Abstract
PURPOSE: 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 remainsin 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. CONCLUSION: 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.
Similar content being viewed by others
References
Reitamo JJ, Hayry P, Nykri E, Saxen E. The desmoid tumor. I Incidence, sex, age and anatomical distribution in the Finnish population. Am J Clin Pathol 1982;77:665–73.
Gurbuz AK, Giardiello FM, Petersen GM,et al. Desmoid tumors in familial adenomatous polyposis. Gut 1994;35:377–81.
Arvanitis ML, Jagelman DG, Fazio VW, Lavery IC, McGannon E. Mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 1990;33:639–42.
Vasen HF, Griffioen G, Offerhaus GJ,et al. The value of screening and central registration of families with familial adenomatous polyposi: a study of 82 families in The Netherlands. Dis Colon Rectum 1990;33:227–30.
Penna C, Tiret E, Parc R,et al. Operation and abdominal desmoid tumors in familial adenomatous polyposis. Surg Gynecol Obstet 1993;177:263–8.
Waddell WR. Treatment of intra-abdominal and abdominal wall desmoid tumors with drugs that affect the metabolism of cyclic 3′,5′-adenosine monophosphate. Ann Surg 1975;181:299–302.
Clark SK, Phillips RK. Desmoids in familial adenomatous polyposis. Br J Surg 1996;83:1494–504.
Brooks AP, Reznek RH, Nugent K, Farmer KC, Thomson JP, Phillips RK. CT appearances of desmoid tumours in familial adenomatous polyposis; further observations. Clin Radiol 1994;49:601–7.
Lynch HT, Fitzgibbons R Jr, Chong S,et al. Use of doxorubicin and dacarbazine for the management of unresectable intra-abominal desmoid tumors in Gardner's syndrome. Dis Colon Rectum 1994;37:260–7.
Posner MC, Shiu MH, Newsome JL, Hajdu SI, Gaynor JJ, Brennan MF. The desmoid tumor. Not a benign disease. Arch Surg 1989;124:191–6.
Sheridan R, D'Avis J, Seyfer AE, Quispe G. Massive abdominal wall desmoid tumor: treatment by resection and abdominal wall reconstruction. Dis Colon Rectum 1986;29:518–20.
Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig EB. Intra-abdominal fibromatosis: a pathological analysis of 130 tumors with comparison of clinical subgroups. Am J Surg Pathol 1990;14:335–41.
Lotfi AM, Dozois, RR, Gordon H,et al. Mesenteric fibromatosis complicating familial adenomatous polyposis: predisposing factors and results of treatment. Int J Colorect Dis 1989;4:30–6.
Calne RY, Pollard SG, Jamieson NV,et al. Intestinal transplant for recurring mesenteric desmoid tumour. Lancet 1993;342:58–9.
Hial V, Horakova Z, Shaff FE, Beaven MA. Alteration of tumor growth by aspirin and indomethacin: studies with two transplantable tumors in mouse. Eur J Pharmacol 1976;37:367–76.
Kinzbrunner B, Ritter S, Domingo J, Rosenthal CJ. Remission of rapidly growing desmoid tumors after tamoxifen therapy. Cancer 1983;52:2201–4.
Benson JR, Baum M. Breast cancer, desmoid tumours, and familial adenomatous polyposis—a unifying hypothesis. Lancet 1993;342:1561–2.
Colletta AA, Wakefield LM, Howell FV,et al. Antioestrogens induce the secretion of active transforming growth factor beta from human fetal fibroblasts. Br J Cancer 1990;62:405–9.
Patel SR, Evans HL, Benjamin RS. Combination chemotherapy in adult desmoid tumors. Cancer 1993;72:3244–7.
Author information
Authors and Affiliations
Additional information
Read at the meeting of The American Society of Colon & Rectal Surgeons, Philadelphia, Pennsylvania, June 22 to 26, 1997.
About this article
Cite this article
Sagar, P.M., Möslein, G. & Dozois, R.R. Management of desmoid tumors in patients after ileal pouch-anal anastomosis for familial adenomatous polyposis. Dis Colon Rectum 41, 1350–1355 (1998). https://doi.org/10.1007/BF02237046
Issue Date:
DOI: https://doi.org/10.1007/BF02237046