肾上腺意外瘤指南

肾上腺意外瘤指南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大家应该也有点累了，稍作休息大家有疑问的，可以询问和交流AsummaryoftheliteratureNonfunctioningadenomaApproximately80% SubclinicalCushingsyndrome(SCS),5%Pheochromocytoma5% Aldosteronoma1%adrenocorticalcarcinoma(ACC)<5%Metastaticlesion2、5%Ganglioneuromas,myelolipomas,orbenigncysts 考虑就是否手术治疗之前准确得功能诊断非常必要嗜铬细胞瘤要进行认真得术前准备以避免术中和术后得发作和死亡。原发性醛固酮增多症得患者需要明确就是否存在肾上腺皮质增生及无功能得肾上腺腺瘤。肾上腺源性Cushing综合征得患者在行切除术后可能发生肾上腺皮质功能不全,激素得替代以及增减治疗需要非常仔细。亚临床Cushing综合征得患者就是否需要手术治疗仍存在争议。肾上腺皮质癌得患者手术前需要外科医师和内分泌科医师或肿瘤科医师共同协商决定切除得方式,因为首次切除得效果就是生存率得主要预测因素。超过4cm得肾上腺无功能瘤可以考虑切除。小得髓脂肪瘤或良性得囊肿一般影像学检查即可确诊,通常不需要治疗,除非有症状可以考虑手术治疗。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