EXTREMITY MALIGNANT PERIPHERAL-NERVE SHEATH TUMORS (NEUROGENIC SARCOMAS) - A 10-YEAR EXPERIENCE

Background: Prior studies have shown a high rate of local recurrence a nd a dismal overall prognosis in malignant peripheral nerve sheath tum ors (MPNSTs), Methods: Thirty-three patients who had undergone primary treatment for localized extremity MPNSTs between 1982 and 1992 were r eviewed. These cases were derived from a prospective database of 890 a dult extremity soft-tissue sarcomas (STS), MPNSTs were compared with o ther extremity STS. Results: MPNSTs were more often high grade and dee p compared with other extremity STS (94% vs. 72% [p = 0.009] and 97% v s. 76% [p = 0.01], respectively), Location (upper or lower extremity), size (> 5 cm vs. less than or equal to 5 cm), and status of margins a fter surgical resection (positive or negative for disease) did not dif fer between the two groups. When deep and high-grade MPNSTs were compa red with deep and high-grade STS, a more aggressive local treatment wa s applied to MPNSTs with a higher number of amputations for MPNSTs (32 %) compared with STS (9%; p < 0.001), In order to obtain adequate marg ins, 16 of 21 MPNSTs arising from major nerves required either amputat ion (n = 8) or nerve resection (n = 8). Adjuvant radiotherapy was used in 48% of deep and high-grade MPNSTs, and 3-year local disease-free s urvival was 70%. Survival of deep and high-grade MPNSTs was comparable with other deep and high-grade STS (3-year survival 50% vs, 69%, resp ectively; p = 0.1). Conclusion: MPNSTs show adverse clinicopathologic features compared with other STS, However, when treated aggressively, MPNSTs have an outcome similar to other deep and high-grade extremity STS.