intrahepatic cholangiocarcinoma

What is it?

Cholangiocarcinoma is a primary neoplasm of the bile duct that occurs as a result of malignant transformation of cholangiocytes, the cells that make up the epithelium of the bile ducts. According to the anatomical location along the gall tree, it can be classified into:

  • peripheral or intrahepatic (CCI), which occurs in small bile ducts in the liver;
  • extrahepatic (CCE), which occurs in the area of the bile ducts outside the liver and, in turn, is divided into:
  • Hilar’s or Klatskin’s tumor, occurs where the right and left bile ducts meet the common bile ducts;
  • Distal, coming from the terminal part of the biliary tract.

Root and distal cholangiocarcinoma are often manifested with obstructive symptoms (including jaundice). In contrast, peripheral cholangiocarcinoma, which occurs inside the liver, looks like a hypodensal volume formation, and, therefore, in most cases is not associated with jaundice.

Causes and risk factors

CCI is the second most common primary liver neoplasm after hepatocellular carcinoma and accounts for 10-15% of all liver neoplasms. In contrast to extrahepatic cholangiocarcinoma (CCE), there has been a significant increase in the incidence of CCI in recent years. Probably, the reason for this is multifunction, but definitely an earlier and more accurate diagnosis plays a big role.

Some of the known risk factors for CCI are primary biliary cirrhosis and primary sclerosing cholangitis, hepatolithiasis, cystic transformation of the bile ducts, parasitic biliary tract infection, and metabolic syndrome.

Which are the symptoms?

Unlike extrahepatic cholangiocarcinoma, which is often manifested by jaundice, CCI is often an accidental finding detected by instrumental tests performed for other reasons or with unspecified abdominal symptoms. Less often, symptoms of the onset of the disease are pain in the epigastrium, weight loss and palpable formation in the upper quadrants of the abdomen.

How is it diagnosed?

It is sometimes difficult to distinguish cholangiocarcinoma, and before making a final diagnosis (sometimes only by histological examination by percutaneous biopsy), it is necessary to exclude that this is a metastasis associated with a neoplasm localized elsewhere (more often in relation to the digestive tract).

Suggested exams

How is it treated?

Surgical treatment is preferred: approximately 50-60% of patients are operable at the time of diagnosis. The type and degree of liver resection is dererminated based on the location and size of the lesion, characteristics that affect the operational risk along with overall clinical condition of the patient.

Patients who are non-operable at the time of diagnosis are referred for a chemotherapy, and liver resection is reviewed depending on the response to it.

Suggested procedures

Where do we treat it?

Within the San Donato Group, you can find intrahepatic cholangiocarcinoma specialists at these departments:

Are you interested in receiving the treatment?

Contact us and we will take care of you.