Klatskin tumor

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Disease Overview

Klatskin tumors are are a type of cholangiocarcinoma that begins in an area called the hilum, where the left and right bile ducts join and leave the liver. It is the most common type of cholangiocarcinoma, accounting for more than half of all cases.[3898][3899][12368][12369] Symptoms usually don’t present until advanced stages of disease, when jaundice is the most common feature. Other symptoms include abdominal pain, unintentional weight loss, and a general feeling of being unwell (malaise).[12368][12369] 

The cause of Klatskin tumors is unknown.[3899] Studies suggest that a combination of genetic, environmental, and lifestyle factors (multifactorial) likely influence whether a person will develop cholangiocarcinoma. Because Klatskin tumors are often discovered after they have spread, they can be challenging to treat.[12368] Surgical removal of the tumor and relief of bile duct blockage are the main goals of treatment.[3898][3899][12369]


Synonyms

  • Klatskin's tumor
  • Perihilar cholangiocarcinoma

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National Organization for Rare Disorders