Hereditary adrenocortical carcinoma (ACC) is a rare form of cancer that forms in one of the outer layers (cortex) of the adrenal glands, which are located on top of the kidneys. The adrenal glands produce hormones that have important functions like controlling blood pressure and sex hormones in the body. Tumors from ACC cause the adrenal glands to produce too many of these hormones. Symptoms of ACC include stomach pain, high blood pressure, frequent need to urinate, deepening of the voice, and weight gain.
Hereditary ACC can be caused by a number of gene mutations. We inherit our genes in pairs, one from each parent typically. Some of these mutations are inherited dominantly, which means only one copy of the mutation is necessary for an individual to have the disease. Other mutations are inherited recessively, which means an individual must inherit two copies of the mutation (one from each parent) to get the disease. Two of the major genes linked to this condition are the TP53 and the IGF2 genes.
ACC can be diagnosed with a urine test (to check levels of cortisol, a hormone released by the adrenal glands), a blood test (to check potassium and sodium levels), and an MRI (to check for visible tumors) amongst others. Treatment options can include surgical removal of the tumor and a specific type of chemotherapy to help remove any remaining cancer after surgery.
If you or a family member has been diagnosed with hereditary adrenocortical carcinoma, talk to your doctor about the most current treatment options. Support groups are also a good source of information.
Description Last Updated: Aug 29, 2018