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University of Pennsylvania

3451 Walnut Street
Philadelphia, PA 19004
United States

Background


Beckwith-Wiedemann Syndrome (BWS) is a childhood overgrowth and cancer predisposition disorder due to changes on chromosome 11. Genetic changes similar to those observed in BWS are widespread and have been reported in isolated breast, pancreatic, adrenal, liver, renal, prostate, and colon cancers.

Pediatric brain tumors are one of the most common malignancies among children and have been one of the leading causes of cancer-related deaths in the recent times. More than 4000 children in the U.S are diagnosed with some form of pediatric brain tumor everyday ranging from the low grade to the high grade tumors and about 7% of the reported brain tumors occur in children with ages between 0-19 years. High grade tumors like glioblastoma multiforme (GBM) are especially difficult to treat since they grow and spread very rapidly within the brain tissue.

While five-year survival rates for survivors of childhood brain tumors have slowly improved to 66% because of advances in care, brain tumor survivors report the lowest quality of life of all childhood cancer survivors. Treatment can affect cognitive and physical functioning in a variety of ways, including neurodevelopmental late effects (changes in overall I.Q., slower processing speed, decline in short-term memory, poor attention span and impaired organizational skills).

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