Malignant peripheral nerve sheath tumor of the vulva: a multimodal treatment approach

Abstract

Background: Malignant peripheral nerve sheath tumors (MPNSTs) are rare in the gynecological population and have a high risk for local and distant failures. Multimodal management of a patient with MPNST of the vulva and review of the literature are outlined.

Case: A 34-year-old woman presented with a complaint of a rapidly increasing pelvic mass, pain, and difficulty ambulating. A disfiguring 20 x 20-cm vulvar mass was identified and a recurrent MPNST diagnosed. Therapy included external-beam radiation, anterior pelvic exenteration with pelvic reconstruction, and adjuvant chemotherapy without complication.

Conclusion: It is recommended that for malignant peripheral nerve sheath tumors of the vulva, complete surgical resection be performed with adjuvant radiation and chemotherapy in selected cases.