Neuroendocrine Tumor

Treatment Options

This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. 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 treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options.

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.

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 and stage of neuroendocrine tumor, possible side effects, and the patient’s preferences and overall health. 

Surgery

Surgery is the main treatment for both pheochromocytoma and Merkel cell cancer. It is the removal of the tumor, along with a small amount of healthy tissue around the tumor, called the margin. A surgical oncologist is a doctor who specializes in treating cancer using surgery. In pheochromocytoma, laparoscopic surgery may be performed. Laparoscopic surgery is a less invasive type of surgery that uses three or four small incisions where a laparoscope (a thin, lighted tube) attached to a video camera is inserted through one opening to guide the surgeon. Surgical instruments are inserted through the other openings to perform the surgery. 

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. 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.

Radiation therapy is generally used when a neuroendocrine tumor has spread or is in a location that makes surgery difficult or impossible.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. 

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. A chemotherapy regimen 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. There are many clinical trials underway to study new drugs for neuroendocrine tumors. 

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished. Recent studies have found that a combination of fluorouracil (Adrucil, 5-FU), doxorubicin (Adriamycin), and streptozocin (Zanosar) can reduce the side effects of cancer and cancer treatment for some patients. 

Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal 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. As a result, doctors can 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. 

Two targeted therapies, sunitinib (Sutent) and everolimus (Afinitor) have demonstrated benefit in patients with pancreatic neuroendocrine tumors. Talk with your doctor about possible side effects of these medications and how they can be managed.

Other medications

Often, pheochromocytoma is treated with alpha-adrenergic blockers (medicines most commonly used to lower blood pressure) in addition to surgery. Beta-blockers may be used to control a fast or irregular pulse.

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. 

Palliative/supportive care

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer 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 can help a person at any stage of illness. People often receive treatment for the cancer 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.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive 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 is addressed as quickly as possible. 

Recurrent neuroendocrine tumors

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED.

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. 

If the cancer does return after the original treatment, it is called recurrent cancer. 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 therapies described above (such as surgery, radiation therapy, and chemotherapy) but 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.

People with recurrent cancer 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. 

Metastatic neuroendocrine tumors

If cancer has spread to another location in the body, it is called metastatic cancer.

Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan.

Your health care team may recommend a treatment plan that includes a combination of surgery, radiation therapy, chemotherapy, and/or targeted therapy. Supportive care will also be important to help relieve symptoms and side effects.

For many patients, a diagnosis of metastatic cancer can be 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.

If treatment fails

Recovery from cancer is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.

This diagnosis is stressful, and it may be 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.

Palliative care given toward the end of a person’s life is called hospice care. 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. 

Clinical Trials

Doctors and scientists are always looking for better ways to treat patients with neuroendocrine tumors. To make scientific advances, doctors create research studies involving people, called 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 managing the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Patients 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 neuroendocrine tumors. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with neuroendocrine tumors.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. 

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.

Coping with Side Effects

Fear of treatment side effects is common after a diagnosis of cancer, 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 or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for neuroendocrine tumor are described in detail within the Treatmentsection. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your overall health.

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 a neuroendocrine tumor. 

In addition to physical side effects, there may be psychosocial (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. 

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.

After Treatment

After treatment for a neuroendocrine tumor ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years.

While there are no standard guidelines for follow-up care after treatment of a neuroendocrine tumor, people who have had surgery should be seen by their doctor three months after their operation for a physical examination, blood tests, and a CT scan. After that, follow-up care should include a physical examination and blood tests approximately every six to 12 months, with additional imaging studies (such as x-rays) as needed.

People recovering from a neuroendocrine tumor are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. 

Latest Research

Doctors are working to learn more about neuroendocrine 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.

New chemotherapy and drug combinations. A study has shown that the drug octreotide (Sandostatin) can slow tumor growth in patients with a neuroendocrine tumor of the midgut (the lower part of the small intestine) that has spread. Drugs used in chemotherapy—such as fluorouracil (Adrucil, 5-FU), leucovorin (Wellcovorin), and oxaliplatin (Eloxatin)—work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing.

Targeted therapy and combined treatments. Monoclonal antibodies are a type of targeted therapy that can block tumor growth in different ways. Some block the ability of tumor cells to grow and spread. Others find tumor cells and help kill them or carry tumor-killing substances to them.

Bevacizumab (Avastin) is a type of monoclonal antibody. It blocks a process called angiogenesis, the making new blood vessels. Because a tumor needs the nutrients found in blood vessels to grow and spread, the goal of anti-angiogenesis therapies is to “starve” the tumor. When combined with chemotherapy, it may kill more tumor cells than chemotherapy alone. Bevacizumab may also stop the growth of neuroendocrine tumors by blocking blood flow to the tumor. Another anti-angiogenic drug being studied for neuroendocrine tumors is sorafenib (Nexavar), which may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth and by blocking blood flow to the tumor.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current neuroendocrine tumor treatments in order to improve patients’ comfort and quality of life.