21st Century Adult Cancer Sourcebook: Extrahepatic Bile Duct Cancer - Clinical Data for Patients, Families, and Physicians
Edition 1.0 - September 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
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PART ONE
Chapter 1A: Extrahepatic Bile Duct Cancer Patient Information
Chapter 2A: Extrahepatic Bile Duct Cancer Health Professional Information
Chapter 3A: Extrahepatic Bile Duct Cancer Clinical Trials
PART TWO
Chapter 1B: Levels of Evidence for Adult and Pediatric Cancer Treatment Studies (NCI)
Chapter 2B: Glossary of Clinical Trial Terms
Chapter 3B: Clinical Trials Background Information
Chapter 4B: Cancer Clinical Trials -The Basic Workbook
Chapter 5B: Cancer Clinical Trials - The In-Depth Program
Chapter 6B: Clinical Trials at NIH
Chapter 7B: How To Find A Cancer Treatment Trial: A Ten Step Guide
Chapter 8B: Taking Part in Cancer Treatment Research Studies
Chapter 9B: Cancer Clinical Trials
Chapter 10B: Access to Investigational Drugs
Chapter 12B: Taking Time: Support for People with Cancer
Chapter 13B: Facing Forward - Life After Cancer Treatment
Chapter 14B: Chemotherapy and You
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PART ONE
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Chapter 1A: Extrahepatic Bile Duct Cancer Patient Information
Extrahepatic Bile Duct Cancer Treatment
Last Modified: 08/19/2011
Patient Version
General Information About Extrahepatic Bile Duct Cancer
Key Points for This Section
* 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.
* Having colitis or certain liver diseases can increase the risk of developing extrahepatic bile duct cancer.
* Possible signs of extrahepatic bile duct cancer include jaundice and pain.
* Tests that examine the bile duct and liver are used to detect (find) and diagnose extrahepatic bile duct cancer.
* Certain factors affect prognosis (chance of recovery) and treatment options.
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.
Anatomy of the extrahepatic bile duct. The extrahepatic bile duct is part of a network of small ducts in the liver that collects bile. Bile flows into the right and left hepatic ducts which join together to form the common hepatic duct. The extrahepatic bile duct is the part of the common hepatic duct that is outside the liver. Bile flows through the extrahepatic bile duct and into the cystic duct to the gallbladder where it is stored. When food is being digested, bile flows back through the cystic duct and into the common bile duct where it is released into the small intestine.
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.
Glossary Terms
abdomen (AB-doh-men) * The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
bile (bile) * A fluid made by the liver and stored in the gallbladder. Bile is excreted into the small intestine, where it helps digest fat.
bile duct (bile dukt) * A tube through which bile passes in and out of the liver.
biopsy (BY-op-see) * The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
blood (blud) * A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
catheter (KA-theh-ter) * A flexible tube used to deliver fluids into or withdraw fluids from the body.
cell (sel) * The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
chronic (KRAH-nik) * A disease or condition that persists or progresses over a long period of time.
common bile duct (KAH-mun bile dukt) * A tube that carries bile from the liver and the gallbladder through the pancreas and into the duodenum (the upper part of the small intestine). It is formed where the ducts from the liver and gallbladder are joined. It is part of the biliary duct system.
contrast material (KON-trast muh-TEER-ee-ul) * A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.
CT scan (… skan) * A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
cyst (sist) * A sac or capsule in the body. It may be filled with fluid or other material.
diagnosis (DY-ug-NOH-sis) * The process of identifying a disease, such as cancer, from its signs and symptoms.
digestion (dy-JES-chun) * The process of breaking down food into substances the body can use for energy, tissue growth, and repair.
disorder (dis-OR-der) * In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.
drain (drayn) * In medicine, to remove fluid as it collects; or, a tube or wick-like device used to remove fluid from a body cavity, wound, or infected area.
duct (dukt) * In medicine, a tube or vessel of the body through which fluids pass.
endoscope (EN-doh-SKOPE) * A thin, tube-like instrument used to look at tissues inside the body. An endoscope has a light and a lens for viewing and may have a tool to remove tissue.
