Adrenocortical Carcinoma (ACC): A Patient Guide

Adrenocortical Carcinoma (ACC) is an extremely rare and complex cancer originating in the outer layer of the adrenal glands. Because it affects only about one or two people per million annually, it is a condition that requires highly specialized, multidisciplinary care from experts who understand its unique behavior ^[1]. These tumors are often “functioning,” meaning they produce an excess of hormones like cortisol or androgens, leading to physical symptoms that can sometimes be the first sign of the disease ^[2]. Successfully navigating ACC begins with a clear understanding of your specific tumor’s biology, as treatment decisions are heavily influenced by detailed pathology ^[3].

The most critical information your team will gather comes from the tumor’s Ki-67 index, a measurement of how fast the cells are dividing, and its ENSAT stage, which describes how far the cancer has spread ^[4][5]. These results, alongside surgical margins and other health factors, inform your risk of the cancer returning and help determine whether you will need additional treatments. Complete surgical removal, performed as an “open” procedure by a specialized surgeon to ensure the tumor remains intact, is the primary treatment with the potential for a cure in localized disease ^[6][7].

For selected patients at higher risk of recurrence, surgery may be followed by the use of mitotane, a powerful medication designed to destroy any remaining microscopic adrenal cells ^[8]. While mitotane is a cornerstone of therapy, it is a challenging drug that requires careful management. It effectively shuts down your body’s ability to produce its own cortisol, making glucocorticoid replacement therapy a life-saving necessity ^[9]. Living with mitotane means being prepared for emergencies, including carrying an injection kit and understanding your individualized plan to adjust your steroid dose during times of illness or stress ^[10].

As you move beyond active treatment, your care shifts toward a rigorous surveillance strategy. This primarily involves scheduled imaging, which may be complemented by sophisticated blood or urine hormone testing in certain cases ^[11]. While the road ahead requires patience and constant vigilance, being treated at a high-volume center with a dedicated team ensures you have the most advanced tools available to manage your health and focus on recovery ^[6].

Note: ACC management differs for localized, recurrent, metastatic, functioning, and nonfunctioning tumors. This guide provides an overview, but does not replace an individualized plan developed by a multidisciplinary tumor board.