Keratocystic odontogenic tumor
Synonyms: KTOC · Odontogenic keratocystoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare odontogenic tumor characterized by an unilocular or multilocular cyst most commonly located in the posterior body and lower ramus of the mandible, often surrounding the crown of the third molar. Histopathologically, the lesion shows a lining of parakeratinized stratified squamous epithelium with palisading hyperchromatic basal cells. Patients may be asymptomatic or present with local infection and/or signs and symptoms of mass effect. Recurrence is rare after complete surgical removal. Some patients have multiple cysts (metachronous or synchronous), especially in the context of nevoid basal cell carcinoma syndrome (Gorlin syndrome).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Keratocystic odontogenic tumor | KTOC · Odontogenic keratocystoma |
| fr | Tumeur odontogéne kératokystique | KTOC · Kératokyste odontogène |
| de | Keratozystischer odontogener Tumor | KTOC · Keratozyste, odontogene |
| es | Tumor odontogénico queratoquístico | KTOC · Queratocistoma odontogénico · Queratoquiste odontogénico |
| it | Tumore cheratocistico odontogeno | Cheratocisti odontogena · KTOC |
| nl | Keratocystische odontogene tumor | KTOC · Odontogeen keratocystoom |
| pt | Tumor odontogénico queratoquístico | |
| pl | Guz zębopochodny rogowaciejący | Zębopochodny guz keratocystyczny |
| cs | Keratocystický odontogenní tumor | KTOC · Odontogenní keratocysta |
| uk | Кератокістозна одонтогенна пухлина | KTOC · Одонтогенна кератоцистома |
| zh | 角化囊性牙源性肿瘤 | 牙源性角化囊肿 |
| tr | Keratokistik odontojenik tümör | KTOC · Odontojenik keratokistoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D16.4 | NTBT | index | validated |
| ICD-11 | 2E83.1 | NTBT | index | validated |
| ICD-11 | 2E83.Z | NTBT | index | validated |
| MONDO | 0018648 | E | validated | |
| UMLS | C1708604 | 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
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/447777
- This record as JSON: /data/d/447777.json