SAT-323 Thoracic Paraganglioma Presenting with Recurrent Cerebrovascular Events

Background: Thoracic spine paraganglioma precipitating acute stroke is extremely rare. We report a case of a patient with thoracic paraganglioma at high risk for spinal cord compression with recurrent cerebrovascular events. Clinical Case: A 59 year old male was admitted for transient right hemiplegia after recent admission for right lacunar CVA. The patient was noted to have episodes of labile hypertension, sinus tachycardia and paroxysmal diaphoresis. Five days after admission, he developed ataxia with right pronator drift and was treated with intravenous thrombolytics for suspected acute CVA. Incidentally, an 8 cm thoracic paraspinal necrotic mass invading T10 and T11 vertebral bodies with impending cord compression was found on imaging. CT-guided needle biopsy of the mass was performed. Pathology showed an oval and spindle cell neoplasm staining positive for synaptophysin and chromogranin, favoring paraganglioma. Chromogranin A was elevated 472 ng/mL (25 - 140 ng/mL). Plasma free metanephrines were 32 g/mL (n < 57 pg/mL) and plasma free normetanephrines were 7690 pg/mL (n < 148 pg/mL). MIBG scan showed focus of increased uptake at T10 and T11 as well as in the left posterior eighth rib and peri-aortic region. Subsequently, the patient developed acute right leg weakness and dysmetria; MRI brain confirmed acute infarct in the posterior limb of the left internal capsule. Alpha blockade followed by beta blockade was initiated. After approximately 2.5 weeks of alpha blockade, the patient underwent spinal angiogram with tumor embolization followed by successful en bloc tumor resection by neurosurgery and cardiothoracic surgery. Final pathology showed a 7.5 cm paraganglioma which demonstrated some aggressive pathologic features including Ki67 index>20% and 10% focal necrosis. Post-operative plasma free normetanephrines decreased to 568 pg/mL and metanephrines were undetectable. Genetic testing utilizing Ambry Genetics PGLnext panel was negative. Conclusion: A differential diagnosis of paraganglioma should be considered in any patient presenting with recurrent stroke and paraspinal mass. Biopsy should be avoided. Timing of surgical resection to decrease risk of further cerebrovascular compromise is challenging and requires a multidisciplinary approach (1). Locally invasive paragangliomas are rare and are often associated with SDHB mutations; however, a full genetic analysis did not identify any known genetic mutations. Reference: (1) Oak, S., Javid, M., Callender, G., Carling, T., Gibson, C. Management of pheochromocytoma in the setting of acute stroke. AACE Clinical Case Reports 2018 4:3, e245-e248.