Primary cardiac lymphoma (PCL) is a rare neoplasm involving the heart, pericardium or both. Patients with PCL have a median survival of approximately 7 months based on literature. We report a unique case of a 63-year-old woman presenting with worsening exertional dyspnoea and symptoms of superior vena cava obstruction. Investigations revealed a large right atrial (RA) tumour with obstructive features. In the past, PCL was frequently diagnosed at postmortem but with modern imaging and biopsy techniques, this allows early diagnosis and treatment of this rapidly fatal disease. Our patient had a trans-jugular venous biopsy of the RA mass under trans-oesophageal echocardiography guidance. This confirmed the diagnosis of PCL. She subsequently completed seven cycles of chemotherapy and is currently in remission 20 months after diagnosis.
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