ORPHA:55881
Adamantinoma
Synonyms: Adamantinoma of long bones
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare, primary low-grade malignant bone tumor that occurs in more than 80% of cases on the anterior surface of the tibia (tibial dyaphysis). Most cases are symptomatic or present with pain, swelling, bowing deformity or pathological fracture. Metastases especially in the lungs may be observed.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Adamantinoma | Adamantinoma of long bones |
| fr | Adamantinome | Adamantinome des os longs |
| de | Adamantinom | Adamantinom der langen Knochen |
| es | Adamantinoma | Adamantinoma de huesos largos |
| it | Adamantinoma | |
| nl | Adamantinoom | Adamantinoom van de lange beenderen |
| pt | Adamantinoma | |
| pl | Szkliwiak | = en |
| cs | Adamantinom | = en |
| uk | Адамантинома | Адамантинома довгих кісток |
| zh | 釉质瘤 | 长骨成釉细胞瘤 |
| tr | Adamantinom | Uzun kemiklerin adamantinoması |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 102660 | E | validated | |
| ICD-10 | C40.2 | NTBT | index | validated |
| ICD-11 | 2B5J | NTBT | index | validated |
| ICD-11 | XH8F52 | E | specific | validated |
| MONDO | 0002422 | E | validated | |
| MeSH | D050398 | E | validated | |
| UMLS | C0334556 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:68411Rare bone tumor
rare bone diseases; rare neoplastic diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.01 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Lifetime Prevalence | 1-9 / 1 000 000 | 0.11 / 100 000 | Worldwide | Value and class | Validated | 22033323[PMID]_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.
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HPO phenotypes
3 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0002653 | Bone pain | |
| Occasional (29-5%) | ||
| HP:0003072 | Hypercalcemia | |
| HP:0002756 | Pathologic fracture | |
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/55881
- This record as JSON: /data/d/55881.json