Bile Duct Cancer (Cholangiocarcinoma)

Here are some facts about bile duct cancer (cholangiocarcinoma) you should know

Bile duct cancer develops in the cells that line the bile ducts, the liver's drainage system for bile. Bile is collected in bile ducts, which drains into the gallbladder and finally into the small intestine where it aids digestion. Bile duct cancer is also known as cholangiocarcinoma.

About 2,500 new cases of bile duct cancer are diagnosed in the United States each year. The bile drainage system may develop this type of cancer in three general locations:

• It affects the bile ducts located within the liver (intrahepatic)
• Located just outside the liver (extrahepatic or perihilar) where the bile ducts exit the liver
• Located near where the bile ducts enter the intestine (called the ampulla of Vater), far outside of the liver (distal extrahepatic).

The most common Bile duct cancers occur in the perihilar region of the liver and the least common ones occur inside the liver.

How is bile duct cancer caused and what are the risk factors?

Age is associated with an increased risk of bile duct cancer. A slow-growing cancer, bile duct cancer invades local structures, which is why it is often diagnosed late in the disease process when the bile ducts become blocked. The blockade prevents bile from draining into the gallbladder and intestine. The blockage may cause inflammation of the liver (hepatitis) or pancreas (pancreatitis), depending on where it occurs.

Patients who develop bile duct cancer have a very low risk of developing it. However, chronic inflammation of the bile ducts may increase the risk of this cancer. A primary sclerosing cholangitis (especially if it is associated with ulcerative colitis) can cause chronic inflammation in the liver, as well as chronic liver diseases such as hepatitis B, hepatitis C, chronic alcoholic hepatitis, and cirrhosis.

It is associated with an increased risk of liver infection when certain parasitic infections are found in the Far East.

Gallstones are not linked to bile duct cancer, but stones in the liver can increase the risk. The North American population does not commonly experience liver stones, but Asians do.

There are rare congenital diseases that increase the risk of bile duct cancer, such as Lynch II syndrome (hereditary nonpolyposis colorectal cancer associated with bile ducts and other tumours) and Caroli's syndrome (portal hypertension with hepatic fibrosis and bile duct cysts).

A Native American has a six-fold greater risk of developing bile duct cancer. The risk is also higher for Asian Americans. Similarly, bile duct cancer is more prevalent in Israel and Japan, but is extremely rare in North America.

What are the symptoms and signs of bile duct cancer?

Bile cannot normally drain from the liver, where it is produced, resulting in the initial symptoms of bile duct cancer. Hepatitis results. Cholangiocarcinoma symptoms include yellowing of the skin and eyes (jaundice), itching, abdominal pain, bloating, and weight loss. There may be a low-grade fever and darkening of the urine and stool.

Symptoms may not appear until tumors have grown in size and metastasized from their original site. Pain in the abdomen is usually a late symptom and may be associated with a tender, enlarged liver. It usually occurs in the right upper quadrant of the belly.

How do physicians diagnose bile duct cancer?

Cancer of the bile duct can be diagnosed based on history and physical examination. The only initial sign of yellow/orange skin and eyes is painless jaundice. The history may include review of alcohol use, drug use, or recent illnesses that may be associated with hepatitis, or liver inflammation. Some other cholangiocarcinoma symptoms include weight loss, loss of appetite, weakness, loss of energy, and easy bruising or bleeding (factors that clot the blood are made in the liver, so loss of liver function may lead to decreased clotting factors in the bloodstream).

Tenderness in the abdomen can be detected by a physical examination, especially in the right upper quadrant beneath the ribs (where the liver is situated). An enlarged liver can be palpated or felt on examination in about a quarter of patients with bile duct cancer. When the patient undergoes a general examination, he or she is usually jaundiced, with yellow-tinged skin. In the white portion (sclera) of the eye or under the tongue, this can be seen most easily.

Liver function is often assessed with blood tests. Hepatic enzymes (AST, ALT, GGT, and alkaline phosphatase), cell count, electrolytes, blood pressure, urea nitrogen, creatinine, and INR/PTT (international normalized ratio/partial thromboplastin time), as well as PT (prothrombin time).

A blood test cannot diagnose bile duct cancer specifically. A tissue sample is obtained by biopsy by a surgeon, gastroenterologist, or interventional radiologist, and then examined under a microscope by a pathologist.

The liver, gallbladder, bile ducts, and other surrounding organs can be evaluated using imaging. Tests such as ultrasound, CT scan, and MRI may be performed to detect a tumor and its location.

