21st Century Adult Cancer Sourcebook: Gastrointestinal Stromal Tumors (GIST) - Clinical Data for Patients, Families, and Physicians
Edition 1.0 - October 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
Questions? Suggestions? Comments? Concerns? Please contact the publisher directly at
Remember, the book retailer can't answer your questions, but we can!
* * * * * * * * * * *
Smashwords Edition, License Notes
This ebook is licensed for your personal enjoyment only. This ebook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each person you share it with. If you're reading this book and did not purchase it, or it was not purchased for your use only, then you should return to Smashwords.com and purchase your own copy. Thank you for respecting the hard work of this author.
* * * * * * * * * * * *
IMPORTANT NOTE: Information in this e-book is not a substitute for professional medical advice. If you have or suspect that you have any illness, you must consult with a physician or professional healthcare provider! Call 911 and get to the nearest emergency room if you have serious or worsening symptoms.
This material represents a snapshot in time, with authoritative information formatted for ebook reading that was up-to-date at the moment of publication. For the latest cancer updates, please be sure to visit the NCI website:
From our Guide to Leading Medical Websites, here are three valuable sites with authoritative cancer information:
OncoLink * http://www.oncolink.upenn.edu/
eMedicine.com * http://www.emedicine.com/
American Cancer Society (ACS) * http://www.cancer.org/
* * * * * * * * * * * *
This is a privately authored news service and educational publication of Progressive Management. Our publications synthesize official government information with original material - they are not produced by the federal government. They are designed to provide a convenient user-friendly reference work to uniformly present authoritative knowledge that can be rapidly read, reviewed or searched. Vast archives of important data that might otherwise remain inaccessible are available for instant review no matter where you are. This e-book format makes a great reference work and educational tool. There is no other reference book that is as convenient, comprehensive, thoroughly researched, and portable - everything you need to know, from renowned experts you trust. For over a quarter of a century, our news, educational, technical, scientific, and medical publications have made unique and valuable references accessible to all people. Our e-books put knowledge at your fingertips, and an expert in your pocket!
PART ONE
Chapter 1A: Gastrointestinal Stromal Tumors (GIST) Patient Information
Chapter 2A: Gastrointestinal Stromal Tumors (GIST) Health Professional Information
Chapter 3A: Gastrointestinal Stromal Tumors (GIST) 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
* * * * * * * * * * * *
PART ONE
* * * * * * * * * * * *
Chapter 1A: Gastrointestinal Stromal Tumors (GIST) Patient Information
Gastrointestinal Stromal Tumors Treatment
Patient Version
Last Modified: 09/19/2011
General Information About Gastrointestinal Stromal Tumors
Key Points for This Section
* Gastrointestinal stromal tumor is a disease in which abnormal cells form in the tissues of the gastrointestinal tract.
* Genetic factors can increase the risk of having a gastrointestinal stromal tumor.
* Possible signs of gastrointestinal stromal tumors include blood in the stool or vomit.
* Tests that examine the GI tract are used to detect (find) and diagnose gastrointestinal stromal tumors.
* Very small GISTs are common.
* Certain factors affect prognosis (chance of recovery) and treatment options.
Gastrointestinal stromal tumor is a disease in which abnormal cells form in the tissues of the gastrointestinal tract.
The gastrointestinal (GI) tract is part of the body’s digestive system. It helps to digest food and takes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from food so they can be used by the body. The GI tract is made up of the following organs:
* Stomach.
* Small intestine.
* Large intestine (colon).
Gastrointestinal stromal tumors (GISTs) may be malignant (cancer) or benign (not cancer). They are most common in the stomach and small intestine but may be found anywhere in or near the GI tract. Some scientists believe that GISTs begin in cells called interstitial cells of Cajal (ICC), in the wall of the GI tract.
Genetic factors can increase the risk of having a gastrointestinal stromal tumor.
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.
