Department of Endocrinology, St. Bartholomew's Hospital and the London School of Medicine, London, United Kingdom.
Endocr Pract. 2012 Nov-Dec;18(6):e153-7. doi: 10.4158/EP12136.CR.
To outline the potential role for adrenal venous sampling in the diagnosis and management of adrenocorticotropic hormone (ACTH)-independent Cushing syndrome (CS).
We present a case description and discuss the management of a 59-year-old woman with an 8-year history of weight gain, centripetal obesity, a round plethoric face, skin thinning, easy bruising, hirsutism, and progressive muscle weakness.
The patient reported a prior personal history of asthma, type 2 diabetes mellitus, hypertension, dyslipidemia, and bilateral leg ulcers, but she denied having any personal or family history of endocrinopathy and was not taking any corticosteroid medication. Elevated midnight serum cortisol, failure to suppress cortisol levels with a low-dose dexamethasone suppression test, and undetectable plasma ACTH all indicated ACTH-independent CS. Additional investigations including dynamic tests and adrenal imaging were supported by adrenal venous sampling in order to make a diagnosis and formulate a management plan. She was ultimately noted to have bilateral functioning adrenal nodules (adenoma and adenolipoma) and underwent successful bilateral laparoscopic adrenalectomy, with postoperative glucocorticoid and mineralocorticoid replacement.
Adrenal venous sampling may be an important step in the differential diagnosis of CS and localization of the source of cortisol excess. It may distinguish pheochromocytoma or benign nonfunctioning adrenal nodules from cortisol-secreting adenomas and may avoid unnecessary bilateral adrenalectomy. It can also ensure that the correct operation is completed, if required, and thus avoid the increased morbidity and mortality associated with repeated surgical interventions.
概述肾上腺静脉采样在促肾上腺皮质激素（ACTH）非依赖性库欣综合征（CS）的诊断和治疗中的潜在作用。
我们介绍了一个病例描述，并讨论了一位 59 岁女性的治疗管理，该女性有 8 年的体重增加、向心性肥胖、圆胖的面容、皮肤变薄、容易瘀伤、多毛症和进行性肌肉无力病史。
患者报告有哮喘、2 型糖尿病、高血压、血脂异常和双侧腿部溃疡的既往个人病史，但否认有内分泌病史家族史，也未服用任何皮质类固醇药物。午夜血清皮质醇升高、小剂量地塞米松抑制试验不能抑制皮质醇水平、以及血浆 ACTH 无法检测，均提示为 ACTH 非依赖性 CS。包括动态检查和肾上腺影像学在内的其他检查均支持肾上腺静脉采样，以做出诊断和制定治疗计划。最终，她被发现有双侧功能性肾上腺结节（腺瘤和腺脂肪瘤），并成功接受了双侧腹腔镜肾上腺切除术，术后进行糖皮质激素和盐皮质激素替代治疗。
肾上腺静脉采样可能是 CS 鉴别诊断和定位皮质醇过多来源的重要步骤。它可以区分嗜铬细胞瘤或良性无功能肾上腺结节与分泌皮质醇的腺瘤，并可避免不必要的双侧肾上腺切除术。它还可以确保在需要时完成正确的手术，从而避免与反复手术干预相关的发病率和死亡率增加。
