Combined hepatocellular carcinoma and…

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Combined hepatocellular carcinoma and cholangiocarcinoma

ORPHA:529852Disease

Also called Combined HCC-CC · Combined hepatocellular-cholangiocarcinoma · Hepatocholangiocarcinoma · cHCC-CC

What it is

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.

Key facts

Age of onset
Adult, Elderly
Inheritance
Not applicable
Classified as
Disease

Recorded for the broader condition

Prevalence
1-5 / 10 000 (annual incidence)Carcinoma of gallbladder and extrahepatic biliary tract

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

ICD-10 codes

C22.0ICD-10 uses a narrower term — shared with 8 other rare diseases
C22.1ICD-10 uses a narrower term — shared with 3 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0044791UMLS C0221287

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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