Nodular fasciitis
Synonyms: Pseudosarcomatous fasciitis · Pseudosarcomatous fibromatosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare soft tissue tumor characterized by a solitary mass-forming fibrous proliferation that usually occurs in the subcutaneous tissue, composed of uniform fibroblastic/myofibroblastic cells displaying a loose growth pattern. Upper extremities, trunk, and head and neck are most frequently affected. The lesion typically grows rapidly and almost always measures less than five centimeters in diameter. Macroscopically, it may appear circumscribed or infiltrative but is not encapsulated. Recurrence after excision is very rare, and metastasis does not occur.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Nodular fasciitis | Pseudosarcomatous fasciitis · Pseudosarcomatous fibromatosis |
| fr | Fasciite nodulaire | Fasciite pseudo-sarcomateuse · Fibromatose pseudosarcomateuse |
| de | Noduläre Fasziitis | Fibromatose, pseudosarkomatöse · Pseudosarkomatöse Fasziitis |
| es | Fascitis nodular | Fascitis pseudosarcomatosa · Fibromatosis pseudosarcomatosa |
| it | Fascite nodulare | |
| nl | Nodulaire fasciitis | Pseudosarcomateuze fasciitis · Pseudosarcomateuze fibromatose |
| pt | Fascite nodular | |
| pl | Guzkowe zapalenie powięzi | = en |
| cs | Nodulární fascitida | = en |
| uk | Вузликовий фасціїт | Псевдосаркоматозний фасціїт · Псевдосаркоматозний фіброматоз |
| zh | 增生性筋膜炎 | 假肉瘤性纤维瘤病 |
| tr | Nodüler fasiit | Psödosarkomatöz fasiit · Psödosarkomatöz fibromatoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M79.8 | NTBT | attributed | validated |
| ICD-11 | FB51.2 | NTBT | index | validated |
| MONDO | 0004187 | E | validated | |
| UMLS | C0410005 | E | validated | |
| MedDRA | 10065988 | 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 (2):
- ORPHA:71209Rare soft tissue tumor
rare neoplastic diseases - ORPHA:79382Subcutaneous tissue disease
rare skin diseases
No children.
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| MYH9 | Part of a fusion gene in | Assessed | HGNC:7579 | ENSG00000100345 | 160775 | 22q12.3 | myosin heavy chain 9 | 21826056[PMID] |
| USP6 | Part of a fusion gene in | Assessed | HGNC:12629 | ENSG00000129204 | 604334 | 17p13.2 | ubiquitin specific peptidase 6 | 21826056[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
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/477742
- This record as JSON: /data/d/477742.json