Bilateral Malignant Peripheral Nerve Sheath Tumor Associated with a Massive Osteoma of the Mandible

Introduction: Malignant peripheral nerve sheath tumor (MPNST) is a highly aggressive soft tissue sarcoma, which is uncommon in the maxillofacial area. Case Report: We present a rare case of bilateral MPNST with an osteoma in the Mandible. Clinically, massive bilateral swelling in the mandibular angle with rapid growth for 9 months was seen. Results: Histological evaluation showed the proliferation of malignant spindle cells arranged in a fascicular pattern with infiltrating margins. Immunohistochemical evaluation was positive for S100 protein and vimentin and negative for CD 68, Desmin, and CD31. Resection the soft tissue lesions with debulking osteoma did not show any recurrence in five years follow-up. Conclusion: Surgical excision may be an essential treatment option to eradicate MPNST.