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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13~65岁,平均年龄为37.2岁。确诊时病程为1~156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。
Objective To investigate the clinical diagnosis and treatment of pulmonary adrenocorticotrophic hormone (ACTH) syndrome. Methods The data of 20 patients with ectopic ACTH syndrome caused by pathologically confirmed lung lesions from September 1985 to May 2013 were retrospectively analyzed. Results Of the 20 patients, 12 were males and 8 were females. At the age of 13 to 65 years old, the average age was 37.2 years old. The duration of diagnosis of 1 to 156 months. All patients had the clinical manifestations of ectopic ACTH syndrome, all of which were located by chest computed tomography (CT). Serum cortisol (P <0.001) and blood ACTH concentration (P = 0.001) after operation were significantly decreased. No perioperative deaths. The average follow-up time was 47.9 months, with no recurrence or death. Conclusions Pulmonary lesion is the most common cause of ectopic ACTH syndrome, which is mainly pulmonary neuroendocrine tumors. Carcinoid carcinoma is the most common type. Pulmonary lesions are more difficult to diagnose and treat. CT is the best method to locate the lesions. Early detection of concurrent surgical removal of lesions is the key to treatment.