Highlights
- •Current management of paediatric ependymoma.
- •Update on the evolving molecular biology.
- •Shifting paradigm of treatment profile targeting molecular alterations.
Abstract
Ependymomas are the third commonest paediatric central nervous system (CNS) tumour,
accounting for 6–12% of brain tumours in children. The management of these tumours
has seen considerable changes over the last two decades, leading to a significant
improvement in outcomes. However, despite advances in neurosurgical, neuroimaging
and postoperative adjuvant therapy, management of these tumours remain challenging,
and recurrence occurs in over 50% of cases, particularly when complete resection is
not achieved prior to conformal radiotherapy. To-date no chemotherapeutic regimen
has proven to be of significant clinical benefit. Predicting tumour behaviour and
defining robust correlates of disease outcome based on histopathology and clinical
characteristics remains suboptimal. Paucity of cell lines, failure to develop ideal
animal models of these tumours, have precluded better understanding of the oncogenic
drivers, undermining development of targeted therapies. Over the last few years breakthrough
in the understanding of the molecular biology, are now providing clues to therapeutic
insights. It is clear that even with histopathological similarities, these are genetically
heterogeneous tumours with diverse clinical outcomes. Rapid evolution of data based
on genome-wide DNA methylation patterns, have now identified nine molecular subgroups
in these tumours, across three anatomic compartments which include supratentorial
(ST), posterior fossa (PF) and the spinal locations. More recently based on transcriptome
profiling, two subgroups (group A and B) of PF ependymoma have been identified with
distinct molecular, clinical characteristics and specific chromosomal aberrations.
This review includes current management, evolving molecular biology and the shifting
paradigm of treatment profile that targets molecular alterations in paediatric ependymoma.
Keywords
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Article info
Publication history
Published online: November 18, 2016
Accepted:
October 12,
2016
Received in revised form:
September 25,
2016
Received:
July 6,
2016
Identification
Copyright
© 2016 Elsevier Ltd. All rights reserved.
