Bile Duct Cancer (Cholangiocarcinoma)

Bile Duct Cancer (Cholangiocarcinoma)

The bile ducts are a series of thin tubes that go from the liver to the small intestine. Their major job is to move a fluid called bile from the liver and gallbladder into the small intestine, where it helps digest the fats in food. Different parts of the bile duct system have different names.

Bile duct cancer is also called cholangiocarcinoma. It is a rare disease that begins in the bile ducts.

People who have chronic (long-standing) inflammation of the bile ducts have an increased risk of developing bile duct cancer. Smaller stones that form in the bile ducts (bile duct stones) or pass into them from the gallbladder (gallstones) can lead to this type of chronic inflammation. Other inflammatory conditions can increase the risk of developing bile duct cancer:

Ulcerative Colitis

Ulcerative colitis is an inflammation of the large intestine. It is often associated with inflammation of the bile ducts, which is called primary sclerosing cholangitis. Ulcerative colitis can progress to cancer, particularly in people exposed to carcinogens, such as cigarette smoke.

Biliary Parasites

Although rarely seen in the United States, food- or water-borne parasites that live in the bile ducts are common in Asia and raise the risk of developing bile duct cancer.

Congenital Bile Duct Cysts (Choledochal Cysts)

These bile-filled sacs are connected to the common bile duct. Congenital bile duct cysts are typically diagnosed in childhood. The lining of these sacs often contains precancerous cells that increase the risk of developing cancer later in life.

Chronic Hepatitis C

This inflammatory disease is a risk factor for cancer of the intrahepatic bile ducts. Chronic (long-standing) hepatitis C is also the most common risk factor for liver cancer. Read more about liver cancer risk factors here.

Smoking

Studies have suggested that intrahepatic bile duct cancer is more common among heavy smokers.

Age

Bile duct cancer occurs most often in older people. The average age of diagnosis in the United States is 72.

Diabetes

Diabetes can slightly raise the risk for intrahepatic bile duct cancer.

Bile Duct Cancer (Cholangiocarcinoma) Signs & Symptoms

Bile duct cancer usually causes few symptoms until it reaches an advanced stage and has spread to other organs and tissues. However, people with this disease may experience the following symptoms or signs, often because a tumor is blocking a bile duct.

The following signs and symptoms may be caused by bile duct cancer or another condition. Check with your doctor if you experience any of them.

Jaundice

Jaundice is a condition is which the skin and the whites of the eyes become yellow, urine darkens, and the color of stool is lighter than normal. This can occur because a bile duct tumor has grown large enough to block the bile duct.

Very Itchy Skin

Intense itching can be caused by a buildup of bile salts and bilirubin that collects in the skin.

Loss of Appetite

Bile duct cancer may make it uncomfortable to eat or make you feel like you don’t want to eat at all.

Weight Loss

The loss of a significant amount of weight without trying can be a sign of many cancers, including bile duct cancer.

Fever

An increase in body temperature without any explanation can be an early sign of cancer, including bile duct cancer.

Belly Pain or Bloating

The pain or bloating typically occurs in the upper right part of the belly.

Fatigue

Fatigue is a common symptom of many types of cancer, including bile duct cancer.

Bile duct cancer is also called cholangiocarcinoma. Most bile duct cancer is adenocarcinoma. This growth begins in the mucus glands that line the insides of the bile ducts.

Bile duct tumors can occur in the main bile ducts outside the liver (extrahepatic) or within the liver (intrahepatic).

Extrahepatic Bile Duct Cancer

Most bile duct cancers — about 90 percent — develop in a bile duct outside the liver (extrahepatic). They can form in two regions:

Hilar Bile Duct Cancer

Hilar bile duct cancer is also called a Klatskin tumor or perihilar cholangiocarcinoma. It is found just outside the liver where the two main bile ducts meet as they leave the liver. About two-thirds of extrahepatic tumors are hilar bile duct cancer.

Distal Bile Duct Cancer

These tumors are found in the bile duct nearest the small intestine.

Intrahepatic Bile Duct Cancer

About 10 percent of bile duct cancer develops in the small duct branches within the liver. Intrahepatic bile duct tumors are often confused with primary liver cancer (hepatocellular carcinoma), a type of cancer that begins in the liver.

If you have symptoms that suggest you may have bile duct cancer, it’s vital that your doctor has a clear and complete understanding of what’s causing them. When making a diagnosis, our experts take the time to learn about your medical history and understand your overall health. We conduct a thorough physical examination. You may have some of the following tests.

