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Endocrine 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 Endocrine Tumors. To see other pages, use the menu. Think of that menu as a roadmap to this full guide.

About the endocrine system

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

For example, part of the pancreas is made up of specialized cells clustered together in islands within the organ, called islets of Langerhans. These cells make different types of hormones. The most important hormone is insulin, which helps control the amount of sugar in the blood.

Part of the endocrine system is the neuroendocrine system, which is made up of cells that are a cross between traditional endocrine cells, or hormone-producing cells, and nerve cells. Neuroendocrine cells are found throughout the body in organs such as the lungs and gastrointestinal tract. They perform specific functions, such as controlling the flow of air and blood through the lungs and controlling how quickly food moves through the gastrointestinal tract.

About endocrine tumors

A tumor begins when healthy cells in the body change and grow out of control, 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. A benign tumor means the tumor will not spread.

An endocrine tumor is a mass that affects the parts of the body that secrete hormones. Because an endocrine tumor starts in the cells that make hormones, the tumor itself can make hormones and cause serious illness.

There are several types of endocrine tumors. For more specific information on each type, select a name below:

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

Endocrine 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 learn they have 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.

As described in the Introduction, there are different types of endocrine tumors. Visit the guide for each type of tumor for the statistics on the number of people diagnosed and survival rates. Please note that these can vary widely and depend on many different factors, so it's important to talk with your doctor about statistics you find. Learn more about understanding statistics.

The next section in this guide is Risk Factors. It offers drawings of body parts often affected by this disease. Or, use the menu to choose another section to continue reading this guide. 

Endocrine 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 tumor, most do not directly cause the disease. 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 may raise a person’s risk of developing specific types of endocrine tumors:

  • Age. Different age groups are more likely to develop different types of endocrine tumors. Pheochromocytoma is most common in people between age 40 and 60. Merkel cell cancer is most common in people older than 70. Thyroid cancer can generally occur at any age, but anaplastic thyroid cancer is usually diagnosed after age 60. Older infants (10 months and older) and adolescents can develop medullary thyroid cancer, especially if they carry the RET proto-oncogene mutation (see below). For a gastrointestinal carcinoid tumor, the average age at diagnosis is 55 to 65. For a carcinoid tumor of the appendix, the average age at diagnosis is about 40. For a lung carcinoid tumor, the average age at diagnosis is between 45 and 55. Children rarely develop a carcinoid tumor.

  • Gender. There are 3 men who develop pheochromocytoma for every 2 women with the same diagnosis. Men are also more likely to develop Merkel cell cancer than women. However, women are 2 to 3 times more likely to develop thyroid cancer than men.

  • Race. A gastrointestinal carcinoid tumor is more common among black people than white people, and black men have a higher risk than black women. Among white people, men and women have the same risk. White people are most likely to develop Merkel cell cancer; however, some black people and Polynesians have developed this type of cancer. White people and Asian people are more likely to develop thyroid cancer.

  • Family history of multiple endocrine neoplasia type 1. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition that increases the risk of developing a tumor of 3 glands: pituitary, parathyroid, and pancreas. It is also estimated that about 10% of gastrointestinal carcinoid tumors are associated with MEN1.

  • Other family history/genetics. For pheochromocytomas, about 10% of diagnoses are linked to hereditary causes. A history of parathyroid tumors in a family with MEN1 may raise a person’s risk of developing a parathyroid tumor. People with other hereditary conditions are at higher risk of developing adrenal gland tumors, including Li-Fraumeni syndrome and Carney complex. Carney complex can also raise the risk of a pituitary gland tumor. In addition, some types of thyroid cancer are associated with genetic factors:

    • An abnormal RET gene, which can be passed from parent to child, may cause medullary thyroid cancer. Not everyone with an altered RET gene will develop cancer. Blood tests and genetic tests can find the gene. Once the altered RET gene is found, a doctor may recommend surgery to remove the thyroid before cancer develops. People with medullary thyroid cancer should talk with their doctor about genetic testing to find out if they have a mutation of the RET proto-oncogene. If so, genetic testing of siblings and children will be recommended.

    • A family history of medullary thyroid cancer increases a person's risk. People with the syndrome multiple endocrine neoplasia type 2 (MEN2) are also at risk for developing medullary thyroid cancer and other cancers.

    • A family history of goiters increases the risk of papillary thyroid cancer.

    • A family history of precancerous polyps in the colon increases the risk of papillary thyroid cancer.

    Learn more about the basics of genetic testing.

  • Immune suppression. People with the human immunodeficiency virus (HIV), the virus that causes acquired immunodeficiency syndrome (AIDS), and people whose immune systems are suppressed because of organ transplantation 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 developing the cancer. Learn more about protecting your skin from the sun.

