Townsend score) on mortality risk,
immunofluorescence and second harmonic generation imaging of by period of diagnosis, examining temporal patterns of inequalities.
fibrillar collagen. An in vitro co-culture evaluated combining TEPA with Results: Increasing deprivation was associated with significantly
anti-GD2 antibody therapy followed by combinatorial in vivo studies higher risk of death for children diagnosed with all cancers combined
using the NSX2>A/J syngeneic model. (HR: 1.05 (1.01-1.09)) and leukaemia (HR: 1.09 (1.01-1.17)) between
Results: Single cell RNA-sequencing analysis revealed copper chelation 1997 and 2001. Whilst we observed a trend towards reducing inequal-
skewed the abundance and metabolic activity of tumor and immune ities over time in these groups, a contrasting trend was observed for
cells. Treatment upregulated intrinsically low peptide presentation and CNS tumours whereby sizeable inequalities in mortality risk remained
produced several neo-antigens to promote the anti-tumor immune for cases diagnosed until 2012 (HR: 1.07 (0.99-1.15)). South Asian chil-
response. Results were supported by cytokine profiling with the upreg- dren with lymphoma had a 15% reduced chance of surviving at least
ulation of responses consistent with T cell, natural killer cell, and five-years after diagnosis compared to non-south Asians, across the
M1-macrophage activation. In situ infiltration was verified quantita- study period.
tively using Opal immunofluorescence with treatment weakening the Conclusions: Even in the United Kingdom, with a universally accessible
collagen matrix. Copper chelation was found to enhance the efficacy healthcare system, socio-economic and ethnic disparities in childhood
of anti-GD2 therapy in both in vitro and in vivo models, slowing tumor cancer survival exist. Findings should help inform where resources
growth and extending survival. should be directed to provide all children with an equitable survival
Conclusions: This study fortifies the role of copper in modulating the outcome following a cancer diagnosis.
neuroblastoma tumor microenvironment to overcome immune eva-
sion strategies and promote the anti-tumor immune response. Findings
provide crucial evidence in support of integrating clinically approved O191 / #609 IMPACT OF CHRONIC HEALTH CONDITION
copper chelators with immunotherapy to improve patient outcomes BURDEN ON SUBSEQUENT QUALITY-OF-LIFE AMONG ADULT
and survival. SURVIVORS OF CHILDHOOD CANCER: A REPORT FROM ST.
JUDE LIFETIME COHORT STUDY (SJLIFE)

O190 / #572 SOCIO-ECONOMIC AND ETHNIC INEQUALITIES Madeline Horan1 , Deokumar Srivastava2 , Nickhill Bhakta3 , Tara
IN CHILDHOOD CANCER SURVIVAL, YORKSHIRE, UK Brinkman4 , Zhaoming Wang1 , Kevin Krull4 , Kirsten Ness1 , Leslie
Robison1 , Melissa Hudson5 , I-Chan Huang1
Kirsten Cromie1 , Nicola Hughes2 , Sarah Milner2 , Paul Crump1 , Jacob 1 St. Jude Children’s Research Hospital, Epidemiology And Cancer Control,
Grinfeld3 , Anna Jenkins4 , Paul Norman5 , Susan Picton3 , Charles Memphis, United States of America; 2 St. Jude Children’s Research Hospi-
Stiller6 , Daniel Yeomanson4 , Adam Glaser2 , Richard Feltbower1 tal, Biostatistics, Memphis, United States of America; 3 St Jude Children’s
1 University of Leeds, Leeds Institute For Data Analytics, Leeds, United King- Research Hospital, Oncology, Department Of Global Pediatric Medicine,
dom; 2 Leeds Institute of Medical Research, School Of Medicine, Leeds, Department Of Epidemiology And Cancer Control, Memphis, United States
United Kingdom; 3 Leeds Teaching Hospitals NHS Trust, Leeds Childrens Hos- of America; 4 St. Jude Children’s Research Hospital, Epidemiology And Can-
pital, Leeds, United Kingdom; 4 Sheffield Children’s NHS Foundation Trust, cer Control, Psychology, Memphis, United States of America; 5 St. Jude
Haematology And Oncology, Sheffield, United Kingdom; 5 University of Children’s Research Hospital, Oncology, Memphis, United States of America
Leeds, School Of Geography, Leeds, United Kingdom; 6 NHS Digital, National
Cancer Registration And Analysis Service, Oxford, United Kingdom Background and Aims: Childhood cancer survivors often experience
quality-of-life (QOL) declines. It is critical to prevent worsening QOL
Background and Aims: Despite the critical importance of socio- by intervening on modifiable risk factors. We evaluated the impact
economic and ethnic inequalities in childhood cancer survival given the of baseline risk factors, including global and specific chronic health
potential years of life lost, national estimates of social or ethnic gradi- condition (CHC) burden, on future QOL.
ents in mortality over extended periods are not available. The study Methods: Participants included 4,294 adult survivors of childhood
aims to describe the effects of area-based deprivation and ethnicity cancer from SJLIFE who completed surveys and clinical assessments
on the survival of children diagnosed with cancer in the past 20 years, at two timepoints (T1 , T2 ) during follow-up care (median: 4.3 [range
focusing specifically on the extent to which prognostic disparities have 0.5-10.8] years apart). QOL was assessed using the SF-6D, a health-
changed over time. utility-based metric from 0-1 with higher scores indicating better QOL.
Methods: Cancer registration data (1997-2016) for 2,674 children (0- Individual CHCs were graded using a modified version of the Common
14 years) from the Yorkshire Specialist Register of Cancer in Children Terminology Criteria for Adverse Events. Grade 2-4 CHCs were cate-
and Young People were analysed. Kaplan-Meier five-year survival rates gorized into 10 organ systems (e.g., cardiovascular, endocrine). Global
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S92 of S635 ABSTRACTS

CHC burden was categorized into four levels (none/low, medium, high, Universiteit Amsterdam, Department Of Paediatric Oncology, Amsterdam,
very high). Multivariable regression examined associations of global Netherlands; 13 Dutch Childhood Oncology Group, Childhood Oncology,
CHC burden or individual CHCs (two separate models) at T1 with QOL Utrecht, Netherlands; 14 Princess Maxima Center for Pediatric Oncology,
at T2 , adjusting for age/sex, socio-economic (e.g., education, insurance), Stem Cell Transplantation Unit, Utrecht, Netherlands; 15 Leiden Univer-
and lifestyle factors (e.g., smoking, physical activity) at T1 . sity Medical Center, Department Of Internal Medicine, Leiden, Nether-
Results: Survivors with high (B: -0.028, p-value: 0.006) and very high lands; 16 Willem Alexander Children’s Hospital/Leiden University Medical
(B: -0.058, p-value: <0.001) global CHC burden at T1 had significantly Center, Department Of Pediatrics, Leiden, Netherlands; 17 Wilhelmina Chil-
poorer QOL at T2 versus survivors having no or low global burden. dren’s Hospital, University Medical Center Utrecht, Department Of Pedi-
Survivors with grade 2-4 cardiovascular (B: -0.021, p-value: 0.004), atric Endocrinology, Utrecht, Netherlands; 18 University Medical Center
musculoskeletal (B: -0.023, p-value: 0.013), and neurologic (B: -0.041, Utrecht, Central Diagnostic Laboratory, Utrecht, Netherlands; 19 Erasmus

p-value: <0.001) CHCs had significantly poorer QOL at T2 versus Medical Center, Department Of Clinical Chemistry, Rotterdam, Nether-
survivors with no or grade 1 CHCs. Current versus never smokers lands; 20 Erasmus Medical Center, Department Of Internal Medicine,
(B: -0.047, p-value: <0.001) and survivors with public versus private Section Endocrinology, Rotterdam, Netherlands; 21 Dutch Childhood Can-
health insurance (B: -0.055, p-value: <0.001) at T1 had poorer QOL cer Organisation, -, de Bilt, Netherlands; 22 Erasmus Medical Center,
at T2 . Oncode Institute And Department Of Molecular Genetics, Rotterdam,
Conclusions: Higher global CHC burden (dominant by cardiovas- Netherlands
cular, musculoskeletal, neurologic CHCs) and poor socio-economic
and lifestyle factors at baseline are risk factors of subsequent Background and Aims: Childhood cancer survivors seem to be at
poorer QOL among childhood cancer survivors. Early interven- increased risk of frailty and sarcopenia, two partly overlapping aging
tions targeting these modifiable risk factors may improve survivors’ phenotypes that have been associated with adverse health outcomes.
QOL. However, evidence on prevalence and risk factors of frailty and sar-
copenia is limited. We investigated this in a well-defined national
cohort of Dutch childhood cancer survivors diagnosed from 1963-
O192 / #243 FRAILTY AND SARCOPENIA WITHIN THE 2001.
EARLIEST NATIONAL DUTCH CHILDHOOD CANCER SURVIVOR Methods: 2,003 childhood cancer survivors aged 18-45 years at invi-
COHORT: A DCCSS-LATER STUDY tation were included (mean age at participation 33.1±7.2 years). We
defined prefrailty and frailty according to modified Fried criteria, and
Jenneke Van Atteveld1 , Demi De Winter1 , Vincent Pluimakers1 , Marta sarcopenia based on the EWGSOP2 definition. Associations between
Fiocco2,3,4 , Rutger-Jan Nievelstein1,5 , Monique Hobbelink5 , Leontien demographic, treatment-related, endocrine, as well as lifestyle-related
Kremer1,6,7 , Cecile Ronckers1 , Martha A. Grootenhuis1 , Heleen factors and both conditions were estimated with multivariable logistic
Maurice-Stam1 , Wim Tissing8,9 , Andrica De Vries1,10 , Jacqueline regression models.
Loonen11 , Eline Van Dulmen-Den Broeder12 , Helena Van Der Pal1 , Results: In survivors with complete frailty measurements (n=1,114,
Saskia Pluijm1 , Margriet Van Der Heiden-Van Der Loo1,13 , A. Birgitta 55.6% of participants) or complete sarcopenia measurements
Versluijs14 , Marloes Louwerens15 , Dorine Bresters1,16 , Hanneke Van (n=1,472, 73.5%), the percentage of prefrailty, frailty, and sarcopenia
Santen1,17 , Imo Hoefer18 , Sjoerd Van Den Berg19,20 , Jaap Den was 20.3%, 7.4%, and 4.4%, respectively. In the model for prefrailty in
Hartogh21 , Jan Hoeijmakers1,22 , Sebastian Neggers1,20 , Marry Van the full cohort (n=2,003), underweight and obesity, cranial irradiation
Den Heuvel-Eibrink1,10 (CRT), total body irradiation (TBI), cisplatin dose ≥600 mg/m2 , growth
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, hormone deficiency (GHD), hyperthyroidism, bone mineral density
Utrecht, Netherlands; 2 Leiden University Medical Center, Medical Statis- (BMD, Z-score ≤-1 but >-2, and Z-score ≤-2), and folic acid deficiency
tics Section, Department Of Biomedical Data Science, Leiden, Netherlands; were statistically significant. For frailty, associated factors included
3 Princess Maxima Center for Pediatric Oncology, Trial And Data Center, age at diagnosis between 10-18 years, underweight, CRT, TBI, cisplatin
Utrecht, Netherlands; 4 Leiden University, Mathematical Institute, Leiden, dose ≥600 mg/m2 , higher carboplatin doses, cyclophosphamide equiv-
Netherlands; 5 University Medical Center Utrecht, Department Of Radi- alent dose ≥20 g/m2 , hyperthyroidism, BMD Z-score ≤-2, and folic acid
ology And Nuclear Medicine, Utrecht, Netherlands; 6 University Medical deficiency. Male sex, lower body mass index (continuous), CRT, TBI,
Center Utrecht, Wilhelmina Children’s Hospital, -, Utrecht, Netherlands; hypogonadism, GHD, and vitamin B12 deficiency were significantly
7 Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam, associated with sarcopenia.
Pediatrics, Amsterdam, Netherlands; 8 Princess Maxima Center for Pedi- Conclusions: Our findings show that frailty and sarcopenia occur
atric Oncology, Supportive Care, Utrecht, Netherlands; 9 University Medical already at a mean age of 33 years in childhood cancer sur-
Center Groningen, Department Of Pediatric Oncology, Groningen, Nether- vivors, i.e., conceivably more than three decades earlier than
lands; 10 Sophia Children’s Hospital/Erasmus Medical Center, Department in the general population. Early recognition and interventions
Of Paediatric Oncology, Rotterdam, Netherlands; 11 Radboud University for endocrine disorders and dietary deficiencies may be cru-
Medical Center, Department Of Haematology, Nijmegen, Netherlands; cial in minimizing the risk of (pre)frailty and sarcopenia in this
12 Emma Children’s Hospital/Amsterdam University Medical Center, Vrije population.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S93 of S635

FREE PAPER SESSION (FPS) than the control group (32.3±9.7 mL/kg/min) (p=0.015). The inter-
vention group had a better Sit-To-stand (29.7±5.4 vs 23.2±5.4 reps,
FPS 13: SUPPORTIVE CARE - EXERCISE, CARDIAC HEALTH AND p<0.001), Timed-Up-and-Go (3.4±0.4 vs 5.4±0.6 seconds, p<0.001),
EARLY WARNING SYSTEMS 30-09-2022 2:00 PM - 3:30 PM and Handgrip strength (26.9±12.0 vs 21.6±10.4 kg, p=0.014). Balance
scores were comparable between groups.
O193 / #1236 EFFECTS OF A PHYSICAL ACTIVITY Conclusions: Peer-supported, supervised, in-hospital, physical activity
PROGRAM FOR CHILDREN AND ADOLSECENTS WITH CANCER during treatment can cardiorespiratory fitness and physical function
FROM DIAGNOSIS TO ONE-YEAR POST TREATMENT ON after ended treatment.
CARDIORESPIRATORY FITNESS AND PHYSICAL FUNCTION

Martin Fridh1 , Peter Schmidt-Andersen2 , Anna Pouplier3 , Troels O194 / #1316 GET STRONG TO FIGHT CHILDHOOD
Thorsteinsson4 , Henrik Hasle5 , Kjeld Schmiegelow6 , Hanne Larsen2 CANCER: AN EXERCISE INTERVENTION FOR CHILDREN AND
1 Copenhagen University Hospital, Rigshospitalet, Department Of Pedi- ADOLESCENTS UNDERGOING ANTI-CANCER TREATMENT
atrics And Adolescent Medicine, The Juliane Marie Center, Copenhagen, (FORTEE)
Denmark; 2 University of Copenhagen, Rigshospitalet, Paediatric Oncol-
ogy Research Laboratory, Department Of Paediatrics And Adolescent Elias Dreismickenbecker1 , Francesca Lanfranconi2 , Sandra Stössel1 ,
Medicine, Copenhagen, Denmark; 3 University hospital of Copenhagen, Marie Neu1 , Lisa Ploch1 , Norbert Paul3 , Christian Ruckes4 , Adriana
Rigshospitalet, Paediatric Oncology Research Laboratory, Depart- Balduzzi2 , Peter Wright5 , Stan Windsor5 , Joachim Wiskemann6 ,
ment Of Paediatrics And Adolescent Medicine, København, Denmark; Inaam El-Rajab6 , Céline Gravot7 , Veronika Picmanova7 , Rodolf
4 Copenhagen University, Department Of Nutrition, Exercise And Sports, Mongondry8 , Wilhelm Bloch9 , Katie Rizvi10 , Martin Fridh11 , Alejandro
Copenhagen N, Denmark; 5 Aarhus University Hospital, Department Of Lucia12 , Carmen Fiuza-Luces13 , Miriam Götte14 , Filippo Spreafico15 ,
Pediatrics And Adolescent Medicine, Aarhus, Denmark; 6 Rigshospitalet, Barbara Konda16 , Lidija Kitanovski17 , Marco Sowa-Israel18 , Tobias
Department Of Paediatrics And Adolescent Medicine, Copenhagen, Baader19
Denmark 1 University Medical Center of the Johannes Gutenberg-University Mainz,
Childhood Cancer Center Mainz, Mainz, Germany; 2 Fondazione Monza e
Background and Aims: Childhood cancer survivors experience Brianza per Il Bambino e La Sua Mamma, Clinica Pediatrica, Università Degli
impaired cardiorespiratory fitness and physical function restricting Studi Di Milano Bicocca, Monza, Italy; 3 University Medical Center of the
their possibilities to engage in social activities including sport, leisure Johannes Gutenberg-University Mainz, Institute For The History, Philosophy
activities and school. The study objective was to determine the effects And Ethics Of Medicine, Mainz, Germany; 4 University Medical Center of the
of classmate-supported, supervised, in-hospital physical activity Johannes Gutenberg-University Mainz, Interdisciplinary Centre For Clini-
program during treatment on primarily cardiorespiratory fitness and cal Studies (izks), Mainz, Germany; 5 Oxford Brookes University, Department
secondarily on physical function one-year after ended treatment. Of Sport And Health Science And Social Work, Oxford, United Kingdom;
Methods: National non-randomized controlled trial including 6 Heidelberg University Hospital and National Center for Tumor Diseases,
schoolchildren aged 6–18 years at diagnosis treated with chemo- Department Of Medical Oncology, Working Group Exercise Oncology, Hei-
/radiotherapy. Seventy-five of 120 included children in the intervention delberg, Germany; 7 Concentris research management GmbH, Concentris
group (61% boys, 13.4±3.1 years), and 33 of 58 in control group (58% Research Management Gmbh, Fürstenfeldbruck, Germany; 8 Centre de Lutte
boys, 13.5±2.5 years) participated in testing post-treatment. Anthro- Contre le Cancer Léon Bérard, Prevention Cancer Environment Depart-
pometrics and cancer diagnoses were comparable between groups ment, Lyon, France; 9 German Sport University Cologne, Department Of
(p>0.05). The intervention consisted of: (i) supervised in-hospital Molecular And Cellular Sport Medicine At The Institute Of Cardiology
physical activity from diagnosis and throughout intensive treatment; And Sports Medicine, Cologne, Germany; 10 Youth Cancer Europe, Youth
(ii) 90-minutes general educational session on cancer and therapy Cancer Europe, Cluh-Napoca, Romania; 11 Department of Pediatrics and
in the child’s school class; and (iii) selection of two classmates as Adolescent Medicine, University Hospital Rigshospitalet, Copenhagen, Den-
ambassadors who took turns to be co-admitted to the hospital and mark; 12 Universidad Europea de Madrid, School Of Doctorate Studies And
support the child’s physical training during the daytime. The primary Research, Madrid, Spain; 13 Hospital 12 de Octubre Research Institute,
outcome was cardiorespiratory fitness (VO2 peak, mL/min/kg) (sex, ’imas12’, Physical Activity And Health Laboratory, Madrid, Spain; 14 Essen
age, diagnosis adjusted). Secondary outcomes were Sit-To-stand, University Hospital, Exercise Oncology Working Group, Essen, Germany;
Timed-Up-and-Go, Handgrip strength, and balance test scores. Trial 15 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Oncology Unit,

registration: 11. January 2013. Clinicaltrial.gov NCT01772849 and Milan, Italy; 16 Forma 3D Ltd, Forma 3d, Ljubljana, Slovenia; 17 University
NCT01772862. Medical Center Ljubljana, Division Of Pediatrics, Department Of Haema-
Results: All children had ambassadors allocated. In the intervention tooncology, Ljubljana, Slovenia; 18 Nurogames GmbH, Nurogames, Cologne,
group, a total of 3364 individual and 726 group sessions were held, with Germany; 19 Pixformance Sports GmbH, Pixformance Sports, Dallgow-
a median of 34 session [4-85]. One-year post-treatment termination, Döberitz, Germany
VO2 peak was higher in the intervention group (37.4±5.7 mL/kg/min)
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S94 of S635 ABSTRACTS

Background and Aims: Cancer is the leading cause of death by non- dam, Netherlands; 6 Amalia Children’s Hospital, Radboud university medical
communicable diseases in children in Europe. During cancer treatment, center, Pediatric Cardiology, Nijmegen, Netherlands; 7 Tel Aviv Sourasky
patients’ morbidity is increased due to e.g. physical inactivity and Medical Centre, Sackler School of Medicine, Tel Aviv University, Pediatric
cancer-related fatigue. Personalised exercise training during the inten- Cardiology, Tel Aviv, Israel; 8 Emma Children’s Hospital, Amsterdam UMC,
sive phase of cancer treatment in children and adolescents is a promis- University of Amsterdam, Pediatric Oncology, Amsterdam, Netherlands;
ing therapy to mitigate above mentioned issues. However, evidence for 9 Medical imaging, Radboud university medical center, Cardiology, Nijmegen,

using exercise to counteract fatigue and improve health-related quality Netherlands; 10 The Hospital for Sick Children, The University of Toronto,
of life is lacking in paediatric oncology. Pediatric Cardiology, Toronto, Canada; 11 Great North Children’s Hospital
Methods: The FORTEe research project intends to evaluate a per- and Translational and Clinical Research Institute, Newcastle University Cen-
sonalised and standardised exercise intervention in 450 children, tre for Cancer, Newcastle University, Hematology/oncology, Newcastle upon
adolescents and young adults undergoing cancer treatment in nine cen- Tyne, United Kingdom; 12 Institut Gustave Roussy, Radiation Epidemiology,
tres across Europe. This randomised, controlled multicentre trial aims Villejuif, France; 13 The Hospital for Sick Children, Hematology/oncology,
to generate high evidence for an innovative, patient-centred exercise Toronto, Canada; 14 University Medical Center Utrecht, Wilhelmina Chil-
treatment as part of the standard of care. Experiences and exper- dren’s Hospital, Pediatrics, Utrecht, Netherlands; 15 Emma Children’s Hos-
tise in paediatric exercise oncology within Europe were merged to pital, Amsterdam UMC, University of Amsterdam, Pediatrics, Amsterdam,
develop specific exercise training and testing protocols which are to be Netherlands; 16 City of Hope National Medical Center, Population Sciences,
implemented with the help of digital, innovative technologies. Duarte, United States of America
Results: The elaborated protocols are standardised to enable adapted
personalised exercise training. A meticulous approach to tailored Background and Aims: Childhood cancer patients are at risk for
exercise includes a sensitive functional evaluation system to set the anthracycline-induced cardiotoxicity, which may be prevented by
appropriate exercise dosage, aiming at defining type, frequency and dexrazoxane. However, there are concerns about its safety and there
intensity. Precision exercise training protocols are adapted and person- is little guidance on its use in children. To facilitate global consensus,
alised to the cancer patient’s clinical phenotype. The intensity, time and the International Late Effects of Childhood Cancer Guideline Har-
volume of exercise are adjusted to the cancer treatment intensity and monization Group developed a transparent evidence-based clinical
to each patient’s clinical condition and response. For this purpose, dif- practice guideline for dexrazoxane administration in children diag-
ferent modalities are included that allow training sessions in-person nosed with cancer at age 21 years or younger who will be treated with
but also remotely, supported by innovative technologies. anthracyclines.
Conclusions: As a progress beyond the current state-of-the-art, FOR- Methods: A panel of 20 international multidisciplinary specialists
TEe has the ambition to implement paediatric exercise oncology as (pediatric hematology-oncology, pediatric cardiology, pharmacology,
an evidence-based standard in clinical care for all childhood cancer epidemiology, and guideline methodology) performed a systematic
patients worldwide. Acknowledgment: This project has received fund- literature review, developed evidence summaries, appraised the evi-
ing from the European Union’s Horizon 2020 research and innovation dence, and formulated recommendations on the basis of evidence,
programme under grant agreement N. 945153 clinical judgement, and consideration of benefits versus the harms.
The GRADE Evidence-to-Decision framework was used to translate
evidence to recommendations.
O195 / #307 PRIMARY CARDIOPROTECTION WITH Results: We identified 18 eligible studies, both randomized and non-
DEXRAZOXANE IN CHILDHOOD CANCER PATIENTS EXPECTED randomized trials, assessing the use of dexrazoxane in patients with
TO RECEIVE ANTHRACYCLINES: RECOMMENDATIONS FROM different types of malignancies. Given the dose-dependent risk of
THE INTERNATIONAL LATE EFFECTS OF CHILDHOOD CANCER anthracycline-induced cardiotoxicity, we concluded that the benefits
GUIDELINE HARMONIZATION GROUP likely outweigh the risk of subsequent neoplasms when the cumulative
doxorubicin or equivalent dose is ≥250 mg/m2 (very low to low qual-
Elvira Van Dalen1 , Esmée De Baat1 , Renée Mulder1 , Melissa Hudson2 , ity evidence, moderate recommendation). The decision to administer
Matthew Ehrhardt2 , Frederike Engels3 , E (Lieke) Feijen1 , Heynric dexrazoxane or not should be shared by the patient/family and health-
Grotenhuis4 , Jan Leerink1,5 , Livia Kapusta6,7 , Gertjan Kaspers1,8 , care provider after careful consideration of the potential harms and
Remy Merkx9 , Luc Mertens10 , Roderick Skinner11 , Wim Tissing1 , benefits. No recommendation could be formulated for cumulative dox-
Florent De Vathaire12 , Paul Nathan13 , Leontien Kremer1,14,15 , orubicin or equivalent doses of <250 mg/m2 . Also, no recommendation
Annelies Mavinkurve-Groothuis1 , Saro Armenian16 could be made for the use of dexrazoxane in patients treated with the
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, anthraquinone mitoxantrone.
Utrecht, Netherlands; 2 St. Jude Children’s Research Hospital, Oncology, Conclusions: This guideline aims to improve health outcomes
Memphis, United States of America; 3 Princess Máxima Center for Pedi- by assisting decision-making about when to co-administer
atric Oncology, Pharmacy, Utrecht, Netherlands; 4 University Medical Center dexrazoxane with anthracycline treatment. Further research
Utrecht, Wilhelmina Children’s Hospital, Pediatric Cardiology, Utrecht, is encouraged to determine the long-term efficacy and
Netherlands; 5 Amsterdam University Medical Center, Cardiology, Amster- safety of dexrazoxane in childhood cancer patients and to
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S95 of S635

determine its role in patients who are expected to receive and translated into English. Using MAXQDA, thematic content anal-
mitoxantrone. ysis explored transcript segments coded for “stage of change” and
compared stakeholder types and study sites.
Results: Stakeholders important for PEWS adoption included PEWS
O196 / #815 STAGE OF CHANGE: STRATEGIES TO implementation leaders, clinical staff, hospital leadership, and other
PROMOTE SUSTAINED USE OF A PEDIATRIC EARLY WARNING PEWS champions. Key identified strategies for movement through
SYSTEM IN RESOURCE-LIMITED PEDIATRIC ONCOLOGY the stage of change involved presentation of evidence demonstrating
CENTERS IN LATIN AMERICA PEWS effectiveness, enthusiastic individuals serving as models for oth-
ers in the same clinical role, implementation of pilot programs before
Marisa Cristin Woo1 , Gia Ferrara2 , Maria Puerto-Torres2 , Srinithya scale-up, and policies enforced by hospital leadership facilitating habit-
Gillipelli2,3 , Paul Elish4 , Hilmarie Muniz-Talavera2 , Alejandra ual use of PEWS. Effective engagement strategies targeted hospital
Gonzalez-Ruiz2 , Miriam Armenta5 , Camila Barra6 , Rosdali leadership early during PEWS adoption to provide programmatic legiti-
Diaz-Coronado7 , Cinthia Hernández González8 , Susana Juárez macy and motivate clinical staff. Pilot programs allowed clinical staff to
Tobias9 , Jose De Jesus Loeza10 , Alejandra Méndez-Aceituno11 , Erika see benefits of PEWS in practice and support movement through the
Montalvo12 , Eulalia Penafiel13 , Estuardo Pineda14 , Dylan Graetz2 , stage of change towards maintained PEWS adoption.
Teresa Kortz15 , Asya Agulnik2 Conclusions: This study identifies strategies to promote sustained use
1 University of California, San Francisco, Institute For Global Health Sci- of PEWS that can serve as recommendations for resource-limited cen-
ences, San Francisco, United States of America; 2 St. Jude Children’s Research ters implementing PEWS or other evidence-based interventions that
Hospital, Department Of Global Pediatric Medicine, Memphis, United States improve hospital outcomes. Our findings highlight the importance of
of America; 3 Baylor College of Medicine, School Of Medicine, Houston, tailoring strategies to the motivations and needs of each stakeholder
United States of America; 4 Emory University, Rollins School Of Public type.
Health, Atlanta, United States of America; 5 Hospital General de Tijuana,
Department Of Pediatric Oncology, Tijuana, Mexico; 6 Hospital Dr Luis Calvo
Mackenna, Department Of Pediatric Oncology, Santiago, Chile; 7 Instituto O197 / #1149 HOSPITAL FACTORS IMPACTING
Nacional de Enfermedades Neoplásicas, Department Of Pediatric Oncology, SUSTAINABILITY OF PEDIATRIC EARLY WARNING SYSTEMS IN
Lima, Peru; 8 Hospital Infantil Teletón de Oncología, Department Of Pedi- RESOURCE-LIMITED PEDIATRIC ONCOLOGY CENTERS
atric Oncology, Querétaro, Mexico; 9 Hospital Central Dr Ignacio Morones
Prieto, Department Of Pediatrics, San Luis Potosí, Mexico; 10 Hospital Centro Asya Agulnik1 , Sara Malone2 , Maria Puerto-Torres1 , Adolfo
Estatal de Cancerología, Department Of Pediatric Oncology, Xalapa, Mex- Cardenas1 , Yichen Chen1 , Dylan Graetz1 , Kim Prewitt2 , Carlos
ico; 11 National Unit of Pediatric Oncology, UNOP, Department Of Pediatric Acuña3 , Ivania Alfonso4 , Shillel Alvarez5 , Leticia Aradi6 , Daniela Arce
Critical Care, Guatemala, Guatemala; 12 Hospital Oncológico Solca Núcleo Cabrera7 , Mariuxy Barragan8 , Rosario Batista9 , Carolina Bravo10 ,
de Quito, Department Of Pediatric Critical Care, Quito, Ecuador; 13 Instituto Maria Cach11 , Gloria Ceballo12 , Cristal Chacon-Gaviria13 , Mayra
del Cáncer SOLCA Cuenca, Department Of Pediatric Oncology, Cuenca, Chavez-Rios14 , Euge Costa15 , María Cuencio-Rodríguez16 , Mario
Ecuador; 14 Hospital Nacional de Niños Benjamín Bloom, Department Of Delgado17 , Rosdali Diaz Coronado18 , Clara Perez-Fermin19 , Ever
Pediatric Oncology, San Salvador, El Salvador; 15 University of California, Fing20 , Teresa Garcia-Sarmiento21 , Wendy Gomez Garcia22 , Cinthia
San Francisco, Department Of Pediatrics, San Francisco, United States of Hernández González23 , Yajaira Valentine Jimenez Antolinez24 , Susana
America Juárez Tobias25 , Esmeralda Leon26 , Idalia Lozano27 , Angelica
Martinez28 , Sheybi Miralda29 , Erika Montalvo30 , Carlos
Background and Aims: Pediatric Early Warning Systems (PEWS) are Pérez-Alvarado31 , Monica Quijano-Lievano32 , Nancy Reyes33 , Edwin
evidence-based interventions that support early detection of hospital- Sanchez34 , Rocio Sanchez35 , Marcia Serrano36 , Veronica
ized children at risk for clinical deterioration. Relevant to successful Soto-Chavez37 , Isidoro Tejocote-Romero33 , Sergio Valle38 , Annie
implementation of PEWS, “stage of change” characterizes stakeholder Vázquez39 , Daniel Villarroel40 , Meenakshi Devidas41 , Douglas Luke2 ,
support for PEWS across a continuum based on their willingness and Virginia Mckay2
effort to adopt a new intervention. This study identifies strategies that 1 St. Jude Children’s Research Hospital, Department Of Global Pediatric
support stakeholders through the stage of change towards sustained Medicine, Memphis, TN, United States of America; 2 Washington University
use of PEWS. in St. Louis, Brown School, St. Louis, United States of America; 3 Hospital Dr.
Methods: At five pediatric oncology centers in Peru, Mexico, El Luis Calvo Mackenna, Pediatric Critical Care Unit, Santiago, Chile; 4 Hospital
Salvador, and Ecuador, semi-structured in-depth interviews were con- Saint - Damien, Pediatrics, Haiti - Port Au Prince, Haiti; 5 Benemérito Hos-
ducted with 71 staff involved in PEWS implementation. Purposive pital General con Especialidades ĺJuan María de Salvatierraĺ, Pediatrics,
sampling was used to select centers requiring variable time to complete campeche, Mexico; 6 Centro Medico Nacional Siglo XXI, Pediatrics, Mexico,
PEWS implementation with low-barrier centers requiring three to Mexico; 7 Hospital Pediatrico de Sinaloa, Hemato-oncology Unit, Culia-
four months and high-barrier centers requiring ten to eleven months. can, Mexico; 8 SOLCA Cuenca, Oncology, Cuenca, Ecuador; 9 Hospital Jose
Interviews were conducted in Spanish, professionally transcribed, Domingo De Obaldia, Oncology, Chiriqui, Panama; 10 SOLCA Guayaquil,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S96 of S635 ABSTRACTS

Oncology, Guayaquil, Ecuador; 11 Hospital General Agustin O’Horan, Oncol- characteristics associated with CSAT scores were evaluated using
ogy, Merida, Mexico; 12 Hospital del Niño "Jose Renan Esquivel", Oncology, Wilcoxon Sum-rank test, Kruskal-Wallis rank sum test, or Spearman’s
Panama, Panama; 13 Hospital Infantil de Especialidades de Chihuahua, rank correlation, as appropriate.
Oncology, Chihuahua, Mexico; 14 Hospital para el Niño Poblano, Oncology, Results: We received 813 responses (response rate 70%, center
Puebla, Mexico; 15 Hospital del Niño de la Santísima Trinidad, Oncology, range 27-100%) with a median CSAT score of 4.3 (of 5, center
Cordoba, Argentina; 16 Hospital Universitario Austral, Pediatrics, Buenos range 3.6-4.7). Among centers, five were in a low-middle income,
Aires, Argentina; 17 Hospital Nacional de Niños, Oncology, San Jose, Costa 33 upper-middle income, and one high-income country; country
Rica; 18 Instituto Nacional de Enfermedades Neoplásicas, Department Of income level was not associated with CSAT score (p=0.27). There
Pediatric Oncology, Lima, Peru; 19 Hospital Infantil Regional Universitario was also no relationship between CSAT score and pediatric can-
Dr. Arturo Grullon, Pediatrics, Santiago, Dominican Republic; 20 Hospital cer volume (5-800 new diagnoses annually), hospital type (general,
General Celaya, Oncology, Celaya, Mexico; 21 Hospital de Especialidades oncology, women’s/children’s, pediatric multidisciplinary, or pediatric
Pediatricas, Oncology, Tuxtla Gutiérrez, Mexico; 22 Dr. Robert Reid Cabral oncology), funding structure (public, private, or mixed), teaching vs.
Children’s Hospital, Pediatric Oncology, Sto Domingo, Dominican Repub- non-teaching hospital, or nurse-to-patient ratio (range: 1 nurse/2.5-10
lic; 23 Hospital Infantil Teletón de Oncología, Department Of Pediatric patients).
Oncology, Querétaro, Mexico; 24 Hospital Universitario "Dr. José Eleuterio Conclusions: This cross-sectional evaluation of resources for
González", Universidad Autónoma de Nuevo León, Hematology, Nuevo PEWS sustainability across a large, diverse group of resource-
León, Mexico; 25 Hospital Central Dr Ignacio Morones Prieto, Department limited pediatric oncology hospitals did not identify hospital
Of Pediatrics, San Luis Potosí, Mexico; 26 Hospital Guillermo Almenara characteristics associated with PEWS sustainability. These find-
Irigoyen, Oncology, Lima, Peru; 27 Hospital San Jose, Tec de Monterrey, ings suggest that sustainability of evidence-based interventions
Oncology, Monterrey, Mexico; 28 Hospital General de Tijuana, Pediatric like PEWS may be based on modifiable factors, rather than
Hemato-oncology Unit, Tijuana, Mexico; 29 Hospital Escuela Universitario, strictly related to a hospital’s structure, funding, or resource-
Oncology, Tegucigalpa, Honduras; 30 Hospital Oncológico Solca Núcleo de level.
Quito, Department Of Pediatric Critical Care, Quito, Ecuador; 31 Centro

Estatal de Cancerología "Dr. Miguel Dorantes Mesa", Oncología Pediatrica,


Xalapa, Mexico; 32 Centro Medico Imbanaco, Oncology, Cali, Colombia; O198 / #1183 ASSESSMENT OF THE QUALITY OF
33 HOSPITAL MATERNO INFANTIL ISSEMYM, Pediatric Oncology, TOLUCA, INTERDISCIPLINARY COMMUNICATION (CRITCOM) AROUND
Mexico; 34 Hospital Nacional de Ninos Benjamin Bloom, Pediatrics, San PATIENT DETERIORATION IN PEDIATRIC
Salvador, El Salvador; 35 Instituto Regional De Enfermedades Neoplásicas HEMATOLOGY-ONCOLOGY CENTERS OF VARIABLE
Del Sur, Oncology, Arequipa, Peru; 36 Hospital Caja Petrolera de Salud RESOURCE-LEVELS
Santa Cruz, Pediatrics, Santa Cruz, Bolivia; 37 Hospital Civil de Guadala-
jara, Pediatrics, Guadalajara, Mexico; 38 National Unit of Pediatric Oncology, Jocelyn Rivera1 , Sara Malone2 , Maria Puerto-Torres3 , Kim Prewitt4 ,
UNOP, Pediatric Critical Care, Guatemala, Guatemala; 39 Hospital Nacional Firas Sakaan5 , Zebin Al Zebin6 , Anita Arias7 , Parthasarathi
Edgardo Rebagliati Martins, Pediatrics, Lima, Peru; 40 Hospital del Niño Bhattacharyya8 , Sanjeeva Gunasekera9 , Sherry Johnson10 , Joyce
Manuel Ascencio Villarroel, Pediatrics, Cochabamba, Bolivia; 41 St Jude Kambugu11 , Erica Kaye12 , Belinda Mandrell13 , Jennifer Mack14 ,
Children’s Research Hospital, Global Pediatric Medicine, Memphis, United Jennifer Mcarthur7 , Alejandra Méndez-Aceituno15 , Lisa Morrissey16 ,
States of America Rana Sharara-Chami17 , Jennifer Snaman14 , Liz Sniderman18 , Douglas
Luke2 , Dylan Graetz5 , Asya Agulnik5
Background and Aims: Pediatric Early Warning Systems (PEWS) 1 Hospital Infantil Teleton de oncologia, Emergency Medicine, Queretaro,
aid early identification of deterioration in hospitalized children with Mexico; 2 Washington University in St. Louis, Brown School, St. Louis, United
cancer. While successful PEWS implementation is possible in resource- States of America; 3 St. Jude Children’s Research Hospital, Department
limited settings, sustainability of PEWS has not been assessed long- Of Global Pediatric Medicine, Memphis, TN, United States of America;
term. This study evaluates hospital resources needed for PEWS 4 Washington University in St. Louis, Brown University, St. Louis, United
sustainability in these settings and their relationship to hospital char- States of America; 5 St. Jude Children’s Research Hospital, Department
acteristics. Of Global Pediatric Medicine, Memphis, United States of America; 6 3.
Methods: Clinical staff using PEWS at 39 diverse Spanish-speaking Pediatric Hematology and Oncology, King Hussein Cancer Cente, Depart-
pediatric cancer centers from 14 countries in Latin America involved ment Of Critical Care, Amman, Jordan; 7 St. Jude Children’s Research
in a collaborative to implement PEWS were included. Resources for Hospital, Division Of Pediatric Critical Care, Memphis, United States of
PEWS sustainability was assessed using the Clinical Sustainability America; 8 Tata Medical Center, Department Of Pediatric Oncology Criti-
Assessment Tool (CSAT), which evaluates clinical capacity to sustain cal Care, Kolkata, India; 9 National Cancer Institute, Paediatric Oncology,
PEWS through 35 items on a Likert-type scale (1-5, higher scores rep- Maharagama, Sri Lanka; 10 St. Jude Children’s Research hospital, Global
resenting better sustainability) across seven domains. Responses to Pediatric Medicine, Memphis, United States of America; 11 Uganda Can-
individual items were averaged for an overall CSAT score and individ- cer Institute, Pediatric Oncology, Kampala, Uganda; 12 St. Jude Children’s
ual responses were averaged for a hospital-level CSAT score. Hospital Research Hospital, Oncology, Memphis, United States of America; 13 St. Jude
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S97 of S635

Children’s Research Hospital, Nursing, Memphis, United States of America; FREE PAPER SESSION (FPS)
14 Dana Farber Cancer Institute, Pediatric Hematology/oncology, Boston,
United States of America; 15 National Unit of Pediatric Oncology, UNOP, FPS 14: PPO - SIBLINGS, PARENTS AND ROBOTS 30-09-2022
Pediatric Critical Care, Guatemala, Guatemala; 16 Boston Children’s Hospi- 2:00 PM - 3:30 PM
tal, Hematopoietic Stem Cell Transplant Unit - 6west, Boston, United States
of America; 17 American University of Beirut Medical Center, Department Of O199 / #1156 SIBACCESS: DEVELOPMENT OF A NEW
Pediatrics And Adolescent Medicine, Beirut, Lebanon; 18 Stollery Children’s TRAUMA-FOCUSED INTERVENTION FOR ADOLESCENT
Hospital, Northern Alberta Children’s Cancer Care Program, Edmonton, SIBLINGS OF YOUTH WITH CANCER
Canada
Kristin Long1 , Christina Amaro2 , Kathryn Davis1 , Marcella
Background and Aims: High-quality care requires strong interdis- Mazzenga1 , Nicole Cardona1 , Anna Muriel3 , Melissa Alderfer2
ciplinary communication. However, no tools exist to evaluate com- 1 Boston University, Psychological & Brain Sciences, Boston, United States of

munication around patient clinical deterioration. We describe the America; 2 Nemours Children’s Health, Center For Healthcare Delivery Sci-
development and pilot testing of CritCom, a bilingual (English and ence, Wilmington, United States of America; 3 Dana Farber Cancer Institute,
Spanish) assessment tool to assess the quality of interdisciplinary com- Pediatric Psychosocial Oncology, Boston, United States of America
munication around patient deterioration for children hospitalized with
cancer. Background and Aims: Siblings of youth with cancer frequently report
Methods: CritCom was developed using a literature review sup- high levels of cancer-related posttraumatic stress. The importance
plemented with input and analysis from a focus group composed of addressing siblings’ needs has been extensively documented and
of 25 multidisciplinary global experts in interdisciplinary commu- is a standard of care in pediatric oncology. Yet, there are no well-
nication and pediatric oncology. This process occurred over six established sibling interventions. The few existing interventions are
phases: domain definition and development, content validity among poorly matched to siblings’ clinical presentations regarding posttrau-
international experts, face validity through international cognitive matic stress, and these interventions inadequately consider cultural
interviews, translation, and linguistic consistency in both languages. aspects of families’ treatment preferences. Siblings’ likelihood of get-
Following the initial validation process, we are conducting a pilot ting much-needed support is further limited by their absence from the
study of CritCom among healthcare and an international group hospital, where most supportive programs are based. This research
of multidisciplinary professionals and countries to evaluate the aims to develop a new culturally-informed intervention guided by the
instrument. pediatric medical traumatic stress framework that addresses system-
Results: We are piloting CritCom in 30 countries (10 Spanish and atic barriers to supporting siblings through a telehealth approach. The
20 English-speaking countries), comprising 408 healthcare workers SibACCESS (Acceptance, Coping, Communication, Engagement, and
from 28 English- and 31 Spanish-speaking pediatric cancer cen- Social Support) program is based on trauma-focused CBT and focuses
ters. Participants volunteered for the CritCom pilot via an online on increasing siblings’ exposure to and processing of cancer-related
survey. We will examine the feasibility, usability, and reliability cues.
of CritCom in an international and multidisciplinary population. Methods: Parents and adolescent siblings of youth with cancer (N=18)
Reliability will be assessed using internal consistency measures, completed semi-structured interviews to gather feedback on the pro-
including Cronbach’s alpha. Confirmatory factor analysis will eval- posed goals and format of SibACCESS. Purposive sampling ensured
uate the subscale among the seven CritCom domains (actionable, that the sample represented breadth across cancer type, treatment
tone, mechanisms & modes, empowerment, clarity, collaboration & status, and sibling race/ethnicity, age, and gender. Data were analyzed
teamwork, and systems). The pilot will allow an initial analysis of using applied thematic analysis.
hospital and participant characteristics associated with the per- Results: Across cultural backgrounds, siblings and parents indicated
ceived quality of interdisciplinary communication at participating overwhelming support for SibACCESS. They were enthusiastic about
centers. This is the first study to obtain comparable data on ele- the group-based format, emphasis on skill-building, and traumatic
ments of interdisciplinary communication around patient deterioration stress focus. Families suggested ways to increase parent involvement,
globally. noting that parents’ cancer-related posttraumatic stress may affect
Conclusions: CritCom results provide a quantitative, their ability to support siblings. They discussed ways to meet families’
center-specific assessment of the quality of interdisci- preferences for a shorter program while ensuring a therapeutic dose.
plinary communication around patient deterioration that Conclusions: Findings warrant moving forward to a pilot trial of SibAC-
can be used to improve communication and quality of care CESS. By addressing sibling-specific barriers to care and targeting
globally. siblings’ unique psychosocial needs, SibACCESS is responsive to the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S98 of S635 ABSTRACTS

striking need for sibling services. Better support is expected to improve O201 / #689 TRAJECTORIES OF CANCER-RELATED
siblings’ psychosocial functioning and reduce the burden of cancer on TRAUMATIC STRESS AMONG SIBLINGS OF CHILDREN WITH
families. CANCER WITHIN TWO YEARS OF DIAGNOSIS

Melissa Alderfer1 , Simran Kripalani2 , Christina Amaro1 , Alison Taggi


O200 / #1871 THE PAT SIBLING MODULES: PSYCHOMETRIC Pinto1 , Amanda Lewis1 , Aimee Hildenbrand1 , E. Anne Lown3 , Kristin
PROPERTIES OF A SIBLING PSYCHOSOCIAL RISK SCREENER Long4
1 Nemours Children’s Health, Center For Healthcare Delivery Science, Wilm-

Kathryn Davis1 , Melissa Alderfer2,3 , Emily Pariseau1 , Amanda Lewis2 , ington, United States of America; 2 Rowan University, Cooper Medical
Anne Kazak2,3 , Kristin Long1 School, Camden, United States of America; 3 University of California San
1 Boston University, Psychological & Brain Sciences, Boston, United States Francisco, School Of Nursing, San Francisco, United States of America;
of America; 2 Nemours Children’s Hospital, Center For Healthcare Delivery 4 Boston University, Psychological & Brain Sciences, Boston, United States of

Science, Wilmington, United States of America; 3 Sidney Kimmel Medical America


College, Thomas Jefferson University, Philadelphia, United States of America
Background and Aims: Altered family responsibilities, roles,
Background and Aims: Psychosocial screening is recommended to and routines after a pediatric cancer diagnosis impact all fam-
connect siblings of youth with cancer to appropriate psychosocial ser- ily members. While many siblings of children with cancer are
vices. The lack of validated sibling-specific screening tools is a barrier to resilient, they are at risk for emotional and behavioral problems
routine screening. The PAT Sibling Modules were recently developed to including cancer-related post-traumatic stress (PTS). This study
address this barrier. The Modules are comprised of two sibling-specific explored trajectories of sibling PTS within two years after cancer
psychosocial screeners: the baseline version, which parents complete diagnosis.
shortly after cancer diagnosis, and the follow-up version, which parents Methods: Siblings (aged 8-17; M=11.2 years) across the United
complete months after diagnosis and includes additional questions to States were recruited from the rosters of SuperSibs powered by
capture siblings’ ongoing adjustment to cancer. Specific items differ Alex’s Lemonade Stand Foundation. The cohort sequential longi-
across sibling age groups. The current study aims to validate the PAT tudinal design had three data collection points ∼6 months apart
Sibling Module follow-up version. beginning at 6- or 12-months post-diagnosis. Siblings (N=229;
Methods: Parents were recruited from a national sample of fam- 42% of those approached) completed the Child Post-Traumatic
ilies facing childhood cancer. Parents (N=252) completed the PAT Stress Disorder Symptom Scale. Half (54%) were female; 67%
Sibling Module follow-up version for all siblings ages 0-17 in the fam- were non-Hispanic White. The children with cancer (M=8.7 years
ily (N=483, M=9.52 years) at three time points between 6 and 24 old) were diagnosed with liquid (48%), solid (35%), brain (12%),
months post-cancer diagnosis. Half of siblings were female (51%); 64% or other cancers (5%). Latent Class Growth Analysis (LCGA) and
were non-Hispanic White. Parents also completed the Strengths and Growth Mixture Modeling (GMM) identified PTS patterns across
Difficulties Questionnaire (SDQ) for one sibling aged 8-17 (N=235, time.
M=10.93 years). Convergent and predictive validity were examined Results: Fit statistics supported a linear pattern of change across time.
using cross-sectional and longitudinal analyses. LCGA suggested a three-class model, while GMM suggested five or six
Results: At each time point, the PAT Sibling Module follow-up version classes. The three-class model included a large group (61%) with stable
showed strong (ages 3-4, 5-9, and 10+ versions; Kuder-Richardson 20 mild PTS, a smaller group (35%) with stable moderate PTS, and a small
range: 0.81-0.90) or adequate (ages 0-2 version; Kuder-Richardson 20 group (4%) with severe PTS improving slightly across time. Four-class
range: 0.62-0.84) internal consistency. The PAT Sibling Module showed models replaced the stable moderate PTS class with a group improv-
strong convergent validity at each time point (r’s>0.5, p’s<0.001). ing from moderate to mild PTS (17-21%) and a group worsening from
Regarding predictive validity, PAT Sibling Module scores explained mild to moderate PTS across time (16-17%). The five and six-class
a significant amount of the variance in SDQ difficulties scores at models included 2+ classes with few participants (< 3%), suggesting
subsequent time points (R2 range: 0.46-0.49; p’s<0.001). irreproducibility.
Conclusions: Findings support the use of the PAT Sibling Module Conclusions: A substantial subset of siblings of children with can-
follow-up version as a reliable and valid screener for sibling psychoso- cer experiences moderate to severe cancer-related PTS following
cial risk following cancer diagnosis. Validation of a sibling-specific diagnosis. Additional research should identify factors associated with
screener is a necessary first step to identifying and addressing sib- increasing PTS or sustained severe symptoms across time to inform
lings’ unmet psychosocial needs and improving trajectories of sibling interventions. Acknowledgement: Funding provided by the Andrew
functioning. McDonough B+ Foundation
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S99 of S635

O202 / #557 WORRIES AND ANXIETY IN PARENTS OF O203 / #661 HOW TELEPRESENCE ROBOTS SUPPORT
LONG-TERM SURVIVORS OF CHILDHOOD CANCER: A REPORT SCHOOL AGED CHILDREN’S SOCIAL CONNECTEDNESS TO
FROM THE SWISS CHILDHOOD CANCER SURVIVOR THEIR SCHOOL DURING CANCER TREATMENT
STUDY-PARENTS
Mette Weibel1,2,3 , Sofie Skoubo4 , Inger Hallström2 , Lykke Bertel5,6 ,
Salome Christen1 , Katharina Roser1 , Erika Harju1 , Fabienne Kjeld Schmiegelow1,3 , Hanne Larsen1,3
Gumy-Pause2,3 , Luzius Mader4 , Janine Vetsch5 , André O. Von 1 Rigshospitalet, Department Of Paediatrics And Adolescent Medicine,
Bueren2,3 , Gisela Michel1 Copenhagen, Denmark; 2 Lund University., Department Of Health Sciences,
1 University of Lucerne, Department Of Health Sciences And Medicine, Faculty Of Medicine, Lund, Sweden; 3 University of Copenhagen, Faculty
Luzern, Switzerland; 2 University Hospital of Geneva, Pediatric Oncology Of Health Sciences, Copenhagen, Denmark; 4 National Rehabilitation Cen-
And Hematology Unit, Department Of Women, Child And Adolescent, ter for Neuromuscular Diseases, National Rehabilitation Cen, Aarhus C,
Geneva, Switzerland; 3 University of Geneva, Cansearch Research Plat- Denmark; 5 Aalborg Centre, Aalborg Centre For Problem Based Learning
form For Pediatric Oncology And Hematology, Department Of Pediatrics, In Engineering Science And Sustainability Under The Auspices Of Unesco,
Gynecology And Obstetrics, Faculty Of Medicine, Geneva, Switzerland; Aalborg, Denmark; 6 Aalborg University, The Technical Faculty For It And
4 University of Bern, Institute Of Social And Preventive Medicine, Bern, Design, Aalborg Eas, Denmark
Switzerland; 5 Institute of Applied Nursing Science, Eastern Switzerland Uni-
versity Of Applied Sciences, Department Of Health Sciences, St. Gallen, Background and Aims: Telepresence-robots can facilitate virtual
Switzerland inclusion and allow children to participate remotely in social and aca-
demic activities. Children with cancer have high absenteeism during
Background and Aims: Having a child diagnosed with cancer is a treatment. In this study we explore how telepresence-robots sup-
devastating experience and many parents worry about the long-term port the social connectedness of children with their classmates during
health of their child. We aimed to compare worries and anxiety cancer treatment.
in parents of adult childhood cancer survivors (CCS-parents) with Methods: The study is designed as a feasibility study. Using con-
parents of the Swiss general population (GP-parents), and evaluate venience sampling, 128 semi-structured interviews were conducted
characteristics associated with worries in CCS-parents. from 2020-2022, including 52 repeated interviews with 30 children
Methods: We conducted a nationwide survey in a population-based diagnosed with cancer (7-18 years), 40 repeated interviews with teach-
sample of CCS-parents (CCS aged ≥20 years at study, ≤16 years ers (n=30) and 36 repeated focus group interviews with classmates
at diagnosis, >5 years post-diagnosis) and GP-parents (at least one (n=145). Further, a total of 15 hours of participant observations were
child aged ≥20 years). We used the Worry and Anxiety Questionnaire conducted in seven classrooms when the child attended school through
(WAQ), and computed the WAQ total score (’worry’; possible range the robot. An abductive approach, based on the theory of Agential
0-80) and cases for General Anxiety Disorder (’anxiety’). Additionally, realism, and a situational analysis were used.
we computed the proportion of parents meeting no caseness criteria Results: Three overarching themes are described 1) Isolation; 2) Per-
(’normal levels of worry and anxiety’). We used multivariable linear sonalization; 3) Inclusion/exclusion. Preliminary findings show that
regression to identify characteristics associated with WAQ total score telepresence-robots allow children to participate in social activities in
in CCS-parents. their classes during hospitalization. The children experienced inclusion
Results: We included 787 CCS-parents (41.0% fathers), on average 24 in social activities, e.g. playing in the schoolyard, informal conversa-
years after diagnosis of their child, and 478 GP-parents (42.3% fathers). tion, and games. However, they experienced a feeling of exclusion
We found that CCS-parents and GP-parents did not differ regarding when teachers or classmates forgot to turn on the robot or over-
’worry’ (16.6 vs. 17.1, p>0.05) or ’anxiety’ (2.6% vs. 3.6%, p>0.05). More looked their presence while being online. Additionally, children felt
CCS-parents had ’normal levels of worry and anxiety’ (79.9% vs 74.3%, exposed when getting additional attention. The ability of classmates
p=0.039). In CCS-parents, mothers (β=3.8; 95%CI:1.4-6.2; p=0.002), to include the child in social activities through telepresence-robots
parents with an only child (β=6.1; 95%CI:2.2-10.1; p=0.003), parents enable them to express interest and empathy for the child. Like-
who experienced pain (β=3.5; 95%CI:0.2-6.9; p=0.036), or disadvan- wise, the telepresence-robot facilitated classmates to understand the
tages because of their child’s former disease (β=8.6; 95%CI:5.0-12.2; cancer disease and reduce their concerns about the child’s condi-
p<0.001), or had current support needs (β=10.0; 95%CI:4.4-15.6; tion. Teachers described that some classmates lost interest in talking
p=0.001) reported higher levels of ’worry’. to the child when they were not verbally active or did not use the
Conclusions: Overall, it is encouraging that most parents of adult telepresence-robot regularly.
childhood cancer survivors report normal levels of worry and anxi- Conclusions: Telepresence-robots participation can positively impact
ety. We identified a group of parents of survivors who are at risk for social connectedness with classmates, and children’s psychosocial
higher levels of worries, and these parents may benefit from additional development. However, telepresence robots require a high level of
support. facilitation and ethical considerations to avoid exclusionary situations.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S100 of S635 ABSTRACTS

O204 / #1852 INTERACTIVE EDUCATION BY A SOCIAL FREE PAPER SESSION (FPS)


ROBOT ON SLEEP HYGIENE AT THE PEDIATRIC ONCOLOGY
OUTPATIENT CLINIC: FEASIBILITY, EXPERIENCES OF FAMILIES, FPS 15: EPIDEMIOLOGY - NEW INSIGHTS FROM REGISTRIES
AND PRELIMINARY EFFECTIVENESS AND ETIOLOGY OF CANCER 30-09-2022 2:00 PM - 3:30 PM

Kelly Van Bindsbergen1,2 , Hinke Van Der Hoek2 , Marloes Van Gorp2 , O205 / #267 ASSOCIATION OF BREASTFEEDING WITH
Mike Ligthart3 , Koen Hindriks3 , Mark Neerincx4,5 , Tanja Alderliesten6 , CHILDHOOD HEMATOLOGIC MALIGNANCIES AND BRAIN
Peter Bosman7 , Hans Merks8 , Martha A. Grootenhuis2 , Raphaele Van TUMORS: A REPORT FROM THE CHILDHOOD CANCER AND
Litsenburg2 LEUKEMIA INTERNATIONAL CONSORTIUM
1 Emma Kinderziekenhuis, Amsterdam UMC, Pediatrics, Amsterdam,
Netherlands; 2 Prinses Maxima Center for Pediatric Oncology, Psycho- Jeremy Schraw1 , Tiffany Chambers1 , Audrey Bonaventure2 , Helen
oncology, Utrecht, Netherlands; 3 Vrije Universiteit Amsterdam, -, Bailey3 , Ana Maria Mora4 , Eve Roman5 , Jacqueline Clavel2 , Maria
Amsterdam, Netherlands; 4 Delft University of Technology, -, Delft, Nether- Karalexi6 , Evangelia Ntzani7 , Sameera Ezzat8 , Wafaa Rashed9 , Erin
lands; 5 TNO, Organization for Applied Scientific Research, -, Soesterberg, Marcotte10 , Logan Spector10 , Elizabeth Milne11 , Catherine Metayer4 ,
Netherlands; 6 Leiden University Medical Cente, -, Leiden, Netherlands; Alice Kang4 , Corrado Magnani12 , Lucia Miligi13 , Juan Manuel
7 Centrum Wiskunde & Informatica, -, Amsterdam, Netherlands; 8 Princess Mejia-Arangure14 , Juan Carlos Nunez-Enriquez15 , Claire
Máxima Center for Pediatric Oncology, Pediatric Oncology, Utrecht, Infante-Rivard16 , Friederike Erdmann17 , Joachim Schuz18 , John
Netherlands Dockerty19 , Eleni Petridou6 , Beth Mueller20 , Michael Scheurer1
1 Baylor College of Medicine, Pediatrics, Houston, United States of America;

Background and Aims: Children with cancer often experience 2 Institut national de la santé et de la recherche médicale, Epidemiology Of
sleep problems, associated with many negative physical and Childhood And Adolescent Cancers Team, Villejuif, France; 3 Curtin Univer-
psychological health outcomes, and lower quality of life. Interven- sity Medical School, Pediatrics, Perth, Australia; 4 University of California
tions to improve sleep in this population are therefore needed. Berkeley School of Public Health, Center For Environmental Research And
We aimed to evaluate interactive education by a social robot Community Health, Berkeley, United States of America; 5 University of York,
on sleep hygiene at the pediatric oncology outpatient clinic Health Sciences, York, United Kingdom; 6 National and Kapodistrian Uni-
regarding feasibility, experiences of families, and preliminary versity of Athens, Division Of Hygiene And Epidemiology, Athens, Greece;
effectiveness. 7 Brown University, Center For Evidence Synthesis In Health, Providence,
Methods: Children between 8-12 years old, receiving anti-cancer United States of America; 8 National Liver Institute, Department Of Epidemi-
treatment, and visiting the outpatient clinic with their parents dur- ology And Preventive Medicine, Cairo, Egypt; 9 Children’s Cancer Hospital
ing the two week period of the study were approached. Researchers Egypt 57357, Research, Cairo, Egypt; 10 University of Minnesota Medical
filled out observation forms regarding feasibility, and parents filled out School, Pediatrics, Minneapolis, United States of America; 11 University of
the Children’s Sleep Hygiene Scale before and two weeks after the Western Australia, Telethon Kids Institute, Perth, Australia; 12 Universitá del
education. Experiences of children and parents were evaluated in semi- Piemonte Orientale, Dipartimento Di Medicina Traslazionale, Novara, Italy;
structured interviews. Open answers were analyzed using a thematic 13 Institute for Cancer Research, Prevention and Clinical Network (ISPRO),
approach. Descriptive statistics were used to describe feasibility and Environmental And Occupational Epidemiology Branch-cancer Risk Fac-
experiences, and a dependent sample t-test to evaluate preliminary tors And Lifestyle Epidemiology Unit, Florence, Italy; 14 Instituto Nacional
effectiveness. de Medicina Genómica, Laboratorio De Genómica Del Cáncer, Mexico
Results: Twenty-eight families participated (58% response rate) and City, Mexico; 15 Instituto Mexicano del Seguro Social (IMSS), Unidad De
all interactions with the robot could be completed. Children and Investigación Médica En Epidemiología Clínica, Unidad Médica De Alta
parents reported they learned something new (75% and 50% respec- Especialidad (umae), Mexico City, Mexico; 16 McGill University, Epidemiol-
tively), wanted to receive education from the robot more often ogy, Biostatistics, And Occupational Health, Montreal, Canada; 17 University
(83% and 75% respectively), and applied sleeping tips from the Medical Center Mainz, Institute Of Medical Biostatistics, Epidemiology
robot at home afterwards (54%). Preliminary effectiveness showed And Informatics (imbei), Mainz, Germany; 18 International Agency for
a statistically significant improvement in sleep hygiene (p = .047, Research on Cancer, Section Of Environment And Radiation, Lyon, France;
d = 0.39). 19 University of Otago, Dunedin School Of Medicine, Dunedin, New Zealand;

Conclusions: Providing education about sleep hygiene in a novel, inter- 20 University of Washington, Epidemiology, Seattle, United States of
active way using a social robot at the outpatient clinic seemed feasible, America
and children and parents were mostly positive. There is preliminary evi-
dence that the sleep hygiene of children improved, but more research Background and Aims: Breastfeeding has been associated with
is needed. reduced risk of acute lymphoblastic leukemia (ALL); however, data on
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S101 of S635

other childhood cancers are lacking and few studies have investigated Methods: We followed a cohort of all children born in Denmark
exclusive breastfeeding. between 1997 and 2018 (n=1,360,230) from birth until date of
Methods: We pooled individual participant data from nineteen stud- leukemia, censoring (death, emigration, 20th birthday) or Dec 31, 2018,
ies in the Childhood Cancer & Leukemia International Consortium whichever occurred first. Cox proportional hazard model was used
to determine whether breastfeeding was associated with ALL, acute to estimate hazard ratios (HRs) for leukemia (any, lymphoid, acute
myeloid leukemia (AML), childhood brain tumors (CBT) collectively, myeloid and other/unspecified) comparing vaccinated (any and spe-
and astrocytoma, medulloblastoma, and ependymoma individually. We cific types) with unvaccinated children. Also, the association for each
estimated odds ratios (ORs) and 95% confidence intervals (CIs) for additional dose was compared with one dose.
breastfeeding (any, <4 months, 4-6 months, >6 months) using uncon- Results: During the follow-up of 14,536,819 person-years, 771 chil-
ditional logistic regression models adjusted for study, sex, birthweight, dren were diagnosed with leukemia (74.4% lymphoid, 16.1% acute
maternal age, maternal education, and history of daycare attendance. myeloid, 9.5% other/unspecified). No association was found for any
We also evaluated the association of exclusive breastfeeding with ALL. childhood vaccination and any leukemia (1.04, 95% CI: 0.50-2.17)
We confirmed findings using random effects meta-analysis. with a change in HR of 0.97 (95% CI: 0.94-1.02) per dose. The cor-
Results: We included 10,782 cases with ALL, 1,690 cases with AML, responding HRs for lymphoid leukemia, acute myeloid leukemia and
1,279 cases with astrocytoma, 709 cases with medulloblastoma, 265 other/unspecified leukemia were respectively 2.76 (95% CI: 0.66-
cases with ependymoma, and 17,189 controls. Breastfeeding was asso- 11.58), 0.67 (95% CI: 0.18-2.59) and 0.29 (95% CI: 0.09-0.99), with
ciated with reduced odds of ALL (OR 0.94, 95% CI 0.88-0.99); this a change in HR of 1.01 (95% CI: 0.96-1.05), 0.92 (95% CI: 0.84-1.00,
association was stronger for breastfeeding >6 months (OR 0.85, 95% p=0.062) and 0.88 (95% CI: 0.78-1.00, p=0.044) per dose. For vaccina-
CI 0.79-0.92) and strongest for exclusive breastfeeding >6 months tion types, no statistically significant associations were found, except
(OR 0.70, 95% CI 0.53-0.92). Breastfeeding was associated with non- for the pneumococcal vaccine being associated with a decreased risk
significantly reduced odds of AML (OR 0.91, 95% CI 0.81-1.04); this of other/unspecified leukemia (HR: 0.32; 95% CI: 0.14-0.74). When
association was statistically significant for breastfeeding ≥4 months including a latency period of 6 months, no statistically significant
(0.82, 95% CI 0.71-0.95). We found no evidence that breastfeeding was associations were observed, and decreased risks were attenuated.
associated with CBT or evaluated subtypes, regardless of duration. We Conclusions: Our findings could not support a protective effect of
observed similar associations in meta-analyses. childhood vaccinations for the risk of leukemia in children, especially
Conclusions: Breastfeeding was associated with reduced odds of ALL for the lymphoid types.
and AML but not CBT. Importantly, we found that exclusive breastfeed-
ing was associated with a greater reduction in the risk of ALL, which has
not ben demonstrated previously in a large, international study. Breast- O207 / #226 OUTCOME DISPARITIES IN CHILDREN,
feeding should be encouraged for leukemia prevention, but additional ADOLESCENTS AND YOUNG ADULTS WITH
research will be necessary to identify modifiable risk factors for CBT MEDULLOBLASTOMA: A POPULATION-BASED ANALYSIS
and facilitate prevention efforts.
Isabel Genecin1 , Hanna Moisander-Joyce2 , Oscar Padilla3 , Hanna
Minns2 , James Garvin2 , Luca Szalontay2 , Cheng-Chia Wu3 , Justine
O206 / #1749 THE ASSOCIATION BETWEEN CHILDHOOD Kahn4 , Robyn Gartrell2
VACCINATIONS AND RISK OF LEUKEMIA IN CHILDREN 1 Columbia University, Vagelos College Of Physicians And Surgeons, New
York, United States of America; 2 Columbia University Irving Medical
Marie Hargreave1 , Susanne Kjaer1 , Friederike Erdmann2,3 , Aya Center, Department Of Pediatrics – Division Of Pediatric Hematol-
Alberts1 ogy/oncology/stem Cell Transplantation, New York, United States of Amer-
1 The Danish Cancer Society Research Center, Virus, Lifestyle And Genes, ica; 3 Columbia University Irving Medical Center, Department Of Radiation
Copenhagen, Denmark; 2 Institute of Medical Biostatistics, Epidemiology Oncology, New York, United States of America; 4 Columbia University Irving
and Infomatics (IMBEI), University Medical Center of the Johannes Guten- Medical Center, Pediatric Hematology, Oncology And Stem Cell Transplant,
berg University, Childhood Cancer Epidemiology, Mainz, Germany; 3 Danish New York, United States of America
Cancer Society Research Center, Childhood Cancer Research Group, Copen-
hagen, Denmark Background and Aims: Medulloblastoma (MB) is the most common
high-grade primary brain tumor in children. Recent registry-based
Background and Aims: A protective effect of vaccinations on studies in children with central nervous system (CNS) tumors have
leukemia risk in children has been hypothesized, in particular for demonstrated that survival outcomes differ by race/ethnicity in mul-
lymphoid leukemia. Findings have, however, been inconsistent and tivariable analyses, with Hispanic patients having highest hazard of
often based on small samples and self-reported information. Using death overall.
nationwide Danish registry data, our aim was to examine associa- Methods: To investigate this finding in MB patients, we exam-
tions between childhood vaccinations and risk of childhood leukemia ined survival in children (0-14 years) and adolescent/young adults
(<20 years). (15-39 years) with MB from 2007-2016 in the 2018 Surveillance
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S102 of S635 ABSTRACTS

Epidemiology and End Results Program database, using Kaplan Meier tings with universal healthcare. Thus, we aimed at investigating this
analysis, log-rank test and Cox proportional hazard ratios (HR) with in a register-based cohort study combining data from Denmark and
95% confidence intervals (CI). Race and ethnicity were categorized Sweden.
according to the United States Census, with Hispanic ethnicity (yes/no) Methods: All children diagnosed with cancer at ages 0-19 years, dur-
analyzed separately from race (Black, White, Asian, Other). ing 1991-2014, were identified from the national cancer registers.
Results: Among 1612 patients, 81% were White, 9% were Black, 8% From national administrative registers we collected information on
were Asian or Pacific Islander, and 2% were from “other” or unknown parental social characteristics. We estimated odds ratios (OR) and 95%
racial groups. 28% of the cohort was of Hispanic ethnicity. Univariate confidence intervals (CI) of early mortality (i.e., death within three
analysis found that Black patients had a significantly higher hazard of months after cancer diagnosis) by parental education, income, employ-
death than White patients (HR=1.55, CI: 1.16 – 2.08, p=0.003). In con- ment, cohabitation, and country of birth using logistic regression. For
trast, Hispanic ethnicity was not significantly associated with outcome Swedish children with acute lymphoid leukemia (ALL), we obtained also
(HR=0.98, CI: 0.79-1.21, p=0.8). Medicaid or no insurance (vs. private) clinical characteristics from the Swedish Childhood Cancer Register.
were each significantly associated with higher risk of death; Medicaid Results: Among the 13,926 children diagnosed with cancer, 355 (2.5%)
(HR =1.23, CI = 1.01 - 1.51, p=0.041); Uninsured (HR=2.07, CI=1.41- died within three months. Early mortality was higher among the dis-
3.02, p=<0.001). Of the treatment modalities analyzed, patients who advantaged groups, with the most pronounced associations observed
received neither chemotherapy nor radiation experienced higher haz- for parental education (maternal: ORadj(Low vs High) 1.65 (95% CI 1.22-
ard of death than patients who received both treatments (HR=3.63, CI 2.23); paternal: ORadj(Low vs High) 1.35 (95% CI 0.97-1.88)), and maternal
2.78-4.76, p=<0.001). income (ORadj(Q1 vs Q4) 1.77 (95% CI 1.25-2.49)). We found attenuated
Conclusions: Consistent with observations in other cancers, racial dis- or null associations for parental social characteristics and later mortal-
parities are observed in patients with MB, with Black race conferring ity (i.e., death one to five years after diagnosis). Parental education and
increased risk of death. Public insurance was also significantly asso- income were not associated with most of the clinical characteristics at
ciated with death, as was not receiving combined-modality therapy. diagnosis of ALL.
Further work is needed to understand the multilevel factors impacting Conclusions: This population-based study revealed inequalities in early
diagnosis, treatment and outcome among children and AYAs with MB mortality from childhood cancer by social background, also in countries
and prospective studies are warranted. with universal healthcare. Social differences occurring early in the dis-
ease course, rather than later, might indicate differences in the timing
of diagnosis.
O208 / #1272 EARLY MORTALITY IN CHILDREN WITH
CANCER IN DENMARK AND SWEDEN: THE ROLE OF SOCIAL
BACKGROUND IN A SETTING WITH UNIVERSAL HEALTHCARE O209 / #751 MORTALITY AFTER A CANCER DIAGNOSIS IN
CHILDREN WITH CANCER-LINKED BIRTH DEFECTS: A
Hanna Mogensen1 , Friederike Erdmann2 , Luzius Mader3 , Mats SWEDISH REGISTER-BASED STUDY OF 40 YEARS
Talbäck1 , Thomas Tjørnelund Nielsen4 , Henrik Hasle5 , Mats Heyman6 ,
Jeanette Winther7,8,9 , Maria Feychting1 , Giorgio Tettamanti1 , Line Christina-Evmorfia Kampitsi, Hanna Mogensen, Maria Feychting,
Kenborg8 Giorgio Tettamanti
1 Karolinska Institutet, Institute Of Environmental Medicine, Stockholm, Karolinska Institutet, Institute Of Environmental Medicine, Stockholm,
Sweden; 2 University Medical Center of the Johannes Gutenberg Univer- Sweden
sity, Institute of Medical Biostatistics, Epidemiology and Infomatics (IMBEI),
Childhood Cancer Epidemiology, Mainz, Germany; 3 University of Bern, Background and Aims: Childhood cancer survival has tangibly
Institute Of Social And Preventive Medicine, Bern, Switzerland; 4 Danish improved over the past decades. Yet, how children with birth defects—
Cancer Society Research Center, Childhood Cancer Research Group, Copen- one of the factors most consistently associated with childhood
hagen, Denmark; 5 Aarhus University Hospital, Department Of Pediatrics cancer—fare is less clear. Evaluating outcomes in these malformations
And Adolescent Medicine, Aarhus, Denmark; 6 Karolinska Institutet, Depart- could help guide clinical practice and decision-making. Thus, we aimed
ment Of Women’s And Children’s Health, Stockholm, Sweden; 7 Aarhus to assess survival after a cancer diagnosis in children with distinct
University, Department Of Clinical Medicine, Faculty Of Health, Aarhus, groups of cancer-linked birth defects: congenital heart disease (CHD)
Denmark; 8 Danish Cancer Society Research Center, Childhood Cancer and neurofibromatosis type 1 (NF1).
Research Group„ Copenhagen, Denmark; 9 Aarhus University, Hospital, Methods: We identified the child population of Sweden with a can-
Aarhus, Denmark cer diagnosis during the years 1970–2015 (n=10,904) through the
National Cancer Register. Diagnoses of CHD (n=263) and NF1 (n=96),
Background and Aims: A growing number of studies have demon- along with recorded deaths in this population, were retrieved from
strated social inequalities in survival from childhood cancer, although Swedish population-based health registers. We evaluated the effect of
the underlying mechanisms remain poorly understood. Moreover, it is these defects on 5-year mortality after a cancer diagnosis using Cox
unclear if social inequalities are seen already for early mortality in set- proportional hazards regression models.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S103 of S635

Results: Among children with CHD, we observed increased 5-year mor- Results: Noticeably more or similar numbers of paediatric cancer
tality after a cancer diagnosis, especially lymphoma (HR 2.80, 95% CI patients were newly diagnosed each month throughout 2020 in com-
1.21–6.48) or neuroblastoma (HR 2.53, 95% CI 1.07–5.98). NF1, on the parison to 2015-2019. ASRs indicated a remarkable increase for
other hand, was associated with decreased 5-year mortality, driven by childhood cancer overall (ages 0-14: 10%, ages 0-17: 12% increase) and
a markedly better prognosis among children with a central nervous sys- across diagnostic groups for 2020 compared to 2015-2019. Estimated
tem (CNS) tumor (HR 0.10, 95% CI 0.03–0.40)—however, mortality risk ASRs for 2021 however suggested this was followed by a decline,
was elevated among children with soft tissue and other extraosseous with the exception for acute myeloid leukaemia and CNS tumours for
sarcomas (STS; HR 2.27, 95% CI 1.10–4.69). which the ASRs remained on the level of 2020. ASRs of lymphoid
Conclusions: In this large, register-based cohort study, children with leukaemia were still elevated compared to 2015-2019, while ASRs for
CHD fared worse after a cancer diagnosis, particularly lymphoma and lymphomas and non-CNS solid tumours have dropped so drastically
neuroblastoma. Children with NF1 experienced favorable five-year that they fell below the respective ASRs from 2015-2019. Results from
survival for some, but not all, tumors typically occurring in the NF1 the qualitative survey indicated that diagnostic processes, timeliness
context: a notably decreased mortality was observed in CNS tumors, of diagnosis, and delivery of treatment were hardly affected during
but outcomes in STS were not favorable. Understanding outcomes in the COVID-19 pandemic, but psychosocial supportive care and non-
children with cancer-linked birth defects is important in the wake of urgent appointments were considerably reduced during the lockdown
improved survival for these defects and childhood cancer both. periods.
Conclusions: Although it is reassuring that we found no signs of
missed or delayed childhood cancer diagnoses throughout 2020-2021,
O210 / #463 MORE CHILDHOOD CANCER DIAGNOSES the underlying reasons for the marked increase in incidence rates
THAN EXPECTED: INCIDENCE, TIME OF DIAGNOSIS AND in 2020 remain speculative. Continued close monitoring of incidence
DELIVERY OF HEALTHCARE AMONG CHILDREN WITH CANCER patterns may shed light on the underlying reasons and contribute to
IN GERMANY DURING THE COVID-19 PANDEMIC understanding disease aetiology.

Friederike Erdmann1,2 , Maike Wellbrock1 , Arndt Borkhardt3 , Claudia


Trübenbach1 , Claudia Spix1 , Martin Schrappe4 , Desiree Grabow1 , NURSING
Joachim Schuz2 , Michael Eichiner5,6
1 Institute of Medical Biostatistics, Epidemiology and Infomatics (IMBEI), NURSING SYMPOSIUM: BASELINE NURSING STANDARDS
University Medical Center of the Johannes Gutenberg University, Child- 30-09-2022 4:30 PM - 5:30 PM
hood Cancer Epidemiology, Mainz, Germany; 2 International Agency for
Research on Cancer, Environment And Lifestyle Epidemiology Branch, Lyon, O211 / #1175 FOUNDATION NURSE TRAINING
France; 3 Heinrich Heine University, Medical Faculty, Düsseldorf, Paediatric PROGRAMME FOR PAEDIATRIC ONCOLOGY NURSES IN
Oncology, Haematology And Clinical Immunology, Düsseldorf, Germany; SUB-SAHARAN AFRICA: ‘TRAINING OF TRAINER’S EVALUATION
4 Christian-Albrechts-University and University Medical Center Schleswig-
Holstein, Department Of Pediatrics And Adolescent Medicine, Kiel, Ger- Elianeth Kiteni1 , Glenn Mbah Afungchwi2,3 , Rachel Hollis4
many; 5 Institute of Medical Biostatistics, Epidemiology and Informatics 1 Muhimbili National Hospital, Pediatrics, Dar Es Salaam, Tanzania;
(IMBEI), University Medical Center Mainz, Division Of Pediatric Epidemiol- 2 Cameroon baptist convention health services, Pediatria Oncology,
ogy, Mainz, Germany; 6 Medical Faculty Mannheim, Heidelberg University, Bamenda, Cameroon; 3 Cameroon Baptist Convention Health Services,
Center For Preventive Medicine And Digital Health Baden-wuerttemberg Pediatric Oncology, Mbingo, Cameroon; 4 Leeds Teaching Hospitals NHS
(cpd-bw), Mannheim, Germany Trust, Nursing, Leeds, United Kingdom

Background and Aims: The unprecedented detrimental conse- Background and Aims: The Sub-Saharan African Nursing Network
quences of the COVID-19 pandemic on cancer care and timely diag- developed a quality assured ‘Foundation’ training programme and
nosis are of increasing concern. We investigated the impact of the learning package for pediatric oncology nursing based on a consen-
COVID-19 pandemic on incidence, time of diagnosis and delivery of sus survey of nurses in the region. The programme aligns to the SIOP
healthcare among paediatric oncology patients in Germany during the Nursing Baseline Standards and has 12 modules. The learning pack-
COVID-19 pandemic. age has core presentations for each module, an aligned competence
Methods: We assessed incident cancer diagnoses in children aged 0-17 framework, and facilitator guide for trainers. Two ‘training of train-
years in Germany in 2020 and 2021 using data of the German Child- ers’ workshops were conducted in Ghana and Malawi. The aim of this
hood Cancer Registry and compared age-standardised incidence rates study was to evaluate the core materials developed, and the ‘training
(ASR) to those of the previous five years (2015–2019). Moreover, we of trainer’ workshops.
conducted a survey with open-ended questions, gathering perceptions Methods: An evaluation was carried out at the end of each workshop.
of the diagnostic process and healthcare delivery during the COVID-19 In Ghana a self-administered evaluation form was completed by par-
pandemic. ticipants; in Malawi a survey link for evaluation was provided and data
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S104 of S635 ABSTRACTS

collected in real time through REDCap. The same questions were used and survivin inhibitors, had substantially higher potency and selectiv-
in both surveys. A 4-point Likert scale was used to evaluate responses. ity over standard chemotherapeutic agents. New agents approved for
Data were analysed using SPSS. adult AML were essentially inactive in pediatric AML. Drug sensitivity
Results: Twenty six nurses from 7 different countries were trained to ex vivo accurately predicted single-agent and combinatorial activities
be trainers. The overall evaluation of the workshop was positive with with cytarabine in cell line- and patient-derived xenografts. Targeted
79% of participants from Ghana rating it as excellent (66% Malawi) resequencing revealed novel mutations of prognostic relevance, such
and 21% as good (33% Malawi). In evaluating the materials presented as KMT2C, in pediatric AML and their vulnerability to targeted agents.
over 50% of participants rated them as excellent. The organisation RNA-sequencing identified distinct gene expression signatures shap-
of the workshops, their structure, content and facilitation were rated ing the response to individual drugs. Administration of venetoclax and
as excellent or good by all participants although the majority recom- bortezomib to two children with refractory AML resulted in rapid blast
mended more time allocation. Participants identified content on how clearance and bridged to curative HSCT.
to deliver training, and the management of paediatric oncology emer- Conclusions: We conducted integrated drug and genomic profiling of
gencies as notably useful. All participants stated their intention to take patient biopsies to build the functional genomic landscape of pedi-
and use the training package in their own institutions. atric AML. Feasibility of adopting precision medicine was successfully
Conclusions: The ‘train the trainer’ workshops conducted in Ghana demonstrated in the clinic. In addition to scientific advancement, the
and Malawi demonstrated that the programme and learning package study will likely reshape the traditional approach for management of
is applicable to various settings in Sub- Saharan Africa and provides children with high-risk AML.
foundation level knowledge and skills to nurses caring for children with
cancer.
FREE PAPER SESSION (FPS)

AWARD SESSION FPS 16: NEUROBLASTOMA NEW TREATMENTS AND CLINICAL


STUDIES 01-10-2022 8:00 AM - 9:30 AM
SCHWEISGUTH PRIZE LECTURE 30-09-2022 4:30 PM - 5:00 PM
O213 / #732 PHASE 1 STUDY OF INTRAVENTRICULAR
O212 / #970 INTEGRATIVE DRUG AND GENOMIC 131I-OMBURTAMAB TARGETING B7H3 (CD276)-EXPRESSING
PROFILING IDENTIFY THERAPEUTIC VULNERABILITIES AND CNS MALIGNANCIES
INFORM PRECISION MEDICINE FOR PEDIATRIC ACUTE
MYELOID LEUKEMIA Shakeel Modak1 , Neeta Pandit-Taskar2 , Brian Kushner3 , Pat
Zanzonico4 , John Humm4 , Maria Donzelli3 , Jason Lewis4 , Serge
Han Wang, Kathy Yuen Yee Chan, Yi Lee Po, Alex Wing Kwan Leung, Lyashchenko4 , Bae Chu2 , Stephen Larson2 , Nai-Kong Cheung3 , Kim
Chi Kong Li, Kam Tong Leung Kramer1
The Chinese University of Hong Kong, Medical Sciences, Hong Kong, Hong 1 MSKCC, Pediatrics, New York, United States of America; 2 Memorial Sloan
Kong PRC Kettering Cancer Center, Radiology, New York, United States of America;
3 Memorial Sloan Kettering Cancer Center, Pediatrics, New York, United
Background and Aims: Background/Aims: Outcomes of children with States of America; 4 Memorial Sloan Kettering Cancer Center, Zuckerman
acute myeloid leukemia (AML) remain suboptimal. Implementation of Research Center, New York, United States of America
targeted therapy based purely on genomics is challenging due to the
complex mutational patterns and scarcity of agents for most lesions. Background and Aims: The prognosis for metastatic tumors of the
This study aims to adopt a functional approach to establish the first central nervous system (CNS) remains dismal, and the need for newer
pediatric AML-specific drug sensitivity pattern and identify candidates therapeutic modalities is critical. The cell-surface glycoprotein B7H3
of immediate clinical relevance. is expressed on a range of solid tumors with restricted expression
Methods: A high-throughput drug screening, comprising 39 targeted on normal tissues. We hypothesized that compartmental radioim-
agents and 6 conventional chemotherapeutics, was performed on 46 munotherapy (cRIT) with the anti-B7H3 murine monoclonal antibody
pediatric AML specimens using an optimized ex vivo culture system. omburtamab injected intraventricularly could safely target CNS malig-
The robustness of drug testing platform for predicting in vivo activities nancies.
was validated in xenograft models. Genomic profiling was complemen- Methods: We conducted a phase I trial of intraventricular 131 I-

tarily performed to identify the genetic markers and mechanisms of omburtamab (clinicaltrials.gov NCT00089245) using a standard 3+3
drug sensitivity/resistance. Patients with refractory AML were treated design. Eligibility criteria included adequate cerebrospinal fluid (CSF)
with targeted agents based on drug profiling results, and assessed for flow, no major organ toxicity, and for patients >dose level 6, avail-
clinical responses. ability of autologous stem cells. Patients initially received 74 MBq
Results: Unsupervised clustering revealed distinct clusters of drug radioiodinated omburtamab to evaluate dosimetry followed by thera-
response. Targeted agents, including Bcl-2, HDAC, proteasome, HSP peutic 131 I-omburtamab dose-escalated from 370-2960 MBq. Toxicity
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S105 of S635

was graded using CTCv 3.0. Dosimetry was evaluated using serial CSF designed to compare EFS outcomes with rates from the Phase 3
and blood sampling, and PET or gamma-camera scans. Patients could Children’s Oncology Group ch14.18 immunotherapy trial, ANBL0032.
receive a second cycle in the absence of grade 3/4 non-hematologic Methods: A DFMO phase 2 trial enrolled a total of 140 HRNB patients
toxicity or progressive disease. at the completion of disease treatment from 2012 to 2016. Patients
Results: 38 patients received 100 radioiodinated omburtamab injec- received 2 years of DFMO and were followed for 7 years. ANBL0032
tions. Diagnoses included metastatic neuroblastoma (n=16) and other enrolled a total of 1328 HRNB patients from 2001 to 2015 who were
B7H3-expressing solid tumors (n=22). 35 patients received at least assigned to treatment with ch14.18 immunotherapy and followed for
1 cycle of treatment with both dosimetry and therapy doses. Acute 10 years. With FDA input, selection rules were defined to identify like
toxicities included <grade 4 self-limited headache, vomiting or fever, groups of treated and control patients. The Kaplan-Meier method and
and biochemical abnormalities. Grade 3/4 thrombocytopenia was the Cox regression analyses were used to compare EFS and overall survival
most common hematologic toxicity. Recommended phase 2 dose was (OS). Additional analysis utilizing propensity score matching (PSM) was
1850MBq/injection. Median radiation dose to the CSF and blood was performed to balance cohorts on baseline demographic and disease
1.01 and 0.04 mGy/MBq respectively, iindicating a high therapeutic characteristics, matching each treated patient with the 3 closest scored
advantage for CSF. Major organ exposure was well below maximum control patients.
tolerated levels. In patients developing anti-drug antibodies (38%), Results: A total of 92 treated patients and 852 control patients met
blood clearance, and therefore therapeutic index was significantly selection criteria. Eighty-seven of the treated group had verified par-
increased. In patients receiving cRIT for neuroblastoma, survival was ticipation in ANBL0032 immediately prior to enrollment in the DFMO
markedly increased (median PFS 7.5 years) compared to historical trial. EFS from end of immunotherapy was significantly improved in the
data. DFMO group (n = 92) vs. control group (n = 852), with hazard ratio
Conclusions: cRIT with 131 I-omburtamab is safe, has favorable dosime- of 0.50 (95% CI: 0.29, 0.84) and p-value of 0.0083. Four-year EFS was
try and may have a therapeutic benefit as adjuvant therapy for 83.7% (95% CI 74.4, 89.8) in the DFMO group versus 71.7% (95% CI
B7-H3-expressing leptomeningeal metastases. 68.5, 74.7) in the control group. OS and PSM analyses demonstrated
consistent results.
Conclusions: Patients treated with DFMO after standard upfront ther-
O214 / #1750 COMPARISON OF EVENT FREE SURVIVAL apy had approximately half the risk of relapse compared to control
(EFS) IN HIGH-RISK NEUROBLASTOMA (HRNB) PATIENTS patients. The PSM comparisons support the benefit of DFMO as a
RECEIVING EFLORNITHINE (DFMO) MAINTENANCE TO AN maintenance treatment for HRNB.
EXTERNAL CONTROL IMMUNOTHERAPY STUDY DATABASE

Javier Oesterheld1 , Genevieve Bergendahl1 , Donald Berry2 , Elizabeth O215 / #1297 RESULTS OF THE DINUTUXIMAB BETA,
Lorenzi2 , Thomas Clinch3 , Jacqueline Kraveka4 , William Ferguson5 , BEVACIZUMAB, IRINOTECAN AND TOPOTECAN
Valerie Brown6 , Don Eslin7 , Derek Hanson8 , Virginia Harrod9 , RANDOMISATIONS OF THE BEACON-NEUROBLASTOMA PHASE
Michael Isakoff10 , Deanna Mitchell11 , Randy Wada12 , Peter Zage13 , 2 TRIAL BY ITCC AND SIOPEN
Giselle Saulnier Sholler1
1 Levine Children’s Hospital, Pediatrics, Charlotte, United States of Amer- Lucas Moreno1 , Rebekah Weston2 , Cormac Owens3 , Juliet Gray4 ,
ica; 2 Berry Consultants, Statistics, Austin, United States of America; Giuseppe Barone5 , Dominique Valteau-Couanet6 , Alba Rubio San
3 USWorldMed, Oncology, Louisville, United States of America; 4 Medical Simon7 , Guy Makin8 , Sucheta Vaidya9 , Marion Gambart10 , Victoria
University of South Carolina, Pediatrics, Charleston, United States of Amer- Castel11 , Natasha Van Eijkelenburg12 , Karsten Nysom13 , Genevieve
ica; 5 Cardinal Glennon Children’s Hospital, Pediatrics, St. Louis, United Laureys14 , Ruth Ladenstein15 , Aurora Castellano16 , Nicolas Gerber17 ,
States of America; 6 Penn State Milton S. Hershey Medical Center and Jennifer Laidler2 , Pamela Kearns2 , Keith Wheatley18
Children’s Hospital, Pediatrics, Hershey, United States of America; 7 St. 1 Vall d’Hebron University Hospital, Paediatric Oncology And Haematology,

Joseph’s Children’s Hospital, Pediatrics, Tampa, United States of America; Barcelona, Spain; 2 University of Birmingham, Cancer Research Uk Clini-
8 Hackensack University Medical Center, Pediatrics, Hackensack, United cal Trials Unit, Birmingham, United Kingdom; 3 Children’s Health Ireland,
States of America; 9 Dell Children’s Hospital, Pediatrics, Austin, United States National Oncology/ Bone Marrow Transplant Centre And Haematology
of America; 10 Connecticut Children’s Medical Center, Pediatrics, Hartford, Department, Dublin, Ireland; 4 University of Southampton, Cancer Sciences
United States of America; 11 Helen Devos Children’s Hospita;, Pediartics, Unit, Southampton, United Kingdom; 5 Great Ormond Street Hospital for
Grand Rapids, United States of America; 12 Kapiolani Medical Center, Pedi- Children NHS Foundation Trust, Paediatric Oncology, London, United King-
atrics, Honolulu, United States of America; 13 University of California San dom; 6 Institute Gustave Roussy, Department Of Paediatrics And Adoles-
Diego School of Medicine, Pediatrics, La Jolla, United States of America cents, Villejuif, France; 7 Hospital Niño Jesus, Oncología Pediátrica, Madrid,
Spain; 8 University of Manchester, Division Of Cancer Sciences, Manchester,
Background and Aims: Long term survival in HRNB patients remains United Kingdom; 9 Royal Marsden Hospital, Children And Young Peoples
a challenge with relapse as the primary cause of mortality. DFMO Unit, Surrey, United Kingdom; 10 CHU Toulouse, Hôpital des Enfants„ Pedi-
has been evaluated as a maintenance therapy in a single arm study atric Hemato-oncology Unit, Toulouse, France; 11 Hospital La Fe, Paediatric
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S106 of S635 ABSTRACTS

Oncology, Valencia, Spain; 12 Princess Máxima Center for Pediatric Oncol- Kim Kramer1 , Jaume Mora2 , Melissa Bear3 , Pat Zanzonico4 , Neeta
ogy, Department Of Solid Tumors, Utrecht, Netherlands; 13 Rigshospitalet, Pandit-Taskar5 , Maria Düring6 , Søren Fabricius7 , Keri Streby8 , John
Paediatric Oncology, Copenhagen, Denmark; 14 University of Ghent, Pae- Rømer9
diatric Oncology, Ghent, Belgium; 15 Children’s Cancer Research Institute, 1 Memorial Sloan Kettering Cancer Center, Pediatrics, New York, United
Paediatric Oncology, Vienna, Austria; 16 Ospedale Bambino Gesù IRCCS, States of America; 2 Pediatric Cancer Center Barcelona, Hospital Sant Joan
Dipartimento Di Onco-ematologia E Terapia Cellulare E Genica, Rome, Italy; de Deu, Oncology, Barcelona, Spain; 3 Riley Hospital for Children Indiana
17 University Children’s Hospital, Department Of Oncology, Zurich, Switzer- University, Pediatrics, Indianapolis, United States of America; 4 Memorial
land; 18 Institute of Cancer and Genomic Sciences, College of Medical and Sloan Kettering Cancer Center, Zuckerman Research Center, New York,
Dental Sciences, University Of Birmingham, Birmingham, United Kingdom United States of America; 5 Memorial Sloan Kettering Cancer Center, Radiol-
ogy, New York, United States of America; 6 Y-mAbs Therapeutics, Biometrics,
Background and Aims: The BEACON phase II trial (NCT02308527) Hørsholm, Denmark; 7 Y-mAbs Therapeutics, Clinical Operations, Hørsholm,
addressed multiple questions in children with relapsed/refractory Denmark; 8 Nationwide Children’s, Hematology & Oncology, Columbus,
high-risk neuroblastoma (RR-HRNB). We present the dinutuximab United States of America; 9 Y-mAbs Therapeutics, Medical, Hørsholm,
beta (dB) randomisation and report progression-free survival (PFS) Denmark
results for all trial randomisations (bevacizumab, irinotecan, topotecan
and dB). Background and Aims: Up to 10% of patients with stage 4 neuroblas-
Methods: Following a factorial multi-arm design, patients aged 1-21 toma develop CNS/leptomeningeal metastases (LM); limited curative
years with RR-HRNB were randomised to 8 arms containing temozolo- therapies exist. Omburtamab is a murine monoclonal antibody that
mide, irinotecan-temozolomide or topotecan-temozolomide alone or targets B7-H3, a transmembrane glycoprotein highly expressed in
in combination with bevacizumab or dB. Primary outcome measure neuroblastoma and other solid tumors. Omburtamab can be labeled
was best response (complete or partial) during the first 6 courses of with iodine-131 and administered into the cerebrospinal fluid via an
treatment, by RECIST or International Neuroblastoma Response Cri- indwelling catheter to achieve a high CSF:blood ratio. We report a
teria for patients with measurable and evaluable disease respectively. planned interim analysis for Trial 101, a phase-2/3 trial of intraventric-
PFS, overall survival (OS) and safety were secondary outcomes. ular 131 I-omburtamab in patients with refractory or relapsed CNS/LM
Results: From 2013 to 2021, 225 patients were randomised as fol- neuroblastoma.
lows: bevacizumab (n=160), irinotecan (n=120), topotecan (n=80) Methods: Trial 101 (NCT03275402) is a single-arm, international
and dB (n=65) randomisations. Baseline characteristics were balanced study (4 US and 1 Spain) in patients aged 0-18 years with radiograph-
between arms. For the dB randomisation, the objective response ically and/or histologically confirmed CNS/LM neuroblastoma. Treat-
rate was 18% with chemotherapy alone and 35% for patients receiv- ment consisted of a 2 mCi 131 I-omburtamab dosimetry dose followed
ing chemotherapy with dB (risk ratio 1.66, 95% confidence interval one week later with 50 mCi 131 I-omburtamab and 4-week observation
(CI) 0.65 to 4.24, 1p=0.19). 1-year PFS was 27% for chemotherapy period. Up to 2 therapy injections were permitted. The primary end-
alone, and 57% for those receiving chemotherapy+dB (HR 0.63, 95% point was CNS/LM progression-free survival (PFS) at 6 months with
CI 0.32 to 1.25, p=0.19). Nine (41%) patients receiving chemother- overall survival (OS) at 12 months as a secondary endpoint. Long-term
apy alone and 13 (30%) receiving chemotherapy+dB had grade follow-up is ongoing.
≥3 toxicities (CTCAE v4.0). Neurotoxicities were more common in Results: As of 1 June 2021, 32 patients were included in the full effi-
patients receiving dB compared to chemotherapy alone (Grade 1- cacy and safety analysis, of which 26 received a dosimetry dose. The
2: 67.4% vs 13.6%, Grade 3: 9.3 vs 0%). Hazard ratios for PFS for estimated CNS/LM PFS at 6 months was 77.2% (95% CI 58.0-88.4),
all other randomisations were: bevacizumab HR=0.84 (95%CI 0.56- and 12-month OS was 73.5% (95% CI 53.8-85.8). There were 19 seri-
1.27), irinotecan HR=0.58 (95%CI 0.38-0.88) and topotecan HR=0.64 ous treatment-emergent adverse events in 13 patients mostly related
(95%CI 0.38-1.08). Within this phase 2 trial, success criteria for to myelosuppression. One patient suffered a fatal intracranial hem-
PFS were met by the irinotecan, bevacizumab and dinutuximab beta orrhage occurring 7 weeks after 131 I-omburtamab treatment in the
randomisations. setting of grade 4 thrombocytopenia.
Conclusions: The BEACON Neuroblastoma trial evaluated three Conclusions: Interim results suggest that targeted radioimmunother-
chemotherapy regimens and two novel agents, identifying promis- apy with intraventricular 131 I-omburtamab may provide a survival
ing combinations for future trials in relapsed neuroblastoma benefit for patients with neuroblastoma and CNS/LM metastases.
patients.

O217 / #393 PROGNOSIS OF INFANTS DIAGNOSED WITH


O216 / #513 RADIOIMMUNOTHERAPY WITH NEUROBLASTOMA DURING 2000-2009 IN SIX EUROPEAN
INTRAVENTRICULAR 131I-OMBURTAMAB IN PATIENTS WITH COUNTRIES
CENTRAL NERVOUS SYSTEM (CNS) NEUROBLASTOMA:
INTERIM RESULTS FROM A MULTINATIONAL TRIAL Eva Steliarova-Foucher1 , Murielle Colombet1 , Jacqueline Clavel2,3 ,
Andrea Gini1 , Juliet Gray4 , Lucy Irvine5 , Claudia Kuehni6 , Brigitte
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S107 of S635

Lacour3,7 , Milena Maule8 , Rafael Peris-Bonet9 , Paola Quarello10 , diagnosis, respectively. Infants with metastatic disease (N=406 of 941)
Ramya Ramanujachar11 , Shelagh Redmond6 , Christina Schindera12 , had a poor prognosis (HR=7.02, p<0.0001). OS was 98.9% (97.6, 99.5)
Claudia Spix13 , Charles Stiller14 , Deborah Tweddle15 , Kate Wheeler16 , with stage 1 and 2 (localised, N=599), 95.9% (92.8, 97.6) with stage 3
Adela Canete9 , Kathy Pritchard-Jones17 (N=291), 87.4% (83.6, 90.4) with stage 4S (N=369) and 75.0% (69.3,
1 International Agency for Research on Cancer, Cancer Surveillance Branch, 79.9) with stage 4 (N=261). Localised tumours represented between
Lyon, France; 2 Groupe Hospitalier Universitaire Paris-Sud, Assistance 21% (Switzerland) and 49% (Italy). MYCN was amplified in 12.5% (96 of
Publique Hôpitaux de Paris (AP-HP), Registre National Des Cancers De 771 studied) patients, whose OS was 48.6% (38.2,58.2) and HR=12.1
L’enfant, Villejuif, France; 3 Université Paris Cité, INSERM UMR 1153, (8.0,18.4).
Epidémiologie Des Cancers Des Enfants Et Des Adolescents (epicea), Paris, Conclusions: Principal predictors of survival were MYCN amplification
France; 4 University of Southampton, Cancer Sciences Unit, Southampton, and stage at diagnosis. Enriching registry data with clinical records is
United Kingdom; 5 NHS Digital, National Cancer Registration And Anal- a labour intensive, but a feasible method to evaluate prognosis of rare
ysis Service, London, United Kingdom; 6 University of Bern, Institute for diseases.
Social and Preventive Medicine, Swiss Childhood Cancer Registry, Bern,
Switzerland; 7 CHRU Nancy, Registre National Des Cancers De L’enfant,
Vandœuvre-lès-Nancy, France; 8 University of Torino, Cancer Registry Of O218 / #1311 CORRELATION WITH DISEASE BURDEN AND
Piedmont, Torino, Italy; 9 University of Valencia, Spanish Registry Of Child- EARLY DETECTION OF RECURRENCE USING A NOVEL
hood Tumours (reti-sehop), Valencia, Spain; 10 Regina Margherita Children’s NUCLEOSOME-BASED PLASMA BASED ASSAY IN
Hospital, University of Turin, Paediatric Onco-haematology, Turin, Italy; LONGITUDINALLY MONITORED NEUROBLASTOMA PATIENTS
11 University Hospital Southampton, Paediatric Oncology, Southampton,
United Kingdom; 12 University Children’s Hospital, Paediatric Haematol- Laura Rey Portela1 , Courtney Himsworth2 , Reda Stankunaite3 , Jake
ogy Oncology, Basel, Switzerland; 13 University Medical Center of the Micallef4 , Giuseppe Barone1 , Catriona Duncan1 , Tanzina Chowdhury1 ,
Johannes Gutenberg University, Institute of Medical Biostatistics, Epidemi- Paola Angelini5 , Debbie Hughes3 , Lorenzo Biassoni6 , Myuri
ology and Informatics (IMBEI), Childhood Cancer Epidemiology, Mainz, Gangaram7 , Michael Hubank3,5 , Louis Chesler3,5 , John Anderson1,2
Germany; 14 NHS Digital, National Cancer Registration And Analysis Service, 1 Great Ormond Street Hospital, Oncology, London, United Kingdom; 2 Great

Oxford, United Kingdom; 15 Newcastle University, Clinical And Translational Ormond Street Institute of Child Health, Developmental Biology And Can-
Research Institute, Newcastle Centre For Cancer, Newcastle upon Tyne, cer, London, United Kingdom; 3 Institute of Cancer Research, Centre For
United Kingdom; 16 Oxford University Hospital NHS Foundation Trust, Pae- Paediatric Oncology Experimental Medicine, London, United Kingdom;
diatric Haematology And Oncology, Oxford, United Kingdom; 17 University 4 Volition, Volition Nucleosomics Technology, Isnes, Belgium; 5 Royal Mars-
College London, Ucl Great Ormond Street Institute Of Child Health, London, den Hospital, Children And Young Peoples Unit, Surrey, United Kingdom;
United Kingdom 6 Great Ormond Street Hospital, Radiology, London, United Kingdom; 7 UCL,

Institute Of Child Developmental Biology And Cancer, London, United


Background and Aims: SIOPEN data from the INES trial (1999- Kingdom
2004) revealed a 5-year overall survival (OS) between 100% in infants
without risk factors and 27% in infants with MYCN amplification. Background and Aims: Neuroblastoma (NB) has a high incidence of
We examined population-based survival of infants (age<1 year) with treatment failure and relapse. Early detection, and monitoring of recur-
neuroblastoma in six countries. rent disease through monitoring of disease status through blood borne
Methods: Records of infants registered with neuroblastoma in 2000- biomarkers could enable more specific and sensitive monitoring, whilst
2009 in the population-based cancer registries of England, France, minimising invasive tests. We hypothesised that circulating nucleoso-
Germany, Italy (Piedmont), Spain (selected regions) and Switzerland mal DNA attached to histone H3.1 could serve as a marker of cancer
were complemented from clinical records with detailed information burden suitable for diagnostic and longitudinal monitoring.
on diagnosis, stage, treatment and outcome. OS with 95% confidence Methods: We have collected serial plasma samples at diagnosis and
interval (95%CI) was calculated using the life-table method and dif- through treatment from a total of 66 children with solid tumours. Using
ferences in outcome between the patients’ subgroups assessed by a novel antibody based immunoassay (Volition nucleosome assay), we
hazard ratio (HR) of death and its 95%CI using Cox models adjusted for measure cell-free DNA associated with histone H3.1 and expressed
country. results as normalised nucleosome concentration. Cell free DNA was
Results: Overall, 1683 infants were diagnosed: between 10% (Italy) also evaluated for tumour-specific mutations using panel and low
and 28% (Germany) in the first month of life. Patients (99%) were coverage whole genome sequencing.
treated in paediatric oncology units. Mean follow-up of survivors was Results: There is a significant reduction in plasma nucleosome concen-
9.9 years. OS for N=1665 infants with follow-up was 91.5% (90.1,92.8) tration between diagnosis and treatment, and between on treatment
and did not differ by country. Girls (N=748, HR=0.7, p=0.03) had bet- and end of treatment groups (2way ANOVA: Diagnosis vs Treatment
ter survival than boys (N=917). Prenatal (N=95, HR=0.4, p=0.06) and =P=<0.0001, Diagnosis vs EOT = P=0.0001). For six neuroblastoma
incidental (N=188, HR=0.5, p=0.006) diagnosis was associated with patients, we have correlated disease burden assessed at different
a better prognosis than postnatal (N=759) or symptomatic (N=661) time points by using MIBG scores with nucleosome values, and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S108 of S635 ABSTRACTS

demonstrated close correlation in all patients. Phenomenologically we of ≤ 0.05 was considered to indicate a statistically significant as
identify rises in nucleosome values that suggest subsequent relapse as sociation.
determined by MIBG scan. Results: We interviewed the parents of 356 children with cancer.
Conclusions: The levels of ctDNA determined by the Volition chro- The median patient delay was 14 days (IQR 6–46.5 days. The most
matin assay correlate well with disease appearance, response to extended delay was in patients with malignant bone tumors (median
treatment and could possibly predict relapse. Moreover, this is an 66, IQR: 14–126). In the multivariable logistic regression analysis, solid
accessible test as ELISAs are already installed in clinical laboratories cancer [OR =5.22, 95% confidence interval (CI): 2.79–9.77, P <0.001],
and therefore could readily be translated to standard of care. Data on and the use of alternative medicine (OR =1.86, 95% CI: 1.13–3.08, P
the same samples assessed by whole genome sequencing and panel =0.015) were associated with patient delay.
sequencing are needed to determine relative sensitivity and specificity Conclusions: Type of cancer and use of alternative medicine are essen-
of nucleosome assessments with the sequencing-based technology. tial factors that cause patient delay more than age and other family
Nucleosome assessment could prove a high throughput and economi- socioeconomic factors. Increasing public knowledge and awareness,
cally viable method for early detection of neuroblastoma diagnosis and and improving access to care should be improved, especially in a
relapse. diagnosis of solid tumors.

FREE PAPER SESSION (FPS) O220 / #1317 IMPLEMENTING THE PEDIATRIC ONCOLOGY
FACILITY INTEGRATED LOCAL EVALUATION (PROFILE):
FPS 17: EPIDEMIOLOGY AND GLOBAL HEALTH 01-10-2022 LESSONS LEARNT FROM OUR FACILITY BETA TESTING
8:00 AM - 9:30 AM EXPERIENCE IN JORDAN

O219 / #740 TYPE OF CANCER AND ALTERNATIVE Sakher Obeidat1 , Iyad Sultan2 , Ghada Abu Alrub2 , Maha Alwawi3 ,
MEDICINE DETERMINE PATIENT DELAY EXPERIENCED BY Patricia Belda4 , Miriam Gonzalez-Guzman4 , Heather Brandt5 , Paola
CHILDREN WITH CANCER: A STUDY IN WEST JAVA Friedrich4 , Rawad Rihani6
INDONESIA 1 King Hussein Cancer Center, Pediatrics, Amman, Jordan; 2 king hussein
cancer center, Pediatric Oncology, Amman, Jordan; 3 King Hussein Cancer
Nur Sari1 , Sultan Devansyah2 , Ismiana Modjaningrat2 , Nur Center, Nursing, Amman, Jordan; 4 St. Jude Children’s Research Hospital,
Suryawan1 , Susi Susanah1 , Lulu Rakhmillah3 , Kurnia Wahyudi3 , Global Pediatric Medicine, Memphis, United States of America; 5 St Jude
Gertjan Kaspers4,5 Children’s Research Hospital, Epidemiology And Cancer Control, Mem-
1 Division of Hematology Oncology Dr. Hasan Sadikin Hospital Bandung, phis, United States of America; 6 King Hussein Cancer Center, Paediatric
Child Health Department, Bandung, Indonesia; 2 Universitas Padjadjaran, Hematology/oncology/bone Marrow Transplant, Amman, Jordan
Faculty Of Medicine, Bandung, Indonesia; 3 Faculty of Medicine Universitas
Padjadjaran, Public Health, Bandung, Indonesia; 4 Emma Children’s Hospi- Background and Aims: PrOFILE is a collaborative experience that
tal, Department Of Pediatric Oncology, Amsterdam, Netherlands; 5 Princess aims to help pediatric hematology-oncology (PHO) teams to define an
Máxima Center, Pediatric Oncology, Utrecht, Netherlands institutional improvement strategy and build local capacity in qual-
ity improvement (QI). In July 2021, the King Hussein Cancer Center
Background and Aims: The majority of patients with pediatric cancer (KHCC) team joined the second Full PrOFILE Beta Testing cohort.
in developing countries present at an advanced stage due to delayed We aim to describe our experience, and lessons learnt implementing
diagnostics, which has been recognized as an important barrier to PrOFILE.
effective care. The objective of the present study was to determine Methods: PrOFILE was implemented in three phases: preparation,
the influence of some clinical and social factors associated with patient assessment, and interpretation and action. We recruited our assess-
delay. ment team and selected three providers to complete QI Basic Certifi-
Methods: This cross-sectional study was conducted at Dr. Hasan cation during the preparation phase. Then, we collected the objective
Sadikin Hospital, using data retrieved from the Indonesian Pedi- and subjective data for the twelve PrOFILE modules (26 forms). Objec-
atric Cancer Registry (IP-Car) (2020–2021) for clinical variables, tive data was collected by the Site Coordinator (SC) and reviewed
completed with interviews with the parents that used structured and approved by the Physician Lead (PL). Subjective data was entered
questionnaires to obtain data on their sociodemographic character- directly by a pool of Point of Care (POC) staff. We conducted six QI
istics. Patient delay was defined as the number of days between the exercises and provided data validation and feedback for five short-
onset of the first symptoms associated with cancer, and the date the interval reports. Finally, we used our score-based and descriptive
patient came to the health facility for the first time. presented using reports to plan our local workshop and develop our institution’s action
the median with IQR and range (minimum and maximum,). Binary plan.
logistic regression analysis model was fitted to identify factors asso- Results: A total of 28 interdisciplinary healthcare providers formed
ciated with patient delay that classified through its median. A p-value the assessment team. Threeselected members completed the QI Basic
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ABSTRACTS S109 of S635

Certification. PL and SC attended a total of 33 mentoring sessions and stage. There was a negative correlation between SES and stage (Spear-
completed 14PrOFILE educational videos. Our form completion rate man rho= -0.178; p<0.001). Patients with solid tumours and lower
was 100% for both objective and subjective data. KHCC site scored SES showed proportionately higher numbers of stage III and IV dis-
74% for Context, 92% for Workforce, 94% for Diagnostic, 94% for ease (p<0.01). This proportion decreased with higher SES categories. In
Therapy, and 100% for Patient and Outcome components. We found the multivariate analyses adjusted for sex, age, tumour type and stage,
discrepancies in awareness level on institutional planning between higher SES was associated with a lower risk of death (p<0.001), indi-
POC staff (42%,range:15-100%) and PL and SC (100%), mainly in the cating that the impact of SES on survival was in excess of any effect
National context and Finances and Resources modules. that could be explained by lower stage disease alone. Five-year OS was
Conclusions: PrOFILE implementation was feasible at our institu- 85.3% in children from H3 households versus 47.7% in children from
tion. Having our institutional leaders’ support was essential. The tool H0 households (p<0.001).
helped us identify our strengths and prioritize opportunities amenable Conclusions: SES significantly impacts cancer survival in South African
for improvement. Multisite PrOFILE implementation is valuable for children. Advocacy to increase social support for the poor is essen-
regional and international benchmarking. tial if we are to achieve equitable improvements in childhood cancer
outcomes with standardised national protocols.

O221 / #1464 SOCIO-ECONOMIC STATUS IS A


DETERMINANT OF CHILDHOOD CANCER OUTCOMES IN O222 / #709 CANCER INCIDENCE AMONG CHILDREN IN
SOUTH AFRICAN CHILDREN UGANDA: A RETROSPECTIVE POPULATION-BASED STUDY,
2013-2017
Marc Hendricks1 , Annibale Cois2 , Jennifer Geel3 , Jaques Van
Heerden4 , Kirsten Donald5 , Mariana Kruger6 Annet Nakaganda1 , Racheal Angom2 , Jackson Orem3 , Joyce
1 University of Cape Town, Haematology/oncology Service, Red Cross War Kambugu2
Memorial Children’s Hospital, Cape Town, South Africa; 2 Stellenbosch Uni- 1 Uganda Cancer Institute, Cancer Epidemiology And Clinical Trials Unit,
versity, Division Of Health Systems And Public Health, Department Of Kampala, Uganda; 2 Uganda Cancer Institute, Pediatric Oncology, Kampala,
Global Health, Cape Town, South Africa; 3 University of Witwatersrand, Uganda; 3 Uganda Cancer Institute, Leadership And Management, Kampala,
Division Of Paediatric Haematology Oncology, Department Of Paediatrics Uganda
And Child Health, Charlotte Maxeke Johannesburg Academic Hospital,
Johannesburg, South Africa; 4 Antwerp University Hospital, Paediatric Background and Aims: Cancer is a leading cause of childhood mor-
Haematology and Oncology, Department of Paediatrics and Child, Antwerp tality in sub-Saharan Africa (SSA). Cancer control requires accurate
University Hospital Health, Antwerp, Belgium; 5 University of Cape Town, estimates of the burden however, estimates for children in the region
Division Of Developmental Paediatrics, Department Of Paediatrics And are based on only six national cancer registries, covering about 5% of
Child Health, Red Cross War Memorial Children’s Hospital, Cape Town, Africa’s children population. This analysis was conducted to estimate
South Africa; 6 Stellenbosch University and Tygerberg Hospital, Department cancer incidence and identify geographic variations among children
Of Paediatrics And Child Health, Cape Town, South Africa with cancer in Uganda.
Methods: From 2018 to 2020, with a retrospective catchment popula-
Background and Aims: Significantly discrepant survival rates have tion approach, we examined data from three regions (Central, Western
been documented in single disease childhood cancer cohorts in South and Eastern). Five districts from each region were selected using strat-
Africa, in which those from higher socioeconomic groups were shown ified random sampling. All newly diagnosed cancer cases from routine
to have a significantly lower risk of death than those from less affluent medical records of all health facilities serving the study populations in
households. This study aimed to determine the impact of socioeco- the period 2013-2017 were included. Cases were coded according to
nomic status (SES) on survival of children with cancer using pooled the International Classification of Diseases for Oncology (ICD-0-03).
South African data. Data was analysed using CanReg5.
Methods: Five databases spanning January 2000 to December 2021 Results: A total of 718 children (0-14 years) with cancer were
were interrogated. SES status was assigned based on a public sec- registered (390 males, 328 females). The overall age-standardized
tor annual household income classification. H0 households (formally incidence rate (ASIR) was 59.5 per million children. Most common
unemployed) received free healthcare. H1, H2 and H3 households cancers were lymphomas (ASIR of 15.6), soft tissue sarcomas (ASIR
paid relative to income. H3 households (highest income) earned more of 9.8), retinoblastoma (ASIR of 8.4), leukemia (ASIR of 7.0) and
than USD24,000 per year. The Spearman test was used to assess cor- renal tumors (ASIR of 6.6). Within the lymphoma group, 40% were
relations between SES and disease stage at diagnosis. Hazard ratios Burkitt lymphomas and in the soft tissue sarcomas group, 46 %
were determined using Cox regression modelling. The Kaplan-Meier were rhabdomyosarcoma and 43% were Kaposi sarcoma. Overall
procedure was used to estimate overall survival (OS) (CI 95%). ASIRs varied from 123.5 per 1,000,000 in Kampala capital city to
Results: Sixteen-hundred patients were eligible for analysis while 56.6 in central region; 41.5 in western region; and 30.5 in Eastern
1,269 solid tumour patients were compared for SES category and region.
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S110 of S635 ABSTRACTS

Conclusions: The incidence rates varied greatly among regions which O224 / #1806 ANALYSIS OF CHILDHOOD CANCER
may reflect existing disparities in children’s cancer services/diagnosis MEDICINE ACCESS IN FOUR EAST AFRICAN COUNTRIES
in Uganda. This study validates a retrospective catchment population
approach to improving cancer surveillance and the results will con- Kadia Petricca1 , Joyce Kambugu2 , William Macharia3 , Jessie
tribute to the development of national childhood cancer registration, Githang’A4 , Festus Njuguna5 , Angela Mcligeyo6 , Aimable
and provide evidence for improving national cancer control in Uganda. Kanyamuhunga7 , Rehema Laiti8 , Deogratias Katabalo9 , Kristin
Schroeder10 , Sumit Gupta11 , Avram Denburg12
1 Peter Gilgan Centre for Research and Learning, SickKids Hospital, Child
O223 / #1746 ACCURATELY FORECASTING PEDIATRIC Evaluative Sciences, Toronto, Canada; 2 Uganda Cancer Institute, Depart-
HEMATOLOGY AND ONCOLOGY PATIENT VOLUMES AT ment Of Paediatric Oncology, Kampala, Uganda; 3 Aga Khan University
TREATMENT CENTERS IN SUB-SAHARAN AFRICA Hospital, Pediatrics, Nairobi, Kenya; 4 University of Nairobi, Human Pathol-
ogy, Nairobi, Kenya; 5 Moi University, Pediatric Oncology, Eldoret, Kenya;
Mark Zobeck1 , Casey Mcatee2 , Hailey Zhang1 , Nmazuo Ozuah2 , Peter 6 Jaramogi Oginga Odinga Teaching & Referral Hospital, Oncology, Kisumu,
Wasswa3 , Robert Langat4 , Elise Ishigami1 , Parth Mehta1 Kenya; 7 College of Medicine and Health Sciences University of Rwanda,
1 Texas Children’s Hospital, Global Hope (hematology/oncology Pediatric Child Health, Kigali, Rwanda; 8 Muhimbili National Hospital, Oncology, Dar
Excellence), Houston, United States of America; 2 Baylor College of Medicine es Salaam, Tanzania; 9 Bugando Medical Centre, Pediatrics, Mwanza, Tanza-
Children’s Foundation, Malawi, Texas Children’s Global Hope, Lilongwe, nia; 10 Duke University Medical Center, Pediatric Oncology, Durham, United
Malawi; 3 Global HOPE, Pediatrics, Kampala, Uganda; 4 Baylor College of States of America; 11 Hospital for Sick Children, Haematology/oncology,
Medicine Childrens Foundation, Texas Children’s Hospital Global Hope Toronto, Canada; 12 The Hospital For Sick Children, Temerty Faculty of
Program, Gaborone, Botswana, Gaborone, Kenya Medicine, University of Toronto, Peditatric Hematology Oncology, Toronto,
Canada
Background and Aims: Forecasting the number of new patients at
pediatric hematology-oncology (PHO) treatment centers in resource- Background and Aims: Reliable access to essential childhood cancer
constrained settings is important to optimize care delivery but can medicines (CCMs) is key to improved childhood cancer outcomes in
be difficult due to the month-to-month variability of the counts and low- and middle-income countries (LMICs). We conducted an analysis
changes in the expected number over time. We conducted a forecast- of CCM access in four East African countries – Kenya, Rwanda, Tan-
ing project to predict the number of new hematology and oncology zania and Uganda – to determine the availability and cost of essential
patients at PHO treatment centers in Botswana (Botswana Treatment cancer medicines and impacts of medicine unavailability on treatment
Center; BTC), Malawi (MTC), and Uganda (UTC) in 2021. interruption for five common pediatric malignancies.
Methods: Monthly counts of new hematology and oncology patients Methods: We analyzed costs and stockout days for 34 essential drugs,
were obtained between October 2016–March 2021. Time series mod- based on 12 months of prospective price and inventory data from eight
els were fit to the data from Oct 2016-Dec 2020. Moving average institutions across the study jurisdictions. SIOP adapted-treatment
and linear trend models were fit to the data with different time peri- regimens were employed to assess the impact of stockouts on treat-
ods. The models were scored with validation data from January-March ment interruptions. A mixed-effects logistic regression model exam-
2021 according to mean average error, root mean squared error, and ined associations between price, procurement efficiency (as median
lag-1 autocorrelation. The models with the top scores were chosen to price ratio), site and drug availability.
forecast the mean and 95% prediction interval for Apr-Dec 2021. Dif- Results: Stockouts for many cytotoxic and supportive care medicines
ferences between the observed and mean predicted (O-P) numbers of were observed across sites, with highest mean unavailability in Kenya
patients were calculated. (49%), Rwanda (39%), and Tanzania (32%). Medicines vulnerable to
Results: Linear trend models performed the best for all sites except for stockouts across ≥ 4 sites included: methotrexate, bleomycin, etopo-
oncology patients at UTC where a moving average model that excluded side, ifosfamide, oral morphine, and allopurinol. Although our analysis
the period of COVID-19 scored the best. The O-P differences for revealed a statistically significant difference in MPR across sites (range
new oncology patients was 0(O-P;45-45) for BTC, +21(129-107) for 0.399-1.28, p<0.01), average MPR at each site was below the inter-
MTC, and +14(186-172) for UTC. The difference for new hematology nationally accepted threshold for efficient procurement (MPR < 1.5).
patients was +2(45-43) for BTC, +47(159-112) for MTC, -12(607-619) No significant association between MPR and unavailability was found.
for UTC. The total forecast error for all sites was +71(1171-1100), or Impacts of stockouts on treatment were noted across the majority of
6.1% of the total patients. All observed counts fell within the 95% pre- sites, with greatest potential for treatment interruptions in Hodgkin
diction intervals except new hematology patients at MTC which fell lymphoma, retinoblastoma and acute lymphoblastic leukemia.
outside the upper limit. Conclusions: Our findings provide detailed evidence of drug cost and
Conclusions: Forecasting models from operations data can pre- availability in East Africa with implications for CCM access in the
dict the total newly diagnosed patients at PHO treatment cen- region. These data can inform support national and regional policy-
ters within an error of 6.1% of the observed total number of making to optimize cancer drug availability and affordability as part of
patients. efforts to improve childhood cancer outcomes in the region.
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ABSTRACTS S111 of S635

FREE PAPER SESSION (FPS) myocardial infarction (HR 2.4 [95% CI 1.2-4.8]) and chronic kidney dis-
ease (3.6 [1.6-8.0]) while those with diabetes were also at increased risk
FPS 18: SURVIVORSHIP - CARDIAC HEALTH AND for future cardiomyopathy (3.8 [1.4-10.5]) or stroke (3.4 [1.3-8.9]).
NEUROCOGNITION 01-10-2022 8:00 AM - 9:30 AM Conclusions: Nearly one-third of young adult survivors of childhood
cancer have prediabetes which increases risk for future cardiovascu-
O225 / #331 PREDIABETES, PROGRESSION TO DIABETES, lar events and renal complications independent of cancer treatment.
AND RISK FOR LATE CARDIOVASCULAR EVENTS AND RENAL Future interventions should target prediabetes as a modifiable risk
COMPLICATIONS AMONG ADULT SURVIVORS OF CHILDHOOD factor to prevent progression to diabetes and subsequent morbidity.
CANCER IN THE ST. JUDE LIFETIME COHORT

Stephanie Dixon1 , Fang Wang2 , Lu Lu2 , Carmen Wilson2 , John O226 / #406 INFORMATICS TOOLS TO IMPLEMENT LATE
Jefferies3 , Daniel Green1 , Thomas Merchant4 , Rebecca Howell5 , CARDIOVASCULAR RISK PREDICTION MODELING FOR
Deokumar Srivastava6 , Angela Delaney Freedman7 , Leslie Robison2 , POPULATION HEALTH MANAGEMENT OF HIGH-RISK
Kirsten Ness2 , Melissa Hudson1 , Wassim Chemaitilly8 , Gregory CHILDHOOD CANCER SURVIVORS
Armstrong2
1 St. Jude Children’s Research Hospital, Oncology, Memphis, United States David Noyd1 , Amanda Janitz2 , Ashley Baker1 , William Beasley1 ,
of America; 2 St. Jude Children’s Research Hospital, Epidemiology And Can- Nancy Etzold3 , David Kendrick4 , Kevin Oeffinger5
cer Control, Memphis, United States of America; 3 University of Tennessee 1 The University of Oklahoma Health Sciences Center, Pediatrics-section Of
Health Science Center, The Cardiac Institute, Memphis, United States of Pediatric Hematology-oncology, Oklahoma City, United States of America;
America; 4 St. Jude Children’s Research Hospital, Radiation Oncology, Mem- 2 The University of Oklahoma Health Sciences Center, Biostatistics And Epi-

phis, United States of America; 5 The University of Texas at MD Anderson demiology, Oklahoma City, United States of America; 3 The University of
Cancer Center, Radiation Physics, Houston, United States of America; 6 St. Oklahoma Health Sciences Center, Stephenson Cancer Center, Oklahoma
Jude Children’s Research Hospital, Biostatistics, Memphis, United States City, United States of America; 4 The University of Oklahoma Health Sci-
of America; 7 St. Jude Children’s Research Hospital, Endocrinology, Mem- ences Center, Medical Informatics, Oklahoma City, United States of America;
phis, United States of America; 8 University of Pittsburgh Medical Center 5 Duke University Medical Center, Medicine, Durham, United States of
Children’s Hospital of Pittsburgh, Pediatrics, Pittsburgh, United States of America
America
Background and Aims: Marked improvements in outcomes for chil-
Background and Aims: Prediabetes is a potentially reversible pre- dren with cancer and robust cohort studies with longitudinal follow-up
cursor to diabetes and subsequent increased risk for cardiovascular inform evidence-based guidelines for survivors at risk for late car-
events; however, little is known about prediabetes in childhood cancer diomyopathy. Clinical informatics tools to integrate data from multiple
survivors. sources have the potential to catalyze population health management.
Methods: Prevalence of prediabetes (fasting glucose 100-125 mg/dL Methods: Two institutional cohorts used distinct approaches to imple-
or HbA1c 5.7-6.4%) and diabetes were assessed in 3529 5+ year sur- ment previously validated late cardiovascular risk prediction modeling
vivors, ≥18 years old (median 30, range 18-65 years) and compared to from the Childhood Cancer Survivor Study (CCSS) for cardiomyopa-
450 community controls stratified by age (10 year groups) and adjusted thy. The Oklahoma Childhood Cancer Survivor cohort was constructed
for race, sex, and BMI. Among survivors with available follow-up, Cox from an institutional cancer registry of survivors diagnosed between
proportional hazards regression estimated risk for progression from 2005 and 2014 (n=382). Data elements (cumulative anthracycline,
prediabetes to diabetes and risk of future cardiovascular events and cumulative chest-directed radiotherapy, alkylator, and platinum expo-
renal complications reported as hazard ratio (HR) with 95% confidence sures) were extracted from Passport for Care (PFC). The Duke Child-
intervals (CI). hood Cancer Survivor Cohort integrated cancer registry and electronic
Results: The prevalence of prediabetes overall was 29.2% (95% CI health record data, using standard query language, to automatically
27.7-30.7) and of diabetes 6.5% (5.7-7.3). In each age strata, survivors extract chemotherapy exposures. Risk groups were compared to the
had significantly higher prevalence of prediabetes and diabetes than Children’s Oncology Group (COG) Long-Term Follow-up Guidelines.
controls (p<0.001; e.g, among those aged 30-39 35.0% of survivors vs. Results: In Oklahoma, of 481 survivors documented in PFC, 29%,
14.3% of controls had prediabetes). Among 695 survivors with predi- 56%, and 15% were classified as low, moderate, high risk for car-
abetes, 68 (10%; median follow-up 5.1 years) progressed to diabetes. diomyopathy, respectively, based on the CCSS late cardiomyopathy
After adjustment for age, sex, race and body composition, risk of pro- risk calculator. At Duke (n=737), 64%, 31%, and 6% were classified as
gression to diabetes was increased by treatment including pancreatic low, moderate, and high risk, respectively. Concordance was modest
radiation ≥10 Gy (HR 2.7 [95% CI 1.1-6.8]) or total body irradiation for high (Kappa = 0.42 and 0.53 for the Oklahoma and Duke cohorts,
(4.4 [1.5-13.1]). Compared to survivors with normal glucose control, respectively) and moderate risk groups (Kappa = 0.46 and 0.46) and
independent of demographic characteristics and relevant treatment good for the low risk group (Kappa = 0.77 and 0.70) compared to COG
exposures, those with prediabetes were at increased risk of future risk groups.
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S112 of S635 ABSTRACTS

Conclusions: Clinical informatics tools represent a feasible approach tality [any one MRF (RR=1.8, 95% confidence interval [CI]: 1.1-3.0);
to leverage discrete data elements regarding key treatment expo- any two (RR=3.8, 95%CI:2.3-6.4); any three (RR=4.2, 95%CI:2.3-
sures from PFC to successfully implement previously validated late 7.6); and ≥ four (RR=3.8, 95%CI:1.8-8.2)], health-related mortality
cardiovascular risk prediction models on a population health level. [any one (RR=2.3, 95%CI: 1.2-4.3); any two (RR=4.9, 95%CI:2.6-9.3);
Alternative strategies to automatically extract data elements from the any three (RR=6.3, 95%CI:3.2-12.7); ≥ four (RR=3.7, 95%CI:1.4-9.5)],
EHR in the Duke cohort support interoperability to catalyze broader and subsequent neoplasm-related mortality, a subtype of health-
impact. related mortality [any one (RR=4.3, 95%CI:1.4-12.9); any two (RR=8.2,
95%CI:2.7-25.0); any three (RR=11.1, 95%CI:3.3-37.3); and ≥ four
(RR=7.0, 95%CI:1.6-29.5)]. Among individual risk factors, only hyper-
O227 / #862 MODIFIABLE RISK FACTORS (MRFS) AND LATE tension (RR=1.8, 95%CI:1.3-2.7, p=0.001) and healthy lifestyle index
MORTALITY IN THE ST. JUDE LIFETIME COHORT STUDY of 0 vs. 4 (RR=3.6, 95%CI:1.0-12.4, p=0.043) were associated with an
(SJLIFE) increased risk of post-baseline all-cause mortality.
Conclusions: MRFs and unhealthy lifestyle are associated with
Matthew Ehrhardt1 , Qi Liu2 , Stephanie Dixon1 , Eric Caron3 , Debbie increased risk of all-cause and health-related mortality in adult sur-
Redd3 , Kyla Shelton3 , Nickhill Bhakta4 , Daniel Mulrooney1 , Tara vivors of childhood cancer.
Brinkman5 , I-Chan Huang3 , Wassim Chemaitilly6 , Angela Delaney
Freedman7 , Alia Zaidi3 , Gregory Armstrong3 , Deokumar Srivastava8 ,
Kirsten Ness3 , Leslie Robison3 , Yutaka Yasui3 , Melissa Hudson1 O228 / #1435 PREVALENCE AND RISK FACTORS OF
1 St. Jude Children’s Research Hospital, Oncology, Memphis, United States of UNHEALTHY LIFESTYLE BEHAVIORS AMONG SURVIVORS OF
America; 2 University of Alberta, Public Health Sciences, Edmonton, Canada; CHILDHOOD CANCER IN THE NETHERLANDS
3 St. Jude Children’s Research Hospital, Epidemiology And Cancer Con-
trol, Memphis, United States of America; 4 St Jude Children’s Research Eline Bouwman1 , Adriaan Penson1 , Maud De Valk2 , Selina Van Den
Hospital, Oncology, Department Of Global Pediatric Medicine, Depart- Oever2 , Helena Van Der Pal2,3 , Eline Van Dulmen-Den Broeder4 ,
ment Of Epidemiology And Cancer Control, Memphis, United States of Nicole Blijlevens1 , Dorine Bresters2 , E (Lieke) Feijen2 , Marry Van Den
America; 5 St. Jude Children’s Research Hospital, Epidemiology And Can- Heuvel-Eibrink2 , Margriet Van Der Heiden-Van Der Loo5 , Cecile
cer Control, Psychology, Memphis, United States of America; 6 University Ronckers6 , Jop Teepen2 , Wim Tissing2,7 , Birgitta Versluys2 , Leontien
of Pittsburgh Medical Center Children’s Hospital of Pittsburgh, Pediatrics, Kremer2,8,9 , Saskia Pluijm2 , Jacqueline Loonen10
Pittsburgh, United States of America; 7 St. Jude Children’s Research Hospi- 1 Radboud University Medical Center, Hematology, Nijmegen, Nether-
tal, Endocrinology, Memphis, United States of America; 8 St. Jude Children’s lands; 2 Princess Máxima Center for Pediatric Oncology, Pediatric Oncol-
Research Hospital, Biostatistics, Memphis, United States of America ogy, Utrecht, Netherlands; 3 PanCare, Pancare, Bussum, Netherlands;
4 Amsterdam University Medical Centers, Department Of Pediatric Oncol-
Background and Aims: The contribution of MRFs toward late mortal- ogy/hematology, Amsterdam, Netherlands; 5 Dutch Childhood Oncology
ity among childhood cancer survivors is unknown. Group, Childhood Oncology, Utrecht, Netherlands; 6 Carl v Ossietzky Uni-
Methods: We characterized mortality among 9,602 ≥5-year survivors versity of Oldenburg, Department Of Health Services Research, Oldenburg,
of childhood cancer diagnosed (1962-2012) and treated at St. Jude Germany; 7 University Medical Center Groningen, Department Of Pediatric
Children’s Research Hospital. Treatments were abstracted from med- Oncology/hematology, Groningen, Netherlands; 8 Emma Children’s Hospital,
ical records and cause of death obtained from the National Death Amsterdam UMC, University of Amsterdam, Pediatrics, Amsterdam, Nether-
Index, supplemented by record review. For a subgroup who partici- lands; 9 Utrecht University and Utrecht Medical Center, Faculty Of Medicine,
pated SJLIFE (n=4,321), baseline clinical study assessments identified Utrecht, Netherlands; 10 Radboud University Medical Center, Department
traditional (hypertension, diabetes, underweight/obesity) and poten- Of Hematology, Nijmegen, Netherlands
tially MRFs (bone mineral deficiency, hypogonadism, hypothyroidism,
adrenal insufficiency) and a healthy lifestyle index [composite score, 0 Background and Aims: Childhood cancer survivors (CCSs) are at an
(unhealthy) to 4 (healthy) of smoking, alcohol intake, body mass index, increased risk for the development of chronic health conditions. These
physical activity, available for 3,694 adult survivors only]. Standardized late effects can be exacerbated by unhealthy lifestyle behaviors. This
mortality ratios (SMRs) were estimated for 5-year survivors using age- study aims to investigate the prevalence of unhealthy lifestyle behav-
sex-race/ethnicity-specific US-population mortality rates. Associations iors in Dutch CCSs compared to their siblings. In addition, we aimed to
between MRFs/lifestyle and post-baseline mortality were assessed assess the risk factors for unhealthy lifestyle behaviors in Dutch CCSs.
using piecewise exponential models, adjusting for attained age, sex, Methods: We included 2677 CCSs (i.e. 5-year survivors of all child-
race, age at diagnosis, income, employment, and insurance status. hood malignancies diagnosed between 1963 and 2001; current age
Results: SMRs were increased for all-cause (7.4, 95%CI:7.0-7.8), ≥18 years) and 992 sibling controls of the Dutch Childhood Cancer
health-related (7.2, 95%CI:6.7-7.8), and external (1.7, 95%CI:1.4-1.9) Survivor Study LATER cohort part 1. Questionnaire data was collected
causes of death. Among adult SJLIFE participants, MRFs (vs. none) on alcohol consumption, drug use, smoking, BMI, and physical activity
were associated with increased risk of post-baseline all-cause mor- levels. Multivariable logistic regression analyses were done to compare
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ABSTRACTS S113 of S635

the prevalence of each unhealthy behavior between CCSs and their Methods: 505 adult survivors of medulloblastoma (58% male, median
siblings, adjusted for confounding (sociodemographic factors, comor- [min-max] 7 [0-20] years at diagnosis, 29 [18-46] years at evalua-
bidities, limb amputation), and to examine risk factors for unhealthy tion) completed the CCSS Neurocognitive Questionnaire (impairment:
lifestyle behaviors within CCSs. scores> 90th %ile of siblings). Treatment exposures were categorized
Results: Prevalence rates of daily alcohol consumption (CCSs: 28.8%, as surgery + craniospinal irradiation (CSI)<30Gy (± chemotherapy);
siblings: 30.6%), daily smoking (CCSs: 14.9%; siblings: 15.0%), any drug surgery + CSI≥30Gy (no chemotherapy); or surgery + CSI≥30Gy +
use in the past year (CCSs: 13.3%; siblings: 12.9%), and BMI ≥25 (CCSs: chemotherapy. Self-reported CHCs were graded using NCI’s CTCAE
36.2%; siblings: 36.1%) did not statistically differ between CCSs and v4.3. Latent class analysis utilized five indicators (employment, mar-
siblings. However, CCSs more often failed to meet the national guide- ital status, independent living, driver’s license, assistance with rou-
lines for physical activity than their siblings (CCSs: 27.0%; siblings: tine/personal care needs) to identify mutually exclusive groups of
18.5%; adjusted OR 1.45, 95% CI = 1.19-1.76). Within CCSs, male sex, functional independence. Multivariable modified Poisson regressions
age ≥45 years, a low and middle educational level, marital status, pres- examined relative risk (RR) of neurocognitive impairment between the
ence of ≥1 comorbidities, and limb amputation were all associated with groups, adjusting for sex, race, age at diagnosis, and age at assess-
an unhealthy lifestyle. ment. Path analysis examined the impact of treatment on functional
Conclusions: This study shows high prevalence rates of overweight and independence, mediated by Grade 2-4 CHCs and/or neurocognitive
physical inactivity in CCSs. This illustrates the need for dietary and impairment.
physical activity interventions personalized to CCSs. The findings will Results: Three latent groups of survivors varying in functional indepen-
also help healthcare professionals to identify CCSs at risk for unhealthy dence were identified: independent (37%), moderately independent
lifestyle behaviors, and thus those who will benefit the most from (non-independent living and unmarried; 21%), and non-independent
lifestyle interventions. (42%). Survivors with impaired task efficiency (RR=1.83, 95% CI,
1.37-2.45), organization (RR=1.33, 95% CI, 1.09-1.61), and emo-
tional regulation (RR=1.26, 95% CI, 1.03-1.55) were at elevated risk
O229 / #211 NEUROCOGNITIVE IMPAIRMENT AND for being non-independent compared to independent. Path analysis
FUNCTIONAL INDEPENDENCE IN ADULT SURVIVORS OF revealed no direct or indirect paths from treatment exposures to
CHILDHOOD MEDULLOBLASTOMA: A REPORT FROM THE non-independence through neurocognitive impairment and/or CHCs.
CHILDHOOD CANCER SURVIVOR STUDY (CCSS) There were, however, significant direct paths from impaired organiza-
tion (β=0.23, p=0.013) and stroke/seizure (β=0.47, p<0.001) to non-
Chiara Papini1 , Mengqi Xing2 , Sedigheh Mirzaei Salehabadi2 , Ingrid independence.
Tonning Olsson3,4 , Katharine Lange5 , Peter De Blank6 , Ralph Conclusions: Neurocognitive impairment and neurologic sequelae
Salloum7 , Deokumar Srivastava2 , Wendy Leisenring8 , Rebecca in medulloblastoma survivors contribute to reduced indepen-
Howell9 , Kevin Oeffinger10 , Leslie Robison1 , Gregory Armstrong1 , dence in adulthood, irrespective of past treatment exposures.
Kevin Krull1,11 , Tara Brinkman1,11 Functional rehabilitation efforts following seizures/strokes
1 St. Jude Children’s Research Hospital, Epidemiology And Cancer Control, and interventions for neurocognitive deficits may pro-
Memphis, United States of America; 2 St. Jude Children’s Research Hospital, mote attainment of independence in long-term survivors of
Biostatistics, Memphis, United States of America; 3 Skåne University Hospi- medulloblastoma.
tal, Paediatric Psychology, Lund, Sweden; 4 Lund University, Clinical Sciences
Lund, Paediatrics, Lund, Sweden; 5 Children’s Minnesota, Pediatric Oncol-
ogy, Minneapolis, United States of America; 6 Cincinnati Children’s Hospital O230 / #837 NEUROCOGNITIVE FUNCTION AND
Medical Center, Brain Tumor Center, Cincinnati, United States of America; HEALTH-RELATED QUALITY OF LIFE IN A NATIONWIDE
7 Nationwide Children’s Hospital, Hematology, Oncology & Bone Marrow COHORT OF LONG-TERM SURVIVORS OF CHILDHOOD BRAIN
Transplant, Columbus, United States of America; 8 Fred Hutchinson Cancer TUMOR
Research Center, Clinical Research Division, Seattle, United States of Amer-
ica; 9 The University of Texas at MD Anderson Cancer Center, Radiation Anne Sophie Lind Helligsoe1,2 , Louise Tram Henriksen1,2 , Line
Physics, Houston, United States of America; 10 Duke University, Medicine, Kenborg3 , Yasmin Lassen-Ramshad4 , Lisa M. Wu5,6 , Jeanette
Durham, United States of America; 11 St. Jude Children’s Research Hospital, Winther2,3,7 , Henrik Hasle1 , Ali Amidi6
Psychology, Memphis, United States of America 1 Aarhus University Hospital, Department Of Pediatrics And Adolescent
Medicine, Aarhus, Denmark; 2 Aarhus University, Department Of Clinical
Background and Aims: Survivors of childhood medulloblastoma are at Medicine, Faculty Of Health, Aarhus, Denmark; 3 Danish Cancer Society
risk of developing chronic health conditions (CHCs) and neurocognitive Research Center, Childhood Cancer Research Group„ Copenhagen, Den-
late effects secondary to their tumor and intensive multimodal thera- mark; 4 Aarhus University Hospital, Danish Centre For Particle Therapy,
pies. The contribution of these morbidities to attainment of functional Aarhus, Denmark; 5 Aarhus University, Aarhus Institute Of Advanced Stud-
independence in adulthood has not previously been examined. ies, Aarhus, Denmark; 6 Aarhus University, Unit For Psychooncology &
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S114 of S635 ABSTRACTS

Health Psychology, Department Of Psychology And Behavioural Sciences, Background and Aims: According to Lancet Oncology Commission
Aarhus, Denmark; 7 Aarhus University, Hospital, Aarhus, Denmark (2020) stark inequalities in access to cancer prevention, diagnostic,
treatment and care for children between Low and Middle-Income
Background and Aims: Survivors of childhood brain tumor are at high Countries (LMIC) and High-Income countries, result in large global
risk of late effects, but limited data exists regarding their neurocogni- variations in survival: estimates of 5-year net childhood cancer survival
tive function and associations with quality of life and symptom burden. show a huge gap between Eastern Africa (8.1%) and North America
Our aim was to examine neurocognitive function in childhood brain (83%). At current levels of access and referral, it’s estimated that 44.9%
tumor survivors and associations with quality of life and symptom of 13.7 million new cases of childhood cancer foreseen for 2020-2050
burden. will go undetected and 11.1 million children will die of cancer. Among
Methods: Five-year survivors of brain tumor over the age of 15 were them 9.3 million (84.1%) will be in LMIC because of current demo-
identified in the Danish Childhood Cancer Registry (n = 423). Eligible graphic trends and differences in survival rates. Since 2018 AFRON
and consenting participants completed neuropsychological tests and and Soleterre implement ABLE+ programme (Awareness for Burkitt
questionnaires assessing quality of life, insomnia, fatigue, anxiety and Lymphoma Eradication) in Northern Uganda in collaboration with St.
depression. Survivors treated with radiation (n = 59) were statistically Mary’s Lacor Hospital, to increase the survival of children with Burkitt
compared with survivors not treated with radiation (n = 102). Cor- Lymphoma (BL), in line with WHO initiative.
relations of neurocognitive function with quality of life and symptom Methods: Developed through multi-stakeholders participatory
burden were performed. methodologies, ABLE+ pursues a holistic and integrated approach to
Results: In total, 170 survivors participated (40.2% participation rate). fill gaps in the different phases of care, focusing on children and their
Sixty-six percent of the survivors who completed neuropsychological caregivers. ABLE+ reinforces hospitals’ and health centers’ doctors’
tests (n = 161) exhibited overall neurocognitive impairment. Neu- and health workers’ capacities; supports them through supervision
rocognitive outcomes for survivors treated with surgery were below with mobile technologies to improve early detection and diagnosis;
normative expectations. Survivors treated with radiation, especially strengthens the referral system to facilitate access to care; provides
whole-brain irradiation, exhibited poorer neurocognitive outcomes psychosocial and educational support to children, increases caregivers’
than survivors not treated with radiation. Furthermore, a number of awareness and empowerment, also through income generating activ-
survivors experienced significant fatigue (40%), anxiety (23%), insom- ities; enhances the sustainability of care through follow-up and home
nia (13%), and/or depression (6%). Survivors treated with radiation visits.
reported lower quality of life and higher symptom burden scores Results: In spite of COVID-19 pandemic disruptions, 143 new BL cases
than survivors not treated with radiation; particularly in physical func- were referred in 4 years (36 cases/year against 17 in 2016), with 0%
tioning, social functioning with symptoms of fatigue. Neurocognitive treatment abandonment in the last 2 years and the mortality rate
impairment was not associated with quality of life or symptom burden. of children treated for BL decreased from 47.05% (2016) to 28.95%
Conclusions: In this study, a majority of the childhood brain tumor sur- (2021).
vivors experienced neurocognitive impairment, reduced quality of life, Conclusions: An innovative, integrated, holistic and cost-effective
and high symptom burden. Although not associated with each other, intervention with high potential social impact should be considered for
it is apparent that childhood brain tumor survivors experience not scaling-up and/or replication in other LMIC.
only neurocognitive dysfunction, but may also experience quality of life
impairments and significant symptom burden.
O232 / #1855 EARLY DIAGNOSIS OF CHILDHOOD CANCER:
ATTITUDES AND KNOWLEDGE OF HEALTH AGENTS IN
CCI SIKASSO HEALTH CENTERS

CCI: IMPROVING EARLY DETECTION AND ACCESS TO CARE Hachimi Amadou Poma, Assetou Cissouma, Mamadou Yalcouyé
01-10-2022 8:15 AM - 9:30 AM hospital of sikasso, Health, sikasso, Mali

O231 / #514 IMPROVING ACCESS TO CHILDHOOD CANCER Background and Aims: BACKGROUND The situation of childhood
CARE IN NORTH UGANDA THROUGH EARLY DETECTION, cancer is alarmingly in sub-Saharan countries where the cure rate does
CAREGIVERS EMPOWERMENT AND CASE FOLLOW-UP not exceed 15 to 20 %. This is due to insufficient means of diagno-
sis, to the delay of treatment and especially to the lack of adapted
Maria Tiziana Andriani1 , Alessandra Radaelli2 , Adrian Ssali3 , Alessio chemotherapy protocols. The delay in diagnosis is based on a complex
Di Carlo4 chain of factors and events complicated by the difficulties. Early diag-
1 AFRON Oncology for Africa, Board, Rome, Italy; 2 Soleterre Founda- nosis is a fundamental objective in oncology. AIM The main aim is to
tion, Programmes, Milan, Italy; 3 Soleterre Foundation, Uganda, Kampala, evaluate the knowledge and attitudes of medical and paramedical per-
Uganda; 4 AFRON Oncology for Africa, Programmes, Rome, Italy sonnel in the early diagnosis of childhood cancer in the health district of
Sikasso.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S115 of S635

Methods: This is a prospective, descriptive, cross-sectional study con- Results: Surveys of EVAT implementation leaders from 38 centers
ducted over 6 months in 2021 on a sample of 120 community health found that most centers teach EVAT to families with the material pro-
workers. Data collection was carried out using a previously tested vided through Proyecto EVAT for teaching hospital staff, including the
structured questionnaire. PEWS scale, algorithm, and a blackboard that shows the traffic light
Results: We interviewed 120 health workers and noted that 36.67% (color status of a patient); only one center used specialized mate-
were general practitioners, 29.17% were nurses, 5.83% were pedia- rial developed specifically for families to carry out this training. To
tricians, 10.8% were midwives, and 17.5% were from other special- standardize those who do not use specific materials, a triptych, an info-
ties. According to them, the first signs of cancer were adenopathy graphic and a video are developing to help centers to communicate
(90.8%), abdominal mass (89.2%), hepatosplenomegaly (75.8%) and EVAT to families.
75.8% stated that there was a predisposition to cancer in children. In Conclusions: While teaching EVAT to families is a universal need, few
front of an abdominal mass 51.6% requested an abdominal ultrasound. standardized resources for this exist. Creating of EVAT educational
92.5% of the agents thought that management depended on early best practices aimed specifically at families will improve commu-
diagnosis. nication between hospital staff and families and improve hospital
Conclusions: The lack of knowledge of the early signs of cancer leads care.
to delayed diagnosis.

FREE PAPER SESSION (FPS)


O233 / #1751 TALKING WITH FAMILIES ABOUT EVAT: A
SPANISH-LANGUAGE PEDIATRIC EARLY WARNING SYSTEM FPS 19: ALL OUTCOMES AND PROGNOSTIC FACTORS
01-10-2022 10:40 AM - 12:10 PM
Tania Conde1 , Dora Soberanis2 , Janeth Quelal3 , Karla Aguilar4 , Camila
Barra5 , Paola Sierra6 , Ma Matilde Nuñez Martinez7 , Jocelyn Rivera8 , O234 / #895 OUTCOMES IN CHILDREN, ADOLESCENTS,
Asya Agulnik9 AND YOUNG ADULTS WITH DOWN SYNDROME AND ACUTE
1 Casa de la Amistad, Casa De La Amistad Para Niños Con Cáncer, I.a.p, LYMPHOBLASTIC LEUKEMIA: A REPORT FROM THE
Cuidad de México, Mexico; 2 St. Jude Children’s Research Hospital, Crital CHILDREN’S ONCOLOGY GROUP
Care, Memphis, United States of America; 3 SOLCA QUITO, Oncology, Quito,
Ecuador; 4 Unidad Nacional de Oncología Pediátrica, Oncology, Guatemala, Karen Rabin1 , Meenakshi Devidas2 , Zhiguo Chen3 , Yunfeng Dai4 ,
Guatemala; 5 Hospital Dr Luis Calvo Mackenna, Department Of Pedi- Johann Hizler5 , Jun Yang6 , Andrew Carroll7 , Nyla Heerema8 , Michael
atric Oncology, Santiago, Chile; 6 Hospital Escuela Universitario, Oncology, Borowitz9 , Brent Wood10 , Kathryn Roberts11 , Charles Mullighan11 ,
Tegucigalpa, Honduras; 7 HOSPITAL INFANTIL TELETÓN DE ONCOLOGÍA, Richard Harvey12 , I-Ming Chen12 , Cheryl Willman12 , Shalini Reshmi13 ,
EnseÑanza De EnfermerÍa, QUERETARO QUERETARO, Mexico; 8 Hospital Julie Gastier-Foster14 , Kelly Maloney15 , Eric Larsen16 , Reuven
Infantil Teleton de oncologia, Emergency Medicine, Queretaro, Mexico; 9 St. Schore17 , Michael Burke18 , Wanda Salzer19 , Naomi Winick20 , William
Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis, Carroll21 , Elizabeth Raetz21 , Mignon Loh22 , Stephen Hunger23 , Anne
United States of America Angiolillo17
1 Baylor College of Medicine/Texas Children’s Cancer Center, Pediatric
Background and Aims: Proyecto EVAT (Escala de Valoración de Alerta Hematology-oncology, Houston, United States of America; 2 St Jude Chil-
Temprana) is a quality improvement collaborative with 73 pediatric dren’s Research Hospital, Global Pediatric Medicine, Memphis, United
oncology centers in 19 countries in Latin American and Europe to States of America; 3 Children’s Oncology Group, Biostatistics, Gainesville,
reduce clinical deterioration in children with cancer through imple- United States of America; 4 University of Florida, Biostatistics, Gainesville,
mentation of a Spanish-language Pediatric Early Warning System United States of America; 5 Hospital for Sick Children, University Of
(PEWS). Recently, how to explain EVAT to families is a common ques- Toronto, Toronto, Canada; 6 St Jude Children’s Research Institute, Depart-
tion among new centers. Therefore, we describe the development of a ment Of Pharmacy And Pharmaceutical Sciences, Memphis, United States
formal processes to guide hospital staff in how to communicate to the of America; 7 University of Alabama at Birmingham, Genetics, Birming-
parent about EVAT. ham, United States of America; 8 Nationwide Children’s Hospital, Pathol-
Methods: This project included three phases. First, EVAT implementa- ogy, Columbus, United States of America; 9 The Johns Hopkins Hospital,
tion leaders were surveyed on strategies of how they teach EVAT to Pathology, Baltimore, United States of America; 10 Children’s Hospital Los
parents. Subsequently, this exploration was discussed with the EVAT Angeles, Pathology And Laboratory Medicine, Los Angeles, United States
steering committee – wich is composed of 27 member leadership of of America; 11 St Jude Children’s Research Institute, Pathology, Mem-
Proyecto EVAT, made of a multidisciplinary team of experts from Latin phis, United States of America; 12 University of New Mexico, Pathology,
America-, to create a manual to describe best practices for how to Albuquerque, United States of America; 13 Nationwide Children’s Hospi-
teach families on EVAT. This was supplemented with visual material to tal, Laboratory Medicine/anatomic Pathology, columbus, United States of
reinforce major topics during training. Finally, the impact of this new America; 14 Texas Children’s Hospital, Global Hematology/oncology Pedi-
training will be measured through satisfaction surveys of hospital staff. atric Excellence, Houston, United States of America; 15 Children’s Hospital
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S116 of S635 ABSTRACTS

Colorado, Hematology/oncology - Pediatric, Aurora, United States of Amer- Nyla Heerema15 , Shalini Reshmi16 , Julie Gastier-Foster17 , Richard
ica; 16 Maine Children’s Cancer Program, Pediatric Oncology, Scarborough, Harvey18 , I-Ming Chen18 , Kathryn Roberts19 , Charles Mullighan19 ,
United States of America; 17 Children’s National Medical Center, Center For Cheryl Willman20 , Naomi Winick21 , William Carroll22 , Rachel Rau23 ,
Cancer And Blood Disorders, Washington, DC, United States of America; David Teachey24 , Stephen Hunger24 , Elizabeth Raetz22 , Meenakshi
18 Children’s Wisconsin, Pediatrics, Milwaukee, United States of America; Devidas25 , John Kairalla1
19 U.S. Army Medical Research and Materiel Command, Pediatrics, Fort 1 University of Florida, Biostatistics, Gainesville, United States of Amer-
Detrick, United States of America; 20 UT Southwestern/Simmons Cancer ica; 2 Seattle Children’s Hospital, University of Washington, Department
Center, Pediatric Hematology/oncology, Dallas, United States of America; Of Pediatrics, Seattle, United States of America; 3 The Johns Hopkins
21 NYU Langone Health, Pediatrics, New York, United States of America; Hospital, Pathology, Baltimore, United States of America; 4 Hospital for
22 Seattle Children’s Hospital, Pediatrics, Seattle, United States of America; Sick Children, Haematology/oncology, Toronto, Canada; 5 Baylor College of
23 Children’s Hospital of Philadelphia, Pediatrics, Philadelphia, United States Medicine/Texas Children’s Cancer Center, Pediatric Hematology-oncology,
of America Houston, United States of America; 6 ImmunoGen, Inc., Managing Direc-
tor, Waltham, United States of America; 7 Children’s Hospital Colorado,
Background and Aims: Patients with Down syndrome (DS) and B- Hematology/oncology - Pediatric, Aurora, United States of America; 8 HARP
acute lymphoblastic leukemia (B-ALL) experience increased rates of Pharma Consulting, LLC, Drug Development, Mystic, United States of Amer-
relapse, toxicity, and death. We report results for DS B-ALL patients ica; 9 Maine Children’s Cancer Program, Pediatric Oncology, Scarborough,
enrolled on Children’s Oncology Group trials conducted from 2003- United States of America; 10 Children’s National Medical Center, Center For
2018. Cancer And Blood Disorders, Washington, DC, United States of America;
Methods: We analyzed data for patients with DS (n = 743) and without 11 Children’s Wisconsin, Pediatrics, Milwaukee, United States of America;
DS (n = 20,067), age 1-30 years, enrolled on four B-ALL standard-risk 12 Uniformed Services University, F. Edward Hebert School Of Medicine,
(SR) and high-risk (HR) trials. Bethesda, United States of America; 13 Children’s Hospital Los Angeles,
Results: Patients with DS exhibited more frequent minimal residual Pathology And Laboratory Medicine, Los Angeles, United States of Amer-
disease (MRD) >0.01% at end-induction (30.8% vs 21.5%, p <0.001) ica; 14 University of Alabama at Birmingham, Genetics, Birmingham, United
compared to non-DS B-ALL. Within patients with NCI HR B-ALL, States of America; 15 Nationwide Children’s Hospital, Pathology, Columbus,
this difference persisted at end-consolidation (34.0% vs 11.7%, p United States of America; 16 Nationwide Children’s Hospital, Laboratory
<0.0001). Five-year event-free survival (EFS) and overall survival Medicine/anatomic Pathology, Columbus, United States of America; 17 Texas
(OS) were significantly poorer for patients with vs without DS over- Children’s Cancer Center, Baylor College of Medicine, Department Of Pedi-
all (EFS 79.0+2.0% vs 87.3+0.3%, p <0.0001; OS 86.8+1.7% vs atrics, Houston, United States of America; 18 University of New Mexico,
93.5+0.2%, p <0.0001), and within NCI SR and HR subgroups. Mul- Pathology, Albuquerque, United States of America; 19 St Jude Children’s
tivariable Cox regression analysis for risk factors associated with Research Institute, Pathology, Memphis, United States of America; 20 Mayo
inferior EFS in DS B-ALL identified age >10 years, initial white Clinic, Cancer Center/laboratory Medicine And Pathology, Rochester, United
blood count ≥50,000/microliter, and end-induction MRD positivity, but States of America; 21 UT Southwestern/Simmons Cancer Center, Pediatric
not cytogenetics or flow cytometric CRLF2 overexpression. Patients Hematology/oncology, Dallas, United States of America; 22 NYU Langone
with DS demonstrated higher 5-year cumulative incidence of relapse Health, Pediatrics, New York, United States of America; 23 Baylor Col-
(11.7+1.4% vs 9.3+0.2%, p = 0.0198), induction death (3.4% vs 0.8%, p lege of Medicine, Children’s Cancer And Hematology Center, Houston,
<0.0001), and death in remission (4.8+0.8% vs 1.7+0.1%, p <0.0001). United States of America; 24 Children’s Hospital of Philadelphia, Pediatrics,
Mucositis, infections, and hyperglycemia were significantly more fre- Philadelphia, United States of America; 25 St Jude Children’s Research
quent in all patients with DS, while seizures were more frequent in Hospital, Global Pediatric Medicine, Memphis, United States of America
patients with DS on HR trials (4.1% vs 1.7%, p = 0.001).
Conclusions: Patients with DS B-ALL exhibit increased rates of treat- Background and Aims: Children’s Oncology Group (COG) therapeu-
ment toxicity, death during treatment, and relapse. Novel therapeutic tic risk stratification algorithms use categorical (yes/no) variables.
strategies are needed to improve these outcomes. We investigated whether using continuous variables assigned with
different weights would improve relapse prediction.
Methods: We retrospectively classified patients (N=21,199 from COG
O235 / #1091 COMPARISON OF CURRENT AND ENHANCED trials AALL0331/0232; AALL0932/1131) using COG’s current risk
RISK STRATIFICATION OF 21,199 CHILDREN, ADOLESCENTS, stratification algorithm. We next developed and validated a multivari-
AND YOUNG ADULTS WITH ACUTE LYMPHOBLASTIC able Cox model for relapse free survival (RFS) using peripheral blood
LEUKEMIA: A CHILDREN’S ONCOLOGY GROUP REPORT minimal residual disease (MRD) at induction day 8, marrow MRD at
induction day 29, white blood cell count, nonlinear age at diagnosis,
Natalie Delrocco1 , Mignon Loh2 , Michael Borowitz3 , Sumit Gupta4 , CNS status, and favorable/unfavorable cytogenetics. We compared the
Karen Rabin5 , Patrick Zweidler-Mckay6 , Kelly Maloney7 , Leonard predictive ability of this model to machine-learning (ML) alternatives:
Mattano Jr8 , Eric Larsen9 , Anne Angiolillo10 , Reuven Schore10 , survival random forest (RF), boosted Cox, and linear support vector
Michael Burke11 , Wanda Salzer12 , Brent Wood13 , Andrew Carroll14 , machine (SVM). We identified four risk groups by selecting cutoffs of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S117 of S635

the COG Prognostic Index (PICOG ) model that maximized the discrim- patients (P = 0.360). Notably, 37 of 40 “NCI” HR ETV6-RUNX1
ination of the predictive model and compared the model-based risk patients who received low-intensity “St. Jude” LR therapy had excel-
groups to COG’s current gold-standard risk groups. lent EFS (97.3% ± 2.8%). For hyperdiploid B-ALL, EFS was worse
Results: Increased PICOG is strongly associated with increased risk of for “NCI” HR (n = 59; 87.6% ± 4.5%) than for “NCI” SR (n = 237;
relapse (testing hazard ratio = 2.55) and has good predictive discrimi- 96.4% ± 1.3%) patients (P = 0.016) but did not differ between “St.
nation (testing concordance index (c-index) 0.74). ML models explored Jude” LR (n = 220; 96.1% ± 1.4%) and “St. Jude” SR/HR (n = 76;
did not sufficiently outperform the PICOG , with best performance from 90.6% ± 3.6%) patients (P = 0.133). Although EFS was similar for
the RF model (training c-index 0.74). For those with moderate relapse “NCI” SR (n = 188; 96.0% ± 1.5%) and HR (n = 32; 96.9% ± 3.2%)
risk in current COG risk classification, the PICOG identifies subgroups hyperdiploid patients classified as “St. Jude” LR (P = 0.719), EFS was
with variable 5-year RFS. Within current standard risk average (SR- worse for “NCI” HR (n = 27; 77.4% ± 8.2%) than for “NCI” SR (n = 49;
Avg) patients, there is an ultra-low risk group (RFS = 0.96) and a group 98.0% ± 2.2%) patients among those receiving “St. Jude” SR/HR
with higher risk (0.85) than typical SR-Avg patients. Similarly, amongst intensified therapy (P = 0.004). The results were similar when only
COG NCI high risk (HR) patients, PICOG identifies 4 groups rang- hyperdiploid patients with DNA indices ≥ 1.16 were evaluated.
ing from 0.96 to 0.65, providing additional discrimination for future Conclusions: Contemporary MRD-directed therapy provides excellent
treatment stratification. outcomes except for NCI HR hyperdiploid patients with slow early
Conclusions: The PICOG can identify patients for whom therapeutic MRD response, who require new approaches. Among NCI HR patients,
intensification may not result in significantly better outcomes while 93% with ETV6-RUNX1 and 54% with hyperdiploid ALL experienced
improving the discrimination of HR patients to allow randomized excellent outcomes with a low-intensity regimen.
interventions with achievable hazard ratios.

O237 / #519 IAMP21 PREDICTS POOR OUTCOMES IN


O236 / #1601 OUTCOMES OF PATIENTS WITH ETV6-RUNX1 B-ACUTE LYMPHOBLASTIC LEUKEMIA: A REPORT FROM THE
AND HYPERDIPLOID B-ACUTE LYMPHOBLASTIC LEUKEMIA CHILDREN’S ONCOLOGY GROUP AALL1131
TREATED IN THE ST. JUDE TOTAL 15 AND 16 STUDIES
Michael Burke1 , Wanda Salzer2 , Zhiguo Chen3 , Meenakshi Devidas4 ,
Katelyn Purvis1 , Yinmei Zhou2 , Seth Karol1 , Jeffrey Rubnitz1 , Raul Lia Gore5 , Anne Angiolillo6 , Reuven Schore6 , Nyla Heerema7 , Andrew
Ribeiro1 , Shawn Lee3 , Jun Yang3 , Cheng Cheng2 , Charles Mullighan4 , Carroll8 , Joanne Hilden5 , Eric Larsen9 , Elizabeth Raetz10 , Naomi
Sima Jeha1,5 , Ching-Hon Pui1,5 , Hiroto Inaba1 Winick11 , William Carroll10 , Stephen Hunger12 , Mignon Loh13
1 St. Jude Children’s Research Hospital, Department Of Oncology, Memphis, 1 Children’s Wisconsin, Pediatrics, Milwaukee, United States of America;
United States of America; 2 St. Jude Children’s Research Hospital, Biostatis- 2 U.S. Army Medical Research and Materiel Command, Pediatrics, Fort
tics, Memphis, United States of America; 3 St Jude Children’s Research Detrick, United States of America; 3 Children’s Oncology Group, Biostatis-
Institute, Department Of Pharmacy And Pharmaceutical Sciences, Mem- tics, Gainesville, United States of America; 4 St Jude Children’s Research
phis, United States of America; 4 St Jude Children’s Research Institute, Hospital, Global Pediatric Medicine, Memphis, United States of Amer-
Pathology, Memphis, United States of America; 5 St Jude Children’s Research ica; 5 Children’s Hospital Colorado and The University of Colorado School
Hospital, Global Pediatric Medicine, Memphis, United States of America of Medicine, Pediatrics, Denver, United States of America; 6 Children’s

National Medical Center, Center For Cancer And Blood Disorders, Wash-
Background and Aims: Children with ETV6-RUNX1 and hyperdiploid ington, DC, United States of America; 7 Nationwide Children’s Hospital,
(> 50 chromosomes) B-acute lymphoblastic leukemia (B-ALL) have Pathology, Columbus, United States of America; 8 University of Alabama
favorable outcomes. St. Jude classification considers these patients at Birmingham, Genetics, Birmingham, United States of America; 9 Maine
(with DNA index ≥ 1.16 if hyperdiploid) provisional low-risk (LR), Children’s Cancer Program, Pediatric Oncology, Scarborough, United States
regardless of age and presenting leukocyte count (National Cancer of America; 10 NYU Langone Health, Pediatrics, New York, United States
Institute [NCI] risk), and upgrades them if minimal residual disease of America; 11 UT Southwestern/Simmons Cancer Center, Pediatric Hema-
(MRD) is ≥1% on day 15 or ≥ 0.01% on day 43 or there is testicular or tology/oncology, Dallas, United States of America; 12 Children’s Hospital of
central nervous system (CNS3) involvement. Philadelphia, Pediatrics, Philadelphia, United States of America; 13 Seattle
Methods: We analyzed outcomes of children (1-18.99 years) with Children’s Hospital, Pediatrics, Seattle, United States of America
these genotypes in the St. Jude Total 15 and 16 studies (2000-2017)
based on NCI and St. Jude risk. Background and Aims: Background: Overall survival (OS) is >90%
Results: Patients with ETV6-RUNX1 (n = 222) or hyperdiploid for pediatric acute lymphoblastic leukemia (ALL). However, patients
(n = 296) B-ALL had 5-year event-free survival (EFS) of 97.7% ± 1.1% with B-ALL and intrachromosomal amplification of chromosome 21
and 94.7% ± 1.4%, respectively. For ETV6-RUNX1, EFS did not (iAMP21) are at very high risk (VHR) of relapse.
differ between “NCI” standard-risk (SR) (n = 182; 97.8% ± 1.2%) Methods: Children’s Oncology Group (COG) AALL1131 enrolled
and high-risk (HR) (n = 40; 97.5% ± 2.6%) patients (P = 0.917) or patients 1-30 years old with newly diagnosed High-Risk (HR) B-ALL.
between “St. Jude” LR (n = 195; 97.4% ± 1.2%) and SR (n = 27; 100.0%) Following induction, iAMP21+ patients were risk-stratified to the VHR
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S118 of S635 ABSTRACTS

cohort, which also included Standard-Risk (SR) B-ALL patients initially ing expression differences in lncRNAs through a high throughput RNA
enrolled on AALL0932, who crossed over to AALL1131 post-induction. sequencing approach.
In total, 330 patients with iAMP21 (142 SR; 188 HR) were enrolled and Methods: Total RNA was extracted from tumor samples at diagno-
treated on AALL1131. sis of 52 pediatric ALL patients treated following the same protocol
Results: At end of induction (EOI), 37.6% of iAMP21+ patients had (SEHOP-PETHEMA 2013) in three Spanish hospitals. RNA libraries
flow cytometry minimal residual disease (MRD) >0.01% versus 21.6% (stranded and without rRNA) were generated and sequenced with a
of iAMP21- patients. The 5-year event-free survival (EFS) and OS for depth of 150 million paired-reads using Illumina technology. Different
NCI HR B-ALL iAMP21+ were 69.7% (95% Confidence Interval (CI), approaches were evaluated for alignment, quantification and differ-
56.0%, 79.8%) and 83.9% (95% CI, 70.5%, 91.6%), respectively. Both 5- ential expression analysis. Kaplan-Meier and Cox Proportional Hazard
year EFS/OS were significantly worse for NCI HR iAMP21+ patients Model methods were used to perform 5-year event-free survival (EFS)
who were EOI MRD-positive: 55.7% (95% CI, 29.2%, 75.7%) and 71.2% analyses.
(95% CI, 38.5%, 88.6%) versus EOI-MRD negative 78.3% (95% CI, Results: A total of 25,783 expressed lncRNAs were identified in our
62.0%, 88.2%) and 90.9% (95% CI, 75.6%, 96.8%) (p=0.002; p=0.007), patient population from 56,846 lncRNAs annotated in LNCipedia.
respectively. The 5-year EFS/OS for NCI SR iAMP21+ patients were Among them, 18 lncRNAs were differentially expressed (padj <0.01;
76.1% (95% CI, 64.6%, 84.3%) and 89.1% (95% CI, 79.6%, 94.3%). log2FC>2/<-2) comparing relapsed (n=7) vs. non-relapsed patients
Similar findings were reported in this NCI SR cohort based on (n=45). Patients were divided into three groups for survival analy-
EOI MRD where both EFS/OS were significantly worse in patients ses: “High expression”, “Low expression”, and “No expression”. From the
with EOI MRD >0.01%: 63.9% (95% CI, 44.1%, 78.3%) and 81.2% 18 significant lncRNAs, 14 showed significant differences (p<0.05) in
(95% CI, 62.9%, 91.1%) versus EOI MRD-negative 85.8% (95% CI, Kaplan-Meier survival analyses. These results point to an important
71.3%, 93.3%) and 96.0% (95% CI, 83.6%, 99.1%) (p=0.001; p=0.009), role of lncRNA expression in treatment response, and warrant further
respectively. downstream studies to understand their role in the pathogenesis of the
Conclusions: Despite post-induction VHR therapy, outcome remains disease and as potential new therapeutic targets.
poor for NCI SR and HR BALL iAMP21+ patients. Patients with Conclusions: Our high throughput approach identified 14 lncRNAs
iAMP21 who are EOI MRD-positive have significantly inferior EFS/OS, associated with relapse and EFS, suggesting that lncRNA expression
warranting new treatment approaches for these patients. pattern could serve as prognostic predictor in pediatric ALL.

O238 / #652 NEW PROGNOSTIC BIOMARKERS IN ACUTE O239 / #719 RACIAL AND ETHNIC OUTCOME DISPARITIES
LYMPHOBLASTIC LEUKEMIA: LONG NON-CODING RNAS AMONG PATIENTS WITH ACUTE LYMPHOBLASTIC LEUKEMIA
ARE NOT FULLY ATTENUATED BY DISEASE PROGNOSTICATORS
Unai Illarregi1 , Nerea Bilbao-Aldaiturriaga1 , Anastasija Jakjimovska1 , OR SOCIOECONOMIC STATUS: A COG STUDY
Ángela Gutierrez-Camino2 , Rafael Del Orbe3 , Itziar Astigarraga4 ,
Mireia Camos-Guijosa5 , Manuel Ramírez6 , Idoia Martin-Guerrero1 , Sumit Gupta1 , Yunfeng Dai2 , Zhiguo Chen3 , Lena Winestone4 , David
Elixabet Lopez-Lopez7 Teachey5 , Kira Bona6 , Richard Aplenc7 , Karen Rabin8 , Patrick
1 University of the Basque Country and IIS Biocruces Bizkaia, Genetics, Zweidler-Mckay9 , Andrew Carroll10 , Nyla Heerema11 , Julie
Physical Anthropology And Animal Physiology, Leioa, Spain; 2 Sainte-Justine Gastier-Foster12 , Michael Borowitz13 , Brent Wood14 , Kelly
University Health Center, Division Of Hematology-oncology, Research Cen- Maloney15 , Leonard Mattano Jr16 , Eric Larsen17 , Anne Angiolillo18 ,
ter, Montreal, Canada; 3 Hospital Universitario Cruces, IIS Biocruces Bizkaia, Michael Burke19 , Wanda Salzer20 , Stuart Winter21 , Patrick Brown22 ,
Hematology Department, Barakaldo, Spain; 4 Hospital Universitario Cruces, Erin Guest23 , Kimberley Dunsmore24 , John Kairalla2 , Naomi Winick25 ,
IIS Biocruces Bizkaia, Pediatrics Department, Barakaldo, Spain; 5 Institut William Carroll26 , Elizabeth Raetz27 , Stephen Hunger28 , Mignon
de Recerca Pediátrica Hospital Sant Joan de Déu, Hematology Labora- Loh29 , Meenakshi Devidas30
tory, Barcelona, Spain; 6 Hospital Universitario Infantil Niño Jesús, Instituto 1 Hospital for Sick Children, Haematology/oncology, Toronto, Canada;
de Investigación Sanitaria Princesa (IIS-IP), Pediatric Oncohematology, 2 University of Florida, Biostatistics, Gainesville, United States of Amer-
Madrid, Spain; 7 University of the Basque Country and IIS Biocruces Bizkaia, ica; 3 Children’s Oncology Group, Biostatistics, Gainesville, United States
Biochemistry & Molecular Biology, Leioa, Spain of America; 4 UCSF Benioff Children’s Hospitals, Allergy, Immunology,
And Bmt, San Francisco, United States of America; 5 Children’s Hospi-
Background and Aims: Acute Lymphoblastic Leukemia (ALL) remains tal of Philadelphia, Paediatrics, Philadelphia, United States of America;
an important cause of death from disease in children. Therefore, the 6 Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, Pedi-
identification of new outcome predictors and therapeutic targets is still atric Oncology, Boston, United States of America; 7 Children’s Hospital of
a need. In this context, long non-coding RNAs (lncRNAs), which rep- Philadelphia, Oncology, Philadelphia, United States of America; 8 Texas Chil-
resent an important fraction of the functional genome that has been dren’s Hospital / Baylor College of Medicine, Pediatrics - Oncology, Houston,
poorly studied to date, could be novel candidates with great potential. United States of America; 9 ImmunoGen, Inc., Managing Director, Waltham,
This work aimed to identify new biomarkers of ALL relapse, analyz- United States of America; 10 University of Alabama at Birmingham, Genetics,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S119 of S635

Birmingham, United States of America; 11 Nationwide Children’s Hospital, relapse, relapses involving the CNS, testicular relapse, and death in
Pathology, Columbus, United States of America; 12 Texas Children’s Hospital, remission. Relapses in these three ethnic/racial groups occurred earlier
Global Hematology/oncology Pediatric Excellence, Houston, United States than relapses in non-Hispanic White or non-Hispanic Asian patients.
of America; 13 The Johns Hopkins Hospital, Pathology, Baltimore, United Conclusions: Substantial disparities in outcome for B-ALL persist by
States of America; 14 Children’s Hospital Los Angeles, Pathology And Labora- race/ethnicity, but are not observed in T-ALL. Underlying mechanisms
tory Medicine, Los Angeles, United States of America; 15 Children’s Hospital may vary between disadvantaged groups. Future studies of relapsed
Colorado, Hematology/oncology - Pediatric, Aurora, United States of Amer- patients, and of access to and quality of care are warranted to inform
ica; 16 HARP Pharma Consulting, LLC, Drug Development, Mystic, United interventions aimed at dismantling racial and ethnic disparities.
States of America; 17 Maine Children’s Cancer Program, Pediatric Oncology,
Scarborough, United States of America; 18 Children’s National Medical Cen-
ter, Center For Cancer And Blood Disorders, Washington, DC, United States CCI
of America; 19 Children’s Wisconsin, Pediatrics, Milwaukee, United States of
America; 20 U.S. Army Medical Research and Materiel Command, Pediatrics, CCI: PROVIDING EDUCATIONAL AND EMOTIONAL SUPPORT
Fort Detrick, United States of America; 21 Children’s Hospitals and Clinics of AND IMPROVING COMMUNICATION 01-10-2022 10:40 AM -
Minnesota, Pediatric Hematology & Oncology, Minneapolis, United States 12:10 PM
of America; 22 The Johns Hopkins Hospital, Oncology, Baltimore, United
States of America; 23 Children’s Mercy Kansas City, Hematology/oncology, O240 / #246 SCHOOLING AFTER THE DIAGNOSIS OF
Kansas City, United States of America; 24 Virginia Tech University, Pediatrics, CHILDHOOD CANCER: PARENT PERSPECTIVES
Roanoke, United States of America; 25 UT Southwestern/Simmons Cancer
Center, Pediatric Hematology/oncology, Dallas, United States of America; Kathy Ruble1 , Lisa Jacobson2 , Lisa Carey2 , Clifton Thornton3 ,
26 NYU Langone Health, Pediatrics, New York, United States of America; Kimberly Milla2 , Juliana Paré-Blagoev4
27 NYU Langone Health, Pediatrics, New York City, United States of Amer- 1 Johns Hopkins University, School of Medicine, Pediatric Oncology, Balti-
ica; 28 Children’s Hospital of Philadelphia, Pediatrics, Philadelphia, United more, United States of America; 2 Kennedy Krieger Institute, Neuropsychol-
States of America; 29 Seattle Children’s Hospital, Pediatrics, Seattle, United ogy, Baltimore, United States of America; 3 Johns Hopkins University, School
States of America; 30 St Jude Children’s Research Hospital, Global Pediatric of Nursing, Nursing, Baltimore, United States of America; 4 Johns Hop-
Medicine, Memphis, United States of America kins University, School of Education, Education, Baltimore, United States of
America
Background and Aims: Previous studies identified racial and eth-
nic disparities in childhood acute lymphoblastic leukemia (ALL) sur- Background and Aims: More than 50% of children treated for cancer
vival. We determined whether disparities persist in contemporaneous will have neurocognitive impacts of therapy, which may require aca-
cohorts and if present, are attributable to differences in leukemia demic supports and influence quality of life (QOL). Over the past 4
biology or socioeconomic status (SES). years, our team has conducted 2 Patient-Centered Outcomes Research
Methods: Patients with newly diagnosed ALL, 0-30 years of age, Institute (PCORI) engagement projects with parents, educators and
enrolled on Children’s Oncology Group (COG) trials between 2004- healthcare providers to better understand and support schooling after
2019 were included. Race/ethnicity was categorized as non-Hispanic cancer. The aims of this presentation are 1) Highlight parent-reported
White, Hispanic, non-Hispanic Black, non-Hispanic Asian, and non- experiences and challenges associated with schooling after cancer,
Hispanic Other. Insurance status served as a proxy for SES. Event-free and 2) Introduce parent informed school integration ‘Roadmaps’ for
and overall survival (EFS, OS) were compared across race/ethnicity. The addressing frequently reported challenges.
relative contribution of clinical and biologic disease prognosticators Methods: Initial parent interviews (n=10) explored the processes and
and SES was examined. challenges associated with schooling after cancer diagnosis. These
Results: The cohort included 21,152 patients. Five-year EFS was data were used to develop a descriptive model and to inform a
87.4%±0.3% among non-Hispanic White patients vs. 82.8%±0.6% [HR nationally disseminated parent survey. 175 parents provided quan-
1.37, 95% confidence interval (95CI) 1.26-1.49; p<0.0001] among His- titative and qualitative data which were analyzed to identify impact
panic patients and 81.9%±1.2% (HR 1.45, 95CI 1.28-1.56; p<0.0001) of schooling challenges on QOL and explore how parents navigate
among non-Hispanic Black patients. Inferior EFS among Hispanic the advocacy required to support schooling in survivorship. Find-
patients was substantially attenuated by disease prognosticators and ings informed development of parent-focused resources addressing
SES (HR decreased from 1.37 to 1.11; p=0.045). The increased risk identified informational needs.
among non-Hispanic Black patients was minimally attenuated (HR 1.45 Results: The model developed reflects 3 themes (Communica-
to 1.32). Disparities in OS were wider than EFS. Disparities were tion/Knowledge/Process) that impact schooling challenges. Parents
restricted to B-ALL patients as no differences in EFS or OS were reported their child was stressed about returning to school (60.2%),
seen in T-ALL based on race/ethnicity. Among patients with B-ALL, that the cancer diagnosis made their child more anxious (70.2%), more
Hispanic, non-Hispanic Black, and non-Hispanic Other patients experi- sensitive to peers (73.4%), and made socializing (58.2%) and fitting
enced higher cumulative incidences of relapse, isolated bone marrow in with peers (49.7%) more difficult. Parent preparedness, access to
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S120 of S635 ABSTRACTS

social and cultural capital, and perceived supportiveness of teach- O242 / #460 THE IMPACT OF THE ARTS ON HOSPITAL
ers were associated with higher school QOL. Six ‘Return to School ADAPTATION AND TREATMENT COOPERATION FOR
Roadmaps’ with actionable information for families were developed PEDIATRIC PATIENTS WITH CANCER
and shared online to improve preparedness for the return-to-school
period following cancer diagnosis. Hazal Hüzmeli1 , Herdem Aslan Genc2 , Aslıhan Ozcan Morey3 , Rejin
Conclusions: Schooling after childhood cancer can be challenging Kebudi4
and requires parents to be knowledgeable advocates. The infor- 1 American Hospital, Pediatrics, Istanbul, Turkey; 2 Koc University Hospital,
mation in this presentation can help parents better understand Psychiatry, Istanbul, Turkey; 3 Koc University Hospital, Pediatrics, İstanbul,
schooling challenges and provide potential tools for addressing Turkey; 4 Istanbul University, Oncology Institute, Pediatric Hematology-
them. oncology, Istanbul, Turkey

Background and Aims: There is a growing awareness of the impor-


O241 / #1784 IMPLEMENTATION OF THE MODEL OF tance of the emotional well-being of children with cancer and their
HYBRID CLASSES IN HOSPITAL SCHOOLS OF FUNDACIÓN families. The treatment team within a pediatric oncology setting should
NUESTROS HIJOS: EXPERIENCE OF OUR STUDENTS AND develop programs that may ease the psychological burden of cancer
TEACHERS treatment. The primary goal of this research is to evaluate the impact
of art making in the hospital setting on the hospital adaptation and
Rita Gangale1 , Dunja Roje2 , Valentina Espinoza3 , Nuria Rossell3 , treatment compliance.
Marcela Zubieta4 Methods: Twenty-five children participated in a cohort study. The
1 Fundación Nuestros Hijos, Dirección Colegios Hospitalarios, Santiago, participants were assigned to two different types of groups: art inter-
Chile; 2 Fundación Nuestros Hijos, Gerencia Técnica, Santiago, Chile; vention (n=15) or standard care (n=10) based on the presence of art
3 Fundación Nuestros Hijos, Extensión Y Desarrollo, Santiago, Chile; leading nurse in the unit. Hospital adaptation and treatment compli-
4 Fundación Nuestros Hijos, Presidencia, Santiago, Chile ance were assessed through surveys filled by nurses caring for each
participant. Additionally, KINDL Health-Related Quality of Life in chil-
Background and Aims: Since 1997 Fundación Nuestros Hijos (FNH) dren and adolescents- oncology module, Generalized Anxiety Disorder
operates two hospital schools in Santiago, Chile. These schools, rec- Assessment, Children’s Depression Inventory, and a symptoms check-
ognized by the Ministry of Education, aim to allow schooling continu- list were filled by children and their parents. Data were analyzed using
ation of children undergoing cancer treatment. Although average year descriptive, correlational, and qualitative techniques. Artworks were
enrollment of 80 - 85 students remains steady, regular class attendance analyzed by 2 independent reviewers.
fluctuates significantly. Due to the COVID-19 pandemic lockdown, Results: support the potential effectiveness of integrating art mak-
the schools were forced to innovate education strategies and imple- ing in the standard psychosocial care for better hospital adaptation
ment an online model accessible for all students. After presential and treatment compliance. Additionally, patients’ artwork provided
classes resumed, a combined modality of on-line and face-to-face has insightful information on their thoughts and feelings about the hospital
been adopted. This project seeks to measure the impact of the hybrid and treatment.
program since its implementation. Conclusions: Engaging in art making in the hospital setting is an effec-
Methods: On-line classes were implemented in the 2020 school year tive tool in improving the adaptation to the hospital and cooperation
and tablets and internet connection were provided to the students. with treatments for pediatric oncology patients. Children are able to
Parents were invited to participate in on-line workshops. Registered communicate better with family and providers through art. Treatment
enrollments and daily class attendance of school years 2019 (in person teams should use art as a supportive tool for higher quality care and as
only), 2020 (on-line only), and second semester of 2021 (hybrid) were means of proper communication with children to gain more insight on
compared. Parents satisfaction surveys regarding on-line classes were the needs of this special patient population. There is need for further
also analyzed. research in this field.
Results: In 2021 (hybrid) attendance improved from 70% to 98%.
Enrollment was nearly 70 students in each of the three years analyzed.
Surveyed parents appreciated the extracurricular workshops and the O243 / #1923 DOCTORS ARE HUMANS
provision of means to connect to classes but they expressed worry
about the many hours of classes. Mercedes Guibelalde Del Castillo
Conclusions: The hybrid model for hospital schools adopted by Son Espases, Pediatric Oncology, Palma, Spain
FNH showed positive results and potential for improvement. It
was effective for delivery of school contents beyond the period Background and Aims: I am a pediatric oncologist. I got cancer. How
of the pandemic, and can be permanently adopted by the hospital can that be possible? : Doctors are human. I was healthy (most of my
schools, allowing all students to continue classes despite their health patients are before they have cancer). I wanted to continue intensify-
situation. ing my knowledge to be a more honest and empathetic doctor, to be
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S121 of S635

able to comment first hand. Something that was not in the Faculty of ment attributes mandatory for providing excellence in PPC. Consensus
Medicine, which I believe should offer: diseases and illness. So maybe from 11 PPC multidisciplinary experts was obtained during four Del-
then Idoctors would not talk nonsense to their patients. phi rounds regarding the most important questions to include in a PPC
Methods: Discusion of the experience of being myself an oncology assessment tool. During the final Delphi round five, expert consensus
patient. You that have worked worked hard to put on your doctor’s was confirmed in a separate group of 36 childhood cancer/palliative
gown; one day in the morning you are a doctor, and now what if is it care clinical providers.
your turn? In the afternoon, it is the patient’s gown that you have Results: Five core elements were developed as the foundation for a
Results: I am grateful that I have been given my second chance. PPC assessment: symptom assessment, goals of care, spiritual beliefs
Opportunity to be a doctor again after being patient. Being an hybrid and values, psychosocial support and grief and bereavement. Symptom
patient-doctor part time. If you are interested I will share with par- assessment, one of the core elements, includes 15 symptoms that all
ents and oncologist how I now I understand things differently: never PPC experts agreed are experienced more than 65% of the time in their
say again “I know how you feel”. patients.
Conclusions: Disease makes you grow. False. It is a humble pie. To doc- Conclusions: The need for a culturally sensitive PPC assessment tool is
tors: please be patient. Humanization of medicine is if your doctor has crucial to promoting excellence in palliative care around the globe. The
time, time, and time to listen to patients and let their stories come Delphi method was an effective tool to develop a consensus on a PPC
into you. It is worth it if you let yourself be touched by your patients’ assessment tool to use with children and their families in sub-Saharan
experiences and their families because someday they will accompany Africa. This standardized approach will focus on symptom assessment
you and improve the quality of care for children and their families at the end
of life.

NURSING
O245 / #264 CHEMOTHERAPY SAFETY: AUDIT OF
NURSING ORAL ABSTRACT PRESENTATIONS: INNOVATIVE PRESCRIPTION AND ADMINISTRATION IN A TERTIARY
METHODS TO STANDARDIZE CARE 01-10-2022 10:40 AM - REFERRAL UNIT
12:10 PM
Isaac Mulyowa1 , Joyce Kambugu2 , Dennis Williams3 , Gemma
O244 / #152 USING THE DELPHI METHOD TO DEVELOP A Barnard3 , Rukundo Beatrice1 , Rose Nankinga1
PALLIATIVE CARE TOOL FOR CHILDREN AND FAMILIES IN 1 Uganda Cancer Institute, Pediatric Oncology Clinic, Kampala, Uganda;
SUB-SAHARAN AFRICA 2 Uganda Cancer Institute, Pediatric Oncology, Kampala, Uganda;
3 Addenbrooks children’s Hospital, Pediatric Oncology, QQ, United Kingdom

Abenawe Cosiate1 , Jessica Casas2 , Kamusisi Chinyundo3 , Rhahim


Bank4 , Babe Gaolebale5 , Annet Nakirulu6 , Goitseone Background and Aims: The Ugandan Cancer Institute (UCI) is a
Maifale-Mburu5 , Joy Hesselgrave2 , Deogratius Bakulumpagi6 , tertiary referral unit for pediatric oncology that receives 700 new
Immaculate Nassanga1 , Jennifer Higgins7 , Marilyn Hockenberry7 referrals annually. As in many LMIC, the staff to patient ratio is low. This
1 Global HOPE Uganda, Nursing, Kampala, Uganda; 2 Texas Children’s Hos- led to concern about the potential for errors in the prescription and
pital, Palliative Care Program, Houston, United States of America; 3 Global administration of chemotherapy, and highlighted the need to improve
HOPE Botswana, Palliative Care, Gaborone, Botswana; 4 Global HOPE overall safety in this area.
Malawi, Nursing, Lilongwe, Malawi; 5 Princess Marina Hospital, Palliative Methods: An audit tool was developed to look at prescription and
Care, Gaborone, Botswana; 6 Global HOPE Uganda, Paediatrics, Kampala, administration of chemotherapy, which was conducted in the clinic and
Uganda; 7 Baylor College of Medicine, Global Hope, Houston, United States inpatient ward over a 2-week period. It included demographic data,
of America diagnosis and staging information, assignment of correct treatment
protocol, pre-chemo checks, details of prescribed chemotherapy and
Background and Aims: In Sub-Saharan Africa there is no standard- supportive medications.
ized approach to paediatric palliative care assessment. Because of this, Results: A total of 83 patients were audited over a 2-week period
there is a critical demand for evidence-based assessment tools that (34 inpatients and 59 outpatients). Some areas of good practice were
identify specialized needs of children and their families requiring pallia- noted, but areas for improvement were also identified. Notable areas
tive care in developing countries. The aim of this project was to develop of good practice included: 94% patients had complete staging docu-
a standardized approach to pediatric palliative care (PPC) assessment mented, and 95% had histological confirmation. 88% had the correct
for use in sub-Saharan Africa. protocol assigned and signed off by a consultant or fellow. 100% had a
Methods: The Delphi method is a group facilitation procedure that documented body surface area. Over 98% had the correct cycle pre-
involves iterative multistage processes designed to transform opinion scribed, and 94% of prescriptions had consultant or fellow sign-off.
into group consensus. In this project Delphi method approach used 98% prescriptions were administered on time. Areas for improve-
five rounds to explore core elements that define the essential assess- ment included fluid charts, antiemetic and supportive medication
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S122 of S635 ABSTRACTS

prescription. 64% had a fluid chart appropriate for the patient’s cated that the training toolkit was effective preparation for nurses. The
chemotherapy and 65% had an appropriate antiemetic prescribed. 66% need for video training tools and edits to workflow tools was identified.
patients had an appropriate prescription for other supportive medi- Four video segments were created to demonstrate aspects of adminis-
cation. No significant harm was identified as a result of any error of tration and handling of blinatumomab and added to the toolkit along
prescription or administration. with revised tools. Future sites will undergo standardized evaluations,
Conclusions: The formation of a multidisciplinary committee of doc- including pre- and post-tests.
tors, nurses and pharmacy was proposed to improve the safety Conclusions: Implementation of novel therapeutics requires multidis-
of chemotherapy prescription and administration, ensuring proto- ciplinary collaboration and discipline-specific training. A standardized
cols were assigned correctly, checked by senior staff, prescrip- training toolkit aids in the scalability of implementation of novel thera-
tions were clear and included appropriate supportive fluids and peutics and could be replicated for other medications. Future sites will
medication. benefit from adaptations and experience from pilot, along with further
adaptations for resource level and language.

O246 / #735 DEVELOPMENT AND IMPLEMENTATION OF A


STANDARDIZED TRAINING TOOLKIT FOR NURSES O247 / #1115 IMPROVING INTRAVENOUS FLUID
ADMINISTERING BLINATUMOMAB IN LOW- AND MONITORING AND DOCUMENTATION AMONG NURSES ON
MIDDLE-INCOME COUNTRIES THE PAEDIATRIC ONCOLOGY WARD - A STUDY AT THE KORLE
BU TEACHING HOSPITAL, ACCRA, GHANA
Liz Sniderman1 , Jennifer L. Pauley2 , Hiroto Inaba3 , Cody Mcmillan3 ,
Laura Tan Mei Lian4 , Caitlyn Duffy3 Wendy Eyiah-Mensah, Evelyn Owiredu
1 Stollery Children’s Hospital, Northern Alberta Children’s Cancer Care Pro- Korle Bu Teaching Hospital, Child Health, Accra, Ghana
gram, Edmonton, Canada; 2 St Jude Children’s Research Hospital, Global
Pediatric Medicine, Memphis, United States of America; 3 St Jude Children’s Background and Aims: Fluid management is an important component
Research Hospital, Oncology, Memphis, United States of America; 4 Khoo of oncology nursing care. It is therefore essential to properly moni-
Teck Puat National University Children’s Medical Institute, Department Of tor and document all intravenous fluids administered to patients with
Pediatrics, Singapore, Singapore cancer. We recognized a challenge with fluid management on the Pae-
diatric Oncology Unit (POU) of the Korle Bu Teaching Hospital (KBTH),
Background and Aims: Novel therapies have led to improved with majority of patients noted to be receiving less than prescribed
outcomes in pediatric cancer care. Successful implementation fluid volumes at shift handover rounds. This resulted in complications
of novel therapies requires specialized training on administra- such as delays with start of chemotherapy and the potential for tumour
tion and management. We describe our experience creating and lysis syndrome. The study aim was to describe the fluid monitoring
implementing a standardized training toolkit for nurses admin- and documentation practices on the unit so that measures, such as
istering blinatumomab in low- and middle-income countries continuous nursing education, could be put in place to improve them.
(LMICs). Methods: A quantitative data collection method using patients’ files
Methods: The core multidisciplinary team of the Blincyto Human- were used, looking specifically at their total intravenous fluid intake
itarian Access Program, a partnership between St. Jude Children’s at end of each day. Eleven (11) patients on intravenous fluids were
Research Hospital and AMGEN, identified key administration and selected at random each day for a week (7days).
management issues for blinatumomab. These were used to create a Results: Majority of the patients (82%) did not receive the required
questionnaire assessing baseline capacity at LMIC pilot sites. Survey amount, with 15% having fluid overload and only 3% getting their
results informed content creation for a training program including required amount. Three potential explanations for the problem
didactic presentations, administration and management guidelines, were identified. 1.Lack of continuous monitoring of fluids after
workflow tools, and caregiver information sheets. Nursing workflow initial set up. 2.Delays in setting intravenous lines when needed.
tools include daily care checklist, infusion record, and quick refer- 3. Inappropriate positioning of the cannula resulting in poor
ence sheet. Training included virtual, synchronous sessions that were flow
recorded and posted to an online classroom with other toolkit materi- Conclusions: There is an urgent need to improve intravenous fluid
als. Following pilot site implementation, feedback sessions were held monitoring and documentation on the unit. This can be done by organ-
to evaluate effectiveness of training and iteratively revise toolkit ising training sessions for staff to educate them on the importance
materials. of intravenous fluids and the need for patients to receive the pre-
Results: Pilot site training included 40 nurses at five sites in two scribed volume over the specified time period. Checklists should also
countries. Two virtual, interactive sessions were held; the first session be developed to remind staff to continuously monitor intravenous flu-
was multi-disciplinary and the second was nursing-specific, focused ids. One nurse should be designated per shift to monitor intravenous
on administration and bedside management. Feedback sessions indi- fluid administration.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S123 of S635

O248 / #120 IMPACT OF AN INTERVENTION TO IMPROVE Beatrice Rukundo


THE ADMINISTRATION TIME FOR THE FIRST DOSE OF UGANDA CANCER INSTITUTE- KAMPALA, Pediatric Oncology, KAMPALA,
ANTIMICROBIALS IN EPISODES OF FEBRIL NEUTROPENIA IN Uganda
CHILDREN WITH CANCER
Background and Aims: Pain is a very unpleasant feeling and dis-
Veronica De La Maza1 , Daniela Simian1 , Juan Pablo Torres1 , María tress. it can be physical or psychological pain. A lot of pharmacological
Elena Santolaya1 , Carolina Robledo2 , Virginia Fierro3 , Karen measures have been used to reduce pain and little attention paid to
Ducasse4 , Soraya Masquiarán5 , Giovanna De Vicco6 , Carolina non-pharmacological measures, which in turn have fewer effects and
Salazar7 , Carolina Facusse8 , Carla Vega9 , Lorena Segovia10 are less costly. Therefore, the purpose of this study was to measure
1 Hospital Luis Calvo Mackenna/ Universidad de Chile, Research Unit, San- the impact of using non-pharmacological measures on chemotherapy-
tiago, Chile; 2 Oncology Unit, Sótero Del Río Hospital, santiago, Chile; induced pain among pediatric cancer patients. These measures include
3 Oncology Unit, Roberto Del Río Hospital, Santiago, Chile; 4 Oncology Unit, occasional dance sessions and watching cartoons.
Gustavo Fricke Hospital, Santiago, Chile; 5 Oncology Unit, San Borja Arriarán Methods: This study enrolled 125 childhood cancer patients, 78
Hospital, Santiago, Chile; 6 Oncology Unit, Valdivia Hospital, Valdivia, Chile; females and 52 males, age group 3-13 years. Occasional dance ses-
7 Oncology Unit, San Juan De Dios Hospital, Santiago, Chile; 8 Oncology Unit, sions and watching of cartoon movies were introduced and shown to
Exequiel González Cortés Hospital, santiago, Chile; 9 Oncology Unit, Antofa- the pediatric cancer patients in the outpatient unit of Uganda Can-
gasta Hospital, Antofagasta, Chile; 10 Oncology Unit, Luis Calvo Mackenna cer Institute. The effects of watching cartoons were assessed while
Hospital, Santiago, Chile administrating chemotherapy (for both Liquid and Solid tumors) using
the FLACC scale. The effects of the dancer sessions were assessed
Background and Aims: Time-to-antibiotic (TTA) administration is a among patients awaiting intramuscular injection of L-asparaginase (L-
widely used quality-of-care measure for children with cancer, fever aspa) using the FLACC scale (Face, Legs, Activity, Cry, Consolability).
and neutropenia (FN). The aim of this study was to determine the The period of assessment was 6 months
TTA and its relationship with clinical outcomes before and after the Results: There were 20 sessions altogether by the end of the sixth
implementation of a new Intervention. month. On average there were 12 children waiting for chemother-
Methods: Experimental, prospective and multicenter study between apy daily. A total of 125 childhood cancer patients, 78 females and
April 2018 and January 2021. We evaluated the TTA, comparing 52 males participated in this study, with an average age of 8 years.
this variable by hospital and presentation location (Emergency Room/ Fifty-nine females –were distracted and 30 males showed varying lev-
Oncology Units) and the clinical outcomes by the following variables: els of distraction. Two patients showed mixed reactions (distracted and
days of fever, days of hospitalization, hypotension, transfer to intensive irritable) on different session days.
care unit, sepsis and mortality, before and after a new Intervention that Conclusions: Generally, this study showed that non-pharmacological
included education, guidelines and system changes. measures are effective in the reduction of distress and pain. This
Results: A total of 469 episodes of FN were enrolled from 9 hospitals method is safe and has no medicinal side effects. Non-pharmacological
in Chile. Comparing before and after the Intervention, the TTA was measures can be easily applied and are likely to require lesser finan-
92 vs 83 minutes, and a significance difference was obtained between cial implications, compared to pharmacological measures. Much as
both periods in the TTA curve (log rank test = 0.0006). In Emergency the study looked at the reduction of pain, it may have an impact on
Room, after the protocol the TTA statistically decreased from 107 to anticipatory nausea and Vomiting
87 minutes (p=<0.001). In multivariate analysis, the TTA after 120
minutes was associated with negative transfer to intensive care unit,
hypotension and sepsis (Odds Ratio 14.7, 95% CI 2.6 – 81.8, p=0.002). FREE PAPER SESSION (FPS)
Conclusions: Our results demonstrate that the local implemen-
tation of an Intervention can reduce TTA. Also we observed FPS 20: SUPPORTIVE CARE - GLOBAL HEALTH AND INFECTION
negative outcomes in episodes with TTA > 120 minutes, OUTCOMES 01-10-2022 10:40 AM - 12:10 PM
therefore, prompt attention, proper testing and antibiotic ther-
apy administration as soon as possible are required in this O250 / #929 TREATMENT ABANDONMENT IN CHILDREN
population. WITH CANCER: ANALYSIS FROM A TERTIARY CARE HOSPITAL
IN A LOW MIDDLE INCOME COUNTRY (LMIC)

O249 / #275 USE OF NON- PHARMACOLOGICAL MEASURES Kajori Nandy, Jayashree Muralidharan, Deepak Bansal, Bhavneet
IN REDUCTION OF CHEMOTHERAPY INDUCED PAIN AMONG Bharti, Richa Jain, Amita Trehan
PEDIATRIC CANCER PATIENTS: A STUDY AT UGANDA CANCER Postgraduate Institute of Medical Education & Research, Pediatrics, Chandi-
INSTITUTE’S PEDIATRIC OUTPATIENT UNIT garh, India
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S124 of S635 ABSTRACTS

Background and Aims: Abandonment is one of the dominant causes of treatment, has the training they need to heal children suffering from
treatment failure in LMIC’s. This analysis was performed to assess the cancer.
magnitude and identify the reasons for abandonment. Methods: In 2012 we initiated a collaboration with African countries in
Methods: Children diagnosed between July 2020 and February 2022 response of a national outreach initiative to reach out to all our neigh-
were followed for 6 months to record abandonment of therapy. Rea- boring nations, to share and disseminate the knowledge and resources
sons for abandonment were recorded by a physical or telephonic of CCHE, thus bridging the gaps in Children’s care. We collaborated and
structured questionnaire. partnered with major hospitals and academic institutions to train local
Results: Abandonment was observed in 122/763 (16.5 %) patients with and regional medical professionals to recognize cancers early, diagnose
upfront refusal in 72(59%) and 50 (41%) abandoning after 4 (IQR 2- them accurately, and treat them effectively through structured training
8) weeks of therapy. Mean age: 4.5 years (0.3-12 years), 71 males and programs developed according to International standards and tailored
51 females. Diagnosis: ALL: 33%; AML: 12%; Neuroblastoma: 11%; to suit the continental needs.
Retinoblastoma: 10% and other solid tumours (17%) with 22/59 solid Results: Throughout our journey, we have succeeded to train physi-
tumours having metastatic disease and 48/122(39.3%) a poor prog- cians from (Ethiopia, Kenya, Kuwait, and Sudan) through our fellowship
nosis. Majority(79%) lived >100km from our center, 11% residing training program in collaboration with DF/BC that engages world-
within 50 km. Twenty percent families were illiterate, 42% fathers renowned hematologists and oncologists to provide on-site and online
being unskilled workers ; unemployed( 3.3%) professionals (5.7%) & training to specialists in pediatric oncology/hematology. We have
self employed( 5%). Average monthly income was USD 200, (GDP per trained more than 600 nurses from different African countries in
capita USD 160). Most families were nuclear (85%), 2/3rd belonging to intensive pediatric hematology/oncology nursing training pursuant.
rural areas. Nearly 70% patients were eligible for financial aid from the We connected more than 100 clinical pharmacists at our partner insti-
government. Reasons for abandonment were multifactorial: finances & tutions with off-site training programs designed to build knowledge
distance from centre being predominant (42% & 19%); belief of no cure and capability in chemotherapy preparation, and pharmacotherapy.
12%; fear of chemotherapy 13%; family problems 12%; poor progno- We also assisted our partners in developing on-site oncology phar-
sis 10%; inability to get leave from work 6.6%. Thirteen (10.6%) took macies. We have partnered with the International agency of Nuclear
alternative medicine. On patient tracking, 14(11%) are on treatment energy to train tens of African and Arabic radiotherapists and Radio-
elsewhere and 13 (10.6%) returned for therapy after 6.5 (2-12) weeks, therapy physicists to be able to function and treat children at their
reducing abandonment to 12.7%. premises.
Conclusions: Abandonment rate was 16.5%, despite fiscal aid in Conclusions: Structured training and partnerships help creating
approximately 70%. Tracking helped identify patients resuming ther- healthcare change agents who can lead their teams and provide better
apy (27/122[22%]). Ensuring holistic support and establishing a patient healthcare to cancer children.
tracking system will help reduce abandonment & improve survival.
Information regarding curability of disease needs widespread dissem-
ination. Additional cancer centers, shared care & telemedicine are the O252 / #1101 IMPACT OF THE COVID-19 PANDEMIC ON
need of the hour for comprehensive care. PEDIATRIC ONCOLOGY PATIENTS. REPORT OF THE
ARGENTINE ONCOPEDIATRIC REGISTRY (ROHA-NET)

O251 / #350 PEDIATRIC ONCOLOGY HEALTHCARE Florencia Moreno1 , Agustina Chaplin1 , Wanda Goldman2 , Maria
CAPACITY BUILDING IN AFRICA; THE CHILDREN’S CANCER Echaide Zingoni3 , Daniel Solorzano4 , Alejandro Risso Vazquez1 ,
HOSPITAL EGYPT 57357 PERSPECTIVE Marcela Palladino5 , Antonio Latella5 , Agustin Cardoso6 , Maria
Sznitowski7 , Patricia Cañazares8 , Mariana Casullo9 , Gisel Fattore10 ,
Manal Zamzam1 , Reham Khedr1 , Norhan Prince2 , Sherif Kamal2 , Cecilia Garbini11 , Pia Alterats12 , Cristina Ferraro13 , Romina Inzeo14 ,
Ahmed Sherbiny2 , Noha Elbasheer2 , Hanafy Hafez1 , Sherif Antonela Di Staso15 , Monica Hernandez16 , Constanza Cafferata17 ,
Abouelnaga1 Lidia Fraquelli5
1 Children’s Cancer hospital Egypt 57357, Pediatric Oncology, Cairo, Egypt; 1 National Cancer Institude-Argentina, Oncopediatric National Program,
2 Children’s Cancer hospital Egypt 57357, Continuous Education Depart- Buenos Aires, Argentina; 2 Sor Maria Ludovica Hospital, Hematology Service,
ment, Cairo, Egypt La Plata, Argentina; 3 Clinica San Lucas, Pediartric, Neuquen, Argentina;
4 Hospital del Niño Jesus, Oncopediatric, S. M. de Tucuman, Argentina;
Background and Aims: The childhood cancer burden has impacted 5 Hospital of Pediatrics SAMIC. Prof. Dr. J. P. Garrahan, Oncopediatric,
Low-Middle income countries (LMICs). Regional networks aim at CABA, Argentina; 6 FLENI, Neuroncology, CABA, Argentina; 7 Hospital Gen-
building capacity while supporting health services. CCHE57357 devel- eral de niños P. de Elizalde, Oncopediatric, CABA, Argentina; 8 Hospital
oped a national pediatric cancer program by designing integrated Materno Infantil "Dr. Héctor Quintana", Oncology, S. S. de Jujuy, Argentina;
frameworks of care and building human resource capacity through 9 Hospital Provincial Neuquen Dr. Castro Rendon, Oncology, Neuquen,
partnerships to provide quality healthcare. Our aim is to ensure that Argentina; 10 National Cancer Institute, Oncopediatric Program, CABA,
every caregiver involved in pediatric cancer care, from diagnosis to Argentina; 11 Hospital Profesor Alejandro Posadas, Oncology, El Palomar,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S125 of S635

Argentina; 12 Hospital Municipal Materno Infantil de San Isidro, Oncol- Background and Aims: Children with acute myeloid leukemia (AML)
ogy, Buenos Aires, Argentina; 13 Hospital de Niños Dr. Ricardo Gutierrez, are at a particularly high risk for infectious complicatons related to
Hematology Service, CABA, Argentina; 14 Hospital General de niños P. de the highly intensive chemotherapy. The aim of the study is to: assess
Elizalde, Oncology, CABA, Argentina; 15 Hospital Aleman, Hematology Ser- the risk factors, infectious complications and assess outcome of febrile
vice, CABA, Argentina; 16 Hospital Intezonal Dr. J. Penna, Oncology, Bahia episodes in children with AML at the Pediatric Oncology Depart-
Blanca, Argentina; 17 Hospital Eva Perón CEPSI, Oncology, Santiago del ment, National Cancer Institute, Cairo University from January 2016
Estero, Argentina to December 2019.
Methods: Infectious complications were evaluated retrospectively
Background and Aims: In the world, the incidence of COVID-19 cases in 621 febrile episodes in101 Patients :were divided into survivors
in pediatrics patients is lower than in adults and the clinical picture and non-survivors according to outcome at end of each episode.
is less aggressive. At the moment, there is little Latin American lit- Each febrile episode was interpreted in correlation with infectious
erature on the behavior of COVID-19 in children with oncological complications .
pathology. The incidence of childhood cancer in Argentina represents Results: Mortality from gram negative bacteremia was 29.9%, in febrile
132 cases per million (1350 cases per year). It is important to know episodes with multidrug resistant gram negative bacteremia: Mortality
the impact and evolution of COVID-19 in order to generate adequate was 39.2 % .In febrile episodes with multidrug resistant gram negative
health policies.Objectives: To characterize the epidemiology, clinical bacteremia and septic shock. Mortality was 71.8 % (p value <0.001).
course, morbidity and mortality of oncopediatric cases with COVID-19. Mortality was high in early chemotherapy phase (intensive timing).
Methods: The cases registered in the ROHA with a diagnosis of Infection related mortality was 39%. In our institute there is epidemio-
SARS-Cov2 by PCR technique between 12/4/2020 and 05/03/2022 logical shift towards gram negative organisms.In clinically documented
were included. The variables analyzed were: sex, age at diagnosis of febrile episodes: Mortality was 13.2 % (p value <0.001). Mortality
COVID-19, clinic upon admission, severity of symptoms, type of tumor rate was 15.3% for patients who presented with pneumonia, as com-
according to the International Classification of Childhood Cancer - pared to 6 % who hadn’t. (P value = 0.001) .It was 25.7% for patients
Third Edition ICCC-3, requirement for critical care, specific treatment who had typhilitis/colitis, as compared to 9% who hadn’t. (P value
for COVID, status and cause of death. <0.001; statistically significant). However, it was 11.7% for patients
Results: 888 oncopediatric COVID-19 cases were registered, who had soft tissue infection, as compared to 9% who hadn’t. In
(female=484), 437(49.2%) Leukemias, 120(13.5%) Central Ner- clinically documented febrile episodes with multidrug resistant gram-
vous System (T-CNS), 89(10.0%) Lymphomas, 66(7.4%) Bone Tumors, negative bacteremia: Mortality was 42.9% (p value =0.122). Mortality
51(5.7%) Soft Tissue Tumors, 37(4.2%) Neuroblastomas, 25(2.8%) from febrile episodes with ICU admission was 58.5 %. (P value <0.001).
Retinoblastomas, 23(2.6%) Kidney Tumors, 14(1.6%) Liver Tumors, Conclusions: Sepsis and septic shock are major causes of mortality .
14(1.6%) Germ cell tumors and other tumors 12(1.4%). 57.2% were Improved management of sepsis during neutropenia may reduce the
symptomatic (n=508). Among these, 75% were febrile, 37% had mortality of pediatric Acute myeloid leukemia. It is Important to trace
neutropenia (n=210). 17.1% (n=152) were diagnosed within a month the predictors that may impact the outcome of febrile episode.
of oncological diagnosis, 20 patients had critical symptoms of severity,
22 patients received specific treatment. Deaths were reported in 112
(58 leukemia/lymphoma, 14 T-CNS) 105 due to progression, sepsis, O254 / #456 REDUCING EMPIRIC ANTIBIOTIC
comorbidity or therapeutic complications. Seven patients (0.8%) ADMINISTRATION IN PEDIATRIC ONCOLOGY PATIENTS WITH
diagnosed with leukemia/lymphoma died from COVID-19, 6 of them NON-NEUTROPENIC FEVER: A SINGLE CENTER QUALITY
older than 10 years of age. IMPROVEMENT INITIATIVE
Conclusions: From the interpretation of the ROHA data we can con-
clude that in pediatric oncologic patients, contrary to what was initially Alexandra Satty1 , Jessica Stiefel1 , Mauricio Rendon Bernot1 , Zahra
expected, morbidity and mortality was not increased. Hudda1 , Gabriela Llaurador1 , Madhavi Lakkaraja1 , Audrey Mauguen2 ,
Susan Seo3 , Julia Glade-Bender1 , Maria Luisa Sulis1 , Farid Boulad1 ,
James Killinger1
O253 / #77 PREDICTORS AND PATTERN OF INFECTION 1 Memorial Sloan Kettering Cancer Center, Pediatrics, NEW YORK, United
RELATED MORTALITY IN PEDIATRIC ACUTE MYELOID States of America; 2 Memorial Sloan Kettering Cancer Center, Epidemiol-
LEUKEMIA, LOW AND MIDDLE INCOME SETTING ogy & Biostatistics, NEW YORK, United States of America; 3 Memorial Sloan
Kettering Cancer Center, Infectious Diseases, NEW YORK, United States of
Ahmed Bayoumi1,2 , Alaa Elhaddad3 , Reham Khedr2 America
1 National cancer institute Cairo University, Pediatric Oncology, Cairo,
Egypt; 2 Children’s Cancer hospital Egypt 57357/ National Cancer Insti- Background and Aims: The management of pediatric oncology
tute - Cairo University, Pediatric Oncology, Cairo, Egypt; 3 National Cancer patients with an indwelling central venous catheter (CVC) who present
instiute, Pediatric Oncology, GIZA, Egypt with fever without severe neutropenia (ANC >500/mcL) varies widely
within and across institutions due to lack of consensus guidelines. At
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S126 of S635 ABSTRACTS

our institution, a retrospective review of oncology patients with a CVC Hematology/oncology, Palo Alto, United States of America; 15 University of
presenting to the Pediatric Ambulatory Care Center (PACC) with non- York, Paediatric Oncology, Yorkshire, United Kingdom; 16 Leeds Children’s
neutropenic fever (NNF) revealed that 97% received at least one dose Hospital, Leeds General Infirmary, Leeds, United Kingdom; 17 The Hospi-
of parenteral antibiotics despite low rates of bacteremia (2.3%). Given tal for Sick Children, Department Of Pharmacy, Toronto, Canada; 18 The

these results, a prospective quality improvement intervention was ini- Hospital for Sick Children, Haematology/oncology, Toronto, Canada
tiated with the goal of decreasing empiric antibiotic administration in
pediatric oncology patients with a CVC presenting with NNF who are Background and Aims: Epidemiology of Clostridioides difficile infec-
low-risk for bacteremia. tion (CDI) in pediatric patients with cancer and hematopoietic stem cell
Methods: An algorithm for assessing patients at low risk of bac- transplant recipients is uncertain. Additionally, a definition of severe
teremia was developed through consultation with local experts. All CDI applicable to these patients is currently not available. Primary
patients undergoing treatment for malignancy presenting to the PACC objective was to describe the prevalence of CDI outcomes among pedi-
at Memorial Sloan Kettering Cancer Center with a CVC and NNF atric patients receiving cancer treatments. Secondary objectives were
were assessed for eligibility. In patients meeting low-risk criteria, blood to describe clinical features of CDI, propose a definition of severe CDI
cultures were sent, and discharge home was recommended without and to determine risk factors for CDI clinical outcomes.
administering empiric antibiotics. Patients were followed for 72 hours Methods: We conducted a multi-center retrospective cohort study.
from initial visit (considered one episode) and all events within this time Inclusion criteria were pediatric patients (1-18 years of age) receiv-
period were noted. Visits were reviewed weekly and positive blood ing cancer treatments with CDI. Primary outcomes were clinical cure,
cultures monitored daily. recurrence, global cure and repeated new CDI episodes. Severe CDI
Results: Between 4/12/2021 – 3/13/2022, 216 episodes met inclusion definition was achieved by reviewing distribution of potential indica-
criteria, of which 8.8% (n=19) received antibiotics at initial visit. Anal- tors of severe CDI and identifying factors by consensus. Univariable
ysis of outcomes in patients not receiving antibiotics (n=197) revealed and multivariable regression was conducted to evaluate risk factors for
a 2.5% rate of bacteremia within the episode, while an additional 2% CDI outcomes.
were admitted for infectious concerns without a positive blood cul- Results: There were 627 eligible patients who experienced 721 CDI
ture. There were no infection-related ICU admissions or deaths in any episodes. Prevalence of clinical cure was 82.9%, recurrence was 9.6%,
patient in whom antibiotics was withheld during this period. global cure was 75.0% and repeated new CDI episode was 12.8%. The
Conclusions: We have shown that withholding antibiotics in a select most common initial antibiotic treatments were oral metronidazole
population of pediatric oncology patients with a CVC who present with (388, 53.8%), intravenous metronidazole (162, 22.5%) and oral van-
NNF is safe and complements ongoing institutional efforts to improve comycin (151, 20.9%). The proposed definition of severe CDI was the
antimicrobial stewardship. presence of colitis, pneumatosis intestinalis, pseudomembranous coli-
tis, ileus or surgery for CDI, occurring in 70 (9.7%) episodes. In multiple
regression, oral metronidazole was significantly associated with higher
O255 / #165 CLOSTRIDIODES DIFFICILE INFECTION IN odds (odds ratio (OR) 1.7, 95% confidence interval (CI) 1.0-2.7) and oral
PEDIATRIC PATIENTS WITH CANCER AND HEMATOPOIETIC vancomycin was significantly associated with lower odds (OR 0.4, 95%
STEM CELL TRANSPLANT RECIPIENTS CI 0.2-0.8) of repeated new episodes.
Conclusions: The prevalence of clinical cure was 82.9% and recurrence
Robyn Loves1 , Gabrielle Haeusler2,3,4,5,6,7 , Thomas Lehrnbecher8 , was 9.6% in pediatric patients receiving cancer treatments. Severe CDI,
Phillip Agyeman9,10 , Elio Castagnola11 , Andreas Groll12 , Marianne as per our proposed definition, occurred in 9.7% episodes. Initial oral
Van De Wetering13 , Catherine Aftandilian14 , Bob Phillips15,16 , Krishna vancomycin was significantly associated with a reduction in repeated
Mohan Chirra1 , Christine Schneider9 , Lee Dupuis1,17 , Lillian Sung1,18 new CDI episodes.
1 The Hospital for Sick Children, Child Health Evaluative Sciences, Toronto,
Canada; 2 Royal Children’s Hospital, Infectious Diseases, Melbourne, Aus-
tralia; 3 Peter MacCallum Cancer Centre, Infectious Diseases, Melbourne, FREE PAPER SESSION (FPS)
Australia; 4 University of Melbourne, Sir Peter Maccallum Department
Of Oncology, Parkville, Australia; 5 University of Melbourne, Pediatrics, FPS 21: EPIDEMIOLOGY - GENERAL 01-10-2022 10:40 AM -
Parkville, Australia; 6 Murdoch Children’s Research Institute, -, Parkville, 12:10 PM
Australia; 7 Pediatric Integrated Cancer Service, -, Victoria, Australia;
8 Johann Wolfgang Goethe University, Pediatric Hematology And Oncol- O256 / #161 NOVEL AND REPLICATED GENETIC
ogy, Frankfurt, Germany; 9 Inselspital Bern University Hospital, Pedi- PREDICTORS FOR NEPHROTOXICITY AND DELAYED
atrics, Bern, Switzerland; 10 Inselspital Bern University Hospital, Pediatric METHOTREXATE CLEARANCE: NATIVE AMERICAN ANCESTRY
Hematology/oncology, Bern, Switzerland; 11 Istituto Giannina Gaslini, Pedi- AND THE GENES SLCO1B1, SLC19A1, ABCC4
atrics, Genova, Italy; 12 University Children’s Hospital, Pediatric Hematol-
ogy/oncology, Muenster, Germany; 13 Princess Maxima Center for Pediatric Mark Zobeck, Melanie Bernhardt, Karen Rabin, Kala Kamdar, Philip
Oncology, Pediatric Oncology, Utrecht, Netherlands; 14 Stanford University, Lupo, Michael Scheurer
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S127 of S635

Baylor College of Medicine, Pediatric Hematology/oncology, Houston, tion Oncology, Chapel Hill, United States of America; 3 Baylor College of
United States of America Medicine, Human Genome Sequencing Center, Houston, United States of
America; 4 University of Southern California, Population And Public Health
Background and Aims: High-dose methotrexate (HD MTX) is a key Sciences, Los Angeles, United States of America; 5 Children’s Oncology
component of treatment for high-risk pediatric acute lymphoblastic Group, Biostatistics, Monrovia, United States of America; 6 National Cancer
leukemia (ALL) but may cause acute kidney injury and prolonged hos- Institute, Clinical Genetics, Rockville, United States of America; 7 University
pitalization due to delayed clearance in some patients. We conducted a of Utah, Pediatrics, Salt Lake City, United States of America; 8 Baylor Col-
retrospective cohort study to identify genetic factors that may predict lege of Medicine, Texas Children’s Hospital Cancer Center, Houston, United
which children are at risk for creatinine increase and prolonged MTX States of America; 9 National Cancer Institute, Genetics, Bethesda, United
clearance. States of America; 10 The Hospital for Sick Children, Pediatrics, Toronto,
Methods: We identified ALL patients who received HD MTX doses Canada; 11 UT Southwestern, Pediatrics, Dallas, United States of America;
between 4,000-5,000mg/m2 at Texas Children’s Cancer Center from 12 Baylor College of Medicine, Texas Children’s Cancer Center, Department
September 2010 to December 2017. We performed germline geno- Of Pediatrics, Houston, United States of America; 13 Seattle Children’s Hos-
typing to determine genetic ancestry and allele status for 49 sin- pital, University of Washington, Seattle, Division Of Hematology/oncology,
gle nucleotide polymorphisms (SNPs) identified from the literature Seattle, United States of America
as related to MTX disposition. Two Bayesian hierarchical ordi-
nal regression models for creatinine increase and for prolonged Background and Aims: Rhabdomyosarcoma is the most common
MTX clearance were developed to estimate the odds ratios (OR) childhood soft-tissue sarcoma. Although germline cancer predis-
and 95% compatibility intervals (95%CI) for the genetic risk fac- position variants (CPVs) contribute to approximately 7% of rhab-
tors and outcomes. Each SNP model adjusted for the effects of domyosarcoma cases, there are limited data regarding whether
genetic ancestry, dose reduction, and use of an intensive MTX level CPVs influence outcome in these patients. We present an anal-
monitoring protocol that previously was demonstrated to lower ysis of the association of CPVs and survival in children with
toxicity. rhabdomyosarcoma.
Results: Native American ancestry, which correlated with self- Methods: We analyzed existing germline exome and survival data
reported Hispanic ethnicity (Pearson correlation 0.84), was asso- on 580 individuals with newly diagnosed rhabdomyosarcoma who
ciated with increased risk for creatinine increase (OR 2.07[95%CI were consented to Children’s Oncology Group protocols. To eval-
1.18-3.69]). Each additional A allele of rs2838958 in SLC19A1 uate the impact of CPVs in 63 cancer predisposition genes on
demonstrated an OR of 1.73(1.24-2.38) for prolonged MTX clear- overall survival (OS) and event-free survival (EFS), we used the
ance and 1.35(0.99-1.81) for increased creatinine. The G allele of Kaplan-Meier estimator and Cox proportional hazards regression
rs7317112 in ABCC4 was associated with prolonged MTX clear- adjusted for clinical covariates. Lastly, we stratified these analyses by
ance (OR 1.70[1.18-2.43]). The G allele of rs2306283 in SLCO1B1 tumor histology and PAX3/7-FOXO1 fusion status, known prognostic
demonstrated a decreased risk for prolonged clearance (0.72[0.53- factors.
0.97]). Leave-one-out cross validation demonstrated that combined Results: In multivariable models, presence of a CPV was signifi-
models with genetic and clinical predictors improved predictions for cantly associated with worse OS (adjusted hazard ratio [aHR]=1.79,
prolonged MTX clearance compared to clinical or genetic predictors P=0.04). Effects were stronger among those with embryonal his-
alone. tology who harbored a CPV in a subset of 24 rhabdomyosarcoma-
Conclusions: We identified genetic predictors of MTX toxicities that associated predisposition genes (OS: aHR=3.00, P=9.27x10-4 ; EFS:
may allow more precise individualized toxicity risk prediction. More aHR=2.33, P=4.86x10-3 ). Specifically, we observed significant effects
work is needed to refine, calibrate, and validate the prediction models. of CPVs in TP53 and HRAS on OS and EFS. Lastly, in children with
fusion-negative rhabdomyosarcoma, survival was significantly worse
for individuals with CPVs compared to those without CPVs in a
O257 / #1436 GERMLINE CANCER PREDISPOSITION rhabdomyosarcoma-associated predisposition gene (OS: aHR=2.85,
VARIANTS ARE ASSOCIATED WITH SURVIVAL IN CHILDREN P=1.12x10-3 ; EFS: aHR=2.30, P=4.43x10-3 ). Survival for children with
WITH RHABDOMYOSARCOMA fusion-negative rhabdomyosarcoma with a CPV compared to children
with fusion-positive rhabdomyosarcoma was not significantly different
Philip Lupo1 , Bailey Martin-Giacalone1 , Dana Casey2 , He Li3 , Michael (OS: P=0.14; EFS: P=0.37).
Scheurer1 , Shannon Dugan-Perez3 , Deborah Marquez-Do1 , Donna Conclusions: We have demonstrated that germline CPVs are asso-
Muzny3 , Richard Gibbs3 , Donald Barkauskas4 , David Hall5 , Douglas ciated with poorer outcomes among children with rhabdomyosar-
Stewart6 , Joshua Schiffman7 , Matthew T. Mcevoy8 , Javed Khan9 , coma. Notably, children with PAX3/7-FOXO1 fusion-negative rhab-
David Malkin10 , Stephen Skapek11 , Rajkumar Venkatramani12 , domyosarcoma with germline CPVs had outcomes similar to chil-
Douglas Hawkins13 , Aniko Sabo3 , Sharon Plon1 dren with fusion-positive rhabdomyosarcoma. Our findings highlight
1 Baylor College of Medicine, Pediatrics, Houston, United States of America; germline CPV status in guiding risk stratification, surveillance, and
2 University of North Carolina School of Medicine, Department Of Radia- cascade testing.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S128 of S635 ABSTRACTS

O258 / #27 SURVIVAL FROM CHILDHOOD LEUKAEMIA: 1 McMaster University, Faculty Of Health Science, Global Health Office,
TEMPORAL PATTERNS IN EASTERN AND WESTERN GERMANY Hamilton, Canada; 2 The Hospital for Sick Children, Haematology/oncology,
SINCE GERMAN REUNIFICATION Toronto, Canada

Maike Wellbrock1 , Hajo Zeeb2 , Claudia Spix1 , Desiree Grabow1 , Background and Aims: Adolescent and young adult (AYA) patients
Arndt Borkhardt3 , Friederike Erdmann1 with cancer are recognized as a vulnerable subpopulation in high-
1 University Medical Center of the Johannes Gutenberg University, Institute income countries (HICs). Though survival gaps between HIC and
of Medical Biostatistics, Epidemiology and Infomatics (IMBEI), Childhood low- and middle-income country (LMIC) children with cancer are well
Cancer Epidemiology, Mainz, Germany; 2 Leibniz Institute for Prevention described, LMIC AYAs have been neglected. We thus conducted a
Research and Epidemiology – BIPS, Prevention And Evaluation, Bre- systematic review to describe cancer outcomes among LMIC AYA.
men, Germany; 3 Heinrich Heine University, Medical Faculty, Düsseldorf, Methods: We captured English-language studies published from 2010
Paediatric Oncology, Haematology And Clinical Immunology, Düsseldorf, onwards reporting LMIC AYA cancer survival outcomes. LMICs were
Germany defined according to World Bank 2019 classifications, while AYAs were
defined as diagnosed between 15-39 years of age. Cohorts were con-
Background and Aims: The reunification of Germany offers a unique sidered as AYA if >75% of patients were AYA, the mean/median age and
opportunity to evaluate survival trends in childhood leukaemia in SD were between 15-39 years, or the range was within 5 years of the
two merging countries whose initial social conditions, welfare and AYA range (i.e. 10-54 years). Cohort characteristics, including country,
healthcare systems were remarkably different. We sought to eval- cancer type, and cancer outcomes were abstracted.
uate temporal patterns and differences of survival in children with Results: Of 6,207 studies identified by the search strategy, 658
leukaemia in Eastern and Western Germany. underwent full-text review; 60 met inclusion criteria. No low-income
Methods: We conducted a register-based survival study assessing five- countries were represented. 44 (73.3%) studies were conducted in
year overall survival from childhood leukaemia. The study population upper-middle-income countries (UMIC), though these represented
comprised all children with a first primary lymphoblastic (LL) or acute only 12 of 55 countries currently classified as UMICs. The most com-
myeloid leukaemia (AML) diagnosed at ages 0-14 years between 1991 mon cancers studied were acute lymphoblastic leukemia (ALL)(n=13
and 2015 in Germany. Cox proportional hazard models estimating haz- studies), breast cancer (n=5), and osteosarcoma (n=3). 5-year overall
ard ratios (HR) with 95% confidence intervals (CI) and Kaplan-Meier survival was highly variable, ranging from 39%-63% for ALL, 60%-85%
curves were used to compare five-year overall survival from LL and for breast cancer and 47%-83% for osteosarcoma.
AML between children residing in Eastern and Western Germany. Conclusions: Though three billion AYA reside in LMICs, their cancer
We also performed trend analyses to assess temporal changes in the outcomes are neglected in current literature. Existing data indicate
comparison of survival estimates over time. variable survival, ranging from comparable to HIC outcomes to sub-
Results: Overall five-year survival from both LL and AML improved stantially inferior. These studies however represent only a limited
over time in both parts of Germany. HRs of the survival comparison number of LMICs and are biased towards UMICs. Systematic efforts
between Eastern and Western Germany for LL decreased by diagnos- to describe and improve LMIC AYA cancer outcomes are required.
tic period (from HR 1.28, 95% CI 0.98, 1.66, for 1991-1995 to HR
0.59, 95% CI 0.34, 1.05, for 2011-2015; p for trend = 0.002), indicat-
ing initially lower survival probabilities in Eastern Germany improving O260 / #210 IMPLEMENTING A PEDIATRIC
to somewhat superior survival probabilities compared to Western Ger- HEMATOLOGY-ONCOLOGY CLINICAL DATA MONITORING AND
many since around 2006. This trend was most pronounced in children EVALUATION PLATFORM IN SUB-SAHARAN AFRICA
aged 5-14 years at diagnosis. Survival estimates for AML in Western IMPROVED DATA ACCURACY, REPORTING TIMELINESS, AND
Germany still slightly exceed those in Eastern Germany, whereas the ANALYTICAL UTILITY
gap has narrowed in recent years.
Conclusions: Children diagnosed with LL at ages 5-14 years in particu- Mark Zobeck1 , Casey Mcatee2 , Hailey Zhang1 , Elise Ishigami1 , Ronald
lar may have benefited from the improvements in socioeconomic con- Naitala3 , Samuel Makuti2 , Lesego Ketumile4 , Bokani Beo4 , Parth
ditions and healthcare in Eastern Germany. Further research including Mehta1
social conditions on an individual level is warranted to address the 1 Texas Children’s Hospital, Global Hematology/oncology Pediatric Excel-
mechanisms underlying the observed survival inequalities. lence, Houston, United States of America; 2 Baylor College of Medicine
Children’s Foundation, Malawi, Texas Children’s Global Hope, Lilongwe,
Malawi; 3 Joint Clinical Research Centre, Texas Children’s Global Hope, Kam-
O259 / #1459 SURVIVAL OUTCOMES FOR ADOLESCENT pala, Uganda; 4 Baylor Botswana Centre of Excellence, Texas Children’s
AND YOUNG ADULT CANCER IN LOW-AND MIDDLE-INCOME Global Hope, Gaborone, Botswana
COUNTRIES: A SYSTEMATIC REVIEW
Background and Aims: Since 2016, Global Hematology-
Krista Ariello1 , Abdel-Nabi Hadi1 , Sumit Gupta2 , Avram Denburg2 Oncology Pediatric Excellence (HOPE) has provided pediatric
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S129 of S635

hematology-oncology care in Uganda, Malawi, and Botswana. Data method. Time trends in incidence were assessed by calculating average
collection for program monitoring and evaluation (M&E) is challenging annual percentage change (AAPC). A parametric survival model was
due to manual data collection and inconsistent variable definitions. We created to compare the multivariable-adjusted risk of dying from WT
conducted a project to improve the accuracy, timeliness, and utility of between two diagnostic periods.
M&E data. Results: The overall world standardized incidence rate (WSR) was 8
Methods: Global HOPE team members from Houston and Africa per million person-years and was constant over time (AAPC -0.8%,
updated the M&E framework, standardized variable definitions, devel- p=0.29). WSR were 7 per million for WT, 0.1 per million for MRTK, 0.3
oped a REDCap™ database, strengthened quality control workflows, per million for CCSK, and 0.2 per million for RCC. Patients with WT had
and automated reporting of key indicators. The new platform was a good prognosis; 5-year OS modestly increased from 88% in 1990-
implemented in April 2021. We compared baseline data from October 2001 to 91% in 2002-2014. Multivariable analysis showed that risk of
2020 to March 2021 to the period from July to December 2021. Accu- dying from WT was not significantly decreased in the latest period (haz-
racy was measured by types and number of data queries from quality ard ratio, 95% confidence interval: 0.7, 0.4-1.3) after adjustment for
control review. Timeliness was measured by the time to query clo- follow-up time, sex, age, and disease stage. Survival of WT decreased
sure. Utility was measured by the number and frequency of variables with increasing disease stage, ranging from 95-100% for stage I-II and
reported to Global HOPE stakeholders. about 80% for stage III-IV to 74% for bilateral disease. Five-year OS
Results: There were 73%(intervention:114/baseline:427) fewer were 81% for RCC, 77% for CCSK, and 20% for MRTK.
queries for new patients in the intervention period versus baseline, Conclusions: Incidence of pediatric renal malignancies in the Nether-
including 90% fewer missing data queries (29/325), 50% fewer clas- lands has been stable since the 1990s. Five-year OS of WT modestly
sification errors (34/69), 48.5% fewer date logic errors (e.g., death increased reaching 91% and was similar to findings for other developed
before birth; 17/33), and 100% fewer date format errors (0/4). Dupli- countries. In contrast to the excellent outcome for most (non-)WT,
cate ID errors increased by 36%(34/25). Query closure time was long-term outcome of MRTK remained inferior.
unmeasurable during the baseline period but estimated to be 60% at
60 days. Of the 754 eligible queries in the intervention period, 44%
were closed within 30 days and 92% within 60 days. The baseline CCI
reporting dashboard included four variables from spreadsheet-based
calculations updated every 3-6 months. The new M&E dashboard CCI: SUPPORTING FAMILIES. AVOIDING ABANDONMENT
includes interactive, granular visualizations of 16 variables generated 01-10-2022 1:10 PM - 2:10 PM
by R scripts which may be updated on-demand.
Conclusions: By improving the M&E framework, data collection, qual- O262 / #499 “MONEY WAS THE PROBLEM”: CAREGIVERS’
ity workflows, and technology use, we improved the accuracy, timeli- SELF-REPORTED REASONS FOR ABANDONING THEIR
ness, and utility of the Global HOPE M&E data and created a platform CHILDREN’S CANCER TREATMENT IN UGANDA
for growth as a learning global health system.
Barnabas Atwiine1,2
1 Mbarara Regional Referral Hospital, Paediatrics And Child Health,
O261 / #565 PEDIATRIC RENAL TUMORS IN THE Mbarara, Uganda; 2 Mbarara University of Science and Technology, Paedi-
NETHERLANDS BETWEEN 1990 AND 2014: STABLE INCIDENCE atrics And Child Health, Mbarara, Uganda
AND SLIGHTLY IMPROVED SURVIVAL OF WILMS TUMORS
Background and Aims: Treatment abandonment contributes signif-
Maya Schulpen1 , Prakriti Roy1 , Marc Wijnen1 , Lieve Tytgat1 , Marry icantly to poor survival of children with cancer in low-and-middle-
Van Den Heuvel-Eibrink1 , Harm Van Tinteren1 , Henrike Karim-Kos1,2 income countries (LMIC). In order to inform an approach to this
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, problem, we investigated why caregivers withdraw their children from
Utrecht, Netherlands; 2 Netherlands Comprehensive Cancer Organization treatment.Treatment abandonment contributes significantly to poor
(IKNL), Department Of Research And Development, Utrecht, Netherlands survival of children with cancer in low-and-middle-income countries
(LMIC). In order to inform an approach to this problem, we investigated
Background and Aims: This population-based study provides a com- why caregivers withdraw their children from treatment.
prehensive overview of trends in incidence and survival of children and Methods: In a qualitative study, carried out in October and Novem-
adolescents diagnosed with renal malignancies in the Netherlands. ber 2020, in-depth interviews were conducted with caregivers of
Methods: Data on all renal malignancies diagnosed in pediatric children who had abandoned cancer treatment at the Paediatric Can-
patients (0-18 years) between 1990-2014 were extracted from the cer Unit of Mbarara Regional Referral Hospital in South-western
Netherlands Cancer Registry [total N=648; Wilms tumor (WT) N=595; Uganda. Recorded in-depth interviews were transcribed and analyzed
malignant rhabdoid tumor of the kidney (MRTK) N=10; clear cell sar- to identify themes of caregivers’ self-reported reasons for treatment
coma of the kidney (CCSK) N=22; renal cell carcinoma (RCC) N=21]. abandonment. The study was approved by the Review and Ethics
Five-year overall survival (OS) was estimated using the actuarial Committee of Mbarara University of Science and Technology.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S130 of S635 ABSTRACTS

Results: Seventy-seven out of 343 (22.4%) children diagnosed with POSTER DISCUSSION SESSION
cancer abandoned treatment during the study period; 20 contactable
and consenting caregivers participated in the study. The median age of POSTER DISCUSSIONS SESSION 01: IDENTIFYING AND
the caregivers was 37 years and most (65%) were mothers. At the time EVALUATING TARGETED THERAPIES 01-10-2022 1:10 PM - 2:10
of this study, eight (40%) children were alive and 5 (62.5%) were males; PM
with a median age of 6.5 years. Financial difficulty, other obligations,
the child falsely appearing cured, preference for alternative treat- O264 / #1471 CELL FREE DNA MOLECULAR PROFILING
ments, belief that cancer was incurable, fear that the child’s death was IDENTIFIES TARGETABLE ALTERATIONS IN PAEDIATRIC
imminent and chemotherapy side-effects were the caregivers’ reasons CANCER PATIENTS AT RELAPSE
for treatment abandonment.
Conclusions: Seeking cancer treatment for children in Uganda is an Reda Stankunaite1,2 , Sally George3,4 , Debbie Hughes1,2 , Ama Brew1,2 ,
expensive venture and treatment abandonment is mainly caused by Claire Lynn5 , Paula Proszek1,2 , Ridwan Shaikh1 , Sabri Jamal1 , Lina
caregivers’ difficult socio-economic circumstances. This complex prob- Yuan1 , Andrea Sottoriva5 , Michael Hubank1 , Louis Chesler3
lem needs to be approached with empathy and support other than 1 The Royal Marsden NHS Foundation, Clinical Genomics, Sutton, United
blame. Kingdom; 2 Institute of Cancer Research, Molecular Pathology Section, Lon-
don, United Kingdom; 3 Institute of Cancer Research, Division Of Clinical
Studies, Sutton, United Kingdom; 4 Royal Marsden Hospital, Children And
O263 / #1399 FURTHER DATA ABOUT THE TREATMENT Young Peoples Unit, Surrey, United Kingdom; 5 Institute of Cancer Research,
ABANDONMENT PROGRAM IN OUAGADOUGOU Centre For Evolution And Cancer, Sutton, United Kingdom

Rolande Kabore Background and Aims: The implementation of liquid biopsies into
hôpital, Pédiatrie, OUAGADOUGOU, Burkina Faso clinical practice is well underway for adult patients, but its appli-
cation to paediatric cancer patients lags behind. Minimally invasive
Background and Aims: to avoid treatment abandonment (TA) is a molecular profiling could provide a powerful platform to guide clin-
major challenge for pediatric oncologist. We conducted a program ical decision-making and deliver precision treatments, particularly
against TA in Ouagadougou. Support was given to families during at the time of relapse when tumour biopsy may not be possible.
this program either through free investigation in children under five However, paediatric cancers have significantly different mutation pro-
(national health system) or thanks to the funds of the program itself. file than adult patients and paediatric-tailored profiling methods are
Primary results were presented during SIOP Africa 2022. We like to needed.
go in-depth into the details on support given and to analyze further Methods: To fill in this gap we have developed a clinically relevant
secondary objectives of our program. pan-paediatric solid tumour NGS capture panel optimised for cell free
Methods: Eligibility criteria were involvement in one of the 3 diseases DNA (cfDNA). We have validated the method to clinical reporting stan-
(Burkitt lymphoma, nephroblastoma or retinoblastoma), potential cur- dards with a limit of detection of 0.125% variant allele frequency and
ability, and parent’s agreement to participate. Eighty-one children were combined it with low coverage whole genome sequencing of the same
included between November 1st 2019 and November 30th , 2020 (54 library to inform on genome wide copy number changes.
Burkitt, 16 nephroblastoma, and 11 retinoblastoma) among 139 pre- Results: We applied this method to time matched cfDNA (from blood)
screened patients (pts) for eligibility. Non curability was the reason of and tissue biopsy samples from 250 paediatric patients with a range
non-eligibility in 34 pts (of whom parents’ refusal of treatment in 5); of solid tumours at relapse. In 134 patients with single nucleotide
and another cause in 24. variants (SNVs) detected in tissue biopsy, 52% of the expected SNVs
Results: To date, among the 81 pts,10 have dropped out during treat- were detected in cfDNA. In patients with high circulating tumour DNA
ment (4 Burkitt,1 retinoblastoma and 5 nephroblastoma), 28 died (15 (ctDNA) levels (>10% ctDNA purity as determined by lcWGS) 96%
during treatment from toxicity or disease and 13 after completing of SNVs expected from tissue sequencing were detected in cfDNA.
treatment), 51 have completed treatment and 5 are currently on treat- Importantly, 40 cfDNA-unique SNVs have been detected in this cohort,
ment. Financial help given was in average 82€ under 5 and 472€ over highlighting the ability of cfDNA profiling to define tumour hetero-
5 years in Burkitt, 77€ in retinoblastoma and 185€ in nephroblastoma. geneity and identify variants missed by tissue profiling. In some cases,
We will present additional data on faster access to care in the whole cfDNA-unique mutations were of clinical significance and could pro-
population upon arrival in the Hospital. vide guidance for possible targeted treatments or enrolment to clinical
Conclusions: although financial support may reduce treatment trials.
dropouts, subsidy is not a guarantee for family adhesion and survival Conclusions: Our method allows agnostic profiling of cfDNA from
rate remains low. A campaign to reduce diagnostic delays associated to patients with different types of solid paediatric cancers and has the
support for families and access to family house near the hospital could potential to replace or complement tissue biopsy testing in many
result in reducing cancer mortality. clinical diagnostics situations.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S131 of S635

O265 / #1796 ANALYSIS OF CELL-FREE DNA IN THE in metastatic disease (e.g., CDK4, CDK6, MDM2) that were absent in
PLASMA OF PATIENTS WITH NON-RESECTABLE, PROGRESSIVE tumor biopsies.
OR RELAPSED PEDIATRIC MALIGNANCIES Conclusions: Although limited sample volumes may impede analyses,
we have demonstrated the applicability of liquid biopsies in person-
Kendra K. Maass1,2 , Pitithat Purachanot3,4 , Paulina Schad1 , Agnes alized pediatric oncology. Further development of a clinical decision
Finster1 , Barbara Jones2,5 , Tatjana Wedig1,2 , Nathalie Schwarz1 , Petra support system for liquid biopsies may aid in optimally realizing in-
Fiesel1,6 , Gnanaprakash Balasubramanian1,3 , Jochen Meyer6,7 , Felix depth spatial and temporal tumor resolution. Our study is expected
Sahm8,9 , Stefanie Zimmermann10 , David Jones5 , Benedikt Brors3 , to guide and accelerate the implementation of liquid biopsies in
Stefan Pfister1,2 , Kristian Pajtler1,2 prospective pediatric clinical trials.
1 Hopp Children’s Cancer Center Heidelberg, Pediatric Neurooncology, Hei-
delberg, Germany; 2 Heidelberg University Hospital, Pediatric Hematology
And Oncology, Heidelberg, Germany; 3 German Cancer Research Cen- O266 / #234 SECOND GENERATION SMALL MOLECULE
ter (DKFZ), Applied Bioinformatics, Heidelberg, Germany; 4 HRH Princess INHIBITORS OF GANKRYIN FOR THE TREATMENT OF
Chulabhorn College of Medical Science, Faculty Of Medicine And Public PEDIATRIC LIVER CANCER
Health, Bangkok, Germany; 5 Hopp Children’s Cancer Center Heidelberg
(KiTZ), Heidelberg, Germany, Division Of Pediatric Glioma Research, Hei- Maria Thomas1 , Manu Gnanamony1 , Dipti Kanabar2 , Aaron Muth2 ,
delberg, Germany; 6 German Consortium for Translational Cancer Research Pedro Dealarcon1 , Nikolai Timchenko3 , Amber D’Souza1
(DKTK), German Cancer Research Center (DKFZ), Clinical Cooperation Unit 1 University of Illinois College of Medicine Peoria, Pediatrics, Peoria, United
Neuropathology, Heidelberg, Germany; 7 Institute of Pathology, Heidelberg States of America; 2 St John’s University, Pharmaceutical Sciences, d, United
University Hospital, Heidelberg, Germany, Department Of Neuropathol- States of America; 3 Cincinnati Children’s Hospital Medical Center, Surgery,
ogy, Heidelberg, Germany; 8 Heidelberg University Hospital, Heidelberg, d, United States of America
Germany, Neuropathology, Institute Of Pathology, Heidelberg, Germany;
9 German Cancer Research Center (DKFZ) and German Cancer Consortium Background and Aims: Gankyrin, a member of the 26S protea-
(DKTK), Heidelberg, Germany, Ccu Pediatric Oncology, Heidelberg, Ger- some, is an overexpressed oncoprotein in hepatoblastoma (HBL) and
many; 10 University Hospital Frankfurt, Frankfurt, Germany, Department Of hepatocellular carcinoma (HCC) that binds to and degrades tumor sup-
Pediatric Hematology And Oncology, Frankfurt, Germany pressor proteins (TSPs). Cjoc42 was the first small molecule inhibitor
of Gankyrin developed, however IC50 values > 50 μM made them
Background and Aims: Circulating cell-free DNA released by tumor unattractive for clinical use. Second generation inhibitors, also called
cells (ctDNA) provides the opportunity to minimally invasively sur- cjoc42 derivatives, demonstrate a stronger affinity to Gankyrin and
vey clinically informative biomarkers and therapeutic targets. The increased cytotoxicity. The aim of this study was to characterize the
INFORM registry aims to identify actionable targets on a person- in vitro effects of three cjoc42 derivatives as single agents and in
alized basis. Relapsed or refractory high-risk tumors, for which no combination with chemotherapy.
further standard of care therapy is available, are characterized by Methods: Experiments were performed in HepG2 (HBL) and Hep3B
next-generation sequencing (NGS) technologies. Our study aims at (pediatric HCC) cell lines, which were treated with 15-256 μM of
integrating liquid biopsies into the diagnostic INFORM pipeline and each compound. We evaluated expression of TSPs, Gankyrin, cell cycle
paving the way for clinical translation of these technologies. markers, and stem cell markers by western blotting and/or real-time
Methods: CtDNA was derived from plasma samples and isolated quantitative reverse transcription PCR. We also performed apoptotic
according to optimized protocols. Depending on the yield of ctDNA, assays through flow cytometry and fluorescent microscopy, synergy
data availability for the primary tumor, and tumor entity, low-coverage assays with doxorubicin and cisplatin, and methylation assays.
whole-genome sequencing (lcWGS), whole-exome sequencing (WES), Results: Treatment with cjoc42 derivatives led to an increase in TSPs
targeted gene panel sequencing or combinations thereof were per- and dose-dependent decrease in stem cell phenotype in both cell lines.
formed. Results were compared and integrated with data obtained An increase in apoptosis was only seen with AFM-1-2 in Hep3B cells.
from standard tumor biopsies and normal control tissues follow- Drug synergy was seen with doxorubicin and antagonism was seen with
ing adjustment of established bioinformatics pipelines to suit liquid cisplatin. In the presence of cjoc42 derivatives, the 20S subunit of the
biopsy-derived data. 26S proteasome was more available to transport doxorubicin to the
Results: The sensitivity and specificity of our approach are presented nucleus, leading to synergy. A decrease in global methylation was seen
within a proof-of-concept study to compare the performance of dif- after combination treatment with cisplatin in HepG2 cells, suggesting
ferent NGS platforms and their utility for analyzing ctDNA samples. that abberant methylation may promote drug antagonism.
We demonstrate robust tumor detection by lcWGS (n=115), muta- Conclusions: Novel therapy is needed for children with chemoresis-
tion tracking by targeted approaches (n=74), and the advantages of tant liver cancer. Small molecule inhibitors for Gankyrin are a promising
the combinatorial use of various approaches (n=64). Despite reliable therapeutic strategy, especially in combination with doxorubicin. As
tumor monitoring, liquid biopsies aided in identifying druggable targets newer Gankyrin inhibitors with increased cytotoxicity are developed,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S132 of S635 ABSTRACTS

in vivo work will be imperative to support the use of these compounds and UK-IMPORT) and AIEOP databases between 1993 and 2021. The
in patients. small size and heterogeneity of this retrospective cohort allowed us
only descriptive statistical analysis.
Results: Thirty-two patients were identified, with 8/32 presenting with
O267 / #1237 TARGETED THERAPIES IN CHILDREN WITH (TNM) stage III disease, and 21/32 with stage IV disease. The majority
RENAL CELL CARCINOMA (RCC): A SIOP-RENAL TUMOR of the patients was diagnosed with translocation type RCC (MiT-
STUDY GROUP RELATED RETROSPECTIVE DESCRIPTIVE STUDY RCC) (21/32), whereas the remaining patients presented with other
histological subtypes (including papillary type RCC in 5 cases and clear-
Julia Sprokkerieft1 , Justine Van Der Beek1 , Filippo Spreafico2 , cell type RCC in 2 cases). Treatment included mono- or combination
Barbara Selle3 , Estelle Thebaud4 , Tanzina Chowdhury5 , Jesper Brok6 , therapy with a large variety of drugs, reflecting individual treatment
Gábor Ottóffy7 , Xiaofei Sun8 , Gema Ramirez9 , Garik Sagoyan10 , Heidi approaches in most patients. Sunitinib alone was often administered as
Segers11 , Dimitrios Doganis12 , Norbert Graf13 , Arnaud Verschuur14 , first choice, predominantly resulting in stable disease (47%), whereas
Lieve Tytgat1 , Marry Van Den Heuvel-Eibrink1 other frequently used drugs were axitinib, cabozantinib, sorafenib and
1 Princess Máxima Center for Pediatric Oncology, Department Of Pediatric nivolumab.
Oncology, Utrecht, Netherlands; 2 Fondazione IRCCS Istituto Nazionale dei Conclusions: This study provides an overview of a unique series of
Tumori, Pediatric Oncology Unit, Department Of Medical Oncology And clinical and treatment characteristics, including disease response, of
Hematology, Milano, Italy; 3 German Cancer Research Center (DKFZ) and advanced-stage and relapsed pediatric RCC patients treated with
German Cancer Consortium (DKTK)„ Hopp Children’s Cancer Center Hei- targeted therapies.
delberg (kitz) & Division Of Pediatric Neurooncology, Heidelberg, Germany;
4 CHU de Nantes, Hôtel Dieu, Nantes, France; 5 Great Ormond Street Hospi-

tal for Children NHS Foundation Trust, Pediatric Oncology, London, United POSTER DISCUSSION SESSION
Kingdom; 6 Rigshospitalet, Copenhagen University Hospital, Department Of
Pediatric Hematology And Oncology, Copenhagen, Denmark; 7 University POSTER DISCUSSIONS SESSION 02: PATIENT-REPORTED
of Pécs, Department Of Pediatrics, Pécs, Hungary; 8 Sun yat-sen Univer- OUTCOMES 01-10-2022 1:10 PM - 2:10 PM
sity Cancer Center, Department Of Pediatric Oncology, Guangzhou, China;
9 Pediatric Hospital Virgen del Rocío, Department Of Pediatric Oncology, O268 / #304 LESS THAN HALF OF TRIALS USE
Sevilla, Spain; 10 N.N. Blokhin National Medical Research Center of Oncol- PATIENT-REPORTED OUTCOME MEASURES TO EVALUATE
ogy, Research Institute Of Pediatric Oncology And Hematology, Moscow, SUPPORTIVE INTERVENTIONS IN CHILDREN WITH CANCER -
Russian Federation; 11 University Hospitals Leuven and Catholic University A SYSTEMATIC REVIEW OF CLINICAL TRIAL REGISTRIES
Leuven, Department Of Pediatric Hemato-oncology, Leuven, Belgium; 12 ‘P
& A. Kyriakou’ Children’s Hospital, Oncology Department, Athens, Greece; Maria Rothmund1,2 , Jens Lehmann2 , Wiebke Moser3 , Teresa De
13 Saarland University Medical Center and Saarland University Faculty Rojas4 , Samantha Sodergren5 , Anne-Sophie Darlington5 , David Riedl2
of Medicine, Department For Pediatric Oncology And Hematology, Hom- 1 University of Innsbruck, Institute Of Psychology, Innsbruck, Austria;
burg, Germany; 14 Hôpital d’Enfants de la Timone, Department Of Pediatric 2 Medical University Innsbruck, Department For Psychiatry, Psychotherapy,
Oncology, Marseille, France Psychosomatics, And Medical Psychology, Innsbruck, Austria; 3 University
Clinic Innsbruck, Department For Psychiatry, Psychotherapy, Psychosomat-
Background and Aims: Renal cell carcinoma (RCC) is a very rare ics, And Medical Psychology, Innsbruck, Austria; 4 Accelerate, Innovation For
renal tumor type in children, and evidence-based treatment guidelines Children And Adolescents With Cancer, Brussels, Belgium; 5 University of
are lacking. Whereas for localized pediatric RCC radical nephrectomy Southampton, School Of Health Sciences, Southampton, United Kingdom
alone is the standard of care, advanced-stage, as well as relapsed
disease, require more extensive treatment. Currently, within the SIOP- Background and Aims: Children with cancer suffer from numerous
Renal Tumor Study Group (RTSG)-2016 UMBRELLA protocol, sunitinib symptoms and side-effects, making supportive interventions indis-
is recommended in case of metastatic or unresectable disease. Knowl- pensable to improve their health-related quality of life (HRQOL). The
edge on the effect of a broader spectrum of targeted therapies or gold standard for evaluating HRQOL are patient-reported outcome
immunotherapy is mainly based on adult RCC studies. This retrospec- measures (PROMs). This systematic review investigated the current
tive descriptive study focuses on the experiences of treatment with practice of clinical outcome assessment (COA) in clinical trials on
tyrosine kinase inhibitors, anti-programmed cell death 1 monoclonal supportive interventions.
antibodies or other immunotherapeutic regimens in advanced-stage Methods: ClinicalTrials.gov and EudraCT were searched for clin-
and relapsed pediatric RCC patients. ical trials investigating supportive interventions in children and
Methods: This study included pediatric patients (0-18 years) with adolescents (≤21 years) with cancer, which have been registered
histologically diagnosed RCC, treated with targeted therapy, identi- between 2007 and 2020. The use of different types of COAs (i.e.,
fied through a call to SIOP-RTSG national coordinators. All included PROMs, performance-based, clinician-, or observer-reported out-
patients were registered in SIOP- (93-01, 2001, 2016 UMBRELLA comes) was analysed, focusing on PROMs. Associations with trial
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S133 of S635

characteristics were investigated using univariate and multivariable of patients ever indicated high distress (DT score ≥8), while 26.6%
analyses. (608/2,290) and 65.6% (1,503/2,290) reported moderate (DT score
Results: Of 4789 identified clinical trials, 229 were included. Among 5-7) and low (DT score 0-4) levels of distress, respectively. Patients
them, 44.1% relied on PROMs, which were the most commonly used with high distress scores were more likely to be female (Odds Ratio
COA. The proportion of trials using PROMs did not significantly dif- [OR]=1.50, 95% Confidence Interval [CI]: 1.11-2.04), newly diagnosed
fer over time. In the multivariable analysis, intervention type (higher (OR=2.07, 95% CI: 1.33-3.21), and older at time of diagnosis (Refer-
PROM use in behavioural vs. medical interventional trials) and cancer ence: 0-5 years; 10-14 yrs: OR=2.97, 95% CI: 1.99-4.45; 15-21 yrs:
type (higher PROM use in mixed and solid tumour samples vs. haemato- OR=3.42, 95% CI: 2.21-5.29). Regarding implementation fidelity, med-
logical samples) were significant predictors of PROM use. The majority ical staff followed screening protocols referring 93.9% (222/231) of
of trials using PROMs (59.6%) measured more than one health domain. high distress encounters to psychology. Of the 78.8% (175/222) of fam-
Physical health was the most frequently assessed domain (92.6%). ilies who did not decline psychology services, psychology consults were
Conclusions: Less than half of registered trials investigating support- provided to 76.0% (133/175) of those in high distress at point-of-care.
ive interventions for children with cancer used PROMs. While this Conclusions: Leveraging EMR technology to provide evidence-based
proportion is higher than in clinical trials evaluating anti-cancer treat- psychosocial screening can successfully reach pediatric oncology
ments for paediatrics, it is inferior to that in adult oncology research. patients, effectively link patients to timely mental health supports, and
Overall, the result is striking since supportive care explicitly aims be easily scaled to other pediatric cancer centers.
to improve patients’ HRQOL, which is best assessed using PROMs.
PROM assessment in clinical trials evaluating supportive interventions
should therefore be the norm rather than the exception. Our system- O270 / #662 DIGITAL HEALTH INTERVENTIONS FOR PAIN
atic review underlines the need to identify and overcome barriers for IN PEDIATRIC ONCOLOGY: STATE OF THE FIELD
PROM implementation and to improve PROM research in paediatric
oncology. Julia Simon1 , Isabel Hooijman1 , Marloes Van Gorp1 , Sasja Schepers1 ,
Wim Tissing2 , Erna Michiels3 , Martha A. Grootenhuis1
1 Princess Máxima Center for Peditric Oncology, Psycho-oncology, Utrecht,
O269 / #909 IMPLEMENTATION OF ELECTRONIC Netherlands; 2 Princess Maxima Center for Pediatric Oncology, Supportive
PSYCHOSOCIAL SCREENING AMONG CHILDREN, Care, Utrecht, Netherlands; 3 Princess Máxima Center for Peditric Oncology,
ADOLESCENTS, AND YOUNG ADULTS WITH CANCER Supportive Care, Utrecht, Netherlands

Jordan Gilleland Marchak1 , Rebecca Williamson Lewis2 , Tonya Background and Aims: Over the past years, a wide range of digi-
Bennett2 , Heather Miller2 , Ann Mertens1 , Karen Wasilewski-Masker1 tal health interventions for pain in children with cancer have been
1 Emory University School of Medicine, Aflac Cancer & Blood Disorders Cen- developed. We aimed to provide an overview of existing interven-
ter At Children’s Healthcare Of Atlanta, Atlanta, United States of America; tions and assess the state of the field. Second, we evaluated aspects
2 Aflac Cancer & Blood Disorders Center, Children’s Healthcare Of Atlanta, slowing down (barriers) or facilitating (facilitators) eventual/potential
Atlanta, United States of America implementation.
Methods: A comprehensive literature search (PubMed, Cochrane,
Background and Aims: Psychosocial screening for oncology patients Embase, and PsycINFO) was carried out to identify peer-reviewed arti-
is considered standard of care, yet most pediatric institutions have not cles which included the use of digital health interventions for pain
implemented evidence-based screening programs. Guided by the RE- in children (0-18), or their parents, with cancer (all diagnoses) dur-
AIM framework, this study evaluated the reach, implementation, and ing active treatment. Corresponding authors of the interventions were
effectiveness of an electronic psychosocial screening program among invited for a semi-structured interview on barriers and facilitators.
outpatient pediatric oncology patients. Results: Of 109 potential records examined, 21 met inclusion cri-
Methods: Participants (N=2,367) included patients aged 2.0-21.9 teria, describing 13 separate digital health interventions monitoring
years (M=10.9, SD=5.4) presenting across 10,062 outpatient oncol- pain in pediatric oncology. Several platforms were used for delivery:
ogy visits from September 2018 to June 2019. At clinic visit check-ins, apps (n=11), a web-portal (n=1) and a wristband (n=1). Functionalities
patients (≥13 years) or caregiver proxies (patients <13 years) electron- consisted of: pain severity monitoring (n=12), providing informa-
ically completed the Distress Thermometer (DT), providing numerical tion about pain(treatment) (n=5) and communication options with
ratings of distress (0-10) via a patient-facing electronic medical record healthcare professionals or peers (n=8). Most publications focused
(EMR) application (i.e., Epic Welcome). EMR data were abstracted to on feasibility/usability/acceptability testing (n=9). Currently there are
characterize the reach and implementation of the e-screening pro- no publications on effectiveness, yet several (n=4) Randomized Con-
gram. Factors associated with high distress scores were evaluated trolled Trials are being carried out. 12/13 project leaders agreed to
using univariate logistic regression. be interviewed. Their working experience within the digital health
Results: The e-screening program reached nearly all patients seen dur- field averaged 12 years. Most barriers related to the organization
ing the study period (96.7%; 2,290/2,376). Overall, 7.8% (179/2,290) (i.e. financial/time resources) and socio-political context (i.e. juridical
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S134 of S635 ABSTRACTS

regulations). Most facilitators related to the intervention itself (i.e. user interference was associated with increased risk of any pharmaco-
friendliness) and end-users (i.e. buy-in from patients/parents/nurses). logic intervention (RR [95% CI]; 1.75 [1.45-2.10] and 2.55 [2.11-3.07],
3/12 project leaders reported using a theoretical model for imple- respectively) and self-medication (1.10 [1.01-1.20] and 1.13 [1.03-
mentation (i.e. Consolidated Framework for Implementation Research 1.25], respectively), but not nonpharmacologic interventions or IM use.
[CFIR], Knowledge-to-Action [KTA] Framework, and Reach, Effective- Three patterns of intervention use were identified: 1) elevated non-
ness, Adoption, Implementation, and Maintenance [RE-AIM]). pharmacologic + IM (43%), 2) elevated self-medication (17%), and 3)
Conclusions: Thirteen interventions were identified and these were elevated nonpharmacologic + IM + self-medication (40%). All classes
mostly apps directed at pain severity monitoring. Yet, little is known had similar rates of pharmacologic intervention use. Pain with interfer-
so far about their effectiveness. Paying attention to common barriers ence increased risk of self-medication and nonpharmacologic + IM +
and facilitators at the early stages of new projects might improve future self-medication compared to nonpharmacologic + IM use.
implementation. Conclusions: Adult survivors experiencing pain are more likely to
use pharmacologic interventions and engage in behaviors reflect-
ing self-medication, suggesting inadequate pain management. Routine
O271 / #1069 PAIN, INTERVENTION USE, AND assessment of pain interventions used by survivors, including both
SELF-MEDICATION IN ADULT SURVIVORS OF CHILDHOOD adaptive and maladaptive strategies, is needed.
CANCER: A REPORT FROM THE ST. JUDE LIFETIME COHORT
STUDY (SJLIFE)
O272 / #439 THE IMPACT OF CANCER-RELATED FATIGUE
Nicole Alberts1 , Mengqi Xing2 , Jennifer Allen3 , Doralina Anghelescu4 , ON HRQOL IN LONG-TERM SURVIVORS OF CHILDHOOD
I-Chan Huang5 , Matthew Ehrhardt6 , Holly Spraker-Perlman6 , William CANCER; A DCCSS LATER STUDY
Greene7 , Sedigheh Mirzaei Salehabadi2 , Leslie Robison5 , Melissa
Hudson6 , Tara Brinkman5 Adriaan Penson1 , Iris Walraven2 , Ewald Bronkhorst3 , Heleen
1 Concordia University, Psychology, Montreal, Canada; 2 St. Jude Children’s Maurice-Stam4 , Martha A. Grootenhuis5 , Wim Tissing6,7 , Helena Van
Research Hospital, Biostatistics, Memphis, United States of America; 3 St. Der Pal8 , Andrica De Vries9 , Marry Van Den Heuvel-Eibrink8,9 ,
Jude Children’s Research Hospital, Psychology, Memphis, United States of Sebastian Neggers8,10 , Birgitta Versluys8 , Marloes Louwerens11 ,
America; 4 St. Jude Children’s Research Hospital, Pediatric Medicine, Mem- Saskia Pluijm8 , Nicole Blijlevens1 , Margriet Van Der Heiden-Van Der
phis, United States of America; 5 St. Jude Children’s Research Hospital, Loo8 , Leontien Kremer8,12,13 , Eline Van Dulmen-Den Broeder14 , Hans
Epidemiology And Cancer Control, Memphis, United States of America; 6 St. Knoop15 , Jacqueline Loonen1
Jude Children’s Research Hospital, Oncology, Memphis, United States of 1 Radboud University Medical Center, Department Of Hematology,
America; 7 St. Jude Children’s Research Hospital, Pharmacy And Pharmaceu- Nijmegen, Netherlands; 2 Radboud University Medical Center, Health
tical Sciences Department, Memphis, United States of America Evidence, Nijmegen, Netherlands; 3 Radboud University Medical Center,
Health Sciences, Nijmegen, Netherlands; 4 Princess Máxima Center for
Background and Aims: Pain is common among adult survivors of child- Pediatric Oncology, Psychology, Utrecht, Netherlands; 5 Prinses Maxima
hood cancer. Multimodal intervention is the gold-standard treatment Center for Pediatric Oncology, Psycho-oncology, Utrecht, Netherlands;
for pain. However, little is known about survivors’ use of pharmacologic 6 University of Groningen, University Medical Center Groningen, Beatrix
and nonpharmacologic interventions, including integrative medicine Children’s Hospital, Department Of Pediatric Oncology And Hematology,
(IM), and self-medication. Groningen, Netherlands; 7 Princess Maxima Center for Pediatric Oncology,
Methods: Adult survivors in the SJLIFE cohort were evaluated for Supportive Care, Utrecht, Netherlands; 8 Princess Máxima Center for
self-reported pharmacologic and nonpharmacologic intervention use. Pediatric Oncology, Pediatric Oncology, Utrecht, Netherlands; 9 Sophia

Modified Poisson regression estimated relative risks (RR) and 95% Children’s Hospital/Erasmus Medical Center, Department Of Paediatric
confidence intervals (CI) for associations between pain and pharmaco- Oncology, Rotterdam, Netherlands; 10 Erasmus Medical Center, Department
logic and nonpharmacologic intervention use, IM, and self-medication. Of Internal Medicine, Section Endocrinology, Rotterdam, Netherlands;
Latent class analysis was conducted to identify patterns of intervention 11 Leiden University Medical Center, Department Of Internal Medicine,
use. Leiden, Netherlands; 12 University Medical Center Utrecht, Wilhelmina
Results: 3,390 survivors (50.4% male; mean [SD] age 30.7[8.4] years; Children’s Hospital, Utrecht, Netherlands; 13 Emma Children’s Hospital,
22 [8.4] years since diagnosis) self-reported pharmacologic (e.g., opi- Amsterdam UMC, University of Amsterdam, Pediatrics, Amsterdam,
oids) and nonpharmacologic intervention use (e.g., physical therapy), Netherlands; 14 Emma Children’s Hospital/Amsterdam University Medical
IM (e.g., acupuncture), self-medication (e.g., illicit drug use), pain Center, Vrije Universiteit Amsterdam, Department Of Paediatric Oncology,
intensity and pain interference, and psychosocial symptoms (anxiety, Amsterdam, Netherlands; 15 Amsterdam University Medical Centers,
depression, sleep, fatigue). Overall, 20% reported moderate to very Medical Psychology, Amsterdam, Netherlands
severe pain and 16% reported moderate to very severe pain with
moderate to extreme daily interference. In models adjusted for age, Background and Aims: Late effects of treatment and their impact
sex, race/ethnicity and psychosocial symptoms, pain with and without on health-related quality of life (HRQOL) has become a key goal of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S135 of S635

childhood cancer survivorship care. One of the most prevalent late traumatic experience) and fatigue levels were examined by performing
effects is cancer-related fatigue (CRF). Since the impact of CRF on two cross-lagged panel analyses. Resilience was included as potential
HRQOL is poorly understood, the current study aimed to investigate moderator. These models included all within-time associations, stabil-
the association between CRF and HRQOL in a nationwide cohort ity paths, and cross-lagged paths. Gender and time since diagnosis were
childhood cancer survivors (CCS). included as covariates.
Methods: Participants were included from the Dutch Childhood Can- Results: In total, 110 patients participated in this study, aged 16-25
cer Survivor Study (DCCSS) LATER cohort, a nationwide cohort of five- years old (average time since diagnosis 12.2 years; 41.8% boys; diag-
year CCS. Participants completed the Checklist Individual Strength nosed with leukemia/lymphoma (49%), solid tumor (15%), brain tumor
(CIS) to indicate CRF (CIS-fatigue ≥35 and duration of symptoms (16%) or other (20%)). Both fear of recurrence (β=0.167, p=0.042) and
≥6 months) and the Short Form-36 (SF-36) and TNO and AZL QOL traumatic experience (β =0.177, p=0.035) at baseline significantly pre-
questionnaire (TAAQOL) as measures for HRQOL. Independent t-tests dicted higher fatigue one year later. The significant interaction effect
were calculated to identify differences in mean HRQOL domain scores suggested that resilience could buffer the effect of fear of recurrence
between CRF and non-CRF participants. The association between on fatigue (β = 0.142, p=0.031). Stability coefficients were high for all
CRF and impaired HRQOL (scoring 2 SD below population norm) was study variables. Girls experienced more fatigue (p<0.001) and fear of
tested using multivariable logistic regression to adjust for potential recurrence (p=0.004) than boys at baseline, while boys showed more
confounders. resilience (p<0.001).
Results: A total of 1,695 CCS participated in the study (23.1% with Conclusions: Interventions that can reduce fatigue will only become
CRF). Mean total scores of all HRQOL domains were significantly lower efficient if we can intervene adequately and instantly at the moment, in
for the CRF group compared to the non-CRF group, with mean dif- the context, according to the needs of the patient. This study suggests
ferences ranging from 14.0 (95%CI 11.4 – 16.7) on sexuality to 41.6 that cancer-related distress can exacerbate fatigue. This information
(95%CI 38.2 – 45.0) on physical work limitations. CRF was associated should be incorporated in therapeutic interventions.
with an impaired HRQOL on all studied domains with adjusted odds
ratio’s ranging from 1.6 (95%CI 1.1-2.4) for the sexuality domain to
30.4 (95%CI 16.4 – 56.2) for the vitality domain. POSTER DISCUSSION SESSION
Conclusions: Understanding the impact of late effects on HRQOL
is crucial when aiming to improve the quality of CCS daily lives. POSTER DISCUSSIONS SESSION 03: PROGNOSTIC
The current study shows CRF to have a negative impact on multiple OBSERVATIONS FROM CLINICAL TRIALS AND NATIONAL
HRQOL domains, indicating the urge of structural screening and mon- COHORTS 01-10-2022 1:10 PM - 2:10 PM
itoring of fatigue symptoms, and developing possible prevention and
intervention techniques. O274 / #154 EFFECTS OF AGE, OBESITY, AND BODY
SURFACE AREA ON ASPARAGINASE-ASSOCIATED TOXICITIES
DURING ACUTE LYMPHOBLASTIC LEUKEMIA INDUCTION
O273 / #431 ASSOCIATIONS BETWEEN CANCER-RELATED THERAPY: A REPORT FROM THE CHILDREN’S ONCOLOGY
DISTRESS AND FATIGUE IN CHILDHOOD CANCER SURVIVORS GROUP

Deveny Vanrusselt1 , Charlotte Sleurs1 , Sofie Prikken2 , Koen Etan Orgel1 , Olga Militano2 , Zhiguo Chen3 , Meenakshi Devidas4 , Luke
Raeymaekers1 , Sabine Verschueren3 , Anne Uyttebroeck1 , Jurgen Maese5 , Rachel Rau6 , Anne Angiolillo7 , Jennifer Mcneer8 , Reuven
Lemiere4 , Koen Luyckx1 Schore7 , Elizabeth Raetz9 , Lewis Silverman10 , Naomi Winick11 , Eric
1 KULeuven, Department Of Oncology, Leuven, Belgium; 2 KU Leuven, Larsen12 , William Carroll9 , Stuart Winter13 , Kimberley Dunsmore14 ,
Pediatric Hemato-oncology, Leuven, Belgium; 3 KULeuven, Department Of Stephen Hunger15 , Mignon Loh16
Rehabilitation Sciences, Leuven, Belgium; 4 UZ Leuven, Pediatric Hemato- 1 Children’s Hospital Los Angeles, Cancer And Blood Disease Institute, Los
oncology, Leuven, Belgium Angeles, United States of America; 2 Children’s Oncology Group, Pharmacy,
Monrovia, United States of America; 3 Children’s Oncology Group, Biostatis-
Background and Aims: A chronic feeling of fatigue occurs in up to tics, Gainesville, United States of America; 4 St Jude Children’s Research
85% of children and adolescents after the completion of their cancer Hospital, Global Pediatric Medicine, Memphis, United States of America;
treatment. Chronic fatigue has a detrimental effect on quality of life, 5 Primary Children’s Hospital, Hematology/oncology, Salt Lake City, United
reintegration in daily life activities and psychosocial functioning of the States of America; 6 Baylor College of Medicine/Texas Children’s Hospi-
patient. Therefore, it is important to define individual risk profiles for tal, Children’s Cancer And Hematology Center, Houston, United States
chronic fatigue. of America; 7 Children’s National Medical Center, Center For Cancer And
Methods: Childhood cancer survivors who were treated in the Uni- Blood Disorders, Washington, DC, United States of America; 8 University
versity Hospital of Leuven, completed two annual questionnaires on of Chicago, Pediatrics, Chicago, United States of America; 9 NYU Langone
fear of recurrence, traumatic experience, resilience and fatigue. The Health, Pediatrics, New York, United States of America; 10 Dana-Farber

associations between cancer-related distress (fear of recurrence and Cancer Institute, Pediatrics, Boston, United States of America; 11 UT South-
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S136 of S635 ABSTRACTS

western/Simmons Cancer Center, Pediatric Hematology/oncology, Dallas, Amanda Treece11 , Lauren Parsons12 , Meryle Eklund13 , Kenneth
United States of America; 12 Maine Children’s Cancer Program, Pediatric Gow14 , Jennifer Aldrink15 , Jeffrey Dome16 , Conrad Fernandez17 ,
Oncology, Scarborough, United States of America; 13 Children’s Hospitals Elizabeth Mullen18
and Clinics of Minnesota, Pediatric Hematology & Oncology, Minneapolis, 1 Cook Childrens Medical Center, Hematology/oncology, Fort Worth,
United States of America; 14 Virginia Tech University, Pediatrics, Roanoke, United States of America; 2 Children’s Oncology Group, Statistics And
United States of America; 15 Children’s Hospital of Philadelphia, Pedi- Data Center, Monrovia, United States of America; 3 Children’s Hospital of
atrics, Philadelphia, United States of America; 16 Seattle Children’s Hospital, Philedelphia, Oncology, Voorhees, United States of America; 4 Vanderbilt
Pediatrics, Seattle, United States of America University/Ingram Cancer Center, Pediatric Oncology, Nashville, United
States of America; 5 Lurie Children’s Hospital, Pathology, Chicago, United
Background and Aims: Asparaginase-associated toxicities (AAT) often States of America; 6 University of Colorado School of Medicine, Children’s
preclude planned chemotherapy for the treatment of acute lym- Hospital Colorado, Department Of Surgery, Division Of Urology, And The
phoblastic leukemia (ALL). As conflicting practices exist for dose Surgical Oncology Program, Aurora, United States of America; 7 Children’s
modifications with older age, obesity and/or large body surface area Healthcare of Atlanta - Egleston, Oncology, Atlanta, United States of Amer-
(BSA), our objective was to examine the association of AAT with these ica; 8 Cincinnati Children’s Hospital Medical Center, Division Of Pediatric
risk factors. Oncology, Cincinnati, United States of America; 9 University of Michigan,
Methods: We examined Induction data from patients ages 1-30 years C.S. Mott Children’s Hospital, Pediatric Surgery, Ann Arbor, United States of
enrolled in the Children’s Oncology Group ALL trials AALL0232 America; 10 Children’s Healthcare of Atlanta, Pediatric Radiology, Atlanta,
and AALL0434. Pegaspargase (2,500 IU/m2 ) was administered with- United States of America; 11 Children’s Hospital of Colorado, Pathology,
out prescribed dose-capping. Prospectively evaluated AATs included Aurora, United States of America; 12 Children’s Hospital of Wisconsin,
hyperbilirubinemia, elevated alanine aminotransferase (ALT), throm- Pathology, Milwaukee, United States of America; 13 Medical University of
bosis, and pancreatitis. Obesity was classified using body mass index South Carolina, Pediatric Radiology, Charleston, United States of America;
(BMI) ≥30 (or ≥95th percentile). BSA was analyzed continuously 14 Seattle Children’s Hospital, Surgery, Seattle, United States of America;
and dichotomized at 1.5m2 (equivalent to pegaspargase 3,750 IU, 15 Nationwide Children’s Hospital, Pediatric Surgery, Columbus, United
the threshold for permissible dose-capping). The association of AAT States of America; 16 Children’s National Hospital, Division Of Oncology,
with end-Induction minimal residual disease (MRD) ≥0.01% was Washington, DC, United States of America; 17 IWK Health Centre and
assessed. Dalhousie University, Department Of Pediatric Hematology/oncology,
Results: Using data from 4,925 patients, multivariable logistic regres- Halifax, Canada; 18 Children’s Hospital Boston/Dana-Farber Cancer
sion analyses found increased risk for any AAT in all age groups Institute, Department Of Pediatric Oncology, Boston, United States of
≥10 years (p=0.002) and in patients with both obesity and high BSA America
(>1.5m2 ) (p<0.0001). Risks for hyperbilirubinemia, ALT elevations, and
thrombosis were increased in patients with high BSA and obesity (OR Background and Aims: The outcomes of Stage I-II Low Risk (LR)
3.5, 95% confidence interval [CI] 2.2-5.7), OR 3.3, 95%CI 1.7-6.6, and Favorable Histology Wilms Tumor (FHWT) patients enrolled to COG
OR 3.1 95%CI 1.5-6.5, respectively), but not in those with high BSA trials and the impact of single segmental chromosomal aberrations
alone. Age was not associated with thrombosis or ALT elevation; risk on outcome has not been previously reported but may inform risk
for pancreatitis was associated with Hispanic ethnicity, but not with stratification and therapy assignment in future therapeutic studies.
other factors. AAT were not associated with MRD ≥0.01%. Methods: We report event-free survival (EFS) of all Stage I and II LR
Conclusions: We report the largest dataset of AAT during ALL induc- FHWT patients who enrolled to the COG Renal Tumors Classifica-
tion. Risk for AAT was increased in patients ≥10 years and in those with tion, Biology and Banking study, AREN03B2 who were treated with
both obesity and high BSA, but not high BSA alone. Dose-capping may standard-of-care EE4A and assess the impact of single LOH 1p, or
not be necessary for patients with high BSA without obesity. ©2022 LOH 16q, and/or 1q gain on EFS. Patients meeting criteria for Very
American Society of Clinical Oncology, Inc. Reused with permission. Low Risk (nephrectomy only) or Standard Risk (combined LOH 1p and
This abstract was previously presented at the 2022 ASCO Annual 16q) were excluded. Kaplan-Meier estimates, log-rank tests, and Cox
Meeting. All rights reserved. proportional hazards models were used to evaluate biomarker effects.
Results: In the overall cohort of LR FHWT patients treated with EE4A,
4-year EFS was 95% for Stage I (CI: 93-98%; n=319) and 87% for Stage
O275 / #1539 IMPACT OF BIOMARKERS ON OUTCOME IN II (95% CI: 84-90%; n=555, p=0.0003). While no adverse biological fac-
STAGE I AND II LOW RISK FAVORABLE HISTOLOGY WILMS tor was significantly associated with EFS among Stage I LR patients, in
TUMORS. A REPORT FROM THE CHILDREN’S ONCOLOGY the subset of stage II LR patients, 4-year EFS with and without LOH
GROUP AREN03B2 PROTOCOL 1p were 79% (95% CI: 67-94%) and 87% (95% CI:84-90%, HR=1.64,
p=0.21); 4-year EFS with and without LOH 16q were 75% (95% CI: 65-
Kelly Vallance1 , Lindsay Renfro2 , Nicholas Evageliou3 , Daniel 86%) and 89% (95% CI:86-92%, HR=2.51, p=0.0004); and 4-year EFS
Benedetti4 , Elizabeth Perlman5 , Nicholas Cost6 , Andrew Hong7 , with and without 1q gain were 63% (95% CI: 44-89%) and 90% (95%
Kathryn Sutton7 , James Geller8 , Peter Ehrlich9 , Geetika Khanna10 , CI:82-99%, HR=4.4, p=0.006).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S137 of S635

Conclusions: Outcomes of Stage I FHWT LR patients were better than POSTER DISCUSSION SESSION
for Stage II LR patients. Adverse biologic factors were associated with
poorer prognosis in Stage II but not Stage I FHWT patients. These POSTER DISCUSSIONS SESSION 04: NOVEL PROGNOSTIC
results support new risk stratification and treatment strategies that we FACTORS 01-10-2022 1:10 PM - 2:10 PM
plan to assess in a prospective clinical trial.
O277 / #768 BEYOND SINGLE GENETIC ABERRATIONS: AN
INTEGRATIVE COPY NUMBER ALTERATION-DRIVEN SCORING
O276 / #1474 GENOTYPE-PHENOTYPE CORRELATIONS IN GUIDE TO REFINE CYTOGENETIC RISK STRATIFICATION IN
PATIENTS WITH GERMLINE WT1-VARIANTS IN A NATIONAL PEDIATRIC ACUTE LYMPHOBLASTIC LEUKEMIA
COHORT
Bálint Egyed1,2 , Gábor Bedics2 , Zsuzsanna Jakab3 , Dániel Erdélyi1 ,
Sophie Van Peer1 , Janna Hol1 , Sanne Egging1 , Maria Lombardi2 , Anja Judit Müller1 , Lili Kotmayer2 , Anne Benard-Slagter4 , Karel De Groot4 ,
Wagner3 , Marry Van Den Heuvel-Eibrink1 , Roland Kuiper1,4 , Marjolijn Szilvia Krizsán2 , Anna Bekő2 , Béla Kajtár5 , László Pajor5 , Ágnes
Jongmans1,5 Vojcek6 , Gábor Ottóffy6 , Anikó Ujfalusi7 , Csongor Kiss8 , Réka Simon9 ,
1 Princess Máxima Center, Pediatric Oncology, Utrecht, Netherlands; Lilla Tiszlavicz10 , Krisztina Csanádi11 , Gergely Kriván12 , Suvi Savola4 ,
2 Amsterdam UMC, Clinical Genetics, Amsterdam, Netherlands; 3 Erasmus Csaba Bödör2 , Gábor Kovács1 , Donát Alpár2
MC, Clinical Genetics, Rotterdam, Netherlands; 4 University Medical Cen- 1 Semmelweis University, 2nd Department Of Pediatrics, Budapest, Hun-
ter Utrecht, Genetics, Utrecht, Netherlands; 5 University Medical Center gary; 2 Semmelweis University, Hcemm-su Molecular Oncohematology
Utrecht, Clinical Genetics, Utrecht, Netherlands Research Group, 1st Department Of Pathology And Experimental Cancer
Research, Budapest, Hungary; 3 Hungarian Pediatric Oncology Network,
Background and Aims: WT1 disorder is characterized by combi- Hungarian Childhood Cancer Registry, Budapest, Hungary; 4 MRC, Holland,
nations of glomerulopathy, urogenital anomalies and Wilms tumor. Amsterdam, Netherlands; 5 University of Pécs Clinical Centre, Department
There is bias towards description of patients with variants in the Of Pathology, Pécs, Hungary; 6 University of Pécs Clinical Centre, Depart-
DNA-binding/Zinc-finger domain of WT1 (exon 8/9). We observed ment Of Pediatrics, Pécs, Hungary; 7 University of Debrecen, Department
an enrichment of variants outside this region in children with Wilms Of Laboratory Medicine, Debrecen, Hungary; 8 University of Debrecen, Divi-
tumor and found it challenging to predict their glomerulopathy risk. sion Of Pediatric Hematology-oncology, Institute Of Pediatrics, Debrecen,
This prompted us to study phenotype-genotype correlations in a Hungary; 9 Velkey László Children’s Health Center, Hemato-oncology And
national cohort of individuals with WT1-variants. Stem Cell Transplantation Unit, Miskolc, Hungary; 10 University of Szeged,
Methods: We approached all Dutch genetic laboratories and requested Department Of Pediatrics And Pediatric Health Care Center, Szeged, Hun-
pseudoanonymized data of all patients with germline WT1-variants. gary; 11 Heim Pál National Pediatric Institute, Kemény Pál Hemato-oncology
Results: We identified 36 patients with (likely) pathogenic WT1- Unit, Budapest, Hungary; 12 Central Hospital of Southern Pest - National
variants (median age at inclusion 13.3 years, range 2.8-58). Seventeen Institute of Hematology and Infectious Diseases, Pediatric Hematology And
patients had truncating variants, 12 of which located outside the DNA- Stem Cell Transplantation Unit, Budapest, Hungary
binding/Zinc-finger domain. All 17 patients had Wilms tumors (median
age 12 months, range: 6-35), two developed glomerulopathy leading Background and Aims: Recently, numerous leukemia-relevant copy
to kidney failure (age 26 and 40 years). Nine patients had missense number alterations (CNAs) were assessed as patient stratification tools
variants, all in the DNA-binding/Zinc-finger domain. Of those, two in pediatric acute lymphoblastic leukemia (ALL). In our multicentric
developed Wilms tumors (age 12 and 18 months), 9/9 glomerulopathy study of the Hungarian Pediatric Leukemia Molecular Profiling Pro-
(median age 1 month, range: 0 months-54 years) and 8/9 kidney failure gram, we aimed to integrate classical cytogenetic risk factors with CNA
(median age 1 month, range: 0-23). Three patients had splice-site vari- profiles for survival prediction.
ants (introns 1, 6, 7). Two developed Wilms tumor (intron 6-7) and 3/3 Methods: Diagnostic bone marrow samples from 260 children with
kidney failure (median age 33 months, range: 10-333). Three patients precursor B-cell ALL were comprehensively screened by digital mul-
had intron 9 splice variants, none developed Wilms tumor, all had kid- tiplex ligation-dependent probe amplification (digitalMLPA, MRC Hol-
ney failure (median age 173 months, range: 24-240). Four patients had land) using ALL-specific D007 probemixes on Illumina MiSeq platform.
a (partial) WT1 deletion, all had Wilms tumor and none glomerulopathy. Bioinformatic analysis was performed using the Coffalyser software
Four patients experienced WT1-related glomerulopathy onset after (MRC Holland).
Wilms tumor (median age 26.5 years, range: 2-40). Results: On average, 5.4 CNAs (gross chromosomal and subchromoso-
Conclusions: We observed remarkable few patients with infan- mal aberrations) were detected per patient with a mean of 2.5 subchro-
tile/childhood glomerulopathy onset when the WT1-variant is outside mosomal alterations. Among patients with high hyperdiploid (HHD)
the DNA-binding/Zinc-finger domain. These patients can however karyotype, the concurrent gain of chromosome 4 and 6 proved to be
be at risk for glomerulopathy later in life. Therefore, we suggest a highly favorable predictor (5-year EFS: 93.2%, other HHD patients:
life-long surveillance for glomerulopathy for these patients and kidney- 60.4%; p=0.0005). Several subchromosomal CNAs in disease-relevant
preserving treatment when affected by Wilms tumor. genes were identified (e.g. CDKN2A/B in 45.6% of patients, PAX5
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S138 of S635 ABSTRACTS

13.4%, IKZF1 11.5%). Prognostic categories defined by CNAs in IKZF1 Methods: Multiplex ligation-dependent probe amplification assay was
and related genes (normal or deleted IKZF1, IKZF1plus ) were refined adapted for formalin-fixed specimens and validated against matched
considering the cytogenetic risk groups suggested by Moorman et al., frozen tissues and genotyping methods, then applied to 61 tumors
which resulted in a more accurate risk stratification (IKAROS-low vs. from 55 Asian and non-Asian patients treated with National Wilms
IKAROS-intermediate, p=0.002; IKAROS-intermediate vs. IKAROS- Tumor Study regimens in Singapore hospitals from 2001-2022, and
high, p=0.048). Finally, we created a novel integrated score system by a validation set (n=14) from a separate Asian population. Log2 copy
proportionally weighting individual cytogenetic aberrations and sub- number ratios and LOH (defined as gain/loss of ≥2 consecutive loci)
chromosomal CNAs (e.g. harboring ETV6-RUNX1 counts +1.5 points; were correlated with clinical variables.
TP53 deletion: -0.25 points). Patient-specific cumulative scores gen- Results: Clustering analysis identified a subgroup of predominantly
erated from the prognostic value of single lesions distinguished two non-Asian and relapsed patients with 1p, 16q loss, and 1q gain, while
favorable (60% of patients; excellent and low risk, 96.1% and 87.9% a subgroup (21%) with predominantly Asian ethnicity, low stage and
5-year EFS; p=0.041) and two unfavorable (40% of patients; high and few relapses was characterized by MYCN exon 2 with/without PAX3
ultra-poor risk, 64.3% and 30.6% 5-year EFS; p=0.004) prognostic gain, and minimal 1p/1q/16q aberrations. Overall, 23.0% of Singapore
groups. cases, and 37.5% of a Vietnamese validation cohort had MYCN exon 2
Conclusions: DigitalMLPA offers a fast and standardizable DNA copy gain. Univariate t-test verified that PAX3 and MYCN gain were signifi-
number analysis, which is implementable in the diagnostic work- cantly associated with Asian ethnicity (P=0.02, P=0.001, respectively);
flow of pediatric ALL and expands genetics-based risk prediction among Asians, PAX3 loss was associated with relapse (P=0.02). WT1
perspectives. LOH was associated with older age and metastases (P=0.02, P=0.03,
respectively), and TP53 LOH with older age, metastases and anapla-
sia (P=0.04, P=0.004, P<0.001, respectively). Notably, 1q gain, but
O278 / #863 UNIQUE PROGNOSTIC ASSOCIATIONS OF not 1p/16q LOH, was associated with relapse (P=0.001). Correspond-
COPY NUMBER CHANGES OF GENOMIC LOCI IN ASIAN ingly, relapse was associated with older age (P<0.05), higher stage and
WILMS TUMOR unfavorable histology (P<0.01).
Conclusions: In Asian Wilms tumor patients, LOH of 1q, but not 1p and
Kia Teng Lim1 , Meng Kang Wong2 , Thi Ngoc Thuy Do3 , Sheng Hui 16q, is prognostic for relapse. Gain of MYCN and its downstream target
Tan2 , Isshani Devaraj4 , Chik Hong Kuick5 , Khawn Tawng6 , Prasad PAX3 is frequent in Asian populations and may uniquely represent a
Iyer2,6,7 , Shui Yen Soh2,6,7 , York Tien Lee2,7,8 , Eric Yeo1,9 , Victor Lee1,9 , marker of favorable outcome that demands further study.
Thuan Chong Quah1,10 , Chi Bao Bui11 , Dang Anh Thu Phan3,12 ,
Kenneth Chang2,5,7 , Amos Loh2,7,8
1 National University of Singapore, Yong Loo Lin School Of Medicine, Singa- O279 / #1201 TRANSCRIPTOME ANALYSIS REVEALS
pore, Singapore; 2 KK Women’s and Children’s Hospital, Viva-kkh Paediatric ASSOCIATION OF NEW LONG NONCODING RNAS WITH POOR
Brain And Solid Tumour Programme, Children’s Blood And Cancer Centre, CLINICAL PROGNOSIS IN PEDIATRIC ADRENOCORTICAL
Singapore, Singapore; 3 Children’s Hospital 1, Department Of Pathology, Ho TUMORS
Chi Minh City, Viet Nam; 4 National University of Singapore, Department
Of Biological Sciences, Singapore, Singapore; 5 KK Women’s and Children’s Luis Fernando Nagano1 , Luciana Veronez2 , Carolina Corrêa1 , Paula
Hospital, Department Of Pathology And Laboratory Medicine, Singapore, Cristina Santos2 , Rosane Queiróz2 , Sonir Antonini2 , Silvia Brandalise3 ,
Singapore; 6 KK Women’s and Children’s Hospital, Paediatric Haematol- José Yunes3 , Luiz Tone2 , Kleiton Borges2,4 , Carlos Scrideli2
ogy Oncology Service, Children’s Blood And Cancer Centre, Singapore, 1 Ribeirão Preto Medical School, University of São Paulo, Department
Singapore; 7 National University of Singapore, Duke Nus Medical School, Of Genetics, Ribeirão Preto, Brazil; 2 Ribeirão Preto Medical School, Uni-
Singapore, Singapore; 8 KK Women’s and Children’s Hospital, Department versity of São Paulo, Department Of Pediatrics, Ribeirão Preto, Brazil;
Of Paediatric Surgery, Singapore, Singapore; 9 National University Hospital, 3 Boldrini‘s Children Center, Pediatrics, Campinas SP, Brazil; 4 Boston Chil-
Division Of Pathology, Singapore, Singapore; 10 National University Hospi- dren’s Hospital, Department Of Endocrinology, Boston, United States of
tal, Division Of Paediatric Haematology Oncology, Singapore, Singapore; America
11 Vietnam National University, School Of Medicine, Ho Chi Minh City, Viet
Nam; 12 University of Medicine and Pharmacy, Department Of Pathology, Ho Background and Aims: Pediatric adrenocortical tumors (pACT) are
Chi Minh City, Viet Nam rare and aggressive adrenal gland neoplasms. However, molecular
markers and therapeutic targets for prognosis and treatment of pACTs
Background and Aims: In Asians, Wilms tumors display uniquely are scarce. Considering that lncRNAs are arising as central regulators
low incidence and favorable biological features. Having previously in oncogenesis and several hallmarks of cancer, the identification of
shown that 1p/16q co-loss of heterozygosity (LOH) was not prog- deregulated lncRNAs in this pediatric tumor is of great interest, and
nostic in Asians, we sought to study the association of copy number new candidates for prognostic markers may emerge.
changes in other genomic loci with relapse and ethnicity in a multiracial Methods: We performed RNA sequencing (RNA-seq) of 14 pACTs sam-
population-based cohort. ples and analyzed the data from an additional pACTs cohort to search
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S139 of S635

for lncRNAs associated with prognosis in pACTs. For the RNA-seq Orthopedic And Trauma Department, Paris, France; 11 Centre Léon Bérard,
experiment, our cohort was subdivided into two groups: patients with Department Of Biopathology, Lyon, France; 12 La Timone University Hospital
death or relapse events (poor prognosis, n=5) and patients without of Marseille, APHM, Marseille, France, Department Of Pediatric Oncology,
events (good prognosis, n=9), once there is no consensus on molecu- Marseille, France; 13 Hopital La Timone, Department Of Medical Oncology,
lar subgroups for pACTS. This criteria was also used for the additional Marseille, France; 14 IUCT oncopole, Department Of Medicine, Toulouse,
pACTs cohort (public dataset: GSE76019, n=34). All correlation tests France; 15 Institut Curie, Department Of Medical Oncology, Paris, France;
were performed based on Spearman’s coefficient. Positive and nega- 16 Institut Gustave Roussy, Department Of Medical Oncology, Villejuif,
tive correlated genes were submitted to pathways enrichment using France; 17 Institut du Cancer de Montpellier, Department Of Radiother-
the Reactome database. apy, Montpellier, France; 18 Institut Bergonié, Department Of Biopathol-
Results: Transcriptome analysis of our in house generated RNA-seq ogy, Bordeaux, France; 19 Institut Curie, Department Of Somatic Genetics,
and GSE76019 revealed four common differentially expressed lncR- Paris, France; 20 Nantes Hospital, Department Of Orthopedic And Trauma
NAs between the good and poor prognostic samples, using log2 Department, Nantes, France; 21 IUCT oncopole, Department Of Biopathol-
fold change > 1 or < −1 & adj. p-value < 0.05 as threshold for both ogy, Toulouse, France; 22 Centre Georges-François Leclerc, Department Of
analysis. LINC01138, CRNDE, PD6G-AS1 were up-regulated while Surgery, Dijon, France; 23 Besançon hospital, Department Of Medical Oncol-
LINC00400 was down-regulated in poor prognosis pACTs. Nine- ogy, Besançon, France; 24 Institu Bergonié, Department Of Medical Oncoly,
teen common differentially expressed mRNAs were shown to be Bordeaux, France; 25 Centre Léon Bérard, Department Of Medical Oncology,
LINC01138 predicted targets and negatively correlated with it. In addi- Lyon, France; 26 Institut Curie, Siredo Oncology Center (care, Innovation And
tion, LINC01138 demonstrated to be highly correlated with the cell Research For Children, Adolescents And Young Adults With Cancer), Paris,
proliferation marker MKI67 and co-regulated genes of LINC01138 France
involved in cell cycle regulation and MHC Class I processing and
presentation. Background and Aims: Some soft tissue sarcomas are consid-
Conclusions: In summary, our study showed associations between ered ultra-rare in children [0-18 year, y], as liposarcoma (LPS),
lncRNAs profiles and pACTs prognosis, in addition to candidate lncR- leïomyosarcoma (LMS) and alveolar soft part sarcoma (ASPS).
NAs that can be used in future studies related to diagnosis/prognosis or No standardized therapeutic guidelines are available for this
as targets for pACTs treatment. (Grant FAPESP: 2014/20341-0; Grant population. This study aimed to compare their clinical presen-
CNPQ: 140305/2020-3). tation and behavior with their counterparts in young adults
[19-30 y].
Methods: National retrospective comparative multicenter study of
O280 / #290 ULTRA RARE SOFT PART SARCOMAS IN patients (0-18 vs. 19-30 y) with LPS, LMS, or ASPS included in the
YOUNG PATIENTS: ARE THEY DIFFERENCES ACCORDING TO "ConticaBase" Sarcoma database, treated between 2010 and 2019. All
AGE OF OCCURRENCE? THE FRENCH NATIONAL NETSARC+ diagnosis have been reviewed by expert pathologists’ members of the
NETWORK EXPERIENCE Netsarc+ network.
Results: Overall, 184 patients were identified: 35 children (19%) and
Anne-Laure Genevois1 , Matthieu Carton2 , Joanna Cyrta3 , Nadege 149 adults (81%). Tumor type distribution was: 14 LPS, 6 LMS and
Corradini4 , Pablo Berlanga5 , Claire Chemin-Airiau6 , Charles Honoré7 , 15 ASPS in the [0-18y], 92 LPS, 40 LMS and 17 ASPS in the [19-
Sophie El Zein3 , Anne-Sophie Defachelles8 , Emmanuelle Bompas9 , 30y] group (p<0.001). In children, LPS were smaller (median, 5cm vs.
Philippe Anract10 , Marie Karanian11 , Angélique Rome12 , Florence 10cm; p=0.005) and LMS were more frequently localized in limbs
Duffaud13 , Christine Chevreau14 , Sarah Watson15 , Axel Le Cesne16 , (83% vs. 30%; p=0.02). ASPS characteristics were similar. The propor-
Carmen Llacer17 , François Le Loarer18 , Gaelle Pierron19 , François tions of high-grade tumors were similar for LPS (7% vs. 5%; P=1.00)
Gouin20 , Anne Gomez Mascard21 , Sylvain Causeret22 , Elsa and for LMS (17% vs. 32%; P= 0.74). After median follow-up of 54
Kalbacher23 , Françoise Ducimetière6 , Maud Toulmonde24 , Jean-Yves months (range: 0-138), children with LPS and ASPS had similar out-
Blay25 , Daniel Orbach26 , Myriam Jean-Denis6 come in term of pattern of relapse, but pediatric patients with LMS
1 Institut Curie, Siredo Oncology Center (care, Innovation And Research developed less distant metastasis [fll1] (0% vs.52%; p=0.025). Three-
For Children, Adolescents And Young Adults With Cancer), University Of year overall survival in children vs. adults was 82% [95%CI, 62-100]
Paris-saclay, Paris, France; 2 Institut Curie, Department Of Biometry, Drci, vs.98% [64-100, p=0.13] for LPS, 100% [100-100] vs. 78% [68-94,
Psl Research University, Paris, France; 3 Institut Curie, Department Of p=0.13] for LMS and 92% [79-100] vs.92% [79-100, p=0.52] for ASPS.
Biopathology, PARIS, France; 4 IHOPe, Léon Bérard center, Department Of Three-year overall survival in children vs. adults was 82% [95%CI,
Pediatric Hematology And Oncology, Lyon, France; 5 Gustave-Roussy, Can- 62-100] vs.98% [64-100, p=0.13] for LPS, 100% [100-100] vs. 78% [68-
cer Campus, Department Of Pediatric And Adolescent Oncology, Villejuif, 94, p=0.13] for LMS and 92% [79-100] vs.92% [79-100, p=0.52] for
France; 6 Centre Léon Bérard, Netsarc+ Transversal Task Force, Lyon, France; ASPS.
7 Institut Gustave Roussy, Department Of Surgery, Villejuif, France; 8 Centre Conclusions: These preliminary results suggest that LPS and ASPS
Oscar Lambret, Department Of Pediatric Oncology, Lille, France; 9 Institut have quite comparable presentation and outcomes regardless of age,
Cancérologie de l’Ouest„ Medecine, Nantes, France; 10 Cochin Hospital, whereas LMS have more favorable outcomes in children.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S140 of S635 ABSTRACTS

POSTER DISCUSSION SESSION O282 / #1691 HYDROCORTISONE OR PLACEBO TO REDUCE


DEXAMETHASONE-INDUCED NEUROBEHAVIORAL SIDE
POSTER DISCUSSIONS SESSION 05: NOVEL APPROACHES TO EFFECTS – FINAL RESULTS OF THE DEXADAYS-2 STUDY
SUPPORTIVE CARE 01-10-2022 1:10 PM - 2:10 PM
Annelienke Van Hulst1 , Erica Van Den Akker2 , Emma Verwaaijen1 ,
O281 / #629 SMARTER SYMPTOM MANAGEMENT FOR Marta Fiocco3,4 , Niki Rensen1 , Raphaele Van Litsenburg1 , Saskia
CHILDREN WITH CANCER: DEVELOPMENT AND EVALUATION Pluijm1 , Hanneke Van Santen1,5 , Rob Pieters1 , Andrea Evers6 , Martha
PLANS OF A SMARTPHONE APP TO MONITOR A. Grootenhuis7 , Marry Van Den Heuvel-Eibrink1
PATIENT-REPORTED OUTCOME MEASURES (RESPONSE) 1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology,
Utrecht, Netherlands; 2 Erasmus Medical Center-Sophia Children’s Hos-
Natalie Bradford1 , Penelope Slater2 , Philippa Fielden3 , Paula pital, Department Of Endocrinology, Rotterdam, Netherlands; 3 Princess
Condon2 , Claire Radford3 , Amanda Carter3 , Rick Walker2 , Ashraf Maxima Center for Pediatric Oncology, Trial And Data Center, Utrecht,
Badat3 , Rachel Edwards2 , Brooke Spencer3 , Anthony Herbert2 Netherlands; 4 Leiden University, Mathematical Institute, Leiden, Nether-
1 Queensland University of Technology, Cancer And Palliative Care Out- lands; 5 Wilhelmina Children’s Hospital, University Medical Center Utrecht,
comes Center- Child And Youth Program Lead, WOOLOOWIN, Australia; Department Of Pediatric Endocrinology, Utrecht, Netherlands; 6 Leiden Uni-
2 Children’s Health Queensland, Oncology, Brisbane, Australia; 3 Children’s versity, Institute Of Psychology, Leiden, Netherlands; 7 Princess Maxima
Health Queensland, Oncology, Brisbane, Australia Centre for pediatric oncology, Psycho Oncology, Utrecht, Netherlands

Background and Aims: Symptoms and associated distress in children Background and Aims: Dexamethasone is a cornerstone of pediatric
diagnosed and treated for cancer is uniformly common, yet struc- acute lymphoblastic leukemia (ALL) therapy, but it can induce seri-
tured and systematic symptom monitoring is not yet commonplace. ous neurobehavioral side effects. Our previous study suggests that
With increasing pressures on the healthcare workforce, improved children who suffer most from these side effects might benefit from
systematic approaches to symptom management are required. addition of a physiological dose of hydrocortisone to dexamethasone
Methods: Following a review of relevant literature, the Sympotm treatment. Here we aimed to confirm this finding in pediatric patients
Screening Pediatrics tool(SSPedi), a Patient-Reported Outcome Mea- with ALL who experience clinically relevant dexamethasone-induced
sure (PROM) validated for children with cancer (4-18 years) was neurobehavioral problems.
selected as an appropriate measure to explore routine assessment of Methods: We performed a double-blind, randomized controlled trial
symptom burden. Medical, nursing and allied health oncology clinicians (RCT) with cross-over design, including 52 children with ALL who expe-
developed algorithms using consensus techniques to ascertain thresh- rienced clinically relevant dexamethasone-induced neurobehavioral
olds of concern for each symptom. Algorithm vetting and refinement problems. A baseline measurement during one dexamethasone course
were achieved through iterative interviews and consultation with clin- was performed, the intervention was administered during the subse-
icians. Self-management evidence-based recommendations for each quent four dexamethasone courses. The intervention consisted of two
symptom were also developed. Each algorithm uses a three-tiered ‘traf- courses hydrocortisone (10mg/m2 /day) in a circadian rhythm, followed
fic light’ model to outline the appropriate management and resources by two courses placebo, or vice versa. Neurobehavioral problems were
to support clinical staff with evidence-based symptom management measured with the parent-reported Strengths and Difficulties Ques-
were also developed. The algorithms were incorporated into a smart- tionnaire. Secondary endpoints were sleep problems, quality of life
phone application (app), and an iterative evaluation is planned to test (QoL), hunger feeling and parental stress, measured with separate
safety, implementation, and efficacy of the app. questionnaires. A generalized mixed model was estimated to study the
Results: Digital solutions provide alternative options for communica- effect of the intervention on all outcomes.
tion, information, and education and harness the best of community Results: Both interventions, hydrocortisone and placebo, significantly
and hospital services. The RESPONSE system is a comprehensive decreased dexamethasone-induced neurobehavioral problems (esti-
approach to symptom management that includes a suite of tools and mated effect hydrocortisone -5.0 (95%-confidence interval (CI) -8.2
resources including a smartphone app that collects PROMs and deliv- to -1.8, p=0.002), estimated effect placebo -7.2 (95%-CI -10.4 to -
ers symptom management advice, as well as written resources for both 4.1, p<0.001). There was no benefit of hydrocortisone compared to
the families of children with cancer and clinicians. placebo. Sleeping problems, hunger feeling and parental stress also
Conclusions: Children with cancer and their families have a distinct decreased significantly during both interventions compared to no
need for support, which includes information and education about intervention.
managing the acute, and long-term impacts of cancer and cancer Conclusions: Our results showed that clinically relevant
treatment. Incorporating technological and digital solutions offer new dexamethasone-induced neurobehavioral problems significantly
ways to consider how families and clinicians communicate and share decreased after intervention with hydrocortisone or placebo in
information. We will evaluate effects on information exchange and pediatric patients with ALL, but no additional beneficial effect of
symptom management for children and families, workflow processes hydrocortisone compared to placebo was found. The next step is to
for clinicians and organisational implications. find the best way to offer these effective interventions in an open-label
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S141 of S635

way to children who experience clinically relevant side effects in Conclusions: After accounting for baseline MTX, the efficacy of glu-
clinical practice. carpidase at inducing CIR in paediatric, adolescent, and YA patients
with MTX<50 μmol/L (CIR: 83%) aligned with that previously reported
in the overall (paediatric+adult) population (CIR: 59%),1 demonstrat-
O283 / #224 GLUCARPIDASE USE IN 86 PAEDIATRIC AND ing no dose adjustment is required in this setting. 1. Widemann et al.
YOUNG ADULT PATIENTS RECEIVING HIGH-DOSE Pharmacotherapy 2014;34:427–39.
METHOTREXATE THERAPY: POOLED SUBGROUP ANALYSIS OF
OPEN-LABEL TRIALS
O284 / #825 RANDOMIZED CONTROLLED TRIAL OF
Katherine Janeway1 , Luis Gros2 , Stefan Schwartz3 , Claire Daugherty4 , NEUTROPENIC DIET VERSUS REGULAR DIET IN PATIENTS
Eva Gallardo5 , Christon Hill4 , Emma Thomas5 , Suzanne Ward4 , UNDERGOING INDUCTION CHEMOTHERAPY FOR ACUTE
Carmelo Rizzari6 LEUKEMIA
1 Dana-Farber Cancer Institute and Harvard Medical School, Boston Chil-
dren’s Cancer And Blood Disorders Center, Boston, United States of America; Perraju Bhaskar Bhuvan Lagudu1 , Venkatraman Radhakrishnan1 ,
2 Vall d’Hebron University Hospital, Vall D’hebron Research Institute And Devleena Gangopadhyay1 , Swaminathan Rajaraman2 , Jayachandran
Department Of Pediatric Hematology And Oncology, Barcelona, Spain; Perumal Kalaiyarasi1 , Prasanth Ganesan1 , Varalakshmi Vijaykumar3 ,
3 Charité – Universitätsmedizin Berlin, corporate member of Freie Uni- Trivadi Ganesan4
versität and Humboldt-Universität zu Berlin, Department Of Hematology, 1 Cancer institute (WIA) Adyar, Medical Oncology, Chennai, India; 2 cancer
Oncology And Tumor Immunology, Berlin, Germany; 4 BTG International Inc., institute (WIA) Adyar, Bio Statistics, chennai, India; 3 Cancer Institute,
Btg International Inc., Conshohocken, United States of America; 5 Protherics Chennai, India, Microbiology, CHENNAI, India; 4 Sriramachandra medical
Medicines Development Ltd., Protherics Medicines Development Ltd., Lon- centreand hospital, Medical Oncology, chennai, India
don, United Kingdom; 6 University of Milano-Bicocca, MBBM Foundation,
Pediatric Hematology-oncology Unit, Department Of Pediatrics, Monza, Background and Aims: Restriction of raw fruits and vegetables (neu-
Italy tropenic diet) is advised to patients receiving treatment for acute
leukemia in low- and middle-income countries (LMIC) to reduce infec-
Background and Aims: To characterize glucarpidase efficacy and tions despite evidence to the contrary from high-income countries. We,
safety in compassionate use (CU) trials of patients undergoing high- therefore, conducted a randomized controlled trial (RCT) to ascertain
dose methotrexate (MTX) therapy. the efficacy of the neutropenic diet in an LMIC setting.
Methods: We performed post hoc analysis of Infants (aged ≥28 days- Methods: Patients aged 1-60 years receiving induction chemother-
<2 years), Children (≥2-<12 years), Adolescents (≥12-<15 years), apy for acute leukemia were randomized to a regular or neutropenic
and Young Adults (YAs) (≥15-<25 years) from 4 multicentre, open- diet between Feb 2018- Mar 2020. Participants followed food safety
label, single-arm, glucarpidase CU trials. Patients had delayed MTX guidelines in both arms. The study’s primary objective was to compare
elimination secondary to renal dysfunction and received glucarpidase the incidence of major infections (pneumonia, urinary tract infection,
(50 U/kg). The primary endpoint was clinically important reduc- bacteremia, fungemia, meningitis, cellulitis, diarrhea, or any infection
tion in plasma MTX (CIR; MTX≤1 μmol/L at all post-glucarpidase considered to be significant by the investigator) between patients
measurements) based on high-performance liquid chromatography receiving the two diets. The secondary objectives were to compare
(HPLC). minor infections, stool microbial flora, and mortality rates.
Results: Eighty-six patients had ≥1 HPLC evaluation and were included Results: We randomized 200 patients, 98 patients to the regular diet
in the efficacy analyses (patients without HPLC data were excluded). arm and 102 to the neutropenic diet arm. The median age was 13-years.
CIR was achieved by 12/15 patients (80.0%) with acute lymphoblastic Major infections occurred in 32% of patients in the regular diet arm
leukaemia, 7/14 (50.0%) with non-Hodgkin lymphoma, 14/47 (29.8%) and 25% in the neutropenic diet arm (P=0.26). There were no statis-
with osteosarcoma, 1/4 (25.0%) where tumour type was ‘other’, and tically significant differences between patients receiving regular diet
4/6 (66.7%) where tumour type was unknown. CIR was achieved by vs. neutropenic diet for blood culture positivity (n= 6 vs. 9), inotropic
0/1 Infants (0.0%), 5/16 Children (31.3%), 7/24 Adolescents (29.2%), support (17 vs. 12), mechanical ventilation (8 vs. 5), third-line antibi-
and 26/45 YAs (57.8%). Median reduction in MTX was ≥98.7% in each otic use (28 vs. 20), minor infections (12 vs. 9), induction mortality (9
age group 15 minutes post-glucarpidase. Patients with baseline central vs.4), and remission status (94% vs. 94%). The stool culture on day 15
MTX<50 μmol/L (35/42; 83.3%) were more likely to achieve CIR than of induction grew multi-drug-resistant bacteria in 37% of patients in
those with MTX≥50 μmol/L (1/37; 2.7%). The safety population com- the regular diet arm and 35% in the neutropenic diet arm (P=0.74). The
prised 271 paediatric/YA patients with ≥1 dose of glucarpidase or with three-year overall survival in the regular diet arm and neutropenic diet
evidence of follow-up after their first glucarpidase dose. The most com- arm was 67.6% and 76.7% (P=0.13).
mon treatment-related adverse event (AE) was paraesthesia, occurring Conclusions: Neutropenic diet compared to regular diet did not pre-
in 3 Adolescents (4.5%) and 6 YAs (5.2%). No other treatment-related vent infections, reduce mortality, or change stool microbial flora in
AE occurred in ≥5% of any age group. patients with acute leukemia
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S142 of S635 ABSTRACTS

POSTER DISCUSSION SESSION O286 / #4 PAEDIATRIC MEDICAL TRAUMATIC STRESS IN


CHILDREN WITH CANCER AND THEIR PARENTS: DIFFERENCES
POSTER DISCUSSIONS SESSION 06: BEST OF GLOBAL HEALTH IN LEVELS OF POSTTRAUMATIC STRESS SYMPTOMS
01-10-2022 1:10 PM - 2:10 PM
Sandra Klašnja1 , Ivana Kreft Hausmeister1 , Marko Kavčič1 , Robert
O285 / #448 THE PRACTICE OF ONCOPSYCHOLOGISTS Masten2
DURING THE FIRST DAYS OF WAR IN UKRAINE 1 University Medical Centre Ljubljana, Division Of Paediatrics, Ljubljana,
Slovenia; 2 University of Ljubljana, Faculty Of Arts, Ljubljana, Slovenia
Olesya Horlenko1 , Vladyslava Andrushchenko2 , Marianna Nych3 , Lilia
Sirokha4 , Alla Antonova4 , Nataliia Shevchenko5 , Liudmyla Baletska6 , Background and Aims: Paediatric medical traumatic stress (PMTS) is
Viktor Pushkarenko7 , Olga Pushkarenko1 a set of children’s and their parents’ psychological and physiological
1 Uzhhorod National University, Children’s Hospital, Uzhhorod, Ukraine; responses to pain, injury, serious illnesses, and other experiences with
2 City Clinical Children’s Hospital № 16, Pediatrics, Kharkiv, Ukraine; the medical environment. Paediatric cancer patients have the highest
3 Charitable Foundation "Zaporuka", Western Ukrainian Specialized Chil- prevalence of PMTS as the illness involves a set of stressors that trigger
dren’s Medical Center, Lviv, Ukraine; 4 National Cancer Institute, Pediatrics, many negative psychological reactions. Posttraumatic stress symp-
Kyiv, Ukraine; 5 NGO Kharkiv Foundation for Psychological Research, Pedi- toms (PTSS) are one of the most common psychopathologies among
atrics, Kharkiv, Ukraine; 6 Uzhhorod National University, Department Of cancer patients. We examined the incidence of PMTS in children with
Psychology, Uzhhorod, Ukraine; 7 Uzhhorod National University, Medicine cancer and their parents due to coping with a serious illness and treat-
Faculty, Uzhhorod, Ukraine ment complications. We analysed the following risk factors for PTSS:
selected groups of individuals, medical interventions, complications,
Background and Aims: Every child in Ukraine has realized that and treatment modalities.
new concepts have appeared in life, including "air alarm", "bomb- Methods: The study involved 183 parents of 133 children and 62 chil-
ing", "war" from February 24, 2022. Children undergoing cancer dren and adolescents who were treated between 2009 and 2019 at
treatment are forced to hide for weeks with the medical team and Clinical Department of Paediatric Haematology and Oncology of Pae-
parents in basements, storage facilities of the hospital to save their diatric Clinic in Ljubljana. We collected the data using The Intensity of
lives. The evacuation of families to safe cities and countries con- treatment rating scale 2.0 [IRT-2], PTSD checklist for Children/Parent
tinues, and we are grateful to everyone who has joined this rescue [PCL-C/PR], The PTSD Checklist for DSM-5 [PCL-5] and The Child
process. PTSD Symptoms Scale for DSM-5 [CPSS-5].
Methods: The supervision group of 10 oncopsychologists, a new Results: PMTS is frequently present in both, children and their parents,
questionnaire indicators of well-being and methods of psychologi- regardless of the cancer type, treatment duration, treatment outcome,
cal assistance were created.Psychological assistance was provided to and child’s age. Mothers, patients with relapse, patients who were diag-
187 children with cancer and 115 parents, during war - 82 and 32 nosed after age 5, patients with more intensive treatment, and parents
respectively. of the latter are at higher risk for PMTS occurrence. Additionally, we
Results: 70% of psychologists left their homes and moved to safer (con- found a decreasing trend of traumatic responses after five or more
ditionally) cities in Ukraine. 85% of children and their parents received years post cancer diagnosis and that parents are more traumatized
crisis psychological assistance. The first states in adults were deceler- than children.
ation, stupor (60%); denial (60%), awareness of the horror of events Conclusions: The systematic prevention of PMTS and endeavour of
(50%); anger (20%); fear, felling of irreversibility (20%). In children: trauma-informed care are required.
anger (50%), fear (43%), sadness for home and family (20%). The most
effective steps of self-help to self-stabilization in psychologists: contact
with relatives, breathing practices, development of a plan for different O287 / #320 PSYCHOSOCIAL DISTRESS OF ADULT
cases, actively involved in the identity of the psychologist; in children: CHILDHOOD CANCER SURVIVORS IN A SOUTH AFRICAN
art techniques to release emotions, establishing frequent supportive COHORT
contact in all possible ways with adults. Approximately 77% of children
and their families were evacuated to other countries to continue treat- Anel Van Zyl, Mariana Kruger, Paul Rogers
ment (which was the main fear of parents). 2 children lost their lives, Stellenbosch University and Tygerberg hospital, Department Of Paediatrics
21 children live in the occupied territories and we haven’t contact with And Child Health, Parow, South Africa
them.
Conclusions: Oncology is always a challenge for both parents and Background and Aims: As childhood cancer survivors (CCSs) may
children. Being a cancer patient during the war is a double test on experience psychosocial late effects, we investigated psychosocial
resilience, where adult support has an important role. The war should distress in a South African cohort.
not deny life, especially for those children who are already personally Methods: CCSs ≥18 years, treated at the Tygerberg hospital pae-
fighting for it. diatric oncology unit 1983 – 2012, completed the Brief Symptom
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ABSTRACTS S143 of S635

Inventory-18 questionnaire. Cronbach’s alpha values were 0.91 (Global validity has not been systematically assessed, which is the first step
Severity Index (GSI)), 0.85 (depression), 0.83 (somatisation) and 0.75 for MRD routine implementation in clinical practice. We assessed the
(anxiety). We compared results utilising GSI T scores of ≥50, ≥57 and prognostic capability of MRD evaluation at EOI in a B-ALL cohort in
≥63. Data collection included demographic data, socioeconomic status, Colombia.
diagnosis, treatment, and cancer-related late effects. Methods: We prospectively collected data in children (<15 years)
Results: Forty CCSs (male: female 0.54:1) with a mean age at ques- with ALL in ten Colombian cities included in VIGICANCER (Child-
tionnaire completion of 24.2 years (median 24; 18 - 51 years) were hood Cancer Surveillance System). In Colombia, MRD is performed
included. The mean follow-up period was 16.6 years; median 7 years. by multiparametric flow cytometry (FACS), following EuroFlow proto-
The majority (23/40;58%) completed school or tertiary education; cols and performed at different institutions without central review or
most were unmarried (36/40; 90%). The cancer diagnoses included validation. We assessed the performance of EOI MRD and associated
haematological malignancies (26/40; 65%) and solid tumours (14/40; event-free survival (EFS) by using Kaplan-Meier and Cox regression
35%). Treatment modalities were chemotherapy (13/40; 32.5%), methods.
chemotherapy and surgery (8/40;20%), chemo- and radiotherapy Results: During the study period (2019-2021), 871 patients with B-
(9/40;22.5%), chemotherapy, surgery, and radiotherapy (6/40; 15%), ALL were included, with median age of 5.5 years (IQR: 3,10). Fifty-two
surgery (1/40; 2.5%) and other (3/40;7.5%). Survivors had a mean num- percent were male and 9% were afro-descendants/native-Colombians.
ber of cancer-related late effects of 5.5. Using a GSI T score of ≥63 Negative MRD (<0.01%) was reported in 74% of patients, low (0.01-
identified 4/40 (10%) of survivors with psychological distress, while a 0.09%) in 19%, intermediate (1.00-4.99%) in 4% and high (≥5%) in 3%.
score of ≥57 identified 13/40 (32.5%) and ≥50 identified 18/40 (45%). MRD results were unavailable in 12% of patients. Six hundred ninety-
Only radiotherapy (p = 0.035) (odds ratio 4.6) and number of cancer- seven patients with EOI MRD contributed to the survival analysis.
related late effects per survivor (p=0.039) (odds ratio 1.89) were Twenty-four-months EFS was 89% (95%CI: 85, 92), 68% (95%CI: 56,
significantly associated with the presence of psychological distress. 77), 41% (95%CI: 18, 62), and 37% (95% CI: 11, 64) in patients with neg-
Conclusions: This South African cohort’s level of psychosocial dis- ative MRD, low MRD, intermediate MRD and high MRD, respectively
tress was at the higher end of ranges reported in the literature: 45% (P-value <0.001). Adjusted hazard rates were 2.7 (95%CI: 1.7, 4.2) for
versus 30% and 44.3% utilising a GSI T score of ≥50 and 32.5% low MRD, 6.1 (95%CI: 3.1, 12.2) for intermediate, and 3.9 (95%CI: 1.9,
versus 13-31.2% utilising a score ≥57. Significant contributing fac- 8.4) for high. Cumulative mortality at EOI was 6%.
tors were radiotherapy and the number of cancer-related late effects. Conclusions: Despite diagnosis capabilities constraints in Colombia,
Survivors with psychosocial distress on screening should be formally EOI MRD, as measured with FACS, retains its prognostic significance.
psychologically assessed. Our findings support the development of standardized treatment
strategies, that include EOI MRD evaluation for risk stratification of
B-ALL, in our country and other MIC.
O288 / #1721 END OF INDUCTION MEASURABLE RESIDUAL
DISEASE AND EARLY EVENT-FREE SURVIVAL IN B-CELL
CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA: A O289 / #44 IMPACT OF A HOLISTIC PAEDIATRIC
REAL-WORLD EXPERIENCE IN COLOMBIA ONCOLOGY NUTRITION PROGRAM: LESSONS LEARNT OVER A
DECADE
Jesus Ardila1,2 , Paula Aristizabal3,4,5 , Roberto Jaramillo1,6 , Luis
Bravo7 , Oscar Ramirez7,8,9 , On Behalf Of Vigicancer Working Group1 Maya Prasad1 , Nirmalya Roy Moulik1 , Shalini Jatia1 , Chetan Dhamne1 ,
1 Fundacion POHEMA, Research Department, Cali, Colombia; 2 Clinica Gaurav Narula2 , Badira Cheriyalinkal Parambil1 , Sripad Banavali1 ,
Imbanaco de Cali, Pediatric Oncology/hematology, Cali, Colombia; Girish Chinnaswamy1
3 University of California, San Diego, Pediatrics, La Jolla, United States of 1 Tata Memorial Centre, Paediatric Oncology, Mumbai, India; 2 Tata Memo-
America; 4 University of California, San Diego, Moores Cancer Center Pop- rial Centre, Homi Bhabha National Institute, Paediatric Oncology, Mumbai,
ulation Science, Disparities And Community Engagement, La Jolla, United India
States of America; 5 Rady Children’s Hospital San Diego, Peckham Center
For Cancer And Blood Disorders, San Diego, United States of America; Background and Aims: Management of malnutrition in children with
6 OncoDiagnóstica, Laboratorio Clínico Continental, Scientific Director, Bar- cancer remains a challenge in Low- Middle Income Countries (LMICs).
ranquilla, Colombia; 7 Cali’s Cancer Population-Based Registry, Universidad We describe our paediatric oncology nutrition program and its impact
Del Valle, Cali, Colombia; 8 Fundacion POHEMA, Scientific Direction, Cali, over the past decade.
Colombia; 9 Clinica Imbanaco de Cali, Bone Marrow Transplantation, Cali, Methods: We evaluated the impact of our nutrition program in accor-
Colombia dance with the International Society of Paediatric Oncology-Paediatric
Oncology in Developing Countries (SIOP-PODC) Nutritional Program
Background and Aims: Measurable residual disease (MRD) at the end Evaluation in the areas of service delivery (number served, increments
of induction therapy (EOI) is a strong survival predictor in childhood in delivery, number of trained care providers), patients at-risk (propor-
acute lymphoblastic leukemia (ALL). In middle-income countries (MIC), tion identified with malnutrition at diagnosis/ follow-up) and efficiency
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S144 of S635 ABSTRACTS

of nutritional interventions (proportion assessed, proportion achieved Background and Aims: To determine clinicopathologic characteristics
healthy weight, clinicians trained ). We analyzed available data for and outcomes of children with rhabdomyosarcoma (RMS) with isolated
trends between 2009 and 2020, and comparisons were made using lung metastases.
the fisher’s t test. This study was approved by our Institutional Ethics Methods: Data was analyzed for 428 patients with newly diagnosed,
Committee. metastatic RMS treated on COG studies from 1999-2013 (D9802,
Results: From 2010 to 2020, 17749 children treated at our centre D9803, ARST0431, ARST08P1). Categorical variables were compared
were beneficiaries of the nutritional program, including assessment using Chi-square or Fisher’s exact tests. Five-year event-free survival
and intervention. During this period, trained paediatric nutritionists (EFS) and overall survival (OS) were estimated using Kaplan Meier
increased from 2 to 8; SIOP-PODC level from 2 to 3-4, and nutri- method and compared between groups (lung only metastases vs. other
tion budget increased 15-fold. At diagnosis (n=5618) and 6-month sites +/- lung) using the log-rank test.
follow-up (n=2674), 59.6% and 51.2% children were undernour- Results: Compared to patients with other metastatic sites (n = 373,
ished, 34.8% and 43% well-nourished and 4.7% and 5.7% overnour- 87.1%), patients with lung-only metastases (n = 55, 12.9%) were more
ished. From 2016 onwards, fewer children were undernourished likely to be 1-9 years of age (58.2% vs. 30.8%, P < 0.0001), have embry-
at follow-up - 69.5% (2016), 60% (2018), 54% (2019) and 55% onal histology (74.6% vs. 25.7%, P < 0.0001), have N0 disease (78.2%
(2020, p<0.001). The program helped train over 500 clinicians in vs. 32.4%, P < 0.01), and have a smaller proportion of primary extrem-
nutrition. ity tumors (7.3% vs. 27.1%, P < 0.0001). Compared to patients with
Conclusions: Improved financial support and capacity building other metastatic sites, patients with lung-only metastatic disease had
have helped build and sustain an effective nutrition program. an improved 5-yr EFS (48.1% vs. 18.8%, p < 0.0001) and 5-yr OS
Priority areas include implementation of best practices, early (64.1% vs. 26.9%, p<0.0001). Patients with lung-only metastases had
nutritional intervention, continued education and locally relevant higher 5-yr EFS and 5-yr OS compared to patients with a single site
research. of metastases not involving lung (EFS: 48.1% vs. 31.2%; OS: 64.1% vs.
49.6%, P<0.0001). In patients with ERMS and lung-only metastatic dis-
ease, there was no significant difference in survival outcomes between
FREE PAPER SESSION (FPS) patients ≥10 years of age and 1-9 years of age (5-yr EFS: 58.3% vs.
68.2%, 5-yr OS: 66.7% vs. 67.7%).
FPS 22: SOFT TISSUE SARCOMA 01-10-2022 3:00 PM - 4:00 PM Conclusions: With aggressive treatment, patients with ERMS and lung-
only metastatic disease have superior 5-yr EFS and 5-yr OS regardless
O290 / #766 CHARACTERISTICS AND OUTCOMES OF of age. Consideration should be given to including patients >10 years
PATIENTS WITH RHABDOMYOSARCOMA WITH ISOLATED with ERMS and lung only metastases in the same group as those <10
LUNG METASTASES: A REPORT FROM THE SOFT TISSUE years in future risk stratification algorithms.
SARCOMA COMMITTEE OF THE CHILDREN’S ONCOLOGY
GROUP
O291 / #172 THE SIGNIFICANCE OF MARGINS IN
Juan Vasquez1 , Leo Luo2 , Susan Hiniker3 , Daniel Rhee4 , Roshni PEDIATRIC NON-RHABDOMYOSARCOMA SOFT TISSUE
Dasgupta5 , Sonja Chen6 , Brenda Weigel7 , Wei Xue8 , Rajkumar SARCOMAS: CONSENSUS FROM THE INTERNATIONAL SOFT
Venkatramani9 , Carola Arndt10 TISSUE SARCOMA CONSORTIUM (INSTRUCT)
1 Yale School of Medicine, Pediatric Hematology/oncology, New Haven,
United States of America; 2 Vanderbilt-Ingram Cancer Center, Department Monika Sparber-Sauer1 , Andrea Ferrari2 , Sheri Spunt3 , Christian
Of Radiation Oncology, Nashville, United States of America; 3 Stanford Uni- Vokuhl4 , Dana Casey5 , Timothy Lautz6 , William Meyer7 , David
versity School of Medicine, Department Of Radiation Oncology, Stanford, Walterhouse8 , Kristian Pajtler9 , Rita Alaggio10 , Andreas Schmidt11 ,
United States of America; 4 Johns Hopkins University School of Medicine, Akmal Safwat12 , Beate Timmermann13 , Patrizia Dall’Igna14 , Sonja
Department Of Surgery, Baltimore, United States of America; 5 Cincinnati Chen15 , Aaron Weiss16 , Daniel Orbach17
Children’s Hospital Medical Center, University of Cincinnati, Division Of 1 Medizinische Fakultät der Universität Tübingen, Pädiatrische Onkologie,
Pediatric General And Thoracic Surgery, Cincinnati, United States of Amer- Stuttgart, Germany; 2 Fondazione IRSCC Istituto Nazionale Tumor, Pedi-
ica; 6 Nationwide Children‘s Hospital, Department Of Pathology And Lab- atric Oncology Unit, Milano, Italy; 3 Stanford University School of Medicine,
oratory Medicine, Columbus, United States of America; 7 University of Palo Alto, Department Of Pediatrics, Stanford, United States of America;
Minnesota, Division Of Pediatric Hematology/oncology, Minneapolis, United 4 University Hospital Bonn, Section Of Pediatric Pathology, Department
States of America; 8 University of Florida, Department Of Biostatistics, Of Pathology, Bonn, Germany; 5 University of North Carolina School of
Gainesville, United States of America; 9 Baylor College of Medicine, Texas Medicine, Department Of Radiation Oncology, Chapel Hill, United States
Children’s Cancer Center, Department Of Pediatrics, Houston, United States of America; 6 Northwestern University Feinberg School of Medicine, Depart-
of America; 10 Mayo Clinic, Department Of Pediatrics, Rochester, United ment Of Surgery, Chicago, United States of America; 7 University of Okla-
States of America homa, College of Medicine, Pediatric Hematology Oncology, Chicago, United
States of America; 8 Northwestern Memorial Hospital Chicago, Pediatric
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ABSTRACTS S145 of S635

Hematology And Oncology, Chicago, United States of America; 9 Heidelberg Jonathan Metts1 , Wei Xue2 , Zhengya Gao2 , Ralph Ermoian3 , Julie
University Hospital, Pediatric Hematology And Oncology, Heidelberg, Ger- Bradley4 , Michael Arnold5 , Roshni Dasgupta6 , Rajkumar
many; 10 Bambino Gesu Children‘s Hospital, Patholgy Unit, Roma, Italy; Venkatramani7 , David Walterhouse8
11 University Children’s Hospital Eberhard Karls University Tübingen, Pedi- 1 Johns Hopkins All Children’s Hospital, Cancer And Blood Disorders Insti-
atric Surgery And Pediatric Urology, Tübingen, Germany; 12 Aarhus Uni- tute, St Petersburg, United States of America; 2 University of Florida, Depart-
versity Hospital, Danish Center For Particle Teherapy, Aarhus, Denmark; ment Of Biostatistics, Gainesville, United States of America; 3 University
13 University Hospital Essen, Department Of Particle Therapy, Essen, Ger- of Washington, Radiation Oncology, Seattle, United States of America;
many; 14 University of Bari, Department Of Emergencies And Organ Trans- 4 University of Florida, Radiation Oncology, Jacksonville, United States of
plantation, Bari, Italy; 15 Nationwide Children‘s Hospital, Department Of America; 5 University of Colorado, Pathology And Laboratory Medicine,
Pathology And Laboratory Medicine, Columbus, United States of America; Aurora, United States of America; 6 Cincinnati Children’s Hospital Med-
16 Maine Medical Center, Department Of Pediatrics, Maine, United States of ical Center, University of Cincinnati, Division Of Pediatric General And
America; 17 Institut Curie, Siredo Oncology Department, Paris, France Thoracic Surgery, Cincinnati, United States of America; 7 Baylor College
of Medicine, Texas Children’s Cancer Center, Department Of Pediatrics,
Background and Aims: The International Soft Tissue Sarcoma Houston, United States of America; 8 Northwestern Memorial Hospital
Database Consortium (INSTRuCT) is a collaboration of the Children’s Chicago, Pediatric Hematology And Oncology, Chicago, United States of
Oncology Group (COG), European pediatric Soft Tissue sarcoma Study America
Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe
(CWS) Soft Tissue Sarcoma Committees devoted to improving patient Background and Aims: Orbital Rhabdomyosarcoma (ORMS) com-
outcomes. The assessment and definition of margin status in child- monly presents as low-risk (localized embryonal) disease with excel-
hood, adolescent and young adult non-rhabdomyosarcoma soft tissue lent 3-year outcomes reported by the Children’s Oncology Group
sarcoma (NRSTS) is controversial. (COG). Long-term follow-up of low-risk ORMS and outcomes of
Methods: The INSTRuCT committee aimed to develop international less-common subgroups of ORMS on COG trials have not been
consensus recommendations around surgical margin assessment and reported.
definition in NRSTS. Methods: Patients with ORMS were identified on seven COG tri-
Results: Clinical staging for NRSTS should be established through als. Demographic information and disease characteristics were col-
multi-disciplinary input from oncologists, surgeons, pathologists, and lected. Survival was determined for the following subgroups: 1)
radiation oncologists and include post-operative assessment using the low-risk ORMS on D9602 and ARST0331, 2) resected (Group I/II)
The R grouping system. R0 stage should only be assigned when a com- low-risk ORMS on D9602 and ARST0331 (including Group IIA who
plete en-bloc resection of the tumor has been accomplished without had a radiation dose reduction from 41.4 to 36 Gy beginning
tumor rupture. Patients who undergo complete primary re-excision with D9602), 3) non-low-risk ORMS on D9802, D9803, ARST0431,
(PRE) <6 weeks following an initial incomplete primary resection are ARST0531, and ARST08P1, and 4) recurrent disease in low-risk
classified as R0(ir) stage. Authors propose to arbitrarily define “R0 mar- patients. Event-free Survival (EFS) and Overall Survival (OS) were esti-
gin” by the absence of tumor cells < 1 mm from the closest margin in any mated by the Kaplan-Meier method and reported with 95% confidence
direction. The R0(ab) sub-classification includes a surgical margin that intervals.
is an anatomic site boundary. If the margin is < 5 mm (close margin), Results: We identified 218 patients with ORMS: the majority were
the tumor should be classified as R1(c). If tumor necrosis is present at male (n=129, 59.17%) and age 1-9 years (n=159, 72.94%). Most
the margin, this can be classified as R1(n). Tumors should be classified tumors were embryonal/botryoid (n=169, 77.52%), under 5 cm
as R1(x) when margins cannot be defined in mm or are unknown. The (n=213, 97.71%), Group III (n=170, 77.98%), and without regional
R2(a/b/c) sub-classification of gross residual tumor differentiates unre- lymph node involvement (n=217, 99.5%). Thirteen of 26 tested were
sected tumor (a), unresectable tumor (b), and unknown/unspecified FOXO1 fusion-negative. For 192 patients on D9602 and ARST0331,
(c). 10-year EFS and OS were 85.49% (76.96%-94.02%) and 95.62%
Conclusions: This consensus aims to establish an evidence-based (90.75%-100%), respectively. Of these, 44 patients with resected low-
framework to be used for common guidelines for future protocols. A risk ORMS (5 Group I, 39 Group IIA) had 10-year EFS and OS of 88.01%
standardized pathology report template, such as that provided by the (72.59%-100%) and 97.62% (90%-100%), respectively. Twenty-six
College of American Pathologists (CAP), is recommended that includes patients with non-low-risk ORMS had 5-year EFS and OS of 88.46%
the distance from the tumor to the inked margins in mm. (75.61%-100%) and 95.83% (87.66%-100%), respectively. Of low-risk
patients with recurrent disease, the 5-year OS from time of recurrence
was 75% (50.5%-99.5%) for 12 patients on ARST0331 and 63.46%
O292 / #61 LONG-TERM OUTCOMES OF PATIENTS WITH (29.84%-97.09%) for 13 patients on D9602.
ORBITAL RHABDOMYOSARCOMA TREATED ON CHILDREN’S Conclusions: Our results support a long-term favorable prognosis for
ONCOLOGY GROUP TRIALS patients with ORMS, including those with non-low-risk disease, and a
significant proportion of patients with recurrent ORMS may achieve
long-term survival.
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S146 of S635 ABSTRACTS

O293 / #847 PROGNOSTIC FACTORS AND TREATMENT IN Conclusions: Local control is mandatory for long-term survival in RD
RELAPSE OF PRIMARY LOCALIZED RMS RMS. Second line CHT with ACCTTIVE is comparable to VIT in HR
patients.
Amadeus Heinz1 , Martin Ebinger1 , Joerg Fuchs2 , Beate
Timmermann3 , Guido Seitz4 , Christian Vokuhl5 , Marc Muenter6 ,
Kristian Pajtler7 , Sabine Stegmaier8 , Thekla Von Kalle9 , Christian FREE PAPER SESSION (FPS)
Kratz10 , Jochen Rößler11 , Gustaf Ljungman12 , Anton Schoenstein13 ,
Monika Sparber-Sauer14 FPS 23: STEM CELL TRANSPLANTATION 01-10-2022 3:00 PM -
1 University Children’s Hospital Tuebingen, Hematology / Oncology, Tübin- 4:00 PM
gen, Germany; 2 University Children’s Hospital - UKT Tuebingen, Paediatric
Surgery And Paediatric Urology, Tuebingen, Germany; 3 University Hospi- O294 / #265 INDIVIDUALISED DOSING OF
tal Essen, Department Of Particle Therapy, West German Proton Therapy ANTI-THYMOCYTE GLOBULIN IN PAEDIATRIC UNRELATED
Centre Essen (wpe), West German Caner Center (wtz), Essen, Germany; ALLOGENEIC STEM CELL TRANSPLANTATION (PARACHUTE): A
4 University Hospital Giessen-Marburg, Campus Marburg, Department Of SINGLE-ARM PHASE II CLINICAL TRIAL
Pediatric Surgery And Urology, Marburg, Germany; 5 University Hospital
Bonn, Section Of Pediatric Pathology, Department Of Pathology, Bonn, Ger- Rick Admiraal1 , Stefan Nierkens1 , Marc Bierings1 , Robbert Bredius2 ,
many; 6 Klinikum Stuttgart, Radiotherapy, Stuttgart, Germany; 7 Heidelberg Ineke Van Vliet3 , Yilin Jiang4 , Marta Lopez-Yurda4 , A. Birgitta
University Hospital, Pediatric Hematology And Oncology, Heidelberg, Ger- Versluijs1 , C. Michel Zwaan1 , Caroline Lindemans1 , Jaap Jan Boelens1
many; 8 Klinikum Stuttgart, Molecular Pathology, Stuttgart, Germany; 1 Princess Maxima Center for Pediatric Oncology, Stem Cell Transplantation

9 Klinikum Stuttgart, Radiology, Stuttgart, Germany; 10 Hannover Medical Unit, Utrecht, Netherlands; 2 Leiden University Medical Center, Pediatrics, d,
School, Pediatric Hematology & Oncology, Hannover, Germany; 11 Inselspital Netherlands; 3 Erasmus Medical Center, Pediatric Oncology, d, Netherlands;
Bern, Pediatrics, Zurich, Switzerland; 12 Uppsala Universitet, Women’s And 4 Princess Maxima Center for Pediatric Oncology, Statistics, d, Netherlands

Children’s Health, UPPSALA, Sweden; 13 University of Mannheim, Faculty


Of Social Science, Mannheim, Germany; 14 Klinikum Stuttgart Olgahospital, Background and Aims: Anti-thymocyte globulin (ATG), used in the
Pediatrics 5 (hematology, Oncology, Immunology), Stuttgart, Germany conditioning of haematopoietic stem-cell transplantation (HSCT) to
prevent graft-versus-host-disease (GVHD) and graft failure, has highly
Background and Aims: Outcome of patients with relapsed disease of variable pharmacokinetics. Overexposure to ATG leads to poor CD4+
localized rhabdomyosarcoma (RD RMS) remain poor despite intensive, T-cell immune reconstitution, which is associated with inferior overall
multimodal treatment. The VIT (vincristine, irinotecan, temozolomide) survival. We hypothesised that individualised ATG-dosing would pro-
combination is considered the new european standard treatment in mote CD4+ immune reconstitution, while still preventing GVHD and
patients with RD RMS after alkylating agent containing regimen in graft failure.
primary disease. Methods: We report the results of a prospective, single-arm, phase 2
Methods: All patients with RD RMS registered in the European clinical trial done at the Princess Máxima Center for Pediatric Oncol-
soft tissue sarcoma registry of the Cooperative Weichteilsarkom ogy to investigate individualised dosing of ATG for allogeneic HSCT
Studiengruppe (n=68; 2009–2018) were retrospectively ana- in paediatric patients. ATG globulin dosing was based on bodyweight,
lyzed regarding characteristics of relapse, treatment and outcome absolute lymphocyte counts, and the stem-cell source, with cumulative
data. doses ranging from 2–10 mg/kg. Patients younger than 18 years receiv-
Results: Patients had a relapse after first-line stratification in the ing a first HSCT with a T-cell repleted graft for any indication were
low/standard risk (SR) group (n=2/16), high risk (HR) group (n=41) eligible for inclusion. Primary endpoint was CD4+ immune reconstitu-
and very high risk (VHR) group (n=9) with the diagnosis of embry- tion (>0⋅05 × 109 CD4+ T-cells per L twice within 100 days [±3] after
onal RMS (n=34), alveolar RMS (fusion positive, n=29) and spindle-cell transplantation). The primary endpoint needed to be met in 38 of 53
RMS (n=5). Secondary treatment regimen consisted of adriamycin, evaluable patients (no death, relapse, or graft failure before day 100).
carboplatin, cyclophsophamide, topotecan, trofosfamide, ifosfamide, Results: Between 2015-2018, 51 evaluable patients were included in
vincristine, etoposide (ACCTTIVE, n=36), topotecan, etoposide, carbo- the study. Median follow-up was 25⋅6 months (IQR 15⋅0–37⋅0) and
platin, cyclophosphamide (TECC, n=12) or other (n=12). Resection was median age was 7⋅4 years (IQR 2⋅8–13⋅2). 29 (50%) of 58 patients
performed in 40/68 (59%) patients and/or radiation in 47/68 (69%). Ini- were female. CD4+ immune reconstitution was reached in 41 (80%,
tial risk stratification, pattern of relapse, time to relapse, best surgery ± 95% CI 67–90, in survival analysis) of 51 evaluable patients, hence
radiotherapy and achievement of 2nd complete remission were signifi- the study met its primary endpoint. There was no difference in CD4+
cant prognostic factors. The regimen ACCTTIVE was superior to TECC immune reconstitution between patients who received different stem-
in the whole cohort, but not when adjusted for initial risk stratification. cell sources (87% [95% CI 61–96] in cord blood, 77% [54–89] in bone
Overall 5-year EFS and OS was 26% and 31%, respectively. Patients marrow [p=0⋅62]).
with RD of SR group had a 5-year OS of 80%, HR patients of 20% and Conclusions: Individualised dosing of ATG led to a significant improve-
VHR patients with 13% (p=0.008). ment in early CD4+ immune reconstitution without increasing GVHD
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S147 of S635

and graft failure incidence. Promotion of early CD4+ immune reconsti- CTLs, 15 ADV CTLs, 5 BK CTLs, and 2 EBV vCTLs. Of the evaluable
tution by individualising anti-thymocyte globulin dose might improve patients, responses were 17-CR, five-PR, three-SD, and one-PD with
outcomes of allogeneic HSCT. an ORR 85%. Two of the patients developed grade II-III aGVHD of the
skin. There was no extensive cGVHD, infusion reactions, or CRS related
to vCTLs.
O295 / #998 HAPLOIDENTICAL VIRAL SPECIFIC CYTOTOXIC Conclusions: The manufacturing of vCTLS using the CliniMACS
T-LYMPHOCYTES (VCTLS) FOR THE TREATMENT OF Prodigy for patients with PID or following SOT or alloHSCT is effi-
REFRACTORY VIRAL INFECTIONS IN PRIMARY cient and the treatment appears safe and effective. Supported by FDA
IMMUNODEFICIENCY (PID), SOLID ORGAN (SOT) OR R01006363.
ALLOGENEIC BMT PATIENTS

Julie Talano1 , Jordan Milner2 , Lauren Harrison2 , Janet Ayello2 , Allyson O296 / #1496 AUTOLOGOUS STEM CELL MOBILISATION
Flower3 , Yaya Chu4 , Bryon Johnson5 , Yimei Li6 , Dean Lee7 , Julia Chu8 , AND -TRANSPLANTATION IN PAEDIATRIC ONCOLOGY; POOR
Christopher Dvorak8 , Lynn O’Donnell9 , Yongping Wang10 , Nancy MOBILIZERS, PLERIXAFOR AND PEGFILGRASTIM
Bunin11 , Mitchell Cairo2
1 Medical College of Wisconsin, Pediatrics Hematology Oncology, Milwau- Caroline Hochheuser1,2 , Maria Rozeman1 , Nina Kunze2 , Nina
kee, United States of America; 2 New York Medical College, Pediatrics, Gelineau1,3 , Carlijn Kuijk2 , Antoinette Jaspers-Bakkers1 , Cor Van Den
Valhalla, United States of America; 3 MSKCC, Pediatrics, NY, United States of Bos4 , Tirza Slager5 , Jozsef Zsiros1 , Wim Tissing6,7 , Kasper Westinga8 ,
America; 4 NYMC, Pediatrics, Valhalla, United States of America; 5 Medical C. Michel Zwaan4 , Carlijn Voermans2 , Lieve Tytgat1 , K.C.J.M Kraal1 ,
College of Wisconsin, Cell Therapy Laboratory, Milwaukee, United States Ilse Timmerman1,2
of America; 6 University of Penn Medicine, Statistics, Philadelphia, United 1 Prinses Maxima Centrum, Solid Tumors, Utrecht, Netherlands; 2 Sanquin
States of America; 7 Nationwide Children’s Hospital, Center For Childhood Research and Landsteiner Laboratory, University of Amsterdam, Depart-
Cancer And Blood Diseases, Columbus, United States of America; 8 USCF, ment Of Hematopoiesis, Amsterdam, Netherlands; 3 Sanquin Research and
Pediatrics Hematology Oncology, San Francisco, United States of America; Landsteiner Laboratory, Academic Medical Centre Amsterdam, University of
9 Ohio State University, Cell Therapy Laboratory, Columbus, United States of Amsterdam, Department Of Experimental Immunohematology, Amsterdam,
America; 10 Children’s Hospital of Philadelphia, Cell Therapy, Philadelphia, Netherlands; 4 Prinses Maxima Center for Pediatric Oncology, Haematoon-
United States of America; 11 Children’s Hospital of Philadelphia, Pediatrics, cology, Utrecht, Netherlands; 5 Erasmus MC, General Practice, Rotterdam,
Philadelphia, United States of America Netherlands; 6 Princess Maxima Center for Pediatric Oncology, Supportive
Care, Utrecht, Netherlands; 7 University Medical Center Groningen, Depart-
Background and Aims: Viral infections cause significant mortality in ment Of Pediatric Oncology/hematology, Groningen, Netherlands; 8 Cell
patients with immunodeficiencies. Due to limitations of antiviral ther- Therapy Facility, University Medical Center Utrecht, Department Of Clinical
apies, adoptive immune therapy has been developed to provide T-cell Pharmacy, Utrecht, Netherlands
immunity. Ex-vivo production of vCTLs is limited by time and quantities
for infusion. Miltenyi Biotec developed the CliniMACS Prodigy which Background and Aims: Paediatric cancer patients receive autologous
isolates virus specific CD4+/CD8+ T cells from donors after incubation hematopoietic stem cell transplantation as part of normal treat-
with viral antigen peptides. The Multicenter Viral CTL Consortium was ment routine. When patients fail to mobilise a sufficient number of
created to treat immunocompromised patients. To demonstrate vCTLs CD34+ cells, alternative mobilisation agents such as plerixafor are
manufactured using the CliniMACS Prodigy for CMV, EBV, AdV, and used. Single injection of pegylated G-CSF (Peg-G-CSF) is used for
BK virus is safe and effective for immunocompromised patients with improved patient comfort. While the efficacy and safety of these is well
refractory viral infections. established in adults, limited data for its use in paediatric patients are
Methods: Patients 0.1-30 years with PID or following SOT or alloHSCT available. We aim to study (i) success of mobilisation, (ii) hematologic
with refractory viral illnesses were eligible. Unmobilized PBMCs were reconstitution and (iii) stem cell quality and -phenotype, compar-
obtained via apheresis. vCTLs were manufactured using the Clini- ing good (GM) and poor mobilisers (PM) and different mobilisation
MACS® Prodigy. HLA mismatched related donor cells were infused regimens.
at 0.5x104 CD3/kg per dose. Infusions of vCTLs Q2 weeks based on Methods: In this multi-centre retrospective study we analysed data
response and toxicity (maximum of 5 infusions). CR=PCR negative and of 281 non-leukemic paediatric patients. For a subgroup of patients
PR=PCR ≥1 log decrease. (n=36), mobilised CD34+ cells were studied by flow cytometry for
Results: Thirty-three patients met eligibility with two PID and 31 expression of homing- and lineage markers. Moreover, apheresis prod-
alloHSCT/SOT. Donors were screened. Four patients became ineligi- ucts of patients with neuroblastoma were tested for tumour cell
ble; n=1 due to death from a viral infection, n=1 parental refusal and presence by qPCR.
n=2 subjects with decreasing viral titers. Seventeen patients received Results: Use of Peg-G-CSF increased CD34+ cell yield 2.6-fold com-
vCTLs from their original haplo BMT donor and 12 received third party pared to daily G-CSF (p<0.001). The addition of plerixafor to G-CSF
haplo-related donor vCTL infusions. Seven patients received CMV was successful (i.e. harvest yield >2*106 CD34+cells/kg) in 75% of PM,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S148 of S635 ABSTRACTS

showing a 2.2-fold increase in CD34+ cells compared to G-CSF only Methods: This post-marketing registry study collected retrospec-
(p=0.005). No significant effects of Peg-G-CSF or plerixafor on hema- tive/prospective real-world data on defibrotide-treated patients from
tologic reconstitution were observed. Within the PM group, plerixafor 53 French HCT centres. VOD/SOS diagnosis was per investigator
led to an increase of CXCR4+ and primitive CD34+ cells in the aphere- discretion using standard criteria. Disease severity was retrospec-
sis product, but the levels did not exceed those of GM. Lineage marker tively/prospectively categorised using paediatric EBMT severity crite-
expression and proliferation rate of CD34+ cells in the graft remained ria. Outcomes included Day 100 survival and complete response (CR;
unaltered. Tumor cells were not mobilized by plerixafor. total serum bilirubin <2 mg/dL and multiorgan failure resolution) and
Conclusions: Our study provides unique data about Peg-G-CSF and treatment-emergent serious adverse events (TESAEs) of interest.
plerixafor in paediatric patients, not only in regards to mobilisation effi- Results: Of 87 paediatric patients in the defibrotide-treated post-
ciency, but also hematologic reconstitution, stem cell phenotype and HCT population, 25 (29%) had neuroblastoma and received auto-HCT.
-quality. We recommend the use of Peg-G-CSF over G-CSF because Median age was 3.7 (range: 1, 16) years. All patients underwent
of superior harvest yields and less patient discomfort. Plerixafor is myeloablative conditioning, 2 (8%) had prior HCT, and 3 (12%) had
considered safe and effective in paediatric patients. advanced disease. Median time from auto-HCT to VOD/SOS diagno-
sis was 17.0 (range: 6, 28) days. At diagnosis, 19 (76%) patients had
ascites and 20 (80%) had refractory thrombocytopaenia; 17 (68%)
O297 / #716 VENO-OCCLUSIVE DISEASE/SINUSOIDAL patients presented with anicteric VOD/SOS. Eight (32%) patients had
OBSTRUCTION SYNDROME (VOD/SOS) AFTER AUTOLOGOUS mild/moderate VOD/SOS, 7 (28%) had severe, and 10 (40%) had very
HAEMATOPOIETIC CELL TRANSPLANTATION (HCT): severe. By Day 100 post-HCT, the proportion of patients with CR was
OUTCOMES OF DEFIBROTIDE-TREATED PAEDIATRIC PATIENTS 92% (95% CI: 74, 99) and KM-estimated survival was 92% (95% CI: 72,
WITH NEUROBLASTOMA FROM THE DEFIFRANCE STUDY 98). Five (20%) patients had TESAEs of interest, including 4 (16%) with
haemorrhage and 1 (4%) with infection.
Christelle Dufour1 , Marion Gambart2 , Virginie Gandemer3 , Lauriane Conclusions: In this post hoc analysis of paediatric patients with neu-
Lemelle4 , Arnaud Petit5 , Dominique Plantaz6 , Cécile Pochon7 , Anne roblastoma who received defibrotide treatment for VOD/SOS after
Sirvent8 , Kobby Asubonteng9 , Deborah Gutierrez10 , Mohamad auto-HCT, Day 100 post-HCT CR and survival were >90% with no
Mohty11 , Jean-Hugues Dalle12 new safety signals, despite a predominance of severe/very severe
1 Gustave Roussy Cancer, Department Of Paediatric And Adolescent Oncol- VOD/SOS. These data provide real-world evidence supporting defi-
ogy, Villejuif, France; 2 CHU de Toulouse, Hôpital des Enfants, Department brotide treatment of VOD/SOS in this high-risk population.
Of Paediatrics – Haematology, Immunology And Oncology, Toulouse, France;
3 University Hospital of Rennes, Department Of Paediatric Haematology-
oncology, Rennes, France; 4 SIREDO Oncology Center (Care, Innovation and FREE PAPER SESSION (FPS)
Research for Children and AYA with Cancer) Institut Curie, Department Of
Paediatric Oncology, Paris, France; 5 Hôpital Armand Trousseau, APHP Sor- FPS 24: RARE TUMORS 01-10-2022 3:00 PM - 4:00 PM
bonne Université, Department Of Paediatric Haematology And Oncology,
Paris, France; 6 CHU Grenoble Alpes, Hôpital Couple Enfant, Department O298 / #441 PRIMARY LUNG CARCINOMA IN CHILDREN
Of Paediatric Immuno-haematology-oncology, Grenoble, France; 7 Hôpital AND ADOLESCENTS: AN ANALYSIS OF THE EUROPEAN
d’Enfants, Department Of Paediatric Haematology-oncology, Nancy, France; COOPERATIVE STUDY GROUP FOR PEDIATRIC RARE TUMORS
8 Hôpital Arnaud de Villeneuve, Department Of Paediatric Haematology- (EXPERT)
oncology, Montpellier, France; 9 Jazz Pharmaceuticals, Department Of Bio-
metrics, Philadelphia, United States of America; 10 Jazz Pharmaceuticals, Michael Abele1 , Calogero Virgone2 , Fiona Wright3 , Dominik
Medical Department, Lyon, France; 11 Hôpital St Antoine, Université Sor- Schneider4 , Coralie Mallebranche5 , Maja Česen Mazič6 , Dragana
bonne, Department Of Clinical Haematology And Cellular Therapy, Inserm Janic7 , Gabriela Guillén8 , Viera Bajčiová9 , Malgorzata Krawczyk10 ,
Umrs 938, Paris, France; 12 Hôpital Robert-Debré, GHU APHP Nord et Uni- Ewa Bień10 , Jelena Roganovic11 , Gianni Bisogno12 , Andrea Ferrari13 ,
versité de Paris, Department Of Paediatric Immuno-haematology, Paris, Daniel Orbach14 , Yves Reguerre15 , Ines Brecht1
France 1 University Hospital Tuebingen, Department Of Pediatrics, Pediatric Hema-

tology & Oncology, Tuebingen, Germany; 2 University of Padua, Pediatric


Background and Aims: VOD/SOS, a potentially fatal complication Surgery, Department Of Women’s And Children’s Health, Padua, Italy;
of HCT, primarily occurs after high-dose alkylating-based condition- 3 University of Cambridge; Wellcome Sanger Institute, Department Of
ing regimens (eg, busulfan-melphalan, thiotepa) followed by allogeneic Pediatrics, Cambridge, United Kingdom; 4 Klinikum Dortmund, University
HCT or autologous HCT (auto-HCT). Auto-HCT is part of standard- Witten/Herdecke, Clinic Of Pediatrics, Dortmund, Germany; 5 CHU Angers,
of-care treatment for patients with high-risk neuroblastoma. This Pediatric Immuno-hemato-oncology Unit, Angers, France; 6 University Hos-
post hoc analysis presents outcomes in paediatric patients with neu- pital Ljubljana, Department Of Pediatrics, Ljubljana, Slovenia; 7 Institute for
roblastoma who received defibrotide treatment for VOD/SOS after Oncology and Radiology of Serbia, Pediatric Oncology, Belgrade, Serbia;
auto-HCT. 8 Hospital Infantil Universitari Vall d’Hebron, Pediatric Surgery Department,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S149 of S635

Barcelona, Spain; 9 Childrens University Hospital, Brno, Department Of Pedi- Gastroenterology, Hepatology, And Nutrition, Philadelphia, United States of
atric Oncology, Brno, Czech Republic; 10 Medical University of Gdansk, America; 4 University of Iowa, Surgery, Iowa City, United States of America
Department Of Pediatrics, Hematology And Oncology, Gdansk, Poland;
11 University of Rijeka, Department Of Pediatrics, Clinical Hospital Cen- Background and Aims: Juvenile Polyposis Syndrome (JPS) is a can-
ter Rijeka, Rijeka, Croatia; 12 University of Padua, Hematology/oncology cer predisposition syndrome characterized in some cases by germline
Division, Department Of Women’s And Children’s Health, Padua, Italy; mutations in BMPR1A or SMAD4. However, in 40-60% of patients the
13 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Oncology Unit, germline driver is unknown. Next-generation sequencing of a cohort
Milano, Italy; 14 Institut Curie, Siredo Oncology Department, Paris, France; of JPS patients identified bone morphogenetic protein receptor 2
15 Félix Guyon University Hospital, Department Of Pediatric Hematology (BMPR2) as a candidate driver in one mutation-negative JPS patient
And Oncology, St Denis, Réunion Island, France with high polyp burden.
Methods: Under an IRB-approved protocol, patient-derived, three-
Background and Aims: Primary lung carcinomas are very rare child- dimensional colonoids were established from adjacent colon and polyp
hood tumors with an incidence of <2/1,000,000 per year as defined tissue of this individual, as well as an individual with a BMPR1A dele-
by the European Cooperative Study Group for Pediatric Rare Tumors tion. Proliferation and metabolic activity of each organoid line were
(EXPeRT). They represent a challenge to treating physicians as there measured by Ki67 staining, EdU labelling, and cell titer glo (CTG) assay
are few reports on these diseases at this age and no specific recom- alongside age- and sex-matched controls. Relative RNA and protein
mendations are available. This collaborative analysis of the EXPeRT expression levels of known drivers and downstream effectors were
group was conducted to improve knowledge about the treatment and examined by RT-qPCR and Western blot, respectively.
prognosis of primary lung carcinomas in pediatric patients. Results: Distinct phenotypic differences are evident between
Methods: A retrospective European analysis of all pediatric patients (0- colonoids from an individual with a BMPR1A deletion and BMPR2
18 years) with primary lung carcinomas prospectively collected in the variant colonoids, such as increased crypt budding and Ki67 staining.
EXPeRT databases between 2000 and 2021 was performed. Clinical CTG assay data indicate higher metabolic activity in BMPR2 variant
data including outcomes were analyzed. colonoids in comparison to normal controls (p= 0.0104). Through
Results: Thirty-eight patients were identified with a median age of 12.8 Western blot analysis, BMPR2 variant polyp colonoids, specifically,
years at diagnosis (range 0 to 17 years). Mucoepidermoid carcinoma show decreased SMAD4 expression and phosphorylation as well as
(MEC) was the most common entity (n=20), followed by adenocarci- decreased BMPR1A expression. Downregulation of BMPR1A and
noma (n=12), squamous cell carcinoma (SCC; n=4), adenosquamous SMAD4 expression is also seen by RT-qPCR.
carcinoma (n=1) and small-cell lung cancer (n=1). Lymph node metas- Conclusions: These data demonstrate that clear phenotypic differ-
tases occurred rarely in patients with MEC (2 cases), and 19 patients ences exist between colonoid lines established from normal controls,
achieved durable remission after surgical resection only. One patient individuals with known JPS predisposition genes, and a novel candi-
with MEC died after progression of metastasis. Patients with histology date driver of disease, BMPR2. Proliferation and protein expression
other than MEC often presented with advanced disease (stage IV in 14 data suggest that both known and candidate genotypes are consistent
of 18 cases) and needed multimodality treatment. They had a combined with a hyperproliferative phenotype. In vitro data support BMPR2 as a
survival rate of 44%. While all patients with SCC died, the 12 patients candidate germline driver of the JPS phenotype, and additional study is
with adenocarcinoma had a survival rate of 50%. ongoing regarding downstream pathway regulation in colonoids with a
Conclusions: Primary lung carcinoma occur rarely in children. While BMPR2 variant.
patients with MEC have a favorable outcome with a survival rate of
95%, patients with other lung carcinoma entities have an unfavorable
outcome despite multimodality treatment strategies. This analysis will O300 / #138 NUT CARCINOMA IN CHILDREN AND
help propose consensus guidelines for diagnosis and therapy. ADOLESCENTS: THE EXPERT EUROPEAN STANDARD CLINICAL
PRACTICE HARMONIZED RECOMMENDATIONS

O299 / #1792 BONE MORPHOGENETIC PROTEIN RECEPTOR Lauriane Lemelle1 , Tim Flaadt2 , Brice Fresneau3 , Antoine
2 (BMPR2) AS A POTENTIAL GERMLINE DRIVER IN JUVENILE Moya-Plana4 , Beate Timmermann5 , Jelena Roganovic6 , Andrea
POLYPOSIS SYNDROME (JPS) Ferrari7 , Giulia Fichera8 , Ulrich Lauer9 , Tal Ben-Ami10 , Dominik
Schneider11 , Christian Vokuhl12 , Stephanie Bolle13 , Elisabeth Fox14 ,
Bridgid Greed1 , Michael Xie1 , Garrett Brodeur1 , Zvi Cramer2 , Maiah Steven Dubois15 , Carlos Rodriguez-Galindo16 , Gianni Bisogno17 ,
Dent1 , Melani Duvall1 , Katherine Hamilton3 , James Howe4 , Tatiana Aurore Surun1 , Ines Brecht18 , Daniel Orbach1
Karakasheva3 , Petar Mamula3 , Christopher Lengner2 , Suzanne 1 Institut Curie, Siredo Oncology Department, Paris, France; 2 University
Macfarland1 Hospital Tübingen, Institute Of Pathology And Neuropathology, Tubingen,
1 Children’s Hospital of Philadelphia, Pediatric Oncology, Philadelphia, Germany; 3 Gustave Roussy, Department Of Pediatric Oncology, Université
United States of America; 2 University of Pennsylvania, Biomedical Sciences, Paris-saclay, Villejuif, France; 4 Gustave Roussy, Department Of Pediatric
Philadelphia, United States of America; 3 Children’s Hospital of Philadelphia, Oncology, Villejuif, France; 5 University Hospital Essen, Department Of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S150 of S635 ABSTRACTS

Particle Therapy, Essen, Germany; 6 University of Rijeka, Department Of Conclusions: This project leads to a consensus strategy based on
Pediatrics, Clinical Hospital Center Rijeka, Rijeka, Croatia; 7 Fondazione international experience with this very rare disease.
IRCCS Istituto Nazionale dei Tumori, Pediatric Oncology Unit, Milano, Italy;
8 University Hospital of Padua, Pediatric Radiology, Padua, Italy; 9 University
E-POSTER VIEWING ABSTRACTS
of Tuebingen, Department Of Internal Medicine Viii, Medical Oncology
And Pneumology, Tubingen, Germany; 10 Kaplan Medical Center, Pediatric
E-Poster Topic: AS01 Surgery - IPSO
Hematology Unit, Rehovot, Israel; 11 Dortmund Municipal Hospital, Clinic
Of Pediatrics, Dortmund, Germany; 12 University Hospital Bonn, Section Of
E-POSTER VIEWING
Pediatric Pathology, Department Of Pathology, Bonn, Germany; 13 Gustave
Roussy, Université Paris-Saclay, Department Of Radiation Oncology, Ville-
EP001 / #1405 SAFETY AND TOLERABILITY OF ANESTHETIC
juif, France; 14 St. Jude, Children’s Research Hospital, Memphis, United
MANAGEMENT OF SUPERSELECTIVE OPHTALMIC ARTERIAL
States of America; 15 Dana-Farber hospital, Children’s Cancer And Blood
CHEMOTHERAPY FOR RETINOBLASTOMA IN CHILDREN
Disorders Center, Boston, United States of America; 16 St Jude Children’s
Research Hospital, Global Pediatric Medicine, Memphis, United States of
Leonid Martynov, Nune Matinyan, Tatiana Ushakova, Vladimir
America; 17 University of Padua, Hematology/oncology Division, Depart-
Polyakov
ment Of Women’s And Children’s Health, Padua, Italy; 18 Children’s Hospital,
Federal State Budgetary Institution National Medical Research Center of
Eberhard-Karls-Universität Tübingen, Pediatric Hematology And Oncology,
Oncology named after N.N.Blokhin» of the Ministry of Health of Russia,
Tübingen, Germany
Pediatric Oncology And Hematology Institute, Moscow, Russian Federation

Background and Aims: NUT carcinoma (NC) is a rare and highly


Background and Aims: Superselective intraarterial chemotherapy
aggressive tumor mainly occurring in adolescents and young adults
(SIAC) significantly reduced number of enucleations in children
(AYA), defined by the presence of a somatic NUTM1 rearrangement.
with retinoblastoma. Life-threatening complication of SIAC, hemody-
Aim is to establish internationally harmonized consensus recommen-
namic instability and bronchospasm (trigemino-pulmonary, trigemino-
dations for the diagnosis and treatment of AYA with NC in the frame-
cardiac reflex), significantly limit the wider implementation of this
work of the European Reference Network for Paediatric Oncology
technique. We aimed to describe our experience of SIAC, to report the
(ERN PaedCan).
serious adverse cardio-respiratory reactions we have observed.
Methods: The European Cooperative Study Group for Pediatric Rare
Methods: Study includes patients (n = 383) underwent SIAC (n = 618)
Tumors (EXPeRT) developed recommendations according to the Con-
under general anesthesia in 2013–2021.
sensus Conference Standard Operating procedure methodology and
Results: Hemodynamic parameters in patients who underwent SIAC
reviewed by external “experts”. No evidence of level I to II exists. Rec-
procedure for the first time (n = 246) were analyzed: in 88% cases
ommendations were developed based on published prospective (Level-
smooth anesthesia was observed, the deviations of blood pressure
III), but more frequently retrospective series (Level-IV), case reports
and heart rate did not exceed 20% of the baseline values. In 12%
(Level-V) and personal expertise (Level-V). In addition, “strength” of
cases, patients who underwent the first SIAC procedure developed
recommendations were categorized by grading (Grade A to E).
bronchospasm of 5-12 seconds after catheterization of a. ophthalmica
Results: Histology is mandatory for the diagnosis of NC, includ-
with microcatheter. Hemodynamic parameters analysis in patients who
ing immunolabelling with anti-NUT antibodies and molecular biology
underwent repeated SIAC procedures (2nd, 3rd, etc.) (n = 137), in 58%
(NUTM1 rearrangement) [Level-V; Grade-A]. Treatment of NC usually
cases, a clinical picture of the trigemino-pulmonary reflex of varying
combines aggressive approaches in multimodal regimens. Chemother-
severity manifested: of a weak degree - 72%, of a moderate degree -
apy should be considered as first-line treatment (neo-adjuvant VAI-PAI
15%, of a severe degree - 13%.
or VDCy-IE) for unresectable or metastatic tumor (i.e. 3 courses),
Conclusions: Adverse cardio-respiratory reactions are commonly
rapidly followed by local treatment [Level-IV; Grade-B]. Referral to a
observed in SIAC for retinoblastoma. The adverse clinical signs repre-
specialized surgical oncology center is highly recommended [Level-V;
sent an autonomic reflex response and all patients should be consid-
Grade-A]. In localized NC, a complete microscopic surgical resection
ered at-risk. Anesthesiologists must be vigilant for adverse reactions
should be attempted whenever and as soon as possible, followed
and deal with them quickly and effectively. However, further investiga-
by primary irradiation (60-70Gy) and involved lymph nodes area
tions are needed to improve the understanding and management of the
[Level-IV; Grade-B]. For head and neck tumors, a systematic neck
described oculo-pulmonary reflex.
dissection might be considered, even if N0 [Level-V; Grade-C]. Adju-
vant post-irradiation chemotherapy is recommended, for a total of
9 to 12 courses [Level-IV; Grade-B]. For first-line resected tumors, EP002 / #1490 PARAMENINGEAL RHABDOMYOSARCOMA:
concomitant adjuvant chemotherapy to RT may be discussed [Level- A RETROSPECTIVE STUDY OF 22 CASES
IV; Grade-B]. Targeted therapies and immunotherapeutic regimens
should be delivered in the setting of prospective trials [Level-V; Emna Ouertani, Yosr Zenzri, Feryel Letaief, Zahra Ghodhbani, Hajer
Grade-B]. Ben Mansour, Dorra Tajina, Amel Mezlini
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S151 of S635

Salah Azaiez Institute, Pediatric Oncology Department, tunisia, Tunisia Methods: The study included a cohort of survivors of Hepatoblas-
toma(HB) and Malignant Germ Cell tumors(MGCT) registered at pedi-
Background and Aims: Parameningeal location in rhabdomyosarcoma atric surgical-oncology clinic from 1994 to 2016. The ototoxic effects
(RMS) is correlated with a poor prognosis. it represents 16% of all rhab- of Cisplatin were evaluated in the patients by means of a pure-tone
domyosarcomas. We aimed by this study to review parameningeal RMS audiometry(PTA) done during follow up.
treated at the pediatric oncology department of Salah Azaiez institute Results: A total 129 patients were deemed as survivors, with a mean
and to identify possible prognostic factors. duration of follow-up in HB and MGCT of 8.47(SD 4.27) years and
Methods: A monocentric retrospective study was conducted over a 7.84(SD 4.25) years. Of the 117 survivors receiving Cisplatin, one
period of 25 years between 1994 and 2019 at the pediatric oncology patient had hearing difficulty at follow up. PTA was performed in 83
department of Salah Azaïz Institute. Our study involved patients with survivors, of which an audiogram was available for 51 survivors for
parameningeal RMS. All data regarding patients were obtained from evaluation, 28 (54.9%) of which were reported normal. Hearing loss
the medical record. was detected in 39(76.4%) out of 51 survivors by the SIOP grad-
Results: Twenty-two patients were included. The mean age at diagno- ing system. Severe hearing loss was detected in 32(62.7%) survivors
sis was 7 years old (range: 2-9 years) with a male predominance. The (grade 3,4). Hearing loss was detected in 26(51%) survivors accord-
most frequent initial symptom was cranial nerve palsy (n=6). In 77% of ing to the Brock’s Grading system. Severe hearing loss was detected in
cases (n=17), the location was the nasopharynx. Histological examina- 6 (11.7%) survivors. The survivors of MGCT had a significantly higher
tion showed embryonal RMS in 91% of patients. Median tumor size was (92.3%) chance at developing hearing loss compared to 60% of HB sur-
72 mm. Eight patients had metastatic disease at diagnosis. Surgery was vivors (p=0.012). A dose of Cisplatin of >/=420mg/m2 was found to
performed in 31% of cases (n=7) with negative margins in 50%. Five be associated with hearing loss, with a sensitivity of 75% and speci-
patients had lymph node involvement was observed in 23% of patients. ficity of 66.67%. The mean age at diagnosis was significantly higher in
Seven patients had radiotherapy. Twenty patients received chemother- children with hearing loss(48.50,SD 47.54months) as compare to chil-
apy. Intent for chemotherapy was neoadjuvant in 54%, adjuvant in 18% dren without hearing loss(24.5,SD 32.12months) (p=0.01928). Age at
and palliative in 18% of patients. Overall survival (OS) was 75% at audiometry did not correlate with hearing loss.
one year and 36% at three years. The prognostic factors influencing Conclusions: The SIOP grading system, being more sensitive, must be
OS were age (5-10 years old) (p=0.008) and lymph node involvement uniformly used to screen for hearing loss. Older age at diagnosis, higher
(p<0.001). Relapse free survival (RFS) was 52% at one year and 31% dose and MGCT survivors need close monitoring.
at three years for patients with localized disease. The prognostic factor
influencing RFS was lymph node involvement (p=0.002).
Conclusions: Parameningeal rhabdomyosarcoma is a particular neo- EP004 / #1666 ANTHROPOMETRIC OUTCOMES IN
plasm with a poor prognosis. Age (5-10 years old) and lymph node SURVIVORS OF PEDIATRIC SOLID TUMORS
involvement were shown to significantly influence survival.
Mehak Sehgal1 , Vishesh Jain2 , Sandeep Agarwala3 , Anjan Dhua2 ,
Sameer Bakhshi4 , Mani Kalaivani5
EP003 / #1602 LONG TERM OTOTOXIC EFFECTS OF 1 All India Institute of Medical Sciences, New Delhi, Pediatric Surgery, New
CISPLATIN IN SURVIVORS OF PEDIATRIC SOLID TUMORS Delhi, India; 2 All India Institute of Medical Sciences, Department Of Pedi-
atric Surgery, New Delhi, India; 3 All India Institute of Medical Sciences,
Mehak Sehgal1 , Vishesh Jain2 , Sandeep Agarwala3 , Anjan Dhua3 , Alok Department Of Paediatric Surgery, New Delhi, India; 4 All India Institue of
Thakar4 , Sameer Bakhshi5 , Mani Kalaivani6 Medical Sciences, Department Of Medical Oncology, New Delhi, India; 5 All
1 All India Institute of Medical Sciences, New Delhi, Pediatric Surgery, New India Institue of Medical Sciences, New Delhi, Biostatistics, New Delhi, India
Delhi, India; 2 All India Institute of Medical Sciences, Department Of Pedi-
atric Surgery, New Delhi, India; 3 All India Institute of Medical Sciences, Background and Aims: The importance of nutritional status in child-
Department Of Pediatric Surgery, New Delhi, India; 4 All India Institute of hood cancer patients cannot be overemphasized, not only due to
Medical Sciences, New Delhi, Otolaryngology, New Delhi, India; 5 All India its potential impact on the disease and survival, but also for its
Institue of Medical Sciences, Department Of Medical Oncology, New Delhi, impact on their survivorship. We sought to evaluate the long-term
India; 6 All India Institue of Medical Sciences, New Delhi, Biostatistics, New arthropometric outcomes in pediatric solid tumor survivors
Delhi, India Methods: The study included a cohort of pediatric solid tumor sur-
vivors registered at pediatric surgical-oncology clinic from 1994 to
Background and Aims: Cisplatin is an integral component of 2016. Anthropometry was noted at the time of presentation, after
chemotherapeutic regimes of childhood tumors, but their use may completion of chemotherapy, and at last follow-up. The z-scores for
impart a significant hearing deficit in long-term survivors. We aim to weight-for-age and height-for-age were calculated using WHO growth
evaluate the long-term auditory effects of Cisplatin in survivors of charts for age <5 years and Indian Academy of Pediatrics growth charts
pediatric solid tumors for age >/= 5 years
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S152 of S635 ABSTRACTS

Results: Of the survivors, 317 survivors, comprising of 48, 81 and 188 were discharged home, an additional 33% were discharged home with
survivors of HB, MGCT and WT respectively, were included in the anal- home healthcare. The median age was 7 (IQR 3-16), with 41.9% female
ysis. The median age at diagnosis was 24.5 (IQR 59-13.2) months, with patients, and 55.2% white patients. Patients were most commonly
range of 5-19.54years of follow-up. On evaluating the z-scores of the covered by private insurance (48%) and received care at an urban aca-
collective cohort of survivors at three time points, we found that the z- demic medical center (100%); 35.5% were transferred to these centers.
scores for height for age, weight for age and BMI showed an improving Patients underwent a median of 13 procedures (IQR 11-20). Median
trend in nutrition. The difference in the prevalence of malnutrition and hospital charges were $1,302,684 ($705,556-$2,167,772). Median
severe malnutrition was found to be statistically significant when any charges per day were $26,887 ($19517-$57066).
two time points were compared during follow-up. A similar trend was Conclusions: ECMO is a lifesaving but resource intensive method of
seen in the individual cohorts of HB, MGCT and WT, which showed 10% resuscitation. Although ECMO has been infrequently used in pedi-
and 7.5%, 10.9% and 9%, 15.7% and 11.4% to be stunted and wasted at atric oncology patients, the survival of these patients is consistent with
last follow-up. We found no significant effect of age at diagnosis on the overall outcomes of non-oncology patients. Further studies, includ-
anthropometric measures at last follow-up. On evaluating BMI in 28 ing international collaborations, are needed to further investigate
adult survivors, we found 43% to be in the underweight category. indications and outcomes of ECMO cannulation in these patients.
Conclusions: Anthrometric measures improve during follow-up, how-
ever, up-to 15% children persist being malnourished which is close to
the national averages. Thus, the effect of therapy improves over time. EP006 / #216 REVIEW OF NEONATAL HEPATIC TUMORS
REQUIRING HEPATIC RESECTION IN THE EARLY POSTNATAL
PERIOD
EP005 / #1157 HEALTHCARE UTILIZATION OF
EXTRACORPOREAL MEMBRANE OXYGENATION IN CHILDREN Hidehito Usui, Norihiko Kitagawa, Masato Shinkai, Kyoko Mochizuki,
WITH CANCER: A NATIONAL EXPERIENCE Yuma Yagi, Kazuyoshi Okumura, Akio Kawami
Kanagawa Children’s Medical Center, Department Of Surgery, Minami-ku,
Harold Leraas1 , Mary Moya-Mendez1 , Victoria Donohue2 , Griffin Yokohama, Japan
Mcdaniel3 , Samith Venkatesh1 , Diego Schaps1 , Lars Wagner4 , M.
Elisabeth Tracy5 Background and Aims: Neonatal liver tumors may require early post-
1 Duke University, Surgery, Durham, United States of America; 2 Duke Uni- natal hepatic resection owing to tumor rupture, respiratory failure, and
versity, Surgery, Los Angeles, United States of America; 3 University of circulatory failure. In this study, we reviewed such cases in our hospital.
Cincinnati, School Of Medicine, Cincinnati, United States of America; 4 Duke Methods: This study included patients diagnosed with liver tumor and
University, Pediatric Hematology/oncology, Durham, United States of Amer- treated with liver resection in the early postnatal period at our hos-
ica; 5 Duke University Medical Center, Pediatric Surgery, Durham, United pital between 1980 and 2021. We retrospectively reviewed medical
States of America and surgical records with respect to data on gestational age and birth
weight, clinical course, surgery, and complications.
Background and Aims: Extracorporeal Membrane Oxygenation Results: Seven patients were included in this study. The time of diag-
(ECMO) is a resource-intensive, lifesaving form of resuscitation which nosis was prenatal in four patients and postnatal in three patients,
is infrequently used in pediatric oncology patients. However, with and the mode of delivery was vaginal in four patients and cesarean in
the use of ECMO increasing overall in the pediatric population, the three patients. The median gestational age was 38 (37–40) weeks, and
experience with ECMO in oncology patients should be updated. We the median birth weight was 3152 (2564–3898) g. The median tumor
sought to describe healthcare utilization and outcomes in pediatric size was 10 (2.5–11.5) cm. The final diagnosis was hepatoblastoma in
oncology patients requiring ECMO cannulation. three patients, hemangioma in two, hamartoma in one, and focal nodu-
Methods: We used the Kids Inpatient Database (2019) to identify lar hyperplasia in one. The diagnosis was not confirmed preoperatively
a cohort of patients admitted with primary diagnosis of malignancy in six patients. The median age at surgery was 6 (0–62) days. Six of
who subsequently underwent ECMO cannulation. This dataset con- seven patients had a critical condition, including two with circulatory
tains a national representative sample of pediatric hospitalizations in failure owing to tumor rupture, one owing to intratumoral hemorrhage,
the United States. We reported descriptive demographics, and base- one owing to abdominal compartment syndrome, and two owing to res-
line disease information. We then quantified length of stay, total piratory failure. Five patients underwent complete hepatic resection
hospitalization charges, and charges per day. without complications; however, one patient required intraoperative
Results: We identified 31 patients with a primary oncology diagnosis resuscitation owing to hyperkalemia from the tumor that resulted in
who underwent ECMO cannulation. The most common malignancies cardiac arrest, and another patient required silo construction, Rex
included acute myeloid leukemia (AML), acute lymphocytic leukemia shunt, and hepatic ductal jejunal anastomosis.
(ALL), and Non-Hodgkins Lymphoma. Children with solid tumors rep- Conclusions: Neonatal liver tumors can lead to serious systemic condi-
resented 22.5% of children cannulated (7/31). In hospital mortality was tions and may require high-risk early postnatal hepatectomy. However,
51.6% (N=16), consistent with overall ECMO outcomes. Of these, 33% they can be managed by quick decision-making and troubleshooting.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S153 of S635

E-Poster Topic: AS05.a Acute Lymphoblastic Leukaemia Vega-Vega7 , Sergio Garay1 , Martha Castro1 , Rocio Clemente-Garces1 ,
Berenice Jarquin-Ramirez1 , Netzi Rivera-Sanchez1 , Julio Moreno1 ,
E-POSTER VIEWING Paola Friedrich6
1 Hospital Infantil Teleton de Oncologia, Subdireccion De Diagnostico Y
EP007 / #856 MINIMAL RESIDUAL DISEASE IN ACUTE Banco De Sangre, Querétaro, Mexico; 2 Hospital O’Horan, Servicios de Salud
LYMPHOBLASTIC LEUKEMIA AND ITS RELATIONSHIP WITH de Yucatan, Pediatric Oncology Unit, Merida, Mexico; 3 Hospital Civil de
OTHER PROGNOSTIC FACTORS – A SINGLE CENTER Guadalajara, Pediatric Oncology, Guadalajara, Mexico; 4 Hospital Pediatrico
EXPERIENCE FROM INDIA de Sinaloa, Hemato-oncology Unit, Culiacan, Mexico; 5 Casa de la Amis-
tad para Niños con Cancer, Project Coordinator, Ciudad de Mexico, Mexico;
Chinmayee Agrawal, Sai Madhuri Boppana, Santhosh Devadas, 6 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis,

Vinayak Maka, Nalini Kilara, Swaratika Majumdar, Rasmi Palassery United States of America; 7 Hospital Infantil Teleton de Oncologia, General
MS Ramaiah Medical College, Medical Oncology, Bengaluru, India Director, Querétaro, Mexico

Background and Aims: In low- and middle-income countries, Min- Background and Aims: The “Bridge Project”, a Mexico in Alliance with
imal Residual Disease (MRD) based therapy-intensification of acute St. Jude (MAS) initiative, seeks to grant access to specialized diagnos-
lymphoblastic leukemia (ALL) isn’t always possible since the test is tic tests to identify cytogenetic and molecular alterations in childhood
expensive, time consuming and requires technical expertise. The pur- acute lymphoblastic leukemia (ALL) patients. From May 2019 to March
pose of this study is to assess the correlation of MRD with other 2022, newly diagnosed patients with ALL from six hospitals in Mex-
prognostic factors at diagnosis. ico have been benefited. We aim to describe results of molecular
Methods: In this retrospective study, patients <40 years of age with characterization for ALL patients in participating MAS centers.
ALL who had immunophenotyping, cytogenetic studies, and CSF analy- Methods: A consensus-derived diagnostic panel including cytomor-
sis done at diagnosis, completed BFM-based induction chemotherapy phology, immunophenotype, DNA index, karyotype (with analysis of
and underwent bone marrow analysis at the end of induction were 20 metaphases), fluorescence in situ hybridization (FISH) (including
included. Patients were classified as B or T cell ALL; NCI standard (SR) KMT2A, ETV6/RUNX1, BCR/ABL, E2A/PBX1 and iAMP21 for B-
or high risk (HR); favorable risk [t(12:21), hyperdiploidy (FR)], poor risk lineage ALL and KMT2A, BCR/ABL, E2A/PBX1, TLX1, TLX3, CDKN2A
[Hypodiploidy, t(9:22), MLL translocation, t(1:19), iAMP21, IKZF1 dele- and TRA/D) for T-lineage ALL, and flow cytometry Minimal Residual
tion (PR)] or intermediate risk (neither FR nor PR) cytogenetic]; CNS Disease (MRD) was used. Samples were centrally analyzed at Hospital
positive or negative; good (GPR) or poor prednisone response (PPR); Infantil Teleton de Oncologia Molecular Genetics Laboratory.
and MRD (<0.01% or >0.01%) at the end of induction. Results: We included 276 children and a total of 1,369 samples were
Results: Between 2015 and 2021, 60 patients met the inclusion crite- analyzed. A diagnosis of ALL was established in 229 (82.9%) cases, of
ria. Median age was 11.5 years; 40 (66.6%) were <18 and 20 (33.3%) which 213 (93%) were B-lineage and 16 (7%) were T-lineage. Stud-
were >18 years old. Male: Female ratio was 1.86. Forty-one (68.3%) ies performed on ALL cases were as follows: Cytomorphology 270
had B-ALL and nineteen (31.6%) T-ALL. Forty-four patients (73.3%) (98.5%), immunophenotype 276 (100%), DNA index 274 (99.2%), kary-
met the NCI HR criteria. PR, IR, and GR cytogenetics were seen in 12 otype 266 (96.3%), FISH 259 (93.8%). MRD was determined for all
(20%), 30 (50%) and 18 (30%) patients respectively. Forty-four patients confirmed ALL cases at previously established timepoints. Significant
(73.3%) had GPR. Seventeen (28.3%) were MRD positive at the end findings include abnormal karyotype in 113 (49.3%), hyperdiploidy in
of induction. Patients with B-ALL (24%), NCI-SR (6%), GR cytogenet- 53 (23.1%), complex karyotype in 15 (6.5%) cases. Recurrent struc-
ics (22%) and GPR (22.7%) had a lower incidence of positive MRD; tural alterations found were t(12;21) in 40 (17.4%), t(1;19) in 14 (6.1%),
NCI risk status had the highest statistical significance [OR 0.12, 95% t(9;22) in 6 (2.6%), gene break MLL/t (4;11) in 9(3.9%), iAMP21 in
CI 0.01-0.97]. 25 (10.9%) of B-lineage cases, and del(9)(p21) in 5 (2.1%), TRA/D
Conclusions: Efforts should be made to have standardized MRD test- (14)(q11.2) rearrangement in 3 (1.3%) of T-lineage cases.
ing routinely available in LMICs. Larger studies may be able to suggest Conclusions: Access to cytogenetic and molecular characterization
other clinical predictors of positive MRD. for chilhood ALL and systematic follow-up evaluation with flow
cytometry MRD have been possible with the "Bridge Project". This
is resulting in better stratification and treatment allocation for
EP008 / #917 CYTOGENETIC AND MOLECULAR patients.
CHARACTERIZATION OF CHILDHOOD ACUTE LYMPHOBLASTIC
LEUKEMIA WITH A CONSENSUS-DERIVED DIAGNOSTIC PANEL.
EXPERIENCE FROM A COLLABORATIVE NETWORK IN MEXICO EP009 / #552 CLINICOPATHOLOGICAL FEATURES AND
PROGNOSTIC VALUE OF P16 (CDKN2) GENE DELETION
Dinora Aguilar Escobar1 , Pablo Gonzalez-Montalvo2 , Hugo AMONG PEDIATRIC ACUTE LYMPHOBLASTIC LEUKEMIA
Romo-Rubio3 , Daniela Arce Cabrera4 , Nataly Mercado-Cardenas5 , PATIENTS
Naomi Echeandia-Abud6 , Karla Guerrero-Gomez5 , Lourdes
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S154 of S635 ABSTRACTS

Anna Avagyan1,2 , Arusyak Ivanyan3 , Gevorg Tamamyan1,2 , Lala Lilian Bachuba1 , Anna Massawe1 , Godlove Sandi1 , Lulu Chirande2 ,
Vagharshakyan1 , Mery Petrosyan1,2 , Anna Hovhannisyan4,5 , Medea Hadija Mwamtemi1
Anastasiadi1 , Lusine Krmoyan1,2 , Narine Gazaryan6 1 Muhimbili National Hospital, Pediatrics And Child Health, Dar es Salaam,
1 Hematology center after prof. R. Yeolyan, Pediatric Cancer And Blood Tanzania; 2 Muhimbili University of Health and Allied Sciences, Pediatrics
Disorders Center Of Armenia, Yerevan, Armenia; 2 Department of Pedi- And Child Health, Dar es Salaam, Tanzania
atric Oncology and Hematology, Yerevan State Medical University After
M. Heratsi, Yerevan, Armenia; 3 Hematology center after prof. R. Yeolyan, Background and Aims: Febrile neutropenia (FN) is common in Acute
Department Of Hematology, Yerevan, Armenia; 4 Hematology center after Lymphoblastic Leukemia (ALL). Malnutrition predisposes children with
prof. R. Yeolyan, Molecular Biology And Cytogenetic Department, Yere- ALL to FN by alterations of body metabolism which aggravates low
van, Armenia; 5 “EcoSense” Diagnostic Centre and Laboratory, Laboratory body immunity. The aim of this study was to determine the prevalence
Of Genetics, Yerevan, Armenia; 6 Institute of Physiology, Laboratory Of of malnutrition in ALL children with FN and to compare it with ALL
Toxinology And Molecular Systematics, Yerevan, Armenia children without FN
Methods: A retrospective cross sectional study involved data from the
Background and Aims: The p16 is a tumor-suppressor protein files of children aged 0 to 10 years diagnosed with ALL admitted at
encoded by a CDKN2 gene located in 9p21, which deletion has been Muhimbili National Hospital from January 2019 to December 2020.
found in various types of tumors. The aim of this study is to investi- Data included Age, Sex, ALL type, body weight on admission, history of
gate the clinicopathological features and prognostic value of p16 gene fever with body temperature ≥ 38◦ C at any point from diagnosis to the
deletion in pediatric Acute lymphoblastic leukemia (ALL). end of remission induction treatment, and absolute neutrophil count
Methods: The Hematology Center of Armenia is the only medical <1/mm3 during fever presentation. Weight for Age was interpreted
institution in Armenia performing diagnostics and treatment of pedi- using WHO Z scores. Malnutrition was defined as Mild to moderate
atric ALL patients. A retrospective analysis of 81 pediatric patients, underweight (>-3 to -1SD) and Severe underweight (≤-3 SD). FN was
diagnosed with ALL between January 2019 and December 2021, was defined as body temperature of ≥ 38◦ C with Absolute neutrophil count
performed. Data were analyzed with the IMB SPSS version 23 sta- <1/mm3 . Frequency percentages, Pearson Chi square test and Fischer
tistical software. We carried out descriptive statistical analysis and exact test were used in data analysis
presented all the continuous variables by means, medians, and stan- Results: A total of 59 children, 28(47.5%) males and 31(52.5%) females
dard deviations (SD). The overall survival rate was estimated using the were included in the study. Mean age was 5.2 ± 2.0 years. Children with
Kaplan-Meier method. FN were 45(76.3%) and those without FN were 14(23.7%). FN mostly
Results: We analyzed 81 patients, 14 (17.2%) of which had p16 dele- affected age group 1 to 5 years (p value 0.004). The prevalence of Mal-
tion. The annual incidence ranges from 7% to 27%. 92.9% of cases were nutrition in children with FN was 28.9% where severe underweight
B-cell and 7.1% were T-cell precursor ALL. No significant association was 11.2% and mild to moderate underweight was 17.7%. Prevalence
was detected with age, sex, performance status (hepatosplenomegaly, of Malnutrition in children without FN was 28.6% (mild to moderate
lymphadenopathy, mediastinal mass, CNS involvement), laboratory underweight), none had severe underweight (0%)
indications (hyperleukocytosis, neutropenia, HGB, thrombocytopenia, Conclusions: Severe malnutrition predisposes ALL children to FN.
LDG, peripheral and bone marrow blast cells), immunophenotypic Early nutritional intervention is important in children with ALL to
features and other cytogenetic abnormalities (TEL/AML1, BCR/ABL, reduce the incidence of FN.
MLL, IGH, and C-MYC rearrangement, P53 deletion). All patients were
treated according to the ALLIC-BFM-2009 protocol. Two patients
(11.8%) abandoned treatment during the induction regimen. The dis- EP011 / #1620 LONG NON-CODING RNAS CONTRIBUTING
tribution between standard, intermediate and high-risk groups was FOR CRLF2 OVEREXPRESSION IN ACUTE LYMPHOBLASTIC
equable (28.6%). One patient had disease progression after the induc- LEUKAEMIA
tion therapy. The 2-year overall survival (OS) was 92%.
Conclusions: No significant correlation between the p16 gene deletion Thayana Barbosa1 , Ana Luiza Maciel1 , Caroline Poubel1,2 , Mariana
and clinicopathological feathers, as well as the prognostic impact, was Boroni2 , Marcela Mansur1,3 , Mariana Emerenciano1
detected. The 2-year OS of the p16 deletion positive ALL is compati- 1 Instituto Nacional de Câncer – INCA, Acute Leukaemia Riosearch Group,
ble with the 2-year OS of all pediatric ALL cases. However, due to the Division Of Clinical Research, Research Centre, Rio de Janeiro, Brazil;
limited number of recruited patients and the short period of follow-up 2 Instituto Nacional de Câncer – INCA, Bioinformatics And Computational
further investigation is needed. Biology Laboratory, Research Centre, Rio de Janeiro, Brazil; 3 University of
Oxford, Department Of Paediatrics, Children’s Hospital, John Radcliffe Hos-
pital And Mrc Weatherall Institute Of Molecular Medicine - Wimm, Oxford,
EP010 / #343 MALNUTRITION AND FEBRILE NEUTROPENIA United Kingdom
IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA AT
MUHIMBILI NATIONAL HOSPITAL Background and Aims: CRLF2 overexpression (CRLF2-high)
has been associated with unfavourable prognosis in B-cell acute
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S155 of S635

lymphoblastic leukaemia (B-ALL) cases. The presence of CRLF2 rear- were addressed in this retrospective cohort study of sequential
rangements (CRLF2-r) and CRLF2 F232C mutations can explain only outcomes.
half of the cases with this gene overexpression. Long non-coding RNAs Methods: Of 217 children with ALL, 63% with standard risk disease,
(lncRNAs) play a role in the development and progression of leukaemia treated between 2000 and 2015 on Dana Farber Cancer Institute pro-
and can interfere in the transcriptional regulation of protein-coding tocols, 69 received alendronate for a mean of 87 weeks after dual
genes. In this scenario, we hypothesise that the dysregulation of energy X ray absorptiometry (DXA). DXA was repeated after comple-
lncRNAs might be a potential mechanism underlying CRLF2-high in tion of alendronate and again, in a subgroup of 32 children, 5-9 years
B-ALL patients. later. Lumbar spine areal bone mineral density (LSaBMD) Z scores were
Methods: We included 126 diagnostic B-ALL cases from the TAR- obtained and values corrected for height, age and weight (HAW) were
GET cohort and delineated their molecular profile based on WGS and calculated for subjects 3-18 years of age.
RNA-seq data. Differentially expressed (DE) lncRNAs in CRLF2-high Results: Almost 80% (172/217) of the children remain in continuous
patients were identified using DESeq2 v.1.28.1. Lncpath package was complete remission at a mean of 14.5 years from diagnosis. Of those
used to obtain functional pathways influenced by the DE lncRNAs. The who received alendronate, which was almost uniformly well tolerated,
DE lncRNA-targets experimentally validated were obtained from the 7/69 (10.3%) relapsed compared to 19/89 (21.3%) who did not receive
LncRNA2Target v3.0 database, and the interactions were tested by the drug (p=0.06). The mean unmodified LSaBMD Z score rose from -
correlation analysis using RNA-seq TARGET data. All analyses were 1.78 to -0.47. This gain was statistically significant for both unmodified
conducted using the GRCh37-hg19 genome as reference. (p<0.0001) and HAW corrected Z scores (-1.32 to -0.42, p<0.0001).
Results: A total of 293 up- and 70 down-regulated DE lncRNAs There was a modest, statistically insignificant median reduction (0.045)
were identified in CRLF2-high patients. KEGG analysis demonstrated of LSaBMD Z score (p=0.09) subsequently in the subgroup (N=32) on
that these DE lncRNAs were mainly involved in ribosome and leuko- long term follow-up.
cyte transendothelial migration pathways. Among them, we identified Conclusions: Alendronate is well tolerated and appears to be mod-
five potential interactions lncRNA-target positively correlated includ- erately and sustainably effective in osteopenic children with ALL.
ing RPL34-AS1-MIR3663, LINC00161-MIR21, LINC00161-MIR590, Whether it offers protection against relapse of leukemia needs further
PWRN1-MIR21 and PART1-MIR149. Interestingly, RPL34-AS1 and study.
MIR21 were also closely correlated with CRLF2, showing a high
expression in CRLF2-high patients (p=0.036 and p=0.004, respec-
tively). Considering the functional role of these lncRNAs in cell pro- EP013 / #382 AMPK EPIGENETICALLY REPROGRAMS GENE
liferation, invasion, migration and apoptosis, we will validate these EXPRESSION AND PROMOTES ADAPTATION/SURVIVAL IN
potential interactions using B-ALL cell lines as models. RESPONSE TO ENERGY/METABOLIC STRESS THROUGH
Conclusions: Our findings indicate a potential mechanistic role for BINDING TO A CHROMATIN-ASSOCIATED TRANSCRIPTION
these lncRNAs interactions on leukemogenesis which could unravel COMPLEX IN ACUTE LYMPHOBLASTIC LEUKEMIA
novel biomarkers, and clarify how the expression of CRLF2 is regulated.
Julio Barredo1 , Guangyan Sun2 , Guy Leclerc3
1 University of Miami Miller School of Medicine, Pediatrics, Medicine,
EP012 / #293 SAFETY AND EFFICACY OF ALENDRONATE Bichemistry And Molecular Biology, Miami, United States of America;
TO TREAT OSTEOPENIA IN CHILDREN DURING THERAPY FOR 2 Univerisity of Miami Miller School of Medicine, Pediatrics, Miami, United
ACUTE LYMPHOBLASTIC LEUKEMIA States of America; 3 University of Miami MIller School of Medicine, Pedi-
atrics, Miami, United States of America
Ronald Barr1 , Paula Macdonald2 , Amy Cranston2 , Misha Virdee1 , Troy
Farncombe3 , Uma Athale1 Background and Aims: Survival rates for relapsed/refractory acute
1 McMaster Unversity, Pediatrics, Hamilton, Canada; 2 Hamilton Health Sci- lymphoblastic leukemia (ALL), the most common cancer in children
ences, Pediatrics, Hamilton, Canada; 3 Hamilton Health Sciences, Nuclear and adolescents, remain poor. We and others have reported that
Medicine, d, Canada ALL cells are vulnerable to energy/ER-stress conditions mediated
by AMPK activation. We hypothesized that in ALL cells undergoing
Background and Aims: Low bone mineral density (osteopenia) energy/metabolic stress, activation of AMPK epigenetically modifies
is encountered commonly in children with acute lymphoblastic the transcriptome to promote a compensatory survival/adaptation
leukemia (ALL) before, during and after therapy. Prior experience response.
with alendronate, a third generation bisphosphonate administered Methods: To identify genome-wide genes regulated by direct associa-
orally, demonstrated high tolerability and evident clinical efficacy. tion of AMPK to chromatin in response to energy/metabolic stress, we
Moreover, some bisphosphonates have exhibited apparent anti- constructed T-ALL (CCRF-CEM/CN2) stable cell line expressing HA-
cancer activity. However, concerns have been expressed about the AMPKα2 and performed ChIP-seq assays using HA and Rpb1 antibod-
long term safety and utility of such agents in children, includ- ies in CN2 cells grown in RPMI ± glucose for 24h. To correlate the level
ing the occurrence of hematological abnormalities. These concerns of AMPKα2 recruitment with the level of gene mRNA expression, we
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S156 of S635 ABSTRACTS

used RNA-seq. Interactions between AMPK and chromatin-associated Department Of Pathology And Experimental Cancer Research, Budapest,
proteins were identified using co-immunoprecipitation (Co-IP) and Hungary
kinase assays.
Results: ChIP-seq identified 612 gene loci exhibiting recruitment of Background and Aims: Acute lymphoblastic leukemia (ALL) is the
HA-AMPKα2. Among them, 431 were unique to untreated control most common malignancy of childhood. Whole transcriptome-based
whereas 171 were unique to no-glucose conditions. RNA-seq indi- sequencing studies revealed distinct, gene expression-based ALL sub-
cated that of 3497 genes altered by AMPK activation, two-thirds groups. These subgroups may provide prognostic or predictive infor-
were downregulated. Among these, we uncovered a cluster of his- mation and therefore improve clinical outcome. In this study, we aimed
tone genes exhibiting both decreased recruitment of HA-AMPKα2 to determine ALL subgroups using an RNA-based sequencing panel,
to chromatin and mRNA downregulation. Further ChIP-qPCR using that investigates 1385 clinically and diagnostically relevant genes. As
AMPKα2 antibody confirmed these data in KASUMI-2 (Bp-ALL). Co- alternative splicing events are relatively poorly investigated in pedi-
IP uncovered that AMPKα2 interacted with putative members of atric ALL, we started developing an alternative splicing based subgroup
an AMPK/chromatin-associated transcription factor complex includ- classification, complementing gene expression- based information.
ing the TATA-Box Binding Protein Associated Factor (TAF), integrator Methods: Diagnostic bone marrow samples from 191 children with B-,
(INT), and RNA polymerase II. More importantly, we uncovered for and T-cell precursor ALL were sequenced using the Illumina TruSight
the first time that TAF1 is phosphorylated by the AMPKα2β1γ1 RNA Pancancer kit on the Illumina MiSeq platform. Gene-expression
heterotrimer. levels were determined using kallisto, and alternative splicing events
Conclusions: Our data show that in response to energy/metabolic were analyzed by SUPPA2. Normalization of RNA-seq data was fol-
stress, AMPKα2 binds directly to a chromatin-associated lowed by principal component analysis, hierarchical clustering of genes
transcription complex to epigenetically reprogram gene or samples and differential expression or differential splicing analysis
expression promoting cellular adaptation/survival. Further between known subgroups.
elucidation of AMPK’s interactions with members of this puta- Results: Hierarchical clustering of samples and genes at the gene
tive AMPK/chromatin-associated transcription complex may expression level revealed 10 patient subgroups, that is comparable
lead to unique opportunities for epigenetic-based therapeu- with literature information. Clustering of alternative splicing events
tic interventions or combination strategies in relapse/refractory detected 9 groups that showed a different sample composition com-
ALL. pared to gene expression- based groups. Gene expression-based sam-
ple groups showed a 65% mean overlap with existing WHO-subgroups,
while only 31% overlap was found in alternative splicing-based groups.
EP014 / #1682 DEVELOPMENT OF A GENE EXPRESSION One sample group was separated by gene expression as well as
AND SPLICING BASED CLASSIFICATION METHOD FOR alternative splicing and was dominated by T-ALL samples.
PEDIATRIC ACUTE LYMPHOBLASTIC LEUKEMIA USING RNA Conclusions: Our results show that categorization of ALL patient-
PANEL SEQUENCING subgroups based on gene expression may be feasible using not only
whole transcriptome sequencing, but also panel sequencing technolo-
Gábor Bedics1 , Anna Bekő1 , Tibor Nagy2 , Szilvia Krizsán1 , Borbála gies. This could improve molecular diagnostics of pediatric ALL, while
Péterffy1 , Lajos László Hegyi1 , Gergely Kriván3 , Bálint Egyed4 , Dániel lowering costs. Additionally, results indicate that splicing analysis is
Erdélyi4 , Gábor Kovács4 , Béla Kajtár5 , Gábor Ottóffy6 , Csongor Kiss7 , feasible with panel sequencing technologies and may broaden the
Anikó Ujfalusi8 , György Péter9 , Katalin Bartyik10 , Csaba Bödör1 , spectrum of clinical diagnostics in pediatric ALL.
Endre Sebestyén11
1 Semmelweis University, Hcemm-su Molecular Oncohematology Research
Group, Department Of Pathology And Experimental Cancer Research, EP015 / #926 TIME TO DIAGNOSIS OF HEMATOLOGICAL
Budapest, Hungary; 2 University of Debrecen, Department Of Biochemistry MALIGNANCIES IN CHILDREN IN AN ONCOLOGY UNIT OF A
And Molecular Biology, Debrecen, Hungary; 3 Central Hospital of Southern COUNTRY WITH LIMITED RESOURCES
Pest - National Institute of Hematology and Infectious Diseases, Pedi-
atric Hematology And Stem Cell Transplantation Unit, Budapest, Hungary; Sarra Benmiloud, Mohamed Hbibi, Moustapha Hida
4 Semmelweis University, 2nd Department Of Pediatrics, Budapest, Hun- University Hospital Hassan II, Pediatrics, Fez, Morocco
gary; 5 University of Pécs Clinical Centre, Department Of Pathology, Pécs,
Hungary; 6 University of Pécs Clinical Centre, Department Of Pediatrics, Background and Aims: The prognosis of hematological malignancies
Pécs, Hungary; 7 University of Debrecen, Division Of Pediatric Hematology- in pediatrics remains poor in developing countries. One of the main
oncology, Institute Of Pediatrics, Debrecen, Hungary; 8 University of Debre- causes is the delay in diagnosis. Studies assessing the factors associ-
cen, Department Of Laboratory Medicine, Debrecen, Hungary; 9 Heim Pál ated with the time-to-diagnosis of hematological malignancies are rare
Children’s Hospital, Hemato-oncology Unit, Budapest, Hungary; 10 Faculty in Africa. The objective of our study is to identify diagnostic delays in
of Medicine, University of Szeged, Department Of Paediatrics And Pae- hematological malignancies in children and analyze the risk factors for
diatric Health Care Center, Szeged, Hungary; 11 Semmelweis University, diagnostic latency.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S157 of S635

Methods: We performed a study of 112 cases of children with Methods: Bone marrow samples from pediatric patients with sus-
hematological malignancies referred to the Pediatric Hematology pected ALL were obtained before the start of treatment. Mononuclear
and Oncology Unit of University Hospital Hassan II of Fez between cells were isolated with Ficoll-Hypaque, then, proteins were isolated
January 2017 and January 2020. We recorded patients’ data through for further identification by shotgun proteomic analysis using a mass
parents’ interview and review of the medical records. We stud- spectrometer coupled to liquid chromatography and applying compu-
ied the time intervals between onset and final diagnosis and start tational tools for identification and analysis. Patients were grouped
of treatment, and investigated associated factors with shorter according to MRD levels (good response: <0.01, slow response: 0.01-
intervals. 0.99) at the end of induction treatment.
Results: The median latency to diagnosis in our study is 66.14 days. Results: Four samples were analyzed, two patients had a good response
The median physician interval is higher than the median patient inter- and two had a slow response. Identification of a large number of
val (37 vs. 28 days). Acute leukemia and the transfer to our unit after proteins (886-1724) per patient was achieved, from which 631 were
the first consultation are associated with a shorter delay of the physi- identified in all patients. A principal component analysis revealed that
cian interval (23.3 days, P=0.003, and 14.2 days, P=0.00 successively). the expression profile of patients is more similar between patients
Isolated lymphadenopathy as a revealing sign is associated with a pro- who have the same type of response to treatment when measured by
longed delay of the patient’s interval (40.9 days, P=0.017). The gender, MRD. In addition, differentially expressed proteins in poor responders
the age, the family size, the distance from the hospital, the parents’ describe a cell cycle activation through MYC, YAP1, and SRF.
employment and level of education, and the type of health insurance Conclusions: This methodology is useful to identify proteins that have
have no significant association with the diagnostic latency. clinical use as diagnostic biomarkers, that are used to determine the
Conclusions: The index of suspicion for hematological malignancy in efficacy of treatment, or as therapeutic targets. Finally, mass spectrom-
children remains low in our country. Our findings shows that acute etry proteomics is useful for identifying therapeutic targets in precision
leukemia and the fast transfer to a specialized unite of cancer are fac- medicine strategies.
tors of shorter diagnosis delay. Thus, the increase in practitioner and
public awareness, and the establishment of a well-identified circuit of
the patient would reduce diagnostic delays. EP017 / #411 FEASIBILITY OF A STANDARDIZED
NEXT-GENERATION SEQUENCING PANEL FOR PRECISION
MEDICINE IN PEDIATRIC ACUTE LYMPHOBLASTIC LEUKEMIA
EP016 / #410 COMPARISON OF PEDIATRIC ALL
LYMPHOBLAST’S PROTEIN EXPRESSION PROFILES ACCORDING Jorge Bermudez-Lugo1 , Viridiana Hernandez-Diaz2 , Horacio Reyes
TO TREATMENT RESPONSE MEASURED BY MINIMAL Vivas3 , Jesus Oria-Hernandez3
RESIDUAL DISEASE 1 Casa de la Amistad para Niños con Cancer, Mexico En Alianza Con St.
Jude, Mexico city, Mexico; 2 Universidad del Valle de Mexico, Escuela De
Jorge Bermudez-Lugo1 , Jose Correa Basurto2 , Marlet Ciencias De La Salud, Mexico city, Mexico; 3 Instituto Nacional de Pediatria,
Martinez-Archundia2 , Juan Martinez Aguilar3 , Horacio Reyes Vivas4 , Laboratorio De Bioquimica Genetica, Mexico city, Mexico
Marta Zapata-Tarrés5
1 Casa de la Amistad para Niños con Cancer, Mexico En Alianza Con St. Jude, Background and Aims: Conventionally, improvements in survival of
Mexico city, Mexico; 2 Instituto Politecnico Nacional, Laboratorio De Diseño pediatric patients with Acute Lymphoblastic Leukemia (ALL) have
Y Desarrollo De Nuevos Fármacos E Innovación Biotecnológica, Mexico city, been based on the optimization of chemotherapy schemes and a risk-
Mexico; 3 Universidad Nacional Autonoma de Mexico, Red De Apoyo A La oriented intensity assignment strategy. Recent evidence shows that
Investigacion, Mexico city, Mexico; 4 Instituto Nacional de Pediatria, Lab- precision medicine, which looks for interpersonal tumor differences at
oratorio De Bioquimica Genetica, Mexico city, Mexico; 5 Fundación IMSS, the molecular level in order to provide tailoring of medical treatment
Reserach Coordination, Mexico City, Mexico to the individual characteristics of patients. The aim of this study was
to evaluate the utility of a standardized next-generation sequencing
Background and Aims: In Mexico, cancer represents the leading cause (NGS) panel to find targets for directed therapies in pediatric patients
of death by disease. Almost half of the cases are due to acute Lym- with ALL.
phoblastic leukemia (ALL). Once chemotherapy treatment is started Methods: Bone marrow samples from seven pediatric patients diag-
98% of patients will achieve remission. Despite the use of clinical, bio- nosed with ALL were preserved in paraffin and analyzed by NGS (Foun-
chemical, immunological, and genetic variables, risk stratification is not dationOne Heme); the targets included 406 genes and 31 selected
yet precise enough to predict which patients will relapse once remis- introns involved in DNA rearrangements, and RNA of 265 genes known
sion is achieved, although it is recognized that treatment response is to participate in oncogenesis or to be modified by target therapy.
the best predictor of the outcome. This study aims to describe the Results: BET domain inhibitors were predicted to have biological
lymphoblast’s protein expression profile from pediatric patients diag- effects in two patients with rearrangements of IGH. MEK inhibitors
nosed with B-ALL according to their response to remission induction could be used in one patient who had an alteration of NRAS. One
treatment. patient had an alteration of JAK2 predicted to be sensitive to
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S158 of S635 ABSTRACTS

ruxolitinib. An alteration in PIK3CA was found in one patient, suggest- malnutrition, area deprivation indices, oral/dental health, pharmacoge-
ing the benefits of using mTOR inhibitors. A patient had an alteration nomics, and the microbiome. Furthermore, we continue to improve the
in FLT3, indicating a possible biologic effect by using kinase inhibitors, precision and accuracy of risk prediction models for severe mucositis.
multikinase inhibitors, tyrosine kinase inhibitors, or MEK inhibitors.
Two patients showed no positive results.
Conclusions: The presence of molecular targets with known drug- EP019 / #1508 IMPROVING SURVIVAL BY DECREASING
triggered biological effects was found in five of seven patients, indicat- INDUCTION MORTALITY AND ENHANCING SUPPORTIVE CARE:
ing that this standardized NGS-sequencing panel could be useful for A SUSTAINABLE STEPWISE APPROACH IN PEDIATRIC
precision medicine in pediatric ALL. It is expected that improvements PATIENTS WITH ALL IN WESTERN KENYA
in patient survival could be reached by using targeted drugs in addition
to standard schemes. George Bogonko1 , Festus Njuguna1 , Gilbert Olbara2 , Frederick
Acknowledgments: We deeply thank Roche who kindly provided the Odongo1 , Sandra Langat3 , Martha Kipngetich1 , Saskia Mostert4,5 ,
tests for this work. Gertjan Kaspers6,7 , Terry Vik1,8
1 Moi University, Pediatric Oncology, Eldoret, Kenya; 2 Moi Teaching and
Referral Hospital, Pediatric Oncology, Eldoret, Kenya; 3 Academic Model
EP018 / #1462 PRELIMINARY ANALYSIS OF RISK FACTORS Providing Access to Healthcare (AMPATH), Pediatric Oncology, Eldoret,
FOR SEVERE MUCOSITIS IN PEDIATRIC PATIENTS WITH ACUTE Kenya; 4 Princess Maxima Center for Pediatric Oncology, Pediatric Oncology,
LYMPHOBLASTIC LEUKEMIA Utrecht, Netherlands; 5 Emma Children’s Hospital, Amsterdam UMC, Vrije
Universiteit, Pediatric Oncology, Amsterdam, Netherlands; 6 Princess Máx-
Melanie Bernhardt, Rachel Harris, Mark Zobeck, Eric Schafer, Austin ima Center, Pediatric Oncology, Utrecht, Netherlands; 7 Emma Children’s
Brown Hospital, Amsterdam UMC, Vrije Universiteit, Pediatric Oncology, Ams-
Baylor College of Medicine, Pediatric Hematology/oncology, Houston, terdam, Netherlands; 8 Indiana University School of Medicine, Pediatrics,
United States of America Indianapolis, United States of America

Background and Aims: High-dose methotrexate (HD-MTX) is a crit- Background and Aims: Since 2010, we have treated children with
ical component of acute lymphoblastic leukemia (ALL) treatment. acute lymphoblastic leukemia (ALL) on resource-adapted protocols at
Racial/ethnic disparities in MTX toxicities have been reported, includ- Moi Teaching and Referral Hospital (MTRH) in Western Kenya. Work-
ing a disparate risk of MTX neurotoxicity and delayed clearance. ing with twining partners from Princess Máxima Center for Pediatric
Patient-related risk factors for severe MTX-associated mucositis are Oncology in the Netherlands and Indiana University in the United
unknown. Refinement of these risk factors may help predict patients at States, we recently published outcomes of children diagnosed with
greatest risk, which may help reduce morbidity and mortality by offer- ALL from 2010-2016. We showed increased mortality during induction
ing opportunities for early intervention. We evaluated the incidence of phase and high rates of treatment abandonment. Initially, our induction
MTX-associated mucositis following doses of HD-MTX in an ethnically phase comprised of vincristine, doxorubicin, and prednisone with even-
diverse population of patients with ALL. tual addition of L-asparaginase. Based on these results, we focused
Methods: We retrospectively reviewed patients diagnosed with ALL on strategies to mitigate both causes of poor outcome. This included
and treated with HD-MTX (5 g/m2) at Texas Children’s Cancer Center active enrollment of families into health insurance, structured parental
(2010-2017). Clinical, treatment, and sociodemographic factors were education, and use of flow cytometry to improve diagnostic accuracy.
systematically collected from the electronic medical record. Mucositis Doxorubicin was dropped from our induction regimen as the supply of
was graded according to a modified CTCAE scale. Mixed effects multi- Vincristine, Prednisone, and L-asparaginase was improved.
variable logistic regression was used to estimate the odds ratios (OR) Methods: Children diagnosed with ALL from 2017- 2020 were iden-
and corresponding 95% confidence intervals (CI) for the association tified from our registry. A retrospective review was done of the
between clinical factors and mucositis. outcomes of 161 children receiving modified treatment with improved
Results: We identified 316 patients who received a total of 1,110 social or financial support for diagnostic testing, insurance coverage,
doses. The majority of patients were male (58%) and self-reported His- and transportation.
panic ethnicity (53%). One-third (n=108, 34%) of patients experienced Results: Mortality in induction (6 weeks) decreased from the historical
at least one episode of Grade 3 or 4 mucositis. Patients who did not rate of 26% to 13%. Abandonment of treatment was reduced from 26%
clear MTX by hour 24 had a significantly higher odds of Grade 3 or to 8%. Overall survival at three years improved from 20% to over 50%.
4 mucositis (OR 2.16, p=0.05), though this was not significant in an Each of these comparisons achieved statistical significance (p<0.003 or
adjusted model of risk. In our preliminary analysis, the risk of severe better).
mucositis was not associated with sex, race, ethnicity, or obesity. Conclusions: Sustainable improvement in the care of children
Conclusions: The ability to predict which patients are at greatest risk with ALL in low- and middle-income countries happens as many
of mucositis remains a challenge. We continue to evaluate potential small steps, that add up to significant advances in outcomes. Our
risk factors, including age, leucovorin dosing, use of oral glutamine, studies highlight the importance of collaboration, need for proper
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S159 of S635

documentation of data, and continuous quality improvement strate- Conclusions: A multivariate predictive model including CCL-2,
gies adapted to available resources. Future strategies will include TNF-alpha, and S100A12 could improve the ability to pre-
improved recognition and diagnosis of ALL, proper risk stratification, dict MRD compared to conventional risk-group stratification
risk-adapted protocols for management of central nervous system alone.
disease, improved supportive care, and further reduction of treatment
abandonment.
EP021 / #1713 OUTCOME OF INFANTILE ACUTE
LYMPHOBLASTIC LEUKEMIA TREATED AT KING HUSSEIN
EP020 / #1266 PHAGOCYTE-RELATED S100 PROTEINS AND CANCER CENTER
CYTOKINES IN ACUTE LYMPHOBLASTIC LEUKEMIA AT
DIAGNOSIS AND FOLLOW-UP MAY IMPROVE PREDICTION OF Rawan Budair1 , Iyad Sultan1 , Amal Abu Ghosh1 , Hasan Hashem2 ,
MINIMAL RESIDUAL DISEASE Rawad Rihani3 , Mayada Abu Shanap2
1 king hussein cancer center, Pediatric Oncology, Amman, Jordan; 2 king Hus-

Ninna Brix1,2 , Christoph Kessel3 , Dirk Foell3 , Henrik Hasle1 , Birgitte sein cancer center, Pediatric, Amman, Jordan; 3 King Hussein Cancer Center,
Klug Albertsen1 , Niels Bruun4 , Søren Hagstrøm2 , Troels Herlin1 Paediatric Hematology/oncology/bone Marrow Transplant, Amman, Jordan
1 Aarhus University Hospital, Department Of Pediatrics And Adolescent
Medicine, Aarhus, Denmark; 2 Aalborg University Hospital, Department Background and Aims: Infantile Acute lymphoblastic leukemia
Of Pediatrics And Adolescent Medicine, Aalborg, Denmark; 3 University (ALL) is rare; despite excellent cure rates in most children
Hospital Muenster, Department Of Pediatric Rheumatology And Immunol- with ALL; infants continue to have poor outcomes with over-
ogy, Muenster, Germany; 4 Aalborg University Hospital, Units Of Clinical all survival of 30-40%. Historically, relapse and therapy related
Biostatistics, Aalborg, Denmark mortality have been the major leading cause of treatment
failure.
Background and Aims: Few studies investigated S100 proteins and Methods: Retrospective chart review for all infants (age less than one
single cytokines in ALL at diagnosis and found low levels of S100A9, year) treated at King Hussein Cancer Center per modified total XV
S100A12, and IL-18 compared to both juvenile idiopathic arthri- protocol.
tis and other autoinflammatory diseases but higher levels than in Results: Between Feb2005 and Jan2022 a total of 23 patients (13
healthy controls. The aim of this study was to evaluate whether males) were treated and followed for a median of 71 months (range, 2-
biomarkers of inflammation like phagocyte-related S100 proteins and 205). Median age at diagnosis was 6.7 months (range, 2.5-11.5). Median
a panel of cytokines at diagnosis could predict the outcome of ALL WBC at diagnosis was 99k/μl (rang, 0.7-570). CNS status at diagnosis
expressed as minimal residual disease (MRD), relapse, and death. were: CNS1 (N=18), CNS2 (N=3) and CNS3 (N=2). Immunophe-
Further we evaluated the level of the biomarkers at diagnosis and dur- notypes were consistent with B-ALL (CD10 negative, n=15; CD10
ing antileukemic therapy and their correlation with basic laboratory positive, n=7) and, T-ALL (n=1). MLL gene rearrangement was posi-
values. tive in 15 patients (65%). Five patients had positive MRD >=0.01%
Methods: In this retrospective cohort study, we evaluated the serum at the end of induction. The estimated 5-year-EFS and OS were 62%
levels of biomarkers of inflammation like phagocyte-related S100 pro- and 69%, respectively. No prognostic factors were identified using
teins and a panel of cytokinesin 150 ALL patients diagnosed at Aalborg univariate and multivariate analysis. Mortalities were due to disease
and Aarhus University Hospitals from January 2001 to December relapse/progression (N=4) or infection (N=2).
2018. Serum concentrations of the proteins were determined by mul- Conclusions: The outcome of infants with ALL at our institution is in
tiplexed bead array assay on a MAGPIX instrument using Luminex line with published literature. This highlights the importance of upfront
software. We performed internal validation using repeated ’10- fold intensive therapy and optimal supportive care: Pre-emptive admissions
cross- validation’ computing the area under the curve (AUC) as well during intensive blocks, prophylactic anti-fungals, nutritional support
as positive and negative predictive values in order to evaluate the and immunoglobulin replacement.
predictive performance.
Results: The levels of S100A9, S100A12, IL-1beta, IL-12p70, IL-13, IL-
17, IL-18, and MPO serum levels increased significantly as chemother- EP022 / #1578 CAR T-CELL PEDIATRIC PATIENTS ADMITTED
apy was initiated. The difference was most pronounced for S100A9 and TO A PEDIATRIC INTENSIVE CARE UNIT (PICU): SUPPORTIVE
S100A12, which had strong positive correlations with the neutrophils TREATMENT AND OUTCOMES
counts. In contrast, TNF-alpha, IL-6, IL-10, CCL-2, MMP-3, and CD25
serum levels decreased after chemotherapy. In a multivariate predic- Marina Caballero Bellón1 , Sara Bobillo Pérez2 , Anna Alonso
tive models with MRD as the outcome, AUC increased from 75% (95% Saladrigues1 , Anna Faura Morros1 , Albert Català1 , Monica Balaguer
CI 66-81%) when using initial risk group stratification alone to 83% Gargallo2 , Iolanda Jordan Garcia2 , Susana Rives1
(95% CI 73-91%) including the biomarkers TNF-alpha, S100A12, and 1 Pediatric Cancer Center Barcelona (PCCB), Hospital Sant Joan de Déu
CCL-2. Barcelona, University of Barcelona, Pediatric Hematology And Oncology,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S160 of S635 ABSTRACTS

Barcelona, Spain; 2 Hospital Sant Joan De Déu, Pediatric Intensive Care Unit, Results: The mean age of the patients were 6 (2-16) years. There were
Esplugues de Llobregat, Spain 32 (78%) standard risk patients. Thirty-four (82%) B-ALL and 7 T-ALL
patients with a mean duration of follow-up 107 months were treated.
Background and Aims: Chimeric antigen receptor (CAR) T-cell CD 19 Central nervous system involvement was present in 3 patients. Mini-
therapy is an effective treatment for relapsed or refractory B-cell acute mal residual disease at 28. day was negative in 36 (87%) patients. The
lymphoblastic leukemia (ALL). It can be associated to life-threatening 10-year overall survival was 85.2%. The only cause of death in our
toxicities, being cytokine release syndrome (CRS) and immune effec- study was due to infections (14.6% [6/41 patients]). Only one patient,
tor cell-associated neurotoxicity syndrome (ICANS) frequent adverse with t(9;22) BCR-ABL translocation, relapsed after seven years and
effect. PICU admission is often required. Our main purpose is to attained remission with allogeneic stem cell transplantation.
describe clinical characteristics, treatment and outcomes of patients Conclusions: Our success rate is promising but the infectious complica-
admitted to PICU. tions are still the most common cause of death in developing countries
Methods: Prospective observational cohort study conducted in a ter- (etc. Turkey) during treatment of children with ALL.
tiary pediatric hospital from 2016-2021. Children who received CAR-T
therapy admitted to PICU were included. Epidemiological, clinical char-
acteristics, ICANS and CRS scales (ASTCT scale), supportive treatment, EP024 / #977 THERAPEUTIC MECHANISMS OF CD9
length of stay (LOS) and mortality variables were collected. NEUTRALIZING ANTIBODIES IN PEDIATRIC B-CELL
Results: CAR-T cells (41BB construct, 18 Tisa-cel/CTL019, 5 ARI- PRECURSOR ACUTE LYMPHOBLASTIC LEUKEMIA
0001 cells) were infused in 59 patients. Twenty-three patients (38.9%)
required PICU admission, LOS was 4 days (IQR 2-7) and hospital LOS John Tak Kit Cheung, Chi Zhang, Wing Hei Ng, Kathy Yuen Yee Chan,
24 days (IQR 16-38). Median age was 8 years (IQR 6-11). Patients Han Wang, Chi Kong Li, Kam Tong Leung
admitted to PICU presented higher tumoral burden before infusion: The Chinese University of Hong Kong, Department Of Paediatrics, Hong
23%(IQR 5-72) vs. 0(0-4), p<0.001. Main reasons for admissions were Kong, Hong Kong PRC
CRS (n=19, 82.6%) and neurotoxicity (n=3, 13%). Fourteen patients
(60.9%) required inotropic support, 13 (56%) received respiratory Background and Aims: Despite advances in CD19- and CD22-based
support, 5 of them mechanical ventilation. Regarding specific CAR-T immunotherapies for children with relapsed/refractory B-cell precur-
toxicities, 15 patients (65.2%) received tocilizumab, 4(17.4%) anakinra, sor acute lymphoblastic leukemia (BCP-ALL), a substantial proportion
4(17.4%) siltuximab and 10 (43.5%) steroids. Fourteen patients (60.9%) of cases subsequently relapse with antigen loss, underscoring the need
had neurological involvement, one of them was severe (ICANS 4). to develop salvage therapies. We recently demonstrated that neutral-
Patients who required inotropic drugs had higher procalcitonin and fer- izing antibodies against CD9 endowed potent single-agent activities
ritin levels (p<0.05). Two patients died during PICU stay (8.7%), both in patient-derived xenografts of refractory BCP-ALL and enhanced
because of refractory CRS-macrophage activation syndrome. There chemosensitivity. This study was designed to further characterize their
were no differences in relapse rate after CAR-T (p=0.396). underlying mechanisms.
Conclusions: PICU admission after CAR-T therapy is frequent, mainly Methods: Standard parameters of assessing cell death, including phos-
due to CRS. Supportive treatment allowed effective management of phatidylserine externalization, mitochondrial potential dissipation,
toxicities and high survival. Our data confirm that high tumoral burden cytochrome c release and caspase activation, were characterized by
is a risk factor for severe CRS and ICANS and thus PICU flow cytometry in BCP-ALL cell lines following exposure to anti-CD9.
Proteins shaping different modes of cell death was investigated by
Western blotting and compared with vincristine, coupled with genome-
EP023 / #845 ACUTE LYMPHOBLASTIC LEUKEMIA PATIENTS wide expression microarray, qPCR and functional validation to dictate
FROM A TERTIARY CENTER IN TURKEY the downstream pathways.
Results: Anti-CD9 significantly increased Annexin V+ cells compar-
Fatma Betul Cakir, Dilvin Çelik Ateş ing with IgG control. Loss of mitochondrial potential and release
Bezmialem Vakif University, Pediatric Hematology And Oncology, Istanbul, of cytochrome c were also witnessed following antibody exposure.
Turkey Surprisingly, anti-CD9 did not mediate changes in proteins related
to apoptosis (caspase-3/7/8/9, PARP, Bax/Bim/Bad/Bcl-2/Bcl-xL/Mcl-
Background and Aims: Acute lymphoblastic leukemia (ALL) is the 1), necroptosis (RIP/MLKL), autophagy (LC3/Beclin-1/Atg-5/Atg-7) or
most common type of cancer in children accounting for approximately mitophagy (DRP-1), whereas vincristine induced classical apoptosis
25% of all pediatric cancers and 5-year survival is approximately 85% reversible by a pan-caspase inhibitor. Anti-CD9 differentially regu-
in developed countries. We reviewed our ALL patients in order to lated 192 genes enriched with E2F transcription targets and G2M
evaluate their outcome and survival. cell cycle checkpoint. Up-regulation of DRAM1, IKZF2 and RANBP3L,
Methods: A total of 41 (16 female, 25 male) patients with ALL diag- and down-regulation of ARRDC3 were validated in 10 BCP-ALL cell
nosed between 2011 to 2022 were included in the study. All patients lines and 17 patient samples. Low expression of IKZF2 was associ-
were stratified and treated according to COG Protocols. ated with poor outcomes in a pediatric BCP-ALL cohort, and that
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S161 of S635

overexpression of IKZF2 significantly suppressed leukemia prolifera- EP026 / #113 EFFECTIVENESS OF A METHOTREXATE-FREE
tion, phenocopying the beneficial effects of anti-CD9. CHEMOTHERAPY REGIMEN IN LOW-RISK ACUTE
Conclusions: Anti-CD9 exhibits its leukemia suppressive activities via LYMPHOBLASTIC LEUKEMIA FOR UNINSURED HISPANIC
induction of a previously unrecognized form of cell death possibly CHILDREN
involving reactivation of the tumor suppressor IKZF2. This study col-
lectively provides the first mechanistic description of anti-CD9 in Julia Colunga Pedraza1 , Rodrigo Ortiz Neiva1 , Perla Colunga
BCP-ALL and a promising therapeutic target worth to be considered in Pedraza1 , Paola Friedrich2 , Hernán Ramírez Durán1 , Sergio Ramírez
future clinical trials. Cortinas1 , Ingrid López Reyna1 , Yajaira Valentine Jimenez Antolinez1 ,
Manuel De La O Cavazos3 , Oscar González Llano1
1 Hospital Universitario "Dr. José Eleuterio González", Universidad
EP025 / #415 TREATMENT OUTCOME OF CHILDREN WITH Autónoma de Nuevo León., Hematology, Monterrey, Mexico; 2 St.

