支气管肺类癌导致的异位ACTH综合征

目的通过对支气管肺类癌引起的异位ACTH综合征患者的临床资料分析，总结其临床特点，以提高对该病的认识和诊治水平。方法回顾性分析北京协和医院1984～2009年经病理证实的13例支气管肺类癌引起的异位ACTH综合征患者的临床资料。结果13例患者就诊时的平均年龄35．4岁，就诊时的病程为19．9±18．3个月（1．3～60个月），从初诊至发现肺部占位的时间为15．6±36．1个月（1．0—132个月）。11例（84．6％）患者存在低血钾（2．60±0．61mmol／L）。4例（30．8％）患者大剂量地塞米松抑制试验不被抑制。11例（91．7％）患者初诊时进行了肺CT检查发现了肺部病变。肿瘤平均直径为2．1cm。结论支气管肺类癌是导致隐性异位ACTH综合征的常见原因，但在临床上有时与库欣病很难鉴别。因此对于ACTH依赖的库欣综合征应常规筛查肺部病变，对可疑病例进行全面检查以明确肿瘤定位。

Ectopic ACTH syndrome due to bronchopulmonary carcinoid tumor

Objective To investigate the clinical and biological features of bronchopulmonary carcinoid tumor associated Cushing’s syndrome.Methods The clinical data of 13 patients with ACTH-dependent Cushing’s syndrome with bronchopulmonary carcinoid tumor were reviewed retrospectively.Results The average age was 35.2(23 - 64) years old and all the patients showed typical manifestations of Cushing’s syndrome.The duration of disease at initial presentation was 19.9±18.3months(1.3 - 60months).Hypokalemia 2.60±0.61mmol/L was observed in 11 patients.High - dose dexamethasone suppression test revealed suppressible in 4 cases(30.8%).Pituitary microadenoma was detected in 3 patients (23.1%).Lung lesions were identified in 11 of 12 patients who underwent chest CT during the initial presentation. The average diameter of the pulmonary tumor was 2.1 cm.Before pulmonary tumor resection,4 patients received hypophysectomy, pituitary irradiation,or adrenalectomy.Conclusions Bronchopulmonary carcinoid tumor is a common source of occult ectopic ACTH syndrome,but may be difficulf to be differentiated with Cushing’s disease.ACTH-dependent Cushing’s syndrome should be routinely screened,and suspected tumors be carefully localized.