ORPHA:99971
Well-differentiated liposarcoma
Synonyms: ALT · Atypical lipoma · Atypical lipomatous tumor · WDLS
- Type
- Histopathological subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Well-differentiated liposarcoma (WDLS), the most common type of liposarcoma (LS), is a slow growing, painless tumor usually located in the retroperitoneum or the limbs. It is composed of proliferating mature adipocytes.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Well-differentiated liposarcoma | ALT · Atypical lipoma · Atypical lipomatous tumor · WDLS |
| fr | Liposarcome bien différencié | ALT · Lipome atypique · Tumeur lipomateuse atypique · WDLS |
| de | Liposarkom, hochdifferenziertes | ALT · Lipom, atypisches · Tumor, atypischer lipomatöser · WDLS |
| es | Liposarcoma bien diferenciado | ALT · Lipoma atípico · Tumor lipomatoso atípico · WDLS |
| it | Liposarcoma ben differenziato | ALT · Lipoma atipico · Tumore lipomatoso atipico · WDLS |
| nl | Goed gedifferentieerd liposarcoom | ALT · Atypisch lipoom · Atypische lipomateuze tumor · WDLS |
| pt | Lipossarcoma bem diferenciado | |
| pl | Tłuszczakomięsak dobrze zróżnicowany | ALT · Atypowy guz tłuszczowy · Tłuszczak atypowy · WDLS |
| cs | Dobře diferencovaný liposarkom | ALT · Atypický lipom · Atypický lipomatózní nádor · WDLS |
| uk | Добре диференційована ліпосаркома | ALT · Атипова ліпома · Атипова ліпоматозна пухлина · WDLS |
| zh | 高分化脂肪肉瘤 | 非典型脂肪性肿瘤 |
| tr | İyi diferansiye liposarkom | ALT · WDLS · Atipik lipom · Atipik lipomatöz tümör |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C49.9 | NTBT | index | validated |
| MONDO | 0005103 | E | validated | |
| UMLS | C1370889 | 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:69078Liposarcoma
rare neoplastic diseases
No children.
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Genes
3 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| CDK4 | Role in the phenotype of | Assessed | HGNC:1773 | ENSG00000135446 | 123829 | 12q14.1 | cyclin dependent kinase 4 | |
| HMGA2 | Role in the phenotype of | Assessed | HGNC:5009 | ENSG00000149948 | 600698 | 12q14.3 | high mobility group AT-hook 2 | |
| MDM2 | Role in the phenotype of | Assessed | HGNC:6973 | ENSG00000135679 | 164785 | 12q15 | MDM2 proto-oncogene |
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.51 / 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.
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HPO phenotypes
3 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001482 | Subcutaneous nodule | |
| Occasional (29-5%) | ||
| HP:0012211 | Abnormal renal physiology | |
| HP:0002579 | Gastrointestinal dysmotility | |
Run the differential on the 1 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99971
- This record as JSON: /data/d/99971.json