Juvenile nasopharyngeal angiofibroma
Synonyms: JNA
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Childhood
- Status
- active
Juvenile nasopharyngeal angiofibroma (JNA) is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the nasopharynx, which affects mainly young and adolescent males (onset usually occurring between 7-19 years of age) and that presents as a mass in the nasopharynx and nasal cavity, leading to manifestations such as nasal obstruction, epistaxis, profound facial swelling, proptosis or diplopia. Although slowly progressive, it has a high rate of recurrence and sometimes invades adjacent structures.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Juvenile nasopharyngeal angiofibroma | JNA |
| fr | Angiofibrome nasopharyngé juvénile | = en |
| de | Nasopharyngeales Angiofibrom, juveniles | = en |
| es | Angiofibroma nasofaríngeo juvenil | = en |
| it | Angiofibroma nasofaringeo giovanile | = en |
| nl | Juveniel nasofaryngeaal angiofibroom | = en |
| pt | Angiofibroma nasofaríngeo juvenil | = en |
| pl | Naczyniakowłókniak młodzieńczy nosogardła | = en |
| cs | Juvenilní nasofaryngeální angiofibrom | = en |
| uk | Ювенільна ангіофіброма носоглотки | = en |
| zh | 青少年鼻咽血管纤维瘤 | 青少年鼻咽血管纤维瘤 |
| tr | Jüvenil nazofarengeal anjiyofibrom | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D10.6 | NTBT | index | validated |
| ICD-11 | 2E90.6 | NTBT | index | validated |
| MONDO | 0017340 | E | validated | |
| UMLS | C1367536 | 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:98061Rare otorhinolaryngologic tumor
rare otorhinolaryngological 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 |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.6666 / 100 000 | Worldwide | Value and class | Validated | 28199045[PMID] |
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
8 annotations · validated 2019-07-24| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0030429 | Juvenile nasopharyngeal angiofibroma | Diagnostic criterion |
| Frequent (79-30%) | ||
| HP:0000651 | Diplopia | |
| HP:0000421 | Epistaxis | |
| HP:0000282 | Facial edema | |
| HP:0001742 | Nasal congestion | |
| HP:0000520 | Proptosis | |
| Occasional (29-5%) | ||
| HP:0002516 | Increased intracranial pressure | |
| Very rare (<4-1%) | ||
| HP:0012198 | Juvenile colonic polyposis | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 30345941[PMID]_29752322[PMID]_4284864[PMID]_15951557[PMID]_19626637[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/289596
- This record as JSON: /data/d/289596.json