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Cholangiocarcinoma

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Overview

Cholangiocarcinoma is a type of bile duct cancer arising in any part of the biliary tree. More than 90% of cases are ductal adenocarcinomas and the rest are squamous cell tumours.

It is often asymptomatic in its early stages most people develop symptoms in advanced disease.

Epidemiology

  • Cholangiocarcinoma has an incidence of 1-2 per 100,000 per year in the UK
  • Most cases are in people >60 years old
  • It is more common in Southeast Asia due to an increased incidence of liver flukes (parasitic infections of the liver and bile ducts) 

Risk Factors

Causes of chronic biliary duct inflammation are risk factors:

Presentation

Overview

Most patients are asymptomatic in the early stages and develop symptoms of advanced disease. Features include:

  • Painless jaundice – the most common feature
    • Around 35% of patients may have abdominal pain similar to the symptoms of biliary colic, but persistent
  • Associated features of obstructive jaundice:
    • Pruritus
    • Pale stools
    • Dark urine
  • Constitutional symptoms: unexplained weight loss, anorexia, malaise
  • Courvoisier’s law:
    • A painless palpable mass in the right upper quadrant with jaundice is unlikely to be due to gallstones and is more likely due to pancreatic cancer or cholangiocarcinoma
  • Regional lymph node metastases:
    • Often periumbilical lymphadenopathy (Sister Mary Joseph nodes) and left supraclavicular lymphadenopathy (Virchow’s node)

Investigations

Overview

Investigations may include:

  • Liver function tests (LFTs):
    • May show an obstructive pattern: elevated bilirubin, gamma-GT, and ALP
    • If hepatic invasion occurs, transaminases may be elevated
  • CA 19-9:
    • Often elevated, but has poor sensitivity and also may be elevated in pancreatic cancer
    • Often used to screen for cholangiocarcinoma in people with PSC
  • Abdominal ultrasound:
    • May identify a suspicious mass
  • CT thorax, abdomen, and pelvis:
    • Identifies tumour and metastases
  • Magnetic resonance cholangiopancreatography (MRCP):
    • Assesses the biliary tree in more detail
  • Endoscopic retrograde cholangiopancreatography (ERCP):
    • Allows the collection of a biopsy for histological confirmation
    • May be used to place stents to relieve obstruction

Management

Overview

The management of cholangiocarcinoma is decided by a multidisciplinary team meeting. Surgery is possible at early stages, but less than 1/3 of people are suitable for surgery at diagnosis. Radiotherapy and chemotherapy may also be used.

In most cases, curative treatment is not possible. Palliative treatment can involve stenting to relieve obstruction and palliative chemotherapy/radiotherapy.

Prognosis

  • Average survival is around 12-18 months from diagnosis as many people present with unresectable or metastatic disease
  • Even with aggressive surgery, radiotherapy, and chemotherapy, 5-year survival rates are around 10-40%

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