Subtype
Pilocytic Astrocytoma
CI-CAN-00008240Explore in graph →
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Subtype
CI-CAN-00008240Explore in graph →
Variants & evidence
29 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 |
|---|---|---|---|---|---|---|---|---|
| BRAF Mutation + BRAF Fusion1 | ||||||||
| (diagnostic) | Pilocytic Astrocytoma | Diagnostic | C | Supports Positive | 3 | submitted | EID7149Genetic alterations in 96 patients with pilocytic astrocytoma (PA) were evaluated. In addition to KIAA1549-BRAF fusions, other BRAF mutations and fusions were identified. Known MAPK activating events… (full text at CIViC) PMID 23817572 · Jones et al., 2013 · Open in CIViC | civic |
| BRAF T599dup1 | ||||||||
| Unspecified therapy | Pilocytic Astrocytoma | Predictive | C | Supports N/A | 3 | submitted | EID2990Pilocytic astrocytoma (PA) is emerging as a tumor entity with dysregulated Ras/Raf/MEK/ERK signaling. The well-known BRAF(V600E) mutation was found in 6/64 (9.38%) of our samples. Furthermore, 2/64 (3… (full text at CIViC) PMID 21190184 · Eisenhardt et al., 2011 · Open in CIViC | civic |
| BRAF V600E4 | ||||||||
| Dabrafenib + TrametinibCombination | Pilocytic Astrocytoma | Predictive | A | Supports Sensitivity Response | 1 | submitted | EID11313This study is part of an ongoing open-label, single-arm, phase 2 Rare Oncology Agnostic Research (ROAR) basket trial enlisting 45 patients (31 with glioblastoma) into the high-grade glioma cohort (oth… (full text at CIViC) PMID 34838156 · Wen et al., 2022 · Open in CIViC | civic |
| Selumetinib | Childhood Pilocytic Astrocytoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID7485In a phase 2 trial, patients with pediatric low-grade glioma after at least one standard therapy were treated with MEK1/2 inhibitor selumetinib. In stratum 1, nine patients with WHO grade I pilocytic… (full text at CIViC) PMID 31151904 · Fangusaro et al., 2019 · Open in CIViC | civic |
| Vemurafenib | Childhood Pilocytic Astrocytoma | Predictive | C | |||||
| BRAF V600E + BRAF Fusion1 | ||||||||
| Selumetinib | Pilocytic Astrocytoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID11316In this phase II trial on patients with pediatric low-grade glioma were assigned to six unique strata according to histology, tumour location, NF1 status, and BRAF aberration status. This study report… (full text at CIViC) PMID 31151904 · Fangusaro et al., 2019 · Open in CIViC | civic |
| FGFR1 Mutation1 | ||||||||
| (diagnostic) | Pilocytic Astrocytoma | Diagnostic | C | Supports Positive | 3 | submitted | EID7152Two new recurrent alterations within the kinase domain of FGFR1 in Pilocytic Astrocytoma (PA) were identified. The p.Asn546Lys variant (3 of 96 samples) had been shown in another study to increase kin… (full text at CIViC) PMID 23817572 · Jones et al., 2013 · Open in CIViC | civic |
| FGFR1 N546K1 | ||||||||
| Pemigatinib | Pilocytic Astrocytoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID10325Case report of a 32-year old male patient with pilocytic astrocytoma. Genomic profiling (FoundationOne) revealed a FGFR1 N546K mutation. The patient was enrolled in a phase I/II trial of pemigatinib a… (full text at CIViC) PMID 35507888 · Capone et al., 2022 · Open in CIViC | civic |
| ROS1 Fusion1 | ||||||||
| Entrectinib | 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 |
| H3-3A K28M1 | ||||||||
| (diagnostic) | 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 |
| NTRK2 Fusion1 | ||||||||
| (diagnostic) | Pilocytic Astrocytoma | Diagnostic | C | Supports Positive | 2 | accepted | EID8653A case report showing a two-year-old girl with an unusual pilocytic astrocytoma (PA) with both classic- and anaplastic- appearing tumors. A custom NGS panel identified a novel KANK1-NTRK2 fusion in bo… (full text at CIViC) PMID 29804288 · López et al., 2019 · Open in CIViC | civic |
| BRAF Fusion7 | ||||||||
| (diagnostic) | Pilocytic Astrocytoma | Diagnostic | B | Supports Positive | 5 | accepted | EID7148Genetic alterations in pilocytic astrocytoma (PA) were evaluated. Whole-genome sequencing of normal (blood) and tumor samples (n=96) was performed along with corresponding RNA-Seq (n=73) and mate-pair… (full text at CIViC) PMID 23817572 · Jones et al., 2013 · Open in CIViC | civic |
| 〃 | Pilocytic Astrocytoma | Diagnostic | B | Supports Positive | 4 | accepted | EID7319This study identified a novel rearrangement event between the uncharacterized gene KIAA-1549 and BRAF in 66% (29 of 44) of pilocytic astrocytoma but not in 244 higher-grade astrocytomas that were eval… (full text at CIViC) PMID 18974108 · Jones et al., 2008 · Open in CIViC | civic |
| (oncogenic) | Pilocytic Astrocytoma | Oncogenic | D | Supports Oncogenicity | 3 | accepted | ||
| KRAS Mutation1 | ||||||||
| (diagnostic) | Pilocytic Astrocytoma | Diagnostic | C | Supports Positive | 3 | submitted | EID7150Genetic alterations in 96 patients with pilocytic astrocytoma (PA) were evaluated. Two new alterations with double KRAS mutations (p.[Glu63Lys]+[Arg73Met] and p.[Leu19Phe]+[Gln22Lys] ) were observed. PMID 23817572 · Jones et al., 2013 · Open in CIViC | civic |
| NF1 Mutation1 | ||||||||
