Objective:To evaluate the clinical pathology and imaging profile of the primary central nervous system diffuse large B-cell lymphoma (PCNS-DLBCL),and to investigate the features of PCNS-DLBCL with regard to immunohistochemistry,gene rearrangement and microRNA expression.Methods:The imaging,pathological and immu-nohistochemical data from 25 patients with identified PCNS-DLBCL were collected and analyzed,and a gene rear-rangement examination was performed for the heavy and light chains.The microRNA was extracted from paraffin-em-bedded tissue blocks of the patients with typical PCNS -DLBCL (n=10),germinal center-diffuse large B-cell lymphoma (GC-DLBCL,n=10)and non-germinal center-diffuse large B-cell lymphoma (NGC-DLBCL,n=10),and subject to microarray hybridization,in order to evaluate the difference between the three patient populations. Results:For the PCNS-DLBCL,≥2 involved cerebral lobes were usually observed (10/25),particularly including the frontal lobe (6/25 ).As seen in all the patients,large lymphocytes with an appearance similar to that of the lym-phoblasts were distributed around the blood vessels in a ring-like manner.The immunohistochemical study revealed a diffuse strong expression of CD20 and CD79a and absent expression of CD3,CD5,CD23 and CyclinD1 in 25/25 pa-tients with PCNS-DLBCL.The presence of CD10 (3/25,12%),Bcl -6 (19/25,76%)and Mum -1 (22/25, 88%)was also confirmed.Gene rearrangement determination showed a monoclonality of B -lymphocyte in 24/25 (96%)patients.As revealed by microRNA microarray hybridization,the 788 microRNAs or corresponding fragments increased ≥2 folds,and 401 microRNAs or corresponding fragments decreased ≥0.5 folds,were detected in PCNS-DLBCL when compared with those in NGC-DLBCL;and 611 increased≥2 folds and 229 decreased≥0.5 folds were found when compared with the GC-DLBCL.Conclusion:PCNS-DLBCL is commonly seen in old males and characterized by multiple involvements.The immunohistochemical examination showed an origin from activated B-lymphocytes in the majority of the cases.As demonstrated by microRNA microarray hybridization,the diagnostic mi-croRNA-associated biomarker(s)may be present as a matter of fact that the expression of microRNA in PCNS-DL-BCL is quite different from those in NGC-DLBCL and GC-DLBCL.With respect to the number of differently ex-pressed microRNAs,PCNS-DLBCL is somewhat similar to GC-DLBCL as indicated by the comparable outcome of the two isoforms.%目的：观察原发性中枢系统弥漫大B细胞淋巴瘤（PCNS-DLBCL）临床病理学、影像学特点，探讨其免疫组化、基因重排检测及microRNA表达特征。方法：收集确诊的PCNS -DLBCL 25例，观察并分析影像学、病理组织学、免疫组化标记特点，并进行重链和轻链基因重排检测。对其中10例典型PCNS-DLBCL、10例颅外生发中心来源的弥漫大B细胞淋巴瘤（GC-DLBCL）和10例颅外非生发中心来源的弥漫大B细胞淋巴瘤（NGC-DLBCL）的石蜡包埋组织块微切割提取microRNA，进行芯片杂交，对三者间的差异性进行比较。结果：PCNS-DLBCL多累及两个或两个以上脑叶（10/25例），其中额叶受累最为常见（6/25例）。所有病例均可见中心母细胞样的大淋巴细胞围绕血管呈靶环样生长。免疫组化染色结果显示，25/25例均弥漫强阳性表达CD20、CD79a，不表达CD3、CD5、CD23、CyclinD1，Ki-67阳性率在50％-90％（平均80％），仅有3例表达CD10（占12％），19例表达Bcl-6（占76％），22例表达Mum-1（88％）。基因重排检测显示24/25例呈B细胞单克隆性（96％）。microRNA芯片杂交显示，与NGC-DLBCL比较，升高2倍及以上者788个microRNA或片段，下降0．5倍以下者401个microRNA或片段；与GC-DLBCL比较，升高2倍及以上者611个microR-NA或片段，下降0．5倍以下者229个microRNA或片段。结论：PCNS-DLBCL以老年男性好发，常累及多个部位，免疫组化显示大部分为活化B细胞来源，基因重排检测B细胞单克隆性高，microRNA芯片杂交显示， PCNS-DLBCL microRNA表达明显不同于颅外NGC-DLBCL和GC-DLBCL，可能存在microRNA诊断性标志物。从microRNA表达差异的数量看，PCNS-DLBCL与颅外GC-DLBCL差异较小，此与二者的预后接近相关。
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