Gadage, VijayaKembhavi, SeemaKumar, PrabhashShet, Tanuja2012-10-112012-10-112011-07Gadage 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.http://imsear.searo.who.int/handle/123456789/142052Primary 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.enCardiac tumordiffuse large B-cell lymphomanon-Hodgkin′s lymphomaAdultAntibodies, Monoclonal, Murine-Derived --administration & dosageAntigens, CD20 --biosynthesisAntigens, CD45 --biosynthesisAntineoplastic Agents --administration & dosageAntineoplastic Combined Chemotherapy Protocols --administration & dosageB-Lymphocytes --immunologyCyclophosphamide --administration & dosageDoxorubicin --administration & dosageFatal OutcomeForkhead Transcription Factors --biosynthesisGene Expression ProfilingHeart Neoplasms --complicationsHeart Neoplasms --diagnosisHeart Neoplasms --drug therapyHeart Neoplasms --pathologyHistocytochemistryHumansImmunohistochemistryInterferon Regulatory Factors --biosynthesisLymphoma, B-Cell --complicationsLymphoma, B-Cell --diagnosisLymphoma, B-Cell --drug therapyLymphoma, B-Cell --pathologyMaleMicroscopyPhenotypePrednisone --administration & dosageRadiography, ThoracicRecurrenceRepressor Proteins --biosynthesisSuperior Vena Cava Syndrome --diagnosisSuperior Vena Cava Syndrome --pathologyTomography, X-Ray ComputedTreatment OutcomeVincristine --administration & dosagePrimary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype.Article