The genes in cells carry the hereditary information received from a person’s parents. The risk of GIST is increased in people who have inherited a mutation (change) in a certain gene. In rare cases, GISTs can be found in several members of the same family.
GIST may be part of a genetic syndrome, but this is rare. A genetic syndrome is a set of symptoms or conditions that occur together and is usually caused by abnormal genes. The following genetic syndromes have been linked to GIST:
* Neurofibromatosis type 1 (NF1).
* Carney triad.
Possible signs of gastrointestinal stromal tumors include blood in the stool or vomit.
These and other symptoms may be caused by GIST. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
* Blood (either bright red or very dark) in the stool or vomit.
* Pain in the abdomen, which may be severe.
* Feeling very tired.
* Trouble or pain when swallowing.
* Feeling full after only a little food is eaten.
Tests that examine the GI tract are used to detect (find) and diagnose gastrointestinal stromal tumors.
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.
* 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.
* 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).
* Endoscopic ultrasound and biopsy. Endoscopy and ultrasound are used to make an image of the upper GI tract and a biopsy is done. An endoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the mouth and into the esophagus, stomach, and first part of the small intestine. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography. Guided by the sonogram, the doctor removes tissue using a thin, hollow needle. A pathologist views the tissue under a microscope to look for cancer cells.
If cancer is found, the following tests may be done to study the cancer cells:
* Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
* Mitotic rate: A measure of how fast the cancer cells are dividing and growing. The mitotic rate is found by counting the number of cells dividing in a certain amount of cancer tissue.
Very small GISTs are common.
Sometimes GISTs are smaller than the eraser on top of a pencil. Tumors may be found during a procedure that is done for another reason, such as an x-ray or surgery. Some of these small tumors will not grow and cause symptoms or spread to the abdomen or other parts of the body. Doctors do not agree on whether these small tumors should be removed or whether they should be watched to see if they begin to grow.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
* How quickly the cancer cells are growing and dividing.
* The size of the tumor.
* Where the tumor is in the body.
* Whether the tumor can be completely removed by surgery.
* Whether the tumor has spread to other 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.
abnormal (ab-NOR-mul)
* Not normal. An abnormal lesion or growth may be cancer, premalignant (likely to become cancer), or benign (not cancer).
antibody (AN-tee-BAH-dee)
* A protein made by plasma cells (a type of white blood cell) in response to an antigen (a substance that causes the body to make a specific immune response). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.
antigen (AN-tih-jen)
* Any substance that causes the body to make a specific immune response.
benign (beh-NINE)
* Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.
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.
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.
carbohydrate (KAR-boh-HY-drayt)
* A sugar molecule. Carbohydrates can be small and simple (for example, glucose) or they can be large and complex (for example, polysaccharides such as starch, chitin or cellulose).
Carney triad (KAR-nee TRY-ad)
* A very rare disorder marked by tumors of the gastrointestinal tract (usually the stomach), tumors that form in embryonic nervous tissue in the head, neck, and torso, and tumors that form in cartilage in the lungs. Sometimes tumors also form in the adrenal glands and esophagus. Carney triad is most common in young females.
cell (sel)
* The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
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.
digestion (dy-JES-chun)
* The process of breaking down food into substances the body can use for energy, tissue growth, and repair.
digestive system (dy-JES-tiv SIS-tem)
* The organs that take in food and turn it into products that the body can use to stay healthy. Waste products the body cannot use leave the body through bowel movements. The digestive system includes the salivary glands, mouth, esophagus, stomach, liver, pancreas, gallbladder, small and large intestines, and rectum.
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.
endoscopic ultrasound (en-doh-SKAH-pik...)
