Pediatric embryonal brain tumors in the molecular era |
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| Authors: | Bryan K. Li Salma Al-Karmi Annie Huang |
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| Affiliation: | 1. Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, ON, Canada;2. Arthur and Sonia Labatt Brain Tumour Research Centre, Hospital for Sick Children, Toronto, ON, Canada;3. Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, ON, Canada;4. Arthur and Sonia Labatt Brain Tumour Research Centre, Hospital for Sick Children, Toronto, ON, Canada |
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| Abstract: | ABSTRACTIntroduction: Embryonal brain tumors (EBTs) are highly aggressive malignancies predominantly affecting children. They include medulloblastoma (MB), atypical rhabdoid/teratoid tumors (ATRT), pineoblastoma (PB), embryonal tumor multiple rosettes (ETMR)/C19MC-altered tumors, and newly recognized embryonal tumors with FOXR2 activation or BCOR alteration.Areas covered: This review will provide a comprehensive overview and updated of the literature on each of these EBTs. The evolution from location- and histopathology-based diagnosis to more specific and robust molecular-based classification schemes, as well as treatment modalities, will be discussed.Expert commentary: The subgrouping of EBTs with multi-omic profiling has had important implications for risk stratification and discovery of targetable driver pathways. However, these innovations are unlikely to significantly improve survival among high-risk patients until robust preclinical studies are conducted, followed by validation in biology-informed clinical trials. |
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| Keywords: | Atypical teratoid rhabdoid tumor ATRT C19MC-altered tumor embryonal tumor multiple rosettes ETMR medulloblastoma pineoblastoma |
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