摘要
目的探讨胚胎发育不良性神经上皮肿瘤（DNT）的临床病理特征、影像学特点、治疗及预后。方法应用光镜及免疫组织化学染色对5例DNT进行观察分析。结果主要临床症状为顽固性癫痫发作,神经系统检查无阳性体征。MRI病变呈囊实性改变,无强化、无瘤周水肿及占位效应。肿瘤组织形态类似,由神经胶质-神经元成分构成,周围有少突胶质细胞样细胞（OLC）,其组织学特点是瘤组织黏液变明显,多见网状微囊变,单个神经元漂浮在微囊的黏液样基质中。免疫组织化学染色示神经元及部分OLC突触素、神经核抗原（NeuN）及S-100阳性,OLC胶质纤维酸性蛋白染色阴性,Ki-67低增殖活性。4例随访2-7年,术后均无复发。结论 DNT属良性肿瘤（WHOⅠ级）,手术切除即可治愈。结合临床表现、影像学及病理形态学和免疫组织化学结果DNT才确诊。
Objective To study the clinicopathologial and radiological features,treatment and prognosis in the patients with dysembryoplastic neuroepithelial tumors（DNT）.Methods The clinical and pathological data of 5 patients with DNT were analyzed retrospectively.The expressions of S-100 protein,synaptophysin,NeuN and glial fibrillary acidic protein（GFAP）in DNT were determined by immunohistochemical technique.Results Intractable partial seizure was the main symptom in all the patients,who had no other sign of nervous system.There was heterogeneous（cystic and solid）degeneration and there was no enhancement and peritumorous edema on MRI in all the patients,in whom there was no mass effect.The histomorphology of DNT was similar to oligodendroglioma.DNT consisted of the oligodendrocyte-like cells（OLC）,neurons and astrocytes.DNT had obvious mucoid and microcystic degeneration.These neurons were often depressively drifted in the mucoid matrix of the microcysts.The expressions of synaptophysin,NeuN and S-100 protein were positive in the neurons and some OLC of DNT.The expression of GFAP in the OLC of DNT was negative.The expression of ki-67 was positive in few cellar nuclei of DNT.Conclusions DNT is a benign tumor（WHO gradeⅠ）which can be cured by surgical excision.DNT did not recurre in 4 patients followed up.A definite diagnosis of DNT may be made by the clinical manifestations,radiological and histological examination,and immunohistochemical staining.
出处
《中国临床神经外科杂志》
2010年第5期270-272,共3页
Chinese Journal of Clinical Neurosurgery
