Cholangiocarcinoma

Abstract

Cholangiocarcinoma represents a diverse group of epithelial cancers united by late diagnosis and poor outcomes. Specific diagnostic and therapeutic approaches are undertaken for cholangiocarcinomas of different anatomical locations (intrahepatic, perihilar, and distal). Mixed hepatocellular cholangiocarcinomas have emerged as a distinct subtype of primary liver cancer. Clinicians need to be aware of intrahepatic cholangiocarcinomas arising in cirrhosis and properly assess liver masses in this setting for cholangiocarcinoma. Management of biliary obstruction is obligatory in perihilar cholangiocarcinoma, and advanced cytological tests such as fluorescence in-situ hybridisation for aneusomy are helpful in the diagnosis. Liver transplantation is a curative option for selected patients with perihilar but not with intrahepatic or distal cholangiocarcinoma. International efforts of clinicians and scientists are helping to identify the genetic drivers of cholangiocarcinoma progression, which will unveil early diagnostic markers and direct development of individualised therapies.

Figure 1. Integrative approach to (A) diagnosis and (B) individualised medicine in cholangiocarcinoma

FISH=fluorescence in-situ hybridisation. SDS-PAGE=sodium dodecyl sulfate polyacrylamide gel electrophoresis.

Figure 2. Potential cells of origin in intrahepatic cholangiocarcinoma

PV=portal vein. HA=hepatic artery. BD=bile duct. HC=hepatic cell.

Figure 3. Approach to management of (A) intrahepatic and (B) perihilar cholangiocarcinoma

HCC=hepatocellular carcinoma. CA 19-9=carbohydrate antigen 19-9. Reproduced with modifications from reference 69 by permission of Elsevier.

Figure 4. Imaging studies of a patient with perihilar cholangiocarcinoma of the left hepatic duct

Note prominent left hepatic duct dilatation with obstruction of the left hepatic duct system on the CT scan (A), MRI (B), endoscopic retrograde cholangiography (C), and magnetic resonance cholangiography (D); (D) also shows bilateral obstruction of the biliary system at the right and left hepatic bile duct confluence.