Islet Cell Tumors

1 What are Islet Cell Tumors?

Islet cell tumors are abnormal cells that form in the tissues 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. Malignant islet cell tumors are a rare type of pancreatic cancer.

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.

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

Islet cell tumors may or may not cause symptoms.

Islet cells make and release hormones into the blood. Islet cell tumors may be functional (the hormones that are released cause symptoms) or nonfunctional (the hormones that are released do not cause symptoms) tumors:

Functional 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

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

There are different kinds of functional islet cell tumors

Islet cells make different kinds of hormones such as gastrin, insulin, and glucagon. Types of functional islet cell tumors 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).

Glucagonomas are often malignant. 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 islet cell tumors 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 developing islet cell tumors.

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 islet cell tumors.

2 Symptoms

People with an islet cell tumor may experience the following symptoms or signs.

Sometimes, people with an islet cell tumor do not show any of these symptoms, or these symptoms may be caused by a medical condition that is not a tumor.

Symptoms of a gastrinoma

  • Ulcers, caused by too much stomach acid
  • Diarrhea

Symptoms of an insulinoma

Symptoms of a glucagonoma

  • Hyperglycemia, which causes frequent urination, increased thirst, and increased hunger
  • Rash that spreads on the face, abdomen, or lower extremities

Symptoms of a VIPoma

  • Watery diarrhea
  • Too little potassium in the blood, which can cause an irregular heartbeat, muscle cramping and weakness, and decreased reflexes
  • Too little acid in the stomach, which can cause digestive problems and poor absorption of vitamins and nutrients
  • Flushing or redness of the face, neck, or chest
  • Fatigue
  • Nausea

Symptoms of a somatostatinoma

  • Type 2 diabetes
  • Gallstones
  • Steatorrhea, a condition where the body cannot absorb fat, causing oily and loose stools with a particularly bad odor
  • Diarrhea
  • Weight loss
  • Too little hydrochloric acid in the stomach, which can cause digestive problems and poor absorption of vitamins and nutrients

Other symptoms

  • Abdominal pain
  • Jaundice, which is yellowing of the skin and whites of the eyes
  • Vomiting blood
  • Sweating
  • Rapid heart rate
  • Anxiety
  • Headache
  • Convulsions
  • Loss of consciousness
  • Clouding of vision
  • Unexplained weight gain or loss
  • Inflamed mouth and tongue
  • A mass or lump in the abdomen

If you are concerned about one or more of the symptoms or signs on this list, please talk with your doctor. Your doctor will ask how long and how often you’ve been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If a tumor is diagnosed, relieving symptoms remains an important part of your care. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

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3 Causes

There is no specified cause of islet cell tumors.

Many of the risk factors that have been identified for pancreatic cancer (ductal adenocarcinomas of the pancreas) are not risk factors for islet cell tumors / pancreatic neuroendocrine neoplasms. For example, cigarette smoking doubles the risk of pancreatic cancer, but it does not appear to be a factor in the development of islet cell tumors / pancreatic endocrine neoplasms.

Several genetic (familial) syndromes predispose to pancreatic neuroendocrine neoplasms. These are important to recognize for three reasons.

First, because these genetic syndromes are caused by genetic changes that can be inherited, other family members may be at risk.

Second, these genetic syndromes predispose to (increase the risk of) more than one tumor type. Individuals with one of these syndromes therefore have an increased risk of developing pancreatic and extra-pancreatic neuroendocrine tumors.

Finally, these syndromes are important because they provide insight into the biology of pancreatic neuroendocrine neoplasms. Because the genes that cause these genetic syndromes are known, scientists can understand the cellular pathways which lead to the development of pancreatic neuroendocrine neoplasms. The hope is that a better understanding of these pathways will lead to better treatments.

Multiple Endocrine Neoplasia Type 1, abbreviated MEN-1, is a familial syndrome caused by inherited mutations in the MEN1 gene on chromosome 11.

The MEN-1 gene codes for the menin protein, and patients with an inherited mutation in the MEN1 are predisposed to develop tumors of the pituitary (the small "master" gland at the base of the brain), the parathyroids (four small glands in neck which help control blood calcium levels), and the pancreas.

Von Hippel-Lindau Syndrome, abbreviated VHL, is a familial syndrome caused by inherited mutations in the VHL gene on chromosome 3.

Patients with von Hippel-Lindau are predisposed to developing tumors in a number of organs including

  • the brain (hemagioblastoma),
  • the eye (hemagioblastoma),
  • the kidney (renal cell carcinoma),
  • the adrenals (pheochromocytoma).

