Trial Information

Examination of the Multiple Genetic and Molecular Targets as Therapeutic Options for Patients With Ependymoma Treated by the Phase II Children's Oncology Group Study ACNS0121

OBJECTIVES:

- To examine the prognostic role of histopathological variables, in particular cellular
density, mitotic count, and tumor cell invasion in intracranial pediatric ependymomas.

- To study whether hTERT expression and telomere dysfunction correlate with
progression-free survival (PFS) and overall survival (OS) in pediatric intracranial
ependymoma.

- To perform a genome-wide copy number screen and validation of copy number abnormalities
(CNAs) on formalin-fixed paraffin-embedded (FFPE) ependymomas using Affymetrix
Molecular Inversion Probe (MIP) arrays and interphase fluorescence in situ
hybridization (iFISH).

- To evaluate associations between infiltration of immune markers and PFS as well as OS
in pediatric ependymoma.

- To examine the role of 1q gain and 9p deletion in pediatric ependymomas by exploring
their association with PFS and OS in a multivariable model.

- To establish the frequency and clinicopathological associations of mutations in genes
involved in Notch pathway signaling.

OUTLINE: Archived tumor tissue samples are analyzed for cellular density, mitotic count,
tumor cell invasion, hTERT expression, telomere dysfunction, 1q gain, 9p deletion, and
genetic mutations by IHC, Affymetrix Molecular Inversion Probe (MIP) arrays, and
fluorescence in situ hybridization (FISH). Results are then correlated with patient-outcome
variables and known risk factors, namely gender, age at diagnosis, tumor location
(infratentorial vs. supratentorial), tumor grade (differentiated vs anaplastic), and extent
of surgery as well as pathologic variables.