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Islet Cell Tumor - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

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 is made up of ducts with small sacs called acini at the end. It produces specialized proteins called enzymes that 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 hormones, specifically insulin. Insulin is the substance that helps control the amount of sugar in the blood.

About pancreatic cancer

Cancer begins when healthy 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 grow and spread to other parts of the body. A benign tumor means the tumor can grow but 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 may 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. Too much stomach acid can cause severe ulcers, 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 production of several other hormones, such as growth hormone, insulin, and gastrin.

The next section in this guide is Statistics, and it helps explain how many people are diagnosed with this type of tumor and general survival rates. Or, use the menu on the side of your screen to choose another section to continue reading this guide.    

Islet Cell Tumor - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

ON THIS PAGE: You will find information about how many people are diagnosed with 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 found. 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 five-year survival 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, so the actual risk for a particular individual may be different. 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.

The next section in this guide is Medical Illustrations and it offers drawings of body parts often affected by this disease. Or, use the menu on the left side of your screen to choose another section to continue reading this guide.

Islet Cell Tumor - Medical Illustrations

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

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.

The next section in this guide is Risk Factors, and it explains what factors may increase the chance of developing an islet cell tumor. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Islet Cell Tumor - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

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 the tumor. 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 in three glands: pituitary, parathyroid, and pancreas.

The next section in this guide is Symptoms and Signs, and it explains what body changes or medical problems this type of tumor can cause. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

 

Islet Cell Tumor - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

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.   

Symptoms of a gastrinoma

  • Ulcers, caused by too much stomach acid

  • Diarrhea

Symptoms of an insulinoma

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

  • Confusion

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.

The next section in this guide is Diagnosis, and it explains what tests may be needed to learn more about the cause of the symptoms. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Islet Cell Tumor - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

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 an islet cell tumor, but 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.

  • Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that a tumor is present, but only a biopsy can make a definite diagnosis. A pathologist then analyzes the sample(s) removed during the biopsy. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

  • 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 liquid 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, 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 part of the small intestine. This type of ultrasound gives a clearer picture of the pancreas and other organs. Learn more about endoscopic techniques.

  • 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 liquid 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 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. This test takes place over several days. For more information about octreotide, see the Treatment Options section.

  • Other imaging tests. 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 and grading.

The next section in this guide is Stages and Grades, and it explains the system doctors use to describe a cancerous tumor. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Islet Cell Tumor - Staging

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

ON THIS PAGE: You will learn about how doctors describe a cancerous tumor’s growth or spread. This is called the stage. In addition, you can read about how doctors evaluate and compare cancer cells to health cells, called grading. 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 to 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 that is smaller than 2 centimeters (cm) acts like a benign tumor almost all of the time.

Staging system for islet cell tumors

There is no standard staging system for a cancerous 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 location

  • A tumor in the pancreas that is found in multiple locations

  • A tumor that has spread to the lymph nodes or to 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. If the cancer does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

Grade

Doctors also describe this type of tumor by its grade, which describes how much tumor cells look like healthy cells when viewed under a microscope and how quickly the tumor cells appear to be multiplying.

The doctor compares tissue taken from the tumor with healthy tissue. Healthy tissue usually contains many different types of cells grouped together. If the tumor looks similar to healthy tissue and contains different cell groupings, it is called well differentiated or a low-grade tumor. If the cancerous tissue looks very different from healthy tissue, it is called poorly differentiated or a high-grade tumor. If it is somewhere in between, it is called moderately differentiated or a medium-grade tumor.

The tumor’s grade can help the doctor predict how quickly the cancer will spread. In general, the lower the tumor’s grade, the slower it will grow and spread and the better a person’s prognosis.

Information about the tumor’s stage and grade will help the doctor recommend a specific treatment plan. The next section in this guide is Treatment Options. Or, use the menu on the side of your screen to choose 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, 06/2015

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 known 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 About 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. Care teams also include a variety of other health care professionals, including physician assistants, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

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, grade, 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. 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 the pancreas. Surgery is the removal of the tumor and some 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. During this operation, 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 the basics of 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 and Signs 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 also can help stop the tumor 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.

Another hormone therapy, lanreotide (Somatuline), was also recently approved for the treatment of islet cell tumors. Similar to octreotide, it is also a somatostatin analogue.

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 (see below).

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. This helps doctors 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. Learn more about the basics of targeted treatments.