ERCP * A procedure that uses an endoscope to examine and x-ray the pancreatic duct, hepatic duct, common bile duct, duodenal papilla, and gallbladder. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. The endoscope is passed through the mouth and down into the first part of the small intestine (duodenum). A smaller tube (catheter) is then inserted through the endoscope into the bile and pancreatic ducts. A dye is injected through the catheter into the ducts, and an x-ray is taken. Also called endoscopic retrograde cholangiopancreatography.
esophagus (ee-SAH-fuh-gus) * The muscular tube through which food passes from the throat to the stomach.
extrahepatic bile duct (EK-struh-heh-PA-tik bile dukt) * The part of the common hepatic bile duct (tube that collects bile from the liver) that is outside the liver. This duct joins a duct from the gallbladder to form the common bile duct, which carries bile into the small intestine when food is being digested.
extrahepatic bile duct cancer (EK-struh-heh-PA-tik bile dukt KAN-ser) * A rare cancer that forms in the part of the bile duct that is outside the liver. The bile duct is the tube that collects bile from the liver and joins a duct from the gallbladder to form the common bile duct, which carries bile into the small intestine when food is being digested.
fever (FEE-ver) * An increase in body temperature above normal (98.6 degrees F), usually caused by disease.
fine-needle aspiration biopsy (... NEE-dul AS-pih-RAY-shun BY-op-see) * The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.
fluid (FLOO-id) * A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.
gallbladder (GAWL-bla-der) * The pear-shaped organ found below the liver. Bile is concentrated and stored in the gallbladder.
hepatic (heh-PA-tik) * Refers to the liver.
infection (in-FEK-shun) * Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.
injection (in-JEK-shun) * Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
jaundice (JAWN-dis) * A condition in which the skin and the whites of the eyes become yellow, urine darkens, and the color of stool becomes lighter than normal. Jaundice occurs when the liver is not working properly or when a bile duct is blocked.
liver (LIH-ver) * A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.
liver function test (LIH-ver FUNK-shun ...) * A blood test to measure the blood levels of certain substances released by the liver. A high or low level of certain substances can be a sign of liver disease.
MRI * A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.
organ (OR-gun) * A part of the body that performs a specific function. For example, the heart is an organ.
palliative therapy (PA-lee-uh-tiv THAYR-uh-pee) * Treatment given to relieve the symptoms and reduce the suffering caused by cancer and other life-threatening diseases. Palliative cancer therapies are given together with other cancer treatments, from the time of diagnosis, through treatment, survivorship, recurrent or advanced disease, and at the end of life.
pancreatic duct (PAN-kree-A-tik dukt) * Part of a system of ducts in the pancreas. Pancreatic juices containing enzymes are released into these ducts and flow into the small intestine.
parasite (PAYR-uh-SITE) * An animal or plant that gets nutrients by living on or in an organism of another species. A complete parasite gets all of its nutrients from the host organism, but a semi-parasite gets only some of its nutrients from the host.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun) * An exam of the body to check for general signs of disease.
prognosis (prog-NO-sis) * The likely outcome or course of a disease; the chance of recovery or recurrence.
PTC * A procedure to x-ray the hepatic and common bile ducts. A contrasting agent is injected into the liver or bile duct, and the ducts are then x-rayed to find the point of obstruction. Also called percutaneous transhepatic cholangiography.
quality of life (KWAH-lih-tee ... life) * The overall enjoyment of life. Many clinical trials assess the effects of cancer and its treatment on the quality of life. These studies measure aspects of an individual’s sense of well-being and ability to carry out various activities.
recurrent cancer (ree-KER-ent KAN-ser) * Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.
risk factor (... FAK-ter) * Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.
small intestine (... in-TES-tin) * The part of the digestive tract that is located between the stomach and the large intestine.
sonogram (SAH-noh-gram) * A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called ultrasonogram.
spiral CT scan (SPY-rul … skan) * A detailed picture of areas inside the body. The pictures are created by a computer linked to an x-ray machine that scans the body in a spiral path. Also called helical computed tomography.
stage * The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.
