腎上腺意外瘤指南Adrenal Incidentaloma guidelines

1、Adrenal Incidentaloma guidelines AACE/AAES OutlineDefinitionPrevalenceAnatomy and Physiology ReviewDiagnostic WorkupsConclusionsDefinition“Mass lesion greater than 1 cm in diameter discovered “accidentally” during a radiographic examination performed for indications other than an evaluation for adre

2、nal disease.”Management of the clinically inapparent adrenal mass (incidentaloma). NIH State-of-the-Science Conference Statement Feb 4-6, 2002.PrevalenceAutopsies: 87,065 cases: 6% with adrenal adenomasAbdominal CT (61,054 CT scans reviewed): 4% with adrenal adenomasNow approaches the 8.7% incidence

3、 reported in autopsy series Incidence Increases with AgeEndocrine and Metabolism Clinics of North America . 2000; 29(1):159-185Three Main QuestionsIs the adrenal mass hormonally active?Is the mass benign or malignant?Does the patient have a history of a previous malignant lesion?Is it metastatic?Ana

4、tomyhttp:/sealion/view_photo.php?set_albumName=album265&id=AdrenalAnatomyhttp:/sealion/view_photo.php?set_albumName=album265&id=AdrenalAnatomyPrimary AldosteronismCushings SyndromeDHEA-sPheochromocytomaFrequency of FindingsMulticenter study of 1096 casesNonfunctioning adenoma: 85%Subclinical Cushing

5、s syndrome: 9%Pheochromocytoma: 4%Aldosteronomas: 2%Mantero et al. 85 (2): 637. (2000) Frequency of FindingsAllolio, B., Adrenal Incidentalomas.Adrenal Disorders, ed. C.G. Margioris AN. 2001, Totowa: Humana Press Inc. A summary of the literatureNonfunctioning adenoma Approximately 80% Subclinical Cu

6、shing syndrome (SCS), 5%Pheochromocytoma 5%Aldosteronoma 1%adrenocortical carcinoma (ACC) 5 % Metastatic lesion 2.5%Ganglioneuromas, myelolipomas,or benign cysts考慮是否手術(shù)治療之前準(zhǔn)確的功能診斷非常必要 嗜鉻細(xì)胞瘤要進(jìn)行認(rèn)真的術(shù)前準(zhǔn)備以避免術(shù)中和術(shù)后的發(fā)作和死亡。原發(fā)性醛固酮增多癥的患者需要明確是否存在腎上腺皮質(zhì)增生及無(wú)功能的腎上腺腺瘤。腎上腺源性Cushing綜合征的患者在行切除術(shù)后可能發(fā)生腎上腺皮質(zhì)功能不全，激素的替代以及增減治療

7、需要非常仔細(xì)。亞臨床Cushing綜合征的患者是否需要手術(shù)治療仍存在爭(zhēng)議。腎上腺皮質(zhì)癌的患者手術(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)商決定切除的方式，因?yàn)槭状吻谐男Ч巧媛实闹饕A(yù)測(cè)因素。超過(guò)4cm的腎上腺無(wú)功能瘤可以考慮切除。小的髓脂肪瘤或良性的囊腫一般影像學(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。 Algorithm for the evaluation and management of an adrenal incidentaloma*Reimage in 3 to 6 months and annually for 1 to 2 years; repe

8、at functional studies annually for 5 years.If mass grows more than 1cm or becomes hormonally active, then adrenalectomy is recommended.Hyperfunctioning Hormonal EvaluationSubclinical Cushings SyndromePheochromocytomaPrimary AldosteronismSex hormone-secreting adrenocortical tumorsSubclinical Cushings

9、 SyndromeHypercortisolism without clinical manifestations of Cushings syndrome Most frequent hormonal abnormality in adrenal incidentalomas Subclinical Cushings SyndromeCentral obesityFacial roundingBuffalo humpEasy bruisingPurple striaeProximal muscle weaknessEmotional/cognitive changesSubclinical

10、Cushings SyndromeIncrease risk for:HypertensionDyslipidemiaImpaired glucose tolerance Type 2 DMAtherosclerosisOsteoporosis?Tauchmanova L, et. al. Patients with subclinical Cushings syndrome due to adrenal adenoma have increase cardiovascular risk. JCEM 2000; 85:1440.Subclinical Cushings SyndromeBioc

