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Chordoma is a slow-growing bone cancer (a sarcoma) of the skull base and spine that arises from leftover embryonic notochord cells. Complete surgery followed by high-dose proton or carbon-ion radiotherapy controls most tumours, and the whole disease depends on a single transcription factor, brachyury, which vaccines and degraders are now trying to hit.
Chordoma arises from notochordal remnants along the axial skeleton (clivus, mobile spine, sacrum). Nearly all tumours express the T-box transcription factor brachyury (TBXT), a lineage dependency rather than a mutation: a common germline TBXT variant raises risk, and germline TBXT duplication causes familial chordoma. Conventional and chondroid chordomas are indolent but locally destructive; dedifferentiated and poorly differentiated chordomas are aggressive. Poorly differentiated chordoma, seen mainly in children, is defined by SMARCB1 (INI1) loss, placing it in the SWI/SNF-deficient family with epithelioid sarcoma and ATRT.
Curative treatment is en bloc resection with negative margins where anatomy permits, followed by high-dose radiotherapy, because the tumour is radioresistant at conventional doses and sits against the brainstem, cranial nerves or sacral roots. Proton and carbon-ion therapy deliver 70 Gy-equivalent or more while sparing neural tissue, and definitive particle therapy is used when resection is impossible. Systemic options are limited: imatinib (PDGFRB-expressing disease, phase 2 Stacchiotti 2012) and afatinib (EGFR) give disease stabilisation more often than shrinkage, and are not approved. Tazemetostat is being studied in INI1-negative poorly differentiated chordoma on the basis of its epithelioid sarcoma activity.
The frontier is brachyury itself: a yeast-based brachyury vaccine (GI-6301) and adenoviral brachyury vaccines have been tested with radiotherapy, and brachyury degraders and CDK7/9 inhibitors that lower TBXT expression are in preclinical or early clinical development. The Chordoma Foundation coordinates a global registry, biobank and drug-screening pipeline, a model for how a rare cancer community can run its own research programme.
About one case per million people per year, at any age; skull-base tumours are more common in children, sacral tumours in older adults.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
En bloc resection with negative margins by a spine or skull-base team, followed by high-dose proton or carbon-ion radiotherapy; intralesional surgery is associated with early recurrence.
Definitive particle therapy (proton or carbon-ion) to 70 Gy-equivalent or higher; stereotactic photon radiosurgery where particles are unavailable.
Clinical trial preferred. Imatinib (PDGFRB-positive), afatinib or other EGFR inhibitors, or sorafenib give mainly disease stabilisation; tazemetostat was used for INI1-negative poorly differentiated chordoma until Ipsen withdrew it from all markets in March 2026.
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Query for this cancer: (TITLE:"Chordoma" OR ABSTRACT:"Chordoma" OR TITLE:"Childhood chordoma" OR ABSTRACT:"Childhood chordoma" OR TITLE:"Clival chordoma" OR ABSTRACT:"Clival chordoma" OR TITLE:"Sacral chordoma" OR ABSTRACT:"Sacral chordoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Chordoma, not a curated reading list.
First description of the notochord-derived tumour.
Vujovic and colleagues show near-universal nuclear brachyury expression.
Yang and colleagues (Nat Genet).
Stacchiotti and colleagues: disease stabilisation in most patients, few objective responses.
First international recommendations on surgery, radiotherapy and systemic therapy (Lancet Oncol).
WHO classification of soft tissue and bone tumours, fifth edition.
Phase 2 of GI-6301 yeast-brachyury vaccine with radiation (NCT02383498).
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Take on an empty stomach; a high-fat meal cuts exposure by half.
Take with a meal and a large glass of water.
Take without food (1 hour before or 2 hours after).
Possible QT prolongation. Check ECG and electrolytes; review other QT-prolonging drugs.
See all on the product pages:AfatinibImatinibSorafenib·Printable cards in the navigator
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Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.