Abstract

ABSTRACT Objective: To present a case of an aggressive adrenocortical carcinoma producing both ectopic cortisol and androgens and to provide an extensive literature review of similar cases. Methods: A case presentation is provided that illustrates clinical, biochemical, radiologic, and pathologic findings. We also conducted a PubMed search for similar cases and summarize 15 other cases of malignant adrenal rest tissue. Results: A 50-year-old woman presented with symptoms of Cushing syndrome and virilization. Urine free cortisol and serum adrenal androgens were significantly elevated. Computed tomography of the abdomen revealed a 12-cm mass within the right kidney. Subsequently, the patient underwent right radical nephro-adrenalectomy. The tumor showed a high-grade neoplasm centered in the kidney with polymorphism, highly atypical nuclei, abundant mitotic activity, and necrosis. The Ki-67 proliferation index was 25 to 30%. Immunohistochemical staining of the neoplastic cells favored an adrenal origin. The ...

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