Cancer Family
Anaplastic Malignant Neoplasm
CI-CAN-00000065Explore in graph →
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- C36025
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Cancer Family
CI-CAN-00000065Explore in graph →
Variants & evidence
176 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 |
|---|---|---|---|---|---|---|---|---|
| EGFR VIII3 | ||||||||
| Epidermal Growth Factor Receptor Tyrosine Kinase Inhibitor | GlioblastomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 4 | accepted | EID77249 patients (26 pretreatment tissues available for analysis) with recurrent glioblastomas who were treated with EGFR TKIs were analyzed in this study. Results were confirmed in an independent cohort o… (full text at CIViC) PMID 16282176 · Mellinghoff et al., 2005 · Open in CIViC | civic |
| Erlotinib + GefitinibSubstitutes | GlioblastomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID1128Expression of EGFRvIII and PTEN was evaluated in 26 patients with glioblastomas treated with erlotinib or gefitinib and no EGFR or Her2/neu kinase domain mutations. 7 patients responded to treatment w… (full text at CIViC) PMID 16282176 · Mellinghoff et al., 2005 · Open in CIViC | civic |
| Rindopepimut | GlioblastomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | accepted | EID971In a Phase II clinical trial, patients with Glioblastoma Multiforme (GBM), positive for EGFR VIII deletion mutation (N=65), were treated with the Rindopepimut cancer vaccine, and standard adjuvant te… (full text at CIViC) PMID 25586468 · Schuster et al., 2015 · Open in CIViC | civic |
| VOPP1 Fusion1 | ||||||||
| (oncogenic) | GlioblastomaCURATED_BROADER | Oncogenic | C | Supports Oncogenicity | 1 | submitted | EID12552Exome DNA sequencing was performed in 291 glioblastomas, 42 with whole genome sequencing, to update the TCGA dataset. Whole Genome Sequencing was performed on 42 of the samples, and ten of these demo… (full text at CIViC) PMID 24120142 · Brennan et al., 2013 · Open in CIViC | civic |
| VSTM2A Fusion1 | ||||||||
| Bevacizumab/Lomustine Regimen | GlioblastomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 2 | submitted | EID11101EGFR::VSTM2A was identified by RNA-seq in tumor sample derived from 1/78 recurrent glioblastoma patients who benefited from combined beva/CCNU (bevacizumab in combination with lomustine) in the BELOB … (full text at CIViC) PMID 26762204 · Erdem-Eraslan et al., 2016 · Open in CIViC | civic |
| NTRK3 Fusion1 | ||||||||
| (oncogenic) | GlioblastomaCURATED_BROADER | Oncogenic | C | Supports Oncogenicity | 1 | accepted | EID10507From a retrospective review of 38,095 tumor samples from 33,997 patients using DNA sequencing (MSK-IMPACT) and RNA sequencing (MSK-Fusion panel) identified 87 tumors with NTRK1-3 fusions. One case of… (full text at CIViC) PMID 31375766 · Solomon et al., 2020 · Open in CIViC | civic |
| NTRK3 Fusion4 | ||||||||
| (oncogenic) | Gliosarcoma | Oncogenic | C | Supports Oncogenicity | 2 | submitted | EID12603In the STARTRK-NG Phase 1/2 trial of entrectinib in pediatric patients, one patient (4 yo) with Biphasic and spindled epithelioid glial neoplasm had ETV6::NTRK3 fusion. The patient received che… (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| (diagnostic) | Anaplastic Sarcoma of the Kidney | Diagnostic | C | Does Not Support Negative | 1 | submitted | EID10421In 4 cases of Anaplastic sarcoma of the kidney ETV6::NTRK3 fusion was negative. PMID 17895746 · Vujanić et al., 2007 · Open in CIViC | civic |
| Entrectinib | GlioblastomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 3 | accepted | ||
| EZH2 Overexpression1 | ||||||||
| (prognostic) | GlioblastomaCURATED_BROADER | Prognostic | B | Supports Poor Outcome | 2 | submitted | EID8006Kaplan-Meier analysis of EZH2 gene expression showed that elevated expression is significantly associated with shorter disease-free survival in GBM (p=1.3e-12). PMID 32180229 · Li et al., 2020 · Open in CIViC | civic |
| 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 Fusion1 | ||||||||
| 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 |
| 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 |
| 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 G35R5 | ||||||||
| (diagnostic) | Brain GlioblastomaALIAS | Diagnostic | B | 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 | |||||||
| H3-3A G35V3 | ||||||||
| (diagnostic) | Brain GlioblastomaALIAS | Diagnostic | C | 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 | civic |
