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髓母细胞瘤的起源部位:与分子亚群相关的手术观察

The Site of Origin of Medulloblastoma: Surgical Observations Correlated to Molecular Groups.

作者信息

Ciobanu-Caraus Olga, Czech Thomas, Peyrl Andreas, Haberler Christine, Kasprian Gregor, Furtner Julia, Kool Marcel, Sill Martin, Frischer Josa M, Cho Anna, Slavc Irene, Rössler Karl, Gojo Johannes, Dorfer Christian

机构信息

Department of Neurosurgery, Medical University of Vienna, 1090 Vienna, Austria.

Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, 1090 Vienna, Austria.

出版信息

Cancers (Basel). 2023 Oct 7;15(19):4877. doi: 10.3390/cancers15194877.

Abstract

Developmental gene expression data from medulloblastoma (MB) suggest that WNT-MB originates from the region of the embryonic lower rhombic lip (LRL), whereas SHH-MB and non-WNT/non-SHH MB arise from cerebellar precursor matrix regions. This study aimed to analyze detailed intraoperative data with regard to the site of origin (STO) and compare these findings with the hypothesized regions of origin associated with the molecular group. A review of the institutional database identified 58 out of 72 pediatric patients who were operated for an MB at our department between 1996 and 2020 that had a detailed operative report and a surgical video as well as clinical and genetic classification data available for analysis. The STO was assessed based on intraoperative findings. Using the intraoperatively defined STO, "correct" prediction of molecular groups was feasible in 20% of WNT-MB, 60% of SHH-MB and 71% of non-WNT/non-SHH MB. The positive predictive values of the neurosurgical inspection to detect the molecular group were 0.21 (95% CI 0.08-0.48) for WNT-MB, 0.86 (95% CI 0.49-0.97) for SHH-MB and 0.73 (95% CI 0.57-0.85) for non-WNT/non-SHH MB. The present study demonstrated a limited predictive value of the intraoperatively observed STO for the prediction of the molecular group of MB.

摘要

髓母细胞瘤(MB)的发育基因表达数据表明,WNT-MB起源于胚胎下菱形唇(LRL)区域,而SHH-MB和非WNT/非SHH MB则起源于小脑前体基质区域。本研究旨在分析关于起源部位(STO)的详细术中数据,并将这些发现与与分子组相关的假设起源区域进行比较。对机构数据库的回顾确定了1996年至2020年间在我们科室接受MB手术的72名儿科患者中的58名,这些患者有详细的手术报告、手术视频以及可供分析的临床和基因分类数据。根据术中发现评估STO。使用术中定义的STO,对分子组的“正确”预测在20%的WNT-MB、60%的SHH-MB和71%的非WNT/非SHH MB中是可行的。神经外科检查检测分子组的阳性预测值在WNT-MB中为0.21(95%CI 0.08-0.48),在SHH-MB中为0.86(95%CI 0.49-0.97),在非WNT/非SHH MB中为0.73(95%CI 0.57-0.85)。本研究表明,术中观察到的STO对MB分子组的预测价值有限。

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