Diffuse Leptomeningeal Glioneuronal Tumor: A Unique Leptomeningeal Tumor Entity

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is an entity introduced in 2016 World Health Organization classification of tumors of the central nervous system. The tumor occurs very rarely. Due to the lack of specific clinical and radiological features, biopsy is necessary to be performed and histological and immunohistochemical testing is essential to reach the diagnosis. A 4.5-year-old girl presented with a history of headache, vomiting, and right eye convergent squint. Imaging revealed multiple enhancing lesions located supra- and infratentorially and intramedullary. Histopathological examination demonstrated diffused growth of neoplastic cells. Molecular testing revealed KIAA1549-BRAF fusion and the diagnosis of DLGNT was stated.

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“…DLGNT is often diagnosed as Whipple's disease, 6 tuberculous meningitis, 8,17 or leptomeningeal gliomatosis.…”

Section: Discussionmentioning

confidence: 99%

“…DLGNT is often diagnosed as Whipple's disease, 6 tuberculous meningitis, 8,17 or leptomeningeal gliomatosis. 9,18 Our patient initially was suspected to have neurosarcoidosis.…”

Section: Chen Et Almentioning

confidence: 99%

Diffuse Leptomeningeal Glioneuronal Tumor in a 4.5-year-old Girl: A Case Report and Review of the Literature

Gabryś

Kuzaj

Pawełczak

et al. 2020

Journal of Pediatric Neurology

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“…The authors need to be commended on detailing the updated analysis of all the cases of this newly described disease in the literature. We managed a child with the same disease, and radiology colleagues at our institute published it last year [3]. However, there are some points in the authors' report which need further clarification in terms of management of the case.…”

Section: Dear Editormentioning

confidence: 99%

Letter to “Diffuse leptomeningeal glioneuronal tumour (DLGNT) with hydrocephalus as an initial symptom: a case-based update”

et al. 2020

Self Cite

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“…RGNT (39)(40)(41), PGNT (42,43) and GNTNI (44) were added to the WHO classification in 2007 (1). In 2016, the classification added diffuse leptomeningeal glioneuronal tumors (DLGNT) (1,2,(45)(46)(47)(48)(49)(50)(51) where the number of published case reports, pathological studies and reviews has grown since (Figure 1) (35).…”

Section: Pathological Molecular and Imaging Featuresmentioning

confidence: 99%

“…Tumors with a high Ki-67 index (≥5) are more likely to exhibit perilesional edema and ring enhancement on magnetic resonance imaging (MRI) (10). DLGNT was added to the WHO classification in 2016 and publications have increased since (1,2,(45)(46)(47)(48)(49)(50)55). In DLGNT, MRI is generally consistent with diffuse leptomeningeal enhancement predominantly and multiple cystic-solid lesions along the neural axis (49,59,87) but may present more atypically as well (45,88).…”

Section: Imaging Featuresmentioning

confidence: 99%

Glioneuronal Tumors: Insights into a Rare Tumor Entity

Krauze

2021

Gliomas

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Glioneuronal tumors are a group of rare neoplasms made up of neural and glial components in heterogenous proportions, generally exhibiting WHO grade I clinical behavior. These tumors affect infants, children and young adults, but are also described in adults and the elderly. They are strongly associated with seizures. Tumor subtypes described under the umbrella of glioneuronal tumors are actively evolving but to date comprise central, extraventricular and lipo-neurocytoma, desmoplastic infantile astrocytoma and ganglioglioma, diffuse leptomeningeal glioneuronal tumor, dysembryoplastic neuroepithelial tumor, papillary glioneuronal tumor, rosette-forming glioneuronal tumor of the fourth ventricle, rosetted glioneuronal tumor with neuropil-like islands, gangliocytoma, ganglioglioma, anaplastic ganglioglioma and paraganglioma. They vary in radiographic appearance, with some exhibiting large heterogenous solid/cystic masses. With large scale genetic and molecular analyses ongoing, classification continues to evolve. Seizure management and surgical resection represent the cornerstones of management, with the use of systemic agents and radiation lacking conclusive results. Optimal management requires multidisciplinary discussion including

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Diffuse Leptomeningeal Glioneuronal Tumor: A Unique Leptomeningeal Tumor Entity

Cited by 28 publications

References 11 publications

Diffuse Leptomeningeal Glioneuronal Tumor in a 4.5-year-old Girl: A Case Report and Review of the Literature

Diffuse Leptomeningeal Glioneuronal Tumor in a 4.5-year-old Girl: A Case Report and Review of the Literature

Letter to “Diffuse leptomeningeal glioneuronal tumour (DLGNT) with hydrocephalus as an initial symptom: a case-based update”

Glioneuronal Tumors: Insights into a Rare Tumor Entity

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