Giant cell tumor of bone
Synonyms: GCT of bone · Osteoclastoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare bone sarcoma characterized by a usually benign space-occupying lesion, which is nevertheless locally aggressive and massively damaging to surrounding bone tissue. The tumor is composed of giant multinucleated cells (osteoclast-like cells), mononuclear macrophages, and mononuclear stromal cells which secrete pro-myeloid and pro-osteoclastic factors. Metastasis and malignant transformation are rare, but the recurrence rate is high.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Giant cell tumor of bone | GCT of bone · Osteoclastoma |
| fr | Tumeur osseuse à cellules géantes | Ostéoclastome · TCG osseuse |
| de | Riesenzell-Tumor des Knochens | GCT des Knochens · Osteoklastom |
| es | Tumor óseo de células gigantes | Osteoclastoma · TCG de hueso |
| it | Tumore dell'osso a cellule giganti | GCT dell'osso |
| nl | Reusceltumor van bot | GCT van bot · Osteoclastoom |
| pt | Tumor ósseo de células gigantes | |
| pl | Wielkokomórkowy guz kości | GCT kości |
| cs | Obrovskobuněčný nádor kostí | GCT kostí |
| uk | Гігантоклітинна пухлина кісток | GCT кісток · Остеокластома |
| zh | 骨巨细胞瘤 | 骨巨细胞瘤 |
| tr | Dev hücreli kemik tümörü | Kemik GCT'si · Osteoklastom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D48.0 | NTBT | index | validated |
| ICD-11 | 2F7B | NTBT | index | validated |
| ICD-11 | 2F9B | NTBT | index | validated |
| ICD-11 | XH0492 | BTNT | specific | validated |
| ICD-11 | XH4TC2 | BTNT | specific | validated |
| MONDO | 0005674 | E | validated | |
| MeSH | D018212 | E | validated | |
| UMLS | C0206638 | E | validated | |
| GARD | 13046 | E | validated | |
| MedDRA | 10005969 | 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:223727Bone sarcoma
rare bone diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.1404 / 100 000 | Worldwide | Value and class | Validated | WHO Classification of Tumours[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | WHO Classification of Tumours[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/363976
- This record as JSON: /data/d/363976.json