Wilms tumor( or nephroblastoma) is a malignant neoplasm that affects, as a rule, the tissues of one kidney. Cases of bilateral pathology are extremely rare( from 5 to 10%).
As the most common type of cancers of the children's genito-urinary tract, nephroblastomas often occur in toddlers of three to four years of age. After five years the probability of occurrence of this pathology in children is sharply reduced.
According to the American medical statistics, Wilms' tumor is diagnosed annually in eight white children from one million of their peers at the age of fifteen. The frequency of nephroblast development in children of the Negroid race is twice as high.
What is Wilms' tumor?
Wilms tumor, named after the German doctor Max Wilms, who managed to substantiate its histogenesis in his monograph written at the end of the XIX century, has many synonymous names:
- embryonic nephroma;
- kidney adenosarcoma;
- embryonic nephroma;
- tumor of Birch-Hirschfeld.
All of these terms refer to the same disease and are widely used in the medical literature.
The photo shows Wilms tumor
For quite some time, Wilms' tumor develops inside a tight capsule, not germinating through its shell and not giving metastases. Expanding to a solid size, it begins to squeeze and dislodge the surrounding tissue.
In the last stages of development, the tumor grows through the membrane and, using the lymphogenous and hematogenous pathway, metastasizes into internal organs( up to the bottom of the vena cava and right atrium).
Wilms tumor is most often detected in children of two or three years of age, although there are single cases of lesions of newborns and adult patients.
- The true causes of nephroblast development are still unknown. It has been established only that the impetus to the beginning of the tumor process gives all possible disturbances of renal embryogenesis in the process of intrauterine development of the fetus.
- Cases of familial nephroblast, transmitted by hereditary path from parents to offspring, are extremely rare( their share is not more than 1.5%), so there can be no question of the hereditary nature of this pathology.
- One of the risk factors is belonging to the female sex: Wilms' tumor is more often observed in girls.
- Statistical data allow us to conclude that there is a racial predisposition to the development of nephroblastoma in representatives of the Negroid race. Black children suffer this type of cancer twice as often as children with white skin.
Wilms tumor is quite an insidious disease, because at the initial stage of its development it proceeds asymptomatically, without causing the child any pain. Even with a fairly large neoplasm, he continues to look visually healthy.
Parents should be alarmed to find out from their baby:
- Pale skin.
- Even a small amount of blood in the urine.
- Tendency to constipation( the presence of this symptom can be explained by the squeezing of the intestines by the proliferating tissues of the tumor).
- Frequent nausea.
- Tendency to a slight increase in blood pressure.
- Febrile state.
- Complaints of abdominal pain. A long absence of appetite.
- Weight loss.
Without being specific, these symptoms may not necessarily indicate nephroblastoma, but it is better to insure and show the baby to a qualified specialist as soon as possible.
In a disease that has reached a late stage, parents can find in the tummy of their baby a dense, painless lump with a smooth or uneven surface.
Pain syndrome in nephroblastoma is a very rare phenomenon caused by the spread of a cancerous tumor to the nearest organs. The painfulness of the process is explained by the squeezing of sensitive nerve endings and internal organs( diaphragm, liver, retroperitoneal tissue), as well as a significant stretching of the fibrous renal capsule.
Nephroblastoma in children
Being a typically childish tumor due to intrauterine malformation of the embryo, nephroblastoma is most often found in small patients between the ages of two and four.
Wilms tumor is often combined with numerous congenital malformations of the child, for example:
- by cryptorchidism - undescended testicles in the scrotum of boys;
- anisidium - partial formation or total absence of the iris of the eyes;
- hemihydrotrophy - a fairly rare anomaly, in which there is an uneven development of different halves of the body;
- hypospadias - a pathology characterized by an atypical location of the urethral opening in boys.
Nephroblastoma in adults
Despite the fact that Wilms' tumor develops mainly in young children, in medical practice there are single cases of this disease in patients whose age is within the range of six to sixty-five years.
