Abstract

INTRODUCTION: Intrahepatic cholangiocarcinoma (ICC), often an incidental finding is a rare tumor of the epithelial cells of the biliary ducts that originate in the liver and extrahepatic bile ducts. Serum alpha-fetoprotein (AFP), as a tumor marker of hepatocellular carcinoma (HCC), and carbohydrate antigen 19-9 (CA 19-9), as a tumor marker of ICC, have been widely used in clinical practice. Although, a subset of patient with ICC (20-25%) present with elevated AFP levels, usually >100 ng/mL and very rarely >1000 ng/mL, its role remains significant in prognosis of ICC. Herein, we discuss a case of Hepatitis B virus (HBV) associated ICC in a young individual with exceptionally high serum AFP levels. CASE DESCRIPTION/METHODS: A 45-year-old male with no apparent past medical history was seen for complaints of fatigue, nausea, anorexia, and associated 34-lb weight loss of 6-weeks duration. Initial labs revealed a chronic HBV infection and an obstructive pattern on liver function tests. Computerized tomography (CT) scan of the abdomen showed a large heterogeneous mass originating from porta hepatis and invading the left lobe of the liver with multiple liver metastases and metastatic lymphadenopathy of the porta hepatis. CT also revealed a mass in the gallbladder along with thrombosed portal vein and intrahepatic biliary dilation (Figures 1 and 2). Magnetic resonance cholangiopancreatography (MRCP) confirmed initial CT findings. Labs revealed an AFP level of 9,715 ng/mL and CA 19-9 level of 554 units/mL. Percutaneous transhepatic cholangiography (PTC) and tissue biopsy revealed liver parenchymal poorly differentiated adenocarcinoma. Attempted internalization of a biliary stent by interventional radiology (IR) was unsuccessful. Management options were discussed with the patient and family who opted for a palliative approach without chemotherapy. DISCUSSION: Intrahepatic cholangiocarcinoma (ICC) is a rare tumor of the epithelial cells of the biliary ducts originating in the liver and extrahepatic bile ducts. ICC tends to be aggressive due to lymph node involvement at diagnosis with intrahepatic, and peritoneal dissemination. Several factors have been implicated in the pathogenesis of ICC including hepatitis B and C viral infections. Role of AFP, often a marker of HCC, is a significant one in prognostication of ICC. Elevated AFP levels, especially with HBV-associated ICC carries a favorable prognosis. Unfortunately, in the case of our patient, due to the advanced nature of his condition, his prognosis remained poor.

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