肺部病变所致异位促肾上腺皮质激素综合征的临床诊治经验

目的探讨肺部病变导致异位促肾上腺皮质激素(ACTH)综合征的临床诊治经验。方法回顾性分析1985年9月至2013年5月间经手术病理证实的肺部病变引起的异位ACTH综合征20例患者的资料。结果 20例患者中,男性12例,女性8例。就诊时的年龄13⁶5岁,平均年龄为37.2岁。确诊时病程为165岁,平均年龄为37.2岁。确诊时病程为1¹56个月。所有患者均存在异位ACTH综合征的临床表现,均由胸部计算机断层扫描(CT)定位肺部病变。手术治疗后血清皮质醇(P<0.001)及血ACTH浓度(P=0.001)均明显下降。无围手术期死亡病例。平均随访时间47.9个月,暂无复发及死亡病例。结论肺部病变是导致异位ACTH综合征的最常见原因,主要为肺部神经内分泌肿瘤,且以类癌为最常见,诊断及治疗均存在较大难度,CT是病变定位的最佳方法,早期发现并行手术切除病灶是治疗的关键。

Clinical experience of diagnosis and treatment of ectopic adrenocorticotropic hormone syndrome caused by lung diseases

Objective To investigate clinical experience of diagnosis and treatment of ectopic adrenocorticotropic hormone （ACTH） syndrome （EAS） caused by lung diseases. Methods From September 1985 to May 2013 in Peking Union Medical College Hospital, the outcome of surgical procedures and followup of consecutive 20 cases of EAS caused by lung diseases were retrospectively analyzed. Results The average age was 37.2 （ 13 -65） years old and the duration of disease was （28.8 ±37.0） months （ 1 - 84 months）. All the cases had clinical and biochemical evidences for the diagnosis of Cushing＇s syndrome. All the pulmonary sources were located by thoracic computed tomography （CT） eventually. The concentration serum cortisol and ACTH was significantly decreased after surgery （P 〈 0. 001 and P = O. 001 respectively） with no perioperative death. After muhidisciplinary therapy, the median follow-up time was （47.9 ± 40. 2 ） mo and got no relapse or death cases. Conclusion Lung diseases are the most common sources of EAS. To get confirmed diagnosis and therapy in the early days is still very challenging for clinicians. Thoracic CT scan is the key point to locate the sources. Surgery is the best way to eliminate the disease and to get favourable prognosis.