Esthesioneuroblastoma
Synonyms: Olfactory neuroblastoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
Esthesioneuroblastoma (ENB) is a rare malignant neoplasm of the sinonasal cavity, arising from the basal layers of olfactory neuroepithelial cells in the superior nasal vault, which usually occurs in the 5th to 6th decades of life and is characterized clinically by non-specific symptoms such as progressive ipsilateral nasal block, sinusitis, facial pain, intermittent headaches, hyposmia/dysosmia, rhinorrhea and epistaxis as well as proptosis, diplopia and excessive lacrimation due to orbital extension. With early treatment and in the absence of distant metastases, ENB appears to have a good prognosis (compared to other superior nasal malignancies), despite a high rate of cervical metastases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Esthesioneuroblastoma | Olfactory neuroblastoma |
| fr | Esthésioneuroblastome | Neuroblastome olfactif |
| de | Ästhesioneuroblastom | Olfaktorisches Neuroblastom |
| es | Estesioneuroblastoma | Neuroblastoma olfativo |
| it | Estesioneuroblastoma | Neuroblastoma olfattivo |
| nl | Esthesioneuroblastoom | Olfactorisch neuroblastoom |
| pt | Estesioneuroblastoma | |
| pl | Nerwiak węchowy zarodkowy | Nerwiak węchowy zarodkowy |
| cs | Estezioneuroblastom | Olfaktoriální neuroblastom |
| uk | Естезионейробластома | Олфакторна нейробластома |
| zh | 鼻腔神经胶质瘤 | 嗅神经母细胞瘤 |
| tr | Estezionöroblastom | Olfaktör nöroblastoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C30.0 | NTBT | index | validated |
| ICD-11 | 2C20.3 | E | specific | validated |
| ICD-11 | XH50L1 | E | specific | validated |
| MONDO | 0016029 | E | validated | |
| MeSH | D018304 | E | validated | |
| UMLS | C0206717 | E | validated | |
| GARD | 2197 | 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:251870Central nervous system embryonal tumor
rare neurological 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 |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.02 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | <1 / 1 000 000 | — | Europe | 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/1957
- This record as JSON: /data/d/1957.json