Cancer Cell
Volume 20, Issue 2, 16 August 2011, Pages 143-157
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Article
Delineation of Two Clinically and Molecularly Distinct Subgroups of Posterior Fossa Ependymoma

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Summary

Despite the histological similarity of ependymomas from throughout the neuroaxis, the disease likely comprises multiple independent entities, each with a distinct molecular pathogenesis. Transcriptional profiling of two large independent cohorts of ependymoma reveals the existence of two demographically, transcriptionally, genetically, and clinically distinct groups of posterior fossa (PF) ependymomas. Group A patients are younger, have laterally located tumors with a balanced genome, and are much more likely to exhibit recurrence, metastasis at recurrence, and death compared with Group B patients. Identification and optimization of immunohistochemical (IHC) markers for PF ependymoma subgroups allowed validation of our findings on a third independent cohort, using a human ependymoma tissue microarray, and provides a tool for prospective prognostication and stratification of PF ependymoma patients.

Highlights

► There are two molecularly distinct groups of posterior fossa ependymoma ► The two subgroups are transcriptionally, genetically, and clinically divergent ► The two subgroups are distinguished by different signaling pathways ► IHC allows for routine subgroup assignment and patient prognostication

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These authors contributed equally to this work