Adult extraskeletal osteosarcoma

Summary

Adult extraskeletal osteosarcoma (MONDO:0004374) is a disease and 7 clinical trials. Top therapeutic interventions include pazopanib, ifosfamide, and romidepsin. A subtype of bone cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 7

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	adult extraskeletal osteosarcoma
Mondo ID	MONDO:0004374
DOID	DOID:7827
NCIT	C7925
UMLS	C0278985
MedGen	83016
GARD	0023967
Is cancer (heuristic)	no

Also known as: adult extraosseous osteosarcoma · adult extraskeletal osteosarcoma · extraosseous osteosarcoma of adults · extraskeletal osteosarcoma

Data availability: 2 cell lines.

Disease family

This is a subtype of bone cancer. 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 body system or component › musculoskeletal system disorder › musculoskeletal system cancer › bone cancer › adult extraskeletal osteosarcoma

Related subtypes (11): bone chondrosarcoma, skull cancer, bone carcinoma, small cell osteogenic sarcoma, peripheral primitive neuroectodermal tumor of bone, sternum cancer, chest wall bone cancer, primary bone lymphoma, bone sarcoma, bone marrow cancer, cancer affecting bone of limb skeleton

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: 7.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	2
PHASE3	1
PHASE2/PHASE3	1
PHASE1/PHASE2	1
PHASE1	1
Not specified	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
NCT00346164	PHASE3	COMPLETED	Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma
NCT00112463	PHASE2	COMPLETED	Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma
NCT01154452	PHASE1/PHASE2	COMPLETED	Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma
NCT01532687	PHASE2	COMPLETED	Gemcitabine With or Without Pazopanib in Treating Patients With Refractory Soft Tissue Sarcoma
NCT00720174	PHASE1	COMPLETED	Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma
NCT05515068	Not specified	NOT_YET_RECRUITING	Registry For Children, Adolescents And Adults With Osteosarcoma And Biologically Related Bone Sarcomas

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
PAZOPANIB	4	6
IFOSFAMIDE	4	2
ROMIDEPSIN	4	1
VISMODEGIB	4	1
CIXUTUMUMAB	2	1
CHEMBL4068768	0	2
CHEMBL4171277	0	2
CHEMBL4066465	0	1