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: TALEN-mediated apc mutation in Xenopus tropicalis phenocopies familial adenomatous polyposis: Tadpoles and froglets derived from embryos injected with TAL effector nucleases targeting the apc gene rapidly developed intestinal hyperplasia and other neoplasms observed in FAP patients, including desmoid tumors and medulloblastomas.