Chordoma

Summary

Chordoma (MONDO:0008978) is a disease caused by TBXT (GenCC Strong), with 4 cohort genes and 52 clinical trials. Molecularly, EGFR Expression confers sensitivity to Lapatinib in Chordoma (CIViC Level B); 1 further subtype–drug associations are mapped below. Top therapeutic interventions include dasatinib anhydrous, imatinib, and afatinib.

At a glance

• Prevalence: <1 / 1 000 000 (United States) [Orphanet-validated]
• Causal gene: TBXT (GenCC Strong)
• Cohort genes: 4
• ClinVar variants: 17
• Clinical trials: 52
• Precision-medicine evidence (CIViC): 2 subtype–drug associations

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.06	United States	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	chordoma
Mondo ID	MONDO:0008978
MeSH	D002817
OMIM	215400
Orphanet	178
DOID	DOID:3302
ICD-11	898231522
NCIT	C2947
UMLS	C0008487
MedGen	40277
GARD	0001303
MedDRA	10008747
NORD	931
Is cancer (heuristic)	no

Also known as: CHDM · chordoma · chordoma (disease) · chordoma, malignant · chordoma, susceptibility to · notochordal sarcoma

Data availability: 17 ClinVar variants · 1 GenCC gene-disease record · 1 HPO phenotype · 34 cell lines.

Disease family

An umbrella term covering 4 Mondo subtypes.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › embryonal neoplasm › notochordal tumor › chordoma

Subtypes (4): skull base chordoma, spinal chordoma, chondroid chordoma, poorly differentiated chordoma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

ClinVar germline variants

17 retrieved; paginated sample, class counts are floors:

10 conflicting classifications of pathogenicity, 3 uncertain significance, 2 benign, 1 pathogenic/likely pathogenic, 1 pathogenic

ClinVar	Variant (HGVS)	Gene	Classification	Review
38155	NM_000059.4(BRCA2):c.8350C>T (p.Arg2784Trp)	BRCA2	Pathogenic/Likely pathogenic	criteria provided, multiple submitters, no conflicts
51493	NM_000059.4(BRCA2):c.3599_3600del (p.Asp1199_Cys1200insTer)	BRCA2	Pathogenic	reviewed by expert panel
1697217	NM_000059.4(BRCA2):c.2141A>C (p.Glu714Ala)	BRCA2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
38104	NM_000059.4(BRCA2):c.7565C>T (p.Ser2522Phe)	BRCA2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
38139	NM_000059.4(BRCA2):c.8117A>G (p.Asn2706Ser)	BRCA2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
96793	NM_000059.4(BRCA2):c.3517A>T (p.Ile1173Phe)	BRCA2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
141588	NM_024675.4(PALB2):c.1042C>A (p.Gln348Lys)	PALB2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
216750	NM_024675.4(PALB2):c.3103A>G (p.Ile1035Val)	PALB2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
225856	NM_024675.4(PALB2):c.2755G>A (p.Val919Ile)	PALB2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
232781	NM_024675.4(PALB2):c.3494C>T (p.Ser1165Leu)	PALB2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
419370	NM_024675.4(PALB2):c.1600T>G (p.Ser534Ala)	PALB2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
837352	NM_024675.4(PALB2):c.398G>C (p.Ser133Thr)	PALB2	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
1697216	NM_000059.4(BRCA2):c.6902A>G (p.Glu2301Gly)	BRCA2	Uncertain significance	no assertion criteria provided
1697218	NM_000059.4(BRCA2):c.2143G>T (p.Gly715Ter)	BRCA2	Uncertain significance	no assertion criteria provided
52248	NM_000059.4(BRCA2):c.7010C>T (p.Thr2337Ile)	BRCA2	Uncertain significance	criteria provided, multiple submitters, no conflicts
37942	NM_000059.4(BRCA2):c.5070A>C (p.Lys1690Asn)	BRCA2	Benign	reviewed by expert panel
41551	NM_000059.4(BRCA2):c.4779A>C (p.Glu1593Asp)	BRCA2	Benign	reviewed by expert panel

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 3 · Orphanet: 21 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

GenCC gene–disease validity (cohort genes)

the Disease column is the GenCC-asserted condition — a cohort gene’s strongest validity may be for a related predisposition syndrome.

