Desmoid tumors/aggressive fibromatosis (DTs/AF) are cytological bland fibrous neoplasms originating from the musculoaponeurotic structures throughout the body. The exact cause still remains unknown, however, they may present sporadically or as a manifestation of a hereditary syndrome called familial adenomatous polyposis
A Medline search was conducted and published articles in different studies from 2000 to the present were reviewed.
More research is needed to illustrate both the prognostic and predictive factors of the targeted therapy and the value of their combinations with or without other treatment modalities to get the best result for the treatment of advanced DTs/AF.