Myxoid glioneuronal tumor of the septum pellucidum in pediatric patients: a case report and comprehensive review of the literature

Myxoid glioneuronal tumor of the septum pellucidum is an uncommon clinical entity, previously referred to dysembryoplastic neuroepithelial tumor located in the septum pellucidum.

This study was conducted following PRISMA guidelines. A comprehensive literature search was performed in the PubMed/MEDLINE, Web of Science, and Scopus databases. In addition to the literature review, we report a rare case of a 10-year-old male patient with septal myxoid glioneuronal tumor who underwent endoscopic tumor resection as an exemplary case.

A total of 10 pediatric patients from 7 published records were included in this review. An analysis encompassing 11 patients, including the present patient, was performed. The mean age of the patients was 11.36 ± 3.35 years, with a male predominance. The most common presenting symptoms were headache (50%), and seizures (37.5%). Nine patients had microsurgical tumor resection, while 2 patients underwent endoscopic tumor resection. Seven patients underwent gross total or near total resection, while 4 had subtotal resection, and the prognosis for all patients was good.

Myxoid glioneuronal tumor of the septum pellucidum represents a rare and relatively benign pathology with typical localization and histopathological features. Surgical resection is the primary treatment modality, aiming for maximal safe resection while preserving neurological function. However, complete resection may not always be achievable due to the infiltrative nature of these tumors and their proximity to critical structures. Generally, the prognosis for these tumors is favorable.