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Annals of Surgery:
doi: 10.1097/SLA.0000000000000734
Original Articles

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†,‡,§

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Abstract

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.

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