TY  - JOUR
AU  - Hwang, Eugene I
AU  - Kool, Marcel
AU  - Burger, Peter C
AU  - Capper, David
AU  - Chavez, Lukas
AU  - Brabetz, Sebastian
AU  - Williams-Hughes, Chris
AU  - Billups, Catherine
AU  - Heier, Linda
AU  - Jaju, Alok
AU  - Michalski, Jeff
AU  - Li, Yimei
AU  - Leary, Sarah
AU  - Zhou, Tianni
AU  - von Deimling, Andreas
AU  - Jones, David
AU  - Fouladi, Maryam
AU  - Pollack, Ian F
AU  - Gajjar, Amar
AU  - Packer, Roger J
AU  - Pfister, Stefan
AU  - Olson, James M
TI  - Extensive Molecular and Clinical Heterogeneity in Patients With Histologically Diagnosed CNS-PNET Treated as a Single Entity: A Report From the Children's Oncology Group Randomized ACNS0332 Trial.
JO  - Journal of clinical oncology
VL  - 36
IS  - 34
SN  - 1527-7755
CY  - Alexandria, Va.
PB  - American Society of Clinical Oncology
M1  - DKFZ-2018-01724
SP  - 3388
PY  - 2018
AB  - Children with histologically diagnosed high-risk medulloblastoma, supratentorial primitive neuroectodermal tumor of the CNS (CNS-PNET), and pineoblastoma (PBL) have had poor survival despite intensive treatment. We included these patients in this Children's Oncology Group trial. Molecular profiling later revealed tumor heterogeneity that was not detectable at protocol inception. Enrollment of patients with CNS-PNET/PBL was subsequently discontinued, and outcomes for this part of the study are reported here.In this phase III, four-arm prospective trial, consenting children age 3-22 years with newly diagnosed CNS-PNET were randomly assigned (1:1) to receive carboplatin during radiation and/or adjuvant isotretinoin after standard intensive therapy. Primary outcome measure was event-free survival (EFS) in the intent-to-treat population. Molecular tumor classification was retrospectively completed using DNA methylation profiling.Eighty-five participants with institutionally diagnosed CNS-PNETs/PBLs were enrolled. Of 60 patients with sufficient tissue, 31 were nonpineal in location, of which 22 (71
LB  - PUB:(DE-HGF)16
C6  - pmid:30332335
DO  - DOI:10.1200/JCO.2017.76.4720
UR  - https://inrepo02.dkfz.de/record/141201
ER  -