Cancer Family
Notochordal Tumor
CI-CAN-00000294Explore in graph →
Non-malignant15 active trials1 evidence items
- NCIt
- C7063
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Cancer Family
CI-CAN-00000294Explore in graph →
Variants & evidence
14 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 Expression1 | ||||||||
| Lapatinib | Chordoma | Predictive | B | Supports Sensitivity Response | 2 | accepted | EID198418 advanced progressing chordoma patients that were positive for EGFR by at least one technique (immunohistochemistry and/or phospho-arrays, real-time polymerase chain reaction for EGFR ligands, FISH)… (full text at CIViC) PMID 23559153 · Stacchiotti et al., 2013 · Open in CIViC | civic |
| SMARCB1 Deletion1 | ||||||||
| (diagnostic) | Poorly Differentiated Chordoma | Diagnostic | B | Supports Positive | 2 | rejected | EID11179Copy number profiling revealed loss of chromosome 22q including the SMARCB1 locus as the only recurrent alteration in 7 of 7 poorly differentiated chordomas. FISH or MLPA was used to confirm heterozy… (full text at CIViC) PMID 27067307 · Hasselblatt et al., 2016 · Open in CIViC | civic |
| SMARCB1 Loss6 | ||||||||
| (diagnostic) | Poorly Differentiated Chordoma | Diagnostic | B | Supports Positive | 3 | submitted | EID11170All 7 of 7 poorly differentiated chordomas exhibited loss of SMARCB1 expression while SMARCB1 expression was retained in 14 conventional chordomas. Copy number profiling revealed loss of chromosome 2… (full text at CIViC) PMID 27067307 · Hasselblatt et al., 2016 · Open in CIViC | civic |
| 〃 | Poorly Differentiated Chordoma | Diagnostic | B | Supports Positive | 2 | submitted | EID113369 of 9 poorly differentiated chordomas tested by IHC exhibited loss of SMARCB1 expression. 7 of 8 tested by FISH showed homozygous deletion of SMARCB1. PMID 29119645 · Owosho et al., 2018 · Open in CIViC | civic |
| (prognostic) | Poorly Differentiated Chordoma | Prognostic | B | Supports Poor Outcome | 2 | submitted | ||
| TBX2 Expression1 | ||||||||
| (diagnostic) | Poorly Differentiated Chordoma | Diagnostic | B | Supports Positive | 2 | rejected | EID11171All chordomas including 14 conventional chordomas and 7 poorly differentiated chordomas exhibited nuclear expression of brachyury (TBX2) by IHC whereas only 2 of 30 atypical teratoid/rhabdoid tumors w… (full text at CIViC) PMID 27067307 · Hasselblatt et al., 2016 · Open in CIViC | civic |
| TBXT Amplification1 | ||||||||
| (oncogenic) | Chordoma | Oncogenic | D | Supports Oncogenicity | 1 | submitted | EID11173shRNA knockdown of TBXT in the JHC7 chordoma cell line with TBXT amplification resulted in differentiation and growth arrest. PMID 21699479 · Hsu et al., 2011 · Open in CIViC | civic |
| TBXT Expression2 | ||||||||
| (diagnostic) | Poorly Differentiated Chordoma | Diagnostic | B | Supports Positive | 2 | submitted | EID11172All chordomas including 14 conventional chordomas and 7 poorly differentiated chordomas exhibited nuclear expression of brachyury (TBXT) by IHC whereas only 2 of 30 atypical teratoid/rhabdoid tumors w… (full text at CIViC) PMID 27067307 · Hasselblatt et al., 2016 · Open in CIViC | civic |
| Nivolumab | Chordoma | Predictive | C | Supports Sensitivity Response | 3 | submitted | EID12067This pediatric patients harboured a chordoma with high TBXT expression when compared with TCGA and POG570 data, and a high CD8+ infiltration score. The patient was treated with nivolumab, and experien… (full text at CIViC) PMID 34931022 · Williamson et al., 2021 · Open in CIViC | civic |
| TBXT Gain2 | ||||||||
| (oncogenic) | Chordoma | Oncogenic | D | Supports Oncogenicity | 1 | submitted | EID11174Knockdown of TBXT in the U-CH1 chordoma cell line with polysomy of chromosome 6 decreased proliferation and induced a senescence-like phenotype. PMID 21171078 · Presneau et al., 2011 · Open in CIViC | civic |
| 〃 | Chordoma | Oncogenic | D | Supports Oncogenicity | 1 | submitted | EID11175Knockdown of TBXT in the U-CH1 chordoma cell line with polysomy of chromosome 6 decreased proliferation and induced a senescence-like phenotype. PMID 21171078 · Presneau et al., 2011 · Open in CIViC | civic |
Data updated 11 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.
Seven pediatric patients with poorly differentiated chordomas, associated with loss of SMARCB1, had a median overall survival of 9 months (95% confidence level of 6 to 12 months).
PMID 27067307 · Hasselblatt et al., 2016 · Open in CIViC
| civic |
| (diagnostic) | Poorly Differentiated Chordoma | Diagnostic | C | Supports Positive | 1 | submitted | EID113402 of 2 poorly differentiated chordomas exhibited 22q loss, including SMARCB1, with no mutations identified. PMID 31135077 · Shih et al., 2019 · Open in CIViC | civic |
| Tazemetostat | Poorly Differentiated Chordoma | Predictive | C | Supports Sensitivity Response | 2 | submitted | EID11178In the dose expansion of phase I pediatric trial (NCT02601937) of the EZH2 inhibitor tazemetostat, 2 of 6 patients with SMARCB1-deficient poorly differentiated chordoma had a partial response and 1 of… (full text at CIViC) | civic |
| 〃 | Poorly Differentiated Chordoma | Predictive | C | Supports Sensitivity Response | 1 | submitted | EID11338A patient with SMARCB1-deleted poorly differentiated chordoma received the EZH2 inhibitor tazemetostat (800 mg/ twice daily) for 4 weeks as part of a phase II trial followed by radiation at the time o… (full text at CIViC) PMID 30642912 · Gounder et al., 2019 · Open in CIViC | civic |