Midline Glioma in Adults: Clinicopathological, Genetic, and Epigenetic Analysis

Enomoto, Toshiyuki; Aoki, Mikiko; Hamasaki, Makoto; Abe, Hiroshi; Nonaka, Masani; Inoue, Takeshi; Nabeshima, Kazuki

doi:10.2176/nmc.oa.2019-0168

Cited by 30 publications

(39 citation statements)

References 28 publications

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“…The same phenomenon was also founded in some study concerning H3K27M mutated DMG in adults 9,10 . From 30 cases combined with the 171 cases reported in literature, Toshiyuki et al found that the status of H3K27M is not related to the prognosis in patients who are older than 40 11 . However, another research reported that H3K27M does not correlate with the prognoses of adults 12 .…”

Section: Discussionmentioning

confidence: 99%

A Clinicopathological Analysis on Diffuse Midline Glioma in Adults

Nie

et al. 2021

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Purpose: Diffuse midline glioma (DMG), H3K27M mutant is a new entity that has become widely recognized. However, studies concerning DMG in adult patients remains rare. We did a retrospective study covering the largest amount of patients to date to analyze the clinicopathological characteristics of DMG in adult. Methods: We reviewed 117 cases of adult DMG, collected their clinical and imaging data along with pathological results including H3K27M. Summarized their features and the connection with overall survival in different age groups.Results: Among 117 cases, most tumors were located at the thalamus, 39 patients had H3K27M mutation, of whom 38 demonstrated down regulation of H3K27me3. The average overall survival of H3K27M-mutant gliomas was 13 months, while that of 78 H3K27M wild-type gliomas were 11.8 months. For young patients (age＜35), The median survival time of the H3K27M-mutant was 20.1 months, while that of the H3K27M wild-type was 39.5 months. For older patients (age≥35), the median survival time of the H3K27M-mutant was 22.3 months, while that of the H3K27M wild-type was 17.1 months. The OS of patients who received biopsies, subtotal resections, and total resections were 15.8, 17.6, and 11.6 months respectively. Conclusion: The DMG in adults mainly occurred in the thalamus. H3K27M mutations tend to happen more frequently in young adults, and this genetic alteration results in a worse outcome only in young patients. For old patients, age and the approach of surgery are independent prognostic factors. Patients received biopsy instead of total resection had a better prognosis.

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Section: Discussionmentioning

confidence: 99%

A Clinicopathological Analysis on Diffuse Midline Glioma in Adults

Nie

et al. 2021

Preprint

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“…The willingness to biopsy midline glioma patients has a direct bearing on our ability to understand their biology. Based on samples from these lesions, the histone H3 K27M somatic mutation has been identified in pediatric and adult tumors [13,18]. This has led to a new WHO 2016 classification of "diffuse midline glioma, H3 K27M-mutant" [19].…”

Section: Discussionmentioning

confidence: 99%

Variations in attitudes towards stereotactic biopsy of adult diffuse midline glioma patients: a survey of members of the AANS/CNS Tumor Section

et al. 2020

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Purpose Diffuse midline gliomas are rare midline CNS malignancies that primarily affect children but can also affect adults. While radiation is standard treatment, prognosis remains fatal. Furthermore, due to its sensitive anatomic location, many physicians have been reluctant to perform biopsies without potential for improved prognosis. However, recent advancements in molecular-targeted therapeutics have encouraged greater tissue sampling. While the literature reflects this progress, the landscape of how clinicians actually manage these patients remains unclear. Our goal was to assess the attitudes of current practicing neurosurgical oncologists towards management of adult diffuse midline gliomas, reasons behind their practices, and factors that might influence these practices. Methods We created and distributed a survey with 16 multiple choice and open-ended questions to members of the Tumor Section of the Congress of Neurological Surgeons. Results A total of 81 physicians responded to the survey. Although time since training and volume of glioma patients did not significantly affect the decision to consider clinical trials or to offer biopsy, those that operated on fewer gliomas (< 25/ year) were more likely to cite surgical morbidity as the primary reason not to biopsy these midline locations. Further, surgeons with access to more advanced molecular testing were significantly more likely to consider clinical trial eligibility when offering biopsies. Conclusion Factors that affect the management of diffuse midline gliomas and the role of biopsy are relatively uniform across the field, however, there were a few notable differences that reflect the changes within the neuro-oncology field in response to clinical trials.

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“…Molecular analysis revealed that some 70-80% of DIPGs carry H3 K27M mutation, and are typically IDH-wildtype [11,21]. Tumours harbouring this mutation have remarkably poor prognosis despite treatment with adequate chemotherapy (temozolomide) and radiotherapy [11,15]; though this association in adult cases has recently been questioned [7,18]. The poorer prognosis compared to H3 wildtype gliomas may be related to the specific point mutation resulting in gliomagenesis through epigenetic changes.…”

Section: Discussionmentioning

confidence: 99%

A longitudinally extensive H3 K27M-mutant diffuse midline glioma in an elderly patient clinically mimicking central nervous system inflammation: a case report

Babarczy¹,

Reisz²,

Szabó³

et al. 2020

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Diffuse midline gliomas, H3 World Health Organization (WHO) grade IV represent a distinct glioma entity with a predominantly paediatric presentation and remarkably poor prognosis. This report presents a case of a 73-year-old woman with a diffuse midline glioma, H3 K27M-mutant, WHO grade IV with a remarkable longitudinal extension, extending from the cervical myelon to the basal ganglia. On imaging, the lesion was predominantly suggestive of inflammatory oedema, and it was clinically associated with progressive hemi-and later tetraparesis with severe autonomic and bulbar symptoms. Laboratory examinations suggested a generalized inflammatory process; however, neither infectious nor autoimmune aetiology could be confirmed. Biopsy was deemed unfeasible given the critical localization. Presuming a seronegative autoimmune encephalomyelitis, high-dose corticosteroid therapy and plasma exchanges were conducted, resulting in a modest but transient relief. The patient passed away two months after hospitalization. Neuropathological examination of the lesion revealed a high-grade diffuse glioma with H3 K27M mutation (grade IV). Although originally considered as a paediatric entity, our case confirms reports from recent years that diffuse midline gliomas, H3 K27M-mutant, WHO grade IV can occur in adults, even among the elderly, and can mimic inflammatory alterations, posing diagnostic difficulty. Our case is one of the oldest patients reported with this pathology, the oldest with an extensive diffusely infiltrating growth pattern, and with the most extensive lesion reported in adulthood.

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Midline Glioma in Adults: Clinicopathological, Genetic, and Epigenetic Analysis

Cited by 30 publications

References 28 publications

A Clinicopathological Analysis on Diffuse Midline Glioma in Adults

A Clinicopathological Analysis on Diffuse Midline Glioma in Adults

Variations in attitudes towards stereotactic biopsy of adult diffuse midline glioma patients: a survey of members of the AANS/CNS Tumor Section

A longitudinally extensive H3 K27M-mutant diffuse midline glioma in an elderly patient clinically mimicking central nervous system inflammation: a case report

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