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Cholangiocarcinoma—controversies and challenges

Nature Reviews Gastroenterology & Hepatology volume 8, pages 189–200 (2011)Cite this article

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Abstract

Cholangiocarcinomas are a diverse group of tumors that are presumed to originate from the biliary tract epithelium either within the liver or the biliary tract. These cancers are often difficult to diagnose, their pathogenesis is poorly understood, and their dismal prognosis has resulted in a nihilistic approach to their management. The two major clinical phenotypes are intrahepatic, mass-forming tumors and large ductal tumors. Among the ductal cancers, lesions at the liver hilum are most prevalent. The risk factors, clinical presentation, natural history and management of these two types of cholangiocarcinoma are distinct. Efforts to improve outcomes for patients with these diseases are affected by several challenges to effective management. For example, designations based on anatomical characteristics have been inconsistently applied, which has confounded analysis of epidemiological trends and assessment of risk factors. The evaluation of therapeutic options, particularly systemic therapies, has been limited by a lack of appreciation of the different phenotypes. Controversies exist regarding the appropriate workup and choice of management approach. However, new and emerging tools for improved diagnosis, expanded indications for surgical approaches, an emerging role for locoregional and intrabiliary therapies and improved systemic therapies provide optimism and hope for improved outcomes in the future.

Key Points

  • Cholangiocarcinomas are a diverse group of tumors that occur within the liver and biliary tract and are thought to originate from the epithelial lining of the biliary tract

  • The two major clinical phenotypes of cholangiocarcinoma are intrahepatic and ductal tumors (further divided into lesions that arise at the liver hilum or the extrahepatic ductal system)

  • Macroscopic (mass-forming, sclerosing or polypoid) and microscopic (cholangiocellular or hepatocellular) are additional subclassifications that might correlate with biological behavior and therapeutic response

  • Inconsistent use of designations based on anatomical characteristics has confounded analysis of epidemiological trends and a lack of appreciation of the different phenotypes has limited the evaluation of systemic therapies

  • Appropriate evaluation and choice of management approach requires a multidisciplinary approach; a multimodality approach to the management of patients with these cancers should be considered

  • Emerging new tools for improved diagnosis, expanded indications for surgical approaches, an emerging role for locoregional and intrabiliary therapies and improved systemic therapies might improve outcomes in the future

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Figure 1: Classification of biliary tract cancers.
Figure 2: Approach to management of intrahepatic cholangiocarcinoma.
Figure 3: Approach to management of ductal cholangiocarcinoma.

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Acknowledgements

I apologize to the many excellent contributors to this field whose work is not acknowledged in the reference list owing to space limitations. This work was supported by NIH grant DK069370.C. P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape, LLC-accredited continuing medical education activity associated with this article.

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    Tushar Patel

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Patel, T. Cholangiocarcinoma—controversies and challenges. Nat Rev Gastroenterol Hepatol 8, 189–200 (2011). https://doi.org/10.1038/nrgastro.2011.20

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  • DOI: https://doi.org/10.1038/nrgastro.2011.20

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