Paediatric oncology
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PAEDIATRIC PAEDIATRIC ONCOLOGYONCOLOGY
Dr. v veera ratnakar reddyDr. v veera ratnakar reddySenior resident fmmcSenior resident fmmc
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Neoplasms of infancy and Neoplasms of infancy and childhoodchildhood
Benign>malignant Benign>malignant Incidence of malignancy:1-15 yrs - Incidence of malignancy:1-15 yrs -
1.3 /10,000 /year but leading 1.3 /10,000 /year but leading cause of death after accidents in cause of death after accidents in the West.the West.
Most malignant tumours in Most malignant tumours in children arise from children arise from hematopoietic,nervous and soft hematopoietic,nervous and soft tissues.tissues.
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PEDIATRIC (VS) ADULT PEDIATRIC (VS) ADULT NEOPALSMSNEOPALSMS
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Difference between adult & Difference between adult & Paed tumoursPaed tumours
Association between abnormal Association between abnormal development (teratogenesis) & development (teratogenesis) & tumour induction.tumour induction.
Prevalence of constitutional genetic Prevalence of constitutional genetic abnormalities or syndromes that abnormalities or syndromes that predispose to cancerpredispose to cancer
Tendency of malignancy to undergo Tendency of malignancy to undergo differentiationdifferentiation
Improved survival Improved survival
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Benign tumoursBenign tumours
Hemangiomas “port wine stain”Hemangiomas “port wine stain” Lymphangiomas (cystic hygroma)Lymphangiomas (cystic hygroma) Sacrococcygeal teratomaSacrococcygeal teratoma NaeviNaevi
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Sacrococcygeal teratomasSacrococcygeal teratomas
Germ cell neoplasmGerm cell neoplasm 1:40,000 live births1:40,000 live births Mass in the sacrum and Mass in the sacrum and
buttocksbuttocks Composed of elements of Composed of elements of
> 1 germ cell > 1 germ cell layer.mixture of layer.mixture of elements.elements.
Neural origin determines Neural origin determines the behaviourthe behaviour
< 2 months-benign.< 2 months-benign.
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Signs of Childhood CancerSigns of Childhood Cancer
CContinued, unexplained weight lossontinued, unexplained weight lossHHeadaches, often with early morning vomitingeadaches, often with early morning vomitingIIncreased swelling or persistent pain in bones, joints, back, or ncreased swelling or persistent pain in bones, joints, back, or legslegsLLump or mass, esp. in the abdomen, neck, chest, pelvis, or ump or mass, esp. in the abdomen, neck, chest, pelvis, or
armpitsarmpitsDDevelopment of excessive bruising, bleeding, or rashevelopment of excessive bruising, bleeding, or rash
CConstant/recurrent infectionsonstant/recurrent infectionsAA whitish color behind the pupil whitish color behind the pupilNNausea 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 palenessEEye or vision changes which occur suddenly and persistsye or vision changes which occur suddenly and persistsRRecurrent or persistent fevers of unknown originecurrent or persistent fevers of unknown origin
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WHY DIFFICULT TO DIAGNOSE WHY DIFFICULT TO DIAGNOSE CHILDHOOD CANCER?CHILDHOOD CANCER?
Vague manifestationsVague manifestations
Childhood malignancies are rareChildhood malignancies are rare
Doctor’s reluctance to consider Doctor’s reluctance to consider cancer diagnosiscancer diagnosis
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Pediatric Cancer SubtypesPediatric Cancer Subtypes
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PREDISPOSING FACTORSPREDISPOSING FACTORS GeneticGenetic MutationsMutations Defects in DNADefects in DNA Neurocutaneous disordersNeurocutaneous disorders Immuno deficienciesImmuno deficiencies Chromosomal abnormalitiesChromosomal abnormalities Infections Infections E.B virus E.B virus HIVHIV Environmental Environmental ChemotherapyChemotherapy Ionising radiationIonising radiation Electromagnetic radiationElectromagnetic radiation
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ACUTE LYMPHOBLASTIC ACUTE LYMPHOBLASTIC LEUKEMIALEUKEMIA
Classification:Classification: L1 – Predominantly small lymphoblasts L1 – Predominantly small lymphoblasts
with little cytoplasm.with little cytoplasm. L2 – Larger and pleomorphic with more L2 – Larger and pleomorphic with more
cytoplasm irregular nuclear shape cytoplasm irregular nuclear shape with prominent nucleus with prominent nucleus L3 – Have finely stippled and homogenous L3 – Have finely stippled and homogenous
nuclear chromatin, prominent nucleoli nuclear chromatin, prominent nucleoli deep blue cytoplasm with prominent deep blue cytoplasm with prominent vacuolation.vacuolation.
