Malignant Nervous System Neoplasm

An MRI of 26-year-old man presenting with motor weakness and language difficultly revealed a lesion in the left frontal lobe that extended across the corpus callosum. Histologic analysis of the biopsy… (full text at CIViC)

PMID 34041811 · Shepherd et al., 2021 · Open in CIViC

Phase II trial of sunitinib in 40 patients with first recurrence of primary glioblastoma. Median PFS 2.2 mo, median OS 9.2 mo, 5 stable disease >=6 months, no objective responses, so primary study end… (full text at CIViC)

PMID 24311637 · Hutterer et al., 2014 · Open in CIViC

A retrospective study of 178 glioblastoma patients found that LRP1B deletion (complete transcript, N=4 or focal transcript deletion of >150kb, N=13) was associated with a lower progress free survival … (full text at CIViC)

PMID 26428308 · Tabouret et al., 2015 · Open in CIViC

Through the analysis of 10 patient-derived glioblastoma cells, AMG232 was found to have increased sensitivity in comparison to RG7112 with the average IC50 of 76nM vs. 720nM; thus indicating a 9.5 fol… (full text at CIViC)

PMID 30022047 · Her et al., 2018 · Open in CIViC

A 62 year old female was diagnosed with glioblastoma (GBM) WHO grade 4. Treatment included radiation, temozolomide and cediranib. MET amplification was found by FISH, and ALK and MET inhibitor crizoti… (full text at CIViC)

PMID 22162573 · Chi et al., 2012 · Open in CIViC

An international multi-institutional study identified108 patients with a methylation profile of supratentorial ependymoma, ZFTA fusion-positive, by whole genome DNA methylation profiling. The study fo… (full text at CIViC)

PMID 37287693 · Ng et al., 2023 · Open in CIViC

In a series of 54 cases of ependymoma diagnosed in children </= 3 years, methylation profiling of all tumours was undertaken as part of a prospective clinical trial (SJYC07 - maximal safe surgical res… (full text at CIViC)

PMID 30976811 · Upadhyaya et al., 2019 · Open in CIViC

In a trial of 68 glioblastoma patients treated with either temozolomide or semustine, MGMT expression was found to be inversely correlated with survival. Patients in the MGMT high expression group (n=… (full text at CIViC)

PMID 24248532 · Wang et al., 2014 · Open in CIViC

In patients with glioblastoma, those with methylated MGMT promoters have better overall survival than those with unmethylated promoters.

PMID 15758010 · Hegi et al., 2005 · Open in CIViC

MGMT promoter methylation was associated with increased overall and disease-free survival as well as tumor regression in patients with gliomas.

PMID 11070098 · Esteller et al., 2000 · Open in CIViC

The T allele of the rs16906252 SNP was associated with MGMT methylation and low protein expression. Germline presence of the T allele increased the risk of glioblastoma development (adjusted odds rati… (full text at CIViC)

PMID 25910840 · Rapkins et al., 2015 · Open in CIViC

In this study, patients with low MGMT expression, compared with patients with high MGMT expression, had a significantly higher response rate (55% v 7%, respectively; P = .004) and improved PFS (median… (full text at CIViC)

PMID 17442989 · Chinot et al., 2007 · Open in CIViC

In 41 glioblastomas treated with temozolomide (TMZ) therapy (MGMT-immunopositive: n = 22, negative: n = 19), both progression free survival and overall survival were significantly better in MGMT-immun… (full text at CIViC)

PMID 21331613 · Watanabe et al., 2011 · Open in CIViC

The expression of a set of miRNAs was evaluated by qRT-PCR in 124 astrocytoma (grade I-IV) samples and 60 adjacent normal tissue samples in order to determine miRNAs with altered expression in astrocy… (full text at CIViC)

PMID 20219352 · Zhi et al., 2010 · Open in CIViC

In an analysis of 13 cases of supratentorial tumors with ZFTA rearrangements without RELA involvement, all were found to have DNA methylation profiles similar to ZFTA::RELA supratentorial ependymoma. … (full text at CIViC)

PMID 34389065 · Tauziède-Espariat et al., 2021 · Open in CIViC

Group 3 medulloblastomas with MYC amplifications are often associated with large cell/anaplastic histology, young patients, and overall poor prognosis.

PMID 15328187 · Lamont et al., 2004 · Open in CIViC

In a cohort of 1596 diagnostic tumor samples taken from patients enrolled in the Children's oncology group (COG) neuroblastoma biology protocol ANBL00B1, 1579 samples were annotated with MYCN amplific… (full text at CIViC)

PMID 25517749 · Bresler et al., 2014 · Open in CIViC

In a patient derived xenograft from a sonic hedgehog driven medulloblastoma patient, MYCN amplification conferred to resistance to SMO inhibition with Sonidegib. This PDX was shown to be responsive t… (full text at CIViC)

PMID 23291299 · Kim et al., 2013 · Open in CIViC

In a Children's Oncology Group correlative study, 242 pretherapy tumours from patients enrolled on the phase III high-risk neuroblastoma trial ANBL0532 (NCT00567567) underwent ultra-deep targeted sequ… (full text at CIViC)

PMID 40036726 · Berko et al., 2025 · Open in CIViC

A preclinical study investigated the sensitivity of PARP inhibitors in Neuroblastoma with MYCN-amplified (IMR-32) cells and found a decrease in cellular viability in comparison to the non-MYCN amplifi… (full text at CIViC)

PMID 32577161 · King et al., 2020 · Open in CIViC

Low NF1 protein expression correlated positively with responses to binimetinib in neuroblastoma cell lines.

