Cholangio carcinoma

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Cholangio carcinoma is a rare and deadly kind of cancer that affects the bile ducts. The bile ducts are a series of tubes that carry bile from the liver to the gall bladder. They also transport it to the gut for the digestion of fats in the food. Cholangio carcinoma usually develops in the parts of the bile duct that lie outside the liver, and it rarely occurs in the parts of the bile duct that lie in the liver. Read More

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Cholangio carcinoma

Cholangio carcinoma is a rare and deadly kind of cancer that affects the bile ducts. The bile ducts are a series of tubes that carry bile from the liver to the gall bladder. They also transport it to the gut for the digestion of fats in the food. Cholangio carcinoma usually develops in the parts of the bile duct that lie outside the liver, and it rarely occurs in the parts of the bile duct that lie in the liver. Cholangio carcinoma can also be referred to as bile duct cancer, and while it can occur at any age, it usually happens in older adults (50 years and above). Most individuals receive a cholangiocarcinoma diagnosis after cancer has already spread outside the bile duct, which makes the prognosis of the disease to be very poor. However, recent advancement in therapy has begun to change that.

Cholangio carcinoma
Picture courtesy: Johns Hopkins Medicine

What are the types of cholangiocarcinoma?

types of cholangio carcinoma
Picture courtesy: Memorial Sloan Kettering Cancer Center

Cholangiocarcinoma is divided into types based on the locations or occurrences of cancer in the bile duct. The divisions are:

  • Intrahepatic cholangiocarcinoma – this is the type that occurs in the parts of the bile duct that lies within the liver. It is very rare and can sometimes be classified as a type of hepatic cancer.
  • Hilar cholangiocarcinoma – this is the type that occurs in the parts of the bile duct just outside the liver. It can also be referred to as perihilar cholangiocarcinoma.
  • Distal cholangiocarcinoma – this is the type that occurs in the parts of the bile ducts closest to the small intestines. This type can also be referred to as extrahepatic cholangiocarcinoma.

What causes cholangiocarcinoma?

It is not clear what exactly causes the cellular changes that cause cholangiocarcinoma. However, mutations form in the DNA of the cells of the bile duct. These mutations cause the cells to multiply in an unregulated manner and form a mass of cells which is referred to as a tumor. These cells can spread to neighboring healthy tissues or distant parts of the body.

Risk factors associated with cholangiocarcinoma

Some of the factors that can increase the risk of an individual developing cholangiocarcinoma include:

  • Older age
  • Smoking
  • Diabetes
  • Liver fluke
  • Chronic liver disease
  • Primary sclerosing cholangitis
  • Bile duct problems
  • Obesity
  • Exposure to toxins
  • Inflammatory bowel disease
  • Cirrhosis of the liver
  • Human immunodeficiency virus

What are the symptoms of cholangiocarcinoma?

symptoms of cholangio carcinoma
Picture courtesy: The Waiting Room – Karger Publishers

The symptoms experienced vary based on the location and the advancement of the disease, but some symptoms can include the following:

  • The skin and whites of the eyes turn yellowish
  • Dark urine
  • Intensely itching skin
  • Abdominal pain, which can extend to the back
  • Fever
  • Chills
  • Pale or light-colored stool
  • Nausea and vomiting
  • Unexplained weight loss
  • Fatigue

How is cholangiocarcinoma diagnosed?

A healthcare provider evaluates the symptoms, takes a detailed medical history, and conducts a physical examination of the individual. The confirmatory tests of cholangiocarcinoma may include one or more of the following tests:

  • Liver function tests – these are tests that examine the body for high levels of liver enzymes that indicate that the liver may be malfunctioning or that there is a bile duct blockage in the body.
  • Tumor marker tests – these tests examine the blood or urine for substances like proteins which could indicate the presence of cancer.
  • Abdominal ultrasound – this is an imaging test where ultrasound is used to visualize the abdominal organs.
  • Endoscopic retrograde cholangiopancreatography – This is where a catheter and an endoscope are used to examine the bile ducts; the endoscope is passed into the mouth to the small intestines. The procedure is done while the patient is sedated. The catheter delivers a contrast dye to outline the shape of the bile duct in an X-ray. In this procedure also, any blockages to the bile duct can be opened back up.
  • MRI – This is an imaging test that can be used to visualize the liver, bile ducts, gall bladder, pancreas, and pancreatic duct. A specialized test called magnetic resonance cholangiopancreatography (MRCP) is used.

When these imaging tests indicate the presence of cancer, the doctor will then perform a biopsy, where a tissue sample is taken from the patient and examined under a microscope to confirm the presence of cancer. As well as confirming the presence of cancer, a biopsy can also inform the healthcare provider of the stage of cancer. Staging is the process of classifying the spread or metastasis of cancer. This information also helps to plan the treatment of cancer. The staging of a bile duct cancer takes into consideration the size of the cancer and the spread of the tumor from the bile duct to other parts of the body, like blood vessels, lymph vessels and lymph nodes, nearby organs, and distant organs.

Treatment

The treatment plan for cholangio carcinoma is dependent on the location of the cancer and its spread. Surgical removal of the bile duct can be used to treat the disease if it is caught in the early stages. However, most cases of bile duct cancers, before they are discovered, have spread to other organs and tissues. This results in the use of a combination of therapies to treat the disease. The therapies include:

Surgery 

Bile duct removal through surgeries can be done with different techniques. Bile duct removal surgery is done when the cancer has not spread. A partial hepatectomy which is the removal of the bile duct and some parts of the liver is used when cancer has mildly spread to the liver. A Whipple procedure is a special procedure where the bile duct, gallbladder, part of the pancreas, stomach, and small intestines are removed when the cancer has significantly spread to other organs. A palliative procedure where some symptoms of the cancer, like blockages, are eased, can be done, and a stent may be placed in the bile duct to help drain and remove any blockages.

Radiation therapy 

This is where powerful beams of radiation are used to destroy tumors. Radiation may be done after surgery to eliminate any remaining cancer cells. It can also be done before surgery in order to shrink the tumors before surgically removing them.

Chemotherapy

This is where drugs are used to destroy cancer cells. This therapy can also be used to shrink the cancer cells before surgery is done to remove the tumor. In some cases where the cancer is too widespread for surgery, chemotherapy may be used to reduce the symptoms of cancer and prolong the life expectancy of the patient.

Targeted therapy 

These are treatments that target a specific part of the cancer cells. Targeted therapies can target specific proteins in the abnormal cancer cells and prevent them from carrying out their function, thereby disrupting the cellular process of that cell and keeping them from multiplying.

Immunotherapy

This therapy helps to boost the body’s immune system in order to help it fight the cancer. In bile duct cancer, some cancer cells contain a protein that prevents the immune cells from attacking the cancer. Immunotherapy disables those proteins, making them vulnerable to attack by the immune system.

Prevention

There is no exact way to prevent cholangiocarcinoma, but some of the risks can be reduced in the following ways:

  • Stopping alcohol abuse and smoking
  • Maintaining a healthy body weight
  • Getting a hepatitis b vaccination
  • Avoiding exposure to dangerous chemicals.

What is the outlook on individuals with cholangiocarcinoma?

When the tumor is removed completely, which is possible in the early stages of the disease, there is a chance of the individual being completely cured. Also, the prognosis is less morbid if the cancer has not spread to the liver. The prognosis is bad, however, when the cancer has spread out to many other organs, making the condition inoperable, reducing the five-year survival rate from around 15% to less than 2%.