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Variants & evidence
9 evidence items mapped to this entity or its descendants, grouped by molecular profile, then therapy. 50 items per page.
| Therapy | Type | Level | Direction · significance | Rating (1–5) | Status | Evidence | Source |
|---|---|---|---|---|---|---|---|
| NTRK2 Fusion1 | |||||||
| Entrectinib | Predictive | C | Does Not Support Sensitivity Response | 2 | accepted | EID12053In the STARTRK-NG (NCT02650401) phase 1/2 trial, a 10 year old patient with prior chemotherapy treatment who had Ganglioglioma in the Spine, with a tumor category of low grade glioma, and WHO histolog… (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| BRAF T599dup1 | |||||||
| Dabrafenib + TrametinibCombination | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID9789A pediatric case of ganglioglioma was previously reported (PMID: 29434027) to harbor a BRAF p.T599dup variant (observed at VAF of 5.2%). The patient had a partial resection and was then treated with V… (full text at CIViC) PMID 33637608 · Miller et al., 2021 · Open in CIViC | civic |
| BRAF V600E2 | |||||||
| Vemurafenib | Predictive | C | Supports Sensitivity Response | — | submitted | EID3778A grade I refractory brainstem ganglioglioma patient harboring BRAF V600E mutation was associated with partial response to vemurafenib monotherapy. The patient experienced significant clinical improve… (full text at CIViC) PMID 26579623 · Aguilera et al., 2016 · Open in CIViC | civic |
| Cobimetinib + VemurafenibCombination | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11310In this case study, a 32-year-old man, previously fit and well, presented with a two-month history of vertigo, right visual disturbance, dysphagia, and ataxia. Brain MRI revealed a large mixed solid a… (full text at CIViC) PMID 31985841 · Yau et al., 2020 · Open in CIViC | civic |
| AXIN2 P455K + BAX L76R + BRAF V600E + CDKN2A Deletion + CDKN2B Deletion + TET2 E796KCDKN2BTET2BAXAXIN2BRAFCDKN2A1 | |||||||
| Cobimetinib + VemurafenibCombination | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11314In this case study, a 28-year-old male with BRAF V600E-mutant anaplastic ganglioglioma was started with vemurafenib at 960 mg twice daily in March 2015 for tumour recurrence despite one surgical resec… (full text at CIViC) PMID 30120137 · Touat et al., 2018 · Open in CIViC | civic |
| NTRK2 Fusion1 | |||||||
| Entrectinib | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID11852In 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 (9 yo) with … (full text at CIViC) PMID 35395680 · Desai et al., 2022 · Open in CIViC | civic |
| H3-3A K28M2 | |||||||
| (diagnostic) | 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 |
| 〃 | 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 |
| IDH1 R132H1 | |||||||
| (diagnostic) | Diagnostic | B | Supports Negative | 3 | submitted | EID6125Testing of 98 tumors diagnosed as gangliogliomas identified IDH1 R132H mutations in 8 patients. Mutation was associated with increased risk of recurrence (p=0.0007), malignant transformation and/or de… (full text at CIViC) PMID 21314850 · Horbinski et al., 2011 · 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.