Gliomas in Children

Filbin, Mariella G.; Sturm, Dominik

doi:10.1055/s-0038-1635106

Cited by 17 publications

(8 citation statements)

References 79 publications

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“…However, a large proportion are high-grade gliomas (HGGs), such as diffuse intrinsic pontine glioma (DIPG) (73,74). Despite treatment, these tumors invariably progress rapidly, with a median survival of less than 1 year (75). Medulloblastomas are aggressive embryonal tumors that account for approximately 10%-15% of pediatric brain tumors (70,72).…”

Section: Immune Profiling Of Pediatric Solid Tumorsmentioning

confidence: 99%

Immune profiling of pediatric solid tumors

Terry¹,

Meyran²,

Ziegler³

et al. 2020

Journal of Clinical Investigation

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text of cancer, this may mean that tumor-specific T cell clones are sequestered. This has been shown in preclinical studies in melanoma, in which anti-RANKL rescued melanoma-specific T cells and enhanced anti-PD-1 (124). There are several clinical trials testing anti-RANKL plus anti-PD-1 in melanoma and non-small cell lung carcinoma (Supplemental Table 1), but no pediatric trials. This combination could potentially be very effective in pediatric osteosarcoma.Overall, many clinical trials are combining immune checkpoint inhibitors with immunomodulatory agents to improve response in adult cancers, but few trials in children. Once the safety and efficacy of these combinations are established, they will likely be extended to pediatric malignancies. This will result in new treatment combinations for childhood cancers that otherwise would be nonresponsive to immunotherapies, and will meaningfully improve patient outcomes.

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Section: Immune Profiling Of Pediatric Solid Tumorsmentioning

confidence: 99%

Immune profiling of pediatric solid tumors

Terry¹,

Meyran²,

Ziegler³

et al. 2020

Journal of Clinical Investigation

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“…Children with low grade gliomas (WHO grade 1 and 2) have an excellent prognosis when these lesions can be totally resected, but often require adjuvant therapy when gross total resection cannot be achieved. Children with high grade gliomas (WHO grade 3 and 4) have a poor prognosis, despite aggressive multimodal therapy and no standard therapy, other than surgical resection and radiotherapy, has been established [6].…”

Section: Introductionmentioning

confidence: 99%

Phase I study of vemurafenib in children with recurrent or progressive BRAFV600E mutant brain tumors: Pacific Pediatric Neuro-Oncology Consortium study (PNOC-002)

et al. 2020

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Background: BRAF V600E mutation is present in a subset of pediatric brain tumors. Vemurafenib is an oral, selective ATP-competitive inhibitor of BRAF V600E kinase. The goal of this multi-center study conducted through the Pacific Pediatric Neuro-Oncology Consortium (PNOC) was to determine the recommended phase 2 dose (RP2D) and dose limiting toxicities (DLTs) in children < 18 years with recurrent or progressive BRAF V600E mutant brain tumors. Results: Nineteen eligible patients were enrolled. Eleven patients had received three or more prior therapies. Data reported are from the start of treatment for the first patient (April 30 2014) through August 31 2019. The RP2D was defined as 550 mg/m 2 twice daily after DLT criteria adjustment for rash. Related grade ≥ 3 adverse events included secondary keratoacanthoma (n = 1); rash (n =16); and fever (n = 5). Subjects received a median of 23 cycles (range 3-63). Four patients remain www.oncotarget.com

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“…Glioma acts as a common malignant primary brain tumor, and the significant progress of related treatment in the past ten years fails to improve its weak prognosis 22 , 23 . The early screening and the prediction of clinical outcomes before various treatments may contribute to the optimization of therapeutic schedules for doctors, which then promoted the long-term survivals of glioma patients 24 , 25 .…”

Section: Discussionmentioning

confidence: 99%

Diagnostic and Prognostic Potentials of Long Noncoding RNA ELF3-AS1 in Glioma Patients

Mei

2020

Disease Markers

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Objective. Accumulating evidence implies that long noncoding RNAs (lncRNAs) play a crucial role in predicting survival for glioma patients. However, the potential function of lncRNA ELF3-antisense RNA 1 (ELF3-AS1) in tumors remained largely unclear. The aim of this study was to explore the expression of lncRNA ELF3-antisense RNA 1 (ELF3-AS1) and evaluate its functions in glioma patients. Patients and Methods. ELF3-AS1 expressions were examined by RT-PCR in 182 pairs of glioma specimens and adjacent normal tissues. The receiver operating characteristic (ROC) curve was performed to estimate the diagnostic value of ELF3-AS1. The chi-square tests were used to examine the associations between ELF3-AS1 expression and the clinicopathological characters. The overall survival (OS) and disease-free survival (DFS) were analyzed by log-rank test, and survival curves were plotted according to Kaplan-Meier. The prognostic value of the ELF3-AS1 expression in glioma patients was further analyzed using univariate and multivariate Cox regression analyses. Loss-of-function assays were performed to determine the potential function of ELF3-AS1 on the proliferation and invasion of glioma cells. Results. The ELF3-AS1 expression level was significantly higher in glioma specimens compared with adjacent nontumor specimens (p<0.01). A high expression of ELF3-AS1 was shown to be associated with the WHO grade (p=0.023) and KPS score (p=0.012). ROC assays revealed that high ELF3-AS1 expression had an AUC value of 0.8073 (95% CI: 0.7610 to 0.8535) for glioma. Using the Kaplan-Meier analysis, we found that patients with a high ELF3-AS1 expression had significantly poor OS (p=0.006) and DFS (p=0.0002). In a multivariate Cox model, we confirmed that ELF3-AS1 expression was an independent poor prognostic factor for glioma patients. The functional assay revealed that knockdown of ELF3-AS1 suppressed the proliferation and invasion of glioma cells. Conclusions. Our findings confirmed that ELF3-AS1 functions as an oncogene in glioma and indicated that ELF3-AS1 is not only an important prognostic marker but also a potential therapy target for glioma.

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Gliomas in Children

Cited by 17 publications

References 79 publications

Immune profiling of pediatric solid tumors

Immune profiling of pediatric solid tumors

Phase I study of vemurafenib in children with recurrent or progressive BRAFV600E mutant brain tumors: Pacific Pediatric Neuro-Oncology Consortium study (PNOC-002)

Diagnostic and Prognostic Potentials of Long Noncoding RNA ELF3-AS1 in Glioma Patients

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