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Clinical and molecular characteristics of pediatric low-grade glioma complicated with ventriculo-peritoneal shunt related ascites

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Abstract

Introduction

Ventriculo-peritoneal shunt (VPS) related ascites is a rare complication of pediatric low grade gliomas (pLGG). Physiopathology of this complication is not fully understood and there is paucity of data regarding the molecular profile of pLGG gliomas complicating with ascites and the optimal management of this unusual event.

Methods

International multi-institutional retrospective analysis of patients diagnosed with BRAF altered pLGG and ascites arising as a complication of VPS. Demographics, tumor characteristics, therapeutic approaches and outcomes were recorded.

Results

Nineteen patients were identified. Median age at diagnosis was 14 months (R: 2–144). Most patients (17; 89.4%) presented with lesions involving the optic pathway. Mean tumor standard volume was 34.8 cm2 (R: 12.5–85.4). Pilocytic Astrocytoma was the most frequent histological diagnosis (14;7 3.7%). Eight (42.1%) tumors harbored BRAF V600-E mutation and seven (36.8%) KIAA1549 fusion. The onset of ascites was documented at a median time of 5 months following VPS insertion. Four (21%) patients were managed with paracentesis only, 7(36.8%) required both paracentesis and shunt diversion, 7(36.8%) required only a shunt diversion and 1 (5.2%) patient was managed conservatively. Chemotherapy regimen was changed in 10 patients following ascites. Eight patients received targeted therapy (4 dabrafenib/4 trametinib) and 5 were radiated. There were eleven survivors with a median OS of 69 months (R: 3–144).

Conclusions

Ascites is an early feature in the clinical course of young patients with midline BRAF altered pLGG, with high mortality rate observed in our cohort. The hypothesis of ascites as an adverse prognostic factor in pLGG warrants further prospective research.

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Data availability

All data analyzed and generated during this study are included in this published article.

Code availability

Not applicable.

Importance of the study

Our cooperative multi institutional work describes to our best knowledge the largest case series of a extremely rare complication in the course of pediatric Low Grade Glioma (pLGG): Ascites related to ventriculo-peritoneal shunt; and provides with a molecular characterization of this subset of gliomas which are enriched in BRAF V600E mutation. Our hypothesis of ascites as an adverse prognostic factor in pLGG warrants further prospective research.

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Funding

The authors have not disclosed any funding.

Author information

Author notes

  1. Álvaro Lassaletta and Eric Bouffet denotes co-senior authorship.

Authors and Affiliations

  1. Department of Pediatric Oncology, Hospital Infantil Virgen del Rocío, Avda/Manuel Siurot S/N CP, 41003, Seville, Spain

    Palma Solano-Páez & Eduardo Quiroga

  2. Division of Neuro-Oncology, The Hospital for Sick Children, Toronto, ON, Canada

    Adriana Fonseca & Eric Bouffet

  3. Department of Hematology/Oncology, Hospital de Pediatría SAMIC Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina

    Lorena V. Baroni & Candela Freytes

  4. Department of Pediatrics, Hematology/Oncology, King Hussein Cancer Center, Amman, Jordan

    Nisreen Amayiri

  5. Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain

    Marta P. Somarriba

  6. Department of Pediatric Neurosurgery, Hospital Infantil Virgen del Rocío, Seville, Spain

    Mónica Rivero & Javier Márquez

  7. Department of Pediatric Hematology and Oncology, Hospital Universitario Niño Jesús, Madrid, Spain

    Álvaro Lassaletta

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  1. Palma Solano-Páez
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by PS-P. The first draft of the manuscript was written by PS-P and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript. This manuscript is not under consideration at any other publication.

Corresponding author

Correspondence to Palma Solano-Páez.

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Conflict of interest

Authors disclose non-financial funding directly or indirectly related to the work submitted for publication.

Ethical approval

This retrospective chart review study involving human participants was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The Human Investigation Committee (IRB) of the participant Institutions approved this study.

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Solano-Páez, P., Fonseca, A., Baroni, L.V. et al. Clinical and molecular characteristics of pediatric low-grade glioma complicated with ventriculo-peritoneal shunt related ascites. J Neurooncol 157, 147–156 (2022). https://doi.org/10.1007/s11060-022-03956-2

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  • DOI: https://doi.org/10.1007/s11060-022-03956-2

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