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Wilms tumor: symptoms, treatment

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

Wilms tumor concept

Wilms tumor, or, as it is also called,nephroblastoma, refers to the malignant diseases of the kidneys, which appear most often in childhood. It is usually diagnosed at the age of 2-5 years. Wilms tumor in adults is quite rare, therefore, little studied.

wilms tumor

Unfortunately, children who suffer fromdisease, most often have other abnormalities in their development. It is believed that the formation of a tumor can begin in the prenatal period, since it is already possible to detect it in a two-week baby.

Wilms tumor is usually localized withon the one hand, but there are cases of bilateral kidney damage. If you look at a diseased organ, it can be noted that its surface is uneven, there are areas of hemorrhage and necrosis on the section, in some places there are cysts. In this case, the patient is said to have a Wilms cystic tumor.

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 goes through several stages in its development:

  1. At the very beginning nephroblastoma 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 still no metastases.
  3. Further development is accompanied by the germination of the capsule, and the lesion affects the adjacent organs, the lymph nodes.
  4. Metastases are found in the liver, lungs, bones.
  5. At the last stage of its development, Wilms' tumor in children affects already two kidneys.

wilms tumor in children

Probably, it is not worth explaining that the earlier a tumor is diagnosed, the more optimistic the prognosis can be given to the patient.

Symptomatology of the disease

Since pathology can be diagnosed at different stages, the signs may differ depending on this. Most often, if there is a Wilms tumor, the symptoms are as follows:

  • general malaise;
  • weakness, and if the child is very small, he is constantly sleeping;
  • a decrease in appetite;
  • there is a decrease in weight;
  • in some cases, there is a high temperature;
  • the skin becomes pale;
  • blood pressure may increase;
  • blood test shows increased ESR and decreased hemoglobin;
  • blood appears in the urine;
  • in severe cases, intestinal obstruction can be observed, as the tumor squeezes it.

With any disease, the baby's behavior 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 tumorwell felt by hand. In this case, the baby feels pain, as squeezing of neighboring organs occurs, which prevents them from functioning normally. Some patients have an enlarged abdomen if a Wilms tumor is diagnosed. The photo below shows it well.

wilms swelling symptoms

When a large number of metastases appear,the child starts to lose weight sharply Most often death occurs as a result of pulmonary insufficiency and excessive exhaustion of the body. Remarkably, 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.

But it is not only the Wilms tumor that is most often diagnosed, it is usually accompanied by other developmental disorders.

Accompanying illnesses

It rarely happens that a given tumor is diagnosed as the only developmental disorder. Most often, it is accompanied by comorbidities:

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

Wilms tumor diagnosis

When nephroblastoma is located only in thethe beginning of its development, to make the correct diagnosis is quite difficult, since it is not observed pronounced symptoms. For this reason, in most cases, treatment begins already when the tumor is at stage 3 or 4.

Parents need to keep a close eye oncondition of the child, and if pallor, increased pressure, reduced tone are observed, it is possible to probe the tumor, then you should immediately undergo a full examination. It usually includes the following types of diagnostics:

  1. Blood and urine test. In the urine proteinuria, gross hematuria is detected, and ESR is increased in the blood, 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, the slightest changes in the shape of the organ and impaired function are noticeable.
  3. Using ultrasound tomography, it is also 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, it is possible to see not only a tumor in one kidney, but also to analyze the condition of the second, as well as to look for the presence of metastases in neighboring organs.
  6. For large sizes, tumors are usually prescribed.angiography. This is done in order to ascertain the location of the nephroblastoma of relatively large vessels, so that during the operation the surgeon has an idea of ​​the situation.
  7. Ultrasound and CT can determine the degree of tumor metastasis to organs and tissues.

Wilms tumor on ultrasound

Currently medical research methodsare at a level of development that allows you to make the correct diagnosis in almost 95% of cases. But in 5% errors still occur, for example, if an ultrasound scan of a multilocular cyst was mistaken for a Wilms tumor, then only during the operation can it be detected. With ultrasound scanning, the accuracy is 97%, and with CT it is slightly less. Although there are cases when a Wilms tumor is combined with a cyst.

Further examination is aimed at detectingmetastases, then to choose the right tactics of treatment of the disease. If a Wilms tumor develops in children, the treatment is prescribed in each specific case, taking into account the characteristics of the organism and the stage of the disease development.

Causes of nephroblastoma

Accurately say what is causingtumor development, impossible. It is believed that the cause is a mutation in the DNA of cells. In a small percentage of cases, it is transmitted from parents to children. But more often than not, researchers find no connection between heredity and the development of Wilms' tumor.

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

  1. Female. According to statistics, girls are more likely to suffer from this disease than boys.
  2. Hereditary succession. Although cases of marital disease are quite 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 the exact causes of the development of the Wilms tumor have not yet been established.

Nephroblastoma treatment

As a rule, for the treatment of any disease cancome up from different sides. When a Wilms tumor is detected in children, treatment is prescribed based on the results of a histological examination. Experts believe that a good histology gives a more favorable prognosis. But this does not mean that bad tests are a signal to stop treatment.

If we talk about nefroblastoma, then for its treatment using the following methods:

  • operative intervention;
  • radiation therapy.

Chemotherapy also gives positive results in Wilms tumors.

wilms tumor in children

Tactics of treatment is chosen 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 nephroectomy. It is of several types:

  1. Simple. In this case, the surgeon removes the completely affected kidney during the operation. If the second organ is healthy, then it can work “for two”.
  2. Partial nephroectomy. During this operation, the doctor removes only the cancer inside the kidney and the adjacent damaged tissue. Most often, such manipulation is forced to be carried out, if the second kidney is already removed or also the patient.
  3. Radical removal. Not only the kidney with the surrounding structures is removed, but also the ureter, adrenal gland. Lymph nodes can also be removed if cancer cells are found in them.

