Chondrosarcoma

Summary

Chondrosarcoma (MONDO:0008977) is a disease caused by EXT1 (GenCC Strong), with 3 cohort genes and 53 clinical trials. Molecularly, IDH1 R132 confers sensitivity to Ivosidenib in Chondrosarcoma (CIViC Level A). Top therapeutic interventions include loperamide, dasatinib anhydrous, and enasidenib.

At a glance

• Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
• Causal gene: EXT1 (GenCC Strong)
• Cohort genes: 3
• ClinVar variants: 127
• Clinical trials: 53
• Precision-medicine evidence (CIViC): 1 subtype–drug association

Clinical features

Epidemiology

Prevalence records

3 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.24	Europe	Validated
Lifetime Prevalence	1-9 / 100 000	3.55	Europe	Validated
Point prevalence	1-9 / 100 000		Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	chondrosarcoma
Mondo ID	MONDO:0008977
EFO	EFO:0000333
MeSH	D002813
OMIM	215300
Orphanet	55880
DOID	DOID:3371
NCIT	C2946
SNOMED CT	443520009
UMLS	C0008479
MedGen	3054
GARD	0006055
MedDRA	10008734
Is cancer (heuristic)	no

Also known as: chondrosarcoma · chondrosarcoma (disease) · chondrosarcoma of bone · chondrosarcoma, malignant · chondrosarcoma, somatic mutation

Data availability: 127 ClinVar variants · 1 GenCC gene-disease record · 1 HPO phenotype · 81 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 › cancer › sarcoma › chondrosarcoma

Related subtypes (21): rectum sarcoma, ectomesenchymoma, spindle cell sarcoma, colon sarcoma, sarcomatosis, dendritic cell sarcoma, orbit sarcoma, sarcoma G1, uterine corpus sarcoma, giant cell tumor of soft tissue, lymphangiosarcoma, endometrioid stromal sarcoma, myeloid sarcoma, small cell sarcoma, osteosarcoma, reticulum cell sarcoma, Ewing sarcoma, sarcoma of cervix uteri, soft tissue sarcoma, mast cell sarcoma, bone sarcoma

Subtypes (4): bone chondrosarcoma, myxoid chondrosarcoma, localized chondrosarcoma, mesenchymal chondrosarcoma

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

127 retrieved; paginated sample, class counts are floors:

95 uncertain significance, 12 pathogenic, 11 conflicting classifications of pathogenicity, 5 likely pathogenic, 3 pathogenic/likely pathogenic, 1 likely benign

