原发性中枢神经系统淋巴瘤26例的临床病理分析

Clinicopathological analysis of 26 patients with primary lymphoma of central nervous system

目的:探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphomas,PCNSL)的临床病理特征、诊断与鉴别诊断及预后。方法:收集2013至2020年上海市第一人民医院诊断的26例PCNSL的临床病理资料,结合光镜、免疫组织化学及原位杂交进行分析。结果:全部病例均经活检或切除术后病理确诊,临床以肢体运动障碍为最常见症状,大脑半球最常受累;24例为弥漫性大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL),免疫组织化学表达B细胞标记,其余2例分别为淋巴瘤样肉芽肿(lymphomatoid granulomatosis,LYG),伴EBER阴性和非特指型外周T细胞淋巴瘤(non-specific peripheral T-cell lymphoma,PTCL-NOS),伴EBER阳性。17例患者获得随访资料,随访1~8年,其中6例至今存活,11例死亡,生存期为1~30个月。结论:PCNSL较少见,临床特征缺乏特异性,诊断需与胶质瘤、转移癌、感染等鉴别。非生发中心细胞样(germinal centre B-cell-like,GCB)型DLBCL相对多见,预后更差,需在明确病理分型的情况下进一步优化治疗方案。

Objective:To investigate the clinicopathological characteristics of primary central nervous system lymphomas(PCNSL).Methods:The clinicopathological data of 26 patients with PCNSL diagnosed in Shanghai General Hospital from 2013 to 2020 were collected and analyzed by light microscopy,immunohistochemistry and in situ hybridization.Results:All patients were pathologically confirmed by biopsy or resection.Limb movement disorder was the most common symptom,and cerebral hemisphere was most frequently involved.Twenty-four patients had diffuse large B-cell lymphoma with B cell markers expressed by immunohistochemistry,and the remaining 2 patients had lymphomatoid granulomatosis(LYG),with EBER negative,and non-specific peripheral T-cell lymphoma(PTCL-NOS)with EBER positive.Seventeen patients were followed up for 1–8 years,of which 6 survived and 11 died,with survival ranging from 1 to 30 months.Conclusion:PCNSL is rare and lacks specificity in clinical features. Diagnosis depends on pathology to distinguish it from glioma, metastasis, and infection. Non-GCB type DLBCL is relatively common and has a worse prognosis, which requires further optimization of the treatment plan under the condition of clear pathological classification.