Cholangiocarcinoma (Bile Duct Cancer) Overview

Cholangiocarcinoma is an uncommon cancer that develops in the bile ducts, small tubes that carry digestive fluid (bile) from the liver to the gallbladder and small intestine. As food moves through the gastrointestinal tract, bile is released to aid digestion and promote the absorption of fats.

Cholangiocarcinoma is classified based on its site of origin. The most common type is perihilar (hilar) bile duct cancer, which develops in the hilum, the point where the left and right hepatic ducts join to exit the liver. Other types include intrahepatic bile duct cancer, which forms inside the liver, and distal bile duct cancer, which forms near the small intestine.

Most cholangiocarcinoma tumors are further classified as adenocarcinomas, which originate in the mucus-producing cells that line the inside of the bile ducts. Other, less common classifications include sarcomas, lymphomas and small cell cancers.

Accounting for less than 3% of all gastrointestinal cancers, cholangiocarcinoma is relatively rare overall, with approximately 1 to 2 cases diagnosed per 100,000 people annually.

What causes cholangiocarcinoma?

Cholangiocarcinoma is caused by harmful DNA changes in the cells that line the bile ducts. These mutations disrupt cellular function, causing the cells to multiply rapidly and live beyond their normal lifespan. Excess cells then build up, bind together and form tumors. Scientists do not yet fully understand the precise mechanisms behind the genetic changes that lead to the development of bile duct cancer.

What are the risk factors for cholangiocarcinoma?

Known risk factors for bile duct cancer include:

• Bile duct malformations (choledochal cysts)
• Chronic bile duct inflammation
• Primary sclerosing cholangitis
• Bile duct stones
• Cirrhosis
• Hepatitis B and C
• Parasitic liver infections, such as liver flukes
• Exposure to certain chemicals, such as those used in the rubber or wood-finishing industries
• Smoking and other forms of tobacco use
• Obesity and excess body weight

What are the signs and symptoms of cholangiocarcinoma?

Early-stage bile duct cancer may not have noticeable symptoms. As the tumor progresses, it may cause:

• Yellowing of the skin and eyes (jaundice)
• Dark urine
• Pale stools
• Itchy skin
• Abdominal pain
• Loss of appetite
• Unintended weight loss
• General fatigue.

How is cholangiocarcinoma diagnosed?

Usually, bile duct cancer is diagnosed through a combination of lab and imaging tests. Common diagnostic tools include:

• Blood work to check liver function and measure the levels of certain tumor markers, such as carbohydrate antigen 19-9 (CA 19-9), in the blood
• Ultrasound scans
• Computed tomography (CT) scans
• Magnetic resonance imaging (MRI) scans

The definitive diagnostic test for cholangiocarcinoma is a biopsy, which involves taking a small sample of suspicious tissue for microscopic examination by a pathologist, who can identify cancerous cells. Additionally, specialized procedures, such as endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC), may be used to help the physician get a detailed view of the bile ducts and guide the biopsy.

How is cholangiocarcinoma treated?

Bile duct cancer treatment can vary based on the location and stage of the tumor and the patient’s overall health. Common options include:

• Surgery – If the cancer is localized and operable, surgery may involve removing parts of the affected bile duct, liver and some nearby tissues to eliminate the tumor.
• Chemotherapy – Chemo may be used to shrink the tumor before surgery, treat an inoperable tumor or reduce the risk of recurrence after surgery.
• Radiation therapy – High-energy beams may be precisely delivered to the tumor site to target and destroy cancerous cells, either as a primary treatment or combined with surgery and chemotherapy.
• Targeted therapy – Specialized drugs may be designed to focus on the specific genetic mutations or proteins known to fuel the growth of the tumor.
• Liver transplantation – A liver transplant may be an option if the cancer is confined to the liver and bile ducts.

Benefit from world-class care at Moffitt Cancer Center

Moffitt takes a multispecialty approach to cancer care. We designed our Gastrointestinal Oncology Program to provide our patients convenient access to a comprehensive range of diagnostic, treatment and supportive services in a single location.

At Moffitt, each patient is evaluated, treated and monitored by a collaborative team of highly experienced and specialized professionals, including board-certified surgeons, medical oncologists, radiation oncologists, radiologists, endocrinologists, pathologists, dietitians, social workers and oncology nurses. We ensure that each patient benefits from multiple expert opinions at every phase of their cancer journey.

As the only National Cancer Institute-designated Comprehensive Cancer Center based in Florida, Moffitt has also received widespread acclaim for its groundbreaking research. Through our robust clinical trials program, we offer our patients unique opportunities to be among the first to benefit from promising new cholangiocarcinoma treatments, such as anti-angiogenesis therapies, epidermal growth factor receptor (EGFR) inhibitors, photodynamic therapies (PDT), molecular therapies, immunotherapies, hyperthermia therapies and radiosensitizers.

For these reasons and more, Moffitt is redefining the way bile duct cancer is treated. If you would like to learn more about cholangiocarcinoma, you can request an appointment with a specialist at Moffitt by calling 1-888-663-3488 or submitting a new patient registration form online. We do not require referrals.

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