Abstract
Desmoid tumors (DTs) are deep fibroblastic neoplasms that arise from musculo-aponeurotic stromal elements. DTs may result in significant morbidity by infiltrating vital anatomic structures. Their mortality is often due to the local aggressiveness, most commonly when intra-abdominal in location. Some indolent DTs can be observed expectantly; infiltrative tumors require an aggressive and multidisciplinary approach and are offered conservative therapies such as nonsteroidal anti-inflammatory drugs or antiestrogens when surgery is not feasible. Comparably, chemotherapy is considered for those cases not amenable to surgery or radiation. Bowel resection and at times intestinal transplantation may be necessary. However, DTs may recur postsurgery making long-term management of these patients. Herein, we review the genetics, clinical presentations, outcomes, and treatments of DTs.