Pugeat M, Saez J M, Riou J P, Merchak A, Tourniaire J
Ann Endocrinol (Paris). 1979;40(6):567-9.
A 31 years old man with an adrenocortical carcinoma was studied. Clinically he had a bilateral and recidiving gynecomastia and showed high urinary oestrogens, 17 cetosteroids, tetra-hydro-desoxy-cortisol and pregnandiol excretion with normal cortisol production. A partial increase on ACTH, no suppression on dexamethasone and no variation on HCG administration were observed. The surgical resection of the tumor normalized this urinary excretion. The serum dehydro-epiandrosterone (DHEA) and sulfate (DHEAS), oestrone, oestradiol, androstenedione (A) levels were greatly elevated. No variations of the cortisol, A, DHEA and DHEAS was noted after ACTH injection. In vitro the lack of ACTH's action was related to an anomaly of ACTH receptor with normal protein kinase activity.
对一名31岁的肾上腺皮质癌男性患者进行了研究。临床上，他患有双侧复发性男性乳房发育症，尿雌激素、17-酮类固醇、四氢脱氧皮质醇和孕二醇排泄量高，而皮质醇分泌正常。观察到促肾上腺皮质激素（ACTH）有部分升高，地塞米松无抑制作用，人绒毛膜促性腺激素（HCG）给药后无变化。肿瘤的手术切除使这种尿液排泄恢复正常。血清脱氢表雄酮（DHEA）及其硫酸盐（DHEAS）、雌酮、雌二醇、雄烯二酮（A）水平大幅升高。注射ACTH后，皮质醇、A、DHEA和DHEAS无变化。在体外，ACTH作用的缺乏与具有正常蛋白激酶活性的ACTH受体异常有关。
