Oncology

Chordoma

Also known as sacrococcygeal chordoma, clival chordoma, brachyury/T gene tumor

Chordoma is a rare, slow-growing but locally aggressive malignant bone tumour arising from remnants of the embryonic notochord and occurring predominantly at the skull base (clivus), mobile spine, and sacrococcygeal region. The transcriptio

ORPHA:178 ↗Gene TBXT (T gene)Prevalence 1 in 1,000,000 (approximately 300 new cases/year in the USA)Onset Adult (median age 50s; clival tumours present earlier)Sporadic; rare familial cases with TBXT duplication

11

studies recruiting now

as of 7 Sept 2026

75

studies registered in total

as of 7 Sept 2026

11

countries with a recruiting site

as of 7 Sept 2026

3 Oct 2024

most recent study posted

among recruiting studies

Recruiting trials

Showing the 5 most recently updated recruiting studies, as recorded 7 Sept 2026. Live status on each study page.

See all 11 recruiting studies

Where recruiting studies are running

Countries with at least one recruiting site among the studies above, 7 Sept 2026. Tap a country to search trials there.

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Patient organisations

Chordoma FoundationPatient association
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Registry: Chordoma Foundation Patient Registry · Join ↗. Registries connect patients to researchers and often hear about trials first.

About Chordoma

Chordoma is a rare, slow-growing but locally aggressive malignant bone tumour arising from remnants of the embryonic notochord and occurring predominantly at the skull base (clivus), mobile spine, and sacrococcygeal region. The transcription factor brachyury (encoded by TBXT) is a specific and sensitive diagnostic marker overexpressed in virtually all chordomas, and germline duplication of TBXT is associated with familial predisposition. Despite its slow growth, chordoma is characterised by high rates of local recurrence after surgery, resistance to conventional radiotherapy doses, and a paucity of effective systemic therapies.

Common clinical features

Sacrococcygeal pain: dull, progressive lower back or tailbone pain, often present for years before diagnosisCranial nerve palsies from clival tumours: diplopia (CN VI palsy most common), facial numbness, or dysphagiaBowel and bladder dysfunction from sacral nerve root compressionMotor weakness or myelopathy from spinal cord compression by vertebral chordomasNasal obstruction, epistaxis, or headache from large clival tumours extending into the nasopharynxPalpable presacral or posterior pharyngeal mass on examinationDistant metastases to lung, bone, liver, and lymph nodes in advanced disease

From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.

Treatments being studied

7 in clinical development, from Open Targets (CC BY 4.0). Not medical advice.

Phase 2Imatinib
Phase 2Afatinib (Gilotrif)
Phase 2Cetuximab (Erbitux)
Phase 2Palbociclib (Ibrance)
Phase 1Nivolumab (Nivolumab bms)
Phase 1Nilotinib
Phase 1Pemetrexed (Alimta)

Before you apply

Things trial teams commonly ask about for Chordoma. Not eligibility rules; those are set by each study.

  • Brachyury (TBXT) immunohistochemistry is the diagnostic standard — confirm pathology report documents positive brachyury staining to satisfy most trial eligibility requirements.
  • Prior treatment history (surgery and proton beam or carbon ion radiotherapy) significantly affects eligibility; document the number of prior resections, radiation doses, and fields treated.
  • Molecular profiling for targetable alterations (CDKN2A/B deletion, PI3K pathway, receptor tyrosine kinase expression) is increasingly required for biomarker-selected trials — request comprehensive tumour genomic profiling if not already performed.

Related conditions

Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).