Given the extreme rarity of this pathology in adult patients, it is necessary to note the lack of systematic information about the features of its clinical course, treatment and prognosis in this category of patients.
According to most sources, Wilms' tumor in adult patients:
- is characterized by extremely rapid growth;
- leads to the formation of tumor thrombi, significantly narrowing or completely overlapping the lumen of the inferior vena cava and renal veins;
- differs early in the dissemination( the spread of the pathological process from a closed foci throughout the body).
The clinical course of the Wilms tumor in adult patients includes a characteristic triad of symptoms observed in renal diseases:
- pain syndrome;
- presence of a tumor that is palpable;
- pronounced hematuria( the presence of blood in the urine).
No specific complaints, characteristic exclusively for nephroblastoma, were observed in adult patients.
Given the lack of systematic observation of adult patients with nephroblastoma, clearly developed recommendations for their management at the moment simply does not exist.
Most experts are of the opinion that the best tactic for treating Wilms tumor in adult patients is a combination of surgical methods, radio and chemotherapy, but none of these methods has been developed in detail:
- did not reveal the optimal amount of surgical intervention;
- does not specify the exact dosage of drugs used in chemotherapy;
- has not developed the optimal mode of radiation exposure.
Stages of tumor
In the development of nephroblastoma, specialists distinguish five stages:
- Disease of the first stage is localized in the tissues of only one kidney. Not delivering the baby the slightest suffering, it is easily eliminated by surgery. In the leading US clinics, almost 96% of patients with the first stage of the disease survive.
- Wilms tumor, reaching the second stage, begins to germinate into the tissues surrounding the cancer-affected kidney. And at this stage with nephroblastoma you can cope exclusively surgically. Survival of such patients exceeds 91%.
- The third stage nephroblastoma leaves the affected kidney and metastasizes to the nearest lymph nodes and organs located in the abdominal cavity. Completely eliminate it by surgery is no longer possible. The prognosis of survival( under condition of application of methods of modern therapy) is reduced to 90%.
- The fourth stage of the Wilms tumor is considered to be neglected. On the lymphatic ducts cancer cells enter the most remote parts of the body: brain tissue, bones and lungs. However, at this stage, the survival prognosis is about 80%.Of course, we are talking about the treatment conducted by leading specialists in the conditions of modern, well-equipped clinics.
- The fifth stage neuroblastoma affects the tissues of both kidneys of the patient.
When suspected of Wilms tumor, a number of instrumental diagnostic tests are prescribed:
- Radiography of the abdominal organs, revealing the presence or absence of metastases.
- Radioisotope renal examination( renoscintigraphy), which allows to evaluate both the total and individual kidney function.
- Ultrasonic tomography, which allows to determine the size and location of malignant neoplasm.
- Intravenous( excretory) urography. This method allows you to clearly see the contours of the affected kidney, identify abnormalities in the functioning and in 70% of cases confirm the preliminary diagnosis.
- Magnetic resonance imaging with intravenous contrast.
- Renal arteriography, which reveals the presence of tumor thrombi in the lumens of blood vessels.
- Angiography showing the location of the tumor and large blood vessels. This information is necessary for a specialist who is going to operate on the patient.
- Chest X-ray( to exclude the presence of metastases in the lungs) in five projections.
- Computer tomography, which helps to determine the degree of metastasis of nephroblastoma in various tissues and organs.
Laboratory studies are limited to the following:
- Clinical blood and urine tests. In patients with nephroblastoma in the urine, protein and blood are detected. A blood test reveals an elevated level of ESR and the presence of anemia.
- Biochemical blood test.
- Bone puncture.
Methods of therapy for patients with
Treatment of Wilms tumor must be combined, combining surgery, radio and chemotherapy.
