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Paediatric oncology


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Paediatric oncology

  1. 1. PAEDIATRICPAEDIATRIC ONCOLOGYONCOLOGY Dr. v veera ratnakar reddyDr. v veera ratnakar reddy Senior resident fmmcSenior resident fmmc
  2. 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.
  4. 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
  5. 5. Benign tumoursBenign tumours  Hemangiomas “port wine stain”Hemangiomas “port wine stain”  Lymphangiomas (cystic hygroma)Lymphangiomas (cystic hygroma)  Sacrococcygeal teratomaSacrococcygeal teratoma  NaeviNaevi
  6. 6. 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
  7. 7. 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
  8. 8. 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
  9. 9. Pediatric Cancer SubtypesPediatric Cancer Subtypes
  10. 10. 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
  11. 11. 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.
  12. 12. ALL-L1 TYPEALL-L1 TYPE
  13. 13. ALL-L2 TYPEALL-L2 TYPE
  14. 14. ALL-L3 TYPEALL-L3 TYPE
  15. 15. 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
  16. 16. 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
  17. 17. Differential diagnosisDifferential diagnosis  Aplastic anemiaAplastic anemia  MyelofibrosisMyelofibrosis  Infections mononucleosisInfections mononucleosis
  18. 18. 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
  19. 19. 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
  20. 20. 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
  21. 21. Treatment:Treatment:  Anthracycline andAnthracycline and  cystosine arabinosidecystosine arabinoside   Antibiotics & antifungalsAntibiotics & antifungals
  22. 22. Hodgkins diseaseHodgkins disease  PathologyPathology  Reed Sternberg cell isReed Sternberg cell is the Hallmark featurethe Hallmark feature
  23. 23. 4 Histologic subtypes:4 Histologic subtypes:  Lymphocyte predominantLymphocyte predominant  Nodular sclerosingNodular sclerosing  Mixed cellularMixed cellular  Lymphocyte depletedLymphocyte depleted
  24. 24. 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
  25. 25. ANN ARBOR STAGINGANN ARBOR STAGING  Stage 1Stage 1 ::Single lymphnode or a single extralymphaticSingle lymphnode or a single extralymphatic site or 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
  26. 26. 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
  27. 27. TreatmentTreatment  MOPP or ABVDMOPP or ABVD  Six cycles minimumSix cycles minimum  Prognosis:Prognosis: 70-90%70-90% 
  28. 28. 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??
  29. 29. Gross appearence of tumour.Gross appearence of tumour.
  30. 30. Microscopy of HPEMicroscopy of HPE
  31. 31. 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
  32. 32. 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.
  33. 33. DiagnosisDiagnosis  CT, MRICT, MRI →→massmass  Tumor markersTumor markers →→ HVA,HVA,  VMAVMA  BiopsyBiopsy
  34. 34. TreatmentTreatment  Surgical excisionSurgical excision  Chemotherapy- Cisplatin, Doxorubicin,Chemotherapy- Cisplatin, Doxorubicin, vincristine, cyclophospamidevincristine, cyclophospamide   RadiotherapyRadiotherapy
  35. 35. Wilm’s tumorWilm’s tumor
  36. 36.  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
  37. 37.  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
  38. 38. Treatment-Treatment-  U/L tumor – surgical removalU/L tumor – surgical removal  Vincristine, actinomycin & doxorubicinVincristine, actinomycin & doxorubicin  RadiotherapyRadiotherapy
  39. 39. 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
  40. 40. Day 3Day 3 5 th day5 th day
  41. 41. Non Hodgkins lymphomaNon Hodgkins lymphoma  3 Histologic subtypes3 Histologic subtypes ––  LymphoblasticLymphoblastic  Large cellLarge cell  Small non cleavedSmall non cleaved
  42. 42. 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
  43. 43. 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
  44. 44. TreatmentTreatment  Surgical excision may precedeSurgical excision may precede  CHOP – 6 cyclesCHOP – 6 cycles  OrOr  3 cycles + 6m – 6mp + mtx3 cycles + 6m – 6mp + mtx
  45. 45. 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?
  46. 46. Morphology of retinoblastomaMorphology of retinoblastoma
  47. 47. Fundus examinationFundus examination
  48. 48. 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.
  49. 49. 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)