Object: In this study the authors review the outcomes in pediatric patients who presented with seizures and underwent resection of dysembryoplastic neuroepithelial tumors (DNETs). The authors focus on the diagnostic evaluation and surgical techniques that facilitate gross-total tumor resection and subsequent freedom from seizures.
Methods: Eighteen patients between the ages of 1 month and 13 years who presented with seizures underwent resection of DNETs between January 1992 and December 2004. Preoperative evaluation included magnetic resonance (MR) imaging and interictal scalp electroencephalography (EEG) in all patients, functional MR imaging in eight patients, video monitoring with ictal scalp EEG in 12 patients, interictal single-photon emission computerized tomography (SPECT) scanning in one patient, and ictal SPECT scanning in two patients. Thirteen patients underwent one-stage procedures, whereas five underwent two-stage procedures (implantation of monitoring electrodes followed by tumor resection), either for functional language mapping (three patients) or due to inconclusive preoperative data (two patients). Intraoperative electrocorticography (ECoG) was performed in 17 patients and led to resection of the cerebral cortex beyond the tumor margins in 10 of them. According to operative reports, gross-total tumor resections were achieved in all patients, but one child had minimal residual tumor on postoperative MR images that has remained stable. The only surgical complication was a transient third cranial nerve palsy. Over a median follow-up duration of 1.6 years, all patients are seizure free and without radiographically detected tumor recurrence.
Conclusions: Dysembryoplastic neuroepithelial tumors are a highly treatable cause of epilepsy in children. Excellent rates of complete tumor resection and seizure control with minimal morbidity can be attained using intraoperative ECoG and two-stage surgical procedures when appropriate.
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Pediatr Dev Pathol
December 2024
Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Tumors are increasingly defined by molecular alterations but approach to cases with discordant histologic and molecular features is unclear. Myxoid glioneuronal tumor (MGNT), histologically similar to dysembryoplastic neuroepithelial tumor (DNET), is characterized by dinucleotide mutations in gene (K385L or K385I). Here, we report K385L mutation in a neonatal high-grade glioma.
View Article and Find Full Text PDFChilds Nerv Syst
November 2024
Department of Pathology, Cukurova University School of Medicine, Adana, Turkey.
Introduction: Myxoid glioneuronal tumor of the septum pellucidum is an uncommon clinical entity, previously referred to dysembryoplastic neuroepithelial tumor located in the septum pellucidum.
Methods: This study was conducted following PRISMA guidelines. A comprehensive literature search was performed in the PubMed/MEDLINE, Web of Science, and Scopus databases.
Neuropathology
November 2024
Department of Neurosurgery and Neurooncology, 1st Medical Faculty, Charles University, Military University Hospital Prague, Prague, Czech Republic.
Rosette-forming glioneuronal tumors (RGNTs) with FGFR1 tyrosine kinase domain internal tandem duplication (FGFR1 ITD) is exceedingly rare, with only a few cases reported in the literature. Hereby we present a case of a tumor with RGNT morphology occurring in area of septum pellucidum of 43-year-old male. The tumor showed FGFR1 ITD, no PIK3CA, PIK3R1 or NF1 alterations and inconclusive methylation profile with match for class of "low-grade glial/glioneuronal/neuroepithelial tumors".
View Article and Find Full Text PDFRadiol Case Rep
January 2025
Radiology Department, Faculty of Medicine and Pharmacy, Specialities hospital, Mohammed V University, Rabat, Morocco.
Dysembryoplastic neuroepithelial tumors (DNETs) are benign cortical tumors frequently associated with medically incurable focal epilepsy. These tumors occur most commonly in children. Given the fact that they rarely become malignant, the long-term prognosis in terms of mortality is excellent, however its similar appearance with other tumors of the central nervous system increases the potential for misdiagnosis and the risk of a pejorative clinical evolution.
View Article and Find Full Text PDFSeizure
November 2024
Department of Pediatrics and Adolescent Medicine, MedUniWien, Vienna, Austria.
Purpose: Imaging and resection strategies for pediatric gangliogliomas (GG) and dysembryoplastic neuroepitheliomas (DNET) presenting with epilepsy were retrospectively analyzed in a consecutive institutional series of surgically treated patients.
Methods: Twenty-two children (median 8 years, 3-18 years) presented with seizures for 30 months median (14-55.2 months) due to a histologically verified GG/DNET.
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