Korean J Otolaryngol-Head Neck Surg.
2005 Apr;48(4):551-553.
Malignant Peripheral Nerve Sheath Tumor Arising from Plexiform Neurofibroma in Neurofibromatosis Type 1
- Affiliations
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- 1Department of Otolaryngology-Head and Neck Surgery, Cancer Research Institute and Chungnam National University College of Medicine, Daejon, Korea. rohjl@cnu.ac.kr
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.
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