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chordoma
Chordoma
Chordoma arises from notochordal remnants; skull base (~35%), sacrococcygeal (~50%), mobile spine (~15%); TBXT overexpression in >95%; physaliferous cell histology; proton RT + surgery standard; no FDA-approved systemic agent; imatinib, sorafenib, mTOR inhibitors active.
Entry Metadata
| Field | Value |
|---|---|
| ID | chordoma |
| Name | Chordoma |
| Status | draft |
| Last reviewed | 2026-06-06 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Stacchiotti S, Longhi A, Ferraresi V, et al. Phase II study of imatinib in advanced chordoma. J Clin Oncol. 2012;30(9):914-920. · PubMed 22330157
- Yang XR, Ng D, Alcorta DA, et al. T (brachyury) gene duplication confers major susceptibility to familial chordoma. Nat Genet. 2009;41(11):1176-1178. · PubMed 19801977