Zayed A, Stock J L, Liepman M K, Wollin M, Longcope C
Division of Endocrinology, University of Massachusetts Medical School, Worcester.
J Endocrinol Invest. 1994 Apr;17(4):275-8. doi: 10.1007/BF03348975.
A 45-year-old man presented with gynecomastia, hypertension and a large left adrenal mass. Further evaluation revealed elevated serum concentrations of estrogen, estrone sulfate, androstenedione, dehydroepiandrosterone, dehydroepiandrosterone sulfate, deoxycorticosterone, and aldosterone and increased 24-hour urinary 17-ketosteroid and free cortisol excretion. Removal of a 10 kg adrenocortical carcinoma led to normalization of the hormone concentrations and partial resolution of the gynecomastia. There was no clinical evidence of metastases. Incubation of tumor slices demonstrated that the tumor had an active aromatase and sulfotransferase. We estimated that about half the serum estrone arose from peripheral conversion of androstenedione. Feminizing adrenocortical carcinomas are rare and this case is unusual given the lack of clinical metastases and the probable dual source of estrogen from tumor as well as from the peripheral conversion of tumor-derived androgens.
一名45岁男性出现乳腺增生、高血压及左侧肾上腺巨大肿块。进一步检查发现血清雌激素、硫酸雌酮、雄烯二酮、脱氢表雄酮、硫酸脱氢表雄酮、脱氧皮质酮和醛固酮浓度升高，24小时尿17 - 酮类固醇和游离皮质醇排泄增加。切除一个10千克的肾上腺皮质癌后，激素浓度恢复正常，乳腺增生部分消退。无转移的临床证据。肿瘤切片培养显示肿瘤具有活性芳香化酶和磺基转移酶。我们估计约一半的血清雌酮来自雄烯二酮的外周转化。女性化肾上腺皮质癌罕见，该病例不同寻常，因为缺乏临床转移证据，且雌激素可能来源于肿瘤以及肿瘤衍生雄激素的外周转化这一双重来源。
