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Medulloblastoma : experimental models and reality

Neumann, Julia E. (author)
Univ Med Ctr Hamburg Eppendorf, Inst Neuropathol, Hamburg, Germany.
Johansson, Fredrik K., 1975- (author)
Uppsala universitet,Neuroonkologi,Science for Life Laboratory, SciLifeLab
Schueller, Ulrich (author)
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.
Univ Med Ctr Hamburg Eppendorf, Inst Neuropathol, Hamburg, Germany Neuroonkologi (creator_code:org_t)
2017-07-19
2017
English.
In: Acta Neuropathologica. - : Springer Science and Business Media LLC. - 0001-6322 .- 1432-0533. ; 134:5, s. 679-689
  • Research review (peer-reviewed)
Abstract Subject headings
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  • 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.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Cancer and Oncology (hsv//eng)

Keyword

Medulloblastoma
Mouse model
SHH
WNT
Group 3
Group 4

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