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.
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.
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.
Recurrent Extrahepatic 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.
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.
Two 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.
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 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.
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 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.
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.
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 Web site.
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: 7/31/2008...#6135
