A comprehensive review of paediatric low-grade diffuse glioma: pathology, molecular genetics and treatment.
Gliomas are the most common central nervous system neoplasms affecting children and can be both high- and low-grade. Paediatric low-grade glioma may be either World Health Organization grade I or grade II. Despite being classified as grade II diffuse astrocytoma, these neoplasms arising in children are distinct clinically and molecularly from their adult counterparts. They do not tend to progress to higher grade lesions and only rarely harbour an IDH mutation. Here, we review the clinical, histologic and molecular features of paediatric grade II diffuse glioma, highlighting their diagnostic criteria, prevalence across brain locations, their most common molecular features and how to test for them, and lastly the current status of therapeutic options available for their treatment.
The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G1X8, Canada.|Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, ON, Canada.
Scott
S
Ryall
The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G1X8, Canada.|Division of Haematology and Oncology, The Hospital for Sick Children, Toronto, ON, Canada.
Uri
U
Tabori
The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G1X8, Canada. cynthia.hawkins@sickkids.ca.|Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, ON, Canada. cynthia.hawkins@sickkids.ca.|Division of Pathology, The Hospital for Sick Children, Toronto, ON, Canada. cynthia.hawkins@sickkids.ca.
Cynthia
C
Hawkins
D001932:Brain Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D019941:Cyclin-Dependent Kinase Inhibitor p16; D050763:Cyclin-Dependent Kinase Inhibitor p18 / Q000235:genetics; D017353:Gene Deletion; D005910:Glioma / Q000235:genetics* / Q000473:pathology / Q000628:therapy*; D006657:Histones / Q000235:genetics; D006801:Humans; D007521:Isocitrate Dehydrogenase / Q000235:genetics; D058990:Molecular Targeted Therapy; D009154:Mutation; D009367:Neoplasm Staging; D011518:Proto-Oncogene Proteins / Q000235:genetics; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D051496:Receptor, Fibroblast Growth Factor, Type 1 / Q000235:genetics; D015534:Trans-Activators / Q000235:genetics; D016159:Tumor Suppressor Protein p53 / Q000235:genetics
C000614131:CDKN2A protein, human; D019941:Cyclin-Dependent Kinase Inhibitor p16; D050763:Cyclin-Dependent Kinase Inhibitor p18; C000609950:H3-3A protein, human; D006657:Histones; C088831:MYBL1 protein, human; D011518:Proto-Oncogene Proteins; C495901:TP53 protein, human; D015534:Trans-Activators; D016159:Tumor Suppressor Protein p53; D007521:Isocitrate Dehydrogenase; C496348:FGFR1 protein, human; D051496:Receptor, Fibroblast Growth Factor, Type 1; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf
Clinical trials; Histology; Incidence; Low-grade diffuse glioma; Molecular events; Paediatric