Cardiac paraganglioma is one of the most infrequent primary cardiac tumours, with <50 cases reported in the literature. Left atrium is the most common location. This is a case of a 57-year-old gentleman who presented with 7 year history of gradually increasing cranial perspiration, irritability, anxiety, unexplained feelings of fear, facial erythema, hypertension and atrial fibrillation. Following investigations of atrial fibrillation, a cardiac MRI scan revealed a mass between the left atrium and right pulmonary artery and a subsequent biomedical testing revealed significantly elevated urinary normetadrenaline and 3 methoxytyramine levels. A MIBG scan confirmed localised uptake in the mediastinal mass situated behind the aorta, below the pulmonary artery, with no evidence of distant metastasis. The mediastinal tumour excised via a median sternotomy, proved morphologically and immunophenotypically, to be most in keeping with a paraganglioma. The genetic studies showed an alteration in one copy of the SDHB gene, confirming a hereditary phaeochromocytoma and paraganglioma syndrome. Mediastinal paragangliomas as well as being rare as described in the medical literature, remain a surgical challenge due to the hypervascular character and firm adhesion to adjacent mediastinal structures. Cardiac paragangliomas may compress cardiac structures and invade adjacent structures, pertaining a high cardiovascular morbidity and mortality risk without treatment.
02 Dec 2019
UK and Ireland Neuroendocrine Tumour Society