Primary central nervous system lymphoma: overview of current treatment strategies.

Primary central nervous system lymphoma (PCNSL) is an uncommon and aggressive variant of non-Hodgkin lymphoma that involves the brain, eyes, leptomeninges, or spinal cord. Therapeutic progress has been modest, and our understanding of the molecular mechanisms that drive this disease is limited. Clinicians treating PCNSL face a challenge to balance the need to administer aggressive regimens to achieve a cure with the risks of delayed neurotoxicity after treatment. The standard treatment is a methotrexate-containing chemotherapy regimen. The timing and dose of whole-brain radiation therapy is controversial, given the significant risks of late neurotoxic effects, particularly in elderly patients.