Intrahepatic Cholangiocarcinoma



Intrahepatic Cholangiocarcinoma


Matthew M. Yeh, MD, PhD









Intrahepatic cholangiocarcinomas generally arise in noncirrhotic livers. This gross photograph shows a white-tan, firm, and distinct mass in a background of noncirrhotic liver.






Intrahepatic cholangiocarcinomas form irregular glandular structures with an infiltrative appearance, surrounded by a prominent stroma. The nuclei are hyperchromatic and pleomorphic.


TERMINOLOGY


Definitions



  • Malignant adenocarcinoma arising from bile duct epithelium within liver


ETIOLOGY/PATHOGENESIS


Multistep Carcinogenesis



  • Chronic inflammation may be common pathogenic pathway


CLINICAL ISSUES


Epidemiology



  • Age



    • Average age at presentation is 60 years old


  • Gender



    • Equal frequency in men and women


  • Ethnicity



    • Very prevalent in Asia, particularly in northeastern Thailand (associated with liver fluke infestation)


Presentation



  • 10-20% of primary liver malignancies



    • Incidence and mortality rates have been increasing in several regions around world


    • Incidence has also increased 3x in past few decades in USA


  • Most patients are diagnosed with advanced stages of disease


  • Symptoms



    • Abdominal pain


    • Weight loss


    • Malaise


    • Jaundice


Laboratory Tests



  • Serum level of CA19-9 is currently most important tumor marker


  • Alkaline phosphatase and bilirubin variably elevated


Prognosis



  • Long-term survival is dismal


Risk Factors

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Intrahepatic Cholangiocarcinoma
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