1. PAEDIATRICPAEDIATRIC
ONCOLOGYONCOLOGY
Dr. v veera ratnakar reddyDr. v veera ratnakar reddy
Senior resident fmmcSenior resident fmmc

2. Neoplasms of infancy andNeoplasms of infancy and
childhoodchildhood
 Benign>malignantBenign>malignant
 Incidence of malignancy:1-15 yrs - 1.3 /Incidence of malignancy:1-15 yrs - 1.3 /
10,000 /year but leading cause of death10,000 /year but leading cause of death
after accidents in the West.after accidents in the West.
 Most malignant tumours in childrenMost malignant tumours in children
arise from hematopoietic,nervous andarise from hematopoietic,nervous and
soft tissues.soft tissues.

3. PEDIATRIC (VS) ADULTPEDIATRIC (VS) ADULT
NEOPALSMSNEOPALSMS

4. Difference between adult & PaedDifference between adult & Paed
tumourstumours
 Association between abnormalAssociation between abnormal
development (teratogenesis) & tumourdevelopment (teratogenesis) & tumour
induction.induction.
 Prevalence of constitutional geneticPrevalence of constitutional genetic
abnormalities or syndromes thatabnormalities or syndromes that
predispose to cancerpredispose to cancer
 Tendency of malignancy to undergoTendency of malignancy to undergo
differentiationdifferentiation
 Improved survivalImproved survival

6. Benign tumoursBenign tumours
 Hemangiomas “port wine stain”Hemangiomas “port wine stain”
 Lymphangiomas (cystic hygroma)Lymphangiomas (cystic hygroma)
 Sacrococcygeal teratomaSacrococcygeal teratoma
 NaeviNaevi

7. Sacrococcygeal teratomasSacrococcygeal teratomas
 Germ cell neoplasmGerm cell neoplasm
 1:40,000 live births1:40,000 live births
 Mass in the sacrumMass in the sacrum
and buttocksand buttocks
 Composed ofComposed of
elements of > 1 germelements of > 1 germ
cell layer.mixture ofcell layer.mixture of
elements.elements.
 Neural originNeural origin
determines thedetermines the

8. Signs of Childhood CancerSigns of Childhood Cancer
CContinued, unexplained weight lossontinued, unexplained weight loss
HHeadaches, often with early morning vomitingeadaches, often with early morning vomiting
IIncreased swelling or persistent pain in bones, joints, back, or legsncreased swelling or persistent pain in bones, joints, back, or legs
LLump or mass, esp. in the abdomen, neck, chest, pelvis, orump or mass, esp. in the abdomen, neck, chest, pelvis, or armpitsarmpits
DDevelopment of excessive bruising, bleeding, or rashevelopment of excessive bruising, bleeding, or rash
CConstant/recurrent infectionsonstant/recurrent infections
AA whitish color behind the pupilwhitish color behind the pupil
NNausea which persists or vomiting with or w/o seizureausea which persists or vomiting with or w/o seizure
CConstant tiredness or noticeable palenessonstant tiredness or noticeable paleness
EEye or vision changes which occur suddenly and persistsye or vision changes which occur suddenly and persists
RRecurrent or persistent fevers of unknown originecurrent or persistent fevers of unknown origin

9. WHY DIFFICULT TO DIAGNOSEWHY DIFFICULT TO DIAGNOSE
CHILDHOOD CANCER?CHILDHOOD CANCER?
 Vague manifestationsVague manifestations
 Childhood malignancies are rareChildhood malignancies are rare
 Doctor’s reluctance to consider cancerDoctor’s reluctance to consider cancer
diagnosisdiagnosis

10. Pediatric Cancer SubtypesPediatric Cancer Subtypes

11. PREDISPOSING FACTORSPREDISPOSING FACTORS
 GeneticGenetic
 MutationsMutations
 Defects in DNADefects in DNA
 Neurocutaneous disordersNeurocutaneous disorders
 Immuno deficienciesImmuno deficiencies
 Chromosomal abnormalitiesChromosomal abnormalities
 InfectionsInfections
 E.B virusE.B virus HIVHIV
 EnvironmentalEnvironmental
 ChemotherapyChemotherapy
 Ionising radiationIonising radiation
 Electromagnetic radiationElectromagnetic radiation

