Primary cardiac lymphoma causing right atrial occlusion

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.