A contemporary review of molecular candidates for the development and treatment of childhood medulloblastoma
Introduction Medulloblastoma is the most common pediatric central nervous system tumor; however, the causes are not well established. There has been some emphasis on mutations in developmental pathways and their impact on tumor pathology in hereditary diseases, but, in order to better understand the...
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Veröffentlicht in: | Child's nervous system 2013-03, Vol.29 (3), p.381-388 |
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Format: | Artikel |
Sprache: | eng |
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Zusammenfassung: | Introduction
Medulloblastoma is the most common pediatric central nervous system tumor; however, the causes are not well established. There has been some emphasis on mutations in developmental pathways and their impact on tumor pathology in hereditary diseases, but, in order to better understand the nature of diseases like medulloblastoma, other mechanisms also require attention.
Purpose
The purpose of this review is to provide an overview of the main genes involved in neurodevelopment, their downstream targets, and modulatory links by growth factors. Occurrence of pediatric brain tumors including medulloblastoma are mostly sporadic, but some hereditary diseases like Li–Fraumeni syndrome, Gorlin’s syndrome, Turcot’s syndrome, and Rubenstein–Tarbi syndrome are known to contribute their development as consequences of germline mutations at specific points: DNA-repairing gene Tp53 for Li–Fraumeni syndrome or Patch for Gorlin’s, and apoptosis-related gene product adenomatous polyposis coli for Turcot’s disease.
Conclusion
Intracellular relations at molecular level and future therapeutics that specifically target the corresponding pathways should be well understood in order to prevent and cure childhood medulloblastoma. |
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ISSN: | 0256-7040 1433-0350 |
DOI: | 10.1007/s00381-012-2014-3 |