Evolving Concepts in the Management of Desmoid Tumors.

Abstract

DT is a rare monoclonal fibroblastic neoplasm with an unpredictable biologic behavior. Over the past 15 y, the worldwide approach to desmoid tumor (DT) has shifted dramatically from surgery, when feasible, to frontline active surveillance in the majority of cases, to select patients who truly require treatment. Approximately 50% of cases demonstrate indolent disease, while the remaining patients may require active treatment. Surgery and radiotherapy still have limited indications, but 3 recent randomized trials have provided new insights into medical treatments. This review will highlight the evolution of global concepts and strategies.