11、hemical abnormalitiesElevated urine free cortisolLow or suppressed ACTH Blunted diurnal variation No cortisol suppression after 1 mg overnight dexamethasone suppression test- BEST SCREENING TEST!1. Mantero F, et al. Hormone Res 47:284289, 19972. Montwill J, et al. The O/N DST is the procedure of cho

12、ice for screening for Cushings syndrome. Steroids 1994; 59:2296Dexamethasone Suppression Test1 mg dexamethasone at 11PMMeasure cortisol at 8 AM the next morningNormal: cortisol 30 and PAC 20 ng/dL90% spec and sensitivity for PAIf screening test is positive- need to confirm with saline suppression te

13、st, adrenal venous sampling and imagingmidnight salivary cortisol, or a 2-day low-dose dexamethasone suppression testmidnight salivary cortisol, or a 2-day low-dose dexamethasone suppression testHyperfunctioning Hormonal EvaluationSubclinical Cushings SyndromePheochromocytomaPrimary AldosteronismSex

14、 hormone-secreting adrenocortical tumorsSex hormone-secreting Adrenocortical TumorsRareTypically occur in the presence of clinical manifestations (hirsutism or virilization)HirsutismSex hormone-secreting Adrenocortical TumorsRareTypically occur in the presence of clinical manifestations (hirsutism o

15、r virilization)Routine screening for excess androgens and estrogens is not warrantedHormonal Workup Summary3 hormonal tests necessary for workup of adrenal incidentaloma:1 mg overnight dexamethasone suppresion testPlasma or urinary fractionated metaneprinesPlasma aldosterone concentration and plasma

16、 aldosterone concentration/plasma renin activity ratio (PAC/PRA).TreatmentAll patients with documented pheochromocytoma and primary aldosteronism should undergo surgeryNo prospective, randomized trials for Subclinical Cushings Syndrome but concensus is to proceed with surgery if the patient is young

17、Three Main QuestionsIs the adrenal mass hormonally active?Is the mass benign or malignant?Does the patient have a history of a previous malignant lesion?Is it metastatic?Primary Adrenal CarcinomaVery rare: 5 cases per 1 million populationSmall size corresponds to better prognosis5 year survivalOvera

18、ll: 16%Localized disease (stage I and II): 42%Metastases: 5.3%Imagingcomplex solid and cystic, calcified massPatient with Known Malignancy10-40% of patients with known malignancy have adrenal metastases at autopsyMost common primaryBreastLungKidneyMelanomaLymphomaAssessment of Malignant PotentialSiz

19、eImaging Phenotype (features)SizeProbability of malignancy increases with sizeIn a study involving 887 patients with adrenal incidentalomas, 90% of patients with adrenal carcinomas has tumor 4 cm (National Italian Study Group, 1997)adrenal carcinomas 2%(6cm)SizeMayo Clinic Study342 Patients with adr

20、enal incidentaloma retrospectively evaluatedTumor diameter averaged 2.5 cmMost malignant tumors measured 5 cmIncidentally discovered adrenal tumors: an institutional perspective. Herrera MF; Grant CS; van Heerden JA; Sheedy PF; Ilstrup DM. Surgery 1991 Dec;110(6):1014-21 SizeConsensus StatementMass

21、6 cm should be removedMass 4 cm can be monitoredMass between 4-6 cm: Criteria other than size should be used to dictate surgery vs. monitoringManagement of the clinically inapparent adrenal mass (incidentaloma). NIH State-of-the-Science Conference Statement Feb 4-6, 2002.Assessment of Malignant Pote

22、ntialSizeImaging PhenotypeImage Phenotype- CT ScanHounsfield unit (HU)- semiquantitative method for measuring x-ray attenuationWater= 0 HUAdipose tissue= -20 to -150 HUKidney= 20 to 50 HUBone= 1000 HULipid rich mass are benignHU10 on unenhanced CT= benign adenoma 100%Image Phenotype- CT ScanRetrospe

23、ctive analysis of 151 patients with adrenal massesHU10 or a combination of tumor size 4cm and HU 60% at 10 min= no cancerWash out 60% at 10 min= high risk for malignant lesionImaging- metastasesMRIEqually effective as CTAdenomas are isointense with the liver on T2 weighted imagesCarcinomas are hyperintense compared to the liver on T2 weighted imagesFNACytology from FNA cannot distinguish benign adrenal mass vs. malignantIt can distinguish adrenal tissue from metastasesFNA is useful only in distinguishing