Primary cardiac diffuse large B-cell lymphoma presenting with cardiac tamponade: a case report

Abstract

Background: Primary cardiac tumours 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 (PCL) 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 PCL remains a combination of chemotherapy with immunotherapy, with the addition of low-dose colchicine to prevent recurrence of malignant pericardial effusion.

Keywords: Cardiac tamponade; Case report; Diffuse large-cell B-cell non-Hodgkin lymphoma; Malignant pericardial effusion; Primary cardiac lymphoma.

Figure 1

Transthoracic echocardiography on admission illustrating diffuse pericardial effusion (*) with a right-sided cardiac mass in the atrioventricular groove (arrow) [parasternal (A), subcostal (B), and apical (C) approach)].

Figure 2

Cardiac magnetic resonance imaging work-up of a primary cardiac diffuse large B-cell lymphoma surrounding the right coronary artery. Four chamber imaging shows a mass located in the right atrioventricular groove and surrounding the right coronary artery, on iso- to hyperintense on T₁-weighted spectral presaturation with inversion recovery (Figure 2A) and T₂-weighted turbo spin-echo (Figure 2B) sequences. Short axis imaging shows the extension of the mass from the proximal to the distal segment of the right coronary artery (arrows), on the T₁-weighted spectral presaturation with inversion recovery sequence (Figure 2C) and short-TI inversion recovery (Figure 2D) sequences. Short axis rest perfusion shows no early contrast enhancement of the mass surrounding the right coronary artery (Figure 2E and F), while inhomogeneous contrast enhancement can be observed on T₁-weighted images with fat suppression 10 min after intravenous administration of gadolinium (Figure 2G).

Figure 3

CT coronary angiography (curved MPR reconstructions) shows complete patency of the right coronary artery without luminal stenosis or external compression by the mass.

Figure 4

¹⁸F-fluorodeoxyglucose positron emission tomography–computed tomography in the axial plane shows pathological tracer uptake of the mass (arrow in Figure 4A), with complete metabolic response after three cycles of rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone (Figure 4B).