Biliary tract cancer is a rare form of cancer that primarily affects the body’s bile ducts. There are two primary forms of biliary tract cancer: bile duct cancer, known as cholangiocarcinoma, and gallbladder cancer.
The bile tract itself is made out of ducts and tubes—it’s the system that transports bile from the liver into the small intestine. Bile itself is necessary for the body to help digest food. The biliary tree is made up of ducts that carry bile produced by the liver into the small intestine. It plays a significant role in a functioning digestive system. During digestion, bile is brought to the gallbladder and then released into the small intestine through these ducts.
Biliary cancers are a group of heterogeneous tumors, which come from the epithelial lining cells of the liver’s small ducts, along with the main biliary system ducts to the gallbladder. This group includes cholangiocarcinoma, gallbladder cancer, intrahepatic and extrahepatic bile duct cancers, and ampullary carcinoma. Currently, the incidences of these cancers are not well known, given the difficulty of adequately establishing accurate diagnoses. It’s a relatively rare disease accounting for an estimated 10 to 20% of liver cancers.
Ultimately, cancer begins when cells within the body start to grow out of control. Just about any cell in the body can become cancerous, which can spread to other areas of the body. From what is understood about these diseases, each subtype of biliary tract cancer is classified depending on where cancer first developed.
Cholangiocarcinoma can form anywhere along the bile ducts. Intrahepatic cancers are first found inside the liver’s bile duct, while extrahepatic cancer begins in ducts apart from those in the liver. Historically, cholangiocarcinoma covers both EHC and IHC. Approximately half of bile duct cancers involve what is called a Klatskin tumor. These form in the right hepatic joins, with the left hepatic duct in the liver. Cancer that starts in the common bile duct is called bile duct cancer, but if multiple tumors are found along various areas of the bile duct, the disease is classified as multifocal bile duct cancer.
Cholangiocarcinoma is usually incurable at the time of diagnosis. This cancer forms along the thin tubes of the bile ducts. Two bile ducts are vital: one that comes from the liver and the gallbladder. These two specific ducts connect to form a common tube that leads towards the small intestine. Bile duct cancer is found in this area and typically occurs among people who are older than the age of 50.
There are different forms of cholangiocarcinoma. They’re identified based on where cancer occurs in the bile ducts.
- Intrahepatic cholangiocarcinoma – Appears in areas of the bile ducts found within the liver. This type makes up about 5 to 10% of bile duct cancers, and it can sometimes be misdiagnosed as liver cancer.
- Hilar cholangiocarcinoma – Occurs in bile ducts not found within the liver, and is also called perihilar cholangiocarcinoma. Klatskin tumors are most frequently found in this variety of cancer.
- Distal cholangiocarcinoma – Found closer to the small intestine, at the opposite end of where perihilar cancer is found. This type makes up about a quarter of all bile duct cancers.
The gallbladder is a pear-shaped organ found in the upper abdomen. Small and located next to the liver, this is where bile is primarily stored as the liver produces it, aiding the body in digesting and storing fats. Sadly, this form of cancer is difficult to diagnose, as there are often no main signs or symptoms. Because the gallbladder itself tends to stay hidden away in the body’s processes, cancer can grow undetected. If found early, the prognosis for the patient can be quite good. However, when the diagnosis comes at the late stage, the prognosis can be unfavorable.
Even though gallbladder cancer is far rarer than bile duct cancer, there are multiple varieties of gallbladder cancers as well. The biggest category of gallbladder cancer is adenocarcinomas; this cancer starts at the gland-like cells in the digestive tract’s organs. Roughly 90% of gallbladder cancers are adenocarcinomas. Papillary adenocarcinoma is one of the essential subtypes to know about, as it has a far better prognosis than all the other types. It’s unlikely to spread to other organs and lymph nodes. Other subtypes include the non-papillary adenocarcinomas (75% of the class) and mucinous adenocarcinomas.
