title: Medulloblastoma: Tumor Biology and Relevance to Treatment and Prognosis Paradigm.
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.
authors.affiliation: The Arthur and Sonia Labatt Brain Tumor Research Centre, Division of Neurosurgery, The Hospital for Sick Children, the University of Toronto, Suite 1503, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.|Department of Neurosurgery, Cantonal Hospital of Aarau, Aarau, Switzerland.
authors.forename: Daniel
authors.initials: D
authors.lastname: Coluccia
authors.affiliation: The Arthur and Sonia Labatt Brain Tumor Research Centre, Division of Neurosurgery, The Hospital for Sick Children, the University of Toronto, Suite 1503, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
authors.forename: Carlyn
authors.initials: C
authors.lastname: Figuereido
authors.affiliation: The Arthur and Sonia Labatt Brain Tumor Research Centre, Division of Neurosurgery, The Hospital for Sick Children, the University of Toronto, Suite 1503, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
authors.forename: Semra
authors.initials: S
authors.lastname: Isik
authors.affiliation: The Arthur and Sonia Labatt Brain Tumor Research Centre, Division of Neurosurgery, The Hospital for Sick Children, the University of Toronto, Suite 1503, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
authors.forename: Christian
authors.initials: C
authors.lastname: Smith
authors.affiliation: The Arthur and Sonia Labatt Brain Tumor Research Centre, Division of Neurosurgery, The Hospital for Sick Children, the University of Toronto, Suite 1503, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. james.rutka@sickkids.ca.
authors.forename: James T
authors.initials: JT
authors.lastname: Rutka
mesh_terms: D000818:Animals; D002528:Cerebellar Neoplasms / Q000235:genetics / Q000473:pathology / Q000628:therapy; D006801:Humans; D008527:Medulloblastoma / Q000235:genetics / Q000628:therapy; D064847:Multimodal Imaging; D011379:Prognosis; D012008:Recurrence; D012307:Risk Factors
keywords: Gene expression; Medulloblastoma; Molecular subgroups; Risk stratification; Target therapies