Bile duct cancer: symptoms, causes, treatment and prognosis

Medically reviewed: 12, December 2023

Read Time:14 Minute

Cancer in the bile duct: signs and stages of bile duct cancer, tumor in the bile duct

Bile duct cancer, or cholangiocarcinoma, is known as a rare type of malignant tumor. It is difficult to deal with because it is often diagnosed in late stages, is located in “inconvenient” places, from where it is very difficult to remove it surgically.

The liver has a system of tubes called bile ducts that resemble a tree. These tubes join to make smaller tubes, then two larger tubes, and finally the main liver tube.

Types of Bile Duct Cancer

Bile duct cancer is classified depending on where the malignant tumor is located and what histological structure it has (what types of cells it consists of).

Histological types

Most often, a malignant tumor develops from the cells lining the bile ducts. Such tumors are called adenocarcinomas. They are diagnosed in 90–95% of cases. Other histological types are much less common:

  • squamous cell carcinoma;
  • lymphomas – tumors of lymphoid tissue;
  • sarcomas are tumors of connective tissue.

Metastatic tumors are common in the liver. They are composed of cells characteristic of other types of cancer (since the primary tumor is in a different organ), and treatment tactics will differ from those indicated for hepatocellular carcinoma (liver cancer) or cholangiocarcinoma. Therefore, it is important to carry out differential diagnosis.

Classification depending on location

There are two types of tubes that carry bile. Some are in the liver and some are outside the liver.
There are two different types of cholangiocarcinomas, based on where the tumor grows in the body: intrahepatic and extrahepatic.

In turn, cancer of the extrahepatic bile ducts is divided into two types:

  1. Proximal (hilus) cholangiocarcinomas are located closer to the liver, before the formation of the common bile duct. This is the most common localization – it occurs in approximately 60% of cases. 20 out of 100 people with bile duct cancer have distal cholangiocarcinomas. They are near the duodenum. It’s hard to know where a tumor is in the bile ducts or the head of the pancreas before surgery.
  2. Malignant tumors of the intrahepatic bile ducts account for about 20% of cases. This type of cancer can be difficult to distinguish from liver tumors called hepatocellular carcinoma.

How does bile duct cancer develop?

From the moment of birth, altered cells that are not similar to normal ones constantly appear in the human body. Their appearance is caused by the influence of various chemicals, environmental factors, health problems, heredity and random failures. Almost all of them are detected and destroyed by the immune system, but some of them are hidden from the attention of our natural defenses or resist them. They survive, gradually multiply and create many copies of themselves.

Over time, a tumor forms from them, which not only pushes the surrounding tissues apart, but grows into them and into neighboring structures. What makes it extremely dangerous are the properties of the cells of which it consists:

The most important natural mechanism of “programmed” death does not work for them – apoptosis, thanks to which all body tissues are renewed in a timely manner. As a result of such a “breakdown,” they exist for an extremely long time and do not die at the end of their normal life cycle. They multiply quickly and uncontrollably.

Bile duct cancer

Unlike healthy ones, altered cells can function not in one, strictly defined tissue, but in different ones. They enter the blood and lymphatic vessels. The lymphatic system complements the cardiovascular system. Lymph circulates in it – a liquid that washes all the cells of the body, delivers necessary substances to them and takes away waste. In the lymph nodes, which act as “filters,” hazardous substances are neutralized and removed from the body. systems, spread to various areas of the body, become established in them and form metastases – new tumors.

These properties allow the tumor to affect important structures and disrupt the functioning of the entire body.

The liver does not just work as a “filter” of the body, removing substances dangerous to the body. This vital organ is involved in metabolism, creates and stores energy, and also produces bile, a substance necessary for the digestion of fats contained in food. This fluid is excreted through many ducts – small tubes that combine to form the common hepatic duct.

The gallbladder that accumulates it is connected to it by the cystic duct, which passes into the common bile duct – the common bile duct – passing through the pancreas and flowing into the first, initial part of the small intestine – the duodenum.

Reasons for the development of the bile duct cancer

Malignant tumors develop as a result of mutations that occur in cells. It is impossible to name the exact cause of these mutations in each specific case. There are risk factors – conditions that increase the likelihood of developing the disease. For example, it is known that men get sick more often than women, and the average age of people diagnosed with bile duct cancer is 65 years.

Inflammation

Primary sclerosing cholangitis is a rare sickness where the tubes that help bile move in the body get swollen and hard. We don’t know why it happens, but it can make cancer more likely to occur. Cancer develops in 5-10% of people with primary sclerosing cholangitis.

Anomalies in the development of the bile ducts

Some people from birth have protrusions on the wall of the common bile duct – common bile duct cysts. Sometimes their symptoms appear in early childhood, and in some cases the disease does not manifest itself for many years. In 30% of cases it leads to cancer.

Caroli syndrome is a rare hereditary pathology in which the intrahepatic bile ducts become dilated, and malignant tumors can develop in them.

