Alexis Plasse, Raphael Levy, Arnault Tauziède-Espariat, Thibault Agripnidis, Jean-François Hak, Charles-Joris Roux, Thomas Samoyeau, Alexis Ollitrault, Kevin Beccaria, Lelio Guida, Thomas Blauwblomme, Jacques Grill, Christelle Dufour, Léa Guerrini-Rousseau, Franck Bourdeaut, François Doz, Pascale Varlet, Nathalie Boddaert, Volodia Dangouloff-Ros
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Our aim was to find imaging features distinguishing these tumors and to develop a diagnostic score.</p><p><strong>Patients and methods: </strong>A retrospective study spanning September 1999 to May 2021 involved pediatric patients with thalamic gliomas, categorized into H3K27-altered DMG and LGG groups. Preoperative imaging, including morphology, diffusion, and arterial-spin-labeling perfusion, was reviewed blindly and compared between the two groups. A diagnostic score was formulated based on significant findings. Results were validated using an internal and external validation cohort.</p><p><strong>Results: </strong>Sixty-six patients were included (median age, 9 years; interquartile range [IQR] [5-13]; 38 girls) with 37 DMG H3K27-altered and 29 LGG. DMG H3K27-altered tumors exhibited larger volumes (median 64 cc, IQR [36-88] vs 26 cc, IQR [15-37], p < 0.001), greater heterogeneity in T2-weighted signal and enhancement (62% [22/37] vs 31% [9/29], p = 0.04, and 97% [32/33] vs 56% [15/27], p = 0.0008, respectively), lower minimum relative apparent diffusion coefficient (ADC) (0.92 (IQR [0.76-1.23]) vs 1.55 (IQR [1.40-1.72]), p < 0.001), and higher relative maximum cerebral blood flow (CBF) levels (2.08 (IQR [1.48-3.05]) vs 0.84 (IQR [0.45-1]), p < 0.001). A diagnostic score integrating tumor diameter, solid content predominance, relative ADC, and relative CBF achieved 100% sensitivity and specificity in distinguishing DMG H3K27-altered tumors from LGG (full score available for 36 patients), with good results in external and internal validation cohorts (12 patients).</p><p><strong>Conclusion: </strong>The morphological, diffusion, and arterial spin labeling imaging characteristics of pediatric thalamic tumors enable excellent differentiation of DMG H3K27-altered and LGG.</p>","PeriodicalId":19822,"journal":{"name":"Pediatric Blood & Cancer","volume":" ","pages":"e31704"},"PeriodicalIF":2.4000,"publicationDate":"2025-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"MRI-Based Score to Recognize Thalamic Glioma Grade in Children: Morphology, Diffusion, and Arterial-Spin-Labeling Perfusion.\",\"authors\":\"Alexis Plasse, Raphael Levy, Arnault Tauziède-Espariat, Thibault Agripnidis, Jean-François Hak, Charles-Joris Roux, Thomas Samoyeau, Alexis Ollitrault, Kevin Beccaria, Lelio Guida, Thomas Blauwblomme, Jacques Grill, Christelle Dufour, Léa Guerrini-Rousseau, Franck Bourdeaut, François Doz, Pascale Varlet, Nathalie Boddaert, Volodia Dangouloff-Ros\",\"doi\":\"10.1002/pbc.31704\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Purpose: </strong>Thalamic gliomas are found predominantly in children and can be classified into two main types with different prognoses and management: diffuse midline glioma (DMG) H3K27-altered and low-grade glioma (LGG). 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DMG H3K27-altered tumors exhibited larger volumes (median 64 cc, IQR [36-88] vs 26 cc, IQR [15-37], p < 0.001), greater heterogeneity in T2-weighted signal and enhancement (62% [22/37] vs 31% [9/29], p = 0.04, and 97% [32/33] vs 56% [15/27], p = 0.0008, respectively), lower minimum relative apparent diffusion coefficient (ADC) (0.92 (IQR [0.76-1.23]) vs 1.55 (IQR [1.40-1.72]), p < 0.001), and higher relative maximum cerebral blood flow (CBF) levels (2.08 (IQR [1.48-3.05]) vs 0.84 (IQR [0.45-1]), p < 0.001). 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引用次数: 0
