Histiocytic sarcoma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare histiocytic tumor characterized by a malignant proliferation of cells showing morphological and immunophenotypic features of mature tissue histiocytes. Most cases occur in extranodal sites, most commonly the intestinal tract, skin, and soft tissue. Patients may present with a solitary mass, lymphadenopathy, a skin rash or numerous tumors on the trunk and extremities, lytic bone lesions, hepatosplenomegaly with pancytopenia, intestinal obstruction, and/or systemic symptoms. The neoplasm is aggressive with typically poor therapy response.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Histiocytic sarcoma | |
| fr | Sarcome histiocytaire | |
| de | Sarkom, histiozytäres | |
| es | Sarcoma histiocítico | |
| it | Sarcoma istiocitico | |
| nl | Histiocytair sarcoom | |
| pt | Sarcoma histiocítico | |
| pl | Mięsak histiocytarny | |
| cs | Histiocytární sarkom | |
| uk | Гістіоцитарна саркома | |
| zh | 组织细胞肉瘤 | |
| tr | Histiyositik sarkom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C96.8 | E | specific | validated |
| ICD-11 | 2B31.1 | E | specific | validated |
| MONDO | 0019479 | E | validated | |
| MeSH | D054747 | E | validated | |
| UMLS | C0334663 | E | validated | |
| MedDRA | 10076876 | 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:98288Macrophage or histiocytic 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/86896
- This record as JSON: /data/d/86896.json