Key Points for This Section

There are different types of treatment for patients with islet cell tumors. Five types of standard treatment are used: Surgery Chemotherapy Hormone therapy Hepatic arterial occlusion or chemoembolization 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 islet cell tumors.

Different types of treatments are available for patients with islet cell tumors. 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.

Five types of standard treatment are used:

Surgery

An operation may be done to remove the tumor. One of the following types of surgery may be used:

• Enucleation: Surgery to remove the tumor only. This may be done when cancer occurs in one place in the pancreas.
• Whipple procedure: A surgical procedure in which the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct may be removed. Enough of the pancreas is left to make digestive juices and insulin. The organs removed during this procedure depend on the patient's condition.
• Distal pancreatectomy: Surgery to remove the body and tail of the pancreas. The spleen may also be removed.
• Total gastrectomy: Surgery to remove the whole stomach.
• Parietal cell vagotomy: Surgery to cut the nerve that causes stomach cells to make acid.
• Liver resection: Surgery to remove part or all of the liver.
• Radiofrequency ablation: The use of a special probe with tiny electrodes that kill cancer cells. Sometimes the probe is inserted directly through the skin and only local anesthesia is needed. In other cases, the probe is inserted through an incision in the abdomen. This is done in the hospital with general anesthesia.
• Cryosurgical ablation: A procedure in which tissue is frozen to destroy abnormal cells. This is usually done with a special instrument that contains liquid nitrogen or liquid carbon dioxide. The instrument may be used during surgery or laparoscopy or inserted through the skin. This procedure is also called cryoablation.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated.

Hormone therapy

Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy are used to reduce the production of hormones or block them from working.

Hepatic arterial occlusion or chemoembolization

Hepatic arterial occlusion uses drugs, small particles, or other agents to block or reduce the flow of blood to the liver through the hepatic artery (the major blood vessel that carries blood to the liver). This is done to kill cancer cells growing in the liver. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine.

Chemotherapy delivered during hepatic arterial occlusion is called chemoembolization. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with the substance that blocks the artery and cuts off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body.

The blockage may be temporary or permanent, depending on the substance used to block the artery.

Supportive care

Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for islet cell cancer may include treatment for the following:

• Stomach ulcers may be treated with drug therapy such as:
  • Proton-pump inhibitor drugs such as omeprazole, lansoprazole, or pantoprazole.
  • Histamine blocking drugs such as cimetidine, ranitidine, or famotidine.
  • Somatostatin-type drugs such as octreotide.
• Diarrhea may be treated with:
  • Intravenous (IV) fluids with electrolytes such as potassium or chloride.
  • Somatostatin-type drugs such as octreotide.
• Low blood sugar may be treated by having small, frequent meals or with drug therapy to maintain a normal blood sugar level.
• High blood sugar may be treated with drugs taken by mouth or insulin by injection.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI Web site ¹.

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

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 cancer, premalignant (likely to become cancer), or benign (not cancer).

A chemical that gives off hydrogen ions in water and forms salts by combining with certain metals. Acids have a sour taste and turn certain dyes red. Some acids made by the body, such as gastric acid, can help the body work the way it should. An example of an acid is hydrochloric acid.

A blood vessel that carries blood from the heart to tissues and organs in the body.

A tube through which bile passes in and out of the liver.

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 tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.

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.

A colorless, odorless gas. It is a waste product made by the body. Carbon dioxide travels in the blood from the body’s tissues to the lungs. Breathing out clears carbon dioxide from the lungs.

A flexible tube used to deliver fluids into or withdraw fluids from the body.

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.

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

A procedure in which the blood supply to the tumor is blocked surgically or mechanically and anticancer drugs are administered directly into the tumor. This permits a higher concentration of drug to be in contact with the tumor for a longer period of time.

Treatment with drugs that kill cancer cells.

A drug usually used to treat stomach ulcers and heartburn. It is also commonly used in a regimen to prevent allergic reactions.

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.

Treatment using more than one anticancer drug.

