Primary central nervous system lymphoma (PCNSL) is a non-Hodgkin's lymphoma (NHL) confined to the craniospinal axis without evidence of systemic spread. It should be distinguished from nodal or extranodal NHL that has disseminated to the CNS, which is a different clinical entity. In this chapter, we will discuss the epidemiology, pathology, clinical presentation, diagnosis, and treatment of PCNSL in immunocompe-tent patients. PCNSL that develops in the context of acquired immunosuppression will be discussed in Chapters 66 and 67.

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