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小儿WNT型髓母细胞瘤术中失血倾向:一项回顾性观察队列研究

Pediatric WNT medulloblastoma predisposition in intraoperative blood loss: a retrospective observational cohort study.

作者信息

Zhang Zaiyu, Wu Yuxin, Zhao Xueling, Ji Wenyuan, Li Lusheng, Zhai Xuan, Liang Ping, Cheng Yuan, Zhou Jianjun

机构信息

Department of Neurosurgery, Children's Hospital of Chongqing Medical University, Chongqing, China.

National Clinical Research Center for Child Health and Disorders, Chongqing, China.

出版信息

Front Neurol. 2024 Jul 2;15:1386121. doi: 10.3389/fneur.2024.1386121. eCollection 2024.

DOI:10.3389/fneur.2024.1386121
PMID:39015321
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11249760/
Abstract

INTRODUCTION

Molecular subgroups influence the vascular architecture within medulloblastomas, particularly the wingless (WNT) subgroup, which contributes to its propensity for primary tumor hemorrhage. Whether this mechanism affects intraoperative blood loss remains unknown. This study aimed to assess the association between WNT medulloblastoma and the predisposition for blood loss.

METHODS

This was a retrospective observational study using data from a neuro-oncology center comprising molecular data on patients treated between December 31, 2014, and April 30, 2023. Differences between WNT and other subgroups in the risk of primary outcome-intraoperative blood loss were assessed using multivariable-adjusted linear regression.

RESULTS

Of the 148 patients included in the analysis, 18 patients (12.2%) had WNT, 42 (28.4%) had sonic hedgehog (SHH) -wildtype, 7 (4.7%) had SHH -mutant, and 81 (54.7%) were non-WNT/ non-SHH. The WNT subgroup more frequently underwent primary intratumoral hemorrhage (22% vs. 3.8%; = 0.011). The median intraoperative blood loss was 400.00 (interquartile range [IQR] 250, 500) mL for WNT and 300.00 [200, 400] mL for the other subgroups (p = 0.136), with an adjusted β of 135.264 (95% confidence intervals [CI], 11.701-258.827; = 0.032). Similar results were observed in both midline and noninfiltrative margin medulloblastoma.

DISCUSSION

WNT medulloblastoma is typically associated with primary intratumoral hemorrhage and intraoperative blood loss. The validity of determining the surgical approach based on predicted molecular subtypes from imaging data is questionable. However, attempting to engage in risk communication with patients in a molecular-specific way is worthwhile to validate.

摘要

引言

分子亚群会影响髓母细胞瘤内部的血管结构,尤其是无翅型(WNT)亚群,这导致其原发性肿瘤出血倾向增加。这种机制是否会影响术中失血尚不清楚。本研究旨在评估WNT髓母细胞瘤与失血易感性之间的关联。

方法

这是一项回顾性观察研究,使用了一家神经肿瘤中心的数据,这些数据包含2014年12月31日至2023年4月30日期间接受治疗患者的分子数据。使用多变量调整线性回归评估WNT亚群与其他亚群在主要结局(术中失血)风险上的差异。

结果

纳入分析的148例患者中,18例(12.2%)为WNT型,42例(28.4%)为音猬因子(SHH)野生型,7例(4.7%)为SHH突变型,81例(54.7%)为非WNT/非SHH型。WNT亚群原发性肿瘤内出血更为常见(22%对3.8%;P = 0.011)。WNT亚群术中失血量中位数为400.00(四分位间距[IQR]250,500)mL,其他亚群为300.00[200,400]mL(P = 0.136),调整后的β为135.26(95%置信区间[CI],11.701 - 258.827;P = 0.032)。在中线和非浸润边缘髓母细胞瘤中均观察到类似结果。

讨论

WNT髓母细胞瘤通常与原发性肿瘤内出血和术中失血相关。基于影像学数据预测的分子亚型来确定手术方式的有效性值得怀疑。然而,尝试以分子特异性方式与患者进行风险沟通是值得验证的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6db/11249760/8779fcb3793b/fneur-15-1386121-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6db/11249760/ed025618b916/fneur-15-1386121-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6db/11249760/8779fcb3793b/fneur-15-1386121-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6db/11249760/ed025618b916/fneur-15-1386121-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e6db/11249760/8779fcb3793b/fneur-15-1386121-g002.jpg

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