SAT529 An Uncommon Case Report Of A Paraaortic Paraganglioma

Abstract

Abstract Disclosure: E. Askar: None. J.R. Fredrick: None. S. Anantha: None. K.G. Reddy: None. Background: Extraadrenal pheochromocytomas, also known as paragangliomas are uncommon neuroendocrine tumors that develop from sympathetic ganglia chromaffin cells. These tumors rarely cause hypertension, in less than 0.2% of patients. Although pheochromocytomas are known to be secreting catecholamines, paragangliomas are less likely to be functional. Here we report a rare case of Catecholamine secreting Paraganglioma presenting with hypertension. Clinical Case: A 28 -year-old female with past medical history of Goldenhar syndrome presented to our outpatient institution complaining of headache, palpitations and high blood pressure measurements at home with systolic blood pressure ranging between 160-200 mm Hg for a few months prior to presentation. Her only medication was propranolol for hypertension. At presentation, blood pressure was 126/72 mm Hg, heart rate was 76 beats per minute. Physical examination revealed no significant findings. Abdominal exam did not reveal any palpable masses. The patient denied any history of substance, tobacco, or alcohol abuse. No family history was found for any similar masses or hypertension. Laboratory findings were significant for elevated normetanephrines at 785 pg/mL (18 to 111) with normal metanephrines level. CT abdomen was significant for 2.5 cm enhancing left paraaortic mass separate from kidneys and adrenal glands. The patient was started on doxazosin and propranolol was switched to atenolol. After her blood pressure was adequately controlled with the alpha and beta blockade, she was referred to a tertiary facility for excision of the mass. Robotic excision of the mass was performed successfully without any significant blood pressure variations noted intraoperatively. The diagnosis of a well differentiated extraadrenal pheochromocytoma was confirmed by pathology, and the patient was referred for outpatient genetic testing and endocrinology follow up. Conclusion: This is an extremely rare case of paraganglioma given its presentation and location of the mass. The previously reported paragangliomas usually present as ER visits with severe sympathetic symptoms but it is uncommon to present as an outpatient for secondary hypertension workup. In the literature, we have found case reports of catecholamine secreting paragangliomas which guided the management of this uncommon case as mentioned above. Clinicians must be aware of this rare presentation of paraganglioma in order to properly diagnose and manage the condition.