Objective To investigate the clinical manifestations of primary central nervous system lymphoma(PCNSL) and explore the effective methods of diagnosis and treatment for immunocompetent PCNSL patients.Methods The clinical, laboratory,imaging and pathological data from 17 cases with PCNSL in our hospital from March 2006 to December 2008 were analyzed.Four patients received stereotactic brain biopsy while the other were given surgical treatment.Fifteen patients were received both radiotherapy and chemotherapy after stereotactic brain biopsy or resection.Results Sixteen of 17 cases were followed up for 9 -46 months.The mean survival time was 19 months and 2-year survival rate was 69.2％.Conclusion PCNSL has no specific clinical manifestations,the pathological examination might be the only reliable method to confirm PCNSL.Recurrence may occur after surgical alone, the combination of high-dose MTX chemotherapy and radiotherapy was an effective and safe therapeutic option, which might improve tumor respense rates and survival rates.%目的 探讨非免疫缺陷原发性中枢神经系统淋巴瘤(PCNSL)的诊治特点.方法 回顾分析2006年3月至2009年4月经病理组织学证实且免疫功能正常的17例PCNSL患者的临床资料.13例手术完全或部分切除,4例行立体定向活检术;术后15例给予化疗和放疗,2例未行放化疗.化疗采用大剂量甲氨喋呤(HD-MTX)2.0 g/m2静滴,每周1次,连用3周;第4周开始放疗,6例脑脊液异常者行全中枢神经系统放疗,9例脑脊液正常者仅行全脑放疗.结果 6例HD-MTX 加全中枢神经系统放疗中5例(83.3%)出现3级白细胞减少,未出现其他3级以上毒副反应.随访9～46个月,2例未行放化疗者术后1个月复发;15例行放化疗中1例术后3个月复发,1例失访,13例未复发存活,2年生存率69.2%.结论 PCNSL临床表现缺乏特异性,确诊依赖于病理诊断;手术仅有诊断意义,采用HD-MTX化疗联合放疗的综合治疗模式可提高疗效及生存率,且相对安全.
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