Wilms tumor: symptoms, treatment

There is not a single organ in the human body that would always remain healthy under any circumstances. Unfortunately, many diseases and defects are laid down during intrauterine development. We will talk about one of them in our article. Many people are unacquainted with Wilms' tumor. What it is, some do not even guess. In the article, we will try to understand the symptoms, causes and tactics of the treatment of this disease.

The concept of Wilms tumor

Wilms tumor, or, as it is also called, nephroblastoma, refers to malignant kidney diseases that occur most often in childhood. Usually it is diagnosed at the age of 2-5 years. Wilms tumor in adults is rare, so little has been learned.

Unfortunately, children who suffer from this disease have most often other deviations in their development. It is believed that the formation of a tumor can begin in the prenatal period, since a two-week-old baby can already detect it.

Wilms tumor is usually localized on one side, but there are also cases of bilateral renal damage. If you look at the diseased organ, you can notice that its surface is uneven, sections of hemorrhages and necrosis are visible on the incision, in some places cysts are present. In this case, it is said that the patient has a cystic tumor of Wilms.

At first, the tumor is localized only inside the kidneys. With the subsequent development, it grows into neighboring tissues and organs - thus spreading metastases.

Stages of development of the disease

This malignant tumor develops in several stages:

1. At the very beginning of the nephroblastoma it is located only in one kidney. Children during this period may not experience discomfort and have no complaints.
2. The tumor begins to spread beyond the kidney, but there are no metastases yet.
3. Further development is accompanied by sprouting of the capsule, and the lesion affects adjacent organs, lymph nodes.
4. Metastases are found in the liver, lungs, bones.
5. At the last stage of its development, Wilms' tumor in children is already affected by two kidneys.

Probably, it is not necessary to explain that the earlier the tumor is diagnosed, the more optimistic the forecast can be given to the patient.

Symptomatology of the disease

Since it is possible to diagnose pathology at different stages, the signs depending on this may vary. Most often, if there is a Wilms tumor, the symptoms are as follows:

• general malaise;
• weakness, and if the child is very small, then he constantly sleeps;
• decreased appetite;
• observed weight loss;
• in some cases, there is a high temperature;
• skin becomes pale;
• may increase blood pressure;
• blood test shows increased ESR and decreased hemoglobin;
• appears in the urine;
• in severe cases it is possible to observe an obstruction of the intestine, since the tumor squeezes it.

In any disease, the behavior of the baby changes, and Wilms' tumor in children is no exception. Mom can always immediately suspect that something is wrong with the child.

When the disease goes far, the tumor is well palpated with the hands. In this case, the baby feels pain, as the neighboring organs are squeezed, which prevents them from functioning normally. In some patients, the abdomen increases if Wilms' tumor is diagnosed. The photo below shows it well.

When a large number of metastases appear, the child begins to lose weight sharply. Most often, death occurs as a result of pulmonary insufficiency and excessive exhaustion of the body. What is noteworthy, the occurrence of metastases depends, rather, not on the size of the tumor, but more on the patient's age. It turns out that very young children with such a diagnosis are much more likely to recover completely.

But most often not only Wilms' tumor is diagnosed, it is usually accompanied by other developmental abnormalities.

Concomitant diseases

It is rare that this tumor is diagnosed as the only deviation in development. Most often it is accompanied by accompanying pathologies:

• hypospadias, when the urethra in boys opens somewhere in the middle of the penis or even in the perineum;
• enlarged internal organs, for example, a large tongue;
• one limb is larger than the other;
• cryptorchidism;
• absence of an iris in the eye( a feeling is created that the pupil is not surrounded by anything).

Diagnosis of Wilms tumor

When nephroblastoma is only at the very beginning of its development, it is difficult to make a correct diagnosis, as there is no pronounced symptomatology. It is for this reason that in most cases treatment is started already when the tumor is in 3 or 4 stages.

