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Last updated: August 19, 2022
Revisions: 23

Last updated: August 19, 2022
Revisions: 23

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Cholangiocarcinoma is a malignancy of the biliary system. It can occur at any site along the biliary tree, although predominantly arises in the extrahepatic biliary system.

The incidence is 1-2 per 100,000 population per year in Western societies, with over two-thirds of these cases in patients >65yrs. There is a higher incidence in South-East Asia due to the association with chronic endemic parasitic infections from liver flukes.

Histologically, 95% of bile duct cancers are adenocarcinomas, arising from cholangiocytes within the biliary tree. The remaining majority are squamous cell carcinomas, with other rarer types of bile duct cancers include sarcomas, lymphomas, and small cell cancers.

Klatskin Tumours

The most common site for bile duct cancers is at the bifurcation of the right and left hepatic ducts, termed Klatskin tumours.

They are typically slow-growing tumours that invade locally and metastases to local lymph nodes, before spreading distally to the peritoneal cavity, lung, and liver.

In this article, we shall look at the risk factors, clinical features and management of a patient with cholangiocarcinoma.

Risk Factors

The main risk factors for cholangiocarcinoma include:

  • Primary sclerosing cholangitis (lifetime risk of 10-20%)
  • Ulcerative colitis
  • Infective (Liver flukes, HIV, hepatitis virus)
  • Toxins (Chemicals in rubber and aircraft industry)
  • Congenital (Caroli’s disease, choledochal cyst)
  • Alcohol excess
  • Diabetes mellitus

Figure 1 – The biliary tree

Clinical Features

Cholangiocarcinoma (both intra- and extrahepatic) is generally asymptomatic until a late stage in the disease.

Patients will present typically with jaundice, along with potential pruritus, steatorrhoea, non-specific abdominal pain, or dark urine. In advanced disease, weight loss and lethargy can occur.

On examination, jaundice and cachexia are often evident. Courvoisier’s law can be applied in clinical assessment.

Courvoisier’s Law

Courvoisier’s law states that in the presence of jaundice and an enlarged or palpable gallbladder, malignancy of the biliary tree or pancreas should be strongly suspected as the cause is unlikely to be gallstones.


Laboratory Investigations

Biochemical investigations will confirm an obstructive jaundice (elevated bilirubin, ALP and ɣGT). The tumour markers CEA and CA19-9 may also be elevated.

Radiological Investigations

Ultrasound scanning may initially show dilated biliary ducts, however CT imaging or MRI imaging is required for diagnosis.

Endoscopic retrograde cholangiopancreatography (ERCP) is required to obtain a tissue sample, which will delineate the exact location of the lesion and take brushings of the area.

Staging of the disease is best done via CT imaging. Angiography may be used in pre-operative planning to image the hepatic arteries and portal vein.

Fig 2 - Imaging for Cholangiocarcinoma. (1) CT scan showing cholangiocarcinoma (circled) (2) ERCP showing common bile duct stricture and dilatation of the proximal duct

Figure 2 – Imaging for Cholangiocarcinoma. (1) CT scan showing cholangiocarcinoma (circled) (2) ERCP showing common bile duct stricture and dilatation of the proximal duct


The definitive cure for cholangiocarcinoma is complete surgical resection*. However, the majority of patients have inoperable disease at the time of presentation, with only 10-15% suitable for surgical intervention.

Intrahepatic or Klatskin tumours require a partial hepatectomy and reconstruction of the biliary tree. Patients with distal common duct tumours require a pancreaticoduodenectomy (either a Whipple’s procedure or a Pylorus preserving pancreaticduodenectomy (PPPD)).

Radiotherapy may be used in some cases as adjunct or neoadjunct therapy. There is limited evidence at present for the use of post-operative chemotherapy.

*However, unfortunately around half of patients undergoing resection for curative intent have a recurrence within 5 years


Most cases will end up only having palliative management. The palliative treatment options in cholangiocarcinoma include:

  • Stenting – ERCP may be used to stent the bile duct and relieve obstructive symptoms
  • Surgery – Surgical bypass procedures may be required if the obstruction cannot be relieved by stenting
  • Medical – Palliative radiotherapy or chemotherapy options can be discussed with the patient


In cholangiocarcinoma, there is an increased risk of biliary tract sepsis.

Long term survival is generally poor and treatment should be aimed at good symptom relieving management. The average survival is 12-18 months from diagnosis, as most patients have unresectable disease at the time of presentation.

In patients undergoing aggressive surgery, 5 year survival rates are 10-40%. Survival appears to be better for distal tumours.

Key Points

  • Cholangiocarcinoma is a relatively rare malignancy, 95% of which are adenocarcinomas
  • Patients will present often in late-stage disease, commonly with obstructive jaundice
  • The gold-standard investigation is via ERCP with brushings
  • Definitive cure for cholangiocarcinoma is complete surgical resection, yet most patients will only be suitable for palliative management