Adrenocortical carcinoma (ACC) is rare but one of the most malignant endocrine tumors. This article reviews and summarizes the current knowledge about the treatment of ACC. The epidemiology and molecular events involved in the pathogenesis of ACC are briefly outlined. The different diagnostic tools to distinguish benign from malignant adrenocortical tumors, including biochemical analysis and imaging, are discussed. The surgical treatment of ACC has evolved in the last 2 decades. The different surgical alternatives for the treatment of ACC in the context of primary, recurrent, or metastatic disease are reviewed, and the remaining challenges and controversies are discussed.
Keywords: Adrenocortical carcinoma; Adrenocortical tumor; Chemotherapy for adrenocortical carcinoma; Metastatic adrenocortical carcinoma; Mitotane treatment; Open adrenalectomy.
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