ON THIS PAGE: You will learn about the different types of treatments doctors use for people with a neuroendocrine tumor (NET) of the pancreas. Use the menu to see other pages.
This section explains the types of treatments, also known as therapies, that are the standard of care for a pancreas NET. “Standard of care” means the best treatments known. Information in this section is based on medical standards of care for a pancreas NET in the United States. Treatment options can vary from one place to another.
When making treatment plan decisions, you are encouraged to discuss with your doctor whether clinical trials offer additional options to consider. A clinical trial is a research study that tests a new approach to treatment. Doctors learn through clinical trials whether a 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 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.
How a pancreas NET is treated
In cancer care, different types of doctors who specialize in tumors, called oncologists, 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 other health care professionals, such as physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, dietitians, physical therapists, occupational therapists, and others. Learn more about the clinicians who provide cancer care.
Treatment options and recommendations depend on several factors, including:
The exact location of the primary tumor
The stage of the tumor
Tumor grade and differentiation
Whether the tumor secretes hormones (functional vs. non-functional; see Introduction)
Whether somatostatin receptors are present (based on results of 68Ga DOTATATE PET test; see Diagnosis)
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 conversations 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 important for pancreas NETs because there are different treatment options. Learn more about making treatment decisions.
The common types of treatments used for a pancreas NET are described below. Your care plan also includes treatment for symptoms and side effects, an important part of care.
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 computed tomography (CT) scans or sometimes magnetic resonance imaging (MRI; see Diagnosis)
Active treatment usually only begins if the tumor shows signs of growing or spreading. If active surveillance is an option, talk with your doctor about how often you will need to have the tests and scans.
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. However, this type of surgery generally does not cure a NET. If no surgery is possible, it is called an “inoperable” or "unresectable" 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. Read more about Whipple procedure.
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 (this is called splenectomy).
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. Learn more about pancreatic enzyme replacement therapy (PERT). 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. For grade 1 and grade 2 pancreatic NETs, more treatment after surgery is usually not needed.
Therapies using medication (updated 12/2023)
The treatment plan may include medications to slow down or destroy tumor cells. Medication may be given through the bloodstream to reach tumor cells throughout the body. When a drug is given this way, it is called systemic therapy. Medication may also be given locally, which is when the medication is applied directly to the cancer or kept in a single part of the body.
This treatment is generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.
Medications are often given through an intravenous (IV) tube placed into a vein using a needle, as a pill or capsule that is swallowed (orally), or as intramuscular (IM) or subcutaneous (SC) injections. If you are given oral medications to take at home, be sure to ask your health care team about how to safely store and handle them.
The type of medications used for a pancreas NET include:
Peptide receptor radionuclide therapy (PRRT)
Each of these types of therapies are discussed below in more detail. A person may receive 1 type of medication at a time or a combination of medications 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, causing unwanted side effects or reduced effectiveness. Learn more about your prescriptions by using searchable drug databases.
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 tumor.
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. Talk with your health care team about how side effects will be managed or prevented.
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 and limits damage to healthy cells.
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 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 include diarrhea, nausea, vomiting, fatigue, and high blood pressure.
HIF2a inhibitor. Belzutifan (Welireg) is a drug that targets HIF2a, which is a protein that can support the growth of tumor cells. It can be used to treat a pancreas NET that does not require immediate surgery in people with VHL syndrome.
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 tumor cells.
Talk with your doctor about possible side effects for a specific medication and how they can be managed.
Chemotherapy is the use of drugs to destroy tumor cells, usually by keeping the tumor cells from growing, dividing, and making more cells.
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.
The most common combination for pancreas NETs is called CAPTEM, which is a combination of capecitabine (Xeloda) and temozolomide (Temodar).
Other combination for pancreas NETs are:
Capecitabine and oxaliplatin (Eloxatin), which is a combination called CAPEOX.
Fluorouracil (5-FU) can also be used with oxaliplatin, which is a combination called FOLFOX.
Carboplatin or cisplatin combined with etoposide, all available as generic drugs, given intravenously. This regimen is usually used only for poorly differentiated disease (carcinomas) or very rapidly progressing well-differentiated grade 3 NETs.