ClinVar	Variant (HGVS)	Gene	Classification	Review
1075642	NM_000127.3(EXT1):c.752del (p.Phe250_Leu251insTer)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
2036900	NM_000127.3(EXT1):c.356_357del (p.Tyr119fs)	EXT1	Pathogenic/Likely pathogenic	criteria provided, multiple submitters, no conflicts
2495	NM_000127.3(EXT1):c.1019G>T (p.Arg340Leu)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
2497	NM_000127.3(EXT1):c.528_535del (p.Lys177fs)	EXT1	Pathogenic	no assertion criteria provided
2500	NM_000127.3(EXT1):c.1018C>T (p.Arg340Cys)	EXT1	Pathogenic/Likely pathogenic	criteria provided, multiple submitters, no conflicts
2503	NM_000127.3(EXT1):c.962+3_962+6del	EXT1	Pathogenic/Likely pathogenic	criteria provided, multiple submitters, no conflicts
265130	NM_000127.3(EXT1):c.1468del (p.Leu490fs)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
265131	NM_000127.3(EXT1):c.1469del (p.Leu490fs)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
265339	NM_000127.3(EXT1):c.538_539del (p.Leu181fs)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
279804	NM_000127.3(EXT1):c.1468dup (p.Leu490fs)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
4531242	NM_000127.3(EXT1):c.599G>A (p.Trp200Ter)	EXT1	Pathogenic	criteria provided, single submitter
456062	NM_000127.3(EXT1):c.1431dup (p.Ser478fs)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
837149	NM_000127.3(EXT1):c.4C>T (p.Gln2Ter)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
931284	NM_000127.3(EXT1):c.2077del (p.Ala693fs)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
932048	NM_000127.3(EXT1):c.1551G>A (p.Trp517Ter)	EXT1	Pathogenic	criteria provided, multiple submitters, no conflicts
2431522	NM_000127.3(EXT1):c.1836del (p.Lys611_Trp612insTer)	EXT1	Likely pathogenic	criteria provided, single submitter
2675179	NM_000127.3(EXT1):c.2055+1G>C	EXT1	Likely pathogenic	criteria provided, single submitter
2675190	NM_000127.3(EXT1):c.2055+2dup	EXT1	Likely pathogenic	criteria provided, single submitter
3241451	NM_000127.3(EXT1):c.650_653delinsTTTG (p.Ala217_Lys218delinsValTer)	EXT1	Likely pathogenic	criteria provided, single submitter
3767120	NM_000127.3(EXT1):c.1031C>T (p.Ser344Phe)	EXT1	Likely pathogenic	criteria provided, single submitter
134197	NM_000127.3(EXT1):c.122G>A (p.Ser41Asn)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
134199	NM_000127.3(EXT1):c.1135G>A (p.Val379Ile)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
134201	NM_000127.3(EXT1):c.1430C>G (p.Pro477Arg)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
1522496	NM_000127.3(EXT1):c.974G>A (p.Arg325Gln)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
1623931	NM_000127.3(EXT1):c.124G>A (p.Gly42Ser)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
2143012	NM_000127.3(EXT1):c.1486C>T (p.Pro496Ser)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
2169615	NM_000127.3(EXT1):c.635G>C (p.Gly212Ala)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
2445271	NM_000127.3(EXT1):c.106C>T (p.Arg36Trp)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
2445286	NM_000127.3(EXT1):c.1196A>T (p.Asp399Val)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications
2675162	NM_000127.3(EXT1):c.977A>G (p.Glu326Gly)	EXT1	Conflicting classifications of pathogenicity	criteria provided, conflicting classifications

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 6 · 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
EXT1	Strong	Autosomal dominant	chondrosarcoma	6

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
EXT1	Orphanet:321	Multiple osteochondromas
EXT1	Orphanet:502	Trichorhinophalangeal syndrome type 2
EXT1	Orphanet:55880	Chondrosarcoma
IDH1	Orphanet:163634	Maffucci syndrome
IDH1	Orphanet:251576	Gliosarcoma
IDH1	Orphanet:251579	Giant cell glioblastoma
IDH1	Orphanet:296	Ollier disease
IDH1	Orphanet:86845	Acute myeloid leukaemia with myelodysplasia-related features
IDH1	Orphanet:99646	Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria
IDH2	Orphanet:163634	Maffucci syndrome
IDH2	Orphanet:251589	Anaplastic astrocytoma
IDH2	Orphanet:251598	Protoplasmic astrocytoma
IDH2	Orphanet:251601	Fibrillary astrocytoma
IDH2	Orphanet:251604	Gemistocytic astrocytoma
IDH2	Orphanet:251627	Oligodendroglioma
IDH2	Orphanet:251630	Anaplastic oligodendroglioma
IDH2	Orphanet:251656	Oligoastrocytoma
IDH2	Orphanet:251663	Anaplastic oligoastrocytoma
IDH2	Orphanet:296	Ollier disease
IDH2	Orphanet:79315	D-2-hydroxyglutaric aciduria
IDH2	Orphanet:86845	Acute myeloid leukaemia with myelodysplasia-related features

Cohort genes → proteins

3 cohort genes, 3 distinct canonical proteins.

