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: Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: Analysis of 114 patients at a single institution : Desmoid-type fibromatoses (DFs) are rare soft-tissue neoplasms with frequent local recurrence. We sought to determine the prognostic factors that are predictive of recurrence-free survival (RFS) for these tumors.