In 2011, the U.S. Food and Drug Administration (FDA) approved two targeted treatments for locally advanced or metastatic islet cell tumors. 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 mouth sores, 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.

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. Generally, chemotherapy is used to treat islet cell tumors that are moderately to poorly differentiated, quite large, or are no longer responding to hormonal or targeted therapies.

Systemic chemotherapy gets into 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. Common drug combinations used for islet cell tumors include capecitabine (Xeloda) and temozolomide (Methazolastone, Temodar), which are given orally, or either carboplatin (Paraplatin) or cisplatin (Platinol) combined with etoposide (Toposar, VePesid), which is given intravenously.

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 the basics of 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 type of immunotherapy given as injections under the skin. It 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 used as frequently nowadays because of the development of other more effective therapies.

Learn more about the basics of immunotherapy.

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 is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process.

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, emotional support, 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 palliative 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 can be 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 getting 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, or targeted therapy. As noted above, hepatic artery occlusion or embolization may be used if cancer has spread to the liver. Palliative 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 having “no evidence of disease” or NED. 

A remission may be temporary or permanent. This uncertainty causes many people to worry 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 your 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. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the treatments 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. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

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 the tumor cannot be cured or controlled, the disease may be called advanced or terminal.

This diagnosis is stressful, and advanced disease 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 in this guide is About Clinical Trials, and it offers more information about research studies that are focused on finding better ways to care for people with islet cell tumors. Or, use the menu on the side of your screen to choose 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, 06/2015

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.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for patients with an islet cell tumor. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the FDA was previously tested in 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.

Deciding to join a clinical trial

People 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, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” However, placebos are usually combined with standard treatment in most cancer clinical trials. 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.

Patient safety and informed consent

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.

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. 

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for an islet cell tumor, learn more in the Latest Research section.

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.

In addition, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

The next section in this guide is Latest Research, and it explains areas of scientific research currently going on for this type of tumor. Or, use the menu on the side of your screen to choose 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, 06/2015

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.

  • Anti-angiogenesis therapy. As discussed in the Treatment Options section, anti-angiogenesis therapies are a type of targeted therapy used to treat advanced islet cell tumors. A number of new anti-angiogenesis therapies from the same family of drugs as sunitinib are being tested in clinical trials for islet cell tumors. These drugs are all taken orally.

    Another anti-angiogenic drug is bevacizumab (Avastin), which is a monoclonal antibody given through an intravenous (IV) tube placed into a vein using a needle. It is already approved to treat colon, lung, kidney, and brain cancers. A recent study showed that combining bevacizumab with everolimus and octreotide increased the chances that an islet cell tumor would shrink and increased the time it took for a tumor to grow or spread. However, people who took bevacizumab did not live longer than those who did not. Bevacizumab treatment also caused more side effects than the standard treatment.

  • Other targeted therapies. In addition to anti-angiogenesis therapies, new drugs that block the mTOR pathway, as well as other signaling pathways important in the survival, growth, and spread of islet cell tumors, are also being studied.

  • Peptide receptor radionuclide therapy. This technique 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. Peptide receptor radionuclide therapy is being actively explored and is used most notably in Europe.

  • Palliative 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.

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding islet cell tumors, explore these related items that take you outside of this guide:

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

  • Visit ASCO’s CancerProgress.Net website to learn more about the historical pace of research for pancreatic cancer. Please note this link takes you to a separate ASCO website.

  • Visit the website of the Conquer Cancer Foundation to find out how to help support research for every cancer type. Please note this link takes you to a separate ASCO website. 

The next section in this guide is Coping with Side Effects, and it offers some guidance in how to cope with the physical, emotional, and social changes that cancer and its treatment can bring. Or, use the menu on the side of your screen to choose 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, 06/2015

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 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. 

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.

Talking with your health care team about side effects

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 Follow-Up Care section of this guide or talking with your doctor.

The next section in this guide is Follow-up Care, and it explains the importance of check-ups after cancer treatment is finished. Or, use the menu on the side of your screen to choose another section to continue reading this guide. 

Islet Cell Tumor - Follow-Up Care

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

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

Care for people diagnosed with an islet cell tumor doesn’t end when active treatment has finished. Your health care team will continue to check to make sure the tumor has not returned, manage any side effects, and monitor your overall health. This is called follow-up care.

This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. There are no established guidelines for follow-up care after treatment for an islet cell tumor. However, in general, people who have had surgery should see 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. Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will also ask specific questions about your health. Some people may have blood tests or imaging tests as part of regular follow-up care, but testing recommendations depend on several factors including the type and stage of cancer originally diagnosed and the types of treatment given.

Managing long-term and late side effects

Most people expect to experience side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. In addition, other side effects called late effects may develop months or even years afterwards. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on the type of cancer, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may also have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to ask about any concerns you have about your future physical or emotional health. ASCO offers forms to help create a treatment summary to keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed.

This is also a good time to decide who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the general care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your cancer care will lead your follow-up care, be sure to share your cancer treatment summary and survivorship care plan forms with him or her, as well as all future health care providers. Details about your cancer treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship, and it describes how to cope with challenges in everyday life after a diagnosis. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Islet Cell Tumor - Survivorship

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

ON THIS PAGE: You will read about how to with challenges in everyday life after being diagnosed with a tumor. To see other pages, use the menu on the side of your screen.

What is survivorship?

The word survivorship means different things to different people. Two common definitions include:

  • Having no signs of an islet cell tumor after finishing treatment.

  • The process of living with, through, and beyond the diagnosis. According to this definition, survivorship begins at diagnosis and includes people who continue to have treatment over the long term, to either reduce the risk of recurrence or to manage chronic disease.

In some ways, survivorship is one of the most complex aspects of the experience because it is different for every person.

Survivors may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain of how to cope with everyday life.

Survivors may feel some stress when frequent visits to the health care team end following treatment. Often, relationships built with the health care team provide a sense of security during treatment, and people miss this source of support. This may be especially true as new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexuality and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing,

  • Thinking through solutions,

  • Asking for and allowing the support of others, and

  • Feeling comfortable with the course of action you choose.

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the center where you received treatment.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very important role in supporting a person diagnosed with a tumor, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving in this article.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make positive lifestyle changes.

People recovering from an islet cell tumor are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

In addition, it is important to have recommended medical check-ups and tests (see Follow-Up Care) to take care of your health. Rehabilitation services may also be recommended, and this could mean any of a wide range of services such as physical therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent and productive as possible.

Talk with your doctor to develop a survivorship care plan that is best for your needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note these links will take you to other sections of Cancer.Net:

  • ASCO Answers Cancer Survivorship Guide: This 44-page booklet (available as a PDF) helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about what comes next after finishing treatment.

  • Survivorship Resources: Cancer.Net offers an entire area of this website with resources to help survivors, including for survivors in different age groups.

The next section offers Questions to Ask the Doctor to help start conversations with your health care team. Or, use the menu on the side of your screen to choose 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, 06/2015

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.

Questions to ask after getting a diagnosis

  • What type of tumor do I have?

  • 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?

Questions to ask about choosing a treatment and managing side effects

  • 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?

  • How experienced are you in caring for patients with this type of tumor?

  • 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?

  • What support services are available to me? To my family?

  • Whom should I call for questions or problems?

  • Is there anything else I should be asking?

Questions to ask about having surgery

  • What type of surgery do you recommend?

  • Can you explain what will happen during the surgery?

  • How long will the operation take?

  • Will I need to stay in the hospital for this surgery? For how long?

  • Can you describe what my recovery from surgery will be like?

  • 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?

  • What are the possible long-term effects of having this surgery?

  • What can be done to prevent or manage these side effects?

Questions to ask about having chemotherapy, targeted therapy, or immunotherapy

  • What types of medication do you recommend?

  • What is the goal of this treatment?

  • How will the treatment be given? Through an IV or orally?

  • How often will I receive this treatment? For how long?

  • Will I receive this treatment at a hospital or clinic? Or will I take it at home?

  • How can I best prepare myself for this treatment?

  • What side effects can I expect during treatment?

  • What are the possible long-term effects of taking this medication?

  • What can be done to prevent or manage these side effects?

Questions to ask about planning follow-up care

  • What is the risk of the tumor returning? Are there signs and symptoms I should watch for?

  • What long-term side effects or late effects are possible based on the treatment I received?

  • What follow-up tests will I need, and how often will I need them?

  • How do I get a treatment summary and survivorship care plan to keep in my personal records?

  • Who will be coordinating my follow-up care?

  • What survivorship support services are available to me/my child? To my family?

The next section in this guide is Additional Resources, and it offers some more resources on this website beyond this guide that may be helpful to you. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Islet Cell Tumor - Patient Information Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 06/2015

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about care and treatment. 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.

Beyond this guide, here are a few links to help you explore other parts 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.