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Diseases & Conditions

Extrahepatic Bile Duct Cancer

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).
  • 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.
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.

Stages of Extrahepatic Bile Duct Cancer

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 is usually 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:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

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.

  • 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.

Stage I

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.

Stage II

In stage II, cancer has spread through the wall of the perihilar bile duct to nearby fatty tissue or to the liver.

Stage III

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 (vessels that carry blood to and from the liver).
  • 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 (vessels that carry blood to and from the liver).
Stage IV

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 (a vessel that carries blood away from the liver) or both branches of the portal vein;
    • the hepatic artery (a vessel that carries blood to the liver);
    • 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.

Stage I

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

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.
Stage III

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.

Stage IV

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.

Treatment Option Overview

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:

Surgery

The following types of surgery are used to treat extrahepatic 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

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

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 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 website.

Radiation sensitizers

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.
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.

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.

Treatment Options for Extrahepatic Bile Duct Cancer

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:

  • Stent placement or biliary bypass to relieve blockage of the bile duct may be done before surgery to relieve jaundice.
  • Surgery, with or without external-beam radiation therapy.
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:

  • Stent placement or biliary bypass with or without internal or external radiation therapy, as palliative treatment to relieve symptoms and improve the quality of life.
  • Chemotherapy.
  • A clinical trial of new ways to give palliative radiation therapy, such as combining it with hyperthermia therapy, radiosensitizers, or chemotherapy.
  • A clinical trial of new drugs and drug combinations.

For more information from the NCI, please write to this address:

NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322

U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 800.4.CANCER 800.422.6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 800.332.8615. Information about ongoing clinical trials is available from the NCI website.

Source: National Institutes of Health; National Cancer Institute

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 9/6/2012...#6135