IMG-05 Imaging characteristics of CNS NB-FOXR2 – a retrospective and MULTI-INSTITUTIONAL DESCRIPTION OF 25 CASES

BACKGROUND: The CNS neuroblastoma with forkhead box R2 (CNS NB-FOXR2) activation is one of the new embryonal tumor entities in the WHO CNS tumor classification 2021. The data currently available suggest a comparably favourable outcome after craniospinal irradiation and chemotherapy. The entity is poorly characterized on imaging. We therefore aimed to describe imaging features in a multi-center patient cohort. MATERIAL AND METHODS: Molecularly and histologically confirmed CNS NB-FOXR2 tumors were retrospectively collected at six pediatric oncology centers. The imaging features on preoperative MRI and CT data were recorded by 8 experienced pediatric (neuro)radiologists in consensus. RESULTS: Twenty-five patients were identified (13 females). The majority of tumors were large at presentation (mean 115 ± SD 83 ml), showed mostly none or little perifocal edema, enhanced only partly, were often calcified and/or hemorrhagic, were always T2w hyperintense to gray matter, and often had cystic and/or necrotic components. The min. mean ADC values were relatively low (mean 687.8 ± SD 136.3 in 10-6mm2/s). The tumors originated never infratentorial and were never centered in the midline. Metastases were rare. CONCLUSIONS: For the first time, imaging characteristics of the recently described CNS NB-FOXR2 are described in a multi-center cohort. The results of this study may assist in diagnosing these tumors preoperatively. Collaborative pooling of cases is important to gain insight into typical imaging features of rare tumors, especially in view of the increasing molecular-driven subtyping.