* A procedure in which an endoscope is inserted into the body. An endoscope is a thin, tube-like instrument that has a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal organs to make a picture (sonogram). Also called endosonography and EUS.
endoscopy (en-DOS-koh-pee)
* A procedure that uses an endoscope to examine the inside of the body. An endoscope 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.
esophagus (ee-SAH-fuh-gus)
* The muscular tube through which food passes from the throat to the stomach.
gastrointestinal stromal tumor (GAS-troh-in-TES-tih-nul STROH-mul TOO-mer)
* A type of tumor that usually begins in cells in the wall of the gastrointestinal tract. It can be benign or malignant. Also called GIST.
gastrointestinal tract (GAS-troh-in-TES-tih-nul trakt)
* The stomach and intestines. The gastrointestinal tract is part of the digestive system, which also includes the salivary glands, mouth, esophagus, liver, pancreas, gallbladder, and rectum.
gene (jeen)
* The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.
genetic (jeh-NEH-tik)
* Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
hereditary (huh-REH-dih-tayr-ee)
* Transmitted from parent to child by information contained in the genes.
imaging (IH-muh-jing)
* In medicine, a process that makes pictures of areas inside the body. Imaging uses methods such as x-rays (high-energy radiation), ultrasound (high-energy sound waves), and radio waves.
immunohistochemistry (IH-myoo-noh-HIS-toh-KEH-mih-stree)
* A technique used to identify specific molecules in different kinds of tissue. The tissue is treated with antibodies that bind the specific molecule. These are made visible under a microscope by using a color reaction, a radioisotope, colloidal gold, or a fluorescent dye. Immunohistochemistry is used to help diagnose diseases, such as cancer, and to detect the presence of microorganisms. It is also used in basic research to understand how cells grow and differentiate (become more specialized).
inherited (in-HAYR-ih-ted)
* Transmitted through genes that have been passed from parents to their offspring (children).
injection (in-JEK-shun)
* Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
laboratory test (LA-bruh-tor-ee...)
* A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.
large intestine (larj in-TES-tin)
* The long, tube-like organ that is connected to the small intestine at one end and the anus at the other. The large intestine has four parts: cecum, colon, rectum, and anal canal. Partly digested food moves through the cecum into the colon, where water and some nutrients and electrolytes are removed. The remaining material, solid waste called stool, moves through the colon, is stored in the rectum, and leaves the body through the anal canal and anus.
lens (lenz)
* A clear disk that focuses light, as in a camera or microscope. In the eye, the lens is a clear, curved structure at the front of the eye behind the pupil. It focuses light rays that enter the eye through the pupil, making an image on the retina (light-sensitive layers of nerve tissue at the back of the eye).
malignant (muh-LIG-nunt)
* Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.
microscope (MY-kroh-SKOPE)
* An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.
mineral (MIH-neh-rul)
* In medicine, a mineral is a nutrient that is needed in small amounts to keep the body healthy. Mineral nutrients include the elements calcium, magnesium, and iron.
mitotic rate (my-TAH-tik rayt)
* A measure of how fast cancer cells are dividing and growing. To find the mitotic rate, the number of cells dividing in a certain amount of cancer tissue is counted. Mitotic rate is used to help find the stage of melanoma (a type of skin cancer) and other types of cancer. Higher mitotic rates are linked with lower survival rates. Also called MR.
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.
mutation (myoo-TAY-shun)
* Any change in the DNA of a cell. Mutations may be caused by mistakes during cell division, or they may be caused by exposure to DNA-damaging agents in the environment. Mutations can be harmful, beneficial, or have no effect. If they occur in cells that make eggs or sperm, they can be inherited; if mutations occur in other types of cells, they are not inherited. Certain mutations may lead to cancer or other diseases.
neurofibromatosis type 1 (NOOR-oh-FY-broh-muh-TOH-sis ...)
* A rare genetic condition that causes brown spots and tumors on the skin, freckling in skin areas not exposed to the sun, tumors on the nerves, and developmental changes in the nervous system, muscles, bone, and skin. Also called NF1.
nutrient (NOO-tree-ent)
* A chemical compound (such as protein, fat, carbohydrate, vitamin, or mineral) contained in foods. These compounds are used by the body to function and grow.
organ (OR-gun)
* A part of the body that performs a specific function. For example, the heart is an organ.
pathologist (puh-THAH-loh-jist)
* A doctor who identifies diseases by studying cells and tissues under a microscope.
