Adrenocortical Carcinoma in Children

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. Adrenocortical carcinoma is also called cancer of the adrenal cortex.

Childhood adrenocortical carcinoma 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.

The risk of adrenocortical carcinoma is increased by having a certain mutation (change) in a gene or 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 extra hormones).

Most tumors of the adrenal cortex in children are functioning tumors. The extra 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.

Extra estrogen hormone may cause the growth of breast tissue in male children.

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.
• X-ray of the chest, abdomen, and bones.
• CT scan.
• MRI.
• PET scan.
• Biopsy (the mass is removed during surgery and then the sample is checked for signs of cancer).

Other tests used to diagnose adrenocortical carcinoma 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-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 hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones 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 in the organ or tissue that makes it. The blood may be checked for testosterone or estrogen. A higher than normal amount of these hormones 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.

Treatment of adrenocortical carcinoma in children may include the following:

• Surgery to remove the tumor with or without chemotherapy.

Treatment of recurrent adrenocortical carcinoma in children may include the following:

A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

The prognosis (chance of recovery) is good for patients who have small tumors that have been completely removed by surgery. For other patients, the prognosis depends on the following:

• Size of the tumor.
• Whether the tumor has spread to other parts of the body, including the lymph nodes.
• Child's age.
• Whether the covering around the tumor broke open during surgery to remove the tumor.
• Whether the child has developed masculine traits.
• These tumors can spread to the kidneys, lungs, bones, and brain.