Cancer Family
Neuroepithelial, Perineurial, and Schwann Cell Neoplasm
CI-CAN-00000039Explore in graph →
Non-malignant864 active trials6 approved drugs159 evidence items
- NCIt
- C35562
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Cancer Family
CI-CAN-00000039Explore in graph →
Variants & evidence
380 evidence items mapped to this entity or its descendants, grouped by molecular profile, then therapy. 50 items per page.
| Therapy | Cancer | Type | Level | Direction · significance | Rating (1–5) | Status | Evidence | Source |
|---|---|---|---|---|---|---|---|---|
| H3-3A K282 | ||||||||
| (diagnostic) | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | B | Supports Positive | 3 | submitted | EID9608In this retrospective study, 112 adolescents and young adults (AYA; between 15 and 25 years of age; median 21 years) who had a histopathological diagnosis of high-grade gliomas were analyzed. The H3K2… (full text at CIViC) PMID 32025728 · Roux et al., 2020 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | submitted | EID9812Sturm, et al. (2012) analyzed 3 subgroups of glioblastoma samples using different methods: by sequencing (460 samples), by methylation profiling (59 pediatric and 77 adult samples), and with tissue-mi… (full text at CIViC) PMID 23079654 · Sturm et al., 2012 · Open in CIViC | civic |
| H3-3A K28M27 | ||||||||
| (diagnostic) | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | A | Supports Positive | 5 | submitted | EID8626Diffuse midline gliomas, H3 K27M-mutant is a newly defined entity in the 2016 WHO Classification. It primarily occurs in children (but sometimes in adults) and include tumors previously referred to as… (full text at CIViC) PMID 27157931 · Louis et al., 2016 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 4 | submitted | EID6449Of a set of 338 pediatric brain tumors, Gielen et al identified the K27M mutation in 35 of 129 glioblastomas (27.1%) and in 5 of 28 (17.9%) anaplastic astrocytomas. The authors state that H3F3A K27M c… (full text at CIViC) PMID 23429371 · Gielen et al., 2013 · Open in CIViC | civic |
| 〃 | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | B | |||||
| H3-3A K28R2 | ||||||||
| (diagnostic) | Anaplastic AstrocytomaCURATED_BROADER | Diagnostic | C | Supports Positive | 1 | submitted | EID9836Yoshimoto, et al. (2017) analyzed 411 gliomas of Japanese patients (median age of 46 years (ranged between 1 to 87 years)). Direct sequencing revealed H3F3A p.K27R in one 37 years of age anaplastic as… (full text at CIViC) PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC | civic |
| 〃 | OligodendrogliomaCURATED_BROADER | Diagnostic | C | Supports Positive | 1 | submitted | EID9838Yoshimoto, et al. (2017) analyzed 411 gliomas of Japanese patients (median age of 46 years (ranged between 1 to 87 years)). Direct sequencing revealed H3F3A p.K27R in one 51 years of age oligodendrogl… (full text at CIViC) PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC | civic |
| H3-3A MUTATION2 | ||||||||
| (prognostic) | Childhood Low Grade Glioma | Prognostic | B | Supports Poor Outcome | 4 | accepted | EID7189Using Sanger sequencing, H3F3A mutations in patients with pediatric low-grade gliomas (PLGGs) were identified (K27M: 13/280; G34R: 5/280). Kaplan-Meier survival analysis showed that H3F3A mutations we… (full text at CIViC) PMID 29948154 · Yang et al., 2018 · Open in CIViC | civic |
| (diagnostic) | Glioma | Diagnostic | B | Supports Positive | 4 | submitted | EID9417Whole exome sequencing was performed on the glioblastomas of 48 patients (between 3 and 20 years of age). The authors also analyzed gliomas of 784 patients of various ages. The G34R/G34V variant has b… (full text at CIViC) PMID 22286061 · Schwartzentruber et al., 2012 · Open in CIViC | civic |
| H3-3B K28I1 | ||||||||
| (oncogenic) | Diffuse Midline Glioma, H3 K27-Altered | Oncogenic | C | Supports Oncogenicity | 2 | submitted | EID13073Two adult patients with diffuse midline glioma (DMG) harboring a somatic H3F3B K27I mutation were reported. Next-generation sequencing on postoperative tissue was performed using a 539-gene panel (Si… (full text at CIViC) | civic |
| H3C14 K27M1 | ||||||||
| (diagnostic) | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | C | Supports Positive | 1 | submitted | EID10611Molecular characterisation of a series of 62 diffuse midline gliomas, H3 K27-altered, revealed a single case of a novel histone H3 variant - H3C14 (HIST2H3C) p.K28M (K7M). The variant occurred in the … (full text at CIViC) PMID 26399631 · Castel et al., 2015 · Open in CIViC | civic |
