Adrenocortical carcinoma is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland.

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 control the body's use of protein, fat, and carbohydrates.
• Cause the body to have masculine or feminine characteristics.

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 more hormones than normal). Most adrenocortical tumors are functioning. The hormones made by functioning tumors may cause certain signs or symptoms of disease.

The adrenal medulla makes hormones that help the body react to stress. Cancer that forms in the adrenal medulla is called pheochromocytoma and is not discussed in this summary. For more information, see Pheochromocytoma and Paraganglioma.

Adrenocortical carcinoma and pheochromocytoma can occur in both adults and children. Treatment for children, however, is different than treatment for adults. For more information, see Childhood Adrenocortical Carcinoma Treatment and Childhood Pheochromocytoma and Paraganglioma Treatment.

Having certain genetic conditions increases the risk of adrenocortical carcinoma.

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. Talk with your doctor if you think you may be at risk.

Risk factors for adrenocortical carcinoma include having the following hereditary diseases:

• Li-Fraumeni syndrome.
• Beckwith-Wiedemann syndrome.
• Carney complex.

Symptoms of adrenocortical carcinoma include pain in the abdomen.

These and other signs and symptoms may be caused by adrenocortical carcinoma:

• A lump in the abdomen.
• Pain the abdomen or back.
• A feeling of fullness in the abdomen.

A nonfunctioning adrenocortical tumor may not cause signs or symptoms in the early stages.

A functioning adrenocortical tumor makes too much of one of the following hormones:

• Cortisol.
• Aldosterone.
• Testosterone.
• Estrogen.

Too much cortisol may cause:

• 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 may cause:

• High blood pressure.
• Muscle weakness or cramps.
• Frequent urination.
• Feeling thirsty.

Too much testosterone (in women) may cause:

• 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 signs or symptoms.

Too much estrogen (in women) may cause:

• Irregular menstrual periods in women who have not gone through menopause.
• Vaginal bleeding in women who have gone through menopause.
• Weight gain.

Too much estrogen (in men) may cause:

• Growth of breast tissue.
• Lower sex drive.
• Impotence.

These and other signs and symptoms may be caused by adrenocortical carcinoma or by other conditions. Check with your doctor if you have any of these problems.

Imaging studies and tests that examine the blood and urine are used to diagnose adrenocortical carcinoma.

The tests and procedures used to diagnose adrenocortical carcinoma depend on the patient's signs and symptoms. The following tests and procedures may be used:

• Physical exam and health 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 are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol.
• High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol or if the pituitary gland is telling the adrenal glands to make too much cortisol.
• 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.
• 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 glands. A contrast dye is injected into the adrenal arteries. As the dye moves through the arteries, 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 glands. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the veins, 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.
• MIBG scan: A very small amount of radioactive material called MIBG is injected into a vein and travels through the bloodstream. Adrenal gland cells take up the radioactive material and are detected by a device that measures radiation. This scan is done to tell the difference between adrenocortical carcinoma and pheochromocytoma.
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The sample may be taken using a thin needle, called a fine-needle aspiration (FNA) biopsy or a wider needle, called a core biopsy.

Certain factors affect the prognosis (chance of recovery) and treatment options.

The prognosis 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.
• The grade of tumor cells (how different they look from normal cells under a microscope).

Adrenocortical carcinoma may be cured if treated at an early stage.