Papillary intralymphatic angioendothelioma
Synonyms: Dabska tumor · PILA · Primary intralymphatic angioendothelioma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly, Infancy
- Status
- active
A rare vascular tumor characterized by an ill-defined, slowly growing, asymptomatic cutaneous plaque or nodule mostly involving the limbs, in fewer cases the trunk. The tumor is composed of lymphatic-like channels with prominent intraluminal papillary tufts with hyaline cores lined by hobnail endothelial cells. It is locally aggressive, while metastasis is rare. Infants and children are much more often affected than adults.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Papillary intralymphatic angioendothelioma | Dabska tumor · PILA · Primary intralymphatic angioendothelioma |
| fr | Angioendothéliome papillaire intralymphatique | Angioendothéliome intralymphatique primaire · Tumeur de Dabska |
| de | Papilläres intralymphatisches Angioendotheliom | Dabska-Tumor · PILA |
| es | Angioendotelioma intralinfático primario | Tumor de Dabska |
| it | Angioendotelioma intralinfatico primitivo | |
| nl | Primair intralymfatisch angio-endothelioom | Dabska-tumor |
| pt | Angioendotelioma intralinfático primário | |
| pl | Pierwotny wewnątrzlimfatyczny guz typu angioendotelioma | Guz Dąbskiej |
| cs | Primární intralymfatický angioendoteliom | Dabské tumor |
| uk | Папілярна інтралімфатична ангіоендотеліома | Пухлина Дабська · PILA · Первинна інтралімфатична ангіоендотеліома |
| zh | 原发性淋巴管内血管内皮瘤 | 达布斯卡肿瘤 |
| tr | Primer intralenfatik anjioendotelyoma | Dabska tümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D48.1 | NTBT | attributed | validated |
| MONDO | 0018714 | E | validated | |
| UMLS | C0346087 | 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 | 30 cases | Worldwide | Case(s) | Validated | 21238627[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_21238627[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/458768
- This record as JSON: /data/d/458768.json