Adrenocortical Carcinoma

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. They make up 0.5-2 cases per million. The majority of these tumors produce excess hormones. The excess can alter hormonal balance.

The cause is unknown.

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

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

Approximately 40% of adrenocortical carcinoma do not secrete any hormone. These do not have any specific symptoms. They are discovered either by accident or as part of an evaluation of abdominal pain.

Other tumors are functional. This means are hormonally active. Excess hormones may produce symptoms such as:

• Diabetes
• High blood pressure
• Weakening of the bones

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

• 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
• Conn’s syndrome (Hyperaldosteronism)—2% of cases—excess aldosterone, which helps the body maintain normal levels of sodium and potassium
• Cushing’s syndrome (Hypercortisolism)—30% of cases—excess cortisol which helps the body respond to stressful situations and infections
• 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.

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

Tests may include the following:

• Adrenal angiography and/or venography —tests that use contrast dye to help confirm the presence of tumors that may be blocking blood vessels; a blood sample may be obtained to check hormone levels
• Blood tests to look for hormones from the adrenal glands
• CT scan of the abdomen —a type of x-ray that uses a computer to make pictures of structures inside the abdomen
• MRI of the abdomen —a test that uses magnetic waves to make pictures of structures inside the abdomen
• PET/CT scan —a test that uses radioactive glucose to identify the location of tumors in the body
• Urine tests (24 hour cortisol)
• X-ray —a test that uses radiation to take a picture of structures inside the body

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

Further testing 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 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:

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