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Intrahepatic and Hilar Cholangiocarcinomas: Epidemiology, Basic Principles of Treatment, and Clinical Data

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Radiation Therapy for Liver Tumors

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Abstract

Cholangiocarcinoma, or cancer arising from bile duct epithelium, represents about 10–25% of primary liver cancers worldwide. Intrahepatic cholangiocarcinoma occurs in the bile ducts within the hepatic parenchyma, while extrahepatic tumors occur from the junction of the hepatic ducts to the common bile duct. Hilar cholangiocarcinoma is a subset of extrahepatic disease occurring at or near the junction of the left and right hepatic ducts. Chronic inflammation of the biliary tree from such processes such as liver fluke infection and primary sclerosing cholangitis confers a higher risk of developing cholangiocarcinoma. Diagnosis generally requires multiple modalities including laboratory tests, imaging studies, and often endoscopic procedures and biopsy. Cholangiocarcinoma is now staged according to subtype, with unique systems specific to intrahepatic, hilar, and extrahepatic diseases. As the only confirmed curative intervention for the treatment of cholangiocarcinoma, surgical intervention in the form of resection or liver transplant should be considered in all patients with localized disease. Locoregional therapies may provide some benefit to patients with localized disease who are not surgical candidates. Patients with metastatic cholangiocarcinoma may benefit from combination chemotherapy with gemcitabine and cisplatin, though prognosis remains poor. This chapter reviews the epidemiology, risk factors, and pathogenesis of intrahepatic and hilar cholangiocarcinomas, as well as the basic principles of diagnosis, staging, and treatment.

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  1. Division of Medical Oncology, Department of Medicine, University of Colorado Anschutz Medical Campus, Mail Stop 8117, 12801 East 17th Avenue, Room 8125, Aurora, CO, 80045, USA

    S. Lindsey Davis MD

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  1. Department of Radiation Oncology, University of Texas Southwestern Medical Center, Dallas, Texas, USA

    Jeffrey Meyer

  2. Department of Radiation Oncology, University of Colorado Comprehensive Cancer Center, Aurora, Colorado, USA

    Tracey Schefter

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Lindsey Davis, S. (2017). Intrahepatic and Hilar Cholangiocarcinomas: Epidemiology, Basic Principles of Treatment, and Clinical Data. In: Meyer, J., Schefter, T. (eds) Radiation Therapy for Liver Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-54531-8_15

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