Abstract
Medulloblastoma (MB) was first described as a distinct neuropathological entity about a century ago, but it is still responsible for significant morbidity and mortality. Over the last decade, we have entered an era where our understanding of the molecular genetics of MB may soon play a role in directing patients’ management and eventually affecting their prognosis and outcome. In this review, we will describe our current knowledge of the presumed cell of origin of MB. We discuss the role of inherited genetic syndromes associated with MB such as Gorlin Syndrome. We review the development of the normal cerebellum in the context of signaling pathways which, if altered, can lead to MB formation. And finally, we will discuss the significance of molecular genetics in the prognosis of MB patients and then summarize the potential areas for further research.
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Acknowledgements
Supported by: Canadian Cancer Society, Grant number: 2011-700751; Pediatric Brain Tumor Foundation of the United States, Brain Tumour Foundation of Canada, Wiley Fund at the Hospital for Sick Children and b.r.a.i.nchild. JTR is a Scientist of the CIHR.
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Nadi, M., Faria, C., Rutka, J.T. (2014). Pathogenesis of Medulloblastoma: Role of Molecular Genetic Alterations. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 12. Tumors of the Central Nervous System, vol 12. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-7217-5_9
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