Composite hemangioendothelioma
Synonyms: Polymorphous hemangioendothelioma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare vascular tumor characterized by a poorly circumscribed, infiltrative nodular lesion with vascular differentiation, centered in the dermis and subcutis. The tumor is composed of histologically benign, intermediate, and malignant components. Typical is an admixture of different components which include epithelioid and retiform hemangioendothelioma, spindle cell hemangioma, angiosarcoma-like areas, and benign vascular lesions. Predilection sites are the distal extremities. Many patients have a history of lymphedema. Local recurrence is frequent, while metastasis is rare.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Composite hemangioendothelioma | Polymorphous hemangioendothelioma |
| fr | Hémangioendothéliome composite | |
| de | Hämangioendotheliom, zusammengesetztes | Polymorphes Hämangioendotheliom |
| es | Hemangioendotelioma compuesto | Hemangioendotelioma polimorfo |
| it | Emangioendotelioma composito | Emangioendotelioma polimorfo |
| nl | Samengesteld hemangio-endothelioom | Composiet hemangio-endothelioom · Polymorf hemangio-endothelioom |
| pt | Hemangioendotelioma composto | |
| pl | Naczyniakośródbłoniak złożony | = en |
| cs | Složený hemangioendoteliom | = en |
| uk | Композитна гемангіоендотеліома | |
| zh | 复合性血管内皮瘤 | |
| tr | Kompozit hemanjioendotelyoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D48.1 | NTBT | attributed | validated |
| MONDO | 0018712 | E | validated | |
| UMLS | C1304513 | 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:673473Borderline vascular tumor
rare developmental anomalies during embryogenesis; rare circulatory system diseases; 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 |
|---|---|---|---|---|---|---|
| Cases/families | 39 cases | Worldwide | Case(s) | Validated | 25666464[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_25666464[PMID] |
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/458758
- This record as JSON: /data/d/458758.json