The presentation, treatment, and outcome of 12 patients with high-grade neurogenic sarcoma (NS) of the extremity were compared with those of 21 patients with high-grade extremity soft-tissue sarcoma of nonneural origin in a retrospective study from January 1976 to January 1988. Pain and neurologic deficit were more common in the NS group. Limb-sparing surgery was carried out with equal frequency in both groups. Local recurrence was six times more frequent in the NS group at three-year follow-up (59% vs 10%). Width of resection margin was the dominant prognostic variable bearing on local control after limb-sparing surgery. Anatomic and functional constraints tended to limit resection margin in patients with NS arising from mixed motor-sensory or predominantly motor nerves.
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