CAJ | 학술논문

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。
목적：탐토흉선종류도치이위촉신상선피질격소（ACTH）종합정적림상특점，제고대해병적인식。방법회고성분석북경협화의원1987년11월지2012년11월16례유흉선종류인기적이위ACTH종합정환자적자료。결과16례환자（남10례，녀6례）취진시적평균년령위33.8세（13～59세），취진시평균병정위（16.4±22.7）월（1～84개월）。소유환자균존재고흔종합정림상표현，영상학검사우기시흉부CT시정위종류적중요검사방법。수술치료후혈청피질순급혈ACTH농도균현저하강（P치분별위0.000급0.014）。1례위수술기사망。경종합치료급수방，중위생존기위35개월。결론흉선종류시이위ACTH종합정상견원인지일，조기진단급치료존재흔대난도，응결합림상다충검사수단병다과회진명학진단，채용이수술위주적종합치료방법。
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.