Hepatic cystic hamartoma
Synonyms: Biliary hamartoma · MHL · Mesenchymal hamartoma of liver · VMC · Von Meyenburg complexes disease
- Type
- Disease
- Group
- Disorder
- Age of onset
- Infancy
- Status
- active
Hepatic cystic hamartoma, also named Mesenchyma hamartoma of liver, is a rare benign liver tumor of childhood, usually before the age of 2, of mesenchymal origin and variable clinical presentation (abdominal dissension, abdominal mass, pain, vomiting and signs of inferior vena cava compression).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hepatic cystic hamartoma | Biliary hamartoma · MHL · Mesenchymal hamartoma of liver · VMC · Von Meyenburg complexes disease |
| fr | Hamartome kystique hépatique | Hamartome biliaire · Hamartome mésenchymateux du foie · MHL · Maladie des complexes de von Meyenburg · VMC |
| de | Zystisches Leberhamartom | Biliäre Hamartome · Leberhamartom, mesenchymales · MHL · VMC · Von-Meyenburg-Komplex |
| es | Hamartoma quístico hepático | Enfermedad de los complejos de Von Meyenburg · HMH · Hamartoma biliar · Hamartoma mesenquimal hepático · VMC |
| it | Amartoma cistico epatico | Amartoma biliare · Amartoma mesenchimale del fegato · MHL · Malattia dei complessi di von Meyenburg · VMC |
| nl | Hepatisch cystisch hamartoom | Biliair hamartoom · MHL · Mesenchymaal hamartoom van lever · VMC · Ziekte van von Meyenburg-complexen |
| pt | Hamartoma hepático quístico | |
| pl | Torbielowaty guz wątroby o utkaniu hamartoma | Choroba kompleksów Von Meyenburga · Hamartoma dróg żółciowych · Mezenchymalny guz wątroby o utkaniu hamartoma · MHL · VMC |
| cs | Hepatální cystický hamartom | Biliární hamartom · MHL · Mesenchymový hamartom jater · VMC · Nemoc von Meyenbergova komplexu |
| uk | Кістозна гамартома печінки | Біліарна гамартома · Мезенхімальна гамартома печінки · MHL · VMC · Хвороба комплексів фон Мейєнбурга |
| zh | 肝脏胆囊错构瘤 | Von Meyenburg综合征 |
| tr | Karaciğer kistik hamartom | MHL · VMC · Biliyer hamartom · Karaciğerin mezenkimal hamartomu · Von Meyenburg kompleks hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D13.4 | BTNT | attributed | validated |
| ICD-11 | 2E92.7 | NTBT | index | validated |
| MONDO | 0018308 | E | validated | |
| UMLS | C1333971 | E | validated | |
| GARD | 2651 | E | validated | |
| MedDRA | 10074610 | 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: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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 260 cases | Worldwide | Case(s) | Validated | 26858497[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 26858497[PMID]_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/386
- This record as JSON: /data/d/386.json