Desmoid-type fibromatosis is a rare disease that does not have metastatic potential. It can be locally aggressive or regress spontaneously. For this reason, and because many of these tumors are inoperable or surgery is associated with significant morbidity, nonoperative treatment modalities are now more commonly employed to manage these lesions. Active observation, ablative therapies, and systemic treatments such as doxorubicin-based chemotherapy or sorafenib can be prescribed to minimize morbidities associated with the disease and surgical intervention. A thorough understanding regarding desmoid biology and the relative indications for multimodality therapies is essential to triage patients for appropriate interventions. This review contains 5 figures, and 29 references. Key Words: active observation, β-catenin, desmoid, familial adenomatous polyposis, fibromatosis, soft tissue tumors
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