胸部神经内分泌肿瘤所致异位促肾上腺皮质激素综合征诊疗及预后

摘要

目的 探讨胸部神经内分泌肿瘤所致异位促肾上腺皮质激素综合征(ectopic adrenocorticotropic hormone syndrome,EAS)的手术诊治经验,并分析相关预后因素.方法 1983年12月至2013 年12月北京协和医院收治并经手术病理证实的胸部神经内分泌肿瘤引起的EAS 33例,其中男性20例,女性13 例,回顾其临床表现、影像学资料、手术为主的综合治疗方案、围手术期并发症及随访情况,计算生存期并分析相关的预后因素. 结果 33例患者就诊时中位年龄33岁(13~65岁),中位病程12个月(1~156个月).所有患者均存在库欣综合征的临床表现,均由胸部CT定位病变来源.手术治疗后血清皮质醇及促肾上腺皮质激素浓度明显下降(P<0.05).围手术期死亡1例(1/33,3.0%),术后出现并发症2例(2/33,6.1%).33例患者5年存活率为65.8%,其中病变来源于肺部患者的5年存活率明显优于病变来源于胸腺患者(84.2%比36.3%,P<0.05).类癌与不典型类癌5年存活率分别为76.2%和45.7%(P=0.056);Ⅰ-Ⅱ期病变与Ⅲ-Ⅳ期病变5年存活率分别为80.0%和50.5%(P=0.072).结论 胸部神经内分泌肿瘤所致EAS早期诊断及治疗存在较大难度,胸部CT是病变定位的最佳方法,肺部病变预后明显优于胸腺来源病变,病理类型及分期可能是影响其预后的因素.%Objective To discuss the surgical management and prognosis factors of ectopic adrenocorticotropic hormone syndrome (EAS) caused by thoracic neuroendocrine tumors(NETs).Methods The clinical manifestations,radiological findings,outcome of surgical treatment and follow-up of consecutive 33 cases(20 males and 13 females) of EAS caused by thoracic NETs from December 1983 to December 2013 in Peking Union Medical College Hospital were retrospectively analyzed.Results The median age was 33 years old (13~65 years)and the median duration of disease was 12 months(1~156 months).Cushing`s syndrome was demonstrated in all cases by clinical and biochemical evidences.All the thoracic NETs were located by computed tomography (CT) eventually.The serum cortisol and adrenocorticotropic hormone levels decreased significantly after surgery(both P<0.05).One patient(3.0%) died after surgery and two patients(6.1%) had postoperative complications.The overall 5-year survival rate was 65.8%.Pulmonary diseases had better survival rate versus thymic diseases(84.2% vs.36.3%,P<0.05).The 5-year survival rate was 76.2% in typical carcinoid and 45.7% in atypical carcinoid(P=0.056).The 5-year survival rate was 80.0% in stage I and Ⅱ,50.5% in stage Ⅲ and Ⅳ respectively(P=0.072).Conclusions Due to the varity and atypical clinical features,early diagnosis and therapy in EAS is very challenging for clinicians.CT scan plays a key role in localization of the thoracic NETs.Pulmonary diseases have better prognosis than thymic diseases.Pathological type and stage of tumors might be prognostic factors for this rare syndrome.