Optimal therapy for desmoid tumors: current options and challenges for the future: Influence Statistics

Expert Impact

Concepts for which they have has direct influence: Desmoid tumors , Desmoid tumor , Aggressive fibromatosis , Fibromatosis aggressive , Tumors aggressive , Aggressive humans , Beta catenin .

Key People For Desmoid Tumors

Top KOLs in the world
#1
Murray, F Brennan
gastric cancer soft tissue pancreatic adenocarcinoma
#2
Jyrki J REITAMO
desmoid tumor ulcerative colitis intramuscular myxoma
#3
Pekka J Häyry
chronic rejection cytomegalovirus infection renal allografts
#4
Alessandro Gronchi
retroperitoneal sarcoma soft tissue neoadjuvant chemotherapy
#5
Robin K S Phillips
familial adenomatous polyposis anal fistula capsule endoscopy
#6
Paolo Giovanni Casali
soft tissue rare cancers italian sarcoma

Optimal therapy for desmoid tumors: current options and challenges for the future

Abstract

. Desmoid tumors, or aggressive fibromatosis, are rare, locally infiltrative neoplasms caused by mutations that activate β-catenin. Although these tumors do not metastasize, they are difficult to manage due to variability in tumor presentation and behavior. A variety of treatment options exist, including surgery, radiotherapy, chemotherapy, hormone therapy, isolated limb perfusion, cryoablation and tyrosine kinase inhibitors. Treatment-induced morbidity and poor local control rates, combined with spontaneous stabilization of some desmoid tumors, have allowed watchful waiting to recently emerge as a front-line management option. This has emphasized the need to better understand tumor behavior in order to differentiate between tumors that may stabilize and those that may progress. Here, we review the most recent findings in desmoid tumor biology and treatment options for this enigmatic disease.