Resection of a functioning intrapericardial paraganglioma associated with succinate dehydrogenase B mutation. (March 2019)

• Record Type: Journal Article
• Authors: Siejka, Dylan A; Vittorio, Alexander F; Thakur, Sameer; Burgess, John R; Hardikar, Ashutosh
• Is Part Of: SAGE open medical case reports. Volume 7(2019)
• Publication Date: 2019-03
• Subjects: Paraganglioma -- cardiothoracic surgery -- succinate dehydrogenase -- succinate dehydrogenase subunit B -- intrapericardial; Clinical medicine -- Case studies -- Periodicals; 616.09
• DOI: 10.1177/2050313X19839530
• Languages: English
• ISSNs: 2050-313X

Abstract

Functional paragangliomas are rare neuroendocrine tumours that secrete catecholamines and are infrequently found in the mediastinum. We report a case of a young male with symptoms of catecholamine excess and a personal and family history of the paraganglioma predisposing succinate dehydrogenase subunit B mutation. The lesion had anatomical intrapericardial juxtaposition to important cardiac anatomy and posed the significant challenge of dissection at surgery. The lesion was successfully resected via sternotomy on cardiopulmonary bypass and confirmed histopathologically as paraganglioma. Intrapericardial paraganglioma is rare and treatment is difficult and time critical considering the proximity of cardiac anatomy as well as malignant potential.