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Variants & evidence
26 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 |
|---|---|---|---|---|---|---|---|---|
| CASP8 Overexpression1 | ||||||||
| Conatumumab | Bone Ewing SarcomaALIAS | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID831In-vitro and in-vivo sensitivity to DR5 antibody conatumumab was correlated with expression of caspase-8. PMID 26291055 · Kang et al., 2015 · Open in CIViC | civic |
| CDKN2A Loss1 | ||||||||
| Linsitinib + PalbociclibSubstitutes | Ewing Sarcoma | Predictive | D | Supports Sensitivity Response | 2 | accepted | EID1879A patient-derived xenograft (PDX) from a patient with cyclin-dependent kinase inhibitor 2A/B (CDKN2A/B) loss and FUS-ERG fusion positive Ewing's sarcoma was created. The PDX was treated in four cohort… (full text at CIViC) PMID 27286459 · Murakami et al., 2016 · Open in CIViC | civic |
| CDKN2B Loss1 | ||||||||
| Linsitinib + PalbociclibSubstitutes | Ewing Sarcoma | Predictive | D | Supports Sensitivity Response | 2 | accepted | EID1880A patient-derived xenograft (PDX) from a patient with cyclin-dependent kinase inhibitor 2A/B (CDKN2A/B) loss and FUS-ERG fusion positive Ewing's sarcoma was created. The PDX was treated in four cohort… (full text at CIViC) PMID 27286459 · Murakami et al., 2016 · Open in CIViC | civic |
| ERG Fusion3 | ||||||||
| (diagnostic) | Ewing Sarcoma | Diagnostic | B | Supports Positive | 5 | accepted | EID475The t(21;22) rearrangement resulting in the EWSR1::ERG fusion is associated with the diagnosis of Ewing sarcoma. EWSR1::ERG is the second most common gene fusion reported in this disease. PMID 23706910 · Warren et al., 2013 · Open in CIViC | civic |
| 〃 | Ewing Sarcoma | Diagnostic | C | Supports Positive | 3 | accepted | EID10338Molecular analysis of 30 Ewing tumors (designated as Ewing's sarcomas and primitive neuroectodermal tumors (PNET) in this 1994 paper) from patients with well-documented clinical course was performed. … (full text at CIViC) PMID 7524604 · Zoubek et al., 1994 · Open in CIViC | civic |
| 〃 | Ewing Sarcoma | Diagnostic | C | Supports Positive | 2 | accepted | EID11603An analysis of 85 small blue round cell tumors (SBRCTs) that were negative for EWSR1, CIC, or BCOR by FISH identified 6 FUS::ERG and 1 FUS::FEV fusions. Clinicopathologic characterization of these 7 … | |
| FLI1 e7::e61 | ||||||||
| (prognostic) | Ewing Sarcoma | Prognostic | B | Supports Better Outcome | 4 | accepted | EID431In patients with Ewing's sarcoma, those with type 1 EWS-FLI1 fusions had longer overall survival than those with other types of EWS-FLI1 fusions. PMID 9552022 · de Alava et al., 1998 · Open in CIViC | civic |
| FLI1 Fusion7 | ||||||||
| (diagnostic) | Ewing Sarcoma | Diagnostic | B | Supports Positive | 5 | accepted | EID478t(11;22) rearrangements of EWSR1-FLI1 are diagnostic of ewing sarcoma, and are observed in 90% of cytogenetic samples. PMID 23706910 · Warren et al., 2013 · Open in CIViC | civic |
| 〃 | Bone Ewing SarcomaALIAS | Diagnostic | B | Supports Positive | 4 | accepted | EID1752A total of 85 unrelated cases of Ewing's sarcoma were examined and 83% were found to harbor the t(11;22)(q24;q12) chromosomal abnormality knwon to result in the EWSR1::FLI1 fusion. PMID 3163261 · Turc-Carel et al., 1988 · Open in CIViC | civic |
| 〃 | Bone Ewing SarcomaALIAS | Diagnostic | B | Supports Positive | 3 | accepted | ||
| FGFR1 Amplification1 | ||||||||