stent (stent) * A device placed in a body structure (such as a blood vessel or the gastrointestinal tract) to keep the structure open.
stomach (STUH-muk) * An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.
surgery (SER-juh-ree) * A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom (SIMP-tum) * An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
tissue (TIH-shoo) * A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer) * An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.
ulcerative colitis (UL-seh-RUH-tiv koh-LY-tis) * Chronic inflammation of the colon that produces ulcers in its lining. This condition is marked by abdominal pain, cramps, and loose discharges of pus, blood, and mucus from the bowel.
ultrasound (UL-truh-SOWND) * A procedure in which high-energy sound waves are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.
vein (vayn) * A blood vessel that carries blood to the heart from tissues and organs in the body.
x-ray (EX-ray) * A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.
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Stages of Extrahepatic Bile Duct Cancer
Key Points for This Section
* 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.
* There are three ways that cancer spreads in the body.
* The following stages are used for extrahepatic bile duct cancer:
* Stage 0 (Carcinoma in Situ)
* Stage I
* Stage II
* Stage III
* Stage IV
* Extrahepatic bile duct cancer can also be grouped according to how the cancer may be treated. There are two treatment groups:
* Localized (and resectable)
* Unresectable, recurrent, or metastatic
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, 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.
Glossary Terms
abdomen (AB-doh-men) * The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
abdominal (ab-DAH-mih-nul) * Having to do with the abdomen, which is the part of the body between the chest and the hips that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
abnormal (ab-NOR-mul) * Not normal. An abnormal lesion or growth may be cancer, premalignant (likely to become cancer), or benign (not cancer).
bile duct (bile dukt) * A tube through which bile passes in and out of the liver.
biopsy (BY-op-see) * The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
blood (blud) * A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
blood vessel (blud VEH-sel) * A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.
bone cancer (bone KAN-ser) * Primary bone cancer is cancer that forms in cells of the bone. Some types of primary bone cancer are osteosarcoma, Ewing sarcoma, malignant fibrous histiocytoma, and chondrosarcoma. Secondary bone cancer is cancer that spreads to the bone from another part of the body (such as the prostate, breast, or lung).
breast cancer (brest KAN-ser) * Cancer that forms in tissues of the breast, usually the ducts (tubes that carry milk to the nipple) and lobules (glands that make milk). It occurs in both men and women, although male breast cancer is rare.
cancer (KAN-ser) * A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.
capillary (KA-pih-layr-ee) * The smallest type of blood vessel. A capillary connects an arteriole (small artery) to a venule (small vein) to form a network of blood vessels in almost all parts of the body. The wall of a capillary is thin and leaky, and capillaries are involved in the exchange of fluids and gases between tissues and the blood.
carcinoma in situ (KAR-sih-NOH-muh in SY-too) * A group of abnormal cells that remain in the place where they first formed. They have not spread. These abnormal cells may become cancer and spread into nearby normal tissue. Also called stage 0 disease.
cavity (KA-vih-tee) * A hollow area or hole. It may describe a body cavity (such as the space within the abdomen) or a hole in a tooth caused by decay.
cell (sel) * The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
colon (KOH-lun) * The longest part of the large intestine, which is a tube-like organ connected to the small intestine at one end and the anus at the other. The colon removes water and some nutrients and electrolytes from partially digested food. The remaining material, solid waste called stool, moves through the colon to the rectum and leaves the body through the anus.
common bile duct (KAH-mun bile dukt) * A tube that carries bile from the liver and the gallbladder through the pancreas and into the duodenum (the upper part of the small intestine). It is formed where the ducts from the liver and gallbladder are joined. It is part of the biliary duct system.
diagnostic procedure (DY-ug-NAH-stik proh-SEE-jer) * A type of test used to help diagnose a disease or condition. Mammograms and colonoscopies are examples of diagnostic procedures. Also called diagnostic test.
extrahepatic bile duct (EK-struh-heh-PA-tik bile dukt) * The part of the common hepatic bile duct (tube that collects bile from the liver) that is outside the liver. This duct joins a duct from the gallbladder to form the common bile duct, which carries bile into the small intestine when food is being digested.
