肾细胞癌的分类-compressedppt课件.ppt

ClassificationofRenalCarcinoma RenalUnit CK19 THP CD10 RCC UEA 1 RenalCarcinoma ClearCellCarcinoma Granularcellcarcinoma ClearCellCarcinoma ChromophobeCarcinoma PapillaryCarcinomaTypeITypeII Collectingductcarcinoma Oncocytoma MetanephricAdenoma Adenofibroma Unclassifiedandunusualcarcinoma Case1 37yrmale Historyofcerebellarhemangioblastoma renaltumorandcystGross Tumordiameter5X5X2 5cm cutsurfaceyellowSomecystsinthetumor RiskfactorforVonHippelLindausyndromeYoungerthan50yearsMultifocalunilateralorbilateraltumorsFamilyhistoryofrenalcancerorotherVHLrelatedtumorGermlinemutationanalysisMissensemutationofVHLgeneon3p25 26 DiagnosticCriteriaforVHLSyndrome NofamilyhistoryHemangioblastomaofCNSorretina VHLassociatedextraneuraltumorsWithfamilyhistoryHemangioblastomaofCNSorretinaorVHLassociatedextraneuraltumors Case1 DiagnosisVHLassociatedclearcellrenalcellcarcinoma VHLsubtype HB Hemangioblastoma RCC Renalcellcarcinoma PCC Pheochromocytoma Pathogenesis CohenHandMcGovernFJ NEnglJMed 2005 353 23 pVHLintumorinitiation SporadicClearCellCarcinoma Pathogenesis CohenHandMcGovernFJ NEnglJMed 2005 353 23 FuhrmanNuclearGrade 4tiered Note Applyinclearcellcarcinomaandpapillarycarcinoma MultilocularCysticRenalCellCarcinoma MultilocularCysticRenalCellCarcinoma ModernPathol201023 931 936 Mostofthecasesshowedchromosome3pdeletion Verygoodsurvival HereditaryRCC HereditaryleiomyomatosisandRCCBirt Hogg Dub syndromeHyperparathyroidism JawtumorsyndromeFamilialPapillaryThyroidCancerConstitutionalchromosome3translocationsHereditarypapillaryrenalcancer HereditaryleiomyomatosisandRCC LeomyomaorleomyosarcomaofskinoruterusUnilateralpapillaryrenalcarcinomaFumarateHydratase FH mutation1q42 Birt Hogg Dub Syndrome FacialfibrofolliculomaandtricodiscomaLungcystwithriskofspontaneouspneumothoraxColonicpolypsRenaltumors oncocytomaorchromophobecarcinomaFolliculin BHD genemutation17p11 2 Birt Hogg Dub Syndrome Hyperparathyroidism JawTumorSyndrome AutosomaldominantParathyroidadenomas carcinoma Ossifyingfibromaofthemaxillaormandible RenalcystsorrenaltumorWilm stumor mixedepithelial stromaltumor papillarycarcinomaHPT JT parafibromin mutation1q21 q32 JInternMed 2003253 6 634 FamilialPapillaryThyroidCancer FamilialPTCAssociatedwithRenalCellNeoplasia1q21Notc MetassociatedHereditaryrenalpapillarycacinomaNotCowdensyndromeNotMEN2A Case2 45yrmaleRightkidneymass PapillaryRenalCarcinoma Type1PapillaecoveredbysmallcellswithscantycytoplasmSinglelayeronthepapillarybasementmembrane Type2HighernucleargradewitheosinophiliccytoplasmPseudostratifiednucleionpapillarycores Type1 Type2 Case2 DiagnosisPapillaryRenalCellCarcinoma TypeI PrognosisofPRCC Conventionalpapillaryrenalcarcinoma75 withduplicationofchromosome7 proto oncogeneMET CohenHandMcGovernFJ NEnglJMed 2005 353 23 PapillaryRenalCancerinvolvedinHereditarySyndromes HereditarypapillaryrenalcancerHereditaryleiomyomatosisandRCCBirt Hogg Dub syndromeHyperparathyroidism JawtumorsyndromeFamilialPapillaryThyroidCancer PapillaryAdenoma GrignonDJ EbleJN Papillaryandmetanephricadenomasofthekidney SeminDiagnPathol 1998 15 41 53 Definition Tumorswithpapillaryortubulararchitectureoflownuleargradeand5mmindiameterorsmallerPapillaryepithelialneoplasmoflowmalignantpotentialGrade1papillarytumorsbetween0 5 2cmarestrictlydefinedascarcinoma