Molecular profiling of primary central nervous system lymphomas - Predictive and prognostic value? Review


Authors: Ho, K. G.; Grommes, C.
Review Title: Molecular profiling of primary central nervous system lymphomas - Predictive and prognostic value?
Abstract: Purpose of review Primary central nervous system lymphoma (PCNSL) is a rare but aggressive variant of non-Hodgkin lymphoma. The diagnostic gold standard remains the pathologic review of tumor tissue mainly collected though biopsies. The majority of PCNSL are diffuse large B cell lymphoma (DLBCL). Biopsies are invasive procedures, and there have been efforts to develop minimally invasive diagnostic testing using serum and cerebral spinal fluid. This article reviews multiple markers that could potentially serve as future diagnostic tools and predictors of treatment response. Recent findings Many studies have attempted to classify DLBCL into different subtypes for prognostic purposes using methods such as immunohistochemistry. PCNSL often falls under the activated B-cell-like subgroup, and further genomic sequencing has identified alterations in genes within the B-cell receptor signaling axis at increased frequencies. Two such genes, MYD88 and CD79B, implicate the involvement of the NF-kB (nuclear factor kappa-light-chain enhancer of activated B cells) pathway, and targeted agents to this pathway are currently being used in the treatment of relapsed/refractory PCNSL. Summary Although recent genomic profiling of PCNSL has increased the understanding of drivers in this disease and has also led to the introduction of targeted inhibitors, these markers have not yet been used for diagnostic and/or prognostic purposes. Further studies will need to evaluate if they hold great diagnostic potential. © 2019 Wolters Kluwer Health, Inc. All rights reserved.
Keywords: immunohistochemistry; signal transduction; treatment response; review; pathophysiology; primary central nervous system lymphoma; cancer diagnosis; biological marker; immunoglobulin enhancer binding protein; tumor marker; myeloid differentiation factor 88; predictive value; tumor microenvironment; molecular pathology; b lymphocyte receptor; survival prediction; diffuse large b cell lymphoma; b-cell receptor; cancer prognosis; human; cd79b antigen; molecular fingerprinting; activated b-cell subtype; cluster of differentiation 79b; myeloid differentiation primary response 88
Journal Title: Current Opinion in Neurology
Volume: 32
Issue: 6
ISSN: 1350-7540
Publisher: Lippincott Williams & Wilkins  
Date Published: 2019-12-01
Start Page: 886
End Page: 894
Language: English
DOI: 10.1097/wco.0000000000000759
PUBMED: 31592789
PROVIDER: scopus
PMCID: PMC7141780
DOI/URL:
Notes: Source: Scopus
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  1. Christian Grommes
    150 Grommes
  2. Ka-Wai Grace Ho
    6 Ho