Cholangio Carcinoma



INTRODUCTION
Cholangiocarcinoma is a cancer of the cells lining the bile ducts. This cancer is rare in the western world, but is more common in other parts of the world. It starts in the small glands that lie in the walls of the bile ducts and therefore, it is also called glandular cancer (or in more technical language, adenocarcinoma). If it arises in bile ducts that are within the body of the liver, it is called Intrahepatic. If it arises in the bile ducts that are outside the body of the liver, it is called extra-hepatic.

RISK FACTORS
People with congenital or hereditary defects of the bile ducts are at a higher risk of developing this cancer. The risk also increases if there is a longstanding inflammation of the bile ducts. An inflammatory condition of the bowel, called ulcerative colitis, also increases the risk. Infection with Fluke worms, which is not uncommon in some parts of the world such as Asia and Africa, is also known to be associated with an increased risk.

SIGNS AND SYMPTOMS
Due to the growth of cholangiocarcinoma, the flow of bile stops, therefore the bile spills over into the blood circulation and can cause yellowness of the eyes and skin because of its yellow colour. Bile is also excreted in the urine through kidneys, therefore the colour of urine darkens and also because bile is not entering into the bowel, the colour of stools becomes lighter. Bile also contains bile salts, which can accumulate in the skin and can cause itching, which can be quite distressing. The yellow colour of the skin or jaundice can be seen in other diseases as well, for example in gallstones, blocking the flow of bile. Some people can feel feverish or shivery. In case of jaundice, which progresses over a few weeks, medical advice should be sought.

DIAGNOSIS
Usually the patient will consult his GP, who would send him to a specialist in the hospital. The specialist will take a history of the illness and will do a clinical examination. After that usually the following tests are done:

Liver Function Tests or LFTs
Some liver or biliary enzymes can increase in amount due to the obstruction of bile flow. Their quantity can be easily measured in the blood.

Ultrasound Scan
This test is totally painless and the doctor usually asks you to lie down on a couch. A jelly like material is put on the skin of the tummy on top of the liver. A small instrument of the size of a microphone is put on the skin. This instrument omits ultrasound waves, which bounce back from the internal organs of the tummy. These sound waves are analyzed with the help of a computer and a picture of the internal organs can be developed and this can take about 15-20 minutes. 4-6 hours before the test, you will be asked to drink clear fluid. Milk or fizzy drinks are usually not allowed.
 
CT Scan
With the help of the x-rays usually of the horizontal slice images of the body are taken. These images can be seen on the screen. You will be asked to fast 4-6 hours before the test and just before the test a special drink is given so that the quality of the x-ray can be improved and just before the scan an intravenous injection is given to highlight the internal blood vessels. This test is totally painless and can take between 10-30 minutes. Usually the patient is advised to drink plenty of fluids after the test.

MRI Scan
This test is somewhat similar to the CT scanning and the difference is that instead of x-rays, magnetic waves are used. In this test also the patient is required to lie on the table from half an hour to one hour. There is a metallic capsule around the patient, which can cause claustrophobia in some patients. The machine can be quite noisy as well, for this reason usually earmuffs are provided. If you like, you can listen to the music of your choice and usually with the help of a microphone you can talk to the technician. During the test, one of your friends or relatives can stay in the room to support you.

ERCP
In addition to requiring images of the bile duct, if there is an obstruction in the flow of bile, it can be bypassed. A long flexible camera tube is passed into the stomach and the small bowel, after sedating the patient. When it reaches the first part of the small bowel (duodenum), the pancreatic and the bile ducts are seen to be opening in a small opening, an x-ray is injected into this opening, which facilitates acquiring x-ray images. If there is a blockage in the flow of bile, it can be diagnosed. If cancer is suspected, a brush is passed through the blockage and some tissue cells are removed, which can be examined under the microscope. To restore the flow of bile, a small tube (stent) can be passed through the obstruction so that bile starts draining again. With this procedure, the jaundice is reduced and the patient is relieved of the troublesome itching. Quite often the patient is asked to stay in the hospital overnight after this test. Antibiotics are given just before the test.

PTC
If ERCP is unsuccessful or impossible, this test is done. The patient is asked to fast 4-6 hours before the test and the skin is frozen with the help of a local anesthetic in the right lower part of the chest. With the help of a fine needle a thin wire is passed through the liver into the bile duct. A stent can be slipped over this wire so that the obstruction to the bile flow can be overcome. The other end of the stent is left in the small bowel to restore the flow of bile. Usually the patient is to asked to stay in the hospital after this test. Similarly a brush can be passed over the wire into the suspected area so that the cancer cells can be removed and examined under the microscope.

