Follicular dendritic cell sarcoma
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
A rare dendritic cell neoplasm characterized by a proliferation of spindled to ovoid cells with morphological and immunophenotypic features of follicular dendritic cells. Conventional follicular dendritic cell sarcomas are negative for EBV. The tumor arises as a painless, slow-growing mass in lymph nodes (most often cervical), extranodal sites (such as tonsils, gastrointestinal tract, soft tissue, mediastinum, or lung, among others), or both. Paraneoplastic pemphigus may occur in rare cases. Predictive factors are tumor size, presence of coagulative necrosis, mitotic count, and presence of significant cytological atypia.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Follicular dendritic cell sarcoma | |
| fr | Sarcome des cellules folliculaires dendritiques | |
| de | Sarkom, follikuläres dendritisches | |
| es | Sarcoma de células dendríticas foliculares | |
| it | Sarcoma a cellule follicolari dendritiche | |
| nl | Folliculaire dendritische celsarcoom | |
| pt | Sarcoma de células foliculares dendríticas | |
| pl | Mięsak z komórek dendrytycznych grudki | |
| cs | Sarkom z folikulárních dendritických buněk | |
| uk | Фолікулярна дендритноклітинна саркома | |
| zh | 滤泡树突状细胞肉瘤 | |
| tr | Foliküler dendritik 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 | 2B31.5 | E | specific | validated |
| MONDO | 0005764 | E | validated | |
| MeSH | D054740 | E | validated | |
| UMLS | C1260325 | E | validated | |
| MedDRA | 10075332 | 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 (2):
- ORPHA:289656Epstein-Barr Virus-associated mesenchymal tumor
rare neoplastic diseases - ORPHA:3394Soft tissue sarcoma
rare neoplastic diseases
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/86902
- This record as JSON: /data/d/86902.json