Gene	Classification	Inheritance	Disease	Records
TBXT	Strong	Autosomal dominant	chordoma	3

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
TBXT	Orphanet:178	Chordoma
TBXT	Orphanet:397927	Sacral agenesis-abnormal ossification of the vertebral bodies-persistent notochordal canal syndrome
EGFR	Orphanet:251576	Gliosarcoma
EGFR	Orphanet:251579	Giant cell glioblastoma
BRCA2	Orphanet:1331	Familial prostate cancer
BRCA2	Orphanet:1333	Familial pancreatic carcinoma
BRCA2	Orphanet:145	Hereditary breast and/or ovarian cancer syndrome
BRCA2	Orphanet:178	Chordoma
BRCA2	Orphanet:227535	Hereditary breast cancer
BRCA2	Orphanet:319462	Inherited cancer-predisposing syndrome due to biallelic BRCA2 mutations
BRCA2	Orphanet:440437	Familial colorectal cancer Type X
BRCA2	Orphanet:654	Nephroblastoma
BRCA2	Orphanet:667662	Breast implant-associated anaplastic large cell lymphoma
BRCA2	Orphanet:694963	Inflammatory breast cancer
BRCA2	Orphanet:70567	Cholangiocarcinoma
BRCA2	Orphanet:84	Fanconi anemia
PALB2	Orphanet:1333	Familial pancreatic carcinoma
PALB2	Orphanet:145	Hereditary breast and/or ovarian cancer syndrome
PALB2	Orphanet:178	Chordoma
PALB2	Orphanet:227535	Hereditary breast cancer
PALB2	Orphanet:84	Fanconi anemia

Cohort genes → proteins

4 cohort genes, 4 distinct canonical proteins.

Evidence partition

Subset	Genes
civic_only	1
multi_evidence	3

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
TBXT	HGNC:11515	ENSG00000164458	O15178	T-box transcription factor T	gencc
EGFR	HGNC:3236	ENSG00000146648	P00533	Epidermal growth factor receptor	civic_evidence
BRCA2	HGNC:1101	ENSG00000139618	P51587	Breast cancer type 2 susceptibility protein	clinvar
PALB2	HGNC:26144	ENSG00000083093	Q86YC2	Partner and localizer of BRCA2	clinvar

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
TBXT	T-box transcription factor T	Involved in the transcriptional regulation of genes required for mesoderm formation and differentiation.
EGFR	Epidermal growth factor receptor	Receptor tyrosine kinase binding ligands of the EGF family and activating several signaling cascades to convert extracellular cues into appropriate cellular responses.
BRCA2	Breast cancer type 2 susceptibility protein	Involved in double-strand break repair and/or homologous recombination.
PALB2	Partner and localizer of BRCA2	Plays a critical role in homologous recombination repair (HRR) through its ability to recruit BRCA2 and RAD51 to DNA breaks.

Protein-family classification

Druggable: 1 · Difficult: 2 · Unknown: 1 · Druggable fraction: 0.25

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

Family	Genes	Fold	FDR
Kinase	1	6.9×	0.424
Scaffold/PPI	1	4.3×	0.424
Transcription factor	1	2.1×	0.538
Other/Unknown	1	0.5×	0.962

Per-gene assignment

Symbol	Family	Druggable?	EC	InterPro (top 3)
TBXT	Transcription factor	no		TF_T-box, TF_Brachyury, p53-like_TF_DNA-bd_sf
EGFR	Kinase	yes	2.7.10.1	Rcpt_L-dom, Prot_kinase_dom, Ser-Thr/Tyr_kinase_cat_dom
BRCA2	Other/Unknown	no		BRCA2_repeat, NA-bd_OB-fold, BRCA2_OB_1
PALB2	Scaffold/PPI	no		WD40/YVTN_repeat-like_dom_sf, PALB2_WD40, WD40_repeat_dom_sf

Expression context

Cohort genes with no expression data: 0.

