[Prognosis in familial adenomatous polyposis. Results from the Polyposis Registry].

Regional and national polyposis registries have been established all over the world over last decades, with the aim to improve survival in patients with familial adenomatous polyposis (FAP). The Danish Polyposis Register was founded in 1971 and coordinates screening and prophylactic treatment. At the end of 1992 the register included 321 histologically verified FAP patients: 142/205 probands (69%) had colorectal cancer at the time of diagnosis of FAP versus only 2/116 call-up cases (2%). The 10-year cumulative crude survival was 94% among call-up cases versus only 41% among probands (p < 0.00001), and the survival increased significantly after establishment of the Polyposis Register in 1971. We conclude that centralized registration with identification and prophylactic examination of relatives at risk results in a substantial improvement of the prognosis.