颅骨内板下尤文肉瘤／原始神经外胚层肿瘤：一例报告并文献复习  

EWS/PNET under lamina interna cranii: one case report with review of literatures

作　　者：孙翠云[1] 于士柱[1] 

机构地区：[1]天津医科大学总医院神经病理室,天津市神经病学研究所,300052

出　　处：《中国现代神经疾病杂志》2009年第6期605-607,共3页Chinese Journal of Contemporary Neurology and Neurosurgery

摘　　要：目的探讨颅内尤文肉瘤/原始神经外胚层肿瘤的临床病理学特征。方法对1例颅骨内板下尤文肉瘤/原始神经外胚层肿瘤患者的临床特征及组织形态学和免疫组织化学染色进行分析,并复习相关文献。结果男性患者,56岁。头部CT检查显示,右侧顶枕部颅骨内板下不均匀明显强化影,边界清晰光滑,周围可见较丰富血管。肿瘤细胞呈弥漫性、一致性片状密集分布,细胞质稀少,细胞核呈圆形或类圆形、深染,核分裂象多见。肿瘤细胞CD99蛋白、神经丝蛋白表达阳性;S-100蛋白、白细胞共同抗原、CD45RO蛋白、CD20蛋白、免疫球蛋白λ轻链和κ轻链、嗜铬素A、神经元特异性烯醇化酶表达阴性;Ki-67抗原标记指数为16.74%。结论颅内尤文肉瘤/原始神经外胚层肿瘤临床罕见,影像学表现具有一定诊断意义,明确诊断依赖特征性的细胞遗传学改变和免疫学表型。尤文肉瘤/原始神经外胚层肿瘤是组织形态学多样的小圆细胞恶性肿瘤,与其他类型的小圆细胞恶性肿瘤难以鉴别,须依靠免疫组织化学检测方能明确诊断。Objective To investigate the clinicopathologieal features of Ewing sarcoma/primitive neuroectodermal tumor （EWS/PNET）. Methods The clinical manifestations, imaging and histopathological features and immunophenotype were analysed in one case of EWS/PNET, with review of the literatures. Results The patient was a 56 year-old man. CT examination revealed a distinct enhanced mass under right lamina interna cranii with many blood vessels around it. The borderline is clear and smooth, and the density is not homogeneous. Histologically, the tumor cell distribution was diffuse and uniform patchy intensive, and the tumor cells contained only a few cytoplasm and many undifferentiated round cells with round or irregular hyperchromatic nuclei. Abundant mitotic figures were present. Immunohistochemical analysis showed positive expression for CD99 protein and neurofilament （NF） in tumor cells. The tumor cells presented negative for S-100 protein （S-100）, leukocyte common antigen （LCA）, CD45RO protein, CD20 protein, immunoglobulin λ light chain and κ light chain, chromogranin A （CgA） and neuron-specific enolase （NSE）. The Ki-67 labeling index was 16.74%. Conclusion EWS/PNET is a rare tumor. The imaging features can offer certain diagnostic significance. Histologically, EWS/PNET can be a diagnostic challenge because many tumors exhibite features of the so-called small round cell tumor. Molecular genetic study and immunohistochemical study may be valuable in differential diagnosis. Fluorescence in situ hybridization （FISH） and （or） reverse transcription- polymerase chain reaction （RT- PCR） are valuable adjuncts for the evaluation of undifferentiated small round cell tumors, particularly in cases with indeterminated histological and （or） immunohistochemical features.

关 键 词：脑肿瘤 肉瘤 Ewing 神经外胚瘤 原始 免疫组织化学 

分 类 号：R739.4[医药卫生—肿瘤]