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Hilar Cholangiocarcinoma

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Surgical Diseases of the Pancreas and Biliary Tree

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Abstract

Cholangiocarcinoma is a rare primary malignancy of the bile duct epithelium. Hilar cholangiocarcinoma accounts for 60–70% of all cholangiocarcinomas and originates near the confluence of the right and left hepatic ducts. Established risk factors include liver fluke infection, choledochal cysts and primary sclerosing cholangitis. Tumours may exhibit infiltrative, mass-forming or polypoid characteristics and are typically adenocarcinomas on histologic analysis. Lesions may be classified by anatomic, clinical or prognostic features. Patients are commonly asymptomatic until the development of biliary obstruction. Clinical evaluation includes laboratory analysis, cross-sectional imaging and a detailed assessment of hepatic anatomy and synthetic function. Preoperative preparation may require biliary decompression or portal vein embolization. Clinical management is largely determined by the extent of disease. Resection with negative margins is the first-line therapy when feasible. Neoadjuvant chemoradiation followed by liver transplantation may be considered for patients with locally unresectable disease or those with primary sclerosing cholangitis. However, a majority of patients receive definitive chemoradiation due to advanced disease at initial presentation. Overall 5-year survival approaches 30% but can surpass 60% in selected patients undergoing successful R0 resection. Unfortunately, despite aggressive surgical approaches, rates of recurrence and metastasis after surgery surpass 50% and 40%, respectively.

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Authors and Affiliations

  1. Johns Hopkins Hospital, Baltimore, MD, USA

    Bradley N. Reames

  2. The Urban Meyer III and Shelley Meyer Chair in Cancer Research, Department of Surgery, The Ohio State University, Wexner Medical Center, Columbus, OH, USA

    Timothy M. Pawlik

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  1. Gastrointestinal Surgery, Medanta – The Medicity, Gurgaon, Haryana, India

    Savio George Barreto

  2. Professor of Surgery, HBP/Upper GI Surgeon, Auckland City Hospital, University of Auckland, Auckland, New Zealand

    John A. Windsor

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Reames, B.N., Pawlik, T.M. (2018). Hilar Cholangiocarcinoma. In: Barreto, S., Windsor, J. (eds) Surgical Diseases of the Pancreas and Biliary Tree. Springer, Singapore. https://doi.org/10.1007/978-981-10-8755-4_14

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