Extracolonic Manifestations of Familial Adenomatous Polyposis After Proctocolectomy | Colorectal Cancer | JAMA Surgery | JAMA Network
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Original Article
February 1, 2005

Extracolonic Manifestations of Familial Adenomatous Polyposis After Proctocolectomy

Author Affiliations

Author Affiliations: Colorectal Unit, Department of Surgery B (Drs Tulchinsky, Keidar, Goldman, Klausner, and Rabau) and Gastroenterology (Dr Strul), Tel Aviv Sourasky Medical Center, affiliated with the Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel. Dr Keidar is now with the Department of Surgery B, Meir Hospital, Kfar Saba, Israel.

Arch Surg. 2005;140(2):159-163. doi:10.1001/archsurg.140.2.159

Hypothesis  Extracolonic manifestations have a major effect on the morbidity and mortality of patients with familial adenomatous polyposis following proctocolectomy.

Design  Case review study.

Setting  Colorectal unit, university-affiliated hospital.

Patients  Fifty patients (25 males and 25 females) with familial adenomatous polyposis WHO underwent proctocolectomy between January 1988 and October 2003.

Interventions  Ileal pouch–anal anastomosis (n = 41), Kock pouch (n = 1), end ileostomy (n = 6). Two patients underwent total colectomy with an ileorectal anastomosis.

Main Outcome Measures  Clinical follow-up and telephone interview; contact with clinicians following up patients elsewhere.

Results  The patients’ median age at surgery was 33 years. The mean length of follow-up was 74 months. Four patients were lost to follow-up. Extracolonic manifestations were diagnosed in 38 patients (76%). Twelve patients had 14 desmoid tumors: 7 were treated surgically and 7 medically (these patients received celecoxib and tamoxifen citrate therapy). Of the 41 patients who underwent upper gastrointestinal tract endoscopy, 11 developed duodenal and/or ampullary adenomas. Three patients had endoscopic polypectomy and 1 underwent a Whipple operation. Among the 29 patients who underwent pouchoscopy, 5 had pouch adenomas and 3 had adenomas that were found in the rectal stump. Two patients died—one of a huge mesenteric desmoid tumor and the other of an aggressive mesenteric malignant fibrous histiocytoma.

Conclusions  Long-term morbidity and mortality were strongly related to the development of mesenteric tumors and ampullary-duodenal polyps. Early detection of desmoid tumors, duodenal, pouch, and rectal cuff adenomas by periodic computed tomography, gastroduodenoscopy, and pouchoscopy, respectively, may allow control by medical therapy, endoscopy, or limited surgical procedures. In most patients control of desmoid tumors was achieved using a combination of celecoxib and tamoxifen citrate therapy.