Malignant peripheral nerve sheath tumor of the oral cavity.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, highly aggressive, soft tissue sarcomas of ectomesenchymal origin that account for 3% to 10% of all sarcomas. They arise de novo or from prexisting benign neurofibromas. The diagnosis of PNST has been described as one of the most difficult nd elusive diagnoses in soft tissue diseases because f its nonspecific clinical and histopathologic presenation. The authors report a rare case of MPNST of he oral cavity and discuss the histopathology, treatent, and prognosis of the condition.