Cancer starts when cells in the body begin to grow out of control. Cells in nearly any part of the body can become cancer, and can spread to other areas of the body. To learn more about how cancers start and spread, see What Is Cancer?

Bile duct cancer starts in a bile duct. To understand this cancer, it helps to know about the normal bile ducts and what they do.

About the bile ducts

Survival rates are often used by doctors as a standard way of discussing a person’s prognosis (outlook). Some people may want to know the survival statistics for people in similar situations, while others might not find the numbers helpful, or might even not want to know them. If you decide that you don’t want to know them, stop reading here and skip to the next section.

When discussing cancer survival statistics, doctors often use a number called the 5-year survival rate. The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is diagnosed. Of course, some of these people live much longer than 5 years.

Five-year relative survival rates, such as the numbers below, assume that some people will die of other causes and compare the observed survival with that expected for people without the cancer. This is a better way to see the impact of the cancer on survival.

To get 5-year survival rates, doctors have to look at people who were treated at least 5 years ago. Improvements in treatment since then may result in a better outlook for people now being diagnosed with bile duct cancer.

There are some important points to note about the survival rates below:

These statistics come from the National Cancer Institute’s SEER program and are based on people diagnosed with bile duct cancer in the years 2000 to 2006. SEER does not separate these cancers by AJCC stage, but instead puts them into 3 groups: localized, regional, and distant. Localized is like AJCC stage I. Regional includes stages II and III. Distant means the same as stage IV.

SEER also does not separate perihilar bile duct cancers from distal bile duct cancers. Instead, these are grouped together as extrahepatic bile duct cancers.

Intrahepatic bile duct cancer

Extrahepatic bile duct cancer

Survival rates are often based on previous outcomes of large numbers of people who had the disease, but they can’t predict what will happen with any particular person. Many other factors can also affect a person’s outlook, such as their age and overall health, and how well the cancer responds to treatment. Even when taking these other factors into account, survival rates are at best rough estimates. Your doctor can tell you how the numbers above apply to you, as he or she knows your situation best.

Bile duct cancer is an uncommon cancer, which in some ways makes it harder to study than more common cancer types. But research into the causes, diagnosis, and treatment of bile duct cancer is currently being done in many medical centers throughout the world.

Surgery

Doctors are constantly improving the surgical techniques used to treat bile duct cancers and looking for ways to make more people eligible for surgery. One potential option, a liver transplant, was discussed in the section “ Surgery for bile duct cancer.”

Other options are also being explored. For example, sometimes surgery to remove the cancer might technically be possible, but it can’t be done because it might not leave enough healthy liver behind after the operation. One option might be to cut off the blood supply to the part of the liver that’s going to be removed (known as portal vein embolization). As this part of the liver shrinks, the other part of the liver grows to compensate. After several weeks, there might be enough healthy liver on this side to go ahead with the operation to remove the tumor.

Radiation therapy and chemotherapy

Researchers are looking at newer ways of increasing the effectiveness of radiation therapy. Some techniques, such as three-dimensional conformal radiation therapy (3D-CRT), intensity modulated radiation therapy (IMRT), and stereotactic body radiation therapy (SBRT), are widely available and allow doctors to better aim radiation to affect only the tumor and to spare nearby normal tissues. Other radiation techniques such as intra-operative radiation therapy (IORT) and proton beam radiation therapy may be helpful but are not widely available.

Other forms of radiation therapy are also being studied. For example, doctors are looking at whether radioactive stents placed inside bile ducts might help shrink tumors and keep the ducts open longer than standard stents. Another approach being studied is the injection of tiny radioactive beads into the hepatic artery (known as radioembolization). The beads lodge in the blood vessels near the tumor, where they give off small amounts of radiation.

Doctors are also testing different combinations of chemotherapy (chemo) drugs with radiation therapy, both on their own and before and after surgery.

In general, the effects of chemo against bile duct cancer have been found to be limited, but newer drugs and combinations of drugs are being tested. Newer ways to give chemo are also being studied. For example, trans-arterial chemoembolization (TACE) combines embolization (blocking off blood vessels supplying the tumor) with chemotherapy. Most often, this is done by using tiny beads that give off a chemo drug to plug up the hepatic artery.

