两例 von Hippel-Lindau 综合征患者的脂细胞肿瘤。
Lipid cell tumors in two women with von Hippel-Lindau syndrome.
机构信息
From the Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Urologic Oncology Branch and Surgical Pathology Branch, National Cancer Institute, and Clinical Endocrinology Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland; and University of Toledo College of Medicine, Toledo, Ohio.
出版信息
Obstet Gynecol. 2010 Aug;116 Suppl 2(Suppl 2):535-539. doi: 10.1097/AOG.0b013e3181e25458.
BACKGROUND
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.
CASES
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.
CONCLUSION
Lipid cell ovarian tumors should be considered in women with von Hippel-Lindau presenting with adnexal mass, amenorrhea, and hirsuitism.
背景
脂质细胞瘤是一种罕见的功能性卵巢肿瘤。它们尚未在患有希佩尔-林道综合征（一种常染色体显性抑癌基因突变，与肾细胞癌和其他血管肿瘤相关）的女性中报道过。
病例
两名患有希佩尔-林道综合征和肾肿瘤的女性因继发闭经、多毛症和计算机断层扫描（CT）上看到的复杂附件肿块而接受评估。第一位患者患有已知的肾癌和双侧附件肿块，其中一个有中央坏死。由于冷冻切片无法排除转移性肾细胞癌，因此进行了双侧输卵管卵巢切除术。第二位患者在人绒毛膜促性腺激素（hCG）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。在这两个病例中，最终的病理均显示为分泌睾酮的脂质细胞瘤。
结论
对于出现附件肿块、闭经和多毛症的患有希佩尔-林道综合征的女性，应考虑脂质细胞瘤。
Zotero 插件