肾上腺意外瘤指南Adrenal Incidentaloma guidelines.ppt

AdrenalIncidentalomaguidelines AACE AAES Outline DefinitionPrevalenceAnatomyandPhysiologyReviewDiagnosticWorkupsConclusions Definition Masslesiongreaterthan1cmindiameterdiscovered accidentally duringaradiographicexaminationperformedforindicationsotherthananevaluationforadrenaldisease Managementoftheclinicallyinapparentadrenalmass incidentaloma NIHState of the ScienceConferenceStatementFeb4 6 2002 Prevalence Autopsies 87 065cases 6 withadrenaladenomasAbdominalCT 61 054CTscansreviewed 4 withadrenaladenomasNowapproachesthe8 7 incidencereportedinautopsyseries IncidenceIncreaseswithAge EndocrineandMetabolismClinicsofNorthAmerica 2000 29 1 159 185 ThreeMainQuestions Istheadrenalmasshormonallyactive Isthemassbenignormalignant Doesthepatienthaveahistoryofapreviousmalignantlesion Isitmetastatic Anatomy http shutterbug ucsc edu sealion view photo php set albumName album265 id Adrenal Anatomy http shutterbug ucsc edu sealion view photo php set albumName album265 id Adrenal Anatomy PrimaryAldosteronism Cushing sSyndrome DHEA s Pheochromocytoma FrequencyofFindings Multicenterstudyof1096casesNonfunctioningadenoma 85 SubclinicalCushing ssyndrome 9 Pheochromocytoma 4 Aldosteronomas 2 Manteroetal 85 2 637 2000 FrequencyofFindings Allolio B AdrenalIncidentalomas AdrenalDisorders ed C G MargiorisAN 2001 Totowa HumanaPressInc Asummaryoftheliterature NonfunctioningadenomaApproximately80 SubclinicalCushingsyndrome SCS 5 Pheochromocytoma5 Aldosteronoma1 adrenocorticalcarcinoma ACC 5 Metastaticlesion2 5 Ganglioneuromas myelolipomas orbenigncysts 考虑是否手术治疗之前准确的功能诊断非常必要 嗜铬细胞瘤要进行认真的术前准备以避免术中和术后的发作和死亡 原发性醛固酮增多症的患者需要明确是否存在肾上腺皮质增生及无功能的肾上腺腺瘤 肾上腺源性Cushing综合征的患者在行切除术后可能发生肾上腺皮质功能不全 激素的替代以及增减治疗需要非常仔细 亚临床Cushing综合征的患者是否需要手术治疗仍存在争议 肾上腺皮质癌的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的方式 因为首次切除的效果是生存率的主要预测因素 超过4cm的肾上腺无功能瘤可以考虑切除 小的髓脂肪瘤或良性的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 repeatfunctionalstudiesannuallyfor5years Ifmassgrowsmorethan1cmorbecomeshormonallyactive thenadrenalectomyisrecommended HyperfunctioningHormonalEvaluation SubclinicalCushing sSyndromePheochromocytomaPrimaryAldosteronismSexhormone secretingadrenocorticaltumors SubclinicalCushing sSyndrome HypercortisolismwithoutclinicalmanifestationsofCushing ssyndromeMostfrequenthormonalabnormalityinadrenalincidentalomas SubclinicalCushing sSyndrome CentralobesityFacialroundingBuffalohumpEasybruisingPurplestriaeProximalmuscleweaknessEmotional cognitivechanges SubclinicalCushing sSyndrome Increaseriskfor HypertensionDyslipidemiaImpairedglucosetoleranceType2DMAtherosclerosisOsteoporosis TauchmanovaL et al PatientswithsubclinicalCushing ssyndromeduetoadrenaladenomahaveincreasecardiovascularrisk JCEM2000 85 1440 SubclinicalCushing sSyndrome BiochemicalabnormalitiesElevatedurinefreecortisolLoworsuppressedACTHBlunteddiurnalvariationNocortisolsuppressionafter1mgovernightdexamethasonesuppressiontest BESTSCREENINGTEST 1 ManteroF etal HormoneRes47 284 289 19972 MontwillJ etal TheO NDSTistheprocedureofchoiceforscreeningforCushing ssyndrome Steroids1994 59 2296 DexamethasoneSuppressionTest 1mgdexamethasoneat11PMMeasurecortisolat8AMthenextmorningNormal cortisol 1 8 g dL 5ug dl SpecificityofDSTis72 82 100 Sensitivity75 100 58 