Bile Duct Cancer Explained
- Bile duct cancer, or cholangiocarcinoma, is a rare, aggressive cancer in the bile ducts, the tubes that transport the digestive fluid bile from the liver to the intestine.
- This cancer differs from liver cancer, requiring unique screening, diagnosis and treatment methods.
- Late-stage symptoms include jaundice (yellowing of the skin and whites of the eys) and abdominal pain, emphasizing the importance of early detection.
- It’s more common in people over 50 and those who have certain liver conditions or genetic issues.
This cancer does have some treatment options. And the earlier it’s caught, the more promising the outcomes.
Read MoreWhat is the biliary system?
The biliary system, also known as the bile duct system, includes your liver, gallbladder, and bile ducts working together to make and transport bile, a digestive fluid. It’s purpose is to help your body efficiently use the food you eat and mange the waste products. The liver produces bile, which breaks down fats in your food, making them easier to absorb.Bile ducts are tubes that carry bile from your liver to your gallbladder and then to your small intestine when needed for digestion. The gallbladder stores bile until your body requires it to digest fats, providing a vital role in your digestive process.
What is Bile Duct Cancer?
When the bile duct system works smoothly, you absorb all of the nutrients from your food properly. However, when any part of the biliary system is diseased,problems can arise. Bile duct cancer can occur in any part of the bile duct system. It is classified into two main types:
- Intrahepatic (within the liver)
- Extrahepatic (outside the liver)
“Bile duct cancers can also affect the bile duct system that runs from within the liver to outside the liver. So we have what are called intrahepatic cholangiocarcinomas, that’s bile duct cancer within the liver. And extrahepatic bile duct cancers which are cholangiocarcinomas that are outside of the liver,” Dr. Elliot Newman, the chief of surgical oncology at the Northwell Health Cancer Institute at Lenox Hill Hospital in Manhattan, tells SurvivorNet.
Bile duct cancer often first shows up in the later stages with subtle symptoms that can be mistaken for less serious conditions.
“Unfortunately bile duct cancers often don’t have symptoms. Patients usually seek medical assistance once symptoms appears. That usually means that the cancer has grown bigger and more advanced,” Dr. Eskander notes.
Common symptoms include:
- Jaundice (yellowing of the skin and eyes due to a build up of bile in the system)
- Itching
- White or clay-like stool (acholia)
- Dark urine (choluria)
- Abdominal pain
- Unexplained weight loss
- Fever
- Fatigue
- Night sweats
Eskander notes that bile duct cancer accounts for about 3% of digestive system cancers. Each year in the US, this translates to roughly 12,000 new cases outside the liver and an increasing number of cases diagnosed inside the liver.
The rising number of cases may be due to improved diagnosis, changing disease classifications, and higher rates of conditions like cirrhosis (liver scarring) and hepatitis C, which elevate the risk of developing this cancer.
Risk Factors for Bile Duct Cancer
Some people are more likely to get bile duct cancer (cholangiocarcinoma) because of certain risk factors, but for many patients, doctors can’t pinpoint a specific reason.
In the United States and Europe, the biggest risks are from a condition called primary sclerosing cholangitis (PSC), a liver disease causing inflammation and scarring of bile ducts, and diseases that affect the liver and bile ducts, like choledochal cysts (fibropolycystic liver disease).
Fibrocystic liver diseases
Fibrocystic liver diseases (FDLs) are rare conditions affecting the liver’s bile ducts due to developmental issues before birth, often linked to genetics. These diseases involve the malfunction of cilia, tiny hair-like structures on cells that play a key role in cellular functions and movement.
The diseases can affect different parts of the bile duct system.
- When they affect the small ducts inside the liver, they cause a condition known as congenital hepatic fibrosis that causes scarring that affects the liver’s normal function.
- When they affect the larger ducts, they cause a condition known as Caroli disease, marked by swelling and expansion of the bile ducts inside the liver.
Both of these can lead to the formation of small abnormal growths and widening of the bile ducts.
Primary Sclerosing Cholangitis
Primary sclerosing cholangitis (PSC) is a condition where the bile ducts inside and outside the liver become inflamed and scarred. This can lead to narrowing of the ducts.
