鼻胶质瘤和鼻脑膜脑膨出的诊断与鉴别诊断  

Diagnosis and differential diagnosis of nasal glioma and meningeal encephalocele

作　　者：金玉兰[1] 田澄[1] 韩一丁[1] 刘红刚[1] 

机构地区：[1]首都医科大学附属北京同仁医院病理科,北京100730

出　　处：《诊断病理学杂志》2011年第1期16-19,共4页Chinese Journal of Diagnostic Pathology

基　　金：国家教委留学回国人员启动基金;北京同仁医院科研基金

摘　　要：目的通过对鼻胶质瘤和脑膜脑膨出的临床病理特征、免疫表型和影像学观察,探讨其诊断与鉴别诊断。方法通过HE和免疫组化染色分析2例鼻胶质瘤和9例脑膜脑膨出并复习相关文献。结果 2例鼻胶质瘤均为女婴,年龄<2岁,表现为鼻咽部肿物,自出生即有呼吸道阻塞症状,影像学未见颅底骨质缺损。鼻腔脑膜脑膨出9例,男性4例、女性5例,平均年龄25岁;其中8例病变位于鼻腔内,右侧5例,左侧2例,双侧1例;鼻腔清水样涕为主要症状;另1例肿物位于鼻背部,部分突入左鼻腔及鼻前庭。9例均有颅底骨质缺损或骨质不连续等影像学表现。巨检:鼻胶质瘤无包膜,呈结节状,表面光滑,灰粉色,质较硬,切面呈实性,部分区有囊腔形成;脑膜脑膨出呈灰白色不整形软组织,组织无包膜。镜检:鼻胶质瘤由大小不一的神经胶质岛和相互交错的血管脂肪纤维组织组成,部分区可见神经元、脉络丛、室管膜、色素性视网膜上皮及垂体样结构。可以用胶质纤维酸性蛋白(GFAP)、上皮膜抗原(EMA)、S-100蛋白及垂体激素抗体的免疫组化染色来证实各种组织的存在。脑膜脑膨出镜下形态较单一,主要以脑膜及神经胶质为主,少数病例(3/9)有神经元。结论鼻胶质瘤是罕见的先天性疾病,与鼻腔脑膜脑膨出的鉴别诊断关键在于结合病理形态与影像学观察。Objective To investigate the clinical and pathologic features,immunological phenotypes,imaging findings,and differential diagnosis of the nasal glioma and meningeal encephalocele.Methods H-E and immunohistochemical staining were performed in two cases of nasal glioma of nasopharynx and nine cases of nasal meningoencephalocele,and the related literatures were reviewed.Results Both two cases of nasal glioma were female and less than 2 years old,with symptoms of respiratory obstruction since birth.There was no defect on skull base bone on the imagining.The nine cases of nasal meningoencephalocele included four men and five women,with an average age of 25 years.Eight cases had the lesions in the nasal cavity,of which five was in right nose,three in left,and bilateral in one.Water-like nasal drop was the main symptom.The other one oringinated in nasal bridge,extending to left cavity and fossae.All of the nine cases had skull base bone defect or uncontinuous bone line on imaging.On gross examination,the nasal glioma was nodular or round with smooth surface but no capsule.The cut surface was solid,ash color,and harder consistency.The cavity formation could be seen.Meningoencephalocele was grey-white and irregular soft tissue without capsule.Microscopically,the nasal glioma consisted of glial islands of different size and crisscrossing angiolipofibrous tissue,with occasional neurons,choroid plexus,ependymal arranged fissures,retinal pigment epithelium and pituitary tissue,which could be confirmed by IHC of GFAP,S-100 protein and pituitary hormone antibodies.Meningoencephalocele had a simpler microscopic morphology and only a small number of cases（3/9） had neurons.Conclusion Nasal glioma is a rare congenital lesion and differential diagnosis from nasal meningoencephalocele mainly depends on the pathological features and imaging.

关 键 词：鼻胶质瘤 脑膜脑膨出 鼻咽部 形态学 

分 类 号：R730.264[医药卫生—肿瘤]