SeverebipolardepressionandseverealcoholismcangivefalsepositiveresultsIftheDST8AMserumcortisolisabnormal thenbaselineACTH serumand24 hoururinarycortisolshouldbeobtainedandmidnightsalivarycortisol ora2 daylow dosedexamethasonesuppressiontestisneededtoconfirmautonomy HyperfunctioningHormonalEvaluation SubclinicalCushing sSyndromePheochromocytomaPrimaryAldosteronismSexhormone secretingadrenocorticaltumors Pheochromocytoma RarebutfatalcatecholaminesproducingtumorIncidence 2 8 millionpeople yearAccountfor5 ofadrenalincidentalomaRuleof10s 10 extra adrenal 10 bilateral 10 familial 10 malignantAsidefromcatecholamines itcanalsosecretedopamine ACTH PTH calcitonin VIP Pheochromocytoma Classictriads SuddensevereheadacheDiaphoresisPalpitations94 specificity 91 sensitivityinhypertensivepopulation Pheochromocytoma 19 76 ofpheoareundiagnoseduntilafterdeath80 ofpatientwithunsuspectedpheowhounderwentsurgeryoranesthesiawilldieAlthoughradiographiccharacteristicscangivesomeclues EnhancementwithIVonCTHighsignalintensityonT2weightedMRIProminentvascularityThustheneedforscreening Imaging Silent8cmpheo Pheochromocytoma AvailableTests Plasmafractionatedfreemetanephrines24 hoururinaryfractionatedmetanephrinesandcatecholaminesPlasmacatecholaminesUrinarytotalmetanephrinesUrinaryvanillylmandelicacidWhichtestisbest LiteratureSupports SensitivitywashighestforfractionatedPLASMAfreemetanephrines 99percent Usingreceiveroperatingcharacteristiccurves sensitivityvaluesatdifferentupperreferencelimitswerehighestforfractionatedplasmafreemetanephrines Fractionatedplasmafreemetanephrineswerethebesttestforexcludingpheochromocytomaandshouldbethediagnostictestoffirstchoice JAMA2002 LiteratureSupports PLASMAfreemetanephrines BESTscreeningtestWhenthetestisnegative practicallyrulesoutpheoCost 100pertestURINARYmetanephrines lesssensitiveUrinaryVMAisoutdated PresentedattheFirstInternationalmeetingonAdrenalDisease 2002BrazJMedBiolRes33 10 2000 Whenthetestisnegative noothertestsareneeded NIHState of theScienceConferenceStatement FinalStatement7 16 2002 Plasmafreemetanephrinesarerecommendedasthetestofchoiceforexcludingorconfirmingthediagnosisofpheochromocytoma Managementoftheclinicallyinapparentadrenalmass incidentaloma NIHState of the ScienceConferenceStatementFeb4 6 2002 Disagreement LiteratureSearch Thefirstinitialtestofchoiceforlowriskpatientsisthe24 hoururinaryfractionatedmetanephrinesandcatecholamines Althoughelevatedlevelsoffractionatedplasmametanephrineshavehighsensitivityforpheo 99 thetesthasalowspecificity 85 andthusshouldbeusedwhensuspicionishigh Whattodo PlasmafractionatedmetanephrinesSens 97 100 Spec85 89 UrinaryfractionatedmetanephrinesandcatecholaminesSens 91 Spec98 HyperfunctioningHormonalEvaluation SubclinicalCushing sSyndromePheochromocytomaPrimaryAldosteronismSexhormone secretingadrenocorticaltumors PrimaryAldosteronism 1 ofadrenalincidentalomacharacterizedby highbloodpressureRefractorytotreatmenthypokalemiaWeakness crampssuppressedreninactivitymetabolicalkalosis PrimaryAldosteronism PatientswithprimaryaldosteronismhasincreasedriskforcardiovasculardiseaseThusnecessarytoscreenallpatientswithadrenalincidentalomaforPAScreeningtestis PACandPAC PRAratioPAC PRA 30andPAC 20ng dL90 specandsensitivityforPAIfscreeningtestispositive