Adrenal cortical carcinoma (ACC)

This page was reviewed under our medical and editorial policy by

Katherine Poruk, MD, Surgical Oncologist

This page was reviewed on December 2, 2021.

When cancerous cells develop in the cortex of adrenal glands, it’s known as adrenal cortical carcinoma (ACC), or adrenocortical carcinoma. ACC is a rare type of cancer that may not be discovered until it grows large enough to cause symptoms.

Most ACC tumors are functioning, meaning they increase hormone production to higher-than-normal rates. However, some are nonfunctioning, meaning there’s no increase in hormone production. Symptoms of ACC often result when functioning tumors release excess hormones.

Your adrenal glands are located on top of each of your kidneys, in the innermost area of the abdominal region. They consist of:

  • Outer adrenal cortex, which produces the hormones cortisol, aldosterone and adrenal androgens
  • Inner adrenal medulla, which produces the nervous system hormone adrenaline

These hormones perform a host of functions throughout your body, including:

  • Help balance your body’s water and sodium levels
  • Keep your blood pressure in control
  • Regulate how your body uses carbohydrates, protein and fat
  • Determine whether your body has masculine or feminine features

ACC symptoms

In general, if you have an ACC tumor, you may experience:

  • Pain in your abdomen or back
  • Noticeable abdominal lump
  • Feeling of fullness in the region

While a nonfunctioning ACC tumor may not cause early symptoms, a functioning ACC tumor causes different symptoms depending on the type of hormone it produces, including cortisol, aldosterone, testosterone or estrogen. If you experience any of the following symptoms, consult with your doctor.

If it produces excess cortisol, you may:

  • Gain weight in your face, neck and torso, while your arms and legs remain thin
  • Notice your face becoming red, round and full
  • Develop a fatty lump behind your neck
  • Note a deepening of your voice and swelling of your genitals or breasts, regardless of gender
  • Experience muscle weakness
  • Develop high blood sugar or high blood pressure

If it produces excess aldosterone, you may:

  • Experience high blood pressure
  • Feel thirsty or urinate often
  • Experience cramps or weakness in your muscles

If it produces excess testosterone in women, symptoms may include:

  • Hair on the face, back or arms
  • Acne
  • Balding
  • Deepening voice
  • Ceasing of menstrual cycle

If it produces excess estrogen in men, symptoms may include:

  • Increase in breast tissue
  • Decrease in sex drive and/or impotence

If it produces excess estrogen in women, symptoms may include:

  • Irregular menstrual cycle for those who’ve not yet gone through menopause
  • Vaginal bleeding in those who’ve gone through menopause
  • Weight gain

ACC causes

The exact cause of ACC is unknown, but research has found that changes to your DNA may result in adrenal gland cells becoming cancerous. DNA mutations to oncogenes, which are responsible for cell division and growth, and tumor suppressor genes, which normally inhibit cancerous cells from dividing and/or cause them to die, may result in these genes not working properly, allowing cancer to grow.

ACC risk factors

Although ACC tumors may develop without a known cause, inherited genetic mutations are thought to account for at least 50 percent of these cancers, according to the Genetic and Rare Diseases Information Center. Certain rare inherited diseases may put you at increased risk for ACC, though having one doesn’t guarantee you’ll develop it. These inherited disorders include:

  • Li-Fraumeni syndrome (LFS), caused by mutations to the TP53 gene
  • Beckwith-Wiedemann syndrome, which includes larger-than-normal features and other symptoms such as increased cancer risk
  • Carney complex, caused by genetic mutations characterized by dark marks on the skin, as well as tumors in the skin, endocrine glands, nerves and heart

ACC diagnosis

In order to diagnose ACC, a doctor will consider your symptoms and personal health history and conduct a physical examination to inspect any abnormalities, such as masses and other signs of cancer.

Additionally, you may undergo one or more of the following tests.

  • 24-hour urine test: Urine is collected over a 24-hour period to measure levels of certain adrenal hormones and other compounds. High amounts may indicate ACC.
  • Low-dose dexamethasone suppression test: After being given a small dose (or doses) of dexamethasone, the level of cortisol is measured from blood or urine collected over three days.
  • High-dose dexamethasone suppression test: After you’re given a high dose (or doses) of dexamethasone, the level of cortisol is measured from blood or urine collected over three days.
  • Blood chemistry study: The levels of sodium, potassium or other substances in your blood are measured; an imbalance may signify ACC.
  • Computed tomography (CT) scan: Detailed images of your internal tissues and organs are taken with a computer that is connected to an X-ray machine. Sometimes, a dye may be given to you intravenously (via an IV) or orally, so that the area is more visible during the scan.
  • Magnetic resonance imaging (MRI): Images are taken of your internal abdominal region using magnets.

If the results from the testing above yielded inconclusive results for ACC, additional testing may include:

  • Adrenal angiography: A contrast dye is injected into your adrenal arteries, and X-rays are taken to identify any blockages in blood flow.
  • Adrenal venography: A contrast dye is injected into your adrenal veins, and X-rays are taken to identify any blood flow blockages. A blood sample may be retrieved to check for hormone level abnormalities.
  • Positron emission tomography (PET) scan: Radioactive glucose is injected intravenously, and an image is taken of the area. Cancerous cells appear more prominently on the image because they take up more of the glucose solution than normal cells.
  • MIBG scan: MIBG, a radioactive substance, is injected sparingly into your veins. As it travels through your bloodstream, your adrenals absorb the material. A radiation-measuring device is used to distinguish ACC from another type of cancer.
  • Biopsy: A sample of suspicious tissue or cells may be removed to be examined microscopically.

ACC treatment

Treatment for ACC depends on the extent and stage of the tumor. It has a greater chance of success if the cancer hasn’t spread. To determine whether your cancer has metastasized, you'll typically undergo imagine procedures such as a CT scan or an MRI.

Depending on whether the ACC has spread, treatments may vary:

  • If the tumor has not spread to distant organs and it can be removed, you may be offered surgery. Your adrenal gland may be removed through an incision in the abdomen (during a procedure called an adrenalectomy), as well as any tissue or lymph nodes that are cancerous.
  • If the tumor cannot be removed due to its size or location, you may be offered radiation therapy, which is used to kill cancer cells or stop them from growing. You may be given external radiation therapy, in which a radiation machine from outside your body targets the cancer, or internal radiation therapy, in which a radioactive substance is delivered directly into the tumor or close to the cancer.

If the tumor cannot be removed due to its location, or if it has spread to distant organs, you may have several options:

  • Chemotherapy: Chemotherapy drugs help to prevent cancerous cells from growing by killing them or preventing their division.
  • Immunotherapy: This treatment uses medications that boost your immune system’s response against cancer.
  • Targeted therapy: This type of treatment targets cancerous cells while preserving your body’s healthy cells.
  • Clinical trials: Tests of new cancer treatments are always underway; ask your care team if you qualify for one.

ACC survival rate

The survival rate depends on the stage of the tumor at the time of discovery. The five-year survival rate for ACC tumors that can be surgically removed is about 38 percent to 46 percent, according to the National Cancer Institute. For stage 4 tumors, the five-year survival rate is less than 20 percent.

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