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

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.