A tumor occurs when cells in the body become abnormal and multiply without control or order, forming a growth of tissue. A tumor can be benign or malignant. A benign tumor is not cancerous and usually can be removed without it causing much harm. A malignant tumor is cancerous and can be harmful if not found early and treated. It can invade and damage the body’s healthy tissues and organs.
The endocrine system consists of cells that produce hormones. Hormones are chemical substances that are formed in the body and carried in the bloodstream to have a specific regulatory effect on the activity of other organs or cells in the body. For example, a component of the pancreas is made up of specialized cells clustered together in islands within the organ, called islets of Langerhans. These cells produce multiple hormones, the most critical one being insulin, which is a substance that helps control the amount of sugar in the blood.
An endocrine tumor is a growth that affects the parts of the body that secrete hormones. Because an endocrine tumor arises from cells that produce hormones, the tumor itself can produce hormones and cause serious illness.
In addition, a neuroendocrine tumor originates from the hormone-producing cells, specifically those cells of the neuroendocrine system. The neuroendocrine system consists 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, and 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.
There are several types of endocrine tumors. For more specific information on each type, select a name below:
Adrenal gland tumor (includes information on adenomas and adrenocortical carcinoma)
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)
Since there are different types of endocrine tumors (listed above), please read the Cancer.Net 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.
Survival statistics should be interpreted with caution. Estimates are based on data from thousands of cases of this type of tumor in the United States each year, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with an endocrine tumor. Because the survival statistics are often measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this tumor.
A risk factor is anything that increases a person’s chance of developing a tumor. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of a tumor, most do not directly cause cancer. Some people with several risk factors never develop a tumor, while others with no known risk factors do. However, knowing your risk factors and communicating them to your doctor may help you make more informed lifestyle and health-care choices.
The following factors can raise a person’s risk of developing specific types of an endocrine tumor:
Age. Pheochromocytoma is most common between 40 and 60. Merkel cell cancer is most common in people over 70. Thyroid cancer can occur at any age. Anaplastic thyroid cancer is usually diagnosed after 60. Older infants (10 months and older) and adolescents can develop medullary thyroid cancer, especially if they carry the RET proto-oncogene mutation. 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. 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 are at 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, there have been reported cases in black people and Polynesians. White people are more likely to develop thyroid cancer than black people.
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 estimated that approximately 10% of gastrointestinal carcinoid tumor cases are associated with MEN1.
Other family history/genetics. Ten percent of pheochromocytoma cases are linked to hereditary causes. A history of parathyroid tumors in a family with MEN may raise a person’s risk of developing a parathyroid tumor. People with other certain 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. Some types of thyroid cancer are associated with genetics:
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 detect the gene. Once the altered RET gene is identified, a doctor may recommend surgery to remove the thyroid before cancer develops. People with medullary thyroid carcinoma should talk to their doctor about genetic testing to determine if a mutation of the RET proto-oncogene is present. 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.
Immune suppression. People with the human immunodeficiency virus (HIV), the virus that causes acquired immune deficiency syndrome (AIDS), and people whose immune systems are suppressed because of an 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.
Radiation exposure. Exposure to moderate levels of radiation therapy may increase the risk of papillary thyroid cancer and follicular thyroid cancer. Such 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 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, malformed red blood cells.
People with an endocrine tumor may experience the following symptoms. 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. If you are concerned about a symptom on this list, please talk with your doctor.
Most symptoms of an endocrine tumor are specific to the type of tumor. For more information, see each individual cancer type. Some common symptoms of an endocrine tumor are:
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)
Doctors use many tests to diagnose a tumor and determine if it if is cancerous and, if so, if it has metastasized (spread). 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 the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
The type of cancer suspected
Severity of 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 from the biopsy is analyzed by a pathologist (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 are:
Fine needle aspiration. This procedure is usually performed in a doctor’s office or clinic. A local anesthetic may be injected into the skin near the tumor before the biopsy to numb the area. The doctor inserts a thin needle into the tumor and removes cells and some fluid. The procedure may be repeated two or three times to obtain samples from different areas 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.
Surgical biopsy. If the needle aspiration biopsy is not conclusive, the doctor may suggest a biopsy in which the tumor and possibly other tissues in the area will be removed by surgery. This procedure is most often done under general anesthesia and on an outpatient basis.
