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Primary Mediastinal Large B-Cell Lymphoma

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Rare Lymphomas

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

Abstract

Primary mediastinal large B-cell lymphoma (PMBCL) is a unique type of B-cell lymphoma probably arising from a putative thymic medullary B cell. It constitutes 6–10 % of all diffuse large B-cell lymphomas (DLBCL), occurring more often in young females. It is characterised by a diffuse proliferation of medium to large B cells associated with sclerosis and a degree of compartmentalisation. CD30 staining is observed in the vast majority of cases (~80 %), although it is weaker and less homogeneous than in cHL and anaplastic large-cell lymphoma. Molecular analysis shows it to be distinct from other types of DLBCL. PMBCL is characterised by an invasive anterior mediastinal mass, often producing local compressive symptoms. Some retrospective analyses suggested that it might respond better to third-generation chemotherapy regimens than to the more commonly used CHOP. However, the addition of rituximab reduced these differences and R-CHOP is now the most widely used treatment, as it is for other types of DLBCL. The role of consolidation with local radiotherapy, which is often used to treat residual mediastinal masses, still remains unclear. Treatment with R-CHOP followed by radiation therapy has excellent results, with a 5-year survival of 70–85 %. The role of FDG-PET scanning is the subject of prospective studies, and it is hoped that in the future, this will allow the de-escalation of radiation therapy based upon more accurate prognostic information as treatment proceeds.

Pathology: Jan Delabie and Scott Rodig

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Authors and Affiliations

  1. Cancer Research UK Centre, University Hospital Southampton, Southampton, UK

    Peter Johnson

  2. Department of Pathology, Oslo University Hospital, Oslo, Norway

    Jan Delabie

  3. Department of Pathology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Scott Rodig MD

  4. Division of Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University of Rome, Rome, Italy

    Maurizio Martelli

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  1. Peter Johnson
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  2. Jan Delabie
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  3. Scott Rodig MD
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  4. Maurizio Martelli
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Correspondence to Peter Johnson .

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Editors and Affiliations

  1. Medizinischen Klinik und Poliklinik III, Universität München Klinikum Großhadern, München, Germany

    Martin Dreyling

  2. Division of Hematology/Oncology and Cancer Center, University of Virginia Health System, Charlottesville, Virginia, USA

    Michael E. Williams

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Johnson, P., Delabie, J., Rodig, S., Martelli, M. (2014). Primary Mediastinal Large B-Cell Lymphoma. In: Dreyling, M., Williams, M. (eds) Rare Lymphomas. Hematologic Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-39590-1_10

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  • DOI: https://doi.org/10.1007/978-3-642-39590-1_10

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