Liver adenomatosis
Synonyms: Hepatic adenomatosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare neoplastic disease characterized by the presence of ten or more hepatocellular adenomas in a background of normal appearing hepatic parenchyma. The majority of reported cases are female. There is no association with steroid use. The condition is considered benign, although the risk of complications (such as malignant transformation or spontaneous rupture with intraperitoneal hemorrhage) is much higher than in isolated hepatic adenoma. Hepatocellular carcinoma develops in less than 10% of cases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Liver adenomatosis | Hepatic adenomatosis |
| fr | Adénomatose hépatique | Adénomatose du foie |
| de | Adenomatose der Leber | Hepatische Adenomatose |
| es | Adenomatosis hepática | Adenomatosis de hígado |
| it | Adenomatosi del fegato | Adenomatosi epatica |
| nl | Adenomatose van lever | Hepatische adenomatose |
| pt | Adenomatose hepática | |
| pl | Gruczolakowatość wątroby | = en |
| cs | Adenomatóza jater | = en |
| uk | Аденоматоз печінки | Аденоматоз печінки |
| zh | 肝腺瘤病 | = en |
| tr | Karaciğer adenomatozu | Hepatik adenomatoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D13.4 | NTBT | attributed | validated |
| ICD-11 | 2E92.7 | NTBT | attributed | validated |
| MONDO | 0035447 | E | validated | |
| UMLS | C5680136 | 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:506210Rare disorder potentially indicated for liver transplant
rare transplant-related disorders - ORPHA:306636Rare tumor of liver and intrahepatic biliary tract
rare hepatic diseases; rare neoplastic 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_26011213[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/566841
- This record as JSON: /data/d/566841.json