Description

What are islet cell tumors?

Stage Explanation

Stages of islet cell cancer Gastrinoma Insulinoma Glucagonoma Miscellaneous Recurrent

Treatment Option Overview

How islet cell cancer is treated Treatment in a clinical trial

Gastrinoma
Insulinoma
Glucagonoma
Miscellaneous Islet Cell Tumors
Recurrent Islet Cell Tumors
To Learn More About Islet Cell Tumors (Endocrine Pancreas)
Get More Information From NCI
Changes to This Summary (05/06/2008)
About PDQ

Description

What are islet cell tumors?

An islet cell tumor is a mass of abnormal cells that forms in the endocrine (hormone -producing) tissues of the pancreas. Islet cell tumors may be benign (noncancer) or malignant (cancerous). Islet cell cancer is rare.

The pancreas is about 6 inches long and is shaped like a thin pear, wider at one end and narrower at the other. The pancreas lies behind the stomach, inside a loop formed by part of the small intestine. The broader right end of the pancreas is called the head, the middle section is called the body, and the narrow left end is the tail.

The pancreas has two basic jobs in the body. It produces digestive juices that help break down (digest) food, and hormones (such as insulin) that regulate how the body stores and uses food. The area of the pancreas that produces digestive juices is called the exocrine pancreas. About 95% of pancreatic cancers begin in the exocrine pancreas. The hormone-producing area of the pancreas has special cells called islet cells and is called the endocrine pancreas. Only about 5% of pancreatic cancers start here. This summary has information on cancer of the endocrine pancreas (islet cell cancer). (See the PDQ summary on Pancreatic Cancer Treatment ¹ for more information on cancer of the exocrine pancreas.)

The islet cells in the pancreas make many hormones, including insulin, which help the body store and use sugars. When islet cells in the pancreas become cancerous, they may make too many hormones. Islet cell cancers that make too many hormones are called functioning tumors. Other islet cell cancers may not make extra hormones and are called nonfunctioning tumors. Tumors that do not spread to other parts of the body can also be found in the islet cells. These are called benign tumors and are not cancer. A doctor will need to determine whether the tumor is cancer or a benign tumor.

A doctor should be seen if there is pain in the abdomen, diarrhea, stomach pain, a tired feeling all the time, fainting, or weight gain without eating too much.

If there are symptoms, the doctor will order blood and urine tests to see whether the amounts of hormones in the body are normal. Other tests, including x-rays and special scans, may also be done.

The chance of recovery (prognosis) depends on the type of islet cell cancer the patient has, how far the cancer has spread, and the patient’s overall health.

Stage Explanation

Stages of islet cell cancer

Once islet cell cancer is found, more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. The staging system for islet cell cancer is still being developed. These tumors are most often divided into one of three groups:

1. islet cell cancers occurring in one site within the pancreas,
2. islet cell cancers occurring in several sites within the pancreas, or
3. islet cell cancers that have spread to lymph nodes near the pancreas or to distant sites.

Treatment of islet cell cancer depends on the type of tumor, the stage, and the patient’s general health. The following types of islet cell tumors are found:

The tumor makes large amounts of a hormone called gastrin, which causes too much acid to be made in the stomach. Ulcers may develop as a result of too much stomach acid.

The tumor makes too much of the hormone insulin and causes the body to store sugar instead of burning the sugar for energy. This causes too little sugar in the blood, a condition called hypoglycemia.

This tumor makes too much of the hormone glucagon and causes too much sugar in the blood, a condition called hyperglycemia.

Other types of islet cell cancer can affect the pancreas and/or small intestine. Each type of tumor may affect different hormones in the body and cause different symptoms.

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the pancreas or in another part of the body.

Treatment Option Overview

How islet cell cancer is treated

Different types of treatment are available for patients with islet cell 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 (taking out the cancer).
• Chemotherapy (using drugs to kill cancer cells).
• Hormone therapy (using hormones to stop cancer cells from growing).

Surgery is the most common treatment of islet cell cancer. The doctor may take out the cancer and most or part of the pancreas. Sometimes the stomach is taken out (gastrectomy) because of ulcers. Lymph nodes in the area may also be removed and looked at under a microscope to see if they contain cancer.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in the vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body.

Hormone therapy uses hormones to stop the cancer cells from growing or to relieve symptoms caused by the tumor.

Hepatic arterial occlusion or embolization uses drugs or other agents to reduce or block the flow of blood to the liver in order to kill cancer cells growing in the liver.

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.

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. In the following lists of treatments for the different types of islet cell tumors, a link to search results for current clinical trials is included for each section. These have been retrieved from NCI's clinical trials database. For some types of islet cell tumors, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Gastrinoma

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Surgery to remove the stomach (gastrectomy).
3. Surgery to cut the nerve that stimulates the pancreas.
4. Chemotherapy.
5. Hormone therapy.
6. Hepatic arterial occlusion or embolization to kill cancer cells growing in the liver.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with gastrinoma ².

Insulinoma

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Chemotherapy.
3. Hormone therapy.
4. Drugs to relieve symptoms.
5. Hepatic arterial occlusion or embolization to kill cancer cells growing in the liver.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with insulinoma ³.

Glucagonoma

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Chemotherapy.
3. Hormone therapy.
4. Hepatic arterial occlusion or embolization to kill cancer cells growing in the liver.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with glucagonoma ⁴.

Miscellaneous Islet Cell Tumors

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Chemotherapy.
3. Hormone therapy.
4. Hepatic arterial occlusion or embolization to kill cancer cells growing in the liver.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with islet cell tumor ⁵.

