Intrahepatic Cholangiocarcinoma Complicated by Non-Cirrhotic Portal Hypertension and Variceal Bleeding: A Case Report

Abstract

Intrahepatic cholangiocarcinoma (ICC) is the second most common primary liver malignancy after hepatocellular carcinoma and is often diagnosed at an advanced stage because early symptoms are non-specific. Portal hypertension is an uncommon complication of ICC and is usually related to malignant involvement of the portal venous system rather than cirrhotic remodelling of the liver parenchyma. We report the case of a 50-year-old woman with type 2 diabetes mellitus who presented with a four-month history of progressive cholestatic jaundice, dark urine, pale stools, generalised pruritus, intermittent epigastric pain, ascites, and subsequent massive upper gastrointestinal bleeding with haematemesis and melaena. Clinical and endoscopic findings were consistent with portal hypertension. Laboratory investigations showed severe normocytic anaemia, marked cholestasis, normal aminotransferase values, preserved coagulation parameters, negative hepatitis B and C serology, elevated carbohydrate antigen 19-9, and normal alpha-fetoprotein. Abdominal ultrasonography, computed tomography, and magnetic resonance imaging demonstrated a large intrahepatic mass measuring 93 x 71 x 85 mm, with intrahepatic biliary dilatation, ascites, hilar lymphadenopathy, homogeneous splenomegaly, and encasement of both right and left portal vein branches, without radiological features of cirrhosis. Upper gastrointestinal endoscopy revealed grade 3 oesophageal varices with red wale signs and portal hypertensive gastropathy; endoscopic variceal ligation was successfully performed. Percutaneous ultrasound-guided biopsy confirmed adenocarcinoma with glandular structures, consistent with ICC, while the non-tumorous liver parenchyma was unremarkable. This case illustrates that ICC may rarely present with non-cirrhotic portal hypertension and variceal bleeding caused by malignant portal venous obstruction, and that malignancy should be considered when portal hypertension occurs without cirrhosis.