| H3C2 K27M7 | ||||||||
| (diagnostic) | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | B | Supports Positive | 3 | accepted | EID9546Whole-genome sequencing (WGS) was performed on the diffuse intrinsic pontine gliomas (DIPGs) and matched germline DNA of 7 pediatric patients. Targeted sequencing was performed on an additional 43 DIP… (full text at CIViC) PMID 22286216 · Wu et al., 2012 · Open in CIViC | civic |
| (oncogenic) | Diffuse Midline Glioma, H3 K27-Altered | Oncogenic | B | Supports Oncogenicity | 2 | accepted | EID13177The recurrent HIST1H3B (now known as H3C2, encoding H3.1) p.K27M mutation was identified in human diffuse intrinsic pontine glioma (DIPG) samples and was associated with a significant global reduction… (full text at CIViC) PMID 23539183 · Lewis et al., 2013 · Open in CIViC | civic |
| (diagnostic) | Anaplastic AstrocytomaCURATED_BROADER | Diagnostic | C | Supports | ||||
| H3C4 K28M1 | ||||||||
| (oncogenic) | Posterior Fossa Ependymoma, Group A (PFA)ALIAS | Oncogenic | C | Supports Oncogenicity | 2 | accepted | EID13074Mariet et al. identified a somatic H3C4 (HIST1H3D) p.Lys28Met (K27M) mutation in a posterior fossa group A ependymoma (EPN_PFA) from an 8-year-old male. The tumor showed H3 K27M immunoreactivity, loss… (full text at CIViC) PMID 36104744 · Mariet et al., 2022 · Open in CIViC | civic |
| HDAC9 p.L33R1 | ||||||||
| (diagnostic) | GlioblastomaCURATED_BROADER | Diagnostic | D | Supports Positive | 4 | submitted | EID9240The study uses HDAC inhibitors, 4-PB and TSA, have differential effects on growth, differentiation, and apoptosis in glioma cells. The authors perform TUNNEL assay and used immunofluorescence to show … (full text at CIViC) PMID 18360709 · Svechnikova et al., 2008 · Open in CIViC | civic |
| HGF EXPRESSION1 | ||||||||
| MET Tyrosine Kinase Inhibitor SGX523 | GlioblastomaCURATED_BROADER | Predictive | D | Supports Sensitivity Response | 2 | accepted | EID1707Three metastatic glioblastoma cell lines were compared with their parental cell lines with regard to differential gene expression. HGF signalling was among the top pathways identified. Exogenous HFG l… (full text at CIViC) PMID 22203985 · Xie et al., 2012 · Open in CIViC | civic |
| HOXC10 Overexpression2 | ||||||||
| (prognostic) | Glioma | Prognostic | B | Supports Poor Outcome | 4 | submitted | EID10147Public glioma datasets were analyzed from The Cancer Genome Atlas (TCGA) and the Gene Expression Omnibus. HOXC10 was seen to be significantly elevated in gliomas compared to non-tumor brain tissues, a… (full text at CIViC) PMID 30429891 · Tan et al., 2018 · Open in CIViC | civic |
| Bevacizumab | Glioma | Predictive | D | Supports Sensitivity Response | 3 | submitted | EID9949In determining the mechanisms of angiogenesis in gliomas, an in-depth preclinical analysis of HOXC10 overexpression in response to a vascular endothelial growth factor A (VEGFA) inhibitor bevacizumab … (full text at CIViC) PMID 30429891 · Tan et al., 2018 · Open in CIViC | civic |
| HSP90B1 Overexpression1 | ||||||||
| Onalespib + TemozolomideCombination | Glioma | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID10153In vivo overexpression of HSP90 was observed in segments of the glioma-containing tissue versus the non-tumor-bearing areas in the forebrain of nude mice implanted with bioluminescent intracranial gli… (full text at CIViC) PMID 28679777 · Canella et al., 2017 · Open in CIViC | civic |
| IDH1 R1322 | ||||||||
| (prognostic) | GlioblastomaCURATED_BROADER | Prognostic | B | Supports Better Outcome | 5 | accepted | EID480In patients with a glioblastoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients. PMID 19228619 · Yan et al., 2009 · Open in CIViC | civic |
| 〃 | Anaplastic AstrocytomaALIAS | Prognostic | B | Supports Better Outcome | 5 | accepted | EID481In patients with anaplastic astrocytoma, those with mutations at R132 of IDH1 had better overall survival than wild-type patients. PMID 19228619 · Yan et al., 2009 · Open in CIViC | civic |
Data updated 21 hours agoSource updated unknownsource: civic (CC0)
Evidence levels, directions and ratings are those assigned by CIViC curators. "Submitted" items have not completed curation review. This is not treatment guidance.
