Review ArticlesCentral Nervous System Lymphoma Approach to Diagnosis and TreatmentCorreia, Carlos Eduardo MD∗; Schaff, Lauren R. MD∗,†; Grommes, Christian MD∗,†Author Information From the ∗Department of Neurology, Memorial Sloan Kettering Cancer Center †Weill Cornell Medical School, Department of Neurology, New York, NY. Reprints: Christian Grommes Memorial Sloan Kettering Cancer Center, 1275 York Avenue New York, NY 10065. E-mail: [email protected]. This article was supported, in part, by the MSK Cancer Center Support Grant (P30 CA008748), National Institutes of Health. Conflicts of Interest and Source of Funding: C.G. is consultant for BTG, KITE, and ONO. For the remaining authors, none were declared. The Cancer Journal: 5/6 2020 - Volume 26 - Issue 3 - p 241-252 doi: 10.1097/PPO.0000000000000449 Buy Metrics Abstract Central nervous system lymphoma (CNSL) is a rare form of extranodal non-Hodgkin lymphoma. Central nervous system lymphoma can be primary (isolated to the central nervous space) or secondary in the setting of systemic disease. Treatment of CNSL has improved since the introduction of high-dose methotrexate and aggressive consolidation regimens. However, results after treatment are durable in only half of patients, and long-term survivors may experience late neurotoxicity, impacting quality of life. Given the rarity of this disease, few randomized prospective trials exist. This leaves many questions unanswered regarding optimal first-line and salvage treatments. Recent advances in the knowledge of pathophysiology of CNSL will hopefully help the development of future treatments. This review gives an overview of the epidemiology, pathophysiology, clinical presentation, diagnosis, and treatment of immunocompetent patients with CNSL. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.