It is a cancer that is mainly originating in the cortex region of adrenal gland, which produces steroids. There are two regions in the adrenal gland namely adrenal cortex and adrenal medulla. Adrenal cortex has three layers: Glomerulosa, reticulata, fasciculata. Glomerulosa secretes Mineralocrticoids; Reticulata secretes glucocortcoids while fasiculata sceretes the Andrgens. Now, the tumour of adrenal cortex of a gland can produce excessive secretion of these hormones, So, there is manifestation like Cushig syndrome, Conn's disease, virilization in females and feminization in males. It is a rare type of cancer. Variety of adrenocortical carcinoma is clear cell adrenocortical carcinoma, myxoid adrenocortical carcinoma, adenosquamous carcinoma, oncocytic adrenocortical carcinoma.

The genes like h19, insulin growth factor, p57 are important for the development of the foetus. Expression of the h19 gene is markedly reduced in both nonfunctioning and functioning adrenalcortical carcinomas. This cancer is commonly seen in people who have family history of Li-Fraumeni syndrome, which is characterized by the inactivation of tumour suppressor genes p53 and RB (retinoblastoma) located on 17th and 13th chromosomes may get changed. There is also a loss of activity of the p57kip2 gene product in virilizing adenomas and adrenal cortical carcinomas. Thus the cause of this cancer is basically a defect in genes.