Langerhans cell sarcoma
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
A rare dendritic cell tumor characterized by an aggressive, high-grade neoplasm derived from Langerhans cells, most commonly extranodal and multifocal, involving the skin and underlying soft tissue, as well as lung, liver, spleen, and bone. Primary nodal involvement is seen in a minority of patients. Immune-phenotyping and the presence of Birbeck granules on ultrastructural examination reveal the Langerhans cell derivation of the neoplastic cells. Prognosis is generally poor.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Langerhans cell sarcoma | |
| fr | Sarcome des cellules de Langerhans | |
| de | Langerhans-Zell-Sarkom | |
| es | Sarcoma de células de Langerhans | |
| it | Sarcoma a cellule di Langerhans | |
| nl | Langerhanscelsarcoom | |
| pt | Sarcoma de células de Langerhans | |
| pl | Mięsak z komórek Langerhansa | |
| cs | Sarkom z Langerhansových buněk | |
| uk | Клітинна саркома Лангерганса | |
| zh | 朗格汉斯细胞肉瘤 | |
| tr | Langerhans hücre sarkomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C96.4 | NTBT | inclusion | validated |
| ICD-11 | 2B31.3 | E | specific | validated |
| MONDO | 0019480 | E | validated | |
| MeSH | D054752 | E | validated | |
| UMLS | C1260327 | E | validated | |
| GARD | 10491 | E | validated | |
| MedDRA | 10078782 | 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:98289Dendritic cell tumor
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
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/86897
- This record as JSON: /data/d/86897.json