Cholangiocarcinoma is the most common primary tumor of the biliary system,
arising from the bile ducts epithelium.
It has been divided into intrahepatic,
perihilar and distant types,
with perihilar or Klatskin tumors being most common - 40-60% of all cases /1/.
The main histopathologic type is adenocarcinoma (95% of cases) /2/.
Its peak prevalence is during the 7th decade of life,
male patients with a slight predilection /2/.
Main risk factors include primary sclerosing cholangitis (PSC),
hepatolithiasis,
choledocal cyst,
bile stasis.
Some genetic factors are also discussed /3/.
The main clinical symptoms include obstructive jaundice and different non-specific symptoms – pruritus,
abdominal pain,
weight loss,
fever and chills in additional inflammation.
Classification:
According to the Japanese Liver Cancer Group and based on the pattern of tumor growth they can be classified as 1.
Mass forming (exophytic) 2.Periductal (infiltrating) 3.Intraductal (polypoid) and 4.Mixed (mass-forming and periductal).
The infiltrating type is the most common type of Klatskin tumors /4/.
Bismuth-Corlette classification describes different types according to the extent of ductal involvement:
Type I: Tumors below the bifurcation of common hepatic duct
Type II: Tumors involving the bifurcation; but not extending into the main right and left ducts
Type III: Tumors infiltrating the right (III a) or the left (III b) hepatic duct
Type IV: Tumors involving both the right and left hepatic ducts
Treatment options:
Surgery is the only curative therapy if a complete tumor excision is possible.
It is necessary to resect the whole tumor with free margins and still leave enouph liver for adequate liver function /5/.
It depends on the tumor site and size,
the degree of duct infiltration,
involvement of surrounding vessels,
lymph nodes,
organs.
In patients with advanced disease only adequate palliation may be possible,
including stent placement,
biliary bypass surgery,
etc.
Still there is lack of an effective adjuvant treatment /6/.