Peripheral primitive neuroectodermal tumor of bone
diseaseOn this page
Also known as bone peripheral neuroepitheliomabone tissue peripheral primitive neuroectodermal tumorbone tissue peripheral primitive neuroectodermal tumourosseous peripheral neuroepitheliomaperipheral neuroectodermal tumor of boneperipheral neuroectodermal tumor of the boneperipheral neuroectodermal tumour of boneperipheral neuroectodermal tumour of the boneperipheral neuroepithelioma of boneperipheral neuroepithelioma of the boneperipheral primitive neuroectodermal tumor of bone tissueperipheral primitive neuroectodermal tumour of bone tissue
Summary
Peripheral primitive neuroectodermal tumor of bone (MONDO:0002981) is a cancer and 3 clinical trials. Top therapeutic interventions include dexrazoxane, ifosfamide, and vincristine. A subtype of bone cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Clinical trials: 3
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | peripheral primitive neuroectodermal tumor of bone |
| Mondo ID | MONDO:0002981 |
| DOID | DOID:4388 |
| NCIT | C8776 |
| UMLS | C0855009 |
| MedGen | 163179 |
| GARD | 0023312 |
| Anatomy (UBERON) | UBERON:0002481 |
| Is cancer (heuristic) | yes |
Also known as: bone peripheral neuroepithelioma · bone tissue peripheral primitive neuroectodermal tumor · bone tissue peripheral primitive neuroectodermal tumour · osseous peripheral neuroepithelioma · peripheral neuroectodermal tumor of bone · peripheral neuroectodermal tumor of the bone · peripheral neuroectodermal tumour of bone · peripheral neuroectodermal tumour of the bone · peripheral neuroepithelioma of bone · peripheral neuroepithelioma of the bone · peripheral primitive neuroectodermal tumor of bone · peripheral primitive neuroectodermal tumor of bone tissue · peripheral primitive neuroectodermal tumour of bone tissue
Data availability: 7 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 › peripheral primitive neuroectodermal tumor of bone
Related subtypes (11): bone chondrosarcoma, skull cancer, bone carcinoma, small cell osteogenic sarcoma, sternum cancer, chest wall bone cancer, adult extraskeletal osteosarcoma, 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: 3.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE3 | 2 |
| Not specified | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT02306161 | PHASE3 | ACTIVE_NOT_RECRUITING | Combination Chemotherapy With or Without Ganitumab in Treating Patients With Newly Diagnosed Metastatic Ewing Sarcoma |
| NCT01231906 | PHASE3 | COMPLETED | Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma |
| NCT06068075 | Not specified | ACTIVE_NOT_RECRUITING | Liquid Biopsy in Ewing Sarcoma and Osteosarcoma as a Prognostic And Response Diagnostic: LEOPARD |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| DEXRAZOXANE | 4 | 3 |
| IFOSFAMIDE | 4 | 2 |
| VINCRISTINE | 4 | 2 |
| ETOPOSIDE PHOSPHATE | 4 | 1 |
| TOPOTECAN HYDROCHLORIDE | 4 | 1 |
| GANITUMAB | 3 | 1 |