3 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

Bucket	Genes
narrow (1-5 tissues)	0
moderate (6-20)	0
broad (>20)	4
unknown	0

Top tissues across cohort

Tissue	Cohort genes
male germ line stem cell (sensu Vertebrata) in testis	2
secondary oocyte	2
pancreatic ductal cell	1
primordial germ cell in gonad	1
gingiva	1
gingival epithelium	1
nipple	1
ventricular zone	1
buccal mucosa cell	1
oocyte	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
TBXT	42	tissue_specific	marker	primordial germ cell in gonad, pancreatic ductal cell, male germ line stem cell (sensu Vertebrata) in testis
EGFR	285	ubiquitous	marker	nipple, gingiva, gingival epithelium
BRCA2	184	ubiquitous	marker	male germ line stem cell (sensu Vertebrata) in testis, secondary oocyte, ventricular zone
PALB2	232	ubiquitous	yes	secondary oocyte, buccal mucosa cell, oocyte

Protein interactions among cohort

Intra-cohort edges: 1.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
EGFR	18,421
PALB2	5,641
BRCA2	4,839
TBXT	1,028

Intra-cohort edges

A	B	Sources
BRCA2	PALB2	biogrid_interaction, intact, string_interaction

Structural data

PDB: 4 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
EGFR	P00533	388
TBXT	O15178	56
BRCA2	P51587	14
PALB2	Q86YC2	4

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 74. Enrichment computed across 4 evidence-associated genes (4 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 4 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
Impaired BRCA2 binding to PALB2	2	228.4×	5e-04	BRCA2, PALB2
Defective homologous recombination repair (HRR) due to BRCA1 loss of function	2	211.5×	5e-04	BRCA2, PALB2
Defective HDR through Homologous Recombination Repair (HRR) due to PALB2 loss of BRCA1 binding function	2	211.5×	5e-04	BRCA2, PALB2
Defective HDR through Homologous Recombination Repair (HRR) due to PALB2 loss of BRCA2/RAD51/RAD51C binding function	2	211.5×	5e-04	BRCA2, PALB2
Resolution of D-loop Structures through Synthesis-Dependent Strand Annealing (SDSA)	2	196.9×	5e-04	BRCA2, PALB2
Homologous DNA Pairing and Strand Exchange	2	190.3×	5e-04	BRCA2, PALB2
Resolution of D-loop Structures through Holliday Junction Intermediates	2	150.3×	7e-04	BRCA2, PALB2
HDR through Homologous Recombination (HRR)	2	95.2×	0.001	BRCA2, PALB2
Impaired BRCA2 translocation to the nucleus	1	951.7×	0.008	BRCA2
Impaired BRCA2 binding to SEM1 (DSS1)	1	951.7×	0.008	BRCA2
PLCG1 events in ERBB2 signaling	1	713.8×	0.009	EGFR
PTK6 promotes HIF1A stabilization	1	407.9×	0.014	EGFR
Epithelial-Mesenchymal Transition (EMT) during gastrulation	1	356.9×	0.014	TBXT
Inhibition of Signaling by Overexpressed EGFR	1	317.2×	0.014	EGFR
EGFR interacts with phospholipase C-gamma	1	285.5×	0.014	EGFR
EGFR Transactivation by Gastrin	1	285.5×	0.014	EGFR
Defective homologous recombination repair (HRR) due to PALB2 loss of function	1	237.9×	0.014	BRCA2
HDR through MMEJ (alt-NHEJ)	1	219.6×	0.014	BRCA2
ERBB2 Activates PTK6 Signaling	1	203.9×	0.014	EGFR
Diseases of DNA Double-Strand Break Repair	1	203.9×	0.014	BRCA2
Defective homologous recombination repair (HRR) due to BRCA2 loss of function	1	203.9×	0.014	BRCA2
Formation of axial mesoderm	1	203.9×	0.014	TBXT
GRB2 events in EGFR signaling	1	190.3×	0.014	EGFR
TFAP2 (AP-2) family regulates transcription of growth factors and their receptors	1	190.3×	0.014	EGFR
SHC1 events in EGFR signaling	1	178.4×	0.014	EGFR
Constitutive Signaling by EGFRvIII	1	178.4×	0.014	EGFR
ERBB2 Regulates Cell Motility	1	178.4×	0.014	EGFR
Formation of definitive endoderm	1	178.4×	0.014	TBXT
PI3K events in ERBB2 signaling	1	167.9×	0.014	EGFR
Signaling by ERBB2 ECD mutants	1	167.9×	0.014	EGFR

