Neurosurgical Review

, 31:359

The molecular genetics of medulloblastoma: an assessment of new therapeutic targets

Authors

  • Carlos G. Carlotti Jr
    • Division of Neurosurgery, Ribeirão Preto Medical SchoolUniversity of São Paulo
  • Christian Smith
    • The Division of Neurosurgery, Suite 1503, The Hospital for Sick Children, and The Arthur and Sonia Labatt Brain Tumour Research CentreThe University of Toronto
    • The Division of Neurosurgery, Suite 1503, The Hospital for Sick Children, and The Arthur and Sonia Labatt Brain Tumour Research CentreThe University of Toronto
Review

DOI: 10.1007/s10143-008-0146-4

Cite this article as:
Carlotti Jr, C.G., Smith, C. & Rutka, J.T. Neurosurg Rev (2008) 31: 359. doi:10.1007/s10143-008-0146-4

Abstract

Medulloblastoma is the most common pediatric primary malignant intracranial neoplasm. The 5-year survival rates vary from 40% to 70% depending on clinical prognostic criteria, and many of the patients who survive exhibit long-term neurocognitive and/or neuroendocrine sequelae. Because of these results, research is required to increase our understanding of the basic biology of medulloblastoma, helping to refine patient stratification, decrease side effects of treatments, identify novel prognostic markers, and discover new less toxic therapies. The recognition that some medulloblastomas occur in familial cancer syndromes has led to some important discoveries in the molecular pathogenesis of medulloblastoma. These syndromes provide us with clues regarding alterations in key signaling or growth factor activation pathways that contribute to medulloblastoma formation. A better understanding of the molecular pathways involved in medulloblastoma formation may allow the discovery of new drugs that act on specific targets, yet many steps must still be taken before clinical use of new drugs. In addition, the identification of a novel signaling pathways in medulloblastoma is often accompanied by the quest for novel pharmacotherapeutics that have the potential to act favorably on this disease.

Keywords

MedulloblastomaFamilial cancer syndromesHedgehog pathwayWnt pathwayNotch pathwayCyclopamine

Copyright information

© Springer-Verlag 2008