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Neuroendocrine Tumor - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

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 on the side of your screen. Think of that menu as a roadmap to this full guide.

About the endocrine system and endocrine tumors

The endocrine system is made up of cells that make hormones. Hormones are chemical substances that are produced by the body and carried through the bloodstream to have a specific effect on the activity of other organs or cells in the body.

A tumor begins when normal cells change and grow uncontrollably, forming a mass. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body if it is not found early and treated. A benign tumor means the tumor will not spread and 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 itself can produce hormones and cause serious illness.

About neuroendocrine tumors

A neuroendocrine tumor begins in the hormone-producing cells of the body’s neuroendocrine system, which is made up of cells that are a cross between traditional 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. They perform specific functions, such as regulating the air and blood flow through the lungs and controlling the speed at which food is moved through the gastrointestinal tract.

Types of neuroendocrine tumors

There are many types of neuroendocrine tumors. This section focuses on three specific types: pheochromocytoma, Merkel cell cancer, and neuroendocrine carcinoma. Other types of cancer 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. Eighty percent (80%) of people with pheochromocytoma have a tumor on only one adrenal gland, 10% have tumors on both glands, and 10% have a tumor outside the adrenal glands.

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

Neuroendocrine carcinoma. Approximately 60% of neuroendocrine tumors cannot be described as a specific type of cancer other than neuroendocrine carcinoma. Neuroendocrine carcinoma can start in a number of places in the body, including the lungs, brain, and gastrointestinal tract.

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Neuroendocrine Tumor - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

ON THIS PAGE: You will find information about how many people learn they have this type of tumor each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

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. The five-year survival rate is the percentage of people who survive at least five years after the tumor is detected, excluding those who die from other diseases. The five-year survival rate of people with neuroendocrine tumors varies and depends on several factors. Check the section for the specific type of neuroendocrine tumor (see the Overview section) for more information.

More than 1,500 people in the United States are diagnosed with Merkel cell cancer each year. The five-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.

Survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of tumor, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with a neuroendocrine tumor. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this tumor. Learn more about understanding statistics.

Sources: American Cancer Society and National Cancer Institute.

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Neuroendocrine Tumor - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

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 on the side of your screen.

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 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 two women who develop pheochromocytoma, three 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 have developed the disease.

Family history. Ten percent (10%) of pheochromocytomas are linked to hereditary 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.

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.

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Neuroendocrine Tumor - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

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 on the side of your screen.

People with a neuroendocrine tumor may experience the following symptoms or signs. Sometimes, people with a neuroendocrine tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer.

Pheochromocytoma

  • Hypertension (high blood pressure)
  • Anxiety attacks
  • Fever
  • Headaches
  • Sweating
  • Nausea
  • Vomiting
  • Clammy skin
  • Rapid pulse
  • Heart palpitations

Merkel cell cancer

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

Neuroendocrine carcinoma

  • Hyperglycemia, which is a high level of a sugar called glucose in the blood and causes frequent urination, increased thirst, and increased hunger
  • Hypoglycemia, which is a low level of glucose in the blood and 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
  • Persistent cough or hoarseness
  • 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
  • Headache
  • Anxiety
  • Gastric ulcer disease
  • Skin rash

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

If you are concerned about one or more of the symptoms or signs on this list, 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 helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Neuroendocrine Tumor - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose a tumor, determine whether it is cancerous, and if so, find out if it has spread to another part of the body, called metastasis. Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether a cancerous tumor has spread. This list describes options for diagnosing this type of tumor, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of tumor suspected
  • Signs and symptoms
  • Previous test results

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

Blood/urine tests. The doctor may need samples of the patient’s blood and urine 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 over a period of time for people who sometimes have symptoms of pheochromocytoma. During the glucagon stimulation test, glucagon, a hormone produced by the pancreas that helps the body process carbohydrates, is injected into a vein, and blood samples are drawn at specific times to measure the 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 three 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 three-dimensional picture of the inside of the body with an x-ray machine. 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 pill 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 pill to swallow.

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

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.

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 helps explain the different stages for this type of tumor. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.

Neuroendocrine Tumor - Staging

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

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 on the side of your screen.

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 cancer'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 cancers.

