Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ�)

General Information About Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)

Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas.

The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine.

There are two kinds of cells in the pancreas:

• Endocrine pancreas cells make several kinds of hormones (chemicals that control the actions of certain cells or organs in the body), such as insulin to control blood sugar. They cluster together in many small groups (islets) throughout the pancreas. Endocrine pancreas cells are also called islet cells or islets of Langerhans.

• Exocrine pancreas cells make enzymes that are released into the small intestine to help the body digest food. Most of the pancreas is made of ducts with small sacs at the end of the ducts, which are lined with exocrine cells. This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment for information on exocrine pancreatic cancer.

A pancreatic NET may also be called a pancreatic endocrine tumor (PET), islet cell tumor, islet cell carcinoma, or pancreatic carcinoid.

Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis.

Pancreatic NETs may or may not cause symptoms.

Pancreatic NETs may be functional (the hormones that are released cause symptoms) or nonfunctional (the hormones that are released do not cause symptoms) tumors:

• Functional tumors make one or more hormones, such as gastrin, insulin, and glucagon, that cause symptoms. Most functional tumors are benign (not cancer).

• Nonfunctional tumors make substances that do not cause symptoms. Symptoms are caused by the tumor as it spreads and grows. Most nonfunctional tumors are malignant (cancer).

Most pancreatic NETs are functional tumors.

There are different kinds of functional pancreatic NETs.

Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following:

• Gastrinoma: A tumor that forms in cells that make gastrin. Gastrin is a hormone that causes the stomach to release an acid that helps digest food. Both gastrin and stomach acid are increased by gastrinomas. When increased stomach acid, stomach ulcers, and diarrhea are caused by a tumor that makes gastrin, it is called Zollinger-Ellison syndrome. A gastrinoma usually forms in the head of the pancreas and sometimes forms in the small intestine. Most gastrinomas are malignant (cancer).

• Insulinoma: A tumor that forms in cells that make insulin. Insulin is a hormone that controls the amount of glucose (sugar) in the blood. It moves glucose into the cells, where it can be used by the body for energy. Insulinomas are usually slow-growing tumors that rarely spread. An insulinoma forms in the head, body, or tail of the pancreas. Insulinomas are usually benign (not cancer).

• Glucagonoma: A tumor that forms in cells that make glucagon. Glucagon is a hormone that increases the amount of glucose in the blood. It causes the liver to break down glycogen. Too much glucagon causes hyperglycemia (high blood sugar). A glucagonoma usually forms in the tail of the pancreas. Most glucagonomas are malignant (cancer).

• Other types of tumors: There are other rare types of functional pancreatic NETs that make hormones, including hormones that control the balance of sugar, salt, and water in the body. These tumors include:

  • VIPomas, which make vasoactive intestinal peptide. VIPoma may also be called Verner-Morrison syndrome.

  • Somatostatinomas, which make somatostatin.

  These other types of tumors are grouped together because they are treated in much the same way.

Having certain syndromes can increase the risk of pancreatic NETs.

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.

Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs.

Different types of pancreatic NETs have different signs and symptoms.

Symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes. Some tumors may not cause symptoms. Conditions other than pancreatic NETs can cause the symptoms listed below. Talk to your doctor if any of these problems occur.

Signs and symptoms of a non-functioning pancreatic NET

A non-functioning pancreatic NET may grow for a long time without causing symptoms. It may grow large or spread to other parts of the body before it causes symptoms, such as:

• Diarrhea.

• Indigestion.

• A lump in the abdomen.

• Pain in the abdomen or back.

• Yellowing of the skin and whites of the eyes.

Signs and symptoms of a functioning pancreatic NET

The symptoms of a functioning pancreatic NET depend on the type of hormone being made.

Too much gastrin may cause:

• Stomach ulcers that keep coming back.

• Pain in the abdomen, which may spread to the back. The pain may come and go and it may go away after taking an antacid.

• The flow of stomach contents back into the esophagus (gastroesophageal reflux).

• Diarrhea.

Too much insulin may cause:

• Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.

• Feeling a fast heartbeat.

Too much glucagon may cause:

• Skin rash on the face, stomach, or legs.

• High blood sugar. This can cause headaches, frequent urination, dry skin and mouth, or feeling hungry, thirsty, tired, or weak.

• Blood clots in the lung. This can cause shortness of breath, cough, or pain in the chest. Blood clots in the arm or leg can cause pain, swelling, warmth, or redness of the arm or leg.