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.
protein (PROH-teen)
* A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.
radioisotope (RAY-dee-oh-I-suh-tope)
* An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.
recover (ree-KUH-ver)
* To become well and healthy again.
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.
scientist (SY-en-tist)
* A person who has studied science, especially one who is active in a particular field of investigation.
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.
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.
stool (stool)
* The material in a bowel movement. Stool is made up of undigested food, bacteria, mucus, and cells from the lining of the intestines. Also called feces.
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.
syndrome (SIN-drome)
* A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.
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.
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.
vitamin (VY-tuh-min)
* A nutrient that the body needs in small amounts to function and stay healthy. Sources of vitamins are plant and animal food products and dietary supplements. Some vitamins are made in the human body from food products. Vitamins are either fat-soluble (can dissolve in fats and oils) or water-soluble (can dissolve in water). Excess fat-soluble vitamins are stored in the body’s fatty tissue, but excess water-soluble vitamins are removed in the urine. Examples are vitamin A, vitamin C, and vitamin E.
vomit (VAH-mit)
* To eject some or all of the contents of the stomach through the mouth.
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.
Table of Links
1 http://www.cancer.gov/cancertopics/pdq/treatment/unusual-cancers-childhood/Patient/Page5#Section 437
Stages of Gastrointestinal Stromal Tumors
Key Points for This Section
* After a gastrointestinal stromal tumor has been diagnosed, tests are done to find out if cancer cells have spread within the gastrointestinal tract or to other parts of the body.
* There are three ways that cancer spreads in the body.
* The results of diagnostic and staging tests are used to plan treatment.
After a gastrointestinal stromal tumor has been diagnosed, tests are done to find out if cancer cells have spread within the gastrointestinal tract or to other parts of the body.
The process used to find out if cancer has spread within the gastrointestinal (GI) tract or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. The following tests and procedures may be used in the staging process:
* 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.
* 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.
* 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).
* Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
* Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
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 results of diagnostic and staging tests are used to plan treatment.
For many cancers it is important to know the stage of the cancer in order to plan treatment. However, the treatment of GIST is not based on the stage of the cancer. Treatment is based on whether the tumor can be removed by surgery and if the tumor has spread to other parts of the abdomen or to distant parts of the body.
Treatment is based on whether the tumor is:
* Resectable: These tumors can be removed by surgery .
* Unresectable: These tumors cannot be completely removed by surgery.
* Metastatic and recurrent: Metastatic tumors have spread to other parts of the body. Recurrent tumors have recurred (come back) after treatment. Recurrent GISTs may come back in the gastrointestinal tract or in other parts of the body. They are usually found in the abdomen, peritoneum, and/or liver.
* Refractory: These tumors have not gotten better with treatment.
Glossary Terms
abdomen (AB-doh-men)
* The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
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.
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).
bone scan (bone skan)
* A technique to create images of bones on a computer screen or on film. A small amount of radioactive material is injected into a blood vessel and travels through the bloodstream; it collects in the bones and is detected by a scanner.
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.
cell (sel)
* The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
chest x-ray (chest EX-ray)
* An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.
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.
gastrointestinal tract (GAS-troh-in-TES-tih-nul trakt)
* The stomach and intestines. The gastrointestinal tract is part of the digestive system, which also includes the salivary glands, mouth, esophagus, liver, pancreas, gallbladder, and rectum.
GIST
* A type of tumor that usually begins in cells in the wall of the gastrointestinal tract. It can be benign or malignant. Also called gastrointestinal stromal tumor.
glucose (GLOO-kose)
* A type of sugar; the chief source of energy for living organisms.
injection (in-JEK-shun)
* Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
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.
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 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.
malignant (muh-LIG-nunt)
* Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts 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.
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.
peritoneum (PAYR-ih-toh-NEE-um)
* The tissue that lines the abdominal wall and covers most of the organs in the abdomen.
