Hirata Y, Sakamoto N, Yamamoto H, Matsukura S, Imura H, Okada S
Cancer. 1976 Jan;37(1):377-85. doi: 10.1002/1097-0142(197601)37:1<377::aid-cncr2820370148>3.0.co;2-l.
A 51-year-old woman with typical Cushing's syndrome of about 9 years duration was shown to have a gastric carcinoid tumor. Plasma levels of ACTH and cortisol were elevated and lacked the normal diurnal rhythm. Urinary excretion of steroids was unaffected by the administration of either metyrapone or dexamethasone. Fluctuation in urinary steroid excretion, as well as transient hypokalemic alkalosis and glycosuria suggested periodic hormonogenesis. The extirpated gastric carcinoid was shown to contain immunoreactive ACTH and beta-MSH. However, the biologic ACTH activity was undetectable by in vivo steroidogenic assay. By gel filtration, it was demonstrated that both tumor and plasma ACTH was predominately "big" ACTH. Although postoperatively she developed hypoadrenocorticism severe enough to require ACTH treatment, her pituitary-adrenal function was gradually restored. This is the first documented case of ectopic ACTH syndrome caused by gastric carcinoid in which successful cure was achieved by surgery.
一名患有典型库欣综合征约9年的51岁女性被发现患有胃类癌肿瘤。血浆促肾上腺皮质激素（ACTH）和皮质醇水平升高，且缺乏正常的昼夜节律。给予甲吡酮或地塞米松后，尿类固醇排泄不受影响。尿类固醇排泄的波动以及短暂的低钾性碱中毒和糖尿提示周期性激素生成。切除的胃类癌显示含有免疫反应性ACTH和β-促黑素（β-MSH）。然而，通过体内类固醇生成测定法未检测到生物活性ACTH。通过凝胶过滤证明，肿瘤和血浆ACTH主要是“大”ACTH。尽管术后她出现了严重到需要ACTH治疗的肾上腺皮质功能减退，但她的垂体-肾上腺功能逐渐恢复。这是首例有文献记载的由胃类癌引起的异位ACTH综合征，通过手术成功治愈。
