Adrenocortical cancer: recent clinical and molecular advances

Adrenocortical cancer (ACC) is an uncommon disorder that remains a challenge to the surgeon and oncologist. When the disease is localized to the adrenal gland and readily amenable to surgical resection, reasonable 5-year survival rates are possible. Locally invasive disease carries a poorer prognosis, and metastatic disease is uniformly fatal within 1 year. In this review, we summarize the current knowledge regarding the clinical management of ACC and the molecular mechanisms underlying the disease.

The clinical manifestations, staging, and current treatment for ACC has been well documented. Surgery is still the mainstay of treatment, but identifying molecular targets for chemotherapeutic agents or monoclonal antibodies would be a great advance. At present, our understanding of pathogenic mechanisms is crude; however, the molecular events regulating this aggressive disease are beginning to emerge, especially in the last few years. The advent of laparoscopic adrenalectomy has also created its own dilemmas regarding the appropriate surgical approach to the large, potentially malignant adrenal mass.

The challenge in the management of this disease lies in understanding the molecular mechanisms that underlie the development of ACC with the diagnostic and therapeutic benefits that would ensue.