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One hundred and ninety-four patients with extra-abdominal desmoid tumors, most of them in the extremities, have been treated at the Mayo Clinic. One hundred and thirty-two patients (68 per cent) experienced a recurrence at an average of 1.4 years after the first treatment. A greater tendency for recurrence was evident in female patients, in patients who were more than thirty years old, in certain anatomical locations (especially the foot and calf), and most importantly after treatment by intralesional or marginal excision. In view of the excellent prognosis for survival, we recommend wide local excision when anatomically feasible or marginal excision and postoperative radiation therapy when function of the extremity would be severely compromised if excision with wide margins were done. A recurrent lesion that does not appear to be growing should be followed until evidence of growth of the lesion precipitates a secondary wide excision.