Chromosome Deletion Predicts Outcome in Neuroblastoma Patients
May 18, 2005 - 1:44:38 AM

Researchers from the Children's Hospital of Philadelphia and the Children's Oncology Group found that patients with neuroblastoma who are missing a portion of one of their chromosomes are less likely to survive than those without this genetic aberration, providing potentially important information to guide therapy.

Neuroblastoma is a cancer of the nervous system that mainly affects children and can be very difficult to treat successfully. Although a combination of chemotherapy, radiation therapy, and surgery can help patients with advanced neuroblastoma achieve remission, many patients experience a relapse of their disease.

"Neuroblastoma is easily cured in some children and is very aggressive in others," said Edward F. Attiyeh, MD, a Hematology/Oncology Fellow at Children's Hospital of Philadelphia working in the lab of John M. Maris, MD, and the study's lead author. "However, our tools for determining who is at greatest risk of relapse have been imprecise. This new genetic marker could help identify those patients who could potentially benefit from more intensive therapy."

Investigators analyzed tissue samples from 917 children with neuroblastoma to determine the relationship between disease progression and a deletion in the "q" arm of chromosome 11. Each chromosome has two arms: a long "q" arm and a short "p" arm.

Patients with the 11q deletion were more likely to have their cancer progress or die of the disease. Three years after diagnosis, 50% of those with an 11q deletion experienced progression of their disease or death, compared with 26% of those without this deletion. Fewer patients with chromosome 11q deletion survived three years compared with those without the deletion (65% vs. 83%).

"The identification of this genetic marker can help us better define neuroblastoma, improve our ability to design therapies, and potentially improve patient survival," concluded Dr. Attiyeh. "Our hope is to find one or more genes on chromosome 11q that are involved in the development of aggressive neuroblastoma, and that these specific genes might be useful as therapeutic targets."

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