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JOURNAL OF NEUROSURGICAL SCIENCES

A Journal on Neurosurgery


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Journal of Neurosurgical Sciences 2018 February;62(1):46-50

DOI: 10.23736/S0390-5616.17.04152-2

Copyright © 2017 EDIZIONI MINERVA MEDICA

language: English

Epidemiology, molecular classification and WHO grading of ependymoma

Jens-Martin HÜBNER 1, Marcel KOOL 1, Stefan M. PFISTER 1, 2, Kristian W. PAJTLER 1, 2

1 Division of Pediatric Neurooncology, German Consortium for Translational Cancer Research (DKTK), German Cancer Research Center (DKFZ), Heidelberg, Germany; 2 Department of Pediatric Hematology and Oncology, Heidelberg University Hospital, Heidelberg, Germany


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Ependymoma can arise throughout all compartments of the central nervous system with prevalence for intracranial and spinal location in children and adults, respectively. The current histopathology based WHO grading system distinguishes grade I, II ‘classic’, and III ‘anaplastic’ ependymoma. However, analysis of multiple cohorts of intracranial ependymoma demonstrate a wide variance in the utility of the grade II versus grade III distinction as a prognostic marker that may additionally be confounded by the anatomic compartment. Recent (epi)genomic profiling efforts have identified molecularly distinct groups of ependymoma arising from all three anatomic compartments of the central nervous system that outperform the current histopathological classification regarding clinical associations. These advances have led to the cognition that molecular classification should be part of all future clinical trials in ependymoma patients. Clinical management of intracranial ependymomas (WHO Grade II/III) is challenging and molecular classification based risk stratification may help to intensify treatment and surveillance in high-risk patients but to de-escalate therapy in certain patient groups at low risk for recurrence. Finally, experience of neurosurgeons, and other disciplines, as well as intensified co-operation between all stakeholders involved hold promise to finally improve outcome of patients affected with ependymoma.


KEY WORDS: Ependymoma - Neoplasm grading - Neoplasms, neuroepithelial

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Publication History

Issue published online: December 5, 2017
Article first published online: September 8, 2017
Manuscript accepted: July 17, 2017
Manuscript received: July 10, 2017

Cite this article as

Hübner JM, Kool M, Pfister SM, Pajtler KW. Epidemiology, molecular classification and WHO grading of ependymoma. J Neurosurg Sci 2018;62:46-50. DOI: 10.23736/S0390-5616.17.04152-2

Corresponding author e-mail

k.pajtler@dkfz-heidelberg.de