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Primary CNS lymphoma

Primary CNS lymphoma (PCNSL) is an infiltrative, primarily large B-cell lymphoma confined to the CNS or eyes at the time of diagnosis, with an incidence of 5 per million. Because of its rarity, phase III trials to determine the best treatment regimen have been difficult to perform and no single standard of care exists. Current guidelines suggest initial high-dose methotrexate-based (HDMTX) chemotherapy is the most active regimen. In general, 3 first-line chemotherapy treatment approaches are currently used by most PCNSL investigators: (1) HDMTX-based chemotherapy with or without subsequent whole-brain radiotherapy (WBRT); (2) induction HDMTX-based chemotherapy followed by high-dose chemotherapy consolidation with or without autologous stem cell transplantation1; and (3) standard dose HDMTX-based chemotherapy with mechanisms to increase delivery to the CNS such as blood–brain barrier disruption (BBBD).2