Primary cardiac lymphoma presenting with cardiac tamponade and complete heart block: case report

Abstract

Background: Primary cardiac lymphoma (PCL) is rare and its presentation can be variable. Thorough workup of suspected PCL or other cardiac tumours typically incorporates a range of imaging techniques and laboratory investigations but ultimately diagnosis is confirmed by histological analysis of myocardial tissue.

Case summary: An 80-year-old Caucasian female presented with complete heart block and symptomatic cardiac tamponade. A pericardiocentesis was performed and a dual-chamber permanent pacemaker was implanted for the management of her complete heart block. Subsequently, a right atrial mass was discovered on imaging and the patient underwent endomyocardial biopsy of the mass. Histological analysis of the sample confirmed a primary cardiac lymphoma. The patient opted to forgo treatment with chemotherapy and died from her disease 1 month later.

Discussion: Cardiac arrhythmias can occur in PCL due to infiltration of conduction pathways. Characterization of cardiac masses on various imaging modalities and laboratory tests guides diagnosis. Tissue diagnosis is required to confirm PCL. The location of a cardiac mass may present technical challenges when undertaking a biopsy, so the best approach to tissue sampling should be considered on an individual basis. Without treatment survival is less than one month but with modern chemoimmunotherapy five-year survival may exceed 50%.

Keywords: Cardiac tamponade; Case report; Complete heart block; Endomyocardial biopsy; Multimodality cardiac imaging; Primary cardiac lymphoma.

Figure 1

Third-degree heart block with small QRS complexes on electrocardiogram.

Figure 2

Transthoracic echo shows a large circumferential pericardial effusion measuring 2.7 cm at its largest, with evidence of right chamber collapse.

Figure 3

Lobulated soft tissue mass extending into the musculature of the right atrium and bilateral pleural effusions on computed tomography thorax.

Figure 4

Transoesophageal echocardiogram and fluoroscopy-guided endomyocardial biopsy of cardiac mass. A steerable Agilis catheter is used to position the bioptome at the anterior right atrial wall to obtain tissue sample.

Figure 5

Histology and immunohistology of myocardial tissue: (A) Histology shows myocardial tissue infiltrated by pleomorphic medium to large-sized lymphoid cells with round vesicular nuclei and prominent nucleoli (haematoxylin and eosin staining; ×40). (B) Immunohistochemistry shows tumour cells strongly positive for CD20 (× 40). (C) Immunohistochemistry using Ki-67 shows a high proliferation index of ∼70% (× 40).