• Diarrhea.

• Weight loss for no known reason.

• Sore tongue or sores at the corners of the mouth.

Too much vasoactive intestinal peptide (VIP) may cause:

• Very large amounts of watery diarrhea.

• Dehydration. This can cause feeling thirsty, making less urine, dry skin and mouth, feeling tired, headache, or dizziness.

• Low potassium level in the blood. This can cause muscle weakness, aching, or cramps, numbness and tingling, frequent urination, and feeling a fast heartbeat, confused, or thirsty.

• Cramps or pain in the abdomen.

• Weight loss for no known reason.

Too much somatostatin may cause:

• High blood sugar. This can cause headaches, frequent urination, dry skin and mouth, or feeling hungry, thirsty, tired, or weak.

• Diarrhea.

• Steatorrhea (very foul-smelling stool that floats).

• Gallstones.

• Yellowing of the skin and whites of the eyes.

• Weight loss for no known reason.

Lab tests and imaging tests are used to detect (find) and diagnose pancreatic NETs.

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.

• Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as glucose (sugar), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.

• 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.

• AbdominalCT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, 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).

• Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactiveoctreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

• Abdominal ultrasound: An ultrasound exam used to make pictures of the inside of the abdomen. The ultrasound transducer is pressed against the skin of the abdomen and directs high-energy sound waves (ultrasound) into the abdomen. The sound waves bounce off the internal tissues and organs and make echoes. The transducer receives the echoes and sends them to a computer, which uses the echoes to make pictures called sonograms. The picture can be printed to be looked at later.

• Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with 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 tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.

• Angiogram: A procedure to look at blood vessels and the flow of blood. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages.

• Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.

• Intraoperative ultrasound: A procedure that uses high-energy sound waves (ultrasound) to create images of internal organs or tissues during surgery. A transducer placed directly on the organ or tissue is used to make the sound waves, which create echoes. The transducer receives the echoes and sends them to a computer, which uses the echoes to make pictures called sonograms.

• Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are several ways to do a biopsy for pancreatic NETs. Cells may be removed using a fine or wide needle inserted into the pancreas during an x-ray or ultrasound. Tissue may also be removed during a laparoscopy (a surgical incision made in the wall of the abdomen).

• 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 blood. The radioactive material collects in bones where cancer cells have spread and is detected by a scanner.

Other kinds of lab tests are used to check for the specific type of pancreatic NETs.

The following tests and procedures may be used:

Gastrinoma

• Fasting serum gastrin test: A test in which a blood sample is checked to measure the amount of gastrin in the blood. This test is done after the patient has had nothing to eat or drink for at least 8 hours. Conditions other than gastrinoma can cause an increase in the amount of gastrin in the blood.

• Gastric acid secretion test: A test to measure the amount of acid made by the stomach. A tube is inserted through the nose or throat, into the stomach. Gastrin or insulin is injected into the patient, which causes the stomach to make stomach secretions (gastric acid). Four samples of gastric acid are taken through the tube 15 minutes apart. These four samples are used to find out the lowest and highest amounts of gastric acid made during the test and the pH level of the gastric secretions.

• Secretin stimulation test: If the gastric acid secretion test result is not normal, a secretin stimulation test may be done. The tube is moved into the small intestine and samples are taken from the small intestine after a drug called secretin is injected. Secretin causes the small intestine to make acid. When there is a gastrinoma, the secretin causes an increase in how much gastric acid is made and the level of gastrin in the blood.

• Calcium infusion test: A test to measure the amount of gastrin in the blood after a drug called calcium gluconate is infused. Blood samples will be taken to measure the amount of gastrin in the blood at set times.

• Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

Insulinoma

• Fasting serum glucose and insulin test: A test in which a blood sample is checked to measure the amounts of glucose (sugar) and insulin in the blood. The test is done after the patient has had nothing to eat or drink for at least 24 hours.

• C-peptide suppression test: A test in which a blood sample is checked to measure the amount of C-peptide in the blood. Insulin is injected into a vein to lower the patient?s blood sugar. This should decrease the amount of insulin and C-peptide that the body releases into the blood. In patients who have insulinoma, the insulin and C-peptide levels do not drop because the tumor is also releasing insulin and C-peptide into the blood.

Glucagonoma

• Fasting serum glucagon test: A test in which a blood sample is checked to measure the amount of glucagon in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours.

Other tumor types

• VIPoma

  • Serum VIP (vasoactive intestinal peptide) test: A test in which a blood sample is checked to measure the amount of VIP.

