From the Department of Medical Physics and Clinical Engineering (D.R., P.S.M.) and Department of Radiology (R.A.D., T.J.), Nottingham University Hospitals Trust, Derby Rd, Nottingham NG7 2UH, England; Mental Health & Clinical Neurosciences Unit, University of Nottingham, Nottingham, England (D.R., R.A.D., P.S.M.); Department of Pediatric Radiology, Necker Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France (R.C.); Department of Radiology & Nuclear Medicine, VU University Medical Center, Amsterdam, the Netherlands (E.S.A.); Department of Radiology, Leeds Teaching Hospitals, Leeds, England (D.W.); Institute for Diagnostic and Interventional Neuroradiology, Würzburg University, Würzburg, Germany (M.W.M.); Divisions of Molecular Pathology and Cancer Therapeutics, The Institute of Cancer Research, London, England (C.J., A.M.); Department of Pathological Anatomy and Cytology, Centre Hospitalier Sainte Anne, Paris, France (P.V.); Department of Biostatistics, Centre Oscar Lambret, Lille, France (M.C.L.D.); Department of Haematology and Oncology, Great Ormond Street Hospital, London, England (D.H.); Pediatric Oncology and Hematology Unit, Hospital La Fe, Valencia, Spain (A.C.); Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy (M.M.); Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Medical University of Vienna, Vienna, Austria (A.A.A.); Auckland Radiation Oncology, Auckland City Hospital, New Zealand. (F.S.); F. Hoffmann-La Roche, Basel, Switzerland (G.Z., J. Garcia); Department of Pediatric and Adolescent Oncology and Unit 981 from the Institut National de la Santé et de la Recherche Médicale, Gustave Roussy, Université Paris-Saclay, Université Paris-Sud, Villejuif, France (G.V., J. Grill); and Institute of Cancer and Genomic Sciences, University of Birmingham, and Birmingham Children's Hospital, Birmingham, England (A.P.).
Radiology. 2022 Jul;304(1):174-182. doi: 10.1148/radiol.211464. Epub 2022 Apr 12.
Background Diffuse midline gliomas (DMG) are characterized by a high incidence of mutations and poorer outcome. The HERBY trial has provided one of the largest cohorts of pediatric DMGs with available radiologic, histologic-genotypic, and survival data. Purpose To define MRI and molecular characteristics of DMG. Materials and Methods This study is a secondary analysis of a prospective trial (HERBY; ClinicalTrials.gov identifier, NCT01390948) undertaken between October 2011 and February 2016. Among 121 HERBY participants, 50 had midline nonpontine-based tumors. Midline high-grade gliomas were reclassified into DMG mutant, wild type with enhancer of zest homologs inhibitory protein overexpression, epidermal growth factor receptormutant, or not otherwise stated. The epicenter of each tumor and other radiologic characteristics were ascertained from MRI and correlated with the new subtype classification, histopathologic characteristics, surgical extent, and outcome parameters. Kaplan-Meier curves and log-rank tests were applied to determine and describe survival differences between groups. Results There were 42 participants (mean age, 12 years ± 4 [SD]; 23 girls) with radiologically evaluable thalamic-based DMG. Eighteen had partial thalamic involvement (12 thalamopulvinar, six anteromedial), 10 involved a whole thalamus, nine had unithalamic tumors with diffuse contiguous extension, and five had bithalamic tumors (two symmetric, three partial). Twenty-eight participants had DMG mutant tumors; there were no differences in outcome compared with other DMGs ( = 4). Participants who underwent major debulking or total or near-total resection had longer overall survival (OS): 18.5 months vs 11.4 months ( = .02). Enrolled participants who developed leptomeningeal metastatic dissemination before starting treatment had worse outcomes (event-free survival, 2.9 months vs 8.0 months [ = .02]; OS, 11.4 months vs 18.5 months [ = .004]). Conclusion Thalamic involvement of diffuse midline gliomas ranged from localized partial thalamic to holo- or bithalamic with diffuse contiguous spread and had poor outcomes, irrespective of subtype alterations. Leptomeningeal dissemination and less than 50% surgical resection were adverse risk factors for survival. Clinical trial registration no. NCT01390948 © RSNA, 2022 . See also the editorial by Widjaja in this issue.
背景 弥漫性中线胶质瘤（DMG）的特征是突变发生率高，预后较差。HERBY 试验提供了最大的儿科 DMG 队列之一，其中包括可获得的影像学、组织学-基因型和生存数据。
目的 定义 DMG 的 MRI 和分子特征。
材料和方法 本研究是对一项前瞻性试验（HERBY；临床试验.gov 标识符，NCT01390948）的二次分析，该试验于 2011 年 10 月至 2016 年 2 月进行。在 121 名 HERBY 参与者中，50 名患有中线非脑桥基肿瘤。中线高级别胶质瘤被重新分类为 DMG 突变型、 野生型伴 Zeste 同源物增强子抑制蛋白过表达、表皮生长因子受体突变型或其他未分类。从 MRI 确定每个肿瘤的中心点和其他影像学特征，并将其与新的亚型分类、组织病理学特征、手术范围和生存参数相关联。Kaplan-Meier 曲线和对数秩检验用于确定和描述组间的生存差异。
结果 有 42 名（平均年龄，12 岁±4[SD]；23 名女孩）参与者的丘脑基 DMG 可进行影像学评估。18 名部分丘脑受累（12 名丘脑豆状核，6 名前内侧），10 名全丘脑受累，9 名单侧丘脑肿瘤弥漫性连续延伸，5 名双侧丘脑肿瘤（2 名对称，3 名部分）。28 名参与者患有 DMG 突变型肿瘤；与其他 DMG 相比，预后无差异（ = 4）。接受主要减瘤术或全切除或近全切除的参与者具有更长的总生存期（OS）：18.5 个月比 11.4 个月（ =.02）。入组参与者在开始治疗前发生脑膜转移扩散的预后较差（无进展生存期，2.9 个月比 8.0 个月[ =.02]；OS，11.4 个月比 18.5 个月[ =.004]）。
结论 弥漫性中线胶质瘤的丘脑受累范围从局部性部分丘脑到全或双侧丘脑，伴有弥漫性连续蔓延，预后较差，与 亚型改变无关。脑膜扩散和手术切除不足 50%是生存的不利危险因素。
临床试验注册号 NCT01390948 © RSNA，2022 。也请参阅本期 Widjaja 的社论。
