未经证实:弥漫性软脑膜神经胶质神经元肿瘤是由少突胶质细胞样细胞组成的罕见软脑膜肿瘤,其特征是神经元分化和缺乏异柠檬酸脱氢酶基因突变。
未经证实:我们旨在分析临床进展,病理特征,儿童弥漫性软脑膜神经胶质瘤的放射学发现,以及临床放射学数据的相关性。
未经批准:我们搜索了MEDLINE,PubMed,和WebofScience来确定病例报告,原创文章,并回顾了2000年至2021年间发表的讨论弥漫性软脑膜神经胶质瘤的文章。
UNASSIGNED:该分析包括来自43项先前研究的145名儿科患者。
未经证实:有关患者病理的数据,MRI表现,临床症状,和进展被收集。影像学分类与病理结果之间的关系使用卡方检验。使用Kaplan-Meier曲线分析总生存期。
UNASSIGNED:实质肿瘤主要位于颈椎和胸椎的髓内区域,以及此类肿瘤易于发生1p缺失(χ2=4.77,p=0.03)和KIAA1549-BRAF融合(χ2=12.17,p<0.001)的患者。中位生存时间为173个月,而生存曲线在72个月前显著下降。实质肿瘤位置与总生存期相关(p=0.03),接受KIAA1549-BRAF(+)治疗并接受化疗的患者临床病程较好(p<0.001).
UNASSIGNED:由于弥漫性软脑膜神经胶质瘤的罕见性,该分析包括病例报告而不是连续治疗的患者,这可能引入了偏见。
未经批准:临床早期整合,病态,放射学检查结果对于适当管理这种肿瘤是必要的,因为这可能使早期治疗和改善预后。
UNASSIGNED: Diffuse leptomeningeal glioneuronal tumors are rare leptomeningeal neoplasms composed of oligodendrocyte-like cells characterized by neuronal differentiation and a lack of isocitrate dehydrogenase gene mutation.
UNASSIGNED: We aimed to analyze the clinical progression, pathological characteristics, and radiological findings of diffuse leptomeningeal glioneuronal tumors in children, as well as the relevance of clinico-radiological data.
UNASSIGNED: We searched MEDLINE, PubMed, and Web of Science to identify case reports, original articles, and review articles discussing diffuse leptomeningeal glioneuronal tumors published between 2000 and 2021.
UNASSIGNED: The analysis included 145 pediatric patients from 43 previous studies.
UNASSIGNED: Data regarding patient pathology, MRI manifestations, clinical symptoms, and progression were collected. The relationship between imaging classification and pathological findings was using chi-square tests. Overall survival was analyzed using Kaplan-Meier curves.
UNASSIGNED: Parenchymal tumors were mainly located in the intramedullary areas of the cervical and thoracic spine, and patients which such tumors were prone to 1p-deletion (χ2 = 4.77, p=0.03) and KIAA1549-BRAF fusion (χ2 = 12.17, p<0.001). The median survival time was 173 months, and the survival curve fell significantly before 72 months. Parenchymal tumor location was associated with overall survival (p=0.03), patients with KIAA 1549-BRAF (+) and treated with chemotherapy exhibited a better clinical course (p<0.001).
UNASSIGNED: The analysis included case reports rather than consecutively treated patients due to the rarity of diffuse leptomeningeal glioneuronal tumors, which may have introduced a bias.
UNASSIGNED: Early integration of clinical, pathological, and radiological findings is necessary for appropriate management of this tumor, as this may enable early treatment and improve prognosis.