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.
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.
Authors: Silvia Buonamici; Juliet Williams; Michael Morrissey; Anlai Wang; Ribo Guo; Anthony Vattay; Kathy Hsiao; Jing Yuan; John Green; Beatriz Ospina; Qunyan Yu; Lance Ostrom; Paul Fordjour; Dustin L Anderson; John E Monahan; Joseph F Kelleher; Stefan Peukert; Shifeng Pan; Xu Wu; Sauveur-Michel Maira; Carlos García-Echeverría; Kimberly J Briggs; D Neil Watkins; Yung-mae Yao; Christoph Lengauer; Markus Warmuth; William R Sellers; Marion Dorsch Journal: Sci Transl Med Date: 2010-09-29 Impact factor: 17.956
Authors: Lowell Evan Michael; Bart A Westerman; Alexandre N Ermilov; Aiqin Wang; Jennifer Ferris; Jianhong Liu; Marleen Blom; David W Ellison; Maarten van Lohuizen; Andrzej A Dlugosz Journal: Neoplasia Date: 2008-12 Impact factor: 5.715