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cellsarcomaClearOfkidney1cellsarcomaClearOfkidney1CONTENTS1Introduction2Clinicalpresentation3CTfindings4Differentialdiagnosis5Therapyandprognosis2CONTENTS1Introduction2Clin1Introduction

Clearcellsarcomaofthekidney(CCSK)isanuncommonrenalneoplasmofchildhood.Itaccountsfor2–5%ofallpediatricrenalmalignancies31Introduction3IntroductionItisknownforitsmorphologicdiversity,aggressivebehaviour,tendencytorecurandmetastasistoboneCCSKisthesecondmostcommonrenaltumorinchildrenwithameanageofdiagnosisoverlappingwithWilmstumor4IntroductionItisknownforitIntroductionBonemetastasesoccurin40to60%ofpatientswithCCSK,unlikelessthan2%ofpatientswithWilms’tumorCCSKwasfirstdescribedas“bonemetastasizingrenaltumorofchildhood”sinceithaspredilectionforskeletalmetastasis5IntroductionBonemetastasesocThepeakincidenceisbetween3and5yearsofage.Itisveryrareininfantsyoungerthan6monthsIntroduction

Itisthemostfrequentlymisdiagnosedrenaltumorinchildren,asitisunusual,hasvariedmorphology,andtherearenospecificdiagnosticmarkers6Thepeakincidenceisbetween2ClinicalpresentationMostofthechildrenweretakentothehospitalforabdominalmass72ClinicalpresentationMostof01abdominalpain03hematuria02abdominaldistentionClinicalpresentation801abdominalpain03hematuria02a3CTfindingsheterogeneousenhancementwithattenuationlessthanthatofnormalrenalparenchymacontainareasoflowattenuationcorrespondingtonecrosisandcystscysticfoci93CTfindings9CTfindingsAxialandcoronalimageshowalargerighthypochondrialsofttissuemass,almosttotallyinfiltratingtherightkidneywithonlyalittleresidualnormalrenalparenchyma2.10CTfindingsAxialandcoronaliCTfindingsSolidcomponentpresentedheterogeneousenhancement

withalargecysticareaandshowedtumorvesselswithhighdensity11CTfindingsSolidcomponentpreDiagnosisCTfindingsRenalclearcellcarcinoma12DiagnosisCTfindingsRenalclea4Differentialdiagnosis1.Wilmstumor2.Malignantrhabdoidtumor3.Renalcellcarcinoma4.Lymphoma134Differentialdiagnosis1.WilmsWilmstumor01.heterogeneoussolidrenalmasswitha“clawsign”02.tumorextensionintotherenalveinandIVCin5-10%03.mostfrequentlymetastasizestothelungs,bilateralin4-13%Malignantrhabdoidtumor(MRT)01.diagnosedbetween0-3yearsofage02.extensivemetastasisatthetimeofdiagnosis03.synchronousbraintumor21Differentialdiagnosis14WilmstumorMalignantrhabdoidLymphomamultiplebilaterallow-attenuationmassesunilateralsolitarylow-attenuationmass,largeconglomeratemasses,orunilateralnephromegalylymphadenopathyelsewhereRenalcellcarcinomamakesup1%ofallpediatricrenalmalignanciessmalleratpresentationcalcifiedin25%43Differentialdiagnosis15LymphomaRenalcellcarcinoma5TherapyandprognosisForsuspiciousrenallesions,itiscrucialtoensurepromptdiagnosisinordertoselecttheappropriatesurgicalprocedureandtreatment165TherapyandprognosisForsuspTherapyandprognosisTherapyradicalnephrectomychemotherapyradiotherapyWhileprognosistraditionallyhasbeenpoor,newertreatmentregimensshowrelapse-freecancer-specificsurvivalrateof100%forrevisedstage1CCSKandoverallsurvivalrateforpatientswithCCSKof83%prognosis17TherapyandprognosisTherapyraTherapyandprognosisToconcludeCCSKisafrequentlymisdiagnosedtumorduetotremendousmorphologicdiversitywhereintheprimarycontributionofIHC

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