Interdigitating dendritic cell sarcoma
Synonyms: Interdigitating cell sarcoma · Reticulum cell sarcoma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare dendritic cell tumor characterized by a neoplasm composed of spindle to ovoid cells with phenotypic features similar to those of interdigitating dendritic cells. Solitary lymph node involvement is common, although extranodal localization (in particular skin and soft tissue) has also been reported. Patients usually present with an asymptomatic mass, sometimes with systemic symptoms such as fatigue, fever, and night sweats. Generalized lymphadenopathy, splenomegaly, or hepatomegaly may be seen in rare cases. The clinical course is generally aggressive.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Interdigitating dendritic cell sarcoma | Interdigitating cell sarcoma · Reticulum cell sarcoma |
| fr | Sarcome des cellules dendritiques interdigitées | Sarcome des cellules interdigitées · Sarcome des cellules réticulaires interdigitées |
| de | Interdigitierendes dendritisches Zellsarkom | Interdigitierendes Sarkom der dendritischen Zellen · Zellsarkom, retikuläres |
| es | Sarcoma de células dendríticas interdigitante | Sarcoma de células interdigitantes · Sarcoma de células reticulares |
| it | Sarcoma a cellule dendritiche interdigitate | Sarcoma a cellule interdigitate · Sarcoma a cellule reticolari |
| nl | Sarcoom van interdigiterende dendritische cellen | Sarcoom van interdigiterende cellen · Sarcoom van reticulumcellen |
| pt | Sarcoma de células dendríticas interdigitantes | |
| pl | Mięsak ze splatających się komórek dendrytycznych | = en |
| cs | Sarkom z interdigitujících dendritických buněk | Sarkom z interdigitujících buněk |
| uk | Інтердигітальна дендритноклітинна саркома | Інтердигітальна клітинна саркома · Саркома з клітин сітківки |
| zh | 指突状树突细胞肉瘤 | 指突状树突细胞肉瘤 |
| tr | İç içe geçmiş dendritik hücre sarkomu | Retikulum hücreli sarkom · İnterdijitasyon hücre sarkomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 267730 | E | validated | |
| ICD-10 | C96.4 | NTBT | inclusion | validated |
| ICD-11 | 2B31.4 | E | specific | validated |
| MONDO | 0009975 | E | validated | |
| MeSH | D054739 | E | validated | |
| UMLS | C1260326 | E | validated | |
| MedDRA | 10038804 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | — | Europe | Class only | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
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/86900
- This record as JSON: /data/d/86900.json