Gonzalez-Quarante Lain Hermes, Fernández Carballal Carlos, Agarwal Vijay, Vargas Lopez Antonio J, Gil de Sagredo Del Corral Oscar Lucas, Sola Vendrell Emma
Department of Neurosurgery, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
Department of Neurosurgery, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
World Neurosurg. 2017 Jan;97:755.e5-755.e10. doi: 10.1016/j.wneu.2016.10.034. Epub 2016 Oct 15.
Angiocentric glioma is a very uncommon low-grade tumor, predominantly occurring in pediatric patients, that was first described in 2005 and was codified 2 years later as a new central nervous system primary tumor. We herein report an exceptionally rare case of an elderly patient with angiocentric glioma. Only one additional case of angiocentric glioma in a patient older than 65 years has been hitherto reported.
An 83-year-old male patient presented at our institution complaining of a 1-month history of progressive weakness of his right hand and difficulty performing fine movements. Magnetic resonance imaging of the brain was performed, and fluid-attenuated inversion recovery and T2-hyperintense diffuse cortico-subcortical lesion were reported. A neuronavigation-guided frontal craniotomy was performed to expose the premotor cortex, motor cortex, Rolandic sulcus, and postcentral gyrus. Intraoperative mapping showed that the tumor was close to the shoulder area. Therefore, only partial resection was safely feasible. Pathology report described astrocytic neoplastic cells affecting mainly the cortex and piamater with the classic finding of subpial palisading, with no endothelial invasion or atypia. Neoplastic cells were positive for glial fibrillary acidic protein, epithelial membrane antigen, Wilms tumor protein-1, P16, and P53. Low proliferative activity was seen (Ki-67 < 2%). Abundant gliovascular structures were also reported.
Considering the morphologic and immunohistochemical data, the final pathologic diagnosis was angiocentric glioma. Furthermore, a thorough review of the literature was performed with the purpose of updating and summarizing the main clinical, radiologic, and pathologic features of this rare tumor.
血管中心性胶质瘤是一种非常罕见的低级别肿瘤,主要发生于儿童患者,于2005年首次被描述,并于两年后被编入新的中枢神经系统原发性肿瘤。我们在此报告一例极为罕见的老年血管中心性胶质瘤患者。迄今为止,仅报道过一例65岁以上的血管中心性胶质瘤患者。
一名83岁男性患者因右手进行性无力1个月及精细动作困难前来我院就诊。进行了脑部磁共振成像检查,报告显示有液体衰减反转恢复序列及T2高信号弥漫性皮质-皮质下病变。在神经导航引导下行额部开颅术,以暴露运动前皮质、运动皮质、中央沟和中央后回。术中定位显示肿瘤靠近肩部区域。因此,仅能安全地进行部分切除。病理报告描述星形细胞瘤性细胞主要累及皮质和软脑膜,具有软膜下栅栏状排列的典型表现,无内皮侵犯或异型性。肿瘤细胞胶质纤维酸性蛋白、上皮膜抗原、肾母细胞瘤蛋白-1、P16和P53呈阳性。增殖活性较低(Ki-67<2%)。还报告有丰富的胶质血管结构。
综合形态学和免疫组化数据,最终病理诊断为血管中心性胶质瘤。此外,为更新和总结这种罕见肿瘤的主要临床、影像学和病理特征,对文献进行了全面回顾。