PEDIATRICS Vol. 79 No. 3 March 1987, pp. 394-398
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Nonsurgical Management of Children With Recurrent or Unresectable Fibromatosis

Beverly Raney MD1, Audrey Evans MD1, Linda Granowetter MD1, Louise Schnaufer MD1, Antonia Uri MD1, and Philip Littman MD1

1 From the Division of Oncology, Department of Pediatrics, and the Departments of Surgery and Pathology, Children’s Hospital of Philadelphia, and the Department of Radiation Therapy, Hospital of the University of Pennsylvania, and the University of Pennsylvania School of Medicine, Philadelphia

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

Key Words: fibromatosis

Submitted on May 1, 1986
Accepted on May 22, 1986




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