Some relatively uncommon varieties of gallbladder cancer are squamous cell carcinomas, sarcomas, and lymphomas.
Biliary Tract Cancer
For both significant classes of biliary tract cancer, significant signs or symptoms may not appear in early stages. Therefore, it can be quite difficult to detect. Frequently diagnosis occurs when the cancer has already reached its more advanced stages. Also, even though people of nearly any age can be diagnosed with biliary tract cancer. It’s often senior individuals around age 70 who are diagnosed with the disease.
Cholangiocarcinoma symptoms may include:
- Jaundice – As bile is secreted by the liver and then sent to the intestine via bile ducts, jaundice occurs when the liver is unable to release bile as needed. Bile itself contains a yellow-green chemical called bilirubin. If the body does not adequately process this substance, it backs up into the bloodstream instead and settles into different parts of the body. Jaundice is one of the most common symptoms of this type of cancer.
- Pale or greasy stool – Bilirubin is also responsible for the brown color in stool, so a lack of it creates a lighter color. If the cancer is blocking the release of the bile and pancreatic substances, the body may not be able to properly digest fatty foods, resulting in greasy stools coming from undigested fat.
- Dark-colored urine – As bilirubin levels in the blood elevate, it can affect urine, making it darker.
- Itching – Excess bilirubin can trigger itchy skin.
- Abdominal pain – Generally only seen in later stages, significantly large tumors may cause pain at the right side below the ribs.
Other uncommon signs and symptoms are loss of appetite, fever, nausea, and vomiting.
Gallbladder cancer can be even harder to detect early; it doesn’t produce signs and symptoms until the cancer is already well advanced or if the tumor it’s caused has become unusually large.
Some critical signs and symptoms that gallbladder cancer does produce are:
- Abdominal pain – Most people suffering from this form of cancer will feel pain in the upper right area of their abdomen or belly.
- Nausea and vomiting – Some individuals experience vomiting.
- Jaundice – As mentioned earlier, this is a symptom that occurs when the liver fails to release bile as needed. If the cancer becomes large enough to block bile ducts, bilirubin builds up in the patient’s blood and spreads to different areas of the body. The yellowing of jaundice can be most evident in the white area of the eyes.
- Belly lumps – If cancer has also blocked the bile ducts, the gallbladder itself is at risk for swelling. The disease can even spread to nearby parts of the liver and can be felt by doctors during a physical test or by diagnostic imaging.
Other less common potential symptoms include weight loss, loss of appetite, abdominal swelling, fever, itchy skin, dark urine, and pale stool. In other words, both gallbladder cancer and bile duct cancer can have similar symptoms but have different primary symptoms.
One important thing to remember about these cancers is that they are rare, and there is a chance that a different condition may cause some of the symptoms. It’s critical to consult a doctor to be able to make the correct diagnosis and prognosis for the patient.
Biliary Tract Cancer
So what causes biliary tract cancers? Cholangiocarcinoma is caused by DNA mutations in bile duct cells. DNA is the genetic material that functions as a set of instructions to give each cell in the body its purpose. If cells replicate out of control, they eventually form a tumor or a mass of cancerous cells. However, the specific conditions that lead to these genetic mutations remain unclear.
Gallbladder cancer forms when the DNA of healthy gallbladder cells also mutates. Instead of dying, the cells replicate out of control, forming a tumor that can grow well beyond the gallbladder and eventually spread toward other organs or parts of the body. The cancer typically begins around the glandular cells lining the inner surface of the gallbladder itself.
Cholangiocarcinoma itself is a rare occurrence in the Western world, with an incidence estimated at 0.5 to 2 people per 100,000. Roughly 8,000 people in the US are diagnosed with this disease every year. Rates are higher in Southeast Asia, as there may be parasitic infections that cause cancer to manifest. Asian countries may have up to 40 times higher rates of cholangiocarcinoma.
