Bile Duct Cancer

About

Dr. Deepak Chhabra

Surgical Oncologist

MS. (Bom), DNB, M.R.C.S. (Edin. UK)

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  1. What is cancer
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The world knows this man as a consultant oncosurgeon with vast experience in Gastrointestinal and Hepatobiliary-Pancreatic cancers,who specializes in stapled anastomosis for bowel cancers and sphincter saving resections for rectal cancers. In short, he is the person to be contacted for all kinds of cancer related cases.

Richa AgrawalPancreatic Cancer

Thank you Dr Deepak for all you have done for mum, for putting the smile back of her face. I wish you success in all your future endeavors and know that you will continue to touch peoples lives and help them be healthy - the way you have done for my mum….. and me.

Nicola MathiasStomach Cancer

For 67years I've led a very healthy life and thank the "Almighty" for it. However as I said all good things come to an end some time and so be it. Out of the blues was detected the dreadful illness "C" which had shown no signs or symptoms whatsoever..... Time had now come for me to choose a very efficient Oncologist for my treatment.

Patient

My mother was diagnosed of colon cancer, and I was recommended to see Dr Deepak Chhabra for consultation.
The first impression of Dr Chhabra was… he is so young! But after consulting him we realized his level of experience and there was a sense of confidence he spilt over us.We knew we could trust him.....

Neelu GroverColon Cancer
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The bile ducts

The bile duct is a thin tube, about 4 to 5 inches long, that reaches from the liver to the small intestine. The major function of the bile duct is to move a fluid called bile from the liver and gallbladder to the small intestine, where it helps digest the fats in foods.

Different parts of the bile duct system have different names. In the liver, it begins as many tiny tubes (ductules) where bile collects from the liver cells. The ductules come together to form small ducts, which then merge into larger ducts and eventually the left and right hepatic ducts. The ducts within the liver are called intrahepatic bile ducts. These ducts exit from the liver and join to form the common hepatic duct (at the hilum of liver). About one third of the way along the length of the bile duct, the gallbladder (a small organ that stores bile) attaches by a small duct called the cystic duct. The combined duct is called the common bile duct. The common bile duct passes through part of the pancreas before it empties into the first part of the small intestine (the duodenum), next to where the pancreatic duct also enters the small intestine.

About

Types of bile duct cancers

Bile duct cancers are commonly called CHOLANGIOCARCINOMAs.

Cancers can develop in any part of the bile duct and, based on their location (see picture below), are divided into 4 groups:

  • Intrahepatic bile duct cancers
  • Hilar bile duct cancers
  • Mid bile duct cancers
  • Distal bile duct cancers

Intrahepatic bile duct cancers: These cancers develop in the smaller bile duct branches inside the liver. They can sometimes be confused with cancers that start in the liver cells, which are called hepatocellular carcinomas, and are often treated the same way. Only about 1 out of 10 bile duct cancers are intrahepatic.

Hilar bile duct cancers: These cancers develop at the hilum – where the hepatic ducts have joined and are just leaving the liver. They are also called “Klatskin tumors”. These are the most common type of bile duct cancer.

Mid Bile duct cancers: These cancers are rare and are found at the mid level of bile duct. More often than not these cancers are a part of cancers of the Gallbladder involving the bile duct.

Distal bile duct cancers: These bile duct cancers are found further down the bile duct, closer to the small intestine. Because these bile ducts are outside of the liver, these cancers are also known as extrahepatic bile duct cancers

More than 95% of bile duct cancers are of the adenocarcinoma type. Adenocarcinomas are cancers of glandular cells that can develop in several organs of the body. Bile duct adenocarcinomas develop from the mucus glands that line the inside of the duct. Cholangiocarcinoma is another name for a bile duct adenocarcinoma.

Causes

The exact causes of bile duct cancers is not know. But some factors can increase your risk of developing them.

A risk factor is anything that affects your chance of getting a disease like cancer. Different cancers have different risk factors. For example, exposing skin to strong sunlight is a risk factor for skin cancer. Smoking is a risk factor for cancers of the lung, larynx (voice box), colon, bladder, kidney, and many other organs.