Targeted therapy

Newer drugs are being developed that work differently from standard chemo drugs. These drugs target specific parts of cancer cells or their surrounding environments.

One target of several newer drugs is tumor blood vessels. Bile duct tumors need new blood vessels to grow beyond a certain size. Sorafenib (Nexavar^®), bevacizumab (Avastin^®), pazopanib (Votrient^®), and regorafenib (Stivarga^®) are examples of drugs that target blood vessel growth and are being studied against bile duct cancer.

Other new drugs have different targets. For example, EGFR, a protein that helps cells grow, is found in high amounts on some cancer cells. Drugs that target EGFR have shown some benefit against several types of cancer. Some of these drugs, such as cetuximab (Erbitux^®) and panitumumab (Vectibix^®) are now being studied for use in people with bile duct cancer, usually in combination with chemotherapy or other targeted drugs.

Drugs known as MEK inhibitors, such as trametinib (Mekinist^®), are also being studied for use against bile duct cancer.

The stage of a cancer describes how much cancer is in the body. It helps determine how serious the cancer is and how best to treat it. Doctors also use a cancer's stage when talking about survival statistics.

The stage is determined by the results of the physical exam, imaging and other tests (described in How Is Bile Duct Cancer Diagnosed?), and by the results of surgery if it has been done.

The American Joint Committee on Cancer (AJCC) TNM system

A staging system is a standard way for the cancer care team to sum up the extent of a cancer. The main system used to describe the stages of bile duct cancer is the American Joint Committee on Cancer (AJCC) TNM system. There are actually 3 different staging systems for bile duct cancers, depending on where they start:

• Intrahepatic bile duct cancers (those starting within the liver)
• Perihilar (hilar) bile duct cancers (those starting in the hilum, the area just outside the liver)
• Distal bile duct cancers (those starting farther down the bile duct system)

Bile duct cancer does not usually cause signs or symptoms until later in the course of the disease, but sometimes symptoms can appear sooner and lead to an early diagnosis. If the cancer is diagnosed at an early stage, treatment might be more effective.

When bile duct cancer does cause symptoms, it is usually because a bile duct is blocked.

Jaundice

Jaundice is yellowing of the skin and eyes. Normally, bile is made by the liver and released into the intestine. Jaundice occurs when the liver can’t get rid of bile, which contains a greenish-yellow chemical called bilirubin. As a result, bilirubin backs up into the bloodstream and settles in different parts of the body. This can often be seen in the skin and the white part of the eyes.

Jaundice is the most common symptom of bile duct cancer, but most cases of jaundice are not caused by cancer. Jaundice is more often caused by hepatitis (inflammation of the liver) or a gallstone that has traveled to the bile duct. But whenever jaundice occurs, a doctor should be seen right away.

Itching

Excess bilirubin in the skin can also cause itching. Most people with bile duct cancer notice itching.

Light-colored/greasy stools

Bilirubin contributes to the brown color of bowel movements, so if it doesn’t reach the intestines, the color of a person’s stool might be lighter.

If the cancer blocks the release of bile and pancreatic juices into the intestine, a person might not be able to digest fatty foods. The undigested fat can also cause stools to be unusually pale. They might also be bulky, greasy, and float in the toilet.

Dark urine

When bilirubin levels in the blood get high, it can also come out in the urine and turn it dark.

Abdominal (belly) pain

Early bile duct cancers usually do not cause pain, but more advanced cancers may cause abdominal pain, especially below the ribs on the right side.

Loss of appetite/weight loss

People with bile duct cancer may not feel hungry and may lose weight (without dieting).

Fever

Some people with bile duct cancer develop fevers.

Nausea and vomiting

These are not common symptoms of bile duct cancer, but they may occur in people who develop an infection (cholangitis) as a result of bile duct blockage. They are often seen along with a fever.

