Adrenocortical carcinoma

ACC is most common in children younger than 5 years old and adults in their 40s and 50s.

The condition may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor.

ACC can produce the hormones cortisol, aldosterone, estrogen, or testosterone, as well as other hormones. In women the tumor often releases these hormones, which can lead to male characteristics.

ACC is very rare. The cause is unknown.

Symptoms of increased cortisol or other adrenal gland hormones may include:

• Fatty, rounded hump high on the back just below the neck (buffalo hump)
• Flushed, rounded face with pudgy cheeks (moon face)
• Obesity
• Stunted growth (short stature)
• Virilization -- the appearance of male characteristics, including increased body hair (especially on the face), pubic hair, acne, deepening of the voice, and enlarged clitoris (females)

Symptoms of increased aldosterone are the same as symptoms of low potassium, and include:

• Muscle cramps
• Weakness
• Pain in the abdomen

The health care provider will perform a physical exam and ask about your symptoms.

Blood tests will be done to check hormone levels:

• ACTH level will be low.
• Aldosterone level will be high.
• Cortisol level will be high.
• Potassium level will be low.
• Male or female hormones may be abnormally high.

Imaging tests of the abdomen may include:

• Ultrasound
• CT scan
• MRI
• PET scan

Primary treatment is surgery to remove the tumor. ACC may not improve with chemotherapy. Medicines may be given to reduce production of cortisol, which causes many of the symptoms.

The outcome depends on how early the diagnosis is made and whether the tumor has spread (metastasized). Tumors that have spread usually lead to death within 1 to 3 years.

The tumor can spread to the liver, bone, lung, or other areas.

Contact your provider if you or your child has symptoms of ACC, Cushing syndrome, or failure to grow.

Tumor - adrenal; ACC - adrenal

Allolio B, Fassnacht M. Adrenocortical carcinoma. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 107.

National Cancer Institute website. Adrenocortical carcinoma treatment (Adult) (PDQ) - health professional version. www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-pdq. Updated August 25, 2022. Accessed October 14, 2022.

Yeh MW, Livhits M, Duh Q-Y. The adrenal glands. In: Townsend Jr CM, Beauchamp RD, Evers DM, Mattox KL, et al, eds. Sabiston Textbook of Surgery. 21st ed. St Louis, MO: Elsevier; 2022:chap 40.

Updated by: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.