Leukemia staging

Prognostic Factors in Pediatric ALL

Outcome
Risk factor Favorable Unfavorable
Age 1-9 years <1 and ≥10 years
Gender Female Male
Race Caucasian, Asian African, American
Node, liver, and spleen Absent Massive
enlargement
WBC at diagnosis <50x109 ≥50x109
FAB morphologic type L1 L2
DNA Index >1,16 <1,16
Response to induction therapy on No peripheral blasts Peripheral blasts
d28
CNS status CNS 1 CNS 2 or 3
Cytogenetics Hyperploidy, trisomies 4 and 10 Hypodiploidy, t(9;22), t(4;11)
Molecular genetics TEL-AML1 MLL gene rearrangement
Immunophenotype Precursor B-cell T-cell, mature B-cell
Acute Myeloid Leukemia
Genetically defined prognostic groups in pediatric AML
 AML WHO Classification

AML WHO Classification

Copyrights apply
FAB classification
Chronic myeloid leukemia
Criteria for CML Chronic, accelerated, and blast crisis
according to WHO
Hodgkin Lymphoma and
Non-Hodgkin Lymphoma
Lymph Nodes
Hodgkin Stage Description

Lymphoma I Involvement of a single lymph node region or lymphoid structure

(e.g., spleen, thymus, Waldeyer ring)

II Involvement of two or more lymph node regions on the same side

Costwolds Staging of the diaphragm (the mediastinum is a single site)
Classification
III Involvement of lymph node regions or structures on both sides of
diaphragm
1 With or without splenic hillar, celiac, or portal nodes
2 With paraaortic, iliac, mesenteric nodes
IV Involvement of extranodal site(s) beyond that designed “E”

NB: Waldeyer ring:
-palatine tonsil
-lingual tonsil
-nasopharyngeal adenoid
A No B-symptoms
B Fever, drenching, night sweats, weight loss >10% per 6 months
X Bulky disease (>1/3 widening of mediastinum, >10cm maximum
dimension of nodal mass)
E Involvement of a single extranodal site contiguous or proximal to
known nodal site
Hodgkin Risk Groups used in Selected Trials in
Pediatric Hodgkin Lymphoma
Lymphoma

Risk Group Children’s Oncology German Multicenter Stanford/Dana Farber

Group Studies Consortium/St. Jude
Low Risk Stage IA/IIA; Stage IA/IB Stage IA/IIA;
No bulk or ‘E’ IIA No bulk
Intermediate IA; bulk or ‘E’, IIB IA; no bulk
Risk IB, IIA; bulk or ‘E’, IIIA; ‘E’ IB, IIA; no bulk,
IIB, IIA, IVA IIIB IIB, III, IV
High Risk IIIB, IV IIB; ‘E’,
IVB IIIA/B; ‘E’,
IIIB, IVA/B
Non-Hodgkin Histopathologic Categories of Non-Hodgkin
Lymphoma in Children and Adolescents
Lymphoma
WHO Immuno- Clinical Presentation Chromosome
Classification phenotype Translocation
Lymphoblastic T-cell Mediastinal, bone marrow MTS 1/p16ink4a; del TAL 1
Lymphoma Pre B-cell Skin, bone, mediastinal t(1;14)(p34; q11),
t(11; 14)(p12; q11)
Burkitt and Mature B-cell Intra-abdominal (sporadic), jaw t(8; 14)(q24; q32)
Burkitt-like (endemic), head and neck (non-jaw, t(2; 8)(p11; q24)
Lymphomas sporadic), bone marrow, CNS t(8; 22)(q24; 211)
Diffuse large B- Mature B-cell Nodal, abdominal, bone, primary Not well characterized in
cell lymphoma CNS, mediastinal children
Anaplastic large- T-cell or Null-cell Variable, but systemic symptoms T(2; 5)(p23; q35);
cell lymphoma, often prominent Less common variant
systemic translocations involving
AUK
Ket: pemeriksaan kromosom belum dapat dilakukan di RSCM
Non-Hodgkin St. Jude (Murphy) Staging System
Lymphoma
Stage Description
I A single extranodal tumor or single anatomic nodal area with exclusion of mediastinum and
abdomen
II A single or extranodal tumor with regional nodal involvement,
2 or more nodal or extranodal tumors on the same side of the diaphragm, or
a primary gastrointestinal tract tumor (completely resested) with or without regional node
involvement
III 2 or more nodal or extranodal tumors on the opposite site of the diaphragm,
Any primary intrathoracic tumors (mediastinal, pleural, or thymic),
Extensive primary intra-abdomen disease, or
any paraspinal or epidural tumors
IV Involvement of central nervous system and/or bone marrow, regardless of other sites of
involvement
OSTEOSARCOMA
 Histologically classified
3 major type :

