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Islet Cell Tumor - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Islet Cell Tumor. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About the pancreas

The pancreas is a pear-shaped gland located in the abdomen between the stomach and the spine. It is about six inches long and is made up of two major components:

Exocrine component. This component, which is made up of ducts with small sacs called acini at the end, produces enzymes. These specialized proteins are released into the small intestine to help the body digest and break down food, particularly fats.

Endocrine component. This part of the pancreas is made up of specialized cells clustered together in islands within the organ, called islets of Langerhans. These cells make specific hormones, specifically insulin. Insulin is the substance that helps control the amount of sugar in the blood.

Types of pancreatic cancer

Cancer begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor will not spread.

The most common type of pancreatic cancer is called ductal adenocarcinoma, or simply, adenocarcinoma. This type of pancreatic cancer begins in the exocrine component. Learn more about adenocarcinoma of the pancreas.

Much less commonly, a tumor can develop in the islets of Langerhans, the endocrine component. These tumors can be referred to as islet cell tumors, pancreatic islet cell tumors, pancreatic endocrine tumors, Islet of Langerhans tumors, or pancreatic neuroendocrine tumors. The rest of this section focuses on islet cell tumors.

Subtypes of islet cell tumors

An islet cell tumor can be either benign or cancerous. An islet cell tumor may also be classified as either functioning or nonfunctioning. The cells in a functioning islet cell tumor produce hormones that cause specific symptoms, while a nonfunctioning islet cell tumor does not cause hormonal symptoms. Most islet cell tumors are nonfunctioning. Because of a lack of symptoms, these tumors are usually diagnosed at a more advanced stage.

There are five major classifications of functioning islet cell tumors that are based on the hormone the cells normally make. However, some tumors can make two or more of these hormones at the same time.

Gastrinoma. A gastrinoma is an islet cell tumor that makes too much gastrin, a hormone that causes acid production in the stomach, and can result in severe ulcers. This is a condition called Zollinger-Ellison syndrome.

Insulinoma. This type of tumor makes too much insulin, causing hypoglycemia, also known as low blood sugar. An insulinoma is more likely to be noncancerous. Only 10% become cancerous.

Glucagonoma. A glucagonoma is an islet cell tumor that makes too much of the hormone glucagon. Unlike an insulinoma, a glucagonoma causes hyperglycemia, a condition where there is too much sugar in the blood.

VIPoma. A VIPoma starts in the cells in the pancreas that make vasoactive intestinal peptide (VIP), a hormone that helps move water into the intestines. Too much VIP can cause chronic, watery diarrhea, which causes a condition called Verner-Morrison syndrome.

Somatostatinoma. A somatostatinoma usually develops in the head of the pancreas. A somatostatinoma may make somatostatin, a hormone that stops the secretion of several other hormones, such as growth hormone, insulin, and gastrin.

To continue reading this guide, use the menu on the side of your screen to select another section.    

Islet Cell Tumor - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find information about how many people learn they have this type of tumor each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

Islet cell tumors are uncommon, with about 1,000 new cases diagnosed each year in the United States. They account for about 3% to 5% of all pancreatic cancers.

The five-year survival rate is the percentage of people who survive at least five years after the tumor is detected, excluding those who die from other diseases. The five-year survival rate of people with an islet cell tumor is about 42%. The survival rate depends on a variety of factors, including whether the tumor can be removed using surgery. If a tumor is localized and can be removed, the five-year survival rate is about 55%. If a tumor cannot be removed, the rate is about 15%. 

Cancer survival statistics should be interpreted with caution. Estimates are based on data from many cases of this type of tumor in the United States each year, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with an islet cell tumor. Learn more about understanding statistics.

Source: National Cancer Institute.

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Islet Cell Tumor - Medical Illustrations

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find basic drawings of the pancreas and other nearby organs. To see other pages, use the menu on the side of your screen.

Pancreatic Cancer Anatomy

Larger image

The pancreas is made up of the head, body, and tail. Pancreatic cancer most commonly develops in the head of the pancreas.

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Islet Cell Tumor - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of tumor. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause it. Some people with several risk factors never develop a tumor, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

Currently, the only factor researchers have found that may raise a person’s risk of developing an islet cell tumor is a hereditary condition called multiple endocrine neoplasia, type 1 (MEN1). MEN1 increases the risk of developing tumors of three glands: pituitary, parathyroid, and pancreas.

