ORPHA:293202
Epithelioid sarcoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare, soft tissue tumor characterized by high incidence of local recurrence, regional lymph node involvement and distant metastases. It commonly affects the soft tissue under the skin of a finger, hand, forearm, lower leg or foot, less often other areas of the body.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Epithelioid sarcoma | |
| fr | Sarcome épithélioïde | |
| de | Epitheloidsarkom | |
| es | Sarcoma epitelioide | |
| it | Sarcoma epitelioide | |
| nl | Epithelioïd sarcoom | |
| pt | Sarcoma epitelióide | |
| pl | Mięsak nabłonkopodobny | |
| cs | Epiteloidní sarkom | |
| uk | Епітеліоїдна саркома | |
| zh | 上皮样肉瘤 | |
| tr | Epiteloid sarkom |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C49.9 | NTBT | index | validated |
| ICD-11 | 2B5F.2 | NTBT | index | validated |
| ICD-11 | XH4F96 | E | specific | validated |
| MONDO | 0017387 | E | validated | |
| UMLS | C0205944 | E | validated | |
| GARD | 10181 | E | validated | |
| MedDRA | 10015099 | 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:3394Soft tissue sarcoma
rare neoplastic diseases
No children.
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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 / 1 000 000 | — | Europe | Class only | Validated | RARECARE surveillance of rare cancers in Europe 2011[OTHER] |
| Point prevalence | <1 / 1 000 000 | — | Europe | Class only | Not yet validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.04 / 100 000 | United States | Value and class | Validated | 1922430[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.
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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/293202
- This record as JSON: /data/d/293202.json