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1型神经纤维瘤病中的胶质母细胞瘤:一种独特的实体——聚焦手术意义及我们经验的文献综述

Glioblastoma in NF1: A Unique Entity-A Literature Review Focusing on Surgical Implication and Our Experience.

作者信息

Garbin Elisa, Nicolè Lorenzo, Magrini Salima, Ceccaroni Yuri, Denaro Luca, Basaldella Luca, Rossetto Marta

机构信息

Academic Neurosurgery, Department of Neurosciences, University of Padova, 35128 Padova, PD, Italy.

Unit of Pathology, Angel Hospital, 30174 Mestre, VE, Italy.

出版信息

Curr Oncol. 2025 Apr 21;32(4):242. doi: 10.3390/curroncol32040242.

DOI:10.3390/curroncol32040242
PMID:40277798
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12025599/
Abstract

Glioblastoma in patients affected by NF1 germline mutation (NF1-associated GBM) represents a unique heterogeneous clinical and pathological entity. We have reviewed the few cases reported in the literature and they seem to have a better response to standard therapy and overall survival than GBM in the non-NF1 population. We present two cases of long-survival NF1 patients with GBM. Case 1 was a 38-year-old woman with cerebellar GBM who underwent surgical asportation and the Stupp protocol many times with an overall survival of 117 months. Case 2 was a 47-year-old woman with GBM in the eloquent area of the right frontal lobe; she underwent surgical asportation and the Stupp protocol with an overall survival of 25 months. The data analysis demonstrates that NF1-associated GBM patients could be considered long-term survivors.

摘要

患有NF1种系突变的患者中的胶质母细胞瘤(NF1相关胶质母细胞瘤)是一种独特的异质性临床和病理实体。我们回顾了文献中报道的少数病例,与非NF1人群中的胶质母细胞瘤相比,它们似乎对标准治疗和总生存期有更好的反应。我们介绍了两例长期存活的NF1相关胶质母细胞瘤患者。病例1是一名38岁患有小脑胶质母细胞瘤的女性,她多次接受手术切除和Stupp方案治疗,总生存期为117个月。病例2是一名47岁患有右侧额叶功能区胶质母细胞瘤的女性;她接受了手术切除和Stupp方案治疗,总生存期为25个月。数据分析表明,NF1相关胶质母细胞瘤患者可被视为长期存活者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e17/12025599/9ad340d47757/curroncol-32-00242-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e17/12025599/1f4a261df951/curroncol-32-00242-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e17/12025599/9ad340d47757/curroncol-32-00242-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e17/12025599/1f4a261df951/curroncol-32-00242-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e17/12025599/9ad340d47757/curroncol-32-00242-g002.jpg

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本文引用的文献

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Cancer Lett. 2024 Apr 28;588:216711. doi: 10.1016/j.canlet.2024.216711. Epub 2024 Feb 27.
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Clinicopathological and Immunohistochemistry Study of a Long Survivor of Giant Cell Glioblastoma in a Patient With Neurofibromatosis 1: Case Report.1型神经纤维瘤病患者巨大细胞胶质母细胞瘤长期存活者的临床病理及免疫组化研究:病例报告
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Prognostic evaluation of re-resection for recurrent glioblastoma using the novel RANO classification for extent of resection: A report of the RANO resect group.
使用新的 RANO 切除范围分类对复发性胶质母细胞瘤再次切除的预后评估:来自 RANO 切除组的报告。
Neuro Oncol. 2023 Sep 5;25(9):1672-1685. doi: 10.1093/neuonc/noad074.
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Radiother Oncol. 2023 Jul;184:109663. doi: 10.1016/j.radonc.2023.109663. Epub 2023 Apr 13.
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Expanded analysis of high-grade astrocytoma with piloid features identifies an epigenetically and clinically distinct subtype associated with neurofibromatosis type 1.具有 毛细胞型星形细胞瘤 特征的高级别星形细胞瘤的扩展分析确定了一种与神经纤维瘤病 1 型相关的具有独特表观遗传和临床特征的亚型。
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