肾上腺意外瘤指南adrenal incidentaloma guidelines

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1、Outline,Definition Prevalence Anatomy and Physiology Review Diagnostic Workups Conclusions,Definition,“Mass lesion greater than 1 cm in diameter discovered “accidentally” during a radiographic examination performed for indications other than an evaluation for adrenal disease.”,Management of the clin

2、ically inapparent adrenal mass (incidentaloma). NIH State-of-the-Science Conference Statement Feb 4-6, 2002.,Prevalence,Autopsies: 87,065 cases: 6% with adrenal adenomas Abdominal CT (61,054 CT scans reviewed): 4% with adrenal adenomas Now approaches the 8.7% incidence reported in autopsy series,Inc

3、idence Increases with Age,Endocrine and Metabolism Clinics of North America . 2000; 29(1):159-185,Three Main Questions,Is 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?,Anatomy,http:/shutterbug.uc

4、sc.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,Primary Aldosteronism,Cushings Syndrome,DHEA-s,Pheochromocytoma,Frequency of Findings,Multicenter study of 1096 cases Nonfunctioning aden

5、oma: 85% Subclinical Cushings syndrome: 9% Pheochromocytoma: 4% Aldosteronomas: 2%,Mantero et al. 85 (2): 637. (2000),Frequency of Findings,Allolio, B., Adrenal Incidentalomas. Adrenal Disorders, ed. C.G. Margioris AN. 2001, Totowa: Humana Press Inc.,A summary of the literature,Nonfunctioning 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,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Cush

7、ing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。,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.,Hyperfunctioning Hormonal Evaluation,Subclinical Cushings Syndrome Pheochromocytoma Primary Aldosteronism Sex hormone-sec

9、reting adrenocortical tumors,Subclinical Cushings Syndrome,Hypercortisolism without clinical manifestations of Cushings syndrome Most frequent hormonal abnormality in adrenal incidentalomas,Subclinical Cushings Syndrome,Central obesity Facial rounding Buffalo hump Easy bruising Purple striae Proxima

10、l muscle weakness Emotional/cognitive changes,Subclinical Cushings Syndrome,Increase risk for: Hypertension Dyslipidemia Impaired glucose tolerance Type 2 DM Atherosclerosis Osteoporosis?,Tauchmanova L, et. al. Patients with subclinical Cushings syndrome due to adrenal adenoma have increase cardiova

11、scular risk. JCEM 2000; 85:1440.,Subclinical Cushings Syndrome,Biochemical abnormalities Elevated urine free cortisol Low 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

12、:284289, 1997 2. Montwill J, et al. The O/N DST is the procedure of choice for screening for Cushings syndrome. Steroids 1994; 59:2296,Dexamethasone Suppression Test,1 mg dexamethasone at 11PM Measure cortisol at 8 AM the next morning Normal: cortisol 1.8g/dL (5ug/dl) Specificity of DST is 72-82% (1

13、00%) Sensitivity 75-100% (58%) Severe bipolar depression and severe alcoholism can give false positive results If the DST 8AM serum cortisol is abnormal, then baseline ACTH, serum and 24-hour urinary cortisol should be obtained and midnight salivary cortisol, or a 2-day low-dose dexamethasone suppre

14、ssion test is needed to confirm autonomy,Hyperfunctioning Hormonal Evaluation,Subclinical Cushings Syndrome Pheochromocytoma Primary Aldosteronism Sex hormone-secreting adrenocortical tumors,Pheochromocytoma,Rare but fatal catecholamines producing tumor Incidence: 2-8/million people/year Account for

15、 5% of adrenal incidentaloma Rule of 10s: 10% extra-adrenal, 10% bilateral, 10% familial, 10% malignant Aside from catecholamines, it can also secrete dopamine, ACTH, PTH, calcitonin, VIP,Pheochromocytoma,Classic triads: Sudden severe headache Diaphoresis Palpitations 94% specificity; 91% sensitivit

16、y in hypertensive population,Pheochromocytoma,19-76% of pheo are undiagnosed until after death 80% of patient with unsuspected pheo who underwent surgery or anesthesia will die Although radiographic characteristics can give some clues: Enhancement with IV on CT High signal intensity on T2 weighted MRI Prominent vascularity Thus the need for screening,Imaging,Silent 8 cm pheo,Pheochromocytoma,Available Tests: Plasma fractionated free metanephrines 24-hour urinary fractio

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