目的：探讨异位促肾上腺皮质激素（adrenocorticotrophic hormone，ACTH）综合征的诊断与外科治疗。方法：对1996 年1月至2016年12月北京协和医院收治的异位ACTH综合征57例的临床资料进行总结，其中男32例、女25例，年龄11~68岁，平均32岁。血ACTH显著升高，平均77.6 pmol/L（16.5~365.6 pmol/L，正常值为＜10.1 pmol/L），垂体MRI未见占位，CT检查双侧肾上腺均有不同程度弥漫性或结节样增生。按照不同治疗方案选择分为3组：A组25例，未发现异位肿瘤的异位ACTH 综合征，行双侧或单侧肾上腺切除的手术治疗；B组16例，发现异位肿瘤行肿瘤根治性切除术；C组16例，非手术治疗。分析不同治疗方法疗效及预后。结果：57例患者中有40例随访6个月至10 年，A组因病情凶险程度不同、术中情况各异，采取的治疗方式也有差别，部分为分期双侧肾上腺切除或仅行单侧肾上腺切除术，其中4例死于严重库欣综合征(Cushing’s syndrome)导致的糖尿病、肺部感染，18例存活至今，3例失访，存活率为81%（18/22）。B组16例行异位肿瘤根治性切除者，5例术后0.5~6.0年死于肿瘤复发， 3例存活至今，8例失访，存活率为37.5%（3/8）。C组4例放射疗法加化学药物治疗者1年内均死于肿瘤转移、糖尿病或肺部感染， 6例单纯化学药物治疗者均于1年内死于肺部感染，另6例失访，无1例存活。结论：异位ACTH 综合征的治疗难度大，靶腺切除是有效的治疗方法，尤其适用于库欣综合征临床症状严重而原发肿瘤无法定位者。

Objective： To investigation the diagnosis and treatment of ectopic adrenocorticotrophic hormone (ACTH) syndrome. Methods: The clinical characters of 57 cases of ecotopic ACTH syndrome from Jan. 1996 to Dec. 2016 were collected and analyzed. The 57 cases included 32 males and 25 females. The age ranged from 11 to 68 years ( average 32 years). ACTH levels significantly increased from 16.5 to 365.6 pmol/L, with average 77.6 pmol/L (normal range ＜10.1 pmol/L). The pituitary MRI did not found lesions. The CT showed that their bilateral adrenal glands diffused small nodular changes or nodular hyperplasia. The 57 cases were divided into 3 groups according to different treatment options. In the study, 25 ectopic ACTH syndrome cases (44%) were group A, without identified source of ectopic hormone, were treated with bilateral or unilateral adrenalectomy due to the severity of the disease and difficulty of operation. Group B was composed of 16 cases (28%) diagnosed as ectopic ACTH syndrome by finding ectopic ACTH tumors and surgical resection. Group C included 16 cases (28%) with nonsurgical therapy. Different treatment results and prognosis were analyzed. Results: In the study, 40 cases of the 57 had been followed up for 6 months to 10 years. In group A, of the 25 cases with bilateral or unilateral adrenalectomy, 4 died of diabetes and severe pulmonary infection, 18 survived, and 3 were lost to the follow-up, and the survival rate was 81% (18/22). In group B, of the 16 cases with radical tumor resection, 5 died of tumor recurrence 0.5-6.0 years after operation, 3 survived, and 8 were lost to the follow-up, and the survival rate was 37.5% (3/8). In group C, of the 16 non-operation patients, 4 with radiotherapy and chemotherapy died of metastases, diabetes or pulmonary infection, 6 with chemotherapy died of pulmonary infection within 1 year and the others were lost to the follow-up, and the survival rate was 0. Conclusion: Ectopic ACTH syndrome is difficult to treat. Adrenalectomy is effective for the management of ectopic ACTH syndrome, especially for those patients with severe Cushing’s syndrome, but the primary tumor can not be located.