Cardiac Paraganglioma Discovered Incidentally

Abstract

Background

Paragangliomas are rare neuroendocrine tumors, arising from neural crest cell lines in the autonomic system. The increased detection of these tumors in recent years can be attributed to advances in imaging techniques.

Case Summary

A 59-year-old man, presenting for a routine primary care evaluation, was incidentally noted to have a 2/6 diastolic murmur. Echocardiography and cardiac magnetic resonance characterized a large, circumscribed mass within the atrioventricular groove of the right ventricle. Coronary angiography demonstrated that this mass was highly vascularized, supplied by the right coronary artery. Serologic work-up revealed significantly elevated serum epinephrine and urinary dopamine levels. DOTATATE-positron emission tomography/computed tomography confirmed the diagnosis of a cardiac paraganglioma.

Discussion

Cardiac paragangliomas are associated with significant morbidity due to catecholamine secretion and rarely malignant potential. Cardiac paragangliomas can also be asymptomatic, making diagnosis challenging. Given their rarity, there is a paucity of evidence-based guidelines regarding diagnostic and management strategies for these tumors.

Take-Home Messages

Cardiac paragangliomas require a combination of serological and imaging tests for diagnosis. Multidisciplinary evaluation is essential for developing effective management strategies.