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Primary central nervous system lymphoma

  • Neurological Update
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Abstract

Primary central nervous system lymphoma (PCNSL) is a rare type of non-Hodgkin lymphoma (NHL) manifesting in the brain, spinal cord, cerebrospinal fluid and/or eyes, in the absence of systemic manifestations. With an increasing incidence and a 30% 5-year overall survival if promptly treated, timely diagnosis and subsequent treatment is paramount. The typical MRI appearance for PCNSL is a solitary or multiple T2-hypointense, homogeneous gadolinium-enhancing lesion with restricted diffusion. Dexamethasone treatment might compromise and delay the diagnosis. Hallmark of treatment is induction with intravenous high-dose methotrexate consisting polychemotherapy followed by consolidation treatment. Consolidation treatment consists of either whole brain radiotherapy (WBRT) or autologous stem cell transplantation (ASCT). Given the (cognitive) side effects of WBRT, ASCT is increasingly being used as the first choice of treatment.

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Author information

Authors and Affiliations

  1. Department of Neurology, Medisch Spectrum Twente, Enschede, The Netherlands

    Myrthe E. de Koning & Matthijs van der Meulen

  2. Department of Radiology, Medisch Spectrum Twente, Enschede, The Netherlands

    Jurrit J. Hof

  3. Laboratory of Pathology Eastern Netherlands, Hengelo, The Netherlands

    Casper Jansen

  4. Department of Hematology, Erasmus MC Cancer Institute, University Medical Center Rotterdam, Rotterdam, The Netherlands

    Jeanette K. Doorduijn

  5. Department of Neurology, Erasmus MC Cancer Institute, University Medical Center Rotterdam, Rotterdam, The Netherlands

    Jacoline E. C. Bromberg

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  1. Myrthe E. de Koning
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Correspondence to Myrthe E. de Koning.

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de Koning, M.E., Hof, J.J., Jansen, C. et al. Primary central nervous system lymphoma. J Neurol 271, 2906–2913 (2024). https://doi.org/10.1007/s00415-023-12143-w

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