Rare cancer called cholangiocarcinoma begins in the bile ducts. Thin tubes called bile ducts transport bile, a fluid that aids in food digestion, from the liver and gallbladder to the small intestine.
A cholangiocarcinoma diagnosis is typically given to a patient when the cancer has already metastasized outside of their bile ducts. The prognosis (chance of recovery) is typically not good, and it is challenging to treat. A rising number of focused treatments and clinical studies are beginning to alter that, though.
Types Of Cholangiocarcinoma
The three main types of cholangiocarcinoma are intrahepatic, perihilar, and distal.
Intrahepatic Cholangiocarcinoma (ICC):
ICC is the rarest type of cholangiocarcinoma, accounting for 10-20% of all cases. It arises from the smaller bile ducts within the liver and can be difficult to diagnose early on because it may not cause any symptoms until later stages of the disease. ICC tends to be more aggressive and has a worse prognosis than other types of cholangiocarcinoma.
Perihilar Cholangiocarcinoma (PCC):
PCC, also known as Klatskin tumor, is the most common type of cholangiocarcinoma, accounting for 50-60% of all cases. It occurs at the junction where the right and left hepatic ducts meet to form the common hepatic duct. PCC can cause obstruction of the bile ducts, leading to symptoms such as jaundice, itching, and abdominal pain.
Distal Cholangiocarcinoma (DCC):
DCC occurs in the lower part of the bile duct, near the point where it enters the small intestine. It accounts for 20-30% of all cases of cholangiocarcinoma. DCC tends to be less aggressive than ICC and PCC, but can still cause significant symptoms and have a poor prognosis if not diagnosed and treated early.
Read More:- https://www.cancerresearchuk.org/about-cancer/bile-duct-cancer/types
Other less common types of cholangiocarcinoma include peripheral cholangiocarcinoma, which occurs in the smaller bile ducts within the liver but is distinct from ICC, and hilar cholangiocarcinoma, which occurs in the same location as PCC but is a distinct subtype with different features and treatment considerations.
Causes And Risk Factors Of Cholangiocarcinoma
The exact cause of cholangiocarcinoma is not yet fully understood, but several risk factors have been identified. One of the most significant risk factors is primary sclerosing cholangitis (PSC), a chronic inflammatory condition of the bile ducts that can eventually lead to their destruction and scarring. People with PSC have a significantly increased risk of developing cholangiocarcinoma, with estimates suggesting that up to 20% of PSC patients will eventually develop the cancer.
Another significant risk factor is liver fluke infection. Liver flukes are parasitic flatworms that infect the bile ducts of humans and other animals. Infection with liver flukes is particularly common in Southeast Asia, where it is estimated that up to 25 million people are infected. The flukes can cause chronic inflammation and damage to the bile ducts, which can eventually lead to the development of cholangiocarcinoma.
Exposure to certain chemicals and toxins is also a significant risk factor for cholangiocarcinoma. For example, exposure to thorium dioxide, a radioactive compound that was once used in X-ray imaging, has been linked to an increased risk of cancer. Additionally, exposure to certain industrial chemicals such as vinyl chloride, nitrosamines, and asbestos has also been associated with an increased risk.
Inherited genetic mutations can also increase the risk of cholangiocarcinoma. For example, mutations in the genes BRCA1 and BRCA2, which are commonly associated with breast and ovarian cancer, have also been linked to an increased risk of bile duct cancer. Other genetic conditions, such as Lynch syndrome, can also increase the risk.
Chronic viral infections can also increase the risk of cholangiocarcinoma. For example, infection with hepatitis B or C can cause chronic inflammation and damage to the liver, which can eventually lead to the development of bile duct cancer. Additionally, infection with the human immunodeficiency virus (HIV) has also been linked to an increased risk of cholangiocarcinoma.
Certain lifestyle factors can also increase the risk of cholangiocarcinoma. For example, heavy alcohol consumption has been associated with an increased risk of cancer, as has smoking. Obesity and a sedentary lifestyle have also been linked to an increased risk.
Read More:- https://www.cancer.gov/types/liver/bile-duct-cancer/causes-risk-factors
Finally, there is evidence to suggest that age and gender may also play a role in the development of cholangiocarcinoma. Cancer is more common in people over the age of 50, and it is slightly more common in men than in women.
Symptoms Of Cholangiocarcinoma
The symptoms of cholangiocarcinoma can vary depending on the location and stage of the cancer.
Early-stage cholangiocarcinoma may not cause any symptoms, or the symptoms may be very mild and non-specific. As the cancer grows and spreads, however, the symptoms can become more severe and may include:
- Abdominal pain: One of the most common symptoms of cholangiocarcinoma is abdominal pain, which may be dull or sharp and may be felt in the upper right side of the abdomen. The pain may be intermittent or constant, and it may be aggravated by eating or drinking.
- Jaundice: Jaundice is a condition that occurs when the liver is unable to process bilirubin, a substance produced when red blood cells break down. Bilirubin then accumulates in the bloodstream and causes yellowing of the skin and whites of the eyes. In cholangiocarcinoma, jaundice can occur when the tumor blocks the bile ducts and prevents the flow of bile.
