恶性嗜铬细胞瘤的治疗

恶性嗜铬细胞瘤的治疗第1页，课件共35页，创作于2023年2月8/15/20231Introduction

ruleof10sforpheochromocytoma(PCC)

10%bilateral10%extra-adrenal10%extra-abdomen10%malignant10%familial10%children10%normalbloodpressure第2页，课件共35页，创作于2023年2月8/15/20232IntroductionThemostfrequentsiteofmetastasesistheskeletonAdditionalsitesareliver,retroperitoneumwithlymphnodes,CNS,pleura,andkidney第3页，课件共35页，创作于2023年2月8/15/20233Malignantvs.BenignCurrently,thereisnoeffectivecureformalignantpheochromocytoma.Therearealsonoreliablehistopathologicalmethodsfordistinguishingbenignfrommalignanttumors.Malignancyrequiresevidenceofmetastasesatnon-chromaffinsitesdistantfromthatoftheprimarytumor.第4页，课件共35页，创作于2023年2月8/15/20234Metastaticdiseaseinpheochromocytomamaybepresentatthetimeofinitialdiagnosisormayonlybecameevidentaftersurgicalremovaloftheprimarytumor,usuallywithin5years,butsometimes16ormoreyearslater.第5页，课件共35页，创作于2023年2月8/15/20235Duetotherarityofthetumor,clinicalstudiesaboutpheochromocytomasufferfromafragmentednatureandusuallyinvolvetoosmallanumberofcasestoreachconclusiveresults.第6页，课件共35页，创作于2023年2月8/15/20236Becausethereiscurrentlynoeffectivecureformalignantpheochromocytoma,mosttreatmentarepalliative,butinsomecasesmayreducetumorburdenandprolongsurvival.Withouttreatment,the5-yearsurvivalisgenerallylessthan50%.Thecourse,however,canbehighlyvariablewithoccasionalpatientslivingmorethan20yearsafterdiagnosis.第7页，课件共35页，创作于2023年2月8/15/20237Oncemalignancyisdiagnosed,therapyisgenerallydirectedatcontrollingbloodpressure,butmayalsoincludetumordebulking.第8页，课件共35页，创作于2023年2月8/15/20238AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization第9页，课件共35页，创作于2023年2月8/15/20239AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization第10页，课件共35页，创作于2023年2月8/15/202310PrimarysurgicalresectionisthetreatmentofchoicewheneverpossibleLimiteddisease:curativeintentionExtendeddisease:stilltobeconsideredinthefirstplacefordebulkingandaspalliativetreatment(Mundschenketal.1998)第11页，课件共35页，创作于2023年2月8/15/202311ProblemWhensignsofregionalinvolvementordistantdiseaseareabsent,thereiscurrentlynoreliablepreoperativediagnostictestthatcandifferentiatebetweenmalignantandbenignpheochromocytomasShouldpheochromocytomasizeinfluencesurgicalapproach?第12页，课件共35页，创作于2023年2月8/15/202312Acomparisonof90malignantand60benignpheochromocytomas

(WenT.Shenetal.2004)ComparisonoftumorsizeforbenignpheochromocytomasandmalignantpheochromocytomaswithlocaldiseaseonlySizedoesnotreliablypredictmalignancyinpheochromocytomaswithlocaldiseaseonly第13页，课件共35页，创作于2023年2月8/15/202313Malignant(n