  • Radiation exposure. Exposure to moderate levels of radiation therapy may increase the risk of papillary thyroid cancer and follicular thyroid cancer. Potential sources of exposure include the following:

    • Low-dose to moderate-dose x-ray treatments used before 1950 to treat children with acne, tonsillitis, and other head and neck problems may increase the risk of papillary and follicular thyroid cancer.

    • People who have been treated with radiation therapy for Hodgkin lymphoma or other forms of lymphoma in the head and neck are at an increased risk for developing papillary and follicular thyroid cancer.

    • One or more exposures to radioactive iodine (also called I-131), especially in childhood, may increase the risk of papillary and follicular thyroid cancer. Sources of I-131 include radioactive fallout from atomic weapons testing during the 1950s and 1960s, nuclear power plant accidents (such as the 1986 Chernobyl nuclear power plant accident and the 2011 earthquake and tsunami that damaged nuclear power plants in Japan), and environmental releases from atomic weapon production plants.

  • Diet low in iodine. Iodine is needed for normal thyroid function. In the United States, iodine is added to salt to help prevent thyroid problems.

  • Stomach conditions. People with diseases that damage the stomach and reduce acid production have a greater risk of developing a stomach carcinoid tumor. In particular, this includes pernicious anemia, a type of anemia characterized by very large, abnormal red blood cells.

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.

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

  • Thickening or lump in any part of the body

  • Persistent pain in a specific (localized) area

  • Anxiety, including panic attacks

  • Irritability

  • Depression

  • Confusion

  • Nervousness

  • Fatigue

  • Fever

  • Sweating

  • Headaches

  • Nausea

  • Vomiting

  • Diarrhea

  • Changes in bowel or bladder habits

  • Unexplained weight gain or loss

  • Jaundice (yellowing of the skin and whites of the eyes)

  • Facial flushing (redness and warm feeling over the face)

  • Intestinal bleeding

Most symptoms of an endocrine tumor are specific to the type of tumor. For more information, see each individual tumor type.

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 an endocrine 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.  

Endocrine 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 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 an endocrine tumor:

  • 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. A pathologist then analyzes the sample(s).  A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

    There are many types of biopsies. The type of biopsy performed will depend on the location of the tumor. There are 2 common types of biopsies used in the diagnosis of an endocrine tumor:

    • Fine needle aspiration. This type of biopsy is usually performed in a doctor’s office or clinic. A local anesthetic (medication to block the awareness of pain) may be injected into the skin near the tumor first to numb the area. The doctor then inserts a thin needle into the tumor and removes cells and some fluid. The procedure may be repeated 2 or 3 times to get samples from different parts of the tumor. The report prepared by the pathologist can be positive (meaning there are cancerous cells), negative (meaning there are no cancerous cells), or undetermined (unclear).

    • Surgical biopsy. If the needle aspiration biopsy is undetermined, then the doctor may suggest a biopsy in which the tumor and possibly other tissue in the area will be removed by surgery. This biopsy is often done under general anesthesia and as an outpatient procedure, meaning you will not need to stay overnight in the hospital.

  • 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 Treatment Options).

  • Laboratory tests. The doctor may need to collect samples of the patient’s blood, urine, and stool to check for abnormal levels of hormones, glucose, and other substances.

  • Neurological examination. An evaluation of the central nervous system may include testing of the reflexes, motor and sensory skills, balance and coordination, and mental status.

Imaging tests

  • Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancer, appear dark.

  • 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 tumors. 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.

  • Endoscopy. An endoscopy allows the doctor to see inside the body with a thin, lighted, flexible tube called an endoscope. The person may be sedated as the tube is inserted through the mouth, down the esophagus, and into the stomach and small bowel. Sedation is the use of medication to become more relaxed, calm, or sleepy and block the awareness of pain. A colonoscopy is a type of endoscope that is inserted through the anus into the colon. It can be used to diagnose a tumor in the lower section of the digestive system.

  • 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.

  • 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 the 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.

  • Positron emission tomography (PET) or PET-CT scan. A PET scan is usually combined with a CT scan (see above), 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 a patient’s body. This sugar substance is taken up by cells that use the most energy. Because a tumor tends to use energy actively, it absorbs more of the radioactive substance. However, because some endocrine tumors grow very slowly, it means they use less energy; this means a PET scan may not be as helpful as other tests in the diagnosis.

  • Radionuclide scanning (octreotide scan). A small amount of a radioactive hormone-like substance is injected into a vein. A special camera is then used to show where the radioactivity accumulates. This procedure is useful for finding where an endocrine tumor has spread, especially if it has spread to the liver. The test takes place over more than 1 day.

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

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

Endocrine 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 tumor’s growth or spread. This is called the stage. To see other pages, use the menu.

Staging is a way of describing where the 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 of 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.

