<prompt> A 4 years old girl presented with abdominal lump. Bone scan is needed in:Wilms tumorNeuroblastomaRhabdomyosarcomaPNET  <response>: Ans. b. Neuroblastoma Ref: Schwartz 9e p1449-1450; Sabiston 19e p1859-1861; Bailey 25e p814-815; Harrison 19e 10 e-2t, 101e-3, 18e p836; Ghai 7e p590-591A 4 years old girl presented with abdominal lump. Bone scan is needed in Neuroblastoma as metastasis is present in 60- 70 of patients at the time of diagnosisQ and MC site of metastasis in older children are bonesQ.Neuroblastoma:MC presentation: Fixed, lobular mass extending from the flank toward the midlineQ of the abdomen.Most 80 cases present before 4 years and peak incidence is 2 yearsQ of age.Metastasis is present in 60-70 of patients at the time of diagnosisQMC site of metastasis in older children are bonesQ long bones facial bones, skull particularly sphenoid, bone marrow and LN. NeuroblastomaArise from neural crestQ and may originate anywhere along the distribution of sympathetic chainMC tumor diagnosed in infants 1 year of ageQMC intra-abdominal malignancy in childrenQSporadic in majority of casesMC site: Adrenal 30Q Paravertebral reteroperitoneumQ 28 Posterior mediastinumQ 15 PeIvis 5 Cervical areaQAssociated with neurofibromatosis, Hirschprungs disease, heterochromia, fetal hydantoin, fetal alcohol syndrome and Freidreichs ataxiaSpontaneous regression is unique behaviour especially in stage 4SQ.Pathology:Classic neuroblastomas: Small, primitive-appearing cells with dark nuclei, scant cytoplasmMitotic activity, nuclear breakdown karyorrhexisQ, and pleomorphism maybe prominent.Homer-Wright pseudo-rosettesQ can be foundImmunochemical detection of neuron-specific enolaseQClinical Features:MC presentation: Fixed, lobular mass extending from the flank toward the midlineQ of the abdomen.Most 80 cases present before 4 years and peak incidence is 2 yearsQ of age.Metastasis is present in 60-70 of patients at the time of diagnosisQMC site of metastasis in older children are bonesQ long bones facial bones, skull particularly sphenoid, bone marrow and LN.In infants metastasis is confined to liver or subcutaneous tissueQ.Lung metastasis are rareQ in neuroblastomaOrbital metastasis commonly present with peri-orbital ecchymoses and proptosis called as Raccoon eyesQ.Infants with stage 4S may display cutaneous metastasis called as blueberry muffin lesionsQ.Chronic watery diarrheaQ due to secretion of VIP and opsoclonus-myoclonusQ Dancing eyes, dancing feetQ are unusual paraneoplstic manifestations.Diagnosis:Anemia, thrombocytopenia or thrombocytosis more commonIncreased LDH, ferritin, urinary catecholamines and neuron specific enolaseX-ray or CT: Stippled calcificationQ MC abdominal tumor to demonstrate calcification prior to chemotherapyDrooping Lily sign: Neuroblastoma displaces kidney inferolaterallyQMRIQ is superior to CT in assessing vessel encasement, vessel patency, spinal cord compression and bone marrow involvement.MIBG scan or SRS are used in the diagnosis of primary, residual and metastatic neuroblastoma.MIBG is one of the single best studies to document the presence of metastaticQ disease.Appearance of neuroblastoma in bone marrow may simulate the appearance of ALLQ, differentiation can be done by monoclonal antibody phenotypeQ.Treatment:Localized neuroblastoma: ExcisionQUnresectable tumor: Biopsy, initially treated by chemotherapy and radiotherapy followed by surgical resection of residual tumorQDisseminated disease: ChemotherapyQ Cyclophosphamide, vincristine, dacarbazine, doxorubicin, CisplatinPrognosis:Shimada classificationQ describes prognosis based on the degree of differentiation, mitosis-karyorrhexis index, presence or absence of schwannian stromaQ.Prognostic Factors in NeuroblastomasVariableFavorableUnfavorableStageStage 1, 2A, 2B, 4SQStage 3, 4QAge18Q months18 monthsEvidence of schwannian stroma and gangliocytic differentiationPresentQAbsentMitosis-karyorrhexis index2005000 cells2005000 cellsDNA ploidyHyperdiploid or near-triploidQNear-diploidN-MYCNot amplifiedAmplifiedQChromosome 17q gainAbsentPresentQChromosome 1p lossAbsentPresentQChromosome 11q lossAbsentPresentQTRKA expressionPresentQAbsentTRKB expressionAbsentPresentQTelomerase expressionLow or absentHighly expressedQDevita 9e p1766-1769; Nelson 18e p2140; Smith 17e p339-343; Campbell 10e p3714-3722; Bailey 25e p1308Wilms TumorWilms tumor: MC primary renal tumor of childhood 2-5 yearsQ.Wilms tumor: 2nd MC malignant abdominal tumor in children MC is neuroblastoma.Arise from kidney, composed of three elements- blastema, epithelium and stromaQ.MC presenting feature is asymptomatic abdominal mass or swellingQ.Mostly unilateral.Characterized by triad of abdominal mass, fever and microscopic hematuriaQ.Fever typically resolve after tumor resectionMC site of metastasis: LungsAssociated malformationsWAGR SyndromeQ: It consists of aniridia, genital anomalies and mental retardation. The risk of Wilms tumor is increased by 33 in this syndromeQ.Denys-Drash SyndromeQ: It consists of gonadal dysgenesis Male pseudohermaphroditism, nephropathy leading to renal failure. Majority of patients with this syndrome have renal failure.Beckwith-Wiedmann SyndromeQ: It consists of enlargement of body organs, hemi-hypertrophy, renal medullary cysts and abnormal large cells in adrenal cortex, macroglossia, omphalocele.Diagnosis:USG most important investigationQ or CT abdomen for staging.MRI is superior to other imaging modalities in delineating nephroblastomatosis elements.Calcification tends to be more crescent shaped, discrete and peripheralQ in comparison of finely stippled calcification of neuroblastoma.Treatment:Surgical excision is treatment of choice.Routine exploration of contralateral kidney is not necessary if imaging is satisfactory and does not suggest bilateral process.In unfavorable histology, Radiation therapy should be started within 10 daysQ after nephrectomy, chemotherapy should be started 5 days after surgeryQ.Chemotherapy: VCD Vincristine Cyclophosphamide Doxorubicin or dactinomycinWhole lung irradiation is recommended for pulmonary metastasis.Preoperative treatment should be consideredSolitary kidneyQBilateralQ renal tumorsTumor in horse shoe kidneyQTumor thrombus in IVC above the level of hepatic veinsQRespiratory distress due to metastaticQ diseasePrognosis:The histologyQ of Wilms tumor and tumor stage is identified as most important determinant of prognosisQ Histology Stage.Pediatric Tumors MC malignant tumor of infancy MC extracranial solid tumor in children MC abdominal malignancy in childrenNeuroblastoma MC primary malignant renal tumor of childhoodWilms tumor MC renal tumor of infancyCongenital mesoblastic nephroma MC soft tissue tumor in infants and childrenRhabdomyosarcoma MC solid tumor of childhoodBrain tumor MC cancer of childhoodLeukemia 30 Brain tumors 22 