- Itching: When bilirubin accumulates in the bloodstream due to cholangiocarcinoma, it can also cause itching, especially in the hands and feet. The itching may be severe and may not respond to over-the-counter remedies.
- Fatigue: Cholangiocarcinoma can cause fatigue, which is a feeling of extreme tiredness or weakness. This can be due to the cancer itself or the stress it places on the body.
- Nausea and vomiting: Cholangiocarcinoma can cause nausea and vomiting, which may be related to the tumor or the body’s response to the cancer.
- Loss of appetite: Cholangiocarcinoma can cause a loss of appetite, which may lead to unintended weight loss.
- Pale stools: In cholangiocarcinoma, the bile ducts can become blocked, which can cause pale stools or stools that are white or gray in color.
- Dark urine: When the bile ducts are blocked in cholangiocarcinoma, bilirubin can accumulate in the bloodstream and cause dark urine.
- Fever: In some cases, cholangiocarcinoma can cause a fever, which is a sign that the body is fighting an infection or inflammation.
- Enlarged liver: In some cases, cholangiocarcinoma can cause the liver to become enlarged, which may be felt as a mass in the upper right side of the abdomen.
It’s important to note that these symptoms are not specific to cholangiocarcinoma and can occur with other conditions as well. If you are experiencing any of these symptoms, it’s important to see a healthcare provider to determine the cause and receive appropriate treatment.
Read More:- https://www.cancercenter.com/cancer-types/bile-duct-cancer/symptoms
Complications of Cholangiocarcinoma
Cholangiocarcinoma can cause a variety of complications, which can affect multiple systems in the body. Some of the most significant complications of cholangiocarcinoma include:
- Jaundice: One of the most common symptoms of cholangiocarcinoma is jaundice, which is caused by the buildup of bilirubin in the body. Bilirubin is a yellow pigment that is created when the liver breaks down old red blood cells. When the bile ducts are blocked or narrowed by cholangiocarcinoma, bilirubin cannot be excreted from the body properly, leading to the yellowing of the skin and eyes.
- Bile duct obstruction: Cholangiocarcinoma can cause a blockage or narrowing of the bile ducts, which can prevent bile from flowing into the small intestine. This can cause a backup of bile in the liver, leading to inflammation, infection, and scarring.
- Liver damage: Cholangiocarcinoma can also damage the liver, leading to the accumulation of toxins in the body. This can cause a range of symptoms, including fatigue, confusion, and loss of appetite.
- Ascites: In advanced cases of cholangiocarcinoma, the liver can become damaged to the point where it cannot function properly. This can cause a buildup of fluid in the abdomen, a condition known as ascites. Ascites can cause swelling and discomfort in the abdomen, as well as difficulty breathing.
- Malnutrition: Cholangiocarcinoma can cause malabsorption of nutrients in the small intestine, leading to malnutrition. Malnutrition can cause a range of symptoms, including weakness, fatigue, and weight loss.
- Metastasis: Cholangiocarcinoma can metastasize, or spread to other parts of the body, including the liver, lungs, and bones. Metastatic cholangiocarcinoma can cause a range of symptoms, depending on where the cancer has spread.
- Pain: Cholangiocarcinoma can cause pain in the abdomen, as well as the back and shoulders. This pain can be caused by a variety of factors, including the pressure of the tumor on surrounding organs and tissues.
- Bleeding: Cholangiocarcinoma can cause bleeding in the digestive tract, leading to black, tarry stools and vomiting blood.
- Infection: Cholangiocarcinoma can also increase the risk of infection, particularly in the bile ducts and liver. Infections can cause a range of symptoms, including fever, chills, and abdominal pain.
- Depression: The diagnosis of cholangiocarcinoma can cause significant psychological distress, which can lead to depression and anxiety. These mental health conditions can cause a range of symptoms, including sadness, loss of interest in activities, and difficulty sleeping.
Diagnosis Of Cholangiocarcinoma
There are several diagnostic tests that can be used to diagnose cholangiocarcinoma. These include:
- Blood Tests: Blood tests can be used to check for elevated levels of liver enzymes and bilirubin, which can be a sign of cholangiocarcinoma. Additionally, tumor markers such as CA 19-9 can be elevated in some cases.
- Imaging Tests: Imaging tests such as ultrasound, CT scan, and MRI can be used to visualize the bile ducts and detect any abnormalities, such as a tumor or blockage.
- Endoscopic Retrograde Cholangiopancreatography (ERCP): This procedure involves the insertion of a flexible tube with a camera and a light (endoscope) through the mouth and into the small intestine. Dye is then injected into the bile ducts, which allows for visualization and identification of any abnormalities or blockages.
- Magnetic Resonance Cholangiopancreatography (MRCP): MRCP uses MRI technology to create detailed images of the bile ducts without the need for an invasive procedure.
