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Case Reports in Otolaryngology
Volume 2012 (2012), Article ID 936735, 3 pages
http://dx.doi.org/10.1155/2012/936735
Case Report

A Case Report of a Malignant Peripheral Nerve Sheath Tumor of the Oral Cavity in Neurofibromatosis Type 1

1Department of Otorhinolaryngology, School of Medicine, Istanbul Medipol University, Istanbul, Turkey
2Kulak Burun Bogaz Anabilim Dalı, Istanbul Medipol Universitesi, Kosuyolu, Kadikoy, 34718 Istanbul, Turkey
3Department of Otorhinolaryngology, Academic Hospital, Istanbul, Turkey

Received 28 September 2012; Accepted 17 October 2012

Academic Editors: E. Mevio and M. B. Naguib

Copyright © 2012 Özmen Öztürk and Alper Tutkun. 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

Patients with neurofibromatosis type 1 develop both benign and malignant tumors at an increased frequency. Most of the malignant peripheral nerve sheath tumors (MPNSTs) are considered as high-grade sarcomas originating from tissues of mesenchymal origin. It is generally accepted that MPNSTs occur in about 2% to 5% of neurofibromatosis patients. In this paper, we present a 16-year-old male patient with neurofibromatosis who developed MPNST of the retromolar area. The mass enlarged rapidly in a period of 6 weeks. The patient was treated surgically, and a tumor mass with a diameter of  cm was excised, but after 8 months a recurrence was observed at the same site. The sarcomatous change in a neurofibroma has an extremely poor prognosis, so patients with neurofibromatosis should be closely monitored for a possible malignancy. A rapid change in size of a preexisting neurofibroma, infiltration of the adjacent structures, intralesional hemorrhage, and pain indicate a possible malignant transformation to MPNST.