ACUTE LYMPHOBLASTIC LEUKEMIA ACCORDING TO Jude Children’s Research Hospital, Oncology, Memphis, United States
RISK-GROUP STRATIFICATION: 20-YEAR PERIOD EXPERIENCE of America; 3 Hospital Universitario "Dr. José Eleuterio González",
IN A SINGLE INSTITUTION Universidad Autónoma de Nuevo León., Pediatrics, Monterrey,
Mexico
Eun Sang Yi1 , Young Bae Choi2 , Juyeon Lee2 , Na Hee Lee3
1 Korea University Guro Hospital, Korea University College of Medicine, Background and Aims: The high rates of toxicity and mortality
Pediatrics, Seoul, Korea, Republic of; 2 Ajou University Hospital, Ajou Uni- related to the treatment of patients with acute lymphoblastic leukemia
versity School of Medicine, Pediatrics, Suwon, Korea, Republic of; 3 Cha (ALL) in low- and middle-income countries (LMIC) have generated the
Bundang Medical Center, Cha University, Pediatrics, Sungnam, Korea, need to adapt treatment protocols according to the capacity of sup-
Republic of port therapy to less intensive regimens, especially in low-risk (LR)
patients. The primary objective of our study was to determine the
Background and Aims: Acute lymphoblastic leukemia (ALL) is the 3-year overall survival (OS) and event-free survival (EFS) in LR ALL
most common malignant disease in children and adolescents. In this protocol.
study, the treatment outcomes and prognostic factors of ALL patients Methods: One hundred and seven children were diagnosed
were investigated during a 20-year period in a single institution. with ALL from 2017 to 2019 in University hospital “José Eleu-
Methods: This study analyzed patients aged <19 years old who were terio González”. LR was defined as ≤6 years, with ≤20,000
diagnosed with ALL at Ajou University Hospital from January 2001 to leukocytes, without extramedullary infiltration, good response
December 2019. Patients were classified and treated under the low- to steroids, and minimal residual disease (MRD) ≤0.01% after
risk (LR), standard-risk (SR), high-risk (HR), and very HR (VHR) groups. the induction phase. The chemotherapy regimen on an out-
Results: Among 112 patients, there were 7 patients (6.3%) in LR, 37 patient basis includes 4 doses of anthracycline, 15 doses
(33.3%) in SR, 52 (46.4%) in HR, and 16 (14.3%) in VHR groups. Relapse of L-asparaginase, high-doses of methotrexate were not
occurred in 22 patients, with 15 occurring during ALL treatment and 7 included.
occurring within a median 18 months following the end of chemother- Results: Twenty-seven patients (34%) met LR criteria. The median
apy (range, 2–53 months). The 10-year relapse-free survival (RFS) and follow-up was 41 (17-58) months. Of 22 evaluated patients (81%), the
overall survival (OS) were 76.6 ± 4.5% and 83.3 ± 4.8%, respectively. karyotype was normal in 13 (59%), 5 (9%) had hyperdiploidy, and 4
The RFS and OS according to risk groups were as follows: LR: 68.6% were not analyzable quality. There were no induction deaths. Nine
± 18.6%, 80.0 ± 17.9%; SR: 83.5% ± 6.8%, 100%; HR: 76.1% ± 7.3%, patients (33%) received their chemotherapy scheme completely as out-
81.1 ± 5.8%; and VHR: 63.0% ± 13.3%, 63.3 ± 16.6%. On multivariate patients. Thirteen patients (48%) never had treatment interruption for
analysis, white blood cell count >200,000/mm3 at diagnosis (relative more than 7 days. The 3-year OS was 96.3%, and EFS was 82.4%. One
risk: 5.120, 95% confidential interval [CI] 1.454–18.028, P = 0.011) patient presented a bone marrow late relapse, and other presented an
and BCR/ABL mutation (relative risk: 3.863, 95% CI 10.98–13.591, P isolated early relapse to the CNS. Currently both alive and in a second
= 0.035) were associated with a lower RFS. On subgroup analysis, the remission. One patient died during maintenance, in remission, due to
LR group had a lower OS than the SR group (80.0 ± 17.9% vs 100%, P = viral pneumonia.
0.025). Conclusions: The treatment of low-risk Hispanic ALL patients
Conclusions: Our study indicated that patients with hyperleukocytosis with our adapted regimen showed promising results with
at diagnosis and BCR/ABL mutation exhibited a lower RFS. The OS of good 3-year OS and EFS. Also, it was possible to coun-
the LR group was lower than that of the SR group. Therefore, the treat- teract the lack of hospital beds and poor support therapy
ment for these patients should be modifed to improve the treatment in our environment due to low toxicity and no induction
outcomes. mortality.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S162 of S635 ABSTRACTS

EP027 / #397 LOW TOXICITY BORTEZOMIB-BASED Wik4 , Johan Malmros5 , Mats Heyman5 , Helene Hallböök6 , Goda
OUTPATIENT REGIMEN IN RELAPSED/REFRACTORY ACUTE Vaitkevičienė7 , Laimonas Griskevicius8 , Ólafur Gísli Jónsson9 , Kjeld
LYMPHOBLASTIC LEUKEMIA IN THE TARGETED THERAPY ERA Schmiegelow10 , Birgitte Klug Albertsen1
1 Aarhus University Hospital, Department Of Paediatrics And Adolescent
Julia Colunga Pedraza, Ingrid López Reyna, Andrés Gómez-De León, Medicine, Aarhus N, Denmark; 2 Uppsala University, Department Of
Perla Colunga Pedraza, Héctor Martínez-Espinosa, Victor Pharmacy, Uppsala, Sweden; 3 Oslo University Hospital, Department Of
Mingura-Ledezma, Galia Aliaga Orellana, Luz Barbosa Castillo, Yajaira Pediatric Hematology/oncology, Oslo, Norway; 4 Oslo University Hospi-
Valentine Jimenez Antolinez, Oscar González Llano tal, Department Of Haematology, Oslo, Norway; 5 Karolinska Institutet,
Hospital Universitario "Dr. José Eleuterio González", Universidad Autónoma Department Of Women’s And Children’s Health, Stockholm, Sweden;
de Nuevo León., Hematology, Monterrey, Mexico 6 Uppsala University Hospital, Department Of Medical Sciences, Haema-
tology, Uppsala, Sweden; 7 Vilnius University, Faculty Of Medicine, Clinic
Background and Aims: There are multiple treatment strategies Of Obstetrics And Gynecology, Vilnius, Lithuania; 8 Vilnius University
in patients with refractory or relapsed (R/R) acute lymphoblastic Hospital, Hematology, Oncology And Transfusion Medicine Center,
leukemia (ALL). Currently, the use of agents targeting surface receptors Vilnius, Lithuania; 9 Children’s Hospital, Landspitali, University Hos-
such as CD19, CD20, and CD22, including bispecific T cell engagers, pital, Children’s Medical Center, Reykjavik, Iceland; 10 Rigshospitalet,

monoclonal antibodies, and chimeric antigen receptor (CAR) T cells, Department Of Paediatrics And Adolescent Medicine, Copenhagen,
have shown promising results; however, their costs make their use Denmark
unaffordable in low or middle-income countries. Therefore, alternative
chemotherapy combinations and repurposed agents should continue Background and Aims: PEG-asparaginase is an indispensable part
to be explored. We aimed to describe our experience with a borte- of the multiagent treatment of acute lymphoblastic leukemia (ALL).
zomib (BZ) based regimen in children, adolescents, and young adults However, PEG-asparaginase treatment comes with substantial toxi-
diagnosed with R/R ALL. city leading to discontinuation of therapy, which threatens survival.
Methods: We performed a retrospective analysis between 2014-2020 The most common toxicity is hypersensitivity, defined as either clinical
of patients who received our bortezomib-based regimen. The reinduc- allergy or silent inactivation both associated with increased clear-
tion protocol consisted of BZ 1.3 mg/m2 /day on days 1, 4, 8, and 11; ance and inactivation of PEG-asparaginase. Pharmacokinetic (PK)
dexamethasone 10 mg/m2 /day for 21 days, vincristine 1.5 mg/m2 /day analyses are essential in detecting inactivation of PEG-asparaginase.
on days 1, 8, 15, and 22; doxorubicin 50 mg/m2 /day or mitoxantrone on Therefore, we aimed to investigate the possibility to identify PK
day 1 IV; L-asparaginase 6000 U/m2 (nine doses). Treatment response parameters to predict hypersensitivity before the allergic reaction
was assessed on day 30. occurred.
Results: We included 35 patients; the median age was 12 years (3- Methods: Patients with ALL aged 1-45 years treated according to
33). A complete response (CR) was achieved by 23 patients (65%); the ALLTogether Pilot Protocol from December 2018 to December
13 patients achieved CR with MRD negativity (CRxMRD). 17.1% 2021 in the Nordic and Baltic countries were eligible. A total of 2,228
achieved partial response after 1 cycle. No treatment-related mortal- samples from 256 patients were analyzed real-time for asparaginase
ity occurred, 21 patients (60%) were not hospitalized, and 24 (68%) enzyme activity (AEA) levels. A transit compartment model to char-
did not require blood component transfusions, 23 patients (65%) did acterize the PK of PEG-asparaginase was developed, representing
not have significant treatment-related adverse events. Indications for increased clearance over time. AEA was represented by the sum of all
hospitalization were neutropenic fever (n=9), one event of mild pan- compartments.
creatitis, and one event of superficial venous thrombosis of the upper Results: Inactivation of PEG-asparaginase was identified in 44 of 256
limb. One patient presented severe neurological toxicity with seizures. patients (17.2%); 9 patients with silent inactivation (20.5%), 12 patients
Eighteen patients (52.9%) underwent hematopoietic stem cell trans- with mild allergy (27.3%) and 23 patients with severe allergy (52.3%).
plantation consolidation, The 5-year OS and RFS were 41% and 37.2% Hypersensitivity mainly occurred after 4th or 5th dose (n=22 (50%)
in the whole group. and n=6 (13.6%), respectively). A 10-compartmental transit model that
Conclusions: The addition of BZ to a simple protocol of chemother- allowed an exponential increase in clearance over time described the
apy is effective, safe, economically, and logistically feasible in the low AEA-time profile best. The model divided patients into stable or expo-
resources setting. nentially increasing clearance over time. All patients with inactivation
of PEG-asparaginase demonstrated an increased clearance over time.
Patients with clinical symptoms demonstrated the most prominent
EP028 / #1227 PHARMACOKINETICS AND increase.
IMMUNOGENCICITY OF THE FIRST DOSES OF Conclusions: PK analysis of AEA enables early identification of
PEG-ASPARAGINASE changing clearance, which opens new possibilities to predict
inactivation of PEG-asparaginase contributing to improvement of
Merete Dam1 , Maddalena Centanni2 , Lena Friberg2 , Mats Karlsson2 , treatment.
Line Stensig Lynggaard1 , Inga Maria Johannsdottir3 , Hilde Skuterud
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S163 of S635

EP029 / #446 AN IMPLEMENTATION APPROACH TO related to process and structure were identified as opportunities for
CHARACTERIZE SUCCESSFUL FACTORS OF INTERNATIONAL improvement.
COLLABORATION IN A DRUG-DONATION PROGRAM AND Conclusions: As a result of this survey, additional structure will be
FACILITATION EFFORT established to improve transparency of processes. As new partners
join the project and their dynamics evolve, we will reevaluate col-
Caitlyn Duffy1 , Niharendu Ghara2 , Hiroto Inaba1 , Sima Jeha3 , laborator responses overtime. Integration of this tool provides an
Naureen Mushtaq4 , Gaurav Narula5 , Jennifer L. Pauley6 , Victor opportunity to identify areas for improvement and guide decisions
Santana7 , Liz Sniderman8 , Nickhill Bhakta9 , Heather Brandt10 to foster long-term, sustainable engagement amongst international
1 St Jude Children’s Research Hospital, Oncology, Memphis, United States collaborations.
of America; 2 Tata Medical Center, Department Of Pediatric Hematology
And Oncology, Kolkata, India; 3 St Jude Children’s Research Hospital, Oncol-
ogy, Department Of Global Pediatric Medicine, Memphis, United States EP030 / #1206 HIGH DOSE METHOTREXATE USE IN
of America; 4 Aga Khan University Hospital, Oncology, Karachi, Pakistan; CHILDREN WITH HIGH RISK ALL IN A LMIC- A 10-YEAR
5 Tata Memorial Centre, HBNI, Medical Oncology, Mumbai, India; 6 St Jude EXPERIENCE
Children’s Research Hospital, Global Pediatric Medicine, Memphis, United
States of America; 7 St Jude Children’s Research Hospital, Oncology, Global Ayesha Arshad Ali1 , Salman Soomar2 , Sadaf Altaf1 , Asim Belgaumi1 ,
Pediatric Medicine, Memphis, United States of America; 8 Stollery Children’s Naureen Mushtaq1 , Zehra Fadoo1
Hospital, Northern Alberta Children’s Cancer Care Program, Edmonton, 1 Aga Khan University, Dept Of Oncology, Aku, karachi, Pakistan; 2 Aga Khan

Canada; 9 St Jude Children’s Research Hospital, Oncology, Department Of University, Dept Of Emergency Medicine, Aku, karachi, Pakistan
Global Pediatric Medicine, Department Of Epidemiology And Cancer Con-
trol, Memphis, United States of America; 10 St Jude Children’s Research Background and Aims: High dose methotrexate has proven to be very
Hospital, Epidemiology And Cancer Control, Memphis, United States of effective in treatment of high-risk Precursor B Acute Lymphoblastic
America Leukemia in children. Cytotoxic concentrations in lymphoblasts can
cause severe adverse effects such as neutropenia, mucositis, renal and
Background and Aims: International partnerships and supply chain hepatic injury. Treatment related toxicity warrants precautions such as
innovation have been identified as promising solutions to bridge hyperhydration, monitoring levels, leucovorin rescue and urine alka-
the pediatric cancer survival gap. Collaborator engagement lever- linization. This study aims to provide tolerance of HDMTX in LMIC
ages complementary expertise to improve system level integration of setting.
evidence-based interventions. Despite recent emphasis on the crit- Methods: Records of patients with high risk ALL between 2010-
ical nature of engagement, there has been little formal reporting 2019 were reviewed retrospectively, data recorded in Resonance
of partnership composition. As part of a larger effort to evalu- study manager. All patients were treated with a uniform treatment
ate the implementation of blinatumomab in low- and middle-income regimen based on the BFM protocol. Interim maintenance included
countries, this study examined the experiences of collaborators in HDMTX 5gm/m2 biweekly for 4 doses. TALL patients were shifted
a blinatumomab drug-donation program after two years of program to Capizzi MTX. Study variables included sex, age, disease type,
involvement. blood counts, methotrexate levels, creatinine levels and liver function
Methods: We hypothesized that the collaborative approach estab- tests.
lished at program initiation would result in improved collaborator satis- Results: 372 doses of high dose methotrexate were administered to
faction. An electronic survey comprised of 47 questions was developed. 105 HR ALL patients, 72 (68.5%) males, median age of 9.5 years. Most
This included 40 close-ended questions from the Wilder Collaboration patients had B ALL(n=98, 93.3%), while 7(6.7%) had T-ALL. The median
Factors Inventory 2nd ed., a tool identifying 20 factors that influence methotrexate level at 24 hours was 7.14 (IQR 2.34-12), at 48 hours was
collaboration success. Responses were scored on a 5-point Likert-type 1.41 (IQR 0.98-4.56) & at 72 hours was 0.29 (IQR 0.02-4.2) umol/L. The
scale. mean hospital stay for administration of each dose of HD MTX was 4.8
Results: The survey was administered to 16 collaborators (14/16, days. The most common side effects were vomiting and diarrhea (n=20,
87.5% response rate), with diverse, multidisciplinary composition; 5.4%) followed by leukopenia (n=19, 5.1%), dermatological reactions
nurse practitioner (n=1), pharmacists (n=2), physicians (n=6) (n=18, 4.8%) and increased creatinine levels(n=15, 4.03%).Thrombo-
(United States [3], India [3], and Pakistan [1]), industry (n=4), cytopenia was reported in (n=7, 1.88%) and mucositis(n=4, 1.07%).
and non-governmental organization (n=1). Females comprised Median follow up time of the study group was 19.5 months (IQR 7.5-35
43% of respondents. Average scores >4 represented partnership months) with an overall survival of 70% (95% CI).
strengths. Twelve out of twenty collaborative factors received Conclusions: High dose MTX was well tolerated in our study pop-
scores >4.5. Factor ratings for “mutual respect and trust,” “shared ulation from LMIC with acceptable side effects needing aggressive
stake in process and outcome”, and “shared vision” scored highest support, adequate hydration and leucovorin rescue based on MTX
(>4.9) indicating strengths in the partnership with an overall pro- level monitoring. This encourages other centers in using HDMTX as
gram satisfaction of 9.28 on a 10-point scale. Ratings for factors standard of treatment for HR BALL.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S164 of S635 ABSTRACTS

EP031 / #1579 PEGASPARGASE LEVELS IN INFANTS WITH sisted when sub-analyzed by treatment cycle and age. There were no
ACUTE LYMPHOBLASTIC LEUKEMIA: A REPORT FROM THE pegaspargase hypersensitivity reactions reported.
CHILDREN’S ONCOLOGY GROUP TRIAL AALL15P1 Conclusions: This represents the largest cohort of infant ALL patients
with SAA levels reported. All reported levels were >/=0.1 IU/mL
Kelly Faulk1 , John Kairalla2 , Meenakshi Devidas3 , Emily Hibbitts2 , (proposed minimum therapeutic level) and the majority were >/=0.4
Andrew Carroll4 , Nyla Heerema5 , Holly Kubaney6 , Amanda August7 , IU/mL (proposed optimal therapeutic level), suggesting that, despite
Melinda Pauly8 , Daniel Wechsler8 , Rodney Miles9 , Joel Reid10 , Todd a reduced BSA-based pegaspargase dose compared to older chil-
Druley11 , Cynthia Kihei12 , Lia Gore1 , Elizabeth Raetz13 , Stephen dren, infants achieve adequate asparagine depletion. No SAA levels
Hunger14 , Mignon Loh15 , Patrick Brown16 , Erin Guest7 were indicative of silent inactivation and there were no patients with
1 University of Colorado, Children’s Hospital Colorado, Center For Can- hypersensitivity, highlighting that the asparaginase immune response
cer And Blood Disorders, Aurora, United States of America; 2 University relationship may differ in infants. Ongoing analysis will assess for cor-
of Florida, Biostatistics, Gainesville, United States of America; 3 St Jude relation of SAA levels with patient demographics and incidence of
Children’s Research Hospital, Global Pediatric Medicine, Memphis, United pegaspargase-associated toxicities.
States of America; 4 University of Alabama at Birmingham, Genetics, Birm-
ingham, United States of America; 5 Nationwide Children’s Hospital, Pathol-
ogy, Columbus, United States of America; 6 Dell Children’s Medical Center, EP032 / #287 DOES DIETARY ASPARAGINE AFFECT
Children’s Blood And Cancer Center, Austin, United States of America; PEGASPARGASE THERAPY AND THE GUT MICROBIOME? A
7 Children’s Mercy Kansas City, Hematology/oncology, Kansas City, United PILOT STUDY IN MICE
States of America; 8 Children’s Healthcare of Atlanta, Aflac Cancer And
Blood Disorders Center, Atlanta, United States of America; 9 University Zara Forbrigger1,2 , Ketan Kulkarni1,2 , Andrew Stadnyk1,3,4
of Utah, Pathology, Salt Lake City, United States of America; 10 Mayo 1 Dalhousie University, Pathology, Halifax, Canada; 2 IWK Health Cen-
Clinic, Division Of Oncology Research, Rochester, United States of America; tre, Division Of Hematology/oncology, Department Of Pediatrics, Halifax,
11 Washington University School of Medicine, St. Louis Children’s Hospital, Canada; 3 Dalhousie University/IWK Health, Pediatrics, Halifax, Canada;
Division Of Hematology And Oncology, St. Louis, United States of America; 4 Dalhousie University, Microbiology & Immunology, Halifax, Canada

12 St. Mary’s Medical Center, Pediatrics, West Palm Beach, United States
of America; 13 NYU Langone Health, Pediatrics, New York, United States Background and Aims: Pegaspargase (PEG) is key to the treat-
of America; 14 Children’s Hospital of Philadelphia, Pediatrics, Philadelphia, ment of pediatric Acute Lymphoblastic Leukemia. PEG depletes blood
United States of America; 15 Seattle Children’s Hospital, University of Wash- asparagine (Asn), killing leukemic but not healthy cells. Guidelines on
ington, Department Of Pediatrics, Seattle, United States of America; 16 The optimum dosing of PEG are unclear. Asn is made by healthy cells and
Johns Hopkins Hospital, Oncology, Baltimore, United States of America diffuses from the gut. We sought to determine if dietary Asn can impact
PEG-mediated Asn depletion and its association with gut bacteria.
Background and Aims: Asparaginase is an essential component of Methods: Pre-diet blood and stool samples from 10 mice were col-
therapy for acute lymphoblastic leukemia (ALL). Very limited data lected. The mice were then divided into 2 cages and given an Asn rich or
on pharmacokinetics and therapeutic drug monitoring using serum depleted diet. Blood and stool were sampled after 72 days (post-diet).
asparaginase activity (SAA) levels exist in infants. We aimed to describe The mice were then injected with 200 IU/kg PEG. Four days post-PEG
SAA levels in infants with ALL and correlate with age/dose or treatment stool and 5-day post-PEG blood samples were collected. Stool 16S
cycle. ribosomal DNA was sequenced. Blood Asn was measured using mass
Methods: AALL15P1 enrolled infants with ALL between 2017-2020, spectrometry.
treating with pegaspargase dosed at indicated units/m2 as follows: Results: Blood Asn levels were similar between post-diet samples. Asn
Induction: <7days 1250; 7days-6months 1750; 6-12months 2000; was depleted in all post-PEG samples. Analysing bacteria within a diet,
Post-induction: <6months 1650; 6-12months 1875; >/=12months the depleted diet had 36 species change in abundance between base-
2500. An optional study obtained SAA levels, recommended on day 7 line and post-diet, and the rich diet had 17. Of the 17, 8 changed in the
following administration. same direction for both diets. The depleted diet had 15 species change
Results: 78 patients enrolled on AALL15P1, and 34 patients submit- abundance from post-diet to post-PEG, the rich diet had 1. Comparing
ted 60 SAA levels. Levels were measured during Induction (n=30), diets, 29 species were different between groups at baseline, 11 post-
Interim Maintenance (n=17) and Delayed Intensification (n=13). Age diet, and 26 post-PEG. Three changed post-diet but were similar at
at the time of pegaspargase administration varied from 1-17 months. baseline, and 19 changed post-PEG that were similar post-diet.
There were no significant demographic differences between patients Conclusions: Blood Asn was depleted in mice with PEG regardless
with at least one level and those without. All SAA levels were >/=0.1 of dietary Asn. Thus, modifying dietary Asn before PEG is unlikely
IU/mL and the majority (n=55, 92%) were >/=0.4 IU/mL. Mean lev- to be impactful. Despite differences at baseline, we observed bac-
els increased with prescribed dose (and thus age), with mean levels of terial changes based on diet and PEG injection, which require fur-
0.57 IU/mL for <6 months (n=10), 0.82 IU/mL for 6-11 months (n=31), ther investigation. The impact of dietary Asn on PEG efficacy over
and 1.31 IU/mL for >/=12 months (n=19) (p<0.001). This trend per- a longer period should be explored, to determine whether Asn
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S165 of S635

depletion can be sustained and if changes in gut bacteria have any EP034 / #790 EARLY RESULTS AND COST-ANALYSIS OF
impact. MAS-ALL-18 TREATMENT GUIDELINE FOR ACUTE
LYMPHOBLASTIC LEUKEMIA IN MEXICO

EP033 / #1460 DEVELOPMENT OF RAPID MOLECULAR Pablo Gonzalez-Montalvo1,2 , Hugo Romo-Rubio3 , Daniela Arce
TESTING TO DIAGNOSE PEDIATRIC CANCERS IN Cabrera4 , Dinora Aguilar Escobar5 , Nataly Mercado-Cardenas6 , Karla
SUB-SAHARAN AFRICA Guerrero-Gomez6 , Naomi Echeandia-Abud7 , Paola Friedrich7
1 Hospital O’Horan, Servicios de Salud de Yucatan, Pediatric Oncol-
Julie Gastier-Foster1 , Emporia Hollingsworth2 , Dolores ogy Unit, Merida, Mexico; 2 Universidad Autonoma de Yucatan, School
Lopez-Terrada2 , Kevin Fisher2 , Angshumoy Roy2 , Peter Wasswa3 , Of Medicine, Merida, Mexico; 3 Hospital Civil de Guadalajara, Pediatric
Nmazuo Ozuah4 , Parth Mehta1 , Joseph Lubega1 , Carl Allen1 , David Oncology, Guadalajara, Mexico; 4 Hospital Pediatrico de Sinaloa, Hemato-
Poplack1 , Fredrick Lutwama5 oncology Unit, Culiacan, Mexico; 5 Hospital Infantil Teleton de Oncologia,
1 Texas Children’s Hospital, Global Hematology/oncology Pediatric Excel- Subdireccion De Diagnostico Y Banco De Sangre, Querétaro, Mexico; 6 Casa
lence, Houston, United States of America; 2 Texas Children’s Hospital, Pathol- de la Amistad para Niños con Cancer, Project Coordinator, Ciudad de
ogy, Houston, United States of America; 3 Global HOPE, Pediatrics, Kampala, Mexico, Mexico; 7 St. Jude Children’s Research Hospital, Global Pediatric
Uganda; 4 Texas Children’s, Global Hope, Lilongwe, Malawi; 5 Makerere Medicine, Memphis, United States of America
University, Biomedical Research Centre, Kampala, Uganda
Background and Aims: Acute Lymphoblastic Leukemia (ALL) was
Background and Aims: Risk stratification and molecular targeting treated until June 2019 in Mexico mainly using TOTAL-XV based pro-
have been key to increasing cure rates for pediatric cancers in high- tocols, with an early treatment-related mortality/abandonment rate
income countries. Precise diagnosis and successful treatment in low- (TRMR) of 12% during induction and 25% in the first year of treatment.
resourced settings is hindered by insufficient pathology infrastructure. Since July 2019, a treatment guideline based on strategic deintensifica-
The Global HOPE program aims to improve outcomes for pediatric tion of therapy in carefully selected patients, based on genetic profiling
cancer in Sub-Saharan Africa (SSA). The goal of this study was to and minimal residual disease, has been used in 4 centers within the
develop rapid molecular assays to improve pediatric cancer diagnoses Mexico in Alliance with St. Jude (MAS) collaborative network.
in low-resourced settings. Methods: We compared our preliminary results with the MAS-ALL-18
Methods: The NanoString nCounter platform was chosen due to mini- treatment guideline with results from ALL patients treated at MAS-
mal technical expertise required, ability to test sub-optimal RNA, and affiliated centers prior to July 2019, focusing on early TRMR at end
turn-around-time (2-3 days). To address the high frequency of gene of induction and during the first year of therapy. We also calculated
fusions in pediatric cancers, custom panels were designed to detect approximate chemotherapy expenditure per patient for TOTAL-XV
439 specific breakpoints of fusions associated with hematologic malig- standard and high-risk, and MAS-ALL-18 favorable, intermediate and
nancies and 436 fusions for solid tumors. The NanoString Cancer Copy high-risk, and estimated cost per 100 patients considering risk-group
Number Variation (CNV) assay was tested to determine the effective- assignment distribution documented for both cohorts.
ness of detecting aneuploidy and gene amplification, also common in Results: Our cohort prior to July 2019 included 578 patients, of which
pediatric cancers. 18.7% were treated as standard and 81.3 as high-risk. We previ-
Results: Validation of the custom gene fusion panels was performed ously reported early results from a MAS-ALL-18 cohort, among which
using >140 samples with known fusion status. The results had a 49.5% of patients were classified as favorable, 20.8% as intermedi-
99% accuracy after follow-up sequencing of any potential false nega- ate and 29.7 as high-risk. Early TRMR for 138 MAS-ALL-18 patients
tive/positive results. Testing the CNV assay confirmed that amplifica- who have completed 1 year of treatment was 7.4% during induc-
tion and CNV in a diploid background are easily detectable in pediatric tion, and 15.4% during the first year of treatment. Chemotherapy
cancer samples. In the presence of high chromosome number, an alter- expenditure per patient was estimated at 10,353.51USD for stan-
native analysis algorithm is required to reset the background ploidy, dard and 15,510.78USD for high-risk TOTAL-XV and 8,008.00USD
similar to microarray analysis of aneuploid cancer samples. for favorable, 13,041.25USD for intermediate and 14,774.38USD for
Conclusions: Validation studies of the NanoString assays confirmed high-risk MAS-ALL18 patients. Chemotherapy expenditure per 100
them as highly accurate, tolerant of variable nucleic acid quality, and patients, considering risk-group assignment distribution documented
requiring small sample amounts. Global HOPE is currently implement- for both cohorts, was estimated at 1,454,637.05 for TOTAL-XV and
ing the platform in SSA, starting with the custom gene fusion panels. 1,106,441.80 for MAS-ALL-18.
Testing will allow children in SSA to be treated according to the specific Conclusions: MAS-ALL-18 has proven to have lower early TRMR at
molecular characteristics of their cancers to maximize their chances end-of-induction (7.4 Vs 12%) and after one year of therapy (15.4 Vs
of a cure, the same approach now considered standard-of-care in the 25%), whilst permitting an estimated 24% cost reduction in chemother-
US. apy expenditure.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S166 of S635 ABSTRACTS

EP035 / #1003 RB TRANSCRIPTIONAL COREPRESSOR 1 Russian Federation; 2 Russian Medical Academy of Continuing Professional
(RB1) GENE DELETIONS ADVERSELY AFFECT THE OUTCOME IN Education, Ministry of Health of Russia, Clinical Pharmacology And Therapy,
PEDIATRIC B-CELL ACUTE LYMPHOBLASTIC LEUKEMIA Moscow, Russian Federation

Sanjeev Kumar Gupta1 , Sameer Bakhshi2 , Ritu Gupta1 , Preity Background and Aims: Acute lymphoblastic leukemia (ALL) in chil-
Sharma1 , Smeeta Gajendra1 , Gadha Leons1 , Deepam Pushpam2 dren is a potentially curable disease, but patients still suffer adverse
1 AIIMS, New Delhi, Laboratory Oncology, Delhi, India; 2 AIIMS, New Delhi, drug reactions that sometimes require dose reduction or discontin-
Medical Oncology, Delhi, India uation of cytostatics and contribute to a decrease in overall therapy
effectiveness. One of the main drug used in ALL therapy protocols
Background and Aims: There is a need for newer biomarkers to is methotrexate, which treatment is complicated by the develop-
improve the risk-stratification of B-ALL. The aim of the study was to ment of hepatotoxicity (HT). HT can be caused by various genetic
estimate the prevalence and effect of RB transcriptional corepres- polymorphisms as seen with ABCB1 and SLCO1B1. In this study,
sor1(RB1) deletion in childhood B-ALL, when present alone or along we examined the relationship of single nucleotide polymorphisms
with IKAROS family zinc finger1 (IKZF1) deletion. (SNPs) of the SLCO1B1 (T521C rs4149056) and ABCB1 (rs4148738
Methods: The pediatric B-ALL cases were analyzed for RB1 and C>T, rs2032582, rs1128503, C3435T rs1045642) genes with the
IKZF1 deletions using multiplex ligation-dependent probe amplifica- development of clinically relevant HT in children treated with high-
tion(MLPA). dosed (>1g/m2) methotrexate. Aim. To evaluate the association of
Results: There were 357 pediatric B-ALL patients with a median pharmacogenetics (PG) indexes with HT of methotrexate during ALL
age of 7 years(1-18). This included 320 BCR-ABL1 negative and 37 therapy.
BCR-ABL1 positive patients. Overall, 33/357(9.2%) patients had RB1 Methods: 67 children with ALL who received methotrexate therapy
deletions (RB1del); 30/320(9.4%) BCR-ABL1 negative and 3/37(8.1%) with ALL IC-BFM 2009 protocol were enrolled the study. To assess
BCR-ABL1 positive. The IKZF1 deletions (IKZF1del) were present the side effects, the patients were evaluated using laboratory meth-
in 70(19.6%) patients. The concomitant RB1 and IKZF1 deletions ods and NCI toxicity scales (CTCAE v5.0 2018). We used a real-time
(RB1del/IKZF1del) were present in 11(3.1%) cases. RB1 deletions PCR method to study gene’s SNP. The material was a peripheral
alone in absence of IKZF1 deletions (RB1del/IKZF1wt) were seen blood. Material was sampled once, irrespective of the duration of
in 22 cases. Their comparison was done with 59 cases with IKZF1 methotrexate therapy.
deletions alone with no RB1 deletion (RB1wt/IKZF1del) and cases Results: Mean age of the patients was 7.56±4.8. The HT stage 1-2st.
without any RB1 or IKZF deletions (RB1wt/IKZF1wt). The post- was in 44.8%, 3-4st. - 55.2%. Toxicity was assessed in the postcy-
induction remission rate (55.6%;p=0.052) was worst in the group tostatic period, the relationship with polymorphisms of these genes
with both RB1 and IKZF1 deletion (RB1del/IKZF1del) compared with was assessed using contingency tables, as follows: Pearson’s χ2 for
81.8% in RB1del/IKZF1wt, 78.7% in RB1wt/IKZF1del & 86.6% in ABCB1C 3435T rs1045642 and GT was 6.7, p-value 0.347, for ABCB1
RB1wt/IKZF1wt. The median EFS (8.9 months;p=0.0001) and median rs2032582 – 9.206, p-value: 0.162, for ABCB1 rs1128503 – 6.692, p-
OS (11.5 months;p=0.007) were least in the RB1del/IKZF1del group value 0.669, ABCB1 rs4148738 – 7.582, p-value 0.577, for SLCO1B1
compared to RB1del/IKZF1wt (28.5, 41.2 months), RB1wt/IKZF1del T521C rs4149056 – 5.919, p-value 0.432. Thus, the compared signs
(22.6, 30.3 months) and RB1wt/IKZF1wt (48.8 months, median OS not were statistically unsignificant.
reached) respectively. Conclusions: Determination of polymorphisms of genes providing
Conclusions: RB1 deletions were seen in 9.2% with similar distribution cytostatic transport and metabolism, PGx aspects of toxicity, is a
in BCR-ABL1 negative and positive B-ALL subgroups. The concomitant promising and dynamically developing area of clinical oncology.
RB1/IKZF1 deletions were seen in 3.1% of B-ALL patients. The patients
with concomitant RB1/IKZF1 deletions had much poorer outcomes
than cases without any of these deletions or having RB1 deletions or EP037 / #996 PATTERNS AND OUTCOMES OF ACUTE
IKZF1 deletions alone. Thus, the presence of RB1 deletions and IKZF1 CENTRAL NERVOUS SYSTEM COMPLICATIONS DURING
deletions, particularly if present together, can be used as a predictive TREATMENT OF CHILDHOOD ACUTE LYMPHOBLASTIC
marker for poor outcome in B-ALL. LEUKEMIA, NATIONAL CANCER INSTITUTE-CAIRO UNIVERSITY
EXPERIENCE

EP036 / #1220 ANALYSIS OF PHARMACOGENETIC Asmaa Hamoda1 , Samah Semary2 , Mohammad Bedair3 , Ayda
INDICATORS OF METHOTREXATE HEPATOTOXICITY DURING Youssef4 , Hanafy Hafez5
TREATMENT ACUTE LYMPHOBLASTIC LEUKEMIA IN CHILDREN 1 National Cancer Institute - Cairo University, Children’s Cancer hospital
Egypt 57357, Pediatric Oncology, cairo, Egypt; 2 clinical oncology, Beni-suef
Timur Valiev1 , Oxana Gurieva1 , Marina Savelyeva2 University, Children’s Cancer hospital Egypt 57357, Clinical Oncology, cairo,
1 Pediatric Oncology and Hematology Research Institute of N.N.Blokhin Egypt; 3 Damietta Cancer Institute,egypt, Pediatric Oncology, cairo, Egypt;
National Research Cancer Center, Hemoblastoses Chemotherapy, Moscow, 4 National Cancer Institute -cairo university, Radiodiagnosis, cairo, Egypt;
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S167 of S635

5 National Cancer Institute - Cairo University, Children’s Cancer hospital Background and Aims: Chimeric antigen receptor (CAR) T-cell ther-
Egypt 57357, Pediatric Oncology, Cairo, Egypt apy is a novel treatment for children with relapsed or refractory acute
lymphoblastic B leukemia. Although complete remission rates are high,
Background and Aims: Central nervous system (CNS) complications it involves serious health risks. The most frequent complications are
are heterogeneous, varying from very mild and transient symptoms to cytokine release syndrome (CRS) and neurotoxicity. Almost 30-50%
extremely severe and debilitating or even lethal syndromes.The study of pediatric patients require intensive treatment and close monitor-
aimed to assess the incidence, risk factors, patterns, and outcomes ing in pediatric intensive care units (PICU). The aim of this study is
of different CNS complications during treatment of pediatric acute to describe CAR-T patients’ clinical characteristics and follow up in a
lymphoblastic leukemia (ALL) Tertiary Pediatric Hospital.
Methods: A retrospective study included 390 patients with ALL, Methods: Retrospective study including CAR-T infusions between Jan-
treated according to St. Jude total XV protocol at National Cancer uary 2020 and March 2022. Epidemiological and clinical data were
Institute, Cairo University between January 2012 to December 2017. collected.
Clinical and radiological findings of all patients, were reviewed and Results: Ten patients received CART therapy. The median age was
analyzed. 8.8 years (1.25-20.8). There were nine cases of bone marrow relapse
Results: among the whole study cohort, there were 39(10%)patients and one combined relapse. Two primary refractory leukemias, four
diagnosed with different types of CNS complications.Cerebrovascular refractory relapses and four second leukemia relapses. 100% of second
Events were the most frequent, diagnosed in 19 (4.9%) patients, relapses were after hematopoietic stem cell transplantation (HSCT).
12(3.1%)patients diagnosed with posterior reversible encephalopathy The median time to relapse after HSCT was 13 months (12-17) and
syndrome (PRES), and 6 (1.5%) patients diagnosed with leukoen- the median minimal residual disease prior to infusion was 23.6% (0.46-
cephalopathy, Both CNS infections and leukemic infiltrates were 76.7%). Seven patients developed CRS, 57% grade 3 or more. Three
diagnosed in one patient each. The incidence of CNS complica- patients developed neurotoxicity, 66% grade 3 or more. Seven cases
tions was significantly higher in patients older than 10 years old(P (70%) required PICU admission, two of them only for monitoring.
value 0.04), those with initial high-risk disease, and in patients who Hemofiltration was necessary in three cases. Five cases required cor-
were classified as CNS III status Most of patients (79.5%) with ticoisteroids, six Tocilizumab, four Siltuximab and two Anakinra. Four
CNS achieved complete recovery, while 6(15.4%) patients died, and patients relapsed after infusion (100% CD19-) and two lost B-cell
2(5.1%) patients developed residual neurological deficits. Regarding aplasia during the next months. 100% suffered from hypogamma-
the onset of CNS complications, there were 15 (38.4%) patients globulinemia. One patient died by fulminant CRS and three due to
who had complications during the induction of remission phase of progression. Nine patients achieved complete remission (CR). At the
treatment, 12 (30.8%) patients had complications during the early moment six patients are alive with CR. The median of follow up is 10
maintenance phase of treatment, 9 (23.1%) patients had compli- months (7-20).
cations during the late maintenance phase of treatment, and 3 Conclusions: In patients with relapse or refractory ALL, CAR-T cell
(7.7%) patients had complications during the consolidation phase of therapy has achieved lasting remission. These patients often need
treatment PICU admission and monoclonal antibody treatment due to its side
Conclusions: Patients with older age at presentation,high-risk dis- effects and complications.
ease,and initial CNS III status were at higher risk of developing acute
CNS complications during treatment of childhood ALL. Despite of high
recovery rate in patients with CNS complications, still 15% of them died EP039 / #610 TREATMENT OUTCOME IN PEDIATRIC ACUTE
that warranted prompt intervention. LEUKEMIA WITH HYPERLEUKOCYTOSIS AT HOSPITAL
INFANTIL DE MÉXICO FEDERICO GÓMEZ

EP038 / #1703 CLINICAL AND MANAGEMENT OF PATIENTS Miguel Angel Palomo Colli, Pamela Taquichiri Cruz, Maria Fernanda
WITH CAR-T THERAPY IN A TERTIARY PEDIATRIC HOSPITAL Hidalgo Martinez, Luis Enrique Juarez Villegas, Marco Murillo
Maldonado
Rocio Vila De Frutos1 , Blanca Herrero Velasco2 , Sara Vinagre Hospital Infantil de México Federico Gómez, Oncology, Ciudad de México,
Enríquez3 , Ana Moral Larraz4 , Ines Leoz Gordillo1 , Veronica Cantarin Mexico
Extremera5 , Montserrat Nieto Moro4 , Luis Madero López3
1 HOSPITAL INFANTIL UNIVERSITARIO NIÑO JESÚS, Hematooncology, Background and Aims: Hyperleukocytosis caused by acute leukemia
MADRID, Spain; 2 HOSPITAL INFANTIL UNIVERSITARIO DEL NIÑO JESUS, (AL) is associated with early morbidity and mortality due to hyper-
Oncology, MADRID, Spain; 3 Hospital Infantil Universitario Niño Jesús, viscosity arising from the excessive number of leukocytes. This study
Oncología Pediátrica, Madrid, Spain; 4 HOSPITAL INFANTIL UNIVERSI- was designed to assess the incidence of hyperleukocytosis, survival
TARIO NIÑO JESÚS, Intensive Care, MADRID, Spain; 5 HOSPITAL INFANTIL outcomes, and adverse features among pediatric AL patients with
UNIVERSITARIO NIÑO JESÚS, Neurology, MADRID, Spain hyperleukocytosis.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S168 of S635 ABSTRACTS

Methods: Between January 2018 and December 2020, 52 children patients had CNS involvement, and there was only one patient in group
with previously untreated AL and hyperleukocytosis were enrolled at 2 who relapsed with CNS. One patient died in each of the two groups,
the Hospital Infantil de México Federico Gòmez The medical charts of and the cause of death was septic shock during neutropenic state.
these patients were retrospectively reviewed. The 5-year event-free survival (EFS) was 88.1±5.0% and 91.9±4.6%
Results: Of the 52 children with AL 35 had initial leukocyte counts of (p=0.336) in group 1 and group 2, respectively. The 5-year overall sur-
>100×10(9)/L, and 9 patients had a leukocyte count of >250×10(9)/L. vival (OS) was 95.2±3.3% and 95.7±4.3% (p=0.593) in group 1 and
and 8 with leukocite count of > 400 x 10(9)/L. Eighty-eight had acute group 2, and there were no statistically significant. Comparing the
lymphoblastic leukemia and twelve acute myeloid leukemia. Thirty- toxicity, the rate of hospitalization due to delayed chemotherapy or
one were females (59.6%) . Three had SNC3 status at diagnosis, 9 neutropenic fever was higher in group 1, but it wasn’t statistically sig-
were unclassified. Common early complications during induction ther- nificant. Although the rate of hepatotoxicity was high in the 2 group,
apy included tumor lysis syndrome (63.5%) renal dysfunction (28.8%), it didn’t require hospitalization, and other toxicities were favorable,
respiratory distress (28.8%) and central nervous system hemorrhage resulting in a relatively low hospitalization rate.
(5.8%). Infectious were presents in 36.5% of the cases. All patients Conclusions: In our study, escalating IV MTX without leucovorin rescue
were treated with hyperhidration, urinary alcalinization, blood trans- during the IM phases didn’t improved EFS compared with standard IM
fusions, antibiotics if they required. Eighteen(34.6%) were treated with regimen with oral MTX.
leukapheresis and 11 required hemodyalisis. Twelve patient died, 6 in
the group of leukapheresis. The reduction of leukocite counts were
observed in 15 patient, in three there was no reduction. In the group EP041 / #679 INDUCTION FAILURE IN CHILDREN WITH
on acute lymphoblastic leukemia 37 were responders to prednisone. ACUTE LYMPHOBLASTIC LEUKEMIA IN JAPAN:
The complete remission (CR) rate for the pediatric AL patients with CCLSG/KYCCSG/JACLS/TCCSG JOINT STUDY
hyperleukocytosis was 86% . The estimated 3-year overall survival of
AL children with hyperleukocytosis was 64.3% However in the group Chihaya Imai1 , Atsushi Sato2 , Mitsuteru Hiwatari3 , Yasuto
of patients with leukapheresis the overall survival was 49.6%. and 69% Shimomura4 , Toshinori Hori4 , Souichi Suenobu5 , Toshihiko Imamura6 ,
in patients without leukapheresis. Junichi Hara7 , Daisuke Hasegawa8 , Hiroyuki Takahashi9 , Daisuke
Conclusions: The optimal management hyperleukocytosis is still uncer- Tomizawa10 , Hiroshi Moritake11 , Takashi Taga12 , Keizo Horibe13 ,
tain, and there are no randomized studies demonstrating one is Katsuyoshi Koh14 , Atsushi Manabe15 , Yasuhiro Okamoto16
superior to each other. 1 Niigata University Graduate School of Medical and Dental Sciences,
Pediatrics, Niigata City, Japan; 2 Miyagi Children’s Medical Center, Hematol-
ogy/oncology, Sendai, Japan; 3 Graduate School of Medicine, The University
EP040 / #562 TREATMENT OUTCOME FOR ESCALATING of Tokyo, Department Of Pediatrics, Tokyo, Japan; 4 Aichi Medical Univer-
INTRAVENOUS METHOTREXATE IN CHILDREN WITH sity, Pediatrics, Nagakute, Japan; 5 Oita University, Pediatrics, Yufu, Japan;
STANDARD-RISK ACUTE LYMPHOBLASTIC LEUKEMIA: A 6 Kyoto Prefectural University of Medicine, Graduate School of Medical
SINGLE-CENTER ANALYSIS Science, Pediatrics, Kyoto, Japan; 7 Osaka City General Hospital, Pediatric
Hematology And Oncology, Osaka, Japan; 8 St. Luke’s International Hospital,
Ju Kyung Hyun, Young Tak Lim Pediatrics, Tokyo, Japan; 9 Toho University Omori Medical Center, Pediatrics,
Pusan National University Yangsan Hospital, Korea, Pediatrics, Yangsan, Tokyo, Japan; 10 National Center for Child Health and Development, Chil-
Korea, Republic of dren’s Cancer Center, Tokyo, Japan; 11 Faculty of Medicine, University of
Miyazaki, Pediatrics, Miyazaki, Japan; 12 Shiga University of Medical Sci-
Background and Aims: Recently, survival rate have significantly ence, Pediatrics, Otsu, Japan; 13 Nagoya Medical Center, Clinical Research
improved for children with ALL, with 5-year survival rates over 90%, Center, Nagoya, Japan; 14 Saitama Children’s Medical Center, Hematol-
especially in standard risk (SR) group. This report analyzed the out- ogy/oncology, Saitama, Japan; 15 Hokkaido University Graduate School of
come of children treated on escalating IV methotrexate (MTX) without Medicine, Pediatrics, Sapporo, Japan; 16 Kagoshima University Graduate
leucovorin rescue during the 2 cycle of interim maintenance (IM) School of Medical and Dental Sciences, Pediatrics, Kagoshima, Japan
phases, compared with existing standard IM regimen.
Methods: From January 2010 to December 2020, patients diagnosed Background and Aims: Reports from the Western countries showed
and treated for SR ALL at Pusan National University Children’s Hos- subjects who failed to achieve complete remission after induction
pital were included. Patients diagnosed before September 2015 were chemotherapy (‘induction failure’ [IF]) survived poorly even in the mod-
treated with standard IM regimen and defined as group 1, and all sub- ern era. The characteristics and prognosis of subjects who experienced
sequent diagnosed patients were treated with escalating IV MTX and IF have not been poorly understood in Japan.
defined as group 2. The results was analyzed in these two groups. Methods: The clinical data of patients, who were enrolled in CCLSG
Results: Total 100 standard-risk ALL patients were diagnosed. Among ALL2000, CCLSG ALL2004, KYCCSG ALL96, KYCCSG ALL02, JACLS
100 patients, 42 patients were included in group 1, and the remaining ALL 97, JACLS ALL-02, TCCSG L99-15, or TCCSG L04-16 (during
52 were included in group 2. At the time of initial diagnosis, none of the the period from 1996 to 2009) and failed to achieve remission after
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S169 of S635

one course of induction therapy, were retrospectively collected and lyzed the BMI Z-score changes over the course of treatment for the
analyzed. main risk factors related with obesity (sex, age, and ALL risk at diagno-
Results: Eighty-nine patients with IF (1.8%) were identified among sis), in which no significant differences were found. However, a positive
4,956 participants. The 5-year overall survival (5y-OS) was 43.0% +/- correlation was found between the first and last Z-score means. (r=
5.5% (95%CI: 32.0%-53.4%). No difference was found in the survival 0.567, p<0.0001).
rates between patients with B-ALL and T-ALL. In the B-ALL cohort Conclusions: Ideally ALL survivors should be healthy children by the
(n=64), 5y-OS was significantly higher in NCI-SR Patients than NCI-HR end of treatment, it is important that we focus in the prevention of
patients (p=0.031). In the T-ALL cohort (n=25), 5y-OS was significantly overweight and obesity of these patients from the diagnosis of disease.
lower in patients with CNS-3 than those without (p=0.017). Due to
limited data availability, we were unable to analyze the effect of re-
induction chemotherapy on survival. We found no survival benefit in EP043 / #349 ASSESSMENT OF DISSEMINATED
the use of allogeneic SCT. INTRAVASCULAR COAGULATION IN CHILDREN DURING WITH
Conclusions: The IF rate in the current study seems comparable to that ACUTE LYMPHOBLASTIC LEUKAEMIA DURING INDUCTION
observed in the Ponte di Legno international collaborative study (2.4% CHEMOTHERAPY IN A TERTIARY CARE HOSPITAL IN
among 44,017 patients; NEJM 2012). The 5y-OS in the current study BANGLADESH
was also comparable to that reported by the Ponte di Legno group
study (5y-OS 32% +/- 1%). Md Anwarul Karim, Sharmin Akter, Chowdhury Yakub Jamal
Bangabandhu Sheikh Mujib Medical University, Pediatric Hematology And
Oncology, DHAKA, Bangladesh
EP042 / #1134 WEIGHT VARIABILITY IN A PEDIATRIC
POPULATION DURING TREATMENT OF ACUTE Background and Aims: The overall cure rate for childhood acute
LYMPHOBLASTIC LEUKEMIA WITH A GRADUATED INTENSITY lymphoblastic leukemia has improved from virtually zero to the cur-
PROTOCOL FOR LOW-INCOME COUNTRIES rent event-free survival rate (EFS) of more than 90%. Disseminated
intravascular coagulation is the commonest hemostatic abnormality
Yajaira Valentine Jimenez Antolinez, Hernán Ramírez Durán, in patients with acute lymphoblastic leukemia. It might cause seri-
Samnatha Fierro Saenz, Lilia Edith Carrizales Torres, Juan Molina De ous hemorrhagic complications and warrants proper medical attention
La Garza, Nestor Ibarra Salazar, Lesly Cruz Santana, Julia Colunga in due time. Aim of the study was to detect the frequency, identify
Pedraza, Oscar González Llano the risk factors and outcome of disseminated intravascular coagula-
Hospital Universitario Dr. Jose Eleuterio Gonzalez UANL, Pediatric Hema- tion in children with acute lymphoblastic leukemia during induction
tology, Monterrey, Mexico chemotherapy.
Methods: This prospective observational study was carried out in
Background and Aims: Obesity is a well-documented problem associ- 55 diagnosed cases of ALL children in the department of pediatric
ated with childhood acute lymphoblastic leukemia (ALL) with increas- hematology and oncology, Bangabandhu Sheikh Mujib Medical Uni-
ing body mass index often observed during therapy, and it can diminish versity, Bangladesh from November 2020 to October 2021. Patients
the quality of life of survivors. Our objective was to describe the were kept on follow-up with risk-directed UKALL 2003 regimen.DIC
weight variability during treatment of ALL patients, and to identify was detected by using the International Society of Thrombosis and
which subgroups were affected by overweight and obesity at the end Haemostasis scoring system by using Prothrombin time, Platelet count,
of therapy. S. fibrinogen, and D-dimer at baseline, on day 7, 14, and 21. Statistical
Methods: Records of pediatric patients between the ages of 2 and 15 analysis was obtained by using SPSS-version 22.
years with ALL between 2011 and 2015 at the Hospital Universitario Results: Out of 55 patients, DIC was encountered in 12 (21.8%)
in Monterrey, Mexico were reviewed. We evaluated weight, and height patients. At diagnosis, DIC was found in 8 patients (14.54%), on day 7,
at each treatment phase, and from the beginning of follow-up until the DIC was found in 4 patients (8%).A total of 8(14.54%) patients have
last visit to the clinic. Patients were classified in four groups using BMI developed DIC at diagnosis, among them 4(50%) had persisted DIC
z-score adjusted by age and sex using WHO tables. after starting chemotherapy and 4 patients resolved. But, 4 patients
Results: Of the remaining 80 patients, median age was 5 (2-15) years, (8.5%) newly developed DIC during hospitalization (p=0.001) on day 7.
of which 45(56%) were male, and 35(44%) were female. At diagnosis The use of Daunorubicin had 6.252 times significantly increased risk to
45(56%) had high-risk disease, and 36(44%) where low risk. Median developed DIC. Patients with DIC had more bleeding 10(83.3%) than
follow up in months was 74.5 (1-121). BMI z-score increased from the non-DIC group 20(46.6%). The mortality rate was higher,3(25.0%)
induction to consolidation (mean= 0.34 ± 0.1, p< 0.001), from consol- in the DIC group and 6(14.0%) in the non-DIC group.
idation to intermediate maintenance (mean= 0.32 ± 0.07, p<0.0001), Conclusions: The frequency of DIC was 21.8% during the induc-
and from intensification to the first year after diagnosis (mean=0.23 ± tion period, use of daunorubicin was identified as the risk factor
0.1, p=0.03). BMI increased from diagnosis to follow-up (mean=0.81± for the development of DIC. Mortality was higher in patients with
0.19, p<0.0001) In a subgroup of 52 (65%) survivor patients, we ana- DIC.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S170 of S635 ABSTRACTS

EP044 / #1163 OUTCOMES OF CHILDREN WITH RELAPSED Rahmadani Lestari1 , Claudia Adelin1 , Alexandra Pangarso1 , Sutaryo
ACUTE LYMPHOBLASTIC LEUKEMIA- EXPERIENCE FROM INDIA Sutaryo1 , Anjo Veerman1,2
1 Universitas Gadjah Mada, Department Of Pediatrics, Yogyakarta, Indone-
Upasana Karthik, Dhwanee Thakkar, Anjali Yadav, Sunisha Arora, sia; 2 Emma Children’s Hospital, Amsterdam UMC, Vrije Universiteit Amster-
Neha Rastogi, Satya Yadav dam, Pediatric Oncology, Amsterdam, Netherlands
Medanta, The Medicity Hospital, Pediatric Haematology- Oncology And
Bone Marrow Transplantation, Gurugram, India Background and Aims: Lost to follow up (LOFU), defined as
no contacts after cessation of maintenance treatment is one of
Background and Aims: Paediatric Acute Lymphoblastic the obstacles to evaluate the effectiveness of ALL - protocols.
Leukemia(ALL) is highly curable with the current treatment, but In the low- and middle- income countries (LMIC), there is still
about 15%patients relapse after an initial response. The published inequality to access health facilities or communication services.
5year OS ranges between 36- 50% after first relapse. These figures We would like to describe the feasible way to contact the LOFU
are further affected in developing countries where treatment cost, patients.
infections and abandonment are major hindrances to successful Methods: Data were obtained from 362 patients diagnosed with ALL
outcomes. Data from developing world is meagre. We aim to discuss from February 2013 until November 2018 who were treated for 2
the outcomes of children with relapsed-ALL. years using ALL-Protocols 2013 and 2016 in Sardjito General Hos-
Methods: Medical records of children diagnosed as first relapse of pital, Yogyakarta, Indonesia, a LMIC. All LOFU patients who had not
ALL between October2016 and December2021 were retrospectively visited the clinic for more than 6 months between January - June
analysed for outcomes. Patients were risk-stratified and initiated on 2021 were contacted by short message, mobile phone call, or let-
treatment as per BFM-REZ-2002. Treatment was modified based on ters to the parents or to the local Health Centers if no response was
patients’ condition and response to therapy. Targeted therapy aided obtained.
remission achievement in 7patients. Category S3, S4 and MRD>0.1% Results: A total of 182 patients were off therapy. The median time
by Flowcytometry post F1+F2 were considered indications for HSCT. from diagnosis was 4 years. There were 111/182 (61%) LOFU patients.
Results: We managed 23patients of first relapse of ALL, with Among them, 93/111 (83.8%) were successfully contacted, 68/111
median age 9years(range:3-16years)(Males-18, Females-5). ALL (61.3%) by short message and mobile phone call, 25/111 (22.5%) via
Phenotype: precursor-B-cell-19/23, T-cell-4/23. Original disease letter, and no response was obtained from 18/111 (16.2%) patients.
treatment comprised of BFM-95protocol-15patients, ICICLE- Of the 93 patients, 85 (91.4%) were alive and 8 (8.6%) died. Two
3patients, others-5patients. Median time to relapse-20months patients had sequelae after chemotherapy and two patients experi-
from initial diagnosis(range:8-57months). Relapse site: Isolated enced relapse.
bone-marrow-13patients, isolated extra-medullary-4, combined- Conclusions: To contact LOFU patients by phone and formal letters
6patients. Extramedullary sites:CNS -7, testis-2, one each in bone and was in our situation effective for more than four-fifths of patients.
mediastinal-mass. Patient risk-stratification upon relapse:S1group- Therefore, simple and cheap methods can be effective to decrease the
2patients, S2group-9, S3group-4 and S4group-8patients. Sixteen number of patients lost to follow-up. Long term follow-up is essential
patients were considered for HSCT. Four of twenty-three didn’t to analyze the results of ALL treatments. A LMIC should have special
achieve CR2. Nineteen patients achieved CR2, of which 12underwent consideration for long term follow-up.
HSCT(4-Matched-sibling, 8-Haploidentical) and 7were managed with
only chemotherapy-radiotherapy. Of these, 7/19 relapsed after CR2.
Follow-up ranged from 12-1992days. On last follow-up, the OS and EP046 / #1739 PH(+) ALL. EXPERIENCE OF 20 YEARS IN
EFS of the cohort is 47% and 39% respectively. Mortality: refractory ONE GREEK PEDIATRIC ONCOLOGY DEPARTMENT
disease -6patients and treatment related mortality- 6patients(sepsis-
5, arrythmia-1). Analyzing the outcomes of sub-groups, the OS and Evgenia Magkou1 , Ksenia Loseva1 , Dimitrios Doganis1 , Marina
EFS: S1group-100% and 50%, S2group-66% and 55%, S3group-50% Servitzoglou1 , Maria Nikita1 , Eleni Kosmidi2 , Margarita Mpaka1
and 50%, S4group-12.5% and 12.5%. Amongst those who underwent 1P & Aglaia Kyriakou Children’s hospital, Oncology Department, Athens,
HSCT in CR2 the OS and EFS was 41.6% each. Greece; 2 Mitera Children’s Hospital, Department Of Pediatric And Adoles-
Conclusions: Treatment of relapsed-ALL is possible and should cent Hematology-oncology, Athens, Greece
be pursued. HSCT is a good treatment option for high-risk
patients. Background and Aims: Acute lymphoblastic leukemia(ALL) is the most
common malignancy in children. There are some subtypes of leukemia
associated with specific chromosomal translocations. The identifica-
EP045 / #636 LOST TO FOLLOW-UP DURING TREATMENT tion of these rearrangements is of great prognostic value, and can
OF ALL IN LMIC SINGLE CENTER EXPERIENCE IN play a major role in the selection of appropriate anti-leukemic therapy.
YOGYAKARTA, INDONESIA Ph(+)ALL t(9;22)(q34;q11), was associated with poor prognosis until
we included TKIs in therapy.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S171 of S635

Methods: All Ph(+)ALL treated in our department from 01.01.2002- Results: We identified 630 patients: 550 (87.3%) with B-ALL, and
31.12.2021 were analyzed. Characteristics, response to therapy and 80 (12.7%) with T-ALL. Twenty-eight (5.1%) patients with B-ALL and
final outcome were studied. 12 (15.0%) with T-ALL developed thrombosis, most often occurring
Results: From 01.01.2002-31.12.2021 445 children were treated with during Induction (63%) or Consolidation (20%) cycles. In unadjusted
ALL.Seven of them(1,57%)were found to be Ph(+).Additional cytoge- analyses, increasing year of life (OR, 1.16; 95%CI, 1.09-1.24), T phe-
netic abnormalities were detected in 5/7.Female were 3/7(42,8%).The notype (OR, 3.29; 95%CI, 1.55-6.64), d-dimer >20 μg/ml at diagnosis
median age was 5,75 years(3,4 -13,3).Six patients were diagnosed with (OR, 6.7; 95%CI, 1.4-25.9), and being overweight at diagnosis (OR, 2.5;
common B-ALL and 1 with biphenotypic T-ALL+B-ALL.All children 95%CI, 1.3-4.9) were associated with increased odds of thrombosis.
were treated with backbone BFM-HR protocols(BFM 95,ALLIC 2009) The strongest association with thrombosis was observed with periph-
and TKIs from Day15.Two patients underwent BMT in CR1.At diag- erally inserted central catheters (PICC) (OR, 6.0; 95%CI, 3.0-12.4). On
nosis median value(MV) of WBC was 17400/ml(3700 to 334200),MV adjusted analysis, T phenotype (OR, 3.9; 95%CI, 1.2-12.1), use of PICC
of Hb 10,4gr/dl(5,3 to 12,0)and MV of Plt was 153000/ml(16000 to lines (OR, 4.1; 95%CI, 1.4-13.8), being overweight (OR, 3.3; 95%CI, 1.1-
225000).All seven on Day8 were PDN good responders.On Day15 10.8), and diagnostic d-dimer ≥20 μg/ml (OR, 8.9; 95%CI, 1.4-46.8)
BM was M1 to 6/7 and M3 to 1/7.FC-MRD on Day15 was negative were independently associated with thrombosis risk.
in 3/7,<10 in 3/7 and >10 to 1/7.On Day33 5/7 patients had FC- Conclusions: Thrombosis during ALL therapy occurs most often early
MRD negative whereas 2 boys had MRD 0,01 and 0,4.Six patients on in therapy among patients with easily identifiable risk factors. These
Day33 had quantitative BCR-ABL/ABL negative whereas 1 patient had findings merit validation, and they identify a population in whom
BCR-ABL/ABL 3,31.Five of them are in CR1 for 0,58y(still under ther- intervention with prophylactic anticoagulation may be most beneficial.
apy),10,25y,14,08y,16,25y and 18,33y and 2/7 relapsed in 15 and 50
months from diagnosis.One of the relapsed patients underwent BMT
and is in CR2 for 7,9y.The second one underwent BMT in CR2 but EP048 / #1431 SYNERGY OF VENETOCLAX AND DUAL
relapsed 8 months after BMT and died with progression of his disease. MTOR/PI3K INHIBITION IN BCP-ALL
Conclusions: EFS of our patients with Ph(+) ALL is 71,5% and OS is
85,7%.The use of TKIs improved the EFS and the OS in children with Alexandra Niedermayer, Stefanie Enzenmüller, Felix Seyfried,
Ph(+)ALL and our results are comparable to the ones of the national Klaus-Michael Debatin, Lüder Meyer
literature. Ulm University Medical Center, Department Of Pediatrics And Adolescent
Medicine, Ulm, Germany

EP047 / #417 RISK FACTORS FOR THROMBOSIS IN Background and Aims: Deregulation of cell death is a hallmark of
CHILDREN RECEIVING ASPARAGINASE-BASED many cancers contributing to leukemogenesis and treatment failure
CHEMOTHERAPY FOR ACUTE LYMPHOBASTIC LEUKEMIA in B-cell precursor acute lymphoblastic leukemia (BCP-ALL). We pre-
viously showed that deficient apoptosis signaling is an indicator for
Casey Mcatee1 , Gengwen Tian1 , Clay Cohen1 , Melanie Bernhardt2 , poor outcome in ALL. Cell death induction is counter-regulated by
Melissa Richard1 , Michael Scheurer1 , Philip Lupo1 , Rosa Diaz1 anti-apoptotic molecules like BCL-2 and MCL-1 and selective inhi-
1 Baylor College of Medicine, Pediatric Hematology And Oncology, Houston, bition of BCL-2 by venetoclax showed clinical activity in CLL and
United States of America; 2 Texas Children’s Hospital, Global Hope (hematol- promising results in preclinical and first clinical studies in ALL. Previ-
ogy/oncology Pediatric Excellence), Houston, United States of America ously, we identified that hyperactivation of the mammalian target of
rapamycin (mTOR) signaling pathway in ALL cells is associated with
Background and Aims: Thrombosis is a known complication of acute inferior relapse-free survival. Here, we evaluated anti-leukemia activ-
lymphoblastic leukemia (ALL) therapy, but there are few strategies ities of BCL-2 (venetoclax) and mTOR/PI3K pathway (NVP-BEZ-235)
for identifying at-risk patients; thus, prophylactic anticoagulation is inhibition in BCP-ALL and analyzed the interplay of both inhibitors.
rarely utilized. The purpose of this study was to identify risk factors Methods: Half maximal effective concentrations (EC50 ) were assessed
for thrombosis during treatment for ALL to identify patients who may in BCP-ALL cell lines (N=8) and patient-derived xenograft (PDX) sam-
benefit most from prophylactic anticoagulation. ples (N=10). Protein expression was assessed by immunoblotting.
Methods: We conducted a retrospective cohort study of all chil- Synergies were analyzed in dose-response matrices (Bliss synergy
dren with newly diagnosed ALL treated at Texas Children’s Hos- model).
pital for the time period 2012-2020. The primary outcome was Results: We analyzed sensitivities of BCL-2 inhibition in BCP-ALL
incident deep venous thrombosis, dural sinus thrombosis, or pul- samples. Interestingly, we identified high expression of anti-apoptotic
monary embolism during therapy, identified through electronic query MCL-1 in venetoclax insensitive ALL cell line and PDX samples point-
of the medical record followed by manual review of radiology ing to MCL-1 as a mediator of venetoclax insensitivity. Exposure of
reports. We used multivariable logistic regression to determine ALL cells to NVP-BEZ235 resulted in decreased phosphorylation of the
odds of thrombosis given demographic, clinical and/or laboratory downstream targets S6 and 4E-BP1, reduced cellular proliferation and
variables. downregulated MCL-1 protein expression. 4E-BP1 has been described
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S172 of S635 ABSTRACTS

as translational regulator of MCL-1 and mTOR inhibition associated indicating mutual binding/dependence and potential effectivity of
MCL-1 downregulation might prime venetoclax insensitive ALL to concomitant inhibition. Importantly, combining BCL-2/BCL-XL and
cell death induction by BCL-2 inhibition. Interestingly, co-treatment MCL-1 inhibitors resulted in synergistic activity (mean Bliss synergy
with venetoclax and NVP-BEZ-235 synergistically reduced cellular score of +8.77; N=5).
proliferation and induced cell death (max. Bliss scores >40) in cell Conclusions: In summary, we identified sensitivity, on-target and syn-
line and PDX-ALL samples including apoptosis deficient, venetoclax- ergistic activity of the dual BCL-2/BCL-XL inhibitor AZD4320 together
insensitive, poor outcome ALL. with inhibition of MCL-1.
Conclusions: Taken together, we show anti-leukemia activity of simul-
taneous PI3K/mTOR and BCL-2 inhibition priming apoptosis deficient,
venetoclax insensitive BCP-ALL samples to synergistic cell death EP050 / #1012 THE TETRASPANIN CD9 SHAPES
induction along with downregulation of anti-apoptotic MCL-1. GLUCOCORTICOID SENSITIVITY IN PEDIATRIC B-CELL
PRECURSOR ACUTE LYMPHOBLASTIC LEUKEMIA

EP049 / #1644 SYNERGISTIC AND ON-TARGET ACTIVITY OF Wing Hei Ng, Chi Zhang, John Tak Kit Cheung, Han Wang, Kathy Yuen
DUAL BCL-2/BCL-XL INHIBITION TOGETHER WITH INHIBITION Yee Chan, Chi Kong Li, Kam Tong Leung
OF MCL-1 The Chinese University of Hong Kong, Department Of Paediatrics, Hong
Kong, Hong Kong PRC
Maren Wichert, Alexandra Niedermayer, Stefanie Enzenmüller,
Klaus-Michael Debatin, Lüder Meyer, Felix Seyfried Background and Aims: Resistance to glucocorticoids (GCs) con-
Ulm University Medical Center, Department Of Pediatrics And Adolescent fronts therapeutic success in B-cell precursor acute lymphoblastic
Medicine, Ulm, Germany leukemia (BCP-ALL). Identification of predictive biomarkers and mech-
anisms underlying GC resistance is of scientific/clinical importance.
Background and Aims: Targeting the intrinsic apoptosis pathway This study aims to demonstrate the association between CD9 and GC
has become a promising treatment approach in acute lymphoblastic susceptibility and uncover its mechanisms driving resistance.
leukemia (ALL). Selective BCL-2 inhibition (venetoclax) has shown het- Methods: BCP-ALL cell lines (n=6) and primary lymphoblasts (n=18)
erogeneous activity in ALL and other apoptosis regulators like MCL-1 with differential CD9 expression were profiled for their sensitivity
and BCL-XL promote insensitivity. However, targeting BCL-XL resulted to prednisolone (Pred) and dexamethasone (Dex). Association of CD9
in thrombocytopenia. The dual BCL-2/BCL-XL inhibitor AZD4320 with patient responses to Pred prophase was investigated in a pediatric
and its drug conjugate AZD0466 demonstrated anti-tumor activity BCP-ALL cohort (n=182). Functional impact of CD9 on GC suscep-
in hematological cancers, but only transient thrombocytopenia. Here, tibility was determined by gain- and loss-of-function experiments in
anti-leukemia activities of combined BCL-2/BCL-XL and MCL-1 inhibi- BCP-ALL cells, coupled with mechanistic dissection of pathways down-
tion (AZD5991) were analyzed and compared to other BH3-mimetics stream of GC receptor NR3C1 through genomics, transcriptomics and
investigating on-target activities and combination effects. proteomics.
Methods: Half maximal effective concentrations (EC50 ) were assessed Results: CD9- and CD9+ cell lines had distinct drug sensitivity profiles,
in seven B-cell precursor ALL cell lines and 19 patient-derived with the former showing resistance to GCs but not other chemother-
xenograft (PDX) samples. Protein expression and complexes were apeutics. CD9- samples exhibited resistance to Pred (IC50s: 8,185 vs.
assessed by immunoprecipitation and immunoblotting. BH3-profiling 268 nM) and Dex (IC50s: 2,761 vs. 31 nM) comparing with CD9+
was performed to determine dependencies on BCL-2 family proteins. samples. More poor Pred responders were identified in CD9- than
Combination effects were analyzed by dose-response matrix analyses. CD9+ patients (19.4% vs. 6.2%, P=0.018). CD9 overexpression in CD9-
Results: First, we determined activities of dual BCL-2/BCL-XL cells (SEM, KOPN-8) significantly enhanced sensitivity to GCs by 2.8-
(AZD4320), BCL-2 (venetoclax), BCL-XL (A-1331852) and MCL-1 17.7-fold, whereas CD9 knockout in CD9+ cells (697) decreased GC
(AZD5991, S63845) inhibition. Sensitivity to dual BCL-2/BCL-XL inhi- sensitivity. The expression and cytoplasm-to-nucleus translocation of
bition correlated significantly with BCL-2 (N=19; rs =0.63; p=0.004) GC receptor was similar in CD9- and CD9+ cells but CD9 was phys-
but not BCL-XL inhibition, and sensitivities to both MCL-1 inhibitors ically interacted with NR3C1. Upon GC stimulation, the presence of
also correlated (N=19; rs =0.66; p=0.002). Interestingly, highest CD9 facilitated DNA binding of NR3C1 and increased the number and
sensitivities (lowest EC50 values) were found for dual BCL-2/BCL-XL magnitude of GC-responsive genes (TSC22D3, ZBTB16). CD9- cells
inhibition (p<0.001). Mechanistically, analysis of protein complexes showed constitutive activation of MAPK pathway and were preferen-
showed disrupted binding of the apoptosis executor BIM to BCL-2 and tially susceptible to reversal of GC resistance by the MEK1/2 inhibitor
BCL-XL upon BCL-2/BCL-XL inhibitor exposure, while BIM-binding to trametinib.
MCL-1 was reduced upon MCL-1 inhibition, demonstrating on-target Conclusions: CD9 confers GC susceptibility through modulation of
activity of both drugs. Functionally (BH3-profiling), BCL-2/BCL-XL GC signaling in childhood BCP-ALL. Patients with CD9- phenotype
inhibition induced increased MCL-1-dependence, while inhibition of might be more prone to trametinib therapy for counteracting GC
MCL-1 resulted in a shift towards dependence on BCL-2 and BCL-XL, resistance.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S173 of S635

EP051 / #1411 HEALTH OUTCOMES IN ADULT SURVIVORS Pavlov University, R. M. Gorbacheva Research Institute, Saint Petersburg,
OF CHILDHOOD ALL AND THEIR SIBLINGS – A NATIONAL Russian Federation
LONG TERM FOLLOW UP
Background and Aims: Patients with relapsed/refractory (R/R) acute
Katarina Aili1 , Jens Nygren1 , Susann Arvidsson1 , Maria Olsson2 , B - lymphoblastic leukemia (B-ALL) following allogeneic hematopoietic
Marianne Jarfelt2 stem cell transplantation (allo-HSCT) have a poor prognosis. Blinatu-
1 Halmstad University, School Of Health And Welfare, Halmstad, Sweden; momab is effective salvage option for R/R patients after allo-HSCT,
2 University of Gothenburg, Department Of Oncology, Institute Of Clinical however relapses still occur in most patients. We suppose, that adding
Sciences, Sahlgrenska Academy, Gothenburg, Sweden infusions of donor lymphocyte (DLI) to blinatumomab may improve
immunoadoptive pressure on leukemic cells. We evaluated overall
Background and Aims: The treatment of acute lymphoblastic survival (OS), incidence of responses and graft-versus-host-disease
leukaemia (ALL) has improved and resulted in increased survival (GVHD) in R/R B-ALL children, who were treated with combination
and reduction in late effects. However, health outcomes of survivors immunotherapy blinatumomab and DLI after allo-HSCT.
can be affected later in life. An increased understanding of health Methods: The data of 15 children with the median age 10 (0-18) years,
outcomes is therefore important to be able to provide promotional who underwent immunotherapy with blinatumomab and DLI after allo-
and supportive resources. The aim of this study is to investigate health HSCT, were analyzed. Allo-HSCT was performed in complete remission
outcomes in adult survivors of childhood ALL (ASCALL) and their (CR) in 5 (33%), MRD+ in 2 (13%)., relapse – in 8 (53%) pts. Hap-
siblings. loidentical donor was in 11 (73%) pts. Myeloablative conditioning was
Methods: A national cross-sectional study including ASCALL, diag- used in 9 (60 %) pts. All pts received cyclophosphomyde on day +3,
nosed between 1985 and 2007, registered in the Swedish Childhood +4. Indication for therapy was MRD+ in 6 (40%), relapse - in 9 (60%)
Cancer Registry. 440 (51%) out of 860 ASCALL and 135 siblings pts. Blinatumomab was administrated after prior chemotherapy in 5
responded to a questionnaire. T-test and chi square test were used to (33%) relapsed pts. The first dose of DLI (median =1*106 CD3+/kg)
compare groups regarding HRQL (SF-36) health related factors includ- was administrated during the first course blinatumomab in 12 (80%)
ing sleep quality/quantity, stress, anxiety and depression (DASS 21), pts. Six pts (40%) received 2-3 courses of therapy.
several dimensions of fatigue (MFI 20), prevalence of chronic pain, Results: At the median follow up 10 months OS is 73%. CR was
diabetes, cardiovascular disease, headache/migraine, rheumatism, and achieved in 12 (80%) children, the median duration of remission was 7
workability (WAI). months (1-52, months). Bone marrow relapse occurred in 5/12 (40%).
Results: The mean age of the ASCALL and their siblings was 30.9y Grade III acute GVHD was reported in 2 pts, classic chronic GVHD – in
(19y -49y) and 32.5y (18y-54y) respectively. 49% of ASCALL and 2 (mild and severe), moderate overlape syndrome – in 1 child. All cases
62% of the siblings were female. Time since diagnose was 24.1 y of death were associated with relapses of leukemia.
(13y-36y). ASCALL had significantly lower sleep quality (22% poor Conclusions: Сombination of blinatumomab and DLI may induce
sleepers vs 10% p=0.01), quantity (p=0.049), and mean workabil- durable remission in patients with R/R B-ALL. This immunotherapy
ity (8.17 vs 8.66, p=0.032), higher scorings on fatigue (physical is safe even in children after haplo-HSCT. Further experiences are
fatigue (12.09 vs 9.41, p=<0.001), reduced motivation (12.07 vs necessary for finally outcomes.
8.21, p<0.001), and reduced activity (12.85 vs 9.90, p=<0.001))
than the siblings. No significant differences between the groups on
BMI, chronic pain, depression, anxiety,stress, prevalence of diabetes, EP053 / #1176 VITAMIN D LEVELS AND METABOLIC
cardiovascular disease, or rheumatism. The ASCALL scored signifi- SYNDROME AT DIAGNOSIS IN CHILDHOOD LYMPHOBLASTIC
cantly worse on the general health domain (SF-36) (64.9 vs 72.3, LEUKEMIA
p=0.002).
Conclusions: Adult survivors of childhood ALL report more fatigue, Claudia Paris1 , Ana Zepeda2 , Paulina Gallardo1 , Ana María Quezada1 ,
more sleeping problems, poorer general health and poorer workability Alicia Bustamante1 , Leticia Rojas1
compared to a group of siblings. The differences indicate that adult ALL 1 Hospital Luis Calvo Mackenna, Oncologia, Santiago, Chile; 2 Universidad de

survivors may be more vulnerable to poor health later in life. Valparaiso, Tecnologia Medica, Santiago, Chile

Background and Aims: Vitamin D is associated with alterations in


EP052 / #1081 OUTCOME OF IMMUNOTHERAPY WITH bone metabolism, immunity, and metabolic syndrome (MS). It has been
BLINATUMOMAB AND DONOR LYMPHOCYTE INFUSIONS IN described those children with acute lymphoblastic leukemia (ALL) and
PEDIATRIC R/R B-ALL AFTER ALLO-HSCT low vitamin D values at diagnosis are associated with lower survival.
The study aimed to determine the prevalence of low levels of Vitamin
Liubov Tsvetkova, Olesya Paina, Zhemal Rakhmanova, Polina D and risk factors associated with MS in ALL.
Kozhokar, Anastasiya Frolova, Elena Babenko, Tatyana Gindina, Elena Methods: Descriptive and prospective study including patients was
Semenova, Ludmila Zubarovskaya conducted between 2017- 2022. Patients were evaluated from
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S174 of S635 ABSTRACTS

November 2017 to March 2022. Vitamin D levels, waist circumference, PEGAsp and adverse events associated with COVID-19 mRNA
arterial pressure (AP), triglycerides, HDL-cholesterol, and glucose lev- vaccination.
els were recorded at study entry. Vitamin D deficiency was defined as Results: Forty patients with PEGAsp allergy (grade 2: n=12; grade 3:
a serum < 20 ng/mL, insufficiency was 20–30 ng/mL, and sufficiency n=9; grade 4: n=4) received COVID-19 vaccination at a median of
>30 ng/mL. Cook’s criteria for < 10 years old and 10 or more years old 54.4 months (range 2-154 months) after documented hypersensitiv-
International Diabetes Federation’s criteria were used to diagnose MS. ity. Of those, 33 (82.5%) had been challenged with an alternative form
This study was Approval by Ethical Committee. The chi-square test was of Asparaginase and 30/33 (91%) had not experienced hypersensitivity
used to analyze the relationship between the variables. with an alternative formulation, suggesting PEG as the hypersensitivity
Results: Fifty-eight patients were enrolled, and 11 of them were trigger for the majority. All patients received both doses of the vaccine,
excluded. Finally, 47 were studied. The median age at the time of diag- and no patients experienced any allergic or anaphylactic symptoms
nosis was 7,58 years, and the median time of follow-up post-diagnosis from the vaccination.
was 22 months. Twenty-seven patients were males. Vitamin D lev- Conclusions: Based on our institutional experience, COVID-19 mRNA
els were deficient at 55%, insufficient at 28%, and sufficient at 17% vaccination appears to be safe in patients with PEGAsp allergy despite
of patients. Twenty-six percent had MS at diagnosis. There were no the presence of PEG in the vaccine. The specific molecular weight of
differences in the incidence of metabolic syndrome between patients PEG may play a critical role in triggering a reaction. As efforts are
with sufficient vitamin D levels and those with deficient or insuffi- underway to vaccinate all eligible patients to COVID-19 for individual
cient levels. All relapsed patients (N=7) and one patient died from protection and herd immunity, these data suggest that patients with
COVID infection in remission. All patients have low levels of vitamin PEGAsp allergy should be included.
D (insufficient =1 and deficient=7).
Conclusions: It is essential to study vitamin D at the time of diagno-
sis, supplement with vitamin D throughout treatment and periodically EP055 / #1126 PREDICTORS OF PRE-MAINTENANCE
assess vitamin D levels. DELAYS DURING THERAPY AND ASSOCIATIONS WITH
OUTCOME AMONG CHILDREN WITH ACUTE LYMPHOBLASTIC
LEUKEMIA: A POPULATION-BASED STUDY
EP054 / #860 COVID-19 MRNA VACCINATION APPEARS
SAFE IN PEDIATRIC PATIENTS WITH A HYPERSENSITIVITY Serina Patel1 , Paul Gibson2 , Mylene Bassal3 , Qing Li4 , Uma Athale2 ,
REACTION TO PEGYLATED ESCHERICHIA COLI Vicky Breakey2 , Sumit Gupta5
L-ASPARAGINASE 1 Children’s Hospital, London Health Sciences Centre, Department Of Pedi-
atrics, London, Canada; 2 McMaster Children’s Hospital, Division Of Haema-
Amir Reza Pashmineh Azar1 , Nicole Wolfset2,3 , Charles Phillips1,3 , tology/oncology, Hamilton, Canada; 3 Children’s Hospital of Eastern Ontario,
Madison Stein1 , Susan Rheingold1,3 , Jennifer Heimall2,3 , Caitlin Pediatric Hematology/oncology, Ottawa, Canada; 4 Institute for Clinical
Elgarten1,3 Evaluative Sciences, Cancer Research Program, Toronto, Canada; 5 Hospital
1 Children’s Hospital of Philadelphia, Oncology, Philadelphia, United States for Sick Children, Haematology/oncology, Toronto, Canada
of America; 2 Children’s Hospital of Philadelphia, Allergy And Immunology,
Philadelphia, United States of America; 3 Perelman School of Medicine, Uni- Background and Aims: Therapy delays occur in children with acute
versity of Pennsylvania, Pediatrics, Philadelphia, United States of America lymphoblastic leukemia (ALL), causing stress among caregivers. Lim-
ited data exists on whether prolonged delays lead to inferior outcomes.
Background and Aims: L-Asparaginase is an established component of Methods: Children <18 years at diagnosis of ALL were identified at
acute leukemia therapy. PEG-Asparaginase (PEGAsp), Escherichia coli pediatric centers in Ontario, Canada between 2002-2012, treated
L-asparaginase linked to polyethylene glycol (PEG) is the common first- according to Children’s Oncology Group protocols for standard- or
line formulation, however, hypersensitivity reactions to PEGAsp occur high-risk ALL. Abstractors obtained demographic, disease, treatment,
in 10-15% of patients, with PEG component suggested as the anti- and outcome-related data, including dates of therapy phases. Phase-
genic culprit. As current mRNA COVID-19 vaccines contain PEG, albeit specific delays were calculated by comparing actual duration to that
of different molecular weight, the safety of administration of these expected by protocol. Pre-Maintenance phase-specific delays were
vaccines to leukemia patients with prior PEGAsp hypersensitivity has summed to obtain “total delay” at the time of Maintenance. Predic-
been questioned. The purpose of this case series is to describe a sin- tors of prolonged delay (>/=75th percentile) were determined through
gle institution’s experience administering the COVID-19 vaccination to logistic regression. The association between prolonged delay and
patients with PEGAsp allergy. event-free and overall survival (EFS, OS from start of Maintenance) was
Methods: We performed a retrospective chart review of pedi- adjusted for demographic and disease factors.
atric patients with PEGAsp hypersensitivity who received Results: Among 489 patients, the longest delays were associated with
COVID-19 mRNA vaccination at the Children’s Hospital of Intensified Consolidation [median 14 days, interquartile range (IQR)
Philadelphia (CHOP). Chart abstraction was performed to deter- 7-23] and Intensified Delayed Intensification (14 days, IQR 8-21).
mine the occurrence and grading of hypersensitivity reactions to Among 477 (97.5%) children reaching Maintenance therapy, median
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S175 of S635

total delay was 29 days (IQR 13-50), higher among those receiving EP057 / #773 OUTCOMES OF MSK-NY-II THERAPY IN
high-risk therapy (49, IQR 34-61) vs. standard-risk therapy (16, IQR 8- PEDIATRIC AND YOUNG ADULT PATIENTS WITH PRECURSOR
29). In multivariable analysis, predictors of prolonged delay (>50 days) B-CELL ACUTE LYMPHOBLASTIC LEUKEMIA
included high-risk ALL and positive end Induction minimal residual dis-
ease. Prolonged delay was not associated with inferior EFS [hazard Kavitha Ramaswamy, Christopher Forlenza, Neerav Shukla, Tanya
ratio (HR) 1.4, 95th confidence interval (95CI) 0.7-2.7; p = 0.40] but Trippett, Maria Luisa Sulis, Peter Steinherz
was with inferior OS (HR 4.8, 95CI 1.3-17.1; p = 0.01). When adjusted Memorial Sloan Kettering Cancer Center, Department Of Pediatrics, New
for other disease-related variables, the magnitude of association with York, United States of America
OS remained similar though statistical significance was lost (HR 4.1,
95th CI 0.9-17.5; p = 0.07). Background and Aims: Despite improving cure rates in children with
Conclusions: These results can inform counselling of caregivers of Acute lymphoblastic leukemia (ALL), high risk (HR) ALL poses unique
children with ALL on phase specific delays. Prolonged delays may be challenges. Intensified therapy has been the standard for augmenting
associated with increased risk of poor outcomes, though validation in increased early remission and improving long term survival. Treatment
larger cohorts is required. with the New York II protocol (MSK-NY-II) intensified chemotherapy
regimen was developed to improve efficacy and reduce toxicity. We
report here our center’s experience treating pediatric and young adult
EP056 / #1963 HAPLOIDENTICAL HEMATOPOYESIS STEM patients with precursor B-cell (pre-B) ALL with MSK-NY-II at Memorial
CELL TRANSPLANTATION FOR RELAPSED ACUTE Sloan Kettering Cancer Center (MSKCC).
LYMPHOBLASTIC LEUKEMIA IN CHILDREN TREATED IN Methods: We retrospectively analyzed all pediatric and young adult
URUGUAY patients from 2000-2016, <30 years of age, with de novo pre-B ALL
treated at MSKCC with the MSK-NY-II protocol.
Maria Ines Pereira Pereyra1 , Mariela Castiglioni2 , Anaulina Silveira3 , Results: We identified a total of 124 patients. Median starting age was
Luis Castillo2 6.8 years [0.1-29.6] with median initial bone marrow blasts of 61%
1 perez scremini, Montevideo, montevideo, Uruguay; 2 Pereira Rosell Hos- [1-99]. CNS3 involvement was seen in 3.2% (4/124) of patients and
pital Pérez Scremini Fundation, Montevideo/UY, Montevideo, Montevideo, no testicular involvement identified. There were 94.1% (96/102) of
Uruguay; 3 Pereira Rossell Hospital Perez Scremini Foundation, Hematology evaluable patients who achieved a complete remission (CR) of <5%
Oncology Department, Montevideo, Uruguay bone marrow blasts by D29 of Induction. There were 21 patients
(16.9%) who received an allogeneic hematopoietic stem cell trans-
Background and Aims: The prognosis of relapsed Acute Lymphoblas- plant in CR1. Median time to relapse was 736 days [113-1208] with
tic Leukemia (ALL) remains por due to resistance to chemotherapy 15 total relapses (13.1%); 9 medullary, 4 extramedullary, and 2 com-
agents. This study evaluate the efficacy and feasibilty of Haploiden- bined relapses. Overall survival (OS) at 5-years was 88.7%. MSK-NY-II
tical Hematopoietic Cell Transplantation (Haplo HSCT) for pediatric cumulative doxorubicin dose was 300mg/m2. Cardiac dysfunction with
relapsed ALL. abnormal shortening fraction was observed in 1 patient. There were
Methods: 40 patients with relapsed ALL, 14 underwent Haplo 2 infectious deaths during MSK-NY-II therapy, 1 during Induction and
HSCT between 2010 and 2021 at the Hospital Pereira Rossell 1 with PJP pneumonia during Maintenance. There was a low total
(Montevideo-Uruguay. All patients were transplanted in Com- number of inpatient days, median 26.5 days [range 0-157].
plete Remission (CR) following different protocols, only 3 had Conclusions: The intensive MSK-NY-II protocol for pre-B ALL was tol-
positive MRD(0.1%,0.6%,1.9%). Conditioning regimens employed erated well and outcomes in this large single institution cohort are
were TBI based in 11 patients. Graft versus host disease similar to that of contemporary regimens for pre-B ALL. MSK-NY-II
(GVHD) prophylaxis was administered with cyclosporine and warrants consideration in patients with HR pre-B ALL.
methotrexate.
Results: 8 patients received peripheral blood and 6 received bone
marrow as the stem cell source. Among 14 patients who achieved EP058 / #1663 LOW FREQUENCY OF T(12;21) B-CELL
engraftment, acute GVHD ocurred in 8 patients. (GI6, GII4,GIII1,GIV ACUTE LYMPHOBLASTIC IN COLOMBIA: A REPORT FROM
1, skin was the most comon organ involve) and chronic GVHD VIGICANCER WORKING GROUP
was observed in 6(GI 2, GII 2, GIII 2,GIV 2 Skin was involve in
the mayority of cases and severe cases was because of the Lung). Oscar Ramirez1,2,3 , Paula Aristizabal4,5,6 , Roberto Jaramillo7,8 , Luis
The 5 year overall survival (OS) between al LAL relapsed patients Bravo2,7 , On Behalf Of Vigicancer Working Group7
was 32.5% +_7.4% and in patients who underwent and haplo was 1 Fundacion POHEMA, Scientific Direction, Cali, Colombia; 2 Cali’s Can-
33.3% +- 12%, leukemia free survival rates were 16% and 20% cer Population-Based Registry, Universidad Del Valle, Cali, Colombia;
respectively. 3 Clinica Imbanaco de Cali, Bone Marrow Transplantation, Cali, Colom-
Conclusions: Haploidentical transplantation is a feasible solution for bia; 4 University of California, San Diego, Pediatrics, La Jolla, United
relapsed leukemia patient in Uruguay with promesing results. States of America; 5 University of California, San Diego, Moores Cancer
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S176 of S635 ABSTRACTS

Center Population Science, Disparities And Community Engagement, La objective of this study was to describe the nutritional status and
Jolla, United States of America; 6 Rady Children’s Hospital San Diego, Peck- unscheduled hospitalized days due to infectious processes on the
ham Center For Cancer And Blood Disorders, San Diego, United States survival of children with acute lymphoblastic leukemia.
of America; 7 Fundacion POHEMA, Research Department, Cali, Colom- Methods: A retrospective cohort study was designed to describe the
bia; 8 OncoDiagnóstica, Laboratorio Clínico Continental, Scientific Director, survival of a convenience sample of patients according to their nutri-
Barranquilla, Colombia tional status and the number of unscheduled days of hospitalization in
a referral center in the state of Jalisco, Mexico.
Background and Aims: Children with acute lymphoblastic leukemia Results: 203 patients were included. The male-female ratio was 1.5.
(ALL) and t(12;21) (ETV6-RUNX fusion) have excellent overall survival. Ninety-six children (47.2%) had an optimal nutritional status at diagno-
Prevalence in children is approximately 20-25%, however reports sug- sis, whereas this was not optimal in 107 patients (52.8%). 34 patients
gest prevalence has geographical and ethnic variability. We assessed (16.7%) presented global malnutrition. According to the body mass
the prevalence of t(12;21) in children with ALL from Colombia. index, 10% of the patients were overweight and 12% were obese.
Methods: We conducted a cross-sectional study to estimate the preva- The average number of days of unscheduled hospitalization for chil-
lence of the t(12;21) in patients with newly diagnosed B-cell ALL dren without optimal nutritional status was higher in the remission
(<15 years of age) included in 27 pediatric oncology units (POU) in induction and consolidation treatment stage (8.3 and 2.3 days, respec-
10 Colombian cities reporting to VIGICANCER (Childhood Cancer tively) related to children with optimal nutritional status (8.2 and 1.2
Surveillance System). In Colombia, detection of t(12;21) is performed days respectively). In the statistical analysis, a significant association
by fluorescence in situ hybridization (FISH) at several commercial between survival and nutritional status was not found
laboratories; however, no centralized validation is carried out. Conclusions: In this cohort, although nutritional status was not asso-
Results: From January 2019 to December 2021, VIGICANCER ciated with lower survival in children with ALL, we found that
included 965 children with ALL (871 B-cell, 80 T-cell; 14 most children do not have an optimal nutritional status at diagnosis,
mixed/unclassified). In the B-cell ALL cohort, the median age was therefore they are more susceptible to complications secondary to
5.5 years (IQR: 3, 10), 52% were male, 9% were afro-descendants chemotherapy toxicity.
and/or native Colombians, and 50% had public insurance. FISH for
t(12;21) was performed in 67% of patients and for t(9;22) in 83%.
Frequencies for t(12;21) and t(9;22) were 11% (95%CI: 8, 13) and 5% EP060 / #2002 RISK FACTORS FOR TREATMENT
(95%CI:3, 7), respectively. We found an 8% prevalence of t(12;21) in ABANDONMENT FOR CHILDREN WITH ACUTE
patients with public health insurance vs. 14% in semi-private (P=0.02). LYMPHOBLASTIC LEUKEMIA. A STUDY FROM A TERTIARY
There was high heterogeneity in detection among the different POUs, CARE HOSPITAL IN MEXICO
and in two, the prevalence was 20-28%
Conclusions: Prevalence of t(12;21) in B-cell childhood ALL in a size- Jorge Ramirez Melo1 , Sergio Gallegos1 , Daniel Moreira2 , Hugo
able sample of patients in Colombia was lower than reported in other Romo-Rubio1 , Jessica Santoyo1 , Oscar Gonzalez Ramella1 , Fernando
regions. Two POUs achieved the expected prevalence reported in high- Sanchez1 , Paola Friedrich2
income countries. There were differences by insurance, suggesting that 1 Hospital Civil de Guadalajara, Pediatric Oncology And Hematology Ser-
the low prevalence in our sample could be related to the quality of vice, GUADALAJARA, Mexico; 2 St. Jude Children’s Research Hospital, Global
FISH methodology used in Colombia. Our findings impact the survival Pediatric Medicine, Memphis, United States of America
of Colombian children and present an opportunity to improve ALL diag-
nosis, classification, and treatment in resource-constrained settings by Background and Aims: In low and middle-income countries, one of
developing targeted strategies. the main causes of treatment failure is treatment abandonment. This
study shows the characteristics of children with acute lymphoblastic
leukemia and its associated risk factors for abandonment in the first 90
EP059 / #1998 IMPACT OF NUTRITIONAL STATUS ON days of treatment.
SURVIVAL AND UNSCHEDULED DAYS OF HOSPITALIZATION Methods: A retrospective cohort study was designed that included
FOR INFECTIOUS PROCESSES IN CHILDREN WITH ACUTE patients younger than 18 years of age diagnosed in an oncology unit
LYMPHOBLASTIC LEUKEMIA in a middle-income country.
Results: 643 children were included; the average age of the patients
Jorge Ramirez Melo, Sergio Gallegos, Hugo Romo-Rubio, Jessica was 7.1 years. Family nucleus status was recorded in 616 patients,
Santoyo, Fernando Sanchez of which, 93 cases (15%) had a single-parent family. Regarding
Hospital Civil de Guadalajara, Pediatric Oncology And Hematology Service, the place of residence (n=638) 335 children (52%) lived out-
GUADALAJARA, Mexico side the urban area of the city. As for parents, incomplete pri-
mary education was the most frequent level of education; mothers
Background and Aims: In children with acute lymphoblastic leukemia and fathers 40% and 33% respectively. Forty-one patients (6.4%)
(ALL), the most important cause of hospitalization is infections. The abandoned treatment. The economic household-income monthly
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S177 of S635

average was $6039 MXN. With the logistic regression model, a sig- Conclusions: In this cohort of patients, changes in the survival rate
nificant association was found with single-parent family (p=0.000), were significantly associated with the implementation of the Total XV
father’s age <30 years (p=0.000), positive minimal residual disease Therapy and adjustment in the treating personnel
after induction treatment (p=0.001) and residence outside the city
(p=0.02)
Conclusions: The socioeconomic factors associated with treatment EP062 / #354 CHILDHOOD LATE BONE MARROW RELAPSES
abandonment were related to the family burden that cannot be IN B CELL PRECURSOR ACUTE LYMPHOBLASTIC LEUKEMIA IN
covered by families who have to travel longer distances from care SPAIN. ANALYSIS OF TREATMENT OUTCOMES
centers, in some cases in single-parent families, and according to
the father’s age, being the families with parents under 30 years of Eduardo Ramos Elbal1 , María Del Pilar Guerrero Gil2 , Berta González
age are at higher risk. In this study, children who did not achieve Martínez3 , Alvaro Lassaletta4 , Jose Antonio Salinas Sanz5 , Rosa
remission after induction treatment had a higher risk of treatment Adan-Pedroso6 , Pablo Velasco7 , Jose Luis Fuster Soler8
abandonment. 1 Hospital Clínico Universitario Virgen de la Arrixaca, Pediatric Hemato-
oncology, El Palmar, Spain; 2 University of Murcia, Medicine, Murcia,
Spain; 3 Hospital Universitario La Paz, Pediatric Hemato-oncology, Madrid,
EP061 / #2007 A MIXED-METHOD APPROACH TO THE Spain; 4 Hospital Infantil Universitario Niño Jesús, Pediatric Neuro-oncology,
ANALYSIS OF TRENDS IN SURVIVAL RATE OF CHILDREN WITH Madrid, Spain; 5 Hospital Universitario Son Espases, Pediatric Hemato-
ACUTE LYMPHOBLASTIC LEUKEMIA. EXPERIENCE OF SINGLE oncology, Palma de Mallorca, Spain; 6 Hospital Universitario de Cruces,
INSTITUTION IN A MIDDLE-INCOME COUNTRY Pediatric Hemato-oncology, Barakaldo, Spain; 7 VALL D’HEBRON HOSPI-
TAL, OncologÍa Y HematologÍa PediÁtrica, BARCELONA, Spain; 8 Hospital
Jorge Ramirez Melo1 , Sergio Gallegos1 , Daniel Moreira2 , Hugo Clínico Universitario Virgen de la Arrixaca, Pediatric Hemato-oncology, El
Romo-Rubio1 , Jessica Santoyo1 , Oscar Gonzalez Ramella1 , Fernando Palmar, Spain
Sanchez1 , Paola Friedrich2
1 Hospital Civil de Guadalajara, Pediatric Oncology And Hematology Ser- Background and Aims: Acute lymphoblastic leukemia (ALL) is the
vice, GUADALAJARA, Mexico; 2 St. Jude Children’s Research Hospital, Global most common cancer in childhood, with relapse occurring in 10-15% of
Pediatric Medicine, Memphis, United States of America cases. In Spain, the current therapeutic recommendation (LAL/SEHOP-
PETHEMA 2015) for relapse is based on the IntReALL SR and HR
Background and Aims: In low-and middle-income countries, protocols and recommend allogeneic stem cell transplantation (HSCT)
the survival of children with acute lymphoblastic leukemia to patients with B-cell precursor (BCP) late bone marrow relapse and
(ALL) decreases dramatically in the initial treatment phase. inadequate response (minimal residual disease ≥ 0.1%) after induction.
The objective of this study was to analyze the survival Here we describe our overall results and analyse the impact of this
trend of pediatric patients with ALL in the first 90 days of stratification.
treatment. Methods: Data from pediatric patients with late bone marrow relapse
Methods: A sequential mixed-method approach was used. of BCP ALL recorded within the SEHOP/PETHEMA 2015 registry from
Interventions that decreased early events were found using January 2015 to April 2021 were reviewed. Overall survival (OS),
the qualitative model. The trend of the annual percentage event-free survival (EFS), cumulative incidence of relapse (CIR) and
change (APC) of the survival rate was analyzed with the Jump treatment-related mortality (TRM) were calculated both in the whole
Model and Comparability Ratio and time periods that pre- cohort and stratified by response to induction (MRD < 0.1% vs MRD ≥
sented changes with statistical significance (p <0.05) were 0.1%). The Kaplan-Meier method, Log Rank test and Gray’s methods
identified were used to estimate and compare OS, EFS, CIR and TRM with the
Results: 12 semi-structured interviews were applied using seven cate- help of R statistical software.
gories that described the main interventions implemented to improve Results: A total of 42 patients were included. Median follow-up was
the survival rate. 643 children were included from 2007 until April of 33 months (range 7-47). The 3-year OS, EFS, CIR and TRM were of
2021. After one year of implementing the Total XV Therapy (2012), 81.1%, 60.7%, 33.4% and 11.2%, respectively in the entire cohort. We
a change in the trend of the event-free survival rate (EFS) was iden- found no statistically significant differences in OS (87.3% vs 71.4%; p =
tified (APC=+2.08) and was statistically significant. The EFS trend 0.3), EFS (72.7% vs 42.9%; p = 0.052), CIR (27.9% vs 39.3%; p = 0.43)
rates increased in 2009 once there was an adjustment in the treat- and TRM (4.5% vs 21.4%; p = 0.16) among good responders versus poor
ing personnel and chemotherapy dose intensity protocol improvement responders.
(APC=+7.85) although it was not statistically significant. There was a Conclusions: Overall results are comparable to those previously
point in 2014 when the overall survival rate (OS) trend (APC=+1.8) had reported with the ALL-REZ 2002 BFM study. The application of HSCT
a significant increase. This was one year after the leukemia clinic staff was able to rescue a proportion of patients with poor response
increased and a pediatric infectious disease specialist was included on to inducction after relapse which is translated into non-significant
the team differences in survival results.
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S178 of S635 ABSTRACTS

EP063 / #1339 ANALYSIS OF THE OUTCOME OF PRE-B EP064 / #270 MEDICATION INDUCED DIABETES DURING
STANDARD RISK RELAPSES REGISTERED IN THE INDUCTION THERAPY IN PEDIATRIC ACUTE LYMPHOBLASTIC
SEHOP-PETHEMA 2015 GUIDELINES LEUKEMIA: IMPACT OF RISK GROUPING

Eduardo Ramos Elbal1 , Inés Fernández Sánchez2 , Berta González Katie Ross1 , Ketan Kulkarni2 , Teresa Pinto3 , Tamara Macdonald2
Martínez3 , Rosa Adan-Pedroso4 , Alvaro Lassaletta5 , Jose Manuel 1 Dalhousie University, Faculty Of Medicine, Halifax, Canada; 2 IWK Health
Vagace Valero6 , Pablo Velasco7 , Jose Luis Fuster Soler8 Centre, Division Of Hematology/oncology, Department Of Pediatrics, Hali-
1 Hospital Clínico Universitario Virgen de la Arrixaca, Pediatric Hemato- fax, Canada; 3 IWK Health Centre, Division Of Endocrinology, Department
oncology, El Palmar, Spain; 2 University of Murcia, Medicine, Murcia, Spain; Of Pediatrics, Halifax, Canada
3 Hospital Universitario La Paz, Pediatric Hemato-oncology, Madrid, Spain;
4 Hospital Universitario de Cruces, Pediatric Hemato-oncology, Barakaldo, Background and Aims: Medication induced diabetes (MID) is common
Spain; 5 Hospital Infantil Universitario Niño Jesús, Pediatric Neuro-oncology, during induction therapy for pediatric acute lymphoblastic leukemia
Madrid, Spain; 6 Complejo Hospitalario de Badajoz, Pediatric Hemato- (ALL) and has potentially significant negative consequences. Reported
oncology, Badajoz, Spain; 7 VALL D’HEBRON HOSPITAL, OncologÍa Y risk factors for MID are variable with limited data comparing patients
HematologÍa PediÁtrica, BARCELONA, Spain; 8 Hospital Clínico Univer- treated with standard-risk (SR) vs high-risk (HR) regimens. This study
sitario Virgen de la Arrixaca, Pediatric Hemato-oncology, El Palmar, aims to evaluate the incidence and risk factors for MID during induc-
Spain tion in patients with ALL from the Maritimes over a 20-year period,
assess the complication rates of MID, and compare the risk of MID in
Background and Aims: The classification fo early combined relapses SR versus HR regimens.
of pre-B phenotype in the standard risk group is controversial. Methods: We performed a retrospective single-center study of 262
In fact, the Children Oncology Group classifies these patients in patients (142 males, 120 females) diagnosed with ALL at the IWK
the high-risk group and the Nordic Society of Paediatric Haema- Health Centre from 2000 to 2019. Demographic and treatment data
tology and Oncology found an overall survival of only 38%, which was extracted from the Pediatric Oncology Research Database and
are intermediate results between the rest of the standard-risk and EMRs.
the high risk relapses. We analyse our own results in a Spanish Results: Twenty-two patients developed MID (8.4%). Patients with
cohort of patients undergoing treatment with an IntReALL-based MID were significantly older (10.3 vs 6.2 years, p < 0.001), had
approach. higher BMI z-scores (1.2 vs 0.3, p=0.003), and had higher rates of
Methods: Data from all patients registered in the Spanish registry Trisomy 21 (9.5% vs 1.3%, p=0.012). Patients with MID had signifi-
"SEHOP-PETHEMA 2015" between January 2015 and April 2021 cantly higher rates of CNS disease (36.4% vs 14.2%, p=0.007) but did
with a diagnosis of standard-risk first relapse were reviewed. Over- not have increased rates of infection, relapsed disease, or death. HR
all survival (OS), event-free survival (EFS), cumulative incidence of patients (n=122) had significantly more complications than SR patients
relapse (CIR) and treatment-related mortality (TRM) of pre-B early (n=140) including MID (13.1% vs 4.3%, p=0.01), CNS disease (23.8%
combined relapses were calculated and compared with the rest of vs 9.3%, p=0.001), infection (68.3% vs 45.7%, p<0.001), relapsed dis-
standard-risk pre-B first relapses. The Kaplan-Meier method was ease (10.7% vs 4.3%, p=0.047), and death (11.5% vs 1.4%,<0.001). HR
used for the estimation of the survival functions and the Log-Rank patients treated with 28-days of prednisone developed significantly
test was use for comparisons. Gray’s method was used for the anal- more MID than those treated with 14-28 days of dexamethasone
ysis of CIR and TRM. All these calculations were performed with (21.5% vs 3.5%, p=0.003) and were significantly older (12.7 vs 4.2
R. years, p<0.001).
Results: There were 71 cases of first relapse of standard-risk Conclusions: Older age, higher BMI, CNS disease, Trisomy 21, and
pre-B phenotype, of wich 7 were early combined relapses. EFS steroid type were risk factors associated with MID in our cohort. HR
was significantly lower in the early combined relapse group com- patients developed significantly more complications including MID.
pared with the other standard-risk relapses (33.3% vs 76.2%; Screening for MID should be routine during ALL induction therapy,
p=0.04). Furthermore, this was consequence of an increased particularly for those with HR disease.
CIR (50% vs 10.1%; p=0.048), with no difference in TRM (16.7%
vs 12.1%; p=0.074). OS was also lower, although this differ-
ence did not reach statistical significance (41.7% vs 82.3%; EP065 / #818 TREATING RELAPSED B-ALL IN IN CHILDREN
p=0.08). WITH A SETTING-ADAPTED MITOXANTRONE BASED
Conclusions: In early combined relapses of pre-B phenotype, we INTENSIVE CHEMOTHERAPY PROTOCOL (TMH RALL
found inferior results than in other standard-risk pre-B relapses PROTOCOL)-EXPERIENCE FROM TATA MEMORIAL HOSPITAL
due to a higher incidence of second relapses. This should lead us MUMBAI
to consider treatment intensification in this group, using new ther-
apies or throug the inclusion of these patients in the high-risk Nirmalya Roy Moulik1 , Jayesh Agiwale1 , Swetha Reddy1 , Ram
group. Mohan1 , Chetan Dhamne1 , Akanksha Chichra1 , Gaurav Narula2 ,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S179 of S635

Prashant Tembhare1 , Dhanlaxmy Shetty2 , Subramanian Pg1 , Sripad Bone Marrow Transplantation And Immune Deficiency, Cincinnati, United
Banavali1 States of America
1 Tata Memorial Centre, Paediatric Oncology, Mumbai, India; 2 Tata Memo-
rial Centre, HBNI, Medical Oncology, Mumbai, India Background and Aims: Bridging therapy (BT) is indicated for most
children with B-ALL during the period while awaiting manufac-
Background and Aims: The outlook of relapsed ALL in LMICs is partic- ture of tisagenlecleucel (tisa-cel), a CD19 directed CAR-T therapy.
ularly dismal due high treatment related toxicity, inadequate resources Both conventional chemotherapy agents and immunotherapies have
and unavailability of targeted therapy. We report our experience of been used. We aim to investigate outcomes after tisa-cel ther-
using a locally adapted mitoxantrone based protocol for non-high risk apy for patients treated with conventional or immunotherapy-based
(HR) relapses. BT
Methods: Eighty-two children with non-HR B-cell ALL relapses were Methods: Patients treated with tisa-cel at our institution with
treated on TMH rALL-18 protocol between November 2018-January bone marrow disease (with or without extramedullary disease) were
2021. The protocol (adapted from COG/UKALL-R3/Int-Re-ALL), com- included. Patients with isolated extramedullary disease were excluded
prising of 7 blocks of multi-agent chemotherapy (including mitox- as systemic BT was infrequently administered.
antrone in induction) followed by local radiation and maintenance, Results: 33 patients received systemic BT. 24 received conven-
underwent serial modifications based on our experience with initial tional chemotherapy and 9 received immunotherapy. There was no
patients. Major modifications included reducing number of dexam- consensus conventional chemotherapy regimen; methotrexate con-
ethasone pulses during induction, dose-reduction and spacing apart taining regimens were most common (n=12). 8/9 patients receiving
of high-dose cytarabine during Block-3. Unlike in the original proto- immunotherapy received inotuzumab and 1/9 received blinatumomab.
cols, we introduced blood count cut-offs for various post-induction One patient in each group died of infectious toxicity before infusion.
time-points. Failure to achieve MRD negative or molecular remission at day 28
Results: Thirty/82 and 52/82 patients were early and late relapses after infusion occurred in 5/23 (21.7%) conventional and 2/8 (25%) of
respectively. Though induction chemotherapy was outpatient based, immunotherapy recipients. Amongst responders, 6/18 (33%) conven-
65/82 patients needed hospitalization for supportive care of which tional BT patients and 3/6 (50%) immunotherapy BT patients relapsed.
22 (26.8%) had blood-stream infection, 20(24.4%) required ICU care 1 relapse was CD19-, occurring in a patient who received inotuzumab.
and 12(14.6%) died. Forty-five of 60 assessable patients (75%) cleared There was one second malignancy in a patient with Li-Fraumeni. At
MRD post-induction. Post-induction toxic deaths were seen in 7 last follow up (median 493 days [ range 76-2548 days]), 15/23 (65.2%)
patients (5 in Block 3, 1 during maintenance and 1 post-transplant). conventional BT patients and 5/8 (62.5%) were alive. B-cell aplasia
Multidrug resistant organism (MDRO) sepsis was seen in 8/19 toxic was maintained for at least 6 months in 8/23 (34.7) conventional BT
deaths. No further block 3 deaths were encountered post modification patients and 2/8 (25%) immunotherapy BT patients although 2 addi-
of cytarabine dose and interval. Till last analyzed 175 grade 3/grade 4 tional patients in the immunotherapy group went to planned HSCT
toxicity events were seen in induction and 182 further episodes dur- prior to 6 months.
ing the rest of the phases taken together. The EFS and OS of the cohort Conclusions: No obvious differences were seen in initial response,
were 53.9±7.5% and 68.4±8% respectively at median follow-up of 10 relapse, or survival between patients treated with conventional and
(2-39) months. immunotherapy BT. As low disease burden at time of infusion is a posi-
Conclusions: Toxic events including MDRO infections remain a tive prognostic factor, it is reasonable to bridge with whatever regimen
major hurdle in the LMICs. Toxicity can be mitigated by local is felt to be most likely to give the lowest disease burden with tolerable
adaptation of protocols that strike a fine balance between effi- toxicity.
cacy and tolerability. Until the time, targeted therapies are freely
accessible, treatment with setting-adapted chemotherapy proto-
cols can produce acceptable outcomes at least in the non-HR EP067 / #1857 RELIABILITY OF PERIPHERAL BLOOD
relapses. MINIMAL RESIDUAL DISEASE ON DAY 8 OF INDUCTION FOR
RISK STRATIFICATION OF PEDIATRIC PRECURSOR B ACUTE
LYMPHOBLASTIC LEUKEMIA
EP066 / #1127 THE CHOICE OF CONVENTIONAL
CHEMOTHERAPY OR IMMUNOTHERAPY AS BRIDGING Muhanad Shathly1 , Iyad Sultan2 , Hasan Hashem3 , Rawad Rihani4 ,
THERAPY DOES NOT APPEAR TO IMPACT CLINICAL RESPONSE Mayada Abu Shanap3 , Amal Abu Ghosh5
TO CD19 DIRECTED CAR-T THERAPY IN PEDIATRIC B-ALL 1 king Hussain cancer center, Pediatric, amman, Jordan; 2 King Hussein
Cancer Center, Pediatrics, Amman, Jordan; 3 king Hussein cancer center,
Jeremy Rubinstein1 , Christa Krupski2 , Christopher Dandoy2 , Ruby Pediatric, Amman, Jordan; 4 King Hussein Cancer Center, Paediatric Hema-
Khoury2 , Stella Davies2 , Christine Phillips1 tology/oncology/bone Marrow Transplant, Amman, Jordan; 5 king hussein
1 Cincinnati Children’s Hospital Medical Center, Oncology, Cincinnati, cancer center, Pediatric Oncology, Amman, Jordan
United States of America; 2 Cincinnati Children’s Hospital Medical Center,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S180 of S635 ABSTRACTS

Background and Aims: During the early phases of treatment for child- Results: On 8 days of steroid therapy, 8.4% of patients were pred-
hood (ALL), MRD is a statistically significant predictor of prognosis . For nisone poor responders. Bone marrow response on day 15 was M1
outcome prediction and therapy stratification in pediatrics with ALL, (<5% blasts) in 80.9% patients, M2 (5 ≥ to <25% blasts) in 14.4%, and
flow-cytometric monitoring of MRD in the bone marrow (BM) during M3 (≥25% blasts) in 4.7% patients. On day 33 bone marrow response
induction therapy is commonly used. Although using peripheral blood M1 was in 88.9%, M2 - in 9.1%, and M3 - in 2.0% of patients. According
(PB ) instead of BM has been adopted by several leukemia groups, data to risk stratification, 77,6% of the patient were qualified for SR, 17,7%
on its effectiveness is limited - IR, and 4,7% as HR. Complete remission was achieved in 98,4% of
Methods: This is a prospective study on a cohort of 64 children with patients. 10y OS for all patients was 91±1,6%. The OS for the SR was
B- ALL, who were treated at the King Hussein Cancer Center (KHCC 91,2±1,9%, 93,3±3,1% for IR, and 73,1±10,6% for HR, p<0,05. 10y EFS
)(ALL 1102 protocol). Patients underwent PB MRD assessment on day for SR, IR and HR was 86±2%, 83,3±5,1%, and 67,7±11,5%, respec-
8 of induction, followed by BM MRD evaluation on day 15 And at end tively. p<0,05. 10y DFS for SR group was 86±2%, for IR - 83,3±5,1%,
of induction and 67,7±11,5% - for HR, p<0,05.
Results: 69 patients were included (median age, 4.98 years; range, 1.5 Conclusions: ALL IC-BFM 2002 is highly effective protocol for treat-
to 18.2). Median WBC was 34.43k/ul . Risk stratification was : HR ment of children with ALL in Russian pediatric oncology centers. This
(N=23), SR (26), LR(n=18) and VLR (n=1). Cytogenetics were favor- program, based on inexpensive and reproducible stratification criteria,
able in 16 (24%), neutral in 39 (58%) and unfavorable in 12(18%). On has been successfully carried out in both large and small centers.
day 8, 41 had negative MRD and on day 15, 40 were negative.Day
35 showed negative MRD in 46 (72%). Day 8 and day 15 BM showed
significant correlation (R=0.64, p<0.001). When correlated with EOI EP069 / #873 IKZF1 PLUS PROFILE AND OUTCOME
MRD, day 15 showed significant correlation (R=0.54, p<0.01), while ANALYSIS IN A COHORT OF PEDIATRIC B-CELL ACUTE
day 8 did not (R=0.23, P=0.82). Studied for prediction of EOI, the AUC LYMPHOBLASTIC LEUKAEMIA
for day 8 (AUC=0.65) was significantly less than day 15(AUC=0.85)
(p=0.033). Using an MRD level of 0.1 % for days 8 and 15, 53 samples Minu Singh1 , Prateek Bhatia1 , Amita Trehan1 , Pragna Hc1 , Rozy
were concordant while 7 were not. Thakur1 , Neelam Varma2
Conclusions: Results of our study showed that although the day 8 and 1 Postgraduate Institute of Medical Education and Research, Pediatrics,
day 15 MRD levels were significantly correlated. However, the day 15 Chandigarh, India; 2 Postgraduate Institute of Medical Education and
marrow MRD measurement was the one significantly correlating with Research, Haematology, Chandigarh, India
the EOI MRD. This study is still ongoing and recruiting patients. More
data will be available for analysis that may strengthen our results. Background and Aims: IKZF1 deletion occurs in 15% of paediatric B-
cell ALL (B-ALL) and has been shown to be associated with an increased
risk of relapse and poor outcome. Recently described IKZF1Plus profile
EP068 / #1196 LONG-TERM TREATMENT RESULTS OF shows association with worse minimal residual disease (MRD), poor
PEDIATRIC AND YOUNG ADULT ACUTE LYMPHOBLASTIC prednisolone response (PPR) and high cumulative incidence of relapse.
LEUKEMIA WITH ALL IC-BFM 2002 PROTOCOL The current study aimed to evaluate the frequency and prognostic
impact of IKZF1 and IKZF1Plus deletion in a cohort of pediatric B-ALL.
Timur Valiev, Meri Shervashidze Methods: A total of 178 pediatric B-ALL cases were analysed for IKZF1
Pediatric Oncology and Hematology Research Institute of N.N.Blokhin deletion and IKZF1Plus profile using MLPA based probes (P-335 and
National Research Cancer Center, Hemoblastoses Chemotherapy, Moscow, P-202). IKZF1Plus profile was defined by presence of IKZF1 deletion
Russian Federation with an additional deletion of CDKN2A/2B, PAX5 or PAR1 region, in
the absence of ERG deletion.
Background and Aims: Acute lymphoblastic leukaemia (ALL) is the Results: The mean age of cohort was 5.3 years and mean TLC
most common childhood cancer. The great progress in treatment has at presentation was 82000/ul. Forty eight patients (N=175, 27%)
been made with BFM (Berlin-Frankfurt-Munster) protocols. Overall had a positive MRD and eleven (N=176, 6.2%) had remission fail-
survival rate in pediatric patients with ALL is over 90%. We present the ure post induction. Sixty three (N=118, 54%) patients were positive
results of Pediatric ALL Russian group with ALL IC-BFM 2002 protocol. for recurrent translocations and/or hyperdiploidy. As per final risk
Evaluated long-term overall survival (OS), event-free survival (EFS) and categorisation, 78/178(44%) were high risk, 58(33%) intermediate
disease- free survival (DFS) for ALL with ALL IC-BFM 2002 protocol. and 41(23%) were of standard risk. Forty (N=178, 22%) cases had
Methods: From 2003 to 2021, 451 patients with primary ALL were an IKZF1 deletion, out of which 27 (67%) had an IKZF1Plus pro-
enrolled in ALL IC-BFM 2002 protocol. Median age was 7.2 year file. Relapse free survival (RFS) and event free survival (EFS) at 4
(range 0-21). Т-ALL was diagnosed in 150 (33,3% of patients), В-ALL years for IKZF1 deletion group was statistically poor as compared
301 (66,7%). At diagnosis and after induction therapy, the patients to non-IKZF1 deletion group (58% vs. 78%, p=0.046; 38% vs. 60%,
were stratified into three groups: standard (SR), intermediate (IR), and p=0.0103); however no difference for overall survival (OS) was noted.
high-risk (HR) groups. RFS and EFS at 4 years was even worse for IKZF1Plus group as
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S181 of S635

compared to non- IKZF1Plus group (50% vs. 70%, p=0.026; 36% vs. third of our infants were infected with MDR/XDR bacteria and these
58%, p=0.0103). were predominantly GNB.
Conclusions: IKZF1 deletions is associated with unfavorable clinical
outcome in pediatric B-ALL cohort. Our data highlights the importance
of routine incorporation of testing for IKZF1 deletion and plus profile EP071 / #633 RISK AND BENEFIT OF LESS INTENSIVE
for better risk stratification in treatment trials. TREATMENT FOR STANDARD RISK GROUP OF CHILDHOOD
ALL, AN EXPERIENCE IN LMIC

EP070 / #1348 BACTERIAL ISOLATES AMONG INFANTS Sutaryo Sutaryo1 , Rahmadani Lestari1 , Claudia Adelin1 , Anjo
WITH LEUKEMIA Veerman1,2
1 Universitas Gadjah Mada, Department Of Pediatrics, Yogyakarta, Indone-
Iyad Sultan1 , Anwar Al-Nassan2 , Sawsan Mubarak3 , Mayada Abu sia; 2 Emma Children’s Hospital, Amsterdam UMC, Vrije Universiteit Amster-
Shanap4 dam, Pediatric Oncology, Amsterdam, Netherlands
1 King Hussein Cancer Center, Pediatrics, Amman, Jordan; 2 King Husien
Casncer Center, Pediatric, AMMAN, Jordan; 3 King Hussein Cancer Center, Background and Aims: It is challenging to develop less toxic
Infection Control, Amman, Jordan; 4 king Hussein cancer center, Pediatric, chemotherapy regimen in LMIC with limited resources. ALL2013
Amman, Jordan showed too many toxic deaths and abandonments. Our study pro-
vides an experience of two protocols, Indonesian ALL2013 SR and
Background and Aims: Infantile leukemia has worse outcomes when a lower intensity protocol, ALL2016 SR. The major changes in
compared to other childhood leukemias. Understanding patterns of ALL2016 were omitting the anthracycline in induction, shortening
bacterial infections and spectrum of antibimicrobial resistance may the pulses of two weeks dexamethasone in maintenance into one
help in managing these patients. week, and rescheduling L-asparaginase from three to two times a
Methods: Patients with infantile leukemia diagnosed from Jan- week.
uary2006 and January2021 were identified. We linked there records Methods: SR group was defined as age 1-10 years, leucocyte count less
to our microbiology records. We selected only first-isolates to than 50x109 /L, no CNS involvement, no mediastinal mass, no T-cell
remove duplicates and repeated positive cultures caused by the same phenotype, and good prednisone response. The 4-year-pOS and pEFS
pathogen. Antimicrobial resistance were used to label samples as were analyzed using Kaplan Meier method.
Negative, Multidrug-resistant (MDR),and extensively drug-resistant Results: ALL2013 SR included 106 patients and ALL2016 91 patients.
(XDR) according to the Clinical Laboratory Standards Institute (CLSI) ALL2016 showed a non-significant advantage for SR patients (4-year-
guidelines. pEFS 56.0% vs 47.2%; P=0.220 and 4-year-pOS 70.3% vs 61.3%;
Results: We identifed 32 patients (ALL, 18; AML, 14; 48% females). P=0.166) due to less toxic deaths (7% vs 20%; P=0.011) and less aban-
The median age was 0.58 year (range, 0.14-1). There were 102 iso- donment (5% vs 11%; P=0.142). The cumulative incidence of relapses
lates, with blood (N=86, 84%) being the most common site. The median in ALL2016 was 40% and in ALL2013 34% (P=0.432). Relapses
number of isoltes per patient were 2(range,1-8) and the median time frequently occurred between weeks 80-120.
of infection after diagnosis was 3.8months(range, 0.1-32). There were Conclusions: Overall, the outcome improved. The less intensive treat-
71 gram-positive(GPB) and 31 gram-negative(GNB) bacteria. GNB rep- ment succeeded to reduce toxic deaths and abandonments, although at
resented 40% of peripheral blood cultures, but only 15% of central the cost of more relapses.
catheter cultures. The most common pathogens were coagulase-
negative staphylococcus species(CONS, N=44, 43%), followed by
Viridans Group Streptococcus(VGS, N=11, 11%), E. Coli(N=8, 7.8%), EP072 / #124 ASPARAGINASE-SPECIFIC ANTIBODY
Klebsiella pneumoniae(N=8, 7.8%), Pseudomonas aeruginosa(N=5, MEASUREMENT IN PEDIATRIC ACUTE LYMPHOBLASTIC
4.9%) and Streptococcus pneumoniae(N=4, 3.9%). Sixteen isolates LEUKEMIA (ALL) UNDER NATIVE E. COLI
were labeled as MDR(16%): E coli(N=7), Klebsiella pneumoniae(N=5), ASPARAGINASE-BASED AND ITS POTENTIAL FACTORS
Staphylococcus aureus(N=2), Enterococcus faecium(N=1) and Serra- ASSOCIATION
tia odorifera(N=1). Only one respiratory sample grew an XDR Acineto-
bacter calcoaceticus. These resistant isolates(N=17) were identified in Piti Techavichit, Phumin Chaweephisal, Hansamon Poparn, Kanhatai
11 patient (median=1 isolate per patient). Estimated 4-week survival Chiengthong, Supanun Lauhasurayotin, Darintr Sosothikul
after an infection was 94%+/-2.3% and was not different according to Integrative and Innovative Hematology/Oncology Research Unit., Depart-
site of isolate, antimicrobial resistance or gramstain. Also, there was no ment Of Pediatrics, Bangkok, Thailand
difference in survival after having multiple isolates.
Conclusions: Bacterial isolates among infants with leukemia are pre- Background and Aims: Asparaginase is one of the chemotherapy
dominantly gram-positive. Survival of our patients following these backbones for pediatric acute lymphoblastic leukemia (ALL) treat-
infections was acceptable and reflects adequate supportive care. One ment. But its foreign protein origin can produce the antibody which
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S182 of S635 ABSTRACTS

causes an allergic reaction together with lowering activity made drug but a genetic profile capable of sensitively predicting response to
ineffectiveness. chemotherapy induction, improving risk classification, and predicting
Methods: The primary objective is to prove the association of survival is greatly needed. Our objective was to identify genes associ-
asparaginase-specific antibodies with post-48-hours activity. The sec- ated with the response to induction chemotherapy in pediatric patients
ondary objective is to study the potential factors influencing the anti- with B-cell ALL.
body concentration. A single-center cross-sectional study conducted Methods: RNA sequencing and genome-wide DNA methylation assay
from September 2020 through June 2021 at King Chulalongkorn were done in blasts from 27 patients newly diagnosed with B-cell ALL.
Memorial Hospital, Thailand. 51 eligible patients (25 males and 26 Statistics analyses were done using DEseq2 and Partek Flow software.
female) age between 1-15 years old were enrolled. Native E. coli We selected genes that were differentially expressed (DEGs), differ-
asparaginase was administrated intramuscularly. Whole blood was entially methylated, and had a correlation between both. Response to
drawn for measuring post-48-hours activity from the last dose (21 induction treatment was assessed by minimal residual disease (MRD)
cases), and asparaginase-specific antibody at any time (51 cases) which detected by flow cytometry and analyzes were performed between
later processed with the coupled enzymatic reaction. patients who did or did not respond to induction treatment at two-time
Results: The empirical optimal cut-off of antibodies was newly cal- points (15 and 33 days) and complete response (patients who did or did
culated which shows a ratio of 2.2 times above healthy volunteers. not respond on time points). Selected genes were verified with RT-PCR
The mean activity is 1.94 and 2.42 ng/ml for an antibody positive and in a different cohort of patients.
negative group respectively. The asparaginase antibody was proved Results: We identified 50 DEGs in common for all comparisons.
non-linear, independently invert correlation with activity by multivari- Differential methylation analysis showed that 4 of them had differ-
ate mix-model analysis (p-value: 0.031 (95%CI: -1.51 to -0.07). The entially methylated CpGs and had a correlation between expression
asparaginase allergic reaction and asparaginase accumulation dose and methylation levels. Six more interesting genes that met the
were proved not associated but some inflammatory event as proved- previous criteria and were present only in the complete response
invasive fungus infection shows significant positive trend considering were also further validated by RT-PCR. Genes DAPK1, BOC, ITGA6,
as confounding factor (p-value <0.001, 95%CI: 5.06 to 10.90). MIR4435-2HG, and NDPC1 have been confirmed to be overexpressed
Conclusions: Due to weak invert and non-linear antibody-activity rela- in non-responder patients.
tionship and no standard cut-off positivity criterion, our opinion still Conclusions: We identified 5 genes present in patients with poor
affirms to discourage employing the asparaginase-specific antibody response to induction chemotherapy and could be used as predic-
measurement for justification of the treatment change. Furthermore, tive biomarkers of response to chemotherapy in Colombian pediatric
this trial raises the concern of cross-reaction of antibodies with some patients with B-cell ALL.
inflammatory/infection event.

EP074 / #257 MINIMAL RESIDUAL DISEASE COMPARISON


EP073 / #743 EXPRESSED AND DIFFERENTIALLY BETWEEN IG/TCR PCR VERSUS NGS ASSAYS IN CHILDREN
METHYLATED GENES ASSOCIATED WITH INDUCTION WITH PHILADELPHIA CHROMOSOME-POSITIVE ACUTE
TREATMENT RESPONSE IN PEDIATRIC PATIENTS WITH B-CELL LYMPHOBLASTIC LEUKEMIA: A REPORT FROM THE COG
ACUTE LYMPHOBLASTIC LEUKEMIA AALL1631 STUDY

Yulieth Torres-Llanos1 , Jovanny Zabaleta2 , Nataly Cruz-Rodriguez3 , Thai Hoa Tran1 , Shalini Reshmi2 , Ilan Kirsch3 , John Kairalla4 , Sarah
Sandra Quijano4 , Paula Guzman5 , Iliana De Los Reyes5 , Ana Infante6 , Tasian5 , Kirk Schultz6 , Elizabeth Raetz7 , Mary Shago8 , Andrew
Liliana Lopez7 , Alba Combita1 Carroll9 , Meenakshi Devidas10 , Stephen Hunger5 , Mignon Loh11 ,
1 Instituto Nacional de Cancerología, Cancer Biology Group, BOGOTA, Lewis Silverman12
Colombia; 2 Louisiana State University Health Sciences Center, Depart- 1 CHU Sainte-Justine, Pediatrics, Montreal, Canada; 2 Nationwide Children’s

ment Of Interdisciplinary Oncology, New Orleans, United States of Amer- Hospital, Laboratory Medicine/anatomic Pathology, Columbus, United
ica; 3 Universidad Industrial de Santander, Microbiology School, Bucara- States of America; 3 Adaptive Biotechnologies, Translational Medicine,
manga, Colombia; 4 Pontificia Universidad Javeriana, Microbiology Depar- Seattle, United States of America; 4 University of Florida, Biostatistics,
ment, Bogotá, Colombia; 5 Hospital Militar Central, Pediatrics Deparment, Gainesville, United States of America; 5 Children’s Hospital of Philadel-
Bogotá, Colombia; 6 Hospital Universitario San Ignacio, Pediatrics Depar- phia, Pediatrics, Philadelphia, United States of America; 6 BC Chil-
ment, Bogotá, Colombia; 7 Universidad Nacional de Colombia, Statistics dren’s Hospital and Research Institute, Pediatrics, Vancouver, Canada;
Deparment, Bogotá, Colombia 7 NYU Langone Health, Pediatrics, New York, United States of Amer-
ica; 8 Hospital for Sick Children, Pediatric Laboratory Medicine, Toronto,
Background and Aims: Colombian survival rate for pediatric acute Canada; 9 University of Alabama at Birmingham, Genetics, Birming-
lymphoblastic leukemia (ALL) is less than 60%, which could be related ham, United States of America; 10 St Jude Children’s Research Hos-
to genetic and epigenetic alterations specific to our population. Some pital, Global Pediatric Medicine, Memphis, United States of Amer-
genetic alterations are used to classify patients into a risk group, ica; 11 Seattle Children’s Hospital, Pediatrics, Seattle, United States of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S183 of S635

America; 12 Dana-Farber Cancer Institute, Pediatrics, Boston, United States associated with hematological and hepatic toxicities with germline
of America genetic variations being determinants of interpatient variability in sus-
ceptibility, drug response, and toxicity. NUDT15 & TPMT are the main
Background and Aims: Minimal residual disease (MRD) assessment SNP’s implicated, incidence varying with ethnicity. This prospective
by immunoglobulin/T-cell receptor (Ig/TCR) polymerase chain reaction study aimed at evaluating the prevalence of SNP’s in children who
(PCR) is currently being used in the international pediatric Philadel- tolerated lower doses of 6-MP.
phia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) Methods: One hundred and ninety children with ALL (age: 5.29±2.9
trial EsPhALL2017/COG AALL1631 for risk stratification. MRD con- years) were evaluated for the NUDT15 (c.415C>T) & TPMT *2 (c.238
cordance has previously been demonstrated between Ig/TCR PCR and G>C), *3A (c.460 G>A and c.719 A>G),*3B (c.460 G>A) and *3C (c.719
flow cytometry. We sought to assess concordance of MRD assessment A>G) SNP prior to start of MT. Data with regards to 6-MP dosing and
between conventional Ig/TCR PCR and next-generation sequencing toxicities over 6 months of MT was noted.
(NGS) assays. Results: NUDT15 and TPMT SNP’s were demonstrated in 12% and
Methods: MRD was assessed in all patients on AALL1631 by Ig/TCR 4.5% patients respectively (all heterozygous, one patient testing pos-
PCR at end-induction IB; those with MRD <5x10-4 were classified itive for both). The dose of 6-MP in patients with the NUDT15
as standard-risk (SR), whereas patients with MRD >=5x10-4 were SNP was lower (49±8.1mg/m2/day vs. 54±6mg/m2/day (p=.002). Ten
considered high-risk (HR) and assigned to hematopoietic stem cell patients with NUDT15 SNP received ≥50/mg/m2/day but had higher
transplant (HSCT). Residual diagnostic and end-induction IB samples dose interruptions [4 weeks (IQR: 2-8] as compared to those with-
from consenting patients were assessed for NGS MRD and sub- out SNP [2weeks (IQR1-4)] (p=.06). Only 2/7 children with TPMT
sequently compared to Ig/TCR MRD to determine concordance as SNP required dose reduction. Fifty seven of 190 children on MT
related to MRD-based HSCT recommendations. MRD values were (30%) received ≤50mg/m2/day of 6-MP (mean 45.46±5.9mg/m2/day),
calculated using the kappa statistic for agreement above chance. with 13/57 receiving ≤40mg/m2/day (36.3±4.8 mg/m2/day). Fif-
Results: Sixty-seven patients had matched samples available for MRD teen children (26%) who received lower doses had a demonstrable
assessment at end-induction 1B by both Ig/TCR PCR and NGS. NGS SNP (13: NUDT15 and 2 TPMT). Five patients (38%) who received
MRD was evaluable for all 67 patients and stratified as 62 SR and 5 ≤40mg/m2/day demonstrated a SNP (NUDT15 in all).
HR. In contrast, Ig/TCR PCR results were inevaluable for 3 patients Conclusions: Only 26% cases of ALL on MT tolerating lower doses
(unsatisfactory sample quality) and indeterminate (positive, but not demonstrated a known SNP. A lower dose in 42/57 children could
quantifiable) in 4 patients. Of the remaining 60 patients, 55 met SR not be attributed to the known SNP’s. Whole exome sequencing
and 5 HR criteria using Ig/TCR PCR. There was only 1 discordant case (WES) or large scale GWAS (Genome wide association studies) stud-
between the two methods for MRD-based HSCT recommendation ies are required to identify novel candidate SNPs that are likely to
among these 60 patients with a kappa statistic for agreement above be associated with 6mercaptopurine myelotoxicity in south east Asian
chance of 0.88. population
Conclusions: NGS and Ig/TCR PCR assays were highly concordant in
MRD assessment for risk stratification in pediatric Ph+ ALL patients
enrolled on AALL1631. The NGS assay yielded MRD results amenable EP076 / #8 PHARMACOGENOMICS OF
for risk stratification in 100% patients compared to 89.6% for the VINCRISTINE-INDUCED PERIPHERAL NEUROPATHY IN
Ig/TCR PCR methodology. These data support the use of NGS MRD CHILDREN WITH CANCER: A SYSTEMATIC REVIEW AND
testing for risk stratification in pediatric Ph+ ALL. META-ANALYSIS

Aniek Uittenboogaard, Celine Neutel, Johannes Ket, Festus Njuguna,


EP075 / #991 SINGLE NUCLEOTIDE POLYMORPHISMS IN Alwin Huitema, Gertjan Kaspers, Mirjam Van De Velde
THIOPURINE ASSOCIATED MYELOSUPPRESSION IN Amsterdam UMC - Location VUmc, Pediatric Oncology/-hematology, Ams-
MAINTENANCE THERAPY IN ACUTE LYMPHOBLASTIC terdam, Netherlands
LEUKEMIA: THE MYSTERY REMAINS
Background and Aims: Vincristine-induced peripheral neuropathy
Amita Trehan1 , Minu Singh1,2 , Prateek Bhatia2 , Deepak Bansal1 , Richa (VIPN) is a debilitating side-effect of vincristine in children with can-
Jain1 cer. It remains a challenge to predict which patients will suffer from
1 Advanced Pediatrics Center, Postgraduate Institute of Medical Educa- VIPN. Pharmacogenomics may explain an individuals’ susceptibility to
tion and Research, Pediatric Hematology Oncology Unit, Chandigarh, India; side-effects. In this systematic review and meta-analysis, we describe
2 Postgraduate Institute of Medical Education and Research, Pediatrics, the influence of pharmacogenomic parameters on the development of
Chandigarh, India VIPN in children with cancer.
Methods: PubMed, Embase and Web of Science were searched up to
Background and Aims: Mercaptopurine (6-MP), the mainstay of 30-09-2021. In total, 1597 records were identified and 21 studies were
maintenance therapy (MT) in acute lymphoblastic leukemia (ALL), is included. A random-effects meta-analysis including nine studies was
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S184 of S635 ABSTRACTS

performed for the influence of cytochrome P450 (CYP) 3A5 expression models. We added an interaction factor to test whether evolution over
on the development of VIPN. time is subject to age.
Results: Single-nucleotide polymorphisms (SNPs) in four transporter- Results: The strongest decreases in Z-scores are observed after induc-
associated genes (e.g. ATP binding cassette subfamily C member 2 tion therapy in quadriceps strength (Z=-1.36 to Z=-2.62), SBJ (Z=-1.15
(ABCC2)), three metabolism-associated genes (e.g. vitamin D recep- to Z=-2.22) and 6MWT scores (Z=-2.20 to Z=-3.57). Age at diagno-
tor (VDR)), six cytoskeleton-associated genes (e.g. centrosomal protein sis is a significant predictor for tibialis anterior strength (p=0.025),
72 (CEP72)), one hereditary neuropathy-associated gene (solute car- SBJ (p<0.001) and 6MWT (p<0.001) performance. Adolescents (>13
rier family 5 member 7 (SLC5A7)), and ten genes previously unrelated years) tend to have lower results on all performed tests from diagnosis
to vincristine or neuropathy (e.g. Ewing’s tumor-associated antigen 1 onwards. Muscle strength seems to recover 6 months after treatment
(ETAA1)) were associated with VIPN. CYP3A5 expression status was (Z=-0.8 for quadriceps strength, Z=0.5 for tibialis anterior strength).
not significantly associated with VIPN (pooled odds ratio 0.69, 95% However, 6MWT and SBJ scores remained below expected levels,
confidence interval 0.38-1.26, I2 = 50%, τ2 = 0.33). The comparison especially for adolescents (Z=-1.63 and Z=-2.58 respectively).
and interpretation of the results of the included studies was limited Conclusions: We conclude that future interventions might need to tar-
due to heterogeneity in the study population, treatment protocol and get early decline in PF after induction phase, the low functional mobility
assessment methods and definitions of VIPN. and endurance post-treatment, specifically in the adolescent sub-
Conclusions: Pharmacogenomic parameters have a significant influ- group. These observations can be used to better tailor individualized
ence on VIPN in children with cancer and show potential for clinical physiotherapy throughout treatment.
relevance. However, CYP3A5 expression status was not a significant
risk factor. Independent replication is essential to validate the clinical
significance of the reported associations. Future research should aim EP078 / #1044 EVALUATION OF SKIN TOXICITY PROFILE OF
for prospective VIPN assessment in a discovery and replication cohort. HIGH DOSE METHOTREXATE THERAPY IN CHILDHOOD ACUTE
Ultimately, the goal would be to stratify patients based on the presence LYMPHOBLASTIC LEUKEMIA
of SNPs and provide a tailored dosage that limits the risk of VIPN while
maintaining the highest therapeutic efficacy. Tandra Harish Varma, Priyakumari Thankomany, Manjusha Nair,
Binitha Rajeshwari, Guruprasad Cs, Prasanth Vr
Regional Cancer Centre, Department Of Pediatric Oncology, Thiruvanan-
EP077 / #340 PHYSICAL FITNESS THROUGHOUT thapuram, India
CHEMOTHERAPY IN CHILDREN WITH ACUTE LYMPHOBLASTIC
LEUKAEMIA Background and Aims: High dose Methotrexate(HDMTX) has a vari-
ety of cutaneous side effects. In this study we assessed the skin toxicity
Annelies Vriens1 , Sabine Verschueren2 , Deveny Vanrusselt1 , Marine profile of HDMTX in acute lymphoblastic leukemia(ALL) in a Low-
Van Hollebeke2 , Thierry Troosters2 , Marjoke Gielis1 , Veerle Dirix2 , Middle Income countries(LMIC) setting with detailed monitoring of
Charlotte Sleurs2 , Anne Uyttebroeck1 clinical and lab parameters along with limited serum MTX estimation.
1 KULeuven, Department Of Oncology, Leuven, Belgium; 2 KULeuven, Methods: This is a prospective observational study done from 1st Jan-
Department Of Rehabilitation Sciences, Leuven, Belgium uary 2019 to 31st December 2019. Children with age<14 years diag-
nosed with High Risk ALL who received a total of 4 HDMTX courses
Background and Aims: Due to improving survival rates of patients were included. Administration of HDMTX was done by trained nurs-
with childhood acute lymphoblastic leukaemia (ALL), there is an ing staff at a dose of 5gram/metre2 in an inpatient setting with strict
increasing focus on long-term effects of treatment. ALL and its treat- hydration and alkalinization with urine pH monitoring. Data regarding
ment cause a range of physiological changes, interfering with normal skin and toxicity profile, leucovorin rescue and serum MTX levels start-
physical functioning. So far, it remains unclear how physical fitness (PF) ing at 54-hour from start of HDMTX until subsequent normalization
(including muscle strength, functional mobility and endurance) evolves (<0.2micromole/liter) were analyzed.
throughout treatment for ALL. Results: During the study period, 488 HDMTX infusions were admin-
Methods: Sixty-two patients treated for ALL according to the istered. Skin toxicity was noted in 13.1%(n-64) of infusions. Most
EORTC 58081 protocol underwent physical testing at nine timepoints common manifestation was focal and diffuse hyperpigmentation(n-
throughout their 2-year-treatment (last measurement at 6 months 25). Others included itchy papular rash(n-14), bullous lesions(n-11),
post-treatment). Four tests were conducted. Quadriceps and tibialis skin peeling(n-6), perioral, perianal and eyelid excoriation(n-4), itchy
anterior muscle strength were assessed using a hand-held dynamome- crusty rash (n-3) and generalized desquamation(n-1). Mean day of
ter. Standing broad jump test (SBJ) and six-minute walk test (6MWT) onset was 6.4(SD 2.01) days from start of HDMTX. Majority were
were assessed for functional mobility and endurance, respectively. managed with supportive care. One infusion required intravenous
Z-scores were calculated based on gender- and aged-matched test- methyl prednisolone and extra doses of folinic acid. Mean duration for
specific normative values, which were predicted based on time of symptom resolution was 5.4(SD 1.82) days. Among all the 4 HDMTX
assessment, ALL risk group and age at diagnosis, using linear mixed infusions, the incidence of skin toxicity progressively decreased with
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S185 of S635

highest in 1st cycle(26.2%) and lowest in 4th cycle(4.1%)(p-0.001). A cut-off point of ≥5 had the highest specificity of 96% and sensitivity
There was no relation between delayed MTX clearance with skin toxic- of 74% for functional sarcopenia.
ity. Among other toxicities, skin toxicity had significant association with Conclusions: We adapted the SARC-F to a pediatric version and con-
thrombocytopenia and infection(P-0.017 and 0.001 respectively). firmed its excellent accuracy for identifying functional sarcopenia and
Conclusions: Skin toxicity can be observed in limited number of infu- defined a clinically useful cut-off in a pediatric hemato-oncology set-
sions (<15%) when HDMTX is given at 5 gram/meter2 with highest ting. This easy self-report score can identify children that may need
incidence being in the 1st cycle. Nearly all skin manifestations are physiotherapy interventions during and shortly after treatment.
transient and can be managed with supportive care.

EP080 / #713 POINT-OF-CARE MUSCULOSKELETAL


EP079 / #249 NOVEL ADAPTION TO THE PEDIATRIC SARC-F ULTRASOUND CORRELATES WITH BODY COMPOSITION,
SCORE TO CLASSIFY PEDIATRIC HEMATO-ONCOLOGY MUSCLE STRENGTH AND PHYSICAL PERFORMANCE IN
PATIENTS WITH FUNCTIONAL SARCOPENIA CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Emma Verwaaijen1 , Patrick Van Der Torre1 , Josef Vormoor1,2,3 , Rob Emma Verwaaijen1 , Annelienke Van Hulst1 , Jeroen Molinger2 ,
Pieters1 , Marta Fiocco1,4 , Annelies Hartman5 , Marry Van Den Annelies Hartman3 , Martha A. Grootenhuis1 , Erica Van Den Akker4 ,
Heuvel-Eibrink1 Marry Van Den Heuvel-Eibrink1
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, 1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology,
Utrecht, Netherlands; 2 University Medical Center Utrecht, Utrecht Can- Utrecht, Netherlands; 2 Erasmus Medical Center, Department Of Intensive
cer Center, Utrecht, Netherlands; 3 Newcastle University, Cancer Institute, Care, Rotterdam, Netherlands; 3 Erasmus Medical Center-Sophia Children’s
Newcastle upon Tyne, United Kingdom; 4 Leiden University, Mathematical Hospital, Department Of Pediatric Physiotherapy, Rotterdam, Nether-
Institute, Leiden, Netherlands; 5 Erasmus Medical Center-Sophia Children’s lands; 4 Erasmus Medical Center-Sophia Children’s Hospital, Department Of
Hospital, Department Of Pediatric Physiotherapy, Rotterdam, Netherlands Endocrinology, Rotterdam, Netherlands

Background and Aims: The SARC-F questionnaire is recommended Background and Aims: Non-invasive and reliable tools to measure
as a screening tool for sarcopenia in elderly, and consists of five self- muscle mass and muscle quality in children with acute lymphoblastic
reported questions addressing strength, walking, rising, stairclimbing leukemia (ALL) are limited.
and falling. The aim of this study was to investigate the accuracy of The aim of the study was to explore the association of muscle
the pediatric SARC-F (PED-SARC-F), for identifying sarcopenia in pedi- ultrasound outcomes with standard-of-care assessments of body com-
atric hemato-oncology patients, including determination of a cut-off position, muscle strength and physical performance in children with
for clinical use. ALL.
Methods: Patients 3-20 years, under active treatment or within 12 Methods: In this cross-sectional study we included Dutch ALL patients,
months after treatment cessation at the hemato-oncology department aged 3-18 years during maintenance therapy.
of the Princess Máxima Center for Pediatric Oncology, were eligi- Bilateral ultrasound measurements of the rectus femoris (RF) muscles
ble. Patients had a physiotherapy assessment including PED-SARC-F, were captured using a portable linear array transducer connected to
as part of standard of care. The physiotherapy assessment consisted a tablet. Artificial intelligence image analysis was used to estimate RF
of muscle strength measures (handheld dynamometry), physical per- muscle thickness, cross-sectional area, intramuscular adipose tissue
formance (various tests) and muscle mass (bio-impedance analyses). (IMAT) and raw pixel intensity (RPI), as a proxy for glycogen.
Structural sarcopenia was defined as low muscle mass in combination Assessments of body composition (bio-impedance analysis and thigh
with low muscle strength and/or low physical performance. Functional circumference), muscle strength (hand-held dynamometry) and physi-
sarcopenia indicated low muscle strength combined with low physical cal performance (timed up and go test [TUG] and time to rise from floor
performance. test [TRF]) were performed during the same visit.
Multiple logistic regression models were estimated to study the asso- Spearman’s rank correlation analyses (rho) were calculated to study
ciations between PED-SARC-F, and the endpoints: structural and correlations between ultrasound outcomes and measures of body
functional sarcopenia. To evaluate which PED-SARC-F cut-off point composition, muscle strength and physical performance.
(0-10) provides the most accurate classification of functional sarcope- Results: Muscle ultrasound was performed in 60 patients, 37/60 boys
nia, the area under the receiver operating characteristic curve (AUCs), (61.7%), median age 6.1 years (range: 3–18.8 years).
sensitivity and specificity per point were calculated. RF thickness and cross-sectional area correlated moderately with mus-
Results: In total, 215 assessments were included, 62% were performed cle mass (rho=0.58, rho=0.6), handgrip strength (rho=0.6, rho=0.65),
in boys and median age was 12.9 years (IQR: 8.5-15.8). knee extension strength (rho=0.65, rho=0.68), and highly with thigh
The PED-SARC-F had an AUC of 0.90 (95%CI = 0.84-0.95) for circumference (rho=0.76, rho=0.78). IMAT correlated moderately
functional sarcopenia and 0.69 (95%CI = 0.57-0.80) for structural with TUG (rho=0.52) and TRF (rho=0.53), i.e. increased fat infiltra-
sarcopenia. tion correlated with slower performance. RPI correlated moderately
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S186 of S635 ABSTRACTS

with handgrip strength (rs=-0.48), knee extension strength (rho=0.51), manual filters to discard false-positive rearrangements improving the
and TUG (rho=0.5), lower glycogen correlated with lower strength and precision to 36.7% (cell lines) and 84% (patients).
slower performance. All p-values were <0.001. Conclusions: The five fusion caller pipelines worked well individually
Conclusions: Muscle ultrasound may be useful for measuring mus- but missed some fusions. The best strategy to achieve a high sensitiv-
cle mass and muscle quality in children with ALL, allowing rapid and ity is to combine results from all five callers and keep those fusions
non-invasive assessments. Further validation using golden standard called by 3/5 pipelines. Moreover, manual filtering would improve
assessments in children is needed to determine the accurateness in precision.
pediatric populations.

EP082 / #828 IMMUNOPHENOTYPIC AND CHROMOSOMAL


EP081 / #643 COMPARATIVE ASSESSMENT OF DIFFERENT CHARACTERIZATION OF KMT2A GERMLINE ACUTE
BIOINFORMATIC PIPELINES FOR GENE FUSION DETECTION LYMPHOBLASTIC LEUKEMIA IN INFANTS
FROM RNA-SEQ DATA IN ACUTE PEDIATRIC LEUKEMIA
Hiroki Yoshihara1 , Takako Miyamura2 , Takao Deguchi3 , Toshinori
Clara Vicente Garcés1,2 , Joan Maynou3,4 , Guerau Fernández3,4 , Elena Hori4 , Tomohiko Taki5 , Kunihiko Moriya6 , Yuki Arakawa7 , Mariko
Esperanza Cebollada1,2 , Montserrat Torrebadell1,2,5 , Albert Eguchi8 , Kunihiro Shinoda9 , Yuhki Koga10 , Katsuyoshi Koh7 , Atsushi
Català2,5,6 , Susana Rives2,5,6 , Mireia Camós1,2,5 , Nerea Vega García1,2 Manabe11 , Keizo Horibe12 , Daisuke Tomizawa3
1 Pediatric Cancer Center Barcelona (PCCB), Hospital Sant Joan de 1 St. Luke’s International Hospital, Pediatrics, Tokyo, Japan; 2 Osaka Uni-
Déu Barcelona, Hematology Laboratory, Esplugues de Llobregat, Spain; versity, Pediatrics, Suita, Japan; 3 National Center for Child Health and
2 Pediatric Cancer Center Barcelona (PCCB), Institut de Recerca Sant Joan Development, Children’s Cancer Center, Tokyo, Japan; 4 Aichi Medical Uni-
de Déu, Leukaemia And Paediatric Haematology Disorders, Developmental versity, Pediatrics, Nagakute, Japan; 5 Kyorin University Faculty of Health
Tumors Biology Group, Esplugues de Llobregat, Spain; 3 Hospital Sant Joan Sciences, Medical Technology, Mitaka, Japan; 6 Tohoku University Graduate
de Déu Barcelona, Genetics Medicine Section, Esplugues de Llobregat, Spain; School of Medicine, Pediatrics, Sendai, Japan; 7 Saitama Children’s Medical
4 Institut de Recerca Hospital Sant Joan de Déu, Neurogenetics And Molec- Center, Hematology/oncology, Saitama, Japan; 8 Ehime University Gradu-
ular Medicine, Esplugues de Llobregat, Spain; 5 Instituto de Salud Carlos III, ate School of Medicine, Pediatrics, Toon, Japan; 9 Gifu Municipal Hospital,
Centro De Investigación Biomédica En Red De Enfermedades Raras (ciberer), Pediatrics, Gifu, Japan; 10 Kyushu University, Pediatrics, Fukuoka, Japan;
Madrid, Spain; 6 Pediatric Cancer Center Barcelona (PCCB), Hospital Sant 11 Hokkaido University Graduate School of Medicine, Pediatrics, Sapporo,
Joan de Déu Barcelona, University of Barcelona, Pediatric Hematology And Japan; 12 Nagoya Medical Center, Clinical Research Center, Nagoya, Japan
Oncology, Barcelona, Spain
Background and Aims: KMT2A germline (KMT2A-g) acute lym-
Background and Aims: Background: RNA sequencing (RNA-seq) is a phoblastic leukemia (ALL) configure approximately 20% of ALL in
well-established tool for detecting gene fusions in acute leukemia. Mul- infants, and show distinct clinical and genetic features (e.g., NUTM1
tiple bioinformatics pipelines have been developed to analyze RNA-seq rearrangement), and better prognosis compared with KMT2A gene-
data in leukemia, but an agreed gold standard has not been established. rearranged (KMT2A-r) ALL. However, its pathophysiology remains
Aim: To compare and define an integrative bioinformatics pipeline to obscure due to the rarity of the disease. We aimed to investigate
detect gene fusions in acute pediatric leukemia. immunophenotypic and chromosomal characteristics in infants with
Methods: Applicability assessment of five fusion calling pipelines KMT2A-g ALL.
(Arriba, deFuse, CICERO, FusionCatcher, and STAR-Fusion) individu- Methods: Patients registered to the Japanese Pediatric
ally, and development of a set of scripts that standardize and combine Leukemia/Lymphoma Study Group MLL-10 trial (Tomizawa D. Blood
fusion results of all of them to detect leukemia driver fusion genes in 2020) were analyzed.
data from cell lines and pediatric patients. Results: Fifteen infants with KMT2A-g ALL were identified. Median
Results: We analyzed RNA-seq data from 18 cell lines and 15 previ- age at diagnosis was 307 days, and three patients were <180 days
ously characterized pediatric patients with leukemia. Each algorithm old. Skewing towards male sex was observed (67%). Median white
individually called most of the fusions with similar sensitivity and preci- blood cell counts at diagnosis were 43.7 x109 /L. No patients showed
sion. However, not all rearrangements were called, so choosing a single CNS involvement. Unsupervised clustering analysis of leukemic cell
pipeline might cause the missing of important fusions. To solve this, we immunophenotype grouped many of the KMT2A-g ALL patients,
integrated the results of the five algorithms in just one pipeline. We which differentiated from the KMT2A-r ALL patients. Dominant cell
compared the output from the agreement of 5/5, 4/5, and 3/5 algo- surface antigens expressed in KMT2A-g ALL were CD10, CD20,
rithms. The maximum sensitivity was achieved with the agreement of CD24, cytoplasmic CD22, and cytoplasmic Igμ, whereas expression of
3/5 algorithms, with a sensitivity of 79% in cell lines and a sensitivity of CD34, NG2, CD15, and CD65 were low. Karyotype of KMT2A-g ALL
100% in patient data. The obtained precision for the agreement of 3/5 showed chromosomal translocation, deletion, or addition of chromo-
algorithms was 10.2% and 23% for cell lines and patients, respectively, some 15 in 7 cases among the 13 cases analyzed. One case showed
due to the high rate of false-positive variants. We applied different t(1;19)(q23;p13.3) and TCF3-PBX1. Minimal residual disease at the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S187 of S635

end of induction was negative in all patients analyzed either by flow diomyocytes as opposed to daunorubicin. Exposure of BCP-ALL cells
cytometry and Ig/TCR PCR. Three cases showed poor response to to gimatecan triggered dose- and time-dependent cell death, G2/M
initial prednisolone therapy; one patient developed secondary acute cell cycle arrest, caspase/PARP/Bax cleavage, suggestive of extrinsic
myeloid leukemia with KM2TA-MLLT3, but was successfully treated apoptosis. Transcriptome profiling of gimatecan-treated cells revealed
with hematopoietic cell transplantation. The 3-year and 5-year EFS up-regulation of the p53 tumor suppressive and down-regulation of the
and OS rates of all infants with KMT2A-g ALL were 93.3% and 100%, MYC oncogenic pathways. Effectors downstream of p53 such as Chk1
respectively. and Chk2 were activated by gimatecan, indicating DNA damage.
Conclusions: KMT2A-g ALL showed distinct immunophenotypic and Conclusions: Gimatecan is an exceptionally potent agent against child-
karyotypic profiles compared to KMT2A-r ALL. While aberration of hood BCP-ALL. Its tolerable toxicity profile favors further development
chromosome 15 suggests involvement of NUTM1 gene rearrange- for resistant leukemia and should be prioritized for clinical trials.
ments, further analysis will be needed to elucidate mechanisms of this
rare disease.
E-Poster Topic: AS05 SIOP Scientific programme / AS05.b Myeloid
Leukemias, Myelodysplastic and Myeloproliferative Syndromes
EP083 / #1358 THE TOPOISOMERASE-I INHIBITOR
GIMATECAN POTENTLY SUPPRESSES B-CELL PRECURSOR E-POSTER VIEWING
ACUTE LYMPHOBLASTIC LEUKEMIA WITHOUT CONCURRENT
CARDIOTOXICITY EP084 / #181 CHILDHOOD ACUTE PROMYELOCYTIC
LEUKEMIA IN IRAQ: A 16-YEAR EXPERIENCE IN A PEDIATRIC
Chi Zhang1 , Po Yi Lee1 , Hung Sing Li1 , Kathy Yuen Yee Chan1 , Wai CANCER REFERRAL CENTRE IN BAGHDAD
Man Leung1 , Maxwell Kwok2 , Po Yee Chung1 , Wing Hei Ng1 , John Tak
Kit Cheung1 , Han Wang1 , Chi Kong Li1 , Wai Yip Lam3 , Benjamin Li3 , Mazin Al-Jadiry1 , Anna Testi2 , Hasanein Ghali1 , Samaher Fadhil3 ,
Ellen Ngar Yun Poon2 , Kam Tong Leung1 Amir Al-Darraji3 , Raghad Al-Saeed3 , Ahmed Sabhan3 , Maria Luisa
1 The Chinese University of Hong Kong, Department Of Paediatrics, Hong Moleti2 , Valentina Arena4 , Robin Foà2 , Salma Al-Hadad1
Kong, Hong Kong PRC; 2 The Chinese University of Hong Kong, School Of 1 College of Medicine, University of Baghdad, Oncology Unit-Children Wel-
Biomedical Sciences, Hong Kong, Hong Kong PRC; 3 Lee’s Pharmaceutical fare Teaching Hospital-Medical City., Department Of Pediatrics, Baghdad,
(HK) Limited, Lee’s Pharmaceutical (hk) Limited, Hong Kong, Hong Kong PRC Iraq; 2 Sapienza University, Hematology, Department Of Translational And
Precision Medicine, Rome, Italy; 3 Oncology Unit, Children Welfare Teach-
Background and Aims: Topoisomerase-II inhibitors such as daunoru- ing Hospital-Medical City, Pediatrics, Baghdad, Iraq; 4 Fondazion GIMEMA,
bicin are the standard chemotherapeutic agents for treatment of acute Central Office, Rome, Italy
leukemia. However, their well-recognized myelosuppressive and car-
diotoxic natures could mediate severe complications and unwanted Background and Aims: Despite advances in the treatment of children
late effects. This study aims to evaluate the efficacy, toxicity and mech- with acute promyelocytic leukemia (APL), survival rates in low-income
anism of a topoisomerase-I inhibitor, gimatecan, in a preclinical setting countries remain poor. In September 2003, within a collaborative pro-
of childhood leukemia. gram between Children-Welfare-Teaching-Hospital in Baghdad and
Methods: Activities of gimatecan were screened in vitro on BCP-ALL Hematology, “Sapienza”, University of Rome, a specific all-trans retinoic
and AML cell lines, and on CD34+ hematopoietic stem/progenitor cells (ATRA)-based protocol was designed for the management of Iraqi
(HSPCs). Efficacy in vivo was assessed in cell line- and patient-derived children with APL and adapted to local difficulties.
xenografts of leukemia. Hematologic toxicity was evaluated on an ani- Methods: Children (age<17 years) with a morphological diag-
mal model of stem cell transplantation. Cardiotoxicity was measured nosis of APL entered the study. Treatment consisted of ATRA
on human iPSC-derived cardiomyocytes. Mechanisms of action were (25mg/m2 /dayx30days) induction, associated with an anthracycline
dissected by RNA-seq and validated by Western blot. for high-risk (HR; baseline WBC>10x109 /L) patients and for those
Results: Gimatecan had a markedly higher potency against leukemia with an increasing WBC during ATRA therapy; three anthracyline-
cell lines (median IC50: 1.2 nM) than standard chemotherapeutics based consolidation courses and 2 years standard maintenance with
(median IC50s:182-361 nM), with profound selectivity on BCP-ALL ATRA (14days/3months). From June 2010, ATRA was introduced in
over AML (3.6-fold) and HSPCs (75-fold). Oral administration of single- each consolidation course. Intrathecal prophylaxis was given to HR
agent gimatecan resulted in complete disease remission in xenografts patients. Arsenic trioxide was not available.
of BCP-ALL cell lines bearing favorable (ETV6-RUNX1), intermediate Results: From September 2003 to August 2019, 118 APL children (M/F
(TCF3-PBX1), adverse (KMT2A-AFF1) and fatal (TCF3-HLF) cytoge- 64/54; hypergranular 81; variant 37; HR 68) were diagnosed in Bagh-
netics, and was equally effective in patient-derived xenografts of dad (first cohort 54; second cohort 64). Six (3 HR) died before starting
refractory childhood BCP-ALL failing salvage chemotherapy or upfront therapy (hemorrhages) and 4 refused treatment for parents’ decision.
immunotherapies. Gimatecan exhibited a moderate suppression of Ninety-four/108 (87%) evaluable children achieved a complete remis-
normal hematopoiesis, and did not mediate prominent damages on car- sion (CR); 12 (11%) died during induction (HR 9), due to hemorrhages in
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S188 of S635 ABSTRACTS

8, differentiation-syndrome in 3, infection in 1; 2 abandoned therapy. 50% and 63% respectively. Of the features analysed, only high initial
Thirty-one (33%) relapsed in the bone marrow (first-period 20/31); 2 TLC(>50000/uL) showed association with poor outcome(p<0.05).
are alive in second CR. The 5-year overall survival and event-free sur- Conclusions: Our data suggests that outcome of t(8;21)positive pedi-
vival rates are 61.8% and 64.1% for the entire patients’ series; 51.7% atric AML is inferior than what was traditionally believed. Further
and 43.5% for first cohort and 68.4% and 55.5%, for second. Baseline research into role of other co-existing molecular markers may help in
WBC was a risk factor for induction mortality (HR 75%, low-risk 25%; identifying this ‘not-so-favourable’ subgroup where treatment may be
p=0.011). intensified to achieve long-term cure.
Conclusions: Induction therapy with ATRA and anthracycline con-
firmed its efficacy with a virtual absence of resistant disease. Early
death, mainly due to hemorrhage, remains a major cause of failure. EP086 / #1646 REAL WORLD OUTCOME OF CHILDREN
ATRA extended consolidation improved the overall results. WITH ACUTE MYELOID LEUKEMIA PRESENTING TO A
TERTIARY CARE CENTER IN INDIA

EP085 / #838 PEDIATRIC ACUTE MYELOID LEUKEMIA WITH Chetan Dhamne1 , Shyam Srinivasan2 , Nirmalya Roy Moulik3 ,
T(8;21)- IS IT REALLY FAVOURABLE? Akanksha Chichra3 , Dhanlaxmi Shetty4 , Prashant Tembhare4 ,
Subramanian Pg3 , Nikhil Patkar4 , Gaurav Narula1 , Sripad Banavali3
Sunisha Arora1 , Dhwanee Thakkar2 , Upasana K2 , Anjali Yadav2 , Neha 1 Tata Memorial Centre, Homi Bhabha National Institute, Paediatric Oncol-

Rastogi2 , Satya Yadav2 ogy, Mumbai, India; 2 Tata Memorial Centre, Homi Bhabha National Insti-
1 Medanta- the Medicity, Pediatric Hemat-oncology And Bmt, Gurgaon sec- tute, Pediatric Oncology, Mumbai, India; 3 Tata Memorial Centre, Paediatric
tor, India; 2 Medanta- the Medicity, Pediatric Hemat-oncology And Bmt, Oncology, Mumbai, India; 4 Tata Memorial Center, Homi Bhabha National
Gurgaon sector, India Institute, Hemato-pathology, Navi Mumbai, India

Background and Aims: t(8;21) is a common recurrent cytogenetic Background and Aims: Treatment of pediatric AML remains a chal-
abnormality in pediatric Acute Myeloid Leukemia(AML) and is consid- lenge in the LMICs as well as HICs. We report real-world data of
ered to have favourable prognosis. However, recent reports suggest pediatric AML treated in a tertiary-care center in India.
heterogeneity in survival outcomes in this subgroup. We report clinical Methods: We analysed outcomes of AML patients <15 year of age
features and outcome of our cohort of childhood t(8;21)positive AML. who received atleast 1 week of treatment at our center. Initial
Methods: Data of 13consecutive t(8;21)positive AML treated between workup included cytogenetics, flowcytometry and molecular studies.
January2014- December2021 was retrospectively analysed with pri- Patients at high risk of infections received oral metronomic chemother-
mary aim to analyse OS and EFS. Sub-analysis was done to decipher apy(OMCT) prior to 3+7 induction followed by 3 cycles of high-dose
the difference in outcome amongst subgroups based on age, gender, cytarabine +/- cladribine and 1 year of oral maintenance therapy.
initial TLC, presence of additional cytogenetic abnormality, MRD pos- Bone-marrow responses were assessed by morphology and 10-color
itivity at end of 2nd induction, myeloid sarcoma, aberrant CD19 and flowcytometry at the end of each chemotherapy cycle until negative.
maintenance chemotherapy. A positive BM-MRD was defined as ≥0.01% by flowcytometry.
Results: Demographics: male-9, female-4; mean age-10.3years Results: Two-hundred-fourteen children <15 years of age were anal-
(Range:3-15years). At diagnosis, myeloid sarcoma-3patients, CNS ysed. Ninety-eight received OMCT while 116 received 3+7 induction
involvement-1patient. AML1-ETO fusion transcript was detected by upfront. Total 194 received Induction I of which 152 proceeded
PCR in all patients. Combination of t(8;21) with loss of sex chromo- to consolidation. Total 130 patients completed prescribed intensive
some was the most common cytogenetic abnormality(n=6), 3patients chemotherapy. Median age was 8 years(0.1 – 16.0), Male:Female ratio
had aberrant CD19 expression on flowcytometry. One patient aban- was 2.05:1, median baseline count of 25.02(0.02 – 418)x103 /μL and
doned treatment after 1st induction chemotherapy and was excluded median follow up was 26 months (95% CI: 23.96-28.04). Two year EFS
from analysis. Twelve patients received 2cycles of 3+7(Daunorubicin and OS were 39.5 ± 3.6 % and 42.6 ± 3.6 % respectively. After exclud-
45mg/m2 x3days, Cytarabine200mg/m2 x7days) followed by 4cycles ing 4 patients who had M3 marrow at the end of induction, 55/149
high-dose Cytarabine(12gm/m2), 1patient received MRC AMLXII (36.91%) patients were MRD positive. MRD positive patients had a sig-
protocol. All patients achieved morphological remission post 2induc- nificantly poor 2 year EFS of 31.1 ± 7.5 % vs 66.2±5.4% (p=0.00035)
tion cycles, whereas negative MRD (<0.1% by flowcytometry) and who were MRD neg. Those who cleared MRD at the end of consoli-
molecular remission seen in 6/8 and 8/10 patients respectively. Six dation still had an EFS of 43.5%+11.9% as compared to 17.5± .6% for
patients relapsed with mean interval of 8.8months after diagnosis and those who continued to be MRD positive (p = 0.005).
received Hematopoietic stem cell transplant(HSCT) in CR2. Of the Conclusions: Treatment of AML remains a challenge. MRD at the end
transplanted patients, 2died due to treatment-related complications of induction I was a significant predictor of outcome in pediatric AML
and 3 had 2nd relapse (2-died,1-lost to follow-up). Median follow treated with 3+7 induction. Newer therapies are needed to improve
up duration-22months (Range:4-120months) with EFS and OS of outcomes without increasing infection related mortality.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S189 of S635

EP087 / #772 MESOTHELIN TARGETING CHIMERIC ANTIGEN ical Sciences- New Delhi, Division Of Pediatric Oncology, Department Of
RECEPTOR THERAPY FOR PEDIATRIC ACUTE MYELOID Pediatrics, New Delhi, India
LEUKEMIA
Background and Aims: Acute promyelocytic leukemia (APL) has
Christina Fong1 , Kyohei Misawa2 , William Ray Vista2 , Srijita distinct morphologic, biologic, and clinical features. Diagnosis is pre-
Banerjee2 , Prasad S. Adusumilli2 dominantly based on morphology which is characterized by presence
1 Memorial Sloan Kettering Cancer Center, Department Of Pediatrics, New of abnormal promyelocytes with bilobed nuclei and frequent Auer
York, United States of America; 2 Memorial Sloan Kettering Cancer Center, rods and a cytogenetic/molecular hallmark of t(15;17)(q22;q21) PML-
Department Of Thoracic Surgery, New York, United States of America RARA. APL patients account for 2-20% of pediatric AML. Aim of the
study is to discuss immunophenotic profile of pediatric APL.
Background and Aims: Despite advances in targeted therapies, one in Methods: Children of age <18 years with APL diagnosed in the depart-
four children with AML will relapse. There remains an urgent unmet ment of Laboratory Oncology were retrospectively analyzed. The
need for improved therapies. About 25% of pediatric AML patients diagnosis was made based on morphological, phenotypical, cytogenetic
aberrantly express mesothelin (MSLN). We aimed to assess the effi- and molecular features.
cacy of a second generation MSLN targeting chimeric antigen receptor Results: Out of total 302 paediatric AML patients, 30 (9.9%) were
(CAR) T cell with a 28-zeta costimulatory domain (M28z) against pedi- diagnosed as APL (median age-8.5 years, range 1 year -18years, M:F-
atric AML. We hypothesized that M28z CAR T cells have antitumor 2.3:1). Common presenting symptoms were fever (83.3%) and bleeding
efficacy against MSLN+ AML. (73.3%). Median Hemoglobin was 68 g/L(range:3.9- 10.3g/L), TLC
Methods: AML human cell line, Kasumi-1, was retrovirally transduced was 11. 82x109 /L (range:0.9- 256.41x109 /L), platelets were 27x109 /L
to stably express MSLN and GFP-firefly-luciferase fusion protein. In (range:6-182x109 /L) with peripheral blood and bone marrow promye-
vitro efficacy at varying effector to target (E:T) ratios was evaluated locyte percentage 76% (0-98%) and 90% (70-98%), respectively.
using luciferase assays. 3x106 MSLN+ tumor cells were injected sys- Around 90% had classical hypergranular and 10% had hypogranular
temically via tail vein into NSG mice to produce clinically relevant morphology. As per Sanz risk stratification, 14.3% had low, 25% inter-
mouse models with high disease burden. 5x105 M28z CAR T cells mediate and 60.7% high risk disease. On immunophenotyping, 24(80%)
(E:T 0.2:1) were injected systemically at day 7. Overall Survival was were CD34-HLADR-, 5(16.7%) were CD34+HLADR- and 1(3.3%) was
analyzed via Kaplan-Meier estimate and compared between groups CD34+HLADR+. All patients were positive for cMPO, CD13, CD33
using a log-rank test. Secondary outcomes included tumor burden as and CD9. Other IPT marker as CD38, CD64, CD117, CD123, CD25,
measured noninvasively with bioluminescent imaging (BLI) and flow CD4, CD2, CD56, CD15, CD18,CD11b, CD19 and CD7 were positive
cytometry of bone marrow/spleen. in 96.6%, 89.7%, 83.4%, 55.2%, 50%, 31%, 25.9%, 20.7%, 16.7%, 10.3%,
Results: In vitro assays demonstrated >50% MSLN+ tumor cell lysis 10.3%, 10% and 6.7%, respectively.
with E:T as low as 1:1, without toxicity against MSLN- controls. M28z Conclusions: APL constitute 9.9% of total paediatric AML in our
treated mice (E:T 0.2:1) demonstrated significantly prolonged survival cohort. All the cases were positive for cMPO, CD13, CD33 and
(median 62 days, p <0.0007). Control mice were euthanized at median CD9. A combination of cMPO, CD34, HLADR, CD117, CD33, CD15,
38 days for progressive disease, as measured by BLI and flow cytom- CD11b/CD11c, CD9, CD18 with CD45 can diagnose APL with high
etry after necropsy. Disease burden was eradicated by 7 days after specificity and sensitivity by excluding other hypergranular AML and
treatment in the M28z group. By 62 days, the M28z mice did not show also in cases with CD34 and HLA-DR negative cases of AML that can
signs of acute cytokine release (no weight loss, reduced activity, or pose a diagnostic dilemma on morphology or on flow-cytometry.
malaise).
Conclusions: M28z CAR T cells specifically target MSLN+ cells in vitro
and significantly prolong survival in vivo at low E:T ratios wiithout off- EP089 / #1480 ANALYSIS OF BOSUTINIB FOR CHRONIC
target effects. Second generation MSLN Targeting CAR T cells could be MYELOID LEUKEMIA IN THE MULTICENTER, PROSPECTIVE
an alternative therapy for MSLN+ pediatric AML patients. OBSERVATIONAL STUDY OF PEDIATRIC CHRONIC MYELOID
LEUKEMIA (JPLSG CML-08)

EP088 / #1834 IMMUNOPHENOTYPIC PROFILE OF Dai Keino1,2 , Akihiko Tanizawa2 , Akihiro Watanabe2 , Masaki Ito2 ,
PAEDIATRIC ACUTE PROMYELOCYTIC LEUKEMIA Chikako Tono2 , Haruko Shima2 , Yuki Yuza2 , Hideki Muramatsu2 ,
Hiroyuki Shimada2
Smeeta Gajendra1 , Ritu Gupta1 , Sanjeev Kumar Gupta1 , Garima Jain2 , 1 Kanagawa Children’s Medical Center, Department Of Hematol-
Sameer Bakhshi3 , Rachna Seth4 , Saroj Singh1 , Sandeep Rai1 , Ranjit ogy/oncology, Kanagawa, Japan; 2 Japanese Pediatric Leukemia/ Lymphoma
Sahoo3 , Atul Sharma3 , Lalit Kumar3 Study Group, Cml Committee, Nagoya, Japan
1 AIIMS, New Delhi, Laboratory Oncology, Delhi, India; 2 National Institute
of Pathology, Pathology, New DElhi, India; 3 All India Institue of Medical Background and Aims: Bosutinib is a second-generation tyrosine
Sciences, Medical Oncology, New Delhi, India; 4 All India Institute of Med- kinase inhibitor (TKI) indicated for treatment of chronic myeloid
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S190 of S635 ABSTRACTS

leukemia (CML) in adult patients. The safety and efficacy of bosutinib RT-PCR, and fragment analysis were utilized for targeted molecular
in pediatric patients have not yet been evaluated, except for only one panel.
pediatric patient reported. In this study, we report cases of chronic Results: A total of 70 patients of AML aged ≤18 years were enrolled
phase (CP) CML treated with bosutinib as a sub-analysis of the Japan in this study. The median age in this cohort was 6 years (IQR; 3,6
Pediatric Leukemia/Lymphoma Study Group (JPLSG) CML-08 study. years), and males were predominant. About one-fifth of patients had
Methods: The JPLSG CML-08 study enrolled 78 patients with CP malnutrition. The median duration of symptoms before presentation
CML under 18 years of age diagnosed between October 2009 to the hospital was 25 days (IQR; 10,45 days). Fever was the com-
and September 2014. Among them, 9 patients were treated with monest symptom, followed by bleeding, pallor, proptosis, and fatigue.
bosutinib. The cytogenetic analysis revealed abnormal karyotype/cytogenetics
Results: The median age at diagnosis was 11.1 (4.0-17.0) years. Males (AKC) in 45 (64.3%) patients and normal karyotype/cytogenetics (NKC)
and females were 7 and 2, respectively. the 1st -line TKIs were ima- in 25 (35.7%). The common chromosomal abnormalities were t(8;21)
tinib (n=8) and dasatinib (n=1). The median age at start of bosutinib translocation (31.42%), monosomy/deletion 7 (7%), complex karyotype
was 15.1 (7.7-21.5) years. The patients were treated with bosutinib (4%) and monosomal karyotype (2%). AML-ETO fusion (37%) and
as the 3rd -line (n=6), the 4th -line (n=2), and the 5th -line (n=1). The CBFB-MYC11 gene fusion (2%) were detected by RT-PCR and FLT3-
reasons of changing the TKI were resistance (n=6) and intolerance TKD (1.5%) by PCR. Fragment analysis revealed NPM1(7%) mutation
(n=3). The median starter dose was 79 (50-410) mg/m2 and the median and FLT-ITD (8%). The remission was achieved in 90.5% of enrolled
maximum dose was 320 (250-400) mg/m2 . The median duration of patients post-induction-1. The median follow-up period of our patients
bosutinib administration was 27 (5-40) months, with 5 patients contin- was 221 days (IQR 28;415 days). The median event-free survival (EFS)
uing bosutinib and 4 switching to other TKIs (3 resistant, 1 intolerant). in all patients was 11 months (95% CI, 5-12.5). The three-year overall
BCR-ABL1 mutation analysis was performed in 6 patients before bosu- survival probability (pOS) was 57% in all patients.
tinib administration, and 35bp insertion could be observed in exon 8/9 Conclusions: The delayed seeking to a health care facility and malnutri-
of 3 patients. Grade 3 toxicities, such as hypertension, elevated liver tion are still prevalent in developing countries yet the overall survival
enzyme, creatine kinase and phosphorus levels, as well as grade 1/2 has increased considerably. The majority of patients had AKC. The
toxicities, such as diarrhea, rash, musculoskeletal pain, abdominal pain, paucity of survival data in pediatric AML from developing countries
headache, fever, elevated bilirubin, and hematotoxicity (anemia and lays out the importance of the conducted study.
neutropenia), were reported.
Conclusions: Adverse event profile was similar to the adult one. Bosu-
tinib demonstrated manageable toxicity in the treatment of CP CML EP091 / #1548 CHRONIC MYELOID LEUKEMIA IN LMICS-
patients. CHANGING PARADIGM OF CARE-LOOKING BEYOND
TYROSINE KINASE INHIBITORS

EP090 / #666 CLINICAL PROFILE, CYTO-MOLECULAR Piali Mandal1,2 , Rachna Seth3 , Jagdish Prasad Meena3 , Aditya Gupta3 ,
ANALYSIS AND OUTCOME OF PEDIATRIC ACUTE MYELOID Ritu Gupta4
LEUKEMIA: A REPORT FROM TERTIARY CARE HOSPITAL IN 1 Kalawati Saran Children’s Hospital, Department Of Pediatrics, New Delhi,

RESOURCE-CONSTRAINED SETTING India; 2 All India Institute of Medical Sciences, Division Of Pediatrics, Depart-
ment Of Pediatrics, New Delhi, India; 3 All India Institute of Medical Sciences,
Harshita Makkar1 , Jagdish Prasad Meena1 , Nivedita Pathak1 , Aditya Division Of Pediatric Oncology, Department Of Pediatrics, New Delhi, India;
Gupta1 , Sameer Bakhshi2 , Ritu Gupta3 , Anita Chopra3 , Deepshi 4 All India Institute of Medical Sciences, Laboratory Oncology Unit, B.r.a.irch,

Thakral3 , Rachna Seth1 New Delhi, India


1 All India Institute of Medical Sciences, Division Of Pediatric Oncology,
Department Of Pediatrics, New Delhi, India; 2 All India Institue of Med- Background and Aims: There has been a paradigm shift in the man-
ical Sciences, Department Of Medical Oncology, New Delhi, India; 3 All agement of chronic myeloid leukemia (CML) after the introduction of
India Institute of Medical Sciences, Department Of Laboratory Oncology, tyrosine kinase inhibitors (TKIs). In LMICs, it remains a less focused
B.r.a.irch, New Delhi, India area. This study was aimed to investigate the clinical presentation,
evolution in the management with the availability of generic forms of
Background and Aims: Despite the advancements in diagnostic tech- second-generation TKIs, and reasons for poor compliance.
niques and discovery of newer chemotherapeutic agents, the outcome Methods: A retrospective study was performed, and all the patients
of acute myeloid leukemia (AML) remains dismal. The objectives of this with CML from January 2015 through January 2022 were reviewed.
study were to investigate clinical profile, cyto-molecular analysis, and Results: Twenty-seven patients diagnosed with CML were included in
survival outcome of pediatric AML. the study. The mean age was 8.8 years (range 2.5-14 years). The most
Methods: This prospective study was carried out from October 2018 common symptoms were abdominal distension (100%), pallor (100%),
to December 2020 in a tertiary care hospital. Karyotype and cytoge- and fever (89%). All the patients were positive for BCR-ABL1 (p210)
netics analysis was done to identify chromosomal aberrations and PCR, fusion transcript, detected by RT-PCR. The most common presentation
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S191 of S635

was CML-chronic chase (CP) 22 (81%), and 5 (19%) de novo blast crisis increased total homozygosity. A long contiguous stretch of homozygos-
(BC) [de novo lymphoid BC (n=3), extramedullary lymphoid BC (n=1), ity was detected on ch 9, ch11, and ch19. The gains were observed on
and myeloid BC (n=1)]. There was progression in two patients with ch 8, 9, 14, 19, 21, and 22, and the losses were seen on ch 7 and 10. The
CML-CP, one to each lymphoid and myeloid BC. Imatinib was the 1st gains were more common than losses (8 vs 2). Monosomy was observed
line in all cases earlier and recently shifted to 2nd generation TKI as 1st in three patients. Post-induction-1, 86% patients achieved remission.
line for BC. Two patients with the suboptimal response received 2nd Conclusions: NKC-AML patients have genetic abnormalities that
generation TKIs. The treatment for all the patients with BC included can be detected by more advanced techniques like NGS and CMA.
chemotherapy and TKI. The transplant could be performed on only These genetic abnormalities play a role in risk stratification that may
one patient with progression. Five patients with BC and one with CP remain hidden in otherwise NKC-AML and eventually confer a poor
expired. Non-compliance to follow-up as well as to medication was prognosis.
a major issue. The reasons for non-compliance include unawareness,
distance, social issues, drug availability, and financial constraints. This
issue has been tried to be resolved by teleconsultation recently. EP093 / #968 EFFICACY OF POST-TRANSPLANT
Conclusions: Compliance with follow-up and drugs is an PROPHYLACTIC THERAPY IN CHILDREN WITH HR AML
important issue in LMICs. Repeated counseling, increasing DEPENDING ON MRD STATUS BEFORE ALLO-HSCT
awareness among the medical community and the patients, and
telemedicine remain the mainstay of improving follow-up and Zhemal Rakhmanova, Olesya Paina, Svetlana Razumova, Polina
compliance. Kozhokar, Anastasiya Frolova, Luibov Tsvetkova, Elena Babenko,
Tatyana Gindina, Alexander Alyanskiy, Ildar Barkhatov, Elena
Semenova, Ludmila Zubarovskaya
EP092 / #672 MOLECULAR EVALUATION OF GENE Pavlov University, R. M. Gorrbacheva Research Institute, Saint Petersburg,
MUTATION PROFILE AND COPY NUMBER VARIATIONS IN Russian Federation
PEDIATRIC ACUTE MYELOID LEUKEMIA
Background and Aims: Relapse of AML remains one of the main
Jagdish Prasad Meena1 , Nivedita Pathak1 , Aditya Gupta1 , Sameer causes of mortality after allo-HSCT. The role of prophylactic therapy
Bakhshi2 , Ritu Gupta3 , Rachna Seth1 after allo-HSCT has not been determined, especially with depen-
1 All India Institute of Medical Sciences, Division Of Pediatric Oncology, dence on MRD(±) status before allo-HSCT in children with AML.
Department Of Pediatrics, New Delhi, India; 2 All India Institue of Medical One of the options for relapse prophylaxis in this case is usage of
Sciences, Department Of Medical Oncology, New Delhi, India; 3 All India DLI±hypomethylating agents. The aim: studying the effect of anti-
Institute of Medical Sciences, Laboratory Oncology Unit, B.r.a.irch, New leukemia prophylaxis on overall and relapse-free survival (OS and
Delhi, India RFS) of children with AML, depending on the MRD(±) status before
allo-HSCT.
Background and Aims: Cytogenetically normal acute myeloid Methods: The retrospective analysis included 57 patients (pts.), age
leukemia (AML) is currently categorized as intermediate-risk, yet this 0.5-18 years, who were in MRD(+) or MRD(-) AML remission at the
group is quite heterogeneous. The objectives of this study were to time of allo-HSCT. Patients with primary graft failure, relapse within 2
investigate the mutation profile of targeted genes and copy number months after alloHSCT, GVHD 2-4 grade who didn’t allow performing
variations (CNVs) in normal karyotype and normal cytogenetics (NKC) prophylaxis of disease recurrence were excluded from analysis. Post-
AML and correlate it with treatment response. transplant prophylaxis of AML received 16 pts.: 1. 5-azacytidine 20-70
Methods: This prospective study was conducted from October 2018 mg/m2 from 3 to 5 courses in 7 (44%) pts, 2. DLI in 6 (37%) pts (total
to December 2020. The next-generation sequencing (NGS) (30 gene СD3+/kg dose was 1,5х106 - 2x108 ), 3. DLI + hypomethylating agents
panel) and chromosomal microarray analysis (CMA) using Affymetrix in 3 (19%) pts. Patient’s characteristics, including remission status,
Cytoscan 750 K GeneChip were performed in NKC-AML. intensity of conditioning regimen, donor type, stem cell source, GVHD
Results: A total of 94 patients aged ≤18 years were screened. After prophylaxis in groups with/without post-transplant AML prophylaxis
excluding 24 patients, 70 patients with AML underwent conventional were comparable.
karyotyping/cytogenetic analysis. Forty-five (64.3%) had abnormal Results: Median follow up is 68 months. Post-transplant prophylaxis
karyotype/cytogenetics (AKC) and 25 (35.7%) had NKC. Twenty-three of relapse in pts with MRD(+)status before allo-HSCT increased RFS
out of 25 NKC-AML were further processed for gene mutation pro- - 100% vs 36.4% without post-transplant disease prophylaxis (p =
file and CNVs using NGS and CMA, respectively. Twenty-two out of 0.008), but did not affect on OS - 87.5% vs 92%, respectively (p =
23 (95.7%) patients were detected to have mutations in various genes. 0.268). In high risk AML pts with MRD(-) post-transplant prophylaxis
The common mutations were: NRAS (5), NPM1 (4), CEBPA (4), KRAS of relapse had no effect on OS - 88.7% vs 100% (p = 0.083) and RFS -
(3), KIT (3), RUNX1 (2), NOTCH1 (2), WT1 (2), GATA1 (2), FLT3 (2), 87.5% vs 92 % (p = 0.671), respectively.
GATA2 (2), KMT2D (2), FLT3-TKD (2), PHF6 (2). Copy number varia- Conclusions: Post-transplant prophylaxis of relapse is advisable in
tions (CNVs) were detected in nine patients (39%), and the 4 (17%) had children with AML with MRD(+) before allo-HSCT.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S192 of S635 ABSTRACTS

EP094 / #1528 RESULTS OF ALLOGENEIC HEMATOPOIETIC EP095 / #1533 NEW ANTIGENS FOR A CAR-T THERAPY IN
STEM CELL TRANSPLANTATION WITH REDUCED-INTENSITY JUVENILE MYELOMONOCITIC LEUKEMIA
CONDITIONING REGIMEN IN PEDIATRIC CHRONIC MYELOID
LEUKEMIA PATIENTS FAILING FIRST- AND Nerea Matamala1,2 , Beatriz Ruz-Caracuel1,2 , Jordi Minguillón1,2 ,
SECOND-GENERATION TYROSINE KINASE INHIBITORS Carlos Rodríguez1 , Adela Escudero1,2 , Alfonso Navarro Zapata2 ,
THERAPY Cristina Ferreras2 , Antonio Pérez Martínez2,3
1 Hospital La Paz Institute for Health Research, Instituto De Genética
Polina Sheveleva1 , Olesya Paina2 , Anna Osipova1 , Tatyana Gindina2 , Médica Y Molecular (ingemm), Madrid, Spain; 2 Hospital La Paz Institute for
Ekaterina Izmailova1 , Ildar Barkhatov2 , Elena Semenova2 , Elena Health Research, Translational Research In Pediatric Cancer, Madrid, Spain;
Morozova3 , Ludmila Zubarovskaya2 3 University Hospital La Paz, Pediatric Hemato-oncology, Madrid, Spain

1 RM Gorbacheva Research Institute Pavlov University, Hemopoietic


Stem Cell Transplantation, Saint-Petersburg, Russian Federation; Background and Aims: Juvenile myelomonocytic leukemia (JMML) is
2 Pavlov University, R. M. Gorrbacheva Research Institute, Saint a rare leukemia with high morbidity and mortality, whose only curative
Petersburg, Russian Federation; 3 Pavlov First St. Petersburg State option at present is hematopoietic stem cell transplantation. CAR-
Medical University, Rm Gorbacheva Research Institute, Pavlov Univer- T Therapy against CD19 antigen has obtained impressive results in
sity, St. Petersburg, Russian Federation, Санкт-Петербург, Russian patients with acute lymphoblastic leukemia. Given that CAR-T therapy
Federation has significantly lower development costs and time than other conven-
tional therapies, translational research with this advance therapy in
Background and Aims: While tyrosine kinase inhibitors (TKIs) rare diseases such as JMML presents a great opportunity. In this work
are first-line therapy in children with chronic myeloid leukemia we identified potential cell surface antigens differentially associated
(pCML), allogeneic hemopoietic stem cell transplantation (allo- with tumors in JMML, good candidates for a CAR-T therapy.
HSCT) remains the only curative option in spite of associated Methods: Using Gene Expression Omnibus database we selected 2
toxicity/mortality. This study evaluates efficacy and toxicity of allo- published RNAseq data from hematopoietic stem cells in a cohort of
HSCT with reduced-intensity conditioning (RIC) in patients with 11 JMML patients and 9 controls (GSE111895 and GSE183252 series).
pCML. Next, we performed a differential expression analysis with edgeR using
Methods: Among 50 pediatric patients with a median age of 12(1- an FDR (False Discovery Rate) < 0.05 as a threshold. Different bioinfor-
18) years 15(30 %) did not respond to first- and second-generation matic tools, such as The in silico Human Surfaceome and The Human
TKIs and were included into allo-HSCT cohort. At the moment of Protein Atlas, were used to select those cell surface proteins that are
allo-HSCT patients achieved complete hematologic (CHR; 13/15, 86%) not highly expressed in healthy tissues.
and cytogenetic response (CCyR; 7/15, 46%). None of the patients Results: We identified around 1,000 differentially expressed genes in
had complete molecular response (CMR), 2 were non-responders. HSC from patients and controls. Of these, 700 overexpressed proteins
In 4 cases BCR-ABL kinase domain mutations were found; T315I on the cell surface were identified. In order to identify specific antigens,
mutation in 3 cases, M244V and M351 mutations in 1 patient we analyzed the expression of the 30 best candidates in healthy tissues
with lymphoid blast crisis. In 1 case DDX41 mutation was found. using in silico tools, selected the genes with low or undetected mean
Most (14) patients received RIC prior to grafting with bone mar- expression in 44 healthy human tissues, and identified the best ones for
row (n=7) or peripheral blood stem cells (n=8) from matched related a potential CAR-T therapy in JMML, such as VNN1, GPR174 or MILR1.
(n=4 ), matched (n=7) or mismatched unrelated (n=3), and hap- Conclusions: The proteins identified in this study are potential candi-
loidentical (n=1) donor. Calcineurin inhibitors-based GVHD prophy- dates for a CAR T therapy in JMML. This therapy would be of great
laxis in 6 and post-transplant cyclophosphamide-based one in 9 benefit for patients with this type of severe leukemia, with limited
patients. treatment options and very poor outcome.
Results: Thirteen (86%) patients engrafted on a median of
D+19(D+11-D+23). In 2 cases a second allo-HSCT was per-
formed after primary graft failure, then in 1 a third one due to EP096 / #2006 PEDIATRIC ACUTE MYELOID LEUKEMIA –
second graft failure (SGF). On D +100, 13 pts (86 %) achieved a OUTCOME FROM A GOVERNMENT FACILITY IN NORTH INDIA
CHR and CCyR, with 9 (60 %) patients also reaching CMR. Four
patients received donor lymphocyte infusions due to molecu- Nita Radhakrishnan1 , Manideepa Maji2 , Shruti Saxena1 , Savitri Singh1 ,
lar relapse (n=3) or SGF(n=1). With a median follow-up of 90 Tushar Avasti1 , Somiya Singh1
months the overall survival was 80%. Three patients died due to 1 Post Graduate Institute of Child Health, Department Of Pediatric Hematol-

acute (n=1) or chronic (n=1) graft-versus-host disease, infection ogy Oncology, Noida, India; 2 PGICH, Pediatric Hematology And Oncology,
(n=1). NOIDA, India
Conclusions: Allo-HSCT with RIC is an effective and relatively safe
therapeutic modality in patients with pCML failing to achieve response Background and Aims: Childhood AML is a heterogeneous dis-
to first- and second-generation TKIs. ease with survival of >70 % in developed countries. In developing
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S193 of S635

countries, it varies from 25-55%. We evaluated the profile and out- received intensive chemotherapy from January 2014 to Decem-
come of childhood AML at our centre. ber 2019 were retrospectively analyzed. All patients received stan-
Methods: Prospectively collected data from April-2017 to March- dard 3+7(Daunorubicin+Cytarabine) induction followed by 3-4 cycles
2022 is reported. Children aged 0–18 years diagnosed with AML & of cytarabine-based consolidation chemotherapy. Azole-based anti-
MPAL were included. Children presenting at relapse were included. fungal prophylaxis was administered to all patients.
Treatment consisted of 2 cycles of cytarabine with anthracycline Results: A total of 277 patients were treated with 997 intensive
followed by high dose cytarabine based intensification. APML was chemotherapy cycles. Febrile neutropenia was observed during 65%
managed as per PETHEMA 2005 protocol. Relapsed AML was man- of these cycles. Focus of infection included, gastrointestinal (n=121,
aged with 2 cycles of FLAG followed by bone marrow transplantation. 18.6%), skin and soft tissue (n=103, 15.8%), lung (n=95, 14.6%),
Venetoclax-Azacytidine was used in those either refractory to FLAG or paranasal sinuses (n=14, 2.1%) and none (n=272, 42%). Culture posi-
not fit. tive sepsis was seen in 140 (21.5%) patients, including gram-negative
Results: 47 patients with AML (AML: 36, APML: 8, MPAL: 3) were bacilli (n=108, 77%), gram-positive cocci (n=30, 21.4%) and fungi
included. 3 children with relapsed AML were referred here for further (n=2, 1.4%). An MDRO was in 44% of culture-positive sepsis, which
treatment. Median age of 8.5 years (0.4-18 years), M:F 1.6:1. Of 44 den- was most commonly Klebsiella pneumoniae (56%). The incidence of
ovo cases, 9 children had high-risk, 18 had intermediate and rest had proven/probable fungal infections was 7.5%. Overall, the incidence of
favourable -isk genetic profile. FLT3-ITD was noted in 8. 1 had RAM sepsis-related mortality was 12.6% with the majority occurring during
phenotype. All achieved morphological remission at end of induction. 4 induction (7.2%). MDRO sepsis contributed to 75% of all sepsis-related
children died during induction and 3 died during intensification follow- deaths in induction.
ing sepsis. 2 children of other causes (GATA2 def, cardiac arrhythmias). Conclusions: Gram-negative sepsis, in particular, MDRO accounted for
7 children discontinued treatment and 7 relapsed. Out of 3 relapsed the majority of toxic deaths among paediatric AML at our centre. Hos-
patients, 2 continued to be refractory post relapse chemotherapy and pital antibiotic stewardship programs along with the incorporation of
one child with APML in CR3 with no sibling donor underwent autol- novel treatment strategies including antibiotic cycling and granulocyte
ogous BMT 2 years ago and remains in CR. 26 children are alive and transfusions will help in curtailing MDRO sepsis.
in complete remission (follow up 1 month-7 years post completion of
treatment) 55% EFS was noted in the entire cohort. In APML, 7 are in
CR; 1 LTFU. Response to Ven-Aza (n=3) was disappointing in relapsed EP098 / #1519 PROGNOSTIC SIGNIFICANCE OF
settings. METHYLATION PROFILE AND RNA EXPRESSION LEVEL OF
Conclusions: The survival data of AML is sparse from LMIC. With WILMS TUMOR 1 GENE IN PRIMARY CASES OF ACUTE
standard protocols, more than 50% AML children survive the disease. MYELOID LEUKEMIA

Pranay Tanwar1 , Harsh Goel1 , Anita Chopra1 , Amar Ranjan1 , Jagdish


EP097 / #827 PATTERNS AND PREVALENCE OF INFECTION Prasad Meena2 , Aditya Gupta2 , Ganesh Viswanathan3 , Sameer
AND RELATED MORTALITY IN PEDIATRIC ACUTE MYELOID Bakhshi4 , Maroof Khan5
LEUKEMIA – A SINGLE CENTER EXPERIENCE FROM INDIA 1 All India Institute of Medical Sciences, Laboratory Oncology Unit, Dr.b.r.a.
Institute Rotary Cancer Hospital, New Delhi, India; 2 All India Institute of
Tanveer Ahmed Shaikh1 , Shyam Srinivasan1 , Chetan Dhamne2 , Medical Sciences, Pediatric Oncology, New Delhi, India; 3 All India Institute
Akanksha Chichra2 , Nirmalya Roy Moulik2 , Gaurav Narula3 , Gaurav of Medical Sciences, Hematology, New Delhi, India; 4 All India Institute of
Salunke1 , Sanjay Biswas1 , Sripad Banavali3 Medical Sciences, Medical Oncology, New Delhi, India; 5 All India Institute
1 TATA MEMORIAL HOSPITAL, HOMI BHABHA NATIONAL INSTITUTE, of Medical Sciences, Biostatistics, New Delhi, India
Paediatric Oncology, MUMBAI, India; 2 Tata Memorial Hospital, Homi
Bhabha National Institute, Paediatric Oncology, Mumbai, India; 3 Tata Background and Aims: Acute myeloid leukemia (AML) is a genetically
Memorial Centre, Homi Bhabha National Institute, Paediatric Oncology, heterogeneous neoplastic disorder characterized by clonal expansion
Mumbai, India of myeloid precursor cells. Wilms tumor-1 (WT1) gene encodes a
zinc-finger transcription factor involved in controlling cellular growth,
Background and Aims: Infection-related deaths remain a major cause differentiation, and proliferation of AML. There has been conflicting
of concern in the management of paediatric acute myeloid leukemia reports about association of WT1 gene expression with prognosis of
(AML). In India, unlike in the west, multi-drug resistant gram-negative AML. However epigenetic impact of WT1 is largely unknown.
organisms (MDRO) contribute significantly to treatment-related mor- Methods: This study aimed to investigate the RNA expression lev-
bidity and mortality. Hereby, we report the patterns of infections and els of the WT1 gene in BM and PB samples obtained from 69 AML
related mortality among paediatric AML patients receiving intensive patients (69 at diagnosis, 51 in hematological remission (day-28), and
chemotherapy at a tertiary care center in India. 9 relapse) using quantitative real-time PCR. The methylation status of
Methods: Data collected from Electronic Medical Records of de- the WT1 promoter was also analyzed using methylation-specific poly-
novo paediatric AML patients younger than 15 years who have merase chain reaction (MSP) in all 69 cases at all time points. Finally, we
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S194 of S635 ABSTRACTS

correlated the methylation profile and gene expression level of WT1 with ATRA/ATO at induction and in one patient throughout treat-
gene to study the course of disease ment requiring prolonged and repetitive hospitalizations. All other
Results: WT1 gene expression at the time of diagnosis, and relapse patients continued treatment in outpatient basis. Initial hospitalization
group were significantly higher when compared with the value at the period for patients treated with chemo/ATRA was 40 days. Comparing
time of remission or healthy control samples (p= <0.001). Moreover, transfusion requirements, total PLT and PRBC transfusions were sta-
higher levels of WT1 mRNA expression were found to be inversely cor- tistically significant less in ATRA/ATO group (p=0.016 and p=0.009,
related with normal hematopoiesis and positively associated with age, respectively). On day 28, all patients achieved molecular remission and
high marrow blast counts, M4 subtype, cytogenetic unfavorable risk remain in remission up to last observation time point.
groups, resistance to therapy, an inferior outcome, and a greater inci- Conclusions: Our experience concurs with reported studies mainly in
dence of disease relapse compared to patients with low WT-1 mRNA adults, showing high efficacy rates and acceptable toxicity profiles with
expression. Robust hypermethylation of WT1 promoter was observed ATRA/ATO treatment for pediatric patients with APL.
in 72% of cases of AML. Patients having hypermethylated profile of
WT-1 gene had inferior relapse-free survival compared to normal WT1
patients. Significant positive correlations (p<0.001) between the WT1 EP100 / #1230 EFFECT OF ANTIBIOTIC PROPHYLAXIS ON
expression and methylation levels with bone marrow blast counts at VIRIDANS GROUP STREPTOCOCCI BLOODSTREAM
both initial diagnosis and after induction therapy was observed. INFECTIONS IN DUTCH PEDIATRIC PATIENTS WITH ACUTE
Conclusions: Together, these findings support that WT1 gene MYELOID LEUKEMIA: A TWO-CENTER RETROSPECTIVE STUDY
overexpression–hypermethylation signature is a distinctive feature
that positively associates with the leukemic burden in AML. Romy Van Weelderen1,2 , Kim Klein1,2,3 , Bianca Goemans1 , Wim
Tissing4 , Tom Wolfs1,5 , Gertjan Kaspers1,2
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology,
EP099 / #1667 EXCELLENT REMISSION RATES AND Utrecht, Netherlands; 2 Emma Children’s Hospital, Amsterdam UMC, Vrije
SIGNIFICANT LESS TOXICITY WITH CHEMOTHERAPY-FREE Universiteit Amsterdam, Pediatric Oncology, Amsterdam, Netherlands;
APPROACH FOR ACUTE PROMYELOCYTIC LEUKEMIA (APL) 3 Wilhelmina Children’s Hospital/University Medical Center Utrecht, Pae-
diatrics, Utrecht, Netherlands; 4 Princess Maxima Center for Pediatric
Tatiana Soultana Tziola, Georgia Avgerinou, Elena Solomou, Maria Oncology, Supportive Care, Utrecht, Netherlands; 5 Wilhelmina Children’s
Filippidou, Ilona Binenbaum, Katerina Katsibardi, Stavros Glentis, Hospital, Department Of Infectious Diseases, Utrecht, Netherlands
Kleoniki Roka, Efthymia Rigatou, Antonia Vlachou, Antonis Kattamis
"Aghia Sophia" Children’s Hospital, Division Of Pediatric Hematology Oncol- Background and Aims: Viridans group streptococci (VGS) remain a
ogy, First Dpt Of Pediatrics, National & Kapodistrian University Of Athens, prevalent cause of bloodstream infections (BSIs) during pediatric acute
Athens, Greece myeloid leukemia (AML) treatment. The associated high morbidity and
mortality highlights the need to identify interventions to decrease VGS
Background and Aims: Acute Promyelocytic Leukemia (APL) in chil- infections. We evaluated the effect of different prophylactic antibiotic
dren is rare (5-7% of AML). Standard of care is a combination of regimens on the occurrence of (VGS) BSIs.
chemotherapy with All-Trans Retinoic Acid (ATRA). There are lim- Methods: Medical records of 83 children (0-18 years) diagnosed with
ited data regarding chemotherapy-free approach using ATRA and de novo AML at the Amsterdam UMC, location VUmc (n=42; January
Arsenic Trioxide (ATO) in children. This retrospective study shows our 1998-June 2018) and Princess Máxima Center (n=41; June 2018-
experience with chemotherapy-free approach in children with APL. March 2021) were studied. Within the study period, four consecutive
Methods: The study included children with APL treated at our depart- treatment protocols were used. Pneumocystis jiroveci pneumonia
ment from 2012 until 2021. Efficacy was estimated by remission rates, prophylaxis included co-trimoxazole. Gram-negative prophylaxis with
while toxicity by hospitalization days and use of blood products. fluoroquinolones was introduced in 2006. Gram-positive prophylaxis
Results: Ten patients were included, treated according to AML-BFM comprised either no prophylaxis, penicillin, teicoplanin, or macrolides.
protocols. Mean age at diagnosis was 12.9 (range: 9-15) years and BSI was defined as fever with a positive blood culture. Pearson’s χ2 test
mean time of follow-up 3.5 (range: 0.5-9.5) years. Diagnosis was con- was used to test differences in proportions. Two-sided p-values ≤0.05
firmed by cytogenetics and molecularly. Seven patients were stratified were considered statistically significant.
as standard and three as high risk. Hemorrhagic diathesis was present Results: Included patients underwent 323 chemotherapy courses, of
at diagnosis in all patients (mean platelet count: 36,670/μL, mean pro- which 21 (6.5%) were excluded because antibiotics were given ther-
thrombin time (PT) of 16.63 sec). Patients treated with ATO/ATRA apeutically during the whole aplastic period (n=19), death during the
attained PT normalization within two weeks (range: 5-14 days), signif- chemotherapy course (n=1), or a treatment-related protocol violation
icantly sooner compared to patients with chemo/ATRA (range: 25-40 (n=1). Of the 302 evaluable chemotherapy courses, 94 (31.1%) were
days). All patients developed fever at least once during induction. given without prophylaxis, 137 (45.4%) with penicillin, 48 (15.9%) with
None developed differentiation syndrome. Severe headaches and nau- teicoplanin, 10 (3.3%) with macrolides, and 13 (4.3%) with two dif-
sea, indicating pseudotumor cerebri, occurred in all patients treated ferent regimens, which were added to the above. The corresponding
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S195 of S635

occurrence of BSIs and VGS BSIs, respectively, with these four pro- EP102 / #597 TREATMENT OF BLASTIC PLASMACYTOID
phylactic regimens were 68/102 (66.7%) and 25/102 (24.5%), 51/149 DENDRITIC CELL NEOPLASM IN PEDIATRIC PATIENTS WITH
(34.2%) and 21/128 (14.1%), 3/51 (5.9%) and 0/51 (0%), and 7/13 TAGRAXOFUSP, A CD123-TARGETED THERAPY
(53.8%) and 2/10 (20%) (p<0.0001 and p=0.001).
Conclusions: The administration of teicoplanin antibiotic prophylaxis Luciana Vinti1 , Naveen Pemmaraju2 , Anna Pawlowska3 , Branko
was significantly associated with a decrease in (VGS-associated) BSIs Cuglievan2 , Joseph Lasky4 , Albert Kheradpour5 , Nobuko Hijiya6 ,
and seems a suitable and effective intervention in pediatric AML. Anthony Stein7 , Soheil Meshinchi8 , Craig Mullen9 , Tariq Mughal10 ,
A randomized trial in the NOPHO-DB-SHIP consortium is ongoing Emanuele Angelucci11
to address this important issue, including possible disadvantages of 1 IRCCS Ospedale Pediatrico Bambino Gesu, Pediatric Hematology And
teicoplanin prophylaxis. Oncology, Rome, Italy; 2 University of Texas MD Anderson Cancer Cen-
ter, Department Of Leukemia, Houston, United States of America; 3 City of
Hope National Medical Center, Pediatrics, Duarte, United States of Amer-
EP101 / #1525 INTRODUCING DIAGNOSTIC TESTING FOR ica; 4 Cure 4 The Kids Foundation, Pediatric Hematology/oncology, Las
CHRONIC MYELOID LEUKEMIA IN A PUBLIC HOSPITAL Vegas, United States of America; 5 Loma Linda University Children’s Hospi-
SETTING IN WESTERN KENYA tal, Pediatric Hematology/oncology, Loma Linda, United States of America;
6 Columbia University Irving Medical Center, Pediatric Hematology, Oncol-
Gail Vance, Millicent Orido, Teresa Lotodo, Nicholas Kigen, Ryan ogy, And Stem Cell Transplantation, New York, United States of America;
Stohler, Terry Vik 7 City of Hope National Medical Center, Hematology And Hematopoietic
Indiana University, Medical And Molecular Genetics, Indianapolis, United Cell Transplantation, Duarte, United States of America; 8 Fred Hutchinson
States of America Cancer Research Center, Clinical Research Division, Seattle, United States
of America; 9 Golisano Children’s Hospital, University of Rochester, Pedi-
Background and Aims: Chronic myeloid leukemia (CML) is a myelo- atrics, Rochester, United States of America; 10 Stemline Therapeutics Inc,
proliferative disorder characterized by proliferation of the granulocytic Research And Clinical Drug Development, New York, United States of Amer-
cell line. The incidence of CML in Africa is estimated at 2,000 cases ica; 11 IRCCS Ospedale Policlinico San Martino, Hematology And Cellular
per year with children 0-19 years representing approximately 10% of Therapy Unit, Genova, Italy
the total cases. The disorder is associated with a poor prognosis with-
out treatment. Tyrosine kinase inhibitors are approved for treatment in Background and Aims: Blastic plasmacytoid dendritic cell neoplasm
adults and children with confirmed disease. Diagnostic testing for CML (BPDCN), a rare and aggressive hematologic malignancy, derives from
in the public setting in Kenya is limited and not covered by the National plasmacytoid dendritic cells that overexpress CD123. Pediatric cases
Health Insurance Foundation (NHIF). We report the establishment of of BPDCN are uncommon, limiting available robust safety and effi-
diagnostic testing for CML in the AMPATH Reference Laboratory (ARL) cacy data. A first-in-class, CD123-directed therapy, tagraxofusp (TAG,
and Moi University Teaching and Referral Hospital (MRTH) in Eldoret, SL-401) is the only treatment approved by the FDA for patients aged
Kenya. ≥2 years with newly diagnosed (1L) and relapsed or refractory (R/R)
Methods: CML is characterized by the t(9;22)(q34;q11.2) resulting in a BPDCN. TAG is also approved by the EMA for 1L treatment of adult
reciprocal exchange of ABL1 and BCR creating a chimeric fusion gene patients with BPDCN. Herein we present safety and efficacy data of
and protein with accelerated tyrosine kinase activity. Fluorescence TAG therapy in pediatric patients with BPDCN.
in situ hybridization (FISH) methodology with the BCR/ABL1 probe Methods: Pediatric case reports of BPDCN were collected across
set was applied to both peripheral blood and bone marrow smears. the US and Europe. Data were summarized descriptively; analyses
Patients with an elevated white count and clonal granulocytic prolif- included tumor response, survival, and safety.
eration were identified. Two slides of each specimen type were split Results: Eight pediatric patients with BPDCN were treated with TAG.
for concordance studies between the ARL and the Indiana University Median age was 15.5 years (range 2–21 years). All patients received 12
School of Medicine FISH laboratory. Both laboratories followed the mcg/kg TAG throughout all treatment cycles [1–4 cycles], including a
same protocol utilizing probes from the same vendor. 2-year-old patient who also received 7 mcg/kg at second relapse. Five
Results: Fifteen of 16 samples (94%) including both suspected cases of eight patients experienced adverse events (AE): two had decreased
and controls were concordant with WBC, hematological review and albumin, two had increased transaminases, and one experienced capil-
FISH results. There was one discordant peripheral blood specimen lary leak syndrome. All AEs were manageable and resolved. Three 1L
that was negative at ARL and positive in the Indianapolis FISH lab. patients achieved a complete response, one 1L patient showed partial
Additional samples are under review at both laboratories. response and one R/R patient had a minor response. One 1L and 1 R/R
Conclusions: Concordance studies reported here support the fea- patient (each) had stable disease and one R/R patient had disease pro-
sibility of diagnostic testing for CML in Western Kenya. Validated gression. Five patients were bridged to stem cell transplant following
diagnostic testing is the first step to a rapid confirmation of CML and TAG treatment.
NHIF support for testing and tyrosine kinase inhibitor therapeutic Conclusions: Here we expand the knowledge base of BPDCN treat-
coverage. ment in pediatric patients. TAG treatment was tolerated in all patients,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S196 of S635 ABSTRACTS

showing a manageable safety profile and promising efficacy that gest that pharmacogenetic profiles could be incorporated in the initial
bridged 63% of our cohort to stem cell transplant. risk evaluation of Colombian pediatric patients with AML.

EP103 / #899 PHARMACOGENETICS OF ABCB1, CDA, DCK, EP104 / #1016 GENOMIC ALTERATIONS AND OUTCOMES
GSTT1 AND GSTM1 AND OUTCOMES IN A COHORT OF IN A PEDIATRIC ACUTE MYELOID LEUKEMIA COHORT FROM
PEDIATRIC ACUTE MYELOID LEUKEMIA PATIENTS FROM COLOMBIA
COLOMBIA
Luz Yunis1,2 , Adriana Linares3,4 , Gisela Barros3,4 , Johnny Garcia3,4 ,
Luz Yunis1,2 , Adriana Linares3,4 , Nelson Aponte4,5 , Gisela Barros4,5 , Nelson Aponte3,4 , Laura Niño3,4 , Gloria Uribe5 , Edna Quintero5 , Jose
Johnny Garcia3,4 , Laura Niño3,4 , Gloria Uribe6 , Edna Quintero6 , Juan Perez6 , Leyla Martinez6 , Juan Yunis1,7
Yunis1,2 1 Grupo de patología molecular, Universidad Nacional de Colombia, Instituto

1 Grupo de patología molecular, Universidad Nacional de Colombia, Instituto De Genética, Bogotá, Colombia; 2 Servicios Médicos Yunis Turbay y Cía S.A.S.
De Genética, Bogotá, Colombia; 2 Servicios Médicos Yunis Turbay y Cía S.A.S. Bogotá D.C., Colombia., Instituto De Genética, Bogotá, Colombia; 3 HOMI
Bogotá D.C., Colombia., Instituto De Genética, Bogotá, Colombia; 3 HOMI Fundación Hospital Pediátrico La Misericordia, Bogotá, Colombia, Pedi-
Fundación Hospital Pediátrico La Misericordia, Bogotá, Colombia, Pedi- atric Oncology/hematology Unit, Bogotá, Colombia; 4 Universidad Nacional
atric Oncology/hematology Unit, Bogotá, Colombia; 4 Universidad Nacional de Colombia-HOMI Fundación Hospital Pediátrico La Misericordia, Bogotá
de Colombia-HOMI Fundación Hospital Pediátrico La Misericordia, Bogotá D.C, Colombia, Grupo De Oncohematología Pediátrica, Bogotá, Colombia;
D.C, Colombia, Grupo De Oncohematología Pediátrica, Bogotá, Colom- 5 HOMI Fundación Hospital Pediátrico La Misericordia, Bogotá, Colombia,
bia; 5 Fundación HOMI, Hospital pediátrico la Misericordia, Oncology Unit, Pathology Unit, Bogotá, Colombia; 6 Clínica Infantil Colsubsidio, Bogotá
Bogotá, Colombia; 6 HOMI Fundación Hospital Pediátrico La Misericordia, D.C, Colombia., Unidad De Hemato-oncología, Bogotá, Colombia; 7 Servicios
Bogotá, Colombia, Pathology Unit, Bogotá, Colombia Médicos Yunis Turbay y Cía S.A.S. Bogotá D.C., Colombia., Instituto De
Genética, Bogotá, Colombia
Background and Aims: Different studies have shown that ABCB1,
CDA, DCK, GSTT1 and GSTM1 gene variants are related to drug toxic- Background and Aims: Few studies identifying genomic aspects in
ity in acute myeloid leukemia (AML) patients. Our aim was to identify pediatric acute myeloid leukemia patients have been reported in Latin
the association between single nucleotide variants (SNV) in ABCB1, American countries. The aim of this study was to identify genomic
CDA and DCK and the presence or absence of GSTT1 and GSTM1 alterations, clinical characteristics and outcomes in a cohort of pedi-
genes with clinical outcomes and toxicity in pediatric patients with de atric patients with AML.
novo acute myeloid leukemia. Methods: Patients with confirmed de novo acute myeloid leukemia
Methods: Fifty-one pediatric patients with confirmed de novo acute until 18 years of age were included. Cytogenetics and conventional
myeloid leukemia were included. We used a SNaPshot assay to eval- FISH analysis for t(8,21), Inv16, and KMT2A rearrangements, along
uate ABCB1, CDA and DCK variants. GSTT1 and GSTM1 deletions with genomic analysis for 30 genes panel and 119 fusions by next
were analyzed by conventional PCR. Clinical outcomes and toxicity generation sequencing and rapid testing for FLT3, NPM1 and CEBPA
associations were evaluated using odds ratios and Chi-square analysis. genes were performed. Genomic data was correlated with treatment
Results: ABCB1 (1236G>A,rs1128503) GG genotype had a 6.8 OR response and outcomes.
(p 0.044) for cardiotoxicity. ABCB1 (3435G>A,rs1045642) geno- Results: Fifty-one patients were analyzed, 67.4% had a cytogenetic
type GG had a 4.51 OR (p 0.032) for transaminitis, genotype AA abnormality. Genetic variants were identified in 74.5% of patients.
was a protective factor against relapse 0.69 OR (p 0.025). CDA FLT3 variants (ITD or TKD D835) were found in 14/51 patients
(-451G>A,rs532545) and (79A>C,rs2072671) genotypes CC/AA (27.4%), followed by NRAS (21.6%), KRAS (13.7%) and WT1 and KIT
were associated with increased risk for mucositis and liver toxicity (11.8%). Patients were stratified as high risk (66.6%) after the end of
4.84 OR (p 0.016), while genotypes CT/CA were a protective fac- induction. During treatment, 96% of patients had at least one toxic-
tor against these adverse reactions 0.27 OR (p 0.026). For ABCB1 ity event. Twenty-two out of 33 (66%) patients who received HSCT
(1236G>A,rs1128503/2677C>A/T,rs2032582/3435G>A,rs1045642) had a related toxicity event. FLT3-ITD was associated with relapse
combined genotypes AA/AA/AA, a strong association was found with and measurable residual disease >10 at day 21 11.25 OR (CI 1.89-
death after HSCT 13.73 OR (p 0.009); combined genotypes and CDA 66.72, p 0.006, and 10 OR (CI 1.61-62, p 0.018), respectively. NRAS
(79A>C,rs2072671) or (-451G>A,rs532545) genotypes GG/CC/GG was associated with death during induction 16.71 OR (CI 1.51-184.59,
and CA/CT were associated with MRD >0.1. We did not find any p 0.022).
association between GSTT1 and GSTM1 null alleles with clinical or Conclusions: To date, this is the first study in Colombian pediatric
toxicity outcomes in this cohort. AML patients with a complete characterization from a clinical and
Conclusions: This is the first study of AML pharmacogenetics in Colom- genomic standpoint. Our study highlights the importance of a rapid
bia, a country with a highly admix population. Our results highlight the and systematic incorporation of genetic analysis in pediatric AML in
importance of genetic analysis in pharmacogenetic response and sug- Colombia, as it directly affects treatment decisions and outcomes. The
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S197 of S635

incorporation of targeted therapies with conventional chemotherapy is EP106 / #1425 AN EFFECTIVE COMBINATION THERAPY
an increasingly urgent need in pediatric patients. FOR THE TREATMENT OF PEDIATRIC PLASMA CELL MYELOMA
TYPE POST-TRANSPLANT LYMPHOPROLIFERATIVE DISORDER

E-Poster Topic: AS05 SIOP Scientific programme / AS05.c Lymphomas Mihai Dumbrava1 , Mira Kohorst2 , Paul Galardy2 , Asmaa Ferdjallah2 ,
Shakila Khan2 , Alexis Kuhn3
E-POSTER VIEWING 1 Mayo Clinic, Alix School Of Medicine, Rochester, United States of America;

2 Mayo Clinic, Pediatric And Adolescent Medicine, Rochester, United States


EP105 / #1843 A BRIEF OVERVIEW TO PEDIATRIC GRAY of America; 3 Mayo Clinic, Department Of Pharmacy, Rochester, United
ZONE LYMPHOMAS States of America

Eda Ataseven1 , Gulcihan Ozek1 , Nazan Ozsan2 , Serra Kamer3 , Yavuz Background and Aims: Post-transplant lymphoproliferative disorder
Anacak3 , Serap Aksoylar1 , Mehmet Kantar1 (PTLD) is a serious complication of immunosuppressive therapy follow-
1 Ege University Faculty of Medicine, Pediatric Oncology, Izmir, Turkey; 2 Ege ing solid organ or hematopoietic cell transplantation. Here we report
University Faculty of Medicine, Pathology, Izmir, Turkey; 3 Ege University the first use of the CD38 monoclonal antibody daratumumab for the
Faculty of Medicine, Radiation Oncology, Izmir, Turkey treatment of plasma cell myeloma (PCM) type PTLD in a pediatric
patient. While the standard initial approach for PTLD is the reduc-
Background and Aims: Gray zone lymphoma(GZL) is a rare B-cell lym- tion of immunosuppressive therapy with or without the addition of
phoma in childhood and adolescence, representing features between rituximab, the optimal therapy for PCM-PTLD is less clear.
diffuse large B-cell lymphoma(DLBCL) and classical Hodgkin lym- Methods: We present the cases of three pediatric patients with PTLD
phoma. Due to its rarity, standard treatment options are lacking. including various degrees of plasma cell involvement and review their
Methods: Herein, we describe three patients with GZL. clinical and pathologic characteristics, therapeutic interventions, and
Results: Case-1: A 16-year-old female presented with cervical and outcomes. All data were acquired through review of the electronic
supraclavicular LN swelling. The supraclavicular LN biopsy revealed medical record.
a GZL(LBCL+cHL) with mediastinal, cervical, supraclavicular, axillary, Results: Three pediatric patients were treated for plasma cell pre-
and lung involvement (stageIII). The patient received chemother- dominant PTLD at our institution between 2010-2020. The first (a
apy according to the B-NHL BFM 2012. At the end of the therapy, 12-year-old female, status post heart transplant) and second (a 15-
PET-CT demonstrated a new axillary LN, which was shown to be year-old female, status post heart transplant) received a standard
a GZL(LBCL+HL). The patient received second-line treatment with rituximab-based approach and unfortunately relapsed, requiring addi-
3×R-ICE followed by HD-BEAM with ASCT.She received brentux- tional chemotherapy. The third patient was a 9-year-old male, status
imab vedotin (BV) maintenance after ASCT. The patient has been in post kidney transplant, who was diagnosed with PCM-PTLD (CD20
CR for 18 months. Case-2: A 14-year old female was admitted with negative, CD38 positive) and treated with bortezomib, dexametha-
cough and swelling of cervical LN. Initial diagnosis revealed classic sone, and daratumumab. Despite the paucity of data governing the
HL, stage IIA. The patient received chemotherapy and radiotherapy use of daratumumab in pediatric patients, our pediatric-adapted dara-
according to the GPOH-HD 95. After eight months, she presented tumumab administration approach allowed for safe administration
with axillary LN, and relapse was shown to be a GZL(LBCL+cHL).The of 4 cycles. This combined treatment regimen, including the anti-
patient received treatment with 3×B-IGEV followed by HD-BEAM CD38 monoclonal antibody daratumumab, was safe, well-tolerated,
with ASCT.She received BV maintenance after ASCT.The patient has and resulted in a sustained remission for 2 years.
been in CR for 18 months. Case-3: A 16-year-old male presented Conclusions: In the third case for a pediatric patient, the combination
with cervical LN,and biopsy revealed a GZL(LBCL+cHL) with mediasti- therapy of bortezomib, dexamethasone, and daratumumab was well-
nal, cervical, supraclavicular, and abdominal LN involvement(stageIII). tolerated and produced remission from PCM-PTLD. This treatment
The patient received chemotherapy according to the B-NHL BFM regimen is encouraging and supports further evaluation in larger stud-
2012. PET-CT demonstrated a complete remission at the end of the ies, as well as further discussion of daratumumab maintenance therapy
therapy, and he received involved-field irradiation.After two months, for the sustained remission of PCM-PTLD.
he relapsed with mediastinal, abdominal, and lung involvement. The
LN biopsy was shown to be HL.The patient received 3×B-IGEV fol-
lowed by HD-BEAM with ASCT. He received BV maintenance after EP107 / #1770 INFRADIAPHRAGMATIC HODGKIN’S
ASCT. LYMPHOMA IN PEDIATRIC CANCER PATIENTS
Conclusions: GZL remains a therapeutic challenge.Most of the time,
it has inferior outcomes compared to LBCL and HL and a ten- Nashwa Ezz Eldeen1 , Emad Moussa2 , Mohamed Saad Zaghloul3 ,
dency to relapse. Prospective studies evaluating effective treatment Madeha Elwakeel4 , Eman Khorshed5,6 , Maha Omran7 , Asmaa
approaches are highly needed. Hamoda8
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S198 of S635 ABSTRACTS

1 national cancer insititute, children cancer hospital of Egypt (57357), Morozova, Yuri Punanov, Natalia Mihailova, Alexander Kulagin,
Pediatic Oncology, Cario university, Egypt; 2 department of clinical oncol- Ludmila Zubarovskaya
ogy,Menufeya university, children cancer hospital of Egypt (57357), Clinical Pavlov First St. Petersburg State Medical University, Rm Gorbacheva
Oncolgy, cairo, Egypt; 3 Children’s Cancer hospital Egypt 57357/ National Research Institute, Pavlov University, St. Petersburg, Russian Federation,
Cancer Institute - Cairo University, Radiotherapy, Cairo, Egypt; 4 National Санкт-Петербург, Russian Federation
Cancer Institute - Cairo University, Egypt/ Children’s Cancer hospital
57357, Cairo, Egypt, Radiology, Cairo, Egypt; 5 Children’s Cancer hospi- Background and Aims: The prognosis in pediatric relapsed or refrac-
tal Egypt 57357/ National Cancer Institute - Cairo University, Surgical tory (R-R) NHL is unfavorable; no matter what form of modern
Pathology, Cairo, Egypt; 6 National Cancer Institute - Cairo University, Egypt/ treatment is adopted. New treatment approaches need to be intro-
Children’s Cancer hospital 57357, Cairo, Egypt, Surgical Pathology, Cairo, duced.
Egypt; 7 national cancer insititute, cairo unversity, Nuclear Medicine, cairo, Methods: We used CPIs in 11 children with R-R NHL. The median age
Egypt; 8 National Cancer Institute - Cairo University, Children’s Cancer was 12 (2–17) years. The distribution of the patients by diagnosis was
hospital Egypt 57357, Pediatric Oncology, cairo, Egypt as follows: PMBCL (n = 4), PTCL (n = 4), DLBCL (n = 1), LBL (n = 2).
The median number of prior lines of therapy was 3 (2–5). Refractory
Background and Aims: Infradaiphragmatic Hodgkin’s lymphoma (HL) NHL was observed in 7 cases, and 4 patients had had multiple relapses
is a rare disease occurring in <5% of pediatric patients. The prognostic (≥ 3). Nivolumab was administered at a dose of either 1 mg/kg (n =
impact of an infradiaphragmatic localization of this lymphoma is con- 4) or 3 mg/kg (n = 3) every 2 weeks, Pembrolizumab - at a dose of 2
troversial.we aimed to evaluate the baseline clinicopathologic features, mg/kg every 3 weeks (n = 4). The median number of CPI doses received
prognostic factors and outcome of pediatric patients with infradi- by the patients was 3 (1–12). In 7 patients, CPIs were administered as
aphragmatic HL diagnosed and treated at children cancer hospital monotherapy, in 4 – in combination with cytostatic agents. Once remis-
57357 sion was achieved, we used hematopoietic stem cell transplantation for
Methods: Between 2007 and 2020, All patients with histologically con- consolidation.
firmed clinical stage I/II infradiaphragmatic HL retrospectively were Results: Response to the CPI therapy was observed in 4 out of 11
evaluated including clinical presentation, initial laboratories & radi- patients (complete response – in 2 patients); stabilization - 1 patient.
ological findings, response to initial treatment & their outcome in Only patients with PMBCL (n=3) and PTCL (n=1) responded to the
comparison with supradiaphragmatic stage I/II treatment. Two responders were PDL1-postive. At the median follow-
Results: Among 999 Hodgkin’s lymphoma staged I/II, there were up of 257 (13–1668) days, 6 patients were alive, with 4 of them
35 infradiaphragmatic HL patients with male to female ratio 2.5 :1, remaining in durable remission.
the mean age was 10 years, 28.6%(10/35) of cases were histologi- Conclusions: This paper is one of the first reports on the success-
cally NLPHL while 37.1% (13/35) were histologically Mixed Sclero- ful use of CPIs in children with R-R NHL. PMBCL and PTCL turned
sis CHL, 37%.1(13/35) of patients presented with B-symptoms, In out to be responsive to the treatment. This therapy can be used
54.3%(19/35) of cases ESR was less 30, 62.9% of patients (22/35) did to achieve remission or possibly even cure in children whose only
not receive Radiotherapy, 14.3% (5/35) of patients relapsed, Over- option would be palliative care if they were treated with standard
all Survival was 87.7% while Event Free survival was 81.3% at 60 approaches.
months, OS of the patient with negative interim PET/CT was 100%
versus 50% in patients with inadequate response at 60 months with
significant P-value:0.04.OS according to diaphragmatic site was highly EP109 / #241 PROGNOSTIC SIGNIFICANCE OF PD1, PD-L1
statistically significant P-value: 0.009 (87.7% vs 98.3%) infradiaphrag- EXPRESSION AND METABOLIC ACTIVITY ON 18F-FDG PET/CT
matic HL versus supradiaphragmatic,Correlation of EFS according to IN REFRACTORY/RELAPSING PEDIATRIC HODGKIN LYMPHOMA
diaphragmatic site was not statistically significant P-value:0.075 how-
ever infradiaphragmatic HL EFS was 81.39% which is still lower than Reham Khedr1 , Eman Khorshed2 , Hany Abdelrahman1 , Omneya
supradiaphragmatic stage I/II 91.5% at 60 months. Hassanein3 , Madeha Elwakeel4 , Mohamed Saad Zaghloul5 , Asmaa
Conclusions: in spite of infradiaphragmatic HL cases does not carry Hamoda6
high risk features still this category of the patients has lower OS 1 Children’s Cancer hospital Egypt 57357/ National Cancer Institute -
& EFS in comparison to patients who had supradiaphragmatic initial Cairo University, Pediatric Oncology, Cairo, Egypt; 2 Children’s Cancer hos-
presentation which mandate further studies. pital Egypt 57357/ National Cancer Institute - Cairo University, Surgical
Pathology, Cairo, Egypt; 3 Children’s Cancer hospital Egypt 57357, Clinical
Research, Cairo, Egypt; 4 Children’s Cancer hospital Egypt 57357/ National
EP108 / #1499 IMMUNE CHECKPOINT INHIBITORS IN Cancer Institute - Cairo University, Radiology, Cairo, Egypt; 5 Children’s Can-
PEDIATRIC NON-HODGKIN LYMPHOMAS cer hospital Egypt 57357/ National Cancer Institute - Cairo University,
Radiotherapy, Cairo, Egypt; 6 National Cancer Institute - Cairo University,
Andrei Kozlov, Ilya Kazantsev, Tatiana Yukhta, Polina Tolkunova, Children’s Cancer hospital Egypt 57357, Pediatric Oncology, cairo, Egypt
Asmik Gevorgian, Artem Potanin, Egor Zakharov, Vadim Baykov, Elena
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S199 of S635

Background and Aims: Hodgkin Lymphoma (HL) is a unique disease Children Hospital, Department Of Pediatric Hematology-oncology, Athens,
entity both in its pathology and the young patient population that it Greece
primarily affects. Several meta-analyses have demonstrated that high
PD-L1 expression levels are correlated with adverse clinical and patho- Background and Aims: Pediatric Hodgkin Lymphoma (HL) is the third
logic features. This study aims to evaluate the correlation between the most common childhood malignancy. Nowadays, it is a highly curable
expression of PD-L1 and clinicopathological features, as well as the disease and this success is mainly related to the treatment risk-
prognostic significance of PD-L1 expression with regard to interim PET adaptive strategies applied. Aim:the retrospective study of the clinical-
response in relapsing / refractory pediatric HL epidemiological data and survival-rate of pediatric HL during the
Methods: We measured the expression of PD-1/PD-L1 in the last 10 years in Greece and the evaluation of possible late-occurring
baseline diagnostic samples of children with relapsing/ refractory adverse events in this cohort.
classical HL. The results were correlated with the pathologi- Methods: We retrospectively reviewed data from the medical records
cal subtypes as well as the clinical outcome in relation to PET of children diagnosed, treated, and followed up in all the Pediatric
response. Oncology Departments in Greece, during the period 2010-2020.
Results: Of the 88 included patients, 77% had advanced-stage HL. Demographic, clinical, radiological, laboratory data, treatment proto-
PD-1 expression was detected in 50% of cases, whereas PD-L1 (mem- col and follow-up data were evaluated. All patients were followed-up
branous) was expressed by tumor cells in 60% of the cases, and strongly until the end of 2021. Distributions of Overall Survival (OS) and
expressed in 16% of cases. Notably, PD-L1 (cytoplasmic) was detected Disease-Free Survival (DFS) were assessed with Kaplan–Meier curves.
in 55% of the cases. There was a significant difference in the expres- Results: Data were collected from 183 patients. Median age was
sion levels of PDL-1 between the different pathological subtypes (p = 12.93 years (range: 3.62-20.45 years). The cohort study included
0.006). OS of patients with PD-L1expression (Cytoplasmic) was 83% vs 105 males and 78 females. The most common subtype was nodu-
91% in patients with absent expression (P=0.001). There was no prog- lar sclerosis in 132/183 (72.1%). Three patients (1,63%) had Nodular
nostic significance of PD-L1 expression with regard to PET response lymphocyte-predominant Hodgkin lymphoma (NLPHL). Seventy-six
(p=0.31). patients (41.8%) were diagnosed with early-stage disease, while 106
Conclusions: Although PD-L1 expressions did not show statistical (58.2%) with advanced stage. B symptoms were present in 39/183
significance with well-established prognostic factors, our preliminary (21.3%). Since 2013, all patients in Greece were treated according to
data indicate that pathological subtypes and cytoplasmic expression the EURONET-PHL protocol, 134 (82.2%) patients with EURONET-
of PD-L1 may have a prognostic implication on survival in pediatric PHL-C1 and 29 with EURONET-PHL-C2. A total of 179/183 patients
HL. are alive. Relapse occurred in 14/183. Second malignancies are
reported in 4 patients, 2 solid tumors (thyroid gland and breast) and 2
hematological malignancies (acute myelogenous leukemia). Endocrine
EP110 / #1006 HODGKIN LYMPHOMA. TEN YEARS abnormalities (abnormal thyroid function and gonadal dysfunction)
EXPERIENCE FROM THE HELLENIC WORKING GROUP were reported in 17.7% of the patients with available data. Overall
survival for the whole cohort was 97.8% and DFS was 90.7%.
Maria Kourti1 , Andromahi Papagianni2 , Dimitrios Doganis3 , Katerina Conclusions: Excellent survival outcome has been achieved, in this
Katsibardi4 , Charikleia Kelaidi5 , Nikolaos Katzilakis6 , Eleni Dana7 , cooperative Greek study. Longer follow-up will give valuable insight in
Aikaterini Kesari8 , Maria Ioannidou2 , Maria Nikita3 , Georgia the long-term outcome and late toxicity in order to optimize first-line
Avgerinou4 , Vasiliki Tzotzola9 , Kyriaki Kotsoglanidou1 , Iordanis and relapse treatment.
Pelagiadis6 , Ioulia Peristeri8 , Helen Kosmidis7 , Eftichia Stiakaki6 ,
Evgenia Papakonstantinou1 , Sophia Polychronopoulou5 , Antonis
Kattamis4 , Margarita Baka3 , Emmanuel Hatzipantelis2 EP111 / #908 ANAPLASTIC LARGE CELL LYMPHOMA,
1 “Hippokration” General Hospital, Thessaloniki, Greece, Paediatric Oncol- EXPERIENCE IN 28 YEARS OF A PEDIATRIC CENTER IN
ogy Department, Thessaloniki, Greece; 2 Aristotle University of Thessaloniki, ARGENTINA
AHEPA Hospital, Children’s & Adolescent’s Hematology-oncology Unit, 2nd
Department Of Paediatrics, Thessaloniki, Greece; 3 ‘P & A. Kyriakou’ Chil- Ludy López, Silvina Romano, Vanesa Gimenez, Eugenia Altamirano,
dren’s Hospital, Department Of Pediatric Hematology-oncology, Athens, Marcela Aznar, Maria Cuello, Wanda Goldman, Claudia Ruiz, Virginia
Greece; 4 “Aghia Sophia” Children’s Hospital, Division Of Pediatric Hematol- Schuttenberg, Alejandra Costa
ogy Oncology, First Dpt Of Pediatrics, National & Kapodistrian University Hospital Inter zonal agudo especialista en pediatría "Sor María Ludovica",
Of Athens, Athens, Greece; 5 “Aghia Sophia” Children’s Hospital, Department Provincia De Buenos Aires, La Plata, Argentina
Of Pediatric Hematology-oncology, Athens, Greece; 6 University Hospital
of Heraklion, Department Of Pediatric Hematology-oncology, Heraklion, Background and Aims: Anaplastic large cell lymphoma (ALCL) rep-
Greece; 7 Mitera Children’s Hospital, Department Of Pediatric And Ado- resents 10 to 15% of pediatric Non-Hodgkin lymphomas (NHL).
lescent Hematology-oncology, Athens, Greece; 8 “Aghia Sophia” Children’s Different clinical, pathological and molecular characteristics are still
Hospital, Bone Marrow Transplantation Unit, Athens, Greece; 9 Aghia Sophia being evaluated as prognostic factors. To analyse clinical and biological
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S200 of S635 ABSTRACTS

characteristics and therapeutic response of pediatric patients diag- file or database reviews were conducted on all patients with BSIs to
nosed with ALCL. determine clinical outcomes.
Methods: retrospective review analysis of clinical records of patients Results: Microbiologically confirmed BSIs were observed in 30(15%)
with ALCL from January 1994 to January 2022. Diagnosis confirmed of the 196 peripheral blood cultures performed. The mean age was 7
according to WHO criteria. Clinical, histopathological and treatment years (SD ±3.6) and 18(62%) were male. Patient files were reviewed
data were collected. Therapeutic schemes based on BFM90 and in 24/30(80%) and the remaining 6/30(20%) clinical files were missing,
ALCL99 protocols. Statistical analysis: SPSS 20.0 Software. so data was obtained from a database. Nineteen of 30(67%) BSIs were
Results: One hundred forty patients with NHL were admitted, 18 identified in patients with leukemia or lymphoma. Thirteen of 24(54%)
patients (12.8%) with ALCL. Fifteen patients were evaluable, median patients had received >48 hours of antibiotics before blood culture
age 7.9 years old (r3-15.3), 53% male. Median follow-up: 4.1 years collection. Thirty-seven bacterial isolates were identified, including
(IQR0.3-9.2). Eleven patients (73%) presented B symptoms. Nodal 5 patients with multiple isolates. Gram-negative organisms were
commitment (100%). Most affected areas were: cervical, mediastinal identified in 28(75%) of 37 isolates [9(32%) Klebsiella spp., 4(14%)
and abdominal. Twelve patients (80%) presented extranodal involve- Escherichia coli, 3(11%) Pseudomonas; and 12(43%) other]. Majority
ment. Twelve patients presented advanced stage (III/IV) according to of gram-negative isolates were resistant to ceftriaxone (15/28,53%),
St.Jude staging system. Four patients presented Karnofsky Score (KPS) but sensitive to piperacillin-tazobactam (3/3,100%) and meropenem
<50, this being a factor associated with lower OS compared to those (24/28,86%); and 8 multi-drug resistant (MDR) isolates sensitive to
with KPS ≥50 (OS 25% vs 90%, p0.013). Eight patients had LDH >500 meropenem only. Gram-positive organisms were identified in 9(24%)
IU/L. ALK determination in 11 patients: ten positive and one nega- of 37 isolates [7/9(78%) staphylococcal spp.; all resistant to penicillin].
tive. Twelve patients (80%) achieved full remission. One patient (6.6%) Sixteen (53.3%) of 30 patients with a BSI died within 2 weeks of the
relapsed 4 years after diagnosis, undergoing second line treatment culture.
and autologous hematopoietic stem cell transplantation Four patients Conclusions: BSI are common among pediatric cancer patients in
(26.6%) died within first months of treatment: 2 due to infectious com- Lilongwe, Malawi, with increased MDR bacterial isolates and high mor-
plications; one due to disease progression refractory to treatment; and tality. This study is limited by pre-treated cultures and incomplete data.
another due to respiratory failure during prephase. The 5-year OS for Future prospective studies comprehensively looking at BSI among
this group was 73% and the EFS was 64%. febrile neutropenic patients are urgently needed to improve clinical
Conclusions: the incidence and OS of ALCL was similar to that care and outcomes.
reported. Deaths were observed in the first months of treatment.
KPS<50 at admission was associated with poorer survival. Early
clinical suspicion, timely diagnosis and adequate clinical support deter- EP113 / #1162 OEPA/COPDAC THERAPEUTIC REGIME IN
mined the evolution in this population. CHILDREN WITH ADVANCED-STAGE HODGKIN LYMPHOMA
AND OMISSION OF RADIOTHERAPY IN ADEQUATELY
RESPONDING PATIENTS: EXPERIENCE FROM A TERTIARY CARE
EP112 / #1052 MICROBIOLOGICAL PROFILE OF BLOOD CENTER IN MÉXICO
STREAM INFECTIONS IN PEDIATRIC CANCER PATIENTS AT
KAMUZU CENTRAL HOSPITAL, IN LILONGWE, MALAWI Marsenia Meneses Lazo1 , Manuel Martínez1,2 , Oscar Gonzalez
Ramella1 , Fernando Antonio Sánchez Zubieta1
Gugulethu Mapurisa1 , Gerald Tegha1 , Apatsa Matatiyo1 , Samuel 1 Hospital Civil de Guadalajara Dr. Juan I Menchaca, Hemato-oncología
Makuti2 , Rizine Mzikamanda2 , Casey Mcatee2,3 , Nmazuo Ozuah2 , Pediátrica, Guadalajara, Mexico; 2 Hospital Civil de Guadalajara Dr. Juan I
Katherine Westmoreland1,4 Menchaca., Hemato- Oncología Pediátrica., Guadalajara, Mexico
1 UNC Project Malawi, Cancer Program, Lilongwe, Malawi; 2 Baylor College
of Medicine Children’s Foundation, Malawi, Texas Children’s Global Hope, Background and Aims: Although the majority of children and adoles-
Lilongwe, Malawi; 3 Baylor College of Medicine, Pediatric Hematology And cents diagnosed with advanced-stage Hodgkin lymphoma will achieve
Oncology, Houston, United States of America; 4 University of North Carolina, remission and cure, it has been associated with significant long-term
Pediatric Hematology Oncology, Chapel Hill, United States of America side effects. The objective of this study is to describe the results of
the use of the OEPA/COPDAC therapeutic regimen in patients with
Background and Aims: Febrile neutropenia is a serious treatment- high-risk Hodgkin’s lymphoma. In addition to investigating whether
related complication. There is scant literature on the microbiological radiotherapy can be safely omitted in patients with an adequate
profile of bloodstream infections (BSIs) in pediatric cancer across response.
Sub-Saharan Africa (SSA). Methods: A retrospective study of patients younger than 18 years
Methods: A cross-sectional study from January 1, 2019, to December with high-risk Hodgkin’s lymphoma, was done from January 2017 to
31, 2021, of pediatric cancer patients aged <16 who had a peripheral February 2022 in a tertiary care center in Mexico
blood culture performed. Routine bacteria identification and antibi- Patients received 2 cycles of OE*PA ( vincristine, etoposide, pred-
otic susceptibility testing of bacterial isolates were performed. Patient nisone, and doxorubicin ) and 4 cycles of consolidation therapy with
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S201 of S635

COPDAC ( cyclophosphamide, vincristine, prednisone, and dacar- dose regimens used in SSA to higher intensity BFM-based regimens.
bazine ). Radiation therapy was omitted in patients with complete Children were followed prospectively for clinical outcomes. We eval-
remission to induction chemotherapy. uated the distribution of clinical phenotypes and compared response,
Results: Twenty-one patients were included. The mean age at the pre- treatment-related mortality (TRM), and survival by chemotherapy
sentation was 11.6 years, male predominance (61.9%), B symptoms protocol using Kaplan-Meier survival analysis.
were present in 61%, bulky disease in 71.4%, and CHIPS score of 3 in Results: Fifty-six children were included in the analysis, with a median
33%. Histopathology was reported: mixed cellularity 57.2%, nodular age at diagnosis of 8 years, and 64% were male. Fifty-two (92.9%) had
sclerosis 38.1%, and rich in lymphocytes 4.7%. Burkitt lymphoma, while 7.1% had Diffuse Large B Cell lymphoma.
After therapy with 2 cycles of OE*PA, 81% had complete remission, The abdomen was the commonest disease site at 71.4%, while jaw
so radiotherapy was omitted and consolidation therapy was contin- masses accounted for 17.9%. The distribution of risk strata (based
ued. Only 19% had partial remission, so involved-field radiotherapy on Murphy Stage and LDH) was 83%, 15%, and 2% for high, inter-
was started at a dose of 25 Gy. mediate, and low risk, respectively. The initial 13 patients (23.2%)
With a median follow-up of 36 months, we had 0% abandonment of received the 1g/m2 methotrexate regimen with 30.8%(4) remissions,
therapy, 0% relapses and progressive disease, and no deaths. Overall 0.08%(1) TRM, and 38.5% and 23% 1-year overall and relapse-free
survival and event-free survival were 100%. NCI-CTC grade 2 and 3 survival, respectively. We then escalated therapy for the subsequent
hematologic toxicities were observed in 9.5% and 38% respectively. 31 children to include doxorubicin (50mg/m2 ), with 48.4%(15) remis-
NCI-CTC grade 1 sensory neurotoxicity was 42.8%. sions, 0.1%(3) TRM, and 74% and 70% 1-year overall and relapse-free
Conclusions: The OEPA/COPDAC regimen is well tolerated with survival, respectively.
acceptable toxicity; radiotherapy could be omitted in patients with Conclusions: MBCL in SSA is predominantly high-risk abdominal
an adequate response to intensified induction, avoiding late repercus- Burkitt lymphoma warranting higher intensity chemotherapy. Escala-
sions; however longer follow-up is needed. tion of BFM-based chemotherapy was safe and resulted in improved
outcomes in our experience. Future goals are to continue this diag-
nostic, risk stratification, and chemotherapy escalation strategy and
EP114 / #1670 EVOLVING CLINICAL EPIDEMIOLOGY OF disseminate it to other centers in SSA.
PEDIATRIC BURKITT/MATURE B CELL LYMPHOMA IN
SUB-SAHARA AFRICA (SSA) AND INTENSIFICATION OF
CHEMOTHERAPY TO IMPROVE OUTCOMES EP115 / #760 APPLICATION OF WEIBULL AFT PARAMETRIC
REGRESSION MODEL ANALYSIS FOR PATIENTS WITH
Rizine Mzikamanda1 , Irene Nzamu2 , Ronald Naitala3 , Barnabas AGGRESSIVE LYMPHOMA IN AMPATH KENYA
Atwiine4 , Robert Langat5 , Deo Munube2 , Ruth Nammazi6 , Peter
Wasswa7 , Joseph Lubega8 Dennis Njenga1 , Terry Vik2 , Peace Mbengei3 , Gilbert Olbara4 , Festus
1 Baylor College of Medicine Children’s Foundation, Malawi, Texas Chil- Njuguna5
dren’s Global Hope, Lilongwe, Malawi; 2 Makerere University, College Of 1 Ampath, Hemato-oncology, Eldoret, Kenya; 2 Indiana University School of
Health Sciences, Pediatrics Department, Kampala, Uganda; 3 Joint Clinical Medicine, Pediatrics, Indianapolis, United States of America; 3 Ampath, Blp,
Research Centre, Texas Children’s Hospital Global Hope Program, Kam- Eldoret, Kenya; 4 Moi Teaching and Referral Hospital, Pediatric Oncology,
pala, Uganda; 4 Mbarara University of Science and Technology, Department Eldoret, Kenya; 5 Moi University, Pediatric Oncology, Eldoret, Kenya
Of Paediatrics, Mbarara, Uganda; 5 Baylor College of Medicine Childrens
Foundation, Texas Children’s Hospital Global Hope Program, Gaborone, Background and Aims: Most models used in survival analysis assume
Botswana, Gaborone, Kenya; 6 Makerere University, Department Of Pae- a distribution-free model meaning no assumption has to be made about
diatrics, Kampala, Uganda; 7 Global HOPE, Pediatrics, Kampala, Uganda; the survival times distribution. Approximating the survival distribution
8 Texas Children’s Hospital, Global Hematology/oncology Pediatric Excel- that includes log-normal, log-logistic, exponential, and Weibull distri-
lence, Houston, United States of America butions, we can employ parametric failure time analysis in parametric
survival models. We aim to examine the use of the Weibull model in the
Background and Aims: Mature B Cell Lymphoma (MBCL) is the com- parametric-failure analysis of children with NHL-B in a case of western
monest lymphoma in children, with survival in SSA lower than in Kenya.
high-income countries. This study aimed to demonstrate the evolution Methods: A retrospective review of records that collected data that
of clinical phenotypes and the feasibility and benefit of chemotherapy included clinical data, diagnostic procedures as well as treatment
intensity escalation for MBCL to improve children’s survival in the SSA outcomes. Multivariate regression analysis was performed and the
setting. possible prognostic factors were put in the Weibull model and the Cox
Methods: From March 2019 to December 2021, children under 18 proportional hazard model. The Akaike Information Criterion (AIC)
years with MBCL were diagnosed using standard WHO criteria and was used to find the best-fitted model.
risk-stratified by BFM criteria. The treatment strategy was to escalate Results: The prognostic factors included in the Weibull model gave
chemotherapy intensity methodically and serially from traditional low- more accurate estimates than the estimates from the Cox model.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S202 of S635 ABSTRACTS

The AIC showed that the data fit the Weibull model better than the rate in February 2025 will be increased to 355 (95% CI, 276.35518 -
Cox model (218 and 230 respectively). Comparing the parametric and 434.9839) based on forecasting with the ARIMA model.
the Weibull regression models, the Weibull model fitted well with Conclusions: The increasing trend of NHL-B will be continued which is
the non-parametric model. The hazard rates for age (1.088;95% CI, considered based on the ARIMA method result. These results suggest
0.9942-1.1343 and 1.082;95% CI, 0.9973 1.1430) and sex (1.889; 95% that analyzing a time series ARIMA model helps to prognosticate the
CI, 0.3873 1.6500 and 1.870;95% CI 0.4373 1.8681) are more than 1 prevalence of NHL-B and improve the forecast precision.
both in the multivariate Weibull and Multivariate Cox models respec-
tively implying that there is an increased risk of mortality from BL as
age increases same as the sex. EP117 / #509 EFFECTS OF TUMOR LOCALIZATION ON THE
Conclusions: The Cox model assumes a proportional hazard model and SURVIVAL FOR NHL-B
is distribution-free implying that if the assumption doesn’t hold, then
the model is likely to lead to unreliable conclusions. Thus, in cases Gilbert Olbara1 , Terry Vik2 , Festus Njuguna3 , Peace Mbengei4 , Dennis
where the distribution of population survival data is likely to be iden- Njenga4
tified, a parametric approach to the accelerated failure time analysis is 1 Moi Teaching and Referral Hospital, Pediatrics, Eldoret, Kenya; 2 Indiana
more powerful compared to the semi-parametric and non-parametric University School of Medicine, Pediatrics, Indianapolis, United States of
methods. America; 3 Moi University, Pediatric Oncology, Eldoret, Kenya; 4 Ampath, Blp,
Eldoret, Kenya

EP116 / #762 A TIME SERIES ARIMA MODEL APPROACH TO Background and Aims: In sub-Saharan Africa, Burkitt lymphoma; an
FORECASTING THE PREVALENCE OF BURKITT’S LYMPHOMA aggressive cancer of B lymphocytes with high mortality in low-income
IN AMPATH KENYA countries is a disease of children. We aim to evaluate the relevance of
primary tumor location as a prognostic factor in the survival of patients
Dennis Njenga1 , Terry Vik2 , Gilbert Olbara3 , Festus Njuguna4 , Moses with BL.
Elvis Oburah5 , Peace Mbengei6 Methods: Retrospective review of histological cases diagnosed and
1 Ampath, Blp, Eldoret, Kenya; 2 Indiana University School of Medicine, Pedi- confirmed BL in infants from 2018 to 2021 (n=65). Patients were cate-
atrics, Indianapolis, United States of America; 3 Moi Teaching and Referral gorized by primary tumor site into abdomen (80%, n = 52), jaw (18.46%,
Hospital, Pediatric Oncology, Eldoret, Kenya; 4 Moi University, Pediatric n = 12), and jaw and abdomen (1.54%, n = 1) groups. Subgroup analy-
Oncology, Eldoret, Kenya; 5 Moi Teaching And Referral Hospital-AMPATH, sis was used for comparative analysis of Event-free survival (EFS) and
Hematology And Oncology, ELDORET, Kenya; 6 Ampath, Hemato-oncology, overall survival (OS) rates.
Eldoret, Kenya Results: Fifty-two patients (80%),12 patients (18.46%), and 1patient
(1.54%) presented with abdominal, jaw, jaw-abdomen tumors respec-
Background and Aims: A time-series study determines the proba- tively. Twenty-two patients (42.31%) with abdominal tumors had CR
ble model and predicts its values in the future to facilitate statistical compared to 23 patients (44.23%) and 7 patients (13.46%) with PD
analysis of the variables based on time. Prevalence and incidence of and PR respectively. Six patients (50%) with jaw tumors had PD while
cancers are getting increased with aging and population growth; thus, 5 patients (41.67%) and 1 male (8.33%) had a CR and PR respec-
this study aims to predict the prevalence rate per 100 population of tively. Thirty-four patients (65.38%) with abdominal tumors were alive,
aggressive lymphoma in children in a case study of western Kenya using 16(30.77%) were deceased, and 2(3.85%) were LTFU. Six patients
time series models. (50%) with jaw tumors were alive, 6 (50%) were deceased. Median EFS
Methods: Retrospective review of NHL-B cases from May 2018 to was 324 days (95% CI, 294-532) and median OS was 310 days (95%
August 2020. The time variable was each month of the study years CI, 294-437). Median EFS for abdominal tumors was 320 days (95% CI,
and using the number of daily registered cancers in each month, the 294-539), jaw tumors were 324 days (95% CI, 229-502). Median OS for
time series of the monthly period prevalence rate per 100 popula- abdominal tumors was 310 days (95% CI, 294-497), jaw tumors were
tion was designed. ARIMA methods were used to design a probable 324 days (95% CI, 294-502) and jaw-abdomen was 299 days
prediction model based on the autocorrelation model (ACF) and par- Conclusions: Tumor localization to the jaw is associated with an
tial autocorrelation model (PACF). The goodness of fit of the final improved survival rate both from EFS and OS. Those localized to the
model was assessed using Akaike Information Criterion (AIC), Bayesian abdomen had a worse survival rate compared with jaw tumors which
Information Criterion (BIC), and Box Ljung. may be due to biological differences between tumor sites.
Results: The model parameters namely, the p and q parameters were
estimated as 0 and 1 respectively and the best ARIMA model was con-
sidered as ARIMA (p=0, d=1, q=1) that only had 1 moving average EP118 / #645 PREVALENCE AND CHARACTERIZATION OF
component and a difference of 1. This model had the least AIC (114.59) PEDIATRIC EBV-NEGATIVE POST-TRANSPLANT
and BIC (117.73). The number of NHL-B cancer cases in May 2018 LYMPHOPROLIFERATIVE DISORDER: SINGLE CENTER STUDY
was 3 with a prevalence rate of 4.615. This study showed that the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S203 of S635

Maegan Ford1 , Sonika Reddy2 , Marc Richmond3 , Mercedes Fatima Ezzahra Rida, Siham Cherkaoui, Mouna Lamchahab, Meryem
Martinez4 , Prakash Satwani1 , Manuela Orjuela-Grimm5 Qachouh, Mohamed Rachid, Abdellah Madani, Nissrine Khoubila
1 Columbia University Irving Medical Center, Division Of Pediatric Hema- university hospital center IBN ROCHD, Clinical Hematology And Pediatric
tology, Oncology, And Stem Cell Transplant, New York, United States of Oncology, Casablanca, Morocco
America; 2 Columbia University Irving Medical Center, Department Of Pedi-
atrics, New York, United States of America; 3 Columbia University Irving Background and Aims: Hodgkin’s lymphoma (HL) is a pathology that
Medical Center, Division Of Pediatric Cardiology, New York, United States can be cured in the first line in the majority of cases in children and
of America; 4 Columbia University Irving Medical Center, Division Of Pedi- young adults. The therapeutic failure rate found in the literature is 10%
atric Gastroenterology And Transplant Hepatology, New York, United States in the favorable group and 15 to 20% in the advanced stages. In our
of America; 5 Columbia University, Epidemiology, Department Of Pediatrics: context, the salvage of refractory Hodgkin’s lymphoma by chemother-
Hematology, Oncology, And Stem Cell Transplantation, New York, United apy followed by autologous hematopoietic stem cell transplantation
States of America sometimes remains difficult given the lack of resources that can delay
treatment. The objective of our work is to draw up the clinical, thera-
Background and Aims: Post-transplant lymphoproliferative disorder peutic and evolutionary profile of patients refractory to the first line of
(PTLD) is the most common malignancy in pediatric post-solid organ treatment for Hodgkin’s lymphoma.
transplant (SOT) patients. While the association of Epstein-Barr Virus Methods: This is a monocentric retrospective study, conducted over
(EBV) with the development of PTLD is well recognized, little is known 7 years and 5 months (January 2011-May 2019) at the Casablanca
about EBV-negative pediatric PTLD. Therefore, we aimed to charac- hematology center. All patients aged under 21 with the diagnosis of
terize differences between pediatric EBV-positive and EBV-negative Hodgkin’s lymphoma were included.
PTLD to better understand the pathogenesis of EBV-negative PTLD. Results: 19 patients were collected, 13 of whom were refractory to
Methods: We retrospectively reviewed clinical data from SOT patients first-line treatment and 6 cases of relapse after a first complete remis-
diagnosed with PTLD in a single pediatric center from 2005 to 2021. sion, representing an overall rate of 12.7% and 6% respectively. In the
Data included demographics, SOT type, time from SOT transplant to table below, we summarize the characteristics of patients with pri-
PTLD diagnosis, PTLD site, histopathological morphology by WHO cri- mary failure. Relapsed patients had a median age of 12.5 years [10-18]
teria, and tumor markers, including EBV. Cases were compared by and the median time to relapse was 24 months [15-69]. The latter
tumor EBV expression using χ2 , Mann-Whitney, and Fisher’s Exact were in the EORTC unfavorable prognosis group. In the first line, 5
tests. patients had received the OEPPA/COPDAC protocol and one patient
Results: We identified 60 patients meeting the inclusion criteria. Of ABVD. As a catch-up protocol, 4 patients had received the ICE protocol
those, 25% (n=15) had EBV-negative PTLD while 75% (n=45) had EBV- and 2 the DHAP protocol. Five patients had undergone HSC autograft
positive PTLD. The distribution of sex and race/ethnicity did not differ and at the final status 3 patients were put in maintained complete
by tumor EBV status. EBV-negative PTLD was more likely to occur remission.
in heart transplant patients (73.3% v. 42.2%, p <0.04). The median Conclusions: Catching up with refractory or relapsed Hodgkin’s lym-
interval (months) between SOT and PTLD diagnosis was longer in phoma leads to an increase in toxicity in this vulnerable population,
EBV-negative PTLD (105) than EBV-positive PTLD (28.5) (p<0.02). which limits our therapeutic choices.
EBV-negative PTLD seemed more frequent in the gastrointestinal tract
(42.9%) than EBV-positive PTLD (24.4%) (p =0.18), while other sites
were similarly distributed between the two groups. The distribution EP120 / #1722 CLINICAL FEATURES AND OUTCOMES OF
of morphologic subtypes did not differ overall between EBV positive ADOLESCENTS AND YOUNG ADULTS WITH B-CELL
and negative PTLD, but those with plasmacytic/plasmablastic features NON-HODGKIN LYMPHOMA MANAGED IN AN
were more likely to be EBV-negative PTLD (33.3% v. 9.3%, p<0.04). UNDERPRIVILEGED SETTING: A MULTICENTER COHORT STUDY
Expression of CD30, CD20, or CD138 by immunohistochemistry did
not differ by tumor EBV status. Ligia Rios1 , Denisse Castro2 , Daniel Enriquez3 , Jacqueline Montoya
Conclusions: Our single institution report suggests that EBV- Vasquez4 , Arturo Zapata4 , Eddy Hernandez4 , Thanya Runciman5 ,
negative PTLD exhibits distinct characteristics that differ from Esmeralda Leon6 , Bryan Valcarcel7
EBV-positive PTLD, especially in time from SOT to development of 1 Hospital Edgardo Rebagliati Martins, Pediatric Oncology, Lima, Peru;
PTLD. Cooperative group research can help guide treatment for EBV- 2 Hospital Nacional Edgardo Rebagliati, Oncology, lima, Peru; 3 Instituto
negative PTLD, particularly in those with plasmacytic/plasmablastic Nacional de Enfermedades Neoplásicas, Oncology, lima, Peru; 4 Instituto
features. Nacional de Enfermedades Neoplásicas, Pediatric Oncology, Lima, Peru;
5 Hospital Nacional Guillermo Almenara, Oncology, Lima, Peru; 6 Hospital
Nacional Guillermo Almenara, Pediatric Oncology, Lima, Peru; 7 The George
EP119 / #780 REFRACTORY OR RELAPSED HODGKIN’S Washington University, Milken Institute School Of Public Health, Washing-
LYMPHOMA IN CHILDREN AND YOUNG ADULTS ton, United States of America
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S204 of S635 ABSTRACTS

Background and Aims: There is a paucity of real-world data on out- ABVD (Doxorubicin-Bleomycin-Vinblastine-Dacarbazine) in patients
comes in adolescents and young adults (AYAs). We aimed to describe with R/P HL.
the clinical characteristics and survival outcomes of AYA treated in a Methods: This retrospective study included 132 pediatric patients
resource-constricted setting. with R/P HL (median age 13.9 years) treated from July-2012 to
Methods: We performed a multicenter retrospective cohort study on December-2020 with GV (n=50) or ICE (n=82). Patients were eval-
patients aged 15-39 years with a diagnosis of diffuse large B-cell lym- uated for RR to second-line, toxicities, factors predicting response at
phoma (DLBCL) or Burkitt lymphoma (BL) from 2008 to 2018. The R/P, overall survival (OS), and event free survival (EFS).
Kaplan-Meier method and long-rank test were used to evaluate overall Results: The RR was 54.3% to ICE and 28.6% to GV (P=0.004). Fac-
survival (OS) and progression-free survival (PFS). tors predicting poor RR were a higher stage, progressive disease
Results: We identified a total of 95 patients, 90 (95%) with DLBCL (PD) during first-line or early relapse, female sex, age ≥13 years, B-
and 5 (5%) with BL. The median age of all patients was 33 years symptoms, ESR >50 mm/h, WBC ≥13.5x109 /L, hemoglobin <10.5
(range, 15-39). Of all patients, 54% had a good performance sta- gm/dL. On multivariate analysis, only PD during first-line or early
tus[BsVv1], and 62% presented with early-stage cancer at diagnosis. relapse (P=0.002), and B-symptoms (P=0.002) were independent fac-
RCHOP-like (88%) was the most frequent frontline regimen[BsVv2], tors to poor RR. Treatment-related mortalities were 2.4% for ICE and
only 2% received a pediatric regimen. The overall response rate was 2% for GV (P=0.86). Of the 380 ICE and 235 GV cycles given, grade-
89%[BsVv3] (23% partial response and 66% complete response), the 3/4 neutropenia, grade-3/4 thrombocytopenia, and grade-3 anemia
recurrence/progression was observed in 31% of patients. Second-line were seen more frequent in ICE (98.9%, 83.9%, and 71% respectively)
chemotherapy was given to 28% and 5% had a stem cell transplant. than GV regimen (18.7%, 4.6%, and 8% respectively) (P<0.00001).
With a median follow-up of 49 months (95% CI 29-60), the 5-year OS Fever neutropenia necessitating hospital admission occurred in 55.5%
and PFS were 78% and 67%, respectively. of ICE cycles compared with 2.1% of GV (P<0.00001) and clinically
Conclusions: This study report that AYAs with B-cell NHL mostly documented infections in 17.9% vs 2.1%, respectively (P<0.00001).
present with early-stage cancer at cancer diagnosis and had high treat- Grade-3 hypokalemia, hypomagnesemia, hemorrhagic cystitis, blood-
ment response rates. Despite the limitations to providing cancer care in stream infection, and septic shock were reported in 29.5%, 26.8%,
Peru, we observed similar survival outcomes to those reported in high- 8.1%, 5%, and 5.8% of ICE cycles, but none in GV. The 3-year OS was
income countries. Efforts should be made to increase the availability 69.3±10.6% for ICE and 74±12.9% for GV (P=0.3); while the 3-year
of novel cancer-directed therapy and ensure treatment compliance EFS was 39.3±11.4% and 24.9± 12.5%; respectively (P=0.001).
in AYAs to further improve the survival outcomes in resource-limited Conclusions: ICE regimen had a better RR and EFS, but a higher toxicity
populations. profile than GV. However, toxic mortality was similar for both regimens.

EP121 / #758 GEMCITABINE AND VINORELBINE (GV) EP122 / #966 REDUCING THE BURDEN OF ONCOLOGY
VERSUS IFOSFAMIDE, CARBOPLATIN, ETOPOSIDE (ICE) CHEMORADIOTHERAPY AND RADIATION EXPOSURE FROM
REGIMEN IN TREATING PEDIATRIC PATIENTS WITH DIAGNOSTIC IMAGING BY UTILIZING TARGETED
RECURRENT OR PROGRESSIVE HODGKIN’S LYMPHOMA IMMUNOTHERAPY IN CHILDREN, ADOLESCENTS AND YOUNG
ADULTS WITH HODGKIN LYMPHOMA
Ahmed Mahdy1 , Asmaa Hamoda2 , Ahmed Zaher3 , Eman Khorshed4 ,
Madeha Elwakeel5 , Omneya Hassanein6 , Iman Sidhom7 Ana Xavier1 , Jessica Hochberg2 , Anthony Audino3 , Matthew Barth4 ,
1 Children’s Cancer Hospital Egypt 57357, Pediatric Oncology, Cairo, Egypt; Rodney Miles5 , Samir Kahwash6 , Stephan Voss7 , Suzanne Braniecki2 ,
2 National Cancer Institute - Cairo University, Egypt/ Children’s Cancer hos- Chitti Moorthy8 , Saro Armenian9 , Matthew Ehrhardt10 , Megan Lim11 ,
pital 57357, Cairo, Egypt, Pediatric Oncology, Cairo, Egypt; 3 National Can- Lauren Harrison2 , Stanton Goldman12 , Mitchell Cairo2
cer Institute - Cairo University, Egypt/ Children’s Cancer hospital 57357, 1 University of Alabama at Birmingham, Pediatrics, Birmingham, United
Cairo, Egypt, Nuclear Medicine, Cairo, Egypt; 4 National Cancer Institute - States of America; 2 New York Medical College, Pediatrics, Hawthorne,
Cairo University, Egypt/ Children’s Cancer hospital 57357, Cairo, Egypt, Sur- United States of America; 3 Nationwide Children’s Hospital/The Ohio State
gical Pathology, Cairo, Egypt; 5 National Cancer Institute - Cairo University, University, Pediatrics, Columbus, United States of America; 4 Roswell Park
Egypt/ Children’s Cancer hospital 57357, Cairo, Egypt, Radiology, Cairo, Comprehensive Cancer Center, Pediatrics, Buffalo, United States of America;
Egypt; 6 Children’s Cancer hospital Egypt 57357, Clinical Research, Cairo, 5 University of Utah, Pathology, Salt Lake City, United States of Amer-
Egypt; 7 National Cancer Institute - Cairo University/Children’s Cancer ica; 6 Nationwide Children’s Hospital/The Ohio State University, Pathology,
hospital Egypt 57357, Pediatric Oncology, Cairo, Egypt Columbus, United States of America; 7 Boston Children’s Hospital/Harvard
Medical School, Radiology, Boston, United States of America; 8 Westchester
Background and Aims: Pediatric Hodgkin lymphoma (HL) is a curable Medical Center, Radiation Medicine, Valhalla, United States of America;
disease; however, at relapse or progression (R/P) the optimal salvage 9 City of Hope National Medical Center, Population Sciences, Duarte, United

regimen is unclear. This study aimed to compare the response rate States of America; 10 St. Jude Children’s Research Hospital, Oncology, Mem-
(RR), toxicities, and outcome of GV with ICE regimen after first-line phis, United States of America; 11 University of Pennsylvania, Pathology
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S205 of S635

And Laboratory Medicine, Philadelphia, United States of America; 12 Texas nosed paediatric NHL. 2) Evaluate prognostic value of BMI detected by
Oncology-Medical City Dallas, Pediatrics, Dallas, United States of America PET/CT
Methods: 55 consecutive, newly diagnosed treatmentnaive NHL
Background and Aims: Significant chronic health conditions contin- patients (68% boys) who underwent baseline PET/CT and BMB
ues to increase over time among pediatric, adolescent, and young recruited. FDG uptake evaluated qualitatively on fivepoint scale (0-
adult (CAYA) classical Hodgkin lymphoma (cHL) survivors. Targeting 4) scores 3/4 were taken positive for BMI. Results compared by two
the tumor microenvironment (TME) and tumor-specific antigens is blinded NMphysicians. Groups: True positive: 1) Focal uptake matches
emerging as effective and safe treatments for cHL patients. Recently, site of positive BMB 2) No obvious CTchanges indicating alternative
we completed a phase 2 trial evaluating the use of antibody-drug conju- underlying pathology 3) Followup scan demonstrates disappearance of
gate targeting CD30 (brentuximab vedotin, Bv) and regulatory B-cells lesion post treatment. False positive: Positive scan not under above
(rituximab, RTX) to risk-adapted chemotherapy in newly diagnosed 3 criteria. True negative: Negative scan matches negative BMB. False
cHL CAYA patients. The combination was safe and resulted in signifi- negative: Negative scan mismatched with positiveBMB. Prognostic
cant reduction to toxic chemotherapy and radiation therapy (RT), while value of BMI demonstrated on PET/CT evaluated by OS/ 3-year PFS.
keeping superior outcomes (5-year OS/EFS 100%; Hochberg/Cairo, Results: Sensitivity and specificity of PET/CT in detecting BMI were
JITC 2022). Adding the checkpoint inhibitor nivolumab to chemoim- 95%, and 100% respectively. BMB had similar specificity however the
munotherapy with RTX + Bv may allow further anthracycline dose sensitivity was 24%. PET/CT demonstrated higher accuracy of 99%
reduction and RT in intermediate-/high-risk cHL in CAYA. versus 81% of BMB. The negative predictive value of PET/CT scan was
Methods: This is a multicenter study for patients with intermediate- higher (98%) than BMB. BMI showed a strong correlation with PFS of
and high-risk cHL. Intermediate-risk cHL patients receive 2 cycles 86% vs 97%; and OS of 83% vs 100% (p value<0.05 ) demonstrated in
of Bv, doxorubicin, vinblastine, dacarbazine, and RTX (Bv-AVD-R). PET/CT positive and negative cases.
Rapid early responders (RER) or slow early responders (SER) by Conclusions: 18 F-FDG PET-CT demonstrated higher diagnostic accu-
FDG-PET scan receive 2 or 4 cycles of Bv, vinblastine, dacarbazine, racy than BMB. BMB maybe avoided in aggressive NHL, wherein
nivolumab, and RTX (Bv-NVD-R), respectively. High-risk cHL patients PET/CT scan has high sensitivity and specificity. In indolent NHL, how-
receive 2 cycles of Bv-AVD-R. RERs by FDG-PET scan receive 4 ever BMB has a higher diagnostic value. We suggest that PET/CT be
cycles of Bv-NVD-R; SERs receive 2 cycles of Bv, nivolumab, dox- used as complementary tool to BMB for BMI evaluation.
orubicin, vinblastine, dacarbazine and RTX (Bv-NAVD-R), followed by
4 cycles of Bv-NVD-R. RT will be given to SER patients not achiev-
ing CR. Patients age ≥ 3 and ≤ 39 years will be enrolled with a E-Poster Topic: AS05 SIOP Scientific programme / AS05.d Stem Cell
primary objective to assess safety/feasibility of adding nivolumab Transplantation
to chemoimmunotherapy with RTX + Bv in intermediate-/high-risk
cHL. E-POSTER VIEWING
Results: Two patients have been enrolled to date and have had no DLT.
Accrual is ongoing. Additional results will be presented at the meeting EP124 / #625 OPTIMIZING CYCLOSPORINE (CSA) DOSE
Conclusions: Targeting the TME (regulatory B-cells) and PD1/PD-L1 POST ALLOGENEIC HEMATOPOIETIC STEM CELL
axis is a promising approach in CAYA with cHL. (NCT05253495). TRANSPLANTATION IN PEDIATRIC CANCER PATIENTS

Mennatallah Elnaggar1 , Hanafy Hafez2 , Amr Abdallah2 , Mahmoud


EP123 / #1412 EVALUATION OF BONE MARROW Hamza3 , Marwa Khalaf4 , Alaa El-Haddad2
INVOLVEMENT IN PAEDIATRIC NON-HODGKIN LYMPHOMA: 1 Children Cancer Hospital Egypt 57357, Clinical Pharmacy- Bone Marrow
18F-FDG PET/CT VERSUS BONE MARROW BIOPSY Transplantation, Cairo, Egypt; 2 National Cancer Institute - Cairo University,
Children’s Cancer hospital Egypt 57357, Pediatric Oncology, Cairo, Egypt;
Surekha Yadav 3 Children Cancer hospital Egypt 57357, Clinical Research, Cairo, Egypt;
army hospital R&R, subroto park, Department Of Nuclear Medicine, delhi 4 Benisuif-University, Pharmacology, Benisuif, Egypt

cantonment, India
Background and Aims: Cyclosporine A (CSA) dosing has been com-
Background and Aims: Non-Hodgkin lymphoma (NHL), fourth most plicated by considerable intra-patient and inter-patient variability in
common malignancy in chilren is characterized by high propensity to pharmacokinetics, which is affected by different factors such as patient
bonemarrow involvement (BMI). Accurate evaluation of BMI is essen- age, and concurrent drug therapy. This study assessed various factors
tial for staging and affects management, treatment and prognosis. Its that might affect CSA dose and subsequent trough plasma level.
an indicator of infusion-related reaction with rituximab. Bone marrow Methods: A retrospective study included pediatric cancer patients who
biopsy (BMB) explores limited bonemarrow; potentially missing focal underwent Allogeneic Hematopoietic Stem Cell Transplant at the Chil-
involvement and is invasive. Aims: 1) Evaluate the diagnostic accuracy dren’s Cancer Hospital Egypt 57357 from October 2012 to August
of 18 F-FDG PET/CT in detecting BMI compared to BMB in newly diag- 2016 from matched related donors(MRD) with CSA as part of their
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S206 of S635 ABSTRACTS

GVHD prophylaxis regimen. CSA initial dose was 1.5mg/kg every 12 transplant and 92% used enteral nutrition first-line. Enteral and par-
hours and then titrated according to the level and drug toxicity. The enteral nutrition were initiated under similar criteria. Forty-two per-
desired level was between 200 to 250 ng/ml. cent of centers used gastrostomies in children with poor nutritional
Results: A total of 119 patients were included during the study period, status prior to admission or likely to refuse a nasogastric tube. In
with a median age of 9.9 years. Fluconazole as a prophylactic antifun- those not using them, 76% of clinicians felt some children should be
gal was used in 54.6% of the patients while voriconazole in 43% of offered a gastrostomy. Clinicians perceived less displacements (78%)
them. Female patients and those who are older than 9 years old or and cosmetic appearance (69%) as the most common advantages
received concomitant voriconazole had reached the target CSA level of gastrostomies over nasogastric tubes. Risk of surgery (92%) and
earlier with low initial doses. Also, those who received voriconazole tube/stoma complications (58%) were the most common perceived
had delayed CSA clearance and required lower CSA doses compared to gastrostomy problems.
other antifungal agents. A higher probability (above 90%) to reach the Conclusions: Centers employ proactive and similar approaches to
desired plasma level with doses ≤1.5mg/kg BID for those who are >9 nutritional counselling, screening, assessment and interventions. Gas-
years old and on voriconazole. Younger patients ≤9 years and on non- trostomy use divided practice and opinion with differences in use
voriconazole antifungal required a CSA dose of more than 1.5 mg/kg and perceived advantages, but agreement on potential complications.
BID with a probability to reach the desired plasma level of around 80%. Despite their risks clinicians wanted to utilize gastrostomies more
CSA toxicity was reported in 21% of the patients. in clinical practice. Placement requires careful consideration of the
Conclusions: Initial CSA dose individualization should consider patient risks, benefits and family preferences. Future research needs to inves-
age and concomitant antifungal used. Patients who are > 9 years old or tigate complications, outcomes and family experiences of gastrostomy
receive concomitant voriconazole require initial lower doses of CSA. feeding in these children.

EP125 / #50 NUTRITION SUPPORT PRACTICES AND EP126 / #707 EXPERIENCE OF PEDIATRIC HAPLOIDENTICAL
OPINIONS TOWARD GASTROSTOMY USE IN PEDIATRIC BONE STEM CELL TRANSPLANTATION- REAL- WORLD DATA FROM A
MARROW TRANSPLANT CENTERS: A NATIONAL SURVEY TERTIARY CARE CENTRE

James Evans1 , Dan Green2 , Graeme O’Connor1 , Julie Lanigan3 , Faith Akanksha Garg1 , Kamlesh Shah1 , Kinnari Patel1 , Ambika Vachhani1 ,
Gibson4,5 Hardikkumar Solanki1 , Apurva Patel2 , Harsha Panchal2 , Sandip Shah1
1 Great Ormond Street Hospital for Children, Dietetics, London, United 1 Gujarat Cancer and Research Institute, Medical Oncology, Ahmedabad,
Kingdom; 2 University of Sheffield, School Of Health And Related Research India; 2 Gujarat Cancer and Research Institute, Medical Oncology (pediatric
(scharr), Sheffield, United Kingdom; 3 University College London, Great Oncology), Ahmedabad, India
Ormond Street Institute Of Child Health, London, United Kingdom; 4 Great
Ormond Street Hospital for Children, Centre For Outcomes And Experience Background and Aims: With the success of post-transplant cyclophos-
Research In Children’s Health, Illness And Disability (orchid), London, United phamide based platform and improved clinical care, the number of
Kingdom; 5 University of Surrey, School Of Health Sciences, Guildford, United haploidentical stem cell transplants (HaploSCT) have surged over the
Kingdom last decade. However, data from low-middle income countries (LMIC)
is scarce. We aimed to evaluate the outcome of haploSCT in children
Background and Aims: Previous surveys have shown deviations in and adolescent population (<18 years) at our centre.
nutritional practices from international guidelines during bone marrow Methods: We conducted a retrospective analysis of the haploSCT
transplant (BMT). Guidelines recommend enteral nutrition first-line performed between January 2015 and February 2022. The graft
and nasogastric tubes are the mainstay for its provision. Gastros- versus host disease (GVHD) prophylaxis was post-transplant
tomies provide an alternative, yet their use is less common. This survey Cyclophosphamide (PTCy) with Mycophenolate-mofetil and
aimed to investigate nutrition support practices in United Kingdom Cyclosporine/tacrolimus. All patients were transfused peripheral
pediatric allogeneic BMT centers and compare clinicians’ opinions on blood stem cells from donors. Supportive care was given as per unit
gastrostomy use. protocol. Post-transplant, patients continued regular follow up. Overall
Methods: An online survey was administered to all 12 centers. One survival (OS) was calculated using the Kaplan–Meier method.
response was requested from the lead BMT dietician, clinical nurse Results: Twenty-five patients underwent haploSCT. Median age was
specialist and consultant in each center. Questions concerning gas- 11 Years (range 3-18 years). Benign hematological disorders included
trostomies were answered by all clinicians. In addition, the dietician aplastic anemia (n=3), Inherited bone marrow failure syndromes
answered questions regarding nutritional practices. (n=4), and primary immunodeficiency in one case. Malignant disorders
Results: A 100% response rate was achieved from 12 centers (36/36 included relapsed acute lymphoblastic leukemia (n= 9), relapsed acute
clinicians). Nutritional counselling was provided in 92% of centers myeloid leukemia (n=6), myelodysplastic syndrome (n=1) and hodgkin
before and routinely throughout admission, 83% screened on and reg- disease (n=1). Sibling donors were used in 10 patients, and parents
ularly throughout admission, 83% assessed nutritional status before in 15. Most common conditioning regimen used was Fludarabine +
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S207 of S635

Busulphan based (n=19, 76%). Engraftment rates were 80% (n=20) ies will explore the effect of cardiorespiratory fitness and the level of
with acute graft versus host disease in 24% (n= 6) and cytomegalovirus activity during HCT admission and effect on patients’outcomes.
(CMV) reactivation in 60% (n=15) patients. Hemorrhagic cystitis was
seen in 64% (n=16). Non-relapse mortality was 36% (n=9). Relapse
related mortality was 24%. The median follow-up was 12 months and EP128 / #315 HAPLOIDENTICAL HEMATOPOIETIC STEM
OS was 40%. Nine out of 25 patients are alive and on regular follow-up. CELL TRANSPLANTATION WITH ANTITHYMOCYTE GLOBULIN
Conclusions: HaploSCT with a PTCy platform is a cost-effective, AND LOW DOSE POST-TRANSPLANT CYCLOPHOSPHAMIDE; A
promising modality of treatment in children and adolescents who SINGLE-CENTER RETROSPECTIVE STUDY
don’t have matched sibling or matched unrelated donors. However,
long-term outcomes in LMICs remain poor. CMV reactivation, hem- Hee Young Ju1 , Hee Won Cho1 , Ji Won Lee1 , Ki Woong Sung1 , Hong
orrhagic cystitis and multi-drug resistant bacterial infections remain a Hoe Koo1 , Hye Lim Jung2 , Keon Hee Yoo3
challenge. 1 Samsung Medical Center, Department Of Pediatrics, Seoul, Korea, Repub-
lic of; 2 Kangbuk Samsung Hospital, Department Of Pediatrics, Seoul,
Korea, Republic of; 3 Sungkyunkwan University, Samsung Medical Center,
EP127 / #876 FUNCTIONAL STATUS AND OUTCOMES IN Department Of Pediatrics, Seoul, Korea, Republic of
PEDIATRIC AND YOUNG ADULT PATIENTS UNDERGOING
HEMATOPOIETIC CELL TRANSPLANT Background and Aims: Hematopoietic stem cell transplantation from
a haploidentical donor (haplo-HCT) are increasingly used in patients
Anne Gonzales1 , Rachel Bican1 , Joseph Stanek2 , Rolla Abu-Arja2 lacking a matched donor, but the optimal strategy needs to be defined.
1 Nationwide Children’s Hospital, Division Of Clinical Therapies, Colum- This study aims to review the outcome of haplo-HCT with antithy-
bus, United States of America; 2 Nationwide Children’s Hospital, Division mocyte globulin with low dose post-transplant cyclophosphamide
Of Hematology, Oncology And Bone Marrow Transplantation, Columbus, (ATG/LD-PTCy) in a single center for pediatric patients.
United States of America Methods: A retrospective analysis was done on patients who under-
went haplo-HCT with ATG/LD-PTCy at Samsung Medical Center
Background and Aims: Pediatric and young adult patients undergoing between 2019 and 2021. ATG was administered 2 mg/kg/day for 2 days
hematopoietic cell transplantation (HCT) often experience decreased (d-7, d-6) and cyclophosphamide 14.5 mg/kg/day was given for 2 days
functional mobility and deconditioning. The benefits of functional sta- (d-3, d-2). As for PTCy, cyclophosphamide 25 mg/kg/day was admin-
tus on outcomes in patients undergoing HCT is established in the istered at d3 and d4, and tacrolimus with mycophenolate mofetil was
literature. Physical therapy intervention can directly address the phys- given from d5 for graft-versus-host disease (GVHD) prophylaxis.
ical sequelae effects of HCT that include functional mobility, functional Results: Total of fourteen patients (7 male) underwent haplo-HCT
strength and endurance. Transplant Energize Me Patient Outcome with ATG/LD-PTCy. The most common diagnosis was acute myeloid
(TEMPO©) is a multidisciplinary therapy standard of care that aims leukemia (AML; n=4), followed by acute lymphoblastic leukemia (ALL;
to optimize patient outcomes in these areas. In TEMPO©, the novel n=3). Neutrophil and platelet engraftment was achieved at a median
Functional Mobility Score (FMS©) is an objective outcome measure of 16 and 37 days in 13 patients. One patient with engraftment fail-
completed by physical therapists. The Karnofsky Performance Status ure underwent 2nd haplo-HCT, but ALL relapsed at 3 months despite
Scale and Lansky Play Performance Scale (K/L) are gold standards for successful engraftment. Two patients with AML relapsed within 4
this population and are subjective in measure. We hypothesize that a months. In patients with successful engraftment, the natural killer cells
patient’s functional status, measured by FMS© and K/L, at admission recovered most early, followed by B cell recovery. The overall and
and discharge will have an inverse relationship on outcomes such as relapse-free survival rates were 83.3% and 74.1% at 6 months, and
length of stay (LOS) and time to engraftment. 71.4% and 74.1% at 12 months, respectively, with a median follow-up
Methods: A retrospective chart review included patients with data of 8.7 months. Acute GVHD occurred in 11 patients, of which 10 were
on functional status at HCT admission and discharge as well as infor- grade I or II.
mation on outcomes. Data were summarized descriptively. Spearman Conclusions: Haplo-HCT with ATG/LD-PTCy could achieve engraft-
correlation coefficients were used to estimate the correlation between ment in a high proportion of patients, and disease-free status was
FMS© and K/L performance scores and outcomes. maintained except for early relapse cases. Based on these findings,
Results: A total of 37 patients were included in this study. Patients ATG/LD-PTCy might be a feasible option for patients undergoing
were 57% male and had median age at HCT of 12 years. There was a haplo-HCT.
mild negative correlation between FMS© at discharge and length of
stay (rs =-0.40, p=0.0156).
Conclusions: The number of patients in this small study presents a lim- EP129 / #434 IS MIXED T-CELL CHIMERISM PREDICTIVE OF
itation. This exploratory study identifies that these metrics alone are RELAPSE IN PEDIATRIC ACUTE LYMPHOBLASTIC LEUKEMIA
not robust enough to evaluate functional status and the multifactorial AFTER ALLOGENEIC STEM CELL TRANSPLANTATION?
relationship of function to LOS and time to engraftment. Future stud-
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S208 of S635 ABSTRACTS

Saadiya Khan, Zainab Alsaif, Khawar Siddiqi, Abdullah Al-Jefri, Ali (AML) post hematopoietic stem cell transplant (HSCT) to investigate
Alahmari, Awatif Alanazi, Hawazen Alsaedi, Mouhab Ayas, Ibrahim its significance as a predictor of relapse of primary disease and overall
Ghemlas survival.
King Faisal Specialist Hospital & Research Center, Pediatric Oncology, Methods: Forty-one pediatric patients with AML underwent allogeneic
Riyadh, Saudi Arabia HSCT from 2012-2017 at our institution. Twenty-eight fared beyond
Day+120 and are the focus of this report. Mixed T-cell lymphocyte
Background and Aims: Chimerism studies post hematopoietic stem Chimerism (MC) was defined as >1.0% of recipient’s DNA spiking any
cell transplant (HSCT) are dynamic. The aim of this study was to review time during post-HSCT follow-up at or beyond Day+30.
our data on presence of recipient cells in post-HSCT pediatric patients Results: Median age at transplant was 6.9 years (range, 0.1-13.9),
with acute lymphoblastic leukemia (ALL) to study its significance as a 14(50.0%) were boys. For 20 (71.4%) bone marrow (BM) was the
predictor of relapse of their primary disease. source of stem cells, 1 (3.6%) peripheral blood and for 7 (25.0%) cord
Methods: Forty-six transplant naïve pediatric patients with ALL who blood. All patients received a myeloablative conditioning regimen with
underwent allogeneic HSCT from 2012-2017 at our institution were GvHD prophylaxis. Donor was a full matched relative in 16 (57.1%) and
analyzed. Thirty-seven lived beyond Day+120 and are evaluated Haploidentical in 5 (17.9%) while unrelated in the remaining 7 (25%)
herein. Mixed T-cell Chimerism (MC) was defined as >1.0% of recip- recipients transplanted with CB. Rate of post-HSCT relapse of primary
ient’s DNA spiking any time during post-HSCT follow-up beyond disease or progression was 7.1% (n=2) with a median time to relapse
Day+30. of 6.7 months from transplant. Rate of MC post-HSCT from Day+30
Results: Median age at transplant was 8.8 years (range, 0.5-14.0), till Day+100 was 57.1% (n=16), Day+100 to Day+1825 40.0% (n=10
19(51.4%) were boys. Two(6.1%) had BCR/ABL and 3(9.1%) had MLL of 25 evaluable) and day+30 through Day+1825 was 64.3% (n=18 of
gene re-arrangement out of the 33 patients followed till Day+720. 28). With a median follow-up of 58.4 months (95% CI: 48.2-68.5) and
Bone marrow (BM) was the stem cell source in 28(75.7%), peripheral mortality rate of 21.4% (n=6 of 28), cumulative probability of overall
blood in 1(2.7%) and cord blood for 8(21.6%) patients. All patients survival of our cohort at 5 year was 77.5%±8.1%. MC during D+100,
received myeloablative conditioning regimen with cyclosporine and D+100 to Day+1825 or throughout till Day+1825 was not signifi-
methotrexate as graft versus host disease (GVHD) prophylaxis. Post- cantly associated with relapse of primary disease, mortality or overall
HSCT relapse rate of primary disease was 51.4% (n=19) with a median survival.
time to relapse of 6.7 months from transplant. Relapse sites were blood Conclusions: MC was not found to be a significantly associated with
in 13 (68.4%), CNS in 5 (26.3%) and bone marrow in 1 (5.3%). Post post-HSCT relapse of primary, mortality rate or overall survival in
HSCT overall rate of MC till Day+720 was 51.4% (19 of 37). MC till pediatric AML patients.
Day+100 was seen in 45.9% (17 of 37) and beyond Day+100 in 12.9%
(4 of 31 evaluable) patients. MC during D+100, D+100 to Day+720 or
throughout till Day+720 was not significantly associated with relapse EP131 / #1179 OUTCOME OF INFANT YOUNGER THAN 1
of primary disease (relapse rate: 11, 57.9%, 9, 52.9% and 3, 75%; YEAR WITH ACUTE LYMPHOBLASTIC OR MYELOID LEUKEMIA
P=0.517, 1.00 and 0.284 respectively). FOLLOWING INTENSIVE CHEMOTHERAPY AND
Conclusions: Chimerism analysis is an essential tool for post-HSCT HEMATOPOIETIC STEM CELL TRANSPLANTATION
follow-up of engraftment. MC was not observed to be a significant
predictor of post-HSCT relapse of primary disease in our patient Bo Kyung Kim, Eun Sun Song, Jung Yoon Choi, Kyung Taek Hong, Hong
cohort. Yul An, Hyun Jin Park, Hyoung Jin Kang
Seoul National University College of Medicine, Pediatrics, Seoul, Korea,
Republic of
EP130 / #1248 MIXED T-CELL LYMPHOCYTE CHIMERISM IN
PEDIATRIC ACUTE MYELOID LEUKEMIA AFTER ALLOGENEIC Background and Aims: Infant leukemia is rare aggressive disease
HEMATOPOIETIC TRANSPLANT and has poor prognosis. And, neurocognitive dysfunction is an impor-
tant long-term problem after transplantation of children. We reported
Saadiya Khan, Ibrahim Jaffari, Khawar Siddiqi, Abdullah Al-Jefri, Ali the characteristics and outcomes including neurocognitive problem of
Alahmari, Awatif Alanazi, Hawazen Alsaedi, Ibrahim Ghemlas, Mouhab infant acute lymphoblastic leukemia (ALL) and acute myeloid leukemia
Ayas (AML) with intensive chemotherapy and hematopoietic stem cell
King Faisal Specialist Hospital & Research Center, Pediatric Oncology, transplantation (HSCT).
Riyadh, Saudi Arabia Methods: We present the results of 20 patients underwent allogeneic
HSCT with infant ALL and AML at Seoul National University Chil-
Background and Aims: It is importance to identify the origin of dren’s Hospital from 1995 to 2020. All patients used busulfan-based
the newly developing hematopoietic system in patients undergoing myeloablative contioning regimen. Patients who visited a psychiatric
transplant for malignancies. We reviewed our data on mixed t-cell outpatient clinic after transplantation were considered patients with
chimerism (MC) in pediatric patients with acute myeloid leukemia predisposition of neurocognitive problems.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S209 of S635

Results: In all 20 patients, the median age at diagnosis and HSCT avoidance of unpasteurised dairy products (94%, P=0.50), raw or
was 7.2 months (range, 2.8-11.1 months) and 14.5 months (range, 7.5- undercooked meat (94%, P=0.34), unpasteurised patê (88%, P=0.39),
22.4 months). The patients included 16 ALL (80%) and 4 AML (20%). raw shellfish (94%, P=0.45), probiotics (75%, P=0.42). A lack of consis-
Eight patients received HSCT from unrelated peripheral blood stem tency in advice around the water source used on wards, vacuum packed
cell (PBSC), 7 from unrelated cord blood, 2 from haploidentical PBSC, meats and fish and unpeeled fruits and vegetables was apparent. There
and others from related bone marrow, unrelated bone marrow, and was great variation between which guidelines were being referenced
related PBSC. There was no engraftment failure. The cumulative inci- at each centre, with trust own guidance being the most common (31%).
dence of acute graft-versus-host disease (GVHD) with grade II-IV was Conclusions: Food safety guidance for neutropenic patients differs
31% and those with grade III-IV was 15.6%. Chronic GVHD appeared greatly across UK centres, with some practices seeming outdated and
in only one case with moderate severity. The 5-year overall survival non-evidence based. A national review of food safety guidance needs to
(OS) and event-free survival rates (EFS) and treatment-related mor- be considered to provide a standardised approach for this population.
tality (TRM) were 84.4%, 83.3%, and 17.1%, respectively. Patients who
visited a psychiatric outpatient clinic after transplantation were 37.3%
(median 2.7 years after transplantation, range 2.0-9.1 years). EP133 / #1664 HEMATOPOIETIC STEM CELL
Conclusions: This study showed favorable outcomes of infant ALL and TRANSPLANTATION FOR CHILDREN IN JORDAN 2003-2021:
AML after intensive chemotherapy and HSCT during long period. And ACTIVITY AND TRENDS
it is considered necessary to follow-up the neurocognitive function in
patients who underwent intensive chemotherapy and HSCT at an early Rawad Rihani1 , Mayada Abu Shanap1 , Eman Khattab1 , Abdelghani
age. However, the number of patients was not large enough, further Tbakhi2 , Hasan Hashem1 , Iyad Sultan3
studies are needed for these high-risk patients. 1 King Hussein Cancer Center, Paediatric Hematology/oncology/bone Mar-
row Transplant, Amman, Jordan; 2 king Hussein cancer center, Department
Of Cell Therapy & Applied Genomics, Amman, Jordan; 3 king hussein cancer
EP132 / #561 A CROSS SECTIONAL SURVEY TO REVIEW center, Pediatric Oncology, Amman, Jordan
FOOD SAFETY PRACTICES WITHIN PAEDIATRIC ONCOLOGY
AND STEM CELL TRANSPLANT CENTRES IN THE UNITED Background and Aims: The first comprehensive pediatric HSCT pro-
KINGDOM gram in Amman-Jordan was established in 2003 at King Hussein
Cancer Center (KHCC).We describe activities and trends by Pediatric
Alisa Morris1 , Gemma Renshaw1 , Graeme O’Connor1,2 HSCT Program at KHCC.
1 Great Ormond Street Hospital for Children NHS Trust, Department Of Methods: Retrospective data review was conducted between 2003 to
Dietetics, London, United Kingdom; 2 UCL Institute of Child Health, Depart- October 2021.
ment Of Infections And Inflammation, London, United Kingdom Results: Between January, 2003 and October 2021, 1051 HSCTs were
performed with a median age of 8 years (0.13-31), of which 58%
Background and Aims: Neutropenia is a common complication of were males and 72 % were Jordanians (n=758). Median follow-up
chemotherapy, which poses a high risk of infection and mortality. was 2.9 years (0.087-12.5). Allografts accounted for 77% of HSCTs
Neutropenic diet has historically been recommended for those under- (n=811); whereas, autografts accounted for 23 %(n=240). Allogeneic
going high dose chemotherapy (HDC) for malignancy or stem cell HSCTs included full-matched family/related donors in 79%(n=641),
transplants (SCTs). The rationale behind the diet is to reduce risk of haploidentical in 16%(n=132) and unrelated donors in 5%(n=38). Stem
foodborne infection by avoiding foods considered to be high micro- cell sources included PBSCs in 75%(n=788), BM 21%(n=221) and
bial risk. However, evidence for this diet is limited and there is lack of CBUs 4% (n=38) of the HSCTs. Myeloablative and reduced intensity
national consensus guidelines. Aim: To establish what food safety guid- conditioning were employed in 71 % and 27% of HSCTs, respectively;
ance is being implemented across centres in the United Kingdom (UK) and no conditioning in 2%. Malignant conditions were the main indica-
providing paediatric SCTs or HDC. tions for HSCTs (56%;n=591), whereas, non-malignant accounted for
Methods: Twenty two principal treatment centres (PTCs) and centres 44%(n=460). The most common indication for allogeneic HSCTs were
providing SCTs registered with the British Society of Blood and Mar- leukemia (37%;n=297), followed by hemogloinopathies (26%;n=212),
row Transplantation and Cellular Therapy or the Children’s Cancer bone marrow failures in 17%(n=137) and immune deficiencies in
and Leukemia Group were identified. Specialist dietitians employed 11%(n=88); while solid tumors (69%;n=165), followed by lymphomas
by these centres were contacted to complete an online question- (41%;n=99) were the main indications for autologous HSCTs. The
naire regarding food safety guidance implemented at their centres 5-year OS and EFS for all HSCTs were 81% ±2.3 and 75%±3.4, respec-
for paediatric patients undergoing HDC or SCT. Questions related to tively; and for allogeneic 84%±3.1 and 77%±2.2. Cumulative incidence
restricted foods, specific guidelines implemented, ward food provision of TRM at 1 year for allogeneic HSCTs was 1.9%, compared to 5% in
and timings of food provision. previous era (before 2010),p=0.004). Whereas, for autologous HSCT
Results: Responses received from 16/22 centres (73%). Many aspects was 0.54%. Disease progression/ relapse of underlying condition was
of the neutropenic diet were consistent across centres, including the main cause of mortality (76%).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S210 of S635 ABSTRACTS

Conclusions: Implementing contemporary practices, expertise of the ing approach to overcome poor graft performance following CB
treating teams and robust supportive care practices contributed to transplantation.
significant improvement in HSCTs outcomes at KHCC.A constant
increment in HSCTs numbers performed at KHCC is evident. In par-
ticular, haploidentical HSCTs are increasingly employed as a readily EP135 / #1259 PRETRANSPLANT MYELOID AND IMMUNE
available alternative donor option. SUPPRESSION (PMIS) FOR HIGH-RISK THALASSEMIA PATIENTS
RECEIVING MATCHED RELATED DONOR ALLOGRAFT

EP134 / #409 INTERFERON-GAMMA-PRIMED Eman Khattab, Rawad Rihani, Hasan Hashem, Mayada Abu Shanap,
MESENCHYMAL STEM CELLS ENHANCE THE ENGRAFTMENT Iyad Sultan
OF HUMAN CORD BLOOD HEMATOPOIETIC STEM CELLS IN king Hussein cancer center, Pediatric, Amman, Jordan
NSG MICE
Background and Aims: Patients with high risk thalassemia have a
Ju Yong Song1,2 , Byungchan Kim1,2 , Jeehyun Kim2 , Mihyang Jin2 , higher incidence of transplant related complications and graft fail-
Soyeon Shin3 , Keon Hee Yoo3 ure.The introduction of PMIS, mainly for alternative donor transplants,
1 Sungkyunkwan University, Department Of Health Sciences And Technol- is reported to be feasible and associated with good outcomes. We are
ogy, Saihst, Seoul, Korea, Republic of; 2 Samsung Medical Center, Depart- reporting our results applying PMIC in MRD transplants for high risk
ment Of Pediatrics, Seoul, Korea, Republic of; 3 Sungkyunkwan University thalassemia patients.
School of Medicine, Department Of Pediatrics, Seoul, Korea, Republic of Methods: This is a retrospective chart review of patients who received
PMIS for high risk thalassemia patients(age more than 14years or class
Background and Aims: Hematopoietic stem cell transplantation 3 thalassemia )receiving matched related donor allografts, according to
(HSCT) is a curative option for a variety of hematologic diseases. our new guidelines which were in effect since Oct2020. Accordingly,
Umbilical cord blood-derived HSCs (CB-HSCs) transplantation has these patients received fludarabine 200 mg/m2 intravenously (iv) over
shown relatively poor engraftment compared with other sources 5 days in combination with dexamethasone 25 mg/m2/day iv over 5
due to the limited cell numbers in a CB unit. Studies have demon- days, given as two cycles 3 weeks apart in association with hydrox-
strated that co-transplantation of mesenchymal stem cells (MSCs) with yurea, followed by fludarabine and busulfan based reduced toxicity
HSCs enhances the engraftment of HSCs. We aimed to investigate conditioning (RTC).
if interferon-gamma (IFN-γ)-primed MSCs could further promote the Results: Seven patients received PMIS since Oct2020.None of these
engraftment of human CB-HSCs than naïve MSCs. patients required admission after these infusions.Median age at trans-
Methods: MSCs derived from Wharton jelly with or without IFN- plant was 16.5 (range, 4-26.5 years), median ferritin 1020(440-7300
γ-priming were analyzed by single cell RNA-sequencing. Then, we ng/ml); median liver span15.9 (9.6-17cm);median absolute lymphocyte
transplanted 1 x 104 CB-CD34+ cells (Group A), CD34+ cells plus naïve count prior to transplant 500(range,200-1200).None of the patients
MSCs (Group B), and CD34+ cells plus IFN-γ-primed MSCs (Group C) in had significant liver fibrosis as confirmed by pretransplant biopsy. The
busulfan-conditioned NSG mice. At 6 weeks post-transplant, engraft- median time of neutrophil and platelets engraftment was on days
ment rates were compared among groups by measuring the percentage 13(range, 13-20) and 18(range, 15-22), respectively. After a median
of human CD45+ cells in bone marrow by flow cytometry. follow up of 192 days (range, 101-499), five patients have full donor
Results: Single cell RNA-sequencing revealed that 729 genes were chimerism (above 95% donor cells), and 2 have stable mixed chimerism.
up-regulated and 477 genes were down-regulated in IFN-γ- primed- There were no signs of acute or chronic graft vs host disease detected
MSCs. Gene Ontology analysis showed that most of those changes in any of our patients. Five patients developed molecular CMV reacti-
are related to external stimuli, defense responses and protein bind- vation but with no clinical manifestations. One patient developed BK
ings. The median percentage of human CD45+ cells in bone marrow of cystitis that was treated conservatively.
Groups A to C was not statistically different (3.54%, 3.61%, and 4.49%, Conclusions: Applying PMIS for high-risk thalassemia patients receiv-
respectively; P=NS). The highest engraftment rate achieved in each ing MRD allografts was not associated with significant toxicity.This
group was 7.58% (Group A), 13.20% (Group B), and 20.80% (Group C), approach may improve the results for this group of patients and we
respectively. will continue using PMIS for our high-risk transplants.Viral reactivation
Conclusions: This study demonstrated that gene expression pat- needs close monitoring.
terns of MSCs change upon IFN-γ-priming. With a limited number
of CD34+ cells, co-transplantation of IFN-γ-primed MSCs tended
to promote engraftment better than naïve MSCs. Additional stud- EP136 / #385 IMMUNE RECONSTITUTION AFTER
ies are needed to verify the engraftment promoting effect of IFN- HAEMATOPOIETIC STEM CELL TRANSPLANTATION IN
γ-primed MSCs by limiting CD34+ cell dose and optimizing con- CHILDREN WITH LEUKEMIA: A BRAZILIAN INSTITUTION
ditioning for engraftment. Our findings suggest co-transplantation EXPERIENCE
of IFN-γ-primed MSCs with CB could be a novel and promis-
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S211 of S635

Antonella Zanette, Andréia Wasem, Julia Dereti, Ana Luiza Rodrigues Department Of Medical Genetics And Cell Biology, Institute Of Biomedicine,
Hospital Erastinho, Bmt Unit, Curitiba, Brazil Göteborg, Sweden; 3 University of Gothenburg, Department Of Labora-
tory Medicine, Göteborg, Sweden; 4 Karolinska Institutet, Department Of
Background and Aims: Allogeneic hematopoietic stem cell trans- Oncology-pathology, Stockholm, Sweden
plantation (HSCT) is an established treatment for children with high
risk and relapsed leukemias, certain lymphomas, and various non- Background and Aims: Neuroblastoma is the most common extracra-
malignant diseases. Despite intensive efforts to control T cell and nial solid tumor in children, and the high-risk disease remains a ther-
natural killer (NK) cell engraftment and recovery, the accomplishment apeutic challenge. Neuroblastoma originates from neural crest cells
of optimal balance between acute graft-versus-host disease (aGVHD), and impairs neuronal differentiation during early embryonal develop-
chronic graft-versus-host disease, viral infections and graft-versus ment. Teneurins (TENM1-4) are cell adhesion molecules that are highly
leukemia/tumor effect remains a challenge. Increasing evidence sug- expressed during embryonal development and function in differentia-
gests that early and rapid lymphocyte recovery following (HSCT) is tion. We and others have identified somatic mutations and structural
associated with better survival. aberrations of TENM genes in tumors, including neuroblastoma. This
Methods: A retrospective analysis of patients diagnosed with acute study aims to identify the importance of TENM4 in neuroblastoma
leukemia who underwent HCT between August 2018 and June 2021, growth, tumorigenicity, and differentiation.
in the BMT Unit of Erastinho’s Hospital. We studied the dynamic anal- Methods: siRNA-mediated knock-down and RNA sequencing of
ysis of immune recovery with regard to potential factors affecting its TENM1-4 were performed in neuroblastoma cells to analyze the
speed, including age, type of HCT, diagnosis, graft-versus-host disease molecular effects of TENM1-4. CRISPR-Cas9-mediated knockouts of
(GvHD), and cytomegalovirus (CMV) infection reactivation. Absolute TENM4 (TENM4-/- ) in SK-N-BE(2) cells were evaluated for detailed
counts of different lymphocyte subsets and immunoglobulin serum molecular changes, proliferation, differentiation, and tumorigenicity.
levels were determined in peripheral blood of patients. Results: siRNA-mediated knockdown of TENM4 significantly
Results: 20 patients were included, 5F/15M, age 1-19 years old (M- decreased proliferation in all investigated neuroblastoma cell lines.
10). They all underwent a myeloablative regimen; haploidentical donor Furthermore, knockdown of TENM4 led to upregulation of genes
(70%), male (60%), and a peripheral blood cell source (70%). Viral infec- associated with neuronal differentiation and downregulation of genes
tions occurred in 90% mainly of cytomegalovirus (55%). Acute-GVHD related to cancer-associated pathways. Two TENM4-/- clones from the
occurred in 55% and chronic GVHD in 70%. CRISPR-Cas9 gene-edited SK-N-BE(2) were uncovered; both clones
Conclusions: Distinct kinetics of recovery were observed in each sub- induced a neuronal differentiation-like morphology with impaired
population. NK cells are the first to recover, followed by memory T clonogenic capacity compared to wildtype cells. Both knockout clones
cells, meaning they could derive from the graft, while the number of displayed decreased MYCN expression in comparison to wild-type
B cells and auxiliary T cells (CD4+) increases only six months after cells. Importantly, TENM4-/- cells did not lead to tumor formation
the transplant. The factors analyzed interfere in the thymic production when grafted into nude mice as opposed to wild-type SK-N-BE(2) cells
of T lymphocytes and the recovery of CD4+ lymphocytes. The results that formed tumors. We further examined primary neuroblastomas
emphasize the importance of quantifying lymphocytes subpopulations and detected a significantly higher protein and mRNA expression
in the peripheral blood as a way to improve monitoring in patients with of TENM4 in high-risk vs. non-high-risk and MYCN-amplified vs.
acute leukemia who underwent a bone marrow transplant, in order to non-MYCN amplified tumors.
evaluate the best moment to revaccinate these individuals. Conclusions: Our data suggest that TENM4 is expressed in a
subpopulation of neuroblastomas with MYCN-amplification,
and plays an important role in neuroblastoma growth and dif-
E-Poster Topic: AS05 SIOP Scientific programme / AS05.e ferentiation. TENM4 may be a potential therapeutic target in
Neuroblastoma neuroblastoma.

E-POSTER VIEWING
EP138 / #1135 SINGLE CELL ANALYSIS OF HUMAN BONE
EP137 / #676 CHARACTERIZATION OF TENEURIN-4 AND METASTATIC NEUROBLASTOMA UNRAVELS EMERGING
ITS ROLE IN NEUROBLASTOMA TUMORIGENESIS IMMUNE CELL POPULATIONS IN THE BONE MARROW

Sara Abu Ajamieh1 , Teodora Andonova1 , Adena Pepich1 , Thale Olsen1 , Adele Alchahin1 , Bethel Tesfai Embaie1 , Shenglin Mei2 , Bronte
Conny Tümmler1 , Subazini Thankaswamy-Kosalai2 , Chandrasekhar Manouk Verhoeven1 , Thale Olsen1 , Peter Kharchenko2 , John Inge
Kanduri2 , Susanne Fransson3 , Tommy Martinsson3 , Ninib Baryawno1 , Johnsen1 , Per Kogner1 , Ninib Baryawno1
Anders Näsman4 , Per Kogner1 , John Inge Johnsen1 , Malin 1 Karolinska Institutet, Women And Children’s Health, Solna, Sweden;
Wickström1 2 Harvard Medical School, Biomedical Informatics, Boston, United States of
1 Karolinska Institutet, Department Of Women’s And Children’s Health, America
Stockholm, Sweden; 2 Sahlgrenska Academy, University of Gothenburg,
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S212 of S635 ABSTRACTS

Background and Aims: Introduction: Neuroblastoma (NB) is a pedi- The University of Tokyo, Department Of Pediatrics, Tokyo, Japan; 7 National
atric solid tumor with frequent metastasis. Patients with metastasis Cancer Center Hospial, Department Of Pharmacy, Tokyo, Japan
account for 50% of all NB cases at diagnosis, where 70% have bone
marrow involvement. To better understand the cancer progression and Background and Aims: This study aimed to determine the safety,
metastatic processes, revealing unique relationships between tumor pharmacokinetics, and recommended phase-2 dose (RP2D) of alec-
cells and cell populations in the bone marrow microenvironment is tinib, a highly selective ALK (anaplastic lymphoma kinase) inhibitor, for
essential for future therapeutic strategies. Japanese pediatric patients with relapsed/refractory malignant solid
Methods: Bone marrow aspirates were taken from eleven clinically tumors or malignant lymphoma.
diagnosed neuroblastoma patients. Five patients had clinically con- Methods: We conducted a single-center, phase 1 trial for patients aged
firmed “tumor infiltration” in their bone marrow, two of which had 3–18 years who were refractory or intolerant to standard treatment.
confirmed bone metastasis. Six patients had “no tumor infiltration”, Patients confirmed to have an ALK overexpression by immunohisto-
of which we sequenced the matched primary tumor from two of chemistry or ALK alteration by fluorescence in situ hybridization, or
the patients. Bone tumors were dissociated into single cells. Both next generation gene sequencing were eligible. The primary endpoint
tumor and bone marrow samples underwent red blood cell removal, was the incidence of dose-limiting toxicity.
FACS sorting (CD235neg ) and proceeded for single-cell RNA sequenc- Results: Nine patients with a median age of 7 years (range 4–15 years)
ing (scRNA-seq) using the 10X Genomics platform (Chromium 3’ v2 were enrolled between May 2018 and January 2020, among whom
kit, 10x Genomics). Bone marrow aspirates were enriched for: 1) eight had neuroblastoma and one had rhabdomyosarcoma. No dose-
mesenchymal cells (PDGFRapos ), adrenergic tumor cells (CD24pos ), limiting toxicity was observed, and the level 2 dose of this study was
Schwann cell precursor (SCP)-like cells (ERBB3pos ) together with determined as the RP2D. Grade ≥3 adverse effects were only observed
CD235neg cells, 2) solely sorting CD235abneg . These were combined in 4 patients (44.4%), with lymphopenia (22.2%), anemia (22.2%), neu-
prior to scRNA-seq. tropenia (11.1%), and fever (11.1%) being the most common grade ≥3
Results: scRNA-seq revealed eleven major cell populations including adverse events associated with alectinib. Japanese children had sim-
immune, tumor and erythroid cells. Tumor cells infiltrating the bone ilar pharmacokinetic data as Japanese adults. Although all patients
marrow in the metastatic patients showed an adrenergic phenotype were confirmed to be positive by immunohistochemistry, ALK alter-
(PHOX2B, TH, MYCN, DBH). We identified a significant increase in ation could not be confirmed via NGS in seven patients NGS were
total cell fraction of immature B cells, NK CD56bright cell populations, performed, and no objective responses were observed in the patients
as well as a distinct macrophage population in the metastatic patients enrolled.
compared to the non-metastatic neuroblastoma patients. Conclusions: Alectinib was safe and well-tolerated in Japanese chil-
Conclusions: Our discoveries provide novel biological insight for the dren with malignant solid tumors. The RP2D determined in this trial
bone and bone marrow metastatic field of neuroblastoma. Further was 150, 300, 450, and 600 mg/day for patients <15, ≥15 and <25,
evaluation might potentially bring novel and important insights to neu- ≥25 and <35, and ≥35 kg, respectively. After this trial, enrollment
roblastoma research, and most importantly, a better understanding on of pediatric patients began in an ongoing phase 2 study with alec-
how to target advanced and heterogenous disease at earlier stages tinib for patients with solid tumors with ALK alteration identified via
prior to metastatic spread. next generation gene sequencing. Funding: Japan Agency for Medical
Research and Development and Chugai Pharmaceuical Co.Ltd. Clinical
Trial Information: JMA-IIA00354
EP139 / #657 SAFETY AND PHARMACOKINETICS OF
ALECTINIB FOR JAPANESE PEDIATRIC PATIENTS WITH
RELAPSED/REFRACTORY MALIGNANT SOLID TUMORS OR EP140 / #1461 DISPENSING ORAL TEMOZOLOMIDE IN
MALIGNANT LYMPHOMA (NCCH1708): A SINGLE-CENTER CHILDREN: PRECISION AND STABILITY OF A NOVEL
PHASE I TRIAL READY-TO-USE LIQUID FORMULATION IN COMPARISON WITH
CAPSULE DERIVED MIXTURES
Ayumu Arakawa1 , Sawako Tomatsuri2 , Akihiko Yoshida3 , Shinji
Kohsaka4 , Junko Takita5 , Mitsuteru Hiwatari6 , Kentaro Watanabe6 , Caroline Lemarchand1 , Hugues Bienaymé1 , André Rieutord2 , Lionel
Yukari Hoshina2 , Kenta Anjo2 , Ryo Sadachi2 , Ryunosuke Machida2 , Tortolano2,3 , Maxime Annereau2 , Jeremy Bastid1
Reiko Makihara7 , Yoshimasa Saito7 , Kenichi Nakamura2 , Chitose 1 ORPHELIA Pharma, Orphelia Pharma, Paris, France; 2 Gustave Roussy,
Ogawa1 Clinical Pharmacy Department, Villejuif, France; 3 Université Paris-Saclay, Ea
1 National Cancer Center Hospital, Department Of Pediatric Oncology, 401 Matériaux Et Santé, Ufr Pharmacie, Châtenay-Malabry, France
Tokyo, Japan; 2 National Cancer Center Hospital, Clinical Research Sup-
port Office, Tokyo, Japan; 3 National Cancer Center Hospital, Department Background and Aims: Parents and caregivers often overcome the
Of Diagnostic Pathology, Tokyo, Japan; 4 National Cancer Center Research lack of commercially available pediatric formulations by mixing adult
Institute, Division Of Cellular Signaling, Tokyo, Japan; 5 Kyoto University, dosage forms with food. This study aimed at assessing the risks asso-
Department Of Pediatrics, Kyoto, Japan; 6 Graduate School of Medicine, ciated with handling of temozolomide (TMZ) capsules in comparison
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S213 of S635

with a ready-to-use oral suspension specifically formulated for children Childrens Hospital NHS Foundation Trust, Paediatric Oncology, Liverpool,
(Ped-TMZ). United Kingdom; 8 University Hospitals Bristol and Weston NHS foun-
Methods: TMZ capsules were opened and their content (equivalent to dation trust, Department Of Pediatric Surgery, Bristol, United Kingdom;
90 mg) mixed with food vehicles. Similar dose was sampled from Ped- 9 Newcastle University, Newcastle University Centre For Cancer, Newcastle
TMZ. TMZ and its degradation product, amino-imidazole-carboxamide upon Tyne, United Kingdom; 10 Great Ormond Street Hospital for Children,
(AIC), were assayed using UV-HPLC at 0, 30 and 60 min. Statistical Department Of Paediatric Oncology, London, United Kingdom; 11 Royal

analysis was performed to evaluate TMZ recovery, AIC amount and Marsden Hospital, Children And Young Peoples Unit, Surrey, United King-
impact of operator on accuracy. Acceptance criteria were pre-defined dom; 12 Royal Manchester Children’s Hospital, Paediatric Surgery, Manch-
for TMZ (95.0-105.0%) and AIC (<1%) content. ester, United Kingdom; 13 University of Manchester, Division Of Cancer
Results: Mean TMZ recovery was 96.6±1.2% for Ped-TMZ and Sciences, Manchester, United Kingdom; 14 Glasgow Children’s Hospital,
91.0±1.5% and 91.6±1.4% for the capsule-derived preparations with Paediatric Surgery, Glasgow, United Kingdom; 15 University of Liverpool,
apple juice and applesauce, respectively. The recovery of TMZ in Ped- Institute Of Life Course And Medical Sciences, Liverpool, United Kingdom;
TMZ was systematically and significantly higher than that observed 16 Great North Children’s Hospital, Paediatric Surgery, Newcastle upon Tyne,

after handling of TMZ capsules (p<0.0001) and within specifications. United Kingdom
The recovery of TMZ from capsules in food never met acceptance cri-
teria. In addition, the 4 tested food vehicles (applesauce, cream, milk, Background and Aims: Ganglioneuroma (GN) and ganglioneuroblas-
purée) had a significant effect on TMZ stability (p=0.0042) and AIC sig- toma intermixed (GNBi) are neuroblastic tumours with typically a
nificantly increased with time in 3 of the 4 vehicles (p<0.0001). Only benign clinical course. To accurately discriminate between GN/GNBi
1/72 preparations from capsules in food met the acceptance crite- and the more aggressive nodular ganglioneuroblastoma (GNBn) phe-
ria, whereas Ped-TMZ showed no TMZ loss and AIC remained within notype surgical resection is required however rate(s) of discrepancy
specifications. between initial lesion biopsy and resection pathology are unknown.
Conclusions: This study demonstrated a significant impact of han- This study therefore analyses discrepancy rate(s) between histology
dling TMZ capsules on dosing accuracy with a mean deliverable dose diagnosis with initial biopsy and in the resected surgical specimen as
decreased by 9%, and possibly even greater in routine practice as part of a wider review in management of GN/GNBi.
complete food intake by the child is unlikely. Rapid degradation of Methods: Patients 0-24 yrs diagnosed with localized GN or GNBi
TMZ was evidenced in certain food vehicles. When combined with the between 1990-2020 and who underwent initial biopsy followed by
known effect of food on TMZ absorption, mixing capsule content with surgical resection of the tumour were eligible.
food may significantly reduce TMZ exposure, highlighting the need of Results: 112 of 242 patients with GN/GNBi were registered from
age-appropriate TMZ formulations. 11 UK CCLG centres during the study period. Thirty-seven patients
(33%) had a new tumour phenotype documented in their final pathol-
ogy. In 25 (22%) index cases diagnosis changed from GN to GNBi. In
EP141 / #1089 PHENOTYPE SHIFT IN TUMOUR PATHOLOGY 12 (11%) patients the changes were significant leading to a modifi-
WITH GANGLIONEUROMA AND GANGLIONEUROMA cation in oncological management (in 3 from GN to GNBn; in 6 from
INTERMIXED BETWEEN INITIAL LESION BIOPSY AND GNBi to GNBn; in 2 from GNBi to differentiating NB and in a sin-
SURGICAL RESECTION - A UK CCLG NATIONWIDE STUDY gle patient from GNBi to poorly differentiated neuroblastoma). Raised
urinary VMA, HVA and MIBG avidity were found to be independent
Annita Budzanowski1 , Katherine Burnand2 , Bruce Okoye2 , Kate predictors of more aggressive pathology (p=<0.05, Fisher’s exact test).
Cross1 , Kate Wheeler3 , Rhiannon Collins3 , Amy Drawbridge3 , One GNBn patient died from acute surgical complications and another
Jonathan Neville4 , Juliet Gray4 , Nigel Hall4 , Ramya Ramanujachar4 , index case with poorly differentiated neuroblastoma is still receiving
Max Pachl5 , Snigdha Reddy5 , Susanne Gatz5 , Carla Kielrulff6 , Lisa ongoing active treatment 15 years after diagnosis. Five patients have
Howell7 , Chun Sui Kwok7 , Rajeev Shukla7 , Barry Pizer7 , Timothy ongoing symptoms.
Rogers8 , Robin Garrett-Cox8 , Nadeem Alkhafaji8 , Deborah Tweddle9 , Conclusions: In this study 33% of patients had a shift in their final
Vicky Anne Carruthers9 , Giuseppe Barone10 , John Anderson1 , tumour pathology with 11% cases being significant. Raised urinary
Sucheta Vaidya11 , Sally George11 , Nichola Seymour12 , Sarah VMA, HVA or MIBG avidity should raise clinical suspicion of a more
Braungart12 , Guy Makin13 , Michael Jacovides14 , Paul Losty15 , Hany aggressive pathology. Individualised management is crucial to offset
Gabra16 , Paola Angelini11 poor outcomes.
1 Great Ormond Street, Paediatric Surgery, London, United Kingdom; 2 St
George’s Hospital, Paediatric Surgery, QT, United Kingdom; 3 Oxford Univer-
sity Hospital NHS Foundation Trust, Paediatric Haematology And Oncology, EP142 / #769 COMBINATORIAL THERAPY OF IL-21
Oxford, United Kingdom; 4 University of Southampton, Cancer Sciences SECRETION ONCOLYTIC VIRUS AND ANTI-ROR1 CAR NK
Unit, Southampton, United Kingdom; 5 Birmingham children’s hospital, Pae- CELLS AGAINST NEUROBLASTOMA: SIGNIFICANT EFFECT
diatric Surgery, Birmingham, United Kingdom; 6 Royal Aberdeen Children’s IN-VITRO AND IN-VIVO
Hospital, Paediatric Oncology, Aberdeen, United Kingdom; 7 Alder Hey
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S214 of S635 ABSTRACTS

Yaya Chu1 , Meijuan Tian1 , Uksha Saini2 , Timothy Cripe2 , Elaine Adam-De-Beaumais1 , Gwenael Le Teuff13 , Gilles Vassal1 , Birgit
Mardis3 , Dean Lee4 , Stanley Riddell5 , Kevin Cassady2 , Mitchell Cairo1 Geoerger1 , Pablo Berlanga1
1 New York medical college, Pediatrics, Valhalla, United States of Amer- 1 Gustave Roussy Cancer Campus, Department Of Pediatric And Adolescent

ica; 2 The Ohio State University, Center For Childhood Cancer And Blood Oncology, VILLEJUIF, France; 2 Institut Curie, Siredo Oncology Center (care,
Diseases, Columbus, United States of America; 3 Nationwide Children’s Hos- Innovation And Research For Children And Aya With Cancer), Paris, France;
pital, The Steve And Cindy Rasmussen Institute For Genomic Medicine, 3 Institute of Pediatric Hematology and Oncology (IHOPe), Léon Bérard
Columbus, United States of America; 4 Nationwide Children’s Hospital, Cen- Cancer Centre, Lyon, France; 4 University Hospital of Nancy, Pediatric Oncol-
ter For Childhood Cancer And Blood Diseases, Columbus, United States of ogy, Nancy, France; 5 University Hospital Centre of Strasbourg, Department
America; 5 4Fred Hutchinson Cancer Research Center, Program In Immunol- Of Tumor Pediatrics, Strasbourg, France; 6 La Timone University Hospital
ogy, Seattle, United States of America of Marseille, APHM, Marseille, France, Department Of Pediatric Oncol-
ogy, Marseille, France; 7 Hopital d’Enfants Armand Trousseau, Pediatrics,
Background and Aims: Novel therapies are desperately needed Paris, France; 8 CHU de Nantes, Oncologie Et Hématologie Pédiatrique,
for children with recurrent and/or metastatic neuroblastoma (NB) Nantes, France; 9 CHU Toulouse, Hôpital des Enfants„ Pediatric Hemato-
(Chu/Cairo, JITC, 2021). ROR1 is highly expressed by most NB. oncology Unit, Toulouse, France; 10 Centre Oscar Lambret, Department Of
Our group has successfully expanded peripheral blood NK cells Pediatric Oncology, Lille, France; 11 CHU de Bordeaux, Oncologie Et Hema-
(exPBNKs) with feeder cells and electroporated CAR mRNA to tologie Pédiatrique, Bordeaux, France; 12 CHU d’Angers, Unité Hémato-
exPBNKs (Chu/Cairo, Cancer Immunol Res, 2015). Oncolytic herpes onco-immunologie Pédiatrique, Fédération De Pédiatrie, Angers, France;
simplex viruses (oHSVs) have been safely used in clinical trials for 13 Institut Gustave Roussy, Université Paris-Saclay, Service De Biostatistique

a wide range of cancers (Cassady, et al, JITC, 2021). We sought to Et D’Épidémiologie, Villejuif, France
determine the anti-tumor efficacy of the therapy combination of oHSV
engineered to express human IL21 with anti-ROR1 CAR engineered Background and Aims: Neuroblastoma is the most common extra-
exPBNK cells (CAR exPBNKs) against ROR1+ NB. cranial solid tumor in children. In case of relapse/refractory high-
Methods: ExPBNKs were expanded and electroporated with anti- risk disease, long-term survival is poor. The European prospective
ROR1-CAR mRNA (Chu/Cairo, JITC, 2021). oHSV C134 was modified precision medicine trial MAPPYACTS defined molecular profiles of
to express hIL-21 gene (C021). The supernatants containing C134 and relapse/refractory pediatric malignancies to suggest the most adapted
C021 were generated as previously described (Cassady, et al, JITC, targeted treatment (Berlanga et al, Cancer Discovery 2022). The aim
2021). In-vitro cytotoxicity of CAR exPBNKs against NB cell lines were of this study was to analyze the outcome of the neuroblastoma cohort
examined by ELISA assays of IFN-g, granzyme and perforin levels. In- and identify recruitment barriers of these patients.
vivo hIL21 secretion and anti-tumor effect of the C021 with CAR Methods: We analyzed clinical data, clinical molecular tumor board
exPBNKs was examined utilizing human NB xenografted NSG mice. (CMTB) reports and follow-up of patients with neuroblastoma included
Results: C021 at MOI 0.025 or CAR exPBNKs significantly inhibited in MAPPYACTS (NCT02613962).
NB growth compared to controls. The combination of C021 and CAR Results: 104 of 787 included patients (13%) had neuroblastoma. Data
exPBNKs significantly enhanced the killing of NB (p<0.05) with signifi- of the first 46 patients included in 3 French centers are presented
cantly enhanced secretion of IFN-g (p<0.05), granzyme B (p<0.05) and here. For them, 49 biopsy or surgery procedures were performed,
perforin (p<0.05) and significantly enhanced expression of NK activat- 33 had successful sequencing analysis and 26 at least one potential
ing marker CD25 (p<0.05) compared to controls. Our in-vivo animal actionable alteration. Patients had 1 to 6 (median, 2) prior treatment
study showed that NB infected with C021 secreted hIL21 and the com- lines and received 0 to 5 (median, 1) treatments after the CMTB rec-
bination of C021 and CAR exPBNKs reduced tumor burden in human ommendation. Among the 26 patients with actionable alterations, 6
NB xenografted NSG mice compared to the untreated group (p<0.05) (23%) received a matched treatment, 3 (12%) within an early phase
and the CAR exPBNKs-treated group (P=0.056). trial. 23 patients were not included in a clinical trial according to their
Conclusions: Our data demonstrate the significant anti-tumor efficacy molecular alteration because: patient in remission after prior treat-
of combining C021 with anti-ROR1 CAR exPBNKs to therapeutically ment (6, 23%), death within 90 days after CMTB (4, 15%), another
target NB in-vitro and in-vivo. (Funded by U54 CA232561). non-targeted therapy as per physician’s choice (6, 23%), no trial open
and received matched treatment off-label (3, 12%), parent’s refusal
to experimental therapy (2, 8%), biomarker trial not open (1, 4%), 1
EP143 / #861 RECRUITMENT BARRIERS OF PATIENTS WITH patient without data. Data including all patients will be presented at the
NEUROBLASTOMA IN EARLY PHASE TRIALS: ANALYSIS OF THE meeting.
EUROPEAN PRECISION MEDICINE TRIAL MAPPYACTS COHORT Conclusions: Our results demonstrate that although most
patients with neuroblastoma have potentially actionable alter-
Jordane Chaix1 , Gudrun Schleiermacher2 , Nadege Corradini3 , Pascal ations, there are limited adapted relevant clinical trials. It further
Chastagner4 , Natacha Entz-Werle5 , Nicolas Andre6 , Judith Landman highlights the importance of performing molecular profiling
Parker7 , Estelle Thebaud8 , Marion Gambart9 , Anne-Sophie early to maximize the chance of patients to benefit from this
Defachelles10 , Stéphane Ducassou11 , Emilie De Carli12 , Tiphaine approach.
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ABSTRACTS S215 of S635

EP144 / #1634 PRECISION MEDICINE IN CHILDHOOD 1 Karolinska Institutet, Department Of Women’s And Children’s Health,
CANCER – A REPRESENTATIVE CASE OF NBL Stockholm, Sweden; 2 Harvard Medical School, Department Of Biomedical
Informatics, Boston, United States of America
Constance De Montlaur1,2 , Reidun Aesoy1,2 , Annette Brenner2 , Rakel
Brendsdal Forthun3 , Randi Hovland3 , Emmet Mccormack1 , Lars Background and Aims: Neuroblastoma (NB) is the most common
Herfindal1 , Maria Winther Gunnes2 extracranial solid tumor of childhood. MYCN amplification is a fre-
1 University of Bergen, Department Of Clinical Science, Bergen, Norway; quent genetic abnormality, representing a critical stratifying prognos-
2 Haukeland University Hospital, Department Of Paediatrics, Bergen, Nor- tic marker. Given that MYCN plays a key role in NB tumorigenesis and
way; 3 Haukeland University Hospital, Section For Cancer Genomics, Bergen, aggressiveness, TH-MYCN transgenic mouse model is widely used in
Norway NB research, which is characterized by the overexpression of MYCN
under the TH promoter. This overexpression gives rise to tumors exclu-
Background and Aims: Over 80% of paediatric cancer patients will sively in the sympathoadrenal system, reflecting NB features. We
become long-term survivors, however two-thirds of all childhood can- aimed to explore the transcriptional landscape of TH-MYCN tumors by
cer survivors will suffer from late effects after their treatment, which single-cell RNA sequencing (scRNA-seq) and utilize the bioinformati-
might have significant impact on their quality of life. Thus, there is cally predicted essential interactions to establish NB organoid models
a great need for new therapy approaches more precisely tailored (tumoroids).
for each individual paediatric patient. Through translational research, Methods: Tumors from four homozygous and four hemizygous TH-
combining molecular diagnostics with preclinical cancer models, we MYCN mice were dissociated into single cells, enriched for viable non-
aim to predict better treatment options for patients in order to improve erythroid cells, profiled by scRNA-seq (10x Chromium), sequenced on
their outcomes. the NextSeq platform (Illumina), and analyzed using pagoda2 and seu-
Methods: are included in Results. rat. Immunofluorescence was used to validate the bioinformatically
Results: A paediatric patient presented with high-risk neuroblastoma predicted cell types. Harnessing scRNA-seq and spatial data, cul-
(NBL), with primary tumour in the left adrenal gland and multiple ture conditions for TH-MYCN tumoroids were optimized. Tumoroids
skeletal metastases and bone marrow involvement. Through NGS, were established and expanded for immunofluorescence whole-mount
MYCN amplification in combination with 11q deletion were identi- staining.
fied in the primary tumour, although these genetic alterations usually Results: scRNA-seq of TH-MYCN tumors revealed 16 major cell popu-
are mutually exclusive. There were no ALK aberration, ATRX or TERT lations, spanning stromal, immune, and tumor compartments. MYCN+
rearrangements. The patient received treatment according to the tumor cells predominantly resembled sympathoblasts (Isl1, Stmn1,
European SIOPEN HR-NBL1 high-risk NBL treatment protocol with Nefl), while chromaffin cells (Th, Dbh, Chga) were rare. Immunofluo-
some modifications and is now in ongoing complete remission. Per- rescence staining confirmed that tumors predominantly consisted of
forming droplet digital PCR on blood samples, we were able to monitor proliferating sympathoblasts. Tumor cell nests were surrounded by
the tumour burden, which in concordance with more invasive imag- supportive stromal and immune cells. The adrenergic tumor cell com-
ing and bone marrow sampling indicated complete response during position in TH-MYCN tumors was highly reminiscent of human NB.
treatment and follow-up . Both primary cell cultures and mural patient Interestingly, we discovered transitioning immature adrenergic tumor
derived xenograft (PDX) models were established from this NBL and cells (Sox11, Ptprs, Atrx). Furthermore, ex vivo tumoroid cultures
diverse treatments were tested with the aim to identify drug can- were robust and highly proliferative. Whole-mount staining of tumor-
didates for individualized therapy in case of refractory or relapsed oids revealed adrenergic Phox2b+ expression, neurite outgrowth,
disease. The primary cells showed resistance to most of the drugs proliferative inner core, and apoptotic outer edge of the tumoroids.
tested, including Temozolomide, Cyclophosphamide and Etoposide. A Conclusions: This comprehensive atlas of TH-MYCN tumors provides
combination of Venetoclax and Idasanutlin was found to have great a resource for unmasking novel therapeutic targets. Tumoroid-based
synergistic impact on proliferation of the primary cells. In vivo PDX drug screening offers a tool for tailoring precision medicine.
drug experiments are on-going.
Conclusions: We postulate that a combination therapy of Venetoclax
and Idasanutlin may be an effective treatment option for patients with EP146 / #228 USP44 DRIVES DISEASE AGGRESSION AND IS
NBL presenting similar genetic profile. A POTENTIAL THERAPEUTIC TARGET IN NEUROBLASTOMA

Sajjad Hussain1 , Tibor Bedekovics1 , Ying Zhang1 , Asma Ali1 , Lucia


EP145 / #339 SINGLE-CELL TRANSCRIPTOMICS REVEAL Paolini1 , Thomas Ekstrom1 , Hina Mahmood1 , Shizhen Zhu2 , Steven
TUMOR CELL DIVERSITY IN NEUROBLASTOMA TRANSGENIC Johnsen1 , Paul Galardy1
MOUSE AND ORGANOID MODELS 1 Mayo Clinic, Pediatric And Adolescent Medicine, Rochester, United States
of America; 2 Mayo Clinic, Biochemistry And Molecular Biology, Rochester,
Bethel Tesfai Embaie1 , Hirak Sarkar2 , Adele Alchahin1 , Jörg Otte1 , United States of America
Thale Olsen1 , Shenglin Mei2 , Peter Kharchenko2 , Ninib Baryawno1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S216 of S635 ABSTRACTS

Background and Aims: Neuroblastoma is the most common solid tify cellular pathways promoting survival as potential therapeutic
tumor outside of the CNS that contributes disproportionately to can- targets.
cer deaths in the young. Recent data, and the relative lack of recurrent Methods: Drug-resistant NB cell models were generated using cyto-
nucleotide mutations in this disease, suggests that epigenetic and post- toxics included in current chemotherapy protocols for HR-NB patients.
translational events play an important role in the pathogenesis of Drug-resistant cells were characterized using gene expression and
this disease. De-ubiquitinating enzymes (DUBs) are a class of highly DNA methylation profiling, ChIP-seq assays and NGS sequenc-
drugable targets that regulate myriad cellular events including his- ing. Functional in-vitro and in-vivo assays were performed. Paired
tone ubiquitination. We therefore sought to identify de-ubiquitinating diagnosis-relapse tumor samples were used for validation. High-
enzymes that contribute to the development, progression, or therapeu- throughput screening (HTS) was performed to test cytotoxicity of
tic responses in neuroblastoma. 2,400 FDA approved compounds (concentration range 1-0.1 μM) in
Methods: We performed a computational screen of 95 de- both drug-resistant and native NB cells.
ubiquitinating enzymes followed by validation using three independent Results: Drug-resistant cells showed changes of expression levels
neuroblastoma datasets. We used model systems including neurob- of genes involved in cell differentiation, migration and DNA dam-
lastoma cell lines, genetically modified mouse cells, and a transgenic age repair processes. A significant portion of these genes have been
zebrafish system to study the role of USP44 in neuroblastoma. reported previously in NB associated with mesenchymal state and
Results: Through a computational screen, increased expression of drug-resistance. Functionally, drug-resistant cells showed defects on
USP44 - an enzyme that targets ubiquitinated histone H2B - had the cell proliferation, cell cycle and colony-forming capability whereas
greatest impact on neuroblastoma survival compared with 94 other enhanced invasion and in-vivo tumor growth capacities. DNA methyla-
enzymes. The impact on survival was confirmed in three indepen- tion profiling identified extensive hypermethylation across the genome
dent datasets where USP44 was correlated with measures of disease of drug-resistant cells, whereas hypomethylation affected super-
aggression. We observed a direct relationship between USP44 and enhancers previously described in NB mesenchymal phenotype. HTS
proliferation, migration, and invasion in three independent cell lines. identified cytotoxicity to compound treatment in drug-resistant cells,
Depletion of the histone H2B ubiquitin ligase RNF20 produced similar with 50 compounds inducing cell death (more than 80%) at low
results. Enforced expression of USP44 impaired neurite outgrowth in concentration (1μM). Compounds with high-activity target molecules
cells cultured with retinoic acid. Transgenic expression of USP44 accel- associated with apoptosis/cell cycle/DNA damage (60%), infection
erated the development of MYCN-driven neuroblastoma in a zebrafish (37%), metabolism (15%) and protein tyrosine kinase (9%) pathways.
model. Conclusions: We have identified a set of differentially expressed genes
Conclusions: Our data lead us to conclude that USP44 has oncogenic and signalling pathways potentially underlying acquired resistance in
activity in neuroblastoma likely through removing ubiquitin from his- our NB model. HTS identified active compounds against our drug-
tone H2B. As this epigenetic mark affects global gene expression and resistant NB cells, providing insight into potential vulnerabilities of
has been tied with differentiation, we hypothesize that USP44 con- interest for therapeutic opportunities.
tributes to the differentiation block in these tumors. The ability of
USP44 silencing to inhibit cell proliferation and metastatic behavior
suggests that it may be an attractive therapeutic target in high-risk EP148 / #1261 CHARACTERIZATION OF HIGH RISK
neuroblastoma. NEUROBLASTOMA. POTENTIAL BIOMARKERS FOR
STRATIFICATION OF HIGH RISK NEUROBLASTOMA

EP147 / #1642 MOLECULAR MECHANISMS OF Alícia Garrido1 , Sara Perez1 , Marta Garcia1 , Laura Garcia1 , Isadora
CHEMORESISTANCE IN NEUROBLASTOMA PATIENTS Lemos1 , Eva Rodríguez2 , Oscar Muñoz Aznar1 , Mariona Suñol2 ,
Soledad Gómez-González1 , Cinzia Lavarino1
Marta Garcia1 , Soledad Gómez-González1 , Alicia Garrido-Garcia1 , 1 Fundació Sant Joan de Déu, Developmental Tumor Biology Laboratory,
Oscar Muñoz Aznar1 , Isadora Lemos1 , Mariona Suñol2 , Carlota Barcelona, Spain; 2 Hospital Sant Joan de Déu, Barcelona, ) Department Of
Rovira2 , Cinzia Lavarino1 Pathology, Barcelona, Spain
1 Hospital Sant Joan de Déu, Pediatric Cancer Center Barcelona (pccb),
Barcelona, Spain; 2 Hospital Sant Joan de Deu, Pathology, Barcelona, Spain Background and Aims: In the clinical practice, patients with high-risk
neuroblastoma are treated uniformly without further stratification.
Background and Aims: The emergence of drug-resistance is the However, high-risk neuroblastoma embodies a heterogeneous group
major cause of cancer treatment failure. Patients with high-risk neu- of tumors, whereby patients can display response to treatment and
roblastoma tumors (HR-NB) are treated with intensive multimodal long-term outcome or develop early progressive disease with poor out-
therapy, however, approximately 60% suffer disease relapse. Achiev- come. We investigated high-risk neuroblastoma to identify epigenetic
ing cure after relapse is challenging due to tumor heterogeneity and and molecular alterations underlying the divergent clinical evolution
the development of resistance. We have investigated the molecu- and treatment response of these tumors, and to identify biomarkers for
lar mechanisms of therapy-induced resistance in HR-NB, to iden- their stratification into distinct subgroups.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S217 of S635

Methods: We analyzed DNA methylation microarray and gene Results: Here, we report that NB patients with high expression of
expression data from more than 500 high-risk neuroblastoma sam- MTHFD1 have a shorter progression-free survival (P=0.002) and the
ples obtained at diagnosis. Snap-frozen and formalin-fixed paraffin- expression levels of MTHFD1 and MYCN are positively correlated
embedded samples were used for pyrosequencing, phospho-kinase (R=0.82). Suppression of MTHFD1 disturbs NADPH redox homeosta-
array, immunoblotting and immunohistochemical analyses. Patients sis, accelerates cell death, and suppresses growth (P<0.05 for all).
with disseminated neuroblastoma diagnosed after 18 months of Also, inhibition of MTHFD1 suppresses NB cell growth in cell-based
age, or with MYCN amplified tumors were classified as high-risk. xenografts (n=6 mice per group). We determined that MTHFD1 is
Cox-regression models and machine-learning analysis were used for transcriptionally activated by MYCN. The elevation of oxidative stress
survival analyses. Survival curves were estimated by Kaplan-Meier through MTHFD1 knockdown or the use of methotrexate, an antifolate
method and compared by log-rank test. Pathway analysis was per- drug, sensitizes cancer cells to JQ1, targeted therapy for NB.
formed using R package KEGGREST, ConsensusPathDB-MaxPlanck Conclusions: Our study identifies that MTHFD1 confers redox home-
and R package topGO. ostasis and promotes growth and suppressed apoptosis in NB cells,
Results: We identified distinct DNA methylation profiles within high- providing new targets and strategies for the treatment of neurob-
risk neuroblastoma. Cox-regression models and machine-learning lastoma. The synergistic antitumor effects of MTX and JQ1 have
analysis, identified differentially methylated CpG sites that defined important clinical significance.
two subgroups of patients with substantially different overall survival
(5-year OS: 91.48% versus 8.11%). Moreover, we identified methyla-
tion markers that could distinguish these clinically relevant subgroups EP150 / #792 MICRO-RNA IN NEUROBLASTOMA- NOVEL
of tumors. Integrative analysis of DNA methylation and matching gene DIAGNOSTIC AND PROGNOSTIC MARKERS
expression data, identified differential expression of genes involved in
cellular metabolism, purine biosynthesis and AKT/mTOR cell signaling. Aditya Gupta1 , Saumyaranjan Mallick2 , Jagdish Prasad Meena1 , Disha
Protein expression analysis identified high levels of proteins involved Kakker2 , Ravi Majhi1 , Aijaz Ahmad2 , Rachna Seth1 , Venkateswaran
in IMP metabolism and increased activation of AKT/mTOR pathways in Iyer2
highly aggressive neuroblastoma. 1 All India Institute of Medical Sciences- New Delhi, Division Of Pediatric
Conclusions: We have identified (epi)genetic changes underlying the Oncology, Department Of Pediatrics, New Delhi, India; 2 All India Institute
heterogenous behavior of aggressive neuroblastoma, and revealed of Medical Sciences, Pathology, New Delhi, India
altered pathways of interest for potential therapeutic options. We
identified a set of markers that enabled stratification of high-risk Background and Aims: The microRNAs(miRNA) are short-length RNA
neuroblastoma into clinically relevant subgroups. fragments which number approximately 2000 in mammals. These are
approximately 22 nucleotide long and have a role in RNA silencing and
post-transcriptional regulation of gene expression. Differential expres-
EP149 / #1025 MODULATION OF REDOX HOMEOSTASIS BY sion of miRNA has been found in various tumors. The detection of the
INHIBITION OF MTHFD1 IN MYCN AMPLIFIED miRNA in the neuroblastoma(NB) patients has potential of use as a
NEUROBLASTOMA: MECHANISMS AND THERAPEUTIC diagnostic and prognostic modality.
IMPLICATIONS Methods: This was prospective study including 8 cases of NB . Dur-
ing biopsy from cases with suspected NB, one extra tissue-core was
Jinqiu Guan, Mengjia Song, Feifei Sun, Juan Wang, Jia Zhu, Junting collected in RNA- later and stored at -80◦ C. Three cores were sent
Huang, Suying Lu, Zijun Zhen, Yizhuo Zhang in neutral buffer formalin for histopathological examination. After
Sun yat-sen University Cancer Center, Pediatric Oncology, Guangzhou, confirmation of diagnosis of NB, the total RNA was extracted. Next
China generation sequencing(miRNA seq) was done in S5(Ion-torrent) using
540-chip. Small RNA-seq pipeline of ion- reporter was used for data
Background and Aims: Neuroblastoma (NB) is the most common solid analysis and quantification. Three cases of Wilms tumor(WT) were
tumor in childhood. MYCN amplification is tightly associated with poor used as control.
prognosis of pediatric NB. Methylenetetrahydrofolate dehydrogenase, Results: A total of 8 patients of NB with median age of 13.5m(4-72m)
cyclohydrolase and formyltetrahydrofolate synthetase 1 (MTHFD1) is months were included. The size of the NB core biopsy ranged from
a key enzyme in the folate cycle. However, the underlying mechanisms 1-1.5cms. Six were poorly differentiated NB while 2 were of differen-
of MTHFD1 in MYCN amplified neuroblastoma is still unknown. tiating type. Five patients had metastatic tumor and in one of these
Methods: We investigated MTHFD1 expression, clinical relevance, the n-myc gene was amplified. Seven patients got chemotherapy. Four
redox modification, and molecular mechanisms using the NB cells patients are alive(one post autologous-HSCT), 2 patients are on palli-
and tissues (n=63). The antitumor synergistic effects of Methotrex- ation and 2 patients have died. On global miRNA expression profile of
ate (MTX) and JQ1 on NB tumorigenesis were evaluated in vitro and the NB patients miRNA 451a, 19b-3p, 106b-5p and 21-5p were consis-
in vivo. Data analysis used Kaplan-Meier, Pearson’s correlation, and tently high .The data was normalised by logarithmic transformation.The
two-sided Student’s t-test. Principal component analysis showed two distinct clusters of NB and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S218 of S635 ABSTRACTS

WT tumor miRNA expression profile,indicating the miRNA expression EP152 / #128 INCREASED CD11B+-CD49B+NATURAL
is homogenous and unique for each. KILLER (NK) CELL TUMOR INFILTRATION AFTER
Conclusions: miRNA451a,19b-3p,106b-5p and 21-5p can serve as CO-ADMINISTRATION OF ANTI-PD-1/PD-L1 ANTIBODIES IN A
potential diagnostic and prognostic biomarkers for NB and can be MURINE NEUROBLASTOMA MODEL
utilised as liquid-biopsy markers. The miRNA expression profile of
NB can serve to differentiate from WT which has similar clinico- Seiichiro Inoue1 , Yutaka Horiuchi2 , Yuta Takeuchi1 , Takashi
radiological presentations. Murakami2 , Akio Odaka1
1 Saitama Medical Center, Saitama Medical University, Department Of Pedi-

atric Surgery, Kawagoe, Japan; 2 Saitama Medical University, Department Of


EP151 / #1650 PLASMA CELL-FREE DNA ANALYSIS Microbiology, Iruma-gun„ Japan
UNCOVERED TUMOR HETEROGENEITY AND CLONAL
EVOLUTION DURING TREATMENT FOR RELAPSED Background and Aims: The infiltration of immune cells into the tumor
NEUROBLASTOMA microenvironment plays an important role in the anti-tumor immune
response. Immune checkpoint inhibition has been shown to suppress
Fiorella Iglesias Cardenas1 , Prachi Kothari1 , Dana Tsui2 , Ellen Basu1 , tumors in a mouse neuroblastoma model but does not inhibit tumor
Stephen Roberts1 , Nai-Kong Cheung1 , Brian Kushner1 , Shakeel growth. Here, we investigated in vivo anti-tumor effect of simultane-
Modak1 ous administration of anti PD-1/PD-L1 antibodies and further analyzed
1 Memorial Sloan Kettering Cancer Center, Department Of Pediatrics, New the relationship between tumor growth inhibition and infiltration of
York, United States of America; 2 PetDx, INC., Research, San Diego, United immune regulatory cells infiltration.
States of America Methods: Mouse neuroblastoma cells (Neuro-2a) were subcuta-
neously inoculated into A/J mice, and anti-PD-1/ PD-L1 monoclonal
Background and Aims: Treatment for relapsed neuroblastoma antibodies (mAbs; 200 μg/mouse) were intraperitoneally administered
remains a challenge. Plasma cell-free DNA (cfDNA) can detect tumor five times (on day 7,8,9,12 and 14 after the tumor cell inocula-
mutations non-invasively and inform neuroblastoma therapy. tion). Tumor weight was assessed, and CD4+ CD25+ Fox P3+ cell and
Methods: Plasma samples were collected from patients with high-risk CD3- CD49b+ cells were detected by flow cytometry, regulatory T cell
neuroblastoma (HR-NB) at disease progression after informed con- and natural killer (NK) cell in the spleen and tumors of tumor-burden
sent. CfDNA analysis was done by targeted next-generation sequenc- mice were measured.
ing (NGS) using the CLIA-approved Foundation One Liquid (324 genes) Results: Simultaneous administration of anti-PD-1/PD-L1 mAbs sig-
assay or the MSK-IMPACT (322-468 genes) platform. nificantly and moderately inhibited tumor growth in 80% of the mice,
Results: 57/86 (66%) patients harbored at least 1 pathogenic genomic whereas no tumor suppression was not observed in the remain-
alteration in their cfDNA (mean=2, range 0-21): 43/64 (67%) by ing 20% of the mice. The ratio of CD4+ CD25+ FoxP3+ cells in the
Foundation One Liquid and 14/22 (64%) by MSK-IMPACT. Recur- spleen was not significantly different among the treatment group:
ring alterations for the entire cohort included MYCN amplification significant, moderate tumor suppression and isotype control. In addi-
(n=8 patients), ALK point mutations (n=14); mutations and/or promo- tion, CD4+ CD25+ tumor infiltrating lymphocytes were observed from
tor alterations in genes involved in chromatin remodeling (ATRX n=6 all groups, but FoxP3 expression in these cells were not observed.
ARID1A, n= 3, ARID1B n=2, TERT n=3 and ATM n= 3), and mutations Compared to isotype-treated or moderator tumor-suppressed mice,
in RAS-MAPK pathway (KRAS n=3, NRAS n=6, BRAF n=5, PTPN11 a significant increase in tumor infiltrating CD3- CD11b+ CD49b+ NK
n=7, NF1 n=11). Other mutations included those in FGFR1 (n=5), MET cells were observed in mice with significantly suppressed tumors. Ratio
(n=2) and IGF1R (n=1). NGS data on the primary tumor prior to relapse of NK cell infiltration and tumor weight were negatively correlated in
was available in 57 patients. In 23 (40%) patients, new genomic alter- the mAbs co-administered group.
ations appeared in their cfDNA not detected in the pre-relapse tumor. Conclusions: CD4+ CD25+ FoxP3+ regulatory T cells did not con-
Therapy based on cfDNA analysis was instituted in 16/57 patients tribute the extent of tumor suppressive by co-administration of
(28%) (using inhibitors of ALK, MET and BRAF in 14, 1 and 1 patients, anti-PD-1/PD-L1 antibodies. The promotion of CD11b+ CD49b+ NK
respectively). After treatment, 12 patients had follow up cfDNA sam- cell infiltration into the tumor microenvironment may play an impor-
ples; new mutations were detected as disease progressed, enriched for tant role in the anti-tumor immune response induced by immune
the RAS-MAPK (n=6) and ALK (G1202R, n=1) pathways among others. checkpoint inhibition in neuroblastoma.
Conclusions: CfDNA analysis detected genomic aberrations in patients
with HR-NB at relapse. Such analysis permits a relatively noninvasive
profiling of tumor heterogeneity and clonal evolution, with the poten- EP153 / #729 STRUCTURAL DISRUPTION OF BAF
tial to guide therapy. When soft tissue is unavailable or if disease is CHROMATIN REMODELLER IMPAIRS NEUROBLASTOMA
limited to skeletal sites, cfDNA is a viable alternative for studying muta- METASTASIS BY REVERTING AN INVASIVENESS EPIGENOMIC
tions. cfDNA monitoring should be more widely adopted in therapeutic PROGRAM
trials for HR-NB.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S219 of S635

Carlos Jimenez1 , Roberta Antonelli1 , Mariona Nadal2 , Pablo Latorre2 , Swaminathan K1 , Maya Prasad1 , Venkata Rama Mohan Gollamudi1 ,
Laura Devis-Jauregui3 , Carme Solé2 , Marc Masanas1 , Adrià Molero1 , Badira Parambil1 , Shyam Srinivasan1 , Sneha Shah2 , Vasundhara Patil3 ,
Josep Roma1 , Aroa Soriano1 , Josep Sanchez De Toledo4 , David Akshay Baheti3 , Jifmi Manjali4 , Nehal‘ Khanna4 , Siddhartha Laskar4 ,
Llobet-Navas3 , Francesc Posas2 , Eulalia De Nadal2 , Soledad Gallego Pranoti Kini5 , Rajesh Patil5 , Ratna Sharma5 , Mamta Manglani6 , Mukta
Melcón1,5 , Lucas Moreno1,5 , Miguel Segura1 Ramadwar7 , Sajid Qureshi8 , Girish Chinnaswamy1
1 Vall Hebron Research Institute, Childhood Cancer And Blood Disorders, 1 Tata Memorial Hospital, Homi Bhabha National Institute, Pediatric Oncol-

Barcelona, Spain; 2 Institute for Research in Biomedicine, Cell Signaling ogy, Mumbai, India; 2 Tata Memorial Hospital, Homi Bhabha National
Group, Barcelona, Spain; 3 Bellvitge Biomedical Research Institute, Molec- Institute, Nuclear Medicine, Mumbai, India; 3 Tata Memorial Hospital, Homi
ular Mechanisms And Experimental Therapy In Oncology, L’Hospitalet de Bhabha National Institute, Radiodiagnosis, Mumbai, India; 4 Tata Memorial
Llobregat, Spain; 4 Catalan Institute of Oncology, Catalan Institute Of Oncol- Hospital, Homi Bhabha National Institute, Radiation Oncology, Mumbai,
ogy, L’Hospitalet de Llobregat, Spain; 5 Vall d’Hebron University Hospital, India; 5 MCGM-Comprehensive Thalassemia Care, Pediatric Hematology-
Pediatric Oncology And Hematology, Barcelona, Spain Oncology and BMT centre, Bone Marrow Transplant Centre, Mumbai,
India; 6 MCGM-Comprehensive Thalassemia Care, Pediatric Hematology-
Background and Aims: Epigenetic programming during development Oncology and BMT centre, Bone Marrow Transplant Unit, Mumbai, India;
is essential for the determination of cell lineages, and its alteration 7 Tata Memorial Hospital, Homi Bhabha National Institute, Pathology, Mum-

contributes to the initiation of embryonal tumors. In neuroblastoma, bai, India; 8 Tata Memorial Hospital, Homi Bhabha National Institute,
neural crest progenitors block their natural differentiation course into Surgical Oncology, Mumbai, India
sympathoadrenergic cells, leading to an aggressive and metastatic
pediatric cancer. The study of the epigenetic regulators responsible for Background and Aims: Outcomes of High-risk neuroblastoma (HR-
oncogenic epigenomic networks is crucial to develop new epigenetic- NBL) in Lower-Middle Income Countries (LMICs) are adversely
based therapies against these tumors. mSWI/SNF ATP-dependent affected by toxicities, non-availability of(or delay in) Autologous-
chromatin remodeling complexes act genome-wide translating epige- stem cell transplant(Auto-SCT) and immunotherapy. Herein, we study
netic signals into opened chromatin states. Our aim is to understand the clinical profile and outcomes of HR-NBL treated with intensive
the role of mSWI/SNF complexes in the control of the oncogenic protocol without immunotherapy.
epigenomes of neuroblastoma in order to unveil new targets and to Methods: Treatment-naive children with biopsy-proven HR-NBL from
develop new epigenetic-based therapeutic strategies for this pediatric July 2018 to December 2020 were retrospectively analysed. INRG
cancer. stratification was used based on MIBG scan, FDG-PETCT scan,
Methods: Functional characterization of mSWI/SNF complexes in neu- bone marrow studies, N-Myc/SCA reports. Rapid COJEC induc-
roblastoma cells was performed by proteomic, transcriptomic and tion and surgery of primary after achieving metastatic Complete
chromatin accessibility analyses, and the effects of its inhibition on Response/Very Good Partial Response(mCR/VGPR) was followed by
oncogenic features were assessed with in vitro functional assays and Auto-SCT and radiotherapy(RT). If end-induction bone marrow was
neuroblastoma metastasis mouse models. involved, 2 cycles of TVD(topotecan,vincristine,doxorubicin) were
Results: Neuroblastoma cells contain the three main mSWI/SNF sub- administered. Oral maintenance consisting cis-retinoic acid, cyclophos-
types, but only BAF complex disruption through silencing of its phamide, etoposide and celecoxib was administered post RT. Sur-
key structural subunits ARID1A and ARID1B impairs proliferation vival was analysed by log rank test and prognostic factors by cox
by promoting cell cycle blockade. Genome-wide chromatin remod- proportional-Hazard model using SPSS(Version-26).
eling analysis coupled with whole transcriptome data revealed that Results: Among 64 eligible patients, median age was 39months(16-
BAF disruption results in the epigenetic repression of an extensive 128months) with suprarenal primary in majority[42(65.6%)]. Molec-
invasiveness-related expression program, involving integrins, cad- ular data was available for 43 of whom 17(39.5%) had N-Myc
herins and key mesenchymal regulators, thereby reducing extracellular amplification. All but one were metastatic at presentation with
matrix adhesion and invasion in vitro, together with a drastic inhibition bone-marrow, bone, lymph-nodes, lung metastases seen in 47(73%),
of neuroblastoma metastasis initiation and growth in vivo. 26(46%), 3(4.6%), 2(3.1%), respectively. At the end of Rapid COJEC
Conclusions: We define a novel ATPase-independent role for BAF induction, mCR/mVGPR was achieved in 39(60.9%). There were
complex in the maintenance of an epigenomic program that allows neu- 6(9.4%) toxic-deaths, 5(7.8%) progression, 1(1.5%) palliation due to
roblastoma invasiveness and metastasis, urging the need for new BAF poor general condition. Half(7/14) of those who received TVD attained
pharmacological structural disruptors for its therapeutic exploitation mCR. Out of 42 who underwent surgery, 31 achieved gross total
in metastatic neuroblastoma. resection. Thirty-eight(59.4%) underwent Auto-SCT(toxic-death post
Auto-SCT-1). At a median follow-up of 29months (95%CI:9.0-20.99),
3year EFS/OS of the entire cohort was 24±7.2% and 25.2±5.8%, and of
EP154 / #1396 PROGNOSTIC VARIABLES AND OUTCOME those who completed the multi-modality treatment was 32.7±11.1%
OF HIGH RISK NEUROBLASTOMA TREATED IN A TERTIARY and 36.6±8.7% respectively. On multivariate analysis, PET response
CANCER CENTRE IN INDIA (in mCR/mVGPR) [HR:2.9(1.01-8.53,p=0.04)] and gross total surgical
resection[HR:3.1(1.03-9.88,p=0.49)] were prognostic.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S220 of S635 ABSTRACTS

Conclusions: Outcomes of HR-NBL even with a strategy including Conclusions: The long-term prognosis of prenatally diagnosed NB was
Auto-SCT is suboptimal in LMICs. Persistent bone marrow disease excellent. Multidisciplinary discussion since detection of fetal masses
and inadequate metastatic response had a poor outcome in spite of and institutional collaboration are important for organization of timely
intensive therapy. postnatal NB management.

