Leiomyosarcoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare soft tissue sarcoma characterized by a malignant space-occupying lesion most commonly located in the retroperitoneum or the inferior vena cava, but also other soft tissues, and composed of cells showing distinct features of smooth muscle cells. The tumor presents with mass effect depending on the location. It is capable of both local recurrence and distant metastasis, while lymph node metastasis is rare. Prognosis largely depends on tumor location and size.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Leiomyosarcoma | |
| fr | Léiomyosarcome | |
| de | Leiomyosarkom | |
| es | Leiomiosarcoma | |
| it | Leiomiosarcoma | |
| nl | Leiomyosarcoom | |
| pt | Leiomiosarcoma | |
| pl | Mięsak gładkokomórkowy | |
| cs | Leiomyosarkom | |
| uk | Лейоміосаркома | |
| zh | 平滑肌肉瘤 | |
| tr | Leiomyosarkom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C49.9 | NTBT | attributed | validated |
| MONDO | 0005058 | E | validated | |
| MeSH | D007890 | E | validated | |
| UMLS | C0023269 | E | validated | |
| GARD | 6880 | E | validated | |
| MedDRA | 10024189 | 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:289656Epstein-Barr Virus-associated mesenchymal tumor
rare neoplastic diseases - ORPHA:3394Soft tissue sarcoma
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 | Unknown | — | Worldwide | Class only | Validated | WHO Classification of Tumours[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | WHO Classification of Tumours[OTHER] |
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/64720
- This record as JSON: /data/d/64720.json