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Medulloblastoma: experimental models and reality
Univ Med Ctr Hamburg Eppendorf, Inst Neuropathol, Hamburg, Germany..
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Neuro-Oncology. Uppsala University, Science for Life Laboratory, SciLifeLab.ORCID iD: 0000-0002-8460-4367
Univ Med Ctr Hamburg Eppendorf, Inst Neuropathol, Hamburg, Germany.;Univ Med Ctr Hamburg Eppendorf, Dept Pediat Hematol & Oncol, Hamburg, Germany.;Res Inst Childrens Canc Ctr, Martinistr 52, D-20251 Hamburg, Germany..
2017 (English)In: Acta Neuropathologica, ISSN 0001-6322, E-ISSN 1432-0533, Vol. 134, no 5, p. 679-689Article, review/survey (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
2017. Vol. 134, no 5, p. 679-689
Keywords [en]
Medulloblastoma, Mouse model, SHH, WNT, Group 3, Group 4
National Category
Cancer and Oncology
Identifiers
URN: urn:nbn:se:uu:diva-346784DOI: 10.1007/s00401-017-1753-3ISI: 000413247100001PubMedID: 28725965OAI: oai:DiVA.org:uu-346784DiVA, id: diva2:1192452
Available from: 2018-03-22 Created: 2018-03-22 Last updated: 2018-03-22Bibliographically approved

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Johansson, Fredrik K.

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