摘要
背景与目的:胚胎发育不良性神经上皮肿瘤(dysembryoplastic neuroepithelial tumor,DNT)是一种少见的良性混合性神经元-胶质肿瘤,探讨其临床病理学特征及鉴别要点。方法:回顾性分析苏州大学附属第一医院和苏州市立医院2009年3月—2021年1月经病理学检查确诊的6例DNT患者的资料,归纳总结其临床病理学特征、影像学特征及免疫表型,并进行随访。结果:患者以不同程度的肢体抽搐、癫痫样发作为主要症状。肿瘤位于颞叶4例,顶叶1例,额叶1例。磁共振成像(magnetic resonance imaging,MRI)主要表现为囊性病灶,其内可见分隔及“三角征”或T1低信号,T2高信号,周围水肿不明显。光镜下肿瘤由特征性的胶质神经元成分构成,间质黏液样变性,以少突胶质样细胞为主,可呈束状、巢状、微囊状分布,其间散在单个的神经元或增生的星形胶质细胞,局灶可见砂粒体样钙化。免疫组织化学检测显示,神经元NeuN(+)、Syn(+)、MAP2(+),少突胶质样细胞Olig-2(+)、S-100(+),增生的星形胶质细胞GFAP(+),P53野生型表达,Ki-67≤2%。6例患者均行手术治疗,术后未进行放疗或化疗。5例患者获得随访,其中1例于术后3年癫痫复发。结论:DNT是一种手术可治愈的肿瘤,联合临床表现、影像学检查及病理学检查可确诊,无需放疗和化疗。
Background and purpose:Dysembryoplastic neuroepithelial tumor(DNT)is a rare and benign mixed neuronal-glial tumor.This paper was to study the clinicopathological features and the key points of differential diagnosis of DNT.Methods:The data of 6 cases with DNT diagnosed by pathological examination in The First Affiliated Hospital of Soochow University and Suzhou Municipal Hospital from March 2009 to January 2021 were collected,the clinicopathological features,imaging characteristics and immunohistochemical phenotype were retrospectively analyzed,and the patients were followed up.Results:The main symptoms of the patients were limb convulsions and epilepsy.Four tumors were located in temporal lobe,1 in parietal lobe,and 1 in frontal lobe.On magnetic resonance imaging(MRI),tumors mainly showed cystic lesions with septum and“triangle sign”,T1-hypointensity and T2-hyperintensity.Peripheral edema was inconspicuous.The histopathological hallmarks were so called specific glioneuronal element with myxoid matrix,floating neurons or proliferative astrocytes scattering among oligodendrocyte-like cells,which distributed in bundles,nests,microcysts with focal calcification.Immunohistochemistry showed that scattered neurons expressed Syn,NeuN and MAP2,oligodendrocyte-like cells expressed Olig-2 and S-100,and GFAP was expressed in proliferative astrocytes.The expression of P53 was wild-type.The index of Ki-67 was less than or equal to 2%.All patients received surgical treatment without radiotherapy or chemotherapy.Five patients were followed up,and one of them had recurrent epilepsy 3 years after operation.Conclusion:DNT can be cured by surgery,and it can be diagnosed by combination of clinical features,imaging examination and pathology without the need for radiation and chemotherapy.
作者
何晓顺
焦伟娟
郭凌川
黄山
吴玉锦
黄仁鹏
HE Xiaoshun;JIAO Weijuan;GUO Lingchuan;HUANG Shan;WU Yujin;HUANG Renpeng(Department of Pathology,The First Affiliated Hospital of Soochow University,Suzhou 215006,Jiangsu Province,China;Department of Pathology,Suzhou Municipal Hospital,Suzhou 215008,Jiangsu Province,China;Department of Imaging,The First Affiliated Hospital of Soochow University,Suzhou 215006,Jiangsu Province,China)
出处
《中国癌症杂志》
CAS
CSCD
北大核心
2021年第9期822-827,共6页
China Oncology
