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 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.

Having certain genetic conditions increases the risk of developing 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. 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.

Possible signs of adrenocortical carcinoma include pain in the abdomen and certain physical changes.

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.