PMON42 A unique case of Norepinephrine secreting Head and Neck Paraganglioma
Authors: Davis Clifton , Drincic Andjela , Kotwal Anupam , Pachigolla Srujana , Surdell Daniel , Zhang Chi 
Published: November 2022

Introduction

Head and neck Paragangliomas (HNPGLs) are rare, slow growing neuroendocrine tumors arising from parasympathetic and sympathetic paraganglia in the head and neck region. Traditionally, HNPGLs were presumed to be rarely secretory compared to thoracoabdominal sympathetic PGLs(&lt;5%) and most of them are thought to be identified due to compressive symptoms or incidentally on imaging performed for screening in genetically predisposed individuals. However, most recent evidence has suggested that &gt;10% of HNPGLs can be secretory although data has also shown that typically catecholamines.

Case Presentation

A 55-year-old man presented to the emergency room with worsening headaches, right sided neck pain and dizziness. Physical examination revealed multiple cranial neuropathies including right facial weakness with ipsilateral lagophthalmos, tongue atrophy, dystonic and atrophic sternocleidomastoid muscle, and vagal deficit. Blood pressure was markedly elevated at 227/108 mmHg. Computed tomography scan of head and neck revealed a large bony destructive lesion involving the right skull base measuring 9.0×7.0 cm, with involvement cervical vertebral bodies, as well as ventriculomegaly suggestive of obstructive hydrocephalus with mass effect on the brain stem and cerebellum. History revealed knowledge of this skull base mass for 13 years. Initially the mass was 3.2 cm in the largest dimension with elevated 24-hour urine normetanephrine 1113 mcg/day, Ref: 50-650 mcg/day and dopamine 667 mcg/day Ref: 60-440 mcg/day. He didn't seek further medical care at that time due to his family's opposition to traditional medicine. Magnetic resonance imaging upon this admission revealed 9.0×6.0×5.5 cm expansile, hypervascular, destructive mass at the right skull base with pronounced mass effect and brainstem compression with significant narrowing at the foramen magnum. 24-hour urine normetanephrines were significantly elevated at 4229 mcg/day, ref: 114-865 mcg/day, as well as elevated plasma Normetanephrine at 11.58 nmol/L, ref: 0.00-0.89 nmol/L, and urine Dopamine 10,071 mcg/day, ref: 71-485 mcg/day. Antihypertensive treatment was initiated with alpha blockade followed by additional beta blockade. After multidisciplinary discussion, the decision was made to pursue external radiation therapy given the considerable risk associated with the surgery due to location and vascularity. Genetic testing is pending at the time of abstract submission.

Conclusion

Recent evidence has suggested that &gt;10% HNPGLs can be secretory as opposed to previous assumption that they are rarely secretory. Norepinephrine is the most common catecholamine secreted by HNPGL and dopamine is very rare. We present a unique case of HNPGL when surgery was deferred for more than a decade due to opposition to traditional medicine resulting in a large norepinephrine and dopamine secreting tumor (10 times the upper limit of normal) and multiple compressive cranial neuropathies.

Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.