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Islet Cell Tumor - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

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.   

Gastrinoma

  • Ulcers caused by too much stomach acid
  • Diarrhea

Insulinoma

  • Hypoglycemia, which causes fatigue, nervousness and shakiness, dizziness or light-headedness, seizures, and fainting episodes
  • Confusion

Glucagonoma

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

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

Somatostatinoma

  • Type 2 diabetes, sometimes called adult-onset 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, 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 and treatment. 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.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Islet Cell Tumor - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose a tumor and find out if it is cancerous. Some tests may also determine which treatments may be the most effective. If the tumor is cancerous, imaging tests may be used to find out whether the cancer has spread to another part of the body, called metastasis. This list describes options for diagnosing this type of tumor, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of tumor suspected
  • Signs and symptoms
  • Previous test results

In addition to a physical examination, the following tests may be used to diagnose an islet cell tumor:

Laboratory tests. The doctor may take samples of blood, urine, and/or stool to check for abnormal levels of hormones, glucose, and other substances.

Molecular testing of the tumor. Your doctor may recommend running laboratory tests on a tumor sample to identify specific genes, proteins, and other factors unique to the tumor. Results of these tests will help decide whether your treatment options include a type of treatment called targeted therapy. For more information see the Treatment Options section.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow.

Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. Tumors generate different echoes of the sound waves than healthy tissue does, so when the waves bounce back to a computer and are changed into images, the doctor can find masses inside the body. There are different types of ultrasounds. During a standard transabdominal ultrasound, the probe is placed on the outside of the abdomen. However, these images are often not clear enough to diagnose small changes in the pancreas and other organs. During an endoscopic ultrasound, the probe is connected to the end of a thin, flexible, lighted tube called an endoscope and carefully slid down the esophagus into the stomach and duodenum, the top of the small intestine. This type of ultrasound gives a clearer picture of the pancreas and other organs. Learn more about endoscopic techniques in this additional article on Cancer.Net.

X-ray. An x-ray is a way to create a picture of the structures inside of the body using a small amount of radiation. Sometimes, the patient will be asked to swallow barium, which coats the mouth and throat, to provide better detail on the x-ray. This is called a barium swallow.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow. Neuroendocrine tumors of the pancreas are sometimes best shown using an MRI, especially if they have spread to the liver.

Octreotide scan. An octreotide scan is a special type of nuclear medicine scan used to find an islet cell tumor and where it may have spread. A person is given a small amount of the hormone octreotide that has a radioactive substance called Indium-111 attached. Because islet cell tumors have receptors for a similar hormone on their surface, the octreotide attaches to the tumor and causes it to show up on pictures taken by a gamma camera. The test takes place over several days. For more information about octreotide, see the Treatment Options section.

Other newer nuclear medicine scans that use different radioactive isotopes (for example, DOTA-TATE, which uses Gallium-68) are being evaluated in clinical trials as alternative ways to diagnose islet cell tumors. 

After diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is an islet cell tumor, these results also help the doctor describe the tumor; this is called staging.

The next section helps explain the different stages for this type of tumor. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.

Islet Cell Tumor - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will learn about how doctors describe a cancerous tumor’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the tumor's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor decide what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. For example, people with a smaller tumor may not need surgery, while many people with a larger tumor do. This is because an islet cell tumor smaller than 2 centimeters (cm) in size acts like a benign tumor almost 100% of the time.

There is no standard staging system for an islet cell tumor, so the doctor will most likely classify the tumor into one of the following groups:

  • A tumor in the pancreas that is only found in one site
  • A tumor in the pancreas that is found in multiple sites
  • A tumor that has spread to the lymph nodes or other parts of the body. Lymph nodes are tiny, bean-shaped organs that help fight infection.
  • A recurrent tumor, which is a tumor that has come back after treatment

Information about the tumor’s stage will help the doctor recommend a treatment plan.  The next section helps explain the treatment options for this type of tumor. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.

Islet Cell Tumor - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will learn about the different ways doctors use to treat people with this type of tumor. To see other pages, use the menu on the side of your screen.

