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Published in: Acta Neuropathologica 2/2012

01-08-2012 | Original Paper

Distinct disease-risk groups in pediatric supratentorial and posterior fossa ependymomas

Authors: Catherine Godfraind, Joanna M. Kaczmarska, Mehmet Kocak, James Dalton, Karen D. Wright, Robert A. Sanford, Fredrick A. Boop, Amar Gajjar, Thomas E. Merchant, David W. Ellison

Published in: Acta Neuropathologica | Issue 2/2012

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Abstract

No reliable classification is in clinical use for the therapeutic stratification of children with ependymoma, such that disease risk might be identified and patients treated to ensure a combination of maximal cure rates and minimal adverse therapeutic effects. This study has examined associations between clinicopathologic and cytogenetic variables and outcome in a trial cohort of children with ependymoma, with the aim of defining a practical scheme for stratifying this heterogeneous tumor. Intracranial ependymomas (n = 146) from children treated on the RT1 trial at St. Jude Children’s Research Hospital were evaluated for the status of multiple pathological features. Interphase FISH (iFISH) defined the status of loci on chromosomes 1q (EXO1), 6q (LATS1) and 9, including 9p21 (CDKN2A). Data relating to these clinicopathological and cytogenetic variables were compared with survival data in order to model disease risk groups. Extent of surgical resection was a significant determinant of outcome in both supratentorial and infratentorial compartments. Tumor cell density and mitotic count were associated with outcome among children with posterior fossa ependymomas (n = 119). Among pathologic features, only brain invasion was associated with outcome in children with supratentorial ependymomas (n = 27). For posterior fossa tumors, gain of 1q was independently associated with outcome and in combination with clinicopathological variables defined both a two-tier and three-tier system of disease risk. Among children developing posterior fossa ependymomas treated with maximal surgical resection and conformal radiotherapy, key clinicopathological variables and chromosome 1q status can be used to define tiers of disease risk. In contrast, risk factors for pediatric supratentorial tumors are limited to sub-total resection and brain invasion.
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Metadata
Title
Distinct disease-risk groups in pediatric supratentorial and posterior fossa ependymomas
Authors
Catherine Godfraind
Joanna M. Kaczmarska
Mehmet Kocak
James Dalton
Karen D. Wright
Robert A. Sanford
Fredrick A. Boop
Amar Gajjar
Thomas E. Merchant
David W. Ellison
Publication date
01-08-2012
Publisher
Springer-Verlag
Published in
Acta Neuropathologica / Issue 2/2012
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI
https://doi.org/10.1007/s00401-012-0981-9

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