https://orcid.org/0000-0001-6391-7244
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Stephani C Wang, Chris W Pan, Overcoming the diagnostic challenges in a high-risk invasive primary cardiac lymphoma, European Heart Journal - Cardiovascular Imaging, Volume 21, Issue 6, June 2020, Page 707, https://doi.org/10.1093/ehjci/jez324
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Extract
Primary cardiac lymphoma is uncommon and challenging to diagnose and treat. A 58-year-old man presented to the emergency room with acute chest pain and shortness of breath and was found to be in complete heart block. Medical history was significant for cardiac tamponade requiring emergent pericardial window, cauda-equina syndrome, and thrombocytopenia diagnosed several months prior to the admission. Aetiologies were unclear despite extensive work-up, including negative pericardial fluid cytology. Transthoracic echocardiogram revealed new right atrial masses, recurrent pericardial effusion. Subsequent transoesophageal echocardiogram (TOE) disclosed diffuse intra/pericardial masses (Panel A). Cardiac magnetic resonance imaging (MRI) revealed extensive masses (*) in epicardiac, intracardiac, intramyocardial, and interatrial septal regions (Panel B). The masses invaded epicardial atrioventricular, encasing the right coronary artery (white arrow) and left circumflex artery (black arrow) (Panel C). In addition, there was a mass along the anterior medial surface abutting the pericardial lining. Coronary computed tomography (CT) angiogram reinforced above findings. Tissue diagnosis was challenged by severe refractory thrombocytopenia. Eventually, a temporary transvenous pacemaker was placed, followed by left robotic assisted transthoracic biopsy of the mass abutting the pericardial lining. Final cytology showed primary cardiac large B-cell lymphoma. Chemotherapy (EPOCH-R) was then initiated, and 3 days after treatment, a repeat TOE showed significant regression in tumour burden (Panel D). This was a rare case of an invasive primary cardiac lymphoma with complete heart block, refractory thrombocytopenia, and paraneoplastic cauda-equina syndrome. This complex case highlights the significance of multiple diagnostic modalities including echocardiogram, coronary CT angiogram, and cardiac MRI in addition to minimally invasive tissue biopsy in diagnosing cardiac tumours.
