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RT Journal Article
SR Electronic
T1 MR Imaging and Clinical Characteristics of Diffuse Glioneuronal Tumor with Oligodendroglioma-like Features and Nuclear Clusters
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1523
OP 1529
DO 10.3174/ajnr.A7647
VO 43
IS 10
A1 Benesch, M.
A1 Perwein, T.
A1 Apfaltrer, G.
A1 Langer, T.
A1 Neumann, A.
A1 Brecht, I.B.
A1 Schuhmann, M.U.
A1 Cario, H.
A1 Frühwald, M.C.
A1 Vollert, K.
A1 van Buiren, M.
A1 Deng, M.Y.
A1 Seitz, A.
A1 Haberler, C.
A1 Mynarek, M.
A1 Kramm, C.
A1 Sahm, F.
A1 Robe, P.A.
A1 Dankbaar, J.W.
A1 Hoff, K.V.
A1 Warmuth-Metz, M.
A1 Bison, B.
YR 2022
UL http://www.ajnr.org/content/43/10/1523.abstract
AB 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 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 = 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 86±13%, 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.DNETdysembryoplastic neuroepithelial tumorHITBrain Tumor NetworkDGONCdiffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clustersPNETprimitive neuroectodermal tumor