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横纹肌样脑膜瘤:脑膜瘤罕见亚型的临床病理分析。

Rhabdoid meningiomas: Clinicopathological analysis of a rare variant of meningioma.

机构信息

Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India.

Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore 560029, India.

出版信息

Clin Neurol Neurosurg. 2021 Aug;207:106778. doi: 10.1016/j.clineuro.2021.106778. Epub 2021 Jun 24.

Abstract

INTRODUCTION

Rhabdoid Meningiomas (RM) are rare malignant type of meningiomas, classified as grade III in the WHO classification. Only a few case series have been reported, and factors affecting prognosis are still unclear.

METHODS

We did a retrospective chart review of all the RMs diagnosed in our institute between 2007 and 2019. Demographic profile, clinical status, imaging, surgical procedures used, post-operative course, adjuvant therapy and follow-ups were reviewed. Histopathological slides were also reviewed.

RESULTS

There were 11 patients with RM who underwent 17 surgical procedures between them. Median age was 26 years. On imaging, four had lesions in skull base, three in convexity and four in parasagittal region. Five patients had lesions which had bled and two had leptomeningeal dissemination. Two patients underwent Simpson's grade 1 excision, seven underwent grade 2 and one patient each underwent grade 3 and 5 excisions. One patient presented with poor sensorium and underwent surgery but ultimately succumbed. All reported patients had Rhabdoid features (>50%). Features of anaplasia were seen in four cases and atypical meningioma in others. The median progression-free-survival and overall survival was 6 months and 9 months, respectively. Female gender (n = 5; p = 0.032) and patients who received radiotherapy (p = 0.030) had a survival advantage. Location of the tumor (p = 0.43), presence of hemorrhage in the lesion (p = 0.49), grade of excision (p = 0.40) and WHO pathological grade (p = 0.11) did not have a statistically significant survival benefit.

CONCLUSION

Female gender and adjuvant radiotherapy were associated with survival advantage in our sample. Large studies are required to establish the factors associated with survival.

摘要

简介

横纹肌样脑膜瘤(RM)是一种罕见的恶性脑膜瘤类型,在世界卫生组织(WHO)分类中被归类为 3 级。仅有少数病例系列报告,影响预后的因素仍不清楚。

方法

我们对 2007 年至 2019 年期间在我院诊断的所有 RM 患者进行了回顾性图表审查。回顾了人口统计学特征、临床状况、影像学、使用的手术程序、术后过程、辅助治疗和随访。还回顾了组织病理学切片。

结果

共有 11 名 RM 患者,共进行了 17 次手术。中位年龄为 26 岁。在影像学上,4 例病变位于颅底,3 例位于凸面,4 例位于矢状旁区。5 例患者有病变出血,2 例有软脑膜播散。2 例患者行 Simpson 1 级切除术,7 例行 2 级切除术,1 例分别行 3 级和 5 级切除术。1 例患者出现意识障碍并接受手术,但最终死亡。所有报告的患者均具有横纹肌样特征(>50%)。4 例有间变特征,其他病例有非典型脑膜瘤特征。无进展生存期和总生存期的中位数分别为 6 个月和 9 个月。女性(n=5;p=0.032)和接受放疗的患者(p=0.030)具有生存优势。肿瘤位置(p=0.43)、病变出血(p=0.49)、切除分级(p=0.40)和 WHO 病理分级(p=0.11)与生存无显著相关性。

结论

在我们的样本中,女性和辅助放疗与生存优势相关。需要进行大型研究以确定与生存相关的因素。

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