Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

B-Cell Lymphoma, Mediastinal, Diffuse, and Large

  • Horatiu Olteanu
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-66796-6_6

Synonyms

Primary mediastinal (thymic) large B-cell lymphoma

Definition

Primary mediastinal (thymic) large B-cell lymphoma (PMLBCL) is a diffuse large B-cell lymphoma (DLBCL) of putative thymic origin, arising in the anterior mediastinum and showing distinct clinical and pathologic features (Cazals-Hatem et al. 1996; Johnson and Davies 2008).

Clinical Features

  • Incidence

    PMLBCL is an uncommon neoplasm, representing 2–4% of non-Hodgkin lymphomas and approximately 6% of all adult lymphomas.

  • Age

    Young adults are most commonly affected, with a median age of presentation of 35 years, although the age range is fairly broad (15–73 years, in a series of 29 cases).

  • Sex

    There is a female predominance (male to female = 1:2) in those diagnosed with PMLBCL.

  • Site

    Clinically, patients present with a bulky, anterior mediastinal mass, which commonly extends to the pericardium, pleura, or lung and causes superior vena cava invasion or compression. There is very rare systemic, lymph node, CNS, and bone...

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References and Further Reading

  1. Cazals-Hatem, D., Lepage, E., Brice, P., Ferrant, A., d'Agay, M. F., Baumelou, E., et al. (1996). Primary mediastinal large B-cell lymphoma. A clinicopathologic study of 141 cases compared with 916 nonmediastinal large B-cell lymphomas, a GELA (“Groupe d’Etude des Lymphomes de l’Adulte”) study. American Journal of Surgical Pathology, 20(7), 877–888.CrossRefPubMedGoogle Scholar
  2. Johnson, P. W., & Davies, A. J. (2008). Primary mediastinal B-cell lymphoma. Hematology/American Society of Hematology Education Program, 2008, 349–358.Google Scholar
  3. Rieger, M., Osterborg, A., Pettengell, R., White, D., Gill, D., Walewski, J., et al. (2011). Primary mediastinal B-cell lymphoma treated with CHOP-like chemotherapy with or without rituximab: results of the Mabthera International Trial Group Study. Annals of Oncology, 22(3), 664–670.CrossRefPubMedGoogle Scholar
  4. Savage, K. J., Al-Rajhi, N., Voss, N., Paltiel, C., Klasa, R., Gascoyne, R. D., et al. (2006). Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience. Annals of Oncology, 17(1), 123–130.CrossRefPubMedGoogle Scholar
  5. Steidl, C., & Gascoyne, R. D. (2011). The molecular pathogenesis of primary mediastinal large B-cell lymphoma. Blood, 118(10), 2659–2669.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Medical College of WisconsinMilwaukeeUSA
  2. 2.Department of Pathology, Mayo ClinicRochesterUSA