Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab


  • Justin L. Mott
  • Gregory J. Gores
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_1106-8



Cholangiocarcinoma refers to malignancy within the biliary duct system (Bile Duct Neoplasms) and is distinct from gallbladder cancer. Cholangiocarcinomas generally have features of biliary tract epithelium, such as a glandular appearance, small regular nuclei, and scant cytoplasm. Tumor cells often express cytokeratins, mucin, and cancer-associated antigen 19-9, a carbohydrate antigen that is used as a tumor marker in serum. It is thought to be a sialylated Lewis blood group antigen. CA19-9 levels are elevated in many gastrointestinal malignancies including cholangiocarcinoma and pancreatic cancer, as well as some nonmalignant conditions such as cholangitis and peritoneal inflammation/infection. Patients who have a genetic deficiency in a fucosyltransferase specified by the Le gene are Lewis a-b- and are unable to make this antigen; thus CA19-9 testing in Lewis a-b- patients can be falsely negative.
  • 95 % of bile duct...


Epidermal Growth Factor Receptor Primary Sclerosing Cholangitis Gallbladder Cancer Intrahepatic Cholangiocarcinoma Endoscopic Retrograde Cholangiography 
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Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Department of Biochemistry and Molecular BiologyUniversity of Nebraska Medical CenterOmahaUSA
  2. 2.Miles and Shirley Fiterman Center for Digestive Diseases, Division of Gastroenterology and HepatologyMayo Clinic College of MedicineRochesterUSA