A new study reveals key molecular indicators that could help doctors select the best form of treatment for patients with neuroblastoma - the most common type of cancer in infants. The new study, which involved a genomic analysis of over 400 neuroblastomas, suggests that tumors with telomere maintenance mechanisms and certain genetic alterations are at higher-risk and may need to be treated more aggressively than tumors without these features. Neuroblastoma is a pediatric tumor that arises from early nerve cells of the sympathetic nervous system. Some neuroblastomas are fatal despite treatment, others respond well to it, and still others undergo spontaneous regression without any treatment whatsoever. A molecular classification scheme that predicts the likely clinical course of the disease at the time of diagnosis could help oncologists select the best treatment for each patient. Previous research had suggested a link between clinical outcome and the presence or absence of mutations in genes known to influence the maintenance of telomeres (DNA sequences capping the ends of chromosomes). Sandra Ackermann and colleagues sequenced over 400 pre-treatment neuroblastomas and identified molecular features that correlate with distinct clinical outcomes. According to Ackermann et al., low-risk tumors lack telomere maintenance mechanisms; intermediate-risk tumors harbor telomere maintenance mechanisms; and highest-risk tumors harbor telomere maintenance mechanisms in combination with mutations in RAS and/or p53 pathway genes.
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Journal
Science