20例异位ACTH综合征的诊断和治疗  

Diagnosis and treatment of 20 cases of ectopic ACTH syndrome

作　　者：陆召麟[1] 郭爱丽[1] 张洁萍[1] 曹琰[1] 王月[1] 宋文英[1] 史轶蘩[1] 张志庸[1] 李泽坚[1] 刘鸿瑞[1] 刘彤华[1] 严洪珍[1] 张涛[1] 周觉初[1] 何家琳[1] 

机构地区：[1]中国医学科学院

出　　处：《中华内分泌代谢杂志》1999年第6期331-334,共4页Chinese Journal of Endocrinology and Metabolism

摘　　要：目的　总结经验，以改进对异位ＡＣＴＨ 综合征的诊治。方法　对本院１９８６ ～１９９６ 年间收治的２０ 例异位ＡＣＴＨ 综合征进行临床分析。结果　所有病例临床和生化测定均符合库欣综合征的诊断。临床上，本病低血钾碱中毒、水肿、肌萎缩和皮肤色素沉着比较明显。大剂量地塞米松抑制试验７５ ％ 病例不被抑制，血ＡＣＴＨ／ＮＰＯＭＣ水平较高，但这些与垂体性库欣综合征（库欣病）有重叠。影像检查发现肿瘤是关键。本组２０ 例中１８ 例肿瘤在胸部。１５ 例进行病理检查都证实为神经内分泌肿瘤，其中９ 例免疫组化染色ＡＣＴＨ 阳性。手术切除肿瘤及转移瘤是治疗之首选，对未能切除或未完全切除的可进行局部放疗。结论　鉴别诊断很困难，肿瘤定位非常重要。手术治疗是首选，疗效与肿瘤类型有关。Objective To improve the diagnosis and treatment of the ectopic ACTH syndrome. Methods Clinical data of 20 cases of ectopic ACTH syndrome were analysed in this hospital in the period of 1986～1996. Results All the cases had clinical and biochemical evidences for the diagnosis of Cushing's syndrome. Hypokalemic alkalosis, edema, myoatrophy and hyperpigmentation were significant. Large dose dexamethasone inhibition test and measurement of plasma ACTH level were more useful as compared with other methods. Imaging was the key point in the tumor localization. Chest X ray was important, because the tumors in 18 out of 20 cases were located in chest. 15 cases had pathological evidences of ectopic ACTH secretion, and 9 of these were ACTH positive by immunohistochemical assay. Surgical resection was the first choice of treatment. Local irradiation was also useful for metastatic cases. Conclusion It was difficult in differentiation diagnosis and multi methods should be considered. Localization of tumor is of uttermost importance. Surgical resection was the first choice of treatment.

关 键 词：ACTH综合征 异位性 诊断 治疗 

分 类 号：R586.9[医药卫生—内分泌]