Cancer of extrahepatic bilious ways

Description:

Cancer of extrahepatic bilious ways – the rare malignant disease affecting a part of bilious canals which are outside a liver.

Cancer of extrahepatic bilious channels is more often observed at the age of 60-70 years. In half of cases the general bilious channel is surprised.

The tumor represents an adenocarcinoma of various differentiation with infiltrative type of growth. The tumor on an extent of channels grows, involves a hepatic vein and an artery, a portal vein, a pancreas, a duodenum. Metastasises are formed in regional lymph nodes and a liver. Clinical symptoms and the same diagnostic methods, as at cancer of a gall bladder.

Reasons of cancer of vnepechnochny bilious ways:

All factors increasing probability of development of cancer of extrahepatic bilious channels are called risk factors. Risk factor existence, as well as its absence, does not mean obligatory development of malignant pathology. If the patient considers that he is inclined to development of cancer, it is necessary to consult with the doctor. Such morbid conditions belong to risk factors:
- Primary sclerosing cholangitis.
- Chronic ulcer colitis.
- Cysts of bilious channels.
- A liver clonorchosis (infection with the Chinese fluke – the worm getting to an organism at the use of the infected fish).

Colitis, and also some diseases of a liver increase risk of development of cancer of extrahepatic bilious channels.

Pathogeny:

Holzinger et al. consider 4 phases of a pathogeny of biliary carcinogenesis:
The I phase the chronic inflammation caused by anatomic anomalies, a sclerosing cholangitis, autoimmune diseases, parasites, carcinogens.
The II phase the gennotoksichesky disturbances leading to damages of DNA and mutations.
The III phase the dysregulation of the DNA reparative mechanisms and apoptosis allowing to survive to the mutating cells.
The IV phase further morphological evolution of premalignizirovanny cells in a cholangiocarcinoma.

Symptoms of cancer of extrahepatic bilious ways:

The tumor of extrahepatic bilious ways can be shown by jaundice and a pain syndrome.

The listed below symptoms can be as a consequence of a tumor of extrahepatic bilious ways, and other diseases. Therefore in the presence of at least one of complaints should see a doctor immediately:
- Jaundice (yellow coloring of skin and whites of the eyes).
- Abdominal pain.
- Fever.
- Skin itch.

Jaundice - an obligatory sign of defeat of the main bilious channels. In half of observations it comes suddenly. At other patients the dozheltushny symptomatology is noted on average for 2-3 months. Usually jaundice happens intensive and resistant. However at localization of primary tumor in the place of triple connection of channels or in one of share channels, it can have wavy or recurrent character. Heavier course of mechanical jaundice with bystry development of symptoms of a hepatic decompensation is observed at the full obturation of the main channels causing shutdown of a tank of a gall bladder. Considering it, clinically it is necessary to distinguish proximal and distal obturation of extrahepatic bilious ways with border at the level of triple connection of channels. Significant increase in a liver, and for distal - a positive symptom of Courvoisier is characteristic of high tumoral stenoses. However increase in a gall bladder at mechanical jaundice not always indicates the obturation level as it can be disconnected as a result of blockade of a vesical channel by a tumor.

The cholangitis at cancer of channels is observed at 38-55% of patients. Approximately with the same frequency pains are noted. Considerable weight loss at cancer of channels seldom manages to develop; this symptom is more characteristic of a pancreatic cancer.

Diagnosis:

Apply the tests directed to researches of bilious channels and a liver to diagnosis of cancer of extrahepatic bilious ways.

The following diagnostic tests and procedures can be carried out:
- Survey of the patient for definition of the general condition of the patient, existence of symptoms of a disease at it (any signs and symptoms which are not found at the healthy person). It is necessary to examine testicles regarding a swelling, hypostasis or pain at a palpation (palpation). It is also necessary to ask the patient on his way of life, addictions postponed earlier diseases, which is carried out treatment attentively.
- Ultrasonography: at ultrasonography high-frequency sound waves (ultrasound) are reflected from internals and fabrics, such as testicles and there is an echo signal. Echo signals create a picture of fabrics and bodies of an organism which call sonogrammy. The received image can be printed out and later to estimate repeatedly in dynamics.
- KT-scanning: a series of the detailed pictures of all body made under different corners is carried out. Pictures are displayed the computer connected to a X-ray apparatus. For receiving more sharp image of bodies and fabrics, introduction to a vein or intake of a contrast agent is possible. Such procedure is called the computer tomography computerized by a tomography or computerized by an axial tomography.
- MRT (magnetic and resonant tomography): to data the method of a research is based on the radio-magnetic radiation by means of which receive a series of images of internals of the person. These pictures are brought to the monitor of the computer and, if necessary, to a film. Such procedure is called still a nuclear and magnetic and resonant tomography.
- ERHP (endoscopic retrograde holangiopankreatografiya): the method is based on X-ray research of the channels transporting bile from a liver and a gall bladder in a small intestine. Sometimes cancer of bilious ways narrows their pass or completely it is blocked by bile current, causing jaundice. The endoscope is entered through an oral cavity into a gullet, a stomach and an initial part of a small intestine. The endoscope is the thin tool in a form reminding a tube with the lighting device and special lenses for the review. The catheter (a small hollow tube) is entered into the pancreatic canal via the endoscope. On a catheter a contrast agent is entered, and x-ray films are carried out. In case of narrowing of a channel a tumor it is possible to enter into it a thin tube and to renew its passability. It is also possible to receive suspicious sites of fabric which then are subject to a research under a microscope regarding detection of cancer.
- Transdermal transhepatic holangiografiya (ChChHG): X-ray inspection of a liver and bilious channels. The fine needle is entered through skin under a costal arch into a liver, inject a contrast agent and carry out pictures of a liver and bilious ways. If passability of bilious ways is broken, the thin tube under the name a stent for drainage of bile from bilious ways to a small intestine or to a special package outside a body is inserted into a liver.
- Biopsy: removal of cells or a piece of fabric for a further research under a microscope regarding existence in them of tumoral process. The sample of material can be received by means of the fine needle entered into the bile-excreting canal during radiological or ultrasonography. Such procedure is called a fine-needle aspiration biopsy. Usually the biopsy is carried out to time of ERHP or ChChHG. Perhaps also removal of the site of fabric during an operative measure.
- Functional trials of a liver: in a sample of blood the content of certain substances which are produced by a liver is defined. The increased concentration of these substances in blood testifies to the liver disease caused by a tumor of bilious channels.

Cancer therapy of extrahepatic bilious ways:

The chance of recovery (forecast) of the patient and the choice of a method of treatment are influenced by certain factors.

The forecast (chance of recovery) and the choice of a method of treatment is defined by the following:
- Cancer stage (process distribution only on biliary tract or and on other bodies and fabrics).
- Whether the full surgical oncotomy is possible.
- Defeat by a tumor of the lower or upper parts of channels.
- For the first time the diagnosed disease or a recurrence (return) of a disease.

The choice of a method of treatment also depends on the symptoms caused by a tumor. Cancer of extrahepatic bilious channels usually is found already in the started cases when radical operation is not possible any more. Palliative treatment can facilitate symptoms of a disease and improve quality of life of the patient.

After establishment of the diagnosis of cancer of extrahepatic bilious channels the patient needs to carry out still some additional examination for establishment of extent of distribution of process only on biliary tract or and on other bodies and fabrics.

This process of definition of prevalence of malignant process in an organism is called a disease stadirovaniye. On the basis of data of a stadirovaniye establish a disease stage. It is necessary to know a stage for the choice of optimum treatment planning.

Stadirovaniye of cancer of extrahepatic bilious channels usually is followed by a laparotomy. On an abdominal wall do a section through which investigate abdominal organs regarding their defeat by malignant process, and also removal of fabrics or an intake of liquid for their further studying under a microscope. For definition of a stage of a disease also biopsies are considered given X-ray analyses, a laparoscopy. Sometimes a laparoscopy carry out before a laparotomy for definition of prevalence of a tumor. If cancer in the started stage and the surgeon cannot remove completely a new growth, he has the right not to carry out a laparotomy.

