Bile Duct Cancer
Bile duct cancer is a rare disease in which cancer cells form in the ducts that are outside the liver (also called the extrahepatic bile duct).
The extrahepatic bile duct is made up of two parts:
- Common hepatic duct, which is also called the perihilar part of the extrahepatic duct.
- Common bile duct, which is also called the distal part of the extrahepatic duct.
The extrahepatic bile duct is part of a network of ducts (tubes) that connect the liver, gallbladder, and 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 ducts, which lead out of the liver. The two ducts join outside the liver and form the common hepatic duct. Bile from the liver passes through the hepatic ducts, common hepatic duct and cystic duct and is stored in the gallbladder.
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.
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. Talk with your doctor if you think you may be at risk. Risk factors include having any of the following disorders:
- Primary sclerosing cholangitis.
- Chronic ulcerative colitis.
- Choledochal cysts.
- Infection with a Chinese liver fluke parasite.
These and other signs and symptoms may be caused by bile duct cancer or by other conditions. Check with your doctor if you have any of the following:
- Jaundice (yellowing of the skin or whites of the eyes)
- Pain in the abdomen
- Itchy skin
Tests that examine the bile duct and liver are used to detect (find) and diagnose 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).
- PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
- 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, including part of a lymph node, 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.
- Tumor marker test: A procedure in which a sample of blood, urine, or tissue is checked to measure the amounts of certain substances made by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the body. These are called tumor markers. Carcinoembryonic antigen (CEA) and CA 19-9 are associated with extrahepatic bile duct cancer when found in increased levels in the body.
After extrahepatic bile duct cancer has been diagnosed, tests are done to find out if cancer cells have spread within the bile duct or to other parts of the body.
The process used to find out if cancer has spread within the bile duct or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.
Bile duct cancer may be staged following a laparotomy. A surgical incision is made in the wall of the abdomen to check the inside of the abdomen for signs of disease and to remove tissue and fluid for examination under a microscope. The results of the diagnostic imaging tests, laparotomy, and biopsy are viewed together to determine the stage of the cancer. Sometimes, a laparoscopy will be done before the laparotomy to see if the cancer has spread. If the cancer has spread and cannot be removed by surgery, the surgeon may decide not to do a laparotomy.
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood:
- Tissue. The cancer spreads from where it began by growing into nearby areas.
- Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessel to other parts of the body.
- Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
- Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
- Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if extrahepatic bile duct cancer spreads to the liver, the cancer cells in the liver are actually extrahepatic bile duct cancer cells. The disease is metastatic extrahepatic bile duct cancer, not liver cancer.
There are two staging systems for extrahepatic bile duct cancer.
Extrahepatic bile duct cancer has two staging systems. The staging system used depends on where in the extrahepatic bile duct the cancer first formed.
- Perihilar or proximal extrahepatic bile duct tumors (perihilar bile duct tumors) form in the area where the bile duct leaves the liver. This type of tumor is also called a Klatskin tumor.
- Distal extrahepatic bile duct tumors (distal bile duct tumors) form in the area where the bile duct empties into the small intestine.
The following stages are used for perihilar extrahepatic bile duct cancer:
Stage 0 (Carcinoma in Situ)
In stage 0, abnormal cells are found in the innermost layer of tissue lining the perihilar bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.
In stage I, cancer has formed in the innermost layer of the wall of the perihilar bile duct and has spread into the muscle and fibrous tissue of the wall.
In stage II, cancer has spread through the wall of the perihilar bile duct to nearby fatty tissue or to the liver.
Stage III is divided into stages IIIA and IIIB.
- Stage IIIA: The tumor has spread to one branch of the hepatic artery or of the portal vein.
- Stage IIIB: The tumor has spread to nearby lymph nodes. Cancer has also spread into the wall of the perihilar bile duct and may have spread through the wall to nearby fatty tissue, the liver, or to one branch of the hepatic artery or of the portal vein.
Stage IV is divided into stages IVA and IVB.
- Stage IVA: The tumor may have spread to nearby lymph nodes and has spread to one or more of the following:
- the main part of the portal vein or both branches of the portal vein;
- the hepatic artery;
- the right and left hepatic ducts;
- the right hepatic duct and the left branch of the hepatic artery or of the portal vein;
- the left hepatic duct and the right branch of the hepatic artery or of the portal vein.
- Stage IVB: The tumor has spread to other parts of the body, such as the liver.
The following stages are used for distal extrahepatic bile duct cancer:
Stage 0 (Carcinoma in Situ
In stage 0, abnormal cells are found in the innermost layer of tissue lining the distal bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.
In stage I, cancer has formed. Stage I is divided into stages IA and IB.
- Stage IA: Cancer is found in the distal bile duct only.
- Stage IB: Cancer has spread all the way through the wall of the distal bile duct.
Stage II is divided into stages IIA and IIB.
- Stage IIA: Cancer has spread from the distal bile duct to the gallbladder, pancreas, small intestine, or other nearby organs.
- Stage IIB: Cancer has spread from the distal bile duct to nearby lymph nodes. Cancer may have spread through the wall of the distal bile duct or to the gallbladder, pancreas, small intestine, or other nearby organs.
In stage III, cancer has spread to the large vessels that carry blood to the organs in the abdomen. Cancer may have spread to nearby lymph nodes.
In stage IV, cancer has spread to other parts of the body, such as the liver or lungs.
Extrahepatic bile duct cancer can also be grouped according to how the cancer may be treated. There are two treatment groups:
Localized (and resectable)
The cancer is in an area where it can be removed completely by surgery.
Unresectable, recurrent, or metastatic
- Unresectable cancer cannot be removed completely by surgery. Most patients with extrahepatic bile duct cancer have unresectable cancer.
