A rare type of cancer, cholangiocarcinoma affects the bile ducts, thin tubes that transport bile from the liver and gallbladder to the small intestine. As part of the bile duct system, fats are digested and toxins are removed. Cholangiocarcinoma occurs in various parts of the bile ducts, either inside or outside the liver.
The exact cause of cholangiocarcinoma is not known, but some factors may increase the risk of developing this cancer. These include:
Chronic inflammation and scarring of the bile ducts, such as in primary sclerosing cholangitis (PSC), a condition that affects some people with inflammatory bowel disease (IBD).
Infection with parasites that live in the bile ducts, such as liver flukes, which are common in some parts of Asia.
Congenital abnormalities of the bile ducts, such as choledochal cysts, which are dilated and irregular bile ducts that are present at birth.
Cirrhosis of the liver, which is a condition that causes scarring and damage to the liver due to various causes, such as hepatitis, alcohol abuse, or fatty liver disease.
Hepatitis B or C, which are viral infections that can cause inflammation and damage to the liver and bile ducts.
Exposure to certain chemicals or toxins, such as asbestos, dioxins, or thorium dioxide, which are used in some industries or medical procedures.
Smoking, obesity, diabetes, and alcohol abuse, which are lifestyle factors that can affect the health of the liver and bile ducts.
Cholangiocarcinoma often does not cause any symptoms until it has grown large or spread to other organs. Some of the possible symptoms include:
Jaundice, which is a yellowing of the skin and eyes due to the buildup of bilirubin, a pigment in bile, in the blood.
Itching, which is caused by the irritation of the skin by bile salts, which are substances in bile that help digest fats.
Abdominal pain, which is usually felt on the right side, just below the ribs, where the liver and gallbladder are located.
Weight loss, which is due to the reduced appetite and malabsorption of nutrients caused by the blockage of bile flow.
Fever, night sweats, and chills, which are signs of infection or inflammation in the bile ducts or liver.
Dark urine and pale stools, which are due to the reduced amount of bile in the urine and stools.
To diagnose cholangiocarcinoma, the doctor may perform several tests, such as:
Blood tests, which can check the levels of liver enzymes, bilirubin, and tumor markers, which are substances produced by some cancers.
Using imaging tests, such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), or positron emission tomography (PET), the location and size of the tumor can be determined.
As part of endoscopic retrograde cholangiopancreatography (ERCP), a flexible tube with a camera and light (endoscope) is used to examine the bile ducts and pancreas. To make the bile ducts more visible on X-rays (cholangiograms), the doctor can inject dye into them, and a tissue sample (biopsy) can be taken for further analysis.
The location and size of the tumor can be determined using imaging tests such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), or positron emission tomography (PET).an X-ray and allows the doctor to take a biopsy if needed.
A biopsy, which involves removing a small piece of tissue from the tumor or lymph nodes nearby for microscopic examination, is the only way to confirm the diagnosis of cholangiocarcinoma and determine its type and grade.
Cholangiocarcinoma is treated in several ways, depending on the location, size, and stage of the tumor, the patient's overall health, and the availability of specialized care.
Surgery involves the removal of the tumor and some of the surrounding healthy tissue. Although this is the only treatment that can cure cholangiocarcinoma, it is only available to patients whose cancer has not spread beyond the bile ducts or liver. The type of surgery depends on the location of the tumor and may involve removing part of the liver (hepatectomy), part of the bile ducts (bile duct resection), or removing the entire gallbladder and nearby lymph nodes (cholecystectomy). The surgeon may also create a bypass to restore bile flow (biliary drainage) between the liver and the intestines.
To kill or stop cancer cells from growing, chemotherapy uses drugs. In addition to shrinking the tumor before surgery (neoadjuvant chemotherapy), adjuvant chemotherapy can reduce the risk of recurrence after surgery (adjuvant chemotherapy), palliative chemotherapy can be used to treat advanced or inoperable cholangiocarcinoma. In addition to giving chemotherapy intravenously, you can also give it orally. As a combination therapy, gemcitabine and cisplatin are commonly used for cholangiocarcinoma.
In radiation therapy, high-energy rays or particles are used to kill or damage cancer cells. The main treatment for advanced or inoperable cholangiocarcinoma (palliative radiation therapy) is radiation therapy given before or after surgery to reduce the tumor's size or kill any remaining cancer cells. A radiation treatment can be administered externally (external beam radiation therapy) or internally (brachytherapy). A machine directs radiation outside the body to the tumor, while brachytherapy uses a radioactive source placed inside or near the tumor.
Using drugs that target specific molecules or pathways involved in the growth and survival of cancer cells, targeted therapy involves killing them. For advanced or inoperable cholangiocarcinoma, targeted therapy can be combined with chemotherapy or radiation therapy. A number of targeted drugs are approved or under investigation for cholangiocarcinoma, including sorafenib, erlotinib, cetuximab, bevacizumab, and pembrolizumab.
The treatment of advanced or inoperable cholangiocarcinoma is immunotherapy, which uses drugs that stimulate the immune system to recognize and attack cancer cells. It can be used alone or in combination with other treatments. Nivolumab, ipilimumab, and atezolizumab are among the immunotherapy drugs approved or under investigation for cholangiocarcinoma.
The prevention of cholangiocarcinoma is not possible in most cases, as the cause is unknown and the risk factors are often unavoidable. However, some general measures that may reduce the risk or improve the outcome of cholangiocarcinoma include:
Avoiding exposure to chemicals or toxins that may damage the liver or bile ducts, such as asbestos, dioxins, or thorium dioxide.
Getting vaccinated against hepatitis B and C, which are viral infections that can cause chronic liver disease and increase the risk of cholangiocarcinoma.
Treating any underlying conditions that may affect the bile ducts, such as primary sclerosing cholangitis, choledochal cysts, or bile duct stones.
Adopting a healthy lifestyle that includes eating a balanced diet, maintaining a healthy weight, exercising regularly, limiting alcohol intake, and quitting smoking.
Getting regular check-ups and screening tests, especially for people who have a high risk of cholangiocarcinoma, such as those with primary sclerosing cholangitis, cirrhosis, or a family history of the disease. Screening tests may include blood tests, imaging tests, or endoscopic procedures to detect any abnormal changes in the bile ducts or liver.
Conclusion
This type of cancer affects the bile ducts and is rare and aggressive. It usually doesn't cause symptoms until it has spread to other organs, making it difficult to diagnose and treat. Depending on the stage and location of the tumor and the patient's overall health and preferences, surgery, chemotherapy, radiation therapy, targeted therapy, and immunotherapy are all possible treatment options. With early detection and treatment, cholangiocarcinoma usually has a poor prognosis, but it may improve. Despite the fact that cholangiocarcinoma cannot be prevented in most cases, there are some general measures to reduce or improve its risk, including avoiding toxins, getting vaccinated against hepatitis, treating any underlying conditions, adopting a healthy lifestyle, and getting regular check-ups and screenings.
