Current options and perspectives of systemic therapy for advanced or metastatic adrenocortical cancer

Adrenocortical cancer is an orphan tumor with poor prognosis. The combination of EDP chemotherapy regimen and mitotane is the standard for the first-line therapy. But there are no effective options for the second and consequent lines of therapy. The standard of second-line therapy is the combination of gemcitabine, capecitabine and mitotane, which provides an objective response in 4-7 % of patients. Achievement of the therapeutic concentration of mitotane is the most important predictive factor of efficiency of GemCap +mitotane regimen, and, therefore, it is recommended to continue mitotane therapy after progression to mitotane. Recently, many researches regarding the efficiency of targeted and immunotherapy of adrenocortical cancer have been published. However, there are no standards for the third and subsequent lines of treatment. This review outlines the current views and perspectives of systemic therapy for advanced and metastatic adrenocortical cancer.