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Chondrosarcoma (CS) epidemiology has been studied by a number of authors using national cancer registry cohorts. Many reports share the common findings of a slight increase in incidence, but not all. The patterns and causes for these changes are divergent while reflection concerning methodological challenges are often missing. We have performed a structured literature review to find national analyses of CS incidence published from 2010 to 2020. We included eight studies of national incidence of CS, summarise their findings and patterns of change. We further discuss explanations given for these changes to better understand the real patterns and raise awareness in their interpretation. Reported crude incidence ranges from 0.27 per million per year overall in Saudi Arabia to 5.4 in the Netherlands. Four studies from the USA, England, Switzerland and France report age standardised rates of 2.0–4.1 per million per year overall. While some countries report stable patterns, most report a slight increase. The Netherlands is the only country reporting a large increase, driven by a 10-fold increase in the incidence of ACT/grade 1 CS during the study period. We challenge the explanations given for this and suggest that this most likely is a result of variable interpretation and definition of CS at the lower levels of disease aggressiveness. This should raise awareness to possible over-treatment of CS in the Netherlands. The most likely national incidence of CS of bone is between 2–4 per million per year. Three modern reports present an incidence of 3.4–4.1 per million per year.

The national incidence of chondrosarcoma of bone; a review