MANAGEMENT OF DESMOID TUMORS IN PATIENTS AFTER ILEAL POUCH-ANAL ANASTOMOSIS FOR FAMILIAL ADENOMATOUS POLYPOSIS

PURPOSE: The aim of this study was to review the management of desmoid tumors in patients who have undergone ileal pouch-anal anastomosis fo r familial adenomatous polyposis and their outcome. METHODS: A retrosp ective review of case notes was undertaken in a series of 196 patients with familial adenomatous polyposis who had had an ileal pouch-anal a nastomosis. Eleven patients were identified in whom desmoid tumor had developed (4 male; median age, 30 (range, 16-43) years). RESULTS: Desm oid tumors developed in the abdominal wall alone in 4 of 11 patients. Three enlarging tumors were resected with no subsequent recurrence. No patient received adjuvant treatment and pouch function remained stabl e at a median follow-up of eight years. Mesenteric desmoid tumors deve loped in seven patients; the pouch has been excised in two because of ischemia; two other patients have had defunctioning stomas created bec ause of slow progression of their desmoid tumors. One patient had a si ngle-lumen pouch excised and a new S-pouch inserted, with no subsequen t recurrence of the desmoid tumor, and in two patients the mesenteric desmoid tumor has remained unchanged. All of these patients received l ong-term Clinoril(R) (sulindac, Merck & Co., Inc., West Point, PA). Al though there has been no significant deterioration in pouch function i n patients in whom the pouch remains in situ, the pouch function was s ignificantly worse compared with a control group of patients after ile al pouch-anal anastomosis for familial adenomatous polyposis in whom d esmoid tumors did not develop. CONCLUSION: Desmoid tumors developed in 5.6 percent of 196 patients after ileal pouch-anal anastomosis for fa milial adenomatous polyposis. No patient with abdominal-wall desmoid t umor had a recurrence after local excision. By contrast, mesenteric de smoid tumors eventually resulted in pouch failure in four of seven aff ected patients.