12. ACUTE LYMPHOBLASTICACUTE LYMPHOBLASTIC
LEUKEMIALEUKEMIA
 Classification:Classification:
 L1 – Predominantly small lymphoblasts with littleL1 – Predominantly small lymphoblasts with little
cytoplasm.cytoplasm.
 L2 – Larger and pleomorphic with moreL2 – Larger and pleomorphic with more
cytoplasm irregular nuclear shapecytoplasm irregular nuclear shape
 with prominent nucleuswith prominent nucleus
 L3 – Have finely stippled and homogenousL3 – Have finely stippled and homogenous
nuclear chromatin, prominent nucleoli deep bluenuclear chromatin, prominent nucleoli deep blue
cytoplasm with prominent vacuolation.cytoplasm with prominent vacuolation.

13. ALL-L1 TYPEALL-L1 TYPE

14. ALL-L2 TYPEALL-L2 TYPE

15. ALL-L3 TYPEALL-L3 TYPE

16. Clinical Features:Clinical Features:
 Initially nonspecific – Anorexia, IrritabilityInitially nonspecific – Anorexia, Irritability
and lethargy.and lethargy.
 Pallor, bleeding, petechiae and feverPallor, bleeding, petechiae and fever
lymphadenopathy, splenomegaly,lymphadenopathy, splenomegaly,
hepatomegaly, bone pain and arthralgiahepatomegaly, bone pain and arthralgia
 Rarely headache and vomitingRarely headache and vomiting

17. DiagnosisDiagnosis
 Anemia, thrombocytopenia,Anemia, thrombocytopenia, ↓↓WBC countWBC count
 Presence of blast cells on peripheralPresence of blast cells on peripheral
smearsmear
 Bone marrow – Leukemic lymphoblastsBone marrow – Leukemic lymphoblasts

18. Differential diagnosisDifferential diagnosis
 Aplastic anemiaAplastic anemia
 MyelofibrosisMyelofibrosis
 Infections mononucleosisInfections mononucleosis

19. TreatmentTreatment
 Remission inductionRemission induction
ContinuationContinuation
 VincristineVincristine
 6 mercaptopurine6 mercaptopurine
 PrednisonePrednisone
 MethotrexateMethotrexate
 AsparginaseAsparginase ReinforcementReinforcement
 Vicristine andVicristine and
prednisoneprednisone
 Prognosis overall cure rate 80%Prognosis overall cure rate 80%
 Chromosomal transactions t(22:9), t (4:10) – poor prognosisChromosomal transactions t(22:9), t (4:10) – poor prognosis

20. Acute myeloid leukemiaAcute myeloid leukemia
 Clinical features:Clinical features:
 Anemia, Thrombocytopenia andAnemia, Thrombocytopenia and
neutropenianeutropenia
 Pallor, fatigue, petichae, epistaxisPallor, fatigue, petichae, epistaxis
 Chloromas may occurChloromas may occur
 Investigation:Investigation:
 25% myeloblasts in bone harrow25% myeloblasts in bone harrow
 F.A.B. Classifications into 7 typesF.A.B. Classifications into 7 types

21. Classification:Classification:
 Myeloblastic, no maturation Mo & M1Myeloblastic, no maturation Mo & M1
 Myeloblastic, some maturation M2Myeloblastic, some maturation M2
 Hypergranular promyelocytic M3Hypergranular promyelocytic M3
 Myelomonocytic M4Myelomonocytic M4
 Monocytic M5Monocytic M5
 Erythroleukemia M6Erythroleukemia M6
 Megakaryocytic M7Megakaryocytic M7

22. Treatment:Treatment:
 Anthracycline andAnthracycline and
 cystosine arabinosidecystosine arabinoside

 Antibiotics & antifungalsAntibiotics & antifungals

23. Hodgkins diseaseHodgkins disease
 PathologyPathology
 Reed Sternberg cell isReed Sternberg cell is
the Hallmark featurethe Hallmark feature

24. 4 Histologic subtypes:4 Histologic subtypes:
 Lymphocyte predominantLymphocyte predominant
 Nodular sclerosingNodular sclerosing
 Mixed cellularMixed cellular
 Lymphocyte depletedLymphocyte depleted

25. Clinical featuresClinical features
 Painless, firm, cervical or supraclavicularPainless, firm, cervical or supraclavicular
adenopathy is the most commonadenopathy is the most common
presenting sign. Rarelypresenting sign. Rarely
hepatosplenomegalyhepatosplenomegaly
 Less commonLess common:: Pruritus, lethargy andPruritus, lethargy and
anorexiaanorexia

