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Cholangiocarcinoma

Bile duct cancer

Cholangiocarcinoma (CCA) is a rare cancerous (malignant) growth in one of the ducts that carries bile from the liver to the small intestine.

Causes

The exact cause of CCA isn't known. However, many of these tumors are already quite advanced by the time they are found.

CCA may start anywhere along the bile ducts. These tumors block off the bile ducts.

Both men and women are affected. Most people are older than 65.

People with the following health problems may have a higher chance of developing CCA:

Symptoms

Symptoms of CCA may include any of the following:

  • Fever and chills
  • Clay-colored stools and dark urine
  • Itching
  • Loss of appetite
  • Pain in the upper right abdomen that may radiate to the back
  • Weight loss
  • Yellowing of the skin (jaundice)

Exams and Tests

Your health care provider will perform a physical exam. Tests will be done to check for a tumor or blockage in the bile duct. These may include:

  • Abdominal CT scan
  • Abdominal ultrasound
  • X-ray of the bile ducts (PTCA)
  • Procedure that uses a viewing scope to look at the bile ducts (ERCP), during which tissue may be taken and looked at under a microscope

Blood tests that may be done include:

  • Liver function tests (especially alkaline phosphatase or bilirubin levels)
  • Complete blood count (CBC)

Treatment

The goal is to treat the cancer and the blockage it causes. When possible, surgery to remove the tumor is the treatment of choice and may result in a cure. Often the cancer has already spread locally or to another area of the body by the time it is diagnosed. As a result, surgery to cure the cancer is not possible.

Chemotherapy or radiation may be given after surgery to decrease the risk of the cancer returning. But the benefit of this treatment is not certain.

In select cases, a liver transplant can be tried.

Endoscopic therapy with stent placement can temporarily relieve blockages in the biliary ducts. This may also relieve jaundice when the tumor cannot be removed.

Support Groups

You can ease the stress of illness by joining a support group with members who share common experiences and problems.

Hospice is often a good resource for people with CCA that cannot be cured.

Outlook (Prognosis)

Completely removing the tumor allows some people to survive with the possibility of complete cure.

If the tumor cannot be completely removed, a cure is generally not possible. With treatment, about one half of affected people live a year, and about one half live longer, but rarely beyond 5 years.

Possible Complications

Complications of CCA include:

  • Infection
  • Liver failure
  • Spread (metastasis) of tumor to other organs

When to Contact a Medical Professional

Call your provider if you have jaundice or other symptoms of cholangiocarcinoma.

References

Blechacz B, Gores GJ. Tumors of the bile ducts, gallbladder, and ampulla. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 69.

National Cancer Institute website. Bile duct cancer (cholangiocarcinoma) treatment (PDQ) – health professional version. www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq. Updated March 14, 2018. Accessed June 11, 2018.

Rajkomar K, Koea JB. Intrahepatic cholangiocarcinoma. In: Jarnagin WR, ed. Blumgart's Surgery of the Liver, Biliary Tract and Pancreas. 6th ed. Philadelphia, PA: Elsevier; 2017:chap 50.

  • Digestive system

    Digestive system

    illustration

  • Bile pathway

    Bile pathway

    illustration

    • Digestive system

      Digestive system

      illustration

    • Bile pathway

      Bile pathway

      illustration

     

    Review Date: 5/14/2018

    Reviewed By: Preeti Sudheendra, MD, oncologist at the MD Anderson Cancer Center at Cooper, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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