Wilms Tumour - This Battle Which I Must Fight: Cancer in Canada's Children and Teenagers

WILMS TUMOUR

Wilms tumour arises within the kidney before birth. It represents 93 percent of tumours of the kidney in children and teenagers and accounts for four percent of childhood cancers. Each year in Canada, approximately 50 children are diagnosed with this tumour - three-quarters of them before the age of five years. This cancer occurs about 20 percent more often in girls than boys (Table 1).

Wilms tumour is suspected when a lump is felt within the child's abdomen. A mass within the kidney is confirmed by an ultrasound or computed tomography (CT) scan of the abdomen. The extent of tumour spread is determined by X ray examinations and scans, and by careful inspection of the abdomen during surgery to remove the tumour. The definitive diagnosis of Wilms tumour is made by examining a specimen under the microscope. Wilms tumour can spread to adjacent tissues and lymph nodes within the abdomen, or via the bloodstream to the lungs and, occasionally, to the liver.

The rarity of this tumour makes study of causal factors difficult; however, there is evidence that genetic factors play a major and complex role in Wilms tumour. (21) For a few children, susceptibility to Wilms tumour is inherited. Twelve to 15 percent of children with Wilms tumour have specific congenital defects including aniridia (lack of the iris of both eyes), hemihypertrophy (overgrowth of one side of the body), and abnormalities of the genito-urinary tract.(22)

Prognosis depends on the extent of the disease at diagnosis and the type of Wilms tumour. Children with Wilms tumour in one kidney are treated by removal of the affected kidney and with chemotherapy. Children with high-risk features of Wilms tumour receive more intensive chemotherapy, as well as radiation therapy to the areas of the body where the tumour is or was located. The management of Wilms tumour involving both kidneys is complex and tailored to the characteristics of both the child and the disease. Every effort is made to preserve kidney function. The treatment includes surgery (often multiple operations), chemotherapy and occasionally radiation therapy.

Wilms tumour is a good example of a success story in childhood cancer. Thanks to the work of international Wilms tumour study groups, more than 90 percent of children with early stage favourable types of this tumour, and over 60 percent of children with advanced or high-risk types now experience long-term disease-free survival and may be considered cured.(28,29,131) In Canada, five-year survival for children with all types of kidney cancer diagnosed between 1985 and 1988 was over 90 percent (Table 7).

... I had one kidney out a year and a half ago. The surgery lasted a long time for everyone but me! I had radiation .. Radiation doesn't hurt and it feels like your in STAR WARS. I had 3 chemo drugs for about a year. They made me puke a lot. The first time Chemo was quite funny because it doesn't hurt. It wasn't funny after I got sick. The Vincristine made my bowels quit so for a while I was the ENEMA QUEEN! Cancer isn't that bad and you meet lots of people. So stay strong, be good and get better. Miranda, age 15

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