Li Tiemin, Aihemaitiniyazi Adilijiang, Zhang Huawei, Wei Da, Hu Yue, Guan Yuguang, Zhou Jian, Qi Xueling, Wang Mengyang, Wu Bin, Zhu Mingwang, Zhang Linpeng, Luan Guoming, Liu Changqing
Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, No. 50, Yikesong, Xiangshan, Haidian District, Beijing, 100093, China.
Department of Neurosurgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, 100020, China.
Neurol Sci. 2025 Jan;46(1):427-436. doi: 10.1007/s10072-024-07721-3. Epub 2024 Aug 5.
Angiocentric glioma (AG), a benign tumor identified within the last two decades, was officially included in the 2007 WHO Classification of Tumors of the Central Nervous System, WHO grade I. The tumor is relatively rare, with only approximately 100 cases reported. We aim to complement the characteristics and long-term prognosis of AG, as well as to detect MYB-QKI fusions.
The characteristics of all cases collected between 1 March 2009 and 1 March 2023 at the Beijing Sanbo Brain Hospital, Capital Medical University, were summarized and analyzed. Additionally, all fourteen patients were tested for MYB-QKI fusions.
AG more predominantly occurs in adolescents (median age 16.5-year-old), and commonly presents with drug-resistant epilepsy. AG is frequently localized in the supratentorial regions and only one patient is in the brainstem. Brain parenchyma atrophy, and stalk-like signs can observe in imaging. Pathologically, tumor cells are perivascular pseudorosettes, presenting immunoreactivity for GFAP, S-100, Vimentin, "dot-like" staining for EMA, and low proliferative activity. Focal cortex dysplasia was observed in four patients. Twelve of fourteen (85.7%) patients were found with MYB-QKI fusions. Completely surgical resection typically has a satisfactory prognosis with long-term follow-up.
AG is a rare benign tumor with a favorable prognosis after complete resection, characterized by refractory epilepsy, frequently occurring in adolescents. MYB-QKI fusions were detected in most AG patients, as a good defining genetic alteration pathologically. The potential presence of focal cortical dysplasia (FCD) may affect the prognosis of epilepsy.
血管中心性胶质瘤（AG）是过去二十年内发现的一种良性肿瘤，于2007年被正式纳入世界卫生组织（WHO）中枢神经系统肿瘤分类，为WHO I级。该肿瘤相对罕见，仅报道了约100例病例。我们旨在补充AG的特征和长期预后情况，并检测MYB-QKI融合。
总结并分析了2009年3月1日至2023年3月1日期间在首都医科大学附属北京三博脑科医院收集的所有病例的特征。此外，对所有14例患者进行了MYB-QKI融合检测。
AG多发生于青少年（中位年龄16.5岁），常见表现为耐药性癫痫。AG常位于幕上区域，仅1例位于脑干。影像学上可观察到脑实质萎缩和茎状征。病理上，肿瘤细胞呈血管周围假菊形团，对GFAP、S-100、波形蛋白呈免疫反应，对EMA呈“点状”染色，增殖活性低。4例患者观察到局灶性皮质发育异常。14例患者中有12例（85.7%）检测到MYB-QKI融合。完全手术切除后长期随访通常预后良好。
AG是一种罕见的良性肿瘤，完全切除后预后良好，以难治性癫痫为特征，常见于青少年。大多数AG患者检测到MYB-QKI融合，是一种良好的病理定义性基因改变。局灶性皮质发育异常（FCD）的潜在存在可能影响癫痫的预后。
