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Progression and Management of Duodenal Neoplasia in Familial Adenomatous Polyposis: A Cohort Study

Serrano, Pablo E. MD, MPH, MSc*; Grant, Robert C. MA; Berk, Terri C. MSSA; Kim, Dowan HBSc§; Al-Ali, Hassan MD§; Cohen, Zane MD†,‡; Pollett, Aaron MD; Riddell, Robert MD; Silverberg, Mark S. MD; Kortan, Paul MD; May, Gary R. MD; Gallinger, Steven MD, MSc†,‡,§

doi: 10.1097/SLA.0000000000000734
Original Articles

Objective: To describe the natural history and outcomes of surveillance of duodenal neoplasia in familial adenomatous polyposis (FAP).

Background: Duodenal cancer is the most common cause of death in FAP.

Methods: Cohort study of patients prospectively enrolled in an upper endoscopic surveillance protocol from 1982 to 2012. The duodenum was assessed by side-viewing endoscopy and classified as stage 1 to 5 disease. Endoscopic and/or operative interventions were performed according to stage.

Results: There were 218 patients in the protocol (98 with advanced stage). They had a median of 9 endoscopies (range: 2–25) over a median of 11 years (range: 1–26). Median age at diagnosis of stage 3 disease (adenoma: 2.1–10 mm) was 41 years and stage 4 disease (adenoma >10 mm) was 45 years. Median time from first esophagogastroduodenoscopy to stage 4 disease was 22.4 years. The risk of stage 4 disease was 34.3% [95% confidence interval (CI) 23.8–43.4] at 15 years. In multivariate analysis, sex, type of colorectal surgery, years since colorectal surgery, and stage were significantly associated with risk of progression to stage 4 disease. Five of 218 (2.3%) patients developed duodenal cancer at median age of 58 years (range: 51–65). The risk of developing duodenal cancer was 2.1% (95% CI: 0–5.2) at 15 years.

Conclusions: Patients with advanced duodenal polyposis progress in the severity of disease (size and degree of dysplasia); however, the rate of progression to carcinoma is slow. Aggressive endoscopic and surgical intervention, especially in the presence of large polyps and high-grade dysplasia, appears to be effective in preventing cancer deaths in FAP.

This review analyzes the long-term outcomes of 218 patients with familial adenomatous polyposis enrolled in a prospective upper endoscopy surveillance program. We found a 34.3% (95% confidence interval: 23.8–43.4) risk of progression to high-stage duodenal disease by 15 years and a 2.1% (95% confidence interval: 0–5.2) risk of developing duodenal cancer at 15 years.

*Department of Surgery, McMaster University, Hamilton, Ontario, Canada

Samuel Lunenfeld Research Institute, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada

Familial Gastrointestinal Cancer Registry, Mount Sinai Hospital, Toronto, Ontario, Canada

§Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada

Department of Pathology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada; and

Division of Gastroenterology, St Michael's Hospital, University of Toronto, Toronto, Ontario, Canada.

Reprints: Steven Gallinger, MD, Toronto General Hospital, 10EN-215, 200 Elizabeth Street, Toronto, ON M5G 2C4, Canada (

Disclosure: There are no conflicts of interest to disclose for this study. The authors attest that they have herein disclosed any and all financial or other relationships that could be construed as a conflict of interest and that all sources of financial support for this study have been disclosed.

Presented in part at the 16th Annual Meeting, Collaborative Group of the Americas on Inherited Colorectal Cancer (CGA-ICC), October 2012, Boston, MA.

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