Synonyms
Primary mediastinal (thymic) large B-cell lymphoma
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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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.
Johnson, P. W., & Davies, A. J. (2008). Primary mediastinal B-cell lymphoma. Hematology/American Society of Hematology Education Program, 2008, 349–358.
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.
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.
Steidl, C., & Gascoyne, R. D. (2011). The molecular pathogenesis of primary mediastinal large B-cell lymphoma. Blood, 118(10), 2659–2669.
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Olteanu, H. (2018). B-Cell Lymphoma, Mediastinal, Diffuse, and Large. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_6
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DOI: https://doi.org/10.1007/978-3-319-66796-6_6
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