Recurrent Islet Cell Tumors

Treatment depends on many factors, including what treatment the patient had before and where the cancer has come back. Treatment may be chemotherapy, or patients may want to consider taking part in a clinical trial.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent islet cell carcinoma ⁶.

To Learn More About Islet Cell Tumors (Endocrine Pancreas)

For more information from the National Cancer Institute about islet cell tumors (endocrine pancreas), see the Islet Cell Tumors Home Page ⁷.

For general cancer information and other resources from the National Cancer Institute, see the following:

• What You Need to Know About™ Cancer - An Overview ⁸
• Understanding Cancer Series: Cancer ⁹
• Staging: Questions and Answers ¹⁰
• Chemotherapy and You: Support for People With Cancer ¹¹
• Radiation Therapy and You: Support for People With Cancer ¹²
• Coping with Cancer ¹³
• Support and Resources ¹⁴
• Cancer Library ¹⁵
• Information For Survivors/Caregivers/Advocates ¹⁶

Get More Information From NCI

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

Chat online

The NCI's LiveHelp® ¹⁷ online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write to us

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

NCI Public Inquiries Office

Suite 3036A

6116 Executive Boulevard, MSC8322

Bethesda, MD 20892-8322

Search the NCI Web site

The NCI Web site ¹⁸ provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator ¹⁹. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

Changes to This Summary (05/06/2008)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:

• Information about patients taking part in clinical trials was added to the Treatment Option Overview ²⁰ section.
• Links to ongoing clinical trials listed in NCI’s PDQ Cancer Clinical Trials Registry were added to the treatment sections.
• A new section called “ To Learn More ²¹ ” was added. It includes links to more information about this type of cancer and about cancer in general.
• The “Get More Information from NCI” section (originally called “To Learn More”) was revised.

About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site ¹⁸. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." 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.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site ²². Descriptions of the trials are available in health professional and patient versions. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Not normal. An abnormal lesion or growth may be cancerous, premalignant (likely to become cancer), or benign.

The blocking of an artery by a clot of foreign material. This can be done as treatment to block the flow of blood to a tumor.

Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body.

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.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is 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.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

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 a clinical study.

Frequent and watery bowel movements.

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.

Refers to tissue that makes and releases hormones that travel in the bloodstream and control the actions of other cells or organs. Some examples of endocrine tissues are the pituitary, thyroid, and adrenal glands.

A pancreatic cell that produces hormones (e.g., insulin and glucagon) that are secreted into the bloodstream. These hormones help control the level of glucose (sugar) in the blood. Also called islet cell and islet of Langerhans cell.

A pancreatic cell that produces enzymes that are secreted into the small intestine. These enzymes help digest food as it passes through the gastrointestinal tract.

A tumor that is found in endocrine tissue and makes hormones (chemicals that travel in the bloodstream and control the actions of other cells or organs).

An operation to remove all or part of the stomach.

A hormone produced by the pancreas that increases the level of glucose (sugar) in the blood.

The major blood vessel that carries blood to the liver.

One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.

Treatment that adds, blocks, or removes hormones. For certain conditions (such as diabetes or menopause), hormones are given to adjust low hormone levels. To slow or stop the growth of certain cancers (such as prostate and breast cancer), synthetic hormones or other drugs may be given to block the body’s natural hormones. Sometimes surgery is needed to remove the gland that makes a certain hormone. Also called hormonal therapy, hormone treatment, or endocrine therapy.

Higher than normal amount of glucose (a type of sugar) in the blood. Hyperglycemia can be a sign of diabetes or other conditions. Also called high blood sugar.

Abnormally low blood sugar.

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.

A pancreatic cell that produces hormones (e.g., insulin and glucagon) that are secreted into the bloodstream. These hormones help control the level of glucose (sugar) in the blood. Also called endocrine pancreas cell and islet of Langerhans cell.

A rare cancer that forms in the islets of Langerhans cells (a type of cell found in the pancreas). Also called pancreatic endocrine cancer.

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

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.

Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.

The National Cancer Institute, 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. The National Cancer Institute 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 National Cancer Institute Web site at http://www.cancer.gov. Also called 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.

A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.

A tumor that is found in endocrine tissue but that does not make extra hormones (chemicals that travel in the blood and control the actions of other cells or organs).

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.

A disease in which malignant (cancer) cells are found in the tissues of the pancreas. Also called exocrine cancer.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

The likely outcome or course of a disease; the chance of recovery or recurrence.

To come back or to return.

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.

A picture of structures inside the body. Scans often used in diagnosing, staging, and monitoring disease include liver scans, bone scans, and computed tomography (CT) or computerized axial tomography (CAT) scans and magnetic resonance imaging (MRI) scans. In liver scanning and bone scanning, radioactive substances that are injected into the bloodstream collect in these organs. A scanner that detects the radiation is used to create pictures. In CT scanning, an x-ray machine linked to a computer is used to produce detailed pictures of organs inside the body. MRI scans use a large magnet connected to a computer to create pictures of areas inside the body.

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.

The part of the digestive tract that is located between the stomach and the large intestine.

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.

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.

In medicine, treatment that experts agree is appropriate, accepted, and widely used. Health care providers are obligated to provide patients with standard therapy. Also called standard of care or best practice.

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.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

Treatment using substances that travel through the bloodstream, reaching and affecting cells all over the body.

A group or layer of cells that work together to perform a specific function.

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 cancerous), or malignant (cancerous). Also called neoplasm.

The formation of a break on the skin or on the surface of an organ. An ulcer forms when the surface cells die and are cast off. Ulcers may be associated with cancer and other diseases.

Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.

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.