Clinic of Endocrinology, Diabetes and Metabolic Diseases, Belgrade, Serbia,
Faculty of Medicine, University of Belgrade, Belgrade, Serbia,
Front Horm Res. 2019;53:100-107. doi: 10.1159/000494906. Epub 2019 Sep 9.
About 1% of ovarian tumors that comprise testicular cell types can cause hyperandrogenism followed by characteristic virilization. Androgenic group of tumors originated mainly from sex-cord stromal ovarian tumors are including steroid cell tumors, Leydig tumors, granulosa cell tumors, Sertoli cell tumors, Sertoli-Leydig cell tumors, gonadoblastomas, and some other rare forms as ovarian metastases from neuroendocrine tumors. Germline or somatic mutations in some genes like DICER1, STK11, and FOXL2 are associated with the development of some sex cord-stromal ovarian tumors. Basal serum testosterone concentrations above 7 nmol/L could indicate an androgen-secreting tumor. Other ovarian and adrenal androgens should be determined and functional endocrine testing including low-dose dexamethasone suppression test, gonadotrophin-releasing hormone (GnRH) agonist test, imaging methods, and selective venous sampling should be performed. Surgery is the first-line treatment for most of the tumors. Women who are not good surgical candidates could benefit from use of GnRH agonist to control hyperandrogenism. In some cases, chemotherapy and/or radiation therapy is required while some tumors respond on antiangiogenic agents used alone or in combination with chemotherapy. Metabolic implications and long-term outcomes of ovarian androgen-secreting tumors are unknown and require more detailed follow-up in multicentric and longitudinal clinical studies.
约 1%的含有睾丸细胞类型的卵巢肿瘤可导致高雄激素血症，随后出现特征性的男性化。来源于性索间质的雄激素肿瘤主要包括类固醇细胞瘤、间质细胞瘤、颗粒细胞瘤、支持细胞瘤、支持-间质细胞瘤、性腺母细胞瘤以及一些其他罕见形式的卵巢神经内分泌肿瘤转移。一些基因（如 DICER1、STK11 和 FOXL2）的种系或体细胞突变与一些性索间质卵巢肿瘤的发展有关。基础血清睾酮浓度高于 7 nmol/L 可能提示存在分泌雄激素的肿瘤。应确定其他卵巢和肾上腺雄激素，并进行功能性内分泌测试，包括小剂量地塞米松抑制试验、促性腺激素释放激素（GnRH）激动剂试验、影像学方法和选择性静脉采样。手术是大多数肿瘤的一线治疗方法。不适合手术的女性可以受益于 GnRH 激动剂来控制高雄激素血症。在某些情况下，需要化疗和/或放疗，而一些肿瘤对单独使用或联合化疗的抗血管生成药物有反应。卵巢雄激素分泌肿瘤的代谢影响和长期结局尚不清楚，需要在多中心和纵向临床研究中进行更详细的随访。
