Cholangiocarcinoma is a malignant tumor of the bile ducts within the liver (intrahepatic), or leading from the liver to the small intestine (extrahepatic). It is a rare tumor with a poor outcome for most patients.
Bile is the substance manufactured by the liver that aids in the digestion of food. Bile ducts are channels that carry the bile from the liver to the small intestine. Like the tributaries of a river, the small bile ducts in the liver converge into two large bile ducts called the left and right hepatic ducts. These exit the liver and join to form the common hepatic duct. The gallbladder, which concentrates and stores the bile, empties into the common hepatic bile duct to form the common bile duct. Finally, this network of bile ducts is called the biliary tract.
Bile duct cancer originates from the cells that line the inner surface of the bile ducts. A tumor may arise anywhere along the biliarytract, either within or outside of the liver. Bile duct tumors are typically slow-growing tumors that spread by local invasion of neighboring structures and by way of lymphatic channels. However, once this particular type of tumor starts to grow enough to cause symptoms, it is very fast growing and difficult to treat. The presence of bile duct stones within the intrahepatic bile ducts indicates a pre-cancer warning sign.
There are two known causes:
Hepatitis C is a blood-borne infectious, viral disease that is caused by a hepatotropic virus called Hepatitis C virus (HCV). The infection can cause liver inflammation (hepatitis) that is often asymptomatic, but ensuing chronic hepatitis can result later in cirrhosis (fibrotic scarring of the liver) and liver cancer.
Parasite (Opisthorchis viverrini-Southeast Asia liver fluke), found in raw fish and the water supply in Southeast Asia, which lodge in the bile duct of the liver and damage cells, and over time through a biological process, turns into cancer.
Opisthorchis viverrini is a trematode parasite that attacks the area of the bile duct. Opisthorchis viverrini infection predisposes for Cholangiocarcinoma, a cancer of the bile ducts of the liver.
I CANNOT STRESS ENOUGH HOW IMPORTANT IT IS TO HAVE
YOUR BILE DUCTS CHECKED BEFORE ANY SYMPTOMS OCCUR.
SIGNS & SYMPTOMS
Tumors produce symptoms by blocking the bile ducts.
- Gastrointestinal discomfort
- Clay colored stools
- Dark urine
- Jaundice, which is yellowing of the skin and eyes
- Abdominal pain that may extend to the back
- Loss of appetite
- Unexplained weight loss
Certain laboratory test of the blood may aid in the diagnosis. The most important one is the test for elevated bilirubin levels in the bloodstream. Levels of alkaline phosphatase and CA 19-9 may also be elevated.
Computed Tomography (CT) Scan - An x-ray that uses a computer to provide an image of the inside of the abdomen.
Magnetic Resonance Imaging (MRI) Scan - This test uses magnetic waves to create an image.
Ultrasound - This test uses high-frequency sound waves that echo off the body to create a picture.
Endoscopic Retrograde Cholangiopancreatography (ERCP) - During an ERCP, a flexible tube is inserted down the throat and into the stomach and small intestine. By injecting dye into the drainage tube of the pancreas, your doctor can see the area more clearly.
Endoscopic Ultrasound (EUS) - EUS involves passing a thin, flexible tube called an endoscope through the mouth or the anus to exam the lining and walls of the upper and lower gastrointestinal tract and nearby organs such as the pancreas and gall bladder. The endoscopic is equipped with a small ultrasound transducer that produces sound waves that create a viewable image of the digestive track. When combined with fine needle aspiration, EUS becomes a state-of-the-art, minimally invasive alternative to exploratory surgery to remove tissue samples from abdominal and other organs. It also may be used to determine the cause of symptoms such as abdominal pain, to evaluate growth to diagnose diseases of the pancreas, bile duct and gall bladder when other tests are inconclusive and to determine the extent of certain cancers of the digestive tract.
Percutaneous Transhepatic Cholangiography (PTC) - By injecting dye into the bile duct through a thin needle inserted into the liver, blockages can be seen on x-ray.
Bile Duct Biopsy and Fine Needle Aspiration - A tiny sample of the bile duct fluid or tissue is removed and examined under a microscope.
Surgery and radiation therapy are the two most common treatment:
Surgery - If the cancer is small and has not spread beyond the bile duct, your doctor may remove the whole bile duct and make a new duct by connecting the duct openings in the lever to the intestine. Lymph nodes also will be removed and examined under the microscope to see if they contain cancer. If the cancer has spread and cannot be removed, your doctor may perform surgery to relieve symptoms. After complete removal of the tumor, 30 to 40 percent of patients survive at least five years, with the possibility of being completely cured.
Radiation therapy is the use of high-energy x-rays to kill cancer cells and shrink tumors.
There are two main types of radiation therapy:
External-Beam Radiation Therapy - Radiation comes from a machine outside the body.
Internal Radiation Therapy - Materials that produce radiation, called radioisotopes, are put into the area where the cancer cells are found through thin plastic tubes.
There is a new radiation therapy which shows promise:
Intensity Modulated Radiation Therapy (IMRT) for the most advanced form of conformal radiotherapy. IMRT employs a powerful, advanced computer program that plans a precise dose of radiation in three dimensions based on individual tumor size, shape and location. Remarkably, it allows for higher radiation doses than traditional radiotherapy methods, while sparing more of the surrounding healthy tissue.
There are some therapies that are currently being studied in clinical trials for the treatment of Cholangiocarcinoma, including:
Chemotherapy - Uses of drugs to kill cancer cells.
Biological Therapy - Uses the body's immune system to fight cancer.
Photodynamic Therapy - Uses a specific type of light and photosensitizing agent to kill cancer.
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