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Department of Surgery »  Conditions & Procedures »  Bile Duct Cancer (Cholangiocarcinoma)

Bile Duct Cancer (Cholangiocarcinoma)

Bile Duct Cancer (Cholangiocarcinoma) is a cancer of the bile ducts which drain bile from the liver into the small intestine. Other biliary tract cancers include pancreatic cancer, gallbladder cancer, and cancer of the ampulla of Vater. Cholangiocarcinoma is a relatively rare adenocarcinoma (glandular cancer), with an annual incidence of 1-2 cases per 100,000 in the Western world, but rates of cholangiocarcinoma have been rising worldwide over the past several decades.

Bile Duct System

The bile duct system begins in the liver where bile collects from liver cells in tiny tubes called "ductules" . The ductules unite to form small ducts, which become progressively larger and finally into the left and right hepatic ducts. Ducts within the liver are referred to as intrahepatic bile ducts. When they emerge  from the liver, they join to form the common hepatic duct at the hilum, where important structures such as blood vessels enter the liver.

Approximately a third of the way down the length of the bile duct, the gallbladder, which stores bile, is attached to the hepatic duct via a small cystic duct. The merged duct is known as the common bile duct. The common bile duct passes through a portion of the pancreas and merging with the pancreatic duct as they enter the first part of the small intestine known as the duodenum.   

Bile duct cancer is classified by its location in the biliary system, also called "biliary tree". The  vast majority (≈ 95%) are  adenocarcinomas, cancers of glandular cells that can develop in most organs of the body. Bile duct adenocarcinomas derive from the mucus glands lining the inside of the duct. The precise medical term for bile duct adenocarcinoma is cholangiocarcinoma. "Cholangio" means "pertaining to the bile duct". Bile duct cancers that arise inside the bile ducts of the liver are known as intrahepatic; those outside the liver as extrahepatic. Note: Not all bile duct tumors are cancerous. Benign (non-cancerous) bile duct tumors include bile duct hamartomas and adenomas.  

UCSF045_ExtrahepaticBileDuctAnatomy.jpg

Intrahepatic Bile Duct Cancers

Intrahepatic bile duct cancers develop in the bile duct branches of the liver. While compising only 10% of bile duct cancers, their incidence is thought to be on the rise. Cholangiocarcinomas within the liver are not the same as cancers which develop in the liver cells known as hepatocellular carcinomas, although the two are sometimes confused.

Extrahepatic Bile Duct Cancer

Perihilar Bile Duct Cancers ("Klatskin Tumors")

Perihilar or "hilar" bile duct cances develop in the upper part of the biliary tree  hilum, the region where the left and right hepatic ducts join and exit the liver.  Klatskin tumors are the most common bile duct cancer.

Distal Bile Duct Cancers

Distal (lower) bile duct cancers arise in the lower half of the biliary tree closer to the small intestine. These cancers are intimately associated with the pancreas because it is here that the bile duct passes through  the pancreas as it enters the duodenum.  Patients with distal bile duct cancer often have symptoms similar to pancreatic and cancer of the ampulla of Vater, the point at which pancreatic and common bile ducts join. Distal bile duct tumors, pancreatic cancer and ampullary cancer are often referred to as periampullary tumors because they present in patients with the same symptoms. 

Extrahepatic Bile Duct Cancer

Having colitis or certain liver diseases can increase the risk of developing .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.

Stages

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:

  • 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.

The following stages are used for extrahepatic bile duct cancer:

Stage 0 (Carcinoma in Situ)

In stage 0, abnormal cells are found in the innermost layer of tissue lining the extrahepatic 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 stage IA and stage IB.

  • Stage IA: Cancer is found in the bile duct only.
  • Stage IB: Cancer has spread through the wall of the bile duct.

Stage II

Stage II is divided into stage IIA and stage IIB.

  • Stage IIA: Cancer has spread to the liver, gallbladder, pancreas, and/or to either the right or left branch of the hepatic artery or to the right or left branch of the portal vein.
  • Stage IIB: Cancer has spread to nearby lymph nodes and:
    • is found in the bile duct; or
    • has spread through the wall of the bile duct; or
    • has spread to the liver, gallbladder, pancreas, and/or the right or left branches of the hepatic artery or portal vein.

Stage III

In stage III, cancer has spread:

  • to the main portal vein or to both right and left branches of the portal vein; or
  • to the hepatic artery; or
  • to other nearby organs or tissues, such as the colon, stomach, small intestine, or abdominal wall.

Cancer may have spread to nearby lymph nodes also.

Stage IV

In stage IV, cancer has spread to lymph nodes and/or organs far away from the extrahepatic bile duct.

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

The cancer cannot be removed completely by surgery. The cancer may have spread to nearby blood vessels, the liver, the common bile duct, nearby lymph nodes, or other parts of the abdominal cavity.

Treatment Options 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.

Two types of standard treatment are used:

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.

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.

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.

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 spinal column, 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.

Biologic therapy

Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

Treatment Options by Extent of Disease

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:

  • 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.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry 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 Extrahepatic Bile Duct Cancer

Treatment of unresectable extrahepatic bile duct cancer 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.
  • A clinical trial of hyperthermia therapy, radiosensitizers, chemotherapy, or biologic therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with unresectable 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.

Recurrent Extrahepatic Bile Duct Cancer

Treatment of recurrent extrahepatic bile duct cancer may include the following:

  • Palliative treatment to relieve symptoms and improve quality of life.
  • A clinical trial.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent 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.

Recurrent Bile Duct Cancer

Recurrent extrahepatic bile duct cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the bile duct or in other parts of the body.

 

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