=

29)Benign(n

=

55)Tumorsize(mean±SD)6.1±3.1cm5.3±2.3cm<2cm012.0-3.9cm9104.0-5.9cm6256.0-7.9cm5138.0-9.9cm53≥10cm43第14页，课件共35页，创作于2023年2月8/15/202314MalignantPCCspresentingwithonlylocaldiseasecannotbediscriminatedfrombenignPCCsbysizealone.WhenPCCsdonothaveevidenceofinvasionordistantmetastasesandthesurgeonacquiresanappropriatelevelofexperience,themajorityofthesetumorscanbesafelyresectedlaparoscopically.第15页，课件共35页，创作于2023年2月8/15/202315Laparoscopicadrenalectomyforpheochromocytomashouldbeconvertedtoopenadrenalectomyfordifficultdissection,invasion,adhesions,orsurgeoninexperience第16页，课件共35页，创作于2023年2月8/15/202316SurgicalapproachTransabdominalapproachisnecessaryminimallyinvasiveproceduresretroperitonealapproachesshouldbeabandonedtodefinitelypreservethetumorcapsuleandperformtotallymphadecectomy(Orchardetal.1993)第17页，课件共35页，创作于2023年2月8/15/202317SecondaryTumorsNoexperiencewithadjuvantpreandpostoperativeradiationexistsGenerallyaremultipleRadicalsurgicalresectionisoftenimpossibleOthertreatmentmodalitieshavetobeconsidered第18页，课件共35页，创作于2023年2月8/15/202318AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization第19页，课件共35页，创作于2023年2月8/15/202319第20页，课件共35页，创作于2023年2月8/15/202320131I-MIBGisthetreatmentofchoiceforallunresectable,MIBGpositivetumors58casesofmalignantPCCtreatedby131I-MIBG—therapeuticresultsandadverseevents(ZHURuisenetal.1999)第21页，课件共35页，创作于2023年2月8/15/202321Patientswereclassifiedinto3groupsaccordingtotheirtumorsize<8cm3(11cases),8～20cm3(21cases),>20cm3(26cases)Ingroup1,themeanabsorptiondosepergramoftumorwasabove1000cGy.Aftertreatment,tumorsdisappearedorshrinkedinallpatients第22页，课件共35页，创作于2023年2月8/15/202322Ingroup2,theabsorptiondosewassimilartothatofgroup1,butthemeanabsorptiondosepergramwas717.6cGy,andtumormassregressionwas36%;76%reducedurinarycatecholamineIngroup3,theabsorptiondosepergramtumortissuewas277cGy,and30%tumorenlargement,20%died;theremaining50%symptomaticimprovementwithoutanychangeintumorsize第23页，课件共35页，创作于2023年2月8/15/202323131I-MIBGisofcertaintherapeuticeffectivenessofsymptomaticimprovementCompletetumormassdisappearancehasonlybeenfoundinsmalltumorsTreatmentwith131I-MIBGshouldbeinstitutedimmediatelyaftersurgicalresectiontoeradicatetheresidualtumorcellsandtopreventrecurrencesBonemarrowsuppressionistemporaryandnotdosagerelated第24页，课件共35页，创作于2023年2月8/15/202324In1997,Lohetal.publishedareviewoftheworldwideexperienceinvolving116patientstreatedwith131I-MIBGformalignantpheochromocytoma.Overall,therewasasymptomaticresponsein76%,ahormonalresponsein45%,andtumorregressionin30%.Theactivityof131I-MIBGpersingledosewas96–300mCi,andthemeancumulativeactivitywas490±350mCi.OnlyfiveCRsto131I-MIBGwerereported.第25页，课件共35页，创作于2023年2月8/15/202325LimitationsNotallpatientswithmultiplemetastasesofmalignantpheochromocytomashavesufficientuptakeofMIBGtoallowMIBGtherapyMIBGnegativelesionscoexistwithMIBGpostivelesions,requiringcombinedtreatment第26页，课件共35页，创作于2023年2月8/15/202326Asasingleagent,131I-MIBGhaslimitedefficacyintreatingmalignantpheochromocytoma.Itsuseincombinationwithothercytotoxicagents,asiscurrentlybeingstudiedinpatientswithneuroblastoma,mayresultinadditionalbenefit(Sissonetal.1999)第27页，课件共35页，创作于2023年2月8/15/202327AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization第28页，课件共35页，创作于2023年2月8/15/202328Onlysparsedataonchemotherapeuticregimensareavailable,mostoftheminreportsoffewcasesThemostwell-establishedregimenisCVD(Averbuchetal.1988)CTX750mg/m2d1,VCR1.4mg/m2d1,Dacarbazine600mg/m2d1,2Cycle21days第29页，课件共35页，创作于2023年2月8/15/202329TheCVDregimenwasbasedonthetreatmentforadvancedneuroblastoma.Thisregimenhasbeenreportedtoproducegoodresponsesinmalignantpheochromocytoma,butthemediandurationofremissionis21monthsCompletelong-termdiseaseremissionswithchemotherapyhavenotbeenreported.第30页，课件共35页，创作于2023年2月8/15/202330AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCom