Background: We report a case of a 51-year-old man with functioning cardiac paraganglioma diagnosed subsequent to a primary coronary event. Following a myocardial infarction aged 39; he was incidentally found to have a cardiac tumour in the pericardial sac. This was assumed to be benign and followed up with serial imaging. Recent scans suggested a possible paraganglioma with evidence of very slow tumour growth leading to an endocrinology referral. On further questioning he did report episodic sweating with palpitations & light-headedness. Investigations He had significantly raised urinary catecholamine levels (noradrenaline 2072 & 1793 nmol/24 hrs, normetarenaline 3482 & 2788 nMol/24 hours) CT chest confirmed the mass lying close to the left ventricle, as previously noted, now measuring 44x42 mm. MIBG scanning confirmed functional neuroendocrine tissue in the concordant area. Genetics- Heterozygous SDHD gene mutation Management -On a combination of alpha and beta-blockers. -Awaiting coronary angiography before proceeding for tumour resection -Family has been offered genetic screening.
Discussion: Cardiac paragangliomas are extremely rare benign tumors with less than 60 reported cases in the literature. Paragangliomas most commonly occur in the third to fourth decade of life typically with symptoms of catecholamine excess. These tumors are hormonally active in approximately 35% to 50% of cases, and their incidence of malignancy is approximately 10%. Although mostly sporadic, occasional familial cases are reported in the literature from a variety of genetic diseases. Echocardiography, coronary angiography and MIBG scanning are useful diagnostic modalities for paragangliomas. Surgical resection, though technically challenging, remain the gold standard treatment. Given their increased risk of recurrence, close follow up is warranted. Our case has a two-fold significance: firstly it adds to the current pool of cases of cardiac paragangliomas; secondly it raises awareness amongst endocrinologists regarding this rare & diagnostically challenging condition but with a favourable outcome following early intervention.
Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.
Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.