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一例间变性成星形细胞瘤罕见病例的13年长期随访:差异何在?

Thirteen-year long-term follow-up in a rare case of anaplastic astroblastoma: What makes the difference?

作者信息

Allison Callum Martin, Scoones David, Batra Arun, Sinclair Georges

机构信息

Department of Surgery, Royal Victoria Infirmary, Newcastle-upon-Tyne, UK.

Department of Neuropathology, James Cook University Hospital, Middlesbrough, UK.

出版信息

Surg Neurol Int. 2022 May 27;13:221. doi: 10.25259/SNI_1065_2021. eCollection 2022.

Abstract

BACKGROUND

Astroblastomas are uncommon neuroepithelial tumors of the central nervous system with a distinct, yet, controversial radiological, histological, and molecular profile. Debatable differences between low- and high-grade astroblastoma have been reported in the medical literature; indeed, despite the increasing relevance of molecular genetic profiling in the realm of astroblastoma, its application is still in its early stages. As a result, the diagnostic criteria for astroblastoma remain undecided with yet no real consensus on the most ideal management.

CASE DESCRIPTION

This report describes a case of astroblastoma diagnosed 13 years ago in a young woman who despite six episodes of recurrence, transformation, and progression was able to retain a perfomace status of 0 by World Health Organization standard, throughout.

CONCLUSION

This report discusses the clinical, radiological, histological features, and management of this rare tumor with an extraordinarily long survival, with an aim to strengthen the literature on management options. To the best of our knowledge, this is the longest surviving case of anaplastic astroblastoma reported in the available medical literature.

摘要

背景

星形母细胞瘤是中枢神经系统罕见的神经上皮肿瘤,具有独特但存在争议的放射学、组织学和分子特征。医学文献中报道了低级别和高级别星形母细胞瘤之间存在有争议的差异;实际上,尽管分子基因谱分析在星形母细胞瘤领域的相关性日益增加,但其应用仍处于早期阶段。因此,星形母细胞瘤的诊断标准仍未确定,对于最理想的治疗方法也尚未达成真正的共识。

病例描述

本报告描述了一名13年前被诊断为星形母细胞瘤的年轻女性病例,该患者尽管经历了6次复发、转变和进展,但始终能够保持世界卫生组织标准下的0级表现状态。

结论

本报告讨论了这种罕见肿瘤的临床、放射学、组织学特征及治疗情况,该肿瘤具有超长生存期,旨在加强有关治疗选择的文献报道。据我们所知,这是现有医学文献中报道的间变性星形母细胞瘤存活时间最长的病例。

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