Bile Duct Adenocarcinoma

Cholangiocarcinoma, Bile duct cancer

Bile duct adenocarcinoma, also called cholangiocarcinoma, is a rare and aggressive form of cancer that starts in the thin tubes carrying bile from your liver and gallbladder to your small intestine. Most people are diagnosed after the cancer has already spread, making it difficult to treat, but ongoing research continues to improve treatment options and outcomes.

Table of contents

• What Is Bile Duct Adenocarcinoma?
• The Bile Duct System
• Types of Bile Duct Cancer
• Signs and Symptoms
• Causes and Risk Factors
• How It Is Diagnosed
• Treatment Options
• Living With Advanced Disease
• Support and Coping

What Is Bile Duct Adenocarcinoma?

Bile duct adenocarcinoma is a type of cancer that forms in the slender tubes called bile ducts^[1]. These ducts connect your liver to your gallbladder and to your small intestine, carrying a digestive fluid called bile that helps break down fats during digestion^[2].

This cancer is also known as cholangiocarcinoma. The most common type of bile duct cancer starts in the mucous gland cells that line the bile duct and is classified as adenocarcinoma^[24]. It is a rare disease, with about 8,000 people in the United States developing it each year^[3].

Cholangiocarcinoma is an aggressive cancer, which means it spreads quickly. Most people receive a diagnosis after the cancer has already spread outside of their bile ducts, making it difficult to treat^[3]. The disease occurs mostly in people older than age 50, though it can occur at any age, and is most common in people around age 70^[1][3].

The Bile Duct System

To understand bile duct cancer, it helps to know how the bile duct system works. Bile is a fluid made by the liver to help digest fats. The bile ducts are a network of tubes that carry this fluid from the liver^[2].

Many small ducts inside the liver collect bile. These small ducts come together to form the right and left hepatic ducts, which lead out of the liver. The two ducts join outside the liver to form the common hepatic duct. The cystic duct connects the gallbladder to the common hepatic duct^[2].

When food is being digested, bile stored in the gallbladder is released and passes through the cystic duct to the common bile duct and into the small intestine^[2].

• Liver
• Gallbladder
• Small intestine
• Bile ducts
• Pancreas

Types of Bile Duct Cancer

Doctors divide cholangiocarcinoma into different types based on where the cancer occurs in the bile ducts. The three main types are intrahepatic, perihilar, and distal cholangiocarcinoma^[1][2].

Intrahepatic cholangiocarcinoma occurs in the parts of the bile ducts within the liver. This type forms in the bile ducts inside the liver and is sometimes classified as a type of liver cancer. Only a small number of bile duct cancers are intrahepatic, accounting for about 10% of cases^[1][7].

Perihilar cholangiocarcinoma, also called hilar cholangiocarcinoma or a Klatskin tumor, occurs in the bile ducts just outside of the liver. This type is found in the area where the right and left bile ducts exit the liver and join to form the common hepatic duct. It is the most common form of cholangiocarcinoma, accounting for approximately 50% of cases^[1][3][7].

Distal cholangiocarcinoma occurs in the portion of the bile duct nearest the small intestine. This type is found in the area where the ducts from the liver and gallbladder join to form the common bile duct. The common bile duct passes through the pancreas and ends in the small intestine. Distal cholangiocarcinoma accounts for about 40% of cases^[1][2][7].

Perihilar cholangiocarcinoma and distal cholangiocarcinoma are also known as extrahepatic bile duct cancers because they form outside the liver rather than inside it^[3].

Signs and Symptoms

Cholangiocarcinoma symptoms don’t usually start until the cancer advances and blocks a bile duct^[3]. This means that early-stage bile duct cancer often has no symptoms at all.

When symptoms do appear, they may include yellowing of the skin or whites of the eyes, a condition called jaundice. This happens when bile cannot flow normally and builds up in the body^[1][2]. Other symptoms related to blocked bile flow include dark urine and light-colored or clay-colored stools^[2][3].

Additional signs and symptoms of bile duct cancer include itchy skin, pain in the abdomen (especially on the right side underneath the ribs), fever, fatigue, nausea and vomiting, and unexplained weight loss^[2][3].

Cholangiocarcinoma isn’t usually painful in the early stages. But a large tumor can cause pain that may feel concentrated in the right side of your abdomen, underneath your ribs. For some people, the pain may shift to other regions in their abdomen or back^[3].

These symptoms can be caused by bile duct cancer or by other conditions. If you have any of these symptoms, it’s important to check with your doctor^[2].

Causes and Risk Factors

Experts don’t know exactly what causes cholangiocarcinoma. However, health conditions that cause chronic, or long-term, inflammation in your bile ducts may play a role^[3].

Ongoing damage from inflammation can cause changes in cell DNA. DNA contains the instructions that tell cells how to behave. Damaged DNA can cause problems with how cells grow and divide, creating tumors that damage tissue^[3].

Worldwide, cholangiocarcinoma is more common in Southeast Asia, where bile duct cancer can be a complication of clonorchiasis, a chronic infection associated with a Chinese liver fluke parasite^[3].

Many bile duct cancers are multifocal, meaning they start in more than one place in the bile ducts at the same time^[7].

How It Is Diagnosed

If your doctor suspects cholangiocarcinoma, you may undergo one or more tests to confirm the diagnosis. There are no routine screening tests to check for bile duct cancer before signs and symptoms occur^[2].

Blood tests can measure your liver function and give your doctor clues about what’s causing your signs and symptoms. A tumor marker test may check the level of carbohydrate antigen (CA) 19-9 in your blood. This is a protein that’s overproduced by bile duct cancer cells. However, a high level of CA 19-9 doesn’t necessarily mean you have bile duct cancer, as this result can also occur in other bile duct diseases^[9].

