Adrenocortical carcinoma (ACC) is a rare cancer for which few treatment options have been available. Currently, the best available treatment involves combination chemotherapy with the adrenolytic drug mitotane, although the response rate remains modest. Over the past 10 years there has been renewed interest in the field owing to the recognition that targeted therapies may provide new avenues for effective treatment of this deadly disease. Molecular analyses have revealed specific signaling alterations in ACC, and advances in drug development have generated the tools to block these pathways. Although convincing evidence for the effectiveness of targeted therapies is not currently available, these studies are in progress and should shift the prognosis of this disease in the years to come.
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