Adrenocortical Carcinoma

What is adrenocortical carcinoma?

Adrenocortical carcinoma is cancer that forms in the outer layer (cortex) of the adrenal glands. There are two adrenal glands, one located at the top of each kidney.

The adrenal cortex makes hormones — including cortisol, testosterone, aldosterone and estrogen. These hormones control male and female traits and body functions such as blood pressure and response to stress.

An adrenocortical carcinoma (ACC) is a cancerous adrenal tumor that forms in the adrenal cortex. There are two types of ACC:

• Functioning ACC tumor: A functioning ACC tumor causes your adrenal gland to make more hormones than it normally should.
• Nonfunctioning ACC tumor: This type of tumor doesn’t affect hormone production.

How common is adrenocortical carcinoma?

Adrenocortical carcinoma is very rare. Doctors diagnose only about one case per 1 million people each year. ACC affects more women than men.

Who is at risk of getting adrenocortical carcinoma?

People with certain inherited genetic conditions have a higher risk of developing adrenal cancer. In about 50% of adrenocortical carcinoma cases, the disease runs in families. Genetic conditions that increase ACC risk include:

• Beckwith-Wiedemann syndrome.
• Carney complex.
• Familial adenomatous polyposis (FAP).
• Li-Fraumeni syndrome.
• Lynch syndrome.
• Multiple endocrine neoplasia (MEN1).
• Neurofibromatosis Type 1 (NF1).
• Von Hippel-Lindau (VHL) syndrome.

What causes adrenocortical carcinoma?

Researchers don’t know exactly why some people develop cancerous adrenal tumors. People born with certain genetic conditions have a higher risk of the disease.

Gene mutations (changes) can also occur spontaneously, without being inherited. Experts don't fully understand how genetic changes that cause adrenocortical carcinoma occur.

What are the symptoms of adrenocortical carcinoma?

Adrenal tumors cause symptoms in two ways:

• A functioning ACC can cause symptoms related to the overproduction of hormones.
• If the adrenal tumor grows large enough, it may press on nearby organs. This pressure can cause pain or other symptoms.

Depending on the cause, symptoms of adrenocortical carcinoma may include:

• Abdominal (belly) pain.
• Breast enlargement in boys or men (gynecomastia).
• Early puberty in boys or girls.
• Growth of excess facial or body hair in women.
• High blood pressure.
• High blood sugar.
• Weight gain in the face, neck and trunk (but not arms and legs).

What are the complications of adrenocortical carcinoma?

Without treatment, adrenal tumors can continue to grow. As they get bigger, tumors may press against other organs. This pressure can affect how well those organs work or cause other symptoms.

Functioning adrenocortical carcinomas will continue to overproduce hormones that control various body functions. Different hormones produce different symptoms.

Too much cortisol can lead to Cushing’s syndrome, with symptoms including:

• High blood pressure.
• High blood sugar.
• Weight gain in the face, neck and trunk.
• A lump of fat between the shoulder blades.
• Hair growth on your face and body.

Too much aldosterone can cause Conn’s syndrome, with symptoms including:

• Excessive thirst.
• High blood pressure.
• Cramps or muscle weakness.
• Frequent need to urinate.

Too much testosterone in women can cause:

• Hair growth on your face and body.
• Balding.
• Deepening voice.
• Lack of menstrual periods.

Too much estrogen in women can cause:

• Irregular periods.
• Bleeding after menopause.
• Weight gain.

Too much estrogen in men can cause:

• Low sex drive.
• Erectile dysfunction.
• Breast growth.

How is adrenocortical carcinoma diagnosed?

Your healthcare provider will start with a physical exam and ask about any symptoms you’re experiencing. If your symptoms point to adrenal cancer, you may have:

• Imaging tests, such as MRI, CT scan or PET scan to look for tumors.
• Blood and urine tests to check hormone levels.
• A dexamethasone suppression test to measure cortisol levels.
• Biopsy to determine if a tumor is cancerous.

What are the stages of adrenal cancer?

If a biopsy reveals that the adrenal tumor is cancerous, your healthcare providers will determine the extent of the disease. They will measure the tumor to learn if cancer cells have spread beyond the adrenal gland to other parts of your body. This process is called staging.

• Early adrenal cancer: In stage 1 or stage 2, the tumor is still relatively small and hasn’t spread beyond the adrenal gland.
• Advanced adrenal cancer: Stage 3 and stage 4 adrenal cancer means the tumor has spread to other parts of your body. Usually, cancer cells spread first to nearby lymph nodes. Advanced cancer can also spread to other organs, tissues, blood vessels and bones.

How is adrenocortical carcinoma managed or treated?

The first treatment is usually surgery to remove the tumor. Your surgeon will remove your adrenal gland and may need to remove other nearby tissue, too.

To stop cancerous cells from spreading to other areas of your body, your treatment plan may include:

• Radiation.
• Chemotherapy.
• Immunotherapy.

Can adrenocortical carcinoma be prevented?

Experts know very little about what causes adrenal cancer. There are currently no recommendations for how to prevent it.

Adrenocortical carcinoma often runs in families. If one family member has the disease, everyone in the family should have genetic testing. If you have an increased risk of ACC because of an inherited condition, ask your provider about regular screening for early detection.

What is the prognosis (outlook) for people with adrenocortical carcinoma?

Prognosis depends on the tumor’s location and size, and whether cancer has spread beyond the adrenal glands. The prognosis is better in people who are younger or when the tumor is small.

In some cases, completely removing the tumor with surgery can cure cancer. Tumors that have metastasized (spread to other areas of the body) are harder to treat. New treatments continue to improve survival rates for people with adrenocortical carcinoma.

When should I call the doctor?

You should call your healthcare provider if you experience:

• Abdominal pain.
• Feeling of fullness in your abdomen.
• Symptoms of hormonal imbalances.

What questions should I ask my doctor?

You may want to ask your healthcare provider:

• Should my family or I have genetic testing?
• What’s the best treatment based on the stage of my tumor?
• Will the symptoms related to hormone overproduction go away when the tumor is treated?
• Are there any clinical trials for adrenocortical carcinoma treatment I should consider?

A note from Cleveland Clinic

Adrenocortical carcinoma is a very rare type of cancer that forms in the outer lining of the adrenal glands. When diagnosed early, a combination of surgery, radiation or chemotherapy often provides a cure. More advanced cases can be difficult to treat, but new therapies continue to improve survival rates.