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.
A treatment called 177Lu-dotatate (Lutathera) is approved by the FDA for treating advanced pancreas NETs and advanced GI NETs that are easily seen on a PET scan. This form of radiation therapy is called peptide receptor radionuclide therapy (PRRT). It is a radioactive drug that works by binding to a cell’s somatostatin receptors, which may be present on certain tumors. After binding to the receptors, the drug enters the cell, allowing radiation to damage the tumor cell.
Common side effects of this drug include low levels of white blood cells, red blood cells, and platelets; high blood sugar levels; nausea and vomiting; and fatigue. There are also rare but serious risks of kidney problems and secondary blood cancers, such as myelodysplastic syndrome and leukemia. Talk with your doctor about ways these can be avoided or managed. Other forms of PRRT are also being researched in clinical trials.
Immunotherapy uses the body's natural defenses to fight cancer by improving your immune system’s ability to attack tumor cells.
Interferon alfa-2b (Intron A) is a type of immunotherapy that has been used to treat NETs in the past, but it is not currently recommended.
Learn more about the basics of immunotherapy.
Radiation therapy is the use of high-energy x-rays or other particles to destroy tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist.
In general, the most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. External-beam radiation therapy is not regularly used to treat pancreas NETs. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.
Other forms of radiation therapy include radioembolization (see below) and PRRT, a form of radiation therapy that can be given as an infusion (see above).
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. When it is combined with radiation, it is called radioembolization.
It is not yet clear if any form of embolization 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.
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 and 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 and supportive 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 and supportive care along with treatment for the tumor often have less severe symptoms, better quality of life, and report that 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, such as chemotherapy, surgery, or radiation therapy, to improve symptoms.
Before treatment begins, talk with your doctor about the goals of each treatment in the recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative and supportive care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.
Cancer care is often expensive, and navigating health insurance can be difficult. Ask your doctor or another member of your health care team about talking with a financial navigator or counselor who may be able to help with your financial concerns.
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.
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. Your doctor will recommend treatments based on several factors, such as the grade of the tumor, the degree of differentiation, if it is functional or non-functional, and if the tumor tests positive for somatostatin receptors. Read more about grade and degree of differentiation.
Somatostatin analogs, chemotherapy, and targeted therapy may be used alone or in combination to treat a metastatic pancreas NET. In many cases, somatostatin analogs will be the first treatment recommended, followed by PRRT, chemotherapy, and/or targeted therapy. For people with well-differentiated grade 3 disease, the treatment plan may include somatostatin analogs, chemotherapy, and/or PRRT.
Participation in clinical trials is encouraged. Palliative and supportive care will also be important to help relieve symptoms and side effects. Hepatic artery 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. Medications and radiation therapy may also be offered to help relieve symptoms.
For many people, a diagnosis of metastatic cancer is very stressful and difficult. You and your family are encouraged to talk about how you feel with doctors, nurses, social workers, or other members of your health care team. It may also be helpful to talk with other patients, such as through a support group or other peer support program.
The information in this section is based in part on the ASCO guideline, “Systemic Therapy for Tumor Control in Metastatic Well-differentiated Gastroenteropancreatic Neuroendocrine Tumors.” Please note that this link will take you to a different ASCO website.
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).
If a recurrence happens, a new cycle of testing will begin to learn as much as possible about it. 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 a recurrent pancreas NET. Whichever treatment plan you choose, palliative and supportive care will be important for relieving symptoms and side effects.
People with a recurrent tumor sometimes experience emotions such as disbelief or fear. You are encouraged to talk with your health care team about these feelings and ask about support services to help you cope. Learn more about dealing with recurrence.
Recovery from a pancreas NET is not always possible. If the tumor cannot be cured or controlled, the disease may be called advanced or incurable.
This diagnosis is stressful, and for some 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.
Planning for your future care and putting your wishes in writing is important, especially at this stage of disease. Then, your health care team and loved ones will know what you want, even if you are unable to make these decisions. Learn more about putting your health care wishes in writing.
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 your doctor or a member of your palliative 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.