Benign epithelial tumor of salivary glands
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
Benign epithelial tumor of salivary glands is a rare neoplastic disease characterized by the presence of a tumor located in the parotid, sublingual, submandibular and/or minor salivary glands, which presents with a wide spectrum of clinical features depending on the location, size and type of salivary gland involved, usually manifesting as a slow-growing, painless, commonly solitary mass, rarely associated with facial nerve palsy or nasal/airway obstruction.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Benign epithelial tumor of salivary glands | |
| fr | Tumeur épithéliale bénigne des glandes salivaires | |
| de | Speicheldrüsentumor, epithelialer benigner | |
| es | Tumor epitelial benigno de las glándulas salivales | |
| it | Tumore epiteliale benigno delle ghiandole salivari | |
| nl | Goedaardige epitheliale tumor van speekselklieren | |
| pt | Tumor epitelial benigno das glândulas salivares | |
| pl | Łagodny guz nabłonkowy ślinianek | |
| cs | Benigní epiteliální nádor slinných žláz | |
| uk | Доброякісна епітеліальна пухлина слинних залоз | |
| zh | 唾液腺良性上皮性肿瘤 | |
| tr | Tükürük bezlerinin selim epitel tümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 181030 | BTNT | validated | |
| ICD-10 | D11.0 | BTNT | specific | validated |
| ICD-10 | D11.7 | BTNT | specific | validated |
| ICD-11 | 2E91 | E | specific | validated |
| ICD-11 | 2E91.0 | BTNT | specific | validated |
| ICD-11 | 2E91.1 | BTNT | specific | validated |
| MONDO | 0017168 | E | validated | |
| UMLS | C5190858 | 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:276142Rare tumor of salivary glands
rare gastroenterological diseases; rare neoplastic diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:454821OBSOLETE: Pleomorphic salivary gland adenoma referred to · inactive
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| HMGA2 | Part of a fusion gene in | Assessed | HGNC:5009 | ENSG00000149948 | 600698 | 12q14.3 | high mobility group AT-hook 2 | 23821214[PMID] |
| PLAG1 | Part of a fusion gene in | Assessed | HGNC:9045 | ENSG00000181690 | 603026 | 8q12.1 | PLAG1 zinc finger | 21394649[PMID]_23821214[PMID] |
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/276148
- This record as JSON: /data/d/276148.json