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Adrenocortical Carcinoma (ACC)

What is adrenocortical carcinoma?

Adrenocortical carcinoma, or ACC, is a cancer of the adrenal glands, which are two small triangular-shaped glands that sit on top of each kidney. The outside of these glands is called the adrenal cortex. The adrenal cortex makes important hormones that help your body control water balance, blood pressure, stress response, and cause the body to have male or female traits. ACCs form in the adrenal cortex.

An ACC may be functioning, which means it makes more hormone than normal, or non-functioning, which means it has no effect on hormone production. A functioning ACC tumor often makes too much of the hormones cortisol, aldosterone, testosterone, or estrogen.

How common is adrenocortical carcinoma?

ACC is very rare, affecting around one case diagnosed in one million people in the US. It is more common in females than males.

How is adrenocortical carcinoma diagnosed?

ACC can cause pain in the abdomen, high blood pressure, acne, overgrowth of hair, and voice deepening. Other symptoms of ACC are different for females and males, since it can change hormone levels. Females may have an overgrowth in female genitalia and facial hair. Males may have abnormal penis growth or early puberty changes like increased muscle growth and body hair.

Lab Tests: If you have symptoms of ACC, your doctor will order lab tests of your urine and blood to check your hormone levels.

Imaging: Your doctor will use imaging tests such as ultrasound, CT, X-ray, PET, and MRI scans to look at the size of the tumor and whether it has spread to other parts of your body.

Biopsy: To check if the tumor is ACC, your doctor may perform a biopsy, taking a small sample from the tumor with a needle. A pathologist will study cells from the sample under the microscope to see what kind of tumor it is.

ACC is rare but, another type of tumor in the adrenal glands, adrenocortical adenoma, is quite common. Adrenocortical adenoma is not as dangerous as ACC. It can be difficult to tell the difference between them because they are both found in the adrenal glands and the cells can look similar. Getting the correct diagnosis is very important to determine the best treatment.

How is adrenocortical carcinoma treated?

Surgery: Surgery is used to remove as much of the ACC as possible. Small ACCs are often cured with surgery.

Chemotherapy: When the ACC tumors are large, or the cancer cells have spread to other parts of the body, chemotherapy is used with surgery.

Does adrenocortical carcinoma run in families?

ACC runs in families 50% of the time. Genetic testing is recommended for all close relatives of people with ACC.
When you have ACC, you may have other conditions that increase your chance of getting cancer. Genetic testing helps determine if you or your family members are at risk of developing ACC and other diseases. Genetic counseling is often recommended to help you understand the risks to you and your family members.

How does adrenocortical carcinoma form?

Scientists are always working to understand how cancer forms but it can be hard to prove. Because ACC can run in families, we know that changes in genes linked to Li-Fraumeni Syndrome, Beckwith-Wiedemann Syndrome, and Carney complex are important in causing ACC.

What is the prognosis for someone with adrenocortical carcinoma?

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:

  • Where the tumor is in your body
  • If the cancer has spread to other parts of your body
  • How much of the tumor was taken out during surgery

If you want information on your prognosis, it is important to talk to your doctor. NCI also has resources to help you understand cancer prognosis.

Doctors estimate ACC survival rates by how groups of people with ACC have done in the past. Because there are so few people with ACC, these rates may not be very accurate. They also don't consider newer treatments being developed.

If the ACC is small when it is found, prognosis is good and cure is likely. If the ACC is already large or has spread to other parts of the body, treatment is more difficult and the five-year survival rate is 36% to 46%.