Ameloblastic carcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare odontogenic tumor characterized by aggressive clinical course and local destruction, occurring in mandible more often than in maxilla. The most common symptom is a rapidly progressing painful swelling, but it may present as a benign cystic lesion or as a large, rapidly growing mass with ulceration, bone resorption and teeth mobility, as well. The tumor may metastasize, most commonly to the cervical lymph nodes and the lungs.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Ameloblastic carcinoma | |
| fr | Carcinome améloblastique | |
| de | Karzinom, ameloblastisches | |
| es | Carcinoma ameloblástico | |
| it | Carcinoma ameloblastico | |
| nl | Ameloblastisch carcinoom | |
| pt | Carcinoma ameloblástico | |
| pl | Rak ameloblastyczny | |
| cs | Ameloblastický karcinom | |
| uk | Амелобластна карцинома | |
| zh | 成釉细胞癌 | |
| tr | Ameloblastik karsinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C41.1 | NTBT | index | validated |
| ICD-11 | 2B5J | NTBT | index | validated |
| MONDO | 0006079 | E | validated | |
| UMLS | C1314678 | E | validated | |
| GARD | 11855 | E | validated | |
| MedDRA | 10083863 | 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):
Parents in the classifications (1):
- ORPHA:314425Rare odontogenic tumor
rare odontological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 40 cases | Worldwide | Case(s) | Validated | 22493395[PMID]_19674470[PMID]_23251064[PMID]_23099227[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/314422
- This record as JSON: /data/d/314422.json