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Neuroendocrine Tumor - Introduction

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Neuroendocrine Tumor. To see other pages, use the menu. Think of that menu as a roadmap to this full guide.

About the endocrine system

The body’s endocrine system is made up of cells that produce hormones. Hormones are chemical substances that are carried through the bloodstream that have a specific effect on the activity of other organs or cells in the body.

About endocrine and neuroendocrine tumors

A tumor begins when healthy cells change and grow out of control, forming a mass. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body if it is not found early and treated. A benign tumor means the tumor can grow but will not spread. A benign tumor usually can be removed without it causing much harm.

An endocrine tumor is a mass that begins in the parts of the body that produce and release hormones. Because an endocrine tumor develops from cells that produce hormones, the tumor can also produce hormones. This can cause serious illness.

A neuroendocrine tumor begins in the hormone-producing cells of the body’s neuroendocrine system, which is made up of cells that are a combination of hormone-producing endocrine cells and nerve cells. Neuroendocrine cells are found throughout the body in organs such as the lungs and gastrointestinal tract, including the stomach and intestines. Neuroendocrine cells perform specific functions, such as regulating air and blood flow through the lungs and controlling how quickly food moves through the gastrointestinal tract.

Types of neuroendocrine tumors

There are many types of neuroendocrine tumors. This section focuses on 3 specific types: pheochromocytoma, Merkel cell cancer, and neuroendocrine carcinoma. Other types of tumors that begin in hormone-producing cells are described in their own sections on Cancer.Net, including endocrine tumors, carcinoid tumorsthymoma, thyroid cancer, and islet cell tumors.

  • Pheochromocytoma. Pheochromocytoma is a rare tumor that begins in the chromaffin cells of the adrenal gland. These specialized cells release the hormone adrenaline during times of stress. Pheochromocytoma most often occurs in the adrenal medulla, the area inside the adrenal glands. This type of tumor increases the production of the hormones adrenaline and noradrenaline, which increase blood pressure and heart rate. Even though a pheochromocytoma is usually benign, it may still be life-threatening because the tumor may release large amounts of adrenaline into the bloodstream after injury. Among people with pheochromocytoma, 80% have a tumor in 1 adrenal gland, 10% have tumors in both glands, and 10% have a tumor outside the adrenal glands.

  • Merkel cell cancer. Merkel cell cancer is a highly aggressive, or fast-growing, rare cancer. It starts in hormone-producing cells just beneath the skin and in the hair follicles. It is usually found in the head and neck region. Merkel cell cancer may also be called neuroendocrine carcinoma of the skin or trabecular cancer.

  • Neuroendocrine carcinoma. Around 60% of neuroendocrine tumors cannot be described as anything other than “neuroendocrine carcinoma.” Neuroendocrine carcinoma can start in a number of places in the body, including the lungs, brain, and gastrointestinal tract.

The next section in this guide is Statistics. It helps explain how many people are diagnosed with this type of tumor and general survival rates. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will find information about how many people are diagnosed with this type of tumor each year. You will also learn some general information on surviving the disease. Remember, survival rates depend on several factors. To see other pages, use the menu.

Each year an estimated 8,000 people in the United States are diagnosed with a neuroendocrine tumor that starts in the gastrointestinal tract, which includes the stomach, intestine, appendix, colon, or rectum. Most cases are diagnosed in people who are in their early 60s.

The 5-year survival rate tells you what percent of people live at least 5 years after the cancer is found. Percent means how many out of 100. The 5-year survival rate of people with neuroendocrine tumors varies and depends on several factors, including where the tumor is located. Check the section for the specific type of neuroendocrine tumor (see the Introduction section) for more information.

More than 1,500 people in the United States are diagnosed with Merkel cell cancer each year. The 5-year survival rate of people with Merkel cell cancer is about 60%. It is much higher if the cancer is found early, before it has spread to the lymph nodes or distant parts of the body.

It is important to remember that statistics on how many people survive this type of cancer are an estimate. The estimate comes from data based on thousands of people with this cancer in the United States each year. So, your own risk may be different. Doctors cannot say for sure how long anyone will live with a neuroendocrine tumor. Also, experts measure the survival statistics every 5 years. This means that the estimate may not show the results of better diagnosis or treatment available for less than 5 years. Learn more about understanding statistics.