Bile duct cancer is not common, and these symptoms and signs are more likely to be caused by something other than bile duct cancer. For example, people with gallstones may have many of these same symptoms. There are many far more common causes of abdominal pain than bile duct cancer. And hepatitis (an inflamed liver most often caused by infection with a virus) is a much more common cause of jaundice. Still, if you have any of these problems, it’s important to see your doctor right away so the cause can be found and treated, if needed.

A risk factor is anything that affects your chance of getting a disease like cancer. Different cancers have different risk factors. Some risk factors, like smoking, can be changed. Others, like a person’s age or family history, can’t be changed.

But having a risk factor, or even several risk factors, does not mean that a person will get the disease. And many people who get the disease may have few or no known risk factors.

Researchers have found several risk factors that make a person more likely to develop bile duct cancer.

Certain diseases of the liver or bile ducts

People who have chronic (long-standing) inflammation of the bile ducts have an increased risk of developing bile duct cancer. Several conditions of the liver or bile ducts can cause this.

• Primary sclerosing cholangitis is a condition in which inflammation of the bile ducts (cholangitis) leads to the formation of scar tissue (sclerosis). People with this condition have an increased risk of bile duct cancer. The cause of the inflammation is not usually known. Many people with this disease also have inflammation of the large intestine called ulcerative colitis.
• Bile duct stones, which are similar to, but much smaller than gallstones, can also cause inflammation that increases the risk of bile duct cancer.
• Choledochal cysts are bile-filled sacs that are connected to the bile ducts. (Choledochal means having to do with the common bile duct.) The cells lining the sac often have areas of pre-cancerous changes, which increase a person’s risk for bile duct cancer.
• Liver fluke infections occur in some Asian countries when people eat raw or poorly cooked fish that are infected with these tiny parasite worms. In humans, these flukes live in the bile ducts and can cause bile duct cancer. There are several types of liver flukes. The ones most closely related to bile duct cancer risk are Clonorchis sinensis and Opisthorchis viverrini. Liver fluke infection is rare in the US, but it can affect people who travel to Asia.
• Abnormalities where the bile duct and pancreatic duct normally meet can allow digestive juices from the pancreas to reflux (flow back “upstream”) into the bile ducts. This backward flow also prevents the bile from being emptied through the bile ducts as quickly as normal. People with these abnormalities are at higher risk of bile duct cancer.
• Cirrhosis is damage to the liver from irritants such as alcohol and diseases such as hepatitis that cause scar tissue to form. Studies have found it raises the risk of bile duct cancer.
• Infection with hepatitis B virus or hepatitis C virus increases the risk of intrahepatic bile duct cancers. This may be at least in part due to the fact that long-term infections with these viruses can also lead to cirrhosis.

Other rare diseases of the liver and bile duct that may increase the risk of developing bile duct cancer include polycystic liver disease and Caroli syndrome (a dilation of the intrahepatic bile ducts that is present at birth).

Inflammatory bowel disease

Inflammatory bowel disease includes ulcerative colitis and Crohn’s disease. People with these diseases have an increased risk of bile duct cancer. This is not explained completely by the link between ulcerative colitis and primary sclerosing cholangitis.

Older age

Older people are more likely than younger people to get bile duct cancer. Most people diagnosed with bile duct cancer are in their 60s or 70s.

Ethnicity and geography

In the United States, the risk of bile duct cancer is highest among Hispanic Americans and Native Americans. Worldwide, bile duct cancer is much more common in Southeast Asia and China, largely because of the high rate of infection with liver flukes in these areas.

Obesity

Being overweight or obese can increase the risk of cancers of the gallbladder and bile ducts. This could be because obesity increases the risk of gallstones and bile duct stones. But there may be other ways that being overweight can lead to bile duct cancers, such as changes in certain hormones.

Exposure to Thorotrast

A radioactive substance called Thorotrast (thorium dioxide) was used as a contrast agent for x-rays until the 1950s. It was found to increase the risk for bile duct cancer, as well as some types of liver cancer, which is why it is no longer used.

Family history

A history of bile duct cancer in the family seems to increase a person’s chances of developing this cancer, but the risk is still low because this is a rare disease. Most bile duct cancers are not found in people with a family history of the disease.

