Dendritic cell sarcoma not otherwise specified
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare dendritic cell tumor characterized by a neoplasm composed of spindled to ovoid cells with phenotypic features similar to those of normal indeterminate cells. The tumor cells consistently express S100 protein and CD1a, while langerin, specific B- and T-cell markers, CD30, the histiocytic marker CD163, and the follicular dendritic cell markers CD21, CD23, and CD35 are negative. Birbeck granules are absent on ultrastructural examination. Patients typically present with multiple papules, nodules, or plaques of the skin. Primary lymph node or splenic involvement is less common. Systemic symptoms are usually absent. The clinical course is highly variable.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Dendritic cell sarcoma not otherwise specified | |
| fr | Sarcome des cellules dendritiques sans autre spécification | |
| de | Dendritisches Zell-Sarkom, andernorts nicht klassifiziert | |
| es | Sárcoma de células dendriticas sin otra especificación | |
| it | Sarcoma a cellule dendritiche non atrimenti specificato | |
| nl | Dendritische celsarcoom niet anders gespecificeerd | |
| pt | Sarcoma de células dendríticas, não especificado | |
| pl | Mięsak z komórek dendrytycznych nie sklasyfikowany w inny sposób | |
| cs | Sarkom z dendritických buněk jinak nespecifikovaný | |
| uk | Дендритноклітинна саркома, не специфікована | |
| zh | 未分类树突细胞肉瘤 | |
| tr | Dendritik hücre sarkomu, başka türlü tanımlanmamış |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C96.4 | NTBT | attributed | validated |
| ICD-11 | 2B31.Y | NTBT | index | validated |
| ICD-11 | XH8Q19 | E | specific | validated |
| MONDO | 0019482 | E | validated | |
| MONDO | 19482 | E | validated | |
| MONDO | 4380 | E | validated | |
| UMLS | C1301364 | 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/86903
- This record as JSON: /data/d/86903.json