Disease · MONDO_0008978

chordoma

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. chordoma. Diseases, record MONDO_0008978. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0008978

On this page

Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: chordoma; Open Targets
Open Targets ID: MONDO_0008978; Open Targets
Other names: Chordoma (morphologic abnormality), Chordomas, chordoma (disease), chordoma, malignant, notochordal sarcoma, notochordoma; Open Targets
Disease Ontology: DOID:3302; Disease Ontology
MeSH: D002817; MeSH
NCI Thesaurus: C2947; NCI Thesaurus
Orphanet: ORPHA:178; Orphanet
Highest stage of its drugs: Phase 2, for 16 of its 28 drugs, from ClinicalTrials.gov; Open Targets

Drugs28

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
NIVOLUMAB	CHEMBL2108738	Antibody	Phase 2	ClinicalTrials.gov	5	5	5	Drugs and targets
AFATINIB	CHEMBL1173655	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
CETUXIMAB	CHEMBL1201577	Antibody	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
IMATINIB MESYLATE	CHEMBL1642	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
PALBOCICLIB	CHEMBL189963	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
PEMETREXED	CHEMBL225072	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
REGORAFENIB	CHEMBL1946170	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
CAMRELIZUMAB	CHEMBL4297715	Antibody	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
CATEQUENTINIB HYDROCHLORIDE	CHEMBL5314570	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
DURVALUMAB	CHEMBL3301587	Antibody	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 28

See all 28 drugs

Targets46

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
ABL1	Bcr/Abl fusion protein	4	Bcr/Abl fusion protein inhibitor; Tyrosine-protein kinase ABL inhibitor	Phase 2	https://conductscience.org/library/genes/25	Drugs and targets
BCR	Bcr/Abl fusion protein	3	Bcr/Abl fusion protein inhibitor	Phase 2	https://conductscience.org/library/genes/613	Drugs and targets
KIT	Mast/stem cell growth factor receptor Kit	3	Stem cell growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/3815	Drugs and targets
PDCD1	Programmed cell death protein 1	3	Programmed cell death protein 1 antagonist; Programmed cell death protein 1 inhibitor	Phase 2	https://conductscience.org/library/genes/5133	Drugs and targets
PDGFRB	Platelet-derived growth factor receptor	3	Platelet-derived growth factor receptor beta inhibitor; Platelet-derived growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/5159	Drugs and targets
CD274	Programmed cell death 1 ligand 1	2	Programmed cell death 1 ligand 1 inhibitor; Programmed cell death 1 ligand 1 other	Phase 2	https://conductscience.org/library/genes/29126	Drugs and targets
CTLA4	Cytotoxic T-lymphocyte protein 4	2	Cytotoxic T-lymphocyte protein 4 inhibitor	Phase 2	https://conductscience.org/library/genes/1493	Drugs and targets
EGFR	Epidermal growth factor receptor	2	Epidermal growth factor receptor erbB1 inhibitor	Phase 2	https://conductscience.org/library/genes/1956	Drugs and targets
ERBB2	Receptor tyrosine-protein kinase erbB-2	2	Receptor protein-tyrosine kinase erbB-2 inhibitor	Phase 2	https://conductscience.org/library/genes/2064	Drugs and targets
KDR	Vascular endothelial growth factor receptor	2	Vascular endothelial growth factor receptor 2 inhibitor; Vascular endothelial growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/3791	Drugs and targets

1 to 10 of 46

See all 46 targets

Trials24

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT02601937	EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma	Phase 1	109	2024-10-03	Rhabdoid Tumors, INI1-negative Tumors, Synovial Sarcoma, Malignant Rhabdoid Tumor of Ovary	TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT02815995	Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes	Phase 2	57	2025-06-12	Advanced and/or Metastatic Sarcoma	DURVALUMAB, TREMELIMUMAB	Its record names the disease	ClinicalTrials.gov
NCT03190174	Nivolumab (Opdivo®) Plus ABI-009 (Nab-rapamycin) for Advanced Sarcoma and Certain Cancers	Phase 1/2	34	2025-02-17	Ewing Sarcoma, PEComa, Epithelioid Sarcoma, Desmoid Tumor, Chordoma, Non Small Cell Lung Cancer, Urothelial Carcinoma, Melanoma, Renal Cell Carcinoma, Squamous Cell Carcinoma, Hepatocellular Carcinoma, Classical Hodgkin Lymphoma, Colorectal Cancer, MTOR Activating Mutation	NIVOLUMAB, SIROLIMUS	Its record names the disease	ClinicalTrials.gov
NCT03886311	Talimogene Laherparepvec, Nivolumab and Trabectedin for Sarcoma	Phase 2	200		Sarcoma	NIVOLUMAB, TALIMOGENE LAHERPAREPVEC, TRABECTEDIN	Its record names the disease	ClinicalTrials.gov
NCT04055220	Efficacy and Safety of Regorafenib as Maintenance Therapy After First-line Treatment in Patients With Bone Sarcomas	Not applicable	168		Bone Sarcoma, Osteosarcoma, Ewing Sarcoma, Chondrosarcoma, Undifferentiated Pleomorphic Sarcoma, Leiomyosarcoma, Angiosarcoma	REGORAFENIB	Its record names the disease	ClinicalTrials.gov
NCT02389244	A Phase II Study Evaluating Efficacy and Safety of Regorafenib in Patients With Metastatic Bone Sarcomas	Phase 2	163		Ewing Sarcomas, Chondrosarcomas, Osteosarcomas, Chondroma, CIC-Rearranged Sarcoma	REGORAFENIB	Its record names the disease	ClinicalTrials.gov
NCT04042597	Anlotinib Hydrochloride Versus Imatinib Mesylate in Locally Advanced, Unresectable or Metastatic Chordoma	Phase 2	60		Effect of Drugs, Quality of Life, Chordoma, Advanced Cancer	CATEQUENTINIB HYDROCHLORIDE, IMATINIB MESYLATE	Its record names the disease	ClinicalTrials.gov
NCT00150072	Efficacy and Safety of Imatinib in Chordoma	Phase 2	55		Chordoma	IMATINIB MESYLATE	Its record names the disease	ClinicalTrials.gov
NCT05407441	Tazemetostat+Nivo/Ipi in INI1-Neg/SMARCA4-Def Tumors	Phase 1/2	49		Atypical Teratoid Rhabdoid Tumor, INI1 (SMARCB1)-Deficient Primary CNS Malignant Tumors, SMARCA4-deficient Primary CNS Malignant Tumors, Malignant Rhabdoid Tumor (MRT), Rhabdoid Tumor of the Kidney (RTK), Epithelioid Sarcoma, Chordoma	IPILIMUMAB, NIVOLUMAB, TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT03083678	Afatinib in Locally Advanced and Metastatic Chordoma	Phase 2	43		Chordoma	AFATINIB	Its record names the disease	ClinicalTrials.gov

1 to 10 of 24

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Comparisons in its trials0

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

None in this release.

Papers from its trials11

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
37429302	RESULT	1	PubMed
24089446	RESULT	1	PubMed
20572041	RESULT	1	PubMed
38501690	DERIVED	1	PubMed
37147496	DERIVED	1	PubMed
35934010	DERIVED	1	PubMed
34266694	DERIVED	1	PubMed
33895682	DERIVED	1	PubMed
31488216	DERIVED	1	PubMed
31401903	DERIVED	2	PubMed

1 to 10 of 11

See all 11 papers from its trials

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0008978

GET https://conductscience.org/api/diseases/v1/MONDO_0008978/drugs?page=2

API reference For AI assistants