Surgical excision is the usually recommended treatment of
Fibromatosis in children. However the invading nature of this
tumor is responsible for recurrences leading to mutilating surgery and eventually to death despite the histologically benign appearance. We observed two cases of decrease under lengthy
chemotherapy. In the first case, a 4-year-old boy with a huge abdominal wall
tumor had two biopsies leading to the diagnosis of
fibromatosis. The
tumor measured 15 x 14 x 10 cm, and compressed the right ureter when
chemotherapy was started.
Actinomycin 1,500 microgram/m2 at Day 1 and
vincristine 1.5 mg/m2 at Day 1 and Day 8. This courses were repeated every four weeks.
Tumor decreased slowly but constantly. After 17 months it measured only 5 cm in diameter. The biopsy confirmed the nonprogressive nature of the residual
fibromatosis. In the second case, a 12-year-old boy had a
tumor of the left anterior thoracic wall. The histologic study diagnosed a
fibromatosis. Two courses of IVA (
ifosfamide,
vincristine,
actinomycin), did not lead to any decrease. A wide excision was achieved but left invaded edges. Then the child was treated by
actinomycin 750 microgram/m2 at Day 1 and
vincristine 1.5 mg/m2 at Day 1 and Day 8, one course every four weeks during ten months. At this time biopsies revealed a
scar without progressive
fibromatosis. This two cases show the interest of a
chemotherapy in extensive
fibromatosis. A mild treatment but for a long time seems to succeed where more intensive courses have failed.