Imaging Characteristics of CNS Neuroblastoma- FOXR2 : A Retrospective and Multi-Institutional Description of 25 Cases

• Published in: American journal of neuroradiology : AJNR Vol. 43; no. 10; pp. 1476 - 1480
• Main Authors: Tietze, A, Mankad, K, Lequin, M H, Ivarsson, L, Mirsky, D, Jaju, A, Kool, M, Hoff, K V, Bison, B, Löbel, U
• Format: Journal Article
• Language: English
• Published: United States American Society of Neuroradiology 01.10.2022
• Subjects: Central Nervous System Neoplasms - diagnostic imaging; Child; Child, Preschool; Female; Forkhead Transcription Factors; Humans; Magnetic Resonance Imaging; Male; Neoplasms, Germ Cell and Embryonal; Neuroblastoma; Pediatrics; Retrospective Studies
• ISSN: 0195-6108; 1936-959X
• DOI: 10.3174/ajnr.A7644

The 5th edition of the World Health Organization Classification of CNS tumors defines the CNS neuroblastoma 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- , which have been poorly characterized thus far. On the basis of a previously published cohort of tumors molecularly classified as CNS neuroblastoma- , 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. 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 mm /s). The tumors were always supratentorial. Metastases were infrequent (20%) and, when present, were of nodular appearance and leptomeningeal. In our cohort, CNS neuroblastoma 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.