DOI: 10.36347/SASJS.2019.v05i09.009

Pages: 373-376

Desmoid tumors (TD) are rare tumors that infiltrate tissues and have no metastatic potential. They are most often sporadic and in 5 to 10% of cases associated with familial adenomatous polyposes (PAF). They usually appear in the young adult, more rarely in the child. We report the case of a 21-year-old who had undergone total colectomy due to PAF with de novo APC mutation, usually not associated with TD. Three years later, she presented an occlusive sd with vomiting. The CT scan revealed a mesenteric TD, about 20 cm long axis invading the ileo rectal anastomosis. The procedure consisted of a resection of the tumor of the part of the rectum invaded with preparation of a low ileo rectal anastomosis. This observation recalls the importance of regular screening of patients with FAP, especially after surgery, regardless of age and type of mutation. Progress remains to be made to determine the risk factors for developing TD in these subjects and to treat them effectively. Current therapeutic management remains difficult because of the infiltrative and recurrent nature of these tumors and requires a multidisciplinary opinion.