Multicentric low-grade glioma: A systematic review of a rare neuro-oncological disease

Abstract

Introduction

Multicentric and multifocal gliomas are rare and mainly described in high-grade gliomas, however, they have rarely been reported with LGG in about 2–10 % of all cases. This study aims to identify the reported multicentric low-grade gliomas (mLGGs) in literature and review their pathologies, management, and outcomes.

Methods

A systematic search using a pre-defined search strategy was conducted across three databases (PubMed, Cochrane Library, and Scopus). Following the PRISMA guidelines, relevant articles were selected. The data including demographic details, clinical presentations, lesion locations, pathology, neurosurgical interventions, extent of resection, adjuvant therapies, and survival outcomes were reported.

Results

We identified 36 patients across 17 studies. Presenting symptoms varied, with seizures (27.7 %) and headaches (22.2 %) being the most common. Typical imaging features involve hypo- to isotense signals on T1-weighted images and hyperintensity on T2-weighted images, with MR spectroscopy aiding in differentiation. Histological consistency across tumor sites was observed in 29 cases, with some variability in a few. Survival was 66.6 % among patients, and initial reports in the 1960s indicated high mortality due to intracranial pressure shifts. Adjuvant therapies included chemotherapy (14 patients) and radiotherapy (9 patients), though many cases lacked complete therapy data. Although chemotherapy and radiotherapy lacked a significant impact on progression-free survival, early, extensive resection remains advocated, with a mean progression-free survival of 30.14 months.

Conclusion

Most of the current evidence surrounding mLGG consists of case reports with few retrospective case series. Early, extensive resection appears to be the most effective approach for managing mLGG, while adjuvant therapies have limited impact on progression-free survival, highlighting the need for more comprehensive molecular profiling to guide treatment. Further research into standardized protocols for adjuvant therapies and long-term outcomes is essential to optimize survival and improve management of unresectable or recurrent cases.