Coppola Fiorenza, Bison Brigitte, Tietze Anna, Dangouloff-Ros Volodia, Lequin Maarten, Sudhakar Sniya, Jacques Thomas S, Palomäki Maarit, Alves Cesar Ap, Mankad Kshitij, Driever Pablo Hernáiz, Horn Svea, Behrens Lars, Hargrave Darren R, Morana Giovanni, Löbel Ulrike
From the Neuroradiology Unit, Department of Diagnostic and Interventional Radiology, A.O.U. Città della Salute e della Scienza di Torino, Turin, Italy (FC, GM). Diagnostic and Interventional Neuroradiology, Neuroradiological Reference Center for the HIT-Studies of the GPOH, Faculty of Medicine, University of Augsburg, Augsburg, Germany (BB), Charité Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt- Universität zu Berlin, Germany (AT), Pediatric Radiology Department, AP-HP, Hôpital Universitaire Necker-Enfants Malades, 75015, Paris, France, and Université Paris Cité, Paris, France (VD), Edward B. Singleton Department of Radiology, Texas Children's Hospital and Baylor College of Medicine, 6701 Fannin Street, Suite 470, Houston, TX 77030, USA (ML), Department of Radiology, Division of Neuroradiology, Great Ormond Street Hospital, Great Ormond Street, WC1N 3JH London, UK (SS, KM, UL), Department of Developmental Biology & Cancer, UCL GOS Institute of Child Health, and Department of Histopathology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK (TJ), Department of Radiology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland (MP), Radiology Department, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA (CA), Department of Pediatric Oncology and Hematology, Charité-Universitätsmedizin Berlin, Berlin, Germany (PHD, SH), Department of Diagnostic and Interventional Neuroradiology, University Hospital Augsburg, Stenglinstraße 2, 86156, Augsburg, Germany (LB), University College London Great Ormond Street Institute of Child Health, London, UK (DH), Department of Neurosciences, University of Turin, Turin, Italy (GM).
AJNR Am J Neuroradiol. 2025 Jun 24. doi: 10.3174/ajnr.A8894.
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.
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.
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).
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.
WHO = World Health Organization; DA-MYB = Diffuse astrocytoma, or .
弥漫性星形细胞瘤，MYB或MYBL1改变型是儿童型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存在基因相关性。弥漫性星形细胞瘤，MYB或MYBL1改变型的影像学特征鲜为人知。在我们的临床工作中，我们在一部分患者中发现了一种相对具有特征性的影像学表现，即一个大的、弥漫性半球形肿瘤伴中央血管移位，我们将其称为“烟花征”。因此，本研究的目的是描述该征象在一大组患者中的出现频率以及其他任何额外的影像学特征。
这项国际回顾性研究纳入了来自7个国家的40例患者。我们记录了临床、基因和标准放射学特征，重点关注“烟花征”的存在情况，以及这些患者中均有报道的T2WI与FLAIR之间的不匹配情况和FLAIR边缘的存在与否。
大多数肿瘤为单侧，位于大脑半球（最常见于顶叶、颞叶和额叶），T1WI呈低信号，T2WI呈高信号。无肿瘤表现出弥散受限。16例患者出现“烟花征”。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“烟花征”（p=0.01）和T2/FLAIR不匹配（p=0.005）显著相关。
儿童弥漫性星形细胞瘤，MYB1或MYBL1改变型的影像学特征各异，“烟花征”仅在一部分患者中出现。它通常见于半球形大肿瘤患者，但也见于脑干病变。始终未出现弥散受限，强化少见。超过50%的患者出现T
