You have two adrenal glands, one at the top of each kidney. The outer layer of the adrenal gland is the adrenal cortex. The adrenal cortex produces hormones that control body functions and male and female traits. Cancerous tumors that form in the adrenal cortex are called 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:
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Adrenocortical carcinoma is very rare. Doctors diagnose only about one case per 1 million people each year. ACC affects more women than men.
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:
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.
Adrenal tumors cause symptoms in two ways:
Depending on the cause, symptoms of adrenocortical carcinoma may include:
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:
Too much aldosterone can cause Conn’s syndrome, with symptoms including:
Too much testosterone in women can cause:
Too much estrogen in women can cause:
Too much estrogen in men can cause:
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:
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.
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:
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.
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.
You should call your healthcare provider if you experience:
You may want to ask your healthcare provider:
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.
Last reviewed by a Cleveland Clinic medical professional on 09/09/2021.
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