During the operation can alwaysIt turns out that the neighboring tissues are damaged, then you need to navigate the situation and remove all infected. In severe cases, when nephroblastoma (Wilms tumor) affects both kidneys in a child, it is necessary to remove both organs in order to save the baby’s life.

After bilateral nephrectomy, the child is prescribedhemodialysis to clear the blood of toxins. It is possible to make life after the removal of both kidneys of full value only after the operation to transplant this organ from a donor, which is quite difficult to find. Such patients sometimes stand in a queue for transplantation.

After surgery to remove the tumor, it is sent.on the analysis to study the specificity of cancer cells, their aggressiveness, the rate of reproduction. This is necessary to select the most effective course of chemotherapy.

Chemotherapy in the treatment of nephroblastoma

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

During such treatment chemotherapy drugs are prescribed that contribute to the destruction of cancer cells. In medical practice for this purpose are most often assigned:

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

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

  • blood process is broken;
  • hair follicles are damaged, and this is caused by baldness during chemotherapy;
  • Gastrointestinal cells suffer, which causes loss of appetite, vomiting, nausea;
  • immunity decreases, which makes the body more susceptible to various infections.

chemotherapy for Wilms tumor

Parents are faced with taking medication for chemotherapy, asking the doctor about possible side effects and taking the necessary measures to support the child’s body.

If the child is forced to assign a high dosedrugs, the doctor must warn that this may cause the destruction of bone marrow cells, and offer them freezing. After the treatment they can be defrosted and introduced into the body of the baby, they will take up their direct responsibilities for the production of blood cells. So the bone marrow, which was injured during chemotherapy, will recover.

Radiation therapy

If Wilms' tumor progresses strongly,treatment necessarily includes more and radiation therapy. It is combined with chemotherapy if the nephroblastoma is at 3-4 stages of its development. Radiation therapy helps kill cancer cells that “slipped away” from the surgeon during surgery.

During such a procedure it is important thatthe child was lying motionless, because rays are directed directly to the tumor zone. If the patient is very small and his immobility is problematic, then before the procedure he is given a small dose of sedatives.

Irradiation requires adherence to accurate recommendations. The point at which the rays will be directed is marked with a dye. Everything that should not get into the irradiation zone is covered with special shields.

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

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

The doctor must prescribe drugs that will ease the patient's condition and relieve side effects.

Therapy depending on the stage of the disease

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

  1. In the first and second stages of tumor development, whenthe process affects only one kidney and the cancer cells are not aggressive, the treatment, as a rule, consists in the removal of the affected organ and subsequent chemotherapy. Radiation therapy may also be required.
  2. Stage 3 and 4 of nephroblastoma require anotherapproach. The tumor extends beyond the kidney, so it can no longer be removed without affecting the adjacent organs. In this case, surgical removal of a part of the tumor, followed by chemotherapy and radiation, is prescribed.
  3. Stage 5 is characterized by the appearance of pathology inboth kidney. Partial removal of the tumor is carried out, while adjacent lymph nodes are affected. After surgery, a course of radiation and chemotherapy. If both kidneys are to be removed, dialysis is prescribed for the patient, and then transplantation of healthy organs is required.

Children are all different, and each individualbody reaction to tumor therapy. Before agreeing to a treatment plan, parents need to discuss all 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

Every year there are new methods and methods for treating various diseases. This is particularly true for cancer tumors, since their mortality is high.

In many countries, not only adults, but also childrencan participate in the clinical trials of the latest treatment for swollen Wilms. Parents may ask the doctor if such tests are carried out in our country, whether there is an opportunity to take part in them.

If there is a probability, the doctor should givefull information about the safety of treatment. It is worth noting that often participation in such experimental trials gives an additional chance in the fight against ailment.

But the doctor must also warn thatIn the course of treatment, unpredictable side effects that have not occurred before may occur, and no one can guarantee a 100% cure.

Relapse

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

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

Each case is individual, and some patientsin case of recurrence, a high dose of chemotherapy drugs is prescribed, followed by transplantation of stem blood cells. The best effect would be if you combine surgery with radiation and polychemotherapy.

Wilms tumor in history in pregnant

The kidneys in the human body perform one ofthe main functions are to clean the blood of toxins and metabolic products. During pregnancy, they bear double responsibility not only for the state of the mother, but also for the life of a new, developing organism.

a history of Wilms' tumor in a pregnant woman

If the expectant mother underwent surgery to removethe kidneys long before the onset of pregnancy, the prognosis can be quite favorable. Many women safely endure pregnancy and give birth to healthy babies.

But there are exceptions, when a new condition can provoke a relapse of the disease. Then you have to interrupt pregnancy to save the life of a woman.

Occasionally childhood Wilm’s tumorcan provoke the development of pyelonephritis, as well as it can affect the functional development of the fetus. Even though most often the outcome of pregnancy is favorable, the danger of metastasis persists 10–20 years after the removal of the kidney.

Prognosis for a patient with nephroblastoma

To give a specific prediction if availableWilms tumors, it is necessary to assess its size, stage of development and histological analysis. The presence of a tumor of more than 500 g is considered the most unfavorable, and it is of 3-4 stages of development with poor histology.

If a comprehensive adequate scheme is prescribedtherapy, then patient survival is about 60%. At the initial stages, the tumor gives in to even more effective treatment, recovery occurs in 80-90% of people.

Given that special preventive measures suchthe disease does not exist, it can only be recommended to be regularly examined if the child is at risk. The sooner a tumor is detected, the higher the chances of recovery.

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