异位促肾上腺皮质激素综合征的外科治疗

发布时间：2018-04-04 22:17

本文选题：ACTH综合征　切入点：异位　出处：《北京大学学报(医学版)》2017年04期

【摘要】：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。
[Abstract]:Objective: to investigate the diagnosis and surgical treatment of ectopic adrenocorticotrophic hormone (ACTH) syndrome.Methods: the clinical data of 57 cases of ectopic ACTH syndrome treated in Peking Union Hospital from January 1996 to December 2016 were reviewed. There were 32 males and 25 females, aged 1168 years with an average age of 32 years.Serum ACTH was significantly increased (mean 77.6 pmol/L(16.5~365.6 / L, normal value was 10.1 pmol / L). No diffuse or nodular hyperplasia of bilateral adrenal glands was found in pituitary MRI.According to different treatment schemes, 25 patients in group A were divided into 3 groups. There were no ectopic ACTH syndrome, 16 cases were treated with bilateral or unilateral adrenalectomy, 16 cases were found to be treated with radical resection of ectopic tumor and 16 cases were treated with radical resection of tumor.Non-surgical treatment.The curative effect and prognosis of different treatment methods were analyzed.Results 40 of 57 patients were followed up for 6 months to 10 years. The patients in group A had different degrees of illness, different intraoperative conditions and different treatment methods, some of which were bilateral adrenalectomy or only unilateral adrenalectomy.Among them, 4 cases died of diabetes caused by severe Cushing's syndrome (Cushingssyndrome), 18 cases of pulmonary infection have survived and 3 cases have not been visited.The survival rate was 81% 18 / 22%. B group (16 cases) with radical excision of ectopic tumor, 5 cases died of tumor recurrence from 0.5 to 6.0 years after operation, 3 cases survived and 8 cases lost their visit. The survival rate was 37.5% / 8%. 4 cases in group C died of tumor metastasis within 1 year after radiotherapy and chemotherapeutic therapy.All 6 patients with diabetes or pulmonary infection died of pulmonary infection within one year, 6 patients lost their visit and none survived.Conclusion: the treatment of ectopic ACTH syndrome is difficult, target adenectomy is an effective treatment method, especially for Cushing syndrome patients with severe clinical symptoms and the primary tumor can not be located.
【作者单位】：中国医学科学院北京协和医院北京协和医院泌尿外科;
【分类号】：R699.3

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