Pediatric mesenchymal chondrosarcoma

Summary

Pediatric mesenchymal chondrosarcoma (MONDO:0003041) is a disease and 5 clinical trials. Molecularly, HEY1::NCOA2 Fusion confers sensitivity to Trabectedin in Mesenchymal Chondrosarcoma (CIViC Level C); 1 further subtype–drug associations are mapped below. Top therapeutic interventions include pazopanib, ifosfamide, and regorafenib. A subtype of childhood malignant neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 5
• Precision-medicine evidence (CIViC): 2 subtype–drug associations

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	pediatric mesenchymal chondrosarcoma
Mondo ID	MONDO:0003041
DOID	DOID:4546
NCIT	C27374
UMLS	C1332982
MedGen	232063
GARD	0023342
Is cancer (heuristic)	no

Also known as: childhood mesenchymal chondrosarcoma · mesenchymal chondrosarcoma · mesenchymal chondrosarcoma of childhood · pediatric mesenchymal chondrosarcoma

Disease family

This is a subtype of childhood malignant neoplasm. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › childhood malignant neoplasm › pediatric mesenchymal chondrosarcoma

Related subtypes (29): childhood oligodendroglioma, pediatric osteosarcoma, pediatric fibrosarcoma, childhood choroid plexus carcinoma, childhood central nervous system primitive neuroectodermal neoplasm, childhood brain stem neoplasm, pediatric angiosarcoma, pediatric liposarcoma, pediatric lymphoma, childhood malignant mesenchymoma, pediatric myxoid chondrosarcoma, childhood botryoid rhabdomyosarcoma, pediatric intraocular retinoblastoma, childhood cerebral astrocytoma, childhood epithelioid sarcoma, childhood pleomorphic rhabdomyosarcoma, pediatric infratentorial ependymoma, pediatric supratentorial ependymoma, childhood malignant schwannoma, pediatric extraocular retinoblastoma, childhood leukemia, childhood precursor T-lymphoblastic lymphoma/leukemia, malignant childhood germ cell neoplasm, pleuropulmonary blastoma, pediatric hepatocellular carcinoma, childhood malignant kidney neoplasm, childhood malignant melanoma, extrarenal rhabdoid tumor, pediatric high-grade glioma

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

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 5.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	4
PHASE2/PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02180867	PHASE2/PHASE3	ACTIVE_NOT_RECRUITING	Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery
NCT01267955	PHASE2	ACTIVE_NOT_RECRUITING	Vismodegib in Treating Patients With Advanced Chondrosarcomas
NCT04305548	PHASE2	RECRUITING	Study on Trabectedin in Advanced Rearranged Mesenchymal Chondrosarcoma
NCT02048371	PHASE2	COMPLETED	SARC024: A Blanket Protocol to Study Oral Regorafenib in Patients With Selected Sarcoma Subtypes
NCT02821507	PHASE2	COMPLETED	Sirolimus and Cyclophosphamide in Metastatic or Unresectable Myxoid Liposarcoma and Chondrosarcoma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
PAZOPANIB	4	3
IFOSFAMIDE	4	1
REGORAFENIB	4	1
TRABECTEDIN	4	1
VISMODEGIB	4	1
CHEMBL4068768	0	1
CHEMBL4171277	0	1

Precision-medicine subtype map (CIViC)

Drug × molecular subtype: 2 predictive associations from 2 curated evidence items; also 6 diagnostic.

Molecular subtype	Therapy	Effect	Level	CIViC
HEY1::NCOA2 Fusion	Trabectedin	Sensitivity/Response	CIViC C	EID12585
HEY1::NCOA2 Fusion	Imatinib	Sensitivity/Response	CIViC D	EID10798