Cholangiocarcinoma or bile duct cancer is a type of liver cancer that affects the bile ducts inside and outside the liver. These tumors account for one-third of all liver and biliary passage tumors.
Conditions or diseases associated with bile duct tumors include:
- Gallstones – Which may cause persistent irritation of the ducts.
- Liver fluke (parasite) infections.
- Primary sclerosing cholangitis – A disease that damages and blocks bile ducts inside and outside the liver.
- Ulcerative colitis.
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What Is Bile Duct Cancer?
Bile duct cancer is cancer in the tubes that carry bile from the liver to the gallbladder. Bile is fluid your liver makes and uses to digest fats in the food you eat.
Several different tubes form your liver's bile duct network. This network includes small ducts that join to form the left and right hepatic ducts inside the liver.
These ducts then merge and become the common hepatic duct outside the liver.
The common hepatic duct ends at the cystic duct, where the two ducts merge to form the common bile duct.
Bile duct cancer can occur in any of these tubes.
What are the types of bile duct cancer?
Bile duct cancer can begin in any of the bile ducts inside or outside the liver. These cancers have distinct names, based on the cancer's location.
Extrahepatic cholangiocarcinoma starts in the bile ducts outside the liver, including the common hepatic duct and the cystic duct.
There are two types of extrahepatic bile duct cancers:
- Perihilar cholangiocarcinoma, or Klatskin tumor. This cancer forms in the hilum, the place where bile ducts merge when leaving the liver. This is the most common type of bile duct cancer.
- Distal cholangiocarcinoma. This cancer forms in bile ducts outside the liver.
- Intrahepatic cholangiocarcinoma is cancer that grows in the bile ducts inside the liver. This type of bile duct cancer is less common than extrahepatic cancers.
How common is bile duct cancer?
Bile duct cancer isn't very common. Doctors only diagnose about 8,000 people in the U.S. each year.
What causes bile duct cancer?
Cancer occurs when cells grow in ways that aren't normal. Mutations or changes in certain genes cause this abnormal growth.
Some people with bile duct cancer form mutations in the genes that control how cells divide, causing tumors to grow. Others have oncogenes, or gene changes, that can cause cells to become cancerous.
Researchers don't think parents pass these gene changes on to their children, but they're still studying the genetic component.
Bile duct cancer risk factors
You may be at higher risk of bile duct cancer if you have:
- A history of working with certain manufacturing chemicals.
- Bile duct cysts that cause swelling or infection.
- Chronic ulcerative colitis, or ulcers (sores) in the lining of the colon or rectum.
- Cirrhosis of the liver.
- Hepatitis B or hepatitis C.
- Primary sclerosing cholangitis or scarred or inflamed bile ducts.
Complications of bile duct cancer
Left undiagnosed or untreated, bile duct cancer can cause liver problems, such as cirrhosis and liver failure. Sometimes, cancer can metastasize (spread) to other parts of the body.
How can I reduce my risks of Bile duct cancer?
You can help lower your risk of bile duct cancer and other types of cancer by losing weight and maintaining a healthy weight. According to the American Cancer Society, people who eat a healthy diet, maintain a healthy weight, and stay physically active are at lower risk of cancers.
You can also reduce your risk of bile duct cancer by:
- Avoiding chemical exposures.
- Getting vaccinated against hepatitis B, which can prevent HBV infection-related cirrhosis.
- Limiting alcoholic beverages to no more than one per day for women and two per day for me.
- Preventing infections, including bloodborne or sexually transmitted infections (STIs) like HBV, hepatitis C (HCV) and other viruses that attack the liver.
- Quitting smoking – or better yet, never starting.
- Taking precautions with food and water when traveling abroad to avoid parasites.
- Treating hepatitis B or C infections to prevent cirrhosis.
What are the Symptoms of Bile Duct Cancer?
Sometimes, people with bile duct cancer don't have any symptoms when it first begins, known as early-stage cancer.
If cancer spreads or tumors block the bile ducts, the most common symptom is jaundice – a yellow color of the skin and eyes.
Other symptoms can include:
- Dark urine.
- Fatigue.
- Fever.
- Itchy skin.
- Light, or clay-colored, stools.
- Nausea.
- Stomach pain.
- Unexplained weight loss.
When should I see a doctor about my bile duct cancer symptoms?
Because bile duct cancer is so deep within the body, it often is not diagnosed until symptoms begin. If you have symptoms of jaundice – yellowing of the whites of the eyes and the skin – call your primary care provider right away.
