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Case Reports in Obstetrics and Gynecology
Volume 2013, Article ID 217924, 3 pages
http://dx.doi.org/10.1155/2013/217924
Case Report

Vulvar Malignancy in Neurofibromatosis Syndrome

Department of Obstetrics and Gynecology, “Sapienza” University, Viale del Policlinico 155, 00155 Rome, Italy

Received 30 July 2013; Accepted 22 August 2013

Academic Editors: E. Cosmi, D. Hochner-Celnikier, C.-C. Liang, L. Nilas, and R. Shaco-Levy

Copyright © 2013 Angela Musella et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Type 1 neurofibromatosis (NF1) is a dominantly inherited neurologic disorder that affects primarily the skin, bones, and peripheral nervous system. It may be associated with a variety of clinical manifestations including cafe-au-lait spots, skinfold freckling, Lisch nodules, and visceral neurofibromas. Individuals affected by NF1 harbor an increased risk for both benign and malignant tumors. Malignant transformation is usually observed in the form of neurosarcoma. Rarely, NF1 affects the genital tract, and isolated vulvar localization is extremely rare. Here is reported a rare case of a solitary neurosarcoma of the vulva in a 43-year-old woman affected by NF1 syndrome treated with surgical excision. The purpose of this case is to underline the possibility of association between NF1 and genital tract sarcoma and to suggest an accurate evaluation of rapid growth vulvar mass in this setting.