26. ANN ARBOR STAGINGANN ARBOR STAGING
 Stage 1Stage 1 ::Single lymphnode or a single extralymphaticSingle lymphnode or a single extralymphatic
site or organ.site or organ.
 Stage 2Stage 2 :: 2 or more lymphoid regions on the same2 or more lymphoid regions on the same
side of diaphragm or localized involvement of a ELS.side of diaphragm or localized involvement of a ELS.
 Stage 3Stage 3 :: Lymphnode on both sides of diaphragm,Lymphnode on both sides of diaphragm,
may be ccompanied by involvement of spleen or ELS.may be ccompanied by involvement of spleen or ELS.
 Stage 4Stage 4 ::Diffuse or disseminated involvementDiffuse or disseminated involvement
 Also stage A & B- Presence of fever, night sweats &Also stage A & B- Presence of fever, night sweats &
weight lossweight loss

27. DiagnosisDiagnosis
 CBCCBC
 Chest & ABD. CTChest & ABD. CT
 ESR, S.ferritinESR, S.ferritin
 Gallium scanGallium scan
 LFTLFT
 Bone marrow biopsyBone marrow biopsy
 Chest X-rayChest X-ray

28. TreatmentTreatment
 MOPP or ABVDMOPP or ABVD
 Six cycles minimumSix cycles minimum
 Prognosis:Prognosis: 70-90%70-90%


29. one year old child brought withone year old child brought with
the swelling in the b/l orbitalthe swelling in the b/l orbital
region with excessive cry. o/eregion with excessive cry. o/e
mass palpable in the rt lumbarmass palpable in the rt lumbar
region extending in to the iliacregion extending in to the iliac
area diagnosis??area diagnosis??

30. Gross appearence of tumour.Gross appearence of tumour.

31. Microscopy of HPEMicroscopy of HPE

32. NeuroblastomaNeuroblastoma
 Most frequent diagnosedMost frequent diagnosed
neoplasm in infantsneoplasm in infants
 C/FC/F- May develop at any site of- May develop at any site of
sympathetic nervous tissuesympathetic nervous tissue
 →→ involvement – hard fixed mass,involvement – hard fixed mass,
may produce spinal or neural rootmay produce spinal or neural root
compression.compression.
 Cervical involvement- Horner’sCervical involvement- Horner’s
syndromesyndrome
 Fever, Irritability, FTI, bone pain,Fever, Irritability, FTI, bone pain,
HypertensionHypertension
 Opsoclonus- MvoclonusOpsoclonus- Mvoclonus

33. Clinical featuresClinical features
 Abdominal mass, feverAbdominal mass, fever
 Blueberry muffinBlueberry muffin
 Wide metastasisWide metastasis
 Secrete catecholaminesSecrete catecholamines
 Vanillylmandelic acid (VMA)/HomovanillicVanillylmandelic acid (VMA)/Homovanillic
acid (HVA) screening.acid (HVA) screening.

34. DiagnosisDiagnosis
 CT, MRICT, MRI →→massmass
 Tumor markersTumor markers →→ HVA,HVA,
 VMAVMA
 BiopsyBiopsy

35. TreatmentTreatment
 Surgical excisionSurgical excision
 Chemotherapy- Cisplatin, Doxorubicin,Chemotherapy- Cisplatin, Doxorubicin,
vincristine, cyclophospamidevincristine, cyclophospamide

 RadiotherapyRadiotherapy

36. Wilm’s tumorWilm’s tumor

37.  Pathology- usually a solitary growth in anyPathology- usually a solitary growth in any
part of either kidneypart of either kidney
 Stage1: Limited to kidneyStage1: Limited to kidney
 Stage2: Beyond kidneyStage2: Beyond kidney
 Stage3: Confined to abdomenStage3: Confined to abdomen
 Stage4: haematogenous extensionStage4: haematogenous extension
 Stage5: B/L rental involvementStage5: B/L rental involvement

38.  C/FC/F
 Abdominal or flank massAbdominal or flank mass
 ½ have½ have →→ abdominal pain orabdominal pain or
vomiting hypertensionvomiting hypertension
 Diagnosis-Diagnosis-
 USG, CTUSG, CT
 Chest X-ray, CTChest X-ray, CT