Parasitic diseases

The risk of bile duct cancer increases with certain chronic parasitic diseases. For example, one of the risk factors is opisthorchiasis, a pathology caused by flatworms of the genus Opisthorchis. The pathogen is widespread in Southeast Asia, as well as in Russia, Ukraine, and Kazakhstan. Infection occurs by eating contaminated fish.

Other risk factors for bile duct cancer

The following factors increase the likelihood of developing bile duct cancer:

  • Cholelithiasis. Stones irritate the mucous membrane of the bile ducts, causing inflammation, which can result in malignant degeneration.
  • Chronic viral hepatitis B and C.
  • Cirrhosis. With this pathology, normal liver tissue dies and is replaced by liver tissue. Cirrhosis can develop as a result of viral hepatitis or excessive alcohol consumption.
  • Long-term swelling in the colon. People with ulcerative colitis are not likely to get bile duct cancer. This is because 10 out of 100 people with ulcerative colitis also have a condition called primary sclerosing cholangitis. This condition can lead to bile duct cancer.
  • Non-alcoholic fatty liver disease, excessive alcohol consumption, excess weight, diabetes mellitus, smoking, chronic pancreatitis, exposure to radon, asbestos, and dioxins are considered potential risk factors for the disease.

Symptoms of cancer of the bile ducts

In the early stages, the tumor often does not show any signs. Symptoms usually occur when the flow of bile is disrupted. In addition, the clinical picture depends on the location of the malignant tumor. With intrahepatic bile duct cancer, symptoms develop much later, often when the tumor has time to spread widely in the body.

The main manifestations of bile duct cancer:

  • Obstructive jaundice is a condition in which the outflow of bile is disrupted, a toxic breakdown product of hemoglobin, bilirubin, enters the blood, and because of this, the skin and mucous membranes turn yellow.
    Itchy skin also occurs due to increased levels of bilirubin in the blood.
  • Dark urine and light-colored stool – These symptoms occur because bile cannot pass into the intestines, and the body tries to reduce the level of bilirubin in the blood by excreting it through the kidneys.
  • Abdominal pain – localized in the upper right.
  • Decreased appetite.
  • Weight loss for no apparent reason.
  • Increased body temperature.
  • Nausea and vomiting.

Biliary tract cancer is a fairly rare disease. If you are bothered by the symptoms on this list, this does not mean that you have a malignant tumor. Most often, such manifestations indicate other diseases of the liver, gallbladder and pancreas. However, in each case it is necessary to exclude oncological pathologies. Visit your doctor and get examined.

Stages of bile duct cancer

Like all malignant tumors, cancer in the bile ducts is classified into stages in accordance with the generally accepted international TNM system. Category T indicates the size and spread of the primary tumor, N – involvement of regional lymph nodes in the process, M – the presence of distant metastases.

In a simplified form, the stages can be described as follows:

  1. Stage I: The tumor is only in the bile duct and has not spread to nearby tissue.
  2. Stages II–III: cancer cells spread to surrounding tissues and regional lymph nodes.
  3. Stage IV: There are distant metastases.

Classifications by stage for individual cancer locations (intrahepatic, extrahepatic bile ducts) have their own characteristics.

In addition, malignant tumors of the bile ducts are divided into resectable and unresectable. The former can be completely removed surgically, the latter have spread too much or are in a hard-to-reach place.

Pathways of metastasis in bile duct cancer

Since there are no symptoms for a long time, malignant neoplasms of the bile ducts are often diagnosed at late stages, when the tumor has time to grow into the surrounding tissues, spread to the lymph nodes, and give distant metastases. Most often, such tumors metastasize to the lungs, bones, and spread over the surface of the peritoneum – a thin film of connective tissue that lines the abdominal cavity and covers the internal organs.

Diagnostic methods of bile duct cancer

At the moment, there are no screening tests that could be used to massively examine people and identify malignant tumors of the biliary tract in the early stages. Most often, the diagnosis is made after the patient consults a doctor with complaints, and the tumor manages to grow into the surrounding tissues, spread to the lymph nodes and give distant metastases.

Ultrasound examination

Ultrasound is the most accessible and simple diagnostic method with which you can detect malignant neoplasms in the abdominal organs. If a bile duct tumor is suspected, two different types of ultrasound diagnostics can be performed:

  • Transabdominal ultrasound is a procedure that is familiar to many people. The study is carried out with a special sensor through the abdominal wall.
  • EndoUS is a procedure performed during an endoscopy. A miniature ultrasound probe is located at the end of the endoscope, which the doctor inserts into the duodenum. This helps to bring the sensor as close as possible to the tumor and better assess its position, size, and degree of spread to neighboring tissues.

Cholangiography

Cholangiography is an X-ray examination during which the bile ducts are filled with a special contrast solution, as a result of which their contours become clearly visible in the images. This diagnostic method is used to detect ducts blocked by a tumor and determine treatment tactics.

Two types of cholangiography are used:

  • Endoscopic retrograde cholangiopancreatography (ERCP). The doctor inserts an endoscope into the duodenum, then inserts a thin catheter into the bile duct and injects a contrast solution.
  • Percutaneous transhepatic cholangiography is a more invasive procedure and is performed when ERCP is not possible. The bile ducts are stained using a special needle inserted through the skin.

