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Background: Recently recognized as a distinct entity, a myxoid glioneuronal tumor (MGNT) is a rare, low-grade central nervous system tumor. MGNTs are commonly located at the septum pellucidum or in the third ventricle, increasing the likelihood of tumor or treatment-related damage to adjacent structures critical for memory, such as the fornix. Though there have been a handful of case reports of neurosurgical and oncological outcomes of MGNTs, memory outcomes following resection of MGNTs adjacent to the fornix have not been previously reported.
Methods: We present a case of a high functioning female for whom an MRI revealed an incidental finding of an intraventricular tumor adjacent to the fornix bilaterally. The patient underwent resection of the tumor followed by MRI surveillance without additional oncologic intervention. Due to reported cognitive problems, the patient was referred for serial neuropsychological evaluations.
Results: Post-operative MRI following resection revealed cytotoxic edema followed by selective, progressive atrophy of the bilateral anterior fornices. Post-surgically, the patient developed an isolated verbal memory impairment, which persisted one-year post resection with minimal improvement. The memory impairment impacted the patient's everyday functioning, including the ability to work in a cognitively demanding job.
Conclusion: This unique case demonstrates the critical role of the bilateral fornix in verbal memory and underscores the importance of a careful risk/benefit analysis when considering neurosurgical intervention to MGNTs and other intracranial lesions adjacent to this structure during neurosurgical planning.
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http://dx.doi.org/10.3389/fonc.2023.1263556 | DOI Listing |
Pediatr Dev Pathol
March 2025
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.
Childs Nerv Syst
October 2024
Division of Neuroradiology, Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, 170 Elizabeth Street, Toronto, ON, M5G 1E8, Canada.
Glioneuronal tumors (GNTs) are an expanding group of primary CNS neoplasms, commonly affecting children, adolescents and young adults. Most GNTs are relatively indolent, low-grade, WHO grade I lesions. In the pediatric age group, GNTs have their epicenter in the cerebral cortex and present with seizures.
View Article and Find Full Text PDFBrain Commun
May 2024
Department of Pathology, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China.
Current histological classification of low-grade glioneuronal tumours does not adequately represent their underlying biology. The neural lineage(s) and differentiation stage(s) involved and the cell state(s) affected by the recurrent genomic alterations are unclear. Here, we describe dysregulated oligodendrocyte lineage developmental programmes in three low-grade glioneuronal tumour subtypes.
View Article and Find Full Text PDFBJR Case Rep
May 2024
Department of Radiology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310000, China.