Pleomorphic liposarcoma
Synonyms: PLS
- Type
- Histopathological subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Pleomorphic liposarcoma (PLS), the rarest subtype of liposarcoma (LS), is an aggressive, fast growing tumor located usually in the deep soft tissues of the lower and upper extremities. It is characterized by a variable number of pleomorphic lipoblasts and, in contrast to dedifferentiated liposarcoma, it lacks any association with well-differentiated liposarcoma.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pleomorphic liposarcoma | PLS |
| fr | Liposarcome pléiomorphe | |
| de | Liposarkom, pleomorphes | = en |
| es | Liposarcoma pleomórfico | |
| it | Liposarcoma pleomorfo | = en |
| nl | Pleomorf liposarcoom | PLS · Pleiomorf liposarcoom |
| pt | Lipossarcoma pleomórfico | |
| pl | Tłuszczakomięsak pleomorficzny | plS |
| cs | Pleomorfní liposarkom | = en |
| uk | Плеоморфна ліпосаркома | = en |
| zh | 多形性脂肪肉瘤 | = en |
| tr | Pleomorfik liposarkom | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C49.9 | NTBT | index | validated |
| MONDO | 0020562 | E | validated | |
| UMLS | C0205825 | E | validated | |
| MedDRA | 10073138 | 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:69078Liposarcoma
rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.05 / 100 000 | Europe | Value and class | Validated | 22517534[PMID]_[EXPERT] |
| Point prevalence | Unknown | — | Worldwide | 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
2 annotations · validated 2019-06-17| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0012034 | Liposarcoma | |
| Very frequent (99-80%) | ||
| HP:0001482 | Subcutaneous nodule | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 10778771[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99969
- This record as JSON: /data/d/99969.json