But risk factors don’t tell us everything. Having a risk factor, or even several risk factors, does not necessarily mean that a person will get the disease. And many people who get the disease may not have had any known risk factors.

Researchers have found several risk factors that make a person more likely to develop bile duct cancer.

Certain diseases of the liver or bile ducts: People who have chronic (long-standing) inflammation of the bile duct have an increased risk of developing bile duct cancer. Several conditions of the liver or bile duct may cause this.

  • Primary sclerosing cholangitis is a condition in which inflammation of the bile duct (cholangitis) leads to the formation of scar tissue (sclerosis) and an increased risk of bile duct cancer. The cause of the inflammation is not usually known.
  • Ulcerative colitis is a condition that results in inflammation of the large intestine. Some people with ulcerative colitis also develop inflammation in the bile duct, which puts them at increased risk for bile duct cancer.
  • Bile duct stones, which are similar to, but much smaller than gallstones, can also cause inflammation that increases the risk of bile duct cancer.
  • Choledochal cysts are bile-filled sacs that are connected to the bile duct. (Choledochal means having to do with the common bile duct.) The cells lining the sac often have areas of pre-cancerous changes, which increase a person’s risk for developing bile duct cancer.
  • Other abnormalities of the bile ducts: Some people have abnormalities where the bile duct and pancreatic duct normally meet that allow digestive juices from the pancreas to reflux (flow back “upstream”) into the bile ducts. This backward flow also prevents the bile from being emptied through the bile ducts as quickly as normal. These people are at higher risk of bile duct cancer.
  • Other rare diseases of the liver and bile duct that may increase the risk of developing bile duct cancer includepolycystic liver disease and Caroli syndrome (a dilation of the intrahepatic bile ducts that is present at birth).

Liver fluke infections: In some Asian countries, infection by liver flukes (which are tiny parasite worms) can occur by eating poorly cooked fish. In humans, these flukes live in the bile ducts and can cause bile duct cancer. There are several types of liver flukes. The ones most closely related to bile duct cancer risk are called Clonorchis sinensis and Opisthorchis viverrini.

Obesity: Being overweight or obese can increase the risk of developing cancers of the gallbladder and bile ducts. This may be because obesity increases the risk of gallstones and bile duct stones.

Symptoms

Signs and symptoms may not be present until the later stages of bile duct cancer, but in some cases they may lead to an early diagnosis. If you go to your doctor when you first notice symptoms, your cancer might be diagnosed at an early stage, when it is most treatable.

When bile duct cancer does cause symptoms, it is usually because the bile duct is blocked.

Jaundice: Jaundice is yellowing of the skin and eyes that is caused by the build-up of a substance called bilirubin. This is the most common symptom of bile duct cancer. Normally, bile is made by the liver and released into the intestine. Jaundice occurs when the liver cannot get rid of bile, which contains bilirubin. As a result, bilirubin “backs up” into the bloodstream and settles into tissues. Bilirubin is greenish yellow and colours all the body tissues, including the skin and the white part of the eyes, making people with this condition begin to look yellow.

It is important to realize that most cases of jaundice are not caused by cancer. It is more often due to hepatitis (inflammation of the liver) or a gallstone that has traveled to the bile duct. But whenever jaundice occurs, a doctor should be seen right away.

Itching: Excess bilirubin in the blood can also reach the skin, which can cause itching. Most people with bile duct cancer notice itching.

Light coloured stools/dark urine: Bilirubin contributes to the brown colour of bowel movements, so if its flow into the intestine is blocked the colour of a person’s stool might be lighter. When bilirubin levels in the blood get high, it can also come out in the urine and turn it dark.

Abdominal pain: Early bile duct cancers usually do not cause pain, but more advanced cancers may lead to abdominal pain, especially below the ribs on the right side.

Loss of appetite/weight loss: People with bile duct cancer may not feel hungry and may lose weight (without dieting).

Fever: Some people with bile duct cancer develop fevers.

Nausea/vomiting: This is not a common symptom of bile duct cancer, but it may be seen in people who develop an infection (cholangitis) as a result of bile duct blockage. It is often seen along with a fever.

