Malignant triton tumor
disease diseaseOn this page
Also known as malignant neoplasm of peripheral nerve sheath with rhabdomyosarcomamalignant neoplasm of the peripheral nerve sheath with rhabdomyosarcomamalignant peripheral nerve sheath neoplasm with rhabdomyosarcomamalignant peripheral nerve sheath tumor with rhabdomyosarcomamalignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciationmalignant peripheral nerve sheath tumour with rhabdomyosarcomamalignant peripheral nerve sheath tumour with rhabdomyosarcomatous differenciationmalignant tumor of peripheral nerve sheath with rhabdomyosarcomamalignant tumor of the peripheral nerve sheath with rhabdomyosarcomamalignant tumour of peripheral nerve sheath with rhabdomyosarcomamalignant tumour of the peripheral nerve sheath with rhabdomyosarcomaMPNST with rhabdomyosarcomaMPNST with rhabdomyosarcomatous differentiationMTT
Summary
Malignant triton tumor (MONDO:0016757) is a cancer and 2 clinical trials. Top therapeutic interventions include pazopanib and ifosfamide. A subtype of malignant peripheral nerve sheath tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Prevalence: Unknown (Worldwide) [Orphanet-validated]
- Clinical trials: 2
Clinical features
Epidemiology
Prevalence records
1 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Cases/families | 170 | Worldwide | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | malignant triton tumor |
| Mondo ID | MONDO:0016757 |
| Orphanet | 252212 |
| DOID | DOID:6707 |
| NCIT | C4335 |
| SNOMED CT | 404040002 |
| UMLS | C0334616 |
| MedGen | 90816 |
| GARD | 0020743 |
| Is cancer (heuristic) | yes |
Also known as: malignant neoplasm of peripheral nerve sheath with rhabdomyosarcoma · malignant neoplasm of the peripheral nerve sheath with rhabdomyosarcoma · malignant peripheral nerve sheath neoplasm with rhabdomyosarcoma · malignant peripheral nerve sheath tumor with rhabdomyosarcoma · malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation · malignant peripheral nerve sheath tumour with rhabdomyosarcoma · malignant peripheral nerve sheath tumour with rhabdomyosarcomatous differenciation · malignant Triton tumor · malignant tumor of peripheral nerve sheath with rhabdomyosarcoma · malignant tumor of the peripheral nerve sheath with rhabdomyosarcoma · malignant tumour of peripheral nerve sheath with rhabdomyosarcoma · malignant tumour of the peripheral nerve sheath with rhabdomyosarcoma · MPNST with rhabdomyosarcoma · MPNST with rhabdomyosarcomatous differentiation · MTT
Disease family
This is a subtype of malignant peripheral nerve sheath tumor. 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: human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › nervous system cancer › central nervous system cancer › central nervous system sarcoma › malignant peripheral nerve sheath tumor › malignant triton tumor
Related subtypes (6): malignant cardiac peripheral nerve sheath neoplasm, malignant melanocytic neoplasm of the peripheral nerve sheath, childhood malignant schwannoma, epithelioid malignant peripheral nerve sheath tumor, adult malignant schwannoma, malignant glandular tumor of peripheral nerve sheath
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: 2.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2/PHASE3 | 1 |
| PHASE2 | 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 |
| NCT03651375 | PHASE2 | UNKNOWN | Hypofractionated Radiotherapy With Sequential Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| PAZOPANIB | 4 | 3 |
| IFOSFAMIDE | 4 | 1 |
| CHEMBL4068768 | 0 | 1 |
| CHEMBL4171277 | 0 | 1 |
Related Atlas pages
- Drugs: Pazopanib, Ifosfamide