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Unusual Cancers of Childhood (PDQ®): Treatment - Patient Information [NCI] - Unusual Cancers of the Abdomen

Cancer of the Adrenal Cortex

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. Cancer of the adrenal cortex is also called adrenocortical carcinoma.

Childhood cancer of the adrenal cortex occurs most commonly in patients younger than 6 years or in the teen years, and more often in females.

The adrenal cortex makes important hormones that do the following:

  • 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 male or female characteristics.

Risk Factors, Symptoms, and Diagnostic and Staging Tests

The risk of cancer of the adrenal cortex is increased by having any of the following syndromes:

  • Li-Fraumeni syndrome.
  • Beckwith-Wiedemann syndrome.
  • Hemihypertrophy.

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 and these depend on the type of hormone made by the tumor. For example, extra androgen hormone may cause both male and female children to develop masculine traits, such as body hair or a deep voice, grow faster, and have acne. (See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information on the symptoms of cancer of the adrenal cortex.)

The tests and procedures used to diagnose and stage adrenocortical carcinoma depend on the patient's symptoms. They may include:

  • Physical exam and history.
  • Blood chemistry studies.
  • CT scan.
  • MRI.
  • PET scan.
  • Biopsy.

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose cancer of the adrenal cortex include the following:

  • 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 substances in the urine may be a sign of disease in the adrenal cortex.
  • Low-dosedexamethasone 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 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.
  • 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 glands. 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.
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