| 〃 | 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 | |||||||
| H3-3A K281 | ||||||||
| (diagnostic) | 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 K28M10 | ||||||||
| (diagnostic) | 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 |
| 〃 | 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 | |||||||
| H3-3A K28R1 | ||||||||
| (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 |
| H3C2 K27M3 | ||||||||
| (diagnostic) | Anaplastic AstrocytomaCURATED_BROADER | Diagnostic | C | Supports Positive | 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 | civic |
| 〃 | 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 |
| 〃 | Brain GlioblastomaALIAS | |||||||
| 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 |
| IDH1 R1326 | ||||||||
| (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 |
| 〃 | Anaplastic AstrocytomaALIAS | Prognostic | B | Supports Better Outcome | ||||
| IDH1 R132H2 | ||||||||
| (diagnostic) | GlioblastomaCURATED_BROADER | Diagnostic | A | Supports Positive | 5 | submitted | EID8629In the 2016 WHO classification for CNS tumors, IDH-mutant glioblastoma is recognized as a distinct entity. Whereas IDH-wildtype glioblastoma constitutes about 90% of cases and correspond to de novo gl… (full text at CIViC) PMID 27157931 · Louis et al., 2016 · Open in CIViC | civic |
| Pan-Mutant-IDH1 Inhibitor Bay-1436032 | GlioblastomaCURATED_BROADER | Predictive | D | Supports Sensitivity Response | — | submitted | EID2325In an experimental study, glioblastoma cells expressing IDH1 R132H were associated with sensitivity to (bis-2-(5-phenylacetamido-1,2,4-thiadiazol-2-yl)ethyl sulfide), comparable to cells expressing wi… (full text at CIViC) PMID 21045145 · Seltzer et al., 2010 · Open in CIViC | civic |
Data updated 8 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.
In a phase 1/2 trial of entrectinib in pediatric patients, tumors with fusions in NTRK, ROS, or ALK had an overall response (ORR) of 57.7% (95% CI; 36.9-76.7). This included one patient (3 yo) with a… (full text at CIViC)
PMID 35395680 · Desai et al., 2022 · Open in CIViC
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| 〃 | Anaplastic AstrocytomaCURATED_BROADER | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID11853In 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 (2 months ol… (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| 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 |
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| 〃 | 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 |
| 〃 | 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 |
| 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 |
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| 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 |
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| 〃 | 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 |
| 〃 | 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 |
| 〃 | 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 |
| 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 |
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| 3 |
| accepted |
EID348In patients with astrocytoma, the presence of IDH1 R132 mutation is prognostic for better survival compared to patients who harbor wild type mutation. PMID 19933982 · Dubbink et al., 2009 · Open in CIViC |
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| 〃 | GlioblastomaCURATED_BROADER | Prognostic | B | Supports Better Outcome | 3 | accepted | EID349In patients with glioblastoma, those harboring IDH1 R132 mutation has higher overall survival compared to those who do not have IDH1 mutation. PMID 20127344 · Bleeker et al., 2010 · Open in CIViC | civic |
| Cetuximab | GlioblastomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID4032In a clinical study of 63 recurrent glioma patients, de novo glioblastoma patients treated with cetuximab monotherapy (n=27) and harboring IDH1 codon 132 mutation were associated with improved overal… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC | civic |
| 〃 | GlioblastomaCURATED_BROADER | Predictive | B | Supports Sensitivity Response | — | submitted | EID3718In a clinical study of 63 recurrent glioma patients, de novo glioblastoma patients treated with cetuximab monotherapy (n=27) and harboring IDH1 codon 132 mutation were associated with improved overal… (full text at CIViC) PMID 22199315 · Lv et al., 2011 · Open in CIViC | civic |