The liver is the largest organ inside the human body. It is football sized and sits in the upper right part of the abdomen, beneath the diaphragm and above the stomach. The liver is very busy and has more than 500 jobs. Compared to other organs, the liver has an exceptional capacity to repair itself, or regenerate, when injured or damaged.

Cholangiocarcinoma is a rare type of cancer that develops in the bile ducts. There are bile ducts inside the liver and when cancer develops in these it is called intrahepatic cholangiocarcinoma. When bile ducts outside of the liver develop cancer this is called extrahepatic cholangiocarcinoma.

Extrahepatic cholangiocarcinomas are sometimes divided into peri-hilar or hilar cholangiocarcinomas which are in the bile ducts right close to the liver, and distal cholangiocarcinomas which are in the section of the bile ducts closer to where the bile ducts drain into the small intestine.

To learn more about the liver and it’s functions visit – Liver Cancer Lessons

If you or a loved one has been diagnosed with cholangiocarcinoma, take a moment to breathe. After that, your priority is to find out as much as you can about your diagnosis and work with your doctor to make a plan that is right for you or your loved one.

Risk for Cholangiocarcinoma

The liver’s regenerative capacity usually lasts a lifetime but in cases where there is ongoing chronic injury for an extended period of time, the liver exhausts its capacity to repair itself. Once the liver reaches this state of repair exhaustion, further damage results in the replacement of the liver hepatocytes with scar tissue, also called fibrosis, rather than normal functioning liver cells. This makes it harder for the liver to perform its normal functions. When the scar tissue eventually replaces large portions of the liver, it is described as cirrhosis of the liver. Cirrhosis is a potentially life threatening condition.

Some risk factors, like smoking, can be changed while others, like gender, cannot. 

  • Cirrhosis – is severe scarring of the liver.  Cirrhosis is the result of repeated injury and inflammation and can be caused by several different diseases and conditions.  If the cause of the liver injury is reduced or eliminated, it is possible for the liver to slowly repair itself.

  • Primary sclerosing cholangitis (PSC) – is a condition that causes inflammation of the bile ducts and leads to the development of scar tissue, or sclerosis. 

  • Inflammatory Bowel Disease (IBD) – is frequently associated with PSC, which is a major risk factor for cholangiocarcinoma

  • Age – the average age of diagnosis for intrahepatic cholangiocarcinoma is 70 and 72 for extrahepatic cholangiocarcinoma

  • Bile Duct Stones – are very small stones that can cause inflammation in the bile ducts.

  • Choledochal Cysts – this is a very rare congenital (present at birth) condition in which bile filled sacs (choledochal cysts) that are connected to the bile duct often have areas of pre-cancerous growth.

  • Family History – if there is a family history of cholangiocarcinoma there is a slightly increased risk of developing cholangiocarcinoma. Most cholangiocarcinoma is found in people with no family history. People with hereditary non-polyposis colon cancer syndrome (also called Lynch syndrome), in which the ability of the body to repair genetic material or DNA is compromised, are also at higher risk of developing cholangiocarcinoma.

  • Viral Hepatitis – ongoing infection with hepatitis B virus (HBV) or hepatitis C virus (HCV) can lead to the development of chronic hepatitis and cirrhosis.

  • Alcohol and Alcohol Related Cirrhosis – over time, excessive alcohol use can damage and scar the liver leading to cirrhosis

  • Tobacco – smoking increases the risk of liver cancer

  • Liver Flukes – are parasites that are most commonly found in certain Asian countries. Flukes are acquired from eating certain raw or incompletely cooked fish and live in the bile ducts, causing inflammation and scar tissue formation. The most common flukes are Opisthorchis viverrini, Clonorchis sisensis, Schistosomiasis japonica

Staging for Cholangiocarcinoma

Once a diagnosis of cholangiocarcinoma has been made additional tests will be done to determine if the cancer has spread and if so how far. This process, called staging, helps the treatment team understand how much cancer is in the body and helps to inform discussions about treatment options.

The most commonly used system is the TNM system from the  American Joint Committee on Cancer (AJCC). TNM stands for Tumor Node Metastasis and the system classifies cancer stage and defines the extent of the cancer including size, location and number of tumors. TNM Stages are I, II, III, IV.

Cholangiocarcinoma Treatment

Treatment for cholangiocarcinoma will depend upon several factors including the stage of disease. There are multiple treatment options available including:

  • Surgery

  • Radiation

  • Chemotherapy

  • Targeted Therapy

  • Immunotherapy

To learn more about cholangiocarcinoma, treatment options, treatment side effect management and promising research visit our partners and trusted resources.

Supportive (Palliative) Care

Palliative care is supportive care that aims to help a person maintain a high quality of life by managing the symptoms of cancer rather than cure the cancer.

There are many specialties that make up a palliative care team. These may include medical professionals that provide physical, emotional, cultural, spiritual and social support to meet a person’s needs. They also provide support to families and caregivers.

Many people believe that palliative care is hospice. The two are similar because their goal is to provide comfort and care to people living with an illness. You can have palliative care at any age and at any stage of your illness.  Hospice is medical care to help someone with a terminal illness live well for as long as possible.

Clinical Trials

Clinical trials are research studies that involve people. Through clinical trials, new treatments and ways to improve liver cancer care are moved forward. Clinical trials also determine if treatments are safe and effective in the treatment of liver cancer.

 Clinical trials evaluate:

  • New diagnostic methods

  • New drugs and medicines

  • New combinations of treatments

  • New approaches to surgery or radiation

Clinical trials are highly controlled and regulated. They can be sponsored by the federal government, medical institutions, pharmaceutical companies, or private foundations.

Survivorship in Liver Cancers

Once treatment is completed ask about a treatment summary as a way to record the exact diagnosis, anti-cancer treatments and other procedures and tests that were a part of the liver cancer journey. This can become a helpful tool for any doctors that provide care in the future.

Your journey is uniquely yours and liver cancer can be a difficult disease to treat. Consider seeking support to help you and your loved ones navigate the challenges.