TumorDiametersandMetastasisatAutopsy 871patients GrignonandEble SeminDiagPathol 1998 15 41 ProposedDevelopmentalStepsofPRCC KLWang etal HumPathol200738 3 239 Case3 33yr femaleRightkidneymass 2X1 5X0 5cm surfaceyellow Clear CellPapillaryRenalCellCarcinoma Clear celltubulopapillaryrenalcellcarcinomaRenalangiomyoadenomatoustumorRenalcellcarcinomawithprominentleiomyomatousproliferationClearcellrenalcellcarcinomawithdiffusecytokeratin7immunoreactivity Clear CellPapillaryRenalCellCarcinoma Cytokeratin7 carbonicanhydraseIX CA9 HIF 1 GLUT 1 CD10 P504S TFE3 AbsenceofVHLgenealteration Case4 25yearsoldfemaleCTrevealedamassinrightkidneyNephrectomywasperformed TFE 3 RenalcarcinomasassociatedwithXp11 2translocations PapillaryarchitecturecomprisedofclearcellsNestedarchitectureCellswithvoluminousgranulareosinophiliccytoplasmPsammomabodies Xp11TranslocationCarcinoma Heimannetal Fusionofanovelgene RCC17 totheTFE3gene CancerRes2001 61 4130Arganietal PrimaryrenalneoplasmswithASPL TFE3genefusionofalveolarsoftpartsarcoma AmJSurgPathol2001 159 179Arganietal PRCC TFE3renalcarcinomas AmJSurgPathol2002 26 1553 Case3 DiagnosisRenalcarcinomasassociatedwithXp11 2translocations MicrophthalmiaTranscriptionFactor TFEFamily MiTF TFE3 TFEB TFECHomologousDNAbindingdomainRecognizeidenticalDNAsequencesActivatetranscriptionofsimilargenesinvitro AmJSurgPathol2005 t 6 11 p21 q12 RenalCarcinoma Ages6 53years median18yr MorphologyEpithelioidcells predominantlyclearcytoplasmIHCMinimalcytokeratinsexpressionAllHMB45 Melan A AberrantpositiveofTFEBtranscriptionfactor DiagnosticUseofMiTF TFEFamilyProtein ChromophobeRenalCellCarcinoma LargepalecellswithprominentcellmembranesPerinuclearhalosEosinophilicvariant Mucinoustubularandspindlecellcarcinoma Tightlypacked small elongatedtubulesseparatedbypalemucinousstromaTheparalleltubulararraysoftenhaveaspindlecellconfigurationCuboidalorovalnulearshapesandlow gradefeaturesPositiveforEMA AE1 AE3 CK7 CK19 34 E12andAMACR MTSCC Loopoma CollectingDuctCarcinoma CollectingDuctCarcinoma ArisesintheprincipalcellsofthecollectingductsofBelliniComplextubulopapillaryarchitecturePronouncedstromaldesmoplasiaComplexinteranastomosingstructuresCK19 34bE12 UEA 1 CD10 Villin CK19 LowGradeCollectingDuctCarcinoma Tubulocysticcarcinoma MacLennanGT etal Low gradecollectingductcarcinomaofthekidney reportof13casesoflow grademucinoustubulocysticrenalcarcinomaofpossiblecollectingductorigin Urology1997 50 679 684 AminMB etal Tubulocysticcarcinomaofthekidney Clinicopathologicanalysisof29casesofadistinctiveraresubtypeofrenalcellcarcinoma ModPathol2004 17 137A Tubulocysticcarcinoma Tubulesandcysticstructuresofmarkedlyvariablesize separatedbydelicatesepta spiderweb like TubulesarelinedbyasinglelayeroflowcuboidalepithelialcellswithhobnailappearanceIHCsimilartocollectingductcarcinoma RenalMedullaryCarcinoma StronglyassociatedwithsicklecellhemoglobinopathiesTypicallylocatedintherenalmedullaMayariseintheterminalcollectingductsandtheadjacentpapillaryepithelium ReticularormicrocysticgrowthpatternTubuleformationandgrowthindiffusesheetsorsolidnodulesPleomorphicnuclei prominentnucleoli andeosinophiliccytoplasmIHCpositiveforCEA EMA CK19 topoisomeraseIIa RenalMedullaryCarcinoma AcquiredCysticDisease AssociatedRenalCellCarcinoma End stagerenaldisease ESRD 50 Acqu