Key Points for This Section

Extrahepatic bile duct cancer is a rare disease in which malignant (cancer) cells form in the part of bile duct that is outside the liver. Having colitis or certain liver diseases can increase the risk of developing extrahepatic bile duct cancer. Possible signs of extrahepatic bile duct cancer include jaundice and pain. Tests that examine the bile duct and liver are used to detect (find) and diagnose extrahepatic bile duct cancer. Certain factors affect prognosis (chance of recovery) and treatment options.

Extrahepatic bile duct cancer is a rare disease in which malignant (cancer) cells form in the part of bile duct that is outside the liver.

A network of bile ducts (tubes) connects the liver and the gallbladder to the small intestine. This network begins in the liver where many small ducts collect bile, a fluid made by the liver to break down fats during digestion. The small ducts come together to form the right and left hepatic bile ducts, which lead out of the liver. The two ducts join outside the liver to become the common hepatic duct. The part of the common hepatic duct that is outside the liver is called the extrahepatic bile duct. The extrahepatic bile duct is joined by a duct from the gallbladder (which stores bile) to form the common bile duct. Bile is released from the gallbladder through the common bile duct into the small intestine when food is being digested.

Having colitis or certain liver diseases can increase the risk of developing extrahepatic bile duct cancer.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors include having any of the following disorders:

• Primary sclerosing cholangitis.
• Chronic ulcerative colitis.
• Choledochal cysts.
• Infection with a Chinese liver fluke parasite.

Possible signs of extrahepatic bile duct cancer include jaundice and pain.

These and other symptoms may be caused by extrahepatic bile duct cancer or by other conditions. A doctor should be consulted if any of the following problems occur:

• Jaundice (yellowing of the skin or whites of the eyes).
• Pain in the abdomen.
• Fever.
• Itchy skin.

Tests that examine the bile duct and liver are used to detect (find) and diagnose extrahepatic bile duct cancer.

The following tests and procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.



• Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.



• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A spiral or helical CT scan makes detailed pictures of areas inside the body using an x-ray machine that scans the body in a spiral path.



• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).



• ERCP (endoscopic retrograde cholangiopancreatography): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes bile duct cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope is passed through the mouth, esophagus, and stomach into the first part of the small intestine. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs of cancer.



• PTC (percutaneous transhepatic cholangiography): A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. If a blockage is found, a thin, flexible tube called a stent is sometimes left in the liver to drain bile into the small intestine or a collection bag outside the body.



• Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. The sample may be taken using a thin needle inserted into the duct during an x-ray or ultrasound. This is called a fine-needle aspiration (FNA) biopsy. The biopsy is usually done during PTC or ERCP. Tissue may also be removed during surgery.



• Liver function tests: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver disease that may be caused by extrahepatic bile duct cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

• The stage of the cancer (whether it affects only the bile duct or has spread to other places in the body).
• Whether the tumor can be completely removed by surgery.
• Whether the tumor is in the upper or lower part of the duct.
• Whether the cancer has just been diagnosed or has recurred (come back).

Treatment options may also depend on the symptoms caused by the tumor. Extrahepatic bile duct cancer is usually found after it has spread and can rarely be removed completely by surgery. Palliative therapy may relieve symptoms and improve the patient's quality of life.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

A fluid made by the liver and stored in the gallbladder. Bile is excreted into the small intestine, where it helps digest fat.

A tube through which bile passes in and out of the liver.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A flexible tube used to deliver fluids into or withdraw fluids from the body.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

A disease or condition that persists or progresses over a long period of time.

A tube or vessel in the body that carries bile from the liver and gallbladder into the duodenum (the upper part of the small intestine).

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.

A sac or capsule in the body. It may be filled with fluid or other material.

The process of identifying a disease, such as cancer, from its signs and symptoms.

The process of breaking down food into substances the body can use for energy, tissue growth, and repair.

In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.

In medicine, to remove fluid as it collects; or, a tube or wick-like device used to remove fluid from a body cavity, wound, or infected area.

In medicine, a tube or vessel of the body through which fluids pass.

A thin, tube-like instrument used to look at tissues inside the body. An endoscope has a light and a lens for viewing and may have a tool to remove tissue.

A procedure that uses an endoscope to examine and x-ray the pancreatic duct, hepatic duct, common bile duct, duodenal papilla, and gallbladder. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. The endoscope is passed through the mouth and down into the first part of the small intestine (duodenum). A smaller tube (catheter) is then inserted through the endoscope into the bile and pancreatic ducts. A dye is injected through the catheter into the ducts, and an x-ray is taken. Also called endoscopic retrograde cholangiopancreatography.

The muscular tube through which food passes from the throat to the stomach.

The part of the common hepatic bile duct (tube that collects bile from the liver) that is outside the liver. This duct joins a duct from the gallbladder to form the common bile duct, which carries bile into the small intestine when food is being digested.

A rare cancer that forms in the part of the bile duct that is outside the liver. The bile duct is the tube that collects bile from the liver and joins a duct from the gallbladder to form the common bile duct, which carries bile into the small intestine when food is being digested.

An increase in body temperature above normal (98.6 degrees F), usually caused by disease.

The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.

Liquid.

The pear-shaped organ found below the liver. Bile is concentrated and stored in the gallbladder.

Refers to the liver.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

A condition in which the skin and the whites of the eyes become yellow, urine darkens, and the color of stool becomes lighter than normal. Jaundice occurs when the liver is not working properly or when a bile duct is blocked.

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

A blood test to measure the blood levels of certain substances released by the liver. A high or low level of certain substances can be a sign of liver disease.

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.

A part of the body that performs a specific function. For example, the heart is an organ.

Treatment given to relieve the symptoms and reduce the suffering caused by cancer and other life-threatening diseases. Palliative cancer therapies are given together with other cancer treatments, from the time of diagnosis, through treatment, survivorship, recurrent or advanced disease, and at the end of life.

Part of a system of ducts in the pancreas. Pancreatic juices containing enzymes are released into these ducts and flow into the small intestine.

An animal or plant that gets nutrients by living on or in an organism of another species. A complete parasite gets all of its nutrients from the host organism, but a semi-parasite gets only some of its nutrients from the host.

An exam of the body to check for general signs of disease.

The likely outcome or course of a disease; the chance of recovery or recurrence.

A procedure to x-ray the hepatic and common bile ducts. A contrasting agent is injected into the liver or bile duct, and the ducts are then x-rayed to find the point of obstruction. Also called percutaneous transhepatic cholangiography.

The overall enjoyment of life. Many clinical trials assess the effects of cancer and its treatment on the quality of life. These studies measure aspects of an individual’s sense of well-being and ability to carry out various activities.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.

Something that may increase the chance of developing a disease. Some examples of risk factors for cancer include age, a family history of certain cancers, use of tobacco products, certain eating habits, obesity, lack of exercise, exposure to radiation or other cancer-causing agents, and certain genetic changes.

The part of the digestive tract that is located between the stomach and the large intestine.

A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called ultrasonogram.

A detailed picture of areas inside the body. The pictures are created by a computer linked to an x-ray machine that scans the body in a spiral path. Also called helical computed tomography.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

A device placed in a body structure (such as a blood vessel or the gastrointestinal tract) to keep the structure open.

An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

Chronic inflammation of the colon that produces ulcers in its lining. This condition is marked by abdominal pain, cramps, and loose discharges of pus, blood, and mucus from the bowel.

A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.

A blood vessel that carries blood to the heart from tissues and organs in the body.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.