Oral Malignant Peripheral Nerve Sheath Tumors: A Systematic Review of the Case Reports

Abstract

Malignant peripheral nerve sheath tumors (MPNST) originated from cells of the peripheral nerve sheath. These tumors account for 5% of all soft tissue sarcomas. MPNST are extemely rare in the oral and maxillofacial region. Oral Malignant peripheral nerve sheath tumors (OMPNST) arise denovo or from neurofibromatosis type I. d. OMPNST usually presents with a progressive swelling which may be painful.The mean age in patients is about 40 to 46 years. The most common sites OMPNST is the mandible, lips, and buccal mucosa. Radiographic examination of intraosseous tumors reveal irregular destruction of the surrounding bone. Definitive diagnosis of OMPNST is confirmed on the basis of findings histopathological and immunohistochemical evaluation. The prognosis OMPNSTS is poor and to treat by radical surgical excision, radiation therapy and chemotherapy. This systematic review aimed to determine the most significant influential factors in OMPNSTS and evaluate the diagnostic and therapeutic methods in this regard.