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目的观察胚胎发育不良性神经上皮瘤(DNT)的病理形态学及免疫组织化学特点,探讨其与皮质发育不良之间的关系以及组织来源。方法应用光镜和免疫组织化学 EnVision 法对14例 DNT 进行观察分析,并对患者进行长期随访。结果肿瘤位于颞叶的有11例,镜下由神经元和神经胶质成分混合构成,9例可见“特异的胶质神经元结构”。1例为简单型,8例为复杂型,5例为非特异型。11例标本充足的病例10例伴有皮质发育不良改变,表现为分子层和(或)白质内异位神经元数量增多(7例),分子层内可见成行的外颗粒细胞层残留(4例),脑皮质的结构异常(10例),以及出现异常形态的神经细胞。免疫组织化学染色少突胶质样细胞(OLC)均呈现 Olig2的免疫反应性,部分 OLC 表达 nestin、微管相关蛋白2、神经丝蛋白、胶质纤维酸性蛋白,但神经元核抗原呈阴性。癫痫控制结果Ⅰ级12例,Ⅱ级2例,无肿瘤复发。结论 DNT 与皮质发育不良关系密切,应用免疫组织化学染色有助于 DNT 和皮质发育不良的诊断。
Objective To observe the pathological and immunohistochemical features of embryonic dysplastic neuroepithelial neoplasia (DNT) and to explore its relationship with cortical dysplasia and its origin. Methods 14 cases of DNT were observed and analyzed by light microscopy and immunohistochemistry EnVision, and the patients were followed up for a long time. Results Tumors were located in the temporal lobe in 11 cases. Microscopically, neurons and glial components were mixed. Nine cases showed “specific glial neuron structure”. One case was simple, eight cases were complex, and five cases were non-specific. In 11 cases with adequate specimens, 10 cases were associated with cortical dysplasia. The number of ectopic neurons in the molecular layer and / or white matter increased (7 cases), and the outer granule cell layer remained in the molecular layer (4 cases ), Structural abnormalities in the cortex (n = 10), and abnormal neuronal cells. Immunohistochemical staining of oligodendrocyte-like cells (OLC) showed Olig2 immunoreactivity, some OLC expressed nestin, microtubule-associated protein 2, neurofilament protein, glial fibrillary acidic protein, but neuronal nuclear antigen was negative. Epilepsy control results Ⅰ grade 12 cases, Ⅱ grade 2 cases, no tumor recurrence. Conclusions DNT is closely related to cortical dysplasia. Immunohistochemical staining is helpful for the diagnosis of DNT and cortical dysplasia.