Cholangiocarcinoma (CCA) also known as bile duct cancer, is a rare and aggressive type of cancer that affects the bile ducts, which carry digestive fluid (bile) and connect your liver, gallbladder and small intestine. It is known as a difficult to treat cancer with few treatment options1

Approximately, 8,000 people in the U.S. are diagnosed with CCA each year, but the actual number of cases may be higher. The disease can be hard to diagnose, leading CCA to be misclassified as other cancers.2

There are three different ways in which bile duct cancers are categorized, depending on where the cancer starts.3 

Intrahepatic cholangiocarcinoma

  • Occurs in the smaller bile ducts inside the liver. 

Perihilar cholangiocarcinoma

  • Occurs in the bile ducts just outside of the liver.3

Distal bile cholangiocarcinoma

  • Occurs further down the bile duct, closer to the small intestine.3

IDH1 Mutation

Genetic sequencing has identified new potential therapeutic targets for CCA. One genetic alteration of interest is isocitrate dehydrogenase-1 (IDH1), where there are several small molecules being explored as a targeted treatment option.

IDH1 mutations occur in up to 20% of cholangiocarcinoma cases in the US and are not associated with prognosis.

Mutations, like an IDH1 mutation, are changes in the DNA (deoxyribonucleic acid) of a cell, which can play a role in the development of a disease. That is why testing for mutations can help your healthcare team understand which targeted therapies and treatments may work for you. 


Survivorship in CCA begins the day of diagnosis and continues throughout life. It is considered one of the most complex aspects of the cancer experience because it is different for every patient. While treatment may completely destroy or remove the cancer for some people, others may need to maintain regular treatment to control the cancer.6 

The treatment paradigm for this disease has transformed over the past few years, with molecular profiling along for more targeted and personalized treatment options. Always remember to speak with your oncologist and other members of your healthcare team about treatments, tools and resources that can help you navigate your cancer journey. 

Download our latest CCA Fact Sheet to learn more. 

1. Mayo Clinic. Cholangiocarcinoma (bile duct cancer). Accessed March 8, 2021. 

2. American Cancer Society. Key Statistics for Bile Duct Cancer. Accessed March 8, 2021. 

3. American Cancer Society. What is Bile Duct Cancer? Accessed March 8, 2021. 

4. Cripso, F., Pietrafesa, M., Condelli V., Maddalena, F., Bruno G., Piscazzi, A. Sgambato, A., Esposito, F. & Landriscina, M. (2020). IDH! Targeting as a New Potential Option for Intrahepatic Cholangiocarcinoma Treatment-Current State and Future Perspectives. Molecules (Basel, Switzerland), 25(16), 3754. 

5. Boscoe, A., Rolland, C., & Kelley, R. (2019). Frequency and prognostic significance of isocitrate dehydrogenase 1 mutations in cholangiocarcinoma: a systematic literature review. Journal Of Gastrointestinal Oncology, 10(4), 751-765. 

6. American Cancer Society. Living as a Bile Duct Cancer Survivor. Accessed March 8, 2021. 

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