腎上腺意外瘤指南

腎上腺意外瘤指南Definition“Masslesiongreaterthan1cmindiameterdiscovered“accidentally”duringaradiographicexaminationperformedforindicationsotherthananevaluationforadrenaldisease、”Managementoftheclinicallyinapparentadrenalmass(incidentaloma)、NIHState-of-the-ScienceConferenceStatementFeb4-6,2002、PrevalenceAutopsies:87,065cases:6%withadrenaladenomasAbdominalCT(61,054CTscansreviewed):4%withadrenaladenomasNowapproachesthe8、7%incidencereportedinautopsyseries

IncidenceIncreaseswithAgeEndocrineandMetabolismClinicsofNorthAmerica、2000;29(1):159-185ThreeMainQuestionsIstheadrenalmasshormonallyactive?Isthemassbenignormalignant?Doesthepatienthaveahistoryof apreviousmalignantlesion? Isitmetastatic?AnatomyAnatomyAnatomyPrimaryAldosteronismCushing’sSyndromeDHEA-sPheochromocytomaFrequencyofFindingsMulticenterstudyof1096casesNonfunctioningadenoma:85%SubclinicalCushing’ssyndrome:9%Pheochromocytoma:4%Aldosteronomas:2%Manteroetal、85(2):637、(2000)

FrequencyofFindingsAllolio,B、,AdrenalIncidentalomas、AdrenalDisorders,ed、C、G、MargiorisAN、2001,Totowa:HumanaPressInc、11大家應(yīng)該也有點(diǎn)累了，稍作休息大家有疑問(wèn)的，可以詢(xún)問(wèn)和交流AsummaryoftheliteratureNonfunctioningadenomaApproximately80% SubclinicalCushingsyndrome(SCS),5%Pheochromocytoma5% Aldosteronoma1%adrenocorticalcarcinoma(ACC)<5%Metastaticlesion2、5%Ganglioneuromas,myelolipomas,orbenigncysts 考慮就是否手術(shù)治療之前準(zhǔn)確得功能診斷非常必要嗜鉻細(xì)胞瘤要進(jìn)行認(rèn)真得術(shù)前準(zhǔn)備以避免術(shù)中和術(shù)后得發(fā)作和死亡。原發(fā)性醛固酮增多癥得患者需要明確就是否存在腎上腺皮質(zhì)增生及無(wú)功能得腎上腺腺瘤。腎上腺源性Cushing綜合征得患者在行切除術(shù)后可能發(fā)生腎上腺皮質(zhì)功能不全,激素得替代以及增減治療需要非常仔細(xì)。亞臨床Cushing綜合征得患者就是否需要手術(shù)治療仍存在爭(zhēng)議。腎上腺皮質(zhì)癌得患者手術(shù)前需要外科醫(yī)師和內(nèi)分泌科醫(yī)師或腫瘤科醫(yī)師共同協(xié)商決定切除得方式,因?yàn)槭状吻谐眯Ч褪巧媛实弥饕A(yù)測(cè)因素。超過(guò)4cm得腎上腺無(wú)功能瘤可以考慮切除。小得髓脂肪瘤或良性得囊腫一般影像學(xué)檢查即可確診,通常不需要治療,除非有癥狀可以考慮手術(shù)治療。Algorithmfortheevaluationandmanagementofanadrenalincidentaloma*Reimagein3to6monthsandannuallyfor1to2years;repeatfunctionalstudiesannually for5years、Ifmassgrowsmorethan1cmorbeeshormonallyactive,thenadrenalectomyisremended、HyperfunctioningHormonalEvaluationSubclinicalCushing’sSyndromePheochromocytomaPrimaryAldosteronismSexhormone-secretingadrenocorticaltumorsSubclinicalCushing’sSyndromeHypercortisolismwithoutclinicalmanifestationsofCushing’ssyndromeMostfrequenthormonalabnormalityinadrenalincidentalomasSubclinicalCushing’sSyndromeCentralobesityFacialroundingBuffalohumpEasybruisingPurplestriaeProximalmuscleweaknessEmotional/cognitivechangesSubclinicalCushing’sSyndromeIncreaseriskfor:HypertensionDyslipidemiaImpairedglucosetoleranceType2DMAtherosclerosisOsteoporosis?TauchmanovaL,et、al、PatientswithsubclinicalCushing’ssyndromeduetoadrenaladenomahaveincreasecardiovascularrisk、JCEM2000;85:1440、SubclinicalCushing’sSyndromeBiochemicalabnormalitiesElevatedurinefreecortisolLoworsuppressedACTHBlunteddiurnalvariationNocortisolsuppressionafter1mgovernightdexamethasonesuppressiontest-BESTSCREENINGTEST!1、ManteroF,etal、HormoneRes47:284–289,19972、MontwillJ,etal、TheO/NDSTistheprocedureofchoiceforscreeningforCushing’ssyndrome、Steroids1994;59:2296DexamethasoneSuppressionTest1mgdexamethasoneat11PMMeasurecortisolat8AMthenextmorningNormal:cortisol<1、8μg/dL(5ug/dl)SpecificityofDSTis72-82%(100%)Sensitivity75-100%(58%)SeverebipolardepressionandseverealcoholismcangivefalsepositiveresultsIftheDST8AMserumcortisolisabnormal,thenbaselineACTH,serumand24-hoururinarycortisolshouldbeobtainedandmidnightsalivarycortisol,ora2-daylow-dosedexamethasonesuppressiontestisneededtoconfirmautonomyHyperfunctioningHormonalEvaluationSubclinicalCushing’sSyndromePheochromocytomaPrimaryAldosteronismSexhormone-secretingadrenocorticaltumorsPheochromocytomaRarebutfatalcatecholaminesproducingtumorIncidence:2-8/millionpeople/yearAccountfor5%ofadrenalincidentalomaRuleof10s:10%extra-adrenal,10%bilateral,10%familial,10%malignantAsidefromcatecholamines,itcanalsosecretedopamine,ACTH,PTH,calcitonin,VIPPheochromocytomaClassictriads:SuddensevereheadacheDiaphoresisPalpitations94%specificity;91%sensitivityinhypertensivepopulationPheochromocytoma19-76%ofpheoareundiagnoseduntilafterdeath80%ofpatientwithunsuspectedpheowhounderwentsurgeryoranesthesiawilldieAlthoughradiographiccharacteristicscangivesomeclues:EnhancementwithIVonCTHighsignalintensityonT2weightedMRIProminentvascularityThustheneedforscreeningImagingSilent8cmpheoPheochromocytomaAvailableTests:Plasmafractionatedfreemetanephrines24-hoururinaryfractionatedmetanephrinesandcatecholaminesPlasmacatecholaminesUrinarytotalmetanephrinesUrinaryvanillylmandelicacidWhichtestisbest?LiteratureSupportsSensitivitywashighestforfractionatedPLASMAfreemetanephrines(99percent)Usingreceiveroperatingcharacteristiccurves,sensitivityvaluesatdifferentupperreferencelimitswerehighestforfractionatedplasmafreemetanephrines、“Fractionatedplasmafreemetanephrineswerethebesttestforexcludingpheochromocytomaandshouldbethediagnostictestoffirstchoice、”JAMA2002LiteratureSupportsPLASMAfreemetanephrines-BESTscreeningtestWhenthetestisnegative-practicallyrulesoutpheoCost$100pertestURINARYmetanephrines-lesssensitiveUrinaryVMAisoutdatedPresentedattheFirstInternationalmeetingonAdrenalDisease,2002BrazJMedBiolRes33(10)2000Whenthetestisnegative,noothertestsareneeded、NIHState-of-theScienceConferenceStatementFinalStatement7/16/2002“Plasmafreemetanephrinesareremendedasthetestofchoiceforexcludingorconfirmingthediagnosisofpheochromocytoma、”Managementoftheclinicallyinapparentadrenalmass(incidentaloma)、NIHState-of-the-ScienceConferenceStatementFeb4-6,2002Disagreement!LiteratureSearch“Thefirstinitialtestofchoiceforlowriskpatientsisthe24-hoururinaryfractionatedmetanephrinesandcatecholamines、”Althoughelevatedlevelsoffractionatedplasmametanephrineshavehighsensitivityforpheo(99%),thetesthasalowspecificity(85%)andthusshouldbeusedwhensuspicionishigh、Whattodo?PlasmafractionatedmetanephrinesSens:97-100%,Spec85-89%UrinaryfractionatedmetanephrinesandcatecholaminesSens:91%,Spec98%HyperfunctioningHormonalEvaluationSubclinicalCushing’sSyndromePheochromocytomaPrimaryAldosteronismSexhormone-secretingadrenocorticaltumorsPrimaryAldosteronism1%ofadrenalincidentalomacharacterizedby:highbloodpressureRefractorytotreatmenthypokalemiaWeakness,crampssuppressedreninactivitymetabolicalkalosisPrimaryAldosteronismPatientswithprimaryaldosteronismhasincreasedriskforcardiovasculardiseaseThusnecessarytoscreenallpatientswithadrenalincidentalomaforPAScreeningtestis:PACandPAC/PRAratioPAC/PRA>30andPAC>20ng/dL90%specandsensitivityforPAIfscreeningtestispositive-needtoconfirmwithsalinesuppressiontest,adrenalvenoussamplingandimagingmidnightsalivarycortisol,ora2-daylow-dosedexamethasonesuppressiontestmidnightsalivarycortisol,ora2-daylow-dosedexamethasonesuppressiontestHyperfunctioningHormonalEvaluationSubclinicalCushing’sSyndromePheochromocytomaPrimaryAldosteronismSexhormone-secretingadrenocorticaltumorsSexhormone-secretingAdrenocorticalTumorsRareTypicallyoccurinthepresenceofclinicalmanifestations(hirsutismorvirilization)HirsutismSexhormone-secretingAdrenocorticalTumorsRareTypicallyoccurinthepresenceofclinicalmanifestations(hirsutismorvirilization)RoutinescreeningforexcessandrogensandestrogensisnotwarrantedHormonalWorkupSummary3hormonaltestsnecessaryforworkupofadrenalincidentaloma:1mgovernightdexamethasonesuppresiontestPlasmaorurinaryfractionatedmetaneprinesPlasmaaldosteroneconcentrationandplasmaaldosteroneconcentration/plasmareninactivityratio(PAC/PRA)、TreatmentAllpatientswithdocumentedpheochromocytomaandprimaryaldosteronismshouldundergosurgeryNoprospective,randomizedtrialsforSubclinicalCushing’sSyndromebutconcensusistoproceedwithsurgeryifthepatientisyoungThreeMainQuestionsIstheadrenalmasshormonallyactive?Isthemassbenignormalignant?Doesthepatienthaveahistoryof apreviousmalignantlesion? Isitmetastatic?PrimaryAdrenalCarcinomaVeryrare:5casesper1millionpopulationSmallsizecorrespondstobetterprognosis5yearsurvivalOverall:16%Localizeddisease(stageIandII):42%Metastases:5、3%Imagingplexsolidandcystic,calcifiedmassPatientwithKnownMalignancy10-40%ofpatientswithknownmalignancyhaveadrenalmetastasesatautopsyMostmonprimaryBreastLungKidneyMelanomaLymphomaAssessmentofMalignantPotentialSizeImagingPhenotype(features)SizeProbabilityofmalignancyincreaseswithsizeInastudyinvolving887patientswithadrenalincidentalomas,90%ofpatientswithadrenalcarcinomashastumor>4cm(NationalItalianStudyGroup,1997)adrenalcarcinomas2%(<4cm),6%(4-6cm),25%(>6cm)SizeMayoClinicStudy342PatientswithadrenalincidentalomaretrospectivelyevaluatedTumordiameteraveraged2、5cmMostmalignanttumorsmeasured>5cm

Incidentallydiscoveredadrenaltumors:aninstitutionalperspective、HerreraMF;GrantCS;vanHeerdenJA;SheedyPF;IlstrupDM、Surgery1991Dec;110(6):1014-21SizeConsensusStatementMass>6cmshouldberemovedMass<4cmcanbemonitoredMassbetween4-6cm:Criteriaotherthansizeshouldbeusedtodictatesurgeryvs、monitoringManagementoftheclinicallyinapparentadrenalmass(incidentaloma)、NIHState-of-the-ScienceConferenceStatementFeb4-6,2002、AssessmentofMalignantPotentialSizeImagingPhenotypeImagePhenotype-CTScanHounsfieldunit(HU)-semiquantitativemethodformeasuringx-rayattenu