Teaching Point: Familial dilated cardiomyopathy (DCM) predisposes to malignant ventricular arrhythmias and sudden cardiac death, and magnetic resonance imaging (MRI) has important diagnostic value in demonstrating non-ischemic patterns of late gadolinium enhancement (LGE).

Teaching Point: Although magnetic resonance imaging (MRI) plays role in the detection and differentiation of chondrosarcoma (e.g., by cortical breakthrough, peritumoral soft tissue edema, and extra-osseous extension), it is important to be aware of atypical features of common bone tumors.

Background Primary cardiac tumors are extremely rare with an autopsy incidence of 0.05%. They can present with a variety of symptoms, including life threatening arrhythmia and cardiac tamponade. In this case report, we focus on the diagnostic process and management of a primary cardiac lymphoma presenting with cardiac tamponade. Case summary We report on a 71-year-old male presenting with a large pericardial effusion, tamponade, and a mass in the right atrioventricular groove. Multimodality imaging was performed, including transthoracic echocardiography, computed tomography, magnetic resonance imaging, positron emission tomography and computed tomography-guided transthoracic biopsy The final diagnosis of a double-hit diffuse large cell B-cell lymphoma was made, for which treatment consisting of a combination of chemotherapy and immunotherapy was initiated. Low-dose colchicine was also added to the treatment. Discussion Primary cardiac lymphoma remains a very rare diagnosis and this case highlights the need for multimodality imaging and imaging-guided biopsy to differentiate cardiac masses. First-line treatment for primary cardiac lymphoma remains a combination of chemotherapy with immunotherapy, with addition of low-dose colchicine to prevent recurrence of malignant pericardial effusion.

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