胚胎发育不良性神经上皮肿瘤1例报道并文献复习  

Dysembryoplastic neuroepithelial tumor:Report of one case with literature review

作　　者：黄小梅[1] 张帆 江晓春 许金亮 庞金金 卢林明[1] HUANG Xiaomei;ZHANG Fan;JIANG Xiaochun;XU Jinliang;PANG Jinjin;LU Linming(Department of Pathology,Wannan Medical College,Wuhu 241002,China)

机构地区：[1]皖南医学院病理学教研室,安徽芜湖241002 [2]皖南医学院第一附属医院弋矶山医院病理科,安徽芜湖241001 [3]皖南医学院第一附属医院弋矶山医院神经外科,安徽芜湖241001 [4]皖南医学院临床医学院,安徽芜湖241002

出　　处：《皖南医学院学报》2022年第5期506-509,共4页Journal of Wannan Medical College

基　　金：活性生物大分子研究安徽省重点实验室自主研究课题(LAB202004);皖南医学院大学生科研资助金项目(WK2020S01);安徽省高等学校质量工程项目(S202010368107)。

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、诊断及鉴别诊断。方法:报道1例经会诊确诊为DNT患者,回顾性分析其临床资料、病理特征、影像学特点、治疗及预后,并复习相关文献。结果:患者,女性,17岁,临床表现为伴有意识障碍的全面性强直-阵挛发作入院。神经系统检查无阳性体征。影像学检查显示右侧顶叶占位,T1WI呈低信号,T2WI呈高信号,局灶结节样、环形不均匀强化,无占位效应和瘤周水肿。肿瘤组织由少突胶质细胞样细胞、星形细胞及少数神经元等组成,间质富含黏液样基质伴微囊形成,可见典型的胶质神经元结构,未见核分裂像及坏死,周围脑实质伴局灶性皮质发育不良,未检测到BRAF^(V600E)、IDH1/2基因突变及1p/19q杂合性共缺失。在神经导航辅助下手术切除肿瘤+致痫灶,术后患者恢复良好,随访30个月无复发和癫痫发作。结论:DNT是一种较少见的良性神经上皮肿瘤(WHOⅠ级),影像学及病理学正确诊断非常重要,外科手术治疗预后良好,无需放疗和化疗。Objective:To investigate the clinicopathologic and imaging features,diagnosis and differential diagnosis of dysembryoplastic neuroepithelial tumor(DNT).Methods:One case of DNT diagnosed by consultation was reported,and retrospectively analyzed concerning the clinical data,histopathological and imaging features,treatment and prognosis,with related literatures reviewed.Results:A 17-year-old female patient presenting with generalized tonic-clonic seizure with conscious disorder was admitted to our hospital.Neurological examination showed no positive signs.Imaging examination revealed right parietal lobe mass,low signal on T1-weighted images,high signal on T2-weighted images,focal nodular,and annular uneven enhancement,yet no mass effect and edema at the cerebral cortex.The tumor tissue was composed of oligodendrocyte-like cells,astrocytes and a few neurons.The stroma was rich in myxoid matrix with microcystic cavity formation.Specific glioneuronal elements were seen,whereas nuclear mitosis and necrosis were free.Peripheral parenchyma with focal cortical dysplasia was also observed.The BRAF^(V600E) mutation,dehydrogenase-1/isocitrate dehydrogenase-2 mutation and codeletion of 1p/19q were not detected.The tumor and epileptogenic foci were surgically removed with the aid of neuronavigation.The patient recovered well after surgery,with no relapse or seizure during 30 months of follow-up.Conclusion:DNT is a rare benign neuroepithelial neoplasm(WHO gradeⅠ).Accurate imaging and pathological diagnosis are very important.Surgical treatment can lead to better prognosis without radiotherapy or chemotherapy.

关 键 词：胚胎发育不良性神经上皮肿瘤 脑肿瘤 病理 诊断 

分 类 号：R739.4[医药卫生—肿瘤] R365[医药卫生—临床医学]