Vilms's tumor – the high-malignant new growth striking a parenchyma of kidneys. Develops at children of early age. Quite often proceeds asymptomatically and it is found when carrying out the next survey. At germination in cup system there is a makrogematuriya, at germination in surrounding fabrics – a pain syndrome. Weakness, an indisposition, a hyperthermia, increase HELL, nausea, vomiting and violations of a chair can come to light. Vilms's tumor is diagnosed on the basis of data of a X-ray analysis, excretory urography, ultrasonography, KT and MPT. Treatment – surgeries, chemotherapy, radiation therapy.
Vilms's tumor – an embryonic new growth of high degree of a zlokachestvennost. Max Williams – the German surgeon who at the end of the 19th century for the first time described process of a histogenesis of this new growth consisting of epitelialny, stromalny and embryonic cages is called by name. Vilms's tumor is the most malignant new growth of renal fabric at children, makes 20-25% of total of oncological damages of kidneys at patients of early age.
It is usually diagnosed up to 5 years. Can be congenital. Girls and boys suffer equally often, average age of boys at the time of detection of a tumor of Vilms makes 3 years 5 months, girls – 3 years 11 months. In 4-5% of cases has bilateral character. Occurs at adults exclusively seldom, patients with Vilms's tumor make 0,9% of total number of patients with new growths of kidneys. Treatment is performed by experts in the field of oncology and nephrology.
Reasons of a tumor of Vilms
It is established, Vilms's tumor results from genetic mutations, however the reason of mutations in most cases remains to the unknown. At 1-2% of patients hereditary predisposition comes to light (there are cases in a family). The probability of development of a tumor of Vilms increases at some malformations, including – at an aniridiya (an underdevelopment or lack of an iris of the eye of an eye), a gemigipertrofiya (uneven development of different half of a body), a kriptorkhizm and a gipospadiya.
This pathology comes to light at some rare syndromes, for example, at Denis-Dreche's syndrome (includes Vilms's tumor, a men's pseudo-hermaphroditism and disorders of function of kidneys), Wiedemann-Bekwita's syndrome (includes increase in language and defects of internals), a WAGR syndrome (includes Vilms's tumor, intellectual backwardness, lack of an iris, malformations of a reproductive and urinary system) and Klippelya-Trenone's syndrome (includes large birthmarks and expansion of hypodermic veins of the lower extremities, a thickening and lengthening of extremities).
Classification of a tumor of Vilms
In clinical practice usually use classification by stages:
- 1 stage – a new growth within a kidney. Kidney vessels and the capsule of body are not involved. Metastasises are absent.
- 2 stage – Vilms's tumor go beyond a kidney, striking vessels and/or the capsule of body. Mobility of a new growth remains, radical operation is possible. Metastasises are absent.
- 3 stage – Vilms's tumor spread in lymph nodes or an abdominal cavity.
- 4 stage – come to light metastatic cancer of a liver, lungs, bones, back or a brain.
- 5 stage – a new growth have bilateral character.
There is also a classification created taking into account histologic features of a tumor of Vilms. According to this classification distinguish two categories of new growths – with favorable or adverse histologic signs.
Symptoms of a tumor of Vilms
Clinical manifestations are defined by age of the child, a process stage, existence or lack of the secondary centers in lymph nodes and the remote bodies. At initial stages of a tumor of Vilms the asymptomatic current or the erased symptomatology is noted: weakness, slackness, deterioration in appetite, weight reduction, slight increase of temperature. In the analysis of peripheral blood increase SOE and moderate anemia are defined. Rather large tumors of Vilms are probed through a forward belly wall. Asymmetry of a stomach is possible. At big new growths there can be phenomena of intestinal impassability. The breath difficulties caused by a sdavleniye of bodies of a thorax are observed.
