Mure A, Gicquel C, Abdelmoumene N, Tenenbaum F, Francese C, Travagli J P, Gardet P, Schlumberger M
Service de Médecine Nucléaire, Institut Gustave Roussy, Villejuif, France.
J Endocrinol Invest. 1995 Mar;18(3):180-5. doi: 10.1007/BF03347800.
Ectopic ACTH secretion is a rare cause of hypercortisolism. Induced metabolic disturbances are often serious, and the management of such patients may be difficult. We report here our experience with four medullary thyroid carcinoma (MTC) patients with distant metastases in whom an ectopic ACTH syndrome occurred. The clinical presentation was significant by the severity and the rapidity of the hypercortisolism. Diagnosis and follow-up were realized by measurements of plasma cortisol, urinary free cortisol, urinary 17-hydroxycorticosteroid, plasma ACTH, plasma LPH, serum calcitonin and carcino-embryonic antigen. Initial treatment with adrenalytic medical therapy failed to control the disease. Only bilateral adrenalectomy cured the excessive cortisol production, and for a long time despite tumor progression. In conclusion, bilateral adrenalectomy should be considered in MTC patients with Cushing's syndrome even at the stage of distant spread. In fact, with regard to the slow growth rate of most MTC's, they may survive for years.
异位促肾上腺皮质激素（ACTH）分泌是皮质醇增多症的一种罕见病因。由此引发的代谢紊乱通常较为严重，这类患者的治疗可能会很困难。我们在此报告4例伴有远处转移的甲状腺髓样癌（MTC）患者发生异位ACTH综合征的经验。临床表现因皮质醇增多症的严重程度和快速进展而显著。通过检测血浆皮质醇、尿游离皮质醇、尿17-羟皮质类固醇、血浆ACTH、血浆促脂解素、血清降钙素和癌胚抗原进行诊断和随访。最初采用肾上腺溶解药物治疗未能控制病情。只有双侧肾上腺切除术治愈了皮质醇过度分泌，且尽管肿瘤进展，但在很长一段时间内病情得到了控制。总之，即使在远处转移阶段，对于患有库欣综合征的MTC患者也应考虑进行双侧肾上腺切除术。事实上，鉴于大多数MTC生长缓慢，患者可能存活数年。
