Undifferentiated pleomorphic sarcoma
Synonyms: UPS
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
An aggressive sarcoma of soft tissues or bone that can arise from any part of the body, clinically presenting as swelling, mass, pain, pathological fracture and occasional systemic features and is characterized by high local recurrence and significant metastasis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Undifferentiated pleomorphic sarcoma | UPS |
| fr | Sarcome pléomorphe indifférencié | = en |
| de | Sarkom, undifferenziertes pleomorphes | Histiozytom, fibröses malignes |
| es | Sarcoma pleomórfico indiferenciado | |
| it | Istiocitoma fibroso maligno | |
| nl | Ongedifferentieerd pleomorf sarcoom | Ongedifferentieerd pleiomorf sarcoom · UPS |
| pt | Sarcoma pleiomórfico indiferenciado | = en |
| pl | Guz histiocytarny włóknisty złośliwy | = en |
| cs | Nediferencovaný pleomorfní sarkom | = en |
| uk | Недиференційована плеоморфна саркома | = en |
| zh | 未分化多形性肉瘤 | = en |
| tr | Farklılaşmamış pleomorfik sarkom | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C49.9 | NTBT | attributed | validated |
| MONDO | 0002142 | E | validated | |
| MeSH | D051677 | E | validated | |
| UMLS | C0334463 | E | validated | |
| GARD | 6963 | 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 (2):
- ORPHA:223727Bone sarcoma
rare bone diseases; rare neoplastic diseases - ORPHA:3394Soft tissue sarcoma
rare neoplastic diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:293190OBSOLETE: Pleomorphic undifferentiated sarcoma referred to · inactive
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.9 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| Annual incidence | 1-9 / 1 000 000 | 0.9 / 100 000 | United States | Value and class | Validated | National Cancer OTHERitute[OTHER]_17013893[PMID] |
| Point prevalence | 1-9 / 100 000 | — | 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.
HPO phenotypes
9 annotations · validated 2017-10-24| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002814 | Abnormality of the lower limb | |
| HP:0002585 | Abnormality of the peritoneum | |
| HP:0030448 | Soft tissue sarcoma | |
| Frequent (79-30%) | ||
| HP:0003011 | Abnormality of the musculature | |
| HP:0001945 | Fever | |
| Occasional (29-5%) | ||
| HP:0002817 | Abnormality of the upper limb | |
| HP:0002039 | Anorexia | |
| HP:0012378 | Fatigue | |
| HP:0001824 | Weight loss | |
Run the differential on the 3 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/2023
- This record as JSON: /data/d/2023.json