Abstract 14921: Right Atrial Paraganglioma

Abstract

Introduction: Paragangliomas are rare tumors of neural crest cells origin and can be adrenal or extra-adrenal. They can be associated with catecholamine release in some patients and cause systemic symptoms. Cardiac paragangliomas account for less than 1% of all primary cardiac tumors. Case Summary: A 70-year-old woman presented to the emergency department with new-onset chest pain. Vitals were significant for elevated blood pressure but otherwise unremarkable. Physical exam and serum hematology and chemistries were unremarkable. D-Dimer was mildly elevated. Chest CT Angiogram showed a 1.5 cm right lung nodule but no pulmonary embolism. Outpatient Positron Emission Tomography (PET) scan done for further evaluation of the nodule showed no uptake in the lung nodule but a 5cm mass in the right atrium. Cardiac MRI confirmed the atrial mass with suspicion for angiosarcoma. The patient underwent surgery, and the pathology showed paraganglioma. Patient did well after the surgery. Discussion: Cardiac paragangliomas usually present with fatigue, atypical chest pain or heart failure. It can also present with signs of catecholamine excess. Surgery is the preferred treatment for benign paragangliomas. Surgery should be prompt as it has a potential to be malignant. Chemotherapy and radiolabeled agents are additionally used for the malignant types. Conclusions: There should be high clinical suspicion for secondary causes of hypertension in patients presenting with either episodic hypertension or resistant hypertension. Paragangliomas are very rare cardiac tumors and there have been very few reported cases of right atrial paraganglioma. Any suspicion of this rare tumor should prompt aggressive investigation and prompt referral to a center specializing in their removal.