People of all ages can be diagnosed with the disease, though it mostly affects older people. The average age of the US population diagnosed with these types of cancer is 70 years old. For extrahepatic bile ducts, the age is 72 years old. The chances of surviving this type of cancer largely depend on where the disease is found and how advanced it is.
Gallbladder cancer, on the other hand, is somewhat more common. The American Cancer Society estimates nearly 12,000 cases are diagnosed each year, with slightly more women than men affected. A little over 4,000 people die from these cancers every year.
One of the most significant issues for gallbladder cancer is that it doesn’t present symptoms in the early stages, leading to diagnosis only after the disease has already advanced, making it far more challenging to manage and worsening the prognosis. Only one in five gallbladder cancers are discovered in their early stages when there is still ample time to stop it from spreading to other areas of the body. Chances for survival in gallbladder cancer largely depend on the extent of the disease when it is found.
Risk factors are things that affect a person’s chances of contracting a disease. Many bile duct cancer risk factors are chronic or long-standing conditions involving the bile duct, including:
- Primary sclerosing cholangitis
- Bile duct stones
- Choledochal cyst disease
- Liver fluke infections
- Bile duct abnormalities
- Hepatitis B or C
- Polycystic liver disease
- Caroli syndrome
- Inflammatory bowel disease
- Old age
- Ethnicity and geography (particularly prevalent among Hispanics, and is more often found in Southeast Asia and China)
- Non-alcoholic fatty liver disease
- Thorotrast exposure
- Family history
- Chronic pancreatitis (long-term inflammation of the pancreas)
- Infection with HIV (the virus that causes AIDS)
- Exposure to asbestos
- Exposure to radon or other radioactive chemicals
- Exposure to dioxin, nitrosamines, or polychlorinated biphenyls (PCBs)
Risk factors for gallbladder cancer include:
- Porcelain gallbladder (a condition wherein calcium deposits form on the wall of the gallbladder)
- Gender (women are more susceptible than men)
- Age (this type of cancer primarily manifests in older people)
- Ethnicity and geography (Hispanic and Native Americans are more likely than others, while African Americans are less likely than others)
- Choledochal cysts
- Bile duct abnormalities
- Polyps in the gallbladder
- Primary sclerosing cholangitis
- Family history
- Exposure to chemicals used in the rubber and textile industries
- Exposure to nitrosamines
Diagnosis & Testing
Biliary tract cancer can be particularly dangerous because both bile duct cancer and gallbladder cancer don’t show signs in early stages. Many people wouldn’t know that they had these cancers unless they went to a doctor to be diagnosed.
Most bile duct cancers and gallbladder cancers aren’t found until a medical specialist is consulted, and specific tests are performed. When diagnosing both these cancers to determine if a patient has them, the doctor may need to do the following tests:
- Comprehensive medical history and physical exam – Should a patient suspect that they might have bile duct cancer, their complete medical history needs to be examined. A doctor will want to do a physical examination to seek out signs of bile duct cancer or any other health problems that could potentially be causing symptoms.
- Blood tests – Lab tests will be required to look into the state of the liver and the gallbladder. It’s vital to determine whether these two organs are functioning correctly. Blood tests also need to be done to determine the level of bilirubin in the system. The blood will also be screened for tumor markers.
- Imaging tests – Doctors use x-rays, MRIs, CT scans, cholangiography, and sometimes even ultrasound to detect the presence of a tumor in the body. The imaging tests are specifically used to search for suspicious areas and help doctors look for a mass to conduct a biopsy on. It’s also a means to check how far the cancer has spread and allow doctors to make better treatment decisions depending on how advanced the condition has become.
- Biopsy – If imaging tests show proof or suggest that there is a blockage or a mass present, a biopsy is performed so doctors can take a sample of the tissue. Doctors can then perform tests on the sample to determine the advancement of cancer.