EP155 / #169 CONGENITAL NEUROBLASTOMA: PRENATAL EP156 / #360 OCULAR ABNORMALITIES IN PATIENTS
DIAGNOSIS AND CLINICAL COURSE TREATED WITH AN ANTI-GD2 MONOCLONAL ANTIBODY
DINUTUXIMAB BETA
Anastasiya Salomatina1 , Denis Kachanov1 , Olga Izotova2 , Galina
Tereschenko2 , Dmitry Konovalov3 , Vitaly Roschin3 , Alexander Druy4 , Dinara Utalieva1 , Michael Yadgarov2 , Denis Kachanov1 , Anna
Natalia Andreeva1 , Dinara Utalieva1 , Tatyana Shamanskaya1 Smirnova3 , Igor Khamin4 , Natalia Andreeva1 , Margarita Teleshova1 ,
1 Dmitry Rogachev National Medical Research Center of Pediatric Hematol- Smirnova Lilia1 , Roman Moiseenko1 , Tatyana Shamanskaya1
ogy, Oncology and Immunology, Department Of Clinical Oncology, Moscow, 1 Dmitry Rogachev National Medical Research Center of Pediatric Hematol-

Russian Federation; 2 Dmitry Rogachev National Medical Research Cen- ogy, Oncology and Immunology, Department Of Clinical Oncology, Moscow,
ter of Pediatric Hematology, Oncology and Immunology, Department Of Russian Federation; 2 Dmitry Rogachev National Medical Research Center of
Radiology, Moscow, Russian Federation; 3 Dmitry Rogachev National Med- Pediatric Hematology, Oncology and Immunology, Department Of Nuclear
ical Research Center of Pediatric Hematology, Oncology and Immunology, Medicine, Moscow, Russian Federation; 3 Dmitry Rogachev National Med-
Department Of Pathology, Moscow, Russian Federation; 4 Dmitry Rogachev ical Research Center of Pediatric Hematology, Oncology and Immunology,
National Medical Research Center of Pediatric Hematology, Oncology Consultation Department, Moscow, Russian Federation; 4 Dmitry Rogachev
and Immunology, Laboratory Of Molecular Oncology, Moscow, Russian National Medical Research Center of Pediatric Hematology, Oncology and
Federation Immunology, Intensive Care Unit, Moscow, Russian Federation

Background and Aims: Neuroblastoma (NB) is the most common Background and Aims: The aim was to analyze the incidence and the
neonatal malignant solid tumor and amounts to 26% of all prenatally type of ocular abnormalities (OA) in high-risk (HR) neuroblastoma (NB)
diagnosed neoplasms (Isaacs, 2002). The aim was to analyze NB cases patients treated with dinutuximab beta (DTB).
detected in utero and to characterize the disease course and therapy Methods: The medical records of 21 patients who received
outcomes. immunotherapy for HR NB were retrospectively analyzed. 16
Methods: 29 patients with a diagnosis of NB first suspected during (76.2%) patients received DTB in front-line settings, 5 (23.8%) at
the antenatal ultrasound examination for the period 01.2012-07.2021 relapse/progression. All patients underwent a comprehensive oph-
(115 months) were included in the analysis. The diagnosis was made thalmologic examination prior to each course of therapy. At the start
according to the international criteria. Staging was according to INSS, of DNB, 7 patients had OA, where 2/7 wore bifocal reading glasses.
therapy was carried out per the modified German NB2004 protocol. Survival analysis was performed using the Kaplan-Meier method with
Database was locked on 01.07.2021. appearance OA as an event.
Results: The male:female ratio was 0.8:1. The median gestational age Results: Median age at diagnosis of NB was 54 months (range 25–
at the time of the first pathological changes detection (n=25) was 36 119). Male:female ratio – 0.75:1. OA observed in 13/21 (61.9%) cases.
weeks (range 28-40). Adrenal/retroperitoneal masses were detected 3/13 (23%) patients were prescribed bifocals reading glasses for the
in 20/29 (69%), abdominal masses – 7/29 (24%), other topography first time. Following abnormalities were noted: impaired accommo-
– 2/29 (7%). Median age of NB diagnosis was 1.9 months (range dation (n=9), mydriasis (n=3), impaired photoreaction (n=3), anisoco-
0.4-12.7). Most cases were detected before 3 months of age (21/29, ria (n=2), cycloplegia (n=1), no photoreaction (n=1). Patients might
72.4%). In 10 patients (34.5%) of this group, the diagnosis was ver- have several ocular abnormalities at the same time. Age (58 months
ified in the first month of life. Primary tumor topography included (interquartile range (IQR) 43–84) with versus 51 months (IQR 37–
adrenal gland (26/29, 89.7%, among them 8 cases had bilateral involve- 58) without OA, p=0.336) and sex (8/12 (66.7%) females versus 5/9
ment), retroperitoneal space (1/29, 3.4%), posterior mediastinum (55.6%) males, p=0.673) were not associated with the incidence of OA.
(2/29, 6.9%). MYCN was nonamplified in all detected cases (26/26). Patients who had ocular pathology prior to therapy with DTB were
Stage distribution: stage 1 - 17/29 (58.6%), there was one case of stages 4 times less likely to develop ophthalmologic toxicity (0/4 (0.0%) ver-
2, 3 and 4, stage 4S - 9/29 (31.0%). Patients were stratified to low sus 13/17 (76.5%), p = 0.012, odds ratio – 0.24 (0.10–0.55). Based on
(28/29, 96.6%), and intermediate (1/29, 3.4%) risk groups. 6 patients Kaplan-Meier method OA developed in 50% of cases after 36.0 days
in the low-risk group with stage 4S were treated with chemotherapy. from the start of DTB treatment. OA were detected between 2-40 days
Relapse/progression was detected in 3/29 (6.9%) cases. The median of therapy. OA developed in 50% of cases after receiving a total dose of
follow-up period was 46.9 months (range 3.5-99.6). All patients were 128 mg. In our study, no dose-related effect was found between DTB
alive. and OA.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S221 of S635

Conclusions: Ocular abnormalities are a common complication of DTB. Ilya Kazantsev1 , Asmik Gevorgian2 , Tatiana Jukhta1 , Andrey Kozlov1 ,
Careful monitoring of patients by an ophthalmologist is recommended. Polina Tolkunova1 , Margarita Halipskaya1 , Olga Bogdanova1 ,
Alexander Galimov1 , Tatiana Andreeva1 , Nina Subora1 , Olesya
Yudintseva1 , Polina Kuga1 , Alexander Shvetsov1 , Svetlana Safonova1 ,
EP157 / #388 ‘ULTRA-HIGH-RISK’ NEUROBLASTOMA Yuri Punanov1 , Ludmila Zubarovskaya3
SUBGROUP: A TOOL TO PREDICT SURVIVAL IN CHILDREN 1 RM Gorbacheva Research Institute Pavlov University, Pediatric Oncology,
WITH NEWLY DIAGNOSED HIGH-RISK NEUROBLASTOMA Saint-Petersburg, Russian Federation; 2 RM Gorbacheva Research Institute
of Pavlov University, Pediatric Oncology, Saint-Petersburg, Russian Fed-
Mikhail Yadgarov1 , Nikolai Matveev2 , Chaurasiya Kailash1 , Tatyana eration; 3 Pavlov University, R. M. Gorrbacheva Research Institute, Saint
Shamanskaya3 , Denis Kachanov3 , Yury Likar1 Petersburg, Russian Federation
1 Dmitry Rogachev National Research Center of Pediatric Hematology,
Oncology and Immunology, Department Of Pet And Radionuclide Diag- Background and Aims: The long-term results in patients with high-risk
nostics, Moscow, Russian Federation; 2 Russian National Research Medical neuroblastoma (HR NB) are still unsatisfactory, especially if primary
University, Department Of Medical Cybernetics And Informatics, Moscow, resistant or relapse (rrNB) develop. While there is a strong body of
Russian Federation; 3 Dmitry Rogachev National Medical Research Center evidence supporting the use of anti-GD2 antibodies in patients with
of Pediatric Hematology, Oncology and Immunology, Department Of Clinical primary HR NB, it is less commonly used in rrNB cases. Although
Oncology, Moscow, Russian Federation immunotherapy is not yet incorporated into local standard, it was
used within pilot clinical trials recruiting autologous (auto-HSCT) and
Background and Aims: The International Neuroblastoma Risk Group haploidentical hemopoietic stem cell transplantation (haplo-HSCT)
Staging System (INRGSS) and the NB-2004 protocol are important recipients.
tools for risk assessment in patients with neuroblastoma (NB). In Methods: A total of 23 patients with a median age of of 3 (1-15)
recent years, many research groups have attempted to define a years at diagnosis with HR NB (n=19) or rrNB (n=4) treated in RM
subgroup of ‘ultra-high risk’ patients in order to apply alternative ther- Gorbacheva Research Institute were included. All primary patients
apeutic approaches to such patients, but research results are still were initially treated according to national guidelines (modified GPOH
insufficient. The aim of this study was to develop widely available tool NB2004 protocol) reaching complete (CR; n=16) or very good par-
for use at the time of diagnosis to identify a subgroup of ‘ultra-high risk’ tial response (VGPR; n=3). Among 4 patients with rrNB 2 were in CR
patients with NB at risk for very poor outcome. or VGPR, while other 2 achieved stable disease (SD) prior to haplo-
Methods: This retrospective single-centre study included 107 high- HSCT. Immunotherapy consisted of 5 dinutuximab beta cycles (100
risk patients (56 boys, median age 28.4 months, range 1-169 months) mg/m2 each) via prolonged 10-day infusion. One patient with SD it also
with newly diagnosed NB. Cox regression analysis was used to build a received anti-PD1 antibodies (nivolumab).
predictive model. Based on the results of the ROC analysis, the qual- Results: With a median follow-up of 20 months the 2-year EFS in
ity of the model was assessed in a three-stage cross-validation with the auto-HSCT recipients was 78%, which is much better compared to
calculation of the sensitivity/specificity ratio. similar cohort of 96 patients treated in 2008-2019 (p=0.02). Both
Results: Three-year event-free survival was 39.9±5.1%, three-year patients with rrNB achieving response prior to haplo-HSCT are alive
overall survival - 64.7±5.0%. In multivariable regression analysis, fer- and disease-free 18 and 28 months post-transplant. One patient with
ritin levels ≥210 μg/L (risk ratio (RR): 1.78, score 10 if true), the SD died due to disease progression, another relapsed after a long-term
presence of a 1p deletion (RR: 2.08, score: 6), and bone marrow metas- (55 months) response to combined immunotherapy. Immunotherapy
tasis (RR: 2.70, score: 5.5) were significant predictors of an unfavorable was well tolerated in all but 2 cases, in which it was ceased prema-
outcome. The patient is classified in a ‘ultra-high risk’ subgroup if the turely due to neurotoxicity development (both patients still retain the
total score (sum of scores for each predictor) is ≥7. The developed tool response).
was characterized by an acceptable prognostic quality (AUC = 0.768, Conclusions: Immunotherapy provides reproducible results in auto-
sensitivity - 68.3%, specificity - 70.2%). The predictive value of the HSCT recipients. It may also be a feasible post-consolidation strat-
model was stable during validation (p<0.05 at each stage of validation). egy in NB patients with rr NB, although larger cohort studies are
Conclusions: The developed tool for identifying an additional sub- needed.
group of ‘ultra-high risk’ patients with neuroblastoma can be used by
oncologists in clinical practice.
EP159 / #912 LATE COGNITIVE AND ADAPTIVE OUTCOMES
OF PATIENTS WITH NEUROBLASTOMA-ASSOCIATED
EP158 / #1401 IMMUNOTHERAPY AFTER AUTOLOGOUS OR OPSOCLONUS-MYOCLONUS-ATAXIA-SYNDROME: A REPORT
ALLOGENEIC HEMOPOIETIC STEM CELL TRANSPLANTATION IN FROM THE CHILDREN’S ONCOLOGY GROUP (COG)
PATIENTS WITH HIGH-RISK NEUROBLASTOMA, A SINGLE
CENTER EXPERIENCE Prerna Kumar1 , Victoria Willard2 , Leanne Embry3 , Arlene Naranjo4 ,
Brian Labarre4 , Katherine Matthay5 , Pedro Dealarcon1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S222 of S635 ABSTRACTS

1 University of Illinois College of Medicine Peoria, Pediatrics, Peoria, United 1 Princess Maxima Center for Pediatric Oncology, Research, Utrecht, Nether-

States of America; 2 St Jude Children’s Research Hospital, Psychology, lands; 2 Faculty of Veterinary Sciences, Biomolecular Health Sciences,
Memphis, United States of America; 3 UT Health San Antonio, Pediatrics, Utrecht, Netherlands; 3 Sanquin Research, Experimental Immunohematol-
San Antonio, United States of America; 4 University of Florida, Biostatis- ogy, Amsterdam, Netherlands
tics, Gainesville, United States of America; 5 University of California San
Francisco, Pediatrics, San Francisco, United States of America Background and Aims: Liquid biopsies have been studied as diagnostic
modalities in patients with neuroblastoma, focusing mostly on detec-
Background and Aims: Opsoclonus-myoclonus-ataxia syndrome tion of circulating tumor cells and cell-free DNA (cfDNA) from plasma.
(OMAS) is a rare autoimmune disorder of the nervous system that However, these approaches have their specific limitations. Plasma con-
presents with abnormal eye and limb movements, altered gait, and tains more particles that represent potential biomarkers, including
increased irritability. Between 2-4% of children diagnosed with cell-free RNA (cfRNA) and extracellular vesicles (EVs), which are shed
neuroblastoma have neuroblastoma-associated OMAS (NA-OMAS). by every cell in the body. We investigated the potential of cfRNA detec-
Children with NA-OMAS typically present with low-risk disease tion from plasma of patients with neuroblastoma, and whether these
that is cured with surgery alone or surgery and less intensive targets are associated to EVs.
chemotherapy. Despite excellent overall survival, however, patients Methods: We isolated cfRNA from 200ul of plasma from 20 healthy
with NA-OMAS can have persistent significant neurological deficits. controls and from 40 patients with neuroblastoma (10 with localized
COG protocol ANBL00P3, a randomized prospective therapeutic and 30 with metastatic disease). We performed droplet digital PCR
clinical trial for patients with NA-OMAS, demonstrated that the (ddPCR) for neuroblastoma-specific genes (PHOX2B, TH, CHRNA3),
addition of intravenous immunoglobulin (IVIG) to prednisone and genes involved in cell cycle regulation (E2F1, CDC6, ATAD2, DHFR,
risk-adapted chemotherapy significantly improved the one year post- H2AFZ, MCM2) and potential cfRNA reference genes (GUSB, B2M and
diagnosis response rate. Herein we report the evaluation of long-term HPA1a/b) using 4 multiplex assays. cfDNA was also analyzed by ddPCR
neurocognitive and adaptive functioning. for methylated RASSF1A and ACTIN beta. EVs were isolated by size
Methods: Of 53 children enrolled on ANBL00P3, 29 submitted evalu- exclusion chromatography (SEC).
able neurocognitive data at diagnosis and additional scheduled time Results: Thirteen out of 30 samples from patients with metastatic
points and were included in the analyses. Adaptive development was disease were positive for at least one neuroblastoma-specific gene
assessed via the Vineland Adaptive Behavior Scale, and validated, age- in cfRNA, all patients with localized disease were negative. The cell
appropriate tools including the BSID, WPPSI-R, WISC-III were used to cycle genes were expressed in both healthy controls as well as in
assess neurocognitive function. patients, and were not significantly overexpressed, except for DHFR
Results: Eighteen patients were treated with chemotherapy and IVIG which was higher in metastatic disease. Analysis of EVs showed pres-
(IVIG+). The remaining eleven received chemotherapy only. Descrip- ence of the neuroblastoma-specific genes and the cell cycle genes in
tive spaghetti plots suggest that treated patients, regardless of group, the EV-enriched SEC fractions in patients’ plasma. Interestingly, cfDNA
demonstrated relatively stable cognitive functioning and stable to was mostly concentrated in the non-EV SEC fractions.
improved adaptive development over time. Descriptively, the IVIG+ Conclusions: We explore the possibilities of different cfRNA markers
group demonstrated greater improvements in adaptive development from plasma as novel biomarkers in neuroblastoma and we demon-
compared to the chemotherapy-only group. strate that these mRNA markers are mostly concentrated in EV-
Conclusions: Study participants appear to demonstrate stable cogni- enriched SEC fractions. This study forms a starting point for further
tive functioning and stable to improved adaptive functioning over time. investigations into mRNA from plasma as biomarker.
The addition of IVIG to a chemotherapy-only regimen may offer some
additional benefit by further improving long term adaptive outcomes.
While statistical significance is limited by small sample size, findings EP161 / #1776 MAINTENANCE WITH NAXITAMAB
suggest that aggressively treating NA-OMAS may be associated with COMBINED WITH GRANULOCYTE-MACROPHAGE
improved long-term cognitive and adaptive functioning as compared to COLONY-STIMULATING FACTOR AND INTRATHECAL
historical cohorts. TOPOTECAN FOR METASTATIC RETINOBLASTOMA

Cristina Larrosa1 , Juan Pablo Muñoz Perez1 , Margarida Simao


EP160 / #660 CELL-FREE MRNA FROM PLASMA IS Rafael1 , Ana José Navarro1 , Maite Gorostegui1 , Jaume Catala Mora2 ,
ENRICHED IN EXTRACELLULAR VESICLES IN PATIENTS WITH Jaume Mora1,3 , Guillermo Chantada1,4,5,6
NEUROBLASTOMA 1 Pediatric Cancer Center Barcelona, Hospital Sant Joan de Déu, Pediatric
Oncology, Barcelona, Spain; 2 Hospital Sant Joan de Deu, Ophthalmology,
Nathalie Lak1 , Anne Seijger2 , Lieke Van Zogchel1 , Nina Gelineau1 , Esplugues de Llobregat, Spain; 3 Institut de Recerca Sant Joan de Deu, Pedi-
Ahmad Javadi3 , Lily Zappeij-Kannegieter3 , Laura Bongiovanni2 , atric Cancer, Sarcomas And Histiocitosis Group, Esplugues de Llobregat
Anneloes Andriessen2 , Janine Stutterheim1 , C. Ellen Van Der Schoot3 , (Barcelona), Spain; 4 Hospital Austral, Pediatric Oncology, Pilar, Argentina;
Alain De Bruin2 , Lieve Tytgat1 5 National Scientific and Technical Research Council (CONICET), Oncology,
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ABSTRACTS S223 of S635

Buenos Aires, Argentina; 6 Hospital Austral, Department Of Translational Background and Aims: Urinary catecholamine metabolites play a
Pediatric Oncology, Pilar, Argentina role as diagnostic and prognostic biomarker in neuroblastoma. Two
neuroblastoma-specific cell states are defined, namely noradrenergic
Background and Aims: Metastatic retinoblastoma (RB) requires (NOR) and mesenchymal (MES), with possible different catecholamine
intensive chemotherapy due to its poor prognosis, which may lead excretion profiles. In this study we investigated the biology of cate-
to severe complications and/or second neoplasms in patients with cholamine excretion in vivo and in vitro.
germline RB1 mutation, highlighting the need of safer and more effec- Methods: Catecholamine metabolites were measured in 73 high-
tive therapies. Disialoianglioside GD2 is highly expressed in most risk patients and in culture medium of 26 neuroblastoma cell lines.
RB, consequently, anti-GD2 monoclonal antibodies, responsible for The presence of catecholamine enzymes in neuroblastoma cell lines
increasing survival for high-risk neuroblastoma, may be a therapeutic and primary tumors was examined with qRT-PCR, western blot and
option. immunohistochemistry (IHC). Additionally, catecholamine excretion
Methods: Report of two cases with metastatic RB who received five was compared in 3 matched NOR- and MES neuroblastoma cell lines
cycles of subcutaneous (SC) GM-CSF for 5 days at 250 μg/m2 /day and tested after overexpressing TFAP2B and PRRX1.
(days -4 to 0) and 500 μg/m2/day (days 1-5), and naxitamab infused Results: 71 out of 73 patients had at least one elevated metabolite
over 30 minutes at 3 mg/kg/day, days 1, 3, and 5, outpatient. Intrathecal at diagnosis (sensitivity 97%). In 18 out of 30 patients with relapse
topotecan was administered at 0.4 mg/dose for 6 cycles. urinary data was collected, of whom 5 patients where catecholamine
Results: Two patients (“A” and “B”) referred to our center at the age negative (28%). Catecholamine metabolite levels were detected in the
of 18 months and 5 years respectively with bilateral retinoblastoma. medium of 8/26 of the tested cell lines, which was related to the
A systemic metastatic relapse was detected 6 years after diagnosis presence of the enzyme tyrosine hydroxylase (TH). TH protein was
consisting of bone marrow and liver metastasis (“A”) and bone mar- present in all the excreting and absent in all the non-excreting cell
row and minimally disseminated disease in the CNS (qPCR positive lines. IHC staining for TH in primary tumors showed an identical pat-
for CRX on CSF) (“B”). Previous treatment included local (intravitreal tern with all catecholamine positive patients being TH positive (n =
melphalan, tandem intra-arterial topotecan-melphalan, thermother- 70), whereas all catecholamine negative patients were TH negative
apy) and systemic cytostatic therapy (>10 cycles), enucleation of one (n = 3). Finally, catecholamine excretion was observed in all NOR cell
(“A”)/both eyes (“B”) and, in “A”, intrathecal topotecan due to CNS lines (3/3), but absent in all MES cell lines (0/3). Overexpression of
involvement. Both patients received COG-0321-based chemotherapy PRRX1 induced mesenchymal transition and stopped catecholamine
but in patient “A”, a severe hypersensitivity to Cisplatin occurred and, in excretion. TFAP2B overexpression restored catecholamine excretion
“B”, ototoxicity, limiting the use of platinum derivatives. After achieving in NOR non-excreting cell line, but not in a MES cell line.
complete remission, patients underwent consolidation with high-dose Conclusions: Catecholamine negative phenotype is due to downregu-
chemotherapy with autologous stem cell rescue followed by mainte- lation of TH, which might be dictated to the fine balance between NOR-
nance with naxitamab and GM-CSF plus intrathecal topotecan in “B”. and MES cell types.
No major toxicities were reported. Both patients continue without
evidence of disease 20 and 26 months after extraocular relapse.
Conclusions: Naxitamab and GM-CSF maintenance +/- Intrathecal EP163 / #1679 IRINOTECAN AND TEMOZOLOMIDE
topotecan were safe and well tolerated and may become potential ACTIVITY FOR RELAPSED/PROGRESSIVE NEUROBLASTOMA
therapeutic tools for high-risk retinoblastoma. DURING NAXITAMAB IMMUNOTHERAPY

Juan Pablo Muñoz Perez, Maite Gorostegui, Amalia Varo Rodríguez,


EP162 / #278 CATECHOLAMINE-NEGATIVE Alicia Castañeda Heredia, Moira Garraus, Vicente Santa-María,
NEUROBLASTOMA PATIENTS: THE ROLE OF Margarida Simao Rafael, Jaume Mora
NORADRENERGIC-TO-MESENCHYMAL TRANSITION? Hospital Sant Joan de Déu, Pediatric Oncology, Barcelona, Spain

Yvette Matser1 , Iedan Verly2 , André Van Kuilenburg2 , Dilara Background and Aims: Anti-GD2 monoclonal antibodies are effec-
Savci-Heijink3 , Rutger Meinsma2 , René Leen2 , Tim Van Groningen4 , tive in preventing relapse in patients with high-risk neuroblastoma
Jan Koster4 , Matthias Fischer5 , Rogier Versteeg4 , Johan Van Nes4 , (HR-NB) in complete remission and show objective responses (OR)
Lieve Tytgat1 in relapsed/refractory HR-NB patients alone or combined (chemo-
1 Prinses Maxima Centrum, Pediatric Oncology, Utrecht, Netherlands; immunotherapy) with Irinotecan and Temozolamide (I/T). We hypothe-
2 Amsterdam University Medical Center, Department Of Clinical Chem- sized that relapsed/progressive neuroblastoma under naxitamab treat-
istry, d, Netherlands; 3 Amsterdam University Medical Center, Pathology, ment may respond better to rescue I/T (chemo-immunotherapy effect)
d, Netherlands; 4 Amsterdam University Medical Center, Oncogenomics, d, than historical cohorts from the pre-immunotherapy era.
Netherlands; 5 University of Cologne, Center For Molecular Medicine, d, Methods: This is a single-institution retrospective review of all patients
Netherlands with relapse or progressive HR-NB during treatment with naxita-
mab treated between June 2017 and January 2022 and rescued
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S224 of S635 ABSTRACTS

with 2 cycles of irinotecan (50 mg/m2/day x5) and temozolomide Results: Forty-five patients were included, 32 (71.1%) had received
(150 mg/m2/day x5). Disease response was assessed by revised INRC “induction only” and 13 (28.9%) “induction plus maintenance CADO”.
criteria using 123I-MIBG SPECT/CT and four bone marrow aspirates. The median age at diagnosis for the “induction only” and “CADO main-
Results: From June 2017 to January 2022, 165 patients received naxi- tenance” group were 49.5 and 52 months respectively (p=0.9). Both
tamab based immunotherapy and forty-three relapsed or progressed groups compared similarly on sex, primary tumour site, LDH, fer-
during treatment. Nineteen received I/T as recue, 12 because of ritin, stage and MYCN. The total number of cycles administered in
relapse (nine first relapse; two second relapse; and one fourth relapse) the “CADO maintenance” group was 99 cycles (range 1-11, median of
and seven with refractory disease and progression. Median age at the 11). During the CADO cycles, only one neutropenic fever, one non-
time of relapse or progression was 5.9 years (range: 3-15.8). Median neutropenic fever and one platelet transfusion were documented. The
number of naxitamab cycles received before I/T was four (range: 2- median survival times for the “CADO maintenance” group and the
7). Four patients had MYCN amplified tumors. Relapse or progression “induction only” group were 38.5 and 12.6 months (p=0.5) respectively
was isolated to bone in sixteen patients; bone and soft tissue in three while the 2-year OS rates were 46.2% and 34.3% respectively (p=0.5).
including one with bone marrow involvement. Ten patients had ≥ 3 The five-year OS for both groups was <5.0%.
lesions. Five (26.3%) patients presented OR (4 complete and 1 partial Conclusions: In settings where HR-NB therapies are limited, mainte-
response) after two I/T cycles. Thirteen patients had stable disease and nance CADO appears to offer increased survival benefits with minimal
one progressive disease. toxicity during palliation. Studies of larger cohorts to evaluate CADO
Conclusions: The OR rate with I/T in our series compares favorably during HR-NB are recommended.
to studies where I/T is used without immunotherapy suggesting anti-
GD2 mAb as a mechanism of potentiating chemotherapy even when
administered sequentially. EP165 / #749 CLINICOPATHOLOGICAL IMPLICATIONS OF
GANGLIONEUROBLASTOMA INTERMIXED

EP164 / #1760 EVALUATING CADO FOR PALLIATION IN A Behtash Nezami1 , Mohammed Alghamdi1 , Fiorella Iglesias Cardenas2 ,
LOW-INCOME SETTING AMONG UGANDAN CHILDREN Judy Sarungbam1 , Sahussapont Sirintrapun1 , Anuradha Gopalan1 ,
DIAGNOSED WITH NEUROBLASTOMA Yingbei Chen1 , Hikmat Al-Ahmadie1 , Samson Fine1 , Victor Reuter1 ,
Shakeel Modak2 , Satish Tickoo1
Irene Nanyanga1 , Joyce Kambugu1 , Barnabas Atwiine2,3 , Ruth 1 Memorial Sloan Kettering Cancer Center, Pathology, New York City, United

Namazzi4 , Jaques Van Heerden1,5 States of America; 2 MSKCC, Pediatrics, New York, United States of America
1 Uganda Cancer Institute, Department Of Paediatric Oncology, Kampala,
Uganda; 2 Mbarara University of Science and Technology, Paediatrics And Background and Aims: International Neuroblastoma Risk Group
Child Health, Mbarara, Uganda; 3 Mbarara Regional Referral Hospital, Pae- (INRG) consensus pretreatment classification schema stratifies
diatrics And Child Health, Mbarara, Uganda; 4 Makerere University College patients into low, intermediate, and high-risk using the patient age,
of Health Sciences, Mulago Hospital, Department Of Paediatrics And Child clinical stage, histology, 11q aberrations and amplification of MYCN
Health, Kampala, Uganda; 5 Antwerp University Hospital, Department Of gene. We studied patients with the primary pathologic diagnosis of
Paediatric Oncology, Antwerp, Belgium Ganglioneuroblastoma Intermixed (GNBI) - regarded as a tumor with
“favorable” histology by International Neuroblastoma Pathology Clas-
Background and Aims: In low-income settings, therapies are often not sification (INPC) system - to evaluate the correlation of this diagnosis
available during high-risk (HR) neuroblastoma (NB) management and with the clinicopathological features.
treatment is with non-curative intent. Globally the CADO treatment Methods: Retrospective search of the archives of the pathology
protocol (oral cyclophosphamide 50 mg/m2 , IV doxorubicin 35 mg/m2 , department of our institution between 1995 and 2021 revealed 15827
IV vincristine 1.5 mg/m2 ) has been used for palliative purposes in HR- specimens from 2805 patients. The term “Ganglioneuroblatoma Inter-
NB. Our study aimed to determine the value of maintenance CADO mixed” was found in 237 cases. Of the 237 cases, 57 had initial
after induction with OPEC/OJEC on outcomes in Uganda. diagnosis of GNBI. The clinical and follow up data (median 3.3 years,
Methods: A multicentre, retrospective chart review of children range 0.2-13.7), and MYCN amplification status were available for all
younger than 16 years diagnosed with HR-NB between January patients; targeted exome sequencing data was available in 23 patients.
2010 to November 2020, and treated with either OPEC/OJEC Clinical stage was determined using clinical/radiological (including
induction chemotherapy alone or with maintenance CADO in addi- MIBG scanning) data and pathological confirmation.
tion to the induction, was conducted. Clinical characteristics at Results: 25% (14/57) of patients with primary GNBI had metastatic
diagnosis and cytopaenias, numbers of blood product transfusions disease at presentation (INRG stage M). 50% (7/14) of patients with
and infections during CADO, to evaluate treatment toxicity, were metastatic GNBI relapsed, as opposed to 5% (2/43) in locoregional dis-
documented. Kaplan-Meier survival curves were used to deter- ease. MYCN gene amplification was only detected in metastatic group
mine the median survival time and two-year overall survival (OS) (n=2); one of these relapsed. Primary tumors of the abdomen were
rates. more likely to relapse than those of thorax (8 vs. 1 case). Somatic
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ABSTRACTS S225 of S635

alterations were detected in 8 patients (represented in both the EP167 / #1786 DINUTUXIMAB BETA-BASED
groups). CHEMO-IMMUNOTHERAPY FOR RELAPSED/REFRACTORY
Conclusions: Localized GNBI at diagnosis has excellent long-term clin- HIGH-RISK NEUROBLASTOMA: PRELIMINARY RESULTS
ical outcome. However, a proportion of cases with this pathologic
diagnosis have metastases at the time of initial diagnosis. Therefore, Nur Olgun1 , Emre Çeçen1 , Dilek Ince1 , Deniz Kizmazoğlu1 , Birsen
rendering “favorable” histology in the pathology reports of primary Baysal1 , Ayşe Önal1 , Özhan Özdoğan2 , Handan Güleryüz3 , Riza
GNBI in the absence of concurrent clinical and radiologic data may not Çetingöz4 , Ayşe Demiral4 , Mustafa Olguner5 , Ahmet Çelik6 , Serra
reflect the true nature of disease. Very few patients with the initial Kamer7 , Erdener Özer8 , Zekiye Altun9 , Safiye Aktaş9
diagnosis of GNBI show MYCN amplification. 1 Dokuz Eylul University Institute of Oncology, Pediatric Oncology, IZMIR,
Turkey; 2 Dokuz Eylul University, Nuclear Medicine, İZMİR, Turkey; 3 Dokuz
Eylul University, Radiology, İZMİR, Turkey; 4 Dokuz Eylul University, Radi-
EP166 / #1655 INTEGRATIVE ANALYSIS OF FEN1 ation Oncology, İZMİR, Turkey; 5 Dokuz Eylul University, Pediatric Surgery,
EXPRESSION AND PROGNOSTIC IMPACT IN NEUROBLASTOMA İZMİR, Turkey; 6 Ege University, Pediatric Surgery, BORNOVA, Turkey;
7 Ege University, Radiation Oncology, BORNOVA, Turkey; 8 Dokuz Eylul
Hongling Yuan1 , Zekiye Altun1 , Nur Olgun2 University Faculty of Medicine, Pathology, IZMIR, Turkey; 9 Dokuz Eylul
1 Dokuz Eylul University Institute of Oncology, Basic Oncology Department, University Institute of Oncology, Basic Oncology Department, IZMIR,
IZMIR, Turkey; 2 Dokuz Eylul University Institute of Oncology, Pediatric Turkey
Oncology, IZMIR, Turkey
Background and Aims: Relapsed or refractory (R/R) high-risk (HR)
Background and Aims: Neuroblastoma-NB is the most common child- neuroblastoma (NB) has a dismal prognosis. Anti-GD2-mediated
hood solid tumor. Despite advances in diagnoses and treatments, the chemo-immunotherapy has a considerable anti-tumor activity in
prognosis of NB still remains poor. FEN1-Flap endonuclease-1, involves patients with R/R HR-NB. In the present study, we purposed to
in DNA replication, long-patch excision repair, and telomere mainte- investigate the impacts and adverse effects of the combination of
nance. FEN1 overexpression has been reported to be associated with immunotherapy with dinutuximab beta (DB) and chemotherapy in
the different types of cancers and chemotherapy resistance. It might patients with R/R HR-NB.
be a predictive biomarker and therapeutic target in multiple cancers. Methods: Patients of over 12 months with documentation of a HR-
However, the role of FEN1 in NB has not been thoroughly investigated NB diagnosis were eligible at relapse or designation of refractory
now. disease status. Inclusion criteria were as follows: relapsed or refrac-
Methods: NB mRNA expression data was downloaded from UCSC tory, measurable by contrast-enhanced magnetic resonance imaging
XENA. Survival analysis of FEN1 expression were performed using (MRI) and/or computed tomography (CT) or metaiodobenzylguani-
Kaplan Meier. In addition, FEN1 expression data were also extracted dine (MIBG)/ fluorodeoxyglucose (FDG) positron emission tomog-
from Pediatric Neuroblastoma (TARGET, 2018) database. Survival raphy (PET)/CT evaluable disease and/or demonstrated by bone
analysis was performed using cBioPortal. Through STRING tool to marrow aspiration and biopsy. Chemotherapy scheme was irinote-
determine FEN1 binding proteins and based on interacted proteins, can (IV, 50 mg/m2 per dose, on Days 0-4) and temozolomide (PO,
Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes 100 mg/m2 per dose, on days 0-4). Dinutuximab beta was adminis-
(KEGG) enrichment analysis was performed. tered intravenously for 10 days through continuous infusion with 10
Results: 85 NB samples were selected from GDC TARGET-NBL mg/m2 per day (on Days 1-10). The patients received 2 to 12 suc-
dataset. Most of NB patients belonged to high risk group, INSS stage cessive cycles with duration of 28 days each. Disease assessment
4 and 4s with unfavorable histology. Kaplan Meier analysis showed was performed after cycles 2, 4, and 6 and every 2 to 3 cycles
higher FEN1 expression was correlated with poor survival outcomes thereafter.
(p<0.05). FEN1 expression exhibited significant positive association Results: Between January 2020 and January 2022, nineteen (n=19)
with CDCA5 expression(r=0.9). Its overexpression was also related patients received a total of 116 cycles of DB+CT. Objective (com-
with poor survival outcomes(p<0.05) in NB. KEGG analysis suggested plete or partial) responses were achieved in 12/19 (63%) patients,
FEN1 mainly participated in the cell cycle, DNA replication and homol- including complete remission (CR) in 6/19 and partial response (PR)
ogous recombination function. In addition, FEN1 mRNA expression in 6/19. Stable disease was observed in two patients. The remaining
data from 139 TARGET NB patients were also analyzed using cBio- five patients developed bone/bone marrow and soft tissue progres-
Portal, which confirmed FEN1 higher expression was also related with sion after 2-4 cycles of treatment. The most common side effect
poor survival in NB(p<0.05). was fever, which was more common in the first cycles of treatment.
Conclusions: Our study detected that the expression level of FEN1 was Grade ≥3 toxicities were leukopenia (62%), thrombocytopenia (27%),
elevated in NB. Higher FEN1 expression was found to be correlated hypertransaminasemia (25%), fever (14%), and rash/itching (11%),
with poor overall survival. FEN1 may be an independent prognostic respectively.
factor for NB. Further studies are required to explore the relative Conclusions: DB-based chemo-immunotherapy is suitable leading to
mechanisms in NB and the relationship between FEN1 and CDCA5. an encouraging response rate in patients with R/R HR-NB
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S226 of S635 ABSTRACTS

EP168 / #1257 THE OPTIMAL USE AND EFFECTIVENESS OF Jörg Otte1 , Thale Olsen1 , Shenglin Mei2 , Polina Kameneva3 , Bethel
131I-META IODOBENZYLGUANIDINE (131I-MIBG) MOLECULAR Tesfai Embaie1 , Åsa Björklund4 , Anna Johansson4 , Erik Sundström5 ,
RADIOTHERAPY FOR HIGH-RISK AND RELAPSED/REFRACTORY Tommy Martinsson6 , Susanne Fransson6 , Jakob Stenman1 , Huaitao
NEUROBLASTOMA Cheng7 , Vasilios Zachariadis7 , Martin Enge7 , John Inge Johnsen1 , Per
Kogner1 , Igor Adameyko3 , Peter Kharchenko2 , Ninib Baryawno1
Marta Osuna Marco1 , Laura Martín López1 , Isabel Plaza De Las 1 Karolinska Institutet, Department Of Women’s And Children’s
Heras2 , Marta Villa Alcázar1 , Blanca López-Ibor1 Health, Stockholm, Sweden; 2 Harvard Medical School, Department Of
1 HM HOSPITALS/CIOCC, Pediatric Hematology And Oncology Unit, Boad- Biomedical Informatics, Boston, United States of America; 3 Karolinska
illa del Monte, Madrid, Spain; 2 HM HOSPITALS/CIOCC, Nuclear Medicine, Institute, Department Of Physiology And Pharmacology, Solna, Swe-
Madrid, Spain den; 4 SciLifeLab, National Bioinformatics Infrastructure Sweden,
Uppsala, Sweden; 5 Karolinska Institute, Department Of Neurobiol-
Background and Aims: 131 I-mIBG therapy, originally used for refrac- ogy, Care Sciences And Society, Huddinge, Sweden; 6 University of
tory/relapsed neuroblastoma or for palliative bone pain, is being Gothenburg, Department Of Laboratory Medicine, Göteborg, Sweden;
incorporated into induction and consolidation treatments. 7 Karolinska Institute, Department Of Oncology-pathology, Stockholm,
Methods: Retrospective review of patients with high- Sweden
risk/relapsed/refractory neuroblastoma 123 I-mIBG-avid scanning
treated with 131 I-mIBG at our institution over the last 14 years. Background and Aims: Malignant mechanisms of neuroblastoma are
Results: Twelve patients received 21 131 I-mIBG cycles. There were shaped by its developmental origin from the neural crest. Recent
10 boys. Median age was 5.3 years (1.83-22.58). All had metastatic insights into the fetal development of the adrenal gland extended our
disease. Three patients had MYCN amplification. In 7 cases 131 I- knowledge of cellular identities in neuroblastoma. However, its cell-of-
mIBG was used as induction therapy prior surgery [median time origin is still unknown. Schwann cell precursors (SCPs) are migrating
10.5 days before surgery (8-154)] and in another 2 cases, as con- neural crest-derived multipotent stem cells recently described to
solidation prior stem cell transplantation (21 and 23 days before). contribute to adrenal development in humans. Compared to other
In patients undergoing transplantation after surgery, median time migrating neural crest cells, SCPs exist for a longer period of time and
between 131 I-mIBG and transplantation was 76 days (27-154). In 12 serve as a stem cell reservoir for various cell types. Thereby, SCPs
cases 131 I-mIBG was administered for relapsed/refractory disease, constitute a central progenitor cell within a complex system of cellu-
two of them as pain-controlling palliative treatment. Three patients lar transitions. We seek to understand how this embryonic plasticity
received chemotherapy concomitantly. Four patients received 131 I- translates to the origin of neuroblastoma, therapeutic resistance or
mIBG treatment twice, one patient three cycles and another patient relapse.
four. The administered activity of 131 I-mIBG median dose was 200 mCi Methods: We sequenced seventeen human neuroblastoma sam-
(3.7 GBq). Five patients are dead of progressive disease. Four patients ples by single-cell RNA-sequencing and profiled more than 72,000
are in complete remission with a median follow-up time of 8.46 years cells identifying main cell types including immune, mesenchy-
(5.67-11.42), one of them despite harboring MYCN amplification. Tol- mal and neural crest-derived adrenergic cells. Interestingly, we
erance was excellent: one grade 2 hypertension, one grade 2 vomiting identified SCP-like cells connecting the adrenergic and mesenchy-
and one infusion reaction. Median admission time was 3 days (3-5). mal compartments by cellular transitions. To study the SCP-like
Two cases developed grade 4 pancytopenia, with prolonged throm- cells in greater detail, we applied a new multi-omics sequencing
bocytopenia. Eight/nine patients developed chronic hypothyroidism method (DNTR-Seq) that allowed us to jointly analyze the whole
requiring replacement therapy. genome and transcriptome from single cells sorted for specific
Conclusions: 131 I-mIBG therapy is feasible as induction therapy, markers.
consolidation and relapsed/refractory neuroblastoma, especially in Results: We revealed a complex clonal structure of neuroblastoma
chemo-resistant neuroblastomas. Ideally, blood stem cells should be showing an unexpected heterogeneity not only of adrenergic cells but
collected before 131 I-mIBG treatment. When used pre-operatively, it also of malignant SCP-like cells. Importantly, all malignant cells identi-
seems to facilitate surgery. The most important side effect is throm- fied shared one genetic aberration, a gain of Chr17, that we identified
bocytopenia, especially when the bone marrow is affected. Surgery as the first malignant hit initially present only in SCP-like cells. These
should be performed early after the 131 I-mIBG treatment, before the pre-malignant SCP-like cells distinguish from stromal SCPs by the
thrombocytopenia is established. Stem cell rescue ameliorates the overexpression of potential druggable genes such as NGFR or KPNB1.
thrombocytopenia. Conclusions: We identified an unexpected heterogeneity and plastic-
ity in human neuroblastoma relevant for therapeutic resistance and
relapse. Our data suggest that the newly identified malignant SCP-like
EP169 / #832 SCHWANN CELL PRECURSORS IS THE cells is the cell-of-origin in neuroblastoma and possibly act as a cancer
CELL-OF-ORIGIN IN HUMAN NEUROBLASTOMA stem cell.
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ABSTRACTS S227 of S635

EP170 / #18 EFFECT OF KIGELIA AFRICANA FRUIT Background and Aims: Neuroblastoma originates from cells within
EXTRACT ON STAGE 4 NEUROBLASTOMA the neural crest and is most commonly diagnosed in children under
the age of two. In every fourth neuroblastoma patient, whole genome
Laia Pagerols Raluy, Sofia Ahrens, Magdalena Trochimiuk, Birgit Appl, and whole exome sequencing previously revealed at least one somatic
Charlotte Dücker mutation or structural aberration in genes regulating the Rho/Rac
UKE-Hamburg, Paediatric Surgery, Hamburg, Germany signaling pathway; a pathway implicated in neural crest differentia-
tion and migration. These mutations push active Rho, which increases
Background and Aims: Neuroblastoma (NB), an embryonal tumor downstream Rho-associated kinase (ROCK) activation. Formerly,
deriving from the neural crest, is one of the most common solid ROCK was identified as a promising drug target in neuroblastoma and
pediatric tumors. Especially high-risk NBs, e.g. with MYCN amplifi- other cancer diagnoses.
cation, still account for about 12-15% of cancer related deaths in Methods: The pan-ROCK inhibitor RKI-1447 was studied in viabil-
children. Kigelia africana (KA), is a plant used in traditional African ity assay, clonogenic assay and a neuroblastoma transgenic mouse
medicine which has already shown its anti-cancer potential in sev- model, TH-MYCN. Drug combination screening of RKI-1447 with
eral studies. The aim of this study is to evaluate the effect of KA fruit FIMM oncology drug library yielded synergistic partners that were val-
extract on stage 4 high-risk NB cells on terms of cell survival and idated by drug matrices using WST-1. Further testing of synergistic
immunomodulation. concentrations was performed using clonogenic assays, western blots,
Methods: NB cells with and without MYCN amplification and non- immunohistochemistry and a 3D multicellular tumor spheroid (MCTS)
neoplastic cell lines were treated with KA fruit extract at different con- model, composed of fibroblasts and neuroblastoma cells.
centrations. The effect of KA on cell viability and apoptosis rate were Results: Our studies demonstrate that ROCK inhibition by RKI-1447
assessed by bioluminescence-/fluorescence-based assays. Expression suppressed neuroblastoma growth both in vitro in neuroblastoma
levels of EGFR (epidermal growth factor receptor) and the NFkB cell lines and in vivo in TH-MYCN mice. Additionally, RKI-1447
(nuclear-factor kB) subunit p65 were analysed via Western blot and impaired the clonogenic capacity of neuroblastoma cells. Combina-
microscopy. Secretion of pro- and anti-inflammatory cytokines was tional drug screening revealed synergistic effects between RKI-1447
determined by ELISA. and inhibitors of bromodomain and extraterminal (BET) proteins. Com-
Results: At low doses and contrary to non-neoplastic cells, KA-treated bining BET inhibitor ABBV075/mivebresib with RKI-1447 we eval-
NB cell lines show a significant reduced viability and induced cell death, uated the potential of these combinations and observed synergistic
especially in MYCN non-amplified tumor cells. Interestingly, incubation effects in monolayer cell viability assays and MCTS. Immunohisto-
of tumor- and non-tumor cells with KA leads to a regulation of NF- chemistry staining on MCTS indicated increased cell death, measured
kB p65 phosphorylation and EGFR expression. Furthermore, KA shows by cleaved caspase 3, impaired cell viability and decreased tumor
no effect on the cytokines expressed by NB but on several of those growth, in comparison to single drug treatment. Moreover, west-
expressed by non-tumoral cells. ern blot analysis of combined RKI-1447 and ABBV075 treatment in
Conclusions: Our results demonstrate a cytotoxic effect of KA neuroblastoma cells displayed decreased MYCN protein expression.
fruit extract on NB, especially in MYCN non-amplified tumor Conclusions: BET inhibitors have previously demonstrated potential
cell lines, but not on non-neoplastic cells. We propose a KA- for the treatment of neuroblastoma. We show that combination treat-
mediated regulation of cell survival and apoptosis in tumor ments of BET inhibitors with the pan-ROCK inhibitor RKI-1447 may
cells by modulating expression and activation of EGFR and offer a promising therapeutic approach.
NF-kB, respectively. Furthermore, immunomodulation by NB
appears not to be directed by tumor cytokine production
and thus, further research on NB immune-key rolers must be EP172 / #1891 FRACTURE PREVALENCE IN CHILDREN WITH
assessed. HIGH-RISK NEUROBLASTOMA

Megan Scruggs1 , Abby Dryden1 , Jennifer English1 , Sasigarn Bowden2 ,


EP171 / #722 COMBINATIONAL TREATMENT WITH ROCK Summit Shah3 , Joseph Stanek1 , Keri Streby1
INHIBITOR RKI-1447 AND BET INHIBITORS SYNERGISTICALLY 1 Nationwide Children’s Hospital, Division Of Hematology, Oncology And
IMPAIRS NEUROBLASTOMA GROWTH Bone Marrow Transplantation, Columbus, United States of America;
2 Nationwide Children’s Hospital, Pediatric Endocrinology, Columbus,
Adena Pepich1 , Conny Tümmler1 , Sara Abu Ajamieh1 , Quinty United States of America; 3 Nationwide Children’s Hospital/The Ohio State
Vellema1 , Emma Åkerlund2 , Diana Treis1 , Brinton Seashore-Ludlow2 , University, Radiology, Columbus, United States of America
Per Kogner1 , John Inge Johnsen1 , Malin Wickström1
1 Karolinska Institute, Department Of Women’s And Children’s Health, Background and Aims: Children with high-risk neuroblastoma
Stockholm, Sweden; 2 Karolinska Institutet, Department Of Oncology- (HR-NBL) undergo intensive multi-modal therapy that increase
pathology, Stockholm, Sweden their risk of acute and long-term toxicities. The aims of this study
were to determine fracture prevalence and associated clinical
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S228 of S635 ABSTRACTS

characteristics in children with HR-NBL from time of HR-NBL the effects of gender and age at neuroblastoma diagnosis on time to AI
diagnosis. diagnosis. A review of the Pediatric Health Information Systems (PHIS)
Methods: We conducted a retrospective cohort study of patients aged database from 2010 to 2021 was performed to estimate the national
0-30 years with HR-NBL at our tertiary-care institution and within the incidence of AI in HRNBL.
Pediatric Health Information System (PHIS) database from 2010-2021. Results: Among the institutional cohort of 93 HRNBL patients, 13
Results: We discovered a fracture prevalence of 6.7% (n=6) in 89 HR- developed AI (14%). The adrenal gland was the primary tumor site in
NBL patients at our institution. Four patients had a fracture within 61.3%. Two patients developed AI within 6 months and the latest case
6 weeks of therapy completion, one during consolidation, and one 3 was detected at 218 months from time of HRNBL diagnosis. Though
years off therapy. Mean age at diagnosis was 4.9 years and 4 patients not statistically significant, survival curves demonstrate that females
were female. Lower extremities accounted for 5/6 fractures (2 femurs, had a higher probability of developing AI compared to males (22.2%
1 tibia, one metatarsal, one calcaneus) and one fracture in the radius. vs 6.25%; log-rank Chi-square = 1.9310, P = 0.16). When adjusting for
Three patients historically had iodine123-meta-iodobenzylguanidine gender, each year increase in age at HRNBL diagnosis suggests a 96%
(MIBG)-avid disease in the fractured bone (femurs and tibia) and 3 decreased risk of developing AI (P = 0.67). From the PHIS database an
patients had no history of MIBG avidity in the fractured bone. Only AI incidence of 5.2% was identified (95% confidence interval for this
one patient had MIBG avidity (due to disease recurrence) in the frac- prevalence: 4.75-5.70%).
tured femur about 2 months prior to time of fracture. Two out of 3 Conclusions: We report a 5-14% incidence of AI in chil-
patients with vitamin D measurements had insufficiency (serum 25- dren with HRNBL. Larger multi-institutional studies are
hydroxy vitamin D 19 and 21 ng/dL). Four patients had a mean lumbar needed to validate these findings and determine which
bone mineral density Z-score of -0.275 (obtained shortly after frac- patients are at particular risk of developing AI during or after
tures). In review of the PHIS database, we discovered a 3.35% fracture treatment.
prevalence (with a 95% confidence interval: 2.97-3.75%).
Conclusions: Higher prevalence of fractures was observed in our
cohort of children with HR-NBL during and shortly after treat- EP174 / #1842 FAVORABLE OUTCOME OF
ment compared to general pediatric population. Metastatic bone NEUROBLASTOMA PATIENTS WITH MUTATIONS INVOLVING
disease commonly present at time of diagnosis in addition to NEUROBLASTOMA BREAKPOINT FAMILY OF GENES
high-dose chemotherapy are likely major risk factors. Further
studies are needed to determine the optimal time for evalua- Iyad Sultan1 , Hadeel Halalsheh2 , Abdelghani Tbakhi3
tion and intervention to prevent bone health complications in 1 King Hussein Cancer Center, Pediatrics, Amman, Jordan; 2 King Hussein
HR-NBL. Cancer Center, Pediatric, Amman, Jordan; 3 king Hussein cancer center,
Department Of Cell Therapy & Applied Genomics, Amman, Jordan

EP173 / #1788 INCIDENCE OF ADRENAL INSUFFICIENCY IN Background and Aims: Neuroblastoma Breakpoint Family (NBPF)
CHILDREN WITH HIGH-RISK NEUROBLASTOMA genes are altered in patients with neuroblastoma and other malignan-
cies. We used the TARGET-Neuroblastoma dataset to study alterations
Kelli Patterson1 , Kyle Horvath1 , Gregory Metzger1 , Tran Bourgeois2 , in these genes and their relation to risk stratification and outcome of
Joseph Stanek3 , Peter Minneci1 , Jennifer Aldrink1 , Keri Streby3 patients with neuroblastoma.
1 Nationwide Children’s Hospital, Pediatric Surgery, Columbus, United Methods: Clinical data, mutational data, expression tables, and
States of America; 2 Nationwide Children’s Hospital, Research Institute - processed copy number variation per gene were obtained. Com-
Biostatistician Core, Columbus, United States of America; 3 Nationwide parisons of categorical and numeric data were conducted using
Children’s Hospital, Division Of Hematology, Oncology And Bone Marrow the chi-square test and Student’s t test. Kaplan–Meier curves, log-
Transplantation, Columbus, United States of America rank tests, and Cox proportional hazards regression tests were
used to analyze survival data. Log-transformed gene expression
Background and Aims: Patients with high-risk neuroblastoma values of high and low/intermediate risk tumors were com-
(HRNBL) are at increased risk of toxicities due to aggressive, multi- pared. For all tests, p-value ≤0.05 were considered statistically
modal therapy. The incidence of adrenal insufficiency (AI) has not been significant.
reported. Our primary aim was to determine the incidence of AI among Results: Among 1076 patients with mutational data, 24 patients (2.2%)
patients with HRNBL at our institution and nationally. had 30 mutations involving NBPF genes; half (N=15) of these muta-
Methods: A retrospective cohort study was performed at our institu- tions were missense nonsynonymous. Patients with NBPF mutations
tion including patients with HRNBL from 1998 to 2021. Demographic were less likely to have metastatic disease (p=0.02) or high-risk dis-
and clinical characteristics were evaluated in association with AI inci- ease (p=0.004) and had significantly better 5-year OS (92+/-5.6 vs.
dence. Summary statistics were computed and bivariate analyses were 63+/-1.5, p=0.015). When combined with risk stratification in mul-
conducted using Pearson chi-squared tests, Fisher’s exact tests, and tivariable Cox proportional hazard models, lacking NBPF mutations
Wilcoxon tests. Cox hazard regression models were used to examine was associated with a hazard ratio for death of 4.01 (1.00-16.09,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S229 of S635

p=0.050). Combining results of RNA-seq and microarray gene expres- EP176 / #507 SYNERGISTIC EFFECT OF WIP1 INHIBITOR
sion data, high-risk tumors had significantly lower levels of NBPF1, SL-176 AND H3K27 DEMETHYLASE INHIBITOR GSK-J4 ON
NBPF8, NBPF15, and NBPF24 and higher levels of NBPF4, NBPF6 and NEUROBLASTOMA CELLS AND SPHEROIDS
NBPF22p.
Conclusions: Having mutations in NBPF is an independent prognostic Diana Treis, Conny Tümmler, Per Kogner, John Inge Johnsen, Malin
factor predicting favorable outcome, particularly in patients with high- Wickström Näsman
risk neuroblastoma. Differential gene expression shows that different Karolinska Institutet, Department Of Women’s And Children’s Health,
NBPF genes have different expression patterns in low/intermediate- Stockholm, Sweden
and high-risk tumors.
Background and Aims: The phosphatase WIP1, an oncoprotein
encoded by PPM1D on chromosome 17q, impedes DNA damage
EP175 / #277 HARNESSING CRISPR/CAS9 MUTAGENESIS response and is overexpressed in neuroblastoma, correlating to clinical
SCREENING FOR RATIONAL DESIGN OF NEXT-GENERATION prognosis. The WIP1 inhibitor SL-176 has proven anti-neuroblastoma
CAR-NKT THERAPY AGAINST NEUROBLASTOM effect. In a drug combination screening, the most promising candi-
date for combination with SL-176 was GSK-J4, an inhibitor of histone
Gengwen Tian, Xavier Rios, Chunchao Zhang, Janice Drabek, Claudia H3K27 demethylases previously implicated in neuroblastoma and
Amador, Leonid Metelitsa other malignancies.
Baylor College of Medicine, Pediatric Hematology And Oncology, Houston, Methods: Potential synergy was assessed in a range of neuroblastoma
United States of America cell lines using viability assays and dose-response matrices analyzed
with the SynergyFinder tool. For immunoblot, qPCR and RNAseq, IMR-
Background and Aims: Natural killer T cells (NKTs) possess innate 32, SK-N-BE(2) and SK-N-AS cells were exposed to vehicle, SL-176,
antitumor properties that provide advantages over conventional GSK-J4, or the combination. Protein or RNA was extracted after 1-144
T cells for use in cancer immunotherapy. We found that NKTs or 6-72 hours, respectively. We also treated neuroblastoma multi-
expressing a chimeric antigen receptor (CAR) specific for neu- cellular tumor spheroids, observing size and viability and subsequently
roblastoma antigen GD2 showed better tumor infiltration and collecting them for immunohistochemistry.
ability to modify the tumor microenvironment than GD2-CAR Results: Viability assays in five neuroblastoma cell lines confirmed the
T cells. Genes that regulate NKT cell persistence, functional fit- synergistic effect of combining SL-176 with GSK-J4. Tumor spheroids
ness, and central memory differentiation remain largely unknown. treated with the drug combination showed smaller size and decreased
To address this gap, we have developed a CRISPR/Cas9 screen- viability after six days. Immunoblot experiments demonstrated a
ing system to identify genes that regulate the persistence and marked effect on WIP1 downstream targets and apoptosis markers for
antitumor activity of NKTs and GD2-CAR NKTs for therapeutic cells treated with the combination, compared to vehicle or single drug
targeting. treatment which differed little from each other. qPCR confirmed a clear
Methods: We have developed the first CRISPR/Cas9 screening sys- synergism of SL-176 and GSK-J4 with upregulation of p53 downstream
tem for use in human NKTs and GD2-CAR NKTs to identify genes that targets PUMA and p21, where expression in combination- vs. single
regulate survival and proliferation of these cells during in vitro serial drug-treated cells differed by one order of magnitude. In reference to
tumor challenge. As part of this system, we generated a library of guide vehicle, RNAseq data showed 2580 differentially expressed genes in
(g)RNAs (five per gene) specific for a panel of 1,117 immune-related the combination-treated SK-N-BE(2) cells compared to 48 and 140 for
and 48 non-targeting gRNA controls. We have developed methods for cells treated with only SL-176 or only GSK-J4, respectively. For IMR-32
graded lentiviral transduction of NKTs/CAR-NKTs followed by multi- cells, these numbers were 645, 30 and 30.
plexed functional testing and next generation sequencing of gRNA- Conclusions: Combining the WIP1 inhibitor SL-176 and the epigenetic
transduced cells. We also established a system to validate candidate modifier GSK-J4 induces synergistic cytotoxicity in neuroblastoma
genes that includes in vitro and in vivo functional assays. cells. The vast numbers of differentially expressed genes suggest a
Results: Our first screening round in human NKTs yielded nine candi- pervasive effect of this drug combination on transcription.
date genes of interest, which we are currently validating in a series of
functional experiments. We are also performing a CRISPR/Cas9 library
screen in GD2-CAR NKTs challenged with GD2+ neuroblastoma cells EP177 / #1136 INVESTIGATING THE INTERACTIONS OF
in a serial tumor challenge assay. DISTINCT TUMOR SUBPOPULATIONS IN HUMAN
Conclusions: A CRISPR/Cas9-based screening system is feasible for NEUROBLASTOMA USING SINGLE-CELL RNASEQ AND NOVEL
use in human NKTs/CAR-NKTs to identify novel genes that regulate SPATIAL MULTI-OMICS
persistence and antitumor activity of NKTs in an unbiased manner.
Results obtained from this study will ultimately help to enhance the Ioanna Tsea1 , Thale Olsen1 , Jörg Otte1 , Shenglin Mei2 , Bethel Tesfai
antitumor efficacy of NKT cell-based immunotherapy against neurob- Embaie1 , Per Kogner1 , John Inge Johnsen1 , Ninib Baryawno1 ,
lastoma Shahrzad Fard1
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S230 of S635 ABSTRACTS

1 Karolinska Institutet, Women And Children’s Health, Solna, Sweden; patients remains poor. Novel drug combinations are needed to sup-
2 Harvard Medical School, Department Of Biomedical Informatics, Boston, port existing therapies, improve survival, and reduce late effects. Our
United States of America research group has previously demonstrated that the Rho/ROCK sig-
naling pathway is a promising therapeutic target in NB. This study aims
Background and Aims: Neuroblastoma (NB) is the most common and to explore novel drug combinations with focus on the ROCK2-specific
deadly infant malignancy, accounting for approximately 15% of pedi- inhibitor KD025 (Belumosudil, Rezurock™).
atric cancer-related deaths. Survival in the high-risk group is still less Methods: Drug combination screening was performed using KD025
than 50% which creates the need to identify the subpopulation of together with a cancer drug library containing 528 drugs. Cell via-
tumor cells displaying therapy resistance. bility, clonogenicity, and protein expression/ phosphorylation were
Methods: In our study, we used single-cell RNA sequencing to profile evaluated in NB cell lines treated with single drugs or drug combina-
17 NB samples from 15 patients and generated a database of ∼70,000 tions. Furthermore, IncuCyte® Live Cell analysis was used to monitor
single cells. This was followed by spatial multi-omics using a combined NB tumor spheroid growth and assess cell death. The in vivo effi-
multiplex single-molecule DNA and RNA-FISH approach on NB patient cacy of KD025 was evaluated in 9464D allografts and the transgenic
biopsies. TH-MYCN mouse model.
Results: Unbiased single-cell clustering revealed thirteen cell types Results: Our work demonstrates that KD025 impaired cell viabil-
spanning tumor, immune and stromal cells. Among the tumor popula- ity and clonogenicity of NB cell lines, decreased N-Myc levels, and
tion, we found two clusters expressing known mesenchymal (PRRX1, increased phosphorylation of p38 and Akt. Moreover, KD025 pro-
LEPR) and adrenergic (TH, DBH) genes, indicative of mesenchymal moted the release of the danger-associated molecular pattern HMGB1
and adrenergic tumor origin, respectively. These two putative tumor from SK-N-BE(2) cells. In addition, we observed that KD025 impaired
populations were connected by a “bridge” population that expressed tumor growth in 9464D allografts and in homozygous TH-MYCN
key neural crest and Schwann lineage markers (SOX10, S100B) and mice but did not completely suppress tumor growth. An extensive
was annotated as Schwann Cell Precursor–like (SCPs). Transcriptional combinational drug screening revealed several synergistic combi-
profiling of the three distinct tumor populations revealed a contin- nation partners for KD025 including the DRD2 antagonist TIC10/
uous transition to both the MES and ADR lineages via the SCP-like ONC201 and the p97 inhibitor NMS-873. Synergistic effects of these
population which harbored malignant aberrations. To further define drug combinations were confirmed in NB cell lines grown in mono-
the three tumor cell states and investigate a possible transition from layer and tumor spheroids. Immunohistochemistry on tumor spheroids
malignant SCPs to malignant MES and/or ADR cells, we are in the pro- demonstrated that combinations of KD025 and TIC10 or NMS-873
cess of implementing a single-molecule DNA and RNA-FISH approach. reduced the number of Ki67-positive cells and increased the num-
This method allows us to simultaneously, detect and validate abnor- ber of cleaved Caspase-3 positive cells in comparison to single drug
mal DNA allelic expression and study RNA signatures through which treatment.
we distinguish tumor subpopulations from normal stroma cells. Conclusions: In conclusion, combination treatments using the ROCK2-
Conclusions: We conclude that there are three subpopulations of specific inhibitor KD025 together with NMS-873 or TIC10 may be
malignant NB tumor cells, and are in the process of investigating promising therapeutic approaches for NB.
possible links between them. Increasing our understanding of the inter-
actions between SCPs and their downstream tumor subpopulations
may provide novel information aiding in the strategy for therapeutic EP179 / #703 BARRIERS AND FACILITATORS IN THE
targeting of resistance tumor cell types in high-risk NB. TRANSLATION OF NEUROBLASTOMA RESEARCH: A PILOT
STUDY

EP178 / #953 NOVEL DRUG COMBINATIONS FOR Astrid Van Barneveld1 , Kimberley Mosterman2 , Max M. Van Noesel1 ,
NEUROBLASTOMA THERAPY – EXPLORING THE FULL Jan Molenaar1 , Kenneth Fernald2 , Lieve Tytgat1
POTENTIAL OF THE ROCK2-SPECIFIC INHIBITOR KD025 1 Princess Máxima Center for Pediatric Oncology, Department Of Pedi-
atric Oncology, Utrecht, Netherlands; 2 Vrije Universiteit, Faculty Of Science,
Conny Tümmler1 , Adena Pepich1 , Quinty Vellema1 , Sara Abu Amsterdam, Netherlands
Ajamieh1 , Se Whee Park2 , Linda Ljungblad1 , Brinton
Seashore-Ludlow2 , Kasper Karlsson2 , Per Kogner1 , John Inge Background and Aims: The translation of preclinical findings to clin-
Johnsen1 , Malin Wickström Näsman1 ical applications is a time-consuming process that often spans years.
1 Karolinska Institutet, Department Of Women’s And Children’s Health, In neuroblastoma, where overall survival is still only 61%, there is a
Stockholm, Sweden; 2 Karolinska Institutet, Department Of Oncology- striking discrepancy between the size and activity of the preclinical
pathology, Stockholm, Sweden research field and the number of resulting new clinical applications.
In this pilot study, we identified key barriers and facilitators that
Background and Aims: Neuroblastoma (NB) is an aggressive cancer of play a role in the progress of a preclinical discovery towards clinical
early childhood and a clinical challenge as the prognosis for high-risk implementation in neuroblastoma care.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S231 of S635

Methods: Researchers and clinicians in the field of neuroblastoma tissue; and 1 (6.25%) with BM only disease. Relapse in more than one
were asked to provide their views on the process of translation, and compartment occurred in 4 patients (25%): soft tissue and bone in 2
key accelerating and/or delaying factors, using surveys (n=21) and (12.5%); bone and BM in 1 (6.25%); and 1 in all three compartments
semi-structured interviews (n=12). Questions were based on exist- (6.25%). No CNS relapses occurred. Eight (50%) of the 16 relapsed
ing theoretical frameworks and formulated to allow new themes to patients achieved second CR.
emerge. Conclusions: Relapse after first CR consolidated with Naxitamab
Results: Participants identified 6 important barriers and 3 important occurs mostly limited to one compartment, being the bone the most
facilitators in the translational process. Perceived barriers were prac- common site. BM relapse is rare after Naxitamab consolidation. Sec-
tical difficulties to integrate research and clinical data, the current ond CR is highly achievable and may be related to the reduced burden
academic reward system and career disincentives, low incidence of of disease.
patients, regulations and legislation, high research costs and lack of
funding, and a lack of adequate models for translational research. Per-
ceived facilitators were a high level of multidisciplinary collaboration, EP181 / #1727 HIGH RATE OF SERIOUS ADVERSE EVENTS
international collaborations, and a high level of patient willingness to IN HEAVIER PATIENTS RECEIVING NAXITAMAB DOSES >150
participate in research. MG
Conclusions: There is an urgent need to continue to improve and accel-
erate the process of translating neuroblastoma research into clinical Amalia Varo Rodríguez1 , Laura Andrés Zallo1 , Juan Pablo Muñoz
practice. The barriers and facilitators identified in this study provide a Perez1 , Esther Aurensanz Clemente2 , Pedro Arango Sancho3 ,
guide for further research and highlight the areas that need to be pri- Margarida Simao Rafael1 , Moira Garraus1 , Jaume Mora1
oritized to accelerate the translational process for pediatric oncology 1 Hospital Sant Joan de Déu, Pediatric Oncology, Barcelona, Spain; 2 Hospital

patients, specifically those with neuroblastoma. Sant Joan de Déu, Pediatric Cardiology, Barcelona, Spain; 3 Hospital Sant
Joan de Déu, Pediatric Nephrologist, Barcelona, Spain

EP180 / #1676 PATTERN OF RELAPSE IN HIGH-RISK Background and Aims: Naxitamab is an anti-GD2 monoclonal anti-
NEUROBLASTOMA (HR-NB) PATIENTS CONSOLIDATED WITH body (mAb) that has proved effective against GD2 expressing tumors
NAXITAMAB like high-risk neuroblastoma (HR-NB), retinoblastoma, and osteosar-
coma (OS). Clinical trials of naxitamab have reported 60% of patients
Amalia Varo Rodríguez, Elena Soques Vallejo, Maite Gorostegui, Laura experiencing grade 3-4 pain or hypotension. Pharmacokinetics of
Andrés Zallo, Juan Pablo Muñoz Perez, Alicia Castañeda Heredia, anti-GD2 mAbs have shown drug clearance is based on weight and
Jaume Mora age. For naxitamab, AUC was shown to correlate with patient body
Hospital Sant Joan de Déu, Oncology, Barcelona, Spain weight.
Methods: Single institution, retrospective analysis of >50 kg patients
Background and Aims: Consolidation with anti-GD2 immunotherapy who received naxitamab doses >150 mg from June 2017 to Febru-
has proved to be effective in reducing the risk of relapse in high-risk ary 2022. Grade 3 or 4 serious adverse events (SAEs) were evaluated
neuroblastoma (HR-NB). We here aim to describe the pattern of first according to the CTCAE v5. Naxitamab therapy consisted of 2.4-3
relapse in patients diagnosed with HR-NB who received Naxitamab as mg/kg/dose on days 1, 3 and 5 along with subcutaneous GM-CSF for
consolidation. 10 days.
Methods: Single institution, retrospective analysis of HR-NB patients Results: Nine patients received naxitamab doses >150 mg (1 female;
who received Naxitamab consolidation in first CR between June 2017 8 males), 7 for HR-NB and two in second CR for relapsed metastatic
and December 2021 and subsequently relapsed. Consolidation con- OS. Mean age is 17.5 years (11-23). All had grade 1-2 pain, hypoten-
sisted of 5 cycles of subcutaneous GM-CSF and i.v Naxitamab. Disease sion, or allergy. Six (66.6%) of the 9 patients developed G3-4 AEs
was assessed by the modified International Neuroblastoma Response for a total of 10 events. Four (44.4%) patients required hospital-
Criteria using 123I-MIBG SPECT/TC and bone marrow (BM) aspirates. ization. G3-4 events include two orthostatic hypotension (22.2%)
Results: Seventy-six HR-NB patients in first CR were consolidated with requiring treatment with alpha pressors; two myocarditis (22.2%)
Naxitamab and twenty-four (31.57%) relapsed. Fourteen (58,3%) of receiving corticosteroids; one PRES (11.1%); two (22.2%) G3 hypoten-
the 24 relapsed during immunotherapy and 10 (41.6%) off therapy. sion during infusion; two (22.2%) G3 hypertension post-infusion;
Among the 14 that relapsed during treatment, one relapsed after cycle and one with G3 vomiting with secondary dehydration. All SAEs
1, six after cycle 2, two after cycle 4, and five after cycle 5. Eight of the occurred during 1st naxitamab cycle. All SAES resolved. Patients
24 relapsed patients were lost to follow-up. The relapse pattern was with G4 toxicities (orthostatic hypotension and PRES) were taken off
confirmed for 16 patients. Mean age at diagnosis was 2.9 years (1.7– therapy.
7), and four were MYCN amplified. Twelve (75%) of the 16 patients Conclusions: First exposure to naxitamab in heavier patients receiving
relapsed in one compartment: 5 (31.2%) with isolated bone limited to doses >150 mg is associated with a higher incidence of G3-4 SAEs. All
1-3 foci; 3 (18.75%) with >3 bone lesions; 3 (18.75%) with isolated soft SAEs were reversible with proper management.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S232 of S635 ABSTRACTS

EP182 / #835 DINUTUXIMAB BETA TREATMENT IN to MIBG SPECT/CT imaging. 68 Gallium DOTATATE is FDA approved
CHILDREN WITH ADRENAL GLANDS INSUFFICIENCY radiotracer with significanty high sensitivity and specificity in the
evaluation of neuroblastoma patients although data is limited.
Aleksandra Wieczorek1 , Anna Zaniewska-Tekieli2 , Urszula Methods: Since Iodine 123 MIBG shipping became problamtic with
Zebrowska2 , Walentyna Balwierz1 the stoppe international flights amid the COVID pandemic, we eval-
1 Jagiellonian University Medical College, Pediatric Oncology And Hematol- uated neuroblastoma patients for follow-up studies with 68 Gallium

ogy, Krakow, Poland; 2 University Children’s Hospital in Krakow, Pediatric DOTATATE as it was produced loco regionally at our centre.
Oncology And Hematology, Krakow, Poland Results: We imaged 10 follow-up neuroblastoma patients with
Dotanoc PET/CT. Median age was 42 months (6 females and 4 males).
Background and Aims: Employment of dinutuximab beta (DB) in 5 of these patients also had a baseline Dotanoc PET/CT. 8 scans had
patients with high-risk neuroblastoma (NBL) improved survival and positive findings on follow-up imaging. 5 patients were in the high-
combination of DB with chemotherapy is promising treatment option. risk group. No special preparation was required for the Dotanoc scans
Symptoms of adrenal gland (AG) insufficiency and treatment with ias comapred to the MIBG scans. Radiation dosimetry is also more
hydrocortisone (HC) may influence toxicities and results of therapy. favorable.
Methods: From May, 2017 till January, 2022, 73 patients with NBL Conclusions: 68 Gallium Dotanoc PET/CT imaging in neuroblastoma
were treated with DB, including 45 in monotherapy, 27 DB in combi- restaging and response to therapy evaluation is feasible and offers
nation with chemotherapy and 1 DB with Nivolumab. The occurrence several advantages including high spatial resolution, high sensitivity
of AG insufficiency and its influence on toxicities was evaluated. Of and specificity. Advantages also include that it requires no special
73 patients, in 6 (8%) AG insufficiency was diagnosed before start of preparation and a relatively favorable dosimetry profile compared to
DB. Two children received DB as first line and 4 for relapsed/refractory traditional I131 MIBG scans.
disease; all were heavily pretreated.
Results: Of 6 children with AG insufficiency, in 3 no severe adverse
events were observed during DB therapy. In two other patients exac- E-Poster Topic: AS05.f Renal Tumours
erbation of AG insufficiency was observed during fever, diarrhea or
vomiting. One patient had severe exacerbation from the 2nd day of E-POSTER VIEWING
DB infusion without any additional reasons. All symptomatic patients
required increase of HC dose with good effect. In consecutive cycles, EP184 / #984 CLINICO-PATHOLOGICAL FEATURES AND
dose of HC was increased from beginning of the cycle with good effect, THERAPY OUTCOME IN CHILDHOOD WILMS TUMOR:
and additional doses were added if needed. Two children after DB THIRTEEN YEARS EXPERIENCE OF TERTIARY CARE CENTER IN
therapy had disease relapse, the other 4 remain in CR. SAUDI ARABIA
Conclusions: The DB therapy is feasible in children with AG insuffi-
ciency. Patients must be observed very carefully and HC dose must Abdulwahab Alharthi1 , Abdulaziz Bin Mesained1 , Omar Alsharif1 ,
be increased if symptoms occur. Increase of HC dose should be con- Nawaf Alkhayat1 , Mohammad Alshahrani1 , Hasna Hamzi1 , Amal
sidered for next cycles. Symptoms of AE insufficiency may be similar Binhassan1 , Yasser Elborai1,2
to DB toxicities, so it should not be missed before therapy. Although 1 Prince Sultan Military Medical City, Pediatric Hematology-oncology
HC may influence response to DB, its immunosuppressive effect is low Department, Riyadh, Saudi Arabia; 2 National Cancer Institute (NCI), Cairo
and considering poor outcome in HR NBL, these patients should not University, Pediatric Oncology Department, Cairo, Egypt
be excluded from therapy. Influence of HC on treatment results needs
further investigations. Background and Aims: Wilms Tumor (WT) is second most common
abdominal tumor in children. It is usually presented as asymptomatic
abdominal mass in otherwise healthy child. It needs multidisciplinary
EP183 / #2020 NEUROBLASTOMA RESTAGING AND approach (chemotherapy, surgery and radiotherapy). Our aim is to
RESPONSE TO THERAPY EVALUATION: FUTURE OF 68GA assess our management and outcome of WT over a period of 13 years
DOTANOC PET/CT and comparing it with local, regional and international studies.
Methods: We reviewed 37 pediatric patients diagnosed with WT
Surekha Yadav presented to Prince Sultan Military Medical City (PSMMC) a ter-
army hospital R&R, subroto park, Department Of Nuclear Medicine, delhi tiary care hospital in Riyadh, Saudi Arabia, from January-2004 till
cantonment, India December-2016.
Results: Median age at diagnosis 29 (range 4-99) months. Male
Background and Aims: Iodine 123 MIBG is the conventional mode 15/37(40.5%), female 22/37(59.5%). Most common presentation
of investigating neuroblastoma. In certain cases evaluation with FDG was abdominal mass (80%). Associated symptoms: hypertension
PET/CT might also be required. Newer PET agents offer a higher (46%), hematuria (16%), anemia (51%) and hereditary predispo-
sensitivity and accuracy and have been demonstrated to be superior sition syndromes (8%). Right and left sided tumor were equally
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S233 of S635

distributed 15(40.5%) patients each, while bilateral involvement Results: Of 1,477 survivors of childhood cancer with SMNs, 53 devel-
7(19%) patients. Lung was the commonest metastatic site 8/37(21.6%). oped a renal SMN (54 total cases). Of the 54 cases, 47 were a second
Stage I, II, III and IV were 10/37(27%), 10/37(27%), 9/37(24.4%) cancer (43 carcinomas, 3 Wilms, and 1 Ewing sarcoma), 6 carcino-
and 8/37(21.6%); respectively. Favorable histology (FH) and unfavor- mas were a third cancer and 1 carcinoma was a fourth cancer. Among
able histology (UH) were 34/37(91.9%) and 3/37(8.1%); respectively. these, there were 29 female (54.7%), 47 white (88.7%) and 7 Hispanic
Twenty-two (59.5%) patients received pre-operative chemotherapy, (13.2%) survivors. The most common primary cancers were Hodgkins
thirteen (35.1%) received post-operative chemotherapy and two lymphoma (n=9) and neuroblastoma (n=7). The mean ages of primary
(5.4%) had surgery alone. Fifteen(40.5%) patients received radio- malignancy and renal SMN occurrence were 10.1 years and 31.1 years,
therapy after surgery and chemotherapy, twenty(54.1%) received respectively. The SIR of developing any renal SMN was 4.52 (p<0.05;
chemotherapy without radiotherapy, two(5.4%) had surgery alone. 95%CI 3.39-5.89). The 5-year overall survival after a renal SMN was
After median follow up period 60 (range 4-84) months, 33/37(89.2%) 71% (95%CI: 55%-82%).
patients got complete remission while 4/37(10.8%) patients had per- Conclusions: Renal SMNs are rare but occur ∼4.5 times more fre-
sistent/refractory disease. Four patients relapsed after median follow quently in survivors of childhood cancer compared to the general
up period 34 (range 18-46) months, two of them had FH as stage III population. This increased risk should be considered when providing
and IV, the other two patients were stage IV with UH (diffuse/focal long-term care for survivors of childhood cancer.
anaplasia). Four patients died, two were refractory (FH stage IV) and
two were relapsed (UH stage IV). Five-years overall-survival 89.2% and
event-free-survival 78.4%. EP186 / #927 EPIDEMIOLOGIC AND CLINICAL OUTCOMES
Conclusions: Our study demonstrated excellent survival rate for OF PEDIATRIC RENAL TUMORS IN KOREA: A RETROSPECTIVE
WT comparable with local and regional outcomes. Diffuse anapla- ANALYSIS OF THE KOREAN PEDIATRIC HEMATOLOGY AND
sia and advanced stage have strong relationship with relapse and ONCOLOGY GROUP (KPHOG) DATA
mortality.
Hyoung Soo Choi1 , Kyung-Nam Koh2 , Jung Woo Han3 , Hyoung Jin
Kang4 , Ji Won Lee5 , Keon Hee Yoo6 , Ki Woong Sung5 , Hong Hoe Koo5 ,
EP185 / #1203 SUBSEQUENT MALIGNANT NEOPLASMS OF Kyung Taek Hong4 , Jung Yoon Choi4 , Sung Han Kang2 , Hyery Kim2 , Ho
THE KIDNEY AFTER TREATMENT OF PRIMARY MALIGNANCY Joon Im2 , Seungmin Hahn3 , Chuhl Joo Lyu3 , Hee-Jo Baek7 , Hoon
DURING CHILDHOOD: ANALYSIS OF SURVEILLANCE, Kook7 , Kyung Mi Park8 , Eu Jeen Yang9 , Young Tak Lim9 , Seongkoo
EPIDEMIOLOGY, AND END RESULTS DATA FROM 1975 TO 2016 Kim10 , Jae Wook Lee10 , Nack-Gyun Chung10 , Bin Cho10 , Meerim
Park11 , Hyeon Jin Park11 , Byung-Kiu Park11 , Jun Ah Lee11 , Jun Eun
Anthony Bell1 , Arun Rangaswami2 , Trish Murphy2 , Maxwell Meng3 , Park12 , Soon Ki Kim13 , Ji Yoon Kim14 , Hyo Sun Kim15 , Youngeun Ma1 ,
Robert Goldsby2 Kyung Duk Park16 , Sang Kyu Park17 , Eun Sil Park18 , Ye Jee Shim19 , Eun
1 UCSF Benioff Children’s Hospital, Pediatrics, San Francisco, United States Sun Yoo20 , Kyung Ha Ryu20 , Jae Won Yoo10 , Yeon Jung Lim21 , Hoi Soo
of America; 2 UCSF Benioff Children’s Hospital, Pediatric Oncology, San Yoon22 , Mee Jeong Lee23 , Jae Min Lee24 , In-Sang Jeon25 , Hye Lim
Francisco, United States of America; 3 University of California San Francisco Jung26 , Hee Won Chueh27 , Seunghyun Won28
(UCSF), Urology, San Francisco, United States of America 1 Seoul National University Bundang Hospital, Pediatrics, Seongnam, Korea,

Republic of; 2 Asan Medical Center, University of Ulsan College of Medicine,


Background and Aims: Malignant renal neoplasms are rare before age Pediatrics, Seoul, Korea, Republic of; 3 Yonsei University College of Medicine,
45. Age, gender, and genetic risk factors can influence the risk of these Pediatrics, Seoul, Korea, Republic of; 4 Seoul National University College
neoplasms. However, the risk in survivors of childhood cancer is not of Medicine, Pediatrics, Seoul, Korea, Republic of; 5 Samsung Medical Cen-
well established. This study aims to report the incidence of renal sub- ter, Department Of Pediatrics, Seoul, Korea, Republic of; 6 Sungkyunkwan
sequent malignant neoplasms (SMNs) after treatment of a childhood University, Samsung Medical Center, Department Of Pediatrics, Seoul,
primary malignancy. Korea, Republic of; 7 Chonnam National University Hwasun Hospital, Pedi-
Methods: Using the Surveillance, Epidemiology, and End Results atrics, Gwangju, Korea, Republic of; 8 Dongnam Institution of Radiological
(SEER) registry, we identified survivors of childhood cancer who devel- & Medical Sciences, Pediatrics, Busan, Korea, Republic of; 9 Pusan National
oped a renal SMN. Survivors were under 20 years old at the time University School of Medicine, Pusan National University Children’s Hos-
of primary cancer diagnosis and diagnosed between 1975-2016. We pital, Department Of Pediatrics, Yangsan, Korea, Republic of; 10 College

excluded survivors with a primary renal cell carcinoma or adenocarci- of Medicine, The Catholic University of Korea, Pediatrics, Seoul, Korea,
noma, and the SMN had to occur at least 13 months after the primary Republic of; 11 National Cancer Center, Pediatrics, Goyang, Korea, Repub-
cancer diagnosis. The clinical characteristics of the survivors with lic of; 12 Korea University Medical Center, Pediatrics, Seoul, Korea, Republic
SMNs of the kidney (including renal pelvis) were evaluated and stan- of; 13 College of Medicine, Inha University Hospital, Pediatrics, Incheon,
dardized incidence ratios (SIRs[observed/expected]) were calculated. Korea, Republic of; 14 School of Medicine, Kyungpook National University,
Kaplan-Meier estimates were performed to assess survival after renal Pediatrics, Daegu, Korea, Republic of; 15 Inje University Haeundae Paik Hos-
SMN. pital, Pediatrics, Busan, Korea, Republic of; 16 Jeonbuk National University
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S234 of S635 ABSTRACTS

Medical School, Pediatrics, Jeonju, Korea, Republic of; 17 University of Ulsan Background and Aims: Nephroblastoma is the one of most frequent
College of Medicine, Pediatrics, Ulsan, Korea, Republic of; 18 Gyeongsang tumor in children and the aim is to obtain an optimal and diligent
National University School of Medicine, Pediatrics, Jinju, Korea, Repub- diagnosis in the pathology pole of the CRDCE.
lic of; 19 Keimyung University School of Medicine, Keimyung University Methods: This is a retrospective study of pediatric renal tumors. The
Dongsan Hospital, Department Of Pediatrics, Daegu, Korea, Republic of; pieces of nephrectomies were received fresh. The macroscopy and his-
20 Ewha Womans University College of Medicine, Pediatrics, Seoul, Korea, tolpathologic techniques were carried out immediately. We used the
Republic of; 21 Chungnam National University College of Medicine, Pedi- SIOP classification for grade and stage.
atrics, Daejeon, Korea, Republic of; 22 Kyung Hee University College of Results: Over a period of the years 2020 and 2021, sixty (60) renal
Medicine, Pediatrics, Seoul, Korea, Republic of; 23 Dankook University Col- tumors were diagnosed, in the CRDCE, with 46 nephroblastomas, fol-
lege of Medicine, Pediatrics, Cheonan, Korea, Republic of; 24 Yeungnam lowed by rhabdoid tumors (5 cases), clear cell sarcomas (2 cases), and
University College of Medicine, Department Of Pediatrics, Daegu, Korea, neuroblastoma (2 cases). Within the 46 cases of nephroblastoma, the
Republic of; 25 Gachon University Gil Medical Center, Pediatrics, Incheon, gender ratio was around 1 with a slight male predominance, the age
Korea, Republic of; 26 Sungkyunkwan University School of Medicine, Kang- was between 15 years and 7 months with an average of 4 years and
buk Samsung Hospital, Department Of Pediatrics, Seoul, Korea, Republic of; 3 months at the time of diagnosis. Upon receipt of the 46 specimens,
27 Dong-A University College of Medicine, Pediatrics, Busan, Korea, Repub- the macroscopic examination showed a weight ranging from 100g to
lic of; 28 Seoul National University Bundang Hospital, Medical Research 3200g with an average of 570g. The histological examination con-
Collaborating Center, Seongnam, Korea, Republic of firmed the diagnosis and the classification of the cases according to
histological subtypes, the mixed subtype: 14 cases, the regressive sub-
Background and Aims: Renal tumors account for approximately 7% of type: 13 cases, the stromal subtype: 8 cases, the blastematous subtype
all childhood cancers, including Wilms tumor (WT), clear cell sarcoma : 6 cases, the epithelial subtype: 3 cases, finally a necrotic case and a
of the kidney (CCSK), malignant rhabdoid tumor (MRTK), renal cell car- cystic case. 63% of nephroblastomas were classified as stage 2 (inter-
cinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare mediate risk) according to the SIOP classification of 2001, stage 3 and
tumors. We investigated the epidemiology of pediatric renal tumors in stage 1 were respectively at 19.5% and 17.5%.
Korea. Conclusions: According to the findings of our study, nephroblastoma
Methods: From January 2001 to December 2015, data of pediatric remains the most common histological type of pediatric renal tumors.
patients (0–18 years of age) newly-diagnosed with renal tumors at 26 With a predominance of histological subtypes low and intermediate
hospitals were retrospectively analyzed. risk.
Results: Among 439 patients (male 240), the most common tumor was
WT, at 77.9% (n=342), followed by RCC, CCSK, MRTK, CMN, and oth-
ers at 8.2% (n=36), 5.5% (n=24), 3.6% (n=16), 2.7% (n=12), and 2.1% EP188 / #1986 ADVANCED STAGE OF NEPHROBLASTOMA:
(n=9), respectively. Median age at diagnosis was 27.1 months (range EXPERIENCE OF SINGLE CHILDHOOD CANCER UNIT IN
0-225.5) and median follow-up duration 88.5 months (range 0-211.6). KINSHASA - DRCONGO
Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, pro-
gressive disease, second malignant neoplasm, and treatment-related Nina Domo1 , Karim Assani1 , Aleine Budiongo1 , François Beya2 , René
mortality. Five-year overall survival and event free survival were 97.2% Ngiyulu1 , Jean Lambert Ehungu1
and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 1 Kinshasa University Hospital, Pediatrics, Kinshasa, Congo, Republic of;
60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively 2 Kinshasa University Hospital, Pathology, Kinshasa, Congo, Republic of

(P < 0.001).
Conclusions: The pediatric renal tumor types in Korea are similar Background and Aims: Nephroblastoma is the most common malig-
to those of previous reports in other countries. WT accounted for a nant renal tumor in children and accounts for approximately 90% of
large proportion and survival was excellent. Non-Wilms renal tumor kidney cancers. Management of patients diagnosed at advanced stage
included a variety of tumors and showed inferior outcome especially in (stage III and IV) is challenging, especially in the context of sub-Saharan
MRTK. Further efforts are necessary to optimize the treatment and to Africa. We present the experience of the paediatric oncology unit of
analyze the genetic characteristics of pediatric renal tumors in Korea. Kinshasa, DRC.
Methods: This is a retrospective descriptive study, from January 2010
to December 2020 conducted at the paediatric oncology unit of
EP187 / #952 EPIDEMIOLOGICAL AND PATHOLOGICAL Kinshasa university hospital.
ASPECTS OF NEPHROBLASTOMA IN CRDCE TWO FIRST YEAR Results: A total of 96 children was diagnosed with nephroblastoma
OF ACTIVITY during the period of study. Advanced stage (Stage III and IV) was
found in 26 children (30%). Most of them were living in Kinshasa city
Cherif Dial, Gabriel Deguenonvo, Mahmoud Abouzid (81%) with their ages between 2-5 years old (58%).The majority of par-
Cheikh Anta Diop University, Anatomopathology, Dakar, Senegal ents completed at least primary school Abdominal mass was the main
symptom at presentation (96.2%), with fever (56.8), abdominal pain
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S235 of S635

(30.8%) and haematuria (23.2%). Abdominal ultrasound was done for toxicities and 71% veno-occlusive disease(n=5). At a median follow-up
all and chest X-ray for 92%. There was a predominance of stage IV period of 36 months, EFS and OS were 69.5% and 87.8% respec-
(57.7%). Surgery has been done to only 50% of the children and chil- tively; 3-year EFS and OS in 0-2 years,2-4 years,4-10 and >10 years
dren received preoperative and postoperative chemotherapy. No child were 67.6%,73.1%,71.9% and 64.2% and 84%,88%,91% and 88.2%
received radiation therapy. In this study, 53.8% of the children had respectively.
abandoned the treatment and 38.4% died. Only 2 patients (7.6%) are Conclusions: Age < 2 years and >10 years had poorer outcome in
in remission FH WT. Higher incidence of inoperable disease at presentation and
Conclusions: Mortality rate and abandonment of treatment are increased toxicities including death might explain the unexpectedly low
very high and need to be addressed. Improving access to med- outcome in <2 years.
ical care and providing radiation therapy services may help to
increase the cure rate, but early diagnosis should be on top of
priorities. EP190 / #1442 ACCURACY OF CT IN DIFFERENTIATING
WILMS FROM NON-WILMS RENAL TUMOURS IN PEDIATRIC
PATIENTS
EP189 / #816 AGE AS A PROGNOSTIC VARIABLE IN
CHILDREN AND ADOLESCENTS WITH FAVOURABLE Vasundhara Patil1 , Akshay Baheti2 , Swapnil Meshram1 , Sajid
HISTOLOGY (FH) WILMS TUMOUR (WT) Qureshi3 , Suyash Kulkarni2 , Mukta Ramadwar3 , Kunal Gala2 , Nilesh
Sable3 , Girish Chinnaswamy3 , Maya Prasad3 , Nehal‘ Khanna4 , Nitin
Anisha Panda1 , Maya Prasad1 , Badira Cheriyalinkal Parambil1 , Shetty2 , Siddhartha Laskar5
Venkata Rama Mohan Gollamudi1 , Sajid Qureshi2 , Akshay Baheti3 , 1 Tata hospital, Radiology, MUMBAI, India; 2 Tata Memorial Hospital, Pedi-
Vasundhara Patil3 , Nehal‘ Khanna4 , Siddhartha Laskar4 , Mukta atric Oncology, Mumbai, India; 3 Tata Memorial Centre, Paediatric Oncology,
Ramadwar5 , Girish Chinnaswamy1 Mumbai, India; 4 Tata Memorial Centre, Radiation Oncology, mumbai, India;
1 Tata Memorial Hospital, Homi Bhabha National Institute, Paediatric 5 Tata Memorial Hospital, Radiation Oncology, Mumbai, India

Oncology, Mumbai, India; 2 Tata Memorial Hospital, Homi Bhabha National


Institute, Surgical Oncology, Mumbai, India; 3 Tata Memorial Hospital, Homi Background and Aims: Introduction: Pediatric renal tumors are his-
Bhabha National Institute, Radiodiagnosis, Mumbai, India; 4 Tata Memorial tologically diverse, 85% being Wilms tumor (WT) and remaining being
Hospital, Homi Bhabha National Institute, Radiation Oncology, Mumbai, heterogeneous group of non-Wilms tumors (NWT). Lack of specific
India; 5 Tata Memorial Hospital, Homi Bhabha National Institute, Pathology, radiological criteria and tumour markers leads to misdiagnosis. Imag-
Mumbai, India ing needs to identify cases in which radiological uncertainty warrants
preoperative histology. Aim : 1) To assess the utility of baseline CECT in
Background and Aims: There is limited and inconsistent data regard- differentiating WT from NWT. 2) To correlate the imaging findings with
ing the independent impact of age on outcomes of WT; age < 2 years histopathological report (HPR). 3) To evaluate inter-observer variation
is generally considered favorable. We report the presentation and between two dedicated pediatric oncoradiologists.
outcomes with regards to age at presentation. Methods: Type of study: Institutional Ethics Committee approved ret-
Methods: All patients with FH WT treated at our centre from rospective study done in Tata memorial Hospital Study population
January 2013 - December 2020 were analysed. While resectable Comprise of sample size of 81 patients, with baseline WT and NWT
tumours were operated upfront, unresectable tumours received pre- in a ratio of 2 : 1 ie 54 Wilms tumors and 27 non-Wilms tumors.
operative chemotherapy (Vincristine-Dactinomycin (VA) x4 weeks in Methodology CT scans were assessed independently by two pediatric
non-metastatic and VA+Doxorubicin x 6 weeks in metastatic tumors) oncoradiologists blinded to the histpathology and overall impression
followed by delayed nephrectomy. Local radiation and local treatment was recorded as WT, non-WT, or indeterminate. The variations in the
of metastatic sites were also done. Presentation and outcomes (Event opinion of the two observers was also noted. Diagnostic accuracy of CT
free survival (EFS) and overall survival (OS))- were analysed by age. for predicting WT vs non-WT histology was assessed and κ-Coefficient
Results: Of 257 patients treated during this period, 235(91.4%) had value was calculated for the degree of agreement between the two
favourable histology, with median age at presentation of 23 months observers.
(range-3-372 months) and 145(61.7%) were males. Most children Results: The average age presenatation of WT and NWT was statisti-
were aged 0-2 years(n=67),2-4 (n=83) and 4-10 years(n=68) ; only cally significant (p =.001). There was moderate agreement between CT
17 patients were older than 10 years. The incidence of stage 3 diagnosis and HPR of observer 1 (k value of 0.61) and mild agreement in
and 4 disease in 0-2 years, 2-4 years,4-10 years and >10 years observer 2 (k value of 0.32). There was poor interobserver agreement
was 43.2%,62.6%,72% and 64.7% respectively; bilateral WT was in categorizing WT, NWT and indeterminate with K value of 0.196. The
11.9%,2.4%, 4.1% and 5.8%.The proportion of patients with inoperable variors imaging characteristic assessed like tumoral calcification and
disease at presentation was 50.7%, 62.6%,63.2% and 41% respectively. fat, renal sinus, pelvicalyceal system, ureter and vascular involvement,
The incidence of toxic deaths was higher in children <2 years at 57.1% metastasis, adenopathy and subcapsular fluid in Wilms and non-Wilms
compared to 28.5% in >2years, with 13.3 % grade ¾ haematological tumour were stastically not significant.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S236 of S635 ABSTRACTS

Conclusions: There are no specific CT features to differentiate 1 Division of Hematology Oncology, Women’s And Children’s Health, padova,

WT and NWTs with statistical significance. The overall accuracy Italy; 2 Division of Infectious Disease, Women’s And Children’s Hospital,
of CT to differentiate WT from NWT has high sensitivity but low Padua, Italy
specificity.
Background and Aims: Fever is frequent in children with oncological
diseases and can be associated to many infectious causes. Many drugs
EP191 / #318 FAMILIAL WILMS TUMOR ASSOCIATED WITH can also cause fever, as cytarabine, carboplatin or methotrexate. Fever
GERMLINE REST MUTATIONS induced by vincristine (VCR) is frequently observed during clinical
practice, but no reliable data about its characteristics are available.
Liudmila Yasko1 , Maria Kurnikova1 , Yulia Mareeva1 , Anastasiya Methods: We conducted a retrospective study in patients < 18 years
Salomatina2 treated for Wilms tumour at the Division of Pediatric Haematology-
1 Dmitry Rogachev National Medical Research Center Of Pediatric Hematol- Oncology, Padua, between 2010 and 2021. We divided febrile episodes
ogy, Oncology and Immunology, Laboratory Of Molecular Biology, Moscow, in 5 categories: chemotherapy (CT) related fever, infective fever,
Russian Federation; 2 Dmitry Rogachev National Research Center of Pedi- febrile neutropenia, neoplastic fever and “other”. We compared infec-
atric Hematology, Oncology and Immunology, Clinical Oncology, Moscow, tive/neutropenic episodes with the ones related to CT. We recorded
Russian Federation data about characteristics of fever and chemotherapy, blood exams,
radiological investigations, antibiotic therapy, hospitalization and delay
Background and Aims: Wilms tumor (WT) is one of the most com- of treatment in following cycles. Exact Fisher tests were used to com-
mon childhood renal cancer. Although WT is primarily a non-familial pare proportions. Kruskal-Wallis test or ANOVA on ranks were used to
condition, about 5% of cases have one or more relatives with a his- compare continuous distributions. Statistical significance was settled
tory of Wilms tumor. Most of the time, inherited WT cases harbour for any p-value <0.05.
WT1 mutations. Besides that, germline inactivating mutations in REST, Results: We recorded 97 febrile episodes in 36 patients: 28 correlated
CTR9, TRIM28 have recently been considered as WT predisposi- to chemotherapy, and 69 identified as infective fever in patients with
tion genes. This report presents WT cases with detected germline or without neutropenia. CT related fever occurred most frequently in
mutations in REST. This gene is also associated with the gingival fibro- the first weeks of treatment (median 4 weeks) compared to patients
matosis. Aims: presentation of clinical cases with WT associated with with infective fever (median 8 weeks, p<0.0001). The duration of
germline pathogenic variants in REST in children from two unrelated fever resulted statistically significant, with a shorter resolution of the
families. episodes in the group of CT related fever (median: 72 hours, p=0.0052).
Methods: Patient 1 (a female, 4 y.o. with bilateral WT) is a proband from All episodes of CT related fever occurred after the administration of
in vitro fertilization (IVF) triplets, with sister and brother without WT. Vincristine, in combination or not with other drugs, and the majority
Patient 2 (a male, 5 y.o. with unilateral WT) is a proband, who has 2 of them happened within the first 24 hours of treatment (75%). Half
healthy siblings. We analyzed probands‘ DNA extracted from periph- of the patients with CT related fever received antibiotic therapy. Data
eral blood leucocytes with custom-targeted NGS-198 genes-panel. about infectious markers were limited and no statistical differences
The detected variants were checked in family members by Sanger were noted.
sequencing. Conclusions: Rapid recognition of CT related fever is important in
Results: Patient 1 had a pathogenic germline mutation in REST order to avoid extra testing, unnecessary antibiotic therapy and hos-
c.2668_2671del, p.(E891Pfs*6), as well as her sister. The mutation pitalization.
was probably inherited from their deceased father, as he had gingi-
val fibromatosis. Patient 2 had a pathogenic germline variant in REST
c.1035_1036insTA, p.(E346*), as well as his mother and both broth- EP193 / #482 MRI-CHARACTERISTICS OF PEDIATRIC- AND
ers. There were two other WT cases in this family (proband‘s maternal YOUNG-ADOLESCENT RENAL CELL CARCINOMA: A
uncles). Family members with REST mutations from both families had SINGLE-CENTER RETROSPECTIVE STUDY AND LITERATURE
gingival fibromatosis. REVIEW
Conclusions: We have demonstrated familial clinical cases of a
rare WT form, associated with germline pathogenic variants in Justine Van Der Beek1,2 , Ronald De Krijger2,3 , Rutger-Jan
REST. Nievelstein1,2 , Marry Van Den Heuvel-Eibrink2 , Annemieke Littooij1,2
1 University Medical Center Utrecht, Department Of Radiology And Nuclear

Medicine, Utrecht, Netherlands; 2 Princess Máxima Center for Pediatric


EP192 / #386 CHEMOTHERAPY RELATED FEVER IN Oncology, Pediatric Oncology, Utrecht, Netherlands; 3 University Medical
PATIENTS TREATED FOR WILMS TUMOUR Center Utrecht, Department Of Pathology, Utrecht, Netherlands

Arianna Tagarelli1 , Sofia Santoni1 , Maria Carmen Affinita1 , Daniele Background and Aims: Pediatric renal cell carcinoma (RCC) is a
Donà2 , Gianni Bisogno1 rare renal malignancy. Translocation-type RCC (MiT-RCC) is the most
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S237 of S635

frequent subtype in children, in contrast to clear-cell RCC (ccRCC) 7 Saarland University Hospital, Department Of Radiation Oncology, Hom-
in adults. MRI is the preferred imaging modality for assessment burg, Germany; 8 University Hospital, Heidelberg, Section Of Paediatric
of pediatric renal tumors and plays a potential role in their non- Radiology„ Heidelberg, Germany
invasive discrimination. Previous literature has suggested imaging
findings differ between RCC-subtypes, however, studies focusing on Background and Aims: Treatment of nephroblastoma starts with pre-
MRI-characteristics are limited. Therefore, this study aims to identify operative chemotherapy in SIOP trials without proven histology. To
MRI-characteristics of RCC, focused on its presentation in children and test for a higher accuracy in the initial imaging diagnosis additional
young adolescents, through a single-center case-series and literature clinical and tumour-related parameters were analysed.
review. Methods: We conducted a retrospective analysis of a total of 3307
Methods: The identified diagnostic MRI-scans including diffusion- patients with histologically proven paediatric renal tumours enrolled
weighted imaging (DWI) were retrospectively assessed by two in the consecutive SIOP-GPOH trials between 1989 and 2017. Our
observers. An extensive literature review was conducted in Pubmed, focus included Wilms tumour (WT), congenital mesoblastic nephroma
Embase and Cochrane, focusing on MRI-characteristics of RCC- (CMN), clear cell sarcoma (CCSK), malignant rhabdoid tumour of the
subtypes often seen in children and young adults. kidney (MRTK) and renal cell carcinoma (RCC). The different entities
Results: Six pediatric patients with a median age of 12 years (range were compared in view of metastasis, bilaterality, tumour volume (TV)
63-193 months) were included. Two/six patients had MiT-RCC, and and age at diagnosis. Statistical analysis was enhanced by ROC curves
2/6 ccRCC. Median tumor volume was 393 cm3 (range 29-2191 and Pearson correlations.
cm3 ). Five tumors had a hypo-intense appearance on T2-weighted Results: Bilateral tumours occur exclusively in WT (253 patients, 8.6%
imaging, whereas 4/6 were iso-intense on T1-weighted imaging. of WT), with the exception of one patient with CCSK. In patients aged
Four/6 tumors showed well-defined margins, whereas other tumor <3 years with localized disease, CMN (N= 96/1009 (9.5%)) and CCSK
characteristics were often inconsistent among patients. The median (N=57/1009 (5.6%)) occur in addition to WT (N=834/1009 (82.7%)).
apparent diffusion coefficient (ADC)-values on DWI ranged from In TV<100 ml, CMN is present in 54 of 213 (25.4%) patients. MRTK is
0.70-1.20*10-3 mm2 /s. Thirteen articles (46 cases) focusing on MRI- common in the <3 years age group with metastatic disease (N=12/59
characteristics of MiT-RCC could be identified. A majority of the (20.3%) in TV<500 ml). In the age group of 3-7 years, Non-WT is
patients showed T2-weighed hypo-intensity, similar to our MiT-RCC very rare, regardless of metastatic status or TV. In the age group
cases. T1-weighted hyper-intensity, an irregular growth pattern and >7 years, RCC occurs more frequently, especially when TV<150ml.
limited diffusion restriction were often described for MiT-RCC. Tumours >1000ml are observed only in WT and CCSK. None of the
Conclusions: The discrimination of RCC-subtypes and differentiation CMN patients have metastases at diagnosis. For the different entities
from other pediatric renal tumor types based on MRI remains difficult. ROC curves are shown.
Nevertheless, T2-weighted hypo-intensity of the tumor seems a poten- Conclusions: The frequency profiles established for each paediatric
tial distinctive characteristic in our case-series as well as in previous renal tumour allow for a more specific initial diagnosis and can help
literature. This study stresses the importance of gaining specific knowl- radiologists differentiate WT from RCC, CMN, and MRTK. Differenti-
edge of MRI in pediatric- and young adolescent RCC and focusing on ating WT from CCSK remains a challenge. Novel approaches like liquid
innovative techniques. biopsies may help to further increase diagnostic accuracy in the near
future.

EP194 / #725 HOW TO IMPROVE INITIAL DIAGNOSTIC


ACCURACY OF KIDNEY TUMOURS IN CHILDHOOD WITHOUT E-Poster Topic: AS05 SIOP Scientific programme / AS05.g Bone
HISTOLOGY? Tumours

Nils Welter1 , Norbert Graf2 , Gregor Metternich2 , Rhoikos E-POSTER VIEWING


Furtwängler1 , Stefan Wagenpfeil3 , Leo Kager4 , Christian Vokuhl5 ,
Steven Warmann6 , Joerg Fuchs6 , Patrick Melchior7 , Jens Peter EP195 / #1330 IDENTIFICATION OF POTENTIALLY
Schenk8 PATHOGENIC VARIANTS IN CANCER PREDISPOSITION GENES
1 Saarland University Medical Center, Department For Pediatric Oncology IN PEDIATRIC SARCOMAS BY NEXT GENERATION
And Hematology„ Homburg, Germany; 2 Saarland University Medical Center, SEQUENCING
Department For Pediatric Oncology And Hematology, Homburg, Germany;
3 Saarland University„ Department Of Medical Biometry, Epidemiology And Piedad Alba-Pavón1 , Lide Alaña1 , Miriam Gutiérrez-Jimeno2 , Aizpea
Medical Informatics, Homburg, Germany; 4 Medical University Vienna, St. Echebarria-Barona1,3 , Teresa Imízcoz4 , Laura Zaldumbide1,5 , Lorena
Anna Children’s Hospital And Ccri, Department Of Paediatrics, Vienna, Aus- Mosteiro5 , Susana García-Obregón1,6 , Elena Panizo2 , Paula
tria; 5 University Hospital Bonn, Section Of Pediatric Pathology, Department González-Urdiales1,3 , Ricardo López-Almaraz1,3 , Jimena De Pedro1,3 ,
Of Pathology, Bonn, Germany; 6 University Children’s Hospital - UKT Tue- Rosa Adan-Pedroso1,3 , Miguel García-Ariza1,3 , Itziar Astigarraga1,3,7 ,
bingen, Paediatric Surgery And Paediatric Urology, Tuebingen, Germany; Ana Patiño-García2,4 , Olatz Villate1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S238 of S635 ABSTRACTS

1 Biocruces Bizkaia Health Research Institute, Pediatric Oncology Group, Burca Aydin1 , Yunus Gunes1 , Mehmet Ayvaz2 , Ustun Aydingoz3 ,
Barakaldo, Spain; 2 Clinica Universidad de Navarra, Department Of Pedi- Kemal Kosemehmetoglu4 , Nilgun Kurucu1 , Bilgehan Yalçın1 , Ali
atrics, Pamplona, Spain; 3 Hospital Universitario Cruces, Pediatric Hema- Varan1 , Mazhar Tokgozoglu2 , Tezer Kutluk1
tology And Oncology, Barakaldo, Spain; 4 Universidad de Navarra, Cima 1 Hacettepe University Faculty of Medicine, Pediatric Oncology, Ankara,
Lab Diagnostics, Pamplona, Spain; 5 Hospital Universitario de Cruces, Turkey; 2 Hacettepe University Faculty of Medicine, Orthopaedic Surgery,
Osakidetza, Department Of Pathology, Barakaldo, Spain; 6 Faculty of Ankara, Turkey; 3 Hacettepe University Faculty of Medicine, Radiology,
Medicine and Nursing. University of the Basque Country (UPV/EHU), Ankara, Turkey; 4 Hacettepe University Faculty of Medicine, Pathology,
Department Of Physiology, Leioa, Spain; 7 Faculty of Medicine and Nursing. Ankara, Turkey
University of the Basque Country (UPV/EHU), Pediatrics Department, Leioa,
Spain Background and Aims: To evaluate the treatment results of two
regimens for the treatment of osteosarcoma in our center: cis-
Background and Aims: Although sarcomas can occur in the setting platin/doxorubicin (AP) and EURAMOS regimens.
of a cancer predisposition syndrome, this association is often missed. Methods: A file search of 90 patients with newly diagnosed osteosar-
The aim of this study was to identify pathogenic germline variants coma performed and demographic features, surgical procedures,
in pediatric patients with sarcomas, through the analysis of cancer response to treatment and outcome of patients retrospectively ana-
susceptibility genes included in somatic panels, using next-generation lyzed. 59 patients treated with AP regimen from January 2000 to
sequencing (NGS). October 2015 and 31 patients treated with EURAMOS regimen from
Methods: Forty-three pediatric and young adult patients with differ- October 2015 to December 2020 were included into the study.
ent sarcoma subtypes were included in the analysis. Tumor profiling Results: The median age of the patients was 12.9 years (6.5-17.9) and
was performed using the Oncomine™ Childhood Cancer Research M/F ratio was 1.5/1. Primary tumor sites were femur in 51%, tibia in
Assay (Thermo Fisher). Sequencing results were reviewed for poten- 23% and humerus in 11% of the patients; 29% of the patients had
tial germline alterations in genes associated with cancer predisposition metastatic disease, mostly in lungs (19%) and bone (12%). There were
syndromes (CPS). Jongmans’ criteria were considered for patient 59 patients treated with AP regimen and 31 received EURAMOS reg-
selection. imen. Limb-sparing surgery and amputation were performed for 79%
Results: 43 patients were included: 18 were diagnosed with osteosar- and 11% of patients. Local tumor resection could be performed in 7
coma (41.9%), 12 Ewing’s sarcoma (27.9%), 4 rhabdomyosarcoma patients. Histological response to treatment was good in 32% and poor
(9.3%) and 9 other sarcoma (20.9%). Gender: 27 males/16 females. in 69% of the patients, and negative margins could be obtained in
Mean age: 12 years (range 0.6-30.8). Eleven patients were metastatic 94%. No statistical difference for tumor necrosis was found between
at diagnosis (25.6%). Primary sites included lower extremities (53.5%), two regimens. The 5-y EFS and OS rates for all patients were 32.9%
upper extremities (23.3%) and trunk (14%). We identified four patients and 59.6%, respectively. The 5-y EFS and OS rates were 39.9% and
with a family history of cancer, one of which had congenital anoma- 65.3% for patients with non-metastatic disease whereas same rates
lies and excessive toxicity to treatment. Three patients suffered from were 16.9% and 45.7% for metastatic disease (p=0.01, p=0.009). For
excessive toxicity to therapy, one of them had congenital anomalies patients with non-metastatic osteosarcoma 5-y OS rates were 63.6%
and other presented two malignant tumors. Sixteen patients were and 76.2% with AP and EURAMOS regimens, and 5-y EFS rates were
selected with potentially germline pathogenic variants in the fol- 43.1% and 26.5%, respectively.
lowing genes: CDKN2A, NF1, NF2, RB1, SMARCA4, SMARCB1 and Conclusions: Although AP regimen provided considerably high sur-
TP53. Variants were subsequently analyzed in DNA from peripheral vival rates in our group of patients, outcome with EURAMOS regimen
blood. Three patients were excluded due to sample unavailability. increased 5-y OS rate to 76.2% in non-metastatic osteosarcoma. The
The variants in NF1 and CDKN2A in two patients diagnosed with outcome of our patients is comparable with EURAMOS results.
malignant peripheral nerve sheath tumor and osteosarcoma, respec-
tively, were detected in the germline, confirming the diagnosis of a
CPS. EP197 / #1242 THE DETERMINANTS OF TIME TO
Conclusions: The analysis and filtering of somatic mutations in pedi- DIAGNOSIS AND ITS IMPACT ON CLINICAL OUTCOMES IN
atric tumors according to clinical and molecular criteria, could help BONE SARCOMAS: A RETROSPECTIVE OBSERVATIONAL STUDY
to identify pathogenic germline variants in sarcoma. Identifying CPS OF 1329 PATIENTS
is important for the management and genetic counselling of these
patients and their families. Sameer Bakhshi1 , Archana Sasi1 , Shuvadeep Ganguly1 , Sandeep
Agarwala2 , Shah Khan3 , Venkatesan Kumar3 , Deepam Pushpam4
1 All India Institue of Medical Sciences, Medical Oncology, New Delhi, India;

EP196 / #1321 TREATMENT RESULTS OF PEDIATRIC 2 All India Institute of Medical Sciences, Department Of Pediatric Surgery,
OSTEOSARCOMA: COMPARISON OF CISPLATIN/DOXORUBICIN New Delhi, India; 3 All India Institue of Medical Sciences, Orthopaedics, New
AND EURAMOS REGIMENS IN TWO TIME PERIODS Delhi, India; 4 AIIMS, New Delhi, Medical Oncology, Delhi, India
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S239 of S635

Background and Aims: Delayed disease presentation is common diagnosis, lung involvement was present in eight patients, bone marrow
among cancer patients in developing countries. This study aims to iden- in one patient, lymph nodes in two patients, and multi-bone involve-
tify the determinants of time to diagnosis and explore its impact on ment in five patients; four patients were non-metastatic. Treatment of
survival outcomes in bone sarcomas. all patients was started with neoadjuvant chemotherapy. Surgery could
Methods: This is a retrospective single-centre observational study not be performed in seven patients due to extensive disease or mor-
performed in India on subjects with bone sarcomas. We extracted bid surgery; five patients underwent surgery and the rate of necrosis
clinico-demographic details, symptom duration before presentation, was above 95% in two patients and below in three patients. Eleven
and treatment outcomes from hospital records of patients registered patients received radiotherapy to the primary site and/or metasta-
between 2003 and 2018. We performed stepwise univariable and mul- sis areas simultaneously with chemotherapy. In the follow-up, two
tivariable logistic regression to identify the determinants of time to patients developed relapse after treatment discontinuation, and seven
diagnosis. Cox regression analysis was used to identify the impact of patients had progressive/refractory disease. Four patients died due to
time to diagnosis on overall survival. progressive disease, one patient died during neoadjuvant chemother-
Results: A total of 1329 subjects were analysed including 472 patients apy due to typhitis, three patients were out of follow-up as diseased,
(35.5%) with osteosarcoma and 857 patients (64.5%) with primitive four patients are sick and their treatment continues. Two surviving
neuroectodermal tumour (PNET). The median age was 16 years (range: patients have been followed up without any problem for 6 and 3 years,
0.1-71 years) with 92% of patients below 30 years and male:female respectively, since the treatment cut. Overall survival rate was 21% in
ratio of 2.3. Median time to diagnosis was 4 months (IQR: 3-7 months). 3 year; 5 years was 7%.
On univariable logistic regression, age above 16 years, absence of fever, Conclusions: Many studies have investigated the effects of multiple
lower total leukocyte count (<11000/mm3 ), rural residence and dis- factors on survival in patients with pelvic ES, including tumor size, pres-
tance from treating centre over 197 km were associated with longer ence of metastases at diagnosis, and treatment modalities. Presence of
time to diagnosis (more than 4 months). Among these, older age metastasis at the time of diagnosis was found to be the most important
(HR 2.53, p-value <0.01) and rural residence (HR 0.75, p-value=0.04) prognostic factor, and 71% of our cases were metastatic at the time of
remained significant on multivariable analysis. There was no differ- diagnosis, and our 5-year overall survival rate was 7%.
ence in diagnosis interval based on disease type (osteosarcoma versus
PNET), stage (localised versus metastatic) or gender. There was no sig-
nificant association between time to diagnosis and outcomes in the EP199 / #118 A RETROSPECTIVE REVIEW TO MONITOR
whole cohort or in subsets based on disease type or disease stage. THE IMPACT OF EARLY NASOGASTRIC TUBE FEEDING IN THE
Conclusions: Among patients with bone sarcomas, older age and rural ACUTE MYELOID LEUKAEMIA COHORT
residence are associated with delayed time to diagnosis. However,
diagnostic delay does not impact disease survival, suggesting the dom- Julie Fienberg1 , Gemma Renshaw1 , Breeana Gardiner1 , Graeme
inant role of tumour biology in determining treatment outcomes, even O’Connor2
in resource-challenged situations. 1 Great Ormond Street Children’s Hospital, Dietetics, London, United King-
dom; 2 Great Ormond Street Hospital for Children, Dietetics, London, United
Kingdom
EP198 / #1794 PELVIC EWING SARCOMA: A SINGLE
CENTER EXPERIENCE Background and Aims: Background/Aims: Children diagnosed
with acute myeloid leukaemia (AML) undergo intensive chemother-
Birsen Baysal1 , Bülent Erol2 , Gülnur Tokuç1 , Nurşah Eker1 , Rabia apy, which adversely affects nutritional status; side effects include
Şenay3 anorexia, vomiting, mucositis. Proactive nasogastric tube (NG) place-
1 Marmara University Pendik Education and Research Hospital, Pediatric ment can facilitate adequate enteral nutrition provision early in
Hematology And Oncology, Istanbul, Turkey; 2 Marmara University Pendik treatment, offsetting associated malnutrition risk. Aim: to evidence
Education and Research Hospital, Orthopedics And Traumatology, istan- benefit of early enteral nutrition on nutrition outcomes.
bul, Turkey; 3 Marmara University Pendik Education and Research Hospital, Methods: Retrospective data collected from electronic records.
Pediatric Hematology And Oncology, istanbul, Turkey Patients with AML having completed first two chemotherapy cycles
between June 2019-June 2021 without any extended intensive care
Background and Aims: The pelvis is considered one of the most com- stays (>7days) included. Outcomes assessed: weight, z-score, number
mon sites of ES and has a worse prognosis than ES located in the of days on parenteral nutrition (PN), length of hospital stay.
extremity.We aimed to examine patients with pelvic ES treated with Results: Twenty-one patients met inclusion criteria; 52% male. Ages:
multidisciplinary treatment methods in our center. 2months-11years (mean = 2years). NG feeds started within 5 days
Methods: The clinical features and prognosis of 14 pelvic ES cases in 56% during cycle 1: 88% during cycle 2. Comparing patients who
diagnosed between 2010-2021 were evaluated retrospectively. received NG feeds <5days (early NG feeders) and >5 days (late NG
Results: M/F ratio was 0.75. Median age was 14,75 years(2,25years- feeders) of starting chemotherapy, no difference in number of patients
17,5years). Pain and swelling were the most common complaints. At started on PN, or number of PN days. Weight loss in both early & late
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S240 of S635 ABSTRACTS

NG feeders in cycle 1 (-0.5Z and -0.36Z respectively). Cycle 2; observed Conclusions: Our findings suggest that delay in local control beyond
weight gain in early NG feeders(+0.29Z), late NG feeders experienced 20 weeks after diagnosis predict lower EFS in patients with localized,
weight loss (-0.07Z). Irrespective of timing of NG tube insertion, paired extremity-primary osteosarcoma. Timely local control planning by a
t-test showed significant weight loss in cycle 1 (z-score -.49; p<0.000); multidisciplinary team needs to be ensured.
by cycle 2, weight loss was ameliorated with observed weight gain over-
all (z-score +0.07, p= 0.316). In both cycles, length of hospital stay was
longer in late NG feeders (cycle 1: +1day; cycle 2: +6days). EP201 / #659 NUTRITIONAL PROFILE AND OUTCOME OF
Conclusions: Sample was too small to note any significance of early PEDIATRIC PATIENTS WITH MALIGNANT BONE TUMOURS–
versus late NG placement on nutritional status. However, findings re- EXPERIENCE FROM A TERTIARY CANCER CARE CENTRE FROM
enforce importance of early nutrition support with proactive dietetic NORTH EAST INDIA
contact and nasogastric feeding in preventing ongoing significant
weight loss and potential to reduce hospital length of stay. Future Munlima Hazarika, Suhani Barbhuiyan, Sreya Mallik, Momi Barman,
research should include assessing barriers to initiation of early enteral Manoj Kalita, Kahkasha Chaudhury
nutrition. Dr. Bhubaneswar Borooah Cancer Institute, Medical & Paediatric Oncology,
Guwahati, India

EP200 / #573 DELAYED PRIMARY CONTROL IN PATIENTS Background and Aims: Pediatric patients with osteosarcoma and
WITH LOCALIZED EXTREMITY OSTEOSARCOMA IS Ewing’s sarcoma are at an increased risk for developing malnutrition.
ASSOCIATED WITH WORSE OUTCOME Early recognition and intervention are required to prevent morbid-
ity and mortality. This study aims to describe the nutritional profile
Hadeel Halalsheh1 , Taleb Ismael1 , Mohammad Boheisi2 , Ahmad and outcome of these patients with special emphasis on nutritional
Shehadeh3 , Iyad Sultan1 intervention.
1 King Hussein Cancer Center, Pediatric, Amman, Jordan; 2 King Hussein Methods: In this retrospective study, 104 pediatric patients attend-
Cancer Center, Nursing, Amman, Jordan; 3 King Hussein Cancer Center, ing Dr. Bhubaneswar Borooah Cancer Institute between October 2019
Surgery/orthopedic, Amman, Jordan to February 2022 diagnosed with either osteosarcoma or Ewing’s
sarcoma were analysed. Informations were collected from pediatric
Background and Aims: Timing of local control is not well studied in oncology database. Nutritional status was assessed using WHO Z
osteosarcoma. We assessed the impact of delay of local control on scores and Frisanco chart for MUAC in children above 5 years of age.
survival outcome of patients with osteosarcoma. The data was analysed using SPSS V21 software.
Methods: We conducted a retrospective analysis of children (<18 Results: Total 104 cases were studied, of which Ewing’s sarcoma
years) with primary non-metastatic osteosarcoma of the extremities 58(55.8%) and osteosarcoma 46(44.2%). The mean age at diagno-
who presented to King Hussein Cancer Center (KHCC) from Jan2005 sis was 13.9+/- 4.13 years. The male: female ratio was 1.2:1. At
until Mar2020. Patients were treated according to EURAMOS-1 proto- presentation 22(21.2%) were well nourished and 82(78.8%) were mal-
col. Patients’ demographics, disease characteristics and outcomes were nourished. Amongst the female children,72% and males,78.8% were
collected. Events were defined as death, progression or relapse. Cox malnourished. High,low and moderate calorie interventions were given
proportional hazards regression was used for univariate and multivari- to 82(78.8%), 20(19.2%) and 2(1.9%) patients respectively. Out of
ate comparison of different covariates. Data collection and analysis 104 patients,39(37.5%) have completed treatment, 35(33.7%) did not
were done in December 2021. complete treatment, 20(19.2%) are continuing treatment at present,
Results: Eighty-seven patients were included in this analysis, 45 10(9.6%) did not take any treatment. Outcome assessment shows
(51.7%) were females; median age at diagnosis was 12.5 years 56(53.8%) are alive, 34(32.7%) expired and 14(13.5%) lost to fol-
(range, 5.9-18). Eighty-three patients (95%) underwent local control low up. Significant association was found between baseline nutri-
by surgery (Limb salvage surgery in 65 (75% of total), amputation tional status and good outcome (p=0.03) and baseline nutritional
in 18 (20%)), four patients (5%) refused amputation; one of them status and completion of treatment (p=0.028). Significant association
had radiotherapy as local control. After a median follow up of 50.7 was seen between pre intervention and post intervention nutri-
months (range, 5.5-189.9) the 5-year event free survival (EFS) and tional status in osteosarcoma( p=0.005). No significant association
overall survival (OS) were 53%+/-5.7% and 64%+/-6% respectively. was observed between gender and nutritional status/completion of
On univariate analysis local control before 20 weeks, and good histo- treatment/outcome.
logic response (≥90% necrosis) were associated with better EFS rates Conclusions: In developing countries, malnutrition is a major comor-
(p value 0.005 and 0.001 respectively) and OS (p, 0.049 and 0.002, bidity among children. Nutritional status plays an important role in
respectively). On multivariate analysis, both were associated with bet- cancer treatment compliance and outcome. Therefore, nutritional
ter EFS (p, 0.03 and 0.006); however, only good histologic response was interventions should be planned at individual level and population level
associated with better OS (p, 0.003). as well by authority to improve survival and enhance compliance.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S241 of S635

EP202 / #1384 VALUE OF RADIOTHERAPY DOSE ON THE analyzed with univariable and multivariable Cox regression models
OUTCOME IN EWING SARCOMA including known prognostic factors age, sex, tumor volume, surgical
margins and histological response.
Josephine Kersting1 , Andreas Ranft2,3 , Vivek Bhadri4 , Benedicte Results: S and RT was performed in 336 patients (70.4%), and 145
Brichard5 , Stephane Collaud3 , Sona Cyprova6 , Hans Eich7 , Torben patients (29.6%) received definite RT. In the S&RT group radiother-
Ek8 , Hans Gelderblom9 , Jendrik Hardes3,10 , Lianne Haveman11 , apy dose was (a) </=53Gy in 193 (57.4%), (b) 54-58Gy in 118 (35.1%)
Wolfgang Hartmann12 , Peter Hauser13 , Heribert Jurgens14 , Jukka and (c) >/= 59Gy in 25 (7.4%) pts. In the RT group radiotherapy
Kanerva15 , Thomas Kühne16 , Anna Raciborska17 , Jelena Rascon18 , dose was (a) in 17 (11.7%), (b) in 64 (44.1%) and (c) in 64 (44.1%)
Arne Streitbürger3,10 , Beate Timmermann3,19 , Yasmine Uhlenbruch20 , pts. 3y-EFS of the S&RT group was .76 (SE=.03) for (a), .76 (SE=.04)
Philip Heesen21,22 , Victor Rechl1 , Uta Dirksen2,3 for (b), and .69 (SE=.10) for (c) (P=.57), and in the RT group .58
1 University Duisburg-Essen, Faculty Of Medicine, Essen, Germany; 2 West (SE=.13), .69 (SE=.06), and .73 (SE=.06) (P=.63), respectively. Mul-
German Cancer Center, University Hospital Essen, Pediatrics Iii, Essen, Ger- tivariable Cox regression revealed age >/= 15years (HR=2.3 95%CI
many; 3 German Cancer Consortium, Partnersite Essen, Essen, Germany; 1.2-4.4), non-radical margins (HR=2.6 95%CI 1.4-5.0) for the S&RT
4 University of Sydney, Chris O’ Brien Lifehouse, Camperdown, Australia group (Sex P=.81, Histological response P=.20, Tumor volume P=.13,
Faculty Of Medicine And Health, Camperdown, Australia; 5 Université Dose P=.35), and large tumor volume (HR 2.2 (95%CI 1.2-4.0) for
Catholique de Louvain, Cliniques Universitaires Saint Luc, Department Of the RT group as independent factors (Dose P=.15, Age P=.08, Sex
Pediatric Haematology And Oncology, Brussels, Belgium; 6 Charles Uni- P=.40).
versity, Motol Children’s Hospital, Prague, Czech Republic; 7 University Conclusions: We found no impact of RT dose on survival outcomes in
Hospital Muenster, West German Cancer Center Network, Radiotherapy our cohort, whether patients were treated with combined local therapy
And Radiooncology, Münster, Germany; 8 Queen Silvia Children’s Hospital, modality or definitive radiotherapy. The upcoming iEuroEwing trial will
Childhood Cancer Center, Gothenburg, Sweden; 9 Leiden University Med- assess the value of different radiotherapy dose in a randomized manner
ical Center, Dept Of Medical Oncology, Leiden, Netherlands; 10 University to control for potential selection bias.
Hospital Essen, West German Cancer Centre, Clinic Of Orthopedics, Essen,
Germany; 11 Prinses Máxima Centrum voor Kinderoncologie, Pediatric
Oncology, Utrecht, Netherlands; 12 University Hospital Muenster, West Ger- EP203 / #1534 HIGH-DOSE CHEMOTHERAPY FOLLOWED
man Cancer Center Network, Gerhard Domagk Institute For Pathology, BY AUTOLOGOUS STEM CELL TRANSPLANTATION IN
Essen, Germany; 13 Borsod-Abaúj-Zemplén County University Teaching Hos- PEDIATRIC PATIENTS WITH RELAPSED OSTEOSARCOMA
pital, Velkey László Child’s, Health Center, Miskolc, Hungary; 14 University

Children’s Hospital Münster, West German Cancer Center Network, Depart- Sung Han Kang, Young Kwon Koh, Hyery Kim, Ho Joon Im, Kyung-Nam
ment Of Pediatric Hematology And Oncology, Münster, Germany; 15 HUS Koh
Helsinki University Hospital, New Children’s Hospital, Div. Hematology And Asan Medical Center, University of Ulsan College of Medicine, Pediatrics,
Stem Cell Transplantation, Helsinki, Finland; 16 University Children’s Hos- Seoul, Korea, Republic of
pital Basel, Department Of Oncology/ Haematology, Basel, Switzerland;
17 Department of Oncology and Surgical Oncology for Children and Youth, Background and Aims: Patients with relapsed osteosarcoma have
Mother And Child Institute, Warsaw, Poland; 18 Vilnius University, Faculty poor treatment outcomes. High-dose chemotherapy with autologous
Of Medicine, Clinic Of Obstetrics And Gynecology, d, Lithuania; 19 University stem cell transplantation (HDCT/ASCT) has been used in several high-
Hospital Essen, Department Of Particle Therapy, West German Proton risk malignant solid tumors; however, few studies have evaluated their
Therapy Centre Essen (wpe), West German Caner Center (wtz), German role in the treatment of osteosarcoma. We evaluated the effectiveness
Cancer Consortium (dktk), Essen, Germany; 20 St. Josefs Hospital Bochum, of HDCT/ASCT in relapsed pediatric osteosarcoma cases.
University Hospital, Bochum, Patient Representative, Bochum, Germany; Methods: We retrospectively reviewed the medical records of 40
21 University Duisburg-Essen, Pediatrics Iii, Essen, Germany; 22 University of patients diagnosed with and treated for relapsed osteosarcoma at Asan
Zurich, Faculty Of Medicine, Zürich, Switzerland Medical Center and Samsung Medical Center from January 1996 to
July 2019.
Background and Aims: Radiotherapy (RT) is an integral part of Results: The median age of this cohort was 13.4 years (range, 6.1–
Ewing Sarcoma (EWS) therapy. The Ewing 2008 protocol recom- 18.2). The cohort’s 5-year overall survival (OS) was 51.0±0.1% during a
mended radiotherapy doses in a range of 45 to 54 Gy but some median follow-up period of 67.5 months. Twenty-five patients (62.5%)
patients (pts) received other doses of RT. We analyzed the value achieved complete remission (CR) with salvage treatment, and the 5-
of different radiotherapy doses on event-free survival (EFS) in EWS year OS was 82.4±0.1%, whereas none of the remaining 15 patients
patients. who did not achieve CR (P<0.0001) survived. Of the 25 CR cases, 15
Methods: The Ewing 2008 database consists of 534 radiotherapy- underwent subsequent HDCT/ASCT. However, there were no signif-
admitted patients with non-metastatic EWS. Recommended treatment icant differences in the 5-year OS outcomes between patients who
consisted of multimodal chemotherapy and local treatment consist- did and did not receive HDCT/ASCT (83.9±0.1%, 13/15 vs. 80.0±0.1%,
ing of surgery and (S&RT group)/or radiotherapy (RT group). EFS was 8/10, respectively; P=0.923).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S242 of S635 ABSTRACTS

Conclusions: Our results indicate that HDCT/ASCT does not signifi- Marta Podda1 , Nadia Puma1 , Elisabetta Schiavello1 , Filippo
cantly improve survival outcomes in relapsed osteosarcoma. Achieve- Spreafico1 , Carlo Morosi4 , Maura Massimino1 , Monica Terenziani3
ment of CR remains the most crucial factor for good survival outcomes. 1 Fondazione IRCCS Istituto nazionale dei Tumori, Milano, Pediatric Oncol-
ogy, milano, Italy; 2 Fondazione IRCCS Istituto Nazionale dei Tumori, Clinical
Epidemiology And Trial Organization, Milano, Italy; 3 Fondazione IRCCS
EP204 / #1193 DISIALOGANGLIOSIDE GD2 – PATTERN IN Istituto nazionale dei Tumori, Milano, Pediatric Oncology, Milano, Italy;
PEDIATRIC SOLID TUMOR 4 Fondazione IRCCS Istituto nazionale dei Tumori, Milano, Pediatric Radiol-
ogy, Milano, Italy
Svetlana Kulyova1,2 , Elizaveta Prosekina3 , Anna Artemyeva3 , Xenia
Borokshinova3 , Elena Mikchailova3 Background and Aims: The risk of survivors developing a secondary
1 Saint Petersburg State Pediatric Medical University, Department Of Chil- bone sarcoma after receiving treatments for paediatric cancers is well
dren Oncology, St. Petersburg, Russian Federation; 2 NN Petrov Research established. The aim of this study was to assess secondary osteosar-
Centre of Oncology, Children’s Oncology, St. Petersburg, Russian Federa- coma (SOS) patients’ clinical characteristics and clinical outcomes.
tion; 3 NN Petrov Research Centre of Oncology, Department Of Children Methods: The study concerns survivors with a history of primary
Oncology, St. Petersburg, Russian Federation neoplasms (PN) during childhood and adolescence treated with
chemotherapy +/- radiotherapy+/- surgery and subsequently diag-
Background and Aims: Immunotherapy using GD2-targeting anti- nosed with SOS.
bodies has become a component of up-front treatment for high-risk Results: We identified 26 survivors (13 Females, 13 Males) who devel-
neuroblastoma. GD2 has been found at varying levels of expression oped SOS at a median of 7.3 years from diagnosis of the PN, of which
on a number of other tumor types including Ewing’s sarcoma (ES), 5/7 tested had a Li-Fraumeni syndrome. They were a median 8.6 and
osteogenic sarcoma (OS), soft tissue sarcoma (STS). To date, there has 15.1 years of age when their PN and SOS were diagnosed, respec-
been limited research on GD2 expression in listed tumors. The aim of tively. Twenty four out of 26 underwent radiotherapy and 19 were
the study is to investigate the frequency of GD2 expression in pediatric treated with chemotherapy including doxorubicin, for the PN. A con-
solid tumor. sistent number of SOS was located in unfavourable sites (8 hip bone,
Methods: Patients were eligible for inclusion if they satisfied the fol- 6 skull) and all but one received chemotherapy with tailored schedule
lowing criteria: if they were less than 18 years of age and had received a and omission of doxorubicin in 20 cases and 18 out of 26 underwent
diagnosis of refractory solid tumors (5 OS, 4 ES and 4 STS). Flow cytom- surgery: Fifteen patients died of SOS The 5- and 10-year event free
etry was performed to investigate possible membranous expression of survival, overall survival probabilities (95% confidence interval) were
GD2. The percentage of GD2-positive tumor cells was assessed as a 73.1% (57.9-92.3%) / 30.8% (17.3-54.8%) and 92.3% (82.6-100%) /
continuous parameter (0–100%). 69.2% (53.6-89.5%), respectively.
Results: GD2 was found to be expressed in 10 of the 13 (76.9%) tumor Conclusions: Judging from our experience, survival probabilities after
samples. Among the tumor’s subtypes, GD2 expression was observed SOS are lower than observed in patients with primary osteosarcoma
predominantly in OS (ranged from 0% to 91.6%, mean 38.3 ± 21.2%) but not negligible. It is therefore mandatory to discuss the best choice
and STS (ranged from 4.3% to 84.2%, mean 42.5 ± 21.2%). The results of treatment for such patients in terms of their chances of cure and
of GD2 expression in ES ranged from no detectable surface expres- quality of life in a referral center.Judging from our experience, sur-
sion to diffuse and/or intense staining in some tumors (GD2 expression vival probabilities after SOS are lower than observed in patients with
levels ranged from 0.5 to 94.2% from diagnostic biopsy samples of ES, primary osteosarcoma but not negligible. It is therefore mandatory to
mean 25.1 ± 23.1%). discuss the best choice of treatment for such patients in terms of their
Conclusions: The present study revealed the presence of GD2 expres- chances of cure and quality of life in a referral center.
sion in a high proportion of pediatric solid tumors samples, with a
significantly higher proportion of GD2-positive tumors in osteogenic
sarcoma and soft tissue sarcoma versus Ewing’s sarcoma. GD2 in child- EP206 / #1078 GERMLINE VARIANTS IN PEDIATRIC
hood solid tumor might be a suitable tumor-associated antigen that PATIENTS WITH OSTEOSARCOMA REVEALED BY MULTIGENE
could be targeted on the cell surface in patients with a poor prognosis. PANEL TESTING

Vera Semenova1,2 , Tatiana Nasedkina1,2 , Elena Zhukovskaya2 ,


EP205 / #191 SECONDARY OSTEOSARCOMA: A VERY Valentina Kozlova3 , Svetlana Mikhailova3 , Alexander Karelin2
CHALLENGING CHALLENGE 1 Engelhardt Institute of Molecular Biology of the Russian Academy of
Sciences, Laboratory For Biological Microchips, Moscow, Russian Feder-
Cristina Meazza1 , Giovanna Sironi1 , Francesco Barretta2 , Olga Nigro1 , ation; 2 Dmitry Rogachev National Medical Research Center of Pediatric
Giovanna Gattuso1 , Roberto Luksch1 , Luca Bergamaschi1 , Veronica Hematology, Oncology and Immunology, Rehabilitation Center, Moscow,
Biassoni3 , Michela Casanova3 , Stefano Chiaravalli1 , Andrea Ferrari1 , Russian Federation; 3 N.N. Blokhin National Medical Research Center of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S243 of S635

Oncology, Institute Of Pediatric Oncology And Hematology, Moscow, Rus- Hematology-oncology, Heraklion, Greece; 6 National and Kapodistrian Uni-
sian Federation versity of Athens, "Aghia Sophia" Children’s Hospital, Department Of
Pediatric Hematology-oncology, Athens, Greece; 7 Mitera Children’s Hos-
Background and Aims: Osteosarcoma (OS) is the most common bone pital, Department Of Pediatric And Adolescent Hematology-oncology,
tumor in children and young adults with a peak of incidence in the Athens, Greece; 8 Aghia Sophia Children’s Hospital, Department Of Pediatric
second decade of life. Most cases are sporadic but the risk of OS is sub- Hematology-oncology (tao), Athens, Greece
stantially increased by germline variants in cancer-associated genes.
The use of next-generation sequencing (NGS) to reveal the genetic pre- Background and Aims: The survival of high-grade osteosarcoma, the
disposition to OS expands our knowledge of the molecular basis of the most common primary bone tumor in children, in the last 40 years has
disease and is important for patient’s management. reached a plateau. As a pan-European effort has recently been initiated
Methods: A total of 38 patients with OS were enrolled in the study. to further improve survival, we surveyed the common practice of the 7
There were 18 girls and 20 boys aged 3-17 years (mean age 11,3 years). pediatric oncology centers in Greece, to create standardized national
The NGS of genomic DNA was performed on the Illumina platform recommendations.
using a multigene panel of 882 cancer-associated genes. Libraries were Methods: We conducted a survey about the common practice of all
prepared via enrichment by hybridization with KAPA HyperChoice oncological centers in Greece regarding biopsy, use of p-glycoprotein,
probes (Roche). PET experience, chemotherapy, muramyl tripeptide, metastasectomy,
Results: Pathogenic and likely pathogenic mutations predisposed to palliative techniques.
cancer development were revealed in heterozygous state in 9 patients Results: The 2/7 centers usually perform core needle biopsy, 3/7 open,
(23%). Six patients had OS and at least one secondary tumor: nephrob- 2/7 both. None measures p-glycoprotein or modify treatment with
lastoma (n=2), soft tissue sarcoma (n=3), retinoblastoma (n=1). Most p-glycoprotein. The 3/7 perform PET as a routine when diagnosed,
often the mutations occurred in the TP53 gene (n=4). In two cases, the 2/7 before surgery, 2/7 at the end of treatment. All departments, use
patients with TP53 mutation had additional tumor: nephroblastoma EURAMOS protocol. The 3/7 in the past evaluated the response to
or embryonic rhabdomyosarcoma. One patient with retinoblastoma neoadjuvant treatment, with thallium scintigraphy. The 3/7 have given
at age 2 and OS at age 15 carried a novel mutation in RB1 gene muramyl tripeptide in 4 patients. The 6/7 choose amputation surgeries
(c.214_224del), leading to premature stop codon. Other potentially when there is infiltration of blood vessels and nerves (4/7), disease
affected genes were the BRCA1 (2080delA), SMARCAL1 (c.2542G>T), progression (2/7), local recurrence (3/7), as palliative (1/7). Rotation-
RAD51 (splice site mutation:c.905-2_905-1del) and ATM (c.8565T>G) plasty surgeries were used in 3/7 centers for 4 patients. All centers
genes. The pathogenic stop-gained variant in the SMARCAL1 gene was perform prosthetic and expanding surgeries in Greece, while 5/7 sent
previously described in patients with immuno-osseous dysplasia. patients in European centers in the past. Metastasectomy is performed
Conclusions: Germline mutations in cancer associated genes were after the end of neoadjuvant (1/7), after the end of adjuvant (2/7),
revealed in 23% of our patients. Significant proportion (10%) of after surgery but before the end of adjuvant (4/7). Metastasectomy
osteosarcomas were a part of Li-Fraumeni syndrome caused by alter- operations are performed with open surgery (5/7), or minimally inva-
ations in the TP53 gene. Also, mutations in other genes (RB1, BRCA1, sive technique when possible (2/7). In cases of pulmonary recurrence
RAD51, ATM) associated with hereditary cancer syndromes were 5/7 administer recurrent chemotherapy except metastasectomy, 2/7
found. omit chemotherapy individually. None used samarium, or any isotope
to relieve symptoms. The most common chemotherapy for relapse was
ifosfamide-etoposide. New treatments are used individually.
EP207 / #972 COMMON PRACTICE OF THE Conclusions: The common practice in Greece, co-insides with the usual
OSTEOSARCOMA MANAGEMENT IN PEDIATRIC ONCOLOGY practice in Europe. Patients need to be treated in new clinical trials to
CENTERS. A NATIONWIDE REPORT improve survival.

Evgenia Papakonstantinou1 , Margarita Mpaka2 , Mirella Ampatzidou3 ,


Dimitrios Doganis2 , Athanasios Tragiannidis4 , Nikolaos Katzilakis5 , EP208 / #1000 INCIDENCE AND TIME TRENDS OF
Efthymia Rigatou6 , Eleni Dana7 , Emmanuel Hatzipantelis4 , Helen OSTEOSARCOMA. COMPARISON DATA OF SEER, USA WITH
Kosmidis7 , Eftichia Stiakaki5 , Sophia Polychronopoulou8 , Antonis THE GREEK REGISTRY NARECHEM-ST
Kattamis6 , Kyriaki Kotsoglanidou1
1 Ippokratio Hospital, Department Of Pediatric Oncology, Thessaloniki, Evgenia Papakonstantinou1 , Georgios Markozannes2 , Evdoxia
Greece; 2 ‘P & A. Kyriakou’ Children’s Hospital, Department Of Pedi- Bouka3 , Margarita Mpaka4 , Kleoniki Athanasiadou1 , Narechem-St
atric Hematology-oncology, Athens, Greece; 3 Aghia Sophia Children’s Collaborating Group5,6,7,8,9,10,11,12,13,14,15 , Evangelia Ntzani2,16 , Eleni
Hospital, Department Of Pediatric Hematology-oncology, Athens, Greece; Petridou17
4 Aristotelion University of Thessaloniki, AHEPA General Hospital„ Depart- 1 Ippokratio Hospital, Department Of Pediatric Hematology- Oncology,
ment Of Pediatric Hematology-oncology, Thessaloniki, Greece; 5 University Thessaloniki, Greece; 2 School of Medicine, University of Ioannina, Depart-
Hospital of Heraklion, University of Crete, Department Of Pediatric ment Of Hygiene And Epidemiology, Ioannina, Greece; 3 and Health
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S244 of S635 ABSTRACTS

Promotion, Hellenic Society For Social Pediatrics, Athens, Greece; 4 “P Conclusions: Variations in the descriptive epidemiology between the
and A Kyriakou” Children.s Hospital, Department Of Pediatric Hematol- two examined registries with accessible final primary data as well as
ogy And Oncology, Athens, Greece; 5 “P&A Kyriakou” Children’s Hospital, with published data derived from other European countries may imply
C. Zabakidis, A’ Orthopedic Clinic, Athens, Greece; 6 Children’s Hospital variable risk factors shaping the respective geographical and temporal
“P. & A. Kyriakou”, A. Alexopoulou, Children’s & Adolescents Radiother- patterns and need to be further explored.
apy Department, Athens, Greece; 7 ’Mitera’ Children’s Hospital, V. Galani,
Department Of Pediatric And Adolescent Hematology-oncology, Athens,
Greece; 8 Agia Sofia Children’s Hospital, M. Gavra, A. Malama, Depart- EP209 / #1018 SURVIVAL OF OSTEOSARCOMA IN GREECE:
ment Of Medical Imaging And Interventional Radiology, Athens, Greece; PRELIMINARY DATA FROM THE NATIONWIDE REGISTRY FOR
9 AHEPA Hospital, E. Hatzipantelis, M. Vousvouki, Department Of Pediatric CHILDHOOD HEMATOLOGICAL MALIGNANCIES AND SOLID
And Adolescent Hematology-oncology, Thessaloniki, Greece; 10 National TUMORS (NARECHEM-ST; 2010-2020)
and Kapodistrian University of Athens, M. Moschovi, P. Kourou, Pedi-
atric Hematology-oncology Unit, First Department Of Pediatrics, Athens, Evgenia Papakonstantinou1,2 , Georgios Markozannes3 , Evdoxia
Greece; 11 “P. and A. Kyriakou” Children’s Hospital, E. Magkou, M. Nikita, Bouka4 , Margarita Mpaka5 , Kleoniki Athanasiadou1 , Narechem-St
Department Of Pediatric Hematology-oncology, Athens, Greece; 12 “P. and Collaborating Group6,7,8,9,10,11,12,13,14,15,16 , Evangelia Ntzani2,3,17 ,
A. Kyriakou” Children’s Hospital, J. Michelarakis, B’ Orthopedic Clinic, Eleni Petridou2,18
Athens, Greece; 13 “Aghia Sophia” Children’s Hospital, S. Polychronopoulou, 1 Ippokratio Hospital, Department Of Pediatric Hematology- Oncology,
V. Tzotzola, Department Of Pediatric Hematology-oncology, Athens, Greece; Thessaloniki, Greece; 2 Hellenic Society for Social Pediatrics and, Health
14 “Aghia Sophia” Children’s Hospital, K. Stefanaki, Department Of Pathol- Promotion, Athens, Greece; 3 School of Medicine, University of Ioannina,
ogy, Athens, Greece; 15 “P. and A. Kyriakou” Children’s Hospital, A. Strantzia, Department Of Hygiene And Epidemiology, Ioannina, Greece; 4 Hellenic
Pathology Laboratory, Athens, Greece; 16 Brown University, Center For Evi- Society for Social Pediatrics and Health Promotion, Hellenic Society For
dence Synthesis In Health, Providence, United States of America; 17 Health Social Pediatrics And Health Promotion, Athens, Greece; 5 “P and A Kyri-
Promotion, Hellenic Society For Social Pediatrics And, Athens, Greece akou” Children.s Hospital, Department Of Oncology, Athens, Greece; 6 “P&A
Kyriakou” Children’s Hospital, C. Zabakidis, A’ Orthopedic Clinic, Athens,
Background and Aims: Despite success stories in prevention, screen- Greece; 7 Children’s Hospital “P. & A. Kyriakou”, A. Alexopoulou, Chil-
ing, and treatment, cancer remains the second leading cause of dren’s & Adolescents Radiotherapy Department, Athens, Greece; 8 ’Mitera’
childhood (0-14 years) death with increasing incidence trends for sev- Children’s Hospital, V. Galani, Department Of Pediatric And Adolescent
eral disease types. Preliminary data on the descriptive epidemiology Hematology-oncology, Athens, Greece; 9 Agia Sofia Children’s Hospital,
of childhood osteosarcoma are reported in Greece and compared to M. Gavra, A. Malama, Department Of Medical Imaging And Interven-
those of the SEER, USA. tional Radiology, Athens, Greece; 10 AHEPA Hospital, E. Hatzipantelis, M.
Methods: From 839 osteosarcoma cases recorded in SEER collabo- Vousvouki, Department Of Pediatric And Adolescent Hematology-oncology,
rating registries (1975–2017), data on 204 cases (2010-2017) were Thessaloniki, Greece; 11 National and Kapodistrian University of Athens, M.
extracted for the comparative analyses. Nationwide registry for child- Moschovi, P. Kourou, Pediatric Hematology-oncology Unit, First Depart-
hood hematological malignancies and solid tumors (NARECHEM-ST) ment Of Pediatrics, Athens, Greece; 12 “P. and A. Kyriakou” Children’s
data comprise 55 incident cases reported during 2010-2020 in 6 Hospital, E. Magkou, M. Nikita, Department Of Pediatric Hematology-
pediatric Hematology-Oncology Centers and alternative data sources, oncology, Athens, Greece; 13 “P. and A. Kyriakou” Children’s Hospital, J.
apart from the island of Crete accounting for 5% of the total pediatric Michelarakis, B’ Orthopedic Clinic, Athens, Greece; 14 “Aghia Sophia” Chil-
cancer burden. Preliminary analyses were performed for the com- dren’s Hospital, S. Polychronopoulou, V. Tzotzola, Department Of Pediatric
parison of the percentage of cases across the two registries and the Hematology-oncology, Athens, Greece; 15 “Aghia Sophia” Children’s Hospi-
distribution of cases by age and sex. tal, K. Stefanaki, Department Of Pathology, Athens, Greece; 16 “P. and A.
Results: The annual percentages of cases in both registries did Kyriakou” Children’s Hospital, A. Strantzia, Pathology Laboratory, Athens,
not show any significant differences overall for the overlapping Greece; 17 Brown University, Center For Evidence Synthesis In Health, Provi-
years (goodness-of-fit-p=0.95), whereas differences were noted with dence, United States of America; 18 Medical School, National & Kapodistrian
regards to sex and age distribution among the NARECHEM-ST registry University of Athens, Department Of Hygiene, Epidemiology And Medical
which comprised mainly Caucasian populations. Most NARECHEM-ST Statistics, Athens, Greece
cases (82%) were diagnosed after the 10th year of life, without sig-
nificant reduction of the annual percentage of cases for smaller ages Background and Aims: Osteosarcoma is the most common primary
(p=0.469); median age at diagnosis was higher in NARECHEM-ST com- bone sarcoma. We aimed to assess the 5-year overall survival (OS)
pared to SEER (12, 11respectively). A larger male preponderance was and explore prognostic variables among incident cases with high-
estimated in NARECHEM-ST compared to SEER in all age groups (male: grade osteosarcoma aged 0-14 years reported in the NARECHEM-ST
female=1.12 vs 1.04) and in cases 10-14 years (1.25 vs 1.14). The over- Registry.
all NARECHEM-ST AIR was 2.8 without a significant change in the Methods: Preliminary data of the 55 incident pediatric patients (0-14
linear trend (p=0.93). years) with confirmed by biopsy high-grade osteosarcoma, diagnosed
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S245 of S635

between 2010 and 2020 and followed-up to 31-12-2021 in six Pedi- Additionally, Spatial RNA-sequencing was performed to determine
atric Hematology- Oncology Departments were reviewed. One patient localization and potential interactions of cells in TME.
was lost to follow up. Descriptives for age, sex, primary site, stage, Results: Our single-cell RNA-sequencing analysis revealed distinct cell
treatment and survival outcomes were examined; Kaplan-Meier curves types in primary and metastatic osteosarcoma tissues including tumor,
were constructed, and 5-year overall survival (OS) and local and distant endothelial, immune cells, and fibroblasts. We found an impaired
relapse-free survival (RFS) were estimated. immune response with a defective antigen presentation, immunoreg-
Results: The median age at diagnosis was 12 years (4–14 years) and ulatory DCs, pro-tumoral macrophages and increased number of
the male-to-female ratio was 1.12. The most common sites at diag- T-regulatory cells. Furthermore, spatial RNA-sequencing analysis, in
nosis were the extremities (47/55, 85%), skull (3/55, 5%) and trunk combination with single cell data, shows a complex network of inter-
(5/55, 9%), whereas 8 patients had primary metastatic disease (15% actions between tumor cells and the rest of TME cell populations,
stage III). Surgical resection of the primary tumor was performed in especially immune cells.
53 patients. Most of the patients received EURAMOS based proto- Conclusions: Our work identifies an impaired immune response
col. Good response to chemotherapy (necrosis >90%) was recorded in against tumoral cells and a complex network of interactions in TME.
almost 40% cases. Ten patients had a local relapse (70% died), while 20 These findings provide valuable insights on how tumoral cells can inter-
had a relapse on other sites (75% died). At the last follow up (median act with other cells in TME. The pathways through which tumor induces
4 years, range: 0.4-11.8 years) 34 patients were alive. The 5-year OS immunoregulatory phenotype of DCs or pro-tumoral macrophages can
exceeded 63% (95% CI: 48%-75%). The 5-year OS was poorer (41%, be a potential therapeutic target that we need to explore.
p=0.3) in patients with metastatic or relapsed disease. The 5-year
local and distant RFS were 81% (95% CI: 67%-59%) and 62% (95% CI:
47%-73%) respectively. EP211 / #1609 PEDIATRIC BONE AND SOFT TISSUE
Conclusions: OS and RFS of patients with osteosarcoma in Greece is SARCOMAS: CLINICAL PROFILE, MANAGEMENT AND
comparable to that reported by major collaborative trials. As expected, OUTCOME; EXPERIENCE FROM A TERTIARY CARE CENTER IN
survival is influenced by the presence of metastases, and relapsed dis- INDIA
ease. Ongoing clinical cancer registration could facilitate comparison
of outcomes between different study groups and modify treatment. Shweta Pathak1,2 , Pooja Mallya1 , Ravi Joshi1 , Shobha Bediger1 ,
Swathi M1 , Monika Bhukar1 , Gayathri S1 , Sunil Bhat2
1 Narayana health hospital, Paediatric Hemato-oncology And Bmt, Ban-
EP210 / #681 SINGLE-CELL TRANSCRIPTOME ANALYSIS OF glore, India; 2 Mazumder Shaw Medical Center, Paediatric Hemato-oncology
TUMOR AND STROMAL COMPARTMENTS OF OSTEOSARCOMA And Bmt, Bangalore, India
PRIMARY TUMORS AND METASTATIC LESIONS
Background and Aims: Sarcomas are a heterogeneous group of rare
Mónica Pascual García1 , Marc Elosúa2 , Holger Heyn2 , Jaume Mora3 , tumors that arise predominantly from the embryonic mesoderm. The
Salvador Aznar-Benitah1 aim of this study was to analyze the clinical features and outcome of
1 IRB Barcelona, Stem Cells And Cancer, Barcelona, Spain; 2 CNAG, Single pediatric sarcomas.
Cell Group, Barcelona, Spain; 3 Pediatric Cancer Center Barcelona, Hospital Methods: This was a retrospective study conducted at Mazumdar
Sant Joan de Deu, Oncology, Barcelona, Spain Shaw Medical Center at Bangalore, India. Data of all pediatric patients
with bone and soft tissue sarcomas were analyzed from January 2015
Background and Aims: Osteosarcoma is a malignant bone tumor that to February 2022.
most commonly affects children, adolescents, and young adults. The Results: A total of 68 cases were analyzed; 45 were bone sarco-
current standard of care involves surgical resection of the primary mas (62% osteosarcoma and 38% Ewing’s sarcoma) and 23 were
tumor and multi-agent chemotherapy which can result in 5-year sur- soft tissue sarcomas (91% rhabdomyosarcomas ). The male female
vival rates up to 70% for patients with localized disease. However, ratio was 1.1:1. The mean age of presentation of bone tumor was
approximately 20% of patients have clinical detectable metastases at 13 years and soft tissue sarcoma was 7 years. Most common site
diagnosis. In addition, tumors will relapse and generate metastasis in being distal femur 59%(n=26) in bone tumors and orbit 38% (n=8)
a third of patients with localized disease at the time of diagnosis. In in rhabdomyosarcoma.12 patients presented with metastatic disease
patients with metastasis, more than 85% of the metastases occur in the (5 cases of osteosarcoma, 3 cases of Ewing’s sarcomas and 4 cases
lungs and tend to be resistant to chemotherapy. Importantly, the sur- of Rhabdomyosarcomas) with lung being the most common site of
vival of patients with metastatic osteosarcoma has remained virtually metastasis. Among 21 cases of rhabdomyosarcoma;52% presented
unchanged over the past 30 years, with an overall 5-year survival rate as high risk, 38% with intermediate risk and 9.5% presented with
of less than 20%. recurrent disease. All cases of Rhabdomyosarcomas and Ewing’s sar-
Methods: In this study, we employed a single-cell RNA-sequencing comas were treated with COG protocol based on risk stratification,
technology to profile the transcriptomes of individual cells from dis- and Osteosarcomas were treated with Methotrexate based regimen.
sociated primary or metastatic tumors of K7M2 osteosarcoma model. Good histopathologic response (necrosis ≥90%) was achieved in 59%
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S246 of S635 ABSTRACTS

nonmetastatic and 56% metastatic patients .6 cases relapsed (4 chil- Background and Aims: Ewing Sarcoma (EWS) is a rare and highly
dren with rhabdomyosarcoma, 2 with osteosarcoma and 1 with Ewing’s malignant bone tumor. It represents the second most common bone
sarcoma). At a median follow-up of 48 months, the overall survival and tumor in children and adolescents following osteosarcomas. The
disease-free survival of Osteosarcoma was 82% and 75%, Ewing’s sar- sacrum as primary is very rare. Due to the anatomical location, local
coma was 88% and 82% and soft tissue sarcomas was 82% and 65% treatment is challenging especially regarding surgery.
respectively. Methods: To analyze prognostic factors we retrospectively analyzed
Conclusions: The formation of disease management groups/clinics databases of EURO-E.W.I.N.G. 99 and EWING 2008. Both clinical tri-
focused on sarcomas has resulted in better understanding and manage- als included 124 patients (pts) with localized (n=70) or metastasized
ment of these uncommon tumors hence resulted in better outcome in (n=53) sacral EWS. All pts received systemic treatment according to
terms of overall survival. the protocols. For local control 64.3% received definitive radiother-
apy (followed by combined modality treatment (25.2%)) and surgery
alone (4.3%). Some pts had no local treatment (6.1%) mainly due to
EP212 / #1453 FACTORS INFLUENCING OUTCOME OF early relapse. The study endpoint was event free survival (EFS). Fac-
PATIENTS WITH PRIMARY EWING SARCOMA OF THE SACRUM tors probably associated with survival e.g., age, sex, tumor volume, local
treatment modality and applied study protocol were included in the
Victor Rechl1 , Andreas Ranft2,3 , Vivek Bhadri4 , Benedicte Brichard5 , univariate and multivariable analyses.
Stephane Collaud3 , Sona Cyprova6 , Hans Eich7 , Torben Ek8 , Hans Results: Age under 18 years was associated with better outcome (3y-
Gelderblom9 , Jendrik Hardes3,10 , Lianne Haveman11 , Wolfgang EFS: .45 vs .12; P=.03) in patients with metastases at diagnosis. In
Hartmann12 , Peter Hauser13 , Heribert Jurgens14 , Jukka Kanerva15 , general, metastases at diagnosis (3y-EFS: .33 vs .68; P<.001; HR=3.4,
Thomas Kühne16 , Anna Raciborska17 , Jelena Rascon18 , Arne 95% CI 1.7 to 6.6), large tumor volume (3y-EFS: .36 vs .69; P=.002;
Streitbürger3,10 , Beate Timmermann3,19 , Yasmine Uhlenbruch20 , HR=2.1, 95% CI 1.1 to 4.0) or age ≥ 18 years (3y-EFS: .41 vs .60; P=.10;
Josephine Kersting1 , Uta Dirksen2,3 HR=2.6, 95% CI 1.3 to 5.2) were associated with dismal outcome. Inter-
1 University Duisburg-Essen, Faculty Of Medicine, Essen, Germany; 2 West action was seen in patients with definitive radiotherapy compared to
German Cancer Center, University Hospital Essen, Pediatrics Iii, Essen, Ger- other patients by a higher EFS in localized disease in contrast to a lower
many; 3 German Cancer Consortium, Partnersite Essen, Essen, Germany; EFS in metastatic patients (P<.001).
4 University of Sydney, Chris O’ Brien Lifehouse, Camperdown, Australia Conclusions: Young age is associated with a better outcome and inter-
Faculty Of Medicine And Health, Camperdown, Australia; 5 Université action was observed between definitive radiotherapy and metastases
Catholique de Louvain, Cliniques Universitaires Saint Luc, Department Of at diagnosis. Regarding local therapy modality, the anatomical location
Pediatric Haematology And Oncology, Brussels, Belgium; 6 Charles Uni- is decisive and the majority of pts received definitive radiotherapy.
versity, Motol Children’s Hospital, Prague, Czech Republic; 7 University
Hospital Muenster, West German Cancer Center Network, Radiotherapy
And Radiooncology, Münster, Germany; 8 Queen Silvia Children’s Hospital, EP213 / #1918 ANALYZING METHOTREXATE LEVELS: A
Childhood Cancer Center, Gothenburg, Sweden; 9 Leiden University Med- LITERATURE REVIEW
ical Center, Dept Of Medical Oncology, Leiden, Netherlands; 10 University

Hospital Essen, West German Cancer Centre, Clinic Of Orthopedics, Essen, Christineil Thompson Md, Alexis Kuhn Pharmd, Ashley Walter,
Germany; 11 Prinses Máxima Centrum voor Kinderoncologie, Pediatric Catherine Martin
Oncology, Utrecht, Netherlands; 12 University Hospital Muenster, West Ger- Mayo Clinic, Pediatrics, Rochester, United States of America
man Cancer Center Network, Gerhard Domagk Institute For Pathology,
Essen, Germany; 13 Borsod-Abaúj-Zemplén County University Teaching Hos- Background and Aims: Methotrexate is an antineoplastic anti-
pital, Velkey László Child’s, Health Center, Miskolc, Hungary; 14 University metabolite commonly used at high doses in pediatric oncology for the
Children’s Hospital Münster, West German Cancer Center Network, Depart- treatment of certain malignancies such as osteosarcoma, CNS malig-
ment Of Pediatric Hematology And Oncology, Münster, Germany; 15 HUS nancies and lymphomas. Toxicity from methotrexate is widespread
Helsinki University Hospital, New Children’s Hospital, Div. Hematology And involving multiple systems notably including the gastrointestinal,
Stem Cell Transplantation, Helsinki, Finland; 16 University Children’s Hos- hematologic, renal, mucocutaneous and neural systems. Glucarpidase
pital Basel, Department Of Oncology/ Haematology, Basel, Switzerland; reduces the methotrexate concentration by cleaving it into non-toxic
17 Department of Oncology and Surgical Oncology for Children and Youth, metabolites, 4-deoxy-4-amino-N10-methylpteroic acid (DAMPA) and
Mother And Child Institute, Warsaw, Poland; 18 Vilnius University, Faculty glutamate which are eliminated by the liver. High performance liq-
Of Medicine, Clinic Of Obstetrics And Gynecology, d, Lithuania; 19 University uid chromatography is the gold standard for evaluating methotrexate
Hospital Essen, Department Of Particle Therapy, West German Proton levels as DAMPA cross-reacts with immunoassay measurements.
Therapy Centre Essen (wpe), West German Caner Center (wtz), German Methods: A review of existing literature limited between the years
Cancer Consortium (dktk), Essen, Germany; 20 St. Josefs Hospital Bochum, 2000 and 2022 was conducted utilizing the PubMed database using
University Hospital, Bochum, Patient Representative, Bochum, Germany the search terms “methotrexate” linked with the Boolean operator
AND to the terms “high performance liquid chromatography (HPLC)”
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S247 of S635

and “immunoassay”. Results were then analyzed for trends between (n = 30), second (n = 14), and third (n = 9) R/P were 50.3%, 51.3%,
HPLC and immunoassay measurements which recurred consistently and 46.7%, respectively. Multivariate analysis did not identify any
throughout the literature. independent risk factors affecting OS. The 5-year PFS rate of the 30
Results: The initial search resulted in 26 articles. The prominent patients after first R/P was 22.4%, and multivariate analysis identified
trends from these articles were discordance between the commonly histological subtype and curative local surgery as independent risk
used methotrexate immunoassays which overestimated the overall factors influencing PFS. The median relapse-free intervals between
methotrexate concentrations compared to HPLC. Notably, these stud- the first and second R/P and the second and third R/P were 1.06
ies demonstrated a more pronounced overestimation for methotrexate and 1.14 years, respectively. Long (> 6 months) partial response was
concentrations below 0.2 micromoles. observed in three patients treated using temozolomide+etoposide,
Conclusions: In our pediatric patients the methotrexate threshold for irinotecan+carboplatin, or regorafenib.
discharge is set to reduce the risk of toxicity. However from literature Conclusions: OS rate in the patients with osteosarcoma experiencing
HPLC is superior to the more commonly used immunoassay meth- R/P included in this study was markedly higher than that reported pre-
ods which may grossly overestimate methotrexate levels especially at viously, mainly due to the surgical total removal of tumors, even after
lower concentrations. This is of critical importance as in our pediatric subsequent R/P. The recent establishment of salvage chemotherapy
population we run the risk of unnecessarily prolonging hospital admis- or molecular targeted therapy may also increase survival rates in a
sions thus further disrupting quality of life and so must be accurately subgroup of patients.
balanced with the risk of toxicity to our patients.

EP215 / #547 CLINICAL OUTCOMES OF LOCALIZED AND


EP214 / #31 CLINICAL OUTCOME OF PATIENTS WITH METASTATIC OSTEOSARCOMA PATIENTS TREATED BY TWO
OSTEOSARCOMA EXPERIENCING RELAPSE OR PROGRESSION: CONSECUTIVE PROTOCOLS (2006 -2021). RESULTS OF THE
A SINGLE-INSTITUTE EXPERIENCE CHILEAN PEDIATRIC CANCER PROGRAM PINDA

Katsutsugu Umeda1 , Akio Sakamoto2 , Takashi Noguchi2 , Yoshinori Emma Concha1 , Katherine Kopp2 , Carolina Nicklas3 , Claudia Paris4 ,
Uchihara1 , Hirokazu Kobushi1 , Ryo Akazawa1 , Hideto Ogata1 , Satoshi Beatriz Silva2 , Jesus Ortega5 , Gloria Gonzalez6 , Elisa Alcalde7 , Jorge
Saida1 , Itaru Kato1 , Hidefumi Hiramatsu1 , Megumi Uto3 , Takashi Morales8 , Carmen Salgado9 , Pamela Silva10 , Paola Olate11 , Gustavo
Mizowaki3 , Hironori Haga4 , Hiroshi Date5 , Takeshi Okamoto6 , Cea12 , Milena Villarroel13
Kenichiro Watanabe7 , Souichi Adachi8 , Junya Toguchida9 , Shuichi 1 Hospital Luis Calvo Mackenna / Clínica Alemana de Santiago, Oncología
Matsuda2 , Junko Takita1 Pediatrìa, Santiago, Chile; 2 Hospital Luis Calvo Mackenna, Pediatric Oncol-
1 Kyoto University, Department Of Pediatrics, Kyoto, Japan; 2 Kyoto Uni- ogy, Santiago, Chile; 3 Hospital Luis Calvo Mackenna, Pediatric Oncology
versity, Orthopedic Surgery, Kyoto, Japan; 3 Kyoto University, Radiation Unit, Santiago, Chile; 4 Hospital Luis Calvo Mackenna, Oncologia, Santi-
Oncology And Image-applied Therapy, Kyoto, Japan; 4 Kyoto University, ago, Chile; 5 Hospital Luis Calvo Mackenna, Orthopedics, Santiago, Chile;
Diagnostic Pathology, Kyoto, Japan; 5 Kyoto University, Thoratic Surgery, 6 Hospital Luis Calvo Mackenna, Oncologia, santiago, Chile; 7 Hospital

Kyoto, Japan; 6 Otsu Red Cross Hospital, Orthopedic Surgery, Otsu, Japan; Luis Calvo Mackenna, Pathology, Santiago, Chile; 8 Hospital Luis Calvo
7 Shizuoka Children’s Hospital, Hematology And Oncology, Shizuoka, Japan; Mackenna, Clinical Pharmacy, Santiago, Chile; 9 Hospital Exequiel Gonza-
8 Kyoto University, Human Health Science, Kyoto, Japan; 9 Center for iPS Cell lez Cortes, Pediatric Oncology, Santiago, Chile; 10 Hospital Roberto del Rio,
Research and Application, Cell Growth And Differentiation, Kyoto, Japan Pediatric Oncology, Santiago, Chile; 11 Hospital Guillermo Grant Benavente,
Pediatric Oncology, Concepcion, Chile; 12 Hospital Regional de Valdivia,
Background and Aims: Patients with osteosarcoma who experience Pediatric Oncology, Valdivia, Chile; 13 Hospital Luis Calvo Mackenna, Pedi-
relapse or progression (R/P) have a poor prognosis. In the current atric Oncology, Santiago, Chile, Pediatric Oncology, Santiago, Chile
study, we retrospectively analyzed the clinical outcomes of patients
with osteosarcoma who recently experienced R/P and received novel Background and Aims: The aim of this study was to analyze clini-
salvage chemotherapy and/or molecular target therapy. cal outcomes of children with osteosarcoma treated by 2 consecutive
Methods: Between 2000 and 2019, 59 patients younger than 40 years protocols.
old were diagnosed with high-grade osteosarcoma, among which 30 Methods: Patients with high-grade osteosarcoma aged ≤15 years
patients experienced R/P. Clinical data of those patients were ret- were treated with pre and post-operatively chemotherapy. Protocol
rospectively analyzed to identify prognostic and therapeutic factors A, June 2006-December 2015 : Carboplatin, Ifosfamide, Doxorubicin
influencing their outcomes. and Methotrexate for localized patients; Cisplatin, Doxorubicin, Ifos-
Results: Twenty-two patients (73.3%) underwent curative local famide, Etoposide and Methotrexate for metastatic ones Protocol B,
surgery for primary lesion and metastases, one of whom also received January 2016 to March 2021: Cisplatin, Doxorubicin, Methotrexate
local radiotherapy, after first R/P. The 5-year progression-free sur- for all patients with metronomic chemotherapy with oral cyclophos-
vival (PFS) rate of the entire cohort was 51.9%. The 5-year overall phamide and methotrexate in metastatic patients. All patients under-
survival (OS) rates after the last R/P of patients experiencing first went surgery of the primary tumor.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S248 of S635 ABSTRACTS

Results: Protocol A, 131 patients, median follow-up 97 months. Fifty Results: Median percentage of CD99+ , CD45− cells was significantly
% were male, median age 12 years; 38 % presented with lung increased in patients compared with controls (0.002% vs 0%, respec-
metastases, 62% bilateral. One patient with multicentric disease, 4 tively, P < 0.001). Post-cycle 5 CD99+ , CD45− cells were increased in
patients had second cancer. Seventy-eight % underwent conservative 12 patients, of them 11 patients’ disease had either relapsed or pro-
surgery; necrosis 90% in 53%. Overall survival (OS) 64%, localized gressed. Post-cycle 5 CD99+ ; CD45− cells had a 73.3% sensitivity and
75% and metastatic 46%. Event-free survival (EFS) 60%, localized: 97.8% specificity for predicting relapse or progression, whereas base-
69%, metastatic: 46%. Twenty-two patients (27%) localized patients line only had 6.7% sensitivity and 77.8% specificity. The hazard ratio for
relapsed: bone 27%, lung 50%, combined 23%. Forty-seven patients mortality in the post-cycle 5 positive group was 18.4 [95% confidence
died (36%). Protocol B, 64 patients median follow-up 33 months. Fifty- interval (1.86 to 181.46)] times that of the negative group. One year OS
five % were male, median age 12 years.; 56% presented with lung was 91.67%.
metastases, 89% bilateral. Three patients with multicentric disease, Conclusions: Post-cycle 5 CD99+ , CD45− cells in peripheral blood by
2 patients had second cancer Sixty-seven % underwent conservative FC is a strong predictor for relapse, progression, and mortality whereas
surgery; necrosis 90% in 42%. OS: 72%; localized 90% and metastatic baseline is a poor predictor in newly diagnosed patients with ES
58%. EFS 53%; localized: 71%, metastatic: 39%. Seven (25%) localized
patients relapsed: 43% local, 57%, lung. Eighteen patients died (28%).
Conclusions: Our national data demonstrates similar survival rates E-Poster Topic: AS05.h Soft Tissue Sarcomas
as reported by larger series and reaffirms known prognostic fac-
tors.This highlights the importance of centers concentrating expertise E-POSTER VIEWING
and resources. Of note, the high proportion of metastasic patients.
Acknowledgements to Data Manager Mariela Fuenzalida EP217 / #159 RHABDOMYOSARCOMA WITH UNKNOWN
PRIMARY TUMOR SITE. A REPORT FROM EUROPEAN
PEDIATRIC SOFT TISSUE SARCOMA STUDY GROUP (EPSSG)
EP216 / #197 PROGNOSTIC VALUE OF DETECTION OF
CD99+, CD45− CELLS IN PERIPHERAL BLOOD BY FLOW Maria Carmen Affinita1 , Hans Merks2 , Julia Chisholm3 , Stéphanie
CYTOMETRY IN CHILDREN WITH EWING SARCOMA Haouy4 , Angélique Rome5 , Marco Rabusin6 , Bernadette Brennan7 ,
Gianni Bisogno1
Osama Zakzok1 , Wael Zekri2 , Mohamed Elshanshory3 , Nahla 1 Pediatric Hematology, Oncology and Stem Cell Transplant Division.,
Elsharkawy4 , Iman Zaky5 , Asmaa Salama6 , Ahmed Kamel7 , Ismail Department Of Women’s And Children’s Health University Hospital Of
Elantably8 , Shebl Said9 Padova, Padua, Italy; 2 Prinses Máxima Centrum voor Kinderoncologie,
1 57357 children cancer hospital, Pediatric Oncology, Tanta, Egypt; 2 57357 Pediatric Oncology, Utrecht, Netherlands; 3 Children and Young People’s
children cancer hospital, Pediatric Oncology, cairo, Egypt; 3 Tanta uni- Unit„ The Royal Marsden Nhs Foundation Trust And Institute Of Cancer
versity, Pediatric Haematologyand Oncology, Tanta, Egypt; 4 57357 chil- Research, Sutton, United Kingdom; 4 University Hospital Centre, Depart-
dren cancer hospital, Clinical Pathology, cairo, Egypt; 5 57357 chil- ment Of Pediatric Oncology, Montpellier, France; 5 APHM, Department Of
dren cancer hospital, Radiology, cairo, Egypt; 6 children’s cancer hospi- Pediatric Immunology, Hematology And Oncology, Children’s Hospital Of
tal,57357&National cancer institute, Pathology, Cairo, Egypt; 7 children’s La Timone Marseille, Provence-Alpes-Côte d’Azu, France; 6 IRCCS Materno
cancer hospital,57357&National cancer institute, Pediatric Oncology, Infantile Burlo Garofolo, Department Of Pediatric, Hemato-oncology Unit,
Cairo, Egypt; 8 57357 children cancer hospital, Nuclear Medicine, cairo, Bari, Italy; 7 Royal Manchester Children’s Hospital, Manchester, Department
Egypt; 9 Tanta university, Pediatric Hematology And Oncology, Tanta, Egypt Of Pediatric Oncology, Manchester, United Kingdom

Background and Aims: Early detection of metastasis and recurrence Background and Aims: Rhabdomyosarcoma (RMS) is an aggressive
of Ewing sarcoma (ES) is important for early management. This work malignancy, and 20% of children present with metastases at diagno-
aimed to detect CD99+ , CD45− cells in peripheral blood by flow sis. Patients presenting with disseminated disease very occasionally
cytometry (FC) before and during chemotherapy and evaluate their have no clear evidence of a primary tumor mass. Since these patients
prognostic significance. have rarely been investigated, we report on a series of patients with
Methods: This prospective cohort study was carried out on 60 children RMS and unknown primary tumor site registered in the MTS 2008 pro-
newly diagnosed with ES at Children Cancer Hospital-Egypt 57357 and tocol (October 2008 - December 2016) coordinated by the European
40 healthy children control group. Detection of CD99+ , CD45− cells pediatric Soft tissue sarcoma Study Group.
in peripheral blood was accomplished by FC at baseline before treat- Methods: Patients were administered 9 cycles of induction chemother-
ment and after five cycles of chemotherapy. Samples were classified as apy, and 48 weeks of maintenance chemotherapy. Surgery and/or
positive if they had more than the upper limit of cells observed in the radiotherapy was planned after the first assessment of tumor response,
control cases. Correlation between FC results and relapse and overall and implemented after six cycles of chemotherapy. If feasible, radio-
survival (OS) after one year was performed. therapy to all sites of metastasis was recommended.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S249 of S635

Results: We identified 10 patients with RMS and unknown primary EP219 / #1021 PAZOPANIB MAINTENANCE THERAPY FOR
site, most of them adolescents (median age 15.8 years, range 4.6-20.4). EWING SARCOMA IN PEDIATRIC PATIENTS
Nine had fusion-positive alveolar RMS. Multiple organ involvement
was identified in 7 patients, 2 only had bone marrow disease, and 1 only Nihan Bayram1 , Murat Elli1 , Yontem Yaman1 , Isık Odaman Al1 , Kursat
had leptomeningeal dissemination. All patients were given chemother- Ozdilli1 , Dilek Unal2 , Harzem Ozger3 , Sema Anak1
apy, 4 were irradiated, and none had surgery. Three patients underwent 1 Istanbul Medipol University, Pediatric Hematology And Oncology, Istan-
allogeneic bone marrow transplantation. At the time of this analy- bul, Turkey; 2 Istanbul Medipol University, Radiotherapy, Istanbul, Turkey;
sis, only 2 patients are alive in complete remission: 1 had received 3 Acıbadem Hospital, Pediatric Surgery, Istanbul, Turkey

radiotherapy; and 1 had a bone marrow transplant.


Conclusions: RMS with unknown primary tumor occurs mainly in ado- Background and Aims: The prognosis of Ewing sarcoma is still
lescents and is typically fusion-positive alveolar. Radiotherapy may not promising due to remarkable relapse rates. Thus, there
be important, but survival is poor and patients should be offered is an need for novel therapies. Pazopanib is a multi-target
enrollment in investigational trials. tyrosine kinase inhibitor and there have been no reports on
the long-term efficacy of it in pediatric patients with Ewing
sarcoma.
EP218 / #1364 NUTRITIONAL STATUS AND INTERVENTIONS Methods: We report 7 pediatric patients with Ewing sarcoma, received
OF CHILDREN WITH BONE AND SOFT TISSUE SARCOMAS pazopanib in addition to cytotoxic chemotherapy
Results: The median age was 12 (8-15 years). 4 patients had metastatic
Eda Ataseven, Derya Hopanci Bicakli, Mehmet Kantar disease and 3 patients were in the poor-risk group according to Euro
Ege University Faculty of Medicine, Pediatric Oncology, Izmir, Turkey Ewing 2012 Protocol. 6 patients underwent surgery and there was no
viable tumour in the pathological examination. One patient could not
Background and Aims: Malnutrition is an important condition com- be operated due to high morbidity. All patients received radiotherapy.
mon in pediatric oncology and causes many adverse events, including 3 patients underwent high-dose chemotherapy and autologous stem
mortality. This study aims to determine the nutritional status, daily cell transplantation. We started pazopanib to 6 patients as mainte-
food consumption, and nutritional treatments in hospitalized children nance after the end of the treatment at a dosage of 200-400mg/day,
with solid tumors. which was eventually increased to 400-800mg/day. The inoperable
Methods: Patients with bone and soft tissue sarcomas admitted to the patient had intracranial relapse at the 6th month of pazopanib. Other
Ege University Hospital Pediatric Oncology Department between Jan- 5 patients are disease-free with a 7 months median follow-up time
uary 2018-December 2021 (59 patients; F/M: 26/33; mean age:10.3 from the starting of pazopanib (3-13 months). One of the 7 patients
years (0,5-18 years)) were followed. The nutritional status of the had disseminated disease and he received VIT (vincristine- irinotecan-
patients was evaluated with the StrongKids (SK) screening tool within temozolamide) with pazopanib. He has also been followed-up with
24 hours of hospitalization, and nutritional therapy was started for pazopanib as monotherapy for 10 months and he is disease-free. In all
the patients who needed it. Anthropometric measurements (height, cases, whitening of hair was observed. Two patients had mild hepato-
weight, upper-middle arm circumference (MUAC)), and daily food toxicity. One of these patients tolerated the dose reduction well and
consumption were taken in 3 different times. is now monitored. The other patient had to be discontinued at the 8th
Results: Malnutrition was observed in 22% (n =13), moderate risk month of treatment. Median follow-up time from the diagnose is 18
78.0%(n=46) according to StrongKids. According to weight for age months (15-27 months).
32.2%(n=19) of patients were <25%, 33.9% (n=20) of the children had Conclusions: Pazopanib maintenance may be a therapeutic option
<25% MUAC. Children can get mean 47.9% (5-115%) of their daily as a continuation treatment for poor-risk Ewing sarcoma and also
energy requirements and 43% (0.5-120) of their protein requirements may be useful for disseminated disease. More studies are needed to
with oral foods. 33,9% (n=20) of the children were fed with ONS, 13,6% demonstrate its efficacy and safety in pediatric patients.
(n=8) fed by EN, and 1,7% (n=1) fed by TPN. Of the 40 patients, all
3 measurements were completed, 45,8% (n=27) gained weight. The
patients weight for age <25% at the 1st, 2nd and 3rd follow-ups, EP220 / #1392 IMPACT AND THERAPEUTIC EXPLOITATION
respectively were 37.5% (n=15), 45% (n=18), 42.5% (n=17) (p=0.13). OF HYPOXIA IN RHABDOMYOSARCOMAS
Those with SK>4 were respectively 27.5% (n=11), 47.5% (n=19),
27.5% (n=11) (p=0.029), those with MUAC<25 were found to be Carolina Bernauer1 , Lucile Delespaul1 , Louise Howell2 , Ewa
37.5% (n=15), 45% (n=18), 35% (n=14) (p=0.22). Aladowicz1 , Stella Man1 , Carol Box3 , Simon Robinson3 , Janet Shipley1
Conclusions: Malnutrition in pediatric cancers is an important issue 1 The Institute of Cancer Research, Sarcoma Molecular Pathology, London,
that should not be ignored. It can occur at any time during the United Kingdom; 2 The Institute of Cancer Research, Core Research Facili-
follow-up. Multidisciplinary teams regarding malnutrition should mon- ties - Light Microscopy Sutton, London, United Kingdom; 3 The Institute of
itor pediatric cancer patients, and nutritional interventions should be Cancer Research, Radiotherapy And Imaging, London, United Kingdom
performed without delay when necessary.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S250 of S635 ABSTRACTS

Background and Aims: Rhabdomyosarcomas (RMS) are rare aggres- Children’s Hospital, University of Washington, Seattle, Division Of Hema-
sive childhood cancers with poor prognosis at relapse. Hypoxia in tology/oncology, Seattle, United States of America; 11 University Hospital
cancer is associated with resistance to radio- and chemotherapy and is Giessen-Marburg, Campus Marburg, Department Of Pediatric Surgery And
a negative prognostic factor. Data for the extent and effects of hypoxia Urology, Marburg, Germany; 12 Prinses Máxima Centrum voor Kinderon-
in RMS are limited. Moreover, no hypoxia-targeted treatment strategy cologie, Pediatric Oncology, Utrecht, Netherlands; 13 Klinikum Stuttgart
has entered clinical practice, due to a lack of biomarker-led clinical tri- Olgahospital, Pediatrics 5 (hematology, Oncology, Immunology), Stuttgart,
als. The project aims to assess the effect of hypoxia in RMS on patient Germany; 14 Baylor College of Medicine, Texas Children’s Cancer Center,
outcomes, develop a novel, more effective therapeutic strategy based Department Of Pediatrics, Houston, United States of America
on the hypoxia present in RMS.
Methods: Bioinformatic analysis was performed on RMS patient gene- Background and Aims: The survival of patients with localized embry-
expression profiling datasets. Protein and RNA levels of hypoxia onal rhabdomyosarcoma (RMS) completely resected at diagnosis is
markers and sensitivity to backbone drugs were compared in hypoxia above 90%. Most of them have paratesticular, uterus, or vaginal
and normoxia in vitro and three-dimensional models were generated RMS, limiting specific analyses on RMS localized in other anatomic
to model hypoxia. regions. We conducted this international study to define the out-
Results: High levels of hypoxia significantly correlated with worse come for completely resected embryonal RMS at sites other than
overall survival (OS) in fusion negative (FN)-RMS (p=0.0436). A con- paratesticular/uterine/vaginal primary sites,
sistent upregulation of hypoxia inducible factor (HIF) downstream- Methods: We identified 113 patients, aged 0-18 years, enrolled from
targets glucose transporter-1 and carbonic anhydrase-IX, as well as 1/1995 to 12/2016 in Children’s Oncology Group (COG) (64 patients)
reduced sensitivity to irinotecan in hypoxia, was demonstrated in cell and European protocols (49). Genito-urinary non-bladder/prostate
line models. Hypoxia in 3D tumour spheroid cultures of RMS cell lines RMS were excluded. The recommended chemotherapy was VA (vin-
was alleviated by atovaquone, a repurposed antimalarial. Preliminary cristine, actinomycin-D) for 24 weeks or IVA (ifosfamide plus VA)
data suggests that atovaquone sensitises RMS spheroids to irinotecan. in the European protocols and VA for 48 weeks or VAC (VA plus
Conclusions: Tumour hypoxia negatively impacts outcome in patients cyclophosphamide) in the COG protocols.
with FN-RMS and evidence suggests this contributes to resistance Results: The most common primary sites were non-parameningeal
to current treatment options. Atovaquone provides a novel hypoxia- head and neck (40.7%), other (23.9%) and extremities (20.4%). In the
targeted strategy to improve oxygenation in tumours and increase COG studies, 42% of patients received VA and 58% VAC. In Europe
sensitivity to current treatment options. 53% received VA and 47% IVA. With a median follow-up of 97.5
months the 5-year progression free and overall survival was 80.0%
(71.2-86.4) and 92.5% (85.6-96.2), respectively, without significant dif-
EP221 / #132 PATIENTS WITH EMBRYONAL ferences between chemotherapy regimens. Tumor size (< or > 5 cm)
RHABDOMYOSARCOMA COMPLETELY RESECTED AT significantly influenced overall survival: 96.2% (88.6-98.8) vs. 80.6%
DIAGNOSIS: AN INTERNATIONAL ANALYSIS (59.5-91.4), respectively (p 0.01).
Conclusions: Survival of patients with non alveolar RMS completely
Gianni Bisogno1 , Joerg Fuchs2 , Roshni Dasgupta3 , Andrea Ferrari4 , resected at diagnosis is good among tumors arising from non-
Josephine Haduong5 , Timothy Rogers6 , David Walterhouse7 , Beatrice paratesticular/uterine/vaginal sites, and patients may be treated suc-
Coppadoro1 , Wei Xue8 , Christian Vokuhl9 , Douglas Hawkins10 , Guido cessfully with low intensity chemotherapy. To reduce the burden of
Seitz11 , Hans Merks12 , Monika Sparber-Sauer13 , Rajkumar treatment VA for 24 weeks may be considered in children with tumors
Venkatramani14 <5 cm.
1 University of Padua, Hematology/oncology Division, Department Of
Women’s And Children’s Health, Padua, Italy; 2 University Children’s Hos-
pital, Department Of Pediatric Surgery And Urology, Tuebingen, Germany; EP222 / #946 PROGNOSTIC VALUE OF PATIENT-DERIVED
3 Cincinnati Children’s Hospital Medical Center, University of Cincinnati„ XENOGRAFT ENGRAFTMENT IN PEDIATRIC SARCOMAS
Division Of Pediatric General And Thoracic Surgery, Cincinnati, United
States of America; 4 Fondazione IRCCS Istituto nazionale dei Tumori, Milano, Angel Carcaboso, Helena Castillo Ecija
Pediatric Oncology, milano, Italy; 5 Division of Oncology, Children’s Hos- Hospital Sant Joan de Deu, Pediatric Oncology, Barcelona, Spain
pital Orange County, Hyundai Cancer Institute, Orange, United States
of America; 6 University Hospitals Bristol and Weston NHS foundation Background and Aims: The goals of this work were to identify fac-
trust, Department Of Pediatric Surgery, Bristol, United Kingdom; 7 Ann & tors favoring patient-derived xenograft (PDX) engraftment and study
Robert H. Lurie Children’s Hospital of Chicago, Northwestern University the association between PDX engraftment and prognosis in pediatric
Feinberg School of Medicine, Deaprtment Of Pediatrics, Chicago, United patients with Ewing sarcoma, osteosarcoma and rhabdomyosarcoma.
States of America; 8 University of Florida, Department Of Biostatistics, Methods: We used immunodeficient mice to establish PDX from
Gainesville, United States of America; 9 University Hospital Bonn, Section Of patients at hospital Sant Joan de Deu, Barcelona, Spain. We addressed
Pediatric Pathology, Department Of Pathology, Bonn, Germany; 10 Seattle whether PDX tumors retained the main histologic, genomic and
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ABSTRACTS S251 of S635

functional properties of patient tumors during successive passages Lyon, Pediatric Surgery, Bron, France; 15 GHU Henri Mondor, AP-HP, UPEC,
in mice. Sample characterization included histopathology markers, Department Of Dermatology. Centre De Reference Des Neurofibromatoses„
chromosomal profiles (copy number alteration; CNA) and preclinical Créteil, France; 16 Institut Curie, Siredo Oncology Department, Paris, France
treatment assays (efficacy of irinotecan). We studied the association
of the parameter “successful PDX engraftment” with the event free Background and Aims: MPNST are very rare and aggressive tumors,
survival and overall survival of the patients from which the PDX were which affect children, adolescents, and young adults. These tumors
established. appear frequently in the context of type 1 neurofibromatosis (NF1).
Results: We established 30 subcutaneous PDX from patient tumor This study aims to determine risk factors in unselected pediatric
biopsies, with a successful engraftment rate of 44%. Age greater than MPNST in order to define an adapted therapeutic strategy.
12 years and relapsed disease were patient factors associated with Methods: Multicentric national retrospective study of all pediatric
higher engraftment rate. PDX retained histology markers and most patients (0-18 yo) treated for MPNST, confirmed by a systematic
chromosomal aberrations of patient samples during successive pas- pathology review, in France, from 1995 to 2017 and included in the
sages in mice. Treatment with irinotecan resulted in significant activity SIOP MMT-95, EpSSG NRSTS-05 protocols and the French National
in 20 of the PDX and replicated the response of rhabdomyosarcoma Pediatric Tumor Registry (RNCE).
patients. Successive generations of PDX responded similarly to irinote- Results: Overall, 66 patients (median age 13.0 yo [range, 0.1-18.0])
can, demonstrating functional stability of these models. Out of 68 developed a MPNST located in the limbs (36%), trunk (27%), head
tumor samples from 51 patients with a median follow up of 21.2 and neck (21%) and para-vertebral area (15%). Among them, 48% of
months, PDX engraftment from newly diagnosed patients was a prog- patients had NF1, 44% had grade I-II FNCLCC grade tumors while 50%
nostic factor significantly associated with poor outcome (P = 0.040). had grade III (not gradable, 4 cases). A majority of patients had local-
This association was not significant for relapsed patients. In the sub- ized (94%) and N0 (96%) tumors. The therapies consisted of surgery
group of patients with newly diagnosed Ewing sarcoma classified as (94%), chemotherapy (neoadjuvant (36%) and/or adjuvant (27%)) and
standard risk, we found higher risk of relapse or refractory disease radiotherapy (52%). After a median follow-up of 3.8 y [range, 0.0-18.7],
associated to those samples that produced stable PDX models (P = 5-year overall (OS) and event-free survivals (EFS) were respectively
0.0357). 46.7% [95%CI, 35.8-60.8] and 40.8% [95%CI, 30.3-54.9]. In multi-
Conclusions: Our study shows that PDX engraftment predicts worse variate analysis, unfavorable risk factors for overall survival were:
outcome in newly diagnosed pediatric sarcoma patients presence of metastases (OR 6.4 [1.8; 22.0], p=0.001), FNCLCC grade
3 (OR 4.77 [1.4; 15.8], p=0.01), large tumor size (≥10 cm; OR 2.3 [1.1;
4.8], p=0.02) and NF1 status (OR 2.2 [1.1; 4.4], p=0.02).
EP223 / #353 RISK FACTORS IN PEDIATRIC MALIGNANT Conclusions: MPNST had overall poor outcome especially in NF1
PERIPHERAL NERVE SHEATH TUMORS (MPNST): RESULTS patients and in high grade, large and metastatic tumors. These risk fac-
FROM THE FRENCH PEDIATRIC ONCOLOGY SOCIETY (SFCE) tors should be considered to develop a more adapted risk stratification
COHORT and develop new strategies to improve the survival of the patients. Reg-
ular monitoring for early MPNST detection in patients with NF1 also
Jordane Chaix1 , Marie Karanian2 , Nadege Corradini3 , Maria needs to be developed.
Merched1 , Frédérique Larrousserie4 , Louise Galmiche5 , Brigitte
Lacour6 , Aude Marie-Cardine7 , Anne-Sophie Defachelles8 , Veronique
Minard-Colin9 , Angélique Rome10 , Estelle Thebaud11 , Valérie EP224 / #283 METASTATIC RHABDOMYOSARCOMA:
Bernier12 , Hervé Brisse13 , Frederic Hameury14 , Pierre Wolkenstein15 , EVIDENCE OF THE IMPACT OF RADIOTHERAPY ON
Stéphane Ducassou1 , Daniel Orbach16 , Cécilé Vérité1 SURVIVALS, A RETROSPECTIVE SINGLE-CENTER EXPERIENCE
1 CHU de Bordeaux, Oncologie Et Hematologie Pédiatrique, Bordeaux,
France; 2 Centre Léon Bérard, Anatomie Pathologique, Lyon, France; 3 IHOPe, Luca Bergamaschi1 , Stefano Chiaravalli1 , Virginia Livellara1 , Giovanna
Léon Bérard center, Department Of Pediatric Hematology And Oncology, Sironi1 , Olga Nigro1 , Nadia Puma1 , Giovanna Gattuso1 , Carlo Morosi2 ,
Lyon, France; 4 Hôpital Cochin - APHP, Anatomie Pathologique, Paris, France; Patrizia Gasparini3 , Emilia Pecori4 , Ombretta Alessandro4 , Sabina
5 CHU de Nantes, Department Of Pathology, Nantes, France; 6 CHU de Vennarini4 , Lorenza Gandola4 , Maura Massimino1 , Michela
Nancy, Registre National Des Cancers De L’enfant, Nancy, France; 7 CHU Casanova1 , Andrea Ferrari1
de Rouen, Oncologie Pédiatrique, Rouen, France; 8 Centre Oscar Lambret, 1 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Oncology Unit,

Oncologie Pédiatrique, Lille, France; 9 Gustave Roussy, Département De Can- Milan, Italy; 2 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric
cérologie De L’enfant Et L’adolescent, Villejuif, France; 10 APHM, Department Radiology Unit, Milan, Italy; 3 Fondazione IRCCS Istituto Nazionale dei
Of Pediatric Immunology, Hematology And Oncology, Children’s Hospital Of Tumori, Tumor Genomics Unit, Milan, Italy; 4 Fondazione IRCCS Istituto
La Timone Marseille, Provence-Alpes-Côte d’Azu, France; 11 CHU de Nantes, Nazionale dei Tumori, Pediatric Radiotherapy Unit, Milan, Italy
Oncologie Et Hématologie Pédiatrique, Nantes, France; 12 Institut De Can-
cérologie Lorrain, Département De Radiothérapie, Nancy, France; 13 Institut Background and Aims: The prognosis of patients with metastatic
Curie, Département D’imagerie Médicale, Paris, France; 14 Hospices Civils de rhabdomyosarcoma (RMS) remains largely unsatisfactory despite the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S252 of S635 ABSTRACTS

adoption of intensive multimodal therapy. To evaluate the role of the Results: The first relapse occurred within 2-102 months (median 14
different treatments adopted over the years, we retrospectively anal- months) after patients’ first diagnosis, and was local in 47%, metastatic
ysed a cohort of patients <21 years with metastatic RMS, treated from in 34%, and both in 19%. Treatment at relapse included chemotherapy
1990 to 2020 at a referral center for pediatric sarcomas. in 72 patients, radiotherapy in 38, and surgery in 55. The median over-
Methods: Patients were treated using a multimodal approach, which all survival (OS) was 20 months. Post-relapse OS was 56.1%, 25.8% and
included surgery, radiotherapy and chemotherapy (with high-dose 19.1% at 1, 5 and 10 years, respectively. Cox’s multivariable regres-
and maintenance chemotherapy in some cases). Patients’ outcome sion analysis showed that OS was significantly better for patients with
was examined with univariable and multivariable analysis based on local and late relapses (occurring more than 12 months after their first
clinical features and treatment. Concerning radiotherapy, we catego- diagnosis), and for those achieving secondary remission.
rized patients as: radical = radiotherapy to all disease sites; partial = Conclusions: The outcome of patients with recurrent NRSTS is poor.
radiotherapy to ≥ 1 site of disease, but not all; or none. The abovementioned variables (type and time of relapse, and achieve-
Results: The series included 80 patients. Event-free survival (EFS) and ment of secondary remission) were combined in a risk-adapted model
overall survival (OS) at 5 years were 17.3% and 21.3%, respectively. to develop a tool for estimating the chance of salvage, and deciding the
Survivals were significantly associated to radiotherapy, either on pri- best second-line treatment approach.
mary tumor and metastatic sites. In particular, EFS and OS were 70.6%
and 76.0%, respectively, for patients who had radical irradiation. Lung
irradiation was associated to superior survivals in patients with lung EP226 / #1043 THE PROGNOSTIC ROLE OF THE
metastases only (5-year OS 77.8% versus 23.1%). EFS correlated also C-REACTIVE PROTEIN AND SERUM LACTATE
with maintenance chemotherapy, with also OS tending to be better for DEHYDROGENASE IN A SERIES OF PEDIATRIC PATIENTS
patients receiving it. At the Cox’s multivariable analysis, OS correlated AFFECTED BY EWING SARCOMA
significantly with radiotherapy category.
Conclusions: While confirming the overall poor outcome of metastatic Giada Del Baldo1 , Rachid Abbas2 , Maria Antonietta De Ioris1 ,
RMS patients, this study identified radiotherapy – on both primary Valentina Di Ruscio1 , Iside Alessi1 , Evelina Miele1 , Ida Russo1 , Angela
tumor and metastatic sites – as the main variable influencing survival. Mastronuzzi1 , Giuseppe Maria Milano1
As further finding, our study suggested a potential role of maintenance 1 IRCCS Bambino Gesù Children’s Hospital, Department Of Paediatric
chemotherapy in improving survivals. Haematology/oncology, Rome, Italy; 2 CESP, INSERM, Université Paris-sud,
Paris, France

EP225 / #423 PEDIATRIC ADULT-TYPE Background and Aims: Ewing sarcoma (ES) is a rare and aggressive
NON-RHABDOMYOSARCOMA SOFT TISSUE SARCOMAS: pediatric cancer with different biological behaviours. The predictive
SALVAGE RATES AND PROGNOSTIC FACTORS AFTER RELAPSE value of serum lactate dehydrogenase (LDH) and C-reactive protein
(CRP) has not been clearly assessed. The objective of our retrospective
Stefano Chiaravalli1 , Luca Bergamaschi1 , Virginia Livellara1 , Giovanna study was to investigate the prognostic value of LDH and CRP levels in
Sironi1 , Nadia Puma1 , Olga Nigro1 , Giovanna Gattuso1 , Roberto a series of ES pediatric patients.
Luksch1 , Monica Terenziani1 , Filippo Spreafico1 , Cristina Meazza1 , Methods: Between 2004 and 2019, 89 patients with ES were included.
Marta Podda1 , Veronica Biassoni1 , Elisabetta Schiavello1 , Carlo LDH and CRP were measured at diagnosis before treatment initia-
Morosi2 , Maura Massimino1 , Michela Casanova1 , Andrea Ferrari1 tion for all patients. Multivariable Cox models for overall survival (OS)
1 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Oncology Unit, adjusting on commonly reported prognostic factors of Ewing sarcomas
Milan, Italy; 2 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric were used. For biomarkers, a classification and regression tree (CART)
Radiology Unit, Milan, Italy model was used to find a cut-off associated with survival. Kaplan-Meier
estimates by biomarkers levels were derived for OS and progression-
Background and Aims: Though the prognosis for patients with pedi- free survival (PFS) and compared with log-rank tests. All comparison
atric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) is gener- tests were two-sided and considered significant at the 5% level.
ally good, the chances of being cured after relapse are limited. This Results: In univariable analyses, LDH and CRP high levels were asso-
report describes a series of relapsing NRSTS patients treated at a refer- ciated with worst prognosis. In multivariable analyses, only higher
ral center for pediatric sarcoma, investigating the pattern of relapse, LDH value remained associated with a lower OS. For LDH, the opti-
salvage rates, and factors correlating with final outcome. mal cut-off to discriminate survival was 333 UI/L according to the
Methods: The analysis concerned 103 patients <21 years old with CART model. The high LDH level group experienced all the 21 deaths
relapsing adult-type NRSTS treated from 1985 to 2020. For risk- registered in our population (24%) and about 90% of disease progres-
adapted stratification purposes, patient outcome was examined using sions. The 5-year OS was 66.4% in the high LDH level group while no
univariable and multivariable analyses based on patients’ clinical fea- death was observed in the low LDH level group. The 5-year PFS was
tures at first diagnosis, first-line treatments, clinical findings at first 57.9% in the high LDH level group versus 80.4% in the low LDH level
relapse, and second-line treatments. group.
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ABSTRACTS S253 of S635

Conclusions: Our study demonstrates that LDH levels at diagno- Conclusions: HL occurred in up to 19% of survivors of HNRMS depend-
sis strongly correlates to prognosis. Its feasibility with a very low ing on ototoxicity grading scale. Four survivors revealed an atypical, but
cost and its reproducibility in almost all centers, make it suit- recurrent low frequency HL audiometric signature. More research is
able as a potential prognostic biomarker in clinical oncological needed on the relevance of this specific HL pattern and on the develop-
practice. ment of radiotherapy-specific HL classification systems. RT dose-effect
relation showed a trend but more research is needed. Long-term
audiological follow-up is recommended for survivors of HNRMS.
EP227 / #692 HEARING LOSS IN SURVIVORS OF HEAD AND
NECK RHABDOMYOSARCOMA
EP228 / #720 MALIGNANT AND BORDERLINE VASCULAR
Robin Diepstraten1 , Jan Wiersma2 , Reineke Schoot1 , Rutger Knops1 , TUMORS IN CHILDHOOD: A REPORT OF FORTY CASES FROM
Lotje Zuur3 , Annelot Meijer1 , Raquel Dávila Fajardo1,4 , Bradley SINGLE CENTER
Pieters2 , Brian Balgobind2 , Henrike Westerveld2 , Nicole Freling2 , Ludi
Smeele1,3,5 , Arjan Bel2 , Marry Van Den Heuvel-Eibrink1 , Robert Nilgun Kurucu1 , Tezer Kutluk1 , Diclehan Orhan2 , Burca Aydin1 ,
Stokroos6,7 , Alexander Hoetink6,7 , Hans Merks1 , Marinka Hol1,5,6 Bilgehan Yalçın1 , Ali Varan1
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, 1 Hacettepe University Faculty of Medicine, Department Of Pediatric Oncol-

Utrecht, Netherlands; 2 Amsterdam University Medical Centers, Cancer ogy, Ankara, Turkey; 2 Hacettepe University Faculty of Medicine, Depart-
Center Amsterdam, University of Amsterdam, Department Of Radiation ment Of Pathology, Ankara, Turkey
Oncology, Amsterdam, Netherlands; 3 Netherlands Cancer Institute, Depart-
ment Of Head And Neck Surgery, Amsterdam, Netherlands; 4 University Background and Aims: Malignant and intermediate grade vascular
Medical Center Utrecht, Department Of Radiation Oncology, Utrecht, tumors (MIVT) are a rare subtype of soft tissue sarcomas and consist
Netherlands; 5 Amsterdam University Medical Centers, Department Of Max- of histologically and clinically heterogeneous group of neoplasm. The
illofacial Surgery, Amsterdam, Netherlands; 6 University Medical Center objective of this study was to report our institutional experience with
Utrecht, Utrecht University, Department Of Otorhinolaryngology-head And VTM over a 45-year period.
Neck Surgery, Utrecht, Netherlands; 7 Utrecht University, Umc Utrecht Brain Methods: From 1975 to 2021, 336 children with non- rhabdomyosar-
Center, Utrecht, Netherlands coma soft tissue sarcoma (nRMS-STS) were admitted to the Pediatric
Oncology Department of Hacettepe University. Of these, 40 children
Background and Aims: Hearing loss (HL) can be a serious and per- with MIVT were evaluated retrospectively. Patients’ age and gender,
manent adverse effect of childhood cancer treatment and may be the tumor’s subtypes and location, treatment and survival rates were
provoked by systemic, but also local therapies such as head and recorded.
neck radiotherapy (RT) and surgery. The primary aim of this study Results: There were 17 males, 23 females with a mean age of
was to assess the frequency and pattern of HL in survivors of head 33.5±54.8 (0–192) months. Mean duration of symptoms from onset
and neck rhabdomyosarcoma (HNRMS). The secondary aim was to to diagnosis was 5.3±10.5 (0-56) months. The most common presen-
explore the dose-effect relationship between dose to the cochlea and tation symptoms were mass (42.5%) and skin rash (17.5%). The tumor
HL. arises from soft tissue in 45% of cases, skin in 25% of cases. Liver, lymph
Methods: Between 1993 and 2017 Dutch patients treated for nodes bone, lung and heart were the other localization. Histopatho-
HNRMS were evaluated for HL with pure tone audiometry at logically 25 were intermediate tumor including 9 kaposi sarcoma (9
least two years after end of treatment. HL was graded according post-transplant, 4 classical, one endemic), 8 hemangioendothelioma, 4
to the Muenster, International Society for Paediatric Oncology hemangiopericytoma and 4 kaposiform hemangioendothelioma, while
(SIOP) and Common Terminology Criteria for Adverse Events 15 were malignant including 12 angiosarcoma and 3 epitheloid heman-
(CTCAE) v4.03 classification. Deleterious HL was defined as gioendothelioma. Twenty patients underwent surgery, 4 of them
Muenster ≥2b, SIOP ≥2 and CTCAE ≥2. A dose-effect relation received chemotherapy and 5 received both chemotherapy and radio-
was explored for cochlear irradiation dose and HL using logistic therapy. In addition, 8 patients received chemotherapy alone or with
regression. steroid and/or interferon and four received both chemotherapy and
Results: Forty-two HNRMS survivors underwent audiological evalu- radiotherapy. One patient with intermediate group died of non-tumor
ation. According to Muenster, SIOP and CTCAE classification, 19.0% cause. Five-year overall and event free survival in intermediate and
(n=8), 14.2% (n=6) and 11.9% (n=5) suffered from deleterious HL, were 100% and 27.5% in intermediate group, 43.3% and 26.8% in
respectively. Four survivors had low frequencies HL with normal hear- malignant group.
ing or milder hearing loss in the higher frequencies. Maximum cochlear Conclusions: MIVT was rarely seen in childhood. It constitutes 11.9%
radiation dose (D0.1cc) correlated best with HL as opposed to mean of nRMS-STS in our clinics. Intermediate and malignant group consti-
or minimum dose. The dose probability curve shows a trend that HL tuted 62.5% and %37.5, respectively. Prognosis of malignant VT quite
(≥Muenster 2b) occurs in 50% of the ears when receiving a maximum bad. Although no patient died in intermediate VT recurrence rate was
dose of 19.0 GyEQD2 (p=0.075). high.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S254 of S635 ABSTRACTS

EP229 / #166 OUTCOMES OF CHILDREN TREATED WITH 1 Sun yat-sen University Cancer Center, Pediatric Oncology, Guangzhou,
PREOPERATIVE RADIOTHERAPY FOR LOCAL CONTROL IN China; 2 Sun Yat-Sen University Cancer Center, Pathology Department,
PEDIATRIC EWING SARCOMA AND RHABDOMYOSARCOMA Guangzhou, China; 3 Shenzhen Children’s Hospital, Pediatric Oncology,
Shenzhen, China; 4 West China Sencond University Hospital, Sichuan Uni-
Arielle Locke1 , Andrea Lo2 , Melissa Harvey3 , Paul Clarkson4,5 , Sylvia versity, Pediatric Oncology, Sichuan, China; 5 Children’s Hospital of Fudan
Cheng3 University, Pediatric Surgery, Shanghai, China; 6 Nanfang Hospital, Pedi-
1 National University of Ireland Galway, School Of Medicine, Galway, Ire- atric Oncology, Guangzhou, China; 7 Wuhan Children’s Hospital, Pediatric
land; 2 BC Cancer Agency, Radiation Oncology, Vancouver, Canada; 3 BC Oncology, Wuhan, China; 8 ZhuJiang Hospital of Southern Medical Univer-
Children’s, Pediatrics, Division Of Hematology/oncology/bmt, Vancouver, sity, Pediatric Oncology, Guangzhou, China; 9 GUANGZHOU WOMEN AND
Canada; 4 University of British Columbia, Orthopedics, Vancouver, Canada; CHILDRENS MEDICAL CENTER, Pediatric Oncology, Guangzhou, China;
5 BC Cancer Agency, Musculoskeletal Oncology, Vancouver, Canada 10 Beijing Tongren Hospital, Capital Medical University, Pediatrics, Bei-
jing, China; 11 Beijing Children’s Hospital, Surgical Oncology, Beijing, China;
Background and Aims: The optimal local management of pediatric 12 Children’s Hospital of Chongqing Medical University, Pediatric Oncology,
Ewing Sarcoma (ES) and rhabdomyosarcoma (RMS) requires careful Chong qing, China
multidisciplinary consideration, particularly when the tumour is large
or located in a difficult area to address surgically. When radiother- Background and Aims: To uncover the genomic characteristics and
apy (RT) is used in conjunction with surgery, the optimal timing of RT study the clinical value of Next Generation Sequencing (NGS) in pedi-
continues to be debated. Preoperative RT may reduce radiation dose atric soft tissue sarcoma (pSTS), we first report the genomic profiles in
and normal tissue exposure but is a less common approach. The study Chinese pSTS.
objective was to describe treatment outcomes and complications of Methods: Tumor tissue, as well as peripheral blood from 210 psts
children with ES or RMS treated with preoperative RT. patients, were collected in 11 centers in China. Samples were
Methods: This was a retrospective chart review of children aged 0-19 sequenced in a CLIA/CAP-accredited laboratory with a targeted NGS
years presenting to BC Children’s Hospital in Vancouver, Canada with panel (Onco PanScanTM plus; GenetronHealth.inc) consisting of all
a new diagnosis of ES or RMS from 1998- 2020. Descriptive statistics exons of 831 cancer-related genes and mRNA of 395 genes. Bioinfor-
and Kaplan-Meier method were used. matics analysis was conducted using a build-in computational pipeline.
Results: Of the 169 eligible children, 79 (46.7%) were diagnosed with Results: A total of 210 samples consisted of 28 histology types mainly
ES and 90 (53.3%) were diagnosed with RMS. Median follow-up dura- including rhabdomyosarcoma (RMS) (n = 66), Ewing’s sarcoma (n =
tion was 5.86 years [range, 1.60-13.5]. Median age was 11.0 years 25), INI1-deficient sarcoma (n = 22), sarcoma with BCOR genetic alter-
[range, 5-15] and 95 (56.2%) were male. The cohort’s 5-year overall sur- ations (n = 15), undifferentiated sarcoma (n = 13), synovial sarcoma
vival (OS) and progression free survival (PFS) were 65.7% and 60.7%, (n = 8), and other mesenchymal tumors (n = 74). All patients were
respectively. Only thirteen children received preoperative RT, all of with progressed, relapsed, refractory disease, or the diagnosis was dif-
whom all had ES, and primary tumor locations included pelvis (6), tho- ficult, of which 81.0% samples were from initial diagnoses. Genomic
rax (3), lower extremity (2), and abdomen (2). The 5-year OS and PFS for analysis revealed frequent alterations in TP53 (11%), EWSR1-FLI1
patients with ES who received preoperative RT compared to postoper- (10%), BCOR (8%), NTRK fusion (10%), PAX3 fusion (6%), FLI1-EWSR1
ative RT (n=14) were 82.5% vs. 85.8% (P=0.898) and 48.9% vs. 78.7% (5%), MDM2 (5%), CTNNB1 (4%), NF1 (4%), MYCN (4%) and PIK3CA
(P=0.143), respectively. Children treated with preoperative RT had a (4%). BCOR-internal tandem duplication (BCOR-ITD) was detected in
higher rate of surgical complications compared to children treated with 5 patients. In total, we identified 20 germline mutations, with a median
postoperative RT (30.7% vs 21.4%, P=0.674). tumor mutational burden of 0.47 (0-6.57) /Mb. The proportion of con-
Conclusions: Preoperative RT did not impact survival outcomes in our firmed diagnosis was 56.2%. NGS contributed to a change of diagnosis
institutional cohort of children with ES. There are potential advantages in 30 patients (14.3%). Druggable alterations were detected in 56.7% of
of reducing exposure of normal tissue to high doses of RT, and thus patients. Twenty-three patients received the treatment recommended
this approach could be considered in the treatment of children with by genetic testing, four of them with NTRK fusion were recruited in
ES. a matched clinical trial that could be evaluated and showed partial
remission upon Larotrectinib.
Conclusions: NGS profiling revealed the molecular basis of pediatric
EP230 / #1515 NEXT GENERATION SEQUENCING ANALYSIS soft tissue sarcoma in Chinese population, leading to be useful for
OF PEDIATRIC SOFT TISSUE SARCOMA REVEALS CLINICAL diagnosis and clinical management for some patients.
BENEFIT: MULTICENTER DATA FROM CHINA

Suying Lu1 , Yu Zhang2 , Xiuli Yuan3 , Ju Gao4 , Wei Yao5 , Haixia Guo6 , EP231 / #227 COMBINING ONCOLYTIC VIROTHERAPY AND
Hao Xiong7 , Hekui Lan8 , Xiaohong Zhang9 , Feifei Sun1 , Junting SMALL MOLECULE INHIBITORS: A NEW THERAPEUTIC
Huang1 , Juan Wang1 , Zijun Zhen1 , Jia Zhu1 , Xiaofei Sun1 , Weiling STRATEGY FOR SOFT TISSUE SARCOMAS
Zhang10 , Huanmin Wang11 , Shan Wang12 , Yizhuo Zhang1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S255 of S635

Julia Lutsch1 , Uwe Thiel1 , Stefan Burdach1 , Charlotte Middendorf1 , Background and Aims: Around one-third of children and young peo-
Roman Nawroth2 , Sebastian Schober1 , Per Sonne Holm2,3 ple treated for rhabdomyosarcoma (RMS) experience relapsed and/or
1 Klinikum rechts der Isar, School of Medicine, Technical University of refractory (R+R) disease. Second line treatment with curative intent
Munich, Department Of Pediatrics And Children’s Cancer Research Center, may be available but for around 80% this is not successful. Early phase
Munich, Germany; 2 Klinikum rechts der Isar, School of Medicine, Tech- trials explore new and innovative approaches, but are rarely reviewed
nical University of Munich, Department Of Urology, Munich, Germany; or synthesised systematically. We systematically reviewed responses
3 Medical University Innsbruck, Department Of Oral And Maxilllofacial in early phase trials of paediatric R+R RMS to inform future research
Surgery, Innsbruck, Austria and provide accurate information to families and clinicians making
treatment choices in this challenging situation.
Background and Aims: The success of standard therapy for high-risk Methods: Seven databases and six trial registries were searched in
patients is low and new therapeutic concepts are lacking. Oncolytic June 2021. All records were screened in duplicate. Eligible studies
viruses present new promising strategies, but the monotherapy rarely explored interventions aimed at disease control (curative or palliative)
has the potency to induce complete remission. It was shown before in patients under 18 years old with R+R RMS. Measured outcomes
that the addition of Cyclin-Dependent Kinases (CDK) 4/6 inhibitors included survival, disease response and adverse events. Studies were
can increase viral particle formation and cytotoxicity of the virus, while limited to those performed after 2000. No language or geographical
Bromodomain and Extra-Terminal motif (BET) inhibitors promote viral restrictions were applied. Quality assessment used the Downs and
gene expression and production of infectious particles. Therefore, we Black checklist. Given the considerable heterogeneity, synthesis was
assess respective combination therapies to boost the efficacy of the narrative. REFoRMS clinical and parent advisory groups were actively
YB-1-based oncolytic adenovirus XVir-N-31. involved in the review. (PROSPERO: CRD42021266254)
Methods: Here, we study in vitro oncolysis, viral replication, infectious Results: 16,965 records were identified. 197 trial registry records of
particle formation and antitumor immune responses of XVir-N-31 in 174 unique studies, and 115 full texts of completed studies (includ-
combination with inhibitors of CDK 4/6 and BET proteins in clinically ing over 1,100 R+R RMS patients) were included. Study quality was
achievable concentrations. limited by poor and inconsistent reporting. 49 registered trials are
Results: We observe high YB-1 expression in all cell lines which currently ongoing, 16 were withdrawn/suspended, and 11 completed
can be infected and lysed by the virus. Combination therapy greatly but have not reported data. Included studies mostly focus on systemic
enhances cytotoxicity and the interaction of the components is chemotherapy or targeted therapies with minimal data on local control
highly synergistic according to the Chou-Talalay-Test. We show that approaches. Overall response rates across included studies were low.
the combination therapy leads to increased viral replication 24h Adverse events vary dependent on intervention.
and 48h after viral infection. In addition, the combination therapy Conclusions: Improving reporting quality and consistency would facil-
significantly augments infectious particle formation 48h and 72h itate synthesis of early phase studies in R+R RMS. The REFoRMS-SR
after viral infection. Our results also suggest an ameliorated antitu- will be converted into the first living systematic review in children’s
mor immune response after the combination therapy due to MHC cancer, providing an up-to-date resource on early phase studies for
class I upregulation, induction of apoptosis and immunogenic cell researchers, clinicians and families.
death.
Conclusions: Combination therapies of XVir-N-31 and inhibitors of
CDK 4/6 and BET proteins are new promising therapeutic approaches EP233 / #717 A SYSTEMATIC REVIEW AND META-ANALYSIS
for pediatric soft tissue sarcoma in vitro, warranting further evaluation OF THE SIGNS AND SYMPTOMS OF CHILDHOOD SOFT TISSUE
in vivo (phase I clinical trial combining Oncolytic Virotherapy with CDK SARCOMAS
4/6 inhibitors in planning).
Lorna Ni Cheallaigh1 , Shaarna Shanmugavadivel2 , Jo-Fen Liu2 , Lorna
Fern3 , Paul Winyard1 , David Walker4
EP232 / #1600 SYSTEMATIC REVIEW OF EARLY PHASE 1 University College London, Great Ormond Street Institute Of Child
TRIALS FOR CHILDREN AND YOUNG PEOPLE WITH RELAPSED Health, London, United Kingdom; 2 University of Nottingham, Academic
AND REFRACTORY RHABDOMYOSARCOMA: THE REFORMS-SR Unit Of Population And Lifespan Sciences, Nottingham, United Kingdom;
PROJECT 3 University College London Hospitals Foundation Trust, Cancer Clinical Tri-

als Unit, London, United Kingdom; 4 University of Nottingham, Children’s


Lucy Beresford1 , Connor Evans1 , Gemma Bryan2 , Helen Fulbright1 , Brain Tumour Research Centre, Nottingham, United Kingdom
Bob Phillips1,3 , Jess Morgan1,3
1 University of York, Centre For Reviews And Dissemination, York, United Background and Aims: Time to diagnosis (TTD) of child-
Kingdom; 2 University of Surrey, School Of Health Sciences, Guildford, United hood soft tissue sarcomas (STS) has a significant independent
Kingdom; 3 Leeds Teaching Hospitals NHS Trust, Department Of Paediatric association with survival. A greater understanding of clinical
Haematology & Oncology, Leeds, United Kingdom presentation is crucial to informing interventions to effec-
tively reduce TTD. This study aims to provide an overview of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S256 of S635 ABSTRACTS

the signs and symptoms observed in patients with childhood Unit, Milano, Italy; 10 Institut Curie, Siredo Oncology Department, Paris,
STS. France
Methods: Studies detailing the signs and symptoms in >10 patients
aged <18 years at diagnosis, published between 2010 and 2021, were Background and Aims: A subset of rare undifferentiated small round
searched for, without language restrictions. The pooled proportions cell sarcomas harbor new identified specific molecular abnormali-
of 43 signs and symptoms were analysed and ranked by frequency ties: CIC-DUX4/other partner, BCOR-CCNB3/other partner, YWHAE
according to age and tumour location. fusions or BCOR-ITD (internal tandem duplication). “CIC rearranged”
Results: Among 2,462 patients, the most common signs and symptoms (CIC fused) and “BCOR rearranged” (BCOR fused/ITD/YWHAE) sarco-
were lump/swelling (38%), pain (6%), cutaneous changes (4%), localised mas are not well described in youths. Treatment and outcome data of
eye swelling (3%), constitutional symptoms (2%), abnormal full blood pediatric patients with these new rare sarcomas are missing.
count (2%), and cranial nerve deficits (2%). The signs and symptoms Methods: Multi-institutional retrospective analyze of young patients
differed according to location. For head and neck tumours, these (0-21 years) treated for a “CIC” or “BCOR rearranged” soft tissue sar-
were localised eye swelling (20%), lump/swelling (16%), cranial nerve coma (STS). Patients’ data from large European centers of Italy, France
deficits (14%), and/or impaired visual function (6%). For abdomen and the STS registry SoTiSaR were analyzed.
and pelvic tumours, these were urinary symptoms (24%), abdominal Results: Overall, 45 patients (25 males) had CIC-fused (n=27), BCOR-
distension/discomfort (22%), genital swelling (16%), pain (11%), consti- CCNB3 (n=8), BCOR-ITD (n=7), and YWHAE (n=3) STS. Main pri-
tutional symptoms (9%), vaginal bleeding (7%), change in bowel habit maries were limbs (n=15) and abdomino-pelvic (n=15). Median ages
(6%), and/or obstructive jaundice (2%). The most frequent signs and were 14.3 years (ranges: 2.8-20.0) and 8.6 (0.1-19.1) respectively
symptoms differed according to age, reflecting an age-related distri- for “CIC” (27 cases) and “BCOR-rearranged” STS (18 cases; p=0.03).
bution of STS. In patients <5 years, these were lump/swelling (44%), IRS stages were I (n=2), II (n=3), III (n=25) and IV (n=15). Over-
genital swelling (4%), reduced mobility (3%) and/or vaginal bleeding all, 35 patients had large tumors (≥ 5 cm) and 4 nodal involvement.
(2%), while consumptive coagulopathy (16%), cutaneous changes (5%), Patients received chemotherapy (n=45), local surgery (n=34) and/or
and bleeding/bruising/petechiae (2%) were strongly associated with radiotherapy (n=28), with additional high-dose chemotherapy (n=3) or
diagnosis <1 year. Among patients >11 years, the most frequent were maintenance (n=4). After a median follow-up of 41.9 months (IC95%,
lump/swelling (57%), constitutional symptoms (52.4%), pain (28.6%) 28.1-66.0), 23 patients had a tumor event, 17 patients died. Tumor
and/or headaches (19%). events occurred after a median delay of 6.5 months (range, 0.5-
Conclusions: The signs and symptoms of childhood STS differ accord- 114), mainly metastatic ± loco-regional (61%). Three year-event free
ing to tumour location and by age. Recognition of these age-specific survival (EFS) were 43.5% (95%IC, 27.4-69.2) and 42.6% (95%IC, 22.6-
clinical features, alongside a greater insight into their chronological 80.1) respectively for CIC and BCOR-STS (P=0.88). 3Y-overall survival
development, is instrumental in developing effective interventions to were respectively 45.2% (95%IC, 28.0-73.0) and 73.4% (95%IC, 54.1-
successfully reduce TTD. 99.7; P= 0.28). Age, tumor size, and IRS stage do not impact survival
(P>0.05), but outcome was superior in patients receiving anthracy-
clines (P=0.033).
EP234 / #233 VERY RARE SOFT TISSUE SARCOMAS WITH Conclusions: Pediatric patients with these rare entities often present
BCOR, CIC AND YWHAE REARRANGEMENTS IN CHILDREN, with large tumors and metastatic disease. Overall prognosis is unfa-
ADOLESCENTS AND YOUNG ADULTS: AN EUROPEAN vorable, even for small and localized tumors. Anthracyclines-based
EXPERIENCE regimen is important but new treatment options are necessary.

Monika Sparber-Sauer1 , Nadege Corradini2 , Giuseppe Maria Milano3 ,


Gaelle Pierron4 , Matthieu Carton5 , Franck Tirode6 , Daniel Pissaloux6 , EP235 / #276 EPITHELIOID HEMANGIOENDOTHELIOMA IN
Rita Alaggio7 , Christian Vokuhl8 , Andrea Ferrari9 , Daniel Orbach10 CHILDREN: THE EUROPEAN PEDIATRIC SOFT TISSUE
1 Klinikum Stuttgart Olgahospital, Pediatrics 5 (hematology, Oncology, SARCOMA STUDY GROUP (EPSSG) EXPERIENCE
Immunology), Stuttgart, Germany; 2 IHOPe, Léon Bérard center, Depart-
ment Of Pediatric Hematology And Oncology, Lyon, France; 3 Bambino Daniel Orbach1 , Max M. Van Noesel2 , Bernadette Brennan3 , Michela
Gesù Childrens’ Hospital, Department Of Pediatric Hematology And Oncol- Casanova4 , Stefan Schifflers5 , Nadege Corradini6 , Rita Alaggio7 ,
ogy And Of Cell And Gene Therapy, Scientific Institute For Research Gabriela Burrieza8 , Reineke Schoot2 , Pablo Berlanga9 , Ilaria Zanetti10 ,
And Healthcare (irccs)„ Roma, Italy; 4 Institut Curie, Department Of Lisa Hjalgrim11 , Gema Ramirez12 , Andrea Ferrari4
Somatic Genetics, Paris, France; 5 Institut Curie, Department Of Biom- 1 Institut Curie, Siredo Oncology Department, Paris, France; 2 Princess

etry, Drci, Psl Research University, Paris, France; 6 Centre Léon Bérard, Máxima Center, Pediatric Oncology, Utrecht, Netherlands; 3 Royal Manch-
Department Of Biopathology, Lyon, France; 7 Bambino Gesu Children‘s ester Children’s Hospital, Manchester, Department Of Pediatric Oncology,
Hospital, Patholgy Unit, Roma, Italy; 8 University Hospital Bonn, Sec- Manchester, United Kingdom; 4 Fondazione IRCCS Istituto nazionale dei
tion Of Pediatric Pathology, Department Of Pathology, Bonn, Germany; Tumori, Milano, Pediatric Oncology, Milano, Italy; 5 Clinique CHC Montlé-
9 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Oncology gia, Department Of Pediatrics, Liège, Belgium; 6 IHOPe, Léon Bérard
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S257 of S635

center, Department Of Pediatric Hematology And Oncology, Lyon, France; 1 Partners In Health, Oncology, KIGALI, Rwanda; 2 Dana-Farber cancer Insti-

7 Bambino Gesu Children‘s Hospital, Patholgy Unit, Roma, Italy; 8 Hospital tute, Pediatric Oncology, Boston, United States of America; 3 Partners In
Infantil Universitari Vall d’Hebron, Surgical Oncology And Neonatal Surgery, Health, Oncology Program, KIGALI, Rwanda; 4 Butaro Hospital / Rwanda,
Pediatric Surgery Department„ Barcelona, Spain; 9 Gustave-Roussy, Can- Pathology Laboratory, KIGALI, Rwanda; 5 Partners In Health, Research,
cer Campus, Department Of Pediatric And Adolescent Oncology, Ville- KIGALI, Rwanda; 6 University of Colorado, Research, Colorado, United
juif, France; 10 Padova University Hospital, Haematology Oncology Divi- States of America; 7 Butaro Hospital / Rwanda, Pediatrics, KIGALI, Rwanda
sion, Department Of Women’s And Children’s Health, Padova, Italy;
11 Rigshospitalet, Department Of Pediatrics And Adolescent Medicine, Background and Aims: Rhabdomyosarcoma (RMS) is the most com-
Copenhagen, Denmark; 12 Pediatric Hospital Virgen del Rocío, Pediatric mon childhood soft tissue sarcoma. Few studies have assessed its treat-
Oncology And Hematology, Sevilla, Spain ment outcomes in rural low resource settings. We aimed to describe
the characteristics and outcomes of patients with RMS presenting at a
Background and Aims: Epithelioid hemangioendothelioma (EHE) fre- rural cancer center in Rwanda.
quently has an indolent presentation despite potential diffuse tumor Methods: A retrospective chart review was performed on all patients
spread. To better characterize EHE, the European pediatric Soft tissue with RMS presenting at Butaro Cancer of Excellence (BCCOE)
sarcoma Study Group (EpSSG) analyzed all young patients prospec- between January 2013 and December 2019. Patients were treated
tively registered in treatment protocols. using a pediatric protocol adapted from Intergroup Rhabdomyosar-
Methods: Patients with localized EHE were included in the NRSTS- coma Study (IRS I-V) Group. Patient characteristics and outcomes were
05 study (EUDRACT 2005-001139-31) while metastatic tumors were reported using descriptive statistics. Event-free survival (EFS) was esti-
registered in the academic MTS-2008 study (NCT00379457). mated using the Kaplan-Meier where we considered death, disease
Results: Overall, 13 patients with localized and one with metastatic dis- progression, loss to follow-up and palliation events.
ease were included which represents 1% of all non-rhabdomyosarcoma Results: Thirty-four patients were included in this study. The median
cases registered. Sex ratio was 1, and median age 14 years (range, age at presentation was 8.9 years (IQR: 3.1-14.8) and 19 patients
0.1-19). Primary site was limbs (eight cases), trunk (five cases) and (55.9%) were female. The head and neck region was the most common
multifocal metastatic (one case). Tumors were smaller than 5 cm in primary site of disease: 10 (29.4%) in the orbit. On pre-surgical staging,
71% of cases. No patient had regional nodal involvement. IRS groups 22 (64.7%) were categorized as high risk. The most common histologic
were group-I seven cases, II two cases, III four cases and IV one case. subtype was embryonal (n=22, 64.7%). Two-thirds of patients under-
Local therapy was, initial primary surgery (10 cases) followed by an went surgery (n=23, 67.6%), 8 (34.8%) had uninvolved margins and 10
immediate primary re-resection (five cases). In addition, two patients (43.5%) received radiation. At the end of study period, of those who
had delayed surgery (R0, R2). No radiotherapy was delivered. Sys- received planned treatment (n=18), 10 (55.5%) died, 6 (33.3%) were
temic therapy for localized tumors consisted of interferon (2 cases), alive. Of the 16 who did not get all planned treatment, 9 (56.3%) died
vincristine with steroids (1), Temsirolimus (1) and paclitaxel (2 cases). (8 prior to surgery), 1 (6%) was alive. With a median follow-up time
Median follow-up for alive patients was 37 months (range, 6-176). One of 11.2 months, the one-year EFS was estimated to be 52.1% (95% CI:
patient had metastatic relapse and died. The patient with stage IV 34.1-67.3%) while two-year EFS was 21.5% (95%CI: 9.5-36.6).
disease developed tumor progression and received regorafenib, dox- Conclusions: The study demonstrated success in treating RMS in rural
orubicine and interferon, and then died for cardiac failure. At the end of low resource setting with adaptation of the IRSG pediatric proto-
follow-up, 12 patients remain alive off therapy (11 in complete remis- col although, the EFS is lower compared to reports from elsewhere.
sion and one with stable residual 5 years after). Five-year progression Efforts should focus on accessing early care, effective surgery and
free and overall survivals are respectively 82.1% (95%CI: 44.4 – 95.3) comprehensive social support.
and 78.6% (95%CI: 36.1 – 94.4).
Conclusions: EHE is a very rare sarcoma in pediatrics and mainly occurs
in adolescents. For localized disease, outcome is favorable. The role of EP237 / #1004 NEUROFIBROMATOSIS TYPE 2: WHAT DO
systemic therapy for initially unresectable tumors needs to be further WE DO AND WHAT CAN WE DO?
explored.
Roberto Palomar, Francina Lombardi, Agustin Cardoso, Blanca Diez
FLENI, Neuroncology, CABA, Argentina
EP236 / #1531 OUTCOMES OF PATIENTS TREATED FOR
RHABDOMYOSARCOMA AT BUTARO CANCER CENTER OF Background and Aims: Neurofibromatosis type 2 (NF2) is an AD
EXCELLENCE, RWANDA: 2013-2019 disorder, occurring in 1/25,000-40,000 live births, relate to multiple
sensory and motor disabilities throughout their lives, alongside with
Nsanzimana Oscar1 , Leslie Lehmann2 , Shyirambere Cyprien3 , development of different types of tumors, seriously affecting their
Ruhangaza Deogratias4 , Uwamahoro Pacifique5 , Cam Nguyen6 , quality of life. To describe NF2 patient’s situation, who underwent
Iradukunda Esperance7 , Ng’Ang’a Loise5 , Mujyuwisha Louis3 follow-up during childhood or adolescence in a neuro-oncology service.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S258 of S635 ABSTRACTS

Methods: Retrospective analysis of patients under 21 years of age Methods: The antitumoral activity of Ipatasertib was evaluated in
with NF2 treated in the neuro-oncology service of a hospital in Buenos vivo in patient-derived xenograft (PDX) established at HSJD, includ-
Aires, Argentina in the last 7 years. ing FP-RMS and FN-RMS. NOD/SCID mice subcutaneously implanted
Results: Twelve patients with NF2, M:F ratio 3:1. Average age 18 received 100mg/kg or 25mg/kg Ipatasertib PO QD for four weeks.
years, median age at the first consultation 18.5 years (8-21), one of the Blood and tumor samples were collected after a single dose for
patients had not been previously diagnosed. Eleven patients developed pharmacokinetic (PK) analysis. Transcriptomics was accomplished in
more than 3 different types of tumors: multiple schwannomas includ- RMS-PDX, primary cells, and their corresponding RMS patients’ biop-
ing acoustic neuromas (91.6%), peripheral schwannomas (91.6%), sies.
meningiomas (66%), medullary ependymomas (58%) and neurofibro- Results: Ipatasertib induced more than 50% of tumor volume reduction
mas (50%). Seven patients required 3 or more surgical interventions, in 4 out of 8 PDXs evaluated after 10 doses of 100mg/kg. At 25mg/kg
due to neurosurgical treatments or infectious complications, among dose, Ipatasertib slowed tumor growth in a dose-dependent manner
others. 55 total admissions. All patients had hearing loss and visual and the PK analysis exhibited an intratumoral Cmax over 11μM in one
impairments, together with hemiparesis (25%), chronic pain (25%), FN and one FP RMS-PDX. Such concentrations significantly inhibited
epilepsy (17%). Half of the patients received more than 5 daily medi- phospho-S6 and induced PARP cleavage. Functional transcriptomic
cations (30% indicated by psychiatry). Use of bevacizumab in 25% of analysis revealed an enrichment in proliferative pathways mediated
patients show a good clinical and audiometric response. Three of 10 by E2F and MYC targets (ES=0.70 and 0.58) paralleled with a deple-
evaluable patients presented ECOG score > 2 and Lansky < 50. Follow- tion in the expression of myogenesis-related genes (ES= -0.32) in most
up loss: 3 patients, 3 died (20%) and 1 is currently in ICU due to an acute Ipatasertib-sensitive RMS tumors.
SARS-COV19 infection. It is noteworthy that the older the patients Conclusions: Clinically feasible doses demonstrated effectiveness in
were, the more complication the added. blocking the growth of PDX-RMS. RMS tumors and RMS-PDXs more
Conclusions: Multidisciplinary approach is mandatory with initial pal- sensitive to Ipatasertib displayed transcriptional profiles associated
liative care management, given the torpid evolution of the disease with proliferation and poor myogenic differentiation.
and its unfailing progression towards progressive disability. We believe
that therapeutic accompaniment, psychological and spiritual approach
of the patients and their families should be a priority, just as surgical EP239 / #1246 THE ONCOGENIC ROLE OF HEDGEHOG
approaches and imaging controls are today. PATHWAY CO-RECEPTORS IN RHABDOMYOSARCOMA:
EXPANDING KNOWLEDGE TO DISCOVER NEW THERAPEUTIC
TARGETS
EP238 / #1567 TARGETING THE CELLULAR
DEVELOPMENTAL STATE WITH IPATASERTIB AS A NOVEL Josep Roma, Patricia Zarzosa, Soledad Gallego Melcón, Josep Sánchez
THERAPEUTIC OPPORTUNITY FOR AGGRESSIVE De Toledo, Lucas Moreno, Gabriel Gallo, Guillem Pons, Julia Sansa,
RHABDOMYOSARCOMA Natalia Navarro
Vall Hebron Research Institute, Childhood Cancer And Blood Disorders,
Estela Prada1 , Pablo Táboas1 , Inmaculada Hernandez-Muñoz2 , Jaume Barcelona, Spain
Mora3
1 Institut de Recerca Sant Joan de Déu, Developmental Tumor Biology Background and Aims: Rhabdomyosarcoma (RMS) is the most com-
Laboratory, Esplugues de Llobregat (Barcelona), Spain; 2 Fundació Institut mon soft tissue sarcoma in children. The mortality rate for RMS still
Hospital del Mar d’Investigacions Mèdiques, Programa De Recerca Clínica remains between 35-40%. A persistent activation of the Hedgehog
Translacional, Esplugues de Llobregat (Barcelona), Spain; 3 Pediatric Cancer (Hh) signaling pathway is well established and associated with worse
Center Barcelona, Hospital Sant Joan de Deu, Oncology, Barcelona, Spain prognosis and less muscle differentiation. Our group has been the
first in publishing the important role of Hh Ligands in RMS, propos-
Background and Aims: Pediatric Rhabdomyosarcoma (RMS) is a ing an autocrine Hh activation model. The standard pathway activation
developmental tumor that affects individuals from birth to young entails ligand binding to Patched receptor, which allows the activation
adulthood. Patients with metastatic RMS at diagnosis or with relapse of Smoothened and the subsequent activation of Gli proteins, the main
present a dismal prognosis. Previous inconclusive attempts aimed at effectors of the pathway. However, there are other co-receptors (Gas1,
blocking the AKT/mTOR pathway relied upon the sustained activa- CDO and BOC) that are also able to bind with ligands and even seem to
tion of the receptor tyrosine kinase (RTKs) signaling in RMS cells. The be necessary for complete Hh activation.
most transversal attribute across RMS tumors is the expression of myo- Methods: Since the role of these co-receptors in RMS has not yet been
genic lineage genes both in fusion-negative (FN) and fusion-positive characterized, we propose their study with the aim of finding new
(FP, PAX3/7-FOXO1 translocations) RMS subtypes. Here, we postulate molecular targets and opening up new therapeutic possibilities. First
that therapeutic vulnerabilities in RMS might depend on lineage dic- of all, a consistent expression of these co-receptors in RMS tumors
tates. The aim of this study is to describe the transcriptional myogenic was verified, as a previous step to attribute them an oncogenic role.
signature of those RMSs sensitive to the pan-AKT inhibitor Ipatasertib. Moreover, we genetically downregulated BOC and CDO expression
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S259 of S635

and studied the underlying molecular and functional consequences of score during post-test was 76.9% whilst average maximum score
their absence or pharmacollogic inhibition. An animal model is also post-test was 82.9%.
provided to test the effects of the depletion of CDO. Conclusions: The downstream training of PEWs has been effective.
Results: The results obtained permitted us to rule out an essential Similar models will be used for the training of PEWs in other regions in
oncogenic role of BOC. Conversely, CDO genetic or pharmacologic Ghana to improve on early detection, timely and appropriate referral
inhibition caused strong anti-oncogenic effects in vitro as decreased of childhood eye conditions, especially retinoblastoma, for timely and
cell proliferation, arrested cell cycle and, finally, cell differentiation and prompt management.
apoptosis induction. A rmarkable on tumor growth was also observed.
Conclusions: In conclusion, we propose the inhibition of CDO as a novel
and potent therapeutic target against RMS. EP241 / #324 SUSCEPTIBLE AND PROTECTIVE KILLER CELL
IMMUNOGLOBULIN-LIKE RECEPTOR (KIR) POLYMORPHISM IN
UNILATERAL, NON-FAMILIAL RETINOBLASTOMA
E-Poster Topic: AS05 SIOP Scientific programme / AS05.i
Retinoblastoma Madhulika Sharma1 , Usha Singh2 , Kay Poulton3 , Tanvi Bhatia1 ,
Navdeep Mangat1 , Nandita Kakkar4 , Deepak Bansal5 , Ritu Aggarwal1
E-POSTER VIEWING 1 Postgraduate Institute of Medical Education and Research, Department
Of Immunopathology, Chandigarh, India; 2 Postgraduate Institute of Med-
EP240 / #1034 IMPROVING KNOWLEDGE OF PRIMARY ical Education and Research, Oculoplastics And Retinoblastoma Services,
EYECARE WORKERS ON EARLY WARNING SIGNS AND Department Of Ophthalmology, Advanced Eye Center, Chandigarh, India;
SYMPTOMS OF RETINOBLASTOMA IN NORTHERN GHANA 3 Manchester Royal Infirmary, Transplant Laboratory, Manchester, United
Kingdom; 4 Postgraduate Institute of Medical Education and Research,
Ayire Adongo1 , Piera Freccero2 , Vera Essuman3 , Gilbert Bonsaana4 Histopathology, Chandigarh, India; 5 Advanced Pediatrics Center, Postgrad-
1 World Child Cancer, Paediatric Cancer, Accra, Ghana; 2 World Child Can- uate Institute of Medical Education and Research, Pediatric Hematology-
cer, Programme Department, London, United Kingdom; 3 Ophthalmology oncology Unit, Chandigarh, India
Unit, Department of Surgery, University of Ghana Medical School, Accra,
Ophthalmology, Accra, Ghana; 4 Department of Ophthalmology, School of Background and Aims: KIR regulates natural killer (NK) cell activity
Medicine, University for Development Studies, Ophthalmology, Tamale, to detect and eliminate tumor cells via interaction with class-I HLA
Ghana ligands. Both KIR and class-I HLA molecules exhibit extensive polymor-
phism. Inheritance of certain KIR and HLA combinations is associated
Background and Aims: Background Approximately 8000 new cases of with selected malignancies. RB1 inactivation triggers the initiation of
retinoblastoma are diagnosed annually around the world with most of retinoblastoma; however additional alterations are required for tumor
them in LMICs. In Ghana, about 65 – 70 children are affected annu- development. The aim was to explore the protective and susceptible
ally based on reports from general ophthalmologists from peripheral role of KIR/HLA polymorphism in the pathogenesis of retinoblastoma.
eye centres in the country. This paper presents on a strategy for early Methods: Patients with unilateral, non-familial retinoblastoma were
detection of retinoblastoma in a LMIC with funding from Alliance Mon- enrolled as cases. Healthy individuals matched for ethnicity were
diale le Contre Cancer (AMCC) through World Child Cancer (WCC). enrolled as controls. KIR genotyping was performed by sequence-
Objectives Build capacity of Primary Eyecare Workers (PEWs) on early specific primer assay. The investigated KIR genes included: inhibitory
warning signs and symptoms of retinoblastoma Assess the knowledge (2DL1, 2DL2, 2DL3, 2DL4, 2DL5A, 2DL5B), activating (2DS1, 2DS2,
of PEWs on early warning signs and symptoms of retinoblastoma 2DS3, 2DS4*FUL, 2DS4*DEL, 2DS5, 3DL1, 3DL2, 3DL3, 3DS1) and
Methods: Northern, Northeast and Savannah Regions of Ghana were pseudogenes (2DP1, 3DP1*FUL, 3DP1*DEL). Both haplotypic and
selected, and a simple random method used to select health facili- genotypic variations of KIR polymorphism were analyzed. In addition,
ties for the training. PEWs in these facilities were then selected. A HLA ligands were investigated by sequence-specific oligonucleotide
structured question was used to assess PEWs before training on Early assay for HLA-A, B, and C locus.
Warning Signs and Symptom (EWSS) of Retinoblastoma and same tool Results: KIR genotyping was performed in 48 cases and 107 controls.
used post-training to assess knowledge gain. One Trainer of Trainers The mean age of cases was 2.9±2.2 years (range: 0.25-10). Among the
session was carried out for 10 trainers who later conducted 9 training 19 KIR genes, the frequency of KIR2DS4*FUL (p=0.0019) and 2DS5
sessions across the 3 regions for 200 PEWs. (p=0.0095) was increased among cases. The distribution of AA and
Results: In the downstream training, average minimum score between Bx genotypes was similar among cases and controls. The frequency of
pre and post-test improved by 17% whilst average maximum score tel-A/B haplotype was decreased (p=0.0006), and tel-B/B increased
between pre and post-test improved by 7.8% at the end of the training in cases (p=0.021). HLA ligands were investigated in 25 cases and 50
sessions. Results in the pretest showed an average minimum knowl- controls. The frequency of HLA ligands (C1/C2, Bw4, A3/A11) was
edge of 65.4% and this improved to 76.9% average knowledge on similar among cases and controls. However, the KIR/HLA combination
retinoblastoma EWSS at the end of the training. Average minimum frequency for KIR3DS1/HLA-Bw4 was decreased in cases (p=0.006).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S260 of S635 ABSTRACTS

Conclusions: It is the first study to report the association of killer cell cytotoxic T cell-based and include tumor-infiltrating lymphocytes and
immunoglobulin-like receptors in retinoblastoma. KIR2DS4*FUL and T cell receptor-engineered T cells.
KIR2DS5 had a susceptible, and KIR3DS1/HLA-BW4 had a protective
role in retinoblastoma. The results will aid in exploring the therapeutic
potential of NK cell-based therapy for retinoblastoma. EP243 / #1360 RB1 GENE MUTATIONS IN TURKISH
PATIENTS WITH RETINOBLASTOMA

EP242 / #965 DECIPHERING THE IMMUNOGENETIC Demet Akdeniz Odemis1 , Rejin Kebudi2 , Seda Kilic Erciyas1 , Gozde
PROFILE OF RETINOBLASTOMA Kuru Turkcan1,3 , Seref Tuncer1 , Ozge Sukruoglu Erdogan1 , Jamila
Bayramova1 , Betul Celik1 , Busra Kurt Gultaslar1 , Sema Bay
Ritu Aggarwal1 , Madhulika Sharma1 , Usha Singh2 , Kay Poulton3 , Buyukkapu2 , Samuray Tuncer4 , Hulya Yazici1,5
Nandita Kakkar4 , Deepak Bansal5 1 Istanbul University, Oncology Institute, Department Of Cancer Genet-
1 Postgraduate Institute of Medical Education and Research, Immunopathol- ics, Istanbul, Turkey; 2 Istanbul University, Oncology Institute, Pediatric
ogy, Chandigarh, India; 2 Postgraduate Institute of Medical Education and Hematology-oncology, Istanbul, Turkey; 3 Halic University, Faculty of Arts
Research, Oculoplastics And Retinoblastoma Services, Department Of and Sciences„ Department Of Molecular Biology And Genetics, istanbul,
Ophthalmology, Advanced Eye Center, Chandigarh, India; 3 Manchester Turkey; 4 Istanbul University, Istanbul Medical Faculty, Ophtalmology, Istan-
Royal Infirmary, Transplant Laboratory, Manchester, United Kingdom; bul, Turkey; 5 Istanbul Arel University, Arel Medical Faculty„ Department Of
4 Postgraduate Institute of Medical Education and Research, Histopathol- Medical Biology And Genetics, istanbul, Turkey
ogy, Chandigarh, India; 5 Advanced Pediatrics Center, Postgraduate Institute
of Medical Education and Research, Pediatric Hematology Oncology Unit, Background and Aims: Retinoblastoma (RB) is the most common
Chandigarh, India intraocular malignancy in children, caused by mutations in the tumor
suppressor RB1 gene. The aim of this study is to assess the fre-
Background and Aims: Human leukocyte antigen (HLA) genes, also quency/type of RB1 gene mutations in a large cohort of Turkish RB
known as major histocompatibility complex, are highly polymorphic. cases.
HLA class 1 genes are present on most human cells and are involved in Methods: RB1 gene mutation screening was performed in peripheral
antigen presentation to the cytotoxic T lymphocyte. The genetic varia- blood samples of 219 individuals (122 children with RB/14 family mem-
tion of the HLA genes could lead to a heterogeneous immune response bers with RB/83 healthy family members of 47 probands with RB1
which may determine disease susceptibility. There is scarce literature mutations) followed in the Istanbul University, Oncology Institute. All
on HLA polymorphism in retinoblastoma. The aim was to explore the 27 exons and close intronic regions of the RB1 gene were sequenced
potential role of HLA polymorphism as a risk factor for retinoblastoma. for small deletions and insertions using the Sanger sequencing (2013-
Methods: The study was case-control and performed in a single cen- 2018) or Next Generation Sequencing (2019-2021); large deletions
ter to analyze the frequency of HLA class I genes in retinoblastoma. and duplications were investigated both by Multiplex Ligation Probe
Children on treatment or previously treated for unilateral, non-familial Amplification (MLPA) and copy number variation (CNV). Correlation
retinoblastoma were enrolled as cases. Healthy children matched for with demographic and clinical data were evaluated.
ethnicity were included as controls. The genomic DNA was extracted Results: After RB1 mutation screening, mutations were detected
from blood samples of cases and controls and subjected to HLA class in 57/136(41.9%) patients, in 23/84(27.4%)with unilateral, in
1 genotyping. HLA typing was performed at intermediate resolution 30/47(63.8%) bilateral RB, in 3/3 trilateral RB, and1/2 unilateral
using LAB Type® sequence-specific oligonucleotide Class I kits for HLA retinoma. Of these mutations, 45(78.9%) were small genetic rear-
A and B locus. HLA typing was carried out using a LUMINEX LABscan rangements, 12(21.1%) large genetic rearrangements. Frameshift
3D instrument. The interpretation was performed using HLA Fusion™ mutations were found in 11, nonsense in 18, splice error in 11, and
software. missense mutation in 1, synonymous substitution in 2, upstream
Results: Thirty-six cases and 86 controls were enrolled. The mean substitution in 2 patients. Ten novel mutations were found. Three of 83
age of the cases and controls was 3.3±2.3 years (range: 0.58-10) and healthy family members had germline RB1 mutation. The disease was
6.9±3.7 years (range: 0.25-12), respectively. The tumor was intraoc- hereditary in 13(22.8%); and de novo(77.2 %) in 44 of the 57 patients
ular in 27 (75%) and extraocular in 9 (25%). The frequency of the with mutations. Infants (p:0.021); bilateral/trilateral RB (p:0.0001);
HLA-A*24:02 allele was reduced (95% CI 0.13-0.82, OR: 0.33, p- those with light(green-blue) iris color vs dark color (71.4% vs 36.5%.
value: 0.0159) in cases as compared to controls. However, on applying p:0.003) had higher frequency of RB1 gene mutation.
Bonferroni correction, the frequency was not significant (corrected Conclusions: RB1 gene mutation was found in 41.9% of Turkish
p-value: 0.38). children with RB, 63.8% of bilateral, 27.4% of unilateral RB. 10
Conclusions: None of the HLA class 1 genes were associated with novel mutations of the RB1 gene were found according to the
susceptibility to retinoblastoma. The study of the HLA polymorphism Leiden Open Variation Database and the Human Gene Mutation
could aid in exploring HLA-dependent therapies, which are mainly Database.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S261 of S635

EP244 / #1774 AN IMMUNOHISTOCHEMISTRY PANEL IS A frequently high-risk pathology factors (HRPF) (76.25% vs 54.4%;
USEFUL TOOL FOR IDENTIFYING RETINOBLASTOMA p<0.0001), specifically massive choroid (p=0.0013) and retrolaminar
MOLECULAR SUBTYPES optic nerve invasion (p=0.0354). Enucleated eyes of patients that
experienced extraocular relapse (n=32) had a significantly higher
Rosario Aschero1 , Gabriela Lamas1 , Camilo Restrepo2 , Jasmine TFF1 QS (median=210, range 60 to 300) than those with no extraoc-
Francis3 , Maria Cuadrado Vilanova4,5 , Nathalia Grigorovski6 , Gustavo ular relapse (n=208) (median=67.5, range 0 to 300) (p<0.0001). All
Dufort7 , Ezequiel Nespoli1 , Palmira Barriga8 , Paula Schaiquevich9,10 , metastatic sites studied (n=13) were positive for TFF1
Claudia Sampor11 , Adriana Fandiño12 , Jaume Catala Mora13 , Mariona Conclusions: This immunohistochemistry panel is an accessible tool
Suñol14 , Daniela Ottaviani15 , David Abramson3 , Fabiana for identifying retinoblastoma molecular subtypes with possible appli-
Lubieniecki16 , François Radvanyi17 , Guillermo Chantada18 cation when access to multi-omics diagnosis is not available. Sub-
1 Hospital de Pediatría Garrahan, Pathology, Buenos Aires, Argentina; type 2 is associated with HRPFand a higher risk for extraocular
2 Hospital Sant Joan de Déu, Developmental Tumor Biology Laboratory, relapse.
Esplugues de Llobregat, Argentina; 3 Memorial Sloan Kettering Cancer
Center, Ophthalmic Oncology Service, New York, United States of Amer-
ica; 4 Institut de Recerca Sant Joan de Deu, Tractament Del Càncer EP245 / #1910 RETINOBLASTOMA (RB) IN SUB-SAHARAN
Pediàtric, Barcelona, Spain; 5 Hospital Sant Joan de Deu, Pediatric Oncol- AFRICA: A SINGLE TUMOR PROGRAM TO SUPPORT RB
ogy, Barcelona, Spain; 6 Instituto Nacional de Cáncer, Oncology, Rio de MULTIDISCIPLINARY TEAMS AND IMPROVE CURE RATE IN
Janeiro, Brazil; 7 Centro Hospitalario Pereira Rossell, Oncology, Montev- LINE WITH 2030 WHO OBJECTIVES
ideo, Uruguay; 8 Hospital del Niño “Manuel Ascencio Villarroel”, Pathology,
Cochambamba, Bolivia; 9 National Scientific and Technical Research Coun- Karim Assani1 , Fatoumata Sylla2 , Fousseyni Traore3 , Pascal
cil (CONICET), Oncology, Buenos Aires, Argentina; 10 Hospital de Pediatria Sirignano4 , Pierre Bey5 , Laurence Desjardins6
JP Garrahan, Precision Medicine, Buenos Aires, Argentina; 11 Hospital JP 1 University hospital of Kinshasa, DR Congo and AMCC, Pediatrics, Kin-
Garrahan, Pediatric Oncology, Buenos Aires, Argentina; 12 Hospital de Pedi- shasa, Congo, Republic of; 2 IOTA, Ophthalmology, Bamako, Mali; 3 Gabriel
atría Garrahan, Ophtalmology, Buenos Aires, Argentina; 13 Hospital Sant Toure University Teaching Hospital, Oncology Pediatry Unit, Bamako, Mali;
Joan de Deu, Ophthalmology, Esplugues de Llobregat, Spain; 14 Hospital Sant 4 AMCC, Prothelem, Ocularist, Paris, France; 5 Institut Curie, Amcc, Paris,
Joan de Deu, Pathology, Barcelona, Spain; 15 Institut Curie, Equipe Labellisée France; 6 Institut Curie and AMCC, Ophtalmology, Paris, France
Ligue Contre Le Cancer, Paris, France; 16 Hospital de Pediatria JP Garra-
han, Pathology, Buenos Aires, Argentina; 17 Institut Curie, Siredo Oncology Background and Aims: RB is a rare tumor with a potential high (close
Center, Cnrs, Umr144, Paris, France; 18 Hospital Pereira Rossell, Oncology, to 100%) cure rate when a recognized key role of the ophthalmolo-
Montevideo, Uruguay gist is effective. In collaboration with the RB multidisciplinary team,
ophthalmologists are strongly involved in the diagnosis, treatment
Background and Aims: Two molecular subtypes of retinoblastoma (enucleation and local ophthalmological conservative procedures) and
were described by multi-omic analysis and subtype 2 has a higher follow up. We present a RB program implemented since June 2019 with
propensity for metastasis. Since multi-omic analysis is not widely avail- strong involvement of ophthalmologists. This program draws on the
able, we propose an immunohistochemistry panel as a tool to identify experience gained since 2011 with GFAOP and the Bamako team, and
both subtypes. published in 2018.
Methods: Two hundred fifty-five consecutive cases from Argentina Methods: The objective of this 10 year RB program is to improve cure
and Barcelona were evaluated and an enhanced cohort of cases with rate from average of < 20 % to > 70% of expected cases of RB in each
extraocular relapse was enriched with patients from the remaining country by directly supporting the RB teams, thanks to a Swiss Foun-
centers (n=21). Immunohistochemistry included ARR3, CRX, Ki67 and dation and Curie Institute. Five main areas are supported through this
TFF1 antibodies. TFF1 was selected because its high expression in program: training, equipment (for enucleation and eye salvage), early
subtype 2 in transcriptomic analysis and lack of expression in sub- diagnosis campaigns, improvement of data collection and discussions
type 1. The quick score (QS) value was calculated and subtype 2 was on challenging cases.
defined when the QS for TFF1 was>45. Validation of the immuno- Results: Up to date (3rd year of implementation), a network of 31 RB
histochemistry panel was performed against multi-omic analysis in 39 teams from 22 countries is established with 11 early diagnosis multi-
cases. year plan underway. Equipment for eye salvage has been provided in
Results: In 94.8% the immunohistochemistry characterization 16 countries and ophtalmologists have been trained in Curie institute
matched the results obtained by multi-omic analysis. Overall, of 276 or in Bamako. An expert tumor board online meeting is held in regular
cases evaluated, 103 (37.3%) were subtype 1, 160 (58%) subtype 2 and basis or in demand.
13 (4.7%) were not evaluable because of tumor necrosis. Compared Conclusions: This program represent a unique opportunity to
to subtype 1, children with subtype 2 tumors were older at diagno- learn about a single childhood cancer intervention in sub-Saharan
sis (median age 29 vs 14 months; p=0.0003), had more commonly Africa. Bringing ophthalmologists as key actors seem to be
unilateral disease (80.2% vs 68.93%; p=0.006) and presented more essential.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S262 of S635 ABSTRACTS

EP246 / #480 HUMAN PAPILLOMAVIRUS IN CERVICAL Rachel Brennan1 , Sarah Huemmer2 , Lisa Krull2 , Carlos
SMEAR OF MOTHERS OF CHILDREN WITH NON-FAMILIAL, Rodriguez-Galindo3 , Matthew Wilson4,5
UNILATERAL RETINOBLASTOMA: A CASE-CONTROL STUDY 1 St. Jude Children’s Research Hospital, Oncology, Memphis, United States of

America; 2 St. Jude Children’s Research Hospital, Global Pediatric Medicine,


Arti Yadav1 , Manjit Kaur1 , Tanvi Bhatia2 , Ritu Aggarwal2 , Madhulika Memphis, United States of America; 3 St Jude Children’s Research Hospital,
Sharma2 , Vanita Suri3 , Usha Singh4 , Deepak Bansal1 Global Pediatric Medicine, Memphis, United States of America; 4 St. Jude
1 Advanced Pediatrics Center, Postgraduate Institute of Medical Educa- Children’s Research Hospital, Surgery, Memphis, United States of America;
tion and Research, Pediatric Hematology-oncology Unit, Chandigarh, India; 5 University of Tennessee Health Sciences Center, Hamilton Eye Institute,
2 Postgraduate Institute of Medical Education and Research, Immunopathol- Memphis, United States of America
ogy, Chandigarh, India; 3 Postgraduate Institute of Medical Education and
Research, Obstetrics And Gynecology, Chandigarh, India; 4 Advanced Eye Background and Aims: Through a Global Retinoblastoma Need’s
Centre, Postgraduate Institute of Medical Education and Research, Special- Assessment, we identified a need for further education that focused
ity Of Oculoplastics And Retinoblastoma, Department Of Ophthalmology, on multidisciplinary management. The goal of the Online Retinoblas-
Chandigarh, India toma Academy was to provide an educational resource for multidisci-
plinary teams that focused on effective treatment and management of
Background and Aims: The association between HPV and retinoblas- retinoblastoma while examining the impact of resource limitations.
toma is inconsistently reported from 0 to 69%. Interaction between Methods: The course had eight (8) modules to complete over 10-
RB1 tumor suppressor and oncoproteins of DNA viruses is described. weeks. Resources were compiled to meet each learning objective. A
Inactivation of retinoblastoma protein by HPV 16 and 18, suggests a mixture of video lectures, journal articles, readings, and case examples
possible role. We hypothesized maternal HPV to be an etiological fac- were used. Knowledge checks and discussion forums in each module
tor for retinoblastoma. If so, the frequency of HPV in the cervical smear ensured learning objectives were being met and provided an opportu-
in mothers of children with retinoblastoma would be higher. nity for collaboration. Teams completed project proposals at the end of
Methods: The case-control, single-center study was conducted dur- the course to implement in their own centers.
ing 2016-2020. Patients on treatment or previously treated for Results: A total of 285 participants enrolled in the first 10-week
unilateral retinoblastoma, born by vaginal delivery, and lacking a course. 122 participants fully completed the course (43%). 38 cen-
family history of retinoblastoma were identified. The mothers of ters registered which represented 22 different countries and all WHO
these patients/survivors were enrolled as cases. In addition, moth- global regions were represented. There were a wide range of spe-
ers whose children did not have retinoblastoma were enrolled as cialties represented including 72 pediatric hematology/oncologists, 56
controls. A cervical smear was obtained with a cytobrush from the ophthalmologists, 31 pediatric ophthalmologists, and 25 pathologists.
enrolled mothers and run for polymerase chain reaction for HPV. A total of 15 team project proposals were presented.
HPV Genoarray kit was utilized for genotyping and detection of Conclusions: The academy was useful in creating a collaborative,
high-risk (16,18,31,33,35,39,45,51,52,53,56,58,59,66,68) and low-risk global space for multidisciplinary teams to expand their knowledge
[6,11,42,43,44,CP8304(81)] HPV subtypes. of the treatment and management of children with retinoblastoma.
Results: During the study period of 56 months, 42 patients/survivors The discussions during the academy initiated the development of the
with non-familial, unilateral retinoblastoma, born by vaginal deliv- Global Retinoblastoma Advisory Group and the projects that should be
ery were identified. The mothers of the 42 patients/survivors were addressed.
enrolled as cases. The controls included 57 women. The cases and con-
trols did not differ in age at marriage (p=0.670), age at conception
(p=0.170), or rural/urban locality (p=0.744). However, the cases had EP248 / #2019 EXTENDING THE REACH: THE IMPACT OF
a higher socio-economic scale (modified-Kuppuswamy scale: I+II+III: ORBIS CYBERSIGHT OVER ON GLOBAL RETINOBLASTOMA
23.8% vs. 19.2%; p=0.021) and were older (27.4±3.0 vs. 26.1±3.1 OVER TWO DECADES
years; p=0.047) than controls. High-risk HPV (predominantly 16 and
18) were detected in 9 (21.4%) cases and 14 (24.6%) controls (p=0.72). Rachel Brennan1 , Daniel Neely2 , Jonathan Scollard3 , Sarah
Conclusions: The frequency of high-risk HPV in the cervical smears of Huemmer4 , Lisa Krull4 , Carlos Rodriguez-Galindo5 , Hunter Cherwek3 ,
mothers of children with non-familial retinoblastoma was not different Matthew Wilson6,7
from the mothers whose children lacked retinoblastoma. The hypothe- 1 St. Jude Children’s Research Hospital, Oncology, Memphis, United States
sis of maternal transmission of HPV during vaginal delivery to the baby of America; 2 Indiana University School of Medicine, Ophthalmology, indi-
as an etiology for non-familial retinoblastoma was not supported. anapolis, United States of America; 3 Orbis Cybersight, Clinical Services,
New York, United States of America; 4 St. Jude Children’s Research Hos-
pital, Global Pediatric Medicine, Memphis, United States of America;
EP247 / #2016 A GLOBAL ONLINE RETINOBLASTOMA 5 St Jude Children’s Research Hospital, Global Pediatric Medicine, Mem-
ACADEMY phis, United States of America; 6 University of Tennessee Health Sciences
Center, Hamilton Eye Institute, Memphis, United States of America; 7 St.
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ABSTRACTS S263 of S635

Jude Children’s Research Hospital, Surgery, Memphis, United States of ture was determined, with each organization allowed two full members
America and unlimited alternate members. The group nominated and elected a
separate standing committee and global community co-chair, set term
Background and Aims: Orbis is a non-profit organization devoted to limits and proposed meeting schedule and initial project priorities.
blindness prevention and treatment in developing countries. Cyber- Results: The Global Retinoblastoma Advisory Group has 77 mem-
sight, which is an online training and mentorship service for healthcare bers and 13 alternate members, including representation from pedi-
professionals, provides online training and consultations. Cybersight atric oncology, ocular oncology, nursing and research, with plans to
works to increase capacity of healthcare professionals involved in eye expand to include parent/patient representation and other social sup-
health and health systems by providing live webinars, online courses, port members. The standing committee, representing all seven World
and consultations. The goal of Cybersight is to facilitate mentorship Health Organization regions, has 15 members and will begin meeting in
and training between healthcare professionals in developing countries January 2022 to develop working groups for prioritized projects. The
by connecting individuals to specialists in developed countries and top areas of concern include early detection/education for diagnosis,
providing online resources. The telemedicine platform can be used abandonment of care, and acquisition of resources for ophthalmologic
to improve vision and survival outcomes, specifically in children with interventions.
retinoblastoma. Conclusions: Engagement of global stakeholders in developing and
Methods: Each case on Cybersight is assigned a mentee and a mentor implementing initiatives to improve outcomes for children with
that will work together through telemedicine consultations. The first retinoblastoma is essential.
retinoblastoma case in Cybersight occurred in July 2003. There are 10
mentors who specialize in retinoblastoma that help consult on cases.
Results: There have been 978 retinoblastoma cases via Cybersight EP250 / #1980 MULTIMODALITY TREATMENT INCLUDING
since 2003. This averages to 4.4 retinoblastoma cases per month from INTRA-ARTERIAL CHEMOTHERAPY FOR DISSEMINATED
2003 to present. The consultations vary in length based on case. Con- RETINOBLASTOMA
sults have occurred with individuals from 31 countries. 35.7% of cases
are from Guatemala and 32.1% of cases are from Jordan. Nathalia Grigorovski1 , Carla Macedo2 , Clarissa Mattosinho3 , Luiz
Conclusions: Cybersight provides a unique platform that allows for Fernando Teixeira2 , Adriana Fandiño4 , Hugo Gouveia1 , Jose Roberto
global communication and education. This resource is seen to produce Fonseca2 , Flavio Requejo5 , Claudia Sampor4
impactful training opportunities through consultations, specifically in 1 Instituto Nacional de Cáncer, Oncology, Rio de Janeiro, Brazil;
Guatemala, Jordan, and other developing countries. 2 Universidade Federal de São Paulo, Instituto De Oncologia Pediatrica-
graacc, São Paulo, Brazil; 3 Instituto Nacional de Cancer, Ocular Oncology,
Rio de Janeiro, Brazil; 4 Hospital JP Garrahan, Pediatric Oncology, Buenos
EP249 / #2025 FORMATION OF A GLOBAL Aires, Argentina; 5 Hospital JP Garrahan, Department Of Pediatric
RETINOBLASTOMA ADVISORY GROUP Interventional Neuroradiology, Buenos Aires, Argentina

Rachel Brennan1 , Lisa Krull2 , Sarah Huemmer2 , Guillermo Chantada3 , Background and Aims: Extraocular retinoblastoma remains difficult
Matthew Wilson4,5 to cure with current treatments as high dose chemotherapy and radio-
1 St. Jude Children’s Research Hospital, Oncology, Memphis, United States of therapy leading to long-term sequelae. CNS involvement can’t be cured
America; 2 St. Jude Children’s Research Hospital, Global Pediatric Medicine, with conventional treatment. Hence, innovative strategies aiming to
Memphis, United States of America; 3 Hospital Austral, Pediatric Oncology, increase survival rates with lower sequelae are needed.
Pilar, Argentina; 4 University of Tennessee Health Sciences Center, Hamil- Methods: Retrospective report from 3 institutions using a similar
ton Eye Institute, Memphis, United States of America; 5 St. Jude Children’s pilot approach for treating patients with overt orbital retinoblastoma
Research Hospital, Surgery, Memphis, United States of America with (stage IVb) and without CNS involvement (stage III).For stage
III patients, the aim was avoiding orbital radiotherapy with inten-
Background and Aims: Participants in the Global Retinoblastoma sive neoadjuvant chemotherapy and limited surgery followed by 3
Academy recognized similar challenges to caring for children with cycles of intra-arterial chemotherapy(IAC) with topotecan-melphalan
retinoblastoma across regions and sought further opportunities to con- as local consolidation. For stage IV patients, intensive chemother-
nect and learn from one another while developing shared strategies for apy was used alternating with intra-arterial chemotherapy with high
addressing gaps in care. The Global Retinoblastoma Advisory Group dose carboplatin,melphalan and topotecan and also added intrathecal
was organized to partner in the development, support, and implemen- chemotherapy with topotecan.
tation of strategic regional initiatives for improving the survival and Results: From 2016 to 2021, 8 patients were treated (4 stage III; 4
vision outcomes of children with retinoblastoma. stage IVb) (6 unilateral,2 bilateral).All 4 stage III patients received sys-
Methods: Applications for membership were sent via email to academy temic chemotherapy and a median of 3 cycles of IAC and achieved
participants (22 countries) and network members and were posted in complete remission for a median of 34 months (range 15 to 56) with-
the Retinoblastoma Global online community. The basic group struc- out orbital radiotherapy. Stage IVb patients (n=4), 3/4 patients had
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S264 of S635 ABSTRACTS

chiasmatic involvement and suprasellar lesions and 2/4 with lep- patients died of metastatic disease and there was no active disease in
tomeningeal dissemination. Two of 3 patients with chiasmatic involve- 24.
ment achieved complete remission and one of them is disease-free for Conclusions: Our findings suggest that topotecan is active as second
25 months. One case abandoned therapy in complete remission and line chemotherapy in refractory/recurrent retinoblastoma. Advance
was lost to follow-up. The 2 leptomeningeal dissemination patients stage, presence of seeds in general and vitreous seeds in specific are
showed progressive disease and died within 2/3 months. Genomic pro- predictors of poor response to topotecan.
filing of 3 patients of stage IV showed MYCN gains/amplifications and
BCOR mutations in 1.
Conclusions: In this small pilot study,a multimodal approach introduc- EP252 / #602 THE ASSOCIATION BETWEEN MATERNAL
ing intra-arterial chemotherapy at higher doses, in association with NUTRIENT INTAKE DURING PREGNANCY AND THE RISK OF
high dose chemotherapy seems promising for stage III retinoblas- SPORADIC UNILATERAL RETINOBLASTOMA
toma avoiding radiotherapy. Three cases with stage IVb and chiasmatic
disease, which is not curable with conventional therapy, achieved com- Eun Mi Jung1 , Greta Bunin2 , Arupa Ganguly3 , Rebecca Johnson1 ,
plete remission but patients with leptomeningeal dissemination may Logan Spector1
need a different approach 1 University of Minnesota Medical School, Pediatrics, Minneapolis, United
States of America; 2 Children’s Hospital of Philadelphia, Division Of Oncol-
ogy, Philadelphia, United States of America; 3 University of Pennsylvania,
EP251 / #416 TOPOTECAN IN REFRACTORY/RECURRENT Department Of Genetics, Philadelphia, United States of America
INTRAOCULAR RETINOBLASTOMA: OUTCOME AND RESPONSE
PREDICTIVE FACTORS Background and Aims: Previous studies have associated maternal diet
with the risk of retinoblastoma (RB) in offspring. However, most stud-
Hadeel Halalsheh1 , Yacoub Yousef2 , Mona Mohammad2 , Alaa Saleh3 , ies have examined the role of food groups rather than nutrients or
Iyad Sultan1 supplements. The aim of the current study is to investigate the associ-
1 King Hussein Cancer Center, Pediatric, Amman, Jordan; 2 King Hussein ation between maternal nutrient intake during pregnancy and the risk
Cancer Center, Surgery/ophthalmology, Amman, Jordan; 3 King Hussein of sporadic unilateral retinoblastoma in offspring.
Cancer Center, Nursing, Amman, Jordan Methods: A food frequency questionnaire which was modified for use
in a phone interview was completed from 187 sporadic unilateral RB
Background and Aims: Topotecan showed efficacy in retinoblastoma, cases 155 controls. Supplement use during pregnancy was also asked.
we report our experience with systemic topotecan in pediatric patients We performed logistic regression to estimate the odds ratios (ORs) and
with refractory/recurrent retinoblastoma, and we looked for predictive the 95% confidence intervals (CIs).
factors for response. Results: In the unadjusted model, retinol activity equivalents (RAE) and
Methods: This is a retrospective review for patients with refrac- vitamin D had significant inverse associations with the risk of sporadic
tory/recurrent retinoblastoma treated at King Hussein Cancer Cen- unilateral RB (OR: 0.76, 95% CI: 0.62—0.94, p-value: 0.01; OR: 0.82,
ter between Jun2007 and Jul2019 using topotecan. Topotecan was 95% CI: 0.70—0.96, p-value: 0.01). When adjusted for child’s birth year,
given as a second line chemotherapy for refractory/recurrent disease. maternal race, maternal education, and total calorie intake, RAE and
Patients and disease characteristics, response to therapy, eye salvage vitamin D significantly reduced the risk of sporadic unilateral RB (OR:
and survival were depicted. 0.67, 95% CI: 0.51—0.88, p-value: 0.004; OR: 0.78, 95% CI: 0.65—0.94,
Results: Twenty-six patients, 37 eyes with refractory/recurrent p-value: 0.009). In the fully adjusted model, RAE and vitamin D contin-
retinoblastoma were reviewed, all received topotecan as second ued to show an inverse association with the risk of sporadic unilateral
line chemotherapy (3 cycles in 31 eyes, and 4-6 cycles in 6). The RB (OR: 0.68, 95% CI: 0.51—0.91, p-value: 0.01; OR: 0.76, 95% CI:
median age at diagnosis was 6 months (range, 1-17). Seventeen 0.61—0.93, p-value: 0.008).
(38%) were males and 22 (85%) had bilateral disease. Nineteen Conclusions: Our study replicated the previous findings that vita-
eyes (51%) were IIRC group D, 12 (32%) group C, and six were mins A and D may reduce the risk of cancer development. However,
group B. Twenty-two eye (59%) had seeds (sub-retinal in 13 and the mechanisms that maternal nutrient intake influences the risk of
vitreous in 9). Toptecan was given for residual active disease in sporadic unilateral RB in offspring still need to be investigated. Fur-
20 eyes (54%) and recurrent in 17 (46%). Nineteen eyes (51%) ther study is warranted to enhance the understandings of the role of
showed excellent response, five (14%) had partial-response, and maternal nutrient intake in the etiology of sporadic unilateral RB.
13 (35%) showed progression or no response. Twenty-three eyes
(62%) were salvaged (no radiation or enucleation), 10 (27%) under-
went enucleation, and five eyes received radiation. On multivariate EP253 / #168 RETINOBLASTOMA IN TURKEY: CLINICAL
analysis advance IIRC group, presence of seeds and vitreous seeds PRESENTATION AND OUTCOME 1983-2017
subtype were predictive factors for poor response to topotecan (p
values were 0.038, 0.035, 0.024 respectively). At last follow up 2 Serife Ciloglu1 , Rejin Kebudi2 , Gonul Peksayar1 , Samuray Tuncer1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S265 of S635

1 Istanbul University, Istanbul Medical Faculty, Ophtalmology, Istanbul, Health, Toronto, Canada; 8 University of Toronto, Division Of Clinical Public
Turkey; 2 Istanbul University, Oncology Institute, Pediatric Hematology- Health, Dalla Lana School Of Public Health, Toronto, Canada
oncology, Istanbul, Turkey
Background and Aims: FACE-Q is a patient-reported outcome mea-
Background and Aims: Retinoblastoma is the most common primary sure (PROM) that evaluates appearance-based and associated psy-
intraocular malignancy of childhood with different survival rates across chosocial outcomes of craniofacial surgery. This study aimed to assess
the world. The aim of this study is to evaluate the clinical characteris- the content validity of FACE-Q among retinoblastoma (RB) survivors
tics, treatment approach and outcome (patient survival, eye survival) and parents/guardians.
in retinoblastoma cases treated in the Istanbul University, Istanbul Methods: This was a cross-sectional qualitative study. Eligible partic-
Medical Faculty, Department of Ophtalmology and Oncology Institute, ipants included RB survivors ≥8 years old and parents/guardians of
Division of Pediatric Oncology, Turkey between 1983 and 2017. survivors <8 years old. Seven participants per type were recruited, as
Methods: The medical records of 297 patients diagnosed with suggested by COnsensus-based Standards for the selection of health
retinoblastoma between 1983-2017 were evaluated retrospectively. Measurement INstruments (COSMIN) guidelines. In cognitive debrief-
Information on gender, laterality, age at diagnosis, presenting signs, ing interviews, participants reviewed FACE-Q sections measuring eye-
spread of tumor, treatment modality, survival rate, and family history related, appearance-based, and psychosocial outcomes, and provided
were evaluated. feedback on their relevance, comprehensiveness, and comprehensibil-
Results: Of 297 patients (403 eyes), 191 (64.3%) had unilateral and ity relative to the RB experience. Interviews were transcribed, coded,
106 (35.7%) had bilateral involvement. The mean age at diagnosis was and analyzed via constant comparative analysis to confirm or disprove
21.29 ±18,68 (1-216) months for all patients, 25.15 ±18.7 (1-216) the above factors, and identify new concepts and suggestions for mod-
for unilateral and 14.39 ±16.5 (1-180) months for bilateral cases. The ifications. The number of participants offering each specific feedback
most common presenting symptoms were leukocoria (54.2%) and stra- or suggestion was tallied; a minimum of three instances was required
bismus (36.3%). Family history of retinoblastoma was present in 25 to modify the FACE-Q. Applicable FACE-Q questions were adapted
(8.4%) patients. Most of the eyes were ICRB group E (40.9%), and D and subsequently re-evaluated by survivors and parents/guardians for
(20.3%). Systemic chemotherapy, local ophtalmic treatment, intraar- corroboration purposes.
terial chemotherapy, radiotherapy, enucleation was used according to Results: Participants deemed all FACE-Q sections reviewed as relevant
patient’s clinical findings and treatment era. Enucleation was done pri- to the RB experience. To improve the relevance and comprehen-
marily in 58.1% of unilateral, 21.6% of bilateral cases. The frequency siveness of the measure, participants suggested additional concepts,
of enucleation decreased from 64.9% to 32.5% after 2011. The 5-year including those related to prosthetic eyes and visual impairment. While
eye protection rate was 100% in Group A, 88.4% in Group B, 66.7% survivors suggested adding the concept of interpersonal relationships,
in Group C, 55.4% in Group D and 4.5% in group E. The 5-year overall parents indicated that survivors <8 years old are not yet self-conscious
survival of all patients was 97.2 %. or concerned about their appearance. To improve comprehensibil-
Conclusions: The implementation of up-to-date treatments and mul- ity, a common consensus among participants was that certain terms,
tidisciplinary team approach has resulted in high survival and high and existing response options and instructions should be altered to
eye salvage rates in our cohort. The intraocular advanced stage at enhance clarity and appropriateness in relation to RB, visual impair-
presentation is still high which emphasizes the importance of public ment, laterality, and enucleation.
and healthcare staff education. The enucleation rate has significantly Conclusions: The study results suggest key modifications to FACE-Q to
decreased over the years. make it comprehensible, comprehensive, and relevant to RB survivors.
In future, the adapted FACE-Q will undergo field testing for assessment
of validity and reliability.
EP254 / #1737 EVALUATION AND ADAPTATION OF THE
FACE-Q | CRANIOFACIAL PATIENT-REPORTED OUTCOME
MEASURE FOR RETINOBLASTOMA EP255 / #1225 ADJUVANT CHEMOTHERAPY FOR CHILDREN
WITH ADVERSE HISTOLOGY POST-ENUCLEATION FOR
Farheen Khan1,2 , Roxanne Noronha1 , Sara Williams1 , Karen RETINOBLASTOMA – A SINGLE CENTRE EXPERIENCE OVER 20
Wong-Riff3,4 , Asim Ali1,5 , Helen Dimaras1,5,6,7,8 YEARS
1 The Hospital for Sick Children, Ophthalmology And Vision Sciences,
Toronto, Canada; 2 University of Toronto, Institute Of Medical Science, Gerard Millen1 , Joseph Abbott2 , Claire Bowen3 , Manoj Parulekar2 ,
Toronto, Canada; 3 The Hospital for Sick Children, Division Of Plastic And Helen Jenkinson1
Reconstructive Surgery, Surgery, Toronto, Canada; 4 University of Toronto, 1 Birmingham Children’s Hospital, Paediatric Oncology, Birmingham, United

Division Of Plastic And Reconstructive Surgery, Surgery, Toronto, Canada; Kingdom; 2 Birmingham Children’s Hospital, Eye Department, Birming-
5 University of Toronto, Ophthalmology & Vision Sciences, Toronto, Canada; ham, United Kingdom; 3 Birmingham Children’s Hospital, Department Of
6 SickKids Research Institute, Child Health Evaluative Sciences Program, Histopathology, Birmingham, United Kingdom
Toronto, Canada; 7 SickKids Research Institute, The Centre For Global Child
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S266 of S635 ABSTRACTS

Background and Aims: Up to 25% of children with retinoblastoma Methods: The single-center, single-blinded, randomized study was con-
undergoing primary enucleation have evidence of adverse histopatho- ducted during 2019-2021. Patients with newly diagnosed Group D
logical features indicating the need for adjuvant chemotherapy. We or E retinoblastoma were randomised to receive vincristine, etopo-
aimed to review the experience at a single large national referral cen- side, and higher-dose carboplatin (<36 months: 28 mg/kg; ≥36 months:
tre over a 20-year period to correlate pathological features with clinical 840 mg/m2) or standard-dose carboplatin (<36 months: 18.6 mg/kg;
outcome. ≥36 months: 560 mg/m2) chemotherapy. The eyes were evaluated by
Methods: Patients diagnosed with retinoblastoma undergoing pri- examination under anesthesia and ultrasonography at diagnosis and
mary enucleation were identified from the Retinoblastoma Reg- following 3-cycles of chemotherapy. Group E eyes with poor likelihood
istry at a single institution which covers the UK and Ireland (pop- of globe and vision salvage were excluded.
ulation 46 million) and receives 60% of national referrals. Data Results: Thirty-two eyes of 29 patients were enrolled: 17 Group
were reviewed to identify which adverse histopathological feature D and 15 Group E eyes. Baseline demographic and tumor charac-
required adjuvant chemotherapy. This was correlated with patient teristics were similar in the two arms. Among the group D eyes
outcome. (higher-dose: 6; standard-dose: 11), the response of the tumor to
Results: Between 1/1/2002 and 31/12/2021, 83 patients had adverse chemotherapy pertaining to regression pattern (p=1.00), reduction
histological features of whom 62 (75%) were treated with 4 cycles in tumor-size (diameter: p=0.78; height: p=1.00), subretinal fluid
of vincristine, etoposide and carboplatin (VEC). The patients excluded (p=1.00), subretinal seeds (p=0.69) and vitreous seeds (p=0.69) was
from 4 cycles of VEC included those with very high-risk disease comparable between the two arms. The globe-salvage (higher-dose:
(tumour at the cut-end of the optic nerve, scleral involvement) or those 100% vs. standard-dose: 81.8%; p=0.52) and salvage of meaning-
treated on an individualised regimen at the physician’s discretion. The ful vision (higher-dose: 60% vs. standard-dose: 81.8%; p=0.24) were
event free and overall survival for the group receiving 4 cycles of VEC comparable as well. Among the group E eyes (higher-dose: 7; standard-
was 98.4% at a medium time of 13 years from end of treatment. The dose: 8), the response of tumor pertaining to regression pattern
indication for adjuvant chemotherapy was massive choroidal invasion (p=1.00), reduction in tumor-size (diameter: p=0.48; height: p=0.89),
in 22 (35.5%), post-laminar optic nerve involvement in 15 (24.2%), mul- subretinal fluid (p=0.64), subretinal seeds (p=1.00) and vitreous seeds
tiple adverse factors in 15 (24.2%), anterior chamber involvement in 6 (p=1.00) was comparable between the two arms. The globe-salvage
(9.7%) and unknown in 4 (6.4%). The only patient who relapsed and died (higher-dose: 57.1% vs. standard-dose: 12.5%; p=0.12) and salvage
had massive choroidal invasion with tumour found in the peripapillary of meaningful vision (higher-dose: 42.9% vs. standard-dose: 12.5%;
space which has been reported to increase the risk of central nervous p=0.28) were non-significantly higher in the higher-dose arm. No
system relapse. excess treatment-related toxicity was observed in the higher-dose arm.
Conclusions: Our data indicate that 4 cycles of adjuvant VEC are safe Conclusions: Higher-dose carboplatin-based intravenous chemother-
and effective for patients with adverse histopathological features fol- apy did not improve globe or vision salvage in group D or E retinoblas-
lowing primary enucleation for retinoblastoma. Intensification of treat- toma.
ment may be warranted in patients with peripapillary involvement.
Some patients may have treatment safely de-escalated further.
EP257 / #1404 «INVISIBLE» RETINOBLASTOMA: SMALL
PRIMARY TUMORS AND CONTINUED TUMOR GROWTH
EP256 / #601 A SINGLE-BLINDED, RANDOMIZED AFTER TREATMENT REVEALED BY OPTICAL COHERENCE
CONTROLLED TRIAL OF STANDARD VERSUS HIGHER-DOSE TOMOGRAPHY
CARBOPLATIN-BASED INTRAVENOUS CHEMOTHERAPY FOR
GROUP D AND E RETINOBLASTOMA Svetlana Saakyan, Elena Myakoshina, Dgavgarad Ismailova, Robert
Tatzkov
Safal Muhammed M K1 , Pritam Roy1 , Usha Singh2 , Sameeksha Moscow Helmholtz Centre of Eye Diseases, Ocular Oncology Centre,
Gowravajhala2 , Richa Jain1 , Amita Trehan1 , Deepak Bansal1 Moscow, Russian Federation
1 Advanced Pediatrics Centre, Postgraduate Institute of Medical Educa-
tion and Research, Pediatric Hematology Oncology Unit, Chandigarh, India; Background and Aims: Background. Retinoblastoma is a malignant
2 Advanced Eye Centre, Postgraduate Institute of Medical Education and tumor in children whose diagnostic algorithm includes morphomet-
Research, Speciality Of Oculoplastics And Retinoblastoma, Department Of ric research. OCT allows to reveal visible tumors, but its informative
Ophthalmology, Chandigarh, India in diagnosis of «invisible» retinoblastoma requires further study. Aim.
To reveal diagnostic symptoms of «invisible» small primary retinoblas-
Background and Aims: There is limited access to intra-arterial toma and continued tumor growth after treatment by OCT.
chemotherapy for retinoblastoma in low- and middle-income coun- Methods: The analysis of changes of the retina, revealed by OCT
tries. The aim was to compare the efficacy of standard versus higher- at 61 children with retinoblastoma at different stage of treatment
dose carboplatin-based intravenous chemotherapy in patients with with research of 108 tumors (primary small retinoblastoma - 24,
Group D and E retinoblastoma. new «invisible» focus after chemotherapy-5, residual tumour after
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S267 of S635

local treatment by methods of brachytherapy and transpupillary ther- African country. Articles were excluded if retinoblastoma or Africa was
motherapy – 34, chorioretinal scar - 36, «invisible» continued tumor not the primary study focus or location, respectively. Data collected
growth - 9) was carried out. included journal and citation information, authors affiliations and study
Results: OCT-symptoms of 24 primary retinoblastoma: homogeneous type/site/summary.
tumor in inner retinal layers, hyperreflectivity of tumour tissue with Results: The search yielded 1,094 citations. After deduplication and
(18) or without (9) «shadow» effect, equal choroidal profile. There initial title and abstract screening, 119 full-text articles were assessed
were no symptoms by Ret Cam and echography, but new «invisible» for eligibility, and 59 were included in the review. The countries repre-
small retinoblastoma tumors after chemotherapy were carried out by sented included Egypt, Kenya, Nigeria, South Africa, Uganda, Tanzania,
OCT (5). The chorioretinal scar after treatment (36) - flat bright high- Ethiopia, Morocco, Zimbabwe, Democratic Republic of the Congo,
reflective stripe replacing all retinal layers. Residual tumours (36) - Ghana, Mali, Sudan, Tunisia, Côte d’Ivoire, Republic of the Congo, Sene-
elevation, homogeneity of structure and thickening of tomographical gal, and Zambia. Thirty-nine percent involved authors from outside
scan, hyperreflectivity of tumor tissue with «shadow» effect, equal Africa. All articles identified solely through AJOL had entirely African
choroidal profile; on periphery - bright flat highreflective retina were authorship. All studies were primary research studies (81% observa-
diagnosed. There were no symptoms by Ret Cam and echography, but tional, 19% experimental). Experimental studies represented clinical
flat hyperreflective tumor tissue between inner and external retinal (55%), applied basic (36%), and implementation (9%) research, and
layers were revealed by OCT (9). more often involved investigators from outside Africa (55%) than did
Conclusions: The OCT allows to reveal changes in a retina and to diag- observational studies (35%).
nose «invisible» small primary retinoblastoma and continued tumor Conclusions: The results indicate a need for more support of African-
growth after treatment that is necessary for definition of further led experimental research to build the local evidence for retinoblas-
tactics of treatment. toma management. AJOL revealed African-led studies not included in
Western databases, suggesting a publication bias preventing dissemi-
nation of African science.
EP258 / #1738 RETINOBLASTOMA RESEARCH IN AFRICA: A
SCOPING REVIEW
EP259 / #1119 THE ROLE OF INSURANCE AND THE
Roxanne Noronha1 , Amal Rezk2 , Vivian Tam1 , Sadik Sherief3,4 , Vera MEDICAL CARE SYSTEM IN DIAGNOSTIC DELAY IN
Essuman5 , Sameh Soliman1,2,6 , Helen Dimaras1,2,7,8,9 RETINOBLASTOMA
1 The Hospital for Sick Children, Department Of Ophthalmology & Vision
Sciences, Toronto, Canada; 2 University of Toronto, Department Of Oph- Lily Bodinson1 , Luisa López De Mesa1 , Ambar Ruiz1 , Silvia Bhatt
thalmology & Vision Sciences, Toronto, Canada; 3 Addis Ababa University, Carreño1 , Lourdes Cabrera Muñoz2 , M. Veronica Ponce Castañeda3 ,
Department Of Ophthalmology, School Of Medicine, College Of Health Josefina Romero Rendon4 , Manuela Orjuela-Grimm5
Sciences, Addis Ababa, Ethiopia; 4 Menelik II Hospital, Department Of Oph- 1 Columbia university, Epidemiology, New York, United States of Amer-
thalmology, Addis Ababa, Ethiopia; 5 University of Ghana Medical School, ica; 2 Hospital Infantil de Mexico, Federico Gomez, Pathology, Ciudad de
Ophthalmology Unit, Department Of Surgery, Accra, Ghana; 6 University of Mexico, Mexico; 3 Centro Medico Nacional Siglo XXI, Insituto Mexicano de
Alexandria, Ophthalmology Department, Faculty Of Medicine, Alexandria, Seguro Social, Hospital de Pediatria, Infectious Diseases Medical Research
Egypt; 7 SickKids Research Institute, Child Health Evaluative Sciences Pro- Unit, Ciudad de Mexico, Mexico; 4 Centro Medico Nacional Siglo XXI, Insi-
gram, Toronto, Canada; 8 SickKids Research Institute, The Center For Global tuto Mexicano de Seguro Social, Hospital De Pediatria, Ciudad de Mexico,
Child Health, Toronto, Canada; 9 University of Toronto, Dalla Lana School Of Mexico; 5 Columbia University, Epidemiology, Department Of Pediatrics:
Public Health, Toronto, Canada Hematology, Oncology, And Stem Cell Transplantation, New York City,
United States of America
Background and Aims: Achieving equity in retinoblastoma care rep-
resents a global challenge. In Africa, where anticipated and reported Background and Aims: Diagnostic delay is considered to worsen clin-
mortality and morbidity of retinoblastoma is higher than in the rest of ical outcomes in retinoblastoma, though contributors to delay are
the world, the generation of quality local evidence is crucial to inform multifactorial and poorly understood. Our work in a public hospi-
clinical retinoblastoma guidelines. This scoping review aims to identify tal in Mexico showed delay impacts severity and survival in bilateral
the breadth and depth of retinoblastoma research conducted in Africa. (but not unilateral) disease. Other studies suggest healthcare access
Methods: A systematic search of Embase, Medline, Scopus, Web of Sci- may impact disease presentation but this has not been directly exam-
ence, and African Journals OnLine (AJOL) databases was conducted ined. We examined whether diagnostic delay differed between children
to identify peer-reviewed English-language publications published diagnosed in two adjacent children’s hospitals in Mexico City: one
between 1/January/2003 and 28/March/2022. Medical subject head- public (HIM) and one in the employer based social security system
ings ‘retinoblastoma’, ‘Africa’, and individual African country names (IMSS).
were used as search terms. Articles were included if they were Methods: Within the EpiRbMx study, consenting parents of 445 Mexi-
original research articles on retinoblastoma and conducted in any can children with retinoblastoma were interviewed at diagnosis about
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S268 of S635 ABSTRACTS

initial symptoms and household demographic characteristics. Diag- presents a bistability for some range of feedback strengths, relate with
nostic delay or lagtime was defined as the time (months) between the interactions.
parents noting symptoms and diagnosis. Treatment hospital, sociode- Conclusions: In conclusion, the model provides a way for controlling
mographic, and clinical factors were examined as predictors for lagtime the signaling pathways and the evolution of Retinoblastoma using the
in multivariable linear regression. bistability of the system, that can be using as alternative strategies for
Results: Clinical presentation, including disease laterality, age at diag- cancer therapy.
nosis, and presenting symptoms did not differ by hospital, however
mean lag time for HIM children(N=347) was 6.5 (range 0.0 to 66)
months, compared with 4.6 (0.0–24) months for IMSS children (N=96), EP261 / #1874 OPHTHALMIC ARTERY CHEMOSURGERY
p=0.006 . Lag time was associated with strabismus (p=.009) but not IMPROVES TOPOTECAN ORBITAL EXPOSURE COMPARED TO
other clinical presentations. Predictors of diagnostic delay differed SYSTEMIC INFUSION IN PIGS
between the two healthcare systems: paved streets and commuting
time predicted a nearly 2 fold increase in lagtime at the IMSS but were Claudia Sampor1 , Flavio Requejo2 , Javier Opezzo3 , Alan Vater4 ,
not strong predictors of lagtime in the HIM. Importantly the two hos- Marcelo Asprea5 , Eduardo Lagomarsino6 , Adriana Fandiño7 , Jasmine
pitals serve different populations as HIM and IMSS cases differed by Francis8 , David Abramson9 , Guillermo Chantada10 , Paula
socioeconomic factors and living conditions during pregnancy, includ- Schaiquevich4
ing household income, maternal education and presence of home toilet 1 Hospital JP Garrahan, Pediatric Oncology, Buenos Aires, Argentina;
(p<008, all). 2 Hospital JP Garrahan, Department Of Pediatric Interventional Neurora-
Conclusions: Our results show healthcare systems impact diag- diology, Buenos Aires, Argentina; 3 Universidad de Buenos Aires, Catedra
nostic delay. Additionally, sociodemographic predictors of lag De Farmacia Y Bioquimica, Buenos Aires, Argentina; 4 Hospital de Pedia-
time also differ by healthcare system, suggesting that strate- tria JP Garrahan, Precision Medicine, Buenos Aires, Argentina; 5 Hospital JP
gies to decrease delays in diagnosis need to be tailored to care Garrahan, Central Laboratory, Buenos Aires, Argentina; 6 Hospital JP Garra-
settings. han, Pharmacy, Buenos Aires, Argentina; 7 Hospital de Pediatría Garrahan,
Ophtalmology, Buenos Aires, Argentina; 8 Memorial Sloan Kettering Cancer
Center, Ophthalmic Oncology Service, New York, United States of Amer-
EP260 / #810 MATHEMATICAL MODEL FOR ica; 9 Memorial Sloan Kettering Cancer Cen, Ophthalmic Oncology Service,
MULTISTABILITY DETECTION IN RETINOBLASTOMA New York, United States of America; 10 Hospital Pereira Rossell, Pediatric
CONSIDERING GENETIC AND EPIGENETIC LANDSCAPE Oncology, Montevideo, Uruguay

Y. Melissa Romero Chaves1 , J. Roberto Romero-Arias2 Background and Aims: Orbital extension in retinoblastoma is fre-
1 UNAM-IIMAS, Mathematics And Mechanic, Cd. México, Mexico; 2 UNAM- quent in developing countries and is a major cause of death. Despite
IIMAS, Mathematics And Mechanic, Cd. Mexico, Mexico multimodal therapy with high-dose chemotherapy, enucleation, and
radiotherapy has improved life salvage, treatment is still associated
Background and Aims: Retinoblastoma is a childhood cancer of the with high toxicity and mortality. An alternative treatment strategy to
retina which has a striking specificity. Alterations on the molecular cir- improve clinical response would be to enhance chemotherapy delivery
cuitry for RB1 gene develop tumors in the eye in patients before the age to the orbit and minimize systemic exposure. We aimed to study and
of five. Tumorigenesis is the result of a complex interaction of intrin- compare the orbital exposure of chemotherapy after ophthalmic artery
sic and extrinsic processes of cells which promote genomic instability, chemosurgery (OAC) and intravenous (i.v) infusion in an animal model.
resistance to apoptosis, reprogramming and reorganization. Likewise, Methods: Three landrace pigs received topotecan (4mg) 30-minutes
the levels of gene expression, loss of function of suppressor genes, OAC and samples were serially obtained from a peripheral artery
microRNAs and non-coding RNAs in the cellular microenvironment as and from a microdialysis probe inserted into the lateral rectus of the
external factors are decisive to quantify the form in which a tumor infused eye as a surrogate of the orbital vascular irrigation. The ani-
grows and spreads. mal was recovered and after a wash-out period, the animals received
Methods: The number of pathways on which these alterations occur an i.v infusion of 4mg of topotecan; plasma and samples from the non-
are crucial to understand the origin and prognosis of the tumorigene- treated lateral rectus muscle were obtained and all were quantified by
sis. Thus, we propose a mathematical model for genetic and epigenetic HPLC.
landscape, in which the pathways between RB1 and E2F genes play Results: Median muscle exposure calculated as the area under the
a crucial role in the tumorigenesis. The model considers different concentration versus time profile for total topotecan attained after
interactions among the genes RB1, E2F, CDK2, P53, AKT and SYK, OAC was significantly higher than after i.v infusion (139906 ng*h/ml
microRNAs miR-373 and miR-34, and non-coding RNAs miR17∼92 (range: 116202-332938) and 758 ng*h/ml (range: 523-2616), respec-
and let-7 family, that regulate the cell cycle and the progression of RB. tively, p<0.05). The median (range) muscle-to-plasma exposure ratio
Results: Our model provides a quantitative analysis for the nega- was 37 (22-49) and 0.13 (0.11-0.51) after OAC and i.v, respectively.
tive feedback regulation RB1/E2F and demonstrates that the system The median (range) ratio between topotecan exposure attained after
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S269 of S635

OAC to i.v in the muscle was 222.3 (69.6-439.4) but systemic exposure Karel Svojgr1 , Radka Hobzova2 , Martina Kodetova3 , Pavel Pochop3 ,
remained comparable with a median (range) ratio for drug exposure in Jiri Uhlik4 , Katerina Dunovska5 , Martin Sima6 , Ondrej Slanar6 , Jan
plasma of 1.05 (0.95-1.20). Hrabeta1 , Barbara Feriancikova1 , Irina Cocarta2 , Jakub Sirc5
Conclusions: OAC resulted in significantly higher topotecan exposure 1 University Hospital Motol, Pediatric Hematology And Oncology, Praha,
in the lateral rectus muscle of the pig compared to that attained after i.v Czech Republic; 2 Academy of Sciences of the Czech Republic, Institute
while similar systemic exposure was attained after both routes of drug Of Macromolecular Chemistry, Prague, Czech Republic; 3 Charles Univer-
delivery. Patients with orbital retinoblastoma may benefit from OAC. sity and Motol University Hospital, Department Of Ophthalmology, Prague,
Czech Republic; 4 Charles University, 2nd Faculty of Medicine, Department
Of Histology And Embryology, Prague, Czech Republic; 5 Charles University
EP262 / #1265 CAN BONE MARROW EXAMINATION BE and Motol University Hospital, Department Of Medical Chemistry And Clin-
SKIPPED IN CHILDREN WITH LOW STAGE RETINOBLASTOMA? ical Biochemistry, Prague, Czech Republic; 6 Charles University and General
University Hospital in Prague, Department Of Pharmacology, Prague, Czech
Zunaira Shaukat, Afnan Munir, Haleema Saeed Republic
Shaukat Khanum Memorial cancer Hospital & Research Center, Lahore,
Paediatric Oncology, Lahore, Pakistan Background and Aims: Retinoblastoma (Rb) treatment with intra-
arterial and intra-vitreal application of topotecan and melphalan is
Background and Aims: To see the prevalence of bone marrow metas- widely used for therapy of Rb. The aim of our study was to develop a rel-
tases in children with retinoblastoma presenting to our hospital and if atively non-invasive system that delivers a cytotoxic drug by diffusion
bone marrow examinations be skipped in those with intra-ocular, low according to concentration gradient to a vitreous of an eye bulb.
group disease. Methods: Lens-shaped bilayered hydrogel implants loaded with
Methods: Retrospective review of 164 patients with retinoblastoma topotecan (TPT) were developed for trans-scleral diffusion of TPT into
registered at Shaukat Khanum Memorial Cancer Hospital from Jan- an eye bulb. The implants were tested on a rabbit animal model.
uary, 2017 to December, 2018. Data fields included demographics, Results: The implant is composed of inner poly(2-hydroxyethyl
initial presenting symptoms, duration of symptoms, laterality, local methacrylate) (pHEMA) layer – thickness 1.25±0.02mm - loaded with
group as per EUA, MRI findings, overall stage, metastatic sites, treat- TPT and outer poly(2-ethoxyethyl methacrylate) (pEOEMA) layer -
ment modalities used, disease and patient status at 1 and 2 years follow thickness 0.78±0.1mm - that TPT does not absorb and is impermeable
up. Quantitative variables were analyzed using descriptive statistics. for TPT; pEOEMA is a shield for surrounding tissues. pHEMA and
Results: A total of 164 patient charts were reviewed. Eight patients pEOEMA without TPT are non-toxic for tissues confirmed in-vitro on
could not complete their workup, so were excluded. Of 156 patients, cell lines and by chorioallantoic membrane assay. TPT-loaded pHEMA
male to female ratio was 1. Median age at presentation was 24 months. in-vitro is cytotoxic against Y79 Rb cell line (100% cytotoxicity until
Family history of retinoblastoma was found in 16% patients. Major ini- 6th round of repeated fresh cell culture). In-vitro release of TPT from
tial symptom was leucoria in 70% patients followed by squint in 11%. pHEMA has two-phases (50% of TPT is released in first 24hours, sub-
Median duration of symptoms was 3 months (range 1 week – 4 years). sequent in 13 days). In an animal rabbit in-vivo experiments, the lens-
Bilateral disease was found in 30% (46) children. Majority patients shaped TPT-pHEMA/pEOEMA implants were successfully implanted
presented with advanced disease. There were 42% (66) patients with to the posterior segment of the eye bulb with minimal macroscopic
group D eyes, 30% (46) with group E eyes and 10% (16) with localized and microscopic toxicity. To increase trans-scleral diffusion of TPT,
extraocular spread at presentation. Distant metastases were seen in transconjunctival cryotherapy was used. Median vitreous total TPT
9% (14) patients. Globe was salvaged in 33% (52) patients only while exposures (AUC) were 455.6 ng.h/ml, median plasma AUC were 50.3
66% (104) patients underwent enucleation at some point during their ng.h/mL, plasma exposure accounts 11-12% of vitreous exposure.
treatment. Of 156 patients reviewed, bone marrow involvement by Conclusions: The pilot study confirms the ability of the bilayered
retinoblastoma was seen in 3.8% (n=6). All these patients had either hydrogel implant to deliver TPT into the eye-bulb in an adequate con-
group E eyes or local extraocular spread. centration and minimal toxicity. Supported by MH CZ – RVO, UH
Conclusions: If findings of MRI and EUA are combined for retinoblas- Motol 00064203, GA UK 907019, AV21 Strategy (Czech Academy of
toma patients, children with group D eyes can be spared of performing Sciences)
routine bone marrow examination;saving money, time and avoiding
morbidity.
EP264 / #503 GERMLINE ALTERATIONS IN HEREDITARY
RETINOBLASTOMA SURVIVORS WITH SUBSEQUENT
EP263 / #867 HYDROGEL IMPLANTS FOR TRANS-SCLERAL MALIGNANT NEOPLASMS
DELIVERY OF TOPOTECAN INTO EYE BULB – PROOF OF
CONCEPT ON ANIMAL MODEL Gabriela Villanueva1 , Mercedes Paladino1 , Daiana Ganiewich1 ,
Claudia Sampor2 , Andrea Llera1,3 , Guillermo Chantada1,3
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S270 of S635 ABSTRACTS

1 Hospital Austral, Department Of Translational Pediatric Oncology, Pilar, Bruce Morland9 , Margarita Sala10 , Maria Rosa Sarrias1 , Carolina
Argentina; 2 Hospital JP Garrahan, Pediatric Oncology, Buenos Aires, Armengol1
Argentina; 3 National Scientific and Technical Research Council (CONICET), 1 Childhood Liver Oncology Group (c-LOG), Health Sciences Research Insti-
Oncology, Buenos Aires, Argentina tute Germans Trias i Pujol (IGTP), Childhood Liver Oncology Group,
Badalona, Spain; 2 Children’s Hospital Bambino Gesù, IRCSS, Pathology,
Background and Aims: Subsequent malignancies (SMNs) in hereditary ROME, Italy; 3 Hospital Vall d’Hebron, Pathology Department, Barcelona,
retinoblastoma (RB) survivors have been broadly documented in the Spain; 4 University Hospital La Paz, Pathology Department, Madrid, Spain;
literature. However, it is unclear if germline genetic alterations, other 5 Hospital Universitari Vall d Hebron, Pediatric Oncology, Barcelona, Spain;
than mutations in RB1, play a role in the development of SNMs in this 6 University Hospital La Paz, Pediatric Surgery Department, Madrid, Spain;
population 7 International Breast Cancer Study Group Coordinating Center, Oncology,
Methods: Whole exome sequencing (WES) was performed on DNA Bern, Switzerland; 8 Medical University of Gdansk, Department Of Surgery
extracted from peripheral blood from 5 RB survivors who developed And Urology For Children And Adolescents, Gdansk, Poland; 9 Birmingham
SMNs, using SureSelect Clinical ResearchExome v2 followed by next Children’s Hospital, Department Of Pediatric Oncology, Birmingham, United
generation sequencing. Variant calling and interpretation was done Kingdom; 10 Hospital Universitari de Girona Dr. Josep Trueta, Oncology,
using open-source tools (GATK Haplotype Caller, CPSR) and man- Girona, Spain
ual curation. Genetic profile was correlated with clinicopathological
characteristics, type of SMNs and treatment exposure. Background and Aims: The main pediatric liver tumors are hepato-
Results: We analyzed five hereditary RB survivors with history of a blastoma (HB) and pediatric hepatocellular carcinoma (HCC). Whilst
SMN (renal cell carcinoma, sebaceous cell carcinoma, osteosarcoma, HB appears at early ages, HCC is mainly diagnosed in adoles-
alveolar rhabdomyosarcoma and leiomyosarcoma). All subsequent cents and it is usually associated to underlying metabolic diseases.
neoplasms occurred in the irradiated field except for the renal cell HCN-NOS (Hepatocellular malignant neoplasm) is a recent entity
carcinoma. Aside from pathogenic variants in RB1 in all patients, we with histopathological features of HB and HCC. Due to the rar-
did not identify any other recurrent pathogenic germline gene vari- ity of the disease (<1 case/million children), the biology of HCC
ants. After an exhaustive search that included genes associated with and their similarity with HB has been poorly explored. To char-
DNA repair and cancer predisposition genes, we identified 10 shared acterize pediatric HCC at the genomic, transcriptomic and epige-
variants of unknown significance and one likely pathogenic variant in nomic level and to perform a comparative study with HB and HCN-
the gene ASAH2. None of them were apparently associated with the NOS.
SMN’s phenotype. In addition, a heterozygous nonsense variant in the Methods: We performed a genomic (SNP-array), epigenomic
XPC gene was detected in one patient that developed a sebaceous cell (EPIC/850k) and transcriptomic (HTA2.0array) studies on 8 HCC
carcinoma. and compared them with already published omics data of 31 HB, 2
Conclusions: We speculate that the loss of one functional allele of XPC HCN-NOS and 19 non-tumor liver (NT) samples (Carrillo-Reixach
in one patient could have affected the repair of the DNA damaged by et al, JHepatol, 2020). The results obtained were validated in an
radiotherapy exposure and might have played a role in the develop- independent series of 21 HCC, 56 HB, 7 HCN-NOS and 8 NTs using
ment of a SMN. Other than that, there is no sufficient evidence from QUAlu and Nanostring techniques for assessing DNA methylation and
this cohort to associate additional germline mutations to the devel- gene expression.
opment of SMNs. The exposure to carcinogenic treatments such as Results: We identified CTNNB1 gene mutations in 73%, 50% and
radiotherapy in a RB1-mutated genetic background may be the main 36% of HB, HCN-NOS and HCC, respectively. Promoter-TERT muta-
responsible for the development of SMNs. tions were found in HCN-NOS (22%) and HCC (8%) but not in
HB. All except one of the FL-HCC cases with frozen samples had
DNAJB1:PRKACA fusion-transcript; the only case without this fusion,
E-Poster Topic: AS05.j Liver Tumours had a deletion in the exon2 in PRKCA. The genome of HCC had higher
number of chromosomal losses than HB (p <0.0001). HCC was char-
E-POSTER VIEWING acterized by a CpG-island hypermethylation. Two different imprinting
regions at 14q32 (DLK1/DIO3) and 15q11.2 (SNORD115/116) were
EP265 / #422 COMPARATIVE MOLECULAR STUDY upregulated in HB and HCC. Finally, we identified SPINK1 gene
BETWEEN PEDIATRIC HEPATOCELLULAR CARCINOMA AND (Serine-peptidase-inhibitor, kazal-type-1) as overexpressed gene in
HEPATOBLASTOMA HCC that was strongly associated with prognosis of patients with
liver cancer in training (Log-Rank=0.008) and validation sets (Log-
Juan Carrillo-Reixach1 , Álvaro Del Río-Álvarez1 , Laura Royo1 , rank=0.012).
Montserrat Domingo-Sàbat1 , Rita Alaggio2 , Marta Garrido3 , Laura Conclusions: We identified molecular differences between
Guerra4 , Constantino Sabado Alvarez5 , Francisco Hernández6 , pediatric HCC and HB and a common prognostic
Manuel López-Santamaría6 , Rudolf Maibach7 , Piotr Czauderna8 , factor.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S271 of S635

EP266 / #1754 DELAYED PRESENTATION, ADVANCED AND 1 Dmitry Rogachev National Medical Research Center of Pediatric Hema-
METASTATIC DISEASE ARE ASSOCIATED WITH POOR tology, Oncology and Immunology, Department Of Clinical Oncology,
OUTCOMES IN PRIMARY PEDIATRIC LIVER Moscow, Russian Federation; 2 Dmitry Rogachev National Medical
TUMOR-HEPATOBLASTOMA- STUDY FROM TERTIARY CARE Research Center of Pediatric Hematology, Oncology and Immunology,
HOSPITAL IN PAKISTAN Department Of Pediatric Surgery, Moscow, Russian Federation; 3 B.V.

Petrovsky Russian Scientific Center of Surgery, Department Of Liver


Palwasha Rehman1 , Sophia Aslam1 , Najma Shaheen2 , Haleema Saeed1 Transplantation, Moscow, Russian Federation; 4 Moscow Oncological
1 Shaukat Khanum Memorial cancer Hospital and Research Centre, Pediatric Hospital №62, Division Of Surgery, Moscow, Russian Federation; 5 Russian
Oncology, Lahore, Pakistan; 2 Shaukat Khanum Memorial Hospital, Pediatric Children’s Clinical Hospital, Department Of Ultrasound Diagnostics,
Oncology, lahore, Pakistan Moscow, Russian Federation; 6 Dmitry Rogachev National Medical Research
Center of Pediatric Hematology, Oncology and Immunology, Depart-
Background and Aims: Primary hepatic tumors are rare entity; Hepa- ment Of Radiology, Moscow, Russian Federation; 7 Dmitry Rogachev
toblastoma is the most common childhood liver tumor. Over the years National Medical Research Center of Pediatric Hematology, Oncology
the overall survival of hepatoblastoma has increased from 35% to and Immunology, Department Of Anaesthesiology, Moscow, Russian
90%.Risk stratified treatment protocols have been proposed over the Federation; 8 N.N. Blokhin National Medical Research Center of Oncol-
years with the intention of reduced therapy for lower risk patients ogy, Institute Of Pediatric Oncology And Hematology, Moscow, Russian
compared to intensified treatment for higher risk patients. In our Federation
study we aim to determine the risk factors in children diagnosed with
hepatoblastoma and its effects on outcomes. Background and Aims: Risk-adapted therapy is the stan-
Methods: A retrospective study from June 2007 till January 2021 dard of care for hepatoblastoma (HB). The aim of this study
was carried out at Shaukat Khanum Memorial Cancer Hospital and was to analyses the efficacy of cisplatin monotherapy in
Research Centre-Pakistan. a large population of Russian patients with standard-risk
Results: Total 56 patients diagnosed with hepatoblastoma presented HB.
with mean age of 26.2 months (SD ± 21.1 months). Out of them Methods: For the period 02.2012 - 12.2019 (95 months) 60 patients
42 (75%) were males whereas 14 (25%) were females. The common with standard-risk HB were treated within the framework of the
presenting feature was abdominal distension present in 23 patients cooperation of two large volume centers. The SIOPEL criteria were
(41.1%). High risk disease was most seen in our cohort, present in 28 used for stratification into risk groups. Throughout the study period,
patients (50%) followed by standard risk disease present in 15 patients standard-risk patients received therapy according to the SIOPEL-
(26.8%). Surgical resection was offered to only 53.8% patients whereas 3 SR protocol, including cisplatin monotherapy (Perilongo G, 2009).
in 32.1% disease was unresectable. At the end of treatment, complete Survival was assessed by the Kaplan-Meier method. For the pur-
remission was seen among 17 patients (30.4%), disease progression poses of this study, overall survival (OS), event-free survival (EFS),
in 11 patients (19.6%). Death as first event was seen in 16 patients where any change in chemotherapy regimen towards its escalation
(28.6%). Patients with advanced disease had poor prognosis (p value were considered as an additional event, and progression-free survival
0.002). Significant number of patients (17.9%) with high risk and very- (PFS) were calculated. The survival analysis was carried out on 15.01.
high risk disease abandoned the therapy. The 4-year overall survival 2021.
seen was 48.5% whereas event free survival was 30%. Results: 54/60 (90%) patients were treated with cisplatin monother-
Conclusions: Advanced disease is commonly seen in low-middle apy and included in the final analysis. Median age at diagnosis was
income countries due to delayed presentation resulting in poor out- 11.3 months (range 0.0-87.7). Male:female ratio - 0.86:1. Distribu-
comes, although free treatment is offered there is still high number tion by PRETEXT stages: I - 14 (25.9%), II - 30 (55.6%), III - 10
of abandonment seen among our patients. Nationwide campaign is (18.5%) patients. The median alpha-fetoprotein level at the time of
needed to increase awareness regarding early detection of hepatoblas- diagnosis was 162979 ng/ml (range 129 - 2000000). Modification
toma. of therapy was required in 3/54 patients. Median follow-up was
47.1 months (range 2–99). Among 54 patients 52 (96.3%) are alive,
2 (3.7%) patients died (1/2 - complications of surgical treatment).
EP267 / #351 STANDARD-RISK HEPATOBLASTOMA: RESULTS Relapses/progressions were noted in 4/54 (7.4%) patients, one of
OF CISPLATIN MONOTHERAPY IN THE EXTENDED GROUP OF whom died due to disease progression. 3-year OS was 98.1% (95% CI
RUSSIAN PATIENTS 94.6-100), EFS - 85.1% (95% CI 75.5-94.6), PFS - 90.5% (95% CI 82.5-
98.4).
Roman Moiseenko1 , Dmitry Akhaladze2 , Andrey Filin3 , Eduard Kim4 , Conclusions: Our data are consistent with the original studies of the
Gavriil Rabaev2 , Elena Feoktistova5 , Alexey Metelin3 , Galina SIOPEL group and convincingly confirm the effectiveness of cisplatin
Tereschenko6 , Nikolay Merkulov2 , Vladislav Shchukin7 , Tatyana monotherapy in patients with HB of the standard risk group in the
Shamanskaya1 , Svetlana Varfolomeeva8 , Denis Kachanov1 Russian Federation.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S272 of S635 ABSTRACTS

EP268 / #580 HEPATOBLASTOMA: SERVICES AVAILABLE E-Poster Topic: AS05 SIOP Scientific programme / AS05.k Germ Cell
ACROSS PEDIATRIC ONCOLOGY EAST & MEDITERRANEAN Tumours
(POEM GROUP) COUNTRIES
E-POSTER VIEWING
Lama Dakik1 , Safal Muhammed M K2 , Anas Obeid3 , Raya Saab4 , Amita
Trehan2 EP269 / #301 GUIDELINE-CONGRUENT CARE IS
1 American University of Beirut, Faculty Of Medicine, Beirut, Lebanon; ASSOCIATED WITH INCREASED SURVIVAL AMONG
2 Advanced Pediatrics Center, Postgraduate Institute of Medical Educa- ADOLECENTS AND YOUNG ADULTS WITH PRIMARY
tion and Research, Pediatric Hematology Oncology Unit, Chandigarh, MEDIASTINAL GERM CELL TUMORS: A POPULATION-BASE
India; 3 American University of Beirut Medical Center, Depart- STUDY
ment of Pediatrics, Children’s Cancer Center Of Lebanon, Beirut,
Lebanon; 4 American University of Beirut Medical Center, Depart- Renata Abrahao1 , Qian Li1 , Frances Maguire2 , Marcio Malogolowkin3 ,
ment Of Pediatrics, Children’s Cancer Center Of Lebanon, Beirut, A. Lindsay Frazier4 , Theresa Keegan1 , Elysia Alvarez5
Lebanon 1 Center for Oncology Hematology Outcomes Research and Training
(COHORT), Division of Hematology and Oncology, University of California
Background and Aims: Access to complete care in oncology is Davis, Internal Medicine, Sacramento, United States of America; 2 California
essential for good outcomes. This analysis evaluated the chal- Cancer Reporting and Epidemiologic Surveillance Program, University of
lenges in management of hepatoblastoma in the POEM group California Davis Comprehensive Cancer Center, Internal Medicine, d, United
countries. States of America; 3 University of California Davis Comprehensive Can-
Methods: A Microsoft survey was sent three times cer Center, Pediatrics, d, United States of America; 4 Dana-Farber Cancer
to 96 centers in 25 countries, and responses were Institute, Pediatric Oncology, d, United States of America; 5 University of
analyzed. California Davis, Pediatrics, Sacramento, United States Minor Outlying
Results: Twenty-nine respondents from 27 institutions across 15 Islands
countries participated. Fourteen centers (48%) diagnosed ≤ 3 hep-
atoblastoma cases /year, while 7 centers (24%) diagnosed 15-30 Background and Aims: Most prognostic factors and outcomes for
patients /year. Alfa-fetoprotein testing was available at all centers, primary mediastinal germ cell tumors (PMGCT) in adolescents and
turnaround time being ≤3 days in 22 (76%). Imaging modalities young adults (AYAs, 15–39 years) are obtained from single institutions
(CT and/or MRI) were available in all, and 28/29 centers employed or clinical trials with limited sample size. We aimed to assess, at the
the PRETEXT staging. Fourteen centers (48%) performed a diagnos- population-level, the impact of the delivery of guideline-congruent care
tic biopsy in >90% of cases, while 10 (34%) reported a biopsy in (GCC), treating physician specialty, and location of care on survival
≤50%. Thirteen centers (45%) estimated ≤25 % cases and 5 (18%) among AYAs with PMGCT.
reported 50-75% present with metastatic disease. Twenty-one cen- Methods: We used data from the California Cancer Registry (CCR) to
ters had in-house surgery, 9 reported availabilities in the same city, ascertain all AYAs diagnosed with a PMGCT from 2004 to 2018, and
and 1 in multiple locations. Surgery was performed by pediatric to identify detailed treatment information from the CCR text-fields.
surgeons in 18 centers, onco-surgeons in 9, and general surgeons GCC, defined based on the National Comprehensive Cancer Network
in 4. Four centers (14%) had access to liver transplant. Only 35% and Children Oncology Group (COG) guidelines, includes chemother-
reported >90% of surgeries occurring on time, with lack of the- apy alone (BEP: bleomycin, etoposide and cisplatin) or chemotherapy
atre or need for transplant being the main reasons for delay. Ten plus surgery. Multivariable Cox proportional regression was used to
centers (34%) reported that >90% of surgeries resulted in com- examine the associations between all-cause survival and GCC, location
plete excision with negative margins, whereas 11/29 (38%) reported of care (COG/NCI-Designated Cancer Center versus other centers)
≤75% of surgeries resulting in negative margins. One fifth of centers and treating physician specialty, adjusting for sociodemographic and
reported requiring a second opinion for histopathology. Neoadjuvant clinical factors. Results are presented as hazard ratios (HR) and
chemotherapy was used by all. One third of respondents reported corresponding 95% confidence intervals (CI).
a delay in therapy in >90% patients, due to cytopenia or lack of Results: Of 300 patients, 56% were of Hispanic race/ethnicity, 42%
beds. had late-stage disease (III/IV), 55% were treated by adult Hematolo-
Conclusions: This analysis provides an overview of the limitations in gists/Oncologists, 27% received all their care at COG/NCI-designated
multidisciplinary treatment of hepatoblastoma in the POEM countries. cancer centers, and 50% received GCC. The most common histology
Results will be used to enhance network collaborations to improve type was seminoma (40%). At five years after diagnosis, 189 (63%)
care, and to identify barriers for surgical availability and timeliness of patients were alive. AYAs with non-seminomas and late-stage disease
care. were about 3-times more likely to die than those with seminoma
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S273 of S635

tumors or earlier (I/II) stage disease (HR=3.14, CI 1.88–5.24 and HR= Conclusions: GCTs present in children with heterogeneous histology
2.60, CI 1.72–3.93, respectively). GCC (versus non-GCC) was associ- and localization. Metastatic character is associated with increased
ated with improved survival (HR=0.66, CI 0.44–0.99). We found no disease mortality and is more common in patients with extragonadal-
differences in survival by treating physician specialty, location of care extracranial localization. Beyond surgery, allocation of watchful wait-
or sociodemographic factors. ing and chemoradiation as well as chemo intensification in selected
Conclusions: GCC was independently associated with improved sur- patients is determinant for outcomes.
vival, underscoring the importance of following clinical practice guide-
lines when caring for the high-risk population of AYAs with PMGCT.
EP271 / #294 RETROSPECTIVE STUDY ON STAGE IV
EXTRACRANIAL GERM CELL TUMORS IN JAPAN: A REPORT
EP270 / #1731 GERM CELL TUMORS: INTRACRANIAL FROM THE GERM CELL TUMOR COMMITTEE IN JAPAN
GONADAL, AND EXTRACRANIAL-EXTRAGONADAL CHILDREN’S CANCER STUDY GROUP

Kondylia Antoniadi1 , Vasiliki Tzotzola2 , Vassilios Papadakis1 , Yuki Arakawa1 , Yuya Sato2 , Shuichiro Uehara3 , Hiroko Sato4 , Reina
Charikleia Kelaidi1 , Mirella Ampatzidou1 , Sofia Papargyri1 , Kalliopi Hoshi3 , Tsuyoshi Imai5 , Shigeki Yagyu6 , Isamu Saeki7 , Dai Keino8 ,
Stefanaki3 , Sophia Polychronopoulou1 Motonari Nomura9 , Mai Watakabe1 , Hiroaki Ono10 , Yoshiyuki
1 “Aghia Sophia” Children’s Hospital, Department Of Pediatric Hematology- Kosaka11 , Keita Terashia12 , Yoshiaki Kinoshita13 , Tatsuo Kuroda14
oncology, Athens, Greece; 2 Aghia Sophia Children Hospital, Department Of 1 Saitama Children’s Medical Center, Department Of Hematology / Oncol-
Pediatric Hematology-oncology, Athens, Greece; 3 “Aghia Sophia” Children’s ogy, Saitama, Japan; 2 Dokkyo Medical University, Department Of Pediatrics,
Hospital, Pathology Department, Athens, Greece Tochigi, Japan; 3 Nihon University School of Medicine, Department Of
Pediatric Surgery, Tokyo, Japan; 4 Yamagata University, Department Of Pedi-
Background and Aims: To present the long-term experience and out- atrics, Yamagata, Japan; 5 Shikoku Medical Center for Children and Adults
come of patients with Germ cell tumors(GCTs) of a single Pediatric Ehime, Japan, Department Of Pediatric Hematology/oncology, Kagawa,
Hematology-Oncology center. Japan; 6 Kyoto Prefectual University of Medicine, Department Of Pediatrics,
Methods: We analyzed 83 patients with GCTs(34/83 boys) with Kyoto, Japan; 7 Hiroshima University Hospital, Department Of Pediatric
median age 8.2 years(range, 6 days-16 years) diagnosed and treated in Surgery, Hiroshima, Japan; 8 Kanagawa Children’s Medical Center, Depart-
our Department from 1977 to 2020. ment Of Hematology/oncology, Kanagawa, Japan; 9 Osaka University Hos-
Results: GCT were gonadal in 58/83, intracranial 7/83, extracranial- pital, Department Of Pediatric Surgery, Osaka, Japan; 10 Kyushu University
extragonadal 16/83 and 2/83 unspecified.Of 58 gonadal cases, 57/58 Hospital, Department Of Pediatrics, Fukuoka, Japan; 11 Hyogo Prefec-
were GCTs and 1/58 gonadoblastoma, 24 mature teratomas, 17 imma- tural Kobe Children’s Hospital, Department Of Hematology And Oncology,
ture teratomas, 7 yolk sac tumors, 4 dysgerminomas, 2 embryonal Hyogo, Japan; 12 National Center for Child Health and Development, Chil-
carcinomas and 3 mixed GCT tumors.One patient developed tes- dren’s Cancer Center, Tokyo, Japan; 13 Niigata University, Department Of
ticular embryonal carcinoma as a second malignancy after Burkitt Pediatric Surgery, Niigata, Japan; 14 Keio University School of Medicine,
lymphoma. 56/58 patients underwent total surgical excision and 16/58 Department Of Pediatric Surgery, Tokyo, Japan
received platinum-etoposide based chemotherapy.Disease-free and
overall survival were 88% and 100% in patients with primary testic- Background and Aims: While stage IV germ cell tumors have shown
ular disease and 90% and 94% respectively, in patients with ovarian particularly poor prognosis, little consolidated information has been
cancer.Two patients with metastatic ovarian tumor histology died due available given its rarity. Therefore, the Germ Cell Tumor Commit-
to disease progression. Seven patients presented with intracranial tee of the Japan Children’s Cancer Study Group (JCCG) planned a
GCT;3 immature teratomas(1 with yolk sac component), 4 germi- retrospective survey to understand the actual situation in Japan.
nomas. Three patients underwent complete surgical excision and Methods: A retrospective survey from January 1, 2000 to December
four biopsy only.All patients underwent platinum-based chemother- 31, 2019 among patients under 20 years of age upon diagnosis was
apy, 1/7 received 2 autologous transplantations for teratoma, 4/7 planned to ascertain the actual status of stage IV germ cell tumors
additional radiotherapy. Two patients relapsed, and one died. Six- based on the classification of the Children Oncology Group staging in
teen patients presented with extracranial-extragonadal GCT localized Japan.
in:abdomen N=5, mediastinum N=3, sacrococcygeal region N=6, heart Results: Data were obtained from 50 cases across 31 institutes, among
N=1, vagina N=1.Histologically:8 mature teratoma, 1 immature ter- whom 30 and 20 were males and females, respectively. The median
atoma, 6 patients had yolk sac tumor, and 1 yolk sac/immature age at diagnosis was 4.0 years (0.6–18.8), with 19 cases at the age
teratoma. Three patinets were metastatic(lungs/liver/vagina). Four- of ≥11 years and 31 cases at the age of <11 years. As for surgical
teen patients underwent complete surgical excision, and three biopsy treatment, 36 patients underwent only biopsy upon initial diagnosis,
only. Six patients received platinum-etoposide based chemotherapy. eight patients underwent aggressive resection, and six patients did not
One patient died due to disease, and one patient developed a second undergo surgery, including biopsy, and received chemotherapy based
malignancy. on tumor markers alone. The most common pathological diagnosis was
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S274 of S635 ABSTRACTS

yolk sac tumor with 32 cases. All patients received chemotherapy, and Conclusions: Our data shows satisfactory outcomes in pediatric GCT in
40 underwent second-look surgery, including five of the six patients LMIC setting with standard therapy, though longer follow-up is needed
who did not undergo biopsy prior to chemotherapy. The 5-year over- to determine final survival and late-effects of treatment. Measures to
all survival was 64.3% (48.8%–76.2%), whereas the 5-year event-free curb high TRA rate of 8.6% can further improve outcomes.
survival was 63.4% (47.2%–75.8%). Patients younger and older than 11
years had a 5-year overall survival of 77.6% (56.4%–89.4%) and 42.1%
(20.4%–62.5%), respectively, with only the age of ≥11 years being an EP273 / #1756 ANALYSIS OF SURVIVAL AND LATE EFFECTS
independent prognostic factor (p = 0.003). OF PATIENTS TREATED FOR CHILDHOOD VAGINAL
Conclusions: In pediatric patients with extracranial germ cell tumors, MALIGNANT GERM CELL TUMOR
the age of ≥11 years was an independent poor prognostic fac-
tor. Age should be considered separately to select the optimal Robin Coppin1 , Hélène Martelli2 , Cyrus Chargari3 , Helene
treatment. Sudour-Bonnange4 , Daniel Orbach5 , Cécile Faure Conter6 , Cécilé
Vérité7 , Marie Nolla8 , Laure Saumet9 , Christophe Piguet10 , Florent
Guerin2 , Catherine Patte1 , Brice Fresneau1
EP272 / #1549 EPIDEMIOLOGY & OUTCOMES OF 1 Gustave Roussy, Department Of Pediatric Oncology, Université Paris-
PEDIATRIC EXTRACRANIAL GERM CELL TUMOR FROM A saclay, VILLEJUIF, France; 2 AP-HP Paris-Saclay, Paediatric Surgery, Le
TERTIARY CARE HOSPITAL IN NORTH INDIA Kremlin Bicêtre, France; 3 GUSTAVE ROUSSY, Department Of Radiation
Therapy, VILLEJUIF, France; 4 Centre Oscar Lambret, Pediatry, Lille, France;
Nidhi Dhariwal1 , Soumitra Saha2 , Vikramjit Kanwar1 , Shyam 5 Institut Curie, Siredo Oncology Department, Paris, France; 6 IHOPE,

Srinivasan3 Pediatry, bron, France; 7 CHU de Bordeaux, Oncologie Et Hematologie


1 Homi Bhabha Cancer Hospital, Pediatric Oncology, Varanasi, India; 2 Homi Pédiatrique, Bordeaux, France; 8 CHU TOULOUSE, Pediatric Oncology,
Bhabha Cancer Hospital, Surgical Oncology, Varanasi, India; 3 Tata Memorial TOULOUSE, France; 9 CHU MONTPELLIER, Pediatric Oncology, MONTPEL-
Hospital, Pediatric Oncology, Mumbai, India LIER, France; 10 CHU LIMOGES, Pediatric Oncology, LIMOGES, France

Background and Aims: Germ cell tumors(GCT) constitute only 3.5% of Background and Aims: Vaginal malignant germ cell tumors (GCT)
childhood malignancies but have excellent outcomes with surgery and are rare GCTs occuring in children <2years-old. The question of
chemotherapy as mainstay of treatment. We share our experience with local treatment is crucial to control disease but can lead to late
pediatric extracranial GCT in a Lower-Middle Income Country(LMIC). effects.
Methods: Electronic medical record of children upto 15 years of age Methods: We included children treated for vaginal GCT in French
registered in our unit from June 1st 2018 through October 31st 2021 TGM95 and TGM2013 studies or referred to Gustave Roussy in
and diagnosed with extracranial GCT was reviewed. Data pertaining first-line during the inter-study period. Patients were classified
to clinical characteristics, tumor markers, imaging, management and as standard-risk (SR: localized disease and AFP<10000ng/ml) or
follow-up was retrieved. Cases were risk stratified as per COG criteria high-risk (HiR: metastatic and/or AFP>10000ng/ml) and were
into low risk(LR), intermediate risk(IR) and high risk(HR) groups. treated with 3-5 VBP (vinblastine-bleomycine-cisplatin) or 4-6
Results: Fifty-seven patients with extracranial GCT were registered VIP (etoposide-ifosfamide-cisplatin) courses, respectively. Con-
during the study period of which 46 patients with de novo GCT servative surgery (partial vaginectomy) and/or brachytherapy
were analysed (11 operated patients outside with presenting with were used for local treatment in cases of post-chemotherapy
recurrence were excluded) with median age 5.5 years(Range-0.5-15 residue.
years) and male:female ratio 0.64:1. Two-third cases were gonadal Results: Fourteen patients were included (median age =12
GCTs (63.04%; 21-Ovarian, 8-Testicular) while 36.95% were extrag- months, range=6-20). Six of them (43%) were classified as HiR
onadal (8-sacrococcygeal, 3-gastric, 2-mediastinal, 2-retroperitoneal, (AFP>10000ng/ml: n=4; lung metastases: n=2). After first-line
1-parotid and 1-gluteal). Thirty-five(76.08%) patients had malignant chemotherapy, one patient (SR-group) did not achieve AFP normal-
GCT(MGCT) with 24 yolk sac tumors, 9 mixed GCT, 1 dysgerminoma ization, leading to secondary VIP, whereas both metastatic patients
and 1 choriocarcinoma, while 11(23.91%) had mature/immature ter- had lung metastases cleared by chemotherapy. A vaginal post-
atoma. With a median follow-up of 15 months, the 1-year event free chemotherapy residue (median size=8mm, range=1-24) was observed
survival(EFS) and overall survival(OS) for MGCT was 76.4% and 89% in 13/14 patients (8SR/5HiR), treated by complete resection in 9/13
respectively. Ten events seen in our cohort included 4 relapses, 4 (6SR/3HiR) with viable malignant cells in 3/9 (2SR/1HiR), incom-
treatment refusal/abandonment(TRA) and 2 deaths. For LR, IR and plete microscopic resection with viable malignant cells completed
HR groups, the 1-year EFS was 100%, 83.7% and 62.5%(p=0.28) with by brachytherapy in 2/13 (2SR), and by brachytherapy only in 2/13
1-year OS being 100%, 93.7% and 80.2%(p=0.24) respectively. On (2HiR, including 1 with residual viable cells on post-chemotherapy
univariate and multivariate analysis, age, baseline AFP, risk group, biopsy). Among the 5 patients with viable disease (4SR/1HiR), 4
chemotherapy backbone (PEb vs JEb) and degree of resection did not patients (3SR/1HiR) received 2 adjuvant chemotherapy courses (VIP
significantly impact survival of MGCTs. or Bleomycin-Carboplatin-Doxorubicin). One patient relapsed (SR)
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S275 of S635

just after surgery (no residual cells) and was treated with 4 VIP and the other one mixed GCTs, comprising 60% choriocarcinoma and 40%
brachytherapy. At last news (median follow-up=4.6years, range= dysgerminoma.
0.5-16), all patients remained in complete remission. Regarding long- Conclusions: MTOR, KIT, ATM, KRAS, PIK3CA alterations are con-
term toxicity, 5 patients had vaginal sequelae with synechiae and/or sistently described, suggesting that these are the main driver genes
stenosis (including 4 after brachytherapy). of pediatric GCTs. Further cohort studies are needed to elucidate
Conclusions: Childhood vaginal GCTs carry very favorable progno- these findings and to improve clinical management, leading to better
sis with risk-adapted chemotherapy and local treatment, to remove therapeutic alternatives.
post-chemotherapy residue, preferably with conservative surgery to
minimize local late effects. Adjuvant chemotherapy remains to evalu-
ate. EP275 / #1954 THE IMPACT OF PEI (CISPLATININ,
ETOPOSIDE AND IFOSFAMIDE) USED FOR CHILDHOOD HR
(HIGH RISK STAGE IV) GERM CELL TUMOR. THE BRAZILIAN
EP274 / #788 PRESENCE OF SOMATIC MUTATIONS WITHIN STUDY GROUP EXPERIENCE
MTOR, KIT, ATM, KRAS AND PIK3CA IN PEDIATRIC PATIENTS
WITH GERM CELL TUMORS Luiz Fernando Lopes1 , Carla Macedo2 , Ana Glenda Santarosa Vieira3 ,
Marcelo Miloni4 , Thaíssa Faria5 , Flavia Watusi6 , Gisele Martins3 ,
Janaina Galvão1 , Ana Glenda Santarosa Vieira2 , André Lengert1 , Rui Neysimelia Villela7
Reis1 , Luiz Fernando Lopes3 , Adriane Evangelista1 , Mariana Pinto4 1 Barretos Children’s Cancer Hospital, Pediatric Oncolgy, BARRETOS, Brazil;

1 Barretos Cancer Hospital, Molecular Oncology Research Center, Bar- 2 Universidade Federal de São Paulo, Instituto De Oncologia Pediatrica-
retos, Brazil; 2 Barretos Children’s Cancer Hospital, Pediatric Oncology, graacc, São Paulo, Brazil; 3 Barretos Children’s Cancer Hospital, Pediatric
BARRETOS, Brazil; 3 Barretos Children’s Cancer Hospital, Pediatric Oncology, BARRETOS, Brazil; 4 GACC - Grupo de Assistência à Criança com
Hospital, Bairro Paulo Prata, Barretos, San Paulo, Brazil; 4 Barretos Câncer, Pediatric Oncology, São José dos Campos, Brazil; 5 Barretos Chil-
Cancer Hospital, Molecular Oncology Research Center, BARRETOS, dren’s Cancer Hospital, Data Manager, BARRETOS, Brazil; 6 Hospital Jose
Brazil de Alencar, Ped Oncology, Brasilia, Brazil; 7 Hospital Infantojuvenil Barretos,
Bone Marrow Transplantation, Barretos, Brazil
Background and Aims: Background and aims Germ cell tumors
(GCTs) are a rare group of neoplasms that affect about 3.5% of Background and Aims: The Brazilian Group started January 2009 the
pediatric patients. GCTs are benign or malignant neoplasms occur- 2nd protocol and January 2008 the 3rd ; both have used PEI for HR
ring in gonadal and extragonadal sites. Unlike to many other cancers, group. Good Rosponders received 4 PEI (99 protocol) and 5 PEI (2008
GCTs have a relatively low mutational burden including embry- protocol) (same total dose for etoposide and ifosfamide and platinum
onic epigenetic state, copy number alterations (CNAs), and sin- changed from 420 mg/m2 to 600 mg/m2 ).The poor responders have
gle nucleotide variant (SNV) or somatic insertion/deletion. More- received 2 complementary cycle of PEI (GCT-99 ) or new agents 3 PEI
over, there is a lack of information about molecular alterations in + 3 TIP (GCT-2008).
pediatric GCTs. Therefore, the aim of this study was to perform Methods: From 163 S IV eligible pts, 98 were treated with GCT-99
a genomic profile by whole-exome sequencing (WES) of pediatric and 65 with GCT-2008. Testicular 69, Ovary 42, Sacrococcygeal 27,
GCTs. Mediastinal 11, retroperitoneum 8 and others 6; from 99 prot 60 pts
Methods: We performed the WES using Illumina paired-end sequenc- received 4 cycles PEI, 38/6 cy PEI, from 2008 prot 50/5 cy PEI and
ing strategy of 16 cases and respective matched normal, including 15/3PEI+3TIP, 64 YST, 5 IT, 7 Germinomas, 20 other malignant, 67
ten ovarian, five testicular, and one mediastinal. Data analysis was mixed tumors. Metastasis were 21 liver alone, 53 lung alone, 8 lymph
performed as follows: Mutect, Pindel, and Mutsig for SNVs/Indels; node, 81 combination( including bone, brain).According to Age 70 <11
HMMcopy, Nexus Copy Number and Gistic for CNAs; and Signal years, 34 (11-14 y) and 59 >15 y. Relapsed 32 pts (11 liver/and/or lung,
(Cosmic v3) for mutational signatures. 11 primary site, 4 lymp node, 6 others). BMT were performed for 20 pts
Results: The analyses of Single Base Substitution Signatures showed (6 refractory/or in progression, 12 after relapsed and 2 as consolidation
SBS39 and SBS22 in 68.75% and 12.5% of samples, respectively. 1st remission).
Among the common genes involved in GCTs pathogenesis, we found Results: 10 yearsOS for the 163 pts was 72.0%; age 0-11 ys the
CNAs on chromosomes 4, 7, 8, 10, 12, 21, 22, with gain of KRAS, 10y OS-75.1, 11-14y, 73.4% and >15y 67.4% (ns). Testicular 10y OS
CCND2, CDKN1B, ETV6, KDM5A, RAD52, RECQL, PIK3C2G, CRKL 73.4%, ovary 78.4, sacroc 77.6% and others 52.8 % (ns); prot 99-
genes, and loss of KIT and PTEN genes. Somatic mutations of NYAP2 4 PEI 10yOS was 69.7%, 6 PEI 75.7%, Prot 2008 5 PEI 77.5% and
and RHBDF2 genes were identified in 25% of patients, in addition to 3 PEI+3TIP 59.3% (ns); metastasis at diag 10y OS for lung alone
MTOR (19%), KIT (12%), ATM (12%), KRAS (6%) and PIK3CA (6%) 75.4%, liver alone 71.4%, lymp node 75.0% and combination 70.0
genes. Two patients presented concomitant MTOR and KIT missense (ns). For the patients who received BMT the 5 and 10y OS was
mutations, both with ovarian tumor, which one was dysgerminoma and 50.0%.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S276 of S635 ABSTRACTS

Conclusions: HR group patients do well but this is applicable in the con- mation of the original GCT, a unique aspect of GCT, remains
text of further refinements in risk approaches to therapy. Challenges critical.
remain in our approaches to high risk disease

EP277 / #1150 EVALUATION OF TUMOR GROWTH OF


EP276 / #236 SECOND MALIGNANCIES IN CHILDREN, GERM CELL TUMORS IN XENOGRAFT MODEL
ADOLESCENTS, AND YOUNG ADULTS WITH EXTRACRANIAL
GERM CELL TUMORS: A REPORT FROM THE MALIGNANT Marcela Rosa, Janaina Galvão, Eduardo Cabral, André Lengert,
GERM CELL TUMOR INTERNATIONAL CONSORTIUM Mariana Pinto
Barretos Cancer Hospital, Molecular Oncology Research Center, Barretos,
Priya Mahajan1 , Mark Krailo2 , Negar Fallahazad2 , Brice Fresneau3 , Brazil
Sara Stoneham4 , A. Lindsay Frazier5
1 Baylor College of Medicine, Pediatric Oncology, Houston, United States of Background and Aims: Background and aims: Despite the success
America; 2 Children’s Oncology Group, Statistics, Monrovia, United States of with cisplatin treatment in germ cell tumors (GCTs), a small but signifi-
America; 3 Gustave Roussy, Department Of Pediatric Oncology, Université cant number of patients will relapse. Several studies have been carried
Paris-saclay, Villejuif, France; 4 University College London Hospitals, Pedi- out to overcome cisplatin resistance with the use of other chemother-
atrics, London, United Kingdom; 5 Dana-Farber Cancer Institute, Pediatric apeutic agents, in order to have a significant impact on the survival
Oncology, Boston, United States of America of these patients. An intensively used in vivo model for chemotherapy
tests are the xenograft models, which can be generated through the
Background and Aims: While men with testicular cancer have a tumor cell lines. Therefore, the development of in vivo models in GCTs
two-fold increase in second malignant neoplasms (SMNs), the risk of is extremely important. In this study we have established the xenograft
SMNs in children and adolescents with extracranial malignant germ cell model derived from GCTs cell lines.
tumors (GCTs) is not fully quantified. The Malignant Germ Cell Tumor Methods: For this propose, 1x106 of NTERA-2 and JEG-3 were diluted
International Consortium (MaGIC) dataset includes 1,798 patients in HBSS and matrigel (1/1). They were injected subcutaneously into
from 14 clinical trials from 5 countries. SMNs were identified using the the right flank of athymic male mouse (Mus musculus), and the ani-
MaGIC dataset. mals were observed and weighed weekly. Tumor volume was calculated
Methods: We analyzed the cumulative incidence of SMNs in chil- using the following formula: d x D2 x 0.5. Tumors were removed and
dren with GCT within the MaGIC dataset. Patients managed with stained with hematoxylin and eosin (H&E) and analyzed by pathology
chemotherapy within trials which reported the date and diagnosis of department.
SMN, were analyzed. Results: NTERA-2 tumors started to grow on the 22nd day after inocu-
Results: A total of 917 patients from seven GCT clinical trials lation. The time from the beginning of growth to tumor excision ranged
were included (INT-0097 [n=300], INT-0106 [n=121], P9749 [n=26], from 23 to 40 days. The volume of tumors ranged from 1,871 to 2,476
AGCT01P1 [n=19], AGCT0132 [n=211], TGM95 [n=179], GCIII mm3 . JEG-3 tumors started to grow between the 11th and 20th day
[n=61]). Six hundred and three patients were female (65.8%). Pri- after inoculation. With the exception of one animal, whose growth was
mary tumor site included ovary (38.4%), testes (21.2%), sacrum/coccyx extremely rapid, the time from the beginning of growth to tumor exci-
(21.0%), mediastinum (8.5%), retroperitoneum (5.8%), and other sion ranged from 5 to 9 days. In animals, the tumors had a purplish color
(5.1%). Metastases at diagnosis were noted in 231 patients (25.2%). and a hemorrhagic appearance. The volume of removed tumors ranged
The median follow-up was 66 months. SMNs were identified in 11 from 990 to 4,694 mm3 .
patients with a median time to SMN of 17 months (range 3–94 Conclusions: NTERA-2 and JEG-3 cell lines were able to form tumors
months). SMNs included acute myeloid leukemia/myelodysplastic syn- in mice model, which could be useful not only in basic research but also
drome (MDS) in 6 patients; solid tumors in 6 patients. At least 3 of in the clinical setting.
the SMNs likely represent malignant transformations of teratoma. One
patient had 2 SMNs (MDS and squamous cell carcinoma). The 5-year
cumulative incidence of SMNs was calculated according to coopera- EP278 / #1557 PEDIATRIC TERATOMAS: A REVIEW OF THE
tive group (Children’s Oncology Group (1.1%), France (0.6%), United BRAZILIAN COHORT
Kingdom (0%); p-value=0.61), patient sex (female (0.7%), male (1.3%);
p-value=0.51), tumor site (ovary (0.9%), testes (1.0%), other (0.8%); Ana Glenda Santarosa Vieira1 , Gisele Martins1 , Thaíssa Faria2 , Carla
p-value=0.90), and metastases at diagnosis (no (0.8%), yes (1.3%); Macedo3 , Marcelo Miloni4 , Luiz Fernando Lopes5
p-value=0.49). 1 Barretos Children’s Cancer Hospital, Pediatric Oncology, BARRETOS,
Conclusions: The risk for SMNs is not significantly elevated in Brazil; 2 Barretos Children’s Cancer Hospital, Data Manager, BARRETOS,
this cohort, however long-term follow up of children with GCTs Brazil; 3 Universidade Federal de São Paulo, Instituto De Oncologia
is warranted. Differentiating between treatment-related secondary Pediatrica-graacc, São Paulo, Brazil; 4 GACC - Grupo de Assistência à
malignancy, second malignancy, and somatic malignant transfor- Criança com Câncer, Pediatric Oncology, São José dos Campos, Brazil;
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S277 of S635

5 Barretos Children’s Cancer Hospital, Pediatric Hospital, Bairro Paulo Prata, included site, histopathology, stage, risk groups, baseline AFP, β-HCG
Barretos, San Paulo, Brazil levels, metastatic sites and chemotherapy regimen give n. Data analy-
sis involved quantitative analysis, mean and median calculations, event
Background and Aims: Teratomas are the most common histological free survival (EFS) and overall survival (OS) calculations using Kaplan
subtype of germ cell tumors (GCT) of childhood, classified as mature or Meier curves.
immature, may arise in the gonads or extragonadals. Mature teratomas Results: Sevent-five adolescent patients were recruited. Male: female
(MT) are benign cystic solid tumors and contain well-differentiated tis- ratio was 1:2. Alpha fetoprotein was raised in 56% (42) patients but
sues representative of all three germ cell layers. Immature teratomas level above 10,000 was seen in 21%(n=16) patients. Beta HCG was
(IT) contain neuroectodermal or blastematous tissue, classified by a raised in 21% (n=16). Eighty-one percent had gonadal primaries (n=61;
system introduced by Norris, modified by Gonzales ± Crussi. Brazil- 46 ovarian, 15 testicular). Advanced stage was seen in 80% while
ian patients with extracranial GCTs began to be included in a single 55% had high risk disease. Seminomatous GCTs were found in 1/3rd
database in 1991 and to date there are 4 therapy protocols. Children patients. Sixty-five patients received chemotherapy (JEB: 44%, BEP:
with mature teratoma are treated only with surgical resection, how- 49%, JEV: 3%, VeIP:3.1%). 5-year EFS and OS of whole cohort were
ever, children with immature teratoma with hematogenous metastasis 62% and 75% respectively. 5-year EFS and OS of those receiving car-
receive postoperative chemotherapy. boplatin vs cisplatin were 80% vs 53% (p= 0.01) and 86% vs 67%
Methods: This study describes the pediatric population with mature (p= 0.16) respectively. 5-year EFS and OS of ovarian, testicular and
teratomas and Brazilian pediatric clinical trials (CT99 and GCT2008). extragonadal GCTs were 73% vs 53% vs 36% (p: 0.01) and 82% vs
Analyzes for patients aged 0 to 21 years with a diagnosis of teratoma 78% vs 63% (p= 0.17) respectively. Among ovarian GCTs, 55% received
were included. GCTs mixed with associated teratoma were excluded carboplatin while in testicular and extragonadal GCTs, 65% received
from the analysis. cisplatin-based chemotherapy. There was no difference in EFS of semi-
Results: 414 patients were included among all teratomas, 76% were nomatous and non-seminomatous GCTs (p value=0.2) but OS was
female. Seventy percent were TM (290), with 49.3% ovarian, 11% better in seminomatous tumors (p value=0.03).
testicular, 20% sacrococcygeal and 36.7% other locations. All of Conclusions: Adolescent with testicular and extragonadal pri-
them were considered localized disease and 96% were treated with maries have poorer outcomes than ovarian GCTs. Carboplatin-based
total surgical resection, no patient received chemotherapy and 99% chemotherapy showed better OS and EFS among adolescents
are alive and disease-free. Only 3 patients had recurrence at the in this study. Further studies are needed to identify poor
primary site. Among the 124 ITs, 41.9% ovarian, 11.3% testicular, responders in this group who might benefit from intensifying
27% sacrococcygeal and 25% from other locations (63.6% from the therapy
head and neck). Eighty percent received surgery alone as treatment,
while 13.7% patients (stage IV) received postoperative chemotherapy.
Twelve patients had recurrence and 87.1% are alive and disease-free. E-Poster Topic: AS05.l Rare Tumours and Histiocytosis
Conclusions: This analysis shows an overview of the presentation of
teratomas in children and adolescents in the Brazilian cohort, with the E-POSTER VIEWING
challenge of disseminating knowledge of the behavior of these tumors,
as well as their treatment. EP280 / #640 SPECTRUM OF LIVER DISEASES IN CHILDREN
WITH LANGERHANS CELL HISTIOCYTOSIS

EP279 / #1340 TREATMENT OUTCOMES OF ADOLESCENTS Leni Mathew, Arul Premanad, Rikki John, Deepthi Boddu, Thomas
WITH EXTRA-CRANIAL GERM CELL TUMOR RECEIVING Alex, Hema Srinivasan, Sidharth Totadri
CISPLATIN VS CARBOPLATIN BASED CHEMOTHERAPY-A Christian Medical College Hospital, Pediatric Hematology-oncology, Div Of
RETROSPECTIVE REVIEW Child Health, Vellore, Tamil Nadu, India

Zunaira Shaukat1 , Najma Shaheen2 , Rabia Wali2 Background and Aims: Hepatic Langerhans cell histiocytosis (LCH) is
1 Shaukat Khanum Memorial cancer Hospital & Research Center, Lahore, characterized by proliferation and accumulation of Langerhans cells in
Paediatric Oncology, Lahore, Pakistan; 2 Shaukat Khanum Memorial cancer the liver, causing liver dysfunction or mass lesion. The diagnosis of hep-
Hospital & Research Center, Lahore, Pediatric Oncology, Lahore, Pakistan atic LCH is based on the following: (1) hepatomegaly, defined as a liver
edge greater than 3 cm below the costal margin at the mid clavicular
Background and Aims: To compare outcomes using cisplatin or line (2) liver dysfunction defined as bilirubin > 3 times, transaminases >
carboplatin-based chemotherapy in adolescent extracranial germ cell 3 times of normal, protein < 55 g/L, albumin < 3 g/dL, or by clinical enti-
tumor (GCT) ties including an intrahepatic nodular mass or ascites or edema, not as
Methods: A retrospective analysis of 75 patients with GCT aged 11 a result of other causes (3) histopathological findings of active disease.
years to 17 years 364 days, recruited at Shaukat Khanum Memorial We carried out this retrospective review of cases to study the spectrum
cancer Hospital, from January 2008 to December 2016. Data fields of liver diseases seen in our patients with LCH.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S278 of S635 ABSTRACTS

Methods: Medical records of children diagnosed to have LCH from to diagnosis of 16 days, most of the patients present secondary HLH
2001 to 2020 were reviewed after obtaining IRB approval. Those chil- with infection demonstrated. Prevalence between 2018 and 2021 was
dren who had features of liver involvement were included for further 0.123%, however in 2018 the prevalence was 0.018% and increased
analysis. to 0.377% in 2021 using diagnostic work-up based on literature and
Results: 64/304 children with LCH had hepatic involvement. 45 boys resources to perform a quick diagnosis.
and 19 girls with a median age of 28.9 months at diagnosis. 61 had hep- Conclusions: HLH is a rare entity but with a high mortality rate
atomegaly, 14 had jaundice and 4 had ascites. Abnormal LFT was noted which makes early recognition crucial for appropriate management.
in 38 children; these included hypoalbuminemia (33) transaminitis (27) Using our diagnostic algorithm, we were able to recognize and treat
hyperbilirubinemia (24). 55/64 children had either /or ultrasound /CT prompty patients protocolizing initial pathway to decrease mortality
scans done and the findings were as follows: hepatomegaly (44), coarse index.
echotexture (17) hypoechoic nodules (9), portal hypoechogenecity (5)
sclerosing cholangitis (5). 10 children had liver biopsy; 6, 1 and 3 had
early, late or mixed stages of disease. 15/64 children developed chronic EP282 / #1504 USE OF MAPK PATHWAY INHIBITORS IN
liver disease. PEDIATRIC HISTIOCYTOSIS IN SPAIN
Conclusions: The incidence of hepatic LCH in our cohort was 21%. 60%
of them had biochemical evidence of liver dysfunction. Up to 17% had Itziar Astigarraga1 , Piedad Alba-Pavón2 , Vicente Santa-María3 , David
features other than isolated hepatomegaly on imaging. 23% of those Ruano4 , Mara Andres5 , Lorena Valero Arrese6 , Constantino Sabado
who had hepatic LCH progressed to chronic liver disease. Alvarez7 , Ignacio Gutierrez-Carrasco8 , Maria Jose Ortega9 , Monica
Lopez-Duarte10 , Ana Isabel Gonzalez-Espin11 , Jose Luis Fuster
Soler12 , Susana García-Obregón13
EP281 / #2000 HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS 1 Hospital Universitario de Cruces. IIS Biocruces Bizkaia. Universidad
AT A PEDIATRIC SPECIALTY HOSPITAL. OUTCOMES AND País Vasco UPV/EHU, Unidad De Hematología Y Oncología Pediátrica,
DIAGNOSTIC PATHWAY PROPOSAL FROM 2018 TO 2021 Barakaldo, Spain; 2 Biocruces Bizkaia Health Research Institute, Pediatric
Oncology Group, Barakaldo, Spain; 3 Hospital Sant Joan de Déu, Pediatric
Daniela Arce Cabrera1 , Azucena Resendis Espinosa2 , Scott Macías2 , Cancer Center Barcelona (pccb), Barcelona, Spain; 4 Hospital Universitario
Saul Canizales2 , Miguel García Domínguez3 Niño Jesus, Unidad De Oncologia Pediatrica, Madrid, Spain; 5 Hospital
1 Hospital Pediatrico de Sinaloa, Hemato-oncology Unit, Culiacan, Mexico; Universitario La Fe, Unidad De Hematología Y Oncología Pediátrica, Valen-
2 Hospital Pediátrico de Sinaloa, Pediatrics, Culiacán, Mexico; 3 Hospital cia, Spain; 6 Vall d’Hebron Hospital, Paediatric Oncology And Haematology
Pediatrico de Sinaloa, Allergology Department, Culiacan, Mexico Department, Barcelona, Spain; 7 Hospital Universitari Vall d Hebron, Pedi-
atric Oncology, Barcelona, Spain; 8 Hospital Infantil Virgen del Rocio, Unidad
Background and Aims: Hemophagocytic lymphohistiocytosis (HLH) De Oncologia Pediatrica, Sevilla, Spain; 9 Hospital Universitario Virgen de las
is an acute and rapidly progressive systemic inflammatory disor- Nieves, Unidad De Oncologia Pediatrica, Granada, Spain; 10 Hospital Univer-
der characterized by cytopenias, hypercytokinemia, and hyperfer- sitario Marques de Valdecilla, Department Of Hematology, Santander, Spain;
ritinemia. Common clinical manifestations of HLH are acute fever, 11 Hospital Materno Infantil Jaén, Unidad De Oncohematología Infantil,
lymphadenopathy, hepatosplenomegaly and multiorgan failure that Jaen, Spain; 12 Hospital Clínico Universitario Virgen de la Arrixaca, Pedi-
requires prompt recognition of the symptoms and early treatment. atric Oncohematology, Murcia, Spain; 13 Biocruces Bizkaia Health Research
The main objectives are to describe clinical and demographic charac- Institute. UPV/EHU, Pediatric Oncology Group, Barakaldo, Spain
teristics of the patients who meet the criteria of HLH and develop a
diagnostic algorithm. Background and Aims: The MAPK pathway is one of the most com-
Methods: Patients diagnosed with HLH, between January 2018 and monly altered pathways among childhood neoplasia. BRAF V600E
December 2021 in a pediatric hospital, were included in this study who mutations are common in histiocytosis, mainly in Langerhans Cell His-
met 4/8 criteria from HLH-2004 along with hyponatremia, hepatitis, tiocytosis (LCH). AIM: Review of the off-label use of BRAF inhibitors in
neurological features and elevated LDH mainly in the emergency room. pediatric patients diagnosed with histiocytosis in Spain.
We performed bone marrow aspirate and based on the clinical charac- Methods: Analysis of 127 patients included in LCH-IV international
teristics and laboratory findings, we developed a diagnostic algorithm study from Spain and 12 cases treated with inhibitors. Review of the
for its recognition and treatment substained in the literature with a results of a survey about BRAF-MEK inhibitors use sent to the Spanish
simple pathway where we detected patients with suspicious of HLH Society of Pediatric Hematology and Oncology members.
and according to the first evaluation, we continue diagnostic work-up Results: Since LCH-IV study open in Spain in 2017 until 1/April/2022,
until treatment 127 patients were enrolled, mainly in stratum I (21 multisystemic
Results: We analyzed clinical and laboratory findings from 23 pedi- and 43 polyostotic/CNS risk bone lesions) and VI (54 Natural history)
atric patients, affecting mainly females (56%), fever was the most but also 4 in relapses and 3 in CNS tumors or Neurodegenerative-
frequent clinical sign and hyperferritinemia the most prevalent lab- LCH (ND-LCH) stratum. Among LCH studies, inhibitors were use in
oratory abnormality. The mortality rate was 39%, with a mean time 6 patients, due to ND-LCH in 3 cases, refractory high-risk in 2 and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S279 of S635

high-risk relapse in 1. After the survey, data from 8 Spanish hospitals disease. Blood tests both at diagnosis and follow-up were normal, apart
were collected about six patients. The main causes for inhibitors use from a transiently elevated alanine transaminase level. The infant is
were: Langerhans cell histiocytosis in 11 and Erdheim-Chester Disease now 5 months old, remains well and is developing normally with no evi-
(ECD) in one patient. BRAF inhibitors were used in 12 (6-vemurafenib, dence of bone marrow, skin, or risk-organ involvement of LCH. She will
6-dabrafenib) and MEK in 2 patients (trametinib). Up-front inhibitors continue to be monitored for re-activation.
use was reported only in ECD. Oral tolerance was good, without severe Conclusions: We report a case of self-resolving congenital LCH, con-
adverse effects. Therapy duration ranged from five years to six month sistent with the Hashimoto-Pritzker variant. Importantly, we describe
and most patients are still on-therapy. a novel fusion in LCH, KLC1::RAF1. We hypothesise that this fusion
Conclusions: The chemotherapy was used in 50% of LCH-IV cases drives the constitutive activation of ERK characteristic of LCH. In the
and curative in most patients. BRAF inhibitors in refractory MS-LCH event of re-activation, this RAF fusion may be targeted through MEK
showed rapid tumor regression, but relapse after stopping therapy inhibition.
appeared in most cases. The efficacy in LCH-ND is still controver-
sial. New international trials that incorporate inhibitors are needed,
so that we learn rather than the off-label use. Targeted therapies are EP284 / #1238 TREATING ADRENOCORTICAL CARCINOMA
not enough, but might contribute to reduce burden by chemotherapy WITHOUT MITOTANE LEVEL MONITORING: CHALLENGES AND
in LCH patients. OUTCOMES FROM A LMIC

Rawan Budair1 , Taleb Ismael2 , Hadeel Halalsheh3 , Khalil Ghandour1 ,


EP283 / #258 SELF-RESOLVING CONGENITAL LANGERHAN Iyad Sultan1
CELL HISTIOCYTOSIS (LCH) WITH A NOVEL KLC1::RAF1 1 king hussein cancer center, Pediatric Oncology, Amman, Jordan; 2 King
FUSION Hussein Cancer Center, Pediatric Oncology, Amman, Jordan; 3 King Hussein
Cancer Center, Pediatric, Amman, Jordan
Rowena Guermech1 , Jamie Trotman2 , James Watkins2 , Vasanta
Nanduri3 , C Elizabeth Hook2 , Patrick Tarpey2 , Nick Coleman2 , Shivani Background and Aims: Treatment of adrenocortical carcinoma (ACC)
Bailey1 depends on surgical resection of primary tumor and systemic therapy
1 Cambridge University Hospitals NHS Foundation Trust, Department using chemotherapy and mitotane. While associated with significant
Of Paediatric Haematology And Oncology, Cambridge, United Kingdom; short and long term toxicity, mitotane levels are not readily available
2 Cambridge University Hospitals NHS Foundation Trust, Department in many centers, particularly in LMIC.
Of Pathology, Cambridge, United Kingdom; 3 Watford General Hospital, Methods: This is a retrospective chart review of children (<18years)
Department Of Paediatrics, Hertfordshire, United Kingdom diagnosed with ACC at KHCC between Jan2000 and Dec2021.
Demographics, clinical features at diagnosis, outcome and toxici-
Background and Aims: We report the case of an infant with con- ties were captured from written notes. All patients were labeled
genital Langerhans cell histiocytosis (LCH). She presented at birth as steroid deficient and were given stress dose of hydrocorti-
with ‘blueberry muffin syndrome’, a descriptive term for the pres- sone upon presentation to ER for any febrile illness after starting
ence of multiple blue/purple nodules in the skin. Congenital LCH is mitotane.
rare, and the clinical course can vary from self-resolution to pro- Results: Thirteen patients were included (6 males and 7 females) with
gression to multi-system LCH, and it carries the risk of re-activation. a median age of 3-years (range, 1-16.5). At presentation, 85% had
There is little available molecular data on these cases, particularly link- virilization, 54% had hypertension and 31% had cushingoid features.
ing the relationship between genotype and phenotype in congenital All patients underwent tumor resection. The median tumor weight of
LCH. 116-grams (range 25 – 745mg ). Postoperative stages were 1(N=5,
Methods: Microbiological, haematological, and biochemical testing 38%), 2(N= 3, 23%), 3(N= 2,15%) and 4(N=3, 23%). Eight patients
was undertaken. A skin biopsy was performed for histological and received mitotane with a median peak dose of 3.5gram/m2/day (range
molecular testing as standard-of-care. No tissue was used for experi- 1-4gram/m2/day). This dose was achieved by slow increments from a
mental purposes. starting dose of 1g/m2/day and were maintained for a median of 8
Results: Screening for congenital infections was negative. Histology months (range, 6-18). Toxicity was mainly hematologic and related to
from the skin biopsy was consistent with LCH, and immunostains con- the use of chemotherapy. None of our patients were admitted to the
firmed the diagnosis. A targeted next-generation sequencing panel did intensive care unit. Only 2 patients required modifications of mitotane
not identify V600E, or any other exon 15 BRAF mutations. Whole during therapy due to suspected toxicity. Also, 2 patients needed long-
genome sequencing of paired tumour and germline DNA identified a term steroid replacement. By the time of last follow 2 patients with
somatic KLC1::RAF1 fusion, and no germline mutations. The expres- stage 4 had died while the rest are still alive with no evidence of disease.
sion of the KLC1::RAF fusion was validated through RNA sequencing. Conclusions: The outcome and toxicity of our patients are in line with
Staging investigations for LCH, including skeletal survey, bone marrow published literature. The lack of mitotane level monitoring did not seem
aspirate and ultrasound of liver and spleen revealed no other sites of to impact our patients’ outcome.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S280 of S635 ABSTRACTS

EP285 / #170 ROSAI DORFMAN DISEASE – EXPERIENCES IN group.In this study, can serum EBV PCR titer increases in pediatric
THE GERMAN RARE HISTIOCYTOSES REGISTRY AND cases with EBV-associated cancer be a stimulating factor for the
CONSULTATION CASES disease? search for an answer to the question.
Methods: File data of cases diagnosed with EBV-associated cancer in
Carl Friedrich Classen our center between 2011-2021 were retrospectively reviewed.
University Childrens Hospital Rostock, Ped. Oncology And Palliative Care, Results: 156 cases diagnosed as Non-Hodgkin Lymphoma (NHL),
Rostock, Germany Hodgkin Lymphoma (HL) and Nasopharyngeal Carcinoma (NPC) in
our center between 2011-2021, which are known to be associated
Background and Aims: Rare or Non-Langerhans cell histiocytoses with EBV, were retrospectively analyzed. These cases consisted of
(non-LCH) include very different diseases. In pediatrics, besides juve- 31(19.8%) HL, 10 (6.4%) NPC, 115 (73.7%) NHL cases. EBV PCR pos-
nile xanthogranulomatosis, Rosai-Dorfman disease (RDD), is most itivity was present at the time of diagnosis in 15 (9.6%) cases out of
frequent. 156 cases. Of the positive cases, 3 were HL, 7 were NPC, and 5 were
Methods: In 2012, the German registry/consultation service for non- NHL. Immunodeficiency was also detected in one HL and 2 NHL cases.
LCH - part of International IRHDR - was initiated, including 97 patients One NPC case was excluded from the study because its detailed data
so far. were not available. Considering the follow-ups, relapse or progressive
Results: 20 RDD patients were reported, all but one survivors (3 disease was detected in 6 cases, and it was observed that the EBV
months to 5 years follow-up); 9/10 female/male; 0 were infants, 2 1y PCR titers, which had become negative, increased again at the time of
old, 5 2-4 y old, 12 5-18 y old, 1 >18y. 7 had cervical lymph node relapse/progression in all of these cases. Of these cases, 3 were NPC, 2
involvement only (all <5 y); 4 had CNS involvement only (all 5-8y), 9 were HL, and the other case was diagnosed as relapsed/refractory NHL
had special or multiple organ involvement (most bone, but also kid- with the diagnosis of immunodeficiency. No re-positive EBV PCR titers
neys, skin, glands, and other organs, age from 1 to >18y). In two, were detected in other cases followed up in remission.
a mixed/overlap histiocytosis was found (RDD/Erdheim-Chester dis- Conclusions: We think that EBV PCR follow-ups should be done regu-
ease, RDD/LCH), one patient had H-syndrome (germline), one patient larly in cases diagnosed with EBV-related tumors, especially nasopha-
first had systemic RDD, then Acute Lymphoblastic Leukemia, and died ryngeal cancer, since an increase in EBV titer may be a precursor of the
from it. Genetic analysis was performed in 6; showing 1 ALK, 1 KRAS, disease.
and 1 MAP2K1 mutation, and the H syndrome in 1 case, two were neg-
ative. 7 patients who had cervical lymph node disease only, received
resection followed by observation in 5 cases, and steroids in 2. 4 EP287 / #1875 NASOPHARYNGEAL CARCINOMA IN
patients having CNS involvement were treated by observation after CHILDHOOD: ANALYSIS OF A SERIES OF 50 PATIENTS
resection in 1, steroids in 2, and targeted therapy in 1 case. The sys- TREATED WITH COMBINED CHEMOTHERAPY AND
temic cases received steroids in 2 cases, polychemotherapy in 3 cases, RADIOTHERAPY
and targeted therapy in 1; the others combined therapies or unknown.
Conclusions: RDD is a heterogeneous disease. In localized lymph node Maria El Kababri, Hanane El Kacemi, Rajaa Dahmani, Mohamed El
cases, typically at age 1-5, observation or steroids are appropriate. CNS Khorassani, Mohamed Khattab, Leila Hessissen, Amina Kili
disease is typical for children in school age. Besides, individual courses Mohammed V University. Rabat, Pediatric Hematology And Oncology
like systemic or with mixed/overlap histology or malignant transforma- Department, RABAT, Morocco
tion, may require individual therapies. Molecular analyses are crucial
to enable targeted therapies. Appropriate consultation depends on Background and Aims: Nasopharyngeal carcinoma is a cancer aris-
international registration, which should be propagated. ing from the nasopharynx epithelium. Within the boundaries of the
nasopharynx, the tumour epicentre is frequently seen at the fossa
of Rosenmüller, from where the tumour invades adjacent anatomical
EP286 / #1313 CAN EBV TITER BE USED AS A TUMOR spaces or organs. The aim of our study is to investigate the epi-
MARKER IN EBV RELATED CHILDHOOD MALIGNANCY? demiological, clinical, radiological, therapeutic particularities of the
nasopharyngeal carcinoma (NPC) in children, and to determine prog-
Nurşah Eker1 , Ayşe Tokuç1 , Simay Erdal2 , Cansu Çeliktaş2 nostic factors correlated with outcome and features of a series of 50
1 Marmara University Medical Faculty, Pediatric Hematology And Oncology, cases collected in the Pediatric Hematology Oncology Center, in Rabat
Pendik, Turkey; 2 Marmara University Medical Faculty, School Of Medicine, (Morocco).
Pendik, Turkey Methods: A total of 50 patients with NPC were treated in the Center
of Pediatric Hematology and Oncology in Rabat, from January 2010 to
Background and Aims: Ebstein-Barr virüs (EBV), a member of the December 2019. They were retrospectively analyzed. Overall survival
herpes virus family, causes infectious mononucleosis. It is known that (OS) and disease-free survival (DFS) were calculated.
EBV may play a role in the etiology of patients with B-cell lymphomas, Results: Most patients were males (73%). Median age was 13
Hodgkin lymphoma and nasopharyngeal carcinomas in the childhood years. The main presenting symptoms were neck mass (81%),
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S281 of S635

tinnitus/hearing loss (53%), bloody nasal discharge (23%), headache with regional lymph node involvement and 17 patients (71%) with dis-
(56%) and nasal obstruction (20%). Stage I, II, III, IVA, IVB and IVC tant metastases, in most cases lung (38%), distant lymph nodes (38%)
patients accounted for 0%, 7.7%, 62%, 13.7%, 7.8% and 7.8%, respec- and bone marrow (30%). Approximately half of patients were initially
tively. All patients were treated by neoadjuvant chemotherapy. The misdiagnosed and diagnosis was corrected after NUT immunochem-
complete response rate to chemotherapy was 68%. All patients were istry or NUT fusion sequencing. Chemotherapy was administered in all
treated by radiotherapy: 60 to 70 Gy to primary tumors, 50 to 65 patients; nine patients underwent major surgery and 19 radiotherapy.
Gy to cervical lymph nodes. Locoregional relapses were observed in Median overall survival was 0.75 years (range 0.2-11 years) median
4 patients, metastatic relapses in 4 cases. The 2 and 5- year overall event free survival 0.4 years (range 0.1-11 years), one patient is cur-
survival (OS) rates were 91,7% and 84,3% respectively. Disease- rently treated for a subsequent relapse (1.9 years after diagnosis).
free survival (DFS) was 72%. The main long-term complications of Three long-term survivors (11, 9.1 and 6.6 years after diagnosis) were
therapy were trismus (40%), hearing loss (25%), xerostomia (50%), identified, these cases were associated with non-metastatic cervical
hypothyroidism (8%), growth disorders (10%) and neck fibrosis (30%). disease and non-metastatic disease with BRD3-NUT-fusion.
Conclusions: The majority of patients were diagnosed at advanced Conclusions: As in adults, NUT-carcinoma in pediatric patients is
stages. Children and adolescents with NPC had excellent survival poorly sensitive to conventional therapy in most cases. In a minority
except metastatic disease. The TNM stage was the most relevant of patients long-term survival is possible with multimodal treatment.
prognostic factor. Early diagnosis of undifferentiated or poorly differentiated carcino-
mas to identify the specific rearrangement of NUT-gene is necessary
to initiate the optimal diagnostic and therapeutic strategy.
EP288 / #671 NUT CARCINOMA IN CHILDREN: THE EXPERT
EUROPEAN EXPERIENCE
EP289 / #341 BCOR-CCNB3-FUSION POSITIVE SARCOMA.
Tim Flaadt1 , Lauriane Lemelle2 , Calogero Virgone3 , Tal Ben-Ami4 , THE IMPORTANCE OF TWINNING PROJECTS TO ADDRESS THE
Denis Kachanov5 , Ulrich Lauer6 , Dominik Schneider7 , Andrea Ferrari8 , DIAGNOSTIC LIMITATIONS OF RARE TUMORS IN DEVELOPING
Gianni Bisogno9 , Yves Reguerre10 , Ines Brecht1 , Daniel Orbach2 COUNTRIES
1 University Children’s Hospital, Department Of Pediatric Hematology And
Oncology, Tuebingen, Germany; 2 Institut Curie, Siredo Oncology Depart- Julieta Hoveyan1,2 , Saten Hovhannisyan1,2 , Clodet Stepanians1,2 , Irina
ment, Paris, France; 3 University of Padua, Pediatric Surgery, Department Of Melnichenko1,2 , Armen Mkhitharyan3 , Lusine Hakobyan1,2 , Lilit
Women’s And Children’s Health, Padua, Italy; 4 Kaplan Medical Center, Pedi- Sargsyan1,2 , Gevorg Tamamyan1,2 , Ruzanna Papyan1,2
atric Hematology Unit, Rehovot, Israel; 5 Dmitry Rogachev National Medical 1 Yerevan State Medical University, Department Of Pediatric Oncology And
Research Center of Pediatric Hematology, Oncology and Immunology, Hematology, Yerevan, Armenia; 2 Hematology Center after Prof. R.H.Yeolyan,
Department Of Clinical Oncology, Moscow, Russian Federation; 6 University Pediatric Cancer And Blood Disorders Center Of Armenia, Yerevan, Armenia;
of Tuebingen, Department Of Internal Medicine Viii, Medical Oncology And 3 HistoGen Pathology Center, Histogen Pathology Center, Yerevan, Armenia

Pneumology, Tubingen, Germany; 7 Dortmund Municipal Hospital, Clinic Of


Pediatrics, Dortmund, Germany; 8 Fondazione IRCCS Istituto Nazionale dei Background and Aims: BCOR-CCNB3-fusion positive sarcoma is a
Tumori, Pediatric Oncology Unit, Milano, Italy; 9 University of Padua, Hema- recently defined rare subtype of undifferentiated round cell sarco-
tology/oncology Division, Department Of Women’s And Children’s Health, mas. Like other molecular genetic tumor subtypes, it is a challenging
Padua, Italy; 10 Félix Guyon University Hospital, Department Of Pediatric diagnosis for developing countries.
Hematology And Oncology, St Denis, Réunion Island, France Methods: Here we report a case of a 15-years-old boy diagnosed with
BCOR-CCNB3-fusion positive sarcoma.
Background and Aims: NUT-carcinoma is a rare, probably underdiag- Results: A fifteen-years-old male was admitted to the hospital with
nosed and highly aggressive tumor defined by the presence of a somatic complaint of a painful mass in the left leg. CT revealed pathological
NUTM1 rearrangement. The tumor occurs mainly in adolescents mass in the thickness of the left extensor digitorium longus muscle
and young adults. We analyzed the clinical, radiologic, and biological (5.3x4.9x17.6cm in size) and signs of active hemorrhage. The patho-
features of pediatric patients (≤18 years) with NUT-carcinoma. logical mass was completely resected and histologically hematoma
Methods: This retrospective multicenter international study was was diagnosed. Six months later, the patient returned with the same
based on review of medical records of 24 children with NUT-carcinoma complaints. MRI revealed multichambered pathological mass in the
from 5 countries with specific rearrangement or positive anti-NUT anterior and lateral compartment of the left leg (4.0x2.8x15.6cm
nuclear staining. in size). The patient underwent second surgery, and the lesion was
Results: Twenty-four patients with a median age of 14.6 years (range: completely removed. According to the histological and immunohisto-
3.9–18 years) were analyzed. Thoracic/mediastinal tumors were the chemical examinations low-grade fibromyxoid sarcoma was diagnosed.
primary in 14 patients, and head and neck in 7 cases. One patient Postsurgical CT showed no signs of residual disease and distant metas-
had multifocal tumor with unknown primary, another a vocal cord tases. After the patient was admitted to our clinic, review of paraffin-
and the last one a pancreas primary. Sixteen patients (67%) presented embedded tissue blocks of first surgery performed and diagnosis of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S282 of S635 ABSTRACTS

low-grade fibromyxoid sarcoma was confirmed. After Musculoskeletal Conclusions: Our findings indicated that HCT using
Multidisciplinary Working Group discussion via telemedicine, deci- treosulfan/fludarabine/thiotepa/r-ATG shows a favorable out-
sion was made to review the paraffin-embedded tissue blocks and come with excellent donor chimerism in pediatric patients with
conduct molecular genetic testing in Germany, since there were dis- HLH. Furthermore, haploidentical HCT from an αβ T cell-depleted
crepancies between histological reports and suspicion of recurrent graft could be a possible alternative in patients who lack a matched
disease. The final diagnosis was BCOR-CCNB3-fusion positive undif- related or unrelated donor. Our study’s high incidence of acute GVHD
ferentiated sarcoma, which was evaluated as a recurrent. The lack of requires further modification of the conditioning regimen or GVHD
molecular genetic testing in Armenia led to a delay in diagnosis by 3 prophylaxis.
months.
Conclusions: Diagnosis of rare pediatric tumors is challenging in devel-
oping countries like Armenia. The lack of molecular genetic testing EP291 / #1859 25 YEARS OF LANGERHANS CELL
not rarely leads to misdiagnosis. Twinning projects between clinics HISTIOCYTOSIS EXPERIENCE OF A SINGLE CENTER
of developing and developed countries remain extremely important
for the diagnosis of rare tumors and can reduce disparities in cancer Dilek Ince1 , Deniz Kizmazoğlu1 , Emre Çeçen1 , Handan Güleryüz2 ,
treatment in developing countries. Erdener Özer3 , Nur Olgun1
1 Dokuz Eylul University Institute of Oncology, Pediatric Oncology, IZMIR,
Turkey; 2 Dokuz Eylul University, Radiology, İZMİR, Turkey; 3 Dokuz Eylul
EP290 / #1513 EXPERIENCES WITH TREOSULFAN-BASED University Faculty of Medicine, Pathology, IZMIR, Turkey
CONDITIONING FOR HEMOPHAGOCYTIC
LYMPHOHISTIOCYTOSIS Background and Aims: Evaluation of patients diagnosed with Langer-
hans cell histiocytosis (LCH) and treated in our center.
Sung Han Kang, Young Kwon Koh, Eun Seok Choi, Hyery Kim, Methods: The medical records of patients with LCH were rewieved.
Kyung-Nam Koh, Ho Joon Im The clinical characteristics, treatment details and responses were
Asan Medical Center, University of Ulsan College of Medicine, Pediatrics, analyzed retrospectively.
Seoul, Korea, Republic of Results: There were 60 patients with LCH between 1987-2022. The
median age of diagnosis was 55 months (2.5mos –18yrs), M/F=1.3.
Background and Aims: Hemophagocytic lymphohistiocytosis (HLH) Single system involved LCH (SSIG-LCH) (68%, n:41); involvement sites
challenges hematopoietic stem cell transplantation (HSCT) due to were bone (88%), skin (7%), lymph node (2.5%), lung (2.5%). Nine
the high graft failure rate, relapse, and simultaneous toxicity. This patients had vertebral involvement. Surgery was performed in 66%,
report evaluated the outcome of treosulfan-based conditioning in HLH chemotherapy consisted vinblastin, prednisolone+methotrexate, mer-
patients. captopurine was given in 63%. Radiotherapy (RT) was given in 12%
Methods: Between January 2016 and April 2022, 21 patients with of cases (3 of them vertebral LCH). Relapse occured in 5 cases. The
HLH received HCT with a treosulfan-based conditioning regimen median follow-up time was 6.5yrs (1month -18yrs), 15-years OS rate
using matched sibling (MSD, 1), unrelated (URD, 13), and haploiden- was 100% and 5-year and 15-years EFS rates were 84%. Multisystem
tical family donor (HFD, 7). Nine had familial HLH (3 PRF1 and involved LCH (MSIG-LCH) (32%, n:19); involvement sites were bone
6 UNC13D), 6 had other genetic defects (4 XIAP, 1 XLP1, and 1 (95%), skin (53%), lung (42%), pituitary gland (21%), Liver (16%), Gas-
Griscelli syndrome), and 6 had secondary HLH. The conditioning trointestinal system (16%), spleen (5%). Eight of them had risky organ
regimen consisted of treosulfan, fludarabine, thiotepa, and rabbit (RO) involvement, 4 of 8 patients had lung involvement as RO. Surgery
ATG. The cyclosporine and mycophenolate mofetil were used for was performed in 58% of cases, chemotherapy consisted vinblastin,
GVHD prophylaxis in MSD or URD. For the haploidentical HCT, an prednisolone+methotrexate, mercaptopurine was given in all 19, and
ex vivo αβ T cell-depleted graft was used without pharmacologic RT was given in two patients. Refractory disease (n:2) and relapse
prophylaxis. (n:6) occured in 8 patients. The median follow-up time was 5years
Results: All patients achieved engraftment at a median of 10 days (5months-17yrs), the 2-and 15- years OS rates were 88% and EFS
without late graft failure. One patient developed VOD, which was suc- rates were 39%. One infant had skin+pulmonary LCH (risky organ), and
cessfully treated with defibrotide. Ten (48%) and 6 (29%) patients died with refractory LCH despite anticancer treatment at 21months.
developed grade 2 and 3-4 acute GVHD, respectively. One patient Other infant died from meningococcemia. Remission achieved with
developed moderate chronic GVHD, and 3 developed severe chronic vemurafenib in two cases.
GVHD. Nineteen survived with complete donor chimerism, and 2 died Conclusions: In MSI-LCH group age tends to be younger than
due to pneumonia and asphyxia, respectively. At a median follow-up SSI-LCH, these patients particularly had RO involvement.
of 28 months, the overall survival (OS) and disease-free survival (DFS) Although lung involvement is not considered as risky organ, pul-
were 92.3% and 91.7% at 2 years, respectively. The 2-year probability monary LCH can be fatal especially in the infancy. In relapsed/
of moderate-to-severe chronic GVHD-free and disease-free survival refractory cases, remission can be achieved by anti-BRAF
(CDFS) was 67.9%. drugs.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S283 of S635

EP292 / #851 CARDIAC EVALUATION BEFORE AND AFTER Eftichia Stiakaki5 , Helen Kosmidis4 , Sophia Polychronopoulou3 ,
ORAL PROPRANOLOL TREATMENT IN INFANTILE Margarita Baka2 , Kalliopi Stefanaki8 , Antonis Kattamis1
HEMANGIOMAS 1 “Aghia Sophia” Children’s Hospital, Division Of Pediatric Hematology
Oncology, First Dpt Of Pediatrics, National & Kapodistrian Univer-
Hye Lim Jung, Jihee Kwak, Eun Sil Kim, Aram Yang, Deok Soo Kim, sity Of Athens, Athens, Greece; 2 ‘P & A. Kyriakou’ Children’s Hospi-
Jung Yeon Shim, Jae Won Shim tal, Department Of Oncology, Athens, Greece; 3 “Aghia Sophia” Chil-
Sungkyunkwan University School of Medicine, Kangbuk Samsung Hospital, dren’s Hospital, Department Of Pediatric Hematology-oncology, Athens,
Department Of Pediatrics, Seoul, Korea, Republic of Greece; 4 Children’s Hospital MITERA, Pediatric And Adolescent Oncol-
ogy Clinic, Athens, Greece; 5 University Hospital of Heraklion, Depart-
Background and Aims: Infantile hemangiomas (IHs) are the most com- ment Of Pediatric Hematology-oncology, University Of Crete, Heraklion,
mon vascular tumor developing in children, and oral propranolol, a Greece; 6 Ippokratio Hospital, Department Of Pediatric Oncology, THESSA-
non-selective beta-adrenergic antagonist, is known as first-line treat- LONIKI, Greece; 7 Aristotelion University of Thessaloniki, AHEPA General
ment. This study prospectively assessed cardiac status initially and Hospital„ Department Of Pediatric Hematology-oncology, Thessaloniki,
regularly to establish guidelines for monitoring cardiac function and Greece; 8 “Aghia Sophia” Children’s Hospital, Pathology Department, Athens,
toxicity in IHs treated with propranolol for ≥6 months. Greece
Methods: 64 patients diagnosed with IHs and treated with oral propra-
nolol before 2 years of age at the Department of Pediatrics, Kangbuk Background and Aims: Rhabdoid-tumors(RT) are rare, highly-
Samsung Hospital (Seoul, Korea) with cardiac evaluations between aggressive neoplasms arising from renal (MRT), extrarenal
2017 and 2021, were included. Cardiac evaluations including ECG, 24 soft-tissue(EERT) or central-nervous-system(ATRT). They usually
hour Holter monitoring and echocardiography were performed initially present in infants or young children and have dismal prognosis. Almost
and at 6 to 12 months after treatment with oral propranolol. 10% of pts present with synchronous tumors. If germline-mutations
Results: 64 patients (21 male, 43 female) with IHs successfully under- in SMARCB1 or SMARCA4-genes are found, diagnosis of Rhabdoid-
went cardiac evaluations. The mean percentage of size decrease after 1 Tumor-Predisposition-Syndrome 1 or 2 (RTPS1/RTPS2) is established,
year of oral propranolol treatment was 71.8%. Pre-treatment echocar- respectively. Data from national registries, on this entity are lacking.
diography diagnosed congenital heart disease (CHD) in 25 of 64 Methods: All, 7 pediatric hematology-oncology centers in Greece par-
patients (39.0%), including PDA (3); ASD (6); PFO (6). LV systolic func- ticipated in this retrospective study that covered the period between
tion before and after treatment revealed no statistically significant 1/2012-12/2021. Demographics, tumor characteristics and outcome
differences in ejection fraction, fractional shortening, mitral annular were analyzed.
plane systolic excursion, or global longitudinal strain. However, early Results: Twenty-two pts with RT (11females), with a median age of 10m
(E) and late (A) diastolic mitral inflow velocities, and E/A ratio, together (range:1d-7.5y) were diagnosed in Greece during the study-period.
with early diastolic mitral annular velocity e′ (E/e′ ratio) measure- Diagnosis included ATRT (14pts), EERT (8pts) and MRT(2pts). Two
ments demonstrated a significant increase after treatment. Among pts had synchronous and 1 metachronous tumors. The most common
the RV function measurements, peak systolic tissue velocity (S’) and ATRT-location was posterior-fossa. Upon diagnosis, metastatic lesions
tricuspid annular plane systolic excursion (TAPSE) were significantly were found in 57.1%. RTPS1 was diagnosed in 3 out of the 14pts
improved after treatment (P < 0.05). None of the 64 patients experi- evaluated. All pts underwent surgery (biopsy:6, partial resection:3,
enced adverse events such as arrhythmias, and no significant changes subtotal/total resection:13). Total/subtotal resection was achieved in
in heart rate, PR interval, and QTc, except for QRS duration on ECG 9/14 ATRT and in 3/8 extracranial-RT(ERT). Sixteen pts were treated
during propranolol treatment. according to the EuRHAΒ-Registry investigational-treatment protocol.
Conclusions: Oral propranolol treatment ≥6 months was effec- Two patients did not receive therapy. Radiotherapy as per protocol
tive and safe without significant cardiac toxicity in IHs. Initial was given in 8/22pts (6ATRT, 2ERT). High-dose chemotherapy was
echocardiography is necessary to diagnose CHD. This study sug- administered to 3 children. No treatment-related death occurred. The
gest the cardiac evaluation guidelines for IHs treated with oral median follow-up was 9m(range:1m-8.77y). Overall survival was 50.0%
propranolol. (95%CI15.2-77.4%), 37.5% (95%CI8.7-67.4) and 12.5% (95%CI11.7-
42.2) at 1y, 3y and 5y respectively. Median time of 1st relapse was 5.4m.
Relapse/progression were local, metastatic or combined in 3, 3 and
EP293 / #904 LONGTERM SURVIVORS WITH RHABDOID 12 cases respectively. Only 2pts are long-term survivors in 1st remis-
TUMORS ARE RARE: A NATIONAL REPORT FROM GREECE sion (1ATRT-SHH-subtype for 8.75y and 1MRT 5.25y post-diagnosis
respectively).
Kleoniki Roka1 , Maria Filippidou1 , Dimitrios Doganis2 , Vassilios Conclusions: Due to the rarity and extremely dismal prognosis of
Papadakis3 , Vita Ridola4 , Nikolaos Katzilakis5 , Evgenia these tumors, further investigational efforts within an international
Papakonstantinou6 , Emmanuel Hatzipantelis7 , Antonia Vlachou1 , framework, like in our study, are needed.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S284 of S635 ABSTRACTS

EP294 / #1029 THE ROLE OF GENETIC TESTING IN Nandan Shetye6 , Badira Parambil3 , Akshay Baheti4 , Jifmi Manjali5 ,
DIFFERENTIAL DIAGNOSIS OF HAMARTOMATOUS POLYPOSIS Sneha Shah7
SYNDROMES: SINGLE CENTER EXPERIENCE 1 Tata Memorial Centre, Homi Bhabha National Institute, Pathology, Mum-
bai, India; 2 Tata Memorial Centre, Homi Bhabha National Institute, Surgical
Tatiana Belysheva1 , Tatiana Nasedkina2,3 , Vera Semenova1 , Elena Oncology, Mumbai, India; 3 Tata Memorial Centre, Homi Bhabha National
Sharapova1 , Valentina Kozlova1 , Olga Malikhova1 , Svetlana Institute, Paediatric Oncology, Mumbai, India; 4 Tata Memorial Centre, Homi
Mikhailova1 , Svetlana Varfolomeeva1 Bhabha National Institute, Radiodiagnosis, Mumbai, India; 5 Tata Memo-
1 N.N. Blokhin National Medical Research Center of Oncology, Institute rial Centre, Homi Bhabha National Institute, Radiation Oncology, Mumbai,
Of Pediatric Oncology And Hematology, Moscow, Russian Federation; India; 6 Tata Memorial Hospital, Jaslok Hospital, Ophthalmology, Mumbai,
2 Engelhardt Institute of Molecular Biology of the Russian Academy of India; 7 Tata Memorial Hospital, Homi Bhabha National Institute, Nuclear
Sciences, Laboratory For Biological Microchips, Moscow, Russian Federa- Medicine, Mumbai, India
tion; 3 Dmitry Rogachev National Medical and Research Center of Pediatric
Hematology, Oncology and Immunology, Rehabilitation Center, Moscow, Background and Aims: Intraocular medulloepithelioma, though rare,
Russian Federation forms the second most ocular tumour after retinoblastoma. It arises
from the non-pigmented ciliary epithelium and typically manifests in
Background and Aims: Hamartomatous polyposis syndromes (HPS) the first decade of life. We present a case series of five intraocular
are a heterogeneous group of rare hereditary diseases including juve- medulloepithelioma in the paediatric population.
nile polyposis syndrome, Peutz-Jeghers syndrome, and PTEN hamar- Methods: The cases were retrieved over a period of 10 years from the
toma tumor syndrome (Cowden syndrome). These conditions are electronic medical records at Tata Memorial Hospital, Mumbai. The
characterized by similar clinical manifestations and a high risk of malig- clinical and imaging findings, treatment records, histological features
nancy, but require different follow-up tactics since each syndrome and follow-up data was noted.
carries different organ-specific risks of neoplasia. HPS should also Results: A total of 5 paediatric patients with intraocular medulloep-
be differentiated from other forms of hereditary polyposis (familial ithelioma were included in this series. The age range was from 2 to 15
adenomatous polyposis and MUTYH-associated polyposis). Molecular years. We also found 2 adult cases diagnosed with intraocular medul-
genetic testing in such patients plays a leading role in the differential loepithelioma; both the patients were of age 26 years. The presenting
diagnosis at the early stages of the disease. complaints were loss of vision (most common), followed by redness of
Methods: Retrospective review of hospital records from January 2011 eye and proptosis. Imaging findings demonstrated an anterior cham-
to December 2021, molecular genetic testing. ber mass originating from the ciliary body. The teenage patient had
Results: The study included 20 patients, 13 boys and 7 girls, from 5 to been diagnosed at the age of 3 years, but the family refused treat-
22 years old. The clinical diagnosis of hamartomatous polyposis was ment and the patient presented at a later stage. Interestingly, this
based on diagnostic criteria (Beggs et al., 2010; Jass J. et al., 1988). The patient had limited ocular disease which was treated with enucleation
main symptoms included: abdominal pain in 17 cases (85%), vomiting in followed by radiation therapy. The histology was a non-teratoid medul-
11 cases (55%), mucosal pigmentation in 9 cases (45%), gastrointesti- loepithelioma. The patient had an uneventful follow-up. All the other 4
nal bleeding in 4 cases (20%), and weight loss in 1 case (5%). The results patients showed evidence of extraocular extension, with two patients
of genetic testing were, as follows: 9 patients (45%) had a mutation in developing metastasis to intraparotid lymph node. The patients were
the STK11 gene (Peutz-Jeghers syndrome); 6 patients (30%) carried a treated with enucleation followed by radiation therapy. One patient
mutation in the PTEN gene (Cowden syndrome); 5 patients (25%) had showed recurrence of the disease after completion of treatment, with
mutations in the genes SMAD4 or BMPR1A (juvenile polyposis). Fam- involvement of cervical lymph nodes and vertebral metastases, and is
ily history was known for 14 patients. In 10 families (70%), no other currently on palliative treatment. The other 3 patients are disease free
cases of tumor diseases were observed. In three families, the first-line on follow-up. Histology in all cases showed primitive neuropeithelial
relatives were found to carry mutations in cancer-associated genes. cells, rosette formation and teratoid component in one case.
Conclusions: Molecular genetic testing is indicated in all patients with Conclusions: Intraocular medulloepithelioma is a rare entity, with
suspected HPS to determine the optimal follow-up strategy. Genetic prognosis depending on stage of the tumour. Extraocular extension and
testing is also important for the next of kin of such patients to identify metastatic disease confer a worse prognosis.
asymptomatic carriers of pathogenic mutations and to assess the risk
of developing malignancy throughout life.
EP296 / #1187 COMPARISON OF MODIFIED WEISS AND
WIENEKE SCORING SYSTEM FOR PAEDIATRIC
EP295 / #858 INTRAOCULAR MEDULLOEPITHELIOMA : ADRENOCORTICAL TUMOURS
CLINICO-PATHOLOGICAL FEATURES OF A RARE ENTITY
Mukta Ramadwar1 , Poonam Panjwani2 , Girish Chinnaswamy3 , Maya
Poonam Panjwani1 , Mukta Ramadwar1 , Sajid Qureshi2 , Girish Prasad3 , Sajid Qureshi4 , Vasundhara Patil5 , Nehal‘ Khanna6,7 , Mahesh
Chinnaswamy3 , Maya Prasad3 , Vasundhara Patil4 , Nehal‘ Khanna5 , Patel2
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S285 of S635

1 Tata Memorial Centre, Homi Bhabha National Institute, Pathology, America; 5 Emory University, Department Of Radiation Oncology, Atlanta,
Mumbai, India; 2 Tata hospital, Pathology, MUMBAI, India; 3 Tata United States of America
Memorial Centre, Paediatric Oncology, Mumbai, India; 4 Tata Memo-
rial Centre, Paediatric Surgery, Mumbai, India; 5 Tata hospital, Radiology, Background and Aims: Craniopharyngioma (CP) is a rare brain tumor,
MUMBAI, India; 6 Tata Memorial Centre, Radiation Oncology, Mum- mainstay treatments include surgery and radiation, but have side
bai, India; 7 Tata Memorial Centre, Radiation Oncology, MUMBAI, effects relating to the damage of anatomical structures in the vicinity
India of the tumor. CPs often arise in close proximity to the Circle of Willis.
Patients are at risk for cerebrovascular accidents (CVA). In this study
Background and Aims: Wieneke scoring system is recom- we aim to describe the incidence and temporal relationship of CVA in
mended by recent WHO classification of adrenocortical tumours CP with surgery and/or radiation.
(ACTs). We attempted to study and compare modified Weiss Methods: Data was extracted From IRB approved database of cranio-
(MW) and Wieneke criteria with respect to their clinical pharyngioma patients seen at Children’s Healthcare of Atlanta from
behavior. 2000 to 2021 as a part of retrospective analysis. CVA was diagnosed
Methods: We studied 23 patients with ACTs in our institute from if the patient had symptoms and/or radiologic imaging consistent with
2011 to 2021. Clinical details and follow up were derived from Elec- stroke. Medical course of the disease in relation to timeline of various
tronic medical records. Histopathology was reviewed by applying both treatment modalities was noted.
Wieneke and MW criteria. Results: Of 85 screened patients, 12 patients were found to have CVA.
Results: Age range was from 11 months to 11 years with median of Median age at time of stroke was 10.7 years (range 2.4-21.2). One of
4 years. 13% patients were diagnosed with adrenocortical adenoma 12 patients had intratumoral hemorrhage, all remaining had ischemic
(ACA) by MW criteria while 87% as adrenocortical carcinoma (ACC). stroke. Majority of the CVAs (10/11 patients) occurred 24-48 hours
The range of Weiss score was from 4 to 8. On follow up, (n=12), 66% following surgery involving tumor resection. Neither ommaya nor VP
patients experienced disease relapse while 33% were alive without dis- shunt placement were temporally related to CVA. Two patient events
ease. Patients with relapse had a MW score of 5 and above. 3 of 2 were not temporal related to surgery. Six of 12 patients received radi-
patients with no relapse had a MW score of 7. By Wieneke system, ation therapy prior to CVA. One patient had stenosis of Circle of Willis
a score of 2 and less was applied to 3 (15%), score 3 to 7 (35%) and and had CVA after 3 courses of radiation (1,2 and 8 years before CVA).
score 4 and above to 50% of patients with ACC. One of the patients Vascular stenosis in major vessels was noted in 5of 12 patients.
with Wieneke score 2 had relapse, 3 patients with score 3 had relapse Conclusions: Risk of CVA in CP was about 14%, mostly ischemic,
whereas 2 patients with score 6 were alive without disease. occurred mostly intraoperatively or immediately postoperatively
Conclusions: Diagnosis of adrenocortical carcinoma was offered to within 24-48 hours of surgery involving tumor resection.
86% patients by modified Weiss’s criteria. We attempted to apply
Wieneke criteria retrospectively in order to compare the two scor-
ing systems. Disease relapse was correlated with MW score above EP298 / #959 CLINICAL SPECTRUM AND TREATMENT
5; although 2 patients with score 7 had no disease progression. By OUTCOME OF CHILDREN WITH LANGERHANS CELL
Wieneke criteria, patients in benign and uncertain malignant potential HISTIOCYTOSIS-A SINGLE INSTITUTIONAL EXPERIENCE FROM
category experienced relapse while 2 patients in malignant category INDIA
were well. Our findings are more in endorsement of modified Weiss
scoring system. However, we plan to undertake a prospective applica- Raghwesh Ranjan, Priyakumari Thankamony, Manjusha Nair, Binitha
tion of Wieneke system and review our results with adequate follow R, Guruprasad Cs, Prasanth Vr, Kalasekhar Vijayasekharan
up. Regional Cancer Centre, Department Of Pediatric Oncology, Thiruvanan-
thapuram, India

EP297 / #1177 CEREBROVASCULAR ACCIDENTS IN Background and Aims: Langerhans cell histiocytosis (LCH) has hetero-
PATIENTS WITH CRANIOPHARYNGIOMA. CEREBROVASCULAR geneous clinical manifestations and unpredictable clinical course and
ACCIDENTS IN PATIENTS WITH CRANIOPHARYNGIOMA outcome.We retrospectively analyze children with LCH over 12 year
period.
Prabhumallikarjun Patil1 , Rachel Gray2 , Anna Janss2 , Dolly Aguiera2 , Methods: Children aged ≤ 14 years pathologically diagnosed as
Barunashish Brahma3 , David Wrubel3 , Joshua Chern3 , Adam LCH and treated at our institute between 1st January 2004 to
Goldman-Yassen4 , David Wolf1 , Natia Esiashvili5 , Bree Eaton5 , Claire 31st December 2015 were included for retrospective analysis. The
Mazewski2 extent of disease,treatment protocol and response assessment was
1 Emory SOM, Pediatrics Hematology- Oncology, Atlanta, United States of done as per LCH III trial and three year event free survival (EFS) and
America; 2 Emory SOM, Aflac Cancer Center Pediatrics Hematology- Oncol- overall survival (OS) was calculated.
ogy, Atlanta, United States of America; 3 Emory SOM, Neurosurgery, Atlanta, Results: A total of 64 children were recruited.The median age of the
United States of America; 4 Emory SOM, Radiology, Atlanta, United States of cohort was 2 years (range: 2 months- 13 years).Male to female ratio
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S286 of S635 ABSTRACTS

was 2.2:1. Multisystem LCH (MSLCH) was seen in 32 (50%) patients in toms, headaches, hypotension, respiratory distress and lethargy (no
which 22 children had risk organ involvement. Single system (SS) LCH focal neurological deficit), hepatosplenomegaly and hyperferritine-
single site and multifocal (MF) involvement was present in 23 (35%) mia (20332 ug/L). Treatment included fluid resuscitation, inotropes,
and 9 (15%) children respectively. The most common clinical manifesta- high flow oxygen, antibiotics, IVIG and methylprednisone boluses for
tion was swelling (n=52) followed by skin rash (n=21) with bone (n=42) presumed septic shock. She improved for 72 hours followed by dete-
being the most common system involved.Nine patients (14%) had pro- rioration with worsening hyperferritenemia. Further work-up showed
gressive disease among which two patients lost to follow up and seven 8/8 HLH-2004 diagnosis criteria, pleocytosis, SARS-CoV-2 IgG anti-
patients died.Eight patients had reactivation. Three year EFS and OS bodies and EBV viral load (251766 IU/ml). Treatment included HLH
for the cohort was 68.8% and 92.5% respectively. Three year OS for 2004 induction and Rituximab. HLH improved and EBV viral load
SSLCH Single Site /MF, MSLCH without risk organ involvement was became negative. On week 6, she had HLH reactivation (increasing
100% while it was only 75% in MSLCH with risk organ involvement. ferritin, soluble CD-25 and CD-163), treatment resulted in dexametha-
Three year EFS for SSLCH Single site, SSLCHMF and MSLCH without sone, weekly etoposide, ruxolutinib and ultimately emapalumab prior
risk organ involvement was 95.7%, 62.5% and 63.6% respectively and to hematopoietic stem cell transplant (conditioning Alemtuzumab, Tre-
was 45.3% in risk organ involvement. Long term complication like dia- osulfan and Fludarabine), cyclosporine and mycophenolate for GVHD
betes insipidus, neuropsychiatric problem and dental abnormality was prophylaxis. Post transplant she has remained well with ongoing T cell
present in eleven (17%), two, five (7.8%) children respectively. mixed donor chimerims and no evidence of HLH or recurrent infection.
Conclusions: Multi system LCH with risk organ involvement was asso- Conclusions: COVID-19 could trigger HLH in children with PID, further
ciated with inferior outcome.Progressive disease on chemotherapy collaborative studies are required to further confirm this finding.
was related with high mortality which needs to be addressed especially
in resource limited countries
EP300 / #512 PRKAB2 EMERGES AS A POTENTIAL
PROGNOSTIC BIOMARKER IN PEDIATRIC ADRENOCORTICAL
EP299 / #1898 REFRACTORY HEMOPHAGOCYTIC TUMOR
LYMPHOHISTIOCYTOSIS (HLH) TRIGGERED BY CORONAVIRUS
DISEASE (COVID-19) AND EPSTEIN BARR VIRUS (EBV) Alcides Xavier1 , Luciana Veronez2 , Luiz Nagano1 , Carolina Corrêa1 ,
INFECTION IN A TEENAGER WITH AN UNDIAGNOSED Mirella Baroni1 , Luiz Santos1 , Rosane Queiróz2 , Sonir Antonini2 , José
PRIMARY IMMUNODEFICIENCY (PID) Yunes3 , Silvia Brandalise3 , Luiz Tone2 , Carlos Scrideli2
1 Ribeirão Preto Medical School, University of São Paulo, Genetics, ribeirão
Marta I Rojas Vasquez1,2 , Sneha Suresh3,4 , Niti Lotey1 , Kendra Preto SP, Brazil; 2 Ribeirão Preto Medical School, University of São Paulo,
Lemirre5 Pediatrics, Ribeirão Preto SP, Brazil; 3 Boldrini‘s Children Center, Pediatrics,
1 University of Alberta, Pediatric Oncology, Edmonton, Canada; 2 Stollery Campinas SP, Brazil
Children’s Hospital, Pediatric Hematology Oncology, Edmonton, Canada;
3 Stollery Children’s Hospital, Pediatric Immunology, edmonton, Canada; Background and Aims: Paediatric adrenocortical tumour (ACT) is
4 University of Alberta, Pediatrics, Edmonton, Canada; 5 Stollery Children’s a rare neoplasm with high heterogeneity. Different from adult ACT,
Hospital, Pediatric Oncology, Edmonton, Canada anatomopathological parameters are considered inaccurate in predict-
ing patient’s prognosis, which reflects difficulties in determining the
Background and Aims: Primary (familial HLH) results from an inher- progression of the disease, treatment strategies and tumour biology.
ited immune disorder and secondary from a critical illness (autoim- AMP-activated protein kinases (AMPK), which have the PRKAB2 gene
munity, malignancies or infection) that induces an uncontrollable as a producer of one of the subunits of its complex, exert crucial activ-
excessive immune response resulting in multiorgan failure. EBV can ities for the maintenance of embryonic development, and are strongly
trigger HLH and recently COVID-19 has been reported in few adults. associated with the main hallmarks of cancer. Thus, this study aimed to
We present a HLH case triggered by COVID-19 and EBV in a teenager evaluate the clinical value of the expression of PRKAB2 in children with
with undiagnosed PID. ACT.
Methods: Case report Methods: Gene expression was determined by Real-time PCR (RT-
Results: A 14-year-old female had PMH: colitis, perianal abscesses, qPCR), RNA-sequencing (RNA-seq), and in silico evaluation of two
meningitis, mild varicella post-vaccine, short stature and depres- public datasets of paediatric cases of ACT. CEMITool package was used
sion. Immunological work-up was not conclusive for any specific PID: to identify co-expression gene modules and hub genes using data of our
immunodeficiency panel, NOBI, RTE, Th17, protein vaccine response sequenced samples. Association analysis between PRKAB2 and clinical
and Immunoglobulins normal. Appropriate memory to naive CD4/CD8. features were carried out using Mann-Whitney and log-rank tests.
Low memory B and T reg cells. Genetic Testing Panel PID and whole Results: Co-expression analysis of the RNA-seq data of 14 paediatric
exome sequencing - no causative mutation. FH: recurrent infections tumour samples from our cohort identified the PRKAB2 as a hub gene
and autoimmune disorders in immediate family. She presented early in ACT. In addition, validation analysis of a higher number of sam-
in the pandemic with five days: fever, rash, gastrointestinal symp- ples of our cohort of Brazilian patients (n=56) and data expression of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S287 of S635

the public datasets GSE76019 (n=34) and GSE76021 (n=29) revealed inhibition of Wnt/beta-catenin is shown to reduce NCI–H295 prolifer-
the association between lower levels of PRKAB2 with the ACT ation. Although either TGFbeta ligands and PNU-74654 (50uM) were
unfavourable events recurrence and/or death (p=0.009, p=0.011 and effective independently, the combination therapy had a higher impact
p=0.048, respectively). Furthermore, the underexpression of PRKAB2 in cell viability (P=0.03).
was significantly associated with lower 5-year event-free (0.002) and Conclusions: Collectively, our results suggest that TGF-beta signaling
overall (p=0.003) survival in our cohort (n=56). might have a role in pediatric act progression and in activating the
Conclusions: Collectively, our results suggest that PRKAB2 gene may Wnt/beta-catenin pathway, which could be useful as a potential combi-
be an important player in ACT biology and a promising predictive natorial therapeutic strategy (FAPESP: 2014/203410 - 2018/044770).
marker of prognosis for paediatric patients with ACT. (Grants FAPESP:
2021/10702-9 and 2014/20341-0)
EP302 / #947 OUTCOMES OF CHILDREN WITH
EXTRACRANIAL MALIGNANT RHABDOID TUMORS TREATED
EP301 / #1933 TGF-BETA PATHWAY ACTIVATION AT A TERTIARY CARE CENTRE IN INDIA
COMBINED WITH WNT/BETA-CATENIN INHIBITION AS A
POTENTIAL THERAPEUTIC STRATEGY FOR PEDIATRIC Vignesh Subramani1 , Badira Cheriyalinkal Parambil2 , Maya Prasad2 ,
ADRENOCORTICAL TUMORS Tushar Vora2 , Venkata Rama Mohan Gollamudi2 , Mukta Ramadwar3 ,
Nehal‘ Khanna4 , Siddhartha Laskar4 , Sneha Shah5 , Sajid Qureshi6 ,
Luciana Veronez1 , Carolina Corrêa2 , Régia Lira3 , Pablo Chagas2 , Girish Chinnaswamy2
Keteryne Silva2 , Mirella Baroni2 , Luiz Nagano2 , Paula Cristina 1 Tata Memorial Hospital, Homi Bhabha National Institute, Paediatric
Santos4 , Rosane Queiróz1 , José Yunes5 , Silvia Brandalise5 , Sonir Oncology, MUMBAI, India; 2 Tata Memorial Hospital, Homi Bhabha National
Antonini1 , Luiz Tone1 , Carlos Scrideli1 Institute, Paediatric Oncology, Mumbai, India; 3 Tata Memorial Hospital,
1 Ribeirão Preto Medical School, University of São Paulo, Pediatrics, Ribeirão Homi Bhabha National Institute, Pathology, Mumbai, India; 4 Tata Memo-
Preto SP, Brazil; 2 Ribeirão Preto Medical School, University of São Paulo, rial Hospital, Homi Bhabha National Institute, Radiation Oncology, Mumbai,
Genetics, Ribeirão Preto, Brazil; 3 Federal University of Triângulo Mineiro, India; 5 Tata Memorial Hospital, Homi Bhabha National Institute, Nuclear
Division Of General Pathology, Uberaba, MG, Brazil; 4 Ribeirão Preto Faculty Medicine, Mumbai, India; 6 Tata Memorial Hospital, Homi Bhabha National
of Philosophy, University of São Paulo, Psychology, Ribeirão Preto, SP, Brazil; Institute, Paediatric Surgery, Mumbai, India
5 Boldrini‘s Children Center, Pediatrics, Campinas SP, Brazil

Background and Aims: Extracranial malignant rhabdoid


Background and Aims: Pediatric adrenocortical tumors (ACT) are tumours(MRT) are rare childhood cancers with a poor outcome.
rare and aggressive tumors with generally poor prognosis in advanced We report our experience in children with extracranial MRT treated at
stages and limited treatment options. In this context, the aim of a tertiary hospital in India.
our study was to search for new potential therapeutic targets or Methods: Retrospective analysis of children (<15 years) with
approaches to improve treatment effectiveness by using an in-house pathologically confirmed extracranial MRT, treated between Jan-
RNA sequencing of ACT samples. uary 2011 to December 2020 were performed. Staging was
Methods: Total RNA of ACT tissues from 14 Brazilian patients were by 18 FDG-PET/CT. Initial chemotherapy regimen was adapted
sequenced in order to search for differentially expressed genes (DEGs) from the hospital Ewing sarcoma protocol (Vincristine, Ifos-
between ACT cases that presented (n=5) or not (n=9) recurrence famide/Cyclophosphamide,Etoposide, Doxorubicin) and from 2017
and/or death. Gene expression was validated in additional 56 ACT onwards, 8 cycles of chemotherapy (alternating cycles of Vincristine,
samples by RT-qPCR. In vitro, cell viability and colony formation of Doxorubicin, Cyclophosphamide/Ifosfamide,Carboplatin,Etoposide)
NCI-H295R were evaluated after stimulation with TGF-beta recombi- were administered.Local treatment at 10-12 weeks included surgery
nants alone or in combination with the inhibitor of Wnt/Beta-catenin and/or radiotherapy. Survival analysis was performed by Kaplan-Meier
(PNU-74654). DESeq2 package, Mann-Whitney test, Kaplan-Meier Analysis, analysis of prognostic factors by cox proportional-Hazard
curves and p<0.05 were used for analysis. model(IBM SPSSTM v 24.0).
Results: Among the DEGs, we identified the TGFBR2 as one of the Results: A total of 78 children were diagnosed to have extracra-
top 50 downregulated gene in the cases of ACT with unfavorable nial MRT during the study period. Of these, 25/78 were treated
outcomes. Further validation in a larger cohort (n=56) revealed the with a curative intent and 53 children were palliated in view of
association between TGFBR2 underexpression with worse 5-years disseminated disease. Median age was 25 months(16-34months)
event-free (56% versus 97%) and overall survival (69% versus 97%) and M:F was 1:1.5. Six patients (24%) had renal MRT and nineteen
(P<0.01). In vitro, stimulation of NCI-H295R cells with TGF-beta lig- patients(76%) had extrarenal MRT. Five of 25 patients had metastatic
ands (10ng/ml) decreased cell viability (P=0.03) and colony formation disease(20%) at presentation. Local treatment was surgery in 5(20%),
(P<0.02), which were accompanied by an increase in MYC expression, a radiotherapy in 8(32%) and surgery plus radiotherapy in 9(36%). At
Wnt target-gene. Then, we evaluated whether the combination of TGF- a median follow-up of 14 months(6-21months), 3year EFS/OS was
beta activation and Wnt inhibition would enhance this effect, since the 20.753±5.3%(95%CI:10.28-31.22)/26.7± 6.75%(95%CI:13.4-39.9).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S288 of S635 ABSTRACTS

Among all variables analysed for prognosis in non metastatic tumors, held for discussion of the initiative, regional networks and data
use of adjuvant radiotherapy alone had prognostic impact on EFS ( capture.
p=0.035). Conclusions: This report shows the feasibility of implementing an
Conclusions: Extracranial MRT is a highly aggressive tumour in young international tumor board for low-middle-income countries, focused
children, with very poor outcomes. Radiotherapy as part of local on complex cancers where available expertise is limited. Further
treatment showed some benefit in survival. Further understanding of evaluation of how this translates into final patient management is
the biology of this disease and newer therapeutics are necessary to warranted.
improve the survival of children with extracranial MRT.