Imaging tests help doctors see the bile ducts and surrounding organs. These may include ultrasound, computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans. These tests can detect signs of disease and see whether cancer has spread to other areas of the body, such as the lymph nodes^[24].

Endoscopic tests use a thin, flexible tube with a camera to examine your bile ducts. During endoscopic retrograde cholangiopancreatography (ERCP), a tube equipped with a camera goes through the throat and into the small intestine. A dye enters the ducts through a small hollow tube passed through the endoscope, and tiny tools can be used to take tissue samples^[9].

A biopsy involves removing a small sample of tissue at the tumor site to analyze in a lab. Biopsy results help oncologists plan the best treatment. There are several different biopsy types, including image-guided biopsy and biopsy using ERCP^[24].

Other tests may include endoscopic ultrasound, where an ultrasound device at the end of an endoscope generates images of nearby tissues^[9].

Treatment Options

The treatment you receive depends on whether the cancer can be completely removed with surgery, the size and type of bile duct cancer, where it is located, whether it has spread, and your general health^[12][25].

Cholangiocarcinoma is often diagnosed when it’s advanced, making successful treatment difficult to achieve. In most patients, the tumor cannot be completely removed by surgery and is incurable^[1][7].

Surgery

If bile duct cancer is found early and has not spread, you may be able to have surgery to remove it. This is the main treatment for bile duct cancer that can be removed^[12][25].

Surgery to remove bile duct cancer may involve removing part of the bile duct if the tumor is small and confined to the bile duct only. In other cases, a partial hepatectomy may be performed to remove the part of the liver where cancer is found. For some patients, a Whipple procedure removes the head of the pancreas, the gallbladder, part of the stomach, part of the small intestine, and the bile duct^[10].

After the doctor removes all the cancer that can be seen at the time of surgery, some people may receive chemotherapy or radiation therapy to kill any cancer cells that are left. This is called adjuvant therapy^[10].

If the cancer has spread too far and cannot be removed, you may have surgery to help control symptoms. This is called palliative surgery. It can include procedures to unblock the bile duct, bypass a blockage, or place a stent (a thin, flexible tube) to drain bile^[10][12][25].

Chemotherapy

Chemotherapy uses medicines to kill cancer cells. You may have chemotherapy after surgery to get rid of any remaining cancer and help stop the cancer coming back. You may also receive it to help make the cancer smaller and control symptoms if you are not able to have surgery^[12][25].

Chemotherapy may be given alone or with other treatments such as radiation therapy, targeted medicines, or immunotherapy^[12][25].

Radiation Therapy

Radiation therapy uses high-energy rays of radiation to kill cancer cells. It is not often used to treat bile duct cancer, but you may have radiotherapy after surgery to help stop the cancer coming back, or to help control and improve the symptoms of advanced cancer^[12][25].

Radiation may be given as external radiation therapy from a machine outside the body. New ways to improve the effect of external radiation therapy on cancer cells are being studied, including hyperthermia therapy, where body tissue is exposed to high temperatures^[10].

Targeted Medicines and Immunotherapy

Targeted medicines kill cancer cells in specific ways. Immunotherapy uses medicines to help your immune system kill cancer. You may have immunotherapy if the cancer has spread to another part of your body^[12][25].

Biomarker testing tells doctors how to target their treatments. This testing examines gene changes in the cancer cells to determine which targeted medicines or immunotherapy might work best^[20].

You may have targeted medicines or immunotherapy either on their own or with chemotherapy^[12][25].

Living With Advanced Disease

Advanced bile duct cancer is cancer that has spread outside the bile ducts into nearby lymph nodes or organs, or to parts of the body further away from the bile ducts. It can also mean bile duct cancer that has come back some time after you were first treated^[18].

Unfortunately, advanced bile duct cancer is unlikely to be cured. However, your doctor might suggest treatment to try to slow its growth and relieve symptoms. If you have advanced bile duct cancer and it cannot be cured, the aim of your treatment will be to limit the cancer and its symptoms, and help you live longer^[12][18][25].

Finding out the cancer cannot be cured can be very hard news to take in. You will be referred to a special team of doctors and nurses called the palliative care team or symptom control team. They will work with you to help manage your symptoms and make you feel more comfortable^[12][25].

Your healthcare team can give you medicines to help control some of the symptoms of bile duct cancer. You may also have procedures such as stent placement to relieve blockages in the bile duct, which can help with jaundice and improve your quality of life^[17].

Support and Coping

You are likely to feel a range of powerful emotions if you are diagnosed with bile duct cancer. You might feel shocked, confused, angry, fearful, sad, or numb. These feelings are normal, and there is no right or wrong way to cope with a cancer diagnosis^[18][21].

Getting information about your type of cancer and its treatment can help you feel more able to cope and make decisions. However, taking in information can be difficult, especially when you have just been diagnosed. Make a list of questions before you see your doctor, and take someone with you to help you remember what was said^[18][21].

Talking to other people about how you are feeling can be helpful. Specialist nurses can provide support and information. They are usually your first point of call if you have any questions or concerns^[18][21].

You might find it helpful to talk to friends and family about the cancer, though some people may find this difficult. You may prefer to talk to a counselor or join a support group where you can connect with others affected by cancer^[18][21].

Managing side effects during treatment is important for your quality of life. You may experience fatigue, weight loss, nausea, and diarrhea. Exercise and medication can help manage these symptoms. Because a decreased appetite can cause weight loss, it may help to eat smaller meals and choose full-fat foods^[20].

Caring for your mental health during treatment is equally important. Thoughts and feelings can change over time. Relaxation techniques, self-care activities, and support groups can help you cope emotionally^[20].

Clinical trials continue to research and develop new treatments that can slow cancer spread and improve the outlook for people with cholangiocarcinoma^[3].