Blood Tests

Diagnosing bile duct cancer may begin with a test to measure the amount of bilirubin in the blood. Bilirubin is a chemical that comes from the breakdown of red blood cells and gives bile its yellow color. A high bilirubin level can lead to jaundice (a yellowing of the skin and the whites of the eyes) and mean that the liver or bile ducts are not functioning properly. Other blood tests measure levels of liver enzymes (such as alkaline phosphatase, AST, ALT, and GGT) and can identify abnormal amounts of other substances (such as CEA and CA 19-9). Finding any of these substances in the blood can indicate the presence of bile duct cancer.

Imaging

we use the latest imaging techniques to pinpoint the exact size and location of newly diagnosed bile duct tumors. These details help us determine whether a tumor can be removed by surgery. They also help predict a tumor’s response to other treatments. Imaging helps guide our surgeons and interventional radiologists during many kinds of procedures.

The imaging techniques we use include:

  • CT Scans

CT scans take cross-sectional pictures of the body, helping doctors determine if the cancer cells are only in the bile duct or if they have spread to other areas. We use triphasic CT scans. These take images of the bile ducts, liver, and nearby lymph nodes during three phases of blood flow through the liver.

  • Ultrasound

Ultrasound is useful for detecting the location and number of tumors and whether the tumor involves the main blood vessels. Ultrasound can distinguish whether a mass is cancer or a benign (noncancerous) gallstone disease. Unlike CT, ultrasound does not use radiation.

  • Magnetic Resonance Cholangiopancreatography

This technique uses MRI to show how much a tumor has grown within the bile duct. It helps doctors determine if the tumor can be removed by surgery. More detailed MRI may be needed to see if the tumor has spread to the liver or other organs.

  • Positron Emission Tomography

PET scans can detect whether cancer has spread from the bile duct to other tissues or organs. PET and CT scans are sometimes used together (PET-CT) to pinpoint the exact location of tumors.

              Other Diagnostic Techniques

We may do other tests to learn more about the extent of the tumor and the types of cells involved. These are often performed in an outpatient setting.

 

  • Biopsy

During a biopsy, a doctor removes a small amount of tissue from the area where the cancer is suspected. We use an image-guided technique called fine needle aspiration to get the sample of bile duct cancer. This procedure is usually performed by an interventional radiologist (a doctor who specializes in minimally invasive techniques).

  • Endoscopy

This technique involves inserting an endoscope (a long, narrow tube attached to a camera and light) through the mouth and down the throat to examine the interior lining of the bile ducts.

  • Laparoscopy

In this procedure, a doctor inserts a laparoscope (a thin lighted tube with a camera on its tip) through a small incision (cut) in the abdominal wall to look at the organs in the belly or pelvis. The doctor can then see the size of the cancer and if it has spread to other organs and lymph nodes. This evaluation is also called staging. Tissue samples may be removed for a biopsy during a laparoscopy as well.

  • Surgery
    Sometimes a bile duct tumor is too small to do a biopsy. If cancer is suspected, surgery to remove the tumor may be necessary to confirm the diagnosis.

Bile duct cancer is classified according to the type and stage, from the earliest to the most advanced. The stages of bile duct cancer are based on the location and size of the tumor and how far it has spread.

Cancers at similar stages tend to have a similar outlook and are often treated in much the same way. When we know the stage of the cancer, our doctors can prepare a treatment plan that’s customized specifically to each person’s needs. The TNM classification system groups bile duct cancer into one of four stages. Here is a description of the stages:

Bile Duct Cancer Stages

 

  • Local Local bile duct cancer has not spread outside of the bile duct and can be removed by surgery.
  • Locally Advanced Locally advanced bile duct cancer is located in the bile duct, and it has spread to nearby organs, arteries, or veins but not to distant parts of the body.
  • Metastatic Metastatic bile duct cancer has spread to distant parts of the body and probably requires a treatment other than surgery.
  • Recurrent Recurrent bile duct cancer has come back after treatment. It may need to be staged again (called restaging) using the TNM classification system.

Bile Duct Cancer Treatment Groups

The stages of bile cancer are also generally grouped by how the cancer may be treated. There are two treatment groups:

 

  • Localized (Resectable) Bile Duct CancerThe cancer is in an area, such as the lower part of the common bile duct or hilar area (just outside the liver), where it can be removed completely by surgery.

 

  • Unresectable, Metastatic, or Recurrent Bile Duct Cancer
    Unresectable cancer cannot be removed completely by surgery. Most people with bile duct tumors have unresectable cancer. Metastatic bile duct cancer may have spread to the liver, other parts of the belly, or distant parts of the body. Recurrent bile duct cancer is cancer that has come back after treatment. The cancer may return in the bile ducts, liver, or gallbladder. Less often, it may come back in distant parts of the body

Surgery, chemotherapy, targeted therapy, radiation, or a combination of these may be used to treat bile duct cancer. Depending on the stage of the disease. We will determine the most appropriate treatment plan for you.