• Osteoblastic
• Chondroblastic
• Fibroblastic
Stage Grouping American Joining Committee on
Cancer (AJCC) – TNM Classification and Grading of
Osteosarcoma
STAGE T N M Grade
I A T1 N0 M0 G1,2
B T2 N0 M0 G1,2
II A T1 N0 M0 G3,4
B T2 N0 M0 G3,4
III T3 N0 M0 G3,4
IV A Any T N0 M1a Any G
B Any T N1 Any M Any G
Any T Any N M1b Any G
G1 well differentiated M1a lung
G2 moderate differentiated M1b other distant sites
G3 poorly differentiated
G4 undifferentiated
RHABDOMYOSARCOMA
Histological subtypes
• Embryonal Orbit, head, neck, genitourinary
Infants and young children

• Alveolar
• Pleomorphic Extremities and trunk
Adolesences and adults
Children’s Oncology Group (COG – STS)
Pretreatment Staging System
STAGE T N M Sites of Primary Tumor Tumor Sites
I T1 or T2 N0 or N1 or NX M0 Favorable sites Any size
T1 or T2 Orbit
T1 or T2 Head and neck
T1 or T2 Genitourinary
II T1 or T2 N0 or NX M0 Unfavorable sites a, ≤ 5 cm
T1 or T2 Bladder/Prostate
T1 or T2 Extremity
T1 or T2 Cranial/parameningeal
T1 or T2 Other

M0 absence of metastatic spread N0 absence of nodal spread

M1 presence of metastatic spread beyond the primary N1 presence of nodal spread beyond the primary site
site X unknown N status
STAGE T N M Sites of Primary Tumor Tumor Sites
III T1 or T2 N1 M0 Unfavorable sites a, ≤ 5 cm

T1 or T2 Bladder/Prostate
T1 or T2 Extremity
T1 or T2 N0 or N1 or NX Cranial/parameningeal b, > 5 cm
T1 or T2 Other
IV T1 or T2 N0 or N1 or NX M1 Any site Any size
T1 or T2 All

M0 absence of metastatic spread N0 absence of nodal spread

M1 presence of metastatic spread beyond the primary N1 presence of nodal spread beyond the primary site
site X unknown N status
Hepatoblastoma
EUROPEAN SIOPEL/PRETEXT Presurgical
Staging
STAGE DESCRIPTION
I Tumor involves only one quadrant;
three adjoining quadrants are free of tumor
Standard risk
II Tumor involves two adjoining quadrants;
two adjoining quadrants are free of tumor
III Tumor involves three adjoining quadrants or
two non adjoining quadrants and one quadrant or
two non adjoining quadrants are free of tumor
IV Tumor involves all four quadrants; High risk
there is no quadrant free of tumor
Neuroblastoma
Retinoblastoma
Wilms Tumor
Subtitle
Neuroblastoma
• Suatu tumor embryonal dari sistem saraf simpatis yang berasal dari
primitive neural crest
• Secara histologi terdiri dari dua tipe sel terutama yaitu neuroblast/sel
ganglion dan sel schwann
• Gambaran histologi tipikal pada neuroblastoma undifferentiated adalah
adanya sel bulat kecil, dan blue cell tumor
• Gejala klinik bervariasi tergantung lokasi tumor primernya dan ada
tidaknya metastasis
• Neuroblastoma bisa berasal dari setiap tempat sepanjang sistem saraf
simpatis.
• 65% tumor primer terjadi dalam rongga abdomen.
Sistem Penentuan Stadium (INSS)
International Neuroblastoma Staging System
Stadium 1 Tumor terlokalisasi dengan eksisi luas lengkap, dengan atau tanpa adanya penyakit
residual secara mikroskopik, tidak ada pembesaran KGB ipsilateral dan kontralateral
terhadap tumor secara mikroskopik

Stadium 2A Tumor terlokalisasi dengan eksisi luas tidak lengkap, tidak ada pembesaran KGB yang
ipsilateral dan tidak melekat pada tumor secara mikroskopis

Stadium 2B Tumor terlokalisasi dengan eksisi luas lengkap atau tidak lengkap; didapatkan
pembesaran KGB ipsilateral dan tidak melekat pada tumor, pembesaran KGB
kontralateral harus tidak didapatkan secara mikroskopis

