PSAT002 Unilateral Adrenalectomy as a Treatment Option in Patient with Cyclical Cushing's Syndrome

Abstract

Abstract Background Patients with Cyclical Cushing`s Syndrome (CSS) are at risk for a lengthy timeline of living with the untreated disease. Continual increased amounts of cortisol can lead to increased morbidity and mortality in these patients and finding effective treatments is of the utmost importance. Here we present a treatment course of the patient with CCS who eventually underwent unilateral adrenalectomy, resulting in marked improvements in excessive cortisol- mediated metabolic derangements. Clinical Case In 2014, a 51-yo female with hypertension (HTN), hyperlipidemia (HLD), and type 2 diabetes (T2D) presented to VA endocrinology clinic for the evaluation of incidentally found bilateral adrenal nodules. Based on the extensive biochemical evaluation, the patient was diagnosed with ACTH-dependent Cushing's disease (CD). In the setting of newly diagnosed CD and complex metabolic co-morbidities, in 09/2016 she underwent pituitary resection. However, she remained hypercortisolemic and in 12/2016 she had a second hypophysectomy. Despite surgical efforts, hypercortisolemia persisted. Neuroendocrine imaging studies and chest computed tomography excluded non-pituitary etiology of CD. She was subsequently started on ketoconazole but it was stopped due to side effects. Other CD-directed agents were not approved, so we continued routine management of T2D, HTN, and HLD. In 2018, the patient developed acute coronary syndrome and received percutaneous intervention despite therapy with statin and ezetimibe. Further, since her second hypophysectomy she required sequential addition of hypoglycemic agents and by the end of 2020, exenatide weekly, empagliflozin, and pioglitazone were added to her escalating basal-bolus insulin therapy due to persistent A1C elevation >10%. Continuous evaluation of her hypercortisolemia post-hypophysectomy suggested presence of CCS. To alleviate the metabolic burden of hypercortisolemia, the patient was offered unilateral adrenalectomy which she underwent in 03/2021. Within 6 months after the surgery, she has had unintentional 22-lb weight loss and we stopped both of her anti-HTN medications, discontinued weekly exenatide, and gradually de-escalated her initial daily insulin regimen of approximately 1.0 u/kg to an only basal insulin at 0.15 u/kg dose achieving A1C of 6.5%. There was no biochemical evidence of adrenal insufficiency since adrenalectomy was performed. Conclusion The patient with initial diagnosis of CD, which we later proved being CCS, underwent a unilateral adrenalectomy with the goal of reduction of cortisol burden. This decision was taken with the consideration that this would likely not be curative but intended to improve the course of her metabolic conditions. The 6-month observation following adrenalectomy has proven that we were able to in large extend achieve our goals. Therefore, this treatment approach should be considered for patients with CSS or CD. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.