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Background Rare cancers here defined as those with an annual incidence rate less than 6/100,000 in Europe, pose challenges for diagnosis, treatments, and clinical decision-making. Information on rare cancers is scant. We updated the estimates of the burden of rare cancers in Europe, their time trends in incidence and survival, and provide information on centralization of treatments in seven European countries. Methods We analysed data on more than two million rare cancer diagnoses, provided by 83 cancer registries, to estimate European incidence and survival in 2000-2007 and the corresponding time trends during 1995-2007. Incidence rates were calculated as the number of new cases divided by the corresponding total person years in the population. Five-year relative survival (RS) was calculated by the Ederer-2 method. Seven registries

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SM survival is high and still increasing in European patients. The gap between Northern and Southern and especially Eastern European countries, although still present, diminished over time. Differences in stage distribution at diagnosis may explain most of the geographical differences. However, part of the improvement in survival may be attributed to overdiagnosis from early diagnosis practices.

The distribution of ovarian cancer histology varies widely worldwide. Type I epithelial, germ cell and sex cord-stromal tumours are generally associated with higher survival than type II tumours, so the proportion of these tumours may influence survival estimates for all ovarian cancers combined. The distribution of histological groups should be considered when comparing survival between countries and regions.

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