Treatment of primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin's lymphoma that is typically confined to brain, eyes, and cerebrospinal fluid without evidence of systemic spread. The prognosis of patients with PCNSL has improved during the past decade with the introduction of high-dose methotrexate with or without whole brain radiotherapy. However, despite recent progress, results following treatment are durable in few patients, and therapy can be associated with late neurotoxicity. PCNSL is an uncommon tumor, and no phase III trial has been completed so far, leaving many questions about its optimum first-line and salvage treatments unanswered. This review summarizes the literature regarding the treatment of PCNSL in immunocompetent patients.