Daumas-Duport C
Department of Pathological Anatomy, Sainte-Anne Hospital, Paris, France.
Brain Pathol. 1993 Jul;3(3):283-95. doi: 10.1111/j.1750-3639.1993.tb00755.x.
Dysembryoplastic neuroepithelial tumours (DNTs) are a group of supratentorial cortical benignant lesions that superficially resemble mixed oligo-astrocytomas, oligodendrogliomas or astrocytomas. Clinically these tumours are associated with partial seizures beginning before the age of 20 years, with no neurologic deficit and no stigmata of phacomatosis. In the revised WHO classification, DNTs have been incorporated among the category of neuronal and mixed neuronoglial tumours. This classification describes a histologic variant characterized by the following criteria: cortical location, multinodular architecture--the nodule being made of multiple variants looking like astrocytomas, oligodendrogliomas or oligo-astrocytomas, foci of dysplastic cortical disorganization and the presence of a glioneuronal element showing a columnar structure perpendicular to the cortical surface. A study of 14 cases for which only a specific glioneuronal element could be identified demonstrated that this specific element is sufficient for diagnosing DNTs and that the spectrum of DNTs includes a simple form with a unique glioneuronal element. Preoperative imaging follow-up data, in the series of 23 simple and complex forms, indicated that DNTs are perfectly stable. However, these tumours may show a high MIB 1 labeling index.
胚胎发育不良性神经上皮肿瘤（DNTs）是一组幕上皮质良性病变，表面上类似于混合性少突星形细胞瘤、少突胶质细胞瘤或星形细胞瘤。临床上，这些肿瘤与20岁前开始的部分性癫痫发作有关，无神经功能缺损，也无神经皮肤综合征的体征。在修订后的世界卫生组织分类中，DNTs已被纳入神经元和混合性神经元-神经胶质肿瘤类别。该分类描述了一种组织学变异型，其特征如下：皮质定位、多结节结构——结节由多种类似星形细胞瘤、少突胶质细胞瘤或少突星形细胞瘤的变异型组成、发育异常的皮质结构紊乱灶以及存在显示垂直于皮质表面的柱状结构的神经胶质神经元成分。一项对14例仅能识别特定神经胶质神经元成分的病例的研究表明，这种特定成分足以诊断DNTs，且DNTs的谱系包括具有独特神经胶质神经元成分的简单形式。在23例简单和复杂形式的病例系列中，术前影像学随访数据表明DNTs非常稳定。然而，这些肿瘤可能显示出较高的MIB 1标记指数。
