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Washington University

660 South Euclid Avenue, Campus Box 8018
St. Louis, MO 63110
United States

Background


A major barrier to progress in pediatric brain cancer prevention and control is the lack of understanding of factors that influence its development and outcomes. This limitation is especially relevant to pediatric brain tumors, where few environmental or genetic risk factors have been identified. Studies conducted in individuals with cancer syndromes may increase our understanding of pediatric brain tumor causes due to their extreme sensitivity to tumor development.

Background


During their development, immune cells must intentionally generate and repair breaks in their DNA in order to assemble the genes necessary for a diverse immune response. Multiple signals in early immune cells cooperate to ensure that these DNA breaks are properly repaired. Errors in these processes can derail normal development and trigger transformation into leukemia.

Acute lymphoblastic leukemia (ALL) is the most common cancer in children. Approximately 15% of newly diagnosed ALL cases in children arise from dysfunctional T-cells. T-cell ALL (T-ALL) patients are historically linked with a poor prognosis. DNA methylation is a modification to DNA that does not alter the DNA sequence, but can act with genetic mutations in cancer. DNA methylation introduces marks or "flags" to genes. The exact pattern of methylation flags can turn certain genes on and off.

A major barrier to progress in pediatric brain cancer prevention and treatment is the lack of understanding of factors that influence its development and prognosis. This limitation is especially relevant to pediatric brain tumors, where few environmental or genetic risk factors have been identified. Studies conducted in individuals with cancer syndromes may increase our understanding of pediatric brain tumor causes and poor outcomes. In this study, we will employ an inherited tumor predisposition syndrome (neurofibromatosis type 1; NF1) to study risk factors for pediatric brain cancer.

Todd Druley

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