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Childhood Kidney Cancer ...
Scientists have identified a gene linked to the most common type of childhood kidney cancer in children. The scientists hope this might help determine which young patients are most at risk of dying.
In an article written for the journal ‘Science,’ the Massachusetts
General Hospital researchers said that about 30 per cent of cases of Wilms
tumor, a form of kidney cancer, involve mutations in a gene called WTX, locaded
on the sex-determining X chromosome.
About nine out of every ten cases of childhood kidney cancers are Wilms tumor.
This form of childhood kidney cancer occurs in roughly one in 10,000 children
worldwide. The normal treatment is surgery and chemotherapy, About 80 percent
of patients survive the treatment. The cancer usually appears by the age of five.
"The typical treatment for children with this form of childhood kidney cancer
is you remove the kidney that's affected. If they have tumors in both kidneys,
you take out one kidney and part of the other kidney and then you give chemotherapy," said
Dr. Daniel Haber, director of the Massachusetts General Hospital Cancer Center.
"Twenty percent of these children with this form of childhood kidney cancer
will then have a recurrence of their tumor and die of their disease. So, clearly,
if you could identify them up front, you would give more aggressive treatment," Haber,
senior author of the study, said in an interview.
Haber said he hoped scientists can now determine whether the WTX gene can
help predict the severity of a child's case of Wilms tumor to help guide
treatment. Haber added if doctors were able to determine a child had a less-threatening
case, they could tailor less-intensive treatment.
"There's always an interest in pediatric cancers to try to lessen the amount
of treatment if you could only identify markers of who has less-aggressive disease," Haber
said.
The kidneys filter the blood and rid the body of unneeded water, salt and
waste in the form of urine. Childhood kidney cancers originate in the early
stem cells -- those that will form the organ -- of the kidney's filtering
mechanism, the researchers said.
Scientists in 1990 identified mutations in another gene, called WT1, linked
to childhood kidney cancer, but it is implicated in only about 5 percent
of cases. Haber's team looked at tumor samples from 82 patients to try to
find further genetic abnormalities linked to the disease.
The WTX gene, they found, is in play in cells important to embryonic kidney
development, indicating it may have a significant role in the organ's formation.
The discovery also indicates that X chromosome genes may have a bigger role
in cancer than previously believed, they said.
The form of childhood kidney cancer is also is called nephroblastoma.
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