PMID 26925841 · Woodfield et al., 2016 · Open in CIViC

Sporadic and Neurofibromatosis Type 1 (NF1) related refractory Malignant Peripheral Nerve Sheath Tumors (MPNST) showed no significant clinical benefit when treated with Everolimus in Combination with … (full text at CIViC)

PMID 31427883 · Widemann et al., 2019 · Open in CIViC

This study reported 47 neurofibromatosis type 1 (NF1) patients with biallelic inactivation of NF1 genes were detected in all glial tumors. Targeted capture-based next-generation sequencing (479 cancer… (full text at CIViC)

PMID 35945463 · Lucas et al., 2022 · Open in CIViC

This study reported 47 neurofibromatosis type 1 (NF1) patients with biallelic inactivation of NF1 genes were detected in all glial tumors. Targeted capture-based next-generation sequencing (479 cancer… (full text at CIViC)

PMID 35945463 · Lucas et al., 2022 · Open in CIViC

RNAseq data of 162 glioblastoma patients identified NTRK1 fusions with both NFASC and BCABN. By analyzing exome and RNA data, the NFASC-NTRK1 fusion was found to retain NTRK1's kinase and transmembran… (full text at CIViC)

PMID 24647444 · Kim et al., 2014 · Open in CIViC

In-vitro study in 5 neuroblastoma cell lines (2 with NRAS Q61K mutation). The combination of mTOR and MEK Inhibitors synergistically blocked cell growth in NRAS mutant but not wild type cell lines. Si… (full text at CIViC)

PMID 26821351 · Kiessling et al., 2016 · Open in CIViC

A high level of expression of the TRK proto-oncogene in a neuroblastoma is strongly predictive of a favorable outcome. A tumor with a functional nerve growth factor receptor may be dependent on the ne… (full text at CIViC)

PMID 8441429 · Nakagawara et al., 1993 · Open in CIViC

Patients with tumors expressing high levels of trkC mRNA had significantly longer intervals without disease progression than those with low levels (log-rank, P = 0.03) and a more favorable overall sur… (full text at CIViC)

PMID 7809137 · Segal et al., 1994 · Open in CIViC

The majority of MPNST samples were positive for PARP staining. Overall, moderate to high expression of PARP1 and PARP2 was observed (Table 1)....Overall, treatment continued for 60 d at which point 5 … (full text at CIViC)

PMID 26650448 · Kivlin et al., 2016 · Open in CIViC

In the STARTRK-NG Phase 1/2 trial of entrectinib in pediatric patients, with extracranial solid tumors or primary CNS tumors, aged <22 years with relapsed or refractory disease, tumors with fusions… (full text at CIViC)

PMID 35395680 · Desai et al., 2022 · Open in CIViC

The paper showed that PCNA (p.K164R) inhibits the ubiquitination of PCNA and the variant can render cells sensitive to DNA-damaging agents and reduce activation-induced single-nucleotide substitutions… (full text at CIViC)

PMID 17105346 · Arakawa et al., 2006 · Open in CIViC

In this multicenter, open-label, multi-arm, phase 2 trial, 65 patients with PI3K pathway aberrations (defined by PTEN and pAKT IHC-Status in 95%, genomic testing in 5%) were treated with the PI3K inhi… (full text at CIViC)

PMID 30715997 · Wen et al., 2019 · Open in CIViC

In this multicenter, open-label, multi-arm, phase 2 trial, 65 patients with PI3K pathway aberrations (defined by PTEN and pAKT IHC-Status in 95%, genomic testing in 5%) were treated with the PI3K inhi… (full text at CIViC)

PMID 30715997 · Wen et al., 2019 · Open in CIViC

Kaplan-Meier analysis of PLK1 gene expression showed that elevated expression is significantly associated with shorter disease-free survival in GBM (p=9.1e-11).

PMID 32180229 · Li et al., 2020 · Open in CIViC

Two siblings (6 and 3.5 years) from nonconsanguineous parents were diagnosed with glioblastoma multiforme. Both tumors harbored TP53 mutations and lack of MGMT promoter hypermethylation as poor progno… (full text at CIViC)

PMID 27001570 · Bouffet et al., 2016 · Open in CIViC

Case report of a patient with glioblastoma multiforme and POLE germline mutation. The patient was treated with pembrolizumab and lymphocyte infiltration was noted after resection of a spinal metastasi… (full text at CIViC)

PMID 27683556 · Johanns et al., 2016 · Open in CIViC

In a sonic hedghehog driven medulloblastoma patient derived xenograft harbouring a PTCH1 deletion, SMO inhibition with Sonidegib showed inhibition of proliferation.

PMID 24651015 · Kool et al., 2014 · Open in CIViC

Loss-of-heterozygosity in SHH-driven medulloblastoma was associated with increased PFS under therapy with vismodegib (AKA HhAntag691, GDC0449, GDC-0449). 43 patients total were enrolled for this study… (full text at CIViC)

PMID 26169613 · Robinson et al., 2015 · Open in CIViC

133 sonic-hedghog driven medulloblastomas were sequenced for alterations (WGS or WES). 60 cases exhibited PTCH1 mutations, most of which were likely loss-of-function (spread across the gene and of typ… (full text at CIViC)

PMID 24651015 · Kool et al., 2014 · Open in CIViC