| 4 |
| submitted |
EID9414Whole exome sequencing was performed on the glioblastomas of 48 pediatric patients (between 3 and 20 years of age). The authors also analyzed gliomas of 784 patients of various ages. The K27M variant … (full text at CIViC) PMID 22286061 · Schwartzentruber et al., 2012 · Open in CIViC |
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| (oncogenic) | Diffuse Midline Glioma, H3 K27-Altered | Oncogenic | B | Supports Oncogenicity | 4 | submitted | EID10600In this study, 91 patients with diffuse intrinsic pontine glioma (DIPG) underwent stereotactic biopsy and were analyzed using histone H3 mutation testing, immunohistochemistry, gene expression profili… (full text at CIViC) PMID 26399631 · Castel et al., 2015 · Open in CIViC | civic |
| (diagnostic) | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | B | Supports Positive | 3 | accepted | EID6450Wu et al analyzed 127 pediatric HGGs, including diffuse intrinsic pontine gliomas (DIPGs) and non-brainstem HGGs (NBS-HGGs) by whole genome, whole exome, and/or transcriptome sequencing. In addition t… (full text at CIViC) PMID 24705251 · Wu et al., 2014 · Open in CIViC | civic |
| 〃 | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | B | Supports Positive | 3 | accepted | EID6453Whole genome sequencing (WGS) was performed on the diffuse intrinsic pontine gliomas (DIPGs) and matched germline DNA of 7 pediatric patients. Targeted sequencing was performed on an additional 43 DIP… (full text at CIViC) PMID 22286216 · Wu et al., 2012 · Open in CIViC | civic |
| 〃 | Anaplastic AstrocytomaCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | submitted | EID9800Kleinschmidt-DeMasters, et al. (2018) analyzed the tumor database for patients with the H3 K27M variant and found 28 cases (including 3 meeting WHO grade I and 25 meeting WHO grade II-IV criteria). Th… (full text at CIViC) PMID 29393845 · Kleinschmidt-DeMasters et al., 2018 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | submitted | EID9801Kleinschmidt-DeMasters, et al. (2018) analyzed the tumor database for patients with the H3 K27M variant and found 28 cases (including 3 meeting WHO grade I and 25 meeting WHO grade II-IV criteria). Th… (full text at CIViC) PMID 29393845 · Kleinschmidt-DeMasters et al., 2018 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | rejected | EID9816Sturm, et al. (2012) analyzed 3 subgroups of glioblastoma samples using different methods: by sequencing (460 samples), by methylation profiling (59 pediatric and 77 adult samples), and with tissue-mi… (full text at CIViC) PMID 23079654 · Sturm et al., 2012 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | submitted | EID9824Yoshimoto, et al. (2017) analyzed 411 gliomas of Japanese patients (median age of 46 years (ranged between 1 to 87 years)). Direct sequencing revealed H3F3A p.K27M in 10 glioblastoma cases (median age… (full text at CIViC) PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | submitted | EID9850Buczkowicz, et al. (2014) analyzed 74 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age at diagnosis 6 years, median survival of 10 months… (full text at CIViC) PMID 24705254 · Buczkowicz et al., 2014 · Open in CIViC | civic |
| 〃 | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | B | Supports Positive | 3 | accepted | EID10605This retrospective study analyzed 91 biopsy-confirmed diffuse intrinsic pontine glioma (DIPG) patients using systematic stereotactic or surgical biopsy at diagnosis. Histone H3 alterations were assess… (full text at CIViC) PMID 26399631 · Castel et al., 2015 · Open in CIViC | civic |
