BACKGROUND/OBJECTIVES: To report on the clinical presentation and outcome for 234 patients with extra-abdominal desmoids tumors. METHODS: Since 1977, the authors have treated 234 patients with extra-abdominal desmoid tumors. The patients had an average age of 36.7 and 61% were female. The tumors arose adjacent to muscles or bones and the largest number were in the foot, shoulder thigh and calf. All of the patients were treated by primary surgery. Thirty-seven had additional radiation and eight had chemotherapy. RESULTS: Local recurrence occurred in 39 patients and 23% of the patients required additional surgery. Of great concern were 24 patients who developed multiple sites metachronously, which required further surgery and in many cases caused disability. None of the patients died of disease but 5 required amputations. CONCLUSIONS: The authors concluded that despite the benign nature of the disease, these patients are difficult to treat and the results are sometimes considerably less than optimal.
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