Malignant peripheral nerve sheath tumor of the vulva. A case report.

Background: Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors in the head and neck, and their location in the genitalia, especially the vulva, is very rare.

Case: An 81-year-old woman with a solid, round vulvar tumor, 2 cm in diameter, underwent a radical hemivulvectomy after recurrence of MPNST with no evidence of neural involvement or stigmata of neurofibromatosis. The tumor cells were positive for S-100 protein and negative for cytokeratin, actin smooth muscle, HMB45 and alpha-1-antitrypsin.