ORPHA:252212
Malignant triton tumor
Also known as: MPNST with rhabdomyosarcomatous differentiation · MTT · Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation
Publications
462
69.9th percentile
Trials
2
Interventional, condition-specific
Researchers
1,157
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
Malignant triton tumor (MTT) is a rare aggressive subtype of malignant peripheral nerve sheath tumor (MPNST) characterized histopathologically by focal rhabdomyoblastic differentiation.
How rare: How common this is has not been clearly measured.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0016757
- UMLS:C0334616
- NCIT:C4335
Additional Mondo synonyms (13)
MPNST with rhabdomyosarcoma · malignant Triton tumor · 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 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
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
2/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
462 matched papers (223 in last 10 years) Source
- Phenotype characterisedNot found
No HPO disease–phenotype associations via Monarch for these Mondo IDs
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- Interventional trialPresent
2 matched on ClinicalTrials.gov
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”
Animal models (Monarch / Alliance)
None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.
Monarch fetch 2026-07-29
Therapies
Designations, candidates, and chemicals
FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.
Orphan designation (FDA · EMA)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
No drugs or clinical candidates returned for this Mondo ID on Open Targets.
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
Literature
Is anyone studying this?
462
462 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.
462 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).
223 in the last 10 years · medium confidence · 69.9th percentile (publications denominator)
Phrase hits: 462 · MeSH hits: 0
Who's working on it?
1,157
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Antonescu CR6 papers · 2024
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York.
Papers in Europe PMC - 02Wang J5 papers · 2022
Department of Head and Neck Cancer, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin, 300060, China. 234007811@qq.com.
Papers in Europe PMC - 03Wexler LH4 papers · 2024
Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
Papers in Europe PMC - 04Amr SS3 papers · 2022
Department of Pathology and Laboratory Medicine, Istishari Hospital, Amman 11184, Jordan.
Papers in Europe PMC - 05Dermawan JK3 papers · 2024
Department of Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH.
Papers in Europe PMC - 06Kohashi K3 papers · 2026
Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Papers in Europe PMC - 07liu b3 papers · 2022
Department of Oncology, The Affiliated Drum Tower Clinical Medical College of Nanjing Medical University, Nanjing, Jiangsu 210008, P.R. China ; The Comprehensive Cancer Center of Drum Tower Hospital, Medical School of Nanjing University and Clinical Cancer Institute of Nanjing University, Nanjing, Jiangsu 210008, P.R. China.
Papers in Europe PMC - 08Liu Y3 papers · 2024
Department of Otolaryngology and Head-Neck Surgery, Xiangya Hospital, Central South University, Changsha, Hunan 410008, P.R. China; Otolaryngology Major Disease Research Key Laboratory of Hunan, Xiangya Hospital, Central South University, Changsha, Hunan 410008, P.R. China.
Papers in Europe PMC - 09Wang Y3 papers · 2025
Department of Otolaryngology and Head-Neck Surgery, Xiangya Hospital, Central South University, Changsha, Hunan 410008, P.R. China; Otolaryngology Major Disease Research Key Laboratory of Hunan, Xiangya Hospital, Central South University, Changsha, Hunan 410008, P.R. China.
Papers in Europe PMC - 10xu s3 papers · 2025
Department of Neurosurgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Shandong First Medical University, Jinan, China.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
2
interventional trials for this specific condition
2 interventional trials matched this specific condition name; none in our sample are currently recruiting.
Data as of 11 September 2026
2 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 84.5th percentile).
medium confidence · 84.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
2 interventional trials matched after quoted-phrase search and title/condition post-filter.
No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.
Other registries (secondary)
Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.
raw 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · dropped 0 · fetched 2026-07-30
Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri
No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).
Where to find support
Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.
Orphanet entry for Malignant triton tumor — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Malignant triton tumor" OR "MPNST with rhabdomyosarcomatous differentiation" OR "Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation" OR "MPNST with rhabdomyosarcoma" OR "malignant neoplasm of peripheral nerve sheath with rhabdomyosarcoma" OR "malignant neoplasm of the peripheral nerve sheath with rhabdomyosarcoma" OR "malignant peripheral nerve sheath neoplasm with rhabdomyosarcoma" OR "malignant peripheral nerve sheath tumor with rhabdomyosarcoma" OR "malignant peripheral nerve sheath tumour with rhabdomyosarcoma" OR "malignant peripheral nerve sheath tumour with rhabdomyosarcomatous differenciation" OR "malignant tumor of peripheral nerve sheath with rhabdomyosarcoma" OR "malignant tumor of the peripheral nerve sheath with rhabdomyosarcoma" OR "malignant tumour of peripheral nerve sheath with rhabdomyosarcoma" OR "malignant tumour of the peripheral nerve sheath with rhabdomyosarcoma"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Malignant triton tumor" OR "MPNST with rhabdomyosarcomatous differentiation" OR "Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation" OR "MPNST with rhabdomyosarcoma" OR "malignant neoplasm of peripheral nerve sheath with rhabdomyosarcoma" OR "malignant neoplasm of the peripheral nerve sheath with rhabdomyosarcoma" OR "malignant peripheral nerve sheath neoplasm with rhabdomyosarcoma" OR "malignant peripheral nerve sheath tumor with rhabdomyosarcoma" OR "malignant peripheral nerve sheath tumour with rhabdomyosarcoma" OR "malignant peripheral nerve sheath tumour with rhabdomyosarcomatous differenciation" OR "malignant tumor of peripheral nerve sheath with rhabdomyosarcoma" OR "malignant tumor of the peripheral nerve sheath with rhabdomyosarcoma" OR "malignant tumour of peripheral nerve sheath with rhabdomyosarcoma" OR "malignant tumour of the peripheral nerve sheath with rhabdomyosarcoma"
Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 2 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: MTT
Confidence reasoning
- Preferred label is multi-word and distinctive
- 1 synonym(s) dropped by stoplist (may under-count)
- No label/synonym collisions with other diseases in this corpus
Ingested 2026-07-27T11:01:19.599Z