Abstract
Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal lymphoma primarily involving the brain, spinal cord, or leptomeninges. PCNSL is associated with a relatively poor prognosis compared to other extranodal diffuse large B-cell lymphomas. However, methotrexate-based induction chemotherapy followed by consolidative chemotherapy or high-dose therapy and autologous stem cell transplantation has improved the survival outcome, together with reduced neurotoxicity. Recent studies found that aberrant activation of the B-cell receptor-signaling pathway and activation of the NF-κB are frequent genetic alterations and could be good targets for the treatment of PCNSL. Herein, we have reviewed the current status and recent advances in the biology and management of PCNSL.