An incidental intrapericardial paraganglioma followed up for 11 years

Paragangliomas located in the pericardium are extremely rare and data on its pathophysiology, natural history and management guidelines are lacking. We present a 24-year-old female with an asymptomatic right-sided pericardial hypervascular mass incidentally found on cross-sectional imaging. The biochemical testing showed no evidence of catecholamine hypersecretion and the 123-I metaiodobenzylguanidine scintigraphy revealed an isolated focus of abnormal uptake in the right atrium. She was started on alpha-adrenergic blockade and underwent arterial embolization and surgical excision of the mass. Histopathological examination revealed a well differentiated paraganglioma. Genetic testing was negative. There was no evidence of tumor recurrence, but she abandoned medical follow-up after ten months. Seven years later she returned for preconception counseling and the workup confirmed sustained remission. The pregnancy was uneventful, and she remains stable in active surveillance after eleven years since diagnosis. We describe a pericardial paraganglioma with very unusual features: absence of catecholamine hypersecretion or compressive symptoms, located in the right atrium and apparently sporadic. After a curative intended surgery preceded by arterial embolization and preparation with alpha-blockade, there was complete remission with no evidence of local or extracardiac disease within a very long-term follow-up. We provide more evidence on the characterization, management and long-term outcomes of localized non-secretory pericardial paragangliomas. After surgical treatment, we propose lifelong clinical, biochemical, and imaging surveillance, even for asymptomatic non-secretory tumors.