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Cholangiocarcinoma is a rare cancer that arises from bile ducts cells within the liver. Cholangiocarcinoma can also arise in the bile ducts that are outside of the liver proper. Cholangiocarcinomas are usually first
discovered when a patients describes vague abdominal pain, weight loss, fever, and jaundice (yellowing of the skin). While abdominal imaging with x-ray can be useful in the diagnosis of cholangiocarcinoma, direct imaging of the bile ducts is often necessary. Endoscopic retrograde cholangiopancreatography (ERCP), an endoscopic procedure, is many times the test of choice to make the diagnosis. Although ERCP is an invasive procedure, its advantages include the ability to obtain biopsies and to place stents or perform other interventions to relieve biliary obstruction. Another effective test is endoscopic ultrasound, which can also be performed at the time of ERCP and may increase the accuracy of the biopsy and yield information on lymph node invasion and operability. As an alternative to ERCP, percutaneous transhepatic cholangiography (PTC) may be utilized. Magnetic resonance cholangiopancreatography (MRCP) is a non-invasive alternative to ERCP. 

Carcinoembryonal antigen, also known as CEA-a blood tumor marker for cancer, may be elevated in the blood of patients with this tumor. The blood level of CEA may falsely be elevated in other non-cancerous conditions of the liver where the bile ducts are inflamed or obstructed. In some parts of the world, liver fluke (Fasciola hepatica) infection is a predisposing risk for the development of cholangiocarcinoma.

Patients with primary sclerosing cholangitis, as disease of the bile ducts commonly seen in patients with ulcerative colitis, are also at increased risk for developing cholangiocarcinoma.

Small cholangiocarcinomas may be surgically resected or the patient may undergo liver transplantation.
Patients with cholangiocarcinomas that cannot be surgically resected may be treated with chemotherapy or radiation therapy.