These are symptoms and signs of bile duct cancer, but it is important to remember that they are more likely to be caused by non-cancerous diseases. For example, people with gallstones may have many of these same symptoms. There are many causes of abdominal pain that are far more common than bile duct cancer. And hepatitis (inflamed liver most often caused by infection with a virus) is a much more common cause of jaundice. Still, if you have any of these problems, it’s important to see your doctor right away so the cause can be found and treated, if needed.

Tests

What tests are carried out to diagnose Bile duct cancers?

This section lists common tests that may be performed and it is not necessary for all the tests to be performed for a patient. Your doctor will select the tests that will assist him /her to have the maximum information about the tumor/ disease.

Blood Tests

Tests of liver and gallbladder function: The doctor may order lab tests to find out how much bilirubin is in the blood. Bilirubin is the chemical that causes jaundice. Problems in the bile duct, gallbladder, or liver may cause too much bilirubin to remain in the blood. A high bilirubin count tells the doctor that there may be problems with the bile duct, gallbladder, or liver.

The doctor may also order tests for other substances in your blood, such as albumin, alkaline phosphatase, AST, ALT, and GGT. These are sometimes called liver enzymes or liver function tests. They can also give an indication of bile duct, gallbladder, or liver disease. Higher levels of these substances may point to blockage of the bile duct, but they cannot show if it is due to cancer or some other reason.

Tumor markers

Tumor markers are substances made by cancer cells that can sometimes be found in the blood. People with bile duct cancer may have high blood levels of the carcinoembryonic antigen (CEA) and CA 19-9 tumor markers. High amounts of these substances often mean that cancer is present, but the high levels can be caused by problems other than bile duct cancers. Also, not all bile duct cancers make these tumor markers, so low or normal levels do not mean there is no cancer.

Ultrasound

Ultrasound (US or ultrasonography) is often the first imaging test done in people who have symptoms such as jaundice or pain in the right upper part of their abdomen. On the basis of results of this ultrasound the doctor may decide for the next best investigation.

Computerised tomography (CT) scan

A CT scan is a type of x-ray that gives a picture of organs and other structures (including any tumours) in your body. It is used to see more details of a cancer and its relation to the surrounding organs in your body. It also gives information related to cancer spread into the lymph nodes, liver or lungs.

Magnetic resonance imaging (MRI)

This test is like a CT scan, but it uses magnetism instead of x-rays to build up pictures of the organs in your abdomen. Like a CT scan, MRI is painless and the magnetism is harmless. MRI scan may be used to see the extent of blockage of bile duct and in case the patient is allergic to contrast dye injection used for CT scans. MRI scans provide a great deal of detail and can be very helpful in looking at the bile ducts and nearby organs. Sometimes they can help tell a benign tumor from a malignant one. Special types of MRI scans called MR cholangiopancreatography (MRCP) are used in people who may have bile duct cancer.

Endoscopic ultrasound scan (EUS)

This involves a special endoscope equipped with an ultrasound probe and a small needle at the end. The scope is placed through the mouth into the oesophagus (food pipe) and the first portion of the small intestine for more detailed information about the local spread of the tumor. EUS also allows the physician to get a tissue sample (biopsy) of the tumor.

Endoscopy (ERCP)

In this test, a thin, flexible ‘telescope’ called an endoscope is put into your mouth then passed down your throat into your digestive system so that the doctor can examine you inside. The procedure is called ERCP, or endoscopic retrograde cholangio-pancreatography. Through the endoscope, the doctor can inject a liquid directly into the pancreatic duct and bile duct, allowing images of these organs to appear on x-ray pictures. The test can show blockages and inflammation in these ducts, and allow the doctor to judge whether these are due to cancer or other problems. The doctor is also able to take a tissue or fluid sample through the endoscope, to help with the diagnosis. It can also be used to place a stent (a small tube) into a duct to help keep it open.

Positron emission tomography (PET) scan

This test may be used to build up more information after an MRI or CT scan. PET-CT scan is not necessary for all patients. Your doctor will decide if you need to undergo this scan.