  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. In VIPoma, there is a lower than normal amount of potassium.

  • Stool analysis: A stool sample is checked for a higher than normal sodium (salt) and potassium levels.

• Somatostatinoma

  • Fasting serum somatostatin test: A test in which a blood sample is checked to measure the amount of somatostatin in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours.

  • Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

Certain factors affect prognosis (chance of recovery) and treatment options.

Pancreatic NETs can often be cured. The prognosis (chance of recovery) and treatment options depend on the following:

• The type of cancer cell.

• Where the tumor is found in the pancreas.

• Whether the tumor has spread to more than one place in the pancreas or to other parts of the body.

• Whether the patient has MEN1 syndrome.

• The patient's age and general health.

• Whether the cancer has just been diagnosed or has recurred (come back).

Stages of Pancreatic Neuroendocrine Tumors

The plan for cancer treatment depends on where the NET is found in the pancreas and whether it has spread.

The process used to find out if cancer has spread within the pancreas or to other parts of the body is called staging. The results of the tests and procedures used to diagnose pancreatic neuroendocrine tumors (NETs) are also used to find out whether the cancer has spread. See the General Information section for a description of these tests and procedures.

Although there is a standard staging system for pancreatic NETs, it is not used to plan treatment. Treatment of pancreatic NETs is based on the following:

• Whether the cancer is found in one place in the pancreas.

• Whether the cancer is found in several places in the pancreas.

• Whether the cancer has spread to lymph nodes near the pancreas or to other parts of the body such as the liver, lung, peritoneum, or bone.

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.

Recurrent Pancreatic Neuroendocrine Tumors

Recurrent pancreatic neuroendocrine tumors (NETs) are tumors that have recurred (come back) after being treated. The tumors may come back in the pancreas or in other parts of the body.

Treatment Option Overview

There are different types of treatment for patients with pancreatic NETs.

Different types of treatments are available for patients with pancreatic neuroendocrine tumors (NETs). 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.

Six 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.

• Pancreatoduodenectomy: 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 are 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. This is also called the Whipple procedure.

• 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.

• Liverresection: 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.

• Cryosurgicalablation: 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 cerebrospinal fluid, 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 is 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.

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. Certain types of targeted therapies are being studied in the treatment of pancreatic NETs.

Supportive care

Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for pancreatic NETs 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.

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.

Treatment Options for Pancreatic Neuroendocrine Tumors

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Gastrinoma

Treatment of gastrinoma may include supportive care and the following:

• For symptoms caused by too much stomachacid, treatment may be a drug that decreases the amount of acid made by the stomach.

• For a single tumor in the head of the pancreas:

  • Surgery to remove the tumor.

  • Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid.

  • Surgery to remove the whole stomach (rare).

• For a single tumor in the body or tail of the pancreas, treatment is usually surgery to remove the body or tail of the pancreas.

• For several tumors in the pancreas, treatment is usually surgery to remove the body or tail of the pancreas. If tumor remains after surgery, treatment may include either:

  • Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid; or

  • Surgery to remove the whole stomach (rare).

• For one or more tumors in the duodenum (the part of the small intestine that connects to the stomach), treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct).

• If no tumor is found, treatment may include the following:

  • Surgery to cut the nerve that causes stomach cells to make acid and treatment with a drug that decreases stomach acid.

  • Surgery to remove the whole stomach (rare).

• If the cancer has spread to the liver, treatment may include:

  • Surgery to remove part or all of the liver.

  • Radiofrequency ablation or cryosurgicalablation.

  • Chemoembolization.

• If cancer has spread to other parts of the body or does not get better with surgery or drugs to decrease stomach acid, treatment may include:

  • Chemotherapy.

  • Hormone therapy.

• If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include:

  • Hepatic arterial occlusion, with or without systemic chemotherapy.

  • Chemoembolization, with or without systemic chemotherapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with gastrinoma. 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.

Insulinoma

Treatment of insulinoma may include the following:

• For one small tumor in the head or tail of the pancreas, treatment is usually surgery to remove the tumor.

• For one large tumor in the head of the pancreas that cannot be removed by surgery, treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct)

• For one large tumor in the body or tail of the pancreas, treatment is usually a distal pancreatectomy (surgery to remove the body and tail of the pancreas).

• For more than one tumor in the pancreas, treatment is usually surgery to remove any tumors in the head of the pancreas and the body and tail of the pancreas.

