biostudies-literatureUnknownHwang EINCI NIH HHS<h4>Purpose</h4>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.<h4>Patients and methods</h4>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.Journal of clinical oncology : official journal of the American Society of Clinical OncologyJCO2017764720https://www.ebi.ac.uk/biostudies/studies/S-EPMC9153369biostudies-literatureExtensive 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.PMC9153369U10 CA098543U10 CA180886U10 CA180899R01 CA114567U10 CA098413Williams-Hughes CHwang EIKool MBurger PCBillups CPacker RJvon Deimling AMichalski JOlson JMZhou TBrabetz SJones DTWCapper DJaju APfister SMChavez LFouladi MLi YHeier LGajjar APollack IFLeary SfalseExtensive 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.<h4>Purpose</h4>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.<h4>Patients and methods</h4>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.2018-01-01T00:00:00Z2018 Oct2025-04-05T08:59:32.244Z2024-11-09T21:12:42.836ZS-EPMC91533693033233510.1200/JCO.2017.76.472010.1200/jco.2017.76.4720