Cholangiocarcinomas (bile duct cancer)

Cholangiocarcinoma is the second most common primary cancer of the liver, after hepatocellular carcinoma (HCC). It arises from cells lining the bile ducts (cholangiocytes). 

They can occur within the liver (intrahepatic) or near the liver hilum (perihilar) or beyond the liver (extrahepatic or distal). Although they are rare, these cancers are highly lethal because most are diagnosed at advanced stages. Even in early stages, post-resection their rates of recurrence remain significant. 

CCA is categorised into three types: 

Other common symptoms include: 

Intrahepatic CCAs may present differently as the tumour is in the liver. Affected patients are less likely to be jaundiced. Some patients will have mild or no symptoms, and often only develop symptoms in the advance stage. 

Advanced-stage symptoms include: 

These are some other inflammatory conditions that can increase the risk of developing CCA: 

In some patients, CCA can lead to the formation of a tumour that blocks the bile duct, resulting in the bile drainage system (cholangitis) getting infected. 

Additionally, CCA may lead to the development of cirrhosis and result in scarring and damage to the liver cells, particularly patients with primary sclerosing cholangitis.

Ultrasound 

An ultrasound uses high-frequency sound waves to scan, carries no radiation risk and can be done on a regular basis, especially in individuals who are at risk of cancer, for e.g. hepatitis C carriers. It is however not always accurate and or very specific and sometimes further CT or MRI scans may be indicated for better characterisation of the tumour. 

CT scan 

The CT scan is an x-ray procedure that produces detailed cross-sectional images of the body from different angles. It is a better way of detecting CCA and is crucial for treatment planning. In certain cases, a CT scan may not be enough or is inconclusive, and additional investigations like MRI / MRCP or a PET scan may be performed. 

Blood test 

A blood test that measures the tumour markers called Carbohydrate antigen 19–9 (CA 19-9) can indicate an association with CCA but it should not be used or as a routine screening test. Other tumour markers that are associated are carcinoembryonic antigen (CEA) and CA-125. 

A blood test alone is usually not diagnostic and a further tissue sample or biopsy, i.e. a small tissue or cells sample from the bile duct may be collected through a needle procedure or via endoscopy brushing. 

Other scans or investigations may be required if necessary for diagnosis, staging and/or to guide treatment strategy. 

What are the stages of Cholangiocarcinomas (Bile Duct Cancer)? 

Staging helps your medical team to assess the extent of the cancer in your body, plan treatment and determine your prognosis. Staging guidelines differ depending on the type of CCA. In general, CCA staging factors include: 

Early-stage CCA (stages 1 and 2) is often resectable, meaning it can be removed surgically. Stage 3 usually indicates the cancer has spread to nearby structures and/or lymph nodes. Stage 4, the most advanced, involves metastasis to distant organs and is typically unresectable. In such cases, treatment focuses mainly on palliative care to relieve symptoms and improve quality of life.

The main types of treatment for bile duct cancer include: 

Surgery 

Surgery is often the best treatment for CCA when it is at an early stage and has not spread to nearby blood vessels or distant organs. Other methods have not been shown to be as effective as surgery in treating CCA. However, due to its advanced stage at first presentation, surgery may not be possible for some patients. 

As surgery is the only effective method for treating CCA, it is important that a person with suspected CCA has a proper assessment by a specialist team. The specialist team is led by a liver surgeon known as a Hepato-Pancreato-Biliary (HPB) surgeon. When feasible, surgery is a safe and effective in treating CCA. 

Depending on the location of the cancer, surgery for CCA includes cutting out the tumour (Liver resection) or Enbloc removal of the bile duct and its surrounding organs and lymph nodes (e.g. Pancreaticoduodenectomy or also known as a "Whipple" procedure or Radical bile duct resection). 

A select group of Intrahepatic CCA patients may be suitable for a liver transplantation. 

Clinical trials test new drugs, surgical techniques and strategies for advancing treatment and care. There may be suitable clinical trials available for selected patients. Please consult your doctor for advice.

Before surgery, the anaesthesia team will also assess your fitness for surgery and advise you on various aspects of general anaesthesia and pain control after surgery. 

Specialist nurses are available to offer pre-surgery counselling.

It is important to follow your doctor's advice, attend clinic visits and tests so that timely treatment can be administered if the cancer or other issues arise.

Bile duct cancer is curable if it is detected early and is suitable for surgical removal. In some cases, a liver transplant may be a potential option for curing CCA. However, this is uncommon as bile duct cancer is often diagnosed at a later stage, when it has already spread to other organs. At that point, surgery alone is no longer a curative option. 

The prognosis for CCA is generally poor. The 5-year survival rate for CCA cases that has not spread outside of the bile ducts ranges from 15 - 20%. If the cancer has spread beyond the bile ducts or is diagnosed at an advanced stage, the five-year survival rate drops to less than 5%. 

Despite the dismal prognosis, there are many new treatment options that are now available such as advancements in molecular profiling and targeted therapies. It is important to note that survival statistics are based on data from past years and may not reflect the improved outcomes associated with these newer treatment options. 

Patients should discuss prognosis and treatment choices with their healthcare provider or oncologist, taking into account the diagnosis, stage, type of CCA as well as molecular profiling. 

In the final stages of bile duct cancer, when chemotherapy is no longer effective or the patient is unfit for treatment, patients are usually quite frail and weak. Common symptoms associated at this stage include poor appetite, reduced mobility (patients are often bed-bound), jaundice due to liver failure, swelling in the abdomen and lower limbs, pruritus (itchy skin), recurrent fevers and other symptoms related to the spread of disease. Patients eventually decline from organ failure.