Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): A 10-year experience

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Abstract
Background: Prior studies have shown a high rate of local recurrence and a dismal overall prognosis in malignant peripheral nerve sheath tumors (MPNSTs). Methods: Thirty-three patients who had undergone primary treatment for localized extremity MPNSTs between 1982 and 1992 were reviewed. These cases were derived from a prospective database of 890 adult extremity soft-tissue sarcomas (STS). MPNSTs were compared with other extremity STS. Results: MPNSTs were more often high grade and deep compared with other extremity STS (94% vs. 72% [p=0.009] and 97% vs. 76% [p=0.01], respectively). Location (upper or lower extremity), size (>5 cm vs. ⩽5 cm), and status of margins after surgical resection (positive or negative for disease) did not differ between the two groups. When deep and high-grade MPNSTs were compared with deep and high-grade STS, a more aggressive local treatment was applied to MPNSTs with a higher number of amputations for MPNSTs (32%) compared with STS (9%; pConclusion: 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.