Stadium 3 Tumor unilateral yang tidak dapat dioperasi dan terjadi infiltrasi melewati garis
tengah, dengan/tanpa adanya pembesaran KGB regional atau tumor terlokalisasi
unilateral dengan pembesaran KGB kontralateral regional; atau tumor di garis tengah
dengana adanya perluasan bilateral secara infiltrasi yang tidak dapat dioperasi atau
dengan adanya pembesaran KGB
Sistem Penentuan Stadium (2)

Stadium 4 Tumor primer di bagian manapun dengan penyebaran jauh ke KGB, tulang, sumsum
tulang, hepar, kulir dan atau organ lain

Stadium 4s Tumor primer terlokalisasi (stadium 1, 2a,2b) dengan penyebaran yang terbatas pada
kulit hepar, dan atau sumsum tulang (khusus bayi <1 tahun)
International Neuroblastoma Risk Group
Retinoblastoma
• Tumor yang mengenai syaraf embrionik retina
• Gejala klinis  leukoria, strabismus, mata
merah, nyeri mata, dan penurunan visus.
• Trilateral retinoblastoma  Kombinasi langka
retinoblastoma unilateral atau bilateral disertai
neoplasma intrakranial yang berasal dari
neuroblastik dan biasanya ditemukan di regio
pineal atau suprasellar.
Sistem penentuan stadium
International Classification of Intraocular Retinoblastoma

Group A Small tumors ≤3 mm in size

Group B Large tumors> 3 mm in size or

- Macular RB location (≤ 3 mm to foveola),
- Juxtapapillary RB location (≤ 1,5 mm to disc),
- Clear subretinal fluid ≤ 3 mm fro margin
Group C RB with focal seeds
C1 subretinal seeds ≤3 mm from RB
C2 vitreous seeds ≤3 mm from RB
C3 both subretinal and vitreous seeds ≤3 mm from RB
Group D RB with diffuse seeds
D1 Subretinal seeds >3mm from RB
D2 Vitreous seeds >3mm from RB
D3 Both subretinal and vitreous seeds >3mm from RB
Group E Extensive RB occupying >50% globe or
-neurovascular glaucoma
Opaque media from hemorrhage in anterior chamber,
vitreous, or subretinal space
- Invasion of postlaminar optic nerve, choroid (>2mm),
sclera,orbit, anterior chamber
Sistem Penentuan Stadium (2)
International Retinoblastoma Staging System (IRSS)
Stage 0 Eye has not been removed, patients treated conservatively
Stage 1 Eye enucleated, tumor completely resected on histopathology examination
Stage 2 Eye enucleated, microscopic residual tumor present in the form of
a. Tumor invasion into extrascleral tissue
b. Tumor invasion into cut end of optic nerve
Stadium 3 Regional extension
a. Overt orbital disease
b. Preauricular or cervical lymph node extension
Stadium 4 Metastatic disease
a. Hematogenous metastasis (without CNS involvement)
- Single lesion and multiple lesion
a. CNS extension (with or without any other site of regional metastatic
disease)
- Perichiasmatic lesion, CNS mass, leptomeningeal and CSF disease
Wilms Tumor
• Tumor ganas embrional ginjal yang berasal dari metanefros dan
merupakan tumor ganas ginjal yang terbanyak pada bayi dan anak
• Sekitar 80% terjadi pada anak di bawah 6 tahun dengan puncak
insidens pada umur 2-4 tahun
• Gejala klinis  massa abdomen, (75-90%), hematuri makroskopis,
hipertensi, anemia, penurunan berat badan, infeksi saluran kencing,
demam, dan anoreksia.
Sistem Penentuan Stadium (Menurut National
Wilms Tumor Study)
• Stadium I
• Tumor terbatas di dalam jaringan ginjal tanpa menembus kapsul, tumor ini dapat
direseksi dengan lengkap
• Stadium II
• Tumor menembus kapsul dan meluas masuk ke dalam jaringan ginjal dan sekitar
ginjal yaitu jaringan perirenal, hilus renalis, vena renalis, dan kelenjar limfe para-
aortal. Tumor masih dapat direseksi dengan lengkap.
• Stadium III
• Tumor menyebar ke rongga abdomen misalnya ke hepar, peritoneum, dll
• Stadium IV
• Tumor menyebar secara hematogen ke rongga abdomen, paru-paru, otak, tulang
• Stadium V
• Melibatkan kedua ginjal