Cholangiocarcinoma 2020: the next horizon in mechanisms and management

Cholangiocarcinoma 2020: the next horizon in mechanisms and management

Cholangiocarcinoma (CCA) constitutes a diverse group
of malignancies emerging in the biliary tree. CCAs are
divided into three subtypes depending on their anatomical
site of origin: intrahepatic (iCCA), perihilar (pCCA)
and distal (dCCA) CCA1,2 (Fig. 1). Of note, considered
as an independent entity, mixed HCC–CCA tumours
are a rare type of liver malignancy sharing features of
both iCCA and HCC and presenting an aggressive disease
course and poor prognosis3,4. iCCAs arise above the
second- order bile ducts, whereas the point of anatomical
distinction between pCCA and dCCA is the insertion
of the cystic duct. pCCA and dCCA can also be
collectively referred to as ‘extrahepatic’ (eCCA)5.

In the USA, pCCA is the single largest group, accounting for
approximately 50–60% of all CCAs, followed by dCCA
(20–30%) and iCCA (10–20%)1,6,7. CCA is the second
most common primary hepatic malignancy after hepatocellular
carcinoma (HCC), comprising approximately
15% of all primary liver tumours and 3% of gastrointestinal
cancers1,6,7. CCAs are usually asymptomatic in early
stages and, therefore, often diagnosed when the disease
is already in advanced stages, which highly compromises
therapeutic options, resulting in a dismal prognosis1,8.
CCA is a rare cancer, but its incidence (0.3–6 per 100,000
inhabitants per year)1 and mortality (1–6 per 100,000
inhabitants per year, globally9, not taking into account
specific regions with incidence >6 per 100,000 habitants
such as South Korea, China and Thailand) have been
increasing in the past few decades worldwide, representing
a global health problem.

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