Adrenal cortical carcinoma is a rare endocrine malignancy with a poor long-term prognosis. Accurate diagnosis and preoperative evaluation of the patient presenting with an adrenal mass maximize the opportunity for optimal treatment planning. Surgery still offers the best chance for cure. Despite curability with complete surgical resection, the rate of recurrence is unacceptably high. In metastatic or recurrent disease, systemic treatment options are limited to chemotherapy with or without mitotane. Therapeutic options are often outdated and associated with significant toxicities. A multidisciplinary approach has the best chance for offering optimized management of this lethal disease. Improved understanding of the molecular pathogenesis of this rare malignancy will lead to advancement in the available therapies that may improve this outcome.
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