TUESDAY, March 13 (HealthDay News) -- A newly discovered genetic mutation is more common in teens and young adults than infants with a nerve tissue cancer called neuroblastoma.

The gene with the defect is called ATRX. While this defect was found in many teens and young adults with neuroblastoma, none of the infants with the disease who were tested had this genetic defect. This is important because babies are the ones who most commonly develop neuroblastoma. And, in babies, the disease tends to take a much less aggressive course.

"In infants, neuroblastoma is often treatable. In older patients, it tends to be more clinically aggressive," said study co-author Dr. Alberto Pappo, director of the solid tumor division at St. Jude Children's Research Hospital in Memphis, Tenn.

"About 90 percent of neuroblastomas happen in children less than 10 years old. When it happens in teens and young adults, they usually tend to have poorer clinical outcomes. They relapse over and over again. They can survive for many years with the disease, but they ultimately die of the disease," Pappo noted.

The discovery of the mutation in the ATRX gene is an "exciting, but preliminary finding. We still need to try to determine if this mutation is associated with any significant differences in survival," added Pappo.

Results of the study are published in the March 14 issue of the Journal of the American Medical Association.

Overall survival rates for neuroblastoma are 88 percent for babies under 18 months at the time of diagnosis, 49 percent in children between 18 months and 12 years and just 10 percent in teens and young adults who are diagnosed with the disease, according to background information in the study.

Because the disease takes such a different course depending on a patient's age, researchers have long suspected that there are likely different subsets of neuroblastoma, and that different genetic mutations may account for the differences in prognosis by age.

To see if there were any identifiable differences, the researchers conducted what's known as whole genome analysis on tumor samples from 40 infants, children, teens and young adults with advanced neuroblastoma. The researchers then looked to see if there were any similarities.

The investigators found that mutations in the ATRX gene were present in 100 percent of teens and young adults. Just 17 percent of children under age 12 had this same mutation, and none of the infants tested had it.

Go here to see the original:
Gene Discovery Gives Clues to a Childhood Cancer

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