Adrenal Gland Tumor

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 that function separately:

Adrenal cortex. The outer part of the adrenal gland is called the cortex. The adrenal cortex makes three main 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 normal cells change 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. The symptoms and treatment of an adrenal gland tumor depend on whether the tumor is functioning or nonfunctioning, what hormone(s) is overproduced, and whether the tumor is a primary adrenal gland tumor or a metastases from cancer of another organ.

This section focuses on primary adrenal gland tumors, which include the following:

Adenoma. Adenoma is the most common type of adrenal gland tumor, making up the majority 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 need treatment.

Adrenocortical carcinoma. Although rare, the most common type of cancerous adrenal gland tumors begins in the cortex and is called adrenocortical carcinoma, or adrenal cortical carcinoma. Approximately four to 12 out of one million people 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.

Neuroblastoma. This is a type of childhood cancer that can begin in the adrenal medulla. Learn more about childhood neuroblastoma.

Pheochromocytoma. This type of neuroendocrine tumor most often begins in the adrenal medulla. Learn more about pheochromocytoma

A primary adrenal gland tumor is very uncommon, and exact statistics are not available for this type of tumor 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 than men tend to be diagnosed with adrenal gland tumors.

Statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of tumor 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.

Symptoms and Signs

People with an adrenal gland tumor may experience the following symptoms or signs. 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 or sign 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 or 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 very quickly, increasing the risk of heart attack, stroke, hemorrhage, or sudden death.

Stages

Staging is a way of describing where the tumor is located, if it is cancerous, if or where it has spread, and whether 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. This section covers the standard staging system for adrenocortical carcinoma.

One tool that doctors use to describe the stage is the TNM system. This system judges three factors: the tumor itself, the lymph nodes around the tumor, and whether the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person. There are four stages for adrenocortical carcinoma: 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 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. Specific tumor stage information is listed below.

TX: The primary tumor cannot be evaluated.

T0: There is no primary tumor.

T1: The tumor is 5 centimeters (cm) or less and has not grown outside of the adrenal gland.

T2: The tumor is larger than 5 cm and has not grown outside of the adrenal gland.

T3: The tumor is any size, and it has grown into the area around the adrenal gland but not to nearby organs.

T4: The tumor is any size and has grown into nearby organs, such as the kidney; diaphragm (the
thin muscle under the lungs and heart that separates the chest from the abdomen); larger blood vessels, called the aorta and the vena cava; pancreas; spleen; and liver. 

Node. The “N” in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs that help fight infection. Lymph nodes near where the tumor started are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.

NX: The regional lymph nodes cannot be evaluated.

N0 (N plus zero): The cancer has not spread to the regional lymph nodes.

N1: The cancer has spread to regional lymph nodes. 

Distant metastasis. The “M” in the TNM system indicates whether the cancer has spread to other parts of the body.

M0 (M plus zero): The cancer has not spread to other parts of the body.

M1: The cancer has spread to other parts of the body beyond the nearby organs.

Cancer stage grouping

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage I: The tumor is 5 cm or less and has not grown beyond the adrenal gland or spread to regional lymph nodes or other parts of the body (T1, N0, M0). 

Stage II: The tumor is larger than 5 cm. It has not grown beyond the adrenal gland or spread to regional lymph nodes or other parts of the body (T2, N0, M0).

Stage III: The tumor is described by the following:

• It is 5 cm or smaller and has spread to the regional lymph nodes but not to other parts of the body (T1, N1, M0).
• It is larger than 5 cm and has spread to the regional lymph nodes but not to other parts of the body (T2, N1, M0).
• It is any size and has grown beyond the adrenal gland but not to nearby organs (T3, N0, M0).    

Stage IV: The tumor is described by the following:

• It is any size and has grown into the area around the adrenal gland but not to nearby organs. The tumor has spread to regional lymph nodes but not to other parts of the body (T3, N1, M0).
• It is any size and has spread to nearby organs but not to the lymph nodes or other parts of the body beyond the nearby organs (T4, N0, M0).
• It is any size and has spread to nearby organs. The tumor has spread to the regional lymph nodes but not to other parts of the body beyond the nearby organs (T4, N1, M0).
• The tumor has spread to other parts of the body (any T, any N, M1).

Treatment

For an adrenal gland tumor, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called amultidisciplinary team.

Descriptions of the most common treatment options for an adrenal gland tumor are listed below. Treatment options and recommendations depend on several factors, including the type and stage of the tumor, possible side effects, and the patient’s preferences and overall health. Learn more about making treatment decisions.

Up to 25% of adrenal tumors may be linked to a genetic syndrome (see Risk Factors). When planning treatment, it may be helpful to consult a genetic counselor to learn whether the tumor is associated with a specific syndrome and whether other family members should undergo specific genetic tests. Learn more about genetic testing.

Surgery

Surgery is the removal of the tumor and surrounding tissue during an operation. Adrenalectomy, which is the surgical removal of the adrenal gland with the tumor, may be needed to treat an adrenal gland tumor. A surgical endocrinologist is a surgeon who specializes in treating an endocrine tumor using surgery.

If the tumor is smaller than 5 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 uses a lighted, flexible, tube instrument and makes small incisions in the skin; the technique minimizes pain and post-operative recovery time compared with traditional incisions. It may be performed from the abdomen or back, depending on the location of the tumor and the experience of the surgeon.

However, if the tumor is possibly cancerous or larger than 5 cm to 6 cm, surgery using one large incision in the abdomen or back is recommended. If the patient has previously had abdominal surgery, an incision in the back may be easier.

Surgery is the main treatment for a pheochromocytoma. Learn more about treatment for a neuroendocrine tumor.

If the surgery is performed laparoscopically, the person can usually leave the hospital in two to three days and resume daily activities within a week. If a larger incision is needed, then the person is usually hospitalized for up to five to seven days and should not lift heavy objects for six weeks after surgery.

Adrenal surgery can cause bleeding. The tumor can also make excess catecholamines (stress hormones), and the patient will need to be monitored and receive medication to treat high blood pressure during surgery.

Learn more about surgery.

Hormone therapy

Because adrenal gland tumors may produce excess hormones, the doctor may prescribe various medications to control the levels of these hormones before, during, or after other treatments. For example, if the tumor is producing excess catecholamines, the patient may need a drug to lower the levels of these body chemicals before additional treatment can be given. Or, after surgery, a patient may need medication to block the effects of excess cortisol, a natural steroid that may be produced by the tumor. These drugs include spironolactone (Aldactone), metyrapone (Metopirone), metyrosine (Demser), and streptozin (Zanosar).

Chemotherapy

Chemotherapy is the use of drugs to kill tumor cells, usually by stopping the cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach tumor cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating a tumor with medication. 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.

Mitotane (Lysodren) is a medication used to treat cancer in 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 they 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 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 (schedule) usually consists of a specific number of treatments given over a set period of time. Learn more about radiation therapy.

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.

Palliative/supportive care

A tumor and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the tumor, an 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.

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.   

Recurrent adrenal gland tumor

A remission is when cancer 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 a tumor will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the tumor returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if it does return. Learn more aboutcoping with the fear of recurrence.

If the tumor does return after the original treatment, it is called a recurrent tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence). 

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as surgery, chemotherapy, and radiation therapy) but 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 adrenal gland 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.

Metastatic adrenal gland tumor

If an adrenal gland 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 studying new treatments.

Your health care team may recommend a treatment plan that includes a combination of surgery, radiation therapy, and chemotherapy. Supportive care will also be important to help relieve symptoms and side effects.

For many patients, a diagnosis of metastatic cancer can be 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.