| 〃 | Brain GlioblastomaALIAS | Diagnostic | B | Supports Positive | 3 | accepted | EID11038Whole genome sequencing (WGS) was performed on the diffuse intrinsic pontine gliomas (DIPGs) and matched germline DNA of 7 pediatric patients. Targeted sequencing was performed on an additional 43 DIP… (full text at CIViC) PMID 22286216 · Wu et al., 2012 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 2 | submitted | EID9840Taylor, et al. (2014) presented 26 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age 6 years, median overall survival was 11 months). Whol… (full text at CIViC) PMID 24705252 · Taylor et al., 2014 · Open in CIViC | civic |
| 〃 | Anaplastic AstrocytomaCURATED_BROADER | Diagnostic | B | Supports Positive | 2 | submitted | EID9841Taylor, et al. (2014) presented 26 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age 6 years, median overall survival was 11 months, 20 of… (full text at CIViC) PMID 24705252 · Taylor et al., 2014 · Open in CIViC | civic |
| 〃 | Diffuse AstrocytomaCURATED_BROADER | Diagnostic | B | Supports Positive | 2 | submitted | EID9842Taylor, et al. (2014) presented 26 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age 6 years, median overall survival was 11 months, 20 of… (full text at CIViC) PMID 24705252 · Taylor et al., 2014 · Open in CIViC | civic |
| 〃 | Diffuse Midline Glioma, H3 K27-Altered | Diagnostic | C | Supports Positive | 4 | submitted | EID9792Zhang et al. (2013) studied 151 tumors from 149 patients. The H3F3A p.K27M variant has been reported in 3 tumors (1.9%). The first sample (SJLGG037_D) was of a less than 3-year-old male patient screen… (full text at CIViC) PMID 23583981 · Zhang et al., 2013 · Open in CIViC | civic |
| 〃 | Ganglioglioma | Diagnostic | C | Supports Positive | 3 | submitted | EID9795Kleinschmidt-DeMasters, et al. (2017) analyzed 14 patient’s (age between 6 days and 62 years) gangliomas (GG). The H3F3A p.K27M variant has been reported in 2 GG: first was from 6 years old female met… (full text at CIViC) PMID 28378357 · Kleinschmidt-DeMasters et al., 2017 · Open in CIViC | civic |
| 〃 | Diffuse AstrocytomaCURATED_BROADER | Diagnostic | C | Supports Positive | 3 | submitted | EID9848Buczkowicz, et al. (2014) analyzed 74 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age at diagnosis 6 years, median survival of 10 months… (full text at CIViC) PMID 24705254 · Buczkowicz et al., 2014 · Open in CIViC | civic |
| 〃 | Anaplastic AstrocytomaCURATED_BROADER | Diagnostic | C | Supports Positive | 3 | submitted | EID9849Buczkowicz, et al. (2014) analyzed 74 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age at diagnosis 6 years, median survival of 10 months… (full text at CIViC) PMID 24705254 · Buczkowicz et al., 2014 · Open in CIViC | civic |
| 〃 | Ganglioglioma | Diagnostic | C | Supports Positive | 2 | submitted | EID9798Kleinschmidt-DeMasters, et al. (2018) analyzed the tumor database for patients with the H3 K27M variant and found 28 cases (including 3 meeting WHO grade I and 25 meeting WHO grade II-IV criteria). Th… (full text at CIViC) PMID 29393845 · Kleinschmidt-DeMasters et al., 2018 · Open in CIViC | civic |
| 〃 | Diffuse AstrocytomaCURATED_BROADER | Diagnostic | C | Supports Positive | 2 | submitted | EID9799Kleinschmidt-DeMasters, et al. (2018) analyzed the tumor database for patients with the H3 K27M variant and found 28 cases (including 3 meeting WHO grade I and 25 meeting WHO grade II-IV criteria). Th… (full text at CIViC) PMID 29393845 · Kleinschmidt-DeMasters et al., 2018 · Open in CIViC | civic |