ERCP is a specialized test for examining the bile duct as it enters the duodenum. A gastroenterologist performs ERCP using a fiberoptic camera attached to a flexible viewing tube. After passing through the mouth, the tube is threaded through the stomach and into the first part of the small intestine where the common bile duct enters. This test is commonly used to examine the lining of the esophagus and stomach, but it can also be used to diagnose conditions affecting the bile ducts, such as cancers, gallstones, and abnormal narrowing of the bile duct. Through the tube, dye can be injected into the bile duct opening to outline the bile ducts and detect obstructions. To find cancer cells, biopsy or cell washing can be performed. A gastroenterologist may place a stent during the same procedure if a blockage is found to allow bile to drain.

Interventional radiologists may obtain tissue samples from the liver by threading a needle through the skin.

In order to help direct potential treatment, it is crucial to stage bile duct cancer once it has been diagnosed. TNM staging consists of three parts:

T stands for the primary tumor and the extent to which it has spread locally and invaded other tissues. The liver, gallbladder, pancreas, stomach, and intestine are all involved in bile duct tumors.

The lymph nodes that are involved are N. The more nodes involved and the farther the cancer is from the bile duct, the more severe it is.

The letter M stands for metastasis. Have other parts of the body been affected by the tumor?

Stages of cancer range from 0 to 4, where 0 indicates no tumor, 1 indicates local tumors without spreading to lymph nodes or other body parts, and 4 indicates significant local growth, lymph node involvement, and spread to other body parts.

Staging is important, as is the detection of tumor spread beyond the liver and bile duct, but often the critical staging questions can only be answered during surgery. The surgeon can decide whether to resect or remove the whole tumor during an operation. Resection can dramatically improve survival rates.

How is bile duct cancer treated?

Treatment for bile duct cancer depends on its location and whether it can be completely removed by surgery. People with this form of cancer tend to be older and may be unable to tolerate and recover from a significant operation. The decision regarding surgery needs to be tailored to the individual patient and their situation.

There are also other treatment options that are palliative, not curative, and designed to preserve quality of life. The treatment of bile duct cancer may include chemotherapy and radiation therapy.

Another way to shrink the tumor and control its symptoms is through photodynamic therapy.

If a tumor cannot be surgically removed, radioembolization might be an option. The radioembolization process involves injecting small quantities of radioactive material into the arteries that supply the tumor in order to impair its blood supply, thereby shrinking its size.

ERCP can be used to stent the bile duct, allowing bile to drain out of the gallbladder and liver into the intestine. Symptoms are usually controlled by this method, but the tumor is not treated.

An enlarging tumor may cause significant abdominal and back pain, making pain control a challenging issue. Local anesthetic blocks may be helpful in controlling pain.

All cancer treatments are tailored to the individual patient. To understand treatment options, such as cure versus palliative care or symptom management and quality of life, discussion between the patient, health-care professional, and family is essential. The patient's wishes are paramount.

Complications of bile duct cancer?

Infection of the bile drainage system or cholangitis can result from obstruction of the bile duct.

A bile duct cancer patient may develop cirrhosis. The tumor may obstruct the bile duct, which causes liver cell destruction and scarring. It is particularly true for patients suffering from primary sclerosing cholangitis. Several risk factors are listed for bile duct cancer, including cirrhosis and sclerosing cholangitis.

Other complications may arise as a result of the procedures used to diagnose and treat the cancer. This includes complications associated with surgery, chemotherapy, and radiation therapy.

When does bile duct cancer have a good prognosis? When does bile duct cancer have a good prognosis?

The prognosis of a patient after being diagnosed with bile duct cancer depends on a number of factors, including where the tumor is, whether it has spread, and the general health of the patient. According to specific aspects of the tumor's shape and cell type, patients have a better prognosis when the tumor is located farther away from the liver hilum. Tumors that invade adjacent tissues, have lymph nodes involved, or have spread to distant sites in the body have a worse prognosis.

bile duct cancer survival is 50% after one year, 20% after two years, 10% after three years, and virtually none after five years if left untreated.

Complete removal of the tumor can improve survival, but this depends mostly on its location and whether it has invaded other tissues.

Can bile duct cancer be prevented?

There are no specific prevention methods for bile duct cancer because its cause is unknown. The risk of this cancer may be decreased, however, if liver inflammation and cirrhosis are prevented. Modifying alcohol consumption, getting vaccinated against hepatitis B, and abstaining from risky behaviors may help prevent infection with hepatitis C.

The same is true for all diseases that tend to develop as we age. Living a healthy lifestyle may also prolong life. You should not smoke, eat a balanced diet, keep physically active, and maintain a healthy weight.

What are the statistics for bile duct cancer?

The United States sees approximately 2,500 new cases of bile duct cancer every year, or one case for every 100,000 people.

Approximately 40%-60% of patients with bile duct cancer located in the liver hilum undergo surgery to remove the tumor completely, with an average survival time of 24 months. For patients with a tumor in the same location but unable to be completely removed, the average survival time is 21 months.