Abstract

Eight cases of extraabdominal desmoid tumors in children are reviewed. Seven were located in the pelvis or forearm, and the most common presenting complaint was a slowly enlarging mass. In all cases, diagnosis was established by open biopsy, and initial treatment was by surgical excision. At follow-up (average, 5.8 years), six patients were tumor free. Desmoids are benign tumors that usually carry a good prognosis. There is no difference between the tumor behavior in children and adults. The treatment of choice is wide local excision. If vital structures are involved, it may be more appropriate to preserve function by performing partial tumor excision. For quiescent recurrent tumors, observation is appropriate management, but if further treatment is required, reexcision or radiotherapy may be tried.

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