TY - JOUR T1 - Imaging Characteristics of CNS Neuroblastoma-<em>FOXR2</em>: A Retrospective and Multi-Institutional Description of 25 Cases JF - American Journal of Neuroradiology JO - Am. J. Neuroradiol. DO - 10.3174/ajnr.A7644 AU - A. Tietze AU - K. Mankad AU - M.H. Lequin AU - L. Ivarsson AU - D. Mirsky AU - A. Jaju AU - M. Kool AU - K.V. Hoff AU - B. Bison AU - U. Löbel Y1 - 2022/09/22 UR - http://www.ajnr.org/content/early/2022/09/22/ajnr.A7644.abstract N2 - BACKGROUND AND PURPOSE: The 5th edition of the World Health Organization Classification of CNS tumors defines the CNS neuroblastoma FOXR2 in the group of embryonal tumors. Published clinical outcomes tend to suggest a favorable outcome after resection, craniospinal irradiation, and chemotherapy. This multicenter study aimed to describe imaging features of CNS neuroblastoma-FOXR2, which have been poorly characterized thus far.MATERIALS AND METHODS: On the basis of a previously published cohort of tumors molecularly classified as CNS neuroblastoma-FOXR2, patients with available imaging data were identified. The imaging features on preoperative MR imaging and CT data were recorded by 8 experienced pediatric neuroradiologists in consensus review meetings.RESULTS: Twenty-five patients were evaluated (13 girls; median age, 4.5 years). The tumors were often large (mean, 115 [ SD, 83] mL), showed no (24%) or limited (60%) perilesional edema, demonstrated heterogeneous enhancement, were often calcified and/or hemorrhagic (52%), were always T2WI-hyperintense to GM, and commonly had cystic and/or necrotic components (96%). The mean ADC values were low (687.8 [SD 136.3] × 10−6 mm2/s). The tumors were always supratentorial. Metastases were infrequent (20%) and, when present, were of nodular appearance and leptomeningeal.CONCLUSIONS: In our cohort, CNS neuroblastoma FOXR2 tumors showed imaging features suggesting high-grade malignancy and, at the same time, showed characteristics of less aggressive behavior. There are important differential diagnoses, but the results of this study may assist in considering this diagnosis preoperatively.ATRTatypical teratoid/rhabdoid tumorsCNS NB-FOXR2CNS neuroblastoma FOXR2-activatedCNS-PNETprimitive neuroectodermal tumors of the CNSETMRembryonal tumor with multilayered rosettesITDinternal tandem duplicationWHOWorld Health Organization ER -