ORPHA:3398
Thymic epithelial neoplasm
Synonyms: TEN · Thymic epithelial tumor
- Type
- Category
- Group
- Group of disorders
- Inheritance
- Autosomal recessive, Not applicable
- Age of onset
- Adult
- Status
- active
Thymic epithelial neoplasms (TEN) are rare malignancies arising from the epithelium of the thymic gland. They comprise three sub-types: thymoma, thymic carcinoma, and thymic neuroendocrine carcinoma.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Thymic epithelial neoplasm | TEN · Thymic epithelial tumor |
| fr | Néoplasie épithéliale thymique | TEN · Tumeur épithéliale thymique |
| de | Thymom, epitheliales | TEN |
| es | Neoplasia epitelial tímica | TEN · Tumor epitelial tímico |
| it | Neoplasia epiteliale del timo | TEN · Tumore epiteliale del timo |
| nl | Epitheliaal neoplasma van thymus | Epitheliale tumor van thymus · TEN |
| pt | Tumor epitelial do timo | |
| pl | Nowotwory nabłonkowe grasicy | Guz nabłonkowy grasicy |
| cs | Epiteliální nádor thymu | TEN |
| uk | Новоутворення епітелію тимуса | TEN · Епітеліальна пухлина вилочкової залози |
| zh | 胸腺上皮肿瘤 | 胸腺上皮肿瘤 |
| tr | Timik epitel neoplazmı | TEN · Timik epitel tümörü |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0018079 | E | validated | |
| MONDO | 18079 | E | validated | |
| MeSH | C536905 | E | validated | |
| UMLS | C1266101 | E | validated | |
| GARD | 5201 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:100100Thymic tumor
rare neoplastic diseases
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.17 / 100 000 | Europe | Value and class | Validated | [EXPERT]_RARECARE surveillance of rare cancers in Europe 2011[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.3 / 100 000 | United States | Value and class | Validated | 22406029[PMID] |
| Point prevalence | Unknown | — | 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.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/3398
- This record as JSON: /data/d/3398.json