raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:689430

Adenoid ameloblastoma

Synonyms: AA

Type
Disease
Group
Disorder
Inheritance
Not applicable
Status
active

Orphadata ships no English definition for this code.

1

Names in Orphadata languages

9 of 12 languages
LangNameSynonyms
enAdenoid ameloblastomaAA
frAméloblastome adénoïde= en
deAdenoides Ameloblastom= en
esAmeloblastoma adenoide= en
itAmeloblastoma adenoideo= en
nlAmeloblastoom van adenoïdAA · Ameloblastoom van neusamandel
ptAmeloblastoma adenoide= en
plSzkliwiak migdałka= en
csAdenoidní ameloblastom= en
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D16.4NTBTindexvalidated
ICD-112E83.0NTBTattributedvalidated
ICD-112E83.1NTBTattributedvalidated
MONDO975925Evalidated

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

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

1 direct
GeneAssociationStatusHGNCEnsemblOMIMLocusNameSource of validation
CTNNB1Disease-causing somatic mutation(s) inAssessedHGNC:2514ENSG000001680361168063p22.1catenin beta 135840721[PMID]
5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families30 casesWorldwideCase(s)Validated37540485[PMID]
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidated37540485[PMID]_ORPHANET

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

6

HPO phenotypes

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

7

Sources for this card