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Medulloblastoma: Tumor Biology and Relevance to Treatment and Prognosis Paradigm
Authors:Daniel Coluccia  Carlyn Figuereido  Semra Isik  Christian Smith  James T. Rutka
Affiliation:1.The Arthur and Sonia Labatt Brain Tumor Research Centre, Division of Neurosurgery, The Hospital for Sick Children,the University of Toronto,Toronto,Canada;2.Department of Neurosurgery,Cantonal Hospital of Aarau,Aarau,Switzerland
Abstract:Medulloblastoma is a malignant embryonic brain tumor arising in the posterior fossa and typically occurring in pediatric patients. Current multimodal treatment regimes have significantly improved the survival rates; however, a marked heterogeneity in therapy response is observed, and one third of all patients die within 5 years after diagnosis. Large-scale genetic and transcriptome analysis revealed four medulloblastoma subgroups (WNT, SHH, Group 3, and Group 4) associated with different demographic parameters, tumor manifestation, and clinical behavior. Future treatment protocols will integrate molecular classification schemes to evaluate subgroup-specific intensification or de-escalation of adjuvant therapies aimed to increase tumor control and reduce iatrogenic induced morbidity. Furthermore, the identification of genetic drivers allows assessing target therapies in order to increase the chemotherapeutic armamentarium. This review highlights the biology behind the current classification system and elucidates relevant aspects of the disease influencing forthcoming clinical trials.
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