Treatment of nephroblastoma abroad
Nephroblastoma (Wilms’ tumor) is a kidney cancer in children. A tumor occurs in embryonic tissue and is dangerous for a child’s life.Although it responds well to treatment, especially on condition of early diagnosis.
Nine out of ten kids suffering from nephroblastoma can be cured with the help of surgery, radiation therapy and chemotherapy in the developed countries.
Causes of nephroblastoma
At present, none of the environmental factors result in increase or decrease of the risk of nephroblastoma by effecting pregnant woman or a baby after the birth.
These are known risk factors:
- Age. 75% of patients are kids before 5 years old. Average patients’ age is 3-4 years old. Older kids rarely develop nephroblastoma, adults almost never.
- Race. Africans get sick most often, followed by Caucasians. Asians get this tumor the least often.
- Sex is important, as boys are diagnosed with nephroblastoma more often than girls.
- Genetic predisposition factor shows that approximately 1-2% of patients have relatives suffering from this tumor or those who had it in their medical history.Hereditary form increases the risk of tumor in both kidneys.
- Congenital syndromes like certain genetic and chromosomal abnormalities are associated with nephroblastoma.
- Congenital disease not associated with mutations such as: hypospadias, cryptorchidism, hemyhypertrophy, aniridia significantly increase the risk of kidney cancer.
Symptoms of nephroblastoma
As a rule, disease doesn’t manifest itself at the early stages even when a tumor reaches considerably large size. A child doesn’t cry and doesn’t complain, keeps on leading active lifestyle.
Thus, the first sign is a tumor in the abdominal area palpable by parents or a pediatrician. A tumor becomes visible while growing further, especially if a kid has a thin abdominal wall.
Possible clinical manifestations of nephroblastoma are:
- Decreased appetite
- Blood in the urine
- Inconsistent breathing
Some kids are observed to have increased blood pressure (in case renal artery lumen decreases as a result of its compression or infiltration by the tumor). This can lead to headache, dizziness, bleeding in the eyeball.
Diagnostics of nephroblastoma
The fact of nephroblastoma presence is determined by only instrumental diagnostics with the help of such techniques as:
- Computed tomography
- Chest X-ray (to search for metastases in the lungs)
In majority cases malformation detected in child’s kidney is nephroblastoma. All other cancer types are rarely diagnosed at this age.
Visualization techniques help also to:
- Determine the degree of nephroblastoma spreading
- Plan surgery and radiation therapy treatment
- Assess the effectiveness of the treatment provided
Sometimes, there is a need in differential diagnostics of nephroblastoma and neuroblastoma. Neuroblastoma is another embryonic tumor occurring in the adrenal gland tissues. Kidneys and adrenal glands are located close to each other and often it is hard to understand where exactly has the tumor started.
Levels of catecholamines and their metabolitesin the urine are also identified as a part of differential diagnostics. Increased level of dopamine, vanillylmindal, and homovanilic acid indicates neuroblastoma.
Nonetheless, even without this symptom you cannot say exactly if it is nephroblastoma. 10-15% of neuroblastoma cases are not characterized by the increase of these elements level in the blood. Therefore, a mistake is possible in certain cases. The exact origin of a tumor is identified after the histological test of the tissue sample.
Biopsy is not done separately as a rule. Tissue sample for histological test is taken during the surgery for the tumor removal. Modern German hospitals provide chance to do the test intraoperatively. This way, a doctor is able toadjust the surgery, if necessary.
Type of nephroblastoma is defined based on the histological test. If there are signs of anaplasia (different cell size, large cell nuclei) the tumor is considered unfavorable. If there is no anaplasia - the prognosis is better in this case as these are favorable tumors diagnosed in about 90% of cases.
Treatment of nephroblastoma abroad
Surgery is the treatment basis. Surgeons remove a kidney, ureter, lymph nodes and adipose tissue surrounding the organ.
It is more difficult to treat patients having bilateral nephroblastoma with a surgery. In this case, one of three strategies is used:
- Resection (partial removal) of the left and right kidneys if a tumor is small
- Removal of one kidney fragment, complete removal of another kidney (the organ where the nephroblastoma is large is completely removed)
- Both kidneys removal
When removing two kidneys, patient needs a new organ transplant. Therefore, such an extreme measure goes only if it is really necessaryand large tumors are present in both kidneys. A patient will have to undergo regular hemodialysis (blood purification with the help of “artificial kidney”) after removal of both kidneys while waiting for a new organ.
Chemotherapy is indicated before or after the intervention and is used almost always except for the cases when there are several favorable factors as:
- Nephroblastoma has small dimensions (up to 500 g) and does not go beyond the kidney
- Patient's age is up to 2 years old
Radiation therapy is done before the operation but only if a tumor exceeds kidney borders. Then, radiation helps to reduce the risk of tumor rupture during the surgery. If tumor has still ruptured, they must radiate the entire abdominal cavity.
Negative histological types of nephroblastoma are treated with the help of all three techniques available (surgery, chemotherapy, radiation therapy) regardless of child’s age and tumor size.
Innovative treatments of nephroblastoma
The main research being conducted in the field of nephroblastoma treatment is aimed at finding effective drugs for the patients’treatment with histologically unfavorable types of tumor. There are already medicines showing encouraging results. TheseareIrinotecanandTopotecan.
The possibility of using chemotherapy shock doses in children with unfavorable histological type nephroblastomaunresponsive to standard treatment is also being explored.
Intensive therapy damages the bone marrow, since it affects primarily the cells that quickly divide. Therefore, such patients need stem cells transplantation in the future.
Prognosis for nephroblastoma
Life prognosis is determined by the stage of the pathological process and histological type of tumor. 5-year-survival statistics of patients having favorable type of tumor depending on the stage is:
- Stage 1 – 99%
- Stage 2 – 98%
- Stage 3 – 94%
- Stage 4 – 86%
- Stage 5 – 87%
Prognosis is worse for unfavorable tumor types as this cancer poorly responds to chemotherapy and radiation therapy, grows quicker and spreads metastases earlier. 5-year-survival statistics is:
- Stage 1 – 83%
- Stage 2 – 81%
- Stage 3 – 72%
- Stage 4 – 38%
- Stage 5 – 55%
Nephroblastoma is among the number of those few malignant tumors which are cured in the majority of cases. Rate of a complete cure proved by the 5-year period without recurrence differs from country to country. Developing countries show 70% to 80% of cure. Developed countries, including Germany and Switzerland, ensure that 90% of their patients will be cured.