extrahepatic bile duct cancer (EK-struh-heh-PA-tik bile dukt KAN-ser) * A rare cancer that forms in the part of the bile duct that is outside the liver. The bile duct is the tube that collects bile from the liver and joins a duct from the gallbladder to form the common bile duct, which carries bile into the small intestine when food is being digested.
fluid (FLOO-id) * A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.
gallbladder (GAWL-bla-der) * The pear-shaped organ found below the liver. Bile is concentrated and stored in the gallbladder.
hepatic artery (heh-PA-tik AR-tuh-ree) * The major blood vessel that carries blood to the liver.
imaging procedure (IH-muh-jing proh-SEE-jer) * A type of test that makes pictures of areas inside the body. Some examples of imaging procedures are CT scans and MRIs. Also called imaging test.
incision (in-SIH-zhun) * A cut made in the body to perform surgery.
invasive cancer (in-VAY-siv KAN-ser) * Cancer that has spread beyond the layer of tissue in which it developed and is growing into surrounding, healthy tissues. Also called infiltrating cancer.
laparoscopy (LA-puh-ROS-koh-pee) * A procedure that uses a laparoscope, inserted through the abdominal wall, to examine the inside of the abdomen. A laparoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue to be checked under a microscope for signs of disease.
laparotomy (LA-puh-RAH-toh-mee) * A surgical incision made in the wall of the abdomen.
liver (LIH-ver) * A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.
lymph (limf) * The clear fluid that travels through the lymphatic system and carries cells that help fight infections and other diseases. Also called lymphatic fluid.
lymph node (limf node) * A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.
lymph vessel (limf ...) * A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.
lymphatic system (lim-FA-tik SIS-tem) * The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.
metastasis (meh-TAS-tuh-sis) * The spread of cancer from one part of the body to another. A tumor formed by cells that have spread is called a “metastatic tumor” or a “metastasis.” The metastatic tumor contains cells that are like those in the original (primary) tumor. The plural form of metastasis is metastases (meh-TAS-tuh-SEEZ).
metastatic (meh-tuh-STA-tik) * Having to do with metastasis, which is the spread of cancer from the primary site (place where it started) to other places in the body.
organ (OR-gun) * A part of the body that performs a specific function. For example, the heart is an organ.
pancreas (PAN-kree-us) * A glandular organ located in the abdomen. It makes pancreatic juices, which contain enzymes that aid in digestion, and it produces several hormones, including insulin. The pancreas is surrounded by the stomach, intestines, and other organs.
portal vein (POR-tul vayn) * A blood vessel that carries blood to the liver from the stomach, small and large intestines, spleen, pancreas, and gallbladder. Also called hepatic portal vein.
primary tumor (PRY-mayr-ee TOO-mer) * The original tumor.
recurrent cancer (ree-KER-ent KAN-ser) * Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.
small intestine (... in-TES-tin) * The part of the digestive tract that is located between the stomach and the large intestine.
stage * The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.
stage 0 extrahepatic bile duct carcinoma in situ (... EK-struh-heh-PA-tik bile dukt KAR-sih-NOH-muh in SY-too) * 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 I extrahepatic bile duct cancer (... EK-struh-heh-PA-tik bile dukt KAN-ser) * Stage I is divided into stage IA and IB. In stage IA, cancer is found only in the bile duct. In stage IB, cancer has spread through the wall of the bile duct.
stage II extrahepatic bile duct cancer (... EK-struh-heh-PA-tik bile dukt KAN-ser) * Stage II is divided into stage IIA and IIB. In stage IIA, cancer has spread to the liver, gallbladder, pancreas, and/or to either the right or left branch of the hepatic artery (major blood vessel that carries blood to the liver from the heart) or the right or left branch of the portal vein (major blood vessel that carries blood to the liver from organs in the abdomen). In stage IIB, cancer has spread to nearby lymph nodes and (1) is found in the bile duct; or (2) has spread through the wall of the bile duct; or (3) has spread to the liver, gallbladder, pancreas, and/or the right or left branch of the hepatic artery or portal vein.
