Loading cancer entity…
Loading cancer entity…
Variants & evidence
18 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 |
|---|---|---|---|---|---|---|---|---|
| NR4A3 Fusion2 | ||||||||
| Pazopanib | Extraskeletal Myxoid Chondrosarcoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID9819In this single-arm, open-label phase 2 trial, extraskeletal myxoid chondrosarcoma patients treated with pazopanib were reported and 22/23 patients (one patient died before the primary analysis) were e… (full text at CIViC) PMID 31331701 · Stacchiotti et al., 2019 · Open in CIViC | civic |
| Sunitinib | Extraskeletal Myxoid Chondrosarcoma | Predictive | B | Supports Sensitivity Response | 3 | submitted | EID6378his report confirms the therapeutic activity of sunitinib in extraskeletal myxoid chondrosarcoma. Genotype/phenotype analyses support a correlation between response and EWSR1-NR4A3 fusion. PMID 24703573 · Stacchiotti et al., 2014 · Open in CIViC | civic |
| NR4A3 Fusion + NR4A3 Fusion1 | ||||||||
| Trabectedin | Extraskeletal Myxoid Chondrosarcoma | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID12545In a sub-analysis of a randomized phase II trial in extraskeletal myxoid chondrosarcoma (EWSR1-NR4A3 and TAF15-NR4A3 fusions; n = 2) and mesenchymal chondrosarcoma (HEY1-NCOA2 fusion, n = 6)(trabected… (full text at CIViC) PMID 27418251 · Morioka et al., 2016 · Open in CIViC | civic |
| NCOA2 Fusion8 | ||||||||
| (diagnostic) | Mesenchymal Chondrosarcoma | Diagnostic | B | Supports Positive | 4 | accepted | EID7008A HEY1-NCOA2 fusion joining exon 4 of HEY1 to exon 13 of NCOA2 was identified in 10 mesenchymal chondrosarcomas but was absent from all other sarcoma types tested including other chondrosarcoma subtyp… (full text at CIViC) PMID 22034177 · Wang et al., 2012 · Open in CIViC | civic |
| 〃 | Mesenchymal Chondrosarcoma | Diagnostic | B | Supports Positive | 4 | submitted | EID7025Meningeal hemangiopericytoma (HPC) and mesenchymal chondrosarcoma (MC) are aggressive neoplasms with overlapping clinical presentations and morphology. Thirteen FFPE samples of MC (9 females, 4 males)… (full text at CIViC) PMID 24124145 · Fritchie et al., 2013 · Open in CIViC | civic |
| 〃 | Mesenchymal Chondrosarcoma | Diagnostic | B | Supports Positive | 4 | |||
| IDH1 Mutation1 | ||||||||
| (prognostic) | Chondrosarcoma | Prognostic | B | Supports Better Outcome | 4 | accepted | EID7725In a retrospective study, the authors evaluated the association between IDH1/IDH2 mutation and prognosis in patients with high-grade central chondrosarcoma. Although no association was discovered betw… (full text at CIViC) PMID 31615936 · Zhu et al., 2020 · Open in CIViC | civic |
| IDH1 R1323 | ||||||||
| (diagnostic) | Chondrosarcoma | Diagnostic | B | Supports Positive | 3 | submitted | EID11198Approximately 1200 mesechymal tumors were screened for IDH1 R132 mutations by Sequenom mass spectrometry, including 220 cartilaginous tumors, 222 osteosarcomas and another 750 bone and soft tissue tum… (full text at CIViC) PMID 21598255 · Amary et al., 2011 · Open in CIViC | civic |
| 〃 | Chondrosarcoma | Diagnostic | B | Supports Positive | 2 | rejected | EID11197Approximately 1200 mesechymal tumors were screened for IDH1 R132 mutations by Sequenom mass spectrometry, including 220 cartilaginous tumors, 222 osteosarcomas and another 750 bone and soft tissue tum… (full text at CIViC) PMID 21598255 · Amary et al., 2011 · Open in CIViC | civic |
| Ivosidenib | Chondrosarcoma | Predictive | A | Supports Sensitivity Response | 2 | submitted | ||
| IDH1 R132Q2 | ||||||||
| (oncogenic) | Chondrosarcoma | Oncogenic | D | Supports Oncogenicity | 3 | rejected | EID11195Conditional knock-in of IDH1 R132Q in chondrocytes in of weaned mice resulted in the development of multiple enchondroma-like lesions. Enchondromas are benign cartilage tumors and precursors to malig… (full text at CIViC) PMID 25730874 · Hirata et al., 2015 · Open in CIViC | civic |
| 〃 | Chondrosarcoma | Oncogenic | D | Supports Oncogenicity | 3 | submitted | EID11196Conditional knock-in of IDH1 R132Q in chondrocytes of weaned mice resulted in the development of multiple enchondroma-like lesions. Enchondromas are benign cartilage tumors and precursors to malignan… (full text at CIViC) PMID 25730874 · Hirata et al., 2015 · Open in CIViC | civic |
| IDH2 Mutation1 | ||||||||
| (prognostic) | Chondrosarcoma | Prognostic | B | Supports Better Outcome | 4 | accepted | EID7726In a retrospective study, the authors evaluated the association between IDH1/IDH2 mutation and prognosis in patients with high-grade central chondrosarcoma. Although no association was discovered betw… (full text at CIViC) PMID 31615936 · Zhu et al., 2020 · 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.
