Gene May Help Trigger Children's Kidney Cancer

Discovery of Wilms' tumor mutation has implications for cancer research generally, experts add.

By Amanda Gardner
HealthDay Reporter
Thursday, January 4, 2007; 12:00 AM

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THURSDAY, Jan. 4 (HealthDay News) -- Scientists have discovered that mutated copies of a particular gene are present in about 30 percent of children with Wilms' tumor, the most common type of pediatric kidney cancer.

The finding may one day help physicians decide which patients need more therapy, or less.

"There's always an interest in terms of clinical markers -- how can you predict the 20 percent who are not going to be cured, and those who don't need chemotherapy or more intensive treatment," said senior study author Dr. Daniel A. Haber, director of the Cancer Center at Massachusetts General Hospital in Boston. "We're hoping our gene has some relationship to these issues."

"I hope it's just another key to unlock the secrets to this tumor so that treatment and prognosis continue to improve," added Dr. Monford Custer III, an associate professor of surgery with the Texas A&M Health Science Center College of Medicine. "I could foresee a future where they might utilize this to help understand or predict the tumor's biological behavior, which would be just another resource to guide treatment protocols."

The discovery also marks the first time a tumor-suppressor gene has been found on the sex-determining X chromosome, meaning that only one mutated copy of the gene, not two, would be necessary for cancer to develop. Males carry only one copy of the X chromosome, while females carry two.

"People have speculated about whether there could be tumor-suppressor genes on the X chromosomes and there have been some little hints," said Haber. "This is the first time to show that a gene inactivating a single copy inactivates the entire gene."

The finding may trigger a rush to try to find other cancer-related genes on that chromosome, he added.

"For this reason, nobody has looked at the X chromosome very carefully," Haber said. "This would be a good reason to start looking at the X chromosome."

About one of 10,000 children develop Wilms' tumor, also known as nephroblastoma. Unlike adult kidney cancer, pediatric kidney cancer is relatively easy to treat. About 80 percent of cases are curable with surgery and chemotherapy, Haber said.