Abstract：
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
目标：血管中心胶质瘤（AG），在过去的二十年中发现的良性肿瘤，被正式列入2007年世界卫生组织中枢神经系统肿瘤分类，WHOⅠ级肿瘤比较少见,仅报告了约100例。我们的目的是补充AG的特点和长期预后，以及检测MYB-QKI融合。
方法：2009年3月1日至2023年3月1日在北京三博脑科医院收集的所有病例的特征，首都医科大学,进行了总结和分析。此外,所有14例患者均接受了MYB-QKI融合检测.
结果：AG主要发生在青少年（中位年龄16.5岁），通常表现为耐药性癫痫。AG通常位于幕上区域，只有一名患者位于脑干。脑实质萎缩,在成像中可以观察到类似茎的迹象。病理上,肿瘤细胞是血管周围的假花结，呈现对GFAP的免疫反应性，S-100Vimentin,EMA的“点状”染色，和低增殖活性。在四名患者中观察到局灶性皮质发育不良。发现14例患者中有12例（85.7％）患有MYB-QKI融合。通过长期随访，完全手术切除通常具有令人满意的预后。
结论：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，经常发生在青少年中。在大多数AG患者中检测到MYB-QKI融合，作为一个很好的定义遗传改变病理。潜在的局灶性皮质发育不良（FCD）的存在可能会影响癫痫的预后。
方法：2009年3月1日至2023年3月1日在北京三博脑科医院收集的所有病例的特征，首都医科大学,进行了总结和分析。此外,所有14例患者均接受了MYB-QKI融合检测.
结果：AG主要发生在青少年（中位年龄16.5岁），通常表现为耐药性癫痫。AG通常位于幕上区域，只有一名患者位于脑干。脑实质萎缩,在成像中可以观察到类似茎的迹象。病理上,肿瘤细胞是血管周围的假花结，呈现对GFAP的免疫反应性，S-100Vimentin,EMA的“点状”染色，和低增殖活性。在四名患者中观察到局灶性皮质发育不良。发现14例患者中有12例（85.7％）患有MYB-QKI融合。通过长期随访，完全手术切除通常具有令人满意的预后。
结论：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，经常发生在青少年中。在大多数AG患者中检测到MYB-QKI融合，作为一个很好的定义遗传改变病理。潜在的局灶性皮质发育不良（FCD）的存在可能会影响癫痫的预后。