PET scan (… skan)
* A procedure in which a small amount of radioactive glucose (sugar) is injected into a vein, and a scanner is used to make detailed, computerized pictures of areas inside the body where the glucose is used. Because cancer cells often use more glucose than normal cells, the pictures can be used to find cancer cells in the body. Also called positron emission tomography scan.
primary tumor (PRY-mayr-ee TOO-mer)
* The original tumor.
radioactive (RAY-dee-oh-AK-tiv)
* Giving off radiation.
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.
refractory (reh-FRAK-tor-ee)
* In medicine, describes a disease or condition that does not respond to treatment.
resectable (ree-SEK-tuh-bul)
* Able to be removed by surgery.
scanner (SKA-ner)
* In medicine, an instrument that takes pictures of the inside of the body.
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.
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.
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.
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.
Treatment Option Overview
Key Points for This Section
* There are different types of treatment for patients with gastrointestinal stromal tumors.
* Four types of standard treatment are used:
* Surgery
* Targeted therapy
* Watchful waiting
* Supportive care
* New types of treatment are being tested in clinical trials.
* 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 gastrointestinal stromal tumors.
Different types of treatments are available for patients with gastrointestinal stromal tumors (GISTs). 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.
Four types of standard treatment are used:
Surgery
If the GIST has not spread and is in a place where surgery can be safely done, the tumor and some of the tissue around it may be removed. Sometimes surgery is done using a laparoscope (a thin, lighted tube) to see inside the body. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope is inserted into one of the incisions. Instruments may be inserted through the same incision or through other incisions to remove organs or tissues.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells.
Tyrosine kinase inhibitors (TKIs) are targeted therapy drugs that block signals needed for tumors to grow. TKIs may be used to treat GISTs that cannot be removed by surgery or to shrink GISTs so they become small enough to be removed by surgery. Imatinib mesylate and sunitinib are two TKIs used to treat GISTs. TKIs are sometimes given for as long as the tumor does not grow and serious side effects do not occur.
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change.
Supportive care
If a GIST gets worse during treatment or there are side effects, supportive care is usually given. The goal of supportive care is to prevent or treat the symptoms of a disease, side effects caused by treatment, and psychological, social, and spiritual problems related to a disease or its treatment. Supportive care helps improve the quality of life of patients who have a serious or life-threatening disease. Radiation therapy is sometimes given as supportive care to relieve pain in patients with large tumors that have spread.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site.
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.
Follow-up for GISTs that were removed by surgery may include CT scan of the liver and pelvis or watchful waiting. For GISTs that are treated with tyrosine kinase inhibitors, follow-up tests, such as CT, MRI, or PET scans, may be done to check how well the targeted therapy is working.
Treatment Options for Gastrointestinal Stromal Tumors
Resectable Gastrointestinal Stromal Tumors
Resectable gastrointestinal stromal tumors (GISTs) can be completely or almost completely removed by surgery. Treatment may include the following:
* Surgery to remove tumors that are 2 centimeters or larger. Laparoscopic surgery may be done if the tumor is 5 cm or smaller. If there are cancer cells remaining at the edges of the area where the tumor was removed, watchful waiting or targeted therapy with imatinib mesylate may follow.
* A clinical trial of targeted therapy with imatinib mesylate following surgery, to decrease the chance the tumor will recur (come back).
Unresectable Gastrointestinal Stromal Tumors
Unresectable GISTs cannot be completely removed by surgery because they are too large or in a place where there would be too much damage to nearby organs if the tumor is removed. Treatment is usually a clinical trial of targeted therapy with imatinib mesylate to shrink the tumor, followed by surgery to remove as much of the tumor as possible.
Metastatic and Recurrent Gastrointestinal Stromal Tumors
Treatment of GISTs that are metastatic (spread to other parts of the body) or recurrent (came back after treatment) may include the following:
* Targeted therapy with imatinib mesylate.
