摘要
目的探讨血管中心性胶质瘤(AG)临床病理学特征及预后。方法收集血管中心性胶质瘤2例,常规石蜡切片,行HE染色、免疫组化标记及电镜检测。结果目前国内外文献报道的AG共36例,本文报道2例。AG以儿童和年轻人多发,长期难治性癫是其主要表现形式,常累及额顶叶、颞叶皮质浅层。MRI检查显示实性占位。形态学上表现为单一性双极梭形肿瘤细胞围绕血管中心生长,平行或垂直于血管分布。肿瘤细胞常GFAP、vimentin、S-100和EMA(+),Syn、CgA和p53(-),增殖指数Ki-67≤1%。电镜检测肿瘤细胞呈星形细胞样分化,偶见发育不好的细胞连接,未见微绒毛及腔隙样结构。外科手术切除则癫症状消失,分别随访14个月和3个月MRI显示均无复发。结论 AG为一类新提出的神经系统肿瘤,因其临床以难治性癫为主要表现形式,临床表现和MRI又存在多样性,故诊断主要依赖于病理学。该肿瘤典型的形态学特征、免疫组化及电镜表现均提示其存在星形细胞和室管膜细胞分化,需要与多种常见的神经系统肿瘤进行鉴别。AG治疗以手术切除为主,临床愈后佳。
Objective To investigate the clinicopathological changes and prognosis of angiocentric glioma(AG).Methods Two cases were collected,which were sent for morphologic evaluation using H&E(Hematoxylin & Eosin)-stained sections,immunophenotypic detection by immunohistochemistry(IHC) and electron microscopy(EM).Results To date only 36 cases had been reported in the literature.We reported two additional cases.It often occurred in children and young adult.The medically intractable seizures were its common symptom.It arose superficially in the cortex of frontoparietal lobe and temporal lobe.Magnetic resonance imaging(MRI) revealed masses.Histological evaluation of the lesions revealed an angiocentric growth pattern with monomorphous bipolar tumor cells,longitudinal and/or circumferential orientations of perivascular cells.Tumor cells showed immunoreactivities for glial fibrillary acidic protein(GFAP),vimentin,S-100 protein and epithelial membrane antigen(EMA),but not for synaptophysin(Syn),chromogranin(CgA) and P53.Ki-67 labelling index was 1%.Tumor cells showed astrocytic features when detected by EM,but there were no microvillus and lacunas.The tumors were treated by surgical excision,and the two cases were now seizure-free on monotherapy,and had been followed with MRI at 14 months and 3 months,respectively,with no radiological evidence of tumor recurrence.Conclusions AG is recently defined as a new brain tumor type of the central nervous system.The intractable seizures show its common symptom with various clinical and MRI changes,and its diagnosis is basically by histological examination.It has typical morphology.IHC and EM demonstrates astrocytic and ependymal features,so that it needs to be distinguished from other common central nervous system tumors.Surgical treatment is the primary treatment,with good prognosis.
出处
《诊断病理学杂志》
CSCD
北大核心
2012年第4期266-269,共4页
Chinese Journal of Diagnostic Pathology