Laboratory tests. The doctor may need samples of the patient’s blood, urine, and stool to check for abnormal levels of hormones, glucose levels, 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 contrast medium (a special dye) is injected into a patient’s vein to provide better detail.
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.
A colonoscope 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.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture.
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 substance is injected into a patient’s body and absorbed by the organs or tissues being studied. This substance gives off energy that is detected by a scanner, which produces the images. Because some endocrine tumors grow very slowly, a PET scan may not be helpful in 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 in detecting the spread of an endocrine tumor, especially to the liver. The test takes place over the course of multiple days.
X-ray. An x-ray is a picture of the inside of the body. For instance, a chest x-ray can help doctors determine if the cancer has spread to the lungs.
Staging is a way of describing a cancerous tumor, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (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. This system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to the rest of the body. 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.
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 (spread) to other parts of the body? (Metastasis, M)
Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in even more detail.
Node. The “N” in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs that help fight infection. Lymph nodes near the tumor are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.
Distant metastasis. The “M” in the TNM system indicates whether the cancer has spread to other parts of the body.
Doctors assign the stage of the cancer by combining the T, N, and M classifications.
For more information on the staging of an endocrine tumor, see each individual cancer type.
The treatment of an endocrine tumor depends on the type of endocrine tumor, the size and location of the tumor, if it is cancerous, whether the cancer has spread, and the patient’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.
This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials section.
For more information, please see the Treatment section for the specific cancer type that has been diagnosed. Descriptions of the most common treatment options for an endocrine tumor are listed below.
Surgery
The purpose of surgery is 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.
Side effects of surgery include weakness, fatigue, and pain after the first few days following the procedure. Learn more about surgery.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor's office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time. The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, 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 cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. 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 kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy.
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 hormone therapy is often 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 cancer. It uses materials either made 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 significant side effects including fatigue, depression, and flu-like symptoms. Learn more about immunotherapy.
Targeted therapy
Targeted therapy is a treatment that targets faulty genes or proteins that contribute to cancer growth and development. Early results of clinical trials (research studies) with targeted therapy agents are promising in the treatment of endocrine tumors, but additional research is needed to demonstrate their effectiveness. Learn more about targeted treatments.
Doctors and scientists are always looking for better ways to treat patients with an endocrine tumor. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. The clinical trial may be evaluating a new drug, a new combination of existing treatments, a new approach to radiation therapy or surgery, or a new method of treatment or prevention. Patients who participate in clinical trials are 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.
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 finding new drugs and other therapies is 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 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. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.
For specific topics being studied for an endocrine tumor, learn more in the Current Research section.
A tumor and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.
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. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of tumor, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.
After treatment for an endocrine tumor ends, talk with your doctor about developing a follow-up care plan. Although no established guidelines have been defined for an endocrine tumor, this plan may include regular physical examinations, blood tests, and/or a CT scan about three months after surgery. Follow-up care thereafter should include a physical examination and blood tests approximately every six to 12 months, with additional radiographic imaging studies (such as x-rays) to be performed on an as-needed basis. Discuss any new symptoms with your doctor.
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 healthy living after cancer.
Research for endocrine tumors is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your doctor. Also, be sure to read the Current Research section of the specific cancer type that has been diagnosed.
Combination chemotherapy and surgery. The drug combination of oxaliplatin (Eloxatin) and irinotecan (Camptosar) along with surgery is being studied to treat endocrine tumors.
Antiangiogenesis research. Antiangiogenesis research, which explores how tumors develop their blood supplies and how agents disrupt these blood supplies, may benefit people with endocrine tumors. Vatalanib (PTK787) is an antiangiogenesis agent that may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth and by blocking blood flow to the tumor. Vatalanib is given with the drug octreotide (Sandostatin), which helps control symptoms, such as diarrhea, caused by some tumors.
Regular communication with your doctor is important for making informed decisions about your health care. Consider asking the following questions of your doctor:
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?
What treatment plan do you recommend? Why?
What is my prognosis?
What is the goal of each treatment?
What are the possible side effects of this 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?
If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?
What support services are available to me? To my family?
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, or immunotherapy:
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 it take to give the radiation therapy? How often?
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?
In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease.
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