Clinical data of 15 primary central nervous system lymphoma (PCNSL) children aged ≤18 years admitted to our hospital between May 2013 to May 2023 were retrospectively analyzed. Our goal was to summarize the clinical features of children and investigate the therapeutic effect of a high-dose methotrexate (HD-MTX) based chemotherapy regimen on this disease. The male-to-female ratio was 2.7∶1, and the median age was 7.2 (2.3-16.4) years at diagnosis. The initial clinical symptoms were primarily cranial hypertension, with imaging findings revealing multiple lesions. Pediatric PCNSL with normal immune function has a favorable prognosis with HD-MTX-based chemotherapy. Patients with a stable disease can be treated with minimal or no maintenance. HD-MTX-based chemotherapy remains effective when the disease progresses or recurs after an initial course of non-HD-MTX-based chemotherapy.
回顾性分析首都医科大学附属北京儿童医院2013年5月至2023年5月收治年龄≤18岁的15例非免疫缺陷原发中枢神经系统淋巴瘤（PCNSL）患儿的临床资料，总结临床特点以及基于病理组织亚型的、以大剂量甲氨蝶呤（HD-MTX）为基础的化疗方案的治疗效果。男女比例2.7∶1，中位确诊年龄7.2（2.3～16.4）岁，临床首发症状以颅高压伴颅神经受损为主，影像学以多发病灶为主。免疫功能正常的儿童PCNSL发病率极低，以HD-MTX为基础的化疗整体预后良好，部分病情评估稳定者可减量维持或不予维持治疗。初治以非HD-MTX为主的化疗，病情进展或复发患儿再次予HD-MTX为基础的化疗仍有效。.