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小脑髓母细胞瘤患儿和青年患者的后颅窝综合征:一项回顾性、多中心意大利研究,分析 136 例组织学上同质且连续的患者中的发生率和病理生理学

Posterior fossa syndrome in a population of children and young adults with medulloblastoma: a retrospective, multicenter Italian study on incidence and pathophysiology in a histologically homogeneous and consecutive series of 136 patients.

机构信息

Department of Medicine and Surgery, School of Medicine, University of Milano-Bicocca, Milan, Italy.

Neurosurgery Unit, Ospedale San Gerardo, Monza, Italy.

出版信息

J Neurooncol. 2022 Sep;159(2):377-387. doi: 10.1007/s11060-022-04072-x. Epub 2022 Jun 29.

Abstract

INTRODUCTION

Posterior fossa syndrome (PFS) is a set of debilitating complications that can occur after surgery for posterior fossa tumors. This study aimed to assess the preoperative radiological and surgical risk factors for the onset of PFS in a histologically homogeneous population of children with medulloblastoma and compare it to a similar population of young adults.

METHODS

Included patients underwent posterior fossa surgery for medulloblastoma at 11 Italian neurosurgical wards (2003-2019) and were referred to Fondazione IRCCS Istituto Nazionale dei Tumori in Milan (INT) for postoperative treatments. We collected patients' pre- and post-operative clinical, surgical and radiological data from the INT charts. To compare the distribution of variables, we used the Mann-Whitney and Fisher tests for continuous and categorical variables, respectively.

RESULTS

136 patients (109 children and 27 young adults) were included in the study. Among children, 29 (27%) developed PFS, and all of them had tumors at midline site with invasion of the fourth ventricle. Radiological evidence of involvement of the right superior (39% versus 12%; p = 0.011) or middle cerebellar peduncles (52% versus 18%; p = 0.002) seemed more common in children who developed PFS. Young adults showed an expected lower incidence of PFS (4 out of 27; 15%), that may be due to anatomical, physiological and oncological elements.

CONCLUSIONS

This study confirmed some factors known to be associated with PFS onset and shed light on other debated issues. Our findings enhance an already hypothesized role of cerebellar language lateralization. The analysis of a population of young adults may shed more light on the often-neglected existence of PFS in non-pediatric patients.

摘要

简介

后颅窝综合征(PFS)是一组在颅后窝肿瘤手术后可能发生的使人虚弱的并发症。本研究旨在评估一组组织学同质的儿童髓母细胞瘤患者中,与后颅窝手术相关的术前放射学和手术危险因素,并与类似的年轻成人患者进行比较。

方法

11 家意大利神经外科病房(2003-2019 年)的患者接受了髓母细胞瘤的后颅窝手术,术后在米兰的国家肿瘤研究所(INT)基金会接受了后续治疗。我们从 INT 病历中收集了患者术前和术后的临床、手术和放射学数据。为了比较变量的分布,我们分别使用了 Mann-Whitney 和 Fisher 检验来比较连续变量和分类变量。

结果

共纳入 136 名患者(109 名儿童和 27 名年轻成人)。在儿童中,29 名(27%)发生了 PFS,所有患者的肿瘤位于中线部位,侵犯第四脑室。右侧上(39%对 12%;p=0.011)或中脑小脑脚(52%对 18%;p=0.002)受累的放射学证据在发生 PFS 的儿童中更为常见。年轻成人的 PFS 发生率预期较低(27 名中有 4 名,15%),这可能是由于解剖学、生理学和肿瘤学因素。

结论

本研究证实了一些已知与 PFS 发病相关的因素,并阐明了其他有争议的问题。我们的发现增强了小脑语言侧化的假设作用。对年轻成人患者的分析可能更清楚地揭示了在非儿科患者中经常被忽视的 PFS 存在。

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