The tumor Klatskin, cholangiocarcinoma: prognosis, treatment
Tumor Klatskin call cholangiocarcinoma – cancer originating from epithelial lining of the bile ducts. The disease is relatively rare, is not more than 3% of all malignant tumors, widespread but more common in residents of South-East Asia, Far East, Japan.
Among patients with cholangiocarcinomas is dominated by older people aged 50-70 years, men with this disease is slightly more, probably because they are more likely to suffer sklerosiruuschem cholangitis, which is considered a risk factor for tumors.
Increase the harmful effects of the external environment, increasing the role of carcinogens, along with improved diagnostic capabilities leads to the detection of cancer in younger persons starting from 45 years. Overall, the incidence of cholangiocarcinomas, like many other malignant tumors, showing a tendency to increase.
Attention to the tumor Klatskin due to the fact that the disease is difficult to detect in the early stage and treatment of neglected forms leaves no hope for a cure, but significant prolongation of life. The forecast remains bad after the surgery, patients live an average of about two years, without surgery about 7 months.
Risk factors and causes tumors Klatskin
The exact causes of biliary tract cancer is still not established but essential:
- The presence of stones in the bile ducts and the bladder increases the risk of cancer several times;
- Chronic inflammation (cholecystitis, cholangitis);
- Congenital anomalies of the system delchevalerie;
- Parasitic infestation (opisthorchiasis);
- Pathology of the intestines (Crohn’s disease, ulcerative colitis).
There is a genetic predisposition, traceable in the syndrome of Lynch, when there is colon cancer and the gallbladder due to a genetic mutation.
With a high percentage of infection of the parasites in the gall bladder and passages, associated a greater incidence of cholangiocarcinoma in the far East, Japan and Asia.
Among the possible risk factors also indicate the toxic effects, in particular, the radiopaque substance torocast previously used for diagnosis.
As a result of mechanical irritation of the epithelium of the bile duct stones, chronic inflammation, intoxication is cellular damage, which is especially pronounced on the background of sclerotic processes and dysplasia. Still it is not known which cells are the source of cholangiocarcinoma, but it is highly likely that it appears not from the actual epithelial duct, and from the stem cells of the liver.
The process of malignant transformation is the stage of hyperplasia, metaplasia and dysplasia, which can be considered the initial step in cancerous transformation. Up to 95% of cholangiocarcinoma the structure is a glandular tumors, much less frequently found forms of squamous, mucous, and undifferentiated carcinomas.
Gradually increasing in size in the limited space of the bile duct, the tumor promotes the violation of the outflow of bile right up until its cessation, which leads to the characteristic symptoms of the disease.
The classification cholangiocarcinoma is the cancer type, degree of differentiation, location of tumor, the behavior patterns of the liver and metastasis. Depending on location there are:
- Intrahepatic forms of cancer – make up approximately a quarter of all cholangiocarcinoma, come from the bile ducts within the liver;
- Extrahepatic – can be proximal, striking moves closer to the common bile duct and the bladder and distal located farther in the direction of the outlet hole (in side of duct passes through the pancreas).
The most common is the growth of the proximal extrahepatic cholangiocarcinoma, and, in rare cases it is formed in several moves (multifocal cancer). Tumor Klatskin often called cancer Central part of the system of delchevalerie.
By the nature of growth of the tumor Klatskin can be vnutriportovaya infiltrates and massive, she is able to protrude into the lumen of the duct in the form of a constrained node or a polyp and diffuse sprout tissue.
The stage of the cholangiocarcinoma is determined according to the TNM system. In the first stage the cancer is in the range of mucosal and muscular layers, the second reaches the outer layer of the duct, with the third possible implementation in the liver tissue at a distance of not more than 2 cm, stage IV cancer grows deep in the hepatic parenchyma, possibly to the stomach, pancreas, intestines.
Metastasis occurs primarily through the lymphatic vessels. The first affects the lymph nodes in the hepatic Porta, and then around the pancreas, and in advanced stage the cancer has spread to the celiac, mesenteric, periportal (around the portal vein) lymphatic collectors.
Symptoms of cholangiocarcinoma
In the initial stages of tumor Klatskin any symptoms absent, cancer does not cause pain, bile goes through the ducts. With increasing tumor size narrows the lumen of the bile passage that impedes the movement of the content.
The main signs of lesions of the biliary tract are jaundice and dyspepsia. The skin acquires a greenish tint, characterized by strong itching, provoking scratching. The greenish color and itching caused by reverse suction of the stagnant components of bile in the bloodstream and their deposition in the skin.
Because bile cannot reach the intestine, the stool becomes colorless, while the urine contains bile acids and a large amount of bilirubin will be dark.
Unlike closure of the bile ducts by a stone, tumor obstruction doesn’t cause attacks of biliary colic, that is, jaundice progressively grows without pain. Pain possible with the rapid growth of the cancer, but this occurs infrequently.
