Head and neck paragangliomas are typically slow growing neuroendocrine tumours of neural crest cells. Unlike phaeochromocytoma or thoracic and abdominal paragangliomas, they then to be non-secretory. We present a case of a patient presenting with an incidental finding of a rare, functional neck paraganglioma requiring peri-operative optimization for an upcoming cranial procedure complicated by medication intolerance.
A 69 year old female was discovered to have an incidental 30mm lesion near the left jugular suspicious of paraganglioma on a FDG PET/CT in 2022 completed for the investigation of right pulmonary nodules. A Gallium-68 DOTATATE PET/CT confirmed an intensely DOTATATE-avid likely left glomus vagale paraganglioma with a SUV-max score of 98.1 and Krenning grade 4. Initial plasma metanephrines revealed elevated levels of 3-methoxytyramine 211pmol/L and normetadrenaline 4980pmol/L, with normal metadrenaline levels of 251pmol/L. Over the course of 6 months, she developed intermittent headaches and hypertension with persistently elevated catecholamine levels. She was commenced initially on prazosin 0.5mg daily but switched to 10mg phenoxybenzamine due to poor side effect tolerance consisting of dizziness and feeling of paraesthesia. A few months later, she reported similar issues with phenoxybenzamine and was switched to amlodipine 10mg, which was better tolerated.
In early 2024, she reported recurrent left facial drooping and paraesthesia. An MRI brain and angiography demonstrated an 8mm right middle cerebral artery aneurysm and a seperate 2mm aneurysm at the A2 segment of the anterior cerebral artery. She was discussed in a multidisciplinary meeting and currently planned for hospital admission for peri-operative blood pressure management with alpha-blockade under observation prior to endovascular procedure for the aneurysms.
Standard of care for peri-operative management of patients with catecholamine secreting tumours is alpha blockade to reduce the risk of catecholaminergic crisis. However, the literature remains limited for peri-operative optimization in other surgical interventions, which warrants further research.