Primary mediastinal (thymic) large B-cell lymphoma: a short review with brief discussion of mediastinal gray zone lymphoma.
ABSTRACT Primary mediastinal (thymic) large B-cell lymphoma (PMBCL) is a subtype of diffuse large B-cell lymphoma (DLBCL). It commonly presents as a bulky lesion in the anterior-superior mediastinum with symptoms related to local invasion or compression. Microscopic examination typically shows infiltration of medium-large cells surrounded by collagen fibrosis. The neoplastic cells express B-cell markers, and CD30 often shows heterogeneous staining. Comparative genomic hybridization has identified gains in loci of 9p24 and 2p15 as well as Xp11.4-21 and Xq24-26. Amplification of REL and BCL11A at 2p as well as elevated expression of JAK2, PDL1, and PDL2 at 9p has been demonstrated. Nodular sclerosis classic Hodgkin lymphoma needs to be differentiated from PMBCL and cases with overlapped features have been described as mediastinal gray zone lymphoma. Primary mediastinal (thymic) large B-cell lymphoma carries a favorable prognosis in comparison to conventional DLBCL.
SourceAvailable from: Giorgio Treglia[Show abstract] [Hide abstract]
ABSTRACT: We report a rare case of thymic Hodgkin lymphoma that occurred in an elderly patient and detected by F-FDG PET/CT. A 77-year-old woman with fever of unknown origin underwent F-FDG PET/CT scanning, which showed an area of increased radiopharmaceutical uptake in a mediastinal mass corresponding to a thymic lesion at CT scan. On the basis of the PET/CT findings, the patient was referred to surgery with the clinical suspicious of a thymoma. Histological examination demonstrated the presence of a primary thymic Hodgkin lymphoma instead.Clinical nuclear medicine 01/2013; DOI:10.1097/RLU.0b013e318252e01b · 2.86 Impact Factor
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ABSTRACT: The pathogenesis of primary mediastinal large B-cell lymphoma (PMBCL) is incompletely understood. Recently, specific genotypic and phenotypic features have been linked to tumor cell immune escape mechanisms in PMBCL. We studied 571 B-cell lymphomas with a focus on PMBCL. Using fluorescence in situ hybridization, here, we report that the programmed death ligand (PDL) locus (9p24.1) is frequently and specifically rearranged in PMBCL (20%) as compared to diffuse large B-cell lymphoma, follicular lymphoma and Hodgkin lymphoma. Rearrangement was significantly correlated with over-expression of PDL transcripts. Employing high-throughput sequencing techniques, we characterized novel translocations and chimeric fusion transcripts involving PDLs at base-pair resolution. Our data suggest that recurrent genomic rearrangement events underlie an immune privilege phenotype in a subset of B-cell lymphomas.Blood 02/2014; DOI:10.1182/blood-2013-10-535443 · 9.78 Impact Factor
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ABSTRACT: We report a case of CD20+ grey zone lymphoma (GZL) with Reed Sternberg cells as an unexpected pathological diagnosis of a destroyed right lower lobe after necrotizing pneumonia. These GZLs show overlapping features of classical Hodgkin's lymphoma and diffuse large B-cell lymphoma. GZLs are a rare specific entity of lymphomas, and the aetiology is unknown. The diagnosis is confirmed by pathological, immunohistochemical and molecular analyses. The optimal treatment is unknown. The prognosis of these patients is poor.European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 12/2012; 43(3). DOI:10.1093/ejcts/ezs611 · 2.81 Impact Factor