Adrenocortical cancer (also called cancer of the adrenal cortex) is rare. Certain inherited disorders increase the risk of adrenocortical cancer. Explore the links on this page to learn more about adrenocortical cancer treatment, research, and clinical trials.

Adrenocortical Carcinoma Treatment (Adult)

Adrenocortical carcinoma is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland.
Having certain genetic conditions increases the risk of adrenocortical carcinoma.
Symptoms of adrenocortical carcinoma include pain in the abdomen.
Imaging studies and tests that examine the blood and urine are used to detect (find) and diagnose adrenocortical carcinoma.
Certain factors affect the prognosis (chance of recovery) and treatment options.

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. See the PDQ summary on Pheochromocytoma and Paraganglioma for more information.

Adrenocortical carcinoma and pheochromocytoma can occur in both adults and children. Treatment for children, however, is different than treatment for adults. (See the sections on Cancer of Adrenal Cortex and Pheochromocytoma and Paraganglioma in the PDQ summary on Unusual Cancers of Childhood Treatment for more information.)

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.

Stages of Adrenocortical Carcinoma

After adrenocortical carcinoma has been diagnosed, tests are done to find out if cancer cells have spread within the adrenal gland or to other parts of the body.
There are three ways that cancer spreads in the body.
Cancer may spread from where it began to other parts of the body.
The following stages are used for adrenocortical carcinoma:

Stage I

In stage I, the tumor is 5 centimeters or smaller and is found in the adrenal gland only.

Stage II

In stage II, the tumor is larger than 5 centimeters and is found in the adrenal gland only.

Stage III

In stage III, the tumor is any size and has spread:

to nearby lymph nodes; or
to nearby tissues or organs (kidney, diaphragm, pancreas, spleen, or liver) or to large blood vessels (renal vein or vena cava) and may have spread to nearby lymph nodes.

Stage IV

In stage IV, the tumor is any size, may have spread to nearby lymph nodes, and has spread to other parts of the body, such as the lung, bone, or peritoneum.

Recurrent Adrenocortical Carcinoma

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.

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