Cholangiocarcinoma

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Cholangiocarcinoma

ORPHA:70567Disease

Also called Bile duct cancer · CCA

What it is

Cholangiocarcinoma (CCA) is a biliary tract cancer (BTC) originating in the epithelium of the biliary tree, either intra or extra hepatic.

Key facts

Prevalence
1-9 / 100 000
Age of onset
Adult
Inheritance
Not applicable
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

BRCA1Disease-causing somatic mutation(s)
BRCA2Disease-causing somatic mutation(s)
PTPN3Disease-causing somatic mutation(s)
ROS1Part of a fusion gene

ICD-10 codes

C22.1ICD-10 names this disease exactly — shared with 3 other rare diseases
C24.0ICD-10 uses a narrower term — shared with 3 other rare diseases
C24.8ICD-10 uses a narrower term — shared with 2 other rare diseases
C24.9ICD-10 uses a narrower term — shared with 1 other rare disease

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

GARD 9304MEDDRA 10008593MESH D018281MONDO 0019087OMIM 615619UMLS C0206698

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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