What is Wilm's Tumour?

Wilms' tumour (or nephroblastoma) is a rare type of kidney cancer that primarily affects children. It is known to be the most common type of cancer of the kidneys in children. It usually occurs in children between infancy and 15 years, but mostly affects young children aged 3 to 4 years and the incidence of this tumour decreases after age 5. The disease is slightly more common among girls as compared with boys. In most cases, the tumour occurs in just one kidney, but it can involve both kidneys at the same time (in about 5 percent of children).

Some children with Wilms tumour have a family history of the disease, but it usually does not run in families. According to research, less than 2 percent of cases have an affected relative. Most cases of Wilms tumour occur in children with no affected relative, that is sporadically (by chance), as a result of genetic mutations that affect cell growth in the kidney. The mutations occur spontaneously after birth. Some children with Wilms tumour have one of the three genetic syndromes that predispose a person to Wilms tumour. The three genetic syndromes that are found in children with Wilms tumour include the following:

• WAGR syndrome - the acronym WAGR denotes the four diseases present in WAGR syndrome i.e.Wilms tumour, aniridia (absence of the iris i.e., the colored part of the eye), genitourinary malformations and mental retardation.
• Denys- Drash syndrome.
• Beckwith- Wiedemann syndrome.

Having these genetic syndromes or certain birth defects increases the risk of developing Wilms' tumour in a child. Children at a risk of Wilms tumour should be screened every three months until the age of eight.

Many children with Wilms tumour have no symptoms and appear healthy. Some common symptoms of Wilms tumour include lump in the abdomen and blood in the urine. If kidney tumour is suspected, your doctor will do a surgery to remove the lump. Other treatment options for Wilms tumour include chemotherapy, radiation, biologic therapy or a combination. The size of a tumour is variable and in some cases, it will be very large and may have spread (metastasize) to other body tissues at the time of diagnosis. Lung is the most common site for Wilms tumour to metastasize. Other parts of the body where it can metastasize include the liver, the other kidney, brain and/or bones.

Advances in diagnosis and treatment have improved the prognosis for children with Wilms tumour. Prognosis of most children with Wilms' tumour is very good and it is curable in most children. If a child is diagnosed with Wilms tumor, aggressive therapy is important for the best possible prognosis. Experts recommend long term follow-up care for a child diagnosed with Wilms tumor.