Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype

Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype

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 superi...

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Bibliographic Details
Main Authors: Vijaya Gadage, Seema Kembhavi, Prabhash Kumar, Tanuja Shet
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2011-01-01
Series:Indian Journal of Pathology and Microbiology
Subjects:
Cardiac tumor
diffuse large B-cell lymphoma
non-Hodgkin′s lymphoma
Online Access:http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2011;volume=54;issue=3;spage=591;epage=593;aulast=Gadage
Description
Summary: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.
ISSN:0377-4929

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