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ORPHA:252212

Malignant triton tumor

medium confidenceSubtype of disorder

Also known as: MPNST with rhabdomyosarcomatous differentiation · MTT · Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation

Publications

462

80.4th 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.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

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.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    462 matched papers (223 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. 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)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

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=5449) is 41.

462 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

223 in the last 10 years · medium confidence · 80.4th percentile (publications denominator)

Phrase hits: 462 · MeSH hits: 0

Open Europe PMC search

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.

  1. 01
    Antonescu CR6 papers · 2024

    Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York.

    Papers in Europe PMC
  2. 02
    Wang 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
  3. 03
    Wexler LH4 papers · 2024

    Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York, USA.

    Papers in Europe PMC
  4. 04
    Amr SS3 papers · 2022

    Department of Pathology and Laboratory Medicine, Istishari Hospital, Amman 11184, Jordan.

    Papers in Europe PMC
  5. 05
    Dermawan JK3 papers · 2024

    Department of Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH.

    Papers in Europe PMC
  6. 06
    Kohashi K3 papers · 2026

    Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

    Papers in Europe PMC
  7. 07
    liu 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
  8. 08
    Liu 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
  9. 09
    Wang 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
  10. 10
    xu 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 27 July 2026

2 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 82.4th percentile).

medium confidence · 82.4th 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.

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

India — NPRD

Last verified 2026-07-26

This 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"

Run this search on Europe PMC

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