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Understanding diffuse leptomeningeal glioneuronal tumors

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Abstract

Intoduction

Diffuse leptomeningeal glioneuronal tumors (DLGNTs) pose a rare and challenging entity within pediatric central nervous system neoplasms. Despite their rarity, DLGNTs exhibit complex clinical presentations and unique molecular characteristics, necessitating a deeper understanding of their diagnostic and therapeutic nuances.

Methods

This review synthesizes contemporary literature on DLGNT, encompassing epidemiology, clinical manifestations, pathological features, treatment strategies, prognostic markers, and future research directions. To compile the existing body of knowledge on DLGNT, a comprehensive search of relevant databases was conducted.

Results

DLGNT primarily affects pediatric populations but can manifest across all age groups. Its diagnosis is confounded by nonspecific clinical presentations and overlap** radiological features with other CNS neoplasms. Magnetic resonance imaging (MRI) serves as a cornerstone for DLGNT diagnosis, revealing characteristic leptomeningeal enhancement and intraparenchymal involvement. Histologically, DLGNT presents with low to moderate cellularity and exhibits molecular alterations in the MAPK/ERK signalling pathway. Optimal management of DLGNT necessitates a multidisciplinary approach encompassing surgical resection, chemotherapy, radiotherapy, and emerging targeted therapies directed against specific genetic alterations. Prognostication remains challenging, with factors such as age at diagnosis, histological subtypes, and genetic alterations influencing disease progression and treatment response. Long-term survival data are limited, underscoring the need for collaborative research efforts.

Conclusion

Advancements in molecular profiling, targeted therapies, and international collaborations hold promise for improving DLGNT outcomes. Harnessing the collective expertise of clinicians, researchers, and patient advocates, can advance the field of DLGNT research and optimize patient care paradigms.

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Data availability

No datasets were generated or analyzed during the current study.

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Authors and Affiliations

  1. Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, ONT, Canada

    Inci Yaman Ba**, Julie Bennett, Uri Tabori & Eric Bouffet

  2. Department of Pathology, The Hospital for Sick Children, Toronto, ONT, Canada

    Adrian Levine & Cynthia Hawkins

  3. Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, TN, USA

    Michael C. Dewan

  4. Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, Toronto, ONT, Canada

    Julie Bennett

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  1. Inci Yaman Ba**
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I. Y. B. and E. B. wrote the main manuscript and all authors reviewed the manuscript.

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Correspondence to Inci Yaman Ba** or Eric Bouffet.

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Ba**, I.Y., Levine, A., Dewan, M.C. et al. Understanding diffuse leptomeningeal glioneuronal tumors. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06432-6

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