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

Chen, Wenlin; Kong, Ziren; Ji, Fu; Zhao, Dachun; Wang, Renzhi; Ma, Wenbin; Wang, Yu

doi:10.1007/s00381-019-04481-w

Cited by 28 publications

(31 citation statements)

References 25 publications

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“…3,[5][6][7][8]10 However, in other cases atypical tumor cells with a high mitotic rate or vascular proliferation were noticed. 4,9,[11][12][13][14] The perivascular pseudo-rosette-like arrangements can also be detected. 11 Cases with atypical findings have been also reported.…”

Section: Discussionmentioning

confidence: 96%

“…[5][6][7][8][9][10][11][12][13] Several cases in adults have been reported. 3,8,12 Most common symptoms include nausea and vomiting, 2-4,12-14 headache, [2][3][4][5][6][7]9,11,12,14 seizures, 11,12,15 limbs weakness, 2,4,7,9,11 loss of vision, 9,11 and altered mental state (confusion). 2,8,9 Our patient presented with headache, vomiting, and right eye convergent strabismus.…”

Section: Discussionmentioning

confidence: 99%

“…DLGNTs are typically positive for S100, Olig2, GFAP, and synaptophysin expression, 3,4,6,7,9 but immunoreactivity can differ between cases. The tumor is becoming increasingly recognized as having BRAF and KIAA1549 genes fusions, 1p deletions 2,6,9,13 and rarely 1p/19q codeletion 7,11 but a significant part of DLGNTs are negative for these markers.…”

Section: Discussionmentioning

confidence: 99%

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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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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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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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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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“…Oligodendroglial-like cells can appear in many central nervous system tumors, such as dysembryoplastic neuroepithelial tumor (DNT) [26], rosette-forming glioneuronal tumor (RGNT) [27], papillary glioneuronal tumor [28], diffuse leptomeningeal glioneuronal tumor [29], and gangliogliomas (GGs) [30]. There is only one previous report by Lehman NL and Hattab EM that found scattered oligodendroglial-like cells between astrocytoma cells, which is different from our patient [31].…”

Section: Discussionmentioning

confidence: 99%

Cerebral astroblastoma with oligodendroglial-like cells：A case report

Gu¹,

Yu²,

Xu³

et al. 2020

Preprint

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Background: Astroblastoma is a rare tumor of the central nervous system, with unclear biological behavior and origin. Its histopathological features have been well established. However, to the best of our knowledge, astroblastoma with oligodendroglial-like cells have not been reported. Case presentation: Herein, we reported a case of astroblastoma with atypical pathological features. A 15-year-old girl presented with nausea, vomiting, headache and visual disturbance. Magnetic resonance imaging (MRI) revealed a large neoplasm in the left temporal lobe. Histologically, the tumor showed solid and pseudopapillary structure. The tumor cells were radially arranged around blood vessels, with a single, prominent process, and as astroblastic pseudorosettes. More importantly, typical oligodendroglial-like cells were observed. In addition to membrane staining for EMA, immunohistochemical staining also showed that the tumor cells were positive for GFAP and Vimentin. The oligodendroglial-like cells were positive for GFAP, Vimentin, and Olig-2. The Ki-67 labeling index was about 4%. Sequencing for IDH1 codon 132 and IDH2 codon 172 gene mutations showed negative results. Furthermore, fluorescent analysis revealed neither 1p nor 19q deletion in the lesion. Based on these findings, the tumor was finally diagnosed as astroblastoma. Conclusions: Herein, we reported an extremely rare case of astroblastoma, which was morphologically characterized by the appearance of oligodendroglial-like cells.

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“…We read with great interest the article "Diffuse leptomeningeal glioneuronal tumour (DLGNT) with hydrocephalus as an initial symptom: a case-based update" by Chen et al [1] published recently in Child's Nervous System. The authors have described a case report and systematic review of the relatively newly described rare entity-diffuse leptomeningeal glioneuronal tumor (DLGNT).…”

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

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

Cited by 28 publications

References 25 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

Cerebral astroblastoma with oligodendroglial-like cells：A case report

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

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