RT Journal Article
SR Electronic
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.
FD American Society of Neuroradiology
DO 10.3174/ajnr.A7644
A1 A. Tietze
A1 K. Mankad
A1 M.H. Lequin
A1 L. Ivarsson
A1 D. Mirsky
A1 A. Jaju
A1 M. Kool
A1 K.V. Hoff
A1 B. Bison
A1 U. Löbel
YR 2022
UL http://www.ajnr.org/content/early/2022/09/22/ajnr.A7644.abstract
AB 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