Characteristics of diffuse hemispheric gliomas, H3 G34-mutant in adults
Picart, Thiébaud (Hôpital Neurologique Pierre Wertheimer. Department of Neurosurgery)
Barritault, Marc (Cancer Research Centre of Lyon)
Poncet, Delphine (University Claude Bernard Lyon I)
Berner, Lise-Prune (Hôpital Neurologique Pierre Wertheimer. Department of Neuroradiology)
Izquierdo, Cristina (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Tabouret, Emeline (Aix-Marseille Université. Institut de Neurophysiopathologie)
Figarella-Branger, Dominique (Aix-Marseille Université. Institut de Neurophysiopathologie)
Idbaïh, Ahmed (Sorbonne Université. Institut du Cerveau et de la Moelle épinière)
Bielle, Franck (Sorbonne Université)
Bourg, Véronique (Hôpital Pasteur. Department of Neurology)
Vandenbos, Fanny Burel (Université Côte D'Azur. Institut de Biologie Valrose)
Moyal, Elizabeth Cohen-Jonathan (Centre de Recherches contre le Cancer de Toulouse)
Uro-Coste, Emmanelle (Institut Universitaire du Cancer Toulouse-Oncopole)
Guyotat, Jacques (Hôpital Neurologique Pierre Wertheimer. Department of Neurosurgery)
Honnorat, Jérôme (University Claude Bernard Lyon I)
Gabut, Mathieu (University Claude Bernard Lyon I)
Meyronet, David (University Claude Bernard Lyon I)
Ducray, François (University Claude Bernard Lyon I)

Data:	2021
Resum:	Diffuse hemispheric gliomas, H3 G34-mutant (DHG H3G34-mutant) constitute a distinct type of aggressive brain tumors. Although initially described in children, they can also affect adults. The aims of this study were to describe the characteristics of DHG H3G34-mutant in adults and to compare them to those of established types of adult WHO grade IV gliomas. The characteristics of 17 adult DHG H3G34-mutant, 32 H3. 3 K27M-mutant diffuse midline gliomas (DMG), 100 IDH-wildtype, and 36 IDH-mutant glioblastomas were retrospectively analyzed. Median age at diagnosis in adult DHG H3G34-mutant was 25 years (range: 19-33). All tumors were hemispheric. For 9 patients (56%), absent or faint contrast enhancement initially suggested another diagnosis than a high-grade glioma, and diffusion-weighted imaging seemed retrospectively more helpful to suspect an aggressive tumor than MR-spectroscopy and perfusion MRI. All cases were IDH-wildtype. Most cases were immunonegative for ATRX (93%) and Olig2 (100%) and exhibited MGMT promoter methylation (82%). The clinical and radiological presentations of adult DHG H3G34-mutant were different from those of established types of adult grade IV gliomas. Median overall survival of adult DHG H3G34-mutant was 12. 4 months compared to 19. 6 months (P =. 56), 11. 7 months (P =. 45), and 50. 5 months (P =. 006) in H3. 3 K27M-mutant DMG, IDH-wildtype, and IDH-mutant glioblastomas, respectively. Adult DHG H3G34-mutant are associated with distinct characteristics compared to those of established types of adult WHO grade IV gliomas. This study supports considering these tumors as a new type of WHO grade IV glioma in future classifications.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Estudi clínic ; recerca
Matèria:	Diffuse hemispheric glioma ; H3.3 G34R/V mutation ; H3.3 K27M mutation ; PNET ; Survival
Publicat a:	Neuro-oncology Advances, Vol. 3, Núm. 1 (april 2021) , p. 1-12, ISSN 2632-2498

DOI: 10.1093/noajnl/vdab061
PMID: 34056608

12 p, 874.3 KB

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Documents de recerca > Documents dels grups de recerca de la UAB > Centres i grups de recerca (producció científica) > Ciències de la salut i biociències > Institut d'Investigació en Ciencies de la Salut Germans Trias i Pujol (IGTP)
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