胚胎发育不良性神经上皮肿瘤临床病理分析(附4例报告).docVIP

胚胎发育不良性神经上皮肿瘤临床病理分析(附4例报 告) 【中图分类号】R739?4【文献标识码】A【文 章编号】1672 — 3783(2011)12 — 0069 — 01 【摘要】目的：探讨胚胎发育不良性神经上皮肿 瘤(DNT)的临床表现、影像学检查和病理学特点。 方法：回顾性分析4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。结果：DNT典型的临床表现为难治性癫痫， 大多数于20岁以前发病。病变主要位于幕上大脑，影 像学检查无明显瘤周水肿及占位效应。光镜下瘤组织 结节状分布伴网状囊性变，主要由少突胶质细胞样的 细胞(OLCs)、成熟神经元和星形细胞组成。免疫组织 化学染色示少枝胶质细胞样细胞表达S-100,部分表 达突触素(Syn),不表达胶质纤维酸性蛋白(GFAP)； 神经元细胞表达S —100＞突触素(Syn)和神经元特 异核蛋白(NeuN)。结论：DNT是手术可治愈的良性 病变，无需放疗和化疗。需结合临床表现、影像学及 病理形态学和免疫组织化学结果DNT才确诊。 【关键词】胚胎发育不良性神经上皮肿瘤；临床 病理；诊断；免疫组化 Cliniwpathological analysis of dysembryoplastic neuroepithelial tumors (a report of 4 cases) Hua ng Xin’Zu baohua ? [Abstact】Objective: To investigate clinical,radiological and pathological characteristics in the patients with dysembryoplastic neuroepithelial tumors(DNT). Methods:Clinical and radiological data bases of 4 cases of DNT were retrospectively analyzed with light microscopic and immunohistochemical observation.Results: Refractory epilepsy is a typical clinical manifestation. It always attacks before 20 years old. Most lesions are located in supertentorial space ? There was no periturnoral ederna or space occupying effect on radiologic examination.DNT had obvious mucoid and microcystic degeneration.DNT consisted of the oligodendrocyteJike cells(OLC),neurons and astrocytes? 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? Conclusion: DNT is a surgically curable benign tumor.radiotherapy and chemotherapy are contraindicated.A definite diagnosis of DNT may be made by the clinical manifestations,radiological and histological examination’and immunohistochemical staining. [Key words] dysembryoplastic neuroepithelial tumors ; Clinicopathology; Diagnosic ; Imm un ohistochemistry 胚胎发育不良性神经上皮肿瘤 (dysembryoplastic neuroepithelial tumors, DNT)是一 种神经上皮起源肿瘤，很少见，由Daumas-Duport等 [1]于1988年首先描述并命名,1993年才收入WHO 神经系统分类。光镜下与少突细胞瘤不易区别，两者 预后及治疗方案不同，DNT预后良好，术后不需放疗 或化疗。现收集4例进行回顾性分析，并结合