TY - JOUR

T1 - Cancer in Patients With Familial Adenomatous Polyposis

T2 - A Nationwide Danish Cohort Study With Matched Controls

AU - Karstensen, John Gásdal

AU - Bülow, Steffen

AU - Højen, Helle

AU - Jelsig, Anne Marie

AU - Jespersen, Niels

AU - Andersen, Klaus Kaae

AU - Wewer, Mads Damsgaard

AU - Burisch, Johan

AU - Pommergaard, Hans Christian

N1 - Publisher Copyright: © 2023 The Authors

PY - 2023

Y1 - 2023

N2 - Background & Aims: Familial adenomatous polyposis (FAP) is a hereditary disorder that predisposes patients to colorectal cancer (CRC). Prophylactic colectomy has greatly reduced the risk of CRC. However, new associations between FAP and the risk of other cancers have subsequently emerged. In this study, we assessed the risk of specific primary and secondary cancers among patients with FAP compared with matched controls. Methods: All known patients with FAP up until April 2021 were identified in the nationwide Danish Polyposis Register and paired with 4 unique controls matched by birth year, sex, and postal code. The risk of overall cancers, specific cancer types, and risk of a second primary cancer was assessed and compared with controls. Results: The analysis included 565 patients with FAP and 1890 controls. The overall risk of cancer was significantly higher for patients with FAP than for controls (hazard ratio [HR], 4.12; 95% confidence interval [CI], 3.28–5.17; P <.001). The increased risk was mainly due to CRC (HR, 4.61; 95% CI, 2.58–8.22; P <.001), pancreatic cancer (HR, 6.45; 95% CI, 2.02–20.64; P =.002), and duodenal/small-bowel cancer (HR, 14.49; 95% CI, 1.76–119.47; P =.013), whereas no significant difference was observed for gastric cancer (HR, 3.29; 95% CI, 0.53–20.23; P =.20). Furthermore, the risk of a second primary cancer was significantly higher for patients with FAP (HR, 1.89; 95% CI, 1.02–3.50; P =.042). Between 1980 and 2020, the risk of cancer among patients with FAP decreased by ∼50%. Conclusions: Despite an absolute reduction in the risk of developing cancer among patients with FAP, the risk remained significantly higher than for the background population due to colorectal, pancreatic, and duodenal/small-bowel cancers.

AB - Background & Aims: Familial adenomatous polyposis (FAP) is a hereditary disorder that predisposes patients to colorectal cancer (CRC). Prophylactic colectomy has greatly reduced the risk of CRC. However, new associations between FAP and the risk of other cancers have subsequently emerged. In this study, we assessed the risk of specific primary and secondary cancers among patients with FAP compared with matched controls. Methods: All known patients with FAP up until April 2021 were identified in the nationwide Danish Polyposis Register and paired with 4 unique controls matched by birth year, sex, and postal code. The risk of overall cancers, specific cancer types, and risk of a second primary cancer was assessed and compared with controls. Results: The analysis included 565 patients with FAP and 1890 controls. The overall risk of cancer was significantly higher for patients with FAP than for controls (hazard ratio [HR], 4.12; 95% confidence interval [CI], 3.28–5.17; P <.001). The increased risk was mainly due to CRC (HR, 4.61; 95% CI, 2.58–8.22; P <.001), pancreatic cancer (HR, 6.45; 95% CI, 2.02–20.64; P =.002), and duodenal/small-bowel cancer (HR, 14.49; 95% CI, 1.76–119.47; P =.013), whereas no significant difference was observed for gastric cancer (HR, 3.29; 95% CI, 0.53–20.23; P =.20). Furthermore, the risk of a second primary cancer was significantly higher for patients with FAP (HR, 1.89; 95% CI, 1.02–3.50; P =.042). Between 1980 and 2020, the risk of cancer among patients with FAP decreased by ∼50%. Conclusions: Despite an absolute reduction in the risk of developing cancer among patients with FAP, the risk remained significantly higher than for the background population due to colorectal, pancreatic, and duodenal/small-bowel cancers.

KW - Adenomatous Polyposis Coli Gene

KW - Colorectal Cancer

KW - Familial Adenomatous Polyposis

KW - Gastric Cancer

KW - Pancreatic Cancer

U2 - 10.1053/j.gastro.2023.05.010

DO - 10.1053/j.gastro.2023.05.010

M3 - Journal article

C2 - 37201686

AN - SCOPUS:85166983722

VL - 165

SP - 573-581.e3

JO - Gastroenterology

JF - Gastroenterology

SN - 0016-5085

IS - 3

ER -