Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy arising from the cortex (outer layer) of the adrenal glands. The adrenal cortex produces steroid hormones essential for metabolism, blood pressure, and sexual development. ACC tumors may overproduce these hormones, causing Cushing syndrome (excess cortisol), virilization (excess androgens), feminization (excess estrogen), or hyperaldosteronism. About 50-60% of ACCs are hormonally active, which can lead to earlier diagnosis through hormonal symptoms. Non-functioning tumors are often found incidentally on imaging or when they become large enough to cause pain. Complete surgical resection is the only curative treatment and is the cornerstone of management. For unresectable or metastatic disease, mitotane (an adrenolytic drug that destroys adrenal tissue) combined with etoposide, doxorubicin, and cisplatin (EDP-M) is the standard systemic approach. Prognosis depends heavily on stage at diagnosis and completeness of surgical resection.