Primary central nervous system lymphoma

Myrthe E. de Koning; Jurrit J. Hof; Casper Jansen; Jeanette K. Doorduijn; Jacoline E.C. Bromberg; Matthijs van der Meulen

doi:10.1007/s00415-023-12143-w

Primary central nervous system lymphoma

Myrthe E. de Koning^*, Jurrit J. Hof, Casper Jansen, Jeanette K. Doorduijn, Jacoline E.C. Bromberg, Matthijs van der Meulen

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

5 Citations (Scopus)

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.

Original language	English
Pages (from-to)	2906-2913
Number of pages	8
Journal	Journal of Neurology
Volume	271
Issue number	5
DOIs	https://doi.org/10.1007/s00415-023-12143-w
Publication status	Published - 19 Dec 2023

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Publisher Copyright:
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany 2023.

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title = "Primary central nervous system lymphoma",

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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AU - de Koning, Myrthe E.

AU - Hof, Jurrit J.

AU - Jansen, Casper

AU - Doorduijn, Jeanette K.

AU - Bromberg, Jacoline E.C.

AU - van der Meulen, Matthijs

PY - 2023/12/19

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AB - 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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Primary central nervous system lymphoma

Abstract

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