Though bile duct cancer can affect anyone, it’s more common in individuals older than age 65, according to the National Institutes of Health (NIH). Bile duct cancer is often difficult to treat because most people don’t get a diagnosis until the cancer has already spread to other areas of the body. However, recent medical advances and newer treatment options have improved the outlook for people with this type of cancer.
Intrahepatic Cholangiocarcinoma This type starts in bile ducts in the liver. It’s sometimes confused with cancer that starts in the liver (called hepatocellular carcinoma).Hilar Cholangiocarcinoma The variety occurs in the bile ducts just outside the liver. It’s also called perihilar cholangiocarcinoma.Distal Cholangiocarcinoma This type is located farther down in bile ducts near the small intestine.
The hilar and distal forms of bile duct cancer, which are both found outside the liver, are sometimes grouped together as extrahepatic cholangiocarcinoma. Most bile duct cancers are cholangiocarcinomas, which begin in the gland cells that line the inside of the ducts, according to the American Cancer Society. However, there are also some less common types of bile duct cancers, including:
SarcomasLymphomasSmall cell cancers
Some possible signs and symptoms of bile duct cancer include:
Jaundice (yellowing of the skin or whites of the eyes)Itchy skinWhite- or pale-colored stoolsFatigueNausea or vomitingAbdominal pain on the right side of your bodyFeverNight sweatsUnexplained weight lossDark-colored urine
Liver Function Test This is a blood test that measures certain substances that the liver releases into the blood.Tumor Marker Test It checks your blood for levels of carbohydrate antigen 19-9, a protein that bile duct cancer cells overproduce.Endoscopic Retrograde Cholangiopancreatography Doctors insert a thin tube with a camera down the digestive tract and into the small intestine. They use the camera to view the spot where the bile ducts meet the small intestine. Doctors sometimes also inject a dye into the bile ducts to better visualize them.Imaging Tests Your provider may recommend other imaging procedures to check for signs of cancer. Some common tests for diagnosing bile duct cancer include ultrasound, computed tomography, positron emission tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography.Biopsy A biopsy is a procedure to remove a small piece of tissue to examine under a microscope in the lab.
Doctors stage bile duct cancer by evaluating the size of the tumor and determining whether it’s spread to other areas, including blood vessels, lymph nodes, organs near the bile ducts (such as the liver or gallbladder), or distant organs (such as the lungs, bones, or abdominal cavity), according to the Cleveland Clinic.
Primary sclerosing cholangitis (hardening and scarring of the bile ducts)Bile duct stones (small stones that can cause inflammation)Choledochal cyst disease (bile duct cysts)Cirrhosis (scarring of the liver)Hepatitis B virusHepatitis C virusAbnormalities where the bile and pancreatic duct meetClonorchiasis (a parasite in the liver)Nonalcoholic fatty liver disease (a buildup of fat in the liver cells that’s not due to alcohol use)Polycystic liver disease (a rare, genetic disease of the liver)Caroli syndrome (an abnormality of the intrahepatic bile ducts that is present at birth)
Other factors that many elevate your chances of having bile duct cancer include:
Being Hispanic AmericanLiving in an area where liver parasites are commonHaving type 1 or type 2 diabetesBeing older than 50 years of ageBeing obeseHaving ulcerative colitis or inflammatory bowel diseaseBeing exposed to toxins, such as radon, asbestos, or thorium dioxideHaving a family history of bile duct cancerBeing a current or former smokerHaving an alcohol use disorderHaving human immunodeficiency virus (HIV)
JaundiceInfectionLiver dysfunction or liver failure
According to the American Cancer Society, the five-year survival rate for all stages of the disease is 10 percent for extrahepatic bile duct cancers (those that start outside the liver) and 9 percent for intrahepatic bile duct cancers (those that start in the liver). It’s important to know that these statistics are only estimates based on existing data, and they won’t predict what will happen in every case. Additionally, survival rates may be higher or lower depending on the stage of the disease.
Surgery
Surgery is an option for bile duct cancers that have been caught early and haven’t spread to other areas of the body. Surgeons will try to remove as much of the cancer as possible. If the cancer is more advanced, they may have to also remove liver tissue, pancreatic tissue, and/or lymph nodes. In some cases, doctors might recommend a liver transplant. This involves removing the liver and replacing it with a donor liver. This procedure can sometimes cure people with very specific types of bile duct cancer. However, finding a donor is often challenging, and very few people are candidates for a liver transplant, according to the American Cancer Society.
