Carcinoma of the intra- and extrahepatic biliary tract is a relatively rare cancer. In most cases, the etiology of these cancers is rather obscure. Depending on the location of the carcinoma there are some risk factors identified such as hepatolithiasis and biliary calculi, congenital cystic and dysplastic lesions of the biliary tract, inflammatory bowel disease, thorotrast application, activated biliary bile acids, reflux and stasis of pancreatic juice, infection with the liver flukes Clonorchis sinensis (CL) and Opisthorchis viverrini (OV) and longstanding cholangitis. Multiple studies suggest that carcinogenesis in biliary epithelia is a multiple and multistage process through hyperplasia, metaplasia, adenoma, dysplasia, adenocarcinoma in situ to invasive adenocarcinoma in most cases of biliary cancer. Biliary carcinogenesis is caused by different mechanisms such as DNA damage produced by nitric oxide and by reactive oxygen species in infected and inflamed tissue, specific mutation in protooncogenes and tumor suppressor genes, mechanically damaged tissue by biliary calculi or parasites, reflux and stasis of pancreatic enzymes with activation of biliary acids and some other unidentified factors. Furthermore, some epidemiological facts and prevention strategies in relation to biliary cancer will be addressed based on a review of the literature.

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