Abstract: Objective To analyze the clinicopathological characteristics and the factors affecting the prognosis of intrahepatic
biliary cystadenocarcinoma (IBC). Methods Forty-six patients with histologically confirmed IBC from January, 2000 to April,
2014 were included. The clinical characteristics of the patients with IBC were compared with those of 58 patients with
intrahepatic biliary cystadenoma (IBCA). Kaplan-Meier analysis was used to identify the factors affecting the prognosis of IBC.
Results The 46 patients with IBC included 15 men and 31 women with a mean age of 57.0 ± 10.5 years. Compared with the
patients with IBCA, IBC patients had an older mean age (57.0±10.7 vs 44.3±15.3 years, P=0.03) and a greater proportion of male
patients (15/46 vs 8/58, P=0.02). The differential diagnosis between IBC and IBCA was difficult on the basis of preoperative
laboratory and imaging findings. The median overall survival of IBC patients was 56 months with 1-, 3-, and 5-year survival
rates of 85.9%, 65.2%, and 47.7%, respectively. Gender, surgical approach, tumor growth pattern and distant metastasis were all
significant prognostic factors for the overall survival of the patients. Conclusions IBC is a rare cystic lesion occurring primarily
in middle-aged men. Complete resection is recommended for curative treatment and close follow-up is essential especially for
male patients and patients with tumors exhibiting an invasive growth.