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目的 探讨异位ACTH综合征的诊断与外科治疗效果。 方法 对 1987年 10月～1999年 5月收治的异位ACTH综合征 12例的临床资料进行总结 ,8例行手术治疗 ,其中 2例首次住院发现异位肿瘤者行肿瘤根治性切除术 ;6例库兴综合征症状严重但未找到异位肿瘤者 ,行双侧腺上腺切除术 ,术后加皮质激素替代治疗。非手术治疗 4例 ,其中放疗加化疗 2例 ,单纯化疗 2例。 结果 12例中 11例随访 6个月～ 10年 ,2例行肿瘤根治性切除者 1例术后 6年死于肿瘤复发 ,1例存活至今。 6例双侧肾上腺切除者中 2例死于全身转移 ,1例死于肺部感染 ,存活期分别为 73、6 2、6 0个月 ;3例存活。非手术治疗 4例中 ,2例放疗加化疗者 2年内均死于肿瘤转移和糖尿病 ,2例单纯化疗者 1例半年内死于肺部感染 ,另 1例失访。 结论 双侧肾上腺切除加皮质激素替代疗法是治疗异位ACTH综合征的有效方法 ,尤其适用于库兴综合征临床症状严重而尚未找到异位肿瘤者。对早期发现异位肿瘤者应行肿瘤根治性切除。
Objective To investigate the diagnosis and surgical treatment of ectopic ACTH syndrome. Methods The clinical data of 12 patients with ectopic ACTH syndrome admitted in our hospital from October 1987 to May 1999 were summarized. Surgical treatment was performed in 8 of them. Among them, 2 patients underwent elective radical resection for the first time in hospital. Cases of Cushing syndrome symptoms are serious but did not find ectopic tumors, bilateral adenosinectomy, postoperative corticosteroid replacement therapy. Non-surgical treatment in 4 cases, including radiotherapy plus chemotherapy in 2 cases, chemotherapy alone in 2 cases. Results Of 12 cases, 11 cases were followed up for 6 months to 10 years. One of 2 patients who underwent radical resection of the tumor died of tumor recurrence 6 years after operation, and one patient survived till now. Two of 6 patients with bilateral adrenalectomy died of systemic metastasis, and one died of lung infection with a survival of 73,6 months and 2,600 months respectively. Three patients survived. Among the 4 nonoperative cases, 2 cases died of tumor metastasis and diabetes within 2 years after radiotherapy and chemotherapy, 1 case of 2 chemotherapy alone died of pulmonary infection within 6 months, and the other 1 case was lost. Conclusions Bilateral adrenalectomy plus corticosteroid replacement therapy is an effective method for the treatment of ectopic ACTH syndrome, especially for patients with severe clinical symptoms and no ectopic tumors found in Cushing’s syndrome. On the early detection of ectopic tumors should be radical tumor resection.