Qi X L, Yao K, Duan Z J, Bian Y, Ma Z, Piao Y S, Gong L P
Department of Pathology, Capital Medical University, Beijing 100010, China.
Zhonghua Bing Li Xue Za Zhi. 2018 Sep 8;47(9):664-670. doi: 10.3760/cma.j.issn.0529-5807.2018.09.003.
To investigate the clinicopathologic characteristics and BRAF V600E mutation of brain tumors associated with epilepsy. Totally 250 patients with brain tumors associated with epilepsy were included from March 2008 to August 2017 retrospectively at Sanbo Brain Hospital, Capital Medical University.The clinical manifestations, histological features and BRAF V600E mutation results were collected and analyzed. There were 132 males and 118 females, and the male to female ratio was 1.1∶1.0. The age of patients ranged from 2 to 67 years(mean 22 years). The tumors had obvious local space occupying effect on MRI. The temporal lobe was the most common site (44.4%, 111/250). There were 58.4% (146/250) of ganglioglioma (GG), 24.0% (60/250) of dysembryoplastic neuroepithelial tumor (DNT), 12.8% (32/250) of pleomorphic xanthoastrocytoma(PXA), 4.0% (10/250) of angiocentric glioma (AG) and 0.8% (2/250) of papillary glioneuronal tumor (PGNT). Mixed GG, PXA and DNT morphological structures were found in 9 of patients. Among 250 cases, 35 cases were accompanied by focal cortical dysplasia(FCD). BRAF V600E was seen in 43 of 74 (58.1%) GG and 13 of 28 (46.4%) PXA. The most common pathologic grade of GG, DNT, AG and PGNT was WHO I. Some of the tumor cells from GG (34 cases) showed higher proliferative activity (WHO Ⅱ/Ⅲ). Most cases of PXA were WHOⅡand high proliferative activity was seen in nine cases. The association of low-grade glioneuronal tumors with intractable epilepsy was well-recognized. The most common low-grade glioneuronal tumors were GG.GG may occur in any part of the central nervous system, with a predilection for temporal lobe. Each type of low-grade glioneuronal tumors has its own unique histological morphology, but some may show complex features with 2 or 3 mixed components. The occurrence of BRAF V600E mutations in GG is common, and their detection may be valuable for the diagnosis and treatment in GG.
探讨与癫痫相关脑肿瘤的临床病理特征及BRAF V600E突变情况。回顾性纳入2008年3月至2017年8月首都医科大学三博脑科医院收治的250例与癫痫相关的脑肿瘤患者。收集并分析其临床表现、组织学特征及BRAF V600E突变结果。患者中男性132例，女性118例，男女比例为1.1∶1.0。患者年龄范围为2至67岁（平均22岁）。肿瘤在MRI上有明显的局部占位效应。颞叶是最常见的部位（44.4%，111/250）。神经节细胞胶质瘤（GG）占58.4%（146/250），胚胎发育不良性神经上皮肿瘤（DNT）占24.0%（60/250），多形性黄色星形细胞瘤（PXA）占12.8%（32/250），血管中心性胶质瘤（AG）占4.0%（10/250），乳头状胶质神经元肿瘤（PGNT）占
