Definition

Cholangiocarcinoma is the growth of cancer cells in the bile duct. The bile duct are a number of branching tubes that carry bile from the liver to the gall bladder and small intestine. Bile is a fluid that helps digest food and eliminate waste from the body.

Cholangiocarcinomas can be divided by their location:

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

    common bile duct

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    Causes

    The exact cause is unknown. Gene defects may lead to the growth of cancer cells in the bile duct.

    Many different gene defects have been found in tumors. p53 gene abnormalities and K-ras gene abnormalities are two common gene defects that have been seen.

    Risk Factors

    Factors that may increase your risk of cholangiocarcinoma include:

        
  • Having a chronic condition that causes inflammation of the bile duct such as primary sclerosing cholangitis, ulcerative colitis , bile duct stones, choledochal cysts
  • Cirrhosis of the liver or another condition that affects the liver or bile duct such as polycystic liver disease, Caroli syndrome
  • Infection by parasites called liver flukes (more common in Asian countries)
  • Age older than 65 years
  • Overweight or obesity
  • Exposure to a radioactive substance or toxic chemicals
  • Family history of bile duct cancer
  • 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:

        
  • Jaundice (yellowing of the skin and whites of the eyes)
  • 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.

    Tests may be needed to create images of the liver and bile ducts such as:

        
  • Ultrasound
  • CT scan
  • MRI scan
  • PET scan
  • Other tests may be done to gather more information about the cancer and affected bile ducts such as:

        
  • Endoscopic retrograde cholangiopancreatography (ERCP)—x-rays and a long, flexible tube with a camera to examine the bile duct
  • Angiography —x-rays of the blood vessels
  • Laparoscopy —thin tube with a camera on one end to examine the bile duct
  • Biopsy —a sample of the bile duct cells is removed and examined
  • Treatment

    The treatment plan depends on the size and location of the tumor (stage) and your overall health.

    Surgery

    Surgery may be done to try to remove the cancer. The bile duct reaches into many organs and surgery may involve more than one organ. For example:

        
  • Intrahepatic bile duct cancer—may include the removal of part of the liver or an entire lobe
  • Perihilar bile duct cancer—may include the removal of part of the liver, bile duct, and gall bladder
  • Distal bile duct cancer—may include the removal of part of the pancreas and small intestine
  • If the cancer cannot be removed, other types of surgery may be done to relieve symptoms. A small tube may be placed inside of a cancerous bile duct to allow bile to flow through it.

    Radiation Therapy

    Radiation therapy is used to kill cancer cells and shrink tumors. Different forms include:

        
  • External radiation therapy—radiation directed at the tumor from a source outside the body
  • Internal radiation therapy—radioactive materials placed into the body in or near the cancer cells
  • Radiation may be done along with surgery. It may also be the main treatment if cancer cannot be removed.

    Chemotherapy

    Chemotherapy is the use of drugs to kill cancer cells. This treatment may be used before or after surgery. If the tumor cannot be removed, chemotherapy may be given, either alone or in combination with radiation therapy.

    Clinical Trials

    Clinical trials may be recommended if treatment options are limited for your type of cancer. These trials could offer treatments that are not currently available to most. Talk to your doctor about these options.

    Prevention

    You may be able to lower your risk by:

        
  • Maintaining a healthy weight
  • Eating a healthy diet and exercising regularly
  • Taking precautions to prevent cirrhosis, like getting a hepatitis B vaccine and avoiding or limiting alcohol
  • Not smoking
  • Avoiding exposure to toxins