SARCholangiocarcinomaDFP @SARCholangioDFP

Cholangiocarcinoma (CCA) is classified as intrahepatic CCA (iCCA), perihilar CCA (pCCA), or distal CCA (dCCA). CCA can be mass-forming, periductal-infiltrating, or intraductal-growing.

Intrahepatic mass-forming cholangiocarcinoma shows high T2 signal, ragged thin rim enhancement in the arterial-phase, and gradual centripetal fill-in and poor central enhancement. This is the classic MRI appearance for an intrahepatic mass-forming cholangiocarcinoma.

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Perihilar cholangiocarcinoma is the most common form of cholangiocarcinoma (70%). It is also known as a Klatskin tumor, named after the American pathologist Gerald Klatskin (1910-1986).

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This is the typical post contrast features of cholangiocarcinoma after Eovist administration. Pre-contrast shows low signal. Arterial phase shows heterogenous enhancement with PVP delayed enhancement. Hepatobiliary phase show low retention of contrast with low signal throughout.

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CCA can be classified as intrahepatic, perihilar, and distal. Intrahepatic is located peripheral to the 2nd order bile ducts, peripheral is located between the 2nd order bile ducts and the insertion of the cystic duct. Distal is located distal to the insertion of the cystic duct.

The drawing illustrates the three types of cholangiocarcinoma. The three types being mass-forming, periductal infiltrating, and intraductal growing.

Gross pathology specimen shows the typical appearance of a firm, white mass representing cholangiocarcinoma with a few satellite nodules. The most common risk factor for cholangiocarcinoma in the U.S. is primary sclerosing cholangitis.

Gross pathology specimen of a cholangiocarcinoma directly invading the gallbladder. It has the typical white/tan firm appearance of cholangiocarcinoma.

Mass-forming intrahepatic cholangiocarcinoma. Arterial CT shows ragged rim enhancement. PVP CT shows gradual centripetal enhancement and a satellite nodule. Gross specimen shows a homogeneous, tan, firm mass. Microscope image (H-E) shows fibrous tissue with scattered tumor cells.

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Periductal infiltrating cholangiocarcinoma is a rare type of intraphepaticcholangiocarcinoma. It is characterized by growth along a dilated or narrowed bile duct without mass formation and shows up as diffuse periductal thickening and enhancement along a dilated or irregularly…

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Klatskin tumor (hilar cholangiocarcinoma occurring at the bifurcation of the common hepatic duct) causing biliary duct obstruction. Transhepatic cholangiogram shows a 2 cm long stricture causing dilation of the upstream bile ducts.

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Cholangiocarcinoma(Klatskin tumor). CT shows dilated intrahepatic bile ducts with abrupt tapering near the hilum of the liver where there is subtle soft tissue lesion. This is a common appearance for a Klatskin tumor which can be subtle and difficult to visualize on CT.

Mass-forming intrahepatic cholangiocarcinoma is considered to be a ductal type if it has at least one of the following three features (1) adjacent bile duct dilation, (2) periductal tumor spread, and (3) presence of chronic biliary disease such as intrahepatic stone disease.

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CT shows a large cholangiocarcinoma and a small right pleural effusion indicating there is pathology in the chest. CT through the lower chest shows a lung metastases and a heterogeneously enhancing slightly enlarged mediastinal lymph node representing mediastinal lymphadenopathy.

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Mass-forming intrahepatic cholangiocarcinoma. Arterial CT shows ragged rim enhancement. PVP CT shows gradual centripetal enhancement and a satellite nodule. Gross specimen shows a homogeneous, tan, firm mass. Microscope image (H-E) shows fibrous tissue with scattered tumor cells.

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Klatskin tumor (hilar cholangiocarcinoma occurring at the bifurcation of the common hepatic duct) causing biliary duct obstruction. CT shows significant dilation of the intrahepatic ducts.

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CT shows a cholangiocarcinoma. 20-minute hepatobiliary phase MR image with Eovist (gadoxeticacid) shows central high signal and peripheral low signal. This is the “gadoxetic acid cloud” appearance and is due to central fibrosis with large extracellular spaces retaining Eovist.

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Over half of mass-forming intrahepatic cholangiocarcinoma show a target appearance on DWI. Peripheral restricted diffusion is thought to represent highly cellular and vascular tumor cells. Central lack of restricted diffusion is thought to represent fibrosis and necrosis.

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Mass-forming intrahepatic cholangiocarcinoma. CT shows a liver mass with peripheral irregular enhancement and central hypoenhancement typical of a mass-forming intrahepatic cholangiocarcinoma. There are 3 satellite nodules which are also commonly seen in this entity.

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Perihilar cholangiocarcinoma (Klatskin tumor). MRCP image shows a stricture at the biliary duct confluence due to a cholangiocarcinoma. There is upstream intrahepatic duct dilation.

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