CAJ | 학술논문

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。
목적：탐토뇌막혈관류병적림상병이학특점。방법대5례뇌막혈관류병수술절제표본，응용HE염색화면역조직화학염색진행조직형태학관찰화림상병리분석，병결합국내문헌보도분석해병적림상、병리특점급생물학행위。결과본조공5례환자，남3례，녀2례。년령16～26세，균이전간기병，약물치료효과흠가。병사최장자18년，최단자8개월여。본조5례중，4례병변구위우액협，1례위우섭협；1례합병국조성피층발육불량，1례합병지방류，1례합병혈관류。수술견병변구위우피층내，질초경，혈공일반，무포막。경하개변대치상동，현시병조구뇌피층내사형세포위요증생적소혈관초주정선전식급선와상배렬，부분견사력체형성，부분가견기산성과립소체。병조간가견정상적신경원。종류세포표체상피세포막항원단백급파형단백，망직염색현시양성。결론뇌막혈관류병시일한견적로내량성증생성류양병변，주요위우대뇌피층，이액협、섭협위다견，병리조직특점주요위뇌막상피세포、섬유모세포、혈관주섬유세포재뇌실질내혈관초주정선전식생장，반혈관증생급개화，상위인동급청소년발병，절대다수적산발형환자유장기、난치성전간병사，가반유혈관기형、뇌막류등기타종류병변。
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 acidophilic granular bodies.The cells were positive for EMA and vimentin by immunohistochemistry, 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.