Primary Cardiac Lymphoma Causing Right Atrial Occlusion

Abstract

Primary cardiac lymphomas (PCL) are extremely rare, and diffuse large B-cell lymphoma is a highly aggressive subtype. We report a case that was initially diagnosed as chronic right heart dysfunction. Detailed investigations revealed a large lobulated tumour occluding the right atrium, infiltrating the inter-atrial septum, the roof of the left atrium, and the aortic root. Despite adequate surgical debulking and initial successful tricuspid annuloplasty, the patient succumbed to multi-organ failure. Pathological analysis of the resected tumour confirmed a diffuse large B-cell lymphoma with a proliferation rate of 100 %. What is unique about this case is the size of this rare cardiac tumour, which we believe to be one of the largest described in the literature for a purely intra-cardiac PCL, its aggressive growth rate, and the relatively mild symptomatology until a late stage of the disease.

Key words

Heart disease - cardiovascular surgery - cardiomyopathy

References

• 1 Burke A, Virmani R. Tumors of the heart and great vessels.. In: Rosai J, Sobin L H, eds. Atlas of Tumor Pathology.. Vol. 16 3rd ed. Washington DC: Armed Forces Institute of Pathology; 1996
• 2 Nascimento A F, Winters G L, Pinkus G S. Primary cardiac lymphoma: clinical, histologic, immunophenotypic, and genotypic features of 5 cases of a rare disorder.  Am J Surg Pathol. 2007;  31 1344-1350
• 3 Chalabreysse L, Berger F, Loire R, Devouassoux G, Cordier J F, Thivolet-Bejui F. Primary cardiac lymphoma in immunocompetent patients : a report of three cases and review of the literature.  Virchows Arch. 2002;  441 456-461
• 4 Stefani P M, Laurino L, Zanatta L, Valori L, dei Tos A P, Gherlinzoni F. Primary cardiac onset of disseminated Burkitt's lymphoma.  Ann Hematol. 2008;  87 487-488