Each person has two adrenal glands. An adrenal gland is located on top of each of the body’s two kidneys, and these glands are important to the body’s endocrine (hormonal) system. Each adrenal gland has two main parts, which function separately. The outer part is called the cortex, and the inner part is called the medulla. 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. 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 form a mass. A tumor can be benign (noncancerous) or malignant (cancerous). A tumor in an adrenal gland can sometimes overproduce hormones. When it does, the tumor is called a functioning tumor. A tumor in an adrenal gland that does not produce hormones is subsequently 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 will focus on primary adrenal gland tumors, which include:
Adenoma. The most common (99%) type of an adrenal gland tumor is an adenoma, a benign 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 exceedingly rare, the most common type of malignant adrenal gland tumor affects the cortex and is called adrenocortical carcinoma, or adrenal cortical carcinoma. Adrenocortical carcinoma can be a functioning or nonfunctioning tumor, and occurs in approximately 4 in 1 million to 12 in 1 million individuals. If the tumor is functioning, it may produce more than one hormone.
The symptoms and treatment of an adrenal gland tumor depend on several factors, such as whether the tumor is functioning or nonfunctioning, and specifically, what hormone(s) is overproduced, and whether the tumor is a primary adrenal gland tumor or metastases from a 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 300 to 500 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 frequently found among middle-age adults and older adults.
Cancer statistics should be interpreted with caution. These estimates are based on information 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. Because survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this cancer.
Statistics adapted from the American Cancer Society.
A risk factor is anything that increases a person's chance of developing cancer. 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 cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, 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 malignant adrenal cortical carcinoma is not known. However, people with certain hereditary conditions are at higher risk of developing adrenal gland tumors, including Li-Fraumeni syndrome and Carney Complex. People at high risk for 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)
Specifically for pheochromocytoma, this type of tumor may cause dangerous surges of 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 (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 tumor
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose an adrenal gland tumor:
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.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body.
Blood and urine tests. Blood tests can measure the amounts of natural hormones produced during stress, such as catecholamines and metanephrines, which can detect functional tumors. Additionally, a patient may be asked to take a pill on the evening before the lab tests; the purpose is to look for 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 allows the doctor to track the production rate of various hormones.
Metaiodobenzylguanidine (MIBG) scan. MIBG is a chemical similar to adrenaline that is drawn to a neuroendocrine tumor. A MIBG scan is a nuclear medicine scan that can specifically show a tumor of the adrenal medulla that may not appear x-rays. A MIBG scan takes place over two consecutive days. On the first day, an injection is given in the arm. Several hours later, pictures are taken with a special camera. 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 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. For a tumor that starts in an adrenal gland, proof of metastases usually suggests more aggressive disease.
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.
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 on the abdomen with small telescopes and long instruments. However, if the tumor is greater than 5 cm to 6 cm or possibly malignant, an open surgical approach through the abdomen is recommended. The operation can be performed through incision in the front (anterior) or back (posterior). The decision is usually made by the surgeon. If the patient has had abdominal surgery, the back (posterior) approach may be the easiest. Surgery is mostly used for 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 careful preparation of the patient with medication to eliminate high blood pressure during surgery is necessary.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. 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.
Mitotane (Lysodren) is a medication that is used to treat cancer that affects the adrenal cortex. Mitotane reduces the amounts of adrenocorticoids produced by the adrenal cortex.
The medications used to treat cancer are continually being evaluated. Talking with you doctor is often the best way to learn about the medications you've been prescribed, 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 in order 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 this 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.
Cancer 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 cancer 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 cancer, 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 cancer, 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 cancer and different treatments, along with ways to prevent or control them, visit Cancer.Net's section on Managing Side Effects.
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 adrenal gland tumor is a functional tumor, after surgical removal, the healthy gland should be able to work well enough to compensate. Sometimes, however, the person will need hormone replacement therapy (such as steroids) because the gland that had the tumor will not be able to perform at the same level as the healthy gland. Over time, the person can usually be weaned (gradually reduce the person's dependence on) from the hormone replacement drug. An endocrinologist (a doctor who specializes in disorders of the endocrine system) should carefully monitor this. The endocrine system is a complex collection of hormone-producing glands that control such functions as metabolism, growth, and sexual development. If both adrenal glands need to be removed, long-term medication will be needed to replace the normal function of these glands.
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 an open operation (a larger incision below the rib cage or midline) is necessary, then the person is usually hospitalized for five to seven days, and should not lift heavy objects for six weeks.
Follow-up care may include imaging scans and blood/urine studies every year. Some people with an adrenal gland tumor have a risk of about 10% of developing a tumor of the adrenal gland on the other side. Because of this, people treated for an adrenal gland tumor may want to follow up regularly with an endocrinologist in order 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 center with expertise in treating this type of tumor.
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 about adrenal gland tumors is ongoing. The following advance 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. Immunotherapy is being researched in clinical trials for its effectiveness in treating adrenal gland tumors.
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 my cancer limited to the adrenal gland?
What is the stage of the disease? What does this mean?
What are my treatment options?
Is surgery required? If so, what type of surgery?
How experienced is the surgeon in this type of surgery?
Are there radiation therapy and surgery options to treat my cancer? What does this mean?
Can chemotherapy control my cancer?
What is the goal of each treatment?
What clinical trials are open to me?
What treatment, or combination of treatments, do you recommend? Why?
What are the possible short-term and long-term side effects of each treatment?
What is my prognosis?
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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