Cushing's syndrome due to ectopic corticotropin secretion: Twenty years' experience at the National Institutes of Health
Cushing's syndrome due to ectopic corticotropin secretion: Twenty years' experience at the National Institutes of Health
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DOI：
10.1210/jc.2004-2527
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发表时间：
2005-08-01
影响因子：
5.8
通讯作者：
Nieman, LK
中科院分区：
文献类型：
--
作者：
Ilias, I;Torpy, DJ;Nieman, LK
背景：异位 ACTH 分泌 (EAS) 很难诊断和治疗。我们介绍 1983 年至 2004 年的 EAS 经验。地点：该研究在三级护理临床研究中心进行。患者：研究中包括 90 名患者，年龄 8-72 岁，其中包括 48 名女性。干预和结果测量：测试包括 8 mg 地塞米松抑制、CRH 刺激、岩下窦取样 (IPSS)、计算机断层扫描、奥曲肽 扫描、磁共振成像和/或静脉采样。记录治疗、病理检查和生存情况。结果：86% 至 94% 的患者对 CRH 或地塞米松抑制没有反应，而 67 例中的 66 例 IPSS 呈阴性。为了控制皮质醇增多症，62 名患者接受了药物治疗，其中 33 名患者接受了双侧肾上腺切除术。对 90 名患者中的 67 名进行局部肿瘤成像。手术证实 66 名患者中有 59 名患有 ACTH 分泌肿瘤，治愈率达 65%。非胸腺类癌的定位时间最长。死亡人数包括 35 名肺类癌死亡者中的 3 名、5 名胸腺类癌死亡者中的 2 名、6 名胃泌素瘤死亡者中的 4 名、13 名神经内分泌肿瘤死亡者中的 2 名、2 名甲状腺髓样癌死亡者、5 名嗜铬细胞瘤死亡者、3 名小细胞肺癌死亡者和 17 名隐匿性肿瘤死亡者。患有其他类癌和神经母细胞瘤的患者仍然存活。结论：IPSS 能够最好地识别 EAS。最初定位失败很常见，提示肺类癌。尽管只有 47% 的患者获得治愈，但除小细胞肺癌、甲状腺髓样癌和胃泌素瘤患者外，生存率良好。
Context: Ectopic ACTH secretion (EAS) is difficult to diagnose and treat. We present our experience with EAS from 1983 to 2004.Setting: The study was performed at a tertiary care clinical research center.Patients: Ninety patients, aged 8-72 yr, including 48 females were included in the study.Interventions and Outcome Measures: Tests included 8 mg dexamethasone suppression, CRH stimulation, inferior petrosal sinus sampling (IPSS), computed tomography, octreotide scan, magnetic resonance imaging, and/or venous sampling. Therapies, pathological examinations, and survival were noted.Results: Eighty-six to 94% of patients did not respond to CRH or dexamethasone suppression, whereas 66 of 67 had negative IPSS. To control hypercortisolism, 62 patients received medical treatment, and 33 had bilateral adrenalectomy. Imaging localized tumors in 67 of 90 patients. Surgery confirmed an ACTH-secreting tumor in 59 of 66 patients and cured 65%. Nonthymic carcinoids took longest to localize. Deaths included three of 35 with pulmonary carcinoid, two of five with thymic carcinoid, four of six with gastrinoma, two of 13 with neuroendocrine tumor, two of two with medullary thyroid cancer, one of five with pheochromocytoma, three of three with small-cell lung cancer, and two of 17 with occult tumor. Patients with other carcinoids and ethesioneuroblastoma are alive.Conclusions: IPSS best identifies EAS. Initial failed localization is common and suggests pulmonary carcinoid. Although only 47% achieved cure, survival is good except in patients with small-cell lung cancer, medullary thyroid cancer, and gastrinoma.