【摘要】 目的 探讨儿童继发性中枢神经系统淋巴瘤(SCNSL)的临床特征、疗效及预后影响因素，为SCNSL患儿的诊治及预后评估提供参考。方法 回顾性分析山东大学齐鲁医院儿科2013年1月1日—2024年8月31日收治的SCNSL患儿的临床资料，分析比较预后良好者与预后不良者的差异。结果 研究纳入175例淋巴瘤患儿，SCNSL患儿15例，总患病率8.6%,且均为非霍奇金淋巴瘤(NHL),患病率为10.2%。15例SCNSL患儿初诊中位发病年龄8岁6个月(2岁11个月～16岁);男∶女=14∶1;临床分期以中晚期(Ⅲ-Ⅳ期)为主；常见病理类型依次是Buirkitt淋巴瘤/白血病、T淋巴母细胞淋巴瘤及弥漫大B细胞淋巴瘤。初诊确诊者6例，治疗中确诊者9例。诊断SCNSL时病程中位时间为3个月(10天～12个月);伴神经系统临床表现者33.3%;脑脊液阳性者86.7%;影像学检查阳性者26.7%。常规治疗早期疗效尚可，11例(78.6%)完全缓解，3例(21.4%)未缓解。截至末次随访日期，存活12例，死亡3例，中位生存期16(1～125)个月，1年无事件生存率为66.7%。预后良好组10例占2/3,预后不良组5例占1/3,两组单因素及多因素分析均显示，早期疗效是独立预后危险因素(P<0.05)。结论 NHL患儿SCNSL发生率为10.2%,以男性多见，治疗中诊断者占60%。多数经常规治疗早期可完全缓解，诊断后1年无事件生存率为66.7%,早期疗效是独立预后危险因素。更多还原

【Abstract】 Objective To investigate the clinical characteristics, therapeutic efficacy, and prognostic factors of secondary central nervous system lymphoma(SCNSL) in children, providing references for the diagnosis, treatment, and prognostic assessment. Methods A retrospective analysis of clinical data from children with SCNSL admitted to the Department of Pediatrics, Qilu Hospital of Shandong University, from January 1, 2013, to August 31, 2024, was conducted. The differences between the good prognosis group and the poor prognosis group were compared. Results A total of 175 children with lymphoma were included in the study, of whom 15 had SCNSL, with an overall morbidity rate of 8.6%. All cases were non-Hodgkin lymphoma(NHL), with an morbidity rate of 10.2% in NHL. The median age at initial diagnosis of the 15 SCNSL patients was 8 years and 6 months(range, 2 years and 11 months to 16 years); the male-to-female ratio was 14:1. The majority of cases were in the middle-to-late clinical stages(Ⅲ-Ⅳ), accounting for 86.7%. The most common pathological types were Burkitt lymphoma/leukemia, T-lymphoblastic lymphoma, and diffuse large B-cell lymphoma. Six cases were confirmed at initial diagnosis, and nine cases were confirmed during treatment. The median duration of illness at the time of SCNSL diagnosis was 3 months(range, 10 days to 12 months). Neurological symptoms were present in 33.3% of the patients. Cerebrospinal fluid(CSF) positivity was observed in 86.7% of the patients, while positive imaging findings were seen in 26.7%. The early therapeutic efficacy of conventional treatment was satisfactory, with 11 cases(78.6%) achieving complete remission and three cases(21.4%) showing no remission. As of the last follow-up, 12 patients were still alive, and three had died. The median survival time was 16 months(range, 1 month to 125 months), and the 1-year event-free survival rate was 66.7%. The good prognosis group included 10 cases(two-thirds), while the poor prognosis group had five cases(one-third). Both univariate and multivariate analyses indicated that early therapeutic efficacy was an independent prognostic factor(P<0.05). Conclusions The incidence rate of SCNSL in NHL pediatric patients was 10.2%, with a predominance in males and 60% of cases diagnosed during treatment. Most patients achieved complete remission with conventional treatment in the early stage, and the 1-year event-free survival rate after diagnosis was 66.7%. Early efficacy is an independent prognostic risk factor.更多还原