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 4 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
inner cell mass cell proliferation	2	495.6×	5e-04	BRCA2, PALB2
mesoderm development	2	263.3×	1e-03	TBXT, PALB2
somitogenesis	2	187.2×	0.001	TBXT, PALB2
negative regulation of cardiocyte differentiation	1	4213.0×	0.005	EGFR
double-strand break repair via homologous recombination	2	78.0×	0.005	BRCA2, PALB2
mitotic recombination-dependent replication fork processing	1	2106.5×	0.008	BRCA2
positive regulation of protein kinase C signaling	1	1404.3×	0.010	EGFR
morphogenesis of an epithelial fold	1	1053.2×	0.010	EGFR
response to UV-A	1	1053.2×	0.010	EGFR
negative regulation of mammary gland epithelial cell proliferation	1	842.6×	0.010	BRCA2
regulation of peptidyl-tyrosine phosphorylation	1	842.6×	0.010	EGFR
salivary gland morphogenesis	1	601.9×	0.012	EGFR
anterior/posterior axis specification, embryo	1	526.6×	0.012	TBXT
protein insertion into membrane	1	526.6×	0.012	EGFR
establishment of protein localization to telomere	1	526.6×	0.012	BRCA2
ubiquitin-dependent endocytosis	1	468.1×	0.012	EGFR
response to UV-C	1	421.3×	0.012	BRCA2
ERBB2-EGFR signaling pathway	1	421.3×	0.012	EGFR
telomere maintenance via recombination	1	383.0×	0.012	BRCA2
cerebral cortex cell migration	1	383.0×	0.012	EGFR
cardiac muscle cell myoblast differentiation	1	351.1×	0.012	TBXT
primitive streak formation	1	351.1×	0.012	TBXT
regulation of DNA damage checkpoint	1	280.9×	0.015	BRCA2
eyelid development in camera-type eye	1	263.3×	0.015	EGFR
digestive tract morphogenesis	1	247.8×	0.015	EGFR
centrosome duplication	1	234.1×	0.015	BRCA2
response to X-ray	1	221.7×	0.015	BRCA2
positive regulation of phosphorylation	1	210.7×	0.015	EGFR
xenobiotic transport	1	210.7×	0.015	EGFR
female gonad development	1	200.6×	0.015	BRCA2

Therapeutics

Drugs indicated for this disease

No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Afatinib, Cetuximab, Imatinib, Nivolumab, Palbociclib, Relatlimab.

Drug target analysis

Approved (phase 4): 1 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 3

Druggability breadth: 2 of 4 evidence-associated genes (50%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Genes with an approved drug

The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.

Symbol	Example approved molecule
EGFR	LEVODOPA

Top cohort targets by molecule count

Symbol	Molecules	Max phase
EGFR	175	4
TBXT	0	0
BRCA2	0	0
PALB2	0	0

Drugs targeting cohort genes (top 30)

Molecule	Max phase	Targets in cohort
LEVODOPA	4	EGFR
CLOTRIMAZOLE	4	EGFR
ERLOTINIB HYDROCHLORIDE	4	EGFR
CISPLATIN	4	EGFR
PONATINIB	4	EGFR
AFATINIB	4	EGFR
CHROMIC CHLORIDE	4	EGFR
BACITRACIN	4	EGFR
ZINC CHLORIDE	4	EGFR
LAPATINIB DITOSYLATE	4	EGFR
VEMURAFENIB	4	EGFR
FEDRATINIB	4	EGFR
AXITINIB	4	EGFR
SORAFENIB	4	EGFR
DASATINIB ANHYDROUS	4	EGFR
NICLOSAMIDE	4	EGFR
SELUMETINIB	4	EGFR
TERFENADINE	4	EGFR
ALECTINIB	4	EGFR
NERATINIB	4	EGFR
IBRUTINIB	4	EGFR
AFATINIB DIMALEATE	4	EGFR
CABOZANTINIB	4	EGFR
DACOMITINIB	4	EGFR
DACOMITINIB ANHYDROUS	4	EGFR
CERITINIB	4	EGFR
VANDETANIB	4	EGFR
TRIBROMSALAN	4	EGFR
BOSUTINIB	4	EGFR
BITHIONOL	4	EGFR

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 1.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

Symbol	Assays	Type breakdown
EGFR	6,531	Binding:6211, Functional:173, ADMET:138, Toxicity:9
TBXT	1	Binding:1

Cohort enzymes (BRENDA EC)

Symbol	EC numbers	Names
EGFR	2.7.10.1	receptor protein-tyrosine kinase

Cohort genes with high screening signal

≥100 ChEMBL assays — a studied-ness signal; see Therapeutics for approved-drug status.

Symbol	ChEMBL assays
EGFR	6,531

Pharmacogenomics

Cohort genes with a PharmGKB record: 4; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Chemical tractability of cohort targets

28 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.

