Holangiokartsinoma is a malignant tumor of bilious channels. Symptoms of a disease are generally connected with holestazy (jaundice, a skin itch), also patients can have pains in the right podreberye, sharp loss of body weight. For diagnosis use ultrasonography of abdominal organs, the computer tomography (CT), the positron-emissionuyu tomography (PET), the magnetic and resonant tomography (MRT), a chreskozhny holangiografiya, definition of onkomarker in blood. The main method of treatment of a holangiokartsinoma – quick. The chemotherapy and radiotheraphy are used in a complex with a surgical method or as palliative treatment.
Holangiokartsinoma – a malignant new growth which develops from cages of an epithelium of bilious channels. Meets on all continents, but it is more widespread in Southeast Asia. Frequency of identification of a holangiokartsinoma in the countries of Europe and North America - 1-3 cases on 100 thousand of the population, in Japan – 5,5 cases, in Israel about 7 cases. Holangiokartsinoma makes about 3% of all malignant diseases of digestive tract. In spite of the fact that the tumor meets not really often, this problem is rather urgent. The forecast at this pathology very adverse. Today treatment of a holangiokartsinoma is possible only at identification of process at early stages. Primary diagnostics is carried out most often by the gastroenterologist as patients at first address him. Surgeons and oncologists are engaged in treatment.
The holangiokartsinoma etiology is up to the end not found out. Carry cholelithiasis, congenital malformations of biliary tract to risk factors. High incidence in the countries of Asia is explained with prevalence in this territory of parasitic diseases, such as and .
Many experts note increase in frequency of malignant tumors of biliary tract at patients with primary skleroziruyushchy holangit, chronic inflammatory diseases of intestines, such as disease Krone or nonspecific ulcer colitis. Also holangiokartsiny connect with influence of toxic substances, for example dioxide of thorium which is used as X-ray contrast medicine. Communication between hepatitis C and holangiokartsinomy is up to the end not established.
Presumably, tumors are formed because of damage of cages of an epithelium of biliary tract (mechanical or toxic). On histologic structure the most part from them is an adenocarcinoma (the tumor developing from ferruterous fabric), only 10% identify as skvamozokartsiny (planocellular cancer). On the structure of a holangiokartsinom dense, the ferruterous structure, white color, sometimes differs from the sites struck with a skleroziruyushchy holangit a little.
Classification of a holangiokartsinoma
On localization distinguish the following types of a holangiokartsinoma: intra hepatic, extra hepatic in the field of bifurcation of the general bilious channel (the most frequent localization), the extra hepatic disteel tumors arising on a piece between a faterovy nipple and the upper edge of a pancreas.
Besides, for classification use the TNM system where T (tumor) determines depth of germination of a tumor, N (nodulus) – existence or lack of metastasises in regional lymph nodes, M (metastasis) – existence or lack of the remote metastasises.
Sizes and extent of distribution of tumoral process:
Tx – a tumor cannot be estimated;
T0 – the signs demonstrating existence of a tumor is not present;
Tis – a carcinoma or a tumor of in situ (on the place);
T1 – a tumor is limited to borders of bilious canals, sprouts in a layer of smooth muscles and in fibrous;
T2a – spread of a tumor out of borders of bilious canals and the fabric surrounding them;
T2b – spread of a tumor on nearby hepatic fabrics;
T3 – spread of a tumor on hepatic arteries and a vorotny (portal) vein;
T4 – germination by a tumor of hepatic channels or involvement in process of nearby veins and arteries.
Involvement of lymph nodes: Nx – cannot estimate extent of involvement of regionarny lymph nodes in process; N0 – are absent any metastasises in regionarny lymph nodes; N1 – is metastasises in different regionarny lymph nodes which settle down along veins, bilious channels and arteries of an abdominal cavity; N2 – are struck lymph nodes along the top mezenteralny arteries or along a chrevny artery, near other abdominal organs. Assessment of metastasises: Mx – is unknown or it is impossible to define whether there are metastasises; The patient has no M0 – metastasises; M1 – are revealed the remote metastasises.
To holangiokartsiny classify by the nature of growth as follows: infiltrative (it is shown by diffusion germination of walls of a bubble and surrounding fabrics); polipovidny (grows in a gleam of channels, often has a thin leg); ekzofitny (sprouts on outer side of bilious channels); mixed (has characteristics of all above-mentioned types).
