Malignant nerve sheath tumors comprise approximately 5% of all soft tissue sarcomas; only 8% to 15% of these tumors arise in the head and neck. Most tumors appear as a rapidly expanding nonpainful mass in the face or lateral neck. Reported association with Von Recklinghausen's disease varies from 26% to 70%. Wide surgical excision is generally the recommended primary treatment. Recently, there has been a trend to include postoperative radiation therapy as a primary modality.
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