Evidence partition

Subset	Genes
civic_only	2
multi_evidence	1

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
EXT1	HGNC:3512	ENSG00000182197	Q16394	Exostosin-1	gencc,clinvar
IDH1	HGNC:5382	ENSG00000138413	O75874	Isocitrate dehydrogenase [NADP] cytoplasmic	civic_evidence
IDH2	HGNC:5383	ENSG00000182054	P48735	Isocitrate dehydrogenase [NADP], mitochondrial	civic_evidence

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
EXT1	Exostosin-1	Glycosyltransferase forming with EXT2 the heterodimeric heparan sulfate polymerase which catalyzes the elongation of the heparan sulfate glycan backbone.
IDH1	Isocitrate dehydrogenase [NADP] cytoplasmic	Catalyzes the NADP(+)-dependent oxidative decarboxylation of isocitrate (D-threo-isocitrate) to 2-ketoglutarate (2-oxoglutarate), which is required by other enzymes such as the phytanoyl-CoA dioxygenase.
IDH2	Isocitrate dehydrogenase [NADP], mitochondrial	Plays a role in intermediary metabolism and energy production.

Protein-family classification

Druggable: 3 · Difficult: 0 · Unknown: 0 · Druggable fraction: 1.0

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
Enzyme (other)	3	12.0×	6e-04

Per-gene assignment

Symbol	Family	Druggable?	EC	InterPro (top 3)
EXT1	Enzyme (other)	yes	2.4.1.224	Exostosin, GT64_dom, Nucleotide-diphossugar_trans
IDH1	Enzyme (other)	yes	1.1.1.42	Isocitrate_DH_NADP, IsoCit/isopropylmalate_DH_CS, IsoPropMal-DH-like_dom
IDH2	Enzyme (other)	yes	1.1.1.42	Isocitrate_DH_NADP, IsoCit/isopropylmalate_DH_CS, IsoPropMal-DH-like_dom

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)	3
unknown	0

Top tissues across cohort

Tissue	Cohort genes
descending thoracic aorta	1
saphenous vein	1
stromal cell of endometrium	1
adrenal tissue	1
corpus epididymis	1
jejunal mucosa	1
apex of heart	1
gastrocnemius	1
hindlimb stylopod muscle	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
EXT1	285	ubiquitous	marker	stromal cell of endometrium, saphenous vein, descending thoracic aorta
IDH1	294	ubiquitous	marker	corpus epididymis, jejunal mucosa, adrenal tissue
IDH2	292	ubiquitous	marker	apex of heart, gastrocnemius, hindlimb stylopod muscle

Protein interactions among cohort

Intra-cohort edges: 1.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
IDH1	5,464
IDH2	4,912
EXT1	1,449

Intra-cohort edges

A	B	Sources
IDH1	IDH2	biogrid_interaction

Structural data

PDB: 3 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
IDH1	O75874	61
IDH2	P48735	11
EXT1	Q16394	6

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 12. Enrichment computed across 3 evidence-associated genes (3 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 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate	1	3806.7×	0.003	IDH1
NADPH regeneration	1	1903.3×	0.003	IDH1
NFE2L2 regulating TCA cycle genes	1	1268.9×	0.003	IDH1
Defective EXT2 causes exostoses 2	1	271.9×	0.009	EXT1
Defective EXT1 causes exostoses 1, TRPS2 and CHDS	1	271.9×	0.009	EXT1
Maturation of TCA enzymes and regulation of TCA cycle	1	190.3×	0.010	IDH2
Citric acid cycle (TCA cycle)	1	141.0×	0.012	IDH2
HS-GAG biosynthesis	1	115.3×	0.013	EXT1
Transcriptional activation of mitochondrial biogenesis	1	68.0×	0.020	IDH2
Peroxisomal protein import	1	57.7×	0.021	IDH1
Mitochondrial protein degradation	1	38.1×	0.028	IDH2
Neutrophil degranulation	1	7.7×	0.124	IDH1

