Treatment of desmoid-type fibromatosis

Evaluating the benefit of treatment interventions for desmoid-type fibromatosis has been extremely difficult, because desmoid-type fibromatosis has a highly variable natural history, with partial regressions seen in up to 20% of patients.[2] Large adult series and smaller pediatric series have reported long periods of disease stabilization and even regression without systemic therapy.[10,11]; [12][Level of evidence C2] For instance, in a large placebo-controlled trial of sorafenib in adult patients with desmoid tumors, the patients who received no therapy (observation/placebo) demonstrated a 20% partial regression rate, and 46% of the patients in the placebo group had no progression at 1 year.[2]

Treatment options for desmoid-type fibromatosis include the following: