TN 57 (08-23)

DI 23022.704 Cholangiocarcinoma




Bile Duct Adenocarcinoma; Bile Duct Cancer; Biliary Tract Cancer; cHCC-CC; Combined HCC-CC; Combined Hepatocellular Cholangiocarcinoma; Combined hepatocellular-cholangiocarcinoma; Distal Bile Duct Cancer; Distal Cholangiocarcinoma; Distal Extrahepatic Cholangiocarcinoma; Extrahepatic Cholangiocarcinoma; Hepato Cholangiocarcinoma; Hepatocholangiocarcinoma; Hilar Bile Duct Cancer; Hilar Cholangiocarcinoma; Intrahepatic Cholangiocarcinoma; Perihilar Bile Duct Cancer; Perihilar Cholangiocarcinoma


Cholangiocarcinoma is a cancer that develops in the cells within the thin tubes (bile ducts) that bring the fluid that helps you digest food (bile) from the liver and gallbladder to the small intestine.

Cancer that occurs within the bile ducts is categorized as biliary tract cancer (BTC) and can occur in the bile ducts both inside and outside the liver. Because it is a BTC, cholangiocarcinoma is often incorrectly grouped with gallbladder cancers , which are another type of BTC.

There are three types of cholangiocarcinoma:

  • Extrahepatic or distal cholangiocarcinoma occurs in the portion of the bile duct nearest the small intestine. The cancer may be in the bile ducts as they exit the liver, or in the bile ducts when they end in the small intestine. This is the most common type of bile duct cancer.

  • Intrahepatic cholangiocarcinoma occurs in the parts of the bile ducts within the liver. This type of cancer should not be confused with liver cancer .

  • Hilar cholangiocarcinoma, a subset of extrahepatic cholangiocarcinoma, occurs in hilum, the area where the bile ducts and important blood vessels connect with the liver. This type is also called perihilar cholangiocarcinoma.

Cholangiocarcinoma is rare. About 8,000 people in the U.S. develop this cancer each year. It can occur at any age, but most commonly affects people older than 50. Hispanic Americans have a higher risk than other ethnic groups of developing cholangiocarcinoma. The condition is often diagnosed when it is advanced, making successful treatment difficult.

The cause of cholangiocarcinoma is unknown. However, there are some risk factors that suggest that health conditions that cause chronic (long-term) inflammation in the bile ducts may play a role in its development.

Some factors that may increase the risk of cholangiocarcinoma include:

  • Primary sclerosing cholangitis (inflammation, hardening, and scarring blocking the bile ducts);

  • Cirrhosis of the liver;

  • Hepatitis B or C infection;

  • Biliary tract stones; and

  • Parasitic infection.


Diagnostic testing: A diagnosis of cholangiocarcinoma may be made using:

  • Liver function tests;

  • Tumor marker tests;

  • Abdominal ultrasound;

  • Magnetic resonance cholangiopancreatography (MRCP);

  • Endoscopic retrograde cholangiopancreatography (ERCP);

  • Percutaneous transhepatic cholangiography (PTC); or

  • Biopsy.

Physical findings: Symptoms and physical findings of cholangiocarcinoma may include:

  • Abdominal pain;

  • Dark urine;

  • Fever;

  • Itchy skin;

  • Jaundice (yellowing of skin and whites of eyes);

  • Light-colored stool;

  • Nausea and vomiting; and

  • Unexplained weight loss.

ICD-9: 155.1

ICD-10: C22.1


The prognosis for people with cholangiocarcinoma is usually poor. The five-year survival rate for bile duct cancer that hasn’t spread outside of the bile ducts is 10% to 15%. This rate drops to 2% if the cancer spreads to areas of the body that are far from the bile ducts, such as the lungs. However, newer treatments mean these rates will improve over time.


Treatment for cholangiocarcinoma depends on the location of the cancer and if it has spread.

There are various surgical treatments offered for bile duct cancer, such as:

  • Bile duct removal (bile duct removal if cancer has not spread);

  • Partial hepatectomy (partial removal of the bile duct and sections of the liver);

  • Whipple procedure (removal of the bile duct, gallbladder, and part of the pancreas, stomach, and small intestine);

  • Liver transplant; and

  • Palliative surgery.

Because most bile duct cancers have spread by the time they are diagnosed, a healthcare provider may recommend a combination of multiple treatments, including radiation therapy, chemotherapy, targeted therapy, and immunotherapy.


Suggested MER for Evaluation:

  • Clinical history and physical examination that describes the diagnostic features of the impairment;

  • Biopsy or needle aspiration information found in operative notes, pathology reports, summaries of hospitalization or other medical reports that include details of the surgical and pathological findings; and

  • Results of liver function tests, tumor marker tests, abdominal ultrasound, MRCP, ERCP, or PTC testing.

Suggested Listings for Evaluation:






Prognosis is poor based on confirmed diagnosis alone. Response to treatment is not necessary to meet the listing.




* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

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DI 23022.704 - Cholangiocarcinoma - 08/09/2023
Batch run: 08/09/2023