Abstract

Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma that arises within the brain, spinal cord, leptomeninges, or eyes without evidence of systemic involvement at the time of diagnosis. Its rarity and unique presentation make PCNSL a challenging diagnostic and therapeutic dilemma in the field of neurology. This article aims to review the current understanding of PCNSL, including its epidemiology, pathogenesis, clinical presentation, diagnostic modalities, treatment options, and prognosis. Additionally, we discuss emerging research directions and potential novel therapeutic approaches to improve outcomes for patients with this neurological issue.