There is not a single organ in the human body that would always and under any circumstances remain he althy. Unfortunately, many diseases and defects are laid even during fetal development. We will talk about one of them in our article. Many people don't know Wilms' tumor. What it is, some do not even know. In the article we will try to deal with the symptoms, causes and tactics of treating this disease.
Wilms tumor concept
Wilms tumor, or, as it is also called, nephroblastoma, refers to malignant diseases of the kidneys that appear most often in childhood. It is usually diagnosed at the age of 2-5 years. Wilms' tumor in adults is quite rare, so little studied.
Unfortunately, children who suffer from this disease most often have other deviations in their development. It is believed that the formation of a tumor can begin in the prenatal period, since it can already be detected in a two-week-old baby.
Wilms tumor is usually localized on one side, but there are cases andbilateral kidney injury. If you look at the diseased organ, it can be noted that its surface is uneven, areas of hemorrhage and necrosis are visible on the cut, and cysts are present in some places. In this case, the patient is said to have cystic Wilms' tumor.
At first, the tumor is localized only inside the kidneys. With subsequent development, it grows into neighboring tissues and organs - this is how metastases spread.
Stages of disease progression
This malignant tumor goes through several stages in its development:
- At the very beginning, nephroblastoma is located in only one kidney. Children during this period may not experience discomfort and have no complaints.
- Tumor begins to spread outside the kidney, but no metastases yet.
- Further development is accompanied by the germination of the capsule, and the lesion affects neighboring organs, lymph nodes.
- Metastases are found in the liver, lungs, bones.
- At the last stage of its development, Wilms tumor in children already affects two kidneys.
Probably, it is not worth explaining that the earlier the tumor is diagnosed, the more optimistic the prognosis can be given to the patient.
Symptoms of the disease
Since pathology can be diagnosed at different stages, the signs may vary depending on this. Most often, if Wilms' tumor is present, the symptoms are as follows:
- general malaise;
- weakness, and if the child is completelysmall, he constantly sleeps;
- appetite reduction;
- weight loss observed;
- high temperature in some cases;
- skin becomes pale;
- blood pressure may rise;
- blood test shows elevated ESR and decreased hemoglobin;
- blood appears in the urine;
- in severe cases, intestinal obstruction can be observed, as the tumor compresses it.
With 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 felt by hand. At the same time, the baby feels pain, as neighboring organs are squeezed, which prevents them from functioning normally. In some patients, the abdomen enlarges if Wilms' tumor is diagnosed. The photo below demonstrates this well.
When a large number of metastases appear, the child abruptly begins to lose weight. 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 age of the patient. It turns out that very young children with such a diagnosis are much more likely to make a full recovery.
But not only Wilms' tumor is most often diagnosed, it is usually accompanied by other developmental abnormalities.
Comorbidities
It rarely happens that this tumor is diagnosed as the only developmental abnormality. 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 perineum;
- enlarged internal organs, such as a large tongue;
- one limb is larger than the other;
- cryptorchism;
- absence of the iris in the eye (there is a feeling that the pupil is not surrounded by anything).
Wilms tumor diagnosis
When nephroblastoma is only at the very beginning of its development, it is rather difficult to make a correct diagnosis, since there are no pronounced symptoms. It is for this reason that in most cases treatment is started already when the tumor is in stage 3 or 4.
Parents need to closely monitor the child's condition, and if pallor, high blood pressure, reduced tone are observed, it is possible to probe the tumor, then a full examination should be immediately completed. It usually includes the following types of diagnostics:
- Blood and urine test. Proteinuria, macrohematuria are found in the urine, and ESR is increased in the blood, anemia is noted.
- Urography allows you to make an accurate diagnosis in more than 70% of cases. It is possible to see the contours of the kidney, and in the presence of a tumor, the slightest changes in the shape of the organ and dysfunction are noticeable.
- Ultrasound tomography also makes it easy to see the size of the tumor and its location.
- Wilms tumor onUltrasound looks like a heterogeneous formation with cysts inside.
- 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 one, as well as to see the presence of metastases in neighboring organs.
