source: Journal of Clinical Gastroenterology

year: 2016

authors: Koskenvuo L, Pitkäniemi J, Rantanen M, Lepistö A

GOALS:

Our aim was to determine whether the screening of family members of familial adenomatous polyposis (FAP) patients significantly influences survival, and to gauge the extent of FAP-related causes of death.

BACKGROUND:

The screening of families with FAP has been shown to be profitable in reducing colorectal cancer-related mortality, but conclusions about the screening effect on overall survival has been controversial.

STUDY:

This is a nationwide population-based retrospective cohort study, and the primary outcome of interest was overall mortality and survival. A total of 154 families with at least 1 clinically diagnosed FAP patient between 1963 and 2015 were included. There were altogether 194 probands and 225 call-ups. During the follow-up period, 2639 person-years with 92 deaths among probands were observed and 3634 person-years and 30 deaths among call-ups. We report crude mortality rates and standardized mortality ratios together with descriptive statistics. We compared the survival of probands and call-ups to the population by relative survival method.

RESULTS:

The crude mortality rate among probands was 34.9 per 1000 person-years and 8.3 among call-ups. The standardized mortality ratios for call-ups was 2.47 (confidence interval, 1.69-3.46) and for probands 4.07 (confidence interval, 3.29-4.96) (P=0.014). The relative survival of probands was significantly lower than call-ups (P=0.0018), and 20-year relative survival for call-ups was 94% (88% to 100%). Over two thirds of all deaths were FAP related.

CONCLUSIONS:

Survival of screened family members of FAP patients is comparable to the general population within 20 years after diagnosis. Therefore, participation in surveillance should not be delayed when a family member with FAP has been detected.