Malignant triton tumor

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Malignant triton tumor

ORPHA:252212Histopathological subtype

Also called MPNST with rhabdomyosarcomatous differentiation · MTT · Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation

What it is

Malignant triton tumor (MTT) is a rare aggressive subtype of malignant peripheral nerve sheath tumor (MPNST) characterized histopathologically by focal rhabdomyoblastic differentiation.

Key facts

Age of onset
All ages
Classified as
Histopathological subtype

Recorded for the broader condition

Prevalence
1-9 / 100 000 (annual incidence)Malignant peripheral nerve sheath tumor

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Genes

HTRA1Part of a fusion gene
SH3PXD2APart of a fusion gene

ICD-10 codes

C47.9filed under a broader ICD-10 category — shared with 2 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0016757UMLS C0334616

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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