| 〃 | Pilocytic Astrocytoma | Diagnostic | C | Supports Positive | 1 | rejected | EID9797Kleinschmidt-DeMasters, et al. (2018) analyzed the tumor database for patients with the H3 K27M variant and found 28 cases (including 3 meeting WHO grade I and 25 meeting WHO grade II-IV criteria). Th… (full text at CIViC) PMID 29393845 · Kleinschmidt-DeMasters et al., 2018 · Open in CIViC | civic |
| 〃 | OligodendrogliomaCURATED_BROADER | Diagnostic | C | Supports Positive | 1 | rejected | EID9837Yoshimoto, et al. (2017) analyzed 411 gliomas of Japanese patients (median age of 46 years (ranged between 1 to 87 years)). Direct sequencing revealed H3F3A p.K27R in one 51 years of age oligodendrogl… (full text at CIViC) PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC | civic |
| (prognostic) | Diffuse Midline Glioma, H3 K27-Altered | Prognostic | D | Supports Poor Outcome | 3 | submitted | EID10602Diffuse intrinsic pontine glioma (DIPG) patients harboring a H3.3 K27M mutation have a proneural/oligodendroglial phenotype and a prometastatic gene expression with PDGFRA activation. These mutation c… (full text at CIViC) PMID 26399631 · Castel et al., 2015 · Open in CIViC | civic |
| Dordaviprone | Diffuse Midline Glioma, H3 K27-Altered | Predictive | A | Supports Sensitivity Response | 4 | accepted | EID12695In a pooled analysis of five phase I–II trials and an expanded access program of ONC201 (dordaviprone) for recurrent H3 K27M–mutant diffuse midline glioma (NCT02525692, NCT03295396, NCT03416530, NCT03… (full text at CIViC) PMID 38335473 · Arrillaga-Romany et al., 2024 · Open in CIViC | civic |
| 〃 | Glioma | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID7601In a phase 2 trial, patients with H3 K27M mutant glioma have been treated with DRD2 antagonist ONC201. Among 29 patients, 1 patient experienced complete clinical regression lasting >14 months, and 3 p… (full text at CIViC) | civic |
| 5 |
| submitted |
EID9844Taylor, et al. (2014) presented 26 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age 6 years, median overall survival was 11 months, 20 of… (full text at CIViC) PMID 24705252 · Taylor et al., 2014 · Open in CIViC |
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| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | C | Supports Positive | 3 | submitted | EID9843Taylor, et al. (2014) presented 26 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age 6 years, median overall survival was 11 months, 20 of… (full text at CIViC) PMID 24705252 · Taylor et al., 2014 · Open in CIViC | civic |
| 〃 | Diffuse AstrocytomaCURATED_BROADER | Diagnostic | C | Supports Positive | 3 | submitted | EID9845Taylor, et al. (2014) presented 26 diffuse midline glioma, H3 K27M-mutant (here referred to as diffuse intrinsic pontine glioma) cases (median age 6 years, median overall survival was 11 months, 20 of… (full text at CIViC) PMID 24705252 · Taylor et al., 2014 · Open in CIViC | civic |
| 〃 | Brain GlioblastomaALIAS | Diagnostic | C | Supports Positive | 2 | accepted | EID9583Whole-genome sequencing (WGS) was performed on the diffuse intrinsic pontine gliomas (DIPGs) and matched germline DNA of 7 pediatric patients. Targeted sequencing was performed on an additional 43 DIP… (full text at CIViC) PMID 22286216 · Wu et al., 2012 · Open in CIViC | civic |
| Dordaviprone | Glioma | Predictive | B | Supports Sensitivity Response | 4 | rejected | EID7602In a phase 2 trial, patients with H3 K27M mutant glioma have been treated with DRD2 antagonist ONC201. Among 29 patients, 3 patients had experienced durable partial response by RANO. 10 patients had a… (full text at CIViC) | civic |