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Primary central nervous system lymphoma

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Management of Rare Adult Tumours
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Abstract

Primary Central Nervous System Lymphoma (PCNSL) is a relatively rare localisation of non-Hodgkin’s lymphoma (NHL) that is confined to the brain, the leptomeninges, the eyes, the cerebrospinal fluid (CSF) or the nerve bundles. PCNSL is, as a primary brain tumour, by definition not diagnosed in the setting of widespread localisations outside the central nervous system (CNS) [37]. Therefore, it should be differentiated from the secondary involvement of the CNS which occurs in 5% to 29% of patients with systemic NHL, usually associated with progressive widespread systemic disease and involving often only the meningeal surface. The prognosis of patients with PCNSL with conventional treatment similar to that for other NHL is very poor, with a 5-year survival rate at usually less than 10%. Some rare but distinctive subtypes of PCNSL should be recognised.

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© 2009 Springer-Verlag France, Paris

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Poortmans, P.M.P. (2009). Primary central nervous system lymphoma. In: Belkacémi, Y., Mirimanoff, RO., Ozsahin, M. (eds) Management of Rare Adult Tumours. Springer, Paris. https://doi.org/10.1007/978-2-287-92246-6_3

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  • DOI: https://doi.org/10.1007/978-2-287-92246-6_3

  • Publisher Name: Springer, Paris

  • Print ISBN: 978-2-287-92245-9

  • Online ISBN: 978-2-287-92246-6

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