Malignant peripheral nerve sheath tumors of the head and neck: A clinicopathological study

Abstract

Twenty-seven cases of malignant peripheral nerve sheath tumors involving the head and neck region over a period of 7 years were reviewed. They were graded from 1-3 based on necrosis, mitosis, cellularity, and pleomorphism. Mean age of occurrence was 42 years, with a range of 12-70 years. Male preponderance was noted (M:F = 3.5:1). The most common site of involvement was the neck (44.6%). The main presenting symptom was an enlarging mass. The nerve of origin could be identified in 33% of patients. Treatment consisted of wide excision. The 5-year observed survival was 33%. Fifty-two percent of patients developed local recurrence of disease. Fifteen percent of patients died due to advanced local disease within 18 months of treatment. Distant metastasis was seen in 18.5% of patients. Lymph node metastasis was not seen. At the end of 5 years 15% of patients remained disease free. Large tumor size (> 5 cm) adversely affected the prognosis (P = < 0.1). No significant correlation was noted between the grade of tumor and survival.