Surgery

Surgery is the preferred treatment for bile duct cancer and offers the best chance for a cure. The most effective approach for tumors that have not spread is surgery to remove the bile duct and the nearby lymph nodes. A more extensive surgery may be necessary if the cancer has spread.

Surgery is the preferred treatment for bile duct cancer and offers the best chance for a cure. The location and sensitivity of the bile ducts make surgery challenging. These procedures require a high level of expertise. For tumors that have not spread, the most effective approach is using a traditional open technique. Minimally invasive surgery is generally not used for bile duct surgery.

Partial Hepatectomy

This procedure is often used to treat large bile duct tumors inside the liver (intrahepatic tumors). It involves removing a piece of normal liver tissue, an entire lobe, or a larger part of the liver surrounding the cancer. The remaining section of the liver takes over all of the organ’s functions. Sometimes the liver grows back to its normal size within a few weeks. Before a partial hepatectomy, doctors may use a technique called portal vein embolization to redirect the blood supply to the healthy portion of the liver. This stimulates cell growth in the healthy part of the liver, allowing some people to have a partial hepatectomy who otherwise might have been ineligible for the surgery.

Whipple Procedure (Pancreatoduodenectomy)

The Whipple procedure is commonly used to treat extrahepatic bile duct tumors that are near the pancreas. It is also known as a pancreatoduodenectomy. The Whipple procedure involves removing part of the bile duct, part of the stomach, part of the small intestine, and the head (the rightmost section) of the pancreas. The remaining portions of the stomach, bile duct, and pancreas are then joined to the remaining small intestine so that digestive enzymes can mix with food. This ensures that the pancreatic fluids and bile will flow into the small intestine. Although this procedure is complex, it is safe and effective for many people. The Whipple procedure is also used to treat pancreatic cancer.

 

Symptom Relief for Bile Duct Cancer

                (Cholangiocarcinoma)

Many people are not candidates for surgery by the time bile duct cancer is diagnosed. However, minimally invasive, image-guided procedures can help improve their quality of life by allowing bile to flow through or drain out of a bile duct that is blocked by a tumor. Our surgeons and experts in interventional radiology use tools, such as catheters and needles, to relieve such symptoms as jaundice (yellowing of the skin and the whites of the eyes), itching, nausea, vomiting, and infection.

Biliary Bypass

A biliary bypass connects the bile duct or gallbladder directly to the small intestine. This creates a new way for bile to get around a tumor that is blocking the flow from the bile duct. Our doctors can often perform a biliary bypass using a laparoscope (a thin lighted tube with a camera on its tip) inserted through a small incision (cut) in the belly.

Stent Placement

A stent is a small tube that can relieve a blocked bile duct so bile can flow across a blockage to the small intestine. When possible, the stent is placed using an endoscope (a small tubelike instrument) inserted through the mouth. When that method is not possible, a stent can be placed with a needle inserted through the liver. Imaging is used to identify the blockage and place a stent (or a drainage catheter, a small flexible tube) through the blockage. Some people may need to have a catheter for a while after getting a stent.

Neurolytic Celiac Plexus Block

People with advanced bile duct cancer often have pain. The pain may be caused by cancer cells that have invaded a cluster of nerves near the liver known as the celiac plexus. People who don’t get enough pain relief with conventional drugs may benefit from a procedure called neurolytic celiac plexus block (NCPB). This involves injecting a local anesthetic into the celiac plexus to disrupt the body’s pain signals. NCPB has been shown to reduce pain significantly and improve mood and life expectancy for people with advanced bile duct cancer.

NCPB can be performed with a laparoscope while examining the bile duct to diagnose and stage the cancer. Many other cancer centers perform percutaneous NCPB (through the skin) with a needle. This method is associated with side effects, such as muscle and limb weakness. Laparoscopic NCPB may provide similar or better pain relief than percutaneous NCPB with fewer potential side effects.

NCPB also can be performed using an endoscopic ultrasound. This involves inserting a small probe through the mouth and into the stomach to get a detailed view of the celiac plexus. As with laparoscopic NCPB, endoscopic NCPB may be at least as effective as the percutaneous approach, with fewer potential side effects.

Ablation

Ablation destroys cancer cells by delivering heat or cold through a needle placed into a bile duct tumor. It requires no incisions (cuts) and is effective for some people with isolated tumors who are not candidates for bile duct surgery.

