There is not a single organ in the human body that would always and under any circumstances remain healthy. Unfortunately, many diseases and defects are laid during intrauterine development. We will talk about one of them in our article. Wilms' tumor is unfamiliar to many. What is it, some do not even know. The article will try to deal with the symptoms, causes and tactics of treating this disease.
The concept of Wilms tumor
A Wilms tumor, or, as it is also called, a nephroblastoma, refers to malignant diseases of the kidneys, which appear most often in childhood. She is usually diagnosed at the age of 2-5 years. Wilms tumor in adults is quite rare, therefore, little studied.
Unfortunately, children who suffer from this disease often have other deviations in their development. It is believed that the formation of a tumor can begin in the prenatal period, since already in a two-week-old baby it can be detected.
The Wilms tumor, as a rule, is localized on one side, but there are cases of bilateral damage to the kidneys. If you look at the diseased organ, it can be noted that its surface is uneven, sections of hemorrhages and necrosis are visible in the section, cysts are present in some places. In this case, it is said that the patient has a Wilms cystic tumor.
At first, the tumor is localized only inside the kidneys. With subsequent development, it grows into neighboring tissues and organs - in this way metastases spread.
Stages of the development of the disease
This malignant tumor passes through several stages in its development:
- At the very beginning, nephroblastoma is located in only one kidney. Children during this period may not feel discomfort and have no complaints.
- The tumor begins to spread outside the kidneys, but metastases are still missing.
- Further development is accompanied by the germination of the capsule, and the lesion affects neighboring organs, lymph nodes.
- Found metastases in the liver, lungs, bones.
- At the last stage of its development, Wilms' tumor in children 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 there is a Wilms tumor, the symptoms are detected as follows:
- general malaise;
- weakness, and if the child is very small, then he constantly sleeps;
- decreased appetite;
- weight loss is observed;
- in some cases, a high temperature is noted;
- the skin becomes pale;
- blood pressure may increase;
- a blood test shows an increased ESR and a decrease in hemoglobin;
- blood appears in the urine;
- in severe cases, bowel obstruction can be observed, as the tumor compresses it.
With any disease, the behavior of the baby changes, and the 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 by hands. At the same time, the baby feels pain, since compression of neighboring organs occurs, which prevents them from functioning normally. In some patients, the abdomen enlarges if a 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 failure 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 age of the patient. It turns out that very young children with such a diagnosis are much more likely to recover completely.
But more often than not only Wilms tumor is diagnosed, it is usually accompanied by other developmental abnormalities.
Accompanying illnesses
It is rare enough that this tumor is diagnosed as the only developmental abnormality. Most often, it is accompanied by concomitant pathologies:
- hypospadias, when the urethra in boys opens somewhere in the middle of the penis or generally in the perineum;
- enlarged internal organs, for example, a large tongue;
- one limb is larger than the other;
- cryptorchidism;
- the absence of the iris in the eye (the sensation is that the pupil is not surrounded by anything).
Diagnosis of Wilms Tumor
When a nephroblastoma is only at the very beginning of its development, it is quite difficult to make a correct diagnosis, since no pronounced symptoms are observed. For this reason, in most cases, treatment is started already when the tumor is at stages 3 or 4.
Parents need to closely monitor the condition of the child, and if pallor, high blood pressure, decreased tone are observed, it is possible to feel the tumor, then a full examination should be immediately taken. It usually includes the following types of diagnostics:
- Blood and urine tests. 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 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.
- Using ultrasound tomography, it is also easy to see the size of the tumor and its location.
- The Wilms tumor on an ultrasound scan looks like a heterogeneous formation with cysts inside.
- Using 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 see the presence of metastases in neighboring organs.
- With large tumors, angiography is usually prescribed. This is done in order to determine the location of the nephroblastoma relative to large vessels, so that during the operation the surgeon had an idea of the situation.
- 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 can make the correct diagnosis in almost 95% of cases. But errors still occur in 5%, for example, if an ultrasound scan took a multilocular cyst as a Wilms tumor, then only during the operation it is possible to detect this. With ultrasound scanning, the accuracy is 97%, and with CT a little less. Although there are cases when a Wilms tumor is combined with a cyst.
Further examination is aimed at detecting metastases, so that then you can choose the right treatment tactics for the disease. If a Wilms tumor develops in children, the treatment is prescribed in each case individually, taking into account the characteristics of the body and the stage of development of the disease.
Causes of Nephroblastoma
It is impossible to say exactly what is the cause of the development of the tumor. It is believed that this is due to a mutation in the DNA of cells. In a small percentage of cases, it is transmitted from parents to children. But most often, researchers do not find a 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:
- Female. According to statistics, girls are more likely to get this disease than boys.