- Recurrent cancer is cancer that has recurred (come back) after it has been treated. Extrahepatic bile duct cancer may come back in the bile duct or in other parts of the body.
- Metastasis is the spread of cancer from the primary site (place where it started) to other places in the body. Metastatic extrahepatic bile duct cancer may have spread to nearby blood vessels, the liver, the common bile duct, nearby lymph nodes, other parts of the abdominal cavity, or to distant parts of the body.
At Huntsman Cancer Institute, bile duct cancer is treated by a team of specialists including surgeons, medical oncologists (doctors who treat cancer with medicine), radiation oncologists (doctors who treat cancer with radiation), gastroenterologists (doctors who treat diseases of the digestive system), nurses, dietitians, and social workers.
Different types of treatment are available for patients with extrahepatic bile duct cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Three types of standard treatment are used:
New treatments are being tested in clinical trials.
There are different types of surgery for bile duct cancer.
- Removal of the bile duct: If the tumor is small and only in the bile duct, the entire bile duct may be removed. A new duct is made by connecting the duct openings in the liver to the intestine. Lymph nodes are removed and viewed under a microscope to see if they contain cancer.
- Partial hepatectomy: Removal of the part of the liver where cancer is found. The part removed may be a wedge of tissue, an entire lobe, or a larger part of the liver, along with some normal tissue around it.
- Whipple procedure: A surgical procedure in which the head of the pancreas, the gallbladder, part of the stomach, part of the small intestine, and the bile duct are removed. Enough of the pancreas is left to make digestive juices and insulin.
- Surgical biliary bypass: If the tumor cannot be removed but is blocking the small intestine and causing bile to build up in the gallbladder, a biliary bypass may be done. During this operation, the gallbladder or bile duct will be cut and sewn to the small intestine to create a new pathway around the blocked area. This procedure helps to relieve jaundice caused by the build-up of bile.
- Stent placement: If the tumor is blocking the bile duct, a stent (a thin tube) may be placed in the duct to drain bile that has built up in the area. The stent may drain to the outside of the body or it may go around the blocked area and drain the bile into the small intestine. The doctor may place the stent during surgery or PTC, or with an endoscope.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Chemotherapy uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Learn more about this treatment in our introduction to chemotherapy video.
Clinical trials are studying ways to improve the effect of radiation therapy on tumor cells, including the following:
- Hyperthermia therapy: A treatment in which body tissue is exposed to high temperatures to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation therapy and certain anticancer drugs.
- Radiosensitizers: Drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells.
For more information, visit HCI's clinical trials website.
When you or someone you love is diagnosed with cancer, concerns about treatments and side effects, hospital stays, and medical bills are common. You may also worry about caring for your family, work, or normal daily life.
There are several places you can go for support:
- Your health care team can answer your questions and talk to you about your concerns. They can help you with any side effects and keep you informed of all your treatments, test results, and future doctor visits.
- The G. Mitchell Morris Cancer Learning Center has hundreds of free brochures and over 3,000 books, DVDs, and CDs available for checkout.You can browse the library, perform Internet research, or talk with a cancer information specialist.
- Our Patient and Family Support Services offer emotional support and resources for coping with cancer and its impact on daily life to HCI patients and their families.
- The Linda B. and Robert B. Wiggins Wellness-Survivorship Center offers many programs to increase the quality of life and well-being of HCI patients and their families.
Adapted from the National Cancer Institute's Physician Data Query (PDQ®) Cancer Information Summaries
This information last updated on HCI website December 2013
*If you are interested in a trial that is currently marked *Not Open, please contact the Patient Education team at 1-888-424-2100 or firstname.lastname@example.org for other trial options. Enrollment is updated daily.
Forte Research Systems in partnership with Huntsman Cancer Institute
Douglas G. Adler, M.D.Locations
|Huntsman Cancer Institute||(801) 213-9797|
|Redwood Health Center||(801) 213-9797|
|University Hospital||(801) 213-9797|
|University Hospital||(801) 213-9797|
Specialties: Biliary Cancer, Endoscopic Retrograde Cholangiopancreatography (ERCP), Endoscopic Ultrasound, Endoscopy, Esophageal Diseases, Gastroenterology, Pancreatic Cancer, Therapeutic Endoscopy
Ignacio Garrido-Laguna, M.D., Ph.D.Locations
|Huntsman Cancer Hospital||(801) 585-0193|
|Huntsman Cancer Hospital||(801) 213-4266|
Specialties: Biliary Cancer, Colon Cancer, Medical Oncology, Oncology, Pancreatic Cancer
Sean J. Mulvihill, M.D.Locations
|Huntsman Cancer Institute||(801) 581-7167|
Specialties: Biliary Cancer, Hepatopancreatobiliary (Liver/Pancreas/Biliary) Surgery, Laparoscopy, Liver Cancer, Pancreatic Cancer, Surgery, General
Diseases and Conditions
- Am I At Risk for Bile Duct Cancer?
- Can I Survive Bile Duct Cancer? What Is My Prognosis?
- How Does My Doctor Know I Have Bile Duct Cancer?
- I’ve Just Been Told I Have Bile Duct Cancer
- Statistics About Bile Duct Cancer
- Tell Your Healthcare Team How You Feel During Treatment for Bile Duct Cancer
- Types and Goals of Treatment for Bile Duct Cancer
- Understanding Your Stage of Bile Duct Cancer
- What Are the Symptoms of Bile Duct Cancer?
- What Is Bile Duct Cancer?
- What to Know About Stent Placement for Bile Duct Cancer
- What to Know About Surgery for Bile Duct Cancer
- Do What You Can to Ease Side Effects of Treatment for Bile Duct Cancer
- What to Know About Chemotherapy for Bile Duct Cancer
- What to Know About Radiation Therapy for Bile Duct Cancer