Wilms tümörü: belirtileri, tedavi

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There is in the human body is no body that would always and under all circumstances to stay healthy.Unfortunately, many diseases and defects laid even during fetal development.On one of them we will talk in this article.Many unfamiliar with Wilms' tumor.What is it, some do not even know.This article will try to deal with the symptoms, causes and treatment of this disease tactics.

concept of Wilms tumor

Wilms' tumor, or as it is also called nephroblastoma, is a malignant disease of the kidneys that appear most often in children.Usually it is diagnosed between the ages of 2-5 years.Wilms' tumor in adults is rare, so little studied.

K Unfortunately, children who suffer from this disease are often other deviation in its development.It is believed that the formation of tumors can begin in utero, as already have a two-week baby can detect it.

Wilms' tumor is usually localized to one side, but there are cases, and bilateral renal damage.If you look at the patient body may be noted that its surface is uneven, the cut visible areas of hemorrhage and necrosis, in some places there are cysts.In this case we say that the patient has cystic Wilms tumor.

At first, the tumor is localized within the kidneys.With the subsequent development, it grows into the surrounding tissue and organs - thus spread metastases.

stage of the disease

This malignant tumor in its development several steps:

  1. At the beginning of nephroblastoma is only one kidney.Children at this time may not experience discomfort and have no complaints.
  2. tumor begins to spread beyond the kidney, but metastases are not yet available.
  3. Further development is accompanied by a germination capsules and defeat affect adjacent organs, lymph nodes.
  4. are detected metastases in the liver, lungs, bones.
  5. At the last stage of the development of Wilms tumor in children already struck two kidneys.

probably should not explain that the earlier a tumor is diagnosed, the more optimistic outlook can give the patient.Symptoms of the disease

Since the pathology can be diagnosed in different stages, the signs and depending on this may vary.In most cases, if there is a Wilms' tumor, the symptoms are detected following:

  • malaise;
  • weakness, and if the child is very young, he is constantly awake;
  • decreased appetite;
  • observed weight reduction;
  • in some cases there is a high temperature;
  • skin become pale;
  • may increase blood pressure;
  • blood test shows an increased erythrocyte sedimentation rate and decreased hemoglobin;
  • blood appears in the urine;
  • in severe cases, can be observed intestinal obstruction, as the tumor compresses it.

If any disease is changing the behavior of the child, and Wilms' tumor in children is no exception.Mom always immediately may suspect that a child that something is wrong.

When the disease goes away, then the tumor is well detectable hands.At the same time the baby feels pain because compression of adjacent organs occurs that prevents them from functioning normally.In some patients, increased stomach if diagnosed Wilms' tumor.The photo below shows it well.

When a large number of metastases, the child begins to lose weight dramatically.In most cases, death occurs due to respiratory failure and excessive exhaustion.Remarkably, the appearance of metastases depends rather on the size of the tumor does not, but more on the age of the patient.It turns out that in very young children with this diagnosis much better chance of full recovery.

But more often than not only diagnosed Wilms' tumor, it is usually accompanied by other developmental disorders.

Comorbidities

Enough is seldom that the tumor is diagnosed as the only deviation in development.Most often it is accompanied by comorbidities:

  • hypospadias when the urethra in boys opens somewhere in the middle of the penis, or even in the crotch;
  • enlarged internal organs, for example, large tongue;
  • one limb more than the other;
  • cryptorchidism;
  • absence of the iris of the eye (a feeling that no pupil is surrounded).

diagnosis of Wilms tumor

When nephroblastoma is only at the very beginning of its development, the correct diagnosis is difficult, because no pronounced symptoms.For this reason, in most cases already start treatment when the tumor is on the 3 or 4 steps.

