Sturge-Weber综合征4例颅内病变临床病理分析

发布时间：2018-03-03 13:21

本文选题：Sturge-Weber综合征　切入点：临床病理　出处：《诊断病理学杂志》2014年03期 　论文类型：期刊论文

【摘要】：目的探讨Sturge-Weber综合征(SWS)的临床和影像学表现以及病理形态特征。方法复习4例SWS患者的临床及影像学资料,镜下观察其组织学改变,并复习相关文献。结果男、女性患儿各2例,年龄5~8岁;其中2例面部见葡萄酒色痣,颅内病变累及左顶枕叶2例,左额颞叶及左颞叶各1例。影像学表现为软脑膜表面混杂密度影,增强后明显脑回状强化,伴皮层内条索状或锯齿状钙化。组织学特点:①3例蛛网膜下—软脑膜区大量扩张充血的的薄壁血管,呈海绵状血管瘤样改变;1例表现为静脉型血管瘤;②病变皮层下沿脑回带状分布的多发钙化灶;③皮层内神经元减少,胶质细胞增生,3例伴周围皮层发育不良;④免疫组化染色示皮层内神经元Neu-N(+),增生的胶质细胞GFAP和S-100(+)。结论 Sturge-Weber综合征少见,其正确诊断需结合临床和影像学以及病理形态综合分析。面部血管痣及皮层内沿脑回分布的钙化灶是诊断的重要线索。
[Abstract]:Objective to investigate the clinical and imaging features and histopathological features of Sturge-Weber syndrome. Methods the clinical and imaging data of 4 patients with SWS were reviewed. The age was 58 years, of which 2 cases had wine nevus on face, 2 cases had intracranial lesions involving left parietal occipital lobe, 1 case had left frontotemporal lobe and 1 case left temporal lobe. Histological features: 13 cases of subarachnoid pial area dilated and congested parenchyma vessels, Cavernous hemangiomatous changes were found in 1 case with subcortical lesion of venous hemangioma and decreased neurons in the cortex with multiple calcified foci distributed along the gyrus. The immunohistochemical staining of 3 cases of glial hyperplasia with hypoplasia of the pericortical cortex showed that Neu-Ns, proliferative glial cell GFAP and S-100 were rare in the cortex. Conclusion Sturge-Weber syndrome is rare. The correct diagnosis should be combined with clinical, imaging and histopathological analysis. The calcified foci distributed along the gyrus of the facial vascular nevus and cortex are important clues for diagnosis.
【作者单位】：广州军区广州总医院病理科;广州军区广州总医院神经外科;
【基金】：广东省医学科研基金:WSTJJ20121110412822197806052224
【分类号】：R758.6

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