Definition of Adrenocortical carcinoma:

Adrenocortical carcinoma is a cancer of the adrenal glands.

Causes, incidence, and risk factors:

Adrenocortical carcinoma is most common in children younger than 5 and adults in their 30s and 40s.

Adrenocortical carcinoma may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor.

Adrenocortical carcinoma can produce the hormones cortisol, aldosterone, estrogen, or testosterone, as well as other hormones. In women the tumor often releases these hormones, which can lead to male characteristics.

The cause is unknown. About 2 people per million develop this type of tumor.

Symptoms:

Symptoms that suggest increased cortisol or other adrenal gland hormone production:

• Fatty, rounded hump high on the back just below the neck (buffalo hump)
• Flushed rounded face with pudgy cheeks (moon face)
• Obesity
• Stunted growth in height (short stature)
• Virilization - the appearance of male characteristics, including increased body hair (especially on the face), pubic hair, acne, deepening of voice, and enlarged clitoris (girls)

Symptoms that suggest increased aldosterone production are the same as symptoms of low potassium and include:

• Increased thirst
• Muscle cramps
• Urination
• Weakness

Signs and tests:

A physical exam may reveal high blood pressure and changes in body shape, such as breast enlargement in men (gynecomastia) or male characteristics in women (virilization). Blood tests will be done to check hormone levels:

• ACTH level will be low.
• Aldosterone level will be high.
• Cortisol level will be high.
• Potassium level will be low.

Imaging tests may include:

• Abdominal x-ray
• CT scan of the abdomen
• MRI of the abdomen

Treatment:

Primary treatment is surgery to remove the tumor. Adrenocortical carcinoma may not improve with chemotherapy. Medications may be given to reduce production of cortisol, which causes many of the symptoms.

Expectations (prognosis):

The outcome depends on how early the diagnosis is made and whether the tumor has spread (metastasized). Tumors that have spread usually lead to death within 1 to 3 years.

Complications:

The tumor can spread to the liver, bone, lung, or other areas.

Calling your health care provider:

Call your health care provider if you or your child has symptoms of adrenocortical carcinoma, Cushing syndrome, or failure to grow.