Biliary Tract Cancer
Doctors are still studying biliary tract cancer to determine the best method of treatment for them. By learning about gene and protein changes, they’re hoping to develop newer drugs to target those changes specifically. Chemotherapy is a common cancer treatment that uses drugs to kill cancerous cells. Targeted therapy is one way to treat these forms of cancer and work differently from standard chemotherapy drugs because it is directed at a narrower range of tissue.
Diet & Lifestyle:
While no specific diet has been proven to prevent or treat biliary tract cancer truly, there are diet and lifestyle changes that a person can make to limit the risk as much as possible. Maintaining a healthy weight and eating a balanced diet with a focus on plant foods can boost the body’s health and immune system. Staying active and getting plenty of physical exercises also helps.
Therapy and Procedures:
- Surgery – Two types of surgery may be performed for individuals who have biliary tract cancer. Potentially curative surgery is performed when previous tests show evidence that there is a good chance that the tumor can be safely removed from the body. This is known as resectable surgery. However, only a small percentage of these forms of cancer can be resectable when they are first found. Palliative surgery, on the other hand, is performed to relieve the symptoms produced by cancer and prevent further complications from arising. This improves the patient’s quality of life but does not cure the disease.
- Radiation Therapy – Radiation therapy can use high energy rays and particles targeted to destroy the cancer cells and prevent them from growing further. This can be performed before surgery (neoadjuvant) or after surgery (adjuvant).
Chemotherapy – This therapy uses cancer-killing drugs administered via the mouth or IV. The drugs then spread throughout the body and the bloodstream, even in areas that cancer hasn’t reached yet, preventing it from spreading. While this form of therapy can be helpful for some people, it’s not yet clear how effective it is for biliary tract cancers.
Research & Advancement
Researchers’ major focus in biliary tract cancer is looking for new drugs and new treatments to help patients who suffer from these diseases. These include photodynamic therapies, immunotherapy, radiosensitizers, and other forms of targeted therapy. There is still no consistent cure for this type of cancer, so research seeks to find a cure as well as other treatments that may improve quality of life and extend life.
Clinical trials are a core part of the research process to ensure that new treatments are both effective and safe. In addition to clinical trials, researchers are increasingly making use of real-world evidence, which is information that is collected in the normal course of care for people with these cancers. Reviewing medical records and patient histories is particularly important for biliary tract cancers. Because these are cancers that don’t show visible signs and symptoms, comprehensive medical accounts are necessary to consider the symptoms and to make a correct diagnosis. Electronic health records that provide a very detailed look into the patient’s medical history and treatment are a treasure trove of information about the disease’s progression, signs, causes, and potential risks.
How do I avoid biliary tract cancer, including cholangiocarcinoma and gallbladder cancer?
Getting vaccinated against hepatitis, taking careful precautions to avoid blood-borne diseases, limiting alcohol use, and quitting smoking can all be methods to limit the likelihood of developing these cancers.
I have biliary tract cancer. What can I do to cope?
Ask questions and learn about your disease. Make sure to discuss it thoroughly with your oncologist so that you’re fully aware of what is happening to you and your body, as well as what steps to take next. Staying connected to friends and family, as well as getting connected to patient advocacy organizations, can be helpful in learning about and coping with the disease.
How long do bile duct cancer/gallbladder patients have to live?
Survival rates depend on how advanced the cancer is upon diagnosis. The five-year life expectancy of cholangiocarcinoma is about 30%. On average, patients with bile duct cancer in the liver who undergo surgery to completely remove the tumor live for an average of two years. For incomplete removal, the average is 21 months. For gallbladder cancer, there is a five-year life expectancy is about 60%, but it also largely depends on how far the disease has progressed. It’s important to remember that all these numbers are estimates, and outlooks may be better than they are initially perceived.
Part of understanding and finding better treatments for biliary tract cancer is through the careful study of patient records and tests. PicnicHealth makes it easier to get your complete medical records to aid in managing the disease. Visit PicnicHealth.com to sign up for a free PicnicHealth account if you have cholangiocarcinoma or gallbladder cancer.