1、肾上腺意外瘤指南(优选)肾上腺意外瘤指南Management of the clinically inapparent adrenal mass(incidentaloma).NIH State-of-the-Science Conference Statement Feb 4-6,2002.024680910192029303940495059606970Age(y)Patient(%)Endocrine and Metabolism Clinics of North America.2000;29(1):159-185No cortisol suppression after 1 mg o
2、vernight dexamethasone suppression test BEST SCREENING TEST!Mass between 46 cm:Criteria other than size should be used to dictate surgery vs.Primary AldosteronismUrinary total metanephrines1%of adrenal incidentalomaJCEM 2005;90:871“The first initial test of choice for low risk patients is the 24hour
3、 urinary fractionated metanephrines and catecholamines.monitoringProbability of malignancy increases with sizeRefractory to treatmentThree Main QuestionsSubclinical Cushings SyndromeLiterature SearchBuffalo humpWater=0 HUIs the mass benign or malignant?Sensitivity was highest for fractionated PLASMA
4、 free metanephrines(99 percent)Subclinical Cushings syndrome:9%Primary AldosteronismCushings SyndromeDHEA-sPheochromocytomaMantero et al.85(2):637.(2000)Allolio,B.,Adrenal Incidentalomas.Adrenal Disorders,ed.C.G.Margioris AN.2001,Totowa:Humana Press Inc.A summary of the literaturevNonfunctioning ade
5、noma Approximately 80%vSubclinical Cushing syndrome(SCS),5%vPheochromocytoma 5%vAldosteronoma 1%vadrenocortical carcinoma(ACC)5%vMetastatic lesion 2.5%vGanglioneuromas,myelolipomas,or benign cysts考虑是否手术治疗之前准确的功能诊断非常必要 v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。v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
6、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。v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。v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。Algorithm for the evaluation and management of an adrenal incidentaloma*Reimage in 3 to 6 mont
7、hs and annually for 1 to 2 years;repeat functional studies annually for 5 years.If mass grows more than 1cm or becomes hormonally active,then adrenalectomy is recommended.AtherosclerosisLipid rich mass are benign2001,Totowa:Humana Press Inc.Assessment of Malignant Potential94%specificity;91%sensitiv
8、ity in hypertensive populationcystic,calcified mass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。Primary AldosteronismPrimary AldosteronismPheochromocytomaJCEM 2005;90:871A summary of the literatureHamrahian,et al.2000;29(1):159-185Sex hormonesecreting adrenocortical tumorsAlthough elevate
9、d levels of fractionated plasma metanephrines have high sensitivity for pheo(99%),the test has a low specificity(85%)and thus should be used when suspicion is high.Adipose tissue=20 to 150 HUClassic triads:Literature Search7%incidence reported in autopsy seriesTauchmanova L,et.al.Patients with subcl
10、inical Cushings syndrome due to adrenal adenoma have increase cardiovascular risk.JCEM 2000;85:1440.Silent 8 cm pheoJAMA 2002Presented at the First International meeting on Adrenal Disease,2002Braz J Med Biol Res 33(10)2000When the test is negative,no other tests are needed.Hamrahian,et al.Atheroscl
11、erosissuppressed renin activityCost$100 per testGanglioneuromas,myelolipomas,or benign cystsAtherosclerosisIs the mass benign or malignant?3 hormonal tests necessary for workup of adrenal incidentaloma:Surgery 1991 Dec;110(6):101421If the DST 8AM serum cortisol is abnormal,then baseline ACTH,serum a
12、nd 24hour urinary cortisol should be obtained and midnight salivary cortisol,or a 2day lowdose dexamethasone suppression test is needed to confirm autonomyOn contrastenhanced CT,adenomas exhibit rapid washout compared to nonadenomas(metastases,angiosarcoma,pheo,carcinoma)Although elevated levels of
13、fractionated plasma metanephrines have high sensitivity for pheo(99%),the test has a low specificity(85%)and thus should be used when suspicion is high.Easy bruising“Plasma free metanephrines are recommended as the test of choice for excluding or confirming the diagnosis of pheochromocytoma.Wash out
14、 60%at 10 min=high risk for malignant lesionAldosteronoma 1%Nonfunctioning adenoma:85%Metastases:5.PheochromocytomaFrequency of FindingsProminent vascularityManagement of the clinically inapparent adrenal mass(incidentaloma).NIH State-of-the-Science Conference Statement Feb 4-6,2002midnight salivary
15、 cortisol,or a 2-day low-dose dexamethasone suppression testmidnight salivary cortisol,or a 2-day low-dose dexamethasone suppression test2001,Totowa:Humana Press Inc.Subclinical Cushings SyndromeAldosteronomas:2%Bone=1000 HUAdrenal Disorders,ed.2001,Totowa:Humana Press Inc.FNA is useful only in dist
16、inguishing adrenal tumor from metastasis and infectionOverall:16%1976%of pheo are undiagnosed until after deathAtherosclerosisWhen the test is negative practically rules out pheoIncidence:28/million people/yearday low-dose dexamethasone suppression testCost$100 per testDoes the patient have a histor
17、y of a previous malignant lesion?Pheochromocytoma 5%Aldosteronoma 1%Assessment of Malignant Potentialcomplex solid and cystic,calcified massHerrera MF;Grant CS;van Heerden JA;Sheedy PF;Ilstrup DM.Surgery 1991 Dec;110(6):101421 Management of the clinically inapparent adrenal mass(incidentaloma).NIH State-of-the-Science Conference Statement Feb 4-6,2002.Hamrahian,et al.JCEM 2005;90:871