| Ponatinib | Bone Ewing SarcomaALIAS | Predictive | D | Supports Sensitivity Response | 3 | accepted | EID1247116 Ewing's sarcoma tumor samples were analyzed in this study. 2 patient's samples underwent whole genome sequencing, tumor samples from 51 patients underwent exome sequencing and expression analysis,… (full text at CIViC) PMID 26179511 · Agelopoulos et al., 2015 · Open in CIViC | civic |
| FGFR1 N546K1 | ||||||||
| Ponatinib | Bone Ewing SarcomaALIAS | Predictive | E | Supports Sensitivity Response | 2 | accepted | EID1246116 Ewing's sarcoma tumor samples were analyzed in this study. 2 patient's samples underwent whole genome sequencing, tumor samples from 51 patients underwent exome sequencing and expression analysis,… (full text at CIViC) PMID 26179511 · Agelopoulos et al., 2015 · Open in CIViC | civic |
| FGFR3 Amplification1 | ||||||||
| Pazopanib | Ewing Sarcoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID7835Clinical trial of soft tissue sarcoma patients found no responses to pazopanib + trametinib combination in Ewing sarcoma patients. Comparison was done to previously treated Ewing sarcoma that responde… (full text at CIViC) PMID 28377484 · Subbiah et al., 2017 · Open in CIViC | civic |
| FGFR4 Amplification1 | ||||||||
| Pazopanib | Ewing Sarcoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID7836Clinical trial of soft tissue sarcoma patients found no responses to pazopanib + trametinib combination in Ewing sarcoma patients. Comparison was done to previously treated Ewing sarcoma that responde… (full text at CIViC) PMID 28377484 · Subbiah et al., 2017 · Open in CIViC | civic |
| FLT4 Amplification1 | ||||||||
| Pazopanib | Ewing Sarcoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID7539Clinical trial of soft tissue sarcoma patients found no responses to pazopanib + trametinib combination in Ewing sarcoma patients. Comparison was done to previously treated Ewing sarcoma that respond… (full text at CIViC) PMID 28377484 · Subbiah et al., 2017 · Open in CIViC | civic |
| ERG Fusion1 | ||||||||
| (diagnostic) | Ewing Sarcoma | Diagnostic | B | Supports Positive | 3 | submitted | EID11601An analysis of 85 small blue round cell tumors (SBRCTs) that were negative for EWSR1, CIC, or BCOR by FISH identified 6 FUS::ERG and 1 FUS::FEV fusions. Clinicopathologic characterization of these 7 … (full text at CIViC) PMID 26690869 · Chen et al., 2016 · Open in CIViC | civic |
| FEV Fusion1 | ||||||||
| (diagnostic) | Ewing Sarcoma | Diagnostic | C | Supports Positive | 2 | submitted | EID11602An analysis of 85 small blue round cell tumors (SBRCTs) that were negative for EWSR1, CIC, or BCOR by FISH identified 6 FUS::ERG and 1 FUS::FEV fusions. Clinicopathologic characterization of these 7 … (full text at CIViC) PMID 26690869 · Chen et al., 2016 · Open in CIViC | civic |
| PTPRD R995C1 | ||||||||
| IGF1R Monoclonal Antibody + TemsirolimusCombination | Ewing Sarcoma | Predictive | C | Supports Sensitivity Response | 3 | accepted | EID10061An analysis of 8 ewing sarcoma patients revealed PTPRD mutations in 3 cases (V253I/W775*, T781A, R995C). 2 of 3 cases showed complete response (RECIST criteria) to IGF1R mAb based therapies. The R995C… (full text at CIViC) PMID 23800680 · Jiang et al., 2013 · Open in CIViC | civic |
| PTPRD T781A1 | ||||||||
| IGF1R Monoclonal Antibody + TemsirolimusCombination | Ewing Sarcoma | Predictive | C | Does Not Support Sensitivity Response | 3 | submitted | EID10062An analysis of 8 ewing sarcoma patients revealed PTPRD mutations in 3 cases (V253I/W775*, T781A, R995C). 2 of 3 cases showed complete response (RECIST criteria) to IGF1R mAb based therapies. The thrid… (full text at CIViC) PMID 23800680 · Jiang et al., 2013 · Open in CIViC | civic |
