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.
Subject(s)
Adult , Antibodies, Monoclonal, Murine-Derived/administration & dosage , Antigens, CD20/biosynthesis , Leukocyte Common Antigens/biosynthesis , Antineoplastic Agents/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , B-Lymphocytes/immunology , Cyclophosphamide/administration & dosage , Doxorubicin/administration & dosage , Fatal Outcome , Forkhead Transcription Factors/biosynthesis , Gene Expression Profiling , Heart Neoplasms/complications , Heart Neoplasms/diagnosis , Heart Neoplasms/drug therapy , Heart Neoplasms/pathology , Histocytochemistry , Humans , Immunohistochemistry , Interferon Regulatory Factors/biosynthesis , Lymphoma, B-Cell/complications , Lymphoma, B-Cell/diagnosis , Lymphoma, B-Cell/drug therapy , Lymphoma, B-Cell/pathology , Male , Microscopy , Phenotype , Prednisone/administration & dosage , Radiography, Thoracic , Recurrence , Repressor Proteins/biosynthesis , Superior Vena Cava Syndrome/diagnosis , Superior Vena Cava Syndrome/pathology , Tomography, X-Ray Computed , Treatment Outcome , Vincristine/administration & dosageABSTRACT
Hematopoietic neoplasm coexpressing CD4 and CD56 includes a subset of acute myeloid leukemia with myelomonocytic differentiation, plasmacytoid monocyte tumor, and other immature hematopoietic neoplasms of undefined origin. Herein, we report a CD4+CD56+CD68+ hematopoietic tumor that was thought to be a tumor of plasmacytoid monocytes. This case is unique in the absence of accompanying myelomonocytic leukemia and the faint expression of cCD3 on the tumor cells. The patient was a 22-yr old man presented with multiple lymphadenopathy and an involvement of the bone marrow. Tumor cells were large and monomorphic with an angulated eosinophilic cytoplasm of moderate amount. Nuclei of most tumor cells were eccentric and round with one or two prominent nucleoli. Rough endoplasmic reticulum was prominent in electron microscopic examination. Tumor cells expressed CD4, CD7, CD10, CD45RB, CD56, CD68, and HLA-DR and were negative for CD1a, CD2, sCD3, CD5, CD13, CD14, CD20, CD33, CD34, CD43, CD45RA, TIA-1, S-100, and TdT. cCD3 was not detected in the immunostaining using paraffin tissue, but was faintly expressed in flow cytometry and immunostaining using a touch imprint slide. T-cell receptor gene rearrangement analysis and EBV in situ hybridization showed negative results. Cytochemically, myeloperoxidase, Sudan black B, and alpha naphthyl butyrate esterase were all negative.