The malignant tumors in cholangiocarcinoma, a type of cancer of the bile duct, are rare and aggressive. While in most cases the causes of the disease have not been identified, it can often be linked to other risk factors such as inflammatory diseases, cirrhosis, or infections.
A rare and aggressive form of cancer
Cholangiocarcinoma is a rare and aggressive cancer that affects approximately 8,000 people in the United States1 and 2,000 in France2. It is more common in regions with a higher incidence of infections caused by a parasite that infects the liver. Thailand has the highest incidence in the world (90 cases per 100,000 people1), followed by China and Korea. The actual number of cases is probably underestimated, however, since this form of cancer is difficult to diagnose. It is sometimes wrongly diagnosed as another type of cancer.1
Surgery is the only potentially curative treatment option, but it is only possible for a limited number of patients and the risk of recurrence remains high. Chemotherapy and immunotherapy are the standard treatments for cholangiocarcinoma in patients who are not eligible for surgery or for whom the disease has progressed after surgery. Developments in immunotherapy and new targeted therapies bring greater hope and improved quality of life for patients.
A cancer of the bile ducts, found inside or outside the liver
Cholangiocarcinoma is a primary cancer that develops in the bile ducts that carry the digestive fluid bile from the gall bladder to the small intestine. There are three main types of cholangiocarcinoma3 depending on the location of the tumor in the biliary system:
Although there are considerable regional variations, data suggests that 50 percent of all cases of cholangiocarcinoma occur as an intrahepatic form and extrahepatic the other 50 percent.4
A cancer often linked to medical history
Several factors increase the risk of developing bile duct cancer. Most often, it involves a medical history of previous disease or inflammation of the bile ducts.5 Other risk factors include age (on average, patients are 70 years old6 at diagnosis), obesity, diabetes,and exposure to certain types of chemicals.
Preventing cholangiocarcinoma
Cholangiocarcinoma can be prevented in part through appropriate lifestyle and health measures. Eating a balanced diet rich in fruits and vegetables, combined with regular physical activity, helps maintain a healthy liver. Similarly, limiting alcohol consumption and avoiding smoking are essential measures that protect the liver.
To prevent chronic infections that may lead to this cancer, it is crucial to get vaccinated against the hepatitis B virus (HBV). Treating any existing hepatitis, especially hepatitis B and C, reduces the risk of liver damage that could develop into cholangiocarcinoma. Travel to endemic areas, such as certain Southeast Asian regions where liver parasites are present, requires special precautions: drinking potable water, consuming food that is cooked properly, and following local health recommendations. By combining these various strategies, it is possible to significantly lower the risk of developing cholangiocarcinoma.5
How cholangiocarcinomas are diagnosed
Bile duct cancer is often diagnosed late because many patients display no signs or symptoms. In fact, the first signs of the disease 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) in the case of extrahepatic cholangiocarcinoma. Other symptoms may appear, such as itching, pale stools, dark urine, abdominal pain, weight loss, and fever. However, these symptoms are not specific to the disease and may lead to a delayed cancer diagnosis.2
Cholangiocarcinoma diagnosis is complex and typically requires several examinations:
As part of diagnostic testing for cholangiocarcinoma, molecular tests are also performed on liver or blood samples. Indeed, approximately 40% of patients with cholangiocarcinoma have mutations that are actionable with targeted therapies8.
What are the possible treatment options for cholangiocarcinoma?
Surgical resection is the only potential option for curing cholangiocarcinoma. A full liver transplant may also be considered. It is also recommended that jaundice be treated at the same time as the cancer.
If surgery is not possible, for example when cancer has spread to other organs, it may be treated through immunotherapy or targeted therapies. These treatments can slow or even control progression of the disease.
When combined with chemotherapy, immunotherapy – the aim of which is to boost the body’s natural defenses to fight cancer – brings new hope for cholangiocarcinoma patients. Given the aggressive nature of this disease and the lack of therapeutic options, new treatments (other combinations or molecules) are being developed.
Over the past few years, a number of targeted therapies have been approved. These therapies target the molecular modifications or surrounding tissue that enable the cancer to survive and proliferate. Such therapies block cancer cells from proliferating without damaging healthy cells and are intended for patients in whom a targetable mutation has been identified.
AND SERVIER?
In oncology, our goal is to develop precision therapies that aim to transform the lives of patients affected by rare cancers and in areas with major unmet medical needs. We are a focused and innovative player, developing treatments for digestive cancers (colorectal cancer, gastric cancer, pancreatic cancer, cholangiocarcinoma), brain tumors (glioma), and hematologic cancers (acute myeloid leukemia, acute lymphoblastic leukemia, lymphomas).
We are focusing our R&D programs on two approaches: immuno-oncology and targeted therapies.
WHAT YOU NEED TO KNOW
Frequently Asked Questions (FAQ) – Cholangiocarcinoma
Cholangiocarcinoma is a rare and aggressive cancer of the bile ducts, the tubes that carry bile from the liver to the intestine. It can develop inside the liver (intrahepatic) or outside the liver (extrahepatic).
Diagnosis is often delayed because early symptoms are non-specific (fatigue, abdominal pain, weight loss). More suggestive signs, such as jaundice, generally appear when the tumor is already in advanced stages.
Cholangiocarcinoma is frequently associated with a history of bile duct diseases or inflammation, cirrhosis, or certain parasitic infections. Age, obesity, diabetes, alcohol consumption, and exposure to certain chemicals can also increase the risk.
Diagnosis is based on a combination of examinations: blood tests, medical imaging (ultrasound, CT scan, MRI), biopsy, and sometimes endoscopy. Molecular tests are also performed, as approximately 40% of patients have genetic mutations that can be targeted by specific treatments.
Surgery is the only potentially curative treatment, but it is only possible for a limited number of patients. When surgery is not an option, treatments such as chemotherapy, immunotherapy, or targeted therapies may be offered to slow the progression of the disease.
Sources :
[1] American Cancer Society / Key Statistics for Bile Duct Cancer / https://www.cancer.org/cancer/types/bile-duct-cancer/about/key-statistics.html
Last revised on October 11, 2024
[2] AFEF, Société Française d’Hépatologie [French Hepatology Society]: https://afef.asso.fr/cholangiocarcinome/
[3] Cholangiocarcinoma Foundation / https://cholangiocarcinoma.org/defined/https://cholangiocarcinoma.org/learn-about-cholangiocarcinoma / viewed on November 28, 2024
[4] American Cancer Society / Global Trends in Intrahepatic and Extrahepatic Cholangiocarcinoma Incidence From 1993 to 2012 / https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.32803
[5] American Cancer Society / https://www.cancer.net/cancer-types/bile-duct-cancer-cholangiocarcinoma/risk-factors-and-prevention / viewed on December 4, 2024
[6] Cholangiocarcinoma Foundation / https://cholangiocarcinoma.org/key-statistics/
[7] Bile Duct Cancer. American Cancer Society. [En ligne] 11 October 2024. https://www.cancer.org/cancer/types/bile-duct-cancer.html.
[8] EASL-ILCA Clinical Practice Guidelines on the management of intrahepatic cholangiocarcinoma. Al, Domenico Alvaro et. s.l.: Journal of Hepatology, 2023.