During cholangiography, a stent can be installed and a biopsy performed.

Biopsy

The most reliable way to diagnose a malignant tumor is to obtain a fragment of it and examine it under a microscope. If tumor cells are found in a tissue sample, it is possible to diagnose malignant neoplasms with almost one hundred percent probability. To do this, a biopsy is performed. It can be performed in different ways: during cholangiography or using a special needle that is inserted into the tumor under ultrasound or computed tomography guidance.

Tomography

Computed tomography and MRI, including the use of contrast, help to better assess the size, location and extent of tumor spread, determine whether surgical intervention is possible, and distinguish benign from malignant neoplasms.

Diagnostic laparoscopy

Diagnostic laparoscopy is a surgical procedure during which a puncture is made in the abdominal wall and a special instrument with a video camera is inserted through it – a laparoscope. Using it, the surgeon examines the abdominal cavity. If necessary, other instruments can be inserted through additional punctures.

Laparoscopy makes it possible to assess the stage of bile duct cancer, plan surgical treatment tactics, perform a biopsy, and detect peritoneal lesions – carcinomatosis.

Treatment of bile duct cancer

Therapeutic tactics for bile duct cancer depend on the size, location, stage of the tumor, preservation of liver function, general condition and concomitant diseases of the patient.

Surgical methods

Surgical removal of the tumor is the only treatment method that makes it possible to completely get rid of cancer. Radical surgery is only possible in cases where the tumor is diagnosed at an early stage and is small in size. Unfortunately, such situations are relatively rare.

The type of operation is determined by the localization of the lesion:

  • For neoplasms of the intrahepatic ducts, extensive (removal of an entire lobe) or economical resection of the liver is indicated. At the same time, the remaining part of the organ continues to perform its functions.
  • For neoplasms of extrahepatic ducts, including common bile duct tumors, the affected part of the ducts, as well as part of the liver, gall bladder, lymph nodes, part of the pancreas and duodenum are removed. Such operations are often very complex and can lead to serious complications. They should be performed by surgeons who specialize in such interventions and have experience in performing them.
  • If one small node is detected, but liver function is severely impaired, for example due to cirrhosis, transplantation is indicated. The problem is that it is often difficult to find a donor.

Palliative operations

If radical intervention is impossible and the patient has obstructive jaundice, palliative operations are performed: external and external-internal drainage, installation of stents in the bile ducts. Euroonco doctors have extensive experience in performing such interventions.

Radiotherapy of bile duct cancer

Radiation therapy may be prescribed before or after surgery to shrink the tumor and prevent recurrence. In later stages, it is used independently for the purpose of palliative treatment and to combat symptoms. Most often, irradiation is used from an external source. This procedure resembles radiography, but during it more powerful radiation is used.

In some cases, intrabiliary brachytherapy is used: a probe is inserted into the bile ducts for a short time, which serves as a source of radiation.

Chemotherapy of bile duct cancer

Chemotherapy, like radiation therapy, is prescribed before and after surgical treatment, independently in the later stages, and also supplements liver transplantation. Both systemic chemotherapy and special procedures are used:

  1. Intra-arterial chemotherapy is the injection of drug solutions into the hepatic artery. Because only a small portion of the chemotherapy drug enters the general bloodstream, higher doses can be used without the risk of serious side effects.
  2. Chemoembolization is a procedure during which, in addition to chemotherapy, microscopic embolic particles are injected into the vessel feeding the tumor. They block the lumen of small vessels and disrupt the flow of oxygen and nutrients to the tumor tissue.

Chemoradiation treatment

In some cases, chemotherapy is combined with radiation therapy. This results in more effective destruction of cancer cells, but chemoradiotherapy carries a higher risk of side effects.

Survival prognosis for bile duct cancer

The average five-year survival rate for all patients diagnosed with bile duct cancer is 8%. This low rate is due to the fact that tumors are often diagnosed at late stages, many of them have an “inconvenient” location, and because of this they are difficult to remove.

Forecast of five-year survival at different stages:

  • If the tumor is only in the bile ducts and hasn’t spread further, the chance of survival is 24%.
  • If the tumor has spread to nearby tissue or lymph nodes: 6%.
  • If cancer has spread far away: 1%.

The prognosis for bile duct tumors is not the most favorable. These types of cancer are very difficult to fight. But this does not mean that you need to give up.

Prevention of bile duct cancer

Many risk factors for the disease, such as age, congenital pathologies, and bile duct abnormalities, cannot be influenced. However, there are some ways to reduce the risks:

  • You need to maintain a healthy weight throughout your life, and physical activity and proper nutrition will help with this.
  • Stop smoking and drinking alcohol.
  • Vaccination protects against viral hepatitis B.
  • Avoid promiscuous sex, use condoms – this will help protect against infection with viral hepatitis.
  • If you have been diagnosed with hepatitis B or C, you should see your doctor regularly and receive treatment.

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