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一名绝经后女性因分泌睾酮的支持-间质细胞瘤伴分泌硫酸脱氢表雄酮的肾上腺腺瘤导致高雄激素血症:病例报告及文献复习

Hyperandrogenism due to a testosterone-secreting Sertoli-Leydig cell tumor associated with a dehydroepiandrosterone sulfate-secreting adrenal adenoma in a postmenopausal woman: case presentation and review of literature.

作者信息

Herrera Jorge D, Davidson Jaime A, Mestman Jorge H

机构信息

Section of Endocrinology and Diabetes, Hospital Dr Guillermo Rawson, School of Medicine, Catholic University of Cuyo, San Juan, Argentina.

出版信息

Endocr Pract. 2009 Mar;15(2):149-52. doi: 10.4158/EP.15.2.149.

Abstract

OBJECTIVE

To report a case of hyperandrogenism attributable to the presence of an adrenal adenoma secreting dehydroepiandrosterone sulfate (DHEA-S) and an ovarian Sertoli-Leydig cell tumor secreting testosterone in a postmenopausal woman.

METHODS

The laboratory, radiologic, and pathologic findings in our case are described. In addition, the pertinent literature is reviewed.

RESULTS

A 56-year-old woman presented with a history of gradual increase in facial and body hair, scalp hair loss, male pattern baldness, and deepening of her voice, beginning a few years after spontaneous menopause at age 49 years. She had hypertension, obesity, and type 2 diabetes mellitus. Laboratory tests showed elevated levels of total testosterone (348 ng/dL) and DHEA-S (2,058 microg/dL), and a left adrenal tumor (3 by 4 cm) was detected on abdominal computed tomographic scan. Laparoscopic left adrenalectomy was performed, and the pathologic diagnosis was adrenal adenoma. The DHEA-S returned to normal levels, but the serum testosterone concentration remained elevated. Transvaginal ultrasonography disclosed an ovarian tumor. Bilateral oophorectomy was performed, and an ovarian Sertoli-Leydig cell tumor was diagnosed. The hormonal and clinical picture normalized after this surgical intervention.

CONCLUSION

After extensive review of the literature, we believe that this is the first reported case of a coincidental DHEA-S-secreting adrenal adenoma and a testosterone- secreting ovarian Leydig cell tumor causing signs of virilization.

摘要

目的

报告一例绝经后女性因肾上腺腺瘤分泌硫酸脱氢表雄酮(DHEA-S)以及卵巢支持-间质细胞瘤分泌睾酮导致高雄激素血症的病例。

方法

描述了我们病例中的实验室、影像学和病理学检查结果。此外,还对相关文献进行了综述。

结果

一名56岁女性,自49岁自然绝经后数年开始出现面部和身体毛发逐渐增多、头皮脱发、男性型秃发以及声音变低沉的病史。她患有高血压、肥胖症和2型糖尿病。实验室检查显示总睾酮水平升高(348 ng/dL)和DHEA-S水平升高(2,058 μg/dL),腹部计算机断层扫描检测到左侧肾上腺肿瘤(3×4 cm)。进行了腹腔镜下左侧肾上腺切除术,病理诊断为肾上腺腺瘤。DHEA-S恢复到正常水平,但血清睾酮浓度仍保持升高。经阴道超声检查发现卵巢肿瘤。进行了双侧卵巢切除术,诊断为卵巢支持-间质细胞瘤。此次手术干预后,激素水平和临床症状恢复正常。

结论

在广泛查阅文献后,我们认为这是首例报告的因同时存在分泌DHEA-S的肾上腺腺瘤和分泌睾酮的卵巢间质细胞瘤而导致男性化体征的病例。

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