- The leading therapeutic method is surgical removal of the tumor. The volume of the operation depends on its size and the degree of metastasis. In modern clinics, operations to remove nephroblastoma are performed by minimally invasive( gentle) methods. Laparoscopic removal is performed through micro cuts not exceeding 1.5 cm. Radiofrequency ablation is performed using a catheter. Laser valorisation is performed using a laser. Good results are given by cryoablation, a procedure for freezing tumor tissues.
Infants up to a year with a confirmed diagnosis of nephroblastoma operate almost immediately, older children - after a course of chemotherapy( its duration is from 4 to 8 weeks).
- Chemotherapy is performed before and after surgery. In the latter case, chemotherapy is prescribed no later than five days after surgery. Treatment is carried out with vincristine, cyclophosphamide, dactinomycin, doxorubicin.
- In the treatment of nephroblastoma, which reached the 3-4 stages, the remote radiation therapy is used, which consists in irradiation of the primary tumor site. Radiation is also metastatic foci located in the affected internal organs( liver and lungs).
Schemes of treatment depending on the stage of the disease
Treatment of any disease should always be individual.
Necroblastoma requires special care in the selection of adequate therapeutic measures.
When choosing the tactics of treatment, it is necessary to take into account the age of the patient, the individual characteristics of his body and condition, as well as the stage of the disease.
- The first-stage and second-stage nephroblastoma, localized in the tissues of only one kidney and not characterized by the aggressiveness of cancer cells, can be cured by surgical removal of the diseased organ followed by chemotherapy. In some cases, radiotherapy is used.
- Wilms tumor, reaching the third or fourth stage and leaving the border of the affected kidney, requires a fundamentally different treatment, because when it is removed, adjacent organs are at risk of damage. The surgical intervention at this stage consists in the removal of part of the tumor tissue. After the operation, radiation and chemical therapy are followed.
- The last stage nephroblastoma leads to the defeat of both kidneys. The tactics of surgical intervention at this stage may be different. In some cases, part of the tumor and several affected lymph nodes are removed. After that, radio and chemotherapy is used. In other cases, both diseased kidneys are removed and dialysis sessions are administered to the patient. After this, the patient needs an urgent transplantation of a healthy organ.
Features of nutrition after treatment
Patients( even those who have successfully completed treatment) with Wilms' tumor are forced to adhere to the diet No. 7 developed by the well-known nutritionist Pevzner for life. Its main purpose is to reduce the diuretic load on a healthy kidney.
The maximum energy value of a daily table should not exceed 2500 calories. The permissible amount of liquid is not more than a liter.
Wilms tumor can lead to the development of:
- Arterial hypertension , is inextricably linked to the increase in arterial pressure that arises from the narrowing of the lumen of the blood vessels.
- Kidney bleeding , dangerous large blood loss, a sharp drop in blood pressure, the development of collapse.
- Regional and distant metastases.
- Kidney stone disease.
Special preventive measures to prevent the tumor Wilms at the moment there. The only way to prevent the disease from starting is a regular ultrasound examination of the child's body that is in the risk group, since with a timely begun treatment, the prognosis is usually favorable.
Forecast for patient
In recent years, marked by great success in the diagnosis and treatment of nephrollast, there has been a significant improvement in the prognosis for patients suffering from this disease.
According to US medical statistics, the survival rate of patients in leading country clinics in which the Wilms tumor was detected at the first stage level exceeded 95%, and at the fourth stage 80%.
The realities of Russian clinics are much more modest. The survival rate of small patients is determined by the stage of the disease and its histological form.
- In the first stage of nephroblastoma, up to 90% of patients survive.
- At the second - up to 80%.
- At the third - up to 50%.
- The launched Wilms tumor forms leave a chance for only 20% of patients.
Long-term survival of patients with bilateral synchronous nephroblastomas is up to 80%, with metachronous up to 50%.
The most unfavorable histological forms of the nephroblast are sarcomatous and anaplastic.
If you identify a disease in two-year-old children, their chances of a full recovery are much higher than in older children.
On the first symptoms of the tumor, Wilms will tell the following videos: