Malignant liver and biliary tract tumors are among the most common cancer types. Without treatment, the average life expectancy of patients with malignant tumors of the liver and biliary tract is about 5 months. Secondary metastatic liver lesions are also widespread. The main primary malignancies of the liver are hepatocellular carcinoma and cholangiocellular carcinoma. Some neoplasms are asymptomatic, others are accompanied by mechanical jaundice, especially in Klatskin tumors. This is a rare type of cholangiocarcinoma, which is usually localized in the common bile duct bifurcation. Treatment of Klatskin tumor requires from doctors relevant practical experience, as the neoplasm poorly responds to chemotherapy and radiation therapy. However, in sufficient treatment, the five year survival rates even in patients with diagnosed advanced tumor exceeds 25%.
What is the Klatskin tumor?
This disease is a malignant neoplasm of the hepatic ducts, localized near the junction of the common hepatic and vesicular ducts (i.e. the hepatic duct bifurcation). Klatskin tumor is a very rare but dangerous tumor of the bile duct. It grows slowly, but can reach a large size and damage surrounding organs. A cholangiocarcinoma arises when healthy cells acquire a mutation that causes them to turn into abnormal ones.
Statistically, the incidence of cholangiocellular cancer ranges from 2 to 6 cases per 100,000 population. Klatskin tumor is the most frequent type of cholangiocellular cancer, accounting for about 50% of all cases. This tumor may stay undetected for a long time. Despite its malignancy, it does not progress as quickly as other tumors.
There is no certain factor that causes the Klatskin tumor development. People with sclerosing cholangitis, choledochal cysts, parasitic infections, and other conditions that lead to the chronic inflammation in the bile ducts are in the risk group. There is also a iatrogenic risk factor, i.e. Thorotrast usage. Thorotrast (the substance is thorium dioxide) is the radiologic contrast agent that is used during computed tomography (CT) scan or magnetic resonance imaging (MRI).
Symptoms
Due to the slow progression, this tumor is most often asymptomatic. Symptoms may appear when the tumor reaches large size and starts to damage neighboring organs or blocks bile ducts. As a rule, the symptoms depend on the organs involved and the degree of bile ducts compression. The most common symptoms of Klatskin’s tumors are:
- Jaundice
- Itching
- Fever
- Light colored stools and dark urine
- Loss of appetite
- Weakness or feeling very tired
- Nausea and vomiting
In order not to miss the cancer, patients should have regular medical check-ups. It is worth remembering that early detection of a tumor increases the effectiveness of treatment and reduces the health risks.
Klatskin tumors are perfectly visible on the CT scans and during endosonography. Elevated blood levels of alkaline phosphatase, gamma-glutamyl transpeptidase, and conjugated bilirubin may be also indicative of this pathology. However, Klatskin tumors must be differentiated from primary biliary non-Hodgkin’s lymphomas and autoimmune cholangitis.
First-line treatment
Making the correct diagnosis and determining localization and size of the tumor are the basis for elaborating effective treatment scheme. In modern foreign clinics, one of the most informative visualizing diagnostic methods is endoscopic retrograde cholangiopancreatography.
When the doctor determines the size and localization of the tumor, a therapeutic method is chosen individually for each patient. Often, surgical removal of the tumor is used to treat the initial cancer stages. During the intervention, a surgeon usually removes the lobe of the liver, as this provides the highest chances of receiving negative resection margins. The term “negative resection margins” means that there are no signs of an oncological process in the margins of the operative wound, i.e. the tumor was removed completely and the risks of disease recurrence are low.
In some patients, the preoperative biliary drainage is carried out, as Klatskin tumors are most often detected at the stage of jaundice. The intervention improves liver function and helps a patient to tolerate the subsequent surgery better.
However, surgery cannot be performed in all patients due to the presence of contraindications. The intervention is not performed in tumors that affect contralateral branches of the hepatic artery or the portal vein.
Additional methods of treatment
With the introduction of modern treatment methods, doctors have more sparing and efficient procedures to treat tumors. Today, you can find many novel treatment methods in modern foreign clinics. As a rule, radiation therapy is the most effective treatment for advanced stages of a Klatskin tumor and for inoperable neoplasms. The stereotactic fractionated radiotherapy can also be used for this purpose.
When possible, radiation therapy is used in combination with surgical removal of the tumor. This significantly improves the results of treatment and also reduces the risks of relapse after surgery. The effectiveness of the treatment depends on the stage of the disease, its location, and the skills of your physician.
Chemotherapy is another second-line therapy for patients in whom the surgery can not be carried out. Two-drug chemotherapy cycles are prescribed, with administration of cisplatin and gemcitabine.
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