Endoscopy 2019; 51(04): S127
DOI: 10.1055/s-0039-1681545
ESGE Days 2019 oral presentations
Saturday, April 6, 2019 14:30 – 16:00: Genetic Club H
Georg Thieme Verlag KG Stuttgart · New York

COLORECTAL CANCER IN FAMILIAL ADENOMATOUS POLYPOSIS: RESULTS FROM THE DANISH POLYPOSIS REGISTRY

J Gásdal Karstensen
1   Gastrounit, Danish Polyposis Registry, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark
2   Department of Gastrointestinal Surgery, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
,
J Burisch
1   Gastrounit, Danish Polyposis Registry, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark
,
HC Pommergaard
1   Gastrounit, Danish Polyposis Registry, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark
2   Department of Gastrointestinal Surgery, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
,
H Højen
1   Gastrounit, Danish Polyposis Registry, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark
,
N Jespersen
1   Gastrounit, Danish Polyposis Registry, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark
,
L Aalling
1   Gastrounit, Danish Polyposis Registry, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark
,
P Nordblad Schmidt
1   Gastrounit, Danish Polyposis Registry, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark
,
S Bülow
1   Gastrounit, Danish Polyposis Registry, Copenhagen University Hospital Hvidovre, Hvidovre, Denmark
› Author Affiliations
Further Information

Publication History

Publication Date:
18 March 2019 (online)

 

Aims:

Familial adenomatous polyposis (FAP) is an autosomal dominant disorder that predisposes to colorectal cancer (CRC). The Danish Polyposis Registry is a nationwide, complete registry of FAP patients that coordinates a surveillance program consisting of 1) tracing and genotyping of 1st degree relatives and 2) annual or biannual colonoscopies until referral to colectomy or proctocolectomy. We aimed this study to assess efficacy of the surveillance program including the incidence, prevalence, and crude survival rates of CRC for Danish FAP patients.

Methods:

Data was collected from the Danish Polyposis Registry and the periods 1990 – 99 and 2000 – 17 were compared.

Results:

By the end of 2017, the registry comprised 226 families with 721 affected individuals. While the mean annual incidence rate of FAP was stable from 1990 – 99 (0.19/100,000/year) to 2000 – 17 (0.32/100,000/year) (p= 0.91), the point prevalence increased significantly from 4.86/100,000 in 1999 to 6.11/100,000 by the end of 2017 (p= 0.005). During the period 2000 – 17, FAP related CRC constituted 25/72,218 of all CRC cases (0.03%), which was a significant decrease from 1990 – 99 (26/30,005 cases, 0.09%) (p= 0.001). The risk of CRC was significantly higher for probands (n = 191, 61.6%) compared to call-up cases (n = 5, 1.9%) (p< 0.001). All CRCs in call-up patients were detected at the initial endoscopic evaluation. Thus, no cases were identified in the surveillance program. The life expectancy for probands differed significantly from call-up patients (69.9 years, 95% CI, 66.9 – 72.9 vs 56.1 years, 95% CI, 53.6 – 58.6, p< 0.001); hence, an appropriate tracing and preoperative endoscopic surveillance program prolong life expectancy with 13.4 years for first-degree family members.

Conclusions:

The Danish Polyposis Registry enables close monitoring of FAP patients resulting in a minimized risk of CRC and a prolonged life expectancy within the surveillance programme. Establishment of further national polyposis registries is strongly recommended in order to globally secure high quality patient care for FAP patients.