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
glyoxylate cycle	2	5617.3×	2e-06	IDH1, IDH2
isocitrate metabolic process	2	2246.9×	1e-05	IDH1, IDH2
NADP+ metabolic process	2	1021.3×	4e-05	IDH1, IDH2
2-oxoglutarate metabolic process	2	624.1×	8e-05	IDH1, IDH2
tricarboxylic acid cycle	2	340.4×	2e-04	IDH1, IDH2
hypersensitivity	1	5617.3×	0.001	EXT1
heart field specification	1	5617.3×	0.001	EXT1
lymphocyte adhesion to endothelial cell of high endothelial venule	1	5617.3×	0.001	EXT1
smoothened signaling pathway involved in lung development	1	5617.3×	0.001	EXT1
regulation of phospholipid catabolic process	1	5617.3×	0.001	IDH1
sweat gland development	1	5617.3×	0.001	EXT1
perichondral bone morphogenesis	1	5617.3×	0.001	EXT1
stomach development	1	2808.7×	0.002	EXT1
regulation of phospholipid biosynthetic process	1	2808.7×	0.002	IDH1
mesenchymal cell differentiation involved in bone development	1	2808.7×	0.002	EXT1
negative regulation of glial cell migration	1	2808.7×	0.002	IDH2
negative regulation of matrix metallopeptidase secretion	1	2808.7×	0.002	IDH2
response to leukemia inhibitory factor	1	2808.7×	0.002	EXT1
fluid transport	1	1872.4×	0.002	EXT1
developmental growth involved in morphogenesis	1	1872.4×	0.002	EXT1
response to heparin	1	1872.4×	0.002	EXT1
embryonic skeletal limb joint morphogenesis	1	1404.3×	0.003	EXT1
chondroitin sulfate proteoglycan metabolic process	1	1404.3×	0.003	EXT1
bone trabecula morphogenesis	1	1404.3×	0.003	EXT1
chondrocyte hypertrophy	1	1123.5×	0.003	EXT1
NADPH regeneration	1	1123.5×	0.003	IDH1
hematopoietic stem cell migration to bone marrow	1	1123.5×	0.003	EXT1
tight junction organization	1	1123.5×	0.003	EXT1
chondrocyte differentiation involved in endochondral bone morphogenesis	1	936.2×	0.003	EXT1
fear response	1	936.2×	0.003	EXT1

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): Everolimus, Ivosidenib, Patidegib, Pazopanib.

Drug target analysis

Approved (phase 4): 2 · Phase ≥3: 2 · Phased (≥1): 2 · Undrugged: 1

Druggability breadth: 2 of 3 evidence-associated genes (67%) 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
IDH1	ENASIDENIB
IDH2	ENASIDENIB

Top cohort targets by molecule count

Symbol	Molecules	Max phase
IDH1	10	4
IDH2	7	4
EXT1	0	0

Drugs targeting cohort genes (top 30)

Molecule	Max phase	Targets in cohort
ENASIDENIB	4	IDH1, IDH2
IVOSIDENIB	4	IDH1, IDH2
VORASIDENIB	4	IDH1, IDH2
OLUTASIDENIB	4	IDH1, IDH2
ENASIDENIB MESYLATE	4	IDH2
ZUCLOMIPHENE	2	IDH1
IDH305	2	IDH1
DS-1001B	2	IDH1
SAFUSIDENIB	2	IDH1
CRELOSIDENIB	2	IDH1, IDH2
RANOSIDENIB	2	IDH2
BAY1436032	1	IDH1

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 3.

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

Symbol	Assays	Type breakdown
IDH1	488	Binding:475, Functional:12, ADMET:1
IDH2	84	Binding:84

Cohort enzymes (BRENDA EC)

Symbol	EC numbers	Names
EXT1	2.4.1.224, 2.4.1.225	glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase, N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase
IDH1	1.1.1.42	isocitrate dehydrogenase (NADP+)
IDH2	1.1.1.42	isocitrate dehydrogenase (NADP+)

Cohort genes with high screening signal

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

Symbol	ChEMBL assays
IDH1	488

Pharmacogenomics

Cohort genes with a PharmGKB record: 3; with CPIC/DPWG dosing guidelines: 0.

