Treatment of Wilms tumour abroad
Wilms tumour (nephroblastoma) is a malignant tumour of the kidney found mainly in kids. This illness is named after the doctor who discovered it in 1899 for the first time. It is an embryonic tumour arising from issues of a developing kidney.
The disease is diagnosed before the age of 5 in 75% of cases, though it can be congenital as well. The other 25% of patients are children from 6 to 14 years old. Wilms tumour is rare among adults.
Causes of Wilms tumour
Wilms tumour is triggered by the dysfunction in the development of the kidneys during the prenatal period.
The key risk factors are:
- Parents age. 62% of patients had a mother older than the age of 30 at the time of birth. Father’s age was more than 30 in 50% of kids suffering from Wilms tumour. Hence, the older the parents the higher the risk of nephroblastoma.
- Occupational hazards increases the risk of tumour if a mother or father is intoxicated.
- Presence of kidney development abnormalities is observed in 25-35% of children suffering from Wilms tumour according to various studies.
Symptoms of Wilms tumour
The first sign of the disease in a child is usually a palpable or visually noticeable tumour in the abdominal cavity. It compresses the lymphatic and blood vessels when reaching a large size. As a result, edema is formed in the lower extremities and genital organs.
25% of patients have hematuria which is an a mixture of blood in the urine. This occurs when the tumour sprouts into the calyx-pelvis system.
Signs of intoxication appear:
- Gastrointestinal disorders
- Abdominal pain
Other possible signs of Wilms tumour are:
- Enlarged lymph nodes
- Enlargement of the liver
- The appearance of violet tubercles on the skin
- Laboured breathing
- Tendency to bleedings
Diagnostics of Wilms tumour
Visualization techniques are carried out to confirm the diagnosis:
- Computed tomography
These techniques help to not only detect a tumour in the kidney, but also to evaluate its size, prevalence of the oncologic process, detect metastases including distant ones in the liver, lungs and bones.
Other diagnostic techniques are:
- Determination of catecholamines level in urine used after tumour detection with the help of visualization methods. There is a need to distinguish Wilms tumour from neuroblastoma, which can arise from adrenal tissues.
- Excisional biopsy is carried out when a fragment of the removed tumour is sent for histological examination. The structure of the sample not only makes it possible to ascertain the diagnosis, but also to determine from which cells the nephroblastoma consists. Life prognosis and medical tactics depend on the result. Excisional biopsy is usually performed during a surgical intervention. Histological examination is performed intraoperatively.
Treatment of Wilms tumour abroad
Treatment scheme is defined by the stage of the pathological process and histological type of the tumour.
Stages of Wilms tumour are:
- Stage 1 – a tumour is restricted by a kidney or removed
- Stage 2 – a tumour crossed the kidney borders but was completely removed
- Stage 3 – tumour has large size and metastases are present in the lymph nodes; tumour has remains in the wound edges after its removal or tumour has ruptured during the operation
- Stage 4 – there are distant metastases
- Stage 5 – Wilms’ tumour is present in the both kidneys
Wilms tumour is removed at all the stages as this is a basis of treatment. They use radical or partial nephrectomy. Mostly, a whole kidney is removed.
Doctors also remove the following parts:
- Fat tissue
- Lymph nodes
- The adrenal gland
If a tumour was detected in both kidneys (5% of patients), then one of the following three treatment tactics is applied:
- Both kidneys fragments affected by a tumour are removed (if malformations are smaller in size).
- One kidney is completely removed (if a tumour is large in size) and the second one is partially removed.
- Both kidneys are removed and a patient receives dialysis several times a week. Then it is possible to transplant a donor kidney.
Chemotherapy is used as an additive at all stages. It is rarely used for the first stage, only if the risk of recurrence remains high. In European hospitals medication is prescribed before the intervention, and in the USA – afterwards. It remains unknown which way is more effective.
Radiation therapy is sometimes administered before the intervention, though only if a malformation exceeds the borders of a kidney. It eases the removal of nephroblastoma and lowers the risk of the tumour rupture during the intervention. Radiation of the whole abdominal cavity is done post-operatively if a tumour has ruptured.
Innovative treatment of Wilms tumour
While the favourable histological forms of the Wilms’ tumour are successfully cured by a combination of surgery and drugs, the adverse forms of this cancer continue to be poorly amenable to therapy. Another promising way of treatment is the use of Topotecan and Irinotecan medications. Trials aimed to access their effectiveness are ongoing.
Research is conducted for the possibility to transplant stem cells to a patient. Doctors presuppose that it will help the patients to undergo the more intensive chemotherapy aimed at the destruction of the histologically unfavourable types of Wilms’ tumour. At the moment, it is not known whether such a therapeutic approach will prove to be justified. Treatment technique has not yet been introduced into clinical practice.
Prognosis for Wilms tumour
Prognosis depends on the histological structure of the malformation. They differentiate between the favourable and unfavourable histological (tissue) structures of the Wilms’ tumour. Unfavourable is rarely experienced, which is about 12% of cases.
4-year-survival rate for patients in the developed countries is represented in the table depending on the histological type of the Wilms’ tumour.
The statistics of complete cure differs from state to state. In economically prosperous states, about 90% of children are cured. In developing countries this result can be achieved only in 70-80% of cases.