Compound	Max phase	Cohort target (bioactivity)
LEVODOPA	4	EGFR
CLOTRIMAZOLE	4	EGFR
ERLOTINIB HYDROCHLORIDE	4	EGFR
CISPLATIN	4	EGFR
PONATINIB	4	EGFR
CHROMIC CHLORIDE	4	EGFR
BACITRACIN	4	EGFR
ZINC CHLORIDE	4	EGFR
LAPATINIB DITOSYLATE	4	EGFR
VEMURAFENIB	4	EGFR
FEDRATINIB	4	EGFR
AXITINIB	4	EGFR
SORAFENIB	4	EGFR
NICLOSAMIDE	4	EGFR
SELUMETINIB	4	EGFR
TERFENADINE	4	EGFR
ALECTINIB	4	EGFR
NERATINIB	4	EGFR
IBRUTINIB	4	EGFR
AFATINIB DIMALEATE	4	EGFR
CABOZANTINIB	4	EGFR
DACOMITINIB	4	EGFR
DACOMITINIB ANHYDROUS	4	EGFR
CERITINIB	4	EGFR
VANDETANIB	4	EGFR
TRIBROMSALAN	4	EGFR
BOSUTINIB	4	EGFR
BITHIONOL	4	EGFR

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	1	EGFR
B	Phased (≥1) drug, not yet approved	0
C	Druggable family + PDB, no drug	0
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	3	TBXT, BRCA2, PALB2

