Cholangiocellular carcinoma (CCC) is a rare cancer that arises from the small bile ducts in the liver. The average incidence rate is 2 per 100,000 inhabitants per year, but there has been a continuous increase in the number of new cases and mortality rate in Western countries over the last two decades. The occurrence of CCC is sporadic, and its exact cause is not yet known, but there are several risk factors associated with its development.
Sclerosing cholangitis increases the risk of CCC. Chronic infection of the bile ducts due to long-term presence of gallstones in the liver, chronic viral hepatitis B and C, and parasitic infestations in the bile ducts (especially in Far East countries) are predisposing factors. Recent literature suggests that diabetes and obesity are also linked to CCC.
Symptoms and signs of CCC include upper abdominal pain, night sweats, weight loss, yellow skin discolouration, itching, dark urine, and pale stools.
The diagnosis is usually made incidentally during an ultrasound examination, CT scan, or MRI. Patients typically have elevated levels of the tumour marker CA 19-9. A definitive diagnosis is established through pathohistological examination.
Surgical resection of the liver is the main treatment method, but unfortunately, it is only feasible in 25-30% of cases. In other patients, the tumour cannot be surgically removed due to metastases outside the liver, or the large size, number, and extent of tumour changes in the liver. Although liver transplantation is not a standard method, it may be promising for some patients. Due to the high relapse rate after surgery, postoperative chemotherapy is recommended if surgical resection is not possible. For patients who are not surgical candidates, systemic chemotherapy, biological therapy, and local therapy are options to improve their quality of life.
Reducing the risk of CCC involves a healthy diet, quitting smoking, engaging in physical activity, and avoiding exposure to chemical and biological agents that lead to chronic inflammation of the liver and bile ducts.