| submitted |
EID7026FISH was used to detect HEY1-NCOA2 fusions in 10 paraffin embedded samples from patients diagnosed with mesenchymal chondrosarcoma. HEY1 and NCOA2 are located on same chromosome but generate distinct … (full text at CIViC) PMID 23252872 · Nakayama et al., 2012 · Open in CIViC |
| civic |
| 〃 | Mesenchymal Chondrosarcoma | Diagnostic | B | Supports Positive | 4 | accepted | EID7124FISH was used to detect HEY1-NCOA2 fusions in 10 paraffin embedded samples from patients diagnosed with mesenchymal chondrosarcoma. HEY1 and NCOA2 are located on the same chromosome but generate disti… (full text at CIViC) PMID 23252872 · Nakayama et al., 2012 · Open in CIViC | civic |
| 〃 | Mesenchymal Chondrosarcoma | Diagnostic | B | Supports Positive | 4 | accepted | EID7125Meningeal hemangiopericytoma (HPC) and mesenchymal chondrosarcoma (MC) are aggressive neoplasms with overlapping clinical presentations and morphology. Thirteen FFPE samples of MC (9 females, 4 males)… (full text at CIViC) PMID 24124145 · Fritchie et al., 2013 · Open in CIViC | civic |
| 〃 | Mesenchymal Chondrosarcoma | Diagnostic | B | Supports Positive | 2 | accepted | EID70176 of 6 patients with mesenchymal chondrosarcoma and sufficient tissue tested positive for NCOA2 rearrangments by FISH supporting that NCOA2 rearrangements (presumably HEY1-NCOA2 translocations) are pr… (full text at CIViC) PMID 26146478 · Bishop et al., 2015 · Open in CIViC | civic |
| Imatinib | Mesenchymal Chondrosarcoma | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID10798In two independent in-vitro and PDX models of HEY1::NCOA2 fusion positive mesenchymal chondrosarcoma, imatinib demonstrated a highly selective cytotoxic effect and reduction of growth. PMID 34837064 · Safaric Tepes et al., 2022 · Open in CIViC | civic |
| Trabectedin | Mesenchymal Chondrosarcoma | Predictive | C | Supports Sensitivity Response | 2 | accepted | EID12585In a sub-analysis of a randomized phase II trial in extraskeletal myxoid chondrosarcoma (EWSR1-NR4A3 and TAF15-NR4A3 fusions) and mesenchymal chondrosarcoma (HEY1-NCOA2 fusion)(trabectedin n = 5; BSC … (full text at CIViC) PMID 27418251 · Morioka et al., 2016 · Open in CIViC | civic |
EID11194In a phase 1 study (NCT02073994), 21 patients with advanced IDH-mutant chondrosarcoma received the IDH1-mutant specific inhibitor ivosidenib. Plasma levels of the 2-HG oncometabolite decreased to nor… (full text at CIViC) PMID 32208957 · Tap et al., 2020 · Open in CIViC |
| civic |