Squamous cell carcinoma of the nasal cavity and paranasal sinuses
Synonyms: Squamous cell carcinoma of the nasal cavity and sinuses
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare head and neck tumor characterized by a malignant epithelial neoplasm most commonly arising in the maxillary sinus or nasal cavity, occurring as a keratinizing, a non-keratinizing, or a spindle cell (sarcomatoid) type. Patients may present with nasal obstruction, epistaxis, rhinorrhea, swelling, or (at more advances stages) with facial pain and/or paralysis, diplopia, and proptosis. Patients with paranasal sinus tumors present later and at a higher stage than patients with nasal cavity carcinomas. Risk factors are smoking and industrial exposures. High-risk HPV is most frequently associated with the non-keratinizing type.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Squamous cell carcinoma of the nasal cavity and paranasal sinuses | Squamous cell carcinoma of the nasal cavity and sinuses |
| fr | Carcinome épidermoïde des fosses nasales et des sinus paranasaux | Carcinome épidermoïde des fosses nasales et des sinus |
| de | Plattenepithelkarzinom der Nasenhöhle und Nasennebenhöhlen | Plattenepithelkarzinom der Nasenhöhle und Nebenhöhlen |
| es | Carcinoma de células escamosas de la cavidad nasal y senos paranasales | Carcinoma de células escamosas de la cavidad y los senos nasales |
| it | Carcinoma a cellule squamose della cavità nasale e dei seni paranasali | |
| nl | Plaveiselcelcarcinoom van neusholte en neusbijholten | Plaveiselcelcarcinoom van neusholte en sinussen |
| pt | Carcinoma de células escamosas da cavidade nasal e seios paranasais | |
| pl | Rak płaskonabłonkowy jamy nosowej i zatok przynosowych | = en |
| cs | Dlaždicobuněčný karcinom nosní dutiny a paranazálních dutin | Squamous cell carcinoma of the nasal cavity and sinuses · Dlaždicobuněčný karcinom nosní dutiny a vedlejších nosních dutin |
| uk | Плоскоклітинний рак порожнини носа та приносових пазух | Плоскоклітинний рак порожнини носа та придаткових пазух |
| zh | 鼻腔和鼻旁窦鳞状细胞癌 | = en |
| tr | Burun boşluğu ve paranazal sinüslerin skuamöz hücreli karsinomu | Burun boşluğu ve sinüslerin skuamöz hücreli karsinomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 275355 | NTBT | validated | |
| ICD-10 | D02.3 | NTBT | attributed | validated |
| ICD-11 | 2C20.4 | NTBT | attributed | validated |
| UMLS | C0280332 | 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:67037Squamous cell carcinoma of head and neck
rare otorhinolaryngological diseases; rare neoplastic diseases
No children.
Genes
3 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| ING1 | Disease-causing somatic mutation(s) in | Assessed | HGNC:6062 | ENSG00000153487 | 601566 | 13q34 | inhibitor of growth family member 1 | 10866301[PMID] |
| PTEN | Disease-causing somatic mutation(s) in | Assessed | HGNC:9588 | ENSG00000171862 | 601728 | 10q23.31 | phosphatase and tensin homolog | 11801303[PMID] |
| TNFRSF10B | Major susceptibility factor in | Assessed | HGNC:11905 | ENSG00000120889 | 603612 | 8p21.3 | TNF receptor superfamily member 10b | 9721831[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.35 / 100 000 | Worldwide | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER]_28687376[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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/500464
- This record as JSON: /data/d/500464.json