Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Sep;38(9):1663-1673.
doi: 10.1007/s00381-022-05600-w. Epub 2022 Jul 22.

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

Karol Wiśniewski  1   2 , Michael G Brandel  3 , David D Gonda  3 , John R Crawford  4 , Michael L Levy  3
Affiliations

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

Karol Wiśniewski et al. Childs Nerv Syst. 2022 Sep.

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.

Keywords: Brain tumor; Diffuse leptomeningeal glioneuronal tumor (DLGNT); Predictors; Risk factors; Survival.

PubMed Disclaimer

References

    1. Louis DN, Perry A, Reifenberger G, Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler DO, Kleihues P, Ellison DW (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131:803–820. https://doi.org/10.1007/s00401-016-1545-1 - DOI - PubMed
    1. Rodriguez FJ, Perry A, Rosenblum MK, Krawitz S, Cohen KJ, Lin D, Mosier S, Lin MT, Eberhart CG, Burger PC (2012) Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity. Acta Neuropathol 124(5):627–641. https://doi.org/10.1007/s00401-012-1037-x - DOI - PubMed
    1. Mathews MS, Paré LS, Kuo JV, Kim RC (2009) Primary leptomeningeal oligodendrogliomatosis. J Neurooncol 94(2):275–278. https://doi.org/10.1007/s11060-009-9821-8 - DOI - PubMed - PMC
    1. Dodgshun AJ, SantaCruz N, Hwang J, Ramkissoon SH, Malkin H, Bergthold G, Manley P, Chi S, MacGregor D, Goumnerova L et al (2016) Disseminated glioneuronal tumors occurring in childhood: treatment outcomes and BRAF alterations including V600E mutation. J Neurooncol 128(2):293–302. https://doi.org/10.1007/s11060-016-2109-x - DOI - PubMed
    1. Rodriguez FJ, Schniederjan MJ, Nicolaides T, Tihan T, Burger PC, Perry A (2015) High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN). Acta Neuropathol 129:609–610. https://doi.org/10.1007/s00401-015-1400-9 - DOI - PubMed - PMC

Publication types

LinkOut - more resources