This section outlines treatments that are the standard of care (the best proven treatments available) for an islet cell tumor. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new approach to treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Your doctor can help you review all treatment options. For more information, see the Clinical Trials and Latest Research sections.

Treatment overview

In caring for a person with a tumor, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team.

Descriptions of the most common treatment options for an islet cell tumor are listed below. Treatment options and recommendations depend on several factors, including the type and stage of the tumor, possible side effects, and the patient’s preferences and overall health. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your doctor and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Active surveillance

Sometimes active surveillance, which is also called watchful waiting or watch-and-wait, may be recommended. This is because many islet cell tumors are slow growing and may not grow, spread, or cause problems for many months or years. These types of tumors are often called non-aggressive or indolent. With this approach, the tumor is monitored closely with regular imaging tests, usually CT scans or sometimes MRI (see the Diagnosis section); blood tests; and physical exams. Active treatment usually only begins if the tumor shows signs of growing and/or spreading.

Surgery

Surgery is the most common treatment for an islet cell tumor that is localized, meaning it has not spread outside of the pancreas. Surgery is the removal of the tumor and surrounding healthy tissue during an operation. During surgery, the doctor may need to remove most or part of the pancreas, depending on the location and size of the tumor. A surgical oncologist is a doctor who specializes in treating cancer using surgery.

Common surgical procedures that may be used to treat an islet cell tumor include:

Enucleation. During this surgery, only the tumor is removed.

Whipple procedure. During this procedure, the surgeon removes the head of the pancreas and part of the small intestine, bile duct, and stomach, and then reconnects the digestive tract and biliary system.

Distal pancreatectomy. If the cancer is located in the tail of the pancreas, the most common operation is a distal pancreatectomy, in which the surgeon removes the tail and body of the pancreas, as well as the spleen.

Splenectomy. This is the removal of the spleen.

Gastrectomy. For a gastrinoma, it may also be necessary to take out the stomach to remove ulcers, a procedure called a gastrectomy.

Side effects of surgery include weakness, fatigue, and pain for the first few days following the procedure. The doctor may prescribe medication to help manage these side effects. The patient will need to stay in the hospital for several days and will probably need to rest at home for about one month. Learn more about cancer surgery.

If all or part of the pancreas was removed during surgery, it may be difficult to digest food. A special diet and medicine may help. Also, the doctor can prescribe hormones and enzymes to replace those lost by the removal of the pancreas. Diabetes is another side effect of pancreas removal due to the loss of insulin, which is made by the pancreas. For this, the doctor can prescribe insulin.

Hormone therapy

Hormone therapy may be given to relieve symptoms caused by the tumor (see the Symptoms section). The most common hormone used is octreotide (Sandostatin, OncoLAR), which is similar to one of the hormones normally produced by the body, somatostatin. Many islet cell tumors have receptors for somatostatin on the surface of their cells. When octreotide attaches to these receptors, it blocks the release of hormones by the tumor and can also help stop it from growing. Octreotide can be given either as daily injections under the skin or as monthly injections into a muscle, called Sandostatin LAR Depot. Common side effects of octreotide include gallstones and/or biliary sludge and gastrointestinal problems, such as diarrhea and abdominal discomfort.

Chemotherapy

Chemotherapy is the use of drugs to destroy tumor cells, usually by stopping the cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating tumors with medication.

Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally). A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using these searchable drug databases.

Immunotherapy

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the tumor. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Alpha interferon is a form of immunotherapy given as injections under the skin and is sometimes used to help relieve symptoms caused by an islet cell tumor. However, it can have significant side effects including fatigue, depression, and flu-like symptoms. Alpha interferon is not often used anymore because of the development of other more effective therapies.

Learn more about immunotherapy in this additional article on Cancer.Net.

Targeted therapy

Targeted therapy is a treatment that targets the tumor’s specific genes, proteins, or the tissue environment that contributes to a tumor's growth and survival. This type of treatment blocks the growth and spread of tumor cells while limiting damage to healthy cells. Targeted therapies still have a number of side effects, but they are different from those caused by traditional chemotherapy.

Recent studies show that not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. As a result, doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them.

