Cholangiocarcinoma, also known as bile duct cancer or biliary tract cancer, is a rare type of cancer that develops in the bile ducts of the body. The bile ducts are a group of thin tubes that carry bile (a digestive fluid) from the liver and gallbladder to the intestines.
The liver is an organ that sits under the ribs on the upper right side of the abdomen. It is a component of the digestive system, and has many important functions such as producing bile to dissolve fat and digest food. The bile produced by the liver is sent to the gallbladder (a small, pear-shaped organ located under the liver) to be stored. The liver has two bile ducts, known as the left and right hepatic ducts, which join together outside of the liver to form the common hepatic duct. The cystic duct from the gallbladder joins the common hepatic duct to form the common bile duct. Cancer can develop in any of these bile ducts. In most cases, cholangiocarcinoma occurs as a type of carcinoma.
Cholangiocarcinoma is slightly more common in males, and tends to be diagnosed in people over 70. However, anyone can develop this disease.
Types of Cholangiocarcinoma
There are three primary types of cholangiocarcinoma, which are categorised by the area they develop in.
Intrahepatic cholangiocarcinoma is a cancer that develops in any of the bile ducts within the liver. These types of cancers may be confused with hepatocellular carcinomas of the liver, and are often treated in the same way. Unfortunately, these cancers are often aggressive.
Hilar cholangiocarcinoma, also known as perihilar cholangiocarcinoma or Klatskin tumour, is the most common type of cholangiocarcinoma. It is a cancer that develops at the junction of the right and left hepatic bile ducts in an area known as the hilum. Unfortunately, these cancers are often aggressive.
Extrahepatic cholangiocarcinoma is a cancer that develops in a bile duct outside of the liver. This generally occurs in the common bile duct, and often close to the small intestine. Unfortunately, these cancers are often aggressive.
If a cholangiocarcinoma is detected, it will be staged and graded based on size, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including location, age, stage of disease and overall health.
Treatment for cholangiocarcinomas may include:
- Surgery, potentially including:
- Hepatectomy (removal of part or all of the liver).
- Hepatic lobectomy (removal of a lobe of the liver).
- Whipple procedure/pancreaticoduodenectomy (procedure to remove portions of affected organs, such as the pancreas, gallbladder, stomach, small intestine, and lymph nodes).
- Cholecystectomy (removal of the gallbladder).
- Stent placement (insertion of a thin wire to allow excess fluid to drain).
- Liver transplant.
- Lymphadenectomy (removal of affected lymph nodes).
- Portal vein embolisation.
- Radiation therapy.
- Clinical trials.
- Palliative care.
For more information on the treatment options, please refer to the Rare Cancers Australia treatment options page.
While the cause of cholangiocarcinomas remain unknown, the following factors may increase the likelihood of developing the disease:
- Long term inflammation of the bile ducts, potentially caused by:
- Infection with the virus Hepatitis B or C.
- Fatty liver disease.
- Chronic liver disease.
- Inflammatory bowel diseases, potentially including:
- Crohn’s disease.
- Ulcerative colitis.
- Infection with the liver fluke parasite.
- Bile duct stones.
- Liver cirrhosis.
- Certain inherited conditions, such as:
- Choledochal cysts.
- Cystic fibrosis.
- Lynch syndrome.
- Having a history of smoking.
- Excessive alcohol intake.
- Diabetes (types 1 &2).
- Exposure to certain chemicals (especially in the printing industry).
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Early-stage cholangiocarcinomas may appear asymptomatic. As the cancer progresses, some of the following symptoms may appear:
- Jaundice (yellowing of the skin and eyes).
- Itchy skin.
- Abdominal pain, discomfort and/or swelling.
- Unexplained weight loss/loss of appetite.
- Nausea and vomiting.
- Dark urine.
- Pale or clay coloured stool.
- Fevers and chills.
- A hard and potentially painful lump on the upper right side of the abdomen.
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
If your doctor suspects you have a cholangiocarcinoma, they will order a variety of tests to confirm the diagnosis and refer you to a specialist for treatment.
Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities.
Blood tests are used to assess overall health and detect any abnormalities. Some of these tests may include:
- General blood test to assess overall health.
- Full blood count, which measure the levels of red blood cells, white blood cells and platelets.
- Liver function test.
- Blood chemistry and/or blood hormone studies, which analyse the levels of certain hormones and other substances in the blood.
- Serum tumour marker test (a common tumour marker for cholangiocarcinoma is CA19-9).
The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), ultrasound, x-ray and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and look for signs of metastasis.
Once the location(s) of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells. This can be done by a fine needle aspiration (FNA) or a core needle biopsy (CNB).
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on the rate and depth of tumour growth, susceptibility to treatment, age, overall fitness, and medical history. Generally, early-stage cholangiocarcinomas have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.