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Bioengineering 2018, 5(4), 78; https://doi.org/10.3390/bioengineering5040078

Embryonal Tumors of the Central Nervous System in Children: The Era of Targeted Therapeutics

1
Section of Pediatric Hematology-Oncology, Department of Pediatrics, Wake Forest School of Medicine, Winston Salem, NC 27157, USA
2
Pape Family Pediatric Research Institute, Department of Pediatrics, Oregon Health and Science University, Portland, OR 97239, USA
3
Department of Pediatrics, MD Anderson Cancer Center, Houston, TX 77030, USA
4
Department of Pediatrics, Wake Forest School of Medicine, Winston Salem, NC 27157, USA
5
Humanitas University Medical School, 20090 Milan, Italy
*
Author to whom correspondence should be addressed.
These authors contributed equally the writing and preparation of the article.
Received: 17 August 2018 / Revised: 12 September 2018 / Accepted: 12 September 2018 / Published: 23 September 2018
(This article belongs to the Special Issue Update in Pediatric Neuro-Oncology)
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Abstract

Embryonal tumors (ET) of the central nervous system (CNS) in children encompass a wide clinical spectrum of aggressive malignancies. Until recently, the overlapping morphological features of these lesions posed a diagnostic challenge and undermined discovery of optimal treatment strategies. However, with the advances in genomic technology and the outpouring of biological data over the last decade, clear insights into the molecular heterogeneity of these tumors are now well delineated. The major subtypes of ETs of the CNS in children include medulloblastoma, atypical teratoid rhabdoid tumor (ATRT), and embryonal tumors with multilayered rosettes (ETMR), which are now biologically and clinically characterized as different entities. These important developments have paved the way for treatments guided by risk stratification as well as novel targeted therapies in efforts to improve survival and reduce treatment burden. View Full-Text
Keywords: central nervous system; embryonal tumors; children; medulloblastoma; ATRT; ETMR; molecular biology; targeted therapeutics central nervous system; embryonal tumors; children; medulloblastoma; ATRT; ETMR; molecular biology; targeted therapeutics
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
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Kram, D.E.; Henderson, J.J.; Baig, M.; Chakraborty, D.; Gardner, M.A.; Biswas, S.; Khatua, S. Embryonal Tumors of the Central Nervous System in Children: The Era of Targeted Therapeutics. Bioengineering 2018, 5, 78.

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