Objective: To investigate the clinicopathologic features, treatment and prognosis of the malignant peripheral nerve sheath tumors (MPNST) in oral and maxillofacial region.
Methods: From 1987 to 2008, 11 patients underwent surgical treatment. We retrospectively reviewed clinical character, treatment and follow-up data and performed a literature review.
Results: The most common symptom of the patients was an enlarging mass. Five patients felt numb and/or pain. The immunohistochemical staining showed positive for SMA (5/11), S-100 (7/11), p53 (5/11), Ki-67 (11/11) and vimentin (11/11). The 2-year and 5-year survival rates of the patients were 30% and 9.5% respectively, and the average survival time was 33.9 months.
Conclusion: MPNST is one of the most aggressive tumors with high risk of local recurrence and distant metastasis. Mulitimodal therapy including surgical resection and adjuvant radiotherapy is needed. Complete removal of the tumor is the main modality of treatment and a most important prognostic factor of MPNST.