Chemotherapy
Chemotherapy, which uses drugs to kill cancer cells, may be an option for certain people with bile duct cancer. Common chemotherapy regimens, according to the National Cancer Institute, include:
Gemcitabine (Gemzar) and cisplatin (Platinol)XELOX: capecitabine (Xeloda) and oxaliplatin (Eloxatin)GEMOX: gemcitabine (Gemzar) and oxaliplatin (Eloxatin)Gemcitabine (Gemzar) and capecitabine (Xeloda)
Sometimes, doctors perform the following special procedures to deliver chemotherapy directly to the affected bile duct:
Transarterial Chemoembolization Tiny beads are placed into the blood vessels to stop blood from reaching the tumor. The beads also release chemotherapy drugs to shrink the tumor.Hepatic Artery Infusion This procedure involves using a surgically implanted pump to inject chemo directly into the main artery that supplies blood to the liver.
Radiation
Radiation therapy uses beams of energy to kill cancer cells in the body. According to the American Cancer Society, radiation isn’t a common treatment for bile duct cancer, but it can be used in some situations, such as before or after surgery or for advanced cancers. The treatment may be delivered using the following methods:
External Beam Radiation A machine directs radiation beams at your body.Brachytherapy Radioactive materials are placed inside your body near the cancer site.
Targeted Therapy
Targeted therapy involves using treatments that block specific abnormalities in cancer cells. These therapies may be suitable for people with bile duct cancer caused by a faulty gene that allows proteins to develop on their cancer cells. According to the American Cancer Society, some targeted treatments used for bile duct cancer are:
FGFR2 Inhibitors These block the abnormal FGFR2 protein in bile duct cancer cells. FGFR2 inhibitors include pemigatinib (Pemazyre), infigratinib (Truseltiq), and futibatinib (Lytgobi).IDH1 Inhibitor The medicine ivosidenib (Tibsovo) is an IDH1 inhibitor. It works by blocking the abnormal IDH1 protein.
Immunotherapy
Immunotherapy employs your body’s own immune system to fight cancer. It can be an option for advanced bile duct cancers when other treatments have failed. The immunotherapy medicine pembrolizumab (Keytruda) may help treat some cases of bile duct cancer, according to the National Cancer Institute. In September of 2022, the U.S. Food and Drug Administration also approved the immunotherapy drug durvalumab (Imfinzi) in combination with the chemotherapy regimen of gemcitabine (Gemzar) and cisplatin (Platinol) for people with advanced bile duct cancer. The approval came after a clinical trial found that adding the immunotherapy treatment extended survival by about six weeks in people with advanced biliary tract cancers.
Ablation
Bile duct cancer that forms in the liver may be treated with ablation methods, such as:
Cryotherapy This involves using extreme cold to freeze abnormal tissue.Radiofrequency Ablation This method uses heat to destroy cancer cells.
While these treatments may help control tumors, nearly all will start to grow back in the future, according to the American Cancer Society.
Palliative Treatments
Palliative treatments are used to help relieve symptoms of advanced bile duct cancer, rather than cure the disease. Chemotherapy, radiation, ablation, and surgery may all be used to provide palliative care. Other approaches may include:
Biliary Stent or Catheter If cancer blocks a bile duct, doctors can place a small stent or catheter into the duct to keep it open.Biliary Bypass Surgeons create a bypass around a blocked bile duct by connected parts of the duct or the intestine.Photodynamic Therapy A light-activated medicine that collects in cancer cells is injected into your vein. A few days later, doctors use a special laser to activate the drug and kill cancer cells.Alcohol Injection Doctors inject affected nerves with alcohol to help lessen pain.
Clinical Trials
Clinical trials may be an option if you have bile duct cancer. These research studies test experimental therapies that aren’t yet available to the public. If you participate in a clinical trial, you may receive a novel treatment that wouldn’t be offered to you otherwise.
Quitting smokingAvoiding certain viruses, such as HIV, hepatitis B, and hepatitis CGetting vaccinated against hepatitis BLimiting alcohol consumptionMaintaining a healthy weightAvoiding being around environmental toxins
The average age of diagnosis in the United States is 70 for intrahepatic bile duct cancer and 72 for extrahepatic bile duct cancer. The cancer affects slightly more men than women, according to the NIH. Bile duct cancer is more common in Southeast Asia, where parasite infections that cause the cancer are more widespread.
Liver cancerGallbladder cancerCirrhosisNonalcoholic fatty liver diseaseHepatitis BHepatitis CAlcoholismPrimary sclerosing cholangitisCholedochal cyst diseasePolycystic liver diseaseBile duct stonesClonorchiasisCaroli syndrome
The Cholangiocarcinoma Foundation GI Cancers Alliance Cholangiocarcinoma Support Group (Facebook) American Cancer Society Mayo Clinic Cleveland Clinic American Society of Clinical Oncology Cancer Support Community
