Primary central nervous system (CNS) lymphoma is a challenging subtypes of aggressive non-Hodgkin lymphoma. Emerging clinical data suggest that optimized outcomes are achieved with dose-intensive CNS-penetrant chemotherapy and avoiding whole brain radiotherapy. Anti-CD20 antibody-based immunotherapy as a component of high-dose methotrexate-based induction programs may contribute to improved outcomes. An accumulation of insights into the molecular and cellular basis of disease pathogenesis is providing a foundation for the generation of molecular tools to facilitate diagnosis as well as a roadmap for integration of targeted therapy within the developing therapeutic armamentarium for this challenging brain tumor.
Key points
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Long-term survival and cure is feasible in primary central nervous system lymphoma (PCNSL) without whole brain radiotherapy (WBRT).
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WBRT consolidation is associated with severe neurotoxicity, particularly in patients older than 60.
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High-dose chemotherapy is currently under investigation as first-line consolidation.
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There is a need for novel therapies that target key survival pathways in PCNSL, including activation of nuclear factor-κB survival signaling.
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