Tourniaire J, Rebattu B, Conte-Devolx B, Trouillas J, Grino M, Berger-Dutrieux N, Peix J L, Pugeat M
Clinique Endocrinologique de l'Université Claude Bernard, Faculté Lyon-Sud.
Ann Endocrinol (Paris). 1988;49(1):61-7.
A 58-yr-old man presented a Cushing's syndrome gradually developed for two years, and a cervical tumor. Urinary free cortisol and 17-hydroxy-corticosteroids were elevated and non suppressible under high dose dexamethasone (8 mg a day X 2 days). Plasma calcitonin (7,200 pg/ml), CEA (803 ng/l), beta LPH (624 pg/ml), and CRF (29 pg/ml) were elevated. Total thyroidectomy revealed a medullary carcinoma of the thyroid. Postoperatively the Cushing's syndrome disappeared and plasma CRF became undetectable although plasma calcitonin remained elevated. One out of 3 CRF antisera tested for immunocytology was positive in 10 to 30% of the cells. In tumor extract, CRF (RIA) concentration was 4.75 ng/g. There was no detectable ACTH in the tumor by biochemical as well as immunocytochemical method. In the present report, the next evidences are--for the first time--simultaneously present to demonstrate an ectopic secretion of CRF by a medullary thyroid carcinoma: presence of CRF in systemic blood being undetectable after surgery; cure of the clinical and biological features of Cushing's syndrome after thyroidectomy; characterization of CRF immunoreactivity in tumor. Taken together, the radioimmunological and the immunocytochemical data suggest the production of several molecular forms of CRF.
一名58岁男性，患有逐渐发展两年的库欣综合征及颈部肿瘤。尿游离皮质醇和17-羟皮质类固醇升高，且在高剂量地塞米松（8毫克/天×2天）下不可被抑制。血浆降钙素（7200皮克/毫升）、癌胚抗原（803纳克/升）、β-促脂解素（624皮克/毫升）和促肾上腺皮质激素释放因子（29皮克/毫升）升高。全甲状腺切除术显示为甲状腺髓样癌。术后库欣综合征消失，血浆促肾上腺皮质激素释放因子检测不到，尽管血浆降钙素仍升高。用于免疫细胞化学检测的3种促肾上腺皮质激素释放因子抗血清中有1种在10%至30%的细胞中呈阳性。肿瘤提取物中促肾上腺皮质激素释放因子（放射免疫分析法）浓度为4.75纳克/克。通过生化及免疫细胞化学方法在肿瘤中均未检测到促肾上腺皮质激素。在本报告中，首次同时出现以下证据以证明甲状腺髓样癌异位分泌促肾上腺皮质激素释放因子：术后全身血液中促肾上腺皮质激素释放因子检测不到；甲状腺切除术后库欣综合征的临床及生物学特征得到治愈；肿瘤中促肾上腺皮质激素释放因子免疫反应性的特征。综合来看，放射免疫和免疫细胞化学数据提示产生了几种促肾上腺皮质激素释放因子分子形式。
