Pediatric-type diffuse low-grade gliomas with MYB alterations: Neuroimaging of the Diffuse astrocytomas, MYB or MYBL1-altered.

Fiorenza Coppola, Brigitte Bison, Anna Tietze, Volodia Dangouloff-Ros, Maarten Lequin, Sniya Sudhakar, Thomas S Jacques, Maarit Palomäki, Cesar Ap Alves, Kshitij Mankad, Pablo Hernáiz Driever, Svea Horn, Lars Behrens, Darren R Hargrave, Giovanni Morana, Ulrike Löbel
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Abstract

Background and purpose: Diffuse astrocytoma, MYB or MYBL1-altered is a new tumor type in the family of Pediatric-type diffuse low-grade gliomas and genetically related to angiocentric glioma. Imaging features of Diffuse astrocytoma, MYB or MYBL1-altered are less well known. During our clinical work, we identified a relatively characteristic imaging pattern in a subset of our patients consisting of a large, diffuse hemispheric tumor with displaced central vessels which we termed the "fireworks sign". Therefore, the purpose of this work was to describe the frequency of this sign and any additional imaging characteristics in a large patient cohort.

Materials and methods: This international retrospective study included 40 patients from 7 countries. We recorded clinical, genetic and standard radiological features focusing on the presence of the "fireworks sign" but also the presence or absence of a mismatch between T2WI and FLAIR and a FLAIR rim which have both been reported in these patients.

Results: Most tumors were unilateral and located in the cerebral hemispheres (most frequently in parietal, temporal and frontal lobes), hypointense on T1WI and hyperintense on T2WI. No tumor showed diffusion restriction. The "fireworks sign" was present in 16 patients. Other features included: T2/FLAIR mismatch (24 cases), FLAIR rim (18 cases), necrosis (5 cases) and enhancement (5 cases). Tumor volume was significantly associated with the "fireworks sign" (p=0.01) and T2/FLAIR mismatch (p=0.005).

Conclusions: Imaging features of Diffuse astrocytoma, MYB1 or MYBL1-altered in children are variable and the "fireworks sign" was identified in a subset of patients only. It was usually present in patients with large hemispheric tumors but also observed in brainstem lesions. Diffusion restriction was consistently absent and enhancement infrequent. A T2/FLAIR mismatch was seen in more than 50% of patients.

Abbreviations: WHO = World Health Organization; DA-MYB = Diffuse astrocytoma, MYB or MYBL1-altered.

小儿型弥漫性低级别胶质瘤伴MYB改变:弥漫性星形细胞瘤的神经影像学,MYB或mybl1改变。
背景与目的:弥漫性星形细胞瘤(MYB或MYBL1-altered)是儿科弥漫性低级别胶质瘤家族中的一种新肿瘤类型,与血管中心性胶质瘤有遗传关系。弥漫性星形细胞瘤、MYB或mybl1改变的影像学特征尚不清楚。在我们的临床工作中,我们在我们的患者中发现了一种相对特征性的影像学模式,包括一个大的弥漫性半球肿瘤,中心血管移位,我们称之为“烟火征”。因此,这项工作的目的是在一个大的患者队列中描述该征象的频率和任何额外的影像学特征。材料和方法:本国际回顾性研究包括来自7个国家的40例患者。我们记录了临床、遗传和标准放射学特征,重点是“烟火征”的存在,以及T2WI与FLAIR和FLAIR边缘之间是否存在不匹配,这两种情况在这些患者中都有报道。结果:肿瘤多为单侧,位于大脑半球(多见于顶叶、颞叶和额叶),T1WI呈低信号,T2WI呈高信号。肿瘤未见扩散限制。16例患者出现“烟花标志”。其他特征包括:T2/FLAIR不匹配(24例),FLAIR边缘(18例),坏死(5例)和强化(5例)。肿瘤体积与“烟火征”(p=0.01)和T2/FLAIR不匹配(p=0.005)显著相关。结论:弥漫性星形细胞瘤、MYB1或mybl1在儿童中改变的影像学特征是可变的,“烟花征”仅在一小部分患者中被发现。它通常出现在大半球肿瘤患者中,但也见于脑干病变。扩散限制始终不存在,增强也不常见。超过50%的患者出现T2/FLAIR不匹配。缩写:WHO =世界卫生组织;DA-MYB =弥漫性星形细胞瘤,MYB或mybl1改变。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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