Fast Facts for Patients and Supporters: Cholangiocarcinoma: A cancer of the bile duct and liver Information + Taking Control = Best Outcome

By C. Denlinger and K. Spencer

Cholangiocarcinoma (bile duct cancer) is a cancer of the bile duct and liver. A person can seem healthy and/or can have a variety of symptoms, such as weight loss, bloating, abdominal pain, jaundice (yellowing of the skin and/or eyes), itchy skin, dark urine and/or pale stools. The location of the tumor within the liver/bile duct and its size and spread to other areas of the body will determine the cancer treatment plan. Treatment options may include surgery, chemotherapy, radiotherapy, liver transplant, targeted therapies and clinical trials. This book aims to help patients understand their options, enabling informed decisions, in conjunction with their doctor and other members of the medical team, about their cancer treatment. Table of Contents: • Understanding cholangiocarcinoma • How does cholangiocarcinoma develop? • Symptoms • Seeing your doctor • Diagnostic tests and procedures • Staging • Biomarker testing • Multidisciplinary teams and tumor boards • Surgery • Radiation therapy • Systemic therapy • Treatments and schedules • Infusion therapy • Clinical trials • What new treatments are being studied? • Targeted treatments • Taking part in research • Palliative procedures • Stents • Reducing side effects • Follow-up care • Outlook • If treatment is unsuccessful • Coping • Being supported • What can I do to help myself?

• Language: English
• Publisher: S. Karger
• Release date: Apr 8, 2021
• ISBN: 9783318068146

Book preview

Understanding cholangiocarcinoma

Cholangiocarcinoma is a cancer of the body’s hepatobiliary system, which includes the liver, gallbladder and tubes called bile ducts.

The hepatobiliary system makes, stores and transports bile. Bile helps the body break down fat in the diet and get rid of waste from the body.

The liver makes bile, which is collected in small tubes (ductules) and funneled into increasingly larger ducts as it is carried through the liver. As the bile leaves the liver, the duct system merges into left and right hepatic ducts. These two ducts leave the liver and join at the hilum to form the common hepatic duct.

The gallbladder stores bile. It connects to the hepatobiliary system about one-third of the way down the common hepatic duct, via the cystic duct. This duct is now called the common bile duct, and it continues through the pancreas before reaching the small intestine (the destination for the bile).

Knowledge point

Cholangiocarcinoma is also called bile duct cancer.

There are two main types of cholangiocarcinoma: intrahepatic and extrahepatic.

Intrahepatic cholangiocarcinoma occurs in the bile ducts inside the liver.

Extrahepatic cholangiocarcinoma occurs in the bile ducts outside the liver. These drain bile into the small intestine.

A perihilar tumor is an extrahepatic cholangiocarcinoma that develops at or near the junction of the left and right bile ducts (the hilum). These tumors were previously called Klatskin tumors.

A distal tumor develops in the common bile duct.

Cholangiocarcinomas are rare tumors, accounting for only about 3 in every 100 cancers in the gastrointestinal system. Perihilar tumors are the most common type of cholangiocarcinoma (approximately half), followed by distal tumors (approximately 4 in every 10), and intrahepatic tumors (approximately 1 in every 10).

How does cholangiocarcinoma develop?

The starting point for cholangiocarcinoma is a harmful change (mutation) to the DNA in cells in the bile duct. One of the effects is that cells start to grow and divide without the usual controls. Eventually, a mass of cells forms a tumor.

Doctors do not know exactly what causes the changes in the DNA, though some medical conditions and lifestyle factors increase the risk.

Medical conditions and lifestyle factors that increase the risk

• Bile duct: primary sclerosing cholangitis, primary biliary cirrhosis, bile duct stones or cysts

• Digestive system: inflammatory bowel disease (Crohn’s disease or ulcerative colitis), chronic pancreatitis

• Diabetes

• Chronic liver disease: cirrhosis, hemochromatosis, fatty liver disease (non-alcoholic fatty liver disease or NASH), hepatitis B or C

• Obesity

• Smoking

• High alcohol intake

• Infection with liver fluke (a problem in some South-East Asian countries)

Symptoms

The symptoms of cholangiocarcinoma can vary from person to person. Intrahepatic and extrahepatic cholangiocarcinomas may cause different symptoms. If the tumor blocks the