There are three ways of spread of a tumor on an organism.
Treats them:
- Germination in other fabrics. The malignant new growth sprouts the healthy fabrics surrounding it.
- On lymphatic system (a lymphogenous way). Tumor cells get into lymphatic system and on absorbent vessels can get to other bodies and tissues of the person.
- With a blood flow (gematogenno). Malignant cells get into veins and capillaries and with blood are carried in other bodies and fabrics.

When tumor cells with a blood flow or a lymph are carried on an organism perhaps formation of other (secondary) tumor. This process is called innidiation. Primary tumor and secondary (metastatic) new growths belong to the same type of cancer. For example, if the breast cancer extends to bones, then the tumor cells found in bones are malignant cells of a breast cancer. And the new growth in bones is carried to a metastatic breast cancer, but not to cancer of bones.

Development of cancer of extrahepatic bilious channels is divided into such stages:
Stage 0 or Carcinoma in situ ("cancer on site", lat.)
At a zero stage atypical cells are in the most inside layer of a mucous membrane. These cells can become malignant and get into the healthy fabrics surrounding them. The stage 0 still is called by carcinoma in situ.

The stage І cancer is subdivided into a stage ІА and a stage ІБ.
- Stage ІА: the tumor affects only a bilious channel.
- Stage ІБ: the tumor sprouts a wall of a bilious channel.

Stage ІІ
The stage ІІ is subdivided into a stage ІІ And yes a stage ІІ B.
- Stage ІІ And: the tumor affects a liver, a gall bladder, a pancreas and/or the right or left branch of a hepatic artery, or right/left branches of a portal vein.
- Stage ІІ B: the tumor affects nearby lymph nodes and:
1. it is found in a bilious channel; or
2. extends to a wall of a bilious channel; or
3. affects a liver, a gall bladder, a pancreas and/or the right or left branch of a hepatic artery, or right/left branches of a portal vein.

The recurrence of cancer of extrahepatic bilious channels is a return of a disease after the carried-out treatment. Cancer will burn repeatedly to arise in bilious channels or in other bodies and fabrics.

For cancer therapy of extrahepatic bilious channels there are various methods of therapy.

Some approaches are considered as standards of therapy (the treatment existing today), some – are investigated in clinical tests. Clinical tests are carried out for improvement of the existing methods of therapy or for data acquisition about efficiency of new approaches. If by results of such researches advantage of the studied treatment methods is proved, they can become the new standard of treatment. Many patients can discuss participation in clinical trials with the doctor. In some tests set of the patients who were not receiving treatment yet continues.

Apply 2 methods of standard treatment:
- Surgical method
At cancer of extrahepatic bilious channels perform the following operations:
- Removal of a bilious channel: if the tumor of the small sizes also is completely in a bilious channel, the last can be removed all. Novy Canal forms by connection of the mouth of a channel in a liver with intestines. Also lymph nodes which study then under a microscope for definition of existence in them of tumor cells are removed.
- Partial gepatektomiya: removal of a part of the liver affected with a tumor. The small site of fabric, the whole hepatic lobe or even the most part of a liver with surrounding healthy fabrics can be the deleted part.
- Vippl's procedure: operation consists in removal of a head of a pancreas, gall bladder, a part of a stomach, a piece of intestines and a bilious channel. The remained pancreas is enough for production of digestive juices and insulin.
- Biliary anastomosis: If it is impossible to remove a tumor, but it squeezes a small intestine and promotes accumulation of bile in a gall bladder, creation of a biliary anastomosis is possible. During this operation the gall bladder or a bilious channel cross and again attach to a small intestine on a bypass way, passing a tumor. Such procedure recovers outflow of the bile gathering in a gall bladder and reduces the jaundice caused by it.
- Stenting: if the tumor blocks a gleam of a bilious channel, for ensuring outflow of bile it is possible to insert a stent (a thin tube) into it. Other end of a drainage can выходиь on an external body surface or bend around a tumor and come to an end in a small intestine. The doctor can establish a stent during operation or ChChHG, or by means of the endoscope.

