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Adrenocortical carcinoma is a rare form of cancer that arises in the outer layer (cortex) of the adrenal glands, which are located on top of each kidney. These tumors typically produce excessive amounts of hormones, including cortisol, aldosterone, and/or androgens.

The exact cause of adrenocortical carcinoma is unknown, but certain genetic conditions, such as Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome, may increase the risk. Other risk factors include a family history of the disease, older age, and being female.