How Do You Diagnose Bile Duct Cancer?
Doctors will start with a physical exam to diagnose bile duct cancer.
They also use imaging tests to look for liver damage or signs that your liver isn't working as it should.
These tests include:
Magnetic resonance cholangiopancreatography (MRCP) is a specialized imaging technique. It takes detailed pictures of the inside of the liver and bile ducts. Radio waves and magnets create pictures that doctors can see on a computer.
Doctors may also confirm bile duct cancer with a biopsy, or tissue sample.
Testing for cholangiocarcinoma
In addition to a physical exam, your doctor may order several tests to evaluate:
- The extent and location of the tumor.
- The tumor's proximity to major hepatic (liver) blood vessels.
- Whether the tumor is spreading.
- The quality of liver function.
These tests may include:
Biopsy
People who have abdominal pain might have an exam of the gastrointestinal tract, called endoscopic retrograde cholangiopancreatography (ERCP).
This test helps the doctor detect a narrowing of the duct, which could indicate the presence of a tumor.
If ERCP confirms the existence of a tumor, the doctor may use a brush to take a tissue sample during the procedure. The sample will undergo a biopsy to test for cancer.
Blood tests
Liver function tests measure the amount of bilirubin in the blood. Bilirubin is the product responsible for the yellow color of bile and urine. Knowing someone’s bilirubin levels can help doctors determine whether a tumor is blocking the bile duct.
If tests indicate increased bilirubin and duct obstruction, specialists may place a stent, or tube, in the duct to relieve the blockage. As a result of this procedure, bilirubin levels should return to normal.
Doctors also look for alkaline phosphatase, a liver enzyme. High levels of this substance might be a sign of bile duct cancer.
Doctors also use tumor marker tests to look for substances the body makes when cancer is growing.
Imaging studies
A CT scan may show a dilated bile duct, indicating the presence of a tumor.
Other tests
Other tests may include:
- Angiogram — determines blood vessel involvement and blood flow to the tumor.
- Cholangiography— uses x-rays and a large needle inserted into the liver to examine the gallbladder and bile ducts.
- MRI cholangiogram — inspects the duct that connects the liver to the small intestine.
How Do You Treat Bile Duct Cancer?
UPMC offers several treatment options for people with cholangiocarcinoma.
Liver surgery (resection)
Depending on the size and location of your tumor(s), and the general health of your liver, you may be a candidate for surgical removal.
Surgery, either minimally invasive (laparoscopic) liver surgery or traditional (open) liver surgery, offers the best chance for cure or long-term survival.
UPMC’s liver surgeons are among the most experienced in the United States at minimally invasive liver surgery, which is performed through three or four small incisions in the abdomen. We perform this technique whenever possible to reduce pain, scarring, and recovery time.
Therapies for bile duct cancer
If your tumor is too large to be removed by surgery, UPMC offers innovative therapies to:
- Help shrink the tumor, so surgery is possible.
- Manage your liver disease and extend your life.
Doctors will look at your type of bile duct cancer and your overall health when designing your treatment.
Lifestyle changes to treat bile duct cancer
Lifestyle changes can't cure your cancer, but they can help keep your liver healthy.
These changes include:
- Avoiding heavy alcohol use.
- Exercising.
- Not smoking.
- Staying at a healthy weight.
Medical treatments for bile duct cancer
Doctors have a few options for treating bile duct cancer, including:
- Chemotherapy – Drugs that kill cancer cells by stopping growth during the cell's lifecycle. Chemo affects both healthy cells and cancer cells.
- Immunotherapy – Drugs that help the immune system fight cancer.
- Radioembolization - Radioactive beads deliver radiation to tumors to shrink them. Doctors insert these beads into a thin tube, or catheter, in an artery in your thigh. Then they feed the tube into the hepatic artery to prevent blood flow to the tumor. Sometimes, they add chemo.
- Targeted therapy – Drugs that attack specific types of cancer cells, while leaving healthy cells alone.
Surgery to treat bile duct cancer
Doctors use different surgeries to treat bile duct cancer, such as:
- Bile duct removal – To remove the bile duct if the cancer hasn't spread.
- Partial hepatectomy – To remove the tumor and some of the surrounding tissue, leaving healthy tissue intact.
- Liver transplant – To remove the diseased liver and replace it with a healthy liver from a living donor.
