Biliary cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Abstract

Biliary tract cancer (BTC), comprising <1% of all human cancers and ∼10%–15% of all primary liver cancers, presents mostly in the seventh decade with a small male predominance (male:female ratio of 1.2–1.5:1.0) [1]. They are subclassified as intrahepatic cholangiocarcinoma (iCCA), originating from the biliary tree within the liver, and extrahepatic cholangiocarcinoma (eCCA), outside the liver parenchyma; the latter is further subdivided into perihilar cholangiocarcinoma (pCCA or Klatskin tumour) and distal cholangiocarcinoma (dCCA) [2], with a frequency of 10%–20% iCCA, 50% pCCA and 30%–40% eCCA.