Pancreatic disease may be the first manifestation of VHL, and most patients with VHL eventually develop a pancreatic tumor. The pancreatic tumors in patients with VHL are interesting because they can have unique appearances. Some have a "clear" appearance under the microscope, and others are mixed tumors.

Tuberous Sclerosis Complex (TSC) is a third genetic syndrome which predisposes to pancreatic neuroendocrine neoplasms. Tuberous sclerosis complex is caused by inherited mutations in one of two genes- TSC1 or TSC2.

The TSC1 gene is on chromosome 9 and it codes for the protein hamartin. The TSC2 gene is on chromosome 16 and it codes for the protein tuberin. Patients with tuberous sclerosis complex can suffer from

  • developmental delay,
  • mental retardation and even autism,
  • they are predisposed to develop a number of different tumors.

They are predisposed to develop three different brain lesions- "tubers," subependymal giant cell astrocytomas and subependymal nodules. In fact, the name tuberous sclerosis comes from the brain lesions that these patients develop- Tuber in Latin means swelling and skleros in Greek means hard.

They also can develop distinctive lesions of

  • the skin (hypomelanotic macules and facial angiofibromas),
  • kidney (angiomyolipomas),
  • lungs (lymphangioleiomatosis),
  • heart (rhabdomyomas),
  • eye tumors ( hamartomas).

Most of these are entirely benign growths, but they can cause symptoms depending on their location. Although less common than the other manifestations of tuberous sclerosis, several patients with tuberous sclerosis complex have been reported who developed pancreatic neuroendocrine neoplasms.

Neurofibromatosis type 1, also known as von Recklinghausen disease, is a familial syndrome caused by inherited mutations in the NF1 gene on chromosome 17. The NF1 gene codes for the protein neurofibromin. Patients with neurofibromatosis develop dark patches of skin (café-au-lait spots), and benign (non-cancerous) and malignant (cancerous) tumors of the nervous system.

The benign nervous system tumors include neurofibromas, and the malignant the "malignant peripheral nerve sheath tumor." They can also develop distinctive eye lesions called Lisch nodules, and a small percentage develops somatostatinomas of the pancreas/duodenum.

Clinical genetic testing is now available for these syndromes, but such testing is best done with the help of a trained genetic counselor. Individuals found to have one of these genetic syndromes may benefit from increased screening for early tumors, and, it is our hope, that a better understanding of the genetics of these syndromes will lead to novel gene-specific therapies in the future.

4 Making a Diagnosis

The following tests and procedures may be used to make a diagnosis of islet cell tumor:

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.

Serum tumor marker test

A procedure in which a sample of blood is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers.

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.

Abdominal CT 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 islet cell tumors. 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.

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 islet cell tumors. 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 different types of islet cell tumors.

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 islet cell tumors. 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 8 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.

Venous sampling after arterial stimulation

A procedure used to help find where a tumor has formed in the pancreas. This is done at the same time as an angiogram. Calcium is injected into an artery that goes to one part of the pancreas (the head, body, and tail are tested one at a time). A blood sample is taken from a vein that comes out of the pancreas and is checked for pancreatic hormones. If there is an islet cell tumor in the area of the pancreas that gets blood from the artery that was injected, there will be an increase in the amount of hormone in the blood sample. If the increase is found after injection into the first artery, the tumor is in the area that gets blood from that artery. If there is no increase, another artery is injected and the test continues.

Anti-insulin antibody test

A test in which a blood sample is checked to see if there are antibodies against insulin in it.

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 islet cell tumors. 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.

PPoma

Fasting serum polypeptide test

A test in which a blood sample is checked to measure the amount of pancreatic polypeptide 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 islet cell tumors. 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.

Atropine suppression test

A test used to help find out if the secretion of a polypeptide (protein) by the pancreas is normal or caused by a tumor. A drug called atropine, which can lessen secretions, is injected into a vein. After the injection, pancreatic polypeptide levels in the blood stay the same in patients with tumors, but drop in patients without tumors.

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.

Stages of Islet Cell Tumors (Endocrine Pancreas)

There are no standard stages for islet cell cancer.

The extent or spread of cancer is usually described as stages. There is no standard staging system for islet cell cancer. The tumors are treated based on where the cancer is found:

  • The cancer is found in one place in the pancreas.
  • The cancer is found in several places in the pancreas.
  • 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.
  • The type of treatment depends on the results of tests and procedures used to diagnose islet cell cancer.

The results of the tests and procedures used to diagnose islet cell tumors and determine whether the cancer has spread help decide the type of treatment that will be used.

5 Treatment

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.

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.

