Intraosseous malignant peripheral nerve sheath tumor of maxilla: A case report with review of the literature

Malignant peripheral nerve sheath tumor (MPNST), the principle malignancy of peripheral nerve origin, though rare in the general population, occurs with excessive frequency among patients with neurofibromatosis. This tumor always arises in soft-tissues, usually found in the lower extremities and only 10-12% of all lesions occur in the head and neck region, which makes it a rare entity. The primary intraosseous MPNST is rare and has been reported most frequently in the mandible. This article discusses a case report of MPNST of the left maxilla without a history of benign nerve tissue tumor and the diagnostic difficulties associated with MPNST.