MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children’s Oncology Group ACNS0332 Trial

Jaju A, Hwang EI, Kool M, Capper D, Chavez L, Brabetz S, Billups C, Li Y, Fouladi M, Packer RJ, Pfister SM, Olson JM, Heier LA. MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children’s Oncology Group ACNS0332 Trial. AJNR Am J Neuroradiol. 2019 Nov;40(11):1796-1803. doi: 10.3174/ajnr.A6253. Epub 2019 Oct 10. Erratum in: AJNR Am J Neuroradiol. 2020 Feb;41(2):E9. doi: 10.3174/ajnr.A6406. PMID: 31601576; PMCID: PMC6856443.

Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors has revealed a number of distinct entities and has led to the term “CNS-primitive neuroectodermal tumors” being removed from the 2016 World Health Organization classification. The purpose of this study was to describe the MR imaging findings of histologically diagnosed primitive neuroectodermal tumors and pineoblastomas and correlate them with molecular diagnoses and outcomes. Histologically diagnosed primitive neuroectodermal tumors and pineoblastomas were enrolled in this Children’s Oncology Group Phase III trial, and molecular classification was retrospectively completed using DNA methylation profiling. MR imaging features were systematically studied and correlated with molecular diagnoses and survival.