摘要目的 探讨原发性中枢神经系统(CNS)组织细胞肉瘤的临床病理学特征、诊断、鉴别诊断及预后.方法 对解放军总医院2005至2018年会诊的3例原发性CNS组织细胞肉瘤进行病理分析,结合文献复习并总结.结果 3例患者女性2例,男性1例,年龄36、44、58岁,中位年龄44岁,影像学检查例1考虑脑膜瘤,例2考虑高级别胶质瘤,例3考虑为转移癌或高级别胶质瘤.组织学表现为大量单核组织样细胞呈弥漫分布,破坏脑组织,瘤细胞多形性,体积大,黏附性差,胞质嗜酸或空泡状,核圆形或不规则折叠状,偏位,异型性明显,核分裂象多见,见多核瘤巨细胞,并可见噬血现象,背景中见小淋巴细胞、浆细胞、良性组织细胞、中性粒细胞及嗜酸性粒细胞等反应性细胞;3例中有1例局部见梭形细胞区域.免疫组织化学染色显示肿瘤细胞CD163、CD68、波形蛋白阳性,溶菌酶部分阳性,S?100蛋白局部阳性,2例BRAF V600E阳性,1例CD45、CD45RO、CD4、CD34部分阳性,胶质纤维酸性蛋白(GFAP)、Olig?2、细胞角蛋白、上皮细胞膜抗原(EMA)、SSTR2、CD99、CD117、髓过氧化物酶(MPO)、CD1a、Langerin、CD21、CD23、CD35、CD15、CD30、CD38、CD138等阴性,Ki?67阳性指数30%～75%,网织纤维染色见局部肿瘤细胞团周围丰富.分子检测例2、例3显示BRAF V600E突变.结论 原发性CNS组织细胞肉瘤是罕见的CNS高度恶性肿瘤,确诊依赖病理学诊断,并需与CNS其他淋巴造血系统恶性肿瘤进行鉴别,治疗方法为手术、辅以放化疗,预后较差,完整切除辅以高剂量集中放疗有助于改善预后.

abstractsObjective To study the clinicopathological features, differential diagnosis and prognosis of primary histiocytic sarcoma of central nervous system(CNS). Methods Three cases of CNS histiocytic sarcoma were collected at Chinese People′s Liberation Army General Hospital from 2005 to 2018. Their clinicopathological characteristics were analyzed, and the related literature reviewed. Results The three patients included two females and one male, aged 36, 44, 58 years (median 44 years). MRI showed heterogeneously enhancing lesions which were considered meningioma, high?grade glioma or metastatic carcinoma. Histopathologically there were moderately pleomorphic, mitotically active tumor cells with a loose arrangement, effacing the normal brain tissue. These cells possess abundant eosinophilic cytoplasm, highly atypical nuclei, predominant nucleoli, and hemophagocytosis; multinucleated or spindled forms were also seen, as was background reactive inflammation. The tumor cells were typically positive for CD68, CD163, vimentin and lysozyme, S?100 protein, two of three cases were positive for BRAF V600E,one of three cases was partly positive for CD45, CD45RO, CD4, CD34, and negative for GFAP, Olig?2, CK, EMA, SSTR2, CD99, CD117, MPO, CD1a, Langerin, CD21, CD23, CD35, CD15, CD30, CD38, and CD138. The index of Ki?67 was 30%-75%. Rich reticular fiber network was seen in all cases; BRAF V600E mutation was present in two cases. Conclusions CNS histiocytic sarcoma is a rare malignant tumor; histopathologic and immunohistochemical examination are necessary for the diagnosis and to exclude other primary CNS and hematolymphopoietic tumors. Primary CNS histiocytic sarcoma is treated by surgery, chemotherapy and radiation therapy, but the prognosis is poor. Complete resection combined with high dose focused radiotherapy can improve the prognosis.