A review of the clinical diagnosis and therapy of cholangiocarcinoma

J Int Med Res. 2014 Feb;42(1):3-16. doi: 10.1177/0300060513505488. Epub 2013 Dec 23.


Cholangiocarcinoma (CCA) is the second most common primary hepatic malignancy worldwide. The incidence of intrahepatic CCA is increasing, whereas that of extrahepatic CCA is decreasing. This review looks at the new advances that have been made in the management of CCA, based on a PubMed and Science Citation Index search of results from randomized controlled trials, reviews, and cohort, prospective and retrospective studies. Aggressive interventional approaches and new histopathological techniques have been developed to make a histological diagnosis in patients with high risk factors or suspected CCA. Resectability of the tumour can now be assessed using multiple radiological imaging studies; the main prognostic factor after surgery is a histologically negative resection margin. Biliary drainage and/or portal vein embolization may be performed before extended radical resection, or liver transplantation may be undertaken in combination with neoadjuvant chemotherapy or chemoradiotherapy. Though many advances have been made in the management of CCA, the standard modality of treatment has not yet been established. This review focuses on the clinical options for different stages of CCA.

Keywords: Cholangiocarcinoma; diagnosis; malignant biliary obstruction; photodynamic therapy; review; therapy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Bile Duct Neoplasms / diagnosis*
  • Bile Duct Neoplasms / therapy*
  • Bile Ducts, Intrahepatic / pathology*
  • Cholangiocarcinoma / diagnosis*
  • Cholangiocarcinoma / pathology
  • Cholangiocarcinoma / therapy*
  • Combined Modality Therapy
  • Humans
  • Liver Transplantation
  • Neoplasm Staging
  • Photochemotherapy
  • Prognosis
  • Radiotherapy


  • Antineoplastic Agents