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The adrenocortical carcinoma is rare and aggressive. It has a bimodal presentation, predominantly female, > 20% of cases will be diagnosed incidentally. 43-year-old male, with colic pain in the left flank, weight loss and intermittent fever. Computed tomography with a tumor on the left adrenal with liver metastases, block resection surgery was performed, pathological report of adrenocortical carcinoma with a 7 points of Weiss score and Ki67 40%. Adrenocortical carcinoma is a rare and aggressive neoplasm; the clinical presentation is variable. Systemic therapy is important even in patients with localized disease and independent of surgical approach., (Copyright: © 2021 Permanyer.)

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