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Published on: February 17, 2023
MR Imaging and Clinical Characteristics of Diffuse Glioneuronal Tumor with Oligodendroglioma-like Features and
M Benesch1, T Perwein2, G Apfaltrer3
1From the Division of Pediatric Hematology and Oncology (M.B., T.P.), Department of Pediatrics and Adolescent Medicine.
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.
Insights
Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC) presents distinct MR imaging features. Complete resection appears crucial for favorable long-term outcomes in DGONC patients.
Area of Science:
- Neuro-oncology
- Neuroradiology
- Molecular Pathology
Background:
- Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters (DGONC) is a newly identified molecularly defined CNS tumor.
- Understanding its imaging characteristics is essential for accurate diagnosis and management.
Purpose of the Study:
- To describe the MR imaging and clinical features of patients diagnosed with DGONC.
- To correlate imaging findings with clinical outcomes.
Main Methods:
- Review of preoperative MR images from 9 patients with DGONC.
- Analysis of tumor location, signal characteristics, mass effect, and relationship to vascular structures.
- Correlation of imaging findings with histopathology and clinical follow-up data.
Main Results:
- Tumors were superficial in the frontal/temporal lobes, sharply delineated, and showed low-to-intermediate aggressiveness.
- Homogeneous ground-glass aspect on T2-weighted and FLAIR images was common.
- At a median follow-up of 61 months, 6 patients achieved complete remission, and 2 had stable disease.
Conclusions:
- Radiomorphologic characteristics are highly predictive of DGONC, potentially differing from original histopathologic diagnoses.
- Complete surgical resection may be favorable for long-term disease control.
- Further evaluation in larger series is needed to assess nonsurgical treatment efficacy.
