Wilm’s tumour or Nephroblastoma is a cancer of the childhood which occurs in the kidneys present in the abdomen. It is the most common abdominal tumour in children and accounts for about 5-6% of all childhood cancers. Majority of these cancers occur in children below the age of 5.
Risk factors associated with Wilm’s tumour include congenital syndromes such as WAGR syndrome, Beckwith-Wiedemann syndrome, Denys-Drash syndrome, Sotos syndrome and Perlman syndrome.
Symptoms associated with Wilm’s tumour include and abdominal swelling or a mass, pain in the abdomen, fever and high blood pressure. Those with spread of cancer to other areas will have symptoms such as cough or breathlessness if disease is in the lungs, reduced appetite and weight.
Investigations commonly done to diagnose Wilm’s tumour include and ultrasound of the abdomen, which is the first test which shows a mass in the abdomen arising from the kidney. This is followed up by a CT scan or an MRI scan of the abdomen. A CT scan is also done of the rest of the body to look for spread of cancer to other organs. A biopsy may or may not be done before treatment depending on the results of the scans. Genetic studies done on the biopsy specimen can add value in determining the best treatment options and also advise about prognosis.
Staging of Wilm’s tumour is done by different systems and these are NWTS system, SIOP staging system. Wilm’s tumour is also classified into low, intermediate and hisk risk groups and treatment strategies vary based on that.These staging systems are used after surgery is done and a simplified system is given below.
|Stage 1||The tumour is confined to the kidney|
|Stage 2||The tumour extends to organs or tissues adjacent to the kidney but is completely removed at surgery|
|Stage 3||The tumour involves lymph nodes in the abdomen or has ruptured or has been incompletely removed at operation.|
|Stage 4||There is metastatic disease|
|Stage 5||There is disease in both kidneys|
Treatment of Wilm’s tumour involves a combination of approaches using surgery, chemotherapy and radiotherapy. Majority of patients with Wilm’s tumour are curable. Most patients will have treatment with surgery and chemotherapy. All patients whose tumours are operable have surgery to remove the cancer. This is followed by chemotherapy. Chemotherapy drugs used are Vincristine, Doxorubicin and Dactinomycin. Some centres may offer chemotherapy first and then surgery. Radiotherapy is used for patients with intermediate and high-risk tumours and can last for 4-5 weeks.