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Oncology Conference - Hepatoblastoma
Oncology Conference - Hepatoblastoma
Oncology Conference - Hepatoblastoma
K.N.R.
2 YEARS AND 11 MONTHS OLD/MALE
Date of birth : 06/14/2012
Date of Admission : 04/15/2015
Home Address : Brgy. Angeles, Atimonan, Quezon
City
Religion : Roman Catholic
Informant/s : Mother
Reliability : Good
RIGHT UPPER
QUADRANT
ABDOMINAL MASS
Chief complaint
HISTORY
10
months
PTA
History
2 months
PTA
1 month
PTA
ADMISSION
CT SCAN OF ABDOMEN
Hgb
RBC
Hct
Platelet
WBC
Differential Count
Neutrophils
Metamyelocyte
Bands
Segmenters
Lymphocytes
Monocytes
Eosinophils
Basophils
Reference
Range
115-125
4.5-5.1 x1012/L
0.34-0.37
150-450 x109/L
5-15.5 x109/L
0.50-0.70
0.00-0.05
0.50-0.70
0.20-0.40
0.00-0.07
0.00-0.05
0.00-0.01
04/13/15
102
4.92
0.32
352
11.1
0.48
0.57
0.38
0.05
-
REVIEW OF SYSTEMS
General Survey
Cutaneous:
HEENT:
Respiratory:
Cardiovascular:
Genitourinary
Endocrine:
Nervous/Behavi
(-) loss of consciousness, (-) seizures; (-) mood changes
oral:
Musculoskeletal: (-) joint swelling (-) joint pain
Hematologic
PERSONAL HISTORY
Gestational
27 y/o G1P0
Prenatal checkup: started 6 weeks; total of 10 pre-natal check-ups
Unremarkable history of exposure to viral exanthems and radiation
Hepa B screening: non reactive
OGTT screening: normal
(-) HPN
Bacterial Vaginosis on 3rd trimester-> vaginal suppository for 1
week
PERSONAL HISTORY
Birth
Term via NSD at 39 weeks
Epidural anesthesia
Clear amniotic fluid
BW 3.47 kg, BL 48 cm
PERSONAL HISTORY
Neonatal
APGAR score unrecalled
Nursery for one week due to sepsis (jaundice
and vomiting)
Giant nevi at the back at birth
Newborn screening done unremarkable
Hearing screening done- unremarkable
FEEDING HISTORY
Exclusively breastfed for 7 months approximately every 2-3 hours and for 15
minutes per breast
Complementary feeding at 7 months
Food
Kcal
Daily
Breakfast
Milk (1/4 cup)
Snack
Milk (1/4 cup)
Skyflakes cracker (1 pc)
Lunch
Rice (3tbsp)
Fish soup (1/4 cup)
Fish, fried (1/4)
Milk (1/4 cup)
Afternoon Snack
Milk (1/4 cup)
Dinner
Rice (3tbsp)
Fish soup (1/4 cup)
Fish, fried (1/4)
113
113
40
34
115
53
113
113
56
151
53
ACI
RENI
Percent deficient
954
1070
10.84%
Fine Motor
Receptive
Language
10
Pulls to stand
Crawls/ cruises
12
16
Walks alone
Creeps upstairs
18
Run stiffly
Walks backward
24
scribbling
and vertical
strokes
Expressive
Language
Discriminate
papa and
mama
Speaks few
words
PersonalSocial
Skills
Waves byebye
Kisses on
request
Turns 2-3
pages at a
time
Feeds self
Removes
clothes
Listens to
RED FLAGS
Social Emotional Red Flags
18months lack of simple pretend play (18mo)
Receptive Language
Does not point to 3 body parts (18mo)
Expressive Language
Vocabulary of not more than 35-50words
(24mo)
201
4
FAMILY HISTORY
(+) hypertension, (+) DM, (+) TBmaternal grandfather
(-) family history of malignancy
(-) liver or gastrointestinal disease
(-) asthma, allergy, thyroid disorders,
bleeding disorder
SOCIAL HISTORY
Source of funds: mother (househelper)
Lives in a well-lit, well-ventilated,
adequately spaced condominium (owned
by mothers employer)
No exposure to cigarette smoke
No pets at home
Water consumption: mineral water
IMMUNIZATION
Date Given
Route
Adverse
Reactions
BCG
6/15/12
IM
No reaction
Hep B 1
6/15/12
IM
No reaction
Hep B 2
2/27/13
IM
No reaction
Hep B 3
3/27/13
IM
No reaction
DTP1/OPV1
8/10/12
IM/PO
No reaction
Vaccine
Place
Given
De Los
Santos
Hospital
De Los
