Lipid cell tumors in two women with von Hippel-Lindau syndrome.
Lipid cell tumors in two women with von Hippel-Lindau syndrome.
复制标题
DOI：
10.1097/aog.0b013e3181e25458
复制
发表时间：
2010-08
影响因子：
7.2
通讯作者：
Stratton P
中科院分区：
文献类型：
--
作者：
Wagner M;Browne HN;Marston Linehan W;Merino M;Babar N;Stratton P
脂肪细胞肿瘤是一种罕见的功能性卵巢肿瘤。在患有von Hippel-Lindau综合征（一种常染色体显性肿瘤抑制基因突变，与肾细胞癌和其他血管肿瘤相关）的女性中尚未报道。两名患有von Hippel-Lindau综合征和肾脏肿瘤的女性被评估为继发性闭经、多毛症和计算机断层扫描所见的复杂附件肿块。第一个病人有已知的肾癌和双侧附件肿块，一个中央坏死。由于冷冻切片无法排除转移性肾细胞癌，因此施行双侧输卵管卵巢切除术。第二例患者在人绒毛膜促性腺激素检测证实卵巢肿瘤产生睾酮后接受了右侧输卵管卵巢切除术。两例患者的最终病理学检查均显示睾丸激素分泌性脂质细胞瘤。凡有附件包块、闭经和多毛症的von Hippel-Lindau患者应考虑卵巢脂肪细胞肿瘤。
Lipid-cell tumors are rare, functioning ovarian neoplasms. They have not been reported in women with von Hippel-Lindau syndrome, an autosomal-dominant tumor-suppressor gene mutation that is associated with renal cell carcinoma, and other vascular tumors. Two women with von Hippel-Lindau syndrome and kidney tumors were evaluated for secondary amenorrhea, hirsutism, and complex adnexal masses seen on computed tomography. The first patient had known renal cancer and bilateral adnexal masses, one with central necrosis. Because metastatic renal cell cancer could not be excluded on frozen section, bilateral salpingo-oophorectomy was performed. The second patient underwent right salpingo-oophorectomy after human chorionic gonadotropin testing confirmed that the ovarian tumor produced testosterone. Final pathology in both cases revealed testosterone-secreting lipid cell tumors. Lipid cell ovarian tumors should be considered in women with von Hippel-Lindau presenting with adnexal mass, amenorrhea, and hirsuitism.