needtoconfirmwithsalinesuppressiontest adrenalvenoussamplingandimaging midnightsalivarycortisol ora2 daylow dosedexamethasonesuppressiontest midnightsalivarycortisol ora2 daylow dosedexamethasonesuppressiontest HyperfunctioningHormonalEvaluation SubclinicalCushing sSyndromePheochromocytomaPrimaryAldosteronismSexhormone secretingadrenocorticaltumors Sexhormone secretingAdrenocorticalTumors RareTypicallyoccurinthepresenceofclinicalmanifestations hirsutismorvirilization Hirsutism Sexhormone secretingAdrenocorticalTumors RareTypicallyoccurinthepresenceofclinicalmanifestations hirsutismorvirilization Routinescreeningforexcessandrogensandestrogensisnotwarranted HormonalWorkupSummary 3hormonaltestsnecessaryforworkupofadrenalincidentaloma 1mgovernightdexamethasonesuppresiontestPlasmaorurinaryfractionatedmetaneprinesPlasmaaldosteroneconcentrationandplasmaaldosteroneconcentration plasmareninactivityratio PAC PRA Treatment AllpatientswithdocumentedpheochromocytomaandprimaryaldosteronismshouldundergosurgeryNoprospective randomizedtrialsforSubclinicalCushing sSyndromebutconcensusistoproceedwithsurgeryifthepatientisyoung ThreeMainQuestions Istheadrenalmasshormonallyactive Isthemassbenignormalignant Doesthepatienthaveahistoryofapreviousmalignantlesion Isitmetastatic PrimaryAdrenalCarcinoma Veryrare 5casesper1millionpopulationSmallsizecorrespondstobetterprognosis5yearsurvivalOverall 16 Localizeddisease stageIandII 42 Metastases 5 3 Imaging complexsolidandcystic calcifiedmass PatientwithKnownMalignancy 10 40 ofpatientswithknownmalignancyhaveadrenalmetastasesatautopsyMostcommonprimaryBreastLungKidneyMelanomaLymphoma AssessmentofMalignantPotential SizeImagingPhenotype features Size ProbabilityofmalignancyincreaseswithsizeInastudyinvolving887patientswithadrenalincidentalomas 90 ofpatientswithadrenalcarcinomashastumor 4cm NationalItalianStudyGroup 1997 adrenalcarcinomas2 6cm Size MayoClinicStudy342PatientswithadrenalincidentalomaretrospectivelyevaluatedTumordiameteraveraged2 5cmMostmalignanttumorsmeasured 5cmIncidentallydiscoveredadrenaltumors aninstitutionalperspective HerreraMF GrantCS vanHeerdenJA SheedyPF IlstrupDM Surgery1991Dec 110 6 1014 21 Size ConsensusStatementMass 6cmshouldberemovedMass 4cmcanbemonitoredMassbetween4 6cm Criteriaotherthansizeshouldbeusedtodictatesurgeryvs monitoring Managementoftheclinicallyinapparentadrenalmass incidentaloma NIHState of the ScienceConferenceStatementFeb4 6 2002 AssessmentofMalignantPotential SizeImagingPhenotype ImagePhenotype CTScan Hounsfieldunit HU semiquantitativemethodformeasuringx rayattenuationWater 0HUAdiposetissue 20to 150HUKidney 20to50HUBone 1000HULipidrichmassarebenignHU 10onunenhancedCT benignadenoma100 ImagePhenotype CTScan Retrospectiveanalysisof151patientswithadrenalmassesHU 10oracombinationoftumorsize 4cmandHU 20 excludednon adenomasin100percentofcases Hamrahian etal JCEM2005 90 871 ImagePhenotype CTScan ContrastwashoutOncontrast enhancedCT adenomasexhibitrapidwashoutcomparedtonon adenomas metastases angiosarcoma pheo carcinoma Washoutof 60 at10min nocancerWashout 60 at10min highriskformalignantlesion Imaging metastases MRI EquallyeffectiveasCTAdenomasareisointensewiththeliveronT2weightedimagesCarcinomasarehyperintensecomparedtotheliveronT2weightedimages FNA CytologyfromFNAca