title: Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype.
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.
authors.affiliation: Department of Pathology, Tata Memorial Hospital, Mumbai, India.
authors.forename: Vijaya
authors.initials: V
authors.lastname: Gadage
authors.forename: Seema
authors.initials: S
authors.lastname: Kembhavi
authors.forename: Prabhash
authors.initials: P
authors.lastname: Kumar
authors.forename: Tanuja
authors.initials: T
authors.lastname: Shet
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D018951:Antigens, CD20 / Q000096:biosynthesis; D000970:Antineoplastic Agents / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001402:B-Lymphocytes / Q000276:immunology*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D017809:Fatal Outcome; D051858:Forkhead Transcription Factors / Q000096:biosynthesis; D020869:Gene Expression Profiling; D006338:Heart Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology*; D006651:Histocytochemistry; D006801:Humans; D007150:Immunohistochemistry; D050835:Interferon Regulatory Factors / Q000096:biosynthesis; D017493:Leukocyte Common Antigens / Q000096:biosynthesis; D016393:Lymphoma, B-Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D008853:Microscopy; D010641:Phenotype; D011241:Prednisone / Q000008:administration & dosage; D013902:Radiography, Thoracic; D012008:Recurrence; D012097:Repressor Proteins / Q000096:biosynthesis; D000069283:Rituximab; D013479:Superior Vena Cava Syndrome / Q000175:diagnosis* / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D000099218:Interferon Regulatory Factor-4
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D000970:Antineoplastic Agents; D003520:Cyclophosphamide; D004317:Doxorubicin; D051858:Forkhead Transcription Factors; D050835:Interferon Regulatory Factors; D017493:Leukocyte Common Antigens; D011241:Prednisone; D012097:Repressor Proteins; D000069283:Rituximab; D014750:Vincristine; D000099218:Interferon Regulatory Factor-4; C486626:FOXP1 protein, human; C516769:PTPRC protein, human