Definition

Adrenocortical carcinoma is cancer of the adrenal cortex. The adrenal glands are two glands located just above each kidney. The glands produce important hormones. These hormones regulate heart rate, blood pressure, and many other vital functions. The adrenal cortex is the outside layer of the two adrenal glands.

Cancers of the adrenal cortex are very rare. It occurs in 0.5-2 cases per million. The majority of these tumors produce excess hormones. The excess hormones can change hormonal balance.

Anatomy of the Adrenal Glands

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Causes

The cause is unknown.

Risk Factors

There are few known risk factors for cancers of the adrenal cortex. But, the following factors may contribute:

• Sex: female
• Children under 5 years old
• Adults between 40 to 50 years old
• A genetic defect may cause adrenocortical cancer in some children, but the majority of cases are non-hereditary

Symptoms

Approximately 40% of adrenocortical carcinoma do not secrete any hormone. These people do not have any specific symptoms. Adrenocortical carcinoma is discovered either by accident or as part of a physical examination for abdominal pain.

Other tumors are hormonally active (functional). Excess hormones may produce symptoms such as:

• High blood pressure
• Weakening of the bones
• Diabetes

Other conditions that may result from functional tumors of the adrenal cortex include:

• Cushing’s syndrome (Hypercortisolism)—30% of cases—excess cortisol which helps the body respond to stressful situations and infections
• Conn’s syndrome (Hyperaldosteronism)—2% of cases—excess aldosterone, which helps the body maintain normal levels of sodium and potassium
• Virilization (20% of cases)—women with functional tumors that release males hormones; may deepen voice, cause hirsutism (growing excess hair on the face), and swelling of the sex organs or breasts may occur
• A mixed Cushing’s syndrome and virilization accounts for 35% of all cases
• In young children with functional tumors that release sex hormones, these tumors may cause early onset of puberty

These symptoms may be caused by other health conditions. Anyone experiencing these symptoms should see a doctor.

Diagnosis

Your doctor will ask about your symptoms, and medical and family history. A physical exam will be done.

Tests may include the following:

• Blood tests to look for hormones from the adrenal glands
• Urine tests (24 hour cortisol)
• Your doctor may need detailed pictures of the body. These can be made with:
  • CT scan of the abdomen
  • MRI of the abdomen
  • PET/CT scan
  • Adrenal angiography and/or venography
  • X-ray

After cancer of the adrenal cortex is confirmed, you will be referred to an oncologist. This type of doctor focuses on cancer.

Additional testing will be done to determine what stage (1-4) the cancer has reached. The stage of a tumor is determined by its size and how far it has spread from its point of origin. At the time of diagnosis, 30%-85% of patients are found to have cancer that has spread. The higher the stage, the more dangerous and difficult it is to treat.

Treatment

Treatment depends on the stage of the tumor and your overall health. Talk with your doctor about the best plan for you.

The following methods are used to treat cancer of the adrenal cortex:

Prevention

There are no known preventive measures. Healthy lifestyle choices may reduce your risk for cancers of all types.