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Alexander Birkinshaw, Ravi Kamdar, Simon Aylwin, Olaf Wendler, Daniel Sado, Left atrial paraganglioma in a patient with sarcoidosis, European Heart Journal - Cardiovascular Imaging, Volume 22, Issue 3, March 2021, Page e5, https://doi.org/10.1093/ehjci/jeaa211
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A 57-year-old female presented with palpitations. She had a background of sarcoidosis with lymphadenopathy diagnosed many years previously. On echocardiography, a mass was seen compressing the left atrium (Panel A, arrowed). On cardiovascular magnetic resonance (CMR), thoracic lymphadenopathy was seen (Panel B, arrowed) with a distinct 5 cm × 4 cm mass adjacent to the vertebra and compressing the left atrium (Panel C, arrowed). On first pass perfusion CMR, the mass took up a large amount of contrast within seconds of entering the aorta. On late gadolinium enhancement imaging, the mass was seen to be very heterogeneous (Panel C). Despite the underlying sarcoidosis, the CMR findings suggested that this was not a lymph node, with the findings more suggestive of a haemangioma. There was concern regarding the degree of compression on the left atrium and a plan was made for surgical removal. On angiography, two large vessels from right coronary artery supplied the mass (Panel D, arrowed), raising the possibility of a paraganglioma. After endocrine evaluation, plasma normetadrenaline was elevated at 7544 pmol/L (NR 120–1180). The plasma metadrenaline and plasma 3-methoxytramine were normal. The mass was ‘hot’ on meta-iodobenzylguanidine (MIBG) iodine 123 scan (Panel E, arrowed) with no uptake elsewhere, whereas with Ga68 dotatate there was uptake in both the mass and surrounding lymphadenopathy (Panel F, arrowed). Following pre-treatment with phenoxybenzamine, the mass (which was discovered to originate from the wall of the left atrium itself) was surgically removed, along with a thoracic lymph node. Histology confirmed the former was a paraganglioma with the latter showing sarcoidosis. The plasma normetadrenaline returned to normal following surgery. This case demonstrates the value of multimodality imaging in the work up of cardiac masses.
Supplementary data are available at European Heart Journal - Cardiovascular Imaging online.