Malignant extrarenal rhabdoid tumor of the vulva in an adult

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.