【文档说明】恶性嗜铬细胞瘤的治疗课件.ppt，共(35)页，266.500 KB，由小橙橙上传

转载请保留链接：https://www.ichengzhen.cn/view-247382.html

以下为本文档部分文字说明：

TherapyofMalignantPheochromocytoma恶性嗜铬细胞瘤的治疗LiteratureReport2023/4/282Introductionruleof10sforpheochromoc

ytoma(PCC)10%bilateral10%extra-adrenal10%extra-abdomen10%malignant10%familial10%children10%normal

bloodpressure2023/4/283IntroductionThemostfrequentsiteofmetastasesistheskeletonAdditionalsitesareliver,retroperitoneumwithlymphnodes,CNS,pleur

a,andkidney2023/4/284Malignantvs.BenignCurrently,thereisnoeffectivecureformalignantpheochromocytoma.Therearealsonoreliablehistopathologicalmetho

dsfordistinguishingbenignfrommalignanttumors.Malignancyrequiresevidenceofmetastasesatnon-chromaffinsitesdistantfromthato

ftheprimarytumor.2023/4/285Metastaticdiseaseinpheochromocytomamaybepresentatthetimeofinitialdiagnosisormayonlybecameevidentaftersurgica

lremovaloftheprimarytumor,usuallywithin5years,butsometimes16ormoreyearslater.2023/4/286Duetotherarityofthetumor,clinicalstudiesaboutpheochrom

ocytomasufferfromafragmentednatureandusuallyinvolvetoosmallanumberofcasestoreachconclusiveresults.2023/4/287Becausethereiscurrentlynoeffe

ctivecureformalignantpheochromocytoma,mosttreatmentarepalliative,butinsomecasesmayreducetumorburdenandprolongsurvival.Witho

uttreatment,the5-yearsurvivalisgenerallylessthan50%.Thecourse,however,canbehighlyvariablewithoccasionalpatientslivingmoret

han20yearsafterdiagnosis.2023/4/288Oncemalignancyisdiagnosed,therapyisgenerallydirectedatcontrollingbloodpressure,butmayalsoincludetumordebulk

ing.2023/4/289AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolizatio

n2023/4/2810AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization2023/4/2811Prim

arysurgicalresectionisthetreatmentofchoicewheneverpossibleLimiteddisease:curativeintentionExtendeddisease:stilltobecons

ideredinthefirstplacefordebulkingandaspalliativetreatment(Mundschenketal.1998)2023/4/2812ProblemWhensignsofregionalinvolvementordi

stantdiseaseareabsent,thereiscurrentlynoreliablepreoperativediagnostictestthatcandifferentiatebetweenmalignantandben

ignpheochromocytomasShouldpheochromocytomasizeinfluencesurgicalapproach?2023/4/2813Acomparisonof90malignantand60benignpheochromocytomas(WenT.

Shenetal.2004)Comparisonoftumorsizeforbenignpheochromocytomasandmalignantpheochromocytomaswithlocaldi

seaseonlySizedoesnotreliablypredictmalignancyinpheochromocytomaswithlocaldiseaseonly2023/4/2814Malignant(n=29)Ben

ign(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≥10cm432

023/4/2815MalignantPCCspresentingwithonlylocaldiseasecannotbediscriminatedfrombenignPCCsbysizealone.WhenPCC

sdonothaveevidenceofinvasionordistantmetastasesandthesurgeonacquiresanappropriatelevelofexperience,themajorityofthesetumorscanbesafelyresec

tedlaparoscopically.2023/4/2816Laparoscopicadrenalectomyforpheochromocytomashouldbeconvertedtoopenadrenalectomyfordifficultdissection

,invasion,adhesions,orsurgeoninexperience2023/4/2817SurgicalapproachTransabdominalapproachisnecessaryminimallyinvasivep

roceduresretroperitonealapproachesshouldbeabandonedtodefinitelypreservethetumorcapsuleandperformtotallymphadecectomy(Orchar

detal.1993)2023/4/2818SecondaryTumorsNoexperiencewithadjuvantpreandpostoperativeradiationexistsGenerallyaremultiple

