摘要
目的探讨支气管肺类癌致异位促肾上腺皮质激素(adrenocorticotropic hormone,ACTH)综合征的疾病特点、诊治方法及预后情况。方法选取2007年3月至2016年5月间经北京协和医院诊治并由手术病理证实的支气管肺类癌引起的异位ACTH综合征20例,其中男13例、女7例。搜集并整理20例患者的重要临床数据,并进行统计分析。结果 20例患者就诊时的平均年龄为13～70(38.85±15.31)岁,其中典型类癌14例、不典型类癌6例。所有患者均存在库欣综合征的典型临床表现,均由胸部CT定位胸部病变。手术治疗后血清皮质醇及血ACTH浓度均明显下降。术后并发症3例(15.00%),均为严重肺部感染。随访18例患者,中位随访时间31.5(12～122)个月;其中典型类癌13例、不典型类癌5例;Ⅰa期7例、Ⅰb期1例、Ⅱa期2例、Ⅲa期8例;1例行术后放疗、4例行术后化疗。随访时间内,1例复发转移,无死亡病例。结论支气管肺类癌所致异位ACTH综合征是一种极为罕见的疾病,需要多种检查手段及多专科医师共同诊治;胸部CT是发现病变并评估手术可行性的重要方法,早期手术切除异位ACTH肿瘤是治疗该病的有效手段,且大多可获长期生存。
Objective To investigate the clinical features, diagnosis, treatment and prognosis of ectopic adrenocorticotropic hormone（ACTH） syndrome caused by bronchopulmonary carcinoid. Methods Twenty patients（13 males and 7 females） with ectopic ACTH syndrome caused by bronchopulmonary carcinoid confirmed by surgical pathology were selected from March 2007 to May 2016 in our hospital. We collected and arranged the important clinical data of 20 patients and analyzed carefully. Results The mean age of 20 patients including 14 patients of typical carcinoid and 6 patients of atypical carcinoid was 38.85±15.31 years ranged 13-70 years. All patients had the typical clinical manifestation of Cushing＇s syndrome. All the thoracic lesions were located by thoracic computed tomography（CT）eventually. The concentration of serum cortisol and ACTH significantly decreased after surgery. Postoperative complications in 3 patients（15.00%） were severe pulmonary infection. Eighteen patients including 13 patients of typical carcinoid and 5 patients of atypical carcinoid were followed up. The median time of follow-up was 31.5（12-122） months.There were 7 patients of stage Ⅰa, 1 patient of stage Ⅰb, 2 patients of stage Ⅱa and 8 patients of stage Ⅲa in the patients followed up. One patient underwent postoperative radiotherapy, and 4 patients with postoperative chemotherapy. During the follow-up period, one patient relapsed and there was no death. Conclusion Ectopic ACTH syndrome caused by bronchopulmonary carcinoid is a very rare disease. It is every difficult for clinicians to make early diagnosis and draw up plans of treatment without multidisciplinary collaboration. Chest CT is an important method of finding lesions and assessing the viability of surgery. Surgical treatment in early stage will be effective for this disease. And almost all of patients can achieve long-term survival after complete resection of tumors early.
作者
王学斌
刘磊
王桂阁
张家齐
陈野野
曹磊
刘洪生
李单青
WANG Xuebin;LIU Lei;WANG Guige;ZHANG Jiaqi;CHEN Yeye;CAO Lei;LIU Hongsheng;LI Shanqing(Department of Thoracic Surgery,Peking Union Medical College Hospital,Chinese Academy of Medical Science,Beijing,100730,P.R.China)
出处
《中国胸心血管外科临床杂志》
CAS
CSCD
2018年第11期942-948,共7页
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery
基金
中国医学科学院医学与健康科技创新工程(2017-I2M-1-009)
关键词
支气管肺类癌
异位促肾上腺皮质激素综合征
诊断
手术治疗
预后
Bronchopulmonary carcinoid
ectopic adrenocorticotropic hormone syndrome
diagnosis
surgery
prognosis
