title: Epstein-Barr virus-associated primary central nervous system cytotoxic T-cell lymphoma.
abstract: Primary central nervous system lymphoma (PCNSL) expressing T-cell markers is rare, among which nasal-type extranodal NK/T-cell lymphoma is an extremely rare subtype associated with Epstein-Barr virus (EBV) infection. Here we report the clinicopathologic features of a case of EBV-associated PCNSL showing a cytotoxic T-cell phenotype. The patient, a 73-year-old woman, presented with rapidly progressive mental deterioration. Brain MRI revealed multiple lesions with swelling in the bilateral cerebral hemispheres, which were hypointense on T1-weighted images, hyperintense on T2-weighted and fluid-attenuated inversion recovery images, and slightly hyperintense on diffusion-weighted images. Biopsy specimens from the temporal region showed many medium-sized anaplastic lymphocytic cells with perivascular and angio-invasive patterns in the cortex. Immunohistochemically, the cells were positive for CD3, CD8, T-cell-restricted intracellular antigen-1 (TIA-1), granzyme B and perforin, but negative for CD56 and CD20. In situ hybridization revealed EBV-encoded RNAs in the tumor cell nuclei. A rearrangement study showed T-cell receptor γ-chain gene rearrangement with a clonal appearance. The patient died 6 months after surgery, and a general autopsy revealed no lymphoma cells outside the brain. These cellular profiles are inconsistent with those of extranodal NK/T-cell lymphoma, and have not been previously described. This case appears to represent an unusual CNS manifestation of EBV-associated T-cell lymphoma.
authors.affiliation: Department of Neurosurgery, University of Niigata, Niigata, Japan.
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Ogura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Aoki
authors.forename: Manabu
authors.initials: M
authors.lastname: Natsumeda
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shimizu
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Tomohisa
authors.initials: T
authors.lastname: Saito
authors.forename: Jun
authors.initials: J
authors.lastname: Takizawa
authors.forename: Kouichirou
authors.initials: K
authors.lastname: Okamoto
authors.forename: Go
authors.initials: G
authors.lastname: Hasegawa
authors.forename: Hajime
authors.initials: H
authors.lastname: Umezu
authors.forename: Kouichi
authors.initials: K
authors.lastname: Ohshima
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Fujii
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Kakita
mesh_terms: D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology* / Q000821:virology*; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000473:pathology; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000473:pathology* / Q000821:virology*
chemical_list: D014408:Biomarkers, Tumor
keywords: CD8; Epstein-Barr virus; NK/T-cell lymphoma; cytotoxic T-cell; pathology; primary central nervous system lymphoma