* Targeted therapy with sunitinib, if the tumor begins to grow during imatinib mesylate therapy or if the side effects are too bad.
* Surgery to remove tumors that have been treated with targeted therapy and are shrinking, stable (not changing), or that have slightly increased in size. Targeted therapy may continue after surgery.
* Surgery to remove tumors when there are serious complications, such as bleeding, a hole in the gastrointestinal (GI) tract, a blocked GI tract, or infection.
* A clinical trial of a new treatment.
Refractory Gastrointestinal Stromal Tumors
Many GISTs treated with a tyrosine kinase inhibitor (TKI) become refractory (stop responding) to the drug after a while. Treatment is usually a clinical trial with a different TKI or a clinical trial of a new drug.
Treatment Options in Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with gastrointestinal stromal tumor. 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.
* * * * * * * * * * * *
Chapter 2A: Gastrointestinal Stromal Tumors (GIST) Health Professional Information
Gastrointestinal Stromal Tumors Treatment
General Information About Gastrointestinal Stromal Tumors Treatment
Epidemiology
Although they comprise fewer than 1% of all gastrointestinal (GI) tumors, gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the GI tract.[1] It has been estimated that there are 3,300 to 6,000 new GIST cases per year in the United States.[2] A study based on Surveillance, Epidemiology and End Results (SEER) registry data found that the age-adjusted yearly incidence of GIST in the United States was 6.8 per million from 1992 to 2000.[3] However, the true incidence is not known, in part because many tumors have not been tested for the characteristic KIT or platelet-derived growth factor receptor alpha (PDGFRA) gene mutations. In addition, small, indolent GIST, only a few millimeters in diameter, are common in the general population and are not included in cancer registries.[4,5] GIST are equally distributed across all geographic and ethnic groups and men and women are equally affected. Most patients present between the ages of 50 and 80.[6] The vast majority of GIST are sporadic, but there are rare familial forms associated with the characteristic heritable mutations in the KIT gene (or, rarely, in succinate dehydrogenase genes in Carney-Stratakis syndrome). Familial GIST may present as multiple primary tumors.
Clinical Presentation and Diagnostic Evaluation
GIST can occur anywhere along the GI tract, but most often are found in the stomach or small intestine. The American Joint Committee on Cancer (AJCC) Cancer Staging Manual lists the following approximate distributions:[7]
• Stomach (60%).
• Small intestine (30%).
• Rectum (3%).
• Colon (1-2%).
• Esophagus (<1%).
• Omentum/mesentery (rare).
Less frequently, GIST may arise in the appendix, gallbladder, pancreas, retroperitoneum, and paravaginal and periprostatic tissues.[8] Approximately 20% to 25% of gastric GIST and 40% to 50% of small intestinal GIST are clinically aggressive.[9,10] It has been estimated that approximately 10% to 25% of patients present with metastatic disease.[9,11]
The clinical presentation of patients with GIST varies depending on the anatomic location of the tumor and the tumor size and aggressiveness.[12J The most common presentation of GIST is GI bleeding, which may be acute (melena or hematemesis) or chronic and results in anemia.[10]
GIST patients may also present with:
• An acute abdomen caused by tumor rupture.
• GI obstruction.
• Appendicitis-like pain.
Other clinical symptoms include the following:[2]
• Fatigue.
• Dysphagia.
• Satiety.
Smaller lesions may be incidental findings during surgery, radiologic studies, or endoscopy. The natural history of these incidental tumors and the frequency of progression to symptomatic disease are unknown. There may be a substantial reservoir of small GIST tumors that do not progress to symptomatic stages. For example, a series of 98 consecutive systematic autopsies on adults who died of unrelated causes revealed grossly recognizable gastric tumors (1 mm-6 mm) that were histologically diagnosed as GIST in 22.5% of cases.[5] Sufficient DNA was available for analysis in 26 patients, revealing 13 patients with mutations in KIT exon 11 and one in PDGFRA.