Child's Nervous System

, Volume 28, Issue 4, pp 521–532

Matching mice to malignancy: molecular subgroups and models of medulloblastoma

  • Jasmine Lau
  • Christin Schmidt
  • Shirley L. Markant
  • Michael D. Taylor
  • Robert J. Wechsler-Reya
  • William A. Weiss
Review Paper

DOI: 10.1007/s00381-012-1704-1

Cite this article as:
Lau, J., Schmidt, C., Markant, S.L. et al. Childs Nerv Syst (2012) 28: 521. doi:10.1007/s00381-012-1704-1

Abstract

Introduction

Medulloblastoma, the largest group of embryonal brain tumors, has historically been classified into five variants based on histopathology. More recently, epigenetic and transcriptional analyses of primary tumors have subclassified medulloblastoma into four to six subgroups, most of which are incongruous with histopathological classification.

Discussion

Improved stratification is required for prognosis and development of targeted treatment strategies, to maximize cure and minimize adverse effects. Several mouse models of medulloblastoma have contributed both to an improved understanding of progression and to developmental therapeutics. In this review, we summarize the classification of human medulloblastoma subtypes based on histopathology and molecular features. We describe existing genetically engineered mouse models, compare these to human disease, and discuss the utility of mouse models for developmental therapeutics. Just as accurate knowledge of the correct molecular subtype of medulloblastoma is critical to the development of targeted therapy in patients, we propose that accurate modeling of each subtype of medulloblastoma in mice will be necessary for preclinical evaluation and optimization of those targeted therapies.

Keywords

MedulloblastomaMouse modelsMolecular subgroupsTargeted therapiesPreclinical testing

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Jasmine Lau
    • 1
    • 2
    • 3
    • 4
  • Christin Schmidt
    • 1
    • 2
    • 3
    • 4
  • Shirley L. Markant
    • 5
    • 6
  • Michael D. Taylor
    • 7
    • 8
  • Robert J. Wechsler-Reya
    • 5
    • 6
  • William A. Weiss
    • 1
    • 2
    • 3
    • 4
  1. 1.Department of NeurologyUniversity of CaliforniaSan FranciscoUSA
  2. 2.Helen Diller Family Comprehensive Cancer CenterUniversity of CaliforniaSan FranciscoUSA
  3. 3.Department of Neurological Surgery and Brain Tumor Research CenterUniversity of CaliforniaSan FranciscoUSA
  4. 4.Department of PediatricsUniversity of CaliforniaSan FranciscoUSA
  5. 5.Tumor Development ProgramSanford-Burnham Medical Research InstituteLa JollaUSA
  6. 6.Department of Pharmacology & Cancer BiologyDuke University Medical CenterDurhamUSA
  7. 7.Division of Neurosurgery, Hospital for Sick ChildrenUniversity of TorontoTorontoCanada
  8. 8.Arthur and Sonia Labatt Brain Tumour Research Centre, Program in Developmental and Stem Cell Biology, Hospital for Sick ChildrenUniversity of TorontoTorontoCanada