There are two primary types of biliary (hepatobiliary) cancer: hepatocellular carcinoma, which refers to cancer than develops within the liver, and cholangiocarcinoma, which refers to cancer that develops within the liver's biliary ducts. Of these cancers, hepatocellular carcinoma is the most common.
Cholangiocarcinoma can be further categorized as either intrahepatic biliary duct cancer if the cancer develops within the ducts of the liver, or as extrahepatic biliary duct cancer if cancer occurs outside the liver, in the area that attaches the liver to the gallbladder.
In the United States, the most common cause of cholangiocarcinoma is primary sclerosing cholangitis (PSC), an inflammatory condition of either the intrahepatic or extrahepatic bile ducts. PSC is thought to be an autoimmune disease, which causes blockages within bile ducts within the liver. It may also cause cancer in the ducts as well as liver failure. (In Asia, cholangiocarcinoma most often develops in individuals after certain parasitic infections.)
Biliary (and liver) cancers sometimes produce jaundice (yellow discoloration of the skin) from a blockage of a bile duct or overall liver dysfunction. They also sometimes can lead to abdominal pain, itching, or fluid in the legs (ascites).
Patients with primary sclerosing cholangitis, ulcerative colitis, an inflammatory disease of the colon, or a history of gallstones have a higher risk of cholangiocarcinoma. Other less common causes of cholangiocarcinomas include certain genetic disorders, including some which are associated with other cancers, including colon cancer, and diseases which cause cysts to develop in the liver. A type of dye used in radiology from the 1930 to the 1950s, called Thorotrast, has also been shown to be a risk factor of biliary cancer. Men have a slightly higher risk than women for cholangiocarcinoma.
To diagnose disorders of the duct systems of the gallbladder, liver, and pancreas, doctors may perform an endoscopic retrograde cholangiopancreatography, or ERCP, to determine if the biliary ducts are blocked by a tumor or gallstones. In this procedure, the physician places an endoscope into the esophagus and guides it into the stomach, and the duodenum — the top of the small intestine. From here, the doctor inserts a catheter through the endoscope and into the biliary ducts, injects a contrast dye, and x-rays the area.
If your physician finds that the duct is blocked, he or she can open it by angioplasty, a mechanical widening of a narrowed duct, and by inserting a tiny stent into the duct, if necessary, to keep it open. Your doctor will also take a small tissue biopsy during the endoscopy to determine if cancer is present. He or she also can relieve pain related to the duct systems by establishing drainage into the duodenum or stomach.
Percutaneous transhepatic cholangiography (PTC) is another procedure in which an x-ray is taken of the biliary duct. PTC requires patients to fast for six hours prior to undergoing the test. They also usually receive a sedative shortly before the test begins.
Endoscopic ultrasound scan (EUS) — similar to the ERCP but uses an ultrasound probe placed within the endoscope – can also provide a very detailed view of the pancreas and bile ducts.
If an ERCP cannot be done, or sometimes in addition, doctors will perform a CT scan or MRI.
Your physician will draw a blood sample to measure certain blood cancer markers if biliary cancer is suspected. A biopsy is not always needed for diagnosis if the imaging and blood markers are definitive.
Surgery is usually performed to remove the cancer and any blockages, and offers the best chance of cure for cholangiocarcinoma. We perform most surgeries for biliary cancer laparoscopically.
If the cancer is small and contained within the ducts, the bile ducts with the cancer are removed and those left will be joined to the small bowel so bile will be able to flow. If the cancer has spread to the liver, the section of the liver affected is removed with the bile ducts. If the cancer is more extensive and has spread to surrounding areas, surgeons will generally perform a Whipple procedure, removing the bile ducts, a part of the stomach, part of the duodenum (the top of the small bowel), the pancreas, gallbladder, and the surrounding lymph nodes.
If there are any blockages of the bile ducts and the tumor cannot be removed, surgeons may perform a bypass surgery to join the gallbladder or bile duct to a part of the small intestine to enable bile to flow from the liver to the intestine. If the cancer blocks the duodenum, part of the small bowel may be connected to the stomach to bypass the duodenum.
NewYork-Presbyterian Transplant Institute is one of a very few centers in the US to offer liver transplantation for selected patients with cholangiocarcinoma.
Liver transplantation has been shown to produce much better results than traditional treatments in some patients when combined with chemotherapy and radiation therapy.
Our radiation oncologists offer a sophisticated brachytherapy program, in which very small catheters are threaded to the site of the tumor to deliver a high does of concentrated radiation designed to reach the tumor and while avoiding healthy tissue.