E-Poster Topic: AS05 SIOP Scientific programme / AS05.m Brain


EP303 / #1647 MULTIDISCIPLINARY TUMOR BOARD FOR Tumours
THE LATINAMERICAN PEDIATRIC ONCOLOGY GROUP (GALOP)
RARE TUMOR INITIATIVE E-POSTER VIEWING

Milena Villarroel1 , Nadia Secchieri2 , Doris Calle Jara3 , Ligia Fu4 , EP304 / #809 THE OUTCOME OF TANDEM HIGH-DOSE
Marcela Cordova5 , Bruno Cuturi6 , Roy Rosado7 , Nelson Aponte8 , CHEMOTHERAPY FOR PATIENTS WITH MEDULLOBLASTOMA
Agustin Contreras9 , Ricardo López-Almaraz10 , Ana Rossa11 , Beatriz IN INFANT AND YOUNG CHILDREN: A SINGLE-CENTER STUDY
De Camargo12
1 Hospital Luis Calvo Mackenna, Pediatric Oncology, Santiago, Chile, Wonkee Ahn, Jung Woo Han, Seungmin Hahn, Chuhl Joo Lyu
Pediatric Oncology, Santiago, Chile; 2 Hospital de Câncer Infantoju- Yonsei Cancer Center, Department Of Pediatrics, Seoul, Korea, Republic of
venil de Barretos, Pediatric Oncology, Barretos, Brazil; 3 Hospital del
Niño Dr. Francisco Icaza Bustamante, Pediatric Oncology, Guayaquil, Background and Aims: Medulloblastoma is the most common malig-
Ecuador; 4 Hospital Escuela Universitario, Pediatric Oncology, Teguci- nant brain tumor of childhood, accounting for 25% to 35% of all
galpa, Honduras; 5 Hospital Regional de Talca, Pediatric Oncology, Talca, intracranial tumors. Multimodality therapy including surgery, radio-
Chile; 6 Hospital Pereira Rossell, Hemato Oncology, Montevideo, Uruguay; therapy, and chemotherapy has been treated for patients with medul-
7 Unidad Nacional de Oncología Pediatrica, Pediatric Hematology-oncology, loblastoma, the survival is affected by age, metastatic stage at diagno-
Guatemala, Guatemala; 8 Fundación HOMI, Hospital pediátrico la Miseri- sis, and resection status after surgery. Young children under 3 years
cordia, Oncology Unit, Bogotá, Colombia; 9 Clinica Mar Caribe, Pediatric old are considered to avoid radiotherapy due to neurodevelopmen-
Oncology, Santa Marta, Colombia; 10 Hospital Universitario de Cruces. tal problems. Alternative therapeutic options are needed to overcome
IIS Biocruces Bizkaia, Unidad De Hematología Y Oncología Pediátrica, that.
Barakaldo, Spain; 11 Hospital Garrahan, Pediatric Oncology, Buenos Aires, Methods: From April 2005 through June 2021, we registered patients
Argentina; 12 Instituto Nacional de Cáncer, Pediatric Oncology, Rio de who were below years old and diagnosed with medulloblastoma and
Janeiro, Brazil treated with Korean Society Pediatric Neurooncology (KSPNO) Proto-
col at Yonsei Cancer Center. Six cycles of chemotherapy and tandem
Background and Aims: GALOP’s Rare Tumor Initiative was launched high-dose chemotherapy(HDCT) with autologous stem cell transplan-
in 2011 to improve management and outcomes of children and ado- tation(ASCT) were administered. Outcomes and complications of them
lescents with infrequent tumors in latinamerica. To address lack of were analyzed retrospectively.
standardized therapeutic guidelines, a virtual tumor board was estab- Results: 12 patients were enrolled, and histologic subgroup was
lished for multidisciplinary discussion of these patients. Our aim is to confirmed in 10 patients (4 classic, 2 desmoplastic/nodular, 4 medul-
describe the activity of this tumor board. loblastoma with extensive nodularity), and molecular subgroup of sonic
Methods: Retrospective analysis of the Rare Tumor group monthly hedgehog was confirmed in 9 patients. All patients underwent induc-
meetings, held through the web platform of Cure4Kids, from Septem- tion chemotherapy after tumor resection, and 1 HDCT, and only 9
ber 2013 to March 2022. Cases were presented virtually, including patients underwent 2 HDCT. 2 patients experienced a relapse of lep-
clinical history, complementary studies, and questions for discussion. tomeningeal seeding before the second HDCT, one of them died due
Literature review was performed, and international experts were to the progression of the disease. One patient died from treatment-
invited where possible. related mortality during the first HDCT. Median days to neutrophil
Results: The Cure4Kids’s Rare Tumor Group has 151 registered engraftment are 13.5(IQR 11.7-15.7) and 12(IQR 11-13) days, median
participants from 18 countries, mostly from Latinamerica and days of fever after ASCT are 5.5(IQR 5.0-7.5) and 4(IQR 2-6), in first
Caribbean. Ninety-two cases of de-identified patients from 11 and second HDCT, respectively. Median follow-up days are 3.67 (IQR
countries, were discussed in 74 meetings, by a median of 10 partic- 2.32-6.87) years and 9 patients (75%) are under complete remission.
ipants per session. Fifty-three cases corresponded to rare tumors, Conclusions: Tandem HDCT following chemotherapy to avoid radio-
14 rare sarcomas, 8 cases of tumors with unusual presentation therapy under 3 years old showed promising results in relapse rate. But
and 17 cases for diagnosis. Six administrative meetings were modifications to reduce the risk of HDCT should be considered.
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ABSTRACTS S289 of S635

EP305 / #348 HIGHLIGHTING THE USE OF METHYLATION system (CNS) tumors present a greater nutritional risk than others.
PROFILING IN MOLECULAR SUBGROUP OF This study aimed to determine the nutritional status, daily food con-
MEDULLOBLASTOMA PATIENTS IN A DIFFERENT CLINICAL sumption amounts, and nutritional treatments applied in hospitalized
OUTCOME AND SURVIVAL patients with CNS tumors.
Methods: CNS cancer patients admitted to the Ege University Hospital
Abrar Aljunaid1 , Ibraheem Abosoudah1 , Amal Alseraihy1 , Hassan pediatric oncology clinic between January 2018 and December 2021
Altrabolsi1 , Mohammed Bayoumy1 , Malak Althagafi2 (62 patients; n=29, 46.8% F, n=33, 53.2% M; median age:8.0 (1-17
1 King Faisal Specilaist hospital and research center, Pediatric Oncology, years) were followed in this study. The nutritional status was evalu-
Jeddah, Saudi Arabia; 2 king fahad medical city, Pathology, riyadh, Saudi ated within 24 hours of hospitalization, and nutritional therapy was
Arabia started for the patients who needed it. Height, weight, upper-middle
arm circumference (MUAC)), daily food consumption were taken.
Background and Aims: Medulloblastoma (MB) is aggressive embry- Results: Malnutrition was observed in 46,8% (n =29), moderate risk
onal tumor representing the most frequent primary malignant brain 53.2%(n=33) according to StrongKids. Height for age was <25% in
cancer in children. It classify to four subgroup: wingless (WNT), 33.9% (n=21) of the children, 25-75% in 35.5% (n=22) and >75% in
sonic hedgehog (SHH), group 3, and group 4. Gene expression array, 30.7% (n=19). Weight for age ranged from <25% for 22.6% (n= 27),
next-generation sequencing, and methylation profiling revealed that 25-75% for 22.6% (n=14), and >75% for 34% (n=21). BMI according to
medulloblastomas harbor a paucity of mutations,. Afew data from age of 43.5% (n= 27) <25%, 37.1% (n=23) ranged from 25-75%, 19.4%
Saudi Arabia (SA) and Arab world studing the molecular subgroup and (n=17) >75% is in the range. According to MUAC measurements,
its clinical implications thus we conduct this study to illuminate our 45.2% (n=28) of the children were <25%, 35.5% (n=22) 25-85%, and
population gentic background and implication in clinical outcome and 19.3% (n=12) >85%. Children can get mean 39.1% (min:0,max:115) of
surivival. their daily energy requirements and 38.4% ( mean:0, max:157) of their
Methods: In this study, we analyzed 23 samples of MB treated in a sin- protein requirements with oral foods. Of the children (n=55) 67.2%
gle institute through genome-wide DNA methylation and targeted next (n=37) received <50% of their daily energy requirement and 77.3% of
generation sequencing. them <50% of their protein requirement by orally. 29% (n=18) of the
Results: we conduct an analysis of MB-DNA methylation patterns in children were fed with ONS, 32.3% (n=20) fed by EN, and 3.2% (n=2)
cohort using archival biopsy material ( n = 41). Of the 23 materials fed by TPN.
available for methylation assessments, 13 could be classified into the Conclusions: The frequency of malnutrition is common in children with
major DNA methylation subgroups. We preformed methylation profil- CNS tumors. Multidisciplinary teams regarding nutrition should mon-
ing for our medulloblastoma patients from our archival FFPE biopsy of itor pediatric cancer patients, and nutritional intervention should be
post treated patients in our institute. Our assessments revealed high performed without delay when necessary.
survival rates observed for WNT tumors with exclusively 100% sur-
vival and more commonly in female. Strikingly more than half of our
population is either group 3 or group 4. group 3 showed the most EP307 / #1648 IMPROVEMENT IN SURVIVAL BUT STILL
aggressive outcome with all cases died due to relapse. Also we found LOW AND SLIGHT INCREASE IN THE INCIDENCE TREND RATE
that group 4 showing high metastatic and relapse rate with nearly 75% OF PEDIATRIC BRAIN TUMORS IN ARGENTINA: ARGENTINE
relapse rate complicated by death. PEDIATRIC ONCOLOGY REGISTRY-INC(ROHA)2000-19
Conclusions: In conclusion, we demonstrate that DNA methylation
profiling empower the sub-classification of four disease sub-groups Lorena Baroni1 , Agustina Chaplin2 , Daniel Alderete1 , Blanca Diez3 ,
in archival FFPE biopsy material from MB patients. Moreover, we Mercedes Garcia Lombardi4 , Florencia Moreno5
conclude that the incorporation of DNA methylation biomarkers can 1 Paediatric Hospital Dr Juan P Garrahan, Hematology Oncology, Buenos
significantly improve the accuracy of current disease-risk stratification Aires, Argentina; 2 National Cancer Institude-Argentina, Oncopediatric
These findings have important implications for future clinical disease Registry, Buenos Aires, Argentina; 3 FLENI, Neuroncology, CABA, Argentina;
management in MB cases across the Saudi Arabia and Arab world. 4 Hospital de Niños Ricardo Gutierrez, Oncology, CABA, Argentina;
5 National Cancer Institude-Argentina, Oncopediatric National Program,
Buenos Aires, Argentina
EP306 / #1331 MALNUTRITION RISK AND INTERVENTIONS
FOR CHILDREN WITH CENTRAL NERVOUS SYSTEM TUMORS Background and Aims: Objective: To present the incidence and sur-
vival trends of the pediatric brain tumor (BT) population in Argentina
Derya Hopanci Bicakli, Eda Ataseven, Mehmet Kantar between 2000-19.
Ege University Faculty of Medicine, Pediatric Oncology, Izmir, Turkey Methods: ROHA is a population-based hospital registry that has been
active since 2000 and is part of the National Pediatric Program at
Background and Aims: Nutrition screening and intervention time are the Ministry of Health’s National Institute of Cancer. ROHA’s network
vital during childhood cancer treatment.Though some central nervous data comes from different sources; most are reported by pediatric
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S290 of S635 ABSTRACTS

oncology units from all regions in the country. Estimated coverage is Methods: 39 patients (17 females, median RT age 8 years) treated
92%. Age-standardized incidence rate (ASR) trends were calculated for localized(29) or metastatic(10) medulloblastoma in 2000-2020 and
using Joinpoint regression analysis, which was used to assess trends with spirometric assessment, were considered. Treatment included:
of age-standardized incidence rates over time, and to estimate their SIOP-like-PNET IV(19), high-risk protocol(19), infant protocol with-
annual percentage of change (APC) with 95% of confidence intervals. out RT(1). CSI doses were: 23.4Gy(20), 31.2Gy(8), 36Gy(6) and
OS was calculated using the Kaplan-Meier method and was reported at 39Gy(4); 4 received protons and 34 photons(9 VMAT, 25 3D), 11
5 years (between 2005-14), and 3 years (between 2000-04; 2005-09; hyperfractionated-accelerated-RT; 33 had 6 median CCNU cycles; 6
2010-12). APBSCT.
Results: 27,016 new cases of cancer were recorded between 2000- Results: Median follow-up: 98 months. All patients performed at least
19 with ASR 131.6 (95% CI [124.5-138.6]); 19.7% (5,320) were BT one spirometry at median 5 years after RT. Eight (20.6%) had mildly
with ASR 24.9 (95% CI [21.8-27.9]). Between 2000 and 2019, ASR in pathological spirometries, 8 Forced Vital Capacity (FVC%)<90%. RT
BT increased slightly (APC: 0.7%; 95% CI [0.2; 1.2]; p=0.01). OS at age was not associated with FVC%/ PEF% (p=0.319 and 0.405). A
5 years for BT was 56.4% (54.4-58.3) between 2005-2014 (n:2453). lower Peak Expiratory Flow(PEF%) was marginally associated to APB-
OS at 3 years was 50.5% (47.8-53.0) between 2000-05 (n:1421) com- SCT group (p=0.062) with FVC%(≤90% vs >90%) similar but less
pared with 58.9% (56.4-61.4) between 2006-11 (n:1515) and 63.8% significant(p=0.163). Median FVC%/PEF% were higher in the CCNU-
(61.2-66.3) between 2012-16 (n:1413). group without reaching significance (p=0.436 and 0.062): this was a
Conclusions: Discussion and Conclusion: The incidence of BT in standard-risk group not receiving APBSCT nor higher RT doses. Even
Argentina is similar to other countries in Latin America; however, it though the lung volume encompassed by 5-10 Gy isodoses was greater
remains lower than in North American and European countries. The in VMATvs3D RT(p<0.001 and p=0.015), there were no significant
increase in the BT trend could be related to a better registry of these differences in ventilatory parameters. FVC%/PEF% were negatively
cases. Although survival outcome improvement was observed, it is still associated to CSI dose. Since no relevant lung volume is involved in high
lower than in most developed countries. doses, a multifactorial etiology could be speculated.
Conclusions: Preliminary data show no significant FVC%/PEF% reduc-
tion. Small sample size and differences in spirometry techniques
EP308 / #117 TREATMENT-INDUCED PULMONARY impose larger cohorts accrual to elucidate potential treatment-
TOXICITY IN PATIENTS WITH MEDULLOBLASTOMA: A induced pulmonary impairment in the pediatric population thus vali-
RETROSPECTIVE ANALYSIS ON 2 ITALIAN INSTITUTIONS’ dating the use of spirometry during treatment/follow-up.
COHORTS

Veronica Biassoni1 , Alessandra Busia2 , Francesco Barretta3 , Lorenza EP309 / #1926 DIFFERENCES IN LONG-TERM
Gandola4 , Sabina Vennarini4 , Silvia Meroni5 , Angela Mastronuzzi6 , NEUROPSYCHOLOGICAL PROFILE IN PEDIATRIC SURVIVORS
Giada Del Baldo6 , Silvia Chiesa7 , Emanuele Pignoli5 , Monica OF POSTERIOR FOSSA TUMOR (TFP) TREATED WITH PROTON
Terenziani1 , Emilia Pecori8 , Elisabetta Schiavello1 , Maura Massimino1 RADIOTHERAPY (PRT) COMPARED TO PHOTON
1 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Unit, Milan, RADIOTHERAPY (XRT)
Italy; 2 Fondazione IRCCS Istituto Nazionale dei Tumori, Pneumology Unit,
Milan, Italy; 3 Fondazione IRCCS Istituto Nazionale dei Tumori, Clinical Cristina Boix1 , Laura Mangado1 , Ofelia Martinez2 , Ester
Epidemiology And Trial Organization, Milan, Italy; 4 Fondazione IRCCS Gomez-Navarro1
Istituto Nazionale dei Tumori, Pediatric Radiotherapy Unit, Milan, Italy; 1 Hospital Sant Joan de Deu, Neurology, Barcelona, Spain; 2 Sant Joan de
5 Fondazione IRCCS Istituto Nazionale dei Tumori, Medical Physics Unit, Déu, Oncology, Sant Joan d’Espí, Spain
Milan, Italy; 6 IRCCS Bambino Gesù Children’s Hospital, Department Of
Paediatric Haematology/oncology, Rome, Italy; 7 Fondazione Policlinico Uni- Background and Aims: Ependymoma (EP) is a malignant glial tumor
versitario Agostino Gemelli IRCCS, Oncologic Radiotherapy, Department of the Central Nervous System (CNS). Represents 10% of all Pos-
Of Diagnostic Imaging, Oncologic Radiotherapy And Hematology, Rome, terior Fossa Tumors (PFT) in children. The standard treatment for
Italy; 8 Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Pediatric children with histology of EP, both supratentorial and infratentorial,
Radiotherapy Unit, Milan, Italy consists of maximal resection Surgery (CIR), followed by treatment
with Radiotherapy (RT) and complementary forms with Chemotherapy
Background and Aims: Incidence of iatrogenic pulmonary toxicity (CT). Neuropsychological deficits are common and have a multifactorial
is around 20%. Apart from bleomycin fibrosis, the role of lomus- origin.
tine, HD-thiotepa, autologous stem-cells transplantation(APBSCT) and Methods: The aim was to analyze whether new treatment protocols
their synergy with craniospinal irradiation(CSI) are unclear. To eluci- used with Proton Radiotherapy (PRT) minimize the vulnerability of
date their role in lung-function impairment, we retrospectively eval- long-term cognitive dysfunctions on cognitive functions, compared
uated 39 medulloblastoma patients treated at INT-Milan and OPBG- to the traditional treatment of Photon Radiotherapy (XRT), which is
Rome. already known to be toxic on neurocognitive domains.
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ABSTRACTS S291 of S635

Results: A sample of pediatric patients followed by the Neurology focused. Variables such as the number of surgery and / or RT treatment
Service of the Sant Joan de Déu Hospital, between 2002 and 2021, were not related to neuropsychological variables.
with a history of EP that differed in the modality of treatment with Conclusions: Executive dysfunction and emotional dysregulation, as
radiotherapy (XRT vs PRT) was included. Cognitive test scores for intel- well as attention deficit it appears to be the neuropsychologycal profile.
ligence, memory, visuoconstructive functions and Executive Functions It is important to establish early rehabilitation programs.
(EF) were obtained. The analyses compared the neuropsychological
assessments of each subject greater than or equal to 3 years after
treatment with RT between the treatment modality groups and ages EP311 / #1598 METASTASIC MEDULLOBLASTOMA:
of onset of TFP. The cognitive functions of 23 subjects (6 XRT, 7 PRT RADIOLOGICAL FEATURES AND ITS CORRELATION WITH
and 10 CIR) were analyzed. The PRT group obtained superior results in MOLECULAR SUBGROUPS AND DISSEMINATION PATTERN
relation to the Intelligence Quotient (IQ) compared to the XRT group.
The age of onset of less than 3 years turned out to be a determining Marina Caballero Bellón1 , Jordi Muchart Lopez2 , Vicente
factor on EF, regardless of the RT treatment received. Santa-María1 , Cinzia Lavarino3 , Nagore Gene4 , Ofelia Martinez5 ,
Conclusions: Our findings presuppose the protective factor of PRT in Andres Morales6 , Marta Perez-Somarriba1
relation to XRT in cognitive functions, but at the same time, highlight 1 Pediatric Cancer Center Barcelona (PCCB), Hospital Sant Joan de Déu
the impact of brain damage at an early age of less than or equal to 3 Barcelona, University of Barcelona, Pediatric Hematology And Oncology,
years on long-term neuropsychological sequelae. Barcelona, Spain; 2 Hospital Sant Joan de Déu, Radiology, Esplgues de Llo-
bregat, Spain; 3 Institut de Recerca Sant Joan de Deu, Tractament Del Càncer
Pediàtric, Barcelona, Spain; 4 Hospital Sant Joan de Déu, Pediatric Cancer
EP310 / #1951 PEDIATRIC CRANIOPHARYNGIOMA: Center Barcelona (pccb), Barcelona, Spain; 5 Sant Joan de Déu, Oncology,
DESCRIPTION OF THE LONG-TERM NEUROPSYCHOLOGICAL Sant Joan d’Espí, Spain; 6 Hospital Sant Joan de Deu, Oncology, Barcelona,
AND BEHAVIORAL PROFILE Spain

Cristina Boix1 , Sara Escudero1 , Andres Morales2 , Federico Ramos3 Background and Aims: Medulloblastoma (MB) is the most frequent
1 Hospital Sant Joan de Deu, Neurology, Barcelona, Spain; 2 Hospital Sant malignant childhood brain tumor. At least, four molecular subgroups
Joan de Deu, Oncology, Barcelona, Spain; 3 Sant Joan de Déu Hospital, have been described (WNT, SHH, group3, group4), which are associ-
Neurology, Esplugues, Spain ated with different biology, prognosis, specific MRI characteristics and
patterns of metastatic dissemination. We aimed to determine the imag-
Background and Aims: Craniopharyngioma is a type of benign brain ing features of metastatic MB, its molecular classification and their
tumor that affects the central nervous system (CNS). It has an outcomes.
intrasellar and / or suprasellar location, around the midline. It rep- Methods: Retrospective institutional analytic-observational study
resents between 3 and 5% of childhood brain tumors. Treatment conducted from January 2004-January 2022 in a tertiary-care center.
usually includes neurosurgery and radiation therapy (RT). Hypotha- Pediatric patients with metastatic medulloblastoma at disease onset
lamic involvement of the tumor and secondary injuries to treatment were included. We collected epidemiological and clinical characteris-
lead to a high rate of hypothalamic obesity and neuropsychological tics, treatment received, and outcomes. The molecular subgroup was
deficits. Such long-term cognitive deficits will significantly affect aca- determined by its methylation profile.
demic performance and quality of life. T The main objective is to Results: In the study period, from sixty-three identified patients,
evaluate and determine a neuropsychological profile and the long-term 17 (26.9%) were metastatic. The median age at diagnosis was 5.1
academic development of children with craniopharyngioma compared years (range 2.1-17.5 years), and 58.8% were male. According to
to a healthy control group. In turn, the aim is to study the relationship histopathologic classification, fifteen patients (93.8%) were classic,1
between medical variables and cognitive deficits. (6.3%) desmoplastic. Molecular subgroup analysis showed 2 WNT
Methods: Between 2013 and 2020, a total of 16 subjects were (12.5%), 1 SHH (6.3%), 3 (18.8%) group 3 (G3) and 5 (31.2%) group
assessed. 8 subjects (6 boys and 2 girls) were included. The inclusion 4 (G4). Four patients (25%) were classified as G3/G4 and 1 (6.3%) as
criteria were: age between 6 and 18 years, and have a complete neu- mixed. Five patients (29.4%) were M2 and 12 patients (70.6%) were
ropsychological assessment of 1-2 years after the end of active medical M3 according to Chang staging system. Localization in the cerebel-
treatment. The healthy control groupof the was matched by age and lar hemispheres was only observed in the SHH patient. G3 tumors
gender with the craniopharyngioma grup. characteristically presented homogeneous contrast enhancement. All
Results: Show statistically significant differences beetwen two groups WNT, G3 and G4 were located in IV ventricle. We found no association
in quotient intellectual (p-value = .010), focused attention (p-value = between molecular subgroup and metastatic site (intracranial vs spinal,
.005), working memory (p-value = .021), speed processing (p-value =. Fisher test, p=0.45). All patients with a metastatic lesion in the third
010) and, emotional control (p-value = .006). Only age (onset less than ventricular infundibular recess were G4. Four patients died, all of them
8 years) showed a high correlation with QI, language and attention were either G3 or G3/G4.
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S292 of S635 ABSTRACTS

Conclusions: Our results support what has been previously reported. EP313 / #1586 SURVIVAL OUTCOMES OF CHILDREN AND
Molecular subgrouping can be suggested based upon certain radi- ADOLESCENTS WITH BITHALAMIC GLIOMAS: MULTICENTRIC
ologic characteristics. In our cohort, the presence of a metas- EXPERIENCE AND POOLED DATA ANALYSIS
tasis in the infundibular recess suggests group 4 MB. However,
the dissemination pattern not was associated with any particular Anoop Cherungonath1 , Julia Cockle1,2 , Ajla Wasti1,2 , Erika Pace3 ,
subgroup. Marta Perez-Somarriba1,4 , Bassel Zebian5 , Samantha Hettige6 , Safa
Al-Sarraj7 , Leslie Bridges8 , Thomas Jacques9,10 , Chris Jones11 , Andres
Morales4 , Henry Mandeville2,12 , Paola Angelini1 , Kavitha Srivatsa1 ,
EP312 / #239 OUTCOME OF CHILDREN AND ADOLESCENTS Sucheta Vaidya1,2 , Lynley Marshall1,2 , Fernando Carceller1,2
WITH INTRACRANIAL GERMINOMA TREATED WITH 1 The Royal Marsden NHS Foundation Trust, Children & Young People’s Unit,

CHEMOTHERAPY AND REDUCED DOSE IRRADIATION: A London, United Kingdom; 2 The Institute of Cancer Research, Division Of
BRAZILIAN CONSORTIUM Clinical Studies, London, United Kingdom; 3 The Royal Marsden NHS Foun-
dation Trust, Department Of Radiology, London, United Kingdom; 4 Hospital
Andrea Cappellano1 , Natalia Dassi1 , Bruna Mançano2 , Daniela Sant Joan de Deu, Pediatric Oncology, Barcelona, Spain; 5 Kings College Hos-
Almeida3 , Sergio Cavalheiro4 , Sidnei Epelman5 , Patricia Dastoli4 , pital, Department Of Neurosurgery, London, United Kingdom; 6 St.George’s
Marcos Costa4 , Jardel Nicacio4 , Michael Chen6 , Nasjla Silva1 , University Hospital, Department Of Neurosurgery, London, United Kingdom;
Jonathan Finlay7 7 Kings College Hospital, Department Of Clinical Neuropathology, London,
1 IOP/GRAACC/UNIFESP, Pediatric Oncology, Sao Paulo, Brazil; United Kingdom; 8 St.George’s University Hospital, Department Of Clinical
2 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil; Neuropathology, London, United Kingdom; 9 Great Ormond Street Hospital
3 IOP/GRAACC/UNIFESP, Nursing- Pediatric Oncology, f, Brazil; for Children NHS Foundation Trust, Department Of Histopathology, London,
4 IOP/GRAACC/UNIFESP, Neurosurgery, d, Brazil; 5 H Sta Marcelina/TUCCA, United Kingdom; 10 UCL GOS Institute of Child Health, Developmental Biol-
Pediatric Oncology, d, Brazil; 6 IOP/GRAACC/UNIFESP, Radiotherapy, d, ogy And Cancer Department, London, United Kingdom; 11 The Institute of
Brazil; 7 OSU- Ohio State University, Emeritus Professor Pediatrics And Cancer Research, Division Of Molecular Pathology, London, United King-
Radiation Oncology, d, United States of America dom; 12 The Royal Marsden Hospital and the Institute of Cancer Research,
Department Of Radiation Oncology, London, United Kingdom
Background and Aims: Primary central nervous system (CNS) germ
cell tumors (GCT) are rare malignancies, classified according to their Background and Aims: Bithalamic gliomas (BG) are rare brain tumours
histological components and tumor markers (TM) as either germino- with distinct molecular alterations. Prognosis is dismal. We analysed
mas or non- germinomatous germ cell tumors (NGGCT). The aim of patients with BG from two neurosurgical centres in London and two
the present study is to evaluate the outcome of germinoma patients tertiary Paediatric Neuro-Oncology Units in UK and Spain.
treated with a Brazilian CNSGCT consortium protocol. Methods: Patients aged ≤18 years with clinico-radiological diagnosis
Methods: Since 2013, 57 patients with histology and/or TM consistent of BG between 2000-2021 were eligible. Those with unilateral involve-
with CNSGCT were diagnosed, 43 cases of germinoma with/without ment at presentation were excluded. Clinical, radiological, histological,
HCGß levels ≤ 200mIU/ml, five between 100-200mIU/ml. The treat- molecular, and survival data were collected. Pooled data analysis of
ment plan consists of four cycles of carboplatin/ etoposide fol- reported cases with individualised data was performed.
lowed by 18Gy ventricular field irradiation and primary site(s) boost Results: Sixteen patients were included. Median age 6.3 years
up to 30Gy, and 24Gy craniospinal irradiation for disseminated (range, 4.1-17.9). Male:female ratio 1.3:1. Thirteen (81%) had his-
disease. tology at diagnosis: 7 (54%) low grade gliomas -LGG-, 6 (46%)
Results: Mean age 13.2 years (4.7-25.5y), 29 males. Diagnosis was high grade gliomas -HGG-. Seven (54%) had molecular profiling:
made by TM (n=6), biopsy (n=25) and both (n=10). Two bifocal cases, H3K27-altered (n=4), H3 wild-type (n=3). Among H3K27-altered
with negative TM were treated as germinoma. Primary tumor loca- cases: EGFR and TP53 mutations (n=1); EGFR mutation (n=1); BRAF
tion was pineal (n=18), suprasellar (n=14), bifocal (n=10) and basal V600E, PIK3CA and TERT mutations (n=1); no oncogenic drivers
ganglia/ thalamus (n=1). Fourteen demonstrated ventricular/ spinal found (n=1). Among H3-Wt cases: EGFR and TP53 mutations (n=1);
spread. Second-look surgery occurred in two patients (residual/ ter- PIK3CA and PTPN11 mutations (n=1); no oncogenic drivers found
atoma). Thirty-six patients have achieved complete responses (CR) (n=1). Fourteen (88%) underwent radiotherapy, of which 11 (79%)
after chemotherapy, seven showed residual teratoma/ scar. Radiother- received concomitant Temozolomide. One child had only chemother-
apy was performed as described, except in one case. Toxicity was apy (vincristine/carboplatin/etoposide) and another had no treat-
mostly grade 3/4 neutropenia/ thrombocytopenia during chemother- ment (parental decision). Two patients received targeted drugs post-
apy. At a median follow-up of 40 months, event-free and overall radiotherapy: sirolimus (limited tissue for molecular profiling, but
survival is 100%. mTOR activation on immunohistochemistry) and afatinib (EGFR muta-
Conclusions: This treatment is tolerable and VFI dose reduction to tion), respectively. Median overall survival (OS): 12.5 months (range:
18Gy preserves efficacy with excellent outcome, even in patients with 3.7-188); three long-term survivors: 4.5 years (no biopsy), 11 years
HCGß levels between 100-200mlU/ml. (HGG) and 15 years (LGG), respectively. No significant differences
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S293 of S635

between the survival of patients with LGG or HGG. In the pooled data EP315 / #545 EPENDYMOMA IN CHILDREN: EXPERIENCE
analysis (34 independent cases) median OS was 12.6 months (range: OF A SINGLE-CENTER IN ARGENTINA
12.4-160).
Conclusions: Survival of patients with BG is very poor regardless of Maria Cores1 , Sonia Gonzalez Palumbo2 , Sandra Colli3 , Elena De
the underlying histology, although anecdotal long-term survivors are Mateo3 , Mercedes Garcia Lombardi1
described. EGFR and mTOR pathway alterations are relatively fre- 1 Hospital de Niños Ricardo Gutierrez, Oncology, buenos aires, Argentina;
quent. Access to targeted therapies upfront should be prioritised in 2 Hospital de Niños Ricardo Gutierrez, Oncology, Buenos Aires, Argentina;
clinical trials for individuals with BG. 3 Hospital de Niños Ricardo Gutierrez, Pathology, buenos aires, Argentina

Background and Aims: Ependymomas are the third most common


EP314 / #1637 A COMPUTATIONAL FRAMEWORK FOR THE pediatric CNS tumors (6-10%). Standard of care is gross total resec-
ANALYSIS OF CHILDHOOD CANCER GENOME DATA. tion +/-radiotherapy, with 5 years overall survival rate of 60-80%. New
APPLICATIONS TO MEDULLOBLASTOMA 2021 Who classification identified 10 types of tumors based on local-
ization, histological features.and molecular characteristics. The aims
Iker Núñez-Carpintero1 , Alejandro Tejada-Lapuerta2 , Alfonso are to report experience of an Argentinian single center in diagnosis,
Valencia1 , Davide Cirillo1 treatment and outcome of patients with ependymoma; and to describe
1 Barcelona Supercomputing Center, Life Sciences, Barcelona, Spain; reviewed molecular findings.
2 Helmholtz Zentrum München, Institute Of Computational Biology, Methods: Retrospectively review of records and tumor samples of
München, Germany patients treated at Hospital de Niños Ricardo Gutierrez, between Jan-
uary 2013 and December 2020. Inmunohistochemistry and molecular
Background and Aims: The H2020 project ‘individualizedPaediatric- techniques were done to detect RELA, N- Myc, H3K27me3 status
Cure’ (iPC, GA 826121) combines knowledge-based and machine Results: From 233 patients with CNS tumors in this period, 33(14%)
learning approaches for paediatric oncology aiming to generate com- were ependymomas. 28 evaluable. Male/female ratio 1.25:1. Median
putational models to produce personalised diagnostics and therapies age 60 months (r:8-204m). Localized disease: 26 (93%). All 28 patients
recommendations within a cloud-based platform. Here we introduce undrwent surgery, achieving gross total resection in 62.9% of the
a computational framework that we have developed in the context cases. 64% received radiotherapy. Posterior fossa in 13p (46%). Ten
of the iPC project for the analysis of childhood cancer genome data. were reclassified as PF-A due to H3K27me3 mutation. Six alive in CR
The framework consists of two main components: (1) a model for (median follow up: 72 months), 2 alive with disease, 2 dead due to dis-
the generation of synthetic data and (2) a graph-based model for ease progression Three p were reclassified as PF-B and are alive in
functional analysis. Both models have been applied to the study of CR. Supratentorial 10p(36%). Five RELA positive: 4 p dead due to dis-
medulloblastoma. ease progression, 1 p alive with disease. Five p RELA negative: 3 dead
Methods: The synthetic data generation model, namely an explain- due to disease progression, 2 alive (28 qnd 48 months off treatment)
able variational autoencoder, can be used to augment and interpolate Spinal cord in 5p(18%). One with N- myc amplification. All alive without
available omic data with synthetic instances, which are automatically RDT.
annotated with confidence scores to assess their reliability. The graph- Conclusions: Poor results in patients with supratentorial tumors could
based model, namely a multilayer network, consists of multiple layers be due to lack of radiotherapy. Standard treatment at that time
of bio-entities connected through relational associations, which cap- was limited to surgery alone when CR could be achieved. Patients
ture the complexity of omic information despite the small sample with PF ependymomas had similar results to reported in the liter-
size. ature probably related to frontline radioteraphy for its treatment.
Results: We used the synthetic data generation model to study the This highlights importance of molecular biology to be able to adapt
gene expression characteristics of the four medulloblastoma sub- treatments.
groups (WNT, SHH, G3, G4) and, in particular, those underlying the
unexplored relationship between G3 and G4 subgroups. Furthermore,
thanks to the multilayer network model, we identified the minimal EP316 / #1143 MEDULLOBLASTOMA IN CHILDREN LESS
number of genes, outlined by proteogenomic data, that define molec- THAN FIVE YEARS OLD: A REVIEW OF 20 CASES FROM
ular subgroups of medulloblastoma. This work is published in iScience BRAZIL
(Núñez-Carpintero et al. 2021, doi: 10.1016/j.isci.2021.102365).
Conclusions: Both synthetic data generation and multilayer net- Natalia Dassi1 , Andrea Cappellano1 , Daniela Almeida2 , Maria Teresa
works are emerging as dominant solutions for precision medicine Alves3 , Sergio Cavalheiro4 , Patricia Dastoli4 , Marcos Costa4 , Jardel
as they enable addressing critical challenges for paediatric oncol- Nicacio4 , Frederico Silva5 , Nasjla Silva1
ogy such as privacy-preservation, small data modelling, and explain- 1 IOP/GRAACC/UNIFESP, Pediatric Oncology, Sao Paulo, Brazil;
ability and interpretation of computational analysis of genome 2 IOP/GRAACC/UNIFESP, Nursing- Pediatric Oncology, f, Brazil;
data. 3 IOP/GRAACC/UNIFESP, Pathology Department, Sao Paulo,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S294 of S635 ABSTRACTS

Brazil; 4 IOP/GRAACC/UNIFESP, Neurosurgery, Sao Paulo, Brazil; tal, Athens, Greece, Department Of Neurosurgery, Athens, Greece; 6 ’Aghia
5 IOP/GRAACC/UNIFESP, Radiology, Sao Paulo, Brazil Sophia’ Children’s Hospital Athens, Greece, Department Of Paediatric Radi-
ology (ct & Mri) And Nuclear Medicine, Athens, Greece; 7 German Cancer
Background and Aims: Medulloblastoma (MB) is the most common Research Center (DKFZ) and German Cancer Consortium (DKTK), Heidel-
malignant brain tumor in children less than three-five years. Histolog- berg, Germany, Ccu Pediatric Oncology, Heidelberg, Germany; 8 Heidelberg
ical variants consist of classic, MB with extensive nodularity (MBEN), University Hospital, Heidelberg, Germany, Pediatric Oncology, Hematology,
desmoplastic/nodular (MBDN), large-cell/anaplastic and are staged And Immunology, Center For Child And Adolescent Medicine, Heidelberg,
based on extent of resection, dissemination and more recently, molec- Germany; 9 Heidelberg University Hospital, Heidelberg, Germany, Neu-
ular stratification. The report aim is to present the outcomes of infant ropathology, Institute Of Pathology, Heidelberg, Germany; 10 Heidelberg

MB treated in a middle-income-country between 2016-2019. University Hospital, Heidelberg, Germany, Institute Of Human Genetics,
Methods: In a retrospective analysis, 20 patients were treated accord- Heidelberg, Germany
ing to a modified Head Start backbone protocol followed by myeloab-
lative chemotherapy with autologous stem cell rescue (ASCR). Radio- Background and Aims: Medulloblastoma (MB) is the most common
genomics/ Immunohistochemical correlation was used to stratify into embryonal central nervous system (CNS) tumor in children. Τhe aim
molecular subgroups: MB with wingless (WNT) activation, sonic hedge- of this study was to assess the molecular and clinical profiles of
hog (SHH) activation and non-WNT/non-SHH MB. patients with MB diagnosed in our center, identifying the risk factors
Results: Mean age 2.6 years (1.5-4.8y), 12 males. Mean time to for treatment failure.
initial symptoms to diagnosis was 3.9 months (0.2-12months). Six- Methods: Α 10years (2012-2021) retrospective analysis of the clin-
teen had complete resection, thirteen no metastasis. Fourteen were ical and molecular profile of patients with MB was performed,
classified as MB-SHH, four with P53 protein >50% and six as non- taking into account histopathological assessment, molecular data
WNT/non-SHH. Progression-free survival (PFS) and overall survival (DKFZ/Heidelberg), extent of resection and response to treatment.
(OS) across the entire cohort was 46.2% and 72% at a median follow- Results: Among 37 patients (22 males) with mean age 5.7years
up of 27.5 months. According to histology, PFS and OS for MBDN (9months-16years), surgical resection was performed in 34 (Gross
was 68.6% and 90%, for classic-MB 35% and 67.5%, respectively. Total:19, Partial:15), and only biopsy in 3 patients. Based on CSF cytol-
Two MBDN patients relapsed, both with M+ disease and radio- ogy, brain/spinal MRIs the patients were characterized as M0 (20/37
genomics/immunohistochemical correlation with SHH-activation and patients), M2 (2/37), or M3 (15/37), according to the Chang staging sys-
possible Tp53 mutation (p53>50%); One patient died without disease tem. Histologically, 30 patients were diagnosed with classic MB, 3 with
due to Coronavirus + and meningitis four months after ASCR. Among desmoplastic MB, 3 with large cell/anaplastic (LCA) MB and 1 patient
relapsed classic-MB, one had M+ disease and SHH characteristics and with an embryonal tumor, non-otherwise specified, in whom the diag-
the other three were classified as non-WNT/non-SHH: two also had nosis of MB was suggested by DNA methylation analysis. Molecularly,
M+, one with MYCC+ on histology. Both MBDN and two/four relapsed 18 out of 37 patients have been classified into WNT-activated Medul-
classic-MB are alive after salvage therapy with craniospinal irradiation. loblastoma (3patients), SHH-activated with TP53-wildtype (1 infant
Conclusions: We report the outcome of children less than five years patient) or TP53-mutant (2patients: 1somatic / 1germline), Group 3
with medulloblastoma treated in a middle-income-country, show- (4patients) and Group 4 (8patients). Germline alterations predispos-
ing a very acceptable outcome despite difficulties in referral and in ing to medulloblastoma (NBN, APC, TP53) were detected in 3 patients.
performing properly the molecular evaluation on a routine basis. During the follow-up period (mean: 58months, range: 8.5-111months),
6 patients succumbed to their disease. Specifically, 2 patients with
classic MB (1 classified as Group4) relapsed and succumbed to their
EP317 / #1455 RISK FACTORS OF MEDULLOBLASTOMA: A disease 4 and 6 years after initial diagnosis, 2 patients with metastatic
10 YEARS’ EXPERIENCE OF A SINGLE CENTER LCA-MB (1 classified as Group3) and 2 patients with metastatic classic
MB-SHH-TP53mutant progressed rapidly under their initial treatment.
Maria Filippidou1,2,3 , Kleoniki Roka1 , Antonia Vlachou1 , Kalliopi Conclusions: Our experience concurs with worldwide published series
Stefanaki4 , Georgios Sfakianos5 , Maria Chasiotou6 , Stavros Glentis1 , and shows that the initially disseminated form, anaplastic type, and
Georgia Avgerinou1 , Ilona Binenbaum1 , Florian Selt2,7,8 , Dominik specific molecular groups (Group3 and SHH-TP53mut) are the most
Sturm2,3,8 , Felix Sahm7,9 , Steffen Hirsch2,10 , Kristian Pajtler2,3,8 , Till important risk factors for medulloblastoma treatment failure.
Milde2,7,8 , Stefan Pfister2,3,8 , Antonis Kattamis1
1 “Aghia Sophia” Children’s Hospital, Division Of Pediatric Hematology
Oncology, First Dpt Of Pediatrics, National & Kapodistrian University Of EP318 / #675 A SINGLE CYTOSINE METHYL
Athens, Athens, Greece; 2 Hopp Children’s Cancer Center, (kitz), Heidelberg, PCR-GENOTYPING BASED DECISION SUPPORT SYSTEM FOR
Germany, Heidelberg, Germany; 3 German Cancer Research Center (DKFZ) RAPID CLASSIFICATION OF MEDULLOBLASTOMA
and German Cancer Consortium (DKTK), Heidelberg, Germany, Pediatric
Neurooncology, Heidelberg, Germany; 4 “Aghia Sophia” Children’s Hospital, Soledad Gómez-González1 , Joshua Llano2,3,4 , Marta Garcia1 , Alicia
Pathology Department, Athens, Greece; 5 “Aghia Sophia” Children’s Hospi- Garrido-Garcia1 , Isadora Lemos1 , Mariona Suñol5 , Nagore Gene1 ,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S295 of S635

Oscar Muñoz Aznar1 , José Hinojosa6 , Marta Perez-Somarriba EP319 / #564 CLINICO-EPIDEMIOLOGICAL PATTERN AND
Moreno1,7 , Ofelia Cruz1,7 , Vicente Santa-María1,7 , Andres Morales1,7 , TREATMENT OUTCOME OF PAEDIATRIC MEDULLOBLASTOMA:
Alexandre Perera2,3,4 , Cinzia Lavarino1,7 A SINGLE INSTITUTIONAL EXPERIENCE IN NORTH EAST INDIA
1 Hospital Sant Joan de Déu, Pediatric Cancer Center Barcelona (pccb),
Barcelona, Spain; 2 Universitat Politècnica de Catalunya, B2slab, Depar- Munlima Hazarika1 , Sreya Mallik1 , Suhani Barbhuiyan1 , Mouchumee
tament D’enginyeria De Sistemes, Automàtica I Informàtica Industrial, Bhattacharyya2
Barcelona, Spain; 3 Networking Biomedical Research Centre in the sub- 1 Dr. Bhubaneswar Borooah Cancer Institute, Medical & Paediatric Oncol-
ject area of Bioengineering, Biomaterials and Nanomedicine, (ciber- ogy, Guwahati, India; 2 Dr. Bhubaneswar Borooah Cancer Institute, Radia-
bbn), Madrid, Spain; 4 Institut de Recerca Sant Joan de Déu, (irsjd), tion Oncology, Guwahati, India
Barcelona, Spain; 5 Hospital Sant Joan de Deu, Pathology, Barcelona,
Spain; 6 Hospital Sant Joan de Deu, Pediatric Neurosurgery, Barcelona, Background and Aims: Medulloblastoma is one of the most com-
Spain; 7 Hospital Sant Joan de Deu, Pediatric Oncology, Barcelona, mon malignant brain tumours of childhood with aggressive clinical
Spain behaviour. There is limited published data of paediatric medulloblas-
toma from developing countries like India. The aim of the study is to
Background and Aims: Classification of medulloblastoma (MB) evaluate the clinico-epidemiological pattern and treatment outcome in
into clinically relevant molecular subgroups (WNT, SHH and non- paediatric medulloblastoma in a cancer care centre of North-East India.
WNT/non-SHH) is critical for patient prognostication and treat- Methods: In this retrospective study, we analyzed the medical records
ment. Genome-wide DNA methylation profiling enables precise of 52 children diagnosed with medulloblastoma at Dr. B. Borooah
MB classification, however the implementation of this diagnostic Cancer Institute, Guwahati, Assam from 2015 to 2019.
approach in daily clinical practice is challenging for many centres Results: The median age of diagnosis was 8±3.5 years (range 1-15
worldwide. We have applied a novel approach for MB classifica- years) with male: female ratio of 2.25:1. Headache (73%), vomiting
tion based on single cytosine-methyl PCR-genotyping analysis of a (69.2%), unsteadiness (46.1%) and cranial nerve palsy (11.5%) were the
reduced set of subgroup-specific differentially methylated cytosines, common presenting features. Staging showed that 39 (75%) patients
and developed a decision support system for automated subgroup presented with localised disease, 5 (9.6%) patients with malignant
assignment. cells in CSF, 6 (11.5%) with spinal involvement and 2(3.8%) with dis-
Methods: We analysed DNA methylation microarray data of 4,741 tant metastasis. Majority of the patients (59.6%) had high risk disease.
samples (3,094 MB and 1,647 non-MB tumors), qPCR data of tumor Ventriculo-peritoneal shunt was needed for 37(71.1%) patients. Thirty
samples (50 MBs and 10 non-MB tumors) and blood samples (6 (57.6%) patients underwent total/near total excision and 19(36.5%)
healthy individuals). Synthetic double-stranded DNA was used for had subtotal excision. Four (7.6%) patients below 3 years of age
primer testing and qPCR control references. The multistep deci- received chemotherapy only. Thirty eight (73.07%) patients received
sion support system (DNA methylation status predictor and sub- craniospinal radiation upto 36 Gy followed by posterior fossa boost
group classification) was developed using binary-based classification upto 54 Gy with concurrent vincristine. Adjuvant chemotherapy was
algorithms. received by 18 (34.6%) patients. Majority (48.7%) patients showed par-
Results: We designed, tested and validated allele-specific qPCR geno- tial response and only 3(5.7%) achieved complete response. Among
typing primers capable of discriminating between single base-pair these 52 children, 9 (17.3%) refused treatment. Progression free sur-
changes representing differential methylation states. When tested vival at 2 year, 3 year and 4 year were 42.3%, 28.8% and 19.2% respec-
with tumor DNA, primers showed specific hybridization enabling tively. Overall survival at 2 year, 3 year and 4 year were 48.07%, 34.6%
detection of the methylation state of 93% analysed cytosines, and and 21.1% respectively. Most common treatment related toxicity was
manual classification of 84% of MB samples (100% concordance). febrile neutropenia (23%).
By using binary discrimination algorithms, we developed a DNA Conclusions: The epidemiological patterns are quite similar to the
methylation predictor that enabled automated analysis of qPCR worldwide data. The survival rates are low as compared to other
data, and increased methylation status prediction capacity (AUC studies because of poor follow up data.
0.978, accuracy 93.9% (95%CI [89.9-96.6%]; k=0.88)). A hard-decision
decoding classifier was then developed and validated using multi-
institutional DNA methylation microarray data (n=3,044). MB sub- EP320 / #1102 A NATIONAL PEDIATRIC NEURO-ONCOLOGY
groups were assigned with an accuracy >90%. When tested with TUMOR BOARD INITIATIVE IN SPAIN: RESULTS OF A
tumor DNA qPCR-data, the decision support system assigned sam- WEB-BASED SURVEY TO EVALUATE PARTICIPANT EXPERIENCE
ples to a MB subgroup with >95% accuracy (95%CI [82.25%-99.36%]; UTILIZING THIS RESOURCE
k=0.87).
Conclusions: We have developed a multistep decision support system Laura Illade1 , Carmen Garrido2 , Ofelia Cruz3 , Miguel García-Ariza4 ,
that allows for simple, accurate and cost effective classification of MB Palma Solano-Páez5 , Anna Llort6 , María Baro7 , Adela Canete8 ,
tumors into clinically relevant subgroups using a qPCR, a widespread Carlota Calvo9 , Andres Morales10 , Eduardo Quiroga-Cantero11 ,
molecular technique. Diego Plaza Lopez De Sabando12 , Elida Vazquez-Mendez13 , José
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S296 of S635 ABSTRACTS

Hinojosa14 , Teresa Ribalta15 , Jordi Giralt16 , Marta Alonso17 , Cinzia patient’s medical history. All the participants recommend professionals
Lavarino18 , Idoia Martin-Guerrero19 , Alicia Moraleda20 , Ana to share their cases and would do so again themselves.
Fernández-Teijeiro Álvarez21 , Alvaro Lassaletta22 Conclusions: The Spanish Pediatric Tumor Board initiative provided a
1 Hospital Santiago Compostela, Pediatric Oncology, Santiago de Com- valuable tool for the management of pediatric brain tumors in Spain as
postela, Spain; 2 GREGORIO MARAÑÓN, Pediatrics, Madrid, Spain; 3 Sant it provided a feasible and easy to access continued medical education
Joan de Déu, Oncology, Sant Joan d’Espí, Spain; 4 Hospital de Cruces, Pedi- opportunity for the participants.
atric Oncology, Bilbao, Spain; 5 HOSPITAL INFANTIL VIRGEN DEL ROCIO,
Pediatric Oncology, SEVILLE, Spain; 6 Hospital Universitario Vall d’Hebron,
Pediatric Oncology And Hemathology, Barcelona, Spain; 7 Hospital Doce de EP321 / #620 REMISSION OF HYPOTHALAMIC OBESITY IN
Octubre, Pediatric Oncology, Madrid, Spain; 8 Hospital UiP La Fe, Paediatric PEDIATRIC-ONSET CRANIOPHARYNIOMA DURING
Oncology And Hematology Unit, Valencia, Spain; 9 Hospital Miguel Servet, INTERFERON-BASED TUMOR TREATMENT
Pediatric Oncology, Zaragoza, Spain; 10 Hospital Sant Joan de Deu, Oncol-
ogy, Barcelona, Spain; 11 Hospital Infantil Virgen del Rocio, Pediatric Oncol- Briana Patterson1 , Rachel Gray2 , Claire Mazewski2 , Dolly Aguilera3 ,
ogy, SEVILLE, Spain; 12 Hospital Universitario La Paz, Pediatric Hematology Robert Craig2 , Prabhumallikarjun Patil4 , Anna Janss2
And Oncology, Madrid, Spain; 13 Hospital Vall D’Hebron, Pediatric Radiol- 1 Emory University School of Medicine, Aflac Cancer And Blood Disorders
ogy, Barcelona, Spain; 14 Hospital Sant Joan de Deu, Pediatric Neurosurgery, Center Of Children’s Healthcare Of Atlanta, Atlanta, United States of Amer-
Barcelona, Spain; 15 Hospital Clinic, Pathology, Barcelona, Spain; 16 Hospital ica; 2 Emory SOM, Aflac Cancer Center Pediatrics Hematology- Oncology,
Vall D’Hebron, Radiation Oncology, Barcelona, Spain; 17 Clinica Universi- Atlanta, United States of America; 3 Children Healthcare of Atlanta, Hema-
dad de Navarra, Research, Pamplona, Spain; 18 Hospital Sant Joan de Deu, tology Oncology, Atlanta, United States of America; 4 Emory SOM, Pediatrics
Pediatric Oncology, Barcelona, Spain; 19 University of the Basque Coun- Hematology- Oncology, Atlanta, United States of America
try and IIS Biocruces Bizkaia, Genetics, Physical Anthropology And Animal
Physiology, Leioa, Spain; 20 ASION, Psychology, Madrid, Spain; 21 Hospital Background and Aims: Craniopharyngioma is a rare tumor arising
Virgen de la Macarena, Pediatric Oncology, Sevilla, Spain; 22 Hospital Infantil from the craniopharyngeal duct. Proposed treatments include surgical
Universitario Niño Jesús, Pediatric Neuro-oncology, Madrid, Spain resection, local irradiation, intracystic and/or systemic interferon-
alpha. Although long-term survival rates are high and metastatic
Background and Aims: The Spanish Brain Tumor Group of the Spanish potential is low, local recurrences and damage to adjacent structures
Pediatric Hematology-Oncology Society (SEHOP) launched in January from the tumor, surgery and/or radiation often result in endocrine and
2020 an initiative to allow Pediatric Oncologists to weekly present metabolic complications. Hypothalamic obesity (HO) is reported in 65-
their cases to the group. Between January 2020 and February 2022, 56 80% of craniopharyngioma patients after aggressive surgical resection.
cases were reviewed. This survey explored the participants’ experience HO significantly impairs health-related quality of life, remains difficult
utilizing this resource. to treat, and may reduce survival. We aim to describe the association
Methods: A cross-sectional electronic questionnaire was distributed to between systemic interferon therapies and the trajectory of BMI in
35 participants in 24 institutions through email. pediatric patients with craniopharyngioma-associated HO.
Results: Twenty-six respondents (74%) from all the participating insti- Methods: Subjects entered into an IRB-approved database at a single
tutions completed the survey. All participants (100%) found this a institution between 2000-2021 with the aim of determining progres-
great initiative. Seventy-six percent found the contact form easy, sion free survival included children with craniopharyngioma treated
and 73.1% the format convenient. Only 1 person had internet con- with systemic interferon (Peginterferon alfa-2b, Interferon alfa-2b, or
nectivity problems. Thirty-one percent reported frequent-attendance Peginterferon alfa-2a). We analyzed BMI trends before, during and
(75-100% meetings), 50% reported mid-attendance (50%), and 19% after interferon therapy. Obesity was defined as BMI >/=95%tile.
low-attendance (<25%). The most frequently reported attendance- Results: Twenty-three children (12F/11M) were diagnosed with cran-
barriers were the subspecialist’s workload (44%), the timing of the iopharyngioma at a median age 6 years. At diagnosis, 3 subjects (13%)
teleconference (44%). More than 70% of attendees found the fre- were obese. At initiation of interferon, 8 subjects (35%) (4F/4M) were
quency and duration of the teleconference were sufficient. Cases were obese with median BMI 26.6 kg/m2, median standardized BMI (SDS)
presented at diagnosis (42%), at first relapse (23,1%), or >/= second 2.47. Among these 8 obese subjects, 7 had reduction in BMI during
relapse (31%). In >90% the decisions suggested were very helpful, interferon therapy (median duration 9 months) and one increased in
appropriate and reinforce the decisions of the presenter. In 77% of BMI. Median BMI after interferon was 23.1 kg/m2, BMI SDS 2.0). The
the cases, some complementary study was performed outside the con- BMI reduction in obese children during interferon was followed by
sulting center, mainly methylation studies. The recommendations were rapid rise in BMI in all who stopped interferon, except for one.
followed in almost all cases, were transmitted to the rest of the multi- Conclusions: Systemic interferon-based therapy for craniopharyn-
disciplinary team and were generally well received, being considered gioma may be considered an option for tumor control and may
an improvement in the quality of care. In all cases the decision was have specific off-target benefits in patients who present with co-
transmitted to the families and 92% of those surveyed reflect it in the morbid HO. Additional research is needed to understand the impact of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S297 of S635

interferon associated weight loss on body composition, weight-related In 6DIPG-patients, following progression, re-irradiation resulted in
co-morbidities, and quality of life. temporary symptomatic improvement. In 4 of them, nivolumab was
also administered. The most common regimen for HGG-patients
upon progression was bevacizumab with irinotecan or CCNU. In 7
EP322 / #1110 HIGH-GRADE AND DIFFUSE INTRINSIC patients, targeted therapy was administered after end of RT, based
PONTINE GLIOMAS IN GREEK PEDIATRIC PATIENTS: FIRST on molecular results (3patients mTOR-inhibitor, 2patients Tyrosine-
ΝΑΤΙΟΝAL REPORT kinase-receptor inhibitor, 1MEK-inhibitor and 1 MET-inhibitor).
Overall survival was 58.8%(95%CI32.5-77.8), 41.2%(95%CI18.5-62.6)
Kleoniki Roka1 , Maria Filippidou1 , Vita Ridola2 , Antonia Vlachou1 , and 2.3%(95%CI4.3-38.3) at 1, 2 and 3 years respectively. Median
Vassilios Papadakis3 , Maria Nikita4 , Nikolaos Katzilakis5 , Evgenia overall survival time was 1.29 years.
Papakonstantinou6 , Emmanuel Hatzipantelis7 , Eftichia Stiakaki8 , Conclusions: Our national experience concurs with worldwide pub-
Sophia Polychronopoulou3 , Margarita Baka4 , Helen Kosmidis2 , lished series and shows that pediatric HGG and DIPG, despite aggres-
Antonis Kattamis1 sive treatment continue to have a grave outcome, urgently needing
1 “Aghia Sophia” Children’s Hospital, Division Of Pediatric Hematology other type of approaches. Re-irradiation may offer short survival
Oncology, First Dpt Of Pediatrics, National & Kapodistrian University Of prolongation.
Athens, Athens, Greece; 2 Children’s Hospital MITERA, Pediatric And Adoles-
cent Oncology Clinic, Athens, Greece; 3 “Aghia Sophia” Children’s Hospital,
Department Of Pediatric Hematology-oncology, Athens, Greece; 4 ‘P & A. EP323 / #1441 COMPLEX TREATMENT EFFECTIVENESS IN
Kyriakou’ Children’s Hospital, Department Of Oncology, Athens, Greece; PATIENTS WITH RELAPSED MEDULLOBLASTOMA, RM
5 University Hospital of Heraklion, Department Of Pediatric Hematology- GORBACHEVA RESEARCH INSTITUTE EXPERIENCE
oncology, University Of Crete, Heraklion, Greece; 6 Ippokratio Hospital
of Thessaloniki, Paediatric Oncology Department, Thessaloniki, Greece; Ilya Kazantsev1 , Asmik Gevorgian2 , Polina Tolkunova1 , Denis Kozlov2 ,
7 Aristotelion University of Thessaloniki, AHEPA General Hospital, Depart- Tatiana Jukhta1 , Andrey Kozlov1 , Margarita Halipskaya1 , Olga
ment Of Pediatric Hematology-oncology, Thessaloniki, Greece; 8 University Bogdanova1 , Alexander Galimov1 , Tatiana Andreeva1 , Nina Subora1 ,
Hospital of Heraklion, University of Crete, Department Of Pediatric Olesya Yudintseva1 , Polina Kuga1 , Yuri Punanov1 , Ludmila
Hematology-oncology, Heraklion, Greece Zubarovskaya3
1 RM Gorbacheva Research Institute Pavlov University, Pediatric Oncology,
Background and Aims: The main characteristics of pediatric high- Saint-Petersburg, Russian Federation; 2 RM Gorbacheva Research Institute
grade-gliomas (HGG) worldwide are aggressive clinical and biological of Pavlov University, Pediatric Oncology, Saint-Petersburg, Russian Fed-
behavior, high morbidity and mortality. Data from national registries, eration; 3 Pavlov University, R. M. Gorrbacheva Research Institute, Saint
on this entity are lacking Petersburg, Russian Federation
Methods: All 7, pediatric hematology-oncology centers in Greece par-
ticipated in this retrospective study that covered the period between Background and Aims: Medulloblastoma (MB) is the most common
1/2012-12/2021. Demographics, tumor characteristics and outcome central nervous system malignancy in pediatric population. Despite
were analyzed. majority of patients with MB achieving remission the prognosis in
Results: During the study period, 65 patients(36 females), median relapsed cases is still dismal. Complex intensive therapy including high-
age: 8.48y(range:3 days-16.9y) were enrolled. The most com- dose chemotherapy (HDCT) with autologous hemopoietic stem cell
mon tumor-location were brainstem(41/65patients) and parietal transplantation (auto-HSCT) could improve treatment results.
lobe(12/65patients). DIPG based on imaging studies only was diag- Methods: A total of 60 patients with relapsed MB received auto-HSCT
nosed in 29patients (9/29underwent biopsy). Surgical intervention in 2008-2021. The median age at relapse was 11.5 (5.0-37.0) years.
was performed 46patients(VP-shunt insertion:9, biopsy:24, partial Histological subtypes were classic (n=43), desmoplastic (n=12) and
resection:12, total/subtotal resection:6). Histological diagnosis was anaplastic (n=5). Molecular subgroups were assessed in 23 patients:
feasible in 42patients(16 brainstem-tumors). Astrocytoma gradeIV WNT (n=2), SHH (n=7), Gr.III (n=2), Gr.IV (n=12). MYC and MYC-
was diagnosed in 78.6%(33/42) and gradeΙΙΙ in 21.4%(9/42). Of notice, N amplification was found in 2/19 cases investigated. All patients
3 patients with initial histological findings of low-grade (2gradeII and received polychemotherapy prior to auto-HSCT achieving complete
1gradeI), were upgraded to gradeIV following molecular and DNA- (CR, n=21), partial response (PR, n=32) or stable disease (SD, n=7).
methylation analyses. Out of the 16 biopsied midline-tumors, 9 had In most (n=57) cases Carbo-Eto-Thio HDCT regimen was used. Sub-
a positive H3K27M-immunohistochemistry (diffuse-midline-glioma, sequently, 34 patients received craniospinal irradiation (CSI, n=23) or
DMG), Furthermore, 6/20 patients had Η3F3A-mutation, 4/20 ΕGFR- local radiation therapy (RT, n=11).
overexpression and 3/20 PTEN-loss. Six patients were elected by their Results: Engraftment at a median of 13 (8-37) days past transplant
caregivers to receive treatment in other countries. Patients>3years was registered in 53/60 cases. With a median follow-up of 30 (0.1-
of age (52/59) were treated upfront mainly according to HIT- 164) months from auto-HSCT the 3-year overall survival (OS) is 56.8%
HGG2013 Protocol with radiotherapy-temozolomide (46/52patients). (95%CI 42.9%-68.5%), event-free survival (EFS) is 29.8% (95%CI
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S298 of S635 ABSTRACTS

18.8%-41.6%). All but 2 patients with pre-transplant SD died due to and carboplatinum for a total of twelve courses seems to be effective
disease progression, one still has CR. The better OS and EFS were and provides a relatively good quality of life. Radiotherapy should be
observed in RT recipients (68.7% and 36.9% vs. 40.0% and 16.7%, avoided especially in patients with NF-1.
accordingly; p=0.005) and patients with classic MB (87.2% and 41.5%
vs. 22.2% and 8.3%, accordingly; p=0.002). Grade 4 (CTCAE V 5.0) com-
plications developed in 19.4% cases. Cumulative transplant-related EP325 / #1767 GERMLINE PTCH1 AND SUFU MUTATIONS
mortality was 11.7% (95%CI 1.1%-35.9%). IN PATIENTS WITH INFANT SHH MEDULLOBLASTOMA
Conclusions: Despite relatively high toxicity the complex therapy regi-
men allows achieving response in significant number of patients with Ludmila Papusha1 , Liudmila Yasko2 , Marina Koldasheva1 , Ekaterina
relapsed MB. HDCT with auto-HSCT is effective in patients with CR Salnikova1 , Maria Kurnikova2
and PR. RT improved HDCT results in assessed cohort. 1 Dmitry Rogachev National Medical Research Center Of Pediatric Hema-
tology, Oncology and Immunology, Department Of Pediatric Neurooncology,
Moscow, Russian Federation; 2 Dmitry Rogachev National Medical Research
EP324 / #541 OPTIC PATHWAY GLIOMAS IN CHILDREN: Center Of Pediatric Hematology, Oncology and Immunology, Laboratory Of
DEMOGRAPHIC, CLINICAL CHARACTERISTICS AND RESULTS Molecular Biology, Moscow, Russian Federation
OF AN ADAPTED PROTOCOL
Background and Aims: Germline mutations in PTCH1 and SUFU are
Rejin Kebudi1 , Ülkü Miray Yıldırım2 , Ayca Iribaş2 , Samuray Tuncer3 the main causes of basal cell nevus syndrome (BCNS, Gorlin syndrome,
1 Istanbul University, Oncology Institute, Pediatric Hematology-oncology, GS) detected in 2% patients with medulloblastoma (MB), exclusively
Istanbul, Turkey; 2 Istanbul University, Oncology Institute, Istanbul, Turkey; in SHH MB subgroup. The aim of the study was to investigate the
3 Istanbul University, Istanbul Medical Faculty, Ophtalmology, Istanbul, incidence of Gorlin’s syndrome and clinical outcome in the SHH MB
Turkey cohort.
Methods: We analyzed a cohort of 19 patients (pts) with infant SHH
Background and Aims: Optic pathway gliomas (OPG) constitute 1-5% MB (age < 3y/o) diagnosed between 2016 and 2020.
of childhood brain tumors. The aim of this study is to evaluate patients Results: The diagnosis of GS was genetically confirmed by NGS in 5
with optic pathway gliomas diagnosed/treated/ followed in a single patients, among them 2 had PTCH1 and 3 had SUFU germline muta-
center. tions. As to SUFU mutations, c.895C>T, p.(R299X) (rs1590066162)
Methods: The medical files of 95 patients with OPG diagnosed/treated was found in 2 pts and c.708_711del, p.(D237Cfs*29) was not
between 1990-2021 in the Istanbul University, Oncology Institute previously described. PTCH1 variants (c.290dupA, p.(N96Kfs*43)
were retrospectively assessed for demographic, clinical characteristics, (rs1554708751); c.1160G>A, p.(W387*)) were previously described in
treatment and outcome. pts with Gorlin syndrome. In two cases de novo status of mutations
Results: The median age of 95 patients (48 male, 47 female) was 54 was confirmed (1 PTCH1, 1 SUFU), three others were family cases.
(1-216) months, 58 (61.1%) had NF-1. Seven had diencephalic syn- Among these 5 pts with GS, the median age of MB manifestation was
drome (7.4%). Twenty-seven(28.4%) had Dodge 1, 30(31.6%) Dodge 18 months. Macrocephaly was presented in 4 pts and in 3 pts had
2 and 38(40%) Dodge 3 disease. Median follow up was 10.5 years high birth weight. Development delay was documented in 2 out of 5
(1-31 years). Nineteen (20%) patients had stable disease and were fol- pts. Histologically there were 4 MBEN and 1 DNMB. All pts received
lowed without treatment, 24 patients underwent surgery, 6 patients chemotherapy according to the HIT SKK protocol (3 SKK cycles
recieved radiotherapy (RT). 65 patients received chemotherapy (CT) followed by 2 modified SKK cycles). Intraventricular methotrexate
due to symptomatic/progressive disease. Chemotherapy consisted of was administrated in all cases. All patients are in complete remis-
(adapted SIOP-LGG regimen) vincristine (VCR) 1.5 mg/m2 /dose and sions after the end of treatment with a median follow up for 3.1
carboplatinum 550 mg/m2 /dose every 21 days for three courses with years.
weekly VCR, followed by VCR and carboplatinum every 28 days for a Conclusions: The incidence of Gorlin syndrome in our group of infant
total of 12 cycles (3 cycles induction, 9 cycles consolidation). Ocular SHH is 26%, which is comparable to Waszak et al. data. HIT-SKK
findings in 86 patients were, vision was stable in 67(77.9%), improved regimen with intraventricular methotrexate is an effective treatment
in 18(20.9%), detoriated in 1(1.2%). Radiology (MRI) revealed stable option for these patients.
disease/regression of tumor in 79 patients, and complete regression of
spinal metastasis in 1 patient. Endocrinologic follow-up was done. Ten
year overall survival(S) in all patients, in patients with NF1 and without EP326 / #1362 PROFILE OF PAEDIATRIC OPTIC GLIOMAS -
NF1 were 96%, 98% and 92%, respectively; 10 year event free survival EXPERIENCE FROM A TERTIARY CARE CENTRE IN SOUTH
(EFS) in all patients, in patients with NF1 and without NF1 were 83%, INDIA
87.6% and 77.5% respectively.
Conclusions: In children with, symptomatic/progressive OPG, Shree Hasitha Koneru1 , Bipasha Mukherjee2 , Kirthi Koka2 , Dhaarani
chemotherapy with an adapted regimen consisting of vincristine Jayaraman1 , Julius Scott1 , Sneha Latha1 , Preeta Nair2 , Satish Srinivas3
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S299 of S635

1 Sri Ramachandra Institute of Higher Education and Research, Depart- ogy. In 2017, computed tomography (CT) was acquired at Mbingo
ment Of Paediatric Haematology Oncology, Chennai, India; 2 Sankara Baptist Hosptial (MBH) with a qualified radiologist. Earlier analysis of
Nethralaya, Department Of Orbit, Oculoplasty, Reconstructive & Aesthetic existing hospital-based paediatric oncology registry reported a rarity
Services, Chennai, India; 3 Sri Ramachandra Institute of Higher Education of brain tumours. Our aim was to investigate the epidemiology and
and Research, Department Of Radiation Oncology, Chennai, India outcome of paediatric brain tumours at MBH.
Methods: Method This was a retrospective study by review of records
Background and Aims: Optic Pathway Gliomas(OPG) account for 3– of children below 15 years with brain tumours from September 2017
5% of paediatric brain tumours. Profile and outcome data of these to December 2021. A case review form was used to abstract data on
tumours from developing countries is limited. AIM- To study the clin- demographics, clinical presentation, management and outcome. Only
ical spectrum, imaging, treatment regimens and outcome in patients descriptive analysis were reported.
with OPG at our tertiary care paediatric oncology centre and our Results: About 1700 CT scans have been performed at MBH in
collaborating ophthalmology centre. the study period of which 20 were children below 15 years with
Methods: A retrospective descriptive study of paediatric patients brain tumours; 9 males and 11 females. The diagnoses were medul-
diagnosed with OPG from 2018 to 2022. Clinical characteristics, radio- loblastoma (n=4); ependymoma (n=4); brain stem glioma (n=3); and
logical, treatment details, ocular assessment and overall outcome were metastatic neuroblastoma (n=1), eight other children did not have
analysed. diagnosis. The commonest presenting symptoms were headache (5,
Results: Twenty patients were diagnosed with OPG during the study 25%), hemiplegia (3, 15%), vomiting (3, 15%), impaired vision (5, 25%)
period. Seventy five percent were male. Mean age at diagnosis was and status epilepticus (1, 5%). Eight had a ventriculo-peritoneal shunt,
7.55 years. Neurofibromatosis-1 was present only in 15%. Visual three were referred to another specialized hospital, two referred
disturbances, proptosis were the major symptoms in 80% and 70% abroad, two patients had surgery and chemoradiation, one patient had
respectively. Mean duration of symptoms was 7 months. Bilateral eye only surgery, two placed on palliative care only, and two refused treat-
involvement was noted in 10%. Visual acuity was diminished in 95% ment. After a median follow up of twelve months, ten (50%) are alive,
of patients at diagnosis. Relative-Afferent-Pupillary-Defect was seen in five (25%) dead and five (25%) lost to follow up.
10 patients. Visual-Evoked-Potential (VEP) was done in 11 patients and Conclusions: The availability of CT and the services of a radiologist at
90% had delayed latency. Thirteen patients had isolated optic nerve, Mbingo Baptist Hospital has enabled the diagnosis of brain tumours.
three had isolated chiasmal and four had involvement of both struc- Pursuant to this study, there are ongoing efforts to implement and
tures. Four patients were under observation (low risk features) with establish a network of referral for effective specialist management of
periodic evaluation, 3 were lost to follow up at diagnosis, 8 patients brain tumours.
received only chemotherapy (Vincristine-carboplatin), 2 had under-
gone debulking surgery and 3 patients received radiation (54 Gy) in
view of inadequate response to chemotherapy. At last follow-up, 12 EP328 / #291 METASTATIC DIA/DIG: MOLECULAR
patients underwent visual-assessment of which 75% had persistent CHARACTERIZATION AND TREATMENT RESPONSE
abnormal fundus and 30% had delayed latency in VEP. Six patients
were stable, 3 had progression of disease and 3 patients improved James Petropoulos1 , Zarina Assis2 , Melanie Finkbeiner1 , Clare
radiologically. No death was reported. Gallagher3 , Walter Hader3 , Jennifer Chan4 , Douglas Strother1 , Lucie
Conclusions: Patients with OPG usually present with nonspecific visual Lafay-Cousin1
symptoms. Early diagnosis and multi disciplinary management can 1 Alberta Children’s Hospital, Pediatric Hematology Oncology And Bone
help stabilize the disease, prevent progression and improve outcome Marrow Transplantation, Calgary, Canada; 2 Alberta Children’s Hospital,
in these patients. However, targeted therapy for this rare paediatric Diagnostic Imaging, Calgary, Canada; 3 Alberta Children’s Hospital, Pediatric
tumour needs to be explored by clinical trials. Neurosurgery, Calgary, Canada; 4 Calgary Laboratory Services, Neuropathol-
ogy, Calgary, Canada

EP327 / #1911 BRAIN TUMOURS AT MBINGO BAPTIST Background and Aims: Desmoplastic infantile astrocytoma (DIA) and
HOSPITAL IN CAMEROON: IMPROVED DIAGNOSIS WITH desmoplastic infantile ganglioglioma (DIG) are glioneuronal tumors of
MORE IMAGING CAPACITY early childhood. Surgical resection is usually sufficient to cure these
benign tumors. The presence of metastatic seeding is rare and has
Francine Kouya1 , Glenn Mbah Afungchwi1 , Feudjio Ghislain2 been reported as an adverse prognostic factor. We present 2 cases
1 cameroon baptist convention health services, Pediatric Oncology, of children with recurrent metastatic DIA/DIG to describe their ther-
Bamenda, Cameroon; 2 Cameroon Baptist Convention Health Services, apeutic management and outcome and to highlight the importance
Radiology, Mbingo, Cameroon of molecular characterization of these rare tumors to guide adjuvant
therapy.
Background and Aims: Introduction Reliable diagnostic capacity is Methods: Description of 2 cases including clinical presentation
required for effective childhood cancer management and epidemiol- and response evaluation by MRI.Tumor burden was evaluated by
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S300 of S635 ABSTRACTS

calculating the sum of the maximum bi-perpendicular dimensions of diagnosis, treatment, or follow-up. We have collected an International
the 3 most dominant lesions on MRI. cohort of PLGG which developed an intratumoral hemorrhage.
Results: The first patient developed metastatic recurrence after initial Methods: International, multicentric, observational, descrip-
gross total resection (GTR) of localized DIG. Relapse was treated with tive and retrospective analysis of 28 patients with a PLGG
monthly carboplatin and vincristine (CB/VCR). Complete response who suffered an intratumoral hemorrhage. We gathered data
(CR) was achieved after 15 cycles and the patient has remained in on demographic characteristics, histology, molecular findings,
CR for 5 years. Post-hoc molecular analysis of the tumor revealed a treatment received, and information related to the hemorrhagic
BRAF-RDX fusion. The second patient presented with a disseminated event.
intraventricular relapse following an incomplete resection of a DIA Results: Twenty-eight patients were enrolled onto the study. Median
associated with SPECC1L-NTRK2 fusion. Patient received 2 cycles of age at tumor diagnosis was 10.1 years (range, 0.4 to 17.5 years). Three
CB/VCR with minimal response and was then switched to Larotrec- patients (10.7%) had a cancer predisposition syndrome (2 Neurofibro-
tinib. The patient demonstrated positive treatment response with 67% matosis type 1 and 1 Noonan syndrome). Most frequent histological
and 77% decrease in tumor burden on follow-up MRI at 3 months and subtypes: pilocytic astrocytoma (67.8%), pleomorphic xantoastrocy-
6 months, respectively .The patients has remained on treatment since toma (10.7%) and pilomyxoid astrocytoma (7%). The most common
then with significant clinical improvement. locations: hypothalamic-chiasmatic (32.1%) and posterior fossa (25%).
Conclusions: In our 2 cases, metastatic recurrence was responsive to BRAF status was available in 16 patients (4 had a KIAA1549BRAF
adjuvant therapy leading to CR with conventional chemotherapy in fusion and 2 a BRAFV600E mutation). NGS was performed in 10
one and to VGPR with NTRK inhibitor for the second patient. Early patients, four of them had an FGFR alteration. Regarding therapy, all
molecular characterization of these benign tumors is critical in case patients had initial surgery, with 42.8% needing further surgeries. Sev-
of incomplete resection or metastatic seeding to widen therapeutic enteen patients (60.7%) received at least one chemotherapy regimen,
options and maximize chance of cure. Response with NTRK inhibitor and six patients (21.4%) received radiotherapy. Twenty-four patients
appears rapid and significant but the total duration of treatment (85.7%) had a spontaneous intratumoral hemorrhage; other etiolo-
remains unknown. gies were post-traumatic (10.7%) and post-surgical (3.6%). In thirteen
patients (46.4%), the hemorrhages occurred at the time of diagnosis.
The main treatment strategies were surgery and observation (39.2%
EP329 / #1049 INTRATUMORAL HEMORRHAGE IN each group). Two patients (7.1%) died because of the bleeding event. To
PEDIATRIC LOW-GRADE-GLIOMAS: AN INTERNATIONAL CASE date, 75% of the patients are alive, although 4 of them (14.2%) had a
SERIES recurrence of their hemorrhage.
Conclusions: Pediatric low grade gliomas are at risk of intratumoral
José Luis Moreno-Carrasco1 , Felisa Vázquez-Gómez1 , Lorena Baroni2 , hemorrhage, which occurs usually at diagnosis and spontaneously.
Daniel Alderete2 , Angela Mastronuzzi3 , Michal Zapotocky4 , Elena Despite being a life-threatening situation, mortality and recurrence
Carceller5 , Palma Solano-Páez6 , Eduardo Quiroga-Cantero6 , Maria rates are low in these patients.
Luisa Moreno-Tejero7 , Diana Valencia8 , Diego Plaza Lopez De
Sabando9 , Miguel García-Ariza10 , Manuel Diezi11 , Alvaro Lassaletta1
1 Hospital Infantil Universitario Niño Jesús, Pediatric Neuro-oncology, EP330 / #1050 PROGNOSTIC SIGNIFICANCE OF TUMOR
Madrid, Spain; 2 Paediatric Hospital Dr Juan P Garrahan, Hematology METHYLTRANSFERASE ACTIVITY IN CHILDREN WITH DE
Oncology, Buenos Aires, Argentina; 3 IRCCS Bambino Gesù Children’s NOVO METASTATIC MEDULLOBLASTOMA: RESULTS OF
Hospital, Department Of Paediatric Haematology/oncology, Rome, Italy; INTERCENTER PILOT STUDY
4 University Hospital Motol, Hematology Oncology, Pragues, Czech Repub-
lic; 5 Hospital Gregorio Marañón, Department Of Pediatric Hematology And Andrey Levashov1 , Dmitry Khochenkov2 , Anna Stroganova3 , Svetlana
Oncology, Madrid, Spain; 6 HOSPITAL INFANTIL VIRGEN DEL ROCIO, Pedi- Zagidullina1 , Elizaveta Turina1 , Marina Ryzhova4 , Sergey Gorelyshev5 ,
atric Oncology, SEVILLE, Spain; 7 Hospital Materno-Infantil de Badajoz, Vasiliy Grigorenko1 , Vladimir Polyakov1,6
Department Of Pediatric Hematology And Oncology, Badajoz, Spain; 8 IMAT 1 N.N. Blokhin National Medical Research Center of Oncology, Pediatric
oncomedica, Pediatric Oncology, Santander, Colombia; 9 Hospital Universi- Hematology And Oncology Institution, Moscow, Russian Federation; 2 N.N.
tario La Paz, Pediatric Hematology And Oncology, Madrid, Spain; 10 Hospital Blokhin National Medical Research Center of Oncology, Experimental Diag-
Universitario Cruces, Pediatric Hematology And Oncology, Barakaldo, nostic And Treatment Of Tumor Institution, Moscow, Russian Federation;
Spain; 11 Lausanne University Hospital and University of Lausanne, Pediatric 3 N.N. Blokhin National Medical Research Center of Oncology, Center Of
Hematology-oncology Unit, Division Of Pediatrics, Department "woman- Morphological And Molecular Genetic Tumor Diagnostics, Moscow, Russian
mother-child", Lausanne, Switzerland Federation; 4 N.N. Burdenko National Medical Research Center of Neu-
rosurgery, Department Of Neuropathology, Moscow, Russian Federation;
Background and Aims: Pediatric low-grade gliomas (PLGG) are grade 5 N.N. Burdenko National Medical Research Center of Neurosurgery, Depart-

I and II WHO tumors. Intratumoral hemorrhage is a common complica- ment Of Neurosurgery 1, Moscow, Russian Federation; 6 Pediatric oncology
tion (up to 8-20%), not well reported in the literature. It can happen at and hematology institute, SRI of Pediatric Oncology and Hematology, N.N.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S301 of S635

Blokhin National Medical Research Center, Oncology, Moscow, Russian Neurosurgery, Department Of Neuropathology, Moscow, Russian Federa-
Federation, Head And Neck Tumors, Moscow, Russian Federation tion; 5 N.N. Burdenko National Medical Research Center of Neurosurgery,
Department Of Neurosurgery 1, Moscow, Russian Federation; 6 Pediatric
Background and Aims: The aim of this study was to estimate event- oncology and hematology institute, SRI of Pediatric Oncology and Hema-
free survival (EFS) according to the activity of tumor methyltrans- tology, N.N. Blokhin National Medical Research Center, Oncology, Moscow,
ferases. Russian Federation, Head And Neck Tumors, Moscow, Russian Federation;
Methods: From 2017 till 2018 nineteen patients were included 7 Russian National Research Medical University named after N.I. Pirogov,
in trial. Children underwent adjuvant therapy: ArmB (n=11) - The Department Of Ent Diseases Of The Pediatric Faculty, Moscow, Russian
vincristine/cyclophosphamide/cisplatin/etoposide (OPEC)-based Federation
induction, CSI 36Gy + local RT to the tumor bed up to 54Gy with 5-
azacytidine, 1 cycle OPEC and 2 cycles thiophosphamide/carboplatin Background and Aims: The aim of this study was to estimate event-
with auto stem cell transplantation (auto-SCT); ArmC (n=8) - free survival (EFS) depending on the R/M group and therapeutic
cyclophosphamide/cisplatin-based induction, CSI 23.4 Gy followed by approaches.
2 cycles 5-azacytidine/thiophosphamide/carboplatin with auto-SCT, Methods: From 2008 till 2016 forty eight patients were included
local RT with 5-azacytidine. All patients was older 3 years, male/female in trial. Children underwent adjuvant therapy: for R0M0 – CSI
ratio 11/8. R1 status was revealed in 10 patients, M1, M2, M3 status 23.4Gy, for R1M0 and R0/1M+ – CSI 36.0Gy (instead of 39.6Gy
– in 1, 6 and 12 out of 19. Classic histological variant was identified in and additional irradiation to the metastatic sites up to 50.4–55.8Gy
16 tumor samples, large cell/anaplastic – in 3, MYC gene amplification for M2/M3) + local RT to the tumor bed up to 54Gy (instead of
in 1, MYC-N gene amplification in 3 (2 of them with TP53 mutation), 55.8Gy and primary site up to 59.4Gy for R1) followed by 4 cycles
Iso17q in 6. Molecular groups were determined in 12 samples (SHH vincristine/cyclophosphamide/cisplatin with auto stem cell transplan-
– 2, Group3 – 3, Group4 – 7). MGMT and DNMT1,3a,3b proteins tation. R0M0: n=25, 3–7 years of age/older – 13/12, vermis and 4-th
expression was assessed by immunohistochemical method in 19 ventricle/cerebellar hemisphere (primary site) – 21/4; tumor samples
samples (Score 0-3 – no/weak, Score 4-12 – moderate/strong). – classic/LCA/desmoplastic histology in 22/2/1, MYC-N amplifica-
Results: 3-year EFS was 54.5% in armB, 50.0% and in armC, tion in 1, Iso17q in 2, WNT/SHH/Group3/Group4/no data in 3/1/3/8
median follow-up 29.8 ± 5.8 and 37.7 ± 9.1 months (p=0.915). and 10. R1M0: n=8, 3–7 years of age/older – 5/3, vermis and 4-th
Moderate/strong MGMT and DNMT1,3a,3b proteins expression was ventricle/cerebellar hemisphere – 8/0; tumor samples – classic/LCA
revealed in 8, 12, 10, 8 out of 19 tumor samples. All tumor sam- histology in 7/1, Iso17q in 2, WNT/SHH/Group3/Group4/no data in
ples with MYC/MYC-N gene amplifications were characterized by 1/0/1/2 and 4. R0/1M+: n=15, 3–7 years of age/older - 10/5, ver-
moderate/strong level of DNMT1 protein expression. There was not mis and 4-th ventricle/cerebellar hemisphere – 12/3; M1/M2/M3 –
determined any prognostic significance of these proteins expression 4/2/9; tumor samples – classic/LCA/desmoplastic histology in 13/1/1,
according to age group (3-7 years and older), gender, M+ variants, MYC amplification in 1, MYC-N amplification in 2, Iso17q in 4,
molecular group (SHH/Group3/Group4). WNT/SHH/Group3/Group4/no data in 1/2/3/6 and 3.
Conclusions: There are not enough patients in both arms to understand Results: 5-year EFS was 82.0% in R0M0 group (6 relapses – 1 WNT, 1
the predictive value of the activity of tumor methyltransferases. It is SHH, 3 Group4, 1 secondary osteosarcoma in Group3, 2 events after
necessary to continue the study of these markers, especially in group 5 years), 3–7 years of age/older 88.9% and 58.3% (p=0.044); 62.5%
with MYC/MYC-N gene amplification. in R1M0 group (progression – 1 Group3, relapse – 1 Group 4, 1 case
with fatal septic complication); 33.3% in R0/1M+ group (8 relapses –
of them 1 SHH, 1 Group3, 3 Group4; progression – 1 Group3, 1 case
EP331 / #1501 PROGNOSTIC SIGNIFICANCE OF with fatal septic complication).
REDUCED-DOSE AND REDUCED-VOLUME RADIOTHERAPY IN Conclusions: Reduced-dose and reduced-volume radiotherapy led to a
CHILDREN WITH DE NOVO MEDULLOBLASTOMA: RESULTS OF significant decrease in the EFS rate in children with R1M0 and R0/1M+
INTERCENTER PILOT STUDY (LIKE-SJMB03) status.

Andrey Levashov1 , Anna Stroganova2 , Svetlana Zagidullina1 , Elizaveta


Turina1 , Dmitry Khochenkov3 , Marina Ryzhova4 , Sergey Gorelyshev5 , EP332 / #1335 NATIONAL EXPERIENCE AND USEFULNESS
Vasiliy Grigorenko1 , Vladimir Polyakov6,7 OF CENTRALIZED PATHOLOGICAL AND MOLECULAR REVIEW
1 N.N. Blokhin National Medical Research Center of Oncology, Pediatric IN PEDIATRIC MEDULLOBLASTOMA
Hematology And Oncology Institution, Moscow, Russian Federation; 2 N.N.
Blokhin National Medical Research Center of Oncology, Center Of Mor- Alaña Lide1 , Jon Ander Aguirre-Carrillo1 , Idoia Martin-Guerrero1,2 ,
phological And Molecular Genetic Tumor Diagnostics, Moscow, Russian Laura Zaldumbide1,3 , Lorena Mosteiro3 , Juan Ansede-Bermejo4 ,
Federation; 3 N.N. Blokhin National Medical Research Center of Oncology, Piedad Alba-Pavón1 , Ofelia Martinez5 , Alvaro Lassaletta6 , Felisa
Experimental Diagnostic And Treatment Of Tumor Institution, Moscow, Vázquez-Gómez6 , Eduardo Quiroga-Cantero7 , Anna Llort8 , Palma
Russian Federation; 4 N.N. Burdenko National Medical Research Center of Solano-Páez7 , Laura Illade9 , Miguel García-Ariza1,10
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S302 of S635 ABSTRACTS

1 Biocruces Bizkaia Health Research Institute, Pediatric Oncology Group, Ilaria Liguoro1 , Eva Passone2 , Chiara Pilotto2 , Raffaello Tosolini3 ,
Barakaldo, Spain; 2 Physical Anthropology and Animal Pathology, Faculty of Francesca Fabiani1 , Paola Cogo1 , Tiziana Zilli4
Sciences and Technology, Department Of Genetics, Leioa, Spain; 3 Hospital 1 University of Udine, Department Of Medical Area - Dame, Udine, Italy;
Universitario de Cruces, Osakidetza, Department Of Pathology, Barakaldo, 2 University Hospital of Udine, Pediatric Clinic, Udine, Italy; 3 University Hos-

Spain; 4 Fundación Pública Galega de Medicina Xenómica, Centro Nacional pital of Udine ASUFC, Pediatric Clinic, Udine, Italy; 4 Scientific Institute
De Genotipado, Santiago de Compostela, Spain; 5 Sant Joan de Déu, Oncol- Eugenio Medea, Scientific Institute Eugenio Medea, San Vito al Tagliamento
ogy, Sant Joan d’Espí, Spain; 6 Hospital Infantil Universitario Niño Jesús, (PN), Italy
Pediatric Neuro-oncology, Madrid, Spain; 7 Hospital Infantil Virgen del Rocio,
Pediatric Oncology, SEVILLE, Spain; 8 Hospital Universitario Vall d’Hebron, Background and Aims: As survival outcomes in children with brain
Pediatric Oncology And Hemathology, Barcelona, Spain; 9 Hospital Santiago tumors have been improving over time, cognitive sequelae of treat-
Compostela, Pediatric Oncology, Santiago de Compostela, Spain; 10 Hospital ment are becoming of interest. However, the role of histology and
Universitario Cruces, Pediatric Hematology And Oncology, Barakaldo, Spain surgery in the development of these cognitive deficits remains unclear.
This study aimed to evaluate cognitive functioning in children with
Background and Aims: The implementation of molecular informa- brain tumors before and after surgery.
tion is critical in the classification of pediatric medulloblastoma (MB). Methods: All children with a new diagnosis of primary brain tumor
It’s mandatory not only for the inclusion of cases in clinical tri- were prospectively assessed. Neurocognitive evaluations were per-
als, but also for the initial diagnosis/management. This information formed at diagnosis (T0) and within 14 days after surgery (T1). Intel-
should be reviewed and complemented when it’s no available in some ligence quotient, language, learning and memory, attention, executive
centers. We reviewed the implementation of molecular subgrouping- functions, visuo-constructional and sensorimotor skills were explored.
classification at national level, since it was included in the centralized Results: Twenty patients (12 males, mean age 10.6±3.2 years) were
pathological review. enrolled between January 2019 and December 2021. Tumor localiza-
Methods: Multi-center centralized prospective and retrospective tion was mainly supratentorial (11/20, 55%). The histological type was
study of frozen and FFPE tumor samples at diagnosis of pediatric MB low-grade in 9/20 (45%) patients and high in 11/20 (55%). All chil-
patients from national hospitals, from February 2021 to March 2022. dren underwent surgery, with gross total resection in 15/21 (71%)
Local pathology, centralized histology review, immunohistochemical cases. At baseline, patients showed significant major deficits in sen-
(IHC) subgrouping, and a molecular subgrouping based on the Mini- tence repetition (p=0.002), phonemic fluency (p=0.03), direct span
mal Methylation Classifier (MIMIC) from Schwalbe et al., 2017 were (p=0.006) and copy design tasks (p<0.001) in comparison to nor-
performed. mal values. Intrapatient evaluations did not change between T0 and
Results: Forty-eight cases were analyzed, 33 were prospective, and 45 T1. Children with low-grade lesions did not differ from those with
in frozen tissue. Locally, 45.8% did not have any subgrouping classifi- high-grade disease for baseline and clinical characteristics. At T0, the
cation information. The centralized review subclassified all cases using high-grade group showed lower scores than the low-grade in the Rey-
IHC. MIMIC classified in the non-WNT/non-SHH, 5 tumors (10.4%) in Osterrieth Complex Figure B test (5.6 ±4.4 vs 12.2±0.9; p=0.01)
Group 3 and 29 (60.4%) in Group 4. WNT-activated were 3 cases (6,3%) evaluating visuospatial memory. No significant difference emerged for
and SHH-activated 7 (14.6%). 4 cases (8.3%) were unclassified (2 FFPE T1 evaluations.
and 2 WNT, classified by IHC, monosomy 6 and CTNNB1 mutational Conclusions: Children with brain tumors may present several neu-
status) Comparing with local diagnosis, the centralized review showed rocognitive deficits in memory and visuospatial capabilities, particu-
changes in initial histology in 9 cases (18.7%), and subgrouping clas- larly those with high-grade diseases. Surgery, even when radical, didn’t
sification remained the same in 6 cases (12.5%). In 4 cases (8.3%) the impact cognitive functions in our population, independently of grading.
diagnosis changed, and in 38 cases (79.2%) the molecular subgrouping Prospective protocols with baseline and periodic neurocognitive eval-
classification was added. . uations should be encouraged to early detect functional difficulties and
Conclusions: The centralized pathological and molecular review in develop a tailored treatment approach for the patient.
pediatric MB has allowed us to have a complete and homoge-
neous information in the initial classification of these tumors. Not
all centers have this information available at diagnosis, and a cen- EP334 / #971 PHYSICAL, PSYCHOLOGICAL AND QUALITY
tralized review at a national level allowed confirmation of the OF LIFE EFFECTS OF HORSE-ASSISTED REHABILITATION IN
pathology and added this information in a significant number of CHILDREN AND ADOLESCENTS AFTER ONCOLOGIC
cases. TREATMENT: RANDOMIZED OPEN LABEL CLINICAL TRIAL

Anna Llort
EP333 / #1096 NEUROCOGNITIVE FUNCTIONS IN Hospital Vall d’Hebron, Pediatric Oncology, -, Spain
CHILDREN SURGICALLY TREATED FOR BRAIN TUMORS: A
PRELIMINARY REPORT Background and Aims: Scientific evidence in Horse Assisted Reha-
bilitation shows advantages in physical and psychological variables
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ABSTRACTS S303 of S635

in children and adults, whether healthy or with pathologies. Cancer dren, with particular emphasis in the peculiar imaging features of these
therapy may cause some health implications forlong cancer survivors unusual tumors in the pediatric age.
cancer. AIMS: Analyze if Horse Assisted Rehabilitation applied on Methods: We report two rare cases of primary melanocytic tumors of
padiatric cancer survivors, is safe, well tolerated and could improve the CNS: a primary melanocytic tumor of the occipital area in a 14-
symptoms derived from cancer or its treatments. year-old boy (case 1), and a diffuse leptomeningeal melanomatosis in
Methods: Randomized Open-label Clinical Trial including children (4- another 14-year-old boy (case 2). In contrast to melanocytoma, the dif-
18y) who have completed a treatment for a Brain Tumor. Intervention fuse leptomeningeal melanosis widely infiltrated the leptomeninges,
Group (IG): 24 weekly sessions (45 minutes each) of Horse Assisted and demonstrated features of malignancy. Main neuroimaging mani-
Rehabilitation; Control Group (CG) followed their normal interven- festations will be highlighted, emphasizing the diagnostic challenges
tions. Institutional Review Boards: CEEAH-UAB and Vall d’Hebron and correlating the imaging features with the pathological results.
Hospita. CTG ID: NCT04070131. Results: By neuroimaging, the main characteristic on MRI is the
Results: 17 children (9 girls, 8 boys; mean age: 9.4 y) randomized into hyperintense signal on T1-weighted images, due to the paramagnetic
two comparable groups were included. Horse Assisted Rehabilitation properties of melanin, with hypointense signal on T2-weighted images,
were safe and well tolerated by all IG participants, showing no dis- and with enhancement after gadolinium administration. Pathologically,
comfort or undesirable effects. Parents of IG participants reported the main clue is that these tumors are black or brown depending
improvements in children’s mood, quality of life (QOL), and partic- on their melanin content. In contrast to melanocytoma, the diffuse
ipation in activities. Motor coordination DCDQ07 test (Fine Motor leptomeningeal melanosis widely infiltrated the leptomeninges, and
subscale) showed a significant improvement in the IG (+2.662) com- demonstrated features of malignancy. A review of the current litera-
pared to the CG (0.816, p=0.037). About QOL tests, in both groups ture for main clinical, radiological, surgical, and histological findings of
there were significant improvements, more significant in the IG in the these lesions will be also pesented.
PEDSQL-C test, Health and Physical Activities subscales (CG: +1.40, Conclusions: Primary melanocytic tumors of the CNS are very rare
GI: -3.36, p=0.028), Emotional State (CG:+3.00, IG:-0.54, p=0.043) in the pediatric age. Based on two recent cases seen in our tertiary
and total score (CG:+15.00, IG:-0.91, p=0.032). There were no sig- centre, the main neuroimaging manifestations of these tumors will be
nificant differences between groups in other physical (Ped.and Sitting highlighted, emphasizing the diagnostic challenges and correlating the
Scale, Heart Rate Variability), psychological (Children’s Depression imaging features with the pathological results.
Inventory, State-Trait Anxiety for Children) or quality of life variables.
Conclusions: Horse Assisted Rehabilitation is safe and well tolerated
at every age and physical condition and could improve some physical EP336 / #57 CLINICAL AND PATHOLOGICAL
and QOL parameters in children after cancer treatment. Despite the CHARACTERIZATION OF DISSEMINATED PILOCYTIC
pandemia, who make difficult greater recruitment, It miight be a tool to ASTROCYTOMAS: A RETROSPECTIVE ANALYSIS FROM AN
be used to improve QOL of this particularly fragile population ONCOLOGICAL CENTER

Maristela Facholi1 , Laís Gabriela Maciel1 , Gustavo Teixeira2 , Flávia


EP335 / #1467 CNS PRIMARY MELANOCYTIC TUMORS IN Paula3 , Carlos Roberto Junior4 , Rui Reis3 , Bruna Mançano1
CHILDREN 1 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil; 2 Barretos
Cancer Hospital, Pathology Laboratory, Barretos, Brazil; 3 Barretos Can-
Elida Vazquez-Mendez1 , Ángel Sánchez-Montanez2 , Ignacio cer Hospital, Molecular Diagnosis Laboratory, São Paulo, Brazil; 4 Barretos
Delgado1 , Maria Poca1 , Paola Cano3,4 , Anna Llort5 Cancer Hospital, Pediatric Neurosurgery, São Paulo, Brazil
1 Hospital Vall D’Hebron, Pediatric Radiology, Barcelona, Spain; 2 Hospital
Vall d’Hebron, Pediatric Radiology, Barcelona, Spain; 3 Hospital Vall Background and Aims: BACKGROUND: Among pediatric CNS
D’Hebron, Pediatric Neurosurgery, Barcelona, Spain; 4 Hospital Vall tumors, pilocytic astrocytoma (AP) is the most frequent tumor, pre-
d’Hebron, Pediatric Neurosurgery, Barcelona, Spain; 5 Hospital Vall senting non-malignant features, like slow growth and non-infiltrative
D’Hebron, Pediatric Oncology, Barcelona, Spain traits. Rearrangements in the BRAF gene are the main molecular
alteration. Up to 10% may show dissemination. In such cases, radiation
Background and Aims: Primary melanocytic tumors of the CNS arise therapy, chemotherapy and targeted therapies can be used. AIM: To
from leptomeningeal melanocytes, derived from the neural crest dur- describe clinical and pathological aspects of patients with dissem-
ing embryonic life. They can occur as diffuse or solitary, benign or inated pilocytic astrocytoma at the Barretos Cancer Hospital from
malignant tumors. Very rare in children, they include the diffuse January 2000 to December 2019.
leptomeningeal melanosis, meningeal melanoma, melanocytic menin- Methods: MATERIALS AND METHODS: Longitudinal cohort study,
gioma and primary melanocytoma of the CNS, following the World with retrospective data collection and analysis of clinical and molecu-
Health Organization sub-classification. Our aim is to report two rare lar data from pediatric patients diagnosed with disseminated pilocytic
cases of primary melanocytic tumors of the CNS ocurring in two chil- astrocytoma from 2000 to 2019.
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S304 of S635 ABSTRACTS

Results: From 93 patients diagnosed with AP during this time, 10 PDX tumors were retained when compared with the original patient
(10,7%) had disseminated disease at diagnosis or follow-up. Half tumors.
(5/10, 50,0%) were in the hypothalamus/optical pathway region. Treat- Conclusions: We established a pre-clinical panel of primary cell lines
ment modalities varied according to age and clinical conditions. BRAF and PDX of pediatric brain tumor. We expect that these models
V600E mutation was detected in 3 tumors from 8 analyzed (3/8, allow for understanding tumor biology, identify new biomarkers and
37,5%) and one had KIAA1549:BRAF fusion (1/5, 20,0%). At this to investigate new therapies integrated into personalized medicine
moment, 60% of patients are alive and the overall 3-year survival was strategies.
65%.
Conclusions: This study provided clinical and epidemiologi-
cal data of patients with disseminated AP from a specialized EP338 / #1148 APPLICABILITY OF A NOMOGRAM
group in pediatric neuro-oncology. Findings are similar to those INCLUDING CLINICAL AND MOLECULAR VARIABLES TO
described in the literature regarding the analyses described STRATIFY RISK GROUPS IN PEDIATRIC MEDULLOBASTOMAS
(clinical, radiological, morphological, molecular, therapeutic and
survival). Bruna Mançano1 , Rui Reis2 , Carlos Almeida Junior3 , Gustavo Teixeira4
1 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil; 2 Barretos
Cancer Hospital, Cpom, Barretos, Brazil; 3 Barretos Cancer Hospital, Pedi-
EP337 / #878 PLATFORM OF EXPERIMENTAL MODELS FOR atric Neurosurgery, Barretos, Brazil; 4 Barretos Cancer Hospital, Pathology
PEDIATRIC BRAIN TUMOR Laboratory, Barretos, Brazil

Silvia Teixeira1 , Ana Carolina Baptista Moreno Martin1 , Terence Background and Aims: Medulloblastoma is the most frequent and
Duarte1 , Bruna Mançano2 , Carlos Roberto Junior3 , Rui Reis1 lethal malignant kind of pediatric brain tumors.A complex disease
1 Barretos Cancer Hospital, Molecular Oncology Research Center, Barretos, which comprises, at least, four different molecular subtypes (WNT,
Brazil; 2 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil; SHH, Group 3 and Group 4). These subgroups present with spe-
3 Barretos Cancer Hospital, Pediatric Neurosurgery, São Paulo, Brazil cific characteristics, distinct clinical presentations and different out-
comes.Recently, a nomogram that assesses not only molecular subtype
Background and Aims: Pediatric central nervous system (CNS) tumors but also clinical variables and it can predict more accurately the
are the leading cause of childhood cancer-related death. Then, more survival of the patients in developed countries. AIM: Analyze the
appropriated therapies are lacking, leading to develop pre-clinical mod- applicability of a prognostic nomogram for pediatric medullobastomas
els that are more predictable. In this sense, patient-derived xenografts treated at Barretos Cancer Hospital.
models (PDX) have emerged as an important platform to search for Methods: 85 pediatric patients, between 0 to 18 years, with medul-
a new treatment and to identify new biomarkers in oncology. These loblastomas treated between January 2000 and December 2017 was
models try to mimic the cellular and histopathologic structure, tumor assessed. Clinical pathologic variables were considered and stored at
heterogeneity and are being used for preclinical drug evaluation and RedCap. The molecular subgroups were performed using the NanoS-
personalized medicine strategies. Then, the aim of this study was to tring platform.Variables such as age, type of tumor resection, molecular
established a panel of pre-clinical pediatric brain tumor (PDX and subgroups, presence, or not, of metastasis, and dose the craniospinal
primary cell line) from Brazilian patient. radiotherapy were assessed.
Methods: A total of 23 cancer specimens from patients were obtained Results: 85 patients with descriptive analysis of the sample, the molec-
from July 2021 to March 2022. The surgical specimens were trans- ular classification could be performed on 62 of them due to the
planted to immunodeficient mice NSG or NUDE (Nu/J), or used to lack of biologic material. The distribution of molecular subgroups was
established primary cell culture. Solid tumor samples were cut into SHH - 35.5%, WNT- 19.4%, Group 3 – 16.1%, Group 4 – 29.0%.It
2 mm3 and implanted subcutaneously. The tumor was designated as could be observed that the presence of metastasis (p=0.03),received
generation F0, and the tumor was passaged to mice for further genera- chemotherapy or not (p<0.0001), and the dose of craniospinal radio-
tions (F1, F2. . . ). Pathological tissues (PDX and patient) were subjected therapy (p<0.0001) were associated with worse outcomes. The com-
to hematoxylin-eosin staining and immunohistochemistry. The proto- bined analyses of clinic pathologic variables, and molecular subgroup,
col was approved by institutional Ethics Committee and all patients the nomogram was designed to predict, with a high level of accuracy, 1-
gave written informed consent. Animal experiments were performed year overall survival (AUC=60.9), 3-years overall survival (AUC=77.3)
in compliance with the guidelines of the Institutional Animal Care and and 5-years overall survival (AUC=80). A calculator was designed to
Use Committees (IACUC). predict, in a fast and efficient way, survival in 1, 3 and 5 years.
Results: PDX model (6) and a panel of primary cell lines (16) were estab- Conclusions: This study showed the applicability of the nomogram for
lished of several tumor types (Low- or high-grade glioma, ependymoma, a Brazilian pediatric medulloblastoma and the utility of a calculator
medulloblastoma, meningiomas). The histopathological analysis indi- developed from the nomogram as an easy-to-use tool for the clinic
cated that heterogeneity was recapitulated and the structures of the routine.
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ABSTRACTS S305 of S635

EP339 / #1643 ESTABLISHMENT OF A NEW EPENDYMOMA ter, BARRETOS, Brazil; 5 Barretos Cancer Hospital, Pathology Laboratory,
PRIMARY CELL LINE AND PDX MODEL Barretos, Brazil

Ana Carolina Baptista Moreno Martin1 , Terence Duarte1 , Gustavo Background and Aims: Central nervous system (CNS) tumors are
Teixeira2 , Renato José Da Silva Oliveira1 , Bruna Mançano3 , Carlos the most common malignant neoplasm in children, being the main
Almeida Junior3 , Rui Reis1 , Silvia Teixeira1 cause of morbidity and mortality in the pediatric population. At
1 Barretos Cancer Hospital, Molecular Oncology Research Center, Bar- the Barretos Cancer Hospital, we observed a rise in the number of
retos/SP, Brazil; 2 Barretos Cancer Hospital, Pathology Laboratory, Barretos, patients diagnosed with central nervous system tumors, which are
Brazil; 3 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil the main malignant neoplasms among solid tumors. With the low
overall survival rate of 20% in the country, there is a need for a
Background and Aims: Intracranial Ependymoma is a type of multidisciplinary pediatric neuro-oncology group dedicated to bet-
tumor that accounts 1.6% of all central nervous system (CNS) ter diagnosis and treatment with improved survival and patient care
tumors and can affect adult and child. Cell lines and animal mod- . AIM: Analyze the overall survival in two periods of patients diag-
els are rare and therefore search for new treatments are lim- nosed with brain tumor in the pediatric age group (under 18 years
ited. Thus, the aim of this work was to establish a primary cell old).
line and a PDX (Patient Derived Xenograft) model using tissue Methods: Retrospective and cross-sectional study, analysis of 445
from a patient diagnosed with ependymoma grade 3 according to pediatric patients aged from 0 to 18 years old with a diagnosis of
WHO. CNS tumor, divided into two periods: January/2000 to June/2013
Methods: Patient tissue was collected and taken to our animal house and August/2013 to July/2019. We evaluated gender, age group, his-
facility. Excess vessels and necrotic tissue were removed and then tis- tology, topography, type of treatment (surgery, chemotherapy and/or
sue was divided into 4 pieces for: biobank, pathology, cell culture and radiotherapy) and follow-up.
PDX. For primary culture, tissue was prepared using accutase proto- Results: Of the 445 patients with CNS neoplasia, 198 (44.49%) were
col, incubated 3 times at 37o C for 5 min, cells were neutralized using diagnosed from January/2000 to June/2013 with OS at 2 years of
complete media and centrifuged for 5min to remove enzyme excess. 59.6%, 5 years of 51.5% and 10 years 43.2%. In the second 6-year
Pellet was resuspended in media and plated into T25 flasks. Cells were period, 247 (55.5%) were diagnosed with a 2-year survival of 72.7% and
monitored and splitted after 80% of confluence. For the PDX model, a 5-year survival of 67% with statistical significance (p < 0.0001).
tissue was mixed with matrigel (1:1) and injected subcutaneously into Conclusions: This study showed the importance of the team spe-
the flanks of NSG mouse (P0 – passage 0). Tumor grow was monitored cialized in pediatric neurooncology, bringing significant data to the
using a caliper and once the tumor reached 1.5mm3 , animal was har- literature of a developing country.
vest and tumor was reimplanted into P1 mouse and successively until
P3.
Results: In this study we were able to determine an ependymoma cell EP341 / #208 EARLY RESULTS OF RANDOMIZED STUDY OF
line, that was frozen and thaw, remaining stable in culture. Therefore, RADIOTHERAPY(RT), CONCOMITANT
we established the cell line and injected into NSG mouse reaching P3. NIMOTUZUMAB/VINORELBINE VERSUS MULTIPLE ELECTIVE
Regarding PDX model, using tissue directly from the patient, we were RADIOTHERAPY COURSES WITH CONCOMITANT
able to achieve P3, and tumors from patient and mouse slide were VINORELBINE/NIMOTUZUMAB FOR NEWLY DIAGNOSED
analyzed by our pathologist and were compatible, hence retaining the DIFFUSE INTRINSIC PONTINE GLIOMA (DIPG)
tumor primary characteristics.
Conclusions: We expect that this work will provide a useful and pow- Maura Massimino1 , Veronica Biassoni2 , Angela Mastronuzzi3 ,
erful tool to investigate new therapies using our primary cell line and Elisabetta Schiavello1 , Francesco Barretta4 , Lucia Quaglietta5 , Claudia
PDX model. Milanaccio6 , Emilia Pecori7 , Antonella Cacchione3 , Luna Boschetti1 ,
Valentina Di Ruscio3 , Silvia Chiesa8 , Giuseppe Scimone9 , Salvina
Barra10 , Lucia De Martino5 , Antonia Ramaglia11 , Stefania Picariello5 ,
EP340 / #1814 ANALYSIS OF THE GLOBAL SURVIVAL OF A Antonio Verrico6 , Ombretta Alessandro7 , Francesca Colombo7 ,
REFERENCE CENTER IN PEDIATRIC NEURO ONCOLOGY Sabina Vennarini7 , Marta Podda1 , Giovanna Gattuso1 , Giuseppe
DURING 10 YEARS Cinalli12 , Manila Antonelli13 , Piergiorgio Modena14 , Loris De Cecco15 ,
Francesca Buttarelli16 , Lorenza Gandola17
Maristela Facholi1 , Bruna Mançano1 , Carlos Almeida Junior1,2 , Lucas 1 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Unit, Milan,
Lourenço2 , Luiz Fernando Lopes3 , Rui Reis4 , Gustavo Teixeira5 Italy; 2 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Unit,
1 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil; 2 Barretos Milano, Italy; 3 Ospedale Bambino Gesù, Pediatric Oncology, Rome, Italy;
Cancer Hospital, Pediatric Neurosurgery, Barretos, Brazil; 3 Barretos Chil- 4 Fondazione IRCCS Istituto Nazionale dei Tumori, Clinical Epidemiology
dren’s Cancer Hospital, Pediatric Hospital, Bairro Paulo Prata, Barretos, San And Trial Organization, Milan, Italy; 5 Ospedale Santobono-pausilipon,
Paulo, Brazil; 4 Barretos Cancer Hospital, Molecular Oncology Research Cen- Neuroncology, Naples, Italy; 6 Ospedale Pediatrico Giannina Gaslini,
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S306 of S635 ABSTRACTS

Neuroncology, Genoa, Italy; 7 Fondazione IRCCS Istituto Nazionale dei Mohammed Almahmodi1 , Nahla Mobark1 , Demah A. Almowanes2 ,
Tumori, Pediatric Radiotherapy, Milan, Italy; 8 IRCCS Bambino Gesù Haya N. Alghunaim2 , Nad E. Kattan2 , Ahmad M. Saleh3 , Wael Abdel
Children’s Hospital, Department Of Paediatric Haematology/oncology, Rahman Aljabarat1 , Fahad Alotabi4 , Maqsood Ahmad4 , Amal S.
Rome, Italy; 9 Ospedale San Giovanni di Dio e Ruggi D’Aragona, Radio- Alyahya4 , Ayman Al Banyan4 , Abdulelah A. Alluhaybi4 , Mohammed
therapy, Salerno, Italy; 10 Policlinico S. Martino, Radiotherapy, Genoa, Rayis1 , Zaid G. Alnaqib1 , Ali Abdullah O. Ali Abdullah O. Balbaid5 ,
Italy; 11 Ospedale Pediatrico Giannina Gaslini, Radiology, Genoa, Italy; Amal Abdulsami S. Marie5 , Eric Bouffet6 , Musa Alharbi1
12 Ospedale Pediatrico Santabono-Pausilipon, Neurosurgery, Naples, 1 King Fahad Medical City, Comprehensive Cancer Centre, Department Of
Italy; 13 Università La Sapienza, Neuropathology, Rome, Italy; 14 Ospedale Pediatric Oncology And Bone Marrow Transplant, Riyadh, Saudi Arabia;
S.Anna, Genetics/pathology, San Fermo, Italy; 15 Fondazione IRCCS Istituto 2 King Fahad Medical City, Speech And Audiology Department„ Riyadh,
Nazionale dei Tumori, Experimental Oncology And Molecular Oncology, Saudi Arabia; 3 King Fahad Medical City, Training And Education Research
Milano, Italy; 16 Università La Sapienza, Radiological, Oncological And Department, Riyadh, Saudi Arabia; 4 King Fahad Medical City, Pediatric
Anatomo-pathological Sciences, Rome, Italy; 17 Fondazione IRCCS Istituto Neurosurgical Department, Riyadh, Saudi Arabia; 5 King Fahad Medical
Nazionale dei Tumori, Pediatric Radiotherapy Unit, Milan, Italy City, Radiation Oncology Department, Comprehensive Cancer Centre, dddd,
Saudi Arabia; 6 The Hospital for Sick Children, Division Of Hematology And
Background and Aims: The purposes of this trial were to evaluate the Oncology, Toronto, Canada
feasibility, response, PFS/OS of a randomized study comparing two dif-
ferent RT schedules for DIPG while administering the same systemic Background and Aims: Medulloblastoma MB is the most com-
treatment. mon childhood CNS tumor treated with combined therapy surgery
Methods: Patients: 2-21 years-old with a not-pretreated radiolog- with radiation and cisplatin chemotherapy causing significant hearing
ically verified DIPG (MRI blindly reviewed at diagnosis and every impairment (HI) with impact on child’s quality of life.
12 weeks thereafter) and symptoms duration below 6 months. Methods: Retrospective review for a total of 78 patients with con-
Biopsy was required if suggested by atypical imaging. Vinorelbine firmed MB diagnosis treated at KFMC between 2010 and 2020 (HI)
20 mg/m2+nimotuzumab 150 mg/m2 were administered weekly for was graded using the National Cancer Institute (NCI) Grades (1-
12 weeks; thereafter every other week until tumor progression 4). All patients received 6 weeks of risk adapted CSI radiotherapy
or for up to 2 years. Standard(ST) arm included focal RT at total concurrent with daily oral Etoposide. Patients were treated with 2
dose of 54Gy (1.8Gy/day); for local progression re-irradiation was different maintenance chemotherapy Group 1=30 patients (38.5%)
proposed at 19.8Gy, in case of dissemination craniospinal irradia- treated with German HIT-MED MB protocol 8 Maintenance cycles.
tion(CSI) at 36Gy was adopted. Experimental(SP) arm included three (Cisplatin /lomustine/VCR) Cumulative dose of Cisplatin 560 mg/m2
elective courses of RT at defined timepoints at 36Gy, 19.8Gy and Group 2= 48 patients (61.5%) treated with MB Saudi Arabian Pedi-
19.8Gy with possible reirradiation for relapse at 9 Gy. Incidences of atric Hematology Oncology Society (SAPHOS) protocol 6 Maintenance
local(L) and distant(D) progression were assessed in a competing risk cycle alternating A&B. cycle A: Cisplatin 90 mg/m2 x 1d & 3 weeks of
setting. daily oral Etoposide. cycle B: Cyclophosphamide x 2 d & Vincristine
Results: Aggregated preliminary results are given for 4 Italian centers. Cumulative dose of Cisplatin 270 mg/m2
54 pts were screened and 51 included, 27 in ST, 28 males, median age Results: A total of 78 patients completed the follow up duration up
7 years (range 3-17). Median time of observation was 17.9 months. to 99 months. The median age was 82 months with 66.7% male and
Twelve patients needed a shunt, 10 during treatment; 20 were biop- 33.3% female. Median time of onset of hearing decline was 7 months
sied, in 18 cases according local protocols. 19/20 tumors had H3.3 K27 after start of radiation therapy Significant (HI) (NCI grade 3/4) in 26.9%
mutation. 41 relapsed, 28 locally, 13 with a component of dissemina- of patients, and 23.1% required hearing aid use Using life table analy-
tion. 36 died, one for tracheotomy bleeding. SP irradiation was feasible sis: mean time to develop HI was significantly shorter 10.1 months and
and never produced significant radionecrosis. Median EFS/OS were 16.0 months in group 1, vs 43.6 months and 61.1month in group 2
7.3/12.9 months, respectively; EFS/OS at 1 year were 19.0%/57.3%, Conclusions: Our study showed that incidence of hearing impairment
not differing between patients with local vs. disseminated relapses. was not affected by high radiation dose however Higher Cisplatin
Patients submitted to biopsies had more dissemination (P=0.04) and cumulative dose in German HIT-MED protocol was associated with
less local progression (P=0.077). higher incidence and shorter time to develop HI than MB SAPHOS pro-
Conclusions: Treatment was feasible and OS confirmed previous tocol Our study will form base line for future studies to modify therapy
results obtained in a single center. Randomization results will be later related Toxicity and improve outcome of childhood MB in Saudi Arabia
reported.

EP343 / #49 MANAGEMENT OF CNS TUMOR WITH BCOR


EP342 / #48 INCIDENCE OF HEARING IMPAIRMENT IN INTERNAL TANDEM DUPLICATION WITH MULTIMODALITIES
CHILDHOOD MEDULLOBLASTOMA SURVIVORS TREATED AT THERAPY: SURGERY, INTENSIVE CHEMOTHERAPY, AND
KING FAHAD MEDICAL CITY KFMC SAUDI ARABIA RADIATION
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S307 of S635

Nahla Mobark1 , Musa Alharbi1 , Walid Ballourah1 , Abdulrahman 1 Barretos Cancer Hospital, Molecular Oncology Research Center, Barretos,
Alsultan1 , Ayman Al Banyan2 , Fahad Al Manjomi1 , Zaid G. Alnaqib1 , Brazil; 2 Barretos Cancer Hospital, Pathology Laboratory, Barretos, Brazil;
Fahad Alotabi2 , Ali Abdullah O. Balbaid3 , Malak Abedalthagafi, Md4 3 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil; 4 Barretos
1 King Fahad Medical City, Comprehensive Cancer Centre, Department Of Cancer Hospital, Molecular Diagnosis Laboratory, São Paulo, Brazil
Pediatric Oncology And Bone Marrow Transplant, Riyadh, Saudi Arabia;
2 King Fahad Medical City, Pediatric Neurosurgical Department, Riyadh, Background and Aims: Gliomas are a heterogeneous group of brain
Saudi Arabia; 3 King Fahad Medical City, Radiation Oncology Department, tumors which represents an important cause of cancer related death in
Riyadh, Saudi Arabia; 4 King Fahad Medical City and King Abdulaziz City children and young adults. The identification of gene fusions involving
for Science and Technology„ Genomics Research Department, Saudi Human tyrosine kinases (TK) receptors in glioma cases have been considered
Genome Project, Riyadh, Saudi Arabia of major importance because tumors harboring these rare genetic
events, may be targetable by specific drugs (TK inhibitors) which
Background and Aims: The 2021WHO CNS tumor classification showed promising antineoplastic effects. Unfortunately, the most fre-
includes CNS tumors with internal tandem duplications in the BCL6 quently technologies used for TK gene fusions detection needs high
corepressor (BCOR) gene as a new entity of CNS embryonal tumors quality material and are expensive. In the present study, we aimed to
labelled high-grade neuroepithelial tumors with BCOR alterations identify TK gene fusions (ALK, ROS1, RET, NTRK1, NTRK2 and NTRK3)
(HGNET-BCOR) are characterized by genetic aberrations in the BCOR using an RNA based technology (nCounterâ ) from formalin-fixed,
gene located at Xp11.4, leading to increased expression of BCOR paraffin-embedded (FFPE) glioma samples.
mRNA and distinct DNA methylation profiles. currently no agreement Methods: The RNA was extracted (RNeasy Mini Kitâ , Qiagen) from
on the optimal strategy to manage these rare tumors, which mostly 16 gliomas samples from infant, pediatric and young adults (0.9 to
occur in young children These tumors are usually treated as high grade 21 years) treated at Barretos Cancer Hospital, Brazil. Two different
glioma HGG with upfront radiation therapy with poor outcome nCounterâ panels (Elements Tagset) were used for TK gene fusions
Methods: We report 2.5 years old boy presenting with headache and detection. The findings were confirmed in nine cases using a commer-
vomiting. MRI showed a well-defined left cerebellar mass, hyperin- cial Next Generation Sequencing (NGS) panel (Archer FusionPlex Solid
tense in T2 and hypointense in T1, with restricted diffusion and no Tumorâ ).
spinal CSF seeding metastases. He underwent gross surgical resec- Results: We found one case with NTRK1 fusion based on the imbal-
tion of the tumor initial pathological diagnosis was epithelioid high ance of NTRK1 3’ and 5’ probes (Ratio: 7.15). The presence of NTRK1
grade malignant neoplasm. Brain tumor methylation classifier analysis fusion was confirmed by NGS, which identified the TPR-NTRK1 fusion
of resected tumor tissue confirmed a CNS tumor with BCOR internal in tumor RNA.
tandem duplication (WHO grade 4). Conclusions: nCounterâ showed to be a potential technology for the
Results: The patient was treated per COG ACNS0334 (3 induction identification of a TK fusions using low amount of RNA from a FFPE
cycles of vincristine, cyclophosphamide, cisplatin, etoposide, HDMTX, sample and could be applied in clinical routine of health centers in the
followed by consolidation with 3 cycles of carboplatin and thiotepa future for the detection of targetable gene fusions.
with autologous hematopoietic stem cell rescue). MRI brain before the
start of chemotherapy showed a small recurrent mass within the sur-
gical cavity. post-induction MRI detected stable-sized residual lesion in EP345 / #848 PROFILING PAEDIATRIC BRAIN TUMOUR AND
the surgical cavity; however, post-consolidation MRI showed complete MANAGEMENT IN A RESOURCE-LIMITED SETTING: A SINGLE
resolution of the residual mass. The patient subsequently received CENTRE EXPERIENCE IN UGANDA
craniospinal irradiation (36 Gy [CSI]) with a boost to the tumor bed
up to 54 GY. By the time of writing this report our patient is still in Richard Nyeko1 , Joyce Kambugu1 , Fadhil Geriga1 , Racheal Angom1 ,
complete remission. Jaques Van Herdeen2
Conclusions: Our case showed that this aggressive brain tumor may 1 Uganda Cancer Institute, Pediatrics, Kampala, Uganda; 2 Universitair

respond well to intensive multimodalities therapy Further case studies Kinderziekenhuis Antwerpen, Belgium, Pediatrics, Belgium, Belgium
and international prospective trials are needed to optimize the clinical
management of these rare tumors Background and Aims: The outcome and survival of children with
brain tumours in low-income countries are low. Besides, the epidemi-
ological and treatment data for paediatric brain tumours are grossly
EP344 / #1381 FUSION DETECTION OF PEDIATRIC lacking in these settings owing to a lack of resources and population-
HIGH-GRADE GLIOMA USING NANOSTRING based cancer registries. We assessed the clinical profile, treatment and
outcome of children with brain tumours in Uganda.
Daniel Moreno1 , Luciane Da Silva1 , Edilene De Andrade1 , Leo Methods: We retrospectively reviewed records of chil-
Biscassi1 , Murilo Bonatelli1 , Gustavo Berardinelli1 , Flávia De Paula2 , dren with brain tumours at the Uganda Cancer Institute
Iara Santana2 , Gustavo Teixeira2 , Adriane Evangelista1 , Bruna between 2017 and 2021. Patient and tumour characteris-
Mançano3 , Letícia Leal1 , Rui Reis4 tics, treatment and outcomes were sourced. In addition, we
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S308 of S635 ABSTRACTS

also surveyed the members of the multidisciplinary team Cell transplants (AuHCT). Surgery was initial modality of treatment in
(MDT). 87%. Radiotherapy was given to 63%. By univariate analysis factors
Results: Thirty-five paediatric brain tumour cases were identified, with predicting poor outcome were WHO grading and Extent of disease,
a median age of 8.0 years (IQR 4.0-11.0). Over one-half (57.1%) were while the factors predicting better outcome were type of resection
male, and the median duration of symptoms was six months (IQR and HDCx and AuHCT. In the present study group OS and Event free
3.0-8.5). The majority (62.9%) of the tumours were supratentorial. survival (EFS) at 1yr after diagnosis were 85 % and 82 % respectively
Craniopharyngioma was the most common brain tumour diagnosis .And the subgroup who underwent HDCx and AuHCT, the 2 yr OS and
(25.7%), followed by astrocytoma (14.3%), medulloblastoma (11.4%), EFS after diagnosis were 86 % and 81 % respectively. There was no
and ependymoma (8.6%) and pineoblastoma (8.6%). Surgical resec- Transplant related mortality.
tions were in 9(28.6%) of the cases, of which 6(17.1%) were gross Conclusions: Multimodality management including surgery,
total resections, and 4(11.4%) were subtotal resections. Ten (28.6%) chemotherapy and radiation therapy remains the cornerstone for
of the children received chemotherapy, while 6(17.1%) and 5(14.3%) management of Pediatric Brain Tumors. HDCx followed by AuHCT can
respectively were treated with radiotherapy and palliation. There was deliver optimal outcome in developing countries in the sub group of
an increasing trend in the multidisciplinary management of cases over High risk Pediatric Brain Tumors.
time. Only 17(48.6%) of the children with brain tumours were alive
and active in care, 13(37.1%) lost to follow up/abandoned treatment,
and 3(8.6%) died. The barriers to neuro-oncological care were lack of EP347 / #764 ACUTE TOXICITY OF CHEMOTHERAPY IN
neurosurgical resources (46.7%), imaging (46.6%), diversity of MDT INTRACRANIAL GERM CELL TUMOURS ACCORDING TO AGE
(26.7%), access to radiotherapy (20.0%) and pathology (20.0%).
Conclusions: Lack of neurosurgical resources, imaging, radiotherapy Gilles Palenzuela1 , Gabriele Calaminus2 , Didier Frappaz3 , Andreas
and histopathology, among others, continue to hamper the outcome of Peyrl4 , Nicolas Gerber5 , Solem Kristin6 , Devenney Irene7 , Garre
children with brain tumours in resource-limited settings - compounded Maria Luisa8 , Rolf Dieter Kortmann9 , Alapetite Claire10 , James C.
by high rates of treatment abandonment and loss to follow-up. Nicholson11 , Murray Matthew11 , Schiffler Camille12 , Cécile Faure
Conter13
1 CHU Arnaud de Villeneuve, Hématologie Et Oncologie Pédiatrque, mont-
EP346 / #426 PEDIATRIC BRAIN TUMORS: CLINICAL pellier, France; 2 University Children’s Hospital, Paediatric Haematology
PROFILE, MANAGEMENT STRATEGIES AND OUTCOME And Oncology, bonn, Germany; 3 Institute d’Hémato-oncologie Pédiatrique,
EXPERIENCE FROM A TERTIARY CARE CENTER, INDIA Hémato-oncologie Pédiatrique, Lyon, France; 4 Medical University of Vienna,
Department Of Paediatrics, vienna, Austria; 5 University Children’s Hos-
Swathi P M1 , Pooja Mallya1 , Shobha Badiger1 , Ravi Joshi1 , Shweta pital, Department Of Oncology, Zurich, Switzerland; 6 St. Olav University
Pathak1 , Monika Bhukar2 , Gayathri S1 , Sunil Bhat2 Hospital, Dept For Children And Adolescents, Trondheim, Norway; 7 BOND
1 Mazumder Shaw Medical Center, Paediatric Hemato-oncology And Bmt, Linköping University Hospital, Dept. Paediatric Oncology, Linköping, Swe-
Bangalore, India; 2 Mazumdar Shaw Medical Center, Paediatric Hemato- den; 8 Department of Haemato-Oncology Gaslini Children’s Hospital, Unit
oncology & Bmt, Bangalore, India Of Neurooncology, genova, Italy; 9 Hannover Medical School, Department
Of Paediatric Hematology/oncology, Hannover, Germany; 10 Paris & Pro-
Background and Aims: Pediatric brain tumors are the most common ton Centre, Orsay, Radiation Oncology Department Institute Curie, paris,
solid tumors and leading cause of mortality and morbidity in chil- France; 11 University Hospitals, Department Of Paediatric Haematology And
dren worldwide. With multimodality treatment overall survival (OS) is Oncology, Cambridge, United Kingdom; 12 Centre Léon Bérard, Department
around 70% in developed countries. We aim to describe the patterns of Of Biostatistics, Lyon, France; 13 IHOPE, Pediatry, bron, France
brain tumors in children, to evaluate affect of pretreatment factors and
the treatment itself on the outcome. Background and Aims: Central nervous system germ cell tumours
Methods: A retrospective detailed analysis was performed on series (GCT) usually develop in children, adolescents or young adults. The
of Pediatric Primary Brain Tumors who were referred for chemother- SIOP CNS GCT II European trial includes four courses of pre-
apy in our department from January 2014 to December 2021 based on irradiation chemotherapy. Whether chemotherapy-related acute tox-
presentation, histology, disease extent, treatment and response. icity (CRAT) depends on age remains debated.
Results: Of 68 cases, 66% were males, age ranging between 4 months Methods: CRAT was analysed in this trial according to three age
to 16 years. Medulloblastoma was the most common tumor (34 groups: children (aged ≤11 years), adolescents (aged 12-17 years)
%) followed by Optic nerve glioma (12%), Pilocytic Astrocytoma, and adults (aged ≥18 years) and to type of chemotherapy: carboPEI
Ependymoma and Atypical rhabdoid tumor(6% each).Presentation (alternating etoposide-carboplatin and etoposide-ifosfamide) for non-
with features of raised intracranial pressure was most frequent (68%) metastatic germinoma; PEI (cisplatin, etoposide and ifosfamide) for
followed by Ataxia (37%) and focal neurological deficit (20%). All of standard-risk non-germinomatous GCT (NGGCT); PEI and hyperPEI
68 patients received chemotherapy, of which 31% received High dose (higher dose of ifosfamide and etoposide) for high-risk or poorly
chemotherapy (HDCx) followed by Tandem Autologous Hematopoietic responsive NGGCTs.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S309 of S635

Results: Among 296 patients assessable for CRAT, 105 were children, Methods: Clinical data and tissue samples of a total of 30 primary cases
121 were adolescents and 70 were adults (age max 41 years).Tumour that were diagnosed as PXA (grade 2 and 3) between 2013 and 2021 in
types (and thus chemotherapy) varied across age-groups: adults Rogachev institute were provided for this study.
received mostly carboPEI (79% versus 62% of children and 62% of Results: Of the 30 cases, 9 (30%) were anaplastic PXA (A-PXA). Median
adolescents). Median chemotherapy cumulative doses/m2 were simi- follow up time is 4.0 years. The vast majority of tumors occurred
lar among the three groups. Delay in chemotherapy ≥7 days (mostly in a supratentorial location (n=28), most frequently the frontal lobe
for prolonged marrow aplasia) was noted in 27%, 38% and 19% (n=11), with rare posterior fossa (n=1) and spinal cord (n=1) tumors.
of children, adolescents, and adults, respectively. Grade ≥3 haema- The most frequent alterations were CDKN2A/B deletion (n=19; 68%;
tological and non-haematological CRAT was observed in 94%/31%, 95% CI 49 - 82) and BRAF p.V600E (n=17; 57%; 95% CI 39 - 73). Co-
97%/36% and 77%/21% of children, adolescents, and adults, respec- occurring two of this alterations was in 12 pts (43%; 95% CI 27 - 61).
tively. Grade ≥3 haematological and non-haematological CRAT was Gross total resection (GTR) was achieved for 11 (37%) pts. GTR was
observed in 90%/21%, 92%/42% and 100%/79% of patients treated the main favorable prognostic factor, 3-year PFS is 91% (95% CI 75 –
with CarboPEI, PEI, and PEI/hyperPEI, respectively. No toxic death was 100) compared to 17% (95% CI 5 – 57) for the rest. Neither CDKN2A/B
reported. In multivariate analysis, grade ≥3 overall (haematological deletion nor BRAF p.V600E was significantly associated with PFS after
and non-haematological) CRAT adjusted to treatment type was signif- adjustment for GTR using Cox proportional hazards model (hazard
icantly higher in adolescents treated with CarboPEI when compared ratios 1.1 (95% CI 0.4 – 3.7) and 0.6 (95% CI 0.2 – 1.8) respectively).
with adults: odds ratio: 0.1 [0.02-0.4] but not with children: odds ratio: PXA have higher PFS relative to A-PXA though it wasn’t significant (HR
0.9 [0.1-6.3]. 0.5 (95% CI 0.2 – 1.4) after adjustment for GTR).
Conclusions: Chemotherapy is safe in all age-groups, Conclusions: our results confirm that the vast majority of PXA harbor
but nevertheless with higher haematological and non- either CDKN2A/B deletion or BRAFV600E mutation. In histologically
haematological toxicity in adolescents when compared with defined PXA, tumor WHO grade and GTR remains a strong predictor of
adults. patient survival.

EP348 / #1687 MOLECULAR FINDINGS IN A SERIES OF EP349 / #502 CLINICAL FACTORS, MANAGEMENT AND
CHILDHOOD PLEOMORPHIC XANTHOASTROCYTOMAS WITH OUTCOMES OF PATIENTS UNDER 18 YEARS OLD WITH BRAIN
HISTOPATHOLOGICAL CONFIRMATION TUMORS: SINGLE CENTER EXPERIENCE IN PERU

Andge Valiakhmetova1 , Ludmila Papusha1 , Margarita Zaytseva2 , Claudia Pascual1 , Liliana Vasquez2 , Jose Hernandez3 , Esmeralda
Ekaterina Salnikova1 , Kirill Voronin2 , Liudmila Yasko3 , Alexander Leon3 , Julio Guevara3 , Josue Jimenez4
Druy4 , Alexander Karachunsky2 , Galina Novichkova5 1 Pan American Health Organization, Nmh, Lima, Peru; 2 Pan American
1 Dmitry Rogachev National Medical Research Center Of Pediatric Hema- Health Organization, Non-communicable Diseases, Washington D.C, United
tology, Oncology and Immunology, Department Of Pediatric Neurooncology, States of America; 3 Hospital Guillermo Almenara Irigoyen, Oncology, Lima,
Moscow, Russian Federation; 2 Dmitry Rogachev National Medical Research Peru; 4 Hospital Guillermo Almenara, Pediatrics, Lima, Peru
Center Of Pediatric Hematology, Oncology and Immunology, Moscow,
Russian Federation, Laboratory Of Molecular Oncology, Moscow, Russian Background and Aims: There are few published reports on clini-
Federation; 3 Dmitry Rogachev National Medical Research Center Of Pedi- cal factors, treatment, and survival in children and adolescents with
atric Hematology, Oncology and Immunology, Laboratory Of Molecular brain tumors in low- and middle-income countries in the region of
Biology, Moscow, Russian Federation; 4 Dmitry Rogachev National Medi- the Americas. This study aims to retrospectively analyze demographic
cal Research Center of Pediatric Hematology, Oncology and Immunology, and clinical factors related to treatment and survival in children and
Laboratory Of Molecular Oncology, Moscow, Russian Federation; 5 Dmitry adolescents diagnosed with brain tumors in a tertiary care center in
Rogachev National Medical Research Center Of Pediatric Hematology, Peru.
Oncology and Immunology, Deputy Director General For Scientific And Methods: All cases of patients under 18 years of age with brain tumors
Clinical Work, Head Of The Department Of Hematology, Medical Director, diagnosed in a single-center during 11 years (2007 to 2017) were
Moscow, Russian Federation retrospectively reviewed. Clinical and demographic variables were
analyzed to assess their association with OS and EFS. Variables includ-
Background and Aims: Pleomorphic xanthoastrocytoma (PXA) is a cir- ing age, gender, tumor histology, extent of surgery, origin and time to
cumscribed glioma arising more common in the cerebral hemispheres diagnosis were available for analysis using the Kaplan-Meier method
of children and young adults. Recent molecular studies demonstrate and the Cox hazards ratio regression.
an activating mitogen-activated protein (MAP) kinase mutation (most Results: Seventy-five patients were analyzed (40 were men and 35
frequently BRAF p.V600E hotspot mutation) with cooccurring deletion were women) with a mean age of 7.7 years (0.6-17 years). The
of CDKN2A/B encoding the p16 cell cycle regulator protein in most main clinical symptoms were headache (43 patients, 57.3%), vomit-
PXA. ing (42 patients, 56%), difficulty walking (36 patients, 48%), and visual
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S310 of S635 ABSTRACTS

disturbances (21 patients, 28%). The most frequent clinical signs were Conclusions: Patients with germline RB1 mutations are at risk
hydrocephalus (32 patients, 42.6%), cerebellar signs (28 patients, of developing tumours outside but also within the central ner-
37.3%), visual abnormalities (19 patients, 25.3%), and focal motor vous system (CNS), most commonly pineoblastomas and tumours
signs (18 patients, 24%). The median time to diagnosis was 24 weeks. of the suprasellar or parasellar regions. We have not found
Tumor resection was performed in 68 patients (90.6%), and 37 patients any published association between bilateral retinoblastoma and
(49.3%) received postoperative radiotherapy. The most frequent his- a brainstem tumour in these patients. The association of the
tological subtypes in our study were low-grade gliomas (33.7%) and RB1 germline mutation with this second tumour remains to be
medulloblastomas (29.7%). Overall survival at 1 and 5 years of dis- elucidated.
ease in our series were 78% (CI 95%, 0.67- 0.86) and 74% (CI 95%,
0.62-0.82) respectively and 5-year EFS was 62% (CI 95% 0.47-0.73).
Conclusions: Headache associated with vomiting, hydrocephalus, and EP351 / #1283 ACTIVATION OF THE TGFB1/ERK/MKNK1
cerebellar signs were the most frequent presenting symptoms and SIGNALING PATHWAY DESENSITIZES MYC-DRIVEN
signs in patients analyzed. The diagnosis was late; despite that, survival MEDULLOBLASTOMA CELLS TO ENTINOSTAT
was higher than other Latin American countries.
Nan Qin1 , Daniel Picard1 , Frauke-Dorothee Meyer1 , Sophia-Marie
Götz1 , Lena Blümel1 , Ute Fischer1 , Thomas Beez2 , Guido
EP350 / #1268 INCIDENTAL FINDING OF AN Reifenberger3 , Marc Remke1
ASYMPTOMATIC BRAINSTEM TUMOUR IN A 1 University Hospital Düsseldorf, Department Of Pediatric Oncology, Hema-

RETINOBLASTOMA PATIENT tology, And Clinical Immunology, Düsseldorf, Germany; 2 University Hospital
Düsseldorf, Department Of Neurosurgery, Düsseldorf, Germany; 3 University
Miriam Pavon Mengual1 , Vicente Santa-Maria Lopez1 , Marta Hospital Düsseldorf, Institue Of Neuropathology, Düsseldorf, Germany
Perez-Somarriba Moreno1 , Juan Pablo Muñoz Perez1 , Margarida
Simao Rafael1 , Jaume Catala Mora2 , Mariana Planells Alduvin3 , Marta Background and Aims: Resistance to chemotherapy is a common
Gomez Chiari3 , Ofelia Cruz1 , Andres Morales1 cause of treatment failure in cancer patients and a major problem
1 Hospital Sant Joan de Deu, Pediatric Oncology, Esplugues de Llobregat, facing current cancer research. Targeted modulation of oncogenic
Spain; 2 Hospital Sant Joan de Deu, Ophthalmology, Esplugues de Llobregat, signaling pathway may be used to systematically characterize drug
Spain; 3 Hospital Sant Joan de Deu, Radiology, Esplugues de Llobregat, Spain resistance mechanisms across tumor entities and may help to identify
new therapeutic strategies. Since the transcription factor MYC is aber-
Background and Aims: Incidental findings (IF) are previously undi- rantly activated in many cancers including pediatric malignant brain
agnosed abnormalities that are discovered unintentionally during an tumors, like medulloblastoma (MB), our study focused on MYC-related
examination obtained for a different indication. A recent prospective drug resistance.
observational study published in the United States in a large cohort of Methods: We used high-throughput drug screening and genome-wide
children found that 21.1% presented an IF during a brain magnetic res- dCas9-based transcriptional activation screening.
onance imaging scan (MRI), of which 3.9% required referral for further Results: We performed high-throughput drug screening using
study or intervention. our in-house semi-automated platform and identified the HDAC
Methods: In this report, we present a 3-year-old patient who was inhibitor Entinostat as a drug that shows promising effects in MYC-
diagnosed with a bilateral retinoblastoma with de novo germline RB1 driven MB. Investigating genome-wide dCas9-based transcriptional
mutation at 2 months of age. She underwent treatment with sys- activation screening, potential drug response modulators, mainly
temic and intra-arterial chemotherapy as well as cryotherapy and laser TGFB1/Erk/MKNK1 signaling including neural EGFL like 2 (NELL2),
therapy, achieving complete control of the disease. When the patient were discovered. For further validation, we stably overexpressed
was 30 months old, she had a follow up brain MRI scan showing an NELL2 in MYC-driven MB cells and treated overexpressing cells and
expansive lesion in the pons and bulbopontine junction with important the corresponding control cells with Entinostat. Using PI staining,
exophytic component suggesting a brainstem glioma. Review of previ- cell cycle status was tracked. Entinostat treatment led to modest
ous scans showed that the lesion had been present a year prior, albeit induction of cell death in MYC-driven MB control cells, and sig-
smaller. The patient was asymptomatic and had a normal neurological nificant reduced cell death in MYC-driven MB cells with NELL2-
examination. overexpression.
Results: Due to the slow growing trend of the lesion and the excel- Conclusions: We report that the combination of genetic and pharma-
lent performance status of the patient, it was decided to continue cological approaches is a powerful approach to study drug resistance.
with clinical and imaging surveillance. To this date, she continues to be Our data suggest that activation of the TGFB1/Erk/MKNK1 signaling
asymptomatic and the lesion has not changed significantly in follow up pathway desensitizes MYC-driven MB cells to Entinostat. Synergistic
scans although this IF adds an extra concern to the patient due to her targeting of TGFB1/Erk/MKNK1 signaling and MYC could therefore
cancer predisposition. provide a novel therapeutic option in this aggressive MB subtype.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S311 of S635

EP352 / #1802 PINEOBLASTOMA – THE EXPERIENCE IN A shire, United Kingdom; 2 University of Athens, Department Of Chemistry,
BRAZILIAN PEDIATRIC ONCOLOGY HOSPITAL Inorganic Chemistry Lab, Athens, Greece; 3 NCSR “Demokritos”, Sol-gel
Laboratory, Institute Of Nanoscience And Nanotechnology, Athens, Greece
Jéssica Rodrigues1 , Andrea Cappellano1 , Frederico Silva2 , Patricia
Dastoli3 , Milena Oliveira3 , Maria Teresa Alves4 , Nasjla Silva5 Background and Aims: Paediatric rhabdomyosarcoma is the most
1 IOP-GRAACC-UNIFESP, Paediatric Oncology, Sao Paulo, Brazil; 2 IOP/ common type of soft-tissue cancer in children. It originates in the
GRAACC/UNIFESP, Radiology, Sao Paulo, Brazil; 3 IOP/GRAACC/UNIFESP, head/neck area in 40% of cases. Despite the progress made in the diag-
Neurosurgery, Sao Paulo, Brazil; 4 IOP/GRAACC/UNIFESP, Pathology nosis and the treatment of moderate cases with 75% five-year survival
Department, Sao Paulo, Brazil; 5 IOP/GRAACC/UNIFESP, Pediatric rate, severe cases of rhabdosarcoma have lower survival rate (20%)
Oncology, Sao Paulo, Brazil and poor treatment outcome. Also, current chemotherapeutic agents
lack in specificity and selectivity, resulting in unpleasant side-effects
Background and Aims: Pineoblastomas are rare, malignant embry- for the child. Encapsulating the chemotherapy drugs within nanocon-
onal tumors that account for <1% of all pediatric brain tumors. The tainers (NCs) is one approach to improve drugs’ efficacy and their
study aim is to report the experience of a Brazilian pediatric oncology therapeutic outcome. Due to their nano-size and suitable modified sur-
hospital over 30 years. face, polymeric NCs can cross the blood-brain-barrier without causing
Methods: Twenty-nine children with pineoblastoma were treated any damage. The aim was to synthesize polymeric NCs capable of deliv-
between 1991 to 2021. After maximal surgical resection, the treat- ering chemotherapy drugs at the tumour site to increase their efficacy
ment was performed according to the patient’s age: under 3 to 5-years and reduce their side effects.
the treatment consisted of a modified Head Start backbone protocol Methods: Hollow NCs were synthesised following three steps: 1)
followed by myeloablative chemotherapy with autologous hematopoi- PMAA core synthesis, 2) Coating it with P(MAA-co-EGDMA-co-
etic stem cell rescue (AuHSCR). Focal Irradiation after AuHSCR was NIPAM) copolymers, and 3) Removal of the PMAA core to obtain hol-
reserved for children with residual tumors at the end of consolida- low NCs. Throughout the synthesis steps, polymeric NCs were charac-
tion. For older children, the protocol consisted of 36Gy craniospinal terized structurally by Fourier-Transform-Infrared-Spectroscopy and
irradiation followed by conventional chemotherapy with cisplatin, Dynamic-Light-Scattering, and morphologically by Scanning-Electron-
cyclophosphamide, and vincristine. We recorded pathological, radio- Microscopy. Daunorubicin -as gold-standard drug- and cisplatin were
logical, surgical, and clinical follow-up data. loaded in the nanocontainers. The NCs were used for the following bio-
Results: Eighteen (62%) patients were female. The median age was logical evaluations and rabdomysarcoma-TE671 cell-line: haemolysis
7.23 years (range 0.39 – 17.68). Six (20.6%) patients had metastatic assay, MTT assay, and fluorescence microscopy.
disease at diagnosis. The extent of resection was biopsy in six patients Results: The NCs’ compatibility and loading capacity were investi-
(treated before 2009), subtotal in 14 (48.2%), Near-total in 2 (6,8%), gated. The haemolysis assay showed <10% haemolysed RBCs for the
and Gross total resection in 6 (20.6%). Neoadjuvant chemotherapy concentrations of nanocontainers tested. Daunorubicin and cisplatin
was administered in one patient, who died before definitive resec- were loaded in NCs by 50-65%. Free NCs were shown to be non-
tion. Six patients underwent chemotherapy alone and twenty-two cytotoxic in MTT assay and loaded NCs showed good cellular uptake
received chemotherapy + radiotherapy. Six patients underwent AuH- in fluorescence microscopy.
SCR. Twelve (41.3%) patients experienced tumor relapse or progres- Conclusions: Re-synthesis of the polymeric NCs will take place
sion and died (5y-PFS 49,7%). As of April 2022, 14 patients (48.2%) to further optimise their loading capacity for a sustained drug
had succumbed to their tumor at a median follow-up of 1.9 years. The release and prolonged drug biodistribution, whilst additional exper-
3-year and 5-year Overall Survival (OS) was 57.7% and 43.6% respec- iments will be performed to evaluate their efficacy in other paedi-
tively. The 5y-OS stratified by age was 37,3% vs 53,7% (<5years vs atric brain tumour cell cultures and mouse-models using different
>5years; p=.47). Patients submitted to GTR and NTR had greater OS drugs.
(5y-OS Biopsy: 33,3%, STR 25,6%, NTR 100%, GTR 100%, P= .08).
Conclusions: In our series, unfavorable prognostic, as in the literature,
seems to be related to age and extent of resection. EP354 / #1367 ARE TARGETED THERAPIES CHANGING THE
OUTCOME IN PATIENTS WITH HGG?

EP353 / #1345 ENHANCEMENT OF CHEMOTHERAPY Elena Soques1 , Laia Serra1 , Vicente Santa-María1 , Andres Morales1 ,
DELIVERY AND OUTCOME IN PAEDIATRIC Marta Perez-Somarriba Moreno1 , Miriam Pavon Mengual1 , Sara
RHABDOMYOSARCOMA USING SMART POLYMERIC Perez2 , Nagore Gene3 , Cinzia Lavarino3 , Ofelia Cruz1
NANOCONTAINERS 1 Hospital Sant Joan de Deu, Pediatric Oncology, Esplugues de Llobregat,
Spain; 2 Institut de Recerca Sant Joan de Deu, Tractament Del Càncer
Sara Seriah1,2,3 , Eleni Efthimiadou2,3 , Maria Braoudaki1 Pediàtric, Barcelona, Spain; 3 Hospital Sant Joan de Deu, Developmental
1 University of Hertfordshire, Department Of Clinical, Pharmaceutical And Tumor Biology Laboratory, Esplugues de Llobregat, Spain
Biological Sciences / School Of Life And Medical Sciences, Hatfield, Hertford-
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S312 of S635 ABSTRACTS

Background and Aims: High-grade-gliomas (HGGs) are aggressive metachronous tumors during lifetime but limited knowledge still exists
neoplasms with dismal prognosis. Their treatment is based on maxi- regarding molecular findings in different tumors of an individual
mal safe surgical resection followed by radiation therapy and alkylating patient with RTPS.
drugs. Recent molecular discoveries are leading to new therapies as Methods: Retrospective review of 23 rhabdoid tumor patients diag-
promising strategies. The goal of our study is to evaluate the clinical nosed within 2001-2020 was performed with the focus on RTPS and
impact of a biological approach on the outcome of children with HGG. occurrence of the metachronous tumors. Clinical data was collected
Methods: We performed an institutional retrospective study including and molecular analysis of SMARCB1 status and methylation profiling
patients with histologic diagnosis of HGG. Rradiologic diagnosis alone were performed.
was used in unequivocal diffuse intrinsic pontine gliomas (DIPG). Clin- Results: Four patients out of 7 with confirmed RTPS were diag-
ical and biological data were analyzed. Survival was estimated by the nosed with metachronous rhabdoid tumor with a median 469 days
Kaplan-Meier method and compared by the logrank test. (176 -2311) from the primary diagnosis. Two patients (age 6 and 39
Results: A total of 103 children diagnosed with HGG between 0 months) were initially diagnosed with ATRT, one of them subsequently
and 18 years were studied. Fifty-seven patients were female. Biolog- developed rhabdoid tumor of the kidney (RTK) and one extracranial
ical studies (TP53/Histones/other; sequencing/immunohistochemical rhabdoid tumor (eMRT) of the bladder. Two patients diagnosed with
determinations), were performed in 85 patients (83.4%). The most fre- eMRT of paravertebral soft tissue and RTK respectively later devel-
quent diagnosis was Diffuse-midline-gliomas (DMG) in 66.7% patients oped ATRT. The tumors in all 4 patients showed a different pattern
(56-DIPG, 12-other-K27M-DMG), followed by WT-HGG (12, 11.8%); of genome-wide DNA methylation and copy number aberrations sug-
bithalamic, G34R and NTRK account for 2/2/1 patients (all 3%). No gestive of non-clonal origin between primary tumor and metachronous
histomolecular data was available in 17 patients (16.7%). Surgery malignancy. Patient diagnosed with eMRT (group ATRT- MYC) was con-
was performed in 71 patients (70.3%), only biopsy in 49. Radio- firmed to develop brain ATRT (ATRT–SHH). Patients with brain tumors
therapy was received by 86 patients, mainly focal (90%). Sixty-eight (ATRT-SHH, ATRT–TYR) developed RTK and eMRT with methylation
(67.3%) patients received chemotherapy. Biological agents (Beva- profile of ATRT-MYC. One patient is long term survivor, 19 years and
cizumab or Tyrosine-kinase inhibitors) were given empirically to 38.6% 12 years from the diagnosis of her ATRT and bladder MRT, respectively.
of patients. Targeted therapy was administered in 13 patients (12.9%). Conclusions: Patients with RTPS are at risk of developing
Re-irradiation at progression was performed in 23 patients (22.8%). metachronous rhabdoid tumor. Methylation profile of tumors differs in
The median OS was 12.9 months (95% CI, 11.7 to 17.0). Targeted single patient suggesting non-clonal origin of metachronous tumours.
therapies did not significantly influence OS or PFS. Supported by Grant of Czech Ministry of Health NU21-07-00419
Conclusions: In our series biological/targeted therapy did not trans-
late into improved outcomes for patients with HGG. Broad span of
time and therapies are limitations of this study. The poor prognosis of EP356 / #1070 USE OF COMPASSIONATE MEDICATION IN
these patients encourages to find new therapies to change their dismal CHILDREN WITH BRAIN TUMORS IN ISRAEL
outcome.
helen Toledano1,2 , Gadi Campino2,3 , Abed Abu-Quider4,5 , Hodaya
Cohen6 , Iris Fried7,8 , Myriam Ben-Arush9,10 , Rina Dvir11
EP355 / #1382 METHYLATION PROFILE CONFIRMS 1 Schneider Children’s Medical Center, Pediatric Hematology Oncology,
NON-CLONAL ORIGIN OF METACHRONOUS TUMORS IN RTPS Petach Tikva, Israel; 2 Tel Aviv University, Sackler School Of Medicine,
PATIENTS Tel Aviv, Israel; 3 Chaim Sheba Medical Center, Hemato-oncology Divi-
sion, Edmond & Lily Safra Children’s Hospital, Ramat Gan, Israel; 4 Soroka
David Sumerauer1 , Ales Vicha1 , Lenka Krskova2 , Katerina Vanova1 , University Medical Center, Pediatric Hematology Oncology, Beer-Sheva,
Michal Tichy3 , Josef Zamecnik2 , Lucie Slamova1 , Martin Kyncl4 , Israel; 5 Ben Gurion University of the Negev, School Of Medicine, Beer-
Michal Zapotocky1 Sheva, Israel; 6 Hadassah Medical Center, Pediatric Hematology-oncology,
1 Second Faculty of Medicine, Charles University in Prague, Department Of Jerusalem, Israel; 7 Shaare Zedek Medical Center, Pediatrics, Jerusalem,
Pediatric Haematology And Oncology, V Úvalu, Czech Republic; 2 Second Israel; 8 Hebrew University of Jerusalem, Faculty Of Medicine, Jerusalem,
Faculty of Medicine, Charles University and University Hospital Motol, Israel; 9 Rambam Health Care Campus, Pediatric Hematology Oncology,
Department Of Pathology And Molecular Medicine, Prague, Czech Republic; Haifa, Israel; 10 Technion - Israel Institute of Technology, Rappaport Faculty
3 University Hospital Motol, Second Medical Faculty, Department Of Neuro- Of Medicine, Haifa, Israel; 11 Tel-Aviv Sourasky Medical Center, Pediatric
surgery, Prague, Czech Republic; 4 University Hospital Motol, Department Of Hemato-oncology Department, Tel Aviv, Israel
Imaging Methods, Prague, Czech Republic
Background and Aims: Molecular testing of pediatric brain
Background and Aims: Rhabdoid Tumor Predisposition Syndrome tumors (PBT) is becoming more common, but few biological
(RTPS) is characterized by increased risk of development of rhab- agents are licensed for PBT. Correspondingly, there has been
doid tumors in individuals with germ line mutation of SMARCB1 an increasing off-label use of medications obtained compas-
and SMARCA4 genes. RTPS are prone to develop synchronous and sionately (CM) from drug companies. We aimed to investigate
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S313 of S635

the scale of use of CM for PBT in Israel and the associated Methods: Our reference centre patients’ cohort consisted of 97 clini-
challenges. cally annotated patients with HGG diagnosed between 2000 and 2021.
Methods: We surveyed all seven pediatric oncology centers in the Sanger sequencing was used for screening of the most common HGG-
country from January 2016- March 2022. We collected data on related oncogenic drivers; furthermore we employed whole genome
number of patients receiving off-label medications/year, medication methylation array (Illumina Infinium MethylationEPIC BeadChip) and
class, tumor type, frequency of drug re-orders, staff responsible for for selected samples RNA sequencing and expression profiling.
orders/re-orders, & whether adverse events (AE) or responses were Results: Based on H3 status and previous radiotherapy we separated
reported to the sponsor. our HGG cases into the RIG (radiation-induced glioma), H3mut and
Results: Number of patients on CM/year was as follows: 2016-2, 2017- H3wt groups. In contrast to H3mut(n=35) and RIG(n=11) that were
4, 2018-20, 2019-33, 2020-55, 2021-76 and 2022-73. The majority uniformly fatal, H3wt group contained a proportion of long-term sur-
of requests were for BRAF/MEK inhibitors(i) for low grade gliomas. vivors. In the H3wt group we found patients carrying driver mutations
Less frequent requests included NTRK/ROS/ALKi (9), immune check- in IDH1/2 (n=2) and BRAFV600E (7). Five young patients (under
point inhibitors (6), multiple TKi (2), SHHi (1), EGFRi (2), MTORi (4) & 3) consisted of 3 infant hemispheric gliomas (with NTRK and ROS1
thalidomide (1) . In 2 centers a study coordinator assisted with order- fusions), one gliomatosis cerebri and one brainstem anaplastic astro-
ing/ re-ordering since 2021; in all the others this was managed by the cytoma with MYB/QKI fusion. We also identified a rare EWSR1-PATZ1
neuro-oncologist. Re-orders were placed every 2-3 months. Only seri- gene fusion in one patient. Importantly, long-term survivors recruited
ous AEs were reported to the IRB & supplier, and no response data from these subgroups. On the contrary, four cases of MYCN GBM with
were requested by drug companies. Some patients remain on CM for poor prognosis presented in various locations: one disseminated, one
>5 years. During this period only 1 medication (Larotrectinib) entered gliomatosis cerebri and two with hemispheric tumour. We identified
the Israeli Health Basket for PBT. one patient with “hypermutated” glioblastoma and used targeted ther-
Conclusions: There has been an exponential increase in the use of CM apy with Nivolumab. In three samples of our patients with thalamic
for PBT over the last 7 years; this has not been reflected by licensing glioblastomas, we detected “loss of H3K27-trimethylation” caused by
of these drugs. The burden of managing the ordering and re-ordering is EZHIP overexpression. These tumours proved to be very aggressive
falling on physicians. Collection of data from these patients is sporadic with early metastatic recurrence and dismal prognosis.
or non-existent. A paradigm shift in the administration of CM using uni- Conclusions: Detailed characterization of H3 wild-type HGG is impor-
fied protocols that collect safety and efficacy data could expediate the tant for further understanding of their biological behaviour, diagnos-
licensing of drugs for PBT. tics, prognostication and identification of therapeutic targets.

EP357 / #489 MOLECULAR CHARACTERIZATION OF EP358 / #1817 PARENT-REPORTED DIAGNOSTIC PATHWAYS


UNIQUE BIOLOGICAL SUBGROUPS AMONG H3 WILD-TYPE IN CHILDREN AND ADOLESCENTS WITH A CNS TUMOR IN
HIGH-GRADE GLIOMAS DENMARK

Katerina Vanova1 , Michal Zapotocky2 , Lenka Krskova3 , David Kathrine Synne Weile1,2 , Louise Tram Henriksen3,4 , René Mathiasen5 ,
Sumerauer2 , Ales Vicha2 , Josef Zamecnik3 , Bela Malinova4 , Vladimir Henrik Hasle1 , Jeanette Winther4,6
Benes5 1 Aarhus University Hospital, Dept. Of Pediatrics And Adolescent Medicine,
1 Second Faculty of Medicine, Charles University in Prague, Department Of Aarhus N, Denmark; 2 Aarhus University, Department Of Clinical Medicine,
Pediatric Haematology And Oncology, V Úvalu, Czech Republic; 2 Second Aarhus N, Denmark; 3 Aarhus University Hospital, Department Of Pedi-
Faculty of Medicine, Charles University in Prague, Department Of Pediatric atrics And Adolescent Medicine, Aarhus, Denmark; 4 Aarhus University,
Haematology And Oncology, V Úvalu, Czech Republic; 3 Second Faculty of Department Of Clinical Medicine, Faculty Of Health, Aarhus, Denmark;
Medicine, Charles University and University Hospital Motol, Department 5 Rigshospitalet, Department Of Paediatrics And Adolescent Medicine,
Of Pathology And Molecular Medicine, Prague, Czech Republic; 4 Second Copenhagen, Denmark; 6 Danish Cancer Society Research Center, Childhood
Faculty of Medicine, Charles University and University Hospital Motol, Cancer Research Group„ Copenhagen, Denmark
Department Of Oncology, Prague, Czech Republic; 5 Second Faculty of
Medicine, Charles University and University Hospital Motol, Department Of Background and Aims: Tumors of the central nervous system (CNS)
Neurosurgery, Prague, Czech Republic are the second most common tumors among children with can-
cer. Diverse initial presenting symptoms complicate the diagnostic
Background and Aims: Paediatric high-grade gliomas (HGG) are char- process, which might result in a diagnostic delay leading to higher
acterised by the aggressive biological behaviour with dismal prognosis mortality and a long-term morbidity. Our aim is to map out the
of long-term survival 10-15%. Current molecular-biological diagnostic diagnostic intervals in Danish children and adolescents with CNS
approaches allow for more precise characterization and determination tumors to identify in which interval, the lag is present and where to
of new unique subgroups of HGG. Our aim was to identify novel and aim interventions, thus accelerating diagnostics and enhance timely
rare HGG subgroups within our institution cohort. diagnosis.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S314 of S635 ABSTRACTS

Methods: We identified all Danish patients aged 0-17 years, who had LC3-I to LC3-II conversion and autophagy receptor p62 degradation
survived a CNS tumor diagnosed from 2015–2019 through the Dan- assays. Then, key autophagy genes were knocked out by CRISPR/Cas9
ish Cancer Registry and the Danish National Patient Register. The gene-editing method. Responses to commonly used chemotherapy
custody-holding parent(s) at time of diagnosis, completed a web-based drugs of wild-type and knock-out cells were analyzed in detail and com-
questionnaire, consisting of questions on time (date) of first registered pared. In order to document migration and invasion patterns of these
symptoms, first contact to a physician and the date of diagnosis with a autophagy competent and defective cells, scratch assay and transwell
CNS tumor. In this preliminary study 24 parents participated (N=24). invasion assays were performed.
Results: The parent-reported timeline showed a Total Interval (TI, time Results: Autophagy has been evaluated and compared in pediatric and
from symptom onset to diagnosis) of median: 109.5 days, interquartile adult glioma cell lines. Results showed that adult and pediatric cell lines
range (IQR): 132,5 days. The Patient Interval (PI, time from symptom respond differently to a set of chemotherapy drugs. Chemical inhibition
onset to contact to a physician) of median: 28.5 days, IQR: 53,5 days; of autophagy resulted in different responses in adult and pediatric cell
and a Diagnostic Interval (DI, time from first contact to a physician to lines. Changes in drug efficacies were noted in autophagy competent
diagnosis) of median: 54.5 days, IQR: 115 days. Males tended to experi- versus deficient cells. Migration and invasion capacity of pediatric and
ence shorter TI than females, and patients under the age of 3 years had adult glioma cells were compared. Autophagic activity correlated with
a longer DI than patients above the age of 3 years. aggressive behavior in glioma cells.
Conclusions: Parent-reported diagnostic pathways showed varying Conclusions: p-GMs have poor prognosis and new treatment
ranges in the PI as well as the DI. The intervals ranged from hours to approaches are needed. Manipulation of drug resistance- and tumor
years, and varied by gender and age, supporting the fact that symptoms aggressivity-related pathways, such as the autophagy pathway, might
that occur in children with a CNS tumor vary as well as an urgent need lead to the development of novel combinatory drug regimens affecting
of better knowledge to recognize these tumors more timely. tumor growth and invasion.

EP359 / #854 COMPARATIVE ANALYSIS OF AUTOPHAGY IN E-Poster Topic: AS05 SIOP Scientific programme / AS05.n New
DRUG RESPONSES AND AGGRESSIVE BEHAVIOR OF ADULT Drugs/Experimental Therapeutics
VERSUS PEDIATRIC GLIOMA CELL LINES
E-POSTER VIEWING
Irem Yenidogan1,2,3 , Nesibe Peker1 , Bera Aygun4 , Gamze Deveci1 ,
Dondu Kirmizi1 , Fikret Asarcikli2 , Banu Oflaz Sozmen5 , Goktug EP360 / #440 ENTRECTINIB IN CHILDREN, ADOLESCENTS
Akyoldas6 , Ibrahim Kulac7 , Ihsan Solaroglu1,6 , Fatih Erbey2 , Devrim AND YOUNG ADULTS WITH EXTRACRANIAL SOLID OR
Gozuacik1,8 PRIMARY CNS TUMOURS HARBOURING NTRK1/2/3, ROS1, OR
1 Koç University, Koç University Research Center For Translational Medicine ALK FUSIONS: UPDATED DATA FROM STARTRK-NG
(kuttam), Istanbul, Turkey; 2 Koç University School of Medicine, Paedi-
atrics, Istanbul, Turkey; 3 Koç University, Koç University School Of Medicine, Ami Desai1 , Giles Robinson2 , Ellen Basu3 , Jennifer Foster4 , Amit
Istanbul, Turkey; 4 Istanbul University-Cerrahpasa, Istanbul University- Sabnis5 , Luke Maese6 , Janet Yoon7 , Thomas Cash8 , Christine
cerrahpasa, Cerrahpasa Medical Faculty, Istanbul, Turkey; 5 Koç University Pratilas9 , Yeming Wu10 , Daniel Morgenstern11 , Cornelis Van
School of Medicine, Department Of Paediatrics, Istanbul, Turkey; 6 Koç Uni- Tilburg12 , Michela Casanova13 , Quentin Campbell-Hewson14 , Arnauld
versity School Of Medicine, Department Of Neurosurgery, Istanbul, Turkey; Verschuur15 , Lynley Marshall16 , Dennis T. L. Ku17 , Nadege
7 Koç University School of Medicine, Department Of Pathology, Istanbul, Corradini18 , Huanmin Wang19 , Saibah Chohan20 , Alison Cardenas21 ,
Turkey; 8 Koc University, School Of Medicine, Istanbul, Turkey Katherine Hutchinson22 , Clare Devlin23 , Jade Wulff24 , Amar Gajjar25 ,
Elisabeth Fox26
Background and Aims: Pediatric diffuse gliomas are the most chal- 1 University of Chicago Medical Center, Pediatrics, Section Of Hematol-
lenging pediatric CNS tumors with therapy resistance and poor ogy/oncology/stem Cell Transplantation, Chicago, United States of America;
prognosis. Emerging evidence suggests that the molecular pathway 2 St. Jude Children’s Research Hospital, Division Of Neuro-oncology, Depart-

deregulated in pediatric glioma (p-GM) is different from that of adults. ment Of Oncology, Memphis, United States of America; 3 Memorial Sloan
Autophagy, a cellular clearance system, has been implicated in glioma Kettering Cancer Center, Department Of Pediatrics, New York, United
progression, yet its role in pediatric patients is not well documented. In States of America; 4 Texas Children’s Hospital, Department Of Pediatrics,
this study, we compared the autophagic capacity of adult versus p-GM Hematology-oncology, Houston, United States of America; 5 University of
cell lines. The contribution of autophagy to the aggressive phenotype California, Department Of Pediatrics, San Francisco, United States of Amer-
was evaluated. ica; 6 Primary Children’s Hospital, Department Of Pediatrics Division Of
Methods: The autophagic capacity of adult glioma cell lines T98, U87, Hematology/oncology, Salt Lake City, United States of America; 7 University
and LN2229 were compared to pediatric cell lines SF-188 and SF-8628 of California San Diego, Paediatrics, San Diego, United States of America;
using qPCR and immunoblotting of autophagy-related genes and pro- 8 Children’s Healthcare of Atlanta, Emory University School of Medicine,
teins. The dynamics of autophagy in these cells were evaluated using Aflac Cancer & Blood Disorders Center, Atlanta, United States of America;
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S315 of S635

9 Johns Hopkins University, Department Of Oncology. Division Of Pediatric months (95% CI 1.8–1.9) and median PFS was 27.2 months (95% CI
Oncology, Baltimore, United States of America; 10 Xinhua Hospital Affiliated 18.1–NE).
to Shanghai Jiaotong University School of Medicine, Department Of Pedi- Conclusions: Entrectinib continues to demonstrate rapid and durable
atric Surgery, Shanghai, China; 11 Hospital for Sick Children and University of responses in paediatric patients with extracranial solid or primary CNS
Toronto, Department Of Paediatrics, Toronto, Canada; 12 Heidelberg Univer- tumours harbouring target gene fusions. Acknowledgments: Medical
sity Hospital and German Cancer Research Center (DKFZ), Hopp Children’s writing support, under the authors’ direction, was provided by Lietta
Cancer Center Heidelberg (kitz), Heidelberg, Germany; 13 Fondazione IRCCS Nicolaides, PhD, of Ashfield MedComms, an Ashfield Health company,
Istituto Nazionale dei Tumori, Pediatric Oncology Unit, Milan, Italy; 14 Great and funded by F. Hoffmann-La Roche Ltd.
North Children’s Hospital, Royal Victoria Infirmary, Paediatric Oncology,
Newcastle upon Tyne, United Kingdom; 15 Hôpital de la Timone, Paedi-
atric Oncology, Marseille, France; 16 The Royal Marsden Hospital and The EP361 / #492 THE NEDDYLATION INHIBITOR MLN4924
Institute of Cancer Research, Children And Young People’s Unit, Surrey, ALTERS RING1B ACTIVITY IN EWING SARCOMA
United Kingdom; 17 Hong Kong Children’s Hospital, Department Of Paedi-
atrics & Adolescent Medicine, Hong Kong, China; 18 Institute of Pediatric Elisabet Figuerola Bou1 , María Sánchez-Jiménez1 , Pablo Táboas1 ,
Hematology and Oncology (IHOPe), Léon Bérard Cancer Centre, Lyon, Daniel J García-Domínguez2 , Pol Castellano-Escuder3 , Guillem
France; 19 Beijing Children’s Hospital, Surgical Oncology, Beijing, China; Pascual-Pastor4 , Monica Vilà4 , Angel Montero-Carcaboso4 , Enrique
20 Hoffmann-La Roche Limited, Pdd Data & Statistical Sciences (dss), Mis- De Álava2 , Lourdes Hontecillas-Prieto2 , Jaume Mora1,5 , Sara
sissauga, Canada; 21 Genentech, Inc., Clinical Safety, South San Francisco, Sánchez-Molina1
United States of America; 22 Genentech, Inc., Oncology Biomarker Devel- 1 Institut de Recerca Sant Joan de Deu, Pediatric Cancer, Sarcomas And
opment, South San Francisco, United States of America; 23 Roche Products Histiocitosis Group, Esplugues de Llobregat (Barcelona), Spain; 2 Hospital
Ltd., Pharma Development Oncology And Hematology (pdoh), Welwyn Gar- Universitario Virgen del Rocío, CSIC, CIBERONC, Institute Of Biomedicine
den City, United Kingdom; 24 Genetech, Inc., Oncology (pediatric), South San Of Seville (ibis), Sevilla, Spain; 3 University of Barcelona, Biomarkers And
Francisco, United States of America; 25 St. Jude Children’s Research Hospi- Nutritional & Food Metabolomics, Department Of Nutrition, Food Science
tal, Department Of Oncology, Memphis, United States of America; 26 St Jude And Gastronomy, Food Innovation Network (xia), Barcelona, Spain; 4 Institut
Children’s Research Hospital, Pediatric Oncology, Memphis, United States of de Recerca Sant Joan de Deu, Pediatric Cancer Treatment, Esplugues de
America Llobregat (Barcelona), Spain; 5 Pediatric Cancer Center Barcelona, Hospital
Sant Joan de Deu, Oncology, Barcelona, Spain
Background and Aims: Entrectinib, a CNS-penetrant oral
TRK/ROS1/ALK tyrosine kinase inhibitor, has demonstrated rapid Background and Aims: Ewing sarcoma (EwS) is a highly aggressive
and durable responses in children and adolescents with extracranial bone and soft tissue tumor affecting children, adolescents, and young
solid or primary CNS tumours harbouring target NTRK1/2/3, ROS1 or adults. The fusion oncoprotein EWSR1-FLI1 is the dominant onco-
ALK fusions from the STARTRK-NG trial (NCT02650401; phase 1/2). genic driver that acts as an aberrant transcription factor. EWSR1-FLI1
We present updated safety and efficacy data from this study. binds GGAA microsatellites causing enhancer remodeling and genome
Methods: Patients aged <22 years with relapsed/refractory extracra- reprogramming. Targeting the degradation of fusion oncoproteins or
nial solid or primary CNS tumours were enrolled. Endpoints assessed elements cooperating with EWSR1-FLI1 represent novel therapeu-
include: safety, in all patients who received ≥1 dose of entrectinib; tic approaches under current investigation. E3 ubiquitin ligases (E3)
blinded independent central review of objective response rate (ORR), culminate the final steps of the ubiquitin transfer cascade to a pro-
duration of response (DoR), time to response (TtR) and progression- tein substrate to promote its degradation by the proteasome. The
free survival (PFS), in patients harbouring NTRK1/2/3, ROS1, or ALK activation of some E3 require the post-transcriptional modification
fusions who received ≥1 dose of entrectinib and had ≥6 months of neddylation. MLN4924 is a small molecule inhibitor of neddylation
follow-up (enrolment cutoff: 03 June 2021; data cutoff: 03 Decem- that is highly active in EwS cellular models. We have described the
ber, 2021). Patients were classed as having complete/partial responses E3 RING1B is among the proteins cooperating with the oncogene
(CR/PR) or stable disease using RECIST (solid tumours) or RANO (CNS at enhancers. Here, we explore the effects of MLN4924 treatment
tumours). beyond E3 in EwS.
Results: The safety-evaluable population comprised 59 patients; Methods: Human Gene 2.0 ST microarray was used to charac-
median age: 6.0 years (range: 2.0 months to 20 years). Fifty-five terize transcriptional changes upon MLN4924 treatment in EwS
of 59 patients (93%) experienced ≥1 treatment-related adverse cells. In depth expression and functional studies were performed
event (TRAE), the most frequent being weight gain (37%); thirty-one to characterize the drug activity in vitro and in vivo. Chromatin-
patients (53%) had a Grade 3/4 TRAE. Thirteen patients experi- immunoprecipitation technique (ChIP) was additionally used to vali-
enced ≥1 fracture. There were no AE-related deaths, and no new date the effects of the drug on EWSR1-FLI1 targets.
safety signals were detected. In patients with fusion-positive tumours Results: We present the transcriptional effects of MLN4924 treat-
(n=36), ORR was 61% (95% CI 43.5–76.9; 9 CR and 13 PR), median ment on EwS cells, with DNA damage response and interferon
DoR was 25.4 months (95% CI 16.2–NE), median TtR was 1.9 signaling among the most significantly altered pathways. We
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S316 of S635 ABSTRACTS

further show RING1B among the E3 deregulated by the drug at a ing, with inconclusive but plausible utility for other facets of symp-
transcript and protein level. Our results indicate that the lack of tomatic relief. While there was a paucity of literature documenting
neddylation caused by the drug is behind the in vitro and in vivo anti-cancer effects in human patients, in vitro studies utilising paedi-
degradation of RING1B. Moreover, treatment of EwS cell lines with atric cancer cell models provide sufficient evidence to prompt in vivo
the inhibitor promotes RING1B loss from EWSR1-FLI1-activated studies.
targets as well as eviction of EWSR1-FLI1, causing transcriptional
defects.
Conclusions: MLN4924 deregulates E3, including RING1B, with crit- EP363 / #553 COMPARISON OF POST INDUCTION MRD
ical consequences on EWSR1-FLI1 activity, partially explaining the OUTCOMES OF PEDIATRIC B-ALL TREATED WITH
vulnerabilities of EwS cells to the drug. PEG-ASPARAGINASE VERSUS NATIVE E.COLI ASPARAGINASE

Ayesha Hanif1 , Neelum Mansoor2 , Kishwar Nadeem2 , Sidra


EP362 / #328 THE USE OF CANNABIS DERIVED MEDICAL Maqsood3 , Naeem Jabbar2
PRODUCTS IN THE TREATMENT OF CHILDREN’S CANCER: A 1 Indus Hospital and Health Network, Pharmacy, Karachi, Pakistan; 2 Indus
SYSTEMATIC REVIEW Hospital and Health care Network, Hematology/oncology, karachi, Pakistan;
3 Indus Hospital and Health care Network, Indus Hospital Research Centrei,

Nikolai Gajic1 , Cait Murphy2 , Sarah Ritchie1 , Charlie Saunders1 , karachi, Pakistan
Dermot Murphy3
1 University of Glasgow - GLASGOW, Medicine, GLASGOW, United Background and Aims: L-Asparaginase is one of the main components
Kingdom; 2 University of St. Andrews, Medicine, St Andrews, United of the multi-agent treatment regimen for childhood Acute Lymphoblas-
Kingdom; 3 Royal Hospital for Children, Oncology, Glasgow, United tic Leukemia(ALL). Conventional E. coli L-Asparaginase or Pegylated
Kingdom Asparaginase can be used for the treatment of ALL. Native E. coli L-
asparaginase is being used at The Indus Hospital but debate remains
Background and Aims: Legislative change to cannabis use has regarding its efficacy due to the high rate of positive post-induction
prompted significant interest into the therapeutic utility of cannabis- minimal residual disease (MRD) and localized hypersensitivity reac-
derived medical products, particularly in the field of oncology. tions despite being used in the regimen that is internationally followed.
However, much of this research has focused on adults, leaving Over the period of the last three years, out of 410 cases of pedi-
physicians and caregivers uncertain as to the safety and effi- atric B lymphoblastic leukemia (BALL), 154 (38 %) showed positive
cacy of cannabinoids amongst the paediatric demographic. To this MRD results at post-induction. This frequency of MRD positivity is
end, the aim of this review is to examine the scope of phar- relatively higher than in reported studies using a similar treatment
maceutical cannabis in treatment of paediatric cancer, evaluating protocol.
its utility as an anti-cancer therapeutic as well as symptom relief Methods: A retrospective study includes pediatric patients diagnosed
agent. with B lymphoblastic leukemia. Peg asparaginase which is internation-
Methods: This systematic review was conducted following the PRISMA ally manufactured given through an intramuscular route to 55 patients
guidelines. Key words were contrived and deployed, along with all syn- during the induction phase instead of L-asparaginase which is con-
onyms, across four electronic databases (PubMed, Web of Science, ventionally used in childhood leukemia regimens. MRD results of this
Embase, CINAHL). Initial searches yielded 2217 results. After de- cohort were compared with those who received locally manufactured
duplication, title and abstract screening, full text review and application E. coli asparaginase through the intramuscular route
of outlined exclusion and inclusion criteria, 30 papers were identified Results: A total of 111 patients were included, 55 patients received
and included. intramuscular peg-asparaginase and 56 patients received intramuscu-
Results: The 30 articles comprised of 16 clinical and 14 pre-clinical lar conventional E.coli asparaginase. 31 (56.4%) patients in the peg-
studies. The most frequently reported clinical outcomes were for asparaginase cohort versus 29(51.8%) patients in the E.Coli asparagi-
nausea and vomiting (n = 11), pain (n =4) and anorexia (n = 3). nase cohort show positive MRD results(p=0.628).Only one patient in
Clinical data regarding the anti-cancer effects were limited to just each cohort shows l-asparaginase induced adverse effects. The Peg-
2 case studies reporting 3 patients. Pre-clinical research described Asparaginase group had more positive MRD outcomes in high-risk
use of cannabinoid therapy in models of leukaemia (n = 7), osteosar- patients(26.8% vs 54.5%:p=0.009)
coma (n = 2), and neuroblastoma (n = 2) among other paediatric Conclusions: No significant differences in MRD were identified
cancers (n = 3). 4 studies provided evidence of increase cannabi- between the two groups. This study suggests that both forms
noid receptor (CBR) expression in the tumour cell lines and 8 stud- of L-Asparaginase do not have a significant impact on MRD out-
ies utilised CBR’s successfully as a therapeutic target to induce comes. Further prospective, randomized control trials are needed
apoptosis. to identify factors that impact treatment outcome negatively and
Conclusions: There is conclusive evidence for the effectiveness of assess the optimal treatment that reduces the rate of positive MRD
cannabis-derived medical products in chemotherapy-induced vomit- outcomes
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ABSTRACTS S317 of S635

EP364 / #389 A PILOT RANDOMIZED CONTROLLED TRIAL EP365 / #289 DRUG ACCESS REQUESTS FOR INNOVATIVE
OF VIRTUAL REALITY DISTRACTION TO REDUCE PROCEDURAL THERAPIES FOR PEDIATRIC ONCOLOGY PATIENTS IN CANADA
PAIN DURING SUBCUTANEOUS PORT ACCESS IN YOUTH WITH
CANCER Sandra Judd1 , Gabriel Revon-Riviere2 , Rebecca J Deyell3 ,
Magimairajan Issai Vanan4 , Lucie Pecheux5 , Alexandra P Zorzi6 , Raoul
Jennifer Stinson1 , Amos Hundert2 , Kathryn Birnie3 , Oussama Abla4 , Santiago7 , Thai Hoa Tran8 , Stephanie A Grover9 , Sarah Cohen-Gogo2 ,
Karyn Positano5 , Celia Cassiani2 , Sarah Llyod2 , Petra Tiessen6 , Chitra Daniel Morgenstern2
Lalloo2 , Lindsay Jibb2 1 The Hospital for Sick Children, Department Of Pharmacy, Toronto, Canada;

1 Hospital for Sick Children, Child Health Evaluative Sciences And Chronic 2 The Hospital For Sick Children, Temerty Faculty of Medicine, Univer-
Pain Program, Toronto, Canada; 2 Hospital for Sick Children, Child Health sity of Toronto, Peditatric Hematology Oncology, Toronto, Canada; 3 British
Evaluative Sciences, Toronto, Canada; 3 University of Calgary, Depart- Columbia Children’s Hospital / University of British Columbia, Pediatrics,
ment Of Anesthesiology, Perioperative And Pain Medicine, Calgary, Division Of Pediatric Hematology Oncology Bmt, Vancouver, Canada;
Canada; 4 Hospital for Sick Children, Division Of Haematology/oncology, 4 University of Manitoba / Cancer Care Manitoba, Pediatrics And Child
Toronto, Canada; 5 Hospital for Sick Children, Child Life, Toronto, Canada; Health, Winnipeg, Canada; 5 University of Alberta - Stollery Children’s
6 University of Toronto, Dept. Of Anesthesia & Pain Medicine, Toronto, Hospital, Pediatric Hematology Oncology, Edmonton, Canada; 6 Children’s
Canada Hospital, London Health Sciences Centre, Pediatrics, London, Canada; 7 CHU
of Quebec - Laval University, Pediatrics, Quebec, Canada; 8 CHU Sainte-
Background and Aims: Despite numbing with a E-Poster Topical anes- Justine, Pediatrics, Montreal, Canada; 9 The Hospital For Sick Children,
thetic, youth with cancer have reported pain and distress during needle Genetics And Genome Biology, Toronto, Canada
insertion into a subcutaneous port (SCP). Virtual reality (VR) has been
shown to reduce acute pain in youth. However, research is limited sur- Background and Aims: Innovative therapies are needed to alleviate
rounding the use and safety of VR for procedural pain management the burden of pediatric cancer despite the constant expansion of their
specifically for youth with cancer. This study aimed to determine the indications in the era of precision medicine. The lack of clinical trials
feasibility of implementing a randomized controlled trial (RCT) of VR or approved indications in children for developed therapeutic agents
for SCP needle insertion in youth with cancer and estimate preliminary is a significant barrier for access. This is the first analysis of an exten-
treatment effects. sive Canadian review of innovative drug requests and focuses on drugs
Methods: A single-site pilot RCT comparing VR to iPad distraction requested and their indications.
was conducted in youth aged 8-18 years undergoing cancer treat- Methods: We conducted a retrospective review of access requests
ment. Feasibility (e.g., accrual rate, reasons for non-participation, for anti-cancer therapies (not Health Canada-approved for pediatric
safety) was reported descriptively. Intervention acceptability was indications at the time of application) from 2013 to 2020. Patient,
evaluated by youth, parent, and nurse self-report. Preliminary inter- disease characteristics, drug information and request details were col-
vention effectiveness for youth-reported pain intensity, distress, and lected. We excluded cytotoxic chemotherapy, cellular therapies, and
fear was determined using logistic regression models with out- cytokines.
comes compared between groups using preprocedural scores as Results: We included 312 drug access requests from nine Cana-
covariates. dian tertiary care pediatric oncology centers. Requests increased
Results: Twenty participants were randomized per group, with an aver- from 7 in 2013 to 90 in 2020. Forty-nine different agents were
age age of 12 years. The most common diagnosis was ALL. Most requested: most commonly, targeted agents (including multi tyrosine-
eligible participants (62%) participated, and one withdrew after ran- kinase inhibitors (n=67), inhibitors of MEK (n=64), BRAF (n=25),
domization to the iPad group. Participants cited “not interested”, or mTOR (n=24)), immuno-therapy (37) and antibody-drug conjugates
“comfortable without distraction” as reasons for non-participation. No (22). Combination therapies accounted for 33% (n=103) of the
safety issues were reported. Nurses, parents, and youth found both requests with n=42 for combinations of two innovative therapies.
interventions to be acceptable, with the VR participants reporting sig- Requests were made for patients with solid tumors (39%), CNS tumors
nificantly higher immersion in the distraction environment (P=0.0318). (31%), leukemias/lymphomas (20%), plexiform neurofibromas (7%) and
Although not statistically significant, more VR group participants indi- Langerhans cell histiocytosis (3%). Actionable targets were identified
cated no pain (65% vs. 45%) and no distress (80% vs. 47%) during by molecular profiling in 41% of cases. Most common targetable alter-
the procedure compared with the iPad group. Fear was similar across ations were RAS/MAPK pathway activations (42%, defined as clinical
groups. diagnosis of NF1 or tumor harboring BRAF fusion or mutations). Other
Conclusions: VR was feasible and acceptable to implement as an common actionable genomic alterations included ALK/ROS alterations
intervention during paediatric SCP access. Preliminary effectiveness (7%), defects in DNA repair deficiency pathways and alterations in
results indicate that VR may reduce pain and distress compared with PIK3CA, SWI-SNF subunits, JAK1/2, FLT3, CDK and FGFR genes.
iPad distraction. These data will inform design of a future full-scale Conclusions: There is an increasing number of drug access requests for
RCT. pediatric cancers and non-malignant diseases that are not approved for
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S318 of S635 ABSTRACTS

children or available in clinical trials in Canada. Agents targeting the HSQC spectra of XIAP showed moderate binding with A4, supporting
RAS/MAPK pathways were the most frequently sought after. degradation rather than binding inhibition of XIAP by A4. In MYCN-
amplified neuroblastoma xenografts, A4 10mg/kg twice-weekly was
well-tolerated and significantly prolonged survival. A4 also showed
EP366 / #988 DEGRADATION OF XIAP AS A NOVEL synergism with second-line agents vincristine and topotecan, with 3-
TREATMENT STRATEGY IN HIGH-RISK NEUROBLASTOMA and 6.5-fold effective dose-reduction in BE(2)-C and KELLY, respec-
tively.
Zhang’E Choo1,2 , Megan Wong3 , Nurul Ali Ahamed1,2 , Congbao Conclusions: Engagement and degradation of XIAP by ARTS mimetics
Kang4 , Chik Hong Kuick5 , Kenneth Chang3,5,6 , Sarit Larisch7 , Amos is a novel and effective therapeutic strategy against MYCN-amplified
Loh3,6,8 , Zhi Xiong Chen1,2,3,9 neuroblastoma with intrinsically high XIAP, either alone or in combina-
1 National University of Singapore, Department Of Physiology, Yong Loo Lin tion with current standard-of-care agents.
School Of Medicine, Singapore, Singapore; 2 National University of Singa-
pore, Nus Centre For Cancer Research, Yong Loo Lin School Of Medicine,
Singapore, Singapore; 3 KK Women’s and Children’s Hospital, Viva-kkh EP367 / #994 TARGETING MUTANT DICER TUMORIGENESIS
Paediatric Brain And Solid Tumour Programme, Children’s Blood And IN PLEUROPULMONARY BLASTOMA VIA INHIBITION OF RNA
Cancer Centre, Singapore, Singapore; 4 A*STAR, Experimental Drug Develop- POLYMERASE I WITH CX-5461
ment Centre, Singapore, Singapore; 5 KK Women’s and Children’s Hospital,
Department Of Pathology And Laboratory Medicine, Singapore, Singapore; Kia Hui Lim1 , Megan Wong2 , Esther Hee2 , Huiyi Chen3 , Chik Hong
6 National University of Singapore, Duke Nus Medical School, Singapore, Sin- Kuick3 , Sze Jet Aw3 , Kenneth Chang2,3,4 , Nurfarhanah Syed
gapore; 7 University of Haifa, Cell Death And Cancer Research Laboratory, Sulaiman2,5 , Sharon Low2,4,5 , Septian Hartono4,6 , Anh Nguyen Tuan
Department Of Human Biology And Medical Sciences, Haifa, Israel; 8 KK Tran7 , Summaiyya Ahamed4,8 , Joyce Lam2,4,9 , Shui Yen Soh2,4,9 ,
Women’s and Children’s Hospital, Department Of Paediatric Surgery, Sin- Katherine Hannan10,11 , Ross Hannan10,11,12 , Lucy Coupland10 , Amos
gapore, Singapore; 9 National University Health System, National University Loh2,4,13
Cancer Institute, Singapore, Singapore, Singapore 1 National University of Singapore, Yong Loo Lin School Of Medicine, Singa-
pore, Singapore; 2 KK Women’s and Children’s Hospital, Viva-kkh Paediatric
Background and Aims: XIAP, the most potent mammalian inhibitor Brain And Solid Tumour Programme, Children’s Blood And Cancer Centre,
of apoptosis protein (IAP), critically restricts developmental culling of Singapore, Singapore; 3 KK Women’s and Children’s Hospital, Department
sympathetic neuronal progenitors through apoptotic inhibition, and Of Pathology And Laboratory Medicine, Singapore, Singapore; 4 National
is correspondingly overexpressed in 64% of MYCN-amplified neurob- University of Singapore, Duke Nus Medical School, Singapore, Singapore;
lastoma tumors. ARTS is the only XIAP-antagonist that directly binds 5 National Neuroscience Institute, Department Of Neurosurgery, Singa-
XIAP and promotes its degradation via the ubiquitin-proteasome sys- pore, Singapore; 6 National Neuroscience Institute, Singapore, Department
tem (UPS). We therefore hypothesized that XIAP antagonism via ARTS Of Neurology, Singapore, Singapore; 7 National Cancer Centre Singapore,
mimetics may be an effective treatment for high-risk neuroblastoma. Department Of Oncologic Imaging, Singapore, Singapore; 8 KK Women’s and
Methods: Pan-IAP (SMAC mimetics) and XIAP-specific inhibitors Children’s Hospital, Department Of Diagnostic And Interventional Imag-
(ARTS mimetics) were screened against commercial and patient- ing, Singapore, Singapore; 9 KK Women’s and Children’s Hospital, Paediatric
derived neuroblastoma cell lines, and ranked by efficacy. IAP protein Haematology Oncology Service, Children’s Blood And Cancer Centre, Sin-
expression and cell death were assessed via Western blot, clono- gapore, Singapore; 10 The Australian National University, Department Of
genic and biochemical apoptosis assays. Following CRISPR/Cas9- Cancer Biology And Therapeutics, The John Curtin School Of Medical
mediated XIAP knock-in, XIAP-compound interaction was evaluated Research, Canberra, Australia; 11 The University of Melbourne, Depart-
using NanoBRET™ target-engagement, degradation and ubiquitina- ment Of Biochemistry And Molecular Biology, Parkville, Australia; 12 Peter
tion assays, and NMR spectroscopy. Pharmacokinetics and survival MacCallum Cancer Centre, Na, Melbourne, Australia; 13 KK Women’s and
studies were performed with orthoE-Poster Topic patient-derived Children’s Hospital, Department Of Paediatric Surgery, Singapore, Singapore
xenografts. Drug-interaction indices were computed using Chou-
Talalay method. Background and Aims: Development of novel therapeutics for
Results: Antagonism of XIAP (but not other IAPs) with ARTS mimet- pleuropulmonary blastoma (PPB) has been limited by a lack of
ics, triggered apoptotic death in neuroblastoma cells. XIAP silencing actionable molecular targets and biologically and physiologically-
induced apoptosis and over-expression conferred protection from representative disease models, so outcomes for these Dicer-mutated
drug-induced apoptosis. Among IAP antagonists tested, ARTS mimetic malignancies remain poor. With recent evidence of Dicer-mediated
A4 was most effective against high XIAP-expressing neuroblastoma RNA polymerase I (Pol I) regulation as a synthetic lethal tar-
cells (BE(2)-C, KELLY), and least toxic towards liver and bone marrow- get in mutant Dicer cells, we hypothesized that Pol I inhibition
derived control cells. NanoBRET™ and MG132 assays showed XIAP could abrogate mutant Dicer-mediated accumulation of stalled
engagement and degradation via UPS within 15 minutes of A4 polymerases to induce p53-mediated cell death in PPB tumor
exposure, in a dose-dependent manner. NMR analysis on 1 H-15 N- cells. Thus, we sought to test the utility of Pol I antagonism
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S319 of S635

with first-in-class inhibitor CX-5461 as a treatment strategy for patient for consideration to use a matched, FDA-approved targeted
PPB. agent.
Methods: From a type III PPB tumor sample, an orthoE-Poster Topic Results: To date, 104 patients have received Riley Precision Oncology-
patient-derived xenograft (PDX) model was developed using a novel recommended targeted therapy. A subset of these patients has met
sub-pleural implantation method in NOD-scid mice. Models were char- the Exceptional Responders Initiative definition: tumor regression of at
acterized with micro-magnetic resonance imaging and histopathology, least 30% for six months or more, with an agent that typically results in
and DICER1 variants were profiled in patient and PDX tumors via tar- responses in <10% of individuals on the same treatment. Exceptional
geted next-generation sequencing. Tolerance of dosing regimens were responses were observed in patients with sarcomas and CNS tumors
compared in tumor-bearing animals using Kaplan-Meier method, and with the following biomarker-drug pairings: ALK mutations/gene
treatment response evaluated with immunoblotting and immunohisto- fusions/over-expression – Crizotinib; CDK4 pathway mRNA – Palboci-
chemistry for apoptotic markers. clib; MET gene fusions / over-expression – Cabozantinib; PMS2 gene
Results: PDX tumors retained allele frequency of RNaseIIIa and IIIb loss / high tumor mutational burden – Pembrolizumab; BRAF muta-
hotspot loss-of-function mutations over serial passages, and recapitu- tions – Dabrafenib + Trametinib; BRAF gene fusions – Trametininb;
lated the microenvironment and cardiorespiratory impact of intrapleu- NF1 mutations – Selumetinib; NTRK3 gene fusions – Larotrectinib.
ral PPB tumors. In PDX tumors, 24-hours’ exposure to CX-5461 Conclusions: Pediatric exceptional responders were predicted by the
significantly reduced H3K9 di-methylation associated with silencing presence of FDA recognized Level 1-3 DNA/RNA biomarkers in the
of rDNA repeats (P=0.005), and significantly increased nuclear p53 patients’ tumor genome. These results demonstrate conclusively that
expression (P=0.04) in treatment versus control groups (n=4 each). many high-risk pediatric patients with solid tumors can benefit from a
At the optimum tolerated dosing regimen of 30mg/kg CX-5461 3 Precision Oncology approach.
times/week, PDX mice treated with CX-5461 had significantly smaller
and less hemorrhagic tumors than controls, with significantly reduced
frequency of nuclear Ki67 staining (P=0.03) (n=5 per group). EP369 / #536 A PHASE I STUDY OF NAB-PACLITAXEL
Conclusions: CX-5461-mediated Pol I inhibition induced regression of COMBINED WITH GEMCITABINE FOR PEDIATRIC
PPB PDX tumors, decreased H3K9 methylation, and enhanced p53- RELAPSED/REFRACTORY SOLID TUMORS
mediated cell death. Together, this evidence supports the efficacy of
Pol I inhibition as a tolerable and feasible treatment approach for Jonathan Metts1 , Kevin Ginn2 , Carrye Cost3 , Brittani Froug4 , Amanda
Dicer-mutated PPB. Watt5 , Melissa Schink5 , Stacy Senn5 , Thomas Cash6
1 Johns Hopkins All Children’s Hospital, Cancer And Blood Disorders Insti-
tute, St Petersburg, United States of America; 2 Children’s Mercy Hospitals
EP368 / #282 PROSPECTIVE IDENTIFICATION OF and Clinics, Pediatric Hematology, Oncology, And Bone Marrow Transplan-
EXCEPTIONAL RESPONDERS IN HIGH-RISK PEDIATRIC tation, Kansas City, United States of America; 3 University of Colorado
PATIENTS WITH CANCER School of Medicine, Colorado Children’s Hospital, Center For Cancer And
Blood Disorders, Aurora, United States of America; 4 Johns Hopkins All
Mark Marshall1 , Amy Helvie1 , Michael Ferguson1 , Lisa Langsford1 , Children’s Hospital, Clinical And Translational Research Organization, St
Jennifer Ivanovich2 Petersburg, United States of America; 5 Children’s Healthcare of Atlanta,
1 Indiana University School of Medicine, Pediatrics, Indianapolis, United Aflac Cancer And Blood Disorders Center, Atlanta, United States of Amer-
States of America; 2 Indiana University School of Medicine, Medical And ica; 6 Children’s Healthcare of Atlanta, Emory University School of Medicine,
Molecular Genetics, Indianapolis, United States of America Aflac Cancer & Blood Disorders Center, Atlanta, United States of America

Background and Aims: Despite a high cure rate for most childhood Background and Aims: The maximum tolerated dose (MTD) of
cancers, relapsed and high-risk disease have a much worse progno- nab-paclitaxel in children with relapsed/refractory solid tumors
sis. In adults, genomic tumor profiling can significantly improve poor is 240 mg/m2 given weekly three out of four weeks, which is
patient response rates following relapse by identifying gene alterations significantly higher than adult dosing. We aimed to determine
which can be effectively treated with targeted drugs. These patients the MTD of nab-paclitaxel combined with gemcitabine in this
are often characterized as “exceptional responders”. We report that population.
tumor genomic profiling can prospectively identify exceptional respon- Methods: Patients with relapsed/refractory solid tumors outside the
ders in pediatric patients with cancer. central nervous system enrolled on a Phase I multicenter trial. Eligi-
Methods: Patients with relapsed or aggressive solid tumors were bility included age 6 months to 30 years old, measurable or evaluable
enrolled into the Precision Oncology program at Riley Hospital for disease, and adequate bone marrow, kidney, and liver function. Three
Children. DNA and RNA samples were obtained from FFPE pre- dose levels of nab-paclitaxel were evaluated, 180 [dose level (DL) 1],
served tumor samples and sequenced using NexGen techniques in 210 (DL2), and 240 (DL3) mg/m2 , using a rolling six design, and were
CLIA-certified laboratories. The presence of actionable DNA variants administered alongside fixed-dose gemcitabine intravenously on days
or proto-oncogene mRNA over-expression was used to qualify each 1, 8, and 15 of 28-day cycles.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S320 of S635 ABSTRACTS

Results: Nineteen patients were enrolled and received treatment; 17 was able to continue chemotherapy, immunotherapy or chemo-
were evaluable for dose-limiting toxicity (DLT). The median age was 14 immunotherapy. Before start treatment twelve (66.6%) patients had
years (range: 3-21). Common tumor diagnoses included osteosarcoma <50,000/mm3 platelets (9,000-85,000/mm3). The mean starting dose
(N=10) and rhabdomyosarcoma (N=4). The median number of cycles of Romiplostim® was 4.84 mcg/kg/week (2.7-7.5) and the maximum
received was 2 (range: 1-6). Initially gemcitabine was given at 1000 dose during was 8.14 mcg/kg/week (4.3-10). Five patients (27,7%)
mg/m2 ; however, 2/5 patients evaluable at DL1 had hematologic DLTs showed >50,000/mm3 platelets on week 3, eight (44.4%) on week
including thrombocytopenia and epistaxis, and neutropenia. The study 6 and 11 out of 17 patients (64.7%) patients achieved platelets
was amended to reduce gemcitabine to 675 mg/m2 /dose. No DLTs were >50,000/mm3 on week 12. The mean duration of treatment was 4,69
seen on post-amendment DL 1 and 2. One of 6 patients enrolled on DL3 months. We did not observe any adverse effects limiting the use of
experienced a DLT, a malignant pleural effusion present at enrollment Romiplostim®.
that worsened to grade 3 during cycle 1. Dose level 3 was declared the Conclusions: Romiplostim® was effective and safe in the treatment of
MTD. Common cycle 1 drug-related toxicities were mainly hematologic TRT. The use of Romiplostim® in heavily pre-treated patients with HR-
in nature, though post-cycle 1 toxicities leading to treatment discon- NB may be crucial for access to salvage therapies.
tinuation included elevated transaminases, peripheral neuropathy, and
paclitaxel-associated acute pain syndrome. One patient remains on
active treatment. EP371 / #599 LIPID REPLACEMENT THERAPY FOR THE
Conclusions: The MTD was determined to be nab-paclitaxel 240 TREATMENT OF DIFFUSE MIDLINE GLIOMA
mg/m2 with gemcitabine 675 mg/m2 on days 1, 8 and 15 of 28-day
cycles. An expansion study at the MTD is underway. Claudia Paret1 , Sara Ersali2 , Roger Sandhoff2 , Khalifa El Malki1 , Lea
Roth1 , Pia Wehling1 , Barbara Fliss3 , Adina Lepădatu4 , Katrin
Frauenknecht4 , Jörg Faber1
EP370 / #1701 USE OF ROMIPLOSTIM® IN 1 Center for Pediatric and Adolescent Medicine, University Medical Cen-
THERAPY-RELATED THROMBOCYTOPENIA IN PATIENTS WITH ter of the Johannes Gutenberg-University Mainz, Department Of Pediatric
HIGH-RISK NEUROBLASTOMA Hematology/oncology, Mainz, Germany; 2 German Cancer Center, Lipid
Pathobiochemistry, Heidelberg, Germany; 3 University Medical Center of
Ana Jose Navarro Garcia1 , Juan Pablo Muñoz Perez1 , Cristina the Johannes Gutenberg-University Mainz, Institute Of Legal Medicine,
Larrosa1 , Monica Sanchez Celma2 , Vicente Santa-María1 , Maite Mainz, Germany; 4 University Medical Center of the Johannes Gutenberg-
Gorostegui1,3 University Mainz, Pathology, Mainz, Germany
1 Pediatric Cancer Center Barcelona, Pediatric Oncology, Esplugues de
Llobregat, Spain; 2 Pediatric Cancer Center Barcelona, Pediatric Oncol- Background and Aims: Thus far, genomic, proteomic and epige-
ogy Pharmacy, Esplugues de Llobregat, Spain; 3 Pediatric Cancer Center netic analyses have failed to identify new effective therapies for
Barcelona, Pediatric Oncology, Esplugues de llobregat, Spain H3K27M-mutant diffuse midline gliomas (DMG). Lipids are gener-
ally not exploited for therapy approaches, even if glycosphingolipids
Background and Aims: Therapy-related thrombocytopenia (TRT) is (GSL) are altered in brain tumours and GSL metabolic reprogramming
a major problem in children receiving chemo/radiotherapy and fre- drives neural differentiation. Here we determined the composition of
quently causes delays of treatments and/or dosage reduction. Romi- DMG compared to healthy pontine tissue and exploited the altered
plostim® is a thrombopoietin receptor agonist approved by the Food composition to inhibit DMG growth.
and Drug Administration (FDA) for the treatment of primary immune Methods: Lipids were analysed by thin layer chromatography
thrombocytopenia (ITP) in pediatric and adult patients. Reports about followed by chemical staining or immune overlay as well as by
Romiplostim® in pediatric cancer patients are scarce. We aim to ana- liquid chromatography-coupled tandem mass spectrometry from
lyze the effectiveness and safety of Romiplostim® in patients with DMG primary cells, from a DMG cell line and from post-mortem
persistent TRT and high-risk neuroblastoma (HR-NB). pontine tissue of 3 patients without evidence of brain cancer.
Methods: We conducted a single-institution retrospective analysis of The DMG cell line was treated with a lipid extract and pro-
the whole cohort of HR-NB and TRT patients treated with Romi- liferation was measured in comparison to a neuroblastoma cell
plostim® between July 2017 and June 2021. Romiplostim® was line.
administered as the recommended dose in ITP 1-10mcg/kg/weekly Results: The neutral fraction of pons is rich in cholesterol, sphin-
subcutaneously. Platelet increase was measured at 3, 6 and 12 weeks. gomyelin and cerebroside (GalCer), while the same fraction of DMG
An increase >50,000/mm3 platelets was considered a response. contains less of these, but does express globoside (Gb4Cer). The acidic
Results: We included eighteen patients. Eleven (61.1%) had received fraction of normal pons is rich in sulfatides and complex gangliosides,
autologous stem cell transplant and three (16.6%) craniospinal radio- while the same fraction of DMG is devoid of sulfatide and contains
therapy. Four (22.2%) had morphological bone marrow infiltration less gangliosides, which are not as complex. Treatment with a com-
by NB cells before starting Romiplostim® and seven (38.8%) mini- plex gangliosides extract reduced proliferation of DMG but not of
mal residual disease (MRD) positive by PHOX2B. The whole cohort neurablostoma cells.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S321 of S635

Conclusions: GSL composition may control cell behaviour. Non- clinical data, the vinblastine starting dose was reduced to 75% of
proliferating adult brain cells contain complex gangliosides, while the the single-agent dose (dose level 1; 4.5 mg/m2 ). Two patients were
globo-series of glycolipids is necessary for the maintenance of an undif- included in stratum I and treated with crizotinib (fixed dose; 150
ferentiated stem cell phenotype during embryogenesis. The addition of mg/m2 BID) and weekly i.v. vinblastine.
complex gangliosides may counteract the effect of globoside in DMG. Results: Patient 1, diagnosed with early relapsed ALK-positive ALCL
Lipid replacement therapy has been used in the 80s to treat chronic with bone marrow involvement, was assigned to dose level 1. The
neuromuscular diseases, but was not effective. However, this therapy patient developed a dose limiting toxicity (DLT) on Cycle 1 Day 10
has been not evaluated in the context of brain cancer, and our results (C1D10); namely, grade 3 nausea unresponsive to anti-emetics, last-
warrant further analysis to translate these data into a new therapy for ing 7 days. On C1D10 the patient also developed neutropenic fever
DMG (grade 3) and died 17 days after starting treatment because of liver
failure. Autopsy revealed a fungal infection (Lichtheimia species) and
a suspected secondary hemophagocytic lymphohistiocytosis. Due to
EP372 / #454 UNEXPECTED SEVERE TOXICITY OF this toxicity, patient 2 (relapsed ALK-positive ALCL) was assigned to
CRIZOTINIB IN COMBINATION WITH VINBLASTINE: THE CRISP dose level -1 (3.0 mg/m2 ). This patient developed a DLT on C1D22;
OPEN-LABEL PHASE 1B TRIAL IN PEDIATRIC PATIENTS WITH neutropenia grade 4 (>7 days). Treatment was stopped and neutrope-
ANAPLASTIC LARGE CELL LYMPHOMA (ALCL) (ITCC-053) nia resolved. Vinblastine was administered to both patients despite
not fulfilling starting criteria (neutropenia grade 4). Both patients
Kim Schellekens1,2 , Reineke Schoot1 , Pauline Winkler-Seinstra1,2 , were taken off study before scheduled pharmacokinetic sampling. The
Alwin Huitema1,3,4 , Isabelle Aerts5 , Marion Gambart6 , Auke steering committee decided to terminate stratum 1, assuming further
Beishuizen1 , Natasha Van Eijkelenburg1 , Wilhelm Woessmann7 , de-escalation of vinblastine was not clinically meaningful.
Fabian Knörr7 , Lucas Moreno8 , Michela Casanova9 , Dirk Reinhardt10 , Conclusions: The unexpected severe toxicity described here is prob-
Lynley Marshall11,12 , Louis Chesler11,12 , Giuseppe Barone13 , Judith ably due to a stronger than anticipated CYP3A4 interaction, and
Landman Parker14 , C. Michel Zwaan1,2 , Jasper Van Der Lugt1 possible additional pharmacodynamic interactions. This study empha-
1 Princess Máxima Center for Pediatric Oncology, Department Of Pediatric sizes the importance of (pre-)clinical pharmacological evaluation in
Oncology, Utrecht, Netherlands; 2 Erasmus MC-Sophia Children’s Hospital, trials investigating drug combinations.
Department Of Pediatric Oncology, Rotterdam, Netherlands; 3 The Nether-
lands Cancer Institute-Antoni van Leeuwenhoek, Department Of Pharmacy
& Pharmacology, Amsterdam, Netherlands; 4 Utrecht University Medical EP373 / #517 IDENTIFICATION OF KDM3A AS A
Center, Department Of Clinical Pharmacy, Utrecht, Netherlands; 5 Institut PROMISING PROGNOSTIC MARKER AND THERAPEUTIC
Curie, Department Of Pediatric Oncology, Paris, France; 6 CHU Toulouse, CANDIDATE IN PEDIATRIC ADRENOCORTICAL TUMORS
Hôpital des Enfants, Pediatric Hemato-oncology Unit, Toulouse, France;
7 University Medical Center Hamburg-Eppendorf, Department Of Pediatric Luiz Santos1 , Luciana Veronez2 , Carolina Corrêa1 , Mirella Baroni1 ,
Hematology And Oncology, Hamburg, Germany; 8 Vall d’Hebron University Luiz Nagano1 , Alcides Xavier1 , Rosane Queiróz2 , Sonir Antonini2 ,
Hospital, Department Of Pediatric Oncology And Hematology, Barcelona, Silvia Brandalise3 , José Yunes3 , Luiz Tone2 , Carlos Scrideli2
Spain; 9 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Oncol- 1 Ribeirão Preto Medical School, University of São Paulo, Genetics, ribeirão
ogy Unit, Milan, Italy; 10 University Hospital of Essen, Department Of Preto SP, Brazil; 2 Ribeirão Preto Medical School, University of São Paulo,
Pediatric Oncology And Hematology, Pediatrics Iii, Essen, Germany; 11 The Pediatrics, Ribeirão Preto SP, Brazil; 3 Boldrini‘s Children Center, Pediatrics,
Royal Marsden NHS Foundation Trust, Children And Young People’s Unit, Campinas SP, Brazil
London, United Kingdom; 12 The Institute of Cancer Research, Paediatric
Tumour Biology, Division Of Clinical Studies, London, United Kingdom; Background and Aims: Adrenocortical tumors (ACT) are neoplasms
13 Great Ormond Street Hospital for Children, Department Of Paediatric that affect the adrenal glands. ACT are rare in children, but with an
Oncology, London, United Kingdom; 14 Hopital d’Enfants Armand Trousseau, incidence in Brazil around 10 times higher than worldwide. Treatment
Department Of Pediatrics, Paris, France options for patients with advanced ACT are limited and little effective.
In a previous study using RNASeq, our group identified the KDM3A
Background and Aims: Crizotinib and vinblastine monotherapies have gene as a possible central player in ACT pathology. KDM3A is a histone
shown promising results in ALK-positive ALCL. The combination was demethylase enzyme that acts on the modeling of histones and several
expected to result in a deeper remission status. Here we describe the studies have associated its hyperexpression with the occurrence and
unexpected severe toxicity in two patients treated with crizotinib and worse prognosis in several other tumors. Thus, this study aimed to eval-
vinblastine in an international prospective study. uate the clinical value of the expression of KDM3A and the effect of its
Methods: The CRISP trial (EudraCT: 2015-005437-53), is an ITCC inhibition in vitro.
international multicentric open-label, non-randomized, two-stage Methods: The expression of KDM3A was evaluated and correlated
phase 1b dose finding study, with dose escalation according to the with ACT clinical features in two different datasets of pediatric ACT
escalation with overdose control (EWOC) method. Based on pre- cases (GSE76021, n=34, and GSE76019, n=29) using log-rank and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S322 of S635 ABSTRACTS

Mann-Whitney tests. The effects of KDM3A inhibition in the viabil- independent experiments of combined therapy showed that the addic-
ity, migration, and invasion of NCI-H295R cells were evaluated after tion of the eEF2K inhibitor NH125 decreased cell viability to a greater
treatment with 500uM of the KDM3A inhibitor JIB-04 for 48h. extent than Cisplatin alone, lowering up to four times the necessary
Results: We identified that a higher expression of KDM3A was asso- dose to ensure cell death (p < 0,005).
ciated with lower 5-year overall survival in the GSE76021 dataset Conclusions: Our results reveal that eEF2K is associated with poor
(p=0.027), and event-free survival in both cohorts GSE76019 and prognosis in high-risk MB. Its inhibition decreased cell viability and
GSE76021 (p=0.031 and p=0.045 respectively). In both datasets, the enhanced the Cisplatin effect in vitro, suggesting this gene as a
overexpression of KDM3A was also associated with death (p=0.009 promising therapeutic target in patients of Grp3-MB (Grant FAPESP:
and 0.003, respectively) and relapse (p=0.009 and 0.005, respectively). 2014/20341-0).
In NCI-H295R cells, KDM3A inhibition was confirmed by both mRNA
and protein levels and resulted in significant decrease in cell viability,
invasion, migration, and colony formation compared to control cells EP375 / #1282 PERSONALISED MEDICINE - TARGETED
(p<0.05). THERAPY USAGE IN PAEDIATRIC ONCOLOGY –WHEN, HOW
Conclusions: Our results suggest that KDM3A could be a promising AND AT WHAT COST IN A MEDIUM SIZED PAEDIATRIC AND
treatment candidate for the pediatric ACT, since its overexpression ADOLESCENT ONCOLOGY UNIT
was associated with poor ACT prognosis, and its pharmacological inhi-
bition limited ACT progression in vitro. (Grant FAPESP: 2014/20341-0) Leanne Super, Nataliya Zhukova, Peter Downie, Paul Wood, Michelle
Martin, Tanya Selth, Lisa Janson
Monash Children’s Hospital, Children’s Cancer Centre, Melbourne, Australia
EP374 / #518 COMBINATION OF EEF2K INHIBITION AND
CISPLATIN AS A PROMISING COMBINED THERAPEUTIC Background and Aims: Targeted therapy usage is increasing, more so
APPROACH FOR HIGH-RISK MEDULLOBLASTOMA PATIENTS in adults, than in paediatric and adolescence oncology. With limited
open studies, these medications can only be accessed individually, at
Jéssica De Santis1 , Manuela França1 , Luiz Nagano2 , Rosane Queiróz3 , significant expense in cost and resources. The aim of this paper is to
Luiz Tone3 , Luciana Veronez3 , Carlos Scrideli3 review aspects of the usage in a medium-sized paediatric cancer cen-
1 Ribeirão Preto Medical School, University of São Paulo, Genetics, Ribeirão tre based in Melbourne, Australia that annually treats over 75 new
Preto SP, Brazil; 2 Ribeirão Preto Medical School, University of São Paulo, oncology patients from 0-19 years.
Genetics, ribeirão Preto SP, Brazil; 3 Ribeirão Preto Medical School, Univer- Methods: Data acquired was patient numbers, reason for targeted
sity of São Paulo, Pediatrics, Ribeirão Preto SP, Brazil therapy, access to clinical trials, how the medications were obtained
and cost, and any issues, side effects or benefits.
Background and Aims: Medulloblastoma (MB) is the most common Results: Data was difficult to obtain, with no tracked record of patients
pediatric cancer of the central nervous system and it has been classified on targeted therapy. Some patients were identified from the nation-
in 4 main molecular subgroups: SHH, WNT, Group 3, and Group 4. The wide Precision Medicine Program (PRISM). Over twenty patients were
group 3 MB (Grp3-MB) presents the lower survival rates among them. identified from October 2018 to December 2021. Of twenty-five
The high-risk tumors frequently regulate protein translation mediated patients enrolled on PRISM, twenty had recommendations of a sin-
by eEF2K, an essential gene activated to hold energy intake and survive gle target, and three had multiple targets. There were over eighteen
under unfavorable conditions.Thus, this study aimed to evaluate the targets identified. Fifteen different medications were recommended.
association of eEF2K expression with Grp3-MB, prognosis features, Fifteen of the twenty-three patients had accessible medications, and
and therapeutic potential. eleven commenced on the recommended agent. Eight of these patients
Methods: Associations between clinical features and eEF2K expres- remain on the medication with stable disease or better. Other patients
sion were evaluated by using public datasets of pediatric MBs on targeted therapies are on agents with more established indica-
(GSE85217, n = 763). Genes coregulated with eEF2K were identified tions, which are Government funded, and others are on newer agents
and considered significant when p < 0,001. We also tested an eEF2K suggested by pathology, biology and/or clinical course. Most patients
inhibitor (NH125) in combination with Cisplatin in two Grp3-MB cell were discussed at a multidisciplinary meeting. Most needed individ-
lines (USP-13-Med and D283). ual access to the medication, through hospital drug usage committee
Results: We identified that eEF2K is upregulated in Gr3-MB compared or special and compassionate access from drug companies. Parents did
to other molecular subgroups and its overexpression was associated not pay for the medications.
with lower overall survival (P < 0,001) and metastatic disease (p < Conclusions: Targeted therapies are being increasingly used in pae-
0,001). Among genes positively correlated with eEF2K in MB, we found diatric and adolescent oncology, with potentially improved outcomes,
USP2 (r=0.8) and OTX2 (r=0.72), both associated with a unfavor- but at significant resource and financial costs. Recommendations
able prognosis in Grp3-MB; DOCK9 (r=0.581) and EPHA8 (r=0.55), include improved records of patients on newer/ novel agents. Better
involved in invasion in MB; and PCBP4 (r=0.592), reported as a systems will track usage, cost, access and outcomes and allow more
cisplatin-resistance gene in other tumors. Results obtained from two cost/benefit analysis.
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ABSTRACTS S323 of S635

EP376 / #1231 VINCRISTINE-INDUCED PERIPHERAL Conclusions: The development of VIPN over 1.5-2 years among chil-
NEUROPATHY IN PEDIATRIC ONCOLOGY: LONG-TERM dren who receive vincristine was not associated with administration
FOLLOW-UP DATA OF A RANDOMIZED CONTROLLED TRIAL duration. However, in participants receiving concurrent azole treat-
COMPARING PUSH INJECTIONS WITH ONE-HOUR INFUSIONS ment and in those receiving higher cumulative vincristine dosages,
(THE VINCA TRIAL) one-hour administration resulted in less VIPN compared with push
administration. Therefore, one-hour administration of vincristine may
Aniek Uittenboogaard1 , Marleen Van Den Berg1 , Floor Abbink1 , Jos benefit at least some of the children.
Twisk2 , Inge Van Der Sluis3 , Cor Van Den Bos3 , Marry Van Den
Heuvel-Eibrink4 , Heidi Segers5 , Christophe Chantrain6 , Jutte Van Der
Werff Ten Bosch7 , Leen Willems8 , Gertjan Kaspers9 , Mirjam Van De EP377 / #957 APPLICATION OF THE COMIK PRECISION
Velde1 MEDICINE PROGRAMME FOR CHILDREN AND ADOLESCENTS
1 Amsterdam UMC - Location VUmc, Pediatric Oncology/-hematology, Ams- WITH ULTRA-RARE TUMOURS
terdam, Netherlands; 2 Amsterdam UMC, Department Of Epidemiology And
Biostatistics, Amsterdam, Netherlands; 3 Prinses Maxima Center for Pedi- Lorena Valero Arrese1,2 , Raquel Hladun Alvaro1,2 , Gabriela Guillén
atric Oncology, Oncology, Utrecht, Netherlands; 4 Princess Máxima Center Burrieza2,3 , Sergio López Fernández3 , José Andrés Molino Gahete3 ,
for Pediatric Oncology, Division Of Pediatric Oncology, Utrecht, Nether- Elena Martinez Saez4 , Jessica Camacho Soriano4 , Alexandra Navarro
lands; 5 University Hospitals Leuven and Catholic University Leuven, Depart- Jimenez4 , Marta Garrido Pontnou4 , María Antonia Poca Pastor5 ,
ment Of Pediatric Hemato-oncology, Leuven, Belgium; 6 Clinique CHC du Constantino Sabado Alvarez1,2 , Luis Gros Subías1,2 , Anna Llort
MontLégia, Department Of Pediatrics, Liège, Belgium; 7 Universitair Zieken- Sales1,2 , Josep Roma Castanyer2 , Miguel Segura Ginard2 , Ainara
huis Brussel, Department Of Pediatric Onco-hematology, Brussels, Belgium; Magdaleno Cazon2 , Andrea Vilaplana Blanes2 , Soledad Gallego
8 Ghent University Hospital, Department Of Paediatric Haematology- Melcón2 , Lucas Moreno Martin Retortillo1,2 , Aroa Soriano Fernández2
oncology And Stem Cell Transplantation, Ghent, Belgium; 9 Princess Máxima 1 Vall d’Hebron University Hospital, Paediatric Oncology And Haematol-
Center, Pediatric Oncology, Utrecht, Netherlands ogy, Barcelona, Spain; 2 Vall Hebron Research Institute, Childhood Cancer
And Blood Disorders, Barcelona, Spain; 3 Vall d’Hebron University Hospital,
Background and Aims: Vincristine is one of the mainstays of treat- Surgical Oncology And Neonatal Surgery, Paediatric Surgery Department,
ment in children with cancer. Its main side-effect is vincristine-induced Barcelona, Spain; 4 Vall d’Hebron University Hospital, Pathological Anatomy
peripheral neuropathy (VIPN), a sensory-motoric neuropathy. Thera- Department, Barcelona, Spain; 5 Vall d’Hebron University Hospital, Neuro-
peutic strategies are limited to pain management and dosage adap- surgery Department, Pediatric Neurosurgery Unit, Barcelona, Spain
tations. The VINCA trial is a randomized controlled trial in which
participants were randomized to receive vincristine via push injec- Background and Aims: Massive sequencing techniques have made
tions or one-hour infusions. We report on the long-term effect of possible to identify new therapeutic targets for patients with cancer.
administration duration on VIPN. However, paediatric patients’ access to new therapies in trials remains
Methods: VIPN was measured 1-7 times in 1.5-2.0 years with limited, even more for uncommon (ultra-rare) cancers. We have anal-
the Common Terminology for Criteria for Adverse Events ysed the somatic alterations identified in the COMIK (Cancer OMIcs
(CTCAE) and the pediatric-modified Total Neuropathy Score for Kids) precision medicine programme and the potential access to
(ped-mTNS). Poisson mixed model analysis and logistic general- targeted therapies.
ized estimating equation analysis for repeated measures were Methods: Paediatric patients with ultra-rare high-risk solid tumours at
performed. diagnosis, relapse or progression (excluding neuroblastoma, Ewing sar-
Results: Ninety patients were included (n=45 randomized to push- and coma, osteosarcoma, rhabdomyosarcoma, glioma, medulloblastoma),
n=45 to one-hour administration group), of which 58 were diagnosed who underwent whole exome sequencing of DNA from tumour and
with acute lymphoblastic leukemia. Overall, the number of participants germline for the detection of copy number variants, point muta-
developing VIPN did not significantly differ between the random- tions, small insertions and deletions, tumour mutational burden and
ization groups. In participants receiving concurrent azole treatment mutational signatures.
(n=7 per randomization group), the total CTCAE score was signifi- Results: Thirty-two patients were included from December 2015 to
cantly lower and the risk of severe VIPN was borderline significantly May 2021. Median age was 9 years (range 3 months - 17 years).
lower in the one-hour group compared with the push group (rate ratio Genomic somatic alterations were identified among 62% (20/32) of
0.52, 95% confidence interval (CI) 0.33-0.80, p=0.003, and odds ratio patients, with two or more alterations in nine of them. Thirty-four
0.26, 95% CI 0.06-1.20, p=0.09, respectively). These results were sim- alterations were described: 32% in epigenetic genes, 15% in receptor
ilar in a multivariable analysis. Moreover, if the cumulative vincristine tyrosine kinases and 12% in genes involved in transcription regula-
dosage increased, the difference in the total ped-mTNS score between tion. The median rate of mutations was low, 0.97 mutations/Mb (range
the randomization groups increased in favor of the one-hour group 0.31–4). Fifty-six per cent of the alterations are directly or indirectly
(p=0.006). Of note, findings were not consistent across different VIPN druggable with targeted therapy. This means we can propose therapy
measurement tools. based on molecular findings in 50% of patients. In none of these are
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S324 of S635 ABSTRACTS

approved therapies in paediatrics, and although in almost all cases the tion of apatinib did not seem to significantly increase hematological
drug is under development in paediatric clinical trials, in only 20% there toxicities.
is a trial available in our country. Conclusions: AIT regimen is a potentially effective regimen with man-
Conclusions: COMIK precision medicine programme identified action- ageable AE profile, and is worthwhile for further study in children with
able genomic somatic alterations leading to a targeted therapy recom- relapsed/refractory sarcoma.
mendation in, at least, half of the patients with an ultra-rare tumour.
However, patients did not finally receive a targeted therapy, due to
lack of available clinical trials or drugs ready to use, demonstrating the EP379 / #559 EFFICACY AND SAFETY OF LAROTRECTINIB
limited access to new drugs for this group of patients. IN PAEDIATRIC PATIENTS WITH TROPOMYOSIN RECEPTOR
KINASE (TRK) FUSION CANCER: AN EXTENDED FOLLOW-UP

EP378 / #119 APATINIB, IRINOTECAN AND TEMOZOLOMIDE C. Michel Zwaan1,2 , Cornelis Van Tilburg3 , Francois Doz4,5 , Catherine
(AIT) REGIMEN IN RELAPSED AND REFRACTORY PAEDIATRIC Albert6 , Claudia Blattmann7 , Birgit Geoerger8 , Steven Dubois9 , Noah
SOLID TUMOR Federman10 , Leo Mascarenhas11 , Rama Nagasubramanian12 , Alberto
Pappo13 , Tanya Watt14 , Ricarda Norenberg15 , Laura Dima16 , Esther
Carol Lai Sim Yan1 , Dennis T. L. Ku1 , Chi Kong Li1,2 , Eric Chun Ho Fu1 , De La Cuesta17 , Theodore Laetsch18 , Ruihua Xu19
Jeffrey Ping Wa Yau1 , Matthew Ming Kong Shing1 , Chung Wing Luk1 , 1 Princess Maxima Center for Pediatric Oncology, Stem Cell Transplantation

Godfrey Chi-Fung Chan1,2 Unit, Utrecht, Netherlands; 2 Erasmus MC-Sophia’s Children’s Hospital, Pedi-
1 Hong Kong Children’s Hospital, Department Of Paediatrics And Adolescent atric Oncology, Rotterdam, Netherlands; 3 Heidelberg University Hospital
Medicine, Hong Kong, Hong Kong PRC; 2 The University of Hong Kong, Lks and German Cancer Research Center (DKFZ), Hopp Children’s Cancer Cen-
Faculty Of Medicine, Hong Kong, Hong Kong PRC ter Heidelberg (kitz), Heidelberg, Germany; 4 SIREDO Oncology Center (Care,
Innovation and Research for Children and AYA with Cancer), Institut Curie,
Background and Aims: While irinotecan and temozolomide remains Paris, France; 5 Université de Paris Cité, Pediatrics, Paris, France; 6 Seattle
an essential backbone for treatment of paediatric relapsed/refractory Children’s Hospital and University of Washington School of Medicine,
solid tumors, the addition of apatinib (tyrosine kinase inhibitor, tar- Department Of Hematology-oncology, Seattle, United States of America;
geting VEGFR-2) is a potentially beneficial approach. Its efficacy and 7 Olgahospital, Department Of Haematology And Oncology, Stuttgart, Ger-
safety were retrospectively evaluated. many; 8 Gustave Roussy Cancer Center, Université Paris-Saclay, Department
Methods: This territory-wide study included patients age <19 with Of Pediatric And Adolescent Oncology, Villejuif, France; 9 Dana-Farber Hos-
relapsed/refractory sarcoma, who received AIT regimen during the pital, Children’s Cancer And Blood Disorders Center, Boston, United States
3-year period (April 2019-March 2022) in Hong Kong. Starting dose of America; 10 David Geffen School of Medicine UCLA Jonsson Comprehen-
of apatinib was based on body surface area:125mg (<0.6m2 ), 250mg sive Cancer Center, Pediatric Hematology Oncology, Los Angeles, United
(≥0.6-<1m2 ), 375mg (≥1-<1.5m2 ), 500mg (≥1.5m2 ) PO. Irinotecan States of America; 11 Cancer and Blood Disease Institute, Children’s Hospi-
(50mg/m2 for 5 days IV) and temozolomide (100mg/m2 for 5 days PO) tal Los Angeles, University of Southern California Keck School of Medicine,
were given at a 3-weekly interval. Baseline characteristics, outcome Department Of Hematology-oncology, Los Angeles, United States of Amer-
and adverse events (AEs) were analyzed. ica; 12 Nemours Children’s Hospital, Department Of Hematology-oncology,
Results: Eleven patients received AIT regimen (M:F 7:4) with median Orlando, United States of America; 13 St. Jude Children’s Research Hospital,
age 8.5 years (0.4-16.1 years). They received a median of 6 cycles Department Of Oncology, Memphis, United States of America; 14 University
(2-26) of irinotecan and temozolomide and 6.7 months (0.8-22.5 of Texas Southwestern Medical Center, Department Of Pediatrics, Dal-
months) of apatinib. Disease control rate could reach 91% whereas las, United States of America; 15 Chrestos Concept GmbH & Co. KG, -,
overall response rate was 45%. Best overall response was observed as Essen, Germany; 16 Bayer HealthCare SAS, Medical Affairs, La Garenne-
follows: Ewing sarcoma – 2 partial response(PR), 2 stable disease(SD); Colombes, France; 17 Bayer HealthCare Pharmaceuticals, Inc., Oncology,
BCOR-ITD – 1 SD; rhabdomyosarcoma – 1 PR, 1 SD; clear cell sarcoma Whippany, United States of America; 18 The Children’s Hospital of Philadel-
– 1 SD; undifferentiated sarcoma – 1 complete response(CR), 1 PR, phia, University of Pennsylvania, Developmental Therapeutics Program,
1 progressive disease(PD). Median duration of response was at least Philadelphia, United States of America; 19 Sun Yat-sen University Cancer
4.1 months (0.8-27.3 months), while median overall survival and Center, Department Of Medical Oncology, Guangzhou, China
progression-free survival were 10.6 months (1-27.3 months) and 5.9
months (0.8-27.3 months) respectively. AEs mostly limited to CTCAE Background and Aims: Neurotrophic tyrosine receptor kinase (NTRK)
grade 1-2, including hair hypopigmentation(27%), palmar-plantar gene fusions are oncogenic drivers in various tumour types. Larotrec-
erythrodysthesia syndrome(18%), hypothyroidism(27%), adrenal tinib is a highly selective tropomyosin receptor kinase (TRK) inhibitor
insufficiency(9%), hypertension(9%), anorexia(18%), malaise(18%). approved for treating paediatric and adult patients with TRK fusion
Grade 3 AEs were observed in 4 patients (3 proteinuria, 1 gas- cancer. Larotrectinib demonstrated an objective response rate (ORR)
trointestinal bleeding with pneumatosis intestinalis), which usually of 88% across 78 pediatric pts with non-CNS cancers (van Tilburg et
responded to treatment interruption/dosage adjustments. The addi- al, SIOP 2021). To better determine the efficacy outcomes in a more
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S325 of S635

mature dataset with a longer follow-up, we report here on the first 70 Background and Aims: The accurate diagnosis of childhood cancers
paediatric patients enrolled as of December 2019 with a data cut-off of requires multiple costly techniques and is unavailable for many low-
July 2021. and middle-income countries (LMIC). We aimed to demonstrate the
Methods: Patients aged <18 years with TRK fusion cancer in feasibility of a single, widely available and low-cost molecular sequenc-
larotrectinib clinical trials were included. Responses were investigator- ing platform (Oxford Nanopore Technologies) to accurately diagnose
assessed (RECIST v1.1). pediatric extracranial solid tumors.
Results: Tumor types included infantile fibrosarcoma (57.1%), other Methods: We performed multiplex nanopore mRNA sequencing on
soft tissue sarcoma (35.7%), congenital mesoblastic nephroma (2.9%), MinION flow cells for 61 formalin-fixed and paraffin-embedded (FFPE)
thyroid cancer (2.9%) and melanoma (1.4%). With longer follow-up, diagnostic specimens from children and young adults with solid tumors
the ORR was 87% (95% CI 77–94): 31 (44%) complete response (23 Burkitt lymphoma, 13 diffuse large B-cell lymphoma, 8 Wilms
(CR; including two pending confirmation and nine pathological CR), tumor, 6 Ewing sarcoma, 5 neuroblastoma, 5 rhabdomyosarcoma) and
30 (43%) partial response (PR), seven (10%) stable disease, one (1%) 8 non-malignant lymph nodes. Most specimens were originally pro-
progressive disease and one (1%) not determined. Median time to cessed in LMIC hospitals for clinical purposes. To train a classifier
response was 1.8 months; one patient converted from PR to CR after from gene expression profiles, we used a set of pairwise and one-
≥2 years on treatment. Treatment duration ranged from 1.0 to 62.6+ vs-all partial least-squares regressions and a support vector machine
months. Medians for duration of response and progression-free sur- classifier.
vival (PFS) were 43.3 (95% CI 23.2–non estimable [NE]) and 45.1 Results: FFPE derived nanopore mRNA sequencing averaged 408,097
months (95% CI 22.1–NE); median follow-ups were 34.0 and 33.3 reads per sample, with a mean read length of 163 base pairs, median
months, respectively. Median overall survival (OS) was not reached; read length 137 base pairs, and an average of 53,868 confidently
median follow-up was 35.2 months. The 36-month OS rate was 92%. mapped reads per sample. Three of the 68 (4.4%) cases failed a qual-
Treatment-related adverse events were mostly Grade 1–2. ity control parameter of > 5,000 mapped reads. Of the cases that
Conclusions: With this extended follow-up, larotrectinib demon- passed quality control, 95.6% of specimens (62/65) were correctly clas-
strated durable responses and prolonged PFS (medians >3.5 years), sified into tumor type. The three incorrect cases each had a predicted
and a favourable long-term safety profile in paediatric patients with probability of less than 0.6. Within the rhabdomyosarcoma subgroup,
TRK fusion cancer. This demonstrates the importance of identifying samples were classified into FOXO1 fusion status indirectly using gene
NTRK gene fusions in paediatric solid tumours. expression profiling. Each rhabdomyosarcoma sample (n=5) was cor-
rectly classifed into FOXO1 status with a predicted probability of
greater than 0.7.
E-Poster Topic: AS05.o Tumor Biology, Immunology and Conclusions: We report that it is feasible to use FFPE specimens to
Immunotherapy diagnose childhood cancers using unbiased nanopore mRNA sequenc-
ing. We are now extending this approach to an expanded range of
E-POSTER VIEWING tumor types, defining optimal sequencing depth, testing additional
computational approaches, and field testing the assay within multiple
EP380 / #1881 CLASSIFICATION OF PEDIATRIC LMIC contexts.
EXTRACRANIAL SOLID TUMORS USING LOW-COST
NANOPORE RNA SEQUENCING OF FORMALIN-FIXED
PARAFFIN-EMBEDDED SPECIMENS EP381 / #311 DNA DAMAGE RESPONSE PATHWAY
ALTERATIONS AND DEFICIENCY IN SARCOMAS IN CHILDREN
Jeremy Wang1 , Priya Kumar2 , Teresa Santiago2 , Yuri Fedoriw3 , Sophie AND YOUNG ADULTS
Roush4 , Corbin Jones5 , Charles Mullighan2 , Nickhill Bhakta6 , Thomas
Alexander7 Kurt Statz-Geary1 , Steven Bialick2 , Cesar Serrano3 , Andrew Elliott4 ,
1 University of North Carolina, Department Of Genetics, Chapel Hill, United Phillip Walker4 , Jim Abraham4 , Gina D’Amato2 , Margaret Von
States of America; 2 St. Jude Children’s Research Hospital, Department Of Mehren5 , Jonathan Trent2 , Andrew Rosenberg6 , Elizabeth
Pathology, Memphis, United States of America; 3 University of North Car- Montgomery6 , Aditi Dhir7
olina Chapel Hill, Department Of Pathology And Laboratory Medicine, 1 University of Miami Miller school of Medicine, Medical School, Miami,
Chapel Hill, United States of America; 4 University of North Carolina, Depart- United States of America; 2 University of Miami Miller school of Medicine,
ment Of Pathology And Laboratory Medicine, Chapel Hill, United States of Medical Oncology, Miami, United States of America; 3 Vall d’Hebron Institute
America; 5 University of North Carolina, Department Of Biology, Chapel Hill, of Oncology (VHIO), Gu, Cns And Sarcoma Unit – Oncology Dept., Barcelona,
United States of America; 6 St. Jude Children’s Research Hospital, Depart- Spain; 4 Caris Life Sciences, Research, Irving, United States of America; 5 Fox
ment Of Global Pediatric Medicine, Memphis, United States of America; Chase cancer center/Temple Health, Hematology Oncology, Philadelphia,
7 University of North Carolina, Department Of Pediatrics, Chapel Hill, United United States of America; 6 University of Miami Miller school of Medicine,
States of America Pathology, Miami, United States of America; 7 University of Miami Miller
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S326 of S635 ABSTRACTS

school of Medicine, Pediatric Hematology Oncology, Miami, United States atric Hematology And Oncology, Hannover, Germany; 4 German Cancer
of America Research Center (DKFZ), Applied Bioinformatics, Heidelberg, Germany;
5 HRH Princess Chulabhorn College of Medical Science, Faculty Of Medicine

Background and Aims: Clinically significant alterations in DNA dam- And Public Health, Bangkok, Germany
age response (DDR) pathways contribute to genomic instability and
malignant progression in several carcinomas and solid tumors. While Background and Aims: Individuals with cancer predisposition syn-
ostensibly pathogenic variations have been identified in known and dromes (CPS) have a significant higher risk of developing cancer and
novel cancer genes, we evaluated comprehensive molecular profiles to treatment outcome often improves with early diagnosis. Thus, there
investigate the role of DDR pathway alterations in sarcomas arising in is an urgent need for early and precise tumor detection methods.
pediatric, adolescent, and young adult (AYA) patients. Current standard cancer surveillance measures often rely on radi-
Methods: Sarcoma samples from pediatric and AYA patients aged 0-39 ologic imaging. Complementing surveillance with Liquid Biopsy (LB)
years (N=777), representing 25 histologic subtypes, underwent NGS of blood samples to analyze cell-free DNA (cfDNA) may improve the
DNA (592-gene panel or whole exome, N=777) and RNA (whole tran- detection of tumors at early stages and thereby allow for early inter-
scriptome sequencing, N=475) at a CLIA-certified laboratory (Caris vention. Our study explored the potential of LB analysis as a sensitive
Life Sciences, Phoenix, AZ). Homologous recombination deficiency and minimal-invasive complementary cancer surveillance tool for CPS
(HRD) scores were calculated as a composite of loss of heterozygosity, patients.
telomeric allelic imbalance, and large-scale transitions, using a positive Methods: Our cohort comprised plasma samples of 172 CPS patients
threshold of 42 (N=261). (n=256 samples) and 10 healthy controls. After cfDNA isolation
Results: A pathogenic DDR pathway mutation was noted in 68 according to standardized protocols, we performed low-coverage
(8.8±1.9% [95%CI]) of the total samples. DDR pathway alteration whole-genome-sequencing (lcWGS). Bioinformatic analyses were
rates were highest in angiosarcomas (AS;N=6,30.0±17.6%) and alve- adapted to computationally enhance somatic copy number variations
olar soft part sarcomas (N=3,30.0±24.9%) with >10% mutation (CNVs).
rate in 5 other subtypes: spindle cell sarcoma, not otherwise spec- Results: In comparison to healthy controls, lcWGS data derived from
ified, epithelioid sarcoma (ES), solitary fibrous tumor (SFT) of CNS, CPS patients revealed elevated levels of somatic CNVs in 33 percent
osteosarcoma and leiomyosarcoma (LMS). ATRX was the most fre- of hitherto analyzed individuals (n=81). Ongoing clinical follow-up and
quently altered DDR gene (4.3±1.8% of all samples), with mutations re-evaluation of medical imaging recorded simultaneously to the blood
observed across 9 sarcoma histologic subtypes (range 2.4-15.8%). collection could confirm the LB result in exemplary cases.
ERCC2 was mutated in ES (N=1,6.3±11.9%), AS (N=1,5.0±9.6%), Conclusions: These findings indicate the feasibility of cfDNA analysis
and rhabdomyosarcoma (N=1,1.5±3.0%). Median HRD scores ranged in early detection of somatic CNVs in CPS affected individuals. Fur-
between 20-58.5 across all sarcoma subtypes. High rates of deficient ther validation of LB results by comparative analysis with radiological
HRD (HRDd ≥ 42) were observed in pleomorphic sarcoma(N=4,100%), data and long-term surveillance are ongoing in order to evaluate the
MPNST(N=10,66.7±23.9%), LMS(N=16,61.5±18.7%) and epithelioid sensitivity. Altogether our study aims to increase specificity of estab-
hemangioendothelioma(N=3,60.0±42.9%), while no HRDd tumors lished methods and to integrate additional sensitive techniques for
were observed in clear cell(N=0/3), Ewing sarcoma(N=0/4), and early cancer detection in patients at high cancer risk.
SFT(N=0/3).
Conclusions: DDR pathway alterations are present in numerous his-
tologic subtypes of sarcomas, particularly those that display nuclear EP383 / #1574 MICRORNA-196A SENSITIZES B CELL
pleomorphism and lack characteristic translocations/fusions. Further LYMPHOMA CELLS TO DAUNORUBICIN THROUGH FOX01
research will evaluate the clinical implications of these known and
novel mutations to guide risk stratification and potential therapeutic Kyung-Nam Koh1 , Nayoung Kim2 , Eun Seok Choi3 , Jiyoung Kim3 , Su
options. Hyun Yoon3 , Young Kwon Koh3 , Sung Han Kang3 , Hyery Kim3 , Ho
Joon Im3
1 Asan Medical Center, Pediatrics, Seoul, Korea, Republic of; 2 Asan Medi-
EP382 / #323 IMPLEMENTATION OF LIQUID BIOPSY cal Center, Department Of Convergence Medicine, Seoul, Korea, Republic of;
ANALYSIS IN THE SCREENING OF CANCER PREDISPOSITION 3 Asan Medical Center, University of Ulsan College of Medicine, Pediatrics,
PATIENTS Seoul, Korea, Republic of

Sema Hamurcu1 , Kendra K. Maass1,2 , Christina Dutzmann3 , Pitithat Background and Aims: MicroRNAs (miRNAs) are small regulatory
Purachanot4,5 , Tatjana Wedig1,2 , Nathalie Schwarz1 , Benedikt Brors4 , RNAs that repress gene expression by directly binding to the target
Stefan Pfister1,2 , Christian Kratz3 , Kristian Pajtler1,2 mRNA’s 3’ untranslated region. miRNAs are critical regulators in cel-
1 Hopp Children’s Cancer Center Heidelberg, Pediatric Neurooncology, Hei- lular processes, including stress responses. Thus we were interested in
delberg, Germany; 2 Heidelberg University Hospital, Pediatric Hematology the potential roles of miRNAs in the pathogenesis of B cell leukemia
And Oncology, Heidelberg, Germany; 3 Hannover Medical School, Pedi- and lymphoma. miR-196a was known to be reversed drug resistance
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S327 of S635

in non-small cell lung cancer and gastric cancer. Daunorubicin is one of Methods: We created an international registry of CAR-T recipients
the standard therapeutics for various leukemia and lymphoma. aged 0-30 years infected with SARS-CoV-2 within 2 months prior to or
Methods: MiR-196a was overexpressed in SU-DHL-6 cells, a human any time after CAR-T infusion. Nine centers contributed 78 infections
diffuse large B cell lymphoma cell line, by transfection of a lentiviral in 75 patients. Suspected US omicron infections were defined as those
vector. Cell viability was measured by CCK8 assay (Dojindo), and apop- after December 12, 2021.
tosis was assessed with Caspase3/7 assay (Promega). The cell cycle Results: Of 70 SARS-CoV-2 infections occurring 20 to 3180 days
was investigated with flow cytometry using propidium iodine after fixa- (median 435 days) post-CAR-T, 13 (18.6%) were classified as asymp-
tion. The target gene of miR-196a was predicted by TargetScan, and the tomatic, 37 (52.9%) mild, 11 (15.7%) moderate, 6 (8.6%) severe
expression of FOXO1 was assessed by Q-PCR and Western blotting. COVID-19 and three (4.3%) were classified as multisystem inflamma-
Results: We found that overexpression of miR-196a reduced the via- tory syndrome in children (MIS-C). Twenty (28.6%) infections resulted
bility of SU-DHL-6 cells upon daunorubicin treatment. The reduced cell in admission, with 8 (11.4%) ICU admissions. Median admission length
viability was a result of increased apoptosis. The expression of FOXO1 was 13 days (IQR 5-29.8). Prior to the emergence of the omicron vari-
was directly regulated by miR-196a, which was shown by Q-PCR and ant, 19/47 (40.4%) infections resulted in hospital admission and 7/47
Western blotting. (14.9%) required ICU admission, while after, only 1/23 (4.3%) infec-
Conclusions: We found that miR-196a could sensitize B cell lymphoma tions required admission. Death occurred in 3/70 (4.3%); each had
cells to daunorubicin through FOX01. We further plan to investigate ≥1 coinfection or life-threatening condition. Factors associated with
the underlying mechanism and clinical impact using clinical samples. severe COVID-19 or MIS-C included: >1 comorbidity (OR 27.0 [95%
CI 5.36-208], p<0.0001), coinfection (OR 17.5 [3.46-103], p<0.0001),
and absolute lymphocyte count (ALC) <500 cells/uL (OR 21.6 [3.37-
EP384 / #342 SARS-COV-2 INFECTIONS IN PEDIATRIC AND 428], p=0.006). No patients with severe COVID-19 or MIS-C had a
YOUNG ADULT (YA) RECIPIENTS OF CHIMERIC ANTIGEN suspected omicron infection. In the 8 patients infected with SARS-CoV-
RECEPTOR T-CELL THERAPY: AN INTERNATIONAL REGISTRY 2 pre-CAR-T, infections delayed CAR-T infusions in 4 patients by 15-30
REPORT days.
Conclusions: In a large international cohort of pediatric/YA CAR-T-
Kevin Mcnerney1,2 , Rebecca Richards3 , Paibel Aguayo-Hiraldo4 , Friso recipients, SARS-CoV-2 infections resulted in high rates of hospital
Calkoen5 , Julie Talano6 , Amy Moskop7 , Adriana Balduzzi8 , Jennifer and ICU admissions. Patients with comorbidities, coinfections, and low
Krajewski9 , Hema Dave10 , Colleen Callahan2 , Yimei Li11 , Kara ALC were more likely to experience severe illness. Suspected omicron
Davis12 , Shannon Maude2 infections were associated with milder disease.
1 Johns Hopkins All Children’s Hospital, Cancer And Blood Disorders Insti-
tute, St Petersburg, United States of America; 2 Children’s Hospital of
Philadelphia, Paediatrics, Philadelphia, United States of America; 3 Stanford EP385 / #1341 PRECISION MEDICINE REVEALS A
University, Pediatric Hematology & Oncology, Palo Alto, United States of HETEROGENOUS TUMOUR IMMUNE MICROENVIRONMENT IN
America; 4 Keck School of Medicine of USC, Cancer And Blood Disease Insti- PAEDIATRIC GLIOMAS
tute, Los Angeles, United States of America; 5 Princess Maxima Center for
Pediatric Oncology, Pediatric Oncology, Utrecht, Netherlands; 6 Medical Col- Chelsea Mayoh1 , Deborah Meyran2,3 , Thu Nguyen3 , Colleen Darcy4 ,
lege of Wisconsin, Pediatrics Hematology Oncology, Milwaukee,. WI USA, David Ziegler5,6 , Michelle Haber7 , Joe Trapani8 , Paul Ekert7 , Paul
United States of America; 7 Medical College of Wisconsin, Oncology, Mil- Neeson3
waukee, United States of America; 8 Fondazione Monza e Brianza per Il 1 Children’s Cancer Institute, Bioinformatics, Randwick, Australia; 2 Royal
Bambino e La Sua Mamma, Clinica Pediatrica, Università Degli Studi Di Children’s Hospital, Paediatric Oncology, Parkville, Australia; 3 PeterMac
Milano Bicocca, Monza, Italy; 9 Hackensack Meridian School of Medicine, Callum Cancer Centre, Human Immunology Translational Research Labora-
Pediatrics, Hackensack, United States of America; 10 Children’s National tory, Melbourne, Australia; 4 Royal Children’s Hospital, Pathology, Parkville,
Hospital, Oncology, DC, United States of America; 11 University of Penn Australia; 5 UNSW Sydney, School Of Clinical Medicine, Faculty Of Medicine
Medicine, Statistics, Philadelphia, United States of America; 12 Stanford & Health, Sydney, Australia; 6 Sydney Children’s Hospital, Kids Cancer Cen-
University, Hematology/oncology, Palo Alto, United States of America tre, Sydney, Australia; 7 Children Cancer Institute, Lowy Cancer Research
Centr, Randwick, Australia; 8 PeterMac Callum Cancer Centre, Cancer
Background and Aims: Immunocompromised patients have increased Immunology Program, Melbourne, Australia
risk from SARS-CoV-2 infections. Patients receiving CAR-T for
relapsed/refractory B-cell malignancies are uniquely immunosup- Background and Aims: Glioma is one of the most common subtypes
pressed due to B-cell aplasia. While SARS-CoV-2 mortality rates of brain tumours in children and includes low-grade glioma (LGG) and
of 33% have been reported in adults post-CAR-T, outcomes in high-grade glioma (HGG). Patients with HGG have a poor outcome and
pediatric/YA CAR-T recipients are poorly described. We describe desperately need new therapeutic options. Whilst immune checkpoint
outcomes of pediatric/YA CAR-T recipients with SARS-CoV-2 blockade (ICB) is one option, this has not been successful to date in
infections. patients with HGG. HGG tumour immune microenvironment (TIME)
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S328 of S635 ABSTRACTS

could be not permissive to response to ICB. Thus we aimed to investi- The medulloblastoma panels were carried out using the NanoString
gate paediatric glioma TIME by (1)multiplex IHC to reveal immune cell nCounter PanCancer Immune Profiling. The CodeSet had been pre-
density, their spatial relationships, and MHC-I expression(2) to investi- viously developed by NanoString Technologies comprising 730 genes
gate immune network signalling and immune suppression pathways by and 40 housekeeping genes. All procedures regarding sample prepa-
RNA-sequencing. ration, hybridization, detection, scanning and data analysis were
Methods: We have analysed 15 samples (9 HGG and 6 LGG) with performed according to manufacturer’s instructions.
a combination of techniques: digital spatial profiling on CD45+ , Results: Classic histology was found for 68.2% of the cases and
CD45+ CD3+ , and CD45- regions; Multiplex OPAL-IHC using a pan- 72.7% not presented metastasis at diagnosis. Additionally, 59.1%
immune and T-cell panel (CD68, TMEM119, CD11C, PD1, PDL1, of the patients underwent total surgical resection. The OS for
CD3, CD4, CD8, FOXP3, GFAP); MHC Class-I IHC; RNA-sequencing MBWNT samples was 76.15 months, followed by 44.84 months in
to deconvolute the immune cell mixtures, assess T-cell infiltrative the MBSHH samples and 43.2 and 36.4 months in the MBGPR3 and
lymphocyte signatures and identify immune suppressive pathways. MBGPR4 , respectively. Our results showed that MB subgroups have
Results: Spatial profiling revealed all LGG and a subset of HGG distinct immuno-oncology-profile. Related to this signatures immune
were immune-inflamed, either hot-clustered or hot-diffuse. There was populations, the MBSHH showed that several genes were differen-
a higher proportion of CD4+ PD1+ than CD8+ PD1+ T-cells. All T- tially expressed when compared with other molecular subgroups, and
cell subset densities were directly associated, as were T-cells and the modulations found in these genes suggested the involvement
macrophage densities. However, T-cell and microglia were inversely of myeloid cell populations in an inflammatory tumor microenvi-
correlated. Double-negative T-cells and T helper cells were sig- ronment. In the MBGPR4 , differentially expressed genes were impli-
nificantly spatially diverse, and we saw a significant increase in cated in the dysregulation of T cells, and were associated with poor
CD4- CD8- PD1+ T-cells and CD11c+ APC in LGG and HGG that were prognosis.
hot compared to immune-cold HGG. Conclusions: Our study illustrates the robustness of the nanoString for
Conclusions: The paediatric glioma TIME is heterogeneous and the immune profiling, which can contribute for further subgroup classifica-
TIME phenotypes do not separate according to low vs high-grade tion, and can add information in assessment of specific immunothera-
tumours. Importantly, in this study, we identified a subset of immune peutic targeting strategies to mediate antitumor immunity in patients
‘hot’ HGG that could benefit from ICB. This work stresses the need with medulloblastoma.
for a personalised immune-profiling approach and, with the integration
of RNA-sequencing, can identify biomarkers to classify patients into
immune-altered or -inflamed. EP387 / #938 EXPLORING THE ROLE OF INDIGENOUSLY
DEVELOPED NOVEL HUMANIZED CD19-DIRECTED CHIMERIC
ANTIGEN RECEPTOR(HCAR19) T-CELLS FOR
EP386 / #24 IMPROVING PEDIATRIC MEDULLOBLASTOMA RELAPSED/REFRACTORY B-CELL ACUTE LYMPHOBLASTIC
STRATIFICATION THROUGH THE LENS OF TUMOR LEUKEMIA- A PILOT OPEN-LABEL SINGLE-ARM PHASE-I STUDY
ASSOCIATED IMMUNE MICROENVIRONMENT
Gaurav Narula1 , Rahul Purwar2 , Hasmukh Jain3 , Chetan Dhamne1 ,
Terence Duarte1 , Daniel Moreno1 , Luciane Da Silva1 , Letícia Leal1 , Prashant Tembhare4 , Subramanian Pg4 , Nikhil Patkar4 , Minal
Iara Santana2 , Gustavo Teixeira2 , Carlos Almeida Junior3 , Bruna Poojary5 , Albeena Nisar1 , Deepali Pandit1 , Atharva Karulkar2 , Afrin
Mançano3 , Rui Reis1 Rafiq2 , Aalia Khan2 , Ankesh Jaiswal2 , Prashant Suvasia2 , Sweety
1 Barretos Cancer Hospital, Molecular Oncology Research Center, Barretos, Asija2 , Priyanshi Suvasia2 , Juber Pendhari2 , Shreshtha Shah2 ,
Brazil; 2 Barretos Cancer Hospital, Pathology Laboratory, Barretos, Brazil; Nirmalya Roy Moulik1 , Swaminathan K1 , Shilpushp Bhosle6,7 , Sumathi
3 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil Hiregoudar5 , Shashank Ojha5 , Lingaraj Nayak3 , Jayashree Thorat3 ,
Bhausaheb Bagal3 , Manju Sengar3 , Nishant Jindal3 , Akanksha
Background and Aims: The understanding of immune tumor microen- Chichra1 , Sumeet Mirgh3 , Anant Gokarn3 , Sachin Punatar3 , Sunil
vironment and its role in medulloblastoma progression and immune Rajadhyaksha5 , Navin Khattry3 , Sripad Banavali1
escape is still far from complete. The immune microenvironment of 1 Tata Memorial Centre, Homi Bhabha National Institute, Paediatric Oncol-

medulloblastoma subgroups has distinct immune profiles, and such dif- ogy, Mumbai, India; 2 Indian Institute of Technology-B, Biosciences & Bio-
ferentiation of immunological characteristics suggests that immune engineering, Mumbai, India; 3 Tata Memorial Center, Homi Bhabha National
profiling these subgroups can add information to improve diagnostic Institute, Medical Oncology, Mumbai, India; 4 Tata Memorial Center, Homi
and supports the development of subtype-specific immunotherapeutic Bhabha National Institute, Hemato-pathology, Navi Mumbai, India; 5 Tata
strategies. In this study we aimed to identify the immune expression Memorial Center, Homi Bhabha National Institute, Transfusion Medicine,
profile of molecularly characterized pediatric Brazilian medulloblas- Mumbai, India; 6 Tata Memorial Centre, HBNI, Critical Care And Anesthe-
tomas, and associated it with their clinical-pathologic features. siology, Mumbai, India; 7 Tata Memorial Center, Homi Bhabha National
Methods: A series of 22 previously molecularly characterized pediatric Institute, Critical Care And Anesthesiology, Mumbai, India
medulloblastoma from the Barretos Cancer Hospital were evaluated.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S329 of S635

Background and Aims: Emerging and approved Chimeric Anti- Lopes1 , Elen Oliveira1 , Lisandra Teixeira1 , Fabiana Mello1 , Patricia
gen Receptor(CAR) T-cell therapies for relapsed-refractory(r/r) B-cell Siqueira1 , Francisco Nicanor Macedo4 , Danielle Forny5 , Patricia
Acute Lymphoblastic Leukaemia (B-ALL) are prohibitive for develop- Moura6 , Marcelo Land7 , Carlos Pedreira8 , Jacques Van Dongen9 ,
ing countries due to high-costs and limited expertise. We designed and Alberto Orfao10 , Elaine Costa1
developed a novel Humanized CD19-directed CAR(HCAR19) meeting 1 Universidade Federal do Rio de Janeiro, Cytometry Lab, Rio de Janeiro,
regulatory requirements for a Phase-I trial- the first of its kind in India Brazil; 2 Federal University of rio de Janeiro, Pathology, Rio de Janeiro, Brazil;
and developing countries(CTRI/2021/05/033348). 3 National cancer Institute, Pathology, rio de janeiro, Brazil; 4 Hospital Estad-

Methods: R/r B-ALL patients aged 3-25 years ineligible for Allogenic- ual da Criança, I D’or Institute, Cirurgia Pediatrica, rio de janeiro, Brazil;
Stem Cell Transplant(AlloSCT) and no prior CD19-immunotherapy 5 Federal University of Rio de Janeiro, Pediatric Surgery, rio de janeiro, Brazil;

were included if CD19-expression exceeded 99% blast-population. 6 Hospital Estadual da Criança, I D’or Institute, Pediatric Oncology, rio de
Those with major comorbidities were excluded. Lymphocyte-collection janeiro, Brazil; 7 Universidade Federal do Rio de Janeiro, Pediatric Onco-
protocol on FreseniusKabi-Com.Tec™ was used for product- hematology, Rio de Janeiro, Brazil; 8 Federal University of rio de Janeiro,
manufacture. Bridging-chemotherapy was physician-determined. Systems And Computer Engineering Department, rio de janeiro, Brazil;
HCAR19 cells would be infused to 6 patients at two dose-levels: Dose- 9 Department of Immunohematology and Blood transfusion (IHB), Leiden
1:1x106 /kg; Dose-2:3-5x106 /kg body-weight after Lymphodepletion University Medical Center, Leiden, Netherlands; 10 Translational and Clin-
(Fludarabine-Cyclophosphamide), and observed for toxicity, HCAR19 ical Research Program, Centro de Investigación del Cáncer and IBMCC
in-vivo dynamics, and effects on tumor burden. Disease-response was (CSIC-University of Salamanca), Cytometry Service, Salamanca, Spain
assessed on Day-30 bone-marrow.
Results: To-date 6 patients were enrolled, 3 received Dose-1, 1 Background and Aims: Pediatric cancer is the leading cause of death
Dose-2, 2 withdrawn pre-infusion (rapid-progression:1;consent- in children, being fundamental the early diagnosis for a better out-
withdrawl:1). Three patients at Dose-1 were assessable for come. Neoplastic cells characterization together with the recognition
end-points. Median age was 16-years(range:11-18), M:F-1:2, median of the tumor-immune system interaction can guide a risk-adapted
previous therapy-lines 3(2-3), 2 with high-risk mutations(IKZF- treatment. The multiparameter flow cytometry (MFC) application
del/PAX-5-JAK2 fusion-One; T315-mutated BCR-ABL-One). Median to non-hematopoietic solid tumors (NHST) remains limited. Here
pre-infusion bone-marrow blasts were 28.2%(0.09-37.1%). Product- we designed and prospectively validated a new single 8-color anti-
manufacture was sucessful in all. Toxicities included Grade-2 body combination- solid tumor orientation tube, STOT- for diagnostic
febrile-neutropenia(66%), thrombocytopenia(66%), leukopenia(66%), screening, classification and tumor-infiltrating immune cells of pedi-
which recovered. Product-related toxicities were Grade-2 Cytokine atric cancer by MFC.
release syndrome(CRS)-1(33%) Tocilizumab-responsive, and B-cell Methods: A total of 476 samples from 296 patients with diagnostic
aplasia-1(33%) managed with Intravenous-Immunoglobulin. No suspicion of pediatric cancer were analyzed by MFC vs., conven-
other toxicities were noted. CRS correlated with peak IL-6 levels tional diagnostic procedures. STOT was designed after several design-
on Day-8/11, range:4.8-342 pg/ml. HCAR19 appeared in circula- test-evaluate-redesign cycles based on a large panel of monoclonal
tion by Day-7(median-3.6%;range-2.7-6.3%/circulating T-cells) and antibody combinations tested in 301 samples.
peaked Day-11-14(15.7%-88%/circulating T-cells). One patient had Results: Based on the immunophenotypic pattern of those 301
no expansion in week-2. Of 3 evaluable patients on Day-30, overall samples, final version of STOT consisted of a single 8-color-12
response-rate(ORR) was 67%. At last follow-up 2 patients were alive marker antibody combination (CD99-CD8/nu myogenin/CD4-
(one post-AlloSCT, one CD19-negative relapse), while one patient with EpCAM/CD56/GD2/sm CD3-CD19/cy CD3-CD271/CD45).
no-response at Day-30 expired subsequently. Prospective validation of STOT in 149 samples showed concor-
Conclusions: Autologous-HCAR19 was safe at starting-level Dose- dant results with the patient WHO/ICCC-3 diagnosis in 138/149
1:1x106 /kg with low-toxicities, robust in-vivo dynamics, correlating cases (92.6%). These included: 63/63 (100%) reactive/disease-free
well with pre-clinical studies, and sufficient responses to proceed with samples, 43/44 (98%) malignant and 4/4 (100%) benign NHST and
dose-escalation, establishing the feasibility and pathway for CAR T-cell 28/38 (74%) leukemia/lymphoma cases, being Hodgkin lymphomas not
therapy in India and developing countries. diagnosed by STOT tube. STOT allowed accurate discrimination among
the four most common subtypes of CD45- CD56++ NHST: 13/13
(GD2++ CD271-/+ nu MyoD1- nu myogenin- CD99- EpCAM- ) neuroblast
EP388 / #1674 FLOW CYTOMETRY FOR DIAGNOSIS, oma samples, 2/2 (GD2-/+ CD271+ nu MyoD1- nu myogenin- CD99+ EpC
CLASSIFICATION AND MONITORING OF PEDIATRIC SOLID AM- ) Ewing sarcoma family of tumors, 5/5
TUMOR BASED ON THE EUROFLOW SOLID TUMOR (GD2- CD271++ nu MyoD1++ nu myogenin++ CD99-/+ EpCAM- ) rhabdo
ORIENTATION TUBE - STOT myosarcomas and 7/7 (GD2- CD271-/+ nu MyoD1- nu myogenin- CD99-
EpCAM+ ) Wilms tumors. Among 63 tissue samples, the immune cell
Cristiane De Ferreira-Facio1 , Vitor Botafogo1 , Enrico infiltrate was composed mostly by T-lymphocytes in neuroblastic
Bruno-Riscarolli1 , Amanda Oliveira-Santos1 , Cristiane Milito2 , tumors and hematopoietic tumors (p=0.003) while granulocytes
Patricia Ferrão1 , Maria Clara Canellas1 , Sérgio Romano3 , Daiana predominate in germ cell and soft tissue tumors (p<0.001).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S330 of S635 ABSTRACTS

Conclusions: In summary, here we designed and validated a new EP390 / #475 FINE-TUNING OF CHIMERIC ANTIGEN
standardized antibody combination and MFC assay for diagnostic RECEPTORS VIA BARCODED POOLED SCREENING IMPROVES
screening of pediatric solid tumors that might contribute to fast and EFFECTOR CELL PERSISTENCE AND ANTI-NEUROBLASTOMA
accurate diagnostic orientation and classification of pediatric can- ACTIVITY
cer together with a tumor-immune cell profile description in routine
clinical practice. Xavier Rios, Yibin Chen, Marc Morales, Osmay Pardias San Roman,
Chunchao Zhang, Pavel Sumazin, Leonid Metelitsa
Baylor College of Medicine, Pediatric Hematology And Oncology, Houston,
EP389 / #1363 DEPLETING TUMOR-INFILTRATING MYELOID United States of America
CELLS TO ENHANCE BISPECIFIC ANTIBODY-DRIVEN T CELL
INFILTRATION AND ANTI-TUMOR IMMUNE RESPONSE Background and Aims: Chimeric antigen receptor (CAR) T cell-based
immunotherapy is an effective treatment for hematologic malignan-
Jeong A Park1,2 , Linlin Wang2 , Nai-Kong Cheung3 cies but has shown only modest efficacy for solid tumors such as
1 Inha University Hospital, Pediatrics, Incheon, Korea, Republic of; 2 MSKCC, neuroblastoma. Our goal is to improve the efficacy of CAR-based can-
Pediatrics, New York, United States of America; 3 Memorial Sloan Kettering cer immunotherapies by developing novel barcoded pooled screening
Cancer Center, Pediatrics, New York, United States of America methodology to generate CARs with improved function.
Methods: We developed a combinatorial barcoded cloning strategy in
Background and Aims: T cell immunotherapy has emerged as one of which each CAR has a unique DNA barcode sequence corresponding to
the most promising therapeutic modalities for refractory or relapsed its serially assembled domains. This barcode allows simultaneous quan-
cancers. However, the efficacy has been limited by the immuno- tification of CAR frequencies in pooled populations via next-generation
suppressive tumor microenvironment (TME). TME impedes bispecific sequencing (NGS). Using this method, we simultaneously generated
antibody (BsAb) or chimeric antigen receptor (CAR)-driven T cell infil- 180 CARs based on the 14G2a single-chain variable fragment that
tration, survival, and cytotoxic efficacy. We explored the effects of targets neuroblastoma antigen GD2. We transduced this library into
tumor-infiltrating myeloid cells (TIMs) depleting strategies on BsAb- T cells and performed serial tumor challenges with CHLA255 neu-
driven T cell infiltration, persistence, and in vivo anti-tumor activity. roblastoma cells, assessing proliferation and relative expansion by
Methods: Anti-GD2 BsAb and anti-HER2 BsAb built on IgG-[L]-scFv quantifying changes in CAR barcodes via NGS.
platform were tested against human cancer xenografts using BALB- Results: The top two performing CARs from the screen both con-
Rag2−/−IL-2R-γc-KO (BRG) mice as a stand-alone or as EATs (T tained the 4-1BB costimulatory domain. Further characterization
cells ex vivo armed with BsAb). Depleting antibodies specific for revealed these constructs had lower cell surface expression, which cor-
polymorphonuclear myeloid-derived suppressor cells (PMN-MDSCs), related with decreased TRAIL expression (p-value <0.0001). Tested
monocytic MDSCs (M-MDSCs), and tumor-associated macrophages individually, these CARs mediated improved cell expansion and anti-
(TAMs) were used to study the role of each TIM component. Dexam- neuroblastoma activity compared to a GD2-CAR construct with the
ethasone, an established anti-inflammatory agent, was tested for its CD28 costimulatory domain that is currently being evaluated in phase
effect on TIMs and BsAb-based T cell immunotherapy. The effects of I studies.
TIM depletion were examined by flow cytometry and IHC of tumors, Conclusions: These findings show that our barcoded pooled screen
bioluminescence imaging, and in vivo tumor growth analyses. can generate CARs that mediate more potent anti-neuroblastoma
Results: BsAb-driven T cells recruited myeloid cells into human tumor activity. This improved activity could be due to optimized sur-
xenografts. Each TIM targeting therapy depleted cells of interest face expression of the CAR, leading to decreased apoptosis that
in blood and in tumors. Depletion of PMN-MDSCs, M-MDSCs, and is characteristic of cells that retrovirally-transduced to express 4-
particularly TAMs was associated with increased BsAb-driven T cell 1BB-based CARs. This screening method is readily scalable to test
infiltration into tumors, significantly improving tumor control and thousands of new CAR designs, providing unparalleled insights into
survival in multiple cancer xenograft models. Dexamethasone premed- how different CAR constructs lead to improved phenotypes. This
ication depleted monocytes in circulation and TAMs in tumors, also approach has the potential to revolutionize CAR development and to
enhancing BsAb-driven T cell infiltration and persistence, contributing become a new paradigm for the development of CAR-based cancer
to improved anti-tumor response with survival benefits. immunotherapy.
Conclusions: Depleting TIMs significantly improved the anti-tumor
efficacy of BsAb-based T cell immunotherapy by increasing intratu-
moral T cell infiltration and their persistence. TAM depletion was EP391 / #1075 INTERLEUKIN-15 CO-EXPRESSION INDUCES
more effective than PMN-MDSC or M-MDSC depletion at boosting ROBUST EXPANSION AND ANTITUMOR EFFECT OF GPC3-CAR
the anti-tumor response of BsAb. Dexamethasone premedication also T CELLS AND TOXICITIES CAN BE MITIGATED WITH THE
enhanced the anti-tumor response of EATs against varieties of cancer INDUCIBLE CASPASE-9 SAFETY SWITCH
xenografts in the presence of IL-2.
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ABSTRACTS S331 of S635

David Steffin1 , Nisha Ghatwai2 , Chunchao Zhang2 , Purva Rathi2 , Amy Background and Aims: Cancer predisposition syndromes (CPSs) are
Courtney2 , Bambi Grilley2 , Leonid Metelitsa3 , Pavel Sumazin3 , Helen reported in 8-10% of children with cancer. We sought to study genetic
Heslop1 , Malcolm Brenner1 , Andras Heczey4 predisposition to cancer in children treated at King Hussein Cancer
1 Baylor College of Medicine, Pediatric Hematology/oncology, Houston, Center.
United States of America; 2 Baylor college of Medicine, Pediatric Hema- Methods: This is a retrospective review of patients who were referred
tology/oncology, Houston, United States of America; 3 Baylor College of to our CPS clinic that was established in June2019. Referred patients
Medicine, Pediatric Hematology And Oncology, Houston, United States were screened using Jongmans’ and McGill Interactive Pediatric
of America; 4 Baylor College of Medicine, Hematology-oncology, Houston, OncoGenetic Guidelines (MIPOGG) for cancer predisposition. Next-
United States of America generation sequencing of germline DNA was performed using panels
of genes suited for patients’ diagnosis and family history.
Background and Aims: Glypican 3 (GPC3) is an attractive Results: Forty patients were screened; all were under age of 18-
immunotherapeutic target given its preferential expression in years and of Arab ancestry. All patients fulfilled Jongmans’ and/or
several solid cancers. We previously treated 12 patients with T cells MIPOGG criteria. Thirty patients (75%) had family history of can-
expressing a GPC3-CAR at two doses (DL1: 1 x 107 ; DL2: 3 x 107 /m2 ). cer. Twenty patients had neoplasm indicating CPS (e.g., adrenocorti-
No dose limiting toxicities occurred, but there was minimal CAR T cal carcinoma, . . . ). Congenital or other phenotypic anomalies were
cell expansion; antitumor responses were limited to stable disease detected in 7 patients. Five patients were referred due the diagno-
by RECIST criteria. Further pre-clinical studies demonstrated that sis of two malignancies. Excessive toxicity related to cancer treatment
GPC3-CAR T cells co-expressing IL-15 (15.GPC3-CAR) had superior in was reported in one patient. Primary cancers were hematological
vivo expansion and antitumor activity in mice with hepatocellular car- malignancy(n=21), brain tumor (n=11), solid tumor (n=8), and LCH
cinoma (HCC) xenografts compared to T cells expressing GPC3-CAR (n=1). Pathogenic/Likely pathogenic germline mutations were identi-
alone. Thus, we hypothesized that 15.GPC3-CAR would induce higher fied in 14(35 %) patients across ten known cancer predisposition genes:
CAR T cell expansion and boost antitumor activity in patients with NF1, TP53, MUTYH, SMARCB1, MSH6, CBL, FANCA, DCLRE1C, RB1,
GPC3+ solid tumors. DICER1. VUS variants were detected in 17(40%) patients in the fol-
Methods: The AGAR (NCT04377932) study is a phase 1 clinical trial lowing genes: GATA2, PTCH1, ATM, TP53, PMS2, MSH6, ALK, POT1,
to define the safety and antitumor activity of 15.GPC3-CAR in chil- BRCA1, BRIP1, POLE, RECQL4, CASR, DIS3L2, PALB2, TERT, MET,
dren with relapsed/refractory solid tumors. Toxicity is assessed using RET, BPTF, DUT. Ten patients (25%) had negative testing despite har-
CTCAE v5. Persistence is evaluated by RT-PCR and flow cytometry; boring clinical features of CPS. Genetic counseling encountered many
anti-tumor effect is measured by standard 3D imaging and serum AFP. challenges, mainly performing the testing for extended families and
Results: We infused 3 x 107 /m2 15.GPC3-CAR into 3 patients with suggesting the proper future surveillance for affected family members.
HCC. Two patients experienced modest T cell expansion and progres- Conclusions: By screening our patients carefully, we were able to iden-
sive disease without dose limiting toxicity. In one patient, however, tify a significant number of patients with CPS. Further refinement of
we observed robust expansion of lymphocytes that peaked at week our testing may require WES to detect structural variations and collab-
2, (9.7x109 CAR transgene copies /ml by QPCR and 2935.6 CAR+ T oration with research centers to perform functional testing for patients
cells/μL by flow cytometry). This patient had grade 4 cytokine release with VUS.
syndrome which rapidly resolved after the activation of the inducible
caspase 9 (iC9) safety switch with rimiducid. At week 4 post-infusion,
the primary liver mass and multifocal lung metastases showed marked EP393 / #1406 HARMONIZING GENETIC DATA ELEMENT
decrease in size corresponding with a 94% decrease in serum AFP. MODELING ACROSS CANCER TRIALS
Conclusions: 15.GPC3-CAR T cells can induce robust expansion and
antitumor activity and toxicities can be mediated with rimiducid. Michael Watkins1 , Brian Furner1 , Mei Li1 , Ellen Cohen1 , Bradley
Carlson1 , Sarah Leary2 , Simone Hettmer3 , Junne Kamihara4 , Kirk
Wyatt5 , Samuel Volchenboum1
EP392 / #1535 CLINICALLY RELEVANT GERMLINE VARIANTS 1 University of Chicago, Pediatrics, Chicago, United States of America;
DETECTED BY NEXT-GENERATION SEQUENCING IN PATIENTS 2 Seattle Children’s, Hematology Oncology, Seattle, United States of Amer-
WITH CHILDHOOD CANCER AT KING HUSSEIN CANCER ica; 3 Uniklinik Freiburg - Zentrum für Kinder- und Jugendmedizin, Pediatric
CENTER Oncology, Freiburg im Breisgau, Germany; 4 Dana-Farber Cancer Institute,
Pediatric Oncology, Boston, United States of America; 5 Sanford Health,
Mayada Abu Shanap1 , Iyad Sultan1 , Razan Abu Khashabeh2 , Pediatric Oncology, Fargo, United States of America
Abdelghani Tbakhi2
1 king Hussein cancer center, Pediatric Department, Amman, Jordan; 2 king Background and Aims: The Pediatric Cancer Data Commons (PCDC)
Hussein cancer center, Department Of Cell Therapy & Applied Genomics, has facilitated the creation and data modeling work of 9 pediatric
Amman, Jordan oncology consortia with members from 31 data-contributing sites
across 13 countries. This modeling involves pooling case report forms,
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S332 of S635 ABSTRACTS

extracting concepts, and harmonizing those concepts into common Background and Aims: Central venous access to provide chemother-
data dictionaries for each of our currently-supported 11 diseases. Since apy is the standard of care in children receiving cancer treatment.
the data come from trials that span the past several decades, there are Totally implantable venous-access ports (TIVAP) are the preferred
many differences in conceptual granularity, nomenclature, and concept method for most children and constitute a routine procedure in
organization. high-income countries. However, in resource-limited settings, timely
Methods: The PCDC has extracted genetic data elements from over TIVAP placement represents a complex and multifactorial challenge
125 international case report forms. Along with subject matter experts due to supply shortages, surgical time delays, and lack of standard-
from across the spectrum of pediatric oncology, we have gone through ized processes. We used quality improvement (QI) methodology from
several iterations of a model that would appropriately suit the needs of the Institute for Healthcare Improvement to improve timely TIVAP
each disease type. The format of this model consists of variables and placement at the General Hospital-Tijuana, Mexico.
sets of coded permissible values. Methods: Of the 60 children with cancer diagnosed annually, 29
Results: One of the main principles learned was the appropriate bal- patients were candidates for TIVAP and were included in our QI
ance of conceptual granularity. For example, meaningful harmonization initiative. Baseline data was collected from September 2020 to Jan-
requires accommodating a “common name” for mutations that may uary 2021. Our SMART goal was to decrease the TIVAP placement
include non-standardized descriptors (hotspot, splice site, etc.) and to <42 days from diagnosis. Plan-Do-Study- Act (PDSA) cycle 1 was
accommodating the oft-conflated concepts of “type” and “effect” of a completed from February 2021 to December 2021. TIVAP place-
mutation. The PCDC “Genetic Analysis” model currently consists of 30 ment waiting time (WT) was defined from Day of Diagnosis to Day
variables and 15 value sets. of Placement. The QI strategy included multimodal interventions that
Conclusions: The design of clinical trials is a critical recurring task for combined system change guided by fishbone and key driver diagrams.
some clinicians. While these investigators are experts in their oncolog- Interventions included the establishment of the TIVAP team, ensuring
ical domain, they understandably draw from nomenclature and jargon supplies and surgical time, development and application of guide-
common to that domain. This can make data harmonization across lines/flowhcharts, systemization of communication and processes, and
domains a daunting task–with genetic concepts being particularly dif- hospital leadership support for implementation.
ficult. This PCDC “Genetic Analysis” model is the result of extensive Results: Baseline overall WT mean was 57 days (12-106 days). Mean
harmonization efforts and represents international consensus from WT for children with leukemia and solid tumors were 77 days (57-
key stakeholders from across the pediatric oncology landscape. It can 106 days) and 32 days (12-53 days), respectively. After PDSA1, overall
be a valuable guide to clinicians who will be tasked with the design of WT mean decreased by 44% to 25 days (3-65 days). WT for leukemia
future clinical trials. This is especially timely as genetic analyses play an patients decreased by 42% to 32 days (6-65 days) and for solid tumor
increasingly vital role in cancer research. patients decreased by 53% to 17 (3-29 days).
Conclusions: By using QI methodology, we significantly decreased time
to TIVAP placement. We will continue to implement interventions
E-Poster Topic: AS05.p Supportive Care and Palliative Care based on future PDSA cycles to ensure sustainability of the results over
time.
E-POSTER VIEWING

EP394 / #1813 USE OF QUALITY IMPROVEMENT EP395 / #23 KETAMINE INFUSION FOR NEUROPATHIC PAIN
METHODOLOGY TO IMPROVE TIMELY PLACEMENT OF IN CHILDREN WITH CANCER: KING HUSSEIN CANCER CENTER
TOTALLY IMPLANTABLE VENOUS-ACCESS PORTS IN CHILDREN EXPERIENCE
WITH CANCER IN A RESOURCE-LIMITED SETTING
Anwar Al-Nassan, Shireen Al- Awadi, Iyad Sultan
Gabriela Aguiar1 , Marcela Baltazar1 , Fernanda Buendia1 , Alicia King Husien Casncer Center, Pediatric, AMMAN, Jordan
Sanchez1 , Angelica Martinez1 , Mario Ornelas1 , Gabriela Tamayo1 ,
Maribel Ramirez1 , Marco Aguilera1 , Rebeca Rivera-Gomez1,2 , Paula Background and Aims: Pain control can be challenging to achieve
Aristizabal3,4,5 in children with terminal cancer. Neuropathic and somatic pain sen-
1 Hospital General Tijuana, Pediatric Oncology, Tijuana, Mexico; sations may form a complex of symptoms that do not respond to
2 Universidad Autonoma de Baja California, Facultad De Ciencias De conventional pain medications.
La Salud, Tijuana, Mexico; 3 University of California, San Diego, Pediatrics, Methods: We identified patients managed in our pediatric department
La Jolla, United States of America; 4 University of California, San Diego, who received ketamine during a 4-year period. Their demographics,
Moores Cancer Center Population Science, Disparities And Community pain scores, medication lists and response to ketamine were recorded.
Engagement, La Jolla, United States of America; 5 Rady Children’s Hospital Results: Fifteen patients (5 females; median age 14 years; range, 4.2 to
San Diego, Peckham Center For Cancer And Blood Disorders, San Diego, 24) were included in this analysis. Seven patients had bone sarcoma;
United States of America 4 of them had phantom pain following limb amputation. Patients were
on high doses of narcotics and other adjuvant medications. The median
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ABSTRACTS S333 of S635

pain score at time of starting ketamine infusion was 8 (range, 7 to 10). EP397 / #352 RISK STRATIFICATION TO AID MANAGEMENT
Ketamine was started at 0.1mg/kg/hour in all patients and increased OF FEBRILE NEUTROPENIA IN PAEDIATRIC ONCOLOGY
to a median of 0.2mg/kg/hour (range, 0.1 to 0.5). The lowest pain score PATIENTS: A SINGLE CENTRE STUDY
achieved was 2 (range, 0 to 4). Narcotics were tapered after a median
of 5 days (range, 2 to 13). Ketamine was stopped successfully in 10 Yasmeen Alomari, Sophie Wilne
patients while 5 died of their disease while receiving ketamine. The 4 Nottingham University Hospital NHS Trust, Paediatric Oncology, Notting-
patients with phantom pain benefited the most and were all discharged ham, United Kingdom
with controlled pain after stopping ketamine.
Conclusions: Ketamine resulted in dramatic pain relief in our patients. Background and Aims: Febrile Neutropenia (FN) is the common-
Those with phantom pain benefited the most, indicating potency est fatal complication of anticancer treatment in paediatric oncology
against neuropathic pain. It deserves further evaluation in treating patients with a mortality rate of 2-21%. Risk stratification can be
cancer pain in patients with advanced disease. used to identify children and young people (CYP) at low risk of a life-
threatening cause of FN in whom FN treatment could be stepped
down to out-patient oral antibiotic treatment. In Nottingham Chil-
EP396 / #754 TIMELY REFERRAL TO PEDIATRIC PALLIATIVE dren’s Hospital, CYP admitted with FN were risk stratified using the
CARE SERVICE DECREASES UTILIZATION OF HOSPITAL Swiss Paediatric Oncology Group (SPOG) score. This study aims to
RESOURCES IN CHILDREN DYING WITH CANCER determine whether stepping down treatment to oral antibiotics was
associated with any adverse outcomes.
Anwar Al-Nassan, Shireen Al- Awadi, Eman Khattab, Reem Khalil, Iyad Methods: Data was collected from CYP admitted with FN from
Sultan September 2016 to September 2020. Fever neutropenia episodes
King Husien Casncer Center, Pediatric, AMMAN, Jordan were defined according to the SPOG scoring tool and NICE guide-
line. Admitted CYP with FN were stratified into low and standard
Background and Aims: Demand for services increases towards the risk groups using the SPOG score. Data was collected on duration of
end-of-life in children with terminal cancer. This may be stressful to admission, culture results and any adverse outcomes.
patients and their families while exhausting hospital limited resources. Results: 227 FN episodes were reviewed. Mean age at admission was
Methods: We identified patients who died at our unit over the study 7.17 years (range 0-18). 55% (125) had acute lymphoblastic leukaemia
period (Jan2016-Dec2021) using our palliative care service database. and 59% (74) of these were in their maintenance treatment. 41% (93) of
Service utilization was queried using our medical records. A sheet that FN admissions were classified as low-risk (LR), and 59% (134) as stan-
includes all encounters was then analyzed and utilization was com- dard risk (SR). In the SR group 57% (71) of CYP had a positive blood
pared for the 2 months that preceded palliative care referral and the culture. In the LR group, there were 4 positive blood cultures, all subse-
period after referral. Paired t-student’s test was used for statistical quently shown to be a contaminant rather than true infection. 27% (25)
comparison. in the LR group had a positive respiratory viral swab. There were no
Results: We identified 352 patients who died during the studied period. episodes of sepsis, intensive care admissions or deaths in the LR group.
Most patients (N=328, 90%) had an active DNR order by the time Conclusions: This study demonstrates the safety of treatment with oral
of death. Four families had reversed DNR orders before death. The antibiotics in CYP with low-risk FN stratified after admission using the
average timing of referral to palliative care service was of 51 days SPOG risk stratification tool. Stepping down treatment to oral antibi-
(+/-88) before death. Forty-seven patients (13%) died at home, while otics allows earlier discharge which is beneficial for CYP well-being and
the rest died in hospital units (N=227, 64%), ER (N=9, 3%) or ICU reduces healthcare costs and bed utilisation.
(N=38, 10%). Leukemia patients were more likely to die without DNR
orders (26%) than other patients (6.7%, P<0,001). Service utilization
decreased after palliative care service referral as follows: Number of EP398 / #163 GUIDING PRINCIPLES FOR CARE OF
admissions (average,1.2 per patient before referral to 0.8 after refer- ADOLESCENT AND YOUNG ADULT PATIENTS WITH CANCER IN
ral, P<0.001), number of ER visits (8.7 vs 2.6, P<0.001) and outpatient LATIN AMERICA
visits (4.0 vs. 3.2, P=0.038). Total inpatient days (10.8 vs. 3.7 day per
patient, P<0,001) and ICU days (2.2 vs. 0.1, P<0,001) were significantly Elysia Alvarez1 , Emily Johnston2 , Wendy Gomez Garcia3 , Soad
less after palliative care referral. Home visits were infrequent and rep- Fuentes-Alabi De Aparicio4 , Federico Antillón-Klussmann5 , Ligia Fu6 ,
resented a small percentage of all encounters recorded (0.8%, N=198 Pascale Gassant7 , Ligia Rios8 , Crystal Romero1 , Marcio
visits). Malogolowkin1 , Paola Friedrich9
Conclusions: Service utilization decreased significantly after refer- 1 University of California Davis, Pediatrics, Sacramento, United States of
ral to palliative care service. Most of our patients died with a America; 2 University of Alabama Heersink School of Medicine, Pediatrics,
DNR order in place and approximately 1 out of 8 died at home. Birmingham, United States of America; 3 Dr. Robert Reid Cabral Children’s
Improving our home care program may help in better utilization of Hospital, Pediatric Oncology, Santo Domingo, Dominican Republic; 4 Pan
services. American Health Organization, Non-communicable Diseases, Washington
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S334 of S635 ABSTRACTS

D.C., United States of America; 5 Unidad Nacional de Oncología Pediátrica, Background and Aims: Tumour lysis syndrome (TLS) is an oncologi-
Oncología, Guatemala City, United States of America; 6 Hospital Escuela cal emergency associated with high morbidity and mortality especially
Universitario, Pediatric Oncology, Tegucigalpa, Honduras; 7 Hôpital Saint in centres with limited resources. It is a metabolic disorder charac-
- Damien, Pediatric Oncology, Port-au-Prince, Haiti; 8 Hospital Edgardo terized by hyperuricaemia, hyperkalaemia, hyperphosphataemia, and
Rebagliati Martins, Oncology, Lima, Peru; 9 St. Jude Children’s Research hypocalcaemia, with or without renal insufficiency. TLS usually follows
Hospital, Oncology, Memphis, United States of America chemotherapy but can occur prior to any cancer-related treatment.
Prevention, early detection, and management of TLS help improve the
Background and Aims: Most adolescents and young adults (AYAs: survival of children with cancer. The aim of this study was to determine
ages 15-39) with cancer live in low- and middle-income countries the incidence and factors associated with TLS among children between
(LMIC), including Latin America (LA). However, the majority of what is 0 to 17 years of age, newly diagnosed with cancer, admitted at the
known about this unique group is from high-income countries, includ- Department of Child Health of the Korle Bu Teaching Hospital, Accra,
ing guidelines for care. To fill this gap, we developed an expert panel Ghana
endorsed list of guiding principles for AYA cancer care in LA. Methods: Hospital-based prospective study conducted from Septem-
Methods: We used the modified Delphi method to gather expert ber 2020 to February 2021 on 55 children newly diagnosed with
opinion on guiding principles for the care of AYAs with cancer in cancer. Patients were followed and the socio-demographic, physi-
LA. The expert panel consisted of 25 pediatric and medical hema- cal diagnosis, imaging, laboratory and treatment data were collected.
tologist/oncologists from 15 countries (12 in LA). The panelists were Serum uric acid, creatinine, potassium, phosphate, and calcium were
electronically presented with 18 proposed guiding principles obtained measured before chemotherapy (Day 0/baseline), then after initiation
from literature review and surveys and interviews conducted previ- of chemotherapy on Day + 1 & Day + 3 in all patients. Data was entered
ously with LA physicians. The panelists scored each proposed guiding and analysed by SPSS version 25.
principle’s importance on a 9-point scale. Panelists were able to mod- Results: Among 55 oncology patients, 33(60.0%) were male. Median
ify or recommend additional guiding principles. After the 1st and age was 3.8[IQR 1.9 - 7.1] years. Cancer types were solid tumours
2nd round, the study team modified and added guiding principles based 38(69.1%), acute leukaemia 9(16.4%) and lymphoma 8(14.6%). Most
on panelist’s comments. In each round (3 total), guiding principles cancers 38(69.1%) were high-risk. The overall incidence of TLS was
were considered endorsed if they had a median score of ≥ 7 (with no 10/55(18.2%), with one case of spontaneous clinical TLS, and 70%
participant marking it as <3). cases by Day+1. Metabolic abnormalities were hyperphosphataemia-
Results: The twenty endorsed guiding principles fell into 4 categories: 10/10(100%), hyperuricaemia-8/10(80%), hyperkalaemia-4/10(40%)
Clinical Care (e.g., patients should be able to continue care where and hypocalcaemia-0/10(0%). TLS was present in 2/8(25%) and
they started), Support Services (e.g., support group of peers), Educa- 8/38(21%) participants with lymphoma and solid tumours respectively.
tion for Physicians (e.g., education on presentation of cancer in AYAs), Factors associated with TLS were high-risk disease (p = 0.020) and
and Advocacy (e.g., AYA specific foundations). Unendorsed statements LDH ≥ 1000 IU/L (p = 0.030). There was no TLS-related mortality.
included where patients should receive treatment (pediatric vs adult Conclusions: incidence of TLS in solid tumours was comparable to
center) based on age. lymphoma due to advanced disease at presentation. TLS prophylaxis
Conclusions: This study identified 20 guiding principles for AYA can- should be more aggressive in patients with high-risk disease and LDH
cer care in LA, which can be incorporated into cancer control programs. >=1000 IU/L.
This study serves as a framework for developing guiding principles for
other LMIC with the goal of improving AYA cancer care globally.
EP400 / #240 INCLUSION OF PHARMACISTS IN THE
MULTIDISCIPLINARY TEAM DEVELOPING EMERGENCY
EP399 / #778 INCIDENCE AND FACTORS ASSOCIATED WITH CHEMOTHERAPY PROTOCOLS IMPROVES CHEMOTHERAPY
TUMOUR LYSIS SYNDROME AMONG PAEDIATRIC ONCOLOGY PRESCRIBING AND ADMINISTRATION
PATIENTS AT THE KORLE BU TEACHING HOSPITAL, ACCRA,
GHANA Shauna Arao1 , Jennifer Geel2 , Joyce Kambugu3 , Denise Williams4
1 Uganda Cancer Institute, Department Of Pediatric Oncology, Kam-
Kokou Amegan-Aho1 , Lily Tagoe2 , Ernestina Schandorf2 , Diana pala, Uganda; 2 University of the Witwatersrand, Division Of Paediatric
Dwuma-Badu2 , Nihad Salifu3 , Victoria Adabayeri2 , Catherine Haematology-oncology, Faculty Of Health Sciences, School Of Clinical
Segbefia4 , Lorna Renner4 Medicine, Johannesburg, South Africa; 3 Uganda Cancer Institute, Pedi-
1 University of Health and Allied Sciences, Department Of Paediatrics And atric Oncology, Kampala, Uganda; 4 Cambridge University NHS tRUST,
Child Health, Ho, Ghana; 2 Korle Bu Teaching Hospital, Department Of Child Department Of Paediatric Oncology, CAMBRIDGE, United Kingdom
Health, Accra, Ghana; 3 Greater Accra Regional Hospital, Department Of
Child Health, Accra, Ghana; 4 University of Ghana Medical School, College Background and Aims: Uganda Cancer Institute (UCI) is a cen-
of Health Sciences, Department Of Child Health, Accra, Ghana tralised facility accepting approximately 600 paediatric oncology
patients annually. Chemotherapy medication errors at UCI are partially
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S335 of S635

ascribed to unstandardised chemotherapy prescription and adminis- a patient-centered, drug-focused, and outcome-oriented process of
tration practices. Key challenges include 4-6 weeks’ delay in obtaining drug therapy management. Three main barriers to a pharmacist’s pres-
confirmed histologic diagnoses and low staffing levels. Objective: to ence in paediatric oncology units (POUs) in low- and middle-income
determine whether the introduction of four standardised "emergency" countries previously reported include: a) health system structural con-
protocols improved the accuracy of prescription and administration, straints; b) low level/quality of community pharmacy services and
and staff acceptance thereof. c) educational and professional factors. Aim: identify African POUs
Methods: A multidisciplinary team devised four "emergency" proto- with dedicated pharmacists and describe facilitators and barriers for
cols: 1) cyclophosphamide, vincristine, prednisolone for suspected lym- dedicated POU pharmacists.
phoid malignancies, 2) vincristine, dactinomycin, cyclophosphamide for Methods: SIOP Global Mapping Programme data extracted from a sur-
suspected soft tissue sarcomas, 3) vincristine, carboplatin, etoposide, vey of African paediatric oncology facilities (2018-2020) documented
cyclophosphamide for suspected neural tumours, and 4) carboplatin, dedicated pharmacists in POUs. Expert opinion was sought from the
etoposide, bleomycin for suspected germ cell tumours. A pilot project pharmacist authors to summarise barriers and facilitators for POU
assessed impact of the new protocols on medication errors. pharmacists.
Results: Twenty-Four Files were evaluated. Sixteen (8 doctors, 8 Results: Mapping data indicated that of 47 African countries surveyed,
nurses) assessed the usefulness of the new protocols. Presence of 91/109 responding POUs had a dedicated pharmacist (don’t know=1;
a filed histologic diagnosis was unchanged before and after project missing data=9). Barriers for a dedicated pharmacist include funding,
implementation (9/12 to 10/12). Reasons included insufficient pathol- lack of trained staff, and lack of career guidance. Facilitators include
ogists, one immunohistochemistry machine, stock-outs of reagents. resource allocation, acknowledgment of pharmacist expertise, inter-
Rates of sign-off of chemotherapy prescriptions by qualified medical national non-governmental organisation and professional association
doctors did not change noticeably (4/12 to 6/12) because of unchanged support, mentors, and increased data to advocate for more staff.
high patient volumes with few doctors. There was improvement in pre- Conclusions: Most reporting African POUs have a dedicated pharma-
scription of supportive fluids (2/12 to 12/12) and antiemetics (9/12 cist. The scope of service should be documented and standardised.
to 12/12), attributed to clear protocol guidance. Majority of doctors Barriers should be systematically analysed using a standardised oncol-
(7/8) felt that the tool increased accuracy in prescribing process, and ogy pharmacy scope approach and facilitators exploited to ensure that
2/2 ward nurses and 0/8 outpatient nurses felt that the tools improved all POUs have the essential services of a dedicated pharmacist. The
chemotherapy administration process. SIOP Global Health Network OncoPharmacy Working Group has ini-
Conclusions: Emergency protocol prescription has enabled a system- tiated coordination with 91 African POUs, to assist other units to
atic method to treat patients before histologic results are available. establish clinical pharmacy services. It is critical to produce an essen-
Inclusion of a pharmacist in the MDT facilitated standardisation of tial requirements guideline to document minimum competencies for
prescription and administration. Supportive care improved and errors paediatric oncology pharmacists, detailing the space, equipment, and
decrease, but nursing acceptance and satisfaction are currently low, tools required to treat children with cancer. Future directions include
which warrants further exploration. Such interventions are low cost documenting the impact of dedicated POU pharmacists and how they
and have high impact in LMIC settings. support childhood cancer treatment, nursing occupational safety, and
parent/caregiver medication education

EP401 / #1416 DEDICATED PHARMACISTS IN AFRICAN


PAEDIATRIC ONCOLOGY UNITS: A SIOP GLOBAL MAPPING EP402 / #1649 GLOBAL CHALLENGES IN PEDIATRIC
PROGRAMME REPORT ONCOLOGY CRITICAL CARE IN RESOURCE-VARIABLE
SETTINGS: A ONE-YEAR EXPERIENCE
Shauna Arao1 , Julia Challinor2 , Neil Ranasinghe3 , Atalay Mulu4 , Sherif
Kamal5 , Jennifer Geel6 Anita Arias1 , Firas Sakaan2 , Maria Puerto-Torres2 , Carlos Acuña3 ,
1 Uganda Cancer Institute, Department Of Pediatric Oncology, Kampala, Zebin Al Zebin4 , Parthasarathi Bhattacharyya5 , Jeroselle Bugay6 ,
Uganda; 2 University of California San Francisco, School Of Nursing, San Carolina Delgado7 , Daiane Ferreira8 , Habtamu Gizaw9 , Carmina
Francisco, United States of America; 3 London Stock Exchange group, Lead Guitart10 , Sanjeeva Gunasekera11 , Maribel Ibarra12 , Joyce
Technicians, london, United Kingdom; 4 Addis Ababa University, College Kambugu13 , Reham Khedr14 , Jaime Libes15 , Eliana Lopez-Baron16 ,
of Health Sciences, School of Pharmacy, Department Of Pharmacology Murilo Luiz17 , Angelica Martinez18 , Alejandra Méndez-Aceituno19 ,
And Clinical Pharmacy, Addis Ababa, Ethiopia; 5 Children Cancer Hospi- Erika Montalvo20 , Hong Ren21 , Victor Sanchez-Torres22 , Rana
tal, Pharmacy, Cairo, Egypt; 6 University of the Witwatersrand, Division Of Sharara-Chami23 , Wu Shuiyan24 , Daniel Sousa25 , Margarita Torres26 ,
Paediatric Haematology-oncology, Faculty Of Health Sciences, School Of Silvio Torres27 , Diana Valencia28 , Ivonne Villanueva29 , Huma Zafar30 ,
Clinical Medicine, Johannesburg, South Africa Janet Middlekauff2 , Andrew Pappas2 , Asya Agulnik1,2
1 St. Jude Children’s Research Hospital, Division Of Pediatric Critical Care,
Background and Aims: An oncology clinical pharmacy, part of a mul- Memphis, United States of America; 2 St. Jude Children’s Research Hos-
tidisciplinary approach to curing and managing paediatric cancer, is pital, Department Of Global Pediatric Medicine, Memphis, United States
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S336 of S635 ABSTRACTS

of America; 3 Hospital Dr. Luis Calvo Mackenna, Pediatric Critical Care Results: Aggregated data across 28 centers from 19 different countries
Unit, Santiago, Chile; 4 King Hussein Cancer Center, Pediatric Hematol- of varying economic levels and pediatric critical care services were
ogy And Oncology, Amman, Jordan; 5 Tata Medical Center, Department Of analyzed for performance comparison. Identified common challenges
Pediatric Oncology Critical Care, Kolkata, India; 6 East Avenue Medical Cen- included: 1) shortage of pediatric intensivists resulting in inability to
ter, Pediatric Critical Care Unit, Quezon City, Philippines; 7 Hospital del provide coverage 24 hours a day/7 days a week, 2) lack of updated
Niño DIF Hidalgo, Pediatric Critical Care Unit, Hidalgo, Mexico; 8 Barretos guidelines to direct the care of critically ill PHO patients such as
Children’s Hospital, Pediatric Critical Care Unit, Barretos, Brazil; 9 Jimma chemotherapy-related toxicities, 3) absence of abstinence and with-
University Medical Center, Pediatric Critical Care Unit, Jimma, Ethiopia; drawal symptoms monitoring, 4) inability to obtain blood products
10 Hospital Sant Joan de Déu, 10. pediatric Critical Care Unit, Barcelona, emergently within 1 hour, and 5) lack of collaborative work with other
Spain; 11 National Cancer Institute, Paediatric Oncology, Maharagama, Sri institutions to benchmark outcomes.
Lanka; 12 Centro Medico Nacional Siglo XXI, Pediatric Critical Care Unit, Conclusions: PROACTIVE is a contextually appropriate diagnostic
México City, Mexico; 13 Uganda Cancer Institute, Pediatric Oncology, Kam- tool that can help clinicians and organizations identify challenges in
pala, Uganda; 14 Children’s Cancer hospital Egypt 57357/ National Cancer POCC services and prioritize among multiple quality improvement
Institute - Cairo University, Pediatric Oncology, Cairo, Egypt; 15 University initiatives. Aggregated PROACTIVE data identified common organiza-
of Illinois College of Medicine, Department Of Pediatric Hematology And tional challenges in POCC services that can be used to develop global
Oncology, Peoria, United States of America; 16 Hospital Pablo Tobón Uribe, interventions to improve outcomes for critically ill children with cancer.
Pediatric Critical Care Unit, Medellín, Colombia; 17 Hospital da Criança de
Brasilia, Pediatric Critical Care Unit, Brasilia, Brazil; 18 Hospital General
de Tijuana, Pediatric Hemato-oncology Unit, Tijuana, Mexico; 19 National EP403 / #840 EPIDEMIOLOGY OF CENTRAL VENOUS LINE
Unit of Pediatric Oncology, UNOP, Pediatric Critical Care, Guatemala, (CVL) DYSFUNCTION IN CHILDREN WITH CANCER: RESULTS
Guatemala; 20 Hospital Oncológico Solca Núcleo de Quito, Department OF A PROVINCIAL PROSPECTIVE COHORT STUDY
Of Pediatric Critical Care, Quito, Ecuador; 21 Shanghai Children’s Med-
ical Center, Department Of Pediatric Intensive Care Unit, Shanghai, Uma Athale1 , Jacqueline Halton2 , Leonardo Brandao3 , Laura
China; 22 Instituto Mexicano del Seguro Social, Pediatric Critical Care Wheaton4 , Soumitra Tole5 , Trishana Nayiager1 , Gary Foster6 , Lehana
Unit, Guadalajara, Mexico; 23 American University of Beirut Medical Cen- Thabane6 , Anthony Chan1
ter, Department Of Pediatrics And Adolescent Medicine, Beirut, Lebanon; 1 McMaster University, Pediatrics, Hamilton, Canada; 2 Children’s Hospital
24 Children’s Hospital of Soochow University, Pediatric Critical Care Unit, of Eastern Ontario, Pediatrics, Ottawa, Canada; 3 The Hospital for Sick Chil-
Suzhou, China; 25 A.C. Camargo Cancer Center, Pediatric Critical Care Unit, dren, Pediatrics, Toronto, Canada; 4 Kingston General Hospital, Pediatrics,
São Paulo, Brazil; 26 Clínica Imbanaco Grupo Quirón Salud, Pediatric Crit- Kingston, Canada; 5 London Health Sciences Center, Pediatrics, London,
ical Care Unit, Cali, Colombia; 27 Hospital Universitario Austral, Pediatric Canada; 6 McMaster University, Hei, Hamilton, Canada
Intensive Care Unit, Buenos Aires, Argentina; 28 IMAT Oncomédica, Pediatric
Critical Care Unit, Montería, Colombia; 29 Hospital Teletón de Oncología, Background and Aims: CVL dysfunction (CVL-D) is a common, yet
Pediatric Critical Care Unit, Querétaro, Mexico; 30 The Children’s Hospital not well described complication in children undergoing cancer ther-
and University of Child Health Sciences, Department Of Pediatric Oncology, apy. Hence, we undertook a provincewide prospective cohort study to
Lahore, Pakistan define the epidemiology and risk factors predisposing to CVL-D and its
outcome.
Background and Aims: Over 80% of children with cancer reside in Methods: Children (<18 yrs.) with newly diagnosed non-CNS cancer
low- and middle-income countries which face multiple challenges deliv- and no history of TE or anticoagulation therapy, were recruited at
ering high-quality pediatric onco-critical care (POCC). The PediatRic diagnosis from 5 tertiary-care pediatric oncology centres in Ontario
Oncology cApaCity Assessment Tool for IntensiVe CarE (PROACTIVE) (n=519). Details of demography, cancer diagnosis and therapy, CVL
was launched in 2021 to help organizations evaluate strengths and lim- insertion and removal, CVL complications (CVL-D [defined as persis-
itations in POCC services. In this study, we describe common global tent or recurrent difficulty in infusion, blood draw or both], thrombosis
challenges in POCC services in resource-variable settings identified and infection) while on primary cancer therapy, cancer-outcome and
using PROACTIVE. survival were collected. Analyses were performed to evaluate the
Methods: PROACTIVE is an electronic assessment tool divided into impact of patient and CVL-related factors on the risk of CVL-D.
2 English-language surveys for intensivists and oncologists manag- Results: Of 486 evaluable patients (median age 6.27yrs.; 212 (44%)
ing critically ill pediatric hematology-oncology patients. The tool is females), 182 (37.4%) had CVL-D during cancer-therapy. Of 141 (29%)
arranged into 8 domains and 22 subdomains with answer options in patients that developed CVL-D with first CVL, median time to CVL-D
the dichotomous, numerical, and Likert scale. Aggregated data from was 47 days; 93 (66%) patients had difficult blood draw and 21(15%)
28 centers that completed PROACTIVE between January 2021 and had more than one type of dysfunction. Seventy-eight of 141 patients
March 2022 were retrospectively analyzed and summarized. Indica- (55%) had at least one investigation for CVL-D; 28 (20%) had mechan-
tors with a mean score ≤ 75% in accessibility or performance across ical cause and 5 (3.5%) had thrombotic etiology. Eighty-seven of 141
all centers were classified as common global challenges in POCC. (62%) patients received some CVL-D-directed therapy; tPA in 58
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S337 of S635

(41%), anticoagulation in 3(2%) and 33 (23%) required surgical inter- Conclusions: Cyclophosphamide-based regimens especially with dox-
vention. Patients with CVL-D had significantly more catheter days orubicin or cisplatin have higher potential for CINV. Although aprepi-
(median 756 vs. 387; p=0.008). On multivariable analyses, younger tant was reported as a potent antiemetic drug for CINV, its effect on
age, hematologic malignancies, number of CVLs and CVL care-bundle appetite was mild in our group.
were significantly associated with CVL-D.
Conclusions: CVL-D occurred in 37% of children during cancer-
therapy; younger patients and those with hematological malignancies EP405 / #1988 DEVELOPMENT OF A GLOBAL PALLIATIVE
were at higher risk for CVL-D. Over 20% patients with CVL-D required CARE PROGRAM
either revision or removal of CVL underscoring the need for adequate
evaluation and management of CVL-D. Efforts also need to be directed Michael Mcneil1 , Ximena Garcia2 , Marta Salek1 , Justin Baker3
at prevention of CVL-D. 1 St. Jude Children’s Research Hospital, Department Of Global Pediatric
Medicine, Memphis, TN, United States of America; 2 St. Jude Children’s
Research Hospital, Global Pediatric Medicine, Memphis, United States of
EP404 / #1336 CHEMOTHERAPY-INDUCED NAUSEA AND America; 3 St. Jude Children’s Research Hospital, Division Of Quality Of Life
EMESIS CONTROL IN CHILDREN WITH CANCER: And Palliative Care, Memphis, United States of America
SINGLE-CENTER EXPERIENCE
Background and Aims: Disparities in access to pediatric palliative
Cagri Coskun, Burca Aydin, Pinar Yilmaz, Ebru Oluc, Gozde Arslan, care (PPC) and pain management remain an under-addressed global
Nilgun Kurucu, Bilgehan Yalçın, Ali Varan, Tezer Kutluk health issue, especially in low- and middle-income countries (LMICs).
Hacettepe University Faculty of Medicine, Department Of Pediatric Oncol- Although integration of palliative care (PC) is currently considered a
ogy, Ankara, Turkey quality standard in children with cancer, very few hospitals, countries
and regions have achieved this standard. The St Jude Global Palliative
Background and Aims: Chemotherapeutics have varying degrees Care Program was created to define the current state of PC in under-
of emetogenic potential. This study aimed to document the emeto- served communities worldwide and drive collaborative interventions
genic potential of chemotherapeutics and evaluate the effectiveness of with our global partners to relieve the suffering of patients and families
antiemetic treatment. facing the challenges of pediatric cancer. We describe the program’s
Methods: All patients receiving intermediate, high or very high eme- developmental processes and the challenges and success.
togenic chemotherapy for 6 months (May to December 2021) were Methods: The St Jude Global Palliative Care program was established
included in the study. Chemotherapy-induced nausea and vomiting in 2018 under the leadership of an interdisciplinary team of experts in
(CINV) was defined as acute (<24h) and delayed (24-120h), and overall PPC. It has been developed through 4 fundamental pillars: Research,
(0-120h). Emesis was graded according to NCI CTCAE (Version 4.03). Education, Capacity Building, and Advocacy. Activities in these areas
Results: In total, 167 children with a median age of 6.7 years (ranged have been developed in each of the WHO regions.
0.6-17 years), M/F=1.6) were included in the study. The frequency Results: From December 2018 to March 2022, the St Jude Global
of emesis was 33% in cyclophosphamide-based regimens, 30% in Palliative Care Transversal program has developed 16 educational
cisplatin-based regimens, 25% in anthracycline-based regimens and projects, 7 advocacy activities, 4 projects in capacity building and 4
14% in ifosfamide-based regimens. Emesis was found to be significantly large research studies. These activities have been conducted in 99
increased in patients receiving cyclophosphamide-based chemother- countries from 5 continents. Collaborative work with World Health
apy regimen (p=0.014). The combination of cyclophosphamide with Organization (WHO), Pan American Health Organization (PAHO),
anthracycline (74%) and cyclophosphamide with cisplatin (69%) had International Children’s Palliative Care Network (ICPCN), and the
the highest emetogenic potential. Changes in appetite were found in Worldwide Hospice Palliative Care Alliance (WHPCA) has been essen-
81%, 74%, 73% and 69% in ifosfamide-based, anthracycline-based, tial for developing activities. The program has developed a provider
cyclophosphamide-based and cisplatin-based regimens, respectively. network of 1,280 members from every continent of the world.
Complete protection rates to antiemetic treatment, in acute, delayed, Conclusions: The development of regional activities based on a needs
and overall phases were 91%, 81%, and 76% respectively. The complete assessment is essential to promote the development and prioritization
protection rate was significantly worse in children older than 6 years of PPC according to their locoregional needs and resources. We are
(p=0.032). Aprepitant, 5HT3 antagonist and dexamethasone were currently in the process of developing a global PPC strategic plan that
given for prevention of CINV for high and very high-risk emetogenic we will share at the SIOP meeting.
chemotherapy (n=82). But 47 children could not receive aprepitant
due to insurance or economic reasons, they only received 5HT3 antag-
onists and dexamethasone. No significant protective effect on appetite EP406 / #1298 PREVALENCE AND PERCEPTION REGARDING
was found in children received aprepitant compared to the others (23% COMPLEMENTARY AND ALTERNATIVE MEDICINE USE IN
vs 31%, p=0.3). But aprepitant provided greater complete control rate CHILDHOOD CANCER PATIENTS
of vomiting (84% vs 61%, p=0.004).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S338 of S635 ABSTRACTS

Sameer Bakhshi1 , Shuvadeep Ganguly1 , Ritika Singh1 , Vasudha Data Management, Kolkata, India; 4 Tata Medical Center, Tata Translational
Verma1 , Anubhav Das2 , Ayush Lohiya3 , Shubhangi Sharma1 Cancer Research Center, Kolkata, India
1 All India Institue of Medical Sciences, Medical Oncology, New Delhi, India;

2 ALL INDIA INSTITUE OF MEDICAL SCIENCES, Mbbs Student, New Delhi, Background and Aims: The SARS-CoV-2 pandemic has hindered
India; 3 Super Specialty Cancer Institute & Hospital, Public Health, Lucknow, timely access to paediatric cancer care. Infected children with cancer
India have a mild clinical course. Less well described is the impact of infec-
tion on cancer care on children. Here we report experience at a tertiary
Background and Aims: Complementary and alternative medicine cancer centre in India.
(CAM) use in childhood cancer patients is less explored. We estimated Methods: Beginning with the first infection wave,
prevalence of CAM use among children with cancer and analysed the national/institutional protocols required periodic mandatory test-
role of demographics, disease characteristics and parents’/caregivers’ ing for patients (and where applicable, caregivers) including at first
beliefs on CAM use. presentation, prior to procedures/admissions, with fever or contact
Methods: A cross sectional study was carried out between Decem- with an infected person. Infected patients were advised mandatory
ber 2020 and October 2021 where parent/caregiver of children quarantine (2-3 weeks), Infected patients and family contacts were
(≤18 years) on active treatment of malignancy were included. monitored by telephone and in some cases, were prescribed bridging
A draft interview schedule was prepared to capture the socio- oral chemotherapy. Due to logistic issues, only symptomatic or febrile
demographics, clinical details, details of CAM use, and reasons children were hospitalised in the dedicated Covid-19 ward.
for use/non-use. A 29-item interview schedule was finalized after Results: Over 23 months (March’20-Feb’22), 262 children with can-
expert review for content validation and pilot testing among cer or non-malignant haematological disorders (median age, 7 years;
30 participants for clarity/relevance. Binary logistic regres- interquartile, 4⋅3-11⋅7 years) were diagnosed with SARS-CoV-2 infec-
sion was used to assess impact of socio-demographic variables, tion, including 206 (79%) with haemato-lymphoid cancers and 53
malignancy type (haematological/solid), disease status (newly (20%) with solid tumours. In 46 (17.5%), infection was diagnosed at
diagnoses/relapse), treatment intent (curative/palliative), delay in first presentation, resulting in either delay (21/46, 46%) or failure
seeking treatment (above/below median symptom duration) on CAM to initiate treatment (18/46, 39%). Infection delayed intensive can-
use. cer treatment in 148 (56%), with 96 (65%) experiencing treatment
Results: Total 450 patients (median age 9 years) were included (68.9% delays of ≥ 14 days (median, 20 days, interquartile, 16-24 days).
males; 64% haematological malignancies), with 391 (86.9%) newly Infection less commonly affected maintenance chemotherapy (31/89,
diagnosed cases and 402 (89.3%) patients on curative-intent ther- 35%). Eighteen children (3%) required hospitalisation (median 4.5 days;
apy. Median symptom duration was 1 month. Non-prescribed reme- interquartile, 3-7 days), 2 (11%) with symptomatic Covid-19 and the
dies/therapy was used by 162 (36%) patients, of which 62 (13.7%) rest for treatment/disease-related toxicities. One died due to probable
patients used CAM and 125 (27.7%) patients used home-based reme- SARS-CoV-2-related multisystem inflammatory syndrome.
dies alone/concomitantly. The most common system of CAM used was Conclusions: The SARS-CoV-2 pandemic and the associated restric-
Ayurveda (41/62; 66.1%) followed by Homoeopathy (20/62; 32.2%). tions delayed initiation of cancer treatment and disrupted cancer treat-
Median duration of CAM use was 30 days with median expense of ment intensity in infected children, the impact of which may become
INR 1750 (USD 23). The most common reason of CAM use was sug- apparent with time. Given the protracted pandemic course, institutions
gestion by family member (27/62; 43.5%) while that of non-use of will need to develop dedicated facilities and services to ensure that
CAM was belief in efficacy of modern medicine (152/388; 39.1%). infected children with cancer continue to receive appropriate-intensity
CAM use was more common in patients on palliative-intent therapy treatment.
(OR:2.33; p=0.021) and those with delay in seeking care (OR:2.35;
p=0.003).
Conclusions: Ayurveda is the most commonly used system of CAM in EP408 / #1992 MEDICAL PERCEPTION ON PALLIATIVE
children with cancer. It is more common in palliative-intent therapy and CARE, THERAPEUTIC OBSTINACY, AND END OF LIFE, IN A
may contribute to treatment delay. PEDIATRIC ONCOLOGICAL HOSPITAL, IN SÃO PAULO STATE,
BRAZIL

EP407 / #940 IMPACT OF SARS-COV-2 PANDEMIC ON Dileiny Antunes Geronutti Ayub1 , Erica Boldrini2 , Leticia Miranda1
TREATMENT INITIATION AND DELIVERY IN CHILDREN WITH 1 Hospital de amor, Palliative Care, Barretos, Brazil; 2 Hospital de Amor de
CANCER Barretos, Palliative Care, Barretos, Brazil

Arpita Bhattacharyya1 , Sanjay Bhattacharya2 , Gaurav Goel2 , Jasmin Background and Aims: Cancer is not a unique disease, with a unique
Khatun3 , Parag Das4 , Shekhar Krishnan4 cause. It is a group of distinct diseases, with different causes, symp-
1 Tata Medical Center, Paediatric Haematology Oncology, Kolkata, India; toms, treatments, and prognoses. In childhood and adolescence, cancer
2 Tata Medical Center, Microbiology, Kolkata, India; 3 CanKids KidsCan India, is the first cause of death by disease in all regions of Brazil. Despite
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S339 of S635

the technical and scientific advancements achieved by oncology and lymphoid leukemia. Only 1 participant had evolved palliative status in
the effort of healthcare professionals, many diseases still threaten the the medical record, another 11 had poor prognostic factors. Of the
continuity of life and the aim of the treatment is not only to cure, but 15 respondents, 7 reported never having heard the word “Palliative
also to improve the quality of life, prevent and alleviate the suffering care”. Those who said they knew and understood the word “Palliative
of the patient and her/his family. JUSTIFICATION: The medical staff is Care” demonstrated an association between the term and the distance
familiar with dealing with the disease, however there is great difficulty from cancer treatment. As the main factors for elucidating the theme
in dealing with the particularities of individuals who cannot be cured; for this audience, most highlighted the importance of differentiating
along with the disease, the patients bring with them their fears, doubts, the term palliative care and its association with end-of-life care. The
expectations, as well as those of their families. Treating these patients need for accessibility for clear and effective communication was also
as a whole, tending to their needs, goes beyond care, it becomes a chal- highlighted.
lenge. AIM: To understand the medical perception on palliative care, Conclusions: Understanding the perceptions of adolescents under-
therapeutic obstinacy, and end of life, in a pediatric oncological hospital going cancer treatment on aspects of PC allows health providers
in São Paulo State, Brazil. to establish not only better and appropriate strategies for commu-
Methods: This is a descriptive, qualitative exploratory study. The refer- nication, but also the development of a care plan aligned with the
ence framework used to the analysis of qualitative data is the Method perspective of the patient and his family.
of Content Analysis proposed by Bardin.
Results: The data collected were organized according to the following
themes: Palliative Care in academic training; The lack of knowledge and EP410 / #2001 ASSESSMENT OF PAIN MANAGEMENT
the search for information; Conceptions on Palliative Care; Obstacles KNOWLEDGE BY THE NURSING TEAM OF A CHILDREN’S
faced to reach Palliative Care; Therapeutic obstinacy; End of life and a CANCER HOSPITAL IN A CITY IN THE INTERIOR OF SÃO
peaceful death PAULO
Conclusions: It is evident that issues as palliative care, therapeutic
obstinacy, and end of life are not sufficiently addressed in the train- Dileiny Antunes Geronutti Ayub1 , Erica Boldrini2 , Ana Carolina
ing of healthcare professionals, turning into a difficulty to them, when Oliveira2
facing certain situations during their professional lives. 1 Hospital de amor, Palliative Care, Barretos, Brazil; 2 Hospital de Amor de
Barretos, Palliative Care, Barretos, Brazil

EP409 / #1996 PALLIATIVE CARE IN PEDIATRIC ONCOLOGY: Background and Aims: In view of the difficulties in pain management
CONCEPTIONS OF ADOLESCENTS UNDERGOING CANCER and assessment, this research seeks to identify the level of assessment
TREATMENT of pain management by nursing professionals: nurses and nursing tech-
nicians from a Children’s Oncological Hospital, in a city in the interior
Dileiny Antunes Geronutti Ayub1 , Erica Boldrini2 , Flavia Binati1 of São Paulo .
1 Hospital de amor, Palliative Care, Barretos, Brazil; 2 Hospital de Amor de Methods: It consists of a longitudinal study with prospective data col-
Barretos, Palliative Care, Barretos, Brazil lection through a questionnaire prepared by the researcher, developed
at the Children’s Hospital, with 125 nursing staff collaborators, 62
Background and Aims: Considering the scarcity of research address- technicians and 63 nurses. The questionnaire and the informed consent
ing palliative care in adolescents with cancer diagnosis and treatment were handed by the researcher to the professionals in their respective
in the Brazilian context. It is necessary to investigate the conceptions sectors.
and meanings attributed to palliative care for adolescents with a Malig- Results: A number of 100 professionals were obtained in the research,
nant diagnosis, in order to support the development of educational being 47 nurses and 53 nursing technicians. The sector with the high-
materials that clarify the main issues related to the E-Poster Topic. est number of professionals is the Infirmary. The FACES and Numerical
Methods: This is an exploratory descriptive study with a qualitative scales were the most used (86). Most professionals had a period of
approach. The sample consisted of adolescent patients, aged between experience between five and 10 years (40). Most did not receive any
12 and 19 years, diagnosed with cancer for at least six months and teaching about pain during training and do not have extracurricu-
undergoing treatment at the institution. Data collection was divided lar training (36). Regarding the assessment of patients with cognitive
into two stages. Initially, data were collected to characterize the impairment, the vast majority reported having doubts (46). The most
sociodemographic and clinical profile of the participants. Sequentially, common side effect was dependence (35) and respiratory failure (36).
a semi-structured individual interview was carried out based on 5 When starting laxatives and using opioids, they responded at the
guiding questions. same time (62), but reported having doubts (16). Regarding the use of
Results: Fifteen adolescents participated in this study, among which morphine in sedation (63) reported being used,
46.7% were male, 53% of the sample had solid tumors, with a pre- Conclusions: There is a lack of knowledge regarding the assessment
dominance of the characteristic histological type for osteosarcoma, of pediatric pain, from the use of scales to their management. There is
while 47% presented with hematological neoplasms, thus for acute also a fear of professionals regarding the use of opioids in the pediatric
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S340 of S635 ABSTRACTS

population, as well as a lack of knowledge of pharmacokinetics, side Didi Bury1 , Tom Wolfs2 , Eline Muilwijk3 , Marta Fiocco4 , Rob Pieters5 ,
effects and their correct use for the treatment and management of Roger Brüggemann6 , Wim Tissing5,7
pain. 1 Princess Máxima Center for pediatric oncology, Supportive Care, Bilthoven,

Netherlands; 2 Wilhelmina Children’s Hospital, Department Of Infectious


Diseases, Utrecht, Netherlands; 3 Princess Máxima Center for pediatric
EP411 / #595 THE FIRST YEARS: AN EMBEDDED PEDIATRIC oncology, Department Of Pharmacy, Bilthoven, Netherlands; 4 Leiden Uni-
PALLIATIVE ONCOLOGY CLINIC versity, Mathematical Institute, Leiden, Netherlands; 5 Princess Máxima
Center for Pediatric Oncology, Department Of Pediatric Oncology, Utrecht,
Katharine Brock1 , Nicholas Degroote2 , Anna Roche2 , Karen Netherlands; 6 Radboud university medical center Nijmegen, Department Of
Wasilewski-Masker1 Pharmacy, Nijmegen, Netherlands; 7 University Medical Center Groningen,
1 Emory University, Children’s Healthcare of Atlanta, Pediatrics, Atlanta, Department Of Pediatric Oncology, Groningen, Netherlands
United States of America; 2 Children’s Healthcare of Atlanta, Aflac Cancer
& Blood Disorders Center, Atlanta, United States of America Background and Aims: Invasive fungal disease is frequently diagnosed
during the early phase of childhood acute lymphoblastic leukaemia
Background and Aims: Children with cancer have significant illness (ALL) treatment and is associated with morbidity and mortality. Pro-
burden, suffering, and familial stress. Pediatric palliative care (PPC) phylactic strategies might be beneficial, but first-line azole prophylaxis
improves quality of life and improves end-of-life outcomes, but often is hampered by the interaction with vincristine. A twice-a-week mica-
occurs late in the disease course. Expanding PPC to the outpatient fungin regimen for Aspergillus prophylaxis was therefore evaluated in
clinic improves PPC access and timing. The aims are to describe this study.
the inaugural four years (2017-2021) of one of the U.S’s first aca- Methods: Paediatric patients with ALL (09/2018-07/2020) received
demic, consultative, embedded pediatric palliative oncology (PPO) micafungin twice-a-week (9 mg/kg/dose [max. 300 mg]) during the
clinics, reviewing patient demographics, disease-based and treatment first five weeks of treatment (i.e. induction course), as part of routine
information, visit data, and palliative and end-of-life outcomes. care. A historical control cohort (04/2012-09/2018) was used with-
Methods: This is a single-institution prospective cohort study of pedi- out fungal prophylaxis during this induction course. After the first five
atric oncology patients aged 0 – 27 years seen in a PPO clinic from weeks (i.e. first consolidation course), standard mould-active prophy-
June 2017 through June 2021. Demographics, disease-based data, laxis was used in both groups. The percentage of proven and probable
PPC visit data, and end-of-life outcomes were collected prospectively. Aspergillus infections at the end of the first consolidation course (i.e. at
Descriptive statistics and changes over time were calculated. the end of week 10) was compared between the cohorts.
Results: During the first four years, 248 patients (51.6% male; 58.1% Results: A total of 169 and 643 paediatric patients with ALL were
White; 35.5% Black; 13.7% Hispanic/Latino) were seen in PPO clinic, included in the micafungin (median age, 4 years [range 1-17]; 43.2%
totaling 1,665 clinic visits (median 5, IQR 3, 9). Primary diagnosis was female) and historical cohort (median age, 5 years [range 1-17];
brain tumor (42.3%), solid tumor (37.1%), and leukemia/lymphoma 40.4% female), respectively. The percentage of proven and probable
(17.3%). The first point of PPC contact was PPO (70.6%) for most Aspergillus infections was 1.2% (2/169) in the micafungin cohort ver-
patients versus inpatient consult. Over four years, the proportion of sus 5.6% (36/643) in the historical cohort (p=0.013; Fisher’s exact
PPO consults occurring greater than 90 days from death increased test).
from 39.1% to 85.0%, and median time from PPO consultation to death Conclusions: Twice-a-week micafungin prophylaxis during the induc-
increased from 74 to 226 days (p<0.0001). Among the 136 deceased tion course significantly reduced the occurrence of proven and
patients (54.8%), 77.9% had a do-not-resuscitate, and 72.8% received probable Aspergillus infections in the early phase of childhood ALL
hospice care. When known (n=112), 89.3% died in their preferred loca- treatment.
tion. Early PPO (<12 weeks from diagnosis) and advance care plan
documentation (p=0.03 and p=0.02, respectively) were associated
with family identification of a preferred location of death EP413 / #253 FEASIBILITY OF THREE TIMES WEEKLY
Conclusions: Embedded PPO clinics can be successful, achieve steady SYMPTOM SCREENING IN PEDIATRIC CANCER PATIENTS
growth, and improve end-of-life outcomes through improved access
and timing of PPC delivery. Pediatric cancer centers should work Maryann Calligan1 , Lauren Chakkalackal1 , Grace Dadzie1 , Cassandra
collaboratively to include PPO outpatient services. Tardif-Theriault1 , Sadie Cook1 , Emily Vettese2 , Dilip Soman3 , Susan
Kuczynski4 , Tal Schechter5 , Lee Dupuis6 , Lillian Sung2
1 The Hospital for Sick Children, Child Health Evaluative Sciences, Toronto,
EP412 / #686 MICAFUNGIN TWICE-A-WEEK IS EFFECTIVE Canada; 2 Hospital for Sick Children, Child Health Evaluative Sciences,
FOR PROPHYLAXIS OF INVASIVE ASPERGILLUS INFECTIONS IN Toronto, Canada; 3 University of Toronto, Behavioural Economics At Rotman,
CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKAEMIA Toronto, Canada; 4 Pediatric Oncology Group of Ontario, Opacc, Toronto,
Canada; 5 Hospital for Sick Children, Division Of Haematology/oncology,
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ABSTRACTS S341 of S635

Toronto, Canada; 6 The Hospital for Sick Children, Department Of Pharmacy, but difficulties with family-school-clinician communication and shared
Toronto, Canada knowledge are barriers to access. Hospital-school liaison programs can
provide helpful consultation and facilitate communication. These ser-
Background and Aims: We created the Symptom Screening in Pedi- vices, however, can be costly and inaccessible to low-income families.
atrics Tool (SSPedi) to enable pediatric cancer symptom screening. We piloted a grant-funded liaison program to provide no-cost supports
However, the ability of pediatric patients to self-report symptoms lon- and evaluate impacts.
gitudinally was uncertain. Primary objective was to determine the Methods: The Hospital Education Liaison Program (HELP) was estab-
feasibility of three times weekly symptom reporting by pediatric cancer lished in 2020. Coordinated by a special educator, HELP provides
patients for eight weeks. consultation and facilitates communication among patients’ families,
Methods: We included English-speaking patients 8-18 years of age medical teams, and school teams. Clincian referrals to HELP document
with cancer. Patients were sent reminders by text or email to complete patient demographics, diagnoses, and education-related concerns. AT
SSPedi three times weekly for eight weeks. If patients reported at least HELP intake, the liaison provides psycho-social education regarding
one severely bothersome symptom, a symptom report was emailed education policies and potential schooling supports. The liaison and
to the primary healthcare team. Patient-reported outcomes were family set goals for HELP services engagment. Data regarding parent
obtained at baseline, week 4±1 and week 8±1. Symptom documenta- satisfaction, changes to patient educational services following HELP
tion, intervention provision for symptoms and unplanned healthcare intervention, and school team feedback are collected.
encounters were determined by chart review at weeks 4 and 8. Results: Since fall 2020, 34 patients have been referred to HELP (27.3%
Feasibility threshold was 75% patients achieving compliance with at in treatment, 29.4% recently completed treatment, 45.5%> 1 year
least 60% of SSPedi evaluations. We planned to initially enroll 20 post-treatment). Nearly half (48%) of patients are from low-income
participants and, if feasibility metrics were not met, to enroll succes- families. Most common reasons for referral were family concerns that
sive cohorts of 10 participants until feasible metrics were met or a educational needs were not being met (21.4%) and family confusion
maximum of 60 participants had been enrolled. about the academic support eligibility process (19.1%). All patients
Results: Two cohorts consisting of 30 patients (cohort 1 (n=20) and who engaged with HELP increased their formalized school supports.
cohort 2 (n=10)) were required to meet feasibility metrics. In cohort 1, School teams reported benefiting most from the liaison’s transla-
11/20 (55%) met SSPedi completion threshold. Interventions applied tion of medical information into educatinoal language and actionable
after cohort 1 included engaging parents to facilitate pediatric patient recommendations.
self-report, providing mechanisms to remember username and pass- Conclusions: This education liaison program has, thus far, improved
word and highlighting potential benefits of symptom feedback to patient access to educational supports. HELP offers just-in-time sup-
clinicians. In cohort 2, 9/10 (90%) met SSPedi completion threshold ports while simultaneously building family, clinician, and educator
and thus feasibility was met. Patient-reported outcomes and chart capacity. The program is a potential low-cost/ high-yield intervention
abstracted outcomes were obtained for all participants in cohort 2. for improving childhood cancer survivors’ educational attainment and
Conclusions: Three times weekly symptom reporting by pediatric quality of life. Further research is needed to determine which aspects
patients with cancer for eight weeks was feasible. Mechanisms to of the program characteristics are most impactful for families and
enhance three times weekly symptom reporting were identified and schools.
implemented. Future studies of longitudinal symptom screening can
now be planned.
EP415 / #1942 EFFICACY OF EMPIRICAL TREATMENTS
USED DURING EPISODES OF FEBRILE NEUTROPENIA IN
EP414 / #1393 SUPPORTING CHILDHOOD CANCER PEDIATRIC PATIENTS RECEIVING CHEMOTHERAPY:
SURVIVOR QUALITY OF LIFE AND EDUCATIONAL SYSTEMATIC REVIEW AND NETWORK META-ANALYSIS
ATTAINMENT: PILOTING THE HOSPITAL EDUCATION LIAISON
PROGRAM Osvaldo D. Castelán-Martínez1 , Carlos F. Flores-Torres1 , Karla M.
Silva-Jivaja2 , Miguel Palomo-Colli3 , Martha Sánchez-Rodriguez4
Lisa Carey1 , Kathy Ruble2 , Juliana Paré-Blagoev3 , Clifton Thornton4 , 1 Universidad Nacional Autónoma de México/Facultad de Estudios Supe-
Lisa Jacobson1 riores, Clinical Pharmacology Laboratory., Ciudad de México, Mexico;
1 Kennedy Krieger Institute, Neuropsychology, Baltimore, United States of 2 Universidad Nacional Autónoma de México, Clinical Pharmacology,
America; 2 Johns Hopkins University, School of Medicine, Pediatric Oncology, CDMX, Mexico; 3 Hospital Infantil de México Federico Gómez, Oncology,
Baltimore, United States of America; 3 Johns Hopkins University, School of CDMX, Mexico; 4 Universidad Nacional Autónoma de México/Facultad de
Education, Education, Baltimore, United States of America; 4 Johns Hopkins Estudios Superiores Zaragoza, Research Unit On Gerontology, Ciudad de
University, Nursing, Baltimore, United States of America México, Mexico

Background and Aims: Childhood cancer survivors often experi- Background and Aims: Febrile neutropenia (FN) is the first cause of
ence learning difficulties. Schooling supports can ameliorate impacts hospitalization due to chemotherapy complications and is the second
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S342 of S635 ABSTRACTS

diagnosis in children with cancer visiting the emergency room. Thus, sented to the game designers to make changes. During the iterative
the aim of this systematic review was to synthesize the evidence of process six meetings were held, either face to face or online, in groups
the efficacy of empirical treatments used during episodes of febrile or individually.
neutropenia in pediatric patients receiving chemotherapy, as well as to Results: The children expressed opinions about the RT content and
compare them through a network meta-analysis (NMA). wanted more content about RT to be displayed in the game, which was
Methods: A systematic literature review was carried out in MEDLINE, implemented accordingly. The children conveyed the coping strate-
Scopus, Cochrane Library (CENTRAL), Epistemonikus and LILACS gies they had used during RT which then was implemented in the
without restrictions. In addition, a gray literature was searched in Sco- game. Through the children’s play the investigators could come up
pus Conference and TESIUNAM. Randomized clinical trials (RCT) eval- with ideas to make the game more amusing following the children’s
uating the efficacy of empiric treatments for resolution of FN in pedi- wishes of developing a fun game. The children’s ability to participate
atric patients receiving cytotoxic chemotherapy were included. Odds was affected by the severity of their disease, consequently the prior
ratios (OR) 95% confidence intervals (95%CI) were estimated between plan of participation had to be modified.
the comparisons of the different treatments employing cefepime as Conclusions: Through the children’s participation the game changed in
reference. Direct, indirect, and mixed comparisons were performed several ways. Researchers within healthcare could use similar methods
using a frequentist-type NMA with a random effects approach using to co-create serious games together with children affected by a dis-
the NETMETA package of the statistical program R 4.0.5. ease. Since the severity of the children’s disease varies, it is necessary
Results: One thousand and thirty-six studies were identified as poten- to adjust in accordance with the children’s needs.
tially relevant. After analyzing titles, abstracts, and full titles, 18 studies
with 1547 FN episodes in 1006 pediatric patients were included in
the review. The most frequently used treatment was cefepime alone EP417 / #1038 AN EDUCATIONAL INTERVENTION TO
or in combination (11 RCT), followed by piperacillin/tazobactam (9 IMPROVE LEUCOVORIN ADHERENCE IN CHILDREN RECEIVING
RCT) and ceftazidime (7 RCT). NMA results showed no difference HIGH-DOSE METHOTREXATE IN LILONGWE MALAWI
in efficacy between cefepime and piperacillin/tazobactam (OR= 1.15,
[95%CI, 0.72 to 1.83]); results were similar when other antibiotics were Agness Chitedze1,2 , Nthongase Makamo1,2 , Anthony Stambuli1,2 ,
compared. Melanie Bernhardt3,4 , Nmazuo Ozuah1,2,3
Conclusions: This systematic review with NMA shows that empiric 1 Texas Children’s Global HOPE (Hematology/Oncology Pediatric Excel-
treatment for febrile neutropenia in pediatric cancer patients treated lence), Global Hope Malawi, Lilongwe, Malawi; 2 Baylor College of Medicine
with cytotoxic chemotherapy are similar in efficacy. Children’s Foundation- Malawi, Pediatric Hematology/oncology, Lilongwe,
Malawi; 3 Baylor College of Medicine, Pediatric Hematology/oncology, Hous-
ton, United States of America; 4 Texas Children’s Hospital, Global Hope
EP416 / #428 INVOLVING CHILDREN TREATED FOR (hematology/oncology Pediatric Excellence), Houston, United States of
CANCER IN DEVELOPING A SERIOUS GAME ABOUT America
RADIOTHERAPY
Background and Aims: Leucovorin rescue is essential in reducing
Catarina Cederved1 , Gunn Engvall2 , Gustaf Ljungman2 , Charlotte the risk of high-dose methotrexate toxicity. In our center, all patients
Ångström-Brännström1 , Jon Back3 receiving high-dose methotrexate (>/=1000 mg/m2 ) are given 12
1 Uppsala University, Women’s And Children’s Health, UPPSALA, Sweden; scheduled doses of oral leucovorin rescue, administered to the patient
2 Uppsala Universitet, Women’s And Children’s Health, UPPSALA, Sweden; by their guardian. As part of multidisciplinary rounds, we observed
3 Uppsala Universitet, Informatics And Media, UPPSALA, Sweden a high rate of non-compliance to leucovorin (<30%). We aimed to
improve adherence through a quality improvement project focused on
Background and Aims: Children can experience anxiety and fear in guardian education and tracking of leucovorin administration.
conjunction with radiotherapy (RT). A serious game could prepare chil- Methods: We developed targeted education as the first intervention
dren for the procedure, teach them about the treatment and plausibly in our quality improvement project. All patients were counseled on
reduce their anxiety. A serious game is screen-based and can influence appropriate dosing and administration by nurses and provided with
the player’s view of the displayed phenomenon in the game. To include a leucovorin tracking sheet. This sheet contains 12 check boxes for
children with experiences of RT in the developmental process increases each of the doses of leucovorin, scheduled time of administration, and
the likelihood that the game will be appropriate for children. The aim prescribed dose (number of tablets). Guardians were educated on the
was to describe the contributions made by children through partici- number of tablets to administer, dose timing, and ticked a check box
patory action research during the developmental process of a serious after each dose was administered.
game about RT. Results: Forty-eight patients received high-dose methotrexate
Methods: Nine children (7-10 years old) with firsthand experience of between April 2021 and January 2022. Guardians of 23 patients were
RT were included. Through interviews and participant observations given the form and tablets as scheduled. Wall clocks were provided in
of the children playing the game, a list of proposed changes was pre- each room to help guardians monitor the correct administration time.
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ABSTRACTS S343 of S635

Following implementation of the intervention, 64% of patients had no move to the hospital. This indicates factors directly associated with
missed doses and used the form correctly, while 36% had at least 1 the primary caregivers possibly are elements that favor the lack of
missed dose and did not complete the form correctly. Mucositis was adherence and/or dropout of pediatric cancer patients.
observed in 25% of those who adhered, vs. 50% of those who didn’t.
Most guardians are illiterate and required assistance from a nurse to
help complete the form. EP419 / #1993 USE OF BEDSIDE ULTRASOUND AND
Conclusions: The leucovorin tracking form was helpful in assessing ANTHROPOMETRY TO EVALUATE SARCOPENIA IN CHILDREN
leucovorin adherence. Additional challenges were uncovered, such as WITH CANCER
literacy levels of guardians. We are following up with a study to assess
the impact of this intervention on the rate of methotrexate toxicity in Wilson De Oliveira Jr1 , Mariana Murra2 , Carlos Eduardo Cavalcante3 ,
our patient population. Luisa Junqueira4 , Leticia Tufi4 , Beatriz Queiroz5 , Marco De Oliveira6
1 Hospital de Amor: Unidade Infanto Juvenil, Pediatric Surgery, Barretos,
Brazil; 2 Hospital de Amor, Pediatrics, Barretos, Brazil; 3 Hospital de Amor:
EP418 / #770 PILOT STUDY OF NON-ADHERENCE AND Unidade Infantojuvenil, Pediatric Radiology, Barretos, Brazil; 4 FACISB - Fac-
TREATMENT DROPOUT IN PEDIATRIC CANCER PATIENTS IN A uldade de Ciencias da Saúde de Barretos "Dr Paulo Prata", Barretos School
TERTIARY HOSPITAL IN A LOW- AND MIDDLE-INCOME Of Medicine, Barretos, Brazil; 5 Faculdade Barretos, Nutrition School, Bar-
COUNTRY retos, Brazil; 6 Hospital de Amor, Center Of Epidemiology And Biostatistics,
Barretos, Brazil
Carlos Pérez-Alvarado1 , Jose De Jesus Loeza1 , Diana Reyes1 , Claudia
Morales1 , Elizabeth Cortes1 , Ingrid Ortiz1 , Miriam Gonzalez1 , Zujey Background and Aims: Children with cancer are vulnerable to sar-
Guevara1 , Ismael Kelly-Perez2 , Maria Jose Vazquez1 , Betsabe copenia but defining the condition in this population is difficult due
Vázquez3 to the inconsistency in concepts and limited studies which assess the
1 Centro Estatal de Cancerología "Dr. Miguel Dorantes Mesa", Oncología potential impact of sarcopenia on clinical outcomes. However in the
Pediatrica, Xalapa, Mexico; 2 Universidad Veracruzana, Ingienería Mecan- last decade sarcopenia has been recognized as a negative impact of
inca, Xalapa, Mexico; 3 Universidad Veracruzana, Inteligencia Artificial, anticancer therapy in pediatric patients. This study assesses muscle
Xalapa, Mexico wasting using bedside ultrasound and anthropometry in hospitalized
children at pediatric oncology hospital.
Background and Aims: Background and Objectives: The survival rate Methods: Single-center, prospective cohort study, including consec-
for pediatric cancer in low- to middle-income countries (LMIC) can vary utive children, admitted to the pediatric oncology ward. Quadriceps
from 35 to 60%. Among the factors that may be responsible of this muscle thickness (QMT) and anthropometrics measurements were
are the lack of adherence and/or treatment dropout. In addition to performed at admission, day-1, day-3, day-5, day-7, and then weekly
limitations in the health care system, other causes of dropout and/or until the 14th day of the ward stay.
non-adherence to treatment in pediatric cancer that are directly Results: 56 children with cancer were evaluated at all time points
related to the patients. Among these factors are gender, refusal of of this study. 57% had hematologic malignancy, and 87% were hos-
certain treatments and distance to the treatment center. With the pur- pitalized with an active infection under treatment. According to the
pose of knowing which of these factors influence the lack of adherence Pairwise comparison method, longitudinally, no differences were found
and/or dropout in our patients, we decided to carry out this study. in the QMT from day-0 until day-7. However, between day-7 and day-
Methods: Cross-sectional study of a random sample of newly admit- 14, a distinction was found but no statistically significant (p-value 0.09).
ted patients treated between 2015-2019. Children were grouped into Arm anthropometry (Triceps skinfold thickness, mid-upper arm cir-
three groups: Patients without Dropout or Non-Adherence (PDA), cumference, and upper arm muscle area) presented similar results.
Patients with Non-Adherence (patients who were absent between 15 However, when considering the hospitalization between D1 and D5,
and 29 days from their treatment, PNA) and Patients with Dropout we did not find differences in quadriceps femoral thickness. This can
(PD). χ2 and one-way ANOVA were used. be considered by the nutritional support offered during this period and
Results: During the study period, 600 new cases were registered, of the recovery that occurs in D7. In contrast, the measurement reduction
which a sample of 318 patients was analyzed, 56.65% being boys. The in D14 may reflect the pathology, clinical severity, and/or intolerance to
68.87% were PDA, 26.42% PD and 4.72% PNA. Of all PNA, 53.3% nutritional support.
were boys and 26.7% were diagnosed with leukemia. Of all PD, 54.4% Conclusions: Compared to other body composition assessment tech-
were boys and 32.1% were diagnosed with leukemia. A difference with niques, using this tool in children with cancer reduces the use of other
p=0.051 was found between the three groups in terms of time from tests and avoids additional exposure to ionizing radiation. Therefore,
their house to the hospital in minutes, PDA: 122.12(+107.19), PNA: advancing our understanding of bedside ultrasound evaluation of QMT
113.27(+101.66) and PD: 156.77 (+128.35). in children with cancer may lead to low-cost clinical interventions, such
Conclusions: One factor that influences both non-adherence and treat- as optimizing protein intake following specific guidelines aimed at this
ment dropout in pediatric oncohematologic patients is the time to population.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S344 of S635 ABSTRACTS

EP420 / #1994 HAND-GRIP STRENGTH: A WAY TO 1 McGill University, School Of Physical And Occupational Therapy, Mon-
ENHANCE BODY COMPOSITION EVALUATION IN CHILDREN tréal, Canada; 2 Sainte-Justine University Health Center, Research Center,
WITH CANCER? Montreal, Canada

Wilson De Oliveira Jr1 , Mariana Murra2 , Maynara Da Silva3 , Marielle Background and Aims: Cancer during childhood causes various devel-
Ferreira3 , Maria Clara Rossi4 , Marise Firmino5 , Ricardo Da Costa6 opmental difficulties. Rehabilitation is widely accepted to offer the best
1 Hospital de Amor: Unidade Infanto Juvenil, Pediatric Surgery, Barretos, opportunity to optimize the neurodevelopmental outcome of children.
Brazil; 2 Hospital de Amor, Pediatrics, Barretos, Brazil; 3 FACULDADES BAR- However, the literature in pediatric rehabilitation oncology to guide
RETOS, NutriÇÃo, BARRETOS, Brazil; 4 FACISB - FACULDADE DE CIENCIAS clinical practice is scarce. The purpose of this study is to investigate the
DA SAUDE DR PAULO PRATA, Barretos School Of Medicine, BARRETOS, feasibility of conducting standardized developmental assessments and
Brazil; 5 FACISB - FACULDADE DE CIENCIAS DA SAUDE DR PAULO PRAT, to describe motor performance during and after treatment in children
Barretos Medical School, BARRETOS, Brazil; 6 FACISB - Faculdade de Cien- affected by cancer.
cias da Saúde de Barretos "Dr Paulo Prata", Barretos School Of Medicine, Methods: A longitudinal design was used with two time points; shortly
Barretos, Brazil after diagnosis and two years after diagnosis. Feasibility was eval-
uated by determining the percentage of eligible participants who
Background and Aims: Much has been discussed about the parame- were assessed at each time point. Motor performance was assessed
ters to identify malnutrition in pediatric cancer patients. Considering using the Peabody Developmental Motor Scales 2nd edition [PDMS-
the different social issues that guide decision making, anthropometry is 2] for children > three years old and the Movement ABC 2nd edition
an easily accessible, fast and low-cost tool. Hand grip strength, to eval- [M-ABC2] for children ≥ three years old. Motor performance was
uate the muscle function is well stablished in adult literature and has measured at the two time points and compared to age-specific norms.
been suggested as a promising parameter to evaluate the muscle com- Results: Twenty-five children (range 17 months - five years old at diag-
partment in children. This study aims to describe the use of Hand-grip nosis, 60% male, 88% Caucasian) with various cancer diagnosis (60%
strength in hospitalized pediatric oncology patients and the correlation leukemia) enrolled on a larger trial were eligible for our study. Shortly
of this technique between another anthropometric measurements. after diagnosis, 88% of children were assessed and 76% were able
Methods: A cross-sectional study with prospective data collection, to complete all subtests. Two years after diagnosis, 91% completed
which included patients over 1 year of age up to 18 years of age who the full-assessment. Motor performance decreased significantly from
were hospitalized in the inpatient unit of a developing country, diag- baseline to two years post-diagnosis (p ≤ 0.05) and children had results
nosed with childhood cancer within 3 months. Anthropometric and significantly lower than the norms at both time points (p ≤ 0.01). The
related signs and symptoms data were collected, and the patients were proportion of children with a result under the 15th percentile was 50%
evaluated and questioned about the signs and symptoms 48 hours after at both time points, indicating a high risk of motor impairments.
admission. Conclusions: Preschool children affected by cancer are at high-risk of
Results: 114 children with cancer, with an median age of 11 years developing motor impairments. Early rehabilitation services are there-
were included. Average strength observed was 14.4 kg (95% IC: fore essential to minimize long-term impacts on motor development.
12.5-16.4). When correlated to Z-scores of weight/age (PCC:0.016), This early management of motor development would help to avoid the
height/age (PCC:0.226), BMI/age (PCC:0.176), and triceps skinfold double punishment that these children suffer: cancer itself and the
(PCC:0.252) a very low Pearson correlation coefficient were found, associated reduction of motor development.
however muscle upper arm circunference was moderately correlated
(PCC:0.580, p<0.001) with Hang-grip strength. No difference were
found in Hand-grip strength between malnutrition and obesity groups. EP422 / #1433 EVALUATION OF ICPCN’S PALLIATIVE CARE
Conclusions: In adult-cancer population, Hand-grip strength is a good EDUCATION PROGRAMMES
tool to assess functionality, sarcopenia and cachexia both transversally
and longitudinally. Being able to apply this tool in children as part of Alexandra Daniels1 , Nasur Buyinza2 , Julia Downing2
nutritional monitoring can help in selected cases and older children, but 1 International Children’s Palliative Care Network, Education, Durban, South

it is still necessary to understand its real benefits in pediatric patients. Africa; 2 International Children’s Palliative Care Network, Education, Kam-
pala, Uganda

EP421 / #761 MOTOR PERFORMANCE ASSESSMENT IN Background and Aims: ICPCN’s mission is to achieve the best qual-
PRESCHOOL CHILDREN AFFECTED BY CANCER: A ity of life and care for children and young people with life-threatening
LONGITUDINAL STUDY or life-limiting conditions, their families, and carers worldwide. The
ICPCN’s overall strategic goal for education is to provide high-quality
Catherine Demers1,2 , Annie Brochu2 , Émilie Bertrand2 , Isabelle CPC education which meets an identified global need and to support
Bouchard2 , Caroline Meloche2 , Caroline Laverdière2 , Valerie Marcil2 , and empower the ICPCN network to train from their own locali-
Serge Sultan2 , Daniel Sinnett2 , Daniel Curnier2 ties. Since 2011, both online and face-to-face education and training
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S345 of S635

programmes have been offered and in keeping with the spirit of the EP424 / #658 TRANSLATION AND VALIDATION OF THE
above goal, an evaluation of the education programme needed to CHICHEWA PEDIATRIC PATIENT-REPORTED-OUTCOME-CTCAE
be done. To assess the impact of CPC courses and to shape future TOOL TO MEASURE TREATMENT-RELATED ADVERSE EVENTS
improvements in course content and presentation. AMONG ADOLESCENT AND YOUNG ADULT LYMPHOMA
Methods: An evaluation questionnaire was distributed via Survey PATIENTS IN MALAWI
Monkey to all 5165 participants who had been on the ICPCN educa-
tion programme - this included those who had enrolled on face to face April Evans1,2 , Alyssa Tilly3 , Yolanda Gondwe2 , Maria Chikasema2 ,
as well as the e-learning training programmes over a period of 10 years Agness Manda2 , Bryce Reeve4 , Katherine Westmoreland1,2
(2011 – 2021). 1 University of North Carolina, Pediatric Hematology Oncology, Chapel Hill,

Results: 612 (11.8%) participants responded (490 female, 122 male). United States of America; 2 UNC Project Malawi, Cancer Program, Lilongwe,
33.95% of respondents were aged 35 to 44 years, 24.8% 45 to 54 years Malawi; 3 University of North Carolina, Palliative Care, Chapel Hill, United
and 20.2% 25 to 34 years. Most respondents (40%) were from Europe, States of America; 4 Duke University School of Medicine, Population Health
followed by Sub -Saharan Africa (21.04%) and Asia (16.80%). Online Sciences, Durham, United States of America
courses scored higher than face-to-face sessions in terms of clarity
of information. In relation to usefulness to participants practice and Background and Aims: Internationally, patient-reported out-
overall course ratings face-to-face and online scores were similar. The come (PRO) tools to assess health-related quality of life (HRQoL)
majority of respondents (91%) reported an increase in their knowledge and adverse events (AE) are available, but efforts to translate
of CPC, skills improvement (88%), positive change in attitude (86%), and culturally validate such tools in sub-Saharan Africa (SSA) are
and a change in clinical practice (84%). limited.
Conclusions: The evaluation showed that there was a considerable Methods: The Pediatric PRO version of the Common Terminology Cri-
knowledge gain. The main outcome of both the online and face to teria for Adverse Events (Ped-PRO-CTCAE) includes a core 15 AE
face education initiatives was a positive impact in terms of developing symptoms. Each symptom has 2-3 questions, a 4-point Likert-type
further support services in CPC. scale, and a 7-day recall period. The Ped-PRO-CTCAE was trans-
lated into Chichewa and culturally validated for use in Malawi using
the FACIT translation guidance. Psychometric validation was assessed
EP423 / #14 THE EFFICACY OF NEUTROPENIC DIET IN using Spearman’s correlation for convergent and discriminant validity,
PREVENTING NEUTROPENIA RELATED INFECTIONS AMONG Wilcoxon rank-sum for known group validity, and T-test for responsive-
PEDIATRIC PATIENTS UNDERGOING CHEMOTHERAPY: A ness.
META-ANALYSIS Results: Fifty-Two adolescent and young adult (AYA) patients, with
lymphoma completed the Ped-PRO-CTCAE survey. When compared
Alexander Lloyd Ng, Amanda Christina Dujua to the translated and validated Patient-Reported Outcomes Mea-
Ospital Ng Makati, Pediatrics, Manila, Philippines surement Information System (PROMIS) Pediatric tool, there were
stronger correlations when symptoms matched (ie pain interference
Background and Aims: Neutropenic diet is still being used among can- r=0.57, fatigue r=0.73, depression r=0.62, and anxiety r=0.75). Known
cer patients despite the lack of evidence of its benefit. Several studies group validity testing showed that patients with poor performance
showed lack of effectiveness of this regimen. However, most of these status (ECOG≥2) had higher pain frequency (p<0.001) and pain preva-
studies were done among adult patients. This meta-analysis analyzes lence (p=0.005); and patients with anemia (hgb<9g/dL) had worse
the available data involving pediatric cancer patients who were placed fatigue severity (p<0.001). Ped-PRO-CTCAE when compared to home
on a neutropenic diet during chemotherapy. symptom diary (ie nausea, constipation, pain, mucositis, insomnia and
Methods: A systematic search for randomized controlled trials inves- abdominal pain) were correlated across all matching PRO-CTCAE
tigating the effect of neutropenic diet versus regular diet among domains (p<0.001). Responsiveness was supported when comparing
pediatric patients undergoing chemotherapy was done. Outcomes Ped-PRO-CTCAE scores at T0 and T1 (+5 to < 21 days from T0); T1
of interest were incidence of febrile neutropenia and bacteremia. A exhibited higher mean scores associated with expected worse symp-
fixed-effects meta-analysis was doe to pool the effect of intervention. toms after chemotherapy across all fifteen PRO-CTCAE symptom AEs
Results: A total of 269 patients were included. There was no statisti- (p<0.001).
cally significant difference in the incidence of febrile neutropenia (RR Conclusions: This study found supporting evidence for the valid-
1.16, 95% CI 0.78 to 1.71) and bacteremia (RR 1.57, 95% CI 0.62 to ity of the Chichewa-translated version of the Ped-PRO-CTCAE for
3.96) between the neutropenic diet and regular diet groups. Malawi. This emphasizes an urgent need to address symptomatic
Conclusions: This meta-analysis showed that there is no evidence sup- AEs experienced by children and AYA undergoing cancer treat-
porting the use of neutropenic diet in preventing febrile neutropenia ment in SSA using PRO instruments validated within the local
and bacteremia among pediatric patients undergoing chemotherapy. context.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S346 of S635 ABSTRACTS

EP425 / #71 THE ROLE OF CLINICAL PHARMACIST IN 1 Aga Khan University, Dept Of Surgery, karachi, Pakistan; 2 Aga Khan Uni-
PARENTERAL NUTRITION FOR PEDIATRIC CANCER PATIENTS versity Hospital, Department Of Oncology, Karachi, Pakistan; 3 Aga Khan
IN 57357 CHILDREN CANCER HOSPITAL EGYPT (CCHE) University, Dept Of Oncology, karachi, Pakistan

Nermeen Ezzat1 , Ahmed Wagdy1 , Mohamed Abdelmonem1 , Zeinab Background and Aims: Cancer therapy leads to oral complications
Badr2 , Aya Emad2 , Ahmed El-Zeiny3 , Mohamed Nagy1 , Gulsen Saleh2 , which when left untreated can affect the patient’s quality of life, lead to
Sherif Kamal1 serious systemic infections that can complicate cancer treatment. Early
1 Children Cancer Hospital 57357, Pharmacy, cairo, Egypt; 2 Children Can- comprehensive oral care measures by a trained dental hygienist can
cer Hospital 57357, Clinical Nutrition Department, cairo, Egypt; 3 57357 minimize the risk of these oral and associated systemic complications.
Children’s Cancer Hospital, Supply Chain, cairo, Egypt Methods: From January 2021 to February 2022, a dental hygienist vis-
ited patients in the pediatric oncology unit at the Aga Khan University
Background and Aims: Parenteral nutrition (PN) therapy is a high Hospital Karachi, Pakistan. All patients were examined for cancer ther-
alert medication, complex and critical therapy that requires nutri- apy related oral complications including mucositis based on the World
tion support pharmacist knowledge, skills, and practice experience to Health Organization (WHO) Mucositis scale. After initial screening,
avoid errors in prescribing, compounding, and clinical management of dental hygienist provided an oral care regimen. Interventions included
patients. Aims: Evaluate the role of clinical pharmacist to monitor PN a combination of mouthwashes (anesthetic, analgesic, anti-microbial)
therapy in 57357 CCHE to deliver it in safe and effective way. and oral gels with teaching on the prescribed oral care regimen.
Methods: A retrospective study over 12-month period conducted in Results: A total of 197 pediatric patients were examined by the den-
57357 (CCHE) from 2018 till 2019 . Interventions of clinical pharmacy tal hygienist. This included 126 (64%) males with the age range of 9
for order review were documented and divided into categories . The months to 20 years. Almost half of the patients had leukemia 93 (48%),
data that be collected were patient anthropometric measurements, PN followed by lymphomas 22 (12%), and sarcomas 16 (8%). Other diag-
indication, PN prescription, fluids and electrolytes calculation, moni- noses included Neuroblastoma 5 (2.5%), Medulloblastoma 7 (3.5%),
toring daily laboratory test, calculating caloric requirements, route of Thalassemia Major 8 (4%), Wilms’s tumor 4 (2%). Out of 197 patients
administration (central or peripheral line), calculating osmolarity, daily 99 (50.25%) had mucositis, Grade I in 17 (17%), Grade II - 50(51%),
oral intake and other medication-related problems. However, each of Grade III - 25 (25%), and Grade IV - 7 (7%) of patients . Grade II and IV
these practice helps to support the delivery of safe and effective PN mucositis were common in leukemia patients while Grade III was com-
therapy to patients. mon in sarcoma patients. Continuous monitoring, interventions, and
Results: The total number of pharmacist interventions were 2240 of oral hygiene education resulted in improved oral feeding, compliance,
PN-related problems. The total number of PN orders were 8892 pre- and early discharge.
scribed to 754 patients. The highest number of interventions were Conclusions: Early oral care measures provided by a dental hygienist
incomplete order element 792 (35.3%), low caloric intake 534 (23.8%), can significantly decrease complications, improve diet and compliance
drug -lab interaction 274 (12.2%), wrong volume 155 (6.9%), wrong with medications leading to early recovery. Further studies are needed
stopping date 114 (5%), optimize drug monitoring 83 (3.7%), wrong to measure the level of satisfaction and effectiveness of care in patients
transcription 79 (3.5%), dose calculation error 61 (2.7%), optimize receiving therapeutic care by a dental hygienist.
route 55 (2.5%), medication conversion 32 (1.4%),nurse error 23
(1%), medication not written on cerner 22 (0.98%) and therapeutic
duplication 16 (0.71%). EP427 / #1523 ASSOCIATION OF CVC AND NON-CVC
Conclusions: Clinical pharmacist role is an important pivot in provid- RELATED VTE WITH SURVIVAL IN PEDIATRIC ONCOLOGY
ing an accurate PN order and follow up of the nutritional status of PATIENTS
children suffering from cancer in 57357 CCHE. Many points for order
revision is a must for patient safety and avoid health hazards related Zara Forbrigger1,2 , Tamara Macdonald2 , Ketan Kulkarni1,2
to parenteral nutrition in the hospital. Inter-disciplinary collabora- 1 Dalhousie University, Pathology, Halifax, Canada; 2 IWK Health Cen-
tion between clinical nutrition department and the pharmacy provides tre, Division Of Hematology/oncology, Department Of Pediatrics, Halifax,
parenteral nutrition interventions in an ideal safe way. Canada

Background and Aims: Venous thromboembolism (VTE) has been


EP426 / #1320 INTEGRATION OF DENTAL HYGIENE CARE associated with inferior survival outcome in pediatric oncology
FOR PEDIATRIC ONCOLOGY PATIENTS, EXPERIENCE AT A patients. Little is known about how CVC-related and non-CVC-related
TERTIARY CARE HOSPITAL IN LMIC. A QUALITY CARE VTE differ and their corresponding impact on outcome.
IMPROVEMENT INITIATIVE Methods: This is a retrospective population-based study from Atlantic
Canada (Prince Edward Island, New Brunswick, Nova Scotia, and New-
Shazia Taimoor1 , Zehra Fadoo2 , Naureen Mushtaq3 , Sadaf Altaf3 , foundland). Patient demographics, diagnosis, and thrombosis infor-
Asim Belgaumi3 , Salima Alibhai1 mation was collected from 2000-2019. Patients were divided into a
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S347 of S635

CVC-related (CVC-VTE), non-CVC related VTE (N-VTE), and patients collected by parent-report, and health literacy was measured via bilin-
without a VTE (non-VTE) groups. Patients with both a CVC and gual interview using the Newest Vital SignTM screening tool. Scores
non-CVC related VTE were classified as N-VTE patients. Descriptive indicating low (0 -1), moderate (2 – 3) or high (4 – 6) literacy were calcu-
analysis was run on the population. Kaplan-Meier was run for survival lated; logistic regression measured associations between literacy and
analysis. ANOVA was performed for analysis of ITR-3 scores. sociodemographic factors.
Results: 1342 patients were included in the study. Of this, 91 patients Results: Thirty-five parents (100%) completed demographic surveys,
were diagnosed with a VTE and 57% (n=52) were associated with a 34 (97%) completed literacy screening. Participants were 34% His-
CVC. The M:F ratio for non-VTE patients was 54:47, 62:38 for N-VTE, panic, 17% non-Hispanic Black, 23% Spanish-speaking; 50% were
and 62:38 for CVC- VTE. The most common diagnosis for Non-VTE publicly insured, 17% had less-than high-school education. Mean lit-
and CVC-VTE was leukemias (30% and 50% respectively). The most eracy score was 3.26 (+/- 2.00); 26% scored 0-1 (low literacy). In
common diagnosis for N-VTE patients was sarcoma (33%). When com- univariate models, time from diagnosis was not associated with odds
paring treatment intensity through ITR-3 scores, the results were of low or high-literacy; parents who were unemployed, who preferred
significant across groups (p<0.001). Post-hoc tests showed that non- Spanish, and who did not complete high school however, had increased
VTE patients and CVC-VTE differed significantly (p=0.008). CVC-VTE odds of low literacy. Across all participants, 69% sought additional
and N-VTE patients as well as CVC-VTE and no-VTE patients were teaching about their child’s illness from providers as well as from
similar (p=0.956 and p=0.066 respectively). When looking at sur- supplemental information online.
vival curves, N-VTE had significantly inferior survival in comparison Conclusions: Over 25% of our pediatric oncology parents/caregivers
to CVC-VTE (p<0.001) and non-VTE (p<0.001) patients. CVC-VTE and are at risk of limited health literacy. Univariate analyses suggest
non-VTE patients were similar (p=0.496). associations between parent educational attainment, occupation, pri-
Conclusions: N-VTE patients were associated with inferior survival mary language and literacy level; ongoing analyses are measuring
outcome as compared CVC-VTE and non-VTE patients, suggestive that adjusted associations between sociodemographics and literacy, as well
disease related VTE may be prognostic of worse survival outcome. as between literacy and adverse cancer-related outcomes.
More research looking into the cause of decreased survival in non-CVC
related VTE patients is necessary.
EP429 / #1172 INTRATYMPANIC INJECTION OF
SUSTAINED-EXPOSURE DEXAMETHASONE THERMOSENSITIVE
EP428 / #631 COLLECTING SOCIAL DETERMINANTS OF GEL (OTO-104) FOR PREVENTION OF CISPLATIN-INDUCED
HEALTH DATA AND MEASURING HEALTH LITERACY IN HEARING LOSS IN CHILDREN IS FEASIBLE AND SAFE
PEDIATRIC ONCOLOGY CAREGIVERS: A CROSS-SECTIONAL
COHORT STUDY David Freyer1 , Debra Don2 , Etan Orgel1 , Robert Hayashi3 , Judith
Lieu4 , Jennifer Foster5 , Matthew Sitton6 , James Geller7 , Daniel
Alycia Foti1 , Yujing Yao2 , Zhezhen Jin2 , Justine Kahn3 Choo8 , Arun Rangaswami9 , Kay Chang10 , Brian Greffe11 , Kenny
1 NYP-Columbia University Irving Medical Center, Pediatric Hematology, Chan12 , Alice Lee13 , Eli Grunstein14 , Allison O’Neill15 , Reza Rahbar16 ,
Oncology And Stem Cell Transplant, New York, United States of America; Jeff Anderson17
2 Columbia University Mailman School of Public Health, Department Of Bio- 1 Children’s Hospital Los Angeles, Cancer And Blood Disease Institute,
statistics, New York, United States of America; 3 Columbia University Irving Los Angeles, United States of America; 2 Children’s Hospital Los Angeles,
Medical Center, Pediatric Hematology, Oncology And Stem Cell Transplant, Otorhinolaryngology, Los Angeles, United States of America; 3 Saint Louis
New York, United States of America Children’s Hospital, Pediatric Oncology, St. Louis, United States of Amer-
ica; 4 Saint Louis Children’s Hospital, Otorhinolaryngology, St. Louis, United
Background and Aims: Despite advances in childhood cancer out- States of America; 5 Texas Children’s Hospital, Department Of Pediatrics,
comes, disparities among socially vulnerable populations persist. Hematology-oncology, Houston, United States of America; 6 Texas Chil-
Associations between educational attainment, parent age, English dren’s Hospital, Otorhinolaryngology, Houston, United States of America;
proficiency and cancer outcomes suggest that factors impacting com- 7 Cincinnati Children’s Hospital Medical Center, Division Of Pediatric Oncol-

munication and comprehension may contribute. Health literacy is the ogy, Cincinnati, United States of America; 8 Cincinnati Children’s Hospital,
degree to which individuals can process and act on health information. Otorhinolaryngology, Cincinnati, United States of America; 9 University of
While reports from general pediatrics identify associations between California San Francisco, Pediatric Oncology, San Francisco, United States
low literacy and inferior outcomes, few studies have evaluated poten- of America; 10 Lucille Packard Children’s Hospital, Otorhinolaryngology, Palo
tial associations in pediatric oncology. We assessed health literacy Alto, United States of America; 11 Children’s Hospital of Colorado, Pediatric
among a diverse cohort of pediatric oncology parents, and assessed Oncology, Aurora, United States of America; 12 Children’s Hospital of Col-
associations with self-reported demographics. orado, Otorhinolaryngology, Aurora, United States of America; 13 Morgan

Methods: English or Spanish-speaking parents of children (1 – 18 Stanley Children’s Hospital, Pediatric Oncology, New York, United States of
years) receiving chemotherapy or stem cell transplantation partic- America; 14 Morgan Stanley Children’s Hospital, Otorhinolaryngology, New
ipated in a cross-sectional survey study. Sociodemographics were York, United States of America; 15 Dana-Farber Cancer Institute, Pediatric
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S348 of S635 ABSTRACTS

Oncology, Boston, United States of America; 16 Boston Children’s Hospi- (ON), based on weight-for-height or body-mass-index-for-age z-scores
tal, Otorhinolaryngology, Boston, United States of America; 17 Otonomy, (WHO classification) and mid upper-arm circumference, at diagnosis
Research, San Diego, United States of America and 3-months and 6-months follow-up. Trends in nutritional status
(NS), and impact of changes in NS on outcomes (event-free survival,
Background and Aims: This study’s purpose was to evaluate the EFS and overall survival, OS) were analyzed.
feasibility, safety, and efficacy trends of intratympanic sustained- Results: Data was available in 2,086 children at diagnosis, and 1,245
exposure dexamethasone thermosensitive gel (OTO-104) for prevent- at follow-up treated between January-2018 and December-2019,
ing cisplatin-induced hearing loss (CIHL). median age 6.5 years (IQR 3.3-11 years) with 68.6% boys, all diagnoses
Methods: This IRB-approved, randomized phase 2 trial was con- included. At diagnosis and follow-up, 643(30.8%) and 511(41%) were
ducted at 8 centers in the United States (January-September 2017). WN, 1,360(65.2%) and 663(53.3%) UN and 80(3.8%) and 71(5.7%)
Eligibility criteria were age 0.5-21 years; new diagnosis of neuroblas- ON. During the course of treatment 566(45.5%) gained weight,
toma, hepatoblastoma, osteosarcoma, or extracranial germ cell tumor; 145(11.6%) lost weight and 254(20.4%) improved NS. At median
planned cumulative cisplatin dose ≥ 200 mg/m2 ; normal baseline follow-up of 28 months, 1,445(69.2%) children were well, 390(18.7%)
hearing and otoscopy. Participant ears were randomized for intratym- relapsed/progressed, 173(8.3%) expired, and 73(3.3%) abandoned
panic injection of 0.2 ml OTO-104 6% in one and no treatment in treatment. At 12, 24 and 36 months, EFS was 82.5%, 72.6% and 63%
the other. OTO-104 was administered through a 25-27 gauge nee- and OS 96%, 86.5% and 76.7%. Weight gain during treatment was asso-
dle by an otolaryngologist during other procedural sedation within 72 ciated with improved EFS at 12, 24 and 36 months (96.5%, 86.6%,
hours before cisplatin cycles (including multiday regimens). Feasibil- 76.4%) vs no weight gain (91.1%, 80.3% and 67.7%, p<0.001). Simi-
ity was assessed by successful administration of intended OTO-104 larly, children with improved NS had higher EFS (96%, 86.5%, 76.7%)
doses. Adverse events (AEs) were monitored by physical examination, vs stable or deteriorated NS (92.9%, 82.3%, and 70.3%, p<0.001).
otoscopy, tympanometry, concurrent medications, and audiometry. Conclusions: This large single-center cohort confirms the positive
Results: Eighteen doses of OTO-104 were administered to 11 evalu- prognostic impact of weight gain and improved nutritional status in
able participants at 5 centers (median age 3 years, range 1-14; 6 children with cancer, with beneficial effects lasting for upto 3 years
female; 9 neuroblastoma, 2 osteosarcoma). Five participants received from diagnosis.
1 dose, five received 2 doses, and one received 3 doses. OTO-104 was
administered via intratympanic injection (9) or tympanostomy tubes
(2). Sixteen injections were done during other procedural sedation; EP431 / #478 MANAGEMENT AND OUTCOME OF
2 were done awake. The median interval between OTO-104 and cis- CHILDHOOD SUPERIOR CAVA SYNDROME WITH
platin was 14 hours (range 7-64). OTO-104 injection was successful MEDIASTINAL MALIGNANCY: ANALYSIS OF 42 CONSECUTIVE
in all 18 procedures. At end of study, clinically insignificant tympanic CASES IN A SINGLE CENTER
membrane scabs developed in 5 participants, but no pinna, exter-
nal canal, or middle-ear changes were noted. No tympanogram shifts Yijin Gao1 , Qiushi Yang2 , Yali Han1 , Jiaoyang Cai1
were noted. There were 5 otologic treatment-emergent AEs (2 tran- 1 Shanghai Children’s Medical Center, Department Of Hematology/oncology,

sient mild-moderate otalgia [related] and 1 hypoacusis [unrelated]) and Shanghai, China; 2 Shanghai Children’s Medical Center, Department Of
no related non-otologic treatment-emergent AEs. Sensorineural CIHL Intensive Care Unit, Shanghai, China
developed similarly in treated and untreated ears.
Conclusions: Intratympanic administration of OTO-104 to young chil- Background and Aims: To delineate clinical characteristics, diagnostic
dren is feasible and safe. Further investigation using this route for modalities and outcome of superior vena cava syndrome (SVCS) sec-
preventing CIHL is warranted. ondary to mediastinal malignancy in children through consecutive case
analysis.
Methods: Consecutive patients diagnosed with SVCS (ICD-10 I87.106)
EP430 / #1191 CHANGES IN NUTRITIONAL STATUS AND from 2015 to 2021 at Shanghai Children’s Medical Center (SCMC)
IMPACT ON OUTCOMES: A REPORT according to International Classification of Disease 10th Revision were
analyzed. Patient demographics, baseline characteristics, systemic
Rajul Gala, Shyam Srinivasan, Ram Mohan, Chetan Dhamne, Nirmalya work-up, imaging findings and pathological reports were reviewed
Roy Moulik, Gaurav Narula, Badira Cheriyalinkal Parambil, Akanksha through hospital electronic medical record system. Outcome were col-
Chichra, Girish Chinnaswamy, Maya Prasad lected from oncology center database. The data were censored on
Tata Memorial Centre, Paediatric Oncology, Mumbai, India 1st February 2022 for survival analysis.
Results: Consecutive 42 children (31 males and 11 females) with SVCS
Background and Aims: We analyzed the trajectories in nutritional secondary to newly diagnosed mediastinal malignancy were treated
status and impact on outcomes in children with cancer. at SCMC from 2015 to 2021. The median age was 8.5 years . T-cell
Methods: Children (<18 years) with cancer at our center were classi- lymphoblastic lymphoma (T-LBL) followed by T-cell acute lymphoblas-
fied as undernourished (UN), well nourished (WN) and overnourished tic leukemia (T-ALL) was the most frequent malignancy in this group
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S349 of S635

(25 cases, 59.5% and 7 cases, 16.7%, respectively). Pathological diag- reduction in anxiety or fear of end of life, we observed a greater num-
nosis was confirmed by bone marrow aspiration or thoracentesis in ber of interventions by the psychosocial professionals and also there
14, peripheral lymph node biopsy in 6, and mediastinal biopsy in 22. was an increase in the number of patients who died in the hospital ward
Twenty-seven patients (64.3%) had local anesthesia. Respiratory com- and a decrease in the number of patients who died in the intensive care
plications due to mediastinal mass developed in 3 of 25 patients (20%) unit.
who received general anesthesia. Of all 62 patients with T-LBL treated Conclusions: Pediatric palliative care requires special knowledge and
during the study time period in our hospital, the 3-year event free skills, the provision of PPC for children with cancer allows patients and
survival and overall survival of patients who presented with SVCS at their families to receive support that alleviates the physical, emotional
diagnosis (n=25) were significantly lower than that of those who had and social needs that arise from a life-limiting illness and finally receive
not (n=37) (41.4% vs 79.5% p<0.001 and 43.9% vs 87.5%, p=0.001; high-quality end-of-life care.
respectively).
Conclusions: T-LBL is the most common primary cause of SVCS in pedi-
atric patients with mediastinal mass. Immediate preoperative team EP433 / #1158 LEVERAGING A PALLIATIVE CARE
planning and multidisciplinary emergency management strategies are EDUCATIONAL SERIES TO CREATE A GLOBAL NETWORK
the fundamental for successful clinical management. Diagnosis should
be made in the least invasive manner after an initial stabilization. Ximena Garcia1 , Michael Mcneil1 , Marta Salek2 , Justin Baker3
1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis,

United States of America; 2 St. Jude Children’s Research Hospital, Depart-


EP432 / #195 AT THE END OF LIFE: PEDIATRIC PALLIATIVE ment Of Oncology, Memphis, United States of America; 3 St. Jude Children’s
CARE CAN MAKE A DIFFERENCE IN CARING FOR THE CHILD Research Hospital, Division Of Quality Of Life And Palliative Care, Memphis,
AND FAMILY United States of America

Maria Cuervo1,2 , Ximena Garcia3 , Karen Molina2,4 , Angelica Claros5 , Background and Aims: The mission of the St. Jude Global Palliative
Jhon Bolaños6 , Tatiana Alvarez6 , Daniela Cleves4 Care Transversal Program is to define the current state of palliative
1 Fundacion Valle de Lili, Palliative Care Department, Cali, Colombia; care (PC) in underserved communities worldwide and drive collab-
2 Universidad Icesi, Clinical Medical Sciences, Cali, Colombia; 3 St. Jude orative interventions with our global partners to relieve suffering
Children’s Research Hospital, Global Pediatric Medicine, Memphis, United of patients and families facing the challenges of pediatric cancer. A
States of America; 4 Fundacion Valle de Lili, Maternal And Child Depart- key feature of this mission is to improve educational offerings within
ment, Cali, Colombia; 5 Universidad de la Sabana, Clinical Medical Sciences, pediatric palliative care (PPC) for healthcare professionals providing
Chía, Colombia; 6 Fundacion Valle de Lili, Scientific Research Center, Cali, care for children with cancer. We aim to describe the creation of a
Colombia global PC network through a series of monthly educational meetings
where E-Poster Topics of interest in pediatric palliative oncology are
Background and Aims: WHO defines pediatric palliative care as the discussed.
active total care of the child’s body, mind and spirit, which also involves Methods: An expert panel selected PPC E-Poster Topics focused on
giving support to the family, the aim of this study is to describe the end- PC integration in childhood cancer that highlighted interdisciplinary
of-life care of children with cancer during the end-of-life period who and multicultural perspectives. The educational sessions were held
receive general palliative care or pediatric palliative care in low- and through an electronic platform with simultaneous interpretation avail-
middle-income countries. able. Recordings were available after completion of the session for
Methods: We conducted a retrospective quasi-experimental study viewing.
between January 2013 and December 2020 with deceased pediatric Results: The St. Jude Global Palliative Care Transversal Program held a
cancer patients. Demographic and clinical variables were collected to monthly series between December 2021 and March 2022. In each ses-
describe end-of-life care received before (2013–2017) by general pal- sion, renowned experts in PC from 12 different countries led enriching
liative care team and after (2018–2020) the creation of a pediatric educational sessions on E-Poster Topics such as quality improvement,
palliative care team research, psychosocial standards, and advocacy in PPC. An average of
Results: A total of 180 pediatric patients were evaluated at the end 212 participants from 99 countries across 5 continents joined 4 virtual
of life (100 between 2013–2017 and 80 between 2018–2020). The sessions. Simultaneous translation in Spanish and English was available
median age was 11 years, regardless of sex. Half of the patients had and the recordings have been viewed 436 times. These series led to the
a diagnosis of leukemia (49.8%), 52.7% receive palliative treatment creation of an educational PPC network that now has 1,280 members
for their oncological condition. Regarding symptoms, 72 hours before from every continent.
death, pain treatment was documented for 52.2% of the patients. Conclusions: The Global Educational Series in PC has been successful
Other signs and symptoms, such as dyspnea, seizures, agitation, and in sharing knowledge and as a strategy to create a global PPC net-
irritability, were present, with no differences between groups; how- work among healthcare professionals caring for children diagnosed
ever, it was noted that during PPC interventions, there was a significant with cancer. This educational series will also be used in the future to
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S350 of S635 ABSTRACTS

discuss complex clinical cases, improve patient care, and promote tice are now evidently crucial to determine if and how fibre/prebiotics
clinician resilience. should be used to support people undergoing cancer therapy, especially
children.

