Centralized registration, prophylactic examination, and treatment results in improved prognosis in familial adenomatous polyposis. Results from the Danish Polyposis Register.
BACKGROUND
Over the last few decades numerous regional and national registers have been established all over the world with the aim of improving survival in familial adenomatous polyposis (FAP). The Danish Polyposis Register was founded in 1971 and coordinates the screening and subsequent prophylactic colectomy of FAP patients.


METHODS
The crude cumulative survival in 321 patients (205 probands and 116 call-up cases) with verified FAP was calculated in accordance with the life-table method.


RESULTS
At the time of diagnosis of FAP only 2 of 116 (2%) had colorectal cancer versus 142 of 205 probands (69%). The 10-year cumulative survival was 94% (95% confidence limits, 89-99) in call-up cases compared with only 41% (34-49) in probands (p < 0.00001), and survival improved significantly (p < 0.00001) after the establishment of the Danish Polyposis Register.


CONCLUSIONS
The establishment of a centralized polyposis register has resulted in a substantial improvement of the prognosis in FAP.
Bülow
S
S
Dept. of Surgical Gastroenterology, Hvidovre Hospital, Copenhagen, Denmark.
Bülow
C
C
Nielsen
T F
TF
Karlsen
L
L
Moesgaard
F
F
D011125:Adenomatous Polyposis Coli / Q000453:epidemiology* / Q000517:prevention & control / Q000601:surgery; D015179:Colorectal Neoplasms / Q000453:epidemiology; D003718:Denmark / Q000453:epidemiology; D006801:Humans; D015993:Life Tables; D008403:Mass Screening; D011379:Prognosis; D012042:Registries; D015996:Survival Rate