Geographical and spatial disparities in the incidence and survival of rare cancers in Australia.

Rare cancers collectively account for around a quarter of cancer diagnoses and deaths. However, epidemiological studies are sparse. We describe spatial and geographical patterns in incidence and survival of rare cancers across Australia using a population-based cancer registry cohort of rare cancer cases diagnosed among Australians aged at least 15 years, 2007 to 2016. Rare cancers were defined using site- and histology-based categories from the European RARECARE study, as individual cancer types having crude annual incidence rates of less than 6/100 000. Incidence and survival patterns were modelled with generalised linear and Bayesian spatial Leroux models. Spatial heterogeneity was tested using the maximised excess events test. Rare cancers (n = 268 070) collectively comprised 22% of all invasive cancer diagnoses and accounted for 27% of all cancer-related deaths in Australia, 2007 to 2016 with an overall 5-year relative survival of around 53%. Males and those living in more remote or more disadvantaged areas had higher incidence but lower survival. There was substantial evidence for spatial variation in both incidence and survival for rare cancers between small geographical areas across Australia, with similar patterns so that those areas with higher incidence tended to have lower survival. Rare cancers are a substantial health burden in Australia. Our study has highlighted the need to better understand the higher burden of these cancers in rural and disadvantaged regions where the logistical challenges in their diagnosis, treatment and support are magnified.