ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2019
Unusual radiological and histological presentation of a diffuse leptomeningeal glioneuronal tumor (DLGNT) in a 13-year-old girl.
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 2 of them syntheses that pooled it.
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13 citing papers in PubMed, 2 syntheses or guidelines pooled it, 30 citations in OpenAlex.
- Prognostic factors in diffuse leptomeningeal glioneuronal tumor (DLGNT): a systematic review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2022Pooled it
- Clinical progression, pathological characteristics, and radiological findings in children with diffuse leptomeningeal glioneuronal tumors: A systematic review.Frontiers in oncology · 2022Pooled it
- Diffuse Leptomeningeal Glioneuronal Tumor: A Systematic Review Highlighting Molecular Heterogeneity and Survival Outcome.Cancers · 2026Review
- A case of a (not so) diffuse leptomeningeal glioneuronal tumor with an unusual clinical history.Journal of neuropathology and experimental neurology · 2025Article
- Spontaneous recovery of postsurgical progressive cervical spine kyphosis following intramedullary spinal cord tumor resection in a 4-year-old boy: illustrative case.Journal of neurosurgery. Case lessons · 2024Article
- Diffuse leptomeningeal glioneuronal tumor without KIAA1549-BRAF fusion and 1p detection: a case report and review of literature.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2022Review
- Atypical and aggressive diffuse leptomeningeal glioneuronal tumor in a young adult: A case report and review of the literature.Surgical neurology international · 2022Article
- A case of diffuse leptomeningeal glioneuronal tumor in a 10-year-old boy: First report from Iran.Clinical case reports · 2021Article
- Diffuse leptomeningeal glioneuronal tumour (DLGNT) in children: the emerging role of genomic analysis.Acta neuropathologica communications · 2021Article
- Diffuse leptomeningeal glioneuronal tumor: a double misnomer? A report of two cases.Acta neuropathologica communications · 2020Article
- Diffuse leptomeningeal glioneuronal tumour (DLGNT) with hydrocephalus as an initial symptom: a case-based update.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020Review
- Diffuse leptomeningeal glioneuronal tumor in a child masquerading as an intramedullary spinal pilocytic astrocytoma.Neuro-oncology advancesArticle
- Clinical and molecular features of disseminated pediatric low-grade glioma and glioneuronal tumors: a systematic review and survival analysis.Neuro-oncology advancesArticle
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6 authors at 1 institution in 1 country.
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Abstract
Diffuse leptomeningeal glioneuronal tumors (DLGNTs) are newly recognized as an entity in the 2016 revision of the WHO Classification of tumors of the central nervous system. They typically present as diffuse leptomeningeal infiltrates along the neuraxis with focal and superficial involvement of the parenchyma. Here, we report a DLGNT with unusual radiological and histological features. A 13-year-old girl presented with scoliosis and back pain. Magnetic resonance imaging demonstrated a syrinx from C2 to T11 and an intramedullary mass from T6 to T9-10. No leptomeningeal involvement was recognized. Histological examination of the gross total resection specimen revealed a low-grade neuroepithelial neoplasm predominantly infiltrating the spinal cord and only focally involving the leptomeninges. Chromosome microarray identified co-deletion of the short arm of chromosome 1 and the long arm of chromosome 19 as well as fusion of the KIAA1549 and BRAF genes. Next-generation sequencing demonstrated wild-type alleles at the mutational hotspots of IDH1 (R132) and IDH2 (R140 and R172). In contrast to most reported DLGNTs, the tumor described in this manuscript was characterized by a predominant parenchymal component and only minor leptomeningeal involvement both radiographically and histologically. Our case, therefore, expands the spectrum of radiological and histopathological features of this new entity. It also highlights the critical role of molecular genetic testing in establishing the diagnosis of DLGNT in unusual cases.
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