异位ACTH综合征的诊断与外科治疗

The diagnosis and treatment of ectopic ACTH syndrome

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 study the diagnosis and treatment of ectopic ACTH syndrome. Methods The clinical characters of 12 cases of isotropic ACTH syndrome treated from October 1987 to May 1999 have been analyzed.In the 8 patients underwent open surgery 2 were diagnosed isotropic ACTH syndrome on hospitalization underwent radical tumor resection.6 cases with severe symptom of Cushing's Syndrome,in which the isotropic tumor could not be found,had bilateral adrenorectomy with postoperative replacement of cortical hormone.In the 4 non operated patients,2 were treated with radiotherapy and chemotherapy and 2 with chemotherapy only. Results Eleven cases of the 12 had been followed up for 6 months to 10 years.In the 2 with radical tumor resection,one died of recurrence 6 years after operation and the other one survived.In the 6 cases with bilateral adrenorectomy,2 died of extensive metastases and one died of pulmonary infection.The survival periods were 73,62 and 60 months respectively.Of the 4 non operaed patients,2 with radiotherapy and chemotherapy died of metastases and diabetes.On of the two cases with chemotherapy died of pulmonary infection and the other was lost of follow up. Conclusions Bilateral adrenalectomy with hormonal replacement is effective for the management of ectopic ACTH syndrome especially for those with severe Cushing's syndrome and no tumor mass has been detected.Radical excision of the tumor should be undertaken in case an ectopic tumor has been detected.