CNS lymphoma

Abstract

Central nervous system lymphoma (CNSL) may occur exclusively in the CNS (primary CNS lymphoma, PCNSL) or as a secondary involvement of a systemic lymphoma (secondary CNS lymphoma, SCNSL). The incidence of these lymphomas is still low, but an increasing incidence, particularly in older patients, has been reported in last decades. The pathophysiologic basis for development of lymphoma in the CNS, lacking a lymphatic system, is still a subject of debate. The course of CNSL is usually very aggressive and the prognosis worse compared to non-Hodgkin’s lymphoma involving other extranodal sites and organs. Therapeutic management has to account for the particular pharmacokinetics of the CNS. High-dose methotrexate (HDMTX) is the most active agent and should be considered backbone of therapy; however, the optimal management of CNSL has not been fully established. For long-term survivors, late neurotoxicity is an important issue, particularly when whole-brain radiotherapy (WBRT) was performed. Whenever possible, patients with CNS involvement of lymphoma should be included in clinical trials.