Long-term results with radiation therapy for pediatric desmoid tumors

Purpose: To retrospectively review the treatment and outcome of pediatric patients with desmoid tumor who received radiation therapy at a single institution. Materials and Methods: Thirteen pediatric patients received radiation therapy for desmoid tumor at St. Jude Children's Research Hospital between 1962 and 1998, Only 2 of the patients reviewed received treatment prior to 1976, The median dose of external beam irradiation was 50 Gy, Results: At the time of this report, 10 of 13 patients have had tumors that recurred after radiation therapy and 3 have died from their disease. One additional patient was harboring a recurrence, and 1 had not been followed long enough to suggest that the patient had achieved disease control. One patient remained locally controlled after radiation therapy with long-term follow-up (196 months). The median time to recurrence following radiation therapy was 19 months (range, 3-135 months). Eight of the 13 patients suffered substantial tumor and treatment-related morbidity. Conclusions: Desmoid tumors in pediatric patients are locally aggressive tumors that are likely to recur after radiation therapy, Alternatives to radiation therapy should be sought for the treatment of these tumors, and efforts should focus on low-morbidity therapies aimed at inhibiting the growth of these unique tumors. (C) 2000 Elsevier Science Inc.