Cancer Family
Central Nervous System Neoplasm
CI-CAN-00000118Explore in graph →
Non-malignant1,345 active trials7 approved drugs177 evidence items
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Cancer Family
CI-CAN-00000118Explore in graph →
Variants & evidence
428 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 |
|---|---|---|---|---|---|---|---|---|
| TACC1 Fusion1 | ||||||||
| Futibatinib | GlioblastomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11634In a phase 1 multihistology trial of the FGFR1/2/3/4 inhibitor futibatinib, an objective response rate of 13.7% was seen with responses in a broad spectrum of tumors and across a broad spectrum of FGF… (full text at CIViC) PMID 34551969 · Meric-Bernstam et al., 2022 · Open in CIViC | civic |
| FGFR3 K508M1 | ||||||||
| (oncogenic) | GlioblastomaCURATED_BROADER | Oncogenic | D | Does Not Support Oncogenicity | 2 | submitted | EID11806Rat1A astrocytes cells expressing the FGFR3-TACC3-K508M were tested to evaluate anchorage-independent growth in soft agar (Fig. 2A and table S5) and did not show an ability to grow evidenced by no col… (full text at CIViC) PMID 22837387 · Singh et al., 2012 · Open in CIViC | civic |
| TACC3 Fusion2 | ||||||||
| Erdafitinib | GlioblastomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID13165In the phase II NCI-MATCH EAY131-K2 trial, two patients with WHO grade IV IDH1/2-wildtype glioblastoma harboring FGFR3 fusions experienced progression-free survival exceeding 168 days during erdafitin… (full text at CIViC) PMID 38603650 · Gong et al., 2024 · Open in CIViC | civic |
| Fexagratinib | Diffuse AstrocytomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID9242Phase II study (NCT02465060) from NCI-MATCH Trial (EAY131) Subprotocol W addressed the use of AZD4547 (an FGFR 1-3 inhibitor) in 48 tumor patients carrying FGFR 1-3 variants. Partial responses were ob… (full text at CIViC) PMID 32463741 · Chae et al., 2020 · Open in CIViC | civic |
| FOXO3 Mutation2 | ||||||||
| (prognostic) | Choroid Plexus Carcinoma | Prognostic | A | Supports Poor Outcome | 3 | rejected | EID7223From UniProt: A chromosomal aberration involving FOXO3 is found in secondary acute leukemias. PMID 26822237 · Parsons et al., 2016 · Open in CIViC | civic |
| (functional) | Choroid Plexus Carcinoma | Functional | D | Supports Loss Of Function | 4 | rejected | EID7222Transcriptional activator which triggers apoptosis in the absence of survival factors, including neuronal cell death upon oxidative stress. PMID 26822237 · Parsons et al., 2016 · Open in CIViC | civic |
| FOXR2 Rearrangement3 | ||||||||
| (diagnostic) | Central Nervous System Neuroblastoma, FOXR2-ActivatedALIAS | Diagnostic | B | Supports Positive | 4 | accepted | EID11527In the 2016 study by Sturm et al., the authors performed comprehensive molecular profiling, including DNA methylation arrays, transcriptomics, and genome-wide sequencing, on 323 institutionally diagno… (full text at CIViC) PMID 26919435 · Sturm et al., 2016 · Open in CIViC | civic |
| 〃 | Central Nervous System Neuroblastoma, FOXR2-ActivatedALIAS | Diagnostic | B | Supports Positive | 4 | accepted | EID11528DNA methylation profiling of 84 tumors initially diagnosed as CNS-PNET identified CNS neuroblastoma with FOXR2 activation (CNS NB-FOXR2) as a distinct molecular subgroup, presented in 24% (20/84) of c… (full text at CIViC) PMID 33536079 · Korshunov et al., 2021 · Open in CIViC | civic |
| 〃 | ||||||||
| FUBP1 LOSS-OF-FUNCTION1 | ||||||||
| (diagnostic) | OligodendrogliomaCURATED_BROADER | Diagnostic | D | Supports Positive | 3 | submitted | EID7441FUBP1 truncating mutations occur somatically with high specificity for adult oligodendroglioma and are considered oncogenic in that setting PMID 21817013 · Bettegowda et al., 2011 · Open in CIViC | civic |
| NTRK2 Fusion1 | ||||||||
| Entrectinib | GlioblastomaCURATED_BROADER | Predictive | C | Does Not Support Sensitivity Response | 2 | submitted | EID12605In 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 | civic |
| ROS1 e7::e351 | ||||||||
| Lorlatinib | GlioblastomaCURATED_BROADER | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID7483Ectopic expression of GOPC–ROS1 in Ba/F3 cells induced cytokine-independent growth whereas overexpression of ROS1 alone did not. Using a series of tyrosine kinase inhibitors, lorlatinib was the most e… (full text at CIViC) PMID 30171048 · Davare et al., 2018 · Open in CIViC | civic |
| ROS1 Fusion3 | ||||||||
| Entrectinib | Diffuse Midline Glioma, H3 K27-Altered | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID11849In 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 (4 yo) with … (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| 〃 | Pilocytic Astrocytoma | Predictive | C | Does Not Support Sensitivity Response | 2 | submitted | EID12604In 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 | civic |
| 〃 | Low Grade Glioma | Predictive | C | Supports Sensitivity Response | ||||
| 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 K28M17 | ||||||||
| (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 | |||||
Data updated 2 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.
| Diagnostic |
| B |
| Supports Positive |
| 4 |
| accepted |
EID12521In this Swedish population-based study, the authors retrospectively re-evaluated 71 poorly differentiated supratentorial CNS tumors in children, previously diagnosed as CNS-PNETs, to assess their mole… (full text at CIViC) PMID 36895035 · Schepke et al., 2023 · Open in CIViC |
| civic |
| 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 |
| 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 |