Porterfield John R, Thompson Geoffrey B, Young William F, Chow John T, Fryrear Raymond S, van Heerden Jon A, Farley David R, Atkinson John L D, Meyer Fredric B, Abboud Charles F, Nippoldt Todd B, Natt Neena, Erickson Dana, Vella Adrian, Carpenter Paul C, Richards Melanie, Carney J Aidan, Larson Dirk, Schleck Cathy, Churchward Marilyn, Grant Clive S
Department of Surgery, Mayo Clinic and Mayo Foundation, 200 First Street, S.W, Rochester, MN 55905, USA.
World J Surg. 2008 May;32(5):659-77. doi: 10.1007/s00268-007-9387-6.
Cushing's syndrome (CS), due to multiple etiologies, is a disorder associated with the ravages of cortisol excess. The purpose of this review article is to provide a historical synopsis of surgery for CS, review a recent 10-year period of operative management at a tertiary care facility, and to outline a practical approach to diagnosis and management.
From 1996 to 2005, 298 patients underwent 322 operative procedures for CS at Mayo Clinic, Rochester, Minnesota. A retrospective chart review was carried out. Data was gathered regarding demographics, preoperative assessment, procedures performed, and outcomes. Data are presented as counts and percentages. Five-year survival rates were calculated where applicable by the Kaplan-Meier method. Statistical analysis was carried out with SAS, version 9 (SAS Institute, Inc., Cary, NC).
Two-hundred thirty-one patients (78%) had ACTH-dependent CS and 67 patients (22%) had ACTH-independent CS. One-hundred ninety-six patients (66%) had pituitary-dependent CS and 35 patients (12%) had ectopic ACTH syndrome. Fifty-four patients (18%) had cortisol-secreting adenomas, 10 patients (3%) had cortisol-producing adrenocortical carcinomas, and 1% had other causes. Cure rates for first time pituitary operations (transsphenoidal, sublabial, and endonasal) were 80% and 55% for reoperations. Most benign adrenal processes could be managed laparoscopically. Five-year survival rates (all causes) were 90%, 51%, and 23% for adrenocortical adenomas, ectopic ACTH syndrome, and adrenocortical carcinomas, respectively.
Surgery for CS is highly successful for pituitary-dependent CS and most ACTH-independent adrenal causes. Bilateral total adrenalectomy can also provide effective palliation from the ravages of hypercortisolism in patients with ectopic ACTH syndrome and for those who have failed transsphenoidal surgery. Unfortunately, to date, adrenocortical carcinomas are rarely cured. Future successes with this disease will likely depend on a better understanding of tumor biology, more effective adjuvant therapies and earlier detection. Clearly, IPSS, advances in cross-sectional imaging, along with developments in transsphenoidal and laparoscopic surgery, have had the greatest impact on today's management of the complex patient with CS.
库欣综合征（CS）病因多样，是一种与皮质醇过量危害相关的疾病。本文献综述的目的是提供CS手术治疗的历史概要，回顾一家三级医疗机构近10年的手术治疗情况，并概述诊断和管理的实用方法。
1996年至2005年，明尼苏达州罗切斯特市梅奥诊所的298例患者接受了322例CS手术。进行了回顾性病历审查。收集了有关人口统计学、术前评估、所实施手术及结果的数据。数据以计数和百分比形式呈现。适用时采用Kaplan-Meier方法计算五年生存率。使用SAS 9版软件（SAS Institute, Inc., Cary, NC）进行统计分析。
231例患者（78%）患有促肾上腺皮质激素（ACTH）依赖性CS，67例患者（22%）患有ACTH非依赖性CS。196例患者（66%）患有垂体依赖性CS，35例患者（12%）患有异位ACTH综合征。54例患者（18%）患有分泌皮质醇的腺瘤，10例患者（3%）患有产生皮质醇的肾上腺皮质癌，1%为其他病因。首次垂体手术（经蝶窦、唇下和鼻内）的治愈率为80%，再次手术的治愈率为55%。大多数良性肾上腺疾病可通过腹腔镜治疗。肾上腺皮质腺瘤、异位ACTH综合征和肾上腺皮质癌的五年全因生存率分别为90%、51%和23%。
CS手术对于垂体依赖性CS和大多数ACTH非依赖性肾上腺病因非常成功。双侧肾上腺全切术也可为异位ACTH综合征患者以及经蝶窦手术失败的患者有效缓解高皮质醇血症的危害。不幸的是，迄今为止，肾上腺皮质癌很少能治愈。未来攻克这种疾病可能取决于对肿瘤生物学的更好理解、更有效的辅助治疗以及早期检测。显然，岩下窦采血、横断面成像技术的进步以及经蝶窦和腹腔镜手术的发展，对当今复杂CS患者的管理产生了最大影响。