Two targeted treatments were approved in 2011 for the treatment of patients with locally advanced or metastatic islet cell tumor. These treatments have been shown to significantly delay the growth of islet cell tumors. Both drugs are taken orally each day.

Everolimus (Afinitor). This drug targets a protein called mTOR that is important for cell growth and survival. Common side effects associated with everolimus include sores in the mouth, fatigue, diarrhea, and rash.

Sunitinib (Sutent). This drug’s main target is the receptor of a protein called VEGF. VEGF is important in angiogenesis, the process of making new blood vessels. Because a tumor needs the nutrients delivered by blood vessels to grow and spread, the goal of anti-angiogenesis therapies like sunitinib is to “starve” the tumor. Common side effects associated with this drug include diarrhea, nausea/vomiting, fatigue, and high blood pressure.

Learn more about targeted treatments.

Hepatic artery embolization

Generally used for patients with cancer that has spread to the liver, hepatic artery embolization helps stop cancer growth and spread by temporarily sealing off the blood vessels that supply oxygen and nutrients to the tumor(s). These are typically branches of the hepatic artery. Whether this procedure is recommended for a patient depends on the size, number, and location of the tumor(s) in the liver.

Sometimes this procedure also involves delivering chemotherapy directly into the tumor before blocking the blood supply to that portion of the liver. The drug(s) used during this procedure are either mixed with an oil called lipiodol or loaded into beads/spheres. This is called chemoembolization or transarterial chemoembolization (TACE). Alternatively, radioactively labeled microbeads, such as TheraSpheres or SIR-Spheres, can be delivered during this procedure. This is known as radioembolization or selective internal radiation therapy (SIRT).

These procedures are usually performed by an experienced interventional radiologist and may require an inpatient hospital stay. Side effects include pain around the liver, fever, and temporarily higher levels of liver enzymes as measured by blood tests.

Getting care for symptoms and side effects

A tumor and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the tumor, an important part of care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the tumor and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy or surgery. Talk with your doctor about the goals of each treatment in your treatment plan.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it is addressed as quickly as possible. Learn more about palliative care

Progressive islet cell tumor

Progressive disease is when the tumor becomes larger or spreads, usually while the patient is having treatment. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of disease because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.

Your health care team may recommend a treatment plan that includes a combination of surgery, hormone therapy, chemotherapy, immunotherapy, and targeted therapy. As noted above, hepatic artery occlusion or embolization may be used if cancer has spread to the liver. Supportive care will also be important to help relieve symptoms and side effects.

For most patients, a diagnosis of progressive disease is very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group. 

Remission and the chance of recurrence

A remission is when a tumor cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED. 

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the tumor will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the tumor returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if it does return. Learn more about coping with the fear of recurrence

If an islet cell tumor does return after the original treatment, it is called a recurrent tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence, including whether the tumor’s stage has changed. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above, such as surgery, chemotherapy, immunotherapy, and targeted therapy, but they may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent tumor.

People with a recurrent tumor often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with recurrence.

If treatment fails

Recovery from an islet cell tumor is not always possible. If treatment is not successful, the disease may be called advanced or terminal disease.

This diagnosis is stressful and is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Patients who have advanced disease and who are expected to live less than six months may want to consider a type of palliative care called hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.

Islet Cell Tumor - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat patients with an islet cell tumor. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating an islet cell tumor. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with an islet cell tumor.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

For specific topics being studied for an islet cell tumor, learn more in the Latest Research section.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends. 

Cancer.Net offers a lot of information about clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of tumor.

The next section helps explain the areas of research going on today about this type of tumor. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.

Islet Cell Tumor - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of tumor and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about islet cell tumors, ways to prevent them, how to best treat them, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you.

Targeted therapy. As discussed in the Treatment Options section, anti-angiogenesis therapies are a type of targeted therapy used to treat advanced islet cell tumors. Bevacizumab (Avastin) is a monoclonal antibody given through an intravenous (IV) tube placed into a vein using a needle. It is already approved for the treatment of colon, lung, kidney, and brain cancers. A number of new anti-angiogenesis therapies from the same family of drugs as sunitinib, which are given as a pill or capsule that is swallowed (orally), are also being tested in clinical trials for islet cell tumors.

