Each person has two adrenal glands, one located on top of each of the body’s two kidneys. These glands are important to the body’s endocrine (hormonal) system. Each adrenal gland has two main parts, which function separately:
Adrenal cortex. The outer part of the adrenal gland is called the cortex. The adrenal cortex makes three hormones: cortisol, aldosterone, and dehydroepiandrosterone (DHEA). These hormones carefully control metabolism and body characteristics, such as hair growth and body shape.
Adrenal medulla. The gland’s inner part is called the medulla. The adrenal medulla makes other hormones: epinephrine, norepinephrine, and dopamine. These hormones control the body’s responses to stress, including the “fight or flight” adrenaline surge.
Types of adrenal gland tumors
A tumor begins when cells become abnormal and grow uncontrollably, forming a mass. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).
An adrenal gland tumor can sometimes overproduce hormones. When it does, the tumor is called a functioning tumor. An adrenal gland tumor that does not produce hormones is called a nonfunctioning tumor. A tumor can start in an adrenal gland (called a primary adrenal tumor) or it can begin in another organ, such as the lungs, and then metastasize (spread) to the adrenal glands.
This section focuses on primary adrenal gland tumors, which include:
Adenoma. Adenoma is the most common type of adrenal gland tumor, making up 99% of all adrenal gland tumors. It is a noncancerous nonfunctioning tumor of the adrenal cortex. Also called an adrenocortical adenoma, this tumor usually does not cause symptoms, and, if it is small, often does not require any treatment.
Adrenocortical carcinoma. Although very rare, the most common type of cancerous adrenal gland tumor begins in the cortex and is called adrenocortical carcinoma, or adrenal cortical carcinoma. Approximately 4 to 12 people out of 1 million develop this type of tumor. Adrenocortical carcinoma can be a functioning or nonfunctioning tumor; if the tumor is functioning, it may produce more than one hormone.
Pheochromocytoma. Most cases of this type of cancerous neuroendocrine tumor begin in the adrenal medulla. Review the Cancer.Net Guide to Neuroendocrine Tumor for more information.
The symptoms and treatment of an adrenal gland tumor depend on whether the tumor is functioning or nonfunctioning, and what hormone(s) is overproduced, and whether the tumor is a primary adrenal gland tumor or metastases from cancer of another organ.
Statistics
A primary adrenal gland tumor is very uncommon, and exact statistics are not available for this type of cancer in the United States. It is estimated that approximately 300 adults in the United States are diagnosed each year with adrenal cortical cancer. This type of cancer is much less common than an adrenal adenoma, a benign tumor that is more common for middle-age and older adults. The average age of a person diagnosed with an adrenal gland tumor is between 45 and 50; however, these tumors can occur at any age. More women then men tend to be diagnosed with adrenal gland tumors.
Cancer statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer in the United States, 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 adrenal gland tumor.
Statistics adapted from the American Cancer Society.
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 the tumor. 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 cause of most cases of cancerous adrenal cortical carcinoma is not known. However, people with certain hereditary conditions have a higher risk of developing an adrenal gland tumor, including Li-Fraumeni syndrome and Carney complex. People who have a high risk of developing an adrenal gland tumor because of family history should be examined and evaluated yearly by their doctor.
People with an adrenal gland tumor may experience the following symptoms. Sometimes, people with an adrenal gland 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.
High blood pressure
Low potassium level
Heart palpitations
Nervousness
Anxiety (panic attacks)
Headache
Excessive perspiration
Diabetes
Abdominal pain
Unexplained weight gain
Unexplained weight loss
Weakness
Abdominal stretch marks
Excessive hair growth
Unusual acne
Change in libido (sex drive)
In addition, pheochromocytoma may cause dangerous surges of the adrenal gland hormones that regulate blood pressure and response to stress. A hormonal surge can cause blood pressure to increase dramatically and can dangerously increase the risk of heart attack, stroke, hemorrhage, or sudden death. For more information, see the Symptoms section of Cancer.Net’s Guide to Neuroendocrine Tumor.
Doctors use many tests to diagnose a tumor and determine if it has metastasized. Some tests may also determine which treatments may be the most effective. To diagnose an adrenal gland tumor, blood and urine tests (see below) are done to look for certain substances that may indicate that cancer is present. If the doctor suspects that the cancer has spread from another area of the body, a biopsy may be performed to determine the type 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 tumor suspected
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose an adrenal gland tumor:
Blood and urine tests. Blood tests can measure the amounts of natural hormones produced during stress, such as catecholamines and metanephrines, which can detect a functional tumor. A patient may be asked to take a pill on the evening before the blood and urine tests, which helps detect the normal suppression of production of the hormone cortisol. A 24-hour urine sample, which requires the collection of all urine during that timeframe for laboratory testing, may also be necessary. This helps the doctor track how quickly various hormones are produced. Tell your doctor about any medications that you take, even over-the-counter drugs, because this information is needed to correctly interpret the results.
Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. For an adrenal tumor, a hollow needle is used to collect the tissue. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed during the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).
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.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium is injected into a patient’s vein to provide better detail.
