Types of tumor and why it is important
The bile tree begins with small ducts inside the liver and ends with the common bile duct, which flows into the duodenum. Depending on where the tumor originated, three forms are distinguished, and both symptoms and tactics depend on this. The intrahepatic form grows asymptomatically for a long time and is manifested by pain, weakness, and weight loss. A tumor at the confluence of the ducts, known as a Klatskin tumor, causes early jaundice. The distal form is located closer to the intestine and is similar in appearance to cancer of the head of the pancreas.
- Intrahepatic cholangiocarcinoma
- Portal form, or Klatskin tumor
- Distal cholangiocarcinoma
- Growth along the wall of the duct with its narrowing
- Nodular and intraductal forms of growth
Risk factors
In many cases, no clear cause can be found, but a number of conditions are known in which the risk is significantly higher. Long-term chronic inflammation of the bile ducts plays a key role. The most important is primary sclerosing cholangitis, often associated with inflammatory bowel disease. Congenital bile duct cysts and Caroli's disease, parasitic infestations with liver flukes, chronic viral hepatitis B and C, cirrhosis of the liver, as well as cholelithiasis with stones inside the ducts are significant.
- Primary sclerosing cholangitis
- Bile duct cysts and Caroli disease
- Opisthorchiasis and clonorchiasis
- Chronic hepatitis B and C, liver cirrhosis
- Intrahepatic duct stones
- Chronic inflammatory bowel diseases
- Age over 50, smoking, obesity and diabetes
Symptoms
The picture depends on the location of the tumor. When large ducts narrow, obstructive jaundice comes to the fore: the skin and sclera turn yellow, urine darkens, feces lighten, and painful itching appears. There may be no pain at all at first, so people are often referred to an infectious disease specialist with suspected hepatitis. With the intrahepatic form of jaundice, jaundice may not last long, but you will be bothered by a dull pain on the right side, weight loss, weakness, and sometimes fever. The addition of chills and fever indicates inflammation in the stagnant ducts.
- Yellowness of the skin and sclera
- Itchy skin
- Dark urine, light stool
- Decreased appetite and weight loss
- Dull pain or heaviness in the right hypochondrium
- Weakness, fatigue
- Fever with chills with cholangitis
Diagnostics
The examination begins with biochemistry and ultrasound: bilirubin is increased due to direct bilirubin, alkaline phosphatase and GGT, and ultrasound shows dilated ducts above the obstacle. The main clarification method is MRI with magnetic resonance cholangiography, which shows the entire biliary tree without intervention. Contrast-enhanced CT evaluates tumor spread, blood vessels, and the presence of metastases. Tumor marker CA 19-9 is used as an auxiliary marker: it also increases with simple blockage of the duct. Morphological confirmation is obtained by endoscopic examination with brush biopsy or percutaneous biopsy.
- Bilirubin, alkaline phosphatase, GGT, liver function tests
- Ultrasound of the liver and bile ducts
- Abdominal MRI with cholangiography
- MSCT with contrast for staging
- Tumor markers CA 19-9 and CEA
- Endoscopic cholangiography with brush biopsy
- Endoscopic ultrasound with fine needle biopsy
Treatment
The only method that offers a chance of cure is radical surgery to remove the tumor and part of the liver or ducts. The possibility of surgery is determined by a consultation based on CT and MRI data, taking into account the involvement of blood vessels and the volume of the remaining liver. When the tumor cannot be removed, the main task is to restore the flow of bile: a stent is installed endoscopically or percutaneous drainage is performed. This relieves jaundice and itching, improves well-being and allows chemotherapy. Additionally, radiation therapy and targeted treatment are used taking into account the molecular profile of the tumor.
- Resection of the tumor including part of the liver or ducts
- Liver transplantation according to strict indications
- Endoscopic stenting of the bile duct
- Percutaneous transhepatic drainage
- Chemotherapy, including after surgery
- Radiation therapy and targeted drugs
- Pain relief and treatment of itchy skin
- Nutritional support and supervision by an oncologist
