Fibrohistiocytic inflammatory pseudotumor of the liver
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A subtype of inflammatory pseudotumor of the liver characterized by a benign, well-circumscribed tumor with fibrohistiocytic infiltration (including xanthogranulomatous inflammation, multinucleated giant cells, and neutrophilic infiltration), typically localized in the peripheral hepatic parenchyma. Presentation may be of non-specific symptoms (fever, malaise, and abdominal pain) or as an incidental finding.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Fibrohistiocytic inflammatory pseudotumor of the liver | |
| fr | Pseudotumeur inflammatoire du foie, forme fibrohistiocytaire | |
| de | Fibrohistiozytischer inflammatorischer Pseudotumor der Leber | |
| es | Pseudotumor inflamatorio fibrohistiocítico del hígado | |
| it | Pseudotumore infiammatorio fibroistiocitario del fegato | |
| nl | Fibrohistiocytaire inflammatoire pseudotumor van lever | |
| pt | Pseudotumor inflamatório fibrohistiocítico do fígado | |
| pl | Guz rzekomy fibrohistiocytarny zapalnywątroby | |
| cs | Fibrohistiocytický zánětlivý pseudotumor jater | |
| uk | Фіброгістіоцитарна запальна псевдопухлина печінки | |
| zh | 肝纤维组织细胞炎性假瘤 | |
| tr | Karaciğer fibrohistiyositik inflamatuar psödotümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K75.8 | NTBT | attributed | validated |
| MONDO | 0035312 | E | validated | |
| UMLS | C5681328 | 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:90003Inflammatory pseudotumor of the liver
rare hepatic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_17571078[PMID] |
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/555434
- This record as JSON: /data/d/555434.json