Malignant extrarenal rhabdoid tumor of the vulva in an adult

Abstract

Sarcomas of the vulva account for only 1-3% of all vulvar malignancies. Most common vulvar sarcomas are leiomyosarcomas, malignant fibrohistiocytomas, and aggressive angiomyxomas. Malignant rhabdoid tumor (MRT) of the kidney is a distinctive clinicopathological entity that is recognized as a highly aggressive renal tumor of childhood. Extrarenal malignant rhabdoid tumors have been proposed to exist at several sites, including soft parts. MRT of the vulva is a rare and very aggressive neoplasm. Median survival reported in other studies is 9 months. Only 10 cases have been reported thus far in the English literature. We are reporting the 11 th case who remains disease free 30 months following surgery and radiotherapy till the time of reporting.