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Pathophysiology: Desmoid tumors are cytologically bland fibrous neoplasms originating from the musculoaponeurotic structures throughout the body. Desmoid tumors often appear as infiltrative, usually well-differentiated, firm overgrowths of fibrous tissue, and they are locally aggressive. The synonym aggressive fibromatosis describes the marked cellularity and aggressive local behavior.
Treatment: The primary treatment for desmoid tumor is primary surgery with negative surgical margins. In those patients who refuse surgery or are not surgical candidates, the treatment used is radiation therapy. Pharmacologic therapy with antiestrogens and prostaglandin inhibitors may also be used.
Major research: Low-dose chemotherapy with methotrexate and vinblastine for patients with desmoid tumors: relationship to CTNNB1 mutation status : MTX and VBL treatment every other week is well tolerated and achieved a favorable response in patients resistant to meloxicam treatment, regardless of CTNNB1 mutation status.
