Originally described by Altmeier  and Klatskin , hilar cholangiocarcinoma is an adenocarcinoma of the extrahepatic biliary tree arising from the main left or right hepatic ducts or their confluence. Along with distal bile duct cancer and intrahepatic cholangiocarcinoma, they comprise the spectrum of bile duct cancers that arise from the biliary epithelium. However, unlike those tumors, which can be usually be removed, respectively, with pancreaticoduodenectomy or liver resection alone, the surgical approach to hilar cholangiocarcinoma often combines bile duct resection with concomitant hepatectomy and/or portal vein resection due to the infiltrative nature of the disease. Therefore, a complete, margin-negative resection can be difficult to achieve.
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