Comprehensive approach to diagnosis and treatment of newly diagnosed primary CNS lymphoma

Neuro Oncol. 2019 Feb 19;21(3):296-305. doi: 10.1093/neuonc/noy192.


Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that affects the brain parenchyma, spinal cord, eyes, and cerebrospinal fluid without evidence of systemic, non-CNS involvement. PCNSL is uncommon and only a few randomized trials have been completed in the first-line setting. Over the past decades, the prognosis of PCNSL has improved, mainly due to the introduction and widespread use of high-dose methotrexate, which is now the backbone of all first-line treatment polychemotherapy regimens. Despite this progress, durable remission is recorded in only 50% of patients, and therapy can be associated with significant late neurotoxicity. Here, we overview the epidemiology, clinical presentation, staging evaluation, prognosis, and current up-to-date treatment of immunocompetent PCNSL patients.

Keywords: PCNSL; diagnosis; methotrexate; staging; therapy.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Age Factors
  • Antineoplastic Agents, Alkylating / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Burkitt Lymphoma / diagnostic imaging
  • Burkitt Lymphoma / epidemiology
  • Burkitt Lymphoma / pathology
  • Burkitt Lymphoma / therapy
  • Central Nervous System Neoplasms / diagnostic imaging
  • Central Nervous System Neoplasms / epidemiology
  • Central Nervous System Neoplasms / pathology
  • Central Nervous System Neoplasms / therapy*
  • Chemoradiotherapy
  • Cranial Irradiation / adverse effects
  • Cranial Irradiation / methods*
  • Cytoreduction Surgical Procedures
  • Humans
  • Immunocompromised Host
  • Lymphoma, Large B-Cell, Diffuse / diagnostic imaging
  • Lymphoma, Large B-Cell, Diffuse / epidemiology
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Lymphoma, Large B-Cell, Diffuse / therapy*
  • Lymphoma, T-Cell / diagnostic imaging
  • Lymphoma, T-Cell / epidemiology
  • Lymphoma, T-Cell / pathology
  • Lymphoma, T-Cell / therapy
  • Methotrexate / administration & dosage
  • Neoplasm Staging
  • Neurosurgical Procedures
  • Prognosis
  • Rituximab / administration & dosage
  • Survival Rate


  • Antineoplastic Agents, Alkylating
  • Rituximab
  • Methotrexate