BACKGROUND:Cholangiocarcinoma(CCA)is a lethal cancer of the biliary epithelium,originating from the liver(intrahepatic),at the confluence of the right and left hepatic ducts(hilar)or in the extrahepatic bile ducts.It is a rare malignancy associated with poor prognosis.DATA SOURCES:We searched the PubMed/MEDLINE database for relevant articles published from 1989 to 2008.The search terms used were related to “cholangiocarcinoma” and its “ treatment”.Although no language restrictions were imposed initially,for the full-text review and final analysis,our resources only permitted the review of articles published in English.This review deals with the treatment of cholangiocarcinoma,the principles and the current trends.RESULTS:The risks and prognostic factors,symptoms and differential diagnosis are thoroughly discussed.In addition,the tools of preoperative diagnosis such as endoscopic retrograde cholangiopancreatography,digital image analysis,fluorescence in situ hybridization and magnetic resonance cholangiopancreatography are reviewed.Moreover,the treatment of CCA is discussed.CONCLUSIONS:The only curative treatment available is surgical management.Unfortunately,many patients present with unresectable tumors,the majority of whom die within a year of diagnosis.Surgical treatment involves major resections of the liver,pancreas and bile duct,with considerable mortality and morbidity.However,in selected cases and where indicated,appropriate management with aggressive surgery may achieve a good outcome with a prolonged survival expectancy. BACKGROUND: Cholangiocarcinoma (CCA) is a lethal cancer of the biliary epithelium, originating from the liver (intrahepatic), at the confluence of the right and left hepatic ducts (hilar) or in the extrahepatic bile ducts. It is a rare malignancy associated with poor prognosis. DATA SOURCES: We searched the PubMed / MEDLINE database for relevant articles published from 1989 to 2008. The search terms used were related to “cholangiocarcinoma ” and its "treatment for the full-text review and final analysis, our resources only permitted the review of articles published in English. This review deals with the treatment of cholangiocarcinoma, the principles and the current trends .RESULTS: The risks and prognostic factors, symptoms and differential diagnosis are thoroughly discussed. In addition, the tools of preoperative diagnosis such as endoscopic retrograde cholangiopancreatography, digital image analysis, fluorescence in situ hybridization and magnet ic resonance cholangiopancreatography are reviewed. More over, the treatment of CCA is discussed. CONCLUSIONS: The only curative treatment available is surgical management. Unfortunately many patients present with unresectable tumors, the majority of whom die within a year of diagnosis. resections of the liver, pancreas and bile duct, with considerable mortality and morbidity. Despite, in selected cases and where indicated, appropriate management with aggressive surgery may achieve a good outcome with a prolonged survival expectancy.