Calcifying aponeurotic fibroma
Synonyms: Juvenile aponeurotic fibromatosis · Keasby tumor
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare, superficial fibromatosis characterized by non-malignant, locally invading, fibrosing tumour of differentiated fibroblasts, slowly growing subcutaneously, occurring predominantly distally on the extremities, especially the hands and feet. Histologic examination shows a multinodular pattern with large areas of calcification and fibrosis, and the presence of elongated spindle cells with hyperchromatic plump vesicular nuclei interspersed within fine bands of collagen.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Calcifying aponeurotic fibroma | Juvenile aponeurotic fibromatosis · Keasby tumor |
| fr | Fibrome aponévrotique calcifié | Fibromatose aponévrotique juvénile · Tumeur de Keasby |
| de | Fibrom, aponeurotisches kalzifizierendes | Fibromatose, aponeurotische juvenile · Keasby-Tumor |
| es | Fibroma aponeurótico calcificado | Fibromatosis aponeurótica juvenil · Tumor de Keasby |
| it | Fibroma aponeurotico calcificante | Fibromatosi aponeurotica giovanile · Tumore di Keasby |
| nl | Calcificerend aponeurotisch fibroom | Juveniele aponeurotische fibromatose · Keasby-tumor |
| pt | Fibroma aponevrótico calcificado | |
| pl | Zwapniały włókniak rozcięgna | Guz Keasby'ego · Młodzieńcza włókniakowatość rozcięgna |
| cs | Kalcifikující aponeurotický fibrom | Juvenilní aponeurotický fibrom · Keasbyův nádor |
| uk | Кальцинуюча апоневротична фіброма | Ювенільний апоневротичний фіброматоз · Пухлина Кізбі |
| zh | 钙化性腱膜纤维瘤 | 青少年腱膜纤维瘤病 |
| tr | Kalsifiye aponevrotik fibrom | Keasby tümörü · jüvenil aponevrotik fibromatoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M72.8 | NTBT | attributed | validated |
| ICD-11 | EE61 | NTBT | attributed | validated |
| MONDO | 0016038 | E | validated | |
| MeSH | C000625499 | E | validated | |
| UMLS | C0553647 | 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:199257Superficial fibromatosis
rare skin diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/199260
- This record as JSON: /data/d/199260.json