| (predisposing) | Pilocytic Astrocytoma | Predisposing | C | Supports Predisposition | 2 | rejected | EID12272This 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 | civic |
| PA-NF1 + NF1 Mutation2 | ||||||||
| (diagnostic) | Pilocytic Astrocytoma | Diagnostic | B | Supports Positive | 4 | rejected | EID12267This 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 | civic |
| Trametinib | Pilocytic Astrocytoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID12269This 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 | civic |
| NF1 p.R1276*1 | ||||||||
| (predisposing) | Pilocytic Astrocytoma | Predisposing | C | Supports Predisposition | 3 | rejected | EID12286This 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 | civic |
| NF1 G629R + NF1 Splice Site (c.205-1G>C)2 | ||||||||
| Carboplatin + Selumetinib + Trametinib + VincristineSubstitutes | Pilocytic Astrocytoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID12271This 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 | civic |
| 〃 | Pilocytic Astrocytoma | Predictive | C | Supports Sensitivity Response | 2 | rejected | EID12359This 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 | civic |
| PTPN11 Mutation1 | ||||||||
| (diagnostic) | Pilocytic Astrocytoma | Diagnostic | C | Supports Positive | 3 | submitted | EID7153Genetic alterations in pilocytic astrocytoma (PA) were evaluated. Two alterations were found in the PTPN11 (aka SHP-2) gene, encoding a RAS-MAPK-related adaptor protein and phosphatase. Both alteratio… (full text at CIViC) PMID 23817572 · Jones et al., 2013 · Open in CIViC | civic |
| NTRK2 Fusion1 | ||||||||
| (oncogenic) | Pilocytic Astrocytoma | Oncogenic | C | Supports Oncogenicity | 1 | accepted | EID11449Whole genome sequencing of 96 pilocytic astrocytomas by the International Cancer Genome Consortium (ICGC) PedBrain Tumor Project identified QKI::NTRK2 (e6::e16) fusions in 2 cases (5 yo & 12 yo males)… (full text at CIViC) PMID 23817572 · Jones et al., 2013 · Open in CIViC | civic |
| NTRK2 Fusion1 | ||||||||
| (diagnostic) | Pilocytic Astrocytoma | Diagnostic | C | Supports Positive | 3 | submitted | EID7151Genetic alterations in pilocytic astrocytoma (PA) were evaluated. Two new NTRK2 fusion events were identified in this study. These two NTRK2 fusion events, namely QKI-NTRK2 (2 of 96 samples) and NACC2… (full text at CIViC) PMID 23817572 · Jones et al., 2013 · Open in CIViC | civic |
Data updated 22 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 Sensitivity Response |
| 3 |
| accepted |
EID3777In a case report, a 28 month old patient with a cervicomedullary low grade glioma compatible with pilocytic astrocytoma which was resected had a second lesion consistent with a ganglioglioma 3 years l… (full text at CIViC) PMID 25524464 · del Bufalo et al., 2014 · Open in CIViC |
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| 〃 | Pilocytic Astrocytoma | Predictive | C | Supports Sensitivity Response | — | submitted | EID3772A pediatric pilocytic astrocytoma patient harboring BRAF V600E mutation, BRAF V600E mutation was associated with response to vemurafenib monotherapy. The patient was treated with standard chemotherapy… (full text at CIViC) PMID 24821190 · Skrypek et al., 2014 · Open in CIViC | civic |
EID7337This study identified a novel rearrangement event between the uncharacterized gene KIAA-1549 and BRAF in 66% (29 of 44) of pilocytic astrocytomas. The fusion gene was shown to delete the N-terminal BR… (full text at CIViC) PMID 18974108 · Jones et al., 2008 · Open in CIViC |
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| Selumetinib | Childhood Pilocytic Astrocytoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID7486In a phase 2 trial, patients with pediatric low-grade glioma after at least one standard therapy were treated with MEK1/2 inhibitor selumetinib. In stratum 1, nine patients with WHO grade I pilocytic… (full text at CIViC) PMID 31151904 · Fangusaro et al., 2019 · Open in CIViC | civic |
| 〃 | Childhood Pilocytic Astrocytoma | Predictive | D | Supports Sensitivity Response | 2 | accepted | EID8943In this preclinical experiment, a pediatric pilocytic astrocytoma cell line (DKFZ-BT66) harboring KIAA1549:BRAF fusion was generated from a two year old patient. Treatment with selumetinib resulted in… (full text at CIViC) PMID 28002790 · Selt et al., 2017 · Open in CIViC | civic |
| Sorafenib + VemurafenibSubstitutes | Childhood Pilocytic Astrocytoma | Predictive | D | Supports Resistance | 2 | accepted | EID7402A pilocytic astrocytoma (PA) cell line named DKFZ-BT66 was generated from a 2-year old patient with the disease. Expression of KIAA1549-BRAF fusion in this cell line was identified. Treatment with sor… (full text at CIViC) PMID 28002790 · Selt et al., 2017 · Open in CIViC | civic |
| Trametinib | Childhood Pilocytic Astrocytoma | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID8944In this preclinical experiment, a pediatric pilocytic astrocytoma cell line (DKFZ-BT66) harboring KIAA1549:BRAF fusion was generated from a two year old patient. Treatment with trametinib resulted in … (full text at CIViC) PMID 28002790 · Selt et al., 2017 · Open in CIViC | civic |