| PTPRD V253I1 | ||||||||
| Cixutumumab + TeprotumumabSubstitutes | Bone Ewing SarcomaALIAS | Predictive | C | Supports Sensitivity Response | 4 | accepted | EID1856Report of complete response to IGF1-R antibody, with or without mTOR inhibitor tensirolimus in 2/3 Ewing Sarcoma patients with germline PTPRD mutations (V253I and W775*, R995C). The third patient had … (full text at CIViC) PMID 23800680 · Jiang et al., 2013 · Open in CIViC | civic |
| SLFN11 EXPRESSION1 | ||||||||
| Niraparib + TemozolomideCombination | Ewing Sarcoma | Predictive | D | Supports Sensitivity Response | 3 | submitted | EID5884SLFN11 is an ETS transcription factor response gene and a predictive marker for therapeutic response to topoisomerase I inhibitors and temozolomide-PARP inhibitor combinations in ETS-activated cancers… (full text at CIViC) PMID 25779942 · Tang et al., 2015 · Open in CIViC | civic |
| STAG2 MUTATION1 | ||||||||
| (prognostic) | Bone Ewing SarcomaALIAS | Prognostic | B | Supports Poor Outcome | 4 | accepted | EID1697In a study of 96 Ewing sarcoma tumors, 15% showed heterogenous loss of STAG2 expression in IHC analysis. Of those with STAG2 expression loss, 88% presented with metastatic disease compared to only 27… (full text at CIViC) PMID 25186949 · Crompton et al., 2014 · Open in CIViC | civic |
Data updated 3 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.
PMID 26690869 · Chen et al., 2016 · Open in CIViC
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EID1754An evaluation of alternative strategies for detection of EWS-FLI (FISH break apart, FISH fusion, and RT-PCR). Of the 22 informative EWS/PNETs, 20 cases (91%) were positive by FISH break apart, FISH f… (full text at CIViC) PMID 16258512 · Bridge et al., 2006 · Open in CIViC |
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| 〃 | Ewing Sarcoma | Diagnostic | B | Supports Positive | 3 | accepted | EID10337Molecular analysis of 30 Ewing tumors (designated as Ewing's sarcomas and primitive neuroectodermal tumors (PNET) in this 1994 paper) from patients with well-documented clinical course was performed. … (full text at CIViC) PMID 7524604 · Zoubek et al., 1994 · Open in CIViC | civic |
| 〃 | Bone Ewing SarcomaALIAS | Diagnostic | B | Supports Positive | 2 | accepted | EID1753The authors propose RT-PCR as a diagnostic test for Ewing sarcoma using FFPE materials. Starting with 40 cases, intact RNA could be isolated from 6, and of these the EWSR1-FLI1 fusion was detected in… (full text at CIViC) PMID 9741544 · Park et al., 1998 · Open in CIViC | civic |
| Elimusertib | Ewing Sarcoma | Predictive | D | Supports Sensitivity Response | 2 | submitted | EID12541In preclinical models of FET fusion–positive cancers, suppression of ATM signaling by the EWSR1-FLI1 oncoprotein created dependence on ATR-mediated repair. The ATR inhibitor elimusertib showed potent … (full text at CIViC) PMID 37205599 · Menon et al., 2025 · Open in CIViC | civic |
| HCI-2509 | Ewing Sarcoma | Predictive | D | Supports Sensitivity Response | 2 | accepted | EID12516Preclinical studies demonstrated that the LSD1 inhibitor HCI-2509 reduced proliferation and induced apoptosis in five EWSR1-FLI1 fusion-positive Ewing sarcoma cell lines (A673, TC32, TC71, SK-N-MC, CH… (full text at CIViC) PMID 24963049 · Sankar et al., 2014 · Open in CIViC | civic |