ORPHA:252212

Malignant triton tumor

Synonyms: MPNST with rhabdomyosarcomatous differentiation · MTT · Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation

Type: Histopathological subtype
Group: Subtype of disorder
Age of onset: All ages
Status: active

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

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Malignant triton tumor	MPNST with rhabdomyosarcomatous differentiation · MTT · Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation
fr	Tumeur triton maligne	MPNST avec différenciation rhabdomyosarcomateuse · TTM · Tumeur maligne des gaines nerveuses avec différenciation rhabdomyosarcomateuse
de	Triton-Tumor, maligner	MPNST mit rhabdomyosarkomatöser Differenzierung · MTT · Nervenscheidentumor, maligner peripherer, mit rhabdomyosarkomatöser Differenzierung
es	Tumor tritón maligno	MPNST con diferenciación rabdomiosarcomatosa · MTT · Tumor maligno de vaina nerviosa periférica con diferenciación rabdomiosarcomatosa
it	Tumore maligno del tritone	MPNST con differenziazione rabdomiosarcomatosa · MTT · Tumore maligno delle guaine nervose con differenziazione rabdomiosarcomatosa
nl	Maligne tritontumor	MTT · Maligne tumor van perifere zenuwschede met rhabdomyosarcomateuze differentiatie · Kwaadaardige tritontumor · MPNST met rhabdomyosarcomateuze differentiatie
pt	Tumor tritão maligno	MPNST com diferenciação rabdomiossarcomatosa · MTT · Tumor maligno da bainha dos nervos periféricos com diferenciação rabdomiossarcomatosa
pl	Złośliwy guz Trytona	MPNST z różnicowaniem w kierunku mięśniakomięsaka prążkowanokomórkowego · MTT · Złośliwy nowotwór osłonek nerwowych z różnicowaniem w kierunku mięśniakomięsaka prążkowanokomórkowego
cs	Maligní Tritonův tumor	MPNST s rhabdomyosarkomatózní diferenciací · MTT · Zhoubný poševní nádor periferních nervů s rhabdomyosarkomatózní diferenciací
uk	Злоякісна пухлина тритона	Злоякісна пухлина оболонки периферичного нерва з рабдоміосаркоматозним диференціюванням · МПНСТ з рабдоміосаркоматозною диференціацією · MTT
zh	恶性蝾螈瘤	恶性神经鞘瘤伴横纹肌肉瘤分化
tr	Habis triton tümörü	MTT · Rabdomyosarkomatöz farklılaşma ile seyreden MPNST · Rabdomyosarkomatöz farklılaşma ile seyreden habis periferik sinir kılıfı tümörü

Definition in:

Cross-references

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
ICD-10	C47.9	NTBT	attributed	validated
ICD-11	XH2VV8	NTBT	index	validated
MONDO	0016757	E		validated
UMLS	C0334616	E		validated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

Classification

Preferential parent (Orphanet linearisation):

ORPHA:250908Rare neoplastic disease

Parents in the classifications (1):

ORPHA:3148Malignant peripheral nerve sheath tumor
rare neurological diseases; rare neoplastic diseases

No children.

Genes

2 direct

Gene	Association	Status	HGNC	Ensembl	OMIM	Locus	Name	Source of validation
HTRA1	Part of a fusion gene in	Assessed	HGNC:9476	ENSG00000166033	602194	10q26.13	HtrA serine peptidase 1	27723760[PMID]
SH3PXD2A	Part of a fusion gene in	Assessed	HGNC:23664	ENSG00000107957	619455	10q24.33	SH3 and PX domains 2A	27723760[PMID]

Prevalence and incidence

2 records

Type	Class	Value	Area	Qualification	Validation	Source
Cases/families		170 cases	Worldwide	Case(s)	Validated	24474920[PMID]
Point prevalence	Unknown	—	Worldwide	Class only	Validated	ORPHANET_24474920[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

Sources for this card

Orphanet page: orpha.net/en/disease/detail/252212
This record as JSON: /data/d/252212.json