Adrenocortical carcinoma (ACC) is a rare and aggressive malignant tumor, and management requires a multidisciplinary approach. Although several molecular and immunohistochemical markers have recently evolved as predictors of aggressiveness, none of them has been established for a particular therapeutic strategy. Ki-67 expression has been found to be the single most prognostic factor for recurrence of ACCs. Surgery remains the ‘gold standard’ in resectable tumors. The high recurrence rate and poor outcome of ACCs have prompted use of adjuvant therapy following surgical resection. In unresectable tumors or in metastatic disease, other therapeutic modalities are considered. Mitotane is the standard medical treatment in cases of advanced or aggressive tumors. It requires close monitoring because it is associated with adverse side-effects that may compromise its use. EDP chemotherapy along with mitotane is the recommended therapeutic regimen in metastatic adrenocortical carcinomas not amenable to surgery, but has no significant benefit in the overall survival. New targeted therapies based on tumor biology are being developed in order to improve therapeutic efficacy. Prospective clinical trials should be initiated in low and high risk patients to evaluate current and evolving therapies.
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