ORPHA:33276
Kaposi sarcoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare vascular tumor that is characterized by human herpes virus 8 (HHV-8)-induced endothelial inflammatory neoplasm that develops with various clinically distinct settings, manifesting mostly as cutaneous lesions, or mucosal or visceral involvement.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Kaposi sarcoma | |
| fr | Sarcome de Kaposi | Maladie de Kaposi |
| de | Kaposi-Sarkom | |
| es | Sarcoma de Kaposi | |
| it | Sarcoma di Kaposi | |
| nl | Kaposi-sarcoom | |
| pt | Sarcoma de Kaposi | |
| pl | Mięsak Kaposiego | |
| cs | Kaposiho sarkom | |
| uk | Саркома Капоші | |
| zh | 卡波西肉瘤 | |
| tr | Kaposi sarkomu |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 148000 | BTNT | validated | |
| ICD-10 | C46.0 | BTNT | specific | validated |
| ICD-10 | C46.1 | BTNT | specific | validated |
| ICD-10 | C46.2 | BTNT | specific | validated |
| ICD-10 | C46.3 | BTNT | specific | validated |
| ICD-10 | C46.7 | BTNT | specific | validated |
| ICD-10 | C46.8 | BTNT | specific | validated |
| ICD-10 | C46.9 | BTNT | specific | validated |
| ICD-11 | 1C60.30 | BTNT | specific | validated |
| ICD-11 | 1C61.30 | BTNT | specific | validated |
| ICD-11 | 2B57 | E | specific | validated |
| ICD-11 | 2B57.0 | BTNT | specific | validated |
| ICD-11 | 2B57.1 | BTNT | specific | validated |
| ICD-11 | 2B57.2 | BTNT | specific | validated |
| ICD-11 | 2B57.Y | BTNT | attributed | validated |
| ICD-11 | XH36A5 | E | specific | validated |
| MONDO | 0005055 | E | validated | |
| MeSH | D012514 | E | validated | |
| UMLS | C0036220 | E | validated | |
| GARD | 6814 | E | validated | |
| MedDRA | 10023284 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:673473Borderline vascular tumor
rare developmental anomalies during embryogenesis; rare circulatory system diseases; rare neoplastic diseases - ORPHA:102024Human herpesvirus 8-related disorder
rare neoplastic diseases - ORPHA:3394Soft tissue sarcoma
rare neoplastic diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
26 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.34 / 100 000 | Europe | Value and class | Validated | 22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | 1-9 / 100 000 | — | Europe | Class only | Validated | 22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Lifetime Prevalence | 1-9 / 100 000 | 2.11 / 100 000 | Europe | Value and class | Validated | 22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.051 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.075 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.099 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.092 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.065 / 100 000 | Estonia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.014 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.076 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.027 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.043 / 100 000 | Poland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.05 / 100 000 | Slovakia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.056 / 100 000 | Slovenia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.133 / 100 000 | Croatia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.111 / 100 000 | Ireland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.108 / 100 000 | Latvia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.112 / 100 000 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.597 / 100 000 | Malta | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.915 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.546 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.163 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.278 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.192 / 100 000 | United Kingdom | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.363 / 100 000 | Iceland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.142 / 100 000 | Italy | Value and class | 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.
6
HPO phenotypes
26 annotations · validated 2017-01-24| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0005353 | Recurrent herpes | |
| Very frequent (99-80%) | ||
| HP:0002814 | Abnormality of the lower limb | |
| HP:0001034 | Hypermelanotic macule | |
| HP:0012733 | Macule | |
| HP:0008069 | Neoplasm of the skin | |
| Frequent (79-30%) | ||
| HP:0000479 | Abnormal retinal morphology | |
| HP:0011024 | Abnormality of the gastrointestinal tract | |
| HP:0001743 | Abnormality of the spleen | |
| HP:0001298 | Encephalopathy | |
| HP:0008940 | Generalized lymphadenopathy | |
| HP:0001028 | Hemangioma | |
| HP:0002721 | Immunodeficiency | |
| HP:0005523 | Lymphoproliferative disorder | |
| HP:0200034 | Papule | |
| HP:0200036 | Skin nodule | |
| Occasional (29-5%) | ||
| HP:0002088 | Abnormal lung morphology | |
| HP:0001392 | Abnormality of the liver | |
| HP:0002014 | Diarrhea | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0001004 | Lymphedema | |
| HP:0011793 | Neoplasm by anatomical site | |
| HP:0200035 | Skin plaque | |
| HP:0000988 | Skin rash | |
| HP:0005293 | Venous insufficiency | |
| HP:0001824 | Weight loss | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 12705742[PMID]_11994760[PMID]
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/33276
- This record as JSON: /data/d/33276.json