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

Abstract 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 t...

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Bibliographic Details
Published in:Neuro-oncology (Charlottesville, Va.) Va.), 2019-02, Vol.21 (3), p.296-305
Main Authors: Grommes, Christian, Rubenstein, James L, DeAngelis, Lisa M, Ferreri, Andres J M, Batchelor, Tracy T
Format: Article
Language:English
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Summary:Abstract 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.
ISSN:1522-8517
1523-5866
DOI:10.1093/neuonc/noy192