Neuroendocrine Tumor of the Pancreas: Types of Treatment

Approved by the Cancer.Net Editorial Board, 06/2019

ON THIS PAGE: You will learn about the different types of treatments doctors use for people with a pancreas NET. Use the menu to see other pages.

This section explains the types of treatments that are the standard of care for a pancreas NET. “Standard of care” means the best treatments known. When making treatment plan decisions, you 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. Clinical trials are an option to consider for treatment and care for all stages of cancer. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections of this guide.

Treatment overview

In cancer care, 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 and is especially important for people with a pancreas NET. Health 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 common types of treatments used for a pancreas NET are listed below. Your care plan also includes treatment for symptoms and side effects, an important part of care.

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

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. These types of talks are called “shared decision making.” Shared decision making is when you and your doctors work together to choose treatments that fit the goals of your care. Shared decision making is particularly important for pancreas NETs because there are different treatment options. Learn more about making treatment decisions.

Active surveillance

Active surveillance, which is also called watchful waiting or watch-and-wait, may sometimes be recommended. This approach is used most often for a low-grade pancreas NET 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, which may include:

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

  • 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 pancreas NET 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. A surgical oncologist is a doctor who specializes in treating cancer using surgery. During surgery, the doctor may need to remove most or part of the pancreas, depending on the location and size of the tumor. The surgeon will usually remove some tissue surrounding the tumor, called a margin, in an effort to leave no traces of cancer in the body.

When completely removing the tumor is not possible, debulking surgery is sometimes recommended. Debulking surgery removes as much of the tumor as possible and may provide some relief from symptoms, but it generally does not cure a NET. If no surgery is possible, it is called an “inoperable” tumor. In these situations, the doctor will recommend another type of treatment.

Common surgical procedures that may be used to treat a pancreas NET 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 the body no longer produces insulin. 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.

Therapies using medication

Systemic therapy is the use of medication to destroy cancer cells. Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle, in a pill or capsule that is swallowed (orally), and intramuscular (IM) injections. This type of medication can reach cancer cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

The type of systemic therapies used for a pancreas NET include:

  • Somatostatin analogs

  • Targeted therapy

  • Chemotherapy

  • Immunotherapy

  • Peptide receptor radionuclide therapy (PRRT)

Each of these types of therapies are discussed below in more detail. A person may receive 1 type of systemic therapy at a time or a combination of systemic therapies given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.

The medications used to treat NETs 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. It is also important to let your doctor know if you are taking any other prescription or over-the-counter medications or supplements. Herbs, supplements, and other drugs can interact with cancer medications. Learn more about your prescriptions by using searchable drug databases.

Somatostatin analogs

Somatostatin is a hormone in the body that controls the release of several other hormones, such as insulin and glucagon. Somatostatin analogs are drugs that are similar to somatostatin and are used to control the symptoms created by the hormones released by a pancreas NET. They may also slow the growth of a NET, although they do not generally shrink the tumors.

There are 2 somatostatin analogs used to treat NETs, octreotide (Sandostatin) and lanreotide (Somatuline). Octreotide is available in 2 forms, short-acting is given under the skin (subcutaneously) and long-acting is given as an IM injection. Lanreotide is given as a long-acting subcutaneous injection. The most common side effects are high blood sugars, the development of gallstones, and mild digestive system upset, such as bloating and nausea.

These hormonal therapy drugs are primarily used for people with a low-grade or intermediate-grade pancreas 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.

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, research studies continue 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 pancreas NETs. These treatments have been shown to significantly slow the growth of pancreas NETs. Both drugs are taken by mouth each day. A specific gene mutation does not have to be present in the tumor cells to receive these treatments.

  • 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.

Other targeted therapies for pancreas NETs are being researched in clinical trials. They include drugs that interfere with new blood vessel formation or with specific survival pathways of cancer cells.

Talk with your doctor about possible side effects for a specific medication and how they can be managed.

Chemotherapy

Chemotherapy is the use of drugs to destroy tumor cells, usually by keeping the tumor cells from growing, dividing, and making more cells. Generally, chemotherapy is used for higher grade (grade 3) pancreas NETs, large pancreas NETs, or if hormonal or targeted therapies are no longer working.

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 a combination of different drugs given at the same time.

