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伴有混淆性类固醇反应的成人延髓H3 K27M改变的弥漫性中线胶质瘤的影像学特征

Imaging characterization of an adult H3 K27M-altered diffuse midline glioma of the medulla oblongata with a confounding steroid response.

作者信息

Godi Claudia, Garofalo Paolo, Callea Marcella, Vezzulli Paolo, Barbera Maurizio, Mandelli Carlo, Mazza Elena, Pollo Bianca, Terreni Maria Rosa, Castellano Antonella, Anzalone Nicoletta, Falini Andrea

机构信息

Neuroradiology Unit and CERMAC, IRCCS San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy.

Policlinico Universitario di Monserrato, Cagliari University, Cagliari, Italy.

出版信息

Radiol Case Rep. 2022 Dec 18;18(3):788-793. doi: 10.1016/j.radcr.2022.10.082. eCollection 2023 Mar.

DOI:10.1016/j.radcr.2022.10.082
PMID:36589504
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9794887/
Abstract

We report an uncommon, infratentorial localization of adult H3 K27M-altered diffuse midline glioma arising in a particularly rare site (medulla oblongata). In addition to this unusual presentation, the lesion exhibited a substantial contrast enhancement and size decrease after dexamethasone, generating diagnostic dilemmas. Histology, molecular details, advanced Magnetic Resonance imaging features and differential diagnoses are here described and discussed, as well as common misconceptions about steroid-sensitive mass lesions, and practical difficulties for clinicians involved in the process of making diagnosis.

摘要

我们报告了一例罕见的幕下定位的成人H3 K27M改变的弥漫性中线胶质瘤,其起源于一个特别罕见的部位(延髓)。除了这种不寻常的表现外,该病变在使用地塞米松后显示出明显的对比增强和体积减小,这产生了诊断难题。本文描述并讨论了组织学、分子细节、先进的磁共振成像特征和鉴别诊断,以及关于类固醇敏感的肿块病变的常见误解,以及参与诊断过程的临床医生所面临的实际困难。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/15c3ae634052/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/12b1da1f013c/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/f57f1d15a439/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/ebfba3634029/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/15c3ae634052/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/12b1da1f013c/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/f57f1d15a439/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/ebfba3634029/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/709d/9794887/15c3ae634052/gr4.jpg

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Imaging characterization of an adult H3 K27M-altered diffuse midline glioma of the medulla oblongata with a confounding steroid response.伴有混淆性类固醇反应的成人延髓H3 K27M改变的弥漫性中线胶质瘤的影像学特征
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本文引用的文献

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The 2021 WHO Classification of Tumors of the Central Nervous System: a summary.2021 年世卫组织中枢神经系统肿瘤分类:概述。
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Radiosurgery for Glioblastoma.伽玛刀治疗脑胶质瘤。
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The H3.3 K27M mutation results in a poorer prognosis in brainstem gliomas than thalamic gliomas in adults.在成人中,H3.3 K27M突变导致脑干胶质瘤的预后比丘脑胶质瘤更差。
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Glioblastoma presenting with steroid-induced pseudoregression of contrast enhancement on magnetic resonance imaging.胶质母细胞瘤在磁共振成像上表现为类固醇诱导的对比增强假性消退。
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