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.
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 also 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 if it 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.
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. Cancer 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 a neuroendocrine tumor are listed below. Treatment options and recommendations depend on several factors, including:
The type of neuroendocrine tumor
If it is cancerous and the stage
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 cancer care. Take time to learn about 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.
Surgery is the main treatment for both pheochromocytoma and Merkel cell cancer. During surgery, the doctor removes the tumor along with some surrounding healthy tissue, known as a margin. A surgical oncologist is a doctor who specializes in treating a tumor using surgery.
For pheochromocytoma, laparoscopic surgery may be performed. Laparoscopic surgery is a less invasive type of surgery that uses 3 or 4 small incisions instead of 1 large incision. A thin, lighted tube called a laparoscope that is attached to a video camera is inserted through 1 opening to guide the surgeon. Surgical instruments are inserted through the other openings to perform the surgery.
Before any type of surgery, talk with your doctor about the possible side effects and how they will be managed. Learn more about the basics of cancer surgery.
If removing the tumor using surgery is not possible, it is called an inoperable tumor. In these situations, the doctor will recommend another type of treatment.
Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.
Radiation therapy is generally recommended when a neuroendocrine tumor has spread or is in a location that makes surgery difficult or impossible. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.
For Merkel cell cancer, radiation therapy is often given after surgery for stage I and II disease. This is called adjuvant therapy.
Side effects of radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Learn more about the basics of radiation therapy.
Chemotherapy is the use of drugs to destroy tumor cells, usually by stopping those cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating a tumor with medication.
Systemic chemotherapy gets into the bloodstream to reach tumor 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 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 at the same time. There are many clinical trials underway to study new drugs for neuroendocrine tumors. Learn more about these medications in the Latest Research section.
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. Recent studies have found that a combination of doxorubicin (Adriamycin), fluorouracil (Adrucil, 5-FU), and streptozocin (Zanosar) can reduce the symptoms of a tumor and side effects of its treatment for some patients. Learn more about the basics of chemotherapy and preparing for treatment.
Targeted therapy (updated 03/2017)
Targeted therapy is a treatment that targets the tumor’s specific genes, proteins, or the tissue environment that contributes to its growth and survival. This type of treatment blocks the growth and spread of tumor cells while limiting damage to healthy cells.
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.
In March 2017, the U.S. Food and Drug Administration (FDA) gave accelerated approval to avelumab (Bavencio) to treat patients 12 and older with MCC that has spread to another part of the body. This is called metastatic MCC. Avelumab blocks the programmed death-ligand 1 (PD-L1) pathway. By blocking this pathway, the body’s immune system may be able to more effectively fight cancer cells. There are 2 targeted therapies, everolimus (Afinitor) and sunitinib (Sutent), that have shown some benefit for patients with a pancreatic neuroendocrine tumor, also called an islet cell tumor. Talk with your doctor about possible side effects of these medications and how they can be managed. Learn more about the basics of targeted treatments.
Often, pheochromocytoma is treated with surgery and alpha-adrenergic blockers. These drugs are most commonly used to lower blood pressure. Beta-blockers may be used to control a fast or irregular pulse.
Chemotherapy, targeted therapy, and other medications used to treat neuroendocrine 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 searchable drug databases.
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 a patient's care is relieving his or her 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 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 tumor, such as chemotherapy or surgery. Talk with your doctor about the goals of each treatment in the 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.
Metastatic neuroendocrine tumors
If a cancerous tumor spreads to another part in 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. 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 surgery, radiation therapy, chemotherapy, and/or targeted therapy. Palliative care will also be important to help relieve symptoms and side effects.
For most patients, a diagnosis of metastatic cancer 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 cancer 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 the tumor does return. Learn more about coping with the fear of recurrence.
If a 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 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, radiation therapy, and chemotherapy, 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 cancer. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.
People with recurrent disease 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 cancer recurrence.
If treatment fails
Recovery from a 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 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 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 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. It offers more information about research studies that are focused on finding better ways to care for people with a neuroendocrine tumor. Or, use the menu to choose another section to continue reading this guide.