Management of desmoid tumors in patients after ileal pouch-anal anastomosis for familial adenomatous polyposis.
OBJECTIVE
The aim of this study was to review the management of desmoid tumors in patients who have undergone ileal pouch-anal anastomosis for familial adenomatous polyposis and their outcome.


METHODS
A retrospective review of case notes was undertaken in a series of 196 patients with familial adenomatous polyposis who had had an ileal pouch-anal anastomosis. Eleven patients were identified in whom desmoid tumor had developed (4 male; median age, 30 (range, 16-43) years).


RESULTS
Desmoid 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 stable at a median follow-up of eight years. Mesenteric desmoid tumors developed in seven patients; the pouch has been excised in two because of ischemia; two other patients have had defunctioning stomas created because of slow progression of their desmoid tumors. One patient had a single-lumen pouch excised and a new S-pouch inserted, with no subsequent recurrence of the desmoid tumor, and in two patients the mesenteric desmoid tumor has remained unchanged. All of these patients received long-term Clinoril (sulindac, Merck & Co., Inc., West Point, PA). Although there has been no significant deterioration in pouch function in patients in whom the pouch remains in situ, the pouch function was significantly worse compared with a control group of patients after ileal pouch-anal anastomosis for familial adenomatous polyposis in whom desmoid tumors did not develop.


CONCLUSIONS
Desmoid tumors developed in 5.6 percent of 196 patients after ileal pouch-anal anastomosis for familial adenomatous polyposis. No patient with abdominal-wall desmoid tumor had a recurrence after local excision. By contrast, mesenteric desmoid tumors eventually resulted in pouch failure in four of seven affected patients.
Sagar
P M
PM
Division of Colon and Rectal Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Möslein
G
G
Dozois
R R
RR
D000009:Abdominal Muscles; D011125:Adenomatous Polyposis Coli / Q000150:complications / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D005260:Female; D018222:Desmoid Tumors / Q000150:complications / Q000601:surgery*; D006801:Humans; D008297:Male; D011183:Postoperative Complications; D016737:Proctocolectomy, Restorative; D012189:Retrospective Studies; D012983:Soft Tissue Neoplasms / Q000150:complications / Q000601:surgery*