摘要
目的探讨脑膜血管瘤病的临床病理学特点。方法对5例脑膜血管瘤病手术切除标本，应用HE染色和免疫组织化学染色进行组织形态学观察和临床病理分析，并结合国内文献报道分析该病的临床、病理特点及生物学行为。结果本组共5例患者，男3例，女2例。年龄16～26岁，均以癫痫起病，药物治疗效果欠佳。病史最长者18年，最短者8个月余。本组5例中，4例病变区位于额叶，1例位于颞叶；1例合并局灶性皮层发育不良，1例合并脂肪瘤，1例合并血管瘤。手术见病变区位于皮层内，质稍硬，血供一般，无包膜。镜下改变大致相同，显示病灶区脑皮层内梭形细胞围绕增生的小血管鞘周呈旋转式及旋涡状排列，部分见沙砾体形成，部分可见嗜酸性颗粒小体。病灶间可见正常的神经元。肿瘤细胞表达上皮细胞膜抗原蛋白及波形蛋白，网织染色显示阳性。结论脑膜血管瘤病是一罕见的颅内良性增生性瘤样病变，主要位于大脑皮层，以额叶、颞叶为多见，病理组织特点主要为脑膜上皮细胞、纤维母细胞、血管周纤维细胞在脑实质内血管鞘周呈旋转式生长，伴血管增生及钙化，常为儿童及青少年发病，绝大多数的散发型患者有长期、难治性癫痫病史，可伴有血管畸形、脑膜瘤等其他肿瘤病变。
Objective To investigate the clinicopathologic characteristics of meningioangiomatosis (MA). Methods Five cases of MA were evaluated morphologically by HE and immunohistochemistry on formalin-fixed paraffin-embedded tissue. Clinical information was also obtained. The literature was reviewed. The clinical pathology and biological behavior of MA were discussed. Results Five cases of MA were reported, arising in three males and two females, with an age range of 16 to 26 years at diagnosis. All five subjects had intractable seizure disorders, and the duration of illness ranged from 8 months to 18 years. The lesions were resected from the frontal lobe in four patients, and from the temporal lobe in one. All the lesions were confined to the cortex, firm in consistency, without capsules and had poor blood supply. There was focal involvement of the overlying leptomeninges. Microscopically, they showed characteristic features of MA, such as proliferating microvessels with perivascular cuffs of spindle-cell within the cortex. Some had numerous calcifications, others showed acidophilie granular bodies. The cells were positive for EMA and vimentin by immunohistoehemistry, and for reticulin by histochemical staining. Conclusions MA is a rare, benign hamartomatous lesion of the central nervous system. It usually presents as plaque-like or nodular mass in the cerebral cortex and the overlying leptomeninges, consisting of meningovascular proliferation and leptomeningeal calcification. In some cases the lesion may show perivascular proliferation of elongated spindle-shaped cells. MA usually affects children and young adults, and is located in the frontal or temporal lobes with variable involvement of the overlying leptomeninges. Clinically, most of sporadic cases have a long history of intractable seizures despite multiantiepileptic drugs. MA has also been reported to coexist with arteriovenous malformations, meningiomas and other tumorous lesions.
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2015年第5期310-314,共5页
Chinese Journal of Pathology
基金
解放军总医院临床科研扶持基金(2013FC-TSYS-3011)
关键词
脑膜
血管瘤病
癫痫
Meninges
Angiomatosis
Epilepsy
