Nonsurgical management of children with recurrent or unresectable fibromatosis.

摘要: At The Children's Hospital of Philadelphia, since 1971, six children 3 months to 17 years age with fibromatosis have been treated a combination vincristine, actinomycin D, and cyclophosphamide (VAC). first three patients also received radiation therapy (5,500 rads). Locally recurrent tumors developed in four the after previous operative removal; other two had that could not be removed initially. arose neck (three patients), pelvis (two or foot (one patient). In VAC alone, complete disappearance tumor was confirmed at second operation two, greater than 75% shrinkage on CT scans occurred third, all 4 6 started. who plus therapy, 13 16 months; third no response. Five are free 1, 2, 4, 11 begun; sixth has required multiple operations during last years. We conclude chemotherapy can produce regression some unresectable lesions. administration should considered for whom removal is feasible, would prove mutilating, unlikely long-term control disease.