Diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature: A case report and literature review
A Diffuse Leptomeningeal Glioneuronal Tumor (DLGNT), a rare entity as classified in the World Health Organization’s Fifth Edition of the Classification of Tumors of the Central Nervous System (WHO CNS5), is characterized by oligodendrocyte-like cells with MAPK pathway alterations. This report detail...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2024-06-01
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| Series: | Interdisciplinary Neurosurgery |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2214751924000185 |
| _version_ | 1827316598923329536 |
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| author | Lingxu Chen Junmei Wang Xiaochen Wang Sihui Wang Xuening Zhao Shengjun Sun |
| author_facet | Lingxu Chen Junmei Wang Xiaochen Wang Sihui Wang Xuening Zhao Shengjun Sun |
| author_sort | Lingxu Chen |
| collection | DOAJ |
| description | A Diffuse Leptomeningeal Glioneuronal Tumor (DLGNT), a rare entity as classified in the World Health Organization’s Fifth Edition of the Classification of Tumors of the Central Nervous System (WHO CNS5), is characterized by oligodendrocyte-like cells with MAPK pathway alterations. This report details the case of a 29-year-old female presenting with unique radiological features: extensive spinal cord dissemination involving both parenchyma and leptomeninges, without intracranial involvement. Near-total resection (NTR) was performed, revealing H3K27me3 positivity, a molecular characteristic not previously reported in DLGNTs. We also review recent studies to expand the understanding of DLGNT’s clinical, imaging, and molecular profiles, aiming to assist radiologists and clinicians in accurate diagnosis and timely management. |
| first_indexed | 2024-03-07T23:06:01Z |
| format | Article |
| id | doaj.art-59bb9e3da082454eb9013548b948739d |
| institution | Directory Open Access Journal |
| issn | 2214-7519 |
| language | English |
| last_indexed | 2024-04-24T23:17:12Z |
| publishDate | 2024-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Interdisciplinary Neurosurgery |
| spelling | doaj.art-59bb9e3da082454eb9013548b948739d2024-03-17T07:54:24ZengElsevierInterdisciplinary Neurosurgery2214-75192024-06-0136101972Diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature: A case report and literature reviewLingxu Chen0Junmei Wang1Xiaochen Wang2Sihui Wang3Xuening Zhao4Shengjun Sun5Beijing Neurosurgical Institute, South Fourth Ring West Road, Beijing 100070, ChinaBeijing Neurosurgical Institute, South Fourth Ring West Road, Beijing 100070, ChinaBeijing Neurosurgical Institute, South Fourth Ring West Road, Beijing 100070, ChinaBeijing Neurosurgical Institute, South Fourth Ring West Road, Beijing 100070, ChinaBeijing Neurosurgical Institute, South Fourth Ring West Road, Beijing 100070, ChinaBeijing Neurosurgical Institute, South Fourth Ring West Road, Beijing 100070, China; Department of Neuroradiology, Beijing Tiantan Hospital, South Fourth Ring West Road, Beijing 100070, China; Corresponding author at: Beijing Neurosurgical Institute, South Fourth Ring West Road, Beijing 100070, China.A Diffuse Leptomeningeal Glioneuronal Tumor (DLGNT), a rare entity as classified in the World Health Organization’s Fifth Edition of the Classification of Tumors of the Central Nervous System (WHO CNS5), is characterized by oligodendrocyte-like cells with MAPK pathway alterations. This report details the case of a 29-year-old female presenting with unique radiological features: extensive spinal cord dissemination involving both parenchyma and leptomeninges, without intracranial involvement. Near-total resection (NTR) was performed, revealing H3K27me3 positivity, a molecular characteristic not previously reported in DLGNTs. We also review recent studies to expand the understanding of DLGNT’s clinical, imaging, and molecular profiles, aiming to assist radiologists and clinicians in accurate diagnosis and timely management.http://www.sciencedirect.com/science/article/pii/S2214751924000185Diffuse leptomeningeal glioneuronal tumorNeuroimagingGlioneuronal and neuronal tumorsLeptomeningeal tumor |
| spellingShingle | Lingxu Chen Junmei Wang Xiaochen Wang Sihui Wang Xuening Zhao Shengjun Sun Diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature: A case report and literature review Interdisciplinary Neurosurgery Diffuse leptomeningeal glioneuronal tumor Neuroimaging Glioneuronal and neuronal tumors Leptomeningeal tumor |
| title | Diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature: A case report and literature review |
| title_full | Diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature: A case report and literature review |
| title_fullStr | Diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature: A case report and literature review |
| title_full_unstemmed | Diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature: A case report and literature review |
| title_short | Diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature: A case report and literature review |
| title_sort | diffuse leptomeningeal glioneuronal tumor with atypical radiological and molecular feature a case report and literature review |
| topic | Diffuse leptomeningeal glioneuronal tumor Neuroimaging Glioneuronal and neuronal tumors Leptomeningeal tumor |
| url | http://www.sciencedirect.com/science/article/pii/S2214751924000185 |
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