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目的探讨儿童小脑脑室外神经细胞瘤的临床病理和生物学特点。方法对1例儿童小脑脑室外神经细胞瘤进行组织形态学、影像学和免疫组化研究,并复习相关文献。结果患儿男性,6岁。突发眩晕反复发作3个月。头颅MRI示右侧小脑半球囊性占位,T1等低信号,T2高信号,囊内附壁结节不规则强化。组织学表现为弥漫一致的小圆细胞构成特征性的无核原纤维岛及围绕血管形成菊形团结构,另一特点是瘤细胞具有少突胶质细胞样特征,可见散在的节细胞分化。免疫表型示神经毡基质Syn强(+),肿瘤细胞Syn、NSE、Oligo2、NeuN和NF(+),少数细胞GFAP(+);CD99,S-100,vimentin和EMA均(-);血管CD34(+),Ki-67<2%。结论脑室外神经细胞瘤是一种较为少见的肿瘤,发生于儿童且位于小脑者更为罕见,组织学特点多数为典型性,一般无复发或转移,完整切除预后较好。
Objective To investigate the clinicopathological and biological features of cerebellar extracerebral neuroblastoma in children. Methods One case of children with cerebellar extracerebral neuroblastoma was studied by histomorphology, imaging and immunohistochemistry. The related literatures were reviewed. Results Male children, 6 years old. Sudden onset of vertigo repeated attacks for 3 months. Head MRI showed right cerebellar hemispheric cystic lesions, T1 and other low signal, T2 high signal, intracapsular ciliated nodules irregular enhancement. Histological manifestations of diffuse small round cells constitute a characteristic nuclear fibrillated island and around the formation of vascular chrysotile structure, the other is characterized by tumor cells with oligodendrocyte-like features, see scattered interstitial cell division. The immunophenotype showed that Syn Matrix (+), Syn, NSE, Oligo2, NeuN and NF (+), GFAP (+) and CD99, S-100, vimentin and EMA CD34 (+), Ki-67 <2%. Conclusions Extracerebral neuroblastoma is a rare tumor. It occurs more rarely in children and in the cerebellum. Most of the histological features are typical, usually without recurrence or metastasis, and the prognosis of complete excision is good.