RARECARE - Surveillance of Rare Cancers in Europe

Prevalence
Fifteen-year prevalence for all the rare tumours, except the Epithelial Tumours of
Cervix Uteri, were under the 50 per 100,000, that is the cut off utilized in Europe for
the definition of rare disease. By contrast, Epithelial Tumours of Oesophagus,
Pancreas, Ovary and Stomach have prevalence rates lower than 50 per 100,000 but
annual incidence rates higher than 6 per 100,000. These four tumors are then
classified as frequent according to our incidence-based definition, but rare according
to the standard EU prevalence-based criterion. All these tumours have very poor
survival and therefore low prevalence figures, even in presence of a relatively high
risk of occurrence, at least for the European population.