In addition to anti-angiogenesis therapies, new drugs that block the mTOR pathway (see the Treatment Options section), as well as other signaling pathways important in the survival, growth, and spread of islet cell tumors, are also being studied.

All of the drugs listed above are being investigated in clinical trials, either by themselves, called monotherapy, or in combination with other drugs, including chemotherapies such as capecitabine (Xeloda) and temozolomide (Methazolastone, Temodar).

Hormone therapy. Lanreotide (Somatuline) and pasireotide (Signifor) are somatostatin analogues like octreotide (see the Treatment Options section). These synthetic hormones are similar in chemical structure and function to ones that the body produces naturally. These hormonal agents have been, and are still being, tested in clinical trials.

One other technique that has been actively explored, and is used most notably in Europe, involves attaching radioactive substances to somatostatin analogues. This is similar in principle to octreotide scanning used for the diagnosis of an islet cell tumor, except in this case the radioactive substances being used, such as Yttrium-90, have the ability to destroy islet cell tumor cells. This approach is known as peptide receptor radionuclide therapy.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current islet cell tumor treatments in order to improve patients’ comfort and quality of life.

To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now.

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.

Islet Cell Tumor - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

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 or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for an islet cell tumor are described in detail within the Treatment Options section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the tumor’s stage, the length and dosage of treatment(s), and 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 caregiving 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. Learn more about caregiving.

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. Learn more by reading the After Treatment section or talking with your doctor.

The next section helps explain medical tests and check-ups needed after finishing treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.

Islet Cell Tumor - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will read about your medical care after treatment for an islet cell tumor is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

After treatment for an islet cell tumor ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years.

ASCO offers treatment summary forms to help keep track of the treatment you received and develop a survivorship care plan once treatment is completed.

While there are no established guidelines for follow-up care after treatment of an islet cell tumor, people who have had surgery should be seen by their doctor three months after their operation for a physical examination, blood tests, and CT scan. After that, follow-up care should include a physical examination and blood tests about every six to 12 months, with additional imaging tests, such as x-rays, on an as-needed basis.

People recovering from an islet cell tumor are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about the next steps to take in survivorship, including making positive lifestyle changes.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.

Islet Cell Tumor - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team, to help you better understand your diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your medical care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your care.

General questions

  • What type of tumor do I have?
  • How often do you treat patients with this type of tumor?
  • Is the tumor cancerous or benign?
  • Is the tumor functioning or nonfunctioning? What does this mean?
  • Has the tumor spread outside the pancreas?
  • Can you explain my pathology report (laboratory test results) to me?
  • What are my treatment options?
  • What clinical trials are open to me? Where are they located, and how do I find out more about them?
  • What treatment plan do you recommend? Why?
  • What is the goal of each treatment? Is it to eliminate the tumor, help me feel better, or both?
  • Do I need to start treatment right away?
  • Who will be part of my health care team, and what does each member do?
  • Who will be coordinating my overall treatment and follow-up care?
  • What are the possible side effects of each treatment option, both in the short term and the long term?
  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
  • Could this treatment affect my sex life? If so, how and for how long?
  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before treatment begins?
  • If I’m worried about managing the costs related to my medical care, who can help me with these concerns?
  • Whom should I call for questions or problems?

For people who need surgery

  • Can you explain what will happen during the surgery?
  • Will I need to stay in the hospital for this surgery? For how long?
  • What side effects will there be from my surgery?
  • Will this surgery affect my ability to digest food? How? For how long?
  • Will I need to take medicine, hormones, or enzymes after this surgery? For how long?
  • How can other side effects be treated?

For people who need chemotherapy, targeted therapy, or immunotherapy

  • What types of medication will I receive?
  • How can I best prepare myself for this treatment?
  • What side effects can I expect from this treatment?
  • What will be done to manage these side effects?

After treatment

  • What are the chances that the tumor will return?
  • What follow-up tests do I need, and how often do I need them?
  • What support services are available to me? To my family?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.

Islet Cell Tumor - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 05/2014

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about care and treatment for a person with a tumor. This is the final page of Cancer.Net’s Guide to Islet Cell Tumor. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects being diagnosed with a tumor, both for the patient and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

This is the end of Cancer.Net’s Guide to Islet Cell Tumor. Use the menu on the side of your screen to select another section to continue reading this guide.