Biopsy
As mentioned above, a brush sample can be taken to diagnose cancer. Sometimes, when the size of the cancer is relatively big a needle can be passed through the skin to the cancer, under the guidance of a CT scan or ultrasound scan. A sample is acquired of the cancer, which is called a biopsy.
Laparoscopy
Sometimes to diagnose cancer, the patient is anesthetized and a very small tube is introduced into the abdomen through the skin. Sometimes, a cancer can be directly seen and a biopsy can be taken.

STAGING AND GRADING

Staging
The chances of cure from cancer is increased if its size is small and if it has not spread into other parts of the body and therefore the size and spread of cancer is very carefully identified and measured. This process is called staging and grading. A cancer usually spreads through the body by two means, either through the blood circulation or through another circulatory system called lymphatic system. Lymph is a very thin liquid, which contains white cells, which protect the body against infection, and the lymph vessels are connected together through many small glands, called lymph glands.
Stage 1a:
Cancer is confined to the bile duct.

Stage 1b:
Cancer has gone past the bile ducts, but has not invaded lymph glands or other organs.
Stage 2a:
Cancer has affected liver, pancreas or gallbladder or blood vessels nearby, but so far has not affected the lymph glands.
Stage 2b:
Cancer is affecting the neighboring lymph glands.
Stage 3:
Cancer is affecting large blood vessels, which carry blood to and from the liver or is affecting small bowel, colon or stomach. It is possible that the neighbouring lymph glands are also affected.
Stage 4:
Cancer has spread to the distant parts of the body, for example to the lungs. If after initial treatment the cancer comes back, it is called recurrent cancer.

Grading
Grading is dependent on the nature of the cancer cells as examined through the microscope. A low-grade cancer spreads slowly whereas a high-grade cancer is more likely to spread quickly, therefore it is thought to be more dangerous.

TREATMENT
The treatment for cancer depends on your general health, the location and size of cancer and also on the fact whether or not it has spread outside the bile ducts.

Surgery
If cancer has not spread outside the bile ducts it can be removed through surgery. Surgery is not always possible. The location of the cancer may be such that it is difficult to remove and cannot be fully taken out. The possibility of surgery is determined after doing the tests mentioned above. If it seems that surgery is possible, the patient will be sent to a specialist surgeon. The nature of the operation depends on the extent of cancer.

Removal of Bile Ducts
If the cancer is confined to within the bile ducts, this abnormal area is removed through the operation and the remaining healthy bile ducts are joined up to the small bowel so that the flow of bile can be restored.

Partial Liver Resection
If the cancer affects only a small area of the liver, this area can also be removed along with the affected bile ducts.

Whipple's Operation
If the cancer is a bit bigger and is affecting nearby organs, this operation is undertaken. Bile ducts, a part of the stomach, duodenum, a part of pancreas, the gallbladder and the neighbouring lymph glands are removed and the patient is admitted to the intensive care unit after the operation until he recovers.

Bypass Surgery
Bypass operations can be done in two ways:
1. If the flow of bile is obstructed and there is no other way to open it, the gallbladder or the bile duct is then joined up to the small bowel so that the bile flow is restored. This operation is only done in the situation when the above methods i.e. ERCP and PTC are not possible or are unsuccessful.
2. Another situation when a bypass operation is undertaken is when there is a blockage in the small bowel. In this situation usually the stomach is joined to the small bowel so that the food enters from the stomach into the small bowel bypassing the obstructed area.
Stent Insertion
As mentioned above, a small plastic or metal tube (stent) can be introduced into the bile duct so that the flow of bile can be restored (please see ERCP and PTC above). Quite often the stent will close after a few weeks or months, in which case another stent can be inserted. Sometimes a tube is introduced into the biliary system via PTC and its outer end is connected to a bag and this is only done when the internal drainage of bile is not possible.

Radiotherapy
Sometimes, radiotherapy is undertaken to destroy the cancer cells. Usually complete cure is not possible with this kind of treatment, however its speed of growth can be slowed down.

Chemotherapy
Sometimes, radiotherapy and chemotherapy (treatment with medication) are combined. It is uncertain whether chemotherapy is effective.

CLINICAL TRIALS
It is possible that your doctor may ask you about your willingness to participate in clinical trials. These are treatments which may be beneficial, but this remains to be determined. It is possible that they may be benefit cancer patients in future.

FEELINGS AND EMOTIONS
Feeling very worried and scared would be natural in any person who develops a cancer. This can progress to clinical depression. Counseling and psychiatric help may be needed in some cases and should be sought through your doctor.