title: Primary central nervous system lymphoma: a clinicopathological study of 75 cases.
abstract: OBJECTIVE
  Pathological and clinical data in a large series of immunocompetent patients with primary lymphoma of the central nervous system (PCNSL) were analysed.
  
  
  METHODS
  We immunostained tumour specimens of 75 patients for CD3, CD4, CD5, CD8, CD10, CD20, CD30, CD79a, Bcl-2, Bcl-6, CD138, MUM1, TDT, PAX5, FOXP1 and Ki-67 and performed in situ hybridisation for Epstein-Barr virus (EBV) RNA. Eleven cases were investigated for rearrangements of BCL6, immunoglobulin heavy chain (IGH) and FOXP1 genes using fluorescent in situ hybridisation (FISH).
  
  
  RESULTS
  Histologically, most cases were classified as diffuse large B-cell lymphoma (80.2%) predominantly of centroblastic type. Immunophenotypic profiling revealed that 96% and 4% of cases corresponded to non-germinal centre and germinal centre type, respectively. FISH analysis showed t(3;14)/IGH-BCL6 in 2/11 cases and trisomy 3 in 2/11 cases. FOXP1 rearrangements were not found. At survival analysis, Karnofsky index >80 and presence of Bcl-6 expression showed independent significant association with favourable patient outcome.
  
  
  CONCLUSIONS
  PCNSL represents a histologically and immunophenotypically very homogeneous lymphoma type, probably derived from germinal centre exit B cells. The frequent overexpression of FOXP1 appears not to be related to FOXP1 gene rearrangement. Survival analyses disclosed Bcl-6 expression and high Karnofsky performance score as independent prognostic parameters associated with favourable outcome.
authors.affiliation: Department of Medicine I, Medical University of Vienna, Austria.
authors.forename: Matthias
authors.initials: M
authors.lastname: Preusser
authors.forename: Adelheid
authors.initials: A
authors.lastname: Woehrer
authors.forename: Oskar
authors.initials: O
authors.lastname: Koperek
authors.forename: Andrea
authors.initials: A
authors.lastname: Rottenfusser
authors.forename: Karin
authors.initials: K
authors.lastname: Dieckmann
authors.forename: Brigitte
authors.initials: B
authors.lastname: Gatterbauer
authors.forename: Karl
authors.initials: K
authors.lastname: Roessler
authors.forename: Irene
authors.initials: I
authors.lastname: Slavc
authors.forename: Ulrich
authors.initials: U
authors.lastname: Jaeger
authors.forename: Berthold
authors.initials: B
authors.lastname: Streubel
authors.forename: Johannes A
authors.initials: JA
authors.lastname: Hainfellner
authors.forename: Andreas
authors.initials: A
authors.lastname: Chott
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis*; D016543:Central Nervous System Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D004268:DNA-Binding Proteins / Q000235:genetics; D005260:Female; D051858:Forkhead Transcription Factors / Q000235:genetics; D020869:Gene Expression Profiling; D015321:Gene Rearrangement; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017403:In Situ Hybridization; D053208:Kaplan-Meier Estimate; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D051560:Proto-Oncogene Proteins c-bcl-6; D012097:Repressor Proteins / Q000235:genetics; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: C084103:BCL6 protein, human; D014408:Biomarkers, Tumor; D004268:DNA-Binding Proteins; C486626:FOXP1 protein, human; D051858:Forkhead Transcription Factors; D051560:Proto-Oncogene Proteins c-bcl-6; D012097:Repressor Proteins