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:

Stage I: The primary tumor is 2 centimeters (cm) or smaller at its widest part, and 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 there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

Source: National Cancer Institute.

Information about the tumor’s stage will help the doctor recommend a treatment plan. The next section helps explain the treatment options for this type of tumor. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.

Neuroendocrine Tumor - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

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 on the side of your screen.

This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of tumor. 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 approach to treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Your doctor can help you review all treatment options. For more information, see the 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.

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. 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 a small border of healthy tissue around the tumor, called a margin. A surgical oncologist is a doctor who specializes in treating cancer using surgery.

For pheochromocytoma, laparoscopic surgery may be performed. Laparoscopic surgery is a less invasive type of surgery that uses three or four small incisions instead of one large incision. A thin, lighted tube called a laparoscope that is 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.

If removing the tumor using surgery is not possible, it is called an inoperable tumor, and the doctor will recommend another type of treatment.

Before any type of surgery, talk with your doctor about the possible side effects and how they will be managed. Learn more about cancer surgery.

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. 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 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 radiation therapy.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication.

Systemic chemotherapy is delivered through the bloodstream to reach cancer 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 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. 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 cancer and side effects of cancer treatment for some patients. Learn more about chemotherapy and preparing for treatment.

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 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. 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. Learn more about targeted treatments.

Two targeted therapies, everolimus (Afinitor) and sunitinib (Sutent), have demonstrated some benefit for 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 surgery and alpha-adrenergic blockers, which are medicines that are most commonly used to lower blood pressure. Beta-blockers may be used to control a fast or irregular pulse.

The 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 can help a person at any stage of illness. 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, 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 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. Learn more about palliative care

Metastatic neuroendocrine tumors

If a cancerous tumor 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. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.

Your health care team may recommend a treatment plan that includes surgery, radiation therapy, chemotherapy, and/or targeted therapy. Supportive 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 “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 tumor will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the tumor returning. Understanding the 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, including whether the tumor’s stage has changed. 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 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.

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. Learn more about dealing with cancer recurrence.

If treatment fails

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

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.

Patients who have advanced disease and who are expected to live less than six 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 helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select 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, 04/2014

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 on the side of your screen.

Doctors and scientists are always looking for better ways to treat patients with a neuroendocrine tumor. To make scientific advances, doctors create research studies involving volunteers, 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.

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 a neuroendocrine tumor.

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. Find out more about placebos in cancer clinical trials.

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.

For specific topics being studied for neuroendocrine tumors, learn more in the Latest Research section.

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.

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.

The next section helps explain the areas of research going on today about this type of tumor. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.

Neuroendocrine Tumor - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

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 on the side of your screen.

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 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, 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.

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 carcinoma in clinical trials.

Bevacizumab (Avastin) is a 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.

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.

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 addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.

Neuroendocrine Tumor - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of a neuroendocrine tumor, 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 Treatment Options section. Learn more about the most common side effects of a tumor and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the tumor’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. Learn more about caregiving.

In addition to physical side effects, there may be 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, including concerns about managing the cost of your medical care

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. Learn more by reading the After Treatment section or talking with your doctor.

The next section helps explain medical tests and check-ups needed after finishing treatment for a neuroendocrine tumor. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.

Neuroendocrine Tumor - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

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

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.

ASCO offers treatment summary forms to help keep track of the treatment you received and develop a survivorship care plan once treatment is completed.

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. Learn more about the next steps to take in survivorship, including making positive lifestyle changes.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select 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, 04/2014

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 on the side of your screen.

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.

  • 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 in treating this type of tumor?
  • What organ(s) is the tumor affecting?
  • What are my treatment options?
  • What clinical trials are open to 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?
  • Can surgery be done to remove the entire tumor?
  • Who will be part of my health care team, and what does each member do?
  • Who will be coordinating my overall treatment and follow-up care?
  • May I have some reading materials on my tumor type and medications? Where can I get more information about my diagnosis and treatment?
  • 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?
  • Are the side effects permanent?
  • 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 related to my medical care, who can help me with these concerns?
  • What follow-up tests will I need, and how often will I need them?
  • What support services are available to me? To my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.

Neuroendocrine Tumor - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2014

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 on the side of your screen.

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. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

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