- Biopsy: A biopsy involves the removal of a small sample of tissue from the bile ducts, which can then be examined under a microscope for the presence of cancer cells. Biopsy can be done using various techniques such as endoscopic biopsy or percutaneous biopsy.
- Positron Emission Tomography (PET) Scan: PET scan uses a radioactive tracer to create images of the body and is used to check for the spread of cancer to other parts of the body.
Read More:- https://www.cancer.gov/types/liver/bile-duct-cancer/diagnosis
Treatment Of Cholangiocarcinoma
There are several different types of cholangiocarcinoma, each with their own specific treatment options. The treatment of cholangiocarcinoma typically involves a multidisciplinary approach that includes surgery, chemotherapy, and radiation therapy.
Surgery is often the first line of treatment for cholangiocarcinoma. The goal of surgery is to remove as much of the cancer as possible. The type of surgery that is performed depends on the location and stage of the cancer. In some cases, a liver transplant may be necessary. However, surgery is not always an option, particularly if the cancer has spread to other parts of the body.
Chemotherapy is another treatment option for cholangiocarcinoma. Chemotherapy uses drugs to kill cancer cells or stop them from growing. Chemotherapy may be used before or after surgery, or it may be used as the primary treatment if surgery is not an option. There are several different chemotherapy drugs that can be used for cholangiocarcinoma, including gemcitabine and cisplatin.
Your tumour can be shrunk via systemic chemotherapy, which is administered throughout your body, making surgery possible. Chemotherapy is used to extend life and lessen cancer symptoms if the malignancy is too advanced for surgery. Procedures can sometimes be carried out to deliver chemotherapy directly to your bile duct:
- Transarterial chemoembolization (TACE), which inserts minuscule chemotherapy beads into the blood arteries feeding the tumour using a catheter. To stop blood from reaching the tumour, the beads block the vessel. The beads also release chemo drugs at the same time to reduce the tumour size.
- Using a surgically implanted pump, hepatic artery infusion (HAI) delivers chemotherapy directly into the principal artery that supplies blood to the liver.
Radiation therapy may also be used to treat cholangiocarcinoma. Radiation therapy uses high-energy X-rays to kill cancer cells or stop them from growing. Radiation therapy may be used before or after surgery, or it may be used as the primary treatment if surgery is not an option. There are several different types of radiation therapy that can be used for cholangiocarcinoma, including external beam radiation therapy and brachytherapy.
In addition to these standard treatments, there are several emerging therapies that are being studied for the treatment of cholangiocarcinoma. These include immunotherapy, targeted therapy, and gene therapy.
Immunotherapy is a type of treatment that uses the body’s own immune system to fight cancer. Immunotherapy drugs work by helping the immune system recognize and attack cancer cells. Immunotherapy may be used in combination with other treatments, such as chemotherapy or radiation therapy.
Targeted therapy is a type of treatment that uses drugs to target specific molecules that are involved in the growth and spread of cancer cells. Targeted therapy may be used in combination with other treatments, such as chemotherapy or radiation therapy.
Gene therapy is a type of treatment that involves modifying genes to help the body fight cancer. Gene therapy may be used in combination with other treatments, such as chemotherapy or radiation therapy.
While there is no cure for cholangiocarcinoma, treatment can help to control the cancer and improve quality of life. The best treatment approach depends on several factors, including the stage and location of the cancer, as well as the patient’s overall health and preferences. A multidisciplinary team of doctors and other healthcare professionals can work together to develop a personalised treatment plan for each patient.
In addition to medical treatments, supportive care can also be an important part of the treatment of cholangiocarcinoma. Supportive care can help to manage symptoms and side effects of treatment, as well as provide emotional and social support. Supportive care may include pain management, nutritional support, and counselling.
Read More:- https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq
How is staging for cholangiocarcinoma done?
Your healthcare provider can learn the stage of your cancer from a biopsy in addition to whether you have it. The process of staging involves determining how much cancer is present in your body. Your doctor can better arrange your care with the aid of this information. The size of the tumour and whether the disease has spread outside the bile ducts are factors in determining the stage of bile duct cancer.
- arterial system.
- lymphatic nodes and vessels.
- the liver and gallbladder, which are organs located close to the bile ducts.
- The lungs, bones, or abdominal cavity are distant organs (called the peritoneum).
How likely is it that someone with cholangiocarcinoma will survive?
People who have cholangiocarcinoma typically have a dismal prognosis. 10% to 15% of patients with bile duct cancer who have not spread outside of the bile ducts will survive for five years. If the disease spreads to organs like the lungs that are far from the bile ducts, the survival rate falls to 2%. But because of more recent treatments, these rates will eventually rise.
Note: Bile duct cancer is called cholangiocarcinoma. It’s a rare condition that affects mostly older folks. If you have specific viruses or persistent inflammation in your bile ducts or liver, you are more likely to develop cholangiocarcinoma. The typical course of treatment for this kind of cancer is a combination of surgery, radiation therapy, and/or chemotherapy.
| Homepage | Click Here |