Cholangiocarcinoma of the liver: types, causes and symptoms, diagnosis, treatment, prognosis

August 22, 2017 23:00 | Digestive System

Cholangiocarcinoma( carcinoma or cholangiocellular liver cancer) is a malignant lesion of the bile ducts.The disease is rarely detected, but in the absence of timely treatment quickly leads to death.

What is cholangiocarcinoma of the liver?

The concept of cholangiocarcinoma of the liver Cholangiocarcinoma develops when cancer cells settle on the walls of the bile duct and begin to form a tumor in them.

The bile duct is a thin tube, up to 12 cm long, it branches away from the liver and connects it to the small intestine.

The main function of the duct is the movement of bile into the intestine, where it is necessary to digest the food that enters the body.

The bile duct is divided into several parts.The first part is located in the liver itself and is a small tube, serving to collect bile from the cells of the body.These tubules gradually gather together, forming already larger channels, and are gradually converted into two hepatic canals.

The ducts located in the liver tissues are usually referred to as intrahepatic ducts.The left and right he

patic canal are connected at the outlet of the organ together and form a common bile duct in the portal gates.This duct passes through the thickness of the pancreas, where the duct of this organ joins it.Then they as a single tube go into the gut.

Malignant neoplasm begins to develop from small epithelial cells of the bile duct.

Prevalence of

According to the latest statistics, cholangiocarcinomas occur in 2% of all malignant lesions of the body.

In North America and Europe, this diagnosis is issued to no more than two people per one hundred thousand people.In the countries of the Far East, which include Japan, Korea, China, the biliary carcinoma is found in 20% of cases of all cancers.This is due to the spread of parasitic liver lesions in these countries.

Bile duct cancer is a disease common among the elderly.The average age of those who fell ill from 50 to 70 years is more than men.Over the past three decades, there has been an increase in the incidence of cholangiocarcinomas, and a diagnosis is often made for people who have reached the age of 45 years.

Associate this, primarily with increasing provoking factors and using modern methods of examining liver structures.


Varieties of cancer of the bile ducts It has been accepted to use several classifications of cholangiocarcinomas.By localization, the cancer of this species is divided into:

  • Intrahepatic cholangiocarcinoma. Malignant tumor begins to form in the smallest tubules of the bile ducts located in the liver tissues.Similar is this type of cancer with hepatocellular carcinoma, which affects liver cells.Treatment of these two cancers of the organ is almost identical.Intrahepatic cholangiocarcinoma is detected in one of 10 cases of this type of cancer.
  • Intramuscular cholangiocarcinoma. The tumor begins where two hepatic ducts are connected to one common, that is, the gates of the liver.This form of neoplasm can occur in medicine and is called the Klatskin tumor.It is considered the most common, occurs in 6-7 cases out of 10 when malignant cells of the bile ducts are affected.
  • Distal cholangiocarcinoma. The distal duct is exposed to malignant degeneration, that is, the part that is closest to the small intestine.It is considered an extrahepatic neoplasm and is detected in 2 out of 10 cases of cholangiocarcinomas.

On a macroscopic structure, intrahepatic cancer is divided into three types:

  • Massive.
  • Peripheral infiltrating.
  • Intra-flow.

By the nature of its growth, cholangiocarcinoma happens:

  • Infiltrative. Characterized by germination in the walls of the bladder and surrounding tissues.
  • Polypoid. The tumor grows into the lumen of the ducts and is attached to their walls by means of a thin pedicle.
  • Exophytic. Tumor growth occurs from the inside of the duct to the external.
  • Mixed. This form of cholangiocarcinoma has the symptoms of all three of the above.

Reasons for

For what reasons the bile duct cancer is not exactly established.In some patients with this diagnosis, the effect of provoking factors is revealed, others have no changes in this plan.

The most likely etiological causes of bile duct carcinoma include:

  • Infection with parasitic infections. Most patients in Southeast Asia have a history of infection that develops when they are infected with the hepatic duodenum.The risk of developing cholangiocarcin increases when infected with parasites such as Opisthorchis viverrini, Clonorchis sinensis, Ascaris lumbricoides.
  • Sclerosing cholangitis and chronic bowel diseases , to which primarily include ulcerative colitis, Crohn's disease.
  • Exposure to chemicals. Cancer ducts can occur several years after the introduction of the body radiopaque substance - thorium dioxide.There is a negative effect on liver tissues of chemicals used in wood processing and rubber industries, aircraft construction.
  • Hereditary pathologies of the biliary tract, , include Caroli's disease, the cyst of the bile duct.
  • Congenital malformations of ducts.

It has now been established that gallstones, liver cirrhosis and viral hepatitis do not increase the risk of cholangiocarcin.


