A COHORT STUDY OF CYSTIC-FIBROSIS AND MALIGNANCY

A cohort of 412 patients first attending a cystic fibrosis (CF) clinic between 1961 and 1989 were followed up to 30 June 1989. The number of malignancies observed in the cohort was compared with the number expe cted based on the age, sex and calendar-year-specific cancer registrat ion rates for England and Wales. Four CF patients were diagnosed as ha ving malignancies before 30 June 1989. The tumours were: adenocarcinom a of the terminal ileum; adenocarcinoma of the pancreas, testicular te ratoma, and B-cell lymphoma. This compares with 0.89 malignancies expe cted on the basis of rates in England and Wales (Standardised Registra tion Ratio = 452; 95% confidence interval 122-1150, P = 0.03). The sin gle case of adenocarcinoma of the terminal ileum contrasts with less t han 0.001 expected (P = 0.003) and that of the pancreas with 0.007 exp ected (P = 0.01). A further adenocarcinoma of the pancreas was diagnos ed 2 years after the end of the study period. The two cases of pancrea tic cancer compare with 0.008 expected (P = 0.0001) during the period to mid 1991. On the basis of the present findings and previous case re ports in the literature adenocarcinoma of the pancreas and adenocarcin oma of the terminal ileum may be associated with cystic fibrosis.