Primary central nervous system lymphoma in Miyazaki, southwestern Japan, a human T-lymphotropic virus Type-1 (HTLV-1)-endemic area: clinicopathological review of 31 cases

J Clin Exp Hematop. 2014;54(3):179-85. doi: 10.3960/jslrt.54.179.


Primary central nervous system lymphoma (PCNSL) is a rare and aggressive brain tumor. The aim of this study was to clarify the prevalence of T-cell-type PCNSL (T-PCNSL) in a human T-lymphotropic virus type-1 (HTLV-1)-endemic area of Southwestern Japan. We retrospectively investigated 31 PCNSL cases diagnosed between 1996 and 2013 at the University of Miyazaki Hospital. These cases accounted for 4.4% of all nodal or extranodal malignant lymphomas. Histologically, most of these cases were diagnosed as diffuse large B-cell lymphoma, while only two cases were considered to be low-grade and high-grade B-cell lymphoma (not otherwise specified). No T-PCNSL was found in this series. In addition, Epstein-Barr virus-encoded RNAs were not detected by in situ hybridization in any of the cases. Overall, no T-PCNSL cases were found in 18 years in a region with a high frequency of HTLV-1 seropositivity, namely, Southwestern Japan. This suggests that PCNSL and lymphomas of other anatomical sites are biologically distinct.

MeSH terms

  • Aged
  • Brain Neoplasms / diagnosis
  • Brain Neoplasms / epidemiology
  • Brain Neoplasms / pathology
  • Brain Neoplasms / virology*
  • Disease Progression
  • Endemic Diseases
  • Female
  • HTLV-I Infections / epidemiology*
  • HTLV-I Infections / virology
  • Human T-lymphotropic virus 1 / isolation & purification*
  • Humans
  • Japan / epidemiology
  • Lymphoma, B-Cell / diagnosis
  • Lymphoma, B-Cell / epidemiology
  • Lymphoma, B-Cell / pathology
  • Lymphoma, B-Cell / virology*
  • Lymphoma, Large B-Cell, Diffuse / diagnosis
  • Lymphoma, Large B-Cell, Diffuse / epidemiology
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Lymphoma, Large B-Cell, Diffuse / virology*
  • Male
  • Middle Aged