摘要
目的：丘脑胶质瘤主要见于儿童，可分为两种主要类型，具有不同的预后和治疗：弥漫性中线胶质瘤（DMG） h3k27改变和低级别胶质瘤（LGG）。我们的目的是找到区分这些肿瘤的影像学特征，并制定诊断评分。患者和方法：1999年9月至2021年5月的一项回顾性研究涉及丘脑胶质瘤的儿科患者，分为h3k27改变的DMG组和LGG组。术前影像学，包括形态学、弥散和动脉自旋标记灌注，盲目回顾并比较两组。诊断评分是根据显著的发现制定的。结果通过内部和外部验证队列进行验证。结果：纳入66例患者(中位年龄9岁；四分位间距[IQR] [5-13]；38名女孩)，37名DMG h3k27改变，29名LGG。DMG h3k27改变的肿瘤体积更大（中位数64 cc， IQR [36-88] vs 26 cc, IQR [15-37], p < 0.001）， t2加权信号和增强的异质性更大（62% [22/37]vs 31% [9/29], p = 0.04, 97% [32/33] vs 56% [15/27], p = 0.0008），最小相对表观扩散系数（ADC）更低(0.92 （IQR [0.76-1.23]) vs 1.55 (IQR [1.40-1.72]), p < 0.001）。相对最大脑血流量（CBF）水平较高(2.08 （IQR [1.48-3.05]) vs 0.84 (IQR [0.45-1]), p < 0.001）。综合肿瘤直径、固体含量优势、相对ADC和相对CBF的诊断评分在区分DMG h3k27改变的肿瘤和LGG方面达到100%的敏感性和特异性（36例患者获得满分），在外部和内部验证队列（12例患者）中取得了良好的结果。结论：儿童丘脑肿瘤的形态学、弥散和动脉自旋标记成像特征使DMG h3k27改变和LGG具有良好的分化能力。
MRI-Based Score to Recognize Thalamic Glioma Grade in Children: Morphology, Diffusion, and Arterial-Spin-Labeling Perfusion.
Purpose: Thalamic gliomas are found predominantly in children and can be classified into two main types with different prognoses and management: diffuse midline glioma (DMG) H3K27-altered and low-grade glioma (LGG). Our aim was to find imaging features distinguishing these tumors and to develop a diagnostic score.
Patients and methods: A retrospective study spanning September 1999 to May 2021 involved pediatric patients with thalamic gliomas, categorized into H3K27-altered DMG and LGG groups. Preoperative imaging, including morphology, diffusion, and arterial-spin-labeling perfusion, was reviewed blindly and compared between the two groups. A diagnostic score was formulated based on significant findings. Results were validated using an internal and external validation cohort.
Results: Sixty-six patients were included (median age, 9 years; interquartile range [IQR] [5-13]; 38 girls) with 37 DMG H3K27-altered and 29 LGG. DMG H3K27-altered tumors exhibited larger volumes (median 64 cc, IQR [36-88] vs 26 cc, IQR [15-37], p < 0.001), greater heterogeneity in T2-weighted signal and enhancement (62% [22/37] vs 31% [9/29], p = 0.04, and 97% [32/33] vs 56% [15/27], p = 0.0008, respectively), lower minimum relative apparent diffusion coefficient (ADC) (0.92 (IQR [0.76-1.23]) vs 1.55 (IQR [1.40-1.72]), p < 0.001), and higher relative maximum cerebral blood flow (CBF) levels (2.08 (IQR [1.48-3.05]) vs 0.84 (IQR [0.45-1]), p < 0.001). A diagnostic score integrating tumor diameter, solid content predominance, relative ADC, and relative CBF achieved 100% sensitivity and specificity in distinguishing DMG H3K27-altered tumors from LGG (full score available for 36 patients), with good results in external and internal validation cohorts (12 patients).
Conclusion: The morphological, diffusion, and arterial spin labeling imaging characteristics of pediatric thalamic tumors enable excellent differentiation of DMG H3K27-altered and LGG.
期刊介绍：
Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.
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