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Malignant peripheral nerve sheath tumor of the vulva: a multimodal treatment approach
Nicholas C Lambrou1, Ramin Mirhashemi, Aaron Wolfson
1Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, University of Miami/Jackson Memorial Hospital, Holtz Building, Suite 3003, 1611 NW 12th Avenue, Miami, FL 33136, USA. nlambrou@med.miami.edu
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.