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.
髓母细胞瘤（MB）的发育基因表达数据表明，WNT亚型MB起源于胚胎期下菱唇区域，而SHH亚型MB及非WNT/非SHH亚型MB则源于小脑前体基质区域。本研究旨在通过术中详细数据对肿瘤起源部位进行分析，并将这些发现与分子亚型相关的假设起源区域进行比较。通过对机构数据库的回顾性分析，在1996年至2020年间于我院接受手术治疗的72例儿童MB患者中，筛选出58例具备详细手术记录、手术视频以及可用于分析的临床与遗传学分型数据的病例。肿瘤起源部位依据术中观察结果进行评估。基于术中确定的起源部位，对分子亚型的“正确”预测率分别为：WNT-MB 20%、SHH-MB 60%、非WNT/非SHH-MB 71%。神经外科术中探查对分子亚型判定的阳性预测值为：WNT-MB 0.21（95% CI 0.08–0.48）、SHH-MB 0.86（95% CI 0.49–0.97）、非WNT/非SHH-MB 0.73（95% CI 0.57–0.85）。本研究表明，术中观察到的肿瘤起源部位对髓母细胞瘤分子亚型的预测价值有限。
The Site of Origin of Medulloblastoma: Surgical Observations Correlated to Molecular Groups
肿瘤(癌症)患者之家