Source: American Cancer Society.

The next section in this guide is Risk Factors. It explains what factors may increase the chance of developing this disease. Or, use the menu to choose another section to continue reading this guide. 

Neuroendocrine Tumor - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of tumor. To see other pages, use the menu.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a neuroendocrine tumor, most do not directly cause it. Some people with several risk factors never develop a tumor, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

The following factors can raise a person's risk of developing a neuroendocrine tumor:

  • Age. Pheochromocytoma is most common in people between the ages of 40 and 60. Merkel cell cancer is most common in people older than 70.

  • Gender. Men are more likely to develop pheochromocytoma than women. For every 2 women who develop pheochromocytoma, 3 men will develop the disease. Men are also more likely to develop Merkel cell cancer than women.

  • Race/ethnicity. White people are most likely to develop Merkel cell cancer; however, some black people and people of Polynesian descent develop the disease.

  • Family history. Ten percent (10%) of pheochromocytomas are linked to hereditary, or genetic, causes. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition that increases the risk of developing a tumor in the pituitary gland, parathyroid gland, and pancreas. Multiple endocrine neoplasia type 2 (MEN2) is a hereditary condition associated with medullary thyroid cancer and other types of cancer, including pheochromocytoma.

  • Immune system suppression. People with human immunodeficiency virus (HIV), the virus that causes acquired immune deficiency syndrome (AIDS), and people whose immune systems are suppressed because of an organ transplant have a higher risk of developing a neuroendocrine tumor.

  • Merkel cell polyomavirus (MCV). Research indicates that there is a link between this virus and Merkel cell cancer. MCV is present in up to an estimated 80% of Merkel cell cancers. However, scientists believe MCV is common, while Merkel cell cancer is not. More research is needed to learn the role of MCV.

  • Arsenic exposure. Exposure to the poison arsenic may increase the risk of Merkel cell cancer.

  • Sun exposure. Because Merkel cell cancer often occurs on the sun-exposed areas of the head and neck, many doctors think that sun exposure may be a risk factor for this type of cancer. Learn more about protecting your skin from the sun.

The next section in this guide is Symptoms and Signs. It explains what body changes or medical problems this disease can cause. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu.

People with a neuroendocrine tumor may experience the following symptoms or signs. Sometimes, people with a neuroendocrine tumor do not have any of these changes. Or, the cause of a symptom may be another medical condition that is not cancer.

Symptoms of pheochromocytoma

  • High blood pressure

  • Anxiety attacks

  • Fever

  • Headaches

  • Sweating

  • Nausea

  • Vomiting

  • Clammy skin

  • Rapid pulse

  • Heart palpitations

Symptoms of Merkel cell cancer

  • Painless, firm, shiny lumps on the skin that can be red, pink, or blue

Symptoms of neuroendocrine carcinoma

  • Hyperglycemia, which is a high level of glucose in the blood. Glucose is a sugar that is converted into energy by the body. Hyperglycemia causes frequent urination, increased thirst, and increased hunger.

  • Hypoglycemia, which is a low level of glucose in the blood. It causes fatigue, nervousness and shakiness, dizziness or light-headedness, sweating, seizures, and fainting.

  • Diarrhea

  • Persistent pain in a specific area

  • Loss of appetite or weight loss

  • A cough or hoarseness that does not go away

  • Thickening or lump in any part of the body

  • Changes in bowel or bladder habits

  • Unexplained weight gain or loss

  • Jaundice, which is the yellowing of the skin and whites of the eyes

  • Unusual bleeding or discharge

  • Persistent fever or night sweats

  • Headaches

  • Anxiety

  • Gastric ulcer disease

  • Skin rash

  • Some people also experience nutritional deficiencies before a diagnosis, such as niacin and protein deficiency. Others develop this symptom later.

If you are concerned about any changes you experience, please talk with your doctor. Your doctor will ask how long and how often you’ve been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If a neuroendocrine tumor is diagnosed, relieving symptoms remains an important part of your medical care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. To see other pages, use the menu.

