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

Abstract

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
neurofi bromatosis. 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 diffi culties associated with MPNST.
Key Words: De novo, intraosseous, malignant peripheral nerve sheath tumor, S-100