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目的探讨免疫正常的原发性中枢神经系统淋巴瘤(PCNSL)的生物学行为和临床特征,以帮助临床合理的诊断、治疗及对预后的判断。方法近9年经病理证实的31例PCNSL,其中27例为原发性脑淋巴瘤(PBL),4例为原发性眼淋巴瘤,回顾性分析其临床特征、诊断方法及治疗效果。在活检或手术后,患者主要以放、化疗综合治疗为主。化疗以环磷酰胺+阿霉素+长春新碱+泼尼松或甲氨喋呤+阿糖胞苷方案为主;全脑放疗40～44Gy,局部肿瘤缩野放疗加量11～25.4Gy。对眼部淋巴瘤放疗25.2～30Gy。结果 PCNSL患病率明显上升(增加了2.6倍)。全组患者均为免疫正常者。发病中位年龄57岁(14～83岁),PBL≥60岁占48.2%。脑多发病变者22例(81.5%)。脑立体定向活检确诊20例(1次性成功率90%),手术确诊11例。B细胞淋巴瘤占93.6%,T细胞淋巴瘤占6.4%。有4例患者在外院曾误诊65d至7个月。全组PBL1、3、5年生存率分别为68.2%、52.3%和35.0%。全组中位生存时间37个月。结论 PCNSL常发生在中老年人,B细胞来源为主。PBL临床和影像学很难与其他中枢神经系统肿瘤相区别,易误诊。对PBL疑似患者,脑立体定向活检可提高诊断效率。化、放疗综合治疗可提高疗效。≥60岁者预后不良。
Objective To investigate the biological behavior and clinical features of normal spontaneous central nervous system lymphoma (PCNSL) in order to help clinical diagnosis and treatment and prognosis. Methods Totally 31 cases of PCNSL confirmed by pathology in the past 9 years, including 27 cases of primary brain lymphoma (PBL) and 4 cases of primary ocular lymphoma. The clinical features, diagnostic methods and therapeutic effects were retrospectively analyzed. After biopsy or surgery, patients mainly radiotherapy and chemotherapy based treatment. Chemotherapy with cyclophosphamide + doxorubicin + vincristine + prednisone or methotrexate + cytarabine programs; whole brain radiation 40 ~ 44Gy, local tumor shrinking radiotherapy dose of 11 ~ 25.4Gy. Eye lymphoma radiotherapy 25.2 ~ 30Gy. Results The prevalence of PCNSL was significantly increased (2.6-fold increase). All patients were normal immune. The median age at onset was 57 years (14-83 years), with PBL≥60 years (48.2%). 22 cases of multiple brain lesions (81.5%). Stereotactic biopsy confirmed 20 cases (one-time success rate of 90%), the surgical diagnosis of 11 cases. B-cell lymphoma accounted for 93.6%, T-cell lymphoma accounted for 6.4%. Four patients had been misdiagnosed 65 to 7 months outside the hospital. The whole group of PBL1 3,5-year survival rate split to 68.2%, 52.3% and 35.0%. The median survival time of the whole group was 37 months. Conclusion PCNSL often occurs in middle-aged and elderly people, and the main source of B cells. PBL clinical and imaging difficult to distinguish with other central nervous system tumors, easily misdiagnosed. In patients with suspected PBL, stereotactic brain biopsy can improve diagnostic efficiency. Comprehensive treatment of radiotherapy can improve the curative effect. Aged 60 years or older have a poor prognosis.