stage III extrahepatic bile duct cancer (... EK-struh-heh-PA-tik bile dukt KAN-ser) * Cancer has spread (1) to the main portal vein (major blood vessel that carries blood to the liver from organs in the abdomen) or to both branches of the portal vein; or (2) to the hepatic artery (major blood vessel that carries blood to the liver from the heart); or (3) to other nearby organs or tissues, such as the colon, stomach, small intestine, or abdominal wall. Cancer may also have spread to nearby lymph nodes.
stage IV extrahepatic bile duct cancer (... EK-struh-heh-PA-tik bile dukt KAN-ser) * Cancer has spread to lymph nodes and/or organs far away from the extrahepatic bile duct.
staging (STAY-jing) * Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. It is important to know the stage of the disease in order to plan the best treatment.
stomach (STUH-muk) * An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.
surgeon (SER-jun) * A doctor who removes or repairs a part of the body by operating on the patient.
surgery (SER-juh-ree) * A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
tissue (TIH-shoo) * A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer) * An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.
unresectable (UN-ree-SEK-tuh-bul) * Unable to be removed with surgery.
vein (vayn) * A blood vessel that carries blood to the heart from tissues and organs in the body.
* * * * * * * * * * * *
Treatment Option Overview
Key Points for This Section
* There are different types of treatment for patients with extrahepatic bile duct cancer.
* Three types of standard treatment are used:
* Surgery
* Radiation therapy
* Chemotherapy
* New types of treatment are being tested in clinical trials.
* Radiation sensitizers
* Patients may want to think about taking part in a clinical trial.
* Patients can enter clinical trials before, during, or after starting their cancer treatment.
* Follow-up tests may be needed.
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 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 1.
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. 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.
Glossary Terms
abdomen (AB-doh-men) * The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
bile (bile) * A fluid made by the liver and stored in the gallbladder. Bile is excreted into the small intestine, where it helps digest fat.
bile duct (bile dukt) * A tube through which bile passes in and out of the liver.
biliary (BIH-lee-AYR-ee) * Having to do with the liver, bile ducts, and/or gallbladder.
bypass (BY-pas) * A surgical procedure in which the doctor creates a new pathway for the flow of body fluids.
cerebrospinal fluid (seh-REE-broh-SPY-nul FLOO-id) * The fluid that flows in and around the hollow spaces of the brain and spinal cord, and between two of the meninges (the thin layers of tissue that cover and protect the brain and spinal cord). Cerebrospinal fluid is made by tissue called the choroid plexus in the ventricles (hollow spaces) in the brain. Also called CSF.
chemotherapy (KEE-moh-THAYR-uh-pee) * Treatment with drugs that kill cancer cells.
clinical trial (KLIH-nih-kul TRY-ul) * A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.
diagnosis (DY-ug-NOH-sis) * The process of identifying a disease, such as cancer, from its signs and symptoms.
drain (drayn) * In medicine, to remove fluid as it collects; or, a tube or wick-like device used to remove fluid from a body cavity, wound, or infected area.
drug (drug) * Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.
duct (dukt) * In medicine, a tube or vessel of the body through which fluids pass.
endoscope (EN-doh-SKOPE) * A thin, tube-like instrument used to look at tissues inside the body. An endoscope has a light and a lens for viewing and may have a tool to remove tissue.
external radiation therapy (...RAY-dee-AY-shun THAYR-uh-pee) * A type of radiation therapy that uses a machine to aim high-energy rays at the cancer from outside of the body. Also called external-beam radiation therapy.
extrahepatic bile duct cancer (EK-struh-heh-PA-tik bile dukt KAN-ser)
* A rare cancer that forms in the part of the bile duct that is outside the liver. The bile duct is the tube that collects bile from the liver and joins a duct from the gallbladder to form the common bile duct, which carries bile into the small intestine when food is being digested.
follow-up (FAH-loh-up) * Monitoring a person's health over time after treatment. This includes keeping track of the health of people who participate in a clinical study or clinical trial for a period of time, both during the study and after the study ends.
