Desmoid fibromatosis: a clinicopathologic study of 25 children.

We studied 25 patients under 15 years of age with desmoid fibromatosis (DF). There were 15 boys and 10 girls; 13 were under seven years of age and 12 were above. Histologically, DF was identical to DF in adult patients, except for a higher mitotic rate in children's tumors. The tumors were located on the head and neck (8), upper (7) or lower (6) extremities, or the trunk (4). Patients with head and neck DF received preoperative adriamycin and 5-(dimethyltriazeno) imidazole-4-carboxamide or a combination of vincristine, actinomycin D, and cyclophosphamide. In seven patients, tumor reduction began to occur during chemotherapy, while in one patient, there was no response at all. Five patients underwent conservative resections of their residual tumors, and in two patients, all tumor disappeared necessitating no additional surgery. Follow-up in these patients indicates that 6 have no evidence of disease, 1 is alive with recurrent disease, and 1 is lost to follow-up. Surgery was the primary modality of treatment for the trunk and extremity lesions. Twelve patients had no evidence of disease from 2 months to 25 years and 5 months, two died from complications of chemotherapy, and three were lost to follow-up. Our experience agrees with that of other investigators that DF is best managed surgically. However, preoperative chemotherapy should be strongly considered for patients with tumors occurring in the head and neck areas.