Medulloblastoma model

Abstract

Medulloblastoma, the largest group of embryonal brain tumors, is highly aggressive, with a dismal prognosis for high-risk patients. This disease has historically been classified into five variants based on histopathology. Recent genetic, epigenetic, and transcriptional analyses have categorized tumors into 4-6 subgroups, which are often inconsistent with histopathology subclasses. Mouse models of medulloblastoma are key to improving our understanding of disease progression and developing targeted therapies for patients. In this chapter, we describe different genetically engineered mouse models, and how they fit in the molecular and histopathological subclasses of human medulloblastoma. We discuss the strengths and weaknesses with which these mouse models mirror human disease, highlighting how insights from these models have informed initiation and progression of disease. Lastly, we review targeted therapeutic agent testing in some of these models and discuss future steps and goals in medulloblastoma modeling and research. © 2013 Springer Science+Business Media New York.