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ALL-L1 TYPEALL-L1 TYPE
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ALL-L2 TYPEALL-L2 TYPE
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ALL-L3 TYPEALL-L3 TYPE
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Clinical Features:Clinical Features:
Initially nonspecific – Anorexia, Initially nonspecific – Anorexia, Irritability and lethargy.Irritability and lethargy.
Pallor, bleeding, petechiae and Pallor, bleeding, petechiae and fever lymphadenopathy, fever lymphadenopathy, splenomegaly, hepatomegaly, bone splenomegaly, hepatomegaly, bone pain and arthralgia pain and arthralgia
Rarely headache and vomitingRarely headache and vomiting
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DiagnosisDiagnosis
Anemia, thrombocytopenia, Anemia, thrombocytopenia, WBC WBC count count
Presence of blast cells on peripheral Presence of blast cells on peripheral smear smear
Bone marrow – Leukemic Bone marrow – Leukemic lymphoblastslymphoblasts
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Differential diagnosisDifferential diagnosis
Aplastic anemiaAplastic anemia MyelofibrosisMyelofibrosis Infections mononucleosis Infections mononucleosis
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TreatmentTreatment
Remission inductionRemission inductionContinuationContinuation
VincristineVincristine 6 6
mercaptopurine mercaptopurine PrednisonePrednisone MethotrexateMethotrexate Asparginase Asparginase ReinforcementReinforcement Vicristine and Vicristine and
prednisoneprednisone Prognosis overall cure rate 80% Prognosis overall cure rate 80% Chromosomal transactions t(22:9), t (4:10) – poor Chromosomal transactions t(22:9), t (4:10) – poor
prognosisprognosis
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Acute myeloid leukemiaAcute myeloid leukemia
Clinical features:Clinical features: Anemia, Thrombocytopenia and Anemia, Thrombocytopenia and
neutropenianeutropenia Pallor, fatigue, petichae, epistaxisPallor, fatigue, petichae, epistaxis Chloromas may occurChloromas may occur Investigation:Investigation:
25% myeloblasts in bone harrow 25% myeloblasts in bone harrow F.A.B. Classifications into 7 typesF.A.B. Classifications into 7 types
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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
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Treatment:Treatment:
Anthracycline and Anthracycline and
cystosine arabinosidecystosine arabinoside Antibiotics & antifungalsAntibiotics & antifungals
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Hodgkins diseaseHodgkins disease
PathologyPathology Reed Sternberg Reed Sternberg
cell is the Hallmark cell is the Hallmark featurefeature
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4 Histologic subtypes:4 Histologic subtypes:
Lymphocyte predominantLymphocyte predominant
Nodular sclerosing Nodular sclerosing
Mixed cellularMixed cellular
Lymphocyte depletedLymphocyte depleted
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Clinical featuresClinical features
Painless, firm, cervical or Painless, firm, cervical or supraclavicular adenopathy is the supraclavicular adenopathy is the most common presenting sign. most common presenting sign. Rarely hepatosplenomegalyRarely hepatosplenomegaly
Less commonLess common: : Pruritus, lethargy and Pruritus, lethargy and anorexia anorexia
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ANN ARBOR STAGINGANN ARBOR STAGING
Stage 1Stage 1 ::Single lymphnode or a single Single lymphnode or a single extralymphatic site or organ.extralymphatic site or organ.
Stage 2Stage 2 : : 2 or more lymphoid regions on the 2 or more lymphoid regions on the same side of diaphragm or localized involvement same side of diaphragm or localized involvement of a ELS.of a ELS.
Stage 3Stage 3 : : Lymphnode on both sides of Lymphnode on both sides of diaphragm, may be ccompanied by involvement diaphragm, may be ccompanied by involvement of spleen or ELS.of spleen or ELS.
Stage 4Stage 4 ::Diffuse or disseminated involvementDiffuse or disseminated involvement Also stage A & B- Presence of fever, night Also stage A & B- Presence of fever, night
sweats & weight loss sweats & weight loss
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DiagnosisDiagnosis
CBC CBC Chest & ABD. CTChest & ABD. CT ESR, S.ferritin ESR, S.ferritin Gallium scan Gallium scan LFTLFT Bone marrow biopsy Bone marrow biopsy Chest X-rayChest X-ray
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TreatmentTreatment
MOPP or ABVDMOPP or ABVD
Six cycles minimumSix cycles minimum
Prognosis:Prognosis: 70-90%70-90%
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one year old child brought one year old child brought with the swelling in the b/l with the swelling in the b/l
orbital region with orbital region with excessive cry. o/e mass excessive cry. o/e mass palpable in the rt lumbar palpable in the rt lumbar region extending in to the region extending in to the
iliac area diagnosis??iliac area diagnosis??
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Gross appearence of tumour.Gross appearence of tumour.
