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Review
. 2024 May;271(5):2906-2913.
doi: 10.1007/s00415-023-12143-w. Epub 2023 Dec 19.

Primary central nervous system lymphoma

Myrthe E de Koning  1 , Jurrit J Hof  2 , Casper Jansen  3 , Jeanette K Doorduijn  4 , Jacoline E C Bromberg  5 , Matthijs van der Meulen  6
Affiliations
Review

Primary central nervous system lymphoma

Myrthe E de Koning et al. J Neurol. 2024 May.

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.

Keywords: CNS lymphoma; High-dose methotrexate; Neuro-oncology; Whole brain radiotherapy.

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