Adrenocortical carcinoma

Alternative names

Tumor - adrenal

Definition

Adrenocortical carcinoma is a malignant tumor of the adrenal glands.

Causes, incidence, and risk factors

Adrenocortical carcinomas can produce the hormones cortisol or aldosterone. The disease is most common in two age groups: children younger than 5 years old, 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 more often a functional tumor (secretes hormones).

The cause is unknown. The incidence is about 2 per million.

Symptoms

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

• 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
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)

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.