Malignant Peripheral Nerve Sheath Tumor Arising from Plexiform Neurofibroma in Neurofibromatosis Type 1.
- Author:
Yeo Hoon YOON
1
;
Jong Lyel ROH
Author Information
1. Department of Otolaryngology-Head and Neck Surgery, Cancer Research Institute and Chungnam National University College of Medicine, Daejon, Korea. rohjl@cnu.ac.kr
- Publication Type:Case Report
- Keywords:
Neurofibromatosis type 1;
Plexiform neurofibroma;
Malignant peripheral nerve sheath tumor
- MeSH:
Adult;
Humans;
Neck Dissection;
Neurilemmoma;
Neurofibroma, Plexiform*;
Neurofibromatoses*;
Neurofibromatosis 1*;
Peripheral Nerves*;
Prognosis
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
2005;48(4):551-553
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Malignant peripheral nerve sheath tumor (MPNST) is defined as a malignant tumor arising from cells of the peripheral nerve sheath. The patients with neurofibromatosis type 1 (NF1) have the highest probability of developing MPNST. Even with wide surgical excision and adjuvant chemoradiation, the prognosis of MPNST is generally poor. We experienced a case of MPNST in a 38-year-old man with a long-standing NF1. He had a painless, infraauricular mass of 1 month duration. The patient underwent excision of the mass followed by total parotidectomy and modified neck dissection. Permanent pathologic sections confirmed MPNST. We report this case with a review of the literature.
