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Lipid cell tumors in two women with von Hippel-Lindau syndrome
Marnie Wagner1, Hyacinth N Browne, W Marston Linehan
1From 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.
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.