Parents need to closely monitor the condition of the child, and if there are pallor, high blood pressure, decreased tone, it is possible to test the tumor, you should immediately undergo a full examination.It usually includes the following types of diagnostics:

  1. analysis of blood and urine.Found in the urine proteinuria, gross hematuria, and blood sedimentation rate increased, there is anemia.
  2. Urography allows accurate diagnosis in more than 70% of cases.Can not view outline of the kidneys, and in the presence of the tumor visible slightest changes in body shape and dysfunction.
  3. With the help of ultrasound imaging is also easy to see the size of the tumor and its location.
  4. Wilms' tumor in the US looks like a non-uniform formation of cysts inside.
  5. using X-ray computed tomography is possible to see not only the tumor in one kidney, but also to analyze the state of the second, as well as view the presence of metastases in the adjacent organs.
  6. For large size of the tumor is usually prescribed angiography.This is done in order to further clarify the location nephroblastomas relatively large vessels that during the operation the surgeon had an idea of ​​the situation.
  7. ultrasound and CT can determine the degree of metastasis in organs and tissues.

Currently, medical research methods are at such a level of development that allows the correct diagnosis in almost 95% of cases.But 5% is still errors occur, for example, if an ultrasound multilokulyarnuyu cyst mistaken for Wilms' tumor, it was only during the operation it is possible to detect.Ultrasound scanning accuracy is 97% and in CT a little less.Although there are cases when the Wilms tumor is combined with a cyst.

Further examination is directed to the detection of metastases, then to choose the right tactics treatment of disease.If developing Wilms' tumor in children, treatment is given in each case individually, taking into account the characteristics of the organism and the stage of the disease.

Causes of nephroblastoma

say with certainty that the cause of the tumor is not possible.It is believed that the reason for it is a mutation in the DNA of cells.In a small percentage of cases it is transmitted from parents to children.But most researchers did not find a relationship between heredity and development of Wilms tumor.

Doctors believe that there are some risk factors that lead to the emergence of nephroblastoma.These include the following:

  1. female.According to statistics, girls often suffer from this disease than boys.
  2. hereditary succession.Although cases of family diseases are quite rare.
  3. belonging to the Negroid race.Among this type of disease occurs in population 2 times more often.

In general it can be noted that so far have not established the exact causes of Wilms tumor.

Treatment nephroblastomas

As a rule, treatment of any disease can be approached from different angles.When found Wilms tumor in children, treatment is given on the results of histological examination.Experts believe that a good histology gives a more favorable prognosis.But this does not mean that bad analyzes serve as a signal to stop the treatment.

Speaking of nephroblastoma, is to treat it using the following methods:

  • surgery;
  • radiotherapy.

also gives positive results chemotherapy for Wilms tumor.

Tactics of treatment is chosen individually for each little patient.It will depend not only on the stage of the tumor, but also on the condition of the body of the child.

Surgery

Removing the kidney - is nefroektomiya.It is of several types:

  1. simple.In this case, the surgeon during surgery completely removes the affected kidney.If the second body is healthy, it could work "for two".
  2. Partial nefroektomiya.During this surgery, the doctor removes only the cancerous tumor within the kidney and surrounding tissue damage.Most often such manipulation have to spend if the second kidney has been removed or too sick.
  3. Radical removal.Withdraws not only with the surrounding structures of the kidney, and the ureter, the adrenal gland.Lymph nodes may also be removed if they are found in cancer cells.

During the operation can always find that the surrounding tissue is damaged, then it is necessary to focus on the situation and to remove all contamination.In severe cases, when nephroblastoma (Wilms' tumor) affects both kidneys at the child, it is necessary to save the life of the baby to remove the two bodies.

after bilateral nephrectomy, the child is prescribed dialysis to cleanse the blood of toxins.Make life after removal of both kidneys may be complete only after transplants of organ from the donor, which is quite difficult to find.These patients sometimes for years are in the queue for a transplant.

After surgery to remove the tumor it is sent for analysis to study the specifics of the cancer cells, their aggressiveness, rate of reproduction.It is necessary to find the most effective course of chemotherapy.

chemotherapy in the treatment of nephroblastoma

If the tumor is not possible to remove the treatment is reduced to other methods such as the use of chemotherapy.

During this treatment prescribed chemotherapy, which contribute to the destruction of cancer cells.In medical practice for these purposes often appointed:

  • «Vincristine."
  • «Dactinomycin."
  • «Doxorubicin."

Such treatment is quite difficult for the organism, there are changes in the work of many internal organs, for example:

  • disrupted the process of hematopoiesis;
  • damaged hair follicles, and this is caused by hair loss during chemotherapy;
  • suffer gastrointestinal cells that provokes the loss of appetite, vomiting, nausea;
  • reduced immunity, making the body more susceptible to infections.

Parents should before taking chemotherapy drugs to ask your doctor about possible side effects and to take the necessary measures to support the child's body.

If the child is forced to appoint a high dose of drugs, the doctor should warn you that this may cause destruction of bone marrow cells, and offer them to freeze.After treatment they can be thawed and put into the body baby, they start to their direct responsibilities for the production of blood cells.Since bone marrow damaged by chemotherapy, will be restored.

Radiotherapy

If much progress Wilm's tumor, treatment must include more and radiotherapy.It is combined with chemotherapy if nephroblastoma is 3-4 stage of its development.Radiation therapy helps kill cancer cells that have "escaped" from the surgeon during the operation.

During this procedure, it is important that the child lay motionless, as directly on the tumor sent rays.If the patient is very small and difficult to achieve stillness, the procedure before it is administered a small dose of sedatives.

irradiation requires compliance with precise recommendations.The point on which will be sent rays labeled with dye.All that should be in the area of ​​radiation, is covered with special shields.

all precautions, however, does not protect from the side effects that then arise:

  • nausea;
  • weakness;
  • irritation at the site of exposure;
  • diarrhea.

physician should designate drugs that facilitate the patient's condition and remove the side effects.

Therapy, depending on the stage of the disease

Any illness treatment is always selected individually.This is especially true for children.Wilms' tumor also requires careful analysis and selection of appropriate therapies.It takes into account not only the stage of the disease, and the age of the child, his condition, especially the body.

  1. the first and second stages of tumor development when the process involves only one kidney cancer cells and do not differ aggressive treatment typically involves the removal of the affected organ, and subsequent chemotherapy.It may take more and radiotherapy.
  2. 3 and 4 stage nephroblastomas require a different approach.The tumor extends beyond the kidney, so it is no longer possible to remove without hitting adjacent organs.In this case, the prescribed surgical removal of the tumor, followed by chemotherapy and irradiation.
  3. 5 stage is characterized by the appearance of disease in both kidneys.Carry out a partial removal of the tumor, and thus affect the nearby lymph nodes.After surgery a course of radiation and chemotherapy.If you have to remove both kidneys, the patient can be dialyzed, and then you want to change healthy organs.

Children are all different, and each individual body's response to cancer therapy.Before agreeing to a treatment plan, parents need to discuss all issues with your doctor.They need to know what effect drugs have on the body, how to avoid or mitigate the side effects during treatment.

Clinical trials

Every year there are new techniques and methods for the treatment of certain diseases.This is particularly true of cancerous tumors, as mortality from them is high.

In many countries, not only adults, but children may participate in clinical trials of new treatments for swollen Wilms.Parents can ask your doctor whether such tests are carried out in our country, there is a possibility to take part in them.

If the probability is there, the doctor should give full information on the safety of treatment.It is worth noting that often participate in such experimental tests give an extra chance in the fight against disease.

But the doctor must also be prevented and that in the course of treatment might cause unpredictable side effects that have not been met, as well as 100% cure one can not guarantee.

Relapse

is important not only to cope with the disease, but also to ensure that it did not have a relapse.Unfortunately, it happens with nephroblastoma both during treatment and after it.Take the analysis, and if they are satisfactory, the reserve scheme administered chemotherapy drugs:

  • «Vincristine."
  • «Doxorubicin."
  • "Cyclophosphamide" in combination with "dactinomycin" or "ifosfamide."
  • «carboplatin."

Each case is different and some patients relapse when administered high dose of chemotherapy, followed by transplantation of blood stem cells.Best of all would be the effect if we combine surgery with radiotherapy and polychemotherapy.

history of Wilms' tumor in a pregnant

kidneys in the human body to perform one of the main functions - cleaning the blood of toxins and metabolic products.