This test is combined with a CT scan by injecting a radioactive material in the body to highlight all areas where the tumor has or can spread.

Percutaneous Transhepatic Cholangiography (PTC)

This is another way for your doctor to look at your bile duct. In this procedure, the doctor places a thin, hollow needle through the skin and into a bile duct within the liver. (A local anesthetic is used to numb the area before inserting the needle.) A contrast dye is then injected through the needle, and x-rays are taken as it passes through the bile ducts. Like ERCP, this approach can also be used to take samples of fluid or tissues or to place stents (small, hollow tubes) in the bile duct to help keep it open.

Biopsy

Imaging tests can suggest that a bile duct cancer is likely to be present, but in many cases a sample of bile duct cells or tissue is removed (biopsied) and looked at under a microscope to be sure of the diagnosis.

But a biopsy may not always be done before surgery for a possible bile duct cancer. If imaging tests (ultrasound, CT or MRI scans, cholangiography, etc.) suggest there is a tumor in the bile duct, the doctor may decide to proceed directly to surgery and to treat it as a bile duct cancer.

Treatment

What is the treatment of Bile duct cancer?

Many years of treating cancer patients and testing treatments in clinical trials has helped doctors know what is likely to work for a particular type and stage of cancer. You will be advised on the best treatment for your cancer. This will depend on the type of cancer you have, where it is and how far it has spread and your general health.

Surgery

Surgery For Bile duct cancer

If the tumor is resectable, surgery is usually the main type of treatment for bile duct cancer, as it offers the only reasonable chance to cure the cancer. Radiation therapy and/or chemotherapy may be added after surgery in some cases, or they may be used instead of surgery if the cancer cannot be entirely removed.

Though cancer surgeries are extensive and take longer hours; with advanced medical care, surgeries have become safer and better equipments are now available to perform surgeries. Improved techniques of anaesthesia and post-operative monitoring have significantly contributed to overall safety of surgical procedures for cancers.

There are 2 general types of surgical treatment for bile duct cancer — potentially curative surgery and palliative surgery.

  • Potentially curative surgery is used when imaging tests indicate a good chance that the surgeon will be able to remove all of the cancer. Doctors may use the term resectable to describe cancers they believe can be removed completely (by potentially curative surgery) and unresectable to describe those they think have spread too far or are in too difficult a place to be entirely removed by surgery. Unfortunately, only a small portion of bile duct cancers are resectable at the time they are first found.
  • Palliative surgery may be performed to relieve symptoms or treat (or even prevent) complications, such as blockage of the bile ducts. This type of surgery is performed when the tumor is too widespread to be completely removed. Palliative surgery is not expected to cure the cancer, but it can sometimes help someone feel better and sometimes can even help them live longer.

SURGERY FOR RESECTABLE CANCERS

For resectable cancers, the type of operation depends on the location of the cancer.

  • Intrahepatic bile duct cancer: These cancers have started in bile ducts within the liver. To treat these cancers, the surgeon cuts out the part of the liver containing the cancer. Removing part of the liver is called a partial hepatectomy. Sometimes this means that a whole lobe of the liver must be removed. This is called hepatic lobectomy. As much as 70 percent of the liver may be removed if the remaining liver is healthy. The remaining healthy liver can then take care of the body functions. Also, the liver can re-grow some of the missing part. The new cells grow over several weeks.
  • Hilar bile duct cancer (Hilar Cholangiocarcinoma): These cancers begin where the branches of the bile duct first exit the liver. Surgery for these cancers requires great skill, as the operation is quite extensive. Usually part of the liver must be removed along with the entire bile duct, gallbladder and nearby lymph nodes. Then the surgeon must connect the remaining ducts to the small intestine. (It becomes important for the patient to understand that a major portion of the liver needs to be removed as the bile duct system is connected to that lobe of the liver. So for the patient this is an extensive liver surgery).
  • Distal bile duct cancer: These cancers are further down the bile duct near the pancreas and small intestine. Along with the bile duct and nearby lymph nodes, in most cases the surgeon must remove part of the pancreas and small intestine. The procedure is same as that performed for a pancreatic cancer (Whipple’s operation). See treatment of pancreatic cancer.

