Central Nervous System Lymphoma

Hematol Oncol Clin North Am. 2019 Aug;33(4):597-611. doi: 10.1016/j.hoc.2019.03.008. Epub 2019 May 10.

Abstract

Primary central nervous system lymphoma (PCNSL) is an uncommon subtype of extranodal non-Hodgkin lymphoma (NHL) with less than 1500 cases annually. Incidence of PCNSL has remained stable in the post-highly active antiretroviral therapy era, owing to increasing incidence in elderly, immunocompetent patients. Most PCNSL is diffuse large B cell in origin, with less frequent involvement of T-cell and Burkitt lymphoma. Secondary central nervous system lymphoma is more likely to occur in the relapsed setting of a systemic NHL. Methotrexate forms the backbone of management for prophylaxis and treatment of disease. Treatments are currently under investigation for both disease entities.

Keywords: Central nervous system; Diffuse large B-cell lymphoma; Extranodal; Methotrexate; Non-Hodgkin lymphoma.

Publication types

  • Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Central Nervous System Neoplasms / epidemiology
  • Central Nervous System Neoplasms / pathology*
  • Central Nervous System Neoplasms / therapy*
  • Combined Modality Therapy
  • Humans
  • Immunocompromised Host*
  • Immunotherapy
  • Incidence
  • Lymphoma, Large B-Cell, Diffuse / epidemiology
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Lymphoma, Large B-Cell, Diffuse / therapy*
  • Lymphoma, T-Cell / epidemiology
  • Lymphoma, T-Cell / pathology*
  • Lymphoma, T-Cell / therapy*
  • Radiotherapy