Inflammatory pseudotumor of the liver
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare benign liver tumor characterized by a prominent inflammatory infiltrate and often mimicking a malignant liver neoplasm. The tumor is frequently solitary with a predilection for the right lobe; however, multiple lesions are possible. There are two clinicopathological subtypes: fibrohistiocytic inflammatory pseudotumor of the liver and lymphoplasmacytic inflammatory pseudotumor of the liver. Patients present with non-specific clinical symptoms such as abdominal pain or discomfort, fever, and weight loss. The condition may be associated with other chronic inflammatory or autoimmune diseases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Inflammatory pseudotumor of the liver | |
| fr | Pseudotumeur inflammatoire du foie | |
| de | Pseudotumor der Leber, inflammatorischer | |
| es | Pseudotumor inflamatorio del hígado | |
| it | Pseudotumore infiammatorio del fegato | |
| nl | Inflammatoire pseudotumor van lever | |
| pt | Pseudotumor inflamatório do fígado | |
| pl | Pseudoguz zapalny wątroby | |
| cs | IgG4 asociovaná hepatopatie | Zánětlivý pseudotumor jater |
| uk | Запальна псевдопухлина печінки | |
| zh | 肝炎性假瘤 | |
| tr | Karaciğerin inflamatuar psödotümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K75.8 | NTBT | attributed | validated |
| ICD-11 | DB99.Y | NTBT | index | validated |
| MONDO | 0019528 | E | validated | |
| UMLS | C1333967 | 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:101939Rare parenchymal liver disease
rare hepatic diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 140 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| 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
17 annotations · validated 2021-02-09| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0031140 | Abnormal liver sonography | |
| HP:0002896 | Neoplasm of the liver | Diagnostic criterion |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0030057 | Autoimmune antibody positivity | |
| HP:0001945 | Fever | |
| Occasional (29-5%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0025406 | Asthenia | |
| HP:0001080 | Biliary tract abnormality | |
| HP:0001394 | Cirrhosis | |
| HP:0000819 | Diabetes mellitus | |
| HP:0410369 | Increased hepatitis B virus antibody level | |
| HP:0002018 | Nausea | |
| HP:0002013 | Vomiting | |
| HP:0001824 | Weight loss | |
| Very rare (<4-1%) | ||
| HP:0031964 | Elevated circulating alanine aminotransferase concentration | |
| HP:0031956 | Elevated circulating aspartate aminotransferase concentration | |
| Excluded (0%) | ||
| HP:0006254 | Elevated alpha-fetoprotein | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 29291783(PMID]_25663462[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90003
- This record as JSON: /data/d/90003.json