gallbladder (GAWL-bla-der) * The pear-shaped organ found below the liver. Bile is concentrated and stored in the gallbladder.
hepatectomy (HEH-puh-TEK-toh-mee) * Surgery to remove all or part of the liver.
hyperthermia therapy (HY-per-THER-mee-uh THAYR-uh-pee) * A type of 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 and certain anticancer drugs.
injection (in-JEK-shun) * Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
insulin (IN-suh-lin) * A hormone made by the islet cells of the pancreas. Insulin controls the amount of sugar in the blood by moving it into the cells, where it can be used by the body for energy.
internal radiation therapy (in-TER-nul RAY-dee-AY-shun THAYR-uh-pee) * A type of radiation therapy in which radioactive material sealed in needles, seeds, wires, or catheters is placed directly into or near a tumor. Also called brachytherapy, implant radiation therapy, and radiation brachytherapy.
intestine (in-TES-tin) * The long, tube-shaped organ in the abdomen that completes the process of digestion. The intestine has two parts, the small intestine and the large intestine. Also called bowel.
jaundice (JAWN-dis) * A condition in which the skin and the whites of the eyes become yellow, urine darkens, and the color of stool becomes lighter than normal. Jaundice occurs when the liver is not working properly or when a bile duct is blocked.
liver (LIH-ver) * A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.
lobe * A portion of an organ, such as the liver, lung, breast, thyroid, or brain.
lymph node (limf node) * A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.
NCI * NCI, part of the National Institutes of Health of the United States Department of Health and Human Services, is the Federal Government's principal agency for cancer research. It conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the NCI Web site at http://www.cancer.gov. Also called National Cancer Institute.
organ (OR-gun) * A part of the body that performs a specific function. For example, the heart is an organ.
pancreas (PAN-kree-us) * A glandular organ located in the abdomen. It makes pancreatic juices, which contain enzymes that aid in digestion, and it produces several hormones, including insulin. The pancreas is surrounded by the stomach, intestines, and other organs.
pancreatic juice (PAN-kree-A-tik joos) * Fluid made by the pancreas. Pancreatic juices contain proteins called enzymes that aid in digestion.
PTC * A procedure to x-ray the hepatic and common bile ducts. A contrasting agent is injected into the liver or bile duct, and the ducts are then x-rayed to find the point of obstruction. Also called percutaneous transhepatic cholangiography.
radiation (RAY-dee-AY-shun) * Energy released in the form of particle or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, medical x-rays, and energy given off by a radioisotope (unstable form of a chemical element that releases radiation as it breaks down and becomes more stable).
radiation therapy (RAY-dee-AY-shun THAYR-uh-pee) * The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.
radioactive (RAY-dee-oh-AK-tiv) * Giving off radiation.
radioactive seed (RAY-dee-oh-AK-tiv...) * A small, radioactive pellet that is placed in or near a tumor. Cancer cells are killed by the energy given off as the radioactive material breaks down and becomes more stable.
radiosensitizer (RAY-dee-oh-SEN-sih-TY-zer) * A drug that makes tumor cells more sensitive to radiation therapy.
recur * To come back or to return.
regional chemotherapy (REE-juh-nul KEE-moh-THAYR-uh-pee) * Treatment with anticancer drugs directed to a specific area of the body.
side effect (side eh-FEKT) * A problem that occurs when treatment affects healthy tissues or organs. Some common side effects of cancer treatment are fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores.
small intestine (... in-TES-tin) * The part of the digestive tract that is located between the stomach and the large intestine.
stage * The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.
standard therapy (... THAYR-uh-pee) * Treatment that experts agree is appropriate, accepted, and widely used. Also called best practice, standard medical care, and standard of care.
stent (stent) * A device placed in a body structure (such as a blood vessel or the gastrointestinal tract) to keep the structure open.
surgery (SER-juh-ree) * A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
systemic chemotherapy (sis-TEH-mik KEE-moh-THAYR-uh-pee) * Treatment with anticancer drugs that travel through the blood to cells all over the body.
tissue (TIH-shoo) * A group or layer of cells that work together to perform a specific function.