(1) Sarcoma Biology Laboratory, Memorial Sloan Kettering Cancer Center, 417 East 68th Street, ZRC 445, New York, NY 10065, USA. (2) Department of General Surgery, NYU Grossman School of Medicine, 550 1st Avenue, New York, NY 10016, USA. (3) Gastric and Mixed Tumor Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, H1220, New York, NY 10065, USA. Electronic address: cragoa@mskcc.org.
Publication
Surg Clin North Am
Summary
Desmoid fibromatosis is a rare disease caused by genetic alterations that activate β-catenin. The tumors were previously treated with aggressive surgeries but do not metastasize and may regress spontaneously. For these reasons, in the absence of symptoms and when growth would not induce significant complications, active observation is considered first-line therapy. When intervention is required, surgery can be considered based on anatomy and risk of postoperative recurrence, but increasingly nonoperative therapies such as liposomal doxorubicin or sorafenib are prescribed. Cryoablation, chemoembolization, and high-intensity focused ultrasound can also be used to obtain local control in selected patients.
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