The first symptoms which the gastroenterologist faces are usually connected with impassability of bilious channels. The patient with complaints to yellowing of integuments, an itch. Darkening of urine and decolouration of kalovy masses is often observed. Because of violation of digestion of fats patients lose weight. Decrease in body weight can be also connected with lack of appetite, nausea, vomiting, cancer intoxication. At late stages of a disease pains in the right podreberye develop.
Holangiokartsinoma can become complicated holangity (an inflammation of bilious channels). At patients temperature increases, fever symptoms appear. If the tumor arose against the background of sclerous processes or at cholelithiasis, deterioration in a condition of the patient is noted. Pains, yellowness, an itch amplify, weakness, nausea, a loss of appetite appears.
Unfortunately, symptoms of a holangiokartsinoma are not specific. It is almost impossible to make the diagnosis only on the basis of the anamnesis and survey. The disease should be differentiated with acute viral hepatitises, leptospirosis, holangity, stones or sclerous processes in a gall bladder, cirrhosis, pancreas head cancer.
Diagnostics of a holangiokartsinoma
For diagnosis use tool and laboratory methods. Usual biochemical tests of a liver give information on extent of malfunction of this body, but do not allow to carry out exact diagnostics of a holangiokartsinoma. In blood reveal increase in bilirubin at the expense of direct fraction, alkaline phosphatase. Alaninamintransferaza and aspartateaminotransferase, level of albumine usually within norm.
For diagnostics of a holangiokartsinoma specific markers of oncological diseases have bigger value. At patients reveal CA 19-9 anti-gene though its increased level can be observed also at a pancreatic cancer or a holangita. Substantial increase of an anti-gene to 100 U/ml more at patients with a chronic holangit, most likely, testifies in favor of a holangiokartsinoma. Sometimes it is possible to reveal increase in a kartsinoembrionalny anti-gene; alpha within norm.
More informative are tool methods. In gastroenterology most often for diagnostics of a holangiokartsinoma at the initial stage of inspection use ultrasonography of a liver and a gall bladder. This method allows to reveal tumors of the big size, expansion of channels on some sites. Dopplerometriya of vessels of a liver defines changes in a blood-groove which demonstrate existence of a new growth. Tumors of the small size often are not visualized.
The Computer Tomography (CT) of biliary tract is a little more informative. It helps to reveal increase in lymph nodes, to see tumors of the smaller size. MSKT of abdominal organs determines the level of obstruction of bilious ways. The advanced positron and issue tomography gives the chance to see tumors less than 1 centimeter in size of nodal type. But by means of this technique it is difficult to reveal infiltration forms of a holangiokartsinoma.
For specification of the diagnosis use also some radiographic methods of a research. The endoscopic retrograde cholecystography is carried out for the purpose of identification of the place of obstruction, pathological changes in area of a faterov of a nipple. The method allows to take material for the histologic analysis. The Chreskozhny chrespechenochny holangiografiya is carried out before surgery. It helps to reveal holangiokartsiny in the place of bifurcation of the general bilious channel.
The most informative method is the magnetic and resonant tomography (MRT) of a liver and bilious ways today. The method is noninvasive, does not demand contrast introduction, therefore, rather safe. MRT of a liver allows to see bilious channels and vascular structures in the three-dimensional image, to reveal tumors of the small size, it is correct to estimate extent of defeat, to define tactics of operation and the further forecast for the patient.
Treatment and forecast of a holangiokartsinoma
The main method of treatment of a holangiokartsinoma – quick though it is shown not in all cases. If the tumor sizes small, and it does not sprout a wall of bilious channels, the holedokhotomiya is carried out. In more difficult situations the liver share resection is shown (lobectomy). The large tumor with defeat of nearby structures demands more radical intervention. In such cases perform Whipple's operation: a part of a liver, stomach and duodenum, a gall bladder with channels, a pancreas and regionarny lymph nodes is removed.
Conservative treatment of a holangiokartsinoma is performed after surgical intervention or when operation cannot be executed (palliative therapy). It includes chemotherapy and radiation therapy. For chemotherapy most often use 5-ftoruratsit. Also conduct courses with gemcitabine, tsisplatiny.
The forecast at a holangiokartsinoma adverse. Survival of patients averages 14 months. If the tumor is revealed in time, thanks to treatment life of the patient can be continued to three and even up to five years. But as the disease passes in an initial stage almost asymptomatically, cases of early diagnostics are rare.
Basis of prevention of a holangiokartsinoma – adequate treatment of those diseases which can lead to malignant regeneration. These are stones in a gall bladder, sclerous processes, helminthoses, chronic inflammatory diseases of a large intestine.