Cholangiocarcinoma, also known as bile duct cancer, is a rare and aggressive tumor. It is often associated with medical history such as cirrhosis or infections of the liver. Surgery is the only treatment with curative intent but is only available for a small number of patients, and the risk of recurrence is high. Chemotherapy is the standard treatment for patients with inoperable cholangiocarcinoma, or for those whose cancer has progressed post-surgery. Developments in immunotherapy and new targeted drug therapies bring greater hope and better quality of life for patients.
Intrahepatic or extrahepatic primary bile duct cancer
Cholangiocarcinoma can occur either inside or outside the liver. It is a primary cancer, rather than a metastatic cancer that has spread from the primary tumor to another organ.1 It develops in the bile ducts that carry the digestive fluid bile from the vesicle to the small intestine. There are three main forms of cholangiocarcinoma 2:
- Intrahepatic cholangiocarcinoma, which occurs inside the liver in the small or medium bile ducts.
- Hilar or perihilar extrahepatic cholangiocarcinoma, which occurs where the right and left hepatic ducts join and leave the liver up to the point they meet the gall bladder.
- Distal cholangiocarcinoma, also known as extrahepatic cholangiocarcinoma, which occurs outside the liver after the hepatic bile ducts have joined to form the common bile duct.
There is considerable regional variation, but the latest data suggest that localization of the cholangiocarcinoma is found more or less half the time being intrahepatic and half as extrahepatic. 3
A rare and aggressive form of cancer
Cholangiocarcinoma is considered a relatively rare cancer. In Western Europe, North America and Australasia, estimates of occurrence vary between 0.3 and 3.5 cases per 100,000 every year. To give an example, this prevalence would amount to around 8,000 cases in the United States and 2,000 in France.4
Cholangiocarcinoma is more common in regions with a higher incidence of infections caused by a liver parasite. Thailand (90 cases per 100,000 population) has the highest incidence in the world,4 followed by China and Korea.
The actual number of cases is probably higher, since this form of cancer is hard to diagnose. It is sometimes wrongly diagnosed as another type of cancer.5
Often linked to medical history
Several factors increase the risk of developing bile duct cancer. Frequently, they are related with a history of previous disease or irritation of the bile ducts.6 Age (patients have an average age of around 70 years 7), obesity, diabetes, and exposure to certain types of chemicals are also risk factors.
The main preventive measures are avoiding alcohol abuse and exposure to dangerous chemicals. And if travelling in regions where parasitic infections are common, it is important to only drink purified water and to make sure food has been thoroughly cooked.
Cholangiocarcinoma is extremely aggressive. Fewer than 10% of patients survive five years after diagnosis. Survival is even lower for patients diagnosed at a metastatic stage.8
Diagnosis: imaging and biopsy
Diagnostic tests depend on the signs and symptoms displayed, age, general health, as well as the results of previous tests. Bile duct cancer is often diagnosed late because many patients display no symptoms of the disease. The first signs are often non-specific and appear when the tumor is large and compresses the large bile ducts, which can cause jaundice (yellowing of the skin and whites of the eyes) in extrahepatic cholangiocarcinoma. However, the intrahepatic form may be found by chance in X-rays or MRI taken for other reasons, or show up as an anomaly in blood tests, or because bile secretion is reduced or stops altogether. Unfortunately, there are no reliable biological markers specific to these bile duct cancers.
Imaging alone (ultrasound, scan or MRI) locates the tumor and shows whether it has spread to other organs. It can also help determine if the tumor is operable (i.e. whether it is resectable or unresectable).9 10 Next, if a tumor is suspected, a biopsy may be performed to remove a fragment of tissue for analysis (histology) and to provide more information on the tumor.
Surgery dominates treatment options
Surgical resection is the only potential option for curing cholangiocarcinoma. If surgery is not possible, palliative chemotherapy or radiation therapy may be offered. A full liver transplant may be an option for some people. It is also recommended to treat the jaundice at the same time as the cancer.
New therapies are being developed for this aggressive disease with currently limited treatment options available. The past few years have seen a number of targeted therapies approved by regulators. These therapies target the genes, proteins or surrounding tissue that help the cancer to survive and grow. They block cancer cells from proliferating but without damaging healthy cells.
Immunotherapy aims to boost the body’s natural defenses to fight the cancer and also brings new hope and quality of life for cholangiocarcinoma patients.
Et Servier ?
Servier wants to become a recognized innovative player in treatments for cancer by discovering new ways to address unmet needs in difficult to treat areas, such as gastrointestinal, hematologic, pancreatic and pediatric cancers.
Over 50% of our R&D budget is dedicated to oncology.
WHAT YOU NEED TO KNOW
Cholangiocarcinoma is a rare and aggressive bile duct cancer.It is often diagnosed late as the initial signs are non-specific.
Its occurrence is frequently linked to a historyof bile duct disease or irritation, or to parasitic infections. Obesity and alcohol abuse are risk factors.
Surgical resection is the only potential option for curing cholangiocarcinoma.Targeted therapies and immunotherapy represent new hope for patients.
4 The European Society for Medical Oncology (ESMO) issued new guidelines for biliary cancers http://www.sirtex.com/eu/media/news/news-item?id=25844
5 American Cancer Society. Key Statistics for Bile Duct Cancer. See the website: https://www.cancer.org/cancer/bile-duct-cancer/about/key-statistics.html
8 – Cholangiocarcinoma: classification, diagnosis, staging, imaging features, and management – Springer Science+Business Media New York 2017