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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: 1)The impact of radiotherapy in the treatment of desmoid tumours . An international survey of 110 patients.A study of rare cancer network. 2) Radiation-induced sarcoma: 25-year experiencefrom The Norwegian Radium Hospital. 3) Rhabdomyosrcoma cells have an active estrogen receptor-β-mediated pathway that is highly sensitive to estrogen, and undergo apoptosis in response to 4’OH-tamoxifen.
