A Tietze1, K Mankad2, M H Lequin3, L Ivarsson4, D Mirsky5, A Jaju6, M Kool7,8,9, K V Hoff10, B Bison11, U Löbel2. 1. From the Institute of Neuroradiology (A.T.) anna.tietze@charite.de. 2. Department of Radiology (K.M., U.L.), Great Ormond Street Hospital, London, UK. 3. Department of Radiology (M.H.L.), University Medical Center Utrecht, Utrecht, the Netherlands. 4. Department of Pediatric Radiology (L.I.), Queen Silvias Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden. 5. Department of Pediatric Radiology and Imaging (D.M.), Children's Hospital Colorado, Denver, Colorado. 6. Department of Medical Imaging (A.J.), Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois. 7. Hopp Children's Cancer Center (M.K.), Heidelberg, Germany. 8. Division of Pediatric Neurooncology (M.K.), German Cancer Research Center and German Cancer Consortium, Heidelberg, Germany. 9. Princess Máxima Center for Pediatric Oncology (M.K.), Utrecht, the Netherlands. 10. Department of Pediatric Oncology and Hematology (K.V.H.), Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany. 11. Department of Neuroradiology (B.B.), University Hospital Augsburg, Ausburg, Germany.
Abstract
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.
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.
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