- For large tumors, angiography is usually prescribed. This is also done in order to clarify the location of nephroblastoma relative to large vessels, so that during the operation the surgeon has an idea of the state of affairs.
- Ultrasound and CT can determine the degree of tumor metastasis to organs and tissues.
Currently, medical research methods are at such a level of development that they make it possible to make a correct diagnosis in almost 95% of cases. But in 5%, mistakes still occur, for example, if a multilocular cyst was mistaken for a Wilms tumor on ultrasound, 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 Wilms tumor is combined with a cyst.
Further examination is aimed at detecting metastases, in order to choose the right tactics for treating the disease. If Wilms' tumor develops in children, treatment is prescribed in each case individually, taking into account the characteristics of the organism and the stage of development of the disease.
Causes of nephroblastoma
It is impossible to say exactly what causes the development of a tumor. It is believed that this is due to a mutation in the DNA of cells. In a smallpercentage of cases, it is passed from parents to children. But most often, researchers do not find a link between heredity and the development of Wilms' tumor.
Doctors believe there are some risk factors that lead to nephroblastoma. These include the following:
- Female. According to statistics, girls are more likely to get this disease than boys.
- Hereditary succession. Although familial cases are rare.
- 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 Wilms' tumor have not yet been established.
Treatment of nephroblastoma
As a rule, the treatment of any disease can be approached from different angles. 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 nephroblastoma, then the following methods are used to treat it:
- surgery;
- radiotherapy.
Chemotherapy also works well for Wilms tumor.
Treatment tactics are chosen individually for each little patient. It will depend not only on the stage of tumor development, but also on the state of the child's body.
Surgicalinterference
Removing a kidney is a nephrectomy. It comes in several types:
- Simple. In this case, the surgeon removes the completely affected kidney during the operation. If the second organ is he althy, then it may well work "for two".
- Partial nephrectomy. During such an operation, the doctor removes only the cancerous tumor inside the kidney and adjacent damaged tissue. Most often, such a manipulation is forced to be carried out if the second kidney has already been removed or is also sick.
- Radical removal. Not only the kidney with surrounding structures is removed, but also the ureter, adrenal gland. Lymph nodes may also be removed if cancer cells are found in them.
During the operation, it can always turn out that neighboring tissues are damaged, then you need to navigate the situation and remove everything infected. In severe cases, when nephroblastoma (Wilms' tumor) affects both kidneys in a child, both organs have to be removed to save the baby's life.
After a bilateral nephrectomy, the child is given hemodialysis to cleanse the blood of toxins. It is possible to make life full after the removal of both kidneys only after an operation to transplant this organ from a donor, which is quite difficult to find. Such patients sometimes stand in line for transplants for years.
After the operation to remove the tumor, it is sent for analysis to study the specifics of cancer cells, their aggressiveness, and the rate of reproduction. This is necessary to find the most effective course of chemotherapy.
Chemotherapy in treatmentnephroblastoma
If the tumor cannot be removed, then treatment is reduced to other methods, such as the use of chemotherapy.
During such treatment, chemotherapy drugs are prescribed that help destroy cancer cells. In medical practice, for these purposes, the following are most often prescribed:
- Vincristine.
- "Dactinomycin".
- "Doxorubicin".
Such treatment is quite difficult for the body, there are changes in the work of many internal organs, for example:
- hematopoiesis process is disturbed;
- hair follicles are damaged, causing baldness during chemotherapy;
- cells of the gastrointestinal tract suffer, which provokes loss of appetite, vomiting, nausea;
- immunity decreases, making the body more susceptible to various infections.
Parents should ask the doctor about possible side effects before starting chemotherapy drugs and take the necessary measures to support the child's body.
If a child is forced to prescribe a high dose of drugs, the doctor should warn that this may cause the destruction of bone marrow cells, and suggest freezing them. After the treatment, they can be thawed and introduced into the baby's body, they will begin their direct duties in the production of blood cells. So the bone marrow damaged during chemotherapy will be restored.
Radiation therapy
If strongWilms tumor progresses, treatment necessarily includes radiation therapy. It is combined with chemotherapy if nephroblastoma is at stages 3-4 of its development. Radiation therapy helps kill cancer cells that "elude" the surgeon during surgery.
