What is Cholangiocarcinoma?

Cholangiocarcinoma is a cancerous (malignant) growth in one of the ducts that carries bile from the liver to the small intestine.

What are the Causes?

Cancerous tumors of the bile ducts are usually slow-growing and do not spread quickly. However, many of these tumors are already quite advanced by the time they are found.

A cholangiocarcinoma may start anywhere along the bile ducts. These tumors block off the bile ducts. Both men and women are affected. Most patients are older than 65.

What are the risks linked to this condition?

Bile duct (choledochal) cysts
Chronic biliary and liver inflammation
History of infection with the parasitic worm, liver flukes
Primary sclerosing cholangitis
Ulcerative colitis

Cholangiocarcinoma is rare disease. It occurs in approximately 2 out of 100,000 people.

What are the symptoms?

Symptoms may include any of the following:

Chills
Clay-colored stools
Fever
Itching
Loss of appetite
Pain in the upper right abdomen that may radiate to the back
Weight loss
Yellowing of the skin (jaundice)

Exams and Tests:

Your health care provider will perform a physical exam. Tests will be done to check for a tumor or blockage in the bile duct. These may include:

CT scan of abdomen
Endoscopic ultrasound
Cytology of samples from the bile duct
Endoscopic retrograde cholangiopancreatography (ERCP)
Magnetic resonance cholangiopancreatography (MRCP)
Percutaneous transhepatic cholangiogram (PTCA)
Cholangioscopy
Confocal Endomicroscopye
Blood tests that may be done include:
Liver function tests (especially alkaline phosphatase or bilirubin levels)

Treatment:

The goal is to treat the cancer and the blockage it causes. When possible, surgery to remove the tumor is the treatment of choice and may result in a cure. If the tumor is large, the entire liver may need to be removed and a liver transplant will be needed. Often the cancer has already spread locally or to another area of the body by the time it is diagnosed. As a result, surgery to cure the cancer is not possible.

Chemotherapy or radiation may be given after surgery to decrease the risk of the cancer returning. But the benefit of this treatment is not certain.

Endoscopic therapy with stent placement can temporarily relieve blockages in the biliary ducts and relieve jaundice in patients when the tumor cannot be removed. Laser therapy combined with light-activated chemotherapy medications is another treatment option for those with blockages of the bile duct (photodynamic therapy- PDT).

Outlook:

Completely removing the tumor allows approximately 1 in 5 patients to survive for at least 5 years, with the possibility of a complete cure.

If the tumor cannot be completely removed, a cure is generally not possible. With treatment, about half of these patients live a year, and about half live longer, but rarely beyond 5 years.

Possible Complications:

Infection
Liver failure
Spread ( metastisis) of tumor to other organs

New techniques to diagnose and treat Cholangiocarcinoma:

Cholangiocarcinoma is difficult to diagnose because it has a lot of scar tissue and the cancer cells are difficult to find in the biopsy or scrapings of the bile duct. The new methods can assist if the diagnosis is not be confirmed by usual methods and lack of diagnosis prevents further treatment.

Visual access to the bile duct using a cholangioscope (a camera in the bile duct) and placement of a microscope (Confocal microscope) can help improve diagnosis.

When to Contact a Medical Professional:

Call your health care provider if you have jaundice or other symptoms of cholangiocarcinoma.