Doctors use many tests to find, or diagnose, a tumor. They also do tests to learn if the tumor is cancerous, and if so, if it has spread to another part of the body from where it started. If this happens, it is called metastasis. For example, imaging tests can show if the tumor has spread. Imaging tests show pictures of the inside of the body. Doctors may also do tests to learn which treatments could work best.

For most types of tumors, a biopsy is the only sure way for the doctor to know whether an area of the body has cancer. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

This list describes options for diagnosing this type of tumor. Not all tests listed below will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • The type of tumor suspected

  • Your signs and symptoms

  • Your age and medical condition

  • The results of earlier medical tests

In addition to a physical examination, the following tests may be used to diagnose a neuroendocrine tumor:

  • Blood/urine tests. The doctor may collect blood and urine samples to check for abnormal levels of hormones and other substances. Urine tests check for increased levels of adrenaline in the body. Large amounts of adrenaline can be a sign of pheochromocytoma.

    The glucagon stimulation test and clonidine (Catapres) suppression test are blood tests that measure adrenaline levels for people who sometimes have symptoms of pheochromocytoma. During the glucagon stimulation test, glucagon is injected into a vein. Glucagon is a hormone produced by the pancreas that helps the body process carbohydrates. Blood samples are then drawn at specific times to measure adrenaline levels.

    During the clonidine suppression test, a tablet of the drug clonidine is swallowed, and blood samples are taken at regular intervals over the next 3 hours. Clonidine is used to lower adrenaline levels in the blood, so if these levels do not decrease during testing, it may be a sign of a tumor. Blood pressure and heart rate are also carefully monitored during these tests.

  • X-ray. An x-ray is a way to create a picture of the structures inside of the body, using a small amount of radiation.

  • Computed tomography (CT or CAT) scan. A CT scan creates a 3-dimensional picture of the inside of the body using x-rays taken from different angles. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumor. A CT scan can also be used to measure a tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a liquid to swallow.

  • Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also be used to measure a tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a liquid to swallow.

  • Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. A pathologist then analyzes the sample(s) removed during the biopsy. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. Other tests can suggest that a tumor is present, but only a biopsy can make a definite diagnosis.

  • Molecular testing of the tumor. Your doctor may recommend running laboratory tests on a tumor sample to identify specific genes, proteins, and other factors unique to the tumor. Results of these tests will help decide whether your treatment options include a type of treatment called targeted therapy. See the Treatment Options section for more information.

  • Positron emission tomography (PET) or PET-CT scan. A PET scan is usually combined with a CT scan, called a PET-CT scan. However, you may hear your doctor refer to this procedure just as a PET scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body. Some centers are researching the role of PET or PET-CT scans regarding Merkel cell cancer, for both staging and ongoing monitoring of patients during treatment.

After diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is a cancerous neuroendocrine tumor, these results also help the doctor describe it. This is called staging.

The next section in this guide is Stages. It explains the system doctors use to describe the extent of a cancerous neuroendocrine tumor. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will learn about how doctors describe a cancerous tumor's growth or spread. This is called the stage. To see other pages, use the menu.

Staging is a way of describing where a cancerous tumor is located, if or where it has spread, and whether it is affecting other parts of the body.

Doctors use diagnostic tests to find out the tumor’s stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of cancer.

There is no standard staging system for a neuroendocrine tumor. The stages used to describe a pancreatic neuroendocrine tumor are the same as the stages for pancreatic cancer. The following staging system is commonly used for Merkel cell cancer.

Cancer stage groupings for Merkel cell cancer

Stage I: The primary tumor is 2 centimeters (cm) or smaller at its widest part. The cancer has not spread to the lymph nodes or to other parts of the body. Lymph nodes are tiny, bean-shaped organs that help fight infection.

Stage IIA and IIB: The tumor is larger than 2 cm and has not spread to the lymph nodes or other parts of the body.

Stage IIC: The tumor has spread to nearby bone, muscle, connective tissue, or cartilage but not to the lymph nodes or distant parts of the body.

Stage III: The cancer has spread to the lymph nodes and may have spread to nearby bone, muscle, connective tissue, or cartilage.

Stage IV: The tumor has spread to distant parts of the body, such as the liver, lung, bone, or brain.

Recurrent: Recurrent cancer is cancer that has come back after treatment. If the cancer does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

Source: National Cancer Institute.

