Clinicopathological Features and Outcomes in Primary Central Nervous System Lymphoma: A 10-year Experience

Context Primary central nervous system lymphoma (PCNSL) is a variant of extranodal lymphoma, accounting for 4% of primary central nervous system tumors. PCNSL was more common in immunocompetent individuals. International Extranodal Lymphoma Study Group (IELSG) scoring was used for prognostication. High-dose methotrexate regimens along with radiotherapy improved outcomes in PCNSL. Aims The aim of this study is to analyze the clinical and pathological features, progression-free survival (PFS), and overall survival (OS) in patients with PCNSL. Materials and Methods Data of patients with PCNSL between 2005 and 2016 were retrospectively analyzed. Outcome was analyzed in patients who received chemotherapy. GraphPad Prism software for Windows Version 6 was used to plot the Kaplan-Meier curves for PFS and OS. Log-rank test was used to calculate P values. P < 0.05 was considered as statistically significant. Results A total of 42 patients were available for analysis. Of these, 34 patients who received chemotherapy were evaluable for outcome parameters. The median age at presentation was 46 years (range, 10-75) with male-to-female ratio of 2.2:1. Only 2 (4.7%) patients were HIV positive. Diffuse large B-cell lymphoma (DLBCL) was the most common histology seen in 41 (97.6%) patients. Using IELSG risk scoring, scores of 8 (19%), 19 (45.2%), and 15 (35.8%) were stratified into low, intermediate, and high risk. The median PFS and OS were 11 months (range, 2-72) and 15.9 months (2.4-80.4), respectively. The median OS was 36.2 months (range, 8.8-72), 15.6 months (2-36), and 6.1 months (2.6-12.7) in low-, intermediate-, and high-risk groups, respectively, which was statistically significant (P = 0.0002). Conclusions Immunocompetent patients with PCNSL outnumber immunocompromised patients. DLBCL was the most common histology, and IELSG risk stratification significantly predicts the outcome in PCNSL.