EP434 / #1120 THE EFFICACY OF FIBRE AND PREBIOTIC


INTERVENTIONS ON CLINICAL OUTCOMES IN CANCER AND EP435 / #1438 IDENTIFYING CHALLENGES IN DELIVERY OF
HAEMATOPOIETIC STEM CELL THERAPIES: SYSTEMATIC PEDIATRIC ONCO-CRITICAL CARE IN LATIN AMERICA
LITERATURE REVIEW
Srinithya Gillipelli1,2 , Firas Sakaan2 , Maria Puerto-Torres2 , Carlos
Breeana Gardiner1 , Hannah Wardill2 , Graeme O’Connor1 , Darren Acuña3 , Carolina Delgado4 , Daiane Ferreira5 , Maribel Ibarra6 , Eliana
Hargrave3 , Gary Frost4 , Aaron Lett4 Lopez-Baron7 , Murilo Luiz8 , Angelica Martinez9 , Alejandra
1 Great Ormond Street Hospital for Children, Dietetics, London, United Méndez-Aceituno10 , Erika Montalvo11 , Victor Sanchez-Torres12 ,
Kingdom; 2 School of Biomedicine, The University of Adelaide, Precision Daniel Sousa13 , Margarita Torres14 , Silvio Torres15 , Diana Valencia16 ,
Medicine Theme (cancer Program) South Australian Health And Medical Ivonne Villanueva17 , Janet Middlekauff2 , Andrew Pappas2 , Asya
Research Institute, Adelaide, Australia; 3 Great Ormond Street Hospital for Agulnik2,18 , Anita Arias18
Children, Oncology, London, United Kingdom; 4 Imperial College London, 1 Baylor College of Medicine, School Of Medicine, Houston, United States
Section For Nutrition Research Department Of Metabolism, Digestion And of America; 2 St. Jude Children’s Research Hospital, Department Of Global
Reproduction, London, United Kingdom Pediatric Medicine, Memphis, United States of America; 3 Hospital Dr. Luis
Calvo Mackenna, Pediatric Critical Care Unit, Santiago, Chile; 4 Hospital del
Background and Aims: Cancer treatments cause a range of toxicities Niño DIF Hidalgo, Pediatric Critical Care Unit, Hidalgo, Mexico; 5 Barretos
that impact treatment adherence and patient wellbeing. Dietary Children’s Hospital, Pediatric Critical Care Unit, Barretos, Brazil; 6 Centro
fibre/prebiotics characteristically improve the gastrointestinal- Medico Nacional Siglo XXI, Pediatric Critical Care Unit, México City, Mex-
microenvironment, which consequently benefit downstream effects ico; 7 Hospital Pablo Tobón Uribe, Pediatric Critical Care Unit, Medellín,
pertinent to treating and preventing treatment-related toxicities. This Colombia; 8 Hospital da Criança de Brasilia, Pediatric Critical Care Unit,
systematic review aimed to evaluate the clinical efficacy and side Brasilia, Brazil; 9 Hospital General de Tijuana, Pediatric Hemato-oncology
effects of fibre/prebiotic-based interventions on outcomes in children Unit, Tijuana, Mexico; 10 Unidad Nacional de Oncología Pediátrica (UNOP),
and adults being treated with cancer or undergoing haematopoietic Pediatric Critical Care, Guatemala City, Guatemala; 11 Hospital SOLCA, Crit-
stem cell therapies. ical Care Unit, Quito, Ecuador; 12 Instituto Mexicano del Seguro Social,
Methods: This study was conducted in adherence to PRISMA guide- Pediatric Critical Care Unit, Guadalajara, Mexico; 13 A.C. Camargo Cancer
lines, and the protocol was published prospectively with PROSPERO Center, Pediatric Critical Care Unit, São Paulo, Brazil; 14 Clínica Imbanaco
(CRD42022299428). Four databases (MEDLINE (Ovid), CINHAL, Grupo Quirón Salud, Pediatric Critical Care Unit, Cali, Colombia; 15 Hospital
EMBASE Cochrane CENTRAL) until February 2022 were searched. All Universitario Austral, Pediatric Intensive Care Unit, Buenos Aires, Argentina;
articles were assessed for bias using the Cochrane risk-of-bias tool RoB 16 IMAT Oncomédica, Pediatric Critical Care Unit, Montería, Colombia;
2.0 (for RCTs) and ROBINS-I (for non-RCTs). 17 Hospital Teletón de Oncología, Pediatric Critical Care Unit, Querétaro,
Results: A total 13449 articles were identified, of these, 13 (paedi- Mexico; 18 St. Jude Children’s Research Hospital, Division Of Pediatric
atrics [n= 1], adults [n=12]) met the inclusion criteria (randomised Critical Care, Memphis, United States of America
controlled trials (RCT) [n = 10], observational or non-RCTs [n = 3]). The
risk-of-bias was graded to be serious/high (n=3); moderate/some con- Background and Aims: Hospitalized pediatric hematology-oncology
cerns (n=7); low (n=3). Interventions included prebiotic supplement (PHO) patients are at high risk for critical illness, especially in resource-
(n=8), dietary modification (n=3) and nutrition supplement with added limited settings. PROACTIVE (PediatRic Oncology cApaCity Assess-
fibre/prebiotic (n=2) with prescribed fibre difference ranging from ment Tool for IntensiVe CarE) is a diagnostic tool designed to evaluate
2.4-30g/day. Substantial heterogeneity was identified across a range strengths and limitations in global pediatric onco-critical care (POCC)
of outcomes including gastrointestinal-side effects; gastrointestinal- services. In this study, we use PROACTIVE results to describe common
microbiome; clinical (including pathology); nutrition status and body challenges to POCC in Latin America.
composition; and quality-of-life. Methods: PROACTIVE is an English-language electronic assessment
Conclusions: The scientific rationale for fibre/prebiotics-based inter- tool developed from 119 consensus-derived quality and capacity
ventions for the prevention or management of cancer treatment- indicators arranged into 8 domains and divided into 2 surveys for
related toxicities is compelling. Despite this, our study highlights the intensivists and oncologists managing critically ill PHO patients.
paucity of relevant research, and of which, is highly heterogeneous and PROACTIVE was administered using REDCap between January 2021
is lacking in quality. This is especially pertinent for studies conducted and March 2022. Aggregated data from 15 hospitals in Latin Amer-
in the paediatric setting (n=1), which was exclusively the initial study ica were analyzed and indicators scoring ≤75% across centers
scope. This incomplete understanding is at the detriment of clinical were classified as common challenges in delivery of POCC in the
care. High-quality interventions studies translatable to clinical prac- region.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S351 of S635

Results: We analyzed aggregated data from 15 hospitals located in 8 51 (21.3%) and others 2 (0.8%). Average final dose was 1.2mg/kg
Latin American countries with a wide variety of resources, income- for Ketamine, and 0.13mg/kg for Midazolam. Adjunctive drugs used
levels, and POCC services. The Personnel domain was identified as were Atropine (20, 8.4%), supplementary Oxygen (10, 4.2%), and
presenting most common challenges with 15/18 indicators (83%) scor- Albuterol (8, 3.3%). Adverse effects included desaturation <94% (10,
ing ≤75% for availability or performance. Challenges in this domain 4.2%, Bradycardia (1, 0.4%), Emesis (4, 1.7%). 213 events (89.1%)
included: 1) inability to provide multidisciplinary care to critically ill were uneventful. Sedation was adequate (no need for restraint) in
PHO patients due to lack of trained nurses and specialists (oncologists, 229 events (95.8%). Perception of sedation, final Ketamine/ Midazo-
intensivists, neurosurgeons), 2) Limited training and access to educa- lam dose, need for adjunctive drugs and adverse effects did not differ
tional resources in pediatric critical care for nurses and healthcare significantly when analyzed for gender, procedure or age.
providers, 3) Insufficient team participation in quality improvement Conclusions: Sedation with ketamine and midazolam can be admin-
projects. Other limitations were found in Supportive Services (48%), istered safely for pediatric cancer patients at the oncology ward to
Service Capacity (44%) and Outcomes (40%) domains, while the Medi- facilitate minor procedures in settings where an anesthesiologist or
cation and Equipment domain (14%) was considered a relative strength alternate methods for deep sedation are unavailable.
having <20% of low-scoring indicators.
Conclusions: Training and lack of specialists are major limitations to
high-quality POCC in Latin America. Awareness of common challenges EP437 / #1752 INTERDISCIPLINARY DECISIONS AND
and improvement efforts focusing on the low-scoring indicators can COMMUNICATION PRACTICES: A SURVEY OF PEDIATRIC
help stakeholders tailor interventions to improve POCC services and ONCOLOGY CLINICIANS IN CENTRAL AMERICA AND THE
outcomes for PHO patients. CARIBBEAN

Dylan Graetz1 , Yichen Chen1 , Meenakshi Devidas1 , Federico


EP436 / #392 KETAMINE AND MIDAZOLAM FOR SEDATION Antillón-Klussmann2 , Ligia Fu3 , Karina Quintero4 , Soad Fuentes-Alabi
IN THE PEDIATRIC ONCOLOGY UNIT: SAFETY PROFILE IN De Aparicio5 , Pascale Gassant6 , Erica Kaye7 , Justin Baker8 , Carlos
THREE CENTERS IN LATIN AMERICA Rodriguez-Galindo1 , Jennifer Mack9
1 St. Jude Children’s Research Hospital, Department Of Global Pediatric
Pablo Gonzalez-Montalvo1,2 , Natalia Gonzalez3,4 , Esther Cocom-Hu1 , Medicine, Memphis, United States of America; 2 Unidad Nacional de
Maribel Perez-Lopez1 , Leslie Benitez-Can1 , Natalia Negroe-Ocampo1 Oncología Pediátrica, Oncología, Guatemala City, United States of America;
1 Hospital O’Horan, Servicios de Salud de Yucatan, Pediatric Oncology Unit, 3 Hospital Escuela Universitario, Pediatric Oncology, Tegucigalpa, Honduras;

Merida, Mexico; 2 Universidad Autonoma de Yucatan, School Of Medicine, 4 Hospital del Niño Dr Jose Renan Esquivel, Oncology, Panama, Panama;
Merida, Mexico; 3 Hospital de Niños Santisima Trinidad, Pediatric Oncol- 5 Hospital Nacional de Ninos Benjamin Bloom, Pediatric Oncology, San
ogy, Cordoba, Argentina; 4 Sanatorio Allende, Pediatric Oncology, Cordoba, Salvador, El Salvador; 6 Hôpital Saint - Damien, Pediatric Oncology, Port-au-
Argentina Prince, Haiti; 7 St. Jude Children’s Research Hospital, Division Of Quality Of
Life And Palliative Care, Department Of Oncology, Memphis, United States
Background and Aims: Pediatric cancer patients frequently undergo of America; 8 St. Jude Children’s Research Hospital, Division Of Quality Of
painful minor procedures, such as spinal tap/ intrathecal chemother- Life And Palliative Care, Memphis, United States of America; 9 Dana Farber
apy, bone marrow aspirate, bone marrow biopsy and central line Cancer Institute, Pediatric Hematology/oncology, Boston, United States of
placement. Although deep sedation is ideal for these procedures, this America
is not widely available, particularly in limited-resource settings, so dis-
sociative sedation is frequently used as an alternative. Our aim was Background and Aims: Interdisciplinary care is a core attribute of
to describe the safety profile of sedation with Ketamine and Midazo- high-quality cancer care and is associated with improved patient out-
lam when administered to pediatric cancer patients for painful minor comes. However, we know little about the role of interdisciplinary pedi-
procedures. atric cancer care in low- and middle-income countries. The purpose of
Methods: This was a consecutive case series of 239 events of sedation this study was to explore communication and decision-making among
on pediatric cancer patients in 2 hospitals over a 6-month period. Pro- interdisciplinary clinicians in Central America and the Caribbean.
tocol for sedation included ketamine and midazolam at a starting dose Methods: We conducted a cross-sectional survey of interdisciplinary
of 1mg/kg and 0.1mg/kg respectively. Records were reviewed for final clinicians at five pediatric hematology-oncology centers in Central
ketamine/ midazolam dosing, adverse effects, use of adjunctive drugs America and the Caribbean (Guatemala, Honduras, Panama, El Sal-
and perception of sedation. vador, Haiti). The survey included items adapted from previously vali-
Results: Our series included 209 events from Mexico, and 30 from dated tools investigating frequency of interdisciplinary communication
Argentina; 131 were male (54.8%), and 108 were female (45.2%). Pro- and interdisciplinary decision-making.
cedures included Spinal tap/ intrathecal chemotherapy 150 (62.7%), Results: 174 interdisciplinary team members completed the sur-
Bone marrow aspirate 14 (5.9%), Bone marrow biopsy 4 (1.7%), Cen- vey: nurses (n=60), medical subspecialists (n=35), oncologists (n=22),
tral line placement 18 (7.5%), Bone marrow aspirate + Spinal tap psychosocial providers (n=20), surgeons (n=12) pathologists (n=9),
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S352 of S635 ABSTRACTS

radiologists (n=9), radiation oncologists (n=5). Oncologists reported attendance at 93%, and adherence to wearing Fitbit 79%. There
daily communication with nurses (95%), other oncologists (91%), psy- were no serious adverse events. For limited efficacy, data indicated
chosocial providers (82%), and pharmacists (81%). While 90% of CanMOVE was associated with increased child/adolescent physical
surveyed nurses reported daily communication with other nurses, only activity. Positive impacts were seen in parent and staff behaviour
66% reported daily communication with oncologists and >50% of towards physical activity promotion. Pre/post physical function and
nurses reported never talking to pathologists, radiologists, radiation HRQOL assessments improved.
oncologists, or surgeons. Most clinicians described interdisciplinary Conclusions: CanMOVE is feasible and safe to implement in the pae-
discussion of cancer treatment goals and prognosis (84%), patient pref- diatric oncology setting and positively associated with changes in
erences (81%), and determination of first treatment modality (80%). physical activity behaviour of children/adolescents.
Providers who described more interdisciplinary care had higher job
satisfaction (p=0.04) and perceived a higher level of overall quality of
care (p=0.004). EP439 / #465 PREVALENCE, SEVERITY, TRAJECTORY, AND
Conclusions: Clinicians in Central America and the Caribbean describe PREDICTORS OF SYMPTOM BURDEN AMONG ADOLESCENTS
strong interdisciplinary collaboration contributing to higher job satis- AND YOUNG ADULTS WITH CANCER: A POPULATION-BASED
faction and perceived quality of care. However, nurses in these settings COHORT STUY
experienced more limited interdisciplinary communication and may be
left out of important team care. Additional studies are necessary to fur- Sumit Gupta1 , Qing Li2 , Paul Nathan1 , Norma D’Agostino3 , Nancy
ther define clinical roles on interdisciplinary care teams in LMICs as Baxter4 , Karine Chalifour5 , Natalie Coburn6 , Rinku Sutradhar2
well as their association with patient outcomes. 1 Hospital for Sick Childrenn, Haematology/oncology, Toronto, Canada;
2 Institute for Clinical Evaluative Sciences, Cancer Research Program,
Toronto, Canada; 3 Princess Margaret Cancer Centre, Department Of Sup-
EP438 / #430 PROMOTING POSITIVE PHYSICAL ACTIVITY portive Care, Toronto, Canada; 4 University of Melbourne, Melbourne School
BEHAVIOURS IN CHILDREN AND ADOLESCENTS UNDERGOING Of Population And Global Health, Melbourne, Australia; 5 9Young Adult
ACUTE CANCER TREATMENT: FEASIBILITY OF THE CANMOVE Cancer Canada, N/a, St. John’s, Canada; 6 Sunnybrook Health Sciences
INTERVENTION Centre, Department Of Surgery, Toronto, Canada

Sarah Grimshaw1,2 , Nicholas Taylor2 , Rachel Conyers1 , Nora Shields2 Background and Aims: Adolescents and young adults (AYA) with
1 Murdoch Children’s Research Institute, Cancer, Melbourne, Australia; 2 La cancer are a population at risk of experiencing outcome disparities.
Trobe University, School Of Allied Health, Human Services And Sport, Symptom burdens in this population are not well characterized but are
Melbourne, Australia a significant contributor to poor quality of life.
Methods: All Ontario, Canada AYA aged 15-29 years at diagno-
Background and Aims: Supporting children/adolescents with can- sis between 2010-2018 were linked to population-based healthcare
cer to be more physically active has the potential to improve short- databases. Symptom burden was determined through linkage to
and long-term health outcomes. The objective of this study was to Edmonton Symptom Assessment System-Revised (ESAS) scores, an
assess the feasibility of CanMOVE, a complex, theoretically-informed, 11-point scale routinely obtained at the time of cancer-related out-
behaviour change intervention to promote participation in physical patient visits and collected provincially. Multistate models estimated
activity for children/adolescents undergoing acute cancer treatment. mean duration of severity states [none (0) vs. mild (1 vs. 2 vs. 3) vs.
Methods: A feasibility study using single-group, repeated measures, moderate (4-6) vs. severe (7-9)], trajectories, and associations with
mixed methods design was completed. Participants completed the 10- death. Patient, disease, and treatment-related variables associated
week CanMOVE intervention, which involved structured assessment with severe symptoms were also determined.
and support from a physiotherapist, and provision of a Fitbit (child Results: 4,296 AYA with >=1 ESAS score within a year of cancer
and parent) to achieve a daily steps goal. Feasibility domains demand, diagnosis were included (median age 25 years). The most prevalent
acceptability, implementation, practicality, limited efficacy, and inte- moderate/severe symptoms were fatigue and anxiety (59% and 44%
gration were evaluated. Objective assessment of physical activity, of AYA). Across symptom type, AYA reporting moderate symptoms
physical function, and health-related quality of life (HRQOL) were com- were more likely to subsequently experience improvement vs. worsen-
pleted. Qualitative data were collected via semi-structured interviews ing. Risk of death within 6 months increased with increasing symptom
with participants (parents and children/adolescents) and focus groups burden and was highest in AYA with severe dyspnea (9.0%), pain
with clinical staff. (8.0%), or drowsiness (7.5%). AYA living in the poorest urban neigh-
Results: Twenty families completed CanMOVE, including chil- borhoods were more likely to experience severe symptoms than in the
dren/adolescents (median age 12yrs, range 5-16) with a mix of cancer wealthiest areas, with twice the odds of reporting severe depression
diagnoses. There was high demand for CanMOVE with 95% enrolment [adjusted odds ratio (OR) 1.95, 95th confidence interval (95CI) 1.37-
rate. CanMOVE was acceptable for participants, families and staff. 2.78], pain (OR 1.94, 95CI 1.39-2.70), and dyspnea (OR 1.96, 95CI
All implementation feasibility thresholds were met, with intervention 1.27-3.02).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S353 of S635

Conclusions: AYA with cancer experience substantial symptom burden. EP441 / #1803 COST ANALYSIS OF INCREASED USE OF
Risk of death increased with symptom severity. Interventions target- PEG-GCSF PRIMARY PROPHYLAXIS DURING THE COVID-19
ing cancer fatigue and anxiety, and targeting AYA in lower-income PANDEMIC
neighborhoods, are likely to improve quality of life in this population.
Jessica Hayes1 , Claire Fosbrook2 , Helen Blundell2 , Laura Bengree3 ,
Jessica Bate4
EP440 / #280 ETHNIC-SPECIFIC PREDICTORS OF 1 Southampton Children’s Hospital, Piam Brown Ward, SO YD, United King-

NEUROTOXICITY AMONG PEDIATRIC ACUTE LYMPHOBLASTIC dom; 2 NHS Trust, Piam Brown Ward, SO YD, United Kingdom; 3 University
LEUKEMIA (ALL) PATIENTS FOLLOWING HIGH-DOSE Hospital Southampton NHS Foundation Trust, Piam Brown Ward, Wey-
METHOTREXATE (HD-MTX): A REDUCING ETHNIC DISPARITIES mouth, United Kingdom; 4 University Hospital Southampton NHS Founda-
IN ACUTE LEUKEMIA (REDIAL) CONSORTIUM REPORT tion Trust, Paediatric Oncology, Southampton, United Kingdom

Rachel Harris, Mark Zobeck, Melanie Bernhardt, Karen Rabin, Eric Background and Aims: During the pandemic, UK guidance recom-
Schafer, Heidi Hsiao, Olga Taylor, Philip Lupo, Michael Scheurer, Austin mended prophylactic granulocyte-colony stimulating factor (G-CSF)
Brown or biosimilar pegylated G-CSF to reduce hospital admissions with
Texas Children’s Hospital / Baylor College of Medicine, Pediatrics - Oncology, febrile neutropenia (FN). PEG-GCSF offers the advantage of stat
Houston, United States of America dosing over daily G-CSF injections thereby reducing potential virus
exposure by avoiding home visits by community nurses. This project
Background and Aims: HD-MTX (≥1 g/m2 ) is an important component aimed to review GCSF prescribing patterns, cost analysis and impact
of curative therapy in many treatment regimens for high-risk pediatric of PEG-GCSF on hospital admissions with febrile neutropenia.
ALL. MTX therapy can result in dose-limiting neurotoxicity. There is Methods: A drug spend audit compared annual spend of daily GCSF
growing evidence that neurotoxicity disproportionately affects Latino and PEG-GCSF between September 2019-2020 and September 2020-
children. Thus, we evaluated risk factors for neurotoxicity following 2021 at Southampton Children’s Hospital, UK. The average number of
HD-MTX in an ethnically diverse population of patients with ALL. patients on active treatment over a one-year period is 126. Dispensing
Methods: We evaluated patients aged 0-21 years diagnosed with de records were reviewed for 6 months from May 2021. Chemotherapy
novo ALL from 2010-2017 in the REDIAL Consortium. Time to MTX cycle dates were interrogated for delays caused by low counts.
clearance, serum creatinine elevations from baseline, supportive care, Results: Annual spend on daily GCSF between September 2019-2020
and sociodemographic factors were abstracted from medical records. was £8,206 and between September 2020-2021 £5,594 (32% reduc-
MTX neurotoxicity was defined as a neurologic episode (e.g., seizures tion). The pre-pandemic annual spend over the same time period for
or stroke-like symptoms) occurring within 21 days of HD-MTX and PEG-GCSF was £27,087 and this increased to £44,160 (63% increase).
resulting in subsequent MTX treatment modifications. Mixed effects Within the review period, 18 patients received PEG-GCSF prophylaxis
multivariable logistic regression was used to estimate the odds ratios and no patients were discharged with prophylactic daily GCSF. Of the
(OR) and corresponding 95% confidence intervals (CI) for the asso- eleven patients (61%) previously ineligible to receive PEG-GCSF, 36%
ciation between clinical factors and neurotoxicity, both overall and of chemotherapy cycles were delayed (average 5.2 days).
stratifying on ethnicity. Conclusions: Our results show a significant increase in annual spend
Results: Overall, 417 patients (54.8% Latino) with 1,560 HD-MTX infu- of £14,460 in GCSF prophylaxis from pre-pandemic times. This may
sions were evaluated. Thirty-six patients experienced neurotoxicity offset the bed days cost saving for fewer hospital admissions for FN.
(8.6%), 72% of whom were Latino. Neurotoxicity odds increased with We plan to ascertain the impact of primary PEG-GCSF prophylaxis
older age at diagnosis (OR = 1.29, 95% CI: 1.12-1.49) and follow- use on hospital admissions for FN and evaluate patient and parent
ing a creatinine elevation of ≥50%, compared to those with normal preference regarding GCSF choice. More directive GCSF prescribing
creatinine (i.e., <25% elevation) (OR = 3.82, 95% CI: 1.01-14.38). Pre- guidance should include restricting primary prophylaxis to more inten-
dictors of neurotoxicity differed by ethnicity. In non-Latinos, longer sive chemotherapy regimens and those at higher risk of FN admissions.
time to MTX clearance was the only clinical factor associated with It will be important to evaluate whether other centres have adopted
neurotoxicity (OR = 1.03, 95% CI: 1.01-1.05). Among Latinos, time to different GCSF prescribing practice since the pandemic and impact on
MTX clearance was not associated with neurotoxicity, while creatinine spend.
elevations ≥50% were associated with a five-fold increase in neuro-
toxicity odds (OR = 5.29, 95% CI: 1.41-19.90), compared to normal
creatinine. EP442 / #298 A CROSS SECTIONAL SURVEY TO EXPLORE
Conclusions: Serum creatinine increase ≥50% may be asso- BARRIERS AND FACILITATORS TO IMPLEMENTATION OF
ciated with an increased risk for neurotoxicity, specifically PHOTOBIOMODULATION FOR MUCOSITIS MANAGEMENT IN
among Latino children with ALL, and thus may iden- CHILDREN RECEIVING CHEMOTHERAPY IN THE UNITED
tify candidates for therapeutic and/or supportive care KINGDOM
interventions.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S354 of S635 ABSTRACTS

Claudia Heggie1 , Kara Gray-Burrows1 , Bob Phillips2 , Peter Day1 Background and Aims: Cancer patients constitute an important group
1 University of Leeds, Paediatric Dentistry, d, United Kingdom; 2 University of in pediatric palliative care (PPC). Amongst the aims of PPC is to reduce
York, Paediatric Oncology, d, United Kingdom the practice of futile interventions. Comparative data based on the
provision of PPC is scarce.
Background and Aims: Oral mucositis affects up to 80% of children Methods: Retrospective analytical study based on the clinical records
and young people (CYP) receiving chemotherapy. This can result in pain of deceased cancer patients from at a tertiary hospital in a 10-year-
and reduced oral intake. Hospitalisation may be required for parental period (2010-2019). Patients were classified considering if they were
nutrition and pain relief which may result in delays to scheduled cancer attended or not by the hospital PPC Unit (PPCU). We compared base-
treatment. Photobiomodulation therapy is recommended by NICE and line characteristics and support and invasive measures applied in the
other international bodies to prevent and treat mucositis. This cross last month of life (LMoL).
sectional survey aimed to explore existing paediatric photobiomod- Results: Of 198 patients, 99 (50.0%) were attended by the PPCU. No
ulation practices in the United Kingdom (UK), and the barriers and differences were found regarding sex, age at death, disease duration or
facilitators to implementation of this recommended, evidence-based lines of treatment. Patients with hematological malignancies were less
therapy. prone to receive attention by the PPCU (21.6%) than patients with CNS
Methods: Ethical approval was granted by the University of Leeds. cancer (75.0%) or solid tumors (71.0%; p<0.01). As for interventions
An online mixed-methods survey was administered to representa- performed in the LMoL patients attended by the PPCU were signif-
tives from all UK Children’s Cancer and Leukaemia Group (CCLG) icantly less prone to receive surgery (15.1% vs 4.0%), red blood cell
centres between October 2021-March 2022. Quantitative data under- (77.8% vs 33.3%) and platelet transfusions (74.8% vs 29.3%), invasive
went descriptive statistics. Qualitative data relating to barriers and procedures (58.6% vs 7.1%), palliative sedation (57.6% vs 17.2%) and
facilitators was analysed by two researchers (CH & KG-B) using the died significantly more at home (0% vs 65.7%). Patients attended by
Theoretical Domains Framework (TDF). the PPCU stayed a mean of 16.8 days less at hospital (CI: 13.9-18.7),
Results: A 100% (n=20) response rate was achieved. Two units 8.6 days less in the ICU (CI: 6.1-11.3) and of 4.4 days less in hospital in
in Scotland were delivering photobiomodulation for CYP receiv- their last week of life (CI: 3.5-5.2).
ing chemotherapy prior to haemopoietic stem cell transplant, for Conclusions: Though adjustment for possible confound-
osteosarcoma, non-Hodgkin’s lymphoma, and those with previous ing variables should be made our data supports that PPC
or established mucositis. Four units had plans to implement a ser- provision leads to a decrease in medical procedures in the
vice. In TDF analysis, five domains were most frequently populated: LMoL.
knowledge, skills, environmental context and resources, social influ-
ences and professional roles of Paediatric Dentists and Paediatric
Oncologists. EP444 / #613 NEUROPATHIC PAIN IN PATIENTS WITH
Conclusions: Despite being a recommended supportive care ther- CANCER ATTENDED BY A PEDIATRIC PALLIATIVE CARE UNIT
apy, photobiomodulation is only available in two CCLG units. Lack of
knowledge and skills, and environmental context and resources were Iñigo De Noriega1 , Blanca Herrero Velasco2 , Raquel Jimenez3 ,
identified as barriers. Collaboration with paediatric dental services Ricardo Martino4
was identified as a facilitator in implementation. National networks 1 CS Mejorada, Pediatric Unit, Madrid, Spain; 2 HOSPITAL INFANTIL UNI-
of Paediatric Dentists and Oncologists could be established to stan- VERSITARIO DEL NIÑO JESUS, Oncology, MADRID, Spain; 3 HOSPITAL
dardise protocols and training. Additionally, these networks would INFANTIL UNIVERSITARIO DEL NIÑO JESUS, Pediatric Unit, MADRID,
promote collaboration to address identified barriers and support wider Spain; 4 HOSPITAL INFANTIL UNIVERSITARIO DEL NIÑO JESUS, Pediatric
implementation of this supportive care therapy. Palliative Care Unit, Madrid, Spain

Background and Aims: Neuropathic pain (NP) supposes a problem


EP443 / #596 COMPARISON OF CARE IN THE LAST MONTH for pediatric patients in palliative care being its management often
OF LIFE OF PEDIATRIC CANCER PATIENTS BASED ON THE complex. We aim to describe the prevalence, characteristics and treat-
PROVISION OF PEDIATRIC PALLIATIVE CARE ments used for the management of neuropathic pain in a cohort of
pediatric patients with cancer attended by a pediatric palliative care
Iñigo De Noriega1 , Blanca Herrero Velasco2 , Raquel Jimenez3 , unit (PPCU).
Ricardo Martino4 Methods: Retrospective review of clinical records of patients with
1 CS Mejorada del Campo, Pediatric Unit, Madrid, Spain; 2 HOSPITAL cancer attended by a PPCU in a 10-year period (2010-2019). We col-
INFANTIL UNIVERSITARIO DEL NIÑO JESUS, Oncology, MADRID, Spain; lected general epidemiological characteristics, presence and pattern
3 HOSPITAL INFANTIL UNIVERSITARIO DEL NIÑO JESUS, Pediatric Unit, of NP and the pharmacological treatments used for its management.
MADRID, Spain; 4 HOSPITAL INFANTIL UNIVERSITARIO DEL NIÑO JESUS, We compared baseline characteristics using classic parametrical tests
Pediatric Palliative Care Unit, Madrid, Spain and calculated 95% confidence intervals (CI) for the estimation of
parameters. Statiscal significance was established at p<0.05.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S355 of S635

Results: The records of 171 patients were analyzed with 50.7% were implicated were pain management (68.3%; CI: 60.5-76.2), respi-
presenting NP. Patients were followed for a median of 1.6 months ratory distress (64.0%; CI: 56.0-72.1), skin problems (46.0%; CI: 37.7-
(interquartile range 0.7-4.7). No differences were found regarding 54.4), bleeding (21.6%; CI: 14.7-28.5) and seizures (17.3%; CI: 10.9-
sex or age at the first consult with the PPCU. NP was significantly 23.6). In 80.6% of the cases, nurses participated in the postmortem care
more frequent in patients with solid cancer (68.1%) than in those with of the patient’s body (CI: 73.9-87.2).
CNS cancer (44.6%) and in this group from those with hematological Conclusions: Nurses participated in different aspects of care regarding
malignancies (15.4%). The most frequent treatments used for manag- the use of medical devices and symptom management, as well as pro-
ing NP were gabapentinoids (84.6%; CI: 76.4-92.8). Other treatments viding health education and postmortem care. These aspects should be
included parenteral ketamine (28.2%; CI: 18.0-38.4), tricyclic antide- correctly tackled in their training curriculum.
pressants (12.8%; CI:5.2-20.4), methadone (11.7%; CI:4.3-19.0), nerve
blockage (7.7% CI:1.7-13.7) transdermic 5% lidocaine (3.9%; CI:0-8.2)
and transcutaneous electrical nerve stimulation (1.3%; CI:0-3.8). In 5 EP446 / #906 HEMODIALYSIS IN METHOTREXATE
patients (6.4%; CI:0.8-11.9) NP was considered refractory indicating INTOXICATION
palliative sedation.
Conclusions: In our cohort, NP was present in half of the patients, Miguel Angel Palomo Colli1 , Ramses Michel Zuñiga Casillas1 , Jose
being especially frequent in patients with solid tumors. Further stud- Carlos Romo Vazquez2 , Maria Fernanda Hidalgo Martinez1 , Luis
ies regarding the best therapeutical strategies are needed to study the Enrique Juarez Villegas1 , Monica Mier Cabrera1 , Marco Murillo
best approach for these patients. Maldonado1
1 Hospital Infantil de México Federico Gómez, Oncology, Ciudad de México,

Mexico; 2 Hospital Infantil de México Federico Gónez, Nefrology, Ciudad de


EP445 / #615 NURSING INTERVENTIONS IN DECEASED México, Mexico
PEDIATRIC PATIENTS WITH CANCER
Background and Aims: High-dose methotrexate(HDMTX) induced
Iñigo De Noriega1 , Blanca Herrero Velasco2 , Lourdes Chocarro3 , acute kidney injury is a rare but life-threatening complication.
Raquel Jimenez4 , Ricardo Martino3 Methotrexate is a highly nephro- and hepatotoxic drug used in
1 CS Mejorada, Pediatric Unit, Madrid, Spain; 2 HOSPITAL INFANTIL UNI- cancer protocols, in children and adults. High dose methotrexate
VERSITARIO DEL NIÑO JESUS, Oncology, MADRID, Spain; 3 HOSPITAL therapy may lead to kidney injury and decrease of methotrex-
INFANTIL UNIVERSITARIO DEL NIÑO JESUS, Pediatric Palliative Care Unit, ate clearance, followed by an increase of its serum concentration.
Madrid, Spain; 4 HOSPITAL INFANTIL UNIVERSITARIO DEL NIÑO JESUS, As a result, systemic intoxication may develop. Prophylaxis based
Pediatric Unit, MADRID, Spain on intensive fluid therapy and urine alkalization may not be suffi-
cient to prevent the formation of methotrexate crystals in kidney
Background and Aims: Patients with cancer in pediatric palliative care tubules. The aim of the study was to present 12 cases with toxic
(PPC) present complex needs of care. As a part of the interdisciplinary methotrexate levels treated with hemodialysis at the Hospital Infan-
team nurses participate in several aspects of care but little is known of til de México Federico Goméz between January 2015– December
the interventions that they apply. 2020
Methods: Retrospective review of clinical records of deceased Methods: Clinical data and outcomes of all patients who received
patients with cancer attended by a PPC unit (PPCU) in a 10-year hemodialysis after HDMTX administration were reviewed.
period (2010-2019). We collected general epidemiological charac- Results: Of 737 patients who received HDMTX, 12(1.6%) patients
teristics and nursing interventions (health education, use of medi- received hemodialysis because acute kidney injury and delayed
cal devices, symptom management and postmortem care). For each methotrexate clearance. Six were males, median age was 12.2(SD 3),
intervention we calculated the estimated 95% confidence interval Ten patients had osteosarcoma and 2 acute lymphoblastic leukemia,
(CI). the doses of MTX were 12-10 grm2sc and 5 grm2sc respectively.
Results: The records of 159 patients were analyzed, 58.5% being male At the time of toxicity (hour 24), the median plasma methotrex-
a median age of 9.4 years (interquartile range-IQR: 5.8-14.1) at the ate concentration was 173.7 μM(SD 73.2) and 3.32 (3.9) at hour
first consultation and a median follow-up of 1.4 months (IQR: 0.5- 120. The median peak serum creatinine level during these HDMTX
2.8). Considering the type of cancer, 44.0% had a solid tumor, 37.1% courses was 1.86mg/dL(SD 0.75 mg/dL). Despite intensive fluid ther-
CNS cancer and 18.9 hematological cancer; 61.6% of the patients died apy, urine alkalization and administration of high doses of folinic
at their home. Nursing interventions were registered in 87.4% of the acid (leucovorin)were indicated, methotrexate serum concentration
patients (CI: 82.2-92.6). The most common intervention was providing remained toxic. Reduction of methotrexate concentration (<1.0 μM)
health education (85.6%; CI: 79.7-91.5). Nurses managed respiratory was achieved after 5 events of hemodialysis in 4 patient, 3 events in
devices (69.1%; CI: 61.3-76.8), parenteral devices (68.3%; CI: 60.5- 1, 2 events in 4 and 3 required 2 hemodialysis. Renal function eventu-
76.2), nutritional support devices (36.0%; CI: 27.9-44.0) and urinary ally returned to baseline in all patients, and no patient died because of
catheters (18.7%; CI: 12.1-25.3). The main symptoms in which nurses methotrexate toxicity.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S356 of S635 ABSTRACTS

Conclusions: Hemodialysis is an effective supportive method in EP448 / #992 INTERIM ANALYSIS RESULTS OF SAFETY AND
methotrexate elimination in patients with severe intoxication. We use EFFICACY OF PEGYLATED RECOMBINANT HUMAN
this strategy because Carboxypeptidase is not available in Mexico. GRANULOCYTE COLONY STIMULATING FACTOR(PEG-RHG-CSF)
IN PREVENTING NEUTROPENIA IN CHILDREN WITH TUMOR
AFTER CHEMOTHERAPY
EP447 / #204 RESEARCH INTEREST OF GLOBAL INFECTIOUS
DISEASE TRAINING PARTICIPANTS AND OPPORTUNITIES FOR Feifei Sun1 , Suying Lu1 , Juan Wang1 , Zijun Zhen1 , Spring Chou2 , Lian
BUILDING AND STRENGTHENING RESEARCH CAPACITY IN Jiang3 , Xiuli Zhu3 , Jian Chen3 , Jia Zhu1 , Junting Huang1
LOW- TO MIDDLE-INCOME COUNTRIES 1 Sun yat-sen University Cancer Center, Pediatric Oncology, Guangzhou,
China; 2 SJD Barcelona Children’s Hospital, Operation Director, Apac,
Maysam Homsi1 , Milka Vázquez1 , Linh Pham1 , Sheena Mukkada1,2 , esplugues de llobregat, Spain; 3 Hebei Tumor Hospital, Pediatric, Shiji-
Victor Santana1,3 , Meenakshi Devidas1 , Miguela Caniza1,2,4 azhuang, China
1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis,

United States of America; 2 St. Jude Children’s Research Hospital, Infectious Background and Aims: Data on the use of PEG-rhG-CSF in chil-
Diseases, Memphis, United States of America; 3 St. Jude Children’s Research dren is limited. Therefore, the aims of the study were to investigate
Hospital, Oncology, Memphis, United States of America; 4 University of the safety and efficacy of PEG-rhG-CSF in children with tumor after
Tennessee Health Sciences Center, Pediatrics, Memphis, United States of chemotherapy.
America Methods: Pediatric patients with newly diagnosed malignant tumor
who received chemotherapy and expected to have grade III/IV neu-
Background and Aims: Building research capacity for healthcare pro- tropenia were enrolled. PEG-rhG-CSF (100ug/kg, with maximum 6mg)
fessionals (HCPs) in low- and middle-income countries (LMICs) is was injected subcutaneously once 24 to 48 hours after chemother-
essential to generating local evidence for infection care and preven- apy of first two courses. We evaluated the efficacy and safety of
tion (ICP) interventions to improve outcomes. In 2017 we launched an PEG-rhG-CSF in these patients.
annual Global Infectious Diseases Training Seminar to give HCPs the Results: A total of 166 pediatric patients were screened, of which 2
education and resources to develop as ICP leaders. Here, we share the failed to meet the inclusion criteria, 4 cases were excluded, a total of
outcomes of trainees’ project proposals. 160 patients who completed the study treatment were included in the
Methods: HCPs were trained to develop a project proposal, statistics. Including 58 sarcoma, 33 neuroblastoma, 21 lymphoma, 19
guided through the development of a research question, back- germ cell tumor, 15 brain tumor and 14 other tumor. The median age
ground/relevance, aims and objectives, methods, and references. We of the 160 patients was 6.22 years. In the safety evaluation, among
conducted a qualitative document analysis from 4 cohorts’ proposals the common adverse reactions related to the study drug, the incidence
(2018, 2019, 2020, 2022) to describe common themes. of bone pain, fatigue, muscle soreness, pain at the injection site was
Results: We reviewed 153 proposals from 174 participants repre- 20.0%, 13.13%, 13.13%, 12.50%. Anemia, decreased neutrophil count,
senting 50 countries from all 6 World Health Organization regions. decreased white blood cell count, and decreased lymphocyte count
Research themes were antimicrobial use / resistance (n=50, 33%), occurred in more than 80% patients. The median neutrophil count
pathogen-specific infections (n=25, 16%), device-associated infections recovery time per cycle was 5 days. The incidence of FN in total cycles
(n=17,11%), ICP measures (n=14,9%), general infectious epidemiol- was 29.45% and the median duration was 2 days. There were no dose
ogy (n=10,7%), fever management (n=10,7%), oral mucositis (n=8, adjustments and chemotherapy delays due to adverse events; a total of
5%), diagnostics (n=7, 5%), and other E-Poster Topics (n=12, 8%). Pro- 40.0% of patients received therapeutic antibiotics.
posals targeted patients (n=102, 67%), HCPs (n=32, 21%), caregivers Conclusions: PEG-rhG-CSF appears to be a safe and effective in chil-
(n=2, 1%), other hospital staff (n=2, 1%), or isolated pathogens (n=15, dren with with tumor after chemotherapy. This will become the basis
10%). Study proposals included healthcare chart reviews to random- for PEG-rhG-CSF ’s medication in children in the future, and we will
ized clinical trials, and study designs were cross-sectional (n=6, 4%), further analyze the impact of different tumor subgroups and children’s
retrospective (n=36, 24%), and prospective (n=111, 73%). There was immune function.
1 (1%) systematic literature review, 78 (51%) observational studies,
and 74 (48%) studies with an intervention (treatment alternatives or EP449 / #1864 CONTROL, ALT, BUT DO NOT DELETE; THE
modifications, n=16, 22%; ICP, n=21, 28%; prophylaxis, n=10, 14%; ROLE OF ANTIMICROBIAL LOCK THERAPY (ALT) IN TREATING
education/training, n=9, 12%; clinical decision support tool testing, CENTRAL LINE INFECTIONS IN AN IRISH PAEDIATRIC
n=6, 8%; diagnostics, n=5, 7%; miscellaneous E-Poster Topics, n=7, HAEMATOLOGY/ONCOLOGY PATIENT COHORT
9%).
Conclusions: We identified varied infection-related research areas of Meabh Hyland, Kara Tedford, Sarah Curry, Aisha Alkhaaldi
importance to HCPs caring for children with cancer in LMICs. This Children in Hospital Ireland at CHI Crumlin, Oncology Department, Dublin,
information will help us focus future GID training to address these Ireland
needs.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S357 of S635

Background and Aims: Antimicrobial lock therapy (ALT) is used topre- received HSCT, admitted in a childrens’ hospital in Barcelona (Spain)
vent and treat central line associated blood stream infections (CLABSI) between January 2019-December 2021. During this period, the empir-
in children receiving systemic anti-cancer therapy (SACT). Although ical antimicrobial local protocol for febrile neutropenia included
there is a lack of robust evidence with regard to ALT efficacy in this piperacillin-tazobactam plus amikacin. Meropenem (+/- aminoglyco-
cohort, it has been a component of care at our institution for over 30 side and/or glycopeptide) was first choice drug for patients with known
years. Considerable variations in practice exist between institutions MDR colonization or clinically unstable.
and ALT is not widely accepted as best practice. The aim of this study Results: Overall, 87 positive blood cultures for GNB were identi-
was to retrospectively review and analyse the outcomes of CLABSI, fied from 73 different patients (30 in patients with solid tumours,
which were treated with eithersystemic antimicrobials and ALT or 34 with leukaemia-lymphoma and 9 were HSCT patients). The pre-
exclusively with ALT dominant isolated species were Escherichia coli, Klebsiella spp and
Methods: Retrospective analysis of patients receiving SACT between Pseudomonas spp. The most frequently observed resistance mecha-
2019 and 2021 at Children’s Health Ireland (CHI) at Crumlin. Using the nisms were beta-lactamases hyperproduction (29), extended spectrum
microbiology lab system to identify positive cultures, patient records beta-lactamase(12) and carbapenemases (4).Seven of the patients pre-
were reviewed to collect clinical and treatment details of the CLABSI sented MDR GNB colonization previously to the episode. Resistance to
and the Central Venous Line (CVL) outcome (i.e. salvaged or removed) piperacillin-tazobactam was detected in 13/87 of the isolated microor-
Results: Study included 19 females: 9 males, ages ranging between 1- ganisms and carbapenem resistance in 4/87. All patients were initially
14 years and the most common diagnosis was Acute Lymphoblastic treated with a correct empirical treatment according to in vitro sensi-
Leukemia.An indication of fever (>38◦ C) prompted CVL cultures in all tivity testing except for two (both recovered after targeted treatment).
cases and the most commonly isolated pathogen was coagulase nega- 21 patients required ICU admission and 4 died due to the infection
tive staphylococci. There were 52 CLABSI identified in 28 patients. In despite adequate antimicrobial treatment according to sensitivity test-
49 of these 52 episodes, the line was retained and 25% of these were ing. From the latest, all were patients undergoing intensive non-first
exclusively treated with ALT. line or palliative chemotherapy protocols.
Conclusions: This is the first Irish paediatric study of ALT in tertiary Conclusions: Disease related risk factors and patient’s previous MDR
care. It cannot be stated with certainty that ALT is wholly responsi- colonisations knowledge helped to better target treatment and, con-
ble for the retention of these CVLs; however, we hypothesise that it currently, avoid unnecessary broad-spectrum treatments. Poorer out-
contributes to successful treatment of CLABSI in our population and comes occurred in those patients undergoing non-first line or palliative
permits ambulatory care as opposed to inpatient care. This study has chemotherapy protocols in this cohort, irrespective to the empirical
highlighted the need for more formal, robust research into the use of antimicrobial treatment and microorganism sensitivity profile.
ALT in this high-risk Irish population

EP451 / #1617 PROFILE OF RESPIRATORY VIRAL


EP450 / #1418 OUTCOMES OF GRAM-NEGATIVE INFECTIONS IN A TERTIARY CARE PEDIATRIC
BACTERIAEMIA IN ONCOHEMATOLOGIC PEDIATRIC PATIENTS HEMATO-ONCOLOGY UNIT FROM A DEVELOPING COUNTRY
IN THE ERA OF MULTIDRUG-RESISTANT MICROORGANISMS
Dhaarani Jayaraman1 , Adarsh Kancharla2 , Janani Krishnan3 , Julius
Aina Font I Barceló1 , Ariadna Rigalós Cases2 , Ana Carolina Izurieta Scott1 , Padmasani Venkatraman2
Pacheco3 , Amadeu Gene Giralt4 , Silvia Simo Nebot5 1 Sri Ramachandra Institute of Higher Education and Research, Department

1 Hospital Sant Joan de Déu Barcelona, Pharmacy Department, Esplugues de Of Paediatric Haematology Oncology, Chennai, India; 2 Sri Ramachandra
Llobregat, Spain; 2 Hospital Sant Joan de Déu Barcelona, Paediatrics Depart- Institute of Higher Education and Research, Pediatrics, Chennai, India;
ment, Esplugues de Llobregat, Spain; 3 Hospital Sant Joan de Déu Barcelona, 3 Sankara Nethralaya Hospital, Microbiology/ Virology, Chennai, India

Oncology Department, Esplugues de Llobregat, Spain; 4 Hospital Sant Joan


de Déu Barcelona, Clinical Microbiology Department, Esplugues de Llobre- Background and Aims: Infections remains a challenge in every pedi-
gat, Spain; 5 Hospital Sant Joan de Déu Barcelona, Infectious Diseases Unit, atric hemato-oncology(PHO) unit in developing countries. Prevalence
Esplugues de Llobregat, Spain of viral infections is increasing with 1/3rd of children with cancer having
viral infection.
Background and Aims: Bacteriaemia caused by multi-drug resistant Methods: A prospective descriptive study was done in PHO unit at
(MDR) gram-negative bacteria (GNB) entail high morbidity and mor- our tertiary care centre between January-2020&August-2021. Chil-
tality rates among paediatric cancer and hematopoietic stem cell dren <18 years in PHO unit presenting with fever and/or respiratory
transplant (HSCT) patients. The aim is to describe the characteristics symptoms were enrolled with informed consent. Nasal swabs for viral
and outcomes of GNB episodes occurring in these patients at a referral RT-PCR studies were done apart from standard unit protocol with
hospital in southern Europe. bacterial cultures and antibiotics. Details of demographic data, his-
Methods: Retrospective observational study of all GNB episodes tory, physical findings and lab parameters (CBC/cultures/viral PCR),
occurring in patients <18 years of age with cancer or having treatment and patient outcomes were analyzed.
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S358 of S635 ABSTRACTS

Results: Of the 84 events in 58patients, 16patients had more than by CHROMagar method and routine cultures. The EAT was started on
1episode; 52.4%was noted in <5-years. In our population, we had Day1 of febrile neutropenia (FN) based on colonization data. If no prior
83.3% post-chemotherapy, 5.9% bone marrow transplant recipients colonization was found the escalation strategy was adopted, otherwise
and10.7% with benign hematological conditions requiring immuno- the EAT was de-escalated.
suppressive therapies/ primary immunodeficiencies; the most encoun- Results: The pre-transplant colonization by resistant bacteria was con-
tered diagnosis in 25% being B-Acute lymphoblastic leukemia (ALL). firmed in 54 patients. The routine culturing methods were able to
Fever was noted in 90% followed by respiratory complaints in reveal colonization in 17(22%) cases, but were ineffective in 37(47%).
21%cases; 53.6% were neutropenic (ANC <1500cells/mm3). Median CHROMagar method have shown higher sensitivity being able to find a
duration of illness was 6.9days (range:1-42days) with 88% requiring pathogen in 52(67%) cases with only 2 cases showing positive cultures,
hospitalization,10.7% requiring intensive care and 3.5% succumbing to but not CHROMagar results. In 40(51%) cases the pathogens produced
their illness. In our study population, 57.1% tested positive for viral extended-spectrum beta-lactamases (ESBL), in 7(9%) carbopenemases
pathogen, 10.7% had bacterial infection, 7.1% had mixed infection (Carbo-R) and in 11(14%) cases the Vancomycin-resistant enterococci
and 35% were negative for any pathogen. Rhinovirus was the most (VRE) colonization was confirmed. The FN developed at a median of 4
frequently detected followed by SARS-CoV-2. There was no statisti- (-9 to +14) days past transplant. The escalation strategy was employed
cally significant difference noted in duration of illness or hospital stay in 24% of cases achieving a 42% response rate. The de-escalation strat-
(p=0.106), severity of illness or neutropenia status between those with egy used in 76% of cases with 27 (46%) patients responding first-line
or without virus PCR positivity; however, there was a significant differ- therapy.
ence between neutropenic and non-neutropenic children in duration of Conclusions: The combination of CHROMagar method with routine
hospital stay (p=0.010). cultures allows early colonization detection in order to choose a
Conclusions: Frequency and outcome profile of viral infections were correct EAT strategy.
similar in neutropenic and non-neutropenic children. Identification of a
viral pathogen in a selected cohort (low risk febrile neutropenia&stable EP453 / #1139 NONCOVID-19 VIRAL RESPIRATORY
children) might help in rationalised decision making on antibiotics. INFECTIONS IN CHILDREN WITH CANCER DURING THE
COVID-19 PANDEMIC: A MULTICENTER OBSERVATIONAL
STUDY
EP452 / #1454 EMPIRICAL ANTIBACTERIAL THEAPY BASED
ON COLONIZATION STUDIES IN PEDIATRIC PATIENTS WITH Rejin Kebudi1 , Dilek Kaçar2 , Derya Özyörük2 , Deniz Tuğcu3 , Neşe
SOLID TUMORS RECEIVING HEMOPOIETIC STEM CELL Yaralı2 , Inci Ilhan2 , Ayşenur Bahadır4 , Zeynep Özdemir5 , Aykan
TRANSPLANTATION Özgüven6 , Mehmet Fatih Orhan7 , Ayşen Türedi Yıldırım6 , Canan
Albayrak8 , Ibrahim Kartal8 , Namık Yaşar Özbek2 , Neriman Sarı2 ,
Ilya Kazantsev1 , Asmik Gevorgian2 , Marina Popova3 , Olesya Hüseyin Tokgöz9 , Meryem Albayrak10 , Aylin Canbolat Aydın11 , Nilgün
Yudintseva2 , Julia Rogacheva2 , Tatiana Jukhta1 , Polina Tolkunova1 , Eroğlu12 , Sultan Aydın13 , Hülya Üzel14 , Bulent Zulfikar1 , Mustafa
Andrey Kozlov1 , Margarita Halipskaya1 , Olga Bogdanova1 , Alexander Büyükavcı7 , Hüseyin Gülen6 , Ersin Töret5 , Özcan Bör5
Galimov1 , Tatiana Andreeva1 , Nina Subora1 , Yuri Punanov1 , Ludmila 1 Istanbul University, Oncology Institute, Pediatric Hematology-oncology,
Zubarovskaya4 Istanbul, Turkey; 2 Ankara City Hospital, Pediatric Hematology -oncology,
1 RM Gorbacheva Research Institute Pavlov University, Pediatric Oncology, Ankara, Turkey; 3 Istanbul University Istanbul Medical Faculty, Pediatric
2 RM
Saint-Petersburg, Russian Federation; Gorbacheva Research Institute Oncology, Beyoğlu, Cihangir, Turkey; 4 Karadeniz Technical University, Pedi-
of Pavlov University, Pediatric Oncology, Saint-Petersburg, Russian Federa- atric Hematology -oncology, Trabzon, Turkey; 5 Eskişehir Osmangazi Uni-
tion; 3 RM Gorbacheva Research Institute of Pavlov University, Bone Marrow versity Faculty of Medicine, Pediatric Hematology -oncology, Eskişehir,
Transplant, Saint-Petersburg, Russian Federation; 4 Pavlov University, R. M. Turkey; 6 Celal Bayar University Faculty of Medicine, Pediatric Hematol-
Gorrbacheva Research Institute, Saint Petersburg, Russian Federation ogy -oncology, Manisa, Turkey; 7 Sakarya University Faculty of Medicine,
Pediatric Hematology -oncology, Sakarya, Turkey; 8 Ondokuz Mayıs Univer-
Background and Aims: The carriage of antibacterial resistant strains sity Faculty of Medicine, Pediatric Hematology -oncology, Samsun, Turkey;
by pre-treated patients may be a problem in context of prolonged 9 Necmettin Erbakan University Faculty of Medicine, Pediatric Hematol-
agranulocytosis after hemopoietic stem cell transplantation (HSCT). ogy -oncology, Konya, Turkey; 10 Kırıkkale University Faculty of Medicine,
The pre-transplant intestinal colonization assessment by CHROMagar Pediatric Hematology -oncology, Kırıkkale, Turkey; 11 İstanbul Medeniyet
method may provide more effective detection of resistant pathogen University Faculty of Medicine, Pediatric Hematology -oncology, İstan-
strains. bul, Turkey; 12 Afyon Kocatepe University Faculty of Medicine, Pediatric
Methods: A total of 74 pediatric (median age of 6 years) patients with Hematology -oncology, Afyon, Turkey; 13 Antalya Education and Research
solid tumors (n=63) or non-malignant conditions (n=11) were included Hospital, Pediatric Hematology -oncology, Antalya, Turkey; 14 Dicle Uni-
in June 2020 to March 2022 into an empirical antibacterial therapy versity Faculty of Medicine, Pediatric Hematology -oncology, Diyarbakır,
(EAT) protocol. All patients were assessed prior to autologous (n=56) Turkey
or allogeneic (n=22) HSCT for resistant bacteria intestinal colonization
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S359 of S635

Background and Aims: Acute respiratory tract infections (RTI) are a were revised and data was collected including; demographics, diag-
significant cause of morbidity and mortality in children with cancer, nosis, PCR - COVID, antifungal prophylaxis, radiological findings,
most are precipitated by viral infections. The aim of this study is to Galactomannan, antifungal treatment, and COVID severity. patients
evaluate the nonCOVID-19 viral RTI during the COVID-19 pandemic were classified as possible, probable and proven using the "2020
in children with cancer. ECMM/ISHAM consensus criteria".
Methods: In this multicenter (13 centers) retrospective observational Results: A total of 200 patients were diagnosed with COVID 19 during
study, children and adolescents with cancer who presented with RTI the study period . COVID-19 associated invasive pulmonary aspergillo-
symptoms between January 2020- March 2022, and who had viral sis was found in 22/200 (11%) patients. The primary diagnosis was
RTI confirmed by multiplex polymerase chain reaction were evaluated AML in (10) patients, ALL in (5), post auto-HSCT (2), NHL (1). while
according to demographic, etiological, clinical data and outcome. solid tumors reported in 4 patients (3 with NB and 1 with brain tumor).
Results: A total of 255 episodes in 212 children were evaluated. The CAPA was proven in (2) patients with histopathological diagnosis, prob-
32.1% of episodes occurred in a 16 month period between January able in (12 ) with galactomannan marker more than 1 coupled with
2020 and May 2021 during lockdowns and online distance learn- radiological finding, and possible in ( 8 ) patients with only radiological
ing in Turkey and the remaining 67.8% in a 10 month period after finding suggestive for pulmonary aspergillosis. Although the hemato-
the end of lockdowns and distance learning from May 2021 on. logical malignancy patients were already on antifungal prophylaxis,
Lower RTI comprised 27.5% of episodes. The most common agents breakthrough CAPA was reported in 9/22 (41%). The overall mortal-
were Rhinovirus -Enterovirus (46.3%), Parainfluenza viruses (22.3%) ity was reported in 6 patients (27%) while CAPA attributable mortality
which were detected throughout the year, Influenza viruses (9.8%) was reported in 4 patients ( 18 %).
and Respiratory Syncytial Virus (RSV) (9.4%) which showed peaks in Conclusions: Clinicians caring for pediatric cancer patients with
winter. Parainfluenza viruses, Adenovirus, RSV and Influenza viruses COVID-19 should consider invasive pulmonary aspergillosis, despite
were substantially associated with lower RTI with ratios 47.3%, 36%, being on antifungal prophylaxis, especially with worsening of the clini-
29%, 28% respectively. Complete recovery was observed in 91.7% of cal chest condition. A better understanding of risk factors for adverse
episodes. Complications such as bacterial pneumonia (7.4%), multiple outcomes may improve clinical management in these patients.
organ dysfunction (1.5%), myocarditis (1.1%), hepatitis (1.1%) and Gul-
lian Barre syndrome (0.7%), occurred in 8.6% of episodes. In 30-day
follow-up nine patients died. EP455 / #334 EFFICACY OF LEVOFLOXACIN VERSUS
Conclusions: In children with cancer, the nonCOVID-19 viral RTI AMOXICILLIN/CLAVULANATE AND CIPROFLOXACIN IN THE
episodes were less frequent in the beginning of the pandemic during OUTPATIENT MANAGEMENT OF LOW-RISK FEBRILE
the lockdown and distant education period in comparison to later in the NEUTROPENIA IN CHILDREN; PRELIMINARY RESULTS
pandemic. Viral RTI, albeit in few episodes, may cause morbidity and
mortality in children with cancer, infection control and early diagnosis Reham Khedr1 , Ebtehal Abdelaziz2 , Nashwa Ezzaldin2 , Hadir
are crucial in preventing the spread of infection. Almahellawy3
1 Children’s Cancer hospital Egypt 57357/ National Cancer Institute -
Cairo University, Pediatric Oncology, Cairo, Egypt; 2 National Cancer Insti-
EP454 / #333 COVID-19 ASSOCIATED INVASIVE FUNGAL tute - Cairo University, Pediatric Oncology, Cairo, Egypt; 3 National Cancer
INFECTION AMONG CHILDREN WITH CANCER Institute - Cairo University, Clinical Pathology, Cairo, Egypt

Youssef Madaney1 , Lobna Shalaby2 , Mahmoud Hammad1 , Mervat Background and Aims: Outpatient management of low-risk fever and
Gaber3 , Reem Hassan3 , Abeer Zaki4 , Ibrahim Abdo5 , Reham Khedr1,2 neutropenia should be implemented if close monitoring is accessible
1 Children’s Cancer hospital Egypt 57357/ National Cancer Institute - Cairo and patient compliance is feasible. the use of Amoxicillin/ Clavulanate
University, Pediatric Oncology, Cairo, Egypt; 2 Children’s Cancer hospital plus ciprofloxacin, Levofloxacin, and Moxifloxacin for the treatment
Egypt 57357, Pediatric Oncology, Cairo, Egypt; 3 Children’s Cancer hospital of low-risk febrile neutropenia. There are not enough studies assess-
Egypt 57357, Clinical Pathology, Cairo, Egypt; 4 Children’s Cancer hospital ing the tolerance, safety, and efficacy of these agents. In this study,
Egypt 57357, Clinical Research, Cairo, Egypt; 5 Children’s Cancer hospital we aim to assess the efficacy of single-agent Levofloxacin versus the
Egypt 57357, Clinical Pharmacy, Cairo, Egypt Augmentin /ciprofloxacin regimen used in our institute.
Methods: This is a randomized prospective interventional 2 arm study
Background and Aims: Patients with COVID19 are at risk for sec- of low-risk febrile neutropenia patients presenting to the emergency
ondary complications like invasive aspergillosis. Our study evaluates department at the National Cancer Institute, Cairo University start-
prevalence and outcome of COVID19 associated invasive aspergillosis ing from December 2021 to January 2023. Patients will be randomized
among cancer children at the Children’s cancer hospital Egypt 57357. to double agent ciprofloxacin and amoxicillin-clavulanic acid in com-
Methods: This is a retrospective study including all cancer children parison to single-agent levofloxacin. Follow up of the outpatient cases
diagnosed with COVID-19 and admitted to our hospital during the at; Day 1: Start oral antibiotics, obtain guardians contacts, and assure
period March 2020 - september 2021. The electronic medical records compliance, Day 3: Follow up the patient clinically and follow up count,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S360 of S635 ABSTRACTS

and Day 7: Resolution of infection and stopping of antibiotics regard- significant, acute loss of skeletal muscle mass during this period. Catch-
less of neutropenia. Primary outcomes include Safe marrow recovery up growth was achieved for all cancer types with a significant increase
and improvement of fever in all eligible patients as well as Detecting in weight (chi2 = 40.43, p < 0.001), height (chi2 = 53.79, p < 0.001), BMI
drug-related side effects encountered in both arms of the study. (chi2 = 16.32, p < 0.005)), fat free mass (chi2 = 23.69, p < 0.003) and
Results: A preliminary analysis of the first 30 patients (15 in each skeletal muscle mass (chi2 = 24.19, p < 0.001) after six months.
group) was done. 100% of patients achieved marrow recovery in both Conclusions: Children with cancer are vulnerable for the develop-
arms by D7. Fever subsided in 100% on the Levofloxacin arm com- ment of acute malnutrition with body composition alterations dur-
pared to 60% in the group receiving Augmentin/Ciprofloxacin. only one ing the first two months ofonco-chemotherapy treatment. Monthly
patient on the double agent arm was upgraded to HR and admitted anthropometry and BIA (body composition) measurements assist
to the inpatient. Levofloxacin was tolerable in all patients with no sig- in implementation of timely nutritional interventions to prevent
nificant side effects apart from insomnia reported by the parents in 1 malnutrition.
patient.
Conclusions: Levofloxacin has better efficacy and can be adminis-
tered safely in children with low-risk FN, however close follow-up for EP457 / #526 ANTIBIOTIC AND ANTIFUNGAL USE IN
long-term side effects and monitoring of possible emerging bacterial PATIENTS WITH HEMATOLOGICAL MALIGNANCIES DISRUPTS
resistance is warranted. COMMUNITY LEVEL MICROBIAL ACTIVITIES THAT NORMALLY
ENHANCE COLONIC DEFENSE, INHIBIT INFLAMMATION AND
DECREASE OXIDATIVE STRESS
EP456 / #690 CHANGES IN ANTHROPOMETRICAL STATUS
AND BODY COMPOSITION OF PAEDIATRIC CANCER PATIENTS Katherine Dunn1 , Gloria Rodrigues2 , Zara Forbrigger3 , Tamara
DURING INTENSIVE ONCO-CHEMOTHERAPY Macdonald2 , Ketan Kulkarni2
1 IWK Health Centre, Department Of Pediatrics Division Of Hematology
Ilde-Marié Kellerman1 , Reneé Blaauw1 , Judith Schoeman2 , Mariana & Oncology, Halifax, Canada; 2 IWK Health Centre, Division Of Hema-
Kruger3 tology/oncology, Department Of Pediatrics, Halifax, Canada; 3 Dalhousie
1 Stellenbosch University and Tygerberg Hospital, Division Of Human Nutri- University, Pathology, Halifax, Canada
tion, Department Of Global Health, Cape Town, South Africa; 2 Steve

Biko Academic Hospital/ University of Pretoria, Paediatric Oncology Unit, Background and Aims: Antibiotic and antifungal use is highly preva-
Pretoria, South Africa; 3 Stellenbosch University and Tygerberg Hospital, lent in pediatric patients with hematological-cancers to prevent severe
Department Of Paediatrics And Child Health, Cape Town, South Africa infections and febrile neutropenia. The gut microbiome is impacted by
antibiotic use yielding decreased diversity, reduction in “good” bacte-
Background and Aims: Children with cancer require adequate nutri- ria and increases in “bad” bacteria although there is disagreement on
tional support to prevent malnutrition. This study investigated the exactly which taxa change and limited studies in pediatric oncology
impact of intensive onco-chemotherapy on anthropometrical status patients, whereas the effects of antifungal use are less well-understood
and body composition during the first six months of treatment. and are limited to mouse studies. The composition of the gut micro-
Methods: Anthropometrical status and body composition were mea- biome has implications in the clinical management of hematologic
sured at diagnosis, prior to the initiation of chemotherapy utilis- malignancies. We investigate how antibiotic and antifungal use shapes
ing standardised protocols and validated S10 InBody bio-electrical the microbiome in pediatric patients with leukemia and lymphoma
impedance (BIA) mobile unit. Baseline values for all variables were using both 16SrRNA (16S) and metagenome sequence (MGS) data.
compared to consecutive monthly follow-up measurements to plot Methods: We investigated 134 stool samples from 47 pediatric
changes over time during the first six months. p < 0.05 indicated patients undergoing treatment for acute lymphoblastic leukemia, acute
statistical significance. myeloid leukemia, hodgkin lymphoma and non-hodgkin lymphoma
Results: Forty-three newly diagnosed children (median age 4years, after ethics approval. Antibiotic and antifungal use along with age,
range 0.3–15years; 51% male) participated in the study. There were and sex was collected. Sequences from 16S and MGS were used to
53% haematological malignancies (n = 23) and 47% solid tumours identify microbial taxa. Changes in alpha-diversity were examined
(n = 20). Prevalence of malnutrition varied among anthropometrical using Wilcoxon-test, and multivariable differential abundance analysis
variables, with under-nutrition 14% (mid-upper arm circumference; (MaAsLin2) was used to assess associated changes in the microbiome
MUAC), over-nutrition 9.3% (body mass index; BMI) and stunting with antibiotic and antifungal use, accounting for age differences.
7.1% at diagnosis. MUAC recognised only 14% of patients with actual Results: Antibiotic and antifungal use resulted in decreased diver-
underlying muscle store depletion as per BIA (41.8%). Chemotherapy sity and was associated with a decline in important butyrate pro-
exposure acutely exacerbated existing nutritional depletion during the ducers (Faecalibacterium, Anaerostipes, Dorea, Coprococcus, Blautia,
first two months after diagnosis for all variables except fat mass (FM), Fusicatenibacter). Antibiotic use was associated with an increase in
with contrary effects on cancer type, as haematological malignancies Lactobacillales and Actinobacteria taxa while antifungal use increased
had rapid increases in weight, BMI and FM. All patients had a clinically opportunistic Enterococcus and Gammaproteobacteria taxa.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S361 of S635

Conclusions: This study shows evidence of gut dysbiosis in patients EP459 / #1481 SARS-COV-2 INFECTION IN CHILDREN
with hematological malignancies. Butyrate is important for gastroin- UNDERGOING ONCOLOGIC TREATMENT IN HONG KONG – A
testinal integrity; it inhibits inflammation, reinforces colonic defense, POPULATION-BASED COHORT DURING THE OMICRON WAVE
mucosal immunity and decreases oxidative stress. We find that this
patient population, which could benefit from butyrate’s functions, Anthony P.Y. Liu1,2 , Grace K.S. Lam2 , Wilson Y.K. Chan2 , Terry T.W.
has increased antimicrobial and antifungal use, which is associated Chow2 , Jeanny Cheung2 , Sally C.Y. Wong3 , Wing Leung1,2 , Pamela P.W.
with a significant decline in the microbial community responsible for Lee1,2 , Frankie W.T. Cheng2 , Godfrey Chi-Fung Chan1,2
beneficial butyrate production. 1 The University of Hong Kong, Paediatrics, Hong Kong, Hong Kong PRC;
2 Hong Kong Children’s Hospital, Paediatrics, Hong Kong, Hong Kong PRC;
3 Hong Kong Children’s Hospital, Microbiology, Hong Kong, Hong Kong PRC

EP458 / #105 FERTILITY PRESERVATION AFTER A CANCER


DIAGNOSIS: A STUDY ON KNOWLEDGE, AWARENESS AND Background and Aims: SARS-CoV-2 virus (COVID-19) led to a global
READINESS AMONG PARENTS OF PAEDIATRIC CANCER pandemic disrupting all aspects of healthcare. After a period of limited
PATIENTS IN MALAYSIA local transmission, emergence of Omicron subvariant BA.2 resulted in
rapid surge of cases in Hong Kong. Here we report the features and
Jun Xin Lee1 , Normimi Khatijah Kasim1 , Mohd Faizal Ahmad2 , outcome of COVID-19 infection during the Omicron wave in children
Hamidah Alias1 , Sie Chong Doris Lau1 undergoing anti-cancer treatment.
1 Faculty of Medicine, Universiti Kebangsaan Malaysia, Department Of Methods: Hong Kong Children’s Hospital is the only referral center
Paediatrics, Kuala Lumpur, Malaysia; 2 Faculty of Medicine, Universiti for Pediatric Oncology and Hematopoietic Stem Cell Transplantation
Kebangsaan Malaysia, Department Of Obstetrics And Gynaecology, Kuala (HSCT) in Hong Kong. Based on this population-wide cohort, we stud-
Lumpur, Malaysia ied the clinical features of children on active anti-cancer therapy or
<12-month of HSCT infected with COVID-19 from February-March
Background and Aims: Infertility is one of the most common long- 2022.
term effects experienced by childhood cancer survivors. The concept Results: Among 210 patients fulfilling the above criteria, 47 patients
of fertility preservation (FP) among paediatric oncology patients is (22%) were diagnosed with COVID-19 infection (M:F=35:12). The
still relatively new in developing countries. This study aimed to assess median age at infection was 10.1years (range: 2.1-20.2). Only 2
the knowledge, awareness and readiness for FP among parents of patients received ≥1 dose of COVID-19 vaccine ≥2 weeks before doc-
paediatric cancer patients in Malaysia. umented infection. Thirty-three were diagnosed using RT-PCR, while
Methods: A total of 65 parents participated in this single-centre, cross- 14 were diagnosed using rapid antigen test. Forty-two (89%) patients
sectional study. Validated questionnaires consisting of 10 true/false were symptomatic, with the most common symptoms being fever and
questions were used to determine their oncofertility knowledge; each cough. None developed neurological symptoms. Twenty-seven (57%)
correctly answered question contributed one point to the total score patients required hospitalization, and the median duration of admis-
(maximum 10 points). Another 12 multiple-choice questions were used sion was 5 days (range 1-43). Forty patients had mild symptoms, while
to assess the parents’ awareness and readiness for their child to 2 were considered to have moderate symptom severity (lower respi-
undergo FP before cancer treatment. ratory tract infection in 1, systemic inflammation in 1); remdesivir was
Results: Most of the children in this study were diagnosed with used in 4 patients. None required intensive-care and there was no mor-
leukaemia (n=49, 75.4%), followed by brain tumour (n=4, 6.2%), neu- tality. COVID-19 infection resulted in interruption of therapy in 83%.
roblastoma and lymphoma (n=3, 4.6% each). Majority of them (n=62, Serial COVID-19 testing indicated persistent positivity in some as long
95.4%) were receiving at least one gonadotoxic agent in their treat- as 40 days, including symptomatic re-activation/re-infection in 1.
ment protocol. Half of the parents (n=29, 44.6%) were unaware that Conclusions: SARS-CoV-2 virus resulted in high incidence but mostly
cancer treatment may potentially affect a child’s fertility and almost all mild infection during the Omicron wave for unvaccinated children
(n=61, 93.8%) have not heard of FP before. Mean score for oncofer- undergoing anti-cancer treatment. Treatment interruption was com-
tility knowledge was only 1.31 (SD±1.64), most likely due to lack of mon, persistent viral detection observed and re-activation/re-infection
information given to them by the healthcare providers. Despite being was possible, encouraging vaccination before and after infection.
introduced to the possible option of FP for their child, only half of them (A.P.Y.L. & G.K.S.L. contributed equally)
expressed their interest to discuss this further with their clinicians due
to uncertainty about the procedure as well as concerns regarding the
cost and complications. EP460 / #1037 MOURNING THEMES IN YOUNG CHILDREN
Conclusions: The present study demonstrated a lack of knowledge and AFTER THE DEATH A CLASSMATE: A HYBRID APPROACH FOR
awareness about FP among parents of paediatric cancer patients in THE DEVELOPMENT OF AN EVIDENCE-BASED PICTURE BOOK
our centre. With the increasing number of childhood cancer survivors,
efforts should be made to promote dialogue between providers and the Laura Postma1 , Monique Van Der Werf1 , Marije Gmelig Meyling1 ,
parents to increase their knowledge and awareness on FP. Esther Tijhof1 , Wim Tissing2 , A. A. Eduard Verhagen1 , Erik Loeffen2
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S362 of S635 ABSTRACTS

1 University of Groningen, Beatrix Children’s Hospital, University Medi- Background and Aims: Multiple studies have demonstrated that
cal Center Groningen, Department Of Pediatrics, Groningen, Netherlands; early integration of palliative care (PC) in pediatrics helps with pain
2 University Medical Center Groningen, Department Of Pediatric Oncol- and symptom management, quality of life, alleviates suffering, and
ogy/hematology, Groningen, Netherlands improves communication with families. To understand the structural
barriers and underlying stigma preventing effective delivery of PC to
Background and Aims: Although cure rates of children with cancer children with cancer in Brazil, the “Assessing Doctor’s Attitudes on
have increased dramatically, a portion of children with cancer will not Palliative Treatment” (ADAPT) survey was implemented.
survive their disease. When a child dies, classmates are left behind Methods: Survey questions were updated and translated into Por-
who have to cope with this loss and the emotions involved. We aimed tuguese. A panel of experts from Brazil went through iterative review
to explore the variety of emotions felt by children aged four to eight rounds to improve content validity and cultural sensitivity. Questions
years, after the death of a classmate. We used this to develop and evaluate physician perceptions regarding timing of PC integration,
publish a picture book aimed at supporting these children (and their the scope of palliative treatment, physician responsibility, and ethi-
teachers). cal issues. The survey was electronically distributed to doctors from
Methods: First, we commenced a dual appraised systematic literature any specialty and residents who treat children with cancer in Brazil.
review in multiple databases. Findings from the review formed the Responses were anonymous.
thematic basis for a semi-structured interview study among experts Results: Providers (228) from 16 hospitals from all the 5 regions in
working with bereaved children. These interviews were transcribed, Brazil participated in the survey with a mean alignment to WHO guide-
coded and analyzed qualitatively to determine which emotions had lines of 91%. Although 75% have access to a palliative expert in their
been mentioned. These formed the basis of the picture book we hospital, 48% feel that PC is integrated too late. Over half (54%) stated
developed in collaboration with children’s authors and illustrators. that physicians continue recommending curative treatment even when
Results: After appraisal of 3,247 citations, no studies were identified it has little probability of prolonging life. Most (75%) felt that fami-
describing emotions after the loss of a classmate. We did however lies see the decision to integrate PC as “giving up”. The main barriers
include six studies concerning the bereavement of siblings. Fifteen to early integration of PC were limited physician knowledge on the
experts participated in the interview study. Identified as the most E-Poster Topic (81%), discomfort in raising the E-Poster Topic with fam-
important emotion was sadness, followed by anger, fear and guilt. The ilies (81%), and lack of home-based services (77%). Few participants
experts also noted that young children express their emotions in a felt confident addressing the emotional (30%), spiritual (22%), and grief
more physical way than older children. Subsequently the picture book and bereavement (30%) needs of patients and families
entitled “De Klas Neemt Afscheid” (“The Class Says Farewell”) was Conclusions: Formal education in PC is an important gap to its early
developed, which also includes tools for teachers. integration in the care of children with cancer in Brazil. There is an
Conclusions: When young children lose a classmate, sadness is promi- opportunity to build capacity focused on different patient needs and
nent, although emotions differ per child, according to age, sex, and to work with families to demystify the role of PC in cancer treatment.
relationship with the deceased. We developed an evidence-based pic-
ture book to support children in dealing with this loss. After winning
a science/art-grant, we managed to get this picture book freely avail- EP462 / #1824 GUIDE BASED ON EVIDENCE OF
able for schools in which a child has died or is terminally ill. An English MULTIPROFESSIONAL GUIDANCE FOR FLUID OVERLOAD IN
translation is currently being developed. PEDIATRIC ONCOLOGICAL PATIENTS

Maristela Facholi1 , Martins Neto1 , Luiz Fernando Lopes2 , Andreia De


EP461 / #1116 UNCOVERING THE BARRIERS TO EARLY Paula2
INTEGRATION OF PALLIATIVE SERVICES IN THE CARE OF 1 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil; 2 Barretos
CHILDREN WITH CANCER IN BRAZIL USING THE ADAPT Children’s Cancer Hospital, Pediatric Hospital, Bairro Paulo Prata, Barretos,
SURVEY San Paulo, Brazil

Patricia Loggetto1 , Erica Boldrini2 , Glaucia Costa Murra3 , Dileiny Background and Aims: BACKGROUND: Excessive use of fluids can
Antunes Geronutti Ayub2 , Allan Lima4 , André Oliveira Junior3 , cause tissue edema and organ dysfunction, which can result in high
Monika Metzger1 , Michael Mcneil1 , On Behalf Of The Adapt Brazil levels of morbidity, prolonged length of stay and mortality in pediatric
Working Group2 cancer patients. The multiprofessional team assists in the recogni-
1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis, tion of this clinical condition, bringing an important contribution to
United States of America; 2 Hospital de Amor de Barretos, Palliative Care, this scenario, which impacts on improving patient care and reducing
Barretos, Brazil; 3 Hospital de Amor de Barretos, Pediatric Oncology, Bar- admission to the Pediatric Intensive Care Unit. Thus, the present study
retos, Brazil; 4 Instituto de Medicina Integral Professor Fernando Figueira, is justified for the recognition and early approach of these patients,
Pediatric Oncology, Recife, Brazil aiming to reduce the morbidity and mortality related to fluid over-
load, through the construction of an evidence-based multiprofessional
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S363 of S635

guidance flowchart. AIM: To create a multiprofessional guidance guide ited 16%, oxygen therapy and pronation required 27% . The most
based on evidence of fluid overload in pediatric cancer patients. frequent complication was septic shock. A patient in relapse acute
Methods: Review of articles, for integrative study, selected by research myeloid leukemia died, The hospital stay was 6. All patients continued
in PubMed- MEDLINE, BVS and Cochrane, looking for articles on fluid their cancer treatment, without clinical complications.
overload in pediatric patients. Conclusions: Most of our patients presented moderate disease, 16%
Results: A bibliographic search was carried out in PubMed, Cochrane severe and 5% died. Comorbidities, co-infection, and severe neu-
and VHL databases for studies on water overload in pediatric patients, tropenia increase the risk of severe disease, as well as complications
from 1932 to September 2020. A total of 3692 articles, after removing related to cancer treatment. Our data is small but could be useful for
articles such as those in the Pediatrics area, resulted in general themes: other oncology units in countries with limited resources, no screening
diarrhea, pneumonia and in areas of specialties. Other E-Poster Top- programs and a limited number of tests.
ics were evaluated: neonatology, burns, cardiac surgery, but they did
not contribute to this work, review of duplicate articles and 76 articles
were selected for data extraction according to my inclusion criteria. EP464 / #1872 CHANGES IN ANTHROPOMETRY AND
Conclusions: FO is an important and current E-Poster Topic, as, NUTRITIONAL STATUS IN CHILDHOOD CANCER SURVIVORS IN
reported in the studies found, it is a factor of morbidity and mortal- NEW ZEALAND: A LONGITUDINAL STUDY
ity. In pediatric oncology, the literature is scarce, so further studies are
needed, since excess fluids may be related to reduced survivorship in Amy Lovell1 , Emma Clarke1 , Eveline Van Den Heuvel2 , Erin
these patients, and should therefore be detected earlier if present and Kavanagh3 , Pamela Cheung3 , Andrea Braakhuis1 , Gemma Pugh4
avoided. 1 The University of Auckland, Nutrition And Dietetics, Auckland, New
Zealand; 2 Starship Child Health, ADHB, Paediatric Dietitians, Auckland,
New Zealand; 3 Starship Blood and Cancer Centre, Starship Child Health,
EP463 / #642 CLINICAL COURSE AND EVOLUTION OF Auckland District Health Board, Leap Long Term Follow Up Programme,
CHILDREN WITH CANCER AND SARS-COV-2 INFECTION IN A Auckland, New Zealand; 4 Te Aho o Te Kahu Cancer Control Agency„
PEDIATRIC ONCOLOGY UNIT OF A DEVELOPING COUNTRY National Child Cancer Network, Auckland, New Zealand
(MEXICO)
Background and Aims: Childhood cancer survivors (CCS) are at risk of
Norma Araceli Lopez Facundo, Isidoro Tejocote-Romero, Cecilia malnutrition (over and undernutrition) during treatment and have an
Rodriguez Castillejos, Brenda Gabriela Alonso Sanchez increased burden of chronic disease. This study aimed to explore lon-
HOSPITAL MATERNO INFANTIL ISSEMYM, Pediatric Oncology, TOLUCA, gitudinal changes in anthropometry and determine the prevalence of
Mexico malnutrition during and after treatment.
Methods: Retrospective longitudinal study of CCS in Auckland, NZ,
Background and Aims: Children with cancer have a higher risk of who entered the Late Effects Assessment Programme (LEAP) between
severe Sars-Cov-2 infection, hospitalization and death, compared to January 2018 and December 2020 (N=67). Data were assessed at
healthy children. In low- and middle-income countries, the likelihood of diagnosis, months 3 and 6 of treatment, treatment completion, sur-
severe, critical illness or death from COVID-19 is significantly higher vivorship, and LEAP. Standardised anthropometric data were assessed
than in high-income countries. Aims Describe the clinical course and according to Intensity of Treatment Rating (ITR). A mixed-effects
evolution of children with cancer and Sars-Cov-2 infection in the pedi- model was used to account for repeated measures on the same patient
atric oncology unit of a developing country: Hospital Materno Infantil for anthropometric variables according to ITR.
Issemym, Toluca México Results: Sixty-one percent of patients had some dietetic input. Of
Methods: We included in a cohort children with cancer and Sars-Cov- those, 43% (n=29) saw a dietitian within the first month of diagno-
2 infection corroborated by a positive PCR test. We evaluated clinical, sis. The frequency of dietetic contact varied depending on diagnosis,
demographic, paraclinical characteristics, oncological diagnosis, stage with most patients (39%) receiving nutrition support as an inpa-
of treatment and variables related to the treatment of Sars-Cov-2, tient only. Over 50% of patients had an ITR of 3 or 4. There was
complications and critical interventions during the event, as well as a significant interaction effect between ITR and time since diag-
related to the outcome. Descriptive statistics were performed. nosis for mean zweight and zheight. Patients with an ITR of 2
Results: 54 patients on cancer had criteria for suspected Sars-Cov-2. had a significant increase in zweight at treatment completion com-
PCR test was positive in 18, 10 men and 8 women, 44% > 12 years, 61% pared to diagnosis and patients with an ITR of 3 had a significant
with acute lymphoblastic leukemia . 3 patients debuted with cancer and decrease in zweight at months 3 and 6 of treatment and a sig-
Sars-Cov-2 infection, One patient with relapse of myeloid leukemia. nificant decrease in zheight at month 6, which persisted into sur-
16.6% asymptomatic. The most frequent symptoms were fever in 62%, vivorship. The prevalence of undernutrition (z-score: - 1.0 - - 2.99)
cough 40% and diarrhea in 30%. (28%)severe neutropenia, 55% lym- and overnutrition (z-score ≥ 2.0) was 2% and 10%, respectively at
phopenia < 300/mm3. 2 patients developed multisystem inflammatory diagnosis, increasing nearly six-fold to 11% and four-fold to 18% at
syndrome. Mechanical ventilation and other critical interventions mer- LEAP.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S364 of S635 ABSTRACTS

Conclusions: CCS experience significant changes in standardised patients to promote quality of life and explore goals of care between
anthropometry and nutritional status during treatment and into sur- treatment regimens.
vivorship. Dietetic input only occurred ‘on treatment’, however, these
data highlight the need for continued input in survivorship.
EP466 / #737 ASSESSMENT OF BARRIERS TO PEDIATRIC
PALLIATIVE CARE INTEGRATION FOR CHILDREN WITH
EP465 / #608 TREATMENT OUTCOMES IN CHILDREN WITH CANCER IN LATIN AMERICA
RELAPSED/REFRACTORY SOLID TUMORS: A SINGLE-CENTER
STUDY Michael Mcneil1 , Bella Ehrlich2 , Huiqi Wang1 , Marisol Bustamante3 ,
Veronica Dussel4 , Paola Friedrich5 , Ximena Garcia6 , Srinithya
Matthew T. Mcevoy1 , Elizabeth L. Seashore2 , W. Susan Cheng3 , Gillipelli1 , Wendy Gomez Garcia7 , Dylan Graetz1 , Erica Kaye8 , Monika
Shondra Clay4 , Valeria Smith1 , Jessica Casas5 , Jennifer Foster1 Metzger9 , Carla Sabato Danon10 , Meenakshi Devidas9 , Justin
1 Texas Children’s Hospital, Division Of Hematology-oncology, Houston, Baker11 , Asya Agulnik12
United States of America; 2 UCSF Benioff Children’s Hospitals, Divisions 1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, MEM-
Of Pediatric Hematology-oncology And Pediatric Palliative Care, San Fran- PHIS, United States of America; 2 Brown University School of Medicine,
cisco/Oakland, United States of America; 3 Tulane University, School Of School Of Medicine, Providence, United States of America; 3 Universidad
Public Health And Tropical Medicine, New Orleans, United States of de San Carlos de Guatemala, Palliative Care, Guatemala City, Guatemala;
America; 4 Northern Illinois University, School Of Interdisciplinary Health 4 Center for Research and Implementation in Palliative Care, Palliative Care,

Professions, College Of Health And Human Sciences, DeKalb, United States Buenos Aires, Argentina; 5 St. Jude Children’s Research Hospital, Global Pedi-
of America; 5 Texas Children’s Hospital, Division Of Pediatric Hospice And atric Medicine, Memphis, United States of America; 6 St. Jude Children’s
Palliative Medicine, Houston, United States of America Research Hospital, Global Pediatric Medicine, Memphis, United States of
America; 7 Dr. Robert Reid Cabral Children’s Hospital, Pediatric Oncology,
Background and Aims: Relapsed or refractory (R/R) solid tumors Santo Domingo, Dominican Republic; 8 St. Jude Children’s Research Hos-
remain a significant cause of mortality for children. While upfront ther- pital, Oncology, Memphis, United States of America; 9 St Jude Children’s
apies have been well-studied, the cumulative effect of multiple relapses Research Hospital, Global Pediatric Medicine, Memphis, United States of
and salvage regimens is unknown. We describe the disease trajectory America; 10 Centro de diagnóstico y terapia psicológica, Psychology, Mex-
in patients with R/R solid tumors, from the time of first relapse to time ico City, Mexico; 11 St. Jude Children’s Research Hospital, Department Of
of death or last follow-up. Global Pediatric Medicine, Memphis, United States of America; 12 St. Jude
Methods: We reviewed data from electronic medical records for Children’s Research Hospital, Department Of Global Pediatric Medicine,
patients with primary, malignant R/R solid tumors treated at Texas Memphis, TN, United States of America
Children’s Hospital between 2002-2022. Descriptive analysis was per-
formed along with univariate chi-square and independent sample Background and Aims: Early integration of pediatric palliative care
t-tests. Cox regression analysis was used to evaluate time from relapse (PPC) for children with cancer is critical for the quality of life of both
to death for each variable described. Analysis was performed with patient and family. To improve access to PPC in resource-limited set-
SPSS v24. tings we must understand perceived barriers to early integration. Our
Results: We reviewed 337 patients with R/R solid tumors (female: aim is to understand physician perspectives on ideal versus actual tim-
48%; mean age: 8.33 years; median follow-up after first relapse: ing of PPC integration for children with cancer and uncover barriers to
14.0 months). Most common diagnoses were neuroblastoma (25.8% early integration as identified in Latin America.
of cases), osteosarcoma (16.6%), and rhabdomyosarcoma (15.7%). Methods: The Assessing Doctors’ Attitudes on Palliative Treatment
Patients had a median of 3.03 (IQR: 1-4) progressions or relapses. (ADAPT) survey was developed to evaluate physician knowledge of,
Deceased patients (n=218) had a median of 287 (IQR: 145-583) days beliefs around, and perceived barriers to palliative care integration for
from first relapse to death, and were more likely to have had bone or children with cancer globally. We translated the survey into Spanish
soft tissue sarcoma (p<0.001). Prolonged survival from first relapse to and contextually adapted the tool for use in Latin America. Descriptive
death was associated with specific salvage approaches; increased num- statistics were used to summarize survey data. The McNemar test was
ber of relapses; increased time between diagnosis and first relapse; and used to assess responses regarding the actual versus ideal timing of
increased time between first and second relapse (p<0.05). In a mul- palliative care consultation. Analysis of variance was used to compare
tivariable Cox regression model, the presence of a >21 day break in means for perceived barriers.
salvage therapy was significantly associated with increased time from Results: A total of 831 physicians from 17 countries in Latin Amer-
first relapse to death (p<0.05), while tumor type had no significant ica participated, with a median country response rate of 51.4%. Most
effect (p=0.41). respondents (68.8%) felt palliative care should be involved from cancer
Conclusions: The prognosis for children with R/R solid tumors is poor. diagnosis, but only 14.1% stated that this occurred at their institu-
Delay of salvage therapy did not negatively impact survival after first tion (P<0.001). The highest ranked barriers to early integration of PCC
relapse, implying that this option could reasonably be offered to some were lack of services, personnel, and knowledge about PPC, along with
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S365 of S635

physician and family discomfort about PPC involvement. Respondents Hospital Bonn, Department Of Pediatric Hematology And Oncology, Bonn,
from lower-middle income countries described identified barriers as Germany; 21 University of British Columbia, Division Of Translational Thera-
more challenging to overcome than those from upper-middle and peutics, Department Of Pediatrics, Faculty Of Medicine, Vancouver, Canada;
high-income countries (P<0.001). 22 University Medical Center Utrecht – Wilhelmina Children’s Hospital,
Conclusions: These findings highlight the need for interventions to Department Of Otorhinolaryngology, Head And Neck Surgery, Utrecht,
improve access to resources, didactic training, and clinical education Netherlands; 23 Texas Children’s Hospital, Pediatric Hematology-oncology,
on PPC for physicians in Latin America. Each intervention should be Houston, United States of America; 24 Stellenbosch University and Tyger-
tailored to the needs and opportunities specific to each country to berg Hospital, Department Of Paediatrics And Child Health, Cape Town,
improve quality of life for children with cancer. South Africa; 25 Sydney Children’s Hospital, Kids Cancer Center, Randwick,
Australia; 26 University of Alabama at Birmingham, Department Of Pedi-
atrics, Institute For Cancer Outcomes And Survivorship, School Of Medicine„
EP467 / #1255 EMPOWERING PATIENTS, FAMILIES, Birmingham, United States of America; 27 Great Ormond Street Hospital
CLINICIANS AND TEACHERS ON MANAGING OTOTOXCITY AS for Children National Health Service Trust, Psychological Services, Lon-
AN ADVERSE EVENT OF CHILDHOOD CANCER TREATMENT don, United Kingdom; 28 London, United Kingdom; 29 Birmingham, United
Kingdom; 30 Institute of Cancer and Genomic Sciences, College of Medi-
Annelot Meijer1 , Marry Van Den Heuvel-Eibrink2 , Beth Brooks3 , cal and Dental Sciences, University Of Birmingham, Birmingham, United
Antoinette Am Zehnhoff-Dinnesen4 , Kristin Knight5 , David Freyer6 , Kingdom; 31 Great Ormond Street Hospital for Children National Health
Kay Chang7 , Barbara Hero8 , Vassilios Papadakis9 , A. Lindsay Service Foundation Trust, Department Of Audiovestibular Medicine And
Frazier10 , Claudia Blattmann11 , Rachael Windsor12 , Bruce Morland13 , Cochlear Implant, London, United Kingdom; 32 Great Ormond Street Hospi-
Eric Bouffet14 , Stefan Rutkowski15 , Lieve Tytgat16 , James Geller17 , tal for Children NHS Foundation Trust, Paediatric Oncology, London, United
Lisa Hunter18 , Lillian Sung19 , Gabriele Calaminus20 , Bruce Carleton21 , Kingdom
Hiske Helleman22 , Jennifer Foster23 , Mariana Kruger24 , Richard
Cohn25 , Wendy Landier26 , Martine Van Grotel2 , Lindsey Edwards27 , Background and Aims: Ototoxicity is an early, irreversible
Philippa Simpkin28 , Ollie Simpkin28 , Jawwad Tanvir29 , Keith adverse event occurring in children treated with platinum agents
Wheatley30 , Alexander Hoetink22 , Kaukab Rajput31 , Penelope and cranial irradiation. It can have a severe negative impact
Brock32 on speech-language development and quality of life, especially
1 Princess Máxima Center for Pediatric Oncology, Department Of Research, in very young children. Therefore, it is important to provide
Utrecht, Netherlands; 2 Princess Máxima Center for Pediatric Oncology, information on cancer treatment-related ototoxicity on a global
Division Of Pediatric Oncology, Utrecht, Netherlands; 3 BC Children’s level.
Hospital, Department Of Audiology And Speech Pathology, Vancouver, Methods: Three efforts were established to develop practical knowl-
Canada; 4 Department for Phoniatrics and Pedaudiology, University Hos- edge on several aspects of ototoxicity, including 1) the Ototoxicity Task
pital Münster, Münster, Germany; 5 Oregon Health and Science University, Force (International Society of Pediatric Oncology Supportive Care
Department Of Pediatric Audiology, Portland, United States of America; Committee) consisting of audiological and pediatric oncology experts,
6 Children’s Hospital Los Angeles, Cancer And Blood Disease Institute, Los 2) a multi-disciplinary team including pediatric oncologists, audiolo-
Angeles, United States of America; 7 Lucille Packard Children’s Hospital, gists, a psychologist, a health scientist, and a parent of a child with
Otorhinolaryngology, Palo Alto, United States of America; 8 Children’s Hos- hearing loss, and 3) a Public Advisory Group. Target groups included
pital - University of Cologne, Department Of Pediatric Hematology And childhood cancer patients, survivors, their families, treating clinicians
Oncology, Cologne, Germany; 9 “Aghia Sophia” Children’s Hospital, Depart- and teachers (of the Deaf and Hard of Hearing). The knowledge
ment Of Pediatric Hematology-oncology, Athens, Greece; 10 Dana-Farber obtained can be used upfront, during cancer treatment and for several
Cancer Institute, Pediatric Oncology, Boston, United States of America; years thereafter during survivorship.
11 Olgahospital, Department Of Haematology And Oncology, Stuttgart, Ger- Results: Standardized surveillance recommendations were developed
many; 12 University College London Hospitals National Health Service Trust, for age-appropriate testing, timing and frequency of monitoring during
Department Of Oncology, London, United Kingdom; 13 Birmingham Chil- childhood cancer therapy, based on literature and consensus. Further-
dren’s Hospital, Department Of Pediatric Oncology, Birmingham, United more, an evidence-based open access book chapter was established
Kingdom; 14 The Hospital for Sick Children, Division Of Hematology to inform the target groups on a global level. E-Poster Topics included
And Oncology, Toronto, Canada; 15 University Medical Center Hamburg, the impact of ototoxicity on the young person, and ways to miti-
Department Of Pediatric Hematology And Oncology, Hamburg, Germany; gate or reduce ototoxicity. In addition, the Children’s Liver Tumour
16 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, European Research Network developed educational videos on how
Utrecht, Netherlands; 17 Cincinnati Children’s Hospital Medical Center, to properly support children with ototoxicity in school, along with
Division Of Pediatric Oncology, Cincinnati, United States of America; personal stories of affected childhood cancer survivors and their
18 Cincinnati Children’s Hospital Medical Center, Communication Sciences families.
Research Center, Cincinatti, United States of America; 19 Hospital for Sick Conclusions: The ultimate goal of ototoxicity education is to have
Children, Child Health Evaluative Sciences, Toronto, Canada; 20 University patients and families more involved in the process of cancer treatment
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S366 of S635 ABSTRACTS

and ototoxicity monitoring, and eventually to implement shared deci- EP469 / #79 “YOU DON’T HAVE ANY CHOICE. IF YOU
sion making in clinical practice. Furthermore, it emphasizes the need LEAVE WITH THE CHILD, THE DISEASE WILL WORSEN”:
for clinicians and teachers to support the patient and family who have THEORETICAL APPROACH OF A CLINICAL ETHICS IN AN
to deal with the long-term consequences of cancer treatment. AFRICAN ONCOPEDIATRICS

Léopold Molel Belika1 , Angèle Hermine Pondy Ongotsoyi2 , Grâce


EP468 / #1636 MICROBIOLOGICAL PROFILE OF Joëlle Thérèse Nyemb Mbog3 , Nicodème Djaligue Oumarou4 , Kenn
BLOODSTREAM INFECTIONS IN PEDIATRIC CANCER PATIENTS: Chi Ndi3
A DEVELOPING COUNTRY EXPERIENCE 1 Cameroon Paediatric Oncology Group, General Secretary, Yaoundé,
Cameroon; 2 Mother and Child Centre of The Chantal Biya Foundation,
Sonali Mohapatra1 , Debasish Sahoo1 , Baijayantimala Mishra2 Hematology Oncology Unit, Yaounde, Cameroon; 3 Faculty of Medicine
1 All India Institute of Medical Sciences, Bhubaneswar, Medical Oncol- and Biomedical Sciences of the University of Yaounde 1, Paediatrics,
ogy/hematology, Bhubaneswar, India; 2 All India Institute of Medical Sci- Yaounde, Cameroon; 4 Faculty of Political and Sociological Sciences of the
ences, Bhubaneswar, Microbiology, Bhubaneswar, India Complutense University of Madrid, Philosophy, Yaounde, Cameroon

Background and Aims: Bloodstream infection (BSI) is the most impor- Background and Aims: In a context of medical pluralism, weak univer-
tant cause of morbidity and mortality in pediatric cancer patients. sal health coverage, lack of health insurance, and where the death of
There is a paucity of microbiological data regarding BSIs in pediatric the child is not always accepted, any decision about the patient, while
cancer patients in India. Knowledge of prevailing microbiological pro- respecting a moral code that binds everyone everywhere, becomes a
files will help guide empirical antibiotic therapy in patients with febrile real challenge. Thus, the authors of this article engage in a field that
neutropenia. This study aimed to analyze the microbiological pro- does not yet receive the attention of researchers in Cameroon: clinical
file and sensitivity pattern of the BSIs with underlying risk factors in ethics. The authors lay here the groundwork for a clinical ethics.
children with cancer. Methods: The present study is based on five cases of paediatric
Methods: Blood culture reports of all the pediatric cancer patients at cancers, observed in the Mother and Child Centre of the Chantal
the treating center between February 2019 and January 2022 were Biya Foundation in Cameroon. The selection was based on ethnic-
collected retrospectively. The microbiological profile and sensitivity ity, patient’s relationship with the warden and stage of the disease.
pattern were analyzed. The data collected were analyzed and interpreted on the basis of the
Results: There were 137 positive blood cultures during the study interpretative anthropology of Clifford Geertz.
period. Gram-positive organisms accounted for 54 (39.4%) of all pos- Results: There is no specific profile of caregiver, let alone a known
itive cultures. Growth of gram-negative organisms was detected in respondent, who should act as a mediator between the health care
72 (52.5%) cultures. Candida species were isolated from the rest 11. team and the patient’s family. The emergency of clinical ethics in
Twenty-three episodes of culture positivity were detected in samples Cameroon lies in its codification. Socio-cultural factors linked to the
from central venous catheters. Staphylococcus aureus was the most status of the child in the African culture, the endogenous accounts on
common gram-positive isolate accounting for 48 cases, whereas Pseu- the disease and medical pluralism are all major challenges to be met.
domonas aeruginosa was the most common gram-negative organism. Conclusions: While in the countries of the North, the situations with
Methicillin resistance was detected in 32% of Staphylococcus aureus high ethical stakes are those involving the choice of a therapy, the sus-
isolates while they remained universally sensitive to vancomycin. pension of a protocol, assisted death, euthanasia, etc., in the countries
Among the gram-negative organisms, sensitivity to anti-pseudomonal of the South, these situations are crucially dependent on the socio-
penicillins, aminoglycosides, third-generation cephalosporins, and car- economic and cultural context and on the good faith of the child’s
bapenems were 50%, 52%, 50%, and 60%, respectively. Colistin was parents. In any case, clinical ethics must be thought of in terms of the
sensitive in 92% of isolates, and the rest showed intermediate sensitiv- realities specific to the groups that make up Cameroon.
ity. Antimicrobial resistance was higher in Klebsiella pneumoniae and
Pseudomonas aeruginosa.
Conclusions: Gram-negative organisms were the most commonly EP470 / #1869 COVID-19 IN CHILDREN WITH CANCER:
isolated microbes in the study group. A significantly higher inci- EXPERIENCE AT THE INSTITUTO NACIONAL DE
dence of resistance to the first-line antimicrobial agents was ENFERMEDADES NEOPLASICAS IN PERU
prevalent among gram-negative organisms, particularly with
Klebsiella pneumoniae. Similarly, a high incidence of methicillin Jacqueline Montoya Vasquez1 , Cecilia Ugaz1 , Roxana Morales1 ,
resistance was also observed. It highlights the importance of Rosdali Diaz Coronado2 , Liliana Vasquez1
hand hygiene practices and aseptic barriers with judicious use of 1 Instituto Nacional de Enfermedades Neoplásicas, Pediatric Oncology, Lima,

antibiotics. Peru; 2 INEN, Pediatric Oncology, lima, Peru


15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S367 of S635

Background and Aims: Peru, an upper-middle-income country, translated Brazilian-Portuguese. This study aims to evaluate the valid-
according to the World Bank, was severely affected by the COVID- ity and reliability of the translated version (SCAN pt-BR) at a UMIC
19 Pandemic. Pediatric cancer patients were significantly impacted, pediatric oncology hospital.
because of frequent delays in their access to diagnosis, treatment, and Methods: SCAN is based on six questions specific to identifying
follow-up. Main contributors included economic and social issues, but the nutritional needs of children with. Scores higher than 3-points
also the high viral transmission among these special population indicate a risk of malnutrition. The original tool was validated com-
Methods: The Instituto Nacional de Enfermedades Neoplásicas (INEN) paring with SGNA in a HIC country. To validate the SCAN pt-
is the first referral center for pediatric cancer in Peru. All pedi- BR, we compared to STRONGkids, the only pediatric screening
atric patients attended from May/20 to Mar/22 have been tested for tool translated and validated to Brazilian-Portuguese. The reliabil-
COVID- 19 at admission, in case of respiratory symptoms or fever ity was observed through a test-retest assay. Significance was set at
during hospitalization or previous to any procedure. A prospective p < 0.05
database registry was conducted with data from all patients with Results: 267 children with cancer were evaluated in this study, 35 of
a positive SARS-COV2 test including sociodemographic, clinical, and whom took part in the test-retest essay. The reliability phase identified
outcome characteristics. a substantial inter-rater agreement of translated SCAN (ICC=0,77).
Results: From the early phase of the COVID-19 pandemic, we reg- 188 children (70.4%) had a SCAN pt-BR score ≥3. Although, by
istered 292 pediatric cancer patients with COVID-19 infection. 217 anthropometric evaluation, 11,4% of patients presented malnourished
patients (74.3%) had hematological neoplasms, mainly acute lym- (Z-score ≤-2). According to the ROC curve analysis, the SCAN pt-BR
phoblastic leukemia, and 75 patients had solid tumors. 188 (64.3%) showed excellent accuracy with an AUC of 0.86 (95% CI:0.819,0.911).
were male and 104 patients were females. Thirty percent of patients Using standard cut-off≥3, a sensitivity of 77.5% and specificity of
were neutropenic at diagnosis. We registered 8 deaths: 6 patients with 83.5% were found. Considering the ideal sensitivity of 100% for a
progressive disease, 1 female patient with advanced osteosarcoma and screening tool when the cut-off was set to ≥2 raised the sensibility to
pneumonia that had a positive test at admission, and 1 female patient 96% and decreased specificity to 49.4%
with hepatoblastoma and severe pneumonia. Three patients needed Conclusions: Validated against the STRONGkids, SCAN pt-BR showed
intensive support, of which two of them completely recovered from the good reliability and accuracy with higher specificity than the origi-
COVID-19 infection. Overall, the cohort mortality was 2.9%. nal scale (Se100%, Sp39%) in our pediatric oncology population even
Conclusions: At INEN, we registered the largest cohort of pediatric when the cut-off was decreased to ≥2.However specific conditions and
cancer patients with COVID-19 in Peru. Our results showed that only a populations especially those from UMIC or LMIC countries particular
few patients developed severe respiratory disease, being most of them tools are more sensitive than generic tools and need be adapted for
asymptomatic. Neutropenia was not a risk factor for severe disease. socio-economic reality.
Future research is needed to determine the long-term outcome for
pediatric cancer patients in developing countries.
EP472 / #1999 IS STRONG-KIDS A GOOD NUTRITIONAL
SCREENING TOOL FOR BRAZILIAN CHILDREN WITH CANCER?
EP471 / #1975 IS A 3-POINTS CUTOFF AT SCAN IDEAL TO
SCREEN FOR NUTRITIONAL RISK IN CHILDREN WITH CANCER Mariana Murra1 , Maria Clara Rossi2 , Marise Firmino2 , Marielle
FROM LMIC? Ferreira3 , Maynara Da Silva3 , Wilson De Oliveira Jr4 , Ricardo Costa5
1 Hospital de Amor, Pediatrics, Barretos, Brazil; 2 FACULDADE DE CIEN-
Mariana Murra1 , Wilson De Oliveira Jr2 , Marise Firmino3 , Maria Clara CIAS DA SAUDE DR PAULO PRAT, Epidemiologia, BARRETOS, Brazil;
Rossi3 , Marielle Ferreira4 , Maynara Da Silva4 , Marco De Oliveira5 , 3 FACULDADES BARRETOS, NutriÇÃo, BARRETOS, Brazil; 4 Hospital de
Alexia Alford6 Amor: Unidade Infanto Juvenil, Pediatric Surgery, Barretos, Brazil; 5 Barretos
1 Hospital de Amor, Pediatrics, Barretos, Brazil; 2 Hospital de Amor: School of Health Sciences Dr. Paulo Prata - FACISB, Epidemiologia, Barretos,
Unidade Infanto Juvenil, Pediatric Surgery, Barretos, Brazil; 3 FACULDADE Brazil
DE CIENCIAS DA SAUDE DR PAULO PRAT, Epidemiologia, BARRETOS,
Brazil; 4 FACULDADES BARRETOS, NutriÇÃo, BARRETOS, Brazil; 5 Hospital Background and Aims: Although a significant number of pediatric
de Amor, Center Of Epidemiology And Biostatistics, Barretos, Brazil; nutritional screening tools have been developed, only a few are avail-
6 INTERNATIONAL ATOMIC ENERGY AGENCY, Nutritional And Health, able (and validated) in languages other than English. For example, the
VIENNA, Austria Screening Tool for Risk Of impaired Nutritional status and Growth
(STRONGkids) is one of the most frequently used instruments in
Background and Aims: Nutritional screening aiming at the early assessing the malnutrition risk among pediatric inpatients and one of
detection of malnutrition in hospitalized pediatric patients has been the few translated and validated to Brazilian-Portuguese. Malnutrition
strongly recommended for children with cancer. Murphy et al (2016) in children with cancer can significantly affect outcomes, so nutritional
developed and validated the SCAN (Nutrition Screening Tool for Child- screening aiming at the early detection of malnutrition in hospital-
hood Cancer) tool specifically for pediatric oncology and recently ized pediatric patients has been strongly recommended for children
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S368 of S635 ABSTRACTS

with cancer. This study aims to evaluate the effectiveness of using test it was observed difference between the averages of thumb of
STRONGkids to identify pediatric cancer patients at nutritional risk. malnourished and obese group (P<0.001) and adequate and obese
Methods: The anthropometric assessment was used as the refer- according to MUAC (P=0.011). For triceps skinfold had a difference
ence standard in evaluating the effectiveness of using STRONGkids to between the averages of the thumb in the malnourished and obese
identify pediatric cancer patients at nutritional risk. groups (P=0.002), averages of the risk group of malnourished and
Results: 267 hospitalized children with cancer were evaluated in this obese (P=0.039) and between the averages of the adequate and obese
study and submitted to the STRONGkids tool within 48 hours of groups (P<0.001).
admission. The median score obtained in our sample was 3 points, Conclusions: The correlation between APMT measurements with the
corresponding to medium nutritional risk. In this cohort, 70.7% of chil- anthropometric data did not show a good correlation, not even with
dren were eutrophic by BMI Z-score. The analysis of the correlation the arm circumference which shows to be sensitive for pediatric can-
of anthropometric and body composition measurements found that cer patients, thus, can the EMAP be a tool for early diagnosis of
56.6% of patients with adequate arm-circumference were classified as malnutrition?
medium/high nutritional risk, and 59.4% with adequate triceps skinfold
(p-value 0.043).
Conclusions: STRONGkids seems not to have parameters that guaran- EP474 / #2024 RELEVANCE OF CLINICAL SIGNS AND
tee sensitivity for this population by overestimating the nutritional risk, SYMPTOMS OF MALNUTRITION IN CHILDREN WITH CANCER
which can be a problem in UMIC and LMIC countries where nutrition
assessment and management are readily available in all situations. Wilson Oliveira Junior1 , Marielle Ferreira2 , Maynara Da Silva2 , Marise
Firmino3 , Maria Clara Rossi3 , Mariana Murra4
1 Barretos School of Health Science Dr. Paulo Prata - FACISB, Surgery,
EP473 / #2003 ADDUCTOR POLLICIS MUSCLE THICHNESS barretos, Brazil; 2 FACULDADES BARRETOS, NutriÇÃo, BARRETOS, Brazil;
(APMT) A EASY WAY TO ENHANCE BODY COMPOSITION 3 FACULDADE DE CIENCIAS DA SAUDE DR PAULO PRAT, Epidemiologia,
EVALUATION IN CHILDREN WITH CANCER BARRETOS, Brazil; 4 Hospital de Amor, Pediatrics, Barretos, Brazil

Mariana Murra1 , Maynara Da Silva2 , Marise Firmino3 , Maria Clara Background and Aims: Nutritional status is classically assessed
Rossi3 , Marielle Ferreira2 , Ricardo Da Costa4 , Wilson De Oliveira Jr5 by using anthropometric parameters although changes usually only
1 Hospital de Amor, Pediatrics, Barretos, Brazil; 2 FACULDADES BARRETOS, appear when malnutrition is already installed. In this sense identifying
NutriÇÃo, BARRETOS, Brazil; 3 FACULDADE DE CIENCIAS DA SAUDE early signs and symptoms that indicate the risk of malnutrition could
DR PAULO PRAT, Epidemiologia, BARRETOS, Brazil; 4 FACISB - Faculdade ensure an early intervention to prevent this undesirable outcome.This
de Ciencias da Saúde de Barretos "Dr Paulo Prata", Barretos School Of study aims to characterize signs of malnutrition and gastrointestinal
Medicine, Barretos, Brazil; 5 Hospital de Amor: Unidade Infanto Juvenil, symptoms in children with cancer undergoing the first four months of
Pediatric Surgery, Barretos, Brazil treatment at a UMIC pediatric oncology hospital.
Methods: A cross-sectional study with prospective data collection
Background and Aims: Much has been discussed about the parame- which included patients over 0 to18 y.o of age who were hospital-
ters to identify malnutrition in pediatric cancer patients. Considering ized in the inpatient unit of a developing country, diagnosed with
the different social issues that guide decision making, anthropometry is childhood cancer less 4 months. Anthropometric and related signs
an easily accessible, fast and low cost tool. APMT has been suggested as and symptoms data were collected and the patients were evalu-
a promising parameter to evaluate the muscle compartment. Evaluate ated and questioned about the signs and symptoms 48 hours after
the behavior in pediatric oncology patients with the APMT and the cor- admission.
relation between MUAC and another antrhopometrics measure with z Results: The vast majority of the 81 participants did not show any
score de peso, estatura, (MUAC and tricipal skinfold). early signs of malnutrition(79%) nor symptoms of the upper(66.7%)and
Methods: Cross-sectional study with prospective data collection, low(59.3%) gastrointestinal tracts(GIT).Whether children identified
which included patients aged 0 to 18 years old who were hospitalized malnourished (or at risk of malnutrition) presented more clinical
in the inpatient unit of a developing country, diagnosed with childhood signs and symptoms than normal-weight children correlation analy-
cancer within 3 months. Anthropometric and body composition data sis was performed between the nutritional profile and the frequency
were collected within 48h of their hospitalization. of clinical signs of malnutrition and symptoms of upper and low
Results: 135 patients were included with a mean age of 11 years who GIT.Significant correlations were found between the nutritional sta-
underwent evaluation of the APMT and objective anthropometric data. tus identified by the BMI by age and the presence of clinical signs
They had a mean APMT of 10.79 mm, 95% CI (11.6 - 9.93). When cor- of malnutrition(p=0.026)and upper GIT symptoms(p=0.008).In addi-
relating the thumb measurement with weight z score, height z score, tion correlation was found between the nutritional status obtained
BMI z score, MUAC and triceps skinfold. They showed low correla- through the measurement of MUAC and the occurrence of clini-
tion. When performed the comparison between groups by ANOVA cal signs of malnutrition.The other parameters used for nutritional
test, the data showed normal distribution, After Bonferroni post hoc assessment(Weight-for-ageHeight-for-age AMC and subscapular and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S369 of S635

triceps skinfold thicknesses) did not present a correlation with the Asma Naheed, Hamna Khan, Neelam Abdulqudoos
signs and symptoms presented by the children. The Indus hospital, karachi, pakistan, Psychosocial, Karachi, Pakistan
Conclusions: The clinical signs and symptoms of malnutrition should’nt
be underestimated in hospitalized children since the presence of Background and Aims: Parents of childhood cancer patients signif-
these signs correlates directly with their nutritional status and can icantly benefit from emotional support following their child’s loss in
be identified early during daily assessments during the hospitalization the long run. Using adaptable and diversified supporting services that
period. change as the grieving process progresses is crucial to coping and
mental health of grieving parents. The bereavement program at the
Indus Hospital & Health Network (IHHN) was adopted and developed
EP475 / #1188 LEGACY BUILDING; A WAY OF COPING in collaboration with grieving parents by the Palliative care team of
AMONGST PARENTS OF CHILDREN ON QUALITY OF LIFE the Psychosocial Department to improve and increase institutional
CARE IN PAKISTAN services offered to families during and after a child’s death.
Methods: The St. Jude’s Bereaved Parent Program Mentorship pro-
Hamna Khan, Asma Naheed, Neelam Abdulqudoos gram was contacted for material and permission was granted for
The Indus Hospital, Pediatric Psycho-social, Karachi, Pakistan replication and translation of their material under copyright of St. Jude
Hospital and Research Center. A total of 4 bereaved parents from
Background and Aims: Losing a loved one to a terminal disease can IHHN consented to participate in the bereavement Program, only 3
forever change one’s life, and the emotional toll it takes on families is participants completed the program. Parents were trained on differ-
immeasurable. Research has shown that the grief related to losing a ent skills including, communication, boundary making, confidentiality,
child can be more severe than that of a spouse or any other family mem- spiritual care and self-care to provide secured emotional support to
ber. Given the intensity of grief faced by bereaved parents, the need for mentees. Seven sessions were conducted with the participants, includ-
a multidimensional approach is essential in aiding parents and families ing a focus group discussion. Pre-training, a detailed interview to assess
through their process of grieving. Legacy building has proven benefi- their availability, readiness and understanding of the program.
cial to the parents and their loved ones in various different ways from Results: The developed program was piloted and ran through smoothly,
aiding conversations about remembrance of the child to even help- with parents comprehending, understanding and inculcating the val-
ing them come to terms with the child’s mortality. This exploratory ues and training provided to them. The mentors have begun to build
study based at Indus Hospital & Health Network (IHHN) looks at how their mentor-relationships with the mentees and are on-going with the
grieving parents get help from legacy building. program.
Methods: To better understand the impact of legacy making on Conclusions: This Comprehensive Program can be used as a model for
bereaved parents, this study assessed feelings and thoughts of parents the development of bereavement programs in other Pakistani insti-
and their recommendations for its utilization. Photos captured with tutes to aid bereaved parents overall, leading to enhanced awareness
a polaroid camera and hand prints through oil paints. Parents’ details of grief, parental grief and support systems.
were obtained through electronic medical records. A survey form was
created to explore parents experiences regarding legacy projects. A
total of 6 parents were interviewed over the phone to fill the survey. EP477 / #1838 RESULT OF THE IMPLEMENTATION OF A
Results: All respondents said they would suggest the Legacy build- PEDIATRIC EARLY WARNING SYSTEM (PEWS) IN A
ing program to other families facing end-of-life decisions. Parents HEMATO-ONCOLOGY HOSPITALIZATION AREA IN MEXICO
expressed that these souvenirs helped them significantly with their
grieving process and with focusing on positive memories of their child. Isidoro Tejocote Romero1 , Melisa Najera Castillo1 , Merle
The frequency with which respondents observed or touched their Laffont-Ortiz2 , Cesar Castro Maximino1 , Guadalupe Dominguez
child’s memory varied among subjects. Most of the parents were from Flores1 , Rufina Coyote Rosario1 , Edith Guzman Aguila1
underprivileged backgrounds and did not have any other souvenirs of 1 Hospital para el Niño de Toluca IMIEM, Pediatric Oncology, Toluca, Mexico;

their child. 2 Hospital para el Niño del Instituto Materno Infantil del Estado de México,
Conclusions: The legacy building program is an efficient, hassle free Hemato-oncología, Toluca, Estado de México, Mexico
and cost-effective interventions for bereaved parents and their fam-
ilies. This model if adopted widely can greatly support bereaved Background and Aims: Pediatric Early Warning Systems (PEWS) are
parents. clinical assessment tools that use patient vital signs and symptoms to
detect and respond to clinical deterioration events (CDE). Many hospi-
tal settings in developing countries, such as Mexico, lack systems for
EP476 / #1189 DEVELOPMENT AND IMPLEMENTATION OF the early identification of cancer patients at risk of clinical deterio-
BEREAVED PARENT MENTOR PROGRAM MODEL IN ration. Objective: Show results after implementing a modified PEWS
NOT-FOR-PROFIT HEALTH CARE SETUP, LMIC PAKISTAN (EVAT) in the Pediatric Hemato-Oncology area at the Hospital para el
Niño de Toluca IMIEM.
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S370 of S635 ABSTRACTS

Methods: A modified PEWS was implemented in July 2020 in the Pedi- Results: In 608 FN episodes, the CDR alone identified 39.1% (238/608)
atric Hemato-Oncology area at the Hospital para el Niño de Toluca CSR episodes; 5.9% (14/238) had severe events. Prospectively using
IMIEM. In addition, a retrospective cohort was used to evaluate indi- the SDR, the SHR group included 76.6% (92/120) of episodes; severe
cators of clinical deterioration in the previous year (2019) and in the events occurred in 20% (3/15) of CSR episodes included in the SHR
following year (2021) the implementation of PEWS. group due to elevated procalcitonin ≥0.4 ng/mL. The SHR group had
Results: Before implementing the modified PEWS, 32 EDCs with a significantly more BSI [21.7% (20/92) vs. 0% (0/28); P= 0.007] and ICU
mean of 20.4 days of hospitalization were recorded, and in the subse- admissions [13% (12/92) vs. 3.6% (1/28); P= 0.158]. This group also
quent period, 28 EDCs and a mean of 24.03 days were observed. When had significantly fewer short hospital stays < 3 days [17.4% (16/92) vs.
comparing the periods, a 46% reduction in mortality was observed. In 60.7% (17/28); P= <0.001].
addition, the rate of clinical deterioration events decreased after imple- Conclusions: The SDR with serial procalcitonin aided in identifying
menting the modified PEWS (1.69 vs 1.46 per 1,000 inpatient days). severe events in CSR episodes, but analysis was limited. Institu-
A 3.6% increase in total hospital patient-days was observed; however, tions may consider similar internal evaluation methodology before FN
PICU (Pediatric Intensive Care Unit) days of stay decreased from 5.7 to episode risk stratification.
4.6.
Conclusions: The implementation of PEWS is an effective tool for early
identification of clinical deterioration of patients during hospitaliza- EP479 / #855 PREVALENCE AND OUTCOMES OF
tion, allowing interventions leading to a decrease in childhood cancer CARBAPENEM RESISTANT BLOODSTREAM INFECTION IN
morbidity and mortality. It is expected that the implementation of the CHILDREN WITH CANCER
modified PEWS will grow over time, with multidisciplinary collabora-
tion in order to be integrated into the quality improvement strategy of Garima Nirmal1 , Jithin Tk1 , Gopakumar Kg1 , Parthiban Rudraswamy2 ,
hospital care. Riyas M3
1 Malabar Cancer Centre, Division Of Pediatric Oncology, Moozhikkara,
India; 2 Malabar Cancer Centre, Division Of Microbiology, Moozhikkara,
EP478 / #1790 INTERNAL EVALUATION OF RISK India; 3 Malabar Cancer Centre, Division Of Clinical Research & Biostatistics,
STRATIFICATION TOOL USING SERIAL PROCALCITONIN AND Moozhikkara, India
CLINICAL RISK FACTORS IN PEDIATRIC FEBRILE
NEUTROPENIA: NON-INTERVENTIONAL, SINGLE INSTITUTION Background and Aims: Carbapenem-resistant (CR) infections cause
EXPERIENCE PRIOR TO CLINICAL IMPLEMENTATION major morbidity and mortality.Data on CR infections in children with
cancer are scarce,especially from developing world.The aim of this
C. Nathan Nessle1 , Tom Braun2 , Sung Choi1 , Rajen Mody1 study was to evaluate characteristics and outcomes of bacteremia
1 University of Michigan, Pediatric Hematology Oncology, Ann Arbor, United with CR organisms (CRO) compared to bacteremia with Carbapenem
States of America; 2 University of Michigan, School Of Public Health, Ann sensitive organisms (CSO) in children with cancer.
Arbor, United States of America Methods: This retrospective observational study was conducted in a
tertiary pediatric oncology centre in South India. Data on all blood-
Background and Aims: Risk stratification of pediatric febrile neu- stream infections with Gram negative organisms (CRO and CSO) in
tropenia (FN) is an established concept, yet clinical decision rules (CDR) children with malignancy aged ≤14 years, from August 2017 to July
tools misclassify up to 5% of clinical standard-risk (CSR) episodes 2021 were retrieved. The final outcome was determined as survival
with severe outcomes. The clinical discriminatory performance of a and all-cause death at 28 days after the date of Bloodstream infection
CDR observed in one region cannot be inferred to a different region (BSI) onset.
and patient population. The internal evaluation of a CDR before Results: Sixty four patients with gram negative BSI were identified,
implementation has not been well-described. with 23.4% (n=15) in the Carbapenem Resistant Bloodstream Infec-
Methods: This non interventional cohort study of FN episodes evalu- tion (CR-BSI) group and 76% (n=49) in the CS-BSIgroup. The patients
ated a CDR alone then prospectively with serial procalcitonin before included 39 males (61%) and 25 females (39%), with age ranging from
implementation. The study decision rules (SDR) incorporated serial 1 year to 14 years (median age: 6.2 years). The most common underly-
procalcitonin with a modified, more restrictive CDR (Alexander, et al.) ing disease was hematologic malignancy ( 92.2%, n=59). Children with
recommended by the Children’s Oncology Group. Median time from CR-BSI had higher incidence of prolonged neutropenia, septic shock,
fever onset for each procalcitonin value was 2 and 16 hours, respec- pneumoniae, enterocolitis, altered consciousness and acute renal fail-
tively. The study standard-risk (SSR) group met CSR criteria with serial ure and were associated with 28 day mortality in univariate analysis.
procalcitonin <0.4 ng/mL. The study high-risk (SHR) group met clin- The most common carbapenem resistant gram negative bacilli iso-
ical high-risk (CHR) criteria or CSR with a procalcitonin ≥0.4 ng/mL. lates were Klebsiella species (47%) and E.coli (33%).All carbapenem
Severe events were defined as blood stream infection (BSI), intensive resistant isolates were sensitive to colistin and 33% were sensitive
care unit (ICU) admission, or death. Descriptive and bivariate statistics to Tigecycline. The case-fatality rate was 14% (9/64) in our cohort.
compared the groups and outcomes. The overall 28 days mortality was significantly higher in patients with
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ABSTRACTS S371 of S635

CR-BSI than in those with CS-BSI (28-day mortality: 43.8% vs. 4.2%, Conclusions: Participating hospitals within Greater Kumasi
P = 0.001). demonstrated adequate readiness to provide pediatric can-
Conclusions: Bacteremia with CRO has higher mortality in children cer shared-care services. Palliative care requires specific
with cancer. Prolonged neutropenia, pneumoniae, septic shock, ente- attention.
rocolitis, acute renal failure and altered consciousness were predictors
of 28-day mortality in carbapenem resistant septicemia.
EP481 / #1066 GRANULOCYTE TRANSFUSIONS AS A
LIFESAVING TREATMENT FOR PEDIATRIC PATIENTS WITH
EP480 / #1478 BASELINE ASSESSMENT FOR FACILITY NEUTROPENIA AND SEPSIS
READINESS FOR PEDIATRIC CANCER SHARED-CARE SERVICES
IN GREATER KUMASI, GHANA Rasmi Palassery1 , Arjun Mandade1 , Santhosh Devadas1 , Deepa
Parthiban2 , Vinayak Maka1 , Swaratika Majumdar1 , Nalini Kilara1
Lawrence Osei-Tutu1 , Bernice Eklu2 , Vivian Paintsil3 , Yaa 1 MS Ramaiah Medical College, Medical Oncology, Bengaluru, India; 2 MS
Oppong-Mensah1 , Paul Obeng1 , Barnabas Manlokiya Raymond1 , Ramaiah Medical College, Transfusion Medicine And Blood Bank, Ben-
Comfort Asoogo1 , Abubakari Jaliu4 galuru, India
1 Komfo Anokye Teaching Hospital, Department Of Child Health, Kumasi,
Ghana; 2 World Child Cancer, Data Management, Kumasi, Ghana; 3 Kwame Background and Aims: Severe neutropenic sepsis (SNS) caused by
Nkrumah University of Science and Technology, Child Health, Kumasi, multi-drug resistant (MDR) bacteria is a leading cause of mortality in
Ghana; 4 City Cancer Challenge, C/can Kumasi City, Kumasi, Ghana patients with hemato-lymphoid malignancies and hematopoietic stem
cell transplantation (HSCT) recipients. Granulocyte transfusion (GTX)
Background and Aims: Experts generally recommend patient/family- is a therapeutic option for SNS with limited data on its efficacy. Our aim
centered care for pediatric cancer treatment in centers with suitable was to study the effectiveness of GTX administered to patients with
facilities, specialized skills, and multidisciplinary team capacity. In SNS at our institution.
Ghana access to comprehensive treatment centers is limited. Decen- Methods: Patients who received GTX between April 2017 to March
tralizing some aspects of care may improve the quality of care. A 2022 were included.
collaborative project by the Komfo Anokye Teaching Hospital, KATH, Results: Out of the 25 patients who received GTX, 11 were <18 years
and the City Cancer Challenge, C/CAN implemented in February 2022 old. A total of 22 transfusions happened with 9 patients receiving
aimed to train hospital staff within Greater Kumasi on pediatric cancer >1 transfusion for the same episode of neutropenia. The median age
early warning signs and referral. We aimed to assess the person- was 5 years. Male to female ratio was 2.6. Nine patients were under-
nel, capabilities, and facilities available to provide pediatric cancer going HSCT while two were receiving induction for acute leukemia.
shared-care services among participating hospitals. Patients were on an average of five anti-microbials, with positive bac-
Methods: We conducted a cross-sectional survey among hospital terial blood culture (BC) in only 9 patients. Six (54.5%) patients had
managers, HM and healthcare providers, HCP using a structured ques- MDR Klebsiella pneumoniae in BC, one each had Escherichia coli
tionnaire including a 5-point Likert scale rating by HCPs on their and Pseudomonas aeruginosa and, one patient grew Staphylococcus
hospitals’ readiness. Data were analyzed with Microsoft Excel. aureus (methicillin sensitive); the latter had evidence of fungal pneu-
Results: There were 31 HMs and 63 HCPs from 17 hospitals including 1 monia. The mean granulocyte dose was 2.65 x 108 neutrophils per
Tertiary; 1 Regional; 5 Private; and 10 Secondary (4 Quasi-Government kilogram body weight. No patient experienced any transfusion-related
and 6 District). All hospitals had dedicated pediatric wards. Most hos- adverse effects. Nine patients (81.8%) showed response; 2 patients
pitals had on-site blood banks 15/17(88%); Ultrasound 13/17(76%); progressed and died of sepsis. The average time to resolution of fever
Automated Full Blood Count Analyser 12/17(71%); Blood Chemistry and stopping of therapeutic anti-microbials from the last GTX was 1.45
Analyser 10/17(59%); and X-ray services 10/17(59%). No hospital had and 4.68 days respectively. A paired t-test showed an improved mean
Pediatric Intensive Care facilities, while 16/17(94%) had pharmacists WBC count from 65.45/uL before to 963.63/uL 24 hours after the last
and laboratory scientists, 13/17(76%) hospitals had pediatricians and GTX (p=0.03) and C-reactive protein from 21.05 mg/L to 13.16 mg/L
surgeons. Only 2 hospitals had any palliative care services. Although (p=0.0008).
10/17(59%) could dispense parenteral morphine, only 7/17(41%) dis- Conclusions: GTX offers a safe and effective treatment for our patients
pensed oral morphine. Among the 63 HCP trainees, at least 43(68%) with SNS, especially in the era of MDR bacterial infections.
and 23(37%) agreed that their hospitals could provide “some basic
aspects of pediatric cancer care” and “palliative and end-of-life care”
respectively, while 39(62%) and 46(73%) agreed to being “highly confi- EP482 / #153 RESULTS OF AN EDUCATIONAL COMPONENT
dent in identifying a child with suspected cancer” and providing “initial OF DECREASING TIME TO THERAPY (DOTT) IN PEDIATRIC
care for children receiving chemotherapy with fever or intercurrent CANCER PATIENTS WITH FEBRILE NEUTROPENIA IN PERU
illness” respectively.
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S372 of S635 ABSTRACTS

Claudia Pascual1 , Liliana Vasquez2 , Ligia Rios3 , Miguela Caniza4 , Ana Gainesville, United States of America; 3 University of Wisconsin-Milwaukee,
Mendieta3 , Maria Vargas3 , Walter Delgadillo3 , Carlos Rueda3 , Alexis Psychology, Milwaukee, United States of America; 4 Nationwide Children’s
Holguin5 , Carlos Santillan1 , Maysam Homsi4 , Essy Maradiegue5 Hospital, Pediatric Palliative Care, Columbus, United States of America;
1 Pan American Health Organization, Nmh, Lima, Peru; 2 Pan American 5 Nationwide Children’s Hospital, Pediatric Oncology, Columbus, United
Health Organization, Nmh, Washington D.C., United States of America; States of America
3 Hospital Edgardo Rebagliati Martins, Oncology, Lima, Peru; 4 St. Jude Chil-

dren’s Research Hospital, Global Pediatric Medicine Department, Memphis, Background and Aims: Youth with pediatric cancer may have high
United States of America; 5 Ministry of Health of Peru, Direction Of Strategic symptom burden, and pain is nearly universal near end-of-life. How-
Interventions, Lima, Peru ever, few studies have examined child-report of symptoms and distress
relative to other reports. Thus, we examined concordance between
Background and Aims: In low-and middle-income countries, a lim- child- and proxy-report of the three most common symptoms in the
itation for optimal treatment of children with cancer and febrile context of advanced cancer.
neutropenia (FN) is the delay in administering the first dose of antibi- Methods: As part of a longitudinal study, families of youth with
otics. DoTT is a quality improvement project implemented in Peru and advanced cancer (physician estimated prognosis of <60%, relapse,
aligned to the WHO Global Initiative of Childhood Cancer or refractory disease), ages 5-25, were recruited from a large U.S.
Methods: This study was performed in a Peruvian tertiary care cen- children’s hospital. Parents and youth completed the Memorial Symp-
ter. We defined TTA as the time elapsed between patient′s arrival tom Assessment Scale (MSAS). Average symptom scores based on
to ER and administering the first dose of antibiotic. PTA is the time child-report (n=41), mother-report (n=55), and father-report (n=30)
elapsed between the onset of symptoms and arrival to the hospital. were compared using paired and independent samples t-tests. Pearson
The intervention consisted of a synchronous and asynchronous edu- correlations explored associations between symptom scores.
cational course for pediatricians and pediatric residents on managing Results: Youth reported fatigue (73%), drowsiness (73%), and pain
FN in children with cancer. We compared the TTA between 22 patients (66%) as their most common symptoms. Mothers reported their
admitted from October to November 2020 (after educational interven- child’s top symptoms were fatigue (73%), lack of appetite (71%),
tion) and baseline data of 99 patients admitted from 2020 January to and drowsiness (65%), while fathers reported pain (80%), nausea
2021 August (before intervention). (67%), and fatigue (66%). The mean child-reported MSAS was 1.93
Results: The median age was 5 years, 55 patients were female and 66 (SD=0.46), mother-reported MSAS was 1.98 (SD=0.46), and father-
were male. 104/121 patients had leukemia, and 17/121 patients had reported MSAS was 1.95 (SD=0.51). These scores were correlated
solid malignant tumors. No patients required admission to ICU or died between mother-child, r(29)=0.58, p<0.001; father-child, r(17)=0.63,
of sepsis in the pre-intervention group, in the post-intervention group p=0.01; and mother-father reports, r(20)=0.73, p<0.001. Symptom
one patient required admission to ICU and one patient died of sep- scores did not differ across mother-child, t(28)=-1.27, p=0.92; father-
sis. In the pre-intervention group (97 patients), the median TTA was child, t(16)=-0.11, p=0.21; and mother-father reports, t(19)=0.84,
171 minutes (IQR: 102–293), and in the post-intervention group, the p=0.41. Mothers reported higher symptom burden in girls than boys,
median TTA was 60 minutes (IQR: 35–73). Early results indicate a sta- t(53)=-2.44, p=0.01; Child age was unrelated to symptom scores
tistically significant decrease in the TTA (p=0.001). A long PTA was (p’s>0.05).
observed in both groups (before intervention, median= 1483 minutes) Conclusions: Although the most frequent symptoms varied by infor-
and (post-intervention, median=1290 minutes) (p=0.06). mant, fatigue, drowsiness, and pain were common among youth with
Conclusions: This study suggests the potential impact of an educa- advanced cancer, and there was general agreement on overall symp-
tional intervention in pediatric health care professionals to decrease tom burden. Future work should examine other factors associated with
the TTA in pediatric patients with cancer and FN. The PTA was symptom communication and concordance, as well as ways to improve
very long in both groups demonstrating the need for an additional symptom management. Clinicians should solicit multiple perspectives
intervention to improve these results. of the symptom experience for optimal care.

EP483 / #1711 CONCORDANCE IN SYMPTOM REPORTS EP484 / #433 PAEDIATRIC PALLIATIVE ONCOLOGY ACROSS
AMONG YOUTH WITH ADVANCED CANCER EUROPE: A CROSS-SECTIONAL SURVEY

Valdeoso Patterson1 , Anna Olsavsky1 , Kylie Hill1 , Malcolm Eddy Carolina Pedraza S.1 , Manya Hendriks1 , Eva De Clercq1 , Katja
Sutherland-Foggio1 , Dana Garcia1 , Alexandra Himelhoch2 , Ansley Rüesch1 , Eva Maria Tinner2 , Eva Bergstraesser3 , André O. Von
Kenney3 , Lisa Humphrey4 , Randal Olshefski5 , Micah Skeens1 , Leena Bueren4 , Lars Hjorth5 , Lori Wiener6 , Gisela Michel1
Nahata1 , Cynthia Gerhardt1 1 University of Lucerne, Department Of Health Sciences And Medicine,
1 The Abigail Wexner Research Institute at Nationwide Children’s Hospi- Lucerne, Switzerland; 2 Inselspital, University Children’s Hospital, Depart-
tal, Center For Biobehavioral Health, Columbus, United States of America; ment Of Paediatric Oncology And Hematology, Bern, Switzerland;
2 University of Florida, College Of Public Health And Health Professions, 3 University Children’s Hospital Zurich, University of Zurich, Department
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ABSTRACTS S373 of S635

Of Paediatrics, Paediatric Palliative Care, Zurich, Switzerland; 4 University Background and Aims: Children hospitalised following Haematopoi-
Hospital of Geneva, Department Of Pediatrics, Obstetrics And Gynecology, etic Stem Cell Transplantation (HSCT) experience complex and pro-
Division Of Pediatric Hematology And Oncology, Geneva, Switzerland; longed pain in response to the intensity of this treatment. The aim of
5 Lund University, Skane University Hospital, Department Of Clinical this study is to describe how pain is managed for children during HSCT
Sciences Lund, Paediatrics, Lund, Sweden; 6 National Cancer Institute, NIH, therapy and how contextual factors related to the clinical environment
Pediatric Oncology, Bethesda, United States of America influenced the pain management practices of healthcare providers and
parents.
Background and Aims: Despite substantial improvements in 5-year Methods: A longitudinal qualitative study was conducted and involved
survival rates, almost every fifth child diagnosed with cancer dies. It is naturalistic observations of pain-related care provided to children
increasingly recognized that palliative care (PC) should be an integral (n=29) during HSCT therapy by their healthcare providers (n=10).
part of comprehensive paediatric oncology care. The availability of PC Semi-structured interviews were conducted with healthcare providers
services in paediatric oncology settings is currently unknown. Aim: To (n=14) and parents (n=10) participated in semi-structured interviews
describe the availability of PC services in paediatric oncology centres at two timepoints following transplantation (30 and 90 days).
across Europe. Results: The effectiveness of pain management interventions was
Methods: We contacted paediatric oncology centres across Europe hindered by the multifactorial nature of pain children experienced
and invited one health care professional per centre with experience in and a lack of evidence-based guidelines for the sustained, and often
PC to complete an online questionnaire. long-term, administration of opioids and adjuvant medications with
Results: We included 151 paediatric oncology centres from 27 Euro- analgesic properties. Misconceptions were demonstrated about esca-
pean countries in our analyses. Most centres (97.3%) offer either lating pain management according to pain severity and differentiating
general (32%) or specialized paediatric palliative care (PPC) services between opioid tolerance and addiction. Parents had an active role to
(65.3%). The majority have multidisciplinary care teams (79.5%) and play in the management of pain for children, especially the provision of
offer PC at home (69.5%). Current service capacity is reported lower non-pharmacological interventions. Collaboration with external pain
than demand in 39.1% centres. In most centres (82.1%) PC consultation services and the impact of caring for children in protective isolation
is initiated in case of a refractory neoplasm, or high symptom burden delayed the timely management of pain.
(60.3%). Very few centres (7.3%) offer PC consultation at the time of a Conclusions: There is a pressing need for the creation of evidence-
new cancer diagnosis. Bereavement services including phone calls by based supportive care guidelines for the management of pain post-
team members after the child’s death (68.2%), on-site psychological transplantation to optimise children’s relief from pain. If parents and
counselling (62.9%) and medical debriefing (61.6%) were reported to children are to be involved in how pain is managed, then greater efforts
be an integral part of PC. need to be directed towards building their capacity to make informed
Conclusions: Although caution is warranted due to potential self- decisions.
report bias, results indicate that while PPC are available in most pae-
diatric oncology centres, many cannot completely fulfil the demand.
Distinguishing between common paediatric oncology services and EP486 / #498 CARBAPENEM RESISTANT ENTEROCOCCI
more individualized PPC ones is difficult. Reducing suffering through- (CRE): AN IMPEDIMENT IN THE CARE OF PAEDIATRIC
out the disease process should remain the standard of care for all HEMATOLOGY ONCOLOGY AND HEMATOPOIETIC STEM CELL
children with cancer regardless of disease outcome. Identification of TRANSPLANTATION (HSCT) RECIPIENTS
children and families with the highest symptom burden and early refer-
ral to PC could help meet this standard. Funding: SSPH+ GlobalP3PH Priyank Rajan1 , Ruchira Misra1 , Sujata Mushrif1 , Shaheen Shaikh2 ,
Programme (Marie Curie Grant Agreement Number 801076), Swiss Purna Kurkure1
Cancer League: KFS-4995-02-2020. 1 NH SRCC Children’s Hospital, Paediatric Oncology, Mumbai, India; 2 SRCC

Children’s Hospital, Micobiology, Mumbai, India

EP485 / #646 THE MANAGEMENT OF PAIN FOR CHILDREN Background and Aims: Gram-negative bacteria including CRE, are
HOSPITALISED DURING HAEMATOPOIETIC STEM CELL an important cause of life-threatening infections in children with
TRANSPLANTATION haematological malignancies and HSCT recipients.The prevalence of
CRE related infections is increasing worldwide.This study assesses
Karin Plummer1 , Maria Mccarthy2 , Fiona Newall3 , Elizabeth Manias4 prevalence and outcomes of CRE septicaemia in children undergoing
1 University of Melbourne, School Of Nursing, Parkville, Australia; 2 Murdoch treatment in our unit.
Childrens Research Institute, Social And Mental Health Aspects Of Serious Methods: A retrospective observational study was conducted in the
Illness, Critical Care And Neurosciences, Melbourne, Australia; 3 Royal Chil- department of Paediatric Haematology Oncology & HSCT at SRCC
dren’s Hospital Melbourne, Nursing Research, Parkville, Australia; 4 Deakin Children’s Hospital, managed by Narayana Health. Stool samples for
University, School Of Nursing And Midwifery, Burwood, Australia CRE screening were sent for all high risk patients [Pre HSCT, high risk
Acute Leukaemia(AL), AL in relapse]. The samples were processed on
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S374 of S635 ABSTRACTS

MacConkey’s agar as per standard microbiological methods. Identifi- blood components and G-CSF. Outcome measures: 28d mortality,
cation of organisms and antimicrobial susceptibility (AS) testing were duration of hospital stay, antibiotic days, day of defervescence and
performed as per CLSI guidelines. AS was used as a guideline to start adverse events
antibiotics during febrile neutropenic (FN) episodes. Results: Sixty children were enrolled (GT, n=30; ST, n=30). Demo-
Results: A total of 422 stool samples from 126 patients (M: F- graphic characteristics and baseline investigations were compara-
76:50), mean age 7.68 years (2-17years) were sent from January ble. There was no significant difference in mortality [8(26.7%) v/s
2019 to December 2021. Stool samples were positive for CRE in 2(6.7%); p=0.08], duration of hospital stay [11d(8-15) v/s 9d(6-14.2);
57/126(45.2%). Both stool and blood cultures were positive for CRE in p=0.51] and antibiotic days [23d(15.7-30.5 ) v/s 19d(12.7-28 ); p=0.18]
24/57(42.1%) children during febrile neutropenia suggesting translo- between the 2 groups. Total febrile period before admission [3d(1-
cation from gut. There were 19(79.1%) deaths in the CRE- positive 5.25) v/s 3d(1-7)] and day of defervescence were similar [8d(5-12)
group- 7 patients had CRE positivity in both stool and blood (6-post v/s 8d(5-11), p=0.86]. However, days to achieve ANC >500/mm3 was
HSCT and 1-ALL). 10 deaths were non CRE related infectious reasons, significantly lower in the GT arm [4.5d(3-6.5) v/s 8d(4-11); p=0.01].
mainly fungal/Methicillin Resistant Staphylococcus Aureus(MRSA) and Microbiologically documented infection [9(30%) v/s 7(23%); p=0.77],
2 were disease related. The mean duration after initiation of antibiotics radiologically defined pneumonia [10(33%) in each], volume over-
till death in CRE positive blood cultures was 46 hours (SD ± 25.5 hours). load (4(13.3%) v/s0; p=0.11] and Transfusion related adverse events
There were 18 deaths in the CRE- negative group- 6 deaths were due [1(3.3%) v/s 0, p=1] did not differ significantly.
to sepsis and 12 were disease related conditions, usually relapse. Conclusions: Granulocyte transfusion helped in early neutrophil recov-
Conclusions: CRE infections are potentially fatal especially in post ery but survival benefit could not be attained. Lesser efficacy of GT may
HSCT conditions. Those with both stool and blood cultures positive are be secondary to prolonged febrile period prior to admission. Buffy coat
at a higher risk. Aggressive escalation to higher antibiotics and other derived GT is safe to administer in children with high risk FN.
supportive measures may help to decrease mortality.

EP488 / #875 PERFORMANCE OF THE GOLDEN HOUR


EP487 / #1537 EFFECT OF BUFFY COAT DERIVED PROTOCOL IN FEBRILE NEUTROPENIC PATIENTS AS AN
GRANULOCYTE TRANSFUSIONS IN CHILDREN WITH POST INDICATOR OF THE QUALITY OF CARE; CHANGE AND
CHEMOTHERAPY HIGH RISK FEBRILE NEUTROPENIA IN ADAPTATION DURING THE COVID-19 PANDEMIC
DECREASING MORTALITY –AN OPEN LABEL RANDOMISED
CONTROL TRIAL Jocelyn Rivera1 , Rosa Cabello Alvarado2 , Yasmin Matlalcuatzi
Escobar2 , Gloria Rangel Aranda2 , Leonel Cruz Martínez2 , Francisco
Mohanaraj Ramachandran1 , Aditya Gupta2 , Jagdish Prasad Meena3 , Mendoza Nicio2 , Karla Arciniega Vega2 , María García Tapia2 , Elvia
Rachna Seth4 , Poonam Coshic5 , Rakesh Lodha1 Alarcon Sanchez2 , Jimena López Alvarez2 , Ana Acevedo Arroyo2 ,
1 All India institute of medical sciences, Pediatrics, Newdelhi, India; 2 All India Graciela Conde Galicia2 , Miriam Avila Cuevas2 , Yamili Serrano
Institute of Medical Sciences, Pediatric Oncology, New Delhi, Delhi, India; Cabrera2 , Emmanuel Miranda Rosas3 , Erika Martínez Pérez3 , Fanny
3 All India Institute of Medical Sciences New Delhi, Division Of Pediatric García Rivera3 , Ada Torres Albrincula3 , Ana Sánchez Núñez3 , Maria
Oncology, Department Of Pediatrics, New Delhi, India; 4 All India Institute Perianez Romero3 , Cinthia Hernández González4 , Gabriela Escamilla
of Medical Sciences- New Delhi, Division Of Pediatric Oncology, Depart- Asiain5
ment Of Pediatrics, New Delhi, India; 5 All India Institute of Medical Sciences, 1 Hospital Infantil Teleton de oncologia, Emergency Medicine, Queretaro,
Transfusion Medicine, New Delhi, India Mexico; 2 Hospital Infantil Teleton De Oncologia, Nursing, Queretaro, Mex-
ico; 3 Hospital Infantil Teleton de oncologia, Nursing Supervisor, Queretaro,
Background and Aims: Background: Granulocyte transfusion (GT) in Mexico; 4 Hospital Infantil Teleton de oncologia, Chief Nurse, Queretaro,
febrile neutropenia (FN) may help in marrow recovery and reduce Mexico; 5 Hospital Infantil Teleton de oncologia, Medical Director, Queretaro,
morbidity and mortality by replenishing the deficient granulocytes to Mexico
combat infection. Aims: To study the effect of addition of irradiated
buffy coat derived GT to the standard of care in reducing the 28d Background and Aims: The COVID-19 pandemic posed extraordi-
mortality in children with chemotherapy associated high risk FN. nary challenges to emergency departments (ED) worldwide. These
Methods: Design: Randomised Control trial Duration: Jul’20- Mar’22 challenges could have jeopardized previously developed quality indi-
Setting: Tertiary care teaching institute in northern India Partici- cators, mainly time-dependent outcomes. This study aimed to analyze
pants: Children ≥18y with malignancy and chemotherapy induced whether pandemic-related time constraints and the use of personal
high risk FN. Palliative care, hematopoietic stem cell transplantation, protective equipment (PPE) affected golden hour adherence for febrile
recovery phase and non-availability of group specific granulocyte, neutropenic patients.
were exclusions. Interventions: Irradiated buffy coat derived GT at Methods: In 2019, we implemented a quality improvement method-
10ml/kg every 48h till afebrile for >24h and Absolute neutrophil ology to improve outcomes in pediatric oncology patients with febrile
count(ANC)≥500/mm3 . Standard therapy(ST) included antimicrobials, neutropenia. This methodology fosters multidisciplinary collaboration
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S375 of S635

that enhances results and increases staff awareness of the importance were found in 26/29 (90%) patients. Vitamins A, D, and E were low in 6
of this protocol to patient prognosis. For this analysis, we included (21%), 13 (45%) and 0 patients at diagnosis and in 6 (21%), 11 (38%) and
all febrile neutropenic patients treated in the ED from June 2020 to 1 (3%) patients at 6 months. Cu, Se and Zn were abnormal in 4 (14%),
December 2021 to compare the outcomes of patients who required 5 (17%) and 13 (45%) patients at diagnosis and in 2 (7%), 8 (28%) and
wearing PPE due to suspected covid-19 and those who did not require 15 (52%) patients at 6 months. Albumin was abnormal in 6/16 (38%)
the use of special equipment. patients at diagnosis and 4/7 (57%) of patients at 6 months.
Results: The study population consisted of 92 febrile neutropenic Conclusions: These results suggest that micro-nutrient abnormalities
patients. Of these, 21 patients (22.8%) came from their homes and are common throughout chemotherapy. This provides insight on the
were treated as suspects of COVID-19; 71 patients (77.2%) came from potential worsening of short and long-term adverse events associated
the hospital’s hospice and were not suspected due to the installed iso- with chemotherapy.
lation. Global adherence was reported in 91.3% (84). The adherence in
suspected patients was 85.7% (18) and 92.9% (66) in the control group.
The mean time from triage to antibiotic was 49.9 min and 42.1 min, EP490 / #1023 THE IMPACT OF FERTILITY GUIDELINES AND
respectively. In patients who had a delayed antibiotic administration EDUCATION ON THE RATE OF PRE-THERAPY FERTILITY RISK
time in the suspicious group (3 patients), the leading cause for delay DISCUSSIONS IN PEDIATRIC ONCOLOGY: A RETROSPECTIVE
was difficult intravenous access. COHORT STUDY
Conclusions: Despite challenges and time constraints with suspected
COVID-19 patients, global adherence to the golden hour was main- Cameron Crowell1 , Carol Digout1 , Rodrigo Romao2 , Annette
tained in our patients. The quality improvement methodology helped Flanders1 , Maryjean Howitt1 , Craig Erker1
create lasting staff awareness and collaboration on the importance of 1 IWK Health Centre, Pediatrics, Halifax, Canada; 2 IWK Health Centre,
the protocol, and even in difficult times, this made a difference for our Dalhousie University, Departments Of Surgery And Urology, Halifax, Canada
patients.
Background and Aims: Pediatric cancer patients undergoing treat-
ment are often at risk for infertility or subfertility. Healthcare providers
EP489 / #413 MICRONUTRITION EVALUATION AT TIME OF do not consistently deliver fertility information prior to therapy. Our
A PEDIATRIC CANCER DIAGNOSIS AND DURING objective was to assess if development and implementation of a fertility
CHEMOTHERAPY guideline improves the frequency of pre-treatment fertility discussions
with pediatric cancer families.
Lisi Dredze, Jeffrey Cheng, Jim Potts, Alecia Lim, Paul Rogers Methods: This retrospective cohort study analyzed all consecutively
BC Children’s Hospital and the University of British Columbia, Division Of eligible patients over two time periods. The pre-guideline time period
Pediatric Hematology/oncology/bmt, Vancouver, Canada was 18 months prior to implementation and the post-guideline time
period was 12 months after implementation. Guideline implementa-
Background and Aims: Many children with cancer undergoing tion included education with oncology staff. Eligible patients were <
chemotherapy experience adverse events that could be exacerbated 19 years old at cancer diagnosis and received chemotherapy and/or
due to micro-nutrient abnormalities. We conducted a prospective pilot radiation therapy. Pre-guideline rate of pre-treatment fertility dis-
study to evaluate micro-nutrient and body mass at diagnosis and after cussions was estimated at 40%. An estimated 40 participants in the
6 months of chemotherapy. post-guideline cohort can detect a 30% increase in fertility discussions
Methods: Informed consent/assent were obtained. Height and weight with β 0.8 and α 0.05. Exploratory analysis assessed factors associated
were measured and body mass index (BMI) and corresponding Z-scores with fertility discussions.
were calculated. Blood samples were collected as part of routine blood Results: Ninety-five patients were included. Fifty-seven percent were
work and analyzed for vitamins A, D, E and trace elements copper (Cu), < 10, 26% were 10-15, and 17% were ≥ 16 years old. Pre- and post-
selenium (Se) and zinc (Zn). guideline cohorts are compared in table 1. In the pre-guideline cohort,
Results: Twenty-nine patients (16 male, 55%) with a median (range) 41% of patients had a discussion about risks of fertility impairment
age of 8.2 (1.7-17.6) years participated. Diagnoses included AML documented, while post-guideline cohort had 49% documented (p =
(n=1), Alveolar Rhabdomyosarcoma (n=1), Astrocytoma (n=1), B-Cell 0.531). Exploratory assessment of combined cohorts found males (OR
ALL (n=7), Burkitt Lymphoma (n=3), Ependymoma (n=1), Epithelioid 0.2.7, 95% CI 0.1.2-6.3-0.87) and patients ≥ 10 years old (OR 5.0,
Sarcoma (n=1), Ewing Sarcoma (n=4), Germinoma (n=1), Hodgkin CI 2.1-12.1) were more likely to receive fertility discussions prior to
Lymphoma (n=1), LCH (n=1), Medulloblastoma (n=2), Neuroblastoma therapy. Radiation and cancer type did not influence whether fertility
(n=2), Osteosarcoma (n=1), T-Cell ALL (n=1), and Wilms Tumour discussions occurred.
(n=1). The median BMI was 17.2 (14.4-26.4) kg/m2 and 16.6 (14.0- Conclusions: Implementation of a fertility guideline did not increase
27.7) kg/m2 at diagnosis and after 6 months of chemotherapy, respec- fertility discussions, and less than half of patients had a documented
tively. Fifty-nine and 52% of patients had BMI Z-scores of -1 or lower or fertility discussions. Male patients and age ≥ 10 years old were more
+1 or greater at diagnosis and at 6 months. Micronutrient deficiencies likely to have documented fertility discussions. Effective strategies are
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S376 of S635 ABSTRACTS

needed to improve the rate of discussions regarding fertility risk to EP492 / #695 PEDIATRIC ONCOLOGY SUPPORTIVE CARE IN
ensure families receive this information prior to therapy. INDIA DURING PANDEMIC TIMES: WHAT WE LIVED WITH
AND WHAT WE LEARNT?

EP491 / #1556 TO PREMEDICATE OR NOT? A LOOK AT Nirmalya Roy Moulik, Maya Prasad, Girish Chinnaswamy
PREMEDICATIONS FOR BLOOD PRODUCT TRANSFUSIONS AT Tata Memorial Centre, Paediatric Oncology, Mumbai, India
MSK KIDS
Background and Aims: We conducted a survey among practicing pedi-
Jaclyn Rosenzweig1 , Alexandre Troullioud Lucas1 , Sanam Shahid1 , atric oncologists to assess the modifications made in supportive-care
Sheila Mcthenia1 , Christina Fong1 , Melanie Degliuomini1 , Shipra during the pandemic, specifically if any of those were safe and effective
Kaicker2 , Emily Slotkin1 enough to be practice-changing.
1 Memorial Sloan Kettering Cancer Center, Pediatrics, New York, United Methods: A survey-questionnaire with 27 questions was circulated
States of America; 2 Weill Cornell Medical College, Pediatrics, New York, through the emailing list and WhatsApp/Telegram groups of Indian
United States of America pediatric oncology group in January 2022. Responses were accepted
till 31st March 2022. The questions focused on disruptions in contin-
Background and Aims: Blood product transfusions are given often uation of patient-care over past two years, strategies to minimize the
in pediatric oncology patients. Transfusion-related reactions, though impact of such disruptions, and the potential, if any, for incorporating
uncommon, range in severity from mild, allergic to severe, ana- these modifications into standard practice.
phylactic. Though lacking robust evidence, many patients receive Results: Of seventy-one responses from approximately 250 active
prophylactic premedications to prevent transfusion reactions, lead- members contacted, 39(55%) were from public hospitals and 23(32%)
ing to practice variability, unnecessary treatments, and additional from centers seeing >200 new cases/year. Decline in new patient
costs. We analyzed blood product premedication practices at MSK registration, funding shortage, increase in treatment abandonment
Kids. and delay in maintenance/follow-up visits were reported by 7(9.8%),
Methods: Our retrospective, single-institution review included all 37(52%),44 (62%), and 52(73%). In (25)35.2% centers, scarcity of ICU
pediatric patients who received a blood product transfusion at beds during COVID waves resulted in higher non-COVID mortal-
MSK Kids from January to April 2021, excluding patients with a ity/morbidity. Several centers reduced transfusion cut-offs (23,33%),
complex transfusion reaction history. Data extracted included: demo- used granulocyte stimulating factors more often (21, 30%), increased
graphics, transfusion type (platelet and/or red blood cell (RBC)), use of oral antibiotics in low-risk febrile neutropenia(FN) (29,40%), and
reaction history, premedication(s) administered (acetaminophen, stopped intravenous antibiotics earlier (11,15%). Strategies to curtail
antihistamine, and/or hydrocortisone), and signs/symptoms of abandonment and drug default included tracking phone calls (50,72%),
reactions. couriering medicines to patients’ homes (27,39%) and teleconsulta-
Results: 112/116 patients who received a blood product transfusion tion (43,62%). Post–treatment follow-up frequency and investigations
met inclusion criteria. Median age at transfusion was 8.2 years (range were reduced in 50(70%) centers and 54(76%) started teleconsulta-
0.6 – 17.8). A total of 603 transfusions were given with a median tions; respondents considered these strategies likely to be incorpo-
of 3 transfusions per patient (IQR 2-7). This included 315 (52.2%) rated into routine practice. While 35(49%) respondents supported
platelets and 288 (47.7%) RBCs. Premedications were given for 434 increased use of outpatient chemotherapy, most(70,99%) respondents
(72.0%) transfusions: acetaminophen monotherapy (n=77), diphen- chose to revert to pre-pandemic policies for transfusion and FN.
hydramine monotherapy (n=11), or combination therapy (n=346). Establishment of sustainable shared-care networks was considered a
Of the 34 patients with a transfusion reaction history, 212/217 priority by 44(62%).
(97.7%) of their transfusions were premedicated. 78 patients had no Conclusions: Pediatric oncology services were remarkably compro-
history of a transfusion reaction, yet 222/386 (57.5%) of their trans- mised during the pandemic. Of the many adaptations made to tackle
fusions were premedicated. 19/603 (3.2%) transfusions resulted in the pandemic conditions, virtual follow-up of selected patients and
a reaction including: rash/pruritus (n=17), fever (n=6), respiratory rationalizing post-treatment follow-up and investigations are likely to
symptoms (n=3), and/or hypotension (n=1). No transfusions caused continue into the post-pandemic period.
anaphylaxis. There was no difference in the incidence of transfu-
sion reactions between premedicated (3.2%) and not premedicated
patients (3.0%). No severe sequelae or intensive care unit admissions EP493 / #1658 DEVELOPMENT OF A FACILITY REPORT
occurred. HIGHLIGHTING STRENGTHS AND LIMITATIONS IN PEDIATRIC
Conclusions: Our cohort experienced a low incidence of transfusion ONCO-CRITICAL CARE SERVICES IDENTIFIED USING THE
reactions. Most patients received premedication(s), despite no history PROACTIVE ASSESSMENT
of transfusion reactions, with no clear benefit. An evidence-based algo-
rithm and provider education are needed for blood product transfusion Firas Sakaan1 , Maria Puerto-Torres1 , Zebin Al Zebin2 , Parthasarathi
premedication in pediatric oncology patients. Bhattacharyya3 , Sanjeeva Gunasekera4 , Joyce Kambugu5 , Jaime
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S377 of S635

Libes6 , Angelica Martinez7 , Alejandra Méndez-Aceituno8 , Hong Ren9 , EP494 / #1551 FACTORS CONSIDERED BY PEDIATRIC
Rana Sharara-Chami10 , Silvio Torres11 , Asya Agulnik1,12 , Anita Arias12 ONCOLOGISTS GLOBALLY WHEN APPROACHING TREATMENT
1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, mem- DECISION-MAKING AT DIAGNOSIS
phis, United States of America; 2 3. Pediatric Hematology and Oncology,
King Hussein Cancer Cente, Department Of Critical Care, Amman, Jordan; Marta Salek1 , Amy Porter2 , Lisa Force3 , Dylan Graetz4 , Jean Hunleth5 ,
3 Tata Medical Center, 5. department Of Pediatric Oncology Critical Care, Paola Friedrich6 , Justin Baker2 , Nickhill Bhakta7 , Erica Kaye2
Kolkata, India; 4 National Cancer Institute, Paediatric Oncology, Ethul Kotte, 1 St. Jude Children’s Research Hospital, Department Of Oncology, Memphis,

Sri Lanka; 5 Uganda Cancer Institute, Pediatric Oncology, Kampala, Uganda; United States of America; 2 St. Jude Children’s Research Hospital, Division
6 University of Illinois College of Medicine, Department Of Pediatric Hema- Of Quality Of Life And Palliative Care, Department Of Oncology, Memphis,
tology And Oncology, Peoria, United States of America; 7 Hospital General United States of America; 3 University of Washington School of Medicine,
de Tijuana, Pediatric Hemato-oncology Unit, Tijuana, Mexico; 8 National Seattle Children’s Hospital, Department Of Health Metrics Sciences, And
Unit of Pediatric Oncology, UNOP, Pediatric Critical Care, Guatemala, Department Of Pediatrics, Seattle, United States of America; 4 St. Jude
Guatemala; 9 Shanghai Children’s Medical Center, Department Of Pediatric Children’s Research Hospital, Department Of Global Pediatric Medicine,
Intensive Care Unit, Shanghai, China; 10 American University of Beirut Med- Memphis, United States of America; 5 Washington University School of
ical Center, Department Of Pediatrics And Adolescent Medicine, Beirut, Medicine in St. Louis, Division Of Public Health Sciences, St. Louis, United
Lebanon; 11 Hospital Universitario Austral, Pediatric Intensive Care Unit, States of America; 6 St. Jude Children’s Research Hospital, Global Pediatric
Buenos Aires, Argentina; 12 St. Jude Children’s Research Hospital, Division Medicine, Memphis, United States of America; 7 St Jude Children’s Research
Of Pediatric Critical Care, Memphis, United States of America Hospital, Global Pediatric Medicine, Memphis, United States of America

Background and Aims: PROACTIVE is an electronic assessment tool Background and Aims: Global childhood cancer outcomes are inextri-
arranged into 8 modules, each divided into 2 surveys for intensivists cably linked with available local healthcare infrastructure and cultural
and oncologists managing critically ill pediatric hematology-oncology environments. Yet existing guidelines and resources inconsistently
(PHO) patients. The tool was designed to evaluate strengths and account for specific contextual factors that physicians consider when
limitations in pediatric oncology critical care (POCC) services in making decisions about cancer-directed therapy. Importantly, treat-
resource-limited settings. Our objective is to describe the development ment misaligned with local capacity can lead to patient harm and
of a center-specific report to facilitate interpretation of PROACTIVE compromise health system efficiency and effectiveness. In this study,
results. we sought to understand factors considered by physicians in low-
Methods: The PROACTIVE pilot (January 2021 – June 2021) was con- and middle-income countries (LMICs) when deciding to offer curative
ducted in 10 centers from 10 different countries with a wide range of treatment versus non-curative treatment to children presenting with
POCC services. Surveys were administered in English using REDCap. advanced or incurable cancer at the time of diagnosis.
Collected data were used to create center-specific score-based and Methods: To identify and define factors impacting decision-making, we
descriptive reports highlighting areas of strengths and opportunities used a community-engaged research approach, inviting a global panel
for improvement in POCC services. The clarity, user-friendliness, and of pediatric oncology experts to participate in sequential focus groups.
format of the reports were assessed in 2 focus group meetings with In two initial focus groups, physicians were asked to list factors related
pilot center PROACTIVE leads. Adjustments were made to the reports to the disease, the decision-maker, and other contextual factors. Con-
based on feedback to improve the presentation of PROACTIVE results. tent analysis of focus group data informed the development of a visual
Results: A total of 20 surveys from 10 centers were analyzed and model and semi-structured interview guide, which the global panel
used to develop the PROACTIVE reports. Recommended adjust- reviewed together in the subsequent two focus group sessions.
ments to the reports resulted in a combined score for each mod- Results: 11 pediatric oncologists representing all WHO regions par-
ule, including the results from both the intensivist and oncolo- ticipated, comprising 11-21+ years of medical experience. Participants
gists’ surveys. A separate area highlights the top 5-highest and identified a broad range of factors influencing decision-making, includ-
lowest scored areas to allow easier interpretation of a center’s ing variables unique to LMIC settings such as ability to access diag-
strengths and weaknesses. In focus groups, PROACTIVE reports were nostic tools and interventions, unclear referral pathways, and effects
described as practical and were used by several pilot centers to advo- of war or political instability. Physicians often defaulted to offering
cate for additional resources to improve intensive care and POCC curative therapy even when they recognized that intensive treatment
capacity. would result in excess toxicities and poor outcomes. Member-checking
Conclusions: Center-specific reports facilitated interpretation of yielded consensus development of a semi-structured interview guide
PROACTIVE results and allowed organizations to prioritize multiple to further probe these critical E-Poster Topics with LMIC physicians in
improvement opportunities and benchmark their performance against future work.
others. Global implementation and use of PROACTIVE reports high- Conclusions: Physicians in LMICs face unique factors and challenges
lighting challenges in POCC services can help improve the quality of that influence treatment decision-making. Treatment guidelines should
care and outcomes for critically ill PHO patients in resource-limited account for these factors and offer direction for treatment pathways
settings. supporting curative and non-curative therapies.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S378 of S635 ABSTRACTS

EP495 / #2043 FACTORS INFLUENCING PEDIATRIC views may inform development of educational interventions and
ONCOLOGISTS’ DECISION-MAKING WHEN BALANCING shape guideline development to better support oncologist treatment
CURATIVE AND NON-CURATIVE TREATMENT OPTIONS AT decision-making in LMICs.
DIAGNOSIS IN LOW- AND MIDDLE-INCOME COUNTRIES
(LMICS)
EP496 / #1705 PROSPECTIVE EVALUATION OF OTOTOXICTY
Marta Salek1 , Amy Porter2 , Lisa Force3 , Dylan Graetz4 , Jean DURING PLATINUM CHEMOTHERAPY AMONG PAEDIATRIC
Hunleth5 , Paola Friedrich4 , Justin Baker2 , Nickhill Bhakta4 , Erica PATIENTS
Kaye2 , Catalyst Study Group6
1 St. Jude Children’s Research Hospital, Department Of Oncology, Memphis, Ajay Sankar1 , Priyakumari Thankamony1 , Preethi George2 , Manjusha
United States of America; 2 St. Jude Children’s Research Hospital, Division Nair3 , Binitha Rajeshwari3 , Guruprasad Cs3 , Prasanth Vr3
Of Quality Of Life And Palliative Care, Department Of Oncology, Memphis, 1 REGIONAL CANCER CENTRE, Pediatric Oncology, THIRUVANATHAPU-
United States of America; 3 University of Washington School of Medicine, RAM, India; 2 Regional Cancer Centre, Cancer Epidemiology And Bio-
Seattle Children’s Hospital, Department Of Health Metrics Sciences, And statistics, TRIVANDRUM, India; 3 Regional Cancer Centre, Department Of
Department Of Pediatrics, Seattle, United States of America; 4 St. Jude Pediatric Oncology, Thiruvananthapuram, India
Children’s Research Hospital, Department Of Global Pediatric Medicine,
Memphis, United States of America; 5 Washington University School of Background and Aims: The platinum chemotherapy agents, cisplatin
Medicine in St. Louis, Division Of Public Health Sciences, St. Louis, United and carboplatin are widely used in the treatment of paediatric cancers.
States of America; 6 St. Jude, Onc, Memphis, United States of America Cisplatin causes hearing loss in at least 60% of paediatric patients. In
a young child, this will have a detrimental effect on speech, language,
Background and Aims: Global childhood cancer outcomes are inex- and social development. The study aims to identify the risk of ototoxi-
tricably linked to local and regional economic and social contexts, city associated with platinum compounds and to assess the risk factors
including available healthcare infrastructure and psychosocial support. associated with them.
Treatment misaligned with local capacity can lead to patient harm and Methods: Prospective hospital based observational study of 22
compromise health system efficiency and effectiveness. In this study, patients who received platinum based chemotherapy for newly diag-
we sought to understand factors considered by physicians in LMICs nosed malignancies between 01.03.2020 and 31.08.2021. Children
when deciding how to balance curative and non-curative treatment were assessed with audiometry at baseline and middle of chemother-
options for children presenting with advanced or incurable cancer at apy if feasible and after completion of treatment. Cases with normal
the time of diagnosis. baseline hearing were included in the study. Ototoxicity was diagnosed
Methods: To identify and define factors impacting decision-making, and graded according to SIOP- Boston Ototoxicity Scale.
we used a community-engaged research approach, inviting a global Results: Twenty two cases were included in the study. Seventeen chil-
panel of pediatric oncology experts to participate in four sequential dren received cisplatin (77.3%) while 4 received carboplatin (18.2%)
focus groups. In two initial focus groups, participants were asked to and one (4.5%) received both. Median cumulative dose of cisplatin
identify factors related to the disease, the decision-maker, and addi- was 540 mg/m2 while median cumulative carboplatin dose was 2400
tional contextual factors impacting care. Content analysis of focus mg/m2 . Eighteen cases (81.8%) had ototoxicity [Grade 1: n= 3 (16.6%),
group data informed the development of a visual model and semi- Grade 2: n=6 (33.3%), Grade 3: n= 5(27.2%), Grade 4: n= 4(22.2%)].
structured interview guide to further probe these E-Poster Topics with All 9 children who received radiation to head developed ototoxicity
LMIC physicians, which the working group reviewed together in the and all children with acute malnutrition developed ototoxicity, how-
subsequent two focus group sessions. ever, these associations were not significant. Univariate binary logistic
Results: Eleven pediatric oncologists representing all WHO regions regression revealed significant association of ototoxicity with cumula-
participated. Participants identified numerous factors influencing tive cisplatin dose, cisplatin dose per cycle, cumulative cisplatin dose >
decision-making, confirmed these major categories, and identified 400 mg/m2 . Carboplatin, use of vinca alkaloids, hypomagnesemia were
numerous additional factors such as the ability to access diagnos- not associated with ototoxicity.
tic tools and treatment interventions, lack of established referral Conclusions: Cumulative cisplatin dose, especially dose >400 mg/m2 is
pathways, and financial compromises to treat their child at expense associated with ototoxicity. Close audiological monitoring of children
of the health of the family. Participants recognized that intensive on cisplatin based chemotherapy is warranted.
treatment often resulted in excess toxicities and poor outcomes, yet
they defaulted to offering curative therapy due to perceived lack of
alternatives and lack of direction from available guidelines. Member- EP497 / #1062 MUSCLE STRENGTH, CARDIORESPIRATORY
checking with the working group yielded consensus on semi-structured FITNESS AND PHYSICAL PERFORMANCE IN CHILDREN AND
interview prompts for future work. ADOLESCENTS WITH NEWLY DIAGNOSED CANCER: A
Conclusions: Physicians in LMICs face unique factors and challenges SYSTEMATIC REVIEW AND META-ANALYSIS
that influence treatment decision-making. Findings from future inter-
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ABSTRACTS S379 of S635

Peter Schmidt-Andersen1,2 , Anna Pouplier2 , Anders Larsen3 , Klaus Kruger1 , Emmanuel Adonga6 , Piera Freccero7 , Roberta Fregolent7 ,
Müller2,4 , Jan Christenen1 , Martin Fridh2 Eugene Asagba6 , Saviour Bruce6 , Lorna Renner8
1 Copenhagen University Hospital, Rigshospitalet, Department Of Occupa- 1 Stellenbosch University, Paediatrics And Child Health, Stellenbosch, South

tional And Physiotherapy, Centre Of Head And Orthopedics, Copenhagen, Africa; 2 Greater Accra Regional Hospital, Child Health, Accra, Ghana;
Denmark; 2 Copenhagen University Hospital, Rigshospitalet, Paediatric 3 Columbia University Irving Medical Center, Division Of Pediatric Hema-
Oncology Research Laboratory, Department Of Paediatrics And Adolescent tology/oncology/stem Cell Transplant, New York, United States of Amer-
Medicine, The Juliane Marie Center, Copenhagen, Denmark; 3 Copenhagen ica; 4 ITACI, Instituto da Criança do HCFMUSP/ University of São Paulo,
University Hospital, Rigshospitalet, University Hospitals Centre For Health Hematology-oncology, São Paulo, Brazil; 5 International Initiative for Pedi-
Research (ucsf), Copenhagen, Denmark; 4 University of Copenhagen, Insti- atrics and Nutrition, Division Of Pediatric Hematology, Oncology, & Stem
tute Of Clinical Medicine, Copenhagen, Denmark Cell Transplant, New York, United States of America; 6 World Child Cancer,
Childhood Cancer, Accra, Ghana; 7 World Child Cancer, Programmes, Lon-
Background and Aims: Anti-cancer treatment impairs muscle don, United Kingdom; 8 Korle Bu Teaching Hospital, Child Health, Korle Bu,
strength, cardiorespiratory fitness, and physical performance in chil- Accra, Ghana
dren with cancer throughout the treatment trajectory. However, it is
sparsely investigated how early these impairments occur. We aimed to Background and Aims: The SIOP Global Health nutrition commit-
provide an overview of current evidence regarding muscle strength, tee (2017) administered a survey among Pediatric Oncology Units
cardiorespiratory fitness, and physical performance status of children (POUs), located in Africa, to determine the existing nutritional ser-
with newly diagnosed cancer compared with healthy controls. vices and unmet needs. The most common reported barrier was
Methods: We performed a systematic search in five scientific a lack of education in nutritional assessment and intervention. A
databases, for scientific literature published before January 3, 2021. 2022 survey of Ghanaian POUs found that 90% of POUs were
Studies were eligible if they contained objective measures of muscle merely at levels 1-2 in terms of nutrition capacity. A partnership
strength, cardiorespiratory fitness, or physical performance of children between World Child Cancer (WCC) and the International Initia-
and adolescents (age 6-18) diagnosed with cancer within the first 31 tive for Nutrition and Paediatrics (IIPAN) aims to increase nutri-
days from treatment initiation and reported a comparative analysis to tional knowledge among clinicians caring for children with cancer in
norm-values and/or a study group of healthy sex and aged-matched Ghana.
controls. When possible, random-effects meta-analyses were used to Methods: WCC and IIPAN held a five-day nutritional work-
synthesize the results. shop in Accra, Ghana from 7 to 11 March 2022. Attendees
Results: The search identified 9,036 unique study references. Five included paediatric oncologists, paediatricians, dieticians, nutri-
studies; two RCTs, two quasi-experimental, and one cross-sectional tionists, nursing staff and WCC personnel. The first three days
study, embodying 330 participants, were included for preliminary anal- addressed theory and clinical practice, followed by a two-day prac-
ysis. Of these studies, three reported muscle strength, one reported tical exercise at Korle Bu Teaching and Greater Accra Regional
measures of cardiorespiratory fitness, and all reported physical per- Hospitals in Accra. A standardised questionnaire was com-
formance. The meta-analysis showed a lower muscle strength within pleted by all participants to test pre-training and post-training
the first 31 days of treatment initiation compared with either healthy knowledge.
controls or reference values (mean difference -2.42 kg [95% CI - Results: Seventy-four percent of participants felt they were ‘very
3.94 to -0.89]; I2 = 44%, p = 0.002). All studies reported a reduction knowledgeable’ after the course; compared to 10% prior and 92% felt
in all parameters compared with matched healthy controls, except they could use the knowledge daily. Participants reported that hos-
for two sub-measures of upper body muscle strength and reaction pital practical sessions (30%), determining nutritional requirements
time. Regarding physical performance, studies reported reduced motor (23%) and performing anthropometry (18%) were the most benefi-
development, walking distance, and balance. cial aspects of the training. The mean test grade post-training was
Conclusions: This systematic review indicates that the deterioration 77%. Several barriers were identified that would preclude incorpora-
in cardiorespiratory fitness, muscle strength, and physical perfor- tion into clinical practice namely lack of resources (32%), additional
mance occurs early and may already be impaired before the clinical training needs (18%), and non-supporting colleagues (10%). Almost
diagnosis is given and treatment is initiated. Consequently, physical all participants reported they would benefit from longer training,
rehabilitation is needed from treatment initiation to ameliorate further more on-site training, and more opportunities for subsequent/ongoing
deterioration in general physical performance. training.
Conclusions: This workshop successfully improved knowledge
about nutrition in pediatric oncology. Future planning with
EP498 / #259 A COLLABORATIVE EDUCATION MODEL FOR WCC, IIPAN, and SIOP Global Nutrition Committee will involve
ADVANCING NUTRITIONAL CARE IN AFRICA addressing the barriers to incorporate nutritional assessment
and interventions into clinical practice in pediatric oncology
Judith Schoeman1 , Nihad Salifu2 , Doris Ofosuhene2 , Elena Ladas3 , units.
Michelle Walters3 , Karina Viani4 , Erika Damasco-Avila5 , Mariana
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S380 of S635 ABSTRACTS

EP499 / #361 IMPACT OF MALNUTRITION ON Gnanamani Senguttuvan, Amita Trehan, Richa Jain, Deepak Bansal
PHARMACOKINETICS OF CHEMOTHERAPY IN CHILDREN Postgraduate Institute of Medical Education & Research, Pediatrics, Chandi-
WITH CANCER: A SYSTEMATIC REVIEW garh, India

Sterre Schoon1,2 , Nthongase Makamo3,4 , Aniek Uittenboogaard1,5 , Background and Aims: The pandemic taught us to manage patients via
Melanie Bernhardt6 , Gertjan Kaspers1,5 , Minke Huibers1,7 teleconsultation (TC), previously not a part of routine patient care. This
1 Princess Máxima Center, Pediatric Oncology, Utrecht, Netherlands; study aimed to assess parental satisfaction with TC services provided
2 Amsterdam UMC, location VUmc, Vrije Universiteit Amsterdam, Medicine, during the COVID-19 pandemic.
Amsterdam, Netherlands; 3 Baylor College of Medicine Children’s Founda- Methods: A survey questionnaire containing 6 parts with 35 questions
tion, Pediatric Oncology/-hematology, Lilongwe, Malawi; 4 Texas Children’s in the vernacular was provided to the participants who were families
Global HOPE Program, Pediatric Oncology/-hematology, Lilongwe, Malawi; of children diagnosed with cancer from July 2019 to March 2020. The
5 Amsterdam UMC - Location VUmc, Pediatric Oncology/-hematology, survey took 20-30 minutes to complete.
Amsterdam, Netherlands; 6 Texas Children’s Hospital, Global Hope (hema- Results: Eighty three families consisting of 66 patients with haema-
tology/oncology Pediatric Excellence), Houston, United States of America; tolymphoid malignancies and 17 with solid tumors (median age: 7.13
7 Global Child health group, Amsteram UMC, University of Amsterdam, [2-14] years), 60 on active treatment and 23 having completed ther-
Pediatric Oncology/-hematology, Amsterdam, Netherlands apy participated. Two-thirds belonged to rural areas and 40% were
from the upper-lower socioeconomic strata. Seventy-one percent of
Background and Aims: The majority of children with cancer in low- the participants did not encounter any difficulty in contacting the
and middle-income countries (LMICs) are at risk for severe malnutri- doctors, 19% had occasional problems and 10% reported major diffi-
tion. Malnutrition might affect the pharmacokinetics of chemother- culties in contacting by TC. Most (75/83) families expressed ease in
apeutic agents and understanding this impact is crucial to better communicating problems by TC. Respondents were satisfied with the
understand the impact of nutrition on toxicity and survival. Therefore, time (81/83), explanation (82/83), thoroughness (82/83), and cour-
a systematic review on the effect of malnutrition on pharmacokinetics tesy (78/83) extended to them during TC. The positive impact of TC
of chemotherapy in children with cancer was conducted. was: time-saving (93%), cost-saving (87%), decreased physical exertion
Methods: PubMed, Embase and Cochrane were searched in Octo- (82%), and availability of daily access to care providers (63%). Nearly
ber 2021 to identify eligible studies. Inclusion criteria were studies 75% of the respondents concurred to TC being as good as face-to-face
on chemotherapy pharmacokinetics in children with cancer, assess- consultation and wanted TC to be a part of routine care. Seventy-
ing the effect of malnutrition referring to undernutrition. Risk of bias one (85%) families were very satisfied/satisfied with TC and 13% were
assessment was performed using the Quality Assessment Tool for somewhat satisfied. Patients off therapy preferred physical visits vis-
Quantitative Studies. Malnutrition was defined by the World Health a-vis those on therapy (p=0.0018). Availability of a local physician and
Organization (WHO) criteria and the Gomez’ classification. socioeconomic status had no bearing on the satisfaction/continuation
Results: Four eligible studies with a total of 668 children were included, of TC (p=0.74 & .09)
containing 18% (n=121) malnourished children. One study reported Conclusions: Creative adaptation of technology has resulted in revis-
a significantly prolonged mean clearance rate and increased logAUC iting patient care delivery methods during the pandemic. High satis-
for vincristine among malnourished versus non-malnourished children faction levels (85%) among patients indicate the usefulness of these
(p<0.05). Clearance rates and volume of distribution of methotrexate, applications and the feasibility of a hybrid method of care in pediatric
doxorubicin and etoposide even incline to be lower in malnourished oncology.
children, although not significant.
Conclusions: Decreased clearance rates, increased logAUC, and
decreased volume of distribution among children with malnutrition EP501 / #1408 NEUTROPENIC ENTEROCOLITIS DURING
and cancer are suggestive for significant pharmacokinetic alterations ACUTE MYELOID LEUKEMIA THERAPY IN CHILDREN
of chemotherapy. However, data is scare, groups are small, and 75% of
the studies were performed in high-income countries where nutrition Sai Shiva G1 , Priyakumari Thankamony2 , Manjusha Nair2 , Binitha
status is less compromised compared to LMICs and no children with Rajeshwari2 , Guruprasad Cs2 , Prasanth Vr2 , Kalasekhar
severe acute malnutrition were included. This systematic review high- Vijayasekharan2 , Aleyamma Mathew3
lights the urge for further pharmacokinetic research among severely 1 Regional Cancer Centre, Paediatric Oncology, THIRUVANANTHAPU-
malnourished children with cancer in order to ultimately improve their RAM, India; 2 REGIONAL CANCER CENTRE, Pediatric Oncology, THIRU-
outcome by adapted dosing of anticancer agents. VANATHAPURAM, India; 3 REGIONAL CANCER CENTRE, Cancer Epidemi-
ology And Biostatistics, THIRUVANANTHAPURAM, India

EP500 / #490 SATISFACTION WITH TELECONSULTATION Background and Aims: Neutropenic Enterocolitis(NE)/Typhlitis is a
DURING THE COVID-19 PANDEMIC necrotizing inflammation involving the caecum and the terminal part
of ileum having 24%incidence and 44.8%mortality in paediatric Acute
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S381 of S635

Myeloid leukemia(AML). The aim was to study the clinical profile and Methods: Through national bodies, professional networks, and social
the treatment outcome of NE during paediatric AML therapy. media, we identified PTs and OTs working in services for CYPC in
Methods: Seventeen newly diagnosed AML children aged 0-14 years the UK. An organisational committee was formed, and services were
were prospectively analyzed between 1st May 2021 and 31st March invited to contribute and participate. A diverse training programme
2022.NE was defined by the presence of the clinical triad of abdominal was organised, including training and speakers arranged from CYPC
pain, fever and neutropenia(Absolute neutrophil count less than 500 Principal Treatment Centres (PTC) across the UK. Over the period of
/mm3)or imaging signs (thickened bowel wall) plus two of the clinical one year, feedback was gained from attendees every session, and a
features. AML treatment consisted of 2 induction courses with Cyto- one-year evaluation was performed.
sine arabinoside and Daunorubicin and3 consolidation courses with Results: The training programme is 18 months old and is delivered vir-
high dose Cytosine arabinoside. tually to optimise access. 18 of 19 PTCs in the UK plus five associated
Results: Among 49 chemotherapy cycles( induction-26, consolidation- centres participate in the programme. Over 100 PT & OTs who work
23) administered in 17 children, there were 23(46.9%) episodes of exclusively or partly with CYPC participate, and the average atten-
febrile neutropenia and 8(16.3%) episodes of NE. Six(75%)of these NE dance is 36. 93% were satisfied with the training and all participants
episodes occurred in 1st induction. The clinical triad of fever, abdom- reported gaining knowledge and new information from attending. 73%
inal pain and loose stools were seen in 5 ( 62.5%) patients. NE was rated the programme 5/5 stars and 87% believed the sessions were
radiologically diagnosed in 5(62.5%) children( 2 in ileocaecal junction relevant to their roles.
and 1 each in caecum, descending colon with rectum and rectosig- Conclusions: Through virtual technology, the development and deliv-
moid). The mean wall thickness was 7 mm. Four of the 5 children(80%) ery of national training programmes is highly achievable. These pro-
with a positive blood culture had Gram-Negative(GN) bacteremia and grammes can elicit high levels of participant satisfaction and be rated
septic shock. Of the 6 chemotherapy cycles that had GN bacteremia highly by attendees. They have the potential to enhance collaboration
4 were associated with NE(P-value=0.005). All children were treated and professional development and consequently improve the care of
with meropenam and colistin. Six children required oxygen support CYPC. If done on a global setting, virtual PT & OT programmes have
and 3 required Total parenteral nutrition. Six children recovered while the potential to help bridge the gap between different income/resource
2 expired(both were in 1st induction). GN bacteremia was associated settings with supportive care and rehabilitation interventions.
with mortality in NE(P-value-0.039).
Conclusions: Neutropenic enterocolitis was seen in 16.3% of children
during AML therapy with majority of these occurring in 1st induction. EP503 / #467 PERCEPTIONS OF PHYSIOTHERAPY &
With supportive care the recovery rate was 75% and mortality rate was REHABILITATION SERVICES FOR CHILDREN & YOUNG PEOPLE
25%. (0-25 YEARS) WITH CANCER & THEIR PHYSICAL ACTIVITY
LEVELS DURING THE COVID-19 PANDEMIC

EP502 / #245 THE DEVELOPMENT AND EVALUATION OF A Abu Sidhanee1 , Annie Brochu2 , Pia Delano Baudet3
NATIONAL CHILDREN’S & YOUNG PERSON’S CANCER VIRTUAL 1 UCLH, Paediatric Physiotherapy, London, United Kingdom; 2 CHU Sainte-
TRAINING PROGRAMME FOR PHYSIOTHERAPISTS AND Justine, Physical Therapy, Montreal, Canada; 3 Inmers, Kinesiology, Santiago,
OCCUPATIONAL THERAPISTS Chile

Abu Sidhanee1 , Rebecca Pickford2 , Ross Drillingcourt3 , Isabella Background and Aims: During the COVID-19 pandemic, many health-
Jones4 , Charlotte Betteridge5 care settings worldwide prioritised COVID over other areas such as
1 UCLH, Paediatric Physiotherapy, London, United Kingdom; 2 Royal paediatric oncology rehabilitation. Physical outcomes for children and
National Orthopaedic Hospital, Paediatric Physiotherapy, Stanmore, United young people (CYP) with cancer and their physical activity levels may
Kingdom; 3 UCLH, Therapies & Rehabilitation, London, United Kingdom; have been compromised by these changes and the pandemic in general.
4 Royal Marsden NHS Foundation Trust, Paediatric, Teenage & Young Through this exploratory pilot survey, we aimed to understand phys-
Adult Occupational Therapy, London, United Kingdom; 5 UCLH, Paediatric iotherapists perspectives of physiotherapy and rehabilitation services
Occupational Therapy, London, United Kingdom and the physical activity levels of CYP during the pandemic.
Methods: A survey was used to understand the experience and per-
Background and Aims: Physiotherapy (PT) and Occupational Ther- spectives of physiotherapists working with CYP with cancer across
apy (OT) are essential specialities in the management of children and the world. Questions were structured around the local effects of the
young people with cancer (CYPC). Function and quality of life of CYPC pandemic, the impact on physiotherapy and rehabilitation services,
on treatment, survivors, and those at end of life, can be enhanced and effects on physical activity levels of CYP. The survey was dis-
with access to appropriately trained PTs and OTs. No national pro- tributed to a convenience sample of physiotherapists in low, middle,
grammes previously existed in the UK. We aimed to bring profes- and high-income settings.
sionals together to share knowledge and training to enhance care for Results: 60% of responses were from high-income, 27% from middle
CYPC. income and 13% from low-income settings (n=23). All worked with
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S382 of S635 ABSTRACTS

CYP with cancer. 91% reported some/major disruption to their health- Conclusions: There is a huge unmet need for pain relief with oral
care establishment and 83% reported physiotherapy and rehabilitation morphine in Cameroon. Limited access is at least in part from unduly
services for CYP with cancer were affected in their countries. Factors strict national narcotic drug policies and regulations. Continuous advo-
which caused this included service closure, restrictions, staff redeploy- cacy with the ministry of health is essential to reduce the suffering of
ment and staff sickness. Only 48% felt that services had returned to many
pre-pandemic levels. 83% reported that physical activity levels of CYP
were affected during the pandemic. Factors which caused this were
identified as being lockdown, restrictions, shielding and isolation. 61% EP505 / #1174 UNMET PALLIATIVE CARE NEEDS OF A
felt that physical activity had not yet returned to pre-pandemic levels. CHILD WITH CANCER
Conclusions: Physiotherapy, rehabilitation, and physical activity are
essential to optimise function and quality of life for CYP with can- Mei Sitaresmi1,2 , Alexandra Pangarso1,2 , Sri Mulatsih1,2 , Gertjan
cer. These domains were affected during the pandemic, potentially Kaspers3,4 , Saskia Mostert3,4
compromising outcomes for CYP with cancer. Physiotherapists and 1 DR Sardjito Hospital, Child Health, Sleman, Indonesia; 2 Faculty of
rehabilitation professionals must find strategies to adapt their inter- Medicine, Public Health and Nursing, UGM, Child Health, Sleman, Indone-
vention and optimise physical activity of CYP with cancer to mitigate sia; 3 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology,
the effects of future COVID-19 related disruption. Utrecht, Netherlands; 4 Emma Children’s Hospital, Amsterdam UMC, Vrije
Universiteit, Pediatric Oncology, Amsterdam, Netherlands

EP504 / #25 MORPHINE GAP IN CAMEROON: MORE Background and Aims: In low and middle-income countries, palliative
ADMINISTRATIVE FACILITATION REQUIRED TO REDUCE care has received little or no attention. When children with incurable
SUFFERING cancer continue aggressive treatment, they may suffer unnecessar-
ily from pain, discomfort, and low quality-of-life. Families are often
Alberic Signang1 , Glenn Mbah Afungchwi2 , Kouya Francine1 , Angele not allowed to participate in decision-making whether they want
Pondy Pondy1 to extend the life of their children or focus on relieving pain and
1 cameroon baptist convention health services, Pediatria Oncology, discomfort.
Bamenda, Cameroon; 2 Cameroon Baptist Convention Health Services, Methods: A single case study was conducted to highlight the chal-
Pediatria Oncology, Bamenda, Cameroon lenges facing families of children who die of cancer in Indonesia.
Investigators identified a child with acute lymphoblastic leukemia
Background and Aims: Many patients in Low- and middle-income and studied her medical records. A home visit was conducted in
countries lack access to the opioid medicines that the World Health December 2019 to interview the mother. Two independent inter-
Organization designates as essential for pain control. Disparities in opi- viewers used a semi-structured questionnaire. Informed consent was
oid consumption are partly related to policies affecting opioid access. obtained.
Pain associated with cancer can significantly influence an individual’s Results: A nine-year-old Indonesian girl suddenly suffered from fever
morbidity and quality of life. Therefore, Pain relief is fundamental to episodes and paleness. Her mother brought the child first to a local
quality of life and palliative care. shaman. When symptoms persisted, she was referred to a tertiary
AIM: To evaluate the availability of oral Morphine in relation to pain care referral hospital, where a bone-marrow puncture confirmed the
control need in Cameroon and national opioids regulation policies. diagnosis of acute lymphoblastic leukemia, and treatment was started.
Methods: Analysis of opioid consumption data for Cameroon as pub- The mother experienced a lack of openness in communication about
lished by the international narcotic control board (INCB), followed by her daughter’s treatment and prognosis. She reported that her child
a descriptive literature review of publicly available documents on pain received intensive chemotherapy during the first two years despite a
control needs and opioid regulations for Cameroon using PubMed, poor prognosis and severe side effects. She was not informed about
Medline, Google Scholar, Google, Ministry of Public Health Website the child’s treatment choices during their final illness. Palliative treat-
and National Institute of Statistics Cameroon. ment was ultimately started without informing the family. The mother
Results: The annual consumption of morphine in Cameroon has emphasized that, in retrospect, the family would have preferred to par-
steadily increased from 0.07 mg/capita in 1985 to 0.35 mg/capita (7.6 ticipate in decision-making and opt for a shorter, more comfortable life
kg) in 2012. About 55.3 % of cancer and HIV related deaths are asso- without so much needless pain and suffering.
ciated with moderate/severe pain. Almost all (98%) of patients dying Conclusions: This study highlights the importance to start palliative
of HIV or Cancer have untreated moderate /severe pain. An average care immediately at diagnosis. Both the physical and psychosocial well-
annual import of 3.4kg of Morphine was recorded between 20011 being of patients needs to be closely monitored. Training on open
and 2013, while a minimum of about 183Kg is required for HIV and communication in palliative care is required in universities and hos-
cancer patients only. Importation of morphine is subject to signed pitals to enable shared decision-making and improve quality-of-life of
authorization signed by the minister of public health. children and their families.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S383 of S635

EP506 / #798 SAFETY OF PROCALCITONIN GUIDED Roland Ssemakula1 , Miracle Giraura2 , Joshi Evelyne2 , Peter Wasswa2 ,
DISCONTINUATION OF ANTIBIOTIC THERAPY AMONG Joseph Lubega3 , Nathan Serazin3 , Nicholas Ettinger3 , Susan Torrey3
CHILDREN RECEIVING CANCER CHEMOTHERAPY AND 1 Global HOPE, Pediatrics, kampala, Uganda; 2 Global HOPE, Pediatrics,
HAVING LOW RISK FEBRILE NEUTROPENIA: A RANDOMIZED Kampala, Uganda; 3 Texas Children’s Hospital, Pediatrics, HOUSTON, United
NON-INFERIORITY TRIAL (PROFEN-C STUDY) States of America

Prasanth Srinivasan1 , Jagdish Prasad Meena1 , Aditya Gupta1 , Background and Aims: Gains in survival in programs treating pedi-
Ashutosh Halder2 , Arti Kapil3 , Rm Pandey4 , Rachna Seth1 atric cancer in sub-Saharan Africa have been modest. Improvements
1 All India Institute of Medical Sciences, Division Of Pediatric Oncology, in the quality of supportive care may be impactful. Global HOPE in
Department Of Pediatrics, New Delhi, India; 2 All India Institute of Medi- Uganda is a high-volume referral center with seriously ill patients and
cal Sciences, Department Of Reproductive Biology, New Delhi, India; 3 All limited pediatric critical care resources. Respiratory distress occurs
India Institute of Medical Sciences, Department Of Microbiology, New Delhi, commonly. We implemented a symptom-based management algorithm
India; 4 All India Institute of Medical Sciences, Department Of Biostatistics, for respiratory distress on the hospital ward using quality improvement
New Delhi, India methodology and virtual mentoring.
Methods: Stakeholder interviews and focus groups identified two
Background and Aims: Febrile neutropenia (FN) is one of the most key drivers: access to equipment/medication (E/M) and healthcare
common oncological emergencies. There is no consensus regarding worker (HCW) skills. E/M interventions included identifying gaps in the
the optimal duration of empirical antibiotics. The safety of early restocking process and improving organization of E/M. PDSA cycles,
discontinuation of antibiotics without marrow recovery is not well an E/M checklist, and run charts evaluated, measured, and analyzed
established. In this study we explored the safety of early discontinu- effectiveness of the intervention. For HCW skills, acute care experts at
ation of empirical antibiotics in low-risk FN (LR-FN) without marrow Texas Children’s Hospital virtually trained and mentored local champi-
recovery, utilising procalcitonin (PCT) guided protocol. ons to deliver training. We used before and after assessment of HCW
Methods: In this randomised non-inferiority trial, the children with LR- knowledge acquisition and self-efficacy to evaluate improvement.
FN with afebrile period of at least 24 hours, sterile blood culture and Results: Baseline data were collected for E/M using the checklist.
negative/normalised PCT, were randomised at 72 hours into interven- As the result of a restocking process implemented by nursing and
tion and standard arm. The empirical antibiotics were stopped at 72 pharmacy team members, E/M have been consistently available. An
hours for those in intervention arm regardless of their absolute neu- organization system using a wall hanging with pockets was well-
trophil count (ANC). The patients in standard arm continued to receive received. However, the ward soon moved to a new location that did
antibiotics for at least 7 days or until ANC >500/mm3 . The primary not have a suitable location for it. To support HCW training, the clinical
objective was to compare the treatment failure rates between the team leader identified trainers and a training schedule. Two train-
two arms. The secondary objectives were to compare the duration of ing sessions have been well-received. However, scheduling training
antibiotics and all-cause mortality between the two arms. sessions has been challenging due to HCWs’ clinical responsibilities.
Results: Between February 2020 and October 2021, 46 children with Conclusions: HCWs and program leadership have been enthusias-
LRFN were randomised to intervention arm (n=23) or standard arm tic about the project. Contextual factors (change in location and the
(n=23). Treatment failure was observed in 2/23 (8.7%) of patients in busy clinical environment) have impacted success. Next steps for the
intervention arm compared to 1/23 (4.3%) in the standard arm [RR: 2 E/M intervention include developing a new organizational system and
(95% CI: 0.19 to 20.55); p value = 0.550]. The patients in the interven- ensuring sustainability of the restocking process. For HCWs, a flexible
tion arm had significantly lesser days of antibiotic exposure compared training schedule is needed to accommodate clinical responsibilities.
to those in standard arm (3 days vs 7 days; [p value < 0.001]). None of
the study subjects died due to any cause during the study follow up.
Conclusions: Early discontinuation of antibiotics at 72 hours did not EP508 / #376 PLATELET AND RED BLOOD CELL
result in significantly increased risk of treatment failure. The total dura- TRANSFUSIONS IN CHILDREN WITH CANCER: A CLINICAL
tion of antibiotic exposure was significantly lesser among intervention PRACTICE GUIDELINE
arm. Further, multicentre randomised control studies are needed to
throw some light on this subject. Debbie Stavleu1,2 , Demi Kruimer1 , Renée Mulder1 , Leontien Kremer1 ,
Wim Tissing1,2 , Erik Loeffen1,2
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology,
EP507 / #279 IMPLEMENTATION OF A PRACTICE Utrecht, Netherlands; 2 University Medical Center Groningen, Department
GUIDELINE FOR THE MANAGEMENT OF RESPIRATORY Of Pediatric Oncology/hematology, Groningen, Netherlands
DISTRESS FOR HOSPITALIZED CHILDREN WITH CANCER IN A
RESOURCE CONSTRAINED SETTING THROUGH VIRTUAL Background and Aims: Both platelet and red blood cell transfusions
COLLABORATION play an important role in supportive care in children with cancer. In
current clinical practice, recommendations regarding thresholds for
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S384 of S635 ABSTRACTS

administering platelet or red blood cell transfusions are often not representatives. Multiple in-person meetings were held to rank out-
evidence-based. Therefore, a clinical practice guideline (CPG) was comes, discuss evidence, complete evidence-to-decision frameworks
developed to establish an overview of the available evidence and and formulate recommendations.
provide recommendations for clinicians. Results: Nine studies, including more than 1,400 children, with vari-
Methods: A systematic literature review was performed, including ous study designs formed the evidence base for the recommendations.
dual appraisal of all citations. The GRADE methodology was used to Considering the limited amount of studies in children with cancer, addi-
assess, extract and summarize the evidence. When evidence in chil- tional evidence was also extracted from adult guidelines. Our experts
dren with cancer was limited, additional evidence was extracted from assessed all evidence and translated it, transparently, into recommen-
adult cancer guidelines. A comprehensive multidisciplinary panel was dations. In total, 14 recommendations were made regarding social
assembled, comprising 24 professionals and patient representatives. restrictions in children with cancer. For example, recommendations
Multiple in-person meetings were held to discuss evidence, complete were made on swimming, having pets, visiting the zoo or farm, perform-
evidence-to-decision frameworks and formulate recommendations. ing sports or high-velocity events, attending school or kindergarten,
Results: In total, eight studies including almost 2,000 children with can- use of public transport and more. Some of these recommendations
cer formed the evidence base for the recommendations. The expert have a great impact already as they result in changes in current policy
panel assessed all evidence and translated it, transparently, into rec- and standard of practice.
ommendations. In total, more than 38 recommendations were made Conclusions: In this clinical practice guideline, we provide both
regarding platelet transfusions and red blood cell transfusions in chil- evidence-based recommendations and best practice statements
dren with cancer. Thresholds for prophylactic platelet transfusions regarding social restrictions in children with cancer. With these rec-
were recommended for children with cancer undergoing for example ommendations we provide guidance for clinicians, children and their
a lumbar puncture or line insertion or for children with cancer and parents and contribute to improving quality of life for children with
sepsis. Also, thresholds for red blood cell transfusions were recom- cancer.
mended for children with cancer and for example cardiac or pulmonary
comorbidities, sepsis or undergoing radiotherapy. Some of these rec-
ommendations have a great impact already as they result in changes in EP510 / #373 COMPLICATIONS OF CENTRAL VENOUS AND
current policy and standard of practice. PERIPHERAL INSERTED CENTRAL LINE CATHETERS IN
Conclusions: In this clinical practice guideline, we provide evidence- PEDIATRIC ONCOLOGY PATIENTS. A SINGLE CENTER
based recommendations regarding platelet and red blood cell transfu- EXPERIENCE
sions in children with cancer. With these recommendations we aim to
provide guidance for clinicians and contribute to improving outcomes Afrodite Tsagkaraki, Iordanis Pelagiadis, Maria Stratigaki, Nikolaos
for children with cancer. Katzilakis, Eftichia Stiakaki
University Hospital of Heraklion, Department Of Pediatric Hematology-
oncology, Heraklion, Greece
EP509 / #378 SOCIAL RESTRICTIONS IN CHILDREN WITH
CANCER: A CLINICAL PRACTICE GUIDELINE Background and Aims: Studies comparing types of central venous
catheter (CVC) and their associated complications are particularly
Debbie Stavleu1,2 , Renée Mulder1 , Demi Kruimer1 , Leontien Kremer1 , limited and contradictory in children diagnosed with malignancy and
Wim Tissing1,2 , Erik Loeffen1,2 receiving chemotherapy. Due to the ease of insertion and overall usabil-
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, ity as well as the site of insertion, the PICC line seems an appealing
Utrecht, Netherlands; 2 University Medical Center Groningen, Department choice specially for older children.
Of Pediatric Oncology/hematology, Groningen, Netherlands Methods: This is a ten-year retrospective study, comparing the com-
plication rates among the most commonly applied types of CVCs in a
Background and Aims: In current clinical practice, recommendations Pediatric Hematology-Oncology Unit. Data was mainly collected from
regarding social restrictions for children with cancer are often not the patients’ medical records.
evidence-based. Critically reviewing the evidence and recommenda- Results: In total 192 CVCs were placed in 137 children (119 Hick-
tions is therefore of great importance as these social restrictions (e.g. man/73 PICC lines) with a median age of 8.3±5.6 years(0.4-20.7 years).
swimming, school attendance, sports) can impair the quality of life of In children <10 years of age, the most prevalent choice was Hick-
these children severely. Therefore, a clinical practice guideline (CPG) man line (68%), while for those >10 years PICC line (77.85%). The
was developed to establish an overview of the available evidence and percentage of patients with Hickman catheters who experienced an
provide recommendations for clinicians, children and their parents. infection at the first catheter insertion was 34.5% while with PICC
Methods: A systematic literature review was performed, including line 23.3% (p = 0.102). The median time of infection (95% CI) was 339
dual appraisal of all citations. The GRADE methodology was used to days for Hickman and 288 for PICC line type catheters (1,68 episodes
assess, extract and summarize the evidence. A comprehensive multidis- / 1000 catheter days for Hickman type catheters and 1.70 / 1,000
ciplinary panel was assembled, comprising 21 professionals and patient for PICC lines). The incidence of a thrombotic episode was 22.1% for
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S385 of S635

Hickman and 33.8% for PICC line catheter (p=0.073), with a median Conclusions: CVC-associated candidemia occurred mainly in sup-
time (95% CI) of 126 and 130 days respectively (p=0.430) (0,63 pressed patients. With optimal supportive care, we successfully
episodes / 1,000 catheter days for Hickman type catheters and 1.51 / treated most of these infections. Lines were removed in approximately
1000 catheter days for PICC lines). The overall perforation and acci- two-thirds of our patients.
dental removal/displacement rate was 16.8% for Hickman and 12.3%
for PICC line type catheters (p = 0.400).
Conclusions: The occurrence of complications does not seem to be EP512 / #879 UNMET SUPPORTIVE CARE NEEDS OF
affected by the type of catheter inserted. The age of the patient and the FAMILIES OF CHILDREN WITH CANCER AND OTHER CHRONIC
duration of the catheter placement rather predispose for an infectious ILLNESS: A SYSTEMATIC REVIEW
or thrombotic incident.
Sangeetha Thomas, Victoria White, Linda Byrne, Nicholas Ryan
Deakin Univeristy, Psychology, Burwood Hwy, Australia
EP511 / #864 TREATMENT AND OUTCOMES OF CENTRAL
VENOUS CATHETER (CVC) - ASSOCIATED CANDIDEMIA Background and Aims: Childhood chronic health conditions (CHC),
AMONG CHILDREN WITH CANCER: KING HUSSEIN CANCER like cancer can be complex and challenging with parents expressing
CENTER EXPERIENCE many needs relating to caring for their child. However, whether the
needs of families with a child with cancer differ from families with
Dua’A Zandaki1 , Sawsan Mubarak2 , Anwar Al-Nassan1 , Sakher children with other CHC is not clear as there has been no system-
Obeidat1 , Muhanad Alshathly1 , Rawan Bdeir1 , Tasneem Guzu1 , Iyad atic synthesis of available evidence in this area. To address this gap,
Sultan3 this systematic review examines the unmet Supportive Care Needs
1 King Hussein Cancer Center, Pediatrics, Amman, Jordan; 2 King Hussein (SCN) of families with common pediatric CHC to identify similarities
Cancer Center, Infection Control, Amman, Jordan; 3 King Hussein Cancer and differences in needs and characterise assessment tools.
Center, Pediatrics, AMMAN, Jordan Methods: Electronic searches were performed among the databases
of Medline complete, PsycINFO, CINHAL, and Embase and screened
Background and Aims: The best treatment for CVC associated can- articles published between 1990- April 2021. Qualitative and quanti-
didemia remains obscure. Mainly, the indications for line removal and tative studies involving children aged between 0-18 years, diagnosed
duration of treatment are not well defined. with cancer and other CHC reporting unmet needs were eligible. Stud-
Methods: A retrospective chart review of children treated at our cen- ies focusing on children with genetic or developmental conditions
ter over a 5-year period. Cases were identified via microbiology lab were excluded. Study characteristics, sample demographics, outcome
records in patients having CVC between Jan2016-Dec2020. Data col- measures were extracted from eligible papers.
lected included demographics, diagnosis, CVC type, species identity Results: Of the 5061 papers identified 34 studies were eligible. Cancer
and sensitivity, modes of treatment, and outcomes, defined as 30-day was the focus of 25 studies and other work focused on heart disease,
mortality and CVC removal. asthma, diabetes, renal problems and mixed CHC (n=9). Parents of
Results: There were 25 candidemia episodes in 24 patients with CVC children with cancer reported gaps in information, emotion, and psy-
(median age, 2.4 years; range, 0.2-18.8; males, 72%). These lines were chosocial support. However, practical/caretaking, informational and
ports (37%), CVP (33%), and Hickman (22%). Candidemia occurred at a health care service needs were higher for other conditions. There was
median of 1.7 months after cancer diagnosis (range,0-180), which was a lack of a consistent need assessment across conditions and most
mainly leukemia (32%), solid tumors (24%), and lymphomas (8%). Four- studies (n=11) used a non-validated tool.
teen patients (56%) had an absolute neutrophil count <100/microL Conclusions: While families of children with cancer seem to have fewer
at first positive culture. A positive bacterial culture coincided in 44%, practical and caretaking needs, there is still a gap in the emotional and
and 92% had received broad-spectrum antibiotics within the prior psychosocial support they would like and the support they receive.
2 weeks. Lung infections were detected in 9 patients (36%). Fever Work is needed to develop a valid and reliable measure of need across
was present at initial diagnosis in 89% of patients, and 52% suf- CHC to all comparison and thereby ensure all families with children
fered hemodynamic instability. Non-albicans Candida comprised 64% with CHC received the support they need.
of cultures. Antifungal resistance to fluconazole and voriconazole
was present in 2 and 1 cases, respectively. Amphotericin was the
most commonly used initial agent (44%). An antifungal switch was EP513 / #723 VALIDATION OF A MODIFIED BEDSIDE
needed for different reasons in 17 patients (67%) after a median of PEDIATRIC EARLY WARNING SYSTEM SCORE FOR DETECTION
5 days (range,1-27). Most used second-line agents were caspofun- OF CLINICAL DETERIORATION IN HOSPITALIZED PEDIATRIC
gin (26%), and fluconazole (19%). Median time to clearing culture ONCOLOGY PATIENTS: A PROSPECTIVE COHORT STUDY
was 2 days (range,1-26), and to fever resolution was 3 days (range,0-
33). Sixteen CVCs (63%) were removed. Overall 30-day mortality was Marijn Soeteman1 , Teus Kappen2 , Martine Van Engelen1 , Maartje
18.7%±7.5%. Marcelis1 , Ellen Kilsdonk1 , Marry Van Den Heuvel-Eibrink1 , Edward
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S386 of S635 ABSTRACTS

Nieuwenhuis3 , Wim Tissing1,4 , Marta Fiocco1,5 , Roelie Wösten-Van to determine the use of opioids and evaluate the differences according
Asperen6 to sex, age, pathology, and the occurrence of side effects in pediatric
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, cancer inpatients in a Peruvian hospital.
Utrecht, Netherlands; 2 Wilhelmina Children’s Hospital/University Med- Methods: Retrospective, observational, monocentric study. All infants
ical Center Utrecht, Department Of Anesthesiology, Utrecht, Nether- and children younger than 14 years who initiate opioids in emergency
lands; 3 Wilhelmina Children’s Hospital/University Medical Center Utrecht, or hospitalization at the National Institute of Neoplastic Diseases
Department Of Pediatrics, Utrecht, Netherlands; 4 University Medical Cen- (INEN) between December 2021 and February 2022 were reviewed.
ter Groningen, Department Of Pediatric Oncology/hematology, Groningen, Statistical analysis was descriptive and analytic.
Netherlands; 5 Leiden University, Mathematical Institute, Leiden, Nether- Results: A total of 134 children were identified, 73 patients (54.5.7%)
lands; 6 Wilhelmina Children’s Hospital/University Medical Center Utrecht, were male and 61 (45.3%) female. Tramadol (81%) followed by mor-
Department Of Pediatric Intensive Care, Utrecht, Netherlands phine (19%) were the most used opioids for pain management. The
median age that used tramadol was 8 years. (IQR 4.12) and morphine
Background and Aims: Hospitalized pediatric oncology patients are was 8 years. (IQR 5-10). In the multivariate analysis, the use of tramadol
prone to clinical deterioration. Pediatric early warning system (PEWS) treatment (P<0.001) did not significantly affect the occurrence of side
scores have not been prospectively validated for these patients. We effects. Conversely, the use of morphine (P<0.001) was statistically sig-
determined the predictive performance of a modified BedsidePEWS nificant for the occurrence of side effects. There were no significant
score for clinical deterioration during an inpatient ward admission in differences in the appearance of side effects between the use of tra-
pediatric oncology patients. madol and morphine according to the type of diagnosis, whether they
Methods: This prospective cohort study was conducted in hospitalized were hematological or solid neoplasms, nor the reason for starting the
pediatric oncology patients aged 0 to 18 years at the 80-bed Dutch opioid or the place where treatment was started (emergency or hospi-
pediatric oncology national referral center. The association between talization). The median time of opioid use was 6 days for both tramadol
PEWS score and unplanned PICU admission or cardiopulmonary resus- (IQR 2-10) and morphine (IQR 4-12). The Kruskal Wallis test showed
citation (CPR) was estimated by a Cox proportional hazard model. The that the duration of opioid use was not significant for the development
predictive performance (discrimination and calibration) of the model of side effects.
was assessed by bootstrapping. Conclusions: The use of morphine is still restricted in developing
Results: We included 1137 patients, of which 103 patients experi- countries, so the use of tramadol is preferred. Although there are
enced 130 primary outcome events (127 unplanned PICU admissions restrictions on the use of tramadol, there was no evidence of greater
and 3 CPRs). The modified BedsidePEWS score was significantly asso- development of side effects.
ciated with time to unplanned PICU admission or CPR (hazard ratio
1.65 (95%CI 1.59–1.72) per point increase). Discriminative ability
was moderate, considering a discrimination-index close to zero and EP515 / #741 USE OF COMPLEMENTARY AND
concordance-index of 0.83. Calibration was excellent (index-corrected ALTERNATIVE MEDICINE (CAM) REMEDIES IN CHILDHOOD
slope of 0.99). Positive and negative predictive values at score cut- CANCER PATIENTS - A SURVEY REPORT FROM CHILDREN
off 8, at which escalation of care is required, were 1.4% and 99.9%, CANCER CENTRE OF CAA- NICH,KARACHI
respectively.
Conclusions: The modified BedsidePEWS score is a strong prognostic Imam Uzma, Fahad Mumtaz, Marya Humayun
factor for time to unplanned PICU admission or CPR in pediatric oncol- Child Aid Association,NICH, Pediatric Oncology, Karachi, Pakistan
ogy patients. The score may aid in clinical decision making for timing of
escalation of care. Background and Aims: In children; complementary and alternative
medicine (CAM) including herbal remedies, homeopathic medicines,
hikmat medicines, diet changes, nutritional supplements, vitamins,
EP514 / #1610 PEDIATRIC ONCOLOGY: A DATA ANALYSIS spiritual therapy,use of massage, acupuncture, and aromatherapy have
OF PATIENTS USING OPIOIDS DURING THEIR been used for a variety of chronic illnesses including asthma, arthritis,
HOSPITALIZATION IN PERU cancer, gastrointestinal diseases, and neurological or developmental
disorders.1, 2 This study aims to investigate the prevalence of the use
Cecilia Ugaz1 , Roxana Morales1 , Jacqueline Montoya Vasquez1 , of CAM among pediatric cancer patients in a tertiary care teaching
Georgina Soto1 , Liliana Vasquez2 hospital.
1 Instituto Nacional de Enfermedades Neoplásicas, Pediatric Oncology, Lima, Methods: Caregivers of 287 pediatric cancer patients receiving con-
Peru; 2 Pan American Health Organization, Nmh, Washington D.C., United ventional treatment including chemotherapy for various types of
States of America cancer were enrolled at pediatric oncology department from January
1, 2021 till March 31, 2021.Data was recorded in a Performa with rel-
Background and Aims: Pain in hospitalized children with cancer may evant questions to assess parents’ perspectives about CAMs and their
be unrecognized and undertreated. This study aimed of this study was use.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S387 of S635

Results: 211 (73.5%) caregivers agreed to give response to the sur- gle lumen PICCs were associated with a higher risk of complications
vey.Among them,126 (59.7%) replied in affirmation regarding use of (49% vs. 26%; IRR5.12, CI95%2.76-9.50), severe complications (19%
complementary and alternative remedies. Acute Leukemia (n=66) was vs. 7%; IRR11.96, CI95%2.68-53.42), and early removal (16% vs. 7%;
the most common diagnosis getting CAM. Of them, (79.4%) were IRR8.97, CI95%1.94-41.50). A single lumen PICC, was identified as a
receiving curative intent treatment. M: F ratio was 1.4:1 and 69.8% risk factor for CVC-related CVT when compared to TIVAPs (12% vs.
patients were from outside Karachi. Only 28.5 %( n=36), caregivers 7%, IRR6.98, CI9%1.45-33.57).
informed about CAM use to their physician. Common reasons of Conclusions: The insertion of a TIVAP rather than a PICC should be
using CAM in order of frequency were; feeling of un-wellness with recommended for pediatric patients with HL, especially in the presence
chemotherapy (n=27), loss of appetite (n=25), repeated episodes of of CVT-related risk factors. Future trials should evaluate the efficacy
fever (n=24) and repeated hospitalization (n=15). Natural health prod- and safety of direct oral anticoagulants for the primary prevention
ucts, herbal medicines, appetite stimulants, weight gain products, use of CVT in pediatric patients with a PICC and other CVT-related risk
of vitamins and minerals were reported by 45% (n=56) caregivers factors.
as alternative remedies, followed by spiritual healing in another 39%
patients. The reasons for not using CAM were concern for side effects
and doubt of its effectiveness in two-third patients. EP517 / #495 THE IMPACT OF TASTE AND SMELL CHANGES
Conclusions: Caregivers are hesitant of talking about the use of CAM IN CHILDREN WITH CANCER UNDERGOING CHEMOTHERAPY:
in our survey. To ensure patient safety, healthcare providers including A QUALITATIVE STUDY
doctors and nurses, pharmacists and psychosocial team need to include
CAM use in their data registry and counseling sessions. Mirjam Van Den Brink1,2 , Minke Ter Hedde1 , Emmy Van Den Heuvel2 ,
Wim Tissing1,3 , Remco Havermans2
1 Princess Maxima Center for Pediatric Oncology, Pediatric Oncology,
EP516 / #157 CENTRAL VENOUS CATHETER ASSOCIATED Utrecht, Netherlands; 2 Maastricht University Campus Venlo, Laboratory Of
COMPLICATIONS IN PEDIATRIC PATIENTS DIAGNOSED WITH Behavioural Gastronomy, Centre For Healthy Eating And Food Innovation,
HODGKIN LYMPHOMA: IMPLICATIONS FOR CATHETER Venlo, Netherlands; 3 University of Groningen, University Medical Center
CHOICE Groningen, Beatrix Children’s Hospital, Department Of Pediatric Oncology
And Hematology, Groningen, Netherlands
Ceder Van Den Bosch1 , Judith Spijkerman2 , Marc Wijnen1 , Idske
Kremer Hovinga3 , Friederike Meyer-Wentrup2 , Alida Van Der Steeg1 , Background and Aims: Children receiving chemotherapy often report
Marianne Van De Wetering2 , Marta Fiocco4 , Indra Morsing5 , Auke taste changes. Although this is a bothersome symptom, it is still unclear
Beishuizen2 what the essence of these taste changes are, to what degree concomi-
1 Princess Maxima Center for Pediatric Oncology, Pediatric Surgery, Utrecht, tant smell changes qualify this symptom and how much of an impact it
Netherlands; 2 Princess Maxima Center for Pediatric Oncology, Pediatric has on the life of children with cancer.
Oncology, Utrecht, Netherlands; 3 Van Creveldkliniek University Medical Methods: Semi-structured interviews were used to explore character-
Centre Utrecht, Benign Hematology, Thrombosis And Hemostasis, Utrecht, istics and impact of taste and smell changes in 27 children with cancer
Netherlands; 4 Princess Maxima Center for Pediatric Oncology, Trial And (6-18 years) receiving chemotherapy. Thematic analysis of interview
Data Center, Utrecht, Netherlands; 5 Princess Maxima Center for Pediatric data was performed.
Oncology, Intensive Care / Anesthesiology, Utrecht, Netherlands Results: Interview data could be grouped into three main themes,
namely changes in 1) taste, 2) smell, and 3) eating behavior. As
Background and Aims: The purpose of this study was to determine the expected, most children reported experiencing taste and smell changes
most optimal central venous catheter (CVC) for pediatric patients with just after start of treatment, but changes varied greatly; that is,
Hodgkin lymphoma (HL) in terms of complications. some reported increased taste and smell function, whereas others
Methods: A retrospective study including patients diagnosed with HL reported a decrease. Taste and smell changes (regardless of direc-
from 2015-2021 at the Princess Máxima Center. Patients were fol- tion) negatively impact quality of life, with these changes commonly
lowed from CVC insertion until removal or 06-2021, whichever came described as “disappointing” or “frustrating”. Interestingly, particular
first. The primary outcome was the CVC-related complication inci- chemotherapeutic agents appear strongly associated with taste and
dence rate (IR) per 1 000 CVC-days. Furthermore, the incidence rate smell changes (e.g., methotrexate), prompting sensory-specific coping
ratio (IRR) was calculated by comparing complication IRs between strategies.
peripherally inserted central catheters (PICC) and totally implantable Conclusions: Both taste and smell changes are common in children
venous access ports (TIVAP). Additionally, risk factors for central with cancer. The essence of these changes varies widely, but are gen-
venous thrombosis (CVT) were identified. erally considered bothersome symptoms. Ways to cope with taste
Results: A total of 98 patients were included. The most frequently or smell changes were described by the children, warranting further
observed complications were local irritation/infections (18%;IR0.93), research and offering the opportunity for enhancing patient-centred
malfunctions (15%;IR0.88), and CVC-related CVTs (10%;IR0.52). Sin- care.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S388 of S635 ABSTRACTS

EP518 / #72 DEVELOPMENT OF A QUESTIONNAIRE TO versity of Groningen, University Medical Center Groningen, Paediatrics,
EVALUATE FEMALE FERTILITY CARE IN PEDIATRIC ONCOLOGY, Groningen, Netherlands
A TREL INITIATIVE
Background and Aims: Pediatric palliative care is concerned with
Maria Elisabeth Madeleine Van Der Perk1 , Eglė Stukaitė-Ruibienė2 , relief of suffering of all children with a life threatening disease and
Žana Bumbulienė2 , Goda Vaitkevičienė2 , Annelies Bos3 , Marry Van their families in all domains (physical, psychological, social and spiri-
Den Heuvel-Eibrink4 , Jelena Rascon2 tual). This includes pediatric oncology patients. In 2013, the first Dutch
1 Prinses Máxima Centrum voor kinderoncologie, Pediatric Oncology, multidisciplinary clinical practice guideline for pediatric palliative care
Utrecht, Netherlands; 2 Vilnius University, Faculty Of Medicine, Clinic Of was developed, providing recommendations on relief of symptoms,
Obstetrics And Gynecology, d, Lithuania; 3 UMC Utrecht, Reproductive decision-making and organization of care. Evaluation of the guideline
Medicine And Gynaecology, Utrecht, Netherlands; 4 Princess Máxima Center revealed a need for revision of the recommendations and inclusion of
for Pediatric Oncology, Division Of Pediatric Oncology, Netherlands new recommendations on E-Poster Topics such as psychosocial and
bereavement care, advance care planning and shared decision-making.
Background and Aims: Currently the five-year survival of childhood The aim of this research is to improve provision of pediatric palliative
cancer is up to 80% due to improved treatment modalities. How- care in the Netherlands by developing an updated version of the Dutch
ever, the majority of childhood cancer survivors develop late effects Pediatric Palliative Care guideline.
including infertility. Survivors describe infertility as an important and Methods: A multidisciplinary guideline panel reviewed literature on
life-altering late effect. Fertility preservation options are becoming pediatric palliative care by systematic literature searches. The GRADE
available to pre- and postpubertal patients diagnosed with childhood methodology was used to grade the evidence and to formulate recom-
cancer and fertility care is now an important aspect in cancer treat- mendations. Recommendations were formulated and refined based on
ment. The use of fertility preservation options depends on the quality the evidence, clinical expertise, and patient values. For those E-Poster
of counseling on this important and delicate issue. The aim of this Topics where no evidence was available, recommendations were based
manuscript is to present a questionnaire to determine the impact on other guidelines, clinical expertise and patient values.
of fertility counseling in patients suffering from childhood cancer, to Results: The updated systematic literature search identified 14 ran-
improve fertility care and evaluate what patients and their parents or domized controlled trials and 15 systematic reviews that prompted
guardians consider good fertility care. refinement of recommendations. For 27 out of 42 formulated clin-
Methods: Within the framework of the EU-Horizon 2020 TREL project, ical questions, no evidence was found. This revealed major gaps in
a fertility care evaluation questionnaire used in the Netherlands was knowledge on pediatric palliative care. Based on evidence (if available),
made applicable for international multi-center use. The questionnaire clinical expertise and patient values, more than 100 recommendations
to be used at least also in Lithuania, incorporates patients’ views on various E-Poster Topics in pediatric palliative care were generated.
on fertility care to further improve the quality of fertility care and Conclusions: The updated guideline uses existing evidence and
counseling. national expertise to develop transparent and easy-to-use recom-
Results: evaluate and will be used to improve current fertility care in a mendations to facilitate provision of high quality pediatric palliative
national specialized pediatric oncology center in the Netherlands and a care. The guideline promotes interdisciplinary collaboration and opens
pediatric oncology center in Lithuania. opportunities for international research into the identified knowledge
Conclusions: An oncofertility-care-evaluation questionnaire has been gaps to further improve pediatric palliative care.
developed for pediatric oncology patients and their families specifi-
cally. Results of this questionnaire may contribute to enhancement of
fertility care in pediatric oncology in wider settings and thus improve EP520 / #963 A STITCH IN TIME SAVES NINE: TIMELY USE
quality of life of childhood cancer patients and survivors. OF N-ACETYL CYSTEINE (NAC) FOR CHEMOTHERAPY
INDUCED VENO-OCCLUSIVE DISEASE (VOD), IS IT A
COST-EFFECTIVE ALTERNATIVE?
EP519 / #442 THE DUTCH MULTIDISCIPLINARY CLINICAL
PRACTICE GUIDELINE FOR PEDIATRIC PALLIATIVE CARE Tandra Harish Varma, Thirumala Rupakumar, Kalasekhar
Vijayasekharan, Prasanth Vr, Guruprasad Cs, Priyakumari
Kim Van Teunenbroek1 , Renée Mulder1 , Leontien Kremer1,2,3 , Brigitt Thankamony
Borggreve4 , A. A. Eduard Verhagen5 , Erna Michiels1 Regional Cancer Centre, Department Of Pediatric Oncology, Thiruvanan-
1 Princess Máxima Center for Pediatric Oncology, -, Utrecht, Nether- thapuram, India
lands; 2 University Medical Center Utrecht, Wilhelmina Children’s Hospital,
Utrecht, Netherlands; 3 Emma Children’s Hospital, Amsterdam University Background and Aims: Defibrotide, the conventional antidote
Medical Center, Vrije Universiteit Amsterdam, Pediatric Oncology, Amster- for chemotherapy induced veno-occlusive disease(VOD) is costly
dam, Netherlands; 4 the Netherlands Comprehensive Cancer Organisation, and not accessible to majority getting treated at resource con-
Palliative Care, Utrecht, Netherlands; 5 Beatrix Children’s Hospital, Uni- strained settings. We describe the successful management of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S389 of S635

chemotherapy induced VOD with timely administration of N-acetyl most accurate method to measure it is indirect calorimetry (IC), how-
cysteine(NAC). ever, this method is expensive and not as feasible in clinical settings.
Methods: A total of 4 children developed chemotherapy induced VOD Our aim was to develop an equation for predicting resting energy
during the study period (2019-2021). All these patients received NAC expenditure in pediatric patients diagnosed with oncology based on
at a dose of 100mg/kg loading followed by 50mg/kg over 4hours then easily obtained anthropometric predictive variables.
100mg/kg continuous infusion till complete resolution of clinical and Methods: Cross-sectional study, including pediatric patients (6-18y)
laboratory features of VOD. with cancer diagnoses, the REE is measured by IC, anthropometric,
Results: First case was a 10-year-old boy,with non-metastatic clinical and body composition variables are measured by impedance.
right kidney Wilms tumor with renal vein thrombus who received Predictive equation proposals were derived using multiple linear
3-drug neo-adjuvant chemotherapy regimen. Post-week-6 of Vin- regression analyses.
cristine/Dactinomycin chemotherapy, he developed clinico-radiologic Results: One hundred patients with an average age of 12±2.9y have
features suggestive of VOD. Second case was a 4-year-old girl with been evaluated, according to the diagnostic stratum, 20% had a diag-
B-cell acute lymphoblastic leukemia (B-ALL) on modified BFM-95 nosis of leukemia, 65% solid tumor and 15% brain tumor. Two model
ALL protocol. She presented with clinical and laboratory features proposals were derived: the basic model that considered the vari-
suggestive of VOD during phase-1b consolidation. Ultrasound imaging ables of weight, age, sex (r=0.565, p<0.001), and the morphofunctional
and histopathology biopsy of liver confirmed underlying hepatic model with the variables: weight, age, sex, FFM, (r=0.596, p<0.001).
VOD. Third and fourth cases were of a 2-year girl and an 8-year boy When comparing the average calories vs IC (1179±387kcal), no signif-
with non-metastatic biliary and bladder rhabdomyosarcoma(RMS) icant differences were observed with the basic model (1115±197kcal,
respectively. Both of them developed clinical and laboratory features p=0.132), nor with the morphofunctional model (1101±223, p=0.129).
suggestive of VOD post 1st and 2nd cycle of Vincristine, Actinomycin The basic model presented an average bias of 90 (-479, 660) and
D,Cyclophosphamide (VAC) regimen respectively. In all these cases, the morphofunctional model 73 (-553, 679), compared to the existing
NAC was promptly infused on 2nd admission day and was continued equations: Harris and Benedict -174 (-75, 446), IOM -231 (-911, 449 ),
for a median of 4days(range:3-6days) following which VOD resolved FAO -207 (-830,414).
completely. Dactinomycin was implicated as the causative agent in Conclusions: The proposed equations had the highest predictive accu-
cases-1,3 and 4 and oral 6-mercaptopurine and cyclophosphamide in racy in pediatric patients diagnosed with oncology compared to the
case-2. existing equations.
Conclusions: Timely NAC infusion at the earliest clue of VOD in a
resource constrained setting, may prevent irreversible hepatic dam-
age as well as mortality. Besides being cheap, availability and excellent EP522 / #337 LEGACY-MAKING: EXPLORING CULTURAL
safety profile make NAC an attractive option in chemotherapy induced DIFFERENCES
VOD.
Lori Wiener1 , Amanda Thompson2 , Abigail Fry3 , Sokhna Ndiaye4 ,
Michael Mcneil5
EP521 / #400 DEVELOPMENT OF NEW PREDCITIVE 1 National Cancer Institute, NIH, Pediatric Oncology, Bethesda, United
EQUATION FOR RESTING ENERGY EXPENDITURE IN States of America; 2 Inova Schar Cancer Institute„ Life With Cancer, Fair-
PEDIATRIC PATIENTS WITH ONCOLOGY DIAGNOSIS fax, United States of America; 3 National Cancer Institute, NIH, Pediatric
Oncology Branch, Bethesda, United States of America; 4 University Hospital
Liliana Velasco-Hidalgo1 , Alda-Daniela García-Guzmán1 , Estíbaliz Aristide le Dantec, Pediatric Oncology, Dakar, Senegal; 5 St. Jude Children’s
Amairani Rodríguez-Aguilar2 , Beatriz Adriana Pinzón-Navarro3 , Research Hospital, Pediatric Hematology-oncology, Memphis, United States
Martha Guevara-Cruz4 , Marta Zapata-Tarrés5 , Rocío Cárdenas of America
Cardós1 , Isabel Medina-Vera2
1 National Institute of Pediatrics, Pediatric Oncology, Mexico City, Mexico; Background and Aims: Background: Legacy-making is an intervention
2 National Institute of Pediatrics, Methodology Research Unit, Mexico City, psycho-oncology and palliative care professionals use to help people
Mexico; 3 National Institute of Pediatrics, Gastroenterology And Nutrition living with cancer and their families prepare for end-of-life. Legacy-
Service, Mexico City, Mexico; 4 Instituto Nacional de Ciencias Médicas y making includes activities to preserve memories. There is a dearth of
Nutrición Salvador Zubiran, Departamento De Fisiología De La Nutrición, research on how legacy practices may differ by culture. This study
Mexico City, Mexico; 5 Fundación IMSS, Reserach Coordination, Mexico City, explores legacy practices that pediatric psycho-oncology and palliative
Mexico care professionals of different cultures and countries offer to chil-
dren, adolescents, and young adults (AYAs) with cancer, and perceived
Background and Aims: Accurate resting energy expenditure (REE) barriers to legacy-making.
predictive equations are crucial for designing nutritional strategies for Methods: An interdisciplinary study team designed an anonymous
patients with cancer diagnoses. The REE is the most widely used mea- survey on legacy practices in different countries and cultures. The
sure to estimate energy requirements in the nutritional context; the study instrument contained questions on respondent discipline, legacy
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S390 of S635 ABSTRACTS

practices for pediatric patients, and barriers to discussing legacy cre- Valacyclovir (N = 11, 3%), and IVIG (N = 6, 2%). Breakthrough infection
ation. Questions provided space for open-ended responses. The IPOS occurred in only N=6, 1.6%, all recovered. Only household exposure
Palliative Care Special Interest Group and the St. Jude Global Pallia- was significantly associated with infection. Chemotherapy was discon-
tive Care Transversal Program listserv were sent a survey link inviting tinued in N= 107, 42% of cases for 21-28 days. At the time of this
interested providers to participate. study, 70% of the exposed were alive, wherein 60% were in remission,
Results: 25 participants from 17 countries on 6 continents completed 10% in relapse and 30% expired from disease progression or other
the online survey. Responses were representative of the multidisci- causes.
plinary team, including psychologists, occupational therapists, social Conclusions: Post exposure prophylaxis with oral antivirals is effec-
workers, and physicians from multiple specialties. 57% of partici- tive in preventing infection among pediatric cancer patients. Break-
pants reported discussing creating a legacy with their pediatric and through infections were few and occurred among close household
AYA patients. Meaningful legacies included creative and expressive contacts. Cessation of chemotherapy during the incubation period
art making, creating special moments and memories, photo portraits, was significant cause of treatment delay. Findings from the study will
forming memorial foundations/charities, and participating in advance improve institutional varicella guidelines. Future studies will explore
care planning. Barriers to legacy creation included provider discomfort, benefits of varicella vaccination among low risk pediatric cancer
family reticence, timing, and lack of clinical staff and resources. Vari- patients.
ous cultural processes/considerations were noted, including the role of
family beliefs and lack of familiarity with the concept of legacy.
Conclusions: This study is a first step towards understanding cultural EP524 / #1932 THE NUTRITIONAL STATUS OF CHILDREN IN
differences in legacy-making practices with youth living with cancer. THE PEDIATRIC HEMATO-ONCOLOGY DEPARTMENT IN
Findings indicate that while many providers discuss legacy making with NOUAKCHOTT DESCRIPTIVE OBSERVATIONAL STUDY AIMED
their pediatric patients, ‘legacy’ may be an unfamiliar term in some AT ANALYSIS WITH COLLECTION OF PROSPECTIVE DATA
cultures and can have different meanings within and across cultures
Ekhtelbenina Zein
centre national oncology, Ministere Of Health, Nouakchott, Mauritania
EP523 / #295 OUTCOME OF VARICELLA EXPOSURE IN
PEDIATRIC PATIENTS WITH CANCER AT A TERTIARY Background and Aims: Context: in mauritania one in ten chil-
HOSPITAL: A THREE-YEAR EXPERIENCE dren is malnourished, the malnutrition of children with cancer limits
the therapeutic possibilities. the objective of this work is to take
Hazel Valerie Yu, Faustine Richelle Ong, Aileen Guerzon, Joliza stock of the nutritional status of our patients in order to prepare
Patricia Caneba, Jochrys Estanislao, Pamela Fajardo, Ana Patricia a nutritional management integrated into the care of children with
Alcasabas cancer
Philippine General Hospital, Pediatric Hematology And Oncology, Manila, Methods: Our study is a nutritional survey at the pediatric hemato-
Philippines oncology consultation at the Center National Oncology allowing the
recruitment of 100 children including 64 with cancer and 36 with
Background and Aims: Background/Significance: In the Philippines, benign blood diseases over a period of 3 months.
Varicella vaccine immunization is not included in the national immu- Results: The average age was 8 years The sex ratio is 2.03 in favor
nization program. Annual outbreaks occur during summer months. of boys. 74% of our patients live in urban areas 78% of our patients
For children with cancer, Varicella infection is associated with dis- had a low socio-economic level 42% of children were in school 58%
seminated disease and high mortality rates. Exposures may also of parents were illiterate This study showed the frequency of under-
cause treatment interruptions impacting survival rates. Objectives: To nutrition in our sick children regardless of the etiology. According to
describe the outcome of pediatric cancer patients exposed to varicella the HAS criteria, 50% of children with cancer were malnourished,
infection. 3.1% overweight and 4.7% were obese, 52.8% of non-cancer children
Methods: Retrospective, medical chart review of patients exposed were malnourished 20% of children had localized tumors, 16% had
to varicella from January 2018 to December 2020. Chi-square locally advanced tumors and 11% had metastases. children with metas-
test was used to examine the risk factors for breakthrough tases were undernourished at 63%, and those with localized tumors at
infection. 45%. The cancers that were most associated with malnutrition were
Results: A total of 380 patient exposures were identified in 301 Leukemia at 21.9%, nephroblastoma at 15.6%, retinoblastoma, germ
patients. The most common diagnosis was leukemia (N=195, 51%) fol- cell tumors, and osteosarcoma at 9.37%. 32% of children with can-
lowed by brain tumor (N=38, 10%), retinoblastoma (N=33, 9%) and cer were under chemotherapy, of which 59% were malnourished. 50%
sarcomas (N=33, 9%). Exposures occurred at the outpatient clinic (N of children were anorexic 28% of children had pain 64% of morbid
= 262, 69%), halfway house (N = 62, 16%), home (N= 11, 3%), hospital children were malnourished and cancerous.
party (N= 45, 12%). Majority were unvaccinated N = 374 (98%). Post Conclusions: the nutritional status of our patients justifies the integra-
exposure prophylaxis administered were: Acyclovir (N = 314, 95%), tion of nutritional support in the daily care of our patients.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S391 of S635

E-Poster Topic: AS05.q Psychosocial (PPO) Carolyn Rocha1 , Shilpa Nataraj2 , Nikhil Kumar3 , Bianca Perdomo4 ,
Euyhyun Lee5 , Matthew Banegas6 , Corrine Mcdaniels7 , Paula
E-POSTER VIEWING Aristizabal4,8,9
1 University of California, San Diego, School Of Medicine, La Jolla, United
EP525 / #1719 THE IMPACT OF SOCIAL DETERMINANTS OF States of America; 2 UCSF Benioff Children’s Hospital Oakland, Pediatrics,
HEALTH ON QUALITY OF LIFE AND PSYCHOSOCIAL Oakland, United States of America; 3 UC Irvine Children’s Hospital of Orange
OUTCOMES FOR CHILDREN WITH CANCER County, Pediatrics, Orange, United States of America; 4 University of Cal-
ifornia, San Diego, Pediatrics, La Jolla, United States of America; 5 UCSD
Ashley Anil, May Albee, Emily Moscato, Matthew Hocking Altman Clinical and Translational Research Institute, Biostatistics, La Jolla,
Children’s Hospital of Philadelphia, Behavioral Oncology, Philadelphia, United States of America; 6 University of California, San Diego, Radiation
United States of America Medicine And Applied Sciences, La Jolla, United States of America; 7 San
Diego State University, College of Health and Human Services, School Of
Background and Aims: Social determinants of health (SDH), such as Public Health, Epidemiology And Biostatistics, San Diego, United States of
income, race/ethnicity, and population density influence health-related America; 8 University of California, San Diego, Moores Cancer Center Pop-
quality of life (HRQL) in the context of chronic pediatric conditions. ulation Science, Disparities And Community Engagement, La Jolla, United
There is a need to understand SDH’s impact on HRQL and psychoso- States of America; 9 Rady Children’s Hospital San Diego, Peckham Center For
cial outcomes for children with cancer. To evaluate the impact of SDH Cancer And Blood Disorders, San Diego, United States of America
on HRQL and psychosocial outcomes, hypothesizing that more social
vulnerability, less access to care (e.g., further from hospital, lower pop- Background and Aims: Poverty is associated with poorer health out-
ulation density), and identification with a racially underrepresented comes. Research on household material hardship (HMH) and food
group would be associated with poorer HRQL and psychosocial func- security and associations with social determinants of health (SDoH) in
tioning. An exploratory aim was to determine whether diagnosis type diverse populations is lacking in pediatric cancer. To fill this gap, we con-
or treatment intensity moderated these associations. ducted cross-sectional and longitudinal assessments of HMH and food
Methods: Participants were youth ages 7-14 who completed treat- security in families of children with cancer.
ment within six months for either brain tumor (BT; n=42) or non- Methods: We prospectively enrolled parents of children with newly-
central nervous system solid tumor (ST; n=29). The Total Problems diagnosed cancer at Rady Children’s Hospital-San Diego, a large
subscale of the Child Behavior Checklist (CBCL) assessed psychosocial children’s hospital with high representation of Hispanics. Assessments
difficulties. Youth completed the Pediatric QOL Inventory (PedsQL 4.0) of HMH (food, housing, energy insecurity), food security, and SDoH
to assess HRQL. The Pediatric Neuro Oncology Rating of Treatment (health literacy, acculturation (if Hispanic), socio-demographics) were
Intensity measured treatment intensity. Population density, rural sta- conducted at 0, 3, 6, 12, and 24 months after diagnosis. Univariate
tus, and socioeconomic status (SES) were determined via the U.S. Cen- and multivariate analyses were used to determine associations at each
sus Bureau. Social vulnerability was calculated using the CDC/ATSDR timepoint and longitudinally.
Social Vulnerability Index (SVI), which captures household composi- Results: Of 107 parents included, 55% were Hispanic and 74%
tion/disability, minority/language, and housing/transportation. Analy- married. At baseline, 52% reported HMH and 24% reported food
ses included partial correlations and moderation models using the SPSS insecurity. In univariable analysis, public insurance was associated
PROCESS macro. with HMH (P=0.007) and food insecurity (P<0.001); associations
Results: For the BT group, distance from the hospital was significantly remained in multivariable analysis of HMH (P=0.046) and food insecu-
associated with CBCL, (r=0.35, p=.02) with a trend for HRQL (r=-0.29, rity (P=0.008). In univariable analysis, unmarried status was associated
p=.059). Neither treatment intensity nor diagnosis significantly mod- with HMH (P<0.001) and food insecurity (P<0.001); in multivari-
erated the association between distance and CBCL/HRQL. Other SDH able analysis, associations with HMH (P=0.004) and food insecurity
were not related to outcomes. (P=0.001) remained. In longitudinal analysis, unmarried individuals
Conclusions: Increased distance from the hospital may be associated and those with public insurance had higher likelihood of HMH (OR
with worse HRQL and psychosocial outcomes for BT patients. Future 3.399, P<0.001; OR 2.705, P=0.014) and food insecurity (OR 5.423,
work should investigate the cumulative impact of SDH longitudinally P<0.001; OR 4.091, P=0.011).
with a larger sample, as the cumulative impact of multiple risk factors Conclusions: HMH and food insecurity were highly prevalent in our
may be more substantial than any single factor. sample and associated with unmarried status and public insurance.
These associations persisted over time. Our findings contribute to the
scant literature in diverse populations, emphasizing the importance of
EP526 / #316 HOUSEHOLD MATERIAL HARDSHIP AND financial hardship screening and resources, particularly to underserved
FOOD INSECURITY IN CHILDREN WITH NEWLY DIAGNOSED individuals. Future directions include systematic assessments of HMH
CANCER: ASSOCIATIONS WITH SOCIAL DETERMINANTS OF and food insecurity in children with cancer, including those enrolled
HEALTH in clinical trials, and development and implementation of effective
interventions targeting HMH and food insecurity.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S392 of S635 ABSTRACTS

EP527 / #1388 EVALUATION OF THE PSYCHOLOGICAL EP528 / #1825 STRUCTURED PSYCHOLOGICAL SUPPORT
SUPPORT PROJECT FOR CHILDREN TREATED FOR CANCER IN MODEL IN PEDIATRIC ONCOLOGY ONCOLOGY : SENEGALESE
THE PAEDIATRIC ONCOLOGY UNIT OF ARISTIDE LE DANTEC EXPERIENCE
HOSPITAL (SENEGAL)
El Hadji Makhtar Ba1 , Sokhna Fatou Thioune2 , Soda Mamy Lo2 ,
El Hadji Makhtar Ba1 , Sokhna Fatou Thioune2 , Soda Mamy Lo2 , Momar Camara3 , Ndiaye Awa4 , Ndioba Mbengue1 , Alassane Seck2 ,
Momar Camara3,4 , Alassane Seck4 , Ndioba Mbengue2 , Fatou Ndoye Fatou Ndoye Fall5 , Pape Momar Gueye1 , Fatou Diagne4
Fall4 , Pape Momar Gueye1 , Fatou Diagne5 , Mame Diouf5 1 Université Cheikh Anta Diop, Unité D’oncopédiatrie/oncopediatric Unit
1 Université Cheikh Anta Diop, Unité D’oncopédiatrie/oncopediatric Unit (aristide Le Dantec Hospital), Dakar Peytavin, Senegal; 2 Aristide Le Dan-
(aristide Le Dantec Hospital), Dakar Peytavin, Senegal; 2 Aristide Le Dan- tec Hospital, Oncopediatric, Dakar, Senegal; 3 Université Cheikh Anta Diop,
tec Hospital, Oncopediatric, Dakar, Senegal; 3 Université Cheikh Anta Diop, Chnu Fann, Dakar Fann, Senegal; 4 Aristide Le Dantec Hospital, Pediatry,
Chnu Fann, Dakar Fann, Senegal; 4 Aristide Le Dantec Hospital, Oncopedi- Dakar, Senegal; 5 Aristide Le Dantec Hospital, Oncopediatric, Dakar Fann,
atric, Dakar Fann, Senegal; 5 Aristide Le Dantec Hospital, Pediatry, Dakar, Senegal
Senegal
Background and Aims: Psychological support in paediatric oncology
Background and Aims: The pediatric oncology unit was created since aimed to accompany patients, families and oncopediatri team. The
2000. In a holistic approach, a psycho-oncology team has been inte- originality lies in the fact that it was the first experience of a pae-
grated into the unit since July 2018 thanks to the support of the diatric onco unit with an integrated psycho-oncology team in West
Sanofi-Espoir Foundation. After one year of implementation (from July Africa.
1, 2020 to December 31, 2021), the Foundation wanted to evaluate the Methods: We carried out a retrospective, descriptive study. An evalu-
project. We conducted a survey. The general aim was to evaluate the ation from 1 July to 31 December 2020. Individual interviews, group
perceptions by stakeholders of psychological support activities. activities, psychomotricity, art therapy and story therapy were carried
Methods: A qualitative approach was used. It was based on individ- out. Used tools were genogram, quality of life scale for children and
ual interviews and stories of illnesses order to capture the discourse Spielberg anxiety test for team.
and points of view regarding the perceived effects. The targets of Results: 172 (115 children and 57 families) were consulted. Psycho-
the surveys were the project implementation teams, the authorities, logical support for children consisted of individual psychological inter-
the patients/families, and the associations of the patients’ families views, psychotropic drugs’prescription (delirium, anxiety-depression),
concerned. and "alternative" psychotherapy activities (art-therapy, story ther-
Results: The presence of the psychooncology team was positively apy and psychomotricity). The assessement of children quality of life
appreciated. Their contribution was considered satisfactory. The (Auquei) showed 73% of children with an alteration in one or more
activities mentioned were support during announcements (diagnosis, areas. 69 familial interviews and 39 post-diagnostic consultations were
relapse, palliative care, end of life), skill building on verbal and non- carried out. 14 specific accompaniments were set up for parents with
verbal communication techniques, weekly psychological interviews children at the end of life. 34 families were supported in the grief by
of the oncopediatric team. They fostered a better ability to manage calls. 57 accompanying persons received individual psychological sup-
negative emotions and deal with families or children’s emotional dis- port. Their families’ emotional distress was high (7.8/10 sd = 3.44).
tress. These statements were confirmed by the decrease in anxiety Three focus groups were conducted. Regular psychological monitoring
scores (Spielberg Anxiety Scale). The feeling of being psychologically was provided for oncopediatric team. The focus groups were very safe
supported was verbalized by team members and was seen as motiva- spaces, where the team was invited to share their emotions and all the
tion to continue their work. The alternative psychotherapy activities weight generated by the care and/or deaths of patients. The cohesion
offered were a great source of satisfaction for children and parents. activity was also a group activity bringing together all team members
They included art therapy, story therapy and psychomotricity. Free play (oncology and psycho-oncology), the framework was expressly more
groups for children were organized. These sessions allowed families to flexible and even almost playful.
see their children in a new light. This was reflected in the quality of life Conclusions: It has also enabled the first multidisciplinary pae-
measures (AUQUEI). A decrease of dropouts was noted. The families diatric psycho-oncology team to be set up. Indeed, childhood
raised communication problems. cancers cause profound psychological upheavals in the child
Conclusions: The integration of a multidisciplinary psychooncological and his or her entourage and are the cause of real systemic
team was one of the foundations of the success of this experiment, changes that must necessarily be considered in a holistic care
which is unique in West Africa. approach.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S393 of S635

EP529 / #468 SOCIAL AND COMMUNICATION BEHAVIORS 1 National Cancer Institute, Pediatric Oncology Branch, Bethesda, United
IN PEDIATRIC BRAIN TUMOR SURVIVORS COMPARED TO States of America; 2 Levine Children’s Hospital, Division Of Pediatric
HEALTHY CONTROLS USING THE AUTISM DIAGNOSTIC Psychology And Neuropsychology, Charlotte, United States of America;
OBSERVATION SCHEDULE (ADOS-2) DURING THE COVID-19 3 Children’s Hospital Colorado, Center For Cancer And Blood Disorders,
PANDEMIC Aurora, United States of America; 4 The Johns Hopkins Hospital, Pedi-
atric Oncology, Baltimore, United States of America; 5 National Cancer
Leandra Desjardins1 , Kelly Hancock2 , Meng-Chuan Lai3 , Jacob Institute, NIH, Pediatric Oncology Branch, Bethesda, United States of Amer-
Vorstman3 , Ute Bartels4 , Maru Barrera2 ica; 6 National Institute of Mental Health, Office Of The Clinical Director,
1 CHU Sainte-Justine, Psychology, Montreal, Canada; 2 Sick Kids Hospital„ Bethesda, United States of America
Psychology Department, Toronto, Canada; 3 Sick Kids Hospital, Psychiatry,
Toronto, Canada; 4 Sick Kids Hospital, Pediatrics, Neurooncology, Toronto, Background and Aims: Youth with serious illnesses often experi-
Canada ence medically related emotional and behavioral stressors that can
impact quality of life. Routine screening and assessment of psychoso-
Background and Aims: Pediatric brain tumor survivors (PBTSs) expe- cial factors is a well-documented and evidence-based Standard of
rience significant impairments in social competence. Impairments in Care, that can provide opportunities for appropriate referrals. Follow-
discrete social behaviors of PBTSs were previously identified using the ing a larger study examining feasibility of Checking IN, an e-screening
ADOS-2. Here we compared social behaviors assessed with the ADOS- measure, this study describes patient-reported psychosocial symptom
2 during the Covid-19 pandemic in PBTS and healthy controls (HCs), interference.
and examined cognitive and social adjustment correlates. Methods: Pediatric outpatients aged 8-21 and their caregiver com-
Methods: PBTSs (at least one year post treatment) and HCs, aged 8-16 pleted Checking IN. Participants were receiving outpatient treatment
years and English speaking, were recruited. Participants completed the for cancer (66%) or another chronic illness at 1 of 4 hospital cen-
ADOS-2, WASI-II (Full Scale IQ), WISC-V’s subscales digit span (work- ters. Checking IN is a brief and interactive screening measure designed
ing memory) and coding (processing speed). Caregivers completed the to assess psychosocial symptom interference across the domains of
CBCL. The ADOS-2 is a semi-structured interactive interview to code anxiety, depression, anger, attention, body image, sleep disturbance,
thirty-two behavior items during social interaction, with higher scores fatigue, pain, medication adherence, family relationships, peer relation-
reflecting higher impairment. Recruitment took place during the pan- ships, faith, and school.
demic (March, 2021-March, 2022). Participants and examiners wore a Results: One hundred (100) participants aged 8 to 21 (M=14.27,
surgical face mask during all assessments. SD=3.81) completed Checking IN and their caregiver completed the
Results: 16 PBTSs (mean age 13.24; 56% female) and 16 HCs (mean proxy screener. Generally, patients endorsed low level impact (“a little
age 14.8; 75% female) participated. Diagnoses in PBTSs included low- bit” or “sometimes”) versus high (“often” or “almost always”) of distress
grade glioma, ependymoma, medulloblastoma and germinoma (19% domains. The symptoms most frequently endorsed by youths were
each). Compared to HCs, PBTSs presented with more social prob- Fatigue ((Low: n=50, 50%; High: n=21, 21%), Paying Attention (Low:
lems (p=.03), lower FIQ (p=.07), and lower processing speed (p=.005). n=45, 45%; High: n=16, 16%), Sleep Difficulty (Low: n=46, 46%; High:
ADOS-2 overall scores for PBTS were higher (.08) than for HC n=13; 13%), and Worry (Low: n=46, 46%; High: n=8, 8%). Caregiver
(.03) (p=.07) and were correlated with CBCL social problems (r=.41, proxy responses were concordant with those of youth, evidenced by
p=.056), FSIQ (r=-.48; p=.03) and processing speed (r=-.34, p=.11). absence of significant differences between them on any domain.
Items showing higher impairment in PBTSs compared to HCs included: Conclusions: This study suggests that Checking IN identifies areas of
comments on other’s emotions (44% vs. 0%), using imagination (44% vs. psychosocial distress in youth with serious medical illnesses in the out-
19%), insight into social situations/relationships (19% vs. 0%), offering patient setting across 4 hospital centers. Symptoms of fatigue, sleep,
information (19% vs. 6%), conversation (38% vs. 25%), and reciprocal attention, and worry were endorsed most frequently in these youth
social communication (33% vs. 23%). with a serious illness, as well as on caregiver proxy report. Use of
Conclusions: Compared to HCs, PBTSs showed impairments in social- Checking IN may expand opportunities for providers to appropriately
communication behaviors, as well as cognitive and social adjustment. address problematic symptoms of psychosocial distress.
These findings bear important implications for understanding social
interactions in the context of the pandemic and the development of
targeted social skills programs for PBTSs. EP531 / #704 ADOLESCENT AND YOUNG ADULT (AYA)
CANCER SURVIVORS IN SWITZERLAND: FOCUS ON POSSIBLE
POSITIVE OUTCOMES
EP530 / #619 PEDIATRIC PATIENTS CHECK IN: A LOOK AT
SELF-REPORTED PSYCHOSOCIAL SYMPTOM INTERFERENCE Céline Bolliger1 , Pauline Holmer1 , Silvia Dehler2,3 , Katharina Roser1,4 ,
Gisela Michel1,4
Sima Bedoya1 , Mallorie Gordon1 , Amii Steele2 , Robert Casey3 , Devon 1 University of Lucerne, Department Of Health Sciences And Medicine,
Ciampa4 , Abigail Fry5 , Maryland Pao6 , Lori Wiener1 Luzern, Switzerland; 2 University of Zurich, Institute of Surgical Pathology,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S394 of S635 ABSTRACTS

University Hospital Zurich and Epidemiology, Biostatistics and Prevention Minnesota, Children’s Minnesota, Minneapolis, United States of America;
Institute, Cancer Registry Zurich And Zug, Zürich, Switzerland; 3 Office of 7 University of Arizona, College Of Nursing, Tucson, United States of America

Public Health, -, Vaduz, Liechtenstein; 4 Joint last authorship, Switzerland


Background and Aims: Physical side effects of childhood acute lym-
Background and Aims: Adolescents and young adults (AYAs) are phoblastic leukemia (ALL) therapy are well-described; however, less
diagnosed with cancer during a unique and challenging period of is known about mental health outcomes. The objectives of this study
their life. This experience may result in positive changes called post- were to describe the prevalence of suicidal ideation (SI) during ALL
traumatic growth (PTG) later on. We aimed to 1) describe PTG and therapy and investigates whether SI is influenced by clinical factors or
illness perception in Swiss AYA cancer survivors and 2) determine the physical symptoms.
association between PTG and sociodemographic and cancer-related Methods: Patients age 7-18 years with newly-diagnosed ALL were
characteristics as well as illness perception. recruited from three U.S. childhood cancer treatment centers (2012-
Methods: We conducted a population-based questionnaire survey in 2017). The Children’s Depressive Inventory (CDI-2) was administered
AYA cancer survivors diagnosed 1990-2005 at age 16-25 years, reg- at start of consolidation, delayed intensification (DI), maintenance
istered in the Cancer Registry Zurich and Zug, Switzerland, who had cycle 1 (MC1), and maintenance cycle 2 (MC2). SI was considered
survived at least five years. PTG was assessed using the German present if patients endorsed the “I want to kill myself” item. Logistic
version of the Posttraumatic Growth Inventory (PTGI), and illness regression models evaluated associations between SI and sociodemo-
perception using the Brief illness Perception Questionnaire (BIPQ). graphic factors; depressive symptoms; and symptom clusters identified
We further assessed sociodemographic characteristics, while cancer- using latent class analysis (LCA) of patient-reported pain, nausea,
related information was obtained from the Cancer Registry. Data were fatigue, and sleep also assessed at the start of each phase of therapy.
analyzed using descriptive statistics and univariable and multivariable Results: Participants (n=175) were 51% male, 75% high-/very high-
linear regressions. risk, with a median age of 11.2 years at diagnosis. LCA identified three
Results: Among 469 eligible survivors, 389 could be contacted, and symptom clusters characterized by individuals with a below average
160 were included in the analysis (61.3% male; mean age=34 years, (n=49), average (n=82), and above average (n=44) symptom burden
SD=5.8). The average PTG score was 54.63 (SD=20.24; range: 8- throughout treatment. Overall, 26 patients (14.9%) endorsed SI at
101) and they reported PTG especially in the scales Appreciation of least once during treatment, including 4% at consolidation, 9% at DI,
life (mean=3.23; 95% confidence interval [CI], 3.05-3.40), followed 8% at MC1, and 4% at MC2. In adjusted models, non-Hispanic others
by Personal strength (2.94; 2.77-3.12), Relating to others (2.57; 2.40- were 10.9-times more likely than non-Hispanic whites to endorse SI
2.74), New possibilities (2.35; 2.35-2.54), and Spiritual change (1.46; (p=0.003). The frequency of SI was also higher in patients reporting
1.21-171). Neither sociodemographic nor clinical characteristics were above average symptoms (53.3%) than those with below average symp-
associated with PTG. AYA cancer survivors who perceived follow- tom severity (4.1%, p=0.003). Depressive symptoms were consistently
up care as helpful (p<0.001) and those with high concern about associated with SI.
consequences of the illness (p<0.001) reported higher PTG. Conclusions: SI occurring during the initial year childhood ALL ther-
Conclusions: The majority of Swiss AYA cancer survivors reported to apy is associated with increased physical symptom severity, depressive
have developed some PTG, and thus experienced some positive out- symptoms and certain sociodemographic features. Findings highlight
comes. Finding ways to promote PTG and to identify and address the need for improved screening of mental health problems to mitigate
maladaptive illness perceptions throughout the illness course may help or alleviate symptoms of distress in this at-risk population.
survivors transforming their experience of life-threatening disease into
something meaningful for their future life.
EP533 / #114 INFLUENCE OF ETHNICITY AND PARENTS’
EATING HABITS ON DIET QUALITY IN PEDIATRIC CANCER
EP532 / #767 PREVALENCE AND CORRELATES OF SUICIDAL SURVIVORS
IDEATION REPORTED BY CHILDREN AND ADOLESCENTS
DURING TREATMENT FOR ACUTE LYMPHOBLASTIC LEUKEMIA Acadia Buro1 , Heewon Gray2 , Rachel Sauls3 , Sylvia Crowder1 , Marilyn
Stern4
Austin Brown1 , Kimberly Raghubar1 , Tiffany Chambers2 , Ryan Hill2 , 1 Moffitt Cancer Center, 4117 E Fowler Ave, Tampa, United States of Amer-

Olga Taylor3 , Marilyn Hockenberry4 , M Hooke5 , Pauline Mitby6 , Ida ica; 2 University of South Florida, College Of Public Health, Tampa, United
Moore7 , Michael Scheurer2 States of America; 3 University of South Florida, Department Of Psychology,
1 Baylor College of Medicine, Pediatrics - Oncology, Houston, United States Tampa, United States of America; 4 University of South Florida, Child And
of America; 2 Baylor College of Medicine, Pediatrics, Houston, United States Family Studies, Tampa, United States of America
of America; 3 Texas Children’s Hospital, Baylor College of Medicine, Pedi-
atrics, Houston, United States of America; 4 Baylor College of Medicine, Background and Aims: Pediatric cancer survivors (PCS) exhibit poor
Global Hope, Houston, United States of America; 5 University of Minnesota, adherence to dietary guidelines and have an increased risk of obe-
School Of Nursing, Minneapolis, United States of America; 6 University of sity and endocrine complications. As family and culture play important
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S395 of S635

roles in shaping children’s eating behaviors, research is needed to (27.85%). The evaluation was done at a median age of 5 years (2 to 13
investigate the impact of these factors on children’s diet quality to years) after treatment completion and the median age of time since
tailor nutrition interventions for PCS. The aims of this study were diagnosis was 6 years (2 to 16 years). One or more behavioural prob-
to examine associations in diet quality (1) between Hispanic vs. non- lems were found in 35 (44.30%) patients. Out of these 35 patients,
Hispanic PCS and (2) between PCS and their parents. parents of 23 (65.71%) patients had an awareness prior to the assess-
Methods: This is a cross-sectional analysis of data collected from 46 ment that their child has some behavioural issue(s). Internalizing
PCS with overweight/obesity (off treatment for ≥ 6 months; mean age problems were found in 10/79 (12.66%) patients (attention problems
10 years; 40% Hispanic) and their parents. Participants completed a 1- (n=12, 15.18%), anxiety issues (n=3, 3.79%) and depression (n=2,
day 24-hour dietary recall. The Healthy Eating Index-2015 (HEI-2015) 6.32%). Externalizing problems were found in 19 (24.05%) patients
was calculated to assess diet quality. T-tests were performed to exam- (conduct problems (n=12, 15.18%) and oppositional-defiant behaviour
ine differences in HEI overall and component scores between Hispanic (n=2, 2.53%)). Gender and time since treatment completion & evalu-
vs. non-Hispanic PCS, and associations in HEI scores between parents ation had no significant effect on the patient having internalizing and
and PCS were examined with Pearson correlations. externalizing problems. PTSD was screened in 8/79 (10.12%) patients
Results: The mean HEI score was 50.2 for children and 51.9 for par- while taking cancer as the event of trauma.
ents out of 100. Ethnicity was not associated with overall HEI score, Conclusions: About half (44.30%) of the childhood cancer survivors
but Hispanic PCS had significantly lower total vegetable and fatty acid experience significant long-term behavioural and psychological conse-
scores (p=0.02) compared to non-Hispanic PCS. Significant correla- quences. Because psychological health is a significant factor in person’s
tion was found between child and parent overall HEI scores (r=0.56, quality of life, survivors must be closely monitored for co-morbid
p<0.0001). Children’s and parents’ component scores were correlated behavioural and psychological consequences.
for added sugar (r=0.45, p=0.005), sodium (r=0.42, p=0.005), greens
and beans (r=0.33, p=0.02), whole grains (r=0.39, p=0.007), and fatty
acids (r=0.31, p=0.04). Other findings were not significant. EP535 / #1005 MINDFULNESS AND INTERNALIZING
Conclusions: Findings indicated poor diet quality scores in PCS with SYMPTOMS AS PREDICTORS OF FEAR OF RECURRENCE IN
overweight/obesity and their parents. Significantly high correlations CAREGIVERS OF PEDIATRIC CANCER SURVIVORS
between parent and child diet quality justifies using parents as change
agents in nutrition interventions for PCS. Cultural factors and parent Adora Choquette1 , Anna Jones2 , Brian Potter1 , Elizabeth Barnwell3 ,
role modeling should be considered in future interventions to promote Emily Browne3 , Sandra Jones3 , Sylwia Feibelman1 , Rachel Tillery
healthy eating in this population. Webster4
1 St. Jude Children’s Research Hospital, Department Of Psychology, Mem-
phis, United States of America; 2 Department of Psychology, St. Jude
EP534 / #1429 BEHAVIOURAL AND PSYCHOLOGICAL Children’s Research Hospital, Memphis, United States of America; 3 St. Jude
EFFECTS OF CHILDHOOD SOLID MALIGNANT TUMORS AND Children’s Research Hospital, Transition Program, Memphis, United States
ITS MANAGEMENT of America; 4 St. Jude Children’s Research Hospital, Psychology, Memphis,
United States of America
Mansi Chadha1 , Vishesh Jain1 , Sandeep Agarwala1 , Renu Sharma2 ,
Sameer Bakhshi3 , Anjan Dhua1 , Prabudh Goel1 , Devendra Yadav1 Background and Aims: Despite being common, caregiver fears of their
1 All India Institute of Medical Sciences, New Delhi, Department Of Paedia- child’s cancer recurrence (FCR) after the transition off treatment (TOT)
triic Surgery, New Delhi, India; 2 All India Institute of Medical Sciences, New are associated with negative health outcomes. Patient demographic
Delhi, Department Of Psychiatry, New Delhi, India; 3 All India Institue of and medical factors have been linked to FCR, yet little is understood
Medical Sciences, Department Of Medical Oncology, New Delhi, India about modifiable psychological mechanisms that may be linked to FCR
in caregivers. The goal of this research was to explore psychological
Background and Aims: A large number of survivors of paediatric solid risk (e.g., anxiety, depression) and protective factors (e.g., mindfulness)
tumours experience significant disease or treatment related sequelae. associated with FCR at TOT.
These adverse health outcomes can be both physiological, psycholog- Methods: Participants were 84 caregivers (Mage =41.18, SDage =8.01,
ical, and behavioural. The aim of the present study was to assess the 90.7% mothers, 72% white) recruited from a pediatric oncology outpa-
long-term psychological and behavioural effects faced by the survivors tient clinic as part of a study examining psychosocial factors associated
of solid malignant tumours. with TOT among pediatric cancer survivors and their caregivers. Care-
Methods: Survivors were assessed for behavioural problems with help givers completed the four-item versions of the PROMIS Anxiety and
of Child Behaviour Checklist (6-18 years). They were also screened for Depression scales. FCR was examined via three items from the Pedi-
post-traumatic stress disorder (PTSD) with the help of Children Impact atric Quality of Life – Cancer Module. Mindfulness was examined
of Event Scale (CRIES-13). via the Five Facets of Mindfulness Questionnaire. Regression analy-
Results: Seventy-nine survivors with a median age of 10 years (6-18 ses were used to explore associations between FCR with caregiver
years) were included. There were 57 males (72.15%) and 22 females internalizing symptoms and mindfulness, while controlling for relevant
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S396 of S635 ABSTRACTS

demographic and medical variables. Non-significant variables were agement of complex relational situations with families. Participants
removed from the final models. significantly value being able to share and understand feelings or expe-
Results: Demographic and medical variables were not significantly riences derived from work, as well as feeling supported and understood
associated with FCR and were removed from the model. Model two by the group. There is evidence of an improvement in greater personal
included depression, anxiety, and mindfulness as predictors of FCR and and group care, understanding and support in emotional issues, and
was significant (R2 =.375, F(3,80)=16.032, p<.001). Mindfulness was better ability to express concerns.
negatively associated with FCR (b=-.045, p<.05), and anxiety was pos- Conclusions: The Balint Group is serving as support for health profes-
itively associated with FCR (b=.518, p<.001). When exploring similar sionals as a space to share concerns, as an engine of communication
models with FCR predicting depression and anxiety, the models were and group cohesion, as a facilitator of the understanding of the emo-
non-significant. tional universe, and as a promoter of better care of patients and
Conclusions: Elevated anxiety, but not depression, was significantly families. The participants recommend this resource and wish to con-
associated with FCR, but the reverse was not true (FCR pre- tinue participating considering that it is a medium and long-term aid
dicting anxiety). This suggests interventions targeting caregiver resource.
anxiety at TOT may be of benefit in reducing FCR. Interven-
tions that focus on building mindfulness skills may also serve to
reduce FCR. These will be important avenues to explore in future EP537 / #581 THE NEEDS OF FAMILIIES DURING THE 1ST
research. MONTH AFTER THEIR CHILD IS DIAGNOSED WITH CANCER

Megan Cruise
EP536 / #691 THE BALINT GROUP AS A RESOURCE FOR World Child Cancer, Programmes, London, United Kingdom
EMOTIONAL CARE IN A PAEDIATRIC ONCOLOGY UNIT OF A
TERTIARY HOSPITAL Background and Aims: Through World Child Cancer’s psychosocial
support work, we strive to improve quality of life and wellbeing for
Rosalía González Moraleda1 , Leire Collazos Zabala1 , José María families and to reduce treatment abandonment. We provide financial
Erroteta Palacio2 , Maria Jose Lopez De La Serna3 support to families, along with emotional, practical, and educational
1 Hospital Universitario Cruces - Fundación Aladina, Unidad De Hema- support. Previous studies have shown that treatment abandonment
tología Y Oncología Pediátrica, Vizcaya, Spain; 2 Hospital Universitario mainly happen in the first few weeks or months after a child in a low
Cruces, Servicio De Psiquiatría, Vizcaya, Spain; 3 Hospital Universitario and middle-income country, is diagnosed with cancer. Following these
Cruces, Paediatric Hematology And Oncology, Vizcaya, Spain previous studies, we used the results of a psychosocial support survey
to analyse the needs of families during this initial stage after diagnosis.
Background and Aims: Working in Paediatric Oncology entails Methods: A semi-structured beneficiary feedback survey was used.
high emotional demands and daily exposure to intense affec- The survey focused on 5 key areas: About you, Quality, Impact, Future
tive burden for which it is not easy to cope with. This paper improvements, Support provided by nursing team. The survey was con-
describes and evaluates the operation of a Balint Group as ducted at different stages of treatment: within 1st month of diagnosis,
an emotional protection factor, provider of emotional cop- currently receiving treatment, recently finished treatment, and post
ing resources, and generator of psychosocial well-being in the treatment. For the purpose of this research, we analysed data from
professionals. families who were within the 1st month after diagnosis.
Methods: We evaluated the functioning of a Balint Group in Paediatric Results: Of the 115 surveys completed in 2021, 17% were completed
Oncology of the Cruces University Hospital through the observation by families in the 1st month of their child’s treatment. Respondents
of the E-Poster Topics addressed in sessions and the analysis of a ques- were asked, ‘How important was the financial support you received in
tionnaire filled out by the participants. The group began in 2019 with being able to treat your child?’ The majority (90%) responded ‘Critical
17 participants (doctors, nurses, psychologists and a psychiatrist lead- - without it, we would never have been able to treat our child’. When
ing the group) who meet weekly. Every session a participant raises asked ‘What was the most important type of support you received?’
emotional difficulties related to his work and all together try to under- 65% said financial support for drugs and diagnostic and 25% said finan-
stand such difficulties, overcome them and learn how to cope with cial support for transport costs. 95% of respondents said they would
them. have liked access to a professional to talk to, about how they were
Results: The most frequently addressed E-Poster Topics have been feeling.
team’s communication, personal involvement at work, relational diffi- Conclusions: The survey highlights the critical needs of families during
culties with families, bereavement coping, adequacy of the diagnosis the first month after their child was diagnosed and the importance of
information and emotional support of patients. Those that have been financial and psychosocial support being available for families, in order
of most interest have to do with team’s communication and man- for them to continue with their child’s treatment.
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ABSTRACTS S397 of S635

EP538 / #1274 UNDERSTANDING AND IMPROVING EP539 / #1683 SOCIAL SUPPORT TO DECREASE
FEEDING AND EATING PRACTICES IN CHILDREN WITH ABANDONMENT RATE IN LMICS : EXPERIENCE OF MY CHILD
CANCER IN INDIA: DEVELOPING A MANUALIZED MATTERS PROGRAM IN THE GFAOP PEDIATRIC UNIT IN
PSYCHOLOGICAL FAMILY INTERVENTION SENEGAL

Rhea Daruvala1 , Anne Roefs2 , Leandra Desjardins3 , Sunil Bhat1 , Fatou Binetou Diagne1 , Mame Diouf1 , Ndiaye Awa1 , Catherine
Soumitra Datta4 , Lotte Lemmens5 Patte2 , Anta Niang1
1 Mazumdar Shaw Cancer Center, Department Of Pediatric Hematology, 1 Aristide Le Dantec Hospital, Pediatric Oncology, Dakar, Senegal; 2 French
Oncology And Bmt, bangalore, India; 2 University of Maastricht, Depart- Pediatric Oncology Group(GFAOP), Gustave Roussy Institute, Paris, France
ment Of Clinical Psychological Science Faculty Of Psychology And Neu-
roscience, PO Box, Netherlands; 3 CHU Sainte-Justine, Psychology, Mon- Background and Aims: Pediatric oncology services are very costly
treal, Canada; 4 Tata Medical Center, Palliative Care And Psycho-oncology, for low and middle income countries(LMICS). The pediatric oncology
kolkata, India; 5 University of Maastricht, Department Of Clinical Psy- unit(UOP) in Dakar, Senegal, receives approximately 200 new cases
chological Science Faculty Of Psychology And Neuroscience, maastricht, yearly. The major contributors to treatment failure are poverty, lack
Netherlands of social insurance, and abandonment. In order to promote early diag-
nosis, rapid initiation of treatment and reduce abandonment, My Child
Background and Aims: Paediatric cancers affect up to 400,000 Matters(MCM) program of the Sanofi Espoir Foundation has been sup-
children yearly worldwide(WHO, 2021). Almost half of the pae- porting the UOP since 2007. We describe the social support from June
diatric patients undergoing cancer treatment experience malnutri- 2020 to December 2021 and its impact.
tion(Bauer et al., 2011). In low- and middle income(LAMI) coun- Methods: Monthly financial support was given to families by the social
tries this number is estimated to be even as high as 59%(Sharma worker for biological and radiological exams, chemotherapy adjuvants
et al., 2015). It is therefore important to improve the nutritional and transportation costs. A total amount of 37591,46 euros allowed
status of patients throughout cancer treatment as malnutrition neg- the implementation of the program. Data were collected from the
atively affects disease related outcomes(Sala et al., 2004). The social worker register, and GFAOP-Registry.
aim of the current study was therefore to better understand fac- Results: During the period, 369 children were supported. The mean
tors affecting feeding and eating practices during paediatric cancer age was 6.8 years. The most common cancers were leukemia (26%),
treatment. nephroblastoma (21%), retinoblastoma (6%), Burkitt lymphoma (6%),
Methods: Nurses, doctors, nutritionists, mental-health-care profes- Hodgkin lymphoma (4%). 75% of patients lived outside of Dakar, where
sionals, parents, and children(n=46) in a paediatric cancer hospital in the UOP is located. The average distance from the UOP was 200 kilo-
India were asked to provide inputs on feeding and eating related chal- meters, 43% of families had no accommodation during treatment. The
lenges and strategies using both qualitative focus group interviews and socio-economic level was low in 80%, fathers being involved in infor-
quantitative questionnaires. mal activities with irregular income. 82% of mothers were unemployed,
Results: In all six groups(n=46),feeding and eating related strategies and 90% of the families had no medical insurance. Financial support for
and challenges could be categorized into being cognitive, behavioural, adjuvant chemotherapy was preponderant, followed by biological, radi-
parental, or nutritional factors. Children were capable of identifying ological exams and transportation. Abandonment decrease from 20.3%
what works for them and wanted to be included in decisions around in 2018 to 2.7% in December 2021.
feeding and eating. Parents could benefit from psychoeducation about Conclusions: MCM social support of Sanofi Espoir Foundation showed
cancer and all aspects of its treatment as misconceptions around feed- its effectiveness. It demonstrated that strong political willingness and
ing, eating and nutrition were common. Time pressure and patient sustainable funding initiatives are needed for better management of
volumes often did not allow medical teams to focus on eating prac- children with cancer, and their families, in Senegal.
tices with parents and children as disease and treatment was a priority.
Instead, nurses and nutritionists could play a central role here, as they
spend most time caring for patients, and have experience with feeding EP540 / #1736 BEREAVED PARENTS’ PERSPECTIVE ON
and eating. General mental health professionals could also play a role, COMMUNICATION OF TRANSITION TO PALLIATIVE CARE IN
but may need additional training in dealing with this specific patient PEDIATRIC ONCOLOGY - HUNGARIAN EXPERIENCE
population.
Conclusions: Including various stakeholders in designing psychological Enikő Földesi1 , Szilvia Zörgő1 , Judit Nyirő1 , Katalin Hegedűs1 , Peter
interventions in LAMI countries is practically challenging yet relevant Hauser2
and necessary.Information from phase 1 will further be used to design 1 Semmelweis University, Institute Of Behavioural Sciences, Budapest, Hun-

and test a new manualized psychological family to address FEP’s in gary; 2 Borsod-Abaúj-Zemplén County University Teaching Hospital, Velkey
India. László Child’s, Health Center, Miskolc, Hungary
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S398 of S635 ABSTRACTS

Background and Aims: Transition to palliative care (PC) is a critical Methods: Two community advisory boards (CABs) were formed,
aspect of pediatric oncology requiring a high level of communication including a group of n = 6 Spanish-speaking parents of children who
skills from doctors, which could be best judged by parents of children had completed cancer treatment and a group of n = 5 Spanish-speaking
died in cancer. Our aim was to explore parents’ perspectives regarding parents whose children had begun treatment for cancer in the previ-
the timing of the consultation on implementing PC, as well as facets of ous 16 weeks. Following CBPR principles, multiple meetings with the
verbal and non-verbal communication in Hungary. CABs and academic researchers were held over a period of four years.
Methods: Semi-structured interviews were conducted with parents All meetings were audio recorded and transcribed to identify barriers
who had lost their child to cancer within the past 1-5 years. Interview to optimal quality of life during cancer treatment in the target commu-
transcripts (n=23) were scrutinized with Interpretative Phenomeno- nity as well as culturally-informed intervention components that would
logical Analysis. ameliorate disparities in psychosocial outcomes in Spanish-language
Results: Parents frequently associated palliation with end-of-life care families
and clearly delimited the transition to PC after curative treatments had Results: Four domains of barriers were identified that impacted treat-
been exhausted. Parents were ambivalent regarding using the word ment experience and cancer health outcomes: healthcare system,
“death” during this consultation and often did not receive information healthcare providers, family, and parent/caregiver. A twelve-session
on what to expect (e.g., regarding symptoms) and who to turn to for family-focused intervention was developed that included three tar-
further information or support (e.g., concerning bereavement). gets: health literacy, culturally competent care, and caregiver well-
Conclusions: Although significant progress could be observed ness. Specific intervention components included complementary and
in the organization of pediatric palliative care in Hungary, alternative medicine, psychoeducation, fitness, gardening, culinary
there is still no widely accepted communication method for medicine, and multiple strategies to improve health literacy
communication of transition to sole PC. There is a need for a Conclusions: The use of CBPR is an effective approach to develop cul-
culturally-sensitive approach to refining recommendations on turally relevant interventions that may reduce cancer health disparities
word-use and communication protocol in pediatric PC also in in Spanish-language families impacted by childhood cancer.
Hungary.

EP542 / #203 UNDERSTANDING THE INFLUENCES OF


EP541 / #1985 DEVELOPMENT OF A CULTURALLY ADAPTED HCP-PATIENT INTERACTIONS IN CANCER CARE FOR LGBTQ+
INTERVENTION TO IMPROVE QUALITY OF LIFE IN CHILDREN AND YOUNG PEOPLE
SPANISH-SPEAKING FAMILIES IMPACTED BY CHILDHOOD
CANCER Tamsin Gannon1 , Bob Phillips2 , Daniel Saunders3 , Alison Berner4,5
1 The Royal Marsden NHS Foundation Trust, Paediatric Oncology, Sut-
Michelle Fortier1 , Lessley Torres1 , Haydee Cortes2 , Belinda Campos3 , ton, United Kingdom; 2 University of York, Paediatric Oncology, Yorkshire,
Zeev Kain2 , Lilibeth Torno4 , Carol Lin4 United Kingdom; 3 The Christie NHS Foundation Trust, Radiation Oncol-
1 University of California Irvine, Sue & Bill Gross School Of Nursing, Irvine, ogy, Manchester, United Kingdom; 4 Tavistock & Portman NHS Foundation
United States of America; 2 University of California Irvine, Uci Center On Trust, Gender Identity Clinic, London, United Kingdom; 5 Mount Vernon Can-
Stress And Health, Irvine, United States of America; 3 University of Cal- cer Centre East and North Hertfordshire NHS Trust, Oncology, Northwood,
ifornia Irvine, Chicano/latino Studies, Irvine, United States of America; United Kingdom
4 CHOC Children’s Hospital, Hyundai Cancer Center, Orange, United States
of America Background and Aims: The lesbian, gay, bisexual, transgender, queer
or questioning (LGBTQ+) population experience inequalities through-
Background and Aims: Caregivers of children with cancer experi- out the continuum of cancer care. These are compounded for children,
ence significant psychological distress and cancer-health disparities adolescents and young adults who may be at a critical time for explor-
experienced by marginalized populations further exacerbate dispari- ing their gender identity and sexual orientation, as well as facing a
ties among children with cancer and their caregivers. Efforts to reduce cancer diagnosis. Previous studies have examined knowledge, atti-
health disparities have illuminated the need to unite researchers tudes and behaviours of oncologists treating LGBTQ+ adults and how
with communities in the development and utilization of culturally these might contribute to those inequalities. However, there is lim-
relevant and appropriate health care interventions. Participatory ited research on those treating LGBTQ+ children and adolescents with
research approaches, such as community-based participatory research cancer. We investigated knowledge, attitudes and behaviours of pae-
(CBPR) methods can meet needs for patient-centered methodologi- diatric, teenage and young adult oncology healthcare professionals
cal approaches that engage patients in healthcare improvement and (HCPs) treating and supporting LGBTQ+ patients in the UK with the
decision-making. The purpose of the present study is to describe the aim of identifying ways to improve care for this group.
use of CBPR to develop a culturally adapted intervention to improve Methods: We carried out semi-structed interviews with HCPs in paedi-
quality of life in Spanish-speaking families impacted by childhood atric, teenage and young adult oncology at a UK specialist cancer cen-
cancer. tre. Questions centred around participants’ knowledge, attitudes and
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ABSTRACTS S399 of S635

behaviours regarding management of LGBTQ+ patients in oncology. ated (p=0.794). Most adolescent patients read the study information
Interview transcripts were analysed by inductive thematic analysis. sheet either ‘briefly’ or ‘not at all’ (n=16/23; 70%) and on average had a
Results: We identified 13 themes and subthemes, of which 12 were perceived understanding score of 63.6/100 (n=23, SD=21.7).
mapped to a new framework for improving HCP-patient interactions Conclusions: Our research reinforces the challenge of communicating
in LGBTQ+ Cancer Care. This framework highlights the cyclical nature complex information to families about precision medicine for child-
of knowledge acquisition, attitude shifts and behavioural change. We hood cancer. Our findings have informed the development of animated
identified needs around cultural and organisational change and indi- videos which explain childhood cancer precision medicine processes
vidual HCP education. As an enabler of tailored care, disclosure of and convey key themes of parents’ experiences of the journey.
identity was a major theme and we identified multiple barriers to, and
facilitators of, HCP enquiry and patient disclosure.
Conclusions: Knowledge, attitudes and behaviours of HCPs are inter- EP544 / #1344 PSYCHOSOCIAL ISSUES, COPING
dependent. Our framework, with a particular focus on disclosure, can STRATEGIES AND PSYCHIATRIC DISORDERS IN ADOLESCENT
be used to support HCP education and organisational change. The CANCER PATIENTS REFERRED TO PSYCHO-ONCOLOGY
resulting increase in disclosure and provision of tailored care has SERVICES: AN OBSERVATIONAL STUDY IN A LOW MIDDLE
the potential to improve the patient experiences, engagement with INCOME COUNTRY
healthcare and later outcomes for LGBTQ+ young people with cancer.
Savita Goswami1 , Jayita Deodhar2 , Lekhika Sonkusare1
1 Tata Memorial Hospital, Psychiatry And Psycho-oncology, Mumbai, India;
EP543 / #19 PARENTS’ AND PATIENTS’ UNDERSTANDING 2 Tata Memorial Hospital, Department Of Palliative Medicine & Psychiatry,
OF A PRECISION MEDICINE TRIAL FOR HIGH-RISK mumbai, India
CHILDHOOD CANCER
Background and Aims: Diagnosis of cancer and treatment both are
Jessica Gereis1,2 , Kate Hetherington1,2 , Eden Robertson1 , David challenging for adolescent cancer patients. Adolescent patients cogni-
Ziegler1,3,4 , Claire Wakefield1,2 tion, knowledge and awareness differ from young children and adults,
1 UNSW Sydney, School Of Clinical Medicine, Faculty Of Medicine & Health, resulting in different coping strategies. There are few reports of psy-
Sydney, Australia; 2 Behavioural Sciences Unit, Kids Cancer Centre, Sydney, chosocial concerns and coping strategies in adolescent cancer patients
Australia; 3 UNSW Sydney, Children’s Cancer Institute, Sydney, Australia; in low resource settings. Our study aims to determine psychosocial
4 Sydney Children’s Hospital, Kids Cancer Centre, Sydney, Australia problems, coping strategies and psychiatric disorders in adolescent
cancer patients referred to a specialist Psycho-oncology service in a
Background and Aims: Precision medicine is projected to become metropolitan tertiary cancer hospital in a low middle income country.
the mainstay of childhood cancer care. As such, it is essential that Methods: We conducted a retrospective analysis of psycho-oncology
we develop resources to support patients’ and families’ understand- case records of adolescents with cancer referred to Psycho-Oncology
ing. We aimed to explore patients’ and families’ information needs, and services from Jan 2019 to Dec 2021. Patients on best support-
potential gaps for intervention. ive care alone and incomplete documentation were excluded. We
Methods: One-hundred-and-eighty-two parents and 23 adolescent noted sociodemography, cancer diagnosis and psychosocial prob-
patients participated in a psychosocial sub-study to PRISM, an Aus- lems according to clinical interview and mental status examina-
tralian precision medicine clinical trial for children with high-risk tion as documented in psycho-oncology assessment records. Cop-
cancer, completing questionnaires after study enrolment (Time 0, T0). ing strategies were documented according to Brief COPE. Psy-
Parents also completed a questionnaire and an interview following chiatric diagnosis was done using International Classification of
return of their child’s precision medicine results (Time 1, T1). We used Disease-10.
validated measures and purpose-designed items to explore families’ Results: A total of 194 adolescents with cancer were included in
perceptions of the PRISM participant information sheet and consent the analysis of whom 128 (66%) were male and. 106 (55%) were
form (PISCF), understanding of the information, and factors associated outpatients. Almost half the patients had solid tumours (bone and
with understanding. brain). The most common issues reported were distress, physical
Results: Most parents were satisfied with the PISCF information, say- 140 (72%) and emotional 111 (57%). 154 (79%) were aware of
ing it was at least somewhat clearly presented (n=160/175, 91%) their diagnosis. Adolescents used various coping strategies 168 (86%)
and informative (n=158/175, 90%). Many parents expressed a desire of the used active coping and 130 (67%) coped by seeking emo-
for additional information presented in a more accessible and visu- tional/instrumental support. A psychiatric diagnosis of adjustment
ally engaging format. On average, parents’ actual understanding scores disorder was present only in 41 (21%), situational distress in 21%, emo-
increased between T0 and T1 (55.8/100 to 60.0/100, p=0.012). At tional and behavioural disorder in (12%) and delirium in 11(6%) of the
T0, parents from culturally and linguistically diverse backgrounds patients.
(n=42/177, 25%) had lower actual understanding scores (p=0.010). Conclusions: There is a need for evidence-based, age appropri-
Parents’ perceived and actual understanding scores were not associ- ate interventions to address the psychological issues to enhance
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S400 of S635 ABSTRACTS

coping strategies and mental health of adolescent cancer patients. EP546 / #568 FATIGUE MEDIATES THE RELATIONSHIP
Limitations Small sample size, group with heterogeneous diagno- BETWEEN EMOTIONAL AND COGNITIVE FUNCTIONING IN
sis. Lack of standard measures due to diversity of language and CHILDREN POST-CANCER TREATMENT
culture.
Juliette Greidanus- Jongejan1 , Marloes Van Gorp1 , Raphaele Van
Litsenburg1 , Femke Aarsen1 , Merel Nap-Van Der Vlist2 , Sanne
EP545 / #1745 DETERMINANTS OF QUALITY Nijhof2 , Martha A. Grootenhuis1
PATIENT-CENTERED COMMUNICATION IN PAKISTAN 1 Princess Máxima Center for Pediatric Oncology, Department Of Pedi-
atric Oncology, Utrecht, Netherlands; 2 Wilhelmina Children’s Hospital,
Dylan Graetz1 , Tegan Reeves1 , Gia Ferrara1 , Courtney Staples1 , Alia University Medical Center Utrecht, Department Of Pediatrics, Utrecht,
Ahmad2 , Muhammad Rafie Raza3 , Syed Hamid3 , Asma Naheed4 , Netherlands
Atoofa Najmi3 , Afia Tul Quanita3 , Shabnam Munir3 , Sima Jeha1 ,
Carlos Rodriguez-Galindo1 , Jennifer Mack5 Background and Aims: Children treated for cancer are at risk to
1 St. Jude Children’s Research Hospital, Department Of Global Pediatric develop health-related quality of life problems, including cognitive
Medicine, Memphis, United States of America; 2 The Children’s Hospital and problems. To allow optimizing of interventions, underlying associations
Institute of Child Health, Oncology, Lahore, Pakistan; 3 The Indus Hospi- with other domains such as emotional functioning and fatigue need to
tal and health Network, Paeds Oncology, Karachi, Pakistan; 4 The Indus be clarified. We therefore aim to study emotional functioning, fatigue
Hospital, Psychosocial, Karachi, Pakistan; 5 Dana Farber Cancer Institute, and cognitive functioning in children post-cancer treatment and inves-
Pediatric Hematology/oncology, Boston, United States of America tigate whether fatigue mediates the relationship between emotional
and cognitive functioning.
Background and Aims: Communication is a fundamental aspect of Methods: Children aged 8-18 years who were post-cancer treatment
patient- and family-centered care. Unfortunately, there is a dearth filled out questionnaires to measure emotional functioning, fatigue
of evidence regarding pediatric cancer communication in low- and and cognitive functioning using subscales of the Pediatric Quality of
middle-income countries, where approximately 80% of all children Life Inventory (PedsQL) generic core scales and PedsQL multidimen-
with childhood cancer live. The purpose of this study was to explore sional fatigue scale. Student’s t-test was used to compare with peers
determinants of quality communication within two pediatric cancer of the general population and mediation analysis to address the effect
centers in Pakistan. of fatigue on the relationship between emotional and cognitive func-
Methods: Semi-structured interviews were conducted with 20 multi- tioning. Moderation of relapse of the primary tumor and tumor type
disciplinary pediatric cancer clinicians at Children’s Hospital of Lahore (central nervous system tumor/other) was evaluated. The mediation
and Indus Hospital in Pakistan. Interviews were conducted in English or model was adjusted for sex, age, relapse, tumor type and sleep if they
Urdu and audio recorded. All interviews were transcribed, with simul- were relevant confounders.
taneous translation to English as necessary. Two independent coders Results: A total of 137 children (62.4%, mean age: 13.6, SD +/- 3.3
used inductively derived codes to code each transcript. Thematic years) participated. Lower scores on emotional functioning (Cohen’s d
content analysis focused on barriers and facilitators of high-quality [D]: 0.4), fatigue (D: 0.8) and cognitive functioning (D: 0.6) were found
communication. (P < 0.001) in children post-cancer treatment. A medium association
Results: Pakistani clinicians identified factors that affected the quality was found between emotional and cognitive functioning (standardized
of patient-centered cancer communication. These included character- regression coefficient [β]: 0.27, P < 0.001), which was fully mediated
istics related to the clinician such as communication training, clinician by fatigue (β = 0.16), in a model adjusted for sleep. No significant
distress, and the availability to have recurrent conversations. Patient moderation effect on the model was found.
or family characteristics impacting communication included educa- Conclusions: Outcomes on emotional and cognitive functioning are
tion, literacy level, income status, geography, language, social support decreased and fatigue is increased in children post-cancer treatment.
systems, and empowerment. Finally, factors related to the institu- Fatigue mediates the relationship between emotional and cognitive
tion including setting, available interpreters, documentation, patient functioning. Our results underscore the need to include monitoring
volume, and teamwork, affected communication. Clinicians perceived and intervening on fatigue in clinical interventions to improve cognitive
certain determinants, such as patient volume or the income status of functioning.
a family, as immutable. Other determinants, including clinician com-
munication training or interpreter services, provided opportunities for
improvement. EP547 / #1840 STUDY ON THE EMOTIONAL RESPONSE TO
Conclusions: Multilevel factors serve as either barriers or facilitators MUSIC THERAPY IN ONCOLOGY AND INTENSIVE CARE
for pediatric cancer communication in Pakistan. Identification of these PEDIATRIC UNITS
determinants of communication is an essential step toward interven-
tions aimed at improving patient and family-centered care in resource Mercedes Guibelalde Del Castillo, Pau Catalá, Teresa Ferrer, David
limited settings. Diaz
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ABSTRACTS S401 of S635

Son Espases, Pediatric Oncology, Palma, Spain batim and analysed using Interpretative Phenomenological Analysis,
an approach that is understood via examination of meanings people
Background and Aims: The objective of music therapy in pediatrics impress upon their experience. Group experiential themes and sub
is to promote the humanization of hospital health care and help in themes are presented.
adaptation to illness on admission. This study evaluate the emotional Results: Grandparents, without question, resume their parental role as
response of pediatrics patient to music therapy, taking into account the their adult children retreat towards their childhood ‘nest’ to be pro-
variables of valence (joy), activation, relaxation and dominance (feeling tected and cared for. They also change their ‘hat’ to that of ‘parent’
of self-control). to siblings of their poorly grandchild. This becomes a dominant role,
Methods: Patients (77), up to 16 years of age, admitted to pediatric often without warning, impacting greatly on their normal routine. Their
oncology units and pediatric intensive care unit of Son Espases Hospi- own suffering is intentionally suppressed to give full attention to their
tal (Palma). Quasi-experimental, longitudinal, intrasubject design, with child and family. Grandparents struggle to articulate their own needs
one pre-session music therapy measure and two post-session mea- as they automatically place themselves second, however, when pushed,
sures. The self-assessment Manikin (SAM) scale of measurement of there is a sense of wishing to be acknowledged as taking an active, pri-
emotional variables is completed before, after the session and 3 hours mary care-giving role within their family, together with permission to
after. Trait anxiety is measured with the STAI or STAIC questionnaires process their own emotions in a way that suits their needs.
to check if its values influence the results of the session. The groups Conclusions: A grandchild’s childhood cancer diagnosis can lead to
are analyzed by age (0-8 years and 9-16 years) and type of unit. Ques- signs of traumatic stress for grandparents, yet they suppress their emo-
tionnaire on satisfaction and usefulness of the session were completed tional support needs as their ‘parental nest’ is temporarily filled again. It
by families. is suggested that cancer support services work with parents to ensure
Results: Music therapy has an influence with an increase in valence, that grandparents are also included in support-offers as a matter of
dominance and activation, between the moments before and after the course.
session (p≤0.001) in both age groups and, in the case of relaxation, p
= 0.041 for the group of younger patients and p = 0.011 for the older
ones. The feeling of relaxation is maintained 3 hours after the session EP549 / #1240 SCREENING FOR MENTAL HEALTH
with p = 0.382, in the case of children < 9y ( p = 0.343), in > 8y. It is PROBLEMS IN CHILDHOOD CANCER SURVIVORSHIP: A
considered a positive and useful intervention by users . SYSTEMATIC REVIEW
Conclusions: Music therapy has effects on the emotional variables
studied increasing self-control of pediatric patients and places them at Pauline Holmer, Gisela Michel, Daniela Dyntar, Céline Bolliger
the center of treatment. The relaxation variable remains the longest University of Lucerne, Department Of Health Sciences And Medicine,
in time after the session. Music therapy could be a complementary Luzern, Switzerland
therapy to provide resources to cope with disease in pediatric cancer
patients and in intensive care units Background and Aims: A childhood cancer diagnosis can be followed
by severe psychological sequalae. This is why annual psychological
evaluations and continuous monitoring is recommended for child-
EP548 / #1026 AN EXPLORATION OF THE EMOTIONAL hood cancer survivors. However, these recommendations are rarely
SUPPORT NEEDS OF GRANDPARENTS WHOSE GRANDCHILD implemented in clinical practice. Reasons are: lack of institutional
HAS HAD A CHILDHOOD CANCER DIAGNOSIS resources, health professionals’ uncertainty in dealing with mental
health problems or their unfamiliarity with available screening tools.
Lynda Hill We aimed to systematically review the evidence on screening for men-
University of Chester, Social And Political Science, Chester, United Kingdom tal health problems in childhood cancer survivors: a) which screening
instruments should be used, b) by whom screening should ideally be
Background and Aims: Wakefield et al. (2014) note that little research conducted, and c) what time frame is recommended.
has been conducted on the psychological impact on grandparents of Methods: The databases PubMed, PsycINFO, and CINAHL were
children with cancer despite evidence to suggest that this can be systematically searched for publications concerning childhood can-
challenging. This research explores the lived experiences of grandpar- cer, mental health problems, survivorship and screening. The search
ents whose grandchild has had a childhood cancer diagnosis, taking yielded 1729 potentially relevant articles, out of which 24 were
specific interest in narrative relating to symptoms of distress, coping included in the narrative data analysis.
mechanisms, perceived emotional support needs, potential barriers to Results: We found a wide variety of common (e.g., the Distress Ther-
support and signs of post-traumatic growth. The impact of COVID-19 mometer) and newly developed screening tools for psychological
is also explored. distress, depression, expressed suicidal ideation or behavior, attention
Methods: Twelve grandparents were interviewed using a qualitative deficit hyperactivity disorder, and post-traumatic stress disorder rec-
methodology that focuses on hermeneutic phenomenology. Semi- ommended for screening. Depending on the age of the survivor, type
structured questions were asked. Interviews were transcribed ver- of cancer, and mental health area, it can be advantageous to involve
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S402 of S635 ABSTRACTS

caregivers, teachers, medical professionals, but also research staff Results: Overall, 142 children (mean age, 9.2 years; range, 7.8 to
in the screening process in addition to self-report. Most tools are 10.6 years) were included. During pregnancy, 100 children (70.4%)
designed for regular use, with many aiming to detect mental health were exposed to chemotherapy (only or in combination with other
problems as early as possible. treatments), 18 (12.7%) to surgery only, 17 (12.0%) to radiother-
Conclusions: Contrary to the described obstacles, most tools can be apy (only or in combination), 16 (11.3%) to no treatment and 1
easily administered and evaluated without training and take only a to trastuzumab (0.7%). No significant differences in intelligence,
few minutes to complete. The overview of screening tools for various attention, memory, and behavioural outcomes were found. Gesta-
mental health problems hopefully helps facilitating the transition from tional age at birth predicted Total IQ (F(1,135)=12.075, p<0.001,
recommendations to practice. R2 =0.083), Verbal IQ (F(1,135)=9.665, p=0.002, R2 =0.067), Per-
formance IQ (F(1,125)=6.814, p=0.010, R2 =0.052), Verbal Memory
Span (F(1,123)=11.888, p<0.001, R2 =0.089), Verbal Working Mem-
EP550 / #1293 (NEURO)COGNITIVE CHILD DEVELOPMENT ory (F(1,64)=6.654, p=0.012, R2 =0.096), and Visual Memory Span
AT 9 YEARS AFTER PRENATAL EXPOSURE TO MATERNAL (F(1,122)=5.217, p=0.024, R2 =0.041). Children with a deceased
CANCER AND ITS TREATMENT: REPORT BY THE mother obtained lower scores on Total IQ (t(134)=1.892, p=0.030),
INTERNATIONAL NETWORK ON CANCER, INFERTILITY AND Processing Speed (t(124)=1.712, p=0.045) and Visual Memory Span
PREGNANCY (t(121)=2.861, p=0.002).
Conclusions: Children born after a pregnancy complicated by maternal
Evangeline Huis In ’T Veld1,2 , Indra Van Assche3 , Kristel Van cancer seem to have a normal neurocognitive development at the age
Calsteren3 , Mathilde Van Gerwen1,2,4 , Harm Van Tinteren1 , Elisabeth of 9 years. These are reassuring results. However, caution is indicated
Van Dijk-Lokkart4 , Jurgen Lemiere5,6 , Elyce Cardonick7 , Monica and surveillance of the neurocognitive development is needed in chil-
Fumagalli8,9 , Martina Delle Marchette10 , Martine Van Grotel1 , Marry dren born preterm and in children whose mothers are deceased due to
Van Den Heuvel-Eibrink1 , Lieven Lagae11 , Frederic Amant2,6 her disease.
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology,
Utrecht, Netherlands; 2 Netherlands Cancer Institute, Department For
Gynaecological Oncology, Amsterdam, Netherlands; 3 KU Leuven, University EP551 / #1544 HEALTH-RELATED QUALITY OF LIFE OF
Hospitals Leuven, Department Of Development And Regeneration, Leuven, INDONESIAN CHILDREN WITH SOLID TUMORS
Belgium; 4 Amsterdam UMC, University of Amsterdam, Department Of Child
& Adolescent Psychiatry And Psychosocial Care, Amsterdam, Netherlands; Braghmandita Indraswari1 , Mei Sitaresmi1 , Gertjan Kaspers2,3 , Saskia
5 University Hospitals Leuven, Pediatric Hemato-oncology, Leuven, Belgium; Mostert2,3
6 KU Leuven, University Hospitals Leuven, Department Of Oncology, Leu- 1 Universitas Gadjah Mada, Department Of Pediatrics, Yogyakarta, Indone-
ven, Belgium; 7 Cooper University Health Care, Department Of Obstetrics sia; 2 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology,
And Gynaecology, Camden, United States of America; 8 University of Milan, Utrecht, Netherlands; 3 Emma Children’s Hospital, Amsterdam UMC, Vrije
Department Of Clinical Sciences And Community Health, Milano MI, Italy; Universiteit, Pediatric Oncology, Amsterdam, Netherlands
9 Granda Ospedale Maggiore Policlinico, NICU„ Fondazione Irccs Ca’, Milano

MI, Italy; 10 San Gerardo Hospital, Department Of Obstetrics And Gynaecol- Background and Aims: Advances in childhood cancer treatment have
ogy, Monza MB, Italy; 11 KU Leuven, University Hospitals Leuven, Pediatric improved survival rates in low and middle-income countries (LMIC),
Neurology, Leuven, Belgium such as Indonesia, in recent years. However, not only curing chil-
dren with cancer, but also ensuring a good health-related quality of
Background and Aims: Limited evidence exists on the long-term life (HRQOL) during treatment is of utmost importance. Particularly
effects of prenatal exposure to maternal cancer and its treatment on because childhood cancer treatment can be aggressive and adversely
the (neurocognitive) development of the offspring. The aim of the cur- affect children’s well-being due to side-effects. Few studies focused
rent study was to determine the impact of this antenatal exposure on on HRQOL in LMIC, where supportive care is limited. About 30%
children at the age of nine years. of all childhood cancers consist of solid tumors. This study aims to
Methods: In an international, multicentre (n=5) cohort study (Bel- investigate HRQOL among children with solid tumors in Indonesia.
gium, the Netherlands, Italy, USA) children were prospectively Methods: A cross-sectional study was conducted among children with
assessed using a comprehensive neuropsychological battery, solid tumors in Dr. Sardjito General Hospital, Indonesia. The HRQOL
examining intelligence (IQ), attention and memory. Behaviour was data were obtained from patients and their guardians using Pediatric
assessed using parental behavioural questionnaires. Results were Quality of Life Inventory™ (PedsQL™) 4.0 Generic Core Scale and
compared with test-specific normative data by country and age Pediatric Quality of Life Inventory™ (PedsQL™) 3.0 Cancer Module
using ANCOVA. Sub-analyses with gestational age at birth were (Indonesian version).
performed using linear regression. Children with and without Results: Sixteen children aged 5-18 years and 41 guardians of children
a deceased mother were compared using independent-samples aged 2-18 years participated in this study. Twenty-six (63%) children
t-tests. were boys. Of all children, 9 children (22%) were diagnosed with
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ABSTRACTS S403 of S635

nephroblastoma, 8 children (20%) with neuroblastoma, 6 (15%) with and cognitive fatigue (p<0.001, OR=10.54). Age at diagnosis, serious
retinoblastoma, and 18 (44%) with other solid tumors. In PedsQL™ adverse events, sex, or risk group were not associated with fatigue at
4.0 Generic Core Scale, children reported worse total HRQOL-scores follow-up for any of the subscales.
than parents (66 vs. 72). By contrast, in PedsQL™ 3.0 Cancer Mod- Conclusions: The findings demonstrate that fatigue reported during
ule, parents and children reported equal total HRQOL-scores (80 vs. treatment can predict fatigue at follow-up. Healthcare professionals
80). Procedural anxiety had the worst score of all subscales for both need to be aware that pediatric patients who are fatigued during treat-
groups (58 vs. 70). Although not statistically significant, parents of boys ment need to receive additional attention and timely interventions
reported worse total HRQOL-scores than girls. since cancer-related fatigue will not resolve by itself in the first year
Conclusions: Indonesian children with solid tumors had most problems after end of treatment. Furthermore, these results stress the need for
in procedural anxiety. To improve HRQOL during cancer treatment, multiple assessments during treatment and longitudinal studies focus-
special attention and care is required during intervention procedures ing on following the participants for a longer time-period after end of
to reduce stress and improve coping of these children. treatment.

EP552 / #76 FATIGUE TRAJECTORIES DURING PEDIATRIC EP553 / #232 COGNITIVE AND ADAPTIVE FUNCTION IN
ACUTE LYMPHOBLASTIC LEUKEMIA THERAPY IS ASSOCIATED INFANTS AND TODDLERS BEING TREATED FOR ACUTE
WITH FATIGUE AFTER TREATMENT: A DUTCH NATIONAL LEUKEMIA
LONGITUDINAL COHORT STUDY
Alexander Wallace, Victoria Willard, Jennifer Harman, Lisa Jacola
Elin Irestorm1,2 , Lindsay Steur1,3 , Gertjan Kaspers1,3,4 , Natasha St. Jude Children’s Research Hospital, Psychology - Ms 740, Memphis,
Eijkelenburg1 , Inge Van Der Sluis1,5 , Natasja Dors1,6 , Cor Van Den United States of America
Bos1,3 , Wim Tissing1,7 , Martha A. Grootenhuis1 , Raphaele Van
Litsenburg1,3 Background and Aims: Survivors of childhood acute leukemia are at
1 Prinses Maxima Center for Pediatric Oncology, Psycho-oncology, Utrecht, risk for neurocognitive difficulties that emerge during therapy. Younger
Netherlands; 2 Lund University, Faculty of Medicine, Paediatrics, Lund, age at diagnosis is a risk factor for poorer outcomes. Few studies have
Sweden; 3 Emma Children’s Hospital, Department Of Pediatric Oncology, examined early cognitive and adaptive function in infants and toddlers
Amsterdam, Netherlands; 4 Vrije Universiteit Amsterdam, Pediatric Oncol- during cancer-directed therapy.
ogy, Amsterdam, Netherlands; 5 Sophia Children’s Hospital, Department Of Methods: In this retrospective study, data on cognitive and adaptive
Pediatric Oncology, Rotterdam, Netherlands; 6 Amalia Children’s Hospital, function were abstracted from the medical record for 41 infants and
Department Of Pediatric Oncology, Nijmegen, Netherlands; 7 University of toddlers who completed neurodevelopmental evaluations in our Early
Groningen, Department Of Pediatric Oncology, Groningen, Netherlands Childhood Clinic (53.6% male; mean±SD age at diagnosis = 20.3±9.5
months; age at assessment = 23.6±9.4 months). Patients were evalu-
Background and Aims: Fatigue is one of the most prevalent and dis- ated during treatment for acute lymphoblastic leukemia (ALL; n=30;
tressing symptoms reported by survivors of childhood cancer. There 73.2%) or acute myeloid leukemia (AML; n=11; 26.8%). Results are
is currently a lack of longitudinal studies on cancer-related fatigue, age-standardized Z scores (normative mean = 0, SD = 1).
and especially on the relationship between the course of fatigue dur- Results: Compared to age expectations, patients had significantly
ing treatment and fatigue at follow-up. The aim of the current study lower scores in global cognitive (Z=-1.07, p<0.001) and adaptive
was therefore to investigate if the course of fatigue during treatment, function (Z =-0.70, p<0.001). Patients were significantly below age
treatment intensity, serious adverse events, sex, or age at diagnosis are expectations in adaptive subdomains of social (Z=-0.49, p<0.001),
associated with cancer-related fatigue after treatment. practical (Z=-0.40, p=0.008), motor (Z=-0.76, p<0.001), and commu-
Methods: Participants were 92 children and adolescents diagnosed nication (Z=-0.44, p=0.006), and in cognitive subdomains of fine motor
with acute lymphoblastic leukemia (mean age at diagnosis was 6.26 (Z=-0.53, p=0.036), gross motor (Z=-1.41, p<0.001), and receptive
years). Fatigue was measured with PedsQL Multidimensional Fatigue and expressive communication (Z=-0.84, p=0.006; Z=-0.75, p=0.014).
Scale proxy-reports 5 months after diagnosis, 12 months after diag- There were no significant group differences by diagnosis (ALL v.
nosis, 24 months after diagnosis, and at follow-up 12 months after AML). Among children treated for ALL (53.3% male; age at assess-
end of treatment. The course of fatigue was defined as the fatigue ment = 24.7±9.3 months; time since diagnosis = 2.5±2.3 months),
reported during treatment. The effect of patient and treatment char- females demonstrated significantly worse adaptive motor function
acteristics on the fatigue reported at follow-up was tested through than males (Z =-1.18±0.75 v. -0.52±0.73; p=0.041). Among the ALL
logistic regression analyses. group, females had lower global cognitive scores than males, with
Results: At follow-up, 26% reported general fatigue, 16% sleep-rest moderate effect size (Hedge’s g=0.93; p=0.119).
fatigue, and 22% cognitive fatigue. The course of fatigue during treat- Conclusions: Infants and toddlers treated for acute leukemia show
ment significantly predicted fatigue reported at follow-up for general developmental delay during cancer directed therapy, which high-
fatigue (p=0.036, OR=9.26), sleep/rest fatigue (p= 0.011, OR=15.03), lights the need for early intervention and supportive care to promote
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S404 of S635 ABSTRACTS

developmental gains. Consistent with studies in long-term survivors shown to correlate with improved health outcomes. AF ratings suggest
of ALL, findings suggest that females may be at greater risk for the BMT Bucks Program is acceptable and feasible in our setting.
treatment-related cognitive difficulties.

EP555 / #1377 EDUCATION NEEDS OF CAREGIVERS OF


EP554 / #1868 ADAPTATION AND ACCEPTABILITY OF CHILDREN WITH CANCER IN TANZANIA
TOKEN ECONOMY SHOWN TO IMPROVE ADHERENCE TO
ACTIVITIES OF DAILY LIVING IN PEDIATRIC HEMATOPOIETIC Emma Joo1,2 , Yadurshini Raveendran1 , Erica Sanga3 , Kristin
STEM CELL TRANSPLANT Schroeder1,2
1 Duke University, Duke Global Health Institute, Durham, United States
Torri Jones1 , Rebecca Madden2 , Louisa Rygh2 , Nicolette Hocter3 , of America; 2 Bugando Medical Centre, Oncology, Mwanza, Tanzania;
Madeleine Yeager3 , Stephanie Fooks-Parker4 , Alissa Feirson4 , Laura 3 National Institute for Medical Research, Public Health, Mwanza, Tanzania

Smith5 , Kaci Davis5 , Jason Freedman6


1 University of Pennsylvania/Children’s Hospital of Philadelphia, Depart- Background and Aims: Caregivers play the main role in the support of
ment Of Psychiatry/division Of Oncology, Philadelphia, United States children with cancer, influencing presentation and treatment comple-
of America; 2 Children’s Hospital of Philadelphia, Behavioral Oncology, tion. Therefore, caregiver knowledge about childhood cancer must be
Philadelphia, United States of America; 3 Children’s Hospital of Philadelphia, understood to address the challenges and facilitators for the initiation
Child Life, Philadelphia, United States of America; 4 Children’s Hospi- and completion of pediatric cancer care in Tanzania.
tal of Philadelphia, Social Work, Philadelphia, United States of America; Methods: A descriptive qualitative study was conducted at Bugando
5 Children’s Hospital of Philadelphia, Nursing/oncology, Philadelphia, United Medical Center in Northwest Tanzania. Caregivers of children receiv-
States of America; 6 University of Pennsylvania/CHOP, Oncology, Philadel- ing cancer care completed a focus group discussion to understand their
phia, United States of America knowledge about childhood cancer, and education needs at diagnosis
and during treatment. Data was transcribed, coded, and analyzed by
Background and Aims: Pediatric patients face health risks while thematic content-analysis with the support of NVIVO software.
undergoing hematopoietic stem cell transplant (HSCT) including oral Results: A total of 9 caregivers were in the focus group, 7 female and 2
complications (Elad et al., 2015), deconditioning (Lago et al., 2021), and male participants. The analysis was conducted by the first two authors
bloodstream infections (Dandoy et al., 2016). These risks were signif- with an inter-rater agreement score of 94.8%. Caregiver knowledge on
icantly reduced by engagement in activities of daily living (ADL): oral pediatric cancer was low with many believing prior to their child’s diag-
care, physical activity, and bathing via a token economy during inpa- nosis that children could not get cancer, cancer is caused by witchcraft
tient admission (Best et al., 2016; Hickey et al., 2018). We adapted and that it was an incurable disease. Pediatric cancer education at
and implemented this program with comparable findings, and utilized diagnosis by patient navigators lessened their worries and gave them
acceptability and feasibility (AF) data to guide quality improvement hope that their children could be cured. When asked what key edu-
(QI). cation E-Poster Topics should be provide for caregivers, suggestions
Methods: Our BMT Bucks Program, adapted from ADL 1-2-3 (Hickey included the cause of cancer, duration of their child’s cancer and treat-
et al., 2018) targeted 3 daily ADL and added a 4th , daily weights. Per- ment, and the side effects of treatment, and future impact on fertility.
cent of completed ADL over 21 days was compared across 8 pilot To improve baseline community knowledge, caregivers recommended
patients and matched comparisons using an independent samples t- going into rural communities and using media-based education pro-
test. Caregivers and patients rated 7 AF items via 5-point Likert scale grams including the radio for greater reach, regardless of literacy
from 1 (strongly disagree) to 5 (strongly agree). This survey was also rates.
completed by 26 bedside nurses, and later by 19 additional pediatric Conclusions: Low caregiver knowledge about pediatric cancer led
patients undergoing HSCT after implementing initial QI feedback. to delayed presentation. Cancer education programs had a positive
Results: Pilot patients completed approximately 20% more ADL than impact in caregiver understanding and hopefulness of their child’s
comparison group t(14) = 2.77, p = 0.021*. Initial AF mean rating by diagnosis. Educational interventions should be prioritized in rural com-
bedside nurses was 3.8, whereas patient/caregiver combined AF mean munities, to increase cancer knowledge and improve pediatric cancer
was 4.5. Specifically, QI ratings suggested we increase patient and care- outcomes.
giver engagement in tracking ADL and increase nursing awareness of
results supporting program efficacy. After QI-informed adjustments,
mean acceptability and feasibility rating was 4.8 for caregivers and EP556 / #63 PSYCHOSOCIAL FUNCTIONING OF PARENTS
4.7 for patients. The highest rating for both caregivers (mean = 5.0) OF DUTCH LONG-TERM SURVIVORS OF CHILDHOOD CANCER
and patients (mean = 4.9) was for the item indicating they would
recommend BMT Bucks to others. Mala Joosten1 , Marloes Van Gorp1 , Anne Maas1 , Babet Drenth1 , Alied
Conclusions: Patients participating in the token economy demon- Van Der Aa1 , Leontien Kremer1,2 , Eline Van Dulmen-Den Broeder3 ,
strated significantly increased adherence to ADL, which has been Wim Tissing1,4 , Jacqueline Loonen5 , Helena Van Der Pal1 , Andrica De
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S405 of S635

Vries1,6 , Marry Van Den Heuvel-Eibrink1,6 , Cecile Ronckers1,7 , Dorine to the general population. Only a small proportion experiences symp-
Bresters1,8 , Marloes Louwerens9 , Sebastian Neggers10 , Margriet Van tomatic post-traumatic stress, and post-traumatic stress symptoms are
Der Heiden-Van Der Loo1 , Heleen Maurice-Stam1 , Martha A. associated to other psychosocial outcomes. Illness cognitions may pro-
Grootenhuis1 vide treatment targets in interventions for parents with psychosocial
1 Princess Máxima Center for Pediatric Oncology, Department Of Pediatric problems.
Oncology, Utrecht, Netherlands; 2 Emma Children’s Hospital, Amsterdam
University Medical Center, Vrije Universiteit Amsterdam, Pediatric Oncol-
ogy, Amsterdam, Netherlands; 3 Emma Children’s Hospital/Amsterdam Uni- EP557 / #99 DIAGNOSIS RELATED INFORMATION
versity Medical Center, Vrije Universiteit Amsterdam, Department Of PREFERENCES OF ADOLESCENTS WITH CANCER IN ARMENIA
Paediatric Oncology, Amsterdam, Netherlands; 4 Princess Maxima Center
for Pediatric Oncology, Supportive Care, Utrecht, Netherlands; 5 Radboud Alisa Kamalyan1 , Saten Hovhannisyan1 , Lilit Sargsyan1 , Haykush
University Medical Center, Department Of Haematology, Nijmegen, Nether- Mkhitaryan1 , Arevik Mkrtchyan1 , Ani Hovhannisyan1 , Lusine
lands; 6 Sophia Children’s Hospital/Erasmus Medical Center, Department Hakobyan1 , Ruzanna Papyan1 , Sergo Mkhitaryan2 , Gevorg
Of Paediatric Oncology, Rotterdam, Netherlands; 7 Carl von Ossietzky Uni- Tamamyan1
versity of Oldenburg, Faculty of Medicin and Health Sciences, Health 1 Hematology Center after R.Yeolyan, Pediatric Cancer And Blood Disorders

Services Research, Oldenburg, Germany; 8 Willem Alexander Children’s Center Of Armenia, Yerevan, Armenia; 2 National Center of Oncology after
Hospital/Leiden University Medical Center, Department Of Pediatrics, V.Fanarjian, Department Of Radiation Oncology, Yerevan, Armenia
Leiden, Netherlands; 9 Leiden University Medical Center, Department
Of Internal Medicine, Leiden, Netherlands; 10 Erasmus Medical Center, Background and Aims: According to the legislation of the Republic of
Department Of Internal Medicine, Section Endocrinology, Rotterdam, Armenia, health-related information of adolescents under 18 years old,
Netherlands should be provided to their parents or legal representatives. Parents’
opinion on informing an adolescent about a cancer diagnosis is decisive.
Background and Aims: Childhood cancer has a major impact There is less evidence about health-related information preferences
on psychosocial functioning of parents. The aim of the cur- of adolescents with cancer. This study aims to explore parents’ and
rent study is to describe psychosocial functioning of parents of adolescents’ preferences on disclosure of a cancer diagnosis related
long-term survivors of childhood cancer and to study associated information among the population of Armenia
factors. Methods: This qualitative study was conducted through semi-
Methods: Parents of survivors of the Dutch Childhood Cancer Sur- structured in-depth interviews with 15 adolescents with cancer
vivor Study LATER cohort (diagnosed 1963-2001) were invited to aged 12-18, had been receiving chemotherapy at least one
complete the TNO-AZL Questionnaire for Adult’s HRQoL, General month before the interview and 15 mothers aged 37-53. Two
Health Questionnaire (psychological distress), Self-Rating Scale for study instruments were developed based on the literature
Post-traumatic Stress Disorder, Post-traumatic Growth Inventory, and review, respectively for adolescents and parents. A qualita-
Illness Cognition Questionnaire (acceptance, helplessness and disease tive content analysis with inductive approach was used for data
benefits). HRQoL domain scores (e.g. vitality, aggressive emotions) analysis.
were compared to references using Mann-Whitney U tests with Results: The results of the research indicated that the 15 interviewed
effect size r. Associations of post-traumatic stress and growth with adolescents preferred to be properly informed about their diagno-
other outcomes were studied with Pearson’s r. Child disease vari- sis and treatment specifics. According to the adolescents, coping with
ables and parent illness cognitions were studied as associated factors the disease, handling difficulties, fighting cancer becomes easier when
of HRQOL, distress, post-traumatic stress and post-traumatic growth they are informed appropriately. In comparison with adolescents, 13
using multivariable linear regression analyses. Level of significance was out of 15 interviewed parents stated that awareness of the diagnosis
p<.05. would negatively affect treatment process and mood of adolescents.
Results: Parents (n=661, 56% female, time since child’s diagnosis: 21.3 Moreover, in their opinion, adolescents would not be able to handle
[SD: 3.3] years) reported less aggressive emotions than references the information. However,6 parents changed their non-disclosure atti-
(r=.10–.14). Mothers reported better HRQoL in social functioning, tudes at the end of the treatment. The other important study finding
daily activities and vitality than references (r=.10–.14). Other HRQoL was related to the source of the diagnosis information; adolescents
domains were not different from references. Post-traumatic stress was mentioned that they would prefer to receive information from doc-
symptomatic in 3% and was associated to worse HRQoL and distress tors, since they would provide detailed information in an empathetic,
(r=-.27–.48). Post-traumatic growth was associated to post-traumatic but non-emotional manner, while parents stated that it should be them
stress and better HRQoL (r=.09-.12). Recurrence was associated providing diagnosis information.
to better HRQoL (β=.30–.47). Acceptance was associated to better Conclusions: Conducted study results indicate that adolescents with
(β=.12–.24), and helplessness to worse outcomes (β=.11–.38). cancer need to be involved in diagnosis related communication,
Conclusions: On the long run parents of survivors of childhood can- which can be led by the doctor and conducted in the presence of
cer appear to have limited psychosocial difficulties and HRQoL similar parents.
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S406 of S635 ABSTRACTS

EP558 / #1133 UTILIZATION OF THE AYA Centre Schleswig Holstein- Campus Lübeck, Department Of Pediatric
PSYCHO-ONCOLOGY SCREENING TOOL IN A PEDIATRIC Oncology And Hematology, Lübeck, Germany; 5 Universitätsklinikum des
HOSPITAL AYA PROGRAM Saarlandes, Pädiatrische Onkologie/hämatologie, Homburg, Germany; 6 Dr.
von Hauner University Children’s Hospital- Ludwig-Maximilian-University
Karen Albritton, Daniel Elledge, Antonia Leavitt Munich, Department For Pediatric Hematology- Oncology- Hemostaseology
Cook Children’s Medical Center, Hematology/oncology, Fort Worth, United And Stem Cell Transplantation, München, Germany; 7 University of Vienna,
States of America Institut Für Psychologie Der Entwicklung Und Bildung, Vienna, Austria

Background and Aims: The AYA Psycho-Oncology and Survivorship Background and Aims: The consensus- and evidence-based guideline
Screening Tools were developed to assess distress and concerns of AYA “Psychosocial care in pediatric oncology and haematology” (Schröder
cancer patients during and after treatment. We report on the use of the et al., 2019) defines standards for basic principles of psychosocial care,
distress thermometer and areas of concern reported by AYAs age 15+ framework and structures, psychosocial assessment, and interven-
at our pediatric hospital. tions. However, the issue of translation into clinical practice remains
Methods: AYAs are typically given assessment within 1 month of diag- critical.
nosis, and every 2, 4, or 6 months depending on their previous distress Methods: As part of the second update of the guideline, supporting
score (DT). For off-treatment patients, assessments are typically given and hindering factors regarding implementation were explored in a
every 6 months. Findings are shared with AYA team, medical team, and transnational online survey. Thirty-five questions related to knowledge
other treatment services as needed and entered into EMR. about the guideline, comprehensibility, feasibility, relevance to over-
Results: Between 1/21 and 3/22, we completed 108 DTs in 62 AYAs all treatment concept, benefits, and barriers to implementation were
on-treatment and 37 in 24 AYAs off-therapy. Thirty-two individuals developed and agreed upon by the guideline development steering
completed one screen, 33 have completed two and 10 have completed group. The online questionnaire was distributed to psychosocial staff
3 or 4.The average and median age was 18 (range 15-30). The aver- in the D-A-CH region in a two-month period. A total of 76 respondents
age distress score was 3.3 for on-therapy and 3.5 off-therapy AYAs. completed the survey (43% Clinical Psychology, 20% Social Work, 16%
Thirty-two percent on treatment and 27% off-treatment DT scores Psychotherapy, 9% School, 4% Art therapy, 8% others). Responses were
were 5+ . Among patients on therapy, the most commonly endorsed given on a visual analogue scale, with 0 being the lowest and 100 being
concerns were "Missing doing ’normal stuff’ with friends" (51%), "Bore- the highest.
dom" (44%), "Isolated from friends" (39%) and "Education" (38%). The Results: Knowledge (M=81.15, SD=20.75) and comprehensibility
off-therapy assessment has 76 possible areas of concern; AYAs marked (M=83.86, SD=19.43) of the guideline were rated high, feasibility
an average of 10 items. The most commonly endorsed concerns were significantly lower (M=62.01, SD=29.56). Experienced benefit of the
"Trouble remembering things", "Worry about cancer coming back", guideline was mainly explained by the own evaluation of knowledge,
"Short attention span or concentration", and "Feeling like missed out feasibility, and comprehensibility (regression analysis: F (3; 65) =
on life because of cancer" (each 4%). 20.91; r2 = 0.49; p = 0.00). Within the psychosocial team, the guide-
Conclusions: Most AYAs age 15-30 in a pediatric hospital report low line was regarded integrated into the overall treatment concept by 44%
levels of distress. However, the DT identified 30% with high levels of of the respondents, considered or accepted to be a standard by 42%,
distress and areas of concern allowing for targeted support. Anec- for 14% it was not considered a standard. The experienced importance
dotally there was a sense among clinicians that the tool uncovered of the guideline in the own institution was indicated on average with
concerns that the team was not aware of; we intend to study the added 60.55, with a broad distribution (SD=33.71).
value of the tool to clinicians in future research. Conclusions: A clear discrepancy between defined standards and prac-
tical use of the guideline became apparent. From the factors analyzed,
an action plan was developed with the goal of higher awareness,
EP559 / #1877 IMPLEMENTATION OF PSYCHOSOCIAL changes in structures and frameworks, and targeted interdisciplinary
STANDARDS OF CARE. WHAT HAVE WE LEARNED ABOUT training.
BARRIERS AND SUPPORTING FACTORS?
EP560 / #418 IDENTITY FUNCTIONING AND PERSONALITY
Ulrike Leiss1 , Barbara Grießmeier2 , Bea Schreiber-Gollwitzer3 , Birte IN ADOLESCENT AND EMERGING ADULT SIBLINGS OF
Hesselbarth4 , Iris Lein-Köhler5 , Alexandra Nest6 , Liesa J. SURVIVORS OF PEDIATRIC CANCER
Weiler-Wichtl1 , Doris Hoffmann-Lamplmair1 , Anna Müller1 , Lisa
Laussner1 , Jonathan Fries7 , Hildegard Schröder4 Jurgen Lemiere1,2 , Sofie Prikken1 , Koen Raeymaekers3 , Elise Van
1 Medical University of Vienna, Comprehensive Center For Pedi- Laere3 , Charlotte Sleurs2 , Anne Uyttebroeck1,2 , Koen Luyckx3
atrics/department For Pediatrics And Adolescent Medicine, Vienna, Austria; 1 UZ Leuven, Pediatric Hemato-oncology, Leuven, Belgium; 2 KULeuven,
2 University Hospital Frankfurt am Main, Department Of Pediatric And Department Of Oncology, Leuven, Belgium; 3 KU Leuven, Faculty Of Psychol-
Adolescent Medicine, Frankfurt, Germany; 3 Dritter Orden Clinic, Pediatric ogy And Educational Sciences, Leuven, Belgium
And Youth Medicine Clinic- Spz, München, Germany; 4 University Medical
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ABSTRACTS S407 of S635

Background and Aims: The psychological long-term impact of child- the reliability, validity, usability, and clinical added value of the
hood cancer on siblings is poorly understood, especially concerning PAT.
important self-related constructs such as identity and personality. The Methods: In two pediatric oncological centers in Belgium
objectives of this study were: 1) to compare siblings with control par- (UZGent/UZLeuven) families confronted with a new diagnosis of
ticipants on identity functioning, personality traits, and general well- childhood cancer were asked to complete the PAT and additional
being (depression, self-esteem, satisfaction with life) and to investigate questionnaires (e.g., usability of PAT). The multidisciplinary team rated
associations of identity functioning and personality with demographic, these families in one of the three risk categories (universal, targeted
and clinical characteristics and general well-being in siblings. and clinical). A Spearman rank correlation was used to investigate
Methods: Siblings (n=80; mean age: 19.13; 49% male) were matched agreement between PAT and team scores.
on age and gender with healthy controls (2:1). Identity synthesis and Results: A total of 106 families participated in the study. The comple-
confusion were measured with the identity subscale from the Erikson tion of the PAT was on average 37 days after diagnosis. The reliability
Psychosocial Stage Inventory. The Dimensions of Identity Develop- of the total PAT score (α=.86) and most of the subscales was adequate
ment Scale was used to measure five identity dimensions. Personality to good (α=.64-.88). One subscale was inadequate (Family Beliefs,
traits were measured using the Quick Big Five questionnaire. (α=.45)). Content validity was adequate (r=.30-.62), except for the
Results: Siblings had significantly lower scores than community youth ’social support’ subscale. The usability of the PAT, as rated by the par-
on agreeableness, openness to experience, and conscientiousness, but ents was adequate to good. Based on the total PAT score 68%, 26% and
they did not differ on any of the identity constructs. Siblings reported 6% of the families were classified in the universal, targeted and clini-
lower self-esteem and satisfaction with life. For siblings, age of sibling cal group, respectively. A modest correlation between the PAT and the
at diagnosis was positively associated with the identity dimension of team’s assessment was found (r= .38, p<.001). According to the assess-
exploration in breadth. Sisters reported more identity confusion, less ment of the team, 36%, 48% and 17% were classified in the universal,
identity synthesis, more neuroticism, and openness to experience than targeted and clinical group, respectively.
brothers. Identity synthesis, the identity dimension of identification Conclusions: Our study demonstrates the reliability, validity, and appli-
with commitment, and the personality trait of agreeableness related to cability of the Flemish adaptation of the PAT. More research is required
better well-being in siblings. Exploration in depth was positively related to evaluate the added clinical value of combining different assessments
to satisfaction with life. Extraversion was positively related to satis- (i.c. PAT and multidisciplinary evaluation) of psychosocial risk factors in
faction with life and self-esteem. In contrast, identity confusion, the order to optimize psychosocial support of families confronted with a
identity dimension of ruminative exploration, and neuroticism were child with cancer.
associated with worse well-being.
Conclusions: The finding that siblings are at risk of having low self-
esteem and satisfaction with life is in line with existing literature. Our EP562 / #576 LACK OF POST COMPULSORY EDUCATION
findings suggest that identity functioning and personality can be impor- PREDICTS UNEMPLOYMENT IN TEENAGERS AND YOUNG
tant constructs to consider in relation to general well-being in siblings ADULTS TREATED FOR BRAIN TUMOURS IN CHILDHOOD
of survivors of pediatric cancer.
Malin Lönnerblad1,2,3 , Eva Berglund3 , Maria Åberg4,5 , Klas
Blomgren2,6
EP561 / #1247 RELIABILITY, VALIDITY AND APPLICABILITY 1 Uppsala University, Department Of Women’s And Children’s Health,
OF THE FLEMISH ADAPTATION OF THE PAT: A MULTICENTER Uppsala, Sweden; 2 Karolinska Institutet, Department Of Women’s And Chil-
STUDY dren’s Health, Stockholm, Sweden; 3 Stockholm University, Department Of
Special Education, Stockholm, Sweden; 4 University of Gothenburg, School
Jurgen Lemiere1,2 , Isabeau Van Rompaey1 , Emma Van Heerentals3 , Of Public Health And Community Medicine, Institute Of Medicine, Sahlgren-
Laura Claeys1 , Karen Vandenabeele1 , Linde Van Den Wyngaert1 , Trui ska Academy, Gothenburg, Sweden; 5 Region Västra Götaland, Regionhälsan,
Vercruysse1 , Charlotte Sleurs2 , Nathalie Nolf3 , Marieke Van Schoors3 , Gothenburg, Sweden; 6 Paediatric oncology, Karolinska University Hospital,
Leen Willems3 , Marleen Renard1 , Heidi Segers1,2 Stockholm, Sweden
1 UZ Leuven, Pediatric Hemato-oncology, Leuven, Belgium; 2 KULeuven,

Department Of Oncology, Leuven, Belgium; 3 Ghent University Hospital, Background and Aims: It is well recognized that there is a high risk of
Department Of Paediatric Haematology-oncology And Stem Cell Transplan- late complications after a brain tumour in childhood, which may affect
tation, Ghent, Belgium academic performance. The aim of this study is to explore participa-
tion in post compulsory education and its impact on employment for
Background and Aims: The Psychosocial Assessment Tool (PAT) Swedish teenagers and young adults treated for brain tumours in child-
is a tool to screen for psychosocial risk factors in families con- hood. Differences due to sex, age at diagnosis, and tumour type will also
fronted with a child with cancer. We translated the PAT for the be explored.
Flemish speaking part of Belgium using the forward-backward Methods: Nationwide registry data about 452 individuals, born 1988–
procedure. The aim of the present study was to investigate 1996 and diagnosed with a brain tumour before their 15th birthday, are
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S408 of S635 ABSTRACTS

compared with five times as many controls matched by birth year, sex, ents had or their trust in the medical staff. Parents’ level of education
and place of living. Data were obtained from The Swedish Childhood positively related with willingness to take part in decision-making, as
Cancer Registry and Statistics Sweden. well as with trust in the medical staff.
Results: Preliminary results show that the number of individuals not Conclusions: The results emphasize the importance of trust in and the
attending any post compulsory education was significantly higher support of the medical and psychosocial staff and to the need to be
within the group treated for brain tumours compared with controls attuned to different parents’ needs to increase this trust
(8.2% vs. 4.2%; p<0.001). Significantly fewer individuals treated for
brain tumours attended high schools (83.6% vs. 90%; p<0.001) or uni-
versities (36.5% vs. 45.6 %; p<0.001) compared with controls, while EP564 / #1866 THE INTRODUCTION OF A ROBOTICS
they to a larger extent attended the Swedish folk high schools (15.3% PROGRAMME TO SUPPORT SCHOOL ABSENCE: WHAT HAVE
vs. 8.8%; p<0.001), educational institutions which focus on provid- WE LEARNT FROM FAMILIES SO FAR?
ing an accessible learning environment. Other preliminary analyses
showed that individuals without post compulsory education, treated Rebecca Kirk1 , Susie Willis1 , Anthony Mccarthy2 , Carla Andrews3 ,
for a brain tumour, had been significantly less employed compared with Elaine Klewchuk1 , Anna Poland4
individuals treated for brain tumours with post compulsory education 1 Royal Belfast Hospital for Sick Children, Paediatric Clinical Psychology,
(40.5% vs. 24.8%; p=0.040). This was the same for controls (28.6% vs. BT BE, United Kingdom; 2 Royal Belfast Hospital for Sick Children, Paedi-
10.7%; p<0.001). atric Oncology, BT BE, United Kingdom; 3 Royal Belfast Hospital for Sick
Conclusions: Participation in post compulsory education seems to Children, Paediatric Clinical Psychology, BT BE, United Kingdom; 4 The Chil-
prevent unemployment. Hence, results indicate a need for more knowl- dren’s Cancer Unit Charity, The Children’s Cancer Unit Charity, BT AQ,
edge about post compulsory education tailored to the specifics needs United Kingdom
of the group to compensate for often-experienced late complications
after a brain tumour treatment. Background and Aims: Children with cancer may experience pro-
longed disruptions to their school attendance due to symptoms of
illness, health risks associated with the illness, and effects of treatment.
EP563 / #930 ANXIETY AND DECISION-MAKING AMONG National reports on childhood cancer survivors indicate that this popu-
PARENTS COPING WITH CHILDHOOD CANCER DURING THE lation is at increased risk of long-term social outcomes, which include
FIRST YEAR OF TREATMENT reduced educational attainment and feelings of loneliness (Gurney
et al., 2009). The Robotics programme, associated with The Children’s
Siwar Makhoul Khoury1 , Sharon Egozi2 Cancer Unit Charity (CCUC) at the Royal Belfast Hospital for Sick Chil-
1 tel hai college, Social Work, keryat shmona, Israel; 2 Tel-Hai College, Social - dren (RBHSC), involves the use of telepresence robots as an alternative
Work, Kfar-Vradim, Israel form of education provision. The robots support students to remain
linked in academically and connected to their classmates from their
Background and Aims: Parents of children with cancer face a high hospital bedside and/or home. This qualitative quality improvement
level of distress. One contributing factor to this is their need to study aimed to explore the practicalities of implementing the robots
make life-altering treatment decisions. In order to understand spe- into families’ lives. It also explored any barriers to engagement from a
cific variables that may contribute to parents’ distress and decision- service-delivery perspective.
making preferences, we assessed correlations between parent anxiety, Methods: Semi-structured interviews were conducted with ten par-
decision-making preferences, personal variables (gender, age, religion, ents (eight mothers and two fathers) of ten children under the care
education, socioeconomic, occupational status), cancer characteristics of the paediatric cancer service at RBHSC who had been invited to
(type, stage, time since diagnosis), existing support system, and trust in take part in the Robotics programme. All children had a minimum of
the medical staff. two months absence from school at the time of the family’s intro-
Methods: Sixty-nine parents of children with cancer during the first duction to the robot. Thematic analysis was used to explore the
year after diagnosis were recruited to the study during their child’s data.
hospitalization, and answered the State-Trait Anxiety inventory, the Results: Five main themes emerged; 1 (Hearing about the robot), 2
Control (decision-making) Preferences Scales for Pediatrics and ques- (Acquiring the robot), 3 (Family introduction), 4 (School introduction)
tions about their environmental support and trust in the medical and 5 (Incorporating into school day).
staff. Conclusions: The results of this study highlight the practical con-
Results: Parents’ anxiety level was significantly higher than the general siderations and obstacles for families’ when telepresence robots are
population, especially for state anxiety. Arab parents had higher trait introduced as a way of supporting school absence in the paedi-
anxiety comparing to Jewish parents, and less environmental support. atric cancer population. Using the data from this quality improve-
Higher socioeconomic status and greater extent of employment of the ment study, resources have been developed to support and assist
spouse related to higher levels of state anxiety. Anxiety did not relate families and schools when a child is enrolled onto the Robotics
to any of the cancer properties, nor to the amount of support the par- programme.
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ABSTRACTS S409 of S635

EP565 / #218 MOTHERS’ AND FATHERS’ PERSPECTIVES ON School of Medicine, Department Of Pediatrics, Sapporo, Japan; 3 Hakodate
HEALTH-RELATED QUALITY OF LIFE OF CHILDREN WITH Municipal Hospital, Department Of Pediatrics, Hakodate, Japan; 4 Sapporo
CANCER Medical University Hospital, Division Of Pediatrics, Sapporo, Japan

Andreas Meryk1 , Gabriele Kropshofer1 , Benjamin Hetzer1 , David Background and Aims: Cancer treatment for children is typically long-
Riedl2 , Jens Lehmann2 , Gerhard Rumpold2 , Alexandra Haid1 , Verena term and difficult. Understanding children’s thoughts and attitudes
Schneeberger-Carta1 , Bernhard Holzner2 , Roman Crazzolara1 when they are informed about cancer, while receiving cancer therapy,
1 Medical University of Innsbruck, Department Of Pediatrics, Innsbruck, and while speculating regarding the consequences of treatment, may
Austria; 2 Medical University of Innsbruck, Department Of Psychiatry, be valuable for informing treatment approaches. Children’s thinking
Psychotherapy And Psychosomatics, Innsbruck, Austria should be considered in cases of shared decision making in pediatric
oncology.
Background and Aims: Evaluation of patient-reported outcome mea- Methods: To examine this question, we conducted long-term observa-
surements (PROMs) in pediatric cancer remains controversial, as tion and interviews with seven children with hematologic cancer, aged
parent-proxy reports significantly differ from child self-reports. The 5 to 10 years, in a Japanese pediatric ward and two outpatient clin-
specific aim of this study was to examine agreement between mother ics. The data collection took a total of 211 days, from 2016 to 2020.
and father proxy report on health-related quality of life (HRQOL) in a This study dealt with a large amount of data on observation narratives
sample of pediatric cancer patients. and children’s discourses using a qualitative analysis that combined
Methods: Based on the Pediatric Quality of Life (PedsQL) 4.0 Generic thematic data analysis with longitudinal process analysis.
Core Scales and PedsQL 3.0 Cancer Module, patients and parents Results: We identified five main themes that encompassed children’s
completed monthly HRQOL reports following the diagnosis of cancer. thoughts and attitudes: making promises with doctors, learning about
Intraclass correlations (ICCs) between child self-report and reports of the treatment procedures, taking care of oneself, increasing the range
mother and father were analyzed at the domain level. of activities they can perform, and living an ordinary life. Children par-
Results: Within the first seven days following cancer diagnosis, 32 ticipated in decision making by engaging in pediatric cancer therapy,
dyads of mother-child and 19 dyads of father-child were included in taking care of their own bodies, and increasing their range of activi-
this prospective longitudinal study. Shortly after diagnosis, mother- ties. Children perceived the therapeutic course as a path to living an
child dyads showed moderate and good agreement on all domains of ordinary life, such as going to school, playing with friends, and living
PedsQL Generic, except for social, whereas father-child dyads showed without wearing a mask. These expectations were maintained from the
only moderate agreement on physical and school domain. With ongoing start of cancer treatment to the follow-up period. Children developed
therapy, moderate agreement remained only on the physical domain, cognitively and socially during these processes.
as both mothers and fathers overestimated impairments. The PedsQL Conclusions: A forward-looking attitude toward understanding cancer,
Cancer Module showed moderate and good agreement not only for the accepting treatment, and looking forward to the future were observed
observable domains (e.g. pain and hurt, nausea) but also for worry (0.77 in children with cancer. In addition, children developed throughout the
[95% CI, 0.52-0.89, P<0.001]) in case of mother-child dyads. Fathers processes of cancer treatment. These findings have implications for
tended to overestimate the child’s symptom burden and achieved only shared decision making in pediatric oncology.
moderate agreement for the domain procedural anxiety (0.56 [95%
CI, 0.04-0.86, P=0.02]), whereas mother-child dyads reached excellent
agreement (0.92 [95% CI, 0.81-0.97, P<0.001]). EP567 / #1714 META-ANALYSIS WITH ALARMING FINDINGS
Conclusions: The results suggest that both parent proxy-reports can OF PSYCHOSOCIAL INTERVENTIONS ON PHYSICAL, MENTAL
provide valid information about the HRQOL of the child. However, AND SOCIAL WELLBEING FOR CHILDREN WITH CANCER AND
mothers and fathers provide significantly different information, sug- THEIR FAMILIES IN LOW- AND MIDDLE-INCOME COUNTRIES
gesting that proxy data should be used with caution.
Tooba Nadeem Akhtar, Özge Balkaya, Harriet Miller, Hisham Morsi,
Holly Clark
EP566 / #142 COGNITIONS AND ATTITUDES OF CHILDREN Pediatric Potential Inc., Research, Plymouth, United States of America
WITH CANCER DURING LONG-TERM CANCER TREATMENT:
IMPLICATIONS FOR SHARED DECISION MAKING IN PEDIATRIC Background and Aims: The greatest proportion of children at risk
ONCOLOGY for cancer reside in Low- and Middle-Income Countries (LAMICs).
Psychosocial care is essential to care in most developed settings seek-
Ryoko Michinobu1 , Masaki Yamamoto2 , Yoshiyuki Sakai3 , Keita ing to address stressors accompanying a cancer diagnosis. However,
Igarashi2 , Takahiro Mikami4 , Kotoe Iesato2 , Akira Takebayashi2 , the evidence on psychosocial interventions in LAMICs remains unex-
Hiroyuki Tsutsumi2 , Takeshi Tsugawa2 plored. This meta-analysis aimed to synthesize findings on psychosocial
1 Sapporo Medical University, Center For Medical Education, Department interventions for children with cancer and their families in LAMIC
Of Liberal Arts And Sciences, Sapporo, Japan; 2 Sapporo Medical University settings.
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S410 of S635 ABSTRACTS

Methods: Systematic searches of five databases were conducted to child’s health-related QOL. Children completed the Family Assessment
identify appropriate studies. Data were extracted and ANOVAs and Device-General Functioning Scale (FAD-GF) to assess family function-
meta-regression analyses were performed. ing. Treatment intensity was rated following chart review using the
Results: Out of 3,501 hits, 334 full texts were screened, with 17 stud- Pediatric Neuro Oncology Rating of Treatment Intensity to divide the
ies eligible for inclusion. All interventions had a combined sample sample into low, moderate, and high intensity groups. Moderation
size of 3,140 and covered 7 countries. Interventions comprised fam- models were tested using the SPSS PROCESS macro, covarying for
ilies/parents of children with cancer, only children or both, and were executive functioning.
art, play music-based, spiritual, religious, financial, logistic support, Results: Diagnosis type was a significant moderator between family
counselling psychotherapy-based, sleep, breathing, relaxation, and functioning and QOL, such that more maladaptive family functioning
solution-focused trainings. Primary outcomes were anxiety, depres- was associated with reduced QOL for the BT group, p=.01. Similarly,
sion, fatigue, distress, burnout, quality of life, treatment completion treatment intensity was a significant moderator, such that family func-
and pain reduction. All showed significant relationships between the tioning was associated with QOL for the high intensity treatment
interventions and outcomes (p < 0.05). The overall log odds ratio was group, p=.04.
1.773 (p < 0.0001), implying not providing a psychosocial interven- Conclusions: The current findings indicate that maladaptive fam-
tion increases the likelihood for poorer health and wellbeing outcomes ily functioning is a risk factor for children with BT and higher
in children with cancer and their parents. Additional analysis showed treatment intensities, highlighting a high-risk group to target for
the outcomes were statistically significant on all domains: emotional, screening and family-level intervention to improve QOL. Future
physical, health, and social wellbeing. There is a large heterogeneity in work should examine these relationships longitudinally with larger
samples and research methodology, and a gross under-representation samples.
of LAMICs.
Conclusions: Over the last decade, an alarming note to the childhood
oncology community was observed. Our meta-analysis points at the EP569 / #605 SOCIAL DETERMINANTS OF COGNITIVE
lack of psychosocial interventions for children with cancer and their OUTCOMES AMONG SURVIVORS OF PEDIATRIC BRAIN
families. This study presents an opportunity for SIOP to direct mem- TUMORS FOLLOWING RADIOTHERAPY
ber countries and outreach teams to develop, adapt, and empirically
evaluate more psychosocial interventions in the chosen population. Taylor Mule’1 , Jason Hodges2 , Shengjie Wu3 , Yimei Li3 , Jason
Ashford1 , Thomas Merchant4 , Heather Conklin1
1 St. Jude Children’s Research Hospital, Department Of Psychology, Mem-
EP568 / #1469 FAMILY MATTERS: THE INTERACTION phis, United States of America; 2 St. Jude Children’s Research Hospital,
BETWEEN FAMILY FUNCTIONING AND TREATMENT INTENSITY Department Of Hematology, Memphis, United States of America; 3 St. Jude
IN CONTEXT OF PEDIATRIC CANCER Children’s Research Hospital, Department Of Biostatistics, Memphis, United
States of America; 4 St. Jude Children’s Research Hospital, Department Of
Emily Moscato, May Albee, Ashley Anil, Matthew Hocking Radiation Oncology, Memphis, United States of America
Children’s Hospital of Philadelphia, Behavioral Oncology, Philadelphia,
United States of America Background and Aims: Social determinants of health such as parental
occupation, household income, and neighborhood environment have
Background and Aims: Background: Children with cancer have lower been used to predict cognitive functioning among healthy and ill chil-
quality of life (QOL) compared to peers, yet heterogeneity under- dren; however, few pediatric oncology studies have investigated this
scores a need to understand how risk and resilience factors interact. relationship. The primary study aim was to use the Economic Hardship
Children diagnosed with brain tumors (BT), those with higher inten- Index (EHI) to measure neighborhood-level social and economic condi-
sity treatments (i.e., cranial radiation and intensive chemotherapies), tions to predict cognitive outcomes among children treated for brain
and those with more maladaptive family functioning (i.e., lower cohe- tumors with radiotherapy (RT).
siveness/communication) have lower QOL. Aim: To evaluate how risk Methods: 241 children treated on a prospective, longitudinal, phase II
factors interact to determine if family functioning impacts QOL dif- trial of conformal photon RT (54-59.4 Gy) for ependymoma, low-grade
ferentially depending on diagnosis and treatment intensity. It was glioma, or craniopharyngioma (52% female, 79% White, age at RT=
hypothesized that diagnostic group and treatment intensity would 7.76±4.97 years) completed serial cognitive assessments (intelligence
moderate the relationship between family functioning and QOL, such quotient [IQ], reading, math, adaptive behavior) for ten years. Six US
that family functioning would be more predictive of QOL for BT with census tract-level EHI scores were calculated for an overall EHI score:
higher treatment intensity. unemployment, dependency, education, per capita income, crowded
Methods: Participants included school-aged children (ages 7-14) who housing, and poverty. Socioeconomic measures in the existing litera-
completed cancer treatment within six months for either BT (n=42) ture (i.e., Barratt Simplified Measure of Social Status [BSMSS] based
or non-central nervous system solid tumor (ST; n=29). Caregivers on parental income and occupation, and insurance type- no insurance,
completed the Pediatric QOL Inventory (PedsQL 4.0) to assess their public, private) were also derived.
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ABSTRACTS S411 of S635

Results: Spearman correlations revealed EHI overall score and 3 of EP571 / #189 EXPERIENCES OF CHILDHOOD CANCER
6 subscores associated with the BSMSS (r=-.22 to .37; p< .005), and SURVIVORS AND THEIR PARENTS WITH A PHYSICAL AND
Kruskal-Wallis Tests indicated EHI overall score and 2 of 6 subscores SOCIAL INTERVENTION DURING CANCER TREATMENT - A
associated with insurance type (p< .05). Linear mixed models adjusted RESPECT STUDY
for age at RT and sex indicated a significant impact of EHI (overall,
unemployment, per capita income, poverty) on baseline cognitive per- Natasha Nybro Petersen1 , Hanne Larsen2 , Anna Pouplier1 , Peter
formance (IQ, reading, math, adaptive behavior, p< .05), as well as Schmidt-Andersen2 , Troels Thorsteinsson2 , Kjeld Schmiegelow3 ,
change in cognition over time (IQ, math, p< .05). Martin Fridh2
Conclusions: Neighborhood-level measures of social and economic 1 University hospital of Copenhagen, Rigshospitalet, Paediatric Oncology
conditions such as the EHI are associated with easily-derived socioe- Research Laboratory, Department Of Paediatrics And Adolescent Medicine,
conomic status measures but also account for unique variance in København, Denmark; 2 University of Copenhagen, Rigshospitalet, Pae-
cognitive outcomes. Research is needed to isolate modifiable factors diatric Oncology Research Laboratory, Department Of Paediatrics And
within these social and economic conditions to develop interventions Adolescent Medicine, Copenhagen, Denmark; 3 University of Copenhagen,
that improve cognitive outcomes for childhood cancer survivors. Rigshospitalet, Department Of Child And Adolescent Cancer, Copenhagen,
Denmark

EP570 / #1198 IS MENTORING HEALING? AN ANALYSIS OF Background and Aims: Physically inactivity during treatment results
MENTORS EXPERIENCES AS PART OF THE BEREAVED PARENT in children with cancer remaining inactive post-treatment. Further-
MENTORSHIP PROGRAM IN PAKISTAN more, recurrent hospitalizations often cause prolonged absences from
school and a dramatic reduction in peer interactions. The combination
Asma Naheed, Wasfa Farooq, Hamna Adnan, Neelam Abdulqudoos of school absence, being physically impaired, and lacking peer interac-
The Indus hospital, karachi, pakistan, Psychosocial, Karachi, Pakistan tions negatively impact quality of life for the child with cancer. What
these children and their parents consider to be barriers to physical
Background and Aims: Bereaved Individuals providing support to oth- activity during treatment is pivotal. This study explores experiences of
ers in need of support find this connection meaningful and helpful. childhood cancer survivors and their parents with a combined phys-
A parent-to-parent program that involves training bereaved parents ical and social activity intervention during treatment, including the
learn from their loss to support those recently bereaved. Providing program’s impact on survivors’ physical activity post-treatment.
emotional support to families going through similar experiences could Methods: We designed a phenomenological-hermeneutic qualitative
be healing for mentors. This study is conducted to explore parents’ interview study. Using a criterion-sampling strategy, 18 childhood
experiences and factors involved in the role of mentorship. Their expe- cancer survivors (aged 11-18 years) and their parents were inter-
riences were explored in four domains 1) Their feelings as part of viewed from September 2019 through May 2020. Data analysis used
program 2) Their expectation as mentor3) Their feelings and expecta- a deductive thematic approach focused on meaning.
tion related to interactions with mentee 4) To explore difficulty faced Results: Three themes emerged: 1) being physically active during
at personal level associated with mentorship. hospitalization; 2) peers as motivators; and 3) physical activity post-
Methods: Parents serving as Bereaved mentors at TIH were inter- treatment. During hospitalization, daily motivation to do physical activ-
viewed to explore in four different areas. Their experiences were ity was dependent on the well-being, i.e., the presence of the child’s
explored using semi structured interviews. Interviews were held on treatment side effects. Healthy classmates provided a distraction,
zoom and were recorded. Thematic analysis was used to analyze data. reduced loneliness, and promoted normality for those hospitalized.
Results: Overall parents reflected positive experiences and self- When surplus energy was lacking, some children preferred doing physi-
satisfaction on becoming a part of BPP. Self-learning, self-growth, cal activity alone with a professional. Those who were physically active
and self-healing were major driven forces at mentors level as a in the hospital sustained being physically active post-treatment while
source of satisfaction and participation. Parents shared a positive their parents continued seeking advice about appropriate activity
attitude towards training as well and it helped them explore their levels.
emotions and helped them achieve better communication. Overall Conclusions: Childhood cancer survivors and their parents bene-
analysis reflects mentorship and training as an aided tool to their grief fited from the intervention which also provided guidance to remain
journey. physically active post-treatment. This was particularly true for the
Conclusions: Participating in bereaved parent program serves as posi- participants with leukaemia.
tive experience for parents. Mentorship not only gives meanings of life
to mentors but actually provide them with driving force to continue
legacy of their child and aid them in their grief journey. Because of first EP572 / #322 EXPERIENCES WITH ONLINE PEER VISITS AT
batch of mentors, few participants were interviewed, which lower gen- THREE DANISH PEDIATRIC ONCOLOGY WARDS DURING THE
eralizability, to overcome this limitations next study can be done more COVID-19 PANDEMIC – A QUALITATIVE STUDY
objectively.
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S412 of S635 ABSTRACTS

Natasha Nybro Petersen1 , Hanne Larsen2 , Mette Weibel3 , Karin 2 University of Florida, College Of Public Health And Health Professions,
Nissen4 , Marianne Olsen5 Gainesville, United States of America; 3 University of Wisconsin-Milwaukee,
1 University hospital of Copenhagen, Rigshospitalet, Paediatric Oncology Psychology, Milwaukee, United States of America; 4 Nationwide Children’s
Research Laboratory, Department Of Paediatrics And Adolescent Medicine, Hospital, Pediatric Palliative Care, Columbus, United States of America;
København, Denmark; 2 Rigshospitalet, Department Of Pediatrics And Ado- 5 Nationwide Children’s Hospital, Pediatric Oncology, Columbus, United
lescent Medicine, København, Denmark; 3 Lund University, Department Of States of America
Health Sciences, Faculty Of Medicine, Lund, Sweden; 4 Aarhus University
Hospital, Department Of Pediatrics, Aarhus, Denmark; 5 Aalborg Univer- Background and Aims: While parents of children with cancer
sity Hospital, Department Of Pediatrics And Adolescent Medicine, Aalborg, are at-risk for elevated stress and caregiver strain, they must
Denmark also manage other responsibilities both inside and outside of
the hospital. These challenges may be magnified when the child
Background and Aims: During the COVID-19 pandemic, children with has advanced disease. Thus, we examined how mothers’ and
cancer were at higher risk of social isolation since visits were strictly fathers’ stress, family roles and satisfaction, and social support
reduced to parents’ visits only. To carry on an existing intervention of relate to caregiver strain in the context of advanced pediatric
peer visits during hospitalization, we moderated the intervention to cancer.
an online version during COVID-19. The online visits were provided Methods: Data were from a longitudinal study of families of chil-
during school hours. Research nurses and the children’s teachers facil- dren (aged 5-25) with advanced cancer (i.e., physician-estimated
itated the online visits. This study explores how children with cancer, prognosis<60%, relapsed, or refractory disease). At enrollment, moth-
their peers, teachers, and nurses experienced online visits. ers (n=55; Mage =41.41 years; 87% White) and fathers (n=30;
Methods: Using purposive sampling, children with cancer aged 7-15 Mage =43.95 years; 83% White) reported on the division of 7 family
years (n=15), their peers (n=15), nurses (n=4), and teachers (n=4) par- roles (e.g., medical care of ill child, caring for others, household chores)
ticipated in semi-structured interviews from January 2022 through and their satisfaction with each role. Parents also reported on their
March 2022. The interviews were analyzed using an inductive thematic cancer-specific stress, general stress, social support, and caregiver
approach. strain.
Results: In total, 70 online visits were facilitated from March 2020 Results: Parents reported moderate caregiver strain (Scale: 1-5; Moth-
through March 2022. The online visits varied from 15 minutes to 90 ers: M=2.88, SD=0.67; Fathers: M=2.59, SD=0.59). Fathers reported
minutes. We suggest two central themes: maintaining social attach- family roles were shared equally, whereas mothers reported either
ment through online interaction and facilitating online visits based on sharing roles or completing them independently. Parents were highly
a preliminary analysis. Preliminary findings indicate that the children satisfied with their roles (Scale: 0-3; Mothers: M=2.45, SD=0.70;
are overall pleased with online visits to maintain their social rela- Fathers: M=2.65, SD=0.46). Accounting for income and marital sta-
tions during the COVID-19 pandemic. Regardless, online visits cannot tus, multiple regression analysis, F(7,42)=7.41, p<0.001, R2 =0.55,
replace face-to-face interaction between the children as the content revealed that greater caregiver strain for mothers was associated
and quality may change during the online visits. Findings indicate with greater general stress (β=0.45, p=0.001), greater satisfaction
that technical shortcomings with equipment, e.g., computers, internet with family roles (β=0.25, p=0.04), and lower social support (β=-0.25,
connection, and online meetings platform, negatively impacts the chil- p=0.04). For fathers, F(5,22)=4.34, p=0.01, R2 =0.50, greater caregiver
dren’s experiences with the online visits. Furthermore, the nurses and strain was only associated with greater cancer-specific stress (β=0.48,
teachers experience that the online visits need facilitation, e.g., sug- p=0.02).
gesting games, participating in the games, or introducing conversations Conclusions: In the context of advanced pediatric cancer, fathers
E-Poster Topics to get an experience of flow. may experience caregiver strain as cancer-specific stress increases,
Conclusions: During hospitalization, online visits from peers may sup- whereas mothers’ strain may depend on broader family and social
port children with cancer in maintaining contact with their school and factors. Psychosocial providers should address general and cancer spe-
classmates. cific stress within families, in addition to social support (especially for
mothers). Additional research regarding the family impact of advanced
pediatric cancer is needed.
EP573 / #775 FACTORS ASSOCIATED WITH CAREGIVER
STRAIN AMONG MOTHERS AND FATHERS OF CHILDREN WITH
ADVANCED CANCER EP574 / #999 PSYCHOMETRIC PROPERTIES AND
EXPLORATORY ANALYSIS OF THE CANCER WORRY SCALE IN
Anna Olsavsky1 , Kylie Hill1 , Malcolm Sutherland-Foggio1 , Charis YOUTH SURVIVORS OF CHILDHOOD CANCER AND THEIR
Stanek1 , Alexandra Himelhoch2 , Ansley Kenney3 , Lisa Humphrey4 , PARENTS
Randal Olshefski5 , Leena Nahata1 , Cynthia Gerhardt1
1 The Abigail Wexner Research Institute at Nationwide Children’s Hospi- Michaela Patton1 , Tessa Carrels2 , Brooke Russell1 , Caitlin Forbes2 ,
tal, Center For Biobehavioral Health, Columbus, United States of America; Mehak Stokoe2 , Fiona Schulte3
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S413 of S635

1 University of Calgary, Psychology, Calgary, Canada; 2 University of Cal- for evaluation. Although data suggests the benefit of patient/caregiver-
gary, Cumming School Of Medicine, Calgary, Canada; 3 University of Calgary, oriented research, few pediatric oncology studies have been identified
Oncology, Calgary, Canada that include caregivers as authors. This study assesses the feasibil-
ity of a mixed-methods, caregiver-oriented approach to explore the
Background and Aims: Forty three percent of pediatric cancer sur- impact of the COG KidsCare App on the education/support of pediatric
vivors experience fear of cancer recurrence (FCR); yet, no brief mea- oncology patients/families in Atlantic Canada.
sures have been validated for youth survivors. The aim of this study was Methods: A questionnaire with 23 multiple-choice questions was
to evaluate the psychometric properties of the 6-item Cancer Worry developed using OPINIO software and disseminated to par-
Scale (CWS) in pediatric cancer survivors. ents/caregivers via email. Parents/caregivers were invited to
Methods: Survivors [n=146; 49.3% male, Mage =16.7 years (Range=8- participate in a virtual, one-hour semi-structured interview (13
25)] were recruited from Canada. Participants completed the CWS, open-ended questions), hosted by a parent/caregiver on the study
Benefit-Burden Scale, Pain Catastrophizing Scale, Patient Reported team. All participants were identified by investigators using con-
Outcome Measurement Information System – Anxiety and Depres- venience sampling. Data was analyzed using descriptive statistics.
sion scales, Child Posttraumatic Stress Scale, Pediatric Quality of Participants were invited to share their feedback on the questionnaire
Life Inventory, Intolerance of Uncertainty Scale, and the Posttrau- and interview.
matic Stress Disorder Checklist for the Diagnostic Statistical Manual Results: This pilot study was fully implemented as planned within six
of Mental Disorders (Version 5). Internal consistency was calculated weeks. Five parents/caregivers completed the questionnaire. There
using Cronbach’s alpha (Good = α>0.8). Fit indexes were calculated were no missing responses. Two participated in the semi-structured
using confirmatory factor analysis (target values: X2 , p>.05; GFI>.95; interview; both were engaged and spoke freely for over one hour.
RMSE<.06; CFI>.95; TLI>.95). Pearson correlations were used to All participants agreed that the COG KidsCare App provided helpful
calculate associations between the CWS and other outcomes. educational materials and made them feel better supported. Minor
Results: Internal consistency was good (α=.906). Confirmatory factor wording changes were made to one questionnaire item and two
analysis yielded pattern coefficients relating the latent variable with interview questions to provide better clarity for future participants.
the items (Range=.54-.92). Fit indexes for the model revealed a sta- Conclusions: A mixed-methods approach that includes a par-
tistically significant chi square test, 29.16 (9, N=146), p=.001. The ent/caregiver on the study team is a novel and feasible method of
GFI (.94), RMSEA (.12), CFI (.97), and TLI (.94) suggest that the model exploring the impact of Apps in pediatric oncology. Having a par-
was an adequate fit. The CWS was significantly correlated to anxiety ent/caregiver host a semi-structured interview facilitated honest and
(r=.50, p<.001) and depressive symptoms (r=.38, p<.001), posttrau- open discussion. Preliminary results suggest that the COG KidsCare
matic stress symptoms (r=.50, p<.001), intolerance of uncertainty App improves education and support of parents/caregivers.
(r=.25, p=.008), quality of life (r=-.36, p<.001), disease burden (r=.53,
p<.001), and pain catastrophizing (r=.32, p<.001); but not benefit
finding (p=.126). EP576 / #1882 COMMUNITY STIGMA TOWARDS CHILDREN
Conclusions: Results showed that the 6-item CWS is a good option WITH CANCER IN TANZANIA
to briefly assess FCR in youth, but based on the fit indexes, does not
appear to be a robust measure of FCR. Future research should create a Suhana Posani1 , Erica Sanga2 , Francis Karia3 , Hillary Sued2 , Kathryn
FCR measure from the ground up to better elucidate this construct in Pollak4 , Kristin Schroeder5
younger populations. 1 Duke University, Duke Global Health Institute, Durham, United States of
America; 2 National Institute for Medical Research, Public Health, Mwanza,
Tanzania; 3 Duke Clinical Research Institute, Office Of Clinical Research,
EP575 / #1125 A MIXED-METHODS, CAREGIVER-ORIENTED Durham, United States of America; 4 Duke University, Duke Cancer Insti-
APPROACH IS FEASIBLE TO EXPLORE THE IMPACT OF THE tute, Durham, United States of America; 5 Duke University Medical Center,
CHILDREN’S ONCOLOGY GROUP KIDSCARE APP ON Pediatric Oncology And Global Health, Durham, United States of America
EDUCATION/SUPPORT OF PEDIATRIC ONCOLOGY
PARENTS/CAREGIVERS Background and Aims: In Tanzania, prior research with caregivers
of children with cancer and expert health care providers explored
Meghan Pike, Katrina Macdonald, April Connolly, Bilal Marwa, Melissa how misbeliefs and perceived community reaction and fears lead to
Hum, Stephanie Villeneuve delays in health seeking behavior and increased treatment abandon-
IWK Health Centre, Pediatric Oncology, halifax, Canada ment. To target this internalized stigma and improve outcomes, tailored
interventions are needed at the community level. However, the com-
Background and Aims: The Children’s Oncology Group (COG) munity perspective on stigma towards childhood cancer has not been
recently launched the COG KidsCare App, which contains educational evaluated, a critical knowledge gap.
materials for parents/caregivers. Studies on the impact of Apps in pedi- Methods: This was a descriptive qualitative study conducted in the
atric oncology are limited and there is no published standard method Mwanza region. Community members aged ≥18 years from 1 rural and
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S414 of S635 ABSTRACTS

1 urban community were purposively selected to participate in three tions and analyzed with an inductive approach using thematic
focus group discussions (each with 8-12 respondents) in April 2021. analysis.
Data were transcribed, coded and analyzed by thematic content with Results: Observations were carried out during 50 group- and individ-
the support of NVIVO software. ual structured active play sessions (37 hours), resulting in 83 different
Results: A total of 28 participants were interviewed. Individual and scenic descriptions relating to 15 children (aged 1 to 5, 7 girls, and
health system level stigma was expressed by FGD participants, includ- 8 boys). Preliminary findings indicate three overarching themes: 1)
ing avoidance, severity, public stigma and health practitioner stigma. Wanting to play; 2) Playing together; 3) Gaining confidence. When
In each focus group, participants mentioned the belief that cancer given the opportunity, children willingly participate in active play
was incurable. Public stigma was also highly referred to, with some regardless of being perceived as unwilling or unable by parents or care-
believing that childhood cancer was due to bewitchment and seek- givers. In the structured active play, children, parents, and exercise
ing treatment at the hospital will not help. Structural level stigma professionals play together as equals, teaching the children to take
was not discussed by participants, including policy opposition, financial charge, take turns and be inclusive. With the support of exercise profes-
discrimination, and employment stigma. sionals and parents, the children can gain confidence in their movement
Conclusions: Individual level stigma towards children diagnosed with abilities even when struggling with treatment-related side-effects —
cancer is present within the Tanzanian community. The common belief showing themselves and their parents that they can do it.
that childhood cancer is incurable and may be due to the child being Conclusions: Preschool children with cancer want to play and can be
bewitched likely contributes to delayed health seeking behaviour, their own best gatekeepers in activities and play. Their participation
especially in rural areas. Additional evaluation of organizational level in structured active play during hospitalization may positively impact
stigma is needed, but may be better perceived and evaluated by care- personal and social development.
givers and patients directly. Cultural and contextually relevant aware-
ness interventions are needed to decrease stigma towards childhood
cancer and increase cancer health-seeking behaviour in Tanzanian EP578 / #1822 EXECUTIVE FUNCTIONING IN A
communities. MULTI-ETHNIC SAMPLE OF PEDIATRIC SURVIVORS OF ACUTE
LYMPHOBLASTIC LEUKEMIA (ALL): EXPLORING THE BILINGUAL
ADVANTAGE
EP577 / #1244 “I CAN DO IT”—A QUALITATIVE STUDY ON
HOW REHABILITATION INCLUDING STRUCTURED ACTIVE Kimberly Raghubar1 , Rosalia Costello2 , Johanna Escalante1 , Melissa
PLAY IMPACTS PERSONAL AND SOCIAL DEVELOPMENT IN Richard3 , Michael Scheurer4 , Austin Brown5
PRESCHOOLERS DIAGNOSED WITH CANCER 1 Texas Children’s Hospital/Baylor College of Medicine, Pediatrics - Psychol-

ogy, Houston, United States of America; 2 Texas Children’s Hospital/Baylor


Anna Pouplier1 , Martin Fridh1 , Jan Christensen2 , Peter College of Medicine, Pediatrics-psychology, Houston, United States of Amer-
Schmidt-Andersen1,2 , Helle Winther3 , Hanne Larsen1,4 ica; 3 Baylor College of Medicine, Pediatric Hematology And Oncology,
1 University of Copenhagen, Rigshospitalet, Paediatric Oncology Research Houston, United States of America; 4 Baylor College of Medicine, Pedi-
Laboratory, Department Of Paediatrics And Adolescent Medicine, Copen- atrics, Houston, United States of America; 5 Texas Children’s Hospital, Baylor
hagen, Denmark; 2 Copenhagen University Hospital, Rigshospitalet, Depart- College of Medicine, Pediatrics, Houston, United States of America
ment Of Occupational And Physiotherapy, Centre Of Head And Orthope-
dics, Copenhagen, Denmark; 3 Faculty of Science, University of Copenhagen, Background and Aims: Deficits in executive functioning (EF) are
Department Of Nutrition, Exercise And Sports, Copenhagen, Denmark; common cognitive late effects of treatment for childhood acute lym-
4 University of Copenhagen, Faculty Of Health Sciences, København, Den- phoblastic leukemia (ALL). Latino children are at increased risk of
mark neurotoxic events during treatment, which may predispose them to EF
difficulties post-treatment. Bilingualism has been shown to correlate
Background and Aims: Development of gross motor skills (i.e., jump- with better EF in healthy children and may provide some protection
ing, running, hopping, throwing, and kicking), as well as personal and against therapy-related neurological deficits. The primary objective of
social competencies, are affected by cancer treatment in preschool this study was to extend the investigation of a “bilingual advantage” in
children — with long-term implications. This study aims to explore EF to childhood ALL.
how a structured active play intervention impacts the development of Methods: Performance-based and parent report measures of EF were
preschoolers’ (1-5 years old) social and personal skills expressed by examined at a single time-point in survivorship among monolingual
their behavior, body language, and verbal expressions. English-speaking (n = 44) and bilingual Spanish-English-speaking (n
Methods: Using convenience sampling, the project team conducted = 22) survivors of childhood ALL. Aspects of performance-based EF
12 months of participant observations of the structured active include concept formation, inhibition, and visual and verbal working
play intervention sessions. The observations focused on situa- memory. Parent ratings on the behavioral regulation index and the
tions with social interaction, joy of movement, and confidence in metacognitive index of the Behavior Rating Inventory of Executive
movement. Observations were written as narrative scenic descrip- Function were also collected.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S415 of S635

Results: Participants (56% male) were diagnosed with standard risk icated resource for psychosocial services and no immediate plans to
ALL (64%) at a mean age of 5.40 years. Survivors were evaluated appoint one.
an average of 6 years post-diagnosis. Bilingual children performed Conclusions: Paeds-Oncology centers of Pakistan are lacking in
better than monolingual children on performance-based tasks of inhi- psycho-oncology services for the mental well-being of children battling
bition (p < .05) and verbal working memory (p < .01), and parent cancer. Additional research can be conducted to explore the fac-
ratings of metacognitive aspects of EF (p < .05). The observed effect tors causing constraints in the availability of Paeds-Psycho-Oncology
of bilingualism on parent ratings of metacognitive aspects of EF services in Pakistan.
remained after accounting for SES and was marginally significant for
performance-based inhibition (p = .053). Parent ratings completed by
Spanish-speaking parents correlated with performance-based testing EP580 / #997 QUALITY OF LIFE FOR ADOLESCENT AND
(r = -.69 for inhibition and -.70 for verbal working memory). YOUNG ADULT HEMATOPOIETIC STEM CELL TRANSPLANT
Conclusions: This study presents preliminary evidence of a bilingual PATIENTS AND HEALTHCARE PROVIDERS: A MIXED METHODS
advantage for specific aspects of EF, namely inhibitory control. Bilin- STUDY
gual language exposure and fluency may act as a protective factor
against commonly observed weaknesses in EF among survivors of Rachel Sauls1 , Sylvia Crowder2 , Laura Redwine3 , Farhad Khimani4 ,
childhood ALL. Marilyn Stern5
1 University of South Florida, Department Of Psychology, Tampa, United
States of America; 2 Moffitt Cancer Center, Health Outcomes And Behavior,
EP579 / #1652 ACCESS TO PSYCHO-ONCOLOGY SERVICES Tampa, United States of America; 3 University of Miami, Department Of Pub-
FOR CHILDHOOD CANCER PATIENTS IN PAKISTAN lic Health Sciences, miami, United States of America; 4 Moffitt Cancer Center,
Department Of Blood And Marrow Transplant And Cellular Immunotherapy,
Afia Tul Quanita1 , Muhammad Rafie Raza2 , Wasfa Farooq3 Tampa, United States of America; 5 University of South Florida, Child And
1 The Indus Hospital and Health and Network, Paediatric Oncology, Karachi, Family Studies, Tampa, United States of America
Pakistan; 2 The Indus Hospital and Health Network, Paediatric Oncology,
Karachi, Pakistan; 3 The Indus Hospital and Health Network, Pediatric Background and Aims: Previous research in adolescent and young
Oncology, Karachi, Pakistan adult (AYA) patients suggests an association between hematopoietic
stem cell transplantation (HSCT) and physical dysfunction, resulting
Background and Aims: The burden of cancer in children is increas- in physiological stress impairing quality of life. However, few studies
ing globally. Improvement and access to services are needed to have examined both patients and healthcare providers’ perceptions of
treat this disease as well as enhancing mental well-being, highlight- quality of life while undergoing HSCT. This study aims to address this
ing the need for dedicated pediatric psycho-oncology services for gap in research by 1) gathering information from quantitative surveys
patients and their families. Cancer treatment is a long and dif- at three-time points and 2) completing qualitative semi-structured
ficult journey that becomes exponentially harder without psycho- interviews with HSCT health care providers
social support. This study explored the availability and existing gap Methods: This mixed-methods study assessed AYA HSCT patients (5
of paediatric-oncology services in paediatric-oncology units across females, 6 males[MS1] [MS2]; mean age = 28, range from 23 to 38
Pakistan. years) on quality of life (QOL) using the PROMIS® HealthMeasures
Methods: An online survey was created on google forms and circulated survey at three-time points: before, directly after, and 3-months post-
to unit heads and psychologists across all childhood cancer facilities. A HSCT. Responses ranged on a Likert scale from 1-5, lower scores
total of 11 participants completed the online questionnaire containing indicating greater QOL. T-tests and repeated measures ANOVA were
nine open-ended and eleven multiple-choice questions. used to determine statistical significance across time points using
Results: Out of the 13 units surveyed, 11 centers responded, and SPSS v.27 (p=0.05). Healthcare providers (n=10) participated in semi-
(54.5%) have a psycho-social department existing in their facility of structured interviews post-transplantation. Qualitative themes were
which 4 are dedicated to paediatric oncology. Play area for children identified and integrated with quantitative data.
was present in 8(72%) of facilities. There are only 5(45%) centers Results: Patients reported higher fear levels before as compared to
that have psychologists, 3(27%) only social workers and 3(27%) have directly after and 3-months post-HSCT (p<0.05). Patients reported
none of them. All of the dedicated psycho-social departments had higher fatigue directly after HSCT as compared to before and 3-months
required NGO and welfare support for hiring psychosocial personnel post-HSCT (p<0.05). Health care providers described “stress,” “anxi-
since state allotted positions did not exist. The psycho-social worker ety,” and “physical complications” as common patient reactions during
has always been involved in inpatient counseling, particularly in enu- the HSCT trajectory. When asked about the utility of mindfulness-
cleation and amputation cases in most centers. The majority of the based interventions, HPs indicated that they would likely be “effective,”
centers’ psycho-social departments don’t conduct training sessions for “helpful,” and “beneficial” for patients to reduce stress.
other staff members regarding counseling techniques and the mental Conclusions: Patients reported the highest fear before HSCT and
health of patients. A vast majority of units did not have a full-time ded- greatest fatigue immediately following HSCT. These findings are
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S416 of S635 ABSTRACTS

supported by qualitative interviews conducted with HPs regarding ods for engaging children in exercise in their household should be
stress and anxiety observed in clinic. All health care providers agreed explored further.
that a mindfulness-based intervention for their patients would improve
their quality of life, such an intervention is currently being designed.
EP582 / #486 IMPACT OF COVID-19 PANDEMIC ON
FAMILIES OF CHILDREN WITH CANCER
EP581 / #1232 FACILITATORS AND BARRIERS THAT
INFLUENCE ENGAGEMENT IN EXERCISE INTERVENTION Gnanamani Senguttuvan, Amita Trehan, Richa Jain, Deepak Bansal,
DURING THE FIRST SIX MONTHS OF TREATMENT IN Srinivasan Peyam
CHILDREN AND ADOLESCENTS WITH CANCER: A Postgraduate Institute of Medical Education & Research, Pediatrics, Chandi-
QUALITATIVE STUDY garh, India

Peter Schmidt-Andersen1,2 , Natasha Petersen2 , Anna Pouplier2 , Klaus Background and Aims: The pandemic affected the continuum of care
Müller2,3 , Jan Christenen1 , Martin Fridh2 , Hanne Larsen2,3 in patients & had a harsh effect on the makeup of society disrupting
1 Copenhagen University Hospital, Rigshospitalet, Department Of Occupa- life. This study assessed the impact of the pandemic on families with
tional And Physiotherapy, Centre Of Head And Orthopedics, Copenhagen, children with a malignancy.
Denmark; 2 Copenhagen University Hospital, Rigshospitalet, Paediatric Methods: A survey questionnaire, taking 20-30 minutes (6 parts;
Oncology Research Laboratory, Department Of Paediatrics And Adolescent 35 questions) was provided to the participants (families of children
Medicine, The Juliane Marie Center, Copenhagen, Denmark; 3 University of diagnosed with cancer from July 2019 to March 2020).
Copenhagen, Institute Of Clinical Medicine, Copenhagen, Denmark Results: Families of sixty patients on therapy and 23 who had com-
pleted therapy (median age: 7.13 [2-14] years) participated. Two-third
Background and Aims: Early initiation of physical exercise programs families belonged to rural areas and 40% were from the upper-lower
is needed to limit the physical deterioration caused by inactivity and socioeconomic strata. Social media (n=74, 89%) and medical profes-
treatment-induced toxicities during anti-cancer treatment in children sionals (n=61, 73.5%) were main sources of COVID-19 information.
and adolescents with cancer. We aimed to identify facilitators and bar- Fifty-three percent school going patients transitioned to virtual learn-
riers that influence engagement in an exercise intervention during the ing and 22% quit school. One fourth children became less physically
first six months of treatment. active. Information received about COVID-19 specific to cancer was
Methods: By using a qualitative study design and purposely sampling, perceived adequate by 25% families. Major factors which took a toll
we generated data from semi-structured interviews with children diag- on the psychological wellbeing of the families were (i) risk of child
nosed with cancer (n=9) (6-17 of age) and their parents (n=10). The contracting SARS-Cov-2 infection [n=64, 77%], (ii) greater morbid-
interviews were conducted from December 2021 through February ity/mortality owing to underlying cancer& lack of definitive therapy for
2022. Deductive thematic analysis was used based on three com- COVID 19 [n=58;70%] (iii) fiscal hardship [75%](iv) adequacy of virtual
ponents of self-determination theory: autonomy, competence, and schooling (40%) . Seventy six percent participants reported a nega-
relatedness. tive impact on employment, 25% losing jobs entirely. Handwashing
Results: A preliminary analysis suggests three central themes: 1) Clin- and wearing face masks were the most adhered behavioural modifica-
ical versus household environment (autonomy); Children and parents tions (100% and 97%). Most families avoided public places (96%) and
described that they (children) in general, were motivated for exercise using public transport (52%) with 76% avoiding contact outside home.
when hospitalized if supervised by exercise professionals, despite feel- Twenty percent families experienced problems with regards to delay in
ing less capable of exercise due to presence of side effects. However, diagnosis, appointments and therapy.
their home environment often lacked the equipment and presence of Conclusions: ‘Coronaphobia’ psychological stress was observed
exercise professionals, and therefore, some children and their parents in 75% families with a child with a malignancy. Economic loss
experienced being unmotivated and sedentary. 2) Keeping side effects (75%), modification/absence of schooling (75%) and delayed
at bay (competence); Exercise was described to ameliorate certain side theranostics (20%) resulted owing to an unprecedented disman-
effects, creating confidence in keeping some degrees of fatigue and tled community & social network and disruption of health care
nausea at bay. However, structure and age-appropriate communication facilities.
on physical activity and exercise were sought-after by some parents.
3) Confidentiality with exercise professionals (relatedness); trust and
confidentiality with the exercise professionals during hospitalization EP583 / #1047 PSYCHO SOCIAL PROBLEMS OF CHILDREN
are described as crucial for children to engage in the exercise sessions. AND CAREGIVERS REFERRED TO THE PEDIATRIC PSYCHO
Conclusions: Although exercise interventions may be challenging to ONCOLOGY SERVICES IN A TERTIARY CARE CANCER CENTER
conduct in children during the first six months of anti-cancer treat- IN A DEVELOPING NATION
ment, with fluctuating side effects and hospitalization, children and
parents describe exercise as relevant, motivating, and engaging. Meth- Lekhika Sonkusare1 , Jayita Deodhar2 , Savita Goswami3
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S417 of S635

1 Tata Memorial Hospital, Psychiatry And Psycho-oncology, Mumbai, India; risk population. Little research is available on lifestyle interventions for
2 Tata Memorial Hospital, Department Of Palliative Medicine, mumbai, PCS during the pandemic. This study examines the impact of COVID-
India; 3 Tata Memorial Centre, Psycho Oncology, Mumbai, India 19 on participants and research staff in our multi-site randomized
controlled trial, NOURISH-T+; and whether an intervention target-
Background and Aims: Childhood cancer is considered a crisis not ing parents as role models for change can promote healthy eating and
only for young patients but also for his/her entire family and social exercise in PCS.
environment. The period of disease and treatment is physically and Methods: Thus far, n=80 dyads of PCS (M=10 years) and their par-
emotionally stressful for children and family who must adapt to a ents have been randomly assigned to NOURISH-T+ or comparison,
hospital environment with not only physical but also psychosocial chal- Enhanced Usual Care (EUC). This cross-sectional analysis included
lenges. The aim of this study is to evaluate psychosocial problems of descriptive statistics of baseline demographics, COVID-19-related fac-
children with cancer and caregivers. tors, and lifestyle behavior changes from post-assessment surveys.
Methods: A retrospective analysis of all new referrals of children with Preliminary thematic analysis was conducted for qualitative feedback
cancer and caregivers of pediatric cancer patients referred to a special- from research staff.
ist Psycho-Oncology service in a tertiary cancer care center over the Results: Participants were ≥ 6 months off-treatment, and 20% had
period of 3 years (January 2019 to December 2021) was done. Children received cancer treatment during the COVID-19 pandemic. Mean
up to 12 years of age and on active oncology treatment were included baseline child BMI%ile was 95th . At baseline, many participants
in the present study.Caregivers were independently referred for psy- reported engaging in more home cooking (61%), snacking (50%), and
cho social issues by pediatric oncology unit and they were not related eating out (37%) and less exercise 63%) since the pandemic began.
to the pediatric patients included in this study. Socio demographic and COVID-19 created barriers related to participant recruitment and
clinical details of all referrals were noted. Clinical interview details as engagement, technology access and literacy, and data collection and
noted in Psycho-Oncology case record forms. management, as well as COVID-related challenges (e.g., Zoom fatigue).
Results: We analyzed records of 99 children who met the eligibility cri- Strategies used to overcome these challenges included developing
terion .Sixty three (64%) were male. The median age of patients was trust and rapport with participants, providing support through multi-
9 years. The most common cancers were bone and soft tissue sarco- ple routes of dissemination, and using data management applications
mas 35 (32%) and hematolymphoid 30 (29 %). Majority of psycho social for automation and project management. Post-assessments have indi-
problems reported by children were emotional 68 (69 %) and physical cated that those who participated in the 6-week intervention plus two
65 (66%).Few problems reported by children were practical 12(12%) PCS sessions and one dietitian session reported positive changes in
and family 10 (10%). Among all referred caregivers (83) majority were eating and exercise behaviors.
both the parents in which 36 were mothers (43%) and 37 were fathers Conclusions: Results suggest that the COVID-19 pandemic has
(45%). Most caregivers reported emotional problems 61 (73%) fol- impacted participant lifestyle behaviors, recruitment, and engagement.
lowed by practical problems 27(33%) and family problem (24%) during Nevertheless, preliminary findings suggest that PCS and their care-
their children’s treatment. givers are making positive health behavior change. COVID-19 should
Conclusions: Pediatric patients and caregivers undergo mostly emo- be considered at multiple timepoints throughout the analysis due to its
tional, physical and practical problems. Findings support the need for unique effects on trial implementation and participant experiences.
tailor-made psychological intervention.

EP585 / #1532 PREDICTION OF POST-TRAUMATIC


EP584 / #112 IMPROVING HEALTH BEHAVIORS OF SYMPTOMS 1 YEAR AFTER DIAGNOSIS AMONG CHILDREN
PEDIATRIC CANCER SURVIVORS WITH OBESITY DURING THE WITH CANCER
COVID-19 PANDEMIC: BARRIERS, OPPORTUNITIES, AND
MEASUREMENT CONSIDERATIONS Malcolm Sutherland-Foggio1 , Anna Olsavsky1 , Dana Garcia1 , Bruce
Compas2 , Kathryn Vannatta1 , Cynthia Gerhardt1
Marilyn Stern1 , Heewon Gray2 , Sandra Soca Lozano3 , Acadia Buro4 , 1 The Abigail Wexner Research Institute at Nationwide Children’s Hospi-
Rachel Sauls5 tal, Center For Biobehavioral Health, Columbus, United States of America;
1 University of South Florida, Child And Family Studies, Tampa, United States 2 Vanderbilt Kennedy Center for Research, Human Development, Nashville,
of America; 2 University of South Florida, College Of Public Health, Tampa, United States of America
United States of America; 3 University of South Florida, College Of Edu-
cation, Tampa, United States of America; 4 Moffitt Cancer Center, 4117 E Background and Aims: Children with cancer are at heightened risk
Fowler Ave, Tampa, United States of America; 5 University of South Florida, of stress and distress during treatment. Multiple factors may predict
Department Of Psychology, Tampa, United States of America long-term post-traumatic stress symptoms (PTSS) in children, such as
demographic and family factors, as well as survivor’s stress. However,
Background and Aims: Prevalence of overweight/obesity in pediatric limited research has examined factors involved in PTSS during treat-
cancer survivors (PCS) is 40-50%, yet few programs target this high- ment or early survivorship. Therefore, we examined the association
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S418 of S635 ABSTRACTS

between demographic factors, cancer-related stress, and family com- instruments and procedures; and 3) intervention feasibility and accept-
munication near diagnosis and survivors’ PTSS approximately 1 year ability. Clinical outcomes were collected at baseline, post-treatment
later. (12 weeks), and follow-up (6 months).
Methods: Data were from a longitudinal study of families (N=100) Results: The following progression criteria were met: sample size
of children newly diagnosed with cancer; mothers and children was exceeded within 5 months, with 11.0% enrolled of total popu-
(Mage =13.44; 50% Female; 90% White) completed surveys near lation invited, study dropout rate was 24.0%, intervention dropout
diagnosis (T1) and at 1 year (T2). Mothers reported on sociode- was 23.6%, missing data remained at ≤10% per measure, and no sub-
mographic background and attitudes about cancer communication stantial negative consequences related to participation were reported.
with their child. Children self-reported on cancer-related stress and Intervention adherence was slightly lower than progression criteria
PTSS. Hierarchical regression examined T1 demographic factors, PTSS, (47.9%).
cancer-related stress, and attitudes regarding cancer communication Conclusions: Findings suggest an internet-administered, guided, LICBT
as predictors of T2 PTSS. self-help intervention may represent a feasible and acceptable solution
Results: At T1 and T2, 24.3% and 25.7% of children, respectively, met for parents of children treated for cancer. With minor study proto-
clinical cutoffs for PTSS. PTSS scores were not significantly associated col and intervention modifications, progression to a pilot randomized
with any demographic factors. The regression model predicting PTSS controlled trial (RCT) and subsequent superiority RCT is warranted.
at T2 was significant, F(5,94)=10.05, R2 =0.31, p<0.001. T1 cancer-
related stress (b=0.36, p=0.001) and mother attitudes preferring less
communication with their child about cancer (b=0.23, p=0.01) pre- EP587 / #1260 PARENTAL EXPERIENCES AND MENTAL
dicted higher levels of PTSS at T2, beyond the impact of T1 PTSS HEALTH CONCERNS OF PEDIATRIC ONCOLOGY CARE DURING
(b=0.16, p=0.13). COVID-19 PANDEMIC: A CROSS SECTIONAL STUDY
Conclusions: Children were more likely to exhibit increases in PTSS 1
year following diagnosis when they had greater cancer-related stress, Sangeetha Thomas, Victoria White, Nicholas Ryan, Linda Byrne
as well as mothers who were less inclined to share information with Deakin Univeristy, Psychology, Burwood Hwy, Australia
them about cancer. Providers should encouragement more open atti-
tudes about cancer communication with children to reduce PTSS Background and Aims: Onset of the COVID-19 pandemic posed high
over the first year of treatment. Future research should include more demands on health care systems and introduced modifications to
diverse samples, especially fathers, and examine other predictors of the delivery of care. In Australia, changes include shift to telehealth
PTSS. appointments, restricting procedures, and limiting the number of peo-
ple attending appointments. There is limited evidence on how these
changes impacted parents and children during oncology care. This
EP586 / #549 INTERNET-ADMINISTERED, LOW-INTENSITY study aimed to examine parental experiences during child’s oncology
COGNITIVE BEHAVIORAL THERAPY FOR PARENTS OF care amidst pandemic in Australia.
CHILDREN TREATED FOR CANCER: A FEASIBILITY TRIAL Methods: Cross-sectional online survey conducted with parents of
(ENGAGE) children diagnosed with cancer in the past 5 years when aged between
0-12 years. Participants completed the DASS-21, 17 items assessing
Ella Thiblin, Joanne Woodford, Christina Reuther, Johan Lundgren, the impact of COVID-19 on the child’s medical care, and hospital care
Nina Lutvica, Louise Von Essen experience.
Uppsala universitet, Women’s And Children’s Health, Uppsala, Sweden Results: Forty parents participated (Response rate 48%; 98% mothers).
Children were under different phases of treatment (25%-active treat-
Background and Aims: A sub-group of parents of children treated ment, 30%-maintenance therapy, 42.5%- follow-up). DASS-21 scores
for cancer report mental health difficulties, such as depression and indicated on average parents experience mild depression (M=13.3,
anxiety. There is a lack of evidence-based psychological interventions SD=11.0), and moderate anxiety (M=11.0, SD=10.2)/stress (M=19.6,
for parents, with psychological support needs unmet. An internet- SD=10.2). Majority (88%) maintained face-to-face appointments and
administered, guided, low-intensity cognitive behavioural therapy- felt safe at appointments (M=8.2, SD=2.7). However, 30% reported
based (LICBT) self-help intervention may provide a solution. The aim delays booking appointments, 15% were not able to ask all their ques-
of this study was to examine methodological, procedural, and clinical tions about COVID-19, 13% did not feel safe from COVID-19 during
uncertainties of such and intervention, and related study procedures, hospital visits and 20% were moderately to highly anxious about possi-
to prepare for the design and conduct of a future pilot RCT and ble impact of COVID-19 on child’s health. The majority (53%) reported
subsequent superiority RCT. that they did not receive information about the risk of COVID-19
Methods: The feasibility and acceptability of the intervention and virus on their children. Lack of information was associated with feel-
study procedures was examined using a single-arm feasibility trial ing unsafe at appointments (r= -.445, p<0.05), feeling anxious about
(ENGAGE). Primary objectives examined: 1) estimates of recruitment the impact of COVID on their child’s heath (r=.443, p<0.01) and higher
and retention rates; 2) feasibility and acceptability of data collection scores on DASS-Anxiety (r=.325, p<0.05).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S419 of S635

Conclusions: While many parents experiences of their child’s health EP589 / #889 VOLUNTEERING AT PEDIATRIC
care during the pandemic were positive, there was a need for greater ONCOHEMATOLOGY: AN ANALYSIS OF THE NARRATIVES
information and support in asking questions. Understanding family CONCERNING THE MOTIVATION AND PSYCHOLOGICAL
experiences of pediatric oncology care during pandemic is important WELL-BEING DURING THE COVID-19 EMERGENCY
for developing better health care practices.
Marta Tremolada1,2 , Livia Taverna3 , Sabrina Bonichini1 , Roberta Maria
Incardona1 , Alessandra Biffi2
EP588 / #884 PARENTAL BURNOUT IN PARENTS OF 1 University of Padua, Department Of Development And Social Psychology,
CHILDREN UNDER THERAPY FOR LEUKEMIA: FACTORIAL Padova, Italy; 2 University of Padua, Pediatric Hematology, Oncology And
ANALYSIS OF THE QUESTIONNAIRE AND POSSIBLE Stem Cell Transplant Center, Department Of Woman’s And Child’s Health,
ASSOCIATIONS WITH BEHAVIORAL SYMPTOMS IN THEIR Padova, Italy; 3 Free University of Bozen-Bolzano, Faculty Of Education,
CHILDREN Brixen, Italy

Marta Tremolada1,2 , Sabrina Bonichini1 , Livia Taverna3 , Giulia Background and Aims: The phenomenon of volunteering is very
Marangon1 , Roberta Maria Incardona1 , Alessandra Biffi2 widespread in the world and in Italy with ever-growing numbers (6.63
1 University of Padua, Department Of Development And Social Psychology, million in 2013). The possibility of volunteers to continue their activ-
Padova, Italy; 2 University of Padua, Pediatric Hematology, Oncology And ities in the hospitals have blocked during the Covid19 pandemic. The
Stem Cell Transplant Center, Department Of Woman’s And Child’s Health, aims of this study are to assess the psychological well-being and
Padova, Italy; 3 Free University of Bozen-Bolzano, Faculty Of Education, motivation associated with volunteering in pediatric oncohematology
Brixen, Italy during the March 2020 lockdown.
Methods: Participants were twenty-five volunteers (8 males and 17
Background and Aims: Parental burnout is a global exhaustion of females) at Pediatric Oncoematology Clinic, University of Padua. They
the role of parent, an emotional detachment from children and a loss had an average age of 54.52 years (SD=15.59; range: 26-73), 12 with
of self-perception as a good parent (Roskam & Mikolajczak, 2020). a current job and 13 not working. A socio-demographic questionnaire
Parental chronic oppressive stress affects not only the parent but and a semi-structured interview consisting of twenty-five questions
also the child. This study aims to identify the possible dimensions of were administered online using video calls with Skype. After obtaining
Parental burnout in parents of children with leukemia and its possible the participant’s consent, the entire interviews were audio-recorded
associations with emotional and behavioral symptoms in their children. and fully transcribed.
Methods: Participants who signed the informed consent were prin- Results: A word cloud analysis of the interviews adopting software
cipally mothers (N = 47; 92.2%) while there were only 4 fathers QRS N-vivo identified as the main volunteers’ characteristics the more
(7.8%) recruited at the Pediatric Onco-Hematology Clinic, Univer- recurrent words belonging to Empathy (N=21), Relational skills and
sity of Padua. Their average age was 40.5 years (SD = 7.15; range Discretion / Flexibility (N = 19). The main reasons for the volunteers’
22-54) and they mostly were married or cohabitants (91.1%). Their choice were Expression of values (N=18), Growth (N=16) and Social
children, aged between 3 and 10 years (average age = 6.5 years; SD = (N=10). A Wilcoxon test (Z= -4.207; p= 0.0001) found that the vol-
2.07), underwent the first month of hospitalization for leukemia. They unteers’ experience was significantly reported as Good (Mean=7.76)
were 26 males and 27 females, 83% were Caucasian and 17% non- than Bad (Mean=2.60). Most of the volunteers reported the possibil-
Caucasian. They filled in questionnaires on their Parental Burnout and ity to do online activities with children during lockdown (60%), even if
Child Behavior Check List about their children. the majority perceived this option as difficult to do (90.47%). They used
Results: A Varimax factor analysis found that the best model explained more negative words (Mean = 2.36) than positive ones (Mean = 1.20)
44.4% of variance with two identified factors: “Parental inefficacy per- when speaking about isolation (Z= -1.94; p= 0.05).
ception” and “Parental Exhaustion and Emotional Distance”, with their Conclusions: Volunteers reported more adoption of positive words
Cronbach alphas greater than 0.75. A series of Spearman’s correlations during their activities with the children in the hospitals and they iden-
were run, and significative associations were found between parental tified empathy as the most important characteristic of the volunteer
burnout and children’s internalizing (rho = 0.46; p = 0.01) and exter- profile. Online activities with children during lockdown were consid-
nalizing (rho = 0.57; p < 0.001) symptoms but limited to schooling age ered difficult and they adopted more negative words related to the
children. isolation.
Conclusions: Some aspects of Parental burnout such as exhaustion and
emotional distance could be related to children’s negative symptoms.
The onco-hematological diagnosis is considered as familiar disease and EP590 / #891 SIBLINGS IN PEDIATRIC ONCOHEMATOLOGY
for this reason the psychological interventions should be focused also COMPARED WITH HEALTHY PEERS: PSYCHO-SOCIAL
on parental well-being, reducing their stress, and ameliorating their WELL-BEING AND ASSOCIATED FACTORS
parenting role.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S420 of S635 ABSTRACTS

Marta Tremolada1,2 , Sabrina Bonichini2 , Livia Taverna3 , Roberta Maria Silvana Rosado18 , Mariela Villegas2 , Mariela Pereda9 , Mariela Ríos19 ,
Incardona2 , Alessandra Biffi1 María Benites20 , Dalila Contreras21 , Oscar Velásquez22 , Teresa
1 University of Padua, Pediatric Hematology, Oncology And Stem Cell Trans- Mendez23 , Elisa Perina24 , Nuria Rossell25
plant Center, Department Of Woman’s And Child’s Health, Padova, Italy; 1 Pan American Health Organization, Non-communicable Disease, Wash-
2 University of Padua, Department Of Development And Social Psychol- ington D.C, United States of America; 2 Pontificia Universidad Católica
ogy, Padova, Italy; 3 Free University of Bozen-Bolzano, Faculty Of Education, del Perú, Grupo De Investigación Psicología Y Salud: Entornos Saludables,
Brixen, Italy Lima, Peru; 3 Asociación Nacional de Psicooncología, Anppe, Perú, Peru;
4 Pan American Health Organization, Nmh, Lima, Peru; 5 Instituto Nacional
Background and Aims: Siblings in pediatric oncohematology could de Enfermedades Neoplásicas, Departamento De Pediatría, Lima, Peru;
have negative sequelae such as somatic complaints (Lane & Mason, 6 Ministry of Health of Peru, Direction Of Strategic Interventions, Lima, Peru;

2014) and psycho-social problems (Wallin et al., 2016), but also positive 7 Instituto Nacional de Salud del Niño San Borja, Servicio De Psicología,
social and personal growth (Sloper, 2000). This study aims to: 1. screen Lima, Peru; 8 Instituto Nacional de Enfermedades Neoplásicas, Equipo Fun-
possible emotional and psycho-social difficulties in siblings; 2. study the cional De Salud Mental Oncológica, Lima, Peru; 9 Club de Leones, Distrito
relationship between siblings along patient’s and sibling’s perceptions. H1, Perú, Peru; 10 Instituto Regional de Enfermedades Neoplásicas del Sur,
Methods: Participants were 21 siblings with a mean age of 9.61 years Servicio Social, Arequipa, Peru; 11 Hospital Antonio Lorena, Servicio De
(SD= 2.28; range: 5.8-13), 62% females and 76% firstborn recruited at Oncología Pediátrica, Cusco, Peru; 12 Hospital de Pediatría Especializada y
the Onco-Hematology Clinic, University of Padua. Pediatric patients Cáncer Infantil VIDAWASI, Área De Pedagogía Hospitalaria, Cusco, Peru;
were 21 with a mean age of 7.7 (SD = 3.35; 1.06-15), 86% with 13 Asociación Cultural Peruana Chasqui, Área De Psicología, La Libertad,
leukemias. After the signature of informed consent by parents, several Peru; 14 Hospital Nacional Carlos Seguín Escobedo, Servicio De Oncohema-
self and proxy questionnaires were given to patients and their siblings tología Pediátrica, Arequipa, Peru; 15 Instituto Nacional de Salud del Niño
during the first four months from the diagnosis., i.e. Child Behavior Breña, Servicio De Psicología, Lima, Peru; 16 Asociación Peruana Alemana
Check List (CBCL), Strengths Difficulties Questionnaire (SDQ) and Sib- del Cáncer Infantil (PACAI), Área De Psicología, Cusco, Peru; 17 Instituto

lings Inventory of Behavior (SIB). The same questionnaires were filled Nacional de Salud del Niño San Borja, Servicio De Hematología, Lima,
in also by matched siblings of healthy peers. Peru; 18 Instituto Regional de Enfermedades Neoplásicas del Sur, Servicio
Results: A Wilcoxon test showed no significative differences between De Psicología, Arequipa, Peru; 19 Hospital De Alta Complejidad Virgen de la
clinical and control group along CBCL scores, even if 50% of sib- Puerta, Servicio De Pediatría, La Libertad, Peru; 20 Hospital Regional Lam-
lings in the clinic group showed clinical levels in the internalizing bayeque, Servicio De Psicología, Lambayeque, Peru; 21 Hospital Nacional
symptomatology. Siblings’ age was positively associated with emotive Edgardo Rebagliati Martins, Servicio De Psicología, Lima, Peru; 22 Hospital

(rho=0.6; p=0.009) and conduct problems (rho=0.61, p=0.007) and Nacional Guillermo Almenara Irigoyen, Servicio De Psicología, Lima, Peru;
their birth order was negatively associated with social problems (rho=- 23 Fundación Natali Dafne Flexer, Área De Apoyo Psicológico, Buenos Aires,

0.51, p=0.03). Siblings’ age was negatively associated with Empathy Argentina; 24 Sociedad Latinoamericana de Oncología Pediátrica, Comité
(rho=-0.55; p=0.01) and their birth order was negatively associated Psicosocial, México, Mexico; 25 University of Amsterdam, Amsterdam Insti-
with the total mean of feelings in the fraternal relationship (rho=-0.47; tute For Social Science Research, Amsterdam, Netherlands
p=0.05). A Wilcoxon test found that Empathy (Z= -1.93; p= .05) was
distributed differently between patients (Mean ranks = 8.55) and their Background and Aims: Since 2019, the WHO Global Initiative for
siblings (Mean ranks = 6.5). Childhood Cancer (GICC) has been implemented in Peru. As a for-
Conclusions: No differences were found in the behavioral symptoms’ mal collaboration between the Pan American Health Organization,
frequency comparing clinical and control groups. Older and first birth the Ministry of Health, Childhood-Cancer-International, and La Roche-
siblings reported more emotive and social problems and have lower Posay, a nation-wide project was initiated to improve the psychosocial
empathy. care (PC) of children with cancer in Peru. The aim of this study was to
conduct a national situational assessment, determine the level of com-
pliance of the international PC standards and finally, to develop and
EP591 / #902 A PARTICIPATIVE PROCESS TO ADAPT AND implement local standards, including activities and tools to implement
IMPLEMENT PSYCHOSOCIAL STANDARDS OF CARE IN them.
CHILDHOOD CANCER IN PERU Methods: A pilot mix-method study was conducted for two years
involving several stakeholders, including project managers, local
Viviana Trigoso1,2,3 , Claudia Pascual4 , Liliana Vasquez1 , Essy experts, regional partners and pediatric oncology providers in Peru.
Maradiegue5,6 , Pilar Jimenez7 , Hernan Bernedo8 , Melisa Sierralta3 , A psychosocial GICC committee was formed to design activities and
Lourdes Ruda2,3 , Yurfa Salazar8 , Cecilia Ugaz5 , Roxana Morales5 , tools to implement the PC standards in Peruvian institutions. Six
Daniela Vargas3 , Sara Alvarez9 , Carlota Almiron10 , Walter standards were proposed: psychosocial assessment, psychoeducation
Atausinchi11 , Claudia Bernales12 , Carla Castro3 , Anita Campos13 , and support, mental health care, reintegration and survival resources,
Giovanna Galarza8 , Edgard García14 , Marisa López15 , Karina competences and self-care in health professionals, and transversal
Madueño16 , Alicia Mafaldo17 , Yanina Salazar9 , Oscar Plasencia7 , approaches.
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ABSTRACTS S421 of S635

Results: Between Jul/19 and Mar/22, X professionals from 15 Peru- dards and conduct regional discussions about four questions related to
vian institutions and regional collaborators were involved in the pilot each PC standard, entitled: “what we know”, “what we propose”, “what
study. After a national PC assessment, we determined that interna- works”, and “how we can apply it”.
tional psychosocial standards were poorly provided at most Peruvian Results: Fifteen professionals from six LAC attended 6 online meet-
centers. The development of the conceptual framework of the PC local ings and revisited previous educational materials about PC standards.
standards involved the participation of national and international pro- Six (6) technical fact sheets about psychosocial standards of care
fessionals in a think-tank meeting, 98 online meetings and a webinar. were developed with relevant information to improve the quality of
Four technical products (national psychosocial assessment tool; PC care in LAC childhood cancer institutions. An operative plan has been
guide for parents, PC screening tool, and 4 educational documents), designed to validate the strategies to implement each standard at
and one national meeting were developed to improve PC in childhood different levels according to short, medium, and long-term goals and
cancer in Peru. available resources.
Conclusions: In the present study, we propose a conceptual framework Conclusions: Implementing psychosocial standards contribute not only
defining relevant phases to develop and implement PC local standards to LAC children and adolescents with cancer but also may allow other
in pediatric cancer, through transdisciplinary collaboration. The ongo- low and middle-income countries to replicate this process and improve
ing work of professionals throughout the country has been relevant to PC.
develop materials to implement the PC standards in Peru.