• For tumors that have spread to lymph nodes or other parts of the body, treatment may include the following:

  • Surgery to remove the tumor.

  • Radiofrequency ablation or cryosurgicalablation, if the tumor cannot be removed by surgery.

• For tumors that cannot be removed by surgery, treatment may include the following:

  • Combination chemotherapy.

  • Palliativedrugtherapy to decrease the amount of insulin made by the pancreas.

  • Hormone therapy.

  • Radiofrequency ablation or cryosurgical ablation.

• If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include:

  • Hepatic arterial occlusion, with or without systemic chemotherapy.

  • Chemoembolization, with or without systemic chemotherapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with insulinoma. 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.

Glucagonoma

Treatment may include the following:

• For one small tumor in the head or tail of the pancreas, treatment is usually surgery to remove the tumor.

• For one large tumor in the head of the pancreas that cannot be removed by surgery, treatment is usually pancreatoduodenectomy (surgery to remove the head of the pancreas, the gallbladder, nearby lymph nodes and part of the stomach, small intestine, and bile duct).

• For more than one tumor in the pancreas, treatment is usually surgery to remove the tumor or surgery to remove the body and tail of the pancreas.

• For tumors that have spread to lymph nodes or other parts of the body, treatment may include the following:

  • Surgery to remove the tumor.

  • Radiofrequency ablation or cryosurgicalablation, if the tumor cannot be removed by surgery.

• For tumors that cannot be removed by surgery, treatment may include the following:

  • Combination chemotherapy.

  • Hormone therapy.

  • Radiofrequency ablation or cryosurgical ablation.

• If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may include:

  • Hepatic arterial occlusion, with or without systemic chemotherapy.

  • Chemoembolization, with or without systemic chemotherapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with glucagonoma. 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.

Other Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)

For VIPoma, treatment may include the following:

• Fluids and hormone therapy to replace fluids and electrolytes that have been lost from the body.

• Surgery to remove the tumor and nearby lymph nodes.

• Surgery to remove as much of the tumor as possible when the tumor cannot be completely removed or has spread to distant parts of the body. This is palliative therapy to relieve symptoms and improve the quality of life.

• For tumors that have spread to lymph nodes or other parts of the body, treatment may include the following:

  • Surgery to remove the tumor.

  • Radiofrequency ablation or cryosurgicalablation, if the tumor cannot be removed by surgery.

• For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following:

  • Chemotherapy.

  • Targeted therapy.

For somatostatinoma, treatment may include the following:

• Surgery to remove the tumor.

• For tumors that have spread to distant parts of the body, surgery to remove as much of the tumor as possible to relieve symptoms and improve quality of life.

• For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following:

  • Chemotherapy.

  • Targeted therapy.

Treatment of other types of pancreatic neuroendocrine tumors (NETs) may include the following:

• Surgery to remove the tumor.

• For tumors that have spread to distant parts of the body, surgery to remove as much of the tumor as possible or hormone therapy to relieve symptoms and improve quality of life.

• For tumors that continue to grow during treatment or have spread to other parts of the body, treatment may include the following:

  • Chemotherapy.

  • Targeted therapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with islet cell 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.

Recurrent or Progressive Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)

Treatment of pancreatic neuroendocrine tumors (NETs) that continue to grow during treatment or recur (come back) may include the following:

• Surgery to remove the tumor.

• Chemotherapy.

• Hormone therapy.

• Targeted therapy.

• For liver metastases:

  • Regional chemotherapy.

  • Hepatic arterial occlusion or chemoembolization, with or without systemic chemotherapy.

• A clinical trial of a new therapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent islet cell carcinoma. 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.

To Learn More About Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)

For more information from the National Cancer Institute about pancreatic neuroendocrine tumors (NETs), see the following:

• Pancreatic Cancer Home Page

• Understanding Cancer Series: Targeted Therapies (Advances in Targeted Therapies)

• Targeted Cancer Therapies

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

• What You Need to Know About? Cancer

• Understanding Cancer Series: Cancer

• Cancer Staging

• Chemotherapy and You: Support for People With Cancer

• Radiation Therapy and You: Support for People With Cancer

• Coping with Cancer: Supportive and Palliative Care

• Cancer Library

• Information For Survivors/Caregivers/Advocates

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 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.

Chat online

The NCI's LiveHelp� online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 8:00 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 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 the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.

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. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).

Changes to This Summary (12/16/2011)

The PDQcancer 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.

Editorial changes were made to this summary.

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. 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. 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).