MALIGNANT PERIPHERAL-NERVE SHEATH TUMORS OF THE HEAD AND NECK - A CLINICOPATHOLOGICAL STUDY

Twenty-seven cases of malignant peripheral nerve sheath tumors involvi ng the head and neck region over a period of 7 years were reviewed. Th ey were graded from 1-3 based on necrosis, mitosis, cellularity, and p leomorphism. Mean age of occurrence was 42 years, with a range of 12-7 0 years. Male preponderance was noted (M:F = 3.5:1). The most common s ite of involvement was the neck (44.6%). The main presenting symptom w as an enlarging mass. The nerve of origin could be identified in 33% o f patients. Treatment consisted of wide excision. The 5-year observed survival was 33%. Fifty-two percent of patients developed local recurr ence of disease. Fifteen percent of patients died due to advanced loca l disease within 18 months of treatment. Distant metastasis was seen i n 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 c m) adversely affected the prognosis (P = <0.1). No significant correla tion was noted between the grade of tumor and survival. (C) 1994 Wiley -Liss, Inc.