Biological background of pediatric medulloblastoma and ependymoma: A review from a translational research perspective

¹ Children's National Medical Center, The Brain Tumor Institute, Division of Neurology, Washington, USA
² Erasmus MC, Sophia Children's Hospital, Division of Pediatric Oncology and Hematology, Rotterdam, The Netherlands

^* To whom correspondence should be addressed. E-mail: j.debont{at}erasmusmc.nl.

	Abstract

Survival rates of pediatric brain tumor patients have significantly improved over the years due to developments in diagnostic techniques, neurosurgery, chemotherapy, radiotherapy and supportive care. However, brain tumors are still an important cause of cancer-related deaths in children. Prognosis is still highly dependent on clinical characteristics, such as the age of the patient, tumor type, stage and localization, but increased knowledge about the genetic and biological features of these tumors is being obtained and might be useful to further improve the outcome of these patients. It has become clear that the deregulation of signaling pathways essential in brain development, e.g. SHH, Wnt and Notch, plays an important role in the pathogenesis and biological behavior of especially medulloblastomas. More recently, data have become available about the cells of origin of brain tumors and about the possible existence of brain tumor stem cells. Newly developed array-based techniques for studying gene expression, protein expression, copy number aberrations and epigenetic events have led to the identification of other potentially important biological abnormalities in pediatric medulloblastomas and ependymomas.

Key Words: biological characteristics, ependymoma, epigenetic events, gene expression, medulloblastoma, protein expression, signaling pathways

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