Current Oncology Reports

, 20:69 | Cite as

Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors

  • Patrick Sin-Chan
  • Bryan K. Li
  • Ben Ho
  • Adriana Fonseca
  • Annie HuangEmail author
Pediatric Oncology (G Tian, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Pediatric Oncology


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).


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


Brain tumor Cancer CNS-PNET PNET C19MC ETMR ETANTR Ependymoblastoma Medulloepithelioma Pineoblastoma Pediatrics Therapeutics 


Compliance with Ethical Standards

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.

Human and Animal Rights and Informed Consent

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


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major Importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Patrick Sin-Chan
    • 1
  • Bryan K. Li
    • 1
    • 2
  • Ben Ho
    • 1
  • Adriana Fonseca
    • 1
  • Annie Huang
    • 1
    • 2
    Email author
  1. 1.Arthur and Sonia Labatt Brain Tumour Research Centre, Division of Haematology/OncologyHospital for Sick ChildrenTorontoCanada
  2. 2.Department of Laboratory Medicine and Pathobiology, Faculty of MedicineUniversity of TorontoTorontoCanada

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