What is Hilar cholangiocarcinoma?
Klatskin tumour, also known as hilar cholangiocarcinoma, is a rare bile duct cancer that develops where the bile ducts meet outside the liver. The bile ducts transport bile from the liver to the small intestine, aiding digestion.
Klatskin tumour is a rare condition, affecting approximately 1 in 100,000 people, predominantly occurring in older adults aged 50 to 70, with men being slightly more affected than women.
What is Cholangiocarcinoma?
Cholangiocarcinoma is a rare cancer that starts in the bile ducts, which are small tubes that carry bile from the liver to the intestine. It can grow inside or outside the liver. This cancer is serious and often found late, making treatment harder.
What is Bile?
The liver produces bile, a yellow-green fluid that is kept in the gallbladder. It facilitates the digestion and absorption of fats by breaking them down into smaller pieces. Additionally, bile aids in the body’s absorption of fat-soluble vitamins, including A, D, E, and K. Additionally, it plays a role in removing waste products, such as cholesterol and toxins, from the body through the digestive system.
Disease Causes
There is no well-known cause, however following factors can increase the risk of development of Hilar cholangiocarcinoma.
- Chronic or long-term liver disease (Liver cirrhosis)
- Chronic bile duct infections (such as parasite infection)
- Hepatitis B or C infections
- Excessive consumption of alcohol
- Stones in the bile duct
Signs and Symptoms
Because Klatskin tumours block the bile ducts, symptoms may include:
- Yellowing of the skin and eyes (jaundice).
- Pain in the abdomen (especially in the upper right side).
- Weakness
- Fatigue
- Unexplained weight loss.
- Nausea
- Vomiting
Diagnosis
To diagnose Klatskin tumours, doctors use several tests including::
- Blood Tests: These check for elevated liver function levels and the presence of the CA 19-9 tumour marker, which may indicate cancer.
- Imaging Scans: CT scans, MRIs, and specialized bile duct imaging techniques like MRCP (Magnetic Resonance Cholangiopancreatography) and ERCP (Endoscopic Retrograde Cholangiopancreatography) help visualize the tumour.
- Biopsy: A small tissue sample may be taken to confirm cancer. However, doctors often avoid needle biopsies since they can risk spreading cancer and may disqualify a patient from a liver transplant.
Treatment Options
The best treatment depends on how far the cancer has spread. Here are some options doctors may consider:
- Surgery (if the cancer hasn’t spread too much): Doctors may remove the affected part of the liver, including some bile ducts and nearby lymph nodes. This is called a hepatectomy.
- Biliary Drainage: If the cancer blocks bile flow, it can cause yellowing of the skin (jaundice). This procedure helps drain bile properly, relieving symptoms.
- Liver Transplant (for early-stage cases): In some cases, the damaged liver is completely replaced with a healthy one from a donor. Before this, chemotherapy or radiation may be used to shrink the cancer.
- Chemotherapy & Radiation (when surgery isn’t possible):
- Chemotherapy: Special medications are used to slow or stop the growth of cancer cells.
- Radiation Therapy: High-energy beams target the tumour to shrink it and relieve symptoms.
- Immunotherapy (for advanced cases): This treatment helps the body’s immune system recognize and fight cancer cells.
- Targeted Therapy: Some cancers rely on specific proteins to grow. This treatment blocks those proteins, slowing the cancer’s spread.
Each person’s treatment plan is unique, depending on their health and how advanced the cancer is. The goal is always to either remove the cancer, slow its growth, or improve quality of life.
Prognosis
Klatskin tumours are often diagnosed at a late stage, making them challenging to treat. Only about 9% of patients with bile duct cancer survive beyond five years. The best hope for long-term survival comes with early detection, followed by either surgery or a liver transplant
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