PURPOSE OF REVIEW: Primary central nervous system lymphoma (PCNSL) is a rare tumor, and its optimal management is a matter of controversy. Recent developments of collaborative groups devoted to PCNSL have allowed to conduct randomized trials addressing important issues. In addition, genome wide analysis provided new insights in the PCNSL tumorigenesis.
RECENT FINDINGS: The purpose of the present review is to focus on the most recent findings in the management and the biological advances of PCNSL.
SUMMARY: Cumulating data challenge the role of consolidation radiotherapy after high-dose methotrexate-based polychemotherapy, especially in the elderly, in order to reduce delayed neurotoxicity, support intensive chemotherapy with autologous stem cell transplantation as a therapeutic option in PCNSL, confirm the interest for adding rituximab in the initial treatment, and suggest the importance of B-cell receptor/Toll-like receptor/nuclear factor kappa B signaling pathway activation in PCNSL as a promising target for novel therapies.