胸部肿瘤致异位ACTH综合征6例分析

目的分析胸部肿瘤致异位ACTH综合征患者的临床特点及治疗,以提高诊治水平。方法回顾性分析2000年1月-2013年1月解放军总医院呼吸科、内分泌科及胸外科收治并经病理确诊的胸部肿瘤致异位ACTH综合征患者6例的临床资料及治疗效果。结果 6例患者中,男5例,女1例,平均年龄(50.5±8.9)岁,病程1~8(4.0±3.6)个月。主要临床表现为低血钾、高血压、糖尿病6例,水肿5例,皮肤瘀斑、皮肤变薄及向心性肥胖4例,满月脸、色素沉着3例。患者血ACTH、血皮质醇及24 h尿游离皮质醇水平升高,皮质醇分泌均不被小/大剂量地塞米松抑制试验所抑制。肺组织病理显示小细胞肺癌3例,胸腺类癌2例,支气管类癌1例。胸腺类癌和支气管类癌手术切除疗效较好,小细胞肺癌合并异位ACTH综合征首选化疗,但预后差。结论胸部肿瘤是异位ACTH综合征的常见原因。临床中如果出现低血钾、高血压、高血糖、水肿等症状,血皮质醇及ACTH水平明显增高要高度怀疑异位ACTH综合征,应及时筛查胸部CT及气管镜检查以确定是否为胸部肿瘤致异位ACTH综合征。

A clinical analysis of 6 cases of the ectopic ACTH syndrome due to thoracic neoplasm

Objective To analyze the clinical features of 6 patients with the ectopic ACTH syndrome （EAS） due to thoracic neoplasm. Methods The 6 patients with EAS due to thoracic neoplasm in Chinese PLA General Hospital were retro- spectively analyzed. Results There were 6 patients, 5 males and 1 female, with an average age of （50.5 ± 8.9 ） years, the disease course was 1- 8 （4.0 ± 3.6） months. The main clinical manifestations included hypokalemia（ n = 6）, hypertension （n = 6） , hyperglycemia （ n =6） , edema （ n =5）. Serum ACTH, cortisol, and 24 h urinary free cortisol levels was in- creased, eortisol secretion was not inhibited by small / large dose dexamethasone suppression test. Histopathology showed small cell lung cancer in 3 cases, thymic earcinoid in 2 cases, bronchial carcinoid in one case. Surgical resection was the first choice of treatment for thymic careinoid and bronchial earcinoid with EAS, and had favorable prognosis. For SCLC with EAS, the first choice was chemotherapy, but associated with poor survival. Conclusion Thoracic neoplasm is the common source of EAS. If patient has hypokalemia, hypertension, hyperglycemia, edema, and remarkable elevated plasma ACTH and plasma cortisol, it should be highly suspected EAS. The patients should be timely screened by chest CT scan and bronchoscopy in or- der to give early diagnosis and treatment.