Combined hepatocellular carcinoma and cholangiocarcinoma
Synonyms: Combined HCC-CC · Combined hepatocellular-cholangiocarcinoma · Hepatocholangiocarcinoma · cHCC-CC
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare hepatic tumor characterized by the presence of both hepatocytic and cholangiocytic differentiation within a primary liver carcinoma. The lesion commonly arises in the context of chronic liver disease (such as hepatitis B or C, or steatohepatitis) or exposure to a variety of exogenous agents. Patients may present with signs and symptoms related to the tumor, as well as to the underlying condition. Typical manifestations include right upper quadrant abdominal pain, weight loss, hepatosplenomegaly, jaundice, and ascites. The entity has been associated with a worse prognosis than hepatocellular carcinoma after resection.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Combined hepatocellular carcinoma and cholangiocarcinoma | Combined HCC-CC · Combined hepatocellular-cholangiocarcinoma · Hepatocholangiocarcinoma · cHCC-CC |
| fr | Hépatocholangiocarcinome | Cholangiocarcinome hépatocellulaire combiné · HCC-CC combiné · cHCC-CC |
| de | Kombiniertes hepatozelluläres Karzinom und Cholangiokarzinom | Hepatocholangiokarzinom · Kombiniertes HCC-CC · Kombiniertes hepatozelluläres und Cholangiokarzinom · cHCC-CC |
| es | Carcinoma hepatocelular y colangiocarcinoma combinado | HCC-CC combinado · Hepatocarcinoma-colangiocarcinoma combinado · Hepatocolangiocarcinoma · cHCC-CC |
| it | Carcinoma epatocellulare combinato e colangiocarcinoma | |
| nl | Gecombineerd hepatocellulair carcinoom en cholangiocarcinoom | Gecombineerd HCC-CC · Gecombineerd hepatocellulair-cholangiocarcinoom · Hepatocholangiocarcinoom · cHCC-CC |
| pt | Carcinoma hepatocelular e colangiocarcinoma combinado | HCC-CC combinado · Hepatocolangiocarcinoma · cHCC-CC |
| pl | Rak złożony wątrobowo-komórkowy i dróg żółciowych | = en |
| cs | Kombinovaný hepatocelulární karcinom a cholangiokarcinom | cHCC-CC · Kombinované HCC-CC · Hepatocholangiokarcinom |
| uk | Поєднання гепатоцелюлярної карциноми та холангіокарциноми | cHCC-CC · Комбінований HCC-CC · Комбінована гепатоцелюлярно-холангіокарцинома · Гепатохолангіокарцинома |
| zh | 肝细胞癌合并胆管上皮癌 | Combined hepatocellular-cholangiocarcinoma |
| tr | Kombine hepatoselüler karsinom ve kolanjiyokarsinom | cHCC-CC · Kombine HCC-CC · Hepatokolanjiokarsinom · Kombine hepatoselüler-kolanjiokarsinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C22.0 | BTNT | inclusion | validated |
| ICD-10 | C22.1 | BTNT | inclusion | validated |
| ICD-11 | 2C12.00 | E | specific | validated |
| MONDO | 0044791 | E | validated | |
| UMLS | C0221287 | 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 (3):
- ORPHA:56044Carcinoma of gallbladder and extrahepatic biliary tract
rare hepatic diseases; rare neoplastic diseases - ORPHA:424936Carcinoma of liver and intrahepatic biliary tract
rare hepatic diseases; rare neoplastic diseases - ORPHA:506210Rare disorder potentially indicated for liver transplant
rare transplant-related disorders
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_23034166[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/529852
- This record as JSON: /data/d/529852.json