Rare cancers in Canada, 2006–2016: A population-based surveillance report and comparison of different methods for classifying rare cancers

Abstract Background The cumulative burden from rare cancers has not been adequately explored in Canada. This analysis aims to characterize the occurrence of rare cancers among Canadians and estimate the probability of being diagnosed with a rare cancer among cancer patients with different demographic characteristics. Methods The Canadian Cancer Registry was used for this analysis. Cancer types were classified in three ways: using the SEER site recode scheme; by histology group; and by site/histology group. The age-standardized incidence rate (ASIR) and 95 % confidence intervals (CI) for each cancer type was estimated for diagnoses from 2006 to 2016. Two ASIR thresholds were used to classify cancers as rare:6/100,000/year and 15/100,000/year. Log-binomial regression was used to estimate the adjusted probability of having a rare cancer among those with cancer by age, sex and geographic region. Results Using the 6/100,000/year threshold, the incidence proportion (IP) of rare cancers ranged from 9.7 %(95 %CI:9.6,9.7 %)-17.0 %(95 %CI:16.9,17.0 %), and ranged from 19.2 %(95 %CI:19.1,19.3 %)-52.5 %(95 %CI:52.0,53.0 %) using the Interpretation This analysis yielded evidence that rare cancers comprise a substantial proportion of annual cancer diagnoses among Canadians. Findings from this analysis point to using a lower incidence rate threshold, to generate estimates of the burden of rare cancers that are robust to different cancer classification schemes.