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Endocrine Tumor - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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 Endocrine Tumors. 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

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 regulatory 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 being insulin, which is a substance that 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 regulating the air and blood flow through the lungs and controlling the speed at which food is moved through the gastrointestinal tract.

About endocrine tumors

A tumor begins when normal cells in the body 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. 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:

  • Carcinoid tumors (includes information on both lung and gastrointestinal carcinoid tumors)
  • Islet cell tumor (includes information on gastrinoma, insulinoma, glucagonoma, VIPoma, somatostatinoma, and nonfunctioning tumors)
  • Neuroendocrine tumor (includes information on Merkel cell cancer, pheochromocytoma, and neuroendocrine carcinoma)

To continue reading this guide, use the menu on the side of your screen to select another section.  

Endocrine Tumor - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find information about how many people learn they have this type of cancer 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.

Because there are different types of endocrine tumors (listed in the Overview), read the guide for each type of tumor for specific statistics regarding 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.

To continue reading this guide, use the menu on the side of your screen to select another section.  

Endocrine Tumor - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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 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 between age 40 and 60. Merkel cell cancer is most common in people older than 70. Thyroid cancer in general can occur at any age, while 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. Men are more likely to develop pheochromocytoma than women by a three to two ratio. Men are also more likely to develop Merkel cell cancer than women. However, women are two to three 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 three glands: pituitary, parathyroid, and pancreas. It is also estimated that approximately 10% of gastrointestinal carcinoid tumors are associated with MEN1.

Other family history/genetics. Ten percent of pheochromocytomas 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 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 (for example, 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 functioning. 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.

To continue reading this guide, use the menu on the side of your screen to select another section.  

Endocrine Tumor - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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 an endocrine tumor may experience the following symptoms or signs. Sometimes, people with an endocrine tumor do not show any of these symptoms. Or, these symptoms may be caused by a 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 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 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 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.  

Endocrine Tumor - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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, find out if it is cancerous, and if so, determine whether 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 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. The sample removed during the biopsy is examined by a pathologist. 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. Two of the more common types of biopsies used in the diagnosis of an endocrine tumor include fine needle aspiration and surgical biopsy.

  • Fine needle aspiration 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 beforehand 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 two or three times to get samples from different parts of the tumor. The report done by the pathologist can be positive (meaning there are cancerous cells), negative (meaning there are no cancerous cells), or undetermined (unclear).
  • If the needle aspiration biopsy is undetermined, the doctor may suggest a biopsy in which the tumor and possibly other tissue in the area will be removed by surgery. This procedure is most often done under general anesthesia and on an outpatient basis (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 and test 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 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 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. This test 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 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 (OctreoScan). 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 one 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 helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.  

Endocrine Tumor - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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 on the side of your screen.

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.

One tool that doctors use to describe the stage is the TNM system. TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

  • How large is the primary tumor and where is it located? (Tumor, T)
  • Has the tumor spread to the lymph nodes? (Node, N)
  • Has the cancer metastasized to other parts of the body? (Metastasis, M)

The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer, 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 there is a recurrence, the tumor may need to be staged again (called re-staging) using the system above.

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

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

Endocrine Tumor - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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

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.

Treatment options and recommendations for an endocrine tumor depend on several factors, including the type and stage of the tumor, if it is cancerous, possible side effects, and 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 cancer 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 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 you can expect, and how they can be reduced or managed. Learn more about 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 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 (schedule) 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. 

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 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 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 biologic therapy 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 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. As a result, doctors can 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. For instance, targeted therapies such as sunitinib (Sutent) and everolimus (Afinitor) have been approved for treating some islet cell tumors. Early results of clinical trials (research studies) with targeted therapy drugs for other types of endocrine tumors are promising, 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, as well as the possible side effects for a specific medication and how they can be managed. Learn more about 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 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, surgery, and 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 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 endocrine tumor

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 a combination of surgery, chemotherapy, radiation therapy, hormone therapy, immunotherapy, 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 the 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 cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with this fear

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, 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, 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.

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 treatment is not successful, the disease may be called advanced or terminal.

This diagnosis is stressful, and this 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 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 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.  

Endocrine Tumor - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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 an endocrine 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 manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease. 

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating 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, 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 endocrine 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 cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.  

Endocrine Tumor - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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 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 options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options 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 activity against some of the endocrine tumor subtypes are: fluoropyrimidines, doxorubicin, and streptozotocin. 

Targeted therapy. Targeted therapy is being researched as a treatment option for several types of endocrine tumors, including neuroendocrine tumors and thyroid cancer. The focus 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 tumors.
  • Several drugs are being studied for advanced thyroid cancer that does not respond to surgery and/or I-131 treatment. These include axitinib (Inlyta), sorafenib (Nexavar), pazopanib (Votrient), and motesanib diphosphate. Learn more about research on thyroid cancer
  • Pazopanib and motesanib diphospate are also being studied for 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 neuroendocrine tumors. Learn more about research on neuroendrocrine tumors.  
  • Sunitinib and everolimus have activity against pancreatic neuroendocrine tumors (islet cell carcinoma).

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

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current endocrine tumor treatments in order 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 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.  

Endocrine Tumor - Coping With Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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 tumor, but it may be helpful 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 an endocrine tumor are described in detail within the Treatment Options section for each specific tumor type. Learn more about the most common side effects of this disease and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the stage, the length and dosage or 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 an endocrine 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 cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.  

Endocrine Tumor - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will read about your medical care after treatment 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 an endocrine tumor ends, talk with your doctor about developing a follow-up care plan. 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 three months after surgery. After that, follow-up care should include a physical examination and blood tests approximately every six to 12 months, with additional imaging tests (such as x-rays) performed on an as-needed basis. It is also important to talk with your doctor about any new symptoms you experience. At your check-ups, be sure to ask what types of signs and symptoms to watch for and how quickly you should talk to the doctor about them. 

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 the successful treatment of an endocrine 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 you 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.  

Endocrine Tumor - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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.

General questions:

  • 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 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 cancer, help me feel better, or both?
  • 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?
  • What is my prognosis?
  • What are the possible side effects of each treatment, both in the short term and the long term?
  • 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 related to my medical care, who can help me with these concerns?
  • What support services are available to me? To my family?
  • Whom do I call for questions or problems?

For people who need surgery:

  • Will I need to stay in the hospital for this surgery? For how long?
  • What are the possible side effects of my surgery?

For people who need chemotherapy, targeted therapy, immunotherapy, or hormone 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?

For people who need 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?

After treatment:

  • What are the chances that the tumor will return (recur)?
  • What follow-up tests do I need, and how often do I need them?
  • 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.  

Endocrine Tumor - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

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 on the side of your screen.

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. 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 Endocrine Tumors. Use the menu on the side of your screen to select another section, to continue reading this guide.