Each year, around 8,000 people in the United States receive a diagnosis of bile duct cancer. This means it’s a very rare cancer.

Your liver produces bile to help you digest fats by turning them into fatty acids. Bile passes from your liver into your small intestine via thin tubes called bile ducts. Cholangiocarcinoma (CCA), also known as bile duct cancer, is a type of cancer that begins in your bile ducts. CCA can occur both inside and outside the liver.

It is not yet clear what causes CCA. Since there is no early test for it, you may want to look out for possible symptoms, which include:

However, not everyone who has CCA experiences these symptoms. Also, having any of these symptoms does not mean you have CCA.

In this article, we explore the likelihood of getting CCA, how it progresses, whether it’s curable, and its survival rate.

In comparison to other cancers, CCA is rare. Each year, around 8,000 people in the United States receive a CCA diagnosis. The actual numbers are probably higher, because it’s difficult to detect CCA early and make a diagnosis.

According to the National Cancer Institute, risk factors include:

The American Cancer Society (ACS) mentions that certain liver or bile duct diseases, as well as other conditions, may lead to CCA. These include:

Other risk factors include:

  • older age
  • diabetes
  • exposure to the radioactive material thorium dioxide, or Thorotrast
  • family history of CCA

Most people with CCA receive a diagnosis in their 60s or 70s. In the United States, CCA is most common in Hispanic Americans.

Even though CCA is rare, research shows that it is an aggressive cancer, and your outlook may depend on the cancer stage. According to a 2019 review, the survival rate once the cancer has spread is very low.

Therefore, it’s crucial to see a doctor immediately if you have any of the symptoms listed above and they last for more than a few weeks. If you have CCA, starting treatment earlier may be more likely to slow the progression of the cancer or prevent it from spreading.

Whether CCA is curable typically depends on the cancer stage at diagnosis.

According to the ACS, doctors will prioritize surgery because it’s the only treatment that can offer a cure. Other options are available, but they primarily work to bring on and lengthen remission.

Because of this, doctors categorize CCA into two types: resectable and unresectable. There are several bile duct cancer stages. Stages 0, 1, 2, and some stage 3 cancers are resectable. Most stage 3 and stage 4 cancers are unresectable.

Resectable CCA is localized cancer that has not spread and can be removed with surgery. In this case, the doctor may need to perform imaging tests such as an MRI or CT scan to ensure that the cancer has not spread to other areas, such as the abdomen. They will also need to make sure you are healthy enough for surgery.

Unresectable CCA is cancer that has spread too far for doctors to reach and fully remove with surgery. In this case, your doctor may recommend chemotherapy and radiation therapy to shrink the cancer and follow up with surgery if it becomes localized. You may also be eligible for a liver transplant, but finding a suitable donor can be difficult.

If your doctor decides surgery is not an option, they may recommend targeted therapy or ablation to help relieve some of the symptoms and slow down the cancer’s growth. Ablation involves using extreme heat or cold to try to destroy as many of the cancer cells as possible.

The outlook for CCA depends on several factors, including its stage, how quickly it grows, and whether it has spread to other organs. Generally, the overall 5-year survival rate for this type of cancer is 23% or lower. However, if doctors diagnose and treat the cancer early enough, you may have a better chance of surviving longer than 5 years.

Survivability tends to drop when the cancer spreads regionally to lymph nodes or distantly to other body parts. It also differs based on whether the cancer started inside or outside the liver.

What is a 5-year survival rate?

Health professionals often use 5-year survival rate as a measure of a disease’s outlook. It describes the percentage of people with the disease who are still alive at least 5 years after their diagnosis.

Another commonly used term is “5-year relative survival rate.” This is a measure of how many people with the disease are alive 5 years later compared to people without the disease.

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Here are the 5-year relative survival rates for CCA that starts within the liver, according to the ACS:

  • Localized: 23%
  • Regional: 9%
  • Distant: 3%

Here are the relative 5-year survival rates for CCA that starts outside the liver, according to the ACS:

  • Localized: 18%
  • Regional: 18%
  • Distant: 2%

Bile duct cancer is rare but quite challenging to diagnose. While treatments are available for bile duct cancer, it may not be curable once it has spread. Maybe we could change the last sentence to “Awareness of risk factors can contribute to early detection, which is vital in improving the outlook.