title: Primary central nervous system Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly: a clinicopathologic study of five cases.
abstract: We report five cases of primary central nervous system (CNS) Epstein-Barr virus (EBV)-positive lymphoma of the elderly. This represented an incidence of 4 % of primary CNS diffuse large B-cell lymphoma (DLBCL) after EBV screening in 134 cases. All five patients were 65 years or older with no previous history of congenital or iatrogenic immune deficiencies. The histologic morphology of all the cases was DLBCL, with variable amounts of necrosis. The cell of origin (COO) as determined by the Hans algorithm disclosed germinal center type in 2 cases and non-germinal center type in 3 cases. MYC translocation was not detected, and MYC overexpression was detected in only one case. Three patients died shortly after diagnosis, and the remaining 2 patients were in complete remission for 2 and 10 years, respectively. We conclude that EBV+ DLBCL among the elderly is uncommon in primary CNS lymphoma in the Eastern United States. The patients usually present with a single mass lesion with headache and sensorimotor symptoms. The histologic morphology is DLBCL, but clonal T-cell gene rearrangement may be detected. The outcome varies from case to case and appears to be unrelated to the COO or MYC status.
authors.affiliation: Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, USA.
authors.forename: Siraj El
authors.initials: SE
authors.lastname: Jamal
authors.forename: Shiyong
authors.initials: S
authors.lastname: Li
authors.forename: Renu
authors.initials: R
authors.lastname: Bajaj
authors.forename: Zixuan
authors.initials: Z
authors.lastname: Wang
authors.forename: Lawrence
authors.initials: L
authors.lastname: Kenyon
authors.forename: Jon
authors.initials: J
authors.lastname: Glass
authors.forename: Changlee S
authors.initials: CS
authors.lastname: Pang
authors.forename: Sharathkumar
authors.initials: S
authors.lastname: Bhagavathi
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Peiper
authors.forename: Jerald Z
authors.initials: JZ
authors.lastname: Gong
mesh_terms: D000368:Aged; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology* / Q000821:virology*; D020031:Epstein-Barr Virus Infections; D017809:Fatal Outcome; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000235:genetics / Q000473:pathology* / Q000821:virology*; D008297:Male; D011379:Prognosis; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics
chemical_list: C489427:MYC protein, human; D016271:Proto-Oncogene Proteins c-myc