Neuroendocrine Tumor of the Pancreas: Treatment Options

Approved by the Cancer.Net Editorial Board, 08/2017

ON THIS PAGE: You will learn about the different treatments doctors use for people with this type of tumor. Use the menu to see other pages.

This section tells you the treatments that are the standard of care for this type of tumor. “Standard of care” means the best treatments known. When making treatment plan decisions, patients are encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn whether the new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Your doctor can help you consider all your treatment options. To learn more about clinical trials, 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 include a variety of other health care professionals, such as physician assistants, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

Descriptions of the most common treatment options for a pancreatic neuroendocrine tumor are listed below. Treatment options and recommendations depend on several factors, including:

  • The type, grade, and stage of the tumor

  • Possible side effects

  • The patient’s preferences and overall health

Your care plan may also include treatment for symptoms and side effects, an important part of care. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment 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 approach is used most often for low-grade pancreatic neuroendocrine tumors that may grow slowly and not spread or cause problems for many months or years. With this approach, the tumor is closely monitored with regular tests that include:

  • Imaging tests, usually CT scans or sometimes MRI (see the Diagnosis section)

  • Blood tests

  • Physical examinations

Active treatment usually only begins if the tumor shows signs of growing or spreading.

Surgery

Surgery is the most common treatment for a pancreatic neuroendocrine 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 a pancreatic neuroendocrine 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. The digestive tract and biliary system is then reconnected.

  • Distal pancreatectomy. If the tumor 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 1 month.

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. The doctor can also prescribe hormones and enzymes to replace those lost by the removal of the pancreas. Diabetes is another side effect of pancreas removal because insulin is no longer produced by the body. For this, the doctor can prescribe insulin.

Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have. Learn more about the basics of cancer surgery.

Somatostatin analogs

Somatostatin analogs are drugs that are similar in structure and function to a hormone normally produced by the body called somatostatin. Many pancreatic neuroendocrine tumors have receptors for somatostatin on the surface of their cells. When a somatostatin analog attaches to these receptors, it can help stop the tumor from growing and prevent the release of hormones. Preventing the release of hormones helps relieve symptoms caused by the tumor (see the Symptoms and Signs section).

The 2 most commonly used somatostatin analogs include:

  • Octreotide (Sandostatin). This can be given either as daily injections under the skin or as monthly injections into a muscle. Common side effects of octreotide include gallstones, biliary sludge, and gastrointestinal problems, such as diarrhea and abdominal discomfort.

  • Lanreotide (Somatuline). This is given as a monthly injection deep into the skin. The side effects are similar to those of octreotide.

These drugs are primarily used for people with a low-grade or intermediate-grade pancreatic neuroendocrine tumor.

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.

There are 2 targeted treatments approved by the U.S. Food and Drug Administration (FDA) for locally advanced or metastatic pancreatic neuroendocrine tumors. These treatments have been shown to significantly slow the growth of pancreatic neuroendocrine tumors. Both drugs are taken by mouth 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 targets a protein called VEGF. VEGF is important in angiogenesis, which is 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 ending 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 for intermediate-grade or high-grade pancreatic neuroendocrine tumors, large pancreatic neuroendocrine tumors, or if hormonal or targeted therapies are no longer working.

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, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or combinations of different drugs given at the same time.

Common drug combinations used for pancreatic neuroendocrine tumors include:

  • Capecitabine (Xeloda) and temozolomide (Temodar), which are given orally

  • 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 after treatment is finished.

Learn more about the basics of chemotherapy and preparing for treatment. The medications used to treat tumors 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 (Alferon, Infergen, Intron A, Roferon-A) is a type of immunotherapy given as injections under the skin. It is sometimes used to help relieve symptoms caused by a pancreatic neuroendocrine tumor. However, it can have significant side effects, including fatigue, depression, and flu-like symptoms. Alpha interferon is not often used because other, more effective therapies are available.

Different types of immunotherapy can cause different side effects. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy. Read more about immunotherapy for a pancreatic neuroendocrine tumor in the Latest Research section.

Hepatic artery embolization

Hepatic artery embolization is generally used for patients with cancer that has spread to the liver. This procedure 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 giving 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 ethiodized oil (Lipiodol) or loaded into beads/spheres. This is called chemoembolization or transarterial chemoembolization (TACE). Or radioactively labeled microbeads, such as TheraSpheres or SIR-Spheres, can be given 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 overnight hospital stay. Side effects include pain around the liver, fever, and higher levels of liver enzymes as measured by blood tests for a few days or weeks after treatment.

Getting care for symptoms and side effects

A pancreatic neuroendocrine tumor and its treatment often cause side effects. In addition to treatments intended 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 tumor, may receive palliative care. It works best when palliative care is started as early as needed in the treatment process.

People often receive treatment for the tumor at the same time that they receive treatment to ease side effects. In fact, patients who receive both at the same time 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 tumor, 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. 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 pancreatic neuroendocrine tumor

Progressive disease is when the tumor becomes larger or spreads, usually during treatment. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Also, clinical trials might be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.

Your treatment plan may include 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 a pancreatic neuroendocrine 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 new cycle of testing will begin again to learn as much as possible about the recurrence. After this 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 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 doesn’t work

Recovery from a pancreatic neuroendocrine 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 for many people, advanced disease is difficult to discuss. 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 6 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 talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option 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. It offers more information about research studies that are focused on finding better ways to care for people with pancreatic neuroendocrine tumors. You may use the menu to choose a different section to read in this guide.