Histopathological study of five cases with sporadic meningioangiomatosis
Histopathological study of five cases with sporadic meningioangiomatosis
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DOI：
10.1111/j.1440-1789.2006.00668.x
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发表时间：
2006-06-01
期刊：
影响因子：
2.3
通讯作者：
Zhang, FL
中科院分区：
文献类型：
--
作者：
Wang, Y;Gao, X;Zhang, FL
我们报告五例散发性脑膜血管瘤病，三名男性和两名女性，年龄从12岁到36岁不等的诊断。在1例病例中，在头部创伤后通过MRI偶然发现病变;其他4例受试者有癫痫发作障碍，在手术切除皮质病变后得到改善。大体上，病变切除标本呈发白颜色，质地坚硬。组织学检查显示病变局限于皮质，上覆软脑膜局灶性受累，并显示脑膜血管瘤病的统一特征，如皮质内微血管增生伴血管周围梭形细胞增生。2例病例有大量钙化; 1例与突出的纤维钙化成分相关。免疫组化结果在不同的病例中是不同的。只有波形蛋白始终呈阳性。2例梭形细胞EMA弱阳性。免疫反应与抗CD 34检测梭形细胞的细胞质内观察到的5例中的3例。所有病例的Ki-67增殖指数都很低，小于0.1%。使用Bodian和免疫染色方法，仅在五例病例中的一例中发现了神经原纤维缠结。这些发现表明脑膜血管瘤病病变表现出广泛的临床病理特征，使诊断困难。结合文献讨论了其组织病理学谱和鉴别诊断。由于这种病变是一个独特的临床病理实体和错构瘤的性质，它是重要的是作出正确的诊断，以避免进一步的积极治疗。
We report five cases of sporadic meningioangiomatosis, three males and two females, ranging in age from 12 to 36 years at diagnosis. The lesion was found incidentally by MRI after a head trauma in one case; the other four subjects had a seizure disorders, which improved following surgical resection of the cortical lesions. Grossly, the lesionectomy specimens were of a whitish color and firm consistency. Histological examination revealed that the lesions were confined to the cortex with focal involvement of the overlying leptomeninges, and revealed unifying features of meningioangiomatosis, such as proliferating microvessels with perivascular cuffs of spindle-cell proliferation within the cortex. Two cases had numerous calcifications; one was associated with a prominent fibrocalcifying component. Immunostaining results were variable among the cases. Only vimentin was consistently positive. Some of the spindle cells were weak positive for EMA in two cases. Immunoreactions with anti-CD34 detected within the cytoplasm of the spindle cells were observed in three of the five cases. The Ki-67 proliferation index of all the cases was very low, less than 0.1%. Neurofibrillary tangles were identified in only one of the five cases using the Bodian and immunostaining methods. These findings indicate that meningioangiomatosis lesions show a wide range of clinicopathological features, making diagnosis difficult. A histopathological spectrum and differential diagnoses were discussed with a review of the literature. Since this lesion is a distinct clinicopathological entity and hamartomatous in nature, it is important to make a correct diagnosis in order to avoid further aggressive treatment.