Abstract
The optimal treatment for newly diagnosed and refractory or relapsed primary central nervous system lymphoma (PCNSL) is not fully defined. We review the epidemiology, clinical presentation, and current management strategies for newly diagnosed PCNSL as well as emerging treatments for refractory and relapsed disease. In recent decades, the incidence of PCNSL has increased in the elderly population. With advancements in chemotherapy for PCNSL, survival has improved. However, outcomes remain inferior when compared with other forms of extranodal lymphoma. Additionally, treatments can be associated with clinically significant neurotoxicities. Despite advances in the treatment of PCNSL, current treatment regimens remain suboptimal in terms of response rates and neurotoxicity. Well-tolerated agents, especially for the elderly, are still needed.
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