Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm–a position paper from the Italian and the French Sarcoma Group.

Summary

Desmoid-type fibromatosis (DF) is a rare locally aggressive monoclonal proliferation of myofibroblasts lacking metastatic capacity. It may be observed in nearly every part of the body. Considering the unpredictable behavior and the heterogeneity of this disease, we propose a treatment algorithm approved by the French and the Italian Sarcoma Group, based on a front-line wait and see approach and subsequent therapy in the case of progression.