Summary of Chapter 2: Identification, Surveillance and Management of Polyposis Syndrome

Abstract

The Danish Polyposis Register (DPR) was established in 1971. Probands were ascertained from hospitals, the National Patient Registry and from the Cancer Registry. Their pedigrees were then constructed from genealogical information (from local population registers, parish registers and the Central Population Register) and from medical information (hospitals, specialists in private practice and death certificates). Hitherto unexamined 1. relatives were then offered prophylactic examination with regular proctosigmoidoscopy at regular intervals in the age group 10–60 years and newly diagnosed “call-up cases” referred for prophylactic colectomy. In 1989 the DPR includes 121 families with 381 affected members, of whom 110 are call-up cases. The incidence of colorectal cancer in this group was only 2% versus 67% in probands. The cumulative survival after 10 years was 93% in call-up patients versus only 40% in probands. These differences strongly underline the value of a centralized polyposis register.