Magnetic Resonance Imaging (MRI) of the abdomen during the same hospitalization showed focal dilatation of upper common bile duct distal to confluence of hepatic ducts,

focal dilatation of bile ducts draining the right hepatic lobe, a right hepatic lobe central duct filling defect without adjacent hepatic parenchymal abnormalities, Inhibitors,research,lifescience,medical all suggesting a primary intrahepatic cholangiocarcinoma. The patient underwent an endoscopic retrograde cholangiopancreatography (ERCP) with sweepings of the right hepatic duct irregularity collecting soft tissue material using a Roth net with microscopic examination revealing papillary adenocarcinoma, consistent with primary cholangiocarcinoma (Figure 1). Figure 1 Fragments of the tumor shows malignant ductal epithelium Inhibitors,research,lifescience,medical with papillary and glandular architecture diagnostic of cholangiocarcinoma With evidence of invasion into the right branches of the portal vein the patient was deemed

unresectable after consultation with both local and University Hospital hepatobiliary and transplant surgeons. Treatment was initiated in February 2008 with a combination of gemcitabine and oxaliplatin (GEMOX). After an initial period of disease stability, an MRI after his 12th cycle of GEMOX in September 2008 showed disease progression. He remained on gemcitabine but with progressive disease, the oxaliplatin was changed Capecitabine. Inhibitors,research,lifescience,medical A MRI after only 4 cycles in December 2008 showed continued local progression. In January 2009 his chemotherapy was again changed to 5-FU and Leucovorin. A follow up MRI after only 2 months, in March 2009, again revealed progressive disease with an ill defined enhancing hypovascular mass in segment VII on the liver, associated with segmental biliary dilatation, representing intrahepatic Inhibitors,research,lifescience,medical extension of the cholangiocarcinoma. After progressing on three of the most commonly used chemotherapy regimens,

the patient was started on Sorafenib in May 2009 based on initial phase II trials and case reports suggesting a possible Oligomycin A in vitro benefit of the drug in cholangiocarcinoma (5). The patient had jaundice with a peak total bilirubin of 4.1 mg/dL Inhibitors,research,lifescience,medical prior to initiating treatment with Sorafenib. Soon after starting sorafenib his jaundice resolved and his bilirubin has been within normal limits since Jan 2010 with his latest value being 0.7 mg/dL in December 2012. Since below initiating Sorafenib, imaging of the liver has been performed every 3-4 months with a MRI and continually revealed stable disease. The most recent imaging was a PET/CT in October 2012 which continues to show a stable ill-defined space occupying mass in the liver with no focus of hypermetabolic activity within the mass or anywhere else in the body. Overall, the patient has tolerated treatment very well. He was started on the standard dosing of 400 mg twice a day. He experienced mild diarrhea that has been well controlled with the use of lomotil.