Cholangiocarcinoma is a type of cancer that originates in the bile ducts. The majority are adenocarcinomas. It may affect the bile ducts inside the liver (intrahepatic ducts) or outside the liver (extrahepatic ducts). The most common site is in the perihilar region, where the right and left hepatic duct have joined to become the common hepatic duct, just after leaving the liver.

Key risk factors include:

  • Primary sclerosing cholangitis
  • Liver flukes (a parasitic infection)


TOM TIP: Patients with ulcerative colitis are at risk of developing primary sclerosing cholangitis. Patients that have primary sclerosing cholangitis are at risk of developing cholangiocarcinoma (10-20%). Primary sclerosing cholangitis is the key risk factor worth remembering for your exams. The other notable cause is parasitic infection with liver flukes, which are found in various parts of Southeast Asia and Europe. 



Obstructive jaundice is the key presenting feature to remember. Obstructive jaundice is also associated with:

  • Pale stools
  • Dark urine
  • Generalised itching


Other non-specific signs and symptoms include:

  • Unexplained weight loss
  • Right upper quadrant pain
  • Palpable gallbladder (swelling due to an obstruction in the duct distal to the gallbladder)
  • Hepatomegaly


Courvoisier’s law states that a palpable gallbladder along with jaundice is unlikely to be gallstones. The cause is usually cholangiocarcinoma or pancreatic cancer. 

TOM TIP: Painless jaundice should make you think of cholangiocarcinoma or cancer of the head of the pancreas. Pancreatic cancer is more common, so this is likely the answer in your exams.



Diagnosis is based on imaging (CT or MRI) plus histology from a biopsy. 

Staging CT scan involves a full CT thorax, abdomen and pelvis (CT TAP). This is used to look for metastasis and other cancers.

CA 19-9 (carbohydrate antigen) is a tumour marker that may be raised in cholangiocarcinoma. It is also raised in pancreatic cancer and a number of other malignant and non-malignant conditions.

Magnetic resonance cholangio-pancreatography (MRCP) may be used to assess the biliary system in detail to assess the obstruction. 

Endoscopic retrograde cholangio-pancreatography (ERCP) can be used to put a stent in and relieve the obstruction, and also obtain a biopsy from the tumour.



Management will be decided at a multidisciplinary team (MDT) meeting. 

Curative surgery may be possible in early cases. It may be combined with radiotherapy and chemotherapy.

In most cases, curative surgery is not possible. Palliative treatment may involve:

  • Stents inserted to relieve the biliary obstruction
  • Surgery to improve symptoms (e.g., bypassing the biliary obstruction)
  • Palliative chemotherapy
  • Palliative radiotherapy
  • End of life care with symptom control


Last updated May 2021
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