Common drug combinations for pancreas NETs include:

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

  • Carboplatin or cisplatin combined with etoposide, all available as generic drugs, given intravenously. This regimen is usually used only for grade 3 disease.

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.

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. Interferon alfa-2b (Intron A) is a type of immunotherapy that has been used to treat NETs. Interferon helps the body’s immune system work better and can lessen diarrhea and flushing. It may also shrink tumors.

Different types of immunotherapy can cause different side effects. Interferon alfa-2b may cause flu-like symptoms, fatigue, and low blood counts. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy.

Peptide receptor radionuclide therapy (PRRT)

In 2018, the FDA approved a treatment called 177Lu-dotatate (Lutathera) for advanced pancreas NETs and advanced GI NETs. It is a radioactive drug that works by binding to a cell’s somatostatin receptor, which may be present on certain tumors. After binding to the receptor, the drug enters the cell, allowing radiation to damage the tumor cells. Common side effects include low levels of white blood cells, high enzyme levels in some organs, high blood sugar levels, low level of potassium, and nausea and vomiting. Talk with your doctor about ways these can be avoided or managed. The broader term to describe this treatment is peptide receptor radionuclide therapy (PRRT).

Liver-directed treatment

If cancer has spread to the liver, the treatments below may be used. 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.

Radiofrequency ablation (RFA). RFA destroys a tumor by heating it with an electric current. It is usually used for small liver metastases and does not work well on larger tumors.

Hepatic artery embolization. This procedure blocks the tumor’s blood supply by sealing off the blood vessels leading to the tumor. If embolization is done by itself, it is called bland embolization. When combined with chemotherapy, it is called chemoembolization. It is not yet clear if 1 treatment is more effective than the other. These treatments are usually used for people with metastatic disease that mostly affects the liver, in particular those who have symptoms caused by the size of the tumor or by hormones produced by the tumor.

Radioembolization. This form of radiation therapy involves the use of tiny beads made of glass or resin that contain low levels of a radioactive material called yttrium-90. The beads are put into the blood vessel that sends blood to the tumor in the liver. The beads then become stuck in the liver and deliver the radiation directly to the tumor. This treatment avoids exposing other areas of the body to radiation. There are 2 FDA-approved methods of radioembolization: SIR-Spheres and TheraSphere.

Physical, emotional, and social effects of a pancreas NET

A tumor and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative care or supportive care. It is an important part of your care that is included along with treatments intended to slow, stop, or eliminate the tumor.

Palliative care focuses on improving how you feel during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of tumor, may receive this type of care. And it often works best when it is started right after a diagnosis. People who receive palliative care along with treatment for the tumor 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 and spiritual support, and other therapies. You may also receive palliative treatments similar to those meant to get rid of the tumor, such as chemotherapy, surgery, or radiation therapy.

Before treatment begins, talk with your doctor about the goals of each treatment in the treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative care options.

During treatment, your health care team may ask you to answer questions about your symptoms and side effects and to describe each problem. Be sure to tell the health care team if you are experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative care in a separate section of this website.

Metastatic pancreas NETs

If a tumor spreads to another part of the body from where it started, doctors call it metastatic cancer. 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. Clinical trials might also 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 the types of treatment described above. As noted above, hepatic artery occlusion or embolization may be used if cancer has spread to the liver. Surgery is used to relieve symptoms rather than eliminate the cancer at this stage. If distant metastases are not causing symptoms, surgery may not be needed. Chemotherapy and radiation therapy may also be offered to help relieve symptoms.

Participation in clinical trials is encouraged. Palliative care will also be important to help relieve symptoms and side effects.

For most people, a diagnosis of metastatic cancer is very stressful and, at times, difficult to bear. You and your family are encouraged to talk about how you feel 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 is 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 the tumor does return. Learn more about coping with the fear of recurrence.

If a pancreas NET returns 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 the treatment options. Often the treatment plan will include the treatments described above, such as surgery and therapy using medications, 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. You are encouraged to talk with the health care team about these feelings and ask about support services to help you cope. Learn more about dealing with recurrence.

If treatment does not work

Recovery from a pancreas NET 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 health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.

People who have advanced disease and who are expected to live less than 6 months may want to consider 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 a pancreas NET. Use the menu to choose a different section to read in this guide.