Radiological and Immunostaining Characteristics of H3.3 G34R-Mutant Glioma: A Report of 3 Cases and Review of the Literature

Onishi, Shumpei; Amatya, Vishwa Jeet; Karlowee, Vega; Takeshima, Yukio; Sakamoto, Kazuhiko; Kurisu, Kaoru; Yamasaki, Fumiyuki

doi:10.1159/000511672

Cited by 9 publications

(17 citation statements)

References 16 publications

(24 reference statements)

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“…Leptomeningeal contact was observed in all 12 [20]. Concordant with these results, the two H3 G34-mutant described by Onishi et al exhibited little peritumoral edema given their large size [42].…”

Section: Tumor Margins and Locationsupporting

confidence: 81%

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Imaging features associated with H3 K27-altered and H3 G34-mutant gliomas: a narrative systematic review

et al. 2022

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Background Advances in molecular diagnostics accomplished the discovery of two malignant glioma entities harboring alterations in the H3 histone: diffuse midline glioma, H3 K27-altered and diffuse hemispheric glioma, H3 G34-mutant. Radiogenomics research, which aims to correlate tumor imaging features with genotypes, has not comprehensively examined histone-altered gliomas (HAG). The aim of this research was to synthesize the current published data on imaging features associated with HAG. Methods A systematic search was performed in March 2022 using PubMed and the Cochrane Library, identifying studies on the imaging features associated with H3 K27-altered and/or H3 G34-mutant gliomas. Results Forty-seven studies fulfilled the inclusion criteria, the majority on H3 K27-altered gliomas. Just under half (21/47) were case reports or short series, the remainder being diagnostic accuracy studies. Despite heterogeneous methodology, some themes emerged. In particular, enhancement of H3 K27M-altered gliomas is variable and can be less than expected given their highly malignant behavior. Low apparent diffusion coefficient values have been suggested as a biomarker of H3 K27-alteration, but high values do not exclude this genotype. Promising correlations between high relative cerebral blood volume values and H3 K27-alteration require further validation. Limited data on H3 G34-mutant gliomas suggest some morphologic overlap with 1p/19q-codeleted oligodendrogliomas. Conclusions The existing data are limited, especially for H3 G34-mutant gliomas and artificial intelligence techniques. Current evidence indicates that imaging-based predictions of HAG are insufficient to replace histological assessment. In particular, H3 K27-altered gliomas should be considered when occurring in typical midline locations irrespective of enhancement characteristics.

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Section: Tumor Margins and Locationsupporting

confidence: 81%

“…Only eight studies reported on H3 G34-mutant gliomas, with small numbers. All cases were high-grade histologically, the majority grade 4 [11,40,42,44,53]. Yoshimito identified four G34-mutant tumors amongst 411 consecutive gliomas (1.0%) of all ages, compared to 10 H3 K27-altered gliomas [11].…”

Section: H3 G34-mutant Gliomasmentioning

confidence: 99%

Imaging features associated with H3 K27-altered and H3 G34-mutant gliomas: a narrative systematic review

et al. 2022

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“… 1 , 7 , 14 When immunohistochemistry was used, cut-off percentages for ATRX and TP53 mutation status were set at 10% nuclear staining. 15 …”

Section: Methodsmentioning

confidence: 99%

Systematic review of diffuse hemispheric glioma, H3 G34-mutant: Outcomes and associated clinical factors

