Survival of patients with hereditary colorectal cancer: Comparison of HNPCC and colorectal cancer in FAP patients with sporadic colorectal cancer

Conflicting data exist on the prognosis of hereditary colorectal cancer. HN PCC patients, in particular, are often reported to have a better survival. We examined 2,340 colorectal-cancer patients treated in our Institution: 14 4 HNPCC patients (Amsterdam Criteria), 161 FAP patients and 2,035 patients with sporadic cancer. Data on hereditary-cancer patients treated between 19 80 and 1995 was collected in a registry. The 2,035 sporadic colorectal-canc er patients (controls) included all new cases treated in the Department of Gastrointestinal-Tract Surgery during the same period. Observed survival wa s estimated using the Kaplan-Meier method. Cumulative survival probability was estimated at 5 years within each group and stratified by various clinic al and pathological variables. The age distribution at diagnosis of sporadi c patients was significantly higher than that of FAP and HNPCC patients (me dian 60 years vs. 43 and 49 years; p < 0.0001). In the HNPCC group, 40% had a right cancer location, vs. 14% in the FAP group and 13% in the sporadic- cancer group. In the sporadic group, 51% were early-stage cancers (Dukes A or B) vs. 48.4% and 52.1% in the FAP and HNPCC groups respectively. In the HNPCC, FAP and sporadic-cancer groups, the 5-year cumulative survival rate was 56.9%, 54.4% and 50.6% respectively. Survival analysis by the Cox propo rtional-hazards method revealed no substantial survival advantage for HNPCC and FAP patients compared with the sporadic group, after adjustment for ag e, gender, stage and tumor location. The hazard ratio for HNPCC was 1.01 (9 5% CI 0.72-1.39) and 1.27 (95% CI 0.95-1.7) for FAP patients compared with the sporadic-colorectal-cancer group. (C) 1999 Wiley-Liss, Inc.