Research ArticleORIGINAL RESEARCH
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 and Ulrike Löbel
American Journal of Neuroradiology June 2025, ajnr.A8894; DOI: https://doi.org/10.3174/ajnr.A8894
Fiorenza Coppola
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).
Brigitte Bison
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).
Anna Tietze
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).
Volodia Dangouloff-Ros
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).
Maarten Lequin
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).
Sniya Sudhakar
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).
Thomas S Jacques
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).
Maarit Palomäki
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).
Cesar AP Alves
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).
Kshitij Mankad
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).
Pablo Hernáiz Driever
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).
Svea Horn
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).
Lars Behrens
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).
Darren R Hargrave
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).
Giovanni Morana
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).
Ulrike Löbel
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).
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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
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Accepted Manuscript
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
Pediatric-type diffuse low-grade gliomas with MYB alterations: Neuroimaging of the Diffuse astrocytomas, MYB or MYBL1-altered
American Journal of Neuroradiology Jun 2025, ajnr.A8894; DOI: 10.3174/ajnr.A8894
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Accepted Manuscript
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
American Journal of Neuroradiology Jun 2025, ajnr.A8894; DOI: 10.3174/ajnr.A8894
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