Information about the cancer’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Treatment Options. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

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.

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. 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

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

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

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

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.

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.

Other medications

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.

Neuroendocrine Tumor - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for patients with a neuroendocrine tumor. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the U.S. Food and Drug Administration (FDA) was tested in clinical trials.

Many clinical trials focus on new treatments. Researchers want to learn if a new treatment is safe, effective, and possibly better than the treatment doctors use now. 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 can be some of the first to get a treatment before it is available to the public. However, there is no guarantee that the new treatment will be safe, effective, or better than what doctors use now.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with your doctor about clinical trials for symptoms and side effects. There are also clinical trials studying ways to prevent tumors from developing.

Deciding to join a clinical trial

People 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 a neuroendocrine tumor.

Sometimes people have concerns that, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” However, placebos are usually combined with standard treatment in most cancer clinical trials. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

Patient safety and informed consent

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.

Finding a clinical trial

Research through clinical trials is ongoing for all types of tumors. For specific topics being studied for neuroendocrine tumors, learn more in the Latest Research section.

Cancer.Net offers a lot of information about clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of tumor.

In addition, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

The next section in this guide is Latest Research. It explains areas of scientific research currently going on for this type of tumor. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of tumor and how to treat it. To see other pages, use the menu.

Doctors are working to learn more about neuroendocrine tumors, 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 the growth of neuroendocrine tumors that started in the lower part of the small intestine, called the midgut, and have spread. Drugs used in chemotherapy, such as fluorouracil, leucovorin (Wellcovorin), and oxaliplatin (Eloxatin), work in different ways to stop the growth of tumor cells, either by destroying the cells or by stopping them from dividing.

  • Peptide receptor radiotherapy (PRRT). This treatment method is not approved for use in the United States but is performed at select centers in the European Union and in U.S. clinical trials. In PRRT, a small amount of radioactive material is attached to a protein called octreotide. The receptors in tumor cells attract the octreotide and expose the cells to the attached radioactive material, destroying them.

  • Targeted therapy and combined treatments. Monoclonal antibodies are a type of targeted therapy that blocks tumor growth in different ways. Some block the ability of tumor cells to grow and spread. Others find tumor cells and help destroy them or carry tumor-killing substances to them. Both everolimus and sunitinib have been shown to delay the growth of islet cell tumors in clinical trials.

    Bevacizumab (Avastin) is another type of monoclonal antibody. It is focused on stopping 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 is to “starve” the tumor. When combined with chemotherapy, it may destroy 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.

  • Merkel cell polyomavirus (MCV). As outlined in the Risk Factors section, MCV is present in most but not all Merkel cell cancer tumors. Researchers continue to investigate the link between this common virus and this uncommon type of tumor, including whether the presence or absence of the virus in a tumor could result in different treatment approaches.

  • Immunotherapy. Because of the link between MCV and Merkel cell cancer (see Risk Factors), researchers are looking for ways to boost the body's natural defenses to fight the cancer. Immunotherapy uses materials made either by the body or in a laboratory to improve, target, or restore immune system function.

    A specific area of immunotherapy research is looking at drugs that block a protein called PD-1. PD-1 is found on the surface of T-cells, which are a type of white blood cell that helps the body’s immune system fight disease. PD-1 stops the immune system from destroying cancer cells, so stopping PD-1 from working allows the immune system to better eliminate the disease. Researchers are also testing drugs that target CTLA-4, such as ipilimumab (Yervoy), as well as tyrosine kinase inhibitors and other biologically targeted strategies, to treat Merkel cell cancer.

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

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding neuroendocrine tumors, explore these related items that will take you outside of this guide:

The next section in this guide is Coping with Treatment. It offers some guidance in how to cope with the physical, emotional, and social changes that a tumor and its treatment can bring. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Coping with Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of a tumor and its treatment. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu.

Every treatment for a tumor can cause side effects or changes to your body and how you feel. For many reasons, people don’t experience the same side effects even when given the same treatment for the same type of tumor. This can make it hard to predict how you will feel during treatment.

