The primary goal of a surgical treatment should be the avoidance of the patient dying of colorectal cancer. In patients with multiple polyps in the rectum a total proctocolectomy should be performed with or without an ileal pouch anal anastomosis. In a patient with minimal rectal polyps that can be easily removed and who is compliant with a postoperative surveillance program, an ileorectal anastomosis should be considered. Further clinical and molecular studies will be needed to determine whether age of onset of the polyps or overall age of the patient is more critical in the adenoma to carcinoma sequence of these late presenting FAP patients. The mechanism of action is related to inhibition of prostaglandin synthase enzymes that decrease prostaglandin levels in tissues.
FAP patients are at risk for developing aggressive fibromas or desmoid tumors.
Surgery is a risk factor for the development of these tumors with even small laparoscopic wounds having been reported as having developed tumors.26 Treatment of desmoids should be offered when tumors are enlarging or symptomatic. Surgical excision can be performed for many lesions but some lesions may be unresectable because of proximity to major blood vessels or other organs. Chemotherapy treatments have had some success. More aggressive chemotherapy regimens have also been tried. Summary Familial Adenomatous Polyposis is a genetic disease that is one of the molecular paradigms for the adenoma to carcinoma sequence.