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Chinese Journal of Cancer 2011年第7期30卷 442-449页

作者： Paul A.Northcott Sidney Croul Michael D.Taylor arthur and sonia labatt brain Tumor research Center Hospital for Sick Children

Medulloblastoma is the most common malignant pediatric brain tumor. Despite its prevalence and importance in pediatric neuro-oncology, the genes and pathways responsible for its initiation, maintenance, and progression remain poorly understood. Genetically engineered mouse models are an essential tool for uncovering the molecular and cellular basis of human diseases, including cancer, and serve a valuable role as preclinical models for testing targeted therapies. In this review, we summarize how such models have been successfully applied to the study of medulloblastoma over the past decade and what we might expect in the coming years.

关键词：小鼠模型母细胞遗传工程人类疾病脑肿瘤发病率小儿神经

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Molecular genetics of ependymoma

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Chinese Journal of Cancer 2011年第10期30卷 669-681页

作者： Yuan Yao Stephen C. Mack Michael D. Taylor Division of Neurosurgery Arthur and Sonia LabattBrain Tumour Research Centre Hospital for Sick Children TorontoOntario Canada Department of Laboratory Medicine and Pathobiology University of Toronto Toronto Ontario Canada.

brain tumors are the leading cause of cancer death in children,with ependymoma being the third most common and posing a significant clinical *** mechanism of pathogenesis,reliable prognostic indicators,and effective treatments other than surgical resection have all remained *** recently,ependymoma research was hindered by the small number of tumors available for study,low resolution of cytogenetic techniques,and lack of cell lines and animal *** heterogeneity,which manifests as variations in tumor location,patient age,histological grade,and clinical behavior,together with the observation of a balanced genomic profile in up to 50% of cases,presents additional challenges in understanding the development and progression of this *** these difficulties,we have made significant headway in the past decade in identifying the genetic alterations and pathways involved in ependymoma tumorigenesis through collaborative efforts and the application of microarray-based genetic(copy number) and transcriptome profiling *** characterization of ependymoma unraveled distinct mRNA-defined subclasses and led to the identification of radial glial cells as its cell type of *** review summarizes our current knowledge in the molecular genetics of ependymoma and proposes future research directions necessary to further advance this field.

关键词：分子遗传学室管膜肿瘤发生发病机制手术切除动物模型遗传变异遗传特性

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Diffuse intrinsic pontine glioma:current insights and future directions

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Chinese Neurosurgical Journal 2021年第4期7卷 289-299页

作者： Dilakshan Srikanthan Michael S.Taccone Randy Van Ommeren Joji Ishida Stacey L.Krumholtz James T.Rutka Cell Biology Program The Hospital for Sick Children686 Bay StTorontoON M5G 0A4Canada the arthur and sonia labatt brain tumour research centre The Hospital for Sick Children686 Bay StTorontoON M5G 0A4Canada Department of Laboratory Medicine and Pathobiology University of TorontoTorontoONCanada Division of Neurosurgery Department of SurgeryThe Ottawa HospitalOttawaONCanada Developmental and Stem Cell Biology Program The Hospital for Sick Children686 Bay StTorontoON M5G 0A4Canada Institute of Medical Sciences University of TorontoTorontoONCanada Division of Neurosurgery Department of SurgeryThe Hospital for Sick ChildrenSuite 1503555University AvenueTorontoON M5G 1X8Canada

Diffuse intrinsic pontine glioma(DIPG)is a lethal pediatric brain tumor and the leading cause of brain tumor–related death in *** several clinical trials over the past few decades have led to no significant improvements in outcome,the current standard of care remains fractionated focal *** to the recent increase in stereotactic biopsies,tumor tissue availabilities have enabled our advancement of the genomic and molecular characterization of this lethal *** groups have identified key histone gene mutations,genetic drivers,and methylation changes in DIPG,providing us with new insights into DIPG ***,there has been increased development of in vitro and in vivo models of DIPG which have the capacity to unveil novel therapies and strategies for drug *** review outlines the clinical characteristics,genetic landscape,models,and current treatments and hopes to shed light on novel therapeutic avenues and challenges that remain.

关键词： Diffuse intrinsic pontine glioma Molecular genetics Neuro-oncology Therapeutics Disease models Pediatrics Neurosurgery

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