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Acta Neuropathologica

, Volume 134, Issue 5, pp 679–689 | Cite as

Medulloblastoma: experimental models and reality

  • Julia E. Neumann
  • Fredrik J. Swartling
  • Ulrich Schüller
Review

Abstract

Medulloblastoma is the most frequent malignant brain tumor in childhood, but it may also affect infants, adolescents, and young adults. Recent advances in the understanding of the disease have shed light on molecular and clinical heterogeneity, which is now reflected in the updated WHO classification of brain tumors. At the same time, it is well accepted that preclinical research and clinical trials have to be subgroup-specific. Hence, valid models have to be generated specifically for every medulloblastoma subgroup to properly mimic molecular fingerprints, clinical features, and responsiveness to targeted therapies. This review summarizes the availability of experimental medulloblastoma models with a particular focus on how well these models reflect the actual disease subgroup. We further describe technical advantages and disadvantages of the models and finally point out how some models have successfully been used to introduce new drugs and why some medulloblastoma subgroups are extraordinary difficult to model.

Keywords

Medulloblastoma Mouse model SHH WNT Group 3 Group 4 

Notes

Acknowledgements

The authors thank Dr. R. Gilbertson for providing images on murine WNT medulloblastoma. U.S. is supported by the Fördergemeinschaft Kinderkrebs-Zentrum Hamburg.

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Copyright information

© Springer-Verlag GmbH Germany 2017

Authors and Affiliations

  1. 1.Institute of NeuropathologyUniversity Medical Center Hamburg-EppendorfHamburgGermany
  2. 2.Department of Immunology, Genetics and Pathology, Science for Life Laboratory, Rudbeck LaboratoryUppsala UniversityUppsalaSweden
  3. 3.Department of Pediatric Hematology and OncologyUniversity Medical Center Hamburg-EppendorfHamburgGermany
  4. 4.Research Institute Children’s Cancer CenterHamburgGermany

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