BACKGROUND: Very little data exist on the epidemiology of cholangiocarcinoma in Italy.\ud \ud AIM: We focus on the descriptive epidemiology of cholangiocarcinoma in Italy.\ud \ud METHODS: Data on incidence were obtained from the Italian Association of Tumour Registries while mortality data were obtained from the Italian National Institute of Statistics.\ud \ud RESULTS: A progressive increase of incidence with age was seen for extra-hepatic, intra-hepatic and not otherwise specified cholangiocarcinoma. Crude incidence rates were higher for extra-hepatic cholangiocarcinoma than those for intra-hepatic cholangiocarcinoma and in men compared to women. An increasing incidence trend was observed, from 1988 to 2005, for both extra-hepatic- and intra-hepatic cholangiocarcinoma with a 3-6% yearly increase and with a rate of increase higher for men than for women and for intra-hepatic- than for extra-hepatic cholangiocarcinoma. For intra-hepatic cholangiocarcinoma, the mortality rates progressively increased from 0.15 per million in 1980 to 5.9 per million in 2003, when mortality for this cancer surpassed extra-hepatic cholangiocarcinoma. Mortality rates for extra-hepatic cholangiocarcinoma showed an increasing trend from 1980 to 1994 but, in contrast to intra-hepatic cholangiocarcinoma, a stable or slightly decreasing trend from 1995 to 2003 was observed.\ud \ud CONCLUSIONS: In Italy, cholangiocarcinoma showed a progressive increase in incidence and mortality in the last two decades mainly in intra-hepatic cholangiocarcinoma
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