Autonomic Instability Secondary to Glomus Vagale Paraganglioma

Abstract

Introduction Paragangliomas are rare vascular neuroendocrine tumors that arise from neural crest chromaffin cells that are associated with autonomic ganglion. A small subset of these tumors are found in the head and neck region and originate in the carotid and aortic bodies. Case description A 60-year-old woman presents with recurrent near syncopal episodes for 3 days associated with loss of appetite and 20-pound weight loss for 2 months. Two years ago, the patient presented with similar presyncopal episodes and Holter monitoring at that time showed multiple pauses up to 20 seconds, after which she was referred for dual chamber pacemaker implantation. During that time, a tilt table test and finometer measuring beat to beat blood pressure values was positive for sudden episodic hypotension and bradycardia every 2-3 minutes and were associated with lightheadedness and near syncope. Vitals during hospitalization showed BP ranging from 80s/50s to 130s/80s. Physical exam showed normal cardiopulmonary findings but revealed a right cervical mass along the angle of mandible, mildly tender to palpation. EKG showed an appropriately paced atrial rhythm at 59 beats per minute without any other abnormalities. As a result of her hypotensive episodes, the patient was started on fludrocortisone and pyridostigmine. CT neck with contrast showed a post styloid compartment mass with significant compression of R internal jugular vein and branches of R carotid artery. Subsequent angiography showed 4 × 3 × 3 cm. hypervascular mass in the upper right neck with displacement of carotid arteries and AV shunting into the R internal jugular vein. Findings were consistent with Glomus Vagale Paraganglioma and patient was discharged in stable condition for outpatient evaluation for surgical resection of mass. Discussion Indistinguishable from pheochromocytomas at the cellular level, paragangliomas are divided into catecholamine secreting sympathetic and nonfunctional parasympathetic neoplasms. Although most cases are sporadic, there is a link between paragangliomas and mutation of genes coding for Succinate Dehydrogenase (SDH) enzyme, Von Hippel Lindau, MEN2, and NF1 syndromes. Paragangliomas are usually asymptomatic and present as painless cervical masses. Glomus vagale, a particularly rare type of paraganglioma located near the vagus nerve, can present with tinnitus, dysphagia, or hoarseness. Carotid sinus compression by the cervical mass can lead to symptomatic hypotension and bradycardia, as seen in our case. The diagnosis of non-secretory paragangliomas includes 24-hour urine test to measure catecholamine and metanephrine levels and visualization of tumor via ultrasound, CT, or MRI which can show a “salt and pepper” appearance due to micro hemorrhage and hypervascularization. Treatment includes surgery, embolization, or radiotherapy. Conclusion Paragangliomas are uncommon tumors with glomus paragangliomas being a rare subtype. Because of their cellular composition and anatomic locations, they often cause autonomic instability. Further research should be performed to improve diagnosis and treatment.