As you prepare to start treatment, it is normal to fear treatment-related side effects. It may help to know that your health care team will work to prevent and relieve side effects. Doctors call this part of treatment “palliative care.” It is an important part of your treatment plan, regardless of your age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for a neuroendocrine tumor are described in the Treatment Options section. Learn more about side effects of cancer and its treatment, along with ways to prevent or control them. Changes to your physical health depend on several factors, including the tumor’s stage, the length and dose of treatment, and your general health.

Sometimes, physical side effects can last after treatment ends. Doctors call these long-term side effects. They call side effects that occur months or years after treatment late effects. Treating long-term side effects and late effects is an important part of survivorship care. Learn more by reading the Follow-up Care section of this guide or talking with your doctor.

Coping with emotional and social effects

You can have emotional and social effects as well as physical effects after a tumor diagnosis. This may include dealing with difficult emotions, such as anxiety or anger, or managing your stress level. Sometimes, patients have problems expressing how they feel to their loved ones, or people don’t know what to say in return.

Patients and their families are encouraged to share their feelings with a member of their health care team. You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your needs.

Coping with financial effects

Medical treatment can be expensive. It is often a big source of stress and anxiety for people with a tumor and their families. In addition to treatment costs, many people find they have extra, unplanned expenses related to their care. For some people, the high cost stops them from following or completing their treatment plan. This can put their health at risk and may lead to higher costs in the future. Learn more about managing financial considerations, in a separate part of this website.

Caring for a loved one with a tumor

Family members and friends often play an important role in taking care of a person with a neuroendocrine tumor. This is called being a caregiver. Caregivers can provide physical, practical, and emotional support to the patient, even if they live far away.

Caregivers may have a range of responsibilities on a daily or as-needed basis. Below are some of the responsibilities caregivers take care of:

  • Providing support and encouragement

  • Giving medications

  • Helping manage symptoms and side effects

  • Coordinating medical appointments

  • Providing a ride to appointments

  • Assisting with meals

  • Helping with household chores

  • Handling insurance and billing issues

Learn more about caregiving.

Talking with your health care team about side effects

Before starting treatment, talk with your doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they are likely to happen?

  • What can we do to prevent or relieve them?

Be sure to tell your health care team about any side effects that happen during treatment and afterward, too. Tell them even if you don’t think the side effects are serious. This discussion should include physical, emotional, and social effects of cancer.

Also, ask how much care you may need at home and with daily tasks during and after treatment. This can help you make a caregiving plan.

The next section in this guide is Follow-up Care. It explains the importance of checkups after treatment for a neuroendocrine tumor has finished. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Follow-Up Care

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will read about your medical care after treatment for a neuroendocrine tumor is complete and why this follow-up care is important. To see other pages, use the menu.

Care for people diagnosed with a tumor doesn’t end when active treatment has finished. Your health care team will continue to check to make sure the tumor has not returned, manage any side effects, and monitor your overall health. This is called follow-up care.

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead. 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 3 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 about every 6 to 12 months, with additional imaging studies, such as x-rays, as needed. Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. A tumor can recur because small areas of tumor cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will also ask specific questions about your health. Some people may have blood tests or imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors including the type and stage of tumor originally diagnosed and the types of treatment given.

Managing long-term and late side effects

Most people expect to experience side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. In addition, other side effects called late effects may develop months or even years afterwards. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on the type of tumor, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may also have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to ask about any concerns you have about your future physical or emotional health. ASCO offers forms to help create a treatment summary to keep track of the treatment you received and develop a survivorship care plan once treatment is completed.

This is also a good time to decide who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the general care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of tumor, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your medical care will lead your follow-up care, be sure to share your treatment summary and survivorship care plan forms with him or her, as well as all future health care providers. Details about your treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a diagnosis of a tumor. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Survivorship

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will read about how to with challenges in everyday life after a diagnosis of a tumor. To see other pages, use the menu.

What is survivorship?

The word “survivorship” means different things to different people. Common definitions include:

  • Having no signs of a tumor after finishing treatment.

  • Living with, through, and beyond the diagnosis of a tumor. According to this definition, survivorship begins at diagnosis and includes people who continue to have treatment over the long term, to either reduce the risk of recurrence or to manage chronic disease.

Survivorship is one of the most complicated parts of your care. This is because it is different for everyone.

