Age Makes the Difference

Neuroblastoma ATRX mutation more common in older children

/ Author:  / Reviewed by: Joseph V. Madia, MD

(RxWiki News) By the time neuroblastoma - a common childhood brain cancer - is discovered, it has usually already spread. The younger the child is at the time the disease is found, the better. Scientists are beginning to understand why.

A mutation in the gene ATRX is usually found in older children and adolescents with more advanced forms of neuroblastoma, a cancer that grows in the nervous system. When ATRX is present, the outlook isn't so good for these kids.

"Ask about genetic testing in children with cancer."

Nai-Kong V. Cheung, M.D., Ph.D., of the Memorial Sloan-Kettering Cancer Center, and colleagues designed and conducted a study to find genetic rearrangements that are linked to the age of patients at the time of diagnosis with metastatic neuroblastoma.

This disease has spread in about half of the children diagnosed with this brain tumor, and diagnosis is an outcome predictor, according to Dr. Cheung.

Infants less than 18 months old have the best outlook, with 88 percent beating the disease. For youngsters 12 years old and older, only about one in 10 outlive neuroblastoma.

For this study, the team sequenced the DNA in tumors from 40 patients with metastatic neuroblastoma between 1987 and 2009. These findings were validated by testing the tumors of 64 additional patients.

Here's what they found in both groups:

  • ATRX mutations were found in 44 percent of the adolescents and young adults over the age of 12
  • In infants under the age of 18 months, no ATRX mutations were present.
  • The mutant gene was seen in 17 percent in children between the ages of 18 months and 12 years.

"These results suggest that inactivation [disruption] of the ATRX pathway correlates with older age at diagnosis and may provide a molecular marker and potential therapeutic target for neuroblastoma among adolescents and young adults," the researchers write.

Future studies need to focus on larger groups of participants from around the world to look at the short- and long-term outcomes of children with and without the ATRX mutations.

This study was published in the March 14 issue of JAMA (Journal of the American Medical Association).

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Review Date: 
March 13, 2012
Last Updated:
March 13, 2012