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AIM: To evaluate the long-term outcome and prognostic factors of patients with hilar cholangiocarinoma.METHODS: Ninety-six consecutive patients underwent treatment for malignant hilar bile duct tumors during 1995-2005. Of the 96 patients, 20 were initially treated with surgery (n = 2 R0 / n = 18 R1). In non-operated patients, data analysis was performed retrospectively.RESULTS: Among the 96 patients, 76 were treated with endoscopic transpapillary (ERC, n = 45) and/or percutaneous transhepatic biliary drainage (PTBD, n= 31). The mean survival time of these 76 patients undergoing palliative endoscopic and/or percutaneous drainage was 359 + 296 d. The mean survival time of patients with initial bilirubin levels ＞ 10 mg/dL was significantly lower (P ＜ 0.001) than patients with bilirubin levels ＜ 10 mg/dL. The mean survival time of patients with Bismuth stage Ⅱ (n = 8), Ⅲ (n = 28) and Ⅳ (n =40) was 496 + 300 d, 441 + 385 d and 274 ± 218 d,respectively. Thus, patients with advanced Bismuth stage showed a reduced mean survival time, but the difference was not significant. The type of biliary drainage had no significant beneficial effect on the mean survival time (ERC vs PTBD, P = 0.806).CONCLUSION: Initial bilirubin level is a significant prognostic factor for survival of patients. In contrast,age, tumor stage according to the Bismuth-Corlette classification, and types of intervention are not significant prognostic parameters for survival. Palliative treatment with endoscopic or percutaneous biliary drainage is still suboptimal, new diagnostic and therapeutic tools need to be evaluated.