Adrenocortical carcinoma (ACC) is a cancer of the adrenal glands. The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney.
Tumor - adrenal; ACC - adrenal
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:
Symptoms of increased aldosterone are the same as symptoms of low potassium, and include:
Exams and Tests
The health care provider will perform a physical exam and ask about your symptoms.
Blood tests will be done to check hormone levels:
Imaging tests of the abdomen may include:
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.
When to Contact a Medical Professional
Call your provider if you or your child has symptoms of ACC, Cushing syndrome, or failure to grow.
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. PDQ: adrenocortical carcinoma treatment - health professional version. Updated June 02, 2015. Cancer.gov. www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-pdq#section/all. Accessed October 17, 2016.
Review Date: 8/15/2016
Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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