Congenital epulis
Synonyms: Congenital gingival cell tumor · Congenital granular cell tumor · Neumann tumor
- Type
- Disease
- Group
- Disorder
- Age of onset
- Infancy, Neonatal
- Status
- active
A rare soft tissue tumor characterized by a benign space occupying lesion in neonates, most typically located on the gingival mucosa overlying the anterior alveolar ridge of the maxilla near the canine, although the mandibular region may also be involved. Females are much more frequently affected than males. The tumor mostly presents as a single lesion, potentially interfering with feeding and respiration. Metastasis, malignant transformation, or recurrence after excision have not been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital epulis | Congenital gingival cell tumor · Congenital granular cell tumor · Neumann tumor |
| fr | Epulis congénital | Tumeur de Neumann · Tumeur gingivale à cellules granuleuses congénitale |
| de | Epulis, kongenitale | Granularzelltumor, kongenitaler |
| es | Epulis congénito | Tumor congénito de células gingivales · Tumor congénito de células granulares · Tumor de Neumann |
| it | Epulide congenita | Tumore congenito delle cellule gengivali · Tumore congenito delle cellule granulari |
| nl | Congenitale epulis | Congenitale gingivale celtumor · Congenitale granulaire celtumor · Tumor van Neumann |
| pt | Epulis, congénita | |
| pl | Wrodzony nadziąślak | Wrodzony guz z komórek ziarnistych · Wrodzony ziarniniak dziąsłowy |
| cs | Kongenitální epulis | Vrozený nádor dásní · Vrozený nádor z granulárních buněk |
| uk | Вроджений епуліс | Вроджена клітинна пухлина ясен · Вроджена зерноклітинна пухлина · Пухлина Неймана |
| zh | 先天性牙龈瘤 | 先天性颗粒细胞瘤 |
| tr | Doğumsal epulis | Neumann tümörü · Doğumsal gingival hücreli tümörü · Doğumsal granüler hücreli tümör |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K06.8 | NTBT | attributed | validated |
| ICD-11 | KC23 | NTBT | index | validated |
| MONDO | 0015528 | E | validated | |
| UMLS | C0376319 | 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:71209Rare soft tissue tumor
rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 1 000 000 | — | Europe | Class only | Not yet 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/157826
- This record as JSON: /data/d/157826.json