Abstract 4143616: A Silent Murmur: Incidental Finding of a Cardiac Paraganglioma

Background: Paragangliomas are rare neuroendocrine tumors, arising from neural crest cell lines in the autonomic system. The detection of these tumors has risen in recent years with the growing availability and use of intrathoracic imaging. Case Presentation: A 59-year-old male with a past medical history of HTN, HIV, syphilis, and treated prostate cancer presented to the clinic for a new-patient evaluation and was incidentally found to have a 2/6 diastolic murmur, reportedly present since childhood. He was asymptomatic apart from brief episodes of dizziness when leaning forward or standing during exercise. Family history was notable for an aortic aneurysm in his father and valvular disease in his mother. Clinical Decision Making: A TTE showed a 5.7 x 5.9 cm echogenic mass pushing against the RV and RA. Prospective ECG-gated CT scan of the chest revealed a highly vascular intrapericardial mass (6.0 x 7.7 mm) anterior to the RA and RV with the RCA leading into it. Cardiac MRI showed a large, circumscribed mass moving within the RV-free wall without evidence of depressed RV ejection fraction or significant coronary artery compression. Coronary angiogram found an engorged acute marginal branch feeding the highly vascular mass and the RCA continuing under the mass to give off a small PDA and a small RPL. There were normal filling pressures and no significant obstructive CAD. A multidisciplinary team determined a high concern for cardiac paraganglioma. Serological workup was notable for elevated serum epinephrine (335 pmol/L). 24-hour urinary dopamine was elevated (831.5 ug/TV) while 24-hour urinary catecholamines/metanephrines were normal. A DOTATATE PET/CT scan confirmed presence of a cardiac paraganglioma. Genetic workup did not reveal presence of any succinate dehydrogenase (SDH) mutations. Conclusion: Here we present a highly unusual case of an asymptomatic intra-pericardial paraganglioma in a 59-year-old male. Given the lack of guidelines for management and the complexity of the case due to the location of the tumor, a multidisciplinary team comprising cardiology, endocrinology, and surgery is evaluating the patient, with considerations of surgical removal and CABG to PDA after neurohormonal blockade.