MR Imaging and Clinical Characteristics of Diffuse Glioneuronal Tumor with Oligodendroglioma-like Features and Nuclear Clusters
Benesch, M; Perwein, T; Apfaltrer, G; Langer, T; Neumann, A; Brecht, I B; Schuhmann, M U; Cario, H; Frühwald, M C; Vollert, K; van Buiren, M; Deng, M Y; Seitz, A; Haberler, C; Mynarek, M; Kramm, C; Sahm, F; Robe, P A; Dankbaar, J W; Hoff, K V; Warmuth-Metz, M; Bison, B
(2022) AJNR. American journal of neuroradiology, volume 43, issue 10, pp. 1523 - 1529
(Article)
Abstract
BACKGROUND AND PURPOSE: Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC) is a new, molecularly defined glioneuronal CNS tumor type. The objective of the present study was to describe MR imaging and clinical characteristics of patients with DGONC. MATERIALS AND METHODS: Preoperative MR images of 9 patients with
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DGONC (median age at diagnosis, 9.9 years; range, 4.2-21.8 years) were reviewed. RESULTS: All tumors were located superficially in the frontal/temporal lobes and sharply delineated, displaying little mass effect. Near the circle of Willis, the tumors encompassed the arteries. All except one demonstrated characteristics of low-to-intermediate aggressiveness with high-to-intermediate T2WI and ADC signals and bone remodeling. Most tumors (n ¼ 7) showed a homogeneous ground-glass aspect on T2-weighted and FLAIR images. On the basis of the original histopathologic diagnosis, 6 patients received postsurgical chemo-/radiotherapy, 2 were irradiated after surgery, and 1 patient underwent tumor resection only. At a median follow-up of 61 months (range, 10-154 months), 6 patients were alive in a first complete remission and 2 with stable disease 10 and 21 months after diagnosis. The only patient with progressive disease was lost to follow-up. Five-year overall and event-free survival was 100% and 86613%, respectively. CONCLUSIONS: This case series presents radiomorphologic characteristics highly predictive of DGONC that contrast with the typical aspects of the original histopathologic diagnoses. This presentation underlines the definition of DGONC as a separate entity, from a clinical perspective. Complete resection may be favorable for long-term disease control in patients with DGONC. The efficacy of nonsurgical treatment modalities should be evaluated in larger series.
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Keywords: Brain Neoplasms/diagnostic imaging, Central Nervous System Neoplasms/pathology, Child, Glioma/pathology, Humans, Magnetic Resonance Imaging, Neoplasms, Neuroepithelial, Oligodendroglioma/diagnostic imaging, Clinical Neurology, Radiology Nuclear Medicine and imaging, Journal Article
ISSN: 0195-6108
Publisher: American Society of Neuroradiology
Note: Funding Information: Martin Benesch and Thomas Perwein share first authorship. The Neuroradiologic Reference Center of the Brain Tumor Network Consortium, University Würzburg (grant Nos. 2001.05, 2003.09, 2005.07, 2008.07, 2011.02, 2013.21, 2014.15, 2017.07, 2018.02 to M. Warmuth-Metz, B. Bison) were supported by the German Children’s Cancer Foundation (Deutsche Kinderkrebsstiftung). M. Benesch and T. Perwein receive support from the Styrian Childhood Cancer Foundation (Steirische Kinderkrebshilfe). Please address correspondence to Thomas Perwein, MD, Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Auenbruggerplatz 34/2, 8036 Graz, Austria; e-mail: thomas.perwein@medunigraz.at Indicates article with online supplemental data. http://dx.doi.org/10.3174/ajnr.A7647 Publisher Copyright: © 2022 American Society of Neuroradiology. All rights reserved.
(Peer reviewed)