title: Prognostic significance of the aggregative perivascular growth pattern of tumor cells in primary central nervous system diffuse large B-cell lymphoma.
abstract: BACKGROUND
  Primary central nervous system lymphomas, predominantly diffuse large B-cell lymphomas (PCNS-DLBCL), are aggressive malignancies, and no histopathological variables with independent prognostic value are currently available. The aim of this study is to determine the prognostic value of histopathological variables of PCNS-DLBCL.
  
  
  METHODS
  Aggregative perivascular tumor cells (APVTs) and reactive perivascular T cell infiltrates (RPVIs) in tumor samples from 62 immunocompetent patients with PCNS-DLBCL were histopathologically and immunohistochemically studied. A mouse brain DLBCL model was established to confirm the special morphological features of PCNS-DLBCL. The therapy, overall response rate (ORR), and overall survival (OS) among patients were followed up.
  
  
  RESULTS
  APVT was present in 54 (87%) of the 62 cases, whereas RPVI was present in 20 (32%). Patients with APVT-positive lesions exhibited significantly worse OS, with intermediate to high International Extranodal Lymphoma Study Group (IELSG) scores, compared with patients with RPVI-positive lesions. Among cases of APVT-positive lymphoma, the semiquantitative score of immunostaining of X-box-binding protein (XBP1) and CD44 demonstrated prognostic significance. Multivariate analysis confirmed independent associations between APVT and XBP1 and between CD44 staining and survival.
  
  
  CONCLUSIONS
  The presence of APVT and staining of XBP1 and CD44 are independently associated with survival among patients with PCNS-DLBCL. These features could be routinely assessed in histopathological and immunohistochemical specimens.
authors.affiliation: Department of Pathology, Changhai Hospital, Shanghai, 200433, China.
authors.forename: Miaoxia
authors.initials: M
authors.lastname: He
authors.forename: Changjing
authors.initials: C
authors.lastname: Zuo
authors.forename: Jianjun
authors.initials: J
authors.lastname: Wang
authors.forename: Jianmin
authors.initials: J
authors.lastname: Liu
authors.forename: Binghua
authors.initials: B
authors.lastname: Jiao
authors.forename: Jianmin
authors.initials: J
authors.lastname: Zheng
authors.forename: Zailong
authors.initials: Z
authors.lastname: Cai
mesh_terms: D000328:Adult; D000368:Aged; D000818:Animals; D014408:Biomarkers, Tumor / Q000378:metabolism*; D016543:Central Nervous System Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D004268:DNA-Binding Proteins / Q000378:metabolism; D004195:Disease Models, Animal; D005260:Female; D006801:Humans; D018960:Hyaluronan Receptors / Q000378:metabolism; D007124:Immunoenzyme Techniques; D016130:Immunophenotyping; D016403:Lymphoma, Large B-Cell, Diffuse / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D008297:Male; D051379:Mice; D008807:Mice, Inbred BALB C; D008875:Middle Aged; D054973:Perivascular Epithelioid Cell Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D000071701:Regulatory Factor X Transcription Factors; D015996:Survival Rate; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D014157:Transcription Factors / Q000378:metabolism; D000071717:X-Box Binding Protein 1
chemical_list: D014408:Biomarkers, Tumor; D004268:DNA-Binding Proteins; D018960:Hyaluronan Receptors; D000071701:Regulatory Factor X Transcription Factors; D014157:Transcription Factors; D000071717:X-Box Binding Protein 1; C000605543:XBP1 protein, human; C000605544:Xbp1 protein, mouse
keywords: central nervous system; diffuse large B-cell lymphoma; histopathology; prognosis