Adrenocortical carcinoma

• Illustrations
• Alternative names
• Definition
• Causes, incidence, and risk factors
• Symptoms

• Signs and tests
• Treatment
• Expectations (prognosis)
• Complications
• Calling your health care provider

Alternative names    Return to top

Definition    Return to top

Adrenocortical carcinoma is a malignant tumor of the adrenal glands.

Causes, incidence, and risk factors    Return to top

Adrenocortical carcinomas can produce the hormones cortisol or aldosterone. The disease is most common in two age groups: children younger than 5, and adults in their 30s and 40s.

Adrenocortical carcinoma may be associated with an inherited cancer syndrome. Both men and women develop this tumor. In women it is often a functional tumor (one that secretes hormones).

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

Symptoms    Return to top

Findings suggestive of increased cortisol production:

• Flushed rounded face with pudgy cheeks (moon face)
• Obesity
• Buffalo hump: rounded fatty hump high on the back just below the neck
• Short stature: impaired linear growth, may be less than 5th percentile
• Virilization: increased body hair (hypertrichosis), especially on face, pubic hair, acne, deepening of voice, enlarged clitoris (girls)

Findings suggestive of increased aldosterone production are symptoms of low potassium (weakness, muscle cramps, increased thirst, and urination).

Signs and tests    Return to top

• High blood pressure
• High serum cortisol levels
• Low serum ACTH
• Low serum potassium
• High serum aldosterone
• CT scan of the abdomen (may demonstrate a mass in the adrenal gland)
• Abdominal X-ray
• Abdominal MRI (may demonstrate a mass in the adrenal gland)

Treatment    Return to top

Primary treatment consists of surgical removal of the tumor. Adrenocortical carcinoma may not respond well to chemotherapy. Medications may be given to reduce production of cortisol which is responsible for many of the symptoms.

Expectations (prognosis)    Return to top

The outcome is dependent on how early the diagnosis is made and whether the tumor has spread (metastasized). Metastatic tumors are often fatal within a few years.

Complications    Return to top

A complication is metastasis (frequently to the liver, bone, and lung).

Calling your health care provider    Return to top

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