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Extrahepatic bile duct cancer is a rare disease in which malignant (cancer) cells form in the ducts that are outside the liver.
The extrahepatic bile duct is made up of two parts:
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.
Anatomy of the extrahepatic bile duct. The extrahepatic bile duct is made up of the common hepatic duct and the common bile duct. Bile is made in the liver and flows through the extrahepatic bile duct to the gallbladder where it is stored.
Having colitis or certain liver diseases can increase the risk of 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. Talk with your doctor if you think you may be at risk. Risk factors include having any of the following disorders:
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. Check with your doctor if you have any of the following problems:
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:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
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.
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 extrahepatic 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.
Extrahepatic 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.
The three ways that cancer spreads in the body are:
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone 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.
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 IV is divided into stages IVA and IVB.
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 II is divided into stages IIA and IIB.
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.
There are different types of treatment for patients with extrahepatic bile duct cancer.
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:
The following types of surgery are used to treat extrahepatic bile duct cancer:
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 is a cancer treatment that 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). The way the chemotherapy is given depends on the type and stage of the cancer being treated.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.
Clinical trials are studying ways to improve the effect of radiation therapy on tumor cells, including the following:
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Localized Extrahepatic Bile Duct Cancer
Treatment of localized extrahepatic bile duct cancer may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized extrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Unresectable, Recurrent, or Metastatic Extrahepatic Bile Duct Cancer
Treatment of unresectable, recurrent, or metastatic extrahepatic bile duct cancer is usually within a clinical trial. Treatment may include the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with unresectable extrahepatic bile duct cancer, recurrent extrahepatic bile duct cancer and metastatic extrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
For more information from the National Cancer Institute about extrahepatic bile duct cancer, see the Extrahepatic Bile Duct Cancer Home Page.
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Last Revised: 2013-01-18
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