A procedure in which tissue is frozen to destroy abnormal cells. This is usually done with a special instrument that contains liquid nitrogen or liquid carbon dioxide. Also called cryosurgery and cryosurgical ablation.

The process of identifying a disease, such as cancer, from its signs and symptoms.

Frequent and watery bowel movements.

The process of breaking down food into substances the body can use for energy, tissue growth, and repair.

Removal of the body and tail of the pancreas.

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.

In medicine, the removal of an organ or tumor in such a way that it comes out clean and whole, like a nut from its shell.

A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.

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.

The pear-shaped organ found below the liver. Bile is concentrated and stored in the gallbladder.

An operation to remove all or part of the stomach.

A temporary loss of feeling and a complete loss of awareness that feels like a very deep sleep. It is caused by special drugs or other substances called anesthetics. General anesthesia keeps patients from feeling pain during surgery or other procedures.

An organ that makes one or more substances, such as hormones, digestive juices, sweat, tears, saliva, or milk. Endocrine glands release the substances directly into the bloodstream. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.

A block in blood flow to the liver. It can happen while giving chemotherapy through a catheter in the hepatic artery. Sometimes doctors use drugs or other agents to cause hepatic arterial occlusion on purpose. This block of blood flow to the liver helps kill cancer cells growing in the liver.

The major blood vessel that carries blood to the liver.

A blood vessel that carries blood to the liver from the stomach, small and large intestines, spleen, pancreas, and gallbladder. Also called portal vein.

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

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 endocrine therapy, hormonal therapy, and hormone treatment.

A cut made in the body to perform surgery.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

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.

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.

Into or within a vein. Intravenous usually refers to a way of giving a drug or other substance through a needle or tube inserted into a vein. Also called IV.

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 mass of abnormal cells that forms in the endocrine (hormone-producing) tissues of the pancreas. Islet cell tumors may be benign (not cancer) or malignant (cancer).

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.

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

A temporary loss of feeling in one small area of the body caused by special drugs or other substances called anesthetics. The patient stays awake but has no feeling in the area of the body treated with the anesthetic.

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, 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 chemical compound (such as protein, fat, carbohydrate, vitamin, or mineral) contained in foods. These compounds are used by the body to function and grow.

A drug similar to the naturally occurring growth hormone inhibitor somatostatin. Octreotide is used to treat diarrhea and flushing associated with certain types of tumors.

A drug that inhibits gastric acid secretion.

A part of the body that performs a specific function. For example, the heart is an organ.

A colorless, odorless gas. It is needed for animal and plant life. Oxygen that is breathed in enters the blood from the lungs and travels to the tissues.

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 metallic element that is important in body functions such as regulation of blood pressure and of water content in cells, transmission of nerve impulses, digestion, muscle contraction, and heartbeat.

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.

A procedure that uses radio waves to heat and destroy abnormal cells. The radio waves travel through electrodes (small devices that carry electricity). Radiofrequency ablation may be used to treat cancer and other conditions.

A molecule inside or on the surface of a cell that binds to a specific substance and causes a specific physiologic effect in the cell.

To come back or to return.

Treatment with anticancer drugs directed to a specific area of the body.

Surgery to remove tissue or part or all of an organ.

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 bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spinal column encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spine, and vertebral column.

An organ that is part of the lymphatic system. The spleen makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach.

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.

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 best practice and standard of care.

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.

Care given to improve the quality of life of patients who have a serious or life-threatening disease. The goal of supportive care is to prevent or treat as early as possible the symptoms of a disease, side effects caused by treatment of a disease, and psychological, social, and spiritual problems related to a disease or its treatment. Also called comfort care, palliative care, and symptom management.

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

Treatment with anticancer drugs that travel through the blood to cells all over the body.

Treatment.

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 cancer), or malignant (cancer). 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.

A blood vessel that carries blood to the heart from tissues and organs in the body.

A type of surgery used to treat pancreatic cancer. The head of the pancreas, the duodenum, a portion of the stomach, and other nearby tissues are removed.