Ectopic Cortisol and Androgen-Producing Adrenocortical Carcinoma Arising from Adrenal Rest Tissue in a Kidney: Case Report and Literature Review

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 native adrenal gland was atrophic and free of the tumor. Postoperatively, cortisol and adrenal androgen levels became undetectable. However, her disease unfortunately recurred 5 months later with innumerable peritoneal, mesenteric, omental, and serosal implants within the abdomen and pelvis. She was started on mitotane and mifepristone but unfortunately expired within a few days from a septic shock secondary to Pseudomonas aeruginosa pneumonia. Conclusion: We present an unusual case of a functional adrenocortical carcinoma arising in adrenal rest tissue in a kidney resulting in Cushing syndrome and virilization with very aggressive biology. To the best of our knowledge, no similar case has been reported thus far. Abbreviations: ACC = adrenocortical carcinoma; CT = computed tomography