Parents need to closely monitor the condition of the child, and if there is pallor, high blood pressure, reduced tone, it is possible to probe the tumor, then a complete examination should be performed immediately. It usually includes the following types of diagnosis:

1. Blood and urine test. In urine, proteinuria, macrogematuria is found, and in the blood, ESR is increased, anemia is noted.
2. Urography allows you to make an accurate diagnosis in more than 70% of cases. It is possible to view the contours of the kidney, and in the presence of a tumor, slight changes in the shape of the organ and a violation of the function are evident.
3. Ultrasonic tomography also makes it easy to see the size of the tumor and its location.
4. Wilms tumor on ultrasound looks like a heterogeneous formation with cysts inside.
5. With the help of X-ray computed tomography, one can see not only a tumor in one kidney, but also analyze the condition of the second, and also see the presence of metastases in neighboring organs.
6. For large tumor sizes, angiography is usually prescribed. This is done to determine the location of the nephroblastoma relatively large vessels, so that during surgery the surgeon had an idea of ​​the state of affairs.
7. Ultrasound and CT can determine the extent of tumor metastasis in organs and tissues.

Currently, medical methods of research are at such a level of development that they make it possible to correctly diagnose in almost 95% of cases. But in 5% all the same mistakes occur, for example, if the multilocular cyst was taken for a Wilms tumor on ultrasound, it is only during the operation that it can be detected. At ultrasonic scanning accuracy makes 97%, and at KT is a little bit less. Although there are cases when the tumor of Wilms is combined with a cyst.

Further examination is aimed at detecting metastases, then to choose the right tactics for treating the disease. If the Wilms tumor develops in children, treatment is assigned in each case individually, taking into account the characteristics of the organism and the stage of the development of the disease.

Causes of development of nephroblastoma

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

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

1. Female gender. According to statistics, girls are more likely to have this disease than boys.
2. Hereditary continuity. Although cases of family illness are rare.
3. Belonging to the Negroid race. Among this type of population, the disease occurs 2 times more often.

In general, it can be noted that there are still no precise reasons for the development of Wilms tumor.

Treatment of nephroblastoma

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

In terms of nephroblastoma, the following methods are used to treat it:

• surgery;
• Radiation Therapy.

Also gives positive results in chemotherapy for Wilms tumor.

Tactics of treatment are selected individually for each small patient. It will depend not only on the stage of development of the tumor, but also on the state of the child's body.

Surgical intervention

Kidney removal is a nephrectomy. It can be of several types:

1. Simple. In this case, the surgeon during surgery removes the completely affected kidney. If the second organ is healthy, then it can work "for two".
2. Partial nephrectomy. During such surgery, the doctor removes only the cancerous tumor inside the kidney and the surrounding damaged tissue. Most often, such manipulation is forced to be carried out if the second kidney is already removed or too sick.
3. Radical removal. It is not only the kidney with the surrounding structures that is being sought, but also the ureter, the adrenal gland. Lymph nodes can also be removed if cancer cells are found in them.

During the operation it can always become clear that adjacent tissues are damaged, then it is necessary to navigate the situation and delete all infected. In severe cases, when the nephroblastoma( Wilms tumor) affects both kidneys in the child, it is necessary to remove both organs for the sake of saving the baby's life.

After bilateral nephrectomy, the child is prescribed hemodialysis to clear the blood of toxins. To make life after removal of both kidneys high-grade it is possible only after operation on transplantation of the given body from the donor which is difficult enough to find. Such patients sometimes stand in line for a transplant for years.

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

Chemotherapy in the treatment of nephroblastoma

If the tumor can not be removed, the treatment is reduced to other methods, for example, to the use of chemotherapy.

During this treatment, chemotherapy drugs are prescribed that contribute to the destruction of cancer cells. In medical practice for these purposes, the most commonly assigned:

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

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

• the process of hematopoiesis is disrupted;
• hair follicles are damaged, which causes baldness during chemotherapy;
• cells of the digestive tract suffer, which provokes loss of appetite, vomiting, nausea;
• decreases immunity, which makes the body more susceptible to various infections.

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

If a child is forced to prescribe a high dose of medications, the doctor should warn that this may cause bone marrow cells to be destroyed and offer them to freeze. After the treatment, they can be thawed and introduced into the baby's body, they will start their immediate duties for the production of blood cells. So the bone marrow, damaged during the chemotherapy, will be restored.

Radiation therapy

If the Wilms tumor is greatly progressing, treatment necessarily includes radiation therapy. It is combined with chemotherapy if the nephroblastoma is in the 3-4 stages of its development. Radiation therapy helps kill cancer cells that "escaped" from the surgeon during surgery.

During this procedure, it is important that the child lies still, because the rays are directly directed to the tumor zone. If the patient is very small and his immobility is problematic, then before the procedure he is administered a small dose of sedatives.

