Targeted therapy in advanced desmoid tumors: Current perspectives

Abstract Background 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 (FAP). Although they lack the capacity to establish metastases, DTs/AF may be devastated and occasionally fatal. As a result of the heterogeneity of DTs/AF, treatment needs to be individualized to improve local tumor control and maintain patients’ quality of life. Therefore, after a multidisciplinary approach, all treatment options should be discussed with patients. Where systemic chemotherapy has been shown to be unsuccessful with marked side effects in case of advanced DTs/AF, new therapeutic options are needed. Methods A Medline search was conducted and published articles in different studies from 2000 to the present were reviewed. Conclusion 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.