nephroblastoma - a pathological tumor in the kidney, which is characterized by the manifestation of dizontogeneticheskie meet, usually in children.It is also called embryonal carcinosarcoma, Wilms tumor and fetal nephroma.Typically, this tumor affects only one kidney, but sometimes there are bilateral nephroblastoma.
In some cases, malignant pathology can be localized in the pelvis, groin, ovaries, uterus, retroperitoneal fat.For nephroblastoma characterized by lesions of children from 2 years to 3, but there are also descriptions of cases among adults and infants.
nephroblastoma, on gross examination, resembles large node, for which there is clear demarcation of the renal parenchyma.This tumor can spread through the lymphatic and hematogenous, metastasizing while in the different organs, even germinating in the right atrium and the lower part of the vena cava.
were recently made some progress in addressing the causes of nephroblastoma.Primarily, it
genetic role in the formation of cancer as the cause of its development, supported by a combination of frequent anomalies of other systems and organs.
nephroblastoma for a long time can develop completely asymptomatic.However, found in the peritoneum painless, smooth, dense and has an irregular surface tumor, refers to the first signs of nephroblastoma.
often for months or even years are not detected symptoms characteristic of malignant abnormalities in the body.However, pathological tumor continues to grow slowly, and the progression is gaining so rapidly, resulting in a significant increase in nephroblastoma.At the same time during palpation can detect the tumor in the peritoneum.
subjective condition of the patient can be regarded as satisfactory.When small volumes, nephroblastoma causes uncomfortable sensations, and since they increase visually observed asymmetry of the abdomen and palpation of the tumor itself.
expressed symptoms of intoxication detected only in advanced and severe cases.In 25% of patients diagnosed with gross hematuria, which causes tumor subcapsular rupture, and an increase in pressure associated with giperreninemiey.
Since nephroblastoma throughout the body through the blood and lymph nodal involvement is happening at the gate of the kidneys, liver and para-aortic nodes.Sometimes a clot to form tumors falls in the lower part of the vena cava.
also be observed disorders of the gastrointestinal tract, fever, abdominal pain and malaise with high blood pressure.
to diagnose nephroblastomas pay special attention to the patient's medical history.The important point considered borne infections, hereditary factors associated with malignant disorders, and the presence of congenital anomalies.And to exclude the diagnosis of neuroblastoma differentiation, splenomegaly, hepatomegaly, and polycystic hydronephrosis.
When nephroblastoma in 15% of cases are detected with radiography existing calcifications.Appointment ultrasound imaging helps identify abnormalities in 10% of cases at a time when the kidney does not appear on pielogramme.CT defines the boundaries of tumor formation in the kidney, and around it, identifies the affected lymph nodes, liver, and makes it possible to trace the state of the other kidney.
Patients with nephroblastoma observed anemia as a result of hematuria.But to prevent this pathology administered urine for the presence of catecholamines.
Rare Clinical signs include high blood pressure, which is normal after removal of the kidney.
nephroblastoma sometimes occurs in parallel with glomerulosclerosis syndrome and syndrome-Drash.
nephroblastoma in children
This disease is a form of kidney cancer pathologies encountered, usually in children.
nephroblastoma is considered part of childhood cancer.According to statistics this disease affects an average of eight children of a million under the age of fifteen years.A frequency of cancer among children with dark skin occurs twice.
Mostly nephroblastoma in children of two years and three years of age.All the more so in girls it occurs several months later than in boys.Nephroblastoma is mainly localized in one kidney, but sometimes can affect and two at once.
exact cause of nephroblastoma in children is not fully understood.It is known that the tumor is caused by mutations in cellular DNA structure.Only 1.5% of these mutations are inherited by children from their parents.But as a rule, a certain connection between the development of nephroblastoma and heredity does not exist.
But risk factors include girls who are more often affected, in contrast to the boys;typical family heredity and racial predisposition.
Often nephroblastoma in children is diagnosed in the presence of some congenital anomalies.For example, if aniridia when there is hypoplasia of the iris, or the absolute lack thereof;when hemihypertrophy when there is a significant development of one over the other half of the body;when cryptorchidism;hypospadias.Also nephroblastoma children may be one component of the syndromes such as:-WAGR syndrome, Drash syndrome, Beckwith-Wiedemann.
Symptoms nephroblastoma in children in the early stages may not manifest itself.Little patients at first glance looks perfectly healthy, although it sometimes increases stomach palpable tumor in the peritoneum, pain and some discomfort in the abdomen, is observed hematuria and fever.
When diagnosing a child nephroblastomas determine the stage of the disease.To do this, carry out the instrumental methods of examination.These include chest X-ray, CT and MRI, radioisotope bone scan to help determine the metastasis of cancer.
are five stages flow nephroblastoma.When the first or early stage of tumor lesion is noted only one kidney.In this case, a malignant pathology is easily removed by surgery.When the second stage is determined nephroblastomas spread to nearby tissue, which is also removed during surgery.For the third step, characteristic infiltration of renal disease beyond the space in the lymph nodes located near and in some organs of the abdominal cavity.In this case, a surgical operation is insufficient.
penultimate stage, the fourth, is characterized by the proliferation of nephroblastoma in distant tissues and organs.Fifth stage of the disease celebrate the defeat of both kidneys.
Today recognized as the overall complex therapy for children with this disorder, which involves surgically removing the malignancy, radiation therapy and intensive treatment polihimiopreparatami.
Preoperative therapy today is regarded as debatable question.Children's oncology in the United States considers this inappropriate therapy, until a morphological confirmation of the diagnosis and staging of disease after surgery.Although there is evidence that preoperative irradiation, which greatly facilitates the implementation of radical surgery and significantly reduces the incidence of fractures nephroblastoma.And this, in turn, eliminates the radiation exposure of the entire area of the abdominal space.The same positive effect was achieved, and after chemotherapy before surgery.
Proper and correct strategy in the treatment of children, as a rule, depend on the morphology of the tumor and the stage of nephroblastoma.For tumors with the histological structure of a positive nature lends itself well to appoint beam radiation and chemotherapy treatments.But modern schemes with a combined treatment of such tumors, preference is given chemotherapy.And to treat half of the children who have struck only one kidney tumor, does not prescribe radiation exposure.
When unfavorable histological structure nephroblastomas prescribed both types of treatment, as it is resistant to this therapy.Therefore, for these tumors use aggressive multimodal therapy.
to the standard types of treatment nephroblastomas include surgery to remove the tumor and chemotherapy.Disease stage and histological results will decide the appointment of additional treatment in the form of radiation exposure.
nephrectomy, or the removal of kidney tissue surgically divided into three types of operations.During the simple nephrectomy surgically remove the entire kidney completely, and the remaining kidney, partly enhances its function, filling remote operation of the kidney.
When performing partial nephrectomy removed only nephroblastoma and renal tissue around the kidney.Such an operation is carried out in case of damage or removal of the second kidney.During radical nephrectomy stunned by the kidney is removed, the surrounding tissue, adrenal gland, ureter, and lymph nodes detected with cancer cells.
At bilateral nephroblastoma spend removing both kidneys, then the patient is prescribed dialysis, which involves mechanical cleaning blood from the various toxins.In this situation, the patient is waiting for a donor kidney.
after surgery with the help of laboratory research studying tumor samples for the presence of malignant cells and their sensitivity to chemotherapy.
Today, the most effective chemotherapeutic agents considered Doxorubicin, Vincristine and Dactinomycin.However, this therapy can cause side effects such as bone marrow suppression, nausea, vomiting, sensitivity to infections, loss of appetite, and others. But all these problems go after discontinuation of chemotherapy.
When the third and fourth stage nephroblastomas recommended after surgery appointment ray irradiation and chemotherapy.The use of radiation therapy in addition destroys the tumor cells, which are not removed during surgery.
nephroblastoma refers to the well-cures cancer.About 90% of patients with the disease are cured with the use of modern methods of complex therapy.
forecast nephroblastomas closely associated with the stages of malignant disease.Treatment of recurrence of the disease is characterized by reduced efficiency.Only 40% is possible to achieve a positive result.
Five-year survival rates at the first stage of nephroblastoma is more than 95%, while the fourth - about 80%.
If nephroblastoma diagnosed in children under two years, the chances of a favorable prognosis is much more than if it occurs at a later age.