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What is bile duct cancer (cholangiocarcinoma)?

Bile duct cancer (cholangiocarcinoma) is a rare form of cancer that starts in the cells of the inner lining (epithelium) of the bile duct. The bile ducts are part of the digestive system. Bile is a yellow-green fluid that helps your body digest fats. The bile ducts are tubes that carry bile from the liver and gall bladder to the first part of the small intestine (duodenum).

Liver, Pancreas, and Gallbladder with Callout (Layout) (2)_edited.png

Cholangiocarcinoma is divided into 2 main groups based on where they start – either in the part of the bile duct outside the liver (extrahepatic cholangiocarcinoma or eCCA) or in the part inside the liver (intrahepatic cholangiocarcinoma or iCCA). Extrahepatic cholangiocarcinoma can be further divided into perihilar/hilar cholangiocarcinoma (pCCA) and distal cholangiocarcinoma (dCCA).

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Adapted from the Canadian Cancer Society cancer.ca

Signs and Symptoms

Jaundice is the most common symptom of bile duct cancer. This condition causes a yellowing of the skin and the whites of the eyes. It can also result in dark yellow urine and clay-colored stools. The stool might also become lighter in color.

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Bile contains bilirubin, a yellow-green pigment. When bile flow to the small intestine is obstructed, bilirubin levels rise in the body, leading to jaundice. Additionally, if the cancer blocks the passage of bile or pancreatic juice into the intestine, it can hinder the digestion of fatty foods.

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These and other signs and symptoms may be caused by bile duct cancer or by other conditions.

  • Jaundice (yellowing of the skin or whites of the eyes)

  • Dark urine

  • Clay colored stool

  • Abdominal (belly) pain

  • Fever

  • Itchy skin

  • Nausea and vomiting

  • Weight loss

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​It's important to note that other health conditions can produce similar symptoms. Always consult your medical team if you are experiencing any symptoms.

Adapted from the Canadian Cancer Society cancer.ca

Risk factors

Parasite infections

Such as the infestation with liver flukes (Parasites from eating undercooked fish) that are endemic to southeast Asian regions

Cystic biliary lesion

Patients with choledochal cysts, biliary mucinous cystic neoplasms, or intraductal papillary biliary mucinous neoplasms

Genetic predisposition

With hereditary nonpolyposis colorectal cancer (lynch syndrome), bap1-related tumor predisposition syndrome, multiple biliary papillomatosis, and cystic fibrosis

Primary sclerosing cholangitis

Progressive autoimmune liver disease

Chronic liver disease

With hepatitis B or C, hemochromatosis, metabolic dysfunction-associated steatotic liver disease (MASLD, formerly nonalcoholic fatty liver disease or NAFLD), and cirrhosis

Biliary tree calculi

(Gallstones in the liver or bile ducts) particularly with larger stones or long-term illness

Lifestyle, environmental, and metabolic factors

Such as type 2 diabetes, obesity, alcohol consumption, and cigarette smoking, exposure to thorotrast, or to asbestos and propylene dichloride

Adapted from Bile Duct Cancer Diagnosis by the National Cancer Institute cancer.gov

Diagnosis

Physical exam and health history

The doctor checks for any signs of disease like lumps or anything unusual and reviews the patient's past health habits, illnesses, and treatments

Liver function tests

A blood test checks the levels of bilirubin and alkaline phosphatase released by the liver. High levels can indicate liver disease, possibly from bile duct cancer

Laboratory tests

Various tests using samples of tissue, blood, urine, or other substances help diagnose disease, plan treatments, and monitor the disease over time

CEA and CA 19-9 tumor marker tests

These tests check for tumor markers in the blood. High levels of these markers might indicate bile duct cancer

Ultrasound exam

Uses high-energy sound waves to create images of internal organs. The sound waves bounce off organs and tissues to form a sonogram

CT scan (CAT scan)

Uses a computer linked to an x-ray machine to create detailed pictures of the body. A dye may be used to highlight organs or tissues

Magnetic resonance imaging (MRI)

Uses a magnet, radio waves, and a computer to create detailed images of the body

Magnetic resonance cholangiopancreatography (MRCP)

An MRI specifically for detailed images of the liver, bile ducts, gallbladder, pancreas, and pancreatic duct

Adapted from Bile Duct Cancer Diagnosis by the National Cancer Institute cancer.gov

Treatment

Surgery

Intrahepatic Bile Duct Cancer​

  • Surgically removing part of the liver with the goal of ensuring no cancerous tissue remains offers a chance to cure bile duct cancers within the liver. Sometimes, if a large portion of the liver needs to be removed, doctors may use a procedure called portal vein embolization beforehand. This helps the remaining liver to grow larger and function well after surgery.​

Perihilar Bile Duct Cancer​

  • The specific surgical approach for perihilar bile duct cancer depends on where the tumor is located, how much of the liver is affected, and how close the tumor is to major blood vessels. During surgery, doctors also check the lymph nodes to understand the cancer's prognosis. ​

Distal Bile Duct Cancer​

  • Complete surgical removal with clear margins is the only way to cure cancers of the lower bile duct, which are difficult to treat because they are close to major blood vessels. Lesions are usually treated with pancreaticoduodenectomy (Whipple procedure)

Liver transplant

Liver transplantation might be considered for patients with early-stage cholangiocarcinoma after extremely careful consideration by the medical team.​

Targeted therapy

Patients who have mutations that can be targeted should think about participating in clinical trials testing new therapies. Currently, targeted treatments are approved only for patients whose cancer has worsened or who cannot use standard initial treatments.​

Three drugs have been approved by the Food and Drug administration (FDA) for cholangiocarcinoma including:​

  • Pemigatinib (Pemazyre)​

  • Ivosidenib (Tibsovo) for cholangiocarcinoma with IDH1 mutation

Adjuvant therapy

Some studies suggest that adjuvant therapy after complete surgery might be helpful. For example, ASCO guidelines recommend adjuvant capecitabine for a period of 6 months following curative resection of cholangiocarcinoma.

Chemotherapy & immunotherapy

For certain patients who are generally healthy, doctors might recommend systemic chemotherapy. This standard of care first treatment uses a combination of chemotherapy drugs (Gemcitabine and Cisplatin) and an immunotherapy drug (Durvalumab or Pembrolizumab). These drugs have been known to temporarily reduce the size of tumors in some patients, although it doesn't work for everyone.

Palliative approaches

Palliative approaches focus on relieving symptoms and improving quality of life, usually because the cancer has spread throughout the body or has come back in a different part of the body after treatment.

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Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®)–Health Professional Version, originally published by the National Cancer Institute.

Shroff, R. T. et al., 2019. Adjuvant therapy for resected biliary tract cancer: ASCO clinical practice guideline. Journal of Clinical Oncology, 37(12), 1015-1027.​

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