TNM staging system

One tool that doctors use to describe the stage is the TNM system. Doctors use the results from diagnostic tests and scans to answer these questions:

  • Tumor (T): How large is the primary tumor? Where is it located?

  • Node (N): Has the tumor spread to the lymph nodes? If so, where and how many? 

  • Metastasis (M): Has the cancer metastasized to other parts of the body? If so, where and how much?

The results are combined to determine the stage of cancer for each person. There are 5 stages: stage 0 (zero) and stages I through IV (1 through 4). The stage provides a common way of describing the tumor, so doctors can work together to plan the best treatments.

Recurrent tumor

A recurrent tumor is a tumor that has come back after treatment. If it 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.

For more information on the staging of specific endocrine tumors, see each individual tumor type.

Information about the tumor’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.  

Endocrine 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

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.

Treatment options and recommendations for an endocrine tumor depend on several factors, including:

  • The type and stage of the tumor

  • If the tumor is cancerous

  • Possible side effects

  • The patient’s preferences and overall health

Your doctor may also recommend genetic testing of your tumor to help plan treatment. Your care plan may also include treatment for symptoms and side effects, an important part of medical care. Take time to learn about all of 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.

Descriptions of the most common treatment options for an endocrine tumor are listed below. For more information, please see the Treatment Options section for the specific tumor type that has been diagnosed.

Surgery

The purpose of surgery is typically to remove the entire tumor, along with some of the healthy tissue around it, called the margin. A surgical oncologist is a doctor who specializes in treating cancer using surgery. If the tumor cannot be removed entirely, “debulking” surgery may be performed. Debulking surgery is a procedure in which the goal is to remove as much of the tumor as possible.

General side effects of surgery include weakness, fatigue, and pain for the first few days following the procedure. Depending on the type of surgery, there may be other side effects in the short term and in the long term. Be sure to talk with your health care team before your operation about what side effects to expect and how they can be reduced or managed. Learn more about the basics of surgery.

Chemotherapy

Chemotherapy is the use of drugs to destroy tumor cells, usually by stopping the cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating tumors 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 (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or combinations of different drugs at the same time.

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.

Learn more about the basics of chemotherapy and preparing for treatment. The medications used to treat 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.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist. 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 usually consists of a specific number of treatments given over a set period of time.

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

Hormone therapy

The goal of most hormone therapies is to lower the levels of hormones in the body. Hormone therapy may be given to help stop the tumor from growing or to relieve symptoms caused by the tumor. In addition, for thyroid cancer, hormone therapy will be given if the thyroid gland has been removed to replace the hormone that is needed by the body to function properly.

Immunotherapy

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the tumor. It uses materials made either by the body or in a laboratory to bolster, target, or restore immune system function. Examples of immunotherapy include cancer vaccines, monoclonal antibodies, and interferons.

Alpha interferon is a form of immunotherapy given as an injection under the skin. This is sometimes used to help relieve symptoms caused by the tumor, but it can have severe side effects, including fatigue, depression, and flu-like symptoms. Learn more about the basics of immunotherapy.

Targeted therapy

Targeted therapy is a treatment that targets the tumor’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 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 now taking place to find out more about specific molecular targets and new treatments directed at them.

Depending on the type of endocrine tumor, targeted therapy may be a possible treatment option. Targeted therapies such as sunitinib (Sutent) and everolimus (Afinitor) have been approved for treating some islet cell tumors. Early results from clinical studies have shown that targeted therapy drugs may hold promise in treating other types of endocrine tumors, but more research is needed to prove they are effective. Learn more about these treatments in the Latest Research section.

Because research on targeted therapy is ongoing, please talk with your doctor to learn about targeted therapy treatment options. Also, ask your health care team about the possible side effects for a specific medication and how they can be managed. Learn more about the basics of targeted treatments.

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, and important part of care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care 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, surgery, or radiation therapy. 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 endocrine tumor

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 a combination of surgery, chemotherapy, radiation therapy, hormone therapy, immunotherapy, 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 the 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 the 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, chemotherapy, and radiation therapy 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 tumor. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

People with a recurrent tumor 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 recurrence.

If treatment fails

Recovery from an endocrine 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 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 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 tumor. Or, use the menu to choose another section to continue reading this guide.

Endocrine 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 an endocrine 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 are focused on new treatments, evaluating whether 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 cancer.

Deciding to join a clinical trial

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 an endocrine tumor. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with an endocrine 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 cancer. For specific topics being studied for endocrine tumor, 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.

Endocrine 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 endocrine tumors, ways to prevent them, how to best treat them, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new treatment options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options that are best for you. Also, be sure to read the Latest Research section of the specific endocrine tumor type that has been diagnosed.

