Objective: To assess clinical features and treatment outcomes in immunocompetent patients with primary central nervous system lymphoma (PCNSL). Methods: Sixty-two patients with PCNSL who attended Guangdong General Hospital between January 1998 and January 2012 were included. Survival curves were estimated using Kaplan-Meier survival methodology and statistical significance of continuous was assessed via the Cox proportional hazard model. Results: The median age of the patient cohort was 56 years, and the male to female ratio was 1.14∶1.00. The common presentations were increased intracranial pressure symptoms and neuron damage. Performance status of 54 (54/62, 87.1%) patients were the international prognostic index (IPI) 0-2. Diffuse large B-cell lymphoma (57/62, 91.9%) was most common, and the rest were T-cell lymphoma (4/62,6.4%) and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (1/62, 1.6%). In the series, 32 patients (32/62, 51.6%) had multiple lesions. Involvement of deep structures was found in 30 (30/62, 48.4%) patients. An elevated serum LDH level was detected in 19 (19/62, 30.6%) patients and the Ki-67 index was ≥90% in 38 (38/62, 61.3%) patients. Univariate analysis showed patients who were female, age<60 years, had WHO Eastern Cooperative Oncology Group performance status grade 0-2, single lesion, absence of deep structures involvement and normal LDH level showed better 2-year survival rate and longer median survival time. Significance was only seen in the normal LDH level group. Multivariate Cox regression analysis revealed that radical surgery only and Rituximab+ high-dose of methotrexate+ whole brain radiation therapy (WBRT) were independent prognostic indicators in PCNSL patients (P<0.05). Conclusions: PCNSL is a rare but aggressive tumor with poor prognosis. Patients treated with high-dose of methotrexate combining with rituximab, followed by WBRT have a better prognosis and longer survival time, and thus these could probably be a promising treatment.
目的： 探讨原发性中枢神经系统淋巴瘤的临床特征、组织病理学特点、治疗方案及其与预后的关系。 方法： 收集1998年1月至2012年1月期间广东省人民医院诊治的免疫功能正常的、有完整临床和随访资料、可进行系统性评估的原发性中枢神经系统淋巴瘤病例62例。采用Kaplan-Meier生存分析、Cox风险比例回归模型列出相关生存分析曲线，观察患者预后。 结果： 62例患者中位发病年龄约为56岁，男女发病率为1.14∶1.00，临床首发症状多为颅内压升高以及局灶神经损害。54例（54/62，87.1%）患者为国际预后指数（IPI）0~2。组织学类型上，弥漫性大B细胞淋巴瘤占91.9%（57/62），T细胞淋巴瘤占6.4%（4/62），结外边缘区黏膜相关淋巴组织淋巴瘤占1.6%（1/62）。其中，32例（32/62，51.6%）可见颅内多发病灶，30例（30/62，48.4%）可见深部器官累及。19例（19/62，30.6%）患者乳酸脱氢酶（LDH）升高，38例（38/62，61.3%）Ki-67阳性指数≥90%。单因素生存分析显示，患者性别、年龄、体能状态评分、IPI、是否为单发病变以及有无深处器官侵犯、LDH有无升高、不同的治疗方法与患者的预后密切相关。Cox风险比例回归模型多因素分析显示，应用利妥昔单抗+大剂量的甲氨蝶呤+全脑放疗治疗组患者的预后好，差异具有统计学意义（P<0.05）。 结论： 原发性中枢神经系统淋巴瘤进展快、预后差、临床表现复杂多样，应用利妥昔单抗+大剂量的甲氨蝶呤+全脑放疗治疗组中位总生存时间显著延长，可能是未来治疗方向之一。.
Keywords: Central nervous system neoplasms; Lymphoma, B-cell; Lymphoma, large B-cell, diffuse; Prognosis.