Primary Central Nervous System Lymphoma and Hematopoietic Neoplasms Affecting the Nervous System

Abstract Primary central nervous system lymphoma (PCNSL) is a rare and aggressive variant of extranodal non-Hodgkin lymphoma involving compartments of the central nervous system, including the brain, leptomeninges, eyes, or spinal cord without evidence of systemic disease. PCNSL poses unique challenges to the neuro-oncologist in terms of clinical presentation, extent of disease evaluation, and treatment strategies. This chapter details the epidemiology, pathophysiology, differential diagnosis, investigations, management, and prognosis of PCNSL, with a focus on the most salient points for the neuro-oncologist. CNS involvement from other hematological malignancies will also be briefly discussed.