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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 clinicopathological features, immunophenotypes and imaging findings of nasal glioma and meningoencephalitis in order to explore its diagnosis and differential diagnosis. Methods Two cases of gliomas and nine cases of encephalocele were analyzed by HE and immunohistochemistry, and the related literatures were reviewed. Results Both of the two cases of gliacytes were female infants, aged <2 years, showing nasopharyngeal tumor. There were airway obstruction symptoms at birth, and no skull base bone defect was found in the imaging. 9 cases of nasal meningeal encephalocele, 4 males and 5 females, mean age 25 years old; 8 cases of lesions located in the nasal cavity, 5 cases on the right, 2 cases on the left and 1 case on both sides; Symptoms; another case of tumor located in the back of the nose, partially into the left nasal cavity and nasal vestibule. All 9 cases had skull base defect or discontinuous bone imaging. Giant tumor: no glial glioma capsule, nodular, smooth surface, grayish pink, hard quality, solid section, some areas with cystic cavity formation; meningoencephalocele was gray, non-shaped soft tissue, no tissue Envelope. Microscopic examination: Glioblastoma by varying sizes of glial islands and intertwined vascular fat fibrous tissue, neurons in some areas, choroid plexus, ependymal, pigmented retinal epithelium and pituitary-like structure. The presence of various tissues can be demonstrated by immunohistochemical staining of glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), S-100 protein and pituitary hormone antibodies. Meningeal bulging under a single morphology, mainly in the meninges and glia, a few cases (3/9) have neurons. Conclusions Glioblastoma is a rare congenital disease. The key to the differential diagnosis of nasal encephalocele is the combination of pathology and imaging.