Embolization

In this technique, microscopic beads are injected into blood vessels that feed the bile duct tumor to cut off its blood supply. This can kill the tumor.

Chemotherapy is a drug or a combination of drugs that kills cancer cells wherever they are in the body. You may receive chemotherapy before surgery to shrink a bile duct tumor. This is called neoadjuvant therapy. If you receive chemotherapy after surgery to destroy and cancer cells that may remain, it is called adjuvant therapy.

The standard chemotherapy drugs for bile duct cancer are gemcitabine (Gemzar®) and cisplatin. Other drugs sometimes used include fluorouracil (also called 5-FU), oxaliplatin (Eloxatin®), and capecitabine (Xeloda®). We will carefully tailor your treatment to make sure that it’s as effective as possible while helping maintain your quality of life.

If you have bile duct cancer that has spread, you may receive chemotherapy as the main treatment if surgery is not an option. Research has suggested that the combination of gemcitabine and cisplatin can lengthen the lives of people with bile duct cancer that cannot be removed by surgery.

Chemotherapy is also occasionally given to relieve symptoms due to bile duct cancer, such as a tumor that is pressing on a nerve and causing pain.

Chemotherapy with Hepatic Arterial Infusion

we are evaluating the potential of a new chemotherapy technique called hepatic arterial infusion (HAI) in the treatment of bile duct cancer. HAI involves delivering a high dose of chemotherapy drugs directly to the liver through a tiny pump implanted under the skin in the lower belly. The chemotherapy passes from the liver into the bile ducts. HAI therapy may be used to shrink tumors before surgery.

Targeted Therapies for Bile Duct Cancer

Targeted therapies block specific changes in cancer cells that help them grow and survive or disrupt their blood supply. We are currently investigating several targeted therapies for bile duct cancer.

Ivosidenib blocks an abnormal form of a protein called IDH1. Abnormal IDH1 causes too much of a substance called 2-HG to be produced. Scientists believe that too much 2-HG can fuel the growth of bile duct cancer.

Two other targeted therapies, pemigatinib (INCB054828) and infigratinib (BGJ398) help reduce the production of the abnormal form of a protein called FGFR2 in cancer cells.

Radiation Therapy for Bile Duct Cancer (Cholangiocarcinoma)

 

Radiation therapy uses precisely focused high-energy beams to kill cancer cells. To treat bile duct cancer, our doctors may give radiation alone or in combination with chemotherapy or other treatments. The kind of radiation we recommend depends on the type of bile duct cancer, the location of the tumor, and whether it has spread.

External-beam radiation therapy is the most common type of radiation therapy used to treat bile duct cancer. The radiation may be given alone or in combination with a radiosensitizer. This is a drug that makes the body more sensitive to radiation.

You may have heard of CyberKnife. This is a brand name for a type of radiation therapy called stereotactic body radiation therapy. KIMS uses a similar technology that employs a system called TrueBeam, which incorporates CT imaging into the same device that delivers the radiation. This method destroys tumors with very intense doses of radiation in fewer sessions than standard radiation therapy.

Minimizing Radiation Side Effects

Our doctors use highly sophisticated computer software and 3-D computer images from CT scans to develop individualized plans for each person we care for. This makes it possible to deliver high doses of radiation to a bile duct tumor while sparing surrounding organs and reducing the risk to healthy tissue.

Our doctors use radiation therapy to treat bile duct cancer in several ways:

Before or After Surgery

Radiation is occasionally given before surgery to shrink a tumor so it is possible to remove it.

After a bile duct tumor has been removed, radiation may be given to the area where the tumor once was or in the nearby lymph nodes. This is done to destroy any cancer cells that may remain following surgery.

As the Main Cancer Treatment

Radiation therapy may be used as the main treatment in people with bile duct cancer that has spread throughout the body and cannot be removed by surgery. The radiation will not cure the cancer, but it may help people with advanced bile duct cancer live longer.

As Palliative Therapy

Radiation is sometimes given to people with advanced bile duct cancer to shrink a tumor that is causing discomfort by blocking blood vessels or bile ducts or pressing on nerves.

 

         Living Beyond Bile Duct Cancer 

                (Cholangiocarcinoma)

we know that even after you’ve finished bile duct cancer treatments, you may still need our help. We’re committed to supporting you in every way we can — physically, emotionally, spiritually, and otherwise — for as long as you need us.

We’ve built a program designed for cancer survivors and their families. Our Survivorship Center has many services for you and your loved ones, including support groups, follow-up programs, educational resources, and more.

 

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