- Hereditary succession. Although cases of familial disease are quite 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 the Wilms tumor have not yet been established.
Nephroblastoma Treatment
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 according to the results of a histological examination. Experts believe that a good histology gives a more favorable prognosis. But this does not mean that poor tests serve as a signal to stop treatment.
If we talk about nephroblastoma, then the following methods are used for its treatment:
- surgical intervention;
- radiation therapy.
Chemotherapy for Wilms tumor also gives positive results.
Tactics of treatment is selected individually for each small patient. It will depend not only on the stage of tumor development, but also on the state of the child’s body.
Surgical intervention
Removing a kidney is a nephroectomy. It is of several types:
- Simple. In this case, the surgeon removes a completely affected kidney during surgery. If the second organ is healthy, then it may well work "for two."
- Partial nephroectomy. 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 is already removed or also diseased.
- Radical removal. Not only the kidney with 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, it can always be found out that the neighboring tissues are damaged, then you need to be guided by the situation and remove all infected. In severe cases, when a 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 prescribed hemodialysis to clear the blood of toxins. It is possible to make life after the removal of both kidneys complete only after an operation to transplant this organ from a donor, which is quite difficult 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, and the rate of reproduction. This is necessary in order to choose the most effective course of chemotherapy.
Chemotherapy in the treatment of nephroblastoma
If the tumor cannot be removed, then the treatment is reduced to other methods, for example, 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, 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 is disturbed;
- hair follicles are damaged, which causes baldness during chemotherapy;
- gastrointestinal cells suffer, which provokes loss of appetite, vomiting, nausea;
- immunity decreases, which makes the body more susceptible to various infections.
Parents should start taking chemotherapy drugs to ask the doctor about possible side effects and take the necessary measures to support the child’s body.
If a child is forced to prescribe a high dose of drugs, then 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 immediate responsibilities for the production of blood cells. So the bone marrow damaged during chemotherapy will recover.
Radiation therapy
If the Wilms tumor progresses strongly, the treatment necessarily also includes radiation therapy. It is combined with chemotherapy if the nephroblastoma is at the 3-4th stage of its development. Radiation therapy helps kill cancer cells that have “slipped away” from the surgeon during surgery.
During this procedure, it is important that the child lies still, because the rays are sent directly to the tumor area. If the patient is very small and it is difficult to achieve his immobility, then before the procedure he is given a small dose of sedatives.
Irradiation requires adherence to precise recommendations. The point at which the rays will be directed is marked with dye. Everything that should not fall 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 should prescribe medications that will alleviate the patient’s condition and relieve side effects.
Therapy depending on the stage of the disease
With any ailment, treatment is always selected individually. This is especially true for children. The Wilms tumor also requires careful analysis and the 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 characteristics of the body.
- At the first and second stages of tumor development, when the process affects only one kidney and the cancer cells are not aggressive, the treatment, as a rule, consists in removing the affected organ and subsequent chemotherapy. Radiation therapy may also be required.
- Stage 3 and 4 nephroblastomas require a different approach. The tumor goes beyond the kidneys, so it is already impossible to remove it without touching the neighboring organs. In this case, surgical removal of a part of the tumor is prescribed, followed by chemotherapy and radiation.
- Stage 5 is characterized by the appearance of pathology in both kidneys. Partial removal of the tumor is carried out, while neighboring lymph nodes are also affected. 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 transplantation of healthy organs is required.
Children are all different, and each has an individual 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.
Clinical trials
Every year there are more and more new methods and methods of treating certain diseases. This is especially true of cancerous tumors, since mortality from them is at a high level.
In many countries, not only adults, but also children can participate in clinical trials of the latest treatment methods for Wilms swelling. Parents can ask a doctor if such tests are carried out in our country, whether there is an opportunity to participate in them.
If there is a probability, then the doctor should give full information about the safety of treatment. It is worth noting that often participating in such experimental trials gives an additional chance in the fight against the disease.
But the doctor should also warn that during the treatment process unpredictable side effects may occur that have not previously occurred, 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 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 for some patients, in case of relapse, a high dose of chemotherapy is prescribed, after which blood stem cells are transplanted. The effect will be best if you combine surgery with radiation and polychemotherapy.
Wilms tumor in the history of a pregnant woman
The kidneys in the human body perform one of the main functions - cleansing the blood of toxins and metabolic products. During pregnancy, they have a double responsibility not only for the state 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 pregnancy, then 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 trigger a relapse of the disease. Then you have to interrupt the pregnancy in order 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 the outcome of pregnancy is most often 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 presence of a tumor of more than 500 g and at stages 3-4 of development with poor histology is considered the most unfavorable.
If a comprehensive adequate treatment 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 screening if the child is at risk. The sooner a tumor is detected, the higher the chances of recovery.