RadicalsurgicalresectionisoftenimpossibleOthertreatmentmodalitieshavetobeconsidered2023/4/2819AlternativeofCurrentTherapySu

rgeryRadiopharmaceuticalsCombinedChemotherapyArterialEmbolization2023/4/28202023/4/2821131I-MIBGisthetreatmentofchoiceforallunresectable,MIB

Gpositivetumors58casesofmalignantPCCtreatedby131I-MIBG—therapeuticresultsandadverseevents(ZHURuisenetal.1999)2

023/4/2822Patientswereclassifiedinto3groupsaccordingtotheirtumorsize<8cm3(11cases),8～20cm3(21cases),>20cm3(26cases)In

group1,themeanabsorptiondosepergramoftumorwasabove1000cGy.Aftertreatment,tumorsdisappearedorshrinkedi

nallpatients2023/4/2823Ingroup2,theabsorptiondosewassimilartothatofgroup1,butthemeanabsorptiondosepergramwas717.6cGy,andtumormassregressionwa

s36%;76%reducedurinarycatecholamineIngroup3,theabsorptiondosepergramtumortissuewas277cGy,and30%tumorenlargement,20%died;therem

aining50%symptomaticimprovementwithoutanychangeintumorsize2023/4/2824131I-MIBGisofcertaintherapeuticeffectivenessofsymptomaticimprovementCo

mpletetumormassdisappearancehasonlybeenfoundinsmalltumorsTreatmentwith131I-MIBGshouldbeinstitutedimmediatelyaftersurgicalr

esectiontoeradicatetheresidualtumorcellsandtopreventrecurrencesBonemarrowsuppressionistemporaryandnotdosagerelated2023/4/2825In1997,Lo

hetal.publishedareviewoftheworldwideexperienceinvolving116patientstreatedwith131I-MIBGformalignantpheochromocytom

a.Overall,therewasasymptomaticresponsein76%,ahormonalresponsein45%,andtumorregressionin30%.Theactivityof

131I-MIBGpersingledosewas96–300mCi,andthemeancumulativeactivitywas490±350mCi.OnlyfiveCRsto131I-MIBGwerereported.2023/4/2826Limitations

NotallpatientswithmultiplemetastasesofmalignantpheochromocytomashavesufficientuptakeofMIBGtoallowMIBG

therapyMIBGnegativelesionscoexistwithMIBGpostivelesions,requiringcombinedtreatment2023/4/2827Asasi

ngleagent,131I-MIBGhaslimitedefficacyintreatingmalignantpheochromocytoma.Itsuseincombinationwithothercytotoxicagents,asiscurrentlybeingstu

diedinpatientswithneuroblastoma,mayresultinadditionalbenefit(Sissonetal.1999)2023/4/2828AlternativeofCurrentTherapySurgeryRadiophar

maceuticalsCombinedChemotherapyArterialEmbolization2023/4/2829Onlysparsedataonchemotherapeuticregimensareavailable,mostoftheminr

eportsoffewcasesThemostwell-establishedregimenisCVD(Averbuchetal.1988)CTX750mg/m2d1,VCR1.4mg/m2d1,Da

carbazine600mg/m2d1,2Cycle21days2023/4/2830TheCVDregimenwasbasedonthetreatmentforadvancedneuroblastoma.Thisregimenhas

beenreportedtoproducegoodresponsesinmalignantpheochromocytoma,butthemediandurationofremissionis21monthsCompletelong-termdiseaseremissionswithchemo

therapyhavenotbeenreported.2023/4/2831AlternativeofCurrentTherapySurgeryRadiopharmaceuticalsCombinedChemotherapyTranscatheterArterialEmboli

zation2023/4/2832TAEhasbeensuccessfullyperformedinthetreatmentofmalignantPCCwithlivermetastasesThetherapeuticeffect

sofTAEhavebeendemonstratedtobeenhancedbythecombinationtherapywithanticancerchemotherapy2023/4/2833MitomycinChasbeensuccessfullyusedin

TAEforlivermetastasisinseveralcasesofmalignantPCC.2023/4/28342023/4/2835Malignantpheochromocytoma:past,presentandfuturePastPresentFutureAdrenergic

blockers,α-methyl-paratyrosine&useofotherdrugsforsymptomaticreliefSurgicaldebulking;131I-MIBGradiotherapy;Chemotherapy;Chemoembolizatio

nMoleculartargeting,cancervaccines,genetherapy