Cholangiocarcinoma

Definition

Cholangiocarcinoma is the growth of cancer cells in the bile duct. The bile ducts are tubes that carry bile from the liver to the gallbladder and small intestine. Bile is a digestive fluid.

Bile duct cancers are named by their location.

• Perihilar—where the bile ducts join and leave the liver (most common form)
• Intrahepatic—in the bile duct branches in the liver
• Distal— in the bile duct closest to the small intestine

Common Bile Duct

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Causes

Cancer happens when cells divide without control or order. These cells grow together to form a tumor. They can invade and damage nearby tissues. They can also spread to other parts of the body.

Bile duct cancer may be caused by defects in certain genes. Other causes are likely due to genes and environment.

Risk Factors

Bile duct cancer is more common in people 65 years and older. Other things that may raise the risk are:

• Long term inflammation of the bile duct due to:
  • Bile duct stones
  • Primary sclerosing cholangitis
  • Ulcerative colitis or Crohn disease
  • Choledochal cysts
• Exposure to radioactive substances or toxic chemicals
• Infection by liver fluke parasites (more common in Asian countries)
• Hepatitis B or C
• Excess weight or obesity

Other possible risks may be due to:

• Cirrhosis of the liver and fatty liver disease
• Alcohol use and smoking
• Diabetes
• Infection from bacteria

Symptoms

In the early stages there may be no symptoms. As the cancer grows, symptoms may be:

• Yellowing of the skin and whites of the eyes— jaundice
• Belly pain, nausea, and vomiting
• Fever
• Feeling tired and weak
• Lack of hunger and weight loss
• Itchy skin
• Dark urine or pale stool

Diagnosis

The doctor will ask about your symptoms and health history. A physical exam will be done.

Blood tests will help to check how well the liver and gallbladder are working. These tests may also help to find tumor marker, signs of cancer in the body.

Imaging tests can check the bile duct and nearby structures. These may include:

• Ultrasound
• CT scan
• MRI scan
• PET scan
• Endoscopic retrograde cholangiopancreatography (ERCP)
• Angiography

Other tests may include:

• Laparoscopy —thin tube to examine the bile duct
• Biopsy —a sample of bile duct cells is removed and tested

The exam and test results will be used for staging. This will outline how far and fast the cancer has spread.

Treatment

The goal is to remove the cancer, if possible. Treatment depends on the stage and site of the cancer. It also depends on the person's health. A combination of treatments may be used. Options may include:

• Surgery, to try to remove the cancer, which may include:
  • For perihilar bile duct cancer—part of the liver, bile duct, and gall bladder may be removed
  • For intrahepatic bile duct cancer—part of the liver or an entire lobe may be removed
  • For distal bile duct cancer—part of the pancreas and small intestine may be removed
• Surgery to ease symptoms, such as—a tube or stent to open a blocked bile duct
• Chemotherapy by mouth, injection, or IV—to kill cancer cells
• External or internal radiation—to kill cancer cells and shrink tumors

Prevention

The risk of bile duct cancer may be reduced by treating certain conditions that raise the risk.

Revision Information

• Reviewer: EBSCO Medical Review Board Mohei Abouzied, MD, FACP
• Review Date: 01/2021
• Update Date: 03/19/2021