In the late stages of cholangiocarcinoma of the liver or extrahepatic ducts, soreness and heaviness in the right hypochondrium become characteristic signs associated with enlargement of the liver and bile stasis.
In addition to jaundice patients complain about indigestion. Possible vomiting, diarrhea, nausea, reduction and complete lack of appetite. Lack of bile leads to disruption of digestion and fat absorption, so patients significantly lose weight. In advanced stage tumors expressed Clackin severe weakness, increasing fatigue, may appear fever on the background of cancer intoxication.
Among the complications of the tumor is most likely liver failure, bleeding, secondary inflammatory process in the ducts, abscesses of the liver tissue, sepsis.
When large cholangiocarcinoma, metastases in the gate area of the liver results in compression of the portal vein, which is manifested by enlargement of the spleen, with heaviness in the left upper quadrant and accumulation of fluid in the abdomen (ascites).
Clackin the tumor is located deep in the tissue, so it is impossible to test, but this feature, as the increase of the gallbladder along with the above symptoms may indicate possible cancer.
Diagnosis of cholangiocarcinoma
Diagnosis of cancer of the bile duct requires a variety of instrumental and laboratory tests. Blood tests show increased bilirubin, alkaline phosphatase, whereas the liver enzymes (AST, ALT) and albumin are in the normal range. In General, the analysis of blood is possible a slight increase of leukocytes, especially with concomitant inflammatory process in the later stages there may be signs of anemia, but in General, the laboratory data do not allow accurate diagnosis.
In patients with suspected tumor Klatskin determine the blood tumor markers – CA 19-9, in particular. He increased in pancreatic cancer, but a significant increase in patients with already diagnosed inflammation of the biliary tract with high probability speaks in favor of cancer.
Quite a large amount of data can be obtained using instrumental methods including ultrasound, CT, positron emission tomography, which can help to detect tumors less than 1 cm.
X-ray examination of the bile ducts with contrast allows to determine the closing level of the moves, as pin holes in the intestine. In addition, under these procedures it is possible to perform biopsies for histological examination of tumor tissue. The most informative and safe way to determine the location and size of the neoplasia is MRI.
Treatment of tumor Klatskin
In the way of treatment of cancer of the bile ducts there are significant challenges caused by its location, which determines the poor availability during the operation. Another aggravating factor is the complexity of the diagnosis of early forms of cancer, causing the vast majority of tumors begin to treat in late stages, when prognosis is poor.
The main method of treatment of early-stage cholangiocarcinoma is surgery. If the tumor has not reached a considerable size, had sprouted the wall of the bile duct, it is possible to perform choledochotomy when the common bile duct is dissected in the longitudinal direction, with subsequent excision of the tumor. It also involves stenting of the bile ducts to facilitate bile flow.
With the defeat of the bile ducts within the boundaries of one lobe of the liver shows its removal – lobectomy. The operation may be complemented by the creation of lines of communication between the duodenum and the common bile duct.
The most voluminous is the Whipple, used in patients with large cholangiocarcinomas. The procedure removed a piece of liver neoplasia, portion of the stomach and pancreas, duodenum, gallbladder and its ducts, and the lymph nodes of the affected area.
Radical surgery can be carried out not more than 15% of patients because of neglect, and in connection with the dire state due to comorbidity, which have many elderly patients. Mortality in the postoperative period reaches 40%, which is caused by surgical trauma and the significant volume of removed tissues.
The most radical method of treatment may be a liver transplant, but this method is seldom used because of the prevalence of tumor process and complex selection procedure on transplant.
If it is impossible to carry out radical treatment, surgeons resort to palliative methods – the imposition of the biliary anastomosis, stenting of the duct. These methods do not eliminate the tumor, but help to improve the health of patients and to eliminate some of the symptoms of stagnation of bile (itching, jaundice).
Given the localization of the tumor, and a high percentage of advanced forms of the disease, operations are usually not radical, but palliative, aimed at easing the negative impact of cancer on the patient.
Surgical treatment can be combined with chemotherapy and radiation, although there is no guarantee that this combined effect will help in the fight against cancer.
If it is impossible to perform the surgery or after surgery can be chemotherapy and radiation. In the first case, they aim at the destruction of tumor tissue and alleviating the flow of bile, and the second on prevention of recurrence and progression pathology. Conservative treatment using 5-fluorouracil, platinum drugs, gemcitabine.
Cholangiocarcinoma referred to the tumors with poor prognosis, survival rate with her – about a year and a half, even after surgical treatment. Radical surgery is not always effective and are accompanied by a large operational risks, but very serious complications – sepsis, liver abscesses.
If you were able to detect early cancer and effective treatment, the lifetime can reach 3-5 years, but such cases are rare. Neperedavaemye patients live about six months. In General, the prognosis depends on the degree of differentiation of the cancer, presence of metastases, patient’s age, concomitant background.