Santos
Hospital
Local health
center
Local Health
center
Local Health
center
IMMUNIZATION
DTP2/OPV2
9/12/12
IM/PO
No reaction
DTP3/OPV2
10/12/12
IM/PO
No reaction
Hib 1
2/9/13
IM
No reaction
Hib 2
7/12/13
IM
No reaction
Hib 3
8/20/13
IM
No reaction
Measles
3/27/13
IM
No reaction
MMR
8/16/13
SQ
No reaction
Varicella
10/25/13
SQ
No reaction
Rotavirus
Not Given
Local Health
center
Local Health
center
Private
Pediatrician
Private
Pediatrician
Private
Pediatrician
Private
Pediatrician
Private
Pediatrician
Private
Pediatrician
-
IMMUNIZATION UPDATE
Hepatitis B booster
Hepatitis A
Influenza
PPV
FAMILY PROFILE
Age
Educational
Attainment
Mother
31 y/o
3rd year
college
Father
Healthy
PHYSICAL
EXAMINATION
PHYSICAL EXAMINATION
General: awake, alert, well hydrated, well-nourished, carried
by mother, not in cardiorespiratory distress
Vital Signs: BP: 100/60mmHg CR 90 bpm regular, RR 21
cpm regular, Temp 36.5C
Anthropometrics:
Ht: 95.5 cm (z-score above 0);
Wt: 13 kg (z-score below 0),
WFH (z-score =-1)
Skin: warm, moist skin, good skin turgor, multiple nevus
distributed on face, trunk and extremities with tufts of hair,
largest patch measures: 27x 30 cm on the back extending to
PHYSICAL EXAMINATION
Head: normocephalic, no palpable masses, lesions, no facies
Eyes: eyelashes not matted eyelids not
conjunctiva, anicteric sclera, clear cornea
swollen,
pink
palpebral
PHYSICAL EXAMINATION
Lungs: symmetrical chest expansion, no retractions, equal vocal
fremiti, resonant on other lung fields, clear breath sounds
Heart: adynamic precordium, apex beat at the 4th LICS MCL, no
heaves, no thrills, no lifts, no murmurs
Abdomen: globular abdomen, umbilicus inverted, no visible
peristalsis, normoactive bowel sounds, Liver span 12cm, hard and
nodular liver edge
Genitourinary: ~4.5 penile length, no phimosis, testes descended
Extremities: pulses full and equal on all extremities, no cyanosis,
no edema, no limitation of movement, no clubbing of nails
PHYSICAL EXAMINATION
Neurolo
gic
Upon
admissio
n
SALIENT FEATURES
SALIENT FEATURES
UTZ: hepatomegaly of the right liver lobe
(11.2x6.8x8 cm) with some internal calcification in
the hilar region.
CT scan showed multilobulated solid and necrotic
tumor mass lesion with calcification in the liver
particularly in the right hepatic lobe
Elevated LFT, AFP, LDH
Low Hgb and Hct
Normal PT, PTT, Creatinine
INITIAL ASSESSMENT
Hepatoblastoma
Neurocutaneous Melanosis
Seizure disorder
Global Developmental Delay
APPROACH TO
DIAGNOSIS
PRESENTING
MANIFESTATION
Liver
Mass at
RUQ
DIFFERENTIAL DIAGNOSIS
Salient Features
Hepatoblastoma
HCC
3yo
5-18yo
Abdominal Mass
80%
Abdominal Pain
Elevated LFT
15-30%
30-50%
Elevated AFP
60-70%
50%
Anemia
common
common
Abdominal distention
HEPATOBLASTOMA
SEIZURE DISORDER
NEUROCUTANEOUS
MELANOSIS
GLOBAL
Initial Assessment
Work-Ups
Management
Outcome
3RD HD (4/17/15)
4TH HD (4/18/15)
multivitamins
2.5ml
QD and zinc 10mg/ml
2ml QD
Pediasure 2.5 scoops
in 95 ml to make 110
ml
13.5 kg
Actual caloric intake
700
Pediasure
330
calories
7th HD (4/21/15)
to Referred
anesthesiologist
USG- sedation
prior
procedure
8th HD (4/22/25)
To NPO
for IVF hydration (D5NSS
to 1L)
Histopathologic study
Ibuprofen
200mg/5ml
2ml
Suggested to be started
on lactulose
FETAL PATTERN
FETAL PATTERN
11th-12th
HD 14th
(4/25-26/15)
(4/28/15)
Asymptomatic
Good activity
HD
2D-echo -> no
pericardial
effusion
Ondansetron
as
premedication to
chemotherapy
18TH HD (5/2/15)
Clear nasal discharge
