Impact of screening examinations on survival in familial adenomatous polyposis

Background: Prophylactic family screening and surgery has improved the outc ome of patients with familial adenomatous polyposis (FAP) largely preventin g deaths due to colorectal cancer. The present study compared the mortality rates and causes of death of FAP patients diagnosed by symptoms (probands) or by family screening (call-up). Methods: The study comprised all 236 FAP patients registered in the Finnish Polyposis Registry until the end of Jun e 1998. There were 116 probands and 120 call-up patients with a median age of 36.8 and 22.8 at diagnosis and median follow-up times of 6.3 and 9.9 yea rs, respectively. Cumulative crude and relative survival estimates were cal culated for each group and the causes of death were determined. Results: Th e life expectancy was significantly better in the call-up group than in the probands after colectomy (P < 0.001). The survival rates of the call-up gr oup equaled those expected for a comparable group in the general population up to Is years after colectomy. The main cause of death was colorectal can cer accounting for 54 out of 68 deaths: four in the call-up group (atl rect al stump cancer) and 50 in probands. Upper GI-tract cancer caused four deat hs (periampullary cancer two, stomach cancer two) and two deaths were due t o postoperative pulmonary embolism. Conclusion: The survival of FAP patient s is significantly improved by prophylactic screening and surgery. Further improvement may bz possible by using restorative proctocolectomy instead of colectomy and ileorectal anastomosis and by regular upper GI-tract endosco pic surveillance.