  • Combination chemotherapy and surgery.  The combination of the drugs oxaliplatin (Eloxatin) and irinotecan (Camptosar) along with surgery is being studied to treat endocrine tumors. Other agents that have some effect in treating some endocrine tumor subtypes are fluoropyrimidines, doxorubicin (Doxil, Adriamycin), and streptozocin (Zanosar).

  • Targeted therapy. Targeted therapy is being studied as a treatment option for several types of endocrine tumors, including neuroendocrine tumors and thyroid cancer. The goal of targeted therapy is to stop the growth and spread of a tumor in several different ways. Some of the drugs being studied for endocrine tumors include:

    • Vatalanib may block some of the enzymes needed for cell growth and sustained blood flow to the tumor. Vatalanib is given with the drug octreotide (Sandostatin), which helps control symptoms, such as diarrhea, caused by some endocrine tumors.

    • Several drugs are being studied in the treatment of advanced thyroid cancer that does not respond to surgery and/or I-131 radiation treatment. These include axitinib (Inlyta), sorafenib (Nexavar), pazopanib (Votrient), and motesanib diphosphate. Learn more about research on thyroid cancer.

    • Pazopanib and motesanib diphosphate are also being studied in the treatment of advanced islet cell tumors. In addition, sunitinib and bevacizumab (Avastin) in combination with chemotherapy, octreotide, and everolimus are being studied for patients with advanced islet cell tumors. Learn more about research on islet cell tumors.

    • Bevacizumab is being researched for the treatment of neuroendocrine tumors. Learn more about research on neuroendocrine tumors

    • Sunitinib and everolimus have shown some effect in treating pancreatic neuroendocrine tumors (islet cell carcinoma).

  • 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.

  • Genetic and molecular testing. The genetic testing and the refinement of RET oncogenes (see Risk Factors) is an active area of research that will improve which treatments are chosen and give more precise prognosis. Researchers are also looking at using the molecular biology of the tumor to help diagnose endocrine tumors and predict how well treatment will work. Molecular biology is the study of the structure and function of cells at the molecular level.

  • Immunotherapy. Immunotherapy, also called biologic therapy, is a type of treatment designed to boost the body's natural defenses to fight the tumor. It uses materials either made by the body or in a laboratory to improve, target, or restore immune system function. It is not entirely clear how immunotherapy works. Doctors are looking at checkpoint inhibitors, cell therapy, or cancer vaccines that might be used to treat endocrine tumors.

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

To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now.

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.

Endocrine 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 medical treatment 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 an endocrine 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 is diagnosed. 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 cancer

Family members and friends often play an important role in taking care of a person with an endocrine 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 a tumor.

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 is finished. Or, use the menu to choose another section to continue reading this guide.  

Endocrine 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 the tumor is completed 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. Although there are no specific guidelines on follow-up care for an endocrine tumor, this plan may include regular physical examinations, blood tests, and/or a CT scan about 3 months after surgery. After that, follow-up care should include a physical examination and blood tests approximately every 6 to 12 months, with additional imaging tests (such as x-rays) performed as they are needed.

It is also important to talk with your doctor about any new symptoms you experience. At your checkups, be sure to ask what types of signs and symptoms to watch for and how quickly you should talk to the doctor about them.

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 recurs 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 cancer 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 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 tumor 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 this diagnosis. Or, use the menu to choose another section to continue reading this guide.

Endocrine Tumor - Survivorship

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

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

  • Having no signs of cancer 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 a tumor 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 health 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

  • 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 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 an endocrine 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.  

Endocrine 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 endocrine tumor do I have?

  • Is the tumor cancerous?

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

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

  • Do I need treatment right away?

  • What treatment options do I have?

  • 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 cancer, help me feel better, or both?

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

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

  • Who will be leading my overall treatment?

  • What is my prognosis?

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

  • 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 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 do I call with questions or problems?

Questions to ask about having surgery

  • What type of surgery will I have? 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 having chemotherapy, hormone therapy, immunotherapy, or targeted therapy

  • Which medications will I be receiving?

  • How will each medication be given to me? How often?

  • What does the preparation for this treatment involve?

  • What side effects can I expect from this treatment?

  • What will be done to relieve the side effects?

Questions to ask about having radiation therapy

  • What type of radiation therapy is recommended?

  • What is the goal of the radiation therapy?

  • How long will each session of the radiation therapy take? How often will it be given?

  • What side effects can I expect from this treatment?

  • What will be done to relieve the side effects?

Questions to ask about planning follow-up care

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

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

  • Whom should I call with questions or problems?

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.

Endocrine 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 your care and treatment. This is the final page of Cancer.Net’s Guide to Endocrine Tumors. To go back and review other pages, use the menu.

Cancer.Net includes many other sections about the medical and emotional aspects of an endocrine tumor diagnosis, 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 Endocrine Tumors. Use the menu to select another section to continue reading this guide.