Cholangiocarcinoma is the type of bile duct cancer majorly forming in the bile ducts. Approximately 0.5–2 individuals per 100,000 each year are affected by Cholangiocarcinoma, mainly in the Western region. The individuals belonging to the age group of 70 and 40 are affected primarily by Cholangiocarcinoma. Abdominal pain, yellowish skin, weight loss, generalized itching, and fever are the common symptoms in the case of Cholangiocarcinoma. Other types of bile duct cancer include gallbladder cancer and cancer of the ampulla of Vater. Primary sclerosing cholangitis, ulcerative colitis, cirrhosis, hepatitis C, hepatitis B, infection with certain liver flukes, and some congenital liver malformations are the significant risk factors for Cholangiocarcinoma. Its diagnosis is made by conducting blood tests, medical imaging, endoscopy, and sometimes surgical exploration. The standard treatment for Cholangiocarcinoma includes surgical resection, chemotherapy, radiation therapy, and stenting techniques in palliative treatment.
What is Cholangiocarcinoma?
Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the bile ducts. Symptoms of cholangiocarcinoma may include abdominal pain, yellowish skin, weight loss, generalized itching, and fever. Light coloured stool or dark urine may also occur. Other biliary tract cancers include gallbladder cancer and cancer of the ampulla of Vater 1.
Risk factors for cholangiocarcinoma include primary sclerosing cholangitis (an inflammatory disease of the bile ducts), ulcerative colitis, cirrhosis, hepatitis C, hepatitis B, infection with certain liver flukes, and some congenital liver malformations 2. However, most people have no identifiable risk factors. The diagnosis is suspected based on blood tests, medical imaging, endoscopy, and sometimes surgical exploration. The disease is confirmed by examining cells from the tumor under a microscope. It is typically an adenocarcinoma (Cancer that forms glands or secretes mucin).
Cancer develops when the body’s cells begin to grow out of control. Cancerous cells can arise in practically any part of the body and spread to other parts of the body. To gain a better understanding of cancer and how it begins and spreads. Bile duct cancer, also known as cholangiocarcinoma, starts in a bile duct. To understand this cancer, it helps to know about the bile ducts and what they normally do.
About the bile ducts
The bile ducts connect the liver to the small intestine by a network of tiny tubes. Their major function is to let bile from the liver and gallbladder flow into the small intestine, where it aids in the digestion of lipids in the diet.
The bile duct system is divided into several sections, each with its own name. It starts as a tangle of small tubes in the liver (called ductules). The ductules join together to produce ducts, which are tiny tubes. These connect to form bigger ducts, which then connect to form the left and right hepatic ducts. Intrahepatic bile ducts refer to all of the ducts within the liverThe left and right hepatic ducts exit the liver and join to form the common hepatic duct in an area called the hilum. Lower down, the gallbladder (a small organ that stores bile) is joined to the common hepatic duct by a small duct called the cystic duct. This combined duct is called the common bile duct.
The common bile duct passes through part of the pancreas before it joins with the pancreatic duct and empties into the first part of the small intestine (the duodenum) at the ampulla of Vater.
Cancers of the bile duct are classified according to their location.
Bile duct cancer (cholangiocarcinoma) can start in any part of the bile duct system. Based on where the cancers start(see the picture below), they’re grouped into 3 types:
- Intrahepatic bile duct cancers
- Perihilar (also called hilar) bile duct cancers
- Distal bile duct cancers
Cancers of the bile duct in the intrahepatic bile duct
These tumors begin in the liver’s smaller bile duct branches. They’re sometimes mistaken for malignancies that start in the liver cells (hepatocellular carcinomas), and they’re treated similarly. malignancies of thee duct by location
Bile duct tumours in the perihilar (also known as hilar) region
These tumours begin in the hilum, the junction of the left and right hepatic ducts as they leave the liver. These tumours are also known as Klatskin tumours. Extrahepatic bile duct malignancies include these cancers as well as distal bile duct cancers.
Cancers of the distal bile duct
These tumours are situated near the small intestine, further down the bile duct. These are extrahepatic bile duct cancers, like perihilar malignancies, because they begin outside of the liver.
Cancers that begin in various regions of the bile ducts can produce a variety of symptoms.
Types of bile duct cancer by cell type
Bile duct cancers can also be divided into types based on how the cancer cells look under the microscope.
Nearly all bile duct cancers are cholangiocarcinomas. These cancers are a type of adenocarcinoma, which is a cancer that starts in gland cells. Cholangiocarcinomas start in the gland cells that line the inside of the ducts.
Other types of bile duct cancers are much less common. These include sarcomas, lymphomas, and small cell cancers. Our information does not cover these rare types of bile duct cancer.
Tumors of the bile duct that are benign
Bile duct tumours aren’t always cancerous. Bile duct hamartomas and bile duct adenomas, for example, are benign (non-cancerous) tumours.
Other cancers in the liver
The most common type of cancer that starts in the liver – much more common than cholangiocarcinoma – is hepatocellular carcinoma. This type of cancer starts in the main cells that make up the liver.
Cancers that start in other organs, such as the colon or rectum, can sometimes spread (metastasize) to the liver. These metastatic cancers are not true liver cancers. For example, colorectal cancer that has spread to the liver is still colorectal cancer, not liver cancer. The treatment and outlook for cancer that metastasizes to the liver are not the same as for cancer that starts in the liver. For this reason, it’s important to know whether a tumor in the liver started in bile ducts (cholangiocarcinoma), the liver tissue itself (hepatocellular carcinoma), or another organ and has spread to the liver.
Blood testing, medical imaging, endoscopy, and sometimes surgical investigation are used to suspect the diagnosis 3. A microscope study of cells from the tumour confirms the diagnosis. Adenocarcinoma is the most common kind (Cancer that forms glands or secretes mucin). At the time of diagnosis, cholangiocarcinoma is usually incurable. In certain circumstances, surgical resection, chemotherapy, radiation therapy, and stenting techniques may be palliative treatments. The tumour can be eliminated by surgery in roughly a third of instances involving the common bile duct and less commonly in other places, providing a chance of cure.
Also Read: Dietary Approaches
Chemotherapy and radiation therapy are usually necessary even if surgical removal is successful. Surgery may include liver transplantation in some cases. Even if surgery is successful, 5-year survival rates are generally less than 50%.
Cholangiocarcinoma is uncommon cancer in the Western world, affecting just 0.5–2 individuals per 100,000 each year 4. In Southeast Asia, where liver flukes are widespread, rates are more significant. In some places in Thailand, the annual rate is 60 per 100,000. It usually affects people in their 70s, but it can also affect persons in their 40s who have primary sclerosing cholangitis. In the Western world, This cancer type the liver has become more common.
- 1.Razumilava N, Gores GJ. Cholangiocarcinoma. The Lancet. Published online June 2014:2168-2179. doi:10.1016/s0140-6736(13)61903-0
- 2.Khan SA, Tavolari S, Brandi G. Cholangiocarcinoma: Epidemiology and risk factors. Liver Int. Published online March 24, 2019:19-31. doi:10.1111/liv.14095
- 3.Blechacz B, Gores GJ. Cholangiocarcinoma: Advances in pathogenesis, diagnosis, and treatment. Hepatology. Published online July 2008:308-321. doi:10.1002/hep.22310
- 4.Bergquist A, von Seth E. Epidemiology of cholangiocarcinoma. Best Practice & Research Clinical Gastroenterology. Published online April 2015:221-232. doi:10.1016/j.bpg.2015.02.003