Learn About Cholangiocarcinoma

What is the definition of Cholangiocarcinoma?

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

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What are the alternative names for Cholangiocarcinoma?

Bile duct cancer

What are the causes of Cholangiocarcinoma?

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:

  • Bile duct (choledochal) cysts
  • Chronic biliary and liver inflammation
  • History of infection with parasitic worms, liver flukes
  • Primary sclerosing cholangitis
  • Ulcerative colitis
What are the symptoms of Cholangiocarcinoma?

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)
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What are the current treatments for Cholangiocarcinoma?

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.

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.

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What is the outlook (prognosis) for Cholangiocarcinoma?

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

The survival time varies greatly from several months to several years. Outlook depends on:

  • The location of CCA
  • The cell type of CCA
  • The stage of the tumor
  • The person's age and general health
  • Whether surgery is an option
  • The person's response to treatment

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. You and your family may want to start thinking about end-of-life planning, such as:

  • Palliative care
  • Hospice care
  • Advance care directives
  • Health care agents
What are the possible complications of Cholangiocarcinoma?

Complications of CCA include:

  • Infection
  • Liver failure
  • Spread (metastasis) of tumor to other organs
When should I contact a medical professional for Cholangiocarcinoma?

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

Digestive system
Bile pathway
What are the latest Cholangiocarcinoma Clinical Trials?
Quantification of 68-GaNOTA-Anti-HER2 VHH1 Uptake in Metastasis of Breast Carcinoma Patients and Assessment of Repeatability (VUBAR) - Pilot Study

Summary: Study objective: Cohort 1: To quantify the uptake of 68GaNOTA-Anti-HER2 VHH1 in local or distant metastases from breast carcinoma patients and to assess repeatability of the image-based HER2 quantification. The uptake will be correlated to results obtained via biopsy of the same lesion, if available. Cohort 2: To report on uptake of 68GaNOTA-Anti-HER2 VHH1 in different cancer types that might over...

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A Real-world Study of the Safety and Efficacy of Surufatinib in the Treatment of Biliary Tract Carcinoma

Summary: This is a prospective, single-arm, open-label,multi-center, observational real-world clinical study to observe and evaluate the efficacy and safety of Surufatinib in the treatment of patients with biliary tract cancer (BTC).

What are the Latest Advances for Cholangiocarcinoma?
The Efficacy and Safety of Hepatic Arterial Infusion Chemotherapy Based on FOLFIRI for Advanced Intrahepatic Cholangiocarcinoma as Second-Line and Successive Treatment: A Real-World Study.
Comparison of Different First-Line Systemic Therapies in Advanced Biliary Tract Cancer Based on Updated Random Controlled Trials: A Systematic Review and Network Meta-Analysis.
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Interventional treatment of biliary liver disease.
Who are the sources who wrote this article ?

Published Date: April 29, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Gibson RN, Sutherland TR. The biliary system. In: Adam A, Dixon AK, Gillard JH, Schaefer-Prokop CM, eds. Grainger & Allison's Diagnostic Radiology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 24.

National Cancer Institute website. Bile duct cancer (cholangiocarcinoma) treatment (PDQ) -- health professional version. www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq. Updated June 2, 2022. Accessed June 24, 2022.

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.

Rizvi SH, 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. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 69.