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

Chemical tractability of cohort targets

9 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)
VORASIDENIB	4	IDH1, IDH2
OLUTASIDENIB	4	IDH1, IDH2
ENASIDENIB MESYLATE	4	IDH2
ZUCLOMIPHENE	2	IDH1
IDH305	2	IDH1
DS-1001B	2	IDH1
SAFUSIDENIB	2	IDH1
RANOSIDENIB	2	IDH2
BAY1436032	1	IDH1

Druggability pyramid

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

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	2	IDH1, IDH2
B	Phased (≥1) drug, not yet approved	0
C	Druggable family + PDB, no drug	1	EXT1
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	0

Undrugged target profiles

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

Symbol	ChEMBL assays	Drugged partners (top 3)
EXT1	0	—

Clinical trials & evidence

Clinical trials

Clinical trials: 53.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	20
PHASE2	19
PHASE1	7
PHASE1/PHASE2	5
PHASE3	1
EARLY_PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01182753	PHASE3	UNKNOWN	Trial of Proton Versus Carbon Ion Radiation Therapy in Patients With Low and Inter-mediate Grade Chondrosarcoma of the Skull Base
NCT00496522	PHASE2	ACTIVE_NOT_RECRUITING	Proton Beam Therapy for Chondrosarcoma
NCT01267955	PHASE2	ACTIVE_NOT_RECRUITING	Vismodegib in Treating Patients With Advanced Chondrosarcomas
NCT02389244	PHASE2	ACTIVE_NOT_RECRUITING	A Phase II Study Evaluating Efficacy and Safety of Regorafenib in Patients With Metastatic Bone Sarcomas
NCT04040205	PHASE2	RECRUITING	Abemaciclib for Bone and Soft Tissue Sarcoma With Cyclin-Dependent Kinase (CDK) Pathway Alteration
NCT04278781	PHASE2	ACTIVE_NOT_RECRUITING	AG-120 in People With IDH1 Mutant Chondrosarcoma
NCT04673942	PHASE2	RECRUITING	A Study of AdAPT-001 in Subjects With Sarcoma and Refractory Solid Tumors
NCT06176989	PHASE2	RECRUITING	Enasidenib in IDH2-Mutated Malignant Sinonasal and Skull Base Tumors
NCT00003292	PHASE2	TERMINATED	S9624 Ifosfamide in Treating Patients With Meningeal Tumors
NCT00401388	PHASE2	COMPLETED	A Trial of Perifosine in Patients With Chemo-Insensitive Sarcomas
NCT00464620	PHASE2	COMPLETED	Trial of Dasatinib in Advanced Sarcomas
NCT00543712	PHASE2	TERMINATED	A Study of PRO95780 in Patients With Advanced Chondrosarcoma (APM4171g)
NCT00592748	PHASE1/PHASE2	COMPLETED	Charged Particle RT for Chordomas and Chondrosarcomas of the Base of Skull or Cervical Spine
NCT00928525	PHASE2	COMPLETED	Imatinib in Patients With Desmoid Tumor and Chondrosarcoma
NCT01154452	PHASE1/PHASE2	COMPLETED	Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma
NCT01330966	PHASE2	COMPLETED	Study of Pazopanib in the Treatment of Surgically Unresectable or Metastatic Chondrosarcoma
NCT01336803	PHASE2	COMPLETED	Differentiation of Bone Sarcomas and Osteomyelitis With Ferumoxytol-Enhanced MRI
NCT01553539	PHASE2	COMPLETED	Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery
NCT01560260	PHASE2	COMPLETED	Linsitinib in Treating Patients With Gastrointestinal Stromal Tumors
NCT02008019	PHASE2	SUSPENDED	A Phase II Study of EVEROLIMUS in Patients With Primary or Relapsed Chondrosarcomas
NCT02273739	PHASE1/PHASE2	COMPLETED	Study of Orally Administered Enasidenib (AG-221) in Adults With Advanced Solid Tumors, Including Glioma, or Angioimmunoblastic T-cell Lymphoma, With an IDH2 Mutation
NCT02496741	PHASE1/PHASE2	COMPLETED	Metformin And Chloroquine in IDH1/2-mutated Solid Tumors
NCT02587169	PHASE1/PHASE2	UNKNOWN	Trial of Nilotinib and Adriamycin as Treatment in Liposarcomas and Leiomyosarcomas of Retroperitoneum
NCT02982486	PHASE2	UNKNOWN	A Phase II of Nivolumab Plus Ipilimumab in Non-resectable Sarcoma and Endometrial Carcinoma
NCT05193188	PHASE2	UNKNOWN	A Study of Anlotinib Combined With or Without PD-1 Antibody on Unresectable High-grade Chondrosarcoma
NCT03173976	PHASE1	ACTIVE_NOT_RECRUITING	Anti-Osteoclast Therapy as Neoadjuvant in Treatment of Chondrosarcoma - Phase 1b Trial
NCT04521686	PHASE1	ACTIVE_NOT_RECRUITING	Study of LY3410738 Administered to Patients With Advanced Solid Tumors With IDH1 or IDH2 Mutations
NCT05039801	PHASE1	RECRUITING	IACS-6274 With or Without Bevacizumab and Paclitaxel for the Treatment of Advanced Solid Tumors
NCT00004241	PHASE1	COMPLETED	17-N-Allylamino-17-Demethoxygeldanamycin in Treating Patients With Advanced Epithelial Cancer, Malignant Lymphoma, or Sarcoma
NCT02073994	PHASE1	COMPLETED	Study of Orally Administered AG-120 in Subjects With Advanced Solid Tumors, Including Glioma, With an IDH1 Mutation
NCT03670069	PHASE1	TERMINATED	Itacitinib in Treating Patients With Refractory Metastatic/Advanced Sarcomas
NCT04553692	PHASE1	TERMINATED	Phase 1a/1b Study of Aplitabart (IGM-8444) Alone or in Combination in Participants with Relapsed, Refractory, or Newly Diagnosed Cancers
NCT06645808	EARLY_PHASE1	RECRUITING	PET-imaging of Two Vartumabs in Patients With Solid Tumors
NCT03442465	Not specified	RECRUITING	Assessment of Healing and Function After Reconstruction Surgery for Bone Sarcomas
NCT04055220	Not specified	RECRUITING	Efficacy and Safety of Regorafenib as Maintenance Therapy After First-line Treatment in Patients With Bone Sarcomas
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
NCT05779670	Not specified	RECRUITING	Adherence to a Personalized Home Exercise Program in Patients With Bone Tumor Undergoing Lower Extremity Salvage Surgery

