Cholangiocarcinoma, or bile duct cancer, is a rare disease in which malignant cells form in the bile ducts. There are two types of cholangiocarcinoma: intrahepatic and extrahepatic.

Intrahepatic cholangiocarcinoma forms in the bile ducts inside the liver. Only a small number of bile duct cancers are intrahepatic.

Extrahepatic cholangiocarcinoma, which is far more common, forms in the bile ducts outside of the liver. It can be further categorized by region:

  • Perihilar bile duct cancer forms in the hilum region, the area where the right and left bile ducts exit the liver and join to form the common hepatic duct. Perihilar bile duct cancer is also called a Klatskin tumor.
  • Distal extrahepatic bile duct cancer forms in the distal region, which includes the common bile duct.

Risk factors for developing cholangiocarcinoma include:

  • Ulcerative colitis
  • Primary sclerosing cholangitis
  • Cysts in the bile ducts
  • Infection with a Chinese liver fluke parasite

Symptoms of cholangiocarcinoma can include:

  • Jaundice, or yellowing of the eyes and skin
  • Dark urine
  • Pale or clay-colored stool
  • Abdominal pain
  • Fever
  • Itching
  • Nausea and vomiting
  • Weight loss

A combination of blood tests and imaging tests may be used to diagnose cholangiocarcinoma, but oftentimes an endoscopic procedure, such as an endoscopic retrograde cholangiopancreatography (ERCP), or surgery may be needed.

Cholangiocarcinoma can typically only be cured when it is diagnosed in the early stages. Once the cancer has spread, treatment focuses on symptom relief and quality of life.

If cholangiocarcinoma is diagnosed at an early stage, surgery may be done to remove the tumor. Types of surgery to treat cholangiocarcinoma include:

  • Removal of the bile duct: A surgical procedure to remove part of the bile duct if the tumor is small and in the bile duct only. Lymph nodes are removed and tissue from the lymph nodes is viewed under a microscope to see if the cancer has spread.
  • Partial hepatectomy: A surgical procedure in which the part of the liver where cancer is found is removed. The part removed may be a wedge of tissue, an entire lobe, or a larger part of the liver, along with some normal tissue around it.
  • Whipple procedure: A surgical procedure in which the head of the pancreas, the gallbladder, part of the stomach, part of the small intestine, and the bile duct are removed. Enough of the pancreas is left to produce digestive enzymes and insulin, and the remaining essential digestive organs are reconnected on the inside in a different way than they were before.

If the cancer has spread and cannot be removed, palliative surgery may be done to relieve symptoms and improve quality of life. Types of palliative surgery include:

  • Biliary bypass: A surgical procedure in which the part of the bile duct before the blockage is connected with part of the bile duct that is past the blockage or to the small intestine. This allows bile to flow to the gallbladder or small intestine.
  • Endoscopic stent placement: A surgical procedure in which a thin tube, called a stent, is placed in the bile duct to open it and allow bile to flow into the small intestine or through a catheter that goes to a collection bag outside of the body.
  • Percutaneous transhepatic biliary drainage: A procedure done to drain bile when there is a blockage and endoscopic stent placement is not possible. This is where a catheter, or thin tube, is placed from the skin into the gallbladder to allow bile to drain out of the body. An x-ray of the liver and bile ducts is done to locate the blockage. Images made by ultrasound are used to guide placement of a stent, which is left in the liver to drain bile into the small intestine or a collection bag outside the body.

Radiation Therapy
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The type of radiation therapy used depends on the type and stage of the cancer.

Chemotherapy uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Systemic chemotherapy, which is taken by mouth or injected into a vein or muscle, targets cancer cells throughout the body. Regional chemotherapy, which is placed directly into the affected organ, targets cancer cells in that specific area, or region, of the body. The type of chemotherapy used depends on the type and stage of the cancer.