Recent advances in adrenocortical carcinoma in adults

Adrenocortical cancer (ACC) is a rare and often aggressive malignancy. The overall 5-year survival rate of ACC is less than 30% in part owing to advanced stage of the disease at diagnosis and limited efficiency of therapies when initial surgery is not curative. So far, studies with large cohorts of patients affected by ACC were lacking because of the rarity of the disease; however, recent international and multicenter collaborative studies provide new insights in the management of ACC.

This review summarizes recent findings in the genetic, hormonal evaluation, imaging, and therapies of ACC in adults. There is new promise for the use of 2-[fluorine-18]fluoro-2-deoxy-D-glucose-positron emission tomography and metomidate in initial diagnosis and follow-up. Limited studies support benefit of specific surgical approaches such as loco-regional lymph node dissection and metastasectomy in specific subgroups. New developments in the use of mitotane therapy and its drug interactions, on adjuvant radiotherapy and prospective data on combined chemotherapy, have appeared recently.

These recent findings will provide more evidence-based recommendations in the future to better assist clinicians in the management of patients with ACC. However, there is still an important need to understand the molecular mechanisms underlying this disease to design better therapeutic approaches.