Cholangiocarcinoma is a cancer of the bile ducts which drain bile from the liver into the small intestine. Other biliary tract cancers include pancreatic cancer, gall bladder cancer, and cancer of the ampulla of Vater. Cholangiocarcinoma is a relatively rare adenocarcinoma (glandular cancer), with an annual incidence of 1–2 cases per 100,000 in the Western world, but rates of cholangiocarcinoma have been rising worldwide over the past several decades.
Although there are at least three staging systems for cholangiocarcinoma (e.g. Bismuth, Blumgart, American Joint Committee on Cancer) none have been shown to be useful in predicting survival. The most important staging issue is whether the tumor can be surgically removed, or whether it is too advanced or invasive for surgical treatment. Often, this determination can only be made at the time of surgery.
General guidelines for operability include:
Absence of lymph node or liver metastases
Absence of involvement of the portal vein
Absence of direct invasion of adjacent organs
Absence of widespread metastatic disease
Imaging of the biliary tree
CT scan showing cholangiocarcinoma
Bismuth classification for perihilar cholangiocarcinoma. Shaded areas represent tumor location:
ERCP image of cholangiocarcinoma, showing common bile duct stricture and dilation of the proximal common bile duct.
While abdominal imaging can be useful in the diagnosis of cholangiocarcinoma, direct imaging of the bile ducts is often necessary. Endoscopic retrograde cholangiopancreatography (ERCP), an endoscopic procedure performed by a gastroenterologist or specially trained surgeon, has been widely used for this purpose. Although ERCP is an invasive procedure with attendant risks, its advantages include the ability to obtain biopsies and to place stents or perform other interventions to relieve biliary obstruction. Endoscopic ultrasound can also be performed at the time of ERCP and may increase the accuracy of the biopsy and yield information on lymph node invasion and operability. As an alternative to ERCP, percutaneous transhepatic cholangiography (PTC) may be utilized. Magnetic resonance cholangiopancreatography (MRCP) is a non-invasive alternative to ERCP. Some authors have suggested that MRCP should supplant ERCP in the diagnosis of biliary cancers, as it may more accurately define the tumor and avoids the risks of ERCP.
The Bismuth-Corlette classification:
Limited to the common bile duct. More than 2 cm from the confluence of right and left hepatic ducts.
Less than 2cm from the confluence. Involves the confluence.
type II + involvement of the right hepatic duct.
type II + involvement of the left hepatic duct.
Extending to both right and left hepatic ducts or multifocal involvement.
Stricture at the junction of common bile duct and cystic duct.
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