Crowell

Mata‐Mbemba

Bennett

et al. 2022

Neuro-Oncology Advances

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Background A comprehensive review and description of the clinical features that impact prognosis for patients with diffuse hemispheric glioma, H3 G34-mutant (G34-DHG) is needed. Understanding survival and prognostic features is paramount for clinical advancements and patient care. Methods PubMed, Embase, and Google Scholar were searched for English articles published between January 1, 2012, and June 30, 2021. Eligible studies included patient(s) of any age diagnosed with an H3 G34-mutant brain tumor with at least one measure of survival or progression. Patient level data were pooled for analyses. The protocol was prospectively registered in PROSPERO (CRD42021267764) and PRISMA guidelines were followed. Results Twenty-seven studies met criteria for inclusion. One-hundred and thirty-five patients with an H3 G34-mutant brain tumor were included. Median age at diagnosis was 15.8 years (IQR: 13.3-22.0) with 90% having localized disease. Co-occurring alterations included ATRX mutation in 93%, TP53 mutation in 88%, and MGMT promoter methylation in 70%. Median time-to-progression was 10.0 months (IQR: 6.0-18.0) and median overall survival was 17.3 months (95% CI 15.0-22.9). The median time from progression to death was 5.0 months (IQR: 3.0-11.7). Factors associated with survival duration were age, as patients ≥18 y/o demonstrated longer survival (HR=2.05, 95% CI 1.16-3.62), and degree of upfront resection, as near or gross-total resection demonstrated longer survival compared to those with less than near-total resection (HR=3.75, 95% CI 2.11-6.62). Conclusion This systematic review highlights available clinical data for G34-DHG demonstrating poor outcome and important prognostic features, while serving as a baseline for future research and clinical trials.

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“…A total of 332 abstracts were screened, of which 53 potentially eligible articles were selected for further review. After excluding 41 articles and including one article extracted from the citation review of each relevant article (no case with pathologically proven DHG‐G34m, 25 articles; no radiological findings or images, 14 articles; and insufficient patient‐specific information, 2 articles), 12 articles met the selection criteria for the systematic review 1,6–16 . The year of publication of the selected articles ranged from 2017 to 2021.…”

Section: Resultsmentioning

confidence: 99%

“…After excluding 41 articles and including one article extracted from the citation review of each relevant article (no case with pathologically proven DHG-G34m, 25 articles; no radiological findings or images, 14 articles; and insufficient patient-specific information, 2 articles), 12 articles met the selection criteria for the systematic review. 1,[6][7][8][9][10][11][12][13][14][15][16] The year of publication of the selected articles ranged from 2017 to 2021. With the additional three cases from our hospital, the final study cohort included 59 cases of DHG-G34m.…”

Section: Study Selectionmentioning

confidence: 99%

Neuroimaging features of diffuse hemispheric glioma, H3 G34‐mutant: A case series and systematic review

Kurokawa

Baba

Kurokawa

et al. 2021

Journal of Neuroimaging

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Background and Purpose: Diffuse hemispheric gliomas, H3 G34-mutant (DHGs-G34m), are newly recognized malignant brain tumors characterized by histone gene mutations.However, the neuroradiologic characteristics of these tumors require elucidation. We reviewed the demographic, clinical, and neuroradiological features of DHGs-G34m.Methods: Data were extracted using a database search in MEDLINE, SCOPUS, and Google Scholar in June 2021. Studies assessing pathologically proven DHGs-G34m with each patient's information and neuroradiological findings were included. After screening and reviewing 332 abstracts, 12 articles including 56 cases met the criteria. We also added the findings for three patients evaluated in our hospital. Two board-certified radiologists reviewed all demographic, clinical, and neuroradiological findings of each study. One board-certified pathologist reviewed all pathological data of each study. Kaplan-Meier analyses with log-rank tests were performed to compare the survival between patients with different tumor margin characteristics (well-delineated and ill-defined). Results:The median patient age at diagnosis was 19 years (range, 6-66 years), and 31/59 patients (52.5%) were men. Supratentorial tumors were observed in all patients (59/59, 100%). Frequent contact with leptomeninges (92.3%) and ependymal regions (87.5%) was observed. The 1-and 2-year survival rates after initial surgery were 66.7% and 40.0%, respectively. DHGs-G34m with ill-defined and well-delineated margins showed significant differences in survival (p = .04).Conclusions: DHGs-G34m occur most often in the supratentorial regions of adolescents.Prognosis varies among patients. Evaluation of tumor margins may provide prognostic value.

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Radiological and Immunostaining Characteristics of H3.3 G34R-Mutant Glioma: A Report of 3 Cases and Review of the Literature

Cited by 9 publications

References 16 publications

Imaging features associated with H3 K27-altered and H3 G34-mutant gliomas: a narrative systematic review

Imaging features associated with H3 K27-altered and H3 G34-mutant gliomas: a narrative systematic review

Systematic review of diffuse hemispheric glioma, H3 G34-mutant: Outcomes and associated clinical factors

Neuroimaging features of diffuse hemispheric glioma, H3 G34‐mutant: A case series and systematic review

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