目的:探讨胚胎发育不良性神经上皮肿瘤(DNT)的临床、影像及病理学特征、诊断及鉴别诊断.方法:回顾性分析8例胚胎发育不良性神经上皮肿瘤患者的临床和影像学资料,进行光镜和免疫组织化学染色观察,并获得6例的随访资料.结果:胚胎发育不良性神经上皮肿瘤男性7例,女性1例,年龄为5～19岁,平均年龄13岁,5例以癫痫小发作为主要临床表现,病变均位于幕上,以皮层为主,影像学检查均无明显的占位效应及瘤周水肿。肿瘤细胞主要由少突胶质样细胞(OLC)、神经元和星形细胞组成,4例伴有皮质发育不良。免疫组织化学结果为神经元及部分少突胶质样细胞呈嗜铬素A、突触素及S-100阳性表达;少突胶质样细胞呈胶质纤维酸性蛋白(GFAP)阴性表达,而星形细胞呈GFAP阳性表达;Ki-67抗原标记指数lt;1%。结论: 胚胎发育不良性神经上皮肿瘤为WHOⅠ级良性肿瘤,可结合临床、影像及病理学表现明确诊断,预后良好,无需放疗和化疗。
ObjectiveTo analyze the electro-clinical characteristics and surgical outcome of low-grade developmental tumors in temporal lobe. MethodsThe onset age, seizure duration, seizure types, electroencephalogram and surgical outcome of 49 patients with low-grade developmental tumor of temporal lobe were analyzed retrospectively. ResultsTwo groups of the seizure types were divided. The first group was spasm, the other was focal onset. There were 12 cases in spasm group, with an average onset age of (1.00±0.59) years. The discharge was extensive and multi-brain-area locaded, especially in the temporal montages and the ipsilateral posterior montages. There were 37 cases in second group, with an average onset age of (8.90±8.84) years, mainly including autonomic seizure, tonic seizure and automotor seizure. In this group, the discharge was mainly recorded in the temporal montages, which could spread to the frontal montages and less locaded in posterior montages. The difference of onset age between the two groups was statistically significant (P<0.01). The average follow-up of spasm group was (2.80±1.57) years, and the surgical outcome of all patients in this group were all Engel I (100.00%, 12/12). The focal onset group was followed up for an average of (6.50±4.78) years, and the rate of Engel I was 91.80% (34/37). There was no significant difference between the two groups (P>0.05). ConclusionsFor low-grade developmental tumors in temporal lobe, there are two seizure types, including spasm and focal onset. The onset age of spasm is earlier, while patients with focal onset mostly start at childhood or older, rare in infancy. Surgery has a good effect on the treatment of temporal lobe developmental tumor epilepsy.