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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
connects-toTbxt
connects-toMtor
connects-toFgfr
connects-toPdgf
connects-toPten
connects-toCdk4 6
connects-toCdkn2A
connects-toEwing Sarcoma
connects-toMusculoskeletal System
connects-toBrain
connects-toPhoton
connects-toOsteosarcoma
connects-toOsteoblast
connects-toMeningioma
connects-toFibroblast
connects-toSynovial Sarcoma
connects-toEgfr
connects-toNeuron
connects-toCollagen
connects-toProton
connects-toNervous System
connects-toCarbon
connects-toLung
connects-toImmune System
connects-toOxygen
connects-toOsteoclast
connects-toVegf
connects-toP53
connects-toAkt
connects-toMet
connects-toT Cytotoxic Cell
connects-toCalcium
connects-toMacrophage
connects-toLarge Intestine
connects-toPeripheral Nerve
connects-toLiver
connects-toEndothelial Cell
connects-toElectron
connects-toBone Marrow
connects-toEye
connects-toAntibody
connects-toReproductive System
connects-toSmooth Muscle Cell
connects-toTuberous Sclerosis Complex
connects-toRegulatory T Cell
connects-toHydrogen
connects-toSmarcb1
connects-toAtypical Teratoid Rhabdoid Tumor
connects-toNatural Killer Cell
connects-toYap1
connects-toNeuropathic Pain
connects-toMultiple Myeloma
connects-toStat3
connects-toVenous Thromboembolism
connects-toSepsis
connects-toOsteoporosis
connects-toMajor Depressive Disorder
connects-toAnemia Of Chronic Disease
connects-toWound Healing
connects-toStroke
connects-toCkd
connects-toDigestive System
connects-toEndocrine System
connects-toGeneralized Anxiety Disorder
connects-toRenal System
connects-toCardiovascular System
connects-toRespiratory System
connects-toIntegumentary System
connects-toLymphatic System
connects-toStreptococcus Pneumoniae
connects-toTargeted Therapy
connects-toRhabdomyosarcoma
connects-toCortical Bone
connects-toCancer Chemotherapy
connects-toCheckpoint Inhibitors
connects-toAxonal Transport
connects-toAlveolus
connects-toSchwannomatosis
connects-toHepatic Lobule
connects-toRenal Cell Carcinoma
connects-toOvarian Clear Cell Carcinoma
connects-toNsclc
connects-toEzh2
connects-toKit
connects-toTert
connects-toHif 1Alpha
connects-toMyc
connects-toCyclin D1
connects-toRankl
connects-toIgf 1
connects-toCcl2
connects-toCxcl12
connects-toNotch
connects-toCaspase 3
connects-toPerforin
connects-toWnt Beta Catenin
connects-toTgf Beta
connects-toErk1 2
connects-toPik3Ca
connects-toE2F1
connects-toRb1
connects-toJak1 2
connects-toBcl 2
connects-toGalectin 3
connects-toSmad4
connects-toCgas Sting
connects-toStat1
connects-toFoxo
connects-toGsk 3B
connects-toSrc Kinase
connects-toMdm2
connects-toS100A8 A9
connects-toDnmt3A
connects-toAutophagy
connects-toArid1A
connects-toAmpk
connects-toCcr5
connects-toIl 6
connects-toIl 1B
connects-toIl 33
connects-toComplement C3
connects-toIl 17A
connects-toCalcineurin
connects-toOsteopontin
connects-toMhc Class Ii
connects-toPd 1
connects-toIl 2
connects-toT Helper Cell
connects-toIl 10
connects-toNitric Oxide
connects-toProstaglandins
connects-toXanthine Oxidase
connects-toIl 4
connects-toIl 13
connects-toPhoton
connects-toOsteoblast
connects-toCopper
connects-toZinc
connects-toLeptin
connects-toAdiponectin
connects-toResistin
connects-toNeutrophil
connects-toIfn Gamma
connects-toIl 12
connects-toType I Interferon
connects-toIl 5
connects-toIl 23
connects-toIge
connects-toMast Cell
connects-toDendritic Cell
connects-toC5Ar1
connects-toComplement C5
connects-toFactor H
connects-toTransferrin
connects-toTslp
connects-toBradykinin
connects-toErythropoietin
connects-toHistamine
connects-toMelatonin
connects-toC1 Esterase Inhibitor
connects-toTestosterone
connects-toSerotonin
connects-toProlactin
connects-toOxytocin
connects-toVasopressin
connects-toSelenium
connects-toIodine
connects-toMagnesium
connects-toPhosphorus
connects-toSodium
connects-toPotassium
connects-toIron
connects-toChloride
connects-toNitrogen
connects-toSulfur
connects-toGlp 1
connects-toAngiotensin Ii
connects-toFibronectin
connects-toActivin A
connects-toCgrp
connects-toCalcitonin
connects-toSubstance P
connects-toInsulin Receptor
connects-toAldosterone
connects-toAndrogen Receptor
connects-toNorepinephrine
connects-toAdrenomedullin
connects-toBdnf
connects-toEpinephrine
connects-toRenin
connects-toMyostatin
connects-toAngiopoietin
connects-toCortisol
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