Definition

Cholangiocarcinoma is the growth of cancer cells in the bile duct. The bile ducts are a number of branching tubes that carry bile from the liver to the gallbladder and small intestine. Bile is a fluid that helps with food digestion and the elimination of waste from the body.

Cholangiocarcinomas are named by their location.

• Perihilar—develops at the point where the branches of the bile duct join and leave the liver (most common form)
• Intrahepatic—develops in the bile duct branches that are located in the liver
• Distal—develops in the area of the bile duct that is closer to the small intestine

Common Bile Duct Copyright © Nucleus Medical Media, Inc.

Causes

Cancer occurs when cells in the body divide without control or order. Normally, cells divide in a regulated manner. If cells keep dividing uncontrollably when new cells are not needed, a mass of tissue forms, called a growth or tumor. The term cancer refers to malignant growths. These growths can invade nearby tissues. Cancer that has invaded nearby tissues can then spread to other parts of the body.

Defects on specific genes may lead to the growth of cancer cells in the bile duct. Other causes may be related to a combination of genetics and environment.

Risk Factors

Cholangiocarcinoma is more common in people 65 years and older. Other factors that may increase your chances of cholangiocarcinoma:

• Having a chronic condition that causes inflammation of the bile duct such as primary sclerosing cholangitis, ulcerative colitis, bile duct stones, choledochal cysts
• Excess weight or obesity
• Family history of bile duct cancer
• Conditions that affect the liver or bile ducts such as cirrhosis of the liver, polycystic liver disease, or Caroli syndrome
• Exposure to radioactive substances or toxic chemicals
• Infection by parasites called liver flukes (more common in Asian countries)

Other possible risk factors are:

• Smoking
• Diabetes
• Hepatitis B or C
• Pancreatitis

Symptoms

In the early stages of the cancer, there may not be any symptoms. As the cancer grows, symptoms may include:

• Yellowing of the skin and whites of the eyes— jaundice
• Abdominal pain
• Fever
• Weakness, fatigue
• Decrease in appetite, weight loss
• Itchy skin
• Dark urine, pale stool

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done. Blood tests will help to evaluate how the liver and gallbladder are working. These tests may also help to find tumor marker, indicators of cancer in the body.

Imaging tests can evaluate the bile duct and surrounding structures. These may include:

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

Other tests may may include:

• Laparoscopy —thin tube with a lighted camera on one end to examine the bile duct
• Biopsy —a sample of bile duct cells is removed and examined under a microscope

The physical exam combined with all of your test results, will help to determine the stage of cancer you have. Staging is used to guide your treatment plan. Like other cancers, cholangiocarcinoma is staged from I-IV (1-4). Stage I is a very localized cancer, while stage IV indicates a spread to other parts of the body.

Treatment

The treatment plan depends on the stage of the cancer and your overall health.

Prevention

To help reduce your chances of cholangiocarcinoma:

• Maintain a healthy weight
• Eat a healthful diet and exercise regularly
• Take precautions to prevent cirrhosis, like getting a hepatitis B vaccine, and avoid or limit alcohol
• Do not smoke
• Avoid exposure to toxins