Gene linked to childhood kidney cancer identified

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kidney cancerScientists have identified a gene linked to the most common type of kidney cancer in children, and expressed hope this might help doctors determine which young patients are most at risk of dying.

Writing on Thursday in the journal Science, Massachusetts General Hospital researchers said about 30 percent of cases of the cancer called Wilms tumor involve mutations in a gene called WTX located on the sex-determining X chromosome.

About 90 percent of childhood kidney cancer cases are Wilms tumor. It occurs in roughly one in 10,000 children worldwide. It is treated with surgery and chemotherapy, with about 80 percent of patients surviving. It usually appears by age 5.

The disease also is called nephroblastoma.

"The typical treatment for these children 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 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 tumors 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 Wilms tumor, 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.

Copyright © 2007 Reuters Limited

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