Diffuse cholangiocarcinoma presenting with hepatic failure and extensive portal and mesenteric vein thrombosis

A 64-year-old previously healthy man presented with a 4-week history of vague right upper quadrant abdominal pain. Imaging studies revealed extensive portal, splenic, superior and inferior mesenteric vein thrombosis with mosaic perfusion and wedge-shaped areas of liver perfusion abnormalities. An extensive thrombophilia workup including tests for factor V Leiden, prothrombin G20210A, lupus anticoagulant, paroxysmal nocturnal haemoglobinuria, protein C and S, homocysteine and antinuclear antibody titres were all negative. Other laboratory testing revealed an elevated alkaline phosphatase (340 IU/L). Surgical exploration and catheter-directed thrombolysis were not felt to be feasible given the extensive clot burden. He was started on anticoagulation therapy. Over the next 10 days, he required intensive care unit admission due to progressive hepatic encephalopathy and fulminant liver failure. He continued to decline and eventually died of multiorgan failure. Autopsy revealed extensive, diffuse intrahepatic cholangiocarcinoma that had almost entirely replaced his normal liver parenchyma.