Increase
oral
fluid
intake
1 episode of vomiting
4 episodes of watery
loose stools
Fever,
chills
and
occasional
epigastric
pain
Cefuroxime
500mg/slow IV infusion
q8
21st
HD
(5/5/15)
Febrile episodes,
vomiting,
cervical
lymphadenopath
y
Urine
culture:
gram-negative
bacilli
Acute
Pyelonephritis
22nd
HD
(5/6/15)
Urine
CS:
Klebsiella
pneumonia
Meropenem 260
mg/per slow IV
infueion
24th
HD
(5/8/15)
Pediasure
3.5
scoops
KUB ultrasound
->
bilaterally
enlarged
kidneys
Urinary
retention
Mupirocin
cream
for
left
foot
phlebitis
Less
frequent
febrile eppisodes
Semiformed stools
GCSF
Co-trimoxazole
and TMP+SMX
31st HD (5/15/15)
Cxr
Miconazole Cream
on diaper and
scrotal area
Febrile episodes
Six episodes of soft, mushy
stools
32nd hd
(5/16/15)
CXR->
pneumonia
Febrile
episodes
Vancomycin
TMP+SMX
-> Cotrimoxazole
Occassional
rhonchi
Nonprojectile
vomitus
No
meningeal
and
Babinski
sign noted
33rd HD (5/17/15)
Febrile episodes
Occasional cough
Vomited saliva once
Poor appetite
AC 1200 cm
Mushy yellow bowel
movement
Febrile at 41.3C ->
tepid sponge bath to
IV paracetamol due
to its persistence
36th HD (5/20/15)
patient was highly
febrile
41.2C,
blood culture and
sensitivity test and
urine culture and
sensitivity
were
requested
with
ARD
and
with
sensitivity
to
Cefuroxime,
Meropenem,
PiperacillinTazobactam,
Vancomycin,
Amikcain
37th HD (5/21/15)
blood and urine
culture
and
sensitivity testing
were
facilitated
with
ARD.
PediatricsInfectious service
advised that the
patient be started
on
Cefepime.
PiperacillinTazobactam
was
shifted
to
Cefepime (650 mg
per
slow
IV
infusion over 30
39th HD (5/23/15)
patient had febrile
episode
with
a
maximum
temperature
of
37.7C.
Tepid
Sponge bath was
done
DISCUSSION:
HEPATOBLASTOMA
Epidemiology
Pathophysiology
Clinical Presentation
Diagnosis
Management
HEPATIC TUMORS
Primary hepatic
tumors are rare
in children (12%)
1% of
malignancies in
children (annual
incidence of 1.6
cases per
million)
Lanzkowsky, Philip. Manual of Pediatric Hematology and Oncology. 5th Edition. 2011
EPIDEMIOLOGY
Predominantly in
children below 3 years
old (80%)
Etiology is unknown
associated with FAP,
Beckwith-Wiedermann
Syndrome
Alterations in the
antigen presenting
cell (APC) / B-catenin
pathway
Birth weight
increasing risk as birth
weight decreases
DISORDERS ASSOCIATED
WITH INCREASED RISK
OF HEPATOBLASTOMA
Low-birth-weight infant
Gardner Syndrome
Prader-Willi Syndrome
Meckels Diverticulum
Beckwith-Wiedemann Syndrome
Li-Fraumeni Syndrome
Trisomy 18
Umbilical Hernia
Lanzkowsky, Philip. Manual of Pediatric Hematology and Oncology. 5th Edition. 2011
DISORDERS ASSOCIATED
WITH INCREASED RISK
OF HEPATOBLASTOMA
Familial Adenomatous
Polyposis
Beckwith-Wiedemann
Syndrome
Lanzkowsky, Philip. Manual of Pediatric Hematology and Oncology. 5th Edition. 2011
HEPATOBLASTOMA
EPITHELIAL TYPE
MIXED TYPE
Mixture
CLINICAL PRESENTATION
Common Manifestations
Large, asymptomatic
abdominal mass
3x right lobe compared to the
left lobe
Unifocal
Disease Progression
Weight loss
Anorexia
Vomiting
Abdominal pain
Lanzkowsky, Philip. Manual of Pediatric Hematology and Oncology. 5th Edition. 2011
DIAGNOSIS: LABORATORY
ALPHA-FETOPROTEIN (AFP)
Diagnosis
Monitoring
Elevated in almost all
hepatoblastomas
ANEMIA
Common
THROMBOCYTOSIS
Occurs in 30% of patients
DIAGNOSIS: ANCILLARIES
PLAIN RADIOGRAPHS
Characterize the hepatic mass
DIAGNOSIS: ANCILLARIES
ABDOMINAL ULTRASOUND
Characterize the hepatic mass
FETAL PATTERN
