Intrahepatic cholangiocarcinomas are the second most common primary liver malignancies with an increasing incidence over the past decades. Due to a lack of early symptoms and their aggressive oncobiological behavior, the diagnostic approach is challenging and the outcome remains unsatisfactory with a poor prognosis. Thus, a consistent staging system for a comparison between different therapeutic approaches is needed, but independent predictors for worse survival are still controversial. Currently, four different staging systems are primarily used, which differ in the way they determine the ‚T‘ category. Furthermore, different nomograms and prognostic models have been recently proposed and may be helpful in providing additional information for predicting the prognosis and therefore be helpful in approaching an adequate treatment strategy. This review will discuss the diagnostic approach to intrahepatic cholangiocarcinoma as well as compare and contrast the most current staging systems and prognostic models.
Key words: Intrahepatic cholangiocarcinoma – staging systems – nomograms – liver neoplasms – biliary tract cancer
Abbreviations: AFP: Alpha-fetoprotein; ALP: Alkaline phosphatase; ALD: Alcoholic liver disease; CA: Carbohydrate-antigen; CEA: Carcinoembryonic antigen; EGD: Esophagogastroduodenoscopy; ERCP: Endoscopic retrograde cholangiopancreatography; EUS-FNA: Endoscopic ultrasound-guided fine-needle aspiration; HBV: Hepatitis B virus; HCC: Hepatocellular carcinoma; HCV: Hepatitis C virus; ICC: Intrahepatic cholangiocarcinoma; INR: International normalized ratio; LCSGJ: Liver Cancer Study Group of Japan; LNM: Lymph node metastases; MRCP: magnetic resonance cholangiopancreatography; NAFLD: Non-alcoholic fatty liver disease; PA: Pre-albumin; PSC: Primary sclerosing cholangitis.