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Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review

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Abstract

Background

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare tumor, first described by the WHO Classification of Central Nervous System Tumors in 2016. The clinical course is variable. Most tumors have low-grade histological findings although some may have more aggressive features. The goal of this systematic review was to identify prognostic factors for poor overall survival (OS).

Material and methods

We performed a systematic review using three databases (PubMed, Google Scholar, and Embase) and the following search terms: diffuse leptomeningeal glioneuronal tumor, DLGNT, DLMGNT. Statistical analysis was performed using Statistica 13.3.

Results

We included 34 reports in our review comprising 63 patients, published from 2016 to 2022. The median OS was 19 months (range: 12–51 months). Using multivariable Cox survival analysis, we showed that Ki-67 ≥ 7%, age > 9 years, symptoms of elevated intracranial pressure (ICP) at admission, and the presence of contrast-enhancing intraparenchymal tumor are associated with poor OS. Receiver operating characteristic (ROC) analysis identified Ki-67 ≥ 7% as a significant predictor of poor OS.

Conclusions

Signs or symptoms of increased ICP with imaging findings of diffuse leptomeningeal enhancement should raise suspicion for DLGNT. In our systematic review, Ki-67 ≥ 7% was the most important prognostic factor for OS in DLGNT. The presence of intraparenchymal tumor with contrast enhancement was thought to represent disease progression and, together with patient age, was associated with poor OS.

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Data availability

Raw data are presented in supplementary materials.

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Funding

This work was financially supported by the Polish National Agency for Academic Exchange (the Walczak Programme).

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Conceptualization, K.W.; methodology, K.W., M.G.B., D.D.G.; analysis, K.W., M.G.B.; investigation, K.W., M.G.B., D.D.G.; writing—original draft preparation, K.W., M.G.B; writing—review and editing, K.W., D.D.G., J.R.C, M.L.L.; project administration, K.W.; supervision, K.W., M.L.L., J.R.C. All authors reviewed the manuscript.

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Correspondence to Karol Wiśniewski.

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Wiśniewski, K., Brandel, M.G., Gonda, D.D. et al. Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review. Childs Nerv Syst 38, 1663–1673 (2022). https://doi.org/10.1007/s00381-022-05600-w

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