Diabetes

When taken together, the data from many different studies show that people with diabetes have a higher risk of bile duct cancer. This increase in risk is not high, and the overall risk of bile duct cancer in someone with diabetes is still low.

Alcohol

People who drink alcohol are more likely to get intrahepatic bile duct cancer. The risk is higher in those who have liver problems from drinking alcohol.

Other possible risk factors

Studies have found several other factors that might increase the risk of bile duct cancer, but the links are not as clear. These include:

• Smoking
• Pancreatitis (inflammation of the pancreas)
• Infection with HIV (the virus that causes AIDS)
• Exposure to asbestos
• Exposure to radon or other radioactive chemicals
• Exposure to dioxin, nitrosamines, or polychlorinated biphenyls (PCBs)

We don’t know the exact cause of most bile duct cancers, but researchers have found several risk factors that make a person more likely to develop bile duct cancer (see the section “ What are the risk factors for bile duct cancer?”). There seems to be a link between this cancer and things that irritate and inflame the bile ducts, whether it’s bile duct stones, infestation with a parasite, or something else.

Scientists are starting to understand how inflammation might lead to certain changes in the DNA of cells, making them grow abnormally and form cancers. DNA is the chemical in each of our cells that makes up our genes – the instructions for how our cells function. We usually look like our parents because they are the source of our DNA. But DNA affects more than how we look.

Some genes control when cells grow, divide into new cells, and die. Genes that help cells grow, divide, and stay alive are called oncogenes. Genes that slow down cell division or cause cells to die at the right time are called tumor suppressor genes. Cancers can be caused by DNA changes (mutations) that turn on oncogenes or turn off tumor suppressor genes. Changes in several different genes are usually needed for a cell to become cancerous.

Some people inherit DNA mutations from their parents that greatly increase their risk for certain cancers. But inherited gene mutations are not thought to cause very many bile duct cancers.

Gene mutations related to bile duct cancers are usually acquired during life rather than being inherited. For example, acquired changes in the TP53 tumor suppressor gene are found in most bile duct cancers. Other genes that may play a role in bile duct cancers include KRAS, HER2, and MET. Some of the gene changes that lead to bile duct cancer might be caused by inflammation. But sometimes what causes these changes is not known. Many gene changes might just be random events that sometimes happen inside a cell, without having an outside cause.

If cancer keeps growing or comes back after one kind of treatment, it may be possible to try another treatment plan that might still cure the cancer, or at least keep it under control enough to help you live longer and feel better. Clinical trials also might offer chances to try newer treatments that could be helpful. But when a person has tried many different treatments and the cancer is still growing, even newer treatments might no longer be helpful. If this happens, it’s important to weigh the possible limited benefits of a new treatment against the possible downsides, including treatment side effects. Everyone has their own way of looking at this.

This is likely to be the hardest part of your battle with cancer – when you have been through many treatments and nothing’s working anymore. Your doctor might offer you new options, but at some point you may need to consider that treatment is not likely to improve your health or change your outcome or survival.

If you want to continue to get treatment for as long as you can, you need to think about the odds of treatment having any benefit and how this compares to the possible risks and side effects. Your doctor can estimate how likely it is the cancer will respond to treatment you’re considering. For instance, the doctor may say that more treatment might have about a 1 in 100 chance of working. Some people are still tempted to try this. But it is important to have realistic expectations if you do choose this plan.

Palliative care

No matter what you decide to do, you need to feel as good as you can. Make sure you are asking for and getting treatment for any symptoms you might have, such as nausea or pain. This type of treatment is called palliative care or supportive care.

Palliative care helps relieve symptoms, but is not expected to cure the disease. It can be given along with cancer treatment, or can even be cancer treatment. The difference is its purpose – the main goal of palliative care is to improve the quality of your life, or help you feel as good as you can for as long as you can. Sometimes this means using drugs to help with symptoms like pain or . Sometimes, though, the treatments used to control your symptoms are the same as those used to treat cancer. For instance, radiation might be used to help relieve pain caused by cancer that has spread. Or a stent might be placed in a bile duct to keep it from being blocked by the cancer. But this is not the same as treatment to try to cure the cancer. Some of the treatments that might be used are discussed nauseain the section “ Palliative therapy for bile duct cancer.”

