Cortisol and Aldosterone Adrenocortical Mass in a Previously Healthy Pediatric Patient

Abstract

Introduction: Adrenocortical tumors (ACTs) are rare in the pediatric population compared to the adults, with an incidence of 0.3-0.5 cases per million child-years. Most ACTs are sporadic, but some occur as a component of hereditary cancer syndrome. We report a case of a 16 year old male with single unilateral adrenocortical tumor that was found to secrete cortisol and aldosterone. Case Presentation: 16-year-old male previously healthy, presented to nephrology for elevated blood pressure (BP) and microscopic hematuria evaluation. He was otherwise asymptomatic. Manual BP was noted to be 156/84 mmHg and physical exam was within normal limits. Laboratory evaluation revealed a normal comprehensive metabolic panel. Echocardiography was notable for moderate left ventricular hypertrophy, and a renal ultrasound revealed a heterogeneous solid right adrenal mass which measured 5.6 x 4.5 cm. Adrenal MRI showed a well-defined round 5.7 x 5.0 x 5.2 cm right adrenal mass with heterogeneously T1 isointense and T2 hyperintensity, with no evidence of vascular invasion. The left adrenal gland was normal. Additional workup was notable for an aldosterone level of 18.4 ng/dL (4.0-31.0 ng/dL), plasma renin activity 0.2 ng/ml/h (<6), and elevated aldosterone to renin ratio of 92. The morning cortisol level of 7.7 ug/dL (6.7-22.6), with a 24-urine cortisol level elevated at 70.2 ug/d (<56.0). Low dose and high dose Dexamethasone suppression tests were performed, and the morning cortisol was not suppressed, pointing more towards hypercortisolism from the adrenal gland. ACTH levels were found to be suppressed as well. Due to concerns of possible adrenal crisis during or after surgery, the patient was started on stress dose steroids and then tapered off after several weeks. Patient underwent laparoscopic removal of the mass and right adrenal gland with uncomplicated postsurgey period. Pathology showed an adrenal cortical tumor 6 cm/68gm, indeterminate for malignancy versus benign. Extensive genetic studies revealed that patient was heterozygous for the p.N560(c/1679A>T) variant of unknown significance in the MSH2 gene. His blood pressure continued to remain elevated after surgery, and he was started on lisinopril with minimal improvement. The patient is currently doing well, and his blood pressure is better controlled now with amlodipine. He is undergoing surveillance with laboratory evaluation and imagining every 3-6 months. Discussion: ACTs are extremely rare in pediatric patients. Their presentation might be variable, and even when they are found incidentally, it is crucial to assess for hormonal secretion. Although our patient had no signs of hypercortisolism on physical exam, his ACT was secreting both cortisol and aldosterone. In this case report, we demonstrated a rare case of unilateral ACT causing hyperaldosteronism and hypercortisolism with persistent hypertension following adrenalectomy.