During this procedure, it is important that the child lies still, because the rays are directed directly to the tumor area. If the patient is very small and it is problematic to achieve immobility, then a small dose of sedatives is administered to him before the procedure.
Irradiation requires adherence to precise recommendations. The point to which the rays will be directed is marked with a dye. Everything that should not fall into the radiation zone is covered with special shields.
All precautions, however, do not save from side effects that then occur:
- nausea;
- weakness;
- irritation on the skin at the site of irradiation;
- diarrhea.
The doctor should prescribe drugs that will alleviate 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 baby, his condition, and the characteristics of the organism.
- At the first and second stages of tumor development, when the process affects only one kidney and cancer cells do not differ in aggressiveness,treatment usually consists of removal of the affected organ and subsequent chemotherapy. Radiation therapy may also be needed.
- 3 and 4 stages of nephroblastoma require a different approach. The tumor extends beyond the kidney, so it can no longer be removed without hitting neighboring organs. In this case, surgical removal of part of the tumor is prescribed, followed by chemotherapy and radiation.
- 5 stage is characterized by the appearance of pathology in both kidneys. A partial removal of the tumor is carried out, while affecting the neighboring lymph nodes. After the operation, a course of radiation and chemotherapy is carried out. If both kidneys have to be removed, then the patient is prescribed dialysis, and then a transplant of he althy organs is required.
Children are all different, and each individual reaction of the body to tumor therapy. Before agreeing to a treatment plan, parents should discuss all issues with the doctor. They need to know what effect drugs have on the body, how to avoid or mitigate side effects during treatment.
Conducting clinical trials
Every year there are more and more new methods and ways of treating various diseases. This is especially true for cancerous tumors, as their mortality rate is high.
In many countries, not only adults, but also children can participate in clinical trials of the latest treatments for Wilms' swelling. Parents can ask the doctor if such tests are conducted in our country, if there is an opportunity to take part in them.
If there is a possibility, then the doctorshould give full information about the safety of treatment. It is worth noting that participation in such experimental trials often gives an additional chance in the fight against the disease.
But the doctor should also warn that in the process of treatment there may be unpredictable side effects that have not been seen before, and no one can guarantee a 100% cure.
Relapse
It is important not only to cope with the disease, but also to ensure that there is no relapse. Unfortunately, this happens with nephroblastoma both during and after therapy. Tests are taken, and if they are satisfactory, then backup chemotherapy regimens are prescribed:
- Vincristine.
- "Doxorubicin".
- Cyclophosphamide combined with Dactinomycin or Ifosfamide.
- Carboplatin.
Every case is different and some patients who relapse are given a high dose of chemotherapy followed by a blood stem cell transplant. The best effect will be if you combine surgery with radiation and polychemotherapy.
Wilms' tumor in a pregnant woman's history
The kidneys in the human body perform one of the main functions - cleansing the blood of 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 had an operation to remove a kidney long beforepregnancy, the prognosis can be quite favorable. Many women get through pregnancy safely and give birth to he althy babies.
But there are exceptions when a new condition can provoke a relapse of the disease. Then you have to terminate the pregnancy to save the woman's life.
Sometimes a Wilms tumor transferred in childhood can provoke the development of pyelonephritis, and it can also affect the functional development of the fetus. Even though most often the outcome of pregnancy is favorable, the risk of metastasis remains 10-20 years after the removal of the kidney.
Prognosis for a patient with nephroblastoma
To give a definite prognosis in the presence of a Wilms tumor, it is necessary to assess its size, stage of development and histological analysis. The most unfavorable is the presence of a tumor of more than 500 g and at stages 3-4 of development with poor histology.
If a comprehensive adequate therapy regimen is prescribed, then the survival rate of patients is about 60%. In the initial stages, the tumor lends itself to even more effective treatment, recovery occurs in 80-90% of people.
Given that there are no special measures to prevent such a disease, we can only recommend regular examinations if the child is at risk. The sooner the tumor is detected, the higher the chances of recovery.