Survivors may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after this diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain of how to cope with everyday life.

Survivors may feel some stress when frequent visits to the health care team end following treatment. Often, relationships built with the medical care team provide a sense of security during treatment, and people miss this source of support. This may be especially true as new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexuality and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing,

  • Thinking through solutions,

  • Asking for and allowing the support of others, and

  • Feeling comfortable with the course of action you choose.

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the center where you received treatment.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very important role in supporting a person diagnosed with a tumor, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the tumor diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving in this article.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make positive lifestyle changes.

People recovering from a neuroendocrine tumor are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

In addition, it is important to have recommended medical checkups and tests (see Follow-up Care) to take care of your health. Rehabilitation may also be recommended, and this could mean any of a wide range of services such as physical therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent and productive as possible.

Talk with your doctor to develop a survivorship care plan that is best for your needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note these links will take you to other sections of Cancer.Net:

  • ASCO Answers Cancer Survivorship Guide: Get this 44-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The booklet is available as a PDF, so it is easy to print out.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about what comes next after finishing treatment.

  • Survivorship Resources: Cancer.Net offers an entire area of this website with resources to help survivors, including for survivors in different age groups.

The next section offers Questions to Ask the Doctor to help start conversations with your health care team. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team to help you better understand your diagnosis, treatment plan, and overall care. To see other pages, use the menu.

Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your medical care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your care.

Questions to ask after getting a diagnosis

  • What type of neuroendocrine tumor do I have?

  • Is it cancerous?

  • If so, what is the stage of the tumor? What does this mean?

  • Can you explain my pathology report (laboratory test results) to me?

  • How much experience do you have treating this type of tumor?

  • Which organ(s) is the tumor affecting?

  • May I have some reading materials on my tumor type? Where can I get more information about my diagnosis and treatment?

Questions to ask about choosing a treatment and managing side effects

  • What are my treatment options?

  • What clinical trials are available for me? Where are they located, and how do I find out more about them?

  • What treatment plan do you recommend? Why?

  • What is the goal of each treatment? Is it to eliminate the tumor, help me feel better, or both?

  • What are the possible side effects of this treatment, both in the short term and the long term?

  • What medications will reduce these side effects?

  • Could any of these side effects be permanent?

  • What is the chance that the tumor will come back? Should I watch for specific signs or symptoms?

  • What long-term side effects or late effects are possible based on the treatment I received?

  • Who will be part of my health care team, and what does each member do?

  • Who will be leading my overall treatment?

  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

  • Could this treatment affect my sex life? If so, how and for how long?

  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before cancer treatment begins?

  • If I’m worried about managing the costs of medical care, who can help me?

  • What support services are available to me? To my family?

  • Whom should I call with questions or problems?

  • Is there anything else I should be asking?

Questions to ask about having surgery

  • Can surgery remove the entire tumor? Why or why not?

  • What type of surgery do you recommend? Will lymph nodes be removed?

  • How long will the operation take?

  • How long will I be in the hospital?

  • Can you describe what my recovery from surgery will be like?

  • What are the possible long-term effects of having this surgery?

Questions to ask about other treatments

  • What other type(s) of treatment do you recommend?

  • What is the goal of this treatment?

  • How long will it take to give this treatment?

  • What side effects can I expect during treatment?

  • What are the possible long-term effects of having this treatment?

  • What can be done to relieve the side effects?

Questions to ask about planning follow-up care

  • What follow-up tests will I need, and how often will I need them?

  • How do I get a treatment summary and survivorship care plan to keep in my personal records?

  • Who will be leading my follow-up care?

  • What survivorship support services are available to me? To my family?

The next section in this guide is Additional Resources. It offers some more resources on this website beyond this guide that may be helpful to you. Or, use the menu to choose another section to continue reading this guide.

Neuroendocrine Tumor - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 11/2016

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about medical care and treatment for a person with a tumor. This is the final page of Cancer.Net’s Guide to Neuroendocrine Tumor. To go back and review other pages, use the menu.

Cancer.Net includes many other sections about the medical and emotional aspects of being diagnosed with a tumor, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Beyond this guide, here are a few links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Neuroendocrine Tumor. Use the menu to select another section to continue reading this guide.