Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Primary Mediastinal (Thymic) Large B-cell Lymphoma

  • Gabriel K. GriffinEmail author
  • Scott J. Rodig
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_3813-1

Synonyms

Definition

Primary mediastinal large B-cell lymphoma (PMBL) is a large cell lymphoma of thymic medullary B-cell origin that exhibits clinicopathologic and molecular features that are more similar to classical Hodgkin lymphoma (CHL) than conventional diffuse large B-cell lymphoma (DLBCL). PMBL typically occurs in younger patients, shows a female predominance, and demonstrates characteristic genetic and immunophenotypic features related to activation of the NF-KB and JAK-STAT pathways. In addition, PMBL shows frequent structural alterations of chromosome 9p24 leading to upregulation of the immunoregulatory proteins PD-L1 and PD-L2. Due to these unique features, PMBL is considered as a distinct diagnostic category within the World Health Organization (WHO) Classification of Tumors of Hematopoietic and Lymphoid Tissues.

Clinical Features

  • Incidence

    PMBL comprises approximately 2–4% of incident non-Hodgkin...

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References and Further Reading

  1. Bledsoe, J. R., et al. (2016). The immunophenotypic spectrum of primary mediastinal large B-cell lymphoma reveals prognostic biomarkers associated with outcome. American Journal of Hematology, 91(10), E436–E441.CrossRefGoogle Scholar
  2. Chapuy, B., et al. (2016). Targetable genetic features of primary testicular and primary central nervous system lymphomas. Blood, 127(7), 869–881.CrossRefGoogle Scholar
  3. Chapuy, B. et al. (2018). Comprehensive Genomic analysis of primary mediastinal B-cell lymphoma (abstract). American society of hematology annual meeting. San Diego.Google Scholar
  4. Dorfman, D. M., et al. (2012). Utility of CD200 immunostaining in the diagnosis of primary mediastinal large B cell lymphoma: comparison with MAL, CD23, and other markers. Modern Pathology, 25(12), 1637–1643.CrossRefGoogle Scholar
  5. Dunleavy, K., et al. (2013). Dose-adjusted EPOCH-rituximab therapy in primary mediastinal B-cell lymphoma. The New England Journal of Medicine, 368(15), 1408–1416.CrossRefGoogle Scholar
  6. Green, M. R., et al. (2010). Integrative analysis reveals selective 9p24.1 amplification, increased PD-1 ligand expression, and further induction via JAK2 in nodular sclerosing Hodgkin lymphoma and primary mediastinal large B-cell lymphoma. Blood, 116(17), 3268–3277.CrossRefGoogle Scholar
  7. Gunawardana, J., et al. (2014). Recurrent somatic mutations of PTPN1 in primary mediastinal B cell lymphoma and Hodgkin lymphoma. Nature Genetics, 46(4), 329–335.CrossRefGoogle Scholar
  8. Ritz, O., et al. (2009). Recurrent mutations of the STAT6 DNA binding domain in primary mediastinal B-cell lymphoma. Blood, 114(6), 1236–1242.CrossRefGoogle Scholar
  9. Shi, M., et al. (2014). Expression of programmed cell death 1 ligand 2 (PD-L2) is a distinguishing feature of primary mediastinal (thymic) large B-cell lymphoma and associated with PDCD1LG2 copy gain. The American Journal of Surgical Pathology, 38(12), 1715–1723.CrossRefGoogle Scholar
  10. Steidl, C., et al. (2011). MHC class II transactivator CIITA is a recurrent gene fusion partner in lymphoid cancers. Nature, 471(7338), 377–381.CrossRefGoogle Scholar
  11. Swerdlow, S. H., et al. (2017). WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours of haematopoietic and lymphoid tissue 4th. Geneva. http://publications.iarc.fr/Book-And-Report-Series/Who-Iarc-Classification-Of-Tumours/Who-Classification-Of-Tumours-Of-Haematopoietic-And-Lymphoid-Tissues-2017.
  12. Vigano, E., et al. (2018). Somatic IL4R mutations in primary mediastinal large B-cell lymphoma lead to constitutive JAK-STAT signaling activation. Blood, 131(18), 2036–2046.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of PathologyBrigham and Women’s HospitalBostonUSA