• Zachary J. Senders
  • John B. Ammori
  • Jeffrey M. HardacreEmail author


Cholangiocarcinoma is a cancer of the biliary tract with a poor prognosis for which surgical resection is the only potentially curative treatment. Disease confined to the liver is considered intrahepatic cholangiocarcinoma and may present as vague abdominal pain or could be found incidentally on imaging. Extrahepatic cholangiocarcinoma is generally divided into hilar and distal cancers and usually presents with biliary obstruction. Workup involves delineating the anatomical location of the tumor via cross-sectional imaging. Alternative diagnoses that may mimic cholangiocarcinoma such as hepatocellular carcinoma, pancreatic cancer, metastatic carcinoma, and benign biliary disease must be ruled out. Tissue diagnosis is often not necessary to proceed with surgical treatment if the tumor is resectable. Surgical treatment of cholangiocarcinoma is dictated by anatomic location of the tumor. Intrahepatic cholangiocarcinoma is treated with hepatic resection. Hilar cholangiocarcinoma, or Klatskin tumor, is treated with resection of the involved biliary tract and usually necessitates en bloc liver resection. Liver transplant may be considered in selected patients with these types of tumors. Distal cholangiocarcinoma is treated with pancreaticoduodenectomy. Phase II trials support a modest survival benefit from adjuvant therapy which should be considered.


Cholangiocarcinoma Biliary tract Hepatic resection Pancreaticoduodenectomy Intrahepatic Extrahepatic Klatskin tumor Hilar 


  1. 1.
    Benson AB III. NCCN guidelines: hepatobiliary cancers. NCCN Clin Pract Guidel Oncol. 2017;2:39–47.Google Scholar
  2. 2.
    DeOliveira ML, Cunningham SC, Cameron JL, Kamangar F, Winter JM, Lillemoe KD, et al. Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors. Ann Surg. 2007;245(5):755–62.CrossRefGoogle Scholar
  3. 3.
    Abu-Hamda EM, Baron TH. Endoscopic management of cholangiocarcinoma. Semin Liver Dis. 2004;24(2):165–75.CrossRefGoogle Scholar
  4. 4.
    Jarnagin WR, Fong Y, DeMatteo RP, Gonen M, Burke EC, Bodniewicz BSJ, et al. Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma. Ann Surg. 2001;234(4):507–17. discussion 517-9.CrossRefGoogle Scholar
  5. 5.
    Valle J, Wasan H, Palmer DH, Cunningham D, Anthoney A, Maraveyas A, et al. Cisplatin plus gemcitabine versus gemcitabine for biliary tract Cancer. N Engl J Med. 2010;362(14):1273–81.CrossRefGoogle Scholar
  6. 6.
    Rosen CB, Heimbach JK, Gores GJ. Liver transplantation for cholangiocarcinoma. Transpl Int. 2010;23(7):692–7.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Zachary J. Senders
    • 1
  • John B. Ammori
    • 1
  • Jeffrey M. Hardacre
    • 1
    Email author
  1. 1.Department of SurgeryUniversity Hospitals Cleveland Medical CenterClevelandUSA

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