Fact-sheet: Cholangiocarcinoma
Updated on 09/17/2021 at 12:16 PM
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Definition
Intrahepatic cholangiocarcinomas are adenocarcinomas of the bile ducts. -Klatskin tumor is located at the confluence of the right and left intrahepatic bile ducts: there is upstream dilation of the bile ducts -Intrahepatic bile duct tumors located upstream of the confluence are called "peripheral cholangiocarcinomas", and typically present as well-defined peripheral masses. There is no relationship with underlying cirrhosis or hepatitis B or C infection. Risk factors: parasitic infections (Clonorchis Sinensis), Caroli disease, sclerosing cholangitis.
Clinical features
Peripheral cholangiocarcinoma presents with general symptoms (abdominal pain, weight loss)
Hilar cholangiocarcinoma (Klatskin tumor) more typically presents with progressive jaundice.
Laboratory findings
Cholestasis
Abnormal liver function tests
Ultrasound
Peripheral cholangiocarcinoma: Nonspecific appearance, most often a hypoechoic, well-defined, lobulated, peripheral mass.
Hilar cholangiocarcinoma: rarely detected on ultrasound, dilation of the right and left intrahepatic bile ducts associated with a normal-caliber underlying common bile duct, without detectable biliary lithiasis
CT
Peripheral cholangiocarcinomas:
- hypodense mass whose periphery enhances after i.v. contrast injection starting at the arterial phase.
- during the venous phase, tendency toward concentric, centripetal enhancement sparing the central portion of the tumor.
- frequent capsular retraction overlying the tumor.
- +/- dilation of adjacent bile ducts
Klatskin tumor:
- the "infiltrative" type more typically presents as bile duct wall thickening, generally hypodense at the portal phase after injection and tending to become hyperdense relative to the liver parenchyma at the delayed phase.
- or a hilar polypoid mass syndrome with discordance between the dilation of the intrahepatic bile ducts and the normal downstream caliber of the common bile duct.
MRI
Peripheral cholangiocarcinoma -
sometimes heterogeneous signal with areas of fibrosis -
hypointense on T1 -
hyperintense on T2 -
after intravenous Gadolinium injection, during the arterial phase, the periphery of the tumor enhances. During the venous phase, there is a tendency toward concentric, centripetal enhancement sparing the central portion of the tumor. Delayed-phase enhancement of the fibrous component is suggestive
MRCP: noninvasive reference examination in the workup of biliary strictures
Classification
Bismuth-Corlette classification of Klatskin tumors: - Type 1: Tumor below the confluence without involving it - Type 2: Tumor reaching the roof of the confluence - Type 3: Involves the secondary confluence of the right or left duct - Type 4: Involvement of both secondary confluences of the right and left ducts