Shakur Sophia F, McGirt Matthew J, Johnson Michael W, Burger Peter C, Ahn Edward, Carson Benjamin S, Jallo George I
Departments of Neurosurgery John Hopkins University School of Medicine, Baltimore, Maryland, USA.
J Neurosurg Pediatr. 2009 Mar;3(3):197-202. doi: 10.3171/2008.11.PEDS0858.
Angiocentric glioma was recently recognized as a distinct clinicopathological entity in the 2007 World Health Organization Classification of Tumours of the Central Nervous System. The authors present the first 3 pediatric cases of angiocentric glioma encountered at their institution and review the literature of reported cases to elucidate the characteristics and outcomes of pediatric patients with this novel tumor.
The children in the 3 cases of angiocentric glioma were 10, 10, and 13 years old. Two presented with intractable seizures and 1 with worsening headache and several months of decreasing visual acuity. Twenty-five cases, including the 3 first described in the present paper, were culled from the literature.
In all 3 cases, MR imaging demonstrated a superficial, nonenhancing, T2-hyperintense lesion in the left temporal lobe. Histologically, the tumors were composed of monomorphous cells with a strikingly perivascular orientation that were variably reactive for glial fibrillary acidic protein and epithelial membrane antigen. Surgical treatment resulted in gross-total resection in all 3 cases. By 24, 9, and 6 months after surgery, all 3 patients remained seizure free without focal neurological deficits.
Among 25 cases of angiocentric glioma, seizure was the most common symptom at presentation. Magnetic resonance imaging demonstrated supratentorial, nonenhancing, T1-hypointense, T2-hyperintense lesions. Gross-total resection of this lesion yields excellent results.
血管中心性胶质瘤最近在2007年世界卫生组织中枢神经系统肿瘤分类中被确认为一种独特的临床病理实体。作者报告了他们机构遇到的首例3例儿童血管中心性胶质瘤病例，并回顾已报道病例的文献，以阐明患有这种新型肿瘤的儿童患者的特征和预后。
3例血管中心性胶质瘤患儿年龄分别为10岁、10岁和13岁。2例表现为顽固性癫痫发作，1例表现为头痛加重及数月来视力下降。从文献中筛选出25例病例，包括本文首次描述的3例。
所有3例病例的磁共振成像均显示左颞叶有一个表浅的、无强化的T2高信号病变。组织学上，肿瘤由单形性细胞组成，具有明显的血管周围排列，对胶质纤维酸性蛋白和上皮膜抗原呈不同程度的反应。手术治疗使所有3例病例均实现了大体全切。术后24个月、9个月和6个月时，所有3例患者均无癫痫发作，也无局灶性神经功能缺损。
在25例血管中心性胶质瘤病例中，癫痫发作是最常见的首发症状。磁共振成像显示幕上、无强化、T1低信号、T2高信号病变。该病变的大体全切效果良好。
