Primary central nervous system lymphoma (PCNSL) constitutes a rare group of extranodal non-Hodgkin's lymphoma (NHL) primarily of B cell origin. It occurs in both immuno-competent and immune-compromised patients. High dose methotrexate (HD-MTX) based chemotherapy is the standard therapy. Chemotherapy with whole brain radiation therapy (WBRT) improves response rates and survival compared with WBRT alone. However, due to the increased risk for neurotoxicity with WBRT, recent studies have focused on using chemotherapy alone. Methotrexate based multi-agent chemotherapy without WBRT is associated with similar treatment rates and survival compared with regimens that include WBRT although controlled trials have not been performed. Because of the low incidence of this disease, it is difficult to conduct randomized controlled trials. In this article we have discussed about the past, present and emerging treatment options in patients with PCNSL.
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