目的 探讨罕见中枢神经系统肿瘤——胚胎发育不良性胶质神经上皮肿瘤的临床病理及影像学特征,以提高诊断的准确性.方法 回顾性分析2001年1月至2011年12月7例外科手术后病理证实的胚胎发育不良性胶质神经上皮肿瘤的临床表现、影像学特点、手术方法、组织学特点及免疫组化结果.结果 肿瘤4例位于颞叶,2例位于额叶,1例位于侧脑室及三脑室.具有特征性的“三角征”、“脑回征”等影像学表现和“特异性胶质神经元成分”的病理学表现.全部病例经显微手术治疗,随访1至10年,癫痫控制良好,肿瘤未见复发及转移,效果满意.结论 胚胎发育不良性胶质神经上皮肿瘤具有特征性的影像学和病理学表现,病理组织学“特异性胶质神经元成分”对其诊断和鉴别诊断具有重要价值.%Objective To investigate the pathological and imaging characteristics of dysembryoplastic neuroepithelial tumor (DNT). Methods The data including clinical manifestations, imaging features, surgical methods, fais-tological features and immunohistochemical results was collected and analyzed in 7 cases with DNT from January 2001 to December 2011 retrospectively. Results Tumors were located in the temporal lobe in 4 cases, in the frontal lobe in 2 cases and in the lateral third ventricle in 1 case. DNT had distinctive MRI imaging features such as "triangular-shaped pattern" or "gyriform pattern" and specific pathological features- "specific glioneuronal element". All patients received microsurgery and had undergone follow-up for 1 to 10 years. All patients had satisfied outcomes with well-controlled epilepsy and without relapse or metastasis. Conclusion DNT has typical imaging and pathological features, in which the pathological feature- "specific glioneuronal element" is valuable for the diagnosis and differential diagnosis of DNT.
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