FRI250 Dual Aldosterone And Cortisol Secreting Adrenal Cortical Carcinoma: Pre- And Perioperative Evaluation And Management

Abstract

Abstract Disclosure: R. Rosenberg: None. M.R. Clay: None. C. Raeburn: None. M.E. Wierman: None. Representing 0.3% of all adrenal tumors, adrenal cortical carcinoma (ACC) is a rare and understudied disease. It is estimated that 60% of patients with ACC present with hormone excess syndromes, while the remainder present incidentally or with compressive symptoms from tumor invasion. ACC presentation with a combination of hormonal excess syndromes from different adrenal zones is rare and only described previously in four case reports. We present a case of dual-secreting ACC with hyperaldosteronism and cortisol excess. The previously healthy patient was noted to have new onset hypertension and hypokalemia during a primary care visit. On hormonal evaluation, he was found to have evidence of hyperaldosteronism and ACTH-independent cortisol excess (aldosterone 28.2ng/dL, plasma renin activity 0.1ng/mL/hr, and overnight 1mg dexamethasone suppression test cortisol of 13ug/dL). Imaging revealed a 2.7 x 3.1 x 3.5cm left adrenal mass suggestive of an adenoma. He underwent laparoscopic adrenalectomy and required glucocorticoid replacement for adrenal insufficiency post-operatively. Pathologic review of the gross specimen showed a sizable neoplasm measuring 6.5 x 3.0 x 2.5 cm, much larger than on CT imaging. Microscopic review showed stage T2N0M0 ACC with modified Weiss score of 6 and 68 mitoses per 50 hpf, indicative of aggressive disease. The patient’s hypokalemia resolved post-operatively, and glucocorticoids were tapered within a month. The mechanism of dual aldosterone/cortisol secretion is hypothesized to be due to overlap between genes coding for 11β-hydroxylase and aldosterone synthase, resulting in a chimeric gene with increased secretion of both enzymes, and, therefore, both hormones. Though dual secretion is rare, this case report stresses the importance of screening for cortisol excess, and hyperaldosteronism in the correct clinical setting, in patients with adrenal masses detected on imaging. A complete biochemical hormone evaluation should be performed regardless of the discovery of other hormone secreting syndromes, ideally preoperatively with a multidisciplinary team including endocrinology. The medical team should continue to bear in mind that the existence of one hypersecretory syndrome does not rule out the presence of a second or third, and hormonal excess syndromes may affect patient outcomes peri- and postoperatively. Presentation: Friday, June 16, 2023