Hospice care

At some point, you may benefit from hospice care. This is special care that treats the person rather than the disease; it focuses on quality rather than length of life. Most of the time, it is given at home. Your cancer may be causing problems that need to be managed, and hospice focuses on your comfort. You should know that while getting hospice care often means the end of treatments such as chemo and radiation, it doesn’t mean you can't have treatment for the problems caused by the cancer or other health conditions. In hospice the focus of your care is on living life as fully as possible and feeling as well as you can at this difficult time. 

Staying hopeful is important, too. Your hope for a cure may not be as bright, but there’s still hope for good times with family and friends – times that are filled with happiness and meaning. Pausing at this time in your cancer treatment gives you a chance to refocus on the most important things in your life. Now is the time to do some things you’ve always wanted to do and to stop doing the things you no longer want to do. Though the cancer may be beyond your control, there are still choices you can make.

To learn more

You can learn more about the changes that occur when treatment stops working, and about planning ahead for yourself and your family, in our documents Nearing the End of Life and Advance Directives.

Only a small number of bile duct cancers are found before they have spread too far to be removed by surgery.

The bile ducts are deep inside the body, so early tumors can’t be seen or felt during routine physical exams. There are no blood tests or other tests that can reliably detect bile duct cancers early enough to be useful as screening tests. (Screening is testing for cancer in people without any symptoms.) Because of this, most bile duct cancers are found only after the cancer has grown enough to cause signs or symptoms. The most common symptom is jaundice, a yellowing of the skin and eyes, which is caused by a blocked bile duct.

For some people with bile duct cancer, treatment can remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about cancer coming back. (When cancer comes back after treatment, it is called recurrence.) This is a very common concern in people who have had cancer.

It may take a while before your fears lessen. But it may help to know that many cancer survivors have learned to live with this uncertainty and are living full lives. The Understanding Recurrence section of the website has more about this.

For other people, the cancer may never go away completely. These people may get regular treatments with chemotherapy (chemo), radiation therapy, or other therapies to try to help keep the cancer under control and help relieve symptoms from it. Learning to live with cancer that does not go away can be difficult and very stressful. It has its own type of uncertainty.  Managing Cancer As a Chronic Illness talks more about this.

Follow-up care

If you have completed treatment, your doctors will still want to watch you closely. It’s very important to go to all of your follow-up appointments. During these visits, your doctors will ask questions about any problems you may have. They will examine you and may check lab tests or x-rays and scans to look for signs of cancer or treatment side effects.

Almost any cancer treatment can have side effects. Some may last for a few weeks to months, but others can last the rest of your life. Talk to your cancer care team about any changes or problems you notice and about any questions or concerns you have.

There is not set follow-up schedule for bile duct cancer that all doctors follow. Many doctors recommend blood and/or imaging tests about every 6 months for at least the first couple of years after treatment.

If cancer does recur, further treatment will depend on where the cancer is, what treatments you’ve had before, and your health. For more information on how recurrent cancer is treated, see the section “ Treatment of bile duct cancer based on the situation.” For more general information on dealing with a recurrence, you may also want to see our document When Your Cancer Comes Back: Cancer Recurrence.

It is also very important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen.

Seeing a new doctor

At some point after your treatment, you may be seeing a new doctor who doesn’t know anything about your medical history. It’s important to be able to give your new doctor the details of your diagnosis and treatment. Gathering these details soon after treatment may be easier than trying to get them at some point in the future. Make sure you have this information handy (and always keep copies for yourself):

• A copy of your pathology report(s) from any biopsies or surgeries
• If you had surgery, a copy of your operative report(s)
• Copies of x-rays and other imaging tests (these can often be stored digitally DVD, etc.)
• If you stayed in the hospital, a copy of the discharge summary that the doctor wrote when you were sent home
• If you had radiation therapy, a copy of the treatment summary
• If you had chemo or some other drug therapy, a list of your drugs, drug doses, and when you took them
• The names and contact information of the doctors who treated your cancer