EP593 / #1410 LONG-TERM FUNCTIONING OF CHILDHOOD


EP592 / #1755 STANDARDS FOR THE PSYCHOSOCIAL CARE CANCER SURVIVORS AND THEIR PARENTS: LONGITUDINAL
OF CHILDREN AND ADOLESCENTS WITH CANCER: AN RELATIONS WITH PARENTAL INCOMPETENCE AND
ADAPTED PROPOSAL IN LATIN AMERICA COUNTRIES PARENTING DIMENSIONS

Viviana Trigoso1,2,3 , Liliana Vasquez1 , Soad Fuentes-Alabi De Elise Van Laere1 , Koen Raymaekers1 , Sofie Prikken1,2 , Jurgen
Aparicio1 , Teresa Mendez4 , Elisa Perina5 , Eugenia Ahumada6 , Lemiere1,2 , Janne Vanderhaegen1 , Trui Vercruysse2 , Anne
Alessandra Motta7 , Raquel Molina8 , Eduardo Velasco9 , Mariela Uyttebroeck2,3 , Koen Luyckx1
Villegas2 1 KU Leuven, Faculty Of Psychology And Educational Sciences, Leu-
1 Pan American Health Organization, Non-communicable Diseases, Wash- ven, Belgium; 2 UZ Leuven, Pediatric Hemato-oncology, Leuven, Belgium;
ington D.C, United States of America; 2 Pontificia Universidad Católica 3 KULeuven, Department Of Oncology, Leuven, Belgium

del Perú, Grupo De Investigación Psicología Y Salud: Entornos Saludables,


Lima, Peru; 3 Asociación Nacional de Psicooncología, Anppe, Perú, Peru; Background and Aims: Most childhood cancer survivors tend to
4 Fundación Natali Dafne Flexer, Área De Apoyo Psicológico, Buenos Aires, be resilient in the long term, with benefit finding being frequently
Argentina; 5 Sociedad Latinoamericana de Oncología Pediátrica, Comité Psi- reported. However, some survivors suffer from depressive symptoms
cosocial, México, Mexico; 6 Programa Nacional de Cáncer Infantil de Chile, and cancer-related worries as well. As cancer is considered a ‘family
Comisión De Psicólogos, Chile, Chile; 7 Universidade Federal do Espirito disease’, investigating the role of parental functioning and parent-
Santo, Ufes, Brazil, Brazil; 8 Instituto Nacional del Cáncer, Paraguay, Asun- ing may provide insight into why some youth experience growth
ción, Paraguay; 9 Instituto Oncológico del Oriente Boliviano, Servicio De and others struggle. The current study examined the directionality
Psicología, Santa Cruz de la Sierra, Bolivia of effects among parental incompetence, parenting dimensions (i.e.,
responsiveness, psychological control, and overprotection), and sur-
Background and Aims: The World Health Organization Global Ini- vivor functioning (i.e., depressive symptoms, cancer-related worries,
tiative for Childhood Cancer (GICC) aims to improve outcomes for and benefit finding). Additionally, we investigated if parenting mediates
children with cancer around the world guided by the CureAll frame- the effect of parental incompetence on survivor functioning.
work. The development of the conceptual framework of the standards Methods: Our three-wave longitudinal questionnaire study included
of psychosocial care (PC) began in Peru in 2019. The proposal is aligned 125 Dutch-speaking survivors (14-25 years at baseline) who com-
with the CureAll objectives, in specific the first pillar for centers of pleted their treatment, 114 mothers and 82 fathers. Survivors
excellence and care of children and adolescents diagnosed with cancer, reported (SR) about mothers’ and fathers’ parenting and their own
including quality of care. Expanding and implementing PC standards in functioning. Mothers (MR) and fathers (FR) reported about their par-
Latin American Countries (LAC) is relevant for reducing psychosocial enting and sense of incompetence. Cross-lagged panel models were
risks and promoting coping strategies for patients and their families. estimated for each informants’ perspective on parenting separately.
Methods: A Latin American GICC psychosocial working group was Results: Primarily unidirectional relations were found, i.e., from
established focused on improving PC for children and adolescents parental incompetence to parenting, and from parenting to survivor
with cancer through the implementation of standards of care in LAC. functioning. Different relations were obtained for each informant.
Between Jun/21 and Dec/21, monthly online meetings were scheduled First, parental incompetence positively predicted maladaptive parent-
to review background documents and materials related to the stan- ing SR, MR and FR. Second, maternal responsiveness SR positively
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S422 of S635 ABSTRACTS

predicted benefit finding SR and negatively predicted depressive symp- mainly unidirectional effects occurred. Posttraumatic stress symp-
toms SR, whereas responsiveness MR positively predicted cancer- toms negatively predicted identity synthesis and positively predicted
related worries SR. Overprotection MR positively predicted depressive identity confusion over time, whereas the reverse pattern of associa-
symptoms SR. Finally, one reversed pathway emerged, responsive- tions was found for benefit finding. Several correlated changes were
ness SR negatively predicted parental incompetence. Also, correlated found linking identity formation and psychosocial functioning as well,
changes indicated co-development among these variables. There was attesting to co-development.
no evidence of mediation, as there were no significant pathways linking Conclusions: The current study focused on clarifying the longitudinal
parental incompetence to parenting which, in turn, predicted survivor associations linking identity synthesis and confusion to both generic
functioning. and illness-specific functioning in youth who survived childhood can-
Conclusions: Results support parent-driven processes in survivors’ cer. Several significant pathways emerged that can substantially inform
long-term functioning and a need to consider parents’ sense of incom- both clinical practice and future research.
petence in research and clinical practice. Additionally, our findings
stress considering multiple perspectives when investigating family
dynamics. EP595 / #313 RISK OF ALTERATIONS IN
NEURODEVELOPMENT IN INFANTS AND PRESCHOOLERS
WITH CANCER IN A REFERENCE HOSPITAL IN MEXICO
EP594 / #721 IDENTITY FORMATION AND GENERIC AND
ILLNESS-SPECIFIC FUNCTIONING IN ADOLESCENT AND Liliana Velasco-Hidalgo1 , Marta Zapata-Tarrés2 , Alejandro
EMERGING ADULT CANCER SURVIVORS: A LONGITUDINAL González-Garay3 , Antonio Rizzoli-Córdoba4 , Alda-Daniela
INVESTIGATION INTO DIRECTIONALITY OF EFFECTS García-Guzmán1 , Ana Gabriela Ortiz-Razo1 , Evereth Alejandra
Olmedo-Jiménez1 , Rocío Cárdenas-Cardós1
Janne Vanderhaegen1 , Sofie Prikken1,2 , Elise Van Laere1 , Jurgen 1 National Institute of Pediatrics, Pediatric Oncology, Mexico City, Mexico;
Lemiere1,2 , Laurence Claes1 , Philip Moons3 , Anne Uyttebroeck2,4 , 2 Fundación IMSS, Reserach Coordination, Mexico City, Mexico; 3 National
Sandra Jacobs2,4 , Koen Luyckx1 Institute of Pediatrics, Department Of Methodology Research, Mexico,
1 KU Leuven, Faculty Of Psychology And Educational Sciences, Leuven, Mexico; 4 Hospital Infantil de Mexico Federico Gómez, Development And
Belgium; 2 UZ Leuven, Pediatric Hemato-oncology, Leuven, Belgium; 3 KU Behavior, Mexico City, Mexico
Leuven, Department Of Public Health And Primary Care, Leuven, Belgium;
4 KULeuven, Department Of Oncology, Leuven, Belgium Background and Aims: Systemic and intrathecal chemotherapy can
cause cognitive impairments in the form of lowered concentration,
Background and Aims: Youth who survived childhood cancer gener- attention, and memory power in cancer survivors. However, there is no
ally adjust well psychologically and achieve important developmental information on the effect of cancer treatment on neurodevelopment
milestones through life similar to their peers. Nevertheless, some sur- in cancer patients under five years of age. Therefore, the aim was to
vivors are at greater risk for developing psychological and physical evaluate neurodevelopment in cancer patients under five years of age
problems. To shed light on these individual differences, identity for- through the Infant Development Assessment test.
mation and its interplay with psychosocial functioning need to be Methods: A cross-sectional study was carried out from February 2018
investigated. The aim of the present study was to examine the longitu- to March 2019. Patients with cancer diagnoses outside the central
dinal associations linking personal identity formation and generic and nervous system in any phase of cancer treatment were included.
illness-specific functioning in adolescent and emerging adult childhood Results: A total of 45 patients were included. Regarding the areas of
cancer survivors using three-wave data over a two-year period. development, the following was the result: In the gross motor area, 31
Methods: Dutch-speaking survivors (14 - 25 years) who were treated (68.8%) patients were green, while 35 patients (77.7%) were in the fine
at the pediatric oncology department of the University Hospitals motor area. In the fine motor area, 28% of patients with retinoblastoma
Leuven (Belgium) completed self-report questionnaires addressing and 23% of patients with leukemias and lymphomas were found in red
identity formation, depressive symptoms, life satisfaction, physical (delay) compared to 0% of patients with solid tumors (P = 0.025).The
functioning, cancer-related worries, posttraumatic stress symptoms, final result of the test found that 19 (42.2%) patients were in the green
and benefit finding. A total of 125 survivors participated at baseline, with normal neurodevelopment, 7 (15.5%) in the yellow with a lag in
100 survivors at T2, and 93 survivors at T3. Directionality of effects neurodevelopment, and 19 (42.2%) in the red with a risk of delay in
was examined using cross-lagged structural equation modeling. the development. Of the patients with developmental delay, 52% were
Results: Regarding generic functioning, bidirectional effects occurred. from the leukemia and lymphoma group, 71% from the retinoblastoma
Life satisfaction positively predicted identity synthesis and both life group, and 23% from the solid tumor group (P = 0.06).
satisfaction and good physical functioning negatively predicted iden- Conclusions: The risk of delay and lag in neurodevelopment is frequent
tity confusion over time. Identity synthesis, in turn, positively predicted in cancer patients under five years of age undergoing treatment. How-
life satisfaction and identity confusion negatively predicted good ever, more studies are required to evaluate the effect of treatment in
physical functioning over time. Regarding illness-specific functioning, this group of patients since various factors may influence them.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S423 of S635

EP596 / #696 EXPECTATIONS OF CHILDREN WITH CANCER, EP597 / #1656 RESULTS OF FEASIBILITY - WHAT DOES IT
NEUROMUSCULAR DISEASES OR ANXIETY, AND TEACHERS TAKE TO IMPLEMENT STANDARDIZED PSYCHOSOCIAL TOOLS:
FOR TELEPRESENCE ROBOTS AS A TOOL TO REDUCE “MEIN LOGBUCH – ICH KENNE MICH AUS!” („MY LOGBOOK“)
ABSENCE IN EDUCATION
Liesa J. Weiler-Wichtl1,2 , Verena Fohn-Erhold1 , Alina Kollmann3 ,
Mette Weibel1,2,3 , Sofie Skoubo1,4 , Charlotte Handberg4,5 , Lykke Carina Schneider4 , Karin Dieckmann5 , Verena Rosenmayr6 , Katja
Bertel6,7 , Nonni Steinrud1 , Kjeld Schmiegelow1,2 , Inger Hallström3 Roithner6 , Andreas Wiener7 , Barbara Grießmaier8 , Iris Lein-Köhler9 ,
1 Rigshospitalet, Department Of Paediatrics And Adolescent Medicine, Maximilian Hopfgartner1 , Kerstin Krottendorfer1 , Ulrike Leiss1
Copenhagen, Denmark; 2 University of Copenhagen, Faculty Of Health Sci- 1 Medical University of Vienna, Comprehensive Center For Pedi-
ences, Copenhagen, Denmark; 3 Lund University., Department Of Health atrics/department For Pediatrics And Adolescent Medicine, Vienna,
Sciences, Faculty Of Medicine, Lund, Sweden; 4 National Rehabilitation Austria; 2 PSAPOH, On Behalf Of Logbuch Expert Group, Wien, Austria;
Center for Neuromuscular Diseases, National Rehabilitation Center For 3 Kepleruniversitätsklinikum, Department Of Paediatric Oncology And
Neuromuscular Diseases, Aarhus C, Denmark; 5 Aarhus University, Depart- Hematology, Linz, Austria; 4 CCI-Europe, Managing Director, Vienna, Aus-
ment Of Public Health, Faculty Of Health, Aarhus C, Denmark; 6 Aalborg tria; 5 Medical University of Vienna, Department For Radiology, Vienna,
Centre, Aalborg Centre For Problem Based Learning In Engineering Sci- Austria; 6 General Hospital of Vienna, Department For Psychology And
ence And Sustainability Under The Auspices Of Unesco, Aalborg, Denmark; Psychotherapy, Vienna, Austria; 7 Westdeutsches Protonenzentrum, Univer-
7 Aalborg University, The Technical Faculty For It And Design, Aalborg Eas, sitätsklinikum Essen, Essen, Germany; 8 Universitätsklinik Frankfurt, Klinik
Denmark Für Kinder- Und Jugendmedizin, Frankfurt, Germany; 9 Universitätsklinikum
des Saarlandes, Pädiatrische Onkologie/hämatologie, Homburg, Germany
Background and Aims: School absence negatively influences chil-
dren’s academic advancement and psychosocial wellbeing. This study Background and Aims: Psychosocial care and research are well-
explores expectations for telepresence robots as a tool to reduce documented essential components in pediatric oncology care. Hence,
absence in education. guidelines and standards offer evidence-based framework for qual-
Methods: A qualitative semi-structured interview study. Using con- ity assurance. Nevertheless, actual care is quite heterogenous and
venience sampling, we interviewed 11 children aged 8–17 years with reveals a care gap. Highly complex, system-wide interventions repre-
cancer (n=4), neuromuscular diseases (n=3) or anxiety (n=4) who had sent considerable challenges for operationalization of relevant factors
a high level of school absence (more than 15 days’ absence in a school and evaluation of psychosocial processes compared to single interven-
year), and who had recently encountered a telepresence robot; and tions. To bridge the gap from need to implementation literature reveals
eight of their teachers. A thematical analysis and a deductive approach specific demands and research methods as well as successful imple-
based on the theory of Technological frames were used. mentation strategies. Quality improvement (QI) is an iterative process
Results: The children’s and teachers’ expectations of how telepres- designed to make controlled changes within the health care delivery
ence robots could support them in reducing their school absence were system to provide patients with high-quality care.
identified and structured in three categories and five main themes: Methods: PDSA cycles were applied in all steps of a multilevel, interdis-
1) Nature of technology: a) Learning, b) Sociality, c) Additional sup- ciplinary conceptualization and implementation. We present a protocol
portive resources; 2) Technology strategy: a) Flexible school day; 3) of a mixed methods observational study to gain more insight on
Technology in use: a) New workflows. Children and their teachers had sustainability, implementation and its determinants. Therefore we
positive expectations regarding telepresence robots as being a flexi- observed communication levels (face-to-face discussions, telephone
ble tool to support children with high absence in re-integrating in the conferences, e-mail exchange) combined with regular online Surveys
school environment, both socially and academically. However, in rela- (D-A-CH region). Participants are associated HCP in regular research
tion to inclusion, the expectations across diagnostic groups varied from meetings by the local expert-research group (N=11), expert groups
an opportunity of creating friendships to maintaining existing friend- with multidisciplinary HCP and stakeholders (N=28) for the develop-
ships. Teachers expected that the telepresence robot implementation ment and expert validation of 18 theme booklets, monitoring group
requires additional supportive resources from them such as estab- (quality assurance group/PSAPOH), N=47 Experts in a delphi-survey
lishing blending learning situations, new workflows in education and and N=48 Study coordinators/Collaborators in the multicenter pilot
coordination between family and school. However, the teachers were phase.
willing to allocate the resources because they saw potential in the new Results: Implementation factor “Characteristics of the intervention”
technology as a tool to reduce the high level of absences. reached an overall consensus above 80% and include predominantly
Conclusions: The organization around telepresence robots in school design quality, cost and evidence strength/quality. However, “inner set-
settings is lacking because the implementation process is still ting”, despite acceptable implementation climate due to high response
in an explorative stage. It is a simple technology, but it requires (sites N=27), highlights major obstacles: structural framework, fund-
new workflows and structures in the organization and school ing, research culture, improvement on networks and communication,
environment. personnel resources and pandemic. Hence, we derived and realized
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S424 of S635 ABSTRACTS

expert trainings/training videos, manual, regular meetings (exchange, EP599 / #590 PERCEPTIONS OF PARENTING AMONG
mutual support), FAQs, and organizational assistance. CAREGIVERS AND SCHOOL-AGED SURVIVORS OF
Conclusions: Implementing standards demands the establishment of RETINOBLASTOMA
research & care networks, concrete methods/tools for good scientific
and clinical practice, adaptation of structural framework conditions of Melanie Morse1 , Ibrahim Qaddoumi2 , Sean Phipps1 , Rachel Brennan3 ,
the health care system. Matthew Wilson4 , Carlos Rodriguez-Galindo5 , Kendra Parris1 , Kristin
Goode1 , Victoria Willard6
1 St. Jude Children’s Research Hospital, Psychology, Memphis, United
EP598 / #127 SOCIAL PARTICIPATION AMONG SURVIVORS States of America; 2 St. Jude Children’s Research Hospital, Global Pediatric
OF PEDIATRIC BRAIN TUMORS AS MEASURED THROUGH A Medicine, Memphis, United States of America; 3 St. Jude Chidlren’s Research
DAILY DIARY Hospital, Oncology, Memphis, United States of America; 4 University of Ten-
nessee Health Sciences Center, Hamilton Eye Institute, Memphis, United
Mollie Clark1 , Bethany Means1 , Kristin Goode1 , Niki Jurbergs1 , States of America; 5 St Jude Children’s Research Hospital, Global Pediatric
Heather Conklin1 , Amar Gajjar2 , Victoria Willard1 Medicine, Memphis, United States of America; 6 St Jude Children’s Research
1 St Jude Children’s Research Hospital, Psychology, Memphis, United States Hospital, Psychology, Memphis, United States of America
of America; 2 St Jude Research Hospital, Oncology And Pediatrics, Memphis,
United States of America Background and Aims: Retinoblastoma is the most common intraoc-
ular childhood cancer and is typically diagnosed in young children.
Background and Aims: Pediatric brain tumor survivors (PBTS) expe- With increasing numbers of survivors and improved medical outcomes,
rience difficulties with social functioning. Social participation - the long-term adjustment and psychosocial impacts merit exploration.
frequency of social interactions and the maintenance of relationships The current study examines both caregiver- and patient-reported
- is associated with emotional health. This paper aims to describe perceptions of parenting among school-aged survivors.
the social participation of PBTS to better understand their social Methods: Sixty-nine survivors of retinoblastoma underwent an assess-
functioning. ment of psychosocial functioning. Survivors (age=10.89±1.07 years;
Methods: PBTS (n=64, 54.7% female, 85.9% white, 40.6% medulloblas- 49.3% male; 56.5% unilateral disease, 68.1% treated with enucleation,
toma) were enrolled on a study assessing social functioning. Youth years since diagnosis=9.37±1.16) completed the Parental Bonding
were 10.58 (SD=1.37) years old and 5.24 (SD=2.46) years off-therapy. Instrument to assess perception of one’s parenting relationship with
Youth completed a daily diary which asked participants to record the one’s primary caregiver. Caregivers completed the Parenting Relation-
social interactions experienced daily for seven days across five cate- ship Questionnaire, a complementary assessment of a parent’s view of
gories: social victimization, physical victimization, exclusion, positive their relationship with their child across multiple domains.
social interactions, and participation in social activities. Parents pro- Results: Survivors’ report indicated that parenting was perceived as
vided information about their child’s overall social participation (e.g., high on the care dimension (M=30.32) and within the low range for
number of outside school peer interactions). overprotection (M=14.87). Perceptions of parental care and over-
Results: Three-quarters of participants (n=47) completed the Daily protection did not vary by disease laterality or enucleation status
Diary at least five of seven days. Participants most frequently identi- (all p>0.05). Caregiver report indicated that perceptions of parenting
fied positive social interactions, with an average of 11.38 interactions fell in the average range across assessed domains including attach-
endorsed (SD=7.9). Physical victimization (M=0.79, SD=2.23) was ment (M=50.25), communication (M=53.26), discipline practices
infrequently reported. Twenty-six survivors (40%) indicated experi- (M=49.19), involvement (M=52.53), parenting confidence (M=53.82),
encing one or more negative social interactions during the five-to- and relational frustration (M=45.47). Caregiver perspective of the
seven-day diary period. Of these 26, 53.8% of children engaged in parent-child relationship did not differ by disease laterality or enu-
no clubs or out-of-school activities. Frequencies of social interactions cleation status (all p>0.05). Child-reported perceptions of parental
indicate generally low participation for a large subset of survivors, caring were positively correlated with parental report of attachment
especially when including data from parents: 25% of survivors (n=16) (r(52)=.32, p=0.02) and involvement (r(52)=.33, p=0.02).
recorded less than ten interactions for the entire week and 46.9% Conclusions: Results indicate that survivors of retinoblastoma report
of parents (n=30) reported their child having less than one social an optimal parenting experience, with parents who are caring with-
interaction outside of school per week. out being overly protective. Similarly, parental perspective of the
Conclusions: The use of daily diaries revealed a wide range of parent-child relationship indicated parenting within the average range
social experiences for PBTS. There appears to be a notable across multiple domains. Disease- and treatment-related factors do
number of survivors who are engaging in little to no social par- not appear to impact this relationship. Overall, results suggest that sur-
ticipation during the average week. Lack of participation in vivors of retinoblastoma experience appropriate, responsive parenting
regular social interactions may contribute to PBTS difficulties from caregivers. Future research should continue to examine illness-
with social functioning; however, further work to assess this is related aspects of adjustment to characterize long-term functioning of
needed. survivors.
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ABSTRACTS S425 of S635

EP600 / #1286 QUALITY OF LIFE OF CHILDREN AND 1 Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital, Child Health

ADOLESCENTS WITH CANCER BEFORE AND DURING THE Department, Division Infectious And Tropical Disease, Bandung, Indone-
WAR IN UKRAINE sia; 2 Division of Hematology Oncology Dr. Hasan Sadikin Hospital Ban-
dung, Child Health Department, Bandung, Indonesia; 3 Universitas Padjad-
Olesya Horlenko1 , Liudmyla Baletska2 , Olga Pushkarenko1 , Julia jaran/Dr. Hasan Sadikin Hospital, Clinical Pathology, Bandung, Indonesia
Zhupan3 , Viktor Pushkarenko3 , Agneta Lenchenko1 , Nataliia Sochka1
1 Uzhhorod National University, Children’s Hospital, Uzhhorod, Ukraine; Background and Aims: Almost 700,000 deaths attributable to
2 Uzhhorod National University, Department Of Psychology, Uzhhorod, multidrug-resistance (MDR) occurring each year with Enterococcus
Ukraine; 3 Uzhhorod National University, Medicine Faculty, Uzhhorod, spp., Staphylococcus aureus, K. pneumonia, Acinetobacter baumanii, P.
Ukraine aeruginosin, and Enterobacter spp. (ESKAPE) pathogens became main
concern mainly because of risk to became resistence pathogen, while
Background and Aims: Oncology, COVID-19, war. . . what is the COVID-19 pandemic presents an unprecedented global threat to the
biggest impact on a child’s mental health? May be all three factors at safe and effective care for children with cancer, one of the reason they
once? Can we talk about the quality of life in such conditions? Turning were more prone to be infected and treated with antibiotic irrationally.
to the famous works of Victor Frankl, let’s try to assume our capabil- Aim of this study to discribe the incidence of MDR, extensively drug-
ities. We prioritize understanding the assessment of quality of life in resistant (XDR) and pan drug-resistant (PDR) in febrile neutropenia
children with cancer, as the idea that it is primarily the disease affects patient that admitted during pandemic period that could be present
human life. The war in Ukraine has significantly changed the medical many difficulties come to hospital to seek treatment.
services for children with cancer, so it is important now to investigate Methods: All pediatric oncology patients diagnose with febrile neu-
how the quality of life of a child with cancer has changed. tropenia from January 2020 to Februari 2022 were retrospectively
Methods: The Pediatric Quality of Life Inventory (PedsQL 3.0 Cancer studied. Data consists of demography pattern, underlying disease,
Module) questionnaire was used for 131 people (children aged 5 to 18 number of drawn blood culture and positive results, type of bacte-
and their parents) and divided on two groups:before war in Ukraine (n1 ria, sensitivity pattern. International standard definitions for acquired
= 64) and another during war (n2 = 67). resistance by ECDC and CDC was used as definitions for MDR, XDR
Results: Indicators of the average value according to the scales and PDR bacteria
of the group n1 and n2, respectively (where Xnorm.=100): pain Results: Most of patient came to hospital in severe neutropenia stage
(55.2/79.8); nausea (51.9/79); anxiety associated with procedures (74,6%). Main underlying oncology cases are ALL (38,1%), from 153
(42.8/32.3); anxiety associated with treatment (65.2/56.8); general speciment collected out of 189 subjects, there were 26 (17%) growth
anxiety (39.6/32.5); cognitive problems (63.9/42.1); physical percep- founded from culture. 13, 6, 6, 1 founded in blood, urine, pus, sputum
tion of appearance (68/74,2); communication (71/40). The overall respectively. ESKAPE pathogen dominantly founded in blood with E.
quality of life index x group n1 is the lowest in children/adolescents 13- Coli is the most (46,2%), 11 (84,6%) of them are MDR pathogen while
18 years (x = 52.26) compared to 5-7 years (x = 58.3) and 2-4 years (x = 1(7,6%) is XDR pathogen, there were not any possible PDR pathogen
63.5). In group n2, in general, the quality of life in all age groups of chil- founded.
dren decreased significantly: 5-7 years (x = 47.7), 8-12 years (x = 54.3), Conclusions: The prevalence of MDR pathogens is quite high, but the
13-18 years (x = 45.9). XDR and PDR pathogen is still low, antibiotic for treatment based on
Conclusions: Self-assessment of health is a more powerful predictor guideline and local antibiogram pattern could be rationally use, how-
of mortality and morbidity than many objective indicators of health. A ever, despite the low number, antibiotic resistance remains one of the
structured assessment of the quality of life of children with cancer dur- main challenging issues demanding for further attention expecially in
ing the war is an important final point for further correct treatment and pandemic era.
rehabilitation.

EP602 / #1827 LEVERAGING THE PROFILE TOOL TO


E-Poster Topic: AS05 SIOP Scientific programme / AS05.r Epidemiology, IDENTIFY NATIONAL PRIORITIES AND SUPPORT GLOBAL
Policy and Advocacy INITIATIVE FOR CHILDHOOD CANCER (GICC) COUNTRY
STATUS IN CAMEROON
E-POSTER VIEWING
Glenn Mbah Afungchwi1 , Sona Franklin Mukete2 , Sharmeen Hussain3 ,
EP601 / #412 MICROORGANISM PATHOGEN PATTERN IN Ayomide Omotola3 , Paola Friedrich3 , Nickhill Bhakta3 , Berthe
PEDIATRIC ONCOLOGY PATIENTS WITH FEBRILE Mapoko4 , Andreas Frambo5 , Bernard Njodzeka6 , Rachel Tayou7 ,
NEUTROPENIA ADMITTED TO TERTIARY REFERRAL HOSPITAL Léopold Molel Belika8 , Alberic Signang9 , Francine Kouya9 , Angèle
DURING PANDEMIC CORONAVIRUS-19 Hermine Pondy Ongotsoyi10 , Paul Ndom4
1 Cameroon Baptist Convention Health Services, Cameroon, Bamenda,
Riyadi Adrizain1 , Nur Sari2 , Adhi Sugianli3 Cameroon; 2 Ministry of Public Healthx, National Committee For The Fight
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S426 of S635 ABSTRACTS

Against Cancer, Yaounde, Cameroon; 3 St. Jude Children’s Research Hospital, 1 Barretos Cancer Hospital, Pediatric Neurosurgery, Barretos, Brazil;
Global Pediatric Medicine, Memphis, United States of America; 4 Ministry 2 Barretos Cancer Hospital, Pediatric Oncology, Barretos, Brazil; 3 Barretos
of Public Health, National Committee For The Fight Against Cancer, Cancer Hospital, Cpom, Barretos, Brazil
Yaounde, Cameroon; 5 Clinton Health Access Initiative, Oncology, Yaounde,
Cameroon; 6 Cameroon Baptist Convention Health Services, Paediatric Background and Aims: Pediatric central nervous system (CNS) tumors
Oncology, Bamenda, Cameroon; 7 Ministry of Public Health, Douala General are the most common solid tumors during childhood, but few epi-
Hospital, Duoala, Cameroon; 8 Cameroon Paediatric Oncology Group, Gen- demiological studies in this group. We aimed to characterize the
eral Secretary, Yaoundé, Cameroon; 9 cameroon baptist convention health epidemiological profile of pediatric patients with CNS tumors at Bar-
services, Pediatric Oncology, Bamenda, Cameroon; 10 Mother and Child Cen- retos’s Children and Young Adults Cancer Hospital, a reference for the
tre of The Chantal Biya Foundation, Hematology Oncology Unit, Yaounde, treatment and management of children with CNS tumors in Brazil.
Cameroon Methods: This study retrospectively evaluated patients treated at
Barretos’s Children and Young Adults Cancer Hospital from Septem-
Background and Aims: Paediatric oncology professionals must work ber 2013 to December 2019. 313 patients were collected from the
together with civil society and government to improve childhood REDCap platform and included: histopathology reports, patient demo-
cancer survival in low- and middle-income countries. The Cameroon graphics, symptoms/signs at the time of diagnosis, symptom interval,
National Strategic Plan for the Fight Against Cancer (NSPFAC) aims to and anatomical site of the tumor. Patients were divided into groups 1
improve survival by 25% for all cancers by 2024. To identify priority (less than or equal to 2 years of age) and group 2 (greater than two
actions towards this goal, the Cameroon Paediatric Oncology Group years of age).
and National Committee for the Fight Against Cancer led a national Results: Of the 313 cases reviewed, 53.7% were males, and 46,3%
priority-setting initiative. were females. The average age was 8.5 years. The interval between
Methods: The St Jude Global Paediatric Oncology Facility Integrated the onset of signs and symptoms until diagnosis was three months.
Local Evaluation (PrOFILE) tool was used to identify opportunities for Approximately 5.1% of cases are associated with genetic syndromes.
improvement. A baseline assessment was conducted by institutional The number of patients younger than two years was 35 (11.2%),
representatives at two hub centres and one shared care centre. Data and those ≥ 2 years 278 (88.8%). The most frequent symptoms/signs
was analyzed centrally at St. Jude. Using these data as evidence to recorded at presentation were: headache (59.7%), nausea/vomiting
guide discussion, a hybrid national stakeholder workshop was orga- (39.6%), cranial nerve palsy (27.2%), seizures (17.9%), limb weak-
nized and led by the Ministry of Health. Using a co-design methodology, ness (16.6%), cerebellar syndrome (15.7%), visual changes (15.3%).
four exercises were completed by 49 in-person stakeholders in order to Patients in Group 1 presented different frequencies of signs and
identify 12 priorities. symptoms concerning Group 2, prevailing: vomiting (40%), develop-
Results: From baseline data analysis, strengths and weaknesses were mental delay (25.7%), cranial nerve palsy (17.1%), and limb weakness
identified and recommendations made for six domains including: (17.1%).
national context, workforce, diagnostics, chemotherapy and support- Conclusions: We report important epidemiological information for
ive care, surgery and radiotherapy, and patients and outcomes. The Brazilian pediatric CNS tumors. We identified differences in diagnostic
priority action areas identified were training at all levels; strength- range, signs, symptoms, histological types, and regions affected in chil-
ening cancer registry; and increasing funding for care and research. dren younger than or equal to 2 years of age and children older than
Next steps identified included: develop an action plan in line with the two years.
NSPFAC; organize working groups to address the identified priorities;
engage the World Health Organization to support activities that align
with the CURE ALL Framework. EP604 / #527 IMPACT OF COVID-19 PANDEMIC TOWARDS
Conclusions: By leveraging technical support from St Jude Global, the PEDIATRIC CANCER CARE: A SYSTEMATIC REVIEW
Cameroon MoH leadership and civil society stakeholders have identi-
fied key priorities to support childhood cancer activities nationally. In Radhian Amandito, Angela Kimberly Tjahjadi, Ludi Dhyani Rahmartani
addition, the workshop report was shared with World Health Orga- Universitas Indonesia - Cipto Mangunkusumo Hospital, Child Health,
nization officials to demonstrate ongoing work and request additional Jakarta, Indonesia
support through the GICC.
Background and Aims: Cancer, when identified early, is more likely to
respond to effective treatment. COVID-19 pandemic causes reduction
EP603 / #687 EPIDEMIOLOGY OF PEDIATRIC CENTRAL in services therefore patients experience significant delay in diagnosis
NERVOUS SYSTEM TUMORS: A SINGLE-CENTER EXPERIENCE and treatment. In particular, pediatric cancer patients are considered
FROM 2013 TO 2019 vulnerable subjects despite encompassing around only 5-10% of total
cancer cases. Herewith we perform a systematic review to determine
Carlos Almeida Junior1 , Bruna Mançano2 , Maristela Facholi2 , Lucas the extent of changes in healthcare and the outcome in pediatric cancer
Lourenço2 , Rui Reis3 cases during the COVID-19 pandemic and postpandemic.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S427 of S635

Methods: PubMed, EBSCO Host, the Cochrane Library, and Proquest Results: A mean delay by parents of 39.44 days (63.7 SD) was obtained
were searched up to March 2022 using concepts including COVID-19, (minimum 1 day and maximum 360 days); the mean delay of health per-
pediatric cancer, and impact. Inclusion criteria were: 1) Studies describ- sonnel, also called diagnostic delay, was 26.84 (38.8 SD), (minimum 1
ing timing of diagnosis and treatment of pediatric cancer in COVID-19 day and maximum 210 days); and a mean total delay of 66.28 days (77.3
pandemic period in children; 2) reporting of at least one of the follow- SD), minimum of 5, maximum of 376. The factors associated with pater-
ing outcomes: delay in diagnosis, treatment. We excluded letters and nal delay with statistical significance were age (p 0.021); the smaller
editorials. Risk of bias was assessed by using the ROBINS-I tool. Pri- the child, the less delay time; the type of tumor, having a longer delay in
mary outcome was delayed diagnosis and treatment, with secondary bone tumors (mean of 112 days), and retinoblastoma with a mean delay
outcome was change in hospital visit compared with telemedicine. of 104 days. The factors associated with diagnostic delay by medical
Results: Fifteen studies out of 12.466 hits were identified reporting personnel were diagnostic certainty, having less delay time in the event
from > 10 different countries. There are reported delays in diagnosis of early suspicion of oncological pathology (p 0.01)
varying from 3.5% - 62%, delays in treatment varying from 3.5% - 64.6% Conclusions: The factors associated with total delay are age, type of
(including chemotherapy, radiotherapy), and also a shifting trend into cancer and educational level of the parents. Greater delay attributed
preferring telemedicine over hospital visits. Reasons for delay include to parents
limited service, risk of infection, and COVID-19 infection. Reported
positive impacts include higher appointments via telemedicine, and
receiving cancer care closer to home/decentralized care. There are no EP606 / #1763 HEREDITARY CANCER SYNDROMES, THE
differences between solid and hematological cancers. FORGOTTEN OF LONG-TERM FOLLOW-UP CARE OF PEDIATRIC
Conclusions: The COVID-19 pandemic has negative impacts in the ONCOLOGY: NEUROFIBROMATOSIS TYPE 1, EPIDEMIOLOGICAL
already vulnerable subjects that are pediatric cancer patients ranging OVERVIEW OF A SINGLE INSTITUTION IN MEXICO
from delays in diagnosis into treatment. Even so, the pandemic has
shown us the possibility of employing telemedicine in pediatric can- Ellioth A.F. Quintana-García, Farina Arreguin-González, Ruben
cer healthcare utilization to help in postpandemic recovery and future Sebastián-Barajas, Eduardo J. Baños-Rodriguez, Andrea Padilla-Soto,
pandemics. Gabriela Barrera-Villegas
ISSSTE CMN 20 de Noviembre, Pediatric Oncology, Mexico City, Mexico

EP605 / #1965 FACTORS ASSOCIATED WITH DELAYED Background and Aims: Neurofibromatosis type 1 (NF1), autosomal
DIAGNOSIS OF CANCER IN CHILDREN UNDER 15 YEARS OF dominant disorder, heterozygous loss-of-function mutation in the NF1
AGE IN THE ONCOHEMATOLOGY UNIT OF HOSPITAL DEL gene. It is part of hereditary cancer syndromes (HCS). Patients with
NIÑO OVIDIO ALIAGA URIA NF1 have a decrease in life expectancy of about 15 years compared
with the general population, Cancer is a leading cause of death, these
Soraya Marianela Arancibia Romero1 , Susan Sardinas2 , Patricia Parra patients are susceptible to some type of screening and follow-up care.
Nigañez2 Objective: to know the epidemiological panorama of NF1 in our insti-
1 Hospital del Niño Ovidio Aliaga Uria, La Paz, La paz, Bolivia; 2 Hospital del tution, and to establish the need for an HCS clinic for multidisciplinary
Niño Ovidio Aliaga Uria, Pediatric Oncology, La Paz, Bolivia follow-up.
Methods: Cross-sectional retrospective cohort study in <25 years
Background and Aims: Early diagnosis of childhood cancer is essen- with a diagnosis of NF1, between January 2011 and September 2021,
tial, since it allows treatment in early stages, which results in a better National Medical Center "20 de Noviembre", we obtained demographic
prognosis and a positive effect on quality of life. First contact physi- variables, details of the multidisciplinary follow-up care, we evalu-
cians seldom interpret symptoms in a timely manner and this implies a ated the multidisciplinary follow-up care according to the number
delay in diagnosis; Parents do not always give importance to symptoms, of medical specialty that provided follow-up care: good(>7 physician
which also causes delay. By virtue of knowing these data, the objec- specialties), regular(4-6 physician specialties) and bad(<3 physician
tive of this research was proposed: Identify the factors associated with specialties).
the delay in cancer diagnosis in children under 15 years of age treated Results: During the study period, 115 patients were diagnosed, we
at the Oncohematology Service of the Ovidio Aliaga Uria Children’s eliminated 13, leaving 102 patients, 52 female and 50 male; age range
Hospital. from 1 month to 23 years; mean age: 8.8 years; 62% had no fam-
Methods: It is a non-experimental, retrospective, analytical work, the ily history of NF1, 25% development of plexiform neurofibroma: 35%
information was obtained in direct interviews with the parents and abdominal, 27% head and neck, 19% chest, 19% extremities, 52% had
review of clinical records applied to 104 patients; subsequently ana- bad multidisciplinary follow-up care, 15% regular and 28% good. The
lyzed by calculating the mean and statistical significance for the differ- first contact medical specialty was Medical genetics 45%, and only 14%
ent non-parametric variables using the Kruskal Wallis and Whitney’s presented follow-up care by Pediatric Oncology, 10% course with diag-
Mann U test. nosis of cancer: tumors of the central nervous system 55%; sarcomas
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S428 of S635 ABSTRACTS

18%, and hematological neoplasms 27%; 36% abandoned follow-up Manushak Avagyan1 , Tatev Arakelyan2 , Sharmeen Hussain3 , Nickhill
care and 5% were reported as deaths. Bhakta4 , Eva Steliarova-Foucher1
Conclusions: The care of patients with NF1 requires the teamwork of 1 International Agency for Research on Cancer, Cancer Surveillance Branch,

multiple medical specialties, in order to establish screening and health Lyon, France; 2 Hematology Center after R.Yeolyan, Pediatric Cancer And
education programs, to improve timely detection of cancer and their Blood Disorders Center Of Armenia, Yerevan, Armenia; 3 St. Jude Children’s
life expectancy. Research Hospital, Global Pediatric Medicine, Memphis, United States of
America; 4 St Jude Children’s Research Hospital, Oncology, Department
Of Global Pediatric Medicine, Department Of Epidemiology And Cancer
EP607 / #2004 INFECCIONES ASOCIADAS A LA ATENCIÓN Control, Memphis, United States of America
DE SALUD EN PACIENTES PEDIÁTRICOS CON
INMUNOSUPRESIÓN POR VIRUS DE INMUNODEFICIENCIA Background and Aims: Representative, accurate, accessible, and
HUMANA Y CÁNCER timely data are the cornerstone of cancer research and care. Imple-
mentation of the General Data Protection Regulation (GDPR) of the
Lisbeth Aurenty, Augusto Pereira, Juan García, Tatiana Drummond European Union (EU) in 2018 led to increased individual control of per-
JM de los Ríos Children’s Hospital, Infectious Deseases Department, Cara- sonal data and inhibited health research. We reviewed the reported
cas, Venezuela impediments and proposed solutions to maintain international data
sharing.
Background and Aims: RESUMEN Infecciones Asociadas a la Aten- Methods: Three independent reviewers searched PubMed for arti-
ción de Salud (IAAS) incrementan estancia hospitalaria, morbilidad cles in English published in 2011-2021 addressing E-Poster Topics like
y mortalidad en inmunocomprometidos. Objetivo: Evaluar evolución GDPR, data sharing, information dissemination, health research, and
clínica y pronóstico de pacientes pediátricos con VIH (Virus de cancer research. Papers focusing only on biobanks or genomic research
Inmunodeficiencia Humana) y cáncer que presentan eventos de IAAS were excluded.
hospitalizados. Results: Out of 643 identified publications, 43 met criteria for inclu-
Methods: MÉTODOS Método: estudio observacional, descriptivo, sion. All papers originated in high income countries. The barriers
prospectivo del Hospital de Niños JM de los Ríos en pacientes entre identified through qualitative synthesis were: scarcity of qualified
1 mes - 18 años, durante 2014 – 2017; formulario incluyó demografía, personnel and infrastructure, uncertainty over key definitions, poor
tipo de infección, estancia hospitalaria, tratamiento recibido y evolu- interpretation of rules, fear of penalties, and lack of fit of GDPR for
ción; registrándose base de datos Google drive, analizándose con scientific research. Specific to data sharing within the EU, the imple-
Epi-Info 7 y STATA12 análisis descriptivo general, frecuencias relativas, mentation of GDPR was obstructed by fragmentation and insufficient
porcentajes para variables nominales y medidas de tendencia central harmonization. Interpretation of crucial terms, such as ‘personal data’,
(media y desviación estándar para variables cuantitativas). ‘informed consent’ and ‘data anonymization’ depended on the applica-
Results: Resultados: 5.224 pacientes, 652 presentaron IAAS 12,48% tion of national laws and local expertise. Transfer of data outside the EU
(652/5224), 100 eventos de IAAS en 70 pacientes. Tasa de IAAS: was hampered by the absence of workable legal transfer mechanisms.
12,64%, Densidad de Incidencia: 0,04 día, Sexo: 51% femenino y 49% To improve the availability of data for international research, the pro-
masculino; 36% adolescentes, 26% procedencia Estado Miranda, edad posed solutions included: control of the interpretation of the GDPR,
decimal 8,83 (DE=7,71). Estancia hospitalaria 65,6 días. IAAS más fre- more explicit definitions of terms and processes, and harmonized
cuentes 22% en Leucemia Linfocítica Aguda, 12% Neuroblastoma y application of the regulations.
pacientes VIH estadío C3; IAAS más reportada con 24% bacteriemia. Conclusions: Our review confirmed that GDPR affects cancer
Eventos por paciente de 1 - 4. En 99% tratamientos antibióticos estu- surveillance and research, jeopardizing thus the public interest
vieron combinados. Microorganismos aislados: 13,5% Pseudomonas in learning from contributed data. Data sharing is indispens-
aeruginosa, 9,6% Candida parapsilosis y S. aureus, 7,7%. Klebsiella able, especially in research of rare diseases like childhood cancer.
pneumoniae y Staphylococcus coagulasa negativo. Causas de muerte: Using these data, the ChildGICR programme (https://gicr.iarc.
31,8% insuficiencia respiratoria, 26,3% Bacteriemia Asociada a Catéter fr/childgicr) will develop policy briefs to support global cancer
Vascular, 21% Sépsis bacteriana y 10,5 % falla multiorgánica. Mortali- surveillance.
dad relacionada con IAAS 89,5%.
Conclusions: Conclusión: IAAS modifican evolución y pronóstico en
pacientes pediátricos con inmunosupresión por VIH y Cáncer, primera EP609 / #934 THE CUDDLES FOUNDATION MODEL FOR
descripción en Venezuela y Latinoamérica. ADDRESSING MALNUTRITION IN PEDIATRIC ONCOLOGY
PATIENTS IN INDIA

EP608 / #501 SHARING PERSONAL DATA IN Purnota Bahl1 , Anju Morarka2 , Sripriya Venkiteswaran3
INTERNATIONAL HEALTH RESEARCH: A SCOPING REVIEW 1 Cuddles Foundation, Cuddles Foundation, Mumbai, India; 2 Cuddles Foun-

dation, Research, Knowledge Management And Impact, Mumbai, India;


15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S429 of S635

3 Cuddles Foundation, Cuddles Institute Of Clinical Nutrition (cicn), Mumbai, Background and Aims: Lack of access to essential medicines has lim-
India ited improvements in global childhood cancer survival. FORxECAST
is a pediatric cancer-specific model that predicts future drug quantity
Background and Aims: Malnutrition at diagnosis or/and during treat- and cost using modelled or user-inputted data including resource-
ment in pediatric oncology patients can result in poor outcomes adapted treatment regimens (ATRs), incidence, and local drug pricing.
and survival. Children from a lower socioeconomic group, in particu- We used FORxECAST to predict the drug needs and costs for treating
lar, are at a higher risk of being malnourished. This study highlights acute lymphoblastic leukemia (ALL) in Argentina.
the model which Cuddles Foundation (CF) uses to provide nutri- Methods: FORxECAST was modified to incorporate Argentine treat-
tional support to such children in government hospitals throughout ment protocols. Sensitivity analyses were conducted using in-built
India. consensus ATRs and 2015 international reference pricing from the
Methods: In collaboration with government hospitals, CF first places Management Sciences for Health (MSH) International Medical Prod-
specially trained nutritionists in the pediatric oncology departments ucts Price Guide, adjusted for inflation. Incidence data from the Argen-
and then onboards children who are in need of nutrition support. tinean Oncopediatric Registry (ROHA) and domestic pharmacy retail
The CF nutritionist assesses the clinical condition and nutritional sta- price data, represented as median cost per drug, were incorporated.
tus of the patient, designs a nutrition intervention program, monitors Results: Using MSH prices and modelled disease incidence, the annual
progress and supports the family through parent support meetings. median cost of treating ALL in Argentina was 1.20 million (M) USD
Nutrition aid if required is provided in the form of ration bundles, with Argentine-specific protocols and 1.16 M USD with ATRs. The
nutritional supplements, nutritious snacks and hot meals. Patient data difference in aggregate cost was driven by increased use of IV
is maintained in the internal CF FoodHeals® app, which enables the methotrexate in Argentina protocols compared to ATRs (3.6 vs. 0.1
tracking of nutritional status and ensures uniformity of care across the M mg); however, the total volume of chemotherapy drugs required
various CF centres. remained broadly similar. When adjusting for incidence per ROHA,
Results: Over the last 5 years, CF nutritionists have provided over the cost with Argentine-specific protocols increased to 1.26 M USD.
630,000 counsels. In the year 2020-2021, despite the COVID-19 pan- The largest difference was seen when MSH prices were replaced by
demic, CF provided 101,805 counsels including teleconsultations to Argentina-specific prices with overall cost increasing to 4.73 M USD.
6,123 patients. Nutrition supplements worth USD 218,623, ration bun- Conclusions: FORxECAST can be readily adapted to develop accurate
dles worth USD 368,139, nutritious snacks worth USD 62,616 and hot estimates of drug quantities and prices in the treatment of paediatric
meals worth USD 15,886 were distributed to the recipients. Using data cancer using modelled and user-inputted data. Our results highlight
from the FoodHeals® app, CF was able to assess that 80% of patients the significant impact of local pricing on drug costs in comparison
improved or maintained their nutritional status during treatment and to international references; local prices are essential for accurate
94% of patients counselled, returned for a second visit or continued forecasting of domestic drug budgets. In Argentina, innovative pro-
treatment. curement strategies, regulation of drug pricing, and health technology
Conclusions: Children were able to maintain or improve their nutri- assessment processes are needed to improve sustained and affordable
tional status with support from CF. This study demonstrates that the supply of quality-assured childhood cancer medicines.
CF model is effective in providing essential nutrition support and
counselling to help address malnutrition in children during cancer
treatment. EP611 / #920 IMPACT OF COVID-19 INFECTION IN
CHILDREN WITH CANCER: EXPERIENCE IN A HOSPITAL IN
LATINAMERICA
EP610 / #405 FORECASTING POPULATION-BASED
MEDICINE NEEDS AND COSTS FOR CHILDHOOD ACUTE Carolina Basile1 , Alejandra Casanovas2 , Diego Amaral2 , Ernesto
LYMPHOBLASTIC LEUKEMIA TREATMENT IN ARGENTINA Veber2
1 Hospital de Clinicas Jose de San Martin/ Hospital de Niños Pedro de
Herman Bami1 , Terence Hughes2 , Analia Lopez3 , Edith Elizalde, Pediatric - Oncohematology Section - Aya Group, Buenos Aires,
Grynszpancholc4 , Sumit Gupta5 , A. Lindsay Frazier6 , Avram Denburg7 Argentina; 2 Hospital de Niños Pedro de Elizalde, Pediatric - Oncohematol-
1 The Hospital For Sick Children, Paediatrics, Toronto, Canada; 2 Icahn ogy Section - Aya Group, Buenos Aires, Argentina
School of Medicine at Mount Sinai, Medicine, New York, United States
of America; 3 Instituto Universitario CEMIC (IUC), Public Health, Buenos Background and Aims: While cancer is an established risk factor for
Aires, Argentina; 4 Natalí Dafne Flexer Foundation, Foundation Presi- severe COVID-19 in adults, it has thus far not been considered so in
dent, Buenos Aires, Argentina; 5 Hospital for Sick Childrenn, Haematol- children. The aim of this study is to describe the epidemiological char-
ogy/oncology, Toronto, Canada; 6 Dana-Farber Cancer Institute, Pediatric acteristics, histological type, clinical evolution and repercussions in the
Oncology, Boston, United States of America; 7 The Hospital for Sick Children, treatment of patients with cancer infected by COVID-19.
Haematology & Oncology, Toronto, Canada Methods: Epidemiological and retrospective study. We reviewed the
medical records of patients (p) with COVID-19 treated in the oncology
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S430 of S635 ABSTRACTS

pediatric department of a Hospital from latinamerica from march 2020 (SIR) and 95% confidence intervals were calculated comparing Hawaii
to march 2022. to US rates.
Results: During that period 52p were diagnosed with solid tumors. Results: Within Hawaii, age adjusted incidence rates of leukemia, lym-
Median age: 7 years (2 month -17 years). Male: 30p (58%). Female phoma, neuroblastoma, hepatic tumors, and germ cell tumors were
22p (42%). Eighteen p (34%) underwent surgery only, 34p (65%) similar across all races. Compared to White children, incidence of CNS
received intensive chemotherapy and/or radiotherapy. Seventeen p tumors was lower in Filipinos, Japanese, and Native Hawaiians (NH)
(32%) were infected by COVID-19. Histology: medulloblastoma 5p, (P<.01, P<.05, P<.01), incidence of neuroblastoma was lower in Fil-
ependymoma 1p, high-grade glioma 1p, Wilms tumor 4p, osteosar- ipinos (P<.05), incidence of renal tumors was lower in Japanese and NH
coma 1p, Ewing’s sarcoma 1p, germ cell tumor 1p, rhabdomyosarcoma (P<.01), and incidence of bone tumors was higher in Japanese (P<.01).
1p, cavum carcinoma 1p, angiosarcoma 1p. Clinical manifestations: Relative to the mainland US, incidence rates were similar for leukemia,
11p (58%) asymptomatic, 4p (21%) mild symptoms (no hospitaliza- hepatic tumors, and germ cell tumors in Hawaii. Incidence rates were
tion requirement), 1p (5%) severe respiratory symptoms, mechanical lower than the US for lymphomas for Japanese and NH (SIR0.57, 0.67);
respiratory assistance requirement, was the only one who delayed for CNS tumors in Japanese, Filipino, and NH patients (SIR0.56, 0.45,
his treatment. None have risk factors. No multisystem inflammatory 0.75); for renal tumors in Japanese and NH patients (SIR0.15, 0.15); and
syndrome associated. No deaths for bone tumors in White patients (SIR0.46).
Conclusions: In our cohort, Children with cancer have not gener- Conclusions: Racial and ethnic differences are seen in incidence of
ally had increased incidence or severity of COVID-19 infection. The pediatric cancer subtypes within Hawaii and compared to mainland
COVID-19 infection did not interfere with the oncological treatment of US. Our findings provide an important foundation for future studies
the patients except for one case that began with severe symptoms after to better understand the genetic, immune and socioeconomic factors
surgery. These results are similar to those published in international underlying these differences.
reports.

EP613 / #1208 INVASIVE FUNGAL DISEASE IN PEDIATRIC


EP612 / #910 RACIAL AND ETHNIC DISPARITIES IN ONCOLOGY
PEDIATRIC CANCER INCIDENCE AMONG CHILDREN AND
ADOLESCENTS IN HAWAI’I FROM 1990 TO 2014 Nouria Benmouffok, Adam Roumani, Fatma Zohra Boudouaya,
Rachida Nemmar
Amanda Beaman1 , Lauryn Liao2 , Trevor Hirata2 , Janine Abe3 , Lynne CHU Nafissa Hammoud, Pediatrics A, Algiers, Algeria
Wilkens3 , Lena Winestone4 , Michelle Hermiston5 , Russell Woo6 ,
Stephanie Lim2 Background and Aims: Invasive fungal infections are a major cause
1 University of Hawaii, John A. Burns School Of Medicine, Honolulu, United of death in pediatric oncology, especially among patients under
States of America; 2 University of Hawaii Cancer Center, Division Of Pedi- chemotherapy. This study aims to identify risk factors for invasive
atric Hematology/oncology, Honolulu, United States of America; 3 University fungal infections in pediatric oncology.
of Hawaii Cancer Center, Cancer Epidemiology, Honolulu, United States of Methods: We conducted a monocentric retrospective case-control
America; 4 UCSF Benioff Children’s Hospitals, Allergy, Immunology, And Bmt, multi-cohort study on a population of 30 patients with malignant
San Francisco, United States of America; 5 UCSF Helen Diller Family Com- hemopathies or solid cancers under chemotherapy, admitted in the
prehensive Cancer Center, Pediatric Hematology/oncology, San Francisco, Pediatric Oncology unit of Nafissa Hammoud Hospital in Algiers,
United States of America; 6 University of Hawaii John A. Burns School of amongst which 24 patients were controls, and 6 patients were cases.
Medicine, Pediatric Surgery, Honolulu, United States of America Results: In a total of 30 patients (53.3 % male), 13 patients developed
a fever, from which 6 patients on 13 were identified as invasive fungal
Background and Aims: Racial and ethnic differences in cancer inci- infection cases according to the EORTC/MSG guidelines, with an inci-
dence have been reported for many pediatric cancers and have dence of 20 %. The mean age was 7.47 years old at the admission date.
been attributed to genetic ancestry, infection exposure, and socioe- 04 statistically significant risk factors were identified (p-value < 0.05,
conomic status. As a “minority-majority” state, Hawai’i provides a CI 95 %) : mucositis with an odds ratio (OR) at 10 (1,34 – 74,51), the
unique opportunity for close examination of these differences among most aggressive chemotherapy protocol according to the ITR2 with an
underrepresented groups and to isolate the distinct contributions of OR at 115 (6,10 – 2165,95), severe prolonged neutropenia with an OR
environment and ancestry. We aim to characterize the incidence of at 7,6 (1,07 – 54,09) and severe prolonged lymphopenia with an OR at
common pediatric cancer types between different races and ethnicities 25 (2,27 – 275,71).
within Hawaii and compared to mainland US using the SEER 9 API data. Conclusions: Many conditions were identified as risk factors for
Methods: Incidence rates (per 100,000) were age adjusted by the invasive fungal infections in pediatric oncology, especially severe lym-
direct method, among children and adolescents (aged 0 to 19 years old), phopenia and aggressive chemotherapy. These patients may have to
using SEER 9 API data from 1990-2014. Standardized incidence ratios receive close monitoring or even antifungal prophylaxis.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S431 of S635

EP614 / #1742 INFLUENCING FACTORS IN THE DELAY OF Hope (hematology/oncology Pediatric Excellence), Houston, United States of
INTIAL DIAGNOSIS OF CHILDHOOD CANCER America

Miguel Berger Saunderson1 , Paola Moreno Mendoza1 , Andrea Background and Aims: Texas Children’s Global HOPE is a collab-
Ellis-Irigoyen2 , Lourdes Vega-Vega3 , Gabriela Escamilla Asiain4 , Maria oration focused on advancing pediatric cancer care in sub-Saharan
Del Carmen Esmer Sánchez5 Africa. A major barrier to improving outcomes is the availability and
1 Universidad Anahuac, School Of Medicine, Queretaro, Mexico; 2 Hospital affordability of chemotherapy and supportive care. The purpose of
Infantil Teleton de Oncologia, Pediatric Oncology, Queretaro, Mexico; this project was to create a tool with program-specific inputs to help
3 Hospital Infantil Teleton de Oncologia, General Director, Querétaro, Mex- forecast medication needs of our programs.
ico; 4 Hospital Infantil Teleton de oncologia, Medical Director, Queretaro, Methods: This was an operational and quality improvement initiative
Mexico; 5 Hospital Infantil Teleton de Oncologia, Research, Queretaro, to reduce stock-outs and improve access to medications. We per-
Mexico formed semi-structured interviews with program medical directors at
each clinical site to better understand treatment practices. Historical
Background and Aims: Being the leading worldwide cause of non- patient data were collected from program records. Treatment details
communicable death in minors, low- and mid-income countries have were abstracted from treatment protocols and guidelines identified
more than 80% of the oncological burden and almost 90% of the pae- from semi-structured interviews. A customizable tool was designed
diatric population. The opportune diagnosis is important because of its to incorporate these inputs and forecast immediate and long-range
progression without treatment, contrasting its curability rate at early medication needs.
stages. Paediatric cancer’s recognition is tougher because of unspe- Results: We identified 24 disease states and 28 distinct treatment
cific symptoms which can be confused with more common diseases, protocols through semi-structured interviews and database review.
patients age, and many other factors. Within these protocols, we identified 32 chemotherapy agents for ini-
Methods: A retrospective study was made through 406 electronic files tial forecasting. Excel workbooks were created for each disease state
of 0–17-year-olds, admitted to our hospital through December 2013- with customized inputs (median patient age, body surface area, risk
February 2020 with a high suspicion of cancer. stratification, survival estimates, patient attrition, anticipated program
Results: Mean age was 7 years 10 months old, with a 1:1.3 M:F ratio. growth) and fixed inputs (drug doses, frequency, and cycle details;
In average, patients went to 2.3 physicians (2-8 total) before the can- vial/tablet sizes). Each workbook generates a forecasted number of
cer diagnosis, 37.93% took 0 to 31 days for that diagnosis, 20.69% took vials/tablets for a single disease state. These tools have been used to
32 to 61 days, 12.07% took 61 to 90 days and 29.31% took longer. forecast medication needs for 6 programs in 5 countries for the past
For 259 patients, their first physician was a general practitioner, 80 2 years. Since March 2020, this approach has allowed us to work with
consulted a paediatrician and 67 a subspecialist. Sociodemographic donors to receive an equivalent of over $10 million in medications.
factors were analysed using multivariate analysis to determine their Challenges using the tool include changing clinical practices, practice
influence on the diagnosis delay, such as age, type of cancer, initial outside of guidelines, and unanticipated patient fluctuations such as
symptoms, religion, socioeconomic strata, academic degree of first responses to Covid19.
physician, insurance and academic degree of parents without finding a Conclusions: Forecasting medication needs is an essential compo-
significant difference. Survival in patients diagnosed within the first 30 nent of program planning. Program-specific forecasting allows for
days was significantly better than the rest. tailored inputs and more sensitive analysis of needs, enabling a pro-
Conclusions: We identified a delay in the referral from the first physi- gram to anticipate medication-related costs and strategize approaches
cian to a level 3 hospital, not determined by its specialty, or factors like to prevent medication shortages.
parental academic degree, religion, socioeconomic strata, etc. Diag-
nosis before 30 days is associated with a better overall survival. The
opportune detection signs and symptoms of childhood cancer are well EP616 / #1169 SUSTAINED GROWTH OF A GLOBAL
known, it’s important that first contact healthcare workers know the CLOUD-BASED HOSPITAL-BASED CANCER REGISTRY (HBCR): A
exams that can help rule out that diagnosis. REPORT FROM THE SJCARES REGISTRY COLLABORATIVE
NETWORK

EP615 / #1076 CREATION OF A PROGRAM-SPECIFIC Nickhill Bhakta1 , Gia Ferrara2 , Hilmarie Muniz-Talavera3 , Yichen
FORECASTING APPROACH FOR ESSENTIAL CHEMOTHERAPY: Chen4 , Alia Ahmad5 , Soad Fuentes-Alabi De Aparicio6 , Magda
A GLOBAL HOPE PERSPECTIVE Arreola7 , Tsetsegsaikhan Batmunkh8 , Pascale Gassant9 , Justin
Mulindwa10 , Venkatraman Radhakrishnan11 , Karina Quintero12 , Hilze
Melanie Bernhardt1,2 , Hailey Zhang2 , James Brown2 , Kristina Rodriguez13 , Raya Saab14 , Gevorg Tamamyan15 , Amita Trehan16 ,
Wilson-Lewis2 Meenakshi Devidas17 , Carlos Rodriguez-Galindo17
1 Baylor College of Medicine, Pediatric Hematology/oncology, Houston, 1 St Jude Children’s Research Hospital, Oncology, Department Of Global
United States of America; 2 Texas Children’s Hospital, Texas Children’s Global Pediatric Medicine, Department Of Epidemiology And Cancer Control,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S432 of S635 ABSTRACTS

Memphis, United States of America; 2 St Jude Children’s Research Hospi- of new members and patient registrations in the network have more
tal, Department Of Global Pediatric Medicine, Memphis, United States of than tripled over the past year. Next steps involve deploying quality
America; 3 St. Jude Children’s Research Hospital, Department Of Global control procedures, identifying implementation strategies to support
Pediatric Medicine, Memphis, United States of America; 4 St. Jude Children’s network growth and analyzing patient outcomes.
Research Hospital, Global Pediatric Medicine, Memphis, United States of
America; 5 The Children’s Hospital and Institute of Child Health, Oncology,
Lahore, Pakistan; 6 Hospital Nacional de Ninos Benjamin Bloom, Pediatric EP617 / #1590 A RAPID ASSESSMENT COSTING TOOL TO
Oncology, San Salvador, El Salvador; 7 UNOP, Oncology, Guatemala City, ESTIMATE PUBLIC SECTOR CHILDHOOD CANCER SPECIFIC
Guatemala; 8 National Center for Maternal and Child Health of Mongolia, BUDGET NEEDS FOR NATIONAL CANCER CONTROL PLANS
Oncology, Ulaanbaatar, Mongolia; 9 Hôpital Saint - Damien, Pediatric Oncol- (NCCPS) IN LOW-AND MIDDLE-INCOME COUNTRIES (LMICS)
ogy, Port-au-Prince, Haiti; 10 Cancer Disease Hospital, Pediatric Oncology,
Lusaka, Zambia; 11 Cancer Institute (W.I.A), Medical Oncology, CHENNAI, Nancy Bolous1 , Nester Chekwenda-Makore2 , Miguel Bonilla1 , Grace
India; 12 Hospital del Niño Dr Jose Renan Esquivel, Oncology, Panama, Chingo3 , Joyce Kambugu4 , Justin Mulindwa3 , Mugisha Noleb4 , Inam
Panama; 13 Hospital del Niño Dr Jose Renan Esquivel, Hematology, Panama, Chitsike2 , Nickhill Bhakta1
Panama; 14 American University of Beirut, Pediatrics, BEIRUT, Lebanon; 1 St. Jude Children’s Research Hospital, Department Of Global Pediatric
15 Hematology Center after R.Yeolyan, Pediatric Cancer And Blood Disor- Medicine, Memphis, United States of America; 2 Faculty of Medicine and
ders Center Of Armenia, Yerevan, Armenia; 16 Advanced Pediatrics Center, Health Sciences, University of Zimbabwe, Department Of Paediatrics And
Postgraduate Institute of Medical Education and Research, Pediatric Hema- Child Health, Harare, Zimbabwe; 3 Cancer Disease Hospital, Pediatric
tology Oncology Unit, Chandigarh, India; 17 St Jude Children’s Research Oncology, Lusaka, Zambia; 4 Uganda Cancer Institute, Paediatric Oncology
Hospital, Global Pediatric Medicine, Memphis, United States of America Department, Kampala, Uganda

Background and Aims: The SJCARES Registry Collaborative network Background and Aims: The need to present budget forecasts for
was designed to provide a standardized mechanism for HBCR data col- NCCPs is often challenging, due to urgent time constraints. Using an
lection and support institutional quality improvement. We report the accepted costing framework, we piloted a rapid assessment budget
network growth from the first 18 months of operation. tool using excel, to estimate the specific public sector needs associated
Methods: Data captured between 9/24/2020 and 3/7/2022 are with childhood cancer control.
included. Procedures to join the network are provided on our web- Methods: Publications with data from nine institutions in LMICs clas-
site (https://www.stjude.org/global/sjcares/registry.html). Participat- sified costs into eleven categories. Primary costing data for four
ing institutions are onboarded on a rolling basis. Case registrations are categories (medical personnel, non-medical personnel, hoteling, out-
also rolling, starting with essential information and demographics (pre- patient) were collected in Zambia, Zimbabwe, and Uganda. Pharmacy
sentation date), diagnosis/staging (3-months later), frontline treatment costs were calculated using country-specific modeled data (https://
(6-months later) and follow-up/outcomes (annually). Data entered are chemoglobe.stjude.org/). The remaining six categories (pathology, radi-
identifiable only to the local team. De-identified data are available to ation, imaging, surgery, blood services and administration) were esti-
all Collaborative members for quality control and loco-regional-global mated using proportions of the overall budget, bounded by ranges from
analyses. previous publications. Scale-up scenarios are included based on goals
Results: 108 institutions have signed data use agreements to join collaboratively defined by the local planning teams.
the network. 51/108 network members established local teams and Results: An average of three weeks were required to generate a pre-
23/51 completed the onboarding process. 11/23 have prospectively liminary budget, currently under revision. For Zambia, Zimbabwe and
entered >20 patients’ data into the network. 12/23 either recently Uganda, we estimated a baseline budget of $640,000, $1,100,000,
onboarded or are finalizing operating procedures. 127 individuals have and $1,600,000, respectively. The two largest baseline cost cat-
completed HBCR training. Essential information for 2612 patients egories were medical personnel (43%-49%) and pharmacy (16%-
across all sites are included, an increase of 1974 new patient regis- 31%). Forecasted year-five budgets were $2,200,000, $4,000,000, and
trations over a one year period. Complete demographic and diagnostic $6,600,000. Over the five-year period, pharmacy costs had the high-
data are available for 94.3% (2462/2612) and 92.4% (2413/2612) of est growth (average 10%) driven mostly by assumed improvements in
patients, respectively, with the remainder pending data entry. 96.3% case-finding and intensifying treatment protocols. Medical personnel
(2325/2413) of registered patients have a confirmed cancer diagno- were assumed to be understaffed in year-one, therefore, in addition to
sis, with 79.7% (1854/2325) microscopically verified. Median age is 6.5 accounting for the rising number of patients, an increase of 10% was
years [interquartile range 3-10 years]; 58.0% are male (1516/2612). applied between years one and two, tapering off to 8%, 6% and 4% in
Lymphoid leukemias are the most common malignancy among regis- the following years.
tered cases (835/2325, 35.9%), followed by acute myeloid leukemias Conclusions: The Global Initiative for Childhood Cancer has increased
(202/2325, 8.7%) and nephroblastoma (148/2325, 6.3%). interest in incorporating childhood cancer into NCCPs. By leverag-
Conclusions: The SJCARES Registry Collaborative network is sustain- ing published real-world data from LMICs, we designed a collabo-
able and provides a foundation for collaborative research. The number rative budget tool that ensures NCCPs will not exclude childhood
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S433 of S635

cancer in their budgeting. Our tool offers a feasible solution to meet trie CHU Gabriel Touré, Pédiatrie, BAMAKO, Mali; 6 CHU JRA, Pediatrie,
urgent needs while providing a bridge for more comprehensive, and Antananarivo, Madagascar; 7 Faculty of Medicine and Biomedical Sci-
time-consuming, costing initiatives. ences of the University of Yaounde 1, Paediatrics, Yaounde, Cameroon;
8 8Cliniques Universitaires de Kinshasa, Pediatrie, Kinshasa, Congo; 9 ncer
Surveillance Branch, International Agency For Research On Cancer, LYON,
EP618 / #1799 TUMORS IN NEONATES, EPIDEMIOLOGICAL France; 10 Université Paris Cité, INSERM UMR 1153, Epidémiologie Des
PROFILE AND MANAGEMENT. WHAT IS THE PROGRESS IN Cancers Des Enfants Et Des Adolescents (epicea), Paris, France
ALGERIA?
Background and Aims: In sub-Saharan Africa, data concerning pedi-
Nabila Bouterfas, Ayda Mohand-Oussaid, Zineb Nafai, Messaouda atric oncology are collected by few regional population based reg-
Haridi, Keltoum Nafissa Benhalla istries. In January 2016, the French African Pediatric Oncology Group
Faculty of Medicine of Algiers, Medecine, Rue Lieutenant Mohamed Benarfa, (GFAOP) working in 18 countries set up an international hospital based
El Biar, Algeria cancer registry collecting data on line. Here we report on the charac-
teristics of children attending 7 units from 2017 to 2019 to document
Background and Aims: Background Discovery of neoplasia in needs of dedicated pediatric oncology units.
neonates often poses a diagnostic and therapeutic problem because of Methods: Units that registered at least 120 new cases from 2017-
age of onset, organs functional immaturity and therapeutic procedures 2019 were selected. Units included Abidjan, Bamako, Dakar, Kinshasa,
related risks. Objectives This study aims to analyze the epidemiological Ouagadougou, Tananarive, and Yaoundé. Diagnosis was confirmed
profile and to evaluate the outcome. on radiological, histological or hematological examination, stage was
Methods: Material and methods A retrospective study is conducted examined according to the Toronto Guidelines. Information concerning
between January 2003 and December 2021 at the pediatric unit “Pédi- resources was provided by the GFAOP, or given by units.
atrie A” of CHU Béni Messous, including all patients diagnosed with Results: Data for 2715 children with a cancer attending the units
neonatal tumor. Patients are evaluated according to neoplasia nature over the 3 years was analyzed. The mean age was 6.6; the most fre-
by biochemical studies and imaging exploration, with a follow up time quent age group was 1-4 age group. The annual number of registered
varying from 6 months to 18 years. cancers ranged from 42 in Tananarive to 217 in Ouagadougou. Doc-
Results: We identified 80 patients (43 girls and 37 boys) aged between tor patient ration was above 1 doctor to 75 children in 6 of the
0 and 28 days. 20% were diagnosed in utero. Patients presented with units. The most common malignancies were Burkitt lymphoma (BL)
solid tumor in the vast majority, with leukemia in only two patients. N=563, retinoblastomas (RB) N=438, nephroblastoma N=433, and
The histologic diagnoses were, neuroblastoma (n = 47, 58.7%), ter- acute lymphoid leukaemia (ALL) N=349. Ouagadougou had the largest
atoma/germ cell tumor (n = 17, 21.2%), angioma 6.5%, kidney tumor proportion for both LB and RB with 37% and 27% respectively. ALL
in 3.8%. Two patients were diagnosed with hereditary retinoblastoma represented 63% of all leukemias. Thirty-four brain neoplasms were
through screening (2.5%). There was no patient with primary central registered. Data concerning stage was available for 91% of cases.
nervous system. Sixty patients(75%) underwent treatment includ- Conclusions: The small number of brain tumors reflects the difficul-
ing chemotherapy or/ and surgery whereas 25% of them were just ties of countries to diagnose, often due to lack of essential equipment.
observed. Overall survival is 82.5%. We are aware that the presented pattern may be a skewed represen-
Conclusions: Tumoral pathology in neonatal period is dominated by tation of childhood cancer incidence, as the data come from hospital
solid tumors, particularly neuroblastoma. Management has signifi- registries. Our data highlights the need for development of pediatric
cantly improved in Algeria thanks to advances in imaging and the oncology services, including both diagnostic and human resources and
adoption of the observational strategy concept. the development of hospital and regional population-based cancer
registries.

EP619 / #1619 CHARACTERISTIC OF CHILDREN ATTENDING


PEDIATRIC ONCOLOGY UNITS IN 7 SUB-SAHARAN AFRICAN EP620 / #1885 IMPLEMENTING AND OPTIMIZING THE
COUNTRIES CLINICAL RESEARCH COORDINATOR ROLE IN SIXTEEN
PEDIATRIC HEMATOLOGY AND ONCOLOGY UNITS IN MEXICO
Mallon Brenda1 , Rolande Kabore2 , Line Couitchere3 , Fatou Diagne4 ,
Tankélé Dackono5 , Mbolanirina Narison6 , Angèle Hermine Pondy Erika Casillas-Toral1 , Karla Guerrero-Gomez2 , Miriam
Ongotsoyi7 , Aleine Budiongo8 , Eva Steliarova-Foucher9 , Jacqueline Gonzalez-Guzman3 , Karime Salas-Villa4 , Araceli Lopez-Facundo5 ,
Clavel10 Oscar Gonzalez Ramella6 , Angela Carrillo7 , Maria Del Carmen Esmer
1 GFAOP, Gustave Roussy, Paris, France; 2 2Centre Hospitalier Universi- Sánchez8 , Maria Del Pilar Cubria-Juarez9 , Patricia Loggeto10 , Selene
taire Yalgado Ouédraogo, Pediatrie, Ouagadougou, Burkina Faso; 3 CHU Portillo-Zavala11 , Naomi Echeandia-Abud3 , Paola Friedrich3
TREICHVILLE, Oncology Pediatry Unit, ABIDJAN, Côte d’Ivoire; 4 Aristide 1 Casa de la Amistad para Niños con Cáncer, Crc Regional Manager, Méx-
Le Dantec Hospital, Pediatry, Dakar, Senegal; 5 Service d’Oncologie Pédia- ico City, Mexico; 2 Casa de la Amistad para Niños con Cancer, Project
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S434 of S635 ABSTRACTS

Coordinator, Ciudad de Mexico, Mexico; 3 St. Jude Children’s Research Paul D’Alessandro1 , Jessica Davis1 , Jim Potts2 , Rebecca J Deyell3
Hospital, Global Pediatric Medicine, Memphis, United States of America; 1 British Columbia Children’s Hospital, University of British Columbia,
4 Hospital Pediátrico de Sinaloa "Dr. Rigoberto Aguilar Pico", Hemato- Division Of Pediatric Hematology/oncology/bone Marrow Transplant, Van-
oncology, Culiacan, Mexico; 5 Hospital Materno Infantil del Instituto de couver, Canada; 2 BC Children’s Hospital, Division Of Pediatric Hematol-
Seguridad Social del Estado de México y Municipios, Hemato-oncology, ogy/oncology/bmt, Vancouver, Canada; 3 British Columbia Children’s Hos-
Toluca, Mexico; 6 Hospital Civil de Guadalajara Dr. Juan I Menchaca., pital / University of British Columbia, Pediatrics, Division Of Pediatric
Hemato- Oncología Pediátrica., Guadalajara, Mexico; 7 St Jude Children’s Hematology Oncology Bmt, Vancouver, Canada
Research Hospital, Global Pediatric Medicine, Memphis, United States of
America; 8 Hospital Infantil Teleton de Oncologia, Research, Queretaro, Mex- Background and Aims: Survival outcomes are poor for paediatric
ico; 9 Hospital Infantil Teleton de Oncologia, Pediatric Oncology, Queretaro, oncology patients with relapsed, refractory or progressive disease
Mexico; 10 St. Jude Children’s Research Hospital, Global Pediatric Medicine (RRPD.) Access to early phase clinical trials in Canada is limited. Dis-
Department, Memphis, United States of America; 11 Hospital Infantil de tance as a factor for enrolment on phase I/II trials in RRPD remains
Especialidades del Estado de Chihuahua, Hemato-oncology, Ciudad Juarez, unexplored. Our primary aim was to determine if distance from home
Mexico residence to study site predicted odds of local phase I/II trial enrol-
ment among eligible patients on univariate analysis. Secondary aims
Background and Aims: Clinical research coordinators (CRC) play a included multivariable analyses for impact of distance adjusted for
pivotal role in research study implementation. The CRC role was incor- other variables.
porated in 2017 into Mexico in Alliance with St. Jude’s multidisciplinary Methods: Paediatric oncology patients with RRPD followed at the only
pediatric hematology and oncology (PHO) teams. It replaced the exist- tertiary paediatric cancer centre in British Columbia were identified
ing data manager role as a strategy to address the medical staff’s lack of retrospectively from a relapse registry. Distance was calculated using
protected time for research. We describe the process of implementing postal code and geocoding software (CDXZipStream v5.1.0.3, CDX
and optimizing the CRC role in sixteen MAS collaborating centers. Technologies, Randolph, NJ) and analyzed as a categorical variable.
Methods: The MAS operational team and local PHO team lead led the Results: Between January 2015 and July 2021, 266 patients experi-
recruitment process for each institution. CRCs were gradually incor- enced 396 RRPD events: 75 (28.2%) were eligible for a local phase
porated to assist research and quality improvement (QI) project imple- I/II trial at least once; 191 (71.8%) were never eligible. Of 61 patients
mentation. In December 2021, we used the Institute for Healthcare offered enrolment at first eligible RRPD event, 69% were male, 46%
Improvement Model as a strategy to optimize the CRC role. Virtual had a primary CNS tumour, and median age was 11.8y (range 2.3-
meetings were conducted with an expert panel, core team, and CRC 22.8y.) There was no difference in distance category between those
regularly (biweekly, weekly, and monthly respectively). We developed who enrolled and those who declined (p=0.86.) Males were more likely
a measurement strategy, a driver diagram and carried out several Plan- to enrol (p=0.016). Two-year event-free survival (EFS) and overall
Do-Study-Act (PDSA) cycles. A de-identified survey assessed CRCs and survival were 39.8%±13.3% and 52.6%±13.6%, respectively. EFS/OS
stakeholders’ satisfaction after 14 months of implementation. did not differ with enrolment status, but varied dramatically by dis-
Results: Sixteen healthcare facilities in Mexico have adopted the CRC ease group (p=0.0002; p<0.0001.) Fourteen (5.3%) patients were not
role. Each CRC supports an average of 3 (range 1-5) projects; only offered enrolment per physician preference. Fifteen patients were
50% have a full-time position. We have conducted a total of 48 vir- treated on phase I/II trials at outside centres, and half (47%) enrolled
tual meetings and implemented 40 change ideas targeting five primary on trials that we subsequently opened locally.
drivers: effective documentation systems (32%), defined processes and Conclusions: In British Columbia, 22.9% of patients with RRPD
responsibilities (28%), effective study management (20%), teamwork were offered, and 11.3% enrolled on local early phase trials. Dis-
& communication (12%), and empowerment (8%). CRC’s project data tance from home did not impact enrolment, suggesting that patients
completion and accuracy have been above 94.1%. Ninety-two percent and families make difficult choices to access limited early phase
of CRCs agreed that the training provided and change ideas imple- trials.
mented had improved their performance as CRCs. All stakeholders
were satisfied with the outcomes of the project.
Conclusions: We have demonstrated the feasibility of incorporating EP622 / #1017 THE UNIVERSITY OF CAPE TOWN
the CRC role in PHO units in Mexico. This role may serve as a valuable PAEDIATRIC CANCER DATABASE: RESULTS FROM THE FIRST
model for other countries and clinical departments. YEARS 2019-2021

Alan Davidson1 , Jennifer Moodley2 , Komala Pillay3 , Marc Hendricks1 ,


EP621 / #623 EFFECT OF DISTANCE TO TERTIARY CENTRE Annemie Stewart4 , Jeannette Parkes5
ON ENROLMENT OF PAEDIATRIC PATIENTS WITH 1 UCT and RCWMCH, Haematology/oncology Service, Cape Town, South
RELAPSED/REFRACTORY/PROGRESSIVE CANCER ON PHASE I/II Africa; 2 University of Cape Town, Cancer Research Initiative, Faculty Of
TRIALS IN BRITISH COLUMBIA Health Sciences, Cape Town, South Africa; 3 University of Cape Town,
Anatomical Pathology, Cape Town, South Africa; 4 University of Cape Town,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S435 of S635

Clinical Research Centre, Cape Town, South Africa; 5 University of Cape Université Paris-sud, Paris, France; 5 Bambino Gesù Children’s Hospital„
Town, Department Of Radiation Oncology, Cape Town, South Africa Surgery, Rome, Italy

Background and Aims: The paediatric oncology multidisciplinary Background and Aims: The occurrence of cancer in newborns within
team at the University of Cape Town (UCT) developed a research- the first 28 days of life is uncommon with different clinical presentation
ready database to describe epidemiological profiles, biology, treatment from other age groups.
and outcomes, and determine factors associated with presentation and Methods: Data were obtained through a retrospective chart review
outcome. of patients admitted at Bambino Gesù Children’s Hospital from
Methods: A REDCap database was developed with a Cancer Asso- 1st January 2006 to 31st December 2021. All children histologically
ciation of South Africa grant, which employed an administrator to diagnosed with a tumor within 28 days of life were included. The
consent all new patients, record demographic and social information, medical records of patients were reviewed.
and capture clinical information in real time. Results: We describe the first Italian series over 15-year period of
Results: There were 212 children consented from 2019 to 2021: 109 patients affected by neoplastic disease diagnosed within the first 28
girls and 103 boys. Ages ranged from 1 day to 15.98 years. Only days of life; 74 newborns were diagnosed with neonatal tumor repre-
15% of these families had medical insurance, 16% lived in informal senting 1,5 % of the cancer population in the same period: a prevalence
housing and 12% did not have access to piped water. Seventy-four of germ cell tumor (55%) and neuroblastoma (16%) was observed.
families (35%) reported a relative with cancer, including seven first Surgery was performed in 80% of patients while chemotherapy was
degree relatives (one each from a retinoblastoma and a DICER family) necessary in about 20%. The 5 years OS exceeded 90%; to underline
and two cousins with acute myeloid leukaemia (AML). There were no that deaths related to treatment is the major concern representing
specific or strong correlations between incident and associated can- 80%. A genetic/syndromic condition was detected in 16% of popula-
cers. Patient diagnostic groups included leukaemia (33%), lymphoma tion, and a cancer predisposition syndrome (CPS) was identified in 7
(11%), CNS tumours (14%), embryonal tumours (20%), sarcomas (12%) patients.
and germ cell tumours (6%). Most patients with solid tumours (72%) Conclusions: This series represents the first and largest Italian case
had advanced disease at diagnosis. The estimated 2-year overall sur- series of patients with cancer in the neonatal period. Although it is
vival was significantly different (p=0.013) by disease group: acute a retrospective evaluation, it provides many insights for prospective
lymphoblastic leukaemia (78%), acute myeloid leukaemia (55%), lym- studies. Neonatal tumors represent a rare occurrence with a com-
phoma (96%), central nervous system tumours (60%), neuroblastoma plex genetic landscape considering both CPS and syndromic condition;
(89%), retinoblastoma (75%), Wilms tumour (100%), hepatoblastoma extensive genetic studies should be addressed in all cases in order
(86%), rhabdomyosarcoma (52%) and germ cell tumours (100%). Out- to achieve a better management in term of treatment and for family
comes were poorer for children living in informal housing (61% vs 80%; counselling. The management should be considered with caution and
p=0.04) and without piped water (61% vs 79%; p=0.058). Children in experienced centers considering the impressive 5% mortality rate
with a family history of malignancy did not have significantly poorer related to treatment; in our experience the treatment related mortal-
outcomes. ity rate is mainly due to infective complications.Above all, the outcome
Conclusions: Active inclusion of children and families in a robust is favorable with an excellent OS survival that exceeded 90%, while
database maintained in real time can provide a research-ready plat- relapse or progression is rare.
form for interrogating cohort-specific factors impacting childhood
cancer outcomes, and generate new areas for research.
EP624 / #1845 THE CRITICAL ROLE OF ACADEMIC
CLINICAL TRIALS IN PEDIATRIC CANCER DRUG APPROVALS:
EP623 / #1897 THE FIGHT JUST BORN- NEONATAL CONSIDERATIONS FOR FIT FOR FILING TRIALS
CANCER: RARE OCCURRENCE WITH A FAVORABLE OUTCOME
BUT CHALLENGING MANAGEMENT Bram De Wilde1 , Elly Barry2 , Elisabeth Fox3 , Dominik Karres4 , Mark
Kieran2 , John Manlay5 , Donna Ludwinski6 , Gregory Reaman7 , Pamela
Maria Antonietta De Ioris1 , Francesco Fabozzi1 , Mariachiara Lodi2 , Kearns8
Giulia Vitali3 , Maria Debora De Pasquale3 , Giada Del Baldo2 , Rachid 1 Ghent University Hospital, Department Of Pediatric Hematology Oncology

Abbas4 , Alessandro Crocoli5 , Chiara Iacusso5 , Giuseppe Maria And Stem Cell Transplantation, Ghent, Belgium; 2 Day One Biopharmaceuti-
Milano3 , Annalisa Serra1 , Angela Mastronuzzi1 cals Inc, Day One Biopharmaceuticals Inc, San Francisco, United States of
1 IRCCS Bambino Gesù Children’s Hospital, Department Of Hematol- America; 3 St. Jude, Children’s Research Hospital, Memphis, United States
ogy/oncology, Cell And Gene Therapy, Rome, Italy; 2 IRCCS Bambino of America; 4 European Medicines Agency, Paediatric Medicines Office,
Gesù Children’s Hospital, Department Of Paediatric Haematology/oncology, Scientific Evidence Generation Department, Human Medicines Division,
Rome, Italy; 3 Bambino Gesù Childrens’ Hospital, Department Of Pediatric Amsterdam, Netherlands; 5 Pfizer Inc, Pfizer Inc, New York, United States
Hematology And Oncology And Of Cell And Gene Therapy, Scientific Insti- of America; 6 Solving Kids’ Cancer US, Director Of Research Advocacy,
tute For Research And Healthcare (irccs)„ Roma, Italy; 4 CESP, INSERM, New York, United States of America; 7 U.S. Food and Drug Administration,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S436 of S635 ABSTRACTS

Oncology Center Of Excellence, Office Of The Commissioner, And Office Of D’investigation Clinique De Rennes), Rennes University, Rennes, France;
Oncologic Diseases, Center For Drug Evaluation And Research, Silver Spring, 5 CHU de Brest, Department Of Pediatric Hematology And Oncology, Brest,
United States of America; 8 University of Birmingham, Cancer Research Uk France; 6 CHU de Tours Clocheville, Department Of Pediatric Hematology-
Clinical Trials Unit, Birmingham, United Kingdom oncology, Tours, France; 7 CHU de Poitiers, Pediatric Oncology Unit, Cic 802
Inserm, Poitiers, France; 8 CHU de Nantes Hôtel Dieu, Department Of Pedi-
Background and Aims: Despite changes in both the European and atric Hematology-oncology, Nantes, France; 9 CHU de Rennes Sud, Depart-
American legislation aiming to promote drug development for chil- ment Of Pediatric Hematology And Oncology, Rennes, France; 10 University
dren, availability of approved or licensed new drugs for the treatment Hospital of Caen, Service De Chirurgie Digestive, CAEN, France; 11 University
of cancer in children still lags significantly compared to cancer drug Hospital of Caen, Pediatric Surgery Department, CAEN, France
approval in adults. Therapeutic innovation is traditionally dependent
upon the pharmaceutical industry who own the assets, while in paedi- Background and Aims: The posttreatment period is a key part of
atric oncology, academic consortia dominate the trials landscape. Data the management of pediatric cancer care. At this period, psychosocial
repurposing from these academic trials to obtain regulatory approval effects (scholarly and psychological difficulties) have been described in
of a drug has historically not been a smooth process. pediatric cancer patients and can be prognostic for the success of social
Methods: We convened a Fit for Filing Working Group, under the reintegration. Psychosocial effects and their impact may be related to
auspices of the multi-stakeholder platform ACCELERATE. The group the household’s socioeconomic background. The aim of this study was
included representatives from academia, pharmaceutical industry, to estimate psychosocial difficulties during the posttreatment period
EMA, FDA and patient advocacy. Via interactive case discussions on based on a social deprivation score.
trial data repurposing, surveys and expert consultation we defined Methods: This study is based on a prospective multicentric study
the challenges and formulate recommendations to facilitate academic- database, and focused on the children who had received psychosocial
sponsored trial design and conduct to address the expectations of evaluation during their follow-up after cancer treatment since 2013 to
pharmaceutical companies and Regulatory Authorities. 2020. We retrieved data on their learning and psychological difficul-
Results: We propose a set of recommendations that would facili- ties. Socioeconomic status of the household was estimated by a social
tate more effective academic-industry partnerships and enable the deprivation score.
results of academic-sponsored trials to satisfy a regulatory obligation Results: 1003 patients were analyzed. Learning difficulties at school
or contribute to a submission for marketing authorisation by EMA were noted in 22% of patients. A greater social deprivation was sig-
or approval by FDA. These recommendations cover the collaboration nificantly associated with learning difficulty (OR=1.09 per unit of the
itself but also the specific aspects of trial documentation, essential deprivation score). Tumor relapse, treatment with hematopoietic stem
data, data management and trial resources. The recommendations will cell transplantation, and diagnosis of a CNS tumor remained signifi-
result in an educational resource for the community. cant risk factors. In the subgroup analysis of children with CNS tumors,
Conclusions: To expedite the process of bringing novel treatments to learning difficulties were increased and associated with greater social
children we should grasp the opportunities presented by recent legisla- deprivation. By contrast, psychological difficulties were not associated
tion changes. Successful collaboration between industry and academic with the deprivation score.
investigators with early input from regulatory agencies is necessary Conclusions: There is a link between SE status and learning difficulties
and should include advocate engagement to ensure patient-centric in survivors of childhood cancer. Further investigations should be car-
focus is encouraged. The recommendations of our working group ried out to confirm these results for children with CNS tumors, which is
should help all stakeholders achieve the end goal of marketing autho- the population of the greatest concern.
risation of a product for children with cancer sooner, resulting in better
access to innovation for children with cancer.
EP626 / #948 REVIEW ON THE FIRST 2 YEARS OF
OPERATION OF THE CRDCE
EP625 / #12 IMPACT OF THE SOCIAL DEPRIVATION ON
THE PSYCHOSOCIAL DIFFICULTIES OF PEDIATRIC CANCER Cherif Dial, Gabriel Deguenonvo, Khady Ngom
SURVIVORS: A PROSPECTIVE MULTICENTRIC STUDY Cheikh Anta Diop University, Anatomopathology, Dakar, Senegal

Fanny Delehaye1,2 , Olivier Dejardin1 , Isabelle Pellier3 , Ludivine Background and Aims: The Reference Center for the Diagnosis of
Launay1 , Maxime Esvan4 , Damien Bodet2 , Liana Carausu5 , Julien Childhood Cancers (CRDCE) founded in 2020, aimed to centralize
Lejeune6 , Frédéric Millot7 , Caroline Thomas8 , Virginie Gandemer9 , and complete histological results for pediatric cancers as needed with
Arnaud Alves10 , Julien Rod11 immunohistochemistry.
1 University Hospital of Caen, U1086 Inserm “anticipe”, CAEN, France; Methods: It was a retrospective study collecting epidemiological
2 University Hospital of Caen, Pediatric Hematology And Oncology, CAEN, aspects of pediatric tumors within the first two years of activity.
France; 3 University Hospital of Angers, Department Of Pediatric Hema- We used standard histopathologic method and immunochemistry if
tology And Oncology, Angers, France; 4 CHU de Rennes, Cic 1414 (centre necessary, to confirm the diagnostic.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S437 of S635

Results: During the period of study, 231 samples from children aged ers, pediatricians, nurses, midwives, and other health technicians, as
0 to 16, recorded over a period of time from January 2020 to Decem- part of 33 training workshops. Seventy-six HCG will be participat-
ber 2021, including 126 boys (54.55%) and 105 girls (45.45%) with a ing in a remote teaching program in April-May 2022. Post formative
gender ratio of 1.2. The number of tumor lesions was 171, with 135 onsite evaluation in two pilot districts in Senegal including 44 HCG
cancers as in 78.95%, and 36 benign tumors as in 21.05%. As for the showed the referral of 21 patients. Experimental digital technology is
rest of the lesions that were non-tumor, a number of 60 as in 25.97% under development to support HCG in swiftly referring patients to ser-
of the total samples. The distribution of cancers by gender was lean- vices for children with cancer in two countries (Senegal and Burkina
ing towards boys (73) with a gender ratio of 1.18. Kidney cancer was Faso).
the most common, with nephroblastoma as the predominant histolog- Conclusions: the implementation of effective tools to enhance early
ical type in 41 cases (30.37%) followed by retinoblastoma in 27 cases diagnosis may improve the survival of children with cancer in Sub-
(20%) and lymphomas in 6 cases (4.44%). The immunohistochemical Saharan Africa. Demonstrating such an impact will need further devel-
study was necessary on 32 samples as in 13.85%. Immunohistochem- opment in additional regions and countries in the coming years to
istry was performed with the available antibodies and was conclusive demonstrate its impact on survival.
in 26 cases. Although, this study could not determine the diagnosis for
the rest yet, which was particularly due to insufficient antibody panel.
Conclusions: The results were obtained within a maximum of 10 days, a EP628 / #1959 CHILDHOOD CANCER AT KINSHASA:
review concluded during a local anatomo-pathological staff along with SITUATION IN THE ONLY CHILDHOOD CANCER CENTRE IN
difficult cases, were sent to the i-PATH network for advice. Thanks to THE CONTEXT OF WHO 2030 OBJECTIVES
the GFAOP network.
Aleine Budiongo1 , Nina Domo1 , Karim Assani2 , René Ngiyulu1 , Jean
Lambert Ehungu1
EP627 / #1359 IMPLEMENTATION OF EARLY DIAGNOSIS 1 Kinshasa University Hospital, Pediatrics, Kinshasa, Congo, Republic of;
PROGRAM FOR FIVE CHILDHOOD CANCERS IN SUB-SAHARAN 2 University hospital of Kinshasa, DR Congo and AMCC, Pediatrics, Kin-
AFRICA. A FINAL REPORT FROM GFAOP shasa, Congo, Republic of

Fatou Dieye1 , Mame Diouf2 , Jean Michon3 , S. Aimée Kissou4 , Background and Aims: Cancer is one of the leading causes of death
Aichatou Mahamadou5 , Aissata Barry6 , Line Couitchere7 among children and adolescents in high-income countries. In sub-
1 Franco-African Group on Paediatric Oncology, Paris, Villejuif France, Sene- Saharan Africa, there is lack of data about childhood cancer and its
gal; 2 Aristide Le Dantec Hospital, Pediatric Oncology, Dakar, Senegal; burden is not well known. We describe a single childhood cancer centre
3 GFAOP, Villejuif, PARIS, France; 4 Centre Hospitalier Universitaire Sourô situation in Kinshasa from January 2018 and June 2021.
Sanou, Bobo-dioulasso, Bobo-Dioulasso, Burkina Faso; 5 Centre National de Methods: This is a cross-sectional and descriptive retrospective study
Lutte contre le Cancer, Niamey, Niamey, Niger; 6 Hôpital National Donka, conducted at the pediatric hematology-oncology unit of the Depart-
Conakry, Conakry, Guinea; 7 CHU TREICHVILLE, Oncology Pediatry Unit, ment of Pediatrics of the university hospital of Kinshasa.
ABIDJAN, Côte d’Ivoire Results: We managed 285 children (163 boys and 122 girls) with
median age of 5.74 years (0.15 and 18 years). Retinoblastoma,
Background and Aims: This presentation is an update of the oral pre- leukemia, non-Hodgkin’s lymphoma and nephroblastoma were the
sentation given during SIOP AFRICA 2022 in Sub-Saharan Africa, like most frequent cancers with respectively 74 cases (25.96%), 47 cases
in other low-income countries, delayed diagnosis is the first cause of (16.49%), 45 cases (15.79%) and 42 cases (14 .74%). In addition,
death by cancer in children. Developing early diagnosis tools, train- 137 (48.07%) had access to treatment, 148 (51.93%) abandonned, 4
ings healthcare givers (HCG) as well as raising awareness among (1.40%) were lost to follow-up and 87 (30.53%) died. Remission and
populations is paramount to improve survival rates. relapse rates were 8.07% and 2.81% respectively.
Methods: two workshops on designing and validating childhood cancer Conclusions: Childhood cancers remain a public health threat in the
education and awareness tools were conducted in 2019 with pediatric DRCongo. Sufficient knowledge of its epidemiological situation may
oncology experts and health authorities from Senegal. Educational help to develop effective policies that can improve its cure rate in line
modules on detection of early signs leading to the diagnosis of five main with WHO 2030 objectives.
children’s cancers (Retinoblastoma, Acute Lymphoblastic Leukemia,
Wilms Tumor, Burkitt and Hodgkin Lymphoma) were developed and
harmonized. These modules (manuals, flyers, posters), intended for EP629 / #782 GEOGRAPHIC AND SOCIOECONOMIC
general practitioners, nurses and other healthcare givers have been ANALYSIS OF PEDIATRIC CANCER CASES IN TANZANIA
used to train trainers and then to train healthcare givers. In 2022, these
modules have been digitalized for online teaching. Victoria Donohue1 , Luke Maillie2 , Sebastian Sanchez1 , Heronima
Results: during the 2019-2022 campaign in eight countries in sub- Joas3 , Franco Afyusisye3 , Furaha Serventi4 , Lulu Chirande5 , Shakilu
Saharan Africa, 1110 HCG were trained, including general practition- Jumanne6 , Trish Scanlan7 , Kristin Schroeder3,8,9
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S438 of S635 ABSTRACTS

1 Duke University Medical Center, School Of Medicine, Durham, United Suh3 , Andrew Wellman3 , Monika Metzger1 , Miguel Bonilla5 , Manoo
States of America; 2 Icahn Mt Sinai, School Of Medicine, New York, United Bhakta6 , Michael Sullivan7 , Nickhill Bhakta8
States of America; 3 Bugando Medical Centre, Oncology, Mwanza, Tanzania; 1 St Jude Children’s Research Hospital, Department Of Oncology, Depart-
4 Kilimanjaro Christian Medical Centre, Oncology, Kilimanjaro, Tanzania; ment Of Global Pediatric Medicine, Memphis, United States of America;
5 Muhimbili National Hospital, Pediatrics And Child Health, Dar es Salaam, 2 Albert Einstein Medical Center, Pediatrics, Philadelphia, United States of
Tanzania; 6 University of Dodoma, Oncology, Dodoma, Tanzania; 7 Muhimbili America; 3 St. Jude Children’s Research Hospital, Information Services, Mem-
National Hospital, Pediatric Oncology, Dar Es Salaam, Tanzania; 8 Duke phis, United States of America; 4 St. Jude Children’s Research Hospital,
University Medical Center, Pediatric Oncology, Durham, United States of Global Pediatric Medicine, Memphis, United States of America; 5 St. Jude
America; 9 Duke University Medical Center, Global Health, Durham, United Children’s Research Hospital, Department Of Global Pediatric Medicine,
States of America Memphis, United States of America; 6 University Of Tennessee College of
Medicine – Chattanooga, Oncology, Chattanooga, United States of Amer-
Background and Aims: The Tanzanian National Childhood Cancer ica; 7 Royal Children’s Hospital, Children’s Cancer Center, Department Of
Network (NCCN) includes eleven hospitals, each with individual clin- Paediatrics, Melbourne, Australia; 8 St Jude Children’s Research Hospi-
ical databases. To determine the geographic variation of presenting tal, Department Of Global Pediatric Medicine, Memphis, United States of
patients and potential regional socioeconomic influences, it is impor- America
tant to merge this data into a single National registry.
Methods: Demographic data, diagnosis, and home region was Background and Aims: Resource adapted clinical guidelines such
extracted for all patients presenting to the Tanzanian NCCN in as ARIA (Adapted Resource and Implementation Application) have
2019. Regional socioeconomic data was extracted from published the potential to improve global pediatric cancer care and close the
sources. Geographic distribution of pediatric cancer was determined cancer survival gap. User feedback is a critical preimplementation
and retrofitted against regional socioeconomic data to determine strategy to overcome barriers related to design quality and pack-
correlation. aging, and to improve innovation, utilization, and dissemination. We
Results: In 2019, a total of 826 patients aged <19 presented to the hypothesized that systematic involvement of end-users in the design
NCCN. 58.7% were male and mean age was 6.45 +/- 4.47 Yr. The process will lead to a better design and superior usability of the ARIA
most common diagnoses were Wilms Tumor (n=134), retinoblastoma platform.
(n=133), and ALL (n=125). The regions patients most commonly pre- Methods: Beta-testing was conducted with multidisciplinary global
sented from were Dar es Salaam (n=107), Mwanza (n=73), and Mara collaborators including a 60-minute tutorial followed by a 45-
(n=44), coinciding with the location of the three comprehensive child- question electronic survey. The survey was developed to evaluate
hood cancer treatment hospitals. Regional variations were noted, with the functionality, visual design, and content language of the plat-
hotspots for CNS tumors in Arusha (0.34/100,000) and retinoblastoma form and included 10 close-ended questions from the System
in Geita (1.0/100,000). Regions with the highest overall rates of can- Usability Scale, a simple tool that assesses high level need for
cer positively correlated to the National census reported literacy rate, training, complexity, and usability of a system. Responses included
mobile phone ownership, cement flooring, and percent unemployment. a combination of Likert-type scale responses and open-ended
Conclusions: There is a regional variation among pediatric cancer diag- questions.
noses in Tanzania. Regional cancer incidence positively correlated with Results: Seventy-eight collaborators attended the introductory tuto-
urban designation, likely due to increased diagnostic testing in larger rial with 1,019 unique page views during the test period. Fifty-
cities. However, an inverse correlation with incidence was seen with five respondents completed the survey (71% response rate), includ-
regional percent employment, which will need to be evaluated on an ing 50% early career professionals (< 45 years old) with diverse
individual basis to confirm this finding. This study further delineated geographic representation (15% Africa, 29% Central and South
the regional disparities in pediatric cancer presentation in Tanzania, America, 11% Eurasia, 5% Middle East, 13% North America, 27%
highlighting the need for research into potential environmental and Southeast Asia/Oceania). Themes from real-time feedback during
socioeconomic influences on the observed distribution, and the need the tutorial and 58 submitted comments were categorized. Users
for increased knowledge and diagnostic capacity in regions further found the platform interesting (93%), easy to learn how to use
away from major treatment centers. (89%), and 98% would recommend ARIA to other providers. Ninety-
six percent of respondents agree that ARIA is likely to increase
awareness of the importance of guidelines in pediatric cancer
EP630 / #1288 ADAPTED RESOURCE AND management.
IMPLEMENTATION APPLICATION (ARIA) GUIDELINES: AN Conclusions: Diverse user will improve the effectiveness and plat-
APPROACH TO BETA TESTING OF THE WEB-BASED PLATFORM form satisfaction of the ARIA platform. This is an important con-
sideration for clinical guideline development as it helps tailor the
Caitlyn Duffy1 , Jeremie Hassan2 , Darrell Gentry3 , Sharmeen design, and increase utilization, and impact of these tools on a global
Hussain4 , Andrew Pappas4 , Komal Sodhi3 , Courtney Staples5 , Ed scale.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S439 of S635

EP631 / #1547 THE ROLE OF TIMELY INTERVENTIONS FOR Wasfa Farooq, Natasha Baig, Kulsum Kazi, Muntaha Banglani, Fatima
PATIENT ABANDONMENT IN A COHORT OF PEDIATRIC Habib, Sabrina Merchant, Afia Tul Quanita, Muhammad Rafie Raza
CANCER PATIENTS IN PAKISTAN The Indus Hospital and health Network, Paeds Oncology, Karachi, Pakistan

Natasha Baig, Shabnam Munir, Atoofa Najmi, Wasfa Farooq, Zaman Background and Aims: Despite improvement in 5-year survival
Khan, Sadia Imran, Muhammad Rafie Raza (79.6%) of childhood cancer patients in high-income countries, it
Indus Hospital and Health Network, Pediatric Oncology, Karachi, Pakistan remains a leading cause of disease-related death. In the developing
world, survival stands under 35%, reflecting shortfalls greater than
Background and Aims: Abandonment of childhood cancer treatment just availability of treatment. Discussions surrounding quality of life
compromises the survival of approximately one in seven children (QoL) are key in treatment decisions, physician-patient relationship
worldwide annually. Socio-demographic determinants include afford- and patient abandonment. Unfortunately, a holistic evidence-based
ability issues, long distances, lack of public transport, malnourishment, pediatric QoL measure does not exist for the South Asian context
parental education, daily wage dependency and perceived poor prog- Methods: Four trained volunteers conducted observations in five
noses. Despite free of cost treatment and specialized pediatric oncol- patient areas (in-patient wards, out-patient clinics, ER, day-care and
ogy centres in Pakistan, over 20% children still abandon therapy. Early play area) at Department of Paediatric Hematology/Oncology in Indus
interventions for accommodation support, parental counselling and Hospital & Health Network (IHHN), one of the largest facilities for
education, nutritional evaluation, monitoring missed appointments and childhood cancer in Pakistan. Semi structured interviews of providers
psychosocial involvement are effective ways to improve abandonment and patients were conducted to assess perceptions of treatment
rates in low-middle income countries. experience on QoL.
Methods: A prospective one-year study was initiated by the[DR1] Results: Observations in each area related to environment (hygiene,
Pediatric Oncology Department at Indus Hospital and Health Net- temperature, equipment, overcrowding, ambience, waste disposal and
work (IHHN). Newly registered patients (0 to 16 years) who presented use of technology), providers (demeanour, approachability, tone, eye
to IHHN in December 2021 were enrolled. Non-malignant, pallia- contact, body language, level of engagement and expressions), patients
tive treatment, expired and referred patients were excluded. Weekly (clinging to parents, physical appearance, confidence, eye contact,
meetings were conducted with stakeholders involved in patient care answering and inquiring, mood and expression) and their parents
(physicians, data managers, clinical coordinators, oncology nurses, (demeanour, mood, interaction with provider and child, self-expression,
psychosocial and welfare workers). Data was collected using a compre- body language). Provider interviews showed awareness of social, finan-
hensive case report form and recorded on password protected digital cial and disease related domains of patient QoL, however, emotions,
files. body image and self-esteem were not identified. Providers also cat-
Results: Eighty nine children were included. A large majority presented egorised mental and emotional wellbeing exclusively as a domain of
with leukemia and lymphoma. Over 70% resided outside Karachi, and psychologists. All providers identified guidance and counselling as their
12% were from Afghanistan. Average monthly household income was main tool in improving QoL of children. Parent responses were focused
PKR 24,608 (USD 133) and average number of siblings was 4. Most on social and financial issues and feelings of illness, reflecting limited
common paternal profession was labourer. Residence was provided prioritisation of emotions and relationships, especially in those from
to 12 families and translators to 19. Three children were enrolled in lower socioeconomic backgrounds.
IHHN’s school. Eight were called for missed appointments, 2 of whom Conclusions: As useful as measures of morbidity and mortality rates
returned. Nutritional consults were provided to 14 and psychoso- are, the need of the hour are outcome measures reflecting the patient’s
cial counselling sessions to 30. Thirteen families (7 solid tumours, 6 overall well-being and subjective evaluation of QoL and experience to
hematologic malignancies) abandoned treatment, mainly due to fear of create holistic policies and processes.
surgery and denial of disease. All abandoned families resided outside
Karachi or Pakistan.
Conclusions: Despite interventions to curb abandon- EP633 / #1623 IMPACT OF THE MY CHILD MATTERS
ment rates, pediatric oncology patients often refuse or GRANT ON HOLISTIC CARE IN PEDIATRIC ONCOLOGY UNITS
discontinue treatment. Further efforts are required to under- IN PAKISTAN
stand and address more specific determinants of patient
abandonment. Muhammad Rafie Raza1 , Wasfa Farooq2 , Bashir Ahmed Khan1 ,
Natasha Baig2 , Muhammad Shamvil Ashraf2
1 Indus Hospital and Health Network, Pediatric Oncology, Karachi, Pakistan;

EP632 / #1603 AN ETHNOGRAPHIC EVALUATION OF 2 The Indus Hospital and health Network, Paeds Oncology, Karachi, Pakistan

PROVIDER’S AND PATIENT’S CONCEPTUALIZATION OF


QUALITY OF LIFE IN A PAEDIATRIC HEMATOLOGY/ONCOLOGY Background and Aims: Paediatric oncology in LMICs faces sev-
FACILITY OF AN LMIC eral challenges while attempting to improve overall survival. Lack
of public awareness, inaccessible specialised or quality care, delayed
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S440 of S635 ABSTRACTS

diagnosis and treatment, patient abandonment and absence of an solid tumors. Leukemias followed the LR and HR criteria, according to
all-encompassing national action plan. The My Child Matters (MCM) age and leukemia, with HR < 12 months and > 10 years, and leukocytos
Grant by the Sanofi Espoir Foundation aimed at holistic improve- number >50,000/mm3. The early diagnosis program was administered
ment of childhood cancer in 8 paediatric oncology units in Pakistan online, from October to December/2021.
was re-awarded to the Indus Hospital & Health Network (IHHN) in Results: 534 patients were treated from 2014 to 2021, of these
2019. As a leading tertiary childhood cancer facility in Pakistan, IHHN 276 (52%) were considered HR and 258 (48%) were LR. There
worked with stakeholders to improve and build capacity for paediatric was a predominance of HR over LR in the years 2019, 2020 and
oncology services. 2021, different from the values found in the last five years
Methods: The IHHN team visited partnered units in March and Decem- from 2014 to 2018, where there was a predominance of LR over
ber 2021. A detailed performa was filled regarding the facility, patient HR.
care, data management, human resources, baseline nursing standards, Conclusions: Children and adolescents with cancer during this period
infection control standards and hand hygiene. Chemotherapy prepara- of the COVID19 Pandemic have been diagnosed with advanced dis-
tion was observed and feedback taken from focal persons regarding ease. These data allow us to say that the Covid 19 Pandemic associated
local challenges. Between both visits, educational initiatives were with the interruption of actions aimed at the guidance and training of
launched with goals and timelines created for each unit with constant health professionals may be related to the change in the presentation
communication and mentoring through nursing, physician and data and severity of the disease in children and adolescents with cancer in
managers. the western region of Paraná, Brazil.
Results: Out of eight POU’s, six showed drastic improvement in base-
line nursing standards. Each center now had a dedicated infection
control nurse enrolled in a diploma sponsored by the grant. Reg- EP635 / #583 THE PROFILE TOOL JOURNEY: RESULTS FROM
ular tumor boards and case presentations were initiated through THE SECOND PEDIATRIC HEMATOLOGY AND
IHHN shared telemedicine equipment. Three units now have full time ONCOLOGY(PHO) FACILITIES BETA TESTERS COHORT
psychologists available for patients and families, two of which are
supported by the grant. Childhood cancer registry data is maintained Miriam Gonzalez-Guzman1 , Heather Forrest1 , Patricia Belda1 , Gevorg
by grant appointed data operators, however, hospitals lack organized Tamamyan2 , Tatev Arakelyan2 , Hamidah Alias3 , C-Khai Loh3 , Joyce
medical recordkeeping systems leading to constant challenges. Short- Kambugu4 , Racheal Angom4 , Lorna Renner5 , Catherine Segbefia5 ,
age of chemotherapeutic and pain medications persisted across all Luiz Fernando Lopes6 , Cristina Amendola6 , Omar Chamdine7 , Maher
public hospitals along with increasing patient volumes. El Doussouki7 , Rawad Rihani8 , Sakher Obeidat8 , Susanna Rodriguez9 ,
Conclusions: Establishment of a widespread partnership model is Melissa Dazzell9 , Susi Susanah10 , Nur Sari10 , Syed Hamid11 , Sadia
essential for the promotion and sustainability of childhood cancer Imran11 , Janet Middlekauff1 , Emily Baum1 , Jeannette Kirby1 , Andrew
services in Pakistan. A rounded national action plan is needed to Pappas1 , Yichen Chen1 , Meenakshi Devidas1 , Carlos
implement and coordinate these efforts. Rodriguez-Galindo1 , Paola Friedrich1
1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Mem-
phis, United States of America; 2 Hematology Center after R.Yeolyan,
EP634 / #366 ANALYSIS OF COVID19 PANDEMIC AND THE Pediatric Cancer And Blood Disorders Center Of Armenia, Yerevan, Arme-
EARLY DIAGNOSIS PROGRAM FOR CHILDHOOD CANCER IN nia; 3 Universiti Kebangsaan Malaysia Medical Centre, Paediatrics, Kuala
THE WEST OF PARANÁ- BRAZIL Lumpur, Malaysia; 4 Uganda Cancer Institute, Pediatric Oncology, Kam-
pala, Uganda; 5 Korle Bu Teaching Hospital, Child Health, Korle Bu, Accra,
Carmem Maria Costa Fiori, Aline Rosa Ghana; 6 Barretos Children’s Cancer Hospital, Pediatric Hospital, Bairro
Hospital do Câncer de Cascavel- UOPECCAN, Pediatric Oncology, Cascavel, Paulo Prata, Barretos, San Paulo, Brazil; 7 King Fahad Specialist Hospi-
Brazil tal - Dammam, Pediatric Hematology Oncology, Dammam, Saudi Arabia;
8 King Hussein Cancer Center, Paediatric Hematology/oncology/bone Mar-
Background and Aims: In 2020, Brazil and the world faced an out- row Transplant, Amman, Jordan; 9 Georgetown Public Hospital Corporation,
break of severe acute respiratory syndrome coronavirus 2 (SARS-CoV- Pediatric, Georgetown, Guyana; 10 Dr. Hasan Sadikin General Hospital, Child
2). The early childhood cancer diagnosis program has been developed Health, Jawa Barat, Indonesia; 11 Indus Hospital Health Network, Pediatric
since 2008, training health professionals in the early recognition and Oncology, Karachi, Pakistan
referral of suspected cases and was interrupted in 2019 for restruc-
turing. OBJECTIVE- To analyze the number of cases referred to the Background and Aims: The Pediatric Oncology Facility Integrated
referral service with a diagnosis of cancer and the degree of staging of Local Evaluation (PrOFILE) has been developed and validated in four
patients and compare with the last 5 years before the pandemic. phases: consolidation, pre-alpha, alpha, and beta testing. The Full Ver-
Methods: Medical records of children under 19 years of age were ret- sion PrOFILE underwent Beta-1 testing in five PHO facilities in 2019.
rospectively evaluated from January 2014 to December 2021. Low Twelve institutions participated in Beta-2 from July 2021 to May
Risk (LR) (stage I and II) and High Risk (HR) (stage III and IV) criteria for 2022. Beta-2 aimed to assess the feasibility and utility of short-interval
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S441 of S635

reports, co-design descriptive reports, improve data visualization, and Full and Abbreviated versions of PrOFILE utilizing the RE-AIM
gather feedback on the benefits and challenges of completing the tool. framework.
Methods: During the preparation phase, 12 local assessment teams Methods: Each RE-AIM element is defined as follows: Reach: the
were enrolled in three online platforms and identified three individuals number of facilities completing the assessment phase. Effectiveness:
per team to enroll in basic quality improvement (QI) certification. Dur- the outcomes obtained from the I&A phase. Adoption: the number
ing the assessment phase, teams completed 12 modules, 26 electronic of St. Jude Global regional directors, local regional teams, and third
surveys, 14 educational modules, 6 QI exercises, and 33 weekly online parties willing to implement PrOFILE and the additional assessment
mentoring sessions. A short interval report was generated by PrOFILE tools developed using PrOFILE as the backbone (“sibling tools”). Imple-
component (n=5) two weeks after data entry if the team’s data com- mentation: the fidelity or extent to which PrOFILE was delivered as
pletion rate was ≥ 60%. Participants received final score-based and intended. Maintenance: the institutionalization or autonomously deliv-
descriptive reports during the interpretation and action phase to assist ery of PrOFILE as part of the routine practices of St Jude Global
local workshop and action plan development. regional programs.
Results: A total of 191 healthcare providers from twelve healthcare Results: Reach: 136 PHO facilities (119 Abbreviated & 17 Full versions)
facilities located in five global regions participated. Teams varied in size in 34 countries completed data collection activities from the assess-
(7-32). The final form completion rate was 100% for site coordinators ment phase. Effectiveness: 14/15 cohorts conducted prioritization
and 89% for point of care staff. Only one team was not able to review workshops during the I&A phase (Abbreviated), and 16/17 institutions
the educational modules. Teams conducted an average of 5 QI exercises are expected to complete the I&A phase (Full). Adoption: 5/7 St. Jude
(range 0-6). A total of 29 providers completed the basic QI certification. Global Regions implemented PrOFILE, and it has been delivered in four
All facilities received two short interval reports (range 2-5). Partici- languages (English, Russian, Spanish, and Portuguese). Six additional
pants agreed that it was preferable (88%) and valuable (89%) to review assessment tools were designed using PrOFILE as the backbone. Imple-
results immediately after data collection. mentation: Adaptations occurred in the I&A phase, but fidelity has
Conclusions: Beta testing confirmed the feasibility and utility of the been preserved. Maintenance: 9/15 cohorts delivered the I&A phase
short interval reporting. The approach will be permanently incorpo- autonomously by regional programs.
rated into PrOFILE. The descriptive report and data visualization will Conclusions: PrOFILE implementation has been successfully con-
continue evolving to optimize data visualization and benchmarking. ducted in diverse healthcare settings. Data obtained through PrOFILE
has allowed facilities, countries, and regions to define a strategy to
improve childhood cancer outcomes.
EP636 / #611 UPDATES ON THE IMPACT OF THE
IMPLEMENTATION OF THE ST. JUDE PROFILE TOOL:
APPLICATION OF THE RE-AIM FRAMEWORK EP637 / #1893 COACHING FOR IMPROVEMENT: THE
MEXICO IN ALLIANCE WITH ST. JUDE GOLDEN HOUR
Miriam Gonzalez-Guzman1 , Heather Forrest1 , Patricia Belda1 , QUALITY IMPROVEMENT COLLABORATIVE EXPERIENCE
Jeannette Kirby1 , Cesar Villegas1 , Amalia Valdes-Pena1 , Lilly
Mukoka1 , Marta Jarquin-Pardo1 , Patricia Loggetto1 , Sharmeen Jafet Arrieta1,2 , Lilian Delgado-Espejel3 , Francis Duran1 , Luis
Hussain1 , Ayomide Omotola1 , Taisiya Yakimkova1 , Jose Aponte2 , Asya Delgado1 , Cecilia Enriquez1 , Miriam Gonzalez-Guzman4 , Naomi
Agulnik1 , Ibrahim Qaddoumi1 , Nickhill Bhakta1 , Monika Metzger1 , Echeandia-Abud4 , Karla Guerrero-Gomez5 , Lorena Segovia Weber4 ,
Sima Jeha1 , Janet Middlekauff1 , Emily Baum1 , Andrew Pappas1 , Hilda Hernandez6 , Paula Aristizabal7,8 , Paola Friedrich4
Yichen Chen1 , Yuvanesh Vedaraju1 , Radhikesh Ranadive1 , Meenakshi 1 Institute for Healthcare Improvement, Latin America, Boston, United
Devidas1 , Carlos Rodriguez-Galindo1 , Paola Friedrich1 States of America; 2 Harvard Medical School, Department Of Global Health
1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Mem- And Social Medicine, Boston, United States of America; 3 Casa de la Amistad
phis, United States of America; 2 Centers for Disease Control and Prevention, para Niños con Cáncer I.A.P., Mas, Xochimilco, Mexico; 4 St. Jude Children’s
Informatic Service Branch, Division Of Health Informatics And Surveillance, Research Hospital, Global Pediatric Medicine, Memphis, United States of
Atlanta, United States of America America; 5 Casa de la Amistad para Niños con Cancer, Project Coordina-
tor, Ciudad de Mexico, Mexico; 6 Instituto Nacional de Pediatría, Pediatrics,
Background and Aims: The Pediatric Oncology Facility Integrated Mexico City, Mexico; 7 University of California, San Diego, Moores Cancer
Local Evaluation (PrOFILE) tool has two versions. The Full Ver- Center Population Science, Disparities And Community Engagement, La
sion takes a “look within” a Pediatric Hematology and/or Oncol- Jolla, United States of America; 8 Rady Children’s Hospital San Diego, Peck-
ogy (PHO) facility to define and generate consensus on an insti- ham Center For Cancer And Blood Disorders, San Diego, United States of
tutional improvement strategy. The Abbreviated Version provides America
a “looking across” institutions to identify national, subnational,
and regional opportunities for collaboration. PrOFILE implementa- Background and Aims: Coaching is a key element to the success of
tion occurs in three phases: preparation, assessment, and inter- Quality Improvement Collaboratives (QIC) in low- and middle-income
pretation and action (I&A). We evaluate implementation for the settings where QI capacity and knowledge are limited. In the Mexico
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S442 of S635 ABSTRACTS

in Alliance with St. Jude Collaborative (MAS Collaborative), we used Methods: We developed a five-step process to strategize and imple-
coaching to help teams achieve and sustain their local aim and build ment the virtual PAHO workshop: a) define audience: country, type
local capability. of institutions, positions, and backgrounds of healthcare and policy
Methods: From May 2019 to November 2020, 23 institutions partici- professionals; b) define storytelling goal: emotionally engage partic-
pated in the MAS “Golden Hour” Collaborative, aimed at reducing time ipants and provide a compelling action toolkit; c) build appropriate
to antibiotic administration in children with cancer and febrile neu- storyline: platform, content, group dynamics, length of film; d) present
tropenia to <=60 minutes. A 1-5 score was used to assess progress theoretical frameworks: Theory of Change, Socioecological Model,
over time (1=interest in participating; 5=sustained improvement). CureAll framework: WHO Global Initiative for Childhood Cancer;
Two coaches with quality improvement and clinical expertise were United Nations Sustainable Development Goals, and WHO Health Sys-
assigned to each team. Coaching was delivered through one-hour tems Building Blocks; e) design interactive exercises; f) disseminate
monthly virtual sessions and included: 1) reviewing project’s status workshop results.
(run charts and Plan-Do-Study-Act cycles); 2) providing feedback; 3) Results: The day-long workshop included 8 PAHO countries and 80
conducting team building activities, and 4) exploring challenges and participants representing pediatric oncology, hospital administration,
facilitators. ministries of health, foundations, scientific community, and public
Results: Teams received an average of 12 (8-16) coaching sessions. For health organizations. Outputs of the workshop included: a) sum-
the teams with >=12 coaching sessions, 63% (10/16) achieved the Col- mary report, b) empathy word cloud with live reactions, c) qualitative
laborative aim and had an average progress score of 4.0; compared with responses (quotes); d) stakeholders analysis, and e) a prioritization
57% (4/7) and an average score of 3.43 for those with less <=12. The matrix of country strategic activities. The workshop report included a
frequency of calls was variable due to the lack of availability of team practical how-to guide to replicate workshops in other PAHO or World
members, competing schedules, and the COVID-19 pandemic. Teams Health Organization regions. Participants shared lessons learned and
reported an overall positive experience with the coaching process, and action plans on how to incorporate storytelling in their local settings.
that it helped them to achieve and sustain progress over time. Conclusions: Evidence-based policy making may be informed by effec-
Conclusions: Providing coaching as part of the MAS Collaborative con- tive storytelling, guide decision-making, and contribute to dissemi-
tributed not only to helping teams achieve their aim for the Golden nation of scientific data to build emotional engagement and trust.
Hour but also to building local capability for improvement and transfer- Next steps include testing the strategic roadmap in other regions and
ring knowledge and skills for future projects. Based on the experience examine the role of storytelling using Narrative Policy Framework.
and learnings from the first MAS Collaborative, we have refined
the coaching model for the second larger-scale MAS Collaborative
(ongoing). EP639 / #1884 TIMELINE FOR THE CARE AND OUTCOMES
OF 10 CHILDREN WITH CANCER AT HÔPITAL SAINT-DAMIEN

EP638 / #326 STORYTELLING STRATEGY FOR POLICY Pascale Gassant1,2 , Yvania Alfonso Carreras1 , Maysam Homsi3 , Paola
CHANGE IN GLOBAL CHILDHOOD CANCER: IMPLEMENTING A Friedrich3,4 , Miguela Caniza3,5,6
PAN-AMERICAN HEALTH ORGANIZATION WORKSHOP 1 Hôpital Saint - Damien, Pediatric Oncology, Port-au-Prince, Haiti; 2 St.
Jude Children’s Research Hospital Graduate School of Biomedical Sci-
Irini Albanti1 , Soad Fuentes-Alabi De Aparicio2 , Meghan Shea3 , Liliana ences, Global Child Health, Memphis, United States of America; 3 St. Jude
Vasquez4 Children’s Research Hospital, Global Pediatric Medicine, Memphis, United
1 Harvard Humanitarian Initiative, Brigham And Women’s Hospital, Cam- States of America; 4 St. Jude Children’s Research Hospital, Oncology, Mem-
bridge, United States of America; 2 Pan American Health Organization, phis, United States of America; 5 University of Tennessee Health Sciences
Non-communicable Diseases, Washington D.C., United States of America; Center, Pediatrics, Memphis, United States of America; 6 St. Jude Children’s
3 Persistent Productions, Persistent Productions, Rockport, United States Research Hospital, Infectious Diseases, Memphis, United States of America
of America; 4 Pan American Health Organization, Non-communicable Dis-
eases, Washington D.C, United States of America Background and Aims: Most of the world’s children with cancer live
in low- and middle-income countries (LMICs), where many childhood
Background and Aims: Storytelling has been used to highlight health cancers are not diagnosed or reported, or encounter delays to diag-
disparities and mobilize awareness for critical global pediatric oncol- nosis. Late diagnosis decreases the survival in childhood cancer. We
ogy issues. This study explored how effective and ethical storytelling report the timeline of clinical events and the healthcare outcome of ten
can be a valuable policy-change and advocacy tool. The aim was to children with cancer in Haiti.
describe the strategic planning process to design and implement a Pan- Methods: We selected children with cancer diagnosed between 2019
American Health Organization (PAHO) workshop using storytelling to and 2020 representing the most frequent type of cancer at our insti-
engage stakeholders with national cancer control plan implementation tution. We obtained time intervals between clinical events including
for Central America, Dominican Republic and Haiti, and ensure uptake appearance of cancer signs and symptoms, first time consultation with
of policy impact. a healthcare provider, referral to a pediatric cancer center, evalua-
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S443 of S635

tion by pediatric oncologists, and the definitive diagnosis of cancer. The most common malignancy was lymphoma (574 [38%]: includ-
We extracted the time intervals of the clinical events, the demograph- ing [WKD1] Non-Hodgkin’s lymphoma (387 [67%], of which 301
ics, and the clinical data from the participants’ medical records; we (78%) were Burkitt lymphoma), Hodgkin’s lymphoma (125 [21%]), and
complemented the data with the information obtained from available lymphoma-not otherwise specified (NOS) (57 [10%]). Next most com-
parents. mon were sarcomas (325[WKD2] [22%]: including Kaposi’s sarcoma
Results: We included 10 patients, with ages ranging from 2 to 16 years. (111 [34%]), rhabdomyosarcoma (68 [21%]), osteosarcoma (61 [19%]),
They represented malignant diseases typically seen and treated at and sarcoma-NOS (61 [19%]); and carcinomas (139 [9%]: including
our institution: acute lymphoblastic leukemia, acute myeloid leukemia, squamous cell carcinoma (73 [53%] and carcinoma-NOS (27 [19%]).
Hodgkin lymphoma, Wilms tumor, retinoblastoma, rhabdomyosar- Then, leukemias (95 [6%]): including acute lymphoblastic leukemia (63
coma, Ewing sarcoma, bone tumor and nasopharyngeal carcinoma. We [66%]) and acute myeloblastic leukemia (30 [32%]). Followed by Wilm’s
found that there was a delay time of 68.5 weeks, with delays longer in Tumor (90 [6%]), small round blue cell tumors (SRBCT) (77 [5%]),
patients with solid tumors than hematologic malignancies. The median retinoblastoma (66 [4%]), neuroblastoma (32 [2%]), hepatic tumors (31
delay to obtain pathology’s report was 7 weeks. Causes of delays were [2%]), and germ cell tumors (22 [1%]). 45 (3%) of tumors could not
multifactorial, including socioeconomic and health system factors, as be classified. The proportion of lymphoma-NOS and malignant-NOS
well as communication and speed of diagnostics. decreased post-2013, while SRBCT increased (p<0.01).
Conclusions: Despite limited data, our report shows an important Conclusions: The most common pediatric tumors in UNC-KCH lab-
delay for the cancer diagnosis at our institution that should be oratory are haematolymphoid of which Burkitt’s is the commonest.
improved for better outcomes for children with cancer. Services need to be expanded further to aid in the diagnosis of SRBCTs,
carcinomas, and sarcomas.

EP640 / #1042 PEDIATRIC MALIGNANT TUMORS:


DIAGNOSTIC EXPERIENCES FROM KAMUZU CENTRAL EP641 / #1583 IDENTIFYING CONTEXTUAL FACTORS
HOSPITAL PATHOLOGY LABORATORY IN MALAWI 2011-2013 INFLUENCING THE SUCCESS OF FUTURE QUALITY
IMPROVEMENT PROJECTS IN TWELVE PROFILE BETA TESTING
Yolanda Gondwe1 , April Evans2 , Coxcilly Kampani1 , Fred Chimzimu1 , INSTITUTIONS
Gugulethu Mapurisa1 , Apatsa Matatiyo1 , Ande Salima1 , Agness
Manda1 , Wiza Kumwenda1 , Maurice Mulenga3 , Tamiwe Tomoka1 , Miriam Gonzalez-Guzman1 , Heather Forrest1 , Alisha Pershad2 , Logan
Satish Gopal4 , Yuri Fedoriw1,5,6 , Katherine Westmoreland1,2 Houston3 , Paola Friedrich1
1 UNC Project Malawi, Cancer Program, Lilongwe, Malawi; 2 University 1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis,

of North Carolina, Pediatric Hematology Oncology, Chapel Hill, United United States of America; 2 Rhodes College, Academic Programs, Memphis,
States of America; 3 Kamuzu Central Hospital, Pathology, Lilongwe, Malawi, United States of America; 3 University of Tennessee Health Science Center,
Malawi; 4 National Cancer Institute, Center For Global Health, MD, United College Of Medicine, Memphis, United States of America
States of America; 5 University of North Carolina Chapel Hill, Pathology And
Laboratory Medicine, Chapel Hill, United States of America; 6 University Background and Aims: The Pediatric Oncology Facility Integrated
of North Carolina, Lineberger Comprehensive Cancer Center, Chapel Hill, Local Evaluation (PrOFILE) helps institutions define an improvement
United States of America strategy. During the past nine months, twelve pediatric hematology
and oncology (PHO) institutions joined the second beta testing cohort
Background and Aims: In regions where reliable population-based and implemented PrOFILE. Post-PrOFILE activities will include imple-
pediatric cancer data is scarce, histopathological diagnosis and clas- menting quality improvement projects (QI) as part of their 3-year
sification is crucial for both patient care and surveillance. In 2011, institutional action plan. The Model for Understanding Success in Qual-
diagnostic pathology services became available in Malawi’s capital for ity (MUSIQ) framework identifies contextual factors necessary for
the first time when the University of North Carolina-Kamuzu Cen- successful quality improvement (QI) projects implementation and out-
tral Hospital (UNC-KCH) pathology laboratory was established. The comes. We aimed to identify contextual factors that institutional teams
pathology laboratory is one of two in the country, serving roughly half should consider during future QI project implementation.
of 19 million population. Methods: The site coordinator (SC) and physician lead (PL) from each
Methods: This is a retrospective case series of tumors diagnosed institution completed the MUSIQ 35-item questionnaire before start-
at UNC-KCH pathology laboratory between 2011-2020 for ages 0- ing PrOFILE data collection. We averaged SC and PL scores to generate
18 years. Until 2013, pathologic diagnoses from the laboratory were the final institutional scores for five domains: QI team, microsystem, QI
based on morphology alone. After this, services expanded to include support and capacity, organization, environment, and miscellaneous.
cytology, histology, and immunohistochemistry. Each domain includes between 3-9 contextual factors. A maximum of
Results: 12,761 specimens were received between 2011-2020 from 7 points are assigned to each factor. The highest possible MUSIQ score
5,137 pediatric patients. 1,498 (29%) patients received a malignant was 168. A reasonable chance of success is suggested when scores
diagnosis, median age was 10 (IQR 5-14) and 810 (54%) were male. range from 120 to 168. Each participant center received a 3-page
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S444 of S635 ABSTRACTS

score-based report that invited them to identify areas of opportunity factors organized around six dimensions (external environment, orga-
and next steps. nizational factors, QI support and capacity, microsystem, QI team;
Results: The average MUSIQ score was 127 (range 82-142). Seventy- and miscellaneous) using baseline and end-line surveys. Factors were
five percent (9/12) of the institutions have a reasonable chance of assessed using a 1 to 7 scale (1=totally disagree; 7=totally agree), for a
success when implementing a QI project. The domains with the lowest total score from 25 to 168, where a score >120 means the project has
scores were QI support and capacity, and external environment. Con- a reasonable chance of success.
textual Factors with the lowest scores included: external QI motivators Results: Seventeen (73.9%) teams reported MUSIQ scores for both
(3, range 1-5), external project sponsorship (3, 1-7), team prior QI expe- reporting periods. The average total score was 128 (83-156) at baseline
rience (4, range 1-6), and QI capability (4, range1-7). The domains with and 135 (100-161) at the end of the QIC. The dimensions with the low-
the highest scores include QI team and organization. est average score were environment (3.6; 3.0-4.2) and QI support and
Conclusions: MUSIQ assisted us in recognizing contextual barriers that capacity (4.8; range 3.9-5.3). Eighty-two percent (14/17) of the teams
exist among PrOFILE beta testing institutions. Post-PrOFILE activities had an increase in their overall MUSIQ score and their QI support and
will target some of the identified barriers. capacity scores.
Conclusions: MUSIQ scores provided a foundation for understanding
the context and specific needs and gaps of participating institu-
EP642 / #1914 UNDERSTANDING THE ROLE OF tions that are key to the success of a QIC. Including structural and
CONTEXTUAL FACTORS IN DETERMINING SUCCESS OF QI supports is key to achieving and sustaining results in real-world
QUALITY IMPROVEMENT COLLABORATIVES: EXPERIENCE settings.
FROM THE MAS GOLDEN HOUR COLLABORATIVE

Jafet Arrieta1,2 , Miriam Gonzalez-Guzman3 , Logan Houston4 , Alisha EP643 / #817 IMPLEMENTING THE PEDIATRIC ONCOLOGY
Pershad5 , Cecilia Enriquez1 , Francis Duran1 , Luis Delgado1 , Naomi FACILITY INTEGRATED LOCAL EVALUATION (PROFILE) TOOL
Echeandia-Abud3 , Karla Guerrero-Gomez6 , Lorena Segovia Weber7 , AT A TERTIARY CARE HOSPITAL IN PAKISTAN
Hilda Hernandez8 , Paula Aristizabal9,10 , Paola Friedrich3
1 Institute for Healthcare Improvement, Latin America, Boston, United Syed Hamid
States of America; 2 Harvard Medical School, Department Of Global Health Indus Hospital Health Network, Pediatric Oncology, Karachi, Pakistan
And Social Medicine, Boston, United States of America; 3 St. Jude Children’s
Research Hospital, Global Pediatric Medicine, Memphis, United States Background and Aims: The Pediatric Oncology Facility Integrated
of America; 4 University of Tennessee Health Science Center, College Of Local Evaluation (PrOFILE) tool was developed by St. Jude Global to
Medicine, Memphis, United States of America; 5 Rhodes College, Academic define improvement strategies for increasing childhood cancer survival
Programs, Memphis, United States of America; 6 Casa de la Amistad para rates. It provides a dynamic 360-degree evaluation of health service
Niños con Cancer, Project Coordinator, Ciudad de Mexico, Mexico; 7 St. Jude delivery over 5 components (context, workforce, diagnostics, therapy,
Children’s Research Hospital, Department Of Global Pediatric Medicine, and patients & outcomes).
Memphis, TN, United States of America; 8 Instituto Nacional de Pediatría, Methods: The IHHN participated in PrOFILE Beta-2 testing from
Pediatrics, Mexico City, Mexico; 9 University of California, San Diego, Moores August 2021 to May 2022, conducted in three phases: (i) prepara-
Cancer Center Population Science, Disparities And Community Engage- tion, (ii) assessment, and (iii) interpretation and action. The prepa-
ment, La Jolla, United States of America; 10 Rady Children’s Hospital San ration phase entailed leadership engagement and recruitment of
Diego, Peckham Center For Cancer And Blood Disorders, San Diego, United assessment teams. Twelve modules, 26 electronic forms, and 6 qual-
States of America ity improvement exercises were completed during the assessment
phase. After approval of the physician lead, the reports’ feedback
Background and Aims: A series of contextual factors contribute to the was recorded on the Cure4Kids online portal by the site coordina-
variation in the success of Quality Improvement Collaboratives (QIC) tor. Weekly mentoring sessions with the St. Jude Global PrOFILE
in real-world settings. Between May 2019 to November 2020, 23 Mex- team were continued throughout this period. During the inter-
ican institutions engaged in conducting the first Mexico in Alliance with pretation and action phase, an institutional report was generated
St. Jude Golden Hour Collaborative (MAS Collaborative), improving the to develop a three-year action plan and a 2-day workshop was
percentage of children with cancer and febrile neutropenia who pre- scheduled.
sented to the emergency department and received the first dose of Results: Twenty-eight employees from various disciplines were
antibiotics in ≤60min from 39% to 78%, with varying results across enrolled. The overall form completion rate was 55%. A polar graph
institutions. This study aimed to assess the role of contextual factors was generated with scores <50% for the national context. Radiation
in determining the success of the MAS Collaborative. therapy, surgery, personnel, patients and outcomes, service capacity,
Methods: This QIC followed the Breakthrough Series Model cou- and chemotherapy were scored between 50% and 75%. Scores >75%
pled with a QI capability-building program. We used the Model for were given for finances and resources, facility and local context, ser-
Understanding Success in Quality (MUSIQ) to assess 24 contextual vice integration, supportive care, and diagnostics. Institutional insights
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S445 of S635

highlighted the need for a national cancer control plan, enhancement EP645 / #1575 CURING SAFELY: CO-DEVELOPING A
of referral systems, increasing availability of drugs, strengthening of REGIONAL RESOURCE GUIDE WITH THE WORLD HEALTH
core Pediatric Hematology and Oncology (PHO) teams, and improving ORGANIZATION TO EMPOWER COMMUNITY-BASED
nurse to patient ratios. CLINICIANS CARING FOR CHILDREN WITH CANCER
Conclusions: Team-building activities and a dedicated patient-care
assessment team are needed to effectively integrate multidisci- Sri Andini Handayani1 , Bishnu Rath Giri2 , Gampo Dorji3 , Aye Aye
plinary approach to childhood cancer. PrOFILE feedback reports Khaing4 , Aye Moe Moe Lwin5 , Thida Moe6 , Sanjeeva Gunasekera7 ,
helped identify areas of improvement in local and national contexts Monnie Abraham1 , Miguela Caniza1 , Leeanna Fox Irwin1 , Daniel
and involved institutional and national leadership. Similar health- Moreira1 , Sheena Mukkada1 , Jennifer L. Pauley1 , Jeremy Slone1 , John
care assessment programs must be initiated locally for continuous Spencer1 , Liz Sniderman8 , Catherine G. Lam1 , Curing Safely Working
improvement. Group9
1 St. Jude Children’s Research Hospital; WHO Collaborating Centre for
Childhood Cancer, Global Pediatric Medicine Department, Memphis, United
EP644 / #1351 GLOBAL PROBLEM OF PHYSICIAN DUAL States of America; 2 WHO South-East Asia Regional Office, South-east Asia
PRACTICES: A LITERATURE REVIEW Regional Initiative On Childhood Cancer, Kathmandu, Nepal; 3 WHO Coun-
try Office, Non-communicable Diseases, Lalitpur, Nepal; 4 Yangon Children
Romy Hoogland1,2 , Lisa Hoogland1,2 , Krisna Handayani3 , Mei Hospital, Pediatric Hematology Oncology, Yangon, Myanmar; 5 WHO Coun-
Sitaresmi3 , Gertjan Kaspers1,2 , Saskia Mostert1,2 try Office, Non-communicable Diseases, Yangon, Myanmar; 6 Myanmar
1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, National Childhood Cancer Network, National Coordinator, Yangon, Myan-
Utrecht, Netherlands; 2 Amsterdam UMC, Vrije Universiteit, Pediatric Oncol- mar; 7 National Cancer Institute, Paediatric Oncology, Maharagama, Sri
ogy, Amsterdam, Netherlands; 3 Dr.Sardjito Hosptal/Universitas Gadjah Lanka; 8 Stollery Children’s Hospital, Northern Alberta Children’s Cancer
Mada, Pediatrics, Yogyakarta, Indonesia Care Program, Edmonton, Canada; 9 Curing Safely Working Group, Curing
Safely Working Group, Memphis, United States of America
Background and Aims: Physician dual practices (PDP) is a term used
to describe physicians who combine work in the public and private Background and Aims: A context-sensitive practical guide is key
health-care sector. This study aimed to find evidence of PDP world- to empowering community-based physicians and nurses who play
wide, investigate reasons and consequences of PDP, and compare critical roles in the initial diagnosis, referral, and treatment main-
PDP in high-income (HIC) versus low and middle-income countries tenance for pediatric hematology/oncology (PHO) patients, but who
(LMIC). often have limited time and variable backgrounds. Building on engage-
Methods: In this literature review, the search for PDP evidence ment with the Myanmar National Childhood Cancer Network in 2019,
was conducted in the English language. PubMed and Google St. Jude Children’s Research Hospital and WHO South-East Asia
were searched for relevant publications up to September 30, Regional Office launched Curing Safely in 2021 as a regional Resource
2020. Guide project, recognizing the amplified need for clinicians to access
Results: Of 195 countries, PDP-reports were found in 157 coun- point-of-care practice recommendations during the pandemic.
tries (81%). No significant difference in prevalence of PDP was found Methods: Initial co-design needs assessments with collaborators
between HIC (77%) and LMIC (82%). Most common reason for working across nine disciplines identified content, format, and target audiences.
in private sector was low government salaries in public hospitals (55%). Guided by the Kirkpatrick Model, a toolkit was structured to encom-
This was more reported in LMIC (65%) than HIC (30%; P<0.001). pass evaluation and implementation support, including: 1) a scoring
Most common reason for working in public sector was patient recruit- matrix measuring accuracy, applicability, comprehensiveness, and clar-
ment for private practice (25%). This was more reported in HIC (45%) ity; 2) feedback forms assessing local relevance and incorporating
than LMIC (16%; P<0.001). PDP were described as detrimental to CDC’s Clear Communication index; 3) a prioritization survey; 4) a
the public health-sector in 58% of country-reports. Most common pre/post quiz measuring clinicians’ comfort in managing PHO patients;
adverse consequence was lower quality-of-care in public hospitals and 5) an adaptation guide and introductory materials for co-designers
(27%). LMIC with PDP-reports had more severe corruption (P<0.001), and end-users.
lower current health-expenditure (P<0.01), and higher out-of-pocket Results: Collaborators from 8 core institutions across 6 countries
expenditure (P<0.001) than HIC. The scale of PDP was common and 23 Myanmar network sites provided initial feedback. Eight
in more LMIC (92%) than HIC (60%; P<0.001). Government poli- national ministry-selected teams, including 35 volunteer physicians
cies to address PDP did not differ significantly between HIC and and nurses from 15 tertiary and shared care institutions constituted
LMIC. an expanded regional Working Group. Three guide components inte-
Conclusions: We conclude that PDP were present grating local provider practices and patient safety considerations are
in most HIC and LMIC. In the majority of reports a in phased development: 1) Summaries to Safely Administer 31 Essen-
detrimental effect of PDP on public health-care was tial Medicines (testing underway in English/Burmese); 2) Chapters
described. on 4 prioritized E-Poster Topics (Chemotherapy and Supportive Care
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S446 of S635 ABSTRACTS

Medications; PHO Overview; Pain and Palliative Care; Fever and childhood cancer priorities. Iterative design improvements occurred
Neutropenia); and 3) Poster job aids. based on feedback. Training evolved from lecture-based presentations
Conclusions: The Curing Safely Resource Guide co-development illus- to practice exercises using MURAL. Instant messaging (WhatsApp)
trates a collaborative approach in disseminating regionally tailored rather than video conferencing (Zoom) became the preferred method
best practices to safely manage PHO patients, meeting pressing needs of communication for real-time remote support.
to empower community-based providers where specialists remain Conclusions: Using web-based services, three countries successfully
scarce, and access further constrained by COVID-19. hosted hybrid workshops. Data from these workshops reflect inputs
from key loco-regional-global stakeholders and provide basis for GICC
status. The hybrid approach also substantially reduced costs and
EP646 / #1347 DEPLOYMENT OF A HYBRID WORKSHOP allowed more rapid engagement with new regional partners.
FOR NATIONAL CHILDHOOD CANCER PRIORITY SETTING IN
THE SUB-SAHARAN AFRICAN REGION DURING THE COVID-19
PANDEMIC EP647 / #1032 PET-CT VS CECT FOR RESPONSE
ASSESSMENT IN CHILDHOOD HODGKIN LYMPHOMA- SUBSET
Sharmeen Hussain1 , Ayomide Omotola1 , Patricia Belda1 , Miriam ANALYSIS OF INPOG HL-15-01 STUDY- NEED FOR A POLICY
Gonzalez-Guzman1 , Heather Forrest1 , Glenn Mbah Afungchwi2 , Sona CHANGE
Franklin Mukete3 , Thomas Alexander4 , Aziza Shad5,6 , Julie Broas7 ,
Trish Scanlan8,9 , Paola Friedrich1 , Nickhill Bhakta1 Manas Kalra1 , Sameer Bakhshi2 , Manisha Singh3 , Rachna Seth4 ,
1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis, Nishant Verma5 , Sandeep Jain6 , Venkatraman Radhakrishnan7 , Piali
United States of America; 2 Mbingo Baptist Hospital-Cameroon Baptist Con- Mandal8 , Amita Mahajan9 , Ramandeep Arora10 , Veronique Dinand11 ,
vention Health Services, Pediatric Oncology, Mbingo, Cameroon; 3 Ministry Gauri Kapoor6 , M Sajid12 , S Thulkar13 , A Arora14 , Ankit Taluja15 ,
of Public Health, National Committee For The Fight Against Cancer, Jagdish Chandra8
Yaounde, Cameroon; 4 University of North Carolina School of Medicine, 1 SIR GANGA RAM HOSPITAL, Pediatrics, DELHI, India; 2 All India Institue
Department Of Pediatrics, Chapel Hill, United States of America; 5 Children’s of Medical Sciences, Medical Oncology, New Delhi, India; 3 MAHAVIR CAN-
Hospital at Sinai, Pediatric Hematology Oncology, Baltimore, United States CER SANSTHAN, Oncology, PATNA, India; 4 All India Institute of Medical
of America; 6 The Aslan Project, Co-founder & Medical Director, Wash- Sciences- New Delhi, Division Of Pediatric Oncology, Department Of Pedi-
ington, United States of America; 7 The Aslan Project, Executive Director, atrics, New Delhi, India; 5 KING GEORGE MEDICAL UNIVERSITY, Pediatrics,
Washington, United States of America; 8 Muhimbili National Hospital, Pedi- LUCKNOW, India; 6 RAJIV GANDHI CANCER HOSPITAL AND RESER-
atric Oncology, Dar Es Salaam, Tanzania; 9 Tumaini la Maisha, Ceo, Dar Es ACH CENTRE, Pediatrics, DELHI, India; 7 Cancer Institute (W.I.A), Medical
Salaam, Tanzania Oncology, CHENNAI, India; 8 KALAWATI SARAN CHILDRENS HOSPITAL,
Pediatrics, DELHI, India; 9 INDRAPRASTHA APOLLO HOSPITAL, Pediatrics,
Background and Aims: In-person multinational stakeholder work- DELHI, India; 10 Max Super-Specialty Hospital, Medical Oncology, New
shops during the COVID-19 pandemic were not feasible. To ensure Delhi, India; 11 B J WADIA HOSPITAL, Palliative Care, MUMBAI, India;
sustained progress toward the World Health Organization Global Ini- 12 MAHAVIR CANCER SANSTHAN, Pediatrics, PATNA, India; 13 AIIMS,

tiative for Childhood Cancer (GICC) targets, we designed a blended Radiology, DELHI, India; 14 AIIMS, Pediatrics, DELHI, India; 15 CANKIDS

virtual and in-person hybrid workshop for national priority setting. KIDSCAN, Research, DELHI, India
Methods: Three hybrid workshops were conducted in Cameroon,
Ethiopia, and Tanzania during 2021. Prior to the workshops, 3-5 webi- Background and Aims: The InPOG-HL-15-01 used a risk-stratified
nars were held with country teams to build national engagement, and response-based approach with an ABVD backbone to treat chil-
engage ministries of health and support completion of the St. Jude dren with Hodgkin Lymphoma(HL). Children with bulky disease or
PrOFILE tool. PrOFILE data was entered locally, shared electronically, inadequate response at early response assessment(ERA) after 2 cycles
analyzed at St. Jude, and returned as reports to each center. To support of chemotherapy were assigned to receive radiation. For ERA, PET-
the co-design workshop, local facilitators were identified, a curriculum CT was recommended but not mandatory in view of limited access.
was developed, and online trainings were conducted using Zoom video Although the developed world has moved away from CECT, in many
conferencing and MURAL online collaboration platforms. LMIC, it is still the easily available and preferred modality and this lead
Results: A total of 3, 5, and 5 hospitals agreed to participate in PrOFILE to a natural randomisation. We aimed to compare the impact of using
in Cameroon, Ethiopia, and Tanzania, respectively: all but one com- CECT vs PET-CT for ERA in treatment decisions and outcomes.
pleted data entry. Six local and 8 global facilitators were trained in Methods: 396 children were enrolled and 382 had an ERA at the
Cameroon; 5 local and 8 global in Ethiopia; and 11 local and 2 global assigned time point. Satisfactory response was defined as Deauville
in Tanzania. There were 59 participants in Cameroon (49 in-person, score 3 or less for patients undergoing PET-CT and CR/VGPR for
10 virtual); 64 (50 in-person, 14 virtual) in Ethiopia; and 63 (53 in- patients undergoing CT.
person, 10 virtual) in Tanzania. 17 small groups across all 3 hybrid Results: At ERA, satisfactory response was observed in
workshops successfully completed 4 co-design exercises to identify 12 277/382(72.5%) and this was significantly more in PET-CT(151/186,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S447 of S635

81.2%) as compared to CT based assessments(126/196, 64.3%) Results: Of 312 requests, 95% (N=295) were approved; 17 were
respectively(p<0.001). In 203 patients with non-bulky disease (in rejected or abandoned prior to approval and 276 led to drug adminis-
whom the indication for radiation was entirely dependent on ERA), tration. Forty-nine different agents were requested from 18 different
96/114(84.2%) and 61/89(68.5%) patients achieved a satisfactory companies. Provincial health insurance funding was approved in 28%
response based on PET-CT and CT based ERA(p=0.008) and hence (n=87) cases, with disparities between provinces. Compassionate
a lesser proportion of patients in the PET-CT arm had radiation. The access from a pharmaceutical company was approved in 57% (n=169)
5 year EFSa for children undergoing CECT vs PET-CT was 85.1% of cases. Other requests were either paid out-of-pocket, by patient’s
+/- 2.6% vs 84.6% +/- 2.7%(p=0.969). The 5 year EFS and OS for private health insurance or from hospital funds). The median time to
children undergoing CECT vs PET-CT was 86.6% +/- 2.4% vs 85.1% approval was 6 days (range: 0-190 days). Bridging therapy was required
+/- 2.7%(p= 0.725) and 91.5 +/- 2% vs 94.1% +/- 1.7%(p=0.407) while waiting for approval for 28 cases; treatment plan was delayed in
respectively. 22 cases and patient’s disease status or outcome was impacted in 15
Conclusions: Use of PET-CT for ERA is more likely to indicate satisfac- cases.
tory response reducing the need for radiation. Lesser use of radiation Conclusions: In Canada, inconsistency in provincial funding models for
did not have negative impact on outcomes. An urgent need of policy drugs affects the equity of access to novel cancer treatments for chil-
change and advocacy measures to use PET-CT is likely to reduce late dren. Complex application processes, and the rarity of most pediatric
effects in children with HL. cancer indications may cause treatment delays and impact clinical out-
comes. The need to understand and help surmount barriers in access
to novel cancer therapies spurred the establishment of a National Drug
EP648 / #612 MAPPING THE TERRITORY OF INNOVATIVE Access Navigator.
DRUG THERAPY ACCESS FOR PEDIATRIC ONCOLOGY
PATIENTS IN CANADA EP649 / #1492 SCOPES AND FIRST ANNUAL RESULTS OF
THE NATIONAL REGISTRY OF PAEDIATRIC PATIENTS WITH
Sandra Judd1 , Gabriel Revon-Riviere2 , Avram Denburg2 , Rebecca J NEOPLASTIC DISEASES IN GREECE
Deyell3 , Stephanie A Grover4 , Lucie Pecheux5 , Raoul Santiago6 , Thai
Hoa Tran7 , Magimairajan Issai Vanan8 , Alexandra P Zorzi9 , Sarah Antonis Kattamis1 , Vassilios Papadakis2 , Marina Servitzoglou3 , Anna
Cohen-Gogo2 , Daniel Morgenstern2 Paisiou4 , Apostolos Pourtsidis5 , Nikolaos Katzilakis6,7 , Evgenia
1 Hospital for Sick Children, Department Of Pharmacy, Toronto, Canada; Papakonstantinou8 , Maria Palampougiouki9 , Eleftherios Thireos10 ,
2 The Hospital For Sick Children, Temerty Faculty of Medicine, Univer- Maria Trifonidou11 , Asimo Koukougianni12 , Theoklis Zaoutis13 ,
3 British
sity of Toronto, Peditatric Hematology Oncology, Toronto, Canada; Margarita Mpaka3 , Sophia Polychronopoulou14 , Eleni Kosmidi15 ,
Columbia Children’s Hospital / University of British Columbia, Pediatrics, Maria Ioannidou9 , Eftichia Stiakaki6,7 , Panagiota Mitrou16
Division Of Pediatric Hematology Oncology Bmt, Vancouver, Canada; 4 The 1 “Aghia Sophia” Children’s Hospital, Division Of Pediatric Hematology
Hospital For Sick Children, Genetics And Genome Biology, Toronto, Canada; Oncology, First Dpt Of Pediatrics, National & Kapodistrian University Of
5 University of Alberta - Stollery Children’s Hospital, Pediatric Hematology Athens, Athens, Greece; 2 “Aghia Sophia” Children’s Hospital, Department
6 CHU
Oncology, Edmonton, Canada; of Quebec - Laval University, Pedi- Of Pediatric Hematology-oncology, Athens, Greece; 3 ‘P & A. Kyriakou’ Chil-
atrics, Quebec, Canada; 7 CHU Sainte-Justine, Pediatrics, Montreal, Canada; dren’s Hospital, Department Of Oncology, Athens, Greece; 4 ‘Aghia Sofia’
8 University of Manitoba / Cancer Care Manitoba, Pediatrics And Child Children’s Hospital, Athens, Greece, Bone Marrow Transplantation Unit,
Health, Winnipeg, Canada; 9 Children’s Hospital, London Health Sciences Athens, Greece; 5 Children’s Hospital Mitera, Pediatric And Adolescent
Centre, Paediatrics, London, Canada Oncology Clinic, Athens, Greece; 6 University Hospital of Heraklion, Depart-
ment Of Pediatric Hematology-oncology, University Of Crete, Heraklion,
Background and Aims: Lack of funding for innovative drugs in pedi- Greece; 7 University Hospital of Heraklion, University of Crete, Department
atric oncology impacts drug access in Canada with marked variation Of Pediatric Hematology-oncology, Heraklion, Greece; 8 Ippokratio Hospi-
by disease and geography. Each province controls its own health tal, Department Of Pediatric Hematology- Oncology, Thessaloniki, Greece;
care funding. For unfunded marketed agents, pharmaceutical com- 9 Aristotelion University of Thessaloniki, AHEPA General Hospital, Depart-
panies may have compassionate access programs to obtain access ment Of Pediatric Hematology-oncology, Thessaloniki, Greece; 10 Athens

to expensive, unlicensed therapies. This extensive Canadian review Medical Society, -, Athens, Greece; 11 Floga – Parents’ Association of Chil-
of innovative drug requests focuses on understanding the impact of dren with Cancer, -, Athens, Greece; 12 Karkinaki Non-Profit Organisation, -,
funding and process in the time to drug access. Athens, Greece; 13 University of Athens, School Of Medicine, Athens, Greece;
Methods: We conducted a retrospective review of access requests 14 Aghia Sophia Children’s Hospital, Department Of Pediatric Hematology-
for anti-cancer therapies (not Health Canada-approved for pedi- oncology (tao), Athens, Greece; 15 Mitera Children’s Hospital, Department Of
atric indications at the time of application) from 2013 to 2020. Pediatric And Adolescent Hematology-oncology, Athens, Greece; 16 Hellenic
Patient characteristics, drug information and request details were col- Ministry Of Health, Department Of Therapeutic Protocols And Patient
lected. We excluded cytotoxic chemotherapy, cellular products, and Registries, Athens, Greece
cytokines.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S448 of S635 ABSTRACTS

Background and Aims: National registries are significant tools to was to report the obstacle course of this unit, its victories and
determine incidence and prevalence of rare diseases, like pediatric challenges.
cancer, and enable optimal health resources allocation and respec- Methods: Description of the different steps that led to the creation
tive therapeutic and diagnostic strategies. The aim of this study is to of the Pediatric Oncology Unit (POU) of Bobo-Dioulasso ; mention
describe the structure of the newly-established national registry for of several impediments linked to unsafety and covid-19 ; description
pediatric cancer in Greece and its first annual results. of the care activities since their beginning in January 2019 ; point of
Methods: The Greek Ministry of Health, in collaboration with the challenges and perspectives.
Hellenic Society of Pediatric Hematology-Oncology, along with rep- Results: The regular admissions of children with cancers motivated
resentatives from all the Pediatric Hematology/Oncology units and the initiation of a study making the inventory of fixtures in 2012. The
parents’ and patients’ support groups developed the new national main feature was the refusal of treatment by families, since they were
registry for pediatric cancer. almost systematically referred to Ouagadougou, 365 km away. Formal-
Results: The registry uses the digitalized operating platform ‘IDIKA’, ized activities were started in 2019, with the support of the POU of
that has initially been developed to unify and digitalize the prescrip- Ouagadougou. However, in the context of terrorism and Covid-19, the
tion system. The registry structure follows the patient journey starting development of the new unit was not a priority for local authorities. The
from the diagnosis of the disease. The data captured include 4 sec- unit joined GFAOP in December 2020 : two nurses from CHUSS partici-
tions: demographics, disease characterization, treatment and follow pated in a online training organized by the GFAOP. A collaboration with
up. Disease is reported based on ICD-10 and ICD-O-3 (Toronto classi- a local women LIONS club led to the renovation and equipment of the
fication) systems. Treatment section describes the different modalities unit. Since January 2019, 143 children with cancers have been treated
used (chemotherapy along with the protocol name, radiation therapy, in the pediatric oncology unit of Bobo-Dioulasso, with a predominance
etc), with description of the respective health care provider. The fol- of Burkitt lymphoma followed by leukemia and nephroblastoma.
low up section focuses on sequential update of the patient’s condition Conclusions: Several challenges of which, supply of anti-cancer drugs
and includes data on remission, relapse, therapy continuation, mortal- and fight against dropout from treatment are remaining. Neverthe-
ity and second malignancy. The registry was established in early 2021. less, the perspectives are good, with a better involvement of the
After one year there were 501 registrations, involving 441 patients. highest health authorities in the battle against cancers and other
Data were registered by 14 different health care providers from all the non-communicable diseases.
Pediatric Hematology/Oncology Units in Greece. The number of regis-
tered patients that were diagnosed during 2021 was 178. The majority
of registered patients were diagnosed and followed in Athens. EP651 / #1566 LYMPHOMA AND SOLID TUMORS AMONG
Conclusions: Initial registry use and acceptance is promising. Further ADOLESCENTS 15-19 YEARS OF AGE: SINGLE CENTER
data analysis will help delineate the prevalence of different types of EXPERIENCE
cancer in pediatric population in Greece, the complexity of patient jour-
ney and the evolving needs. Improvements in platform structure and Deniz Kizmazoğlu, Emre Çeçen, Dilek Ince, Nur Olgun
registering process will optimize registry’s use and outcomes. DEÜ Oncology Institute, Pediatric Oncology, İZMİR, Turkey

Background and Aims: AYA (Adolescents and Young Adults) includes


EP650 / #1524 HISTORY AND CHALLENGES OF A YOUNG 15 to 39 years of age. Over 70000 AYAs are diagnosed with cancer
PEDIATRIC ONCOLOGY UNIT IN SUB-SAHARAN AFRICA IN A each year in United States. Adolescent cancers are defined as cancers
CONTEXT OF UNSAFETY AND COVID-19 PANDEMY between 15-19 years of age and only this group can be treated and
followed in pediatric oncology. We aimed to present our adolescent
S. Aimée Kissou1 , Saïdou Savadogo1 , Léa Zerbo1 , Mahamadi patients in this study.
Nakoulma1 , Chantal Bouda2 , Sonia Kaboret3 , Rolande Kabore2 , Methods: A retrospective review of 209 patients between 15-19 years
Diarra Yé3 , Jean Michon4 of age at diagnosis was done. Medical files were evaluated for distribu-
1 Centre Hospitalier Universitaire Sourô Sanou, Pediatrics, Bobo-Dioulasso, tion of cancer types, clinical features, treatment courses, survival rates
Burkina Faso; 2 Centre Hospitalier Universitaire Yalgado Ouédraogo, Pedi- and follow-up times.
atrics, Ouagadougou, Burkina Faso; 3 Centre Hospitalier Universitaire Pédi- Results: Two hundred and nine patients between 15-19 years, who
atrique Charles De Gaule, Pediatrics, Ouagadougou, Burkina Faso; 4 GFAOP, diagnosed with lymphoma and solid tumors at our department
Villejuif, Paris, France in the last 34 years evaluated. The median age at diagnosis was
16.3 yrs. M/F: 1.2 (115/94). Cancer type distribution was as fol-
Background and Aims: The Bobo-Dioulasso pediatric oncology lows: Hodgkin lymphoma (20.6%), NHL (16.3%), CNS tm (13.4%),
unit was born in the difficult context of unsafety due to ter- nonrhabdo sarcoma (10%), germ cell tm (9.6%), Ewing sarcoma
rorism in Burkina Faso and of Covid19 pandemic. Nevertheless, (7.2%), osteosarcoma (6.7%), others (%16.2). Treatment details
local initiatives and external support allowed its creation and the as follows: Chemotherapy (22%), chemotherapy+radiotherapy
care of over one hundred children affected. The aim of this work (24.4%), chemotherapy+radiotherapy+surgery (19.1%),
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S449 of S635

chemotherapy+surgery (19.1%), radiotherapy+surgery (5.8%), jeopardizes equal and fair supply of quality medical services to peo-
surgery (9.6%). Median follow-up time was 36 mos (1mos-28yrs). ple. Patients in the regional areas will face compromised patient care
Non-compliance to treatment was observed in 8% of patients. Overall because of the lack of skilled human resources and facilities.
survival was 71% and event-free survival was 51% for 5 years. Conclusions: Based on the data collected regarding the current sit-
Conclusions: Adolescent cancer patients are different from younger uation and future obstacles, we are proposing the evidence-based
group. Their treatment begins in pediatric oncology department but recommendation from KSPHO. Furthermore, we anticipate that those
after 18 years of age, it continues in adult clinics. As solution, treatment concerns should be discussed in-depth in public hearings and incorpo-
centers special for AYA patients should be considered. rated into national policy for the sake of pediatric cancer patients in
Korea.

EP652 / #1173 PEDIATRIC CANCER TREATMENT ON THE


VERGE OF COLLAPSE IN SOUTH KOREA: REPORT OF THE EP653 / #1305 HEALTH WORKFORCE NEEDS FOR OPTIMAL
KOREAN SOCIETY OF PEDIATRIC HEMATOLOGY-ONCOLOGY CANCER CARE IN RESOURCE-LIMITED SETTINGS: FACTORS
FOR IMPLEMENTING BETTER POLICY AFFECTING EMPLOYEE PRODUCTIVITY IN UGANDA

Hoon Kook1 , Hyery Kim2 , Hyeon Jin Park3 , Hyoung Jin Kang4 , Keon Sharon Kwagala1 , Amos Obote2 , Alfred Jatho3 , Ezra Anecho4 , Annet
Hee Yoo5 , Kyung-Nam Koh2 , Min Sun Kim4 , Nack-Gyun Chung6 , Nakaganda5 , Jackson Orem6
Young Ae Kim7 , Hyeyoung Shin8 , Ki Woong Sung8 1 Uganda Cancer Institute, Human Resource Department, Kampala,
1 Chonnam National University Medical School, Pediatrics, Hwasun-gun, Uganda; 2 Uganda Cancer Institute, Clinical Services, Kampala, Uganda;
Korea, Republic of; 2 Asan Medical Center, University of Ulsan College of 3 Uganda Cancer Institute, Comprehensive Cancer Community Programme,

Medicine, Pediatrics, Seoul, Korea, Republic of; 3 National Cancer Cen- Kampala, Uganda; 4 Uganda Cancer Institute, Research Directorate, Kam-
ter, Center For Pediatric Oncology, Goyang-si, Korea, Republic of; 4 Seoul pala, Uganda; 5 Uganda Cancer Institute, Cancer Epidemiology And Clinical
National University College of Medicine, Pediatrics, Seoul, Korea, Republic Trials Unit, Kampala, Uganda; 6 Uganda Cancer Institute, Leadership And
of; 5 Sungkyunkwan University College of Medicine, Pediatrics, Seoul, Korea, Management, Kampala, Uganda
Republic of; 6 College of Medicine, The Catholic University of Korea, Pedi-
atrics, Seoul, Korea, Republic of; 7 National Cancer Center, National Cancer Background and Aims: Uganda Cancer Institute (UCI) registers about
Survivorship Center, Goyang-si, Korea, Republic of; 8 The Korean Society of 6000 new cancer cases a year. As the numbers continue to overwhelm
Pediatric Hematology-Oncology, Pediatrics, Seoul, Korea, Republic of the healthcare systems, cancer patients face more challenges in obtain-
ing cancer care services, partly due to human resource needs. This
Background and Aims: South Korean pediatric cancer patients have study was conducted to identify the staff needs that affect the delivery
an 85 percent five-year survival rate, demonstrating world-class cancer of efficient cancer care services at UCI.
treatment. However, decreasing birth rates, low medical costs, and a Methods: We conducted a cross-sectional study in May-July 2019.
shrinking pediatrician population raise concerns regarding future pedi- One hundred and five (105) health workers were randomly selected
atric cancer therapy. This study aims to analyze the current situation using proportionate random sampling. Data was collected using a face-
of pediatric cancer treatment from the Korean Society of Pediatric to-face interview-administered questionnaire. We collected data on
Hematology-Oncology (KSPHO). demographics, contractual terms, and workplace issues (physical, psy-
Methods: KSPHO has been conducting the research since April 2021. chosocial and administrative) affecting employee productivity. Data
The study included extensive data searches, big data analyses on was analyzed using Excel and Stata Version 14.0.
national health insurance claim, in-depth interviews, and reviews on Results: Fifty-three percent (53%) were males and 92.6% were
medical systems of pediatric cancer care from the US, Japan, and the between 20-50 years of age. The majority (82.6%) were clinicians.
Netherlands . Although many indicated that the workspace is adequate (81%) with
Results: Although cancer is the leading cause of mortality in children, suitable facilities and equipment (78%), 34-36% lacked adequate pro-
less social, medical, and political attention has been paid to pedi- tective gear and handwashing facilities and 42% had no Standard
atric cancer patients than to adult counterparts in Korea. In 2018, Operating Procedures in their departments. Ninety-two percent (92%)
1,275 children aged 0-17 years were diagnosed with cancer. The Age- did not have all the required skills to perform their responsibili-
Standardized Rate (ASR) was 146.9, below the global ASR of 151.5. ties and 88% indicated that there are no career development and
A decrease in new pediatric cancer specialists accompanied by low on-job training opportunities to bridge the skills gap for staff. The
birth rates (0.84 by 2020) has increased the burden of present pedi- majority (70-85%) did not have good working relationships with their
atric hemato-oncologists. Additionally, investments in pediatric cancer supervisors and lacked adequate support/feedback/communication
resources are relatively limited because pediatricians are seeing less from their immediate supervisors. Eighty-eight (88%) indicated that
number of cancer patients than adult oncologists with current medi- there is no robust employee performance appraisal system and 74%
cal expenses of minimum consideration of age and severity in Korea. said that there are no policies to promote experienced/skilled staff.
Worsening eccentric centralization of patients to the capital city, Seoul Other challenges included a lack of flexible working arrangements to
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S450 of S635 ABSTRACTS

balance work and family (53.6%); colleagues interfering with their work 1 St. Jude Children’s Research Hospital, Department Of Global Pediatric
(77.6%); and lack of teamwork (70%). Medicine, Memphis, United States of America; 2 University of the Philippines
Conclusions: The limited supply of competent and motivated work- - Philippines General Hospital, Division Of Pediatric Hematology-oncology,
force is a great challenge to the provision of optimal cancer care. Devel- Manilla, Philippines; 3 Children’s Cancer Institute, Southern Philippines
oping dynamic human resource management/development strate- Medical Center, Manilla, Philippines; 4 Cancer Warriors Foundation, Can-
gies/systems should remain a top priority for UCI. cer Warriors Foundation, Manilla, Philippines; 5 Philippines Department of
Health, Central Office, Manilla, Philippines; 6 World Health Organization,
Non-communicable Diseases, Manilla, Philippines
EP654 / #1766 ANALYSIS OF THE IMPLEMENTATION OF
THE GOLDEN HOUR PROGRAM AT A CHILDREN’S HOSPITAL Background and Aims: The National Integrated Cancer Control Act
IN A MIDDLE-INCOME COUNTRY (NICCA) and the Universal Health Care (UHC) Act were enacted in
the Philippines in 2019, the same year Philippines was established as
Merle Laffont-Ortiz1 , Antonio Sandoval-Cabrera2 Western Pacific region (WPR)’s first focus country for the WHO Global
1 Hospital para el Niño del Instituto Materno Infantil del Estado de Méx- Initiative for Childhood Cancer. In an archipelago of over 7000 islands,
ico, Hemato-oncología, Toluca, Estado de México, Mexico; 2 Hospital para el access for children with cancer is a recognized concern.
Niño del Instituto Materno Infantil del Estado de México, Hemato-oncología, Methods: Geospatial, health systems and childhood cancer data were
Toluca, Mexico collated as part of the St. Jude SJCARES Systems tool, Country Vital
Signs. Additional geospatial data was obtained from Google Maps,
Background and Aims: Mexico in Alliance with Sj Jude (MAS) is an ini- Malaria Atlas Project and publicly available facility and road network
tiative that seeks to improve the survival of pediatric cancer patients travel datasets. Data on cancer and health systems for children (age
in Mexico. The "Golden Hour" program aims to administer antibiotics 0-19) were compiled from national datasets and World Bank, WHO
in less than 60 minutes in these patients with febrile neutropenia (FN) World Cancer Report 2020, UN population data and GLOBOCAN
to reduce the risk of life-threatening bacterial infection. Since May 2020.
2019, our hospital has implemented the "Golden Hour" program. This Results: Only 28% of children in the Philippines are within one-hour
study aims to determine the benefits of this implementation regarding travel to a public cancer facility. Disparities were noted between
mortality, admission to the intensive care unit, and days of in-hospital regions, with children in Luzon having 1.6-fold increased likelihood of
stay. being near a public cancer facility compared to children in the Visayas
Methods: We determined the percentage of pediatric cancer patients and Mindanao (p < 0.05). In context, the number of public cancer facil-
with FN event who received antibiotics <60 min, percentage of ities per 10,000 cancer patients, a metric previously associated with
patients who required intensive care admission, mortality per event, cancer outcomes, is 0.4 for the Philippines, and maximum of 700 for
and days of hospital stay in the pre-implementation and post- WPR.
implementation phases. Conclusions: Baseline assessment of access to care in the Philippines
Results: A total of 293 events were analyzed, of which 53 corre- prior to full implementation of NICCA and the UHC Act shows gaps
spond to the pre-implementation and 240 to the post-implementation in access to any health facility as well as to public cancer facilities,
phase. The percentage of patients receiving antibiotics in <60 min with disparities across regions. This approach can serve as a template
increased from 16.9% to 71.6% from the implementation. In-hospital for use of geospatial mapping for health systems analysis, especially
stay reduces from 12.67 (3-49) to 9.82 (2-62) days. Finally, the inter- as governments consider strategies to scale-up investments for chil-
vention reduced the percentage of patients who required critical care dren with cancer. Data generated can support national cancer control
admission from 11.5% to 5.4% and the mortality per event from 5.6% planning and population-sensitive allocation of resources.
to 2.1%.
Conclusions: The implementation of the "Golden Hour" program
reduced the days of in-hospital stay, admission to the intensive care EP656 / #830 HEALTH-INSURANCE INFLUENCE ON
unit, and mortality of pediatric cancer patients with febrile neutropenia TREATMENT OUTCOMES OF CHILDHOOD CANCER IN
in our hospital. WESTERN KENYA

Sandra Langat1 , Saskia Mostert2 , Festus Njuguna3 , Terry Vik4 , Gertjan


EP655 / #1778 GEOSPATIAL ANALYSIS OF GAPS IN ACCESS Kaspers2 , Gilbert Olbara5 , Hugo Martijn6 , Cenne Sieben6 , Moniek
TO CARE IN THE PHILIPPINES USING SJCARES COUNTRY Haverkort6 , Dennis Njenga1
VITAL SIGNS 1 Academic Model Providing Access to Healthcare (AMPATH), Pediatric
Oncology, Eldoret, Kenya; 2 Princess Máxima Center for Pediatric Oncol-
John Spencer1 , Ana Patricia Alcasabas2 , Mae Dolendo3 , Carmen ogy, Pediatric Oncology, Utrecht, Netherlands; 3 Moi University, Pediatric
Auste4 , Clarito Cairo5 , Stella Osorio6 , Sri Andini Handayani1 , Oncology, Eldoret, Kenya; 4 Riley Hospital for Children, Pediatrics, Indi-
Catherine G. Lam1 anapolis, United States of America; 5 Moi Teaching and Referral Hospital,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S451 of S635

Pediatric Oncology, Eldoret, Kenya; 6 Emma’s Children Hospital, Pediatrics, atric patients. SOS is a common sequelae of conditioning regimens for
Amsterdam, Netherlands hematopoietic stem cell transplantation with well-described epidemi-
ology and pathophysiology. However, there is a paucity of literature
Background and Aims: Only few government leaders in low and examining SOS secondary to chemotherapy. We conducted a system-
middle-income countries have responded favourably to the interna- atic review of pediatric non-transplant SOS and examined etiology,
tional plea for Universal Health Coverage by the UN, WHO and management, and outcomes.
over 500 health-organisations. Survival of childhood cancer in low Methods: Four databases were searched for non-transplant SOS in
and middle-income countries is often less than 20%. Limited health- pediatric cancer patients between 2000–2021. Two reviewers each
insurance coverage may contribute to these poor survival rates. Our performed screening and full text review before data abstraction. Arti-
study explores the influence of health-insurance status on childhood cles were retained if they included pediatric patients with cancer who
cancer treatment in a Kenyan academic hospital. experienced non-transplant SOS.
Methods: Medical records of all children diagnosed with cancer Results: We screened 2301 records and reviewed 195 full-texts. Of
between 2010 and 2016 were reviewed retrospectively and data on these, 80 articles were included, yielding preliminary data from 546
treatment outcomes (absolute values at time of analysis) and health- patients with SOS: 41 (8%) female, 48 (9%) male, and 457 (84%)
insurance status at diagnosis and during treatment were collected. patients of unknown sex. Mean age was 5.15 years with a range of
Results: Of 763 patients, 28% abandoned treatment, 23% died and one month to 17 years. Underlying diagnoses included ALL (61%),
17% had progressive or relapsed disease resulting in 32% event-free Wilms tumour (WT) (18%), AML (2%), medulloblastoma (2%), and
survival. In total 280 patients (37%) had health-insurance coverage other malignancies (17%). Most common inciting chemotherapy agents
at diagnosis and 483 patients (63%) did not. Of patients without included 6-thioguanine (55%), actinomycin-D (21%), vincristine (17%),
health-insurance coverage, 299 patients (62%) enrolled during can- and cyclophosphamide (10%). SOS was predominantly managed with
cer treatment leading to a total health-insurance registration level supportive measures, however, 98 (20%) patients also received defi-
of 579 patients (76%). Treatment outcome of patients differed per brotide. The overall SOS survival rate was 90%, with recurrence of SOS
health-insurance status (P<0.001). The most likely treatment outcome in three patients. Twenty-five patients died due to SOS and nine as a
in uninsured patients was death (49%) whereas in those with health- result of underlying diagnoses or infection. In patients with ALL and
insurance at diagnosis and those who enrolled during treatment it was WT, survival rates were 99% and 86%, respectively.
event-free survival (36% and 41% respectively). The overall survival Conclusions: This review demonstrates trends in non-transplant SOS.
(P<0.001) and event-free survival (P<0.001) was significantly higher ALL is a frequent underlying cancer diagnosis in SOS, along with WT.
for patients with health-insurance compared to those without. Hazard- Conventional chemotherapies, such as 6-thioguanine, actinomycin-
ratio for treatment failure was 0.30 (95% CI: 0.22-0.39; P<0.001) D, vincristine, and cyclophosphamide have been observed as com-
for patients insured at diagnosis in relation to those without health- mon offending agents. Notably, non-transplant SOS resolves in most
insurance and 0.32 (95% CI: 0.24-0.4,1; P< 0.001) for patients insured patients, largely due to supportive care and novel treatments, such as
during treatment in relation to those without insurance. defibrotide.
Conclusions: Our study findings underline the need for Universal
Health Coverage in low and middle-income countries. Children without
health-insurance had significantly lower chance of event-free and over- EP658 / #1370 THE ST. JUDE PEDIATRIC ONCOLOGY
all survival. Childhood cancer treatment outcomes can be ameliorated FACILITY INTEGRATED LOCAL EVALUATION (PROFILE) BETA 2
by strategies that improve health-insurance access. TESTER EXPERIENCE IN A TERTIARY CENTRE AT KUALA
LUMPUR, MALAYSIA

EP657 / #907 NON-TRANSPLANT HEPATIC SINUSOIDAL C-Khai Loh1 , Sie Chong Doris Lau1 , Norziana Mohd Anuar2 , Noriza
OBSTRUCTION SYNDROME IN PEDIATRIC ONCOLOGY: A Mansor2 , Geok Chin Tan3 , Yin Ping Wong3 , Hafiza Alauddin3 , Marjmin
SYSTEMATIC REVIEW Osman4 , Charng Jeng Toh4 , Fuad Ismail5 , Wai Wai Yang1 , Faizah Mohd
Zaki6 , Patricia Belda7 , Heather Forrest7 , Miriam Gonzalez-Guzman7 ,
Marc Lawrence1 , Janet Wilson1 , Katie O’Hearn2 , Tamara Rader3 , Paola Friedrich7 , Hamidah Alias1
Donna Johnston4 1 Universiti Kebangsaan Malaysia Medical Centre, Department Of Paedi-
1 University of Ottawa, Faculty Of Medicine, Ottawa, Canada; 2 Children’s atrics, Kuala Lumpur, Malaysia; 2 Universiti Kebangsaan Malaysia Medi-
Hospital of Eastern Ontario, Research Institute, Ottawa, Canada; cal Centre, Department Of Nursing, Kuala Lumpur, Malaysia; 3 Universiti
3 Independent Information Specialist, N/a, N/A, Canada; 4 Children’s Kebangsaan Malaysia Medical Centre, Department Of Pathology, Kuala
Hospital of Eastern Ontario, Division Of Hematology/oncology, Ottawa, Lumpur, Malaysia; 4 Universiti Kebangsaan Malaysia Medical Centre,
Canada Department Of Surgery, Kuala Lumpur, Malaysia; 5 Universiti Kebangsaan
Malaysia Medical Centre, Department Of Oncology And Radiotherapy,
Background and Aims: Hepatic sinusoidal obstruction syndrome Kuala Lumpur, Malaysia; 6 Universiti Kebangsaan Malaysia Medical Cen-
(SOS) is a rare and potentially fatal liver disease often seen in pedi- tre, Department Of Radiology, Kuala Lumpur, Malaysia; 7 St. Jude Children’s
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S452 of S635 ABSTRACTS

Research Hospital, Global Pediatric Medicine, Memphis, United States of improvement. PrOFILE, created by the St. Jude Global Metrics and Per-
America formance Unit, is a dynamic 360◦ assessment of health services that
helps teams and institutions define quality improvement strategies.
Background and Aims: Biological and non-biological factors con- The main objective is to gather data needed to understand care deliv-
tribute to pediatric cancer outcomes. Most non-biological factors are ery and develop an action plan prioritizing the areas that most require
amenable to improvement through quality improvement interven- attention.
tions. The Universiti Kebangsaan Malaysia Medical Centre (UKMMC) Methods: PrOFILE was implemented from August 2021 to May 2022
joined the second PrOFILE beta testing cohort to assess actionable at Barretos Children’s Cancer Hospital (BCCC) with guidance from the
non-biological factors and identify opportunities for improvement in St. Jude team. The internal assessment team included a lead physician,
pediatric hematology-oncology (PHO) care delivery. a local coordinator, and 31 local multidisciplinary staff. The 12 modules
Methods: We conducted PrOFILE between August 2021 and March of the tool were completed using DATSTAT (26 electronic forms). Six
2022. The site coordinator (SC) and physician lead (PL) participated in quality improvement exercises were conducted. A final report was pro-
weekly online mentoring sessions. A multidisciplinary team was invited duced by PrOFILE team with quantitative and qualitative data. Tables
to complete the 12 PrOFILE modules. Twenty-six electronic forms col- and graphs for each module identified opportunities for improvement
lecting objective and subjective data were entered in DatStat. The SC that will be discussed at a local workshop in May 2022. Five opportu-
and PL completed 12 asynchronous educational PrOFILE modules, six nities will be identified that will guide action plans for the next three
quality improvement (QI) exercises, a basic certificate in QI methods, years.
and provided feedback on preliminary reports. A 2-day prioritization Results: The form completion rate for the objective data (collected
workshop to define the institutional 3-year action plan was conducted by the site coordinator and approved by the lead physician) and for
using PrOFILE results. the subjective data (31 participants) was 100%. In the score calcula-
Results: Thirteen PHO providers formed the assessment team. The tion for each module, BCCC scored > 75 % in eleven modules and
form completion rate was 100%. All the 12 modules scored between 50 71% in the facility and local context. Finances and resources and radia-
- 75%. Service capacity and facility and local context scored above 70%, tion therapy modules scored the highest (98% and 92%, respectively).
while national context and service integration scored lowest. We found In the impressions section, 146 opportunities for improvement were
an important discrepancy between the SC/PL and the point of care highlighted.
staff’s awareness of institutional planning (100% vs. 27%). An average Conclusions: Conducting PrOFILE provided the local team with insight
of 13 opportunities per PrOFILE module were identified; the person- on how to collect and use our data, promote change and a culture of
nel module had the highest number. Opportunities prioritized during quality locally, plan new projects and disseminate our experience.
the workshop include but are not limited to creating a dedicated ambu-
latory area for PHO patients, improving the overall nurse: patient ratio
and nurse skill mix, promoting access to written protocols/guidelines EP660 / #718 THE BENCHISTA PROJECT: INTERNATIONAL
for safe chemotherapy handling, implementing multidisciplinary care BENCHMARKING OF CHILDHOOD CANCER SURVIVAL BY
meetings for all patients, and a non-punitive standard process to track TUMOUR STAGE
major adverse patient events.
Conclusions: PrOFILE allowed UKMMC relevant stakeholders and Angela Lopez Cortes1 , Laura Botta2 , Fabio Didonè2 , Adela Canete3 ,
the PHO team to systematically capture the impact of non-biological Charles Stiller4 , Lisa Hjalgrim5 , Zsuzsanna Jakab6 , Bernward Zeller7 ,
factors and generate consensus and momentum for addressing them. Gemma Gatta2 , Kathy Pritchard-Jones8 , Benchista Project Working
Group9
1 UCL GOS Institute of Child Health, Ucl Developmental Biology & Cancer
EP659 / #1530 USING THE PEDIATRIC ONCOLOGY FACILITY Research Department, London, United Kingdom; 2 Fondazione IRCCS "Isti-
INTEGRATED LOCAL EVALUATION (PROFILE) TOOL: tuto Nazionale dei Tumori di Milano", INT, Department Of Preventive And
EXPERIENCE AT BARRETOS CHILDREN’S CANCER HOSPITAL IN Predictive Medicine, Milan, Italy; 3 Hospital UiP La Fe, Paediatric Oncol-
BRAZIL ogy And Hematology Unit, Valencia, Spain; 4 NHS Digital, National Cancer
Registration And Analysis Service, Oxford, United Kingdom; 5 Rigshospitalet,
Mario Paula1 , Cristina Amendola1 , Patricia Belda2 , Heather Forrest2 , Department Of Pediatrics And Adolescent Medicine, Copenhagen, Den-
Miriam Gonzalez-Guzman2 , Paola Friedrich2 , Luiz Fernando Lopes1 mark; 6 Semmelweis University, 2nd Department Of Pediatrics, Budapest,
1 Barretos Children’s Cancer Hospital, Pediatric Hematology-oncology Unit, Hungary; 7 University Hospital, Oslo, Division Of Pediatric And Adolescent
Barretos, Brazil; 2 St. Jude Children’s Research Hospital, Global Pediatric Medicine„ Oslo, Norway; 8 UCL GOS Institute of Child Health, Develop-
Medicine, Memphis, United States of America mental Biology & Cancer Research Department, London, United Kingdom;
9 BENCHISTA, Pwg, London, United Kingdom

Background and Aims: Both biological and non-biological factors


determine overall survival in pediatric oncology. Systematic evalua- Background and Aims: Variation in stage at diagnosis of childhood
tion of non-biological factors allows identification of opportunities for cancers (CC) may explain variations in survival rates observed between
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S453 of S635

countries. This project aims to understand these differences and to Methods: Through an IRB-approved protocol, the LFS cohorts at a
encourage the application of the Toronto Staging Guidelines (TG) by pediatric (CHOP) and adult (UPenn) tertiary-care institution were
Population-Based Cancer Registries (PBCRs) to the most common reviewed to identify individuals who had undergone two or more
solid paediatric cancers. whole-body imaging screenings. For each screening, clinical response
Methods: PBCRs within and outside Europe have been invited to was recorded (no intervention needed, imaging follow up either imme-
participate in the BENCHISTA Project. PBCRs will identify all cases diately or in the short term, biopsy, or surgery), and analyzed on initial
of Neuroblastoma, Wilms tumour, Medulloblastoma, Ewing Sarcoma, vs subsequent scans. Cancers were identified as related to first vs
Rhabdomyosarcoma, and Osteosarcoma diagnosed in a consecutive subsequent screening.
three-year period within 2014-2017 and apply the TG at diagnosis. Results: A total of 68 adult patients (28% male) and 50 pediatric
Other non-prognostic factors, treatment and recurrence/progression patients (42% male) were identified as having undergone multiple full-
will be collected. A minimum of 3-year follow-up has to be assured. body screenings. On initial screening, a total of 38% of the adult cohort
Results: Sixty-four PBCRs from thirty countries have committed to and 20% of the pediatric cohort required intervention. On subse-
participate and have agreed a maximally depersonalised, patient-level quent screening, a total of 19% of both cohorts required intervention.
data collection format. Forty-four require a Data Transfer Agreement The most frequent intervention after screening was short-term fol-
(DTA) to comply with data protection regulations; for twenty PBCRs low up imaging. A total of 13 cancers were detected (6 pediatric, 7
the data format and ethical approval are sufficient for data transfer. adult); cancer detection rate was similar between first and subsequent
Due to heterogeneity encountered in legal aspects, fifteen months screenings.
were spent on finalising the DTA; data transfers started in March Conclusions: Rate of intervention after screening was lower in subse-
2022. To standardise TG application by cancer registries, three on-line quent screenings for adult patients, and remained similar for subse-
training workshops led by six tumour-specific clinical experts were quent screenings for pediatric patients. Rate of cancer detection on
held (https://www.ucl.ac.uk/child-health/research/developmental- annual screening was consistent with prior studies and was similar
biology-and-cancer/benchista/training-and-workshops). Based on the between first and subsequent screenings. Next steps to include anal-
pilot study, a total of >8,000 staged cases are expected; stage distri- ysis of screening to include other modalities, especially ultrasound and
bution and survival analyses will be conducted by large geographical colonoscopy.
regions comparable to prior EUROCARE studies.
Conclusions: Despite efforts to harmonise General Data Protection
Regulations across Europe, multiple differences in interpretation and EP662 / #1593 CURRENT STATUS OF PEDIATRIC CANCER
required processes were encountered, causing delays to data transfer. TREATMENT SYSTEM IN JAPAN EVALUATED BY THE QUALITY
The Benchista project has achieved a large-scale collaboration across INDICATORS (QI) FOR CORE AND AFFILIATED HOSPITALS
PBCRs and with their clinical data sources to assign stage at diagnosis
according to international consensus guidelines. This is a key aspect for Kimikazu Matsumoto1 , Hiroyuki Fujisaki2 , Hiromi Komatsu1 , Kenji
improving patient outcomes and stimulating research. Hirai3 , Akihiro Yoneda4 , Miho Kato1 , Tetsuya Takimoto1
1 National Center for Child Health and Development, Children’s Cancer Cen-

ter, Tokyo, Japan; 2 Osaka City General Hospital, Pediatric Hematology And
EP661 / #1662 INTERVENTIONS IN FIRST VERSUS Oncology, Osaka, Japan; 3 Osaka Women’s and Children’s Hospital, Health
SUBSEQUENT FULL BODY SCREENING IN LI-FRAUMENI Information Management Office, Osaka, Japan; 4 National Center for Child
SYNDROME Health and Development, Surgical Oncology, Tokyo, Japan

Suzanne Macfarland1 , Yun Du2 , Laura Kagami1 , Conrad Fernandes2 , Background and Aims: In Japan 15 core hospitals (CHs) for pedi-
Anh Le2 , Madeline Good2 , Melani Duvall1 , Sarah Baldino1 , Jacquelyn atric cancer treatment were elected by the Ministry of Health, Labour
Powers2 , Kristin Zelley1 , Bryson Katona2 , Kara Maxwell2 and Welfare and they designated affiliated hospitals (AHs) in seven
1 Children’s Hospital of Philadelphia, Pediatric Oncology, Philadelphia, regional blocks. We established two quality indicators (QI) to evaluate
United States of America; 2 University of Pennsylvania, Medicine, Philadel- separately 15 CHs and 105 AHs categorized as local main hospitals for
phia, United States of America pediatric cancer treatment. The aim of this study was to analyze the
current status of pediatric cancer treatment system in Japan and to
Background and Aims: Li-Fraumeni Syndrome is a hereditary cancer verify the QIs for CHs and AHs.
predisposition syndrome caused by pathogenic changes in the TP53 Methods: Thirty-one indicators for CHs (11 structure, 15 process and 5
gene. Lifetime cancer risk is between 80-90%, with up to 40% risk of outcome index) and 21 indicators for AHs (10 structure, 8 process and
cancer in childhood. Screening protocols which include annual whole 3 outcome index) were developed in 2015 and 2020 respectively. Data
body and brain MRI have been effective in improving survival through from 15 CHs (100%) and 105 AHs (96.3%) were collected in 2021 and
early cancer detection. Several studies have shown a high rate of can- the common indicators were compared.
cer detection on baseline screening, but as yet none has compared rate Results: For structure indicators, the average number of pediatric can-
of detection on first versus subsequent screenings. cer specialists differed significantly (CHs 5.2, AHs 1.74, p<0.0001).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S454 of S635 ABSTRACTS

Similarly, the average number of certificated surgeons in pediatric nerve sheath tumor-1. Twenty patients (57%) were asymptomatic, the
oncology and certified Child Life Specialists (including the Hospital Play rest of the patients had fever, sore throat and cough. Four patients with
Specialists and Child Care Staffs) differed significantly (CHs 1.93, 2.33; hematological malignancies developed pneumonia, followed by cancer
AHs 0.52, 0.53, respectively. p<0.0001). For process indicators, hospi- progression in two of them. Pancytopenia/thrombocytopenia was
tal days of ALL patients were 58.1 for CHs and 72.6 for AHs (p=0.3815) found in 4 patients probably infected with Omicron variant during the
and the intervention rates by palliative care team were 5.3% for CHs last three months. Overall, the incidence of COVID-19 complications
and 11.0% for AHs (p=0.3311). Outpatient follow-up system of pedi- was 11%, mortality was 0.
atric cancer survivors was equipped in 100% of CHs but only 51.4% for Conclusions: This analysis showed that the rate of severe COVID-
AHs (p=0.0001). 19 infection and mortality among children with cancer in Armenia
Conclusions: There existed obvious difference between CHs and AHs was lower than global estimates and further studies to explore these
in structure indicators, which would reflect the number of pediatric differences are emerging.
cancer patients. To the contrary, difference in process indicators, such
as palliative care or hospital days of ALL, was small, which could partly
be interpreted as treatment of ALL is almost equalized by JCCG in EP664 / #1040 COVID-19 IN CRITICALLY ILL ONCOLOGY
Japan. PEDIATRIC PATIENTS: EXPERIENCE IN A LIMITED-RESOURCE
SETTING

EP663 / #1086 COVID-19 INFECTION IN CHILDREN WITH Sergio Valle, Alejandra Méndez-Aceituno, Juan Carlos Xicay, Thelma
CANCER IN ARMENIA: REPORT FOR THE WHOLE PANDEMIC Velasquez, Ricardo Mack
PERIOD National Unit of Pediatric Oncology, UNOP, Pediatric Critical Care,
Guatemala, Guatemala
Irina Melnichenko1,2 , Ruzanna Papyan1,2 , Yeva Margaryan3 , Elya
Minasyan1,2 , Mariam Minasyan1,2 , Liana Hambardzumyan2 , Lusine Background and Aims: Background: From the beginning of COVID-
Hakobyan1,2 , Samvel Iskanyan1 , Samvel Danielyan1 , Lala 19 pandemic pediatric oncology patients (POP) have been considered
Vagharshakyan1,2 , Gevorg Tamamyan1,2 , Lilit Sargsyan1,2 at considerable risk of develop severe disease and mortality. The risk
1 Hematology Center After Prof. R.H. Yeolyan, Pediatric Cancer And Blood becomes even higher in a limited resource setting. Previous reports
Disorders Center Of Armenia, Yerevan, Armenia; 2 Yerevan State Medical have described the experience of COVID-19 in general population,
University, Department Of Pediatric Oncology And Hematology, Yerevan, however there is not enough evidence of COVID-19 in POP requiring
Armenia; 3 Institute of Cancer and Crisis, Institute Of Cancer And Crisis, critical care. The aim of this work is to describe the experience with crit-
Yerevan, Armenia ically ill POP infected with COVID 19 in a limited resource oncology
hospital in Guatemala, Central America.
Background and Aims: As pediatric patients with cancer are fre- Methods: We perform a review of our internal data base, identifying
quently immunocompromised, they may be more vulnerable to severe POP with COVID-19 diagnosed (by PCR-RT) at PICU admission. Demo-
COVID-19 infection than other children. In a global registry study graphic (age, sex), epidemiology (oncological diagnosis, treatment
of COVID-19 in childhood cancer including 1500 patients, 20% had phase), laboratory [Ferritin, Pro-BNP, D dimer (DD)] data and critical
severe or critical infection, and mortality equal to 4% was higher interventions [mechanical ventilation (VM), sequential organ failure
than that in the general pediatric population. Data about development assessment score (SOFA)] were reviewed and compared between
of COVID-19 complications in children with cancer are limited and survivors and non-survivors.
variable worldwide. The study aimed at describing the incidence of Results: From 371 POP diagnosed with COVID-19 and admitted, 66
COVID-19 infection in children with cancer in Armenia. required PICU between June 2020 and December 2021, oncology
Methods: Prospective analysis of PCR confirmed cases of COVID-19 diagnosis was acute lymphoblastic leukemia (ALL) in 39 POP (59%) and
infection in children with cancer aged 0-18 years during 2020-2022 62% were receiving induction chemotherapy. The overall mortality was
were performed in the Pediatric Cancer and Blood Disorders Center 18%, non survivors (n= 21) had an older median age (12.5 vs 8) in years
of Armenia created in 2019 as a result of the union of three medical and required MV more frequently (57% vs 9%). The median values
units. Based on the fact that it is the only center treating children with of ferritin (1,492ng/mL), Pro-BNP (4,450pg/mL) and DD (2,717ng/mL)
cancer, this is the first report involving all children with cancer infected were higher in the non-survivor’s group, although these tests were not
with COVID-19 during this entire period. performed in every patient. Non survivors also had more organ failure
Results: Between June 2020 and March 2022 of 201 children with with a median pSOFA/SOFA score of 10 points and 2 patients received
cancer 35 cases of COVID-19 infection were confirmed in Armenia. high frequency oscillatory ventilation.
Median age was 8.4, male/female ratio was 1.3. Acute lymphoblas- Conclusions: Patients with ALL in induction phase tend to be
tic leukemia was diagnosed in 15, neuroblastoma-4, lymphoma-5, more likely to develop severe COVID-19 disease. Non-survivors
medulloblastoma-2, Ewing sarcoma-2, rhabdomyosarcoma-2, group required more MV, had more organ failure and higher
osteosarcoma-1, acute myeloid leukemia-1, malignant peripheral values of Pro-BNP, DD and ferritin than survivors. Further
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S455 of S635

studies are needed to determine the statistic significance of these 1 Cuddles Foundation, Research, Knowledge Management And Impact,
findings. Mumbai, India; 2 Cuddles Foundation, Cuddles Institute Of Clinical Nutrition
(cicn), Mumbai, India

EP665 / #1161 COVID-19 INFECTION IN CHILDREN AND Background and Aims: Malnutrition at diagnosis in paediatric oncol-
ADOLESCENTS WITH CANCER DURING THE SECOND AND ogy patients can result in poor outcomes and treatment abandonment.
THIRD WAVE OF THE PANDEMIC IN INDIA: A TERTIARY Children from lower socioeconomic statuses (SES) are at a higher risk
CENTER EXPERIENCE of being malnourished and therefore, Cuddles Foundation (CF) sup-
ports them by providing nutritional aid and supplements. This study
Sonali Mohapatra1 , Debasish Sahoo1 , Baijayantimala Mishra2 explores the association between SES and nutritional status (NS) in
1 All India Institute of Medical Sciences, Bhubaneswar, Medical Oncol- paediatric oncology patients in government hospitals in India.
ogy/hematology, Bhubaneswar, India; 2 All India Institute of Medical Sci- Methods: Self-reported data of CF beneficiaries across 23 hospitals
ences, Bhubaneswar, Microbiology, Bhubaneswar, India were collected in June 2021 and beneficiaries were categorised into
different SES groups basis three parameters; total household income
Background and Aims: Children with cancer are at greater risk for of all earning members, education level and occupation of the head of
COVID-19 infection than their healthy counterparts. We had reviewed household. The Kuppuswamy scale, an established tool used to deter-
68 pediatric cancer patients with COVID-19 in the first wave of mine SES in hospital patients in India, was used. The beneficiary’s NS
the pandemic. This study evaluates an additional 52 children and at diagnosis available on the FoodHeals
R app was then correlated

adolescents with COVID-19 during the second and third waves in India. to their SES. The NS was determined using MUAC or BMI for age
Methods: Data of 52 (37 and 15 in second and third waves, respec- indices and beneficiaries were then categorised as undernourished,
tively) children and adolescents with cancer and under treatment at well-nourished, or overnourished.
our center with RT-PCR confirmed COVID-19 between April-2021 and Results: A total of 667 patients participated in this study. The majority
February-2022 were analyzed. Results were compared with the first of beneficiaries (75%) were categorised into the lower and upper-lower
wave. SES. With regard to NS, our findings indicate that patients in the lower
Results: The median age was 4.7 years with a male to female ratio SES category had the highest percentage of undernourishment (66%)
of 1.6:1. The most common (80.7%) cancer type was acute leukemia. as compared to the upper-lower (58%), middle (55%) and upper-middle
Forty (77%) patients were on myelosuppressive chemotherapy, and classes (47%). As expected, the upper-middle class had the highest
23% were on less intense maintenance chemotherapy. COVID-19 percentage of well-nourished (52%) compared to the other classes,
was asymptomatic/mildly symptomatic in 48 (92.3%) patients and in particular the lower class (33%). The strongest negative correla-
severe/critical in only 1 (1.9%) patient. Coryza/cough was the common- tion was found between SES and undernutrition based on the Pearson
est symptom, and fever was observed in only 40% of cases. One patient correlation test (r = −.64).
with Acute Myeloid Leukemia (AML) and severe/critical COVID-19 and Conclusions: This study indicates that there is a negative correlation
associated neutropenic-enterocolitis succumbed. There was a delay in between SES and undernutrition in pediatric oncology patients. This
treatment in 63% of patients, and the median duration of delay was 21- emphasises the urgent need for nutrition interventions to address the
days. Three (5.8%) patients were repeat positives, and the median time health disparities experienced by children from a lower SES.
to achieve negative RT-PCR was 13 days.
Conclusions: Despite the apprehension of more severe diseases in
children with cancer, our study found that the number of cases and EP667 / #1398 INTRODUCTION TO PEDIATRIC ONCOLOGY
COVID-19 related mortality (4.4% vs. 1.9%) has decreased after the BLENDED CURRICULUM: PILOTING A TIERED APPROACH IN
first wave. We observed a change in clinical symptoms with more chil- SUB-SAHARAN AFRICA
dren having coryza/cough than fever. However, there was no change
in the cancer types and severity of COVID-19. Patients with AML and Daniel Moreira1 , Miguel Bonilla1 , Saman Hashmi1 , Leeanna Fox
COVID-19 and concurrent neutropenic sepsis may be at increased risk Irwin1 , Justin Mulindwa2 , Inam Chitsike3 , Nickhill Bhakta1 , Uma
of morbidity/mortality. Though there was a decrease in the median Athale4
duration of delay in treatment (28-days vs. 21-days), it remains a 1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis,

concern. United States of America; 2 Cancer Disease Hospital, Pediatric Oncology,


Lusaka, Zambia; 3 University of Zimbabwe, Dept Child & Adolescent Health,
Harare, Zimbabwe; 4 McMaster University, Pediatrics, Hamilton, Canada
EP666 / #924 ROLE OF SOCIOECONOMIC STATUS IN
MALNUTRITION IN PEDIATRIC ONCOLOGY PATIENTS IN Background and Aims: Training of future professionals is crucial for
GOVERNMENT HOSPITALS IN INDIA the care of children with cancer. However, workload and compet-
ing responsibilities in low- and middle-income countries (LMICs) can
Anju Morarka1 , Sripriya Venkiteswaran2 limit structured approaches to training. This blended curriculum was
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S456 of S635 ABSTRACTS

conceived to complement institutional education programs for the Background and Aims: Task sharing has been implemented as a
management of children with cancer. response to the shortage of healthcare professionals caring for chil-
Methods: A curriculum development group was formed between sub- dren with cancer in low- and middle-income countries (LMICs). This
Saharan African institutional educators and North American pediatric study sought to describe the tasks assigned to non-oncologists caring
hematologists/oncologists. The curriculum was developed based on for children with cancer and evaluate their comfort level in providing
the needs of local institutions, level of trainees, and pediatric oncology care.
rotation timeframe. Methods: This multicenter study was undertaken at 10 institutions
Results: The curriculum identified 3 levels of learners: a) pediatric in 5 countries. Two surveys were employed to: 1) identify the mod-
residents in training (Level 1); b) physicians, (community- or hospital- els used to implement task sharing, and 2) evaluate the confidence of
based) caring for children with cancer (Level 2); c) fellows or profes- non-specialists working in pediatric cancer units.
sionals committed to pediatric oncology (Level 3). The curriculum for Results: Of the professionals that are engaged in task sharing at
Level 1 learners was piloted at two sites in sub-Saharan Africa to eval- these institutions, general physicians and pediatricians were the most
uate the feasibility, acceptance, and appropriateness of the blended commonly mentioned. Most hospitals have task sharing professionals
curriculum. Overview of the E-Poster Topics was provided by online cover the inpatient (90%) and outpatient (80%) units. These pro-
pre-recorded lectures delivered by experts in the field through the fessionals participate in many tasks, including cancer diagnosis and
Cure4Kids platform. This was complemented by high-yield self-paced risk-stratification (40%), selecting initial chemotherapy plans for new
reading material and context-relevant case-based discussions by an in- diagnosed patients (50%), and modifications of chemotherapy based
person moderator to consolidate the concepts using problem-based on toxicities (60%). These professionals can prescribe chemotherapy
learning. Supervision of delivery of the curriculum and monitoring the (80%). Furthermore, they can perform common procedures in pediatric
progress of learners was the responsibility of local preceptors. The oncology (90%). These professionals usually discuss patients with pedi-
curriculum has been delivered to two groups of pediatric residents atric oncologists (90%). For the individual survey, 15 physicians and
(n=7) at two sites simultaneously allowing educational collaboration. 2 nurses provided responses. Most respondents were very comfort-
The blended curriculum allowed continuation of structured learning able or comfortable with the responsibilities of the scope of practice.
experience during the COVID pandemic. Defining the initial treatment plan and staging of cancers (24%) had
Conclusions: It is feasible to develop and implement a tiered pediatric the greatest number of respondents who felt uncomfortable or very
oncology curriculum combining in-person and asynchronous learning. uncomfortable with these tasks. Respondents mentioned that the
This curriculum was well accepted by local learners and teachers; hardest part of their jobs included the number of patients need to be
quantitative and qualitative data are being collected for evaluation of seen and their complexity and the emotional toll of their job.
the program for modifications prior to broader use. Such curriculums Conclusions: Capable non-oncologists are frequently responsible for
maybe helpful educational resources for programs in LMICs to provide essential steps in the pediatric cancer care continuum. These data can
training opportunities. provide insight into the implementation strategies for task sharing as a
generalizable approach to scale up pediatric cancer care in LMICs.

EP668 / #1571 OPTIMIZATION OF WORKFORCE IN


PEDIATRIC CANCER UNITS IN LOW- AND MIDDLE-INCOME EP669 / #1055 SIGNIFICANT IMPROVEMENT IN SURVIVAL
COUNTRIES: THE EVALUATION OF TASK SHARING AND STABLE INCIDENCE RATE IN CHILDHOOD CANCER IN
ARGENTINA: ARGENTINE ONCOPEDIATRIC REGISTRY-INC
Daniel Moreira1 , Jeremy Slone2 , Allyson Andujar1 , Rosdali Diaz (ROHA-NET). 2000-19
Coronado3 , Helder De Quintal4 , Yessika Gamboa5 , Mariana Kruger6 ,
Roy Rosado7 , Carlos Rodriguez-Galindo8 Florencia Moreno1 , Agustina Chaplin1,2 , Blanca Diez3 , Marcelo
1 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis, Sopinaro1 , Maria G. De Davila1 , Mercedes Garcia Lombardi4 , Dora
United States of America; 2 St. Jude Children’s Research Hospital, Oncology, Loria1
Memphis, United States of America; 3 Instituto Nacional de Enfermedades 1 National Cancer Institude-Argentina, Oncopediatric Registry, Buenos
Neoplásicas, Department Of Pediatric Oncology, Lima, Peru; 4 Red Cross Aires, Argentina; 2 National Cancer Institude-Argentina, Oncopediatric,
Ward Memorial Children’s Hospital, Paediatrics And Child Health, Cape Buenos Aires, Argentina; 3 FLENI, Neuroncology, CABA, Argentina; 4 Hospital
Town, South Africa; 5 Hospital Nacional de Niños, Pediatric Oncology, Pedi- de Niños Ricardo Gutierrez, Pediatric Oncology, Buenos Aires, Argentina
atric Oncology, San José, Costa Rica; 6 Stellenbosch University and Tygerberg
Hospital, Department Of Paediatrics And Child Health, Cape Town, South Background and Aims: To present incidence and survival trends in
Africa; 7 Unidad Nacional de Oncología Pediatrica, Pediatric Hematology- childhood cancer in Argentina in the period 2000-19.
oncology, Guatemala, Guatemala; 8 St Jude Children’s Research Hospital, Methods: ROHA is a population-based hospital registry that has been
Global Pediatric Medicine, Memphis, United States of America active since 2000 and is part of the National Pediatric Program at the
Ministry of Health’s National Institute of Cancer. ROHA’s network data
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S457 of S635

comes from different sources; most are reported by pediatric oncology power for each tumor, and according to the combination of answers
units from all regions in the country. Estimated coverage is 91%. . and their pre-established cut-off points, allowing defined capacity and
Results: In the period 2000-19, 27,016 new cases of cancer were category of POU by tumor. The document has to be completed by hos-
recorded with a ASR 131.6. Between 2000 and 2019, a modest or no pital authorities and a multidisciplinary team. All 30 public POUs were
change in ASR was observed for all common pediatric cancer diag- invited to participate with the ST.
noses (APC: 0.25%; 95% CI [0.2; 0.5]; p=0.0), except for a descent in Conclusions: The ST was carried out by consensus and is a feasible tool
Lymphomas. In the period 2005-2014 (n:13597), the five-year OS for to identify the local capacity for each tumor in the POUs, allowing a
all tumors was 67.6% (66.8-68.4). A three-year OS was observed in stratification of the Argentine oncopediatric public network to achieve
2000-05 (n:7689); 2006-11 (n:7969); and 2012-16 (n:7211). 64.0% adequate, timely and quality care.
(63.0-65.1); 69.7% (68.7-70.7); and 72.7% (71.7-73.7). Lymphoblastic
Leukemia: (n:2254) 76.0% (74.1-77.7); (n:2237) 69.0% (67.1-70.8); and
(n:2175) 76.1% (74.2-77.8). EP671 / #739 DEPLOYMENT OF RESONANCE PATIENT
Conclusions: Incidence rates are comparable to Latin American ones CENTER FOR DATA MANAGEMENT IN A NATIONAL PEDIATRIC
and lower than those in USA and European countries. There is no CANCER UNIT IN URUGUAY
substantial change in incidence for the major pediatric cancers, and
rates have remained relatively stable. Survival is inferior to what was Valeska Tenorio1 , Anaulina Silveira2 , Diana Vargas3 , Ryan Combs4 ,
observed in more developed countries. There were many initiatives Jennifer Lowe4 , Fabiana Morosini5 , Claudio Libertelli6 , Guillermo
developed by the National Cancer Institute, the scientific community, Chantada7 , Aman Patels4 , Mariela Castiglioni1 , Scott Howard4 , Luis
cooperative groups, and non-governmental organizations such as early Castillo5
diagnosis programs, standardization of clinical support practices, and 1 Pereira Rosell Hospital Pérez Scremini Fundation, Montevideo/UY, Monte-

continuing nursing education that may have contributed to improving video, Montevideo, Uruguay; 2 Pereira Rosell Hospital Pérez Scremini Foun-
survival. dation, Montevideo, Montevideo, Uruguay; 3 Pereira Rosell Hospital Pérez
Scremini Foundation, Montevideo, Montevideo, Montevideo, Uruguay;
4 Resonance, Hematology Oncology Department, Memphis, United States of

EP670 / #1084 DEVELOPMENT OF A STRATIFICATION TOOL America; 5 Pereira Rossell Hospital Perez Scremini Foundation, Hematology
FOR PEDIATRIC ONCOLOGY UNIT TO IDENTIFY LOCAL Oncology Department, Montevideo, Uruguay; 6 Natali Dafne Flexer Founda-
CAPACITY TO ACHIEVE ADEQUATE, TIMELY AND QUALITY tion, Hematology Oncology Department, Buenos Aires, Argentina; 7 Hospital
CARE. NATIONAL CANCER INSTITUTE, ARGENTINA Pereira Rossell, Pediatric Oncology, Montevideo, Uruguay

Florencia Moreno, Agustina Chaplin, Daniel Andisco, Gabriel Concetti, Background and Aims: Introduction: There are many challenges for
Monica Confalone the data management in pediatric oncology in countries with limited
National Cancer Institude-Argentina, Oncopediatric National Program, resources. In Uruguay, there is a single national referral center for pedi-
Buenos Aires, Argentina atric cancer and a national cancer registry but no dedicated electronic
pediatric oncology resource capable of detecting and analyzing spe-
Background and Aims: The National Cancer Institute of the Ministry cific data or performing subgroup analysis or outcome measures such
of Health promotes the process of stratification of Pediatric Oncol- as BMT. So, we aimed to create a single electronic data base to generate
ogy Units (POU). This process aims to identify the POU’s capabilities comprehensive data to address this limitation.
to manage different conditions. This will allow to formalize share care Methods: An agreement was signed with Resonance Inc to create a
processes according to levels of increasing complexity. dedicated site at Resonance Patient Center (RPC). The deployment
Methods: The Stratification tool (ST) was prepared by consensus process was divided into 3 phases: 1) Design of the database and train-
including scientific societies, and 74 specialists, working on scientific ing of personnel. 2) Import of historical registry data from the national
rounds and 15 focus meetings. registry data base, 3) Real-time registration of cases.
A pilot was carried out in 8 POU to assess the validity, reliability and Results: First phase: 34 data capture forms were created for each of
sensitivity of the questionnaire. Finally, the ST is self-administrated and the most common diagnosis and clinical situations. All medical, admin-
had 224 questions online. istrative and psychosocial staff (n=34) was trained. Second phase:
Results: The ST evaluates four dimensions (Human Resources, Infras- 6008 cases were imported from the national registry database on
tructure, Equipment, and Comprehensive Care) according to the type October 2021 including demographic, diagnostic and mortality data.
of tumor: Leukemia and Lymphomas, CNS Tumors, Bone Tumors, Survival data was updated from institutional databases and consulta-
Retinoblastoma, Liver Tumors and Other Solid Tumors. The level of tions in the long-term follow-up clinic for 1973 patients, including all
complexity is stratified by tumor type. There are 4 categories: Basic cases undergoing stem cell rescue (n=367). One hundred fifty-five new
complexity and Follow-up Units, Standard, Complex and Maximum cases were added on real time in the third phase. The 3-year overall
Complexity Patient Treatment Centers. The answers were categorized survival of 1320 patients (7 incomplete data) with cancer diagnosed
as “Desirable”, “Mandtory” or “Priority”, each one with its respective from 2010-2019 (n=1320) was 0.82 (SE= 0.02). A future 4th phase is
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S458 of S635 ABSTRACTS

envisioned to include enhancement of collected data including surgery, Sheena Mukkada1 , Esther Majaliwa2 , David Moore3 , Gita Naidu4 ,
radiotherapy and pathology as well as nursing and pharmacy reports. Asya Agulnik5 , Ayomide Omotola6 , Taisiya Yakimkova6 , Tea Reljic7 ,
Conclusions: Safe and comprehensive data management in a coun- Ambuj Kumar7
try with limited resources is feasible with RPC. However, barriers 1 St Jude Children’s Research Hospital, Department Of Global Pediatric
include time-intensive human resources and the need of effective Medicine, Memphis, United States of America; 2 Kilimanjaro Christian Med-
inter-operability of available data systems to enhance the quantity and ical Center, Pediatric/oncology, Moshi Kilimanjaro, Tanzania; 3 Chris Hani
quality of data recorded. Baragwanath Academic Hospital, Paediatric Infectious Diseases, Soweto,
South Africa; 4 Chris Hani Baragwanath Academic Hospital, University of
the Witwatersrand, Department Of Paediatrics And Child Health, Johannes-
EP672 / #1366 UNIDOS PELA CURA GUIDE: PATHS FOR burg, South Africa; 5 St. Jude Children’s Research Hospital, Department Of
IMPLEMENTATION OF A POLICY OF EARLY CHILDHOOD Global Pediatric Medicine, Memphis, TN, United States of America; 6 St. Jude
CANCER DIAGNOSIS Children’s Research Hospital, Global Pediatric Medicine, Memphis, United
States of America; 7 University of South Florida, Research Methodology And
Thaís Vidal, Carolina Motta Biostatistics Core, Tampa, United States of America
Desiderata Institute, Pediatric Oncology, Rio de Janeiro, Brazil
Background and Aims: Lack of process standardization contributes
Background and Aims: In Brazil, cancer is the leading cause of death to poor infectious outcomes in children treated for cancer in
by disease amongst children and adolescents and late diagnosis is still low- and middle-income countries. We aimed to test a standard-
a concern. In this context, Unidos pela Cura (UPC) a policy to promote ized cooperative education and adaptation framework to create
early diagnosis, was developed in Rio de Janeiro. Based on the success evidence-based guidelines for fever management in diverse regional
of the experience, the Desiderata Institute prepared a guide for repli- groups.
cating this strategy in other territories, with the objective of promoting Methods: A multidisciplinary guideline development group was
the same chances of cure for all children. convened in parallel to develop priority questions for a febrile
Methods: The development of the Guide involved two stages: an neutropenia guideline in three regions: Eurasia, South Africa,
exploratory descriptive study of the main UPC’s results achieved and non-South Africa Sub-Saharan Africa. Regional stakeholders
between 2007 and 2021 and the systematization of the paths for the were surveyed to identify priority E-Poster Topics for guideline
implementation of an Early Diagnosis Policy in other cities. inclusion.
Results: The UPC enabled 91% of suspected cases to reach diagnostic Results: Guideline development groups created and disseminated
centers within three days and trained 4378 Primary Care Profession- prioritization surveys that addressed up to 37 E-Poster Topics but
als to identify the symptoms of childhood cancer. The systematization encompassed 26 questions in common. Question categories included:
of this experience identified eight important steps to an early diagnosis definitions of fever and neutropenia, initial management, modifications
policy: (1) Identification and awareness of key actors; (2) Creation of a to therapy, management of prolonged fever and discharge parameters.
Strategic Committee; (3) Knowledge enhancement about the local sce- E-Poster Topics identified only by specific regional groups included:
nario (epidemiological profile and existing services); (4) Preparation of tuberculosis diagnostics, Pneumocytis jirovecii pneumonia; endemic
a term of commitment signed by all interested parties; (5) Organization parasitic diseases. 151 and 52 responses were obtained from Eura-
of an referral flow; (6)Definition of education strategies; (7) Monitoring sia and South Africa, respectively. Sub-Saharan African responses are
of the referred cases and evaluation of the strategy and (8)Consolida- pending. More than 80% of respondents ranked 23/26 (88%) of ques-
tion of the Policy to guarantee its continuity even with changes in public tions as “important/very important” in both regions. Agreement to
management. prioritize a particular question between the two regions was low
Conclusions: Access to early diagnosis is essential to increase the [kappa (95% CI)= -0.054 (-0.130, 0.022)][BN1] .[BN2] [TR3] [BN1]This
chances of cure for children and adolescents with cancer. However, is awkward so I rewrote. Check its true. [BN2]This is weird. Basically
there are still few instruments that help managers to organize the you are saying that of the 3 questions excluded, they did not agree?
referral flow of suspected cancer cases. The elaboration of the UPC [TR3]For the 3 questions, one group included and the other excluded.
Guide aimed to fill this gap by systematizing ways to reduce the time No questions were excluded by both groups. Kappa is a very poor
needed for the diagnosis of childhood cancer. It can also be used as a statistic when inclusion rate is over 80%. Here it is 96% for one group
reference for national public policies. and 92% for the other group.
Conclusions: Best practice for guideline adaptation remains an open
research question. Stakeholders identify many of the same priorities in
EP673 / #1928 GUIDELINE DEVELOPMENT FOR fever management but differences underscore the importance of inter-
MANAGEMENT OF FEVER IN CHILDHOOD CANCER PATIENTS: rogating local priorities. Next steps include training regional groups in
A COMPARISON OF REGIONAL PRIORITIES evidence-based guideline development, proficiency testing, and voting
on recommendations.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S459 of S635

EP674 / #1613 TRANSFORMING TRAINING STRATEGY FOR Goodwin8 , Beverley Neethling6 , Fathima Naby9 , Fareed Omar10 ,
GLOBAL SCALE OF A QUALITY IMPROVEMENT (QI) Helder De Quintal11 , Helena Rabie5 , Joelle Mukendi12 , Kamaline
INTERVENTION Coopsamy9 , Karla Thomas13 , Liezl Du Plessis14 , Mampoi Jonas15 ,
Nickhill Bhakta16 , Sarah-Anne Falcon17 , Tea Reljic3 , Vutshilo
Hilmarie Muniz-Talavera1 , Alejandra Gonzalez-Ruiz1 , Angela Carrillo1 , Netshituni18 , Yasmin Goga6 , Gita Naidu19
Adolfo Cardenas1 , Tania Conde2 , Lane Faughnan1 , Marcela Garza1 , 1 Chris Hani Baragwanath Academic Hospital, Paediatric Infectious Dis-
Alejandra Méndez-Aceituno1 , Maria Puerto-Torres1 , Dora Soberanis1 , eases, Soweto, South Africa; 2 St Jude Children’s Research Hospital, Depart-
Asya Agulnik1 ment Of Global Pediatric Medicine, Memphis, United States of America;
1 St Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis, 3 University of South Florida, Research Methodology And Biostatistics Core,

United States of America; 2 Casa de la Amistad, Casa De La Amistad Para Tampa, United States of America; 4 Stellenbosch University, Division Of
Niños Con Cáncer, I.a.p, Cuidad de México, Mexico Medical Microbiology, Cape Town, South Africa; 5 Tygerberg Hospital, Pae-
diatrics And Child Health, Cape Town, South Africa; 6 Inkosi Albert Luthuli
Background and Aims: Quality improvement (QI) in healthcare has Hospital, Paediatrics And Child Health, Durban, South Africa; 7 Chris Hani
gained considerable momentum in recent years, yet there are signifi- Baragwanath Academic Hospital, Department Of Pharmacology, Johannes-
cant barriers to adopting effective teaching approaches to successfully burg, South Africa; 8 Charlotte Makexe Johannesburg Academic Hospital,
implement QI initiatives in clinical settings. Our work describes educa- Paediatrics And Child Health, Johannesburg, South Africa; 9 Grey’s Hospi-
tional strategies and key interventions used in the implementation of tal, Department Of Paediatrics And Child Health, Pietermaritzburg, South
the QI project, Proyecto EVAT. Africa; 10 Steve Biko Academic Hospital, Paediatrics And Child Health,
Methods: In 2017, Proyecto EVAT was stated by St Jude Children’s Pretoria, South Africa; 11 Red Cross Ward Memorial Children’s Hospital,
Research Hospital in collaboration with 17 pediatric oncology cen- Paediatrics And Child Health, Cape Town, South Africa; 12 Chris Hani
ters in Latin America. Currently, the project has successfully trained Baragwanath Academic Hospital, Department Of Paediatric Hemotology
5 cohorts, encompassing 73 centers in 19 countries. A comparative And Oncology, Johannesburg, South Africa; 13 Frere Hospital, Department
analysis was conducted to evaluate the evolution of Proyecto EVAT Of Paediatrics And Child Health, East London, South Africa; 14 Robert

training material content, delivery, and teaching strategies used for the Simangaliso Hospital, Department Of Paediatrics And Child Health, Kim-
successful scale-up of Proyecto EVAT over the last 5 years. berley, South Africa; 15 Universitas Hospital, Paediatrics And Child Health,
Results: The original project curriculum (designed in 2017) was estab- Bloemfontein, South Africa; 16 St Jude Children’s Research Hospital, Global
lished to deliver core project concepts using a teaching-feedback- Pediatric Medicine, Memphis, United States of America; 17 Childhood Can-
learning process, where materials were adapted on a weekly basis cer Foundation South Africa (CHOC), Choc, Johannesburg, South Africa;
which resulted in lack of structure and slow/inadequate learning. 18 Pietersburg Hospital, Department Of Paediatrics And Child Health,
Lessons learned from this first cohort led to adopting a proactive teach- Polokwane, South Africa; 19 Chris Hani Baragwanath Academic Hospital,
ing approach, with structured lesson plans, setting clear expectations, Department Of Paediatrics And Child Health, Johannesburg, South Africa
and promoting active learning to incite curiosity and higher order
thinking. Our modules include E-Poster Topics in creating baseline Background and Aims: Febrile neutropenia (FN) is a common cause
data, defining problems, identifying, and engaging with stakeholders of interruptions to childhood cancer treatment. Lack of guidance and
and implementation of QI initiatives. Iterative review of learning mate- inconsistent management of FN may contribute to poor patient out-
rials resulted in the use of a teach-practice-apply model to develop comes. We hypothesize that creating an evidence-based guideline for
training materials and employ interactive virtual practices for content FN management across South African institutions is feasible and may
delivery. optimize patient outcomes across centres.
Conclusions: Creative strategies using innovative tools for teaching Methods: A multi-centre, cross-discipline guideline development panel
and practice, while promoting collaborations between physicians and was convened. A guideline prioritisation survey was designed to iden-
nurses can lead to fostering a cultural change in the clinical setting. To tify E-Poster Topics that should be considered for development of a
date, training for Proyecto EVAT has been conducted in 73 hospitals guideline tailored to the South African context. The survey, which con-
across Latin America. We believe we have created a highly adopt- sisted of 51 questions inclusive of 37 practice points, was distributed
able model that can be tailored to any QI intervention for successful to colleagues working in child health disciplines across South Africa in
implementation of the initiative. December 2021 through February 2022.
Results: Fifty-two (74.3%) complete responses were obtained from
the 70 clinicians surveyed. Twenty-eight (53.9%) of the respondents
EP675 / #1439 DEVELOPMENT OF A FEBRILE were paediatric haematologist-oncologists and 12 (23.1%) were gen-
NEUTROPENIA GUIDELINE FOR SOUTH AFRICAN CHILDREN eral paediatricians, mostly (48/52, 92.3%) based at tertiary/academic
WITH CANCER centres. Eight (88.9%) of the nine South African provinces were repre-
sented. E-Poster Topics deemed “very important” (according to >75%
David Moore1 , Sheena Mukkada2 , Ambuj Kumar3 , Andrew of respondents) included: 1) to define fever; 2) to define neutropenia; 3)
Whitelaw4 , Anel Van Zyl5 , Ashendri Pillay6 , Azraa Paruk7 , Bernard to describe how to modify antimicrobial therapy in unstable patients;
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S460 of S635 ABSTRACTS

4) to define patients at high risk for invasive fungal disease; and 5) to EP677 / #1925 PAEDIATRIC CANCER TRENDS IN A
define when to remove indwelling lines in paediatric patients with FN. TANZANIAN TERTIARY HOSPITAL IN DURING THE COVID-19
A further 19 questions were considered either “important” or “very PANDEMIC
important” for inclusion in the guidelines, according to >90% (47/52)
of respondents. Nana Nakiddu1 , Lulu Chirande2 , Trish Scanlan3
Conclusions: Twenty-four E-Poster Topics were considered important 1 Muhimbili University of Health and Allied Sciences, Paediatrics, Dar es
for inclusion in a South African FN guideline. Future steps will be to Salaam, Tanzania; 2 Muhimbili University of Health and Allied Science, Pae-
apply the Grading of Recommendations, Assessment, Development diatrics And Child Health, Dar es salaam, Tanzania; 3 Muhimbili National
and Evaluation (GRADE) approach to develop recommendations for Hospital, Pediatric Oncology, Dar Es Salaam, Tanzania
the prioritized questions. This work will facilitate research to evaluate
the use and impact of the completed guidelines on outcomes of FN in Background and Aims: Drastic measures to curb the spread of
South African children with cancer. COVID-19 in Tanzania led to disruptions in essential services that
compounded the already limited access to peripheral and national pae-
diatric oncology clinics. The study aimed to describe the incidence of
EP676 / #1743 COVID-19: COLLATERAL DAMAGE IN childhood cancers at Muhimbili National Hospital during the pandemic,
CHILDHOOD CANCER IN A SOUTH AFRICAN CENTRE with the overall aim to estimate potential gaps in the continuum of
paediatric cancer care.
Gita Naidu, Diane Mackinnon, Biance Rowe, Thandeka Ngcana, Methods: In this descriptive study, we analyzed hospital medical
Nandipha Sigedle, Douglas Momberg records to identify children aged 10-18 years newly diagnosed with
Chris Hani Baragwanath Academic Hospital, Department Of Paediatrics paediatric cancers between January 2020 and December 2021. Diag-
And Child Health, Johannesburg, South Africa nosis was established by histopathology, flow cytometry and immuno-
histochemistry where applicable. For all children with a confirmed
Background and Aims: Introduction diagnosis, we retrieved demographic and treatment data, as well as the
Studies from high-income countries predominantly reporting on adult outcome of treatment (dead/alive) at the time of their last available
patients, demonstrated detrimental effects of the COVID-19 pandemic medical record during the study period.
on cancer care, including marked reductions in new cancer diagnoses. Results: Of the 745 diagnosed with paediatric cancer, the mean age
There is comparatively little evidence about the effect of the pan- at diagnosis was 6.69±6.34 years. Extracranial solid tumours were
demic on paediatric cancer care, especially in low-and middle-income the commonest 404/745 (54%) with Nephroblastoma 135/745 (18%)
countries. and Retinoblastoma 109/745 (14.6%) diagnosed most frequently. By
Methods: Retrospective clinical data from medical records was December 2021, 213/745 (29%) had died.
extracted for paediatric cancer patients (age ≤ 19) admitted to the Conclusions: The incidence of paediatric cancers during the COVID
paediatric oncology department, Chris Hani Baragwanath Academic pandemic were lower than in pre-pandemic years. A comprehensive
Hospital, for two time periods: the first from March 2018 to February assessment of systemic and socioeconomic bottlenecks is warranted to
2020, and the second from March 2020 to February 2022. improve overall access to paediatric cancer care in Tanzania.
Results: Data for 518 newly diagnosed patients was extracted over the
four years (248 pre-Covid [group 1] and 270 [group 2] during Covid).
The male: female ratio was 1.67 and 1.23, while 6% and 4% were liv- EP678 / #1837 THE IMPLEMENTATION OF A WORKSHOP
ing with HIV. The mean ages of diagnoses were 7.5 and 6.7 years for FOR ADAPTED MANAGEMENT GUIDELINES AND QUALITY
groups 1 and 2 respectively. Haematological malignancies accounted IMPROVEMENT SCIENCES IN AFRICA
for 34.67% and 37.78% for groups 1 and 2, respectively. Hodgkin lym-
phoma accounted for a total of 8.1% of diagnoses in group 1 and 4.4% in Jaques Van Heerden1 , Nickhill Bhakta2 , Marc Hendricks3 , Vivian
group 2, a decrease by 50%. Similarly, the number of children with brain Paintsil4 , Moatasem Eleyadi5 , Alan Davidson3 , Leila Hessissen6 , Joyce
tumours decreased by 48.2% between group 1 and group 2. In group Kambugu7 , Jennifer Geel8
1, 36.6% of solid tumours presented with localized disease and 28.3% 1 Antwerp University Hospital, Paediatric Haematology and Oncology,
with advanced disease, while in group 2, 28.2% and 33.3% presented Department of Paediatrics and Child, Antwerp University Hospital Health,
with localized and advanced disease respectively (p=0.04). Antwerp, Belgium; 2 St Jude Children’s Research Hospital, Global Pediatric
Conclusions: Significant decreases in the number of children present- Medicine, Memphis, United States of America; 3 University of Cape Town,
ing with Hodgkin lymphoma and brain tumours, and children with solid Paediatric Haematology and Oncology Service, Red Cross War Memorial
tumours presenting with advanced disease during the pandemic is wor- Children’s Hospital, Department Of Paediatrics And Child Health, Faculty
rying. The WHO Global Initiative for Childhood Cancer to improve Of Health Sciences, Cape Town, South Africa; 4 Kwame Nkrumah Univer-
outcomes of children with cancer living in low- and middle-income sity of Science and Technology, Department Of Child Health, School Of
countries by 2030 implies that strengthening of campaigns for early Medicine And Dentistry, Kumasi, Ghana; 5 National Cancer Institute, Cairo
diagnosis and referral in children with cancer is therefore needed. University, Egypt & Children Cancer Hospital of Egypt (CCHE-57357).,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S461 of S635

Department Of Pediatric Oncology, Cairo, Egypt; 6 University Mohamed United States of America; 3 Baylor College of Medicine, Pediatric Hematol-
V Rabat, Pediatric Haematology And Oncology Center, Rhabat, Morocco; ogy/oncology, Houston, United States of America
7 Uganda Cancer Institute, Department Of Paediatric Oncology, Kam-
pala, Uganda; 8 University of the Witwatersrand, Division Of Paediatric Background and Aims: In high income countries, pharmacy profes-
Haematology-oncology, Faculty Of Health Sciences, School Of Clinical sionals compound hazardous agents such as chemotherapy using asep-
Medicine, Johannesburg, South Africa tic technique in controlled environments with engineering controls,
such as certified biosafety cabinets in pharmacy cleanrooms. In Low
Background and Aims: The capacity to develop resource-based man- and Middle Income countries (LMICs), chemotherapy is often reconsti-
agement guidelines amongst African paediatric oncology teams is tuted in an open environment due to costs associated with constructing
limited. SIOP Africa and St. Jude Global members developed a work- and maintaining compounding rooms and equipment. Closed system
shop to build interest in quality improvement and implementation transfer devices (CSTDs) may be an alternate approach to minimizing
science, related to adapted management guidelines (AMG) based on occupational exposure, in the absence of these engineering controls.
the WHO 2030 Global Initiative for Childhood Cancer (GICC). We The purpose of this project was to improve the safety of chemother-
describe implementation and participant feedback of the workshop. apy compounding and to demonstrate the utility of CSTDs to reduce
Methods: Four theory-based lectures on Adapted Resource and Imple- surface contamination and thus minimize occupational exposure.
mentation Application (ARIA) guides, implementation science, quality Methods: This was an education-focused, quality improvement project
improvement and guideline methodologies were pre-recorded by con- of aseptic technique and safe handling of hazardous medications. The
tent experts as requested viewing for participants prior to the work- educational intervention was conducted in a blended classroom with
shop. The one-day in-person workshop consisted of three sessions online coursework, case-based virtual discussions, recorded simula-
drawing on the participants’ experience. Sessions included: (1) facili- tions, manufacturer’s training materials, and hands-on training and
tated small group discussions on guideline development and research; validation. Hands-on validation was completed using traditional com-
(2) a hybrid, expert panel discussion on real-world experience of mul- pounding supplies, CSTDs, soy-broth test kits, and fluorescein dye
tidisciplinary teams; (3) facilitated small group discussions to identify test.
barriers and enablers to implementing guidelines for the six GICC index Results: All staff completed the training course and passed the final
cancers. Feedback was captured through an online survey in English exam. Initial hands-on validation revealed visual confirmation of the
and French. efficacy of CSTDs to reduce hazardous drug exposure. The traditional
Results: There were 62 participants (75% Anglophone, 25% Franco- syringe and needle method revealed visual spillage (via fluorescein dye)
phone) and 14 facilitators, from 22 African countries, 4 European on the operator’s gloves, as well we on the exterior of the vial, syringe,
countries and United States. Stakeholders were recruited from radio- needle, and preparation surface. When using the same approach with
therapy, oncology, surgery and nursing. Final outputs included two CSTDs, no visual spillage was detected. A recent re-validation of
worksheets summarising implementation factors for each participant’s practice revealed that out of 15 simulations, the traditional method
setting and the six GICC index cancers. The post-workshop survey was produced 14/15 with surface contamination vs a 0/15 when using the
completed by 38/62 (61.3%) of participants: all felt that the work- CSTD.
shop will influence their practice and 30/38 (79%) reported that >75% Conclusions: CSTDs may be a suitable approach to minimizing surface
material was new to them. At least 71% of Francophone participants contamination and occupational exposure in the absence of highly-
reported minimal trouble in comprehension of the content, which controlled pharmacy compounding. We recommend consideration of
increased to 86% with the aid of translation. CSTDs in this setting and encourage further research in the cost-utility
Conclusions: This workshop demonstrates potential for continental of CSTDs in LMICs.
partnerships in developing AMGs to improve management practices
and outcomes. Future activities will be developed to strengthen
resource-based management of childhood cancers in Africa, and con- EP680 / #362 A REGIONAL VIRTUAL CASE DISCUSSION
sideration must be given to measuring impact of these processes. FORUM IN PEDIATRIC ONCOLOGY: EXPERIENCE OF THE
PEDIATRIC ONCOLOGY EAST AND MEDITERRANEAN GROUP

EP679 / #1051 IMPROVEMENT OF CHEMOTHERAPY Dolly Noun1 , Anas Obeid1 , Asim Belgaumi2 , Iman Sidhom3 , Nisreen
COMPOUNDING VIA EDUCATION AND IMPLEMENTATION OF Khalifa4 , Amita Trehan5 , Khaled Ghanem6 , Hafeez Abdelhafeez7 ,
A CLOSED SYSTEM TRANSFER DEVICE IN UGANDA Monika Metzger7 , Carlos Rodriguez-Galindo7 , Sima Jeha7 , Raya Saab1
1 American University of Beirut Medical Center, Children’s Cancer Center
Mayi Nanyonga1 , Mercy Nalukwago1 , Jeremiah Kateregga1 , Melanie Of Lebanon, Beirut, Lebanon; 2 Aga Khan University, Pediatric Hematol-
Bernhardt2,3 ogy Oncology Unit, Karachi, Pakistan; 3 Children Cancer Hospital Egypt,
1 Texas Children’s Global HOPE (Hematology/Oncology Pediatric Excel- 57357, Pediatric Hematology Oncology Unit, Cairo, Egypt; 4 National Bank
lence), Global Hope Uganda, Kampala, Uganda; 2 Texas Children’s Hos- of Kuwait Specialized Hospital for Children, Pediatric Hematology Oncology
pital, Global Hope (hematology/oncology Pediatric Excellence), Houston, Unit, Sabah, Kuwait; 5 Advanced Pediatrics Center, Postgraduate Institute
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S462 of S635 ABSTRACTS

of Medical Education and Research, Pediatric Hematology Oncology Unit, ogy, ELDORET, Kenya; 5 Ampath, Blp, Eldoret, Kenya; 6 Indiana University,
Chandigarh, India; 6 al-Biruni University Hospital, Basma Pediatric Hematol- Hemato-oncology, ELDORET, Kenya; 7 Baylor College of Medicine, Pedi-
ogy Oncology Unit, Damascus, Syria; 7 St Jude Children’s Research Hospital, atrics, Houston, United States of America; 8 Takeda Pharmaceuticals, Phar-
Global Pediatric Medicine, Memphis, United States of America maceuticals, Asia, Japan; 9 Mount Kenya University, Pharmacy, Nairobi,
Kenya; 10 Mount Kenya University, Pharmaceutical Chemistry, NAIROBI,
Background and Aims: The Pediatric Oncology East & Mediterranean Kenya
(POEM) group aims to share expertise among pediatric oncology
providers across the Middle East, North Africa, and East Asia region. Background and Aims: Good policies and procedures are a prereq-
In 2013, POEM initiated a fortnightly virtual Case Discussion forum, uisite to efficiency in hospitals. Oncology centers are at higher risk of
for peer input on clinical management. Here, we evaluate its scope and harmful incidents due to chemotherapy exposure, toxicity and radia-
impact. tion. This article reports on the measures taken to ensure safety and
Methods: Meeting records from September 2013 till June 2021 were efficiency at work.
reviewed. Detailed minutes were available starting August 2016; Methods: AMPATH Oncology Pharmacy in Moi Teaching and Referral
accordingly, case data between August 2016 and June 2021 were Hospital collaborated with Takeda Pharmaceuticals to develop stan-
analyzed including diagnoses, purpose of presentation and recommen- dard operating procedures to promote safety. Policy implementation
dations. in paediatric oncology clinic was through ECHO Pharmacy online
Results: A total of 140 cases from 14 countries were presented, participation model during the pandemic. Peadiatric pharmacovigi-
respecting patient anonymity. After August 2016, 65 cases were pre- lance reporting protocol promoted reduced medication errors and
sented, with a mean of 14 attendees per meeting (range 5 - 49) from CINV among inpatients. The focus points included the chemother-
21 countries. Reasons for presentation included questions regarding apy compounding area, waste incineration area, paediatric inpatient
histopathologic/molecular diagnosis in 23%, imaging interpretation in pharmacy, chemotherapy administration, main pharmacy dispensing
18%, chemotherapeutic options in 18%, and surgery in 8%. Therapeutic point, cytotoxic drug store and inpatient pharmacovigilance. Manuals
challenges were related to treatment planning in 14%, resource limi- and protocols were developed on safe handling of cytotoxic products,
tations in 12%, surgical difficulties in 9%, and expertise availability in pharmacovigilance reporting, inter and intra facility drug movement,
6%. Peer recommendations included specific chemotherapy regimens patient education and counselling, and sterile chemotherapy prepara-
in 40%, imaging in 29%, same management in 29%, surgery in 26%, radi- tion in aseptic cyto-cabinets.
ation therapy in 17%, molecular testing in 17%, and pathology review Results: There has been improved documentation of chemotherapy
in 15%. Discussion of stem cell transplant indications occurred in 22%. spills in the log sheet with appropriate combatting measures, reduced
Other recommendations were related to anticoagulation, infection exposure of staff to cytotoxic molecules and comprehensive waste
control, clinical trials, and child protection laws. Surgical manage- segregation and incinerations of 1200 ◦ C for chemotherapy exposed
ment of locally advanced hepatoblastoma accounted for 20% of solid materials. Patient compliance to medication and hospital visits also
tumor cases discussed, which has now spurred a retrospective multi- increased because of better advice and counselling by the pharmacy
institutional regional study to better understand the regional needs for team. There were reduced incidences of chemotherapy compound-
this malignancy. Similarly, histopathologic diagnostic limitations were ing and dispensing errors in the paediatric oncology pharmacy due
common in one country, resulting in a twinning project between two to intensified SOP based patient care. This initiative also had an
centers in the region for improving diagnostic infrastructure. unintended positive effect of generating other standard operating pro-
Conclusions: The POEM case discussion forum allows sharing exper- cedures across the unit that facilitated improved both vertical and
tise and sheds light on resource limitations in pediatric cancer manage- horizontal forms of communication. These SOPs have steered to newer
ment across the region, directing efforts for capacity building. oncology facilities benchmark on operations.
Conclusions: SOPs should be considered an essential component of
service delivery and safety in paediatric oncology centres. Proper
EP681 / #1445 STANDARD OPERATING PROCEDURES implementation will not only improve staff safety but also patient
PROMOTES SAFETY AND EFFICIENCY IN PAEDIATRIC welfare and promote equity, efficiency and standard of care.
ONCOLOGY- WESTERN KENYA ONCOLOGY PHARMACIES
BENEFITS
EP682 / #734 THE IMPACT OF A PROJECT ECHO
Moses Elvis Oburah1 , Terry Vik2 , Gilbert Olbara3 , Festus Njuguna4 , TELEHEALTH EDUCATION PROGRAM ON THE REGIONAL
Peace Mbengei5 , John Oguda6 , Tiffany Chambers7 , Cole Abimbola8 , DIAGNOSTIC RATES OF PEDIATRIC CANCER IN KENYA
Bindu Madhavi9 , Ramani Ramalingam10
1 Moi Teaching And Referral Hospital-AMPATH, Hematology And Oncol- Gilbert Olbara1 , Festus Njuguna1 , George Bogonko1 , Martha
ogy, ELDORET, Kenya; 2 Indiana University School of Medicine, Pediatrics, Kipngetich1 , Sandra Langat1 , Frederick Odongo1 , Mary Ann Etling2 ,
Indianapolis, United States of America; 3 Moi Teaching and Referral Hospi- Patrick Monahan3 , Heather Burney3 , Marjorie Treff4 , Terry Vik2 , Tyler
tal, Pediatric Oncology, Eldoret, Kenya; 4 Moi University, Pediatric Oncol- Severance2
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S463 of S635

1 Moi University, Pediatric Oncology, Eldoret, Kenya; 2 Riley Hospital for Background and Aims: Childhood cancer is still a significant public
Children, Pediatrics, Indianapolis, United States of America; 3 IU Simon health issue. Nowadays, there is a large data gap on Puerto Rico (PR)
Comprehensive Cancer Center, Biostatistics And Health Data Science, childhood cancer epidemiology. This small island in the Caribbean has
Indianapolis, United States of America; 4 Indiana University, School Of a genetically unique population that continues to be poorly understood
Education, Bloomington, United States of America in the scientific community. Genetic admixture had a big impact in this
population and there is not updated data regarding pediatric cancer
Background and Aims: Despite recent advances, childhood cancer incidence. In the United States (US), approximately 13,500 pediatric
remains under-diagnosed in low- middle-income countries (LMICs). patients per year are diagnosed with cancer. In PR, the most recent
In Kenya, less than 25% of pediatric patients with cancer receive statistics from 2010-14 showed, a total of 783 children correspond-
a diagnosis. Project ECHO, a telehealth education platform, is rec- ing to an average of 157 cases/year. Cancer remains the second most
ognized for its capacity to address disparities in medically under- common cause of death among children in the US and the sixth leading
served communities. At Moi Teaching and Referral Hospital (MTRH), cause in PR. The primary goal of this study is to create an epidemio-
the only tertiary care center in Western Kenya, an ECHO program logical profile of the island pediatric oncology patients. This database
was piloted to provide instruction and mentorship to providers at will identify cancer incidence in the island and compare it with Hispanic
county hospitals to recognize and evaluate pediatric patients with new children in the US.
malignancies. Methods: The two main pediatric cancer hospitals in Puerto Rico par-
Methods: In January 2020, a pediatric cancer ECHO was implemented. ticipated in this descriptive cross-sectional study. Data was collected
Sessions occurred twice monthly and continued through the COVID- from the electronic medical record in PR and from SEER for Hispan-
19 pandemic. Sessions lasted 75 minutes and included case-based ics in the US. Classification was done based on the ICCC-3 system.
discussion supplemented with a rotating didactic curriculum. Counties Inclusion criteria was restricted to patients within 0-21 years-old with
were found to have high attendance if averaging at least one partici- pathology results confirming the presence of malignancy between
pant per session, moderate attendance if 0.5-1 participant per session, 2016-2018. Data was adjusted per 100,000 habitants and evaluated
and low attendance if <0.5 per session. In addition to the ECHO pro- using ANOVA analysis.
gram, the total new cancer diagnosis from each country was tracked Results: Cancer incidence between 2016-18 was 30.5%/30.4% for
starting in 2018 and continuing through the entire duration of the leukemias, 17.6%/11.8% for CNS malignancies and 16.2%/14.4% for
ECHO. lymphomas in PR/US respectively. This study showed variability among
Results: A total of 37 sessions over 2 years occurred with participants two Hispanic populations that were statistically significant between
from Western Kenya. There were 4 counties with high participation, 6 sex, cancer diagnosis and age group.
with moderate participation, and 19 counties with low participation. Conclusions: There are differences in pediatric cancer incidence
Among counties with high attendance, an average of 10.5 additional among Hispanic populations. This information provides an opportunity
cases per county were diagnosed during the program, compared to 3 to explore other factors such as genetic variability in the role of cancer
additional cases in the moderate group and 1.3 additional cases in the development among Hispanic populations.
low attendance group (p=0.014).
Conclusions: The implementation of Project ECHO led to a significant
increase in pediatric cancer diagnoses in Western Kenya despite the EP684 / #1118 CATEGORIZING DIVERGENT CLINICAL
global pandemic. The greater increase in counties with high participa- PHENOTYPES AMONG CHILDREN AND ADOLESCENTS WITH
tion suggest consistent involvement in this educational program is one KAPOSI SARCOMA IN A PEDIATRIC-SPECIFIC CLASSIFICATION
mechanism to improve care in medically underserved communities,
further validating Project ECHO as a mechanism to reduce disparities Erin Peckham-Gregory1,2 , Liane Campbell1,3,4 , Allison Silverstein5 ,
in LMICs. William Kamiyango6 , Jimmy Villiera6 , Casey Mcatee6,7,8 , Jason
Bacha1,4 , Carrie Kovarik9 , Parth Mehta1,10 , Toni Chanroo1 , Asulwisye
Kapesa4 , Beatrice Malingoti4 , Rizine Mzikamanda6,11 , Nmazuo
EP683 / #1019 PROFILE OF PEDIATRIC ONCOLOGY Ozuah8,11 , Peter Kazembe6 , Carl Allen7,10,12 , Michael Scheurer1,13,14 ,
PATIENTS FROM PUERTO RICO AND HISPANICS FROM UNITED Nader El-Mallawany7,12
STATES 1 Baylor College of Medicine, Pediatrics, Houston, United States of Amer-
ica; 2 Center for Epidemiology and Population Health, Baylor College of
Samuel Pabon-Rivera1 , Anabel Puig Ramos2 , Gilberto Puig Ramos2 , Medicine, Texas Children’s Hospital, Pediatrics, Houston, United States of
Maria Echevarria Escudero2 , Leslie Soto2 , Gloria Colon2 , Alejandra America; 3 Baylor College of Medicine International Pediatric AIDS Ini-
Diaz Rohena2 tiative at Texas Children’s Hospital Houston, Pediatrics, Houston, United
1 UT Health San Antonio, Pediatrics, Division Of Pediatric Hematology- States of America; 4 Baylor College of Medicine Children’s Foundation -
oncology, San Antonio, United States of America; 2 UPR School of Medicine, Tanzania, Mbeya, Tanzania, Pediatrics, Mbeya, Tanzania; 5 Department of
Pediatrics, San Juan, Puerto Rico HospUniversity of Tennessee Health Science Center, Hospice And Palliative
Medicine, Memphis, United States of America; 6 Baylor College of Medicine
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S464 of S635 ABSTRACTS

Children’s Foundation Malawi, Lilongwe, Malawi, Pediatrics, Lilongwe, Chiara Pilotto1 , Ilaria Liguoro1,2,3 , Eva Passone1 , Raffaello Tosolini1 ,
Malawi; 7 Baylor College of Medicine, Pediatric Hematology And Oncol- Maurizio Mascarin4 , Elisa Coassin4 , Valentina Kiren5 , Marco
ogy, Houston, United States of America; 8 Texas Children’s Global HOPE Rabusin5 , Paola Cogo3
Lilongwe, Baylor College Of Medicine, Lilongwe, Malawi; 9 University 1 University Hospital of Udine ASUFC, Pediatric Clinic, Udine, Italy;
of Philadelphia, Dermatology, Philadelphia, United States of America; 2 University Hospital of Udine, Pediatric Clinic, Udine, Italy; 3 University of
10 Texas Children’s Hospital, Global Hematology/oncology Pediatric Excel- Udine, Department Of Medical Area - Dame, Udine, Italy; 4 Centro di Rifer-
lence, Houston, United States of America; 11 Baylor College of Medicine imento Oncologico di Aviano, Aya Oncology And Pediatric Radiotherapy
Children’s Foundation, Malawi, Texas Children’s Global Hope, Lilongwe, Unit, Aviano, Italy; 5 IRCCS Materno Infantile Burlo Garofolo, Department
Malawi; 12 Texas Children’s Global HOPE, Paediatrics Baylor College Of Of Pediatric, Hemato-oncology Unit, Trieste, Italy
Medicine, Houston, United States of America; 13 Baylor College of Medicine,
Hematology-oncology, Houston, United States of America; 14 Texas Chil- Background and Aims: Brain tumors are the second most com-
dren’s Hospital, Baylor College of Medicine, Pediatrics, Houston, United mon cancer in childhood. The purpose of this study was to esti-
States of America mate the incidence of primary central nervous system (CNS) tumors
in a regional cohort of children under 16 years from 2012 and
Background and Aims: The Kaposi sarcoma (KS) T0 versus T1 staging 2021.
classification does not address the unique clinical features of pediatric Methods: We conducted a retrospective epidemiological study of
KS in human herpesvirus-8 endemic regions of Africa. This study seeks primitive lesions, all revised by Central Pathological Review, from our
to define patterns of childhood KS using a pediatric-specific approach. regional database of CNS tumors in children < 16 years. Relapses were
Methods: The Lilongwe Pediatric KS Staging Classification catego- excluded. We calculated the incidence per 100.000 children per year
rizes disease based on clinical phenotype: stage 1=mild/moderate and analyzed the incidence trend of low- vs high-grade tumors in 2-5
KS limited to cutaneous/oral involvement, stage 2=primarily lym- years subgroups (2012-2016 vs 2017-2021). Descriptive statistic with
phadenopathic, stage 3=woody edema KS, stage 4=visceral and/or mean +/- standard deviation (DS) and frequencies were used. Indipen-
severe/disseminated mucocutaneous disease. Characteristics and out- dent Student t-test and Chi-square test were used when appropriate
comes were evaluated from tertiary care pediatric centers in Lilongwe, (p= .05).
Malawi, and Mbeya, Tanzania. Event-free survival (EFS) is defined as Results: In the studied decade, 93 CNS tumors have been diagnosed
sustained complete remission (CR), while progression-free survival with a mean age of 9,2 years +/- 4,7 (44 males, 47,3%) with a mean
(PFS) includes patients with sustained CR plus partial response. incidence of 5,41 cases +/- 1,22 (range 3,9-8,5) per 100.000 inhabi-
Results: Among 171 patients, median age was 9.3 years, 37% (n=63) tants under 16 years per year. We also documented a reversal of the
were female, 87% (n=149) were HIV-positive, and 49% (n=84) were trend with a rising number of high grade diagnosis compared to low-
histologically-confirmed. Breakdown by stage was: 18% (n=31) stage grade. Although homogeneus by age (9,3 vs 9,1 years, p=.83) and sex
1, 33% (n=56) stage 2, 19% (n=33) stage 3, and 30% (n=51) stage 4. (48,8% vs 58,4 of males, p=.38), the second 5-years group (2017-2021)
Stage 4 categorization consisted of pulmonary KS (n=24), GI involve- showed a rising rate of high-grade cases (60% vs 21,95%, p=.000493),
ment (n=10), and disseminated mucocutaneous KS without visceral compared to the first group (2012-2016). In particular, in the last two
disease (n=17). Age (younger stage 2 and older stage 3), severe years (2020-2021) there was a 2.65 (95%CI 1.39-9.1) higher probabil-
CD4 count suppression (lower CD4 for stages 1 and 4), and pres- ity to diagnose a high grade brain tumor than in the previous period
ence of severe anemia and thrombocytopenia (worse for stages 2 and (2012-2019).
4) differed across stages. Estimated 2-year EFS/PFS/overall survival Conclusions: In childhood, CNS tumors incidence has been fairly sta-
(OS) by stage were as follows: stage 1–81%/81%/87%, stage 2– ble in the last ten years in our regional cohort, but a recent concern
50%/50%/63%, stage 3–24%/49%/81% and stage 4–29%/34%/54%. of an increased rate of high-grade diseases emerged according to our
Excluding five deaths within the first week of diagnosis, sub-analysis of data. In case of a confirmation of this trend, a further analysis aiming to
stage 2 lymphadenopathic KS demonstrated superior long-term 6-year understand the reason is needed.
EFS of 70% (95% CI: 49-83) for younger children (<7 years old) versus
27% (95% CI: 8-51) for older children.
Conclusions: This pediatric-specific staging classification categorizes EP686 / #1853 EXPERIENCE OF RADIATION TREATMENT
patients with distinct characteristics and patterns of treatment INTERRUPTIONS AND IDENTIFICATION OF MEASURES TO
response. It may serve as a platform for risk-stratified treatment with IMPROVE CARE AT A TERTIARY CARE UNIVERSITY HOSPITAL
the hope of improving survival. IN LMIC

Bilal Mazhar Qureshi, Yumna Ahmed, Rabia Tehseen, Sehrish Abrar,


EP685 / #1280 INCIDENCE OF BRAIN TUMORS IN AN Ahmed Nadeem Abbasi
ITALIAN REGIONAL COHORT FROM 2012 TO 2021: AN Aga Khan University, Radiation Oncology, Department Of Oncology,
EPIDEMIOLOGICAL STUDY Karachi, Pakistan
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S465 of S635

Background and Aims: Radiation therapy is an essential local control was counted once and changed in diagnosis when appropriate was
modality of many pediatric solid and brain tumors and shall be com- not listed as a new patient. A total number of new cases were reg-
pleted withoug unscheduled gaps to achieve optimum outcomes. Here istered, and data during the pandemic (2020-2021) were compared
we look into the reasons and patterns of gaps in radiation treatment for with previous years (2015-2019). Outcomes were also collected and
children at our institute. analysed.
Methods: Children treated from January 2009-March2021 with RT Results: This study included 2142 new cases across seven years of
with or without GA at Aga Khan University were included. Patients observation, with 551 patients (25.7%) from the COVID-19 pandemic
having treatment gap of one or more days during radiotherapy were and 1591 patients (74.2%) from the baseline period. Acute lymphoblas-
identified and data for demographic details, diagnosis, region of treat- tic leukemia is still the most common type of cancer, with 171 (31%)
ment, duration of gap and reason for treatment interruption was and 484 (30.4%) patients. The ratio between leukemia and the solid
recorded and analyzed. tumor was 1.0: 1.14. Outcomes were worse than in previous years, with
Results: There were 178 events of treatment gap in 118 out of 511 a higher number in diagnosis and treatment, mortality rate, readmis-
patients treated during study period with mean age 8.2years[±4.95) sion rate, relapse, and a lower number of remissions. Associated factors
and 71(60%)male. Most of patients(75%) were referred from another include delayed treatment due to isolation, COVID-19 co-infections,
hospital after discussion in tumor board. All RT plans were peer and severe clinical conditions.
reviewed. Most common diagnoses were sarcomas 57%(67 patients), Conclusions: There was no significant decrease in the yearly num-
CNS 15%(16 patients), lymphomas 14%(16 patients) and renal tumors ber of childhood cancer cases during the COVID-19 pandemic. But
10%(12%) patients. There was a gap of 1-2days in 40(34%)patients, delay in diagnosis and treatment led to worse clinical outcomes. There-
2-7days in 42(36%)patients and ≥8days in 36(30%)patients. Major fore, despite the ongoing pandemic, solutions and extra attention from
reasons included in-patient admission due to fever, neutropenia or families, health care workers, and stakeholders must be given to our
diarrhea in 53 patients, machine breakdown in 29 patients, refusal oncology patients to prevent delays in diagnosis and treatment.
of anesthesia fitness due to chest congestion in 24patients, patients’
social reason in 14 and RT re-planning in 10 patients. Out of 118
patients, 45(38%) were being treated under GA and 73(62%) without EP688 / #1795 PEDIATRIC SOLID TUMOR MANAGEMENT IN
GA. 24 of 45 patients treated under GA had treatment interruption A NATIONAL REFERRAL HOSPITAL IN INDONESIA: CHANGES
because of being unfit for GA before RT. Longer interruption was noted BEFORE AND DURING COVID-19 PANDEMIC
in children treated under GA with 3-7 days gap in 36%patients and
≥8days gap in 42%patients. Ludi Dhyani Rahmartani, Stephen Iskandar, Ganda Ilmana, Liza
Conclusions: The demonstrated prospecte factors which could min- Gintana, Teny Sari
imize treatment interruptions including improvement in supportive Universitas Indonesia - Cipto Mangunkusumo Hospital, Child Health,
care, prevention of infections, maintenance of equipment. Local con- Jakarta, Indonesia
trol can be improved by working on these things, specifically in shared
care tertiary care setting. Background and Aims: In lower-middle-income countries, advanced
investigations for solid tumours are often incomplete and inaccessible.
Patients usually come to our centre with advanced stages and make
EP687 / #237 IMPACT ON CHILDHOOD CANCER CARE DUE it difficult to manage. Delays in diagnosis (including elective surgeries)
TO COVID-19 PANDEMIC IN NATIONAL REFERRAL HOSPITAL and treatment interruptions increase complications and mortality. This
IN INDONESIA study aims to determine pediatric solid tumours management changes
due to the COVID-19 pandemic in Cipto Mangunkusumo Hospital,
Ludi Dhyani Rahmartani, Radhian Amandito, Liza Gintana, Teny Sari Jakarta, Indonesia.
Universitas Indonesia - Cipto Mangunkusumo Hospital, Child Health Methods: This was a retrospective study. Data were obtained from the
Department, Jakarta, Indonesia clinical records of pediatric patients with solid tumours treated in our
oncology unit before the pandemic (January 2015 - December 2019)
Background and Aims: Cipto Mangunkusumo Hospital (CMH) is the and during the pandemic (January 2020 - December 2021). Histol-
national referral hospital in Indonesia. Following the COVID-19 pan- ogy reports were obtained from the histopathology unit. Procedures
demic, many problems occurred due to lockdown areas, increased waiting times and any treatments disruptions were analysed.
suspected cases, and limitation of non-urgent visits to the hospital. This Results: There were 862 and 273 new solid tumour cases treated at our
study analysed the difference in the number of visits for new oncology hospital before and during the pandemic, respectively. The mean age
patients and associated outcomes before and during the COVID-19 of all subjects was 6.9±5.5 years. The differences in the proportion of
pandemic. five most common solid tumor observed in our study before and dur-
Methods: A retrospective review of pediatric oncology patients was ing COVID-19 pandemic: retinoblastoma (23.2% vs 23.4%, p=0.934),
conducted in CMH from January 2015 to December 2021. Data of osteosarcoma (12.2% vs 10.6%, p=0.487), neuroblastoma (7% vs 9.9%,
all newly diagnosed oncology patients were collected. Each patient p=0.11), rhabdomyosarcoma (8.5% vs 5.1%, p=0.07), and non-Hodgkin
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S466 of S635 ABSTRACTS

lymphoma (8.6% vs 4.4%, p=0.02). We found delays in elective surg- care, and the rest on curative treatment. Abandonment rate was
eries and disruptions to chemotherapy and radiotherapy during the observed to be 15%, whereas 10% of patients expired following
pandemic, especially during the high wave period. Associated factors registration.
include reduction in health care services, staff shortages, waiting time Conclusions: Documentation of pediatric oncology cases at a
for COVID-19 test results and treatment disruption due to COVID-19 national level in the form of a comprehensive cancer registry
infection. is essential to establish disease incidence, prevalence and final
Conclusions: Our centre’s most common pediatric solid tumour was outcomes.
retinoblastoma, osteosarcoma, neuroblastoma, rhabdomyosarcoma,
and non-Hodgkin lymphoma. There was no significant decrease in the
total case number of the solid tumour during the COVID-19 pan- EP690 / #1684 AN ACCOMMODATION SUPPORT MODEL
demic. Varieties of diagnostic delays and treatment disruptions were FOR PEDIATRIC ONCOLOGY PATIENTS OF A TERTIARY CARE
observed. Strategies are needed to mitigate the significant delays and HOSPITAL IN KARACHI, PAKISTAN
disruptions in cancer care.
Wasfa Farooq1 , Muhammad Sohaib1 , Zaman Khan2 , Ghulam Qadir
Pathan1 , Muhammad Shamvil Ashraf2 , Muhammad Rafie Raza1
EP689 / #1673 ANNUAL DISEASE BURDEN OF PEDIATRIC 1 Indus Hospital and Health Network, Pediatric Oncology, Karachi, Pak-
ONCOLOGY PATIENTS PRESENTING TO A TERTIARY CARE istan; 2 The Indus Hospital and health Network, Paeds Oncology, Karachi,
HOSPITAL IN PAKISTAN Pakistan

Natasha Baig1 , Wasfa Farooq1 , Yumna Syed2 , Kafeel Naz Sajid2 , Background and Aims: Abandonment of cancer treatment compro-
Muhammad Rafie Raza1 mises the survival of approximately one in seven children worldwide
1 Indus Hospital and Health Network, Pediatric Oncology, Karachi, Pakistan; annually. Despite free of cost treatment facilities and availability of
2 Indus Hospital and Health Network, Electronic Medical Records, Karachi, specialized centers, over 20% oncology cases in Pakistan abandon ther-
Pakistan apy, leading to overall survival of under 35% compared to 80% in high
income countries. To promote social support systems in healthcare,
Background and Aims: Over 90% of children at risk of developing Indus Hospital & Health Network (IHHN) initiated Madina Residency,
cancer reside in low to middle income countries (LMICs) such as Pak- a one-of-a-kind facility for pediatric oncology patients on curative
istan. Unfortunately, overall survival rates fall far below high income treatment.
countries at 35%. For adequate resource allocation and improve- Methods: With the help of donors, IHHN acquired a 25 bedroom
ment of patient outcomes, accurate documentation and analysis of residence building in Karachi in 2019. This residence was offered to
the numbers and presentations of childhood cancer is essential. More- patients originating from remote areas with severely underprivileged
over, registry data is essential for hospitals to effectively manage backgrounds and on active curative treatment. The accommodation,
caseloads and streamline patient care, and contributes significantly food, transportation to the hospital and treatment offered was com-
to local policy making. Due to the absence of a national cancer reg- pletely free of cost.
istry, tracking systems and trained data operators, scarce information Results: Of the total 126 patients accommodated up to December
is available regarding childhood cancer incidence and outcomes in 2021, 90(71.4%) were male, with mean age of 8.2 ± 4.1 years. Distri-
Pakistan. bution of diagnoses displayed highest burden of leukemias 69(54.7%),
Methods: Supported by the My Child Matters grant, Indus Hospi- followed by solid tumors 28(22.2%) and lymphomas 12(9.5%). The
tal and Health Network (IHHN) established a comprehensive multi- average stay of Pakistani patients was 96 ± 86 days and neighboring
institutional hospital-based childhood cancer registry in 2016. Seven countries was 105 ± 85 days, with the longest stays ranging between
other pediatric oncology units across Pakistan also enter data into the 6 months to 1 year, in leukemia and bone tumor patients. Forty three
web-based IHHN registry via trained officers. Annual IHHN data from (34.1%) patients were from Afghanistan and 83(65.9%) from various
January to December 2021 was extracted and analyzed. far flung areas of Pakistan. Average monthly household income was
Results: A total of 1,073 childhood cancer cases registered at IHHN PKR 14,721 (USD 79.68), with an average of 7.3 ± 3.1 family members
in 2021. Gender distribution showed a male predominance of 63.7%, per household. On feedback at checkout, each of these patients stated
with females constituting only 36.3%. Mean age was 8.37 ± 4.41 that the residence facility directly prevented them from abandoning
years. A large majority of patients (58.4%) presented from various treatment.
cities across Pakistan, followed by residents of Karachi (38.4%) and Conclusions: Further studies and cost analysis of accommodation sup-
Afghanistan (3.2%). The most notable malignancies were leukemias, port models is essential for childhood cancer budgets to be invested
lymphomas (18.7%), abdominal tumours (12.3%), CNS tumors (6.4%), in effective preventive strategies. In order to improve overall survival,
soft tissue sarcomas (6.0%), retinoblastoma (5.8%), bone tumours early presentation and compliance to recommended treatment in safe
(5.0%), epithelial neoplasms and melanoma (2.9%), histiocytic disor- and hygienic environments is crucial, especially in low-middle income
ders (1.0%) and others (0.6%).Over 15% patients were on palliative countries.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S467 of S635

EP691 / #1699 DEVELOPMENT OF MULTIDISCIPLINARY Meike Ressing1 , Cornelia Becker1 , Christian Müller2 , Seyed
PEDIATRIC ONCOLOGY TUMOUR BOARDS: PERSPECTIVE Mahmoudpour1 , Gabriele Calaminus3 , Thorsten Langer4 , Friederike
FROM A LOW-MIDDLE INCOME COUNTRY Erdmann1 , Mathias Voigt1 , Melanie Kaiser1 , Claudia Spix1
1 University Medical Center of the Johannes Gutenberg-University Mainz,
Muhammad Rafie Raza1 , Natasha Baig1 , Wasfa Farooq1 , Syed Hamid1 , Institute Of Medical Biostatistics, Epidemiology And Informatics (imbei),
Nida Zia1 , Naema Khayyam1 , Fatima Ambreen1 , Naila Rafiq1 , Bilal Mainz, Germany; 2 Gert and Susanna Mayer Foundation, Foundation,
Mazhar Qureshi2 , Sadia Imran1 , Kishwar Nadeem1 , Quratulain Riaz1 , Wuppertal, Germany; 3 University Hospital Bonn, Department Of Pedi-
Nausheen Yaqoob1 , Kashif Shazlee1 , Fatima Meraj1 , Furqan Sheikh3 , atric Hematology And Oncology, Bonn, Germany; 4 University Hospital for
Muhammad Shamvil Ashraf1 Children and Adolescents, Pediatric Oncology And Hematology, Lübeck,
1 Indus Hospital and Health Network, Pediatric Oncology, Karachi, Pakistan; Germany
2 The Aga Khan University, Department Of Oncology, Karachi, Pakistan;
3 Sick Kids Hospital, Pediatric Oncology, Toronto, Canada Background and Aims: Subsequent neoplasms are one of the sever-
est late effects of the therapy of childhood cancer. In epidemiological
Background and Aims: Multidisciplinary tumour boards (MTBs) are research on this relevant E-Poster Topic, it can be necessary to aggre-
essential for provision of high quality, comprehensive cancer care. gate chemotherapy agents into substance classes, as in our study on
Establishment of MTBs enables the thorough discussion of patient Second Tumours after Tumour Therapy (STATT). Cumulative doses are
diagnoses, tumour staging and management by an expert committee, calculated using conversion factors by substance classes.
thus improving clinical management and outcomes. In countries such The aim of the review was to identify conversion factors from the
as Pakistan, where a national cancer plan is lacking and healthcare literature and to compare them to our novel approach.
systems are plagued with administrative and managerial gaps, MTBs Methods: First, a scoping review was performed in PubMed in August
allow formation of strong multidisciplinary teams to address various 2021 to compile conversion factors used in previous studies. Second,
problems. Moreover, tumour boards provide postgraduate trainees based on a comprehensive list of treatment protocols used in German
with an opportunity to present cases, enhance knowledge and inter- paediatric oncology, we derived alternative conversion factors from
act with national and international faculty and residents from different “typical doses” per substance with a novel approach. Finally, the con-
specialties. version factors were compared on a log scale using Pearson correlation
Methods: The Pediatric Hematology/Oncology (PHO) Department at coefficients and linear regression. Substances were grouped according
Indus Hospital and Health Network (IHHN) has organized and par- to the Anatomical Therapeutic Chemical Code (ATC).
ticipated in multiple MTBs for various pediatric malignancies since Results: The literature search identified 23 studies in which conversion
2015. Two solid tumour MTBs, twice a month in conjunction with factors were used or defined as described above. Mostly, the factors
international faculty and once a month on a multi-institutional level were presented as based on the relative toxicity or potency of the
have been conducted. In collaboration with international specialists, a drugs, though usually without a specific reference. Conversion factors
retinoblastoma and neuro-oncology tumor board each have occurred were available for 49 substances, either from the literature (n = 38) or
once a month. On a local, multi-institutional level, IHHN faculty have from typical doses (n = 41).
conducted monthly leukemia, lymphoma, Wilm’s tumor and pediatric The Pearson correlation coefficient for the 18 substances (excluding
sarcoma MTBs. Due to the COVID-19 pandemic, in-person MTBs were the 12 reference substances) with available factors based on both
shifted to virtual Zoom meetings. principles was r = 0.84 with a slope in linear regression of 0.75.
Results: Over 30 IHHN faculty members have attended and pre- Conclusions: The conversion factors from the two approaches were
sented cases at various MTBs, ranging from oncologists, hematologists, highly correlated and on average very similar. Hence, for substances
radiologists, histopathologists, pediatric surgeons (orthopedic, cardio- for which no conversion factor has been published so far, a factor based
thoracic, head and neck), radiotherapists and ophthalmologists. PHO on a “typical doses approach” may be used in epidemiological research.
fellows and residents in training from hematology, histopathology, Funding: Deutsche Krebshilfe, grant number: 70112099
radiology, general pediatrics and radiotherapy also participated. Over
three thousand cases have been discussed thus far.
Conclusions: Within the realm of pediatric oncology, a patient- EP693 / #1180 INTERNATIONAL ACCREDITATION OF A
centered multidisciplinary approach through the establishment of PEDIATRIC HEMATOLOGY/ONCOLOGY FELLOWSHIP PROGRAM
MTBs is essential to improve patient outcomes and survival. IN GUATEMALA

Roy Rosado1 , Claudia Garrido1 , Daniel Moreira2 , Leeanna Fox Irwin2 ,


EP692 / #176 EQUIVALENT DOSES FOR ANTICANCER Monika Metzger2 , Carlos Rodriguez-Galindo2 , Ricardo Mack1,3 ,
AGENTS USED IN PAEDIATRIC ONCOLOGY: A SCOPING Federico Antillón-Klussmann1,3
REVIEW AND EVALUATION OF A NOVEL APPROACH 1 Unidad Nacional de Oncología Pediatrica, Pediatric Hematology-oncology,

Guatemala, Guatemala; 2 St Jude Children’s Research Hospital, Global


15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S468 of S635 ABSTRACTS

Pediatric Medicine, Memphis, United States of America; 3 Universidad Guatemala City, Guatemala; 6 Barretos Children’s Cancer Hospital, Pediatric
Francisco Marroquin, Facultad De Medicina, Guatemala, Guatemala Hematology-oncology Unit, Barretos, Brazil; 7 IOP/GRAACC/UNIFESP, Pedi-
atric Oncology, Sao Paulo, Brazil; 8 Hospital Civil de Guadalajara Dr. Juan
Background and Aims: Graduate medical education programs in pedi- I Menchaca, Pediatric Oncology, Guadalajara, Mexico; 9 Hospital Italiano,
atric hematology/oncology are necessary to train specialists to provide 9. division Of Pediatric Surgery And Urology, Department Of Pediatrics,
future quality care for children with cancer. The Unidad Nacional de Buenos Aires, Argentina; 10 Hospital Luis Calvo Mackenna, Pediatric Oncol-
Oncología Pediátrica (National Pediatric Oncology Unit, UNOP) is Cen- ogy, Santiago, Chile; 11 Hospital JP Garrahan, Coordinación De Redes Y
tral America’s only hospital solely dedicated to pediatric cancer and Comunicación A Distancia, Buenos Aires, Argentina; 12 Commodin, Ceo &it
was founded in Guatemala City in 2000. In 2003, UNOP, together with Manager, Buenos Aires, Argentina; 13 Pan American Health Organization,
the Universidad Francisco Marroquin Medical School and St. Jude Chil- Non-communicable Diseases, Washington D.C, United States of America;
dren’s Research Hospital, created a fellowship program in pediatric 14 Hospital Pereira Rossell, Pediatric Oncology, Montevideo, Uruguay

hematology/oncology. Our aim is to describe the accreditation process


for the pediatric hematology/oncology program at UNOP. Background and Aims: Experts in most pediatric oncology areas are
Methods: The Accreditation Council for Graduate Medical Education scattered among many centers in Latin America and there are limited
- International (ACGME-I) accreditation process includes two main opportunities for trainees to be exposed to their experience. There-
steps: the accreditation of the institution overseeing a training pro- fore, TeLeo was created under the premise that academic excellence
gram; and the accreditation of the program itself of the trainees, the could be shared among centers by a virtual learning program to favor
faculty, and the program itself. networking and share context-sensitive high-quality content.
Results: After ACGME-I’s initial visit to UNOP in January of 2019 Methods: TeLeo was created with the support of a My Child Mat-
confirmed the areas of improvement, institutional and program level ters grant and promoted by Hospital Sant Joan de Déu, Foundations
changes were embarked upon. ACGME-I accredited UNOP as a spon- Messi and Flexer and the Latin American Society of Pediatric Oncology
soring institution on August 25, 2021. To apply for accreditation of (SLAOP). A digital platform was created to allow participants to access
the pediatric hematology/oncology fellowship program, multiple mod- the learning activities, stored educational content and interact through
ifications were also required. The didactics schedule and rotations social networking tools. Healthcare professionals involved in childhood
schedules were updated, and a system to support fellows’ research cancer care can freely access content at TeLeo after registration.
programs was designed, with a Scholarly Oversight Committee. Also, Results: A board was appointed including representatives from the
web-based evaluations were designed to provide structured evalua- major programs in Latin America. A two-year virtual training program
tions. The accreditation of the fellowship program was obtained on (144 hours) based on the SLAOP educational curriculum was launched
January 22, 2022. The process of accreditation has already improved to support fellowship programs. Weekly sessions in Spanish and Por-
the educational infrastructure and fellowship program educational tuguese, given by local and international experts were scheduled. In
organization, enabling a better training environment. its first edition beginning in 2021, 185 fellows from 40 centers (12
Conclusions: UNOP is the first pediatric hematology/oncology fel- countries) were enrolled. Additional courses were produced to further
lowship program in the world accredited by ACGME-I. The UNOP support the program such as an interactive bone marrow morphology
fellowship program should be considered a model for future regional (40 students, 10 hours), imaging for pediatric oncologists (310 atten-
workforce training programs in pediatric hematology/oncology, as well dees, 12 hours), essential drugs for leukemia treatment (235 assistants,
as in other specialties. 9 hours), as well as a two-day pediatric psycho-oncology meeting (440
attendees). The platform also hosted the 2020 and 2021 SLAOP virtual
congresses and overall, more than 3,000 professionals registered.
EP694 / #1013 TELEO PROGRAMME: TELE-EDUCATION IN Conclusions: TeLeo is a successful program to offer a free online edu-
PEDIATRIC ONCOLOGY AS A TOOL TO SUPPORT TRAINING cational tool for healthcare professionals related to oncology in Latin
PROGRAMMES IN LATIN AMERICA America highlighting the importance of a collaborative network to
provide free and high-quality contents for the future generations of
Claudia Sampor1 , Rocio Alonso2 , Ofelia Cruz3 , Daniel Moreira4 , pediatric oncologists and other professionals related to cancer care.
Federico Antillón-Klussmann5 , Luiz Fernando Lopes6 , Andrea
Cappellano7 , Oscar Gonzalez Ramella8 , Pablo Lobos9 , Julia Palma10 ,
Alejandro Mato11 , Pablo Lechuga11 , Pablo Schiavo11 , Antonio Luna11 , EP695 / #39 IMPACT OF CLINICAL PHARMACIST IN
Diego Rios12 , Liliana Vasquez13 , Andres Morales3 , Guillermo OPTIMIZATION OF DRUG THERAPIES FOR EFFECTIVE
Chantada3,14 OUTCOMES IN HOSPITALIZED PEDIATRIC ONCOLOGY
1 Hospital JP Garrahan, Pediatric Oncology, Buenos Aires, Argentina; PATIENTS
2 Fundacion Natali D Flexer, Project Manager, Buenos Aires, Argentina;
3 Hospital Sant Joan de Deu, Pediatric Oncology, Barcelona, Spain; 4 St. Summyya Shakil, Ayesha Hanif, Arif Ali
Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis, Indus Hospital and Health Network, Pharmacy, Karachi, Pakistan
United States of America; 5 National Pediatric Oncology Unit, Oncology,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S469 of S635

Background and Aims: Background Oncology patients especially A clinical guideline specifically for bone and abdominal tumours will
pediatric patients need special care since they are on multi drug regi- provide evidence-based guidance to allow prompt diagnosis.
mens that are complex in nature. These require close monitoring due Methods: Emails of invitation were sent to healthcare professionals
to potential interactions, precise dosing and adverse reactions. As per (HCPs) to join the Delphi panel. 65 statements were derived from evi-
CDC, approximately 1.3 million ER visits and 350,000 hospitalizations dence review by a multidisciplinary team. Participants were asked to
each year in United States are due to Adverse Drug Events. Being one rank their agreement with the statements by means of a 9-point Likert
of the last safety checks, clinical pharmacists are vital in prevention of scale (1 = strongly disagree; 9 = strongly agree). Statements reached
adverse drug events and near miss prescribing/transcribing errors in consensus if 70% or more rated the statement 7, 8 or 9. Rankings for
clinical practice. Aim Role of clinical pharmacist in improving patient each statement were collated and any statement achieving the pre-
safety and quality of care in hospitalized pediatric oncology patients. determined level of consensus was accepted. Statements not reaching
Methods: This is a retrospective observational study analyzing clini- consensus were rewritten and re-issued in a subsequent round.
cal pharmacist interventions from November 2020-November 2021 in Results: 133 healthcare professionals took part. All statements
pediatric oncology patients at Indus Hospital and Health Network. achieved consensus at the end of two rounds. 96 of 133 (72%) par-
Results: Total 1,149 interventions were done. Addition of drug was ticipants responded to the Round 1 (R1). 62 of 65 (94%) statements
highest i.e., 242(21%), followed by lab based, 167(14.5%) and inappro- achieved consensus in R1 with 29 (47%) gaining more than 90% con-
priate duration, 126(10.9%). Less frequent were wrong route selection, sensus. Three statements did not reach consensus scoring between
wrong dosage form selection, switching to alternate, 2(0.17%) each, 60.9 and 69.1%. All reached numerical consensus at the end of Round
followed by hepatic dose adjustment, 4(0.34%), IV to PO, 8(0.69%) and 2. One statement was deemed similar to another and so was omitted
CS based interventions 9(0.78%). Other interventions include duplica- from the final list.
tion of class/activity 97(8.4%), supra-therapeutic dose 85(7.39%), sub- Conclusions: This consensus process has produced expert guidance for
therapeutic dose 83(7.22%), wrong frequency selection 80(6.96%), a new clinical guideline for suspected bone and abdominal tumours
duplication medication order 60(5.2%), unnecessary drug and interac- for use in both primary and secondary care. This evidence base will
tion 35(3%) each, therapy modification 26(2.2%), drug-disease interac- be translated into awareness tools for the public as part of the Child
tion and renal dose adjustment 19(1.6%) each, wrong drug selection Cancer Smart national awareness campaign.
and inappropriate dose 17(1.47%) each and contraindicated drug
prescribing contributes to 13(1.13%) interventions Considering risk,
significant and highly significant were 597(51.95%) and 545(47.43%) EP697 / #688 DEVELOPING A HARMONISED FRAMEWORK
respectively while 7(0.6%) were lethal. FOR MONITORING OUTCOMES AND IMPACT OF DONOR
Conclusions: Interventions are aimed at improving outcomes. Incorpo- INVESTMENT INTO PAEDIATRIC CANCER SERVICES IN LOW-
rating pharmacist in clinical practice of specialized area of patient care AND MIDDLE-INCOME COUNTRIES
has great potential to reduce drug related problems to a greater extent
thus optimizing therapeutics and improving patient safety. This study Forbes Sharp1 , Glenn Mbah Afungchwi2 , Alison Finch3 , Piera
also highlights need of more training programs of prescribers in terms Freccero1 , Rachel Hollis4
of drug use. 1 World Child Cancer, Programme Department, London, United King-
dom; 2 Cameroon Baptist Convention Health Services, Pediatric Oncology,
Mbingo, Cameroon; 3 University College London Hospitals NHS Foundation
EP696 / #95 A MODIFIED E-DELPHI CONSENSUS PROCESS Trust, Nursing, London, United Kingdom; 4 Leeds Teaching Hospitals NHS
FOR THE DEVELOPMENT OF A CLINICAL GUIDELINE FOR Trust, Nursing, Leeds, United Kingdom
SUSPECTED BONE AND ABDOMINAL TUMOURS
Background and Aims: Improving the scale and quality of paedi-
Shaarna Shanmugavadivel1 , Jo-Fen Liu1 , Ashley Ball-Gamble2 , Angela atric cancer services in low- and middle-Income countries (LMICs)
Polanco2 , Kavita Vedhara1 , David Walker3 , Shalini Ojha1 is often contingent on financial investment from external, institu-
1 University of Nottingham, Academic Unit Of Population And Lifespan Sci- tional donors. Securing longer-term donor investment requires that
ences, Nottingham, United Kingdom; 2 Children’s Cancer and Leukaemia downstream partners (e.g., clinical services) evidence the health out-
Group, Ceo, Leicester, United Kingdom; 3 University of Nottingham, Chil- comes of this investment. World Child Cancer (WCC), a UK-based
dren’s Brain Tumour Research Centre, Nottingham, United Kingdom Non-Governmental Organisation, set out to determine and pilot a
standardised set of indicators to measure the impact of financial invest-
Background and Aims: The incidence of childhood cancer has risen by ments which would satisfy the requirements of institutional donors
15% in the UK and is the leading illness cause of death in children and whilst being feasible to collect and relevant for health care providers
young people (CYP). Childhood cancer poses a diagnostic dilemma to delivering and developing services.
clinicians due to the non-specificity of symptoms. Although the over- Methods: World Child Cancer reviewed 29 indicators from six pro-
all five-year survival is 84%, bone and abdominal tumours still have grammes across Africa and Asia that had invested in scaling up and/or
the lowest survival rates with many experiencing delays to diagnosis. improving quality of paediatric cancer services. A WCC working party
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S470 of S635 ABSTRACTS

of four, led by the Quality Manager and including the programme tunities Threats) exercises were conducted in focus groups. Interviews
coordinator from Cameroon, systematically determined whether: 1. a were transcribed and analyzed for identification of recurrent themes.
common approach/structure to programme design was evident across Results: Interviewees highlighted the following important considera-
the six programmes 2. a standard set of relevant and feasible out- tions for expanding pediatric cancer services that would significantly
come indicators could be developed to measure across the different impact the financial plan: need for incorporation of adolescent patients
programmes 3. these were suitable to satisfy the requirements of insti- to the pediatric care model, improvement of palliative care services,
tutional donors whilst relevant and useful to programme managers and and expansion of laboratory capabilities. Barriers and enabling factors
providers. to financial planning were also identified. Barriers included difficulties
Results: There was a common approach/structure to programme with staff retention, reliance on local donations for operating costs, and
design that was evidenced across different contexts. Twenty-four com- medication access and cost. Enabling factors included mission-driven
mon indicators could be determined, which were then piloted. These leadership, highly skilled subspeciality nursing and nurse training, mul-
indicators satisfied most donor requirements for demonstrating out- tiple national and international partnerships, and focus on future
comes and impact and were also relevant for programme managers and advocacy.
clinical practice. Conclusions: The CMAV/HNBB experience builds on previous anal-
Conclusions: This feasibility study suggests there is scope for a rela- ysis of financial planning practices in LMICs, which continues to
tively simple, standardised approach to measuring the impact of donor emphasize the need for systematic and data-driven financial planning,
investment in paediatric oncology in low- and middle-income countries. consensus-building, and buy-in for the effective projection of costs and
The framework has potential to facilitate increased donor confidence sustainability of new pediatric oncology services.
in long-term, sustainable investment whilst also assuring that relevant
and useful data is available to service providers to monitor the impact
and outcomes of their programmes. EP699 / #1902 HYPOVITAMINOSIS D IN CHILDREN
DIAGNOSED WITH CANCER: SYSTEMATIC REVIEW AND
META-ANALYSIS
EP698 / #312 FINANCIAL PLANNING FOR EXPANSION OF
PEDIATRIC CANCER SERVICES IN SAN SALVADOR, EL Karla M. Silva-Jivaja1 , Osvaldo D. Castelán-Martínez2 , Miguel
SALVADOR Palomo-Colli3
1 Universidad Nacional Autónoma de México, Clinical Pharmacology,
Nitin Shrivastava1 , Irini Albanti2 , Roberto Vásquez Zelaya3 , Soad CDMX, Mexico; 2 Universidad Nacional Autónoma de México, Clinical Phar-
Fuentes-Alabi De Aparicio3 macology Laboratory, CDMX, Mexico; 3 Hospital Infantil de México Federico
1 Dana Farber Cancer Institute, Pediatric Hematology/oncology, Boston, Gómez, Oncology, CDMX, Mexico
United States of America; 2 Harvard Humanitarian Initiative, Brigham And
Women’s Hospital, Cambridge, United States of America; 3 Centro Médico Background and Aims: Vitamin D is an essential hormone for bone
Ayúdame a Vivir/Hospital Nacional de Niños Benjamin Bloom, Pediatric metabolism, growth, immune system regulation, and metabolism. Vita-
Oncology, San Salvador, El Salvador min D deficiency and insufficiency, also known as hypovitaminosis D,
have been associated with the survival of cancer patients, as well as the
Background and Aims: While need for pediatric oncology services appearance of adverse reactions to chemotherapy. Therefore, the aim
exists in low-and-middle-income countries (LMICs), planners have lim- of this systematic review and meta-analysis was to synthesize the evi-
ited access to user-friendly and context-appropriate resources to dence on the prevalence of hypovitaminosis D in pediatric patients at
develop a comprehensive, evidence-based financial plan for the estab- the diagnosis of any type of cancer.
lishment of these services. Leadership at Centro Médico Ayúdame a Methods: A systematic literature review was carried out in MEDLINE
Vivir (CMAV) and Hospital Nacional de Niños Benjamin Bloom (HNBB) without restrictions. Observational studies that determined hypovi-
in San Salvador, El Salvador have identified a need to expand their taminosis D at the diagnosis of any type of pediatric cancer were
pediatric cancer services. This study aims to explore important inter- included. Studies where patients received vitamin D supplementation
nal and external considerations for financial planning for expansion at the time of diagnosis were excluded. The meta-analysis of the preva-
of a pediatric cancer unit at CMAV; use a financial planning tool to lence’s with 95% confidence intervals (95% CI) was conducted under
determine estimated construction, start-up, and operational costs; and a random effects approach using the R program. A subgroup analysis
summarize important lessons learned to improve knowledge-sharing was performed to estimate the proportion of vitamin D deficiency and
globally. insufficiency.
Methods: Key informant interviews were conducted with twenty- Results: Thirty-two studies were identified as potentially relevant.
three clinical and non-clinical CMAV and HNBB staff members includ- After analyzing titles, abstracts, and full titles, 7 studies with 1143
ing physicians, nurses, psychosocial providers, administrators, founda- patients were included in the review. The proportion of hypovita-
tion and hospital leadership, and Ministry of Health representatives. minosis D was 0.37 ([95%CI 0.30 to 0.44], I2 = 83%). In subgroup
Budgeting, what-if scenarios, and SWOT (Strength Weakness Oppor- analysis, the proportion of patients with vitamin D deficiency was 0.45
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S471 of S635

([95% CI 0.34 to 0.56], I2 = 85%), while for insufficiency it was 0.30 EP701 / #1830 ANY CONCERN ABOUT DELAYS IN
([95% CI 0.26 to 0.34], I2 = 0%). DIAGNOSIS OF CHILDHOOD CANCERS DURING COVID-19
Conclusions: This systematic review with meta-analysis shows PANDEMIC?
that pediatric cancer patients have a high prevalence of hypovi-
taminosis D. This deficiency can condition patients to suffer Begum Koc1 , Funda Tekkesin1 , Elif Haccaoglu2 , Beyter Busra2 , Selime
long-term adverse reactions such as lower bone mineralization, Aydogdu1 , Suar Kilic1
especially in patients receiving doses high methotrexate or prolonged 1 Umraniye Training and Research Hospital, Pediatric Hematology And
corticosteroids. Oncology, Istanbul, Turkey; 2 Umraniye Training and Research Hospital,
Pediatrics, Istanbul, Turkey

EP700 / #1512 SCALE OF CHILDHOOD CANCER Background and Aims: Early diagnosis of cancer ensures better prog-
INEQUALITIES: NEW WHO EUROPEAN REGION REPORT nosis with lower metastasis and higher cure rates. The lock-down
precautions during Covid-19 pandemic led to concerns about delayed
Vitaly Smelov, Yuliya Lyamzina, Maria Lasierra Losada, Marilys Corbex diagnosis of malignancies. In this study, we aimed to compare the dura-
WHO Regional Office for Europe, Ncd Management, Division Of Country tion of complaint at home and presence of metastasis at diagnosis
Health Programmes, Copenhagen, Denmark during pre-pandemic and pandemic period in children with cancer.
Methods: We have 300 patients between 0-18 years of age who have
Background and Aims: Strong inequalities remain across the 53 been followed-up in our pediatric oncology clinic, since 2017. In this
Member States of the WHO European Region. They arise along study, all the children with cancer except leukemia were included. Age,
the whole cancer care continuum, from access to care. Avail- gender, cancer type, duration of complaints and presense of metas-
able evidence and information were collated together and ana- tasis at diagnosis were recorded. The duration of complaints before
lyzed in the first report on childhood cancer inequalities across the admission and the presence of metastasis at diagnosis were compared
Region. statistically before and after March 11, 2020, the start point Covid-19
Methods: The report summarized literature in four main areas: the pandemic in our country.
childhood cancer continuum, inequalities across countries, inequali- Results: A total of 161 patients diagnosed with cancer except
ties within countries, and childhood cancer as a driver of inequalities. leukemia between 2017-2022 were analyzed retrospectively. 61% of
Where possible, studies from the 53 countries of the WHO European the patients were male, while 39% were female. These patients were
Region were used. diagnosed with brain tumours (23.6%), lymphomas (23%), rhabdo/non-
Results: Disparities between countries included: incidence rates vary- rhabdomyosarcomas (14.3%), neuroblastoma (13.7%), Ewing sarcoma
ing due to high levels of underdiagnosis/reporting, higher childhood (4.3%), osteosarcoma (3.7%), Wilm’s tumor (3.7%), germ cell tumors
cancer proportion of all cancers in countries with lower HDI scores, (3.1%). Distant metastases were present in 42.3% of the patients.
wider than for adult cancers disparities in survival rates between The number of patients were 59 and 102 before and after the pan-
higher- (HIC), and lower-and-middle-income (LMIC) countries. Lack demic, respectively. The duration of complaint was longer (median 45
of precise and timely universal access to high-quality first-line diag- days) during the pandemic period than before pandemia (median 30
nostics, contemporary therapy, and supportive care result in poorer days) (p<0.05). The presence of metastases at diagnosis was 45.3% in
outcomes. Within individual countries: boys with cancer have poorer the pre-pandemic period, while it was 40% during pandemia with no
outcomes than girls (HIC) but are less frequently underdiagnosed statistical difference (p>0.5).
(LMIC); higher socioeconomic status results in higher survival rates; Conclusions: We concluded that the duration of complaint before diag-
centralized services require patients to travel large distances for nosis was found to be longer during pandemia while this delay did not
care, but dispersed services lack enough expert staff; and data on affect the metastasis rate at diagnosis in children with cancer.
migrants remain limited. A childhood cancer diagnosis can lead to
significant short- and long-term inequalities in the life of the child
(vulnerabilities in physical health, well-being and access to follow- EP702 / #826 ACUTE LEUKEMIAS: DIAGNOSIS AND
up care, worse quality of life and lower academic achievement than CLASSIFICATION CHALLENGE IN LOW- MIDDLE INCOME
peers without cancer) and their entire family (financial difficulties and COUNTRY (LMIC)
poorer quality of life), with more frequent reliance on government
insurance. Awa Toure1 , Fatimata Sall2 , Moussa Ndour3 , Abou Koundio2 ,
Conclusions: Within the framework of the Global Initiative for Abibatou Sall4 , Mame Diouf5 , Fatou Diagne5 , Macoura Gadji6 ,
Childhood Cancer, the current report proposes recommen- Raphael Martine7 , Cherif Dial8
dations on the key steps that are likely to have the greatest 1 Cheikh Anta Diop University/Aristide Le Dantec Hospital, Haematol-
impact in reducing inequalities and is advocating for more data ogy, Dakar, Senegal; 2 Aristide Le Dantec Hospital, Haematology, Dakar,
collection to better understand them across the Region and Senegal; 3 Aristide Le Dantec Hospital, Immunology, DAKAR, Senegal;
beyond. 4 Cheikh Anta Diop University/Dalal Jamm Hospital, Haematology, Dakar,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S472 of S635 ABSTRACTS

Senegal; 5 Aristide Le Dantec Hospital, Pediatry, Dakar, Senegal; 6 Cheikh Background and Aims: In low- and middle-income countries (LMIC),
Anta Diop University, Haematology, Dakar, Senegal; 7 AMCC, Heamatol- hospital-based pediatric cancer registries are often non-existent or
ogy, Paris, France; 8 Cheikh Anta Diop University, Anatomopathology, Dakar, of limited quality. However, these registries are an important tool
Senegal for improving the quality of patient care within hospitals and allow
for multicenter registries and studies. This study on hospital-based
Background and Aims: Acute leukemia (AL) seems to be the most pediatric cancer registries in LMIC maps: 1) all existing literature;
common worldwide childhood malignancy, accounting more than 25% 2) requirements needed for successful implementation; and 3) its
of all childhood cancer. In Senegal, an onco-pediatric unit (UOP) has benefits.
been opened for 20 years, in Dakar, to improve the therapeutic follow Methods: A scoping review, in line with the PRISMA-ScR guidelines
up of patients. In order to improve the diagnosis and to deliver a timely (2020) was conducted searching the following databases: Medline,
accurate diagnosis, a Reference Center for Diagnosis of childhood Pubmed, Cochrane and Web of Science. Additionally, reference lists
Cancers (CRDCE) was created in Dakar. of selected papers were screened for relevance (‘snowballing’ tech-
Methods: Hematologists, pathologists, immunologists, specialist for nique). All peer-reviewed literature on hospital-based pediatric cancer
cytogenetics and clinicians were gathered working together for this registries in LMIC published in English between 1990 and 2021
challenge. was considered eligible for inclusion. Three reviewers independently
Results: During the year 2021, 226 malignancies (67 acute leukemias screened all titles and abstracts and consequently the full texts.
(AL), 39 nephroblastoma, 28 retinoblastoma, 18 lymphomas, 16 neu- Results: In total, 17 articles were included for content analysis. Reports
roblastoma, 16 rhabdomyosarcoma) were diagnosed. Children with AL of hospital-based pediatric cancer registries concerned: Africa (n=8),
referred in the Pediatric Unit were evaluated by cytology, cytochemical Latin-America (n=4), Asia (n=3), and LMIC in general (n=2). The major-
staining and immunophenotyping. The median age of children with AL ity of registries were established through twinning projects around
is 60 months [6 – 228] with the incidence rate of males (55.2%). Periph- 1990. Most cancer registries were not pediatric specific, were pri-
erical blood and myelogram blasts count performed to confirm and marily used to provide epidemiological data, and had limited or no
classify AL according to the FAB classification, cytochemical staining clinical follow-up data. Fifteen studies distinguished the following
was used for some cases. They revealed 44 Acute Lymphoid leukemia requirements: ownership, availability of technical infrastructure, local
(ALL), with 33 new cases and 23 Acute Myeloid Leukemia (AML) with leadership, trained data managers, international coding, sustainabil-
22 first diagnosis. Relapses were represented by 11 ALL and 1 case ity, and funding. Thirteen studies described benefits of registries as
of AML. After the setting of flow cytometry, 33 cases of AL were being vital for: collecting population data on pediatric cancer, evaluat-
immunophenotyped: 21 cases were classified as ALL (11 B-ALL, 10 ing patient care, enabling high quality research, facilitating comparison
T-ALL) and 12 as AML. Immunophenotyping adjusted the cytological with other centers (external benchmarking), and planning of appropri-
diagnosis in 3 cases: 2 patients classified by cytology as ALL, become ate health-services to improve long-term clinical outcomes in LMIC.
AML and one AML was really ALL. Conclusions: Hospital-based pediatric cancer registries are often lack-
Conclusions: The improvement of distinction between lymphoid and ing in LMIC. There is a strong need for easy accessible registries,
myeloid leukemias by flow cytometry is crucially important and actu- inclusive of international standards, to evaluate pediatric cancer care.
ally works in the CRDCE. In fact, cytogenetics abnormalities appear This may ultimately improve survival of children with cancer in LMIC.
to be most important for identifying entities with prognostic and clin-
ical behavior. This will be the next step of our Reference Center.
Acknowledgments: AMCC GFAOP Sanofi Espoir Foundation EP704 / #250 REGIONAL COLLABORATION CAN
ACCELERATE SUCCESS: THE EXPERIENCE OF THE GLOBAL
INITIATIVE FOR CHILDHOOD CANCER IN LATIN AMERICA
EP703 / #1214 HOSPITAL-BASED PEDIATRIC CANCER AND THE CARIBBEAN
REGISTRIES IN LOW AND MIDDLE-INCOME COUNTRIES: A
SCOPING REVIEW Liliana Vasquez1 , Soad Fuentes-Alabi De Aparicio1,2 , Sara Benitez1 ,
Mauricio Maza1 , Karina Braga Ribeiro1 , Monnie Abraham3 , Asya
Laura Van Tinteren1 , Sterre Schoon1 , Aros Salah1 , Festus Njuguna2 , Agulnik3 , Justin Baker4 , Daniel Bastardo Blanco5 , Miguela Caniza6 ,
Pudjo Widjajanto3 , Gertjan Kaspers1,4 , Saskia Mostert1,4 Adolfo Cardenas3 , Carmen Salaverria7 , Courtney Sullivan8 , Erika
1 Princess Máxima Center for Pediatric Oncology, Outreach, Utrecht, Damasco9 , Ximena Garcia3 , Patricia Loggeto3 , Michael Mcneil3 ,
Netherlands; 2 Moi University, Department Of Child Health And Pediatrics, Sandra Luna-Fineman10 , Nuria Rossell11 , Regina Aparecida Garcia De
Eldoret, Kenya; 3 Faculty of Medicine, Public Health and Nursing, Univer- Lima12 , Regina Holanda De Mendonca13 , Viviana Trigoso14 , Lorena
sitas Gadjah Mada - Dr Sardjito Hospital, Department Of Child Health, Segovia Weber3 , Roberto Vasquez2 , Florencia Moreno15 , Paola
Pediatric Hematology Oncology Division, Yogyakarta, Indonesia; 4 Emma Friedrich3 , Silvana Luciani1 , Catherine G. Lam3 , Monika Metzger3
Children’s Hospital, Amsterdam UMC, Vrije Universiteit, Pediatric Oncology, 1 Pan American Health Organization, Non-communicable Diseases, Wash-
Amsterdam, Netherlands ington D.C, United States of America; 2 Hospital Nacional de Ninos Benjamin
Bloom, Pediatric Oncology, San Salvador, El Salvador; 3 St. Jude Children’s
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S473 of S635

Research Hospital, Department Of Global Pediatric Medicine, Memphis, TN, Conclusions: LAC Working Groups’ ongoing dialogue and commitment
United States of America; 4 St. Jude Children’s Research Hospital, Depart- are essential foundations to successfully accelerate GICC implementa-
ment Of Global Pediatric Medicine, Memphis, United States of America; tion through collaboration among multidisciplinary stakeholders. Thir-
5 St. Jude Children’s Research Hospital, Department Of Strategic Communi- teen countries entering the implementation phase are being actively
cation, Education And Outreach, Memphis, TN, United States of America; helped by this experience.
6 St. Jude Children’s Research Hospital, Department Of Global Pediatric
Medicine, Memphis, United States of America; 7 Ayudame a Vivir Founda-
tion, Psychooncology, San Salvador, El Salvador; 8 University of Alabama EP705 / #408 MIGRATION OF CHILDREN WITH CANCER IN
at Birmingham, Center For Outcomes And Effectiveness Research And LATIN AMERICA: A REGIONWIDE CROSS-SECTIONAL SURVEY
Education, Birmingham, United States of America; 9 Columbia University OF PEDIATRIC ONCOLOGY PROVIDERS
Irving Medical Center, Division Of Pediatric Hematology/oncology/stem Cell
Transplant, New York, United States of America; 10 University of Colorado Liliana Vasquez1 , Soad Fuentes-Alabi De Aparicio2 , Patricia Loggeto3 ,
School of Medicine, Pediatric Oncology, Aurora, United States of Amer- Andrea Cappellano4 , Claudia Sampor5 , Julia Palma6 , Milena
ica; 11 Fundación Nuestros Hijos, Extensión Y Desarrollo, Santiago, Chile; Villarroel7 , Diana Valencia8 , Mercedes Garcia Lombardi9 , Wendy
12 University of São Paulo at Ribeirão Preto College of Nursing/PAHO/WHO Gomez Garcia10 , Eva Lezcano Caceres11 , María Victoria Sobrero12 ,
Collaborating Center for Nursing Research Development, Department Of Lilia Garcia13 , Victor Cabrera14 , Ivan Maza15 , Thelma Velasquez16 ,
Maternal-infant And Public Health, Ribeirao Preto, Brazil; 13 Centro Infantil Cecilia Ugaz17 , Jacqueline Montoya Vasquez17 , Rosdali Diaz
Boldrini, Dentistry Unit, Campinas, Brazil; 14 Pontifical Catholic Univer- Coronado17 , Natalia Gonzalez18 , Simone Aguiar19 , Agustin
sity of Peru (PUCP), Research Group On Psychology And Health: Healthy Dabezies20 , Florencia Moreno21 , Susan Sardinas22 , Yessika
Environments, Lima, Peru; 15 National Cancer Institude-Argentina, Oncope- Gamboa23 , Essy Maradiegue24 , Ligia Fu25 , Pascale Gassant26 ,
diatric, Buenos Aires, Argentina Katiuska Moreno27 , Oscar Gonzalez Ramella28 , Magdalena
Schelotto29 , Sandra Luna-Fineman30 , Celia Gianotti Antoneli31 ,
Background and Aims: The Global Initiative for Childhood Cancer Roberto Vásquez Zelaya32 , Patricia Calderon33 , Filomena
(GICC) aims to increase the cure rate for children with cancer globally Moschella34 , Carmen Salaverria35 , Sara Benitez1 , Mauricio Maza1 ,
by improving healthcare access and quality. In Latin America and the Guillermo Chantada29 , Monika Metzger36
Caribbean (LAC), 29,000 children and adolescents develop cancer, and 1 Pan American Health Organization, Non-communicable Diseases, Wash-
10,000 will die yearly. The Pan American Health Organization (PAHO), ington D.C, United States of America; 2 Pan American Health Organization,
St Jude Children’s Research Hospital (SJCRH), and collaborators have Non-communicable Diseases, Washington D.C., United States of America;
joined efforts to improve outcomes of children with cancer in LAC using 3 St. Jude Children’s Research Hospital, Department Of Global Pediatric
the CureAll framework. We report on the LAC Working Groups’ plans Medicine, Memphis, TN, United States of America; 4 IOP/GRAACC/UNIFESP,
and activities to accelerate GICC implementation. Pediatric Oncology, Sao Paulo, Brazil; 5 Hospital JP Garrahan, Pediatric
Methods: CureAll delineates the childhood cancer pathway, includ- Oncology, Buenos Aires, Argentina; 6 Hospital Luis Calvo Mackenna, Pedi-
ing multidisciplinary and family-centered care. In March 2021, PAHO atric Oncology, Santiago, Chile; 7 Hospital Luis Calvo Mackenna, Pediatric
formed and coordinated regional working groups focusing on core Oncology, Santiago, Chile, Pediatric Oncology, Santiago, Chile; 8 Hospital
projects aligned to CureAll pillars and enablers in implement- Universitario de Santander, Pediatric Oncology, Santander, Colombia;
ing the GICC. Seven working groups emerged from regional dia- 9 Hospital de Niños Ricardo Gutierrez, Pediatric Oncology, Buenos Aires,
logues: early detection, treatment abandonment, nursing, palliative Argentina; 10 Dr. Robert Reid Cabral Children’s Hospital, Pediatric Oncology,
care, nutrition, psychosocial, and supportive care. PAHO orches- Sto Domingo, Dominican Republic; 11 Hospital Central Instituto de Previ-
trated regular online meetings under the mentorship and sup- sion Social, Pediatric Oncology, Pediatric Oncology, Asunción, Paraguay;
port of SJCRH’s regional/transversal programs and international 12 Hospital Ramon Carrillo, Pediatric Oncology, Bariloche, Argentina;
mentors. 13 Centro Universitario Contra el Cáncer UANL, Pediatric Oncology, Monter-

Results: Between Apr/21 and Dec/21, 202 subject matter experts rey, Mexico; 14 Hospital Regional Río Blanco, Pediatric Oncology, Orizaba,
attended 43 online meetings to promote the dialogue between stake- Mexico; 15 Hospital Rebagliati, Pediatric Oncology, Lima, Peru; 16 Unidad

holders to improve childhood cancer outcomes. Twenty four (24) Nacional de Oncología Pediátrica, Pediatric Oncology, Pediatric Oncol-
technical outputs were produced in English, Spanish, and Portuguese: ogy, Guatemala, Guatemala; 17 Instituto Nacional de Enfermedades Neo-
4 regional snapshots (palliative care, abandonment of treatment, early plásicas, Pediatric Oncology, Lima, Peru; 18 Hospital Militar Nacional
diagnosis, and nutrition), 3 technical guidelines (nutrition, oral care, de Colombia, Pediatric Oncology, Bogotá, Colombia; 19 IOP/ GRAACC/
and early diagnosis of childhood cancer), 1 technical report (pediatric UNIFESP, Pediatric Oncology, São Paulo, Brazil; 20 Pediatric Hemato
oncology nursing), 6 fact sheets (psychosocial care), and educational Oncology Honorary Advisor Consultant MUCAM, Pediatric Oncology,
and communication materials (2 virtual courses on early diagnosis and Montevideo, Uruguay; 21 Registro Onco-pediatrico Hospitalario Argentino
palliative care, and 8 modules on pediatric palliative care for care- (ROHA, Hospitalbased Pediatric Cancer Registry from Argentina), Registry,
givers). Countries in LAC engaged in GICC dialogues, activities and Buenos Aires, Argentina; 22 Hospital del Niño Ovidio Aliaga Uria, Pedi-
implementation have grown to 19. atric Oncology, La Paz, Bolivia; 23 Hospital Nacional de Niños, Pediatric
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S474 of S635 ABSTRACTS

Oncology, Pediatric Oncology, San José, Costa Rica; 24 Ministry of Health of EP706 / #933 PREVALENCE OF MALNUTRITION IN
Peru, Direction Of Strategic Interventions, Lima, Peru; 25 Hospital Escuela, CHILDREN WITH CANCER: A STUDY OF 3608 CHILDREN
Pediatric Oncology, Tegucigalpa, Honduras; 26 Hôpital Saint - Damien, Pedi- ACROSS INDIA
atric Oncology, Port-au-Prince, Haiti; 27 Hospital Verdi Cevallos Balda -
Hospital especialidades Portoviejo, Pediatric Oncology, Manabi, Ecuador; Sripriya Venkiteswaran1 , Anju Morarka2
28 Hospital Civil de Guadalajara Dr. Juan I Menchaca, Pediatric Oncology, 1 Cuddles Foundation, Cuddles Institute Of Clinical Nutrition (cicn), Mum-
Guadalajara, Mexico; 29 Hospital Pereira Rossell, Pediatric Oncology, Mon- bai, India; 2 Cuddles Foundation, Research, Knowledge Management And
tevideo, Uruguay; 30 University of Colorado School of Medicine, Pediatric Impact, Mumbai, India
Oncology, Aurora, United States of America; 31 Universidade Nove the Jul-
hio, Pediatric Oncology, São Pablo, Brazil; 32 Centro Médico Ayúdame a Background and Aims: Large scale data on malnutrition prevalence in
Vivir/Hospital Nacional de Niños Benjamin Bloom, Pediatric Oncology, San paediatric cancer across India is lacking as most studies till date have
Salvador, El Salvador; 33 Hospital Manuel de Jesus Rivera, Pediatric Oncol- reported centre- or hospital-specific prevalence rates. Hence, the aim
ogy, Managua, Nicaragua; 34 serviciHospital Universitario Dr. LUIS RAZETTI, of this retrospective study was to analyse the prevalence of malnutri-
Pediatric Oncology, Estado Anzoategui, Venezuela; 35 Hospital Nacional de tion in paediatric cancers in 33 government hospitals across India that
Ninos Benjamin Bloom, Pediatric Oncology, San Salvador, El Salvador; 36 St Cuddles Foundation (CF) partners with.
Jude Children’s Research Hospital, Global Pediatric Medicine, Memphis, Methods: FoodHeals
R
is a CF-developed app that collects data from
United States of America patients who have been counselled by a CF nutritionist. Anthropomet-
ric data uploaded between Feb 2021 and Jan 2022 of patients in the
Background and Aims: This study aims to understand the situation induction phase was analysed. The nutritional status of children with
of migrant children and adolescents diagnosed with cancer (MCAC) solid cancers (SC) and those below the age of 5 years were determined
in Latin America and explore the factors that hamper access to using mid-upper arm circumference. Children with haematological can-
care. cers (HC) were graded using the WHO BMI for age index. Children
Methods: A regionwide cross-sectional survey among health care were classified as; mildly-, moderately- or severely-undernourished,
workers from pediatric oncology units in Latin America was con- well-nourished or overnourished.
ducted in March 2022. The self-administered online questionnaire Results: A total of 3608 children between 0-18 years were included in
inquired about the respondent’s perception of these children’s socio- the analysis. The overall prevalence of undernourishment was found to
demographic characteristics, migration-related factors, and access to be 59.4%. However, when stratified according to cancer type, children
cancer treatment. Data analysis was done using descriptive statistics, with SC had a higher prevalence of undernutrition (71.1%) compared
chi-square, and Kruskal-Wallis test. to HC (52.3%). Percentage of mildly-, moderately- and severely-
Results: The majority of the 150 respondents were pediatric oncol- undernourished children suffering from HC was 22.3%, 15.3%, 14.8%
ogists (81%) and pediatricians (6%) from 17 countries. In 2021 and from SC was 18.3%, 20.9%, 31.8%, respectively. The prevalence of
respondents cared for a median of 3.5 MCAC (IQR, 1-9) mainly from well-nourished children was lower in the SC group (26.8%) compared
Venezuela (55%), Bolivia (23%), Nicaragua (17%), Paraguay (12%), and to the HC group (44.0%). Prevalence of overnourished children was
Haiti (11%). Language was an important barrier to access care (17%) higher in the HC group compared to the SC group (3.6% vs 2.1%).There
as only a quarter of the centers offered interpreter services. Twenty- was a statistically significant difference in nutritional status between
three percent of cases reported a history of previous treatment. The SC and HC (p<0.001).
MCAC’s families’ socioeconomic status was perceived as slightly lower Conclusions: More than half the children with cancer that CF sup-
(40%) or much lower (45%) than non-migrant children. Although most ports, across India are undernourished in the induction phase of
respondents (84%) reported that their country guaranteed health chemotherapy. Children with SC are at a higher risk of being severely
coverage to MCACs, only 38% of respondents reported that their coun- undernourished.
tries have national migrant-inclusive health regulations. MCAC usually
required vaccinations (36%), psychosocial (54%), and nutritional sup-
port (38%). Their cancer outcomes were perceived as similar (53%), EP707 / #1376 BRAZILIAN NUTRITIONAL REGISTRY OF
worse (39%), or much worse (8%) than for non-migrant. Respondents CHILDREN WITH CANCER
from countries spending less than 7% of their GDP on health perceived
a significantly higher risk of treatment abandonment (p=0.001), lack of Karina Viani1 , Vicente Odone Filho2 , Mariana Murra3 , Wilson De
health coverage (p=0.001), and difficult access to treatment during the Oliveira Jr3 , Carolina Fernandes4 , Sima Ferman4 , Jullyana Da Rocha
COVID-19 pandemic (p=0.033) for MCAC. Alves5 , Mecneide Mendes5 , Renata Nunes Porto6 , Nádia Dias
Conclusions: A considerable proportion of pediatric oncology Gruezo7 , Isis Magalhães7 , Bruna Lima8 , Carmem Maria Costa Fiori9 ,
providers reported MCAC having difficulties accessing healthcare Mayara Alvarenga10 , Joaquim De Aguirre-Neto10 , Teresa Cardoso
services in Latin America. Countries must work on widespread policies Fonseca11 , Ronald Barr12 , Elena Ladas13
and ensure their implementation to safeguard the well-being of 1 ITACI, Instituto da Criança do HCFMUSP/ University of São Paulo,
migrant children. Hematology-oncology, São Paulo, Brazil; 2 ITACI, Instituto da Criança
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ABSTRACTS S475 of S635

HCFMUSP, Hematology-oncology, São Paulo, Brazil; 3 Hospital de Amor, Gabriela Villanueva1 , Claudia Sampor2 , Julia Palma3 , Milena
Pediatrics, Barretos, Brazil; 4 Instituto Nacional de Câncer, Pediatrics, Rio de Villarroel4 , Diana Valencia5,6 , Oscar Gonzalez Ramella7 , Mercedes
Janeiro, Brazil; 5 Instituto de Medicina Integral Professor Fernando Figueira, Garcia Lombardi8 , María Victoria Sobrero9 , Florencia Moreno10,11 ,
Pediatric Oncology, Recife, Brazil; 6 Hospital Oncológico Infantil Octávio Agustin Dabezies12 , Ligia Fu13 , Sandra Luna-Fineman14,15 , Magdalena
Lobo, Nutrition, Belém, Brazil; 7 Hospital da Criança de Brasília José Alen- Schelotto16 , Rosdali Diaz Coronado17 , Jacqueline Montoya
car, Nutrition, Brasília, Brazil; 8 Hospital do Câncer UOPECCAN, Pediatrics, Vasquez17 , Cecilia Ugaz17 , Eva Lezcano Caceres18 , Wendy Gomez
Cascavel, Brazil; 9 Hospital do Câncer UOPECCAN, Pediatric Oncology, Garcia19 , Victor Cabrera20 , Simone Aguiar21 , Celia Gianotti
Cascavel, Brazil; 10 Santa Casa de Misericórdia de Belo Horizonte, Pedi- Antoneli22 , Pascale Gassant23 , Thelma Velasquez24 , Essy
atric Oncology, Belo Horizonte, Brazil; 11 Santa Casa de Misericórdia de Maradiegue25 , Silvana Luciani26 , Soad Fuentes-Alabi De Aparicio27 ,
Itabuna, Pediatric Oncology, Itabuna, Brazil; 12 McMaster Unversity, Pedi- Andrea Cappellano28 , Yessika Gamboa29 , Lilia Garcia30 , Ivan Maza31 ,
atrics, Hamilton, Canada; 13 Columbia University, Pediatrics, New York City, Natalia Gonzalez32 , Susan Sardinas33 , Katiuska Moreno34 , Guillermo
United States of America Chantada16,35,36 , Liliana Vasquez37,38
1 Hospital Austral, Pediatric Hematology And Oncology, Pilar, Argentina;
Background and Aims: Brazil is composed of five geographically 2 Hospital JP Garrahan, Pediatric Oncology, Buenos Aires, Argentina;
diverse regions regarding population density and socioeconomic 3 Hospital Luis Calvo Mackenna, Pediatric Oncology, Santiago, Chile;
inequalities. There is a lack of high-quality research on nutritional sta- 4 Hospital Luis Calvo Mackenna, Pediatric Oncology, Santiago, Chile, Pedi-
tus of children and adolescents with cancer in Brazil, particularly of atric Oncology, d, Chile; 5 IMAT oncomedica, Pediatric Oncology, Santander,
multicentric studies. A national nutritional registry was designed with Colombia; 6 Hospital Universitario de Santander, Pediatric Oncology, San-
support from IIPAN (International Initiative for Pediatrics and Nutri- tander, Colombia; 7 Hospital Civil de Guadalajara Dr. Juan I Menchaca,
tion) to assess children with cancer from diagnosis through 6 months Pediatric Oncology, Guadalajara, Mexico; 8 Hospital de Niños Ricardo
of treatment. Gutierrez, Pediatric Oncology, Buenos Aires, Argentina; 9 Hospital Ramon
Methods: This is a prospective multicenter study involving 9 pediatric Carrillo, Pediatric Oncology, Bariloche, Argentina; 10 Instituto Nacional
cancer units in Brazil, representing all geographical regions. Children del Cancer, Registry, Buenos Aires, Argentina; 11 Registro Onco-pediatrico
with cancer (0-19 years old) are enrolled at diagnosis and followed- Hospitalario Argentino (ROHA, Hospitalbased Pediatric Cancer Registry
up for the first 6 months of treatment. Information on clinical data from Argentina), Registry, Buenos Aires, Argentina; 12 Pediatric Hemato
including anthropometry, food security, and socioeconomic status are Oncology Honorary Advisor Consultant MUCAM, Pediatric Oncology,
collected at pre-defined timepoints (diagnosis, 3 and 6 months after Montevideo, Uruguay; 13 Hospital Escuela Universitario, Pediatric Oncol-
start of treatment). ogy, Tegucigalpa, Honduras; 14 University of Colorado School of Medicine,
Results: From January 2021 to February 2022, 315 of 619 patients Pediatric Oncology, Aurora, United States of America; 15 Department

registered have completed 6 months of treatment (59% male, median for the Management of Noncommunicable Diseases, Disability, Violence
age at diagnosis 7.2 years). According to region, 6% were from the and Injury Prevention, World Health Organization, Pediatric Oncology,
South, 42% South East, 16% Central West, 20% North, 16% North Geneva, Switzerland; 16 Hospital Pereira Rossell, Pediatric Oncology, Mon-
East. The analysis of nutritional status at diagnosis, defined by mid- tevideo, Uruguay; 17 Instituto Nacional de Enfermedades Neoplásicas,
upper arm circumference (MUAC) z-score, showed 11% undernutrition Pediatric Oncology, Lima, Peru; 18 Hospital Central Instituto de Prevision
and 7% overnutrition; after 6 months of treatment 7% undernutrition Social, Pediatric Oncology, Pediatric Oncology, Asunción, Paraguay; 19 Dr.

and 6% overnutrition (P<0.001). Children under 5 years old (n=128) Robert Reid Cabral Children’s Hospital, Pediatric Oncology, d, Domini-
who were undernourished at 6 months had more antimicrobial days can Republic; 20 Hospital Regional Río Blanco, Pediatric Oncology, Oriz-
per agent in 6 months compared to healthy (117 vs. 37.5, P=0.028) aba, Mexico; 21 IOP/ GRAACC/ UNIFESP, Pediatric Oncology, São Paulo,
and more days on TPN than healthy (P=0.001) and overnourished Brazil; 22 Universidade Nove the Julhio, Pediatric Oncology, São Pablo,
(P=0.005) children. Brazil; 23 Hôpital Saint - Damien, Pediatric Oncology, Port-au-Prince, Haiti;
Conclusions: The Brazilian nutritional registry will provide unprece- 24 Unidad Nacional de Oncología Pediátrica, Pediatric Oncology, Pediatric
dented data on the nutritional status of children with cancer at a Oncology, Guatemala, Guatemala; 25 Ministry of health, Prevention of can-
national level and possible interactions of demographic and socioeco- cer directorate, Ministry, Lima, Peru; 26 Pan American Health Organization,
nomic factors with nutritional and treatment outcomes. These results Pan American Healtnon-communicable Diseases, Washington D.C, United
suggest that undernourished children under 5 years old may have States of America; 27 Hospital Nacional de Ninos Benjamin Bloom, Pediatric
a higher cost of treatment compared to healthy and overnourished Oncology, San Salvador, El Salvador; 28 Instituto de Oncologia Pediátrica -
patients. IOP/GRAACC/UNIFESP, Pediatric Oncology, São Pablo, Brazil; 29 Hospital

Nacional de Niños, Pediatric Oncology, Pediatric Oncology, San José, Costa


Rica; 30 Centro Universitario Contra el Cáncer UANL, Pediatric Oncol-
EP708 / #231 IMPACT OF COVID-19 IN PEDIATRIC ogy, Monterrey, Mexico; 31 Hospital Rebagliati, Pediatric Oncology, Lima,
ONCOLOGY CARE IN LATIN AMERICA DURING THE FIRST Peru; 32 Hospital Militar Nacional de Colombia, Pediatric Oncology, Bogotá,
YEAR OF THE PANDEMIC Colombia; 33 Hospital del Niño Ovidio Aliaga Uria, Pediatric Oncology, La
Paz, Bolivia; 34 Hospital Verdi Cevallos Balda - Hospital especialidades
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S476 of S635 ABSTRACTS

Portoviejo, Pediatric Oncology, Manabi, Ecuador; 35 Hospital Austral, Pedi- leukopenia during 6MP treatment, a key component of curative acute
atric Oncology, Pilar, Argentina; 36 Fundación Perez Scremini, Funda- lymphoblastic leukemia (ALL) therapy. We conducted a retrospective
tion, Montevideo, Uruguay; 37 Pan American Health Organization, Non- analysis examining the frequency of clinically relevant genetic haplo-
communicable Diseases, Washington D.C, United States of America; types in TPMT among patients treated at Texas Children’s Hospital
38 Universidad de San Martín de Porres, Facultad de Medicina, Medicina De (TCH) for ALL.
Precisión, Lima, Peru Methods: We performed TPMT genotyping for patients treated for
ALL at TCH from September 2009 to January 2021 to determine
Background and Aims: The ongoing COVID-19 pandemic strained allele status for 6 haplotypes with evidence of clinical activity in 6MP
medical systems worldwide. We report on the impact on pediatric metabolism. We evaluated the *1 (normal), *1S (synonymous), and
oncology care in Latin American (LATAM) during its first year. *2, *3A, *3B, *3C (pathogenic) haplotypes and classified the patients
Methods: Four cross-sectional surveys were electronically distributed as normal, intermediate, and poor 6MP metabolizers based on diplo-
among pediatric onco-hematologist in April/June/October 2020, and types according to established guidelines. We stratified results by
April/2021 through the Latin American Society of Pediatric Oncology self-identified race and ethnicity and performed Fisher’s exact tests for
(SLAOP) email list and St Jude Global regional partners. independence.
Results: 453 pediatric onco-hematologists from 20 countries Results: Of the 421 patients in the cohort, 392(93.1%) were nor-
responded the first survey with subsequent surveys response rates mal metabolizers, 29(6.9%) were intermediate metabolizers, and none
above 85%. More than 95% of participants reported that treatment were poor metabolizers. Of the 29 intermediate metabolizers, 26
continued without interruption for new and active on-going patients, had the *3A haplotype, 2 had the *3C haplotype, and one was an
though with disruptions in treatment availability. During the first three unknown haplotype due to use of a qualitative assay. Of the 120 non-
surveys, respondents reported suspensions of outpatient procedures Hispanic White patients, 7(5.8%) were intermediate metabolizers. Of
(54.2%), a decrease in oncologic surgeries (43.6%), radiotherapy the 35 Native American patients, 4(11.4%) were intermediate metabo-
(28.4%), stem cell transplants (SCT) (69.3%), and surveillance con- lizers. All 31 non-Hispanic Black patients and 25 Asian/Pacific Islander
sultations (81.2%). Logistic regression analysis showed that at the patients were normal metabolizers. Of the 243 Hispanic patients,
beginning of the first wave, participants from countries with health- 21(8.6%) were intermediate metabolizers. There was no clear asso-
care expenditure below 7% were more likely to report a decrease in ciation between metabolizer status and race (p=0.25) or ethnicity
outpatient procedures (OR:1.84, 95%C:1.19; 2.8), surgeries (OR:3, (p=0.12).
95%CI:1.9; 4.6) and radiotherapy (OR:6, 95%CI:3.5;10.4). Suspension Conclusions: Among our cohort, 7% had a clinically actionable TPMT
of surveillance consultations was higher in countries with COVID-19 metabolizer status. Higher frequencies of intermediate metabolizers
case fatality rates above 2% (OR:3, 95%CI:1.4; 6.2) and SCT suspen- were seen among Native American and Hispanic patients, although the
sions in countries with COVID-19 incidence rate above 100 cases per association did not reach significance. Evaluation of the clinical phe-
100,000 (OR:3.48, 95%CI:1.6; 7.45). Paradoxically, at the beginning notypes of patients with pathogenic haplotypes and their 6MP dosing
of the second wave with COVID-19 cases rising exponentially, most requirements is ongoing.
participants reported improvements in cancer services availability.
Conclusions: Our data show the medium-term collateral effects of the
pandemic on pediatric oncology care in LATAM, which might help delin- E-Poster Topic: AS05.s Survivorship
eate oncology care delivery amid current and future challenges posed
by the pandemic. E-POSTER VIEWING

EP710 / #986 CHARACTERIZATION OF ENDOCRINOPATHIES


EP709 / #1073 FREQUENCY OF CLINICALLY ACTIONABLE IN CHILDREN TREATED FOR MEDULLOBLASTOMAS
TPMT HAPLOTYPES AMONG CHILDREN WITH ACUTE
LYMPHOBLASTIC LEUKEMIA: THE TEXAS CHILDREN’S Nourah Almutlaq1 , Daniel Runco2 , Nadine Haddad1
HOSPITAL EXPERIENCE 1 Indiana University school of medicine, Pediatric Endocrinology, INDI-
ANAPOLIS, United States of America; 2 Riley Hospital for Children, Indiana
Hugh Mair, Mark Zobeck, Melanie Bernhardt, Karen Rabin, Kala University School of Medicine, Pediatrics, Pediatric Hematology/oncology,
Kamdar, Philip Lupo, Michael Scheurer Indianapolis, United States of America
Baylor College of Medicine, Pediatric Hematology/oncology, Houston,
United States of America Background and Aims: Endocrinopathies are common in survivors of
medulloblastoma (MB) especially after radiotherapy (RT). Data com-
Background and Aims: The thiopurine-s-methyltransferase paring outcomes following proton (PRT) and photon radiation therapy
(TPMT) enzyme is responsible for catabolizing thiopurines, such (XRT) are scarce. Even less data exist on the association with var-
as 6-mercaptopurine (6MP), into active and inactive metabolites. Loss- ious chemotherapy regimens for treatment of MB. We aimed to
of-function variants in the TPMT gene increase myelosuppression and characterize endocrinopathies in children with MB, and to examine
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S477 of S635

the association between RT (dose/type) along with chemotherapy Results: The patients were between the ages of 1 and 19, with a
regimens and the development of endocrinopathies. mean of 8.3years. Diagnoses included, ALL(n=4), osteosarcoma(n=3),
Methods: Following IRB approval, charts of children with MB seen at Ewing’s sarcoma(n=3), Wilms’ tumor(n=3), AML(n=3), and DLBCL,
Riley Hospital for Children between 2005 and 2022 were reviewed. Hodgkin’s and Burkitt’s lymphoma(n=1). A total of 79 doses of anthra-
Analysis of patients’ demographics, clinical characteristics, chemother- cyclines (doxorubicin, daunorubicin or idarubicin) were administered.
apy regimen, radiation dose and type, and endocrine sequelae was Baseline echocardiograms at diagnosis were done for all patients, A
done. total of 64 surveillance echocardiograms were done during treatment,
Results: Seventy- eight patients (70.5% male) with MB were identified. a minimum of one scan while on therapy and 23 echocardiograms after
The mean age ± SD at diagnosis was 7.4 ± 3.7 years, and the mean dura- completion of treatment. Out of the 19 patients in this study, 7 were
tion of follow-up was 9.8 ± 5.8 years. Seventy of 78 (89.7%) patients started on cardiac medications (ACE inhibitors, calcium channel block-
received radiation (XRT=25, PRT=45). Endocrinopathies were diag- ers or beta blockers); indications included low LVEF in 2 patients and
nosed in 60 of 78 (76.9%), with growth hormone deficiency being the depressed LV strain, pericardial effusion and hypertension.
most common (44/78, 56.4%). No endocrinopathies were seen in the Conclusions: This pilot project will help in developing a robust sys-
no RT group. Compared to PRT, a higher proportion of patients with tem for anthracycline surveillance. Our data indicates that surveil-
XRT had endocrinopathies (96.0% vs. 80.0%, p = 0.04), central hypothy- lance practices were followed for all new diagnosis and patients on
roidism (68.0 % vs 28.9% P= 0.002) and hypogonadism (40.0% vs chemotherapy. Timely surveillance allowed for immediate identifica-
15.6% P=0.012). In the XRT group, 1 patient developed thyroid nod- tion of compromised cardiac function and initiation of anti-failure
ules and 3 were diagnosed with follicular thyroid cancer. No differences medications. In the LMICs, there is lack of availability of Dexrazox-
were seen in frequency of endocrinopathies for high dose (36-39Gy) ane for cardio protection. Robust surveillance programs allow for
versus standard (23Gy) RT dose, or the chemotherapy regimen. prevention of anthracycline associated cardiotoxicity.
Conclusions: We found a higher frequency of endocrinopathies in
children treated with XRT versus PRT. Similarly, thyroid nodules and
cancers were more common with XRT. While no difference found EP712 / #2012 ANALYSIS OF SURVIVAL IN PATIENTS WITH
in the prevalence of endocrinopathies between those who received ACUTE LYMPHOBLASTIC LEUKEMIA TREATED AT HOSPITAL
high dose vs standard dose RT, RT remains the major risk factor for DEL NIÑO OVIDIO ALIAGA URIA, MANAGEMENT 2014-2019 LA
endocrinopathies in MB highlighting need for long term monitoring. PAZ, BOLIVIA

Soraya Marianela Arancibia Romero, Susan Sardinas


EP711 / #1301 AN AUDIT OF CARDIAC SURVEILLANCE Hospital del Niño Ovidio Aliaga Uria, Pediatric Oncology, La Paz, Bolivia
PRACTICES IN PEDIATRIC ONCOLOGY PATIENTS FOR EARLY
IDENTIFICATION OF ANTHRACYCLINE ASSOCIATED Background and Aims: Leukemia represents the main hematological
CARDIOTOXICITY - A PILOT STUDY malignancy in pediatrics. The incidence registered in the world context
is 20 to 35 annual cases per million inhabitants. In developing coun-
Ayesha Arshad Ali1 , Saleem Sadqani2 , Nadia Ayoub3 , Naureen tries, survival curves oscillate between 60 and 70%, even when the
Mushtaq1 , Asim Belgaumi1 , Zehra Fadoo1 , Sadaf Altaf1 same original protocols applied in cancer centers in developed coun-
1 Aga Khan University, Dept Of Oncology, Aku, karachi, Pakistan; 2 Aga Khan tries are reproduced; Considering that statistical data is a powerful
University, Pediatrics, karachi, Pakistan; 3 Aga Khan University, Pharmacy, tool in decision making, the following objective was set: Estimate the
karachi, Pakistan survival rate in pediatric patients diagnosed with acute lymphoblastic
leukemia treated at the Ovidio Aliaga Uría Children’s Hospital for the
Background and Aims: Anthracyclines can cause serious acute and 2014-2019 period.
chronic cardiovascular side effects. Regular echocardiograms based on Methods: It is a non-experimental, retrospective, analytical work, of
the treatment regimen for early detection and modification of therapy a predictive research level, descriptive tools were applied for numeri-
are recommended for early detection. The audit was designed to assess cal and categorical variables, in addition to Kaplan and Meyer actuarial
compliance in establishing cardiac status at diagnosis and monitoring survival analysis methods; the total number of patients diagnosed with
of cardiac function during and after therapy. ALL in the study period was 147; 122 were selected by means of inclu-
Methods: A retrospective review was carried out to determine sion and exclusion criteria, of which 30 correspond to patients who
baseline cardiac surveillance clinical practice in pediatric oncology completed treatment and began surveillance, on whom the survival
unit at Aga Khan University Hospital between January and June analysis was carried out.
2020, and 19 patients were randomly selected. The online medi- Results: The median survival was 48 months, the cumulative disease-
cal record system was utilized to search their diagnoses, anthracy- free survival was 92% at 5 years and 100% in the first 3 years, since
cline dosages, and surveillance echocardiogram findings. These were there was only one isolated event of death at 42 months of follow-up.
recorded in separate forms on REDCap and analysed to assess Having an average surveillance time of 38 months (3.1 years). The mean
compliance. age of the children under surveillance was 68 months, the relapse event
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S478 of S635 ABSTRACTS

occurred in 3 patients, 1 died and 2 abandoned treatment. The dropout Conclusions: long term NHL survivors, pulmonary and cardiac func-
rate in the study period was 26%, as was the mortality rate, 26%, of tions should be followed up for possible affection. Lipid profiles and
which half died due to causes inherent to the treatment, the majority in weight affection should be monitored closely. Survivorship program for
the first months, the induction phase, and the rest died due to activity NHL is recommended
of the treatment. illness and abandonment.
Conclusions: Median overall survival is 54 months. And the median
disease-free survival is 52 months EP714 / #789 OBESITY AND METABOLIC SYNDROME IN
CHILDHOOD CANCER SURVIVORS – AN INSTITUTIONAL
COHORT ANALYSIS
EP713 / #1831 LONG TERM PULMONARY AND CARDIAC
FUNCTION AFFECTION AMONG NON-HODGKIN LYMPHOMA Swetambri Swetambri1 , Shuvadeep Ganguly2 , Sameer Bakhshi2 ,
SURVIOURS Deepam Pushpam3 , Devanshi Kalra4
1 All India Institute of Medical Sciences, Medical Oncology, New Delhi,
Azza Ayad1 , Asmaa Hamoda2 , Reham Khedr3 , Nahla Elnabarawy4 , India; 2 All India Institue of Medical Sciences, Medical Oncology, New Delhi,
Elhamy Rifky4 , Tamer Diab5 , Leslie Lehmann6 , Alaa El-Haddad7 India; 3 AIIMS, New Delhi, Medical Oncology, Delhi, India; 4 All India Insti-
1 Cancer Children’s Hospital of Egypt CCHE 57357, Pediatric Oncology, tute of Medical Sciences, Department Of Medical Oncology, New Delhi,
Cairo, Egypt; 2 National Cancer Institute - Cairo University, Children’s Cancer India
hospital Egypt 57357, Pediatric Oncology, cairo, Egypt; 3 National Cancer
Institute, Cairo University and Children’s Cancer Hospital Egypt, Egypt, Pedi- Background and Aims: Childhood cancer survivors (CCS) have
atric Oncology, cairo, Egypt; 4 Children’s Cancer hospital Egypt 57357, Can- increased risk of adiposity, metabolic syndrome, diabetes and dys-
cer Survivorship, Cairo, Egypt; 5 Children’s Cancer hospital Egypt 57357, lipidaemia. We analysed the prevalence of obesity, overweight,
Clinical Psychology, Cairo, Egypt; 6 Dana-Farber cancer Institute, Boston abnormal triglycerides (TG), high-density cholesterol (HDL-C)
Children’s Hospital, Boston, United States of America; 7 National Can- and impaired fasting glucose (FPG) in an institutional cohort of
cer Institute - Cairo University, Children’s Cancer hospital Egypt 57357, CCS.
Pediatric Oncology, Cairo, Egypt Methods: CCS (diagnosed ≤21 years), currently ≥ 5 years of age were
included from December 2020 to February 2022. Weight, height, waist
Background and Aims: The survival outcomes of mature B-cell non- circumference (WC) and hip circumference (HC) were measured. FPG
Hodgkin lymphoma (NHL) patients have improved due to advances in and lipid profile were also advised ≥10 years of age. For survivors
treatment. In this study we aim to assess the frequency and trends of ≥16 years, body mass index (BMI) ≥23 and ≥27 were defined as over-
the late effects and their impact on quality of life among these survivors weight and obesity respectively; WC of >90cm (males) and >80cm
at Children Cancer hospital Egypt. (females) or WC:HC ratio (WHR) ≥0.90 (males) and ≥0.85 (females)
Methods: A retrospective study including all patients diagnosed were defined as central adiposity as per WHO Asian recommendations.
with Non-Hodgkin’s lymphoma “Mature B” at CCHE 57357 in the For 5-<16 years, adult BMI of 23 and 27 as per Indian Academy of Pedi-
period between January 2012 till December 2015. All of the patients atrics (IAP) BMI chart were defined as overweight and obesity respec-
were examined for: Cognitive functions (by assessing IQ level using tively and WC≥90th percentile as per IAP-WC chart was considered as
(Stanford-Binet Intelligence Scales, Fifth Edition ‘’SB5’’), Cardiac func- central adiposity. FPG≥100mg/dl, TG≥150mg/dl and HDL-C<40mg/dl
tion, Endocrine (Thyroid functions, Growth curves), Pulmonary (pul- were considered as abnormal as per International Diabetes Federation
monary function test), 5. Quality of life assessment (PedsQL) and Lipid Recommendation.
profile (triglyceride, cholesterol, HDL, LDL) Results: Total 367 survivors were included with median age of 16 years
Results: A total of 345 patients were enrolled, 213 were evaluable with (77.4% males; 61.1% haematological malignancies). Median duration
a male: female ratio 4:1. Staging: stage I (7.5%), II (14.1%), III (58.7%), from treatment completion was 5 years (4 months-19.5 years). The
IV (19.7%). The total mean QoL score 99 ± 0.058, the order of the prevalence of obesity in the cohort was 11.1% (41/367), while another
affected domains, according to severity: physical functioning 92 ± 0.1, 71 survivors (19.3%) were overweight. Central adiposity based on WC
emotional functioning 92 ± 0.15, followed by social functioning 97 ± and/or WHR was observed in 31.3% (115/367) survivors. Total 79
0.08, school functioning 99 ± 0.06. IQ scores (low average 24.4%, high and 82 survivors underwent lipid profile and FPG estimation. Abnor-
average 10.3%, average 63.8%). Pulmonary functions test was eval- mal TG, HDL-C and impaired FPG were observed in 16/79 (20.2%),
uated in 123 patients with affection detected in (57.7%) as follows: 24/79 (30.3%) and 7/82 (8.5%) survivors respectively. Baseline type
(mild degree 34.2%, moderate 14%, sever 9.3%). Cardiac reported of malignancy, duration from treatment completion, and current age
with early onset 22% (from which 63% resolved, 36% persistent), of CCS did not predict development of obesity/ overweight metabolic
late onset 10.3% . Lipid profile was significantly affected (high triglyc- abnormalities.
erides 21.6%, low HDL 16%, high LDL 8.9% and high total cholesterol Conclusions: Obesity/overweight, dyslipidaemia, and impaired FPG
41.8%)while Growth affection: regarding weight (25.4% dropped 2 or are common in CCS in developing country and needs regular multi-
more centiles, height (33.3% dropped 2 or more centiles) disciplinary follow up.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S479 of S635

EP715 / #149 SELF-PERCEIVED HEARING HANDICAP AND EP716 / #383 PREDICTION OF VENTILATORY THRESHOLD
SOCIAL AND BEHAVIORAL OUTCOMES IN ADULT CHILDHOOD WITH A 6-MINUTE WALK TEST IN SURVIVORS OF ACUTE
CANCER SURVIVORS WITH TREATMENT-INDUCED HEARING LYMPHOBLASTIC LEUKAEMIA
LOSS
Émilie Bertrand1,2 , Maxime Caru2 , Valerie Lemay2 , Sophia Morel2 ,
Johnnie Bass1 , Fang Wang2 , Mackenzie Thaxton3 , Sarah Warren4 , Valerie Marcil2 , Caroline Laverdière2 , Maja Krajinovic2 , Daniel
Deokumar Srivastava5 , Leslie Robison2 , Melissa Hudson6 , Kirsten Sinnett2 , Daniel Curnier1
Ness2 , Tara Brinkman2 1 University of Montreal, Faculty Of Medecine, Montreal, Canada; 2 Sainte-
1 ST. JUDE CHILDREN’S RESEARCH HOSPITAL, Rehabilitation Services, Justine University Health Center, Research Center, Montreal, Canada
Memphis, United States of America; 2 ST. JUDE CHILDREN’S RESEARCH,
Epidemiology And Cancer Control, Memphis, United States of America; Background and Aims: Exercise is beneficial for cancer patients. Mea-
3 Memphis Audiology, Audiology, Collierville, United States of America; surement of oxygen consumption and identification of the heart rate
4 University of Memphis, School Of Communication Sciences And Disorders, (HR) at ventilatory threshold remain the gold standard for exercise
Memphis, United States of America; 5 ST. JUDE CHILDREN’S RESEARCH, stress test. However, access to this technology is limited. The 6-Minute
Biostatistics, Memphis, United States of America; 6 St. Jude Children’s Walk Test (6MWT) is a valid and safe field test for assessing aerobic
Research Hospital, Oncology, Memphis, United States of America capacity in survivors. The aim is to validate a specific 6MWT equation
to predict HR at ventilatory threshold.
Background and Aims: Hearing loss (HL) is a prevalent adverse effect Methods: Paediatric ALL survivors (n=154) completed a 6MWT and
in childhood cancer survivors treated with ototoxic therapy that may an incremental cardiorespiratory exercise test on an ergogycle with
negatively impact quality of life. Self-perceived hearing handicap in gas exchange analysis. Participants were randomized into 2 groups
the general adult population is a stronger predictor of reduced qual- to predict the equation (n=107 (70%)) and to validate the equation
ity of life than measured HL alone. Self-perceived hearing handicap (n=47 (30%)). Backward linear regression analyses were used to deter-
and its association with social and behavioral outcomes have not been mine the prediction equation for HR at ventilatory threshold from the
examined in childhood cancer survivors. 6MWT. The root mean square error (RMSE) and the Bland and Alt-
Methods: Adult survivors previously treated with platinum and/or man method were used to measure the accuracy of the predicted HR
head and neck radiotherapy with HL in at least one ear documented at ventilatory threshold on the validation group.
by audiological evaluation were recruited (N=364). Two hundred Results: The equation determined is [HR ventilatory threshold =
thirty-seven survivors (median[range] age at survey 37.0[30.0-45.0] (0.074 x age) + (0.218 x HR end 6MWT) + (0.016 x cumulative dox-
years, 29.1[22.4-35.0] years since diagnosis, 4.0[2.9-7.7] years from orubicin dose) – (0.051 x height) – (0.835 x years since the end
last audiogram to survey) completed the Hearing Handicap Inven- of treatment) – (0.115 x physical activity level) + (0.010 x distance
tory for Adults (total score: 0-16=no handicap, 18-42=mild-moderate 6MWT) + (0.142 x HR rest) – (0.492 x rating of perceived exertion) +
handicap, ≥44=significant handicap) and questionnaires on social and 126.79] (p=0.001, R2 =0.271). The resulting RMSE was 14.5 bpm. Vali-
behavioral outcomes. Survivors were grouped by HL severity accord- dation with the Bland and Altman method showed that more than 95%
ing to Chang ototoxicity criteria: grades 1a-2a=mild-moderate HL of the values fall between the limits of agreement (-28.7 to 28.8) and
vs. grades ≥2b=severe HL. Multivariable logistic regression models the mean bias was 0.085 bpm.
examined associations between HL, hearing handicap, and social and Conclusions: The equation with 6MWT data and disease-specific
behavioral outcomes with adjustment for sex, race, age at HL, and age variables could predict an individualized training level for ALL
at survey. survivors. This study reinforces the utility of assessing the func-
Results: Among 237 survivors, 81 (34.2%) had mild-moderate HL and tional capacity of patients with a 6MWT to propose an individu-
156 (65.8%) had severe HL. Severe HL was associated with increased alized training programme at ventilatory threshold based on their
likelihood for mild-moderate hearing handicap (odds ratio [OR]=3.40, abilities.
95% confidence interval [CI] 1.61-7.18) or significant hearing hand-
icap (OR=7.41, CI 3.14-17.51). Self-perceived hearing handicap was
associated with increased likelihood for social isolation (OR=2.79, CI EP717 / #1936 LONG-TERM NEUROPSYCHOLOGY DEFICITS
1.52-5.10), depression (OR=4.95, CI 1.98-12.35), anxiety (OR=11.25, IN PEDIATRIC CNS TUMOR: REVIEW OF 20 YEARS OF
CI 2.51-50.47), somatization (OR=2.85, CI 1.28-6.37), and reduced EXPERIENCE
personal income (OR=2.22, CI 1.23-4.00).
Conclusions: Severe HL following ototoxic therapy is associated Cristina Boix1 , Laura Mangado1 , Paula Marcote Sinclair2 , Ofelia
with self-perceived hearing handicap. Survivors who reported hear- Martinez3 , Federico Ramos4
ing handicap are at increased risk for adverse social and behavioral 1 Hospital Sant Joan de Deu, Neurology, Barcelona, Spain; 2 Sant Joan de Déu

outcomes. Assessment of hearing handicap may facilitate access to Hospital, Oncology, Esplugues, Spain; 3 Sant Joan de Déu, Oncology, Sant
targeted recommendations and interventions in adult survivors with Joan d’Espí, Spain; 4 Sant Joan de Déu Hospital, Neurology, Esplugues, Spain
HL.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S480 of S635 ABSTRACTS

Background and Aims: Childhood cancer is the second leading cause nificant decrease in post-intervention anxiety and depression, i.e., in
of death. Of all neoplasms, Central Nervous System (CNS) tumors HADS-anxiety (P<0.01), HADS-depression (P=0.04), STAI trait anxiety
account for 30% of cases. Posterior Fossa Tumors (TFP) are the most (STAI-T, P=0.01). State anxiety showed a decrease tendency (STAI-S,
prevalent and account for about 50% of all tumors. Medical advances P=0.07). Program involvement also resulted a non-significant increase
have allowed survival to be around 80% after 5 years. Two-thirds of in sense of coherence (P=0.12). The program was generally evaluated
these children will experience some kind of long-term sequelae. 60% positively as regards quality, extent, degree to which it had met needs,
of survivors will be at risk for some form of neurocognitive impairment. and overall usefulness of the program.
Methods: The groups of populations studied are analyzed according to Conclusions: Time-limited intensive PSR intervention is helpful, and
the location of the tumor and the diagnosed neuropsychological pro- meets needs of help in adjusting to post-treatment life after recent can-
files. Material and Method. Retrospective review of children assessed cer. Intensive face-to-face group rehabilitation intervention can reduce
between 2001 and 2021. A total of 151 patients were reviewed and emotional burden, and endorse sense meaningfulness. Findings justify
analyzed. a larger implementation study to further examine the strengths of this
Results: The localitzation was in a 35% supratentorial versus 65% rehabilitation model, and usefulness in general clinical after-care.
infratentorial. Only 2% of the total sample had no neurocognitive
deficits, whereas 16% had an intellectual disability and the rest a wide
range of neurocognitive focal deficits. All of teh had a scholar support EP719 / #706 CANCER CARE FOR CHILDREN,
amb more than a half repeat course. The group of infratentorial tumors, ADOLESCENTS AND YOUNG ADULTS AND THEIR PATHS TO
teh 75% of children had long-term characteristics typical of Cognitive DIAGNOSIS: AN IRISH EXPERIENCE
Affective Cerebellar Syndrome (SCCA).
Conclusions: The need for specialized neuropsychological assessments Elysha Brennan1 , Niamh O Sullivan1,2 , Peter Mccarthy1,3 , Owen
in the follow-up of these children is demonstrated in order to be able to Smith1,3 , Scheryll Alken1,2
favor adequate school and adaptive achievements. 1 Childrens Health Ireland at Crumlin, Oncology, Dublin, Ireland; 2 St James
Hospital, Oncology, Dublin, Ireland; 3 University College Dublin, School Of
Medicine, Dublin, Ireland
EP718 / #1704 MANAGING THE AFTERMATH OF RECENT
CANCER: A STUDY INVESTIGATING AND EVALUATING A Background and Aims: Improving survival rates of childhood and ado-
MODEL FOR SUPERVISED INTENSIVE GROUP INTERVENTION lescent cancer has meant survivorship, and long-term follow-up and
FOR PSYCHOSOCIAL REHABILITATION care for these patients has been at the forefront of focus in recent
years. There is a paucity of literature examining the experiences of
Krister Boman, Lina Hörnquist, Amelie Björlin, Martin Alskog young people (YP) with cancer in Ireland. We aimed to explore the Irish
Uppsala University, Department Of Women’s And Children’s Health, Upp- experience of cancer care for this group
sala, Sweden Methods: Data was collected via anonymous online survey. Inclusion
criteria were being over 16 years of age and having been diagnosed
Background and Aims: Following diagnosis, young, young adult and with cancer between 0 and 26 years. Data including demographics,
adult cancer patients face dealing with the unwanted consequences path to diagnosis, diagnosis and treatment was collected.
and the challenges of managing post-treatment life. Rehabilitation Results: Sixty-one surveys were completed. Disease types reflected
after the illness and the anticancer treatment has been established as incidence patterns nationally. In the 0–14-year cohort, the common-
a vital part of treatment and surveillance. The aim of this study was est diagnosis was leukaemia (35.7%, n=10), solid tumour (28.6%, n=8)
to implement and evaluate an intensive supervised group model for and CNS tumour (21.4%, n=6). Lymphoma was the commonest diag-
psychosocial rehabilitation (PSR). nosis in the 15–26-year age group (51.5%, n=17 vs 10.7%, n=3 in
Methods: This pre- and post-assessment intervention study included 0-14yo) (p= 0.009). Most patients (85.2%) attended a general practi-
patients diagnosed with cancer, and for whom treatment was in the tioner (GP) prior to diagnosis, with 21.2% reporting >3 GP visits. A
final stage or completed, and who were >3 months from diagnosis. The statistically significant difference was noted between the groups as
PSR intensive intervention was manual-guided, supervised by experi- to who informed YP they had cancer; 76% of over 15s were told by
enced qualified leader, and offered two weekly sessions over 10 weeks. healthcare professionals compared with 36% of 0-14yo (p=0.16). Par-
The effect of the PSR was assessed pre- and post-intervention regard- ticipants were invited to leave comments with one respondent quoting
ing anxiety and depression using the Hospital Anxiety Depression Scale “Nobody properly told me I just figured it out from hearing doctors talk
(HADS), the Spielberger State-Trait Anxiety Inventory (STAI), and sense around me”.
of coherence using the Sense of Coherence Scale. Participants were Conclusions: Multiple visits to GPs reflect data from international
enquired about their subjective experience of the value, usefulness, studies, demonstrating a change in healthcare utilisation prior to a
and efficiency of the PSR program. cancer diagnosis. Differences in communication around diagnosis are
Results: Nine out of 11 patients completed the intensive program. somewhat expected but we must endeavour to include children and
A positive effect of involvement in the PSR was reflected in a sig- young people in discussions about their health. Children and YP with
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S481 of S635

cancer require their unique psychosocial needs to be met with age- EP721 / #1740 LONG-TERM SIDE EFFECTS OF CHILDHOOD
appropriate communication styles and provision of information which CANCER. WHAT ARE PARENTS WORRIES DURING
needs to be prioritised by healthcare professionals. No one should TREATMENT?
overhear their cancer diagnosis.
Mónica Camacho Arias1 , Belén Huguet Rodriguez1 , Carmen Garcia
Baeza1 , Miriam Ruiz Antón1 , Nerea Domínguez2
EP720 / #710 NAMED PATIENT PROGRAM USE OF 1 Hospital 12 de octubre, Oncology, Madrid, Spain; 2 Hospital Universitario
PEDMARK™ TO REDUCE THE RISK OF CISPLATIN-INDUCED Doce de Octubre, Research institute i+12, Pediatrics And Haemato-
OTOTOXICITY IN PEDIATRIC PATIENTS WITH LOCALIZED oncology Service, Madrid, Spain
SOLID TUMORS
Background and Aims: Most survivors of childhood cancer develop
Penelope Brock1 , Giuseppe Barone1 , Heidrun Boztug2 , Makiko Mori3 , long-term side effects due to their cancer treatment or their dis-
Michael Ortiz4 , Ananya Bhattacharya5 , Allison Morgan5 , Edwina ease.These can negatively affect their quality of life. Our objective
Baskin-Bey5 , Donna Mcvey5 is to investigate worries and needs about this E-Poster Topic of
1 Great Ormond Street Hospital for Children NHS Foundation Trust, Paedi- parents of children receiving cancer treatment in our unit,as well
atric Oncology, London, United Kingdom; 2 St Anna Kinderspital, Paediatric as determine how much information about long-term care they
Oncology, Vienna, Austria; 3 Saitama Children’s Medical Center, Haematol- have.
ogy Oncology, Saitama, Japan; 4 Memorial Sloan Kettering Cancer Center, Methods: We performed a 19-question survey about parent’s expe-
Pediatrics, New York, United States of America; 5 Fennec Pharmaceuticals, riences with late effects communication,general worry about late
Clinical, North Carolina, United States of America effects,and specific late effect worries.
Results: 32 parents completed the survey(71.9% female).34.4%
Background and Aims: Background: Cisplatin induced-otoxicity (CIO), had University graduate,21.9% Professional graduate and 37.5%
with permanent hearing loss is observed in 60%-90% of pedi- had Middle School graduate.Children diagnosis were 43.8%
atric patients treated. PEDMARKTM (sodium thiosulfate anhydrous) leukemia/lymphoma,31.3% extracranial solid tumor and 25% CNS
reduced the proportion of patients with hearing loss by half as tumor. 96.9% of children were receiving chemotherapy,21.9% radio-
observed in two Phase 3 studies (SIOPEL 6 & COG ACCL0431). Aim: therapy and in 34.4% surgery was performed. 84.4% thought their
Evaluate the safety of the investigational product (PEDMARKTM ) pro- children would be cured of their cancer. 62.5% of parents referred
vided to pediatric patients via a multi-national Named Patient Program they were quite or very worried about long-term side effects.They
(NPP). were especially worried about cardiotoxicity and second malignan-
Methods: Patients (1 month to <18 years) with a localized solid cies(87.5%),followed by neurocognitive impairment(78.2%),growth
tumor, treatment plans including a cisplatin-containing regimen, and impairment(62.5%) and infertility(59.4%).75% of parents had received
cisplatin infusion time ≤6 hours were eligible. PEDMARKTM is adminis- information during treatment about long-term side effects, but 65.6%
tered intravenously 6 hours after each cisplatin infusion. Demography, said they would have preferred to be better informed, and only 56.3%
tumor type, and adverse events (AEs) were recorded from APR2018 - felt prepared to manage those effects. However,68.8% of parents
DEC2021. thought long-term side effects should not influence cancer treatment
Results: The NPP has been active for 3.6 years as of the data at all, as cure of their children was the main objective.All parents said
cut-off (31DEC2021). Forty-six hospitals in 14 countries requested they would like to receive more information about long-term side
PEDMARKTM , totaling 106 case requests, of which 105 were fulfilled. effects at the end of treatment and all them thought long-term care
The UK encompassed 35% of hospitals and 41% of case requests. The and follow up is necessary.
age range was 1 month to 20 years. Most frequent age ranges were 12- Conclusions: Most parents of children with cancer showed concerns
18 months and 2-3 years (17% each), 1-6 months and 18-24 months about long-term side effects and demand information from the begin-
(13% each), and 3-4 years (12%), respectively. Median weight was 12 ning of treatment.However,during treatment, they think cure is above
kg. Localized tumors included hepatoblastoma (HB) in 90% of patients, sequelae.Early information about long-term toxicity during treatment
of those with known staging, 55% were HB Pretext III/IV. Most fre- might help parents to solve worries,thoughts and doubts of families of
quent ages for patients with HB pretext III/IV was 1-2 years (20%) and children with cancer.
under 1-year (14%). Other tumors included medulloblastoma (n=4);
nasopharyngeal carcinoma (n=3); glioblastoma (n=1), atypical tera-
toid/ rhabdoid tumor (n=1); and osteosarcoma (n=1). One SAE has EP722 / #5 ORGANIC AND STRONG: TEAM BUILDING OF
been reported, a serious AE of Grade 1 metabolic acidosis, which was HIGH SCHOOL TEACHERS ADVOCATING FOR A STUDENT
reversible and did not lead to PEDMARKTM discontinuation. RETURNING TO SCHOOL AFTER TREATMENT FOR
Conclusions: PEDMARKTM was supplied multi-nationally via a NPP to OSTEOSARCOMA
reduce the risk of CIO in pediatric patients of varied ages, tumor types,
and staging, with minimal safety findings. The NPP is ongoing. Chen-Chen Cheng
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S482 of S635 ABSTRACTS

National Kaohsiung Normal University, Special Education, Kaohsiung, Tai- Utrecht, Wilhelmina Children’s Hospital, Pediatrics, Utrecht, Netherlands;
wan 6 Amsterdam UMC location University of Amsterdam, Obstetrics And
Gynaecology, Amsterdam, Netherlands; 7 Amsterdam UMC location Vrije
Background and Aims: When adolescents return to school after Universiteit Amsterdam, Pediatrics, Amsterdam, Netherlands; 8 Dutch

treatment for osteosarcoma of lower extremities, they experience a Childhood Oncology Group, Childhood Oncology, Utrecht, Netherlands;
temporary physical disability before they can walk on their own. With 9 Radboud University Medical Center, Hematology, Nijmegen, Nether-
significant bodily changes, they are faced with teachers’ and peers’ lands; 10 Carl von Ossietzky University of Oldenburg, Faculty of Medicin
questions or misunderstandings regarding their movement, cancer, and and Health Sciences, Health Services Research, Oldenburg, Germany;
health. To add to the body of knowledge of childhood cancer survivors’ 11 Radboud University Medical Center, Obstetrics And Gynaecology,
school reentry and self-advocacy, this qualitative study explores how Nijmegen, Netherlands
three high school teachers joined forces with the school nurse in organ-
ically developing a successful team to advocate for and empower their Background and Aims: Gonadal damage leading to fertility prob-
student with osteosarcoma. lems is a frequently encountered late effect in childhood cancer
Methods: A part of a larger qualitative project of teachers’ experiences survivors (CCS). This study evaluated the desire for children and use
serving students receiving cancer treatment in Taiwan, this study is of reproductive health care among male CCS in comparison to male
focused on how a homeroom teacher, a school counselor, and a desig- siblings.
nated mentor worked together to support a Grade 9 student returning Methods: A nationwide cohort study was conducted as part of the
to school after biological reconstruction. Each teacher was interviewed Dutch Childhood Cancer Survivor LATER study part 1; questionnaire
individually 90-120 minutes. The interview transcripts were analyzed & linkage study. A questionnaire addressing desire for children, repro-
using a thematic analytical method. ductive health care and reproductive outcomes was completed by
Results: The findings were: 1. The organically formed student sup- 1,317 male CCS and 407 male sibling controls.
port team was successful. With students’ best interest in mind and Results: After adjustment for age at assessment, the percentage of
given teachers’ different professional roles, they focused on what they men who had an overall (previous, current or future) desire for chil-
could do for the student and his family in their own capacity. They dren was significantly lower in male CCS compared to the male siblings
trusted and relied on one another to perform their individual task. 2. (74% and 82%, respectively; OR, 0.61; 95% CI, 0.46 to 0.82; P = 0.001).
As the student’s single father was busy working to support the family, The association between survivorship status and desire for children
teachers shared a joint parenting role. 3. Teachers trained the student was attenuated after adjustment marital status, level of education and
to self-advocate by guiding him to ask physicians relevant questions, employment status (OR, 0.83: 95% CI, 0.61 to 1.14; P = 0.250). A pre-
participate in school events, and speak about his cancer experience vious or current desire for children was reported by 491 CCS and 185
in public. 4. The school nurse (not interviewed) checked student’s siblings. Of these CCS, 34% consulted a reproductive specialist com-
wound daily and promptly communicated the student’s needs with the pared to 12% of the siblings (P < 0.001). The percentage of CCS who
teachers. used assisted reproductive techniques (ART) after consulting a repro-
Conclusions: Given the diverse situations of students treated for can- ductive specialist was lower compared to the siblings (41% and 77%,
cer, teachers need to learn about the students and their family situation respectively; P < 0.05). Also, the percentage of men who fathered a
to know what is the best for them. child after ART was lower in CCS compared to siblings (49% and 94%,
respectively; P < 0.05).
Conclusions: The majority of male CCS have a desire for children. More
EP723 / #945 DESIRE FOR CHILDREN AND USE OF survivors consult a reproductive specialist, both the utilization of and
REPRODUCTIVE HEALTH CARE AMONG MALE SURVIVORS OF pregnancy rates after ART are lower compared to their siblings. This
CHILDHOOD CANCER: A DCCSS-LATER 1 STUDY insight is important for counseling of CCS regarding family planning
and fertility issues.
Joyce Claessens1 , Adriaan Penson1 , Ewald Bronkhorst2 , Leontien
Kremer3,4,5 , Eline Van Dulmen-Den Broeder6,7 , Margriet Van Der
Heiden-Van Der Loo3,8 , Wim Tissing3 , Helena Van Der Pal3 , Nicole EP724 / #445 METABOLIC SYNDROME IN CHILDHOOD
Blijlevens9 , Marry Van Den Heuvel-Eibrink3 , Birgitta Versluys3 , ACUTE LYMPHOBLASTIC LEUKEMIA/LYMPHOMA SURVIVORS:
Dorine Bresters3 , Cecile Ronckers3,10 , Iris Walraven2 , Catharina A GLOBAL CAUSE FOR CONCERN
Beerendonk11 , Jacqueline Loonen1
1 Radboud University Medical Center, Center Of Expertise For Cancer Gargi Das1 , Rachna Seth1 , Lakshmy Ramakrishnan2 , Manisha Jana3 ,
Survivorship, Hematology, Nijmegen, Netherlands; 2 Radboud University Vandana Jain4 , Aditya Gupta1 , Jagdish Prasad Meena1
Medical Center, Health Evidence, Nijmegen, Netherlands; 3 Princess Máxima 1 All India Institute of Medical Sciences- New Delhi, Division Of Pediatric
Center for Pediatric Oncology, Pediatric Oncology, Utrecht, Netherlands; Oncology, Department Of Pediatrics, New Delhi, India; 2 All India Insti-
4 Emma Children’s Hospital, Amsterdam UMC, University of Amster- tute of Medical Sciences- New Delhi, Department Of Cardiac Biochemistry,
dam, Pediatrics, Amsterdam, Netherlands; 5 University Medical Center New Delhi, India; 3 All India Institute of Medical Sciences, Department Of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S483 of S635

Radiodiagnosis, New Delhi, India; 4 All India Institute of Medical Science- Division Of Pediatric Endocrinology, Department Of Pediatrics, New Delhi,
New Delhi, Division Of Pediatric Endocrinology, Department Of Pediatrics, India
New Delhi, India
Background and Aims: Serum adipokines (leptin and adiponectin) are
Background and Aims: Metabolic syndrome, obesity, and insulin dysregulated before development of Metabolic syndrome, an impor-
resistance are reported late effects in survivors of childhood Acute tant late effect in childhood Acute Lymphoblastic Leukemia/Lymphoma
lymphoblastic Leukemia (cALL). Our objective was to evaluate the (cALL) survivors. We compared serum levels of adipokines in cALL
prevalence of metabolic syndrome and its components in survivors of survivors and controls, and further studied their role in prediction of
cALL, and to assess predictors for its development. metabolic syndrome in our cohort of survivors.
Methods: Between January-2020 and November-2021, 65 cALL sur- Methods: Forty cALL survivors (aged:10-18 years, and at least 2 years
vivors, aged 7-18 years, and at least 2 years from treatment comple- from treatment completion) and 40 controls, similar for age, sex, body
tion, were enrolled. Relevant clinical details were recorded, and testing mass index, and Tanner stage, were enrolled. Relevant clinical and
for fasting blood sugar, insulin, and lipid profile were done. Body com- treatment details were noted. Body composition by Dual-Energy Xray
position was measured by a Dual-Energy Xray Absorptiometry (DXA) Absorptiometry (DXA) scan, fasting blood sugar, insulin, lipid profile,
scan. and adipokines were evaluated.
Results: The mean (SD) age was 12.7 (±3.2) years and median [IQR] Results: Compared to controls, cALL survivors had higher prevalence
time since diagnosis was 6.5 [5.9;8] years. Majority (74%) of our sur- of metabolic syndrome (8/40 vs 2/40, p=0.042), central obesity (11/40
vivors were male. Primary diagnosis was B cell ALL in most survivors vs 4/40, p=0.042), and hypertension (9/40 vs 2/40, p value=0.024),
(55/65, 85%), remaining were T cell ALL/Lymphoma. A large proportion and had higher median LDL (78.5mg/dL vs 59mg/dL, p=0.008), VLDL
of patients (43/65, 66%) received prophylactic cranial radiotherapy. (20.5mg/dL vs 16mg/dL, p=0.020) and triglyceride levels (102mg/dL
Central obesity was seen in 14/65 (21.5%) children, with 30.8% being vs 80.5mg/dL, p=0.014). Though not statistically different, trends of
overweight/obese. Sarcopenic obesity was seen in 21/65 (33.9%) sur- Leptin (7.39 vs 4.23ng/mL, p=0.207) and derived Leptin-Adiponectin
vivors. Eleven survivors (16.9%) had metabolic syndrome, with 37/65 ratio (LAR; 1.44 vs 0.80, p=0.598) were higher in cALL survivors while
(56.9%) fulfilling atleast 1 criteria of metabolic syndrome. Hyperten- adiponectin levels were similar. Receiver Operator Curve analysis
sion and impaired fasting glucose (>100mg/dL) were seen in 12/65 revealed largest area under curve (AUC) at 16.3ng/mL for leptin [AUC:
(18.5%) children each. Triglyceride >150mg/dL and HDL <40mg/dL 0.887, (0.780-0.934), sensitivity 88%, specificity 78%] and 4.65 for LAR
was seen in 16/65 (24.6%) and 24/65 (36.9%) survivors, respectively. [AUC: 0.910, (0.801-1.000), sensitivity 88%, specificity 91%]. Survivors
Insulin resistance was seen in 32/65 (49.2%) of survivors. On multi- who were obese/overweight [37.8 (3.78-377.91], or had higher Leptin
variable regression, presence of increased fat mass/Height z-score on [1.11 (1.02-1.19), p=0.006], LAR [1.52 (1.12-2.07), p=0.006], per-
DXA-scan, was the only factor associated with an increased risk of centage fat z-score [4.75 (1.43-15.77), p=0.011] and fat mass/height
metabolic syndrome [OR:11.44 (2.85-45.88), p=0.001]; age at diagno- z-score [7.11 (1.89-26.80), p=0.004], had a statistically significant
sis/assessment, chemotherapy or radiotherapy, did not have a direct increased risk of developing metabolic syndrome in univariate analysis
impact. but not in the multivariable regression analysis.
Conclusions: Metabolic syndrome and its components are frequent Conclusions: Serum adipokines and DXA-scan variables can be used
late effects in cALL survivors from low middle income countries. for surveillance of metabolic syndrome in cALL survivors of low mid-
DXA scan for body composition assessment, is a cost effective tool, dle income countries. This may help in implementing early preventive
which may be incorporated globally in survivorship guidelines, to steer measures, improving the quality of life of these survivors.
appropriate preventive and rehabilitative interventions.

