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Adrenocortical Carcinoma Treatment (PDQ®)
Topics in This Section
- General Information About Adrenocortical Carcinoma
- Stages of Adrenocortical Carcinoma
- Recurrent Adrenocortical Carcinoma
- Treatment Option Overview
- Treatment Options by Stage
- Stage I Adrenocortical Carcinoma
- Stage II Adrenocortical Carcinoma
- Stage III Adrenocortical Carcinoma
- Stage IV Adrenocortical Carcinoma
- Treatment Options for Recurrent Adrenocortical Carcinoma
- Get More Information From NCI
- Changes to This Summary (07/18/2007)
- About PDQ
There are two adrenal glands. The adrenal glands are small and shaped like a triangle. One adrenal gland sits on top of each kidney. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla.
The adrenal cortex makes important hormones that:
- Balance the water and salt in the body.
- Help keep blood pressure normal.
- Help manage the body's use of protein, fat, and carbohydrates.
- Cause the body to have masculine or feminine characteristics.
The adrenal medulla makes hormones that help the body react to stress.
Adrenocortical carcinoma is also called cancer of the adrenal cortex. A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make hormones). The hormones made by functioning tumors may cause certain signs or symptoms of disease.
Cancer that forms in the adrenal medulla is called pheochromocytoma. See the PDQ summary on Pheochromocytoma Treatment for more information.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for adrenocortical carcinoma include having the following hereditary diseases:
- Li-Fraumeni syndrome.
- Beckwith-Wiedemann syndrome.
- Carney complex.
These and other symptoms may be caused by adrenocortical carcinoma:
- A lump in the abdomen.
- Pain the abdomen or back.
A nonfunctioning adrenocortical tumor may not cause symptoms in the early stages.
A functioning adrenocortical tumor makes too much of a certain hormone (cortisol, aldosterone, testosterone, or estrogen). Having too much of a certain hormone may cause the following symptoms:
Too much cortisol
- Weight gain in the face, neck, and trunk of the body and thin arms and legs.
- Growth of fine hair on the face, upper back, or arms.
- A round, red, full face.
- A lump of fat on the back of the neck.
- A deepening of the voice and swelling of the sex organs or breasts in both males and females.
- Muscle weakness.
- High blood sugar.
- High blood pressure.
Too much aldosterone
- High blood pressure.
- Muscle weakness or cramps.
- Frequent urination.
- Feeling thirsty.
Too much testosterone (in women)
- Growth of fine hair on the face, upper back, or arms.
- Acne.
- Balding.
- A deepening of the voice.
- No menstrual periods.
Men who make too much testosterone do not usually have symptoms.
Too much estrogen (in women)
- Irregular menstrual periods in women who have not gone through menopause.
- Menstrual bleeding in women who have gone through menopause.
Too much estrogen (in men)
- Growth of breast tissue.
- Lower sex drive.
- Impotence.
These and other symptoms may be caused by adrenocortical carcinoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of these problems occur.
The tests and procedures used to diagnose adrenocortical carcinoma depend on the patient's symptoms. The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of cortisol or 17-ketosteroids. A higher than normal amount of these in the urine may be a sign of disease in the adrenal cortex.
- Low-dose dexamethasone suppression test: A test in which one or more small doses of dexamethasone is given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days.
- High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone is given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days.
- Blood chemistry study: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as potassium or sodium, released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
- Blood tests: Tests to measure the levels of testosterone or estrogen in the blood. A higher than normal amount of these hormones that may be a sign of adrenocortical carcinoma.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). An MRI of the abdomen is done to diagnose adrenocortical carcinoma.
- Adrenal angiography: A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.
- Adrenal venography: A procedure to look at the adrenal veins and the flow of blood near the adrenal gland. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
- PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
The prognosis (chance of recovery) and treatment options depend on the following:
- The stage of the cancer (the size of the tumor and whether it is in the adrenal gland only or has spread to other places in the body).
- Whether the tumor can be completely removed in surgery.
- Whether the cancer has been treated in the past.
- The patient's general health.
Adrenocortical carcinoma may be cured if treated at an early stage.
The process used to find out if cancer has spread within the adrenal gland or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process:
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen or chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- MRI (magnetic resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium may be injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- Adrenal angiography: A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.
- Adrenal venography: A procedure to look at the adrenal veins and the flow of blood near the adrenal gland. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
- Cavagram: A procedure to look at the inferior vena cava and the flow of blood through the inferior vena cava. A contrast dye is injected into a blood vessel. As the contrast dye moves through the blood vessel to the inferior vena cava, a series of x-rays are taken to see if there are any changes to the inferior vena cava and the flow of blood through the inferior vena cava.
- Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the vena cava, and make echoes. The echoes form a picture of body tissues called a sonogram.
- Adrenalectomy: A procedure to remove the entire adrenal gland. A tissue sample is viewed under a microscope by a pathologist to check for signs of cancer.
In stage I, the tumor is 5 centimeters or smaller and is found only in the adrenal gland.
In stage II, the tumor is larger than 5 centimeters and is found only in the adrenal gland.
In stage III, the tumor can be any size and may have spread to fat or lymph nodes near the adrenal gland.
In stage IV, the tumor can be any size and has spread:
- to fat or organs and to lymph nodes near the adrenal gland; or
- to other parts of the body. Adrenocortical carcinoma commonly spreads to the lung, liver, bones, and peritoneum (the tissue that lines the abdominal wall and covers most of the organs in the abdomen).
Recurrent adrenocortical carcinoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the adrenal cortex or in other parts of the body.
Different types of treatments are available for patients with adrenocortical carcinoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
Surgery to remove the adrenal gland (adrenalectomy) is often used to treat adrenocortical carcinoma. Sometimes the nearby lymph nodes are also removed.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Mitotane may be used to treat adrenocortical carcinoma. Mitotane stops the adrenal cortex from making hormones and relieves symptoms caused by the hormones.
Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Treatment of stage I adrenocortical carcinoma is usually surgery (adrenalectomy). Lymph nodes may be removed if they are larger than normal.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I adrenocortical carcinoma.
Treatment of stage II adrenocortical carcinoma is usually surgery (adrenalectomy). Lymph nodes may be removed if they are larger than normal.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II adrenocortical carcinoma.
Treatment of stage III adrenocortical carcinoma may include the following:
- Surgery (adrenalectomy with or without removal of lymph nodes).
- A clinical trial of radiation therapy for tumors that cannot be removed by surgery.
- A clinical trial of mitotane for tumors that cannot be completely removed by surgery.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III adrenocortical carcinoma.
Treatment of stage IV adrenocortical carcinoma may include the following as palliative therapy to relieve symptoms and improve the quality of life:
- Mitotane therapy.
- Radiation therapy to bones where cancer has spread.
- Chemotherapy followed by surgery to remove the tumor.
- Surgery to remove the tumor.
- Surgery to remove cancer from places where it has spread.
- A clinical trial of chemotherapy or biologic therapy.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV adrenocortical carcinoma.
Treatment of recurrent adrenocortical carcinoma may include the following as palliative therapy to relieve symptoms and improve the quality of life:
- Surgery to remove the tumor.
- Surgery to remove the cancer from places where it has spread.
- A clinical trial of chemotherapy or biologic therapy.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent adrenocortical carcinoma.
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The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
PDQ contains cancer information summaries.
The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.
The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.
Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.
PDQ also contains information on clinical trials.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.