Symptoms of cholangiocellular liver cancer Since the bile ducts are small in size, the growing tumor quickly causes their obstruction, that is, compression.

And this is what influences the appearance of the first symptoms of cholangiocarcinoma - the development of icterus of the skin and mucous membranes is noted, skin itching occurs.

The patient can pay attention to the color change of the stool - it becomes light, and the urine is accordingly dark.With the defeat of the ducts, the outflow of bile to the intestine is also disturbed, which in its turn becomes the cause of not complete assimilation of fats.

This change, as well as lack of appetite, vomiting and intoxication lead to a noticeable weight loss.Pain in the right hypochondrium occurs already in the late stages of cholangiocarcinoma.

If the growth is already on the background of sclerosing cholangitis, then patients notice a worsening of the already familiar with the illness.That is, itching, pain, yellowness, weakness, nausea, and appetite decrease.

Stages of development

To determine the stage of cholangiocarcinoma, oncologists use the TNM system.In this classification T denotes the depth of germination of a cancerous tumor, N - the presence of metastases in regional lymph nodes, M - metastasis of distant organs.

Degree of spread of cancer:

  • Tx - the depth of the emerging tumor can not be determined;
  • T0 - no signs characteristic of a tumor of this species;
  • Tis - the tumor is located only at the place of its formation;
  • T1 - the neoplasm is located within the bile ducts, its germination in the smooth muscle and fibrous layer is noted.
  • T2a - tumor growth is detected for the bile ducts and adjacent tissues;
  • T2b - neoplasm captures nearby liver tissue;
  • T3 - the tumor spreads to the hepatic arteries and the portal vein;
  • T4 - the tumor completely germinates the hepatic ducts and passes to the nearest to the organ blood vessels.

Degree of involvement in the cancerous process of the lymph nodes:

  • Nx - the degree of involvement of regional lymph nodes in the cancer process can not be assessed;
  • N0 - there are no metastases in the lymph nodes;
  • N1 - metastases are detected in the lymph nodes located along the ducts, veins and arteries of the abdominal cavity;
  • N2 - the cancerous process affects the lymph nodes located along the celiac or upper mesenteric arteries.

Metastatic evaluation:

  • Mx - no possibility to determine distant metastases;
  • M0 - no metastases were detected;
  • M1 - Metastases in distantly located organs are defined.

According to this cancer detection system, the cholangiocarcinoma has the following stages:

  • Stage 0 - Tx N0 M0.
  • Stage I - T1 N0 M0.
  • Stage II - T2 N0 M0.
  • Stage III- T1-2 N1-2 M0.
  • Stage IVa - T3 N0-2 M0.
  • Stage IVb - T1-3 N0-2 M1.


Diagnosis on cholangiocarcinoma of the liver A patient with a suspected biliary duct cancer is prescribed a series of standard examinations:

  • Ultrasound examination shows enlarged bile ducts and neoplasms in the liver.
  • CT and MRI
  • PET.This type of tomography determines tumors even up to 1 cm in diameter.
  • Endoscopic retrograde pancreatocholecystography( ERCPG).In this examination, a contrast agent is introduced, which allows one to detect obstruction of the ducts and neoplasms in them in the picture taken.
  • Biopsy.

Analyzes for tumor markers with cholangiocarcinoma show the presence of CEA antigen and CA 19-9.

Treatment of bile duct cancer

Effective treatment of cholangiocarcinoma is possible at an early stage by performing an operation.But, unfortunately, the cancer of this species at the initial stages is only discovered by chance.

Palliative methods of treatment are used for the relief of health: stenting of the biliary tract, chemotherapy, radiation exposure.

If there is no possibility of a radical operation and resort to organ transplantation, then resort to the use of endoprostheses and relieve the patient from plugging the bile ducts.It positively affects the general state of health, itchy and reduces the risk of purulent inflammation of the ducts.

Prognosis and prevention of cholangiocellular liver cancer

For , the cholangiocarcinoma of the liver is characterized by slow growth.The average survival rate of patients with this diagnosis is 14 months, but reaches 5 years.If there is extrahepatic cholangiocarcinoma, the operation allows to achieve good results of treatment, which significantly prolongs life.

An unfavorable prognosis is displayed if an inoperable tumor and distant metastases are diagnosed.With such violations, only palliative treatment is possible, but even it prolongs life only for several weeks.

Remote metastases with cancerous lesions of the bile ducts appear late, so they are not the main cause of death of the patient.

Lethal outcome in most cases occurs due to the following complications:

  • A biliary cirrhosis caused by bile duct disorders.
  • Abscesses, developing due to intrahepatic infections.
  • General exhaustion of the body.
  • Sepsis.

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