摘要目的 探讨胚胎发育不良性神经上皮肿瘤(DNT)的临床病理学、影像学特征、治疗及其预后情况.方法 回顾性分析10例经病理确诊的DNT患者资料,归纳其临床病理和影像学特征.结果 临床均以顽固性癫痫为主要症状,肿瘤位于颞叶5例、额叶3例、顶叶2例.CT扫描病灶呈低密度影,MRI呈T1WI低信号,增强后无强化,T2WI高信号,液体衰减反转恢复序列(FLAIR)检查瘤内可见等信号分隔,边缘环状高信号；无占位效应和瘤周水肿.肿瘤由特异性胶质神经元成分构成,包括少突胶质样细胞,“漂浮”神经元和星形胶质细胞.免疫组织化学:OLC阳性表达Olig2和S-100蛋白,神经元和部分OLC表达NeuN、突触素；Ki-67阳性指数均＜1％.治疗:9例行肿瘤全切术,1例为次全切,术后未行放疗和化疗.预后:均获随访,9例无复发,1例复发.结论 DNT确诊需结合临床病理、免疫组织化学及影像学检查.手术效果良好,术中脑电图描记可有效切除癫痫灶,术后无须放疗和化疗.

abstractsObjective To study the clinicopathologic features,radiologic findings,treatment options and prognosis of dysembryoplastic neuroepithelial tumor (DNT).Methods The clinicopathologic and radiologic features were retrospectively analyzed in 10 cases of DNT.Results Intractable partial seizure was the main presenting symptom in all patients. The tumor was located in temporal lobe ( number =5 ),frontal lobe (number =3 ) or parietal lobe (number =2 ).CT scan displayed a hypodense lesion.MRI scan revealed the tumor was non-enhancing T1 WI hypointense and T2WI hyperintense,with internal septation and hyperintense ring around the tumor seen on FLAIR image.There was neither peritumoral edema nor mass effect.Histologically,the tumor showed the presence of glioneuronal element,with oligodendrocyte-like cells, floating neurons, astrocytes and associated microcystic changes, Immunohistochemical study demonstrated positivity for NeuN and synaptophysin in the neurons and some oligodendrocyte-like cells.Olig2 and S-100 protein were also expressed in the oligodendrocyte-like cells.Ki-67 index were lower than 1％ in all cases.Nine cases were treated by complete surgical excision and the remaining case was subtotally excised. No post-operative chemotherapy or radiotherapy was given.One of the 10 cases recurred on follow up. Conclusions Correct diagnosis of DNT requires correlation with clinicopathologic,radiologic and immunohistochemical findings.Complete resection of the tumor and epileptogenic foci is the mainstay of treatment for DNT,with intraoperative EEG monitoring.Post-operative chemotherapy or radiotherapy is not required.