39. Treatment-Treatment-
 U/L tumor – surgical removalU/L tumor – surgical removal
 Vincristine, actinomycin & doxorubicinVincristine, actinomycin & doxorubicin
 RadiotherapyRadiotherapy

40. 3 ½ yr old female child brought3 ½ yr old female child brought
with c/o sudden onset abdwith c/o sudden onset abd
distension rapidly growing indistension rapidly growing in
size on day 1size on day 1

41. Day 3Day 3 5 th day5 th day

42. Non Hodgkins lymphomaNon Hodgkins lymphoma
 3 Histologic subtypes3 Histologic subtypes ––
 LymphoblasticLymphoblastic
 Large cellLarge cell
 Small non cleavedSmall non cleaved

43. Clinical featuresClinical features
 Lymphoblastic – Intrathoracic tumor- dyspnea,Lymphoblastic – Intrathoracic tumor- dyspnea,
chest pain, dysphagia, pleural effusionchest pain, dysphagia, pleural effusion
 Large cell – many sitesLarge cell – many sites
 SNCL – abdominal tumor – ABD. Pain,SNCL – abdominal tumor – ABD. Pain,
distension, bowel obstruction, intestinaldistension, bowel obstruction, intestinal
bleeding, perforation may involve CNS or bonebleeding, perforation may involve CNS or bone
marrowmarrow
 Also classified into 4 stagesAlso classified into 4 stages

44. InvestigationsInvestigations
 CBC, S.electrolytes, uric acid, CO+, PO4,CBC, S.electrolytes, uric acid, CO+, PO4,
LDHLDH
 LFTLFT
 Spinal fluid cytologySpinal fluid cytology
 Chest X – ray + CTChest X – ray + CT
 Gallium scan ABD X – ray + CTGallium scan ABD X – ray + CT
 Bone marrow aspirate & biopsyBone marrow aspirate & biopsy

45. TreatmentTreatment
 Surgical excision may precedeSurgical excision may precede
 CHOP – 6 cyclesCHOP – 6 cycles
 OrOr
 3 cycles + 6m – 6mp + mtx3 cycles + 6m – 6mp + mtx

46. Case scenerioCase scenerio
 2 yr old child presented with slow growing2 yr old child presented with slow growing
mass started in the lateral canthal regionmass started in the lateral canthal region
n now attained the size as shown in figure.n now attained the size as shown in figure.
Histopathology showing the following .Histopathology showing the following .
Fundus examination shows the following .Fundus examination shows the following .
What might be differential diagnosis?What might be differential diagnosis?

48. Morphology of retinoblastomaMorphology of retinoblastoma

49. Fundus examinationFundus examination

51. RetinoblastomaRetinoblastoma
 C/F- Leukocoria, strabismus, orbital infalmn,C/F- Leukocoria, strabismus, orbital infalmn,
painpain
 DiagnosisDiagnosis →→ Opthalmology – orbital USG andOpthalmology – orbital USG and
CTCT
 Treatment – u/l enucleatonTreatment – u/l enucleaton
 Laser photocoagulation orLaser photocoagulation or
cryotherapycryotherapy
 B/lB/l →→ enucleation of the more several effectedenucleation of the more several effected
eye.eye.

52. Pediatric cancers that merit
genetics evaluation
• Retinoblastoma (RB1)
• Bilateral Wilms Tumor (WT1)
• Adrenocortical carcinoma (TP53)
• Choroid Plexus Tumor (TP53)
• Rhabdomyosarcoma < 3 yo
(TP53)
• Osteosarcoma < 5-10 yo (TP53)
• Medullary thyroid cancer (RET)
• Atypical teratoid and malignant
rhabdoid tumor
(SMARCB1/INI1/SNF5)
• Hepatoblastoma (APC)
• GIST (SDHA,-B,-C,-D)
• Pheochromocytoma /
paraganglioma (VHL, NF1,
RET, SDHA,-B,-C,-D,-AF2)
• Retinal/cerebellar
hemangioblastoma (VHL)
• Endolymphatic sac tumors
(VHL)
• Optic pathway tumor (NF1)
• Acoustic or vestibular
schwannomas (NF2)
• Basal Cell Carcinoma /
Medulloblastoma (PTCH)
• Bilateral Neuroblastoma
(ALK, PHOX2B)