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, including response to stress and infection. The adrenal cortex is the outside layer of the two adrenal glands.

Cancers of the adrenal cortex are rare. The majority of these tumors produce excess hormones. The excess hormones can change hormonal balance.

Anatomy of the Adrenal Glands

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The cause is unknown.

Risk Factors

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

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

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. Excess hormones may produce symptoms such as:

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

  • Cushings syndrome
  • Conns syndrome
  • Virilization
  • A mixed Cushings syndrome and virilization
  • In young children with functional tumors that release sex hormones, these tumors may cause early onset of puberty

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

Your bodily fluids may be tested. This can be done with:

  • Blood tests
  • Urine tests

Your doctor may need detailed pictures of the body. These can be made with:

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 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.


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:

Adrenalectomy Surgery to Remove the Tumor

Surgery to remove the adrenal tumor is the first treatment approach for most cases. The doctor will also need to remove any surrounding tissues or lymph nodes that contain cancer cells.

These treatments tend to be less effective in treating this type of cancer. They are most often used if the cancer has spread. Chemotherapy may also be used if there is a high chance the cancer will come back.

Other Therapies
  • Mitotane is the drug most often used for this type of cancer, especially for hormonally active tumors. It blocks hormone production by the adrenal gland. It also destroys adrenal cancer cells. This drug does have serious side effects.
  • Other hormone blocking drugs may be given to control the symptoms of excess hormones.
  • Research of new drug and radiation therapies are under way. This includes gene and immunotherapy.

If treatment is successful, you will still need to be screened for reoccurrence of the cancer on a periodic basis.


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