Cancer Family
Nervous System Neoplasm
CI-CAN-00000038Explore in graph →
Non-malignant1,523 active trials9 approved drugs210 evidence items
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Cancer Family
CI-CAN-00000038Explore in graph →
Variants & evidence
502 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 |
|---|---|---|---|---|---|---|---|---|
| ROS1 Fusion1 | ||||||||
| Entrectinib | Low Grade Glioma | Predictive | C | Supports Sensitivity Response | 1 | accepted | EID11855In a phase 1/2 trial of entrectinib in pediatric patients, tumors with fusions in NTRK, ROS1, or ALK had an overall response (ORR) of 57.7% (95% CI; 36.9-76.7). This included one patient (8 yo) with … (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| H3-3A G351 | ||||||||
| (diagnostic) | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | submitted | EID9815Sturm, 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 G35R9 | ||||||||
| (diagnostic) | Diffuse Hemispheric Glioma, H3 G34-Mutant | Diagnostic | B | Supports Positive | 4 | submitted | EID12832This study presents a series of 81 cases of diffuse hemispheric glioma, H3 G34-mutant (DHG G34m). Cases were noted to have either the histological appearance of a typical glioblastoma (73%) or as prim… (full text at CIViC) PMID 26482474 · Korshunov et al., 2016 · Open in CIViC | civic |
| 〃 | Diffuse Hemispheric Glioma, H3 G34-Mutant | Diagnostic | B | Supports Positive | 4 | submitted | EID12856Schwartzentruber et al. performed whole-exome sequencing (WES) on 48 paediatric GBM samples (patients aged 3–20 years, tumour cores containing >90% neoplastic tissue, median 61-fold coverage with 91% … (full text at CIViC) PMID 22286061 · Schwartzentruber et al., 2012 · Open in CIViC | civic |
| 〃 | Brain GlioblastomaALIAS | Diagnostic | B | |||||
| H3-3A G35V6 | ||||||||
| (diagnostic) | Diffuse Hemispheric Glioma, H3 G34-Mutant | Diagnostic | B | Supports Positive | 4 | submitted | EID12833This study presents a series of 81 cases of diffuse hemispheric glioma, H3 G34-mutant (DHG G34m). Cases were noted to have either the histological appearance of a typical glioblastoma (73%) or as prim… (full text at CIViC) PMID 26482474 · Korshunov et al., 2016 · Open in CIViC | civic |
| 〃 | Diffuse Hemispheric Glioma, H3 G34-Mutant | Diagnostic | B | Supports Positive | 4 | submitted | EID12857G34V is the rarer of the two G34 substitutions identified in this stud by Schwartzentruber et al, with only 1/48 tumours in the primary WES cohort harbouring this variant (sample PGBM16), compared to … (full text at CIViC) PMID 22286061 · Schwartzentruber et al., 2012 · Open in CIViC | civic |
| 〃 | Brain GlioblastomaALIAS | Diagnostic | C | |||||
| 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 K28M29 | ||||||||
| (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 |
Data updated 4 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.
| Supports Positive |
| 3 |
| rejected |
EID9416Whole 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 variant has been r… (full text at CIViC) PMID 22286061 · Schwartzentruber et al., 2012 · Open in CIViC |
| civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | submitted | EID9610In 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 | EID9817Sturm, 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 | rejected | EID9820Korshunov, et al. (2016) studied 81 central nervous system (CNS) tumors (located in temporal and parietal lobe in 80% of cases, 88% carried TP53 variant, 95% carried ATRX loss), including: 59 glioblas… (full text at CIViC) PMID 26482474 · Korshunov et al., 2016 · Open in CIViC | civic |
| 〃 | Central Nervous System Embryonal TumorCURATED_BROADER | Diagnostic | B | Supports Positive | 3 | rejected | EID9821Korshunov, et al. (2016) studied 81 central nervous system tumors (located in temporal and parietal lobe in 80% of cases, 88% carried TP53 variant, 95% carried ATRX loss), including: 59 glioblastoma-l… (full text at CIViC) PMID 26482474 · Korshunov et al., 2016 · Open in CIViC | civic |
| 〃 | Anaplastic AstrocytomaCURATED_BROADER | Diagnostic | C | Supports Positive | 3 | submitted | EID9609In 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 |
| 〃 | High-Grade Glioma, NOS | Diagnostic | C | Supports Positive | 3 | submitted | EID9839Yoshimoto, 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.G34R in 4 cases (median age 10 years (ran… (full text at CIViC) PMID 28447171 · Yoshimoto et al., 2017 · Open in CIViC | civic |
| Supports Positive |
| 3 |
| rejected |
EID9415Whole genome 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 G34V variant has been … (full text at CIViC) PMID 22286061 · Schwartzentruber et al., 2012 · Open in CIViC |
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| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | C | Supports Positive | 3 | submitted | EID9822Korshunov, et al. (2016) studied 81 central nervous system tumors (located in temporal and parietal lobe in 80% of cases, 88% carried TP53 variant, 95% carried ATRX loss), including: 59 glioblastoma-l… (full text at CIViC) PMID 26482474 · Korshunov et al., 2016 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Diagnostic | C | Supports Positive | 1 | submitted | EID9818Sturm, 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 |
| 〃 | Central Nervous System Embryonal TumorCURATED_BROADER | Diagnostic | C | Supports Positive | 1 | submitted | EID9823Korshunov, et al. (2016) studied 81 central nervous system tumors (located in temporal and parietal lobe in 80% of cases, 88% carried TP53 variant, 95% carried ATRX loss), including: 59 glioblastoma-l… (full text at CIViC) PMID 26482474 · Korshunov et al., 2016 · Open in CIViC | civic |
| 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 |
| 〃 | Childhood Spinal Cord NeoplasmALIAS | Diagnostic | B | Supports Positive | 3 | submitted | EID9794Gessi, et al. (2015) studied 30 primary spinal diffuse high-grade gliomas (HGG) and 6 diffuse astrocytomas. The H3F3A p.K27M variant has been reported in 16 HGG (including 2 anaplastic gangliogliomas,… (full text at CIViC) PMID 26231952 · Gessi et al., 2015 · 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 |
| 〃 | Childhood Spinal Cord NeoplasmALIAS | Diagnostic | C | Supports Positive | 3 | submitted | EID1080316 of 30 high-grade gliomas (HGG) were found to harbor K27M mutations. The frequency of mutations was similar between adults and children (52% vs 54%). PMID 26231952 · Gessi et al., 2015 · 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 |