Andía Melero Víctor Manuel, García Centeno Rogelio, Fernández Jesús Bayort, Vigovich Carlos, Sánchez García-Cervigón Petra, Jara Albarrán Antonino
Servicio de Endocrinología. Hospital General Universitario Gregorio Marañón. Madrid. España.
Endocrinol Nutr. 2009 Nov;56(9):470-4. doi: 10.1016/S1575-0922(09)72970-5.
Feminizing adrenal tumours are very rare. We report the clinical and hormonal study of a case, a 49 years old male, since his first consultation until his death 6 years after the initial diagnosis, and a review of the other 5 Spanish patients previously published. His initial symptoms were gynecomastia and libido decrease, with increase of plasmatic and urinary oestrogen levels, plasma testosterone near low normal level and a right adrenal gland tumour that, after its removal, showed a benign histology and was classified as an adrenocortical adenoma. Three years after, initial symptoms returned, with oestrogen, glucocorticoid and androgen hypersecretion, tumour local relapse and peritoneal, liver and lung metastasis. After mitotane and aminoglutethimide therapy, hormonal concentrations fell temporary and then raised again until his death 3 years later. The main special feature of this case is the apparently benign initial adrenal tumour with only oestrogen hypersecretion, and its relapse 3 years later with secretion of several steroid hormones, generalized metastasis and no response to medical therapy.
女性化肾上腺肿瘤非常罕见。我们报告了一例49岁男性患者的临床及激素研究情况,从其首次就诊直至初始诊断6年后死亡,并回顾了此前发表的其他5例西班牙患者的情况。他最初的症状是乳腺增生和性欲减退,血浆和尿液雌激素水平升高,血浆睾酮接近低正常水平,右侧肾上腺有一个肿瘤,切除后显示为良性组织学,被分类为肾上腺皮质腺瘤。三年后,最初症状复发,出现雌激素、糖皮质激素和雄激素分泌过多,肿瘤局部复发以及腹膜、肝脏和肺部转移。在接受米托坦和氨鲁米特治疗后,激素浓度暂时下降,随后再次升高,直至3年后患者死亡。该病例的主要特点是最初的肾上腺肿瘤明显为良性,仅分泌过多雌激素,3年后复发,分泌多种类固醇激素,出现全身转移且对药物治疗无反应。