EP726 / #504 RISK AND DETERMINANTS OF REDUCED


EP725 / #556 SERUM ADIPOKINES AS BIOMARKERS FOR BONE MINERAL DENSITY AND FRACTURES IN A NATIONAL
SURVEILLANCE OF METABOLIC SYNDROME IN CHILDHOOD COHORT OF DUTCH CHILDHOOD CANCER SURVIVORS: A
ACUTE LYMPHOBLASTIC LEUKEMIA/LYMPHOMA SURVIVORS DCCSS-LATER STUDY
IN LOW MIDDLE INCOME COUNTRIES: AN EMERGING
CONCEPT Demi De Winter1 , Jenneke Van Atteveld1 , Vincent Pluimakers1 , Marta
Fiocco1,2,3 , Rutger-Jan Nievelstein1,4 , Monique Hobbelink4 , Andrica
Gargi Das1 , Rachna Seth1 , Lakshmy Ramakrishnan2 , Manisha Jana3 , De Vries1,5 , Jacqueline Loonen6 , Eline Van Dulmen-Den Broeder7 ,
Aditya Gupta1 , Jagdish Prasad Meena1 , Vandana Jain4 Helena Van Der Pal1 , Saskia Pluijm1 , Leontien Kremer1,7,8 , Cecile
1 All India Institute of Medical Sciences- New Delhi, Division Of Pediatric Ronckers1 , Margriet Van Der Heiden-Van Der Loo1,9 , A. Birgitta
Oncology, Department Of Pediatrics, New Delhi, India; 2 All India Institute Versluijs1 , Marloes Louwerens10 , Dorine Bresters1,11 , Hanneke Van
of Medical Sciences- New Delhi, Department Of Cardiac Biochemistry, New Santen1,12 , Daniel Olsson13 , Imo Hoefer14 , Sjoerd Van Den Berg15,16 ,
Delhi, India; 3 All India Institute of Medical Sciences, Department Of Radiodi- Jaap Den Hartogh17 , Wim Tissing1,18 , Sebastian Neggers1,16 , Marry
agnosis, New Delhi, India; 4 All India Institute of Medical Science- New Delhi, Van Den Heuvel-Eibrink1,5
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S484 of S635 ABSTRACTS

1 Princess Máxima Center for Pediatric Oncology, Pediatric Oncology, vitamin B12 deficiency, and folic acid deficiency were statistically sig-
Utrecht, Netherlands; 2 Leiden University Medical Center, Medical Statis- nificant. Male sex, obesity, previous/current smoking, and very low
tics Section, Department Of Biomedical Data Science, Leiden, Nether- lumbar spine BMD were significantly associated with reported clinical
lands; 3 Leiden University, Mathematical Institute, Leiden, Netherlands; fractures. Older attained age, platinum drugs, GHD, and low physical
4 University Medical Center Utrecht, Department Of Radiology And Nuclear activity were significantly associated with vertebral fractures.
Medicine, Utrecht, Netherlands; 5 Sophia Children’s Hospital, Erasmus Conclusions: Childhood cancer survivors are at increased risk of any
Medical Center, Department Of Pediatric Oncology, Rotterdam, Nether- fracture. Reduced BMD at follow-up (especially very low lumbar spine
lands; 6 Radboud University Medical Center, Department Of Hematology, BMD) was associated with fractures. In addition, several modifiable risk
Nijmegen, Netherlands; 7 Emma Children’s Hospital, Amsterdam Univer- factors for reduced BMD and vertebral fractures were identified.
sity Medical Center, Vrije Universiteit Amsterdam, Department Of Pediatric
Oncology, Amsterdam, Netherlands; 8 Wilhelmina Children’s Hospital, Uni-
versity Medical Center Utrecht, Utrecht, Netherlands; 9 Dutch Childhood EP726a / #673 DENTAL DEVELOPMENT DISORDERS IN
Oncology Group, Childhood Oncology, Utrecht, Netherlands; 10 Leiden Uni- CHILDREN WHO HAD CHEMOTHERAPY TREATMENT BEFORE
versity Medical Center, Department Of Internal Medicine, Leiden, Nether- THE AGE OF 10 FOR A MALIGNANT DISEASE
lands; 11 Willem Alexander Children’s Hospital, Leiden University Medical
Center, Department Of Pediatrics, Leiden, Netherlands; 12 Wilhelmina Chil- Catharina Dhooge1 , Emilie Delanoye2 , Marike De Vos2 , Sophie Van
dren’s Hospital, University Medical Center Utrecht, Department Of Pediatric Lancker1 , Rita Cauwels3
Endocrinology, Utrecht, Netherlands; 13 Institute of Medicine, Sahlgren- 1 Ghent university hospital, Ped.Hemato/oncology and SCT, Gent, Belgium,
ska Academy, University of Gothenburg, Department Of Internal Medicine 2 University of Gent, Ped hemato/oncology and SCT, Gent, Belgium, 3 Ghent
And Clinical Nutrition, Gothenburg, Sweden; 14 University Medical Center university hospital, Dentistry, Gent, Belgium
Utrecht, Central Diagnostic Laboratory, Utrecht, Netherlands; 15 Erasmus

Medical Center, Department Of Clinical Chemistry, Rotterdam, Nether- Background and Aims: Childhood cancer survivors are at risk to
lands; 16 Erasmus Medical Center, Department Of Internal Medicine, Section develop long term sequelae caused by the treatment. The aim of
Endocrinology, Rotterdam, Netherlands; 17 Dutch Childhood Cancer Organ- this study was to investigate the prevalence of dental abnormalities
isation, -, de Bilt, Netherlands; 18 University Medical Center Groningen, in survivors of childhood cancer treated with multichemotherapy. In
University of Groningen, Department Of Pediatric Oncology, Groningen, addition, a possible effect of type of malignancy and treatment and
Netherlands the occurrence of more and/or more severe dental abnormalities was
examined.
Background and Aims: Childhood cancer survivors are at risk of devel- Methods: Eighty-one patients (age 6 - 20 years) who had been treated
oping skeletal late effects. However, evidence on risk factors for very with chemotherapy for a malignant disease before the age of 10 and
low bone mineral density (BMD, Z-score ≤-2), as well as on risk and who were off therapy for at least 2 years were examined clinically and
risk factors of (vertebral) fractures is limited. We investigated this in radiographically (Planmeca ProMax® 2D). Finally, 69 patients were
a national cohort of Dutch childhood cancer survivors treated from included in the study. For each individual, the permanent teeth with
1963-2002. an abnormal root-to-crown ratio, as well as the number of agenetic
Methods: A total of 2,003 survivors aged 18-45 years at invita- and microdontic teeth were counted. The Individual Defect Index
tion were included (mean age at participation 33.1±7.2 years). BMD (IDeI) was calculated, which quantifies the severity of the developmen-
was assessed by dual-energy X-ray absorptiometry (DXA, n=1,548). tal disorders. The results of the study were compared to reference
We assessed fractures that occurred >5 years after cancer diagno- values from literature. In addition, the possible effect of type of
sis by medical history (n=1,892). Fracture incidence was compared malignancy/chemotherapy was examined.
with Swedish normative data by calculating a standardized incidence Results: At least one tooth development disorder was seen in 66/69
ratio (SIR). Vertebral fractures were evaluated by DXA-assisted verte- patients (95%). Two or more different abnormalities were noted in
bral fracture assessment (n=249). Associations between demographic, 83.3%. Agenesis was diagnosed in 7.2% and microdontia in 30.4%. This
treatment-related, endocrine, as well as lifestyle-related factors and is higher than the normal prevalence of microdontia (2.5%). A higher
reduced BMD and (vertebral) fractures were evaluated using univari- mean IDeI score was observed (score: 13.01, range 0–43) compared to
able or multivariable logistic regression models. normal reference (score: 1.8, range 0–15).
Results: The SIR of any first fracture was 3.53 (95%CI=3.06-4.06) Microdontia and/or severe abnormalities in the root-to-crown ratio are
for male and 5.35 (95%CI=4.46-6.52) for female survivors. Vertebral commonly seen in the neuroblastoma group and agenesis in the Burkitt
fractures were prevalent in 13.3% of evaluable survivors. In the mod- lymphoma pts. The average IDeI score was highest in the ALL category.
els for low (Z-score ≤-1) or very low BMD (Z-score ≤-2), male sex, However, differences between groups were not statistically different.
underweight, shorter follow-up time (continuous), total body irradia- Conclusions: Treatment with multichemotherapy at young age has
tion, cranial irradiation, carboplatin (≥2,000 mg/m2 ), alkylating agents an explicit negative impact on dental development and referral to a
(≥8,000 g/m2 ), hypogonadism, growth hormone deficiency (GHD), specialized pediatric dental team in order to identify developmental
hyperthyroidism, low physical activity, severe vitamin D deficiency, disorders in time is advised.
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ABSTRACTS S485 of S635

EP727 / #883 MAINTAINING GAINS IN SURVIVOR 1 Norwegian University of Science and Technology, Department Of Psychol-

PSYCHOSOCIAL WELLNESS THROUGH VIRTUAL PROGRAMING ogy, Trondheim, Norway; 2 University of Oslo, Department Of Psychology,
DURING A PANDEMIC Oslo, Norway; 3 Lovisenberg Diaconal Hospital, Department Of Research,
Oslo, Norway; 4 University of Oslo, Faculty Of Medicine, Oslo, Norway;
Zoann Dreyer, Larry Geiger, Alicia Howell, Omar Shakeel, Lori 5 Oslo University Hospital, Department Of Pediatric Medicine, Oslo, Nor-
Douglas, Kayla Foster, Joy Griggsby, Doug Suggitt, Tim Porea way; 6 Norwegian University of Science and Technology, Department Of
Baylor College of Medicine, Pediatric Oncology, Houston, United States of Clinical And Molecular Medicine, Trondheim, Norway; 7 St. Olavs Hospital,
America Children’s Clinic, Trondheim, Norway; 8 Norwegian University of Science and
Technology, Department Of Public Health And Nursing, Trondheim, Norway;
Background and Aims: Adult survivors of childhood cancer (LTS) have 9 Norwegian University of Science and Technology, Regional Centre For Child

difficulty adjusting to life. The Periwinkle Foundation (TPF) serves chil- And Youth Mental Health And Child Welfare, Trondheim, Norway
dren with cancer with a variety of programs. In 2018, TPF developed
programming specifically for LTS over age 18 designed to provide a Background and Aims: Long-term survivors of childhood acute lym-
strong social network through positive peer interactions. The program phoblastic leukemia (ALL) demonstrate neurocognitive late effects,
was in person (IP) 2018-2019 but largely virtual due to COVID in including impairments in executive functions (EFs). EFs which are cog-
2020-2021. Our goal was to compare the impact on survivor wellness nitive control processes, enable goal-oriented behavior, and are thus
comparing IP vs. virtual programing. essential for adult life success and wellbeing. Still, relatively few stud-
Methods: Cancer survivors were aged 18-46: pre-pandemic ies have examined neurocognitive functioning in long-term survivors in
46; mid-pandemic 30. One third of LTS had significant physi- adulthood, treated with modern chemotherapy only protocols, which
cal/neuropsychological sequelae. Pre-pandemic, survivors had was the aim of this study.
monthly IP socials and participated in volunteer activities. Mid- Methods: Long-term, adult survivors of childhood ALL (N=53, 51%
pandemic monthly “socials” were largely virtual. To determine the females, mean age=24.40 years, SD=4.41, mean= 14.65 years post-
impact of this novel psycho-social wellness intervention, LTS were diagnosis, SD=3.42) participated in this study assessing intellectual
asked to complete a 5 question SurveyMonkey questionnaire ranking abilities, performance based and self-reported EFs in daily life. Neu-
the impact of the program on their lives as: not important; useful rocognitive performance and self-reported EF complaints were com-
but without significant impact; important; extremely valuable; life pared to population means or medians using one sample t-tests and
changing. Forty of 46 LTS pre-pandemic and 20/30 mid-pandemic Wilcoxon signed rank tests.
program responded. Results were compared between pre-pandemic Results: Survivors demonstrated better general intellectual abilities
(IP) and mid-pandemic (virtual) programming. (p < 0.001), but poorer inhibition (p <0.001) than population mean.
Results: The percent of LTS who rated the impact of this programming Performance of shifting and working memory were not significantly
as life changing or extremely valuable in response to specific questions different from population medians (p > 0.05). Self-reported EF com-
follow with pre-pandemic results preceding mid-pandemic results. plaints were significantly higher than population mean (p <0.001) with
1 –Improved sense of well-being: life changing (75/70%); extremely a strong effect size for the index of metacognitive EFs (MI), and 30%
valuable (20/30%). 2-Helped adjust to cancer history: life changing scoring above clinical cut-off T ≥65. The index of behavior regulation
(65/65%); extremely valuable (13/20%) 3-Improved self-confidence: EFs (BRI) was not significantly different from population mean (p >
life changing (72/70%); extremely valuable (18/25%) 4-Helped estab- 0.05).
lish meaningful relationships: life changing (70/70%); extremely valu- Conclusions: Long-term, adult survivors do not demonstrate poorer
able (20/10%) 5-Helped adjust to/accept ongoing late effects: life performance on most neurocognitive tests compared to the population
changing (68/65%); extremely valuable (15/15%) mean. However, poorer inhibition and higher levels of metacognitive
Conclusions: The impact of TPF LTS Wellness Interventions were EF complaints was found. The latter suggest daily-life problems with
ranked as life changing or extremely valuable in 75-90% of LTS whether working memory, planning, organization and task monitoring. Cogni-
IP or virtual.programming. Establishing a social network of LTS in a tive rehabilitation methods addressing these domains should be exam-
non-medical environment provides critically important peer support ined. Acknowledgements: This study was supported by grants from
which enhances survivor psychological wellness. The Norwegian Cancer Organization and the Norwegian University of
Science and Technology (NTNU).
EP728 / #1256 NEUROCOGNITIVE OUTCOME IN
LONG-TERM, ADULT SURVIVORS OF CHILDHOOD ACUTE EP729 / #1440 EMPLOYMENT STATUS AND OCCUPATIONAL
LYMPHOBLASTIC LEUKEMIA TREATED WITH CHEMOTHERAPY POSITIONS OF CHILDHOOD CANCER SURVIVORS FROM
ONLY DENMARK, FINLAND AND SWEDEN: A REGISTER-BASED
COHORT STUDY FROM THE SALICCS RESEARCH PROGRAMME
Kaja Egset1 , Jan Stubberud2,3 , Siri Weider1 , Ellen Ruud4,5 , Magnus
Hjort6,7 , Mary-Elizabeth Eilertsen8 , Anne Mari Sund9 , Odin Hjemdal1 , Line Frederiksen1 , Camilla Pedersen1 , Hanna Mogensen2 , Luzius
Trude Reinfjell1 Mader3 , Andrea Bautz1 , Mats Talbäck2 , Elli Hirvonen4 , Filippa
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S486 of S635 ABSTRACTS

Norsker1 , Henrik Hasle5 , Nea Malila4 , Laura Madanat-Harjuoja4,6 , EP730 / #1451 PARENTHOOD IN MALE SURVIVORS OF
Maria Feychting2 , Friederike Erdmann1,7 , Jeanette Winther8,9 CHILDHOOD CANCER IN DENMARK, FINLAND AND SWEDEN:
1 Danish Cancer Society Research Center, Childhood Cancer Research A REGISTER-BASED COHORT STUDY FROM THE SALICCS
Group, Copenhagen, Denmark; 2 Karolinska Institutet, Institute Of Envi- RESEARCH PROGRAMME
ronmental Medicine, Stockholm, Sweden; 3 University of Bern, Institute
Of Social And Preventive Medicine, Bern, Switzerland; 4 Cancer Registry Line Frederiksen1 , Hanna Mogensen2 , Luzius Mader3 , Camilla
of Finland, Finnish Cancer Registry, Helsinki, Finland; 5 Aarhus University Pedersen1 , Mats Talbäck2 , Thomas Tjørnelund Nielsen1 , Anja Krøyer1 ,
Hospital, Department Of Pediatrics And Adolescent Medicine, Aarhus, Den- Elli Hirvonen4 , Line Kenborg5 , Anna Holmqvist6 , Laura
mark; 6 Dana Farber Cancer Institute, Boston Children’s Cancer And Blood Madanat-Harjuoja4,7 , Henrik Hasle8 , Nea Malila4 , Maria Feychting2 ,
Disorders Center, Boston, United States of America; 7 University Medical Friederike Erdmann1,9 , Jeanette Winther5,10
Center of the Johannes Gutenberg University, Institute of Medical Biostatis- 1 Danish Cancer Society Research Center, Childhood Cancer Research
tics, Epidemiology and Infomatics (IMBEI), Childhood Cancer Epidemiology, Group, Copenhagen, Denmark; 2 Karolinska Institutet, Institute Of Envi-
Mainz, Germany; 8 Aarhus University, Department Of Clinical Medicine, Fac- ronmental Medicine, Stockholm, Sweden; 3 University of Bern, Institute Of
ulty Of Health, Aarhus, Denmark; 9 Danish Cancer Society Research Center, Social And Preventive Medicine, Bern, Switzerland; 4 Cancer Registry of Fin-
Childhood Cancer Research Group„ Copenhagen, Denmark land, Finnish Cancer Registry, Helsinki, Finland; 5 Danish Cancer Society
Research Center, Childhood Cancer Research Group„ Copenhagen, Den-
Background and Aims: A childhood cancer diagnosis and mark; 6 Lund University, Department Of Clinical Sciences, Lund, Sweden;
late effects of treatment may affect survivors’ possibilities of 7 Dana Farber Cancer Institute, Boston Children’s Cancer And Blood Disor-
employment or highly skilled occupations later in life. In this ders Center, Boston, United States of America; 8 Aarhus University Hospital,
study, we compared the employment and occupational status Department Of Pediatrics And Adolescent Medicine, Aarhus, Denmark;
of childhood cancer survivors with population comparisons and 9 Institute of Medical Biostatistics, Epidemiology and Infomatics (IMBEI),
siblings. University Medical Center of the Johannes Gutenberg University, Childhood
Methods: In a cohort study based on Nordic registers, we identified Cancer Epidemiology, Mainz, Germany; 10 Aarhus University, Department Of
10,461 survivors of childhood cancer diagnosed before age 20 years in Clinical Medicine, Faculty Of Health, Aarhus, Denmark
Denmark, Finland and Sweden since 1971. Survivors were compared
with 48,928 population comparisons matched to survivors by age, sex Background and Aims: A childhood cancer diagnosis, its treatment
and geographical region and 12,605 siblings. Annual outcome infor- and possible late effects may impact whether survivors become par-
mation on employment, unemployment, health-related unemployment ents later in life, but the literature on parenthood in male survivors
and occupational position was obtained from statistical institutes for is sparse. In this study, we compared the probability of parenthood
the period 1980-2017 and assessed in multivariate logistic regression and the number of children among male childhood cancer survivors
analyses from age 30 onwards. with population comparisons and siblings, and we assessed the risks
Results: By 30 years of age, 9.2% (95% CI, 8.6-9.9%) of survivors of congenital malformations in the offspring, as well as the risk of
were unemployed for health reasons. Although this proportion was stillbirths.
slightly smaller for survivors of cancers diagnosed in more recent Methods: In a large Nordic population- and register-based cohort
decades, childhood cancer survivors had considerably higher odds of study, we identified 9,488 male survivors of childhood cancer diag-
health-related unemployment at ages 30, 40 and 50 than population nosed before age 20 years in Denmark, Finland and Sweden since
comparisons (ORage30 , 2.57; 95% CI, 2.35-2.81) and siblings (ORage30 , 1971. Survivors were compared with 45,253 randomly selected males
2.50; 95% CI, 2.15-2.90). Health-related unemployment was particu- from the general population, matched by year of birth and geographical
larly pronounced among survivors of central nervous system tumours region, and 6,541 male siblings of the survivors. Information on parent-
and survivors diagnosed below 15 years of age. We observed no large hood, congenital malformations, and stillbirths was obtained from the
differences in unemployment unrelated to health or in occupational Medical Birth Registers for the period 1973-2017.
position. Results: By age 35 years, 41.4% (95% CI 40.1-42.8%) of childhood
Conclusions: Although our study indicates that many survivors are cancer survivors, 58.9% (95% CI 58.2-59.5%) of population compar-
employed and obtain highly skilled occupational positions to the same isons and 60.5% (95% CI 58.9-62.1%) of siblings had become parents.
extent as the general population and their siblings, we revealed that Survivors of childhood cancer had a lower probability of becoming
some adult survivors of childhood cancer have a substantial burden parents than population comparisons (HR 0.64, 95% CI 0.61-0.67)
of health-related unemployment. These survivors should be offered and siblings (HR 0.63, 95% CI 0.58-0.69) throughout the follow-up
comprehensive survivorship care and interventions for obtaining and period. Moreover, our findings suggest that survivors have fewer chil-
maintaining suitable employment. dren than population comparisons and siblings. Lastly, we observed no
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ABSTRACTS S487 of S635

significant differences between childhood cancer survivors, population of cancer treatment often meant having to reprioritize and shift goal
comparisons and siblings in the probability of fathering a child with a navigation.
congenital malformation, and in the risk of stillbirths. Conclusions: Cancer can disrupt the developmental trajectory for ado-
Conclusions: Our findings point towards lower probabilities of parent- lescent survivors and the adaptation of GET may be an effective means
hood among survivors compared with the general population and their of ameliorating these disruptions.
siblings. Reassuringly, we found no differences in the risk of congenital
malformations in the offspring or risk of stillbirths.
EP732 / #669 FACTORS RELATED TO GROWTH
RETARDATION IN LONG-TERM HIGH-RISK NEUROBLASTOMA
EP731 / #1943 A GOAL-DIRECTED INTERVENTION FOR SURVIVORS TREATED WITH HIGH-DOSE CHEMOTHERAPY
ADOLESCENT CANCER SURVIVORS
Sandrine Haghiri1 , Eleni Karamouza2,3 , Chiraz Fayech1 , Eric
Michelle Fortier1 , Crystle-Joie Agbayani2 , Michael Hoyt3 , Zeev Kain4 , Thebault1,4 , Julie Allard1 , Cécile Thomas-Teinturier5,6 , Christelle
Lilibeth Torno5 , Carol Lin5 Dufour1 , Stephanie Bolle7 , Nadia Haddy8 , Florent De Vathaire6 ,
1 University of California Irvine, Sue & Bill Gross School Of Nursing, Irvine, Dominique Valteau-Couanet1 , Gwenael Le Teuff2,3 , Brice Fresneau1,8
United States of America; 2 University of California Irvine, Psychological 1 Gustave Roussy, University of Paris-Saclay, Department Of Pediatric
Science, Irvine, United States of America; 3 University of California Irvine, Oncology, VILLEJUIF, France; 2 Gustave Roussy, University of Paris-Saclay,
Program In Public Health, Irvine, United States of America; 4 University of Biostatistics And Epidemiology, VILLEJUIF, France; 3 INSERM, University of
California Irvine, Uci Center On Stress And Health, Irvine, United States Paris-Saclay, Oncostat U1018, Labeled Ligue Contre Le Cancer, Villejuif,
of America; 5 CHOC Children’s Hospital, Hyundai Cancer Center, Orange, France; 4 Oscar Lambret Center, Pediatric Oncology, Lille, France; 5 Bicêtre
United States of America Hospital, Pediatric Endocrinology And Diabetology, Le Kremlin-Bicêtre,
France; 6 INSERM, CESP, Gustave Roussy, University of Paris-Saclay, Radi-
Background and Aims: Adolescence is a critical developmental period ation Epidemiology Team, Villejuif, France; 7 Gustave Roussy, University of
that involves negotiating greater independence and autonomy in Paris-Saclay, Radiation Oncology Department, Villejuif, France; 8 INSERM,
social, physical, academic, and professional domains. Accordingly, a CESP, Gustave Roussy, University of Paris-Saclay, Radiation Epidemiology
diagnosis of cancer in this formative period can disrupt achievement Team, VILLEJUIF, France
of developmental norms and this disruption can continue through
survivorship into adulthood. A previously developed psychothera- Background and Aims: High-risk neuroblastoma (HRNB) survivors
peutic intervention that targets mechanisms underlying adjustment treated with high-dose chemotherapy followed by autologous stem
difficulties in young adult cancer survivors – Goal-focused Emotion- cell rescue (HDC-ASCR) experience multiple late effects, including
Regulation Therapy (GET) – is relevant to the adolescent cancer endocrine disorders and gonadal endocrine dysfunction.
population. The purpose of the present study was to gather a rich Methods: We evaluated growth profile after ASCR by using height-
description of cancer experiences with a particular focus on goal for-age (HFA) Z-scores, in a large cohort of 145 five-year disease-free
navigation, coping, and health-related quality of life with adolescent survivors treated for HRNB with HDC-ASCR from 1980-2012 at Gus-
survivors in order to adapt GET. tave Roussy. Association of clinical and therapeutic risk factors with
Methods: A total of 15 adolescent survivors aged 15 – 19 years par- growth curves were evaluated using linear mixed models for repeated
ticipated in individual interviews focused on the following domains: measures data.
Unmet psychosocial needs after treatment, relevance of proposed Results: Heights were available for 138/145 HRNB survivors. Sex-ratio
intervention components, and utilization of the intervention (e.g., M/F=1.12, median age at diagnosis=2.5y (range=0-13.3), median HFA
structure and format of the intervention). Interviews were audio Z-score at diagnosis=0.41 (-2.6-3.5). With a median follow-up of 14.1
recorded, transcribed, and coded for thematic content. years (range=3.3-27), HFA Z-score at last follow-up had decreased,
Results: The following four themes emerged across interviews: aca- with a median HFA Z-score of -0.92 (-3.6-2.2) at a median age of
demic struggles, social functioning, mental health and emotional 18.1y (5.5-33.6). Growth curve analysis showed progressively decreas-
challenges, and goal navigation difficulties. All survivors noted disrup- ing of HFA after ASCR, with no recovery after 15y, resulting in a
tion of academic achievement and challenges navigating academics median HFA Z-score variation of -0.6 (-3.1-2.1) at 15y post-ASCR. Pre-
post-treatment. Related to this struggle were social challenges where existing small height and young age at HDC were important predicting
participants reported difficulty making and maintaining friendships, factors for growth alteration (estimate=0.73 +/-standard-error=0.06,
although most survivors noted their family relationships were stronger p<0.0001 and 0.12 +/-0.002, p<0.0001, respectively). Gonadal insuf-
following their cancer experience. Many adolescents reported strug- ficiency had a negative impact (-0.27 +/-0.05, p<0.0001), whereas
gling with mental health issues, including depression and anxiety, and hormonal replacement therapy (HRT) was beneficial (0.14 +/-0.06,
reported having limited emotion regulation strategies. Finally, many p=0.02). After adjustment for gonadal endocrine dysfunction, HDC
participants noted that the experience of cancer shifted focus to short- regimen was not significantly associated with HFA Z-score, whereas
term, rather than long-term goal attainment and that ongoing effects negative effects were observed with doxorubicin (cumulative doses
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S488 of S635 ABSTRACTS

≥250mg/m2 ) and cisplatin (≥400mg/m2 ), (-0.92 +/-0.18, p<0.0001 and Conclusions: Children who receive CNS-directed treatment or are
-0.38 +/-0.14, p=0.006, respectively). Spinal radiotherapy doses were younger at diagnosis may experience consistently poorer social compe-
not associated with growth impairment (p=0.46). tence over the first 5 years after diagnosis. Interventions should target
Conclusions: In conclusion, we demonstrate for the first time in a large younger children receiving CNS-directed treatment near diagnosis to
cohort of HRNB survivors a frequent growth alteration after HDC- minimize social difficulties over time. Future research including child
ASCR, worsening over time, mainly related to gonadal dysfunction. and father perspectives should examine other predictors of social and
HRT was beneficial, supporting adequate hormonal substitution. The school competence trajectories.
effect of high cumulative doses of doxorubicin and cisplatin before
HDC have to be explored.
EP734 / #1833 OUTCOMES IN PATIENTS WITH SOLID
TUMORS WITH STANDAR CHEMOTHERAPY TREATMENT IN A
EP733 / #1709 FIVE-YEAR TRAJECTORIES OF SOCIAL AND DECENTRALIZED REGION OF SOUTHERN PERU
SCHOOL COMPETENCE AMONG PEDIATRIC CANCER
SURVIVORS Henry Garcia
instituto regional de enfermedades neoplasicas de l sur, Oncologia
Dana Garcia1 , Anna Olsavsky1 , Joseph Rausch1 , Valdeoso Patterson1 , Pediátrica, Arequipa, Peru
Malcolm Sutherland-Foggio1 , Emily Moscato2 , Kathryn Vannatta1 ,
Bruce Compas3 , Cynthia Gerhardt1 Background and Aims: OUTCOMES IN PATIENTS WITH SOLID
1 The Abigail Wexner Research Institute at Nationwide Children’s Hospi- TUMORS WITH STANDAR CHEMOTHERAPY TREATMENT IN A
tal, Center For Biobehavioral Health, Columbus, United States of America; DECENTRALIZED REGION OF SOUTHERN PERU HENRY GAR-
2 Children’s Hospital of Philadelphia, Behavioral Oncology, Philadelphia, CIA P.1 1. Assistant Physician of the Pediatric Oncology Service of
United States of America; 3 Vanderbilt Kennedy Center for Research, Human IREN SUR, Arequipa- Peru BACKGROUND AND OBJECTIVES. can-
Development, Nashville, United States of America cer in children of Latin America is a public health problem, due to
many factors: late diagnosis in advanced stages, cultural socioeco-
Background and Aims: Pediatric cancer survivors may experience nomic difficulties to access oncological services, poor adherence to
poor social and school competence, but limited work has examined treatment, access to drugs that complement standard treatment-
patterns of competence over time or predictors of these trajectories. initiation chemotherapy and second-line options to provide greater
Thus, we examined trajectories of social and school competence across benefit. Lack of availability of modern radiotherapy equipment, etc.
5 years following diagnosis. Central-nervous system (CNS) directed The aim of the present study was to evaluate the results of standard
treatment, diagnostic categories, age at diagnosis, and child sex were chemotherapy treatment in patients treated in a region of southern
examined as predictors of group trajectories. Peru.
Methods: Data (N=326) were from a longitudinal study of children Methods: A total of 189 children and adolescents from 1 to 18 years
with cancer (MAgeAtDiagnosis =10.41, SD=3.91; 47% Female). Mothers old with a diagnosis of solid tumors were evaluated from November
completed the Child Behavior Checklist near diagnosis (T1) and at 2009 to December 2021. Treated with standard chemotherapy only.
one (T2), three (T3), and five (T4) years post-diagnosis. Children were Data were extracted from clinical records and grouped among the main
grouped by whether they received CNS-directed treatment (n=173) and most frequent cancer diagnoses.
within the first year (T2). Group-based trajectory modeling (Nagin, Results: 171/189 (90.5%) of the patients were diagnosed in advanced
2005) examined predictors of trajectory membership, separately for stages III/IV. Being the 66,6% (126/189) most frequent neoplasms:
social and school competence. Osteosarcoma, Wilms tumor, Rhabdomyosarcomas, Lymphomas and
Results: Final models identified two social competence trajectories: Hepatoblastoma. Rare neoplasms were not taken into account. Cases
stable poorer competence (53.1%; 95% CI, 45%-61%) and delayed that were treated completely in another hospital and came only
increases in competence (46.9%; 95% CI, 39%-55%). Age at diag- for control were discarded. It is found that they remain without
nosis (Χ1 2 =14.31 [n=322]; p=0.0002; r2 =0.060) and CNS-directed evidence of the disease (PSEE) in Osteosarcoma 13/35 (37.1%),
treatment (Χ1 2 =5.81 [n=325]; p=0.02; r2 =0.024) individually pre- Wilms tumor (TW) 13/26 (50%), Hepatoblastoma 10/17 (58.82%),
dicted social competence trajectory membership, with younger age Non-Hodgkin lymphoma 11/26 (42.3%), Rhabdomyosarcoma 5/11
and receipt of CNS-directed treatment predicting greater likelihood (45.45%) and Hodgkin lymphoma 3/11 (27.27%). The patients who
of membership in the poorer competence group. When account- died and lost sight were 114/189 (60.31%) y the survivors 63/189
ing for other predictors, only age at diagnosis predicted trajectory (30.33%).
group status (p=.004). Group-based trajectory models also identi- Conclusions: The outcomes still remain unsatisfactory with high mor-
fied three school competence patterns: increasing competence (6.5%; tality, It is necessary have public health strategies to make diagnoses
95% CI, 3.7%-11.1%), decreasing competence (21.4%; 95% CI, 15.7%- in early stages, have drug options to intensify chemotherapy treat-
28.4%), and stable higher competence (72.2%; 95% CI, 64.9%-78.0%). ments and teach to parents to complete treatments and better
Variables of interest did not predict trajectory membership. results.
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ABSTRACTS S489 of S635

EP735 / #528 CLINICIAN PERCEPTIONS OF PASSPORT FOR 1 Baylor College of Medicine, Pediatrics, Houston, United States of America;

CARE FOR SURVIVORSHIP CARE PLAN DELIVERY TO 2 Texas Children’s Hospital, Baylor College of Medicine, Pediatrics, Houston,

SURVIVORS OF CHILDHOOD CANCER United States of America

Maria Monica Gramatges1 , Jason King2 , Ellen Shohet1 , Susan Krause1 , Background and Aims: Tyrosine kinase inhibitors (TKIs) improve
Kevin Musgrave2 , Michael O’Connor2 , Michael Scheurer1 , Marc outcomes for Philadelphia chromosome (Ph)-positive chronic myeloge-
Horowitz1 , C. Michael Fordis2 , David Poplack1 nous leukemia (CML), Ph+ acute lymphoblastic leukemia (ALL), and
1 Texas Children’s Hospital, Baylor College of Medicine, Pediatrics, Hous- Ph-like ALL. However, little is known about the impact of long-term
ton, United States of America; 2 Baylor College of Medicine, Center For TKI exposure in pediatric patients. Our objective was to assess the
Collaborative And Interactive Technologies, Houston, United States of incidence and type of late-onset TKI-related toxicities in children with
America Ph+/Ph-like ALL or CML.
Methods: We performed a retrospective chart review of patients
Background and Aims: The Children’s Oncology Group evidence- under 21 years old diagnosed with CML or Ph+/Ph-like ALL at Texas
based guidelines provide exposure-based risks and recommendations Children’s Cancer Center from 2006-2019 and prescribed a TKI.
for late effects screening of survivors of childhood cancer. The Pass- Patients were excluded who received stem cell transplant (SCT) and
port for Care (PFC) was developed as a web-based clinical deci- who never achieved a durable remission. Data capture began on the
sion support tool to generate a personalized survivorship care plan last day of combination chemotherapy for ALL and one year after
(SCP), derived from guidelines and user-entered exposures. Over 150 diagnosis for CML. Events related to TKI exposure were manually
Long-Term Survivor clinics utilize PFC, generating over 47,000 SCPs. abstracted from the electronic medical record. Descriptive statistics
Our objective was to assess PFC user practices and perceptions of were used to stratify outcomes by diagnosis, exposure to specific TKIs,
impact on clinic workflow, guideline application, and survivor shared and TKI use during data capture.
decision-making. Results: Of the 30 eligible patients, 22 had CML, 7 had Ph+ ALL,
Methods: In June 2021, a 35-item REDCap™ survey was emailed to and 1 had Ph-like ALL. The median follow-up was 6.3 years (range
935 PFC users in 145 clinics: 107 active PFC clinics and 38 inactive. 2.2-14.3). All pericardial (n=3) or pleural (n=3) effusions occurred in
Survey results were summarized and compared with prior 2012 survey patients that continued on a TKI during data capture. Other observed
data using a Chi square test, when applicable. outcomes included hypertension (n=3), ectopy on electrocardiogram
Results: Valid data were available from 142 respondents, represent- (ECG) (n=3), gastrointestinal bleed (n=2), and growth hormone defi-
ing 64 clinics and comprising mostly physicians, advanced practice ciency (n=1). No differences were noted in outcome incidence by
providers (APPs), and nurses. Seventy-one respondents (50%) used diagnosis or TKI exposure type.
PFC to generate an SCP at entry to LTS, and 54 (38%) used PFC at every Conclusions: Long-term complications of TKIs are well-characterized
visit. Data entry was completed by nurses (n=89, 63%), APPs (n=63, in adults, but little is known regarding the long-term impact of these
44%), physicians (n=37, 26%), and data managers (n=13, 9%). Sixty- agents in survivors of childhood leukemia. Our results support assess-
seven respondents (48%) felt data entry was a modest or significant ment of pulmonary, cardiac, and endocrine outcomes in larger child-
barrier to PFC application; however, the majority were satisfied with hood cancer survivor cohorts that continue on TKIs long-term. This
PFC (87%): 72% of respondents felt PFC had a high impact on their study adds to the growing evidence of long-term TKI-associated tox-
ability to accurately apply the guidelines, compared with 41% in 2012 icities and supports ongoing efforts to evaluate the feasibility of TKI
(p<0.001), and 70% felt PFC had a high impact on fostering conversa- discontinuation in children with CML (NCT03817398).
tions with survivors about risk for late effects and screening, compared
with 44% from 2012 (p<0.001).
Conclusions: PFC facilitates guidelines dissemination and uptake, and EP737 / #1800 STRUCTURAL BARRIERS TO SURVIVORSHIP
supports accurate application of guidelines as well as fostering of con- CARE IN SURVIVORS OF CHILDHOOD CANCER
versations regarding survivorship care. The burden of data entry is a
limitation, further corroborated by user prioritization of ‘exposure data Maria Monica Gramatges, Shawki Qasim, Kayla Foster, Omar Shakeel,
pre-population by treatment protocol’ for future PFC modifications. Michelle Fritsch, Mehmet Okcu, Austin Brown, Heidi Russell
Baylor College of Medicine, Pediatric Hematology Oncology, Houston,
United States of America
EP736 / #617 DISTRIBUTION AND FREQUENCY OF
TYROSINE-KINASE INHIBITOR-ASSOCIATED COMPLICATIONS Background and Aims: Consistent long-term follow-up care is impor-
IN SURVIVORS OF PEDIATRIC LEUKEMIAS tant for surveillance and timely treatment of childhood cancer therapy
late effects; however, structural barriers may disproportionately affect
Elizabeth Dong1 , Michael Bruno2 , Jeffrey Kim2 , Melanie Bernhardt2 , at-risk populations. Our objective was to evaluate factors associated
Austin Brown2 , Maria Monica Gramatges2 with loss to follow-up after childhood cancer treatment.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S490 of S635 ABSTRACTS

Methods: We electronically extracted and manually curated electronic high-risk (10.1%), very high-risk (33.1%), no-risk (6.76%), unknown
health records to identify childhood cancer cases diagnosed 2011- (8.11%) and “undocumented” (0.68%). EPR were examined for docu-
2014. Individuals without a cancer diagnosis, treated with surgery mentation of discussions at diagnosis occurring in 116/148 (78.4%)
or observation alone, treated with allogeneic stem cell transplant, or cases. Fertility preservation referral was offered to 64/148 patients.
those that transferred to an outside hospital, relapsed, or died <2 years Males were offered sperm cryopreservation (39), and testicular tis-
from end of treatment (EOT) were excluded. Attendance of at least one sue cryopreservation (1). Females were offered laparoscopic ovarian
Long-Term Survivor (LTS) Clinic visit was the primary outcome. Mul- transposition (1) and ovarian cryopreservation (2). Thirty percent of
tivariable logistic regression was used to estimate odds ratios (OR) males (16/56) declined referral. Reasons for non-referral were not
and 95% confidence intervals (CI) for the association between clinical documented (40/56). In the BMT cohort median age was 17 years.
factors and LTS Clinic attendance. Male:female ratio was 1:0.76. Indications for BMT included malignant
Results: We identified 573 survivors that completed treatment and haematology (64%), non-malignant haematology (30%) and oncol-
were eligible for LTS care. Most were male (57.5%) and/or Latino ogy (7%). Patients were classified as low-risk (2.27%), medium-risk
(52.0%). The median age at diagnosis was 6.7 years (range: 0-20). Over- (4.55%), medium high-risk (6.82%), high-risk (45.5%) and very high-
all, 178 (31.1%) were never seen in LTS clinic. Compared with leukemia risk (38.6%). Thirty-four patients were offered fertility preservation
survivors, lymphoma (OR=2.37, CI: 1.23-4.57), central nervous system and 24 accepted. Sperm cryopreservation (58.3%), testicular tissue
tumor (OR=7.55, CI: 2.43-23.44), and solid tumor (OR=10.23, CI: 4.26- cryopreservation (4.2%) and oocyte cryopreservation (37.5%) were
24.57) survivors were more likely to never attend LTS clinic. Black race offered.
and/or older age at EOT were predictive of lower likelihood of an LTS Conclusions: All patients warrant an informed discussion at diag-
visit (p<0.001). Of those never seen in LTS clinic, 48% had no contact nosis about risk of infertility based on their proposed treatment
with the hospital system 2-5 years after EOT, suggesting loss to follow- regimen. This should be clearly documented in the EPR and their con-
up. Seven percent had ongoing non-oncology contact and 45% had sent. Fertility preservation should be offered to all patients where
ongoing oncology contact, suggesting deficiencies in the LTS referral appropriate.
process.
Conclusions: We identified two barriers to LTS care: unexplained loss
to follow-up and issues with LTS referral. Ongoing efforts include EP739 / #1977 AN ASSESSMENT OF YOUNG PEOPLE LIVING
establishing practice standards for LTS referrals and investigation of BEYOND CANCER AND FACTORS THAT IMPACT THEIR
the impacts of payer status and social determinants on LTS attendance. ATTENDANCE OF THEIR AFTERCARE

Sharon Hou1 , Brianna Henry2 , Fiona Schulte1,3,4


EP738 / #563 A RETROSPECTIVE REVIEW OF FERTILITY 1 University of Calgary, Oncology, Calgary, Canada; 2 University of Cal-
PRESERVATION SERVICES IN A LARGE TEENAGE AND YOUNG gary, Psychology, Calgary, Canada; 3 Alberta Children’s Hospital, Hema-
ADULT CANCER AND BONE MARROW TRANSPLANT CENTRE tology/oncology/blood & Marrow Transplant Program, Calgary, Canada;
4 Cumming School of Medicine, University of Calgary, School Of Medicine,
Sheena Guram, Victoria Grandage Calgary, Canada
University College London Hospital NHS Foundation Trust, Department Of
Paediatric Haematology And Oncology, London, United Kingdom Background and Aims: Young people impacted by childhood cancer
are at risk for medical, psychological, and social late effects. To screen
Background and Aims: Infertility, a distressing late effect of childhood for their risks, receipt of consistent, cancer-specific aftercare is crucial.
cancer, impairs quality of life and contributes to mental health prob- Yet, less than 50% of those that live beyond their cancer attend their
lems. We reviewed our fertility preservation service in a large teenage aftercare and only 35% recognize that they could have a serious health
and young adult (TYA) centre. problem. Aftercare knowledge, core health beliefs, and the sociocul-
Methods: Patients treated by our Haematology, Oncology and Bone tural context of young people living beyond their cancer are critical
Marrow Transplant (BMT) service from 01.01.2020-30.06.2021 were aspects of their outcomes and optimal care. This study 1) examined
included. Data was collected from electronic patient records (EPR). the effect of aftercare knowledge and core health beliefs on adherence
Patients were categorised into risk-groups using the “Children’s Can- to aftercare and 2) described the sociocultural context in which these
cer and Leukaemia Group” (CCLG) Consensus Statement on Oncofer- young people engage in their aftercare.
tility. Methods: Participants completed a cross-sectional online survey
Results: Haematology/Oncology (148) and BMT patients (44) were assessing their cancer history, aftercare attendance and knowledge,
analysed separately. Median age of the haematology/oncology cohort core health beliefs (perceived susceptibility and seriousness to their
was 16 years. Male:female ratio was 1:1.14. Diagnoses included sar- late effects), and sociocultural context (defined as cultural background,
coma (34%), lymphoma (26%), leukaemia (18%), head and neck cancers modified from the Cultural Formulation Interview). Inclusion criteria
(9%), brain tumours (6%) and others (7%). Fertility risk was clas- included those who are currently 18 – 35 years and > 5 years from
sified as low-risk (16.2%), medium-risk (30.0%), medium-high (3%), childhood cancer diagnosis.
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ABSTRACTS S491 of S635

Results: Forty-four participants (30 females, 13 males, 1 non-binary) times (14%), sequelae-related conditions in 20 times (11%), etc. Surg-
with a mean age of 27.3 years (SD = 4.96) completed the study. eries were performed in 95 times. A median age at first admission was
Thirty-two participants (73%) reported engaging in their aftercare and 21 years in bone-joint related disease; it was 22 years in surgical condi-
indicated a significantly greater perceived susceptibility and serious- tion and mental illness; it was 32 years in secondary cancer; and it was
ness to their late effects, compared than those that did not engage in 34 years in pregnancy/childbirth, and digestive/liver disease.
their aftercare, t(32) = -2.38, p < .05, d = .81. Thirteen participants Conclusions: The responsible disease conditions varied widely and the
(29%) reported that their social/cultural background made a differ- age at admission ranged from as young as 12 to as old as 46 years
ence to understanding their aftercare, including their ethnicity, cultural in this study. Relationship between primary diseases and a long-term
values, and family. sequelae should be elucidated in order to provide better benefits for
Conclusions: Core health beliefs of young people living beyond child- all childhood cancer survivors.
hood cancer play an important role in their engagement in their care.
Consideration of the sociocultural context of these young people may
be important to improving their aftercare experience. These find- EP741 / #310 THE ASSOCIATION OF ENVIRONMENTAL
ings clarify possible avenues for clinical intervention to improve their FACTORS WITH NEUROCOGNITIVE OUTCOMES IN SURVIVORS
adherence to aftercare. OF CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA (ALL)

Sarah Nigro1 , Lacey Hall1 , Jennifer Harman1 , Victoria Willard1 ,


EP740 / #256 ANALYSIS OF CHILDHOOD CANCER Heather Conklin1 , Ching-Hon Pui2 , Sima Jeha3 , Lisa Jacola1
SURVIVORS RECEIVING HOSPITALIZATION BENEFITS UNDER 1 St. Jude Children’s Research Hospital, Psychology, Memphis, United States

THE HEART-LINK MUTUAL INSURANCE of America; 2 St. Jude Children’s Research Hospital, Oncology, Memphis,
United States of America; 3 St Jude Children’s Research Hospital, Global
Yasushi Ishida1,2 , Mitsue Hayashi3 , Atsushi Ogawa4 , Shuichi Ozono4 , Pediatric Medicine, Memphis, United States of America
Yuri Okimoto4 , Hiroko Inada4 , Atsushi Kikuta4 , Yoshiaki Kinoshita4 ,
Kimikazu Matsumoto5 , Keizo Horibe4 , Miho Maeda4 , Hiromasa Background and Aims: Survivors of childhood ALL are at risk for
Yabe4 , Akihiro Yoneda6 , Takaaki Yanagisawa4 , Atsushi Manabe4 treatment-related neurocognitive deficits. The role of environmen-
1 Ehime Prefectural Central Hospital, Pediatric Medical Center, Matsuyama, tal factors, including intervention to promote early development and
Japan; 2 Heart-Link Mutual Aid Insurance, President, Niigata, Japan; 3 Heart- socioeconomic status (SES), is not well understood.
Link Mutual Aid Insurance, Vice President, Niigata, Japan; 4 Heart-Link Methods: Participants were 236 children treated on the St. Jude Total
Mutual Aid Insurance, Board Of Director, Niigata, Japan; 5 National Center Therapy Study 16 who completed neurocognitive assessments at the
for Child Health and Development, Children’s Cancer Center, Tokyo, Japan; end of therapy. Neurocognitive outcomes included IQ, attention, pro-
6 National Center for Child Health and Development, Surgical Oncology, cessing speed, executive function, memory, fine motor speed, and
Tokyo, Japan academics, and caregiver ratings of attention, executive function, and
adaptive skills. Participation in rehabilitation services during therapy
Background and Aims: Heart-link Mutual Insurance was established (yes, no) and patient insurance status (private, public; proxy for SES),
in June 2005. Since its establishment, it has been providing life insur- were abstracted from the medical record. Results are age-standardized
ance coverage for children with cancer. We analyzed data from mutual Z-scores (normative mean=0, SD=1).
aid insurance subscribers who received hospitalization benefits. Results: Nearly half (n=110, 46.4%) of patients received rehabilita-
Methods: Study subjects were 86 who received hospitalization ben- tion services during therapy. Groups with and without rehabilitation
efits among total of 465 enrolled in the Heart-Link Mutual Aid Plan did not differ based on patient sex (47.3% female, p=.399), treatment
from 2005 to 2020. We extracted a list of people who received hos- arm (45.5% low risk, p=.929), insurance type (47.7% private, p=.117),
pitalization benefits, and collected information after acquiring the IRB or mean age at diagnosis (7.7 vs. 6.8 years, p=.143). Compared to
permission. The extracted data were analyzed statistically using SPSS those without rehabilitation, the rehabilitation group had more diffi-
Ver 26. culties with attention (Z=-0.28 vs. 0.43, p=.022), executive function
Results: The primary diseases were hematologic tumors (n=56), brain (Z=-0.50 vs. -0.08, p=.003), and adaptive skills (Z=-0.41 vs. -0.13,
tumors (n=5), abdominal tumors (n=8), sarcomas (n=11), and others p=.031). Among the rehabilitation group, there was no difference in
(n=6). Age at onset of primary disease ranged from 0 to 20 years neurocognitive outcomes by insurance. Among those without rehabil-
(median 6.5 years). The number of hospitalizations ranged from 1 to itation, patients with public insurance had worse outcomes than those
10 (median 1 year), age at first admission ranged from 12 to 46 years with private insurance in IQ (Z=-0.04 vs. -0.49, p=.012), processing
(median 28 years), and the interval between the completion of treat- speed (Z=-0.09 vs. -0.64, p=.003), reading (Z=0.20 vs. -0.62, p<.0001),
ment and the first admission ranged from 3 to 35 years (median 16 and math (Z=-0.06 vs. -0.61, p=.035).
years). The accumulated total number of hospitalizations in 86 patients Conclusions: Developmental intervention during treatment for ALL
was 178. Reasons for hospitalization were secondary tumors in 47 and SES are associated with end of therapy neurocognitive outcomes.
times (26%), infectious in 26 times (15%), surgical conditions in 25 Compared to those without rehabilitation, children who required
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S492 of S635 ABSTRACTS

rehabilitation had worse neurocognitive outcomes at the end of ther- EP743 / #950 HEALTHCARE UTILIZATION AND FREQUENCY
apy. Participation in rehabilitation may have mitigated the impact of OF PHYSICIAN VISITS AMONG SURVIVORS OF CHILDHOOD
lower SES, as differences by SES were only observed in the group AND ADOLESCENT CANCER AND POPULATION
without rehabilitation. COMPARATORS IN BRITISH COLUMBIA (BC), CANADA

Katrin Julia Kaal1 , Kim Mcgrail2 , Mark Harrison3 , Mary Mcbride4


EP742 / #698 FEMALE YOUNG ADULT SURVIVORS OF 1 Dalhousie University/Nova Scotia Health, Community Health & Epidemi-
PEDIATRIC CANCER EXPERIENCES OF MENTAL WELL-BEING, ology, Halifax, Canada; 2 University of British Columbia, Center For Health
SELF-IMAGE, AND FERTILITY: A QUALITATIVE STUDY Services And Policy Research, Vancouver, Canada; 3 University of British
Columbia, Faculty Of Pharmaceutical Sciences, Vancouver, Canada; 4 BC
Stina Järvholm1 , Sara Örtengren2 , Sofia Karlsson3 , Marianne Jarfelt4 , Cancer, Clinical Research Center, Vancouver, Canada
Ann Thurin-Kjellberg1
1 Sahlgrenska Academy, University of Gothenburg, Department Of Obstet- Background and Aims: Healthcare utilization and frequency of physi-
rics And Gynecology, Gothenburg, Sweden; 2 Sahlgrenska University Hos- cian visits of survivors of childhood and adolescent cancer have not
pital, Department Of Obstetrics And Gynecology, Gothenburg, Sweden; been studied very widely. Many of the existing studies are out of date,
3 Varberg hospital, Department Of Obstetrics & Gynecology, Gothenburg, often rely on case ascertainment pooled from different institutions or
Sweden; 4 Sahlgrenska, academy, University of Gothenburg, Department Of assessment via self-report. The objective of this work is to assess fre-
Oncology, Gothenburg, Sweden quency of physician visits and the impact of health status on visit rates
among survivors of childhood and adolescent cancer and matched
Background and Aims: Young adult cancer survivors that have gone general population comparators.
through childhood cancer treatment often think about their experi- Methods: The study group consisted of 1,960 survivors diagnosed
ences and express concern about the future. They also report need between 0-19 years from 1970 to 2003, surviving five or more years,
for psychological support and described feeling physically, socially, and alive and resident in BC during the follow-up period (2008-2010) and
mentally marked by their cancer experience. The aim of the present an age and sex frequency-matched population sample of 7,840. Patient
study was to gain in-depth knowledge about how women in their twen- records were linked to provincial physician claims data. Counts of visits
ties with previous childhood cancer treatment experience their fertility to general/family and specialty practitioners were generated. Negative
today and the disease’s effect on their self-image and mental health. binomial regression with and without adjusting for comorbidity (i.e.,
Methods: A qualitive study with semi-structured interview was carried the number of unique major aggregated diagnoses groups during the
out during 2016-18. The study was approved by the regional ethics three years of follow-up) was used.
board, University of Gothenburg, Sweden. Fourteen female survivors Results: Survivor status itself was associated with higher visit rates
of pediatric cancer, treated with chemotherapy and/or radiotherapy, to primary (RR=1.22, 95% CI 1.16 1.26) and specialty care (RR=1.60,
were part of the present study. Median age was 27 years (range 25– 95% 1.51 1.70). The number of major aggregated diagnosis groups
31) and median age at cancer diagnosis was 7.5 years (range 2–13). The was a significant independent predictor of visit frequency with a
interviews were recorded and analyzed by a thematic approach. The stepwise increase in visit rates with each increase in number of
software program ATLAS.ti 8 was used to facilitate the qualitative data major ADGs. Survivors’ health status accounted for a 40% dif-
analysis. ference in the rate of specialist visits (RR=1.19, 95% CI 1.12-
Results: The analysis resulted in three main themes labeled, An experi- 1.26).
ence for better and worse, An everyday life similar and different from Conclusions: Survivors’ health status accounted for a sizable portion
peers and An uncertain fertility. These three main themes contained of estimates, highlighting the importance of taking comorbidity into
a total of 11 sub-themes. The women expressed that their experi- account to accurately estimate utilization and avoid overestimation. It
ences of childhood cancer have both strengthened and impaired their also highlights increased demand/need for healthcare services among
everyday life. Result showed that insufficient information about the survivors, who after taking comorbidity into account still had higher
effects on fertility was given by healthcare staff during the treatment visit rates than population comparators. Healthcare practitioners and
period, and this had led to unnecessary suffering in both practical and policy makers need to be aware of the increased demand and needs of
psychological aspects. this population.
Conclusions: Although it was over 10 years since cancer diagnosis for
all participants it was still present for better and worse. Healthcare
workers need to keep this complexity in mind when facing the “cured” EP744 / #1310 PREMATURE OVARIAN FAILURE IN
young patient. Addressing questions and giving support regarding CHILDHOOD CANCER SURVIVORS IN GREECE
fertility and mental health are wanted.
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ABSTRACTS S493 of S635

Katerina Katsibardi1 , Stavros Glentis2 , Evangelia Nitsa2 , Asimina Turku, Finland; 3 Turku University, Department Of Nursing Science, Turku,
Andritsou2 , Antonia Vlachou1 , Georgia Avgerinou2 , Kleoniki Roka2 , Finland; 4 Turku University, Department Of Computing, Turku, Finland
Maria Filippidou2 , Efthymia Rigatou2 , Spyridoula Chatzinikolaou2 ,
Evangelia Tsironi2 , Antonis Kattamis2 Background and Aims: Background and aims Childhood cancer sur-
1 First Department of Pediatrics, National and Kapodistrian University of vivors require more health monitoring than the average population and
Athens, Aghia Sophia Children’s Hospital, Pediatric Hematology Oncol- they have a higher risk for mental health disorders compared to their
ogy Unit, ATHENS, Greece; 2 First Department of Pediatrics, National siblings. Assessing the need for psychosocial support is essential for
and Kapodistrian University of Athens, Aghia Sophia Children’s Hospital, prevention. This project aimed to automate the process of identifying
Pediatric Hematology Oncology Unit, Athens, Greece childhood cancer patients in need for psychosocial support from elec-
tronic health records (EHRs) with supervised machine learning (ML) in
Background and Aims: Female childhood cancer survivors (CCS) have the form of text classification models.
increased risk of ovarian dysfunction. Our aim is to assess the incidence Methods: We tested three well-known ML-based models to recognize
of premature ovarian failure (POF) in female CCS and evaluate the patients who have received mental health-related care or consultation
AMH in relation to FSH and known single nucleotide polymorphisms from EHRs of 2083 patients. EHRs written in mental health-related
(SNPs) related to premature menopause. units were excluded from the data to see if the models can capture
Methods: POF was considered if FSH>20. AMH (ng/ml) was estimated hidden signals of psychosocial challenges. We used stratified five-fold
into (a) very low: <0.5, (b) low: 0.5–1.0, (c) low normal: 1.0–1.5 and (d) nested cross-validation to evaluate the performance of the models in a
normal: >1.5. The cumulative dose of alkylating agents was calculated binary classification task: no need for support or psychosocial support
using the validated cyclophosphamide equivalent dose (CED) score. needed. Patients belonging to the latter had received psychiatric-level
All survivors were genotyped for SNPs rs1172822 and rs11668344 mental health support or consultation.
in BRSK1 and rs1054875 in FANCI through direct sequencing. Chi- Results: The Random Forest classification model performed best in
square or Fisher’s exact test were applied to test the relationship nested-cross validation with 0.79 mean area under the receiver operat-
between clinical and genetic categorical variables with POF. Mann ing characteristics curve. Performance increases when more EHRs are
Whitney U test was used to compare the age of cancer diagnosis available. Our findings also indicate that nurses with psychosocial sup-
and POF. All analyses were performed using «STATA» software (Stata port training, who retrospectively read the patient notes, agreed more
Corporation, USA). with the model’s predictions than the original recommendations given
Results: 95 female CCS, median age (IQR) at the end of the study in psychiatric consultations of the patients.
13.2 (10.3-17.6) years, median age at diagnosis 5.3 (2.6-11.8) years Conclusions: Automated evaluation of psychosocial conditions may
were included. Survivors were followed for median 5.4 (2.6-11.8) help in the identification of childhood cancer patients who are likely to
years. 76.3% received chemotherapy; median CED 2400 (1900–3800) need mental health-related support later in life.
mg/m2 . POF had 9.5% (9/95) CCS. High FSH had 6.3% (6/95) CCS
and intermediate 2.1% (2/95). Very low AMH was recorded in 6.3%
(6/95) CCS, low in 4.2% (4/95) and low normal in 2.1% (2/9). High EP746 / #779 LONG-TERM FOLLOW-UP OF CHILDHOOD
FSH was strongly associated with low AMH (p<0.001). POF was CANCER SURVIVORS IN AFRICA: A SCOPING REVIEW
significantly associated with hematopoietic stem cell transplantation
(HSCT) (p=0.02) and radiotherapy (p<0.01) but not with chemother- Jesse Lemmen1,2 , Sanne Verhulst1 , Festus Njuguna3 , Terry Vik4 ,
apy (p=0.4). The allele frequencies of the three SNPs were equivalent Gertjan Kaspers1,2 , Saskia Mostert1,2
to the Gnomad European populations, but none of them did associate 1 Emma Children’s Hospital, Amsterdam UMC, Vrije Universiteit, Pediatric
with POF (p>0.5). Oncology, Amsterdam, Netherlands; 2 Prinses Maxima Centrum, Pediatric
Conclusions: The evaluation of AMH had added value in the Oncology, Utrecht, Netherlands; 3 Moi University, Department Of Child
screening for ovarian reserve. More data are needed to eval- Health And Pediatrics, Eldoret, Kenya; 4 Indiana University School of
uate the relationship between SNPs and POF development in Medicine, Pediatrics, Indianapolis, United States of America
CCSs.
Background and Aims: Introduction: The number of children surviv-
ing cancer in Africa is increasing. However, comprehensive knowledge
EP745 / #702 IDENTIFYING CHILDHOOD CANCER PATIENTS about the physical and psychosocial late effects of survivors is lacking,
IN NEED FOR SUPPORT - PSYCHOSOCIAL RISK PREDICTION while this becomes increasingly relevant. Our study maps existing liter-
FROM ELECTRONIC HEALTH RECORDS WITH MACHINE ature regarding the long-term follow-up of childhood cancer survivors
LEARNING in Africa.
Methods: This scoping review follows JBI-guidelines for data extrac-
Päivi Lähteenmäki1 , Akseli Reunamo2 , Sanna Salanterä3 , Hans Moen4 tion, charting and analysis. Databases searched were: OVID Med-
1 Turku University Hospital, Dept Of Pediatric And Adolescent Hematol- line, Embase, African Index Medicus (AIM), Web of Science, Else-
ogy/oncology, Turku, Finland; 2 Turku University, Department Of Biology, vier/Scopus, and Psycinfo. All peer-reviewed literature published in
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S494 of S635 ABSTRACTS

English or French between 1978 and 2021 was considered. Screening an early rehabilitation, <1 year post-treatment (n=20, 76.9%) versus
of titles and abstracts was conducted by two independent reviewers, patients who underwent a late rehabilitation, >1 year post treatment
with a third available in case of doubt, followed by full-text screening (n= 6, 23.1%). In this comparison, given the small sample, descrip-
involving two reviewers. tive measurements, the medians of the different neuropsychological
Results: In total, 82 studies were included for content analysis. Studies variables for each group, were calculated in order to compare said
originated from 9 (17%) of 54 African countries: Egypt (n=45), South- groups.
Africa (n=11), Tunisia (n=7), Morocco (n=6), Nigeria (n=5), Uganda Results: After the intervention, a clear improvement in the executive
(n=3), Cameroon (n=2), Malawi (n=2), Kenya (n=1). Studies addressed and attentional functioning is reflected. This correlates with a greater
following diagnoses: lymphoma (n=15), leukemia (n=13), retinoblas- functionality and autonomy of the child in their daily life, in compari-
toma (n=7), nasopharyngeal carcinoma (n=6), germ-cell tumor (n=6), son to the group that did not perform a cognitive rehabilitation. When
brain tumor (n=5), sarcoma (n=4), nephroblastoma (n=3), ovary tumor we compare the moment of rehabilitation (early versus late), a sim-
(n=1), mixed diagnoses (n=22). Only 20 studies (24%) concerned ilar profile is shown in the cognitive variables of both times, but a
survivors with ≥5 years follow-up. Sixty-three studies described phys- greater functionality and autonomy in school and family day-to-day life
ical late effects, which were classified in 16 categories: cardiology, is evidenced when rehabilitation is early.
secondary malignancy, infertility, neurological, hormonal, gastroen- Conclusions: The improvement in cognitive processing, shows us
terology, nutrition, pulmonary, cutaneous, renal, hematological, bone the validity of these rehabilitation programs in the sub-acute phase,
density, otology, oral-dental, ocular, and cosmetic toxicity. Twenty-one ecological and multidisciplinary program.
studies described psychosocial late effects, which were classified in 5
categories: intelligence, psychological, social reintegration, follow-up
adherence, and quality-of-life malfunctions. EP748 / #537 A10! TRANSITION PROGRAM - ROAD TO
Conclusions: Childhood cancer survivors are emerging in Africa. This ADULT CARE FOR CANCER SURVIVORS
study reveals that literature concerning knowledge and infrastructure
on long-term follow-up of survivors is available from a limited num- Paula Marcote Sinclair, Esther Las Heras
ber of African countries. More countries should focus and report on Sant Joan de Déu Hospital, Oncology, Esplugues, Spain
this E-Poster Topic to assist international workgroups with develop-
ing adapted follow-up guidelines to prevent, identify and monitor late Background and Aims: Background: At Sant Joan de Déu Children’s
effects in survivors. Hospital Barcelona, more than 300 children and adolescents with
newly diagnosed cancer are treated every year. In excess of eighty
percent will survive and 30% with serious late effects. The increasing
EP747 / #774 RESULTS OF THE EARLY survival rate highlights the importance of transitioning for continued
NEURO-REHABILITATION IN PATIENTS WITH BRAIN TUMORS care. A10! is a structured transition-transfer program designed with
the participation of patients, families, and professionals to guaran-
Laura Mangado1 , Cristina Boix1 , Natalia Rodriguez2 , Andres tee the continuity of care within the adult healthcare system. Aims:
Morales1,3 to evaluate the transition process and the satisfaction with the A10!
1 Hospital Sant Joan de Deu, Neurology, Barcelona, Spain; 2 Hospital Sant Program.
Joan de Deu, Rehabilitation, Barcelona, Spain; 3 Hospital Sant Joan de Deu, Methods: We performed a structured interview by phone to 48
Oncology, Barcelona, Spain patients and/or their families who had finished the transition-transfer
A10! Program from June 2020 to February 2022. Additionally, we
Background and Aims: Cognitive functioning requires a constant evaluated the quality of the process through a pre-designed checklist.
interaction between different brain areas. This connectivity can be Results: Forty-eight patients were transferred during the study period,
altered by the different co-adjuvant treatments and by the tumor loca- nine to primary care and 39 to adult hospitals. Forty-four (92%)
tion itself. Due to these aspects, as well as the high vulnerability of a patients received self-care training and were transferred when they
developing brain, an adequate neuropsychological rehabilitation plan felt ready. Five (10%) patients required an extra year of training. Forty-
is necessary at the sub-acute stage, with the aim of achieving greater eight patients had an individualized summary of treatment and plan
functional plasticity. of care. Inconsistencies with old records doubled the expected time
Methods: Two groups of patients were compared in this study. One to produce reports. Adult hospitals’ network received a digitalized
group consisted of 26 patients who completed an eight-month neu- report per patient. Special difficulties were encountered to complete
ropsychological rehabilitation program. The other group, a control a full summary when more than 4 disciplines were involved. Eight per-
group, was made up of 23 patients with the same oncological char- cent of patients did not receive the summary. Periodic clinical sessions
acteristics, but who did not receive this rehabilitation program. For with adult physician are performed to improve coordination of patients
this analysis, the Mann-Whitney test was used. At the same time, from referred to adult hospitals. Eighty-seven percent of patients and/or
the group of patients who underwent rehabilitation treatment, two families and 98% of adult hospital professionals express satisfaction
subgroups were created and compared: patients who had undergone with the program.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S495 of S635

Conclusions: The implementation of the structured transition-transfer multivariable logistic regression, we assessed demographic, car-
A10! Program for children and adolescent cancer survivors to the adult diotoxic and traditional cardiovascular risk factors among CCS, and
healthcare network provided a high satisfaction level among families, their residual risk for cardiac dysfunction compared to siblings.
patients, and professionals. Program improvements through continued Results: CCS were median 27 [range: 14-55] years after diagnosis,
quality control checks is ongoing. 49% were women and 77% received anthracyclines (median dose
180 mg/m2 ) while 30% received RTheart (median prescribed dose
12Gy). Among CCS, 24% had an abnormal LVEF versus 5% of sib-
EP749 / #733 SYSTOLIC DYSFUNCTION IN CHILDHOOD lings (p<0.001). Abnormal GLS occurred in 30% and 11% (p<0.001),
CANCER SURVIVORS ON ECHOCARDIOGRAPHY: SEX-SPECIFIC respectively. Among CCS with normal LVEF, 20% had abnormal
EFFECTS AND RISK BEYOND CARDIOTOXIC EXPOSURES. GLS.
RESULTS FROM THE DCCSS LATER 2 CARD STUDY Risk factors for abnormal LVEF were female sex (OR 1.5; 95%CI
1.1-2.1), younger age at cancer diagnosis (OR 0.95/year increase;
Remy Merkx1 , Jan Leerink2 , E (Lieke) Feijen3 , Esmée De Baat3 , Louise 95%CI 0.91-0.98), anthracycline dose (nonlinear OR), unconventional
Bellersen4 , Elvira Van Dalen5 , Eline Van Dulmen-Den Broeder6 , RTheart fractionation (OR 2.2; 95%CI 1.3-3.9) and, only in women,
Margriet Van Der Heiden-Van Der Loo3 , Marry Van Den RTheart dose (p interaction 0.007). Abnormal GLS was associated
Heuvel-Eibrink7,8 , Chris De Korte1 , Jacqueline Loonen9 , Marloes with female sex (OR 1.8; 95%CI 1.3-2.4), RTheart dose (OR 1.4/10Gy;
Louwerens10 , Angela Maas4 , Sebastian Neggers11 , Cecile 95%CI 1.2-1.6), unconventional fractionation (OR 2.7; 95%CI 1.5-4.9),
Ronckers3,12 , Arco Teske13 , Wim Tissing14,15 , Andrica De Vries3 , Gert diastolic blood pressure (nonlinear OR) and, only in women, with
Weijers1 , Annelies Mavinkurve-Groothuis3 , Helena Van Der Pal3 , anthracycline dose (p interaction 0.019).
Leontien Kremer3 , Wouter Kok2 , Livia Kapusta16,17 Independently of these established risk factors, CCS had a residual risk
1 Radboud university medical center, Medical Imaging„ Nijmegen, Nether- of both abnormal LVEF (OR 2.9; 95%CI 1.4-6.6) and abnormal GLS (OR
lands; 2 Amsterdam University Medical Center, Cardiology, Amsterdam, 2.1; 95%CI 1.2-3.7) compared to siblings.
Netherlands; 3 Princess Máxima Center for Pediatric Oncology, Pediatric Conclusions: Using sex-specific thresholds, female CCS have more
Oncology, Utrecht, Netherlands; 4 Radboud university medical center, Car- cardiac dysfunction than male CCS, with female-sex dependent con-
diology, Nijmegen, Netherlands; 5 Princess Máxima Center for pediatric tributions of cardiotoxic doses. CCS have residual, unexplained risk of
oncology, Pediatric Oncology, Utrecht, Netherlands; 6 Emma Children’s Hos- cardiac dysfunction compared to their siblings.
pital/Amsterdam University Medical Center, Vrije Universiteit Amsterdam,
Department Of Paediatric Oncology, Amsterdam, Netherlands; 7 Sophia
Childrens Hospital, Erasmus Medical Center, Pediatric Oncology, Rotterdam, EP750 / #606 ENDOCRINE SEQUELAE IN FEMALE
Netherlands; 8 Princess Máxima Center for Pediatric Oncology, Division CHILDHOOD CANCER SURVIVORS AT FOLLOW-UP CLINIC AT
Of Pediatric Oncology, Utrecht, Netherlands; 9 Radboud University Medi- A WOMEN’S AND CHILDREN’S HOSPITAL IN URUGUAY
cal Center, Department Of Haematology, Nijmegen, Netherlands; 10 Leiden

University Medical Center, Department Of Internal Medicine, Leiden, Fabiana Morosini1 , Paloma Amarillo1 , Mariana Pintado2 , Germán
Netherlands; 11 Erasmus Medical Center, Department Of Internal Medicine, Peirano2 , Rosa Finozzi2 , Anaulina Silveira1 , Beatriz Mendoza3 ,
Section Endocrinology, Rotterdam, Netherlands; 12 Carl v Ossietzky Univer- Francisco Coppola4 , Luis Castillo1
sity of Oldenburg, Department Of Health Services Research, Oldenburg, 1 Pereira Rossell Hospital Perez Scremini Foundation, Hematology Oncol-
Germany; 13 University Medical Center Utrecht, Cardiology, Utrecht, Nether- ogy Department, Montevideo, Uruguay; 2 Pereira Rossell Hospital Perez
lands; 14 Princess Maxima Center for Pediatric Oncology, Supportive Care, Scremini Foundation, Endocrinology Department, Montevideo, Uruguay;
Utrecht, Netherlands; 15 University Medical Center Groningen, Department 3 Clinicas Hospital, Endocrinology Department, Montevideo, Uruguay;
Of Pediatric Oncology/hematology, Groningen, Netherlands; 16 Amalia Chil- 4 Pereira Rossell Hospital, Ginecology, Montevideo, Uruguay

dren’s Hospital, Radboud university medical center, Pediatric Cardiology,


Nijmegen, Netherlands; 17 Tel Aviv Sourasky Medical Centre, Sackler School Background and Aims: There are scarce reports of long-term follow-
of Medicine, Tel Aviv University, Pediatric Cardiology, Tel Aviv, Israel up (LTFU) and endocrinological impact in female pediatric cancer
survivors in Latin America. We report the experience at a referral cen-
Background and Aims: Childhood cancer survivors (CCS) are at risk ter for women’s and children’s health in Uruguay aiming to describe the
of cardiotoxicity. Global longitudinal strain (GLS) on echocardiography prevalence of endocrine disorders.
may improve our understanding of risk factors for cardiac dysfunction. Methods: A dedicated LTFU clinic for women’s health was set up
Methods: In this cross-sectional Dutch Childhood Cancer Survivor including a pediatric oncologist, endocrinologist, psychologist, social
Study, we included 1,397 ≥5-year CCS treated with anthracyclines, worker and gynecologist. We included all female patients who visited
mitoxantrone, radiotherapy to the heart region (RTheart ), vincristine, the LTFU clinic from 01/2004 to 2011 and thereafter resumed from
cyclophosphamide or ifosfamide, and 277 sibling controls. We com- 2018 to 2020 due to staff availability, who were under 40 years old,
pared prevalence of sex-specific abnormal ejection fraction (LVEF; with cancer diagnosis before 18 years of age and disease-free for at
men <52%, women <54%) and age-/sex-specific abnormal GLS. In least 5 years.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S496 of S635 ABSTRACTS

Results: We included 216 patients. Mean age at cancer diagnosis neuropathy was found in 36.4% of subjects. Cardiotoxicity occurred in
was 7.5 ± 0.3 years. Diagnosis included acute leukemias and lym- 5.3% of subjects.
phomas 55.6%; non CNS solid tumors 36.6%; and CNS tumors 8.7%. Conclusions: The late effect of treatment in childhood cancer survivors
Almost all patients (94.4%) were treated with chemotherapy, while is prevalent. Long-term monitoring using a risk assessment system
43.1% received surgery, 36.6% radiotherapy and 8.8% hematopoi- based on tumor type and treatment history should be carried out to
etic stem cell transplantation (HSCT). In most cases there had been detect late effects in survivors. It is necessary to conduct a cohort study
combinations of treatments. At the last visit, 43.1% of these patients on pediatric cancer patients from the initiation of the treatment to
had at least one endocrine dysfunction. Mean time from end of improve the pediatric cancer survivors’ quality of life.
treatment to detection of endocrine disorders was 4.6 ± 0.6 years.
Endocrine disorders included: metabolic (24.7%), thyrotropic (17.7%),
gonadotropic (15.4%), somatotropic (10.8%),bone (10%) and corti- EP752 / #1509 REMOTE ASSSESSMENT OF PHYSICAL
cotropic (0.9%). The most common disorders were obesity (13.9%), FUNCTION IN ADULT SURVIVORS OF CHILDHOOD CANCER: A
hypothyroidism (13.4%) and ovarian failure (11.1%), and the main risk REPORT FROM THE ST. JUDE LIFETIME COHORT (SJLIFE)
factors for endocrine disorders were radiotherapy (P = 0.007, OR =
1.442, 95% CI1.015 – 3.789) and HSCT (P = 0.017, OR = 1.295, 95% Kirsten Ness1 , Todd Gibson2 , Matthew Krull1 , Sarah Terrell1 ,
CI1.023–2.2115). Matthew Wogksch1 , Deokumar Srivastava3 , Gregory Armstrong1 ,
Conclusions: There was a high prevalence of endocrine late effects Kevin Krull1 , Leslie Robison1 , Melissa Hudson4
in our population, especially in those receiving radiotherapy and 1 St. Jude Children’s Research Hospital, Epidemiology And Cancer Control,
HSCT. A dedicated multidisciplinary team was feasible but there were Memphis, United States of America; 2 National Cancer Institute, Radiation
challenges in sustainability. Epidemiology Branch, Shady Grove, United States of America; 3 St. Jude Chil-
dren’s Research Hospital, Biostatistics, Memphis, United States of America;
4 St. Jude Children’s Research Hospital, Oncology, Memphis, United States of

EP751 / #155 A CROSS-SECTIONAL STUDY ON THE LATE America


EFFECT OF TREATMENT IN INDONESIA’S CHILDHOOD
CANCER SURVIVORS Background and Aims: Childhood cancer survivors have higher rates
of impaired physical function than peers; function appears to decline
Riski Muhaimin, Murti Andriastuti, Wahyuni Indawati, Hikari Sjakti, with age. Less is known about the timing and trajectory of, or about spe-
Setyo Handryastuti, Aman Pulungan cific treatment-related risk factors for, declining function. Addressing
Cipto Mangunkusumo Hospital, Child Health, Jakarta, Indonesia these knowledge gaps requires longitudinal assessment of parame-
ters of physical function in large numbers of survivors, many of whom
Background and Aims: The number of childhood cancer survivors live distant from their cancer treatment center. This study determined
increases every year. Cancer or its various treatments cause late feasibility and validity of remote assessment of hand grip strength, car-
effects that may appear in the future, which also increase the diopulmonary fitness, and usual walking speed in adult survivors of
risks of morbidity and mortality. Currently, no research on late childhood cancer.
effects in childhood cancer survivors has been done in Indone- Methods: Adult survivors of childhood cancer participating in SJLIFE
sia. This study aims to determine late effects in childhood cancer completed assessment of physical function using 3 test conditions
survivors. (in-person, at-home by self-assessment, using remote audio and
Methods: The study was conducted as a cross-sectional study. The video technology). Measures included dynamometer ascertained grip
subjects were childhood cancer survivors who had remission for a min- strength, peak oxygen uptake using cardiopulmonary exercise testing
imum of 6 months when the patients were asked to join the research in for in-person assessment and the two-minute step in place test (peak
Cipto Mangunkusumo Hospital. oxygen uptake=26.56+(0.084*leg lifts)-(0.591*body mass index)) for
Results: A total of 107 subjects aged 6 years to 33 years partici- at home assessments, and walking speed measured over four meters.
pated in this study. The duration of remission was from 6 months Feasibility was evaluated as percentage of persons able to complete all
to 366 months. Based on the medical history, we included acute three assessments, and validity of measures with interclass correlation
lymphoblastic leukemia (56.0%), acute myeloblastic leukemia (4,6%), coefficients (ICC) and Bland Altman methodology. Mean differences
non-Hodgkin’s lymphoma (8.4%), Hodgkin’s lymphoma (2.8%), germ between test conditions were compared with analysis of variance.
cell tumors (4,7%), retinoblastoma (5,7%), and other tumors (16.8%). Results: Of 79 individuals approached, 64 (81.0%) completed the self-
Late puberty was found in 24.6% of subjects. Short stature was found assessment and 60 (75.9%) the remote assessment. Means±Standard
in 37.1% of subjects. Obesity, dyslipidemia, and diabetes mellitus were Deviations for the in-person, self, and remote assessment were:
found in 30.0% of subjects, 45.1% of subjects, and 0.15% of sub- handgrip 39.6±12.9, 38.4±12.7, 38.6±13.6 kilograms (p=0.80,
jects respectively. Decreased bone mineral density was found in 34.9% ICC 0.95); peak oxygen uptake 27.7±9.1, 26.1±6.4, 27.8±6.3
of subjects. The incidence of vitamin D deficiency was 69.8%. Neu- milliliters/kilogram/minute (p=0.35, ICC=0.88); and usual walking
rocognitive impairment was found in 22.9% of subjects. Peripheral speed 1.20±0.20, 1.05±0.25, 0.98±0.66 meters/second (p <0.001).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S497 of S635

Conclusions: Remote assessment of physical function is feasible, and patient survival outcome. More child cancer treatment- support and
measures of handgrip strength and cardiopulmonary fitness are valid. further studies are warranted to confirm activity in the same expanse.
Self- and remote assessment of walking speed overestimate laboratory
measured values, perhaps because participants have to manage their
own stopwatch and may not have adequate reaction time at the start EP754 / #1315 ASSESSING FATIGUE IN CHILDHOOD
and/or end of the path. CANCER SURVIVORS: PSYCHOMETRIC PROPERTIES OF THE
CHECKLIST INDIVIDUAL STRENGTH AND THE SHORT FATIGUE
QUESTIONNAIRE ; A DCCSS LATER STUDY
EP753 / #540 ONCOLOGY-BASED PARTNERSHIP PROMOTES
PATIENT SURVIVAL OUTCOME, A PEDIATRIC BLUEPRINT IN Adriaan Penson1 , Iris Walraven2 , Ewald Bronkhorst2 , Martha A.
WESTERN KENYA MAY REVEAL Grootenhuis3 , Wim Tissing4,5 , Helena Van Der Pal6 , Andrica De Vries7 ,
Marry Van Den Heuvel-Eibrink8,9 , Sebastian Neggers6,10 , Birgitta
Moses Elvis Oburah1 , Dennis Njenga2 , Terry Vik3 , Festus Njuguna4 , Versluys6 , Marloes Louwerens11 , Saskia Pluijm6 , Nicole Blijlevens1 ,
Gilbert Olbara5 , Peace Mbengei2 , Naftali Busakhala6 , Bindu Margriet Van Der Heiden-Van Der Loo12 , Leontien Kremer6,13 , Eline
Madhavi7 , Ramani Ramalingam8 Van Dulmen-Den Broeder14 , Hans Knoop15 , Jacqueline Loonen1
1 Moi Teaching And Referral Hospital-AMPATH, Hematology And Oncology, 1 Radboud University Medical Center, Department Of Hematology,
ELDORET, Kenya; 2 Ampath, Blp, Eldoret, Kenya; 3 Riley Hospital for Chil- Nijmegen, Netherlands; 2 Radboud University Medical Center, Health
dren, Pediatrics, Indianapolis, United States of America; 4 Moi University, Evidence, Nijmegen, Netherlands; 3 Prinses Maxima Center for Pediatric
Pediatric Oncology, ELDORET, Kenya; 5 Moi University, Pediatric Oncol- Oncology, Psycho-oncology, Utrecht, Netherlands; 4 Princess Maxima
ogy, Eldoret, Kenya; 6 Ampath, Hemato-oncology, Eldoret, Kenya; 7 Mount Center for Pediatric Oncology, Supportive Care, Utrecht, Netherlands;
Kenya University, Pharmacy, Nairobi, Kenya; 8 Mount Kenya University, 5 University Medical Center Groningen, Department Of Pediatric Oncol-
Pharmaceutical Chemistry, NAIROBI, Kenya ogy/hematology, Groningen, Netherlands; 6 Princess Máxima Center for
Pediatric Oncology, Pediatric Oncology, Utrecht, Netherlands; 7 Sophia

Background and Aims: The Academic Model Providing Access to Children’s Hospital/Erasmus Medical Center, Department Of Paediatric
Healthcare (AMPATH) oncology was established with a primary goal to Oncology, Rotterdam, Netherlands; 8 Princess Máxima Center for Pedi-
fight the global increase of cancer cases through screening, treatment, atric Oncology, Division Of Pediatric Oncology, Utrecht, Netherlands;
education and training, care for cancer patients and other chronic ill- 9 Sophia Children’s Hospital, Erasmus Medical Center, Pediatric Oncology,
nesses. The objective was to evaluate the impact of partnership to Rotterdam, Netherlands; 10 Erasmus Medical Center, Department Of Inter-
the patient survival outcome among adults and paediatric patients nal Medicine, Section Endocrinology, Rotterdam, Netherlands; 11 Leiden

attending oncology clinics University Medical Center, Department Of Internal Medicine, Leiden,
Methods: A retrospective chart review of patient files was conducted Netherlands; 12 Dutch Childhood Oncology Group, Childhood Oncology,
for 33 mixed paediatric and adult consecutive patients with diseases Utrecht, Netherlands; 13 Emma Children’s Hospital, Amsterdam UMC,
ranging from leukaemia, lymphomas, KS, myeloma, breast and cervical University of Amsterdam, Pediatrics, Amsterdam, Netherlands; 14 Emma

cancers receiving treatment in different AMPATH supported oncology Children’s Hospital/Amsterdam University Medical Center, Vrije Universiteit
facilities from May 2014 to Oct 2019. Different protocols were used Amsterdam, Department Of Paediatric Oncology, Amsterdam, Netherlands;
to determine the number of cycles of treatment for each clinical con- 15 Amsterdam University Medical Centers, Medical Psychology, Amsterdam,

dition. Data was collected at the last cycle of treatment for each file, Netherlands
and at the last follow-up visit and compared to the standard protocol
and documented as either lost to follow up (Lf/u), incomplete(Inco) and Background and Aims: With one in four childhood cancer survivors
complete (compd). Lf/u was reported at ≤ 20%, Inco ≤ 25% and compd (CCS) reporting symptoms of chronic fatigue, it is important to screen
≥ 50%. for and monitor fatigue regularly. Early identification of persons with
Results: The population consisted of 17 males (51.52%) and 16 females fatigue symptoms and, subsequently, providing a personalized inter-
(48.48%). 23 (69.70%) adults and 10 (30.30%) paediatrics. ≥ 80% of the vention can hopefully help to prevent (severe) fatigue to become
sample size reached midway through the treatment protocol. 70% of chronic. Several instruments to measure fatigue exist, although none
paediatric cases sailed through to completion of treatment to the last are validated for use in CCS specifically. The aim of the current study
cycle. Patients received a mean of 5.4 doses (range: 6–8). The aver- was to present psychometric properties of an easy to use fatigue
age time between doses was 25.6 days for the 3 weekly protocol. 24 screening instrument, the Short Fatigue Questionnaire (SFQ), and
were classified with stable disease while 1 with progressive disease. a well-known multidimensional fatigue questionnaire, the Checklist
3 patients were initiated on second-line chemotherapy and the rest Individual Strength (CIS), in a nationwide cohort of CCS.
untraced. Methods: We included 2073 participants from the Dutch Childhood
Conclusions: Donor collaboration enhanced free medication, health- Cancer Survivor Study (DCCSS) LATER cohort. Convergent validity
care support system and accelerated treatment compliance, reduced (correlation with other fatigue questionnaires), structural validity (con-
dropouts, enhanced patient outcome and promoted high paediatric firmatory factor analysis) and internal consistency (Cronbach’s alpha)
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S498 of S635 ABSTRACTS

were calculated for the CIS and SFQ. In addition, test-retest reliabil- perceived themselves at high-risk had higher scores on neuroticism,
ity (correlation, intraclass correlation coefficient (ICC) and weighted anxiety and depression; whereas those whose actual risk was high, had
Cohen’s kappa item scores (Kw) between two measurements within lower scores on those outcomes.
one week), and a cut-off score to indicate severe fatigue, using the Conclusions: There is widespread misperception of risk of infertility
validated CIS-fatigue severity subscale cut-off point of 35 as golden among survivors, with substantial numbers of those who both underes-
standard, were determined for the SFQ. timate and overestimate their risk. Higher perceived risk (regardless of
Results: Pearson’s correlations between CIS/SFQ and other fatigue accuracy) is associated greater psychological distress. Greater atten-
questionnaires were high (>0.8), indicating good convergent validity. tion to educating survivors about fertility risk is necessary, and is an
Confirmatory factor analysis resulted in a four-factor solution for the important part of survivorship care.
CIS and a one-factor solution for the SFQ with Cronbach’s alpha for
each (sub)scale showing good to excellent values (>0.8). Test-retest
reliability of the SFQ was adequate (Pearson’s correlation = 0.88; ICC EP756 / #1565 ESTABLISHING A CHILDHOOD CANCER
= 0.946; Kw ranged 0.31-0.50) and a cut-off score of 18 showed good SURVIVOR’S PROGRAM IN A GENERAL HOSPITAL IN MEXICO:
sensitivity and specificity scores (92.6% and 91.3% respectively). INITIAL STEPS AND LESSONS LEARNED
Conclusions: The current study shows that the SFQ and CIS are good
instruments to assess fatigue in CCS. A cut-off score of 18 for the SFQ Rebeca Rivera-Gomez1,2 , Dora Bastidas1 , Raquel Ruiz1 , Martha
was proposed to easily indicate persons who experience severe fatigue. Garcia1 , Gabriela Murillo1 , Paula Aristizabal3,4,5
1 Hospital General Tijuana, Pediatric Oncology, Tijuana, Mexico;
2 Universidad Autonoma de Baja California, Facultad De Ciencias De
EP755 / #771 RIGHTFULLY WORRIED? ACTUAL VS. La Salud, Tijuana, Mexico; 3 University of California, San Diego, Pediatrics,
PERCEIVED FERTILITY RISK AND RELATIONSHIP TO La Jolla, United States of America; 4 University of California, San Diego,
PSYCHOLOGICAL FUNCTIONING IN AYA SURVIVORS OF Moores Cancer Center Population Science, Disparities And Community
PEDIATRIC CANCER Engagement, La Jolla, United States of America; 5 Rady Children’s Hospital
San Diego, Peckham Center For Cancer And Blood Disorders, San Diego,
Sean Phipps1 , Anandi Ehman2 , Alanna Long1 , Kari Bjornard3 United States of America
1 St. Jude Children’s Research Hospital, Psychology, Memphis, United States

of America; 2 St. Mary’s College of Maryland, Psychology, St. Mary’s City, Background and Aims: Sixty percent of childhood cancer sur-
United States of America; 3 Indiana University School of Medicine, Pedi- vivors experience at least one late-onset therapy-related complication,
atrics, Indianapolis, United States of America requiring long-term follow-up care. Comprehensive programs for can-
cer survivors are scarce in Mexico. We describe the initial steps
Background and Aims: Many survivors of childhood cancer are at risk to implement a childhood cancer survivors’ program (CCSP) at the
for infertility, an issue that may become of greater concern to them General Hospital-Tijuana, Mexico aimed at monitoring and treating
as they reach adolescence and young adulthood. Perceived infertil- late-effects, including prevention of chronic diseases.
ity (whether accurate or not) may impact survivors’ social behavior Methods: A pediatric oncologist (RRG) completed a fellowship in
and psychological functioning. The present study examined the match survivorship in 2019. A multidisciplinary team was established: pedi-
between perceived and actual risk for infertility among AYA survivors, atric oncologists(1), social workers(2), and psychologists(1). In 2019,
and its association with psychological function. patients at least 5 years off-therapy were identified and scheduled
Methods: Survivors of childhood cancer (N = 284; Age range 13- to be seen in the survivorship clinic by the multidisciplinary team in
26; Mean 18.6 years; 52.1% male; time from diagnosis 5-23 years, a “one-stop-shop” model. In 2020, due to the COVID-19 pandemic,
Mean 9.1 years) completed measures of reproductive concerns, social the in-person clinic was paused and a sub-group of patients were
functioning/dating status, personality, and symptoms of anxiety and followed-up by video-calls. In 2021, in-person care resumed. In 2022,
depression. A sample of age/gender matched AYA’s completed a simi- dieticians(1), neuropsychologists(1) and pediatric endocrinologists(1)
lar battery of measures for comparison. Based on review of treatment joined the team.
history, survivors’ risk for infertility was rated on a 4-point scale (no Results: One-hundred forty-three cancer survivors have been identi-
increased risk; low, moderate, and high-risk). fied and 70 have received comprehensive care. Initial barriers included:
Results: As expected, reproductive concerns were greater among lack of dedicated work space; misinformation regarding CCSP purpose
survivors than healthy comparisons (p <.001). However, within the sur- and workflow; incomplete data regarding cancer treatment details in
vivor group, there was only a marginal relationship between perceived charts; difficulty contacting patients who had been discharged from
and actual fertility risk (r = .24, p <.05). Among the 130 survivors iden- the hospital after 5 years off-therapy (per the current practice in
tified at high risk of infertility, 54 (41.5%) perceived their risk as low. A Mexico); and parents/patients doubts regarding returning to the hos-
smaller number of survivors (10.5%) who were at low risk, rated their pital. Complications have been detected in 50% of patients, including:
risk as high. Psychological functioning was significantly related to both neurocognitive deficits (35%), obesity (30%), mental health disorders
perceived and actual fertility risk, but in opposite directions: Those who (25%), ototoxicity (12%).
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S499 of S635

Conclusions: Despite the COVID-19 pandemic and initial barriers, EP758 / #1762 PREFERENCES OF LATINO AND NON-LATINO
50% of childhood cancer survivors have received comprehensive SURVIVORS FOR SURVIVORSHIP EDUCATION AND SERVICES
care. By establishing the CCSP in a general hospital, patients con-
tinue care in our survivorship clinic when transitioning to adults. Omar Shakeel1 , Shiley Aguilar1 , Alicia Howell1 , Ashley Ikwuezunma1 ,
Future steps include: multidisciplinary team strengthening with Olga Taylor1 , Mehmet Okcu1 , Ranjan Bista2 , Juan Carlos Bernini1 , Lisa
continued education, involving pediatric/adult specialists (gener- Kahalley3 , Michael Scheurer1 , Maria Monica Gramatges1
alists, cardiologists, psychiatrists, ophthalmologists), advocating 1 Baylor College of Medicine, Pediatric Hematology Oncology, Houston,
for survivors to gather government resources, and expand the United States of America; 2 El Paso Children’s, Texas Tech University Health
CCSP to provide a high-quality care to survivors in Northwestern Sciences Center El Paso, Pediatrics, El Paso, United States of America;
Mexico. 3 Texas Children’s Hospital, Baylor College of Medicine, Pediatric Psychology,

Houston, United States of America

EP757 / #69 SURVIVAL RATE AND ASSOCIATED FACTORS Background and Aims: There is an unmet need for survivorship edu-
AMONG PATIENTS WITH XERODERMA PIGMENTOSUM AT cation targeting diverse populations of survivors of childhood cancer.
MUHIMBILI NATIONAL HOSPITAL, TANZANIA Our objective was to assess preferences related to accessing sur-
vivorship information, education, and support networks in rural and
Hajaj Mohamed Salum1 , Lulu Chirande2 , Helga Naburi2 metropolitan regions of Texas.
1 Aga Khan Hospital Dar es salaam, Paediatrics And Child Health, Dar Methods: Leveraging the multi-institutional Survivorship and Access
es salaam, Tanzania; 2 Muhimbili University of Health and Allied Science, to Care for Latinos to Understand Disparities (SALUD) cohort, we
Paediatrics And Child Health, Dar es salaam, Tanzania administered a 25-item bilingual survey to adult survivors of child-
hood cancer and parents of younger survivors. All survivors were ≥1
Background and Aims: Xeroderma pigmentosum (XP) is a rare year off-therapy. Responses from survivors vs. parents of survivors
hereditary disease of defective deoxyribunucleic acid repair and Latinos vs. non-Latinos were compared using a Fisher exact test.
defined by extreme sensitivity to sunlight, with a greatly ele- Odds ratios (OR) for the outcomes of interest were calculated with 95%
vated incidence of skin cancers. Mortality mainly occurs from confidence intervals (CI).
2nd decade of life Aim was to determine the survival rate and Results: We received 135 responses from 56 survivors and 79 par-
associated factors among pediatric patients with XP at Muhimbili ents of survivors treated at Texas Children’s Hospital (Houston, n=49),
National Hospital (MNH), Tanzania from June 2011 to December Vannie Cook Children’s Clinic (McAllen, n=70), and El Paso Children’s
2020 (n=16). Respondents were 83% White, 75% Latino, and 27% Spanish-
Methods: A retrospective cohort study with a longitudinal follow up speaking. The mean age of survivor respondents was 22 years (range,
of the pediatric patients with XP at MNH from 2011 to 2020. A 16-32), and 14 years (range, 5-20) for survivors with parent respon-
total of 100 files and database records of registered patients with dents. Most survivors (68%) were >5 years off therapy. From a list of
XP were extracted using a structured data collection tool. Collected 12 E-Poster Topics, both survivors and parents selected ‘risk for second
patient’s information included socio-demographical and clinical char- cancers’ and ‘diet, nutrition, and exercise’ as highest educational pri-
acteristics, and whether alive or dead. Patients with available contact orities, with no difference by ethnicity. Parents were more likely than
information were contacted and consented for a short mobile inter- survivors to seek survivorship information from other survivor fami-
view. Analysis was done using SPSS version 23. Kaplan-Meier survival lies (OR=7.97, CI 1.77, 35.93) and utilize social networks comprised
analysis was used to determine the overall survival rate. Indepen- of survivor families (OR=2.9, CI 1.08, 7.85). Survivors were more likely
dent mortality predictors were assessed in adjusted Cox regression than parents to prefer short videos as a mode of survivorship education
model. delivery (OR=2.41, CI 1.02, 5.70). Non-Latinos were more likely than
Results: Of 100 enrolled patients, half were male. Mean age was 5.4 Latinos to prefer social media as an educational resource (OR=3.70, CI
± 4.1 years. Cutaneous squamous cell carcinoma (cSCC) was found 1.58, 8.68).
in 41% of the patients, ocular squamous cell carcinoma in 25% and Conclusions: Differing preferences for survivorship education and
oral Squamous cell carcinoma in 14%. At the end of the study, 39% of resource utilization by ethnicity and respondent type suggest a need
patients were alive, 35% had been lost to follow up and 26% died. The for adapted content delivery for this vulnerable population.
median survival was 14.1 years (IQR= 11.7 – 17.2). Estimated overall
survival rate was 90%, 64% and 46% at 5, 10 and 15 years respectively.
Those with cSCC had lower survival rate, 87% and 55% at 5 and 10 EP759 / #516 PROGNOSTICATION OF PEDIATRIC
years respectively. Only cSCC was found to be a strong predictor of NASOPHARYNGEAL CARCINOMA PATIENTS USING BASELINE
mortality (HR 2.8, 95% CI 1.0-7.3, P= 0.02) 18F-FDG PET/CT
Conclusions: Survival of patients with XP was progressively decreas-
ing. Cutaneous squamous cell carcinoma was found to be a strong Varun Shukla1 , Sneha Shah1 , Siddhartha Laskar2 , Nehal‘ Khanna3 ,
predictor of mortality. Girish Chinnaswamy2 , Maya Prasad2 , Badira Cheriyalinkal Parambil2
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S500 of S635 ABSTRACTS

1 Mahamana Pandit Madamohan Malaviya Cancer Centre, Nuclear Background and Aims: Background: Carboplatin and cisplatin can
Medicine, Varanasi, India; 2 Tata Memorial Centre, Paediatric Oncology, lead to hearing loss (HL) and quality of life (QOL) deficits in child-
Mumbai, India; 3 Tata Memorial Centre, Radiation Oncology, Mumbai, India hood cancer survivors (CCS). We compared two QOL measures; one
developed for children with HL (HEAR-QL) and a validated measure for
Background and Aims: Paediatric Nasopharyngeal Carcinoma (NPC) CCS (PROMIS). Hypothesis: HEAR-QL would be more sensitive than
is a rare form of malignant cancer. Currently there is paucity of data to PROMIS at identifying QOL deficits in CCS with HL.
support and correlation between metabolic parameters and post treat- Methods: Inclusion criteria: 1) 8-17 years of age, 2) cancer diagno-
ment outcome. Current prognostic indicators are: age of onset, clinical sis, 3) history of ototoxic chemotherapy, 4) at least 6 months post
staging and treatment modality used. therapy, 5) audiogram within 1 year. Exclusion criteria: 1) central ner-
Aim: Assess correlation between metabolic parameters derived from vous system (CNS) malignancy, 2) cranial radiation, 3) CNS surgery,
baseline FDG-PET/CT with patient outcomes (DFS and OS) in patients 4) intrathecal chemotherapy. Participants completed the PROMIS and
with pediatric nasopharyngeal carcinoma (NPC). age appropriate HEAR-QL (-26 or -28) after routine appointments.
Methods: Single centre retrospective observation study, with 64 sub- Results: Fifty individuals were evaluated. Mean age was 12.94 years
jects. Treatment naïve pediatric NPC who underwent baseline FDG (range 8-17), and 56% were male. Twenty-two (44.9%) received cis-
PET/CT were included. Metabolic parameters i.e. SUVmax, SUVmean, platin and 30 (61.2%) carboplatin. Participants with HL (30%) had
metabolic tumor volume (MTV) and total lesion glycolysis (TLG) were significantly lower scores on the HEAR-QL-26 and HEAR-QL-28 than
evaluated from PET/CT data. Univariate analysis of metabolic param- those with normal hearing (mean = 67.9 + 25.6, verses 88.6 + 9.4, p =
eters was done using independent t test. Receiver operating charac- 0.044, and 82.3 + 8.8 verses 94.8 + 3.6, p = 0.016, respectively). Par-
teristic (ROC) curve was generated to calculate cut-off values. Cut-off ticipants with higher SIOP grades (more HL) had lower scores on the
was calculated using Youdens-index for significant variables. Survival HEAR-QL-26 (Grade 0: 88.6 +9.4, Grades 1-2: 77.4 + 10.1, Grades 3-4:
analysis was done using Kaplan Meier analysis 60.4 + 32.8, p = 0.005) and HEAR-QL-28 (Grade 0: 94.8 + 3.6, Grade
Results: Out of 64 subjects, 26 had disease relapse and 17 were dead at 1-2: 84.4 + 0.6, Grade 3-4: 81.3 + 11.1 p = 0.001). Feelings subscale
time of analysis. Univariate analysis using independent t test revealed scores on the HEAR-QL-26 and -28 worsened with higher SIOP grades.
MTV (p<0.001) and TLG (p<0.003) i.e. significant predictors of OS Significant differences were seen in the Environments (HEAR-QL-26),
and DFS. SUVmax and SUVmean - not significant. ROC curve analy- and Hearing Situations (HEAR-QL-28) subscales. The PROMIS failed to
sis revealed area under curve of MTV of 0.861 (p<0.0001) and TLG identify any inferior outcomes with worsening HL.
of 0.815 (p<0.0001) i.e., significant. SUVmax and SUVmean had AUC Conclusions: The HEAR-QL is more sensitive than the PROMIS in iden-
as 0.498 and 0.449 hence not significant. Cut-off for MTV was 67.14 tifying QOL deficits in CCS at risk for HL. The HEAR-QL should be
(Sensitivity 94.1%, Specificity 74.5%, p<0.0001) and TLG 366.09 (Sen- utilized in studies examining QOL of CCS with HL.
sitivity 92.3%, Specificity 65.8%, p<0.0001). Mean survival patients
above cut-off of MTV was 41.72 months (OS) and 28.44 months
(DFS) (p<0.0001), for TLG it was 41.36 months (OS) and 34.55 (DFS) EP761 / #1812 FUNCTIONAL CONNECTIVITY AND
(p<0.0001) ATTENTION IN SURVIVORS OF PEDIATRIC BRAIN TUMORS
Conclusions: MTV and TLG can predict patient adverse outcome inde- AND HEALTHY CONTROLS
pendent of other variables (age, stage etc). SUVmax and SUVmean
were not significant. MTV and TLG can be used to stratify patients for Mehak Stokoe1 , Tiffany Bell1 , Caitlin Forbes1 , Taryn Fay-Mcclymont2 ,
treatment intensification and should be made a part of clinical trials. Signe Bray3 , Catherine Lebel3 , Douglas Strother4 , Gregory Guilcher5 ,
Lucie Lafay-Cousin6 , Keith Yeates7 , Kevin Krull8 , Fiona Schulte9
1 University of Calgary, Cumming School Of Medicine, Calgary, Canada;
EP760 / #630 ASSESSING QUALITY OF LIFE IN CHILDHOOD 2 University of Calgary, Pediatrics, Calgary, Canada; 3 University of Cal-
CANCER SURVIVORS AT RISK FOR HEARING LOSS; A gary, Radiology, Calgary, Canada; 4 Alberta Children’s Hospital, Hematol-
COMPARISON OF THE HEAR-QL AND PROMIS MEASURES ogy Oncology, Calgary, Canada; 5 Alberta Children’s Hospital, Hematol-
ogy/oncology/blood & Marrow Transplant Program, Calgary, Canada; 6 AHS,
Robert Hayashi1 , Anne Spence1 , Susan Hayashi2 , Kara Sauerbuger1 , Section Of Pediatric Hematology Oncology And Bone Marrow Transplanta-
Taniya Varughese,3 , Emily Lafentres2 , Jennifer Henry3 , Judith Lieu4 , tion, Calgary, Canada; 7 University of Calgary, Psychology, Calgary, Canada;
Allison King1 8 St. Jude Children’s Research Hospital, Epidemiology And Cancer Con-
1 Washington University School of Medicine, Pediatrics, st. louis, United trol, Memphis, United States of America; 9 University of Calgary, Oncology,
States of America; 2 Washington University School of Medicine, Pedi- Calgary, Canada
atrics, St. Louis, United States of America; 3 Washington University School
of Medicine, Occupational Therapy, st. louis, United States of Amer- Background and Aims: Treatment of pediatric brain tumors often
ica; 4 Washington University School of Medicine, Otolaryngology, st. louis, impairs social perception (e.g., inhibition, attentional control) in sur-
United States of America vivors. Children receiving radiation therapy are at a greater risk
of impairment in these domains, particularly as the dorsolateral
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S501 of S635

prefrontal cortex (DLPFC) and frontal gyrus may be impacted. This Charles University and University Hospital Motol, Department Of Pedi-
study aimed to identify if DLPFC functional connectivity differences atric Hematology And Oncology, Praha, Czech Republic; 13 Saint-Étienne

exist between survivors and healthy controls. University-Hospital, Department Of Pediatric Hematology And Oncology,
Methods: Thirteen survivors (n=5 glioma, n=2 medulloblastoma, Saint-Étienne, France; 14 Inselspital, Bern University Children’s Hospital,
n=6 other tumours; n=2 chemotherapy, n=12 surgery, n=5 radia- University of Bern, Department Of Pediatric Endocrinology, Diabetology
tion; mean[SD] diagnosis age 5.8[4.1], assessment age 14.4[2.9] years; And Metabolism, Bern, Switzerland; 15 University of Bern, Department
53.8% male) and 19 controls (assessment age 11.7[3.0] years; 57.9% Of Biomedical Research, Bern, Switzerland; 16 Inselspital, Bern University
male) completed resting state functional MRI (3T General Electric Hospital, University of Bern, Division Of Pediatric Hematology/oncology,
Healthcare). The FMRIB Software Library (FSL) was used to assess con- Department Of Pediatrics, Bern, Switzerland
nectivity between the right and left DLPFC as a seed region and the
rest of the brain. Parents completed the attentional control index of the Background and Aims: Hearing impairment can be a late effect of
Behavioral Assessment System for Children-Third Edition (BASC-3). childhood cancer treatment and can lead to impairments in educa-
Higher scores indicate greater distractibility. tion, social attainment, and health-related quality of life (HRQoL).
Results: Stronger connectivity between the right inferior frontal We assessed the impact of hearing impairment on HRQoL in a large
gyrus and left DLPFC was observed in controls only (voxels n=185, European cohort of childhood cancer survivors (CCS).
p=0.0105), no differences between groups were noted in right Methods: Within the PanCareLIFE consortium, we used the com-
DLPFC connectivity. Scores on the attentional control index of bined dataset from four countries (CH, CZ, DE, FR), including 5-year
the BASC-3 did not significantly differ between groups (mean[SD] survivors diagnosed before age 19 years, who were off cancer treat-
survivors=51.9[15.1], controls=44.9[6.3], p=.149). ment and aged 25-44 years at study. Questionnaires were used to
Conclusions: Survivors may have worse connectivity in regions of assess hearing impairment, HRQoL (Short Form 36) and potential
social perception due to the nature of their treatment. Future research confounders including socio-demographic and clinical factors. We
should examine other possible seed regions for connectivity related to performed multivariable linear regression to investigate the role of
social perception with an emphasis on the right inferior frontal gyrus, hearing impairment on HRQoL adjusting for age at survey, sex, edu-
given its role in attention and social cognitive processes. cation, country, period of cancer diagnosis, tumor type, and cancer
treatments.
Results: In total, 6,262 CCS participated, with a mean age at question-
EP762 / #849 IMPACT OF HEARING IMPAIRMENT ON naire survey of 32 years (SD 5). We included 4,627 CCS from Germany,
HEALTH-RELATED QUALITY OF LIFE IN EUROPEAN 804 from Switzerland, 579 from Czech Republic, and 252 from France.
CHILDHOOD CANCER SURVIVORS Fifty-three percent of CCS were female and mean age at diagnosis
was 9 years (SD 5). Hearing impairment was associated with a reduced
Sven Strebel1,2,3 , Katja Baust4 , Desiree Grabow5 , Julianne Byrne6 , physical component (coef. -3.8, 95% CI: -6.4 to -1.1) and mental compo-
Thorsten Langer7 , Antoinette Am Zehnhoff-Dinnesen8 , Rahel nent (-2.9, CI: -5.2 to -0.7). Particularly strong associations were with
Kuonen1,9 , Annette Weiss1,10 , Tomáš Kepák11 , Jarmila Kruseová12 , HRQoL domains of physical functioning (-3.9, CI: -7.0 to -0.8), bodily
Claire Berger13 , Gabriele Calaminus4 , Grit Sommer1,14,15 , Claudia pain (-2.3, CI: -4.0 to -0.5), general health (-4.4, CI: -6.7 to -2.0), vital-
Kuehni1,16 ity (-3.7, CI: -5.9 to -1.6), social functioning (-3.4, CI: -5.8 to -1.0), and
1 Institute of Social and Preventive Medicine, Faculty Of Medicine, Uni- mental health (-2.5, CI: -4.5 to -0.6).
versity Of Bern, Bern, Switzerland; 2 CANSEARCH research platform in Conclusions: This collaborative four-country study suggests that hear-
pediatric oncology and hematology, Department Of Pediatrics, Gynecol- ing impairment following cancer treatment in childhood may affect
ogy And Obstetrics, University Of Geneva, Geneva, Switzerland; 3 Graduate HRQoL. Close monitoring of hearing function with early therapeutic
School for Health Sciences, Faculty Of Medicine, University Of Bern, Bern, support could improve quality of life of young people treated for cancer
Switzerland; 4 University Hospital Bonn, Department Of Pediatric Hema- when they were children.
tology And Oncology, Bonn, Germany; 5 Institute of Medical Biostatistics,
Epidemiology and Infomatics (IMBEI), University Medical Center of the
Johannes Gutenberg University, Childhood Cancer Epidemiology, Mainz, EP763 / #677 THE NUTRITIONAL TRAJECTORY OF
Germany; 6 Boyne Research Institute, Cancer Research, Drogheda, Ireland; CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA: A
7 University Hospital for Children and Adolescents Lübeck, Department Of 10-YEAR FOLLOW UP STUDY FROM A REFERRAL CENTER IN
Pediatric Oncology And Hematology, Lübeck, Germany; 8 Department for SOUTH INDIA
Phoniatrics and Pedaudiology, University Hospital Münster, Münster, Ger-
many; 9 Inselspital, Bern University Hospital, University of Bern, Department Sidharth Totadri, Leenu Joseph, Hema Srinivasan, Magdalenal Robert,
Of Pediatrics, Bern, Switzerland; 10 Bavarian Care and Nursing Author- Deepthi Boddu, Rikki John, Leni Mathew
ity, Amberg, Amberg, Germany; 11 University Hospital Brno & International Christian Medical College Vellore, Pediatric Hematology Oncology Unit,
Clinical Research Center (FNUSA-ICRC), Masaryk University, Department Dept. Of Child Health-1, Vellore, Tamilnadu, India
Of Pediatric Oncology, Brno, Czech Republic; 12 2nd Faculty of Medicine,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S502 of S635 ABSTRACTS

Background and Aims: Overnutrition and undernutrition in children in patients with FAN1 mutations with defective DNA damage repair,
with cancer are associated with adverse events, the risk continuing but has also been infrequently reported in children treated for child-
after completion of treatment. hood cancers with the alkylating agent ifosfamide. To evaluate the
Methods: A file review of children treated for ALL from 2002 to 2012 presence of features of KIN in children with renal function decline and
was performed. The body mass index (BMI) was calculated at diagnosis, a medical history of childhood cancer, we evaluated their history and
end of treatment, and at 5, 8 and 10-years from diagnosis. BMI-centiles renal biopsy results.
were used to categorize the patients as underweight (<5th -percentile), Methods: All consecutive children treated for childhood cancer in the
normal (5th -85th percentile), overweight (85th -95th percentile) and Princess Máxima Center for Pediatric Oncology who were biopsied for
obese (≥95th centile). progressive chronic kidney disease (CKD) with low molecular weight
Results: The study analyzed 179 children with ALL [median age:59- LMW proteinuria of unknown cause between 2018 and 2021 were
months (range:24-182), male:female ratio 2:1]. The immunophenotype included.
was B-ALL, T-ALL and B-myeloid in 147(82%), 26(15%) and 6(3%) Results: Karyomegalic interstitial nephropathy could be diagnosed in
patients, respectively. Fifty-five(31%) patients received cranial irra- all six patients.Features of karyomegaly and senescence were identi-
diation. Of 66(37%) children who were underweight at diagnosis, fied in TECs of these patients by automated morphometric assessment
20(31%), 33(51%) and 12(18%) were underweight, normal and over- of nuclear size distribution, and immunohistochemical markers for
weight/obese after 5-years, respectively (5-year BMI unavailable for DNA damage (γH2AX), cell-cycle arrest (p21+, Ki67-), and nuclear
one patient). Of the 100(56%) children who were normal, 7%, 55% and lamina decay (loss of lamin B1). The number of p21 positive cells
38% were underweight, normal and overweight/obese after 5-years, by far exceeded the typically very small numbers of truly karyome-
respectively. Of the 13(7%) children who were overweight/obese, galic cells. P21 positive TECs were found to contain significantly less
3(23%) and 10(77%) were normal and overweight/obese after 5-years, lysozyme, testifying to defective resorption as an explanation of the
respectively. The median (IQR) BMI Z-score at diagnosis was -1.12(- consistent finding of LMW proteinuria. Moreover, in the 5 patients with
2.40,-0.26). The median (IQR) BMI z-score of the cohort was higher at the largest nuclei, the percentage of p21-positive TECs showed strong
the end of treatment [-0.04(-1.16,1.03), P<0.001], and remained higher inverse correlation with change in eGFR from biopsy to last follow-up
after 5 [0.22(-0.83,1.24), P<0.001] and 10-years [0.30(-0.69,0.99), (R2 =0.93, p<0.01).
P<0.001], respectively. The proportion of overweight/obese individu- Conclusions: Karyomegaly and cellular senescence-associated tubular
als was higher at the end of treatment (25%, P<0.001) and remained dysfunction appear to be a more prevalent, rather than rare, cause of
higher after 5 (34%, P<0.001) and 10 (26%, P=0.001) years. There was otherwise unexplained chronic kidney disease and LMW proteinuria
significant correlation of the baseline BMI Z-score with that observed in children treated for cancer with ifosfamide. This finding may have
at the end of treatment (ρ=0.50, P<0.001), after 5-years important implications for future personalized treatment strategies.
Conclusions: In a large cohort of 179 children treated for ALL, a
ten-year follow up study showed that more than 25% were over-
weight/obese. The BMI Z-score at the time of diagnosis continued to EP765 / #62 WHITE PAPER: ONCO-FERTILITY IN PEDIATRIC
correlate with the Z-score after 10-years. PATIENTS WITH WILMS TUMOR

Maria Elisabeth Madeleine Van Der Perk1 , Nicholas Cost2 , Annelies


EP764 / #571 CELLULAR SENESCENCE IS ASSOCIATED WITH Bos3 , Robert Brannigan4 , Tanzina Chowdhury5 , Andrew Davidoff6 ,
RENAL FUNCTION DECLINE IN CHILDHOOD CANCER Najat C. Daw7 , Jeffrey Dome8 , Peter Ehrlich9 , Norbert Graf10 , James
PATIENTS WITH KARYOMEGALIC INTERSTITIAL NEPHROPATHY Geller11 , John Kalapurakal12 , Kathleen Kieran13 , Marcus Malek14 ,
Mary Mcaleer15 , Elizabeth Mullen16 , Luke Pater17 , Angela Polanco18 ,
Sebastiaan Knoppert1 , Mandy Keijzer-Veen2 , Floris Valentijn1 , Marry Rodrigo Romao19 , Amanda Saltzman20 , Amy Walz21 , Andrew
Van Den Heuvel-Eibrink3 , Marc Lilien2 , Gerrit Van Den Berg2 , Lianne Woods22 , Marry Van Den Heuvel-Eibrink23 , Conrad Fernandez24
Haveman3 , Marijn Stokman4 , Geert Janssens3 , Roel Broekhuizen1 , 1 Prinses Máxima Centrum voor kinderoncologie, Pediatric Oncology,
Roel Goldschmeding1 , Tri Nguyen1 Utrecht, Netherlands; 2 University of Colorado School of Medicine, Chil-
1 University Medical Center Utrecht, Pathology, Utrecht, Netherlands; dren’s Hospital Colorado, Department Of Surgery, Division Of Urology, And
2 Wilhelmina Children’s Hospital, University Medical Center Utrecht, Pedi- The Surgical Oncology Program, Aurora, United States of America; 3 UMC
atric Nephrology, Utrecht, Netherlands; 3 Princess Máxima Center for Utrecht, Reproductive Medicine And Gynaecology, Utrecht, Netherlands;
Pediatric Oncology, Division Of Pediatric Oncology, Utrecht, Netherlands; 4 Northwestern University, Department Of Urology, Evanston, United States

4 University Medical Center Utrecht, Genetics, Utrecht, Netherlands of America; 5 Great Ormond Street Hospital for Children NHS Foundation
Trust, Pediatric Oncology, London, United Kingdom; 6 St. Jude Children’s
Background and Aims: Patients with karyomegalic interstitial Research Hospital, Department Of Surgery, Memphis, United States of
nephropathy (KIN) show a clinical picture of interstitial nephopathy America; 7 MD Anderson Cancer Center, Department Of Pediatrics - Patient
with enlarged, irregular and hyperchromatic nuclei of tubular epithelial Care, Houston, United States of America; 8 Children’s National Hospi-
cells (TECs) on renal biopsy. The histologic pattern was first described tal, Division Of Oncology, Washington, DC, United States of America;
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S503 of S635

9 University of Michigan, C.S. Mott Children’s Hospital, Pediatric Surgery, ethical challenges. Identification of genetic markers of susceptibil-
Ann Arbor, United States of America; 10 Saarland University Medical Cen- ity to gonadotoxic therapy may help to stratify patient risk of
ter, Department For Pediatric Oncology And Hematology, d, Germany; gonadal damage and identify patients most likely to benefit from FP
11 Cincinnati Children’s Hospital Medical Center, Division Of Pediatric methods.
Oncology, Cincinnati, United States of America; 12 Northwestern University, Conclusions: We summarize the literature regarding fertility risk
Department Of Radiation Oncology, Evanston, United States of America; and preservation in WT and make recommendations regarding the
13 University of Washington, Department Of Urology, Seattle, United States approach to onco-fertility in this young population.
of America; 14 UPMC Children’s Hospital of Pittsburgh, Division Of Pedi-
atric General And Thoracic Surgery, ff, United States of America; 15 The

University of Texas MD Anderson Cancer Center, Department Of Radia- EP766 / #34 EXERCISE TOLERANCE AND PHYSICAL
tion Oncology, Houston, United States of America; 16 Children’s Hospital ACTIVITY SHORT AFTER INTENSIVE TREATMENT IN PATIENTS
Boston/Dana-Farber Cancer Institute, Department Of Pediatric Oncology, WITH CHILDHOOD CANCER
Boston, United States of America; 17 University of Cincinnati, Department
Of Radiation Oncology, d, United States of America; 18 National Cancer Miek Hornikx1 , Anne Uyttebroeck2 , Deveny Vanrusselt2 , Charlotte
Research Institute Children’s Group, Consumer Representative, Leicester, Sleurs2 , Marc Gewillig3 , Sabine Verschueren1
United Kingdom; 19 IWK Health Centre, Dalhousie University, Departments 1 KULeuven, Department Of Rehabilitation Sciences, Leuven, Belgium;
Of Surgery And Urology, Halifax, Canada; 20 Ann & Robert H. Lurie Children’s 2 KULeuven, Department Of Oncology, Leuven, Belgium; 3 KULeuven,

Hospital of Chicago, Division Of Hematology, Oncology, Neuro-oncology, Department Of Cardiovascular Sciences, Leuven, Belgium
And Stem Cell Transplant, Chicago, United States of America; 21 Ann &
Robert H. Lurie Children’s Hospital of Chicago, Division Of Hematology, Background and Aims: Patients with childhood cancer are confronted
Oncology, Neuro-oncology, And Stem Cell Transplant, d, United States of with exercise intolerance (EI (VO2 peak<85% predicted)) after treat-
America; 22 Children’s Cancer Therapy Development Institute, Pediatrics, d, ment, with a detrimental effect on quality of life and mortality. Knowl-
United States of America; 23 Princess Máxima Center for Pediatric Oncology, edge on the limiting factor(s) for this EI and its relation with physical
Division Of Pediatric Oncology, Utrecht, Netherlands; 24 IWK Health Centre activity (PA) is essential in order to prescribe individually tailored
and Dalhousie University, Department Of Pediatric Hematology/oncology, d, rehabilitation and to stimulate physical and social reintegration.
Canada Methods: Forty-one patients with childhood cancer (13±3 years; 71%
boys; BMI: 20±4 kg/m2 ), diagnosed with leukemia/lymphoma (61%),
Background and Aims: The survival of childhood Wilms tumor is solid tumor (32%) or brain tumor (7%) and recently finalized (dura-
currently around 90%, with many survivors reaching reproductive tion cancer treatment: 216 [168-270] days) their oncology-related
age. Chemotherapy and radiotherapy are established risk factors for treatment were included in the study. Patients performed a maximal
gonadal damage and are used in both COG and SIOP Wilms tumor symptom-limited cardiopulmonary exercise test (CPET) on a tread-
treatment protocols. The risk of infertility in Wilms tumor patients is mill (4.8 km/h; +2% elevation/min). PA was recorded with a 3-axial
low but increases with intensification of treatment including the use accelerometer (Dynaport MoveMonitor, McRoberts, The Hague), that
of alkylating agents, whole abdominal radiation or radiotherapy to the patients wore for 7 consecutive days. Active time (standing and
pelvis. walking), sedentary time and steps were withheld.
Methods: Both COG and SIOP protocols aim to limit the use of Results: The duration of CPET amounted 7 [6-9] minutes, reaching
gonadotoxic treatment, but unfortunately this cannot be avoided in an inclination of 12 [10-16] %. Exercise tolerance (VO2 peak: 29.7±7.8
all patients. Infertility is considered one of the most important late ml/min/kg (67±16% predicted)) was markedly reduced in patients with
effects of childhood cancer treatment by patients and their families. childhood cancer compared to healthy peers. Eighty-eight percent of
Thus, timely discussion of gonadal damage risk and fertility preserva- patients were defined as exercise intolerant. The majority of patients
tion options is important. Additionally, irrespective of the choice for were peripherally limited (83%). A cardiac limitation was present in
preservation, consultation with a fertility preservation (FP) team is 71% of patients and was predominantly due to a reduced oxygen pulse
associated with decreased patient and family regret and better quality (97%). Hyperventilation (32%) and a ventilatory limitation (12%) were
of life. less prevalent. PA data of 13 patients were available (Active time:
Results: Current guidelines recommend early discussion of the impact 178±67 minutes/day; sedentary time: 515±113 minutes/day; steps:
of therapy on potential fertility. Since most patients with Wilms 6411 [4458-6838]).
tumors are pre-pubertal, potential FP methods for this group are Conclusions: Exercise tolerance is markedly reduced in patients
still considered experimental. There are no proven methods for with childhood cancer short after intensive treatment and mainly
FP for pre-pubertal males (testicular biopsy for cryopreservation caused by deconditioning of peripheral muscles and reduced oxygen
is experimental), and there is just a single option for pre-pubertal pulse. Further research is necessary to study the link with physical
females (ovarian tissue cryopreservation), posing both technical and activity.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S504 of S635 ABSTRACTS

EP767 / #1130 CANCER-RELATED WORRY AS A PREDICTOR EP768 / #542 THE ASSOCIATION BETWEEN SOCIAL
OF 5-YEAR PHYSICAL ACTIVITY LEVEL IN CHILDHOOD SUPPORT AND POSTTRAUMATIC GROWTH WITH IDENTITY
CANCER SURVIVORS STATUS AMONG ADOLESCENT AND YOUNG ADULT CANCER
SURVIVORS
Megan Ware1 , Angela Delaney1,2 , Kevin Krull1,3 , Tara Brinkman1,3 ,
Gregory Armstrong1,4 , Carmen Wilson1 , Daniel Mulrooney1 , Megan Wirtz1,2 , Jennifer Ford2
Zhaoming Wang1 , Jennifer Lanctot1 , Matthew Krull1 , Robyn Partin1 , 1 The Graduate Center, Psychology, New York, United States of America;
Kyla Shelton1 , Deokumar Srivastava5 , Melissa Hudson1,4 , Leslie 2 Hunter College, Psychology, New York, United States of America

Robison1 , Kirsten Ness1


1 St. Jude Children’s Research Hospital, Epidemiology And Cancer Con- Background and Aims: Over 85% of children and adolescents who are
trol, Memphis, United States of America; 2 St. Jude Children’s Research diagnosed with cancer survive for at least 5 years. Alongside successful
Hospital, Department Of Pediatric Medicine, Memphis, United States of survival rates come various late effects of cancer, such as incomplete
America; 3 St. Jude Children’s Research Hospital, Psychology, Memphis, identity development, which can impact health-related quality of life.
United States of America; 4 ST. JUDE CHILDREN’S RESEARCH, Oncology, Social support and posttraumatic growth (PTG) may play a significant
Memphis, United States of America; 5 ST. JUDE CHILDREN’S RESEARCH, role in identity development. By exploring these variables as potential
Biostatistics, Memphis, United States of America drivers of or barriers to identity development among adolescent and
young adult (AYA) cancer survivors, we might elucidate mechanisms
Background and Aims: Cancer-related worry (CRW) is a prevalent that lead to identity development, thus influencing future quality of life.
psychological outcome among childhood cancer survivors; 69% of Methods: AYA cancer survivors (n=153) completed the Inventory of
survivors report concerns about health and/or a future cancer diag- Parent and Peer Attachment, the Posttraumatic Growth Inventory, and
nosis. Elevated CRW in survivors has been associated with reporting the Extended Objective Measure of Ego Identity Status, which groups
risky health behaviors, such as not meeting recommended guide- participants into four identity statuses: diffusion, foreclosure, morato-
lines for physical activity (PA). The aim of this investigation was rium, and achievement. Moderation models were used to assess the
to describe associations between CRW and subsequent level of effect of PTG on the relationship between parent and peer attachment
PA. and identity status.
Methods: CRW was collected at a baseline clinical assessment using Results: Mean age was 21.8 years (SD = 2.55) and 91.5% identified
6 survey items. Using factor analysis, two groups were identified as White, 3.9% as Black/African American, 1.3% each of American
“Body-Focused” and “General Fear”. Total CRW scores were calcu- Indian/Alaska Native and Asian/Pacific Islander, and 7.2% identified
lated as the sum of the average responses to the “Body-Focused” as Hispanic/Latino. Within the sample, 58.2% identified as male and
and “General Fear” items. Moderate and vigorous physical activity 41.8% as female. Thirty-five (24.6%) participants were identified as
(MVPA) minutes were captured 5-years later using accelerometry identity diffused; 24 (16.9%) as foreclosed; 61 (43%) as in moratorium;
and categorized for analysis into meeting or not meeting recom- and 22 (15.5%) as in achievement. There was a significant interac-
mended guidelines (150 minutes moderate and/or 75 minutes vig- tion effect of PTG on the relationship between peer attachment and
orous/week). Logistic regression determined independent associa- foreclosure (B = 0.0050; SE = 0.0019; p = 0.008).
tions between CRW and MVPA adjusting for sex, race, diagnosis, Conclusions: PTG moderated the relationship between peer attach-
age at baseline, and presence of grade 3-5 chronic conditions at ment and the foreclosed identity status. Our data provides preliminary
baseline. evidence for the role that both social support, particularly peer sup-
Results: Among 1,218 participants (49% female, mean [SD] age at base- port, and PTG may affect identity development. Future studies should
line 31.5 [6.87] years, mean [SD] age at follow up 36.8 [7.0] years), assess all three constructs over time to ascertain both change and
25% met PA guidelines and 8% reported CRW (responses “agree” influence.
(score=4) or “strongly agree” (score=5) for individual items, averaged
and summed for total CRW score of 8-10). Participants who identi-
fied as black race (OR 1.78; 95% CI 1.17-2.73) and those who had EP769 / #402 MOVING FORWARD IN PEDIATRIC EXERCISE
neurological conditions (OR 1.75; 95% CI 1.40-2.17) were less likely ONCOLOGY: DEVELOPING TRAINING RESOURCES TO SUPPORT
to meet PA guidelines. Odds of not meeting PA guidelines increased EXERCISE PROFESSIONALS
for every one-point increase in total CRW [BTM4] (OR 1.16; 95% CI
1.08-1.25). Findings were similar for both “Body-Focused” (OR 1.34; Amanda Wurz1 , Emma Mclaughlin2 , Gregory Guilcher3 , Beverly
95% CI 1.15- 1.56) and “General Fear” (OR 1.22; 95% CI 1.08-1.38) Wilson4 , Sara Fisher4 , Carolina Chamorro Vina5 , S. Nicole
factors. Culos-Reed2
Conclusions: Interventions to promote PA among adult survivors 1 University of the Fraser Valley, Kinesiology, Chilliwack, Canada; 2 University

of childhood cancer should consider CRW during implementa- of Calgary, Kinesiology, Calgary, Canada; 3 Alberta Children’s Hospi-
tion. tal, Hematology/oncology/blood & Marrow Transplant Program, Calgary,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S505 of S635

Canada; 4 Stollery Children’s Hospital, Northern Alberta Children’s Cancer atric Oncology/hematology Unit, Bogotá, Colombia; 5 Universidad Nacional
Program, Edmonton, Canada; 5 Kids Cancer Care Foundation of Alberta, de Colombia-HOMI Fundación Hospital Pediátrico La Misericordia, Bogotá
Peer Program, Calgary, Canada D.C, Colombia, Grupo De Oncohematología Pediátrica, Bogotá, Colombia;
6 HOMI Fundación Hospital Pediátrico La Misericordia, Bogotá, Colombia,
Background and Aims: Qualified exercise professionals (QEP) are Pathology Unit, Bogotá, Colombia; 7 Servicios Médicos Yunis Turbay y Cía
crucial to implement safe and effective physical activity (PA), particu- S.A.S. Bogotá D.C., Colombia., Instituto De Genética, Bogotá, Colombia
larly for children and adolescents affected by cancer. Yet, few training
resources are available for QEP working in pediatric oncology, rep- Background and Aims: Deletions in IKZF1 have been associated with
resenting a barrier to implementation, and ultimately sustainable PA poor prognosis in pediatric B-cell precursor ALL (BCP-ALL). This study
delivery. aimed to describe the frequency of IKZF1 and IKZF1 plus deletions in
Methods: To equip QEP with the knowledge, skills, and abilities to a cohort of Colombian patients with BCP- ALL.
implement safe and effective PA, an implementation science frame- Methods: Gene deletions were assessed by multiplex ligation-
work was adopted and we created a comprehensive training protocol dependent probe amplification (MLPA), using a commercially available
(>30 hours) that covers substantive (eg, disease, treatment-related kit (SALSA MLPA P335 ALL-IKZF1 probemix). Between 2018 – 2020,
side-effects, role of PA, progression principles) and practical com- 117 de novo pediatric BCP-ALL patients from Fundación Hospital
ponents (eg, tailoring considerations, fostering autonomy). Training Pediátrico La Misericordia (Bogotá, Colombia) were enrolled. All the
is delivered online asynchronously and synchronously via modules, patients were treated according to BFM – ALLIC 2009 protocol.
workshops, scenario-based competency sessions, and shadowing of Results: IKZF1 and IKZF1 plus deletions were detected in 17 (14.5%)
pediatric oncology PA sessions. QEP are audited during PA delivery to patients. In patients with IKZF1 and IKZF1 plus, we found higher age at
ensure safety and effectiveness (eg, PA progression, behaviour mod- diagnosis compared with no IKZF1 deletions patients (9,52y vs 6.83y)
ification support) and semi-structured interviews are used to assess p=0.01. IKZF1 deletions were detected in 5.98% (n=7) and IKZF1 plus
training components, instructor confidence, and need for ongoing were detected in 8.54% (n=10). In patients with IKZF1 deletions, the
support. median age was 9.15 y, 2 patients had iAMP21, 2 had poor steroid
Results: Within an evidence-based pediatric oncology PA interven- response, 4 were classified as high risk and 2 with residual disease
tion (IMPACT; Trial registration: NCT04956133), QEP onboarding and >0,1% at the end of induction. During the follow up, 1 patient had early
training commenced 10/2021 (n=7) and PA delivery began 03/2022. relapse. Within the group of 10 patients with IKZF1 plus, the median
Audits will commence 04/2022 and first interviews will occur 05/2022. age was 9,8 y, 2 had CNS 3, 2 had t(9;22), 5 had poor steroid response,
Data will be analyzed using descriptive statistics and content analysis. 5 were classified as high risk and 3 with residual disease >0,1% at the
Conclusions: QEP play a vital role in PA implementation and sustain- end of induction. During the follow up, early relapse was observed in 2
ability, yet few resources are available to support QEP in pediatric patients.
oncology. By reporting on our training protocol, it is hoped that oth- Conclusions: These findings support that this cohort of patients has
ers will do the same, enhancing transparency in reporting and moving similar frequency of IKZF1 and IKZF1 plus deletions comparing with
towards standardized QEP training requisites in pediatric oncology. reports from other countries. MLPA could help improving risk strati-
fication of BCP-ALL like other investigation groups propose (AEIOP-
BFM ALL 2017 and DCOG ALL11). We should follow up this cohort to
E-Poster Topic: AS05 SIOP Scientific programme / AS05.a Acute see the impact of IKZF1 in survival outcomes.
Lymphoblastic Leukaemia

E-POSTER VIEWING E-Poster Topic: AS05 SIOP Scientific programme / AS05.b Myeloid
Leukemias, Myelodysplastic and Myeloproliferative Syndromes
EP770 / #1159 FRECUENCY OF IKZF1 AND IKZF1PLUS
DELETIONS IN CHILDREN WITH PATIENTS PEDIATRIC B CELL E-POSTER VIEWING
PRECURSOR ACUTE LYMPHOBLASTIC LEUKEMIA CHILDREN.
AN INTERIM ANALYSIS IN A COLOMBIAN COHORT EP771 / #1819 RELAPSE AFTER ALLOGENEIC
HEMATOPOIETIC STEM CELL TRANSPLANTATION FOR
Cindy Martinez1 , Luz Yunis2,3 , Adriana Linares4,5 , Isabel Sarmiento1 , PEDIATRIC PATIENTS WITH ACUTE MYELOID LEUKEMIA OR
Johnny Garcia1 , Gloria Uribe1,6 , Edna Quintero1,6 , Juan Yunis2,7 MYELODYSPLASTIC SYNDROME: SINGLE INSTITUTION
1 Universidad Nacional de Colombia -Fundación Hospital Pediátrico La Mis- RETROSPECTIVE STUDY
ericordia, Grupo Oncohematología Pediátrica, Bogota, Colombia; 2 Grupo

de patología molecular, Universidad Nacional de Colombia, Instituto De Ana Rahal Guaragna Machado, Angelica Hidalgo Flores, Aline
Genética, Bogotá, Colombia; 3 Servicios Médicos Yunis Turbay y Cía S.A.S. Rodrigues Da Silva, Gabriele Zamperlini-Netto, Ana Bechara Mafra,
Bogotá D.C., Colombia., Instituto De Genética, Bogotá, Colombia; 4 HOMI Tomas Marzagão Barbuto, Lilian Cristofani, Vicente Odone Filho, Julia
Fundación Hospital Pediátrico La Misericordia, Bogotá, Colombia, Pedi- Lopes Garcia, Juliana Folloni Fernandes
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S506 of S635 ABSTRACTS

ITACI / Instituto da Criança / HC-FMUSP, Pediatric Hematology-oncology Background and Aims: Acute lymphoblastic leukemia (ALL) repre-
And Stem Cell Transplantation, São Paulo, Brazil sents the most prevalent pediatric cancer. Trough multicentric trials,
approximately 90% of all pediatric patients with ALL survive for at
Background and Aims: Introduction: Despite high rates of initial least 5 years. In the relapse scenario, allogeneic HSCT is indicated and
remission (>90%) after the first treatment, 40% of pediatric patients survival may reach 70% if the disease is in complete remission. How-
with AML will experience relapse. Allogeneic hematopoietic stem ever, post–HSCT relapses still represent the major cause of treatment
cell transplantation (HSCT) is recommended for children in 1st CR failure, with a dismal prognosis. Objective: Describe the outcomes of
(intermediate- or high-risk cytogenetics or genetic mutations) or in 2nd patients experiencing relapse after HSCT for pediatric ALL.
CR. Risk factors for relapse after transplant include status at the time Methods: We retrospectively analyzed ALL patients relapsed after
of transplant (beyond the first CR) and absence of chronic graft versus HSCT in our institution from January 2015 to September 2021.
host disease (GVHD). Overall survival after relapse is poor and there is Results: In the study period, 46 pediatric patients underwent allo-
no standard treatment in these situations. Objective: Describe the out- geneic HSCT for ALL in our institution. From these, 17 had relapse after
comes of pediatric patients with AML or MDS who experienced relapse allo-HSCT. Two patients were excluded from the analysis for loss to
after allogeneic HSCT. follow-up. Median time from transplant to relapse was 240 days (range
Methods: Methodology: Retrospective analysis of pediatric patients 30-840). Most patients (n=12) relapsing before 1 year after HSCT.
transplanted for AML or MDS who experienced disease relapse. Patients’ characteristics were divided in two tables for early (before 6
Results: From January 2015 to September 2021 30 pediatric patients months - table 1) or late (after 6 months - table 2) relapses. Most of
underwent allogeneic HSCT for AML or MDS in our institution. One the patients (92.8%, n = 13) received classic chemotherapy and 3 also
patient had active extramedullary disease at HSCT and progressed received Donor Lymphocyte Infusion (DLI), and 6 also received target
after transplant. Four patients experienced relapse between 55 days immunotherapy with blinatumomab (n = 5) or inotuzumab (n = 1). Two
and 43 months after transplant. Of these, two patients had MDS patients received a 2nd HSCT. Only 1 patient received exclusive pal-
(RAEB-T) and 2 patients had AML (1st CR and 3rd CR). Transplant liative care (very early relapse). Three patients are still alive and only
characteristics are described in table 1. One patient with MDS had one is disease-free with a good quality of life, 14 months after relapse.
poor functional clinical status and received exclusive palliative care. Twelve patients died, most from disease progression. All patients with
Two patients received were refractory to high-dose chemotherapy and early relapse died before 6 months, while patients with late relapses
died from the disease and infection. One patient relapsed as T-cell ALL survived at least two years.
de novo, received high dose chemotherapy with multiple agents but Conclusions: Survival after relapsing allogeneic HSCT is dismal, espe-
progressed just after, and died 18 months after relapse diagnosis. cially before 6 months. New therapeutic agents (immunotherapy) and
Conclusions: All patients relapsing after HSCT for AML or MDS cellular therapy (CAR-T cells and second HSCT) may change this
in our institution died from disease or toxicity. New agents (with scenario. These children should receive early comprehensive care.
less toxic profile) and cellular immunotherapy may have a role in
these very severe situations. Complementary palliative care could be
beneficial. E-Poster Topic: AS05 SIOP Scientific programme / AS05.l Rare Tumours
and Histiocytosis

E-Poster Topic: AS05 SIOP Scientific programme / AS05.d Stem Cell E-POSTER VIEWING
Transplantation
EP773 / #1835 PRIMARY CARDIAC TUMORS IN CHILDREN:
E-POSTER VIEWING A SINGLE INSTITUTION CASE SERIES

EP772 / #1938 OUTCOMES FOLLOWING RELAPSE AFTER Gabriele Zamperlini-Netto1 , Lilian Cristófani1 , Andressa Vellasco1 ,
HEMATOPOIETIC STEM CELL TRANSPLANTATION FOR Fernanda Gonçalves1 , Gabrielle Bueno1 , Roberto Teixeira1 , Vicente
CHILDREN WITH ALL: A SINGLE-CENTER EXPERIENCE Odone Filho2
1 Instituto da Criança, Pediatric Hematology Oncology, Sao Paulo, Brazil;
Ana Rahal Guaragna Machado1 , Camila Noronha Santos1 , Julia 2 ITACI – Instituto De Tratamento Do Câncer Infantil Faculdade De Medic-
Loureiro Sion1 , Karina Morikawa Morikawa1 , Gabriele ina Da Universidade De São Paulo, Departament De Pediatria, São Paulo,
Zamperlini-Netto2 , Lilian Cristofani1 , Vicente Odone Filho1 , Brazil
Alessandra Araújo Gomes1 , Juliana Folloni Fernandes1 , Julia Lopes
Garcia1 Background and Aims: Cardiac tumors (CT) are a rare condition in
1 ITACI / Instituto da Criança / HC-FMUSP, Pediatric Hematology-oncology childhood. The majority of primary cardiac tumors (PCT) are benign
And Stem Cell Transplantation, São Paulo, Brazil; 2 Instituto da Criança, (60% rhabdomyomas) and about 10% of CT are malignant. Life
Pediatric Hematology Oncology, Sao Paulo, Brazil threatening symptoms (arrhythmia and flow obstruction) and sudden
death are described. Treatment should be tailored according to the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S507 of S635

histological subtype and multiple options are reported. Recently, and reason for delay in treatment initiation were recorded and
mTOR inhibitors have been explored in patients diagnosed with rhab- analyzed.
domyomas given its high prevalence and association with Tuberous Results: Out of 224 patients treated from December,2016-May
Sclerosis (TS). 2021, 199 events of treatment delay recorded in 175patients. The
Methods: Retrospective analysis of CT in patients < 18 years old from mean age of patients was 8.6years (± 4.6, 1-19years) and 109(62%)
2013 to 2021 in a single institution. of the patients were male. Most of the patients 145(83%) were
Results: 7 patients were diagnosed with PCT. Six female and 1 male. 3/7 referred from another institute for radiotherapy at our hospital. One-
were intra-uterine diagnosed, 1/7 at birth, 3/7 after 1 year of age. Clin- fourth of patients were treated under general anesthesia. Most of
ical presentation was heart failure (3/7) and pericardial effusion/chest the patients were being treated for sarcomas 46%(81)patients, lym-
pain (1/7).Biopsy was done in 4/7 cases and pathology report was con- phomas 26%(45)patients, renal tumors 12%(21)patients, CNS tumors
sistent with germ cell tumor (2/7), hemangioma (1/7) and sarcoma 11%(20)patients, and rare tumors 5%(8 patients). Duration of treat-
(1/7). In 3/7 cases a rhabdomioma diagnosis was assumed and tis- ment delay was 11-15days in 73patients,16-20days in 72 patients
sue sample was not obtained. TS complex was confirmed in only 1 and 20-40 days in 30patients. Major reason for the delay identi-
patient. Cancer specific protocol was offered for patients with malig- fied was due to changes recommended in peer review meeting for
nant diagnosis as appropriated (yok sack tumor and sarcoma). Surgery treatment planning in 102 patients. Other reasons were complex
was the treatment choice for an immature teratoma. Steroids were planning in 35patients, treatment machine overbooked in 28patients,
used for the hemangioma treatment and sirolimus was utilized in 1/3 equipment breakdown in 19patients and nonavailability of diagnos-
rhabdomyoma. All patients are alive and no recurrence disease was tic imaging in 15patients. Peer review changes was most observed
observed. reason in 11-15days delay, imaging unavailability in 16-20days delay,
Conclusions: CT in childhood are a rare and heterogeneous group while a combination of complex planning, machine overbooking, and
of disease. Our small case series do not support a treatment rec- breakdown were most common in delay of greater than 20days.
ommendation with respect of malignant tumors, although a disease- Conclusions: The study identifies areas working on which could help
specifc approach is encouraged in the literature. Considering that early initiation of curative local treatment in children. Availability
rhabomyomas are largely the most common CT subtype, and com- of treatment machine, manpower and coordination with pediatric
monly associated with TS, mTOR inhibitor approach is a reasonable, oncologist are essential.
non-invasive option and should be considered in prospective studies
as an alternative for patients with life-threating cardiac dysfunction
rhabdomyoma-related. P002 / #464 SHOULD THE CARDIAC SUBSTRUCTURES BE
DELINEATED FOR THE RADIATION OF MEDIASTINAL
HODGKIN’S LYMPHOMA?
E-Poster Topic: AS02 Radiation Oncology - PROS
Fadoua Bouguerra, Sabrine Tbessi, Asma Selmane, Najla Attia, Semia
PROS POSTER SESSION Kanoun Belajouza, Nadia Bouzid, Samah Tebra
Farhat Hached Hospital, Radiation Therapy Department Sousse, Tunis,
P001 / #1707 IDENTIFICATION OF REASONS AND ROOM Tunisia
FOR IMPROVEMENT IN TIMELY INITIATION OF RADIATION
THERAPY AFTER SIMULATION-A SHARED CARE MODEL FROM Background and Aims: Radiation therapy (RT) in mediastinal
TERTIARY CARE HOSPITAL IN LMIC Hodgkin’s disease (MHD) exposes long survivors to late cardiac com-
plications. The aim of this study is to evaluate the doses received by
Yumna Ahmed, Maria Tariq, Ayesha Arshad Ali, Ahmed Nadeem the heart, the left ventricle (LV) and the left anterior descending (LAD)
Abbasi, Asim Hafiz, Nasir Ali, Sehrish Abrar, Bilal Mazhar Qureshi artery in case of mediastinal irradiation of Hodgkin lymphoma.
Aga Khan University, Department Of Oncology, Karachi, Pakistan Methods: Nine patients were treated for mediastinal MHD in the
radiotherapy department of the Farhat Hached Hospital, over a period
Background and Aims: Timely delivery of radiation therapy (RT) is of 6 years (2014 _2020). The LV and the LAD artery were delineated
vital to local control in many pediatric tumors. The aim of this study was retrospectively as organs at risk in the same simulation scanner on
to review the pattern and reasons of delay in initiating radiotherapy for which the RT was planned.
children treated at our institute and identify the reasons for delayed Results: The mean age was 15.3 years; the sex ratio was 4/5. The cir-
initiation of treatment. cumstance of discovery was mostly a cervical adenopathy. The patients
Methods: Hospital database was reviewed for patients treated were classified as stage II (n=5), stage III (n=2) and stage IV (n=2)
with radiation therapy on pediatric treatment protocol from respectively. All patients received ABVD or BEACOPP chemotherapy
December,2016-May,2021. Patients were identified who had followed by 3D-conformal RT of initial affected sites at a dose of 30Gy
delays in starting RT of more than 10 working days following in 15 fractions +/- boost of 6 Gy on residual sites for stage II and RT
planning CT scan. Data for demographics, diagnosis, duration of residual sites at a dose of 36Gy in 18 fractions for stages III and IV.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S508 of S635 ABSTRACTS

Delineation of cardiac substructures was performed by radiation ther- P004 / #1338 EWING SARCOMA IN CHILDREN :
apy residents with reference to the Contouring Atlas of Duanes et al. EXPERIENCE OF A TUNISIAN CENTER
Dosimetric datas were collected from the planning software. Dose
averages were for the heart (Mean Dose/Max Dose=8.4Gy/32.13Gy), Nadia Bouzid1 , Hayfa Chahdoura1 , Sabrine Tbessi1 , Imene
LAD artery (Mean Dose/Max Dose=3.44/ 18.13 Gy), and LV (Mean Chabchoub2 , Nouha Ammar2 , Semia Kanoun Belajouza1 , Samah
Dose/Max Dose= 1.88 Gy/14.54 Gy), respectively. After a mean Tebra1
follow-up of 30 months, none of our patients presented a major cardiac 1 Farhat Hached Hospital, Radiation Oncology, Sousse, Tunisia; 2 CHU Farhat

event. Hached, Medical Oncology, sousse, Tunisia


Conclusions: The new RT techniques in the treatment of mediasti-
nal MHD may lead to heterogeneity of dose distribution in the Background and Aims: Ewing sarcoma (ES) is a rare, aggressive malig-
heart; indeed, constraints based on the mean heart dose underesti- nant bone tumor that primarily affects children and adolescents. It
mate the cardiac risk associated with RT; the involvement of cardiac represents 10 to 15% malignant bone tumors and 40 to 45% pedi-
substructures influences the cardiovascular prognosis of the patient. atric malignant bone tumors.It is part of neuroectodermal tumors with
high metastatic potential. The purpose of this study was to investigate
patient characteristics, treatment strategies, and outcomes of Ewing
P003 / #1318 RADIOTHERAPY FOR NON-METASTATIC sarcoma.
WILMS TUMOR Methods: From January 2005 to June 2021, 12 patients <18 years of
age with histologically confirmed Ewing sarcoma were enrolled. Data
Nadia Bouzid1 , Rym Zanzouri1 , Sabrine Tbessi1 , Semia Kanoun were collected in the radiation therapy department at the University
Belajouza1 , Imene Chabchoub2 , Samah Tebra1 Hospital Farhat Hached, Sousse, Tunisia.
1 Farhat Hached Hospital, Radiation Oncology, Sousse, Tunisia; 2 CHU Farhat Results: The median age was 11.25 years. The sex-ratio was 1. Local
Hached, Medical Oncology, sousse, Tunisia pain was the initial symptom in 84%.A palpable mass was noted
in 33% of patients at the first visit. The diagnosis of ES was con-
Background and Aims: Wilms tumor (WT) is the most common malig- firmed by immunohistochemistry.The most frequent location was in the
nant renal tumor in children. Current results aftermultidisciplinary femur (11.5%). In relation to the axial skeleton, the spine bone was
treatment, especially for non metastatic WT, are excellent with long- the most common location affected.Initially metastatic disease was
term cure rates greater than 85%. Our aim is to describe clinical observed in only 3 cases.In the majority of our patients therapeutic
characteristics and therapeutic results of non metastatic WT in a approach was based on induction chemotherapy followed by surgery
Tunisian population. and /or radiotherapy followed by consolidation chemotherapy. Euro-
Methods: Descriptive study of 14 patients with non metastatic WT Ewing 99 protocol was the most frequently used (91.6%). Nine children
treated in the Radiation Oncology Departement of the Farhat Hached underwent 3D (5 patients) or 2D (4 patients) radiotherapy with cura-
Hospital, Sousse, Tunisia between 1995 and 2019. tive purposes (doses between 45 - 50.4Gy) and three patients with
Results: Of 24 patients with WT treated, 14 patients had localized dis- palliative purpose.Overall survival at 5 years was 90% while 5-year
ease.The median age was 54 months [18 - 216 months], with a slight progression-free survival was 38%.The prognosis in cases of Ewing’s
female predominance [sex ratio =0.55].The stage distribution of the sarcoma was mainly influenced by the presence of metastases at the
tumors was stage I in1 patient, stage II in 3, and stage III in10 patients. time of diagnosis
Treatment was based on the SIOP9 and SIOP93-01 protocols. All Conclusions: The treatment strategy for ES is characterized by multi-
patients received upfront chemotherapy followed by nephrectomy.The disciplinary collaboration. New approaches include anti-angiogenic
histology was of intermediate risk in10cases and high risk in 4. Radio- therapy, particularly since vascular endothelial growth factor is an
therapy(RT) was indicated in13 cases. two patients were lost of sight apparent downstream target of the ews-fli1 oncogene.
before RT.The median time between surgeryand RT was 18 weeks.
All patients received flank RT, the average dose was 25.9 Gy [14.4-
36Gy] with 1.6-1.8Gy/day using two-dimensional RT in 5 cases and P005 / #1365 MANAGEMENT OF PEDIATRIC
three-dimensional conformal RT in 6 cases. RT was delivered within an MEDULLOBLASTOMA IN SOUSSE : ABOUT 26 CASES
average time of 22 days. Overall tolerance was good. Local recurrence
was observed in 2 patients, those who abondonned treatment,treated Nadia Bouzid1 , Amel Chamsi1 , Sourour Fallah1 , Imene Chabchoub2 ,
by chemotherapy, surgery, and flank RT and metastatic relapse in 1 Samah Tebra1
case treated by chemotherapy and bipulmonary RT. After a median 1 Farhat Hached Hospital, Radiation Oncology, Sousse, Tunisia; 2 CHU Farhat

follow-up of 101 months; 12 patients were in complete remission and Hached, Medical Oncology, sousse, Tunisia
2 patients died.The 5 year survival rate was 85%.
Conclusions: In our population, the characteristics of WT are mostly Background and Aims: Medulloblastoma (MD) is the most common
similar to those reported in the literature. Thus, with appropriate malignant brain tumor in children,comprising 40% of all childhood
treatment, an excellent outcome can be achieved in most cases. posterior fossa (PF) tumors. It is potentially curable, and prognosis
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S509 of S635

depends of the likehood of disseminated disease at the time of diag- These 4 children presented imaging changes :T2 Flair hypersignal for all
nosis. The purpose of this study was to describe clinical characteristics, children associated with a T1 hypersignal in one case. The average
treatment strategies, and outcomes of MD. time to appear for these images was 3.25 months (range 1 month-4
Methods: We retrospectively reviewed 26 patients treated for MD in months). Dosimetric analysis of these 4 cases showed that the the vari-
the Farhat Hached Hospital, Sousse, Tunisia between 1996 and 2020. ability of RBE were incriminated in 2 patients (cases 1 and cases 4) . In
Results: Median age was 7.8 years (1-15) with a sex ratio of 1.The case 3, these images can be explained by the high dose received by the
main reason for consultation was intracranial hypertension syndrome brainstem (average dose: 50Gy).
in 13 patients with an average consultation time of 2 months.Cerebral Conclusions: In our study, the variability of RBE and the high
spinal fluid involvement was detected in 5 children.Overall,25 patients dose received by the brainstem were very likely responsible for
had gross or near-total surgical resection. It was incompleteonly in these imagingchanges. It is necessary to confirm these data by hav-
5 cases. Histologically, MD subtypes were distributed as follows :4 ing more follow-up and important series To carry out prospective
desmoplasmic,4 classic,2 with extensive nodularity and 1 anaplastic.9 studies.
infants were categorized as having high-risk disease.Radiotherapy(RT)
was indicated to all patients but only delivered to 24 at a dose of
23.4 to 36Gy (1.8Gy/fraction) to the craniospinal axis with a com- P007 / #1808 GONADAL DAMAGE FOLLOWING
plement in the PF at a dose of 54 to 56 Gy.21 patients received TREATMENT OF MEDULLOBLASTOMA IN CHILHOOOD
adjuvant Chemotherapy(CT) and 8 had concomitant RT-CT. 4 patients
kept neurological sequelae of which 2 had gait impairment,1 neu- Nadia Bouzid1 , Amel Chamsi1 , Sarra Sghaier1 , Imene Chabchoub2 ,
rocognitive disorders and 1 kept a kineticmutism. Eleven relapses Samah Tebra1
(42.3%) were observed after median time of 11 months (3-76) mostly 1 Farhat Hached Hospital, Radiation Oncology, Sousse, Tunisia; 2 CHU Farhat

localized in the PF (87.5%). Reirradiation was performed in one Hached, Medical Oncology, sousse, Tunisia
case at a dose of 15 Gy in the PF. After a mean follow-up of 39
months,11 deaths were noted,13 children were in full remission Background and Aims: Cranio-spinal irradiation (CSI) for medulloblas-
and 2 lost to follow up. Median overall survival was 22.5 months toma (MB) can impair fertility. Gonadal failure can result from either
(2-248). gonadotropin deficiency, caused by radiation to the brain or direct
Conclusions: In accordance with the literature data, our study suggest damage to the gonads following spinal radiation therapy (RT). The
a potential survival benefit from multimodal treatment in pediatric MD objective of the current study is to estimate the gonade dose received
but recurrence remains high with a rate exceeding 30%. during CSI in pediatric MB.
Methods: We studied retrospectively 5 boys and 6 girls treated
by postoperative CSI for MB in the radiation oncology depart-
P006 / #1409 IMAGING CHANGES IN PEDIATRIC ment,Farhat Hached hospital,Sousse,Tunisia between 2015 and
EPENDYMOMA PATIENTS TREATED WITH PROTON BEAM 2020.
RADIATION THERAPY Results: All patients were prepubertal at diagnosis.The mean age was
5.5 years (4-10 years) with a sex ratio of 0.8 (M/F). Tumors were classi-
Nadia Bouzid1 , Farid Goudgil2 , Stephanie Bolle3 fied into high risk (50%) and standard risk (50%) MB. After total surgical
1 Farhat Hached Hospital, Radiation Oncology, Sousse, Tunisia; removal of the tumor, each child received three-dimensional confor-
2 Protontherpy center, Radiation Oncology, Orsay, France; 3 Gustave mational RT at a dose of 23.4 Gy to 36 Gy on the cerebrospinal axis
Roussy, Université Paris-Saclay, Department Of Radiation Oncology, with boost in the posterior fossa up to 54 Gy.The maximum average
Villejuif, France dose delivered to gonads was 0.85 Gy (0.004-2.1 Gy).The maximum
mean doses delivered to testes and ovaries were respectively 0,5 Gy
Background and Aims: To describe the imaging changes in pediatric (0.1-1.7 Gy) and 1.1 Gy (0.004-2.1 Gy). The pubertal status of each
ependymomas patients treated by proton therapy and trying to find an was defined by the staging technique of Tanner and the hormone blood
explanation by reviewing dosimetry. tests.After an average time of 15.5 months (6-29 months),all 5 boys had
Methods: Descriptive study including 4 children with ependymoma normal testes and normal testosterone concentrations for their stage
treated by proton therapy at the Proton Therapy Center in Orsay of pubertal development.Two boys spontaneously progressed through
between August 2015 and June 2016 and who have developed imag- puberty.For our 6 girls, the ovaries dose estimation enabled us to be
ing changes at the MRI of control.For each case a fusion between CT counselled about future ovarian function and fertility because they
and MRI was performed. hadn’t yet reached menarche.
Results: 4 girls, aged between 18 months and 5 years (median 2 ½ Conclusions: The Dmax of gonads is 3 Gy.It was largely respected in
years). The tumor was supratentorial in 3 cases and infra tentorial in our series without impact on gonadal function temporarily.The risk of
one case. All children had surgery. The surgical time to proton ther- gonadal failure from CSI should be further reduced and possibly elimi-
apy was 41.25 days (range: 28 - 60 days). All children received proton nated if proton therapy is used because there is no scatter dose to the
therapy. The doses varied from 54 to 59.4 GyE (Median, 58 GyE). gonads.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S510 of S635 ABSTRACTS

P008 / #919 AN UPDATE OF TREATMENT OUTCOME OF Mikaela Doig1 , Eva Bezak1 , Nayana Parange1 , Peter Gorayski2 ,
PAEDIATRIC MEDULLOBLASTOMA – A SINGLE INSTITUTION Victoria Bedford3 , Michala Short1
EXPERIENCE IN SABAH WOMEN AND CHILDREN’S HOSPITAL, 1 University of South Australia, Cancer Research Institute, Adelaide, Aus-
EAST MALAYSIA tralia; 2 Royal Adelaide Hospital, Department Of Radiation Oncology, Ade-
laide, Australia; 3 Cancer Voices, Cancer Voices South Australia, Adelaide,
Audrey Chong1 , Flora Chong2 , Asohan Thevarajah3 , Jing Guo Wong4 , Australia
Wern Way Chiew2
1 Sabah Women and Children’s Hospital, Clinical Research Centre, Kota Kin- Background and Aims: Childhood cancer survivors have a risk of late
abalu, Malaysia; 2 Sabah Women and Children’s Hospital, Radiotherapy And effects secondary to treatment. Due to its unique dose deposition, pro-
Oncology, Kota Kinabalu, Malaysia; 3 Sabah Women and Children’s Hos- ton beam therapy (PBT) has the potential to reduce the incidence and
pital, Paediatric Oncology, Kota Kinabalu, Malaysia; 4 Sabah Women and severity of toxicities relative to conventional photon radiation therapy
Children’s Hospital, Paediatric, Kota Kinabalu, Malaysia (XRT), which may translate into improvements in Health-Related Qual-
ity of Life (HRQoL). This systematic review summarises the evidence of
Background and Aims: Treatment of paediatric medulloblastoma has HRQoL in childhood cancer survivors following PBT and XRT.
improved but remains a challenge in low and middle-income countries. Methods: Medline, Embase and Scopus were systematically searched.
This study aims to present the demography and treatment outcome of Studies were included if they collected HRQoL data from children
paediatric medulloblastoma at Sabah Women and Children’s Hospital diagnosed with cancer, using a validated patient-reported outcome
in East Malaysia. measure, and included patients treated with external beam radiation
Methods: This is a 7-year retrospective study. Seventeen children were therapy (PBT or XRT) after the year 2000.
identified between 2015 and 2021. Four were excluded, one due to Results: Fourteen cross-sectional, 15 longitudinal and one mixed meth-
missing records while three did not fulfil the treatment criteria. The ods study were analysed to describe 1,986 childhood cancer survivors.
data was analysed using SPSS version 25. There were minimal differences in HRQoL after XRT or PBT. HRQoL
Results: The mean age at diagnosis is 7.6 years (range from 3.1 to for children with a central nervous system tumour treated with XRT
13.2 years). 53.8% are female. Vomiting, headache and ataxia are the or PBT, captured by Pediatric Quality of Life Inventory Generic Core
commonest presenting symptoms. Median interval between symptom total score, improved with time from treatment delivery. However, the
onset and diagnosis was 7 weeks (range from 4 to 24 weeks). Six chil- change over time could not be quantified due to many studies including
dren presented with metastatic disease. Eleven children (84.6%) were participants at variable time-points following treatment, within a sin-
categorized into high risk group. Histological subtype was reported as gle cohort. No studies analysed the implementation of routine HRQoL
classic medulloblastoma for all children except for one without record assessment during pediatric radiation oncology clinical practice. No
in the histopathological report. Five children (38.5%) received radio- studies described actioning severe or declining HRQoL outcomes.
therapy within ≤5 weeks of surgical resection. Four children (30.8%) Conclusions: Based on the current available evidence, HRQoL out-
completed radiotherapy within 45 days. All children received cran- comes for children receiving XRT or PBT are similar. HRQoL outcomes
iospinal irradiation and tumour bed boost with additional spinal boost for children diagnosed with a central nervous system tumour improve
for two children, followed by chemotherapy with CH 455 Packer Proto- with time from radiation therapy. Recommendations for clinicians and
col. With a mean follow-up of 33 months (range from 12 to 64 months), researchers include the routine collection of pre-treatment HRQoL
the 3 year progression free survival rate is 76.4% ±15.5%. Two chil- baseline assessments, implementation of HRQoL assessment into clin-
dren have relapse at 15 and 36 months respectively, including one with ical practice to action poor patient outcomes, and improved reporting
delayed maintenance chemotherapy due to logistic issue. As of March of radiation dosimetry to identify the impact of prescribed dose.
2022, ten children are still living with three lost to follow-up. Hearing
impairment and hypothyroidism were noted in 7 (53.8%) and 6 (46.2%)
children respectively. P010 / #356 REPORTED DENTAL TOXICITIES AFTER
Conclusions: Our study showed good treatment outcome in children PROTON BEAM THERAPY FOR PAEDIATRIC HEAD AND NECK
treated for medulloblastoma. Optimisation of treatment delivery and CANCERS
logistic support may improve the outcome of paediatric medulloblas-
toma. However further study is needed to address these challenges as Simona Gaito1 , Emma Foster-Thomas2 , Shermaine Pan3 , Eunji
well as to assess overall survival and long term complications. Hwang4 , Peter Sitch3 , Ed Smith3 , Marianne Aznar5,6
1 The Christie Proton Beam Therapy Centre, Proton Clinical Outcomes Unit,

Manchester, United Kingdom; 2 University Dental Hospital of Manchester,


P009 / #268 HEALTH-RELATED QUALITY OF LIFE Restorative Dentistry, FH, United Kingdom; 3 The Christie NHS Founda-
OUTCOMES FOLLOWING PHOTON AND PROTON RADIATION tion Trust, Proton Beam Therapy, Manchester, United Kingdom; 4 Crown
THERAPY FOR CHILDHOOD CANCER Princess Mary Cancer Centre, Radiation Oncology, WESTMEAD, Australia;
5 University of Manchester, Division Of Clinical Cancer Science, School
Of Medical Sciences, Manchester, United Kingdom; 6 The Christie NHS
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S511 of S635

Foundation Trust, Radiotherapy Related Research, Manchester, United Results: Twelve patients were treated with surgery and adjuvant RT
Kingdom between 2011 and 2021. Mean age was 9.2 years (2-20), 50% had
tumors grade 2, 58% had supratentorial tumors, only one patient
Background and Aims: It is known from the literature that the nature (8%) presented with metastatic disease. Only 33% of the patients
and severity of dental disturbances is inversely related to a patient’s had gross total resection (GTR), mean time between surgery and
age and stage of tooth development at the time of radiotherapy. RT was 98 days (34-243), mean time of RT duration was 51 days
Despite this, dental toxicities after radiotherapy are often underre- (41-72). Of the patients without GTR, only in 2 out of 7 the resid-
ported and there is very limited published data following proton beam ual tumor disappeared after RT. Five-year EFS was 55% and OS
therapy (PBT). This service development project aimed to establish the was 71%, no variable was associated with improved outcomes. Two
reported incidence of tooth development disorders in patients treated out of 5 patients with residual tumor after radiotherapy had tumor
with PBT within the Proton Overseas Programme (POP). progression.
Methods: Clinical follow-up data of patients treated with PBT within Conclusions: Survival outcomes of our patients were acceptable com-
the POP is stored in a national database and curated by a dedicated pared to what is reported on literature, even with subtotal resections
outcomes unit at The Christie NHS Foundation Trust. A retrospec- performed in two thirds of our patients.
tive service evaluation utilising this data was conducted to evaluate
the documented assessment of dental toxicities in paediatric head and
neck (H&N) cancer patients treated in the POP. P012 / #1847 DOSE RECEIVED BY THE SCALP AFTER
Results: From 2008-2019, 195 paediatric were treated for H&N malig- RADIOTHERAPY FOR PEDIATRIC MEDULLOBLASTOMA
nancies within the POP, and toxicities grade 3+ were prospectively
recorded. Tooth development disorders were recorded by clinicians Haifa Haj Abdallah1 , Sabrine Tbessi2 , Rym Zanzouri3 , Hayfa
at scheduled outcome appointments in four patients (median follow- Chahdoura2 , Semia Kanoun Belajouza2 , Nadia Bouzid4 , Raouf
up 5 years). Of note, the permanent dentition in one patient aged 3.5 Hammouda2 , Samah Tebra4
years at the time of treatment has severe abnormal tooth develop- 1 FARHAT HACHED HOSPITAL, Radiotherapy, Sousse, Tunisia; 2 Farhat

ment first observed 3.5 years post-treatment. For this patient, mean Hached Hospital, Radiation Oncology, Sousse, Tunisia; 3 salah azaiez insti-
doses of 30Gy and 14.1Gy(RBE=1.1) were delivered to the maxilla and tut, Radiation Therapy, tunis, Tunisia; 4 farhat hached hospital, Radiation
mandible respectively, and were correlated to the dental findings. Therapy, sousse, Tunisia
Conclusions: The dose threshold for tooth development disorders
following either PBT or conventional photon radiotherapy remains Background and Aims: Medulloblastoma accounts for nearly 10%
unknown. It is recommended that the dose to teeth is kept as low of all childhood brain tumors. These tumors occur exclusively in the
as reasonably possible in younger patients, with a proposed restric- posterior fossa. Radiation therapy (RT) remains a critical component
tion of <20Gy in patients <4 years old. The findings in the pre- of multimodality treatment for medulloblastoma, wich have largely
sented patient suggest that dental disturbances can occur with doses evolved resulting in better survival rates. Nevertheless, long-term
<10Gy(RBE=1.1). Further studies evaluating the long-term effect of toxicity is a major concern in this setting.
PBT on dental development in paediatric H&N patients is encouraged. Methods: A retrospective study of 14 patients diagnosed with medul-
loblastoma in the department of radiotherapy of Farhat Hached
hospital between 2013 and 2021.
P011 / #1979 OUTCOMES OF PEDIATRIC EPENDYMOMA Results: The average age was 6 years (4-15 years) .There were 4 girls
TREATED AT A SINGLE TERTIARY CENTER IN MEXICO and 10 boys .The most common manifestations were ataxia . Diagno-
sis was confirmed by characteristic imaging on Magnetic Resonance
Josue Hernandez-Benitez, Jose Muñoz-Lozano, Celia Imaging. It was commonly presented as midline masses in the roof of
Gonzalez-Alcorta, Fernando Alcorta-Nuñez, Jose Arenas-Ruiz the 4th ventricle with associated mass effect and hydrocephalus .The
University Center Against Cancer, Radiotherapy, Monterrey, Mexico average tumor size was 43 mm (17-55 mm) .Five patients had pre-
sented bone marrow enhancement related to metastasis, wich were
Background and Aims: Ependymoma is one of the most frequent pri- confirmed by a lumbar puncture. Nine patients had ventriculoperi-
mary brain tumors in children. Gross total resection is associated with toneal shunt placement. All patients underwent surgery. The excision
improved survival outcomes. Adjuvant radiotherapy (RT) is considered was complete in 73% of cases . Almost all patients were treated with
for most cases. concurrent chemotherapy .All patients were treated with conformal
Methods: We performed a retrospective review of pediatric patients RT.The median RT dose was 54 Grays (30-54Gy) in the posterior fossa,
treated last decade at our center for intracranial ependymoma with and 36Gy (23.6-36Gy) in the craniospinal axis . The average RT dose
the objective to describe 5-year event free survival (EFS) and overall received by the scalp was 34 Gy (32-38Gy).Acute alopecia was pre-
survival (OS). Variables included on analysis were: Tumor grade, tumor sented in 50% of cases commonly localized in the occiput.It was grade
location, extent of resection, time between surgery and RT, RT duration 3 in half of the cases. After a median follow up of 10 months (4-
and extent of residual tumor after RT. 96 months),10 patients was in complete remission, with persistent
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S512 of S635 ABSTRACTS

alopecia in 2 cases.Four patients had metastasic progression,3 of them Angela Mastronuzzi4 , Antonio Ruggiero5 , Maria Antonietta
died. Gambacorta2,3 , Vincenzo Valentini2,3 , Mario Balducci2,3 , Silvia
Conclusions: Cranial irradiation for medulloblastoma can causes Chiesa2 , Daniela Pia Rosaria Chieffo1
inevitable alopecia, wich can affect the survived children’s self 1 Fondazione Policlinico A. Gemelli IRCCS, Clinical Psycology Unit, rome,
image.Therefore we suggest to contour the scalp as an organ at risk to Italy; 2 Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Onco-
minimize the dose received by it. logic Radiotherapy, Department Of Diagnostic Imaging, Oncologic Radio-
therapy And Hematology, Rome, Italy; 3 Università Cattolica del Sacro
Cuore„ Istituto Di Radiologia, Facoltà Di Medicina E Chirurgia, rome, Italy;
P013 / #1957 PLACE OF RADIOTHERAPY IN THE 4 Ospedale Bambino Gesù, Pediatric Oncology, Rome, Italy; 5 Fondazione
TREATMENT OF HIGH RISK NEUROBLASTOMA Policlinico Universitario A. Gemelli IRCCS, Pediatric Oncology, Department
Of Woman And Child Health And Public Health, Rome, Italy
Sara Jdii, Majdouline Houjami, Tarik Chekrine, Zineb Bouchbika, Nadia
Benchakroun, Nezha Tawfiq, Hassan Jouhadi, Souha Sahraoui, Background and Aims: Several studies have shown a significant rela-
Abdellatif Benider tionship between healthcare satisfaction and health-related attitudes
University center IBN ROCHD, Oncology Radiotherapy, Casablanca, and behaviours. The assessment of parental satisfaction is assum-
Morocco ing an increasingly important role also in paediatric oncology. In our
Radiotherapy department a Multi-professional Group (MPG) has been
Background and Aims: Neuroblastoma is the most common extracra- established. MPG aimed to a multidimensional assessment of pedi-
nial solid tumor in children, accounting for approximately 8% of all atric patients (PP) and introduced specific action, such as psychological
childhood cancers and 15% of childhood cancer mortality. We shared preparation and support, and specific tools (audiovisual, digital, etc.)
here our experience, in the treatment of children with high-risk during radiotherapy care-path. MPG felt the need to measure parents’
neuroblastoma treated with radiotherapy. This work study is as an health satisfaction. After elaborating the Italian translation of the Ped-
overview of the current treatment, for high-risk neuroblastoma and a sQL™ module for health satisfaction in Oncology/Hematology, they
glimpse at current research for future therapy. started to administer it to parents during their child’s radiotherapy
Methods: We carried out an analytical descriptive retrospective study, treatment.
between January 2008 and September 2019, concerning 165 cases Methods: The questionnaire consists in 25 item scale grouped in
of neuroblastoma, 32 patients had high-risk neuroblastoma indicating 6 domains: General Satisfaction, Information, Inclusion of Family,
radiotherapy, with a minimum of two years of follow-up. Communication, Technical Skills, Emotional Needs. A 5-point Likert
Results: Our patients received an induction phase of intensive responses scale is utilized, from 1 (Very dissatisfied) to 5 (Very satis-
chemotherapy according to the national protocol, followed by surgery fied). Linearly transformed to a 0-100 scale was performed, then mean
when possible, then a consolidation phase consisting of high-dose score was reported by dimension. The questionnaire was delivered in
chemotherapy with stem cell transplantation, external radiotherapy, the last radiotherapy week by a physician not involved in MPG.
and a final maintenance phase. In our series the radiation dose was Results: Fifteen parents were recruited and the return rate of the ques-
21.6 Gy. All patients were irradiated by conventional fractionation. At tionnaire was optimal. For all domains the mean score was very high
the end of the treatment 22 patients were in general response, four with a mean value >85: mean score for General Satisfaction was 94,
patients failed, three patients lost to follow-up at the end of radiother- for Information was 85, for Inclusion of Family was 87, for Commu-
apy and three patients were in local response but with the persistence nication was 88, for Technical Skills was 88 and for Emotional Needs
of metastases. After a decline of twenty four months, the overall sur- 90.
vival rate was fifty eight percent. The median survival of our series was Conclusions: These preliminary results demonstrate that an assess-
twenty months, and the relapse-free survival at twenty-four months, ment of parental satisfaction could provide valuable information on the
was ten percent. children’s radiotherapy pathway and help clinicians to improve quality
Conclusions: Current research is focusing on further intensification standards of care.
of therapy to improve outcomes and evaluating the role of precision
medicine in this patient population.
P015 / #1387 THE DREAMS CHEST PROJECT EXPERIENCE:
TOKEN ECONOMY FOR INCREASING COMPLIANCE IN
P014 / #941 PILOT STUDY OF THE ITALIAN VERSION OF PEDIATRIC RADIOTHERAPY
THE PEDSQL™ HEALTHCARE SATISFACTION
HEMATOLOGY/ONCOLOGY MODULE WITH PARENTS OF Elisa Marconi1,2 , Francesco Beghella Bartoli1 , Elisa Meldolesi1 , Giulia
CHILDREN UNDERGOING RADIOTHERAPY Panza1 , Loredana Dinapoli2 , Annalisa Serra3 , Giuseppe Maria Milano3 ,
Angela Mastronuzzi3 , Antonio Ruggiero4 , Daniela Pia Rosaria
Elisa Marconi1,2 , Giulia Panza2 , Alessia Nardangeli2 , Elisa Meldolesi2 , Chieffo2 , Maria Antonietta Gambacorta1 , Vincenzo Valentini1 , Mario
Francesco Beghella Bartoli2 , Silvia Di Franco3 , Antonella Cacchione4 , Balducci1 , Silvia Chiesa1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S513 of S635

1 Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Oncologic Rome, Italy, Surgical Oncology Unit – General And Thoracic Surgery Unit,
Radiotherapy, Department Of Diagnostic Imaging, Oncologic Radiotherapy Department Of Surgery, Rome, Italy; 5 Bambino Gesù Childrens’ Hospital,
And Hematology, Rome, Italy; 2 Fondazione Policlinico A. Gemelli IRCCS, Department Of Pediatric Hematology And Oncology And Of Cell And Gene
Clinical Psycology Unit, rome, Italy; 3 IRCCS Bambino Gesù Children’s Therapy, Scientific Institute For Research And Healthcare (irccs)„ Roma,
Hospital, Department Of Paediatric Haematology/oncology, Rome, Italy; Italy; 6 Bambino Gesù Children’s Hospital IRCCS, Surgical Oncology Unit –
4 Fondazione Policlinico Universitario A. Gemelli IRCCS, Pediatric Oncology, General And Thoracic Surgery Unit, Department Of Surgery, Rome, Italy
Department Of Woman And Child Health And Public Health„ Rome, Italy
Background and Aims: The lung is a common site of malignancy
Background and Aims: Radiotherapy (RT) has become an important metastasis; while metastasectomy has long been the standard of care
treatment modality in pediatric oncology, but its delivery to young chil- for many tumor types, many patients are not surgical candidates due
dren with cancer is challenging and general anesthesia is often needed. to disease burden, lesion location, or general patient state. The devel-
The efficacy of psychoeducational interventions to increase treatment opment of less invasive methods of local therapy has allowed a higher
adherence and compliance of the pediatric patient in radiotherapy has proportion of patients with pulmonary metastases to receive local
now been widely demonstrated in the literature, with a reduction in the therapy. However, the use of this approach in pediatric patients has
need for anesthesia and a consequent significant reduction in costs for never been documented. The aim of our study was to assess if per-
the national health system. In our center, a dedicated multidisciplinary cutaneous CT-guided cryoablation is technically feasible and clinically
team aims to increase children’s confidence in radiotherapy staff, space, effective to treat pulmonary metastasis in children and young adults.
and procedure, and consequently reduce the frequency of sedation. Methods: Patients with lung metastasis treated with CT-guided
Methods: Among the psychosocial interventions carried out, specific cryoablation from January 2019 to January 2022 were reported. The
for the ages, we started the project "the Dreams Chest". The project procedure was performed with two cycles of 10 minutes each from -
involved all the pediatric patients in radiotherapy and offer them the 20◦ C to -40◦ C core temperature, followed by 5 minutes of thawing.
opportunity to choose and receive a present on the last day of their Demographic data, radiological and clinical issues were collected.
therapy. The project is based on the rationale of the token economy Results: Five patients (median age: 12.88 years; range 9.43-24.01
method, where today’s radiotherapy session is considered a "token" to years) with pulmonary metastasis treated with CT-guided cryoablation
reach the treasure. were included in the study. Two Ewing sarcomas (one of the rib and one
Results: More than 400 children expressed their dreams through this of the leg), one Wilms tumor, one embryonal sarcoma of the limb, and
project, thanks to a large number of donors and large and small compa- one clear-cell sarcoma of the foot were the initial malignancies. Three
nies who paid for the gifts. The project has proved to be a useful tool lung metastases were found in the latter patient. Seven total lesions
for engaging patients from the first visit, and also to create a commu- were treated with CT-guided cryoablation. After median 6.53 months
nity that supports children and their families. In this way also the value follow-up (range 0.77-11.306 months), six lesions presented dimen-
of the gift, even if with a fixed budget of 40 euros, becomes a personal sional reduction (median 40.88%; range 20.92-60.47%). The patient
value of a co-created path. This contributed to a 6% reduction in seda- with embryonal rhabdomyosarcoma developed metastasis progression
tion, which corresponds to an average savings of more than 45000€ in (35.71% of dimensional increase); additionally, unlike all other metasta-
2021. sis, this lesion had a ground-glass halo. No major or minor complications
Conclusions: The token economy project is an economically sustain- were reported.
able method that can help increase adherence to radiotherapy in Conclusions: Regardless of the histology and clinical features of the
pediatric patients and reduce the use of anesthesia, resulting in lower primary tumor, percutaneous CT-guided cryoablation is a safe, mini-
healthcare costs. mally invasive approach for treating lung metastases in children and
young adults.

P016 / #1356 EARLY REPORT ON FEASIBILITY AND


EFFICACY OF PERCUTANEOUS CT-GUIDED CRYOABLASTION P017 / #1718 DOSIMETRIC IMPACT OF TUMOR TREATING
OF PULMONARY METASTASIS IN CHILDREN AND YOUNG FIELDS TRANSDUCER ARRAYS ON CONCURRENT RADIATION
ADULTS THERAPY FOR PEDIATRIC BRAIN TUMORS

Cristina Martucci1 , Giulia Cassanelli2 , Gian Luigi Natali3 , Alessandro Enzhuo Quan1 , Eun Han1 , Christine Chung2 , Tina Briere1 , Zsila
Crocoli4 , Giuseppe Maria Milano5 , Maria Debora De Pasquale5 , Sadighi3 , Susan Mcgovern4
Giorgio Persano6 , Guglielmo Paolantonio3 , Alessandro Inserra1 1 MD Anderson Cancer Center, Radiation Physics, Houston, United States
1 Bambino Gesù Children’s Hospital IRCCS, Rome, Italy, General And Tho- of America; 2 Sutter Health, Radiation Oncology, Berkeley, United States of
racic Surgery Unit, Department Of Surgery, Rome, Italy; 2 Bambino Gesù America; 3 MD Anderson Cancer Center, Pediatrics, Houston, United States
Children’s Hospital, Interventional Radiology Unit, Rome, Italy; 3 Bambino of America; 4 MD Anderson Cancer Center, Radiation Oncology, Houston,
Gesù Children’s Hospital IRCCS, Rome, Radiology Unit, Department Of Diag- United States of America
nostic Imaging, Rome, Italy; 4 Bambino Gesù Children’s Hospital IRCCS,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S514 of S635 ABSTRACTS

Background and Aims: Previous results suggest that tumor treating children had shunt placed. After the surgery endocrine deficits and
fields (TTF) concurrent with radiotherapy (RT) for glioblastoma yields visual disturbances were present in 72%&57% patients,respectively.
acceptable dosimetry in adults; the impact of TTF on RT dose distribu- Radiotherapy (RT) was applied in 38 children (in 40% in primary treat-
tion in children is unknown. This study was undertaken to evaluate the ment). Stereotactic RT was conducted in 13 cases with median fraction
dosimetric impact of TTF transducer arrays on concurrent photon RT and total dose of 6Gy&15Gy,respectively. Conventional RT was applied
for children with brain tumors. in 25 cases (median total dose of 54Gy) combined in 4 patients with 5-
Methods: CT scans of an anthropomorphic pediatric head phantom 6Gy stereotactic boost. Complications after RT were diagnosed in 13
(approximately 15-year-old) and an infant-head-sized spherical phan- cases with the most common endocrine deficits (in 4),vasculopathies
tom were acquired with and without the arrays. For an infratentorial (3) and secondary tumors (in 2 cases). Median follow-up from the date
tumor, clinical targets and a VMAT treatment plan (54Gy/50Gy to gross of diagnosis was 13 years. During that time 6 patients died and 5-
tumor volume [GTV]/clinical tumor volume [CTV] in 30 fractions) were ,10-&15-years overall survival was 97%,92%&92%,respectively. The
transferred to the phantoms CT sets. For a supratentorial tumor, treat- only factor found to have positive impact on the survival was the
ment plans were created (60Gy/50Gy to GTV/CTV in 30 fractions) for lack of endocrine deficits before the first surgery(p=0.006). The out-
simulated targets on the phantom CT sets. For each tumor location, come of RT was evaluated in 35 cases with stagnation and partial
dose distributions were computed and compared with and without the regression observed in 19&16 cases, respectively. Progression after
arrays. Target coverage metrics were compared, and skin dose was RT was diagnosed in 9 cases and median progression free survival
measured with thermoluminescent and film dosimeters when the same (PFS) was 7.1year. Five-&10-years PFS was 85%&71%, respectively.
plan was delivered with and without the arrays. Repeated RT was applied as part of recurrence treatment in case of 5
Results: For the infratentorial tumor, the arrays reduced CTV D95 children.
by 0.48% and 1.04%, and GTV D95 by 0.31% and 1.41% for the Conclusions: Surgery combined with RT provides satisfactory long-
two phantoms respectively; they increased skin Dmean by 1.10% and term outcome in majority of craniopharyngioma patients. The impact
4.09% respectively from planning study. For the supratentorial tumor, of endocrine deficits and the time of their onset on survival requires
the reduction in target coverage was all <1.0%. Electrodes under the further studies.
direct beam path increased skin dose by an average of 43.3% (0.3Gy –
20.7Gy), but all skin dose measurements stayed within tolerance.
Conclusions: The dosimetric impact of TTF on pediatric head phan- P019 / #1093 MULTIDISCIPLINARY TEAM EXPERIENCE OF
toms receiving concurrent RT resembles that reported in adult studies. 39 VERSUS 13 FRACTIONS OF RADIOTHERAPY FOR
Although the tumor dose is not significantly affected, the skin dose TREATMENT OF DIFFUSE MIDLINE GLIOMA AT A NATIONAL
notably increases due to the bolus effect from the TTF electrodes, REFERRAL CENTRE
which may be mitigated by skin-sparing planning and shifting of the
device during RT. Trung Nguyen1 , Charlotte Betteridge2 , Liz Clark3 , Anna Glacken3 ,
Kristy Cody4 , Claire Hardy4 , Abu Sidhanee5
1 University College London Hospitals NHS Foundation Trust, Children And
P018 / #903 THE ROLE OF INTERDISCIPLINARY Young People’s Cancer Services, London, United Kingdom; 2 University Col-
MANAGEMENT OF CHILDREN WITH CRANIOPHARYNGIOMA lege London Hospitals NHS Foundation Trust, Paediatric Occupational
Therapy, London, United Kingdom; 3 University College London Hospitals
Aleksandra Napieralska1 , Ryszard Sordyl2 , Marek Mandera2 , NHS Foundation Trust, Speech And Language Therapy, London, United
Sławomir Blamek1 Kingdom; 4 University College London Hospitals NHS Foundation Trust, Chil-
1 Maria Sklodowska-Curie National Research Institute of Oncology Gliwice dren And Young People’s Radiotherapy And Proton Beam Therapy, London,
Branch, Radiotherapy Department, Gliwice, Poland; 2 Medical University of United Kingdom; 5 University College London Hospitals NHS Foundation
Silesia, Department Of Pediatric Neurosurgery, Katowice, Poland Trust, Paediatric Physiotherapy, London, United Kingdom

Background and Aims: An analysis of the outcome of children with Background and Aims: The prognosis of diffuse midline glioma (pre-
craniopharyngioma. viously diffuse intrinsic pontine glioma) remains poor, with a 5-year
Methods: A retrospective analysis of patients below 21 years old survival rate of less than 1%. Conventional radiotherapy remains the
treated due to craniopharyngioma in years 1971-2018 was performed. mainstay of treatment, with a total radiation dosage of 5,400 cGy
Standard statistical tests were used to the tumour, delivered in 30 fractions (30#) over 6 weeks, consid-
Results: A total number of 41 patients (median age of 12 years old)was ered standard. However, hypofractionated radiotherapy, with 3,900
evaluated. The most common symptoms were headaches(63%), nau- cGy delivered in 13 fractions (13#) over 2.5 weeks, is increasingly being
sea/vomiting(37%), visual disturbances(44%) and endocrine disor- used. This study aimed to explore the experiences and perceptions of a
ders(20%). Surgery was primary treatment in 40 patients, however paediatric oncology multidisciplinary team (MDT) at a national refer-
only in 20 it was the only one neurosurgical intervention. Two,three and ral centre for radiotherapy and explore opportunities and challenges of
five surgeries were conducted in 12,6&2 cases, respectively. Thirteen the two treatment options.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S515 of S635

Methods: A focus group representative of the MDT including med- non-hematological toxicity grade≥3 occurred in 6 patients (radio-
ical, nursing, and allied health professionals was formed. The group dermatitis, central venous catheter infection, febrile neutropenia,
identified opportunities and challenges of both treatment options from Clostridioides difficile, sepsis and hypernatremia). The hematologic
their own professional perspectives. Themes were further identified, toxicity grade≥3 was related to concomitant chemotherapy, cran-
and a survey was created to understand the extent of MDT consen- iospinal irradiation or both. During the pandemic, 8 COVID19 infec-
sus regarding opportunities and challenges of both treatment options tions were diagnosed, 6 asymptomatic and 2 mild symptoms. In 3
using a Likert-type scale. patients the start of radiation was delayed. However, in 2 patients
Results: Survey responses demonstrated strong consensus across the with high-risk CNS tumors treated with chemotherapy and radiation,
themes identified by the focus group. Respondents felt 30# allowed treatment was not discontinued. The median follow-up was 6.4 months
more opportunities and greater flexibility for MDT input to facilitate (range, 0-21.3 months). Current status of the patients: alive with-
quality intervention, and provided opportunities to develop trust and out disease 68.8%; active treatment 17.2%; alive with disease 9.7%;
rapport with patients and their caregivers. However, longer hospi- deceased 4%.
tal admissions had emotional impacts on patients, their families, and Conclusions: The opening of the Proton Therapy Unit has allowed
health professionals. 13# was felt to result in lesser burden on patients access to this type of precision radiotherapy in Spain, favouring com-
and families. However, it often resulted in reduced opportunities to munication between Pediatric oncology institutions and avoiding inter-
facilitate adequate rehabilitation and to manage certain aspects of the national travel. The COVID19 pandemic did not affect the treatment of
patient’s holistic care. these patients.
Conclusions: Both treatment options offered differing opportuni-
ties and challenges. Given it is generally accepted both treatment
options have comparable results, decisions regarding which option to P021 / #1487 CONTRALATERAL LENS DOSE IN CURATIVE
take should carefully consider the opportunities and challenges. This RADIATION TREATMENT PLANS FOR ORBITAL AND
study identified only professional perspectives and further research is PARAMENINGEAL RHABDOMYOSARCOMA – A SIGLE CENTER
needed to understand perspectives of children and their caregivers. EXPERIENCE

Yumna Ahmed1 , Ahmed Nadeem Abbasi1 , Maham Khan1 , Tooba Ali1 ,


P020 / #1497 TWO-YEAR EXPERIENCE OF A NEW PROTON Asim Hafiz2 , Bilal Mazhar Qureshi1
FACILITY IN MADRID TREATING PEDIATRIC CANCER PATIENTS 1 Aga Khan University, Department Of Oncology, Karachi, Pakistan; 2 Aga
DURING THE COVID PANDEMIC Khan University, Department Of Oncology, Kaachi, Pakistan

Elena Panizo1 , Alvaro Lassaletta2 , Marta Diaz-Villapalos2 , Javier Background and Aims: Radiation therapy is a curative local treatment
Aristu2 , Javier Serrano2 , Jacobo Palma2 , Mauricio Cambeiro2 , Felipe for parameningeal rhabdomyosarcoma (RMS) with good outcome but
Calvo2 can have long term effects due to radiosensitivity of normal structures
1 Clinica Universidad de Navarra, Department Of Pediatrics, Pamplona, like lens. Our aim is to evaluate dose distribution to contralateral lens
Spain; 2 Clinica Universidad de Navarra, Radiation Oncology, Madrid, Spain patients with paramenigeal or orbital RMS treated at our institute.
Methods: Institutional record was searched for children treated with
Background and Aims: We summarize our experience treating Pedi- radiotherapy between January,2009 till May,2021 for non-metastatic
atric cancer patients in the Clínica Universidad de Navarra Proton RMS of orbit or parameningeal primary. Radiation treatment plans
Facility since its opening in May 2020, in the midst of the COVID19 were reviewed for dose distribution to contralateral lens. Mean lens
pandemic. dose was reviewed and tolerance dose criteria (<6Gy) was evaluated.
Methods: We retrospectively review the charts of the Pediatric All plans were peer reviewed according to departmental protocol.
patients treated with proton therapy at our institution. Treatment site, total dose prescribed, treatment volume crossing mid-
Results: Between May 2020 and April 2022, 96 patients received line or not and planning technique (3DCRT or IMRT) were reviewed.
treatment (46 males). Median age was 8.4 years (range, 1.2 – 19.4). Results: A total of 59patients of RMS were identified. The mean
Forty-nine percent required anaesthesia. The median number of days age was 6.8years (1–18years) with 41(69%)males. A total of
elapsed between the simulation and the start of the treatment were 44patients(75%) were treated for parameningeal RMS with dose
13.5 days (range, 6-49). The most frequent tumours were: 66% cen- ranging from 37Gy-56Gy while 15(25%) patients received RT to orbit
tral nervous system; 23% sarcomas; 4% head and neck tumors, among only with dose ranges of 36-56Gy. Average Dmax of contralateral
others. Twenty-five patients received radiation at relapse (7 of them lens was 4.7Gy in patients treated for orbital RMS and 5.3Gy for
received re-irradiation). Treatment modality consisted in focal radia- patients with parameningeal RMS. A total of 23(39%) patients were
tion (69%), craniospinal (27%), whole ventricular (3%), and holocranial planned with 3DCRT and 36(61%) with IMRT both having average
(1%). Median dose was 54 Gy (range, 15-72Gy) focal, and 36 Gy Dmax of 4.7Gy for contralateral lens. Average Mean dose received
(range, 18- 36 Gy) craniospinal. Thirty-six patients received concomi- by contralateral lens in 3DCRT was 1Gy(0.57-3.4Gy) and 6Gy(0.3-45
tant chemotherapy. All patients completed their treatment plan. The Gy) with IMRT. Treatment volume was crossing midline in 27(79%)
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S516 of S635 ABSTRACTS

patients with IMRT and 7(21%) patients with 3DCRT. All the patients P023 / #394 REIRRADIATION (RE-RT) FOR HIGH-GRADE
met tolerance dose criteria except 2 patients whose treatment volume GLIOMA (HGG) IN A 10 YEAR-EXPERIENCE
was crossing the midline.
Conclusions: Our study has shown that mean dose distribution to con- Sabina Vennarini1 , Francesca Colombo1,2 , Francesco Barretta3 , Emilia
tralateral lens was found to be lower with 3DCRT and depends on Pecori1 , Veronica Biassoni4 , Manila Antonelli5 , Ombretta
treatment volume crossing midline. We recommend each patients plan Alessandro1 , Elisabetta Schiavello4 , Luna Boschetti4 , Lorenza
should be individually evaluated with peer review by physician and Gandola1 , Maura Massimino4
physicists for planning with 3DCRT or IMRT technique. 1 Fondazione IRCCS Istituto Nazionale dei Tumori, Pediatric Radiother-
apy, Milano, Italy; 2 University of Milano, Oncology And Hemato-oncology,
Milano, Italy; 3 Fondazione IRCCS Istituto Nazionale dei Tumori, Medical
P022 / #1904 SERVICE PROVISION GAPS IN PEDIATRIC Statistics, Biometry And Bioinformatics Unit, Milano, Italy; 4 Fondazione
RADIOTHERAPY DURING COVID 19 PANDEMIC AT A IRCCS Istituto Nazionale dei Tumori, Pediatric Unit, Milan, Italy; 5 Sapienza
TERTIARY CARE UNIVERSITY HOSPITAL IN PAKISTAN University, Radiological, Oncological, And Anatomo-pathological Sciences,
Roma, Italy
Maria Tariq, Mariam Hina Ghufran, Yumna Ahmed, Bilal Mazhar
Qureshi Background and Aims: Recurrence rate for paediatric/adolescent
Aga Khan University, Radiation Oncology, Department Of Oncology, HGGs exceeds 80%. Radiotherapy(RT) remains the mainstay of
Karachi, Pakistan relapse treatment for symptoms palliation and disease progression
delay.
Background and Aims: The aim of this study was to review the ser- Methods: We re-evaluated charts, including MRI and RT plans,
vice provision delays in radiation therapy for children treated at a of 21 relapsed HGG patients, accrued 2010-2021, not DIPG/not
tertiary care university hospital in a developing country, and identify included in open trials/not secondary, aged under 18 years. All had
the reasons for those gaps. surgery (CR+PR in 14) before RT+chemotherapy(14 temozolomide,5
Methods: Hospital database was reviewed for childred irradiated dur- nimotuzumab+vinorelbine,2 sequential/HD-CT). Two had metastases,
ing covid-19 pandemic (March 2020 – October 2021),. Patients who 3 gliomatosis, 1 two non-contiguous tumors; 10 glioblastoma,5 grade
had gap of one or more day in radiation planning and delivery were 3 HGG,5 diffuse-midline-glioma(DMG)H3.3 mutated, 1 not-defined
identified and analyzed. HGG(all centrally reviewed).
Results: 128 pts were treated with mean age of 9.25 years(±5.41) Results: Fifteen had first RT on tumor bed(54 Gy), 4 whole brain(34-
of which 68%(n=87) were male and 40%(n=51) were treated 39.6 Gy), 1 craniospinal-CSI(35.2 Gy) and 1 on two sites(54 Gy).
under general anesthesia. Most common diagnoses included Median time to relapse/death were 11.5/23.5 months: relapse was
sarcomas(n=32), lymphomas(n=29), renal tumors (n=24) and local in 12(7 marginal),4 disseminated,5 local+disseminated. Re-
CNS(n=14).Simulation was delayed in 27 patients. The common- RT obtained 8 SD,1 PR,1PsPD,1 mix response,10 PD; neurological
est reason was fever/neutropenia in 37%(n=10), social reasons in signs/symptoms improved in 8, were stable in 3. Local reRT was
30%(n=8), anesthesia denied due to chest congestion in 26%(n=7)and given to 12(19.8-30.6 Gy), followed by 6 local(2 marginal) and 4
covid positivity in 7%(n=2).Planning scan was cancelled in 16patients local+disseminated second relapses in 10/12 re-evaluated. The
commonest reason being logistic in 50%(n=8), fever/neutropenia in 4 with dissemination had 1 whole brain(30 Gy),2 CSI(30.6-39.6
25%(n=4), unavailability of laboratory tests in 19%(n=3) and one was Gy),1 spine(39.6 Gy)RT and further relapsed with dissemination(2) and
lost to follow-up. 45/127 (35%)patientsbeing planned for radiotherapy locally+dissemination(1) in 3/four assessed. Five locally+disseminated
had cancellation at start of treatment delivery. Majority cancellations tumors had 3 CSI(23.4-39.6), 1 spine(39.6) and 1 extended local(30
were due to fever/neutropenia in 28%(n=13), logistic reasons in Gy) RT, further progressing locally(2),disseminated(1), psPD(1),
24%(n=11), covid positivity in 23%(n=10), 8%(n=4) each due to n.a.(1). Three had a third RT; three were alive at 19.4,29 and 50.3
covid-PCR not done and anesthesia denied due to chest congestion. months after diagnosis. Median times to progression/survival
On-treatment delays of 125 irradiated patients were recorded.44 after re-RT were 3.7 months(0.6-16.2 months)/6.9 months(0.6-
patients had treatment gaps of which, 1-2day gap was identified in 17.9 months), significantly better for longer interval after 1st RT.
7% (n=3), 3-7day gap in 32% (n=14) patients, while a gap of 8days or Sex, grade and DMG diagnosis did not impact PFS/OS after re-
more occurred in treatment of 57% (n=25) patients. Major reasons RT. In this selected series of only relapsed patients 4/5 patients
were fever/neutropenia in 48%(n=21), anesthesia denied due to chest with CR vs 3/11 noCR/noM+ after surgery had a component
congestion in 29.5%(n=13), social reasons in 13.6%(n=6), machine of dissemination at relapse(P 0.038). No radionecrosis was
breakdown in 4.5%(n=2). appreciated.
Conclusions: This study identifies barriers to care and identifies areas Conclusions: This is the biggest series of re-RT in HGG. A
we need to work upon in this pandemic to provide continuous care to randomized question could answer at reirradiation field exten-
children with cance. Uninterrupted radiaiton treatment is required for sion and better fractionation for efficacy and quality of life
achieving adequate local control as part of overall treament plan. improvement.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S517 of S635

P024 / #1944 HEAD AND NECK RHABDOMYOSARCOMA IN Bushnak3 , Neameh Farhan3 , Adel Alhmouz3 , Khaldoun Alshorman3 ,
A LOW MIDDLE INCOME COUNTRY Donya Qattan3 , Viqaruddin Mohammed1
1 King Faisal Specialist Hospital & Research Centre, Riyadh, Pediatric Hema-

Semia Zarraa1 , Safia Yahiaoui1 , Feryel Letaief2 , Hajer Ben Mansour2 , tology / Oncology, Riyadh, Saudi Arabia; 2 King Faisal Specialist Hospital
Ghaiet El Fide Noubbigh1 , Chiraz Nasr1 , Amel Mezlini2 & Research Center, Pediatric Oncology, Riyadh, Saudi Arabia; 3 King Faisal
1 SALAH AZAIZ INSTITUTE, Radiotherapy, Tunis, Tunisia; 2 Salah Azaiez Specialist Hospital & Research Centre, Riyadh, Nursing, Riyadh, Saudi
Institute, Pediatric Oncology Department, tunisia, Tunisia Arabia

Background and Aims: Rhabdomyosarcoma (RMS) is a soft tissue Background and Aims: Cancer patient experience series of events
mesenchymal tumor that accounts for 5% of all pediatric solid tumors. influencing overall well-being of the patient and families. Pediatric
Thirty percent are head and neck RMS. The treatment is multidisci- Hematology Oncology department at King Faisal Specialist Hospital
plinary, it is based on chemotherapy, surgery and radiotherapy. The & Research Centre, Riyadh serve as tertiary care referral center for
Treatment depends on the tumor site.Objective: Discuss through the cancer care and stem cell transplant. Our aim is to ensure access to
data of our series the clinical and evolutionary profile and the ther- uninterrupted cancer care during COVID-19 pandemic.
apeutic results of pediatric RMS in particular in Tunisia wich is a Methods: Integrated Practice Unit (IPU) covering cancer healthcare
lowmiddle income countris with dificuly acces to radiotherapy. delivery adjusted during the pandemic and Hospital-metrics analysis
Methods: This is a retrospective observational series of 32 children and patient satisfaction outcomes comparing year 2020 (COVID-19
with RMS collected and treated between 1993 and 2017 at the Salah outbreak) to 2021 (living with COVID-19) reported
Azaiz Institute (ISA) in Tunisia. Results: Pediatric oncology IPU adjusted in response to the COVID-19
Results: There were 32 cases. median age was 5.5 years. The predom- outbreak in 2020 through daily bed and operating room huddle and
inant revealing symptoms were: tumor syndrome (62.5%), proptosis weekly interdisciplinary care rounds with prioritization of cases and
(22%) and cranial nerve palsy (15.6%). The diagnosis was confirmed procedures to facilitate uninterrupted therapy as pandemic evolves.
by surgical biopsy in 84.3% of cases. It was an embryonic RMS in 75% An overall increase in new cases by 15% (544 cases in 2021 vs.471
and alveolar in 21.9% . The location was orbital in 34.4% of cases cases in 2020), with 84% increase in outpatient productivity (16856
and parameningeal in 21.9%. Twelve percent of patients had lymph visits in 2021 vs. 13133 visits in 2020), while an overall inpatient pro-
node involvement. All patients were non-metastatic. 93.5% received ductivity increased by 26% with admissions increased by 32% (1334
chemotherapy. Thirty-one percent of patients had surgery, 60% with in 2021 vs.1009 in 2020), inpatient days increased by 36% (1366 days
a complete resection, 10% with R1 resection and 30 with R2 resec- in 2021 vs. 1007 days in 2020) and bed occupancy increased by 15%
tion. RT was delivered in 56.3% of cases at a median dose of 52 Gy (96% in 2021 vs. 81% in 2020), however Average Length of Stay (ALOS)
(40-55Gy) with normfractionation. Forty-six percent of patients expe- reduced by 12% (13.6 days in 2021 vs. 15.5 days in 2020). Patient satis-
rienced a relapse after an average time of 7.3 months, 53.3% with faction improved by 5% with a major difference in inpatient satisfaction
local relapse, both local and distant relapse was noted for 33.3%. score by 8.5% (93.5% in 2021 vs. 85% in 2020). An increase in Stem Cell
. At 8 months, 25% of patients were alive in complete remission, Transplantations (SCT) by 30% (137 SCT in 2021 vs. 105 SCT in 2020)
46.8% alive in progression, 18.8% dead. in univariate and multivari- observed
ate analysis, the only prognostic factor for survival was the surgical Conclusions: In our experience an existing oncology IPU, supported
margins. our ability to adapt and mitigate through evolving pandemic addressing
Conclusions: Surgical margin remains a strong prognostic factor for full cycle of cancer care through comprehensive range of services, IPU
survival of patients with head and neck RMS espacially in our country demonstrated unexpected prospect in the face of adversity improving
with lack of acces to radiotherapy integration and communication throughout continuum of care

E-Poster Topic: AS03 CCI - Childhood Cancer International - focused for P026 / #794 ASSOCIATIONS BETWEEN SLEEP AND MENTAL
parents of children with cancer HEALTH IN SURVIVORS OF PEDIATRIC ACUTE
LYMPHOBLASTIC LEUKEMIA
CCI POSTER SESSION
Sara Cho1 , Sharon Hou1 , Brooke Russell1 , Erin Merz2 , Kathleen
P025 / #1421 PEDIATRIC HEMATOLOGY ONCOLOGY Reynolds1 , Lianne Tomfohr-Madsen3 , Fiona Schulte1
INTEGRATED PRACTICE UNIT (IPU) IN A TERTIARY CARE 1 University of Calgary, Oncology, Calgary, Canada; 2 California State Uni-
SETTING – AN INITIATIVE TO FACILITATE UNINTERRUPTED versity Dominquez Hills, Psychology, Dominguez Hills, United States of
ACCESS TO CARE DURING THE COVID-19 PANDEMIC America; 3 University of Calgary, Psychology, Calgary, Canada

Amani Alkofide1 , Mahasen Alsaleh1 , Ibrahim Ghemlas2 , Awatif Background and Aims: In North America, acute lymphoblastic
Alanazi2 , Antonello Podda1 , Sarah Ramiz1 , Saadiya Khan1 , Diana leukemia (ALL) is the most common diagnosis for children and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S518 of S635 ABSTRACTS

adolescents impacted by cancer. With advances in treatment, survival confirmation at the Specialized Hospital. One hundred eighty types
rates of survivors of childhood ALL have improved substantially over of cancer were confirmed among the cases evaluated by the hospi-
the past decade. However, survivors of childhood ALL live with chronic tals, representing 8% of the referrals. The diagnostic of five hundred
health problems related to their treatment, referred to as late effects. seventy-seven other diseases was also possible because of the UPC’s
Among these late effects, fatigue, and mental health concerns are par- referral flow. Another aspect that deserves to be highlighted is the
ticularly prevalent. The current study therefore aimed to examine how training of health professionals on the signs and symptoms of childhood
mental health (depression and anxiety) is related to sleep in survivors cancer. Four thousand three hundred seventy-eight primary care pro-
of childhood ALL. fessionals were trained in Rio de Janeiro, and 97% of the city’s primary
Methods: Survivors of childhood ALL (n = 45, 58% male, average 5 care units referred suspected cases through the UPC.
years since diagnosis) and between 8 and 18 years old (mage =11.62, Conclusions: Improving early diagnosis is crucial to increase the
SD=2.62) at evaluation were asked to complete a daily sleep diary for chances of cure and the quality of life of children and adoles-
7 consecutive days to determine their wake after sleep onset (WASO) cents with cancer. The UPC produced significant results in Rio
and total sleep time. Their parents completed questionnaires related to de Janeiro by making suspected childhood cancer visible in pri-
their child’s mental health, and demographic information. mary care and ensuring that children with suspected cancer cases
Results: Pearson’s correlations were conducted to determine the reached diagnostic and treatment centers within three working
relationship between sleep and mental health. Child anxiety was sig- days.
nificantly related to total sleep time (r=-0.076, p< .05). Multiple
regression analysis was conducted to determine predictors of sleep,
including participant and clinical characteristics (gender, age at diag- P028 / #1036 THE USE OF SOCIAL MEDIA TO WIDEN
nosis) and mental health (child anxiety). The multiple regression was REACH OF PRACTICAL GUIDANCE FOR UK CHILDREN WITH
significant for predicting WASO F(4,33)=140.831 p=0.049 adjusted CANCER AND THEIR FAMILIES DURING THE COVID-19
r2 =0.153. Age at diagnosis emerged as a significant positive predic- PANDEMIC
tor of WASO (standardized B=0.400, p=0.018) where older age at
diagnosis predicted higher WASO. Claire Cuerden1 , Jessica Bate2
Conclusions: Analysis revealed that those who were diagnosed older 1 University Hopsital Southampton NHS Foundation Trust, Paediatric Oncol-

had more disrupted sleep and those who had higher anxiety had less ogy, Southampton, United Kingdom; 2 University Hospital Southampton
overall sleep. Information on survivors’ mental health and treatment NHS Foundation Trust, Paediatric Oncology, Southampton, United Kingdom
variables may help provide targets for intervention to improve their
quality of sleep and well-being. Background and Aims: The Children’s Cancer and Leukaemia Group
(CCLG) were one of the first professional groups to develop specific
COVID-19 guidance for patients and families internationally. The guid-
P027 / #1466 UNIDOS PELA CURA: OUTCOMES OF THE ance was based on UK Government and Public Health England advice
CHILDHOOD CANCER EARLY DIAGNOSIS STRATEGY IN RIO DE and was regularly updated as further evidence became available. This
JANEIRO, BRAZIL was disseminated to families and professionals online, including via the
CCLG social media channels. The aim of this study was to analyse the
Michele Costa, Carolina Motta online reach of the CCLG COVID-19 guidance and the primary sources
Desiderata Institute, Pediatric Oncology, Rio de Janeiro, Brazil of website traffic.
Methods: The CCLG COVID-19 group convened in March 2020
Background and Aims: In Brazil, childhood cancer is the leading cause and included consultant representation from neuro-oncology, oncol-
of death by disease amongst children between 1 to 19 years old. This ogy, haematology, and bone-marrow transplantation. The group also
paper analyses the main results of the Unidos pela Cura (UPC) strategy, received input from clinical trial management groups and early phase
launched in 2005, with the goal to promote early diagnosis of childhood trial researchers. The first version was published on the CCLG web-
cancer in Rio de Janeiro. The UPC program comprises three strategies: site on 11/03/20. Parents and families provided direct feedback on
training of primary care professionals to suspect cancer; systematiza- each revision via CCLG social media channels. Data analytics from the
tion of a referral flow of suspected cancer cases within 72 hours from CCLG COVID-19 guidance webpage were collated from 11/03/20 to
Primary Care to Specialized Hospitals; monitoring suspected cases 03/10/21.
referred to diagnostic confirmation. Results: There were a total of 90396 views to the CCLG COVID-19
Methods: This is a descriptive, cross-sectional, documentary study guidance webpage from 142 countries during the study period. Most
with a quantitative methodology, based on the results of the 18th views were from the United Kingdom (n=78499, 86.8%), followed by
publication of the Unidos pela Cura report. the United States (n=2753, 3.0%), Australia (n=845, 0.9%), Ireland
Results: Between 2008 and 2020, two thousand one hundred forty-six (n=784, 0.8%) and Canada (n=593, 0.7%). Of the total views, 79245
cases of suspected cancer cases were referred by the UPC program. (87.7%) were from unique IP addresses. Peaks in web traffic correlated
91% of them were referred within 3 working days for the diagnostic with release of updated versions of the guidance. The webpage was
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S519 of S635

primarily accessed through Google (38%), directly (29%) or via Face- appeared to reduce treatment length, larger prospective studies are
book (19%). warranted.
Conclusions: The CCLG COVID-19 guidance has had a wide reach, both
in the UK and internationally. Patients and their families commonly use
the internet to seek healthcare information, including via social media P030 / #219 IMPROVED HOME MANAGEMENT OF ORAL
platforms. It is therefore important to utilise these channels to dis- PEDIATRIC ANTICANCER DRUGS AS A RESULT OF AN
seminate accurate, evidence-based and up-to-date information to the INTERVENTION COMPRISING PRACTICAL TRAINING, WRITTEN
general public. INSTRUCTIONS AND MOVIE CLIPS: A PILOT STUDY

Ranaa Akkawi El Edelbi1 , Staffan Eksborg2 , Jennie Ekman3 , Synnöve


P029 / #1555 ARE CURRENT NUTRITION SUPPORT Lindemalm4
STRATEGIES EFFECTIVE AT IMPROVING NUTRITIONAL STATUS 1 Astrid Lindgrens Childrens Hospital, Department Of Women’s And Chil-
IN CHILDREN AND ADOLESCENTS WITH OSTEOSARCOMA? dren’s Health, stockholm, Sweden; 2 Astrid Lindgrens Childrens Hospi-
tal, Department Of Women’s And Childrens Health, stockholm, Sweden;
Jessica Dadson1 , Laura Sealy2 , Raquel Revuelta Iniesta3 3 Karolinska University Hospital, Division Of Pediatrics, Stockholm, Swe-
1 The University of Exeter, Department Of Natural Sciences, College Of den; 4 Astrid Lindgrens Childrens Hospital, Department Of Clinical Sciences,
Engineering, Mathematics, And Physical Sciences, Exeter, United King- stockholm, Sweden
dom; 2 Bristol Royal Hospital for Children, Department Of Dietetics, Bristol,
United Kingdom; 3 College of Life and Environmental Sciences,The Univer- Background and Aims: Background: Long term treatment of pedi-
sity of Exeter, Department Of Sports And Health Sciences, Exeter, United atric patients with oral anticancer drugs (OADs) requires the par-
Kingdom ents/caregivers to prepare the drug at home. The handling procedures
in the home setting are, however, not regulated by Swedish law and
Background and Aims: Studies investigating the impact of nutri- the parents are often left without guidance on how to handle OADs
tional support (NS) on paediatric osteosarcoma patients’ nutritional in a safe way. Aim: The aim of this study was to increase understand-
status are scarce, despite evidence showing optimal nutritional status ing of how OADs are handled by parents/caregivers in the home setting
improves clinical outcomes. We investigated patterns of change in the before and after an intervention.
nutritional status of osteosarcoma patients receiving NS. Methods: Parents of pediatric cancer patients were observed and
Methods: A retrospective study of patients (aged <18 years) diagnosed videotaped during their handling of OADs in the home setting before
and treated for osteosarcoma at Bristol Royal Children’s Hospital and after the intervention. During the intervention, the parents were
(2015–2020) was performed. Clinical and nutritional data were col- provided with written instructions, movie clips and practical training on
lected over 30-day periods at defined time points from the start of handling the OADs. Four checklists were used to compare and score
treatment (days 0 to 270). BMI and weight Z-scores were calculated the four handling procedures (measuring an oral suspension, cutting
from WHO (2010) and nutritional status was defined as underweight tablets, dissolving tablets, and opening capsules) for each parent before
(BMI -2 SD), over-nourished; overweight [BMI ≥1.05<1.63 SD] and and after the intervention.
obese [BMI ≥1.63 SD]. Energy (EI) Kcal/day and protein intake (PI) Results: The intervention significantly improved the OAD handling
g/day were obtained from medical records, compared against energy procedures among the studied parents. The median score for correct
(ER) and protein requirements (PR) respectively (DRVs 1991) and pre- handling was 19 % (IQR: 3.6 to 30%) before the intervention and 89.5
sented as a percentage difference from the estimated requirement. NS % (IQR: 71.5 to 94.5%) after the intervention (p < 0.0001).
was defined as oral (ONS), enteral feeding (NG/PEG), parenteral nutri- Conclusions: An intervention comprising practical training and infor-
tion (PN) and/or complex NS (CNS: NG+PN). Data was analysed using mation presented in different forms improved the handling of OADs at
descriptive statistics and correlations. home by parents. There is an urgent need to implement this method in
Results: Eleven patients [mean±SD 13.3±2.8 years old, 72.7% female]. all oncology centers in Sweden, educate HCPs to standardize the pre-
Of these, 3 (27.3) were over-nourished, 7 (63.6%) well-nourished, and sentation of information. There is also a great need to provide parents
1 (9.1%) was underweight at the start of treatment. NS was prescribed with age-appropriate oral drug formulations from the local hospital
to 9 (82%) patients. Of these, 8 (73%) received ONS, 4 (36%) NG, 3 pharmacies in Sweden.
(27%) TPN and 1 (9%) CNS. EI% of ER and PI% of PR mean±SD were
-32.2±30.0 and 26.9±82.1 respectively. Correlation between EI and
PI and treatment length were r=-0.6; p=0.06 and r =- 0.32; p=0.36 P031 / #1353 EXPRESSIVE ARTS IN BAUH: CASE STUDIES
respectively. Weight change Z-score from the start until the end of AND REVIEW OF EVIDENCE
treatment was - 0.97±0.36.
Conclusions: We showed high demand for NS in osteosarcoma Manale Elewah
patients, and current NS strategies did not support weight Burg AlArab University Hospital, Support Services, Burg Al Arab, Egypt
maintenance probably attributed to reduced EI. Although this
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S520 of S635 ABSTRACTS

Background and Aims: Pediatric cancer is a leading cause of death assess for toxicity and response (Cohen et al., The Oncologist 2020).
for children, with almost 400,000 new cases diagnosed globally and 33 Articles were subsequently screened for reporting of race and/or eth-
800 new cases estimated among children aged 0-14 years in the East- nicity in baseline patient characteristics and outcomes by race and/or
ern Mediterranean Region (EMR) in 2020. Although childhood cancer ethnicity in the main text, tables, and figures.
is curable for the majority of children when essential diagnostic, ther- Results: In total, 109 articles were included for analysis, 78 (72%) of
apeutic and supportive care services are accessible. The number of which incorporated targeted therapies. The total number of patients
people under the age of 18 in Egypt reached 38 million in 2017, making was 2713 with median age of 11 years (range 3 – 21). Among all
up 40% of total population, the Central Agency for Public Mobiliza- articles, only 36 (33%) reported race or ethnicity in baseline patient
tion and Statistics (CAPMAS) announced. As of January 1, 2021, the characteristics, of which 23 (63.9%) provided information on both race
Egyptian population was estimated at 101.48 million inhabitants. A and ethnicity of trial participants. Trials published in 2014 reported
population of 17,000,000 is being served by BAUH, roughly speaking race/ethnicity most frequently (42%), whereas trials published in
20% or 1/5 of Egypt population. This presentation shall explore the 2016 reported race/ethnicity least frequently (18%). No year-on-year
evidence of art therapy and pediatric cancer patients, shall teach pedi- reporting trends were identified. Only one study described safety or
atric cancer patients/staff how to express their feelings via arts, shall toxicity outcomes by race/ethnicity.
help cancer patient/family to heal from cancer journey, shall highlight Conclusions: Reporting of racial/ethnic representation in early phase
the psychological/psychosocialsocial/rehabilitation support program pediatric oncology clinical trials published between 2012 and 2018
at Burg AlArab University Hospital BAUH. was very limited, inconsistent, and did not improve over time. Addition-
Methods: Weekly art therapy interventions are carried out with the ally, subgroup analyses of safety and efficacy results by race/ethnicity
pediatric patients over the year 2021. These interventions covered fin- was rarely reported. Reporting of representation and outcomes of
gerprints, oregami, clay, music and drama therapy sessions. A sample racial/ethnic minorities in clinical trials needs to be prioritized to bet-
of 180 pediatric cancer patients aged 5 to 15 years old were stud- ter evaluate the extent of demographic underrepresentation, health
ied for emotional change and psychosocial status. Major Depressive disparities, and ultimately improve access to emerging therapies for
Disorder MDD was used to determine the depression, anxiety levels high-risk groups.
which caused impairmnet in social and academic behaviours. Also Gen-
eralized Anxiety Disorder GAD was used to determine the excessive
anxiety and worry. P033 / #1867 INVOLVING PARENTS & CARERS IN THE
Results: At least 2 of the symptoms included in MDD were expressed DEVELOPMENT OF NOVEL DEVICES AND PROTOCOLS FOR
among the pediatric cancer patients at BAUH in the first 2 weeks. The THERAPEUTIC DRUG MONITORING IN PAEDIATRIC
Kubler-Ross change curve was aoolied to determine the emotions of ONCOLOGY
the pediatric cancer patients at BAUH.
Conclusions: Therapeutic art encourages both verbal and non-verbal Lisa Pfaffenrath1 , Neil Ranasinghe2 , Alaric Taylor3 , Deirdre Leyden4 ,
communications of a person’s perceptions and feelings. Jo Ball5 , Neil Davidson5 , Gareth Veal6 , Stefan Guldin1
1 University College London, Department Of Chemical Engineering, Lon-
don, United Kingdom; 2 Paediatric Oncology Reference Team, Port, London,
P032 / #284 REPORTING OF RACIAL/ETHNIC MINORITY United Kingdom; 3 Vesynta ltd, -, London, United Kingdom; 4 NIHR Great
REPRESENTATION IN PHASE I/II PEDIATRIC ONCOLOGY Ormond Street Hospital Biomedical Research Centre, -, London, United King-
CLINICAL TRIALS dom; 5 Great North Children’s Hospital, -, Newcastle upon Tyne, United
Kingdom; 6 Newcastle University, Newcastle University Centre For Cancer,
Aiman Faruqi1 , John Ligon2 , Julia Cohen3 , Srivandana Akshintala1 , Newcastle upon Tyne, United Kingdom
Brigitte Widemann1 , Nirali Shah1
1 National Cancer Institute, Pediatric Oncology Branch, d, United States of Background and Aims: ChromaDose is a NIHR (National Institute
America; 2 University of Florida, Division Of Hematology/oncology, Depart- of Health Research) funded project that is developing a bedside
ment Of Pediatrics, d, United States of America; 3 Merck and Co., Early drug monitoring technology to enable safe and effective personalised
Oncology Development, d, United States of America anthracycline chemotherapy for children. Public and Patient Involve-
ment and Engagement (PPIE) is a core element of the project for
Background and Aims: Racial/ethnic minorities experience inferior feedback and advice on the patient experience with the technology.
cancer outcomes and face systemic barriers to equitable clinical trial Various stakeholder groups are actively involved, including the Young
access, but the extent of underrepresentation, particularly among chil- Persons Advisory Groups at Great Ormond Street Hospital and North
dren and in early phase trials, is incompletely understood. We explored England, and the Paediatric Oncology Reference Team. Herein, we
patterns of reporting of race/ethnicity to evaluate representation are presenting the outcomes obtained during the initial phase of the
across early phase pediatric oncology studies. project.
Methods: Phase I/II clinical trials in pediatric oncology from 2012- Methods: Active involvement of patients and young persons as well
2018 were previously analyzed in a systematic review by our group to as parents and carers was ensured through two separate streams of
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S521 of S635

consultation. For each stakeholder group, an interactive online sur- Moment’,’ Financial Burden’,’ Effect on Relationships’ and ’Valuable
vey was conducted with embedded videos. Live online sessions were Experience’.
subsequently carried out with an interactive whiteboard as well as Conclusions: It is important for clinicians in this field to understand the
individual questionnaires. Participants were shown the data of the sur- experiences of primary caregivers in order to provide effective support.
vey and asked to respond to follow-up questions for selected E-Poster It is hoped that this study may provide healthcare professionals with
Topics centred around participation in a clinical trial for therapeutic a useful insight into understanding the unique experiences of being a
drug monitoring, confidence in a novel blood testing tool, and different primary caregiver of a child who is diagnosed with cancer.
forms of engagement.
Results: Our PPIE activities are planned, carried out and analysed in
close collaboration with the engineering team to ensure that find- P035 / #1164 COMMUNICATION CHARACTERISTICS FOR
ings are directly implemented into the design. Example outcomes FERTILITY PRESERVATION PATHWAY FOR ADOLESCENT AND
from Phase 1 include technical features on displaying the progress YOUNG ADULT CANCER PATIENTS AND THEIR CARE GIVERS
of analysing the blood, quality controls, guidance on appropriate IN KOREA
terminology, and visual appearance.
Conclusions: Our work has been perceived as a model approach that Yoo Sub Shin1 , Mi Na Park2 , Seungmin Hahn3 , Wonkee Ahn3 , Seoug
others should follow (NIHR review). Online and in-person sessions Yeon Kwon3 , Chuhl Joo Lyu3 , Jung Woo Han3
are planned to create patient information resources, provide input 1 Yonsei University College of Medicine, Department Of Urology, Seoul,
on device design and for receiving advice on blood sampling proto- Korea, Republic of; 2 Yonsei Cancer Center, Department Of Pediatric
cols. Outreach events will serve to raise awareness of therapeutic drug Hematoma-oncology, Seoul, Korea, Republic of; 3 Yonsei University College
monitoring and to highlight our approach of co-creation in product of Medicine, Department Of Pediatrics, Seoul, Korea, Republic of
development.
Background and Aims: Fertility preservation (FP) discussion is a
crucial step for adolescent and young adult (AYA) cancer patients; how-
P034 / #59 PRIMARY CAREGIVERS EXPERIENCES OF ever, it is relatively new to Asian countries. This study highlights the
HAVING A CHILD WITH CANCER: A SCOPING LITERATURE quality of communication during FP discussions in Korea.
REVIEW OF QUALITATIVE STUDIES Methods: We performed FP clinical pathways and surveyed details
on the discussion characteristics and satisfaction scales dufing the FP
Elifnur Güneş discussions for AYA cancer patients and their guardians in Yonsei Can-
Manchester Metropolitan Universtiy, Nursing, Manchester, United Kingdom cer Center, Seoul, Korea; Quality of FP discussions and the degree of
satisfaction of the discussions were measured on a scale of 1-7.
Background and Aims: Having a child diagnosed with cancer greatly Results: Out of the 34 patients and their 32 guardians completed
affects the lives of the child and his/her primary caregivers. This the survey. Two guardians did not answered the survey. All respon-
scoping review aimed at summarizing the qualitative studies in the dents reported high overall satisfaction, however several factors were
recent literature on the experiences of primary caregivers living with related with low satisfaction or information quality. On the type of
childhood cancer, identifying key themes and determining the gaps counsellors, both respondent groups showed high overall satisfaction
in the extant literature. Knowledge about primary caregivers’ individ- when their counsellor were physicians rather than other types of care
ual responses and perceptions of the cancer experience may provide providers. For the information quality, the guardians who were pro-
insight into the experiences of the larger family system. vided with verbal and non-verbal communication tools (pamphlets,
Methods: Following PRISMA-ScR guidelines, a systematic scoping internet resources, or others) both were more satisfied with the infor-
review was conducted. Data sources included PubMed, CINAHL, Med- mation quality than the only verbal communications. As for th number
line and Embase (Ovid), Cochrane Library, and AMED. Using key terms of discussion sessions, more than one discussion sessions showed
“childhood cancer”, “primary caregivers” and “qualitative research”; and improved understanding of the concept of FP, higher communication
then hand searched for the time period 2010-2021. Lastly, reference and information quality.
lists and citations of all identified articles were manually searched. No Conclusions: In order to improve FP process for AYA cancer patients,
new articles were identified through this process. we need to adjust the type of counsellors, number of discussion ses-
Results: 729 papers were reviewed, 10 met inclusion criteria. sions, and types of information. This will be the cornerstone of the
This review examined the qualitative research exploring the effective FP communications in Korea.
experiences of primary caregivers’ who has children with can-
cer and discusses common themes across all studies. Qualitative
research makes it possible to gather and analyze individualistic P036 / #217 UNDERSTANDING HEALTHCARE DATA FOR
data on deeper levels. Five themes were identified which were con- CHILDREN’S AND YOUNG PEOPLE’S CANCERS: A
cerned with the effect a diagnosis of cancer can have on primary PARTICIPATORY STUDY
caregivers’ lives. These were: ‘Emotional Roller Coaster’,’ Pivotal
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S522 of S635 ABSTRACTS

Nicola Hughes1 , Ashley Ball-Gamble2 , Kathy Pritchard-Jones3 , Chris Kingdom; 5 Solving Kids’ Cancer US, Director Of Research Advocacy, New
Carrigan4 , Emily Connearn4 , Richard Feltbower1 , Angela Polanco5 , York, United States of America
Lorna Fern6
1 University of Leeds, Leeds Institute For Data Analytics, Leeds, United Background and Aims: Social media communities enable greater
Kingdom; 2 Children’s Cancer and Leukaemia Group, Ceo, Leicester, United awareness of treatments available across borders. Parents/carers of
Kingdom; 3 University College London, Ucl Great Ormond Street Institute children with poor-prognosis cancers who exhaust options in-country
Of Child Health, London, United Kingdom; 4 DATA-CAN, Patient And Pub- may choose to seek therapies abroad. Child Medical Tourism (CMT) is
lic Involvement, Leeds, United Kingdom; 5 Children’s Cancer and Leukaemia controversial, ethically challenging, limited to high-income families, or
Group, Public Contributions, Leicester, United Kingdom; 6 University College those with responsive networks in high-income countries. The national
London Hospitals, Cancer Clinical Trials Unit, London, United Kingdom cost of CMT has not been quantified, nor have the inherent socioe-
conomic disparities been described. This case study highlights the
Background and Aims: In the United Kingdom, healthcare data is col- significant cost to the UK economy when millions of GBP are spent
lected on all patients receiving National Health Service care, including for treatments abroad via support by one disease-specific charity and
children and young people (CYP) with cancer. This data is primarily signals the need for a multistakeholder strategy, to include expert
used to inform service delivery but with special permissions, and with- advocates and clinicians, to prioritize and address the challenges of
out individual consent, can also be reused for research. The use of initiating and conducting clinical trials in the UK.
routinely collected health data in research is an advancing field with Methods: Five years of expenditure on treatment and travel costs for
huge potential benefit, particularly in CYP where case numbers are UK neuroblastoma families are reported, supported by Solving Kids’
small and the impact across the lifecourse can be significant. We aimed Cancer UK.
to determine what CYP and carers knew about health data collection Results: From 3/2017- 3/2022 50 UK families with neuroblastoma
and their views on its use for research. children travelled or intended to travel to the US, Germany and
Methods: CYP and carers were invited to an interactive 2.5 hours Spain for treatments. The therapies included proton beam, surgery,
online workshop comprising of presentations from health data experts, immunotherapy, radio-immunotherapy, chemotherapy regimens, and
case-studies and group discussion. With consent the workshop was relapse prevention. The cost of treatment and travel varies for each
recorded, transcribed verbatim and analysed using thematic analysis. family and can range from £180,000 to more than £300,000. Cam-
Results: Ten young people currently aged ≥16 years and diagnosed paigns for 66 children raised £10,177,974 with ongoing expenditures.
with cancer at age 5-19 years attended. Six carers also participated. In the 5-year period, £5,102,267 was spent abroad for 50 children.
Three key themes emerged: Awareness: Participants were surprised Conclusions: Economic issues of CMT in UK neuroblastoma children
at the volume and depth of data collected and reported receiving highlight the need for developing a national strategy to prioritize the
little information about use of their healthcare data. They reported conduct of clinical trials for innovative therapies at home. This is an
this could lead to mistrust in healthcare sytems. Value: Participants opportunity to call for systemic change and could accelerate evaluation
were happy for their data to be used for research and felt data of more effective treatments for children with cancer, re-directing the
should be more accessible for the purposes of research. Data shar- millions of GBP spent abroad. Other countries may benefit from a sim-
ing: The importance of data sharing in CYP cancers, particularly in ilar effort to prioritize the support of specific clinical trials within their
rarer tumours, to improve outcomes was recognised by participants. national health service.
Participants identified numerous barriers to data sharing includ-
ing mistrust in the media and difficulty accessing their own health
records. P038 / #532 EXPLORING COPING STRATEGIES AMONG
Conclusions: Young people and their carers support the idea of data CAREGIVERS OF CHILDREN WHO HAVE SURVIVED CANCER IN
collection and recognise its importance in research to improve out- JORDAN
comes for CYP. However, education and transparency relating to data
collection and its uses are required. Taleb Ismael, Sherin Al Awady, Sumaya Malkawi, Noor Ismael
King Hussein Cancer Center, Pediatric Oncology, Amman, Jordan

P037 / #1473 PASSPORT TO GLOBAL HEALTHCARE: Background and Aims: Children who have survived cancer and their
ECONOMIC COST TO A HIGH-INCOME NATION caregivers may deal with residual physical, cognitive or social disabil-
ities. There is little research on caregivers’ health and wellbeing after
Vicky Inglis1 , Gail Jackson2 , Gemma Wadsley3 , Hayley Blackwell4 , cancer. In addition, there is no specific research about how caregivers
Donna Ludwinski5 cope with everyday stressors after cancer. This study aimed to explore
1 Solving Kids’ Cancer UK, Head Of Family Support Service, London, the coping strategies that caregivers of children who have survived
United Kingdom; 2 Solving Kids’ Cancer UK, Ceo Skc, London, United cancer utilize to overcome everyday stressors.
Kingdom; 3 Solving Kids’ Cancer UK, Head Of Finance, London, United King- Methods: This study utilized a descriptive survey design. The sam-
dom; 4 Solving Kids’ Cancer UK, Family Support Coordinator, London, United ple consisted of 103 caregivers, visited the clinic at a national cancer
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S523 of S635

center. The study included caregivers of children who were off can- Results: We assessed 107 patients and their parents. The ADI decile
cer treatments for at least two years. Caregivers completed the study within Indiana ranged 1 to 10 (median 5, mean 5.3); national per-
survey. The survey collected caregiver reported demographic infor- centile ranged 7 to 100 (median 71, mean 67.3). Overall COI mean
mation, and the Brief COPE which measures caregivers’ frequency was 3, with sub-scores for education - 2.9, health/environment - 2.6,
of engaging in certain coping strategies. The Brief COPE consisted and social/economic - 3.1. The PedsQL™ was completed by 96 par-
of 14 coping sub-scales, which are Self-Distraction, Active Coping, ents and 91 patients. Physical mean was 67.4 and 71.2, psychosocial
Denial, Substance Use, Use of Emotional Support, Use of Instrumen- mean 67.8, 68.9, and total mean 67.7, 69.8, respectively. Simple linear
tal Support, Behavioral Disengagement, Venting, Positive Reframing, regressions demonstrated a correlation between increasing disparity
Planning, Humor, Acceptance, Religion, and Self-Blame. Data Anal- and decreasing quality of life across all dimensions.
yses included calculating sub-scales’ scores for the fourteen coping Conclusions: This is one of the first studies to associate a decrease in
strategies. pediatric quality of life with disparities of social determinants of health.
Results: The 103 caregivers were 62% mothers, 80% married, 45% fin- These data demonstrate the need for expanded prospective evaluation
ished high school, 50% don’t work outside the house, and 60% have to track social determinants of health that may impact on the quality of
low family income. Result showed that Religious Coping (66%) and life in children diagnosed with CNS tumors.
Acceptance (60%) were the most utilized coping strategies, followed
by Positive Reframing (45%), Active Coping (44%) and Planning (43%).
The least utilized coping strategies in our sample were Humor (5%), P040 / #1759 HOW DOES HAVING A CHILD WITH CANCER
Behavioral Disengagement (8%), and Substance-Use (10%). AFFECT PARENTS’ PSYCHOLOGY?
Conclusions: Caregivers mostly utilize religious coping and acceptance
in dealing with everyday stressors. Because these coping strategies Begum Koc, Funda Tekkesin, Zehra Balik, Aysenur Kanat, Ülkü Miray
do not directly solve stressors, it is important to support caregivers Yıldırım, Selime Aydogdu, Suar Kilic
in choosing and implementing effective coping strategies. Some care- Umraniye Training and Research Hospital, Pediatric Hematology And Oncol-
givers may utilize substance use as a coping strategy, which has ogy, Istanbul, Turkey
negative health effects, there must be direct interventions that targets
these caregivers and their families. Background and Aims: ‘Cancer’ affects parents as well as children
in both physical and psychological aspects. The aim of this study was
to evaluate psychological resilience, hopelessness and intolerance of
P039 / #85 HEALTH-RELATED QUALITY OF LIFE AND uncertainty in parents of children with cancer.
SOCIAL DETERMINANTS OF HEALTH WITHIN CHILDREN WITH Methods: Parents of children with cancer of our clinic were asked to fill
BRAIN AND SPINAL CORD TUMORS a personal information form and the Intolerance of Uncertainty Scale
(IS), the Resilience Scale for Adults (RSA), and the Beck Hopelessness
Kelsey Knight1 , Scott Coven2 , Karl Balsara1 Scale (BHS). These scales consisted of a total of 79 questions were
1 Indiana University Health, Pediatric Neurosurgery, Indianapolis, United answered by the volunteer parents. The results of the questionnaire
States of America; 2 Indiana University Health, Pediatric Hematology Oncol- were statistically compared.
ogy, Hartford City, United States of America Results: Our study group consisted of 84 parents of 84 children.
Demographic features were summarized as: 85% of the parents were
Background and Aims: Social determinants of health (SDOH) have a between ages of 30-49, 94% were married, 81% had 2 or more children,
significant impact on health, well-being, and quality of life (QOL). Val- 58% had lost their jobs during treatment period. 78% of their children
idated tools to assess these disparities have been developed but not with cancer were <12 years old, 60% had solid tumors and 40% had
utilized prospectively in children with central nervous system tumors leukemia. No significant difference was observed in the scores of the
(CNS). Objective: To establish a baseline assessment of health-related intolerance of uncertainty, hopelessness and resilience scales between
quality of life and associated social determinants of health in children the parents of the patients with leukemia and solid tumor(p>.05).
with CNS tumors in Indiana. There was no difference in the psychological evaluations of the par-
Methods: We implemented the Pediatric Quality of Life Inventory™ ents regardless of the cancer type and stage. According to the time
(PedsQL™) for patients (ages 0-21 years) diagnosed with a CNS tumor elapsed after diagnosis; the resilience scores of the parents during the
evaluated in the neuro-oncology clinic from July 2019-January 2022. first month of the treatment were higher than those of the parents dur-
A higher score is associated with better quality of life. Patient’s ing 1-6 months and after one year(p<.05). According to the BHS; the
address was utilized to obtain Area Deprivation Index (ADI) and Child scores were significantly higher among the parents of children whose
Opportunity Index (COI). ADI allows for rankings of neighborhoods treatment had been completed than those who were being actively
by socioeconomic disadvantage at state or national level (1-10: 1 treated(p<.05).
is least disadvantaged). COI measures the quality of resources in a Conclusions: Our study showed that the parents’ psychological sit-
patients’ community, with five categories ranging from very low- to uations were better during treatment period. We suggest that our
very high-opportunity. multi-disiplinary oncologic team including doctors, nurses with a
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S524 of S635 ABSTRACTS

psychologist and a play therapist may strongly impact psychological And Hematology, Rome, Italy; 4 Fondazione Policlinico A. Gemelli IRCCS,
well-being of both children and parents. Clinical Psycology Unit, rome, Italy; 5 Fondazione Policlinico A. Gemelli
IRCCS, Pediatric Neurosurgery, Rome, Italy

P041 / #1088 BELIEFS AS COPING RESPONSE IN PARENTS’ Background and Aims: Pediatric cancer diagnosis is considered a con-
ADJUSTMENT TO CHILDHOOD CANCER dition of traumatic risk for patients and parents. In this population,
COVID-19 may be considered a stressful event that may exacerbate
Inese Lietaviete risk or sequelae. Many studies have investigated parental perceptions
Children’s Clinical University Hospital, Hematooncology, Riga, Latvia during the pandemic, so it is also essential to monitor families over time.
During the COVID-19 pandemic we initially studied the psychological
Background and Aims: Parents of children with cancer face high impacts on parents of the cancer diagnosis of their children and the epi-
uncontrollability and uncertainty of the situation with few possibilities demic event. Then, after one year, we measured if there were changes
to regulate events, but they have the ability to regulate the cognitive in parents’ psychological conditions.
appraisal of the situation. This study aimed to explore the cognitive Methods: Eighty parents of pediatric cancer patients were enrolled
beliefs in response to their children being diagnosed with cancer. in the study at an early stage. At T1 (during the 2020 COVID-19
Methods: A cross-sectional study was conducted among 55 parents emergency) four questionnaires were completed: Impact of Event
(mostly mothers, n=49) having children with cancer. Pre-adapted Lat- Scale-Revised - IES-R; Perceived Stress Scale - PSS; State-Trait Anxiety
vian version of Psychosocial Assessment Tool (PAT) was used to collect Inventory - STAI-Y; Pediatric Quality of Life Inventory Parent Proxy-
information about parental beliefs and stress reactions after diagnosis. report - PedsQL), at T2 (one year later) 3 of the 4 questionnaires (PSS,
Results: Spearman’s rank correlation was computed to assess the STAY, and PedsQL) were administered. We analyzed preliminary data
relationship between parental beliefs and stress reactions whilst con- from this sample and the evolution of scores over time considering the
trolling for diagnose (leukemia (ALL, CML, AML), lymphoma, brain 36 parents who participated in both T1 and T2 measurements.
tumors, sarcoma). There was a negative correlation between the belief Results: In our sample (36/80), the bivariate correlation matrix found
in doctors’ competencies and depressiveness, r(53) = -.34, p < .05, as a significant positive correlation between IES-R and STAY Y-1 (state)
well as positive correlations with the belief in overcoming the illness scores (r = 0.46; P < 0.005) in T1. This correlation was not confirmed
("We are going to beat this"), r(53) = .49, p < .001, and the time since in T2. Post-hoc comparison between mothers (18) and fathers (18)
starting treatment, r(53) = .31, p < .05. Belief in doctor’s competen- showed a significant difference in PSS scores at T2 (t = 4.62; P < 0.001),
cies correlated negatively with belief about child’s pain ("My child will with, respectively: mothers 21.28±3.66 and fathers 15.44±3.90. State
be in a lot of pain"), r(53) = -.28, p < .05. Positive correlations were anxiety levels were maintained high in T2, with 83% of scores exceed-
found between the belief in reason ("Everything happens for a reason") ing the STAI-Y cut-off.
and the belief in strengthening the family ("Our family will be closer Conclusions: Results confirm that parents of pediatric cancer patients
because of this"), r(53) = .36, p < .001, but also with higher level of have high psychological risk and long-term sequelae and that the
depressiveness, r(53) = .27, p < .05, and anxiety, r(53) = .31, p < .05. COVID-19 pandemic likely influenced the maintenance of high lev-
Conclusions: Results suggest that parental beliefs in response to els of anxiety. Psychological interventions for parents are essential in
a child’s cancer diagnose are comparable to emotion-focused cop- oncology services.
ing strategies (secondary control strategies - predictive, vicarious,
interpretative). Some beliefs may simultaneously correlate with antici-
pation of positive outcomes from disease but also with negative stress P043 / #1417 TAKING BACK CONTROL TOGETHER:
reactions (anxiety/depression) in the context of the coping process. FEASIBILITY OF A PROBLEM-SOLVING FOCUSED
INTERVENTION FOR PARENTS OF CHILDREN WITH CANCER

P042 / #1804 PRELIMINARY FINDINGS ON PSYCHOLOGICAL Ariane Levesque1,2 , Vivianne Gravel1,2 , David Ogez1,3 , Valerie
SEQUELAE OF PARENTS OF PAEDIATRIC CANCER PATIENTS, Marcil2,4 , Daniel Curnier2,5 , Émélie Rondeau2 , Daniel Sinnett2,6 ,
DURING AND AFTER THE COVID-19 EMERGENCY Katherine Péloquin1 , Serge Sultan1,2,6
1 University of Montreal, Psychology, Montreal, Canada; 2 Sainte-Justine
Antonella Guido1,2 , Elisa Marconi3,4 , Laura Peruzzi2,4 , Nicola University Health Center, Research Center, Montreal, Canada; 3 University
Dinapoli3 , Gianpiero Tamburrini5 , Giorgio Attina’2 , Silvia Chiesa3 , of Montreal, Anesthesiology, Montreal, Canada; 4 University of Montreal,
Vincenzo Valentini3 , Antonio Ruggiero2 , Daniela Pia Rosaria Chieffo4 Nutrition, Montreal, Canada; 5 University of Montreal, Faculty Of Medecine,
1 Fondazione Policlinico A. Gemelli IRCCS, Clinical Psycology Unit, Rome, Montreal, Canada; 6 University of Montreal, Pediatrics, Montreal, Canada
Italy; 2 Fondazione Policlinico A. Gemelli IRCCS, Pediatric Oncology, Depart-
ment Of Woman And Child Health And Public Health, Rome, Italy; Background and Aims: Following a child’s cancer diagnosis, parents
3 Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Oncologic face unique psychological challenges that have been associated with
Radiotherapy, Department Of Diagnostic Imaging, Oncologic Radiotherapy an increase in their emotional distress in the short- and long-term. This
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ABSTRACTS S525 of S635

study aimed to assess the feasibility of Taking Back Control Together, of the children were acute leukaemia (40%), retinoblastoma (35%) and
an intervention targeting problem-solving abilities to reduce emotional Wilms tumour (15%). Over two-thirds (70%) of the parents admit-
distress in parents of children with cancer. ted to use of alternative medicine. This included spiritual (60%) and
Methods: We assessed the feasibility of the intervention by examin- herbal medicine (35%). Some of the spiritual therapies included oils and
ing its treatment fidelity, reach, and social validity. Two independent ‘anointed’ water from churches, prayer camps and traditional healers.
coders assessed treatment fidelity using a rating scale developed Of those using herbal medicine, 60% were oral, 30% E-Poster Topi-
ad hoc. We assessed reach using the participation rate (i.e., screen- cal and 10% via enema. Of the parents who used alternate medicine,
ing to enrollment ratio, dropout/crossover rates, response rates). We only 20% discussed their use with the medical staff. Worryingly, 15%
assessed the social validity of the intervention using an ad hoc Kazdin- of the caregivers used these alternative therapies concurrently with
Manne social validity toolkit. This toolkit was developed based on chemotherapy. Fourteen caregivers (40%) said they would still recom-
existing recommendations and guidelines for evaluating interventions. mend alternative medicine to others. Majority (90%) recommended
The social validity toolkit measured participants’ perception of the that education be provided to all caregivers on use and effects of
relevance, acceptability, and satisfaction of the intervention. alternate medicine.
Results: Preliminary results suggest an adequate treatment fidelity, Conclusions: An overwhelming proportion of caregivers in
with 77.5% of the components of the intervention being rated by both the POU, KBTH use alternative medicine. Ongoing education
coders as having been administered. Preliminary results on reach indi- on use of alternative medicine is needed for all caregivers
cate that of the 62 families screened, 32 families (51.6%) enrolled in of children receiving treatment for cancer to avoid harmful
the intervention (42 participants, with 30 mothers and 12 fathers). effects.
Of these 42 participants, 16 participants (38.1%) dropped out of the
intervention. The evaluation of the social validity of the intervention is
ongoing. P045 / #477 BARRIERS TO LONG TERM FOLLOW UP IN
Conclusions: Taking Back Control Together is a unique intervention PEDIATRIC HODGKIN LYMPHOMA SURVIVORS
that targets both mothers and fathers of children with cancer. This
study allowed to highlight which aspects of the intervention should be Omar Shakeel, Mehmet Okcu, Hailey Simpson
redesigned to improve its feasibility in future research evaluating the Baylor College of Medicine, Pediatric Hematology Oncology, Houston,
impact of the intervention on the psychosocial outcomes of parents of United States of America
children with cancer.
Background and Aims: Childhood cancer survivors of Hodgkin Lym-
phoma are at high risk of late effects following their cancer treatment.
P044 / #1267 PREVALENCE OF ALTERNATIVE MEDICINE Unfortunately, there are numerous barriers at the patient, provider,
USE BY CAREGIVERS OF CHILDREN RECEIVING TREATMENT hospital, and payor level that may adversely affect their adherence to
AT KORLEBU TEACHING HOSPITAL ACCRA,GHANA long term follow up care.
Methods: We conducted a retrospective chart review on a cohort
Linda Sarpong1,2 , Doris Darko2 of 122 Hodgkin lymphoma survivors diagnosed between 1994
1 Korlebu Teaching Hospital, Ghana., Child Health, Paediatric Oncology, and 2014 and identified patients who were lost to follow up
Accra, Ghana; 2 Korlebu Teaching Hospital, Ghana., Department Of Child after completing treatment (defined as no visits to Texas Chil-
Health, Accra, Ghana dren’s Long-Term Survivor clinic for 2 or more years). Structured
phone interviews were then conducted to determine contributing
Background and Aims: Socio-cultural practices contribute to health- factors.
seeking behaviour in many low and middle-income countries, includ- Results: Of the 122 patients, 56 (45%) were lost to follow up and
ing Ghana. Parents may ascribe spiritual and other causes to their 24 (42%) of these patients were interviewed by phone. 11 (46%) of
child’s cancer and may seek complementary and alternative treatment the patients lost to follow up cited loss of or inadequate insurance
approaches. Such practices may however undermine treatment, lead- as their primary reason for not following up. 8 (33%) of patients indi-
ing to adverse treatment outcomes. The study aim was to describe the cated a lack of education as another reason for their lack of follow-up.
prevalence of alternative medicine use at the Paediatric Oncology Unit Fewer patients reported following up with adult oncology (21%), mov-
(POU) of the Korle Bu Teaching Hospital (KBTH), Accra, Ghana. ing (21%), Covid-19 pandemic (13%), or not wanting to follow-up
Methods: Cross-sectional study using a questionnaire administered (4%).
to caregivers of children with cancer receiving or recently completed Conclusions: Lack of or inadequate insurance and lack of education
treatment at the POU, KBTH. were the two most common reasons for lost to follow-up in LTS clinic
Results: Of the 35 caregivers included in the study, majority (68%) were in a cohort of pediatic Hodgkin’s Lymphoma survivors. These factors
female and about half (53%) were aged between 26-40 years. Less could easily be targeted to improve adherence to care of this vul-
than a third (27%) had no formal education. The children of majority nerable population who are at risk for late effects from their cancer
(84%) were still undergoing treatment. The most common diagnoses treatment.
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S526 of S635 ABSTRACTS

P046 / #1572 PALLIATIVE CARE FOR CHILDREN AND admissions with febrile neutropenia were registered.Total 38 patients
ADOLESCENTS WITH CANCER IN ZAMBIA with confirmed COVID19 infection were enrolled.Mean age at pre-
sentation was 7.47 years +/- 4.60 SD,with male to female ratio of
Wendy Tapalo 4:1.Fifty eight percent of COVID19 infections were community and
Cancer Diseases Hospital, Palliative Care, Lusaka, Zambia 42% where hospital acquired.Mean duration of stay in the hospi-
tal was 16 days +/- 14 SD.Sixty three percent patients were with
Background and Aims: Background Pediatric Palliative care is special- hematological malignancies,18.5% with solid tumors,10.5% patients
ized medical care for children living with serious and chronic illnesses with benign hematological disorders and 8% with Histiocytosis syn-
focusing on relief from symptoms/stress to improve the quality of life. dromes.Forty percent cases were detected during pre-op screening
Palliative care began in Zambia in 1992. The Cancer Diseases Hos- while 60% were investigated for atypical pneumonia.Thirty one
pital sees about 160 newly diagnosed children a year. Aims Explore percent patients were asymptomatic,24% had mild,16% moderate
Care givers perspective of pediatric palliative care at Cancer Diseases and 29% had severe disease.Chest X-ray showed no abnormality in
Hospital. 29% cases,bilateral pulmonary infiltrates in 29%,lobar consolidation
Methods: Structured interviews were conducted with caregivers at in 18.5%,mediastinal widening in 10.5%,ARDS in 10.5% and pleural
Cancer Diseases Hospital by a palliative care nurse and 4 pediatric effusion in 2.5% cases.Delay in the diagnosis of primary disease was
nurses using a questionnaire (25 items). Answer choices: yes, no, never observed in 37%,and treatment delay/interuuption was observed in
and some free text. Interviews conducted in Nyanja (local language) for 66% cases.Forty five percent patients were sent to home quaran-
32 caregivers and English for 8. Caregivers consent was obtained, and tine,32% patients expired,16% discharged;5% were shifted back to
interviews took 20 minutes. oncology ward for further treatment and 2.5% patients left against
Results: Most caregivers (70%) were aware about pediatric palliative medical advice.Supplemental oxygen,mechanical ventilation and
care services, but the rest did not understand the significance of holis- absolute neutrophil counts were the statistically significant factors
tic care. Most (60%) were concerned about the limited number of (p-value <0.05) associated with the outcome.Three peaks of infections
palliative care staff. Most did not want the child to be informed of their were observed in 15 months duration and frequency decreased in the
diagnosis; mothers complained fathers not participating in the child’s second half of study period.
care. Conclusions: Incidence of COVID19 infection seems to be very low in
Conclusions: In Zambia, children are left out of decision-making; care- pediatric hematology/oncology patients. Chemotherapy-induced neu-
givers assume they would be devastated. However, teenagers find tropenia, and respiratory failure is associated with poor outcome. After
solace in talking with nurses about their disease. These issues make the provision of vaccination the frequency of COVID19 infections
it difficult for palliative care nurses to render all services. Most Zam- decreased.
bians believe cancer is due to witchcraft, although most caregivers
expect their children to be cured. Unfortunately, they trust more in tra-
ditional medicines than biomedicine, so children are brought late for P048 / #813 PARENTS’ UNDERSTANDINGS AND
treatment. There is need for more trained personnel (pediatric pallia- EXPERIENCES OF PHYSICAL ACTIVITY IN CHILDHOOD
tive care nurses), nurses advocating for children’s involvement in their CANCER SURVIVORS IN SINGAPORE: A QUALITATIVE STUDY
care and sensitization about palliative care and childhood cancer.
Lindsey Weller1 , Debbie Cavers2 , Neneh Rowa-Dewar2
1 KK Women’s and Children’s Hospital, Physiotherapy, Singapore, Singapore;

P047 / #1734 COVID19 INFECTION IN PEDIATRIC 2 University of Edinburgh, Public Health, Edinburgh, United Kingdom

HEMATOLOGY/ONCOLOGY PATIENTS IN A DEVELOPING


COUNTRY Background and Aims: Childhood cancer survivors are more likely
to be sedentary and have an increased risk of developing chronic ill-
Rahat Ul Ain, Mahwish Faizan nesses and reduced quality of life. The current evidence base affirms
The Children’s Hospital Lahore, Pediatric Hematology/oncology And Bone the efficacy of physical activity to promote health and wellbeing in
Marrow Transplant, Lahore, Pakistan childhood cancer survivors, but few studies explore the of parents of
childhood cancer survivors on physical activity, despite the key role
Background and Aims: To determine the characteristics of COVID19 parents could play in encouraging physical activity. This study aims
infection in pediatric hematology/oncology patients in a developing to explore the understandings and role parents of childhood cancer
country. survivors in Singapore may have in regard to physical activity.
Methods: Prospective cohort study conducted from January 2021 till Methods: Participants were recruited through the Singapore Chil-
March 2022. Pediatric hematology/oncology patients, under 16 years dren’s Cancer Foundation. Semi-structured online interviews were
of age, with a positive PCR for COVID19 infection were enrolled. conducted with seven parents. Each interview lasted about an hour.
Results: During the study period 1784 new patients of pediatric With consent, interviews were recorded, transcribed verbatim and
oncology,833 new patients of pediatric hematology,and 1821 analysed using themati[LW1] c analysis, assisted by QSR NVivo.
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ABSTRACTS S527 of S635

Results: Three main themes were identified; 1) understanding and per- Conclusions: Siblings of children with terminal illness are at substan-
ceived value of physical activity, which describes parental views on tial riskfor PTSS, depression, anxiety, low self-cocept, internalizing,
the importance of physical activity, 2) influences on physical activity, externalizing, and totalproblems. Recommendation: Create supporting
describing the barriers and enablers of physical activity and 3) impact groups for siblings as an ongoing servicewhere siblings of children with
of cancer, which describes the side-effects of cancer that potentially terminal illness as cancer have an opportunity to meet withothers in a
mediate levels of physical activity in childhood cancer survivors. Par- similar situation and share their experiences and feelings.
ents reported that childhood cancer negatively affects quality of life
and participation in physical activity. The determinants of participa-
tion in PA are multifaceted and the socio-ecological and health belief E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
models were used to demonstrate how these factors are interlinked. Project
Conclusions: Participation in physical activity is influenced at an indi-
vidual, family, community and societal level. Improved understanding NURSING POSTER SESSION
acquired through this research can be used to shape paediatric cancer
care practices in Singapore. It also paves the way for further research P065 / #1494 INCREASING KNOWLEDGE FOR THE
in more diverse groups to inform an intervention study to optimise NECESSITY OF LONG-TERM FOLLOW-UP CARE AMONG
support for this population. PEDIATRIC CANCER SURVIVORS: A QUALITY IMPROVEMENT
INTERVENTION

E-Poster Topic: AS04 Nursing / AS04.b Research Shiley Aguilar, Margaret Parmeter, Kiranmye Reddy, Mehmet Okcu
Baylor College of Medicine, Pediatric Hematology Oncology, Houston,
NURSING POSTER SESSION United States of America

P064 / #1379 PSYCHOLOGICAL FUNCTIONING FOR Background and Aims: More than 60 percent of pediatric cancer
SIBLINGS OF CANCER CHILDREN : NURSING STUDY survivors experience at least one treatment-related late effect while
less than half adhere to recommended long term follow-up care. We
Somaya Abouabdou hypothesized that the majority of caregivers/survivors lack knowl-
Faculty of Nursing Suez canal University, Psycho-oncology Nursing, Ismailia, edge regarding the need for long term follow-up and risks for late
Egypt effects.
Methods: Using quality improvement methods we developed an inter-
Background and Aims: Background: The sibling relationship is usu- vention and surveyed solid and brain tumor caregivers or survivors (16
ally the first, most intense, and longest peer relation that an individual years and older) treated with chemotherapy and/or radiation and 3-
will ever have. Therefore, having a brother/sister with terminal illness 24 months off therapy. Survey questions inquired the length of long
like cancer poses a risk to the siblings’ mental health because of the term follow-up, the reason for life-long follow-up and possible late
stress they are under and insufficient support from others. Aim: This effects. Subjects were provided with the correct answers. We explored
study aimed to assess psychological functioning for siblings of cancer whether the questions triggered any anxiety and rated anxiety on a 10-
children in Egypt point scale. We repeated the same process and prospectively assessed
Methods: Design: descriptivecorrelational design was adopted for this percent change from baseline of the following: Correctly acknowledg-
study. Sample: purposeful sample of eighty onesibling of children with ing need for lifelong follow-up care and at least two late effects; How
cancer and their mothers. Tool of data collection: the data wasgath- often anxiety is endorsed and rated greater than 5 out of 10 point
ered through structured interview where the tool was divided into two scale.
parts: the first partincludes: demographic characteristics of the studied Results: To date, we completed two PDSA cycles with 29 patients and
subject, and the second part includesthree adopted tools: (I) The Child 10 with two visits. From first to second visit, correct responses for fol-
PTSD Symptom Scale (CPSS), (II) Beck Youth Inventory(BYI) (Second low up duration increased from 10 to 60 percent. Those who identified
edition), (III) Achenbach’s Child Behavior Checklist (CBCL). at least two late effects correctly increased from 40to 70 percent. Dis-
Results: Allsiblings of children with cancer in this study have met cri- cussing late effects caused anxiety in 60 percent at the first visit and
teria of post-traumatic stresssymptomatology (PTSS) and nearly half half of those rated greater than 5 on the 10 point scale. At the sec-
of them had severe symptoms. In addition, thehighest percentage of ond visit, only 20 percent reported any anxiety and none greater than
them had much lower than average self-concept, severe anxiety andde- 5 points.
pressive symptoms, average anger and disruptive behavior symptoms, Conclusions: A simple educational intervention led to a significant
and clinical range ofthe internalized, externalized and total prob- increase in knowledge regarding need for lifelong follow-up in sur-
lem. Also, there was a significant relationbetween the siblings’ PTSS vivors, their late effects, and a decrease in anxiety associated with late
and their self-concept, anxiety, depression, and internalizing andtotal effects. We will assess whether these improvements will be sustained
problems. at the next time point.
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S528 of S635 ABSTRACTS

E-Poster Topic: AS04 Nursing / AS04.b Research P067 / #421 CHILDREN’S NARRATIVES OF SUPPORT FROM
PARENTS WHEN EXPERIENCING FEAR RELATED TO ACUTE
NURSING POSTER SESSION LYMPHOBLASTIC LEUKEMIA

P066 / #624 VALIDATING A QUESTIONNAIRE TO ASSESS Agneta Anderzén Carlsson1 , Ingela Leibring2
THE USE OF COMPLEMENTARY HEALTH APPROACHES IN 1 Örebro University, University Health Care Research Center, Örebro, Swe-
CHILDREN WITH CANCER: FACE AND CONTENT VALIDITY den; 2 Karlstad University, Faculty Of Health, Science And Technology,
TESTING (PHASE 2) Institution For Health, Karlstad, Sweden

Mohammad Alqudimat1 , Karine Toupin April2 , Paul Nathan3 , Lindsay Background and Aims: Children diagnosed with Acute Lymphoblastic
Jibb1,4 , Charles Victor5 , Jennifer Stinson1,6 Leukemia (ALL) typically undergo intense treatment with frequent hos-
1 University of Toronto, Lawrence S. Bloomberg Faculty Of Nursing, Toronto, pitalizations. Medical, as well as existential fears have been identified.
Canada; 2 University of Ottawa, School Of Rehabilitation Sciences, Ottawa, It has also been found that children’s coping strategies develop during
Canada; 3 The Hospital for Sick Children, Hematology/oncology, Toronto, their illness trajectory. The literature on what children with ALL find
Canada; 4 Hospital for Sick Children, Child Health Evaluative Sciences, to be valuable support from parents when experiencing fear is sparse.
Toronto, Canada; 5 University of Toronto, Institute Of Health Policy, Manage- Thus, the aim of this presentation is to describe what young children
ment And Evaluation, Toronto, Canada; 6 Hospital for Sick Children, Child find to be important support from their parents when experiencing fear
Health Evaluative Sciences And Chronic Pain Program, Toronto, Canada related to ALL.
Methods: The study had a longitudinal descriptive qualitative design.
Background and Aims: Complementary health approaches (CHA) Thirteen children (3 girls and 10 boys), initially 5-9 years old were
such as naturopathy, chiropractic, and yoga are commonly used by interviewed once to three times during their treatment period (approx-
children with cancer globally. Discussions between patients/parents imately 2 months after the diagnosis, after 1 year, and at end of treat-
and health care providers (HCP) about CHA are often infrequent or ment). Data were analyzed using a matrix-based qualitative analysis
superficial and could be facilitated by a standardized questionnaire- method.
based approach. A systematic review by our team showed initial Results: The parents’ physical and emotional closeness was the most
evidence of validity for a paper-based generic questionnaire, the frequently reported support. It eased the children’s medical and exis-
“Which Health Approaches and Treatments are you using?” (WHAT) tential fears. The children also found it supportive when the parents
tool. We are using a sequential approach to adapt and validate the facilitated for them to participate in their care and when the parents
WHAT questionnaire for use by HCP initiating CHA discussions with acted as their advocate. Other supportive measures were offer-
patients/parents. As part of this approach, this study aimed to test the ing distraction, talking to the child about their fears, assisting the
face and content validity of the WHAT questionnaire for children with professionals in alleviating pain and fear, being playful and encour-
cancer. aging. Five children also appreciated when their parents restricted
Methods: Children with cancer, parents, and HCP (n=5-7 from each them, during medical procedures. The experiences of support var-
group per round) were recruited at the Hospital for Sick Chil- ied between children and between different time points during
dren and invited to participate in rounds of individual interviews to treatment.
determine item relevance, comprehensiveness, and comprehensibil- Conclusions: Although being quite young, the children were able
ity of the WHAT questionnaire. A cognitive interview technique was to describe what they found to be supportive when experiencing
used. fear, or for preventing fear. The parental support had an impact
Results: The first round of cognitive interviews was completed by 19 on the child’s emotional, social and physical wellbeing. Profession-
participants (5 children, 7 parents, 7 HCP). Feedback revealed the fol- als should encourage parents to stay with their child, and offer
lowing: short completion time was appropriate for clinical use, and support to the parents, so that they in turn can support their
items were comprehensive. However, the paper-based format was not child.
user-friendly (limited space with a busy layout); the generic items were
vague; and the items should be focused for clinical (as opposed to
research) use. Our team modified the questionnaire to address these E-Poster Topic: AS04 Nursing / AS04.a Education
comments, and changed the current version into an electronic cancer-
specific questionnaire for use in clinical settings. Preliminary results of NURSING POSTER SESSION
the second round completed by 10 participants (4 children, 5 parents,
1 HCP) revealed no need for further changes. P068 / #1233 CARING FOR CARER AND CARE PROVIDERS
Conclusions: The WHAT questionnaire has been modified based on PERSPECTIVE ON COMPASSION FATIGUE: A SHORT VIDEO
children with cancer, parents, and HCP suggestions. The next step is FROM MCM GRANT
measurement property testing of the modified WHAT questionnaire in
pediatric oncology. Shenila Anwarali1 , Rehana Punjwani2
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S529 of S635

1 Aga Khan University School of Nursing and Midwifery, School Of Nursing, clinical challenge. Case Study: Patient is a 14-year-old male who pre-
Karachi, Pakistan; 2 DOW University of Health Sciences, Nursing Services, sented at 11 years of age with a history of migraines, worsening
Karachi, Pakistan headaches, nausea, and vomiting. MRI after emergent EVD placement
revealed a mixed solid and cystic lesion centered in the right temporal
Background and Aims: Around 8000 new cases of Pediatric Oncology lobe and thalamus.
being identified annually in Pakistan. Half of the population coming to Methods: Patient underwent two subtotal resections followed by VP
pediatric oncology centers are staged with advanced disease. This can shunt placement. Complicated pathology findings required consider-
be difficult for healthcare workers as well as patient’s family. ation for standard chemotherapy versus proton radiation therapy. At
Methods: With help of Sanofi Espoir My Child Matters grant- train- this time, he was referred to our Precision Genomics Neuro Oncol-
ing and education of Nurses in palliative care in Pakistan. A video ogy program for tumor molecular characterization. While patient was
was created on identifying needs of caregiver and carers who had recovering, he presented with severe headaches. Imaging revealed
taken care of Pediatric Oncology patients. Psychosocial department 4 overshunting and right sided subdural hematoma, evacuation with burr
Bereaved families were identified along with healthcare providers who holes was performed.
frequently took care of these children. Parent and healthcare workers Results: Somatic tumor testing revealed BRAF V600E mutation and
were invited to explain their journey of being an immediate care- allowed for enrollment in Novartis CDRB436G2201. Patient was
giver. Their expression and feeling were recorded to be later turned assigned to the standard treatment arm and received Carboplatin and
into educational video as exemplar for other families and healthcare Vincristine, per CCG 9952A. Disease burden continued to progress
providers. through cycle 7, and per central review patient was transistioned to the
Results: It was peculiar experience where parents and healthcare Dabrafenib and Trametinib arm of study.
workers were invited to share deepest moments of grief on video. Conclusions: Imaging was performed 5 weeks post treatment transi-
During, interview various theme emerged; communication gap, not tion, which revealed interval reduction of dominate tumoral cyst in the
understanding patient and family’s needs, sibling neglect and social right thalamus and heterogenous solid lesion. Disease burden still con-
support. Parents confronted their children were referred to as degrad- tinues to decrease with therapy. This case illustrates a clear benefit of
ing metaphors, Parents were not provided social support to take care using molecular guided therapy for improvemet of patient quality of life
of their homes and other children, some parents were shouted at for in the treatment of PXA’s.
not being able to follow their routine appointments. Healthcare worker
shared, they grieved same way; explained their compassion fatigue and
ways of dealing with it. P070 / #1920 NUTRITIONAL DISORDERS AMONG
Conclusions: Pediatric Oncology can be strenuous domain for nurses, CHILDREN WITH CANCER RECEIVING CHEMOTHERAPY AT
doctors, support staff, and psychosocial workers. It can be draining THE PAEDIATRIC ONCOLOGY UNIT, KOMFO ANOKYE
physically and emotionally for parents and their families’ especially TEACHING HOSPITAL, GHANA
when outcomes are poor. Caregivers and carers need to focus on their
mental health during this tough time of caring for pediatric oncol- Comfort Asoogo, Vivian Paintsil
ogy patient. Hospital should provide extra support for individuals that Komfo Anokye Teaching Hospital, Department Of Child Health, Kumasi,
are suffering with compassion fatigue, their mental wellbeing will help Ghana
improve outcomes of other patients and families.
Background and Aims: Background Chemotherapy and its outcomes
are potentially detrimental to nutritional status; and as a result, there
E-Poster Topic: AS04 Nursing / AS04.b Research is increasing confirmation that nutritional status might influence the
outcomes of chemotherapy. Mucosal ulceration due to chemotherapy
NURSING POSTER SESSION presenting as cheilosis, mucositis, stomatitis, glossitis, and esophagi-
tis painfully obstruct ingestion of nutriments.Nearly all antineoplastic
P069 / #1626 IMPROVED QUALITY OF LIFE USING drugs produce nausea and vomiting while several results in diarrhea;
MOLECULAR GUIDED THERAPY: A CASE REPORT constipation may also occur. Adequate nutrition during cancer plays a
decisive role in several clinical outcome measures, such as treatment
Morgan Schmitt1 , Beth Armstrong2 response, quality of life, and cost of care. Objective To assess the nutri-
1 Riley Hospital for Children, Pediatric Hem/onc, Indianapolis, United States tional status and treatment outcome of children with cancer receiving
of America; 2 Riley Hospital for Children at IU Health, Hem/onc, Indianapolis, chemotherapy at Komfo Anokye Teaching Hospital, Kumasi.
United States of America Methods: The study used structured questionnaire as the primary
source of data collection to interview key people such as parents, care-
Background and Aims: Pleomorphic Xanthoastrocytoma (PXA) is a takers, and guardians. Secondary data, from hospital folder was also
rare type of central nervous system tumor among children. Despite reviewed.Data that were generated was subjected to both descriptive
the fact that they have a high cure rate, PXA’s remain a significant and statistical analyses using stata11.0
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S530 of S635 ABSTRACTS

Results: Finding showed a significant relationship between anemia and preparing them to be resilient in the face of future public health
dietary diversity score at three months of chemotherapy (p = 0.016). emergencies.
Three months after chemotherapy, participants had moderate anemia
(87.4%) and highest poor DDS compared to normal (2.2%), mild (4.1%)
and severe anemia (6.3%) children with cancer (p = 0.016). This means E-Poster Topic: AS04 Nursing / AS04.a Education
that poor diet diversity could be associated with prevalence of anemia
in children with cancer. NURSING POSTER SESSION
Conclusions: There was high prevalence of wasting and anemia at base-
line, one to three months of chemotherapy. Poor diet diversity was P072 / #1919 EFFECTS IN EDUCATION COMPETENCY
found significantly higher among male children with cancer and lym- SELF-ASSESSMENT OF A BASIC HEALTH EDUCATION ONLINE
phoma participantThere was significant relationship between anemia COURSE FOR HEALTH PROFESSIONALS IN PEDIATRIC
status and dietary diversity at three months of chemotherapy. HEMATOLOGY-ONCOLOGY: A PILOT STUDY

Aprille Banayat1 , Kenneth Henry Goyena2 , Ian Louis Valenzuela1


E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice 1 University of the Philippines Manila, College Of Nursing, Manila, Philip-
Project pines; 2 UP-Philippine General Hospital, Pediatric Hematology/oncology
Ward, Manila, Philippines
NURSING POSTER SESSION
Background and Aims: Patient/family education in childhood can-
P071 / #1518 THE IMPACT OF THE CORONAVIRUS DISEASE cer care is recognized as one of the most effective strategies for
(COVID-19) PANDEMIC ON THE PEDIATRIC ONCOLOGY better patient and family outcomes across the childhood cancer con-
NURSES MENTAL HEALTH IN RABAT MOROCCO tinuum; yet health professionals have limited training on the basics
of health education. This study aims to: (1) describe participants’
Mohammed Bahmou1 , Malika Meskini1 , Younes Labrini1 , Julia current patient/family education practices; and (2) compare their con-
Challinor2 , Leila Hessissen1 fidence level on relevant education competencies pre- and post-course
1 Ibn Sina University Hospital, Pediatric Oncology Department, RABAT, attendance.
Morocco; 2 University of California San Francisco, School Of Nursing, San Methods: A 10-hour online training course on basics of health edu-
Francisco, United States of America cation was implemented for health professionals in childhood cancer
care. Participants answered a pre- and post-course self assessment
Background and Aims: The coronavirus disease (COVID-19) pan- survey to describe their current practices, and measure their confi-
demic has caused a major health crisis in most countries, particularly dence level on relevant education competencies. Descriptive statistics
the mental health of health workers.The impact of the pandemic on were computed, thereafter.
the care of patients followed at the Department of Hematology and Results: Twenty-two (22) participants included 15 (68%) staff nurses,
Pediatric Oncology in Rabat has been well documented. However, no 4 (18%) out-patient clinic staff, and 3 (14%) nurse navigators. All units
study has measured its impact on nurses. Objective: Identify areas in the selected Philippine public end-referral center caring for children
of pediatric oncology nurse mental health most impacted and coping with cancer were represented. Majority (77%) implements an individ-
strategies. ual approach in education. Only 2 (9%) stated that everyone taking
Methods: Study based on a questionnaire derived from the Ameri- care of the child is responsible for education. Most (32%) use teach
can Nurses Association Covid-19 impact survey, translated locally to back method to assess understanding. Confidence level on delivering
French and distributed to all department nurses. The 14 questions health education increased from a mean of 3.5 (SD = 0.67) to 4.4 (SD
coveredemotional feelings, behavior related to stress experienced and = 0.51). Mean self-assessment increased on all relevant competen-
methods of coping. cies pre- and post-course attendance. Assessment of education needs
Results: Nineteen among 22 nurses participated anonymously. Eight increased from a mean of 6.05 (SD = 1.91) to 8.33 (SD = 1.83); provi-
were diagnosed withCOVID 19. Most (17/ 19) felt overwhelmed sion and reinforcement of education on diagnosis, treatment options,
and depressed, but many (17/19) feltoptimistic and confident. The side effect management, and post-treatment care and survivorship
pandemic impacted sleep (10/19) and appetite (8/19).Two nurses from 6.18 (SD = 2.06) to 8.33 (SD = 1.87); promotion of autonomous
experienced domestic abuse. Strategies used to overcome the cri- decision making from 6.27 (SD = 2.12) to 7.92 (SD = 2.15); and educa-
sis included spending time with family or nature, entertainment or tion and reinforcement of adherence from 6.5 (SD = 2.15) to 8.5 (SD =
spiritual practices. 2.24).
Conclusions: The pandemic (COVID-19) impacted the psychological Conclusions: Targeted training should be continued to increase rel-
mental health of pediatric oncology nurses in Rabat. The findingsem- evant education competencies of health professionals in pediatric
phasizes the need of investing in the well-being of nurses, and hematology-oncology.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S531 of S635

E-Poster Topic: AS04 Nursing / AS04.b Research E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
Project
NURSING POSTER SESSION
NURSING POSTER SESSION
P073 / #1015 BEREAVED MOTHERS’ AND FATHERS’
PERCEPTIONS OF COMMUNICATION ABOUT THEIR P074 / #1757 IMPACT OF THE COVID 19 PANDEMIC ON
CHILD’S CANCER DIAGNOSIS AND WHEN THE ILLNESS THE CONTINUITY OF TREATMENT IN PEDIATRIC CANCER IN A
BECAME INCURABLE - A POPULATION-BASED NATIONWIDE UNITY OF ONCOLOGY IN MEXICO
SURVEY
Norma Araceli Lopez Facundo, Jocelyn Becerril, Nancy Reyes,
Cecilia Bartholdson1 , Ulrika Kreicbergs2 , Josefin Sveen3 , Malin Marbella Esquivel, Patricia Estrada, Marta Cervantes Jiménez
Lövgren2 , Lilian Pohlkamp2 HOSPITAL MATERNO INFANTIL ISSEMYM, Pediatric Oncology, TOLUCA,
1 Karolinska Institutet, Women’s And Children’s Health, Stockholm, Swe- Mexico
den; 2 Marie Cedershiölds University, Palliative Research Centre, Stockholm,
Sweden; 3 Uppsala University, Department Of Medical Sciences, Uppsala, Background and Aims: The COVID-19 pandemic impacted health sys-
Sweden tems in unprecedented ways. In Mexico, most third-level hospitals
were converted into hybrid hospitals for the care of patients with Sars-
Background and Aims: A vast majority of parents and children want Cov-2 infection and at the same time for the care of cancer children.
honest communication and openness regarding diagnosis and progno- Strategies were designed to reduce the risk of infection however most
sis but conveying bad news to children and their family is a challenging patients have had contact with infected people. Aims: To know the
task for healthcare professionals. However, research shows that par- impact of the covid 19 pandemic in children with cancer of the unit and
ents often struggle to grasp such challenging information. The aim of oncology of the HOSPITAL MATERNO INFANTIL ISSEMYM in Toluca
this study was to describe bereaved mothers’ and fathers’ perceptions Mexico
of communication of their child’s cancer diagnosis and when the illness Methods: Cross-sectional cohort study. A random representative sam-
became incurable. ple of patients treated in the oncology unit was included . We analized
Methods: This study derives from a population-based nationwide Demographic, clinical, diagnosis and stage of oncological treatment,
postal survey, including 232 parents (135 mothers and 97 fathers) characteristics, Also the treatment conitnuity, punctuality and com-
who had lost a child to cancer 1–5 years earlier. The survey covered pliance were recorded. Reasons for delay and modifications of the
sociodemographic characteristics and study-specific questions about treatment were consigned
the parents’ experiences of their child’s illness and when the illness Results: We included 46 patients, 22 women, 26 men, 23 with
became incurable. leukemias, 26 with solid tumors, indicated treatment was 4 Surgery, 43
Results: Key findings of this study include that almost all (93%) parents chemotherapy, 2 radiotherapy . Treatment continuity decreased from
want information when their child’s illness become incurable. However, 75-90 % before March 2020 to 25-40 % in the first 6 months of pan-
analysis revealed that fathers to a lesser extent than mothers reported demic onset with decrease in punctuality, 36% had delay in treatment
that they were informed. Furthermore, parents reported that 13% of with chemotherapy, due to drug shortage that was aggravated by the
the children did not receive diagnostic information and 56% of the par- pandemic and 35% with delay in compliance in febrile neutropenic
ents reported that their child never received the information that their patients who they had WHO definition of suspected sars cov2. In 80
cancer was incurable. Fewer children were present when a bad prog- % the treatment was modified, 21 % due to toxicity, shortage of med-
nosis was communicated to the family than when the family received ications and delay of care in other areas due to infections in health
diagnostic information. staff
Conclusions: Most parents reported that they would like to know Conclusions: The COVID-19 pandemic has impacted globally, aggra-
when their child’s illness becomes incurable, and more than half of vating problems such as shortages of medicines. The multidisciplinary
the parents reported that their child never received this informa- team was insufficient in our hospitals with limited resources, in addi-
tion. Yet, it is unknown to what extent parents want their child tion the treatment was interrupted in all patients with respiratory
to be informed and reasons for the child being uninformed remain symptoms for 1-3 weeks. Rapid tests have optimized the time of care
unclear. in our hospital
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S532 of S635 ABSTRACTS

E-Poster Topic: AS04 Nursing / AS04.a Education 1 Queensland University of Technology, Cancer And Palliative Care Out-
comes Center- Child And Youth Program Lead, South Brisbane, Australia;
NURSING POSTER SESSION 2 Queensland University of Technology, Cancer And Palliative Care Outcome

Centres, South Brisbane, Australia; 3 Children’s Health Queensland, Oncol-


P075 / #1074 APPLYING THE SIOP NURSING BASELINE ogy, Brisbane, Australia; 4 Children’s Health Queensland, Oncology, South
STANDARDS IN PRACTICE-A NEW NURSE ORIENTATION IN Brisbane, Australia
KORLE-BU TEACHING HOSPITAL
Background and Aims: Children and adolescents experience can-
Enyo Bosumprah1 , Lorna Renner1,2 , Martekie Tackie-Martey3 cer at a time of significant developmental transition. Both disease
1 Korle-Bu Teaching Hospital, Child Health, Accra, Ghana; 2 University of and treatment impact psychosocial well-being in significant, persis-
Ghana Medical School, College of Health Sciences, Department Of Child tent ways. While the impacts are now described, and the need for
Health, Accra, Ghana; 3 Korle-Bu Teaching Hospital, Department Of Child psychosocial care is increasingly well recognized, to date, the barri-
Health, Accra, Ghana ers in access to care have not been well delineated. This is essential
to understand to facilitate access to appropriate care and improve
Background and Aims: Pediatric oncology in lower-and middle outcomes.
income- countries (LMIC) lack basic structure and terms of reference Methods: This study explored the barriers in access to psychoso-
for nursing care of children with cancer. The SIOP nursing baseline cial care for young people. Semistructured, audio-recorded interviews
standards serves as a guide for nurses in developing countries to offer were undertaken with 16 young people. Eligible participants were
quality nursing care for children with cancer. Empowering new nurses diagnosed within the previous 24 months and recruited through the
with skills and knowledge will go a long way to improve survival rates. Queensland Youth Cancer Service (QYCS). Transcribed interviews
The project was to equip new nurses in pediatric oncology with the were analyzed using content analysis.
requiste skills and knowledge. Results: Barriers in access to support were related to person-centered,
Methods: Ten (10) new nurses (100%) were posted to the pediatric service-related, and systemic factors. Barriers experienced at diagno-
oncology unit of the Korle-Bu Teaching Hospital in 2020. The request sis and during treatment were less common compared with barriers
for additional nurses was made using the SIOP nursing baseline stan- after treatment; these were significant and largely related to a lack of
dards as evidence to support more nurses. The nurses completed two holistic, multidisciplinary survivorship care.
weeks of didactic teaching and learning on various E-Poster Topics Conclusions: Barriers in access to psychosocial care are multifacto-
related to pediatric oncology nursing care using the sub saharan Africa rial, although most can be addressed through health-service responses.
Nursing Network educational pakage (SSANN) A pre and postest was Ensuring standardized referral and repeated introduction of psy-
administered. Competency assessment was done for each new nurse chosocial care for young people is imperative, regardless of location
three(3)months after working with senior nurse mentors. of treatment. Flexible services are especially important for patients
Results: Pre and post evaluations revealed that nurses increased their treated across different facilities. The development of comprehensive
knowledge of early signs of childhood cancer. Increased knowledge post-treatment survivorship models of care is also essential. Continued
occurred regarding tumor lysis syndrome with only 10% knowing evaluation of the experience of young people and the barriers they face
about the symptoms before the class and 90% had good understanding is also crucial to ensure responsive service development and promote
afterwards. Evaluations revealed nurses’ ability to quickly intergrate optimal care.
essential skills into nursing care.
Conclusions: The SIOP nursing baseline standards is an evidenced
based pediatric oncology nursing standards that can help improve P077 / #180 CHANGES IN BODY SIZE AND BODY
nursing care of children with cancer in developing countries. The COMPOSITION IN SURVIVORS OF CHILDHOOD CANCER: 7
SSANN educational package also when used in teaching new nurses YEARS FOLLOW-UP OF A PROSPECTIVE COHORT STUDY
posted to the units can help boost their confidence and improve
survival rates for patients. Aeltsje Brinksma1,2 , Esther Sulkers1 , Dorus Kouwenberg2 , Otto
Lelieveld3 , Annemieke Boot4 , Johannes Burgerhof5 , Wim Tissing2,6
1 University of Groningen, University Medical Center Groningen, Depart-
E-Poster Topic: AS04 Nursing / AS04.b Research ment Of Health Sciences, Groningen, Netherlands; 2 Princess Maxima
Center for Pediatric Oncology, Supportive Care, Utrecht, Netherlands;
NURSING POSTER SESSION 3 University of Groningen, University Medical Center Groningen, Beat-
rix Children’s Hospital, Center Of Rehabilitation, Groningen, Netherlands;
P076 / #628 BARRIERS IN ACCESS TO PSYCHOSOCIAL 4 University of Groningen, University Medical Center Groningen, Beatrix
SUPPORT FOR YOUNG PEOPLE WITH CANCER Children’s Hospital, Department Of Pediatric Endocrinology, Groningen,
Netherlands; 5 University of Groningen, University Medical Center Gronin-
Natalie Bradford1 , Lucy Holland2 , Rick Walker3 , Christine Cashion4 gen, Department Of Epidemiology, Groningen, Netherlands; 6 University
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S533 of S635

Medical Center Groningen, Department Of Pediatric Oncology, Groningen, provide well-being on patients in oncology ward are some of the main
Netherlands goals of the physiotherapy in Hospital Sant Joan de Déu.
Methods: A daily exercise program was implemented in September
Background and Aims: Cancer treatment is known to have impact on 2018 till it has to stope by the Covid-19 crises. It consists of differ-
nutritional status, and both underweight and overweight have been ent type of exercise for each day of the week. “Let’s exercise playing
reported in several studies in survivors. A limitation of most studies music” is the Tuesday exercise proposal. It is a link of Physiotherapy
is that they relied on retrospective data or were limited to a sub- (aerobic work, strength exercises, coordination, balance and joint and
group of patients. The current study aims to describe changes in body muscle flexibility) with Music Therapy, to transform these practice into
size and body composition prospectively in a heterogeneous sample of a more playful and enjoyable sessions and provide along with the phys-
childhood cancer survivors and to evaluate associated factors. ical benefits emotional and social ones. All patients of the oncology
Methods: Participants were 66 children diagnosed with heteroge- ward allowed to move out from their room were invited to the pro-
neous malignancies 0-18 y at diagnosis. Data of body size, body gram, which took place in the ward gym. A “Let’s exercise playing music”
composition, and associated factors were collected at diagnosis, one was a 30 minutes session, structured in 4 sections: Activation (aero-
year, and seven years after diagnosis. bic warm-up). Stations (work sites provided with an instrument to play
Results: In the total cohort mean BMI z-score increased during treat- while performing a strength exercise). Session ends with stretching and
ment. In children with hematological and brain malignancies mean relaxation. According to its musical characteristics special songs were
zBMI continued to increase after end of treatment leading to qua- selected to accompany the activity,.
drupling of the prevalence of obesity at seven years follow up. Low Results: The proposal was followed by 45 patients (17 females and
initial zBMI and maternal BMI were associated with increase in mean 28 males in an age range between 5 and 16 years old) with a total
zBMI. Mean fat mass (FM) z-score, already high at diagnosis, increased of 72 interventions. Patients, families and health workers value very
during treatment in children with hematological and brain malignan- positively the program, generating a special, atractive, motivating and
cies and evened out during follow-up. Changes in mean FM z-scores very moving atmosphere. It was well tolerated by patients while they
were predicted by type of malignancy (hematologic/brain malignancy enjoyed the sessions.
versus solid tumor). Mean fat free mass (FFM) z-score started low at Conclusions: The program was easily implemented. It provided
diagnosis, particularly in patients with brain tumors, increased dur- the patients with physical and emocional benefits and facilitated
ing treatment in patients with hematological and brain malignancies, socialization between them. It will be restarted as soon as Covid
though decreased in children with solid tumors. At seven years follow- allows it
up a clear increase to normal was seen. Older age and low mean zFFM
at diagnosis were found to be significant predictors for increase in
mean zFFM. E-Poster Topic: AS04 Nursing / AS04.a Education
Conclusions: The once obtained extra weight and fat mass remained
in survivors of hematological and brain malignancies. This stresses the NURSING POSTER SESSION
importance of life style interventions concurrent with therapy, espe-
cially for those who experience substantial gain in weight and fat mass P079 / #1850 CHEMOTHERAPY/BIOTHERAPY NURSE
during treatment. EDUCATION – CREATING A GLOBAL PROGRAM

Michele Casey1 , Joan Curry2 , Linda Abramovitz3


E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice 1 The Children Hospital at Westmead, Paediatric Oncology, Westmead, Aus-

Project tralia; 2 MD Anderson Cancer Centre, Pediatric Clinical Services, Houston,


United States of America; 3 University of California, San Francisco, School
NURSING POSTER SESSION Of Nursing And Global Cancer Program, San Francisco, United States of
America
P078 / #1378 LET’S EXERCISE PLAYING MUSIC
Background and Aims: The Association of Pediatric Hematol-
Gemma Calaf1 , Nuria Bonet2 , Carles Geli2 , Núria Padrós1 , Meritxell ogy/Oncology Nurses (APHON) has a well-established chemother-
Vigo1 apy/biotherapy curriculum which was created to provide nurses with
1 Hospital Sant Joan de Déu, Rehabilitation, Barcelona, Spain; 2 Hospital key principles and theoretical knowledge to administer chemother-
Sant Joan de Déu, Hospital Amic, Barcelona, Spain apy/biotherapy safely and competently. In a collaborative effort,
a pilot study was conducted to determine the feasibility of uti-
Background and Aims: The Hospital admission of a child or a teenager lizing the APHON provider chemotherapy certification to meet
diagnosed with cancer supposes a limitation of his daily physical activ- the required standard of practice for accreditation in Australia/
ity and his social life. Prevent and improve the lost of fitness and New Zealand.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S534 of S635 ABSTRACTS

Methods: Members of APHON Global Outreach and Chemother- program transitioned to an online group format for youth (6-11 years
apy/Biotherapy Committees held meetings via Zoom with the Aus- and 12-17 years).
tralia/New Zealand nurse leadership team to establish course struc- Methods: Anonymous surveys were distributed amongst 17 regularly
ture and content as well as addressing logistics. The pilot course was attending families (16 kids and 9 teens) to better understand moti-
held over a 4-week period with content for each module presented in 4 vators and barriers of online programming, safety, and satisfaction.
hour blocks virtually. Complimentary access to the Curriculum eBook Another survey was completed for 25 parents of children that never
and handouts via the APHON website were provided. Participants or occasionally joined the program (N=18,7) to understand non-user
were assessed via an exam at the end of the course. barriers.
Results: The course was conducted in May 2021. Seventeen nurses Results: Families primarily participated in the program to (1) improve
from Australia and New Zealand participated in the APHON fitness and (2) increase peer connections. All felt safe. 95% agreed
Chemotherapy/Biotherapy course, representing ten hospitals. All that the sport kit increased engagement during and after the session.
participants were experienced pediatric oncology nurses. There were 83% of parents felt program participation increased their child’s fit-
five instructors, one coordinator and three APHON staff from the ness level and confidence to exercise and 100% would recommend it to
United States who taught and coordinated the program. There was other families. Parents whose child/teen rarely attended PEER online
100% participation and attendance, and all nurses passed the exam recognized that ‘physical activity is important for their child/teens
on first attempt. Weekly debriefings and course evaluations provided recovery’, ‘helps their child/teen to socialize’, and ‘improves focus and
feedback on course content and delivery. Challenges included navi- academics’ (80%, 67%, 83% strongly agree, respectively). Some of the
gating nine time zones, cultural and healthcare system differences, key reasons the children/teens occasionally or never participated in
and access to course materials. Both instructors and participants PEER online were because day/time did not work and/or nervousness
benefited by the exchange of knowledge and clinical practice, teaching to participate. Fatigue was another frequent reason for not participat-
strategies, and discussion about advanced clinical nursing roles and ing, however, 72% of parents indicated an understanding that physical
responsibilities. activity decreases cancer-related fatigue. Finally, 84% mentioned that
Conclusions: This pilot program highlights the importance of global col- their oncologist recommended an active lifestyle after treatment vs
laboration and lays the foundation for future development of shared 60% during treatment.
education resources between countries. This international education Conclusions: This program was engaging, safe, accessible, and
experience promotes a rich opportunity for nurses to reflect, learn and improved confidence to exercise for children affected by cancer. Fun
improve clinical care for patients and families. and inclusion of socialization are key elements to increasing enjoyment
and adherence. Providing a sport kit is recommended. Education
of families and health care teams about exercise benefits and local
E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice physical activity programs are important to decrease barriers to
Project participation.

NURSING POSTER SESSION


E-Poster Topic: AS04 Nursing / AS04.b Research
P080 / #1561 LESSONS LEARNED FROM INNOVATING THE
PEDIATRIC CANCER ENGAGING IN EXERCISE FOR RECOVERY NURSING POSTER SESSION
(PEER) PROGRAM TO AN ONLINE MODALITY DUE TO
COVID-19. USER & NON-USERS PERSPECTIVES P081 / #1588 DETERMINATION OF PAEDIATRIC ONCOLOGY
PROVIDER ATTENTION TO CARE-GIVER BURDEN AND STRESS
Carolina Chamorro Vina1,2 , S. Nicole Culos-Reed1 , Gregory Guilcher3 , MANAGEMENT-:, ZIMBABWE
Fiona Schulte3 , Shaelene Standing4 , Rachel Mcinnes2 , Mackenzie
Murawsky5 Lomtunzi Chidziwa, Tendai Chisamba
1 University of Calgary, Faculty Of Kinesiology, Calgary, Canada; 2 Kids Can- Parirenyatwa Group of Hospitals, School Of Nursing, Harare, Zimbabwe
cer Care Foundation of Alberta, Peer Program, Calgary, Canada; 3 Alberta

Children’s Hospital, Hematology/oncology/blood & Marrow Transplant Pro- Background and Aims: Approximately 250 children/adolescents a
gram, Calgary, Canada; 4 Cumming School of Medicine, University of Calgary, year are diagnosed with cancer in Zimbabwe; all are treated at
School Of Medicine, Calgary, Canada; 5 Faculty of Health Sciences, Univer- Parirenyatwa Group of Hospitals (PGH), the largest tertiary and
sity of Ottawa, Health Sciences, Ottawa, Canada referral hospital. Twenty nurses provide care on Ward A4 Special.
Care-givers are key in the delivery of paediatric palliative care, from
Background and Aims: The focus of the PEER program is to increase diagnosis to end of life. The World Health Organisation has acknowl-
physical literacy, fitness and socialization for children affected by can- edged the significance of family care for sick children, especially in
cer and their siblings. In response to the COVID-19 pandemic, the cases of cancer and other chronic diseases. Aim
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ABSTRACTS S535 of S635

Identify the needs of care-givers of children and adolescents with tion, tunnel infection, CVC-related bloodstream infection, mechanical
cancer at PGH. event, and premature removal were assessed.
Methods: This cross-sectional study was conducted with care-givers Results: During a 213-day period (range, 84-420), one CVC partial
of children with cancer. Eligibility criteria: 20-40 years, speak English, occlusion occurred in HS at 77 days post-insertion, but no complete
Ndebele or Shona, and no cognitive deficit. A researcher-developed occlusion happened in total patients. Eight CVCs (NS and HS in 4 each)
questionnaire based on a E-Poster Topical literature search was cre- were prematurely removed at a median of 226 days (range, 168-420).
ated in collaboration with other hospital-based palliative care special- Reasons for premature removal were documented bacterial infections
ists. The questionnaire included 5 items, scored on a Likert-type scale in 3 (staphylococci in 2 and enterococcus in 1), exit-site infection in 3,
(1- 5) and was given to the care-givers to complete anonymously at the tear in the external portion of catheter in 1 and death without catheter-
hospital. related events in 1 patient. At a median follow-up of 388 days (range,
Results: A total of 50 care-givers participated (10 male and 40 female). 271 to 563), CVC occlusion-free survival was 96% ± 4.4% (100% for
The majority (80%, n=40) expressed that they were extremely devas- NS versus 91% for HS. CVC occlusion-free survival was not different
tated when they heard their child’s diagnosis. All participants indicated between two flushing methods (P>0.05).
they were struggling to cope when asked how the child’s condition Conclusions: Normal saline seems to be as effective as heparinized
had impacted the family’s living conditions and all reported being solution for maintaining patency of placed CVC in pediatric patients.
depressed. Funding of the child’s treatment was (60%, n=30) self- Further study including more cases is warranted to verify our finding.
funded, and (20%, n=10) social welfare and assistance from a non-
governmental organisation.
Conclusions: This study provided evidence that the care-givers of chil- P083 / #271 TAKING OVER CARE FOR UKRANIAN
dren and adolescents with cancer in Zimbabwe suffer from depression CHILDREN WITH CANCER: EXPERIENCES AT THE PRINCESS
due to the child’s illness and the need for funding of the prescribed MAXIMA CENTER
treatment. As health-care providers, and nurses specifically, we need to
cushion the care-givers from the point of diagnosis by providing more L.A. De Koning-Smits1 , K.C.J.M Kraal2
effective counselling and support before and during treatment as well 1 Princess Maxima Center, Nursing Staff, International Office, Utrecht,
as lobbying for financial assistance. Netherlands; 2 Princess Maxima Center, Solid Tumors, International Office,
Utrecht, Netherlands

E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice Background and Aims: The war in Ukraine has caused an acute emer-
Project gency in the care and treatment of children with cancer. It is important
that care for Ukrainian children with cancer can be continued, whether
NURSING POSTER SESSION in the country itself or elsewhere in Europe.
Methods: The International Office of the Princess Maxima Center is
P082 / #936 COMPARISION OF NORMAL SALINE AND a member of the St. Jude4Ukraine group, which coordinates receiving
HEPARINIZED SALINE SOLUTION FOR FLUSING CNTRAL our target group from Ukraine in order to aid to children with cancer in
VENOUS CATHETER IN PEDIATRIC PATIENTS Ukraine. This is carefully coordinated and attuned with all the affiliated
SIOP countries.
Eun Seok Choi, Ho Joon Im, Sung Han Kang, Hyery Kim, Kyung-Nam Results: All children and family members have been picked up from
Koh Poland by plane by our medical team. A medical check has been per-
Asan Medical Center, University of Ulsan College of Medicine, Pediatrics, formed upon arrival in our center; the children were organized by
Seoul, Korea, Republic of specialty and medical teams (including the pharmacist) were ready to
perform a second triage and intake with support of an interpreter. All
Background and Aims: We compared the efficacy of normal saline children have been scheduled for different appointments the next few
(NS) with heparinized solution (HS) as a method of flushing central days. The families have been accommodated by host families, mostly
venous catheter (CVC) in pediatric patients undergoing hematopoietic from our center, these families have been instructed in advance by sev-
stem cell transplantation (HSCT). This was a prospective, randomized, eral professionals from our center. The focus is on providing families
double-blind clinical trial. with daily structure as soon as possible, enabling children to go back to
Methods: Twenty-two pediatric patients had newly placed CVCs for school and giving help from the government (financial, care) they are
allogeneic HSCT between February 14, 2018 and December 3, 2018 entitled to. The use of interpreters and other tools to communicate is
at our center. All patients were implanted with a triple-lumen 12.5 of great importance.
French tunneled-Hickman catheter through internal jugular or subcla- Conclusions: In order to treat children with cancer from Ukraine, it is
vian veins. Of 22 patients, 11 were flushed their CVC with HS and 11 important to focus on a number of themes; legal, medical, psychoso-
with NS. CVC-related complications including occlusion, exit-site infec- cial support for families and for professionals, the following E-Poster
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S536 of S635 ABSTRACTS

Topics are important: Transport, capacity management, housing, cul- different examiners, indicating that QMT is a feasible and easily
ture differences (also in care) and communication. applicable tool.

E-Poster Topic: AS04 Nursing / AS04.a Education E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
Project
NURSING POSTER SESSION
NURSING POSTER SESSION
P084 / #1907 BEDSIDE ULTRASOUND TO ASSESS MUSCLE
MASS IS A FEASIBLE MEASUREMENT TOOL FOR HEALTH P085 / #177 AYA ROOMSERVICE: A MORE PLEASANT STAY
PROFESSIONALS FROM DIFFERENT BACKGROUNDS IN THE HOSPITAL

Wilson De Oliveira Jr1 , Mariana Murra2 , Carlos Eduardo Cavalcante3 , Kleo Dubois, Annemarie Coolbrandt
Leticia Tufi4 , Luisa Junqueira4 , Marco De Oliveira5 , Ricardo Da Costa4 University Hospital Leuven, Oncology, Leuven, Belgium
1 Hospital de Amor: Unidade Infanto Juvenil, Pediatric Surgery, Barretos,
Brazil; 2 Hospital de Amor, Pediatrics, Barretos, Brazil; 3 Hospital de Amor: Background and Aims: In University Hospital Leuven (Belgium), more
Unidade Infantojuvenil, Pediatric Radiology, Barretos, Brazil; 4 FACISB - Fac- than 200 AYAs (Adolescents and Young Adults) are diagnosed with can-
uldade de Ciencias da Saúde de Barretos "Dr Paulo Prata", Barretos School cer every year. These AYAs have age-specific health care needs that
Of Medicine, Barretos, Brazil; 5 Hospital de Amor, Center Of Epidemiology too easily stay undetected and unanswered in adult wards. A multi-
And Biostatistics, Barretos, Brazil disciplinary working group of the Leuven Cancer Institute has taken
important steps to promote age-specific care for AYAs in UZ Leuven.
Background and Aims: Ultrasonography can assist in clinical and ther- One of the initiatives is the optimization of the hospital stay of AYAs in
apeutic diagnosis; it is a non-invasive and operator dependent method. the adult wards.
It is a good tool to assess quadriceps femoris thickness and corroborate Methods: It is very important for young people to get on with their
nutritional data. A E-Poster Topic of extreme importance for the care lives. One of the objectives of the working group was to bring the nor-
of hospitalized children, especially oncological children who present mal living environment of young people into their hospital room. The
greater loss of lean mass. This study aims to evaluate performance idea was to offer them a box full of possibilities to relax during their
and reliability degree of measuring the Quadriceps Muscle Thickness stay and to decorate their room with accessories. Through a survey, the
(QMT), among examiners of different backgrounds (physicians, nurses, AYAs were allowed to decide what exactly should be in the box.
dietitians, medical and nutrition students). Results: This resulted in the AYA Roomservice: the young patient
Methods: A prospective and observational study was performed with chooses from the box what he want to use, just like a real roomservice.
health professionals related to the nutritional care of children with The purpose of the AYA Roomservice is fourfold: decorating the hospi-
cancer. The standardization of measurements and validation of image tal room, distraction, providing information resources and support. In
collection was performed in an 8-hour training structured course with addition to an information folder with ’Good to Know’ tips, there are
a practical evaluation at the end. All parameters were compared to mood lamps, an aroma dispenser, beautiful sheets and nice crockery
the gold standard (radiologist). Pearson correlation coefficient was to make the room ’AYAproof’. In addition, the box also contains board
used to calculate the realibity of the QMT between examiners and games and a tablet with a Netflix and Spotify account.
gold-standard. Conclusions: The project started in two departments in 2018. After
Results: Thirteen individuals from different professional categories a positive evaluation by the young people themselves, the project
participated. The group of participants was heterogeneous in terms was expanded in 2021 to seven other oncology units, including pedi-
of training time, area of expertise, and prior knowledge. In total, atric oncology. The project was made possible thanks to the support
311 images were examined by the trainer and compared to the of the Albert Fund and the help of volunteers. More information:
gold-standard. We observed a substantial (PCC=0.822) correlation AYA@uzleuven.be
between image acquisition using a structured theoretical-practical
course. It was also noted, with statistical relevance (p-value=0.03),
that individuals over 30 years of age presented better adequacy of P086 / #178 MY NEEDZ: A DIGITAL TOOL FOR AYA IN
the images throughout the various measurements. This fact can be UNIVERSITY HOSPITAL LEUVEN
explained that regardless of the contact or not with new technolo-
gies, the time spent in the profession may seem to impact this finding Kleo Dubois, Annemarie Coolbrandt
positively. University Hospital Leuven, Oncology, Leuven, Belgium
Conclusions: Through specific training, evaluating the student in the
different spheres of learning and retention of knowledge, it is possi- Background and Aims: Every year in Flanders (Belgium), slightly less
ble to reach a substantial correlation between measurements made by than 1000 ’adolescents & young adults’ (AYA) are diagnosed with
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ABSTRACTS S537 of S635

cancer. In Univesity Hospital Leuven, approximately 200 AYA are diag- from research and experiences of care providers and AYAs were
nosed with cancer every year. Due to their age and the stage of life in examined.
which they are confronted with cancer, these AYA have age-specific Results: In addition to existing introduction days on AYA-specific
needs that too easily remain undetected and unanswered in adult themes, a more extensive training module was developed. This first
wards. To meet this, we designed a tool within our digital patient AYA basic training in Flanders took place in 2020 and consisted of
platform mynexuzhealth, called MY NEEDZ. a four-day training. Following themes were included: communication
Methods: By offering information and psycho-education, we want to with AYAs and their families, medical aspects, palliative care and AYAs,
meet the needs of AYA and empower them to ask their questions. identity development, impact on self-image, revalidation, sexuality, fer-
The MY NEEDZ tool invites them to think about their own needs and tility, fear of relapse, . . . Given the holistic and interdisciplinary nature
questions, around age-specific themes. Based on recognizable state- of AYA care, we offer this training interprofessionally. In total, across
ments, we explore possible problems and questions typical for AYA. We the introduction days and the first AYA basic training, more than 80
provide information for a better understanding of what they are expe- care providers (doctors, nurses, social workers, psychologists, moral
riencing and give suggestions on how to deal with it. In addition, we also consultants, nurse specialists, . . . ) were trained in specific care for AYAs.
advise which healthcare providers they can contact. If they can’t find Conclusions: In collaboration with its partners, CHi wants to offer
an answer to their question, they are given the opportunity to ask their healthcare professionals more specialized knowledge and skills related
question inside the tool. A healthcare provider will then contact them to AYA care. Introduction days and a basic training were developed
to find out how the AYA can be helped. for this. In the future, further courses, in-depth courses and e-learning
Results: The tool was co-developed and tested by AYA from our AYA will be developed to further stimulate the exchange of knowledge and
Advisory Group. In a next phase (begin 2022), 30 AYA’s will be test- experience about this target group in Flanders (Belgium).
ing the tool in order to make the necessary adjustments. We expect
to offer the tool to every AYA in our hospital at the end of 2022. We
hope to capture questions and needs from AYA that have not yet been P088 / #890 TELEO: A VIRTUAL TOOL FOR NURSING
detecterd or answered. EDUCATION IN LATIN AMERICA
Conclusions: Thanks to the tool My Needz, available in the app mynex-
uzhealth, every AYA can consult age-specific information and ask Mariana Durañona
questions during their oncological trajectory and afterwards. It pro- Hospital Universitario Austral, Pilar, Derqui, Argentina
vides a platform where AYA discover answers to their needs, at any
time. Background and Aims: There are scant virtual education tools for
Latin American (LATAM) nurses; fewer on pediatric oncology or in
Spanish. In 2021, TELEO [Tele-educación en Oncología Pediátrica]
E-Poster Topic: AS04 Nursing / AS04.a Education was created for educating pediatric oncology professionals through
a My Child Matters grant Two free nursing education programs (one
NURSING POSTER SESSION orientation and one continuing education in line with the SIOP Base-
line Standards for Nurses in low- and middle-income countries) were
P087 / #715 TRAINING IN ’CUSTOMIZED CARE FOR AYAS’ developed in Spanish.
FOR HEALTHCARE PROVIDERS IN FLANDERS (BELGIUM) Methods: The first TELEO program (2021), was a weekly, 2-month
online orientation to pediatric oncology nursing. E-Poster Topics
Kleo Dubois taught by expert LATAM and international nurses included oncogene-
Cédric Hèle instituut, Oncology, Mechelen, Belgium sis, chemotherapy care, vascular access, pain management, and others.
Classes limited to 50 nurses for questions and discussion. Later, this
Background and Aims: In 2017, almost 1000 patients between the program was on-demand with free access to recorded classes with
ages of 16 and 35 were diagnosed with cancer in more than 50 different expert nurse support through the TELEO networking platform. The
hospitals in Flanders (Belgium). Appropriate care for AYAs means mul- second TELEO program (2021) was a monthly online 1-hour meeting
tidisciplinary, integrated care for patients who have unique and diverse with no registration requirement. Designed as continuing education for
needs due to their age and stage of life. With this training, the Cédric more experienced pediatric oncology nurses, E-Poster Topics included
Hèle Institute (CHi) wants to raise awareness among caregivers from nursing research, medication errors and second victim issues, palliative
different institutions. sedation, and CAR T-cell therapy.
Methods: In 2019, CHi set up a steering group with the aim Results: Overall, 293 nurses from 14 countries participated in the
of developing a (multi-day) training course on AYA-specific care. two orientation courses: 225 South Americans and 68 Central Amer-
This steering group is composed of care providers from vari- icans. 48 completed the synchronous and 83 the on-demand course.
ous centers (doctors, social workers, psychologists, policy mak- Colombia had the most attendees (70 nurses), followed by Uruguay and
ers, . . . ) and AYAs themselves. In terms of content: good prac- Argentina.The pass rates were 70% (first edition) and 63% (second edi-
tices from abroad (the Netherlands, UK, Australia, . . . ), results tion); total dropout rate was 54%. Ninety-one percent considered the
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S538 of S635 ABSTRACTS

course useful, and 98.8% would recommend it. Future E-Poster Topics needed, and the next step is to test the video call with five families and
indicated educational needs, e.g., bone marrow transplant and pallia- evaluate with a short questionnaire.
tive care. The second program’s monthly meetings were dynamic and
interactive; 237 participated.
Conclusions: TELEO virtual education by regional and international E-Poster Topic: AS04 Nursing / AS04.b Research
expert nurses provides useful training opportunities and a discussion
platform for common LATAM nursing problems . Alternative formats NURSING POSTER SESSION
may provide an enhanced experience and address formal courses’ high
drop-out rates. P090 / #60 THE LIVED EXPERIENCES OF MOTHERS OF
CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKAEMIA IN
MALTA
E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
Project Elaine Formosa1 , Josef Trapani2
1 Sir Anthony Mamo Oncology Centre, Department Of Health, Msida, Malta;

NURSING POSTER SESSION 2 University of Malta, Department Of Nursing, msida, Malta

P089 / #674 DEVELOPMENT OF VIDEO CALL IN A Background and Aims: Acute Lymphoblastic Leukaemia (ALL) is one
FOLLOW-UP PROGRAM FOR CHILDREN NEWLY DIAGNOSED of the most common cancerous illness amongst the paediatric oncol-
WITH CANCER AND THEIR PARENTS ogy population. Most patients receive full treatment for ALL within
the local oncology centre as patients with other diagonosis require
Line Dyrgaard, Gitte Petersen, Eva Hansson international hospitalisation. The inital induction phase of treatment
The state university in Copenhagen, Peadiatic Oncology And Haematology, for ALL is characterised by a lenghty period of hospitalisation which
copenhagen, Denmark ranges between four and six weeks until medically stable to continue
treatment at home. This phase is surrounded by a captivating range
Background and Aims: In 2017, we developed a systematic follow- of emotions felt by parents upon the return home. The transition of
up program at home after the first discharge to ensure that families care shifts from a hospital based setting to a home-care approach.
receive the information, guidance and training they needed to han- Behavioural concerns, fear of the unknown and physical changes of the
dle care and treatment tasks. Families receive the program if they live child along the struggle to deal with oral chemotherapeutic medica-
within 50 km from the hospital. The program is provided by a nurse tions and the care management of central lines are some of the main
from the childhood cancer department. Our survey of the program concerns that trouble parents as they try to manage the challenges
showed that the great majority of the families found the program very brought about by the new ’normality’. The aim of this study sought to
usable and comforting. To give all families access, we want to extend explore the lived experiences of mothers who strive to adapt to the sit-
and provide the follow up-program as a video call to families that can- uation of returning home for the first time with a child diagnosed with
not receive a home visit. As the first step, the aim of this pilot study was ALL.
to develop the video call technology and determine whether it may be Methods: For the purpose of this research, a qualitative method
usable if it was an option. to explore the lived phenomenon encountererd by mothers
Methods: This is an explorative ongoing pilot study at the childhood was used. The reseacher followed the approach of an inter-
cancer department at the university hospital in Copenhagen. We initi- pretative phenomenological analysis (IPA). Data was collected
ated a collaboration with the new Mary Elizabeths Hospital to develop from in-depth semi-structured interviews, conducted with five
the video call technology and conducted interviews with to fami- mothers.
lies. We asked when the videocall should be after the discharge, how Results: Six super-ordinate themes emerged which are mixed
long it could take and which E-Poster Topics they wanted follow-up emotional cycle, relocation, daily struggles of living with
on. ALL, the way forward, shifting perspectives and supportive
Results: The video call is developed in the electronic medical journal encounters.
system ‘EPIC’ but has not yet been tested. The interviews showed that Conclusions: The need of supportive and educational programmes
the families would accept a video call if the department offered it. They was reflected for health professionals and families along the need for
did not think it could replace the follow-up visit at home, but it would employmnet assistance for parents and a structued home health-care
better than no follow-up. based support. More reserach is essential to explore the father’s role
Conclusions: The preliminary results indicate that a video call can be and the difficulties encountered, so as to enhance a favrouable quality
used in the follow-up program instead of a visit at home. More data are of life for all the family.
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ABSTRACTS S539 of S635

E-Poster Topic: AS04 Nursing / AS04.a Education that are specific to the context of each clinic and unique patient pop-
ulation. The next steps will assess patient and family knowledge gain
NURSING POSTER SESSION and acceptability of the information.

P091 / #1138 UTILIZING QUALITATIVE STUDY RESULTS TO


INFORM NOVEL EDUCATIONAL INTERVENTIONS AT A E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
PEDIATRIC ONCOLOGY CENTER IN EL SALVADOR Project

Alvaro Martin Morales Lugo1 , Carmen Salaverria2,3 , Erin Mccann4 , NURSING POSTER SESSION
Soad Fuentes-Alabi De Aparicio5 , Meghan Shea6 , Irini Albanti7
1 Fundación Ayúdame a Vivir, Psychooncology, San Salvador, El Salvador; P092 / #225 MENTORSHIP: NURSES SUPPORTING NURSES:
2 Hospital Nacional de Ninos Benjamin Bloom, Pediatric Oncology, San Sal- A PILOT PROJECT IN THE HAEMATOLOGY/ONCOLOGY/ BLOOD
vador, El Salvador; 3 Ayudame a Vivir Foundation, Psychooncology, San AND MARROW/TRANSPLANT/ CELLULAR THERAPY PROGRAM
Salvador, El Salvador; 4 Cincinnati Children’s Hospital Medical Center, Pedi-
atrics, Cincinnati, United States of America; 5 Centro Médico Ayúdame a Sheila Gandhi
Vivir/Hospital Nacional de Niños Benjamin Bloom, Pediatric Oncology, San Hospital for Sick Children, Heamatology/oncology, MG X, Canada
Salvador, El Salvador; 6 Persistent Productions, Persistent Productions, Rock-
port, United States of America; 7 Harvard Humanitarian Initiative, Oncology, Background and Aims: Pediatric oncology comes with many diffi-
Cambridge, United States of America culties including challenging patient and family interactions, complex
therapies, and palliative management of pediatric patients. Prior liter-
Background and Aims: El Salvador is a low-middle income country ature has shown that novice pediatric oncology nurses are ill-equipped
(LMIC) with a 52% overall survival for childhood cancer, compared to to assess and manage these challenging situations. Coping strate-
80% in high-income countries. Abandonment and lack of treatment gies have not been developed therefore nurses feel overwhelmed and
adherence are major barriers to improving the survival rate in children unsupported. These impacts on nursing morale play a significant role
and adolescents with cancer in LMICs. Previous research at Centro in nursing attrition in the early years of a nurses’ career. The aim of
Médico Ayúdame a Vivir Fundación (CMAVF) in El Salvador identified this pilot project for the haematology/oncology/SCT division at our
common questions and educational needs shared by caregivers and hospital was to provide support and nurture novice nurses as they nav-
health care providers in the early stages of a new cancer diagnosis. igate their first year as independent clinicians with an eventual goal to
Results were subsequently utilized to inform “Day One” videos to com- measure retention at two and five years.
plement the initial discussion following a new diagnosis. Recognizing Methods: Via email, we asked nurses if they would be interested in
the need for further tailored, educational interventions, the healthcare mentoring new staff. Nurses and advanced practice clinicians were
team at CMAVF has created a project entitled “Emely’s Suitcase” to required to have a minimum of five years of Oncology BMT/SCT expe-
provide lighthearted anticipatory guidance at cancer treatment initia- rience. Mentorship education was provided. We developed a process
tion through age-appropriate education aiming to increase treatment where a newly hired nurse can choose a mentor from a profile list.
adherence. Mentorship was a requirement for new hires. The mentor-mentee
Methods: The CMAVF healthcare team conducted qualitative, semi- pair were required to meet four times each year. The mentee pre-
structured interviews with patients, families, and healthcare staff in pared objectives and areas for discussion or reflection. Two surveys
February 2019 and March 2022 to assess the most important infor- administered to the mentees six months apart evaluated the rela-
mation that patients and families need to know related to the initial tionship, the reflective practice element and the effectiveness of the
diagnosis, self-care and hospital procedures and expectations. After relationship.
analyzing the interviews’ common themes, educational resources were Results: A total of thirty nurses were interested in mentoring.
developed for implementation at CMAVF. Seventeen novice nurses participated (100 %). Fifty-three percent
Results: Three educational tools were created for different age groups (9/17) of the mentees completed the survey one year after start-
including a coloring book and doll, simple infographics, informational ing the program. Eighty-nine percent (8/9) of the mentees found the
booklets, and a Pop-Up Book. The CMAVF psychology team piloted relationship supportive and suggested mentorship beyond the first
these materials and solicited feedback from six patients, six fami- year.
lies, two nurse educators, and one pediatrician. A training program Conclusions: Mentorship is an effective method of supporting
was created for volunteers to implement the curriculum to ensure novice nurses in a difficult field as they develop into inde-
sustainability. pendent clinicians. The mentorship program will continue at
Conclusions: Educational needs assessments that are patient- and our center, and nursing attrition at two and five years will be
family-centered can be utilized to develop tailored educational tools evaluated.
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S540 of S635 ABSTRACTS

E-Poster Topic: AS04 Nursing / AS04.a Education Background and Aims: Pediatric patients diagnosed with oncology,
hematology and immunology conditions often experience pain, nausea,
NURSING POSTER SESSION anxiety and other side effects. In an effort to augment traditional treat-
ments, bedside nurses on the hematology/oncology/ hematopoietic
P093 / #1058 SYMPTOMATIC PRESENTATION OF CANCER stem cell transplant units at Boston Children’s Hospital adopted a holis-
PATIENTS WITH ACUTE METHOTREXATE TOXICITY TREATED tic approach to their practice by providing Integrative Therapies (IT). IT
WITH CARBOXYPEPTIDASE are nonpharmacological interventions including Reiki, relaxation mas-
sage, and yoga. As a result of the positive reports and increasing patient
Winston Huh, Sarah Gilani, Emma Cantor, Teresa Rushing, Fariba requests, the IT Program was developed in 2019 and serves to enhance
Navid a patient’s physical, mental, emotional and spiritual well-being. The IT
Children’s Hospital Los Angeles, Pediatrics, Los Angeles, United States of team consists of five specially trained nurses available 30 hours per
America week providing 30-minute IT sessions.
Methods: The IT team created a data collection tool to be completed
Background and Aims: Methotrexate is a common chemother- before and after patient IT sessions including: a radial pulse and breaths
apy agent used in treatment protocols for pediatric cancers. Acute for thirty seconds. Patients completed the Wong-Baker FACES® scale
methotrexate toxicity due to abnormally high methotrexate levels can to measure pain, Baxter Animated Retching Faces (BARF) scale to
lead to multiorgan damage and death, and carboxypeptidase is an measure nausea, Children’s Fear scale to measure fear/anxiety and
approved treatment. Presenting symptoms of acute methotrexate tox- a visual analog scale to measure fatigue. Demographics are also col-
icity are not well described in the literature. The aim of this study is lected. Qualitative data is obtained through open ended questions
to describe early clinical signs and symptoms in patients with acute about the patient’s IT experience. All data was recorded into an Excel
methotrexate toxicity to educate nursing staff, who are often the first spreadsheet.
care providers to note symptoms. Results: There were 946 IT sessions from September 23, 2019 –
Methods: A single institution retrospective medical record review of March 5, 2021. Data was obtained from 751 sessions. Results included
patients who received carboxypeptidase due to methotrexate toxicity patients who experienced one of the symptom variables to start and
from 2006 to present was completed. Data collected on 13 patients completed a pre and post rating of the variable. Of those patients: 63%
included age, gender, dose of methotrexate, methotrexate and creati- reported a decrease in pain, 61% reported a decrease in nausea, 74%
nine levels at 24, 48, 72, 96, and 120 hours, timing and value of peak reported a decrease in anxiety and 54% reported a decrease in fatigue.
methotrexate and creatinine level, presenting symptoms, and timing. Conclusions: Data driven information was acquired to support the
Descriptive statistics were utilized. IT program and its benefits to patient care. The program provides
Results: The 13 patients consisted of 11 osteosarcoma, 1 pleomorphic valuable nonpharmacological interventions, especially for symptom
sarcoma, and 1 lymphoma patient. Five patients had abdominal pain, 3 management.
had back pain, 3 had extremity pain, and 9 had severe nausea/vomiting.
Mean 24-hour methotrexate level was 323 uMol/L (range 17.5 – 826.8
uMol/L), mean peak creatinine level 2.1 mg/dL at 44 hours. Mean P095 / #1211 ADDRESSING GLOBAL HEALTH ISSUES IN
time to symptom onset from start of methotrexate was 16.5 hours OCEANIA- "CLOSING THE GAP IN OUR NEIGHBOURHOOD"
for abdominal pain, 16 hours for back pain, 15 hours for extremity
pain. Nausea and vomiting was seen frequently during the infusion Jayne Harrison
of methotrexate. Mean time from first symptom to carboxypeptidase Royal Children’s Hospital, Children’s Cancer Centre, Melbourne, Australia
dose was 34 hours.
Conclusions: As bedside nurses are most often the first line, early Background and Aims: Global disparities for childhood cancer sur-
symptom recognition is crucial for acute methotrexate toxicity. The vival rates between high-income (HIC) and low-middle income (LMIC)
effectiveness of carboxypeptidase decreases when given more than 60 countries are well documented. The World Health Organisation, St
hours after exposure to methotrexate. Next steps include development Jude Global, and SIOP share the common goal to improve the survival
of an education module for nurses on methotrexate toxicity. of all children with cancer – irrespective of where they live. Encom-
passing land and sea, Oceania is characterized by its vast geography,
P094 / #1106 INTEGRATIVE THERAPIES: A with a diverse mix of ethnicities and cultures. Comprising 18 coun-
NON-PHARMACOLOGICAL NURSING INTERVENTION FOR tries, only Australia and New Zealand [NZ] are classified as HICs; the
SYMPTOM MANAGEMENT remaining 16 are classified as small-island, low-income countries (LIC).
Within the Oceania region there is a wide variation in healthcare pro-
Kristen Guilmette vision which, together with geographic barriers of distance and limited
Boston Children’s Hospital, Hematology/oncology/hematopoietic Stem Cell infrastructure, create challenges to provide support.
Transplant, Boston, United States of America Methods: Method The paediatric oncology workforce in LICs within
Oceania are supported and strengthened by the establishment of
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ABSTRACTS S541 of S635

a multidisciplinary group to provide teaching, training and protocol spond with the children’s feelings of nausea severity. A Wilcoxon signed
development and implementation. rank test was executed to statistically compare outcomes of both
Results: Since 2014, representatives from Australia and NZ have tools.
worked to develop and implement strategies that provide in-country Results: Both tools complied to the feasibility criterium, and the BARF
support and capacity building. These include: international observor- was more feasible than the PeNAT. Scores were lower for children aged
ships and fellowships (nursing and medical), twinning programs and 4 to 8 years old in comparison with older children, but not significantly
in-country visits. To date, key champions in specific regions (Papua New different. The faces of the BARF corresponded significantly more with
Guinea, Timor Leste, Laos and Fiji) have been identified to promote and children’s feelings of nausea than the PeNAT, however, the neutral face
implement oncology nursing education programs. In 2022, the SIOP did not.
Oceania Advisory Board was formalised to further promote and con- Conclusions: Both tools are feasible, however difference between
solidate support. The Board consists of multiple expert members from age groups should be taken into account for communication and
Australia and New Zealand representing medical, nursing, allied health, understanding. Children prefer the BARF’s faces, however, they
psychosocial and family support groups. prefer the PeNAT’s neutral face. Because most children were
Conclusions: Successful cancer care in these regions requires the not nauseous at the time of this study, different outcomes could
Six Baseline Standards for Paediatric Oncology Nursing to be met. have been achieved and should be taken into account for future
Partnering with key stakeholders, SIOP Oceania aims to implement research.
nurse-led training programs that support these international standards
of care. Moreover, strategies will continue to be developed to sup-
port sustainable in-country care of children with cancer and improve E-Poster Topic: AS04 Nursing / AS04.a Education
survival.
NURSING POSTER SESSION

E-Poster Topic: AS04 Nursing / AS04.b Research P097 / #853 EXPECTATION ON THE ROLE OF ADVANCED
NURSE PRACTITIONERS IN PEDIATRIC HEMATOLOGY
NURSING POSTER SESSION ONCOLOGY: A DESCRIPTIVE QUALITATIVE STUDY

P096 / #108 ASSESSING FEASIBILITY AND FACE VALIDITY Mengxue He, Fen Zhou, Nanping Shen
OF TWO NAUSEA INSTRUMENTS: A CLINIMETRIC STUDY IN Shanghai Children’s Medical Center, Pediatric Hematology And Oncology,
CHILDREN WITH CANCER Shanghai, China

Els Haverkate1 , Aeltsje Brinksma1 , Janneke De Man - Van Ginkel2 , Background and Aims: China ranks among the top five countries
Wim Tissing3 in the world with the highest burden of pediatric hematology and
1 Princess Maxima Center for pediatric oncology, Supportive Care, Utrecht, oncology. As a vital position of specialized nursing, the advanced
Netherlands; 2 Leiden University Medical Centre, Gerontology And Geri- practice nurse in pediatric hematology oncology (PHO-APN,
atrics, Leiden, Netherlands; 3 Princess Maxima Center for Pediatric Oncol- as an abbreviation) have been established in developed coun-
ogy, Supportive Care, Utrecht, Netherlands tries for decades, but there is no such position in China. This
study is to explore expectations of stakeholders on the role of
Background and Aims: Chemotherapy causes distressing side effects PHO-APN in China, under the guidance of Hamric’s Advanced
such as nausea. However, control of nausea in children remains Nursing Practice Theory, in order to develop this position in
challenging. Two clinimetric instruments are available for optimizing future.
recognition and management of nausea in children: the Pediatric Nau- Methods: From November 2020 to April 2021, 18 stakeholders, includ-
sea Assessment tool (PeNAT) and the Baxter Retching Faces (BARF) ing advanced nursing practice experts, doctors, nurses, children’s fami-
scale. Nevertheless, more knowledge regarding practical use and chil- lies in this specialty, were purposively selected for one-to-one in-depth
dren’s preferences is needed. This study aimed to assess feasibility and interviews. The semi-structured interview started with a wide range
face validity of both the BARF and the PeNAT according to children of questions, and each lasts 60-90 minutes. Then each interview was
aged 4 to 18 years old with cancer treated in a paediatric oncology transcribed word by word and sentence by sentence. The data were
centre. analyzed by qualitative content analysis, through transcription, cod-
Methods: A quantitative clinimetric study (n=34) was conducted. Fea- ing, generic analysis, description and organization, in QSR NVIVO12 0
sibility and face validity were measured, and scores of the BARF and software.
the PeNAT were compared to each other. Feasibility included three Results: Three themes were extracted as follows. A) Necessity of devel-
items: understanding, ease of use, and communication. Face validity oping the PHO-APN in China: The PHO-APN in China is necessary,
was studied in terms of: to what extent did faces of both tools corre- in terms of policy environment, clinical needs and nurses’ personal
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S542 of S635 ABSTRACTS

development. B) Position responsibilities of PHO-APN: the responsi- E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
bility of PHO-APN should be established based on the clinical needs, Project
including three roles, filling gaps, promoting care quality, and training
and education. C) Selection of PHO-APN candidate: the possible can- NURSING POSTER SESSION
didate should be based on capability and quality, including professional
competency, physical condition, team communication ability, system- P099 / #745 STRATEGIES TO PREVENT FALLS IN
based analysis and decision-making ability, self-learning and research HEMATOPOIETIC STEM-CELL TRANSPLANT (HSCT) PATIENTS
ability.
Conclusions: The position responsibilities of PHO-APN in China should Phuc Ho1,2 , Marissa Johnson2 , Amy Wierzchowski2 , Julie Waitt1 , Lisa
focus on clinical needs, including filling gaps, promoting care qual- Morrissey1 , Caroline Costello1
ity, and training and education, so as to effectively improve the 1 Boston Children’s Hospital, Hematopoietic Stem Cell Transplant Unit -
system efficiency and the prognosis of children. Nurses and doc- 6west, Boston, United States of America; 2 Boston Children’s Hospital,
tors had basically the similar expectations, but there were also Hematopoietic Stem Cell Transplant Unit, Boston, United States of America
differences.
Background and Aims: Pediatric falls are complex, as many factors
influence both the risk of falls and the likelihood of sustaining injury.
E-Poster Topic: AS04 Nursing / AS04.b Research Pediatric falls occur less often and have different associated risks than
in adult hospitalized patients. Fall prevention requires daily risk screen-
NURSING POSTER SESSION ing, education and partnership with families, and ongoing evaluation of
fall prevention strategies. Hematopoietic Stem-Cell Transplant (HSCT)
P098 / #962 RISK FACTORS OF FEBRILE NEUTROPENIA IN patients are at higher fall risk due to factors such as complications of
CHILDREN WITH ACUTE LEUKEMIA disease and treatment, prolonged hospitalization and deconditioning.
On our HSCT unit, staff nurse “fall champions” collaborate with nurs-
Mengxue He, Fengyun Xie ing leadership to collect and analyze falls data monthly. In 2019, it was
Shanghai Children’s Medical Center, Pediatric Hematology And Oncology, noted that the unit’s fall rate was trending upwards, peaking in 2020 to
Shanghai, China 9.61 per 1,000 patient days. A goal was established to decrease patient
falls on the HSCT unit.
Background and Aims: Febrile neutropenia is one of the most com- Methods: In 2019, a Fall Prevention group was formed, comprised
mon and severe complications associated with chemotherapy. The of staff nurse champions, nursing leadership and physical therapists.
investigation is to explore the occurrence and risk factors of febrile The following strategies were implemented over a two year period:
neutropenia in children with acute leukemia in a tertiary pediatric •Development of Higher Fall Risk Category •Creation of targeted
hospital in Shanghai, China. interventions for patients identified as Higher Fall Risk •Initiation of
Methods: A total of 801 pediatric patients with acute leukemia treated purposeful rounding every four hours with vital signs, with shared
with chemotherapy in the department of pediatric hemantology in responsibility between nurses and clinical assistants for rounding and
a tertiary pediatric hospital from January to December, 2020 were documentation •Apparent cause analysis (ACA) reports performed
selected. The demographic, disease-related, treatment-related factors with all falls •Physical Therapy/ HSCT pilot to reduce decondition-
were collected. Logistic regression analysis was used to screen out the ing •Nursing Leadership follow up post falls, engaging parents in
risk factors of FN, and ROC was used to assess the resulting equation. prevention of recurring falls
Results: There were 268 patients occurring the FN, with an incidence Results: The rate of patient falls with injury decreased from 9.61 to
rate of 33.46%. Logistic regression analysis showed that age, dam- 0.95 per 1,000 patient days on the HSCT Unit between January 2020
aged oral mucosa in 5th day after chemotherapy, recurrence, first-time and January 2021.
chemotherapy, cytarabine-containing regimen, neutropenia in 5th days Conclusions: Pediatric patients undergoing Hematopoietic Stem Cell
after chemotherapy were the risk factors of FN. The AUC of this model Transplant are at increased risk of injury due to falls. Targeted interven-
is 0.884. When the cutpoint was 0.2957, the sensitivity and specificity tions can reduce the risk of falls with injury in this vulnerable patient
of the model were 0.881 and 0.822, respectively. population. Collaboration between multiple disciplines to achieve
Conclusions: The incidence of FN in pediatric patients with acute shared goals increases likelihood of success.
leukemia is relatively high, and the established model showed certain
clinical value with moderate predictive capability. Prevention of FN
should be emphasized from the first-time chemotherapy, especially P100 / #560 THE IMPORTANCE OF AN ONCO-FERTILITY
in pediatric patients with recurrence, young age, early neutropenia. PROGRAM FOR PEDIATRIC ONCOLOGY PATIENTS
At the same time, nurses should pay attention to assessment of oral
mucositis, blood routine test, and vital signs, actively prevent and treat Irene Ijgosse1 , Aart Klijn2 , Leendert Looijenga3 , Ralph Oude Ophuis4 ,
oral mucositis and bone marrow suppression. Marry Van Den Heuvel-Eibrink5 , Margreet Veening1 , Annelies Bos6 ,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S543 of S635

Simone Broer6 , Jeanette Van Leeuwen6 , Alida Van Der Steeg7 , Sruthi Aarhus Universitets Hospital, Børn og Unge 1, The Department Of Children
Sriram3 , Maria Elisabeth Madeleine Van Der Perk1,8 , Marianne Van De And Adolescent With Cancer, Aarhus N, Denmark
Wetering9
1 Prinses Maxima Centrum, Pediatric Oncology, Utrecht, Netherlands; Background and Aims: It is a known challenge that adolescents
2 UMCUtrecht, Wilhelmina Childrens Hospital, Urology, Utrecht, Nether- and young adults(AYAs) need help and support during and after can-
lands; 3 Prinses Maxima Centrum, Research, Utrecht, Netherlands; cer treatment. Unfortunately, they sometimes experience that their
4 UMCUtrecht, Clinical Embryology, Utrecht, Netherlands; 5 Princess Máx- desired E-Poster Topics are not addressed. The Australian AYA Oncol-
ima Center for Pediatric Oncology, Division Of Pediatric Oncology, Utrecht, ogy Screening Tool is a validated tool that can be used in consultations
Netherlands; 6 UMC Utrecht, Reproductive Medicine And Gynaecology, with AYAs with cancer and support them identifying, discussing and pri-
Utrecht, Netherlands; 7 Princess Maxima Center, Surgery, Utrecht, Nether- oritizing psychosocial issues that need to be addressed in their care.
lands; 8 Prinses Máxima Centrum voor kinderoncologie, Pediatric Oncology, The tool has been translated into Danish following the World Health
Utrecht, Netherlands; 9 Princess Maxima Center for Pediatric Oncology, Organization’s process of translating and adapting instruments. The
Pediatric Oncology, Utrecht, Netherlands tool has been tested in the Oncology and Haematology department
at AUH on AYAs (+18 years) with good experience. The purpose of
Background and Aims: Introduction; In the Netherlands pediatric this study is to investigate whether the tool can be used with AYAs
oncology care is centralized in one hospital since 2018. 600 new (14-22 years) in the follow-up consultations. Furthermore, we will
patients a year are seen. Of these patients, 25-35 % classify as high investigate the use of split visit, where parents only participate for
risk (HR) for infertility. An onco-fertility program was started navigated a limited period of the consultation, provide increased opportunity
by a nurse-practitioner. The program runs with intense collabora- for AYAs to talk about their concerns and challenges after cancer
tion between the different specialties. All new patients are identified treatment.
according to the international guidelines on fertility care. The fertility- Methods: The Department of Children and Adolescents with Cancer
risk is based on the cyclofosfamide equivalent dose, radiotherapy dose AUH began using the tool with AYAs (14-22 years) in the follow-up
and surgery to the gonadal area. Since 2018 awareness was created by consultations. The consultations include a physician and a nurse, who
teaching sessions among colleagues, nursing staff, and parent associa- incorporate the tool into their consultations with AYAs. After each con-
tion organizations. The onco-fertility program was started as standard sultation, the physician and nurse fill out a questionnaire about their
of care. experience and feasibility of the tool, while AYAs and parents evaluate
Methods: All patients are informed on fertility risk by their oncolo- their experience of the consultation.
gist. All HR children are additionally councelled by the onco-fertility Results: Preliminary results indicate that the tool is promising for AYAs
nurse-practitioner and referred for further counseling to gynecol- to help them express their concerns. Healthcare professionals describe
ogy for ovarian tissue cryopreservation(OTC) or urology for sperm that AYAs open up E-Poster Topics they have not mentioned before,
cryopreservation or testicular biopsy (in research setting). Monthly and the tool helps healthcare professionals get to the point more
the onco-fertility working-group members discuss difficult cases and quickly.
research in the field. Conclusions: Work continues on the applicability of clinical practice,
Results: In 2019, 29 % cases and in 2020 31% of cases and the first results point to a promising psychosocial screening tool
were HR for infertility. In both years 45 % of these cases that Danish healthcare professionals can use with their AYA patients.
had fertility preservation performed. In 2021, 30 % cases
HR were identified, of these HR patients in 44 % preserva-
tion was performed. Reasons for not preserving fertility were E-Poster Topic: AS04 Nursing / AS04.b Research
diverse.
Conclusions: Awareness of the fertility risk in pediatric oncology NURSING POSTER SESSION
patients is necessary. All patients need to be informed and all HR
patients need to be counselled on the risk of infertility and offered P102 / #1562 SETTING A NEW RESEARCH AGENDA IN
fertility preservation before start of their treatment. An active onco- PEDIATRIC CANCER: A JAMES LIND ALLIANCE
fertility program helps to offer the best option for future fertility for PRIORITY-SETTING PARTNERSHIP WITH PATIENTS,
these patients. SURVIVORS, FAMILY MEMBERS, AND CLINICIANS

Lindsay Jibb1,2 , Sarah Calderwood3 , Helena Kirk3 , Rachel Hamilton4 ,


P101 / #558 PILOTING THE DANISH VERSION OF THE Michael Taccone5,6,7,8 , Caroline Wai3 , Antonia Palmer9 , Perri
“AUSTRALIAN AYA ONCOLOGY SCREENING TOOL” AT THE Tutelman10 , Adrienne Co-Dyre3 , Daniel Morgenstern11,12 , Kathy
DEPARTMENT OF CHILDREN AND ADOLESCENTS WITH Brodeur-Robb13 , Geoffrey Fang14,15 , Lily Ren16 , Paul Nathan17,18 ,
CANCER AT AARHUS UNIVERSITY HOSPITAL(AUH) Kathryn Birnie19,20 , Julie Chartrand21,22 , Tatenda Masama17 , Elham
Hashemi1 , Kyobin Hwang1 , Surabhi Sivaratnam1 , Alicia Kilfoy4 , James
Lise Jensen, Louise Hansen Whitlock17,23
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S544 of S635 ABSTRACTS

1 Hospital for Sick Children, Child Health Evaluative Sciences, Toronto, mental, and psychosocial impacts and needed supports; preventing and
Canada; 2 University of Toronto, Lawrence S. Bloomberg Faculty Of Nurs- treating relapsed and refractory cancers; and survivorship issues.
ing, Toronto, Canada; 3 Helena’s Hope, N/a, Toronto, Canada; 4 Hospital for Conclusions: We have identified shared pediatric cancer research
Sick Children, Haematology/oncology, Toronto, Canada; 5 Hospital for Sick priorities in Canada using a rigorous, co-development approach involv-
Children, Neurosurgery, Toronto, Canada; 6 University of Toronto, Temerty ing stakeholders typically not involved in setting research agendas.
Faculty Of Medicine, Toronto, Canada; 7 Childhood Cancer Survivor Canada, The priorities broadly suggest a call to research action on mini-
N/a, Toronto, Canada; 8 University of Ottawa, Department Of Surgery, mizing barriers to accessing care, improving capacity to implement
Ottawa, Canada; 9 Kindred Foundation, N/a, Oakville, Canada; 10 Dalhousie evidence into practice, and the delivery of holistic and psychosocial
University, Department Of Psychology, Halifax, Canada; 11 The Hospital For care.
Sick Children, Temerty Faculty of Medicine, University of Toronto, Pedita-
tric Hematology Oncology, Toronto, Canada; 12 Hospital for Sick Children
& University of Toronto, Department Of Paediatrics, Toronto, Canada; P103 / #288 TENTATIVE MODEL OF IMPAIRED
13 C17 Council Children’s Cancer and Blood Disorders, N/a, Edmonton, NUTRITIONAL STATUS AMONG THAI CHILDREN WITH
Canada; 14 Hospital for Sick Children, Family Advisory Network, Toronto, CANCER: THE INTERVENTION MAPPING APPROACH
Canada; 15 University of Toronto, Faculty Of Engineering, Toronto, Canada;
16 Stanford University, Lane Medical Library & Knowledge Management Donruedee Kamkhoad1,2 , Sheila Santacroce2
Center, Stanford, United States of America; 17 The Hospital for Sick Children, 1 Ramathibodi School of Nursing, Faculty Of Medicine Ramathibodi Hos-
Hematology/oncology, Toronto, Canada; 18 University of Toronto, Institute pital, Mahidol University, Bangkok, Thailand; 2 School of Nursing, The
Of Health Policy, Management And Evaluation, Toronto, Canada; 19 Alberta University Of North Carolina At Chapel Hill, Chapel Hill, United States of
Children’s Hospital, Department Of Anesthesiology, Perioperative And Pain America
Medicine, Calgary, Canada; 20 University of Calgary, Department Of Anes-
thesiology, Perioperative And Pain Medicine, Calgary, Canada; 21 University Background and Aims: Most children diagnosed with cancer receive
of Ottawa, Faculty Of Health Sciences, Ottawa, Canada; 22 Children’s Hos- chemotherapy that causes gastrointestinal (GI) symptoms, which
pital of Eastern Ontario, Children’s Hospital Of Eastern Ontario Research impair their eating behaviors and nutritional status. Impaired nutri-
Institute, Ottawa, Canada; 23 University of Toronto, Department Of Paedi- tional status lowers children’s quality of life (QOL) and places
atrics, Toronto, Canada them at risk for febrile neutropenia in the first year following
diagnosis and for worse survival. Most studies in this research
Background and Aims: Rigorously conducted and well-implemented area were done in Western countries. However, Thai children
pediatric cancer research is needed to improve outcomes for chil- undergo chemotherapy in cultural and other environmental con-
dren and their families during and after therapy. We aimed to conduct texts including the typical Thai diet. Intervention Mapping (IM)
a Canada-wide Priority Setting Partnership with pediatric cancer uses a 6-step iterative process and a socio-ecological perspective
patients, survivors, family members, and clinicians, to identify and pri- to develop interventions to improve health behaviors and out-
oritize research questions to inform the decisions of research funders comes. This study used IM, step 1 to develop a tentative model of
and guide Canadian researchers. impaired nutritional status among Thai children with cancer during
Methods: A steering group oversaw our phased James Lind Alliance chemotherapy.
approach and Canadian childhood cancer partner organizations were Methods: We used IM step 1 and IM core processes (reviewing the
recruited. First, a national online survey collected research uncer- literature, collecting data from key stakeholders) to develop a tenta-
tainties that were collated into indicative questions. Questions were tive multi-level logic model of impaired nutritional status in children
systematically checked against published evidence to identify previ- with cancer. We also considered the Thai culture and standard clinical
ously answered questions. A second national survey was administered practices.
to prioritize questions. A shortlist of these questions was then taken Results: The tentative model includes QOL as the health out-
to a consensus-building workshop, where a decision on the top ten come; impaired nutritional status as health problem; children’s eat-
priorities was made. ing and self-management as the behaviors contributing the prob-
Results: Three hundred and fifty-two respondents submitted over 600 lem; GI symptoms as modifiable determinants of children’s behav-
potential questions in English and in French. Following the removal of iors, and the child’s biological sex and treatment intensity as non-
‘out of scope’ questions, question collation, and our evidence check, 50 modifiable factors contributing the problem. Environmental factors
questions were put forward for prioritization by 201 participants. The include caregiver and clinician attitudes, beliefs, and behaviors (inter-
top 24 questions underwent final prioritization at a workshop attended personal level); and features of the hospital environment (organiza-
by 24 childhood cancer survivors, family members of children who tional level) that may affect children’s eating and self-management
have or had cancer, and multidisciplinary pediatric oncology clinicians. behaviors.
The top ten priorities reflect the breadth of the pediatric cancer con- Conclusions: Step 1 of the IM approach is useful in understanding
tinuum, focusing on access to new and innovative therapies; physical, complex health problems with influencing factors at multi-levels. This
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S545 of S635

tentative model will be refined through collection of data from chil- P105 / #1194 DEVELOPMENT OF A HANDBOOK TO
dren, parents, and pediatric oncology clinicians. The results will inform PROMOTE THE RESILIENCE OF JAPANESE PARENTS OF THEIR
subsequent IM steps to develop a targeted intervention to reduce CHILDREN WITH CANCER
impaired nutritional status and improve QOL for Thai children with
cancer. Chika Kawakami1 , Akiko Araki1 , Miki Ohori2 , Junko Ogawa3 , Rina
Amano1
1 Toho University, Faculty Of Nursing, Tokyo, Japan; 2 Tokyo Healthcare
E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice University, Division Of Nursing, Faculty Of Healthcare, Tokyo, Japan;
Project 3 Shukutoku University, College of Nursing and Nutrition, School Of Nursing,

Chiba, Japan
NURSING POSTER SESSION
Background and Aims: As a factor related to recovery from mighty
P104 / #574 NURSING WORKING GROUP AT SIOP E START; stressors, support to enhance "resilience" has been getting atten-
TO SHARE EXPERIENCES AND STRENGTHEN OUR tion in recent years. The purpose of this study was to develop a
KNOWLEDGE IN A NETWORK self-administered handbook to promote resilience among parents of
children with cancer.
R.A. Karens1 , L.A. De Koning-Smits2 Methods: Data were obtained through semi-structured interviews
1 Princess Maxima Center, Pediatric Oncology, Utrecht, Netherlands; and analyzed using a qualitative approach. All interviews were con-
2 Princess Maxima Center, Nursing Staff, International Office, Utrecht, ducted from March to October 2021. Participants were seven par-
Netherlands ents whose children were undergoing treatment or had completed
treatment. The study was approved by the local ethics commit-
Background and Aims: SIOPE started a nursing group for nurses spe- tee. The analysis revealed the parents’ feelings from the time their
cialized in pediatric oncology, to exchange knowledge and experience child was diagnosed to the present, and the external and internal
and to facilitate research in nursing care. The ultimate aim is to bring factors that they recognized to help them overcome their adver-
pediatric oncology nursing care in Europe to a higher level. The official sities. Items for the handbook were generated from the results,
re-start of this group was in November 2021 and currently 15 coun- and the contents were discussed with experts involved in pediatric
tries participate. The structure of the nursing working group is very oncology.
similar to other SIOPE working groups, with an elected steering com- Results: The handbook’s contended begin with "Treatment Schedule,"
mittee and a chair, developed in accordance with SIOPE guidelines. The followed by "Points to keep in mind after discharge" and "Preparations
aim is to start round table meetings to promote networking between for returning to school" after the completion of treatment. In addi-
the European centers for all nursing professionals of each participating tion, it included not only the sick child but also the family and family
center. life events. The "Stories of Family Members Who Have Fought Disease
Methods: A good organization of these meetings makes the thresh- Before Me" was intended to help parents learn about the experiences
old low to participate. We propose to work with a rotating system, of others and promote their own resilience. Free descriptions were
whereby each time a country is responsible for organizing an online provided for parents to organize their emotions and thoughts, and to
network meeting. The organizing center selects E-Poster Topics for reflect on their own experiences later. The handbook was ring-file style
the round table and from each center nurses with expertise or inter- and can easily hold documents.
est on this E-Poster Topic will be invited to join the round table Conclusions: The structure of this handbook is designed to help
meeting. parents become aware of their psychological recovery through visu-
Results: The result for the nursing group is to engage members to alization of their experiences. However, verification is needed in the
exchange views and encourage debate to identify areas of common future. This work was supported by J SPS KAKENHI Grant Number 17
interest and to prioritize ideas for future development of care. As nurs- K 12374.
ing group SIOP E, we will empower and boost the E-Poster Topics that
are offered in the network. The nursing professionals of the participat-
ing centers will get to know each other better and will benefit of each P106 / #1223 KNOWLEDGE, ATTITUDES AND PRACTICES
expertise. REGARDING PAIN ASSESSMENT AMONGST NURSES WORKING
Conclusions: Networking with nurses improves knowledge. AT PUBLIC-SECTOR PEDIATRIC ONCOLOGY UNITS IN
Live meetings at international conferences and symposia PAKISTAN
are good for connection. We would like to plan a round
table meeting at SIOP Barcelona to invite other nurses. In Bashir Ahmed Khan1 , Natasha Baig2 , Muhammad Rafie Raza1
the future online meetings is an easy way to continue this 1 Indus Hospital and Health Network, Pediatric Oncology, Karachi, Pakistan;

collaboration. 2 The Indus Hospital and health Network, Paeds Oncology, Karachi, Pakistan
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S546 of S635 ABSTRACTS

Background and Aims: Pain in pediatric oncology patients is often with other nurse donor experiences—an exploration of role-
undertreated due to lack of timely assessment and inefficient commu- reversal.
nication between healthcare workers. Improper pain assessment is a Methods: An autoethnographic retroactive and selective accounting
leading cause of poorly managed pain in children, negatively affecting highlights my interactions with the donor experience and makes vis-
quality of life. In high-income countries, pediatric oncology nurses play ible my emotions and thoughts as both “patient” and professional.
a key role in developmentally appropriate pain assessment measures A medical anthropologist assisted in data and experience analysis. A
to identify potential management strategies. However, nurses in low- review and comparison of marrow/stem cell donation process accounts
middle income countries (LMICs) face a deficit of knowledge about pain by nurses from grey literature conducted to include additional role-
assessment tools and management. Owing to differences in availabil- reversal data.
ity of resources, a disparity exists between health-related quality of Results: Medical knowledge of bone marrow transplant and donation
life of cancer patients treated at public and private sector hospitals in processes did not provide the mental preparedness required by a donor
Pakistan. for this nurse. Like most pediatric oncology nurses with experience
Methods: The Indus Hospital and Health Network partnered with caring for patients undergoing a bone marrow transplant, I had not
nine public-sector hospitals nationwide to improve pediatric oncology given much thought to the donor’s experience as our focus is on our
practices. Supported by the My Child Matters grant, training sessions patients. My donor experience significantly raised my consciousness
were conducted for nurses at each public-sector pediatric oncology of the intensity of the donation experience, which now informs my
unit (POU) from March to December 2021. Pain assessment tools practice.
were provided. To assess retention and implementation of practices, Conclusions: Through my donor experience, I have developed greater
a knowledge, attitudes and practices questionnaire was distributed empathy as a nurse. This aligns with the donor experiences of other
online to nurses at each POU. All responses remained anonymous. nurses as documented in grey literature. I believe that all hematol-
Results: Fifty four responses were recorded, 87% female and most ogy/oncology nurses would benefit from hearing the emic perspective
between 26 and 30 years of age. Majority participants held a Diploma of a nurse donor’s story to raise their awareness of the full scope of
in Nursing, were designated Charge Nurses and had over 6 years of donor needs and care. Nurses can provide more holistic care to their
experience. Forty nurses reported routinely assessing pain; the most donor “patients” and have greater respect and appreciation for the
common reason for not doing so was increased workload. Signifi- intricate the process leading up to the donation. By sharing my expe-
cant correlations were observed between routinely performing pain rience of role reversal, nurses will have a unique insight into a complex
assessment and maximum patients per nurse, availability of formal cre- and so far, under-examined aspect of bone marrow donation.
dentialing or certifications at institution and routinely performing pain
assessment, availability of trainings focused on pain assessment and
routinely performing pain assessment, and qualification of nurses and E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
knowledge of non-pharmacological pain assessment methods. Project
Conclusions: Strategies to improve pain assessment knowledge and
practices amongst pediatric oncology nurses in LMICs must be devel- NURSING POSTER SESSION
oped to improve patientcare and clinical outcomes.
P108 / #533 A MULTIDISCIPLINARY APPROACH TO I-131
MIBG THERAPY FOR THE TREATMENT OF NEUROBLASTOMA
E-Poster Topic: AS04 Nursing / AS04.a Education
Elaine Lambrinos1 , Megan Lurvey2 , Jennifer Spidle3
NURSING POSTER SESSION 1 Boston Children’s Hospital, Oncology, Boston, United States of America;
2 Boston Children’s Hospital, Hematology/oncology, Boston, United States of

P107 / #184 THE PEDIATRIC ONCOLOGY NURSE AS America; 3 Dana Farber Cancer Institute, Oncology, Boston, United States of
TRANSPLANT DONOR: THE EMIC PERSPECTIVE America

Rachael Kunkel Background and Aims: MIBG therapy is an effective treatment for
Arkansas Children’s Hospital, Hematology/oncology, Little Rock, United stage IV Neuroblastoma, currently being studied as part of up front
States of America therapy, and also used for refractory and relapsed MIBG-avid Neu-
roblastoma. The purpose of this poster is to share lessons learned in
Background and Aims: Blood/bone marrow donation is an managing this unique treatment through a multidisciplinary approach
essential part of an allogeneic transplant in pediatric oncol- to improving patient care.
ogy. There is significant literature about donation by clinicians, Methods: Clinical and operational challenges were identified that con-
psychologists, and nurses; however, little written by donors. tribute to the fluidity of patient care both prior to admission and
Objective: A self-reflection and assessment by a pediatric oncol- during the hospitalization for MIBG including: pre-admission coordi-
ogy nurse serving as a bone marrow donor, and comparison nation and communication, caregiver compliance with radiation safety
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S547 of S635

guidelines, achievement of adequate anxiolysis, successful Foley Results: Literature for paediatrics was found to be limited, therefore
catheter placement, and nursing discomfort with radiation exposure. the adult literature was included. Five review papers and practice
Results: Multidisciplinary team members are engaged in several ini- guidelines recommended a minimum of 1.5g/kg/d. Three papers rec-
tiatives to streamline the care of MIBG patients. Prior to admission, ommended up to 2.5 g/kg/d for those with malnutrition or gut-graft-
education is provided to caregivers from the oncology team, radiation versus host disease (GvHD). Three paediatric oncology centres were
safety, and child life specialists (including handbooks and a video) which found to use the ASPEN Guidelines and five centres aim for a mini-
is then reinforced by radiation safety and nursing throughout the hos- mum of 1.5g/kg/d. Two centres increased to 2-3g/kg/d or by 20-30%
pitalization. Prior to admission, email communication and pre-admit for severe gut GvHD. The audit of RCH HSCT adolescents found that
huddles occur between the oncology team, pharmacy, pain service, and the mean provision of protein was 1.1g/kg/d which is 73% of the EPR.
nursing to review the patient’s case and hospital plan. Patient exams Conclusions: There is no international consensus on appropriate pro-
and past medical history have been expanded to include a thorough GU vision of protein in paediatric HSCT. The provision of protein in RCH
physical exam and an in-depth medication history (for patients receiv- HSCT adolescents was found to be suboptimal compared to the ASPEN
ing anxiolysis). In addition, monthly meetings with the multidisciplinary guidelines. There is limited evidence to understand whether protein
team provide an opportunity to review areas for improvement with intake impacts on HSCT outcomes. Further research is recommended
past cases and to plan for upcoming treatments. Educational sessions to develop evidenced-based guidelines in this nutritionally vulnerable
have been provided to nursing focused on radiation safety and Foley cohort.
catheter placements.
Conclusions: Collaboration between oncology, nursing, radiation
safety, pain service, urology, nuclear medicine, and child life special- P110 / #1635 THE ROLE OF NURSING IN THE 101 TRIAL
ists is key in the provision of safe and optimal care for patients with WITH OMBURTAMAB FOR PATIENTS WITH CENTRAL
Neuroblastoma undergoing MIBG therapy. Educational materials and NERVOUS SYSTEM RELAPSE OF NEUROBLASTOMA
discussions with patients/caregivers and staff are vital for this complex
therapy. Patients, caregivers, and staff are well supported and benefit Cristina Llanos Príncipe, Sandra López Miralles, Mari Carmen Molero
from using a multidisciplinary approach. Solis
Research Foundation Sant Joan de Déu, Clinical Trials Unit, Esplugues de
Llobregat, Spain
P109 / #905 PRACTICES ON PROTEIN PROVISION IN
ADOLESCENTS UNDERGOING HAEMATOPOIETIC STEM CELL Background and Aims: Conventional systemic treatment for newly
TRANSPLANT: A LITERATURE REVIEW, INTERNATIONAL diagnosed patients with neuroblastoma doesn’t adequately treat the
BENCHMARKING AND RETROSPECTIVE AUDIT central nervous system and leptomeningeal space, which may serve as
a sanctuary site leading to relapse. Besides this, there are significant
Karman Liu1 , Theresa Cole2 toxicities in those children who reach cure, associated with standard
1 The Royal Children’s Hospital, Nutrition & Food Services, Melbourne, therapies (surgery, radiotherapy and chemotherapy). Consequently,
Australia; 2 Royal Children’s Hospital, Allergy & Immunology, melbourne, new treatments, as radiolabeled monoclonal antibodies, are emerging.
Australia Methods: Trial 101 (NCT03275402) is a single-arm, international
study (4 US and 1 Spain) in patients aged 0-18 years with radiograph-
Background and Aims: Protein requirements in adolescents under- ically and/or histologically confirmed neuroblastoma with CNS/LM
going haematopoietic stem cell transplant (HSCT) is unknown. In the metastases. Treatment with 131I-omburtamab consist of a 2 mCi
absence of specific evidence-based guidelines for HSCT, at the Royal dosimetry dose followed one week later by up to two cycles of admin-
Children’s Hospital (RCH) in Melbourne, Australia we use the Ameri- istration of 50 mCi activity and 4-week observation period/cycle.
can Society for Parenteral and Enteral Nutrition (ASPEN) Critically Ill Radiolabeled antibody administration requires a formal safety and
Children Guidelines 2009 for estimated protein requirements (EPR). management training for clinical trial nurses as well as parents. The
The aims of our study are to review the literature and to compare cur- objectives of this study include the development of the training mate-
rent local and international practices in enterally and parenterally-fed rial for patient/parents and environmental safety in the outpatient
HSCT patients. setting for Omburatamb treatment.
Methods: Databases CINAHL, Cochrane Central, EMBASE and MED- Results: The developed protocol for patients treated with radiolabeled
LINE from 2006-2021 were searched in both paediatrics and adults medications include nursing visit with patient and parents prior to
using the terms “protein requirements”, “bone marrow transplant” and first infusion to focus on techniques and recommendations to deal
“haematopoietic stem cell transplant”. To assess international prac- with expected toxicities; qualifying nursing staff for being capable to
tice, 11 international paediatric oncology centres were contacted. A face this specific administrations with adequate monitoring strategies;
retrospective audit of 15 HSCT adolescent patients at RCH was per- enrolment of supportive care professionals to increase patient well-
formed, to assess provision of protein compared with the ASPEN being before, during and after infusion. Specific monitoring registries
recommendation of 1.5g/kg/d. and specific nursing care during infusion.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S548 of S635 ABSTRACTS

Conclusions: Adopting this treatment to our site required an in- P112 / #82 CANCER AND PALLIATIVE CARE IN RURAL
depth analysis of all particular medications and procedures, because SETTINGS IN INDIA
as nurses, we must adapt current caring to new therapies require-
ments and implement appropriate procedures, care and circuits to Nabanita Mandal
ensure patients and their families safety as well as health staff and Narikeldaha Prayas, Palliative Care, Panskura, India
environment safeness.
Background and Aims: In a southern district of West Bengal, India
almost 75% of cancer patient die a sad death of neglect due to lack
P111 / #1671 THE JOURNEY OF CAR-T CELL THERAPY: of awareness about palliative care and low economic level. To identify
PEDIATRIC PATIENT’S PERSPECTIVE and try to solve to the extent possible the main difficulties in giving
palliative care to the terminal cancer patients of the area.
Cristina Llanos Príncipe1 , Georgina Pedrals Portabella2 , Alba Ropero Methods: Home visit by volunteers and enumeration of the problems
Palacios3 , Sandra López Miralles1 as discussed by the patient and their families.
1 Research Foundation Sant Joan de Déu, Clinical Trials Unit, Esplugues de Results: Analysis the following data and identify these main problems.
Llobregat, Spain; 2 Hospital Sant Joan de Déu, Cart Unit, Esplugues de Llo- Patient problems: Pain, vomiting, respiratory distress, fatigue, etc. Our
bregat, Spain; 3 Hospital Sant Joan de Déu, Oncology Unit, Esplugues de volunteers visited terminal cancer patients and their families in our
Llobregat, Spain areas. Family problems: Inability to match work life with the care of the
patients. Adverse attitude of neighbors and local peoples. Social prob-
Background and Aims: Even though the outcomes of pediatric and lems: Lack of awareness of the neighbor of local people about cancer
young adult patients diagnosed with acute lymphoblastic leukemia and palliative care resulting in isolation of the family. Projected Inter-
(ALL) have improved significantly in the past few decades, approx- vention: Trying to relieve the patient’s problems through home based
imately 2–3% of patients will present with refractory disease and medications and intervention by volunteers and family members. Re-
15–25% of patients will relapse. To improve outcomes of patients orientating the attitude of family members through discussions and
with r/r disease, immunotherapies targeting specific B cell antigens other methods of communication i.e. get-together of cancer survivors.
are being developed. With the expanding use of CAR-T cell therapy, Social effort to raise the awareness of neighbors and local people
there is a need to characterize the patient’s experience over time to through discussion and other audio visual method (i.e. poster, leaflet,
guide patients and their families to face physical and emotional roller slide presentation, etc).
coaster experiences. To provide patient-oriented care, it is necessary to Conclusions: We believe that if we are able to continue our program
gather information about their experience. The patient’s perception of for a long enough period the suffering of the terminal cancer patient
the quality of contact with a health professional has a very important and their families might be resolved to a large extent over time.
subjective component. Communication skills, amount of information
provided, treatment and the environment can condition the percep-
tion of quality and are factors that decisively determine the patient’s P113 / #1103 IMPLEMENTATION OF A PEDIATRIC EARLY
experience. WARNING SYSTEM (PEWS) IN A PEDIATRIC ONCOLOGY UNIT
Studies that assess satisfaction with medical care in children treated OF A SPANISH HOSPITAL: ADVANTAGES AND LIMITATIONS
with CAR-T, either directly or indirectly, through the perception of par-
ents or caregivers, are scarce, even though it is a relevant part in the Marta Menchaca1 , Itziar Astigarraga2 , Maria Jose Lopez De La Serna1 ,
evaluation of health systems. Carmen Marcos1 , Pilar Martin1 , Irache Sanz3 , Margarita Diez De
Methods: An ambispective and qualitative study based on semi- Fuentes1 , Ruth Nocedal1 , Esther Benito1 , Maria Jose Liz3 , Amaia
structured individual interviews and focused on r/r B-ALL patients who Polo3 , Mercedes Ruiz De Azua3 , Susana García-Obregón2 , Javier
benefited from CAR-T cell therapy from 7 years of age at the time of Pilar-Orive4 , Ricardo López-Almaraz1
administration of CAR-T therapy. 1 Hospital Universitario de Cruces. IIS Biocruces Bizkaia, Unidad De Hema-
Results: Knowing the experience and perceived needs regard- tología Y Oncología Pediátrica, Barakaldo, Spain; 2 Hospital Universitario de
ing the received care and information provided during the Cruces. IIS Biocruces Bizkaia. Universidad País Vasco UPV/EHU, Unidad De
process of CAR-T therapy and improving care satisfaction Hematología Y Oncología Pediátrica, Barakaldo, Spain; 3 Hospital Universi-
for children and adolescents with B-ALL who undergo CAR-T tario de Cruces, Unidad De Hematología Y Oncología Pediátrica, Barakaldo,
treatment. Spain; 4 Hospital Universitario Cruces. IIS Biocruces, Unidad De Cuidados
Conclusions: Taking into account that a huge amount of our patients Intensivos De Pediatría, Barakaldo, Spain
is treated away from their primary oncology team with which they
are most familiar, the development of a welcome programme for Background and Aims: Hospitalized pediatric cancer patients have
pediatric patients with B-ALL receiving CAR-T therapy, following high-risk of life-threatening complications. Our aim is to analyze the
already treated patients background, could improve future patient’s implementation of validated PEWS in our Pediatric Oncology Unit and
experience. the utility for identifying early clinical deterioration.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S549 of S635

Methods: Descriptive study of the Onco-PEWS implementation pro- Results: The 1st scientific day (March 06, 2021), with 16 attendees
cess in one year (December 2017-December 2018), a pilot study covered line care, cardiopulmonary resuscitation and emergency trol-
(November 2017) and the specific training for Oncology and PICU staff. ley, and the administration and monitoring of Ifosfamide and high-dose
Review of PEWS numerical values (0-5) assigned by nurses (cardiovas- Methotrexate. The 2nd scientific day (October 23, 2021) addressed
cular, respiratory, neurological signs) and family concerns of inpatients transfusion and line care, with transfusion experts (16 attendees). The
below 18 years. The pediatricians performed the clinical evaluation 3rd scientific day (February 15, 2022) On the occasion of Interna-
of cases with abnormal scores. Scores were transferred to an actua- tional Childhood Cancer Day, the nurses presented their activities by
tion algorithm: 0-2(green): nursing assesses every 4 hours, 3-4(yellow): developing posters. The 4th scientific day (February 26, 2022) included
pediatricians notified, and ≥5 (red): mandatory PICU consultation. cytotoxic process, and patient risk management attended by pharmacy,
Results: Admissions at pediatric oncology ward were 454. Rea- physician and the nursing staff (26 attendees). We also include our
sons: chemotherapy (50.9%), fever (24.4%), debut (9.7%), surgery participation in international meetings (SIOP Africa Uganda, virtual
(9%), chemotherapy complications (5.3%). Underlying disease: 212 conferences, daily trainings, and nursing education sessions).
leukemias, 37 lymphomas, 142 solid tumors and 63 brain tumors. Conclusions: In reviewing these sessions, the nurses were input sought
Onco-PEWS complete registered: 51.5%. At admission: 94.3% green, for improvement, update knowledge, develop skills, exchange experi-
5.3% yellow, 0.4% red. At discharge: 98% green. During the stay: 14.7% ences, and improve their profession in Morocco. By striving to achieve
deteriorated Onco-PEWS (1.3% yellow to red, 2.2% red). Notice to the SIOP Baseline Nursing Standards, our nursing staff can increase
pediatrician 14.6%. Therapeutic interventions in 13.2%: 4 yellow and the quality of care and safety of nursing practice. Nursing recom-
1 red Onco-PEWS required surgery. Transfer to PICU in <1 hour in 9 mendations: encourage central lines use, reinforce communication,
patients (1yellow, 8 red). Only one death terminal disease. update basics of cardiopulmonary resuscitation, and cart management,
Conclusions: In our experience, the Onco-PEWS is a feasible and transfusion and chemotherapy risk management.
effective tool that helps to identify clinical deterioration and risk to
PICU transfer. It empowers nurses to identify clinical changes and
allows appropriate response with timely interventions that improves P115 / #1485 PEDIATRIC ONCOLOGY NURSING
outcomes. However, the full mid-registration can be improved. Rapid CONTINUOUS CAPACITY BUILDING IN ETHIOPIA
PEWS deterioration in younger children suggests that narrower age-
specific ranges should be implemented. Esubalew Mezgebu1 , Diriba Hordofa2 , Daniel Wolde3 , Helina
Mekonnen4 , Abdulkadir Gidey4 , Daniel Kefenie4 , Ali Dinkiye5 , Tadele
Bekele5 , Mulugeta Yimer6 , Mulugeta Wassie6 , Julie Broas7 , Aziza
E-Poster Topic: AS04 Nursing / AS04.a Education Shad8 , Julia Challinor9
1 Jimma University Medical center, Pediatric Oncology, JIMMA, Ethiopia;
NURSING POSTER SESSION 2 Jimma University Medical Center, Pediatric Oncology, Jimma, Ethiopia;
3 TikurAnbessa Specialized Hospital, College of Health Sciences, Addis
P114 / #1112 NURSES TRAINING IN MOROCCO : REPORT Ababa University, Pediatric Oncology, Addis Ababa, Ethiopia; 4 Tikur

FROM THE PEDIATRIC ONCOLOGY DEPARTMENT OF RABAT Anbessa Specialized Hospital, Pediatric Oncology, Addis Ababa, Ethiopia;
5 St. Paul Hospital Millennium Medical College, Pediatric Oncology, Addis
Malika Meskini, Younes Labrini, Amina Kili, Maria El Kababri, Leila Ababa, Ethiopia; 6 University of Gondar Comprehensive Specialized Hos-
Hessissen pital, Pediatric Oncology, Gondar, Ethiopia; 7 The Aslan Project, Executive
Ibn Sina University Hospital, Pediatric Oncology Department, RABAT, Director, Washington DC, United States of America; 8 Children’s Hospital at
Morocco Sinai, Pediatric Hematology Oncology, Baltimore, United States of America;
9 University of California San Francisco, School Of Nursing, San Francisco,
Background and Aims: Nursing education in pediatric oncology is United States of America
the cornerstone of all the actions of the strategies for improving the
quality of care. The SIOP Baseline Standard for Pediatric Oncology Background and Aims: As of March 2013, there was no dedicated
Nursing in low- and middle-income countries calls for a minimum of pediatric oncology units (POUs) or specialized care in Ethiopia. Cur-
10 hours/year of continuing education. To cope with modern devel- rently there are active POUs in four cities. Since 2013, >6,100 children
opments, the Children Hospital of Rabat, with 24 pediatric oncology with cancer have been treated and Ethiopian physicians, nurses, and
nurses, must therefore ensure the updating and permanent progress pharmacists trained in childhood cancer care in partnership with
of its nurses. Objective: Report our experience during 2 years (2021- US-based, non-governmental organization, The Aslan Project (Aslan).
2022) of nursing continuing education activities over 4 scientific days. International pediatric oncology nurse educators have conducted mul-
This training was supported by the Francophone African Group of tiple onsite trainings and select Ethiopian pediatric oncology nurses
Pediatric Oncology GFAOP and the Sanofi Espoir foundation have attended trainings in Pakistan and India. Capacity-building goals
Methods: Collect and evaluate all pediatric oncology nursing continu- of the Ethiopian pediatric hematology-oncology (PHO) teams and
ing education trainings to identify gaps for future programming. Aslan experts are to address gaps in care and promote local program
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S550 of S635 ABSTRACTS

ownership. Cancellations of planned training programs due to COVID Methods: We distributed model-1 and a self-administered, anonymous
spurred local leadership; thus, the Rising Nurse Project 2021 was cre- questionnaire survey to 373 health care professionals working at 20
ated. Objective: Develop a standardized Ethiopian pediatric oncology hospitals treating childhood cancer in some areas of Japan.
nursing training curriculum and manual for continuous and sustainable Results: The questionnaire was validated for 108 respondents (valid
professional development. response rate: 29.0%). They were 53 physicians, 26 nurses, 6 psychol-
Methods: Ethiopian PHO physicians and nurse educators, with con- ogists, 5 social workers, and 18 others. The diagram and explanatory
sultation from three international experts, wrote the curriculum for a text of model-1 were understood or generally understood by 72.2%
two-week nursing orientation training, in line with the SIOP Nursing and 84.2% of the respondents, respectively. The suitability for CCS and
Baseline Standards recommendations. Pilot trainings were conducted utility for the health care professionals of the model were recognized
in two sites with post-training assessment by local and international by 76.8% and 80.2% of the respondents, respectively. The most com-
trainers. mon responses that they could be useful were “predicting what CCSs
Results: Twenty-two nurses participated. Interest in expanding train- may experience” and “considering professional support for what CCSs
ing to other centers led to creation of a Pediatric Oncology Nursing may experience”, both at 87.0%. All responses related to understanding
Task Force (PONTF) including Aslan’s international experts. PONTF is model-1 and its possibility for utilization were significantly correlated
working with the Federal Ministry of Health (MOH) to certify the cur- with Spearman’s ρ (p<0.001).
riculum as a national standard and has directed the development of Conclusions: These findings suggest that model-1 can be used by
comprehensive training modules. health care professionals to anticipate what CCSs might experience
Conclusions: Challenges for pediatric oncology nurses include a lack and to consider support for them. However, in order to increase
of professional recognition within health system, pay scale that the potential use of model-1, the diagram should be more easily
fails to reflect specialized work, frequent rotation of nurses, institu- understood.
tional policies, and mental/emotional burdens of patient care. Three
MOH/PONTF workshops are scheduled to complete the curriculum to
address the first challenge. Workshops will culminate in trainings at all P117 / #712 INVESTIGATING THE USEFULNESS OF THE
POUs led by local nurse educators. PROCESS MODEL IN WHICH PARENTS SUPPORT CHILDHOOD
CANCER SURVIVORS AS THEY BECOME INDEPENDENT WHILE
BALANCING HEALTH MANAGEMENT AND SOCIAL LIFE
E-Poster Topic: AS04 Nursing / AS04.b Research
Kyoko Miyagishima1 , Kazuko Ichie2 , Kimiyoshi Sakaguchi3 , Yuka
NURSING POSTER SESSION Kato4
1 Hamamatsu University School of Medicine, Faculty Of Nursing, Hama-
P116 / #700 INVESTIGATION OF THE USEFULNESS OF THE matsu, Japan; 2 Seirei Christopher University, Faculty Of Nursing, Hama-
PROCESS MODEL IN WHICH CHILDHOOD CANCER matsu, Japan; 3 Hamamatsu University School of Medicine, Pediatrics,
SURVIVORS BECOME INDEPENDENT WHILE BALANCING Hamamatsu, Shizuoka, Japan; 4 Shizuoka Children Hospital, Department Of
HEALTH MANAGEMENT AND SOCIAL LIFE Nursing, Shizuoka, Japan

Kyoko Miyagishima1 , Kazuko Ichie2 , Kimiyoshi Sakaguchi3 , Yuka Background and Aims: To provide comprehensive long-term support
Kato4 for pediatric cancer survivors (CCSs), it is necessary for professionals
1 Hamamatsu University School of Medicine, Faculty Of Nursing, Hama- in various sectors to understand the process and factors that influence
matsu, Japan; 2 Seirei Christopher University, Faculty Of Nursing, Hama- the long-term support of children by parents of CCSs. The authors’
matsu, Japan; 3 Hamamatsu University School of Medicine, Pediatrics, previous studies identified “the process of parents’ support as CCSs
Hamamatsu, Japan; 4 Shizuoka Children Hospital, Department Of Nursing, become independent while balancing health management and social
Shizuoka, Japan life from adolescence to adulthood” (model-2). The purpose of this
study is to clarify the validity and applicability of model-2 from the per-
Background and Aims: To provide comprehensive long-term sup- spective of professionals who have experience in supporting CCSs and
port for childhood cancer survivors (CCSs), professionals from various their parents.
departments need to understand the process of long-term change Methods: We distributed model-1 and a self-administered, anonymous
and its influencing factors from the perspective of CCSs. The authors’ questionnaire survey to 373 health care professionals working at 20
previous studies identified “the process of becoming independent hospitals treating childhood cancer in some areas of Japan.
while balancing health management and social life in CCSs from ado- Results: The questionnaire was validated for 108 respondents (valid
lescence to adulthood” (model-1). The purpose of this study is to response rate: 29.0%). They were 53 physicians, 26 nurses, 6 psychol-
clarify the validity and applicability of model-1 from the perspective ogists, 5 social workers, and 18 others. The diagram and explanatory
of professionals who have experience in supporting CCSs and their text of model-2 were understood or generally understood by 87.1%
parents. and 91.6% of the respondents, respectively. The suitability for the CCSs
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S551 of S635

and utility for the health care professionals of the model were recog- Conclusions: The study indicates that there is a gap in the knowledge
nized by 75.9%, and 82.6% of the respondents, respectively. The most base of caregivers of pediatric oncology patients. The findings demon-
common response that they could be useful was "predicting what par- strate that there is a significant requirement for not just nutritional,
ents will experience" at 87.9%, followed by "understanding parents’ health and hygiene counselling but also a necessity for financial and
experiences" at 87.0%. All responses related to understanding model- emotional support.
2 and its possibility for utilization were significantly correlated with
Spearman’s ρ (p<0.001).
Conclusions: These findings suggest that model-2 can be used by E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
health care professionals to understand the experiences of parents of Project
CCSs and to predict what they may experience. However, it should be
considered that some parents may not suit the model. NURSING POSTER SESSION

P119 / #653 PRACTICAL APPROACH TO HIGH-DOSE


E-Poster Topic: AS04 Nursing / AS04.a Education METHOTREXATE INTOXICATION: FOCUS ON NURSING
ASPECTS
NURSING POSTER SESSION
Sofie Moreels, Liesbeth Steendam, Veerle Mondelaers, Annelies
P118 / #922 PERCEPTIONS OF NUTRITIONAL NEEDS AND Mannaerts, Tiene Bauters
SUPPORT OF PEDIATRIC ONCOLOGY PATIENT CAREGIVERS IN UZ Gent, Pediatric Hematology, Oncology And Stem Cell Transplantation,
GOVERNMENT HOSPITALS IN INDIA Gent, Belgium

Anju Morarka1 , Sripriya Venkiteswaran2 Background and Aims: High-dose (HD) methotrexate (MTX) is essen-
1 Cuddles Foundation, Research, Knowledge Management And Impact, tial in the treatment of many childhood cancers. The use of HD-MTX
Mumbai, India; 2 Cuddles Foundation, Cuddles Institute Of Clinical Nutrition can be associated with multiple side effects and requires close mon-
(cicn), Mumbai, India itoring and preventive measures to avoid life-threatening toxicities.
Although preventive measures have always been in place in our hospi-
Background and Aims: Approximately 40% of pediatric oncology tal’s guidelines, we were recently faced with HD-MTX toxicities, which
patients are malnourished at diagnosis in India. Malnutrition during prompted us to perform a root analysis of our practice. We here focus
cancer treatment can lead to greater risk of infections, side effects, on the impact on nursing care.
complications and treatment delays. Caregivers are integral to pre- Methods: A root analysis including identification of the contributing
venting and addressing malnutrition in children with cancer. Therefore, factors, interpretation and possible solutions followed by communica-
caregiver education is crucial to ensure that they have the knowledge tion to the team were performed.
required to manage this responsibility. The aim of this study was to Results: Root analysis revealed that small inter-individual approaches
therefore understand the knowledge, attitudes and practices of care- in follow-up and management of toxicities were uncovered. The infu-
givers of pediatric oncology patients belonging predominantly to the sion setup and number of manipulations were identified as possible
lower socioeconomic group. causes for misinterpretations. To improve patient safety, we imple-
Methods: We administered a structured questionnaire to caregivers of mented a multidisciplinary follow-up document for closer monitoring
oncology patients aged 0 to 18 years in March 2021. The study was of different parameters per time point, including blood sampling (MTX
conducted during the nutrition counselling session that Cuddles Foun- level, creatinine level), hyperhydration volume and infusion time and
dation (CF) provides and questions were designed to elicit data on their dose of folinic acid administration based on the MTX level. Second,
knowledge and attitudes on nutrition, hygiene and health behaviours. current instructions on how to handle urine alkalinisation, hyperhy-
Responses were collected on a 3-point Likert scale (Yes, Somewhat, dration and folinic acid rescue were written out more detailed in a
No). specific intoxication policy document. Third, we reduced the likelihood
Results: Caregivers of 754 patients participated across 31 govern- of errors during the administration of HD-MTX, by reducing the num-
ment hospitals in India. The results indicated that 80.1% of caregivers ber of manipulations on the infusion setup based on suggestions of
did not know the proper foods to feed their child at cancer diag- our nursing team. The hyperhydration is only interrupted to administer
nosis. The findings also demonstrated that most caregivers (58.5%) antiemetics.
were unaware of the proper hygiene practices required to handle Conclusions: Nurses have a key role in the administration of HD-MTX
their child’s food. Unfortunately, even after the caregivers under- and concurrent follow-up. By implementing the follow-up document,
stood the nutritional requirements, 33.9% of caregivers did not have all key factors during HD-MTX administration are now monitored
the means to provide that for their child. The majority of care- more closely. The more detailed instructions are more comprehensive
givers (88.1%) found that the support groups conducted by CF were for all nurses. By using a bottom up insight of the nurses to sim-
helpful. plify the infusion setup, nurses feel more confident during HD-MTX
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S552 of S635 ABSTRACTS

administration. A prospective follow up of this change in practice is 1 University of Leeds, Leeds Institute For Data Analytics, Worsley Building,
currently ongoing. Leeds, United Kingdom; 2 University of Leeds, School Of Medicine, Wors-
ley Building, Leeds, United Kingdom; 3 University of Leeds, Leeds Institute
For Data Analytics, Leeds, United Kingdom; 4 Oxford University Hospitals
E-Poster Topic: AS04 Nursing / AS04.b Research NHS Trust, Paediatric Oncology, Oxford, United Kingdom; 5 MRC Centre
for Reproductive Health, Queens Medical Research Institute, University Of
NURSING POSTER SESSION Edinburgh, Edinburgh, United Kingdom

P120 / #1098 KNOWLEDGE ON NURSING PREVENTATIVE Background and Aims: To generate a resource to support service
MEASURES AMONGST ADOLESCENTS IN GANTSI, BOTSWANA development, evaluation and research, we are establishing a UK Reg-
ister of stored ovarian and testicular tissue (UKSTORE). We performed
Puseletso Mothathelo a series of patient and public engagement consultations to determine
Ministry of Health and wellness, Clinical Services, Ghanzi, Botswana what aspects of data capture, consent and storage would be acceptable
to service users.
Background and Aims: THE ABSTRACT Introduction-cancer remains Methods: Participants were recruited via useMYdata, DATACAN,
a leading cause of death in many developing countries such as Leeds Research Owls, Candlelighters, Children’s Cancer and
Botswana with adolescents included in the statistics. this study was Leukaemia Group and Teenage Cancer Trust. Two 1.5-hour vir-
was carried out to investigate knowledge amongst adolescents in tual meetings were used to explore views of data capture and data
Gantsi, Botswana to find out how much knowledge they have on cancer use for UKSTORE. Following an overview of UKSTORE aims and
screening and prevention measures. objectives, participants were asked to choose answers to 5 multiple
Methods: Design-the study was carried out on 6 adolescents, 3 were choice questions using Mentimeter web-based technology, the online
out of school adolescents who were selected randomly as they come chat function and/or verbal discussion.
for consultations at Gantsi Primary Hospital (OPD), 3 were schooling Results: Twelve young people aged 13-18 and seven adults, including
adolescents from Gantsi Senior who were also selected randomly from three parents of children who had undergone ovarian or testicu-
the school, information was gathered through oral interviews. lar tissue preservation attended the consultations. Quantitative data
Results: -My analysis shows that even though there is information on showed a strong preference to data capture without patient informed
cancer screening and prevention available on different sources, fair consent, including data linkage and retention. Adults prefer to receive
number of adolescents in Gantsi know about them, 1 (17%) said he information about the registry via a printed leaflet and children pre-
do not know anything about cancer, 2 (33%)said they do not know fer a mixture of written and web-based materials. Children preferred
that cancer could be prevented, they thought it is a natural disease, to speak to their doctor face to face. Qualitative data highlighted the
1 (17%) metioned that she is aware of pap smear done on females to importance of whole population data registries, application of an opt-
screen for cervical cancer, she commented that she knows it allows out approach and the reassurance of robust data for the purposes of
for early detection of the disease, 2 (33%) were highly knowlegeable learning and understanding patient needs. A registry of young people
and even elaborated on factors that can expose one to cancer such as who have stored tissue for future fertility was deemed necessary and
active/passive smoking, use of drugs, and also that cancer can come important. The majority of patients consulted would be happy for their
as an opportunistic infection if a person contact other diseases like data to be captured and used without written informed consent.
HIV/AIDS. Conclusions: Qualitative consultation has generated informative and
Conclusions: Conclusion-50% of my participants showed complete no supportive intelligence ensuring ethical and consent issues are appro-
knowledge on cancer prevention and screening, that is a large number priately handled in the development of a new data resource. Acknowl-
therefore I conclude that information dissermination is low, a lot needs edgements: Thank you to all the participants who kindly attended the
to be done to sensitize not only the adolescents in my study but also the meetings.
Gantsi population at large on cancer prevention and screening looking
at the fact that my place of study is one of the areas in Botswana with
high substance abuse and cancer cases. P122 / #319 THE TIMING WHEN THE MOTHER OF A CHILD
UNDERGOING EYE ENUCLEATION DUE TO RETINOBLASTOMA
STARTS MIGRATING SELF-CARE OF ARTIFICIAL EYE TO CHILD
P121 / #1275 PATIENT AND PUBLIC VIEWS ON
INFORMATION, CONSENT AND DATA SHARING IN THE Michie Nagayoshi1 , Kyoko Toju2 , Masaharu Akiyama3 , Takaaki
REGISTRY OF STORED OVARIAN AND TESTICULAR TISSUE FOR Yanagisawa4 , Kohei Takita5 , Kinu Takahashi1
YOUNG PEOPLE IN THE UK 1 The Jikei University, School Of Nursing, Tokyo, Japan; 2 National Cancer
Center Hospital East, Nursing, Chiba, Japan; 3 The Jikei University School
Rebecca Mottram1 , Adam Glaser2 , Richard Feltbower3 , Sheila Lane4 , of Medicine, Department Of Pediatrics, Tokyo, Japan; 4 The Jikei University
Rod Mitchell5 , Richard Anderson5 School of Medicine, Division Of Paediatric Neuro-oncology, Tokyo, Japan;
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S553 of S635

5 Saitama Prefectural University, School Of Health And Social Services, 3- to 10-year-old children undergoing enucleation due to RB who have
Saitama, Japan experience of administrating either ongoing or completed migration
of artificial eye self-care to their children. Mothers were recruited
Background and Aims: Parents of children who underwent eye enu- through RB family liaison groups. This study was approved by the
cleation in an early postnatal period due to retinoblastoma (RB) seek a research ethics committee of the author’s institution.
way of migrating self-care of an artificial eye to their children with little Results: The mean infant age was 6.1 years (SD = 2.0). The mean age
support by specialists. We conducted a study to understand the prob- of enucleation was 19.8 months (SD = 11.9). From 75 subcategories,
lems arising from such a migration process. The objective of this study 26 categories were extracted, including "Parents prepare a space and
was to identify the appropriate shift timing. items for children to facilitate doing self-care," "Encourage children to
Methods: Individual semi-structured interviews were conducted for understand the purpose of self-care," "Wash and wipe to clean the arti-
mothers who have 3- to 10-year-old children with RB under a qual- ficial eye used all day," "Wash and wipe to clean in the orbit and around
itative descriptive design. Participants were 18 mothers, recruited the area that the artificial eye touches," "Hygienically treat an artificial
through RB family liaison groups in Japan. The mean age of mothers eye," "Devise a way to remove eye discharge," "Open eyelid to wear an
at the time of interview was 38.9 years (SD = 3.9). The child’s mean artificial eye with fingers," "Take good care of one’s own sense during
age at the time of eye enucleation was 19.8 months (SD = 11.9). This wear/removal of artificial eye."
study was approved by the research ethics committee of the author’s Conclusions: At the beginning of children’s self-care of artificial eye,
institution. parents took initiative to promote children’s understanding of the pur-
Results: To investigate the timing when mothers began to encourage pose of self-care and increase their motivation in accordance with
their children to take artificial eye care by themselves, 7 categories development of finger dexterity or cognitive function. There were
and 4 large categories were extracted from 16 subcategories. Some methods for the care of an artificial eye. Parents devised an appro-
children began to look after their artificial eyes, as suggested by “My priate procedure according to children’s development and condition
child unconsciously began to remove his/her artificial eye by him- in the orbit and supported treatment-related care such as techniques
self/herself” and “My child became able to wear/remove, clean, and that infants had not gained yet or ocular instillation. Caregiver should
adjust the position in his/her way.” Other children were encouraged by establish a system to help parents at any time.
their mothers, as suggested by “I encouraged him/her not to miss the
timing when my child wanted to imitate me or look after an artificial
eye by himself/herself.” The parental intervention began at their own E-Poster Topic: AS04 Nursing / AS04.a Education
discretion or after “leaning from experience or advice by experts.”
Conclusions: Parents encouraged their children to do self-care when NURSING POSTER SESSION
they were interested in looking after their artificial eye, and sought
an appropriate migration timing because they wanted their children to P124 / #1287 CONTINUOUS NURSING EDUCATION IN
learn self-care before entering preschool/school, where children can UGANDA: A SUSTAINABLE MODEL FOR LOW- AND
no longer be under the supervision of their parents. MIDDLE-INCOME COUNTRIES

Mariam Ndagire1 , Isaac Mulyowa1,2 , Joyce Kambugu1 , Susan


P123 / #901 ACTUAL STATUS OF SELF-CARE ON ARTIFICIAL Nabakooza1 , Fadhil Geriga1
EYE IN CHILDHOOD AFTER ENUCLEATION DUE TO 1 Uganda Cancer Institute, Paediatric Oncology Department, Kampala,
RETINOBLASTOMA Uganda; 2 Uganda Cancer Institute, Pediatric Oncology Clinic, Kampala,
Uganda
Michie Nagayoshi1 , Kyoko Toju2 , Masaharu Akiyama3 , Takaaki
Yanagisawa4 , Kohei Takita5 , Kinu Takahashi1 Background and Aims: BACKGROUND The International Society of
1 The Jikei University, School Of Nursing, Tokyo, Japan; 2 National Cancer Pediatric Oncology PODC Baseline Nursing Standards 2 and 3 state
Center Hospital East, Nursing, Chiba, Japan; 3 The Jikei University Hospi- formalized orientation and continuing education as critical for a suc-
tal, Department Of Pediatrics, Tokyo, Japan; 4 The Jikei University School cessful pediatric oncology service. Specialized education and clinical
of Medicine, Division Of Paediatric Neuro-oncology, Tokyo, Japan; 5 Saitama training for new nurses and formal mandatory continuing nursing
Prefectural University, School Of Health And Social Services, Saitama, Japan education are rarely available in low-income countries and likely con-
tribute to continued disparity in survival. Uganda has no accredited
Background and Aims: In Japan, no guidelines for migrating self-care pediatric oncology nursing program, so nurses learn through men-
of artificial eye to the child undergoing enucleation due to retinoblas- torship from (and observership) of senior nurses, as well online free
toma (RB) have been established. The purpose of this study was to courses. The Uganda Cancer Institute (UCI) pediatric department
identify actual self-care on an artificial eye of RB children. started weekly continuing nursing education (CNE). OBJECTIVE Meet
Methods: Individual semi-structured interviews were conducted increasing demand for specialized knowledge required for childhood
under a qualitative descriptive design. Participants were mothers of cancer nursing practice.
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S554 of S635 ABSTRACTS

Methods: Weekly one-hour CNE sessions are conducted by a senior interventions were implemented to address the three themes that
nurse, and sometimes by a pediatric oncologist, for pediatric nurses emerged from the gap analysis, these included work flow issues, doc-
caring for approximately 500 children with cancer (received annu- umentation, and educational consistency in patient/family education.
ally). CNE sessions delivered to diploma and bachelor-prepared nurses The educational work led to the creation of documents necessary
and occasionally attended by adult oncology nurses, doctors and to guide the nurse in caring for a patient receiving anticoagulation
fellows. Presentations include oncology nursing (e.g., oncologic emer- therapy.
gencies, chemotherapy administration and side-effect management), Results: To evaluate the effectiveness, when nurses discharges a
case studies and nursing implications of treatment protocols. patient, a five question survey is sent determine to determine the
Results: A CNE baseline assessment (by questionnaire with Likert usefulness of the created tools. Each educational tool has received
scale) in October 2018 showed improvement in nurses’ knowledge and a score of > 4 (5 being the highest) on the perceived value of the
attitudes. CNE outcomes include: quality improvement project, suc- document.
cessful My Child Matters 2020 Nursing project funding, and nurses’ Conclusions: The incidence of venous thromboembolism (VTE) has
improved confidence in clinical work and active participation in the been increasing due to the advances in technology and medical care
multi-disciplinary clinical team. in the pediatric population, especailly hospitalized children, patients
Conclusions: A cornerstone of successful treatment of childhood with central venous catheters, and patients recovering from surgical
cancer is the provision of specialized professional care in pediatric procedures (Mahajerin & Croteau, 2017). The vast amount of knowl-
oncology units. Ugandan pediatric oncology nurses manage disease- edge nurses need to know has exceeded any one’s ability to safely
related complications, coordinate care, administer chemotherapy, and and reliably manage each patient’s educational needs. Nurses must
educate patients and families. Our CNE program supports all these be empowered with tools, such as a checklist to provide focused,
activities and has proven to be sustainable and cost-effective. CNE consistent education and care each and every time.
has improved nursing care and multi-disciplinary team integration and
serves as an education model for nurses in other resource-limited
settings. P126 / #1291 OUTREACH PALLIATIVE CARE TO CHILDREN
WITH CANCER AT BANSO BAPTIST HOSPITAL (BBH) AND
MBINGO BAPTIST HOSPITAL(MBH) NORTH WEST CAMEROON:
E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice NINE YEARS (2013-2021) EXPERIENCE
Project
Kaah Joel Nkofon1 , Peter Bernard Hesseling2 , Paul Wharin3 , Kouya
NURSING POSTER SESSION Francine4 , Glenn Mbah Afungchwi4
1 Banso Baptist Hospital-Cameroon Baptist Convention Health Services,
P125 / #1983 ANTICOAGULATION ERROR REDUCTION Pediatric Oncology, Kumbo, Cameroon; 2 Stellenbosch University/Tygerberg
STRATEGIES Children’s Hospital, Pediatric Oncology, Cape Town, South Africa; 3 Beryl
Thyer Memorial Africa Trust, UK, Beryl Thyer Memorial Africa Trust, london,
Colleen Nixon1 , Juliann Duzan1 , Megan Lurvey2 , Emily Drake1 United Kingdom; 4 Mbingo Baptist Hospital-Cameroon Baptist Convention
1 Boston Children’s Hospital, Nursing, Boston, United States of America; Health Services, Pediatric Oncology, Mbingo, Cameroon
2 Boston Children’s Hospital, Hematology/oncology, Boston, United States of

America Background and Aims: Background Annually, about 140 children are
diagnosed with cancer, treated and cared for in the the Cameroon
Background and Aims: Anticoagulation medications are the lead- Baptist Convention Health Services(CBCHS) Childhood Cancer (CHC)
ing cause of acute serious drug events among hospitalized patients. program with approximately 50% chances of been cured. Majority who
Since 2008, reducing patient harm from anticoagulation has been a are not cured and their families need home-based palliative care ser-
Joint Commission National Patient Safety Goal. A safety event resulted vices. Palliative care (PC) remains an essential component for every
when a patient developed a pulmonary embolism after being dis- comprehensive childhood cancer treatment and management. In 2013,
charged with an incorrect enoxaparin prescription. This event led to BBH established an outreach PC with the aim of offering palliative care
a nurse-driven initiative to evaluate the processes at Boston Chil- to children with cancer at home. The same services were later estab-
dren’s Hospital for patients receivng anticoagulation therapy. Aims: lished at MBH in 2014 to serve the same purpose. Aim The main aim
The learner will identify potential opportunities for error reduction of the outreach pediatric palliative care(PPC) services is to offer pain
strategies for patients on high risk medications, specifically anticoag- management, symptoms management, psycho-social and spiritual care
ulants. to children with cancer on palliation at their homes.
Methods: A multidisciplinary group was convened and the root cause Methods: PPC nurse spent two weeks at each of the hospitals. Home
analysis (RCA) highlighted gaps in practice. These included: the order- visits conducted by PC nurse on motorbike every fortnight. Phone con-
ing and prescribing of anticoagulation therapy, the understanding of tact was maintained with families. A well-documented data base on
the treatment plan and outpatient follow-up plan. Specific, targeted each patient status was maintained and updated periodically.
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ABSTRACTS S555 of S635

Results: Over nine years, a total of 234 children with cancer on pal- regulations but also reasoned from ethics of care and virtue ethics
liation(both from BBH and MBH) received PC services with focus on perspectives.
pain and symptom management, psycho-social and spiritual care, 699 Conclusions: Health care professionals and researchers are guided
home visits were conducted which allowed the families to fully engage by care- and research ethical values, and report ethical challenges in
in decision making concerning advanced home end of life care plan as recruitment. There is a need to highlight ethical aspects of pediatric
wells as breaking of cultural barriers, 52bereavement visits were con- research. Promoting research ethical competence among health care
ducted as part of psycho-social and spiritual care, 1618 phone calls professionals and researchers may reduce moral distress and ensure
were made to maintain good communication between families and ethical quality in pediatric research.
health care team as to offer immediate support that was needed before
the next home visit.
Conclusions: An outreach PC service has offered and improved holis- E-Poster Topic: AS04 Nursing / AS04.a Education
tic care, health-related communication and quality of life for children
with cancer and families. It reduced needless hospitalization, promoted NURSING POSTER SESSION
rehabilitation, home-based end of life care and relieved burden of
care-giving. P128 / #1775 STATE OF THE PROFESSIONAL TRAINING OF
NURSING THAT SERVES PEDIATRIC ONCOLOGICAL PATIENTS
IN HEALTH INSTITUTIONS IN MEXICO
E-Poster Topic: AS04 Nursing / AS04.b Research
Ma Matilde Nuñez Martinez
NURSING POSTER SESSION HOSPITAL INFANTIL TELETÓN DE ONCOLOGÍA, EnseÑanza De Enfer-
merÍa, QUERETARO QUERETARO, Mexico
P127 / #1450 RECRUITING CHILDREN WITH CANCER TO
RESEARCH: A QUALITATIVE INTERVIEW STUDY EXPLORING Background and Aims: Background. The Child Cancer Global Initia-
ETHICAL VALUES AND CHALLENGES AMONG SWEDISH tive Nurse Specialists point out that specialized education in pediatric
HEALTH CARE PROFESSIONALS AND RESEARCHERS oncology is insufficient and one of the main concerns related to nursing
practice. In Mexico, as in other middle-income countries, it is neces-
Kajsa Norbäck1 , Anna Höglund1 , Tove Godskesen2 , Sara sary to recognize this need, as well as implement actions to resolve it.
Frygner-Holm3 Objective: To describe the state of education of nurses who treat pedi-
1 Uppsala University, Centre For Research Ethics And Bioethics, Depart- atric patients with cancer diagnosis in different health institutions in
ment Of Public Health And Caring Sciences, Uppsala, Sweden; 2 Marie Mexico.
Cederschiöld University, The Department Of Health Care Sciences, Upp- Methods: In the period from September to November 2021, a survey
sala, Sweden; 3 Uppsala University, Department Of Women’s And Children’s was applied to nurses who provide care to pediatric patients with can-
Health, Uppsala, Sweden cer to learn about their professional education and training in pediatric
oncology.
Background and Aims: Research remains crucial to improve treat- Results: Two hundred ninety-one individuals, with an average age of
ment, survival, and quality of life for children with cancer. However, 34.2 years, from different institutions in Mexico, 58% of public and
recruitment of children to research raises ethical challenges. This study 42% private hospitals, which have 5 and up to 20 beds allocated for
aimed to explore ethical values and challenges in recruitment of chil- onco-care50.9% attended between 2 and 4 patients, 28.9% between
dren with cancer to research, among health care professionals and 5 and 10 patients hospitalized per day and 20% provided ambulatory,
researchers in Sweden. Another aim was to explore health care pro- surgical, administrative, or supervisory care. 0.68% have specialty in
fessionals and researcher’s perceptions of ethical competence in the pediatric oncology, 6.18% specialty in general oncology, 4.4% diploma
context of recruiting children to research. in pediatric onco, 55% monographic courses in the last 3 years related
Methods: An explorative qualitative design, using semi-structured to pediatric oncology, 33.7% do not have training in pediatric oncology
interviews with seven pediatric oncologists and ten nurses. Interviews and 98.6% show interest in formal training.
were analyzed with inductive qualitative content analysis. Conclusions: The World Health Organization aims to increase survival
Results: The analysis resulted in five categories: Establishing to 60% by 2030 by improving diagnosis and treatment in low- and
relationships and trust, Meeting informational needs, Acknowl- middle-income countries. Our results confirm that the professional-
edging vulnerability, Balancing roles and interests, and Ensuring ization needs reach high levels since in the group of 291 respondents
ethical competence. Health care professionals and researchers only 2 are specialists in pediatric oncological nursing, a large percent-
described care-based, research-based and children’s rights-based age only perform short courses. The imperative challenge to solve is
ethical values in recruitment. Further, they reported ethical chal- the financing necessary to achieve and make possible in our environ-
lenges related to informed consent, vulnerability, and shared ment with limited resources the specialization in pediatric oncology
decision-making. They relied on research ethical principles and supported by technology to achieve the greatest reach in our country.
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S556 of S635 ABSTRACTS

P129 / #1805 COMPETENCIES OF THE NURSING Background and Aims: In Sweden, there are about 10,000 adult child
PROFESSIONAL ENTERING A PEDIATRIC ONCOLOGY cancer survivors (ACCS). In 75% of the survivor’s late complication
HOSPITAL: FACING THE REALITY IN MEXICO is found after 30-40 years of follow-up. In Gothenburg at the long-
term-follow up clinic for ACCS from West Sweden, had due to the
Ma Matilde Nuñez Martinez covid-19 situation change from physical to digital clinic. Aim Explore
HOSPITAL INFANTIL TELETÓN DE ONCOLOGÍA, EnseÑanza De Enfer- how a digital visit, by a video clinic was experienced by ACCS.
merÍa, QUERETARO QUERETARO, Mexico Methods: Invitation was sent to the patients with an offer of either dig-
ital or physical clinic, followed by a telephone call for decision of clinic
Background and Aims: Background. Teleton Oncology Children’s Hos- and instructions for digital visits. The visit was attended by the patient
pital receives about 20 newly hired nurses per year. The Task Force and the health care team, consisting of counselor, nurse, doctor. In the
on Pediatric Oncology Nursing in Developing Countries in its basic beginning of the covid pandemic patients had a choice of either digital
standards for safe and effective nursing care recommends assess- or physical visits, but after a few moths it was no choice, all visits were
ing competencies in pediatric oncology patient care from the time of digital. Since clinical investigation was not possible at the digital visit,
recruitment and then training with a formalized induction program. referral to local health care was made when needed. After the visit, a
Objective: To describe the results of professional nursing competen- webbased evaluation during was sent to 139 patients with a response
cies on the care of pediatric oncology patients in evaluation prior to the rate of 47,5 %.
induction course. Results: Patients attending the clinic were 67% digitally, 27% physical
Methods: This was an observational, descriptive, cross-sectional and and 5% a telephone visit. Patients reported the importance of receiving
retrospective research, we analyzed results of 64 employees who information on their previous cancer diagnosis, treatment, and possible
entered in 2019 to 2021. The initial assessment consisted of 35 long-term complications, 80% of the patients reported the clinical visit
multiple-choice items and two additional questions of self-perception fulfilled their needs. Patients having the digital clinic was 73% satisfied
of knowledge and clinical skills; the results were concentrated in Excel and 80% of them would like to attend another digital clinic.
and descriptive statistics are shown. Conclusions: A digital team-based clinic can be an alternative when a
Results: The initial theoretical evaluation of 64 nursing admissions, physical meeting is not possible or when the patient has a long journey.
showed a minimum score of 23, a maximum of 78 and an average of In the post-covid era, digital clinical visit is now offered to patients as
47.2, in education related to pediatric oncology, showed that 3.1% had an option. Acknowledgement - We would like to give special thanks to
a specialty in general oncology, 4.6% had a chemotherapy course, 9.3% all the young adult cancer survivors who participated in the evaluation.
took a related course, and 82% reported not having any of the above.
In self-perception of theoretical and practical knowledge in pediatric
oncology, on a scale where 0 is no knowledge or experience and 5 is E-Poster Topic: AS04 Nursing / AS04.b Research
excellent, 64% were between 0 and 1 in theory, 52% were between 0
and 1 in skill in clinical care. NURSING POSTER SESSION
Conclusions: . The training of nurses in pediatric oncology, a pending
task in university programs, our results confirm the need and impor- P131 / #1109 THE USE OF TRADITIONAL AND
tance of implementing or maintaining the induction program when COMPLEMENTARY MEDICINE AMONG CHILDREN WITH
starting to work in this type of hospital and developing competencies CANCER IN TURKEY: WHAT WE KNOW SO FAR
to ensure the care of this vulnerable group of patients.
Candan Öztürk1 , Murat Bektas2 , Mohammad Alqudimat3
1 Near East University, Faculty Of Nursing, Nicosia TRNC, Cyprus; 2 Dokuz
E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice Eylül University, Faculty Of Nursing, İzmir, Turkey; 3 University of Toronto,
Project Lawrence S. Bloomberg Faculty Of Nursing, Toronto, Canada

NURSING POSTER SESSION Background and Aims: Traditional and Complementary Medicine
(T&CM) is widely used by children with cancer globally. T&CM use
P130 / #567 EXPERIENCES FROM DIGITAL CLINIC AT A varies among ethnic groups because it is generally linked to cultural
LONG-TERM FOLLOW-UP CLINIC FOR ADULTS AFTER background and health beliefs. In Tukey, T&CM use is rooted in the
CHILDHOOD CANCER DURING COVID-19 country’s tradition, and it isn’t only used to relieve symptoms or treat
illnesses. In this study, we reviewed published research articles about
Maria Olsson1 , Marianne Jarfelt1 , Malin Karlsson Damerau2 T&CM use by children with cancer in Turkey to identify T&CM use
1 University of Gothenburg, Department Of Oncology, Institute Of Clin- prevalence rate, used types, and the factors affecting T&CM use.
ical Sciences, Sahlgrenska Academy, Gothenburg, Sweden; 2 Sahlgrenska Methods: ULAKBIM TR, YÖK thesis center, EBSCO, MEDLINE, Else-
University hospital, Onkology, Gothenburg, Sweden vier, ScienceDirect, Psychoinfo, CHINAL, Thomson, and google scholar
were searched. The inclusion criteria were primary studies from Turkey
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ABSTRACTS S557 of S635

involving children with cancer (<18 years) who used any type of T&CM. and contain: a Likert scale-based survey assessing comfort and confi-
Data were extracted by two reviewers. dence; oncologic knowledge-based questions aligned with the survey
Results: Thirteen studies published between 2000 and 2022 were E-Poster Topics; and demographic information. Preliminary data was
included. T&CM use prevalence ranged from 26.6% to 99.4% gathered from April-June 2021. Post-survey data will be compared to
(median=63.5%). All used questionnaires to assess T&CM use preliminary results to analyze the impact of our intervention.
prevalence had no evidence of validity. The most common T&CM Results: Preliminary data found that as residents began their oncology
method was nutritional approaches and herbs (range=44.2%-97%, rotation, they lacked confidence overseeing oncologic emergencies,
median=90.7%), including carob, mulberry, grape molasses, honey, managing chemotherapy and immunotherapy side effects, and provid-
and stinging nettle. The most common reasons for the use were ing supportive care. The education program began in July 2021 and
treating cancer symptoms (range=4.5%-96.8%, median= 58.8%) and will continue through June 2022. 35 physicians have participated to
reducing the side effects of cancer treatments (range=5.2%-90.7, date. Early findings reveal an improvement in residents’ confidence,
median=22.1%). Mostly, patients’ relatives recommended using satisfaction, and knowledge in comfort managing oncologic emergen-
T&CM (range=18%-79.1%, median=%58.8). Most patients/parents do cies (18% increase from baseline) and providing supportive care (28%
not disclose T&CM use to the health care team (range=25%-67.8%, increase from baseline). The complete data analysis will be available for
median=62.4%). presentation at the Congress.
Conclusions: T&CM use is common among children with cancer in Conclusions: A NP-led clinical education program has shown to be ben-
Turkey. The reported T&CM use prevalence by children with cancer eficial in enhancing residents’ confidence and knowledge in caring for
in Turkey varies widely. This could be explained by the inconsistent oncology patients. Residents are empowered with more knowledge,
methodology used in T&CM studies, and the lack of standardized and skills, and support, which may lead to improved patient outcomes for
validated T&CM questionnaires. Also, the ambiguity in the T&CM defi- pediatric cancer patients. This program proved feasible to implement
nition poses challenges when comparing the results across studies (e.g., and could be replicated in other sub-specialty areas.
some studies included surveys asking about multiple T&CM types while
others had a limited number).
E-Poster Topic: AS04 Nursing / AS04.a Education

E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice NURSING POSTER SESSION
Project
P133 / #143 INFORMATION PROVISION TO SUPPORT
NURSING POSTER SESSION PARENT DECISION-MAKING IN HIGH-RISK NEUROBLASTOMA:
THE USE OF ANIMATION AS A METHOD OF INFORMATION
P132 / #223 THE BENEFITS OF IMPLEMENTING A NURSE GIVING
PRACTITIONER-LED ONCOLOGY EDUCATION PROGRAM FOR
PEDIATRIC RESIDENT PHYSICIANS: THE CHILDREN’S HOSPITAL Helen Pearson1 , Katie Johnson2 , Rebecca Parnham3 , Sarah
LOS ANGELES EXPERIENCE Schnellman-Smith4 , Heather Young5 , Naomi Shefford-Thomas6
1 The Royal Marsden NHS Foundation Trust, The Oak Centre For Children
Karishma Patel1 , Megan Bletzacker2 , Susan Cienfuegos-Reid1 , Jill And Young People, Surrey, United Kingdom; 2 University Hospital Southamp-
Anderson1 , Jamie Stokke1 ton NHS Foundation Trust, Paediatric Clinical Trials, Hampshire, United
1 Children’s Hospital Los Angeles, Cancer And Blood Disease Institute, Los Kingdom; 3 Sheffield Children’s NHS Foundation Trust, Paediatrics, Sheffield,
Angeles, United States of America; 2 Primary Children’s Hospital, Center For United Kingdom; 4 The Royal Marsden NHS Foundation Trust, The Oak Cen-
Cancer And Blood Disorders, Salt Lake City, United States of America tre For Children And Young People, Sutton, United Kingdom; 5 Cardiff and
Vale University Health Board, Paediatric Oncology/haematology, Cardiff,
Background and Aims: Pediatric resident physicians (‘residents’) United Kingdom; 6 Parent, Parent, Cardiff, United Kingdom
often feel unprepared for the complexities and unique challenges
of oncology patient care. During their oncology rotation, residents Background and Aims: Background and aims The high-risk neuroblas-
manage care for acutely ill and newly diagnosed oncology patients, fre- toma clinical trial contains randomisations for induction, consolidation,
quently doing so without prior exposure or comprehensive oncologic- and radiotherapy treatment. At diagnosis, parents are given detailed
specific medical education. The aim of our study is to assess residents’ information to make an informed decision in relation to the clinical trial.
comfort and knowledge prior to their oncology rotation and design a Each stage/component of randomised treatment involves further deci-
nurse practitioner (NP) led educational intervention to address gaps. sion making and consent processes for families. Adaptive and engaging
Methods: The NP education program consists of a comprehensive information is needed to help parents understand the complexities
didactic lecture on the first day of each resident rotation, a pocket- and support their decision making for this clinical trial. Health liter-
sized information card, in-depth resource binders, and daily check-ins. acy of individuals in information provision is paramount. Information
Questionnaires are distributed before and after the residents’ rotation should be provided in a variety of ways to maximise understanding
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S558 of S635 ABSTRACTS

and engagement particularly in decision-making (Sheridan et al, 2011). Methods: This cross-sectional study used a study-specific question-
Visual, verbal, and written information is the ‘gold standard’ in the pro- naire to explore perceptions of nurses who had completed the national
vision of information (Sheridan et al, 2011). The aim was to develop educational program. All participants in three cohorts (2012-2014,
animation videos providing concise information on the high-risk neu- 2015-2017, 2017-2019) were invited to participate (30 March-17 May
roblastoma clinical trial. Animated information would complement the 2021). An electronic study-specific questionnaire with response alter-
verbal and written information given to parents providing an additional natives yes and no, or a 4-point Likert-type scale (Not at all, to a small
layer of information provision to support health literacy, understanding extent, to a fairly large extent, to a large extent), was used. Analysis
and decision-making. included descriptive statistics and associations.
Methods: The National Neuroblastoma Nursing Group co-produced Results: Eighty nurses were invited to participate and 59 (74%)
with parents a script of the different phases of treatment within the responded. At the time of the survey, 15 (25%) had left pediatric
high-risk neuroblastoma clinical trial. Animation was created to sup- oncology. Among the remaining 44, 31 nurses (71 %) were working
port the script based on these treatment phases in collaboration with bedside, of which 13 (42%) combined it with a special position. The
a creative design company. Ethical approval was received. number of nurses with special positions (manager, consultant-, contact-
Results: Eight animated videos with screen captions were created , coordinating- or research nurse) increased, after the education, from 9
providing information on: introduction to high-risk neuroblastoma, (20%) to 26 (59%). The vast majority (89%) stated that the knowledge
induction chemotherapy, stem cell harvest, surgery, consolidation from the education to a fairly large/large extent, helped them in their
chemotherapy, radiotherapy, maintenance therapy and end of treat- work and contributed to increased confidence in the interaction with
ment. the patient and the family.
Conclusions: Videos will be available online alongside accessible infor- Conclusions: Education has an impact on the career opportunities in
mation on the clinical trial parent/participant information sheets. clinical practice and contributes to nurses’ confidence and professional
Charity social media, professional Twitter accounts will support dis- work with children, adolescents, and their families. A limitation was the
semination within the parent community. Future evaluation of this relatively small sample. To meet the demands of highly specialized pedi-
project will be conducted once the videos have been integrated into atric oncology care, it is essential to provide continued education to
clinical practice. nurses.

E-Poster Topic: AS04 Nursing / AS04.b Research E-Poster Topic: AS04 Nursing / AS04.a Education

NURSING POSTER SESSION NURSING POSTER SESSION

P134 / #379 THE IMPACT OF A CONTINUED EDUCATION P135 / #1228 “BASIC PEDIATRIC ONCOLOGY COURSE MCM
OF NURSES IN PAEDIATRIC ONCOLOGY: A CROSS-SECTIONAL GRANT” FOR NURSES: IMPLEMENTATION CHALLENGES DUE
EVALUATION WITH GRADUATED NURSES TO PANDEMIC

Pernilla Pergert1 , Maria Olsson2 , Torben Ek3 , Karin Enskär4 , Rehana Punjwani1 , Shenila Ali2
Margaretha Stenmarker3,5 , Margareta Af Sandeberg1 1 DOW University of Health Sciences, Nursing Services, Karachi, Pak-
1 Karolinska Institutet, Department Of Women’s And Children’s Health, istan; 2 Aga Khan University, School Of Nursing And Midwifery, Karachi,
Stockholm, Sweden; 2 University of Gothenburg, Department Of Oncology, Pakistan
Institute Of Clinical Sciences, Sahlgrenska Academy, Gothenburg, Sweden;
3 University of Gothenburg, Department Of Paediatrics, Institute Of Clinical Background and Aims: Only 50 % of the 8000 Pakistani children with
Sciences, Sahlgrenska Academy, Gothenburg, Sweden; 4 Uppsala University, cancer are properly diagnosed and treated, and 40% of those who
Department Of Women’s And Children’s Health, Uppsala, Sweden; 5 Region approach to a medical facility comes with advanced disease difficult
Jönköping County, Futurum – Academy Of Health And Care, Department Of to treat. Unfortunately, there are no palliative care services avail-
Paediatrics, Jönköping, Sweden able for the 1600 children who have advanced disease in public or
private health sectors in Pakistan. There is an urgent need for pallia-
Background and Aims: Research has shown an association between tive care, particularly in the training for health workers and improving
nursing education and patient outcomes. Specialization of nurses has poor availability and/or accessibility to palliative care in terms of fac-
been identified as a nursing priority of the WHO Global Initiative for tors such as medication and bereavement support. Delivering palliative
Childhood Cancer. In Sweden, a national educational program (45 and care requires a specialized set of skills from experience and train-
later 30 credits) in pediatric oncology nursing have been provided ing to provide holistic care to the patient and their family. MCM
since 2003. The aim with the evaluation was to gain knowledge of nursing grant was applied for creating and delivering “Basic Pediatric
nurses’ perceptions of the impact of the national educational program Hematology/Oncology Palliative Care Course” in 2019 for the purpose
in pediatric oncology nursing. of training and education of Nurses from all over Pakistan. Primary
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ABSTRACTS S559 of S635

objective for the grant was to provide Pakistani nurses training in healthcare, and checked in on the children and 3) Seeking familiarity
palliative care away from home was facilitated when the environment children found
Methods: Sanofi Espoir My Child Matters Grant was approved in 2020 themselves in provided them their own space and various forms of
and funds were received. Implementation plan included creating a entertainment.
module and conducting workshops in three different cities with goal Conclusions: School-aged children were able to verbalize what their
of training over 200 Nurses which was not possible due to pandemic best interests were and how participation in care could be facilitated
COVID – 19 travel Restrictions and lockdown. The implementation in the hospital setting. The interrelationships of the children with their
plan was changed and improvised, new plan included developing two parents, healthcare professionals, and the immediate environment
videos and a handbook which can be utilized by Health care workers reflected interactions both within, and between systems. Children in
not only In Pakistan but other countries as well. hospital need to be provided with age-appropriate opportunities to
Results: Palliative care resource material was developed with grant participate in shared decision making to support their best interests.
funds which included two Videos and a Pediatric Palliative Care
Handbook.
Conclusions: Pediatric Palliative care educational material for health P137 / #2009 EXPERIENCES OF THE CAREGIVER OF THE
care professional was developed in local language with keeping in mind CANCER CHILD, WITH A PERIPHERALLY INSERTED CENTRAL
resources and cultural aspects. This is now a resource available online CATHETER (PICC), DURING THEIR TREATMENT AT THE
for all healthcare workers all over the world to be used in all training TELETON ONCOLOGY CHILDREN’S HOSPITAL (HITO)

Ma. De Loudes Ramos Cárdenas1 , Ma Matilde Nuñez Martinez2 ,


E-Poster Topic: AS04 Nursing / AS04.b Research Maria Del Carmen Esmer Sánchez3
1 Hospital Infantil Teletón de Oncología, Nursing, Querétaro, Mexico;
NURSING POSTER SESSION 2 HOSPITAL INFANTIL TELETÓN DE ONCOLOGÍA, EnseÑanza De Enfer-
merÍa, QUERETARO QUERETARO, Mexico; 3 Hospital Infantil Teleton de
P136 / #1342 CHILDREN’S VOICES ON THEIR Oncologia, Research, Queretaro, Mexico
PARTICIPATION AND BEST INTERESTS DURING A HOSPITAL
STAY IN AUSTRALIA Background and Aims: Background: Cancer is the disease with the
highest prevalence worldwide, affects children and adults, the PICC
Mandie Foster1 , Angela Quaye2 , Lisa Whitehead3 , Inger Hallström4 catheter is used for the oncological treatment of children with cancer,
1 Auckland University of Technology, School Of Clinical Sciences, East Auck- care is important to preserve it properly, training the caregiver and the
land City, New Zealand; 2 Lund University, Department Of Health Sciences, health personnel who handle it, avoiding complications or untimely loss
Faculty Of Medicine, Lund, Sweden; 3 Edith Cowan University, School Of of the catheter. Hence the importance of sharing the caregiver’s expe-
Nursing And Midwifery, Perth, Australia; 4 Lund University., Department Of riences and serving as a reference in the use and care of the PICC for
Health Sciences, Faculty Of Medicine, Lund, Sweden the benefit of children with cancer. Objective: To describe the experi-
ences of the caregiver, in the use and care of the PICC, for oncological
Background and Aims: The concepts inherent in defining the best treatment in pediatrics.
interests of a child come from Article 3 of the United Nations Conven- Methods: Material and Methods: It is a descriptive, cross-sectional
tion on the Rights of the Child (UNCRC). Listening to children’s voices investigation, a self-applicable questionnaire was used through a social
is vital to install trust, foster respect, autonomy, self-determination, network platform (WhatsApp) to caregivers of patients with PICC, the
as well as honour social justice and equity However, there is a lack of analysis was carried out through a sampling of finite populations where
information on how children’s best interests are upheld and expressed the margin is 5% and confidence level of 95% the sample size was 49
in hospitals globally. This study aims to explore school-aged children’s caregivers of children. Using descriptive statistics with the support of
experiences about their best interests and participation in care during Excel, the permission to carry out this study was authorized by HITO
a hospital admission. management staff and caregivers.
Methods: A descriptive qualitative design involving in-depth, iterative Results: 49 surveys were conducted, 100% of the sample to caregivers
inductive analysis of child’s interviews. The study was guided by the of children with PICC, 90% mothers and 10% fathers. The longest
UNCRC’s definition of the best interests of the child, Bronfenbrenner’s stay of the catheter was 16 months, the average 6.9 months and the
bioecological model and a child centred care approach. shortest 1 month. The medical diagnosis of the patients was: leukemia
Results: Nine school-aged children (5–15 years old) from one children’s 45.2%, solid tumors 40.6% and post-transplant treatment 14.2%. The
ward in Australia participated. Analysis yielded thirteen categories, reported experiences were: 38% tolerable without discomfort, 31%
six sub-themes, and three themes: 1) Relationships with parents were uncomfortable at first, 28% no discomfort, 2% reported discomfort.
positive when they met their children’s physical and emotional needs Conclusions: The experiences reported by the caregivers
and advocated for them; 2) Relationships with staff were positive were less suffering, they avoided multi-tasking, responsibil-
when staff created opportunities for children to have a say in their ity, courage and affection, with some patients referring to the
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S560 of S635 ABSTRACTS

catheter as: "My tummy, Baby Piccito, Little Pet, Emily and Hose- 1 Prinses Maxima Center for Pedatric Oncology, Sports And Exercise Cen-
legs". ter, Utrecht, Netherlands; 2 Princess Maxima Center for Pediatric Oncology,
Supportive Care, Utrecht, Netherlands; 3 Dutch Childhood Cancer Associa-
tion, Vkn, Utrecht, Netherlands; 4 UMCU, Pediatric Rehabilitation, Utrecht,
P138 / #450 IMPACT OF RESILIENCE AND SOCIAL SUPPORT Netherlands
ON LONG-TERM GRIEF IN CANCER-BEREAVED SIBLINGS: AN
EXPLORATORY STUDY Background and Aims: Since 2018 medical care for children with
cancer in the Netherlands is concentrated in one dedicated Princess
Omid Rasouli1 , Unni Karin Moksnes1 , Trude Reinfjell2 , Odin Hjemdal2 , Máxima Center for pediatric oncology. Stakeholder analysis reveals
Mary-Elizabeth Eilertsen3 that strengthening and continuity in care by allied health professionals
1 Norwegian University of Science and Technology, Department Of Pub- is essential for optimizing care, participation and quality of life.
lic Health And Nursing, Trondheim, Norway; 2 Norwegian University of Methods: Using a mixed methods approach, with quantitative (sur-
Science and Technology, Department Of Psychology, Trondheim, Norway; vey) and qualitative co-creation sessions we determined and identified
3 Norwegian University of Science and Technology, Public Health And wishes and needs among parents of children with cancer and pediatric
Nursing, Trondheim, Norway physiotherapists with respect to continuity of care close to home.
Results: A survey among 98 parents of children with cancer and 176
Background and Aims: Background: Bereavement research has local physiotherapists showed that 96% of parents think it is important
mainly explored potential risk factors associated with adverse out- that the local therapist is aware of the pediatric oncological condition,
comes, and the role of protective factors has received less attention. the side effects and late effects of treatment affecting exercise, and
More knowledge is needed about factors related to unresolved grief that 93% of therapists are enthusiastic about developing a national
in bereaved siblings. This study aimed to assess grief adjustment and network for collaboration (KinderOncoNet). Moreover, 40% of physio-
possible gender differences among bereaved young adults 2–10 years therapists think that they do not have sufficient knowledge to be able
after losing a brother or sister to cancer. We also sought to explore how to give a high-quality treatment, and that they lack opportunities for
resilience and social support influenced their grief. education to gain more knowledge in the field of pediatric oncology in
Methods: A total of 99 young adults (18-26 years) who had lost a relation to physiotherapy care.
brother or sister to cancer between the years 2009 and 2014 were Conclusions: Parents and therapists indicated that a national, multi-
invited to participate in this Norwegian nationwide study. The study- disciplinary care network specialized in pediatric oncology involving
specific questionnaire was completed by 36 participants (36.4%). Social children/parents/survivors and local physiotherapists (and other allied
support during the sibling’s illness, after the death, and during the past health professionals), in which they entrust each other in providing
year, in addition to grief and resilience were measured. care, in which knowledge is available at the right time and place and
Results: Overall, the prevalence of unresolved grief was 47.2 % among build capacity in pediatric oncological care, would improve the acces-
bereaved siblings, whereas 52.8 % had worked through their grief. sibility and continuity of optimal care, participation and quality of
The level of having worked through grief and resilience was simi- life.
lar between male and female siblings. Bereaved siblings with higher
Personal Competence reported lower unresolved grief.
Conclusions: Approximately half of the young adults experience unre- E-Poster Topic: AS04 Nursing / AS04.b Research
solved grief 2-10 years after losing a sibling to cancer. The findings also
highlight the need for long-term support for bereaved siblings to help NURSING POSTER SESSION
improve their resilience and better have worked through their grief.
P140 / #494 AMBIGUOUS EXPECTATIONS OF PARENT
CAREGIVING FOR THE CHILD AND ADOLESCENT WITH
E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice CANCER AT HOSPITAL AND AT HOME – AN ETHNOGRAPHIC
Project STUDY

NURSING POSTER SESSION Louise Roug1 , Ayo Wahlberg2 , Mary Jarden3 , Lisa Hjalgrim1 , Helena
Hansson1
P139 / #381 DEVELOPMENT OF A NATIONAL NETWORK OF 1 University of Copenhagen and University Hospital Copenhagen, Rigshos-
ALLIED HEALTH PROFESSIONALS WORKING WITH CHILDREN pitalet, Department Of Paediatric And Adolescent Medicine, Copenhagen
WITH CANCER TO IMPROVE PARTICIPATION AND QUALITY OE, Denmark; 2 University of Copenhagen, Department Of Anthropology,
OF LIFE (KINDERONCONET) Copenhagen K, Denmark; 3 University Hospital Copenhagen, Rigshospi-
talet, Department Of Hematology, Center For Cancer And Organ Disease,
Lineke Rehorst-Kleinlugtenbelt1 , Wim Tissing2 , Willemijn Plieger- Van København Ø, Denmark
Solkema3 , Martin Beuzel4 , Patrick Van Der Torre1
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ABSTRACTS S561 of S635

Background and Aims: Over the past three decades, complex care Methods: The study was conducted at the Pediatric Oncology and
and treatment has increasingly become the responsibility of parents Hematology Department at Copenhagen University Hospital. This is
as home-based care providers; yet little is known about parents’ care- the second study of the research project INTravenous AntiCancer
giving experiences when considering the variety of care tasks. It is Treatment for children and adolescents at Home (INTACTatHome).
imperative to gain insight into the challenges that parents face when The intervention was developed in a framework of actions for inter-
managing treatment and care of their child with cancer to ensure vention development within the National Institute of Health Research
optimal parent support and prior to further expansion of home-based and UK Medical Research Councils guidelines for developing com-
parent caregiving. The aim of this study was to explore the experiences plex interventions in health care. The development was structured
of children and adolescents with cancer and their parents in manag- in three phases: 1) two workshops with multidisciplinary health
ing different care tasks. It is the first study of the research project professionals (n=12 and n=13) based on the nominal group tech-
’INTravenous AntiCancer Treatment for children and adolescents at nique method and focus groups to identify eligible chemotherapy
Home (INTACTatHome)’, that aims to develop and test an intervention treatments for parent-led home administration, 2) interviews with
of home-based intravenous anti-cancer treatment. parents of children with cancer with experiences of intravenous
Methods: An ethnographic fieldwork comprising participant observa- antibiotics at home, on their opinions of chemotherapy administra-
tion and semi-structured interviewing was conducted from July 2020 tion (n= 9), and 3) pilot test of the developed intervention on three
to December 2020 at the hospital and in the homes of the families. families.
A purposeful maximum variation sampling strategy was applied, and Results: showed that low dose bolus cytarabine was the most eligible
13 families participated in the fieldwork (13 children and adolescents chemotherapy for parent-led home administration. The administration
and 15 parents). Teen of these families were interviewed (five children procedure, and a parent-education program were developed. The pilot
and adolescents and 16 parents). Data was analyzed using qualitative test showed that the parent-led administration procedure was feasible,
thematic analysis. the parent-education program was manageable and corresponded to
Results: Three main themes were identified: 1) Being a “mini-nurse”; parents’ needs, and pilot families were satisfied and preferred home-
2) Dividing care; and 3) Managing anxiety and fear, each based on sepa- based to hospital-based chemotherapy.
rate sub-themes. These themes were bound together by an overarching Conclusions: This development study informs the next phase of the
theme: ‘Ambiguous expectations of parent caregiving’. research project that aims to test the home chemotherapy intervention
Conclusions: This study contributes to a deeper understanding of the in a prospective single-arm intervention study to evaluate feasibil-
varying experiences of parents in managing different care tasks for ity, process, and outcome measures relevant to future larger scale
a child or adolescent with cancer. It underscores the need to estab- evaluation and implementation.
lish clear expectations for parents as caregivers throughout the cancer
treatment trajectory. This perspective is crucial when developing and
implementing future home-based care services. E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
Project

P141 / #1368 DEVELOPING A COMPLEX INTERVENTION OF NURSING POSTER SESSION


INTRAVENOUS ANTICANCER TREATMENT FOR CHILDREN AND
ADOLESCENTS AT HOME P142 / #435 NURSING ROLE IN THE HEMATOPOIETIC STEM
CELL AND BONE MARROW TRANSPLANTATION UNIT IN
Louise Roug1 , Rikke Michelsen1 , Martha Topperzer1 , Mary Jarden2 , RABAT, MOROCCO
Ayo Wahlberg3 , Lisa Hjalgrim1 , Helena Hansson1
1 University of Copenhagen and University Hospital Copenhagen, Rigshos- Rabia Elothemany1 , Younes Labrini2 , Malika Meskini2 , Meriem
pitalet, Department Of Paediatric And Adolescent Medicine, Copenhagen Lakhrissi2 , Maria El Kababri2
OE, Denmark; 2 University Hospital Copenhagen, Rigshospitalet, Depart- 1 CHU AVICENNE RABAT, Hemato-oncolgy Pediatric Department, Rabat,
ment Of Hematology, Center For Cancer And Organ Disease, Copenhagen Morocco; 2 Ibn Sina University Hospital, Pediatric Oncology Department,
OE, Denmark; 3 University of Copenhagen, Department Of Anthropology, RABAT, Morocco
Copenhagen K, Denmark
Background and Aims: Increase number of allografts to 10 in 2022,
Background and Aims: Studies show that parents of children with and elucidate the nursing role for success. This information can also
cancer are willing to provide a variety of home care tasks to avoid serve as a training tool for nurses in the pediatric hemato-oncology
hospitalization. However, intravenous chemotherapy given at home department SHOP Rabat.
is a complex task that impacts patients, caregivers, and health Methods: Data collection performed by participatory observation dur-
professionals. The aim of this study was to develop a feasible ing my nursing work in transplant from October 2020 until March
and safe home chemotherapy intervention for children targeted to 2022, 24-hour shift, and review of transplant files and statistics of the
parents. transplant unit.
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S562 of S635 ABSTRACTS

Results: Over 18 months, 17 autografts and 5 allografts were 3) Need assessed by the family navigator for assistance at any time
completed. Nursing role by transplant phase presented here. Pre- during the treatment, and 4) Relapse of disease. All families to chil-
transplant: perform complete patient biological assessments, HLA dren and adolescents diagnosed with cancer are offered structured
typing for the donor and recipient for allograft, mobilization of stem dialogues identifying areas for assistance throughout the treatment
cells for autograft. Transplant hospitalization: welcome and educate trajectory. The structure entails every-day challenges, concerns, sup-
the child and caregiver on hygiene and transplant procedures. Admin- port, and treatment. Three family navigators share between them the
ister myelo-ablative and non myelo-ablative conditioning treatment. patients according to their diagnosis to ensure individually adapted
Reinject of stem cells or bone marrow. Immediately post-transplant: treatment, care, and rehabilitation, in collaboration with the families.
prevent/treat complications of aplasia and early toxicity of condi- Conclusions: The family navigators work systematically to ensure
tioning. Recovery from aplasia: prevent graph versus host disease in family-adapted nurse navigation to assist the families navigating
allograft patients. Exit from hospitalization: prepare child for discharge the complex cancer treatment and reduce the families experience
and educate child/family for home therapeutic care: diet, hygiene, of psychological distress. An evaluation of families’ experience and
leisure, and school activities. perception of distress is needed, as well as evaluation of the
Conclusions: The nursing role in the pediatric transplant unit is impor- function.
tant to prevent and treat transplant complications and to succeed in
our department’s project of achieving 10 successful allografts per year.
Sharing this knowledge will enable nurses working in settings in other E-Poster Topic: AS04 Nursing / AS04.a Education
limited resource settings to understand the nursing role and scope of
practice in a lower-middle-income country transplant unit to ensure a NURSING POSTER SESSION
good outcome for the child and family.
P144 / #1145 INFANT ACUTE LYMPHOBLASTIC LEUKEMIA:
RECENT ADVANCES IN RESEARCH AND A REVIEW OF
P143 / #668 DEVELOPMENT OF A FAMILY NAVIGATOR STANDARD THERAPY
FUNCTION TO ASSIST FAMILIES NAVIGATING THE COMPLEX
CANCER TREATMENT Danielle Fried1 , Renee Stretchberry2
1 Seattle Children’s Hospital, Cancer And Blood Disorders, Seattle, United
Pernille Sørensen1 , Ragnhild Lilleeidet1 , Trine Rosenholm1 , Pernille States of America; 2 Seattle Children’s Hospital, Cancer And Blood Disorders
Roland1 , Gitte Petersen2 , Martha Topperzer1 Center, Seattle, United States of America
1 University of Copenhagen Rigshospitalet, Department Of Child And Ado-
lescent Cancer, Copenhagen, Denmark; 2 University hospital of Copenhagen Background and Aims: Infant acute lymphoblastic leukemia (ALL) is
Rigshospitalet, Department Of Child And Adolescent Cancer, Copenhagen, a rare, aggressive and distinct subset of pediatric leukemia. Histori-
Denmark cal 6-year event-free survival was previously 46% for patients with
infant ALL. Infant ALL comprises about 4% of all cases of pediatric ALL,
Background and Aims: Multiple challenges arise in a family when a therefore advances in treatment of infant ALL require international
child or an adolescent is diagnosed with cancer, concerning mental, collaboration. The current standard treatment backbone was devel-
social, and physical development. The parents are often burdened by oped from Interfant-99, which was the first large-scale international
numerous care related tasks, in addition to maintaining their work or clinical trial for infant ALL. This presentation aims to summarize cur-
education. Additionally, siblings may suffer from their parent’s pre- rent standard therapy for infant ALL, including recent advances, as well
occupation of the sick child. As part of a national cancer strategy as highlight upcoming clinical trials.
optimising patients’ experience of coherent treatment trajectories, Methods: Performed literature review of large, international, multi-
management at the children’s cancer ward in Rigshospitalet, Copen- center trials for treatment of infant ALL. Reviewed treatment proto-
hagen, Denmark, encouraged the development of a function to support cols and outcomes for Interfant-99, Interfant-06, and a recent phase
the families navigating the complex cancer treatment, facilitate access 2 study using blinatumomab (Van Der Sluis et al, 2021) in addition to
to services, and reduce the families experience of psychological dis- upcoming international clinical trials (Interfant-21 and AALL2122).
tress. Results: The current standard treatment backbone was developed
Methods: Through an iterative process of three months, we conducted from Interfant-99, which was the first large-scale international clinical
fieldwork consisting of several interprofessional meetings with medi- trial for infant ALL. The last large international study for infant ALL was
cal and nursing specialists, and management to describe and map the Interfant-06. The primary aim of this study was to identify if a myeloid-
family navigators’ functions and need for family navigation. style consolidation chemotherapy regimen was superior to lymphoid
Results: We identified four types of needs for family navigation: 1) style for patients with KMT2A rearrangement. Unfortunately, out-
Families with particular attention needs (i.e. single parents, families comes did not differ significantly between the lymphoid and myeloid
with social challenges, or families with competing illness’), 2)Need iden- groups. However, recent clinical trial data by Van Der Sluis et al. (2021)
tified by one or more healthcare professionals within the first months, has shown dramatic improvement in event-free survival for this patient
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ABSTRACTS S563 of S635

population with the addition of blinatumomab immunotherapy with a E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice
one-year event-free survival of 96.2%. Project
Conclusions: After advances in treatment had been generally stag-
nant over the past decade, incorporating targeted therapy with NURSING POSTER SESSION
blinatumomab immunotherapy has shown staggering improvement
in patient outcomes. This has influenced upcoming international P146 / #885 CHALLENGES EXPERIENCED BY NURSES ON
clinical trials for infant ALL (AALL2122 and Interfant-21) to HAND-WASHING WHILE NURSING CHILDREN WITH CANCER
include blinatumomab as part of standard therapy in addition to IN GANTSI PRIMARY HOSPITAL, BOTSWANA
other novel approaches to treatment for patients with KMT2A-
rearrangement. Himeroro Tjinyeka
Gantsi primary hospital, Outpatient Deartment, P O BOX, Botswana

P145 / #869 COMPETENCE AND ATTITUDE OF NURSES ON Background and Aims: Background: Hand hygiene remains the cor-
PAIN MANAGEMENT AMONG CHILDREN WITH CANCER IN nerstone of infection prevention but often an overlooked aspect in
GANTSI PRIMARY HOSPITAL, BOTSWANA infection control. Children with cancers are already immune com-
promised hence hand washing should be practiced effectively and
Himeroro Tjinyeka consistently inorder to reduce the morbidity and mortality of chil-
Gantsi primary hospital, Outpatient Deartment, P O BOX, Botswana dren with cancers from nosocomial infections. WHO have introduced
the Hand washing guidelines even though lower- and middle-income
Background and Aims: BACKGROUND: Pain is among the most countries like Botswana, still experience challenges with infection pre-
common effects of cancer and this have been undertreated and under- vention and control measures like hand washing. Aim: To describe the
recognized. WHO have advocated that children with cancer receive challenges related to hand hygiene experienced by nurses working with
pain management based on the WHO analgesics ladder. However, children with cancer.
poor pain management remains a common problem in Botswana. Methods: A qualitative study in which convenience sampling was used
The goal of pain management is to reduce pain, distress and anxiety, to select 12 nurses from the pediatric ward who completed a self-
and nurses are the key player in pediatric cancer pain manage- administered questionnaire. Thematic data analysis was used for data
ment and therefore is important to identify nurses competency on analysis.
pain management. AIM: To generate baseline data on nurses compe- Results: Themes emerged from participant responses and perceived
tency and attitude in pediatric pain management among children with challenges to hand washing included the following themes: unavail-
cancer. ability of supplies (especially soap and water, lack of time to do hand
Methods: A descriptive design using qualitative methods was used washing due to workload, cultural resistance to hygiene practices from
to conduct the study. The convenience sample consisted of 20 parents. Nurses also stated that hand washing have been replaced by
nurses in Gantsi Primary Hospital who completed a questionnaire hand sanitizing during the COVID-19 era. Nurses also stated that poor
designed in English. Data were analyzed using thematic content infrastructures with no accessibility to sinks and hand washing equip-
analysis. ment, which makes hand washing an inconvenient nurses working with
Results: Nurses stated that they rely on parents to report childre"s pain cancer children.
since it is subjective. They demostrate moderate knowledge on pain Conclusions: The research concluded that the main barrier fo hand-
management but dont follow the WHO pain ladder when managing washing was lack of supplliers and workload, therefore strategies to
pain due to no knowledge about the kadder. They had less knowledge improve infection prevention control should focus mainly on the pro-
on pain assessment methods among children but had a positive attitude vision of suppliers such as soap, water, sanitazing.In summary, our
towards pain managemnt in children and adolescent. They reported study shows that interventions and guidelines aimed at improved hand
reluctance to give opiods due their side effects such as constiption, hygiene in oncology pediatric wards shold be supported by nursing
respiratory failure and fear of adiction. Nurses had good knowledge managers to have shared effeorts and results.
on non pharmacological management of pain, they stated to be using
distraction most. Inadequate pain management training and guidelines
for nurses was reported as the main challenge among children with E-Poster Topic: AS04 Nursing / AS04.b Research
cancer.
Conclusions: Children with cancer should be pain free hence pain NURSING POSTER SESSION
management is a prioritized. however, pain management in chil-
dren with cancer is still a challenge for nurses due to lack of P147 / #162 RELIABILITY AND VALIDITY OF PROXY-SSPEDI
in-service trainings. A few negative attitudes were still preva- AND MINI-SSPEDI IN PEDIATRIC PATIENTS 2-7 YEARS
lent arising from the side effects of opioids hence reluctant to RECEIVING CANCER TREATMENTS
give.
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S564 of S635 ABSTRACTS

Deborah Tomlinson1 , Lee Dupuis2 , Donna Johnston3 , Susan Deborah Tomlinson1 , Lauren Chakkalackal2 , Maryann Calligan2 ,
Kuczynski4 , Serina Patel5 , Tal Schechter6 , Emily Vettese1 , Mark Cassandra Tardif-Theriault2 , Susan Kuczynski3 , Tal Schechter4 , Emily
Mairs1 , Lillian Sung1 Vettese1 , Lee Dupuis5 , Lillian Sung1
1 Hospital for Sick Children, Child Health Evaluative Sciences, Toronto, 1 Hospital for Sick Children, Child Health Evaluative Sciences, Toronto,
Canada; 2 The Hospital for Sick Children, Department Of Pharmacy, Toronto, Canada; 2 The Hospital for Sick Children, Child Health Evaluative Sciences,
Canada; 3 Children’s Hospital of Eastern Ontario, Division Of Hematol- Toronto, Canada; 3 Pediatric Oncology Group of Ontario, Opacc, Toronto,
ogy/oncology, Ottawa, Canada; 4 Pediatric Oncology Group of Ontario, Canada; 4 Hospital for Sick Children, Division Of Haematology/oncology,
Opacc, Toronto, Canada; 5 London Health Sciences Centre, Department Toronto, Canada; 5 The Hospital for Sick Children, Department Of Pharmacy,
Of Pediatrics, London, Canada; 6 Hospital for Sick Children, Division Of Toronto, Canada
Haematology/oncology, Toronto, Canada
Background and Aims: To enable pediatric patient symptom self-
Background and Aims: Symptom Screening in Pediatrics Tool (SSPedi) report, we previously created Symptom Screening in Pediatrics Tool
and proxy SSPedi were developed for symptom screening by children (SSPedi). However, SSPedi does not address the needs of those who
8-18 years old. An important gap was that SSPedi did not address cannot or will not self-report symptoms including some younger
the needs of younger children. The objectives were to evaluate the children. Therefore, we created proxy-SSPedi, which is aimed at pedi-
reliability and validity of proxy-SSPedi (2-7 years) and self-report atric patients receiving cancer treatments who are 2-18 years of
mini-SSPedi (4-7 years). age.
Methods: This multi-center study enrolled guardians of children 2-7 Understanding healthcare professional symptom documentation and
years receiving cancer treatments (proxy-SSPedi) and their children if intervention provision may provide insight into why symptoms may be
they were 4-7 years (mini-SSPedi). The two populations were: (1) More poorly controlled.
symptomatic group where children were receiving active cancer treat- Objectives were to describe symptom documentation and intervention
ment and were in hospital or clinic for four consecutive days; and (2) provision for children 2-7 years of age receiving cancer treatments.
Less symptomatic group where children were receiving maintenance Secondary objectives were to determine the relationship between
therapy for acute lymphoblastic leukemia or had completed cancer symptom documentation and intervention provision with increasing
therapy. Proxy-SSPedi or mini-SSPedi were completed with measures severity of bothersome symptoms as identified by guardian proxy-
of mucositis, nausea, pain, quality of life and overall symptoms. Respon- SSPedi.
dents in the more symptomatic group repeated proxy-SSPedi/mini- Methods: We included guardians of children 2-7 years of age receiving
SSPedi and a global symptom change scale at the second assessment. cancer treatments and seen in hospital or clinic daily for four con-
Results: There were 402 guardians and 326 children included in secutive days. Guardians reported proxy-SSPedi at study entry and
the analysis. Test re-test reliability of proxy-SSPedi showed intra- three days later. Chart review was performed between the day prior
class correlation coefficient (ICC) 0.83 (95% confidence interval (CI) and the day following proxy-SSPedi completion. Symptom documenta-
0.72-0.90). Mean difference in proxy-SSPedi between more and less tion and intervention provision were determined by two independent
symptomatic groups was 9.7, 95% CI 8.3-11.1. Proxy-SSPedi was abstractors.
responsive to change and hypothesized relationships between mea- Results: We enrolled 190 guardians who provided 371 proxy-
sures were observed. With a priori threshold ≥ 0.6, inter-rater ICC SSPedi assessments. The most common severely bothersome
among all dyads and those 6-7 years were 0.54 (95% CI 0.45-0.62) and symptoms were “feeling tired”, “feeling more or less hungry than
0.62 (95% CI 0.50-0.71) respectively. Among participating children, you usually do” and “feeling cranky or angry”. Among those with
other hypothesized reliability and validity thresholds were met. increasing severity of bother, documentation was significantly more
Conclusions: We found that proxy-SSPedi (2-7 years) and mini-SSPedi common for 12 symptoms while intervention was significantly
(4-7 years) exhibited test re-test reliability, internal consistency, more common for 7 symptoms. Intervention was not significantly
known groups validity, convergent validity and responsiveness. How- more common with increasing severity of “feeling tired”, “feeling
ever, interrater reliability was established only for children 6 and 7 more or less hungry than you usually do” and “feeling cranky or
years of age. A dyadic child-guardian approach may be promising for angry”.
future research. Conclusions: Symptom documentation was generally more
common among those with increasing severity of symp-
toms. Intervention was not more common among those with
P148 / #457 SYMPTOM DOCUMENTATION AND increasing severity of fatigue, changes in hunger or anger,
INTERVENTION PROVISION RELATED TO PROXY-SSPEDI which were the most common severely bothersome symp-
ASSESSMENTS IN PEDIATRIC PATIENTS 2-7 YEARS RECEIVING toms. Future efforts should focus on increasing intervention
CANCER TREATMENTS provision.
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ABSTRACTS S565 of S635

E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice coordination with child and family are essential. Better knowledge and
Project understanding about illness and treatment increases the autonomy
of child and parents. To accomplish this goal, the aim was to develop
NURSING POSTER SESSION reliable and easily comprehensible information, in co-creation with
children and parents.
P149 / #1095 ASSESSING THE KNOWLEDGE OF NURSES IN Methods: Together with children and parents, we developed a vision
THE PAEDIATRIC ONCOLOGY UNIT AT TAMALE TEACHING on how to create and implement adequate information. It is cru-
HOSPITAL ON FEBRILE NEUTROPENIA AND ITS MANAGEMENT cial that information is understandable, applicable and findable for
child and parents. The use of positive and supportive language is
Peter Tonlaar, Joy Ajibta, Yaninga Fuseini essential. Age specific information as well as inclusiveness in cul-
Tamale Teaching Hospital, Department Of Child Health, Tamale, Ghana tures and languages are taken into account. Ensuring information
to be understandable, this requires visually clear information, devel-
Background and Aims: Febrile neutropenia (FN) can cause significant oped for a young target audience or illiterate people, using rec-
morbidity and mortality in children with cancer if it is not recognised ognizable animations and visual stories. Each type of information
early and treated promptly. The aim of the study was to assess the uniformly uses the same format, recognizable icons and unambiguous
knowledge of nurses working in the paediatric oncology shared care language.
centre at the Tamale Teaching Hospital on the management of febrile Results: We have developed an entirely new child and parent infor-
neutropenia. mation line. This uniform line is fully integrated into all types of
Methods: A descriptive cross-sectional study with a quantitative data information provided for children and parents, e.g. website, patient
collection method was used. All nurses assigned to the paediatric portal, leaflets, consultation support, etc.
oncology unit were recruited. A structured questionnaire was used in Conclusions: A complete and fully uniform way of providing informa-
the data collection. tion has been implemented in the patient journey for child and parents.
Results: A total of 18 nurses were recruited. Majority of the respon- This optimizes decision-making for healthcare professionals in coop-
dents 14/18(77.8%) had worked in the unit for less than 2 years. Most eration with child and parents. This way, they will be better prepared
of them, 15/18(83.3%) did not have formal training in the management for consults, procedures or treatment. Because of this, joint decisions
of febrile neutropenia and did not even know it was an emergency. The made by child, parents and professionals will be more in line with their
majority did not know the cause, and children were at risk of the con- personal situation and needs.
dition. Also, 13/18 (72.2) of respondents did not know the time within
which to initiate antibiotics upon triage of patients and 16/18 (88.9) did
not know the first-line antibiotics to give. E-Poster Topic: AS04 Nursing / AS04.b Research
Conclusions: Most of the respondents had no formal training in the
management of FN. The majority of them could not recognize FN as NURSING POSTER SESSION
an emergency and when to initiate treatment. Knowledge regarding its
management is important among nurses taking care of children with P151 / #1184 “12 YEARS OF LEARNING TO RELIVE
cancer hence in-service training should be organized regularly among EXPERIENCES OF FATHERS AND MOTHERS IN A
nurses. BEREAVEMENT SUPPORT GROUP.”

