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目的总结异位ACTH综合征的临床表现与检查,分析手术治疗的效果与经验。方法回顾16例确诊的胸腔内肿瘤致异位ACTH综合征病人的临床资料、手术方式以及预后情况。结果所有病人均有典型柯兴综合征的症状,均存在胸腔内肿瘤;按肿瘤手术原则予以根治切除肿瘤;术后血糖升高、低血钾均得以纠正,血ACTH、血皮质醇、24 h尿游离皮质醇均显著下降;术后出现病情反复多为肿瘤远处转移或复发。结论对存在柯兴综合征症状的病人应注意检查胸腔内有无异位分泌的肿瘤灶,根治手术的近期效果十分显著,对纵隔肿瘤复发病人,再次手术可能是较好的选择。
Objective To summarize the clinical manifestation and examination of ectopic ACTH syndrome and analyze the effect and experience of surgical treatment. Methods The clinical data, operation methods and prognosis of 16 patients with ectopic ACTH syndrome were retrospectively reviewed. Results All patients had the typical symptoms of Cushing’s syndrome. All of them had intrathoracic tumors. According to the principle of tumor surgery, the tumors were removed. The postoperative elevated blood glucose and hypokalemia were corrected. Blood ACTH, cortisol, 24 h Urinary free cortisol were significantly decreased; postoperative recurrence of the disease mostly distant metastasis or recurrence. Conclusions Patients with Cushing ’s syndrome should pay attention to examine the presence or absence of ectopic secretion in the chest cavity. The immediate effect of radical surgery is very significant. Reoperation may be a better choice for patients with recurrent mediastinal tumors.