Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype.
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Date
2011-07
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Abstract
Primary cardiac lymphoma (PCL) is a rare and fatal disorder. It may often mimic other common cardiac tumors like cardiac myxoma because of similarities in the clinical presentation. We report a case of PCL of diffuse large B-cell type, in a 38-year-old, immunocompetent male who presented with superior vena cava syndrome that was excised as a myxoma. Histology revealed a large cell population diffusely and strongly expressing CD45, CD20, MUM1/IRF4 and FOXP1 hinting at an activated B-cell (ABC)-like phenotype. After four cycles of Rituximab with CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisolone) the tumor regressed completely but the patient had a relapse and subsequently succumbed to the disease confirming the aggressive nature. The aggressive behavior of PCL may be possibly linked to its ABC-like origin.
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Keywords
Cardiac tumor, diffuse large B-cell lymphoma, non-Hodgkin′s lymphoma
Citation
Gadage Vijaya, Kembhavi Seema, Kumar Prabhash, Shet Tanuja. Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype. Indian Journal of Pathology & Microbiology. 2011 Jul-Sept 54(3): 591-593.