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Rare central nervous system lymphomas
Author(s) -
Kaji Furqaan Ahmed,
MartinezCalle Nicolás,
Sovani Vishakha,
Fox Christopher Paul
Publication year - 2022
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.18128
Subject(s) - medicine , lymphoma , pathology , central nervous system , context (archaeology) , transplantation , methotrexate , meninges , immunology , biology , paleontology
Central nervous system (CNS) lymphomas are rare malignancies characterised by lymphoid infiltration into the brain, spinal cord, cranial nerves, meninges and/or eyes in the presence or absence of previous or concurrent systemic disease. Most CNS lymphomas are of the diffuse large B‐cell lymphoma (DLBCL) subtype for which treatment strategies, particularly the use of high‐dose methotrexate‐based protocols and consolidation with autologous stem cell transplantation, are well established. Other histopathological subtypes of CNS lymphoma are comparatively less common with published data on these rare lymphomas dominated by smaller case series and retrospective reports. Consequently, there exists little clinical consensus on the optimal methods to diagnose and manage these clinically and biologically heterogeneous CNS lymphomas. In this review article, we focus on rarer CNS lymphomas, summarising the available clinical data on incidence, context, diagnostic features, reported management strategies, and clinical outcomes.