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Background: Hepatocellular carcinoma (HCC) with bile duct tumor thrombi (BDTT) is a rare type of primary liver cancer, and its clinical and pathological characteristics remain to be defined.Aims: To investigate the clinical and pathological characteristics of HCC with BDTT.Methods: Among 676 HCC patients who underwent surgical treatment from Dec. 2002 to Dec. 2008 at the author's hospital, HCC with BDTT was identified in 20 patients. The clinical and pathological characteristics of the 20 patients were measured or analyzed retrospectively. The integrity of the involved bile duct was examined macroscopically and microscopically, the expression of liver stem cell markers was investigated by immunohistochemistry, and the Kaplan-Meier method was adopted for evaluating survival.Results: Among the 20 patients, the diameter of the primary tumor was less than 5 cm in 13 patients (range: 0.5-10 cm, mean 4.47±0.68 cm). Most of the primary tumors lacked an intact tumor capsule (15/20, 75%), had simultaneous blood vessel invasion (12/20, 60%), and were poorly differentiated (13/20, 65%). There was no evidence of bile duct wall infiltration by the tumor cells macroscopically or microscopically. The positive rate of the liver stem cell markers c-kit, CD90, CD133, and EpCAM was 90, 90, 85 and 85%, respectively. Postoperative overall survival rates at 1, 2, and 3 years were 73.1, 41.1, and 20.6%, respectively. The log-rank test showed that the overall survival rates were significantly worse for HCC patients with BDTT than for HCC patients without BDTT (P=0.016).Conclusions: HCC with BDTT has aggressive characteristics and the long-term prognosis is extremely dismal. [ABSTRACT FROM AUTHOR]