63 years old female with loss of motivation and appetite

Case provided by Tanja

History
A 63 years old woman suffers from lack of motivation and appetite.

Imaging findings
Abdominal computed tomography shows an intrahepatic mass in the left lobe. The lesion has an irregular margin with capsular retraction. After contrast material administration there is hyperenhancement of the periphery in the arterial phase and gradually increasing central enhancement in the portal venous phase.

Diagnosis
Mass forming intrahepatic cholangiocarcinoma.

Discussion
Cholangiocarcinomas originate from biliary duct epithelium. They are classified into intrahepatic and extrahepatic forms. While intrahepatic forms are defined to be primary liver cancers, extrahepatic forms are divided into cancers of the gallbladder, cancers of extrahepatic ducts including perihilar forms and cancers of the Ampulla of Vater. Intrahepatic cholangiocarcinomas include peripheral mass forming cholangiocarcinoma as the most common type, intraductal intrahepatic cholangiocarcinoma, and periductal infiltrating intrahepatic cholangiocarcinoma.

Cholangiocarcinomas most commonly occur idiopathically. Howeber, cholangiocarcinoma is associated with chronic bile duct inflammation, e.g. primary sclerosing cholangitis, or parasitic diseases of bile ducts (clonorchiasis). Cholelithiasis was not shown to increase risk for cholangiocarcinoma and, therefore, women are not affected preferentially. Mainly elderly adults are affected as the maximum frequency of first clinical manifestation is between 50 and 70 years. Cholangiocarcinomas cause often biliary duct obstruction which leads to jaundice, pruritus, clay-colored stools and dark urine. Further typical symptoms are abdominal pain, weight loss and fever.

Contrast enhanced computed tomography of mass forming cholangiocarcinoma typically shows a heterogeneous irregular mass with peripheral enhancement that gradually increases from arterial to portal venous and delayed phases. An important differential diagnosis is hepatocellular carcinoma, which predominantly occurs in the cirrhotic liver and has arterial hyperenhancement and venous wash-out.

Teaching points

  • Cholangiocarcinomas can originate from every part of the intrahepatic, perihilar and extrahepatic biliary duct system.
  • The most common type is peripheral mass forming intrahepatic cholangiocarcinoma.
  • Imaging features suggesting mass forming intrahepatic cholangiocarcinomas are: irregular margin, capsular retraction, progressive enhancement during dynamic contrast enhanced imaging.

Recommended reading

Choi BI et al.
Imaging of intrahepatic and hilar cholangiocarcinoma.
Abdominal Imaging. 2004 Sept-Oct; 29(5):548-57

 

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