title: Modern concepts in the biology, diagnosis, differential diagnosis and treatment of primary central nervous system lymphoma.
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.
authors.affiliation: Department of Neuropathology, University Hospital of Cologne, Cologne, Germany. martina.deckert@uni-koeln.de
authors.forename: M
authors.initials: M
authors.lastname: Deckert
authors.forename: A
authors.initials: A
authors.lastname: Engert
authors.forename: W
authors.initials: W
authors.lastname: Brück
authors.forename: A J M
authors.initials: AJ
authors.lastname: Ferreri
authors.forename: J
authors.initials: J
authors.lastname: Finke
authors.forename: G
authors.initials: G
authors.lastname: Illerhaus
authors.forename: W
authors.initials: W
authors.lastname: Klapper
authors.forename: A
authors.initials: A
authors.lastname: Korfel
authors.forename: R
authors.initials: R
authors.lastname: Küppers
authors.forename: M
authors.initials: M
authors.lastname: Maarouf
authors.forename: M
authors.initials: M
authors.lastname: Montesinos-Rongen
authors.forename: W
authors.initials: W
authors.lastname: Paulus
authors.forename: U
authors.initials: U
authors.lastname: Schlegel
authors.forename: H
authors.initials: H
authors.lastname: Lassmann
authors.forename: O D
authors.initials: OD
authors.lastname: Wiestler
authors.forename: R
authors.initials: R
authors.lastname: Siebert
authors.forename: L M
authors.initials: LM
authors.lastname: DeAngelis
mesh_terms: D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy*; D006801:Humans