title: Primary central nervous system cytotoxic/suppressor T-cell lymphoma: report of a unique case and review of the literature.
abstract: Peripheral T-cell lymphoma primary to the central nervous system is a rare occurrence. The authors report a case of an 89-year-old woman who presented with a 3-month history of worsening confusion and recent onset of headache, nausea and vomiting, and upper limb tremors. Computed tomography and magnetic resonance imaging examinations demonstrated a 4.5-cm solitary brain mass in the right basal ganglia with compression along the ventricular system. No other lesion was found in the patient. Histologic and immunohistochemical studies of a stereotactic biopsy of the mass showed a T-cell lymphoproliferative lesion positive for CD3, CD8, CD57, and T-cell intracellular antigen 1 and negative for CD4, CD56, CD30, anaplastic lymphoma kinase, and CD20. A monoclonal T-cell receptor-gamma gene rearrangement was detected by polymerase chain reaction analysis of genomic DNA isolated from paraffin-embedded tumor tissue sections. These findings were consistent with peripheral T-cell lymphoma of cytotoxic/suppressor phenotype, resembling the phenotype of T-cell large granular cell leukemia. To the authors' best knowledge, this represents the first reported case of primary brain T-cell lymphoma with a cytotoxic/suppressor immunophenotype. A brief review of the literature of primary brain T-cell lymphoma is also presented.
authors.affiliation: Department of Pathology, University of Manitoba, Winnipeg, Canada.
authors.forename: Dating
authors.initials: D
authors.lastname: Liu
authors.forename: Robert L
authors.initials: RL
authors.lastname: Schelper
authors.forename: David A
authors.initials: DA
authors.lastname: Carter
authors.forename: Bernard J
authors.initials: BJ
authors.lastname: Poiesz
authors.forename: Anthony E
authors.initials: AE
authors.lastname: Shrimpton
authors.forename: Bruce M
authors.initials: BM
authors.lastname: Frankel
authors.forename: Robert E
authors.initials: RE
authors.lastname: Hutchison
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D001921:Brain / Q000473:pathology; D001932:Brain Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D004273:DNA, Neoplasm / Q000032:analysis; D017809:Fatal Outcome; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte / Q000235:genetics; D019672:Genes, T-Cell Receptor / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D016411:Lymphoma, T-Cell, Peripheral / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D016133:Polymerase Chain Reaction; D016176:T-Lymphocyte Subsets / Q000737:chemistry / Q000473:pathology*; D013602:T-Lymphocytes, Cytotoxic / Q000737:chemistry / Q000473:pathology*; D050378:T-Lymphocytes, Regulatory / Q000737:chemistry / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D015703:Antigens, CD; D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm