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.
Keywords:
PCNSL; diagnosis; methotrexate; staging; therapy.
© The Author(s) 2018. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Age Factors
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Antineoplastic Agents, Alkylating / administration & dosage
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
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Burkitt Lymphoma / diagnostic imaging
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Burkitt Lymphoma / epidemiology
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Burkitt Lymphoma / pathology
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Burkitt Lymphoma / therapy
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Central Nervous System Neoplasms / diagnostic imaging
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Central Nervous System Neoplasms / epidemiology
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Central Nervous System Neoplasms / pathology
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Central Nervous System Neoplasms / therapy*
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Chemoradiotherapy
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Cranial Irradiation / adverse effects
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Cranial Irradiation / methods*
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Cytoreduction Surgical Procedures
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Humans
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Immunocompromised Host
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Lymphoma, Large B-Cell, Diffuse / diagnostic imaging
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Lymphoma, Large B-Cell, Diffuse / epidemiology
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Lymphoma, Large B-Cell, Diffuse / pathology
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Lymphoma, Large B-Cell, Diffuse / therapy*
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Lymphoma, T-Cell / diagnostic imaging
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Lymphoma, T-Cell / epidemiology
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Lymphoma, T-Cell / pathology
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Lymphoma, T-Cell / therapy
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Methotrexate / administration & dosage
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Neoplasm Staging
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Neurosurgical Procedures
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Prognosis
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Rituximab / administration & dosage
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Survival Rate
Substances
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Antineoplastic Agents, Alkylating
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Rituximab
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Methotrexate