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Microscopy of HPEMicroscopy of HPE
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NeuroblastomaNeuroblastoma Most frequent diagnosed Most frequent diagnosed
neoplasm in infants neoplasm in infants C/FC/F- May develop at any site - May develop at any site
of sympathetic nervous tissueof sympathetic nervous tissue involvement – hard fixed involvement – hard fixed
mass, may produce spinal or mass, may produce spinal or neural root compression.neural root compression.
Cervical involvement- Cervical involvement- Horner’s syndromeHorner’s syndrome
Fever, Irritability, FTI, bone Fever, Irritability, FTI, bone pain, Hypertensionpain, Hypertension
Opsoclonus- MvoclonusOpsoclonus- Mvoclonus
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Clinical featuresClinical features
Abdominal mass, feverAbdominal mass, fever Blueberry muffinBlueberry muffin Wide metastasisWide metastasis Secrete catecholaminesSecrete catecholamines Vanillylmandelic acid Vanillylmandelic acid
(VMA)/Homovanillic acid (HVA) (VMA)/Homovanillic acid (HVA) screening.screening.
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DiagnosisDiagnosis CT, MRI CT, MRI massmass
Tumor markers Tumor markers HVA, HVA, VMAVMA
BiopsyBiopsy
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TreatmentTreatment
Surgical excisionSurgical excision
Chemotherapy- Cisplatin, Chemotherapy- Cisplatin, Doxorubicin, vincristine, Doxorubicin, vincristine, cyclophospamidecyclophospamide
RadiotherapyRadiotherapy
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Wilm’s tumorWilm’s tumor
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Pathology- usually a solitary growth Pathology- usually a solitary growth in any part of either kidneyin any part 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 involvement Stage5: B/L rental involvement
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C/FC/F Abdominal or flank massAbdominal or flank mass ½ have ½ have abdominal pain abdominal pain
or vomiting hypertensionor vomiting hypertension
Diagnosis-Diagnosis- USG, CTUSG, CT Chest X-ray, CTChest X-ray, CT
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Treatment- Treatment-
U/L tumor – surgical removalU/L tumor – surgical removal
Vincristine, actinomycin & Vincristine, actinomycin & doxorubicindoxorubicin
Radiotherapy Radiotherapy
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3 ½ yr old female child 3 ½ yr old female child brought with c/o sudden brought with c/o sudden
onset abd distension rapidly onset abd distension rapidly growing in size on day 1 growing in size on day 1
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Day 3Day 3 5 th day5 th day
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Non Hodgkins lymphoma Non Hodgkins lymphoma
3 Histologic subtypes 3 Histologic subtypes –– LymphoblasticLymphoblastic
Large cellLarge cell
Small non cleavedSmall non cleaved
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Clinical featuresClinical features
Lymphoblastic – Intrathoracic tumor- Lymphoblastic – Intrathoracic tumor- dyspnea, chest pain, dysphagia, pleural dyspnea, chest pain, dysphagia, pleural effusioneffusion
Large cell – many sitesLarge cell – many sites SNCL – abdominal tumor – ABD. Pain, SNCL – abdominal tumor – ABD. Pain,
distension, bowel obstruction, intestinal distension, bowel obstruction, intestinal bleeding, perforation may involve CNS or bleeding, perforation may involve CNS or bone marrowbone marrow
Also classified into 4 stagesAlso classified into 4 stages
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InvestigationsInvestigations
CBC, S.electrolytes, uric acid, CO+, CBC, S.electrolytes, uric acid, CO+, PO4, LDHPO4, LDH
LFT LFT Spinal fluid cytologySpinal fluid cytology Chest X – ray + CT Chest X – ray + CT Gallium scan ABD X – ray + CT Gallium scan ABD X – ray + CT Bone marrow aspirate & biopsyBone marrow aspirate & biopsy
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TreatmentTreatment
Surgical excision may precedeSurgical excision may precede
CHOP – 6 cyclesCHOP – 6 cycles
OrOr
3 cycles + 6m – 6mp + mtx3 cycles + 6m – 6mp + mtx
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Case scenerio Case scenerio 2 yr old child presented with slow 2 yr old child presented with slow
growing mass started in the lateral growing mass started in the lateral canthal region n now attained the canthal region n now attained the size as shown in figure. size as shown in figure. Histopathology showing the Histopathology showing the following . Fundus examination following . Fundus examination shows the following . What might be shows the following . What might be differential diagnosis?differential diagnosis?
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Morphology of Morphology of retinoblastomaretinoblastoma
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Fundus examination Fundus examination
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RetinoblastomaRetinoblastoma
C/F- Leukocoria, strabismus, orbital C/F- Leukocoria, strabismus, orbital infalmn, paininfalmn, pain
Diagnosis Diagnosis Opthalmology – orbital USG Opthalmology – orbital USG and CTand CT
Treatment – u/l enucleatonTreatment – u/l enucleaton Laser photocoagulation or Laser photocoagulation or
cryotherapycryotherapy B/l B/l enucleation of the more several enucleation of the more several
effected eye.effected eye.
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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)