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Background: Primary central nervous system lymphoma (PCNSL) is a rare disease affecting the brain, leptomeninges, spinal cord, cerebrospinal fluid, or vitreoretinal compartment, without evidence of systemic disease. Prognosis is still poor after intensive methotrexate-based chemotherapy., Methods: Clinical data of 91 patients treated in a tertiary referral center during a 13-year period were retrospectively reviewed., Results: The estimated median progression-free survival and overall survival (OS) for the entire cohort were 39.1 months (95% confidence interval [CI], 14.1-64.0 months) and 54.5 months (95% CI, 28.9-80.1 months), respectively. Estimated 5-year progression-free survival and OS were 37.0% ± 6.5% and 47.5% ± 7.5%. Survival was associated with cycles of methotrexate only in multivariate analysis. Seventy-four patients received methotrexate-based chemotherapy after diagnosis. Thirty-nine patients experienced disease progression. Patients with relapsed/refractory disease had a poor survival, with median second OS (calculated from the date of first disease progression to the time of death from any cause) being 7.2 months (95% CI, 2.5-12.00 months). Three patients responded to ibrutinib after disease progression and incurred no fungal infection., Conclusions: The outcomes of patients with PCNSL treated in our cohort are still poor. Relapse or refractory PCNSL and those not tolerating aggressive chemotherapy urgently require new approaches to improve their still dismal prognosis. Novel agents such as ibrutinib have shown promising clinical activity. Future studies should focus on the predictive biomarkers for the treatment of PCNSL with novel agents to provide precision medicine for PCNSL., (Copyright © 2018 Elsevier Inc. All rights reserved.)