Budd Chiari Syndrome and Intrahepatic Cholangiocarcinoma, An Unusual Combination: Case Report and Review of the Literature

Abstract

Intrahepatic cholangiocarcinoma arising in the setting of Budd Chiari syndrome is uncommon and its prognostic and management implications differ from hepatocellular carcinoma. We report a case of intrahepatic cholangiocarcinoma in a patient with primary Budd Chiari syndrome. Hepatocellular carcinoma is known to occur with Budd Chiari syndrome. It was difficult to differentiate from hepatocellular carcinoma in the presence of increased alfa-fetoprotein levels. The contrast imaging showed features of progressive enhancement in the arterial, portal, and venous phases. A targeted liver biopsy showed histological features typical for cholangiocarcinoma. Immunostaining for cytokeratin 7 and cytokeratin 20 were positive, whereas that for arginase was negative, suggesting an intrahepatic cholangiocarcinoma. The patient was planned for inferior vena cava angioplasty followed by resection for intrahepatic cholangiocarcinoma. Previously, only secondary Budd Chiari syndrome developing in the background of primary liver tumor has been described; no report exists of intrahepatic cholangiocarcinoma arising in background of primary Budd Chiari syndrome.