SAT-325 Episodic Hypertension in a Young Female Due to Ectopic Adrenal Tissue: An Unusual Presentation

Abstract

Introduction: Secondary hypertension due to elevated catecholamine secretion is typically seen in patients with pheochromocytomas and rarely, in those with paragangliomas. We present an unusual case of a young patient with episodic hypertension and elevated metanephrines possibly due to ectopic adrenal tissue. Case: A 31-year-old female with new-onset hypertension and uterine fibroids presented with left upper quadrant abdominal pain and high blood pressure. She had a partial hysterectomy 6 months ago and since then, had persistent abdominal and back pain. Over the past year, she also had worsening anxiety attacks with sudden sensation of flushing and lightheadedness. The episodes lasted for a few minutes several times a week, and were unresponsive to sedatives. On presentation, significant findings included: elevated blood pressure (178/114 mmHg), mild hypercalcemia, and an inappropriately elevated iPTH consistent with primary hyperparathyroidism. Plasma metanephrines were elevated; 24hr 5-HIAA was normal. CT of the abdomen showed a solid, lobulated retroperitoneal mass of 2.1cm with 170 Hounsfield units. Octreotide scan did not show abnormal areas of uptake. The mass was presumed to be a paraganglioma and she underwent surgical resection. The mass seen on imaging turned out to be a hemangioma however an additional mass removed from the area of the aortic bifurcation was found to be ectopic adrenal tissue. Post-op labs showed decreased metanephrines (from 101 to 23pg/mL) and normetanephrines (from 234 to 116pg/mL). Repeat CT scan 4 months later showed no new masses. Post-op, the patient’s symptoms improved but not completely resolved. Discussion: Ectopic adrenal tissue is an exceedingly rare finding in adults. It is more common in the pediatric population and is typically identified incidentally during pelvic surgery where groin exploration is performed. Sullivan et al. studied the prevalence of ectopic adrenal tissue and noted it was discovered in <3% of ~900 children who underwent groin exploration surgery, none of which were female1. With aging, ectopic adrenal tissue typically atrophies due to functioning adrenal glands making it an even less common entity in adults. Our case is even more unusual in that the ectopic tissue appears to be functioning by secreting catecholamines. Furthermore, given the primary hyperparathyroidism in the setting of a potential catecholamine-producing tumor, we pose whether this warrants genetic testing for multiple endocrine neoplasia (MEN), specifically MEN 2A. Reference: 1-J.G. Sullivan, M. Gohel, R.B. Kinder. Ectopic adrenocortical tissue found at groin exploration in children: incidence in relation to diagnosis, age and sex. BJU Int 2005; 95:407-410