Safia Ouarrak, Christ Debs, Can M Nguyen, Jean Perron, Sylvain Trahan, Olivier F Bertrand
BACKGROUND Cardiac paragangliomas are extremely rare neuroendocrine tumors arising from chromaffin cells of neural crest origin. These tumors are typically hypervascular and can derive part of their arterial supply from the coronary circulation. Because of their rarity and heterogeneous clinical presentation, diagnosis can be challenging. Symptoms can result from catecholamine secretion, local mass effect on adjacent cardiac structures, or incidental discovery during imaging studies. In rare cases, cardiac paragangliomas may mimic an acute coronary syndrome despite the absence of obstructive coronary artery disease. CASE REPORT A 67-year-old woman presented with non-ST-segment elevation myocardial infarction in the context of rapid atrial fibrillation. Coronary angiography demonstrated normal epicardial coronary arteries but revealed an intense vascularization of a right retroatrial mass supplied by branches of both the right and left coronary arteries. Subsequent multimodality imaging identified a well-defined hypervascular mediastinal mass highly suggestive of a cardiac paraganglioma. Elevated urinary normetanephrine levels confirmed the secretory nature of the tumor. Given the extensive vascular supply visualized on angiography, preoperative transradial transcatheter embolization of the coronary feeding vessels was performed to reduce the risk of intraoperative bleeding and possibly reduce the tumor size. The patient subsequently underwent complete surgical resection with histopathologic confirmation of cardiac paraganglioma. CONCLUSIONS This case highlights the importance of recognizing a coronary tumor blush, which should prompt comprehensive diagnostic evaluation. In this patient, preoperative embolization combined with complete surgical resection was feasible and associated with a favorable long-term outcome, and may be considered in selected cases.