Treatment of aggressive fibromatosis - A retrospective study of 72 patients followed for 1-27 years

We evaluated prognostic factors for local recurrence-free survival, including expression of estrogen receptors, after surgical treatment of aggressive fibromatosis in 72 patients (53 women) having primary tumors between 1970 and 1998. Their median age at diagnosis was 31 (1 month-77 years) years. 50 patients had extraabdominal and 22 abdominal fibromatosis. Median tumor size was 4 (1-27) cm. 8 patients were treated with an intralesional resection, 32 with marginal, 31 with wide and 1 with radical resection. They were followed for a median of 8 (1-27) years. The overall and local recurrence-free 5-year survival rates were 98% and 73%, respectively. Univariate analysis identified age, compartmentalization and tumor size as prognostic factors for local recurrence-free survival as well as radiotherapy in extraabdominal tumors. In the multivariate analysis, tumor size > 4 cm, extracompartmental location, inadequate margin and age < 32 years were independent negative prognostic factors for local recurrence. None of the tumors expressed estrogen receptors. In conclusion, aggressive fibromatosis has a high local recurrence rate, but a good prognosis, since almost no patients die of their tumor.