Objective: To investigate the characteristics, treatment and outcome of patients with primary central nervous system lymphoma （PCNSL）.
Methods: A total of 37 patients with PCNSL treated in Peking Union Medical College Hospital from June 1999 to June 2012 were enrolled into this retrospective study. The clinical characteristics, results of treatment and prognostic factors were analyzed.
Results: The median age of 37 patients with PCNSL at diagnosis was 57 years（range 17 to 78 years） with a male to female ratio of 2.7:1. The symptoms or signs of elevated intracranial pressure and cognitive dysfunction were the most common initial manifestations. The median time period between onset of symptoms and diagnosis was 1.5 months. The majority of lesions were located in the cerebral hemisphere. At a median follow-up of 50 months, the median overall survival for all treated patients was 36.0 months （95% CI 21.7-50.3 months）, with a progression-free survival of 18.0 months（95% CI 9.1-26.9 months）. The 3-year cumulative survival rate was 46.9%. Compared to chemotherapy alone, combined-modality regimens which did not improve outcome were associated with a greater risk of neurotoxicity.
Conclusion: The prognosis of PCNSL was still poor, and the optimal treatment strategy for these patients should be explored in the future clinical trials.
目的: 探讨原发中枢神经系统淋巴瘤(PCNSL)的临床特征、治疗方案以及预后相关因素。
方法: 回顾性总结北京协和医院自1999年6月至2012年6月收治的37例PCNSL患者临床资料，对患者临床特征、治疗以及预后相关因素进行分析。
结果: 全部37例PCNSL患者中位发病年龄57(17~78)岁，男女比为2.7:1。以颅内高压和智能障碍为最常见临床表现，起病至诊断中位时间1.5(1~24)个月。肿瘤累及部位以大脑半球为主，且多灶病变更为常见。36例经治患者无进展生存(PFS)时间为18.0 (95%CI 9.1~26.9)个月，总生存(OS)时间为36.0 (95%CI 21.7~50.3)个月，3年累计OS率为46.9%。放化疗联合治疗与单纯化疗组PFS时间[16.0 (95%CI 8.9~23.1)个月对26.0(95% CI 2.9~49.1)个月]及OS时间[36.0 (95%CI 2.3~69.7)个月对29.0 (95%CI 23.4~34.6)个月]比较，差异均无统计学意义(P=0.401、0.866)。
结论: PCNSL预后不佳，化放疗联合并不能显著改善患者生存，其最佳治疗方案应需进一步探索。