A 27-year-old otherwise healthy patient was diagnosed with a primitive neuroectodermal tumor of the thumb metacarpal bone of the left hand. Based on a common chromosomal translocation this tumor shows a close relationship to Ewing's sarcoma. Its occurrence in the extremities is uncommon and involvement of the hand is extremely rare. The treatment consisted of neo- and adjuvant chemotherapy and marginal resection of the affected thumb metacarpal bone including periosseous soft tissue and reconstruction of the thumb by an intercalated segmental index pollicization. (J Hand Surg 2003;28A:346-352. Copyright © 2003 by the American Society for Surgery of the Hand.)
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