重度局灶性皮层发育不良和皮层错构性肿瘤病变的临床病理分析

摘要

Objective To observe the clinicpathological characteristics of severe focal cortical dysplasia and epilepsy-relatedcortical hamarticai tomour.And to discuss their morphological changes and correlation.Method Measure the fresh resections of epileptic focus with the same way:cut open,fixed with 10% neutral formalin solution,using paraffin sections,routine staining,special staining and immunohistochemical staining,microscopic observation in detail.They were classified according to the pathological diagnostic criteria of cortical dysplasia,diagnosing with some immunohistochemical indexes and special staining.Results Of all the 133 samples,there were 5 typical FCD Ⅱ B,6 cortical hamartoma related with epilepsy.There were severe layered disorder,typical immature neurons,great nerves,abnormal neurons and balloon-like cells in lesions of 5 FCD Ⅱ B.Except of the same lesions,there were glial neuron tumor-like proliferations.In adittional,there were mucinous background,gliosis and neurons reducing in 6 cortical hamartomas related to epilepsy.Conclusions The pathological form of severe focal cortical dysplasia is similar to that of cortical hamartomas.So they may be the continuous development of the same disease chart.%目的 观察癫痫手术切除脑病变中,重度局灶性脑皮层发育不良(FCD Ⅱ B)与皮层错构性癫痫相关肿瘤(神经元和混合性神经元-胶质瘤)的临床病理特点.探讨两者的形态学变化及相瓦关系.方法 手术切除脑癫痫病灶,新鲜标本进行测量、切开、照相,显微镜下形态学观察.按脑皮质发育不良的病理诊断标准分类.应用一组免疫组化和特染指标协助诊断.结果 在133例癫痫脑病变切除病例中,典型FCD Ⅱ B者5例,皮层错构性癫痫相关肿瘤6例.在5例FCDⅡB中,存在重度脑皮层分层紊乱,典型的不成熟神经元、巨大神经元、异常神经元和气球样细胞.在6例脑皮层错构性癫痫相关肿瘤中,同样有以上病变,另外出现胶质神经元瘤样增生,DNT有黏液性背景、胶质增生及神经元减少.结论 FCD Ⅱ B与皮层错构性癫痫相关肿瘤有许多相似的病理变化,两者可能是癫痫脑病理谱系的连续病变,有待深入研究.