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 Islet Cell Tumors (Endocrine Pancreas)

Gastrinoma

Treatment of gastrinoma may include supportive care and the following:

  • For symptoms caused by stomach acid, 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.
  • For a single tumor in the body or tail of the pancreas, treatment is usually surgery to remove the body and tail of the pancreas.
  • For several tumors in the pancreas, treatment is usually surgery to remove the body and 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.
  • For one or more tumors in the duodenum (the part of the small intestine that connects to the stomach), treatment is usually the Whipple procedure.
  • 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.
  • If the cancer has spread to the liver, treatment may include:
  • Surgery to remove part or all of the liver.
  • Radiofrequency ablation or cryosurgical ablation.
  • 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:
  • Combination 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.

Insulinoma

Treatment of insulinoma may include the following:

  • For one tumor in the head of the pancreas or one tumor smaller than 1 centimeter in the tail, treatment is usually surgery to remove the tumor.
  • For one tumor larger than 1 centimeter in the body or tail of the pancreas, treatment is usually surgery to remove the body and tail of the pancreas.
  • For more than one tumor in the pancreas, treatment is usually 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
  • Radiofrequency ablation or cryosurgical ablation, if the tumor cannot be removed by surgery.
  • For tumors that cannot be removed by surgery, treatment may include the following:
  • Combination chemotherapy.
  • Palliative drug therapy 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 be hepatic arterial occlusion or chemoembolization. Some patients may also receive systemic chemotherapy.

Glucagonoma

Treatment may include the following:

  • For one tumor in the head of the pancreas or one tumor smaller than 1 centimeter in the tail, treatment is usually surgery to remove the tumor.
  • For one tumor larger than 1 centimeter in the tail of the pancreas, treatment is usually surgery to remove the body and tail of the pancreas.
  • For more than one tumor in the pancreas, treatment is usually 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
  • Radiofrequency ablation or cryosurgical ablation, 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 be hepatic arterial occlusion or chemoembolization. Some patients may also receive systemic chemotherapy.

Other Islet Cell Tumors

Treatment of other types of islet cell tumors may include the following:

  • For one tumor in the head of the pancreas or one tumor smaller than 1 centimeter in the tail, treatment is usually surgery to remove the tumor.
  • For one tumor larger than 1 centimeter in the tail of the pancreas, treatment is usually surgery to remove the body and tail of the pancreas.
  • For more than one tumor in the pancreas, treatment is usually 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
  • Radiofrequency ablation or cryosurgical ablation, 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.

If the cancer mostly affects the liver and the patient has severe symptoms from hormones or from the size of tumor, treatment may be hepatic arterial occlusion or chemoembolization. Some patients may also receive systemic chemotherapy.

Recurrent Islet Cell Tumors

Treatment of recurrent islet cell tumors may include the following:

  • Surgery
  • Hormone therapy
  • Regional chemotherapy
  • Hepatic arterial occlusion or chemoembolization, with or without systemic chemotherapy

6 Prevention

There is no known method of preventing islet cell tumors from developing.

Apart from not smoking, the American Cancer Society recommends keeping a healthy weight, and increasing consumption of fruits, vegetables, and whole grains, while decreasing consumption of red and processed meat, although there is no consistent evidence this will prevent or reduce pancreatic cancer specifically.

A 2014 review of research concluded that there was evidence that consumption of citrus fruits and curcumin reduced risk of pancreatic cancer, while whole grains, folate, selenium, and non-fried fish possibly also had a beneficial effect.

In the general population, screening of large groups is not currently considered effective, although newer techniques, and the screening of tightly targeted groups, are being evaluated.

Nevertheless, regular screening with endoscopic ultrasound and MRI/CT imaging is recommended for those at high risk from inherited genetics.

7 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with islet cell tumors.

Fear of treatment side effects is common after a diagnosis of an islet cell tumor, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

There are possible side effects for every cancer treatment, but patients don’t experience the same side effects when given the same treatments for many reasons. That can make it hard to predict exactly how you will feel during treatment.
Side effects depend on a variety of factors, including

  • the tumor’s stage,
  • the length and dosage of treatment(s),
  • your overall health.

Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them.

And, ask about the level of care giving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with an islet cell tumor.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies, including concerns about managing the cost of your medical care.

During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care.

8 Risks and Complications

There are several complications associated with islet cell tumors, which include:

  • Diabetes
  • Hormone crises (if the tumor releases certain types of hormones)
  • Severe low blood sugar (from insulinomas)
  • Severe ulcers in the stomach and small intestine (from gastrinomas)
  • Spread of the tumor to the liver
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