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Published on: June 11, 2019
Primary cardiac lymphoma causing right atrial occlusion
M Cikirikcioglu1, V C Keil, S M Cherian
1Department of Cardiovascular Surgery, University Hospital and Medical Faculty of Geneva, Geneva, Switzerland. mustafa.cikirikcioglu@hcuge.ch
Insights
Primary cardiac lymphoma (PCL), a rare and aggressive diffuse large B-cell lymphoma, can present insidiously. This case highlights a massive intra-cardiac tumor with rapid growth and late-stage, mild symptoms.
Area of Science:
- Cardiology
- Oncology
- Pathology
Background:
- Primary cardiac lymphomas (PCL) are exceptionally rare neoplasms.
- Diffuse large B-cell lymphoma (DLBCL) is an aggressive subtype with a poor prognosis.
- Cardiac tumors can mimic other cardiovascular conditions, delaying diagnosis.
Observation:
- A massive, lobulated tumor was identified, occluding the right atrium and infiltrating surrounding cardiac structures.
- The patient initially presented with symptoms suggestive of chronic right heart dysfunction.
- Despite surgical debulking and tricuspid annuloplasty, the patient experienced multi-organ failure.
Findings:
- Pathological analysis confirmed diffuse large B-cell lymphoma with a 100% proliferation rate.
- The tumor's size is among the largest reported for intra-cardiac PCL.
- Aggressive tumor growth occurred with surprisingly mild symptomatology until late stages.
Implications:
- This case underscores the importance of considering rare cardiac tumors in the differential diagnosis of heart dysfunction.
- The aggressive nature of intra-cardiac DLBCL necessitates prompt and comprehensive management strategies.
- Further research into early detection and novel therapeutic approaches for PCL is warranted.
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.
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