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.
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:
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.
Call your provider if you or your child has symptoms of ACC, Cushing syndrome, or failure to grow.
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, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. 11-18-19: Editorial update.