Neoplasms of the Adrenal Cortex

Abstract

Overview Adrenocortical cancer (ACC) is a rare malignancy, with varied presentations and a poor prognosis. Imaging studies have emerged as central to a work up, and in most cases a biopsy is not needed and should not be performed. Pathologic examination is very valuable with the Weiss criteria used to discriminate a small adenomas from carcinomas. Management is multidisciplinary and includes surgical resection or ablation, oral mitotane, intravenous chemotherapy, and palliative radiation. Surgery remains the only proven curative option, must be considered at presentation, and relapse with laparoscopic resection avoided. Mitotane is approved for the treatment of inoperable functional and nonfunctional ACC and its clinical value is primarily as an anti‐hormonal agent. In a patient whose tumor is producing an excess of hormones mitotane is indispensable, however, its use in the adjuvant setting is uncertain. Replacement steroids are needed. Systemic chemotherapy using mitotane in combination with etoposide, doxorubicin, and cisplatin (EDP‐M) or with streptozotocin (S‐M) are the preferred options. These should not be discarded in favor of a “novel targeted therapy”, none of which have shown any proven efficacy. Increasing evidence suggests that palliative radiation therapy is beneficial for patients with metastatic disease. Radiofrequency ablation (RFA) or cryoablation have emerged as surgical adjuncts or stand‐alone modalities for recurrences. As with most rare diseases, enrollment in clinical trials is strongly encouraged.