Modern concepts in the biology, diagnosis, differential diagnosis and treatment of primary central nervous system lymphoma

ISSN
0887-6924
Date Issued
2011
Author(s)
Deckert, M.
Engert, Andreas
Ferreri, Andres J. M.
Finke, Juergen
Illerhaus, Gerald
Klapper, Wolfram
Korfel, A.
Kueppers, Ralf
Maarouf, M.
Montesinos-Rongen, Manuel
Schlegel, Uwe
Lassmann, Hans
Wiestler, Otmar D.
Siebert, Reiner
DeAngelis, L. M.
DOI
10.1038/leu.2011.169
Abstract
Recent studies addressing the molecular characteristics of PCNSL, which is defined as malignant B-cell lymphoma with morphological features of DLBCL, have significantly improved our understanding of the pathogenesis of this lymphoma entity, which is associated with an inferior prognosis as compared with DLBCL outside the CNS. This unfavorable prognosis stimulated intense efforts to improve therapy and induced recent series of clinical studies, which addressed the role of radiotherapy and various chemotherapeutic regimens. This review combines the discussion of diagnosis, differential diagnosis and recent progress in studies addressing the molecular pathogenesis as well as therapeutic options in PCNSL. Leukemia (2011) 25, 1797-1807; doi:10.1038/leu.2011.169; published online 5 August 2011