At a palpation dense, usually smooth knot is defined painless mobile (on 1 and 2 stages) or motionless (at 3 and 4 stages). Less often Vilms's tumor has a hilly surface. Pains arise at a sdavleniye of nearby bodies, germination of a liver, a diaphragm and zabryushinny cellulose. Blood in urine appears at germination cup systems, it is found less than in 30% of patients with Vilms's tumor. Mikrogematuriya is observed more often than a makrogematuriya. At accession of an infection and a necrosis of a tumor in the analysis of urine the proteinuria and a leykotsituriya come to light. More than a half of patients with Vilms's tumor has an increase in arterial pressure. At emergence of the remote metastasises the clinical picture is supplemented with malfunction of the struck bodies.
Diagnosis of a tumor of Vilms
The diagnosis is exposed taking into account clinical symptomatology, these laboratory and tool researches. At the initial stage as the additional instruction on a possible tumor of Vilms consider the isolated anomalies of development and characteristic syndromes. Appoint the analysis of peripheral blood, the general analysis of urine, blood test on onkomarker and functional kidney tests. Carry out excretory urography for assessment of a state cup systems. Carry out the survey X-ray analysis of a thorax allowing to reveal the sredosteniye shift, increase in level of a diaphragm and the secondary centers in lungs.
Patients with Vilms's tumor are directed to ultrasonography of zabryushinny space, ultrasonography of an abdominal cavity, KT of zabryushinny space, KT of kidneys, MRT of kidneys and other researches. At suspicion on damage of a hollow vein apply a venografiya. For an exception of metastasises in a bone appoint a stsintigrafiya of bones of a skeleton and a sternalny puncture with the subsequent microscopic research of a punktat. For an exception of defeat of TsNS carry out KT and MPT of a brain and spinal cord. Vilms's tumor is differentiated with polikistozy, gidronefrozy, a kidney cyst, thrombosis of a renal vein and other tumors of a kidney.
Treatment and the forecast at Vilms's tumor
The treatment combined includes surgical intervention, chemotherapy and radiotheraphy. Depending on extent of defeat of body are possible a partial or total nefrektomiya. To patients with Vilms's tumor aged about one year operation is performed without the previous chemotherapy. To children 1 years are more senior appoint preoperative chemotherapy within 1-2 months. During surgery carry out audit of both kidneys. At bilateral tumors of Vilms carry out a bilateral partial nefrektomiya or a full nefrektomiya on the one hand and partial – with another.
At extensive damages of both kidneys carried out a full bilateral nefrektomiya earlier. Now preference is usually given to organ-preserving operations in combination with preoperative polychemotherapy, postoperative himio-and radiotheraphy. Excise paraaortal and bryzheechny lymph nodes. Conduct a histologic research of fabric of a tumor of Vilms and lymph nodes for specification of the diagnosis and more exact assessment of prevalence of process. At inoperable new growths appoint palliative treatment: chemotherapy, radiation therapy, symptomatic therapy.
Patients with Vilms's tumor after a full bilateral nefrektomiya are directed to a hemodialysis and put in waiting list for renal transplantation. Treatment of a recurrence combined, includes surgical interventions, radiotheraphy and chemotherapy. When performing antirecurrent therapy of a tumor of Vilms use more aggressive himiopreparata exerting negative impact on system of blood formation therefore in the subsequent transplantation of marrow can be required by patients.
After the termination of a cycle of treatment patients with Vilms's tumor within 2 years monthly undergo ultrasonography of abdominal organs and zabryushinny space, make blood tests and urine. Then inspection is carried out time in three months. The thorax X-ray analysis is twice carried out with a three-months interval, further carry out time to half a year. In the remote period at patients with Vilms's tumor development of the metastasises and malignant new growths provoked by radiation therapy is possible. At women the risk of antenatalny death of a fruit and the child's birth with a low weight increases.
The forecast at Vilms's tumor is defined by a stage and histologic features of a new growth. Five-year survival of patients with Vilms's tumor of the first stage makes 80-90%, the second – 70-80%, the third – 35-50%, the fourth – is less than 20 has some. A recurrence of primary new growth comes to light at 5-15% of patients. Two-year survival in the presence of a recurrence makes about 40%. Adverse predictive factors are certain histologic features of a tumor of Vilms, the age of the patient is younger than 1,5 years or 5 years, interruption of a course of radiation therapy or chemotherapy are more senior.