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.
Wilms tumour is triggered by the dysfunction in the development of the kidneys during the prenatal period.
The key risk factors are:
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:
Other possible signs of Wilms tumour are:
Visualization techniques are carried out to confirm the diagnosis:
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:
Treatment scheme is defined by the stage of the pathological process and histological type of the tumour.
Stages of Wilms tumour are:
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:
If a tumour was detected in both kidneys (5% of patients), then one of the following three treatment tactics is applied:
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.
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 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.
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