- Radiation therapy
Radiation therapy is a method of treatment of a tumor by means of high-frequency x-ray emission or other types of radiation. The method allows to achieve elimination of malignant cells or delay of growth of a tumor.
There are 2 types of radiation therapy. At external radiation therapy beams from the device which is near the patient go to a tumor. At internal radiation therapy radioactive materials gather in needles, tubes or catheters which are entered into the fabrics close to a tumor or directly into a new growth. The choice of a method of radiation therapy depends on type and a stage of malignant process.
In clinical trials new methods of treatment are developed.

The radio sensibilizing means
Clinical trials study possibilities of strengthening of action of radiation therapy on tumor cells. It can be reached in such ways:

- Hyperthermia: impact on body tissues high temperature for destruction of tumor cells or for increase in their sensitivity to radiation therapy and certain anticarcinogenic drugs.

- The radio sensibilizing means: The drugs promoting increase in sensitivity of tumor cells to radiation therapy. At a combination of radiation therapy and the radio sensibilizing means it is possible to destroy much more malignant cells.

- Chemotherapy
The chemotherapy is a method of cancer therapy by means of cytostatic drugs which effect is directed to destruction of tumor cells or delay of malignant growth. At reception of himiopreparat inside, introduction intravenously or intramusculary medicine comes to the general blood stream, destroying the tumor cells (system chemotherapy) circulating on an organism. If the chemotherapy is entered directly into the spinal canal, into the struck body or any cavity of an organism, for example, in belly, it is called regional chemotherapy. The type of introduction of himiopreparat depends on type and a stage of malignant process.

- Biological treatment or biotherapy
The biotherapy is based on resources of immune system of the patient for fight against cancer. The substances produced by an organism or in vitro are used for strengthening, the direction and resuming of natural protective system of the patient. This type of treatment is called still a biotherapy or an immunotherapy.
Patients can ask the doctor about participation in clinical trials.
For some patients participation in clinical trials can be the best choice of a method of treatment. Clinical tests are a part of process of studying of cancer. They are carried out to define whether new methods of treatment of a tumor are effective and safe and as far as they surpass standard approaches to therapy.

The majority of today's standards of treatment of malignant new growths are based on the previous clinical tests. The patients participating in researches can receive standard therapy or to be one of the first who receive new treatment.

The patients participating in clinical trials also influence improvement of methods of cancer therapy in the future. Even, if clinical testing does not lead to opening of new effective methods of controlling with a disease, very often on the basis of the received results it is possible to give answers to the major questions and to help with further studying of a problem. It is possible to take part in clinical tests to, in time or after antineoplastic treatment.

In one researches will see off set of patients without the previous therapy. Other tests study effect of drug at the patients who did not answer the treatment which is carried out before. There are also researches of new methods of prevention of a recurrence (returns) of a disease or decrease in side effects of drug.

After the end of treatment the patient needs to be followed up by a doctor.
After treatment for the purpose of assessment of efficiency of treatment repetition of some diagnostic procedures which are carried out earlier for establishment of the diagnosis or a stage of process can be required. On the basis of the received results the decision on continuation, change or the end of treatment is made. Such procedure is called a restadirovaniye.

Some inspections will regularly need to be repeated even after completion of treatment. Data of such inspections help to estimate a condition of the patient and in time to find a recurrence (return) of a disease. This process is called dispensary observation or regular routine inspections.

The localized cancer of extrahepatic bilious channels
Treatment of the localized cancer of extrahepatic bilious channels can include the following:
- Stenting or a biliary anastomosis for recovery of outflow of bile can be executed before operation for reduction of jaundice.
- Surgical intervention with or without external radiation therapy.

Inoperable cancer of extrahepatic bilious ways
Treatment of inoperable cancer of extrahepatic bilious channels can include the following:
- Stenting or a biliary anastomosis with or without external radiation therapy, as palliative treatment for reduction of manifestations of clinical symptoms and improvement of quality of life of the patient.
- Participation in clinical trials using a hyperthermia, radiosensebilizator, himio-or biotherapies.

Recurrence of cancer of extrahepatic bilious channels
Treatment of a recurrence of cancer of extrahepatic bilious channels can include the following:
- Palliative treatment for reduction of manifestations of clinical symptoms and improvement of quality of life of the patient.
- Clinical trials.