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库欣病经蝶窦手术失败后垂体放疗的长期结果。

The long-term outcome of pituitary irradiation after unsuccessful transsphenoidal surgery in Cushing's disease.

作者信息

Estrada J, Boronat M, Mielgo M, Magallón R, Millan I, Díez S, Lucas T, Barceló B

机构信息

Department of Endocrinology, Clínica Puerta de Hierro, Universidad Autónoma, Madrid, Spain.

出版信息

N Engl J Med. 1997 Jan 16;336(3):172-7. doi: 10.1056/NEJM199701163360303.

Abstract

BACKGROUND

Irradiation of the pituitary is widely considered the most appropriate treatment for patients with Cushing's disease in whom transsphenoidal microsurgery has been unsuccessful. However, there is little information about the long-term efficacy of this treatment.

METHODS

We used external pituitary radiation to treat 30 adult patients with persistent or recurrent Cushing's disease after unsuccessful transsphenoidal surgery. The mean (+/-SD) dose of radiation was 50+/-1 Gy. Pituitary and adrenal function was assessed every six months after radiation therapy. Remission was defined as the regression of symptoms and signs of Cushing's syndrome, normal urinary cortisol excretion, and a low plasma cortisol concentration in the morning after the administration of 1 mg of dexamethasone at midnight.

RESULTS

Twenty-five patients (83 percent) had remissions during a median follow-up of 42 months (range, 18 to 114). The remissions began 6 to 60 months after radiation therapy, but in most cases (22 patients) remission occurred during the first 2 years. None of the 25 patients had a relapse of Cushing's disease after remission was achieved. There was no relation between the response to radiotherapy and sex, age, urinary cortisol excretion before radiotherapy, the interval between surgery and radiotherapy, whether a pituitary adenoma was found by pathological examination, or tumor size. Seventeen patients had a deficiency of growth hormone after radiation therapy, 10 had a deficiency of gonadotropins, 4 had a deficiency of thyrotropin, and 1 had a deficiency of corticotropin.

CONCLUSIONS

Pituitary irradiation is an effective and well-tolerated treatment for patients with Cushing's disease in whom transsphenoidal surgery is unsuccessful.

摘要

背景

对于经蝶窦显微手术失败的库欣病患者,垂体照射被广泛认为是最合适的治疗方法。然而,关于这种治疗的长期疗效的信息很少。

方法

我们对30例经蝶窦手术失败后仍患有持续性或复发性库欣病的成年患者进行了垂体外部照射。平均(±标准差)放射剂量为50±1 Gy。放疗后每六个月评估垂体和肾上腺功能。缓解定义为库欣综合征的症状和体征消退、尿皮质醇排泄正常以及午夜给予1 mg地塞米松后次日早晨血浆皮质醇浓度低。

结果

25例患者(83%)在中位随访42个月(范围18至114个月)期间缓解。缓解在放疗后6至60个月开始,但在大多数情况下(22例患者)缓解发生在头两年。25例患者中无一例在达到缓解后库欣病复发。放疗反应与性别、年龄、放疗前尿皮质醇排泄、手术与放疗间隔、病理检查是否发现垂体腺瘤或肿瘤大小之间无关联。17例患者放疗后生长激素缺乏,10例促性腺激素缺乏,4例促甲状腺激素缺乏,1例促肾上腺皮质激素缺乏。

结论

对于经蝶窦手术失败的库欣病患者,垂体照射是一种有效且耐受性良好的治疗方法。

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