PSC is often found in people with a type of bowel disease called ulcerative colitis (UC); about 40 to 50% of people with PSC have symptoms of UC, and around 90% of people with PSC have UC.
About 30% of people with bile duct cancer also have PSC. Each year, between 0.6% and 1.5% of people with PSC might develop this cancer, and over their lifetime, there’s a 5% to 15% chance they’ll get it. People with PSC usually get bile duct cancer at a younger age, between 30 and 50 years old.
When this condition is present, bile duct cancer becomes harder to diagnose because their bile ducts are already abnormal. Often, bile duct cancer is found within two years of being diagnosed with PSC.
Also, the risk doesn’t seem to depend on how long someone has had the inflammatory disease. However, some studies have shown that it’s not rare for the cancer to show up more than 10 years after someone is diagnosed with PSC.
Other Risk Factors
There are a number of other risk factors for bile duct cancer that include:
- Cirrhosis
- Viral infections (especially for bile duct cancers that starts inside the liver)
- In some countries, infections from a parasite called liver fluke is a common cause of bile duct cancer.
- Genetic conditions, including Lynch Syndrome, BAP1 tumor predisposition syndrome, cystic fibrosis, biliary papillomatosis
- Age, with most patients diagnosed over the age of 50 (with the exception of a PSC diagnosis)
- History of gallstones or bile duct stones
Differences between bile duct cancer and liver cancer
Liver cancer and bile duct cancer are two distinct conditions affecting the liver and its associated bile ducts, each with unique characteristics and implications.
“Primary liver cancer and bile duct cancer are two separate entities but they both affect the same organ: the liver.” – Dr. Newman says.
Liver cancer, primarily hepatocellular carcinoma, originates within the liver itself. It’s often linked to chronic liver conditions like hepatitis or cirrhosis, which can result from alcohol abuse, hepatitis infection, or other liver-damaging conditions.
Liver cancer focuses on the liver’s cells, whereas bile duct cancer targets the pathways critical for bile excretion.
Bile duct cancer is less common than liver cancer and is closely related to bile duct inflammation, blockages, and certain liver diseases.
While both cancers affect the liver region, their origins, risk factors, and impacts on the body differ significantly. Their treatment approaches and prognosis can also vary, emphasizing the importance of distinguishing between these two cancers for effective management
Screening for bile duct cancer
There is no routine screening for bile duct cancer for the overall population.
“Sometimes there are no symptoms early on and screening is not necessarily a slam dunk,” Dr. Eskander says.
However, individuals with known risk factors should undergo regular imaging tests and blood tests to monitor their bile ducts for any signs of cancer.
PSC patients should be screened every 6–12 months. This screening can be performed with imaging tests such as ultrasound or MRI and blood tests.
Magnetic Resonance Imaging
Magnetic Resonance Imaging (MRI) is a non-invasive method that provides detailed images of the bile ducts and surrounding tissues.
It is a powerful tool for detecting bile duct cancer, especially in its early stages. It provides clear images without exposing you to radiation, making it a safe option for screening.
CA 19.9 blood test
Screening for bile duct cancer (cholangiocarcinoma) using the CA 19-9 blood test involves measuring the levels of a tumor marker called carbohydrate antigen 19-9 in your blood.
The CA 19-9 test can be a useful tool for monitoring the progression of bile duct cancer or the effectiveness of treatment in patients who have already been diagnosed. However, due to its lack of specificity, it is not typically used as a primary screening method for bile duct cancer in the general population.
High CA 19-9 levels may indicate bile duct cancer or other conditions, but aren’t exclusive to it and don’t alone confirm a diagnosis.
If your CA 19-9 levels are elevated, your doctor may recommend additional tests, such as imaging scans or a biopsy, to determine the cause and to check for the presence of bile duct cancer.
Questions for your doctor
- What type and stage is my bile duct cancer?
- What are my treatment options?
- Can you explain the benefits and side effects of the recommended treatments?
- Are there any clinical trials or newer treatments available?
- What will insurance cover and what are my out of pocket costs?
<em>Watch: Liver & Bile Duct Cancer: Treatment Progress</em>
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