Abstract

Cholangiocarcinoma (CCA) is the primary cancer of the biliary tree. It is broadly divided into intrahepatic, extrahepatic hilar tumors and extrahepatic distal bile duct tumors, according to its anatomic distribution within the biliary tree [1]. It is an epithelial neoplasm, adenocarcinoma in most cases. Although it comprises about 10%–15% of hepatobiliary neoplasms, its incidence is increasing globally [2, 3]. The annual incidence of CCA is approximately 1.0 per 100,000 in the United States, 7.3 per 100,000 in Israel, 6.5 per 100,000 among American Indians, 5.5 per 100,000 in Japan [4], and 2 per 100,000 in England and Wales [5]. However, this increase is mainly due to a sharp rise in the incidence of intrahepatic cholangiocarcinoma, whereas the incidence of extrahepatic cholangiocarcinoma seems to have declined the last two decades [6]. Using the Surveillance Epidemiology and End Results (SEER) database, which represents 10–14% of the total US population, the age adjusted incidence of intrahepatic CCA has increased by 165%, from 0.32 per 100,000 population during 1975–1979 to 0.85 per 100,000 during 1995–1999 [2, 12]

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