脊髓脑室外神经细胞瘤3例临床病理观察

Extraventrieular neurocytoma of the spinal cord:a clinicopathological analysis of three cases

目的 探讨脊髓脑室外神经细胞瘤的临床病理学特点.方法 应用HE及免疫组化染色对3例脊髓脑室外神经细胞瘤进行形态学分析,并结合文献报道的18例进行比较研究.结果 3例患者中,1例女性46岁,2例男性分别为42岁及25岁,病变分别位于颈2-3脊髓髓内及胸髓.镜下3例组织形态大致相似,肿瘤由大小一致的小圆细胞组成,被纤维、血管分割成团、巢状,部分核周空晕似少突样胶质细胞,其间见散在的节细胞样神经元.另见少许神经毡岛样结构,1例局灶可见血管内皮细胞增生.免疫组化：肿瘤细胞Syn和NeuN（＋）,Ki-67阳性指数2％-5％.结论 脊髓脑室外神经细胞瘤以男性患者居多,发病年龄较脑室内神经细胞瘤年龄偏大,除了骶髓外,所有脊髓节段均可波及.组织学上易出现节细胞分化和形态学间变,术后复发较中枢神经细胞瘤更为多见.

Objective To investigate the clinical and pathological characteristics of extraventricular neurocytoma of the spinal cord.Methods The morphologic and clinical findings for 3 cases of extraventricular neurocytoma （EVN） of the spinal cord were studied,combined with 10 cases reported in the literature,and the features in the clinical pathology and biological behaviour were discussed.Results We reported three cases of EVN of the spinal cord,one females and two males,aged 48,42 and 25 years,respectively,at diagnosis.MRI revealed a mass occupying the entire cervical spinal cord of two-three levels; after operation,one case recurred twice.Histological examination showed that the lesions were composed of uniform cells with small round nuclei,finely speckled chromatin and scant cytoplasm arranged in sheets,ribbons or nodules separated by a fine vascular network.Some cells had perinuclear haloes resembling oligodendroglia.There were nucleus-free areas of neuropil that looked like neuropil island.Dysplastic ganglion cells/neurons,both isolated and in clusters,were scattered among the round cells.Vascular proliferation was found in the case 1.Immunohistochemical examination showed that Syn was strongly positive in the neuropil,immunonegative for tumor cells.MIB-1 score was 2-5％,which so make a diagnosis of the extraventricular neurocytoma of the spinal cord.Reviewing the literature of 18 of extraventricular neurocytoma of the spinal cord plus our three cases,total was 21 cases,15 males and 6 females,with a mean age at onset of 35.26 years （range 1.3-67 years）.The 6 tumors located on the cervical cord,9 in the thoracic cord,5 cases found both in the cervical cord and thoracic cord,only one located in the thoracic and lumbar segments,and it more frequently occured in the cervical cord and thoracic cord.The total resection was performed in 9 cases,partial removal in 12 cases.After operation,4 cases were recurrence.4 cases were subjected to radiotherapy including one of recurrence case.It more commenly appeared ganglion cell differentiation in 9/21 cases.The five cases presented with atypical features including necrosis,vascular proliferation.Mitotic activity was more than 3/10 HPF and MIB-1 labelling index was lager than 5％.Conclusion EVN of the spinal cord shows a male predominance,and age is older than that of central neurocytoma in the onset.All spinal cord levels have been affected except the sacral region.In histology,EVN of the spinal cord exhibits a high degree of ganglionic differentiation,morphologic atypia and more frequent recurrence compared with CN.