Pleomorphic type of malignant fibrous histiocytoma with myxoid stroma of the vulva in a young woman

Malignant fibrous histiocytoma (MFH) of the vulva is extremely rare; to date, there have been nine case reports. Almost all of these cases involved middle-aged women. We encountered a 21-year-old woman with a 4.5 × 2.6 cm superficial, localized, exophytic tumor of the right vulva. Microscopic findings on punch biopsy of the tumor initially suggested a vulvar sarcoma. The patient underwent wide local excision of the vulva. The extirpated specimen demonstrated the pleomorphic type of MFH with myxoid stroma of the vulva. The clinical stage was found to be IIB, based on the American Joint Committee on Cancer staging system. Chromosomal analysis of the tumor using the conventional G-band method was normal (46XX). This seems to be a very rare case of MFH of the vulva in a young woman. Physicians should therefore include MFH in the differential diagnosis of vulvar tumors, even though it is a rare disease.