胸腺肿瘤所致异位促肾上腺皮质激素综合征  

Ectopic ACTH syndrome caused by thymus neoplasms

作　　者：陈野野[1] 李单青[1] 秦应之[1] 黄诚[1] 李力 

机构地区：[1]中国医学科学院 北京协和医学院 北京协和医院胸外科 卫生部内分泌重点实验室,100730

出　　处：《中华临床医师杂志（电子版）》2013年第22期59-62,共4页Chinese Journal of Clinicians(Electronic Edition)

摘　　要：目的探讨胸腺肿瘤导致异位促肾上腺皮质激素(ACTH)综合征的临床特点,提高对该病的认识。方法回顾性分析北京协和医院1987年11月至2012年11月16例由胸腺肿瘤引起的异位ACTH综合征患者的资料。结果 16例患者(男10例,女6例)就诊时的平均年龄为33.8岁(13～59岁),就诊时平均病程为(16.4±22.7)月(1～84个月)。所有患者均存在库欣综合征临床表现,影像学检查尤其是胸部CT是定位肿瘤的重要检查方法。手术治疗后血清皮质醇及血ACTH浓度均显著下降(P值分别为0.000及0.014)。1例围手术期死亡。经综合治疗及随访,中位生存期为35个月。结论胸腺肿瘤是异位ACTH综合征常见原因之一,早期诊断及治疗存在很大难度,应结合临床多种检查手段并多科会诊明确诊断,采用以手术为主的综合治疗方法。Objective To investigate and evaluate the clinical characteristics of ectopic ACTH syndrome （EAS） caused by thymus neoplasms, so as to improve the knowledge of the disease. Methods From 1987-11 to 2012-11 in Peking Union Medical College Hospital, the outcome of surgical intervention and follow-up of consecutive 16 cases of EAS caused by thymus neoplasms were retrospectively analyzed. Results The average age was 33.8（13-59） years old and the duration of disease was （16.4±22.7）months（1-84 months）. All the cases had clinical and biochemical evidences for the diagnosis of Cushing＇s syndrome. Imaging especially thoracic CT was the key point in the tumor localization. The concentration serum cortisol and ACTH was significantly decreased after surgery（P 0.000 and 0.014 respectively）. The median survival time for the series was 35 months by follow-up. Conclusion Thymus neoplasm is one of the most common sources of EAS. To get confirmed diagnosis and therapy in the early days is still very challenging for clinicians. Surgery is the best way to eliminate the source tumor and multidisciplinary therapy should be put into use to these patients.

关 键 词：胸腺肿瘤 ACTH综合征 异位 

分 类 号：R736.3[医药卫生—肿瘤]