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Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors

  • Pediatric Oncology (G Tian, Section Editor)
  • Published:
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Abstract

Purpose of Review

Malignant embryonal brain tumors (EBTs) of childhood span a wide clinical spectrum but can share remarkably similar morphologic features. This overlap presents significant diagnostic challenges, particularly for tumor entities that are rarely encountered in clinical practice and for which diagnostic criteria were poorly defined. This review will provide an update on the evolving characterization and treatment of rare EBTs.

Recent Findings

Rapid advances in genomic tools have led to the discovery of robust molecular markers, and identification of novel tumor types and subtypes for almost all major categories of pediatric brain tumors. These developments have had significant impact on improving the diagnostic classification of the rare EBTs, particularly for tumors with newly recognized C19MC alterations, central nervous system primitive neuroectodermal tumors (CNS-PNET), and pineoblastoma (PB).

Summary

These important developments in the clinical and molecular understanding of rare EBTs are paving the way for novel therapeutic strategies and improved clinical management.

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Author notes

  1. Patrick Sin-Chan and Bryan K. Li contributed equally to this work.

Authors and Affiliations

  1. Arthur and Sonia Labatt Brain Tumour Research Centre, Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Ontario, M5G1X8, Canada

    Patrick Sin-Chan, Bryan K. Li, Ben Ho, Adriana Fonseca & Annie Huang

  2. Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, Ontario, M5S3H7, Canada

    Bryan K. Li & Annie Huang

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  1. Patrick Sin-Chan
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Correspondence to Annie Huang.

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Conflict of Interest

Patrick Sin-Chan was an employee of Regeneron Pharmaceuticals at the time this article was written.

Bryan K. Li declares that he has no conflict of interest.

Ben Ho declares that he has no conflict of interest.

Adriana Fonseca declares that she has no conflict of interest.

Annie Huang declares that she has no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Pediatric Oncology

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Sin-Chan, P., Li, B.K., Ho, B. et al. Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors. Curr Oncol Rep 20, 69 (2018). https://doi.org/10.1007/s11912-018-0717-7

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  • DOI: https://doi.org/10.1007/s11912-018-0717-7

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