Thorner M O, Martin W H, Ragan G E, MacLeod R M, Feldman P S, Bruni C, Williamson B R, Orth D N
Acta Endocrinol (Copenh). 1982 Mar;99(3):364-70. doi: 10.1530/acta.0.0990364.
A patient with a thymic carcinoid tumour causing ectopic ACTH syndrome is presented. The case illustrates the rapid development of the clinical and laboratory findings often associated with ectopic secretion of ACTH, including severe proximal myopathy, emotional lability, and hypokalaemic alkalosis. Interpretation of conventional tests of pituitary-adrenal function was complicated by intermittent secretion of ACTH by the tumour. The results of selective venous sampling for ACTH ruled out pituitary ACTH hypersecretion and were suggestive of a thymic source; computerized tomography of the chest localized the tumour. In vivo and in vitro investigations confirmed excessive ACTH production by the tumour, and surgical resection plus radiotherapy has resulted in resolution of the syndrome. The diagnostic problems created by intermittent secretion of ACTH by these tumours and the pre-operative and post-operative medical management of these patients are discussed.
本文介绍了一例由胸腺类癌肿瘤引起异位促肾上腺皮质激素（ACTH）综合征的患者。该病例说明了常与ACTH异位分泌相关的临床和实验室检查结果的快速发展，包括严重的近端肌病、情绪不稳定和低钾性碱中毒。肿瘤间歇性分泌ACTH使垂体-肾上腺功能的传统检查结果解读变得复杂。ACTH选择性静脉采样结果排除了垂体ACTH分泌过多，并提示胸腺来源；胸部计算机断层扫描确定了肿瘤的位置。体内和体外研究证实肿瘤产生过多ACTH，手术切除加放疗已使该综合征得到缓解。本文讨论了这些肿瘤间歇性分泌ACTH所产生的诊断问题以及这些患者的术前和术后药物治疗。