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
LOPERAMIDE	4	3
DASATINIB ANHYDROUS	4	2
ENASIDENIB	4	2
IVOSIDENIB	4	2
VISMODEGIB	4	2
ABEMACICLIB	4	1
ALLOPURINOL	4	1
CAPIVASERTIB	4	1
FERUMOXYTOL	4	1
IFOSFAMIDE	4	1
PAZOPANIB	4	1
REGORAFENIB	4	1
TAZEMETOSTAT	4	1
CATEQUENTINIB HYDROCHLORIDE	3	1
ITACITINIB	3	1
LINSITINIB	3	1
PATIDEGIB	3	1
PERIFOSINE	3	1
TANESPIMYCIN	3	1
APLITABART	2	1
APOMAB	2	1
BIRINAPANT	2	1
CRELOSIDENIB	2	1
TALFIRASTIDE	2	1
CHEMBL4583196	0	1
CHEMBL4068768	0	1
CHEMBL4171277	0	1
CHEMBL5190225	0	1
CHEMBL5572229	0	1
CHEMBL4786163	0	1

Precision-medicine subtype map (CIViC)

Drug × molecular subtype: 1 predictive associations from 1 curated evidence items; also 2 prognostic, 1 oncogenic, 1 diagnostic.

Molecular subtype	Therapy	Effect	Level	CIViC
IDH1 R132	Ivosidenib	Sensitivity/Response	CIViC A	EID11194