Black cardiac paraganglioma in a multiple paraganglioma syndrome

We describe a unique case of a patient with a 'pigmented' cardiac paraganglioma in a multiple paraganglioma syndrome. She was symptomatic for arrhythmias, hypertensive crises and dyspnoea due to a cardiac tumour, which was richly vascularised from the right coronary artery and was partially obstructing the right atrioventricular inflow. She was operated on, but the mass was not completely resectable due to its relationship with cardiac structures. The histological findings were paraganglioma with abundant, dark granular pigment. To our knowledge, pigment presence has never been cited in surgical cases of cardiac paragangliomas.