Abstract
Case-profile investigation of intrahepatic cholangiocarcinoma associated hepatolithiasis was conducted by the Research Program of Intractable Disease provided by the Ministry of Health, Labor, and Welfare of Japan. From 1990 to 2009, 696 patients with hepatolithiasis including 37 concomitant cholangiocarcinomas were recruited from 26 institutions. Patients with prolonged histories of having stones were dominant in the 1990s, while recently, patients were frequently diagnosed with intrahepatic cholangiocarcinoma concurrent with hepatolithiasis. Patients with cholangiocarcinoma diagnosed with intrahepatic tumors represented the majority and had worse outcomes compared to patients initially diagnosed with stenosis or obstructing stones in the intrahepatic bile ducts. Most patients were histopathologically confirmed and evaluated using historical modalities such as ERC, PTC, or PTCS. Newer modalities such as MRI-DWI and FDG-PET, which became available in the 2000s, were hard to evaluate due to an inadequate number of cases. This work was supported partially by the Research Program of Intractable Disease provided by the Ministry of Health, Labor, and Welfare of Japan.
