Clinicopathological Characterization of Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System

Primary central nervous system diffuse large B-cell lymphoma (CNS DLBCL) is a rare entity, accounting for 3-4% of intracranial neoplasms. This study aimed to investigate the clinicopathological characteristics of primary CNS DLBCL patients and their prognostic implication.We collected 74 cases of clinically and pathologically confirmed primary CNS DLBCL from two institutions. Disease-free survival (DFS) and overall survival (OS) were analyzed based on various clinicopathological parameters.Most cases (83.8%) were classified as activated B-cell immunophenotype by Hans algorithm and cell-of-origin classification did not influence the clinical outcome. On univariate analysis, age (>60 years) and ECOG performance status (≥2) were significantly associated with shorter DFS and OS, and MYC/BCL2 co-expression significantly impacted poor DFS. An anaplastic variant was diagnosed in only 2 cases, but it raised possible association with poor outcome. On multivariate analysis, ECOG performance status and age was associated with poor prognosis.In primary CNS DLBCL, age and performance status revealed the most significant association with prognosis. Cell-of-origin classification was not a significant prognostic factor in contrast to systemic DLBCL.