SURGERY FOR UNRESECTABLE CANCERS

Liver transplant: The surgeon may sometimes remove the whole liver. If the whole liver is removed, it is replaced with healthy liver tissue from a donor. Such type of surgery is called Liver Transplant. To have a liver transplant you need a liver from a donor. It can sometimes take months to find a donated liver that closely matches yours. During this delay, the cancer may continue to grow and you may need to have other treatment to try to control it. Moreover there are strict criteria as to which patient can undergo liver transplant in the setting of cancers. Also after a liver transplant, you have to take drugs to stop your body rejecting the donated liver. These drugs damp down the activity of your immune system and reduce its ability to control the cancer.

Cytoreduction (Debulking) Surgery

Palliative therapy is treatment that is given to help control or reduce symptoms caused by advanced cancer. It is not meant to be a curative treatment. If the cancer has spread too far to be completely removed by surgery, doctors may focus on palliative operations, palliative radiation, and other palliative therapies. Because these cancers tend to advance quickly, doctors try to use palliative therapies that are less likely to affect a person’s quality of life, when possible.

  • Palliative surgery: In some cases a doctor may think that a cancer is resectable based on the information available (imaging tests, laparoscopy, etc.), but once the surgery is started it becomes clear that the cancer is too advanced to be removed completely. At this point the surgeon may do a biliary bypass to allow the bile to flow into the intestines to reduce symptoms such as jaundice or itching.
  • In this palliative procedure, the surgeon creates a bypass around the tumor blocking the bile duct by connecting part of the bile duct before the blockage with a part of the intestine.
  • If a bypass can’t be done, the surgeon may simply ask for a plastic or expandable metal tube (called a stent) to be placed through the bile duct to keep it open.
  • Biliary stent or biliary catheter: If cancer is blocking the bile duct, the doctor may insert a small tube (called a stent or catheter) into the duct to help keep it open. This may be done as part of a cholangiography procedure such as PTC or ERCP.
  • A stent opens the duct to allow the bile to drain into the small intestine, while a catheter drains into a bag outside the body that can be emptied when needed. The stent or catheter may need to be replaced every few months if it becomes clogged and to reduce the risk of infection and gallbladder inflammation.

For bile duct cancers that are resectable, chemotherapy may be used after surgery (often along with radiation therapy) to try to lower the risk that the cancer will return. This is known as adjuvant chemo. Some doctors may use it before surgery for borderline resectable cancers to try to improve the odds that surgery will be successful. This is called neoadjuvant treatment. Chemotherapy may also be used (with or without radiation therapy) for more advanced cancers. By and large there are very few responses to chemotherapy for Bile duct cancers.

Radiation therapy is treatment with high-energy rays or particles that destroy cancer cells. There are different kinds of radiation therapy.

External beam radiation therapy (EBRT): This type of radiation therapy uses x-rays from a machine outside the patient’s body to kill cancer cells. It is the most common form of radiation therapy for bile duct cancer. The treatment is much like getting an x-ray, but the radiation is more intense. The procedure itself is painless. Before your treatments start, the radiation team will take careful measurements to determine the correct angles for aiming the radiation beams and the proper dose of radiation. Each treatment lasts only a few minutes, but the setup time — getting you into place for treatment — usually takes longer. Most often, radiation treatments are given 5 days a week for several weeks.

Three-dimensional conformal radiation therapy (3D-CRT) uses special computers to precisely map the location of the tumor(s). Radiation beams are shaped and aimed at the tumor(s) from several directions, which makes it less likely to damage normal tissues. Most doctors now recommend using 3D-CRT when it is available.

Brachytherapy (internal radiation therapy)

This type of treatment uses small pellets of radioactive material placed next to or directly into the cancer. The radiation travels a very short distance, so it affects the cancer without causing much harm to nearby healthy body tissues. Brachytherapy is sometimes used in treating people with bile duct cancer by placing the pellets in a tube, which is inserted into the bile duct for a short time.