EMBRYONAL PATTERN
PATIENTS SPECIMEN
PATIENTS SPECIMEN
MANAGEMENT
Only patients in
whom complete
resection can be
achieved have a
reasonable chance of
cure
Possible in 40-50% of
patients
Chemotherapy plays
MANAGEMENT: SURGERY
Patients who are candidate for complete
resection include those with:
Tumors confined to the right lobe
Tumors originating in the right lobe that do not
extend beyond the medial segment of the left lobe
Tumors confined to the left lobe
PRETEXT STAGE 1
PRETEXT STAGE 2
Childhood Liver Cancer Treatment - National Cancer Institute. (n.d.). Retrieved from
http://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq#section/_373
PRETEXT STAGE 3
Childhood Liver Cancer Treatment - National Cancer Institute. (n.d.). Retrieved from
http://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq#section/_373
PRETEXT STAGE 4
Childhood Liver Cancer Treatment - National Cancer Institute. (n.d.). Retrieved from
http://www.cancer.gov/types/liver/hp/child-liver-treatment-pdq#section/_373
MANAGEMENT
Stage Management
I and
II
III ad
IV
MANAGEMENT:
CHEMOTHERAPY
Drug
Route
Dosage
Days
Cisplatin
IV over 6
hours
100
1
mg/m^2 or
5Slow IV
600mg/m^ 2
Fluorouraci push over 2/dose
l
2-4minutes
Vincristine IV push
over 1
minute
1.5mg/m^ 2, 9, 16
2/dose
Doxorubici IV over
30mg/m^2 1, 2
n
15minutes /dose
MANAGEMENT:
CHEMOTHERAPY
Drug
Mechanism of Action
Adverse Effects
Cisplatin
Nausea (76-100%)
Vomiting (76-100%)
Nephrotoxicity (28-36%)
Ototoxicity, especially in
children (31%)
Myelosuppression (2530%)
Anaphylaxis (1-20%)
5-Fluorouracil
Loss of appetite
Headache
Nausea Vomiting
Mucositis
Myelosuppression
MANAGEMENT:
CHEMOTHERAPY
Drug
Mechanism of Action
Adverse Effects
Vincristine
Neurotoxicity
Cranial nerve palsies
Jaw pain
Autonomic neuropathy
(obstipation and ileus)
Doxorubicin
Neutropenia (52%)
Anemia (52%)
Leukopenia (42%)
Pruritus (37%)
Nausea (37%)
Stomatitis (37%)
Fatigue (33%)
CHF (30%)
MANAGEMENT:
RADIOTHERAPY
Not curative for intrahepatic disease
because hepatic tumor dose exceeds
hepatic tolerance
Useful for shrinking unresectable disease
PROGNOSIS
5 year Survival Rate
Stage
Percentage
I/II
III
IV
90
60
20
5 year Survival Rate
Pathology
Hepatoblastoma-fetal pattern
Fibrolamellar Carcinoma
Hepatoblastoma-embryonal
pattern
HCC
Prognosis
Favorable
Unfavaorable
JOURNAL APPRAISAL
CLINICAL QUESTION
Among pediatrics patients with
advanced hepatoblastoma treated with
multimodal combination therapy, what is
the mortality rate?
EDUCATIONAL
PRESCRIPTION
RELEVANCE
Yes.
PRIMARY VALIDITY
GUIDELINES
Was there a representative sample of patients
without the outcome at the start of the observation?
Yes
PRIMARY VALIDITY
GUIDELINES
Was follow up sufficiently long and
complete?
Yes.
PRIMARY VALIDITY
GUIDELINES
Were the criteria for determining the prognostic
factor and outcome explicit and credible (unbiased)?
Yes.
PRIMARY VALIDITY
GUIDELINES
Were there adjustments for other
prognostic factors?
None.
Yes.
Yes.
FINAL RESOLUTION
Patients with epithelial type had poorer prognosis as
compared to patients with mixed type. Also, patients with
stage IV has a higher mortality rate (42.1%) as compared to
stage III (21.4%).
Survival rates of patients with HB treated with chemotherapy,
interventional therapy, surgery and APBSCT have been
significantly improved, with 5-year survival rate of about
70%. Hence, multimodality treatment effectively improved
remission rate and prolonged overall survival.
THANK