Metaiodobenzylguanidine (MIBG) scan. MIBG is a chemical similar to adrenaline that will collect in a neuroendocrine tumor. A MIBG scan can show a tumor of the adrenal medulla that may not appear in an x-ray. The scan takes place over two consecutive days. On the first day, an injection of MIBG is given in the arm. Several hours later, pictures are taken with a special camera that can detect if or where in the body the MIBG has collected. The following morning, more pictures are taken, and the process may be repeated if necessary.
Staging is a way of describing a 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 tumor’s stage, so staging may not be complete until all of the tests, or even the surgical removal of the tumor or adrenal gland, 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.
There is no standard staging system for an adrenal gland tumor. A tumor that started in the adrenal gland and has spread to other areas of the body is usually a more aggressive type of tumor. A recurrent adrenal tumor is a tumor that comes back after treatment.
The treatment of an adrenal gland tumor depends on 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.
Surgery
Adrenalectomy, which is the surgical removal of the adrenal gland with the tumor, may be necessary. If the tumor is smaller than 5 centimeters (cm) and, based on size or the way it looks on imaging scans, shows no evidence that it is cancerous, then laparoscopic surgery may be possible. Laparoscopic surgery is a minimally invasive technique in which a surgeon operates with small telescopes and long instruments. This may be performed from the abdomen or back, depending on the location of the tumor and the skill of the surgeon. However, if the tumor is larger than 5 cm to 6 cm or possibly cancerous, surgery using one large incision in the abdomen or back is recommended. If the patient previously had abdominal surgery, an incision in the back may be easier. Surgery is mostly used to remove a pheochromocytoma: for more information, see the treatment section of the Cancer.Net Guide to Neuroendocrine Tumor.
Adrenal surgery can cause bleeding. Also, the tumor can make excess cortisol, or stress hormone, and the patient will need to be monitored and receive medication to treat high blood pressure during 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. Mitotane (Lysodren) is a medication that is used to treat cancer that affects the adrenal cortex. It reduces the amount of adrenocorticoids produced by the adrenal cortex.
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.
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 through Cancer.Net’s Drug Information Resources, which provides links to searchable drug databases.
Radiation therapy
Radiation therapy is the use of high energy x-rays or other particles to kill cancer cells. 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.
Doctors and scientists are always looking for better ways to treat patients with an adrenal gland 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. 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 adrenal gland tumor. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with an adrenal gland tumor.
To join a clinical trial, patients must complete a learning 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. 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.
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.
Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with your doctor about side effects you experience during and after treatment. For more information on the most common side effects of a tumor and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects, based on ASCO’s curriculum.
In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient.
For more information on late effects or long-term side effects, please read the After Treatment section or talk with your doctor.
After treatment for an adrenal gland tumor ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years.
If the affected adrenal gland is still functional after surgery, both glands should be able to provide enough of the hormones needed by the body. Sometimes, a person needs hormone replacement therapy (such as steroids) because the gland that had the tumor will not be able to produce enough hormones as a healthy gland. Over time, the person can usually be weaned (gradually reduce the body’s dependence on) from the hormone replacement drug. An endocrinologist (a doctor who specializes in disorders of the endocrine system) should carefully monitor this process. The endocrine system is a complex collection of hormone-producing glands that control many essential functions. If both adrenal glands need to be removed, long-term medication is needed to replace the hormones normally produced by these glands, and you should wear a medical-alert bracelet to inform caregivers of your condition, in case you are unable to do so.
If the surgery is performed laparoscopically, the person can resume daily activities within a week. Usually, the person leaves the hospital in two to three days. If a larger incision is necessary, then the person is usually hospitalized for up to five to seven days and should not lift heavy objects for six weeks.
Follow-up care may include imaging and blood/urine tests every year. For some people with an adrenal gland tumor, the risk of developing another tumor in the other gland is about 10%. Because of this, people treated for an adrenal gland tumor may need regular check-ups with an endocrinologist to monitor recovery and detect any new tumor growth. Adrenal cortical carcinoma can be very aggressive, and because it is rare, a person may want to enroll in a clinical trial at a medical center with experience treating this type of tumor and follow-up care.
People recovering from an adrenal gland tumor are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about Healthy Living After Cancer.
Research for adrenal gland 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.
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. The effectiveness of immunotherapy as a treatment for an adrenal gland tumor is being researched in clinical trials.
Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:
What type of adrenal gland tumor do I have?
Is the tumor noncancerous or cancerous?
Is the tumor functional or nonfunctional? What does this mean?
Is the tumor located only in the adrenal gland?
Can you explain my pathology report (laboratory test results) to me?
What is my prognosis?
What are my treatment options?
What clinical trials are open to me?
Is surgery required? If so, what type of surgery?
How experienced is the surgeon with this type of surgery?
Are there radiation therapy and surgery options to treat my cancer?
Can chemotherapy control my cancer?
What is the goal of each treatment?
What treatment, or combination of treatments, do you recommend? Why?
What are the possible side effects of each treatment, both in the short term and long term?
How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
Will I need hormone replacement therapy? For how long?
What are the chances the tumor could recur (come back after treatment)?
What follow-up tests will I need, and how often will I need them?
What support services are available to me? To my family?
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