Paula Vega1 , Natalie Rodriguez2 , Chery Palma2 , Josefina Morales2


P150 / #1064 DEVELOPMENT OF NOVEL CHILD AND 1 pontificia universidad católica de chile, Salud Del Niño Y Adolescente,
PARENT INFORMATION LINE TO INCREASE EMPOWERMENT Nuñoa, Chile; 2 hospital roberto del rio, Oncologia, Santiago, Chile
AND THE FAMILY’S AUTONOMY; ESTABLISHED IN
COOPERATION WITH CHILD AND PARENTS Background and Aims: The death of a child from cancer has a great
impact on the parents, due to the disease process, unresolved suffer-
Nicole Van De Ven ing, and because it is considered a "taboo" subject. Twelve years ago, at
Princess Maxima Centre, Ogz, Utrecht, Netherlands the Roberto del Río Hospital in Santiago de Chile, an interdisciplinary
team formed a self-help group called "Aprendiendo a Revivir", whose
Background and Aims: The Princess Máxima Center for Pediatric objective is "To accompany parents and families who have lost a child to
Oncology has combined innovative care, treatment and state of the art cancer in the mourning process" through two annual meetings. Aims:
research into one center. With each step taken in treatment, we are Understand the experiences of parents who have participated in the
committed to ensure continuation of the child’s regular development, meetings auto self group.
as natural as possible, with maximum quality of life. Empowerment of Methods: Qualitative descriptive research. All parents who attended
child and parents is a key priority. Effective communication as well as the meetings on a voluntary basis were invited to participate. A
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S566 of S635 ABSTRACTS

questionnaire with open-ended questions was used: "What did you find reinforced, work was done on a communication model between teams
most difficult during the meeting?", "What did you like most about the that led to the creation of a leading nurse in charge of receiving med-
meeting?" and "What did participate in these meetings mean to you?". ical indications and transmitting them in an organized manner to the
This was answered by 30 parents. A content analysis was carried out nurses dedicated to airway care, vascular access and medication prepa-
according to Krippendorf. ration. Likewise, the need to create a trolley for seriously ill children
Results: Parents are motivated to participate in the meetings because was detected, locating treatment algorithms available in it, as well as a
of the opportunity to talk about their children and remember them in timer and a record to control medication times.
a safe environment. The most complex thing is attending for the first Conclusions: In situ simulations are a useful instrument to evaluate and
time, because of the uncertainty and lack of knowledge about the meet- improve the competence of professionals in emergency care, reinforc-
ing. What is most difficult for them is to talk about their pain and share ing leadership and teamwork, and also allow to detect weaknesses in
it, contain their emotions, and feel guilty about laughing. They liked oncohaematological emergency care. Carrying out periodic drills is a
having a space for reunion and remembrance, sharing their pain in a tool used to assess the impact of improvement actions.
safe environment, and feeling listened to. For the parents, these meet-
ings have meant: Achieving greater personal and spiritual growth; an
instance of reunion with significant people, and feeling understood. P153 / #194 APPLYING THE INITIATIVE OF MEANINGFUL
Conclusions: For parents, having a meeting with a focus on self-help RECOGNITION TO IMPROVE A HEALTHY WORK
provides them with tools to elaborate their grief, in a protected and safe ENVIRONMENT ON THE HEMATOPOIETIC STEM CELL
environment, together with people who are significant to them. TRANSPLANT UNIT

Julie Waitt, Nicole Noble


E-Poster Topic: AS04 Nursing / AS04.c Quality Improvement/Practice Boston Children’s Hospital, Hematopoietic Stem Cell Transplant Unit,
Project Boston, United States of America

NURSING POSTER SESSION Background and Aims: The work environment is a major focus in
healthcare systems across the country due to burnout rates and
P152 / #990 IMPACT OF "IN SITU" PEDIATRIC work-related stressors healthcare providers experience every day. In
ONCOHEMATOLOGY EMERGENCY SIMULATION: addition, Hematopoietic Stem Cell Transplant (HSCT) is a complex
ORGANIZATIONAL AND CARE IMPROVEMENTS treatment course and often a fast-paced work environment leading
to stress and compassion fatigue. Boston Children’s Hospital (BCH)
Pablo Velasco, Estefanía Cámara, Judith Abelló, Anna Llort, Esther incorporated the American Association of Critical-Care Nurses Six
Diaz, Cristina Diaz, Lucas Moreno Standards of a Healthy Work Environment (HWE). The six components
Vall d’Hebron University Hospital, Pediatric Oncology And Hematology, include skilled communication, true collaboration, effective decision-
Barcelona, Spain making, appropriate staffing, meaningful recognition and authentic
leadership (AACN Website). The HWE committee was established with
Background and Aims: To assess the usefulness of the low-complexity representatives, also known as “Champions” from all areas of BCH
simulation carried out "in situ" to promote the competence of pro- including the HSCT Program.
fessionals in the initial care of pediatric oncohematology emergencies Methods: Starting in 2019, each year, the HWE champions from all
(ICPOE) and to identify aspects for improvement both at the organiza- areas of BCH send a universal survey to selected staff members. To
tional and care levels. date, the HSCT Program has completed four annual surveys, com-
Methods: The material used in the simulation were megacode baby prised of nurses, nurse practitioners, nutritionists, clinical assistants
trainer, junior Danny mannequins, and ALSi simulator. and Patient Experience Administrators.
The minutes of the ICPOE simulations were reviewed. Results: Survey results for 2019, had a completion rate 82%, averaged
Results: From June 2018 to December 2021, 12 ICPOE simulations “excellent”, the lowest scoring category being meaningful recognition,
have been carried out. They were performed "in situ" in a room on the 3.74 on a 5.0 scale. The following two years, the HSCT Unit sur-
hospitalization floor and in the procedure area, with the participation vey response rate was 100% completion with results demonstrating
of the usual care team in both locations (pediatricians, anesthesiol- dramatic improvement in meaningful recognition, illustrating a 14%
ogists, nurses, auxilliaries, ancillaries). They lasted two hours with a increase in satisfaction.
basic scheme of briefing, simulation development and debriefing. The Conclusions: Through focus groups and brainstorming sessions, led by
assessment of the simulations made it possible to verify that the global HSCT nursing leadership the Most Valuable Player (MVP) Program was
method applied and the assistance to the patient’s physiological com- created. Staff, families, and patients can put nominations into a box,
promises were correct. Even so, aspects of improvement could be located at a central location. On the first of the month, one name is
detected, especially related to the coordination and communication drawn randomly to select the MVP. All the other nominations are then
of the healthcare team. To this end, the use of the mental model was read aloud with a group of staff members and typed up quarterly to
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ABSTRACTS S567 of S635

share with staff. When selected for the MVP their picture, name, and 1 Children’s National Hospital, Center For Cancer And Blood Disorders,
comments from the nominees are displayed on a designated board on Washington, United States of America; 2 Children’s National Hospital,
the unit as well as given a gift card. Patient Safety, Washington, DC, United States of America

Background and Aims: The Center for Cancer and Blood Disor-
P154 / #447 REDUCING OUTSIDE LAB MONITORING FOR ders (CCBD) at Children’s National Hospital (CNH) serves pediatric
PEDIATRIC PATIENTS WITH SOLID TUMORS: IMPACT ON patients with hematological, immunological, rheumatologic and onco-
SAFETY, COST, AND QUALITY OF LIFE logical diseases including 1600 patients with sickle cell disease, 300
new cancer diagnosis/year, 60 hematopoietic stem cell transplants/
Catherine Wall, Jill Macdonald, Riley Mahan, Allison O’Neill, Gabrielle year with 1,300 outpatient visits/week and an average inpatient census
Peterson of 28.5. There was no overall structure to coordinate quality improve-
Dana-Farber Cancer Institute, Pediatric Oncology, Boston, United States of ment (QI) projects across disciplines and departments, foster a culture
America of cooperation and develop a metric driven process.
Methods: The CCBD QI Council consists of a multidisciplinary and
Background and Aims: Our institution historically monitored blood- multi-departmental team of physicians, nurses and leaders from phar-
work twice weekly for patients with solid tumors to establish white macy, patient safety, laboratory and environmental services, monitors
blood cell count recovery following receipt of daily filgrastim. This ongoing QI projects, identifies new opportunities to improve safe and
frequency of lab monitoring was heavily taxing on care coordina- timely care delivery and initiates new projects. Projects are required to
tion and required twice weekly central line access. At present, most be rigorous using published QI techniques. Progress is monitored and
patients receive peg-filgrastim and require bloodwork to monitor for displayed on a dashboard available to the entire CCBD community.
transfusion needs rather than count recovery. This quality improve- Results: The council has shown effectiveness through individual
ment project aimed to identify the minimum number of surveillance projects such as advancing chemotherapy start time on the day of
labs required, based upon disease and chemotherapeutic regimen, and admission from 70% to less than 50% being started after 5PM and
to provide guidelines to safely reduce the number of home labs for reducing chemotherapy preparation and delivery time to clinic patient
targeted solid tumor diagnoses by 50% over 2 years. from a baseline of 150 minutes to 105 minutes, a 30% improve-
Methods: We sourced data regarding the number of transfusions per- ment. The CCBD QI Council identifies partners with ideas to improve
formed to correlate whether laboratory work was obtained concurrent care; such as a partnership with anesthesia to decrease anesthesia
with transfusion needs or clinical symptoms, or if labs were obtained times during lumbar punctures. Monthly evaluation of data and strong
unnecessarily. We found that only 24% of transfusions were informed partnerships has led to the identification of trends and opportuni-
by home bloodwork. Based on these findings, we developed new ties for sustained improvement such as reduction of hospital acquired
monitoring guidelines categorized by disease and treatment regimen. viral infections during the COVID pandemic, changes in processes to
We conducted educational sessions and longitudinal chart reviews to deliver timely antibiotics to febrile neutropenic patients, improvement
ensure safety. in timely report of laboratory results and reduction in central line
Results: Post-implementation data analysis resulted in a 77% reduc- associated blood stream infections (CLABSI).
tion in home lab draws, exceeding the 50% goal. There were no adverse Conclusions: A multi-disciplinary, multi-department structured QI
events. Estimated cost savings over two years, accounting for oncol- council can lead to a vigorous safety culture through partnership and
ogy provider follow up, price per lab and visiting nurse sessions, was data analysis.
$70,751.
Conclusions: Our findings indicate that there are a cohort of solid
tumor diagnoses that do not require twice weekly bloodwork to mon- PUBLICATION ONLY ABSTRACTS
itor for transfusion needs. By identifying these patient populations,
formulating, and implementing bloodwork guidelines, we have safely Publication Only Topic: AS01 Surgery - IPSO
reduced the number of labs drawn and, in keeping, optimized resource
utilization, cost, and quality of life. Attention to clinical context remains PO001 / #459 PEDIATRIC GLIOBLASTOMA
warranted as additional labs, outside of these guidelines, may be
required to address clinical status or clinical trial requirements. Fadoua Bouguerra1 , Najla Attia1 , Rym Zanzouri1 , Nadhir Kermani2 ,
Abdelhafidh Slimene2 , Semia Kanoun Belajouza1
1 Farhat Hached Hospital, Radiation Therapy Department, Sousse, Tunisia;
P155 / #1917 DEVELOPMENT OF A MULTI-DISCIPLINARY, 2 Neurology Institute, Neurosurgery Department, Tunis, Tunisia

MULTI-DEPARTMENTAL QUALITY IMPROVEMENT COUNCIL


FOR A LARGE PEDIATRIC ONCOLOGY PROGRAM Background and Aims: Glioblastoma is the most frequent and malig-
nant primary brain tumor. However, its occurrence in the pediatric
Rose Szeles1 , Sopneil Bhattarai2 , Birte Wistinghausen1 population is rare and its prognosis remains poor. The first aim is to
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S568 of S635 ABSTRACTS

elaborate the epidemiological and clinical profile of pediatric glioblas- Results: Post operatively she had uneventful recovery and referred to
toma treated in two services of neurosurgery and radiotherapy in pediatric oncology for more adapted cycles chemotherapy. 2years fol-
Tunisia. The second aim is to study the evolutionary profile in terms of low up without any symptoms and no recurrent tumor on MRI control.
survival and recurrence to release the prognostic factors and to report Conclusions: To obtain oncologic resection in genital RMS, surgery
the recent genetic and therapeutical anatomopathological data of this have to be aggressive. Conservative surgery must be an option when
tumor. it is possible with close survey.
Methods: Our work consists of a retrospective descriptive study of a
series of 22 cases of pediatric glioblastoma operated on in the neu- PO003 / #1934 EMBRYONAL RHABDOMYOSARCOMA OF
rosurgery departments of Sousse and Monastir, during a period of 21 THE UMBILICAL LIGAMENT : CASE REPORT AND REVIEW OF
years from 1997 to 2017. It has been managed in collaboration with THE LITERATURE
the radiotherapy department.
Results: The average age of our patients was 13.22 years with extremes Sarra Chennouf
ranging from 5 to 18 years. The sex ratio was 1.75 with a clear male mother and child hospital constantine algeria, Department Of Pediatric
predominance. The average consultation time was 6.9 weeks with Surgery, mansourah, Algeria
extremes ranging from one week to ten months. The reasons for
consultation varied from one patient to another depending on the loca- Background and Aims: Rhabdomyosarcoma (RMS ) is malignant
tion of the tumor. An exeresis assessed as macroscopically complete, tumor arising from mesenchymal cells, and is the most common soft
according to the surgeon’s impressions, was obtained for 16 patients tissue sarcoma in children, making up 4.5% of all diagnosed childhood
(-72.72% of cases). A genetic study was performed on 6 patients. All cancers . He may arise in numerous anatomic locations such as the head
of our 21 surviving patients developed a recurrence. The average time and neck, trunk, extremities and genitourinary tract We present a case
to recurrence was 19;57 months. Finally, the average survival of our of rhabdomyosarcoma arising from an unusual location
patients was 27.9 months. We found that the quality of exeresis and Methods: Case presentation : We describe a case of a 10 years old male
complementary treatment had an impact on the time to recurrence as with history of constipation and a painless suprapubic mass Tru-Cut
well as on the survival. biopsy guided by ultrasound reported an embryonal rhabdomyosar-
Conclusions: Pediatric glioblastoma has specific features that dif- coma (ERMS) A neoadjuvant chemotherapy was administrated . At
ferentiate it from adult glioblastoma. Our study shows that multi- the definitive surgery 03 cm tumor dependent of the remaining left
disciplinary management improves survival and delays recurrence. umbilical artery,located slightly to the left and displacing the bladder
The improvement of the prognosis lies in the development of gene dome was found . Complete resection of the tumor was performed,
therapy. no pathological lymph nodes was observed. Final pathology showed
an embryonal RMS with signs of reponse to chemotherapy, resection
margins were positive. Chemotherapy was then continued and coadju-
PO002 / #1941 VAGINAL RHABDOMYOSARCOMA IN CHILD vant radiotherapy was administrated. At 24 months of follow-up after
CASE REPORT diagnosis, no residual tumor or relapse has been identified.
Results: Final pathology showed an embryonal RMS with signs
Narimane Cheniki of reponse to chemotherapy, resection margins were positive .
mother and child hospital, Pediatric Surgery, constantine, Algeria Chemotherapy was then continued and coadjuvant radiotherapy was
administrated. At 24 months of follow-up after diagnosis, no residual
Background and Aims: Vaginal rhabdomyosarcoma in child Case tumor or relapse has been identified.
report Authors: Cheniki narimane,Chennouf sara, El gouacem Conclusions: This case demonstrates the successful treatment of a
aida,Choutri hichem,Bensebti amina amel,Atrih zoubir Affiliation: rare ERMS tumor arising in the Umbilical ligament of a child using an
Department of pediatric surgery. Constantine. Algeria Background: interdisciplinary approach.
RMS is the most frequent malignant soft tissue tumor in childhood.
Genitourinary tract is the second site affected after head and neck. PO004 / #839 RENAL TUMORS IN CHILDREN. PROGNOSTIC
Bladder and vagina are more frequently touched with botryoid aspect. FACTORS AND TREATMENT OUTCOMES
Methods: Case presentation: A 2 years old female who presented
vaginal bleeding with polyploid mass protrude through the introitus Zaida De Machaj1 , Karina Quintero2 , Eric Chong3 , Jackson Lio4 , Pablo
noticed by mother. A biopsy from vaginal lesion revealed embry- Lezama Del Valle5
onal RMS botryoid form. CT scan and MRI showed a huge vagi- 1 Hospital del Niño Dr José Renán Esquivel, Pediatric Surgery, Panama,
nal mass(14cm.6,5cm.6cm). No bladder or rectum infiltration. No Panama; 2 Hospital del Niño Dr Jose Renan Esquivel, Oncology, Panama,
adenopathy and no compressive urinary tract effect. Osteomedullar Panama; 3 Hospital del Niño Dr Jose Renán Esquivel, Radiology, Panama,
cytology was negative. She received neoadjuvant chemotherapy which Panama; 4 Instituto Oncologico Nacional, Radiotherapy, Panama, Panama;
reduced its size to 4,6cm.3,4cm she underwent a colpectomy resection 5 Hospital Infantil de Mexico Federico Gomez, Surgery, mexico, Mexico

by abdomino perineal approach and peritoneal neo vagina was created.


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ABSTRACTS S569 of S635

Background and Aims: To describe the clinical characteristics of 2021. Review of medical data and treatment outcomes following the
children with renal tumors (RT) and to assess treatment outcomes multidisciplinary oncologic treatment plan’s implementation.
of multidisciplinary management at the Doctor Jose Renan Esquivel Results: Four patients with SGCT were treated at HDNDRJRE. Each
Children’s Hospital (DJRECH) in Panama was a female. At the time of the presentation were: Two newborns
Methods: Patients with RT were treated at the DJRECH in Panama with Altman’s type I and II; one was seven years old, and one was six-
from March 2018 to February 2022. Review of medical records and teen years old, both were classified as Altman type IV. The teenager
treatment results following implementation of the multidisciplinary who had a lumbosacral spinal lipoma operated on at one month of
oncologic surgical protocol for RT according to Children’s Oncology age, and right renal agenesis, presented with constipation and pelvic
Group. pain. The combined anterior and posterior approach was used to treat
Results: Ten patients with RT were analyzed. Eight girls, ranging in age a significant lesion involving the perineal and presacral region, as
from ten months to nine years with an average age of three. Six patients well as abdominal extension. The pathology result: mature retroperi-
at the time of diagnosis were two years old or less. Six had left RT. toneal teratoma and myelomeningocele presacral. Two years follow up,
Four of the cases required a diagnostic biopsy. All received neoadju- normal anal sphincter control. A 7-year-old female patient was dis-
vant chemotherapy except for a 1-year-old female Stage I with a history covered to have a palpable abdominal tumor during a routine physical
of preterm, cleft palate, tracheostomy, tentorium syndrome, and gas- examination. The tumor was resected using a combined anterior and
trostomy who received upfront left radical nephroureterectomy. Each posterior approach. Wound infection, enuresis, and encopresis were
patient received macroscopic total resection; two got hepatic metas- postoperative complications that resolved spontaneously five months
tasectomy and one vena cava thrombectomy. The Pathologic result after surgery. Neurosurgical examination revealed bilateral patellar
was: 7 nephroblastoma with favorable histology and one unfavorable and achilles areflexia at the three-year follow-up. At 29 days of life, two
histology, one primitive neuroectodermal tumor (PNET) with positive babies were operated on. The approach was posterior, in both cases. All
EWSR1(22q12), and one renal rhabdoid. Postoperative complications: patients received upfront surgery for mature teratoma that included
ascites and intestinal obstruction with volvulus. A central venous total excision of the tumor, occlusion of the median sacral artery, and
port system complication was atrial vegetation, which was removed coccygectomy.
through open thoracotomy. Radiotherapy was administered in 9 cases. Conclusions: Combining SGCT and myelomeningocele is a rare
Five cases are under surveillance. One patient relapsed, and one is congenital disease. A combined anterior and posterior surgical
currently undergoing adjuvant treatment. Three died: A female 9-year- approach with coccygectomy permits complete resection in Altman
old with stage IV with unfavorable histology; a 4-year-old female with type IV.
stage II with three relapses; and a 3-year-old male with PNET.
Conclusions: The female gender and presentation age of ≤ 2 years
were the most prevalent clinical features. Unfavorable histology and PO006 / #22 OUTCOME AND COMPLICATIONS AFTER
advanced stage both have a poor prognosis for survival. Patients with SURGERY FOR THYROID CARCINOMA IN PEDIATRIC AGE – AN
favorable histology and early stages need molecular analysis to rule out EVALUATION OF PRACTICE
a poor prognostic marker.
Ahmed Elgendy1 , Emad Shehata2 , Sherif Shehata3
1 Tanta University, Surgical Oncology Unit, Tanta, Egypt; 2 Tanta Univer-
PO005 / #1153 SACROCOCCYGEAL GERM CELL TUMORS sity, Otolaryngology Department, Tanta, Egypt; 3 Tanta University, Pediatric
(SGCT). CLINICAL FEATURES AND SURGICAL APPROACH Surgery Department, Tanta, Egypt

Zaida De Machaj1 , Guzman Aranda2 , Eric Chong3 , Moises Duran4 , Background and Aims: Thyroid carcinomas among the pediatric pop-
Laurent Bruggerman2 ulation are rare tumors that represent only 0.7% of all encountered
1 Hospital del Niño Doctor José Renán Esquivel, Pediatric Surgery, Panama, childhood malignancies. This study aimed to discuss management pro-
Panama; 2 Hospital del Niño Dr José Renán Esquivel, Neurosurgery, Panama, tocol, surgical complications, and outcomes of thyroid carcinoma in
Panama; 3 Hospital del Niño Dr Jose Renán Esquivel, Radiology, Panama, children.
Panama; 4 Hospital del Niño Dr Jose Renan squivel, Pathology, Panama, Methods: We performed a retrospective analysis including 32 patients
Panama who were managed at our institution between January 2011 and
January 2021. Data were analyzed regarding demographics, clinical
Background and Aims: To characterize the clinical characteristics of features, operative details, postoperative complications, and survival
children diagnosed with SGCT and to analyze the therapeutic out- data.
comes of multidisciplinary management at Hospital del Nino Dr. José Results: There were 26 females (81.25%) and 6 males (18.75%).
Renan Esquivel (HDNDRJRE) from March 2018 to December 2021. The median age at operation was 14 years (range: 5-18). Twenty-six
Methods: Patients with SGCT were treated at Panama’s Hospital del (81.25%) patients presented with palpable thyroid swelling. Median
Nino by Dr. José Renan Esquivel from March 2018 to December tumor size was 3 cm (range: 1-7). Metastatic workup did not detect
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S570 of S635 ABSTRACTS

any pulmonary metastases. Total thyroidectomy was performed in PO008 / #1538 METASTATIC MEDULLARY THYROID
25 patients (78%) and 16 of them had additional neck dissection. CARCINOMA (MTC) AND EWING’S SARCOMA AS A SOURCE
Seven patients (22%) underwent hemithyroidectomy and only one of OF ECPUBLICATION ONLY TOPIC ADRENOCORTICOTROPIC
them had a completion thyroidectomy after one week. Conservative HORMONE (ACTH) LEADING TO CUSHING’S SYNDROME
resection was adopted in six children with similar criteria (tumor size
<1.5 cm in one lobe, no extrathyroid extension, differentiated thyroid Dhruv Mahajan1 , Sandeep Agarwala2 , Vishesh Jain3 , Anjan Dhua4 ,
carcinoma, no detected lymph nodes). Postoperative complications Rajesh Khadgawat5 , Nikhil Tandon5 , Manisha Jana6 ,
occurred in eight patients (all had total thyroidectomy) with an overall Devasenathipathy Kandaswamy7 , Rakesh Lodha8
incidence of 25%. Seven patients had transient morbidities that were 1 All India Institute of Medical Sciences, New Delhi., Pediatric Surgery, new
managed conservatively (chylous leak n=1, hypoparathyroidism n=3, delhi, India; 2 All India Institute of Medical Sciences, Pediatric Surgery, New
and nerve palsy n=3). At a median follow-up time of 54 months, four Delhi, India; 3 All India Institute of Medical Sciences, New Delhi, India, Pedi-
patients had relapsed (all underwent total thyroidectomy). The 5-year atric Surgery, south Delhi, India; 4 All India Institute of Medical Sciences,
OS and EFS were 100% and 87.5%, respectively. Department Of Pediatric Surgery, New Delhi, India; 5 AIIMS, Endocrinology,
Conclusions: Operative resection for pediatric thyroid carcinoma can New Delhi, India; 6 All India Institute of Medical Sciences, Department Of
be performed with minimal complications and achieving excellent out- Radiodiagnosis, New Delhi, India; 7 All India Institute of Medical Sciences,
comes. Total thyroidectomy remains the standard procedure of choice New Delhi, India, Radiodiagnosis, New Delhi, India; 8 AIIMS, Pediatrics, New
in the majority of those patients. However, conservative surgery can be Delhi, India
successfully adopted in a well-selected group of children with favorable
long-term results as per our findings. Background and Aims: EcPublication Only Topic Adrenocorticotropic
hormone(ACTH) syndrome(EAS) as a cause of Cushing’s Syndrome(CS)
is not only difficult to diagnose, but also difficult to manage. Medullary
PO007 / #1945 MANAGMENT OF BILATERAL WILM’S thyroid carcinoma(MTC) causing EAS is rarer, especially in the pediatric
TUMOR : CASE REPORT population. The management of fulminant CS in such a patient poses a
complex dilemma.
Aida Elgouacem Methods: A 10-year-old male presented with features of CS with
mother and child hospital constantine algeria, Department Of Pediatric increased weight gain, hyperpigmentation, facial puffiness, easy bruis-
Surgery Constantine Algeria, mansourah, Algeria ing, and decreased height gain. The diagnosis was confirmed by raised
Serum Cortisol levels, 24 hours free urinary cortisol, and dexametha-
Background and Aims: Wilms tumour is an embryonal tumour derived sone overnight test. A raised serum ACTH level and normal MRI
from the metanephros. It is the commonest childhood renal tumour brain led to suspicion of EAS. A 68- Gallium DOTANOC scan revealed
and the third commonest paediatric malignancy. Synchronous bilat- somatostatin receptor-expressing nodules in both lobes of the thyroid
eral Wilms tumours (BWT) represent 4-7% of all Wilms tumours (WT) with cervical nodal and bilateral pulmonary metastasis. A Fine needle
and present at a younger age than unilateral Wilms tumours. The aspiration cytology of the thyroid nodule confirmed the diagnosis of
management of bilateral Wilms tumor (BWT) was non-standardized MTC, corroborated by raised serum calcitonin (>2,000pg/ml) levels.
and suffered from instances of prolonged chemotherapy and incon- There were no features of multiple endocrine neoplasia(MEN). CS-
sistent surgical management which resulted in suboptimal renal and induced hypertension and dyselectrolytemia was barely manageable
oncologic outcomes. Because of the risk of end-stage renal disease by pharmacotherapy. Due to deteriorating clinical condition and unpre-
associated with the management of BWT, neoadjuvant chemotherapy dictable response to inhibitors of adrenal steroidogenesis, an upfront
and nephron-sparing surgery have been adopted as the guiding man- bilateral adrenalectomy was performed to control the symptoms. How-
agement principles. This management strategy balances acceptable ever, the ultimate outcome would remain poor due to metastatic
oncologic outcomes against the risk of end-stage renal disease. medullary carcinoma thyroid.
Methods: We present a case of bilateral Wilms tumor in a nonsyn- Results: The use of DOTANOC in EAS is a useful tool in the identifi-
dromic 12-month-old male. Our management approach included 12 cation of the primary site causing EAS. Bilateral adrenalectomy offers
weeks of preoperative chemotherapy for maximal tumor shrinkage, effective and expeditious management of uncontrollable Cushing syn-
successful staged unilateral nephron-sparing surgery drome, albeit requiring hormone replacement later on.
Results: We advocate for a broader application of nephron-sparing Conclusions: CS is a life-threatening disease in children which could
surgery in bilateral Wilms tumor cases with the goal of preserving renal rarely be caused by ecPublication Only Topic ACTH production by
function without compromising oncologic outcomes MTC. The significant morbidity of CS associated with the poor progno-
Conclusions: We advocate for a broader application of nephron- sis of metastatic MTC poses a daunting therapeutic challenge. Tackling
sparing surgery in bilateral Wilms tumor cases with the goal of fatal CS first by adrenalectomy followed up by resection of the primary
preserving renal function without compromising oncologic outcomes tumor, later on, is a viable treatment option.
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ABSTRACTS S571 of S635

PO009 / #1122 INTRAOPERATIVE FINE NEEDLE BIOPSY OF Background and Aims: Background: The tumours of the skull base in
A METACHRONOUS TESTICULAR TUMOR IN A TEENAGER children are unfrequent patologies requiring interdiciplinary coopera-
tion in surgical approach and further oncological treatment.
Carolina Soares-Aquino1 , Helena Barroca2 , Joana Mafalda Monteiro1 , Methods: During the period of last 3 years we have operated on 5
Nuno Farinha3 , Sofia Vasconcelos-Castro1 children with skull base tumours of different origin and histological
1 Centro Hospitalar Universitário de São João, Paediatric Surgery Depart- classification. The age was from 1 to 13 years. There was one Ewing
ment, Porto, Portugal; 2 Centro Hospitalar Universitário de São João, Serviço sarcoma of petrous bone, one rhabdoid tumour in nasopharyngeal loca-
De Anatomia Patológica, Porto, Portugal; 3 Centro Hospitalar Universitário tion, one infantile myofibromatosis of ethmoid sinus, middle orbit and
de São João, Department Of Paediatric Oncology, Porto, Portugal nasal cavity. One lymfoma of posterior aspect of pyramid bone and pos-
terior fossa and one PNET of middle fossa and petrous bone. Patients
Background and Aims: Metachronous testicular tumors are rare, were operated according to location of tumour using subfrontal com-
and testicular-sparing surgical options are sometimes used in order bined with intranasal approach, and combined approach to middle
to preserve fertility and endocrine function. We aim to describe a and posterior fossa. In 2 of cases the aproache was performed with
case of the use of intraoperative fine-needle biopsy to evaluate malig- maxillofacial surgeon.
nancy, allowing the intraoperative decision of the surgical treatment Results: In 2 patient we achieved complete removal of the tumour
accordingly. (one retrosigmoidal, one anterior skull base location). In 3 patients
Methods: A 16-year old boy with a right testicular mass, later con- the subtotal removal was performed with residual tumour in petrous
firmed as a mixed germ cell tumor (95% embryonal carcinoma, 5% bone and cavernous sinus respectively. In all patients the postopera-
yolk-sac tumor), was referred to our center after right inguinal orchiec- tive course vas uneventful, we had no postoperative complication and
tomy and prosthesis insertion. In preoperative study, serum tumor postoperative morbidity.
markers were negative, there was no mass on the left testicle but Conclusions: Despite mostly bad prognosis of the patients with highly
important microlithiasis was seen on both testis, and there was no malignat tumours involving the base of the scull the radicality of
evidence of pathological retroperitoneal lymph nodes nor metastatic surgery /if possible/ is the initial and most important step in the treat-
disease. One month after surgery, raised alpha-fetoprotein and beta- ment of these unfrequent lesions. It improves the efficacy of further
human chorionic gonadotropin were noted. Doppler ultrasound of the oncological treatment and in our small group of patients we have
left testicle revealed a new left testicular metachronous tumor. After observed the reduction of the clinical symptoms postoperatively.
multidisciplinary discussion, the patient underwent left inguinotomy
and after high cord clamping a fine needle ultrasound-guided biopsy of
the tumor was done. Publication Only Topic: AS02 Radiation Oncology - PROS
Results: Rapid on-site cytological evaluation (ROSE) confirmed the
presence of a malignant germ cell tumor. This way, a left radical orchiec- PO011 / #1836 RADIOSENSITIVITY OF PEDIATRIC NASAL
tomy with insertion of a testicular prosthesis was done, followed by RHABDOMYOSARCOMA CHEMOREFRACTORY: A CASE
totally implanted central venous access introduction during the same REPORT AND REVIEW OF LITERATURE
procedure. The definitive histology confirmed a mixed germ-cell tumor
(60% embryonal carcinoma; 20% yolk-sac tumor; 20% seminoma; <1% Olga Córdova1 , Silvia Guerra2 , Mario Quispe2 , Percy Torres2
cytotrophoblast). Two weeks after surgery the patient began the first 1 Hospital Edgardo Rebagliati Martins, Oncology And Radiotherapy, Lima,
cycle of chemotherapy (bleomycin, etoposide and cisplatin) and he is Peru; 2 Hospital Rebagliati Martins, Oncology And Radiotherapy, Lima, Peru
currently still under treatment.
Conclusions: To our knowledge, this is the earliest metachronous Background and Aims: Rhabdomyosarcoma (RMS) is a rare malig-
presentation described in pediatric age. Intraoperative fine nee- nancy, however it represented the most common soft-tissue sarcomas
dle biopsy after cord clamping did not result in tumor spillage, in pediatrics. RMS has a poor prognosis, because the tumor has a
allowed for a rapid diagnosis and was compatible with definitive tendency towards extensive local and metastatic dissemination. We
histology. presented a case of a nasal RMS pediatric with a dramatic response to
radiotherapy after chemotherapy schemes failed.
Methods: A case report and review of literature
PO010 / #487 RARE TUMOURS OF SKULL BASE IN Results: RMS is an malignant tumor arising in the mesenchymal cells.
CHILDREN, EXPERIENCE OF 5 CONSECUTIVE CASES The majority (40%) are localized in H&N region. The lung is the most
frequent site of distant metastasis. We reported the case of a 6 year-old
Michal Tichy boy with a history of a tumor in the left nasal ala of 3.5 cm, and a histol-
University Hospital Motol, Second Medical Faculty, Department Of Neuro- ogy of embryonal rhabdomyosarcoma. After diagnosis received three
surgery, Prague, Czech Republic different schemes of chemotherapy with a poor response; the surgery
option was postponed and then discarded. The patient has not had
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S572 of S635 ABSTRACTS

regular treatment because of socio economics factors. He was admit- chemotherapy and radiotherapy seems tobe superior to single modal-
ted with a bulky and bleeding tumor mass of approximately 20 cm that ity especially foryoungerchildren.
occupied half of the face, and that made it difficult for adequate feed-
ing. We indicated radiotherapy, initially a dose of 40 Gy (2,5 Gy/fr), but
because of the amazing answer, we decided to increase the dose at 50 PO013 / #1905 RADIOTHERAPY FOR A BLOOM
Gy. He obtained a clinical complete response that allowed him to per- SYNDROME: HOW TO MANAGE?
form his basic functions without difficulty. He left the follow-up and
returned almost 7 months later with a new contralateral mass of 15 Safia Yahiaoui, Semia Zarraa, Ghaiet El Fide Noubbigh, Chiraz Nasr
cm, and we indicated a dose of 40 Gy (2.5Gy/fr). At the moment the SALAH AZAIZ INSTITUTE, Radiotherapy, Tunis, Tunisia
patient presents an 80% clinical response (pending 3 sessions). Inci-
dentally, nodules were evidenced in both pulmonary lobes, so he will Background and Aims: Patients diagnosedwithbloom’s syndrome (BS)
be derived to continue systemic management. have a higher risk of developping multiple cancers due to chromosome
Conclusions: RMS is an aggressive entity with a poor prognosis. Radio- abnormalities. Cancer treatments are often contraindicated or must be
therapy helps to local control. We provided better QOL, but the natural modified for persons with BS. Tha aim of this paper is to report a thera-
history of disease is evidenced peutic management of Hodgkin’s lymphoma occurring in a patient with
bloom syndrome.
Methods: We reported a case of an Hodgkin’s lymphoma occurring in
PO012 / #1880 PEDIATRIC CHOROID PLEXUS CARCINOMA : a patient with bloom syndrome. this case was treated at Salah Azaiz
A CASE REPORT Institute in 2020
Results: We report a case of a 17 yearold male patient diagnosed
Safia Yahiaoui, Semia Zarraa, Ghaiet El Fide Noubbigh, Chiraz Nasr with BS while preparing for the treatment of stage IAa Hodgkin
SALAH AZAIZ INSTITUTE, Radiotherapy, Tunis, Tunisia lymphoma, BS was suspected because of a photosensitivity, and a
microcephaly. the diagnosis was confirmed cytogenetically. he had 2
Background and Aims: Through a pediatric case of choroid plaxus couses of chemotherapy based on EuroNet protocle with a dose reduc-
carcinoma and a review of literature,we report the clinical case the tion with a complete repose after 2 courses of chemotherapy. During
histopathological characteristics of this entity as well as the role of chemotherapy the patient have had Febrile Neutropenia at the first
radiotherapy on its management. course. We choosed a conformal radiotherapy to avoid organ at risks
Methods: we discribed a case treated in salah Azaiz Institute in 2015 (parotid gland ant the thyroid).Target volumes and fields was specified
Results: A 5-year-old infant boy complained of headache and vom- based on the EuroNet protocol with 1.8 gy per fraction to a total dose
iting. The physical examination found a static and kinetic cerebellar of 18 Gy with no boost. the tolerance of radiotherapy was good with
syndrome. a Brain magneticresonanceimaging (MRI) revealed nodu- only a grade I/II radiodermatitis and a grade II radiomucositis.After a
lar lesion at the occipital horn of the leftlateralventricle,it was a follow-up of 8 months the patient is in remission
25*20*22mm sized-mass and it was associated with intaventricular Conclusions: Photosensitivity among patients with BS makes radio-
bleeding. The patient was referredto a neurosugeon,he had a macro- therapy challenging : on one hand it has more serious and threatening
scopicallycomplete excision and thehistologiccharacteristics of the complications such of developping secondary radioinduced cancer and
tumor were Compatible with CPC. Weconductedpostoperative radio- on the other hand the tumor responds so well to radiation. So radio-
therapy (RT) at the dose of 54 Gy in30 fractionsat the rate of 1.8 Gy therapits need to be careful with the technique used, the total dose,
/ session for the tumorbed and 36 Gy for the craniospinal axis. he RT the fractioning. Constant monitoring of the patient during treatment
technique was 3Dto minimize the dose at level of cochlea and temporal is important, in order to prevent and treat toxicities observed and to
lobes. We used photons of 6 - 18 MV for the cranial fields and the tumor know when the treatment needs to be stopped.
bed and photons from 4 to 6MV or electrons for spinal fields.All doses
contraints were respected. The young boy received3 courses of adju-
vant CT based on Vincristine,Etoposide,Cyclophosphamide and Carbo- Publication Only Topic: AS03 CCI - Childhood Cancer International
platin. He wasfollowed up withbrain MRI 3 monthsafter CT whichre-
vealed a cranio-spinal progressive desease. a palliative CT includingTe- A SYSTEMATIC REVIEW ON OUTCOMES OF PARENTAL
mozolamidewasindicated but the childwasdeadafter the 2 nd cure. DISTRESS IN CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA
Conclusions: choroid plexus carcinoma is a high grade subtype.The
treatmentremains a therapeutic challenge. In fact: the rarity of thisen- Ana Ferraz, Martim Santos, M. Da Graça Pereira
titymakesitdifficult to conductrandomizedclinical trials. While,some University of Minho, Research Center In Psychology (cipsi), School Of
evidence suggests that gross total tumor resection remains the most Psychology, Braga, Portugal
important prognostic factor in all choiroid plexus tumors. chemother-
apy and radiotherapy remains a Publication Only Topic of consider- Background and Aims: Distress among parents of children with
able debate therefore more studies are needed. combined adjuvant Acute Lymphoblastic Leukemia (ALL) is common during treatment and
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ABSTRACTS S573 of S635

can persist several years post-diagnosis, impacting the adjustment of Results: A total of 35 children were consulted to our clinic. Half of
parents and children. This review focused on associated variables, pre- them were female. The mean age of them was 7.6±6.4 (median 5.4)
dictors and outcomes of parental distress following the ALL of their years. Three out of four patients had fever. Half of the patients had
child. cough complaint. Only five patients (15%) had Covid-19 contact his-
Methods: PubMed, Web of Science, and PsychINFo databases were tory. Half of the patients had already chronic disease. Four patients
searched for papers published from 1983 to 2021. PRISMA state- (12%) had Covid-19 PCR positive. Ten patients (29%) had Covid-19
ment was followed, and papers were evaluated through a standardized coherent computerized tomography imaging. Fourteen patients (40%)
methodological quality assessment tool (NHLBI). were given Covid-19 treatment with clinical findings. Three patients
Results: Of the 25 papers included, 13 were evaluated as good, seven as were treated at intensive care unit. The first (52%) to consult to pedi-
fair, and five as poor. Twelve papers reported subgroup differences and atric hematology was increased D-Dimer level. Second (20%) reason
11 found potential predictors of parental distress, including sociode- was abnormality in blood count (neutropenia and thrombocytope-
mographic, psychological/psychosocial, family, and illness-related vari- nia). Thrombosis and bleeding disorder were another reasons. The
ables. Significant correlations were found between parental distress mean hospitalization day was significantly higher in patients treated as
and social support, illness cognitions, resilience, as well as contradic- Covid-19 compared to others (9.2 vs. 2.6 days, p<0.01).
tory results regarding the impact of sociodemographic variables on Conclusions: Pediatric patients were also affected from Covid-19 pan-
parental distress. Family cohesion and care burden were associated demic. D-Dimer level, neutropenia, thrombocytopenia and thrombosis
with distress, and healthy coping strategies correlated with lower anx- were most significant consultation reasons to pediatric hematology
iety. Caregiver strain contributed to distress and the overall impact of and oncology clinic.
child illness positively predicted anxiety in mothers and somatizations
in fathers. Parental distress differences were found regarding group
risk and time since diagnosis. Eleven papers explored outcomes of PO016 / #1144 EARLY PREDICTORS OF DISTRESS IN
parental distress, including psychosocial, health, and social/education MOTHERS OF PEDIATRIC CANCER SURVIVORS AT FIVE YEARS
factors. Distress was the most significant factor of family strain. Signifi- POST-DIAGNOSIS
cant correlations were found between parental distress, at diagnosis,
and further psychological adjustment. Most papers reported corre- Jessica Quach1 , Anna Olsavsky1 , Jessica Ralph1 , Kathryn Vannatta1 ,
lations between parental distress and psychological condition, life Bruce Compas2 , Cynthia Gerhardt1
quality, and adjustment of the children, although few studies reported 1 The Abigail Wexner Research Institute at Nationwide Children’s Hospi-
no association. Correlations between maternal depression and child tal, Center For Biobehavioral Health, Columbus, United States of America;
participation in education and social life were found. 2 Vanderbilt Kennedy Center for Research, Human Development, Nashville,
Conclusions: Longitudinal studies are needed to better understand the United States of America
phenomenon and its consequences, especially related to health out-
comes. Future interventions should mainly focus on parents, over time, Background and Aims: Although most pediatric cancer survivors and
on distress reduction and psychological adjustment both in parents and families return to normative levels of adjustment following treatment,
children. some parents experience elevated depressive symptoms and post-
traumatic stress symptoms (PTSS). Thus, we investigated the predictive
roles of sociodemographic factors and stress in mothers’ distress in
PO015 / #1216 PEDIATRIC HEMATOLOGY AND ONCOLOGY survivorship.
CONSULTATION DURING EARLY COVID-19 PERIOD Methods: Mothers (N = 81) reported on sociodemographic factors,
cancer-specific stress, general stress, depressive symptoms, and PTSS
Zeynep Karakas, Mustafa Bilici, Yasin Yilmaz, Gulsah Tanyildiz, Deniz at enrollment (T1) and five years post-diagnosis (T2). Separate hier-
Tuğcu, Serap Karaman, Ayşegül Ünüvar archical regressions examined T1 sociodemographic factors (i.e., age,
Istanbul University, Pediatric Oncology, ISTANBUL, Turkey education, race, ethnicity), general stress, and cancer-related stress as
predictors of T2 maternal distress (depressive symptoms and PTSS).
Background and Aims: Covid-19 pandemic affect allover world. Physi- Additional models examined predictors of change from T1 to T2 by
cians in hospital showed great effort and clinicians were consulted for controlling for T1 distress.
many patients that they followed up. In our study, we collected data Results: On average, mothers reported normative levels of depres-
of patients who were consulted to pediatric hematology and oncology sive symptoms and PTSS at T2. T1 mother age (β = -0.21, p = .05),
clinic. education (β = -0.21, p = .04) and general stress (β = 0.38, p = .002)
Methods: Patients consulted to department of pediatric hematology predicted T2 depressive symptoms, F(6,74) = 5.44, p <. 001, R2 =.306,
and oncology clinic during early Covid-19 period (January2020-June while T1 education was the only significant predictor (β = -0.29, p =
2020) were our study group. Reason for consultation and other clinical .01) of T2 PTSS, F(6,74) = 3.65, p = .003, R2 =.228. The model predict-
data were collected from patients file. Statistical analysis was done by ing change in depressive symptoms was significant, F(7,73) = 6.56, p
using SPSS (v23). < .001, R2 =.386; T1 depressive symptoms were the only significant
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S574 of S635 ABSTRACTS

predictor (β = 0.46, p = .003). The PTSS change model was significant, (MRD) by flow cytometry (except for one who had 0.1% MRD). In
F(7,73) = 3.49, p = .003, R2 =.251; the only significant predictor was one patient blinatumomab was stopped due to hemodynamic instabil-
education (β = -0.28, p = .01). ity and later to grade 3 neurotoxicity, which recovered after stopping
Conclusions: Five years post-diagnosis, mothers of childhood can- treatment. Other patients had no complications. Three remained in CR
cer survivors reported normative levels of distress. Sociodemographic with negative MRD and went for transplant. Two of them died of SCT
factors, including younger age and lower education, may be related complications and one is alive in remission 2 years later. The patient
to long-term distress. Although further longitudinal research is war- with extramedullar disease presented leukemia progression during bli-
ranted, clinicians should screen mothers for distress over time and natumomab and died. One patient in second medullar BCP-ALL relapse
provide targeted services, especially for those who are younger, have received inotuzumab. After two courses achieved CR with negative
less education, and are exposed to more stress near diagnosis. MRD and went for SCT. She did not present side effects nor hep-
atic complications after SCT. She had third extramedullar relapse after
transplant and is currently in forth relapse.
Publication Only Topic: AS05 SIOP Scientific programme / AS05.a Conclusions: Immunotherapy is effective in patients with relapsed
Acute Lymphoblastic Leukaemia BCP-ALL. We present our experience without serious side effects and
good results in 4 of 5 patients as a bridge to transplantation, even
PO017 / #1444 PATIENTS WITH RELAPSED ACUTE though these patients still have poor prognosis due to the disease and
LYMPHOBLASTIC LEUKEMIA TREATED WITH BLINATUMOMAB treatment complications.
AND INOTUZUMAB. EXPERIENCE IN A TERTIARY CARE
HOSPITAL
PO018 / #263 BLINATUMOMAB IN ADOLESCENTS WITH
Rosa Adan-Pedroso1,2 , Jimena De Pedro-Olabarri1,2 , Paula REFRACTORY ACUTE LYMPHOBLASTIC LEUKEMIA AND
González-Urdiales1,2 , Marina Moreno-Diez3 , Aizpea RELAPSED B LYMPHOBLASTIC LYMPHOMA WITH PERSISTENT
Echebarria-Barona1,2 , Miguel García-Ariza1,2 , Ricardo POSITIVE MINIMAL RESIDUAL DISEASE. REPORT OF TWO
López-Almaraz1,2 , Elena Landeta-Callejo4 , Idoia Ancin-Arteaga4 , CASES IN ARGENTINA
Veronica Roldan-Galiacho4 , Javier Arzuaga-Mendez4 , Laura
Garcia-Naveda5 , Paula Arana-Berganza6 , Maria Angeles Gil-Lemus7 , Carolina Basile1 , Mariana Nana2 , Ezequiel Recondo1 , Stella
Itziar Astigarraga1,2,8 Ballestrini3 , Martin Hornos1 , Diego Rosso1
1 Hospital Universitario de Cruces, Pediatric Hemato-oncology, Barakaldo, 1 Hospital de Clinicas Jose de San Martin/ Hospital de Niños Pedro de
Spain; 2 Biocruces Bizkaia Health Research Institute, Pediatric Oncology Elizalde, Pediatric - Oncohematology Section - Aya Group, Buenos Aires,
Group, Barakaldo, Spain; 3 Hospital Universitario de Tenerife, Paediatrics, Argentina; 2 Hospital de Clinicas Jose de San Martin, Pediatric - Oncohema-
Tenerife, Spain; 4 Hospital Universitario de Cruces, Hematology, Barakaldo, tology Section - Aya Group, Buenos Aires, Argentina; 3 Hospital de Clinicas
Spain; 5 Hospital Universitario de Cruces, Genetics, Barakaldo, Spain; Jose de San martin, Pediatric - Oncohematology Section - Aya Group, Buenos
6 Hospital Universitario de Cruces, Immunology, Barakaldo, Spain; 7 Hospital Aires, Argentina
de Cruces, Pharmacy Department, Barakaldo, Spain; 8 Faculty of Medicine
and Nursing. University of the Basque Country (UPV/EHU), Pediatrics Background and Aims: Blinatumomab is a novel bispecific T-cell
Department, Leioa, Spain engager that simultaneously binds CD3-positive cytotoxic T-cells and
CD19-positive B-cells, resulting in selective lysis of tumor cells. It has
Background and Aims: Analyze data from relapsed B cell precur- shown promising results in patients(p) with relapsed or refractory
sor acute lymphoblastic leukemia (BCP-ALL) patients treated with acute lymphoblastic leukemia/lymphoma or those achieving hema-
blinatumomab or inotuzumab in our hospital. tologic response with persistent minimal residual disease(MRD).We
Methods: Retrospective study of 5 children and adolescents with report our experience with the use of Blinatumomab in two adoles-
relapsed BCP-ALL who received blinatumomab (off label) or ino- cents with persistent positive MRD despite intensive chemotherapy.
tuzumab in our Unit as part of salvage therapy. Methods: We retrospectively describe clinical and epidemiological fea-
Results: The median age at the time of blinatumomab administra- tures, feasibility, infusion characteristics, adverse effects and outcome
tion was 9 (3-14) years. The patient who received inotuzumab was of 2p with persistent MRD treated in an Argentinean institution in
18 years. Four patients received blinatumomab. All had first relapse one year. Toxicity was considered according to Common Terminology
BCP-ALL (three early isolated medullar relapse and one early iso- Criteria for Adverse Events 4.0.
lated extramedullar relapse). All received treatment according to Results: Two male patients, age 17, with refractory B-precursor acute
SEHOP-PETHEMA-2015-Rec-ALL relapse protocol. One of them had lymphoblastic leukemia(A) and second relapsed B lymphoblastic lym-
also received two additional chemotherapy courses due to resistant phoma(B). Treated with pediatric protocols, A (1st line treatment) and
disease. Blinatumomab was administered as a bridge to stem cell B (3rd line treatment). Dose and infusion scheme: 28 ug/day maximum,
transplantation (SCT). Prior to blinatumomab patients were in com- intravenous continuous, for 28 days. Patient A received full dose from
plete remission (CR) with negative (<0.01%) minimal residual disease the begging and p B received increasing doses up to full dose. Toxicity: p
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S575 of S635

A grade 4 neurotoxicity (epileptic status) and grade 3 transaminitis; p B PO020 / #1906 SIGNIFICANCE OF RT-PCR AND FISH AS
grade 3 diarrhea and thrombocytopenia. Infusion was stopped and pro- MINIMAL RESIDUAL DISEASE (MRD) IN PEDIATRIC PATIENTS
gressively restarted in p A because of neurological toxicity. No grade WITH ACUTE LEUKEMIA (AL)
5 toxicity. No relapses or progression. We achieved negative MRD in
both p after one cycle. Both p are alive. Rocío Yemeli Buenrostro Aguilar1,2 , Daniela Arce Cabrera2 , Jorge
Conclusions: In our experience, the administration was feasible. Both Guzman2 , Eduardo Altamirano2 , José Rodrigo Lozano2 , Guadalupe
p were induced to complete remission after one course of Blinatu- Valenzuela Aguilar1 , Obdilia Gutierrez Guzman1 , Maité Echeverria1
momab. One p had a severe adverse effect but was able to continue 1 Hospital Pediátrico de Sinaloa, Pediatric Oncology Unit, Sinaloa, Mexico;
treatment with infusion changes. They proceeded to allogeneic bone 2 Hospital Pediatrico de Sinaloa, Hemato-oncology Unit, Culiacan, Mexico

marrow transplant.
Background and Aims: RT-PCR and FISH are useful tools to detect
molecular alterations (MA) in AL. The presence MRD following ther-
PO019 / #281 NUTRITIONAL STATUS OF GUATEMALAN apy for AL has been shown to be an important prognostic marker. The
CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA (ALL) main objective of this paper is to analyze the molecular response (MR)
AND THEIR SIBLINGS. A STUDY OF THE RELATIVE IMPACTS OF by RT-PCR and FISH in patients with MA in AL
DISEASE AND SOCIO-ECONOMIC STATUS (SES) Methods: This study included 74 patients with newly diagnosis of AL,
admitted to a Northwest Hospital of Mexico, between April 2018 and
Paulina Ubico1 , Jessica Blanco1 , Troy Farncombe2 , Marisabel Perez3 , January 2022, all the bone marrow samples were analyzed at diag-
Ana Lucia Molina1 , Gerson Morales1 nostic by flow cytometry (FC), RT-PCR and FISH to determine the
1 Unidad Nacional de Oncologia Pediatrica, Pediatrics, d, Guatemala; immunophenotype and the most common MA. Patients with a MA
2 McMaster University, Nuclear Medicine, d, Canada; 3 Tecniscan, Radiology, identified, were classified to the risk as low, intermediate and high
d, Guatemala according to clinical, laboratory and MA features. MR after remission
induction treatment (RIT) was evaluated by RT-PCR and FISH, patients
Background and Aims: Children with ALL in Guatemala are often mal- without MA were evaluated by FC
nourished at diagnosis and come from families with low SES.Persistent Results: Of the 74 samples. In patients with Acute Linfoblastic
malnutrition during therapy is associated with a poor survival rate Leukemia (n=61), 50% (31) had MA, classified at diagnosis into low
but improvement in nutritional status redresses this adverse out- 19.3% (n=6), intermediate 19.3% (n=6) and high risk 61.3% (n=19).
come.To investigate the respective roles of disease and SES, nutri- One patient (16.6) in low, one patient (16.6) in intermediate and five
tional status was studied in children with ALL and their available patients (26.3) in high risk did not achieve MR after RIT. Patients with
siblings. Acute Myeloid Leukemia (n=13), 92% (n=12) had MA, categorized into
Methods: Children with ALL (N=54) and their siblings (N=17) were low risk 58% (n=7) and high risk 42% (n=5). Two patients (29%) of low
assessed by arm anthropometry and dual energy X ray absorptiome- and two patients (40%) in high risk did not have a MR after RIT
try (DXA). SES was measured by a customised instrument developed Conclusions: The significance of this study is that if we evaluate
at UNOP.The higher the SES score the more disadvantaged the fam- patients with good prognosis MA as t(8;21) and did not achieved MR we
ily. DXA was repeated in the patients at the end of remission induction can modified treatment to improve the prognosis. Patients with high
(N=53) and 6 months from diagnosis (N=46). MA and poor response were t9;22, TRA/D gene disruption and FLT3
Results: At diagnosis 24 children (44.4%) and 2 siblings (11.8%) ITD mutation
were malnourished, p=0.04, defined by mid upper arm circumference
<5th percentile. The median fat mass index (FMI) Z score was lower
in the patients (-1.80) than in the siblings (0.06), p=0.03, with no sig- PO021 / #1386 COMPARISON OF ALL IC-BFM 2009 AND
nificant difference in the median Z score for appendicular lean mass COG PROTOCOL IN TERMS OF SURVIVAL IN PATIENTS
index (ALMI), a surrogate measure of skeletal muscle mass. At the end TREATED WITH ACUTE LYMPHOBLASTIC LEUKEMIA
of remission induction the median FMI Z score had risen to 0.055
(p=0.008) but the median ALMI Z score fell from -1.37 to -3.035 Dilvin Çelik Ateş1 , Fatma Betul Cakir2
(p<0.001), although by 6 months it had risen to -1.93. In a multi- 1 Bezmialem Vakif University, Pediatric Hematology And Oncology, İstanbul,

ple linear model, with increasing SES score the expected Z score for Turkey; 2 Bezmialem Vakif University, Pediatric Hematology And Oncology,
FMI decreased in patients (-0.03+/-0.01, p=0.04) while in siblings it Istanbul, Turkey
increased (0.65+/-0.028, p=0.02).
Conclusions: Children with ALL at UNOP, as elsewhere, gained fat Background and Aims: Various chemotherapy protocols are used in
and lost muscle during remission induction with some restoration of the treatment of acute lymphoblastic leukemia (ALL), which is the most
muscle by 6 months from diagnosis. Siblings were less malnourished common malignant disease in childhood. This study sought to com-
and the study of SES indicated separate influences of ALL and SES on pare the survival analysis of patients who received ALL IC-BFM 2009
nutritional status. therapy and COG therapy.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S576 of S635 ABSTRACTS

Methods: We retrospectively evaluated ALL patients who received Conclusions: This study allowed us to describe the main characteristics
ALL IC BFM 2009 (group1) and COG (group2) treatment between of our population, to create a database that will allow to compare later
2011 and 2019. Survival analysis was performed for both groups. to the patients managed in the POU, according to the new therapeutic
Results: There were 29 patients in Group 1 (15:M/14:F). There were recommendations, begun in 2021.
33 patients in Group 2 (21:M/12:F). In group 1, 3 patients died during
treatment and 2 patients died as relapse during maintenance (%17,2).
In group 2, 1 patient died during treatment and 3 patients died due PO023 / #41 T CELLS IN PEDIATRIC LEUKEMIA
to infection during maintenance (%12,1). There was no significant dif-
ference in survival analysis between the two groups we compared Alejandra Gonzalez, Ana Santizo Gaitan, Pedro Alvarado Reyes,
(p:0.53). Martha Ramirez
Conclusions: Both chemotherapy protocols are successful and widely Hospital General San Juan de Dios, Guatemala, Guatemala, Guatemala
used in Turkey. The absence of a significant difference between the
two treatments will be significant for the centers that will choose the Background and Aims: The T cells inmunophenotype is present in
protocol. 10% of the children with ALL. Is more frecuently associated with
older age, higher leukocyte counts and bulky extramedullary disease.
In the present case, the risk factors and the immediate response to
PO022 / #1269 EPIDEMIOLOGICAL, CLINICAL AND chemotherapy treatment will be evaluated and the current process of
BIOLOGICAL ASPECTS OF ACUTE LYMPHOBLASTIC LEUKEMIA the patient.
IN SUB SAHARIAN AFRICAB: EXPERIENCE OF PEDIATRIC Methods: An 11-year-old male patient, referred from Chiquimula who
ONCOLOGY UNIT IN DAKAR SENEGAL presents two months prior to consultation with jaundice, paleness and
pain in lower extremities. On physical examination he has 1 cm cervical
Mame Diouf1 , Fatou Diagne1 , Ndiaye Awa1 , Abou Koundio2 , Fatimata lymphadenopathies on both sides of the neck and hepatosplenomegaly.
Sall2 , Awa Toure2 , Diedhiou Francis3 , Mallon Brenda3 , Jean Michon4 , Hematology is performed, which shows a white blood cell count of
Leverger Guy4 354 K/Ul, lymphocytes at 285.60%, hemoglobin at 7.80 gr/dl and
1 Aristide Le Dantec Hospital, Pediatry, Dakar, Senegal; 2 Aristide Le Dan- platelets at 32 k/Ul. In blood chemistry, relevant data shows uric
tec Hospital, Haematology, Dakar, Senegal; 3 GFAOP, Villejuif, paris, France; acid in 9.10 and lactate dehydrogenase in 1806. Chest X-ray shows
4 GFAOP, Villejuif, PARIS, France mediastinal widening. Hyperhydration and allopurinol are started
immediately. Peripheral rub is performed which shows the presence
Background and Aims: Acute lymphoblastic leukemia is the leading of blasts. In the bone marrow blasts are shown according to the
cause of pediatric cancer in Senegal. This may be the case in most sub- FAB L2 classification. Immunophenotyping is performed which reports
Saharan countries, but it cannot be demonstrated due to the scarcity ALL of T cells with low CD3, CD7, CD5, CD3cit, positive. Two days
of African data and publications on this disease. Poor technical plat- after diagnosis, a patient with respiratory distress and an increase in
form, insufficient trained human resources and also the similarity with lymph node adenopathy is evidenced. Hematology is performed again,
other endemic diseases such as malaria can often lead to misdiagnosis. which shows an increase in white blood cells by 446 K/IU, so treat-
The treatment recommendation proposed by the GFAOP are well tol- ment with chemotherapy is started immediately due to oncological
erated and lead to cure patients with a good prognosis. In 2021, funding urgency.
from the Laurette Fugain Foundation has enabled the implementation Results: Chemotherapy was started in the patient, starting with
of immunophenotyping in Dakar, leading to the proposal of new rec- cyclofosmide and vincristine, showing a reduction in cervical lym-
ommendations for the management of high-risk ALL as well in order to phadenopathy, hepatosplenomegaly, and jaundice 3 days after admin-
increase patient survival rates. istration. Patient presents decreased white blood cell count and con-
Methods: We retrospectively collected all cases of ALL managed at the tinues with the treatment. Entering remission on day 14 with notable
Dakar POU between January 1, 2017 and December 31, 2020 in order improvements.
to study the epidemiological, clinical, biological and therapeutic char- Conclusions: The recognition of risk factors and extramedullary dis-
acteristics. We also look at treatment tolerance, overall survival and ease is important for timely treatment and avoid future complications.
event-free survival. The use of chemotherapy in oncological emergency helps to improve it
Results: 143 patients were included with a mean age of 7 years and a and have a better prognosis.
sex ratio of 1.4. The average time elapsed between first observed signs
by health care workers and consultation was 4 days. 29% lived in Dakar
and 8 % came from another country. The main features were long- PO024 / #462 USE OF BLINATUMOMAB IN CHILDREN WITH
lasting fever, bone pain, cervical adenopathy and enlarged abdomen. All RELAPSED LLA. INITIAL EXPERIENCE
patients were treated with the LAL GFAOP standard risk protocol. We
noted 9 treatment abandonment. The overall survival is 23.7 % with a Blanca Herrero Velasco, Gloria Miguel Llordés, Rocio Vila De Frutos,
follow up superior to 12 months post diagnosis for 27 children. Sara Vinagre Enríquez, Luis Madero López
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S577 of S635

HOSPITAL INFANTIL UNIVERSITARIO DEL NIÑO JESUS, Oncology, Results: ALL (17), AML (1), Hodgkin lymphoma HL (4), non-Hodgkin
MADRID, Spain NHL (3). ALL sex 6/17 female, ALL 14/17 standard risk, 3/17 high risk;
as per NCI. B-lineage 16/17, T-cell ALL 1/17. All cases were CNS -
Background and Aims: Relapse are the main causes of treatment ve. Bone marrow FISH; trisomy 4,10,17 3/17; t (12;21) 2/17, Ph +ve
failure in childhood B-cell precursor acute lymphoblastic leukemia (B- 1/17; hyper diploidy 56 xy 2/17; t (1;19) 1/17; trisomy 21 1/17; T-cell
ALL). Blinatumomab, a bispecific CD19 × CD3 T-cell engager (BiTE) 1/17. Minimal residual disease MRD <0.01% in 11/17, <0.05% with Ph
antibody, links and directs endogenous CD3+ effector T cells against +ve. Relapses 4/17, 3 died from refractory leukaemia; 1st post stem
CD19+ B cells to induce apoptosis. cell transplant SCT. 2nd refractory T-cell CNS leukaemia, 3rd multiple
Methods: This is a retrospective descriptive study on the use of blina- relapsed ALL with DS. 4th early combined relapse. AML M3 t (15;17)
tumomab in pediatric patients with relapsed B-ALL from March 2016 diagnosed by fish. HL (4) 1st stage 4 relapsed, +ve PET scan, received
to March 2022. 2nd line therapy, autologous SCT. 2nd HL post chemotherapy PET scan
Results: Eight patients were included during the six years of the study. -ve so avoided radiotherapy, 3rd HL infant with extra lymphatic right
Five were male. The median age at the time of blinatumomab infu- orbital mass, died due to multi drug resistant infection and last HL stage
sion was 7.2 years (range 17.2-1 year). Two of the 8 patients (25%) 2B post chemo received IFXRT. 2 cases of Lymphoblastic Lymphoma
had received an allogeneic hematopoietic stem cell transplantation (LL) 1st stage 4 with CNS involvement and paraplegia who received
(HSCT). One patient received blinatumomab within a clinical trial. craniospinal radiotherapy. 2nd LL stage 3 relapsed, received 2nd line
Three patients (37,5%) received two cycles of blinatumomab, all of chemotherapy and 3rd Burkitt lymphoma with +ve PET scan given
whom achieved complete remission with the first cycle with MRD chemotherapy. Survival of ALL 83% & lymphoma 86% are comparable.
0.00%. Five patients (62,5%) achieved complete remission and received EFS of the whole cohort 6/25, 76% and OS 21/25, 84%. causes of death
an allogeneic HSCT as consolidation therapy. The administration of bli- 3 due to recurrent diseases and 1 toxic death.
natumomab did not present significant complications. A girl presented Conclusions: Genetic studies at diagnosis, fish panel of leukaemia and
with an episode of hypotension and capillary leak with withdrawal of end of induction MRD used for risk directed therapy. PET scan is crucial
the blinatumomab infusion for 24 hours, and a patient presented septic for diagnosis, management of lymphoma.
shock with favorable antibiotic response and didn’t require discontin-
uation of blinatumomab infusion. In four patients (50%) it was possible
to use an outpatient drug infusion regimen. The median follow-up of PO026 / #1826 IMPLEMENTATION OF REAL-TIME
patients was 5.4 months (range 5 years-1 month). Four patients (50%) MULTIPLEX RT-PCR IN ACUTE LEUKEMIA MANAGEMENT AT
are alive and disease-free; all of them received HSCT. Two patients died THE HEMOBIOLOGY AND BLOOD TRANSFUSION CENTER OF
(one for disease progression and other for toxicity after HSCT). Two THE MUSTAPHA-ALGIERS HOSPITAL
patients are still alive with disease receiving palliative care.
Conclusions: Targeted immunotherapies as blinatumomab have Amina Khennak, Houda Boudiaf, Issam Frigaa
demonstrated activity in pediatric patients with relapsed B-ALL. In Algiers hospital Mustafa Pasha, Algiers Downtown, Algiers, Algeria
this small study, blinatumomab was shown to be safe and to achieve
complete remission in more than half of the patients with relapsed Background and Aims: Fusion genes play an important role in
B-ALL. hematological malignancies. Nowadays, the diagnosis of acute
leukemia includes the systematic search for a number of fusion
transcripts. These transcripts can be detected by real-time mul-
PO025 / #896 REPORTING OUTCOME OF CHILDHOOD tiplex RT-PCR. Objective: Implementation of real-time multiplex
HAEMATOLOGICAL MALIGNANCIES FROM PRIVATE SECTOR RT-PCR in the diagnosis and the management of acute leukemia at
HOSPITAL: 4 YEAR EXPERIENCE AT DR. SULIMAN FAKEEH the Hemobiology and Blood Transfusion Center of Mustapha-Algiers
HOSPITAL, JEDDAH, SAUDI ARABIA hospital.
Methods: Our study involved 42 cases of pediatric acute leukemia of
Taha Khattab, Lamis Alkhateeb all types. We performed real-time multiplex RT-PCR using a multi-
Dr Suliman Fakeeh Hospital, Pediatrics, Jeddah, Saudi Arabia fusion gene detection kit which allows the search for AML1-ETO
CBFb-MYH11 PML-RARa transcripts associated with AML and TEL-
Background and Aims: Background: Cure rate of childhood acute AML1 E2A-PBX1 MLL-AF4 transcripts associated with ALL. The study
lymphoblastic leukaemia ALL, lymphoma 85%. Progress resulted from was completed in 8 cases by the search for the BCR-ABL (m-BCR)
using risk directed therapy. Objectives: To assess outcome, causes of transcript.
death. Results: Among the sixteen B-ALL cases analyzed, four were posi-
Methods: From October 2017 to October 2021 haematological malig- tive for the TEL-AML1 transcript and two positive for E2A-PBX1. Two
nancies will be reviewed. Event free EFS, overall survival OS will be of the three APL analyzed were positive for PML-RARa. The search
defined. for the other transcripts came back negative. Ultimately, standard
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S578 of S635 ABSTRACTS

operating procedures which cover all aspects of the laboratory work PO028 / #1361 COMPLETE THERAPY REFRACTORINESS IN
with some relating to specimen collection and test procedure and oth- A CHILD WITH AN EXTREMELY COMPLEX KARYOTYPE WITH
ers relating to laboratory organization management and safety have DICENTRIC DIC(9;20)
been written and validated.
Conclusions: The real-time multiplex RT-PCR allows the simultaneous Monika Lejman1 , Marta Malczewska2 , Dorota Sławińska2 , Joanna
search for several fusion transcripts, thus reducing the number and the Zawitkowska2 , Agata Pastorczak3 , Karolina Miarka Walczak3 , Iwona
time of manipulations. It is also a rapid technique, with satisfactory Dachowska Kałwak4 , Krzysztof Kałwak4
sensitivity and specificity and at low cost. 1 Medical University of Lublin, Independent Laboratory Of Genetic Diagnos-

tics, Lublin, Poland; 2 Medical University of Lublin, Department Of Paediatric


Haematology, Oncology And Transplantology, Lublin, Poland; 3 Medical Uni-
PO027 / #969 ACUTE LYMPHOBLASTIC LEUKEMIA IN versity of Lodz, Department Of Pediatrics, Oncology And Hematology, Łódź,
PEDIATRIC AGE GROUP (0-18 YEARS): INITIAL EXPERIENCE OF Poland; 4 Medical University of Wrocław, Clinical Department Of Paedi-
INDUCTION THERAPY FROM A DEVELOPING COUNTRY atric, Bone Marrow Transplantation, Oncology And Haematology, Wrocław,
SETTING IN NORTHEASTERN INDIA Poland

Chandra Kumar, Amrita Banerjee, Nitu Kumari, Pakkiresh Reddy, Background and Aims: The presence of the combination of the multi-
Yogita Chouhan ple chromosomal aberrations in childhood primary BCP-ALL indicates
All India Institute of Medical Sciences, Patna, Paediatrics, Phulwarisharif, an extremely adverse prognosis.
Patna, India Methods: We present a case of a 6-year-old girl who has been admitted
to the Department of Hemato-Oncology with a high fever and severe
Background and Aims: Acute lymphoblastic leukaemia is the most pain in the lower limbs.
common childhood cancer. Around eight to ten thousand children are Results: Bone marrow biopsy revealed 51.6% of lymphoblasts with
diagnosed with ALL every year in India. Our tertiary care institute has B-cell precursor immunophenotype. The FISH test did not show
recently started pediatric oncology unit. It is one the few oncology abnormalities in BCR::ABL1, ETV6::RUNX1, KMT2A and TCF3.
units in this state. In this paper we give a detailed overview about the Karyotype showed complex abnormalities: 44,-X,der(X)t(X;17)(p21;
induction therapy provided to thirty children diagnosed with ALL. p11.2),der(2)t(X;2)(p21;q35),der(4)t(4;20)(q21;q13),dic(9;20)(p13.2;
Aim: To analyse the demographic details, type, response to chemother- q11.2),der(17)t(2;17;4)(q35;p11.2q25;q25).ishder(X)t(X;17)(wcpX+;
apy and other modalities in children with ALL who have completed wcp17+),der(2)t(X;2)(wcpX+;wcp2+),der(4)t(4;20)(wcp4+;wcp20+),
induction chemotherapy. der(9;20)(wcp9+;wcp20+),der(17)t(2;17;4)(wcp2+;wcp17+;wcp4+).
Methods: All children with ALL admitted in our pediatric oncology unit arr[GRCh37] 9p24.3p13.2(203861_36922488)x1-2,17p13.3p11.2
were included in the study. A retrospective analysis of patient files was (64867_17556523)x2∼<2,20q11.21q13.13(31166986_47908276)x2
done. Data collected on demographic details, risk stratification, chro- ∼<2 . IKZF1plus status was negative. Fusion PAX5::NOL4L4
mosomal analysis, laboratory parameters, pauses in chemotherapy and and mutations NRAS(NM_002524.5):c.35G>A (p.Gly12Asp) and
treatment provided with outcome at the end of induction phase was KRAS(NM_033360.4):c.53C>A (p.Ala18Asp) were found in RNA
done. All data analysed using Excel. sequencing test. Treatment was started according to the current
Results: Total 30 children included in the study. Of which twenty two therapeutic protocol with a good response to the first part of therapy.
(73.3%) were males and eight (26.6%) were females. Most children After completion of the consolidation, the girl was diagnosed with very
belonged to age group of 11- 14 years (46.6%).Fever was the most early relapse. The genetic test revealed the same blast clone in relapse.
common presenting symptom seen in twenty (66.6%) children fol- It was decided to change the treatment according to the IntReALL HR
lowed by pain abdomen in ten (33.3%),petechial rash in seven (23.3% 2010. Disease progression was further observed and the patient was
) and pallor in five (16.6%) children. All children were started in BFM - qualified for blinatumomab. During the 1st cycle of blinatumomab, due
2002 protocol. As per protocol risk stratification twenty one children to the lack of treatment effect, the patient was qualified for CAR-T
(70%) were in standard risk (SR), five children were in intermediate cell therapy. The girl received 3 doses of inotuzumab ozogamicin as
risk (16.6%) and four (13.3%) children were in high risk. Four chil- bridging therapy, followed by CAR-T cell therapy (tisagenlecleucel) on
dren had poor prednisolone response. Fifteen (50%) children were 19.07.2021. About 2 months later the girl developed a CD19-negative
diagnosed with febrile neutropenia during the study. Fourteen (46.6%) relapse. First symptoms of imminent relapse were found on day
children achieved remission after induction, nine (30%) children +28 when 0.02% of cells resembling blasts (though CD19-negative)
failed to attain remission and three (10%) expired during induction were confirmed in FCM. It was decided to give further 2 doses of
therapy. inotuzumab ozogamicin with the aim to obtain CR before MUD-HSCT.
Conclusions: Childhood ALL have a good response if managed prop- Further progression of the disease was observed and all subsequent
erly following BFM protocol. Early detection of febrile neutropenia attempts to cure the child (incl. inotuzumab and clofarabine) failed.
and aggressive management leads to less mortality and favourable The blast count reached 80%. As a result of the progression, the girl
outcome. died on 14.12.2021.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S579 of S635

Conclusions: The complex karyotype with dicentric dic(9;20) indicates Background and Aims: In Ghana, majority of the nurses are gen-
a poor prognosis. eral practitioners. Most of the specialty areas are managed by general
nurses with little or no special training. In instances where it is not prac-
ticable to wish away general nurses from working in a highly specialized
PO029 / #550 OUTCOMES OF LOCALLY MODIFIED ST JUDE field, it is appropriate to explore such nurses’ knowledge on the area
CHILDREN’S XIIIB AND XV PROTOCOLS FOR CHILDREN WITH they are to work and plan appropriately before they commence work.
ACUTE LYMPHOBLASTIC LEUKEMIA AT HOSPITAL MATERNO This study was to explore knowledge on childhood cancers of new
INFANTIL ISSEMYM IN TOLUCA, MÉXICO nurses posted to the child health directorate, Komfo Anokye Teaching
Hospital.
Norma Araceli Lopez Facundo, Isidoro Tejocote-Romero, Cecilia Methods: A cross-sectional study using semi-structured question-
Rodriguez Castillejos naires. 64 nurses posted to the child health directorate in 2019 were
HOSPITAL MATERNO INFANTIL ISSEMYM, Pediatric Oncology, TOLUCA, interviewed.
Mexico Results: 15:49 male to female ratio. 21-30 age group, 54(84%) as
majority respondents. 52(81%) were diploma holders, and 12(18%) had
Background and Aims: The average survival at 5 years in children bachelors. 13(20%) said their training was sufficient to enable them
with acute lymphoblastic leukemia (ALL) in Mexico is 50%, it has been nurse children with cancer. 12 (18.7) said they attended lectures on
related to several factors: the delay in diagnosis and toxicity of treat- paediatric oncology. 50(78%) felt that there is a need for an improve-
ment, . The St Jude Children’s Research Hospital Total Therapy XIIIB ment in paediatric oncology teaching in nursing schools. 15(23%) could
and XV Protocols for Children With Acute Lymphoblastic Leukemia identify three broad classification of childhood cancers. 35(54.6%)
have been modified locally in our hospital in search of better results. thought that leukemia is the most common childhood malignancy glob-
Objective To evaluate the results of The St Jude Total Therapy XIIIB and ally, whilst 20(31.2%) said Burkett lymphoma was the commonest
XV Protocols for Children With Acute Lymphoblastic Leukemia locally childhood malignancy in Africa. 25(39%) responded that childhood
modified in our hospital cancer was diferent from adult cancer. 21(32.8%) said childhood can-
Methods: We included in a cohort, children with ALL diagnosed and cer causes were generally unknown. 48(75%) saw chemotherapy as
treated at our hospital, between . We study demographic, clinical and the commonest treatment modality, 5(7.8%) said surgery, 3(4.6%) said
paraclinical variables as well as those related to treatment, toxicity, radiotherapy whilst 8(12.5%) didn’t know. 10(15.6%) said childhood
evolution and outcome. Descriptive statistics as well as bivariate anal- cancer is curable. 10(15.6%), said it largely could not be prevented,
ysis were performed . Survival and the factors that included it were 62(81.1%) said it could be prevented. 26(40.6%) knew diagnostic
analyzed with Kaplan and Meier process.
Results: We included 130 cases of LAL. 56 women and 74 men, 43% Conclusions: Newly qualified nurses’ luck the basic technical knowhow
over 10 years of age, 23% w, 89% assigned to high risk . 33 children to manage children diagnosed with cancer. Nursing training schools
have received the locally modified protocol XV, 83 the protocol XIII. should introduce paediatric oncology nursing into their training cur-
8.4% (11) Died during induction. We observed overall survival of 70% riculum to enable general nurses have at least the basic understanding
and disease-free 65%.30 patients relapsed, 11 to CNS, 16 to Bone of childhood cancers and its management.
Marrow, 3 of protocol XV vs 25 of protocol XIII.. Overall, disease-free
survival for children included in protocol XV lwas 93 and 90 to 60 and
66 months vs. 64 and 57% at 87 and 77 months, respectively (Log-Rank PO031 / #347 A NOVEL CASE OF FIP1L1-PDGFRA FUSION
of 0.02). Late death was attributed to disease progression in 19, IN A PAEDIATRIC PATIENT PRESENTING WITH B-CELL ACUTE
Conclusions: Llocally modifiedSt Jude Total XV Protocol for Children LYMPHOBLASTIC LEUKAEMIA
With ALLseems to improve survival outcomes and decrease the rate of
early and late relapses. Improvements in the care process are neces- Jenna Nunn, David Deambrosis
sary to improve treatment results in our setting. which is the subject of Queensland Children’s Hospital, Oncology Services, Brisbane, Australia
new studies.
Background and Aims: Philadelphia-like (Ph-like) B-Acute Lym-
phoblastic Leukaemia (B-ALL) is a high-risk subgroup of paediatric
PO030 / #777 EVALUATING KNOWLEDGE OF NEWLY B-ALL. Approximately 10% of Ph-Like patients have ABL class gene
RECRUITED NURSES ON CHILDHOOD CANCER IN A TEACHING fusions, which includes the FIP1L1-PDGFRa rearrangement. To this
HOSPITAL IN GHANA point however, a case of FIP1L1-PDGFRa has not been reported in
Paediatric Ph-like B-ALL. Herein, we describe a paediatric patient with
Barnabas Manlokiya Raymond Ph-like B-ALL with the FIP1L1-PDGFRA fusion.
KOMFO ANOKYE TEACHING HOSPITAL, Child Health Directorate, Methods: Not applicable - case report.
KUMASI, Ghana Results: A previously well, 14-year-old female presented with a
one month history of lethargy, palpitations and fevers. She had
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S580 of S635 ABSTRACTS

pancytopenia at presentation (Hb 45 g/L, WCC 1.7 x10ˆ9/L, platelet during the reinduction period. Hepatotoxicity in the form of hyper-
150 x10ˆ9/L, neutrophil 0.64 x10ˆ9/L) and peripheral blasts (0.03 bilirubinemia and hypoalbuminemia was seen in 22/82(26.8%) and
x10ˆ9/L). Initial peripheral blood flow cytometry demonstrated a pop- 17/82(20.7%) children. Pancreatitis was seen in 7/82(8.5%) with grade
ulation of aberrant B-cell precursors. Bone marrow aspirate (BMA) 4 toxicity in 1 child and others having low grade toxicities. Hyper-
identified a small population of blasts with some atypical morpholog- glycemia and hypertriglyceridemia were seen in 18/82 (21%) and
ical features, and flow cytometry showed a 7% population of cells 18/82 (21%) children respectively.
immunophenotypically consistent with B lymphoblastic leukaemia (B Conclusions: L-Asparaginase toxicities include hypersensitivity, pan-
LL)/lymphoma (B LLy). Given the atypical morphological features, a sec- creatitis, CNS thrombosis, hepatotoxicity, hyperglycemia and hyper-
ond BMA was performed and confirmed the presence of B-ALL with triglyceridemia. Monitoring of biochemical parameters is of paramount
an undifferentiated subclone expressing weak CD19 with absence of importance in LMIC centres for early identification of toxicities to
other markers, of unclear significance. Due to declining status of the guide therapy as assays for L Asparaginase activity may not be feasible
patient and presence of an aberrant B-cell population, she proceeded .
with induction treatment per COG AALL1732. Local ALL molecular
panel was negative for common high risk ALL translocations; how-
ever, FISH identified the presence of a CHIC2 deletion, suggestive of PO033 / #1289 ACUTE NEUROLOGICAL COMPLICATIONS
FIP1L1;PDGFRa fusion, subsequently confirmed on molecular fusion DURING THE TREATMENT OF CHILDREN DIAGNOSED WITH
studies. The patient was in a morphological and flow remission at the ACUTE LYMPHOBLASTIC LEUKEMIA: EXPERIENCE OF A SINGLE
end of induction, though hepatosplenomegaly persists, with Imatinib PEDIATRIC INSTITUTION FROM SERBIA
added during consolidation therapy.
Conclusions: Paediatric B-ALL with a FIP1L1-PDGFRa fusion is Jovana Dimic1 , Dejan Skoric1 , Nada Krstovski1 , Jelena Lazic1 , Predrag
extremely rare and our case expands the clinical and pathological Rodic1 , Goran Milosevic1 , Jelena Krcunovic1 , Jovana Jankovic2 , Olga
experience of such a novel childhood leukaemia. Ilic3
1 University Children’s Hospital, Division Of Hematology And Oncology,
Belgrade, Serbia; 2 University Children’s Hospital, Division Of Neonatology,
PO032 / #1390 L-ASPARAGINASE TOXICITY PROFILE Belgrade, Serbia; 3 University Children’s Hospital, Division Of Education,
DURING PAEDIATRIC LYMPHOBLASTIC LEUKEMIA THERAPY- A Belgrade, Serbia
PROSPECTIVE STUDY FROM SOUTH INDIA
Background and Aims: Acute neurologic events (ANE) during
Nandakumar Radhakrishnan1 , Priyakumari Thankamony1 , Preethi chemotherapy treatment are quite common among children with ALL.
George2 , Manjusha Nair1 , Binitha Rajeshwari1 , Guruprasad Cs1 , ANE may present as peripheral or central nervous manifestation.
Prasanth Vr1 , Kalasekhar Vijayasekharan1 Methods: We retrospectively analyzed data of 99 children diagnosed
1 REGIONAL CANCER CENTRE, Pediatric Oncology, TRIVANDRUM, India; with ALL. Patients were divided into two groups. Group 1 included chil-
2 Regional Cancer Centre, Cancer Epidemiology And Biostatistics, TRIVAN- dren without ANE and group 2 included children who developed ANE.
DRUM, India We compared groups based on age, gender, risk stratification, initial
CNS leukemia involvement, and outcome.
Background and Aims: L-Asparaginase is an integral component in Results: Among 99 patients 24 patients had acute neurologic com-
the induction and the reinduction phases of Paediatric anti-leukemia plications. The most common ANE was vincristine-induced peripheral
therapy. L-Asparaginase associated toxicities include hypersensitivity neuropathy (10 of 24 patients), the second most common ANE were
reactions (including anaphylaxis), pancreatitis, hyperglycemia, hepa- cerebrovascular complications (8 of 24 ANE) including intracere-
totoxicity, encephalopathy, coagulation abnormalities, thrombosis and bral hemorrhages and L-asparaginase-related cerebral venous sinus
hyperlipidaemia. This prospective study analysed the toxicity profile of thrombosis. Cytarabine-related convulsions were registered in 2
L-Asparaginase during Paediatric ALL(Acute Lymphoblastic Leukemia) patients. One patient had peripheral facial nerve palsy due to infection
therapy. (mastoiditis). One patient had cyclophosphamide-induced posterior
Methods: All newly diagnosed ALL children (aged 0-14 years) between reversible encephalopathy syndrome. One patient had cerebritis due
April 2021 and February 2022 were monitored for L-asparaginase to a zygomycosis infection. One patient had multiple lacunar strokes
toxicity during the induction (1st month) and reinduction (5th month) after infusion of high dose methotrexate. There were no significant
phases of treatment with biochemical investigations. The toxicities differences in age, risk stratification, and outcome between the two
were graded according to CTCAE.4 Criteria. compared groups. Group 2 had significantly higher CNS leukemia
Results: Eighty two children received L-Asparaginase during the study involvement compared to group 1. Also, the female gender was a
period. Hypersensitivity was seen in 8/82 (9.7%)patients where 7/82 significant risk factor for ANE.
children were during the reinduction. CNS thrombosis was seen in Conclusions: Besides the well-known side effects of chemotherapy
2/82 (2.4%) where 1 child had symptomatic thrombosis while the other drugs, opportunistic infections are the common cause of neurological
child had an asymptomatic thrombosis that spontaneously recanalised symptoms in our cohort. Some ANE such as intracerebral hemorrhages
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S581 of S635

developed due to multifactorial etiology. In our cohort, patients with ing the last 14 months, also previously detected small lesions weren’t
CNS leukemia at diagnosis had a significantly greater risk to develop present.
ANE (including both peripheral and CNS manifestation). Considering Conclusions: ALL rarely presents with an intracranial mass, which can
the high incidence of ANE among children diagnosed with ALL, further lead to diagnostic problems. Our goal is to focus specialists’ attention
studies are needed in order to maximize the recognition, prevention, on this type of ALL presentation.
and treatment of neurological complications.

PO035 / #935 CML BLAST CRISIS (CML-BC) OR PH (P210)


PO034 / #1724 UNUSUAL PRESENTATION OF ACUTE POSITIVE B-ACUTE LYMPHOBLASTIC LEUKEMIA (PH+VE
LYMPHOBLASTIC LEUKEMIA AS A SKULL BASE MASS B-ALL)? THE UNRESOLVED ENIGMA

Clodet Stepanians1 , Julieta Hoveyan2 , Saten Hovhannisyan3 , Mery Kalasekhar Vijayasekharan, Ajay Sankar, Prasanth Vr, Guruprasad Cs,
Petrosyan4 , Mane Gizhlaryan2 , Naira Martirosyan3 , Anahit Binitha Rajeshwari, Manjusha Nair, Priyakumari Thankomany
Zakharyan4 , Medea Anastasiadi5 , Gevorg Tamamyan3 , Lala Regional Cancer Centre, Department Of Pediatric Oncology, Thiruvanan-
Vagharshakyan6 thapuram, India
1 Hematology center after professor R. Yeolyan, Pediatric Oncology And
Hematology, Yerevan, Armenia; 2 Hematology Center after Prof. R.H.Yeolyan, Background and Aims: CML blast crisis(CML-BC) can masquerade as
Pediatric Cancer And Blood Disorders Center Of Armenia, Yerevan, Armenia; Ph-positive B-Acute Lymphoblastic Leukemia(B-ALL),usually identified
3 Hematology Center after R.Yeolyan, Pediatric Cancer And Blood Disorders in retrospect, when the patient relapse with CML after ALL chemother-
Center Of Armenia, Yerevan, Armenia; 4 Department of Pediatric Oncology apy. Although transcript typing at ALL diagnosis(P190 Vs P210) may
and Hematology, Yerevan State Medical University After M. Heratsi, Yere- give a clue to CML-BC, differentiating it from Ph-positive(p210) B-ALL
van, Armenia; 5 Hematology center after prof. R. Yeolyan, Pediatric Cancer has often been difficult. Also,management approach to such Ph pos-
And Blood Disorders Center Of Armenia, Yerevan, Armenia; 6 Yerevan State itive(p210) B-ALL is not well defined. We report here a case of Ph+
Medical University, Department Of Pediatric Oncology And Hematology, B-ALL who relapsed two years post ALL therapy with CML-Chronic
Yerevan, Armenia phase(CML-CP).
Methods: Retrospective audit of medical records of this patient was
Background and Aims: Central nervous system involvement in done. At our centre,Ph positive B-ALL patients received Very high risk
leukemia is usually manifested as a leptomeningeal disease. Leukemia arm of modified BFM-95 ALL protocol. Imatinib(300mg/m2/day) was
intracranial masses are rare and can be a diagnostic challenge. added concurrently to chemotherapy from Phase-1b consolidation till
Methods: We report a case of a 12-years-old girl with a skull base mass end of maintenance chemotherapy.
as the initial manifestation of ALL. Results: A 4-year-old female propositus who presented to us with
Results: The first complaints of the patient were strabismus, diplopia, fever, generalized lymphadenopathy, hepatosplenomegaly, had leuco-
and headaches. CT and MRI examinations revealed a pathological cyte count of 13500/mm3 including 49% blasts in peripheral smear.
mass at the base of the skull (3.5x4x3cm and 5.7x2.4x4.6cm in size Her marrow flow cytometry was suggestive of pre B-ALL and FISH
respectively). She was referred to a pediatric oncologist and a CNS study revealed BCR-ABL fusion in 90% of cells, confirming Ph+ve
tumor was initially suspected. Unexpectedly, CBC with peripheral B-ALL. She had good response to modified BFM-95 regimen with ima-
blood smear showed 9% blast cells. Bone marrow (BM) aspiration tinib. After a treatment free interval of 26months, she developed unex-
detected 56% blasts. Immunophenotyping showed positive expression plained Leucocytosis(WBC-54000/mm3) with peripheral smear show-
for CD19, HLA-DR, CD38, CD79a, TdT, and CD22. The FISH analysis ing myeloid left shift, basophilia and eosinophilia. Her bone marrow
found IGH gene (14q32) tripling in 30% of blasts and hyperdiploidy was in morphologic remission at this point, however peripheral blood
(47-52). Pro-B ALL was diagnosed. CSF was without abnormalities. and marrow RT-PCR identified p210 BCR-ABL fusion transcripts, con-
Considering the neurological symptoms and detected mass she was firming baseline CML-BC with child remaining in Second CML-CP. She
evaluated as CNS positive. Chemotherapy was started according to is currently on Dasatinib and being worked up for stem cell transplant
ALL-IC-BFM 2009 protocol. Hematologic remission was documented. (HSCT).
After the IA protocol patient’s neurological symptoms got worsened. Conclusions: It is important to differentiate CML-BC from Ph+B-
CT showed an increased size of the mass (5.5x3.0x4.0cm) and new ALL,as the standard treatment being HSCT at first remission in
small skull lesions. Skull lesion biopsy with histological and immuno- case of CML-BC,while majority of Ph+ B-ALL can be cured with
histochemical examinations indicated reactive/myelodysplastic bone chemotherapy and TKI combination alone. Presence of P210 BCR-ABL
marrow changes. The patient continued to receive chemotherapy transcript,marked splenomegaly, basophilia,myelocyte bulge at pre-
according to the same protocol, followed by radiotherapy and reached sentation,low LAP score and detection of BCR- ABL1 fusion in other
maintenance therapy. Control MRI has shown that the size of the lineages at remission may points towards CML-BC rather than a Ph
mass has decreased (1.5x2.4x2.6cm) and remained unchanged dur- positive B-ALL.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S582 of S635 ABSTRACTS

PO036 / #1030 TREATMENT ABANDONMENT IN CHILDREN Poznan, Chair And Department Of Physical Pharmacy And Pharmacokinet-
WITH ACUTE LYMPHOBLASTIC LEUKEMIA - AN EXPERIENCE ics, Poznan, Poland; 3 Medical University of Lublin, Laboratory Of Genetic
FROM INDIA Diagnostics, Ii Chair Of Paediatrics, Lublin, Poland

Anjali Yadav, Dhwanee Thakkar, Upasana K, Sunisha Arora, Neha Background and Aims: Systematic fungal infection is a common com-
Rastogi, Satya Yadav plication among children treated because of cancer. We report a case of
Medanta- the Medicity, Pediatric Hemat-oncology And Bmt, Gurgaon sector, a 14-year-old girl who has been admitted to the hospital in Lublin due
India to suspected leukemia.
Methods: On admission, the patient’s condition was severe. A blood
Background and Aims: Pediatric ALL is highly curable and outcomes test showed hyperleukocytosis, an X-ray of chest presented a massive
have improved with better stratification, treatment opportunities and tumor (size 17 x 12 cm) in the mediastinum, live-threatening heart tam-
good supportive care. However, treatment abandonment is the first ponade and pleural fluid. Based on bone marrow examination the girl
obstacle to successful outcomes in these patients. Several known fac- was diagnosed with T-cell acute lymphoblastic leukemia.
tors for treatment abandonment include high cost of treatment, lack Results: The treatment was started according to the current therapeu-
of universal health insurance, travel distance from home to medical tic program. As antifungal prophylaxis the patient received Micafungin.
center, popular belief of lack of long term cure for cancers and belief The induction phase was complicated as follows: tumor lysis syndrome,
in alternate treatments. We describe our experience of treatment massive lower gastrointestinal bleeding, hypovolemic shock, seizures,
abandonment and their outcomes. PRES syndrome, COVID-19 infection, and fungal pneumonia. There-
Methods: Available medical records were analyzed and we attempted fore, AmBisome instead of Micafungin was used for the treatment.
to contact the parent/attendant of the patient who abandoned treat- After the 42nd Day of Protocol I, the patient developed deep aplasia of
ment. Abandonment was classified as Early if treatment was aban- bone marrow, a high fever accompanied by severe pain and swelling of
doned in Induction and late if it was later. the knees and right elbow joints. Laboratory tests showed high inflam-
Results: Eighty-nine patients were diagnosed with Acute Lymphoblas- matory parameters. An ultrasound examination showed fluid in the
tic Leukemia (ALL)/Lymphoblastic Lymphoma (LL) between June2016- above-mentioned joints. It was decided to perform a puncture of the
December2021. Eighteen out of Eighty-nine patients (20%) abandoned knees and elbow joints, and the collected fluid was sent for micro-
treatment. Age range 9m-17y (median - 9y) with M:F::2:1. Fourteen biological tests. The result of joints fluid and urine culture showed
were Precursor B-cell (Pre-B) ALL and remaining were T-cell ALL/LL. Trichosporon asahii pathogen. Subsequently, a CT of the chest revealed
Ten patients could be contacted telephonically for health status & out- a new finding described as a subpleural lesion in lungs. Instead of AmBi-
come. Of the 10 patients who could be contacted, 1/10 had opted some, the girl started Voriconazole therapy. The child tolerated therapy
for alternate treatment and succumbed 3 months from diagnosis, well, and the serum drug concentration is monitored.
9/10 chose to take treatment at another center (alive-4, death-5 at Conclusions: Due to the increased availability of antifungal drugs, the
last follow-up). Among the 6 patients who died, TRM-3, progressive incidence of opportunistic fungal infections is rapidly increasing. A
disease-3. Treatment abandonment was Early- in 12/18, late in 6/18. review of the literature report how rare Trichosporon asahii is. By
Cause of abandonment: 11/18-long travel distance or personal choice, 2020, over 200 cases have been described. Most of the patients were
5/18- financial constraints, 2/18 patients didn’t share the reason. treated oncologically and an infection developed during prolonged
Conclusions: Outcomes of pediatric ALL have improved with con- neutropenia.
temporary treatment protocols. Extension of Universal health cov-
erage schemes across the entire eligible population based economic
stratification, deployment of trained Pediatric Oncologist in all State- Publication Only Topic: AS05 SIOP Scientific programme / AS05.b
supported and Private healthcare set-ups, uniform implementation Myeloid Leukemias, Myelodysplastic and Myeloproliferative Syndromes
of effective treatment protocols which have improved outcomes and
bridging the mental barrier to opt and continue treatment can help PO038 / #955 FEBRILE NEUTROPENIA IN PEDIATRIC
reduce abandonment and improve outcomes. LEUKEMIA: LABORATORY PROFILE AND OUTCOME

Muhammad Bahar, Stefanus Gunawan, Max Mantik, Natharina


PO037 / #520 INVASIVE TRICHOSPORON ASAHII Yolanda, Ernestine Sadeli
INFECTION IN A 14-YEAR-OLD GIRL WITH T-CELL ACUTE Sam Ratulangi University, Department Of Child Health, Manado, Indonesia
LYMPHOBLASTIC LEUKEMIA – CASE REPORT AND REVIEW
Background and Aims: Febrile neutropenia is the most com-
Joanna Zawitkowska1 , Marta Malczewska1 , Matylda Resztak2 , mon severe and common complication of cancer therapy. It
Monika Lejman3 occurs when a neutropenic patient encounters an infectious
1 Medical University of Lublin, Department Of Paediatric Haematology, pathogen due to an immunocompromised state. This study
Oncology And Transplantology, Lublin, Poland; 2 Medical University of aims to describe laboratory profiles and outcomes of febrile
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S583 of S635

neutropenia in pediatric leukemia treated at a middle-income based on International Society of Pediatric Oncology (SIOP) proto-
country. col for developing countries and supportive treatments. One patient
Methods: Medical record of leukemic children with febrile neutrope- had died before starting chemotherapy. Out of treated patients, 72%
nia treated at Estella Pediatric Cancer Center, Manado, Indonesia, was had died, 14% had dropped out, and 14% had survived. Most of the
prospectively analyzed from January 2019-December 2021. Febrile deceased patients (38%) died in induction phase of chemotherapy, 31%
neutropenia was defined as a single oral temperature ≥38.5o C, or a had relapse episode, 24% died in pre-phase of chemotherapy, 7% had
temperature ≥38o C for at least an hour, with an absolute neutrophilic survived.
count of less than 1500 cells/microliter. Conclusions: Mortality rate of pediatric AML patients treated with
Results: Out of 35 cases of febrile neutropenia, 20 cases (57%) were SIOP protocol in developing countries is still high.
male, and 15 cases (43%) were female. Mean age of our subjects were
6 years old. Most of them were acute lymphoblastic leukemia (80%).
The mean value of white blood cell count was 6,899 (300 – 64,100) PO040 / #1560 ACUTE PROMYELOCYTIC LEUKEMIA IN A
cells/microliter, and mean value of absolute neutrophil count was 870 SINGLE REFERRAL CENTER IN NICARAGUA
(80 – 1426) cells/microliter. C-reactive protein as an inflammatory
marker was tested, with mean value of 22.6 (6 – 48) microgram/liter. Wangky Carrasco
Cumulative episodes of febrile neutropenia from all of our cases were hospital civil de guadalajara, Onco Hemato Pediatria, Guadalajara, Mexico
54 episodes; it varied between 1 to 4 episodes per subject. Positive
blood culture was found in 6% of cases. First-line empirical antibiotic Background and Aims: Acute promyelocytic leukemia (APL) is a
treatment (ceftriaxone and gentamicin) showed response in 77.1% of rare condition, refers to a different type of acute myeloid leukemia,
subjects. As much as 23% of subjects did not show clinical response patients are at high risk of hemorrhage. Pooled analysis revealed
after 72 hours of first-line antibiotics, hence escalation to meropenem. incidence in developed countries <2% in contrast with low middle
Almost half (46%) of patients had died; 22% were declared as survivors, income countries in which account an overall incidence 20-25%. Usu-
20% were currently on maintenance phase, 6% were dropped out, and ally involves the translocation 15,17 and PML-RARA. It usually tends
6% had relapsed and currently on chemotherapy. to have better treatment response to trans retinoic acid (ATRA).
Conclusions: Blood culture from febrile neutropenic patients had low To best our knowledge Nicaragua previous study reported a high
positivity rate. Most febrile neutropenic patients responded well to incidence APL almost 50%, there are no recent reports of APL in
first-line antibiotic treatment with ceftriaxone and gentamicin. Nicaragua
Methods: retrospective review of charts
Results: An estimated of 500 childhood leukemia were identify from
PO039 / #958 CHARACTERISTIC AND OUTCOME OF ACUTE January 2015 and November 2019, 21 patients were diagnose with
MYELOID LEUKEMIA AT A PEDIATRIC CANCER CENTER IN APL with an overall incidence of 4.2%. Of the 21 cases identify, 11
INDONESIA (52.4%) were male and 10 were female with a ratio of 1:1.1. the patients
age ranged from 1 to 14 years, by group of age the most affected were
Muhammad Bahar, Stefanus Gunawan, Max Mantik, Natharina between 10-14 years which accounts 12 patients (57.1%). The overall
Yolanda, Ernestine Sadeli mortality rate was 52.4%, 7 patients died due to intracranial and pul-
Sam Ratulangi University, Department Of Child Health, Manado, Indonesia monary hemorrhage, 3 died from secondary sepsis and in 10 the cause
of death was not determined.
Background and Aims: Acute myeloid leukemia (AML) compromises Conclusions: the overall incidence of APL in our study is slightly higher
15-20% of pediatric acute leukemias. Probability of survival in AML has than the reports of developed countries, in line with other studies
increased in developed countries over the past decades. This improve- we found and elevated mortality rate most due to hemorrhage com-
ment of survival may be lower in developing countries may be due plications, however, further studies are needed to determine specific
to lack of essential resources for supportive care, option of intensive predisposition factor for APL in Nicaragua.
care, higher rates of infections and unavailability advanced treatment
options. This study aims to describe the characteristic and outcome of
pediatric AML treated at a middle-income country. PO041 / #1797 CHARACTERISTIC AND OUTCOME OF
Methods: Medical record of pediatric AML patients treated at Estella NILOTINIB IN CHILDREN WITH IMATINIB RESISTANT CHRONIC
Pediatric Cancer Center, Manado, Indonesia was prospectively anal- MYELOCYTIC LEUKEMIA IN CIPTO MANGUNKUSUMO
ysed from January 2019-December 2021. We extracted data of sex, HOSPITAL, INDONESIA
age, diagnosis, treatment, and outcome of pediatric AML.
Results: Out of 29 cases of AML, 15 cases (52%) female male and Ganda Ilmana, Ludi Dhyani Rahmartani
14 cases (48%) were male. Mean age of our subjects were 6 years Faculty of Medicine, Universitas Indonesia, Department Of Child Health,
old. There were 10.3% of our AML patients who had Down syn- Central Jakarta, Indonesia
drome. Twenty-eight patients (96%) were treated with chemotherapy
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S584 of S635 ABSTRACTS

Background and Aims: Tyrosine kinase inhibitors have been approved at diagnosis and the third was 2 years old. Two were males and
for standard treatment in chronic myeloid leukemia (CML) in chil- one female. All patients had a fever, hepatosplenomegaly, thrombo-
dren. However, access to TKIs drugs and quality-assured laboratory cytopenia, monocytosis >1,000/uL, and bone marrow histopathology
diagnoses has been challenging in Indonesia. This report describes consistent with JMML. Mutational analysis showed somatic KRAS
the clinical characteristics and outcomes of children with imatinib- mutations in two patients [(i) c.451-3C>T, exon 4, allele frequency
resistant CML treated with nilotinib in Cipto Mangunkusumo Hospital, 49.1%; (ii) p. Gly12Val, exon 2, allele frequency 29.2%] and no muta-
Indonesia. tions in the third patient; the latter had received Ara-C elsewhere
Methods: The inclusion criteria are children with CML who are resis- before the test. The 1-year-old male with the somatic KRAS mutation
tant to imatinib, based on hematological and molecular response. at exon 4 underwent spontaneous remission and remains so for the last
Results: There are four eligible patients with good adherence to TKI 3 years. The other two required 2 cycles of low dose Ara-C and 6-MP
treatment. AF (4 yo. boy), VT (15 yo. girl), AT (17 yo. girl), and HA (17 followed by a haplo-HSCT from a 5/10 HLA matched mother for one
yo. boy). At initial presentation, all had splenomegaly (Schuffner 3 to 6), patient and a 7/10 HLA matched father for the other. One patient had
all were anaemic (Hb of 7.1 - 9.9 g/dL), and all had high leucocyte count positive donor-specific antibodies and received desensitization with
(37.760-488.700). The peripheral blast was detected up to 9%. The rituximab and plasmapheresis. Conditioning was with Bu-Flu-Mel fol-
bone marrow aspirations were in accordance with CML, either in the lowed by PTCY. Both patients engrafted, experienced no significant
chronic or accelerated phase. At the time of diagnosis, BRC-ABL muta- chemotherapy-induced toxicity, are in remission for 11 months and 4
tions were detected in all patients. After months of imatinib (range 3 – months respectively, receiving decitabine maintenance therapy from 3
14), we evaluated the BCR-ABL quantitatively and found that AF was months post-HSCT.
86%, AT was 43%, VT was 44%, and HA was 58%. Nilotinib was started Conclusions: JMML can have variable outcomes based on the disease
with dose ranged between 150-230 mg/m2 twice daily with mild to genotype. Haplo-HSCT with busulfan-based conditioning and PTCY is
moderate side effects (headache, gastro-intestinal problem, itching and well tolerated. Post-HSCT maintenance therapy with hypomethylating
bone pain). QT prolongation and hepatotoxicity were observed in two agents may produce sustained remission.
patients (VT and HA). One patient (ATP) died after three months of
using nilotinib due to a blast crisis; others survived and continued the
treatment with relatively normal leucocyte count, but thrombocytosis, PO043 / #1890 GEMTUZUMAB OZOGAMICIN FOR
with no symptoms. RELAPSED/REFRACTORY ACUTE MYELOID LEUKEMIA IN
Conclusions: Nilotinib showed better response in our patients com- CHILDREN – SINGLE CENTER EXPERIENCE
pared to imatinib. The dose used was varied from 150-250 mg/m2
twice daily. Common side effects were manageable by monitoring and Katarzyna Pawinska-Wasikowska1,2 , Malgorzata Czogala1,3 , Karolina
dose reduction. Bukowska-Strakova4 , Teofila Książek5 , Szymon Skoczen1 , Walentyna
Balwierz1
1 Institute of Pediatrics, Jagiellonian University Medical College, 31-663
PO042 / #1224 TREATMENT OF JUVENILE Krakow, Poland;, Department Of Pediatric Oncology And Hematology,
MYELOMONOCYTIC LEUKEMIA WITH LOW DOSE Krakow, Poland; 2 Department of Oncology and Hematology, University Chil-
CHEMOTHERAPY AND HAPLO-IDENTICAL HEMATOPOIETIC dren’s Hospital Of Krakow, Krakow, Poland; 3 University Children’s Hospital
STEM CELL TRANSPLANT of Krakow, Department Of Children’s Oncology And Hematology, Krakow,
Poland; 4 Institute of Pediatrics, Jagiellonian University Medical College,
Arjun Mandade, Rasmi Palassery, Santhosh Devadas, Vinayak Maka, Department Of Clinical Immunology, Krakow, Poland; 5 Institute of Pedi-
Swaratika Majumdar, Nalini Kilara atrics, Jagiellonian University Medical College, 31-663 Krakow, Poland;,
MS Ramaiah Medical College, Medical Oncology, Bengaluru, India Department Of Molecular Genetics, Krakow, Poland

Background and Aims: Juvenile Myelomonocytic Leukemia (JMML) is Background and Aims: Gemtuzumab ozogamicin (GO), one of the
a rare and aggressive childhood myeloproliferative disorder. Cytore- first targeted drugs used in oncology, consists of an anti-CD33 mono-
ductive therapy if required, with low-dose cytarabine (Ara-C), 6- clonal antibody bound to a cytotoxic calicheamicin derivative. After the
mercaptopurine (6-MP), or azacytidine, followed by hematopoietic drug was withdrawn in 2010 due to a significantly higher rate of early
stem cell transplantation (HSCT) is the only curative therapy. Here, we deaths, GO regained approval in 2017 for the treatment of adults and
describe our experience of treating JMML with low-dose chemother- children with refractory or relapsed acute myeloid leukemia (AML) and
apy followed by a haploidentical HSCT (haplo-HSCT) with Busulfan- de novo AML.
Fludarabine-Melphalan (Bu-Flu-Mel) conditioning and post-transplant The aim of the study is to retrospectively analyze the effects of
cyclophosphamide (PTCY). treatment and the GO toxicity profile in children with recurrent or
Methods: Case series refractory AML.
Results: Three children were diagnosed with JMML and received care Methods: From January 1, 2010 to February 28, 2022, ten children
at our center between 2018 to 2021. Two patients were a year old received treatment in the Department of Pediatric Oncology and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S585 of S635

Hematology, Institute of Pediatrics, Jagiellonian University Medical urine culture 50% (6/12), fecal culture 100% (10/10), and secretes or
College, (5 girls and 5 boys, mean age 11.6 years). Primary resistance wound based culture 75% (3/4). The majority of bacteria detected was
to treatment was the indication for the use of GO in 3 children, first gram-negative in 26 out of 35 cultures (74%) and among them were K.
relase of AML in 5 children, and the second recurrence in 2 children. pneumonia (8/35, 23%) and E. coli (7/35, 20%). Candida albicans was
Nine children had more than 5% bone marrow leukemia blasts (4.6- detected in 1 patient (3%).
94.5%; median 22%) before starting GO therapy. Most patients (8/10) Conclusions: Infections in children with AML in our center was the
received 1 GO administration in combination with chemotherapy main cause of death. Infections mostly occurred during induction.
(IDA-FLA, FLA, FLAG). The most common causative agent was gram negative bacteria. Fur-
Results: Of the 10 patients, six (60%) achieved a significant reduction in ther research into infection prophylaxis seems of utmost importance
the number of bone marrow leukemic blasts (1.5-5%, median: 1%) with to improve the outcome for children with AML in resource limited
GO, allowing bone marrow hematopoietic cell (SCT) transplantation. settings.
Four children did not respond to GO treatment. Two of the six patients
with a good response to treatment had a rapid relapse after SCT. Four
patients (40%) live in the first or subsequent remission of the disease. PO045 / #1558 PHENOTYPIC PROFILE OF DOMINANT
No significant life-threatening toxicity was observed. CLONE-GUIDED THERAPY IN ACUTE LEUKEMIA OF
Conclusions: The use of GO in severely pretreated children with pre- AMBIGUOUS LINEAGE
vious failures of AML treatment is a possible and effective bridging
therapy to SCT, with an acceptable toxicity profile. Tatiana Soultana Tziola1 , Georgia Avgerinou1 , Elena Solomou1 , Ilona
Binenbaum1 , Maria Filippidou1 , Marianna Tzannoudaki2 , Stefanos
Papadhimitriou3 , Anna Komitopoulou4 , Katerina Katsibardi1 , Stavros
PO044 / #1099 HIGH INFECTION-RELATED MORTALITY IN Glentis1 , Kleoniki Roka1 , Efthymia Rigatou1 , Antonia Vlachou1 ,
CHILDHOOD ACUTE MYELOID LEUKEMIA IN LIMITED Evgenios Goussetis4 , Antonis Kattamis1
RESOURCES SETTING: EXPERIENCE WITH THE INDONESIAN 1 “Aghia Sophia” Children’s Hospital, Division Of Pediatric Hematology
NATIONAL PROTOCOL Oncology, First Dpt Of Pediatrics, National & Kapodistrian University Of
Athens, Athens, Greece; 2 Aghia Sophia Children’s Hospital, Department
Eddy Supriyadi1 , Inggar Armytasari1 , Ignatius Purwanto1 , Bambang Of Immunology And Histocompatibility, Athens, Greece; 3 General Hospi-
Ardianto1 , Pudjo Widjajanto1 , Gertjan Kaspers2 tal of Athens "G. Gennimatas", Department Of Laboratory Hematology,
1 Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada Athens, Greece; 4 Aghia Sophia Children’s Hospital, Stem Cell Transplant
- Dr Sardjito Hospital, Department Of Child Health, Pediatric Hematol- Unit, Athens, Greece
ogy Oncology Division, Yogyakarta, Indonesia; 2 Princess Máxima Center,
Pediatric Oncology, Utrecht, Netherlands Background and Aims: Acute Leukemia of Ambiguous Lineage (ALAL)
represents a rare type of leukemia, comprising 2-5% of cases. Lacking
Background and Aims: In 2012, Indonesian National protocol was an accepted definition along with two separate classification sys-
developed to treat children with acute myeloid leukemia (AML). tems (EGIL and WHO 2008/2016) makes the therapeutic approach
Infection-related mortality (IRM) in Indonesia is considered high com- challenging.
pared to high-income countries. The objective of this study is to Methods: Medical records of patients with ALAL, admitted to our
describe the characteristics and time of occurrence of infections and department the last decade were retrospectively reviewed. Patients’
IRM in children with AML treated according to the Indonesian National characteristics such as age, sex, peripheral blood count at diagnosis,
AML protocol. blasts’ morphology were reviewed along with immunophenotyping,
Methods: Medical records data of 113 pediatric patients with AML cytogenetic and molecular studies. Data regarding treatment proto-
treated from April 2012–September 2018 at Dr. Sardjito Hospi- cols, achievement of complete remission (CR), use of Hematopoietic
tal (Yogyakarta, Indonesia) according to the National Protocol were Stem Cell Transplantation (HSCT), relapse rates and overall (OS) and
reviewed. Basic patient characteristics, type of infections, time of IRM, event-free survival (EFS) were obtained.
and cause of death were recorded. Results: Among 201 children with acute leukemia, 10 patients (6
Results: 95 out of 113 patients with AML met the inclusion criteria. male – 2 infants), with a median age of 5 years (range 0-16), met
Out of 95 patients, 81(85%) suffered from infection with in total 144 the criteria of ALAL. Biphenotypic blast populations were observed
episodes (mean 1.8/patient). Death was seen in 60(63%) patients with in 4 patients and bi-linear in 4 patients; B/My (3 patients), T/My (1
IRM as the cause in 31 patients (33%). The IRM in induction was 45% patient). Two patients presented early switch from ALL to AML M5.
(14/31) patients. The majority of infections was in the gastrointesti- Five patients received initially ALL-directed therapy, with 3 of them
nal tract (57/144, 40%), sepsis (36/144, 25%), and respiratory tract showing persistent myeloid-type blast population after induction, lead-
(33 out of 144, 23%). Infections mostly occurred in the first induction ing to switching to AML-directed therapies, which induced remission.
cycle (50/144, 35%). Culture was done in 85 episodes, and the posi- All patients underwent HSCT at CR1. Five patients started treatment
tivity of culture was recorded as follows: blood culture 27% (26/35), according to AML protocols with all achieving CR and proceeding to
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S586 of S635 ABSTRACTS

HSCT either at CR1 (5 patients) or CR2 (1 patient). Two patients died Publication Only Topic: AS05 SIOP Scientific programme / AS05.c
due to toxicity during HSCT. One patient relapsed and underwent a Lymphomas
second HSCT. One patient presented with extramedullary (testicular)
relapse after HSCT. With a median follow-up time of 3.4 years (range PO047 / #1916 A PARTICULAR PRESENTATION OF
0.5-9), the 3-year OS and EFS are 88.9% and 71.1%, respectively. HODGKIN’S LYMPHOMA IN AN ADOLESCENT: A CASE REPORT
Conclusions: Up to today the optimal treatment approach for this
type of leukemia remains unclear. As shown in our study, using AML Nabila Bouterfas, Nabila Dahmane, Khalida Makhlef, Ayda
protocols and a continuous re-evaluation appears the most promising Mohand-Oussaid, Assia Slimani, Keltoum Nafissa Benhalla
strategy. Faculty of Medicine of Algiers, Medecine, Rue Lieutenant Mohamed Benarfa,
El Biar, Algeria

PO046 / #515 CONSERVATIVE MANAGEMENT OF Background and Aims: Haemophagocytic lymphohistiocytosis HLH is
NEONATES WITH ACUTE MYELOID LEUKEMIA BUT WITHOUT described as a rare complication of lymphohematopoietic malignancies
DOWN SYNDROME and lymphoma especially. It’s associated with a high mortality rate.
Methods: We report a case of a 13 –year- old male presenting
Jacquelyn Klicka-Skeels, Kazuhiro Sabet, Nikolaos Svoronos, Shana with a HLH revealing a Hodgkin lymphoma HL who developed a
Jacobs, Reuven Schore, Birte Wistinghausen splenic rupture and cholecystitis, successfully treated with conven-
Children’s National Hospital, Center For Cancer And Blood Disorders, tional chemotherapy.
Washington, United States of America Results: A 13-year-old male was admitted for acute respiratory dis-
tress and general alteration, with a 12 month history of fever and
Background and Aims: Neonatal acute myeloid leukemia (AML) is rare weight loss. Physical examination revealed a large right pleural effu-
and most commonly associated with Down Syndrome (DS) and somatic sion with hepatomegaly. Imaging explorations confirmed the pleu-
GATA1 mutations, then called transient abnormal myelopoiesis (TAM). ral effusion with the presence of multiple profound lymph nodes
While watchful waiting is the accepted standard of care (SOC) for DS and nodular hepatosplenomegaly. Laboratory data were compatible
TAM, there is no SOC for neonates without DS. We describe three with HLH (Pancytopenia, hyperferritinemia, hypertriglyceridemia and
unique cases of neonatal AML treated with watchful waiting. increased CD25 plasmatic level) with evidence for coagulopathy. After
Methods: Case series excluding tuberculosis, data were promptly suggestive of HL and sev-
Results: Neonate A born at 33 weeks of gestation presented with eral biopsies were realized but inconclusive. During hospitalization,
thrombocytopenia and peripheral blasts of megakaryocytic lineage, the patient experienced acute abdominal pain with icterus. Imag-
but negative for trisomy 21 or GATA1 mutation. Because of prema- ing findings were compatible with splenic rupture and cholecystitis.
turity and absence of any end organ toxicity, watchful waiting was Management consisted of platelet and globular transfusions, antalgics
initiated. Peripheral blasts spontaneously resolved by 3 months of age. and antibiotics. No surgery was performed. However, chemotherapy
At 14 months of age, she developed worsening pancytopenia with was started according to HL protocol with significant clinicobiologi-
peripheral blasts and AML was diagnosed. She received chemotherapy cal improvement. Finally, diagnosis was confirmed and chemotherapy
according to current Children’s Oncology Group protocols and remains was continued. The patient is alive with a follow up time of 18 months
in remission 12 months off therapy. Neonate B had hyperleukocyto- without disease evidence.
sis and peripheral blasts of megakaryocytic lineage. Trisomy 21 and Conclusions: Secondary Haemophagocytic lymphohistiocytosis in
an inactivating GATA1 mutation was restricted to the blast popula- Hodgkin lymphoma is a rare but serious event making diagnosis of
tion. Over the first 6 weeks of life leukocytosis and peripheral blasts Lymphoma more difficult.
resolved without intervention. Clinical picture is consistent with TAM
in a non-DS patient and watchful waiting is continuing. Neonate C was
born with multiple nodular skin lesions with normal blood counts and PO048 / #2021 RARE PEDIATRIC LINFOMA PRESENTATION
differential. Lesional biopsy demonstrated acute myeloid sarcoma with
low-level KMT2A gene rearrangement. Bone marrow evaluation was Marcos Feldman1 , Daniel Cisternas1 , Alejandra Perez1 , Maricruz
without evidence of leukemia, though FISH analysis showed borderline Ormeno1 , Milena Villarroel2
KMT2A rearrangement. While completing the work-up, lesions were 1 Hospital Luis Calvo Mackenna, Oncology, Santiago, Chile; 2 Hospital Luis
starting to regress and watchful waiting is continuing. Calvo Mackenna, Pediatric Oncology, Santiago, Chile, Pediatric Oncology,
Conclusions: Watchful waiting for neonates without DS with clin- Santiago, Chile
ical presentations otherwise consistent with TAM or isolated
acute myeloid sarcoma avoids toxicity of chemotherapy in the Background and Aims: Here’s an unusual presentation of four difer-
neonatal period and allows time for spontaneous resolution and/or ent tipes of pediatric linfoma. Twelve years old, male, no personal or
delaying chemotherapy until a later age with better chemotherapy family history, consulted after 3 motnhs of increased volume of right
tolerance. parotid region. Imaging study is performed, soft tissue ultrasound and
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ABSTRACTS S587 of S635

CT scan compatible with 2 cm nodule on the right parotid. No other sig- diagnosis, clinical manifestations, complications and evolution were
nificant findings. Surgicaly removed, biopsy confirms a marginal zone specified.
lymphoma. International consultation in the Latin American commit- Results: We reported 14 cases of Burkitt Lymphoma. Regarding gen-
tee of lymphomas sugged clinical and imaging follow-up. Nine years old der, 10 (71%) were male and 4 (29%) female. The average age at
male patient who had a amigdalectomy and adenoidectomy in 2014. diagnosis was 7.9 years old. The most frequent clinical manifestations
In 2019 presents left tonsillar hyperplasia. Bleeding tumor is resected were: abdominal pain (n = 10; 71%), lymph node enlargement (n = 3;
from the base of tongue, Biopsy infomrs a diffused Large B Cell Lym- 21%) and vomiting (n = 3; 21%). Of the 14 cases, 10 (71%) reported
phoma. RNM and CT show a solid tumor at the left tongue’s base and abdominal mass at diagnosis. The average time elapsed between symp-
tonsil. Etapification study shows inespecific bilateral and small lungs tom onset and demand for care was 22 days, with 7 (50%) patients
nodules. PET CT is also compatible. Patient starts chemotherapy hav- diagnosed within the first 15 days after symptom onset, 3 (21%) within
ing a good response. Fourteen years old male patient, with no previos the first 30 days, and 4 (29%) over 30 days. Regarding the clinical
history. Initiates with dysphonia, dysphasia, bipalpebral edema, fever, diagnosis, 10 (71%) presented advanced stage (III/IV) at diagnosis.
weight loss, erythematous-violaceous skin lesions, neuropathic pain, Six (43%) patients develop tumor lysis syndrome at the beginning of
facial edema, hepatomegaly and muscle weakness, hypertransami- treatment. Remission induction was achieved in 9 (64%) patients.
nasemia, hypergammaglobulinemia, low-complement and positive anti Conclusions: Given the results presented, it is concluded that efforts
Jo 1 antibodies, low T-Lymphocites, positive EBV-PCR. Iimmunodefi- are extremely important in an attempt to reduce the deaths of chil-
ciency with lymphoproliferative syndrome is considered. Skin biopsy dren with BL in the western region of Paraná, through measures such
informs Hydros Vacciniforme-type lymphoproliferative disorder sec- as diagnosis in earlier stages, since most patients had a more advanced
ondary to active chronic Epstein Barr Virus. Fifteen year-old male, stage diagnosis.
healthy. With back pain and difficult in gait. Spine CT reveals multi-
ple osteolytic lesions in vertebral bodies and fractures on T9. Bone
scintigraphy showed multiple osteolytic lesions with normal labora- PO050 / #1468 CHYLOTHORAX IN A PEDIATRIC PATIENT
tory findings. Biopsy showed Large cell Anaplastic Lymphoma ALK (+). WITH HODGKIN’S LYMPHOMA
Presented hypercalcemia, successfully managed, persistent fever, that
improved after starting chemotherapy. Saten Hovhannisyan1,2 , Lilit Sargsyan1 , Davit Dallakyan3 , Gevorg
Methods: Clinical history review Tamamyan1 , Samvel Iskanyan2 , Lusine Hakobyan1 , Julieta Hoveyan2 ,
Results: We got to stury four different presentetions of very unusual Clodet Stepanians1 , Mery Petrosyan2 , Saten Hovhannisyan1 , Ruzanna
Linfomas in pediatric populatoin. Papyan4
Conclusions: Due to the very different presentations, there has to be a 1 Yerevan State Medical University, Department Of Pediatric Oncology And
very highly suspicion in order to get close to this diagnoses Hematology, Yerevan, Armenia; 2 Hematology Center after Prof. R.H.Yeolyan,
Pediatric Cancer And Blood Disorders Center Of Armenia, Yerevan, Armenia;
3 Sourb Atsvatsamayr Medical Center, Thoracic Surgery, Yerevan, Armenia;
PO049 / #881 BURKITT’S LYNPHOMA: CASE ANALYSIS IN 4 Hematology Center after R.Yeolyan, Pediatric Cancer And Blood Disorders

PEDIATRIC PATIENTS IN WEST PARANÁ- BRAZIL Center Of Armenia, Yerevan, Armenia

Ana Flavia Fiori1 , Aline Rosa2 , Carmem Maria Costa Fiori2 Background and Aims: Untreated chylothorax is associated with
1 HOSPITAL DO CANCER DE CASCAVEL-UOPECCAN, Pediatric, Cascavel, a high rate of mortality among patients with malignancies. Surgical
Brazil; 2 Hospital do Câncer de Cascavel- UOPECCAN, Pediatric Oncology, trauma and malignancies are the most common causes of chylothorax.
Cascavel, Brazil Methods: We report about a 17-years old male with Hodgkin’s Lym-
phoma and chylothorax.
Background and Aims: Burkitt’s Lymphoma (BL) is an aggressive, Results: In January 2022 patient was diagnosed with Hodgkin’s Lym-
mature B-cell malignancy that represents approximately 50% of all phoma at Pediatric Cancer and Blood Disorders Center of Armenia.
childhood Non-Hodgkin’s Lymphoma (NHL). It can manifest in three His first complaints included fever, shortness of breath, enlargement
clinical forms: sporadic, endemic and associated with immunode- of cervical lymph nodes, and weight loss. With pleural effusion and
ficiency, but with epidemiological, clinical and genetic differences. total hydrothorax, the child was referred to the surgical unit where
Objective: To analyze and describe the epidemiological, clinical and lab- pleural aspiration with drainage was performed. Pleural fluid cytol-
oratory characteristics of Burkitt Lymphoma in children under 19 years ogy indicated lymphoproliferative disease. CT examination described
old seen at the Pediatric Oncology reference center in Western Paraná. cervical, infraclavicular, axillar, retroperitoneal lymphadenopathy, and
Methods: A descriptive cross-sectional study was performed mediastinal mass with sizes 9.4x5.3x6.1 cm with the invasion of the
by analyzing the physical and electronic medical records of left brachycephalic, subclavicular, and jugular veins. Biopsy of cervi-
patients diagnosed with Burkitt’s Lymphoma between Jan- cal lymph node with further histological and immunohistochemical
uary 2009 and January 2019, at Hospital Cancer Cascavel - examinations indicated classical Hodgkin’s lymphoma nodular sclero-
UOPECCAN. Data regarding gender, age, histopathological sis subtype, IIIB stage without bone marrow involvement. In February
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S588 of S635 ABSTRACTS

2022, treatment was started with OEPA regimen scheme, and dur- has an excellent outcome compared with the advanced stage. Even post
ing the treatment from pleural drainage 1500 ml of chyle was leaked recurrence transplant has an excellent outcome
in 24hours. Chemotherapy was stopped on day 4 and the patient
was referred to the surgical unit for further management. Conser-
vative treatment with somatostatin analog, parenteral nutrition was PO052 / #488 RESISTANT GASTRIC BURKITT LYMPOMA
not effective and in March 2022 clip closure of ductus thoracicus
was performed. At the same time, the patient received thrombolytic Ksenia Loseva1 , Eugenia Magkou1 , Marina Servitzoglou1 , Dimitrios
therapy for thrombosis of the left brachycephalic vein, left subclavicu- Doganis2 , Maria Nikita2 , Margarita Mpaka3
lar, and left jugular vein. However, during the conservative treatment 1 CHILDREN HOSPITAL PANAGIOTIS & AGLAIA KYRIAKOU, Oncology,
with close monitoring by a pediatric oncologist the child continued ATHENS, Greece; 2 ‘P & A. Kyriakou’ Children’s Hospital, Department Of
chemotherapy by OEPA scheme and radiologic examination described Oncology, Athens, Greece; 3 “P and A Kyriakou” Children.s Hospital, Depart-
a significant decrease in sizes of mediastinal mass and recanalization of ment Of Oncology, Athens, Greece
veins.
Conclusions: Although, there is no consensus on continuation of Background and Aims: Background and aims This case report is pre-
chemotherapy during the conservative treatment of chylothorax, man- sented to demonstrate a rare case of a pediatric patient with gastric
agement of chylothorax, thrombosis, and continuation of chemother- Burkitt lymphoma who, despite excellent prognosis of the disease, had
apy significantly improved health condition of this patient. an extremely resistant tumor.
Methods: A 10-yeared old boy was admitted with weight loss and
epigastric pain during last 3 months. CT scan revealed extensive
PO051 / #1333 OUTCOME OF PEDIATRIC HODGKIN’S wall thickening of the stomach with enlarged local lymph nodes.
LYMPHOMA TREATED WITH ABVD IN A TERTIARY CENTER IN Stomach biopsy revealed Burkitt lymphoma. The patient started
SOUTH INDIA - RETROSPECTIVE ANALYSIS chemotherapy according to the Inter B NHL ritux 2010 Group B
protocol. After the 1st cycle there was no improvement in the imag-
Pallavi Jaju, Rakesh Pinninti ing findings, so the staging was upgraded to Group C. After next
basavatarakam indoamerican cancer institute, Medical Hematooncology, two chemotherapeutic cycles a new imaging test revealed residual
hyderabad, India disease in the stomach, so it was considered as a very resistant
disease. We searched for CMYC, BCL2/ BCL6 rearrangement that
Background and Aims: With data on ABVD (Adriamycin, Bleomycin, were negative. After another cycle of chemotherapy there was no
Vinblastine, Dacarbazine) as an effective regimen in Hodgkin’s lym- response. The patient didn’t meet the criteria for autologous stem
phoma we evaluated as efficacy, overall survival in paediatric popula- cell transplantation so next cycle of chemotherapy followed without
tion . response.
Methods: Between January 2011 to December 2020, 176 patients Results: The patient was deteriorating rapidly: difficulty in swallow-
diagnosed Hodgkin’s Lymphoma received ABVD. Early-stage and bulky ing his saline and stomach ache, controlled by high doses of opioids.
disease received ABVD and radiation.Pet ct directed therapy has been We continued the therapy with a bispecific antibody agent Glofita-
done in 45 % of cases . other underwent evaluation by Contrast- mab (antiCD20-CD3) along with Obinatuzumab (antiCD20). After the
enhanced CT . data have been collected retrospectively for overall first cycle there was a surprising improvement in his clinical condition
survival (OS), Progression free survival (PFS) and on imaging tests. In a month there were peritoneal infiltrations
Results: Out of 176 patients, 4 patients received other than ABVD on abdomen MRI and bone marrow infiltration. Anti CD22 agent,
treatment and 5 cases were lost to treatment. 133 (79.6 %) are males Inotuzumab, was delivered, without any result.
and 34 (20.4 %) are females. Early-stage (stage 1 and 2) are 57.5 % and Conclusions: Results -Conclusions Despite the very good prognosis
Advanced stage (3 and 4) are 42.5 %. Early-stage has CR (Complete Burkitt lymphoma has in children, there is a small group of children
remission) in 94 % and advanced 88 %. Early-stage event-free sur- around 5-15% that have resistant or recurrent disease with a poor
vival (EFS) 105 months (95 % confidence interval) and Advanced stage prognosis. In these cases targeted therapy should be used along with
EFS 93 months .The median follow-up duration for cohorts was 60 bone marrow transplantation.
months. median OS and freedom from treatment failure not reached.
Total events in the cohort were 19 (including relapses, refractory, and
treatment-related mortality ). The total event was 19 events which are PO053 / #1277 PEDIATRIC NODULAR LYMPHOCYTE
11.4 % .out of these early-stage events were 5.2 % and 6.2 % in the PREDOMINANT HODGKIN LYMPHOMA: CASE REPORT AND
advanced stage. Relapsed cases were a total of 6 cases out of their REVİEW OF THE LITERATURE
relapsed cases, 2 cases underwent transplants and their OS was 51
months, one from each stage. Deniz Tugcu1 , Yasin Yilmaz1 , Şifa Şahin1 , Gulsah Tanyildiz1 , Serap
Conclusions: Combined modality with ABVD and radiation ( in specific Karaman1 , Gulsum Coskun1 , Gülçin Yeğen2 , Zuhal Bayramoğlu3 ,
results in excellent outcome in the pediatric population. the early stage Zeynep Karakas1 , Ayşegül Ünüvar1
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S589 of S635

1 Istanbul University, Istanbul Faculty of Medicine, Pediatric Hematology- relapsed or refractory (R/R) HL in children. We aimed to analyze our
oncology, Istanbul, Turkey; 2 Istanbul University, Istanbul Faculty of experience in relapsed or refractory HL treatment.
Medicine, Pathology, Istanbul, Turkey; 3 Istanbul University, Istanbul Faculty Methods: From January 2018 to June 2021 10 children with early
of Medicine, Radiology, Istanbul, Turkey relapse - 3 (30%), late relapse – 2 (20%) and refractory – 5 (50%) HL
were enrolled to the study. Age: 3,5 – 18 y.o. (14,5±2,1). All patients
Background and Aims: Nodular lymphocyte-predominant Hodgkin were treated according to the ViGePP+BV.
lymphoma (NLPHL) is a very rare disease in childhood and adoles- Results: Overall response rate – 100%, complete response (CR) – 7
cence. It differs clearly from classical Hodgkin lymphoma by clinical (70%) partial response – 3 (30%). Disease-free survival (DFS) was
presentation and more favorable outcome. 83±15% (median follow-up 46.2±1.8 months). aHSCT for 6 patients
Methods: In this case report, 9 years old male patient, complained of out of 10. Reasons for the rejection of the aHSCT: 2 patients with late
swelling in the left arm for a year, diagnosed as NLPHL after aksillary relapse with a CR to 2-line chemotherapy, 2 patients with refractory
lymph node biopsy was described with clinical, radiological findings and course who achieved CR after 4 courses of ViGePD+BV - refusal of
treatment modalities. aHSCT at the request of parents. After completion of chemotherapy,
Results: A nine-year-old male patient was admitted with swelling on both patients underwent RT up to 26 Gy, followed by immunother-
the inside of the left arm and axilla a year ago. It was interpreted as apy with nivolumab up to 6 injections. Both patients are alive and in
plexiform neurofibroma in the MRI in other center. The patient had second remission (follow-up 46 months). Out of 10 patients, relapse
not been followed up for a year. Physical and laboratory examination developed in 1 patient with refractory HL. She started nivolumab, up
was unremarkable except for swelling in the left humerus medial to the to 16 injections in total. We achieved CR after 3 months of nivolumab
elbow and redness of 4-5 cm in the axilla. In the MR imaging, multiple administration, remission persists for 14 months.
nodular lesions measuring approximately 3 cm in size were observed Conclusions: Novel targeted agents in the treatment of R/R HL can
in the medial and left axilla. USG and PET-CT imaging showed multiple increase survival in this unfavorable group of patients. Three-year
nodular lymphadenopathy extending from the axilla to the medial left DFS at R/R HD using the ViGePD+BV scheme was 83±15.2%. It is
forearm and into the elbow, and at the level of the superior mesentery necessary to continue the study.
artery in the abdomen. After excision from the axillary lymph node, the
pathology result was reported as NLPHL, stage III. After two cycles of
R-CHOP treatment, very good treatment result was achieved, planned PO055 / #1210 CLINICAL AND IMMUNOLOGICAL FEATURES
to complete 4 cycles. OF LYMPHOBLASTIC LYMPHOMAS IN CHILDREN
Conclusions: NLPHL should be kept in mind in differential diagnosis,
mass that cannot be clarified for a long time with atypical localisa- Timur Valiev, Tatiana Pavlova
tion and slow course . Patients with early stage could be treated Pediatric Oncology and Hematology Research Institute of N.N.Blokhin
by surgical lymphadenectomy alone or associated with not intensive National Research Cancer Center, Hemoblastoses Chemotherapy, Moscow,
chemotherapy. CVP/CHOP chemotherapy with rituximab in advanced Russian Federation
stage has achieved complete responses. Late relapses and the risk of
transformation into aggressive B lymphoma needs extended follow-up. Background and Aims: Lymphoblastic lymphomas (LBL) are the sec-
ond most common variant of NHL, accounting for 25-35% of all NHL
cases. The majority, 75-80%, are lymphomas of T-lymphoblastic origin
PO054 / #841 BRENTUXIMAB VEDOTIN FOR RELAPSED OR (T-LBL), 20-25% are B-lymphoblastic (B-LBL). With the use of modern
REFRACTORY HODGKIN LYMPHOMA: SINGLE CENTER therapy programs, the relapse-free survival (RFS) and overall survival
EXPERIENCE (OS) of children and adolescents with LBL is currently 80%. Taking into
account the fact that acute lymphoblastic leukemias (ALL) and LBL are
Timur Valiev1 , Nataly Tsaplina2 , Kirill Kirgizov2 morphologically and immunophenotypically homogeneous the stan-
1 Pediatric Oncology and Hematology Research Institute of N.N.Blokhin dard of treatment for LBL, regardless of the immunological variant, is
National Research Cancer Center, Hemoblastoses Chemotherapy, Moscow, currently considered to be the protocols for the treatment of ALL of
Russian Federation; 2 Pediatric Oncology and Hematology Research Insti- the BFM group. In the modern literature, there is limited information
tute of N.N.Blokhin National Research Cancer Center, Hemoblastoses about the complex clinical and immunological features of the disease,
Chemotherapy, Москва, Russian Federation correlations with the effectiveness of therapy in patients with LBL.
Based on the immunopfenotype of LBL to evaluate the effectiveness
Background and Aims: Treatment of Hodgkin lymphoma (HL) is suc- of chemotherapy with ALL IC-BFM 2002/2009 protocols.
cessful both in children and adults. But about 10% of patients with HL Methods: The study included 25 patients with primary diagnosed LBL.
are refractory to primary treatment, and up to 30% relapse. Standard Median age was 7.5 years. Treatment was carried out according to ALL
therapy in these cases is based on gemcitabine or carboplatine with IC-BFM 2002/2009 protocols in the period from 2002 to 2022. The
autologous hematopoietic stem cell transplantation (aHSCT). Brentux- analysis of immunological features, the involved structures and their
imab vedotin (BV) could be used to improve the results of therapy for effect on the outcome of therapy was carried out.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S590 of S635 ABSTRACTS

Results: Of the 25 patients with LBL, 22 (88%) had the T-LBL, 3 (12%) – fashion in untreated adults with DLBCL (POLARIX study,
B-LBL. 5-year OS was 92%. The most patients with T-LBL had dissem- NCT03274492), recently published in The New England Journal
inated stages of the disease (III-IV) - 80%, bone marrow involvement - of Medicine, showed a significantly higher progression free survival
in 44%. of 76.7% in the pola-R-CHP group compared to 70.2% in the R-CHOP
Conclusions: LBL are an immunologically heterogeneous group of arm at 2 years (p=0.02). The authors were also able to show that the
tumors with a predominance (88%) of T-LBL. Modern intensive safety profile was similar between both arms. They concluded that in
polychemotherapy makes it possible to achieve 5-year OS in 92% previously untreated intermediate or high-risk patients with DLBCL,
of patients with LBL. Further improvement of treatment regimens the substitution of Pv for vincristine resulted in a lower risk for disease
involves the inclusion of molecular-oriented drugs as novel approaches progression, relapse, or death.
for patients with refractory and recurrent disease, clarification of prog- Methods: A multicenter study for patients with intermediate and high-
nostic risk groups and the search for ways to reduce the toxicity of risk MB-NHL. Groups B and C MB-NHL (Cohort Ia and Ib, respectively)
therapy. patients will receive Pv in addition to RTX + modified FAB/FAB96
regimen.
Results: Forty patients age ≥ 3 and ≤ 39 years will be enrolled with
PO056 / #1068 REDUCING CHEMORADIOTHERAPY AND a primary objective to assess safety/feasibility of adding Pv, to RTX
RADIATION EXPOSURE FROM DIAGNOSTIC IMAGING BY containing FAB/LMB96, with reduced dose anthracycline. Correlative
UTILIZING TARGED IMMUNOTHERAPY IN CHILDREN, aims will assess cardiac, neurocognitive, and health related quality of
ADOLESCENTS AND YOUNG ADULTS (CAYA) WITH MATURE life toxicities; relapse risk with post-therapy MRD by NGS (Cohort I);
LARGE B-CELL LYMPHOMA (RADICAL) and central review imaging to determine feasibility of a risk-adapted
response-based approach of decreasing ionizing radiation exposure of
Anthony Audino1 , Matthew Barth2 , Ana Xavier3 , Jessica Hochberg4 , diagnostic imaging.
Rodney Miles5 , Samir Kahwash6 , Stephan Voss7 , Suzanne Braniecki4 , Conclusions: Adding targeted immunotherapy with Pv to FAB/LMB96
Chitti Moorthy8 , Saro Armenian9 , Matthew Ehrhardt10 , Megan Lim11 , chemotherapy with RTX MAY allow further anthracycline reduc-
Lauren Harrison4 , Stanton Goldman12 , Mitchell Cairo4,13,14,15 tion in intermediate- and high-risk MB-NHL while preserving
1 Nationwide Children’s Hospital/The Ohio State University, Pediatrics, outcomes.
Columbus, United States of America; 2 Roswell Park Comprehensive Can-
cer Center, Pediatrics, Buffalo, United States of America; 3 University of
Alabama at Birmingham, Pediatrics, Birmingham, United States of Amer- PO057 / #989 INDUCTION CHEMOIMMUNOTHERAPY AND
ica; 4 New York Medical College, Pediatrics, Hawthorne, United States of CONSOLIDATION WITH REDUCED TOXICITY CONDITIONING
America; 5 University of Utah, Pathology, Salt Lake City, United States AND ALLOGENEIC STEM CELL TRANSPLANT IN ADVANCED
of America; 6 Nationwide Children’s Hospital/The Ohio State University, STAGE MATURE T/NK-CELL LYMPHOMA/LEUKEMIA IN
Pathology, Columbus, United States of America; 7 Boston Children’s Hospi- CHILDREN, ADOLESCENT, AND YOUNG ADULTS
tal/Harvard Medical School, Radiology, Boston, United States of America;
8 Westchester Medical Center, Radiation Medicine, Valhalla, United States Ana Xavier1 , Lauren Harrison2 , Rodney Miles3 , Stephan Voss4 , Megan
of America; 9 City of Hope National Medical Center, Population Sciences, Lim5 , Mitchell Cairo2
Duarte, United States of America; 10 St. Jude Children’s Research Hospital, 1 University of Alabama at Birmingham, Pediatrics, Birmingham, United
Oncology, Memphis, United States of America; 11 University of Pennsylvania, States of America; 2 New York Medical College, Pediatrics, Hawthorne,
Pathology And Laboratory Medicine, Philadelphia, United States of Amer- United States of America; 3 University of Utah, Pathology, Salt Lake City,
ica; 12 Texas Oncology-Medical City Dallas, Pediatrics, Dallas, United States United States of America; 4 Boston Children’s Hospital/Harvard Medical
of America; 13 New York medical college, Pediatrics, Valhalla, United States School, Radiology, Boston, United States of America; 5 University of Pennsyl-
of America; 14 NYMC, Pediatrics, Valhalla, United States of America; 15 New vania, Pathology And Laboratory Medicine, Philadelphia, United States of
York Medical College, Pediatrics, Valhalla, United States of America America

Background and Aims: Despite therapeutic success of children and Background and Aims: Mature T/NK-cell lymphomas (MTCL) are rare
adolescent and young adults (CAYA) with mature B non-Hodgkin lym- among children, adolescent, and young adult (CAYA) and associated
phoma (MB-NHL), significant chronic health conditions increase over with dismal outcomes. For instance, we reported US outcomes of pedi-
time. Reduced anthracycline dosing by adding dose dense rituximab atric MTCL and found that patients with peripheral T-cell lymphoma
(RTX) to standard chemotherapy does not compromise outcomes in (PTCL), extranodal NK/T-cell lymphoma (ENKL), or hepatosplenic T-
intermediate-risk MB-NHL (4-year OS/EFS 100%; Goldman/Cairo, cell lymphoma (HTCL) have very guarded prognosis (5-year OS 59%,
Leukemia 2021). Preclinical studies have shown that polatuzumab 47%, and 9.6%, respectively), highlighting the urgent need for new
vedotin (Pv) alone and in combination significantly induces cytotox- therapies (Sorge, Cairo, Xavier. BJH 2019). Despite significant progress
icity against MB-NHL (Audino, et al. ASH abstract 2020). A phase in the understanding of lymphoma cell biology, its microenvironment
3 study comparing Pv + R-CHP vs. R-CHOP in a randomized and the development of novel therapies targeting T/NK lymphomas,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S591 of S635

no standard of care treatment has been established for MTCL in CAYA ( IQR 3.75-10.15) years. Majority of patients were males 16(61.5%)
patients. and 17 had malignant disease. Out of which 9(34.6%) had B-Cell
Methods: Multicenter pilot study for patients with de novo stage ALL relapse, 3(11.5%) had AML relapse while 2(7.7%) had CML and
III/IV ENKL/aggressive NK-cell leukemia (cohort 1) or other mature 3(11.5%) had high risk neuroblastoma. There were 5(19.2%) patients
TCL (cohort 2). Cohort 1 receive a combination of daratumumab, dex- with Thalassemia major, 2(7.7%) had aplastic anemia while 1(3.8%)
amethasone, methotrexate, ifosfamide, calaspargase, and etoposide had HLH and 1(3.8%) had sickle cell hemoglobinopathy. Cyclophos-
(D-SMILE). If CR after 2 induction cycles patients undergo consolida- phamide/TBI was used as conditioning regimen in 12(46.1%) patients,
tion with reduced toxicity conditioning (RTC) and allogeneic stem cell Busulfan/Melphalan was used in 3(11.5%) and cyclophosphamide/ATG
transplant (alloSCT). Patients not in CR after induction can receive was used in 3(11.5%) while 8(30.7%) had miscellaneous condition-
pembrolizumab with the goal of achieving an optimal disease sta- ing regimen. There were 16(61.5%) patients who underwent matched
tus before alloSCT. Cohort 2 patients receive brentuximab vedotin, related donor transplant, 7(27%) had haploidentical donor transplant
cyclophosphamide, doxorubicin (Bv-CHP). Cohort 2 in CR after 2 while 3(11.5%) underwent autologous transplant. Acute GVHD was
induction cycles undergo consolidation with RTC and alloSCT. Patients observed in 14(53.8%), chronic GVHD in 5(19.2%), and relapse of
not in CR after induction can receive pralatrexate with the goal of primary disease in 7(27%).
achieving an optimal disease status before alloSCT. Forty patients age Conclusions: Our survival rates are comparable to those from high
≥ 12 months and < 31 years will be enrolled with a primary objective income countries. We infer that there is scope for expanding the
to assess safety and ORR by central imaging review of the 2 induc- program.
tion regimens and safety and feasibility of consolidation with RTC and
alloSCT. Secondary endpoints include EFS, OS, and probability of non-
engraftment, donor chimerism, acute and chronic GVHD, hematopoi- PO059 / #1614 NOSOCOMIAL OUTBREAK OF
etic and immune reconstitution post RTC and alloSCT. NCT03719105. BURKHOLDERIA CEPACIA COMPLEX IN A HEMATOPOIETIC
Clinical trial supported by Pediatric Cancer Research Foundation and STEM CELL TRANSPLANT UNIT
Servier Pharmaceutics.
Results: In progress. Maria El Kababri, Meriem Lakhrissi, Naoual El Ansari, Zineb Isfaoun,
Conclusions: In progress. Chaimaa Drief, Mohamed El Khorassani, Mohamed Khattab, Leila
Hessissen, Amina Kili
Mohamed V University. Rabat, Pediatric Hematology And Oncology Depart-
Publication Only Topic: AS05 SIOP Scientific programme / AS05.d ment, RABAT, Morocco
Stem Cell Transplantation
Background and Aims: Hematopoietic stem cell transplantation
PO058 / #1618 PEDIATRIC HEMATOPOIETIC STEM CELL (HSCT) is a potentially curative treatment for many diseases in
TRANSPLANT: AN INSTITUTIONAL EXPERIENCE FROM THE pediatric oncology and hematology. However, HSCT patients are at
LOW MIDDLE-INCOME SETTING increased risk of severe infections secondary to commensal and oppor-
tunistic microorganisms, such as Burkholderia cepacia complex (BCC).
Asifa Noor, Asim Belgaumi, Zehra Fadoo, Bakhtawar Altaf, Sadaf Altaf Objective: To describe a Burcholderia cepacia complex bacteremia in a
Agha Khan University Hospital, Karachi Pakistan, Department Of Oncology, hematopoietic stem cell transplant unit and to report the actions taken
Karachi, Pakistan for the management of these patients.
Methods: Retrospective study of seven children hospitalized in the
Background and Aims: Our study aims to review clinical features, indi- hematopoietic stem cell transplant unit at the PHOC in Rabat
cations for transplant and outcome for patient who underwent HSCT (Morocco) who presented a Burkholderia Cepacia Complex between
between 2019-2021. There is paucity of literature on pediatric stem October 2020 to May 2021. The clinical data concerning the children,
cell transplant from LMICs where logistical problems are common. the different explorations performed, the microbiological results, their
Methods: Descriptive analysis was performed on all 26 patients who treatments and their evolution were collected. 3 series of environmen-
had HSCT. Outcomes included survival in 3 years, development of tal samples were performed.
graft vs host disease and other bone-marrow transplant complications. Results: The median age of our patients was 4,5 years. The 7 patients
Patient age, gender, primary disease, conditioning regimen, type of pro- who were infected with Burkholderia Cepacia were followed for solid
phylaxis for GVHD, source and dose of stem cell used were evaluated tumors and were hospitalized for autograft. No cases of infection with
as variable. this germ were reported in the allograft patients. The bacterium was
Results: Out of 26, OS was 77% whereas 6(33%) patients died. isolated from blood in all patients and from cultured material in 6
Ten(38.5%) reported other post-transplant complications including patients. In addition, it was not found in any of the environmental
CMV infection, stage-IV VOD, fungal infection and multi-organ dys- samples. The average number of blood cultures positive for B. cepa-
function. EFS was 61.5%. The median age of patients at diagnosis was cia for each patient was 6 with a time to positivity from 8 to 23 hours.
5 (IQR 1.1-10 ) years while median age at the time of HSCT was 7.2 The sensitivity rates to ceftazidime and piperacillin-tazobactam were
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S592 of S635 ABSTRACTS

57%, 100% respectively. Apyrexia was achieved after an average of Background and Aims: Hematopoietic stem-cell transplantation
18 days of intravenous antibiotic therapy and required removal of (HSCT) is curative treatment for several benign and malignant disor-
the implantable venous device and/or central venous catheter in all ders in children. Procedure involves technical expertise and significant
patients. No deaths were reported. The bacteria were not found in any financial liability. Considering the population of India, very few trans-
of the 40 environmental samples taken. plant centres are available with majority being in private sector
Conclusions: B. cepacia infections can be dramatic and lead to death. in metropolitan cities. Many patients with financial constraints are
Despite the investigation conducted the source of this BCC bacteremia deprived of this treatment option. We share our experience of set-
remained unknown. ting up HSCT services in central government teaching hospital in North
India and also present preliminary data.
Methods: Pediatric HSCT was started in January 2020. Except first
PO060 / #1927 STEM CELL TRANSPLANTATION IN two transplants, remaining transplants were carried out in HEPA fil-
MOROCCO: EXPERIENCE OF THE PEDIATRIC HEMATOLOGY ter rooms. Stem cell enumeration was carried out in collaboration with
AND ONCOLOGY DEPARTMENT IN RABAT the flowcytometry laboratory of another hospital. Technicians of the
blood bank were trained for stem cell apheresis during the first two
Maria El Kababri, Meriem Lakhrissi, Mohamed El Khorassani, procedures. Later on, they could carry out the procedure on their own.
Mohamed Khattab, Leila Hessissen, Amina Kili All patients received financial help from government /non government
Mohammed V University. Rabat, Pediatric Hematology And Oncology sources.
Department, RABAT, Morocco Results: Seven patients underwent autologous hematopoetic stem cell
transplant. Male to female ratio was 6:1, age range 2.5 to 10 years.
Background and Aims: The autologous peripheral blood stem cells Indications were relapsed Hodgkin lymphoma (1), refractory Hodgkin
(CSP) are used as a support to the restoration of the hematopoiesis in lymphoma (2) and neuroblatoma stage 4 (4). Patient with relapsed
oncohematology. Hematopoietic stem cell transplantation at pediatric Hodgkin lymphoma (HL) received ABVD, DHAP and ICE whereas the
hematology and oncology department of Rabat is now 7 years old: The other two received ABVD followed by DHAP. Patients with neurob-
first autotransplant was performed in November 2014 and has been lastoma received rapid COJEC followed by TVD. G-CSF (10 μg/kg/day
followed by 56 others. The choice was initially made to use autollo- for 5 days) was used for stem cell mobilization. One patient of neu-
gous HSCT to initiate the teams and to test the procedures. Allogenic roblastoma required plerixafor. Peripheral stem cells were harvested.
HSCT were then programmed and successfully undertaken.The objec- Patients with HL and neuroblastoma received conditioning with BEAM
tive of our work is to report the experience of pediatric hematology and and BuMel protocols respectively. Three patients had engraftment
oncology department of Rabat in the field of haematopoietic stem cell fever between day +7 to day +9. None developed sinusoidal obstruc-
transplantation. tion syndrome (SOS). Follow up ranged from 58 to 730 days. One
Methods: This retrospective study focused on 64 patients treated in patient with neuroblastoma relapsed 4 weeks after ASCT.
pediatric hematology and oncology department of Rabat over a period Conclusions: Setting up of pediatric transplant services is challeng-
from November 2004 to March 2022. ing but feasible in resource-constrained settings and outcomes are
Results: Sixty-four HSCT were performed during this period: fifty encouraging.
seven autografts and 7 allografts. 45 patients were transplanted for
high risk neuroblastoma, 8 for Hodgkin’s disease and 4 for a Burkitt
lymphoma. Allogenic SCT including two cases of immune deficiency PO062 / #1495 AUTOLOGOUS HEMATOPOIETIC STEM CELL
(SCID), 4 aplastic anemia and one case of acute myeloblastic leukaemia. TRANSPLANTATION FOR RETINOBLASTOMA AND
Conclusions: HSCT activity is certainly established with a progres- PLEUROPULMONARY BLASTOMA: SINGLE CENTER
sive and certain acquisition of skills (via national and international EXPERIENCE
referents) but remains subject to several insufficiencies and imperfec-
tions linked to the non-institutionalisation of procedures. To date, the Anna Elfimova1 , Yana Dokuchaeva1 , Karina Sergeenko1 , Nara
contribution of associations is considerable. Stepanyan1 , Yuriy Lozovan1 , Natalia Burlaka1 , Elena Machneva1 ,
Teimur Aliev1 , Irina Kostareva1 , Tatyana Gorbunova2 , Amina
PO061 / #1234 PEDIATRIC HEMATOPOETIC STEM CELL Suleymanova3 , Garik Sagoyan3 , Marina Rubanskaya3 , Anatoliy
TRANSPLANTATION: PRELIMINARY DATA FROM A Kazantcev3 , Tatiana Ushakova3 , Vladimir Polyakov4 , Kirill Kirgizov1 ,
TRANSPLANT CENTRE IN LOW MIDDLE INCOME COUNTRY Svetlana Varfolomeeva3
1 Research Institute of Pediatric Oncology and Hematology of the N.N.
Vineeta Gupta1 , Priyanka Aggarwal1 , Nirali Sanghvi1 , Vineeta Singh1 , Blokhin National Medical Research Center of Oncology, Bone Marrow
Sandip Kumar2 Transplant, Moscow, Russian Federation; 2 Research Institute of Pediatric
1 Institute of Medical Sciences, Department Of Pediatrics, Varanasi, India; Oncology and Hematology of the N.N. Blokhin National Medical Research
2 Institute of Medical Sciences, Department Of Pathology, Varanasi, India Center of Oncology, Head Physician, Moscow, Russian Federation; 3 N.N.
Blokhin National Medical Research Center of Oncology, Research Institute
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S593 of S635

Of Pediatric Oncology And Hematology, Moscow, Russian Federation; 4 N.N. hematopoietic stem cell transplantation (hHSCT) in pediatric acute
Blokhin National Medical Research Center of Oncology, Head And Neck lymphoblastic leukemia. Inotuzumab ozogamicin (INO) could help to
Tumors, Moscow, Russian Federation reach MRD-negative status in B-cell ALL (B-ALL) in children. Usage of
INO could result veno-occlusive disease (VOD). We aimed to describe
Background and Aims: Treatment of children with metastatic or a clinical case of early post-transplant VOD after hHSCT and INO as
relapsed retinoblastoma (RB) and severe pleuropulmonary blastoma “bridge-therapy” in a patient with relapsed B-ALL
(PPB) is still controversial. Autologous hematopoietic stem cell trans- Methods: Six years old boy with 2nd BM relapse of B-ALL was admit-
plantation (aHSCT) plays an important role in the management of rare ted to our center. After chemotherapy according FLAI scheme and 2
tumors in children such as RB and PPB. We aimed to present our expe- courses of immunotherapy with blinatumomab, persistence of MRD
rience in aHSCT for RB and PPB and present safety and tolerability of was found. Taking into account 100% expression of CD22 on tumor
transplantation for these children. cells, in order to achieve an MRD-negative status for hHSCT, a course
Methods: In 2019-2021 three children with metastatic RB and one of immunotherapy with Ino performed. Course included three injec-
patient with refractory PPB received aHSCT. Patients with retinoblas- tions in the form of intravenous infusion: 0.8 mg/sq.m. on day 0 and
toma received systemic chemotherapy and surgery before aHSCT. 0.5 mg/sq.m. on days 8 and 15. No complications were noted during
Conditioning regimen included carboplatin, etoposide and cyclophos- the infusion. MRD-negative status reached. hHSCT from mother with
phamide. The amount of CD34+ cells transplanted – 4.6 – 7x106 /kg. TcR α/β depletion done. Conditioning regimen: TBI + Fludarabine +
Patient with PPB received two lines of systemic chemotherapy and Etoposide
surgery before aHSCT. Conditioning regimen included treosulfan and Results: On day 21 after allo-HSCT, the child developed a clinical
melphalan. The amount of CD34+ cells transplanted – 14,2x106 /kg. picture of VOD: painful hepatomegaly, an increase in hepatic transam-
Results: The median day of engraftment was 14 (11-17). One of the inases, coagulopathy, decreased portal vein blood flow. Defibrotide
patients with RB had febrile neutropenia. Another patient experienced was administered at dose 25 mg/kg/day for 3 weeks. VOD completely
catheter-related bloodstream infection. All of the children had oropha- resolved after this treatment. Today at D+120 after hHSCT patient
ryngeal mucositis (1-3 grade). Two of the patients had neutropenic remains in remission (MRD-negativity and full donor chimerism).
enterocolitis (2 grade). Patient with PPB had oropharyngeal mucosi- Conclusions: INO in this case was the only option to achieve MRD-
tis (1 grade), neutropenic enterocolitis (1 grade) and drug eruption. negativity. VOD developed due to combination of INO and condi-
Two patients with RB and patient with PPB survived; one patient died tioning (including TBI) was treated successfully. Despite of possible
due to progression after 6 months after aHSCT. Median follow-up - 12 complications, INO is effective drug for reaching of MRD-negative
months. status
Conclusions: aHSCT for children with metastatic or relapsed RB and
patients with severe PPB is safe and effective method. Our data
showed acceptable toxicity and potential efficacy. Additional studies PO064 / #1888 ENTEROCOCCUS SPP CONTAMINATED
are required to evaluate the effectiveness and outcomes for these rare AUTOLOGOUS STEM CELL TRANSPLANTATION IN HIGH RISK
tumors. NEUROBLASTOMA

Elya Minasyan1,2 , Diana Soghomonyan3 , Ruzanna Papyan1,2 , Anna


PO063 / #1500 SUCCESSFUL TREATMENT OF Avagyan1,2 , Mery Petrosyan1,2 , Armine Pepanyan4 , Mane
VENO-OCCLUSIVE DISEASE EARLY AFTER HAPLOIDENTICAL Gizhlaryan1,5 , Andranik Shamilyan6 , Samvel Danielyan1 , Lilit
HSCT AND IMMUNOTHERAPY WITH INOTUZUMAB Sargsyan1,2 , Karen Meliksetyan5 , Gevorg Tamamyan1,2 , Lusine
OZOGAMICIN AS “BRIDGE-THERAPY”: CLINICAL CASE Krmoyan5
PRESENTATION 1 Hematology Center after Prof. R.H. Yeolyan, Pediatric Cancer And Blood
Disorders Center Of Armenia, Yerevan, Armenia; 2 Yerevan State Medical
Karina Sergeenko1 , Teimur Aliev1 , Irina Kostareva1 , Yurii Lozovan1 , University, Department Of Pediatric Oncology And Hematology, Yerevan,
Natalia Burlaka1 , Anna Elfimova1 , Nara Stepanyan1 , Elena Armenia; 3 Hematology Center after Prof. R.H. Yeolyan, Laboratory Of Micro-
Machneva1 , Timur Valiev2 , Kirill Kirgizov1 biology And Production Quality Control, Yerevan, Armenia; 4 Hematology
1 1N.N. Blokhin National Medical Research Center of Oncology, Ministry of Center after Prof. R.H. Yeolyan, The Stem Cell Laboratory, Yerevan, Arme-
Healthcare of Russian Federation, Department Of Pediatric Bone Marrow nia; 5 Hematology Center after Prof. R.H. Yeolyan, Bone Marrow / Stem
And Hematopoietic Stem Cell Transplantation, Moscow, Russian Federa- Cell Transplantation Department, Yerevan, Armenia; 6 Hematology Center
tion; 2 Pediatric Oncology and Hematology Research Institute of N.N.Blokhin after Prof. R.H. Yeolyan, Blood Components Storage Department, Yerevan,
National Research Cancer Center, Hemoblastoses Chemotherapy, Moscow, Armenia
Russian Federation
Background and Aims: Enterococcus spp contamination of stem cell
Background and Aims: Complete remission based on minimal resid- products is rare and may occur in various steps of peripheral blood
ual disease (MRD) require for successful outcomes in haploidentical stem cell(PBSC) transplantation. The main challenge of Enterococcus
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S594 of S635 ABSTRACTS

is due to the resistance to antibiotics and the high risk of causing lorlatinib combined with chemotherapy and craniospinal radiotherapy,
endocarditis. a third-generation ALK inhibitor, and in whom we achieved clinical and
Methods: We describe Enterococcus spp contaminated autologous radiological improvement.
stem cell transplantation in relapsed high risk neuroblastoma(HNB) Results: A one-year-old male was admitted with persistent fever
treated at the Pediatric Cancer and Blood Disorders Center of Arme- and fatigue. Physical examination revealed periorbital ecchymoses
nia.The bacteria were isolated from both samples after apheresis and and swelling on the parietal bone. Laboratory analysis revealed pan-
cryopreservation. For isolation and identification we used classical cytopenia, and elevated serum lactate dehydrogenase and ferritin
microbiological methods and the antibiotic susceptibility determined levels. Cranial, thoracic, and abdominal computed tomography (CT)
according to EUCAST 2020 protocol. showed multiple metastatic bone lesions, and a large tumor originat-
Results: The 11 month old boy was diagnosed with low risk neurob- ing from the left adrenal gland. Tru-cut biopsy from the abdominal
lastoma which relapsed after 5 months intilal surgery treatment. After mass confirmed the diagnosis of poorly differentiated NB. Molecular
receiving 1 cycle chemotherapy according to intermediate risk group genetic tests of primary tumors showed MYCN amplification, 11q dele-
protocol in MIBG was described femur metastasis. Started treatment tion positive and ALKF1174 mutation. After six cycles of induction
as HNB. Unfortunately, it was not possible to recruit after the 2nd chemotherapy, a partial response (PR) was achieved, with complete
cycle and 3rd cycle due to technical issues and insufficient quantity response (CR) of bone marrow. The primary left residual tumor was
of CD34+ cells according to HD-Cy+ GCS-F respectively. Following surgically resected. At the end of three cycles of maintenance ther-
cycle 5 of CiE, PBSC harvest collection was completed using plerix- apy, the patient developed multipl CNS metastasis. The hematoma
afor as a mobilization agent.PBSC was evaluated as contaminated with was evacuated surgically, and a ventriculoperitoneal shunt was placed,
Enterococcus spp. Following surgery, the last cycle of chemotherapy tumor excision failed. Tumor excision was not possible. The patient was
and port-a-cath removal, the child received prophylactic antibiotics initiated on lorlatinib (45 mg/m2 ) and a new cycle of chemotherapy
(Vancomycin,Levofloxacin, Piperacillin/Tazobactam) before infusion of including irinotecan (50 mg/m2 ) and temozolomide (100 mg/m2 ) was
the contaminated product according to antibiotic susceptibility results administered. Following the fourth cycle of chemotherapy, the patient
and during all consolidation therapy. No complications were recorded underwent craniospinal radiotherapy. He is still asymptomatic for
due to Enterococcus spp. Patient continued radiation and maintenance eight months on lorlatinib. Except for moderate hypercholesterolemia
therapies intended for protocol. Finishing treatment from September, (grade 2), no significant side effects were observed due to the use of
2021 the child is symptom free until now. lorlatinib
Conclusions: In our case, the isolated bacteria was not reason of hospi- Conclusions: In conclusion, high-risk NB with CNS involvement can be
tal acquired infection, therefore the antibiotic therapy was successful. rarely cured. Investigation of ALK mutations and the use of an ALK
In addition to that, the origin of the Enterococcus spp contaminated inhibitor such as lorlatinib may yield promising results. Further studies
PBSC was the long term catheterization it is important to organize are needed.
PBCS harvest as early as possible during chemotherapy.

PO066 / #1350 MIBG SCINTIGRAPHY INDICATIONS IN A


Publication Only Topic: AS05 SIOP Scientific programme / AS05.e LOW INCOME COUNTRY :EXPERIENCE OF A CENTER IN
Neuroblastoma TUNISIA

PO065 / #1821 THE RESPONSE OF LORLATINIB TREATMENT Imene Chabchoub1 , Tarek Ben Ahmed2 , Raja Sfar3 , Ines Ouaz4 , Nouha
COMBINED WITH CHEMOTHERAPY AND CRANIOSPINAL Ammar5 , Kaouther Chatti4 , Nadia Bouzid6 , Slim Ben Ahmed7
RADIOTHERAPY ON CENTRAL NERVOUS SYSTEM RELAPSE OF 1 CHU Farhat Hached/ Faculty of medecine Sousse, Medical Oncology,
HIGH-RISK NEUROBLASTOMA WITH ALKF1174 L MUTATION sousse, Tunisia; 2 CHU Farhat Hached, Medical Oncology, sousse, Tunisia;
3 CHU Sahloul Sousse, Nuclear Medecine, sousse, Tunisia; 4 CHU Sahloul,
Emre Çeçen1 , Dilek Ince1 , Safiye Aktaş2 , Deniz Kizmazoğlu1 , Handan Nuclear Medecine, sousse, Tunisia; 5 CHU Farhat Hached, Medicaloncol-
Güleryüz3 , Tekincan Aktaş2 , Ceren Kizmazoğlu4 , Nur Olgun1 ogy, sousse, Tunisia; 6 Farhat Hached Hospital, Radiation Oncology, Sousse,
1 Dokuz Eylul University Institute of Oncology, Pediatric Oncology, IZMIR, Tunisia; 7 CHU Farhat Hached, Medical Oncology/ University Of Medecine
Turkey; 2 Dokuz Eylul University Institute of Oncology, Basic Oncology Sousse, sousse, Tunisia
Department, IZMIR, Turkey; 3 Dokuz Eylul University, Radiology, İZMİR,
Turkey; 4 Dokuz Eylul University, Neurosurgery, İZMİR, Turkey Background and Aims: MIBG scintigraphy(MIBG-S) is not
only important for the diagnosis of neuroblastoma, but also
Background and Aims: There is a limited number of reports of suc- for staging and localization of skeletal lesions. If these are
cessful treatment of central nervous system (CNS) metastases in present, MIBG-S follow-up scans are used to assess the patient’s
neuroblastoma (NB) response to therapy. However, the sensitivity and specificity
Methods: In this article, we report a rare case of relapsed ALKF1174L of MIBG-S to detect neuroblastoma varies according to the
mutation–driven NB involving the CNS that was responsive to literature.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S595 of S635

Methods: This is a retrospective study of children treated for neu- 36patients. In 34.3% of the cases, 23MIBGSs were performed for
roblastoma at the department of medical oncology of FarhatHached an initial assessment before treatment in 8 cases and after sev-
Hospital sousse and who had at least one MIBG-S between 1997 - eral courses of chemotherapy in 15cases showing the primary tumor
2014. in all 23 patients, which was abdominal in 17cases (74%), thoracic
Results: We included 36 children with a median of 30 months(1month- in 3cases(13%), abdomino-pelvic in 2cases(8.7%), thoracoabdomi-
9years) and a sex ratio of 2.27. MIBG-S was done for 70% of our nal in 1case (4.3%). Remote metastases were detected in 14 cases
population. .Localised tumors were observed in 13 cases (38.2%). (60.9%).We performed 34 MIBG-S(50.7%) as part of the therapeutic
Treatment combined chemotherapy in 94.4% with surgery for 36.1% evaluation. There were 12 patients with negative MIBG scintigraphy
and radiotherapy for 11% and immunotherapy for 1 case- We per- and 10 patients positive MIBGSscintigraphy and CT scans indicating
formed 34MIBG-S(50.7%) as part of the therapeutic evaluation. There persistent disease, 2 patients with negative scintigraphy and positive
were 12 patients with negative MIBG-S, 10patients with positive CT scans, and 1 patient with a negative CT scan and a negative MIBG-S.
MIBG-S and CT scans indicating persistent disease, 2 patients with In studying the correlation between MIBG-S and imaging in the detec-
negative scintigraphy and positive CT scans, and 1 patient with a neg- tion of metastases during the extension assesment, MIBG scintigraphy
ative CT scan and a negative MIBG scintigraphy. In 15% of the cases, specificity for liver fixations was94%. It visualized bone fixations in 8
10MIBG-S were performed for control.Specificity various from 73each patients with a specificity of 73%. For lymph nodes specificity was 94%.
organ detected from 73% for bone, 83%for bone marrow, 90% for For bone marrow fixations specificity was83%.
lung, 94% either for nodes and liver Tumor residue monitoring was Conclusions: Delay of access MIBG-S in the detection of metastasis
performed in 4 patients by scintigraphy. MIBG-S were negative in 3 and for treatment evaluation is a problem in our country. It is manda-
patients and positive in one case. One patient had a recurrence of tory to improve the procedure of the realization of MIBG-S to obtain
the secondary bone location on CT scan and the MIBG-S was positive an optimal therapeutic management of the patients.
showing a bone recurrence.
Conclusions: MIBG scintigraphy is not only important for the diagnosis
of neuroblastoma, but also for staging and treatment evaluation. Some- PO068 / #365 EPIDEMIOLOGICAL PROFILE OF
times lack of acces to this investigation and the lack of specificity for NEUROBLASTOMA IN A REFERENCE HOSPITAL IN CHILD
some organs should indicate other explorations such as Technicium99 CANCER, CASCAVEL, PARANÁ, BRASIL
Scintigraphy or FDG-TEP if possible
Carmem Maria Costa Fiori, Anna Beatriz Beck, Aline Rosa
Hospital do Câncer de Cascavel- UOPECCAN, Pediatric Oncology, Cascavel,
PO067 / #1105 IMPORTANCE OF MIBG SCINTIGRAPHY IN Brazil
THE TREATMENT OF NEUROBLASTOMA IN TUNISIA
Background and Aims: Neuroblastoma is the most common extracra-
Imene Chabchoub1 , Tarek Ben Ahmed2 , Raja Sfar3 , Ines Ouaz4 , Nouha nial solid tumor in childhood, especially in children under 5 years of age.
Ammar2,5 , Kaouther Chatti4 , Manel Nouira6,7 , Slim Ben Ahmed8 Objective: Evaluate and describe the epidemiological, clinical, and lab-
1 CHU Farhat Hached/Faculty of medecine Sousse, Medical Oncology, oratorial characteristics of Neuroblastoma in patients under 19 years
sousse, Tunisia; 2 CHU Farhat Hached, Medical Oncology, sousse, Tunisia; old treated at a reference center in Pediatric Oncology in Western
3 CHU Sahloul Sousse, Nuclear Medecine, sousse, Tunisia; 4 CHU Sahloul, Paraná, Brazil.
Nuclear Medecine, sousse, Tunisia; 5 Farhat Hached Hospital/ University of Methods: Descriptive cross-sectional study, carried out by analyzing
medecine Sousse Tunisia, Medicaloncology, sousse, Tunisia; 6 University of electronic medical records of patients diagnosed with Neuroblas-
medecine Sousse Tunisia, Nuclear Medecine, sousse, Tunisia; 7 Sahloul Hos- toma between January of 2000 and July of 2020, at the Hospital
pital, Nuclear Medecine, sousse, Tunisia; 8 CHU Farhat Hached, Medical do Câncer de Cascavel – UOPECCAN. Data regarding gender, age,
Oncology/ University Of Medecine Sousse, sousse, Tunisia time from symptom onset to diagnosis, clinical manifestations, staging,
complications, and evolution were evaluated.
Background and Aims: Neuroblastoma is a very heterogeneous dis- Results: 26 cases were recorded. Regarding gender, 12 (46%) were
ease with a variable prognosis. MIBG scintigraphy(MIBG-S) is used to male and 14 (54%) female. The average age of diagnosis was 34
establish the pronsotic groups and evaluate the response to treatment. months. The most frequent clinical manifestations were fever (n=12;
The objective of this work is to evaluate the contribution of MIBG-S in 46%), abdominal mass (n=10; 38%), and abdominal pain (n=7; 27%).
the evaluation of the tumor response to treatment. As for the location, 20 (77%) were in the abdomen, 3 in the medi-
Methods: This is a retrospective study of children treated for neu- astinum (11.5%), 1 (4%) in the orbital region and 1 (4%) intramedullary
roblastoma at the department of medical oncology of FarhatHached at the thoracic level. The mean onset of symptoms until the diag-
Hospital sousse and who had at least one MIBG-S between 1997-2014. nosis was 31.7 days. As for staging in the INSS system, 3 (11.5%)
Results: According to the INSS classification 9patients are stage1, patients had stage 1, 2 (7.5%) patients in stage 3, 20 patients in stage
3patients stage2A, one patient in stage2B, two patients stage3, 4 (77%) and 1 (4%) patient in stage 4S. Out of the 26 patients, 20
and 21patients stage4. Sixty-seven MIBG-S were performed for (76.92%) had metastases at diagnosis, which occurred mainly in bone
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S596 of S635 ABSTRACTS

marrow and bones. At the time of the research, 10 (38.64%) remained PO069a / #1540 VITAMIN D AND OUTCOMES IN PEDIATRIC
alive. PATIENTS WITH HIGH-RISK NEUROBLASTOMA (HR-NB). AN
Conclusions: We can conclude that we still have a high number of UNTAPPED APPROACH IN PEDIATRIC CANCER
patients with advanced stage at diagnosis. It is extremely important
to direct efforts to train health professionals to recognize the dis- Ana Carolina Izurieta Pacheco, Ana Sangros Gimenez, Esther
ease at an early stage to reduce the number of cases already at an Martínez, Sara Perez, Maite Gorostegui
advanced stage and therefore increase the survival rate in children Pediatric Cancer Center Barcelona, Hospital Sant Joan de Déu, Oncology
with neuroblastoma in western Paraná, Brazil. Department, Barcelona, Spain

Background and Aims: A major field on research in the preceding


PO069 / #2026 LEVELS OF PRO-INFLAMMATORY AND decades has unveiled a regulatory role of Vitamin D in the process
ANTI-INFLAMMATORY CYTOKINES IN PEDIATRIC PATIENTS of tumorigenesis and metastases. Thus, pointing out a relationship
WITH NEUROBLASTOMA between Vitamin D deficiency and increased risk for cancer devel-
opment. We aim to describe the prevalence of vitamin D deficiency
Gabriela Hernández Pliego, Silvia Moreno Guerrero, Arturo Ramírez among children with HR-NB and to explore its correlation with clinical
Pacheco, Luz María Rocha Ramírez, María Argelia Escobar Sánchez, outcomes.
Alfonso Reyes López, Juan José Luis Sienra Monge, Luis Enrique Methods: We conducted a cross-sectional study of 184 children with
Juárez Villegas HR-NB from 2017 to 2021. We included all patients diagnosed at
Hospital Infantil de México Federico Gómez, Oncoclogía Pediatrica, CDMX, our center or that arrived from another healthcare-center to continue
Mexico treatment. Serum 25-hydroxyvitamin D levels were measured in sam-
ples taken at the time of diagnosis or arrival and related-to clinical data.
Background and Aims: The profile of cytokines which could be asso- Vitamin D deficiency was defined as a 25-hydroxyvitamin D level below
ciated with risky inflammatory processes. It has been reported that 30ng/ml. A pubmed search was done to compare results with healthy
stromal cells promote the growth and survival of NB cells and improve pediatric population.
resistance to chemotherapy by being a source of multiple inflammatory Results: The overall prevalence rate of vitamin D deficiency among
cytokines and chemokines. To evaluate the immunological contribution HR-NB patients was 81.6%. At least 48.4% had a severe deficiency
through serum levels of pro- and anti-inflammatory cytokines in NB (£20ng/ml). Only 17.9% had normal vitamin D levels. The mean level
compared to control group. of 25-hydroxyvitamin D was 20.2ng/ml. 38.8% were Caucasian and
Methods: Twenty-seven patients from the HIMFG from 2015 to 2019, 49.7% were of Asiatic origin. Solely 6.8% had moderate/severe malnu-
diagnosed with NB, staging according to the INSS criteria. Serum levels trition status. There was no association in terms of complete remis-
by ELISA method of pro-inflammatory cytokines- (IL-1β, TNF-α, IL-6, sion or relapse/refractory disease(p=0.27) nor with osteomedullar
and IFN-ү), chemokines (IL-8, MCP-1) and anti-inflammatory (IL- 10, metastases(p=0.64) and vitamin D status.
TGF-β1) in samples at diagnosis. The clinical information and prog- Conclusions: Vitamin D deficiency is very common among children
nostic factors were obtained from clinical records. Normal pediatric with HR-NB. Half of them encountering a severe vitamin D deficit that
controls were collected from non-cancer patients, who did not present seems not associated with nutritional status. It is of striking interest the
infectious processes. higher prevalence found than in healthy pediatric population. Although
Results: Elevated serum levels of IL-6, IL-8, IL-1β, IL-10, TNFα, IFN- no correlation appear to be between vitamin D status and clinical out-
ɣ, and MCP-1 were observed in NB compared to control group (p comes, prospective studies are needed to determine the role of vitamin
< 0.001), in contrast to the synthesis of TGF-β1. A significant cor- D in this group.
relation between the levels of IL-6 with IL-8 (r= 0.598; p< 0.001).
Survival analysis showed a significant association only between low
levels of IL-6 (p=0.009) and IL-8 (p=0.046) with higher overall sur- PO070 / #1753 CLINICAL CHARACTERISTICS AND
vival in NB. The serum levels of these cytokines and chemokines could OUTCOMES OF PEDIATRIC NEUROBLASTOMA IN NORTH
be possible immunological biomarkers associated with survival. The MACEDONIA
pro-inflammatory component such as high levels of IL-8, IL-6, TNF-α,
IFN-ɣ, and MCP-1, could be possible useful to identify patients with Svetlana Kocheva1 , Aleksandra Jovanovska1 , Kata Martinova1 , Zorica
high risk and monitoring of the disease. It is necessary to evaluate a Trajkova-Antevska1 , Beti Zafirova-Ivanovska2
larger number of patients to confirm these results. 1 University Children’s Hospital, Faculty of Medicine, University “Ss. Cyril
Conclusions: Our results suggest that elevated levels of pro- and Methodius”, Department Of Hematology And Oncology, Skopje, North
inflammatory cytokines and chemokines (IL-8, IL-6, TNF-α, IFN-ɣ, Macedonia; 2 Faculty of Medicine, University “Ss. Cyril and Methodius”, Insti-
and MCP-1) could be involved in the genesis of Neuroblastoma, tute Of Epidemiology And Biostatistics With Medical Informatics, Skopje,
promoting an inflammatory microenvironment that could favor the North Macedonia
development of the disease and affect the prognosis of patients.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S597 of S635

Background and Aims: Neuroblastoma (NB) is the most common neurotransmitter values in blood/urine, tumor location, treatment and
extracranial solid tumor in childhood. Survival of patients with NB outcome were collected.
depends on timely diagnosis and the availability of effective treatment Results: Eight cases reported and an unpublished case from our
and is in general much lower in the developing world. This study aimed institution were included. Median age at presentation was 28.5
to describe epidemiological date, clinical characteristics, and treatment days (1-120). Severe conditions were associated with AHT: respira-
outcomes of children with NB in North Macedonia. tory distress (n=2), metabolic acidosis (n=2), cardiac insufficiency
Methods: We conducted a retrospective descriptive study at the (n=3), hepatic/renal failure (n=1 each), and multiorgan failure (n=1).
Department of Hematology and Oncology of the University Children‘s Urine tests (n=6) showed a median vanillylmandelic acid (VMA) and
Hospital in Skopje which is the unique center in the country for diag- homovanillic (HVA) increase of 2.93 and 2.8 the upper normal val-
noses and treatment of children with cancer. Twenty-five patients with ues (UNV). Plasmatic neurotransmitters noradrenaline, adrenaline,
NB were diagnosed for the period 01.2010 – 12.2021 (12 years). dopamine were inconsistently registered (0.49,4.6 and 33 x UNV
Patients were treated according to the German NB97 protocol and respectively). Compression of the renal hilum was detected in 77% of
POG 9341 modified regimen adapted for NB treatment in low and patients, but alterations in the renin-angiotensin-aldosterone (RAA)
middle income settings. axis were understudied (n=2) (renin 11.6xUNV). Treatment for AHT
Results: The median age at diagnosis was 24 months (range 3 - was implemented (88%), with partial response. Surgery was indicated
66 months). Male to female ratio was 1.7:1. The most common pri- in 7 patients, resolving AHT within 120h maximum. One patient under
mary localization was adrenal gland (91.3%). MYCN amplification chemotherapy (SFOP NBL 94: 5 cycles) underwent subsequent surgery
was observed in 12 (52.2%) cases. Seventeen patients (73.9%) had due to AHT persistence. Remarkably, one untreated newborn died 12
metastatic disease at the time of diagnosis. A disease progression or days after birth due to multiple organ failure.
relapse occurred in 9 patients (39.1%) at a median follow up of 11 Conclusions: It is crucial to be aware of this serious presentation that
months. Four patients (17.4%) had a rapid clinical course of metastatic can jeopardize the excellent prognosis of neuroblastoma in this popula-
disease and died at a median follow up of 1.5 months. The 5-year over- tion. Surgery is the main strategy, together with supportive treatment
all survival rate for all evaluable patients was 30.4%. The 5-year overall of possible life-threatening accompanying conditions, to resolve AHT
survival rate for patients with high risk NB was 17.6% compared to 75% and avoid possible sequelae or a fatal outcome.
for patients with low and intermediate risk NB (P=0.042).
Conclusions: The most of the children with NB in our setting are
present with advanced disease and have a poor survival. The intro- PO072 / #976 CLINICOPATHOLOGIC CHARACTERISTICS,
duction of myeloablative chemotherapy with stem cell rescue and MANAGEMENT, AND OUTCOMES OF CHILDREN WITH
immunotherapy are urgently required to improve prognosis for high NEUROBLASTIC TUMORS: A REPORT FROM ARMENIA
risk NB patients.
Ruzanna Papyan1,2 , Lusine Hakobyan1,2 , Mery Petrosyan1,2 , Julieta
Hoveyan1,2 , Saten Hovhannisyan1,2 , Lilit Sargsyan1,2 , Samvel
PO071 / #985 ARE YOU AWARE NEUROBLASTOMA CAN Iskanyan2 , Gevorg Tamamyan1,2
MIMIC SEPTIC/METABOLIC SHOCK IN NEWBORNS? 1 Yerevan State Medical University, Department Of Pediatric Oncology And
Hematology, Yerevan, Armenia; 2 Hematology Center after Prof. R.H.Yeolyan,
Cristina Larrosa1 , Maria Fabregat1 , Margarita Vancells2 , Cristina Pediatric Cancer And Blood Disorders Center Of Armenia, Yerevan, Armenia
Rivera1 , Andrea Urtasun1 , Laura Andrés Zallo1 , Jaume Mora1 , Maite
Gorostegui1 Background and Aims: Peripheral neuroblastic tumors (PNT)
1 Pediatric Cancer Center Barcelona, Hospital Sant Joan de Deu, Oncology, are the most common extracranial heterogeneous group of
Barcelona, Spain; 2 Pediatric Cancer Center Barcelona, Hospital Sant Joan solid tumors among children. PNT encompasses following his-
De Déu, Pediatric Oncology Surgery, Barcelona, Spain tological variants: neuroblastoma, ganglioneuroblastoma, and
ganglioneuroma. Management varies from observation only to
Background and Aims: Neuroblastoma is the most frequent neoplasm combined therapy. This study aimed to analyze clinicopathologic
in children under 1 year of age. Unlike other neurogenic tumors, the characteristics, management, and outcomes of children diagnosed
association with arterial hypertension (AHT) is less frequent (10%) and with PNT in Armenia.
exceptional in the form of adrenergic crisis, requiring a high index of Methods: A retrospective review of the medical records of 42 children
suspicion for timely management. (≤18 years old) with PNT was conducted, who were diagnosed and/or
Methods: We conducted a search in Pubmed and EMBASE databases treated at the Muratsan Hospital Complex and Hematology center
for "neuroblastoma" and ["adrenergic crisis" or "shock" or "hyperten- after Prof. R. H. Yeolyan between 2008 and 2022.
sion"] restricted to [Title/Abstract]. Cases of patients presenting with Results: During the mentioned period out of 42 patients, 37 (88%)
an adrenergic crisis at <6 months of age were selected by two inde- were diagnosed with neuroblastoma, 4 (9.5%) with ganglioneuroblas-
pendent reviewers. Symptoms associated with arterial hypertension, toma, and 1 (2.3%) child with ganglioneuroma. The median age of the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S598 of S635 ABSTRACTS

patients was 2.5 years. More than half of cases (n=22, 52.3%) had M transplant was done in 64.2% of high-risk cases. Mortality was 15.9%
stage at presentation, 10 (23.8%) had L1 stage, 4 (9.5%) had L2 and 3 with the highest mortality in metastatic disease.The 2-year overall sur-
(7.1 %) children had MS stage. MYC status was detected in 28 (62%) vival rate was 100% in low risk,91% in intermediate-risk, and 71.4% in
cases and was amplified in 14 out of 42 (33.3%) patients. MYC sta- high-risk cases.
tus was possible to detect in Armenia since 2020. Majority of children Conclusions: Majority of children with neuroblastoma in India present
(n=26, 62%) diagnosed with PNT had high-risk disease. MIBG was with advanced-stage disease with a high risk of metastasis. High degree
done only in 16 (38%) children. Median diagnostic delay was 20 days, of clinical suspicion is required for early diagnosis and multimodality
median delay between chemotherapy cycles was 6 days. Complete intervention is the key to successful treatment for good outcomes in
surgical resection was done in 20 patients (57.6%), median delay of sur- these patients.
gical treatment was 17 days. High-dose chemotherapy with autologous
transplantation was first performed at Hematology Center in 2016.
Median delay of HDCT was 11.7 days. Out of 42 patients, 26 (62%) are PO074 / #329 METASTATIC NEUROBLASTOMA AND
still alive, of which 20 children had intermediate or low-risk disease. CONCOMITANT SANFILIPPO DISEASE: IS THERE A LINK?
Conclusions: There are diagnostic and therapeutic limitations, which
have been improved recently. Outcomes of non-high-risk neuroblas- Margarida Simao Rafael1 , Cristina Larrosa1 , Maria Del Mar
tomas are comparable with the data in developed countries, while in O’Callaghan2 , Cinzia Lavarino1 , Ofelia Cruz1
the case of high-risk disease survival remains poor. 1 Hospital Sant Joan de Déu, Pediatric Cancer Center Barcelona (pccb),
Barcelona, Spain; 2 Hospital Sant Joan de Déu, Pediatric Neurology,
Barcelona, Spain
PO073 / #1046 CLINICAL, PATHOLOGICAL PROFILE AND
OUTCOME IN PEDIATRIC NEUROBLASTOMA: EXPERIENCE Background and Aims: Neuroblastoma can be associated with con-
FROM INDIA stitutional genetic aberrations. However, the concurrent diagnosis of
Mucopolysaccharidoses (MPS) with neuroblastoma has never been
Gayathri S1 , Pooja Mallya2 , Shobha Badiger2 , Ravi Joshi2 , Shweta described.
Pathak2 , Swathi P M2 , Monika Bhukar3 , Sunil Bhat2 Methods: Here we present a patient with metastatic neuroblastoma
1 Mazumdar Shaw Medical Center, Paediatric Hemato-oncology And Bmt, and Sanfilippo disease.
Bangalore, India; 2 Mazumder Shaw Medical Center, Paediatric Hemato- Results: A 12-month-old girl was diagnosed with metastatic neuroblas-
oncology And Bmt, Bangalore, India; 3 Mazumdar Shaw Medical Center, toma, with a genomic profile characterized by numerical and segmental
Paediatric Hemato-oncology & Bmt, Bangalore, India chromosomal alterations, including 11q deletion, and no MYCN ampli-
fication. She was treated as per COG intermediate-risk with good
Background and Aims: Neuroblastoma is the 2nd most common tolerance and achieved a complete response by PET-CT scan. Although,
solid extracranial pediatric tumor. Advances in diagnostic methods and some MIBG positive residual lesions remained at the end of treatment.
multidisciplinary management have helped in early diagnosis, better At 3-year-old, a developmental delay prompted neurological assess-
treatment, and improved survival rates. The aim of the study was ment. A peculiar phenotype with mild coarse facial features was pro-
to assess the clinical profile, histopathology, grading, and outcome in gressively appreciated. Alterations in fine motor skills and speech were
children with neuroblastoma. also noted, with some behavioral changes. The genetic study showed a
Methods: This is a retrospective study conducted at a tertiary care constitutional homozygous mutation in SGSH p.Val361fs/c.1080delC,
center in Mazumdar Shaw Medical Center, Bangalore. Data of all pedi- matched with an MPS type IIIA (Sanfilippo Syndrome) diagnosis. She
atric patients with neuroblastoma from January 2016 to June 2021 was enrolled in an international clinical trial with genetic therapy asso-
were analyzed. International Neuroblastoma Staging System and risk ciated with immunosuppressive treatment. On the last follow-up, at
stratification were used. Data analysis was done by SPSS software. All the age of 6, she continues in remission for neuroblastoma; unfortu-
patients were treated with COG protocol based chemotherapy nately, the developmental delay and behavioral disturbances have not
Results: Our study had 44 patients. The male to female ratio was 1.2:1 improved.
with a median age of 39.8 months(8month-154 months). The most Conclusions: When two rare diseases collide in the same patient, a
common clinical presentation was abdominal mass(32.1%). Based on question arises whether this is a random event or if there is any biolog-
INSS stage stage 1,2,3, 4 and 4s was seen in 13.6%,11.4%,9.1%,61.4% ical correlation between the tumor and the constitutional alteration.
and 4.5% cases respectively .Based on INRG staging L1,L2,M and MS Sanfilippo syndrome is an autosomal recessive neurodegenerative
was seen in 27.3%,6.8%,61.4% and 4.5% cases respectively. High risk lysosomal storage disease. It has not been associated with a predis-
was seen in 63.6%, intermediate risk in 25%, and low risk in 11.4%. position to neuroblastoma. However, the patient’s mutation in SGSH
Favorable histology was seen in 59.1% with NMYC positive in 18.2% is at 17q25, which made us study the possible implication of this con-
cases. Bone marrow metastasis was seen in 59% of cases. All patients stitutional feature in her tumor genetic make-up. But no apparent
had received chemotherapy and surgery with upfront surgery in 15.9% connection explains the concomitance of both diseases. Moreover, the
of patients. Radiotherapy was given to 36.7% of cases. Autologous immunosuppressive therapy needed in her clinical trial was worrisome
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S599 of S635

but, it does not increase the relapse rate of developing tumors, such as Conclusions: In conclusion, high-dose chemotherapy plus stem cell res-
neuroblastoma, unlike what might happen in adult cancers. cue follow by cis-retinoic acid for 12 months is well tolerated and could
improve survival in patients with HR-NB.

PO075 / #171 TREATMENT OUTCOME OF HIGH-DOSE


CHEMOTHERAPY PLUS STEM CELL RESCUE IN HIGH-RISK Publication Only Topic: AS05 SIOP Scientific programme / AS05.f
NEUROBLASTOMA IN THAILAND Renal Tumours

Kunanya Suwannaying1 , Piti Techavichit2 , Patcharee Patcharee1 , PO076 / #1686 WILMS TUMOR: SINGLE CENTER
Napat Laoaroon1 , Nattee Narkbunnam3 , Kleebsabai Sanpakit3 , EXPERİENCE
Kanhatai Chiengthong2 , Thirachit Chotsampancharoen4 , Lalita
Sathitsamitphong5 , Chalongpon Santong6 , Panya Seksarn2 , Suradej Emre Çeçen1 , Deniz Kizmazoğlu1 , Esra Karataş2 , Dilek Ince1 , Cenk
Hongeng7 , Surapon Wiangnon8 Umay3 , Oktay Ulusoy4 , Ayşe Demiral3 , Mustafa Olguner4 , Handan
1 Division of Hematology-Oncology, Faculty of Medicine, Khon Kaen Uni- Güleryüz5 , Erdener Özer6 , Nur Olgun1
versity, Department Of Pediatrics, Khon Kaen, Thailand; 2 Integrative and 1 DEÜ Oncology Institute, Pediatric Oncology, İZMİR, Turkey; 2 Dokuz Eylul
Innovative Hematology/Oncology Research Unit., Department Of Pedi- University, Pediatrics, İZMİR, Turkey; 3 Dokuz Eylul University, Radiation
atrics, Bangkok, Thailand; 3 Faculty of Medicine, Siriraj Hospital, Mahidol Oncology, İZMİR, Turkey; 4 Dokuz Eylul University, Pediatric Surgery, İZMİR,
University, Division Of Hematology-oncology, Department Of Pediatrics, Turkey; 5 Dokuz Eylul University, Radiology, İZMİR, Turkey; 6 Dokuz Eylul
Bangkok, Thailand; 4 Faculty of Medicine, Prince of Songkla University, Divi- University Faculty of Medicine, Pathology, IZMIR, Turkey
sion Of Hematology-oncology, Department Of Pediatrics, Hat Yai, Thailand;
5 Faculty of Medicine, Chiang Mai University, Division Of Hematology- Background and Aims: To evaluate the clinical and pathological
oncology, Department Of Pediatrics, Chiang Mai, Thailand; 6 Faculty of characteristics and treatment outcome of Wilms tumour (WT) cases
Medicine, Khon Kaen University, Cancer Unit, Khon Kaen, Thailand; treated in our institution.
7 Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Division Methods: Between 1988-2021, patients with WT were reviewed ret-
Of Hematology-oncology, Department Of Pediatrics, Bangkok, Thailand; rospectively. Twentysix patients were treated accoding to the TPOG-
8 Faculty of Medicine, Mahasarakham University, Department Of Pediatrics, WT protocol, remaining patients were treated according to the NWTS
Mahasarakham, Thailand protocol.
Results: Sixtyone patients were eligible out of 71. The median age
Background and Aims: In 2013, Thai Pediatric Oncology (ThaiPOG) was 44 months, M/F:0.7. Median duration of complaints was one
introduced a national protocol in which high-dose chemotherapy plus month. There were three familial-WT. Genetic abnormalities were
stem cell rescue without immunotherapy. This study aims to eluci- genitourinary abnormalities (n:5), hemihyperplasia (n:3), aniridia (n:1),
date the outcomes of High-risk neuroblastoma (HR-NB) treated via the Denys-Drash (n:1), Beckwith Wiedaman(n:1), DICER 1 syndrome (n:1).
ThaiPOG protocol. Tumour was unilateral in 97% of cases. There was one patient with
Methods: A retrospective cohort review of 48 patients (30 males, 18 extrarenal-WT. Complete tumour resection was performed in all
females), median age 3 years (range 8 months–18 years), treated at except two cases. There were intraoperative tumour rupture (n:6) and
five ThaiPOG treatment centers in Thailand from 2000 to 2018 was partial resection (n:2) in patients without neoadjuvan chemothera-
conducted. phy (n:26). Stage distribution was stage-1 27%, stage-2 17%, stage-3
Results: Eight of the 48 patients revealed MYCN amplification. 20%, stage-4 23%, stage-5 3%. There were 20 metastatic patients
Twenty-three patients (48%) received MIBG treatment prior to high- (pulmonary:18, liver:6, bone:2). Histopathology revealed 76% FH, 24%
dose chemotherapy plus stem cell rescue. The majority of the patients UH. Intraoperative complications were not observed in patients who
achieved complete response or very good response prior to consolida- were given preoperative CT, and complete tumor excision could be
tion treatment. The 5-year overall survival (OS) and event-free survival performed in all of them. Radiotheraphy was performed to the pri-
(EFS) were 45.1% and 40.4%, respectively. We observed that patients mary tumour site in 22 cases, to whole abdomen in ten cases. Median
aged over 2 years had a higher mortality risk, but was not statisti- follow-up-time was 43 months for all cases, and 5-years OS was 83%,
cally significant (HR: 2.66; 95% confidence intervals (CI) 0.92–7.68, P = 10, 15-years OS were 79%. Nine patients relapsed, three of them
0.07). Lower OS and EFS were found in the MYCN amplification group primary, others systemic relapse. Median relapse time:10 months.
when compared with the nonamplification groups (45% [OS], 37.5% Additional three patients had refractory disease. Ten patients died
[EFS] versus 33.3% [OS], 16.6% [EFS], respectively). Cis-retinoic acid with disease progression. 5-years EFS was 68%,10, 15-years EFS were
treatment for 12 months appeared to be a strong prognostic factor that 61%.
could reduce mortality among patients with HR-NB (HR 0.27; 95% CI Conclusions: Obtained survival rates were similar with original proto-
0.09–0.785, P = 0.01). cols. No prognostic factor could be determined owing to the limited
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S600 of S635 ABSTRACTS

patient number but stage and presence of anaplasia are important PO078 / #338 MOVING FORWARD FOR BETTER
criteria. SURVIVAL;FIVE YEARS EXPERIENCE ON CHILDREN WITH
WILMS TUMOR IN NEPAL

PO077 / #1669 METASTATIC NEPHROBLASTOMA : ABOUT Dinesh Koirala1 , Geha Dahal2 , Rameshwor Pokhrel2
22 CASES IN THE CENTER OF TUNISIA 1 tribhuvan university teaching hospital, Paediatric Surgery, kathmandu,
Nepal; 2 tribhuvan university teaching hospital, Paediatric Surgery, Kath-
Imene Chabchoub1,2 , Rihab Ben Jaafar1 , Nouha Ammar3 , Tarek Ben mandu, Nepal
Ahmed1 , Nadia Bouzid4 , Rym Zanzouri5 , Sana Mosbahi6 , Rim
Bourigua1 , Imtinene Belaid1 , Makram Hochlef1 , Leila Ben Fatma1 , Background and Aims: Wilms tumor is the most common renal tumor
Faten Ezzaairi1 , Slim Ben Ahmed7 in childhood and is the second most abdominal tumor presenting in
1 CHU Farhat Hached, Medical Oncology, sousse, Tunisia; 2 CHU Farhat infants and children after neuroblastoma.Majority of children with
Hached/Faculty of medecine Sousse, Medical Oncology, sousse, Tunisia; tumor can be cured by multidiscilinary approach ie chemotherapy,
3 Farhat Hached Hospital/ University of medecine Sousse Tunisia, Med- surgery with or without radiotherapy.The survival is excellent with
icaloncology, sousse, Tunisia; 4 farhat hached hospital, Radiation Therapy, rates exceeding 90%.We present our experience of 5 years treat-
sousse, Tunisia; 5 Farhat Hached Hospital, Radiation Oncology, Sousse, ing wilms tumor in Tribhuvan Unveristy Teaching Hospital. Aim.To
Tunisia; 6 CHU Fattouma bourguiba, Medical Oncology, sousse, Tunisia; enlighten about the delay presentations of wilms tumor in developing
7 CHU Farhat Hached, Medical Oncology/ University Of Medecine Sousse, country like Nepal.
sousse, Tunisia Methods: A retrospective study of 20 patients with wilms tumor was
conducted 2018 to 2022 in Tribhuvan University Teaching Hospi-
Background and Aims: Metastatic nephroblastoma is a challenging tal, kathmandu Nepal.The clinical characteristic,diagnostic methods,
pedatric tumor with a 5 year survival rate over 90% in developed treatment and outcome were analyzed.
countries thanks to multidisciplinary management. However in the low Results: Of the 20 patients, 12 patients were boys with mean age of
middle income countries results are very heterogenous. The aim of our 4 years(range of 2 years -13 years).Eight patients with mass effect like
study is to evaluate the results of SIOP protocol in Tunisia, a limited constipation and respiratory distress.So they had undergone primary
resource country. radical nephrectomy with clinical and radiological diagnosis only.One
Methods: This is a retrospective study of 22 metastatic nephroblas- patient present with rupture of tumors causing abdominal distress
toma children treated with SIOP protocols (93-01 and SIOP2001) in and fever.They received chemotherapy after surgery.rest of the 11
the medical oncology department of Farhat hached university Hospital patients received preopeartive chemotherapy according to NWTSG
from January 1994 to December 2020. protocol.The median folllow up was 2.5 years (range from 1 year -5
Results: Patients with metastatic nephroblastoma represent 26.19% of year).Ultrasonography were generallt obtained every 3 months until
our population. The average age was 4 years( 8months – 8 years) with the children were 5 years of age. Neither recurrence nor death related
a female predominance ( 81.8%).Abdominal mass (65%) is the most to treatment has been observed.Overall and event free survival for 5
relevant symptom. Pulmonary metastasis was noted in 15 patients years is 90% considering two patients who have lost to follow up.
(68.18%), associated with liver metastasis in 3 patients (13.63%) and Conclusions: In developing countries like Nepal many children with
only liver metastasis in 4 patients (18.18%).All patients received 6 wilms tumor present delay with complications of mass effect.Surgery
cycles of neoadjuvant chemotherapy.A partial response was observed continues to play a crtical role in the treatment.Accurate diagnosis,
in 20 patients. All patients underwent radical nephrectomy. The his- safe and complete resection of wilms tumor with chemotherapy is key
tology was favorable in 3 patients, intermediate in 13 patients and elemets in achieving cure of 90% in our series
unfavorable in 5. All patients received postoperative chemotherapy.
Radiotherapy was indicated in 11 patients: 9 tumor bed radiother-
apy and 2 bipulmonary radiotherapy. 2 patients had a resection of PO079 / #182 WILMS TUMOR
lung metastases.The average duration of adjuvant chemotherapy was
20 weeks.Relapsed was observed in 36.3% of cases.Fifteen deaths Ana Santizo Gaitan, Alejandra Gonzalez, Pedro Alvarado Reyes,
were recorded in this series (68.18%).Overall survival for metastatic Martha Ramirez
nephroblastoma is 33.3% versus 76.7% for the localized stage.The Hospital General San Juan de Dios, Guatemala, Guatemala, Guatemala
prognostic factors for survival did not show any significant differ-
ence whatever the site of metastasis, the number, and the histological Background and Aims: Wilms tumor is the most common renal malig-
risk. nancy in children, with approximately 500 new cases per year. In
Conclusions: We noted a poor outcome of our patients compared to the United States, two-thirds of cases of Wilms tumor are diagnosed
the SIOP results. The local treatment of metastases with surgery and before five years of age and 95 percent before 10 years of age. In the
radiotherapy remains a challenge in our center. present case, the current response to treatment will be evaluated.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S601 of S635

Methods: Patient who from birth with abdominal distension. Fifteen of them with adverse characteristics. Disseminated disease at diag-
days prior, she began with decreased appetite, night sweats, nausea, nosis was observed in 35% of patients, 20% of them with isolated
and abdominal pain of moderate intensity located in the mesogastrium. pulmonary metastases. Induction chemotherapy consisted of 6 VIDE
She presented weight loss, so the mother decided to consult and evi- in all patients. Hematological toxicity (93%) and febrile neutropenia
denced an abdominal mass on the left side of 15 cm cephalocaudal and (97%) were the most common adverse reactions. Local treatment of
14 cm in transverse diameter, non-mobile, mild pain on palpation. Lab- primary tumors consisted in surgery in 64% of patients and radiother-
oratories with findings of acute renal failure and tumor lysis syndrome, apy in 46%. Adjuvant chemotherapy consisted of 7 courses of VAI in
for which intravenous fluid management at 3000 cc/m2 and allopuri- 68% and Bu-Mel in 15% of patients. After 7.9 years median follow-
nol is started immediately. A tomography was performed where the left up, 73% of localized good prognosis ES, 67% of the ones with adverse
kidney was reported with loss of normal architecture secondary to the characteristics, 100% of patients with isolated pulmonary metastases
presence of an extensive solid, heterogeneous mass measuring 20.9 and 50% of the other disseminated disease cases were alive without
x 11.8 x 12 cm. Increase in the number and size of peri-aortic lymph evidence of disease. There were 9 recurrences (1 local, 8 at distance)
nodes, predominantly left, the largest measuring 1.3 x 0.8 cm. Labora- and only one patient is still alive. Local control rate was 96%. DFS was
tories with LDH 835 IU/L, creatinine 0.37 mg/dl, uric acid 6.20 mg/dl, 81% and OS was 84% at 5 years. Considering only the localized good
BUN 7.60 mg/dl. prognosis ES, DFS and OS were 87% and 93% at 5 years, respectively.
Results: Patient started chemotherapy with adequate response and Conclusions: Despite the limited number of patients included, real
notable decrease in tumor size. After five weeks of chemotherapy she world data confirmed the survival rates observed in clinical trials.
is taken to surgery for mass resection. Finding retroperitoneal mass Unfortunately, recurrent disease remains a big challenge with a poor
of renal origin of approximately 20 x 20 cm with irregular edges and prognosis.
smooth surface with visualization of the renal hilum of firm consis-
tency and evidence of thickening and fibrosis of the left ureter and
adenopathy. PO081 / #811 ASSESSING SURVIVAL OF PAEDIATRIC
Conclusions: Currently in week fifteen of treatment, with adequate PATIENTS WITH OSTEOSARCOMA ACCORDING TO THE
response. The importance of managing oncological emergencies and THERAPEUTIC TECHNIQUES USED
multidisciplinary management with surgery, oncology is crucial for
excellent results. Maria Moschovi1 , Panagiota Kourou1 , Nikos Vlachogiannis2 , Maria
Nikita3 , Dorothea Stathaki1 , Margarita Mpaka3
1 “Agia Sophia” Childrens’ Hospital, National and Kapodistrian University of

Publication Only Topic: AS05 SIOP Scientific programme / AS05.g Athens, Division Of Hematology-oncology, Athens, Greece; 2 ‘Laiko’ General
Bone Tumours Hospital, National and Kapodistrian University of Athens, First Department
Of Propaedeutic And Internal Medicine And Nosology, Athens, Greece; 3 ‘P &
PO080 / #821 EWING SARCOMA IN PEDIATRIC PATIENTS: A A. Kyriakou’ Children’s Hospital, Department Of Oncology, Athens, Greece
REPORT OF INSTITUTIONAL RESULTS
Background and Aims: Background and aims Osteosarcoma is the
Flavia Fernandes1 , Cristiana Couto2 , Sofia Conde1 , Andreia Pires1 , most common bone malignancy among children with a high mortality
Artur Aguiar1 , Catarina Sousa2 , Cátia Sousa2 , Sílvia Silva2 , Ana rate. The most common approach of treatment is the combination of
Ferreira2 preoperative chemotherapy followed by surgical removal of the tumor.
1 IPO Porto, Radioterapia, Porto, Portugal; 2 IPO Porto, Pediatria Oncológica, The survival of patients with osteosarcoma has been studied, however,
Porto, Portugal there are no existing studies correlating patient’s survival and percent-
age of tumor necrosis after preoperative chemotherapy. Aim of our
Background and Aims: Ewing Sarcoma (ES) is the second most com- study is the assessment of the survival of children with osteosarcoma
mon bone cancer in children and adolescents. About 95% bear the that received preoperative chemotherapy and the correlation with the
chromosomal translocation t(11;22)(q24;q12). Multimodal therapy has percentage of tumor necrosis after the preoperative chemotherapy.
improved ES prognosis in the last decades. This study intends to Methods: A retrospective cohort study was performed. Data received
describe treatment modalities, toxicities and survival rates at our from the medical records of children, of all ages, who were diag-
institution. nosed with osteosarcoma at the “Agia Sofia” and “P&A. Kyriakou”
Methods: A retrospective study was conducted with all pediatric Children’s Hospitals and have completed five years of follow-up after
patients diagnosed with ES and treated in our institution, between the diagnosis.
2010-2019. DFS and OS were estimated using the Kaplan-Meier Results: Regarding demographics, 46.3% of the patients were female,
method. 56.7% of children were older than 12-years-old at the diagnosis
Results: Among the 28 eligible patients, 54% were girls. Median age while 35% of the children lived in urban areas. Regarding histolog-
was 11 years (5-17 years). t(11;22) re-arrangement was detected in ical subtype, the majority of the patients studied were diagnosed
93% of patients. Localized ES was observed in 65% of cases, 16% with osteoblastic osteosarcoma (62.3%), followed by chondroblastic,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S602 of S635 ABSTRACTS

fibroblastic, paraosteal, and mixed-type osteosarcoma. The percentage drome would likely create more risk of secondary malignancy, rather
of tumor necrosis was also calculated. 41.2% of the patients had tumor than tumor recurrence.
necrosis over 90% after the completion of preoperative chemother-
apy. During the 5-year follow-up, it was noted that among patients
with post-chemotherapy tumor necrosis over 90% have, a statistically Publication Only Topic: AS05 SIOP Scientific programme / AS05.h Soft
significant better prognosis and survival rate (p-value<0.05). These Tissue Sarcomas
patients have an 83% less probability of death (p-value<0.05). Further-
more, patients’ survival is correlated with tumor necrosis but not with PO083 / #1033 PROGNOSTIC FACTORS THAT AFFECT
other demographic factors investigated. SURVIVAL IN PATIENTS WITH EWING’S SARCOMA: TREATED
Conclusions: The results of this study suggest that the percentage of IN THE PEDIATRIC ONCOLOGY SERVICE OF THE NATIONAL
the tumor necrosis is a statistically significant prognostic factor regard- THIRD LEVEL MEDICAL CENTER
ing survival of children with osteosarcoma and can potentially be used
as an independent prognostic factor Eduardo Baños, Beth Sainz
National Medical Center, Pediatric Oncology, Mexico City, Mexico

PO082 / #1087 HIGH GRADE OSTEOSARCOMA OF THE Background and Aims: Ewing’s sarcoma (ES) represents the sec-
SKULL IN A LI-FRAUMENI PATIENT ond most common malignant bone tumor in children, with an overall
survival of 72% for patients with localized disease; the outcome for
Samuel Pabon-Rivera, Aaron Sugalski, Gail Tomlinson patients with metastatic disease continues to be poor with 28% OS.
UT Health San Antonio, Pediatrics, Division Of Pediatric Hematology- One of the most important risk factors is the presence of metastases
oncology, San Antonio, United States of America at the time of diagnosis and the site of the metastatic lesions; patients
with extrapulmonary metastatic lesions do significantly worse than
Background and Aims: Osteosarcoma of the skull is an extremely rare patients with isolated pulmonary metastasis. Other well-known risk
neoplasm with an incidence around 1-2% of all skull tumors. Based factors are the site of the primary tumor and the volume and/or size of
on the rare site of tumor, a high index of suspicion should raise con- the tumor. Histologic response, assessed after surgery, is an additional
cern for genetic cancer predisposition syndromes. There are not many important prognostic factor for overall survival; changes in tumor
cases described in the literature and most data are based on case series. volume after induction therapy correlate with histologic response to
These lesions are usually painless and tends to present in the third or chemotherapy.
fourth decade of life. Surgical resection is the mainstay, with complete Methods: A retrospective descriptive observational study was
surgical resection and wide surgical margins have been associated with carried out; in which records of patients with Ewing’s Sarcoma
improved survival. under 18 years of age were reviewed, in the period from Jan-
Methods: This is the case of a 14-year-old female patient with his- uary 2006 to December 2018, clinical and treatment factors were
tory of a solid lesion in the right skull for approximately eleven to analyzed.
twelve months before presentation. Patient was evaluated at an out- Results: A total of 29 patients were included, of which 17 were male
side institution with first partial surgical resection of the lesion that and 12 female with a M:F ratio of 1.4:1, with a mean age at diagnosis of
was consistent with a benign lesion. Despite surgical management, the 10.2 years. For patients with tumors >8cm, a relative risk of 1.41 was
lesion rapidly grew in the next six months, patient had a second surgery obtained, in patients >14 years the HR was 1.57; in patients with pri-
consistent with High Grade Osteosarcoma of the skull. Based on tumor mary bone tumors the HR was 1.27; patients with metastatic disease
rarity and family history of mother who died from breast cancer, high had a HR of 3 and of these those patients with combined metastatic
suspicious for Li-Fraumeni syndrome prompted evaluation for genetic disease had a HR of 2.14; for patients with DHL >250 at diagnosis, the
testing. HR was 0.76.
Results: Pathology slides reviewed in our institution revealed a diag- Conclusions: In our study, the main risk factors associated with mortal-
nosis of High-Grade Osteosarcoma, conventional osteoblastic type. ity in patients with Ewing’s sarcoma were the presence of metastatic
Genetic test revealed a pathogenic variant in the TP53 gene, c.949C>T disease, especially when it occurs in combination. Likewise, there is a
(p.Gln317) consistent with Li-Fraumeni syndrome. Patient received higher risk of mortality in patients >14 years of age and tumors. >8cm
two cycles of neoadjuvant chemotherapy with doxorubicin, methotrex- at diagnosis.
ate, and cisplatin. A surgical approach by neurosurgery and maxillo-
facial surgery revealed a poor response to chemotherapy within the
tumor consistent with 50% of necrosis. PO084 / #870 ASSOCIATION BETWEEN INFLAMMATORY
Conclusions: The modality that has demonstrated improved survival INDICES WITH PROGNOSIS AND OVERALL SURVIVAL IN
rates in patients with skull osteosarcomas is complete surgical resec- PEDIATRIC PATIENTS WITH SOFT-TISSUE SARCOMA
tion with chemo-radiation. Despite treatment, skull osteosarcoma
have a higher rate of local recurrence. The cancer predisposition syn- Andrea Padilla1 , Eduardo Baños2 , Farina Arreguin-González3
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S603 of S635

1 National Medical Center "20 de Noviembre", Pediatric Oncology, Benito There was no history of steroid intake. On examination child had
Juarez, Mexico; 2 National Medical Center "20 de Noviembre", Pediatric tachypnea with subcostal and intercostal retractions, and decreased
Oncology, Del Valle, Mexico; 3 National Medical Center, Pediatric Oncology, air entry in right mammary, infra mammary, axillary and infra axil-
Del Valle, Mexico lary areas. Child had features of Cushing syndrome including moon
facies, hirsutism, generalized swelling, obesity, and hypertension. She
Background and Aims: Soft-tissue sarcomas (STS) in children are a also had hypokalemia. Chest X ray was suggestive of right tho-
heterogeneous group of malignant tumors that arise in primitive mes- racic mass with right pleural effusion. Contrast enhanced computed
enchymal tissue and represent 7% of all pediatric tumors. There is evi- tomography revealed huge soft tissue mass lesion 9x5.7x5.9 cm fill-
dence that inflammation contributes significantly to the development ing in right hemi-thorax causing erosions of right 5th and 6th ribs.
of cancer. Histopathological examination revealed diagnosis of PNET. Investi-
Recently, the Neutrophil-to-lymphocyte ratio (NLR), Platelet-to- gations revealed hypokalemia, fasting hyperglycemia, elevated levels
lymphocyte ratio (PLR) and Systemic Immune-Inflammation Index (SII) of serum cortisol (1750 nmol/mL )and plasma ACTH (109.7 pg/mL).
have been studied as parameters of systemic inflammation in several As an emergency measure, 3 doses of cyto-reductive chemotherapy
diseases that could be associated with the evolution that they present. (vincristine 1 mg/m2 and cyclophosphamide 300 mg/m2) at 12-14
Methods: Observational, analytical, cross-sectional and retrospective days interval was given while awaiting HPE report and stabilization
study. Patients diagnosed with STS from the Pediatric Oncology Ser- from lower respiratory tract infection. Stress and maintenance dose
vice of the "20 de Noviembre" National Medical Center from January of hydrocortisone was given during this time. She had improving fea-
2010 to January 2015, recently diagnosed, under 18 years of age at tures of Cushing syndrome and serum cortisol (25.2 microgram/dL),
diagnosis, with complete blood count were included, their initial blood ACTH levels (26.10 picogram/mL ) were normalised after cytoreduc-
count was reviewed with emphasis on the absolute count of leukocytes, tive chemotherapy. Respiratory distress also improved but the size of
neutrophils, lymphocytes and platelets, calculating the NLR, PLR and tumour was marginally reduced. ACTH and cortisol were confirmed
SII. normal after one week of discontinuation of hydrocortisone. She is on
Results: There is an association of NLR with the presence of metastases VDC induction (Vincristine, Cyclophosphamide and Doxorubicin).
at diagnosis (p=0.025) and mortality (p=0.015) in pediatric patients Conclusions: Paraneoplastic Cushing syndrome should be considered
with STS, with a cut-off value of 2.0. In relation to the PLR, it is asso- in PNET presenting with Cushingoid features. It may respond very fast
ciated as a factor of poor prognosis for mortality with a value of with chemotherapy and does not co-relate with degree of tumour size
p=0.001, with a cut-off value of 100; as well as the SII with a cut-off response.
value of 300,000 with p=0.046, without finding a relationship with the
presence of metastases at diagnosis for PLR and SII.
Conclusions: Systemic inflammation indices are easy parameters to PO086 / #1861 PARAMENINGEAL RHABDOMYOSARCOMA:
obtain as prognostic factors in pediatric patients with Soft Tissue THE EXPERIENCE OF PEDIATRIC HEMATOLOGY AND
Sarcoma. ONCOLOGY CENTER OF RABAT (MOROCCO)

Maria El Kababri, Mohamed El Khorassani, Fatima Zohra El Azraq,


PO085 / #1929 RAPID NORMALISATION OF Mohamed Khattab, Leila Hessissen, Amina Kili
PARANEOPLASTIC ECPUBLICATION ONLY TOPIC Mohammed V University. Rabat, Pediatric Hematology And Oncology
ADRENOCORTICOTROPIC HORMONE (ACTH) SECRETION Department, RABAT, Morocco
AFTER CHEMOTHERAPY IN A CHILD WITH THORACIC
PRIMITIVE NEURO-ECTODERMAL TUMOUR (PNET) Background and Aims: Approximately 30% of childhood rhab-
domyosarcomas arise in the head and neck region; tumors that orig-
Narendra Chaudhary1 , Kundavaram Rajkumar2 , Bhavna Dhingra1 , inate in parameningeal sites account for one half of all lesions in this
Mahesh Maheshwari1 location. Tumors arising in parameningeal sites are at risk for central
1 All India Institute of Medical Sciences, Department Of Pediatrics, Bhopal, nervous system (CNS) extension.The aim of our study is to describe the
Madhya Pradesh, India; 2 All India Institute of Medical Sciences (AIIMS), experience of our center in the management of parameningeal RMS in
Department Of Peditrics, BHOPAL, India children and to evaluate the treatment results.
Methods: Our present study is a retrospective analysis of chil-
Background and Aims: Paraneoplastic ACTH dependant Cushing syn- dren with parameningeal rhabdomyosarcoma (RMS) treated in
drome is very rare in children. We present a case of PNET presenting the Pediatric Hematology and Oncology Center of the children’s
with paraneoplastic Cushing syndrome in which ACTH normalized hospital in Rabat (Morocco), from January 2014 to December
responded with one cycle of chemotherapy. 2018.
Methods: Case report Results: Sixty three new cases of pediatric rhabdomyosarcoma
Results: A one-year-old female presented with generalized swelling attended the pediatric hematology and oncology center of Rabat (5%
all over the body for 30 days and difficulty in breathing for 15 days. of childhood cancers). Thirteen cases of Parameningeal RMS were
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S604 of S635 ABSTRACTS

evaluated (21% of all RMS). The median age at diagnosis was 5 years Conclusions: The DW-MRI allows accurate characterization of
(2- 11 years). Males predominated (9M/4F). The average diagnostic rhabdomyosarcoma, assessment of treatment response, and
time was 2.5 months. Parameningeal location and tumor size were con- differentiation between recurrent tumor and post-therapeutic
firmed by CT and / or MRI in all patients. 11 patients had large tumors> changes.
5cm. Three patients had metastases at the time of diagnosis. The
diagnosis is confirmed by histology in all cases. The most common his-
tological type was the embryonal rhabdomyosarcoma. Chemotherapy PO088 / #1987 INFERIOR CLINICAL OUTCOMES OF
was performed in all patients, according to two protocols: MMT95 and PEDIATRIC RHABDOMYOSARCOMA IN THAILAND: A 16-YEAR
RMS2005. Radiotherapy was performed in 8 patients with a median EXPERIENCE IN A SINGLE TERTIARY INSTITUTION
dose of 50,4Gys. Surgical excision was performed in 2 patients. Five
patients were alive in their last follow-up, 1 of them with disease, Pornpun Sripornsawan1 , Ploypailin Preechawetchakul2 , Songyos
whereas 6 patients died due to the disease and 2 lost to follow-up. Rajborirug3 , Thirachit Chotsampancharoen2 , Surasak Sangkhathat4 ,
Conclusions: Parameningeal rhabdomyosarcome remains a diagnostic Pasuree Sangsupavanich2
and therapeutic challenge with an unfavorable prognosis. Local failure 1 Faculty of Medicine, Prince of Songkla University, Pediatrics, Hatyai, Thai-

is a major problem in children with parameningeal rhabdomyosarcoma land; 2 Prince of Songkla Univeristy, Pediatrics, Hatyai, Thailand; 3 Prince of
and meningeal involvement. Songkla Univeristy, Epidemiology Unit, Hatyai, Thailand; 4 Prince of Songkla
Univeristy, Surgery, Hatyai, Thailand

PO087 / #110 ROLE OF MRI DIFFUSION IN EVALUATION Background and Aims: There is limited data available on the treat-
THE THERAPEUTIC RESPONSE AND PROGNOSTIC OUTCOME ment outcomes of pediatric rhabdomyosarcoma (RMS) in Asian pop-
IN PEDIATRIC PATIENTS WITH RHABDOMYOSARCOMA ulations. Therefore, we aimed to review the baseline characteristics,
clinical outcomes, and prognostic factors in children with RMS from
Inas Elnady1 , Rehab Husein2 , Tarek Rafaat3 , Medhat Rafaat3 Thailand.
1 children cancer hospital of egypt, Pediatric Oncology, Cairo, Egypt; Methods: The data of children under 15 years of age diagnosed with
2 children cancer hospital of egypt, Radiology, Cairo, Egypt; 3 children cancer RMS between 2003 and 2019 from a large tertiary hospital in Southern
hospital of egypt, Radiology, d, Egypt Thailand were retrospectively reviewed. Descriptive statistics were
utilized to describe the clinical characteristics. The Kaplan–Meier
Background and Aims: Background: Childhood rhabdomyosarcoma method was utilized to estimate survival. Cox proportional hazards
accounts for about 3.5% of cancer cases among children 0 to 14 years regression analysis was utilized to determine prognostic factors that
of age. The most common sites are the head and neck. Early detec- affect survival.
tion of treatment response might change therapeutic strategies and Results: A total of 42 children RMS were included in this study. The
unnecessary toxicity might be avoided. The differentiation between median age at diagnosis was 6.4 years (IQR, 2.4–10.2). Among these
post-therapeutic changes and recurrent tumors in RMS is a diagnos- patients, 11 (26%) were older than 10 years, and 13 (31%) presented
tic dilemma. DWI helps predict and monitor treatment response in with metastatic disease at diagnosis. The 5-year overall survival (OS)
RMS, as changes in ADC precede changes in tumor size. For the dif- rate was 39% for all children. Age greater than 10 years (hazard ratio
ferentiation between recurrent tumor and post-therapeutic changes, (HR): 3.3, 95% CI: 1.2–9.2) and metastatic disease at diagnosis (hazard
qualitative and quantitative assessment using DWI seems to be helpful. ratio (HR): 2.8, 95% CI: 1.1–7.5) were independently associated with
Methods: Patients and methods: During the period between April poorer survival. The 3-year OS for children with metastatic disease
2017 to April 2019, 40 pediatric patients with rhabdomyosarcoma (24 (stage IV) was 15% (95% CI: 4.3–55).
male and 16 female) were enrolled prospectively. All patients under- Conclusions: The percentage of metastatic disease in our cohort was
went standard MRI protocol and DW-MRI. PET/CT was done and higher than that in previous reports and may have contributed to a
correlate the SUV/MAX value with the DWI results. MRI diffusion poorer outcome. Age greater than 10 years and metastatic disease at
finding were correlated to different risk factors and survival diagnosis were noted as adverse prognostic factors.
Results: The current study included 40 pediatric patients with RMS,
their ages at the time of the study ranged between 10 months to 15
years. The male patients represent 60%, while the female patients rep- PO089 / #727 A CASE OF INFANTILE FIBROSARCOMA
resent 40% of the total examined cases (24 male and 16 female). The SUCCESSFULLY TREATED
head and neck are the seats of more than 72 %. In diffusion MRI; 28.9%
of cases were hyperintense, 29.4% were hypointense, 9.6% were isoin- Gulsah Tanyıldız1 , Deniz Tuğcu1 , Şifa Şahin1 , Mustafa Bilici1 , Serap
tense while 32% presented mixed signals. The calculated ADC values of Karaman1 , Zeynep Karakaş1 , Bilge Bilgiç2 , Ahmet Salduz3 , Ayşegül
the lesions ranged from 0.30 to 2.8 x 10-3 mm2/sec with a mean value Ünüvar1
of 1.5x10-3 mm2/sec. The calculated SUV/MAX values of the lesions 1 Istanbul University Istanbul Medical Faculty, Pediatric Oncology, Bey-
ranged from 0.90 to 10.90 with a mean value of 4.06. oğlu, Cihangir, Turkey; 2 Istanbul University Medical Faculty, Pathology,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S605 of S635

Beyoğlu, Cihangir, Turkey; 3 Istanbul University Medical Faculty, Orthope- Background and Aims: Tumours can mimic vascular malformations
dics, Beyoğlu, Cihangir, Turkey and vice versa. We report four children with malignant lesions initially
misdiagnosed as benign vascular malformations.
Background and Aims: Infantile Fibrosarcoma (IFS) usually occurs Methods: Retrospective review of children with malignancies mim-
in children aged younger than 1 year.The tumor is usually large icking vascular malformations in our institution, KK Women’s and
at diagnosis while the tumor grows so quickly. Mostly IFS shows Children’s hospital.
t(12;15) (p13;q25) translocation which results in ETV6-NTRK3 (TEL- Results: Case 1: Three-year-old boy with a thigh mass, present from
TRKC) gene fusion.Especially in the pediatric age group where the birth as an erythematous lesion before developing into a soft bluish
use of chemotherapy and radiotherapy is inconvenient selective TRK swelling. Ultrasound revealed 2x1.7x0.9cm heterogeneous subcuta-
inhibitors promise better morbidity rate. neous structure with both arterial and venous vascularity possibly
Methods: Female patient on the first postnatal day applied to the pae- representing vascular malformation. MRI eight months later demon-
diatric haematology and oncology clinic with the complaint of mass in strated significant increase in size of 4x2.7x3.7cm, with histology of
the right foot. A mass was observed including the dorsum and sole of low-grade spindle cell sarcoma on excision. Case 2: Nine-year-old
the right foot, with necrotic areas on it. boy with a right 3x3cm infraclavicular hard mass with bluish overly-
Results: Computed tomography angiography of the extremity showed ing skin, initially suspected to be a bleeding lymphatic malformation.
a heterogeneous hyper-vascular lesion surrounding the tarsal and Ultrasound showed hypoechoic mass with some vascularity. The mass
metatarsal bones; It was found to be compatible with the malignant increased in size rapidly within a month with overlying skin erythema.
mesenchymal tumour image. True-cut biopsy was performed and it was MRI revealed a 10x8cm subcutaneous, solid lobulated mass, suspi-
reported as spindle cell sarcoma. The diagnosis of the patient with cious for mitotic lesion. Histology from incisional biopsy confirmed
ETV6-NTRK fusion signal was confirmed as infantile fibrosarcoma. alveolar rhabdomyosarcoma. Case 3: Four-year-old girl with a grad-
As the treatment regimen; vincristine, actinomycin and cyclophos- ually enlarging forehead lesion over five months. Ultrasound showed
phamide were planned, but because the tumour tissue did not regress 2x2cm superficial anechoic cystic swelling which had high colour flow
in the follow-up and was not suitable for surgical intervention, it with arterial spectral pattern, suggestive of intramuscular haeman-
was switched to Larotrectinib treatment. A reduction in macroscopic gioma. MRI demonstrated an extra-cranial solid lesion with areas of
dimensions was observed in the mass of the patient who was evaluated bony erosion. Incisional biopsy revealed lymphoblastic lymphoma. Full
in the second month of Larotrectinib treatment. blood count and blood film were normal. Case 4: Eighteen-month-old
Conclusions: The morbidity rate of IFS is not high, while morbid- girl with a right cheek swelling from birth, conservatively managed
ity rate is relatively higher and organ loss is possible depending on as a hemangioma. This increased in size with obstruction of vision
organ location.Since IFS seen mostly seen younger than 1 year of from 5 months old. MRI showed 4x6.5x8.5cm mass with histology of
age continuation of chemotherapy may not be possible and heavy embryonal rhabdomyosarcoma on biopsy.
side effects may be observed.The choice of chemotherapy regimen Conclusions: These cases highlight that appropriate treatment of
is of paramount importance. Also, this situation demands selective malignant tumours may be delayed if dismissed as benign vascular mal-
inhibitors other than systemic therapies.Larotrectinib is a selective formations. Any doubts should be resolved with biopsy especially in
TRK inhibitor, it can orally be given at ETV6-NTRK3 fusion-positive patients with older age, solid mass and rapid increase in size.
cases.

Publication Only Topic: AS05 SIOP Scientific programme / AS05.i


PO090 / #1569 MALIGNANT TUMOURS MIMICKING Retinoblastoma
VASCULAR MALFORMATIONS; A REPORT OF FOUR CASES
PO091 / #424 CLINICAL PROFILE, MANAGEMENT AND
D Khawn Tawng1 , Amanda Yap2 , Mya Soe Nwe1 , Mark Koh3 , Amos OUTCOME OF RETINOBLASTOMA: A TERTIARY CARE CENTER
Loh4 , Michaela Su-Fern Seng1 , Rajat Bhattacharyya1 , Ah Moy Tan5 , EXPERIENCE, INDIA
Shui Yen Soh6 , Mei Yoke Chan1
1 KK Women’s and Children’s Hospital, Paediatric Haematology/ Oncol- Monika Bhukar1 , Shobha Badiger2 , Pooja Mallya2 , Ravi Joshi2 , Shweta
ogy, Singapore, Singapore; 2 KK Women’s and Children’s Hospital, Paediatric Pathak2 , Swathi P M2 , Gayathri S2 , Sunil Bhat2
Haematology/oncology, Singapore, Singapore; 3 KK Women’s and Children’s 1 Mazumdar Shaw Medical Center, Paediatric Hemato-oncology & Bmt,
Hospital, Department Of Dermatology, Singapore, Singapore; 4 KK Women’s Bangalore, India; 2 Mazumder Shaw Medical Center, Paediatric Hemato-
and Children’s Hospital, Department Of Paediatric Surgery, Singapore, oncology And Bmt, Bangalore, India
Singapore; 5 KK Women’s and Children’s Hospital, Dept Of Pediatrics, Singa-
pore, Singapore; 6 KK Women’s and Children’s Hospital, Paediatric Haema- Background and Aims: Retinoblastoma (RB) is the most common
tology Oncology Service, Children’s Blood And Cancer Centre, Singapore, primary intraocular malignancy of childhood with incidence of 1 in
Singapore 15000–20000 live births. The overall survival is approximately 88%
with multidisciplinary treatment. This study was aimed to describe the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S606 of S635 ABSTRACTS

clinical characteristics and treatment outcomes in pediatric patients hospital and the French African Paediatric Oncology Group (GFAOP).
with retinoblastoma. Clinical features, management of patients and outcome before and
Methods: This was a retrospective study conducted in a tertiary care after improvement of care were compared.
center at Mazumdar Shaw Medical Center, Bangalore. Data of all Results: A total of 113 patients was registered with 79% unilateral RB,
patients with retinoblastoma from January 2014 to June 2021 was 16.8% bilateral and 2.6% trilateral at the presentation. The mean age
analyzed. at presentation was 2.49 years. Leukocoria was the most common pre-
Results: We had 91 patients of which 46 were male and 45 were senting sign (61.3%) and the extra-orbital form was the most seen at
female. Mean age at presentation was 25 months ranging from 1 diagnosis (60%). From 2008 to 2014, the recruitment of patient was
month to 154 months (31 months versus 17 months in unilateral poor and patients of all stages was treated with a same regimen with
and bilateral cases, respectively) with unilateral presentation of RB no histological analysis after surgery. All patients abandoned of died
in 54% (n=49) and bilateral in 46% (n=42) with positive family his- during treatment and no remission was registered. After 2014, the
tory in 3 patients. Leukocoria was most common presenting symptom recruitment was better and treatment was given according to the stag-
(79%) followed by proptosis (13%). Using the International Classifi- ing at diagnosis. 70.7% of patients had histopathological report after
cation of Retinoblastoma (ICRB); group A, B, C, D & E were 1.5%, surgery and we registered 20% of complete remission after completion
5.3%, 4.5%, 27.1% and 61.6%, respectively. Metastasis to the CNS of treatment.
was seen in 11 patients (including 3 pineoblastoma cases) and 1 had Conclusions: The management of RB is still a problem because of poor
marrow involvement. Most common modalities of treatment included survival. However, improvement of hospital care can increase the sur-
systemic chemotherapy (n=91, 100%), and enucleation (n=44, 48.3%). vival rate. Late presentation and abandonment of curative treatment
Focal therapy with laser photocoagulation, cryotherapy and intrav- need to be addressed.
itreal chemotherapy was administered in 51.6%, 25.2% and 26.3%
patients, respectively. Nine patients (9.9%) received external radio-
therapy (3 plaque brachytherapy and 6 external beam radiotherapy). PO093 / #1250 RETINOBLASTOMA IN JORDAN: INCIDENCE,
Globe preservation was achieved in 46.7% patients with unilateral RB DEMOGRAPHICS, AND SURVIVAL
and 57% patients with bilateral RB. The 5 year overall survival rate was
87.9% (89.6%, and 86% for unilateral RB and bilateral RB, respectively). Hadeel Halalsheh1 , Iyad Sultan2 , Ibrahim Qaddoumi3 , Ibrahim
Conclusions: Globe preservation rate is lower in developing coun- Nawaisheh4 , Yacoub Yousef4
tries, as most of the patients present with advanced disease. Better 1 King Hussein Cancer Center, Pediatric, Amman, Jordan; 2 King Hussein
health education, public awareness, and earlier screening shall improve Cancer Center, Pediatrics, Amman, Jordan; 3 St. Jude Chidlren’s Research
survival. Hospital, Global Pediatric Medicine, Memphis, United States of America;
4 King Hussein Cancer Center, Surgery/ophthalmology, Amman, Jordan

PO092 / #1974 IMPACT OF IMPROVING ACCESS TO Background and Aims: Sufficient epidemiology about intraocular
TREATMENT IN THE MANAGEMENT OF RETINOBLASTOMA IN tumors is missing in the Middle East in comparison to the developed
KINSHASA UNIVERSITY HOSPITAL world. We present an epidemiological analysis of retinoblastoma (RB)
in Jordan to aid national and regional strategies for improved ocular
Aleine Budiongo1 , Jerome Badhoka1 , Nina Domo1 , Karim Assani2 , cancer surveillance and control.
François Beya1 , Moïse Mvitu3 , René Ngiyulu1 , Jean Lambert Ehungu1 Methods: A retrospective cohort of all Jordanian RB (n=124)
1 Kinshasa University Hospital, Pediatrics, Kinshasa, Congo, Republic of; patients diagnosed over a 10-year period (2011-2020). Outcome
2 University hospital of Kinshasa, DR Congo and AMCC, Pediatrics, Kin- measures included incidence, demographics, eye salvage, and
shasa, Congo, Republic of; 3 Kinshasa University Hospital, Ophthalmology, survival.
Kinshasa, Congo, Republic of Results: Retinoblastoma (n=124) patients had no sex predilection, and
leukocoria was the most common presenting sign in 88 (71%) patients
Background and Aims: Retinoblastoma (RB) is the most common followed by squint in 25 (20%) patients. The median age at diagnosis
intraocular malignancy in children. Survival of children with RB in of RB was 15 months (Six and 28 months for bilateral and unilat-
resource-limited countries is very poor compared to high income coun- eral cases, respectively). Fifty-one (41%) patients had bilateral RB, and
tries. This situation can be improved with better access to effective 18 (15%) had familial disease. The mean age-adjusted incidence was
and affordable treatment. We describe the management of RB and 8.2 cases per-million-children per year for children aged five years or
the outcome in the Kinshasa university hospital from January 2008 to less (one per 15620 newborn per year). Fifty-one(41%) had bilateral
December 2019. disease, and 18(15%) had familial disease. Ninety-six(55%) eyes were
Methods: A 12-years retrospective review of all patients treated for group D or E (78% were T3/T4), the overall eye salvage rates were 37%
RB in the Kinshasa university hospital from January 2008 to Decem- (n=27/73) for patients with unilateral disease and 76% (n=78/102)
ber 2019 was conducted. Improvement of care of childhood cancer for patients with bilateral disease, and the five-year survival rate was
was observed after 2014 consecutive to the collaboration between the 96%.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S607 of S635

Conclusions: The incidence of RB in Jordan is comparable to that in Irene Medina Castillo1 , Xochitl Ramirez1 , Jorge Ramirez Melo1 ,
Western countries, and both survival and globe salvage rates are equal Graciela Gonzalez2 , Fernando Antonio Sánchez Zubieta1 , Marilyn
to those in the Western countries because all patients are treated in Gutiérrez1 , Norma Llamas1
one specialized center. 1 Hospital Civil Juan I Menchaca, Hemato-oncology Pediatrics, GUADALA-
JARA, Mexico; 2 Hospital Civil Fray Antonio Alcalde, Ophthalmology Pedi-
atrics, GUADALAJARA, Mexico
PO094 / #886 ADHERENCE TO THE TREATMENT OF
RETINOBLASTOMA IN SUB-SAHARAN AFRICA: STUDY Background and Aims: Retinoblastoma is the most common intraocu-
PERFORMED IN IVORY COAST AND THE DEMOCRATIC lar malignancy in childhood. The purpose of this study was to describe
REPUBLIC OF CONGO (DRC) the clinical presentation and outcomes of the treatment of children
with unilateral retinoblastoma.
Robert Lukamba1 , Aleine Budiongo2 , Ben Monga3 , Jean-Jacques Yao4 , Methods: A retrospective study of 95 patients with unilateral
Gabrielle Chenge5 , Laurence Desjardins6 , Pierre Bey7 , Francois Doz8 , retinoblastoma treated between 2001 and 2021. The electronic
Albert Mwembo3 , Théo Kabesha9 , Oscar Luboya1 records of all patients were used to extract the relevant data.
1 Cliniques universiatires de Lubumbashi, Pediatrics, Lubumbashi, Congo, Results: The median age was 25 months (range 1-120 months), and
Republic of; 2 Cliniques universiatires de Kinshasa, Pediatrics, Kinshasa, the male-female ratio was 1:1.3. Unilateral enucleation was performed
Congo, Republic of; 3 Université de Lubumbashi, Public Health, Lubum- for 83 children (87,3%) and 12 children underwent salvage therapy
bashi, Congo, Republic of; 4 CHU de Treichville, Pediatrics, Abidjan, Côte (12,6%). Most of the patients were classified in stage E (55.7%). 29
d’Ivoire; 5 Cliniques universiatires de Lubumbashi, Ophtalmology, Lubum- patients had positive optic nerve invasion up to the cut end (n=7; 24%),
bashi, Congo, Republic of; 6 Institut Curie, Ophtalmology, Paris, France; prelaminar (n=13; 44.8%), laminar (n=2; 6.8%), and post laminar (n=7;
7 Institut Curie, Amcc, Paris, France; 8 Institut Curie, Siredo Oncology 24.1%). Neoadjuvant chemotherapy was used in 27,3% of cases. 7 chil-
Center (care, Innovation And Research For Children And Aya With Can- dren were treated with conservative treatment with thermotherapy
cer),université De Paris, Paris, France; 9 Université offcicielle de Bukavu, (2), laser (4) intravitreal topotecan (1) and 46 patients underwent post-
Ophtalmology, Bukavu, Congo, Republic of enucleation surveillance. Adjuvant chemotherapy was given in 24,2%
of patients. With a median follow-up of (range 1-167 months), the 5-
Background and Aims: Retinoblastoma is curable in more than 95% year event-free survival (EFS) and the overall survival (OS) was 80 ± 5
of cases in developed countries, whereas mortality remains high in % and 60 ± 5% respectively. 23% abandoned treatment.
low-income countries, especially when the diagnosis is made late or Conclusions: The delay in the early detection of retinoblastoma and
treatment is discontinued. The aim of this work is to determine factors the rate of ocular salvage in this cohort was similar to other low- and
associated with adherence to treatment of retinoblastoma in the Ivory middle-income countries, it was lower related than reports derived
Coast and the Democratic Republic of Congo (DRC). from developed countries. We hypothesize that it could be related to
Methods: A retro-prospective cohort study was carried out. Data was the advanced disease stage at diagnosis.
collected from patient folders and parents’ follow-up records.
Results: A total of 175 children with retinoblastoma were registered
from January 2013 to December 2015. Seventy-six children (43%) Publication Only Topic: AS05 SIOP Scientific programme / AS05.j Liver
were 5 years old and above. Care costs was covered by families Tumours
in 86.9% of cases. Chemotherapy refusal was recorded in 39 cases
(22.3%) and enucleation refusal in 79 cases (45.1%). After 36-months PO096 / #923 A CONSECUTIVE SERIES OF UNUSUAL LIVER
follow-up, we recorded 16.6% deaths, 27.4% treatment drop-outs and TUMORS FROM A SINGLE HOSPITAL
18.3% loss to follow-up. The commonest cause for enucleation refusal
was fear of infirmity. On the other hand chemotherapy refusal and Anju Jacob1,2 , Awadelkarim Omer3,4 , Samina Afzal5 , Abid Qazi3,4
absconding on treatment were due to financial constraints. 1 Mohammed Bin Rashid University of Medicine and Health Sciences
Conclusions: Poor adherence to retinoblastoma management was due (MBRU), Pediatrics Residency, dubai, United Arab Emirates; 2 Al Jalila
to financial constraints, lack of knowledge of the disease as well as of Childrens Specialty hospital, Pediatrics, Dubai, United Arab Emirates; 3 Al
its treatment. Family psychosocial support is needed to improve this Jalila Childrens Specialty hospital, Pediatric Surgery, dubai, United Arab
condition. Emirates; 4 Mohammed Bin Rashid University of Medicine and Health Sci-
ences (MBRU), Pediatrics Surgery, dubai, United Arab Emirates; 5 American
Hospital, Pediatric Oncology, dubai, United Arab Emirates
PO095 / #1946 CLINICAL PRESENTATION AND TREATMENT
OUTCOMES OF UNILATERAL RETINOBLASTOMA: A 21-YEAR Background and Aims: Hepatoblastoma (HB) is the commonest malig-
EXPERIENCE IN MEXICO nant liver tumor in young infants followed by hepatocellular carcinoma,
which is seen in older children.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S608 of S635 ABSTRACTS

Methods: Case Series We present a consecutive case series of six Results: Testicular NGCT: Ten male patients with median age 6.0 years
children with liver tumors, who had unusual presentations or co- (0.3-16 years). Tumor histology included 4 cases of sex cord-stromal
morbidities. Four children presented with abdominal distension; one tumors (Sertoli-Leydig,N=2, mixed Sertoli-Leydig,N=1, juvenile gran-
child presented with isolated precocious puberty (PP)while the other ulosa cell tumor,N=1), 5 cases with primary mesenchymal tumors
child was found incidentally to have a liver tumor at the revision of ven- (rhabdomyosarcoma,Ν=4, leiomyosarcoma,Ν=1) and 1 case of pedi-
tricular peritoneal shunt. Serum alpha-fetoprotein levels were found atric follicular lymphoma. Elevated AFP (605 ng/ml) was detected in
to be elevated in all patients except one. One of the patients was one patient (juvenile granulosa cell tumor). All patients underwent
diagnosed with cat eye syndrome at birth. He had high anorectal mal- total surgical excision and the 5 patients (rhabdomyosarcoma,Ν=4,
formation for which colostomy was performed at birth. Another child leiomyosarcoma,Ν=1) received chemotherapy according to CWS
had chronic kidney disease due to hyperoxaluria and had multifocal treatment protocol. No event or death occurred. Ovarian NGCT: Ten
liver lesions at presentation. female patients with median age 12.5 years (range 8-16 years). Nine
Results: Core needle biopsy of the liver in five out of six children with primary ovarian tumor and 1 with acute lymphoblastic leukemia
confirmed the diagnosis of HB, whereas the 6th patient had fibro- relapse. The primary ovarian tumors included 5 cases of sex cord-
lamellar hepatocellular carcinoma (FLHCC) and underwent primary stromal tumors (Sertoli-Leydig:Ν=1, mixed Sertoli-Leydig with rhab-
resection. All children presented before 3 years of age except the child domyosarcoma component:N=1, juvenile granulosa cell tumor:Ν=1,
with FLHCC who was 11 years old. All children with HB underwent sclerosing stromal tumor:Ν=1, small cell carcinoma of the ovary with
PRE Treatment EXTent of tumor (PRETEXT) staging.FLHCC was com- hypercalcemia (SCCOH):N=1), 4 cases of epithelial tumors (serous
pletely resected with left hepatectomy. One patient was PRETEXT 1 cystadenoma:Ν=3, mucinous tumor:Ν=1). Elevated AFP(204,4 ng/ml)
who underwent primary resection. Another patient with PP, raised β- was detected in 1 patient with mixed Sertoli-Leydig tumor. Nine
hCG and AFP was PRETEXT 1M with multiple lung metastasis and patients underwent total surgical excision and 4 received platinum-
received three blocks of chemotherapy with the very high-risk arm of etoposide based chemotherapy. One patient with SCCOH underwent
SIOP protocol followed by a right hepatectomy. Two patients with PRE- autologous bone marrow transplantation. Two patients died: one due
TEXT 3 and 4 respectively underwent chemotherapy. One patient with to metachronous SCCOH in the contralateral ovary and one due to
PRETEXT 4 with chronic kidney disease due to hyperoxaluria received second primary malignancy. Ten-year EFS and OS were both 55%.
palliative therapy. Conclusions: NGCT are rare tumors in childhood, comprising het-
Conclusions: HB is mostly sporadic but can show a syndrome asso- erogeneous histology. The surgical excision with or without adjuvant
ciation. Precocious puberty in boys invariably has an underlying chemotherapy is the gold standard of therapy. Subsequent primary
pathologic cause that requires prompt comprehensive investigation. malignancies probably due to underlying predisposition syndrome and
Associated disorders with HB pose special decision-making difficulties previous chemotherapy are significant causes of death.
in addition to treatment becoming more demanding.

PO098 / #921 PEDIATRIC AND ADOLESCENTS WITH


Publication Only Topic: AS05 SIOP Scientific programme / AS05.k EXTRACRANIAL GERM CELL TUMORS. EXPERIENCE IN A
Germ Cell Tumours HOSPITAL IN LATINOAMERICA

PO097 / #1426 GONADAL NON GERM-CELL TUMORS; Carolina Basile1,2 , Alejandra Casanovas3,4 , Diego Amaral4 , Ernesto
OUTCOME OF PATIENTS AND LONG TERM EXPERIENCE OF A Veber4
SINGLE CENTER 1 Hospital de Niños Pedro de Elizalde, Pediatric - Oncohematology Section -

Aya Group, Buenos Aires, Argentina; 2 Hospital de Clinicas Jose de San Mar-
Kondylia Antoniadi1 , Vasiliki Tzotzola2 , Vassilios Papadakis1 , tin/ Hospital de Niños Pedro de Elizalde, Oncohematology Section, Buenos
Charikleia Kelaidi1 , Mirella Ampatzidou1 , Sofia Papargyri1 , Kalliopi Aires, Argentina; 3 Hospital de Niños Pedro de Elizalde, Pediatric - Onco-
Stefanaki3 , Sophia Polychronopoulou1 hematology Section, Buenos Aires, Argentina; 4 Hospital de Niños Pedro de
1 “Aghia Sophia” Children’s Hospital, Department Of Pediatric Hematology- Elizalde, Oncohematology Section, Buenos Aires, Argentina
oncology, Athens, Greece; 2 Aghia Sophia Children Hospital, Department Of
Pediatric Hematology-oncology, Athens, Greece; 3 “Aghia Sophia” Children’s Background and Aims: Extracranial Germ cell tumors (GCTs) aris-
Hospital, Pathology Department, Athens, Greece ing in pediatric and adolescents present a set of special challenges.
Revised Risk-based staging for pediatric and adolescent GCTs sepa-
Background and Aims: To present the long-term experience and out- rated patients into low risk - LR (located and alpha fetoprotein (AFP)
come of patients with gonadal non-germ cell tumors (NGCT) of a single minor than 15000ng/ml) and high risk- HR (metastatic and/or AFP
Pediatric Hematology-Oncology center. major than 15000ng/ml) groups. Low risk performs only surgery or
Methods: Twenty patients with NGCT diagnosed during 1977-2020 chemotherapy with Vinblastine/bleomycin/cisplatin (VBP). High risk
are included in the study. The male:female ratio was 0.55 and the undergo chemotherapy with Ifosfamide/Etoposide/cisplatin (VIP). The
median age at diagnosis was 8.9 years (range 4 months-16 years). aim of this study is to describe the epidemiological characteristics,
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S609 of S635

clinical features, histological type and outcome of GCTs in an institution Adjuvant chemotherapy was indicated in 66 patients. With a median
for the last 12 years by pediatric oncologists. follow-up of 264 months (1999-2021), the 5- year overall survival (OS)
Methods: Epidemiological and retrospective study. We reviewed the was 89.4%
medical records of patients (p) with GCTs treated in the oncology Conclusions: This study confirms the good outcome for children with
pediatric department of a Hospital from January 2009 to December testicular tumors. Survival is substantially due to accurate diagnosis
2021. and effective treatment.
Results: Fifty four p. Median age:6 years (18 days-18 years). Male: 35p
(65%) Female 19p (35%). pediatric:50p (92%); adolescents 4p (8%).
Type of tumors: embryonal carcinoma 4p (7%), mature teratoma 19p PO100 / #1536 MEIGS’ SYNDROME WITH ELEVATED SERUM
(35%) (MT), endodermal sinus tumor 17p (31%)(SE), mixed tumor 7p CA125 LEVEL AND URINARY DIVERTICULA IN A YOUNG GIRL
(13%), immature teratoma 7p (13%). Location: testicle 34p (62%), ovary
10p (18%), lungs 1p (2%), neck 1p (2%), abdominal 2p (4%), vulva Shahnoor Islam1 , Akm Morshed2
1p (2%), sacro-coccygeal 5 (3%). Localized primary tumor 39p (72%), 1 Dhaka Medical College Hospital / Ministry of Health and family welfare,
3p (7.6%) with AFP greater than 15,000ng/ml, 1p (2.5%) of them did Paediatric Surgery, Dhaka-, Bangladesh; 2 Directorate General of Medical
VBP. Metastatic 15p (28%), 3p (20%) received VBP, two (13%) of them Education, Pediatric Hematology And Oncology, Dhaka-, Bangladesh
progress and were rescued with VIP. The rest received VIP, 3p (5.5%)
progressed and died due to disease. Background and Aims: Meigs’ syndrome is defined as a condition that
Conclusions: Most of the p treated were pediatric. The most frequent meets the diagnostic criteria : the presence of pleural effusion and
histology were MT and SE. Most patients were localized. Patients who ascites; the existence of benign and solid ovarian tumors with the gross
progressed and died were metastatic. Although it is a small cohort, the appearance of a fibroma (fibroma, thecoma, and granulosa cell tumor);
good evolution of the RH localized patients who received LR treatment, and the ascites and pleural effusion rapidly resolve after removal of the
incentive to consider a modify intensity of treatment. tumor. Meigs’ syndrome with elevated serum cancer antigen 125 (CA
125) levels is unusual. Many reports have suggested that the presence
of ascites is the major factor that inducing mesothelial expression of CA
PO099 / #1990 CLINICAL PRESENTATION AND OUTCOMES 125. We report a case of Meigs’ syndrome with elevated serum CA125
IN CHILDREN WITH TESTICULAR MALIGNANT GERM CELL level, urinary diverticula in a girl.
TUMORS TREATED IN A TERTIARY CENTER IN MEXICO Methods: Case reports An eleven year - old Bangladeshi girl was admit-
ted to our hospital with a lower abdominal mass.Contrast Enhanced
Marilyn Gutiérrez1 , Manuel Martínez2 , Irene Medina Castillo1 , Jorge Computed Tomography Scan of chest, abdomen and pelvis confirmed
Ramirez Melo1 , Fernando Antonio Sánchez Zubieta2 , Rina Medina1 the presence of left sided pleural effusion, ascites with a left ovar-
1 Hospital Civil Juan I Menchaca, Hemato-oncology Pediatrics, GUADALA- ian mass. With a suspicion of Meigs’ syndrome, the patient underwent
JARA, Mexico; 2 Hospital Civil de Guadalajara Dr. Juan I Menchaca., surgical resection of ovarian mass, urinary diverticula and histopatho-
Hemato- Oncología Pediátrica, Hospital Civil De Guadalajara Dr. Juan I logical examination of resected mass showed ovarian fibroma which is
Menchaca, Guadalajara, México, Guadalajara, Mexico benign in nature. Pleural effusion as well as ascites resolved after tumor
resection, confirming a diagnosis of Meigs’ syndrome. The unevenful
Background and Aims: Background/Objectives: In pediatric oncol- postoperative period was associated with returned to normal serum
ogy, testicular tumors are examples of rare neoplasms that present a CA 125 level also.
bimodal pattern of incidence. The objective was to describe the treat- Results: The patient is in good health now and on regular follow up.
ment outcomes of children with testicular malignant germ cell tumors Conclusions: It is important for the clinician to consider the possi-
treated in a tertiary center in Mexico. bility of Meigs’ syndrome in a girl with unexplained pleural effusion,
Methods: Design/Methods: A retrospective study of 66 children ascites and an ovarian tumor. Although a malignant disease should
with testicular tumors treated between 1999 and 2021. The elec- be suspected in all patients with undiagnosed pleural effusion, Meigs’
tronic records of all patients were used to extract the relevant syndrome is a disease curable by tumor resection and should be
data. differentiated from malignancy.
Results: 66 patients were operated on because of testicular tumors.
The presenting symptom was a testicular mass gradually increasing
in size in 51 (51; 77.2%) children. Prepubertal testicular tumors were Publication Only Topic: AS05 SIOP Scientific programme / AS05.l Rare
present in 43 (43;62.1%) children. Trans scrotal surgical approach was Tumours and Histiocytosis
performed on 5 children. Three patients with teratoma histology were
prepubertal children. The most frequent location was the right with 37 PO101 / #473 ADRENOCORTICAL CARCINOMA IN
(37;56%) patients. Alpha-fetoprotein was elevated in 49 cases. Inguinal CHILDREN, EXPERIENCE OF A LATIN AMERICAN HOSPITAL
radical orchidectomy was done. The final histological diagnosis was:
yolk sac tumor in 28 (28; 42.4 %) cases, teratoma in 3 (3; 4.5%) cases. Alejandra Casanovas, Carolina Basile, Diego Amaral, Ernesto Veber
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S610 of S635 ABSTRACTS

Hospital General de Niños Pedro Elizalde, Hematología Y Oncología, Ciudad 18 years. The aim of this report is to describe pediatric rare tumors
Autónoma de Buenos AIres, Argentina observed in Armenia.
Methods: Retrospectively the data of 22 patients were retrieved from
Background and Aims: Adrenocortical carcinoma (ACC) is a rare the medical records of patients treated at the Clinic of Chemother-
tumor in children (0.3-0.4%). According to the Argentine Hospital apy of Muratsan Hospital Complex between 2008-2018, and at the
Oncopediatric Registry, between 2000-2019, 45 patients (p) were reg- department of pediatric oncology of Pediatric Cancer and Blood Disor-
istered. Between 2011-2021, in our hospital, 6p were diagnosed with ders Center of Armenia, Hematology Center after R. Yeolyan between
ACC. The aim of this study is to describe the clinical characteristics, 2018-2021.
treatment and evolution of these patients. Results: Pediatric rare tumors were reported in 22 patients of whom
Methods: The clinical data of six children with diagnoses of ACC were 58% were male, the age range was 1-18 years, the median age was
retrospectively analyzed. 12.5 years. We observed sex cord-stromal tumors (8), nasopharyngeal
Results: Six patientes (5 female) with diagnosis of ACC were admit- carcinoma (3), thyroid carcinoma (2), cutaneous melanoma (2), rare sar-
ted betwen january 2011 and december 2021. Median age was 7 coma histotypes (3) (malignant triton tumor, angiosarcoma, giant cell
years (8m-12y). Clinical presentation: Cushing’s syndrome (2p), pub- tumor); rare brain tumors (2) (embryonal tumor with abundant neuropil
arche (2p), virilization (1p), gynecomastia (1p male). Median tumor and true rosettes, atypical teratoid rhabdoid tumor); salivary gland
volume was 298cm3 (24-1900). Hormonal level: increased cortisol carcinoma (1), pheochromocytoma (1). Metastatic disease at diagno-
and androgens (1p), increased androgens (5p). In 4p, tumor complete sis was observed in 7 patients. All the patients received multimodal
resection was performed. Classification: 2p stage I (S), 2p SIII (tumor treatment including surgery and/or chemotherapy and/or chemora-
rupture/loco-regional involvement), 2p SIV (they were not resected). diotherapy based on the histology of the disease. More than 70% of
Stage III/IV patients received chemotherapy (COG ARAR0332), one the cases were discussed with international experts via telemedicine.
without doxorrubicin for cardiac compromise. None received mitotane. Sixty-five percent of patients are alive at the moment of the report.
By immunohistochemistry, 4p had positive p53 staining, confirming Conclusions: As the cohort was retrospective there were distinctly
gen mutation in 2p. Two patients progressed, one SI (increased testos- unreported cases, however, rare pediatric tumors still remain chal-
terone 6 months after diagnosis, PET-CT: pathological remnant adrenal lenging and close international cooperation is emerging. Additionally,
gland) was rescued with surgery; the other was SIII. This patient collaboration with adult oncologists, pediatric and adult surgeons is
relapsed in the lung 20 months after diagnosis and died 15 months later crucial in the management of rare tumors.
without response to second-line treatment. Metastatic patients died
due to progression 3 and 5 months after diagnosis. SI patients are free
of disease 10 years after diagnosis. The other SIII patient is disease-free PO103 / #1769 MALIGNANT EPIDERMOID SALIVARY
50 months after diagnosis. GLAND TUMOR IN A RUSSIAN ADOLESCENT
Conclusions: Despite the small number of patients, given the low
prevalence of ACC in children, this cohort represents 30% of patienets Laura Martín López1 , Marta Osuna Marco1 , Rosa Alonso Gutiérrez2 ,
diagnosed in our country in 10 years. As described in the liter- Marta Villa Alcázar1 , Blanca López-Ibor1
ature, the outcome of pacients with SI desease was good with 1 HM HOSPITALS/CIOCC, Pediatric Hematology And Oncology Unit, Boad-
surgery alone. New strategies of treatment for advanced stages illa del Monte, Madrid, Spain; 2 HM HOSPITALS/CIOCC, Radiation Oncol-
are necessary to improve their survival. Long term follow up is ogy, Móstoles, Spain
mandatory.
Background and Aims: Malignant salivary gland tumors are excep-
tional in children (1 case per billion/16 years old) and the 35-50%
PO102 / #954 PEDIATRIC RARE TUMORS: EXPERIENCE are mucoepidermoid carcinomas (MEC). This case report refers to an
FROM ARMENIA exceptional etiology needing the expertise of a multidisciplinary head
and neck tumors team.
Shushan Hovsepyan1 , Andrea Ferrari2 , Mariam Minasyan1 , Gevorg Methods: A literature review based on a case study.
Tamamyan1 Results: A previously healthy Russian 13-year-old girl, born nearby a
1 Hematology Center after R.Yeolyan, Pediatric Cancer And Blood Disor- city involved in nuclear accident (1,957), presented with a 4-month
ders Center Of Armenia, Yerevan, Armenia; 2 Istituto Nazionale dei Tumori, asymptomatic right submaxillary mass. Ultrasound and craniocervi-
Pediatric Oncology, Milan, Italy cal CT scan showed a right submandibular adenopathy conglomerate
with a cystic lesion suggestive of abcesified/necrotic adenopathy. Fine
Background and Aims: Childhood rare tumors represent a challenge needle aspiration showed no evidence of tumor cells. The diagno-
for pediatric oncologists. Some of these tumors are relatively common sis was reactive lymphadenopathy due to an infectious process. She
in adults, but distinctly rare in childhood and adolescents. According was referred for follow-up to maxillofacial surgeon under antibiotic
to the European Cooperative Study Group for Pediatric Rare Tumors treatment. Surgical resection was performed eight weeks later. Pathol-
(EXPeRT), the incidence is < 2 per million population up to the age of ogy came back as carcinomatous infiltration. Postoperative review of
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ABSTRACTS S611 of S635

preoperative cervical CT scan was reinterpreted as a tumor within the engagement. The active participation of those directly involved in the
salivary gland. She underwent surgical resection of right tumoral sali- disease process has a huge relevance for health education and for the
vary gland with right supra-omohyoid cervical lymph node dissection. emotional support of those involved.
The pathology report described a high-grade MEC with microscopic
glandular capsule and lymphatic-perineural invasion. Within the exten-
sion study, 40 lymph nodes were removed with no evidence of metas- PO105 / #1192 METASTATIC ADENOCARCINOMA OF
tasis (T2N1M0). Four weeks after surgery she received 60 Gy at boost UNKNOWN PRIMARY IN A 14-YEAR-OLD PATIENT:
and extended elective nodal irradiation of 54 Gy in ipsilateral lymph CHALLENGES IN DIAGNOSIS IN A DEVELOPING COUNTRY
node levels I-III and contralateral levels I-II.
Conclusions: Isolated submandibular lymphadenopathy in children is Melisa Najera Castillo, Erik Rodriguez Marrufo, Isidoro Tejocote
usually reactive to infectious processes or dental infection. Persis- Romero, Miriam Matus Román, José Ubias Osorio
tent adenopathy (>6 weeks) and unresponsive to antibiotic treatment Hospital para el Niño de Toluca IMIEM, Pediatric Oncology, Toluca, Mexico
should be biopsied despite a negative FNA or echogenic diagno-
sis of abscess. Once a tumor is identified, an oncology maxillary Background and Aims: Cancer of unknown origin (CUP) is a biopsy-
surgeon should be involved in diagnosis and staging procedure. Expo- proven metastatic lesion without an identifiable primary tumour;
sure to nuclear waste is etiologically related and warrants long-term 70-80% of the histopathology corresponds to metastatic adenocar-
follow-up. cinoma (MACUP), which usually occurs in older adults of 65 years.
In developing countries, such as Mexico, the diagnosis of MACUP in
paediatrics involves several challenges, generating a poor prognostic
PO104 / #915 NEUROFIBROMATOSIS TYPE 1: DESIGN OF trend.
AN EDUCATIONAL GUIDE Methods: Case report
Results: 14-year-old male with vomiting in coffee grounds, melenic
Simone Mozzilli1 , Liliane Dubois1 , Mariana Freitas1 , Verônica evacuations, weight loss, hyporexia and abdominal pain was admit-
Andrade2 ted with Hb 5 g/dl; transfusion and endoscopy were performed to
1 Instituto Beaba, Board, São Paulo, Brazil; 2 Hospital da Criança, Oncology, find chronic diffuse gastritis and helicobacter pylori; returned 21 days
São Paulo, Brazil later due to hematemesis, severe lumbar pain, and paresthesia in his
lower limbs. Tomography showed osteolytic lesions throughout the
Background and Aims: Neurofibromatosis type 1 (NF1) is a genetic spine and the left third costal arch. Beta-hCG, AFP, CA 19-9 and CEA
condition which causes tumors to grow along the nerves. Because it were normal; LDH and ALP were elevated at 1016 IU/L and 796 IU/L,
is a rare condition, there is little information about it, creating stigmas respectively. Then, metastatic Askin’s tumour was suspected, and after
and prejudices. It emotionally impacts patients, families, caregivers and resection of the rib tumour and bone marrow biopsy, chemotherapy
health professionals, as well the society in general. Assisting in treat- with vincristine, doxorubicin and cyclophosphamide was started.
ment does not only mean providing clinical resources, but providing The histopathological study revealed the presence of metastasis
guidance and sharing information to demystify, inform and build new of malignant neoplasm epithelial compatible with adenocarcinoma;
representations. immunohistochemistry was positive for CKAE1/AE3 and CK20. The
Methods: The methods used to create and develop the material were: treatment was redirected towards a protocol for MACUP; however,
Co-creation, Interdisciplinarity, Design Thinking, Agile Methodology due to the lack of drugs such as taxanes or bevacizumab/erlotinib,
and Patient-and-Family-Centered Design. Co-creation was carried a cycle with carboplatin and etoposide was administered, observed
out with all stakeholders: patients, family members, caregivers and a partial improvement; after discharge, the patient and his family
health professionals (doctors, nurses, psychologists, social workers, decided to suspend the treatment. The palliative care service inter-
nutritionists, physiotherapists, among others) using Information and vened, and the patient died at home.
Communication Technology. Conclusions: MACUP, in paediatrics, is a highly aggressive disease, with
Results: The result of the project was the creation of an educational survivals ranging between 5-12 months; its diagnosis faces severe lim-
book illustrated and adapted to the cognitive capacity of patients. The itations in developing countries since there is no access to studies such
material was divided into two parts. The first part brings informative as PET scans, panendoscopy, electron microscopy, cytogenetics, molec-
chapters about the disease: genetic approach, signs and symptoms, ular biology, etc.; therefore, most patients with this type of disease
diagnosis, management of feelings, search for support and tips. The sec- receive chemotherapy for palliative purposes.
ond part has a glossary of 50 terms used in daily life, from consultations
to exams, with the purpose of understanding and empowering patients
and their families. PO106 / #1695 PLEUROPULMONARY BLASTOMA: REPORT
Conclusions: The production of the guide brought an important impact, OF 1 CASE IN A PEDIATRIC PATIENT UNDER 5 YEARS OF AGE
both in bringing qualified knowledge of this disease, which has not been WITH RECURRING RESPIRATORY DIFFICULTY
disseminated in society, as well as for better strategies of coping and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S612 of S635 ABSTRACTS

Omar Reyes Cruzado, Ana Maria Gloria Paredes Guerra Results: During this period, 2746 patients underwent appendectomy.
Hospital Nacional Edgardo Rebagliati Martins, Oncologia Pediatrica, Jesus In 10 cases (0.36%) pathological analysis confirmed a malignant appen-
Maria, Peru dicular tumor, corresponding to a neuroendocrine tumor (NET), 66%
grade 1 (UICC/AJCC), localized disease. Six were female, median age
Background and Aims: ABSTRACT: Pediatric intrathoracic neoplasms at diagnosis 11.75 years (range 8-14 years). All patients presented
are rare. Most cases are usually due to the involvement of mediasti- symptoms of acute abdomen and had histological features of acute
nal structures such as lymphomas, thymomas or metastases from other appendicitis. Staging was done with chest X-Ray, abdominal ultra-
tumors. However, intraparenchymal neoplasms are even rare. Pleu- sound and bone scintigram or dotatate PET scan in selected patients.
ropulmonary blastomas are rare in the United States, with 10 to 20 No patients presented carcinoma syndrome. Appendectomy was the
cases reported per year, which usually appear in the first 5 to 6 years definitive treatment for all patients. Five year disease-free survival was
of life. It is divided into 3 types: Type I purely cystic, Type II which 100%.
is cystic and solid, and Type III which is clearly solid. We present the Conclusions: The incidence of appendicular malignant tumor was sim-
case of a 3-year-old patient who initially presented with bullae from ilar to the reported series. It’s essential to make histological analysis
the 1st year of age, presenting with a large thoracic mass, under- off all specimens, since in children the main clinical presentation is
went a right upper lobectomy, finding a type II-III PleuroPulmonary acute appendicitis. In follow up, all patients had a favorable evolution,
Blastoma, and subsequently received the protocol RMS 2005 with Ifos- without mortality and without known recurrences.
famide, Vincristine, Actinomycin and Doxorubicin, completing 9 cycles,
and later with maintenance with Vinorelbine and Cyclophosphamide.
In addition, a residual mass with activity was apparently found in the PO108 / #693 A CASE OF ANGISARCOMA MIMICKING
PET-CT, but when it was removed, the following was reported: thymic VASCULAR MALFORMATION
gland with fatty infiltration fibrosis and isolated lymph nodes with non-
specific chronic adenitis. There is no evidence of neoplastic disease Gulsah Tanyıldız, Şifa Şahin, Deniz Tuğcu, Mustafa Bilici, Serap
at the moment. When performing a molecular study on the patient, Karaman, Mehmet Babüroğlu, Bilge Bilgiç, Zeynep Karakaş, Ayşegül
the DICER-1 gene was found. Patient currently presents some cystic Ünüvar
images in the left lung of 10mm. Istanbul University Istanbul Medical Faculty, Pediatric Oncology, Beyoğlu,
Methods: The clinical history of the case was reviewed, in addition to Cihangir, Turkey
the pathology images and the molecular study.
Results: Reduction of tumor mass and response to treatment with RMS Background and Aims: A Case of Angisarcoma Mimicking Vascu-
2005 scheme were observed lar Malformation Vascular lesions are the most common congeni-
Conclusions: Pleuropulmonary blastomas are tumors that require mul- tal anomaly of infancy and childhood. Vascular benign tumors are
tidisciplinary management with both chemotherapy and, above all, infantile hemangioma, congenital hemangioma, kaposiform heman-
surgery. This is the first case with survival and in remission in the giothelioma, pyogenic granuloma. Malignant tumors are classified as
Pediatric Oncology unit of the Hospital Nacional Edgardo Rebagliati angiosarcoma and Kaposi’s sarcoma.In rapidly growing and unre-
Martins from Lima Peru sponsive vascular malformations, malignancy should be excluded by
biopsy.
Methods: CASE A 2.5-month-old female patient applied with the com-
PO107 / #1121 INCIDENCE OF PEDIATRIC MALIGNANT plaint of swelling in the neck region. There was a red-purple colored
TUMORS IN APPENDECTOMIES IN A SINGLE INSTITUTION mass on the left submandibular gland.
Results: A heterogeneous lesion as hemangioma was observed on
Camila Solis, Montserrat Rios, Alejandra Perez, Alejandra Delgado, ultrasonography and it was in the form of a 60*53*41 cm lesion compat-
Gloria Gonzalez, Paloma Gomez, Claudia Paris ible with a hemangioma at the left parotid level, mostly located in the
Hospital Luis Calvo Mackenna, Oncologia, santiago, Chile subcutaneous soft tissue and adjacent to the masticator area in MRI.
Propronolol treatment started in dose 2 mg/kg/d. In laboratory tests
Background and Aims: Appendicular neoplasms are rare tumors; PLT:35.000 /ml, D-Dimer:9150 μg/L, Fibrinogen:185 mg/dl. Kasabach-
the majority is incidentally detected in appendectomies performed Merritt Syndrome was considered. Ultrasonography was performed
to treat acute appendicitis. In children most cases correspond to and suggested that hemangioma complicated by intralesional hem-
neuroendocrine neoplasms. The aim of this study is to describe the orrhage. Propronolol treatment was increased to 3 mg/kg/g, prednol
clinical characteristics, pathology, treatment and outcome of these was started.The color of the mass continued to grew aggressively.
patients. Propronolol was discontinued, sirolimus and weekly vincristine treat-
Methods: After IRB approval, a retrospective-descriptive study of ment were started. Significant reduction in the lesion was observed
biopsies results and review of clinical records with history of appen- with weekly vincristine. Biopsy was performed with suspicion of malig-
dectomy in Hospital Luis Calvo Mackenna between 2012 to 2022 was nancy.The pathology result was reported as angiosarcoma. The lesion
performed. was not found suitable for surgical excision because it is close to
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S613 of S635

the main vascular structures. Bevacizumab was administered in every ocardial biopsy of the lesion. Histopathology was suggestive of Cardiac
two weeks as 10 mcg/kg, combined with ifosfamide (1.8 gr/m2) and fibroma. Child is currently thriving well on anti-failure measures and he
adriamycin (20 mg/m2). After 3 cycles patient developed difficulty in is kept under close follow up.
swallowing. In the MRI, the mass had progressed in spite of treatment Conclusions: Rare pediatric cardiac tumors need appropriate imag-
and there was pressure on the airway. Tracheostomy was performed.In ing and biopsy wherever amenable for accurate diagnosis and further
the follow up patient died due to disease progression. management.
Conclusions: Childhood vascular lesions are usually benign and mostly
hemangioma. Therefore, malignant lesions can be overlooked. It should
be kept in mind in cases with atypical course. Publication Only Topic: AS05 SIOP Scientific programme / AS05.m
Brain Tumours

PO109 / #548 A TALE OF TWO PEDIATRIC CARDIAC PO110 / #1624 MOLECULAR GUIDED THERAPY FOR A
TUMORS IN OUR TERTIARY CANCER CARE UNIT PEDIATRIC PLEOMORPHIC XANTHOASTROCYTOMA: A CASE
REPORT
Kalasekhar Vijayasekharan1 , Raghwesh Ranjan1 , Prasanth Vr1 ,
Guruprasad Cs1 , Deepa Sasikumar2 , Arun Gopalakrishnan2 , Beth Armstrong, Morgan Schmitt
Priyakumari Thankomany1 Riley Hospital for Children at IU Health, Hem/onc, Indianapolis, United
1 Regional Cancer Centre, Department Of Pediatric Oncology, Thiruvanan- States of America
thapuram, India; 2 Sree Chitra Tirunal Institute for Medical Sciences and
Technology, Division Of Pediatric Cardiology, Department Of Cardiology, Background and Aims: Pleomorphic Xanthoastrocytoma (PXA) is a
Thiruvananthapuram, India rare type of central nervous system tumor among children. Despite the
fact that they have a high cure rate, PXA’s remain a significant clini-
Background and Aims: Pediatric cardiac tumors are very rare with an cal challenge. Case Study: Patient is a 14-year-old male who presented
incidence of 0.14%. Majority of these primary pediatric cardiac tumors at 11 years of age with a history of migraines, worsening headaches,
are benign, most common being rhabdomyoma and fibroma. Car- nausea, and vomiting. MRI after emergent EVD placement revealed a
diac sarcomas are the most common malignant variant. Management mixed solid and cystic lesion centered in the right temporal lobe and
of these tumors requires multidisciplinary approach with guarded thalamus.
outcomes among malignant cardiac tumors. Methods: Patient underwent two subtotal resections followed by VP
Methods: Retrospective audit of the medical records found two cardiac shunt placement. Complicated pathology findings required consider-
tumor cases managed at our unit in last 3-years. We intend to report ation for standard chemotherapy versus proton radiation therapy. At
the clinical presentation and management of these two rare cases. this time, he was referred to our Precision Genomics Neuro Oncol-
Results: Case1- A 7-year-old male child who developed vomiting, poor ogy program for tumor molecular characterization. While patient was
appetite was found to have a small right ventricular mass(3.2 x1.9cm) recovering, he presented with severe headaches. Imaging revealed
in 2D-ECHO. PET-CT showed low-grade FDG avidity in primary tumor. overshunting and right sided subdural hematoma, evacuation with burr
Over 2 months, there was increase in size of the mass with right ven- holes was performed.
tricular inflow obstruction and elevation of jugular venous pressure. Results: Somatic tumor testing revealed BRAF V600E mutation and
Cardiac-MRI revealed a T1-isointense, T2-hyperintense right ventric- allowed for enrollment in Novartis CDRB436G2201. Patient was
ular lesion(3cmx5.3cmx4.9cm) reaching upto right ventricle outflow assigned to the standard treatment arm and received Carboplatin and
tract with right atrial dilation. Child underwent emergent thoracotomy Vincristine, per CCG 9952A. Disease burden continued to progress
with tumor debulking, tricuspid valve replacement and bidirectional through cycle 7, and per central review patient was transistioned to the
Glenn shunt. Histopathology confirmed Cardiac Clear Cell Sarcoma. Dabrafenib and Trametinib arm of study.
Child received adjuvant Ifosfamide/Doxorubicin chemotherapy regi- Conclusions: Imaging was performed 5 weeks post treatment tran-
men and was tolerating well with good clinico-radiologic response. sition, which revealed interval reduction of dominate tumoral cyst
Unfortunately,at the end of 6thcycle of chemotherapy he succumbed in the right thalamus and heterogenous solid lesion. Disease bur-
to refractory arrythmia secondary to ifosfamide tubulopathy-induced den still continues to decrease with therapy. This case illustrates a
dyselectrolytemia Case2- A 3-month-old male child with forehead clear benefit of using molecular guided therapy in the treatment of
sweating and tachypnea noticed from post-natal day-17 was found PXA’s.
to have large left ventricular mass(4.5x3.9cm) in 2D-ECHO occu-
pying >75% of the cavity. He was instituted on low-dose diuretic.
Cardiac-MRI showed T1-Isointense, T2-heterogenous left ventricular PO111 / #648 A SURPRISE RESPONSE TO TRAMETINIB AND
lesion with patchy hyper-enhancement and central non-enhancement. DABRAFENIB COMBINATION IN A HIGH GRADE GLIOMA
Suspecting a Cardiac Sarcoma, child underwent image-guided endomy-
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S614 of S635 ABSTRACTS

Fatma Betul Cakir1 , Dilvin Çelik Ateş1 , Ganime Çoban2 , Alpay Alkan3 , Results: There were 16 cases, 9 (56.25%) were male and 7 (43.75%)
Ertuğrul Tekçe4 , Namık Öztanır5 female. The mean age at diagnosis was 7 years and 6 months. Regard-
1 Bezmialem Vakif University, Pediatric Hematology And Oncology, Istan- ing the time between the onset of clinical manifestations and diagnosis,
bul, Turkey; 2 Bezmialem Vakif University, Pathology, Istanbul, Turkey; 3 (18.75%) took less than 15 days, 4 (25%) patients were in the range
3 Bezmialem Vakif University, Radiology, Istanbul, Turkey; 4 Bezmialem Vakif between 15 and 30 days, 3 (18.75%) from 30 to 60 days and 6 (37.5%)
University, Radiation Oncology, Istanbul, Turkey; 5 Bezmialem Vakif Univer- more than 60 days. The most common clinical manifestations were:
sity, Neurosurgery, Istanbul, Turkey vomiting (81.25%) and headache (62.5%). As for clinical staging, 12
(75%) patients had localized disease (stage I/II) and 4 (25%) had dis-
Background and Aims: Gliomas are the most common brain tumor in ease with distant metastasis. Regarding treatment, 15 (94%) patients
children, with high-grade gliomas comprising nearly 15% of all pedi- underwent surgical removal of the tumor (total or partial) and of
atric brain tumors. The BRAF V600E mutation results in activation these 13 (87%) received radiotherapy associated with chemotherapy.
of MEK/ERK pathway and detected in 10-15% of high grade gliomas Autologous bone marrow transplantation was performed in 2 (12.5%)
(HGG). We herein report a 14 yrs old girl with BRAF V600E mutated patients. Of the 16 patients studied, 13 (81.35%) patients remained
HGG who responded almost completely to trametinib and dabrafenib alive until the time of the study, and of these 12 had localized disease,
combination. with a median survival of 6 years.
Methods: A 14 yrs old girl was presented with dizziness and syncope to Conclusions: Medulloblastoma is the most common brain tumor in
the emergency room in which an infratentorial mass was detected on children. Our study collaborates with national and international data
magnetic resonance imaging (MRI). The lesion fulfilled left cerebellar that even in developing countries, we can reach satisfactory lev-
hemisphere and reached pons posteriorly and superiorly. The surgery els of survival. Surgical treatment associated with radiotherapy and
was impossible, so the biopsy was obtained which resulted in HGG with chemotherapy may increase the chance of survival of these patients in
BRAF V600E mutation. developing countries.
Results: After biopsy, the patient underwent focal radiation therapy
(54 Gy in 30 fractions) with concurrent temozolomide (75mg/m2/day).
The lesion was stable after 6 weeks of radiation and the patient was PO113 / #368 BRAIN STEM TUMOR IN CHILDREN AND
started on dabrafenib (4.5mg/kg/day) and trametinib (2mg/day) com- ADOLESCENTS – CASE ANALYSIS IN A REFERENCE CENTER IN
bination. After 3 months of combination therapy, the lesion almost PEDIATRIC ONCOLOGY IN THE WEST OF PARANÁ – BRAZIL
disappeared with a significant response with no adverse effect. She is
still under treatment for six months with almost no lesion on MRI. Carmem Maria Costa Fiori, Juliana Maria Saldanha, Aline Rosa
Conclusions: While BRAF inhibitors sufficiently block this pathway, Hospital do Câncer de Cascavel- UOPECCAN, Pediatric Oncology, Cascavel,
they paradoxically activate MAPK patway, thereby development of Brazil
resistance can limit their effectivity. Dabrafenib and trametinib combi-
nation is a dual therapy in order to overcome resistance to single BRAF Background and Aims: Brainstem tumors represent about 10% to
inhibitors, in which both BRAF and MEK inhibition are targeted. 20% of central nervous system tumors in children. They are considered
tumors with a poor prognosis, regardless of the pathological charac-
teristics. The treatment is based on the isolated or associated use of
PO112 / #363 MEDULOBLASTOMA: A CASE ANALYSIS IN A surgery, chemotherapy and radiotherapy, among which radiotherapy
REFERENCE CENTER IN PEDIATRIC ONCOLOGY IN WESTERN seems to be the only therapeutic option in an attempt to improve
PARANÁ- BRAZIL patient survival. Objective: to describe the clinical presentation, thera-
peutic approaches, evolution and survival of children and adolescents
Carmem Maria Costa Fiori, Marina Silva, Aline Rosa diagnosed with brainstem tumor in an institution in western Paraná,
Hospital do Câncer de Cascavel- UOPECCAN, Pediatric Oncology, Cascavel, Brazil.
Brazil Methods: Descriptive cross-sectional cohort study. The analysis was
performed on patients under 19 years of age, diagnosed with a brain-
Background and Aims: Introduction: Medulloblastoma is the most stem tumor diagnosed at Hospital do Câncer de Cascavel, UOPECCAN,
common malignant brain tumor in childhood. Objective: To analyze the from January 2000 to February 2022.
characteristics and evolution of patients diagnosed with medulloblas- Results: 12 patients, aged 17 to 182 months, median of 81 months
toma at a referral center for pediatric oncology in western Paraná, (6.7 years). 9 (75%) were male and 3 (25%) were female. Gliomas were
Brazil. the most frequent imaging findings. Changes in gait and visual changes
Methods: Retrospective observational study with analysis of medical were the most frequent complaints in 8 (66.6%) and 7 (58.3%) patients,
records of pediatric cancer patients at Hospital do Câncer de Cascavel respectively. Neurological complaints (plegia/paresis) and headache
Uopeccan, from January 2006 to January 2020. Data regarding gender, and vomiting were less frequently present. 10 patients (83.3%) were
age, clinical manifestations, staging and evolution were evaluated. treated with chemotherapy and radiotherapy. 8 (66.6%) died and
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S615 of S635

4 (33.3%) are still undergoing treatment. Survival time ranged from 2 Conclusions: Patient with BRAF-V600E mutated gliosarcoma may
to 27 months, with a median of 12 months. have prolonged survival after repeated safe resection, radiotherapy
Conclusions: Brain stem tumor is still a challenging disease with a poor and the use of temozolomide followed by dual BRAF / MEK inhibitors.
prognosis. Disseminating and warning about the main signs and symp-
toms of this disease in children and adolescents may contribute to early
diagnosis in this population. We hope that, in the near future, we will PO115 / #522 SUCCESSFUL SALVAGE OF RELAPSED
be able to have surgical techniques and/or new therapeutic approaches INTRACRANIAL NON-GERMINAOTEOUS GERM CELL TUMORS
that can help increase the survival of these patients. NGGCTS IN A CHILD WITH RENAL INSUFFICIENCY WITH
NOVEL PLATINUM-FREE CHEMOTHERAPY REGIMEN

PO114 / #1202 PROLONGED SURVIVAL OF A PATIENT Nahla Mobark1 , Walid Ballourah1 , Abdulrahman Alsultan1 , Fahad Al
WITH BRAF-V600E MUTATED PAEDIATRIC GLIOSARCOMA Manjomi1 , Fahad Alotabi2 , Ali Abdullah O. Balbaid3 , Mohammed
TREATED BY REPEATED SAFE SURGICAL RESECTION, FOCAL Rayis1 , Zaid G. Alnaqib1 , Wael Abdel Rahman Aljabarat1 , Jonathan
IRRADIATION, TEMOZOLOMIDE AND DUAL BRAF / MEK Finlay4 , Musa Alharbi1
INHIBITORS 1 King Fahad Medical City, Comprehensive Cancer Centre, Department Of
Pediatric Oncology And Bone Marrow Transplant, Riyadh, Saudi Arabia;
Dennis T. L. Ku1 , Anthony P.Y. Liu1,2 , Carol Lai Sim Yan1 , Eric Chun Ho 2 King Fahad Medical City, Pediatric Neurosurgical Department, Riyadh,
Fu1 , Jeffrey Ping Wa Yau1 , Matthew Ming Kong Shing1 , Chung Wing Saudi Arabia; 3 King Fahad Medical City, Comprehensive Cancer Cen-
Luk1 , Godfrey Chi-Fung Chan2 tre, Radiation Oncology Department, Riyadh, Saudi Arabia; 4 OSU- Ohio
1 Hong Kong Children’s Hospital, Paediatrics, Hong Kong, Hong Kong PRC; State University, Emeritus Professor Pediatrics And Radiation Oncology,
2 LKS Faculty of Medicine, The University of Hong Kong, Paediatrics, Hong Columbus, United States of America
Kong, Hong Kong PRC
Background and Aims: The Outcome for relapsed NGGCT is
Background and Aims: Gliosarcoma (GS) is a rare variant of glioblas- poor. Salvage therapy usually consist of reinduction platinum-based
toma with mixed glial and sarcomatous components exhibiting mes- chemotherapy regimen followed by high-dose-chemotherapy and
enchymal features. Prognosis remains poor with median survival < 1 autologous-stem-cell-rescue (HDC/AuSCR) and re-irradiation with
year despite surgical resection, radiotherapy with adjuvant temozolo- no consensus on optimal management and usually associated with
mide. Advance in molecular studies in GS has not reported survival remarkable toxicity.
benefit from using novel agents. We present an exceptional patient Methods: We present a 12-year-old boy diagnosed with a localized
with GS having prolonged survival of 54 months from initial diagnosis. pineal Non-Germinaoteous Germ Cell Tumors NGGCTs of mixed origin
Methods: Retrospective review of treatment journey of this special with elevated AFP he had ETV and biopsy started on COG ACNS0122
patient including repeated surgery, radiotherapy and chemotherapeu- protocol after receiving first cycle (carbo/Etoposide) he developed
tic agents. acute renal failure investigation showed small dysplastic kidney to
Results: A twelve-year-old boy presented with headache and found avoid nephrotoxicity of platinum agents chemotherapy changed to VBE
left occipital mass with subdural haemorrhage. Gross total resec- (Vinblastine, Bleomycin and Etoposide) post 3rd cycle MRI showed
tion was achieved at diagnosis. Histopathology showed gliosarcoma increase in size of the pineal mass with normal tumor markers repre-
with IHC found BRAF-V600E positive, IDH-wt and H3K27M negative. senting Growing Teratoma Syndrome He had total surgical resection
MGMT methylation was negative by PCR. He received focal irradiation of the tumor Pathology showed predominant teratoma component
at 59.4Gy with adjuvant temozolomide. As there was no measuable He received radiation therapy CSI then another 3 cycles of VBE 4
residual on serial MRI, he completed 6 cycles of pulse temozolomide months following treatment completion he presented with elevated
and stopped. MRI 8-months after completion of treatment showed AFB and new right anterior temporal lesion Spinal MRI and CSF were
recurrence at left cerebellopontine (CP) angle, also C1/2 and T9/10. negative. He had 2 cycles of Salvage Non-nephrotoxic 4-drug regi-
Palliative radiotherapy was given to C1/2 lesion at 24Gy followed by mens GEMPIV Gemcitabine 800 mg/m2 days 1 and 14. Paclitaxel 80
compassionate use of dual BRAF / MEK inhibitors (Trametinib and mg/m2 days 1 and14, Irinotecan 50 mg/m2 daily for 5 days Vinblas-
Dabrafenib). The right CP angle lesion had slow interval growth while tine 6 mg/m2 weekly days 1,8,14 MRI after 2 cycles showed remission
spinal lesions were static. Gross total resections for right CP angle with undetectable AFP then 2 consolidation cycles of etoposide and
lesion were performed at 6 months and 18 months since dual ther- thiotepa (HDC/AuSCR) The 3rd consolidation cycles were cancelled
apy. He had Grade 0 ECOG performance enjoying normal school and due to hematological toxicity
exercise. Medication was well tolerated with Grade 1/2 skin reac- Results: During treatment phases chemotherapy was well toler-
tion only. Unfortunately, he had leptomeningeal progression again with ated doses were adjusted according to his GFR with renal con-
haemorrahage from right CP angle at 32 months after dual therapy, 54 servative and supportive therapy. post (HDC/AuSCR) he experi-
months from initial diagnosis. Surgery was performed for resection and enced delayed hematological recovery with persistent thrombocy-
haemostasis. Repeated molecular study is in progress. topenia responded to Eltrombopag then he had focal Temporal lobe
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S616 of S635 ABSTRACTS

irradiation Currently patients in remission with chronic stage 3 renal life and well tolerating the therapy. Our case show that personalized
Insufficiency treatment approach that address molecular heterogeneity of H3K27M
Conclusions: this case showed that relapsed intracranial NGGCT can glioma are safe and feasible.
be successfully salvaged without platinum-based chemotherapy in
patients with renal insufficiency.
PO117 / #846 LATE RELAPSE OF A METASTATIC ATYPICAL
TERATOID RHABDOID TUMOR (AT/RT) FOLLOWING
PO116 / #70 PERSONALIZED TREATMENT FOR ENTEROVIRUS MENINGOENCEPHALITIS. COULD IMMUNE
MOLECULARLY HETEROGENEOUS DIFFUSE MIDLINE GLIOMA, RESPONSE ACT AS A TRIGGER OF TUMOR RECURRENCE?
H3 K27-ALTERED PAEDIATRIC CASE
Palma Solano-Páez1 , Javier Marquez-Rivas2 , Mónica Rivero-Garvia2 ,
Nahla Mobark1 , Musa Alharbi1 , Yasser S. Bayoumi2 , Ayman Al Patricia Cabrera-Roldán3 , Eloy Rivas4 , Elena Merchante5 , Eduardo
Banyan3 , Zaid G. Alnaqib1 , Wael Abdel Rahman Aljabarat1 , Ali Quiroga-Cantero1
Abdullah O. Balbaid2 , Malak Abedalthagafi, Md4 1 HOSPITAL INFANTIL VIRGEN DEL ROCIO, Pediatric Oncology, SEVILLE,
1 King Fahad Medical City, Comprehensive Cancer Centre, Department Of Spain; 2 Hospital Infantil Virgen del Rocio, Pediatric Neurosurgery, SEVILLE,
Pediatric Oncology And Bone Marrow Transplant, Riyadh, Saudi Arabia; Spain; 3 Hospital Infantil Virgen del Rocio, Radiation Therapy, SEVILLE,
2 King Fahad Medical City, Comprehensive Cancer Centre, Radiation Oncol- Spain; 4 Hospital Virgen del Rocío, Neuro Pathology, SEVILLE, Spain;
ogy Department, Riyadh, Saudi Arabia; 3 King Fahad Medical City, Pediatric 5 Hospital Infantil Virgen del Rocio, Radiology, SEVILLE, Spain

Neurosurgical Department, Riyadh, Saudi Arabia; 4 King Fahad Medical City


and King Abdulaziz City for Science and Technology„ Genomics Research Background and Aims: AT/RT is a highly lethal embryonal CNS
Department, Saudi Human Genome Project, Riyadh, Saudi Arabia tumor affecting early childhood. Despite intensive chemotherapy and
radiation regimens, prognosis remains poor. AT/RT relapses typically
Background and Aims: Diffuse midline glioma, H3 k27-altered (DMG) occur within the first two years after diagnosis and late relapses are
is a type of Paediatric- type diffuse high grade gliomas according to anecdotical.
the 2021 WHO CNS tumors Classification. Diffuse intrinsic pontine Methods: Case report of a late AT/RT relapse. Review of host immune
glioma (DIPG) is another acceptable related term when it located in response and viral mechanisms that could have contributed to tumor
the pons with fatal prognosis. The combination of H3K27M with BRAF recurrence following severe CNS viral infection.
V600 mutations rarely reported in DMG although more commonly in Results: 4-year-old boy diagnosed with a supratentorial metastatic
Paediatric-type low grade gliomas (Diffuse low-grade glioma, MAPK ATRT who achieved complete remission following gross total resec-
pathway-altered). tion, intensive chemotherapy and craniospinal radiation. Surveillance
Methods: We present a twenty-month-old boy, previously healthy, pre- MRI remained clear until 3 years and 8 months following initial tumor
sented with 2 weeks history of unsteady gait, drooling, cranial nerves diagnosis, when the patient was admitted to Intensive Care due to an
palsy MRI imaging showed diffuse pontine mass with classic radiologi- acute episode of decreased level of consciousness, tonic seizures, hypo-
cal features of DIPG. 2.6 x 1.6 x 3.2 (AP x TV x CC) with no evidence for tonia, fever and severe dehydration. CSF and stool PCR were positive
spinal metastases. Patient underwent right retro sigmoid approach and for Enterovirus. The patient was successfully managed with support-
open biopsy of lesion he received focal Radiation Therapy 54GY/30fx ive care, iv Ig and steroids. MRI at ICU discharge did not show any sign
as stander of care of DIPG with mild neurogical improvement Patholog- of tumor recurrence. Three months following encephalitis diagnosis,
ical & molecular Diagnosis was Diffuse midline glioma, H3 k27-altered and 4 years from initial tumor diagnosis, the patient presented with
with Co-occurrence BRAF V600E mutation.Two months after end of aphasia, slow speech, headache and retrograde amnesia. Craniospinal
radiation, he presented with vomiting, and neurological deteriora- MRI demonstrated leptomeningeal contrast enhancement with mul-
tion with new right-side hemiplegia. Imaging studies showed interval tiple nodular lesions suggestive of tumor dissemination. The patient
increase in the pontine lesion with increased edema causing narrowing suffered rapid clinical deterioration and was managed palliatively.
of the fourth ventricle, no active hydrocephalus. Necropsy findings confirmed diffuse infiltration of AT/RT with large
Results: He was started on combination therapy BRAF inhibitor areas of affected brain parenchyma. Multiplex PCR in brain tissue
Dabrafenib and MEK Inhibitor Trametinib as maintenance therapy the necropsy was negative for neurotropic virus including Enterovirus.
patient gradually showed Marked neurological and clinical improve- Conclusions: AT/RT is a rare brain tumor, and many aspects of the
ment. A 6-month MRI after start of targeted therapy showed favorable mechanism of its tumorigenesis remains unknown. To our best knowl-
treatment response with complete resolution of the previously dif- edge, the hypothesis of a relationship between viral infection and
fusion restriction, reduced tumor flow and volume on MR perfusion AT/RT relapse has not been previously considered. We suggest Immune
reduced perilesional edema otherwise almost stabilization of nonen- mechanisms following RNA-Viral infection of the CNS as enterovirus
hancing pontine lesion. could represent a contributor or enhancer of relapse. The role of
Conclusions: poor prognosis of recurrent DIPG is known but our new agents and immunotherapy in AT/RT therapy warrants further
patient is clinically and radiologically stable with excellent quality of research.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S617 of S635

PO118 / #1437 INTERNATIONAL TWINNING PROGRAM IN surgery and the start of treatment was reduced from 2 months
PEDIATRIC NEURO-ONCOLOGY BETWEEN SAN JUAN DE DIOS to 2 weeks for chemotherapy and 4 months to 5 weeks for
HOSPITAL (BARCELONA- SPAIN) AND THE MINISTRY OF radiotherapy.
HEALTH OF ECUADOR Conclusions: A twinning program with online MTB meetings repre-
sents a good tool for dealing with complex cases. Better communication
Viviana Tenezaca1 , Doris Calle Jara2 , Andres Morales3 , Ofelia Cruz3 , between the multidisciplinary team leading to shortening treatment
Vicente Santa-María3 , Marta Perez Somarriba3 , Guillermo Chantada3 , times benefits patients’ outcome. In some cases, limitations in the avail-
Grisel Lafita2 , Victoria Ronquillo4 , Juan Guzman5 , Jinna Aucancela6 , ability of laboratory diagnostic procedures and treatment modalities
Elizabeth Gamarra7 , Soledad Jimenez8 , Juan Ramirez9 , Aliz Borja10 , affected the feasibility of some recommendations.
Yarennys Herrera11 , Julio Guillem12 , Eduardo Montenegro13 , Carlos
Valle14 , Galo Izquierdo14 , Johana Esteves15 , Indira Alejando1 , Katty
Sampedro1 , Mercedes Chimbo1 , Hipólito Escobar1 , Ivonne Quimi1 , Publication Only Topic: AS05 SIOP Scientific programme / AS05.n New
Carolina Vergara1 Drugs/Experimental Therapeutics
1 Hospital del Niño Dr. Francisco Icaza Bustamante, Pediatric, Guayaquil,
Ecuador; 2 Hospital del Niño Dr. Francisco Icaza Bustamante, Pediatric PO119 / #285 A PRACTICAL GUIDE FOR USING PRECISION
Oncology, Guayaquil, Ecuador; 3 Hospital Sant Joan de Deu, Pediatric ONCOLOGY IN LOCAL ONCOLOGY PRACTICES
Oncology, Barcelona, Spain; 4 Hospital Roberto Gilbert Elizalde, Pediatric
Neurosurgery, Guayaquil, Ecuador; 5 Hospital del Niño Dr. Francisco Icaza Mark Marshall1 , Amy Helvie1 , Jennifer Ivanovich2 , Lisa Langsford1 ,
Bustamante, Pediatric Neurosurgery, Guayaquil, Ecuador; 6 Hospital del Michael Ferguson1
Niño Dr. Francisco Icaza Bustamante, Pathologist, Guayaquil, Ecuador; 1 Indiana University School of Medicine, Pediatrics, Indianapolis, United
7 Instituto Nacional del Cancer, Radioterapeutic Oncology, Guayaquil, States of America; 2 Indiana University School of Medicine, Medical And
Ecuador; 8 SOLCA, Pediatric Oncology, Loja, Ecuador; 9 Instituto Nacional Molecular Genetics, Indianapolis, United States of America
del Cancer, Pediatric Oncology, Guayaquil, Ecuador; 10 Hospital AXXIS,
Pediatric Oncology, Quito, Ecuador; 11 Hospital Infantil Baca Ortiz, Pedi- Background and Aims: Clinical advances have begun to introduce the
atric Oncology, Quito, Ecuador; 12 SOLCA Portoviejo, Pediatric Oncology, use of genomic biomarkers into pediatric oncology to help identify
Portoviejo, Ecuador; 13 CERID, Radiologist, Guayaquil, Ecuador; 14 Hospital effective targeted therapies. Unfortunately, clinical oncology sequenc-
Alcívar, Radiologist, Guayaquil, Ecuador; 15 Hospital del Niño Dr. Francisco ing companies focus almost exclusively on detecting genomic alter-
Icaza Bustamante, Pediatric Intensive Care, Guayaquil, Ecuador ations associated with adult cancers. Excellent for diagnosing and
suggesting treatments for adult patients, the bioinformatic reports
Background and Aims: Background: Although there is no national reg- provided by most companies provide questionable value for most pedi-
istry of pediatric cancer in Ecuador, according to our SOLCA Guayaquil atric cancer patients. The goal of the Riley Precision Genomics Program
(Private Cancer Center) population cancer registry 24% of CNS tumors is to work with clinical sequencing companies to provide a genomics
in Ecuador correspond to children under 15 years. After an agreement report that can provide increased value to pediatric cancer patients.
signed on September 2020 between the Hospital San Juan de Dios in Methods: CLIA compliant genomic sequencing companies have been
Barcelona-Spain (HSJD) and the Ministry of Public Health of Ecuador approached to modify their reports to provide a pediatric focus on
(MSP), a twinning program in pediatric neuro-oncology was started gene variant detection, drug-gene associations, and clinical trial iden-
with the Public Children’s Hospital “Dr. Francisco de Icaza Bustamante” tification. Furthermore, publicly available clinical genomic databases
(HFIB) in Guayaquil. The main goal is to stablish a multidisciplinary were utilized in-house to identify additional targeted drug options for
tumor board (MTB) capable to make diagnostic and therapeutic rec- patients which were not provided in the commercial reports. A multi-
ommendations for pediatric patients with CNS tumors. Our aim is to disciplinary Molecular Tumor Board discussed external and internal
evaluate the feasibility of recommendations rendered by the Board. findings to recommend a genomically optimized treatment plan.
Methods: Method: Retrospective study of the online recommenda- Results: Riley has evaluated numerous clinical genomic sequencing
tions from July 2021 to March 2022. Patients under 15 years, with companies. Caris Life Sciences, Foundation Medicine and Tempus cur-
newly diagnosed and relapsed CNS tumors were considered for discus- rently offer the best value to pediatric oncology. The Riley protocol also
sion. All patients’ identification data were anonymized. takes the gene variant and expression data provide by each sequenc-
Results: Twenty-four patients were discussed, (23 HFIB and 1 Solca ing provider and compares it to a number of clinically validated cancer
Loja Hospital). Astrocytoma account of 37.5% of cases. There was genome databases to identify 1) hereditary predisposition variants,
a male predominance (70.8%). The mortality was 8.3%. The rec- 2) improved diagnosis and molecular-subtyping of the tumor, 3) FDA
ommendations were followed-up in 83% of the cases, the rest drugs approved for molecular targets in children and closely related
were not achieved due to lack of molecular testing laboratories. In adult indications and 4) appropriate pediatric clinical trial opportuni-
30% of the cases, the recommendations led to a major change in ties. Detailed information, tables, and database links from the Riley
management. Given multidisciplinary encounters, time lag between clinical experience will be provided.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S618 of S635 ABSTRACTS

Conclusions: The Riley protocol enables oncologists to effectively PO121 / #1785 PRACTICAL CONSIDERATIONS FOR
implement Pediatric Precision Oncology using commercial genomic CLINICAL TRIALS EVALUATING AUTOLOGOUS TCR T-CELL
sequencing companies and modest in-house analysis. Using this proto- THERAPIES TARGETING CANCER TESTIS ANTIGENS TO TREAT
col, relapsed and high-risk patients with validated genomic biomarkers PEDIATRIC SOLID TUMOURS
frequently experience improved survival on targeted therapy over
second-line chemotherapy. Axel Le Cesne1 , Veronique Minard-Colin2 , Tara O’Donohue3 , Vanessa
Fabrizio4 , Erica Elefant5 , Suzanne Kamps5 , Swethajit Biswas6 , Colin
Lunt6 , Erin Van Winkel5 , Dennis Williams5
PO120 / #286 USING PEDIATRIC PRECISION GENOMICS TO 1 Institut Gustave Roussy, Gustave Roussy Cancer Center-ditep, Ville-
IMPROVE THE DIAGNOSIS OF DIFFICULT TUMORS juif, France; 2 Gustave Roussy, Université Paris-saclay, Villejuif, France;
3 Memorial Sloan Kettering Cancer Center, Pediatric Oncology, New York,
Mark Marshall1 , Michael Ferguson1 , Amy Helvie1 , Jennifer United States of America; 4 University of Colorado School of Medicine,
Ivanovich2 , Lisa Langsford1 Pediatric Oncology, Aurora, United States of America; 5 Adaptimmune, Clin-
1 Indiana University School of Medicine, Pediatrics, Indianapolis, ical Development, Philadelphia, United States of America; 6 Adaptimmune,
United States of America; 2 Indiana University School of Medicine, Clinical Development, Abingdon, Oxfordshire, United Kingdom
Medical And Molecular Genetics, Indianapolis, United States of
America Background and Aims: T-cell receptor (TCR) T-cell therapies targeting
cancer testis antigens (CTAs) in human leukocyte antigen (HLA)-
Background and Aims: Pathology plays a key role in the diagno- eligible patients have shown encouraging results in adult metastatic
sis and staging of cancer. Occasionally, some tumors evade accurate solid tumours. Unlike chimeric antigen receptor T-cell therapies
identification, potentially leading to inaccurate outcome predictions that recognizes tumour cell surface markers, TCR T-cell therapies
and less effective treatment selections. With the advent of precision target both intra- and extra-cellular antigens when presented by
genomics, multi-platform analysis can now provide for many pediatric HLA molecules, and therefore may have broader therapeutic util-
tumors a molecular diagnosis based upon DNA and RNA sequencing, ity. This provides an opportunity to address unmet medical need in
methylation analysis and protein expression. We provide four exam- multiple paediatric relapsed/refractory solid tumours. Here we out-
ples of diagnostically challenging pediatric cancer patients. Application line unique considerations when designing a paediatric cell therapy
of deep multi-platform analysis provided revised diagnoses for each study.
patient. Methods: Clinical trials of TCR T-cell therapies should be directed at
Methods: Four pediatric cancer patients with difficult diagnoses were tumour types common to paediatrics and expressing adequate levels
referred to the RIley Precision Oncology program. DNA and RNA of CTA proteins for which screening is needed. Safe and effective doses
samples were obtained from FFPE preserved tumor samples and can be extrapolated from adult studies but lower apheresis volumes
sequenced using NexGen techniques in CLIA-certified laboratories. for cell manufacture may be required. Children should be treated at
Characteristic DNA variants and mRNA gene expression patterns were centres with clinical experience in paediatric apheresis, autologous cell
used to provide a molecular diagnosis. administration, and management of cytokine release syndrome and
Results: Patient 1). RNA-seq of a putative metastatic sacral primitive immune effector cell-associated neurotoxicity syndrome. Additional
neuroectodermal tumor revealed a SSX1-SS18 gene fusion identifying safety mitigations include staggered dosing in Phase 1 trials, mini-
the tumor as a synovial sarcoma. Patient 2). A putative neuroblastoma mizing cross-sectional imaging radiation/anaesthesia exposure, only
was revised to a germ cell tumor due to a lack of NBL gene markers evaluating clinically indicated biopsies, and ensuring age-appropriate
and the presence of genomic biomarkers typical of a germ cell tumor. blood-volume collection for clinical and translational investigations.
Patient 3). Molecular characterization of a tumor with mixed char- Results: Implementation of the described methods facilitates trial exe-
acteristics of both a soft tissue sarcoma and an acute lymphoblastic cution. Clearly outlining the rationale and risk mitigation strategies for
leukemia revealed mutations in the PTEN, CDKN2A/B, KDM6A, LEF1 paediatric populations leads to timely trial approval by institutional
and FAS genes, characteristic of T-cell acute lymphoblastic leukemia. review boards and regulatory authorities. Consideration of children’s
Patient 4). A patient diagnosed with giant cell tumor was found to lack unique needs promotes patient/family/site-staff adherence and col-
the H3F3A G34W mutation typical of GCT of the bone, but did have laboration facilitating enhanced clinical trial oversight, patient safety
the FGFR1 N361I mutation characteristic of giant cell lesion of the monitoring, and quality data generation to support development of
jaw. novel TCR T-cell therapies for the paediatric population. Employing
Conclusions: Integration of a precision genomics program with these concepts, a TCR T-cell therapy trial has been expanded to include
pathology provides access to deep, multi-platform tumor analysis, children ≥10 years old (NCT04044768).
bioinformatic expertise and detailed molecular interpreta- Conclusions: Paediatric TCR T-cell therapy trials are complex, and
tions by a diverse molecular tumor board which can enhance unique aspects outlined should be considered for implementation to
diagnosis. safely evaluate these promising therapies.
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ABSTRACTS S619 of S635

PO122 / #1213 COMPARISON OF CEFTAZIDIME AND Background and Aims: ChromaDose is an NIHR-funded research
CIPROFLOXACIN PROPHYLAXIS IN PEDIATRIC HSCT PATIENTS project led by University College London. We are developing a bedside
blood-monitoring supportive technology to help personalise dosing
Bahador Mirrahimi1 , Shahin Shamsian2 , Shayan Mastoor Tehrani1 , of chemotherapies. This builds on recent evidence that dose adjust-
Kebria Moradi1 ments guided by therapeutic drug monitoring can improve health
1 Shahid Beheshti University of Medical sciences, Clinical Pharmacy, Tehran, outcomes for children with cancer. For more information, please visit
Iran; 2 Shahid Beheshti University of Medical Sciences, Pediatrics, Tehran, www.chromadose.org. We are in the early stages of development and
Iran recognise the importance of gathering the inputs of technology end-
users in establishing designing requirements. The survey’s primary
Background and Aims: Infections were identified as a significant con- objectives
tributor to mortality and morbidity during the pancytopenia period and 1. Establish the clinical pathways for technology adoption
they are considered preventable. Finding an effective systemic antibac- 2. Elucidate regulatory, clinical, and technical barriers to technology
terial prophylaxis can reduce the risk of these infections. This study was adoption
aimed to evaluate the role of ciprofloxacin as prophylaxis in pediatrics. Secondary Objective
Methods: This study was performed as an open-label clinical trial on 1. Establish user preferences of a point-of-care therapeutic drug
70 children admitted to the hematopoietic stem cell transplant ward. monitoring devic
Patients were randomly divided into two groups of 35 patients using Methods: This study is considered as research administering a ques-
software generated block randomization list. The intervention group tionnaire for mixed methodology analysis. Surveys were conducted
received Ciprofloxacin at a dose of 10 mg/kg twice daily and the con- using an online survey platform (Microsoft Forms), comprising a mix-
trol group received Ceftazidime at a dose of 50 mg/kg 3 times daily. ture of closed and open questions. Questions took the form of multiple
Other interventions were the same between the two groups. Demo- choice, Likert scale and free text to capture a variety of responses
graphic data, laboratory data, and the number of fever-free days were and data types. The questionnaire was heavily branched according
recorded for both groups. to the participant response and profession. The survey was exclusive
Results: There was no significant difference in the outcome and days answered by nurses, clinicians, pharmacists, and medical scientific lab
of hospitalization in the control and intervention groups. The mean officers working within paediatric oncology. Identification and recruit-
number of fever-free days in the control group was 4.71± 4.19 days, ment were achieved through 3rd party clinical networks, professional
which increased to 7.23 ± 4.40 days in the intervention group, which is bodies, social media, and other non-NHS channels associated with
a significant increase. RBC, PLT, SrCr, and ANC were not significantly project ChromaDose.
different between the control and intervention groups. The number of Results: The deadline for survey completion is August 31st, therefore
WBCs in the intervention group was significantly less than in the con- results will be fully analysed at the time study completion.
trol group with a P <0.0001. 27 cases (77.1%) of patients receiving Conclusions: These findings will deliver significant benefits to the
ceftazidime and 9 cases (25.7%) of patients receiving ciprofloxacin had paediatric oncology clinical environment, including:
positive culture during this study. • An insight into wider healthcare professional sentiments for thera-
Conclusions: The results of this study indicate that the number of peutic drug monitoring practices within respective patient groups.
fever-free days was higher in the ciprofloxacin group. Regarding • Direct insight for technology development that reduces participant
laboratory data, only the number of white blood cells was signifi- burden and improve the efficiency of their service by tailoring solutions
cantly different between the two groups, which was lower in the specifically to their needs.
ciprofloxacin group. Also, Prophylaxis with ciprofloxacin was more • Alleviation of current bottlenecks and resource constraints in day-to-
effective than ceftazidime in preventing infection with gram-positive day practices of respondents.
and gram-negative bacteria.

PO124 / #483 TARGETED THERAPY FOR MAPK PATHWAY


PO123 / #1860 PAEDIATRIC ONCOLOGY HEALTHCARE ALTERATIONS IN PEDIATRIC TUMORS
PROFESSIONAL ENGAGEMENT TO INFORM THE DESIGN OF
BEDSIDE DRUG MONITORING TOOL: RESULTS FROM A UK Marcelo Urbieta1 , Maria Cores2 , Mercedes Garcia Lombardi2
-WIDE SURVEY 1 Hospital de Niños Ricardo Gutiérrez, Oncología, Ciudad Autónoma de
Buenos Aires, Argentina; 2 25269884, Oncología, Ciudad Autónoma de
Jugal Suthar1 , Alaric Taylor1 , Philip Berry1 , Gareth Veal2 , Stefan Buenos Aires, Argentina
Guldin1
1 University College London, Department Of Chemical Engineering, London, Background and Aims: MAPK pathway mutations are described in
United Kingdom; 2 Newcastle University, Newcastle University Centre For low grade gliomas (LLG), Plexiform Neurofibromas (PN), histiocytic dis-
Cancer, Newcastle upon Tyne, United Kingdom orders (HD), glioblastomas and melanomas among others in pediatric
patients (p)
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S620 of S635 ABSTRACTS

Methods: To report a single Pediatric Oncology Unit experience in the (range,0.9-16), HM=48, ST=41. Full workup followed approved writ-
use of MAPK inhibitors (MAPKI) (vemurafenib, trametinib single or ten informed consent.
associated with dabrafenib) in patients with PN, LGG and HD Results: Twenty-six patients(12/26boys) showed cardiotoxicity. Acute
Results: Nine patients, 4 with PN, type 1 Neurofibromatosis (all local- cardiotoxicity occurred in 2 patients (ΗΜ=2) with decreased ejec-
ized were head and neck), 2 with diagnosis of HD (1 Langerhans Cell tion fraction (EF <55%) along with bradycardia and abnormal ECG.
Histiocytosis (LCH), 1 Histiocytic Sarcoma (HS), BRAF V600 +, 3p Subacute form of cardiotoxicity occurred in 24/26 patients. Two
with LGG (BRAF V600+, 1 optical pathway glioma, 1 thalamic glioma) patients with HM experienced chest pain with elevated troponin. One
The median age at the start of treatment was 9 yrs. old. Three were intermediate-risk AML patient developed abnormal ventricular con-
woman Four patients receive vemurafenib, 5 trametinib as single agent, tractions and was treated with propranolol. Seven patients showed
1 p trametinib plus dabrafenib after progression after using vemu- increased troponin (median 82,45 pg/ml, range 37-98,05) and ECG dis-
rafenib, 1 reinstated trametinib after reactivation. Median of previous turbances (QTc prolongation, flattened T waves). Pericardial-effusion
treatment lines: 2 (1-6). Median time of treatment 11 months (range 3- and QTc-prolongation presented in two patents with HM, respectively.
24). Response: In 9/10 treatment achieved clinical response. complete Three patients showed interventricular septum motility, one of them
remission 3p (LCH, LGG), partial remission, 4p, stable disease 1p (LGG), with increased troponin. Nine patients(HM=4, ST=5) showed reduced
progression,2 p. In 2p with PN underwent to surgery wiht partial resec- ejection fraction and increased troponin value (median 30,57 pg/ml,
tion and improve the life quality. Toxicities: dermatological Grade1-2 range 21,08-67,97). All patients received anthracyclines in a cumula-
(3p) CPK increased with normal troponin The patient with HS died due tive dose <360mg/m2 and no-one was irradiated. All patients showed
to disease progression gradual recovery of cardiotoxicity, over a period of 1-2 months after the
Conclusions: MAPKI were useful drugs in patients with pathologies event. Eight patients received ACE inhibitor or b-blocker. All patients
with molecularly diagnosed or known alterations in the MAPK path- continued full-dose chemotherapy (2/13 with a minor delay) and two
way, in the event of failure of standard treatment lines. Toxicity was patients reduced by 1/3.
aceptable. Defining the duration of treatment is a future challenge, as Conclusions: The early results of our ongoing study, show that mod-
well as the reuse of the same drug in cases of reactivation (as in LCH) erate or severe cardiotoxicity occurred in 30% of hematologic malig-
and its usefulness in the first lines of treatment. nancies and solid tumors within the first year of diagnosis and 10%
of them needed pharmaceutical intervention. The degree and course
of cardiotoxicity seem to differ significantly, suggesting that both: 1.
Publication Only Topic: AS05 SIOP Scientific programme / AS05.p underlying genetic predisposition and 2. presence of various other
Supportive Care and Palliative Care risk factors(both under investigation) contribute to the severity of its
manifestation.
PO125 / #1680 EARLY CARDIOTOXICITY IN CHILDREN
WITH HEMATOLOGICAL MALIGNANCIES AND SOLID TUMORS
PO126 / #1732 COMPARING THE EFFECTIVENESS OF
Kondylia Antoniadi1 , Vasiliki Tzotzola2 , Charikleia Kelaidi1 , Mirella ACUTE PAIN THERAPY IN CHILDREN WITH LEUKAEMIA
Ampatzidou1 , Sofia Papargyri1 , Evangelos Karanasios3 , Maria
Drakopoulou4 , Vassilios Papadakis1 , Petros Nihoyannopoulos4 , Nataliia Adamchuk1 , Nataliia Artomova2 , Oksana Kazmirchuk3
Dimitrios Tousoulis4 , Konstantinos Toutouzas34 , Nikolaos 1 Poltava State Medical University, Department Of Anesthesiology And
Thomaidis5 , Sophia Polychronopoulou1 Intensive Care, Poltava, Ukraine; 2 Municipal enterprise "Poltava municipal
1 “Aghia Sophia” Children’s Hospital, Department Of Pediatric Hematology- clinical children hospital of Poltava city council ", Onco-hematology Unit,
oncology, Athens, Greece; 2 Aghia Sophia Children Hospital, Department Poltava, Ukraine; 3 Rivne Regional Children’s Hospital, Onco-hematology
Of Pediatric Hematology-oncology, Athens, Greece; 3 “Aghia Sophia” Chil- Unit, Rivne, Ukraine
dren’s Hospital, Department Of Cardiology, Athens, Greece; 4 Hippokration
Hospital, First Department Of Cardiology, Athens, Greece; 5 National and Background and Aims: The aim of the study is to determine the most
Kapodistrian University of Athens, Department Of Cardiology, Athens, effective acute pain therapy regimen that provides a sufficient level of
Greece analgesia and does not exacerbate the manifestation of chronic pain.
Methods: The study involved 60 children with acute leukemia,
Background and Aims: Early Cardiotoxicity is the most serious non- aged 6 to 18 years. The patients were divided into groups
hematological toxicity of cancer treatment and is classified as acute (n=20) depending on the methods of anesthesia. Group 1 was
(<1 month) and early onset(< 1year from treatment initiation). This is administered opiates with paracetamol. Group 2 - opiates with parac-
a prospective analysis of the acute and early onset cardiotoxicity and etamol and gabapentin. group 3 - paracetamol with gabapentin,
outcome of patients, with hematological malignancies(HM) and solid opiates were administered only for breakthrough pain. Pain
tumors(ST). intensity, response to anesthesia, type of pain syndrome during
Methods: Between 2018-2021, 89 patients developed early car- chemotherapy, as well as 6, 12 and 24 months after remission were
diotoxicity. Patients’ characteristics: 44/89boys, median-age 10.0years assessed.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S621 of S635

Results: The average intensity of pain according to VAS on the 78th Conclusions: The prevalence of malnutrition in children with cancer at
day of chemotherapy, then 6-12-24 months after remission was: in diagnosis and during treatment ranges between 39.33% and 42.29%
group 1 3.4/3.3/2.3/2.2 points (mild pain); in group 2 3.3/3.3/2.2/1.8 depending on the method used for assessment, being lower with arm
points (mild pain); in group 3 2.6/1.5/1.0/0 (weak pain/no pain). The anthropometry and higher adding serum albumin
assessment of neuropathy according to the DN4 questionnaire on
the 78th day of chemotherapy, then 6-12-24 months after remis-
sion was: in group 1 20/24/24.8/26.3 points (neuropathy); in group PO128 / #135 STUDY AND ANALYSIS OF THE
2 18.9/22/24.7/26.1 points (doubtful result/neuropathy); in group 3 REHABILITATION SERVICES AVAILABLE FOR CHILDREN AND
15.7/14.1/10.8/6.7 (doubtful result). ADOLESCENTS WITH CANCER IN LATIN AMERICA
Conclusions: Chronic pain is formed in all groups. In the groups treated
with opiates as one of the main components of pain relief, the pain Pia Delano Baudet
syndrome is more pronounced, neuropathy is pronounced. Group 1, Universidad Catolica, Mph, Santiago, Chile
who received opiates without gabapentin, developed chronic pain ear-
lier than in group 2. In group 3 (no planned opiate prescription), pain Background and Aims: Rehabilitation is one of the aspects involved in
intensity indicators are the most favorable. The use of gabapentin at the CureALL program, which aims to achieve at least 60% survival for
the start of acute pain treatment significantly reduced the severity of children with cancer globally while ensuring that suffering is reduced
chronic pain. for every child. The role of cancer rehabilitation is key in maintaining
functional aspects and improving quality of life. The demand for pedi-
atric cáncer rehabilitation services will increase as the life expectancy
PO127 / #1924 MALNUTRITION IN PEDIATRIC PACIENTS of these children improves. This is a need that not all countries in Latin
WITH CANCER AT HOSPITAL IN THE NORTHWEST OF MEXICO America are prepared to cover. There is a gap in knowledge regarding
the number of pediatric cancer rehabilitation facilities, trained health-
Rocío Yemeli Buenrostro Aguilar1 , Guadalupe Valenzuela Aguilar1 , care providers, and what barriers exist to access these programs. With
José Rodrigo Lozano1 , Jorge Guzman2 , Eduardo Altamirano2 the support of the Sociedad Latinoamericana de Oncologia Pediatrica
1 Hospital Pediátrico de Sinaloa, Pediatric Oncology Unit, Sinaloa, Mexico; (SLAOP), the aim is to describe the resources to provide rehabilitation
2 Hospital Pediatrico de Sinaloa, Hemato-oncology Unit, Culiacan, Mexico to these children
Methods: A survey was sent to all members of the 21 countries of
Background and Aims: Pediatric patients with cancer are a group at SLAOP. The survey had 10 questions regarding different aspects of
high risk of developing malnutrition as a direct consequence of the dis- rehabilitation, access, and barriers.
ease and its treatment. The malnutrition prevalence varies between Results: Of the 21 countries who are members of SLAOP we had
5% and 50%, depending on the used tool, the type of cancer, time of responses from 19 of them, with a total of n=123 of response rate.
evaluation, and history. There are not unified criteria for nutritional Most providers (98.4%) knows the importance of rehabilitation in
diagnosis in pediatric patients with cancer, in Mexico. The main por- pediatric oncology, 86.2% had rehabilitation teams in their facilities,
pouse of this paper is to determinate the prevalence of malnutrition in and only 2 of them did not work in oncology. Only 19 respon-
pediatric children with cancer at diagnosis and during treatment dents referred to have pediatric oncology rehabilitation educational
Methods: This is a descriptive, longitudinal and prospective study programs. Barriers to access rehabilitation were reported by 83%
which include patients from 6 months to 18 years old, admitted at hos- providers: Approachability (n=20), Acceptability (n=3), Availability
pital in the northwest of Mexico with a newly diagnosed of cancer and (n=64), Affordability (n=35), Appropriateness (n=4) The main barriers
recently start treatment between August 2020 to March 2022. They associated with availability were Distance (n=31) and Lack of health
were classified into 4 groups of neoplasms: Leukemias, Lymphomas, care professionals (n=39).
Solid Tumors (ST), Central Nervous System (CNS) Tumor. Using an Conclusions: While the importance of pediatric oncology rehabilitation
anthropometric evaluation (weight, height, circumference of the upper is known in Latin America, there are many factors that act as barri-
arm, triceps skinfold) and albumin as a biochemical parameter, patients ers to access to care for children and adolescent. It is key to address
were classified into four categories of nutritional status these barriers to improve the quality of life of our Latin American
Results: sixthy-one newly diagnosed patients were studied. The dis- patients.
tribution according to neoplasms was: Leukemias 47.54%, ST 27.86%,
CNS tumor 13.11% and Lymphomas 11.47%, with the highest preva-
lence of malnutrition in Leukemias group. Based in the arm anthropom- PO129 / #1809 COVID-19 IN PEDIATRIC HEMATOLOGY
etry 31.14% severely depleted, 8.19% moderate nutritional depletion, AND ONCOLOGY DEPARTMENT OF RABAT (MOROCCO)
54.09% normal nutritional status and 6.55% overnutrition. With the
serum albumin 23.31%severely depleted, 19.67% moderate nutritional Maria El Kababri, Meriem Lakhrissi, Chaimaa Drief, Zineb Isfaoun,
depletion, 54.09 % normal nutritional status, while the 4.9% albumin Naoual El Ansari, Mohamed El Khorassani, Mohamed Khattab, Leila
sample could not be collected Hessissen, Amina Kili
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S622 of S635 ABSTRACTS

Mohammed V University. Rabat, Pediatric Hematology And Oncology to evaluate the efficacy of MeCHT in the LMIC setting in sub-Saharan
Department, RABAT, Morocco Africa.
Methods: Data was collected from 2016-2021 for patients 2-15 years
Background and Aims: Coronavirus disease 2019 (COVID-19) is an old with a diagnosis of a nonhematopoietic solid tumor that was pro-
infectious disease caused by severe acute respiratory coronavirus 2 gressive after treatment with 1 or 2 lines of chemotherapy. MeCHT
(SARS-CoV-2). Since the beginning of the COVID-19 pandemic, sev- was started when no other curative treatment options were available,
eral concerns have been raised regarding the management of pediatric patients had recovered from all acute toxic effects of earlier therapy,
oncology and hematology patients. These patients are known to be and they had a performance status of 3 or less.
highly immunocompromised due to their underlying disease as well as Results: There were 12 male and 3 female participants with a median
the treatments they receive, suggesting a risk of severe infection and age of 7 years old. Of the patients, 6/15 (40%) had stage 4 disease at
high mortality.Objective: To describe the clinical presentation and evo- presentation/time of diagnosis and 9/15 (60%) received MeCHT. The
lution of COVID-19 in patients followed in the department of pediatric most common adverse event for patients on MeCHT was grade 1-2
hematology and oncology in Rabat (Morocco). hematologic toxicity.
Methods: Retrospective study of 19 cases of COVID-19 in patients fol- Conclusions: MeCHT represents a promising therapeutic option in
lowed at the pediatric hematology and oncology department of Rabat high risk refractory and/or relapsed cancer populations in LMICs.
over a period from March 2020 to February 2022. Potential benefits of MeCHT include long-term disease stabilization
Results: The mean age of the patients was 8 years. Seven patients and significant improvement in the quality of life of patients. Parents
had a hematologic malignancy, while 7 others had a solid tumor. of the patients reported a reduced feeling of “hopelessness.” In the
Three patients had hemoglobinopathy and 2 others had aplastic ane- patients that received MeCHT, more emphasis was placed on end-of-
mia. Comorbidity such as insulin-dependent diabetes was reported in life discussions and patients were able to follow up with the health
one case and trisomy 21 in another case. Screening for SARS-COV- facilities nearest to their homes. Larger studies are needed to look at
2 infection was performed in 17 patients with clinical symptoms and progression-free survival for pediatric cancer patients in sub-Saharan
in 2 others with a positive contact history. Diagnosis of COVID-19 Africa.
infection was withheld based on positive polymerase chain reac-
tion (PCR) for SARS-CoV-2 in 15 patients, while it was withheld
on clinical and radiological arguments in 4 patients. Nine patients PO131 / #84 DIFFICULTIES IN PROVIDING PALLIATIVE CARE
received chemotherapy one month before the diagnosis of COVID-19 IN RURAL INDIA (WEST BENGAL) – EXPERIENCE OF AN NGO
with a mean delay of 8 days. Eight patients did not require hos-
pitalization and received treatment at home. Two patients required Nabanita Mandal
oxygen at the time of diagnosis and were admitted to an inten- Narikeldaha Prayas, Palliative Care, Panskura, India
sive care unit, while 9 patients were hospitalized in an isolation
unit. Background and Aims: As in any developing countries state of West
Conclusions: Pediatric hematology and oncology patients infected Bengal in India has a huge burden of cancer patients in advanced stage
with SARS-CoV-2 do not appear to be at increased risk of severe coming from rural area where awareness regarding the usefulness of
infection compared with the general pediatric population. palliative care in rather poor. Our goal is to give a pain free good quality
of life in these advanced stage cancer patients. Objective of this study
is to identify the main difficulties in achieving the above goal in a rural
PO130 / #1645 AN EXPERIENCE WITH METRONOMIC village setting in India.
THERAPY : A BEAM OF COMFORT Methods: Advanced cancer patients in need of palliative care in various
villages in of rural India were selected for this study. Their symptoms
Michelle Obat1 , Sarah Muma2 , Thomas Johnson3 and managements in that rural surroundings were evaluated by an
1 AIC KIJABE HOSPITAL, Paediatric Oncology, Nairobi, Kenya; 2 AIC KIJABE NGO (under the guidance of a senior palliative care specialist) work-
HOSPITAL, Paediatric Oncology, KIJABE, Kenya; 3 University of South Car- ing in that area. An attempt was made to identify the main obstacles in
olina School of Medicine Greenville, School Of Medicine, Greenville, United getting proper palliative care in a rural setting.
States of America Results: Pain, fatigue are the main symptoms effecting these patients.
In most patients pain and other symptoms control were grossly inad-
Background and Aims: Pediatric cancer survival in high-income coun- equate due to lack of properly trained manpower in the rural India.
tries is above 80% whereas in low-and middle-income countries However regular homecare visits by a group of social workers were of
(LMICs) it is only 10-20%. Few options remain after first- and second- immense help in the last few months of life. NGO team was well guided
line standard treatment protocols in LMICs. Metronomic chemother- by a palliative care specialist.
apy treatment (MeCHT) refers to chronic administration of low doses Conclusions: There is a wide gap of trained manpower in this filled
of chemotherapy for sustained, prolonged, and active plasma levels of in rural areas of India. Dedicated groups from rural area itself need
drugs, producing favorable tolerability. The purpose of this study was encouragement and proper training, so that difficult symptoms can be
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S623 of S635

managed locally along with necessary social and psychological support treatment of pediatric oncology and bone marrow transplant (BMT)
to these patients. patients at our centre.
Methods: All children getting admitted to the oncology and BMT ward
of SRCC Children’s Hospital, managed by Narayana Health were tested
PO132 / #1296 OBJECTIVES AND HEALTHCARE CLINICAL for COVID-19 by Polymerase chain reaction (PCR) prior to admission
ACTIVITY IN AN INTEGRATIVE PEDIATRIC ONCOLOGY UNIT or if febrile. We retrospectively analyzed data of the COVID-19 posi-
tive patients at our hospital for their presentation, absolute neutrophil
Esther Martínez count (ANC), absolute lymphocyte count (ALC), need for intensive care;
Hospital Sant Joan de Deu, Barcelona, Pediatric Oncology, Esplugues de deferment of cancer-directed therapy, and their outcome from 1st April
Llobregat, Spain 2020 to 28th February 2022.
Results: Two hundred and thirty-eight samples were tested in the given
Background and Aims: Describe the objectives and healthcare clini- time period for 84 patients. Of these, 23 patients (27.3%) tested posi-
cal activity of an integrative pediatric oncology unit, showing the care tive for the virus. Eleven patients tested positive during the 1st wave,
model of our center. 5 in the 2nd wave, and 7 in the 3rd wave. Eleven (48%) had neutrope-
Methods: Describe complementary treatments and feasibility analysis nia (ANC <1500 /mm3 ) ; 6 had severe neutropenia ( ANC <500/mm3 );
based on the rate of therapeutic rejection of the treatments offered by & 11 had lymphopenia ( <3000/mm3 ). A complete blood count was
the integrative pediatric oncology unit. Retrospective analysis of the not done for 4 patients. Sixteen patients had fever on presentation,
most frequent indications for pediatric oncology acupuncture. 3 had Fever & diarrhoea; 3 were asymptomatic positive on admis-
Results: In two years, in our integrative oncology unit have evaluated sion for chemotherapy, & 4 were tested because family members
310 patients and made 1,900 visits and 1,210 acupuncture treat- tested positive. The median time for negativity by PCR was 14 days
ments in patients undergoing active treatment and survivors. The most (range: 4 to 31). Six children needed COVID-19 infection-directed ther-
frequent reasons for consultation are: gastrointestinal motility dis- apy; &12 children had their treatments delayed. Two children needed
orders (41.1%) such as nausea, vomiting, diarrhea and constipation; intensive care and there was no mortality due to COVID-19 in our
pain (37.6%) tumor, neuropathic, scar pain, functional, musculoskele- cohort.
tal, headache and abdominal pain; mood disorders (12.7%) such as Conclusions: COVID-19 infection in our cohort was mild with no mor-
psychophysical asthenia, anxiety, insomnia or night terrors; and oth- tality. Possible reasons were that the children were already following
ers (8.6%) such as loss of appetite, allergic reactions or hot flashes. social isolation and wearing masks in public. Outcomes of treatment
The mean age of the patients is 9.5 years, and the rate of therapeutic delays will need to be evaluated in the long term.
rejection is 2.3%.
Conclusions: An integrative oncology unit covers a medical and social
demand, and its objectives are putting the patient in the center PO134 / #122 IMPACT OF PHARMACIST IN OPTIMIZING
to reduce toxicities, improve well-being and quality of life without PATIENT SAFETY BY PROMOTING DOUBLE CHECK
increasing polypharmacy and ensuring patient safety, with the aim of PROCEDURES FROM PRESCRIPTION TO PREPARATION AND
generating a positive impact on the disease. Integrative care model DISPENSING OF CHEMOTHERAPEUTIC DRUGS
is feasible healthcare model that is well accepted by patients and
their families for the management of different pediatric oncological Sidra Mustafa, Naila Fareed
conditions. INDUS HOSPITAL AND HEALTH NETWORK, Clinical Pharmacy, KARACHI,
Pakistan

PO133 / #1045 THE SPECTRUM OF COVID 19 INFECTION Background and Aims: In order to promote patient medication safety,
IN PEDIATRIC ONCOLOGY – SINGLE CENTRE EXPERIENCE we implemented independent double check procedures from making
FROM A DEVELOPING COUNTRY of chemo prescription to dispensing of chemotherapeutic drugs. The
rational of this study is to explore the impact of pharmacist in reducing
Ruchira Misra1 , Priyank Rajan1 , Sujata Mushrif1 , Swati Kanakia1 , the risk of medication errors and preventing side effects of chemother-
Shaheen Shaikh2 , Purna Kurkure1 apeutic drugs. By promoting double checking we can reduce the risk
1 SRCC Children’s Hospital, managed by Narayana Health, Pediatric Hema- of any misadventure related to chemotherapeutic drugs side effects,
tology Oncology And Bmt, Mumbai, India; 2 SRCC Children’s Hospital, dispensing and administration.
Micobiology, Mumbai, India Methods: A single center, retrospective analysis was performed on
all chemotherapy computerized prescription order entry of hospital-
Background and Aims: The COVID-19 pandemic has impacted every- ized and ambulatory patients, in a free of cost tertiary care hospital
one. It was anticipated that immunosuppressed patients would have of Pakistan, who received chemotherapy in the month of June 2021
severe infections and complications. We aimed to study the presenta- –-February 2022. All data is entered in intervention sheet of phar-
tion of COVID-19 over the 3 waves and the implications it had on the macy which has some predefined parameters e.g: wrong dose, wrong
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S624 of S635 ABSTRACTS

drug, addition of drug, wrong chemo phase, Wrong notes etc. Oncology 2020), 2nd Wave (December 2020- May 2021), 3rd Wave (June-
Consultant entered notes in HMIS after patient assessment. Later, November 2021) and 4th Wave (December 2021-February
ambulatory clinic pharmacist will prepare patient specific chemo- 2022). Relative risk was calculated, and one-way ANOVA was
protocol as per physician notes. A physician will check and sign applied.
protocols and enter medication order via CPOE system In pharmacy, Results: There were 1502 admissions to pediatric oncology of which
two chemo pharmacist at processing unit will entertain CPOE orders. 7.72% were under PGH. We registered 6.67 GH/1000 days. The mean
Chemo processing pharmacist, will ensure that all chemo drugs of number of cases was 3.29 (+1.792). The relative risk decreased in the 4
specific patient is prepared as per protocol before dispatching from pandemic periods compared to the pandemic period, this being higher
pharmacy. in the 3rd wave (53.6%). AAT decreased significantly in the 2nd wave
Results: Qualitative intervention data is analyzed by using excel as (n:22, 46. 04, +15.52 min) compared to the pre-pandemic period (n:50,
a data analyzing tool. Interventions are categorized under fixed per- 91.10, + 91.43) (p<0.05).
defined parameters. Percentages of these parameters will accesses the Conclusions: The risk of a patient being reported as PGH decreased
risk and types of errors occured and minimized by parmacist. Pro- during the different waves of the pandemic. The AAT showed a ten-
cedure of individual double check significantly reduces medication dency to decrease throughout the pandemic. This indicates that the HD
errors. strategy, despite modifications and contingencies during the pandemic
Conclusions: Pharmacist is the part of core team of quality care ser- waves, did not show relevant changes.
vices. In an oncology setting, medication errors can cause serious
threats to patients. By changing stratigies we are able to maximize
patient safety. PO136 / #603 ANALYSIS OF THE HOSPITALIZATION TIME,
IN A TERTIARY HOSPITAL IN MEXICO, OF CHILDREN WITH
CANCER WITH CLINICAL DETERIORATION, EVALUATED BY
PO135 / #1543 COMPARISON OF RISK FACTORS AND AAT THE EVAT SCALE
OF PEDIATRIC ONCOLOGY PATIENTS TREATED WITH GH
PROTOCOL IN COVID-19 PANDEMIC IN A TERTIARY HOSPITAL Carlos Pérez-Alvarado1 , Gloria Licona1 , Rodolfo Ortiz1 , Gabriel
IN MEXICO Muñoz1 , Asya Agulnik2 , Rodolfo Risco1 , Alejandro Romero1 , Diana
Reyes1 , Ismael Kelly-Perez3 , Betsabe Vázquez4 , Lorelei Galicia5 ,
Carlos Pérez-Alvarado1 , Jose De Jesus Loeza1 , Jorge Ortiz1 , Joselyn Claudia Morales1 , Rosario Ponce1 , Citlalli Morales1 , Jose De Jesus
Bamaca1 , Santiago Rodriguez1 , Jaxibe Morales1 , Erika Hernandez1 , Loeza1
Ismael Kelly-Perez2 , Betsabe Vázquez3 , Lorelei Galicia4 , Alejandro 1 Centro Estatal de Cancerología "Dr. Miguel Dorantes Mesa", Oncología
Romero1 , Diana Reyes1 , Claudia Morales1 Pediatrica, Xalapa, Mexico; 2 St. Jude Children’s Research Hospital, Depart-
1 Centro Estatal de Cancerología "Dr. Miguel Dorantes Mesa", Oncología ment Of Global Pediatric Medicine, Memphis, TN, United States of Amer-
Pediatrica, Xalapa, Mexico; 2 Universidad Veracruzana, Ingienería Mecan- ica; 3 Universidad Veracruzana, Ingienería Mecaninca, Xalapa, Mexico;
inca, Xalapa, Mexico; 3 Universidad Veracruzana, Inteligencia Artificial, 4 Universidad Veracruzana, Inteligencia Artificial, Xalapa, Mexico; 5 Instituto

Xalapa, Mexico; 4 Instituto Nacional de Pediatria, Pediatría, Ciudad de Nacional de Pediatria, Pediatría, Ciudad de Mexico, Mexico
Mexico, Mexico
Background and Aims: Background and Objectives: One of the main
Background and Aims: Infections associated with febrile neutropenia causes of death in children with cancer is their Clinical Deteriora-
are among the main comorbidities of the pediatric hemato-oncologic tion (CD). For this reason, special attention has been paid to the
patient, even more so in middle to low-income countries, as is Mex- Pediatric Early Warning Scales (PEWS), which are tools that identify
ico. In particular, in our hospital CECan Xalapa, Veracruz, Mexico, in patients at clinical risk. Some of the signs and symptoms of CD patients,
the period 2016-2020, it was shown that 67.61% of patients with such as infections, can be associated with a long hospital stay and
ALL in the induction stage of treatment had some type of infection. a high possibility of death. For this, it was decided to monitor the
The treatment of childhood cancer patients with infection, as well as length of hospital stay of the patient with CD, evaluated by the EVAT,
other hospital processes, have been modified by the current COVID-19 scale in our hospital and show if it is a factor that may favor their
pandemic. Therefore, we carried out this research trying to show the mortality.
differences in terms of relative risk and antibiotic administration times Methods: Cross-sectional study of patients with and without DC
(AAT), in patients treated with the GH protocol (PGH) in the different hospitalized in pediatric oncology between January 2019 and Febru-
waves of COVID-19. ary 2022. For research purposes 3 groups of patients were created:
Methods: Cross-sectional study of all patients with infections Patients who did not present Deterioration (PND), Patients Surviving
who came to outpatient clinic and were treated with GH from Deterioration (PSD) and Patients Not Surviving Deterioration (PNSD).
April 2019 to February 2022. Four groups were formed: pre- Descriptive statistics were performed, as well as Student’s t, χ2 and
pandemic (April 2019-February 2020), 1st Wave (March- November ANOVA according to the nature of the data.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S625 of S635

Results: 1930 admissions to pediatric oncology were recorded, of Conclusions: Educating families of pediatric cancer patients regarding
which 4.76% were CD. There was a significant difference in the per- optimal oral hygiene practices is essential to curb the incidence and
centage of infections in the PNSD (65.4%) and PND group (7.6%). improve overall outcomes of oral mucositis.
The hospitalization time of the PND was longer compared to the PSD
group (p<0.001) and the PNSD (p=0.021). The time between hospi-
tal admission to CD identification was significantly longer in PNSD PO138 / #1527 DETERMINANTS OF BRUXISM IN PEDIATRIC
(p=0.001). PATIENTS WITH ACUTE LYMPHOBLASTIC LEUKEMIA
Conclusions: Patients with CD who do not survive present a longer
hospitalization time, higher frequency of infections, as well as a longer Carlos Avila-Sanchez1 , Jocelyn Rivera-Mijares2 , Maria Del Pilar
time lapse in which CD is detected. All this suggests that there is a pos- Cubria-Juarez3 , Isaac Urrutia-Ballesteros4 , Enoe Cruz-Martinez5 ,
sibility that a longer hospitalization time and a longer time in which CD Aline Suarez-Rayo6 , Monica Ruiz-Leyva7 , Andrea Ellis-Irigoyen3 ,
is identified may lead the patient to an infectious process that increases Lourdes Vega-Vega8 , Gabriela Escamilla-Asiain9
the possibility of death. 1 Hospital Infantil Teleton de Oncologia, Pediatric Dentistry, Queretaro,
Mexico; 2 Hospital Infantil Teleton de Oncologia, Emergency Medicine,
Queretaro, Mexico; 3 Hospital Infantil Teleton de Oncologia, Pediatric Oncol-
PO137 / #1690 INCIDENCE OF ORAL MUCOSITIS IN ogy, Queretaro, Mexico; 4 Hospital Infantil Teleton de Oncologia, Palliative
PEDIATRIC ONCOLOGY PATIENTS AT A TERTIARY CARE Medicine, Queretaro, Mexico; 5 Hospital Infantil Teleton de Oncologia, Pedi-
HOSPITAL IN PAKISTAN atric Neurology, Queretaro, Mexico; 6 Hospital Infantil Teleton de Oncologia,
Psychooncology, Queretaro, Mexico; 7 Hospital Infantil Teleton de Oncolo-
Natasha Baig1 , Wasfa Farooq1 , Yumna Syed2 , Muhammad Rafie Raza1 gia, Physical Therapy, Queretaro, Mexico; 8 Hospital Infantil Teleton de
1 Indus Hospital and Health Network, Pediatric Oncology, Karachi, Pakistan; Oncologia, General Director, Querétaro, Mexico; 9 Hospital Infantil Teleton
2 Indus Hospital and Health Network, Electronic Medical Records, Karachi, de Oncologia, Medical Director, Queretaro, Mexico
Pakistan
Background and Aims: Oral neurotoxicity secondary to chemother-
Background and Aims: Mucositis is a painful condition involving the apy manifests as pain, numbness, paresthesia, and involuntary move-
oral cavity. Cytotoxic radio and chemotherapy regimens cause mucos- ments. Bruxism is characterized by repetitive mandibular movements
alcells to lose their ability to replicate once damaged, leading to without functional purposes, leading to extensive loss of dental struc-
increased susceptibility to infection, mouth ulcers and ultimately, mal- ture if it is possible to overcome their adaptive capacity, negatively
nutrition. Several factors contribute to development of mucositis in impacting patients’ quality of life. This study aims to describe the pedi-
pediatric cancer patients, severe neutropenia being the most impor- atric population with acute lymphoblastic leukemia (ALL) diagnosed
tant. Maintenance of good oral hygiene is essential to prevent oral with bruxism and identify associated factors.
mucositis (OM). Methods: A retrospective review of the clinical records of pediatric
Methods: A retrospective study was conducted including children 0 to patients with ALL was carried out from September 2014 to February
16 years who presented to Indus Hospital and Health Network with 2022. All patients with ALL were evaluated by the Pediatric Dentistry
OM secondary to cancer treatment between April and August 2021. service with a diagnosis of bruxism in Teleton Children’s Oncology
Following ethical approval, patient records were extracted from the Hospital.
Hospital Management Information System. Data was analysed using Results: Of the total patients with ALL evaluated in the hospital
SPSS v23. (n=124), 16 (4 women, 25%, and 12 men, 75%) were diagnosed
Results: Ninety one cases of OM were recorded, with a 57% male pre- with bruxism (12.9%). The age of the study group was between 1.66
dominance, mean age of 7.59 ± 4.36 years and average body mass to 18 years. Of the total number of patients with bruxism, para-
index of 12.6. Incidence was 21.6% and average length of hospital functional oral habits were identified in 7 (43.75%), of whom four
admission was 5.12 ± 4.23 days. Of the graded cases, 28% comprised presented cheilophagia (57.14%), one morsicatio buccarum (14.28%),
WHO grade 1, 43% grade 2, 27% grade 3 and 2% grade 4. Eighty three and 2 showed a combination of both habits (28.57%). At the time of
patients were on chemotherapy, 5 on chemo and radiation therapy, 2 dental evaluation, 13 had a documented diagnosis of peripheral neu-
on palliative care and 1 off treatment. B-ALL accounted for 64% of the ropathy secondary to Vincristine (81.25%), of which 8 (61.5%) had
cases. Absolute neutrophil count ranged between 2.8 to 15296 cells/μL wear on the incisal edges and occlusal surfaces. The dental treatment
(average 1286 cells/μL, mode 70 cells/μL). Eleven patients had a pos- applied in 100% of the patients was using an occlusal guard at night.
itive blood culture. Soda bicarbonate mouthwash was prescribed to In 9 (56.25%) patients, it was necessary to change the guard due to
all, Fluconazole to 87%, Magic mouthwash to 70%, Miconazole cream perforation or wear.
to 55%, Amphotericin B to 21%, Acyclovir to 10% and Nystatin to Conclusions: Dental assessment in patients receiving chemotherapy
2%. Most patients recovered whereas 4 expired before recovery. Six- with oral neurotoxic effects is necessary for the diagnosis and timely
teen children had recurrent episodes of OM, over 80% being leukemia treatment of parafunctional habits that have a negative impact on the
patients. patient’s quality of life.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S626 of S635 ABSTRACTS

PO139 / #235 A PILOT INVESTIGATION OF GDF15 IN PO140 / #868 CHARACTERISTIC OF COVID-19 IN


CHILDREN WITH CANCER PEDIATRIC PATIENTS WITH HEMATOLOGICAL MALIGNANCIES,
TREATED IN THE WESTERN-UKRAINIAN SPECIALIZED
Daniel Runco1 , Linda Dimeglio2 , Charles Vanderpool3 , Danielle PEDIATRIC MEDICAL CENTER
Halsey4 , Teresa Zimmers5
1 Riley Hospital for Children, Indiana University School of Medicine, Pedi- Olha Dorosh1,2 , Olha Troyanovska1,3 , Halyna Lytvyn4 , Iryna
atrics, Pediatric Hematology/oncology, Indianapolis, United States of Amer- Tsymbalyuk-Voloshyn1 , Oksana Vorobel1 , Alla Stepanyuk1 , Khrystyna
ica; 2 Riley Hospital for Children/Indiana University School of Medicine, Bodak1 , Olena Kozlova1,2 , Maria Stasiv4 , Natella Basa4
Pediatrics, Pediatric Endocrinology, Indianapolis, United States of Amer- 1 Western Ukrainian Specialized Pediatric Medical Centre, Department Of
ica; 3 Riley Hospital for Children/Indiana University School of Medicine, Hematology And Intensive Chemotherapy, Lviv, Ukraine; 2 Danylo Halytsky
Pediatrics, Gastroenterology, Indianapolis, United States of America; 4 Riley Lviv National Medical University, Lviv, Ukraine, Of Pediatrics And Neonatol-
Hospital for Children/Indiana University School of Medicine, Pediatrics, ogy Fpge, Danylo Halytsky Lviv National Medical University, Lviv, Ukraine,
Pediatric Hematology/oncology, Indianapolis, United States of America; Lviv, Ukraine; 3 Danylo Halytsky Lviv National Medical University, Lviv,
5 IU Simon Comprehensive Cancer Center, Indiana University School of Ukraine, Department Of Pediatrics N2, Lviv, Ukraine; 4 Danylo Halytsky Lviv
Medicine, Dept Of Surgery, Indianapolis, United States of America National Medical University, Lviv, Ukraine, Of Ppediatric Infectious Diseases,
Lviv, Ukraine
Background and Aims: Growth Differentiation Factor 15 (GDF15),
an inflammatory cytokine in the TGF-β family, is elevated in chronic Background and Aims: It is unclear if the risk of COVID-19 may be
diseases including cancer. In animal models, GDF15 induces anorexia, higher or lower in pediatric patients with hematological malignancies
nausea and vomiting, and is associated with skeletal muscle loss and (HM). The aim of our research is to find the common health problems
lipolysis, suggesting GDF15 as a key mediator of cancer cachexia. caused by SARS-CoV-2 in patients with oncohematological diseases.
Multiple biologicals targeting the GDF15 pathway are in clinical devel- Methods: This was a retrospective study of 21 medical records of
opment; however, current research on GDF15 in cancer is limited to paediatric patients with HM tested positive for SARS-CoV-2. Data
adults. Our study aimed to measure GDF15 in children with cancer regarding age, sex, clinical symptoms and results of investigations were
versus healthy children. collected.
Methods: Children ages 2-21 years with previously untreated cancer Results: 15 patients (71.4%) were diagnosed with acute lymphoblas-
at a single institution were screened and included if planned treat- tic leukaemia (ALL), 4 (19%) with acute myeloid leukaemia (AML),
ment included chemotherapy and excluded if mechanically ventilated, 1(4.7%) with Non-Hodgkin diffuse lymphoblastic lymphoma and 1
on supplemental nutrition, or having comorbidity impairing nutritional with Langergance Cell Histiocytosis. The majority of patients were
ingestion, digestion or absorption. An age- and sex-matched healthy males (66%). The most common symptoms of COVID-19 were: fever,
control was enrolled for each participant with cancer. Centrifuged cough, digestive signs, dermatitis. 2 patents with ALL were diag-
serum from whole blood was frozen. ELISA was performed in a single nosed with venous thrombosis. We didn’t find common changes in
batch with appropriate controls (Human GDF15 Quantikine ELISA). blood count due to the myelosuppressive effect of the previous
Results: Twenty-eight children had analyzable samples (14 with can- chemotherapy. The majority of patients (76%) had mild infections
cer and 14 controls). Participants were primarily male (60.7%), with with SARS-CoV-2 and needed delay with chemotherapy not more
mean age of 9.1 years (SD=5.4 years). Diagnoses included acute lym- than 2 weeks. The course of coronavirus disease didn’t depend on
phoblastic leukemia (N=4), neuroblastoma (N=4), lymphoma (N=3), the status of the disease and the type of cancer treatment. Our
brain tumor (N=1), acute myeloid leukemia (N=1), and osteosarcoma patient with ALL, who had first-line chemotherapy simultaneously
(N=1). Children with cancer had a higher median GDF15 compared with COVID-19, soon died of septicaemia. One of our patient with
to controls (707.2pg/mL versus 330.7pg/mL respectively, p<0.0001 by relapsed AML, who had long delay in AlloBMT after COVID-19,
Mann Whitney two-tailed test). GDF15 was normally distributed in was diagnosed with second relapse and died from disease progres-
controls but demonstrated right skew in children with cancer. GDF15 sion.
values did not differ by age or sex in either group. Conclusions: The study provides analysis of COVID-19 in pediatric
Conclusions: GDF15 was higher in children at cancer diagnosis, regard- patients with HM. Patients infected with SARS-CoV-2 showed favor-
less of age or sex. These data suggest GDF15 may be an important able clinical outcome with 24 months OS of 85% and EFS of 82%. The
inflammatory cytokine present at time of cancer diagnosis with impli- majority of patients had an asymptomatic, mild or moderate course of
cations for cancer-associated anorexia and cachexia. Our planned COVID-19. SARS-CoV-2 increased the risk of liver toxicity and venous
next steps are to examine GDF15 throughout cancer treatment and thrombosis in this group of patients. The patients from analyzed cohort
correlate GDF15 levels with body composition and gastrointestinal needed the same approach for COVID-19 treatment as for the other
symptoms. infective complications.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S627 of S635

PO141 / #73 FUTURE PERSPECTIVES IN ONCOFERTILITY Background and Aims: Treatment abandonment is defined as the
CARE FOR GIRLS WITH PEDIATRIC CANCER absence of curatively directed therapy for four or more weeks without
a medically indicated cause. It’s a problem that encompasses differ-
Maria Elisabeth Madeleine Van Der Perk1 , Anne-Lotte Van Der Kooi2 , ent factors. It’s important to take measures, since whatever the cause
Annelies Bos1,3 , Simone Broer3 , Margreet Veening1 , Jeanette Van of abandonment, it leads to the progression of the disease, a relapse
Leeuwen3 , Hanneke Van Santen1,4 , Wendy Van Dorp5 , Marry Van Den or death. Identify factors associated with the abandonment of cancer
Heuvel-Eibrink6 treatment in order to create strategies to prevent it by intervening in a
1 Prinses Máxima Centrum voor kinderoncologie, Pediatric Oncology, timely manner and thus reduce the rate of abandonment.
Utrecht, Netherlands; 2 Erasmus MC–University Medical Center, Depart- Methods: An analysis of cases of hemato-oncologic malignant disease
ment Of Obstetrics And Gynecology, Rotterdam, Netherlands; 3 UMC will be performed. The records will be analyzed randomly. For the
Utrecht, Reproductive Medicine And Gynaecology, Utrecht, Netherlands; descriptive analysis, averages, medians, frequencies, percentages and
4 Wilhelmina Children’s Hospital, University Medical Center Utrecht, interquartile ranges will be used.
Department Of Pediatric Endocrinology, Utrecht, Netherlands; 5 IJsselland Results: Of 162 patients identified as treatment abandonment, 33
Ziekenhuis, Department Of Obstetrics And Gynecology, Rotterdam, Nether- patients were randomly analyzed. Results: Male:51.5%, Female:48.5%,
lands; 6 Princess Máxima Center for Pediatric Oncology, Division Of Pedi- Infants:18.1%, early-childhood:33.3%, middle-childhood:21.2%, Ado-
atric Oncology, Utrecht, Netherlands lescent:27.2%. The mean age of patients was 6.2 years, and Median
age: 4.5 years, Caregiver age: 15-20 years:3%, 20-30 years:45.4%,
Background and Aims: Infertility is a serious early, as well as late 30-40 years:33.3%, 40-50 years:18.1%, the mean age in caregivers:
effect of childhood cancer treatment. If addressed timely at diagnosis, 32.1 years and the median age: 30 years. Gender of caregiver:
fertility preservation measures can be taken, preferably before start of Female:93.9%, Male:6%. Educational level of the caregiver: Illiter-
cancer treatment. ate:6%, Elementary and middle school:48.5%, High school:27.2%,
Methods: However, pediatric oncologists may remain reluctant to offer College: 15.1%, Unknown:3%. Family composition: Nuclear:60.6%,
counseling on fertility preservation methods, while infrastructures to Single-parent:12.1%, Extended: 21.2%, Reconstituted:6%, Number of
freeze ovarian tissue have become available and are currently con- family members: 1-5:78.7%, 5-10:18.1%, >10:3%. Illness in any of the
sidered standard care for pre- and post-pubertal girls at high risk of main caregivers: Yes:21.2%, No:78.7%. Time of transfer to hospital:
gonadal damage. 0-1 hour:51.5%, 1-2hours:18.1%, 2-3 hours:12.1%, >3 hours:18.1%.
Results: More importantly, risk factors have been identified for can- Household origin: Urban: 78.7%, Rural:21.2%, Parental employment:
cer treatment-related impairment of gonadal function, and the first Formal:12.1%, Informal:87.8%, Tumor type:Leukemia: 54.5.%, Lym-
successful pregnancies have been reported after auto-transplanted phomas:6%, Brain:12.1%, Abdominal:3%, Bone:24.2%, Current patient
ovarian tissue, harvested from children. Additionally, great progress status: Deceased by disease:54.5%, Alive with disease:15.1%, Alive
has been made in the field of ex vivo maturation of oocytes in frozen without disease:21.2%, Unknown: 9%.
ovarian tissue, which provides opportunities for those at risk of ovarian Conclusions: We observed a high mortality rate in patients who aban-
micrometastasis. don treatment. By identifying the abandonment factors, we could
Conclusions: Hence, it is time to counsel girls at risk and make every prevent it by implementing timely strategies to avoid abandonment
effort to viably store their ovarian tissue, now more than ever before. and reduce mortality.

Publication Only Topic: AS05 SIOP Scientific programme / AS05.q PO143 / #682 BENEFITS OF APPLYING AN EARLY
Psychosocial (PPO) DETECTION SCALE

PO142 / #1166 “IDENTIFICATION OF FACTORS ASSOCIATED Tania Gonzalez, M. Àngels Claramonte


WITH THE ABANDONMENT OF TREATMENT IN THE Hospital Sant Joan de Deu, Social Worker (oncology), Esplugues de Llobregat
PEDIATRIC ONCOLOGY PATIENT IN HOSPITAL OF (Barcelona), Spain
GUADALAJARA MEXICO”
Background and Aims: The oncological disease has an impact on both
Mariana Michelle Gómez Del Toro, Erika Casillas Toral, Xóchitl the patient and the family. It also affects all the live projects in which
Ramírez Urenta, Regina Navarro Martín Del Campo, Jorge Macías they are involved. Therefore, the intervention of the social worker in
Toscano, Oscar Gonzalez Ramella, Fernando Antonio Sánchez Zubieta, this area is essential. The social work service intervenes systematically
Manuel Martínez by protocol with all families with a child with a new oncological diagno-
Hospital Civil de Guadalajara Dr. Juan I Menchaca., Hemato- Oncología sis. This initial assessment allows us to identify possible needs, risks, or
Pediátrica, Hospital Civil De Guadalajara Dr. Juan I Menchaca, Guadalajara, vulnerability situations. The family is the focus of our intervention and
México, Guadalajara, Mexico our aim is to offer a comprehensive care. To guarantee this, the inter-
disciplinary and coordinate work with the other care team from the
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S628 of S635 ABSTRACTS

hospital, and the other health, social and education services is a must. the feeling of belonging to the class group and facilitates school
Objectives, to expose: - Social indicators are used to establish the clini- reintegration.
cal social diagnosis. - Model and circuit intervention. - Results collected Conclusions: Social connectivity and peer relationships during cancer
and conclusions. treatment could have a role in improving the quality of life and mental
Methods: During the welcome interview, a social diagnosis is estab- state of patients and their families. It also helps to the normalization
lished to evaluate the family’s vulnerability level (from 1 to 3). From this of illness as part of life. Exploring successful cases such as this one
diagnosis, the team will decide with the family the work plan adapted could help pave the way for the implementation of programmes aimed
to the needs. This plan specifies the type of intervention that the situa- at facilitating this type of inclusive educational interventions.
tion requires, the frequency of follow-up interviews, coordination with
community services, etc. In order to unify criteria, a social assessment
tool has been developed in paediatric oncology based on a set of indica- PO145 / #1151 LESSONS LEARNED BY SPONSORING
tors, including the family APGAR as an instrument for measuring family VIRTUAL CAMPS DURING A PANDEMIC
functioning.
Results: Data collection of new onco-haematological cases attended in Tim Porea, Michelle Fritsch, Larry Geiger
2021: 171 families: Level 1 - 84, level 2 - 48, level 3 - 39. Baylor College of Medicine, Pediatric Oncology, Houston, United States of
Conclusions: This tool allowed systematization of the social diagnosis America
and has made the intervention and the follow-up interviews with fam-
ilies easier. 50% of the cases have required accompaniment during the Background and Aims: Camps for children with cancer improve cop-
process. The rest required a more intensive intervention, favouring the ing skills, emotional acceptance of illness, and they allow patients to
prevention of future difficulties. challenge themselves in a safe environment. They also support siblings
in similar ways. Camps provide an avenue to connection, camaraderie
and belonging. With the COVID pandemic in 2020 camps had to pivot
PO144 / #820 REMOTE CLASSROOM PARTICIPATION VIA to virtual programming.
VIDEOCONFERENCING FOR CANCER PATIENTS WHO CANNOT Methods: The Periwinkle Foundation implements several camping pro-
ATTEND SCHOOL BECAUSE OF HEALTH NEEDS: LESSONS grams. In 2020 they offered virtual camps for each program. Traditional
FROM A SUCCESS STORY activities were adjusted to accommodate a virtual format and new
activities were designed. Supplies for activities were mailed to campers
Francisca Jiliberto1 , Alexandra Canals Delgado2 , Simón Serka3 in advance. With in-person camps attendance is limited to a single sib-
1 Hospital sant Joan de Déu, Hospital School, Barcelona, Spain; 2 Hospital ling per patient whereas in virtual camps attendance for siblings was
Sant Joan de Déu, Hospital School, Barcelona, Spain; 3 University of Liver- unlimited. Patients who were too ill to attend camp in person were able
pool, Public Health, Terrassa, Spain to participate virtually.
Results: Compared to pre-pandemic 2019 figures, attendance for Day
Background and Aims: Cancer treatment can be a frequent cause of Camp in June 2020 was similar (63 vs 60, respectively). Attendance
school absenteeism. While hospital schools and homebound teaching for the overnight Camp Periwinkle in August 2020 was also compara-
programmes aim to ensure educational continuity, social aspects of ble (171 vs 189). The number of participating siblings was increased
school life are often neglected, which can create difficulties in adjust- for both virtual Day Camp (2019 siblings- 17, 2020 siblings- 20) and
ment, feelings of loneliness and social isolation. This study explores Camp Periwinkle (2019 siblings- 59, 2020 siblings- 70). For Camp
the experience of a seven year old leukemia patient, who was able Periwinkle 20 hospital inpatients were able to participate virtually,
to maintain permanent daily contact with her school through remote something not possible with in-person camps. Anecdotal stories from
classroom participation via videoconferencing throughout the treat- hospital staff described inpatients’ days being brightened, with the
ment. At the same time, it seeks to discover the meaning that the staff themselves also benefitting from patients’ excitement. Lessons
stakeholders give to this experience. learned in conducting virtual camp included the crucial need for tech-
Methods: A phenomenological method, which allows participants to nical support. Parents were able to see camp activities for the first
explain and describe their experience, was used. Semi-structured time.
in-depth interviews were held with the stakeholders involved. Conclusions: Virtual camps were feasible to conduct and
Results: This study revealed that a time for adaptation, attempts, increased sibling and inpatient involvement compared to in-
adjustments and learning in front of the new treatment situation by person camps. These benefits argue for the continuation of
the student, the family, the tutor, the class group and the school com- virtual experiences even as the return of in-person camps is
munity is needed. Ignorance of the disease and fear of the possibility ongoing.
of death can be a barrier for both children and adults. The perma-
nent remote classroom participation favors the invisibility of physical
changes due to treatment, helps to maintain positive states of mind and PO146 / #1865 A MOTHER’S DILEMMA
daily healthy routines for both student and family caregiver, increases
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ABSTRACTS S629 of S635

Sokhna Fatou Thioune1 , El Hadji Makhtar Ba2 , Soda Mamy Lo1 , Care - 2006). Thus, psychological support is essential in this process
Ndioba Mbengue2 , Alassane Seck1 , Pape Momar Gueye2 , Fatou of care, inevitably taking place towards death, in the short or medium
Ndoye Fall3 term. The objective of the study was to describe the first experience
1 Aristide Le Dantec Hospital, Oncopediatric, Dakar, Senegal; 2 Université of end-of-life support for children followed in the oncopediatric unit of
Cheikh Anta Diop, Unité D’oncopédiatrie/oncopediatric Unit (aristide Le the Aristide L. D. Hospital and the work following deaths.
Dantec Hospital), Dakar Peytavin, Senegal; 3 Aristide Le Dantec Hospital, Methods: This is a qualitative, descriptive study. The psycho-oncology
Oncopediatric, Dakar Fann, Senegal team provided end-of-life support. The latter took the form of psycho-
logical interviews, on the face-to-face model. They were addressed to
Background and Aims: Cancer remains the major cause of death both the accompanying persons and the child. Family mediations were
among children worldwide and Senegal is not spared. The scarcity sometimes necessary. After the death, telephone interviews followed
of specialized facilities for care, diagnostic difficulties and our socio- during the grief.
cultural beliefs reduce cure rates. The onco-pediatric unit of the Results: During the period from 1 July to 30 December 2020, 59
Aristide Le Dantec Hospital receives an average of 250 new cases of deaths were recorded. 14 families, including parents and children, have
cancer per year. Patients are admitted with accompanying persons, been prepared for the approach of death. 34 families were accompa-
who may be a parent, grandparent, uncle, aunt, brother, sister or sim- nied during the grieving process, usually through telephone interviews.
ple acquaintance of the family. This choice of care and its duration can Because most of the families resided outside the Dakar region or even
often be a source of family upheaval both from an emotional and func- in the countries bordering Senegal.
tional point of view. An upheaval that leads to a redistribution of roles Conclusions: Psychological support in palliative care is essential in the
within the family. Family that can be dislocated with a breakup of the management of childhood cancers. However, it is a lever that remains
parental and/or conjugal couple. to be developed in oncology, in Senegal.
Methods: We report a case study that highlights the socio-cultural
specificities in Senegal that mothers of children with cancer may face
Results: The accompanying persons are mainly mothers who leave PO148 / #892 ADOLESCENTS AND YOUNG ADULTS WITH
behind parents, husbands and the rest of the siblings, who are often ONCOHEMATOLOGICAL PATHOLOGY: TIME AND USE OF
entrusted. And moreover having to face the risks of being repudiated, SOCIAL NETWORKS WITHIN “STRANGER TEENS” PROGRAM
of being abandoned by the husband in favor of a new wife or of being COMPARED WITH HEALTHY PEERS
tossed between the choices of the family in law.
Conclusions: This is how these mothers find themselves in a dilemma Marta Tremolada1,2 , Francesco Vietina2 , Sabrina Bonichini1 , Marta
and even plagued by guilt when the child dies. Pierobon2 , Alessandra Biffi2 , Gianni Bisogno2
1 University of Padua, Department Of Development And Social Psychology,
Padova, Italy; 2 University of Padua, Pediatric Hematology, Oncology And
PO147 / #1397 EXPERIENCE IN THE END-OF-LIFE SUPPORT Stem Cell Transplant Center, Department Of Woman’s And Child’s Health,
OF CHILDREN TREATED IN THE ONCOPEDIATRIC OF SENEGAL Padova, Italy

Sokhna Fatou Thioune1 , El Hadji Makhtar Ba2 , Soda Mamy Lo1 , Background and Aims: It was estimated 4.000 diagnosis of neoplasms
Alassane Seck1 , Ndioba Mbengue2 , Pape Momar Gueye2 , Fatou in adolescents in 2016-2020 years (AIRTUM, 2017). Adolescent and
Ndoye Fall3 , Fatou Diagne4 , Mame Diouf4 , Ndiaye Awa4 Young Adults (AYA) patients used social networks for several purposes
1 Aristide Le Dantec Hospital, Oncopediatric, Dakar, Senegal; 2 Université (Eng et al., 2020). The aims of this study are to analyse the internet
Cheikh Anta Diop, Unité D’oncopédiatrie/oncopediatric Unit (aristide Le time/use and type of communications in AYA patients comparing with
Dantec Hospital), Dakar Peytavin, Senegal; 3 Aristide Le Dantec Hospital, those of healthy peers.
Oncopediatric, Dakar Fann, Senegal; 4 Aristide Le Dantec Hospital, Pediatry, Methods: Participants were 47 AYA patients in Oncohematology Clinic
Dakar, Senegal in Padua and their matched healthy peers. Majority were females (62%)
with a mean age of 17.53 years (SD=2.8), 13 under therapy and 34 off
Background and Aims: Since 1975, rates of new cancer among young therapy, 62% with a solid tumor diagnosis. After the informed consent
people under the age of 20 have increased by about 34%. In sub- signature, AYA were enrolled within the activities of “stranger teens”
Saharan Africa, 92 children per 1,000 live births die of cancer before program. Several self-report questionnaires on social network type of
their fifth birthday. This is 15 times higher than in industrialized use, reasons of using and type of communication were administered
countries (United Nations Inter-agency Group for Child Mortality Esti- adopting a secure online format Limesurvey. Matched healthy teens
mation, 2014). In Senegal, the pediatric oncology unit at Aristide Le were enrolled via web in the schools and in the universities in Veneto
Dantec Hospital receives 250 new cases per year. The cure rate of the and Trentino regions in Italy.
main types of cancer treated is 40%. Palliative care helps to maintain Results: Instagram was the most social used (101 minutes/day), fol-
a good quality of life, clinically and psychologically, for the child and is lowed by Whatsapp (95 minutes/day), Youtube (65 minutes/day)
a support for the family (Francophone Network of Pediatric Palliative and Tiktok (52 minutes/day). The principal reasons of use were
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S630 of S635 ABSTRACTS

boredom (63.8%), communication with friends (51.5%), staying in con- ning of the pandemic. In our population, the infection did not have an
tact with friends (36.2%). Hospitalized AYA used more Ask/Tellonym aggressive course.
than healthy peers (Z=-2.12; p=0.03). The preferred communication
method in sharing positive or negative events related to disease was
face-to-face meetings. Non-hospitalized teens used more both Insta- PO150 / #1630 DEVELOPING MULTIDISCIPLINARITY IN
gram (Z=-3; p=0.003 for sharing positive events; Z=-2.4; p=0.01 for PEDIATRIC ONCOLOGY (PO) IS NECESSARY TO MEET THE
negative events) and Whatsapp (Z=-2.4; p=0.01 for positive events; WHO 2030 GICC TARGET: EXPERIENCE OF THE FRENCH
Z=-2.7; p=0.006 for negative events). Under therapy AYA adopted AFRICAN GROUP OF PEDIATRIC ONCOLOGY (GFAOP)
more Tik Tok (Z=-2.1; p=0.03), Ask/Tellonym (Z=-1.9; p=0.05) e
Twitch (Z=-2.38; p=0.01) than off therapy ones. Women shared more Pierre Bey1 , Fatou Akonde2 , Line Couitchere2 , Lukamba Robert3 ,
positive events on Instagram (Z=-2.41p=0.01) and both positive (Z=- Chantal Bouda4 , Claude Moreira5 , Jean Michon6
2.99; p=0.003) and negative ones (Z=-3.2; p=0.001) on Whatsapp than 1 GFAOP, Vice-president, NANCY, France; 2 CHU TREICHVILLE, Oncol-
men. ogy Pediatry Unit, ABIDJAN, Côte d’Ivoire; 3 CUL, Pediatric Oncology,
Conclusions: Some clinical suggestions could be derived from these Lubumbashi, Congo; 4 Centre Hospitalier Universitaire Yalgado Ouédraogo,
empirical results. Pediatrics, Ouagadougou, Burkina Faso; 5 Institut Jean Lemerle, Pediatric
Oncology, Dakar, Senegal; 6 GFAOP, Villejuif, PARIS, France

Publication Only Topic: AS05 SIOP Scientific programme / AS05.r Background and Aims: Background GFAOP was supporting PO pilot
Epidemiology, Policy and Advocacy units in French speaking African countries since 2000 through train-
ing, common guidelines for 5 curable diseases, providing cancer drugs,
PO149 / #2008 RETROSPECTIVE ANALYSIS OF COVID-19 registration of cases. In 2017, a program, funded by the Bristol-Myers
INFECTION IN PEDIATRIC ONCOLOGY PATIENTS IN URUGUAY Squibb Foundation, was built to develop multidisciplinary care in units
in addition to early diagnosis actions, decresae discontinuation of care
Bruno Cuturi1 , Paloma Amarillo2 , Florencia Nuñez1 , Luis Castillo1 , by support to parents. Aim To improve practically multidisciplinarity
Anaulina Silveira1 , Beatriz Irigoyen1 practice at each step of care.
1 Pereira Rosell Hospital, Pediatric Hemato Oncology, Montevideo, Uruguay; Methods: - A three-day residential interactive seminar with 2 objec-
2 Pereira Rossell Hospital Perez Scremini Foundation, Hematology Oncology tives: . to share general notions about childhood cancers to understand
Department, Montevideo, Uruguay why multidisciplinary care is mandatory . to organize daily multidis-
ciplinary practice: informative reports, regular tumor board meetings
Background and Aims: Uruguay declares a health emergency on (TBM). So, the 16 teams were invited to send 5 doctors involved in the
March 16, 2020, after the World Health Organization (WHO) defines care of children with cancer to participate to one of the seminars. - Fol-
coronavirus 2019 infection (COVID-19) as a pandemic on March lowed by on-site workshops bringing together specialists involved . to
11, 2020. Different actions were implemented, such as protection write local referential for the 5 diseases (+1) . to organize regular TBM
protocols for health personnel and their users. Patients with onco- (local or by twinning).
logical pathology are recognized as a risk group. The description and Results: Eighty-one doctors from 15 teams (19 pediatricians, 16
analysis of pediatric patients with oncological pathology who con- pathologists, 15 pediatric or general surgeons,+1 anesthesiologist, 14
tracted infection during the SARS-CoV2/CoVID-19 pandemic is carried ophthalmologists, 10 radiologists and 6 radiation oncologists) were
out. attending one of the three seminars in June 2018, April and September
Methods: A diagnostic test is performed by protocol to all patients who 2019 in Dakar, with interactive discussions opening to recommenda-
are admitted in our service, have symptoms, had COVID contact or tions. More than 60 referential were written after local workshops up
require an imaging study or planned surgery. Patients who attended to August 2021. Local TBM are now active in 12 teams.
the onco-pediatric service in the period between April 1, 2020, and Conclusions: This program has allowed improvement of multidisci-
March 14, 2022 with positive SARCoV2 tests carried out in our service plinarity among most of the teams with continuous progression.
were included.
Results: A total of 620 patients were attended, of which 52 presented
positive PCR for SARCoV2. 17 had a malignant hematologic disease. 19 PO151 / #367 PANDEMIC-COVID19 DELAYS DIAGNOSIS OF
patients contracted the infection during 2022. Treatment was discon- CANCER IN CHILDREN AND ADOLESCENTS IN WESTERN
tinued in 13 of 33 patients on active treatment, with an average delay PARANÁ- BRAZIL
of 15 days, there were no relapsed patients. 24 patients had symptoms,
of which 17 were respiratory. None of the patients required admission Carmem Maria Costa Fiori, Aline Rosa
to the intensive care unit, and none died. Hospital do Câncer de Cascavel- UOPECCAN, Pediatric Oncology, Cascavel,
Conclusions: CoVID-19 pandemic has created a challenge in the health Brazil
area. Cancer patient care led to treatment delays, mainly at the begin-
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ABSTRACTS S631 of S635

Background and Aims: In 2020, Brazil and the world faced an 16 St. Jude Children’s Research Hospital, Global Pediatric Medicine, Mem-
outbreak of severe acute respiratory syndrome coronavirus 2 (SARS- phis, United States of America; 17 St Jude Children’s Research Hospital,
CoV-2) in which it became a pandemic. In 2020 and 2021we observed Global Pediatric Medicine, Memphis, United States of America
a greater number of children and adolescents arriving at the referral
center with advanced disease (stage III/IV). OBJECTIVE- To evaluate Background and Aims: The AMARTE ( Increasing Resources and Spe-
the number of cancer cases in children and adolescents during the Pan- cialized Training ) Project was created in 2014, under the coordination
demic period (2020-2021), as well as the degree of clinical staging at of Barretos Children’s Cancer Hospital (PIOXII Foundation),joining 13
diagnosis. Childhood Cancer Center localized in several different regions in Brazil
Methods: Medical records were retrospectively evaluated in children . At first, it consisted of weekly meetings for clinical discussions via tele-
under 19 years of age treated from January 2020 to December 2021, conference that allowed for optimization of therapeutic proposals and
in a referral hospital for the diagnosis and treatment of childhood can- diagnostic accuracy . In 2019, the Board of the St. Jude Global program
cer. Low Risk (LR) (stage I and II) and High Risk (HR) (stage III and and the Board of the Pio XII Foundation signed a cooperation agree-
IV) criteria were considered for solid tumors. Leukemias followed the ment to join efforts and create a structured collaborative network of
HR criteria, age < 12 months and > 10 years, and leukocytes number innovation, research and teaching. In September 2020, 13 institutions
>50,000/mm3. from the 5 regions of Brazil have joined in AMAR TE Alliance with
Results: 144 patients were treated, of which 89 (62%) were considered defined objectives : i) Equalize Diagnostics, ii) Standardize Treatment,
High Risk and 55 (38%) Low Risk. There was a predominance of High iii) Epidemiological and Survival Studies and iv) Scientific Development
Risk over Low Risk per year, from 2020 and 2021, different from the and Innovation.
values found in the last five years in 2014 and 2018, where there Methods: An Alliance Management Committee was created, and a
was a predominance of Low Risk over High Risk. Executive Committee with representative members of each Institution
Conclusions: Preliminary data analysis allows us to observe that chil- manages tactical and programmatic programs for improving contin-
dren and adolescents with cancer in the COVID19 Pandemic arrived at uously childhood cancer care, reducing the asymmetry of access to
the referral center with advanced disease at diagnosis, this fact has not diagnostic and therapeutic tools . There are currently seven sub-
been observed since 2014, after the early diagnosis program developed committees meeting periodically: Pediatric Surgery, Palliative Care,
in the region since 2008. It is possible that this increase in high-risk Nursing, Morphology and Flow Cytometry, Pathology and Radiology.
cases may be related to the Covid 19 pandemic and the delay in the In addition, study groups were created to generate and disseminate
demand for specialized medical services. knowledge, to exchange experiences between partner institutions, and
to expand professional relationships, contributing to the strengthen-
ing of AMARTE Alliance. There are currently five study groups in the
PO152 / #1886 AMARTE ALLIANCE IN BRAZIL AS A MODEL following areas: Bioethics, Nutrition, Dentistry, Psychology and Social
TO EQUALIZE DIAGNOSIS AND TREATMENT AMONG Work.
DIFFERENT CENTERS WHO TREAT CHILDHOOD CANCER Results: In May 2021 during a 3 day-workshop, 5 groups defined
practical objetives to be conducted by all members in: diagnostic, treat-
Luiz Fernando Lopes1 , Isis Magalhães2 , Mara Pianovsky3 , Karla ment, palliative care,fundraising and data uniform registry to allow
Rodrigues4 , Marcelo Miloni5 , Viviany Viana6 , Flora Watanabe7 , epidemiological and survival studies
Carolina Alvares8 , Joaquim De Aguirre-Neto9 , Pablo Santiago10 , Conclusions: This Alliance and also the meetings that are occur-
Patricia Carla Lima11 , Manuela Segredo12 , Anna Beatriz Amaral13 , ring show we are in the right direction and good practicing changes
Patricia Loggeto14 , Monika Metzger15 , Paola Friedrich16 , Carlos regarding the 5 working groups are already possible to perceive
Rodriguez-Galindo17
1 Barretos Children’s Cancer Hospital, Pediatric Oncolgy, BARRETOS, Brazil;

2 Hospital da Criança de Brasília José Alencar, Nutrition, Brasília, Brazil; PO153 / #261 ADOLESCENTS AND YOUNG ADULTS WITH
3 Hospital Erastinho, Ped Oncology, Curitiba, Brazil; 4 Hospital das Clinicas CANCER. EXPERIENCE IN A LATINOAMERICAN INSTITUTION
UFMG, Ped Oncology, Belo Horizonte, Brazil; 5 GACC - Grupo de Assistên-
cia à Criança com Câncer, Pediatric Oncology, São José dos Campos, Brazil; Mariana Nana1 , Carolina Basile2 , Ezequiel Recondo2 , Stella
6 Hospital Albert Sabin, Ped Oncology, Fortaleza, Brazil; 7 Hospital Pequeno Ballestrini2 , Martin Hornos2 , Diego Rosso2
Principe, Ped Oncology, Curitiba, Brazil; 8 Hospital de Amor, Ped Oncol- 1 Hospital de Clinicas Jose de San Martin, Pediatric - Oncohematology Sec-
ogy, Porto Velho, Brazil; 9 Santa Casa Belo Horizonte, Ped Oncology, Belo tion - Aya Group, Buenos Aires, Argentina; 2 Hospital de Clinicas Jose de
Horizonte, Brazil; 10 Hospital Sao Vicente de Paula, Ped Oncology, Passo San martin, Pediatric - Oncohematology Section - Aya Group, Buenos Aires,
Fundo, Brazil; 11 Hospital Regional Oeste, Ped Oncology, Chapeco, Brazil; Argentina
12 Hospital Clinicas Botucatu, Ped Oncology, Botucatu, Brazil; 13 Hospital

Clinicas Uberlandia, Ped Oncology, Uberlandia, Brazil; 14 St Jude Research Background and Aims: The spectrum of cancers affecting adolescents
Hospital, Ped Oncology, Memphis, United States of America; 15 St. Jude Chil- and young adults(AYA) is distinct from that affecting both children and
dren’s Research Hospital, Pediatrics, Memphis, United States of America; older people.Cancer in AYA patients(p) has unique clinical,genetic and
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S632 of S635 ABSTRACTS

biological features.Treating this group of p is challenging. We describe Methods: We conducted a cross-sectional survey using a structured
the epidemiological characteristics,clinical features,histological type questionnaire among HCPs attending PEWS training workshops in
and outcome of AYA treated in an latinoamerican institution for the last Agogo and Tamale, Ghana. Extracted Data was analyzed with Microsoft
10 years by pediatric oncologists. Excel©.
Methods: Epidemiological and retrospective study.We reviewed the Results: Overall, 67 trainees completed the survey: 24 CN; 24 MD;
medical records of patients(p) between 15 and 25 years with cancer 19 CHN. While all CN and MD knew that children could have cancer,
treated in a pediatric department of a general hospital in Argentina only 3(16%,p=0.0001) CHN had no knowledge. All CN and MD learnt
from January 2012 to December 2021.We will compare our results about paediatric cancers in their health training institutions whereas
with international reports. the media was the main information source for majority, 9(47%) CHN.
Results: Seventy five p.Median age:16, range(15-25).Male: 43p(57%). More MD (92%) and CN (75%) compared to CHNs (37%) thought PCs
Cancer predisposition syndrome: 1p.Referred from another institu- were common in Ghana. Only 7(10%) respondents (5 MD; 2 CN) had
tion: 38p(50%), pediatric:15p; adult 7p, another region: 16p.Type of any knowledge of the differences between paediatric and adult can-
tumors:solid 43p(57%); hematological 32p(43%).Histology: leukemias cers. Regarding knowledge of PEWS, 24% of MDs and CNs admitted
18p(ALL 13p, AML 4p, CML 1p); lymphomas 14p(hodgkin 6p, lym- any knowledge compared to 16% CHN. Only 12% of respondents knew
phoblastic 3p, diffuse large cell 4p, anaplastic 1p); sarcomas 16p(ewing the Saint Siluan early warning signs of paediatric cancer. About 63%
6p, rhabdomyosarcomas 6p, epithelioid 1p, histiocytic 1p, desmo- MD, 50% Cs, and 37% CHN were confident in identifying PEWS. Half,
plastic 1p, myofibroblastic 1p); germ cell tumor 11p(gonadal 10p); 52% of all respondents including almost all 17(89%) CBHNs did not
osteosarcoma 8p; carcinomas 5p(cavum 1p, adenoid cystic 1p, adrenal know the commonest PC in Ghana.
1p); others 3p. Solid metastatic tumors: 74%. Relapsed hematologi- Conclusions: Significant knowledge gaps on PEWS exist among Ghana-
cal disease:21.8%. Treatment: pediatric protocols 74p.Second disease ian HCPs, especially CHN. Training institutions and audiovisual media
1p.First line treatment:84%. Pregnancies: 2p. Deaths: 29p(38.6%), are viable PEWS educational platforms. While MDs are comparatively
progression/relapse 18p and toxicity 11p. the most PEWS-aware, most HCPs lacked confidence in identifying
Conclusions: Most of the p treated were adolescents.Half of them were them.
referred from another institution.The most frequent histology were
leukemias, lymphomas, sarcomas and gonadal germ cell tumors.First
line treatment was based in pediatric protocols.Our mortality rate PO155 / #1273 UNIQUE PATTERN OF CANCER
was higher than reported, likely related to an elevated proportion of DEVELOPMENT IN PEOPLE WITH DOWN SYNDROME
metastatic solid tumors and relapsed leukemias in our population.
Marta Osuna Marco1,2 , Águeda Tejera2 , Blanca López-Ibor1
1 HM HOSPITALS/CIOCC, Pediatric Hematology And Oncology Unit, Boad-
PO154 / #622 HEALTHCARE PROVIDERS’ KNOWLEDGE OF illa del Monte, Madrid, Spain; 2 Francisco Vitoria University, Instituto De
THE EARLY WARNING SIGNS OF PAEDIATRIC CANCERS IN Investigaciones Biosanitarias, Pozuelo de Alarcón, Spain
GHANA
Background and Aims: Down Syndrome (DS), which is caused by the
Lawrence Osei-Tutu1 , Kwame Akoi1 , Akosua Oforiwaa2 , Ronald presence of an extra chromosome 21, is the most frequent cause of
Miah3 , Ayire Adongo4 , Prince Aryee5 , Nedda Ayi-Bisah1 mental retardation caused by a genetic aberration. They present a
1 Komfo Anokye Teaching Hospital, Department Of Child Health, Kumasi, unique pattern of tumors, which has not been explained to date. Glob-
Ghana; 2 Presbyterian Hospital, Department Of Paediatrics, Agogo, Asante- ally, they have a similar or slightly decreased lifetime risk of developing
Akim, Ghana; 3 Ghana Health Service, Asante-akim North District, Agogo, cancer compared to the general population. However, they have a
Asante-Akim, Ghana; 4 World Child Cancer, Ghana, Korle Bu Teaching Hos- very increased risk of developing certain tumors and, on the contrary,
pital, Accra, Ghana; 5 Roche Products Ghana Limited, Market Access, Policy there are some other tumors which appear only exceptionally in people
And Governmental Affairs, Accra, Ghana affected by DS. We aimed to study the incidence of different tumors in
people with DS.
Background and Aims: Nearly three-quarters of paediatric cancer Methods: We performed and exhaustive literature research, focusing
(PC) patients remain undiagnosed in Ghana, although most children on the studies regarding epidemiological data.
with cancer access primary healthcare services prior to diagnosis. Two Results: - Tumors with a very high incidence: acute lymphoblastic
comprehensive treatment centers (CTCs) combined, record 350 of the leukemia, acute myeloblastic leukemia, testicular cancer. - Tumors with
expected 1200 cases annually. Inadequate awareness of paediatric a similar or slightly higher incidence: retinoblastoma, intracranial and
cancer early warning signs (PEWS) among healthcare providers (HCPs) extracranial germ cell tumors, penile cancer, bone tumors, soft-tissue
contributes to delayed presentation, potentially resulting in a lack of sarcomas. - Tumors with a reduced incidence: central nervous system
urgency in referrals to CTCs and/or undiagnosed deaths. We aimed tumors, glial tumors in childhood, cancer of the oral cavity, upper air-
to assess HCPs comprising clinical nurses (CN); Medical Doctors (MD) way cancer, bronchus cancer, lung cancer, uterine cervix cancer, thyroid
and Community Health Nurses (CHN) knowledge on PEWS. tumors, skin cancer, head and neck tumors. - Tumors with a very low
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S633 of S635

incidence: Breast cancer, prostate cancer, Wilms tumor, medulloblas- Conclusions: This is the first nationwide study using prospective data
toma, neuroblastoma. Many other tumors present controversial data to map childhood cancer diagnostic pathways with diagnostic intervals.
regarding its increased or decreased incidence. Publication of the protocol will allow other nations to follow suit in
Conclusions: The unique pattern of tumors of people with DS is so order to improve outcomes via earliest possible diagnosis.
special that should encourage further studying for the possible causes
for this protection. This is probable secondary to an unbalanced gene
expression caused by the extra chromosome 21. Deeping our knowl- PO157 / #97 THE USE OF A WARD ROUND TEACHING
edge in the molecular alterations secondary to the genetic imbalance TOOL IN A PAEDIATRIC ONCOLOGY DEPARTMENT
would probably increase our understanding of the physiopathology
of these tumors and, thereby, be able to create targeted therapies Trisha Soosay Raj1 , Natacha Omer1 , Amy Gray2
that would be beneficial to both people with Down syndrome and the 1 Queensland Children’s Hospital, Oncology, Brisbane, Australia; 2 Royal
general population. Children’s Hospital, Paediatrics, Melbourne, Australia

Background and Aims: Despite ward rounds being fundamental to


PO156 / #302 THE CHILDHOOD CANCER DIAGNOSIS (CCD) hospital-based clinical training, the reported educational value is low.
STUDY: A UK OBSERVATIONAL STUDY TO DESCRIBE This is exacerbated in busy environments, with missed learning oppor-
REFERRAL PATHWAYS AND QUANTIFY DIAGNOSTIC tunities due to implicit learning, time barriers and lack of ward round
INTERVALS IN CHILDREN AND YOUNG PEOPLE WITH CANCER structure. The STIC framework (Set, Target, Inspect and Close) pro-
vides a learner-centred, structured approach to ward round teaching,
Shaarna Shanmugavadivel1 , Jo-Fen Liu1 , Kavita Vedhara1 , David aimed to enhance education within limited timeframes. We aimed to
Walker1 , Ashley Ball-Gamble2 , Angela Polanco3 , Shalini Ojha1 investigate how the introduction of the STIC framework impacts on
1 University of Nottingham, Academic Unit Of Population And Lifespan Sci- learner-centred teaching within the Paediatric Oncology department
ences, Nottingham, United Kingdom; 2 Children’s Cancer and Leukaemia of a tertiary hospital.
Group, Ceo, Leicester, United Kingdom; 3 Children’s Cancer and Leukaemia Methods: A mixed-methods approach was used to evaluate implemen-
Group, Public Contributions, Leicester, United Kingdom tation of the tool, with two participant groups comprising 16 junior
and senior doctors over three months. Surveys were used to docu-
Background and Aims: In 2018, the World Health Organisation ment junior staff experience on rounds pre- and post-implementation,
declared childhood cancer as a global disease burden and launched the with focus groups and interviews used for all participants to explore
Global Initiative for Childhood Cancer with the aim of improving sur- satisfaction and attitudes to the tool.
vival to 60% globally by 2030. In the UK, some tumour diagnoses are at Results: There was improved learner satisfaction across all domains of
a later stage and mortality is higher when compared with those in other the framework, specifically opportunities for leading clinical encoun-
parts of Europe. Many children and young people (CYP) experience ters and learning on consultant-led rounds. Despite consultants report-
delays to diagnosis which may further contribute to poor outcomes, ing lack of uptake of the tool, trainees reported improved teaching.
however there is a dearth of data. This study aims to understand Consultant beliefs and enthusiasm had a strong impact on trainee sat-
the current pathway of childhood cancer referrals and diagnosis and isfaction, influencing team culture. Trainees placed high value on active
quantify diagnostic intervals in the UK. participation and autonomy for their learning. Factors distinct to teach-
Methods: This is a multicentre observational study including all 20 ter- ing were reported to affect learner satisfaction, such as planning, time
tiary childhood cancer treatment centres in the UK. All CYP (0–18 management and departmental culture including being part of the
years) with a new diagnosis of cancer over the two year study period team and safety to ask questions.
are eligible for inclusion. Data on demographics, clinical symptoms, Conclusions: We demonstrate enhanced teaching despite poor per-
tumour location, stage, clinical risk group, referral routes and diag- ceived uptake, demonstrating potential of the STIC framework with
nostic intervals, as well refractory disease, relapse and survival at 5 further implementation. Our study also highlights that in addition to
years will be collected. Descriptive statistics with stratified analyses a specific teaching focus, consultant engagement and a safe clinical
by age, geographical region and cancer type will be performed. Asso- learning environment are crucial for learning.
ciations between diagnostic intervals/delay and risk factors will also be
explored.
Results: The study is open from 30/09/2020 – 30/04/2023. 806 par- Publication Only Topic: AS05 SIOP Scientific programme / AS05.s
ticipants have been recruited so far; 440 male (54.6%), 657(81.5%) of Survivorship
White ethnicity, 51(6.3%) Asian and 11(1.4%) Black. 398(49.3%) are
under 5 years of age. There are 306(38%) with leukaemia, 112(13.9%) PO158 / #663 EPIDEMIOLOGICAL FOLLOW-UP IN
lymphomas, 92(11.4%) CNS tumours, 58(7.2%) soft tissue sarcomas, CHILDHOOD CANCER SURVIVORS: EXPERIENCE OF A
57(7.1%) renal tumours, 52(6.5%) bone tumours and 48(6%) neurob- HOSPITAL COHORT AT HOSPITAL SANT JOAN DE DÉU
lastomas.
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S634 of S635 ABSTRACTS

Lourdes Arjona1 , Genoveva Maria Llano2 , Isabelle Thierry-Chef3 , Results: Between 2010 and 2020, a total of 312 patients aged 0-18
Ofelia Martinez2 , Elisabeth Cardis1 years old were diagnosed with cancer in our institution. Incidence rate
1 ISGLOBAL, Campus Mar, Barcelona, Spain; 2 Sant Joan de Déu, Oncol- of childhood cancer in our population was 118 cases/million children,
ogy, Sant Joan d’Espí, Spain; 3 ISGLOBAL, Radiation Programme, Barcelona, and mortality rate was 34/million children under 15 years old. Care
Spain emigration, mostly to Buenos Aires city, was 19%. Median age at diag-
nosis was 119 months old (2-223 m), male/female relation 1.2:1. Thirty
Background and Aims: Background and aims In recent decades the seven patients (11%) were under treatment at the time of analysis.
survival of children with cancer has improved dramatically in most Lymphoproliferative pathology accounted 51% of all cancers (leukemia
developed countries, and is currently around 75% in Spain. Childhood 83% and lymphomas 17%), and solid tumors 49%. The mortality was
cancer survivors have an increased risk of developing side effects due around 29% for both groups. Thirty patients (33%) died of progressive
to the treatment and the cancer itself. In Europe (France, England, disease.
Holland) and in the United States, epidemiological studies based on Conclusions: Incidence and mortality of childhood cancer in Santiago
national cohorts of survivors have allowed a detailed description of del Estero is a little lower than reported in the whole country. Despite
these side effects, identifying the risk factors. of being an extensive province, with scattered population, and limited
Methods: At the Hospital San Joan de Déu, recruitment of childhood accessibility to care services, our institution results are similar to those
cancer survivors started in 2012 and a cohort was created from 1980 reported by international literature.
to 2000. A health status and quality of life questionnaire was devel-
oped, similar to those in use in other European cohorts. Recruitment
of subjects was also implemented prospectively, when patients were PO160 / #1271 FACTORS AFFECTING THE ABILITY TO BE
coming back to the hospital for their follow-up consultation. PHYSICAL ACTIVE IN ADULT CHILDHOOD CANCER
Results: For retrospective recruitment, 2022 patients were identified, SURVIVORS
215 (10%) could not be contacted and 494 (24.4%) responded to the
questionnaire. The mean age at the time of participation was 23.87 Laura Jess1,2 , Maria Bäck3,4,5 , Marianne Jarfelt1,6
(6.79 SD). Regarding health status: 61 (13.1%) reported secondary can- 1 Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothen-
cer, 35 (7.54 %) thyroid disease, 25 (5.3%) growth hormone deficiency burg, Department Of Oncology, Gothenburg, Sweden; 2 Närhälsan Bolle-
and 7 (1.5%) diabetes. bygd Healthcare center, Rehabilitation, Bollebygd, Sweden; 3 Institute of
Conclusions: A nationwide follow-up could increase the statistical Medicine, Sahlgrenska Academy, University of Gothenburg, Department Of
power needed for detailed analyses of long-term side effects. The Molecular And Clinical Medicine, Gothenburg, Sweden; 4 Unit of Physio-
results would also allow the development of an appropriate follow-up therapy, Department Of Health, Medicine And Caring Sciences, Linköping
program for these patients. University, Linköping, Sweden; 5 Sahlgrenska University Hospital, Depart-
ment Of Physiotherapy, Gothenburg, Sweden; 6 Long-term follow-up Clinic
for Adult Childhood Cancer Survivors, Department Of Oncology, Gothen-
PO159 / #1108 SURVIVAL RESULTS OF CHILDHOOD burg, Sweden
CANCER AT A SINGLE INSTITUTION OF A DEVELOPING
COUNTRY Background and Aims: Previous studies indicate that adult childhood
cancer survivors have poor levels of physical fitness and that they do
Constanza Cafferata, Monica Luna, Romina Corona, Paola Reichel not achieve physical activity recommendations. A deeper understand-
Centro Provincial de Salud (CePSI) "Eva Peron", Onco Hematology Service, ing of factors that might have an impact on adult childhood cancer
Santiago del Estero, Argentina survivors ability to be physical active is needed in order to identify
individuals in need to receive support to maintain or improve healthy
Background and Aims: Argentina is a large and heterogeneous coun- lifestyle behaviors. The aim of this study was to explore factors that
try, with socioeconomic inequalities and geographic limitations along influence adult childhood cancer survivors ability to be physical active.
it. The aim of this study is to assess survival among pediatric can- Methods: Semi-structured interviews were conducted with twenty
cer patients at a middle complexity institution of Santiago del Estero, adult childhood cancer survivors with a median age of 31(20-47)
Argentina. years. Interviews were transcribed verbatim and analyzed with a latent
Methods: A retrospective analysis of all medical records of the patients content analysis according to Krippendorf.
who attended the Onco Hematology Service at Centro de Salud Infantil Results: Four main categories: “The impact of environmental fac-
(CePSI) “Eva Peron”, between January 2010 and December 2020 was tors”, “Individual factors”, “Consequences of the treatment or
performed. All malignant tumors, considering the International Child- disease” and “Perceived importance of health care” and 12 sub-
hood Cancer Classification (ICCC 3), were analyzed. Benign tumors, categories were identified. Participants expressed a need for
myelodysplastic syndromes, histiocytosis, hemophagocytic syndromes specific and individualized physical activity recommendations,
and retinoblastoma were excluded. Incidence, mortality and survival and extended help during follow-up. Furthermore, participants
were estimated using the Kaplan-Meier method. described growing up in a family where physical activity was
15455017, 2022, S5, Downloaded from https://onlinelibrary.wiley.com/doi/10.1002/pbc.29952 by Cochrane Mexico, Wiley Online Library on [10/07/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
ABSTRACTS S635 of S635

a natural habit as a facilitator for continuing a physical active ipants’ BMI Z-scores after treatment, particularly in the 5-9 and 10-19
lifestyle. age groups.
Conclusions: The results emphasize the gap between acute care and Conclusions: Incidence of LE is similar to other published studies.
follow up-care. Support for childhood cancer survivors to increase or Follow-up of survivors may have long-term benefits. Additional studies
maintain physical activity level should be included both during treat- in Brazil are needed.
ment and follow-up. Physical activity recommendations need to be
individualized and specific in order to increase compliance. Health care
professionals need to tailor support to the whole family, and increase PO162 / #980 OTOTOXICITY SURVEILLANCE: CURRENT UK
parent’s awareness of the importance of building good physical activity PRACTICE AND IMPACT OF PROPOSED IGHG GUIDELINES
habits through childhood and adolescence.
Carmen Soto1 , Krizstina Kiss1 , Rachel Cox2 , Roderick Skinner3
1 UCLH, Cypcs, London, United Kingdom; 2 University Hospitals Bristol
PO161 / #395 LATE EFFECTS OF CHILDHOOD CANCER NHS Foundation Trust, Paediatric Oncology, NU, United Kingdom; 3 Great
TREATMENT IN BRAZILIAN SURVIVORS North Children’s Hospital and Translational and Clinical Research Institute,
Newcastle University Centre for Cancer, Newcastle University, Hematol-
Rachel Martins, Maristela Reis, José Bernardes, Luiz Tone, Carlos ogy/oncology, Newcastle upon Tyne, United Kingdom
Scrideli
Ribeirão Preto Medical School, University of São Paulo, Pediatrics, Ribeirão Background and Aims: High-frequency hearing loss is a well-
Preto SP, Brazil recognised complication of some treatments for childhood cancer.
Surveillance strategies to monitor hearing loss once treatment has
Background and Aims: Childhood cancer survivors (CCS) population completed or as a late-effect are less clearly defined than those during
has increased importantly over the last decades due to major progress active treatment, and the decision to continue monitoring for hearing
reached in the treatment of pediatric cancer. CCS are at risk for mul- loss is up to individual clinicians. Guidelines published in 2019 by the
tiple therapy-related late effects (LE). This study aims to assess LE International Guideline Harmonisation Group (IGHG) for Late Effects
incidence and to identify risk factors in a group of CCS from a Brazilian of Childhood Cancer aim to fill this gap. However, these guidelines
pediatric oncology center. are likely to represent a significant change in practice for some clini-
Methods: Retrospective cohort study with CCS seen in a clinic from cians, with potential implications for audiometry and hearing services.
2002 to 2017, who have received chemotherapy and/or radiation ther- We conducted a survey of UK clinicians to establish 1) their current
apy. Anthropometric and clinical data were assessed and analyzed practice, 2) potential impact of implementing IGHG guidelines.
as descriptive statistics and hypothesis tests. LE cases were defined Methods: An electronic survey was distributed during November
according to published guidelines and classified in grades: mild to life- 2021. Participants described their current practice in three case-
threatening. Z-scores of participants’ body mass index (BMI) were studies based on IGHG guidelines. Participants were also asked if
compared between 3 moments: at diagnosis, at end of treatment and implementing the IGHG guidelines would represent a change in their
at last visit. clinical practice, or not.
Results: Final sample included 245 survivors; median age at diagno- Results: 35 people responded to the survey (25 doctors, 7 clinical nurse
sis was 7 years and median age at recruiting was 16 years; median specialists, and 2 advanced nurse practitioners). There was a wide vari-
follow-up period was 8.5 years. Most frequent primary diagnoses ation in existing clinical practice, and no clear standard of practice was
were acute lymphoblastic leukemia, central nervous system (CNS) identified. The majority (60.6%) of respondents felt that continuing
tumors and lymphomas. Most survivors received chemotherapy only, audiometry surveillance after treatment completion in line with the
and 33% received radiation therapy with or without chemotherapy. LE IGHG guidelines would represent a change from their current practice;
were identified in 53.8% of this cohort, including overweight/obesity 91% of respondents felt that tailoring surveillance to the child’s age
(36.6%), hearing loss (34%) and thyroid gland abnormalities (32.8%). would be a change in their practice.
Medulloblastoma, Ewing sarcoma and other CNS tumors were the dis- Conclusions: Current practice for monitoring hearing-loss after treat-
eases with the highest incidences of LE. In 66% of cases, LE were ment for childhood cancer varies greatly. Although the IGHG guide-
classified as mild or moderate. There was association between radio- lines provide some clarity for clinicians, these recommendations rep-
therapy and incidence of LE, especially thyroid gland abnormalities and resent a significant change in practice. Implementing these guidelines
short stature. Prevalence of overweight was 24.4% and prevalence of will require investment in audiometry services, as well as education and
obesity was 12.2%. There was a trend towards an increase in the partic- dissemination.

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