Vulvovaginal rhabdomyosarcoma
- Type
- Disease
- Group
- Disorder
- Status
- active
Vulvovaginal rhabdomyosarcoma is a rare vulvovaginal tumour, a highly malignant soft tissue sarcoma composed of cells with round to oval or spindle-shaped nuclei and eosinophilic cytoplasm that may show differentiation towards striated muscle cells. It usually affects children and presents with a vulvar or vaginal mass that may be polypoid or grape-like (embryonal subtype) and associated with bleeding and ulceration.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Vulvovaginal rhabdomyosarcoma | |
| fr | Rhabdomyosarcome vulvo-vaginal | |
| de | Rhabdomyosarkom, vulvovaginales | |
| es | Rabdomiosarcoma vulvovaginal | |
| it | Rabdomiosarcoma vulvovaginale | |
| nl | Vulvovaginaal rhabdomyosarcoom | |
| pt | Rabdomiossarcoma do trato genital | |
| pl | Mięsak prążkowanokomórkowy sromu i pochwy | |
| cs | Vulvovaginální rabdomyosarkom | |
| uk | Вульвовагінальна рабдоміосаркома | |
| zh | 外阴横纹肌肉瘤 | |
| tr | Vulvovajinal rabdomyosarkom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C52 | NTBT | attributed | validated |
| ICD-11 | 2C71.Y | NTBT | index | validated |
| UMLS | C4707823 | 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:180312Rare vulvovaginal tumor
rare neoplastic diseases; rare gynecological and obstetric diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/206492
- This record as JSON: /data/d/206492.json