Malignant peripheral nerve sheath tumors of the head and neck: Analysis ofprognostic factors

Malignant peripheral nerve sheath tumors of the head and neck region are un common and may be associated with neurofibromatosis or occur in a sporadic manner. This is a retrospective review of 17 patients with these tumors who were treated at a single institution. Analysis of clinical and pathologic factors that influenced outcome was performed. There were 9 men and 8 women . Seven patients had a history of neurofibromatosis, Radiotherapy was impli cated as a possible etiologic factor in 4 patients. The neck was the most f requently involved site. Overall survival at 5 years was 52%. Survival was improved for women and for patients with low-grade tumors. Age, tumor site, and size had no impact on survival. Survival was worse for patients with n eurofibromatosis than for those with the sporadio form of the disease (P = 0.02), Survival was calculated by the method of Kaplan and Meier. The signi ficance of such results was based on results of the log rank test. Local re currence correlated with tumor size and resection margin status. No local r ecurrences occurred in those patients who had negative margins of resection and received adjuvant radiotherapy. Tumor grade was predictive of the deve lopment of distant metastases. Negative margins of resection are essential for obtaining local control, and the addition of adjuvant radiotherapy may be beneficial in this group. Salvage surgery for local recurrence is possib le in some patients.