Undrugged target profiles

3 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

Symbol	ChEMBL assays	Drugged partners (top 3)
TBXT	1	—
BRCA2	0	—
PALB2	0	—

Clinical trials & evidence

Clinical trials

Clinical trials: 52.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	22
PHASE2	16
PHASE1/PHASE2	7
PHASE1	6
PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01182779	PHASE3	UNKNOWN	Trial of Proton Versus Carbon Ion Radiation Therapy in Patients With Chordoma of the Skull Base
NCT00496119	PHASE2	ACTIVE_NOT_RECRUITING	Proton Beam Therapy for Chordoma Patients
NCT02834013	PHASE2	ACTIVE_NOT_RECRUITING	Nivolumab and Ipilimumab in Treating Patients With Rare Tumors
NCT04416568	PHASE2	ACTIVE_NOT_RECRUITING	Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative Cancers
NCT05041127	PHASE2	ACTIVE_NOT_RECRUITING	Cetuximab for the Treatment of Advanced Unresectable or Metastatic Chordoma
NCT05286801	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Tiragolumab and Atezolizumab for the Treatment of Relapsed or Refractory SMARCB1 or SMARCA4 Deficient Tumors
NCT05407441	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Tazemetostat+Nivo/Ipi in INI1-Neg/SMARCA4-Def Tumors
NCT06625190	PHASE1/PHASE2	RECRUITING	Alpha/Beta T and B Cell Depletion With Zoledronic Acid for Solid Tumors
NCT06787664	PHASE2	ACTIVE_NOT_RECRUITING	A Study of BL-B01D1 in Patients With Locally Advanced or Metastatic Chordoma
NCT06794645	PHASE2	RECRUITING	Pembrolizumab and Pemetrexed for Progressive Chordoma
NCT06957327	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	A Study of ERAS-601 in People With Chordoma
NCT00150072	PHASE2	COMPLETED	Efficacy and Safety of Imatinib in Chordoma
NCT00464620	PHASE2	COMPLETED	Trial of Dasatinib in Advanced Sarcomas
NCT00592748	PHASE1/PHASE2	COMPLETED	Charged Particle RT for Chordomas and Chondrosarcomas of the Base of Skull or Cervical Spine
NCT02383498	PHASE2	COMPLETED	QUILT-3.011 Phase 2 Yeast-Brachyury Vaccine Chordoma
NCT03083678	PHASE2	COMPLETED	Afatinib in Locally Advanced and Metastatic Chordoma
NCT03110744	PHASE2	COMPLETED	CDK4/6 Inhibition in Locally Advanced/Metastatic Chordoma
NCT03190174	PHASE1/PHASE2	COMPLETED	Nivolumab (Opdivo®) Plus ABI-009 (Nab-rapamycin) for Advanced Sarcoma and Certain Cancers
NCT03242382	PHASE2	UNKNOWN	Trial of Palbociclib in Second Line of Advanced Sarcomas With CDK4 Overexpression.
NCT03595228	PHASE2	COMPLETED	BN Brachyury and Radiation in Chordoma
NCT03623854	PHASE2	COMPLETED	Nivolumab and Relatlimab in Treating Participants With Advanced Chordoma
NCT03647423	PHASE1/PHASE2	WITHDRAWN	QUILT-3.091 NANT Chordoma Vaccine vs Radiation in Subjects With Unresectable Chordoma.
NCT04042597	PHASE2	UNKNOWN	Anlotinib Hydrochloride Versus Imatinib Mesylate in Locally Advanced, Unresectable or Metastatic Chordoma
NCT06140732	PHASE2	UNKNOWN	Apatinib Combined With Camrelizumab in Treating Participants With Advanced Chordoma
NCT02989636	PHASE1	ACTIVE_NOT_RECRUITING	Nivolumab With or Without Stereotactic Radiosurgery in Treating Patients With Recurrent, Advanced, or Metastatic Chordoma
NCT00003926	PHASE1	TERMINATED	Amifostine to Protect From Side Effects of PSCT in Treating Patients With Solid Tumors
NCT01175109	PHASE1	UNKNOWN	Study of Imatinib, a Platelet-derived Growth Factor Receptor Inhibitor, and LBH589, a Histone Deacetylase Inhibitor, in the Treatment of Newly Diagnosed and Recurrent Chordoma
NCT01407198	PHASE1	COMPLETED	Nilotinib With Radiation for High Risk Chordoma
NCT03955042	PHASE1	COMPLETED	Pemetrexed for the Treatment of Chordoma
NCT04246671	PHASE1	COMPLETED	TAEK-VAC-HerBy Vaccine for Brachyury and HER2 Expressing Cancer
NCT02986516	Not specified	RECRUITING	Sacral Chordoma: Surgery Versus Definitive Radiation Therapy in Primary Localized Disease
NCT03910465	Not specified	RECRUITING	Children and Adults With Chordoma
NCT04087902	Not specified	ACTIVE_NOT_RECRUITING	Long-Term Longitudinal QoL in Patients Undergoing EEA
NCT04091295	Not specified	AVAILABLE	BLESSED: Expanded Access for DNG64-CAR-V for Advanced Pancreatic Cancer, Sarcoma and Carcinoma of Breast
NCT04832620	Not specified	RECRUITING	Image Assisted Optimization of Proton Radiation Therapy in Chordomas and Chondrosarcomas
NCT05033288	Not specified	RECRUITING	Comparing Carbon Ion Therapy, Surgery, and Proton Therapy for Management of Pelvic Sarcomas Involving the Bone
NCT05861245	Not specified	RECRUITING	Hypofractionated Protontherapy in Chordomas and Chondrosarcomas of the Skull Base
NCT06029218	Not specified	RECRUITING	Analysis of the Toxicity and Efficacy of Daily 1 vs 2 Beam Proton Therapy
NCT07005297	Not specified	NOT_YET_RECRUITING	Clinical Genetics Branch Eligibility Screening Survey
NCT00341627	Not specified	COMPLETED	Genetic Aspects of Chordoma: A Collaboration With SEER Registries to Identify Chordoma Families

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DASATINIB ANHYDROUS	4	2
IMATINIB	4	2
AFATINIB	4	1
AMIFOSTINE	4	1
FLUDEOXYGLUCOSE F 18	4	1
RELATLIMAB	4	1
TAZEMETOSTAT	4	1
CATEQUENTINIB HYDROCHLORIDE	3	1
NOGAPENDEKIN ALFA	3	1
TIRAGOLUMAB	3	1
ALDOXORUBICIN HYDROCHLORIDE	2	1
CHEMBL365847	0	2
CHEMBL4583196	0	1
CHEMBL2347958	0	1
CHEMBL4283673	0	1
CHEMBL4759112	0	1
CHEMBL5572229	0	1
CHEMBL5398431	0	1

Precision-medicine subtype map (CIViC)

Drug × molecular subtype: 2 predictive associations from 2 curated evidence items; also 3 oncogenic.

Molecular subtype	Therapy	Effect	Level	CIViC
EGFR Expression	Lapatinib	Sensitivity/Response	CIViC B	EID1984
TBXT Expression	Nivolumab	Sensitivity/Response	CIViC C	EID12067