Authors: | von Roemeling, C.; Ferreri, A. J. M.; Soussain, C.; Tun, H. W.; Grommes, C. |
Review Title: | Targets and treatments in primary CNS lymphoma |
Abstract: | Primary central nervous system lymphoma (PCNSL) is a rare and highly aggressive lymphoma entirely localized in the central nervous system or vitreoretinal space. PCNSL generally initially responds to methotrexate-containing chemotherapy regimens, but progressive or relapsing disease is common, and the prognosis is poor for relapsed or refractory (R/R) patients. PCNSL is often characterized by activation of nuclear factor kappa B (NF-κB) due to mutations in the B-cell receptor (BCR) or toll-like receptor (TLR) pathways, as well as immune evasion. Targeted treatments that inhibit key PCNSL mechanisms and pathways are being evaluated; inhibition of Bruton’s tyrosine kinase (BTK) downstream of BCR activation has demonstrated promising results in treating R/R disease. This review will summarize the evidence and potential for targeted therapeutic agents to improve treatment outcomes in PCNSL. This includes immunotherapeutic and immunomodulatory approaches and inhibitors of the key pathways driving PCNSL, such as aberrant BCR and TLR signaling. © 2024 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group. |
Keywords: | signal transduction; treatment outcome; genetics; review; cancer recurrence; primary central nervous system lymphoma; methotrexate; genetic analysis; cancer immunotherapy; enzyme inhibition; immunoglobulin enhancer binding protein; drug effect; pathology; central nervous system tumor; central nervous system neoplasms; diagnosis; blood brain barrier; lymphoma; immunomodulation; disease management; drug therapy; mtor signaling; therapy; etiology; molecularly targeted therapy; immune evasion; molecular targeted therapy; b lymphocyte receptor; procedures; pi3k/akt signaling; pcnsl; refractory cancer; tlr; bruton tyrosine kinase; interleukin 1 receptor associated kinase 4; humans; human; btk; tlr signaling; bcr; irak4; bcr signaling; unmet medical need |
Journal Title: | Leukemia and Lymphoma |
Volume: | 65 |
Issue: | 8 |
ISSN: | 1042-8194 |
Publisher: | Taylor & Francis Group |
Date Published: | 2024-01-01 |
Start Page: | 1055 |
End Page: | 1067 |
Language: | English |
DOI: | 10.1080/10428194.2024.2342560 |
PUBMED: | 38659230 |
PROVIDER: | scopus |
DOI/URL: | |
Notes: | Review -- MSK corresponding author is Christian Grommes -- Source: Scopus |