腎上腺意外瘤指南AdrenalIncidentalomaguidelines課件

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

2、for adrenal disease.”Management of the clinically inapparent adrenal mass (incidentaloma). NIH State-of-the-Science Conference Statement Feb 4-6, 2002.精選課件PrevalenceAutopsies: 87,065 cases: 6% with adrenal adenomasAbdominal CT (61,054 CT scans reviewed): 4% with adrenal adenomasNow approaches the 8.

3、7% incidence reported in autopsy series 精選課件Incidence Increases with AgeEndocrine and Metabolism Clinics of North America . 2000; 29(1):159-185精選課件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?

4、Is it metastatic?精選課件Anatomy/sealion/view_photo.php?set_albumName=album265&id=Adrenal精選課件Anatomy/sealion/view_photo.php?set_albumName=album265&id=Adrenal精選課件AnatomyPrimary AldosteronismCushings SyndromeDHEA-sPheochromocytoma精選課件Frequency of FindingsMulticenter study of 1096 casesNonfunctioning adeno

5、ma: 85%Subclinical Cushings syndrome: 9%Pheochromocytoma: 4%Aldosteronomas: 2%Mantero et al. 85 (2): 637. (2000) 精選課件Frequency of FindingsAllolio, B., Adrenal Incidentalomas.Adrenal Disorders, ed. C.G. Margioris AN. 2001, Totowa: Humana Press Inc. 精選課件A summary of the literatureNonfunctioning adenom

6、a Approximately 80% Subclinical Cushing syndrome (SCS), 5%Pheochromocytoma 5%Aldosteronoma 1%adrenocortical carcinoma (ACC) 5 % Metastatic lesion 2.5%Ganglioneuromas, myelolipomas,or benign cysts精選課件考慮是否手術(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)增生及無功能的腎上腺腺瘤。腎上腺源性Cushin

7、g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，因為首次切除的效果是生存率的主要預(yù)測因素。超過4cm的腎上腺無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。 精選課件Algorithm for the evaluation and management of an adrenal incidentaloma*Reimage in 3 to 6

8、months 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.精選課件Hyperfunctioning Hormonal EvaluationSubclinical Cushings SyndromePheochromocytomaPrimary AldosteronismSex hormone-secre

9、ting adrenocortical tumors精選課件Subclinical Cushings SyndromeHypercortisolism without clinical manifestations of Cushings syndrome Most frequent hormonal abnormality in adrenal incidentalomas 精選課件Subclinical Cushings SyndromeCentral obesityFacial roundingBuffalo humpEasy bruisingPurple striaeProximal

10、muscle weaknessEmotional/cognitive changes精選課件Subclinical 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

11、risk. JCEM 2000; 85:1440.精選課件Subclinical Cushings SyndromeBiochemical 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,

12、 19972. Montwill J, et al. The O/N DST is the procedure of choice for screening for Cushings syndrome. Steroids 1994; 59:2296精選課件Dexamethasone 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 scre

13、ening test is positive- need to confirm with saline suppression test, adrenal venous sampling and imaging精選課件midnight salivary cortisol, or a 2-day low-dose dexamethasone suppression testmidnight salivary cortisol, or a 2-day low-dose dexamethasone suppression test精選課件精選課件Hyperfunctioning Hormonal E

14、valuationSubclinical Cushings SyndromePheochromocytomaPrimary AldosteronismSex hormone-secreting adrenocortical tumors精選課件Sex hormone-secreting Adrenocortical TumorsRareTypically occur in the presence of clinical manifestations (hirsutism or virilization)精選課件Hirsutism精選課件Sex hormone-secreting Adreno

15、cortical TumorsRareTypically occur in the presence of clinical manifestations (hirsutism or virilization)Routine screening for excess androgens and estrogens is not warranted精選課件Hormonal Workup Summary3 hormonal tests necessary for workup of adrenal incidentaloma:1 mg overnight dexamethasone suppres

16、ion testPlasma or urinary fractionated metaneprinesPlasma aldosterone concentration and plasma aldosterone concentration/plasma renin activity ratio (PAC/PRA).精選課件TreatmentAll patients with documented pheochromocytoma and primary aldosteronism should undergo surgeryNo prospective, randomized trials

17、for Subclinical Cushings Syndrome but concensus is to proceed with surgery if the patient is young精選課件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?精選課件Primary Adrenal Carcinom

18、aVery rare: 5 cases per 1 million populationSmall size corresponds to better prognosis5 year survivalOverall: 16%Localized disease (stage I and II): 42%Metastases: 5.3%精選課件Imagingcomplex solid and cystic, calcified mass精選課件Patient with Known Malignancy10-40% of patients with known malignancy have ad

19、renal metastases at autopsyMost common primaryBreastLungKidneyMelanomaLymphoma精選課件Assessment of Malignant PotentialSizeImaging Phenotype (features)精選課件SizeProbability of malignancy increases with sizeIn a study involving 887 patients with adrenal incidentalomas, 90% of patients with adrenal carcinom

20、as has tumor 4 cm (National Italian Study Group, 1997)adrenal carcinomas 2%(6cm)精選課件SizeMayo Clinic Study342 Patients with adrenal incidentaloma retrospectively evaluatedTumor diameter averaged 2.5 cmMost malignant tumors measured 5 cmIncidentally discovered adrenal tumors: an institutional perspect

21、ive. Herrera MF; Grant CS; van Heerden JA; Sheedy PF; Ilstrup DM. Surgery 1991 Dec;110(6):1014-21 精選課件SizeConsensus StatementMass 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

22、 inapparent adrenal mass (incidentaloma). NIH State-of-the-Science Conference Statement Feb 4-6, 2002.精選課件Assessment of Malignant PotentialSizeImaging Phenotype精選課件Image Phenotype- CT ScanHounsfield unit (HU)- semiquantitative method for measuring x-ray attenuationWater= 0 HUAdipose tissue= -20 to -

23、150 HUKidney= 20 to 50 HUBone= 1000 HULipid rich mass are benignHU10 on unenhanced CT= benign adenoma 100%精選課件Image Phenotype- CT ScanRetrospective 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

24、 malignant lesion精選課件Imaging- metastases精選課件MRIEqually effective as CTAdenomas are isointense with the liver on T2 weighted imagesCarcinomas are hyperintense compared to the liver on T2 weighted images精選課件FNACytology from FNA cannot distinguish benign adrenal mass vs. malignantIt can distinguish adrenal tissue from metastasesFNA is useful only in distinguishing adrenal tumor from metastasis and in