Intrahepatic cholangiocarcinoma associated with hepatolithiasis.

ABSTRACT

BACKGROUND/AIMS: Despite sporadic reports of cholangiocarcinoma (CC) associated with hepatolithiasis, this entity has not been widely studied. The purpose of this study was to clarify its clinical features and optimal management by studying the 10 patients we have encountered with this condition. METHODOLOGY: There were six women and four men, with a mean age of 61 years. The patients underwent anatomic hepatic resection (n = 5) or biliary drainage (n = 5). The clinical features and results of surgery were studied. RESULTS: The characteristic findings included tumor-related symptoms, irregular ductal stricture or obstruction, and hepatic lobar atrophy with a whitish mass. The tumor and stones were located in the same hepatic lobe. Eight patients had advanced CC with periductal tumor infiltration, while two had in situ carcinoma characterized by intraductal tumor growth, papillary adenocarcinoma, and mucin-hypersecretion. Seven patients died within 6 months after surgery, while the remaining three, including the two with in situ carcinomas and one with an involved node at the dissected hilum, are alive more than 4 years after anatomic hepatic resection. CONCLUSIONS: Recognition of the clinical features of CC associated with hepatolithiasis, which were clarified in this study, is important in treating patients with hepatolithiasis. An anatomic hepatic resection with hilar nodal dissection offers long-term survival in selected patients.