H3K27M, IDH1, and ATRX expression in pediatric GBM and their clinical and prognostic significance

Uppar, Alok Mohan; Sugur, Harsha; Prabhuraj, A R; Rao, Malla Bhaskara; Devi, B Indira; Somanna, Sampath; Arivazhagan, Arimappamagan; Santosh, Vani

doi:10.1007/s00381-019-04222-z

Cited by 9 publications

(10 citation statements)

References 26 publications

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“…Pekmezci et al did not observe a significant difference in survival with respect to ATRX mutation status in IDH-mutant glioblastomas; however, the presence of ATRX mutation in IDH-wild type glioblastomas was associated with better survival [24]. Uppar et al reported that ATRX, IDH and p53 biomarkers did not affect prognosis in paediatric patients with glioblastomas [33]. In the present study, we found that the mean PFS and OS were longer in patients with ATRX mutation than in those without ATRX mutation.…”

Section: Discussionmentioning

confidence: 97%

The importance of IDH1, ATRX and WT-1 mutations in glioblastoma

Gülten

Yalçin

Baltalarli

et al. 2020

pjp

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Numerous genetic pathways associated with glioblastoma development have been identified. In this study, we investigated the prognostic significance of IDH1 and ATRX mutations and WT-1 and p53 expression in glioblastomas and that of surgical methods, radiotherapy and chemotherapy. 83 patients with glioblastomas were retrospectively evaluated. Immunohistochemical analysis was performed for IDH1, ATRX and WT-1 expression. Tumour cells were positive for IDH1 in 9.6% of the patients. In 4.8% of the patients, loss of ATRX expression was observed in tumour cells; 86.7% of the patients were WT-1 positive, and 12.05% of the patients were p53 positive. No statistically significant difference was found in the progression-free and overall survival according to IDH1, ATRX, WT-1 and p53 expression. There was a statistically significant difference in the progression-free and overall survival according to the radiotherapy status. There was a statistically significant difference in the overall survival according to the chemotherapy status. There was no statistically significant difference in the progression-free and overall survival according to the surgical method. IDH1 and ATRX mutations, p53 overexpression and WT-1 expression alone did not have a significant effect on the prognosis of patients with glioblastoma; however, radiotherapy and chemotherapy had a positive effect on survival.

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Section: Discussionmentioning

confidence: 97%

The importance of IDH1, ATRX and WT-1 mutations in glioblastoma

Gülten

Yalçin

Baltalarli

et al. 2020

pjp

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“…For example, brainstem gliomas are generally treated with biopsy or surgical debulking. This surgical strategy was adopted according to the high potential risk of surgical morbidity and the dismal clinical behavior of tumors located within the midline structures that carry a H3K27M mutation ( 14 , 15 ). Evidence has indicated that a greater extent of tumor resection did not provide a prognostic benefit for DMG with H3K27M mutation ( 2 ).…”

Section: Discussionmentioning

confidence: 99%

“…Given the limited treatment options for DMG, our limited data suggests that patients may have a survival benefit with adjuvant RT ( Figure 2 ). Various factors might impact the choices for the adjuvant therapy, such as socioeconomic background, education and personal expectation ( 15 ). In our cohort, we find that low postoperative KPS, lower family psychological expectation and socioeconomic consideration are main reasons behind the high RT drop-out.…”

Section: Discussionmentioning

confidence: 99%

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Clinical Features and Molecular Markers on Diffuse Midline Gliomas With H3K27M Mutations: A 43 Cases Retrospective Cohort Study

Wang

Feng

et al. 2021

Front. Oncol.

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PurposeDiffuse midline gliomas (DMG) with H3K27M mutations have been identified as a rare distinctive entity with unique genetic features, varied molecular alterations, and poor prognosis. The current study aimed to evaluate the clinical characteristics and profile of molecular markers on patients with a DMG harboring H3K27M mutations, and explore the impact of this genetic makeup on overall survival.MethodsWe retrospectively analyzed 43 consecutive patients diagnosed with a DMG harboring H3K27M mutations (age range 3 to 75 years) and treated in a tertiary institution within China between January 2017 to December 2019. Various clinical and molecular factors were evaluated to assess their prognostic value in this unique patient cohort.ResultsThe median overall survival (OS) was 12.83 months. Preoperative Karnofsky Performance Score (KPS) and adjuvant radiotherapy were found to be independent clinical parameters influencing the OS by multivariate analysis (p = 0.027 and p < 0.001 respectively). Whereas extent of tumor resection failed to demonstrate statistical significance. For molecular markers, P53 overexpression was identified as a negative prognostic factor for overall survival by multivariate analysis (p = 0.030).ConclusionLow preoperative KPS, absence of radiotherapy and P53 overexpression were identified as predictors of a dismal overall survival in patients with DMG and H3K27M mutations.

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“…This could provide better intraoperative decision-making about extent of resection. 21,22 Radiotherapy at the dose of 50 to 60 grays fractionated over 6 weeks is a significant compartment of treatment in PHGG. 5 Radiotherapy usually is not used in children younger than 3 years due to its adverse effect on the developing brain.…”

Section: Discussionmentioning

confidence: 99%

Pediatric Non–Brain Stem High-Grade Glioma: A Single-Center Experience

Alimohammadi

Bagheri

Delfani

et al. 2020

Indian Journal of Neurosurgery

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Background Pediatric high-grade gliomas (PHGGs) consist of a heterogeneous class of central nervous system (CNS) neoplasms with a poor prognosis. We aimed to present our 10-year experience in the management of children with high-grade glioma focusing on patients’ survival and related factors. Methods All pediatric patients with high- grade glioma (HGG) who were admitted to our center between May 2009 and May 2018 were investigated. Overall survival (OS) was calculated from the time of diagnosis until the day of death. The impact of suggested variables on survival was evaluated using the univariate and multivariate analyses. Results There were 41 children with non–brain stem high-grade glioma (NBSHGG). The mean OS of patients was 21.24 ± 10.16 months. The extent of resection (p = 0.002, hazard ratio [HR] = 4.84), the grade of the tumor (p = 0.017, HR = 4.36), and temozolomide (TMZ) therapy (p = 0.038, HR = 3.57) were the independent predictors of OS in children with NBSHGG. Age, gender, tumor location, and size of tumor were not associated with the survival of these patients. Conclusion HGGs are uncommon pediatric tumors with an aggressive nature and a poor prognosis. Our results revealed that in NBSHGG cases, children with maximal safe tumor resection and children that received temozolomide therapy as well as children with grade III of the tumor had higher survival.

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H3K27M, IDH1, and ATRX expression in pediatric GBM and their clinical and prognostic significance

Cited by 9 publications

References 26 publications

The importance of IDH1, ATRX and WT-1 mutations in glioblastoma

The importance of IDH1, ATRX and WT-1 mutations in glioblastoma

Clinical Features and Molecular Markers on Diffuse Midline Gliomas With H3K27M Mutations: A 43 Cases Retrospective Cohort Study

Pediatric Non–Brain Stem High-Grade Glioma: A Single-Center Experience

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