Irradiation requires that you follow the correct recommendations. The point at which the rays will be directed is labeled with a dye. All that should not fall into the irradiation zone is covered by special shields.

All precautions, however, do not save from side effects, which then arise:

• nausea;
• weakness;
• irritation on the skin at the site of irradiation;
• diarrhea.

The doctor should prescribe medications that will ease the patient's condition and remove side effects.

Therapy depending on the stage of the disease

For any disease, treatment is always selected individually. This is especially true of children. Wilms tumor also requires careful analysis and the selection of adequate therapies. It takes into account not only the stage of the disease, but also the age of the baby, his condition, and the characteristics of the organism.

1. In the first and second stages of tumor development, when the process affects only one kidney and the cancer cells are not aggressive, the treatment is usually to remove the affected organ and then chemotherapy. Radiation therapy may also be required.
2. 3 and 4 stages of nephroblastoma require a different approach. The tumor extends beyond the kidneys, so it can no longer be removed without touching the neighboring organs. In this case, surgical removal of part of the tumor is appointed followed by chemotherapy and irradiation.
3. Stage 5 is characterized by the appearance of pathology in both kidneys. Carry out partial removal of the tumor, while affecting the neighboring lymph nodes. After the operation, a course of radiation and chemotherapy is conducted. If you have to remove both kidneys, the patient is prescribed dialysis, and then a transplantation of healthy organs is required.

Children are all different, and everyone has an individual reaction to the therapy of a tumor. Before agreeing to a treatment plan, parents need to discuss all the issues with the doctor. They need to know what effect the drugs have on the body, how to avoid or mitigate side effects during treatment.

Conducting clinical trials of

Every year, there are new methods and methods for treating certain diseases. This is particularly true of cancerous tumors, since the mortality from them is at a high level.

In many countries, not only adults, but also children can participate in clinical trials of the newest treatments Wilms swelled up. Parents can ask the doctor if such tests are conducted in our country, whether there is an opportunity to participate in them.

If the probability exists, the physician should give full information about the safety of the treatment. It is worth noting that often participation in such experimental tests gives an additional chance in the fight against the disease.

But the doctor should also warn that in the course of treatment unpredictable side effects can occur which have not been seen before, and no one can guarantee 100% cure.

Relapse

It is important not only to cope with the disease, but also to ensure that there is no recurrence. Unfortunately, this happens with nephroblastoma both during therapy and after it. Analyzes are taken, and if they are satisfactory, then reserve schedules of chemotherapeutic drugs are prescribed:

• Vincristine.
• "Doxorubicin".
• "Cyclophosphamide" in combination with "Dactinomycin" or "Ifosfamide".
• Carboplatin.

Each case is individual, and in some cases a high dose of chemotherapy is prescribed for some patients, after which blood stem cell transplantation is performed. The best effect will be if you combine surgery with radiation and polychemotherapy.

Wilms tumor in anamnesis in a pregnant woman

Kidneys in the human body perform one of the main functions - purification of blood from toxins and metabolic products. During pregnancy, they bear a double responsibility not only for the condition of the mother, but also for the life of a new, developing organism.

If the expectant mother underwent an operation to remove the kidney long before the onset of pregnancy, then the prognosis may be quite favorable. Many women safely endure pregnancy and give birth to healthy children.

But there are exceptions, when a new condition can trigger a relapse of the disease. Then it is necessary to interrupt pregnancy in order to save the life of a woman.

Sometimes a tumor of Wilms transferred in childhood can provoke the development of pyelonephritis, and it can also affect the functional development of the fetus. Even in spite of the fact that most often the outcome of pregnancy is favorable, the risk of metastasis persists and 10-20 years after removal of the kidney.

Prognosis for a patient with nephroblastoma

To give a definite prognosis in the presence of Wilms' tumor, it is necessary to evaluate its size, stage of development and histological analysis. The most unfavorable is the presence of a tumor more than 500 g and in 3-4 stages of development with a poor histology.

If a comprehensive adequate therapy scheme is prescribed, then the patients' survival is about 60%.In the initial